Beruflich Dokumente
Kultur Dokumente
Jeffrey S. Kreutzer
John DeLuca
Bruce Caplan
Editors
Encyclopedia of
Clinical Neuropsychology
With 199 Figures and 139 Tables
Editors
Jeffrey S. Kreutzer, PhD, ABPP, FACRM Bruce Caplan, PhD, ABPP
Rosa Schwarz Cifu Professor of Independent Practice
Physical Medicine and Rehabilitation, and Professor of 564 M.O.B. East, 100 E. Lancaster Ave.
Neurosurgery, and Psychiatry Virginia Commonwealth Wynnewood, PA 19096
University Medical Center USA
Department of Physical Medicine and Rehabilitation brcaplan@aol.com
VCU
P.O. Box 980542
Richmond, Virginia 23298-0542
USA
jskreutz@vcu.edu
All rights reserved. This work may not be translated or copied in whole or in part without the written permission of
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The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such,
is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights.
While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the
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Product liability: The publishers cannot guarantee the accuracy of any information about dosage and application contained in
this book. In every individual case the user must check such information by consulting the relevant literature.
David Michael Scott, in graduate school, first helped me appreciate the importance of learning about the brain and
nervous system. Alexander Manning taught me about the brain and standardized neuropsychological assessment.
Donald Kausch taught me how to administer and interpret the Halstead Reitan and tests designed by Arthur Benton.
My internship supervisors, Muriel Lezak, Larry Binder, Diane Howieson, Richard Erickson, Orin Bolstad, David Shaw,
and Julian Taplin taught me so much about neuropsychology, how to work with families, and inspired me on to a
career in the field. Jeffrey T. Barth, Ronald Ruff, and Harvey Levin, colleagues I worked with on the Traumatic Coma
Data Bank project, helped me learn neuropsychological research methods, brain injury, and how to be patient and
tenacious. Mitchell Rosenthal, Paul Wehman, and Henry Stonnington taught me about rehabilitation, teamwork,
hope, and how to be practical.
JSK
As an undergraduate, Martin Hahn lit the fire within me regarding an interest in science, which launched my pursuit for
advanced education in brain-behavior relations. Dick Burright taught me about critical thinking in science, through a lot
of hard work. Peter Donovick guided me through graduate school, even when the path ahead seemed unclear, and was
the primary reason I discovered my ultimate career path in human neuropsychology. Keith Cicerone rounded out my
skills and provided me with the finishing touches in my education and training and helped me carve out my particular
niche in neuropsychology, which included both research and clinical activities. Joel DeLisa provided me with the fertile
environment I needed to launch my career in clinical neuropsychology, particularly by supporting a research environ-
ment based on my own interests, approach, and skills, allowing me to pursue a career first dreamed as an undergradu-
ate. Lastly and perhaps most importantly, I dedicate this work to all of my post-doctoral and pre-doctoral trainees, too
numerous to list, who have challenged me professionally and personally, and by far have had the most influence on the
success in my career. I hope that I have had but a fraction of an influence on theirs.
JDL
To: Marcel Kinsbourne, who gets the credit (and blame) for awakening my interest in neuroscience in general and
neuropsychology in particular and exposing me to world-class intellects; Leonard Diller, from whom I learned
the joy of immersion in and struggle to master both the historical and contemporary neuropsychology and
rehabilitation literature; Charles Gibson, who gave me (in retrospect, perhaps unwisely) an inordinate amount of
professional freedom in my first real job; Mitchell Rosenthal, who embodied the lesson I learned from my father,
Jerome Caplan, (Be kind, because everyone you meet is fighting a hard battle) and encouraged me to do the same; and
listed last, but most important, my multifaceted partner, Judy Shechter, my intellectual boomerang colleague and
constant source of entertainment.
BC
Acknowledgement
The conceptualization, compilation, and production of the Encyclopedia of Clinical Neuropsychology spanned more
than four years. We set out to develop a uniquely comprehensive, authoritative, indispensable reference work, and we
are hopeful that our goal has been achieved. We owe an immeasurable debt to the many people who supported us
through the course of the project. Foremost, we are grateful to our families for their enthusiastic support, encourage-
ment, and patience. We are indebted to our cadre of esteemed Associate Editors for helping to develop their sections,
recruit contributors, and ensure the presence of consistently high quality entries. We express our appreciation to the
brilliant group of authors whose efforts form the core of our project. We are immensely indebted to the superb Springer
major reference works team including Janice Stern, Anil Chandy, Lydia Mueller, and Oona Schmid who taught us,
encouraged us, kept us organized and on track, and helped us every step of the way. We are also grateful to our students,
patients, and their families from whom we learned much about facing challenges and the value of being practical.
Jeffrey S. Kreutzer
John DeLuca
Bruce Caplan
Preface
It is doubtful that there is a more rapidly evolving psychological specialty than clinical neuropsychology. Every day,
clinicians are challenged to help patients with a widening variety of cognition-compromising disorders including
traumatic brain injury, vascular conditions, brain tumors, developmental disabilities, psychiatric disturbances, and
neurodegenerative disorders. Some practitioners serve pediatric populations, others treat the elderly, and many serve
general adult populations. Some patients have progressive disorders, while others can achieve substantial improvement
over time. Assessment is typically the starting point, with clinicians addressing a myriad of referral questions, which may
relate to the patients ability to work, return to school, manage personal affairs, drive, live independently, or be
considered eligible for disability benefits. Increasingly, clinicians are involved in civil and forensic proceedings,
contributing to decisions about responsibility, competence, and entitlement to damages for injury.
In fulfilling its clinical mandates, clinical neuropsychology relies strongly on its research base. As a hybrid of
cognitive psychology, neuroscience and clinical psychology, clinical neuropsychology investigations are at the forefront
of translational research in brain-behavior relations. The future of both clinical practice and research lies with our
trainees at all levels undergraduate, doctoral and post-doctoral. Easily accessible and frequently updated knowledge
in clinical neuropsychology provides the foundation for the education and training of our future clinical neuropsychol-
ogists. A fundamental aim of this work has been to provide such a resource and, with the online version, to permit
revision and expansion as the field evolves.
Most neuropsychological reference books focus primarily on assessment, diagnosis, functional neuroanatomy, and
descriptions of various disease entities and their higher cortical consequences. To date, none has been encyclopedic in
format. We see it as a mark of the maturity of the field that such a multi-volume publication is now warranted.
Clinicians, patients, family members, researchers and students all recognize that evaluation and diagnosis is only a
starting point for the treatment and restoration process. Few would be satisfied with an end-product consisting only of a
diagnosis and/or description of the patients cognitive topography. During the past decade, treatment services have
proliferated, and neuropsychologists have been in the forefront of these developments because of their special training
and experience. Neuropsychological clinicians now provide a variety of services in addition to assessment including
psychological counseling, neurobehavioral management, cognitive rehabilitation, family intervention, and vocational
rehabilitation in hospitals and community-based settings. In view of this expanded scope of contemporary practice, we
envisioned an encyclopedia containing information pertinent to these activities.
This encyclopedia will serve as a unified, comprehensive reference for professionals involved in the diagnosis,
evaluation, and rehabilitation of children and adults with neuropsychological disorders. It will also provide students and
scientists with the breadth of knowledge needed to build a scientific basis for interventions and treatment for patients.
We hope Encyclopedia of Clinical Neuropsychology is the first place readers turn for factual, relevant, and comprehen-
sive information to aid in delivering the highest quality services.
September 2010
Jeffrey S. Kreutzer
John DeLuca
Bruce Caplan
Editors
Paul Malloy
Michael Franzen
The Warren Alpert Medical School of Brown University
Allegheny Neuropsychiatric Institute
Butler Hospital
Allegheny General Hospital
345 Blackstone Blvd.
4 Allegheny Center
Providence, RI 2906
Pittsburgh, PA 15212
USA
USA
PMalloy@Butler.org
mfranzen@wpahs.org
Douglas I. Katz
Boston University School of Medicine
Sarah A. Raskin
Braintree Rehabilitation Hospital
Department of Psychology and Neuroscience Program
250 Pond Street
Trinity College
Braintree, MA 2184
Hartford, CT 6106
USA
USA
dkatz@bu.edu
Sarah.Raskin@trincoll.edu
Stephanie A. Kolakowsky-Hayner
Director, Rehabilitation Research Stephanie Reid-Arndt
Santa Clara Valley Medical Center School of Health Professions - Health Psychology
Rehabilitation Research Center Ellis Fischel Cancer Center
751 South Bascom Ave. University of Missouri-Columbia
San Jose, CA 95128 Columbia, MO 65211
USA USA
Stephanie.Hayner@hhs.sccgov.org reidarndts@health.missouri.edu
Associate Editors xv
Anthony Y. Stringer
Department of Rehabilitation Medicine
Emory University
1441 Clifton Road NE
Atlanta, GA 30322
USA
Anthony.Stringer@emoryhealthcare.org
List of Contributors
GALYA ABDRAKHMANOVA BRITTANY J. ALLEN
Department of Pharmacology Department of Health Psychology, DC 116.88
Virginia Commonwealth University University of Missouri, Columbia
1112 E. Clay Street, P.O. Box 980524 One Hospital Drive
Richmond, VA 23298-0565 Columbia, MO 65212
USA USA
gabdrakhmano@vcu.edu allenbj@health.missouri.edu
THOMAS M. ACHENBACH
JASON VAN ALLEN
University of Vermont
Clinical Child Psychology Graduate Program
2 Colchester Ave.
University of Kansas
Burlington, VT 05405-0134
1000 Sunnyside Ave
USA
Lawrence, KS 66045
thomas.achenbach@uvm.edu
USA
jvanallen@ku.edu
RUSSELL ADAMS
Department of Psychiatry and Behavioral Science
University of Oklahoma Health Sciences Center KARIN ALTERESCU
P.O. Box 26901 Neuropsycholgy Program
Oklahoma City, OK 73190 Queens College and The Graduate Center of the City
USA University of New York
russell-adams@ouhsc.edu Flushing, NY 11367
USA
karin.alterescu@yahoo.com
CRISTY AKINS
Mercy Family Center
110 Vetrans Memorial Blvd AKSHAY AMARANENI
Metarie, LA 70005 Department of Rehabilitation Medicine
USA Emory University
cristy.akins@gmail.com Atlanta, GA 30322
USA
akshay982@gmail.com
AMY ALDERSON
Emory University/Rehabilitation Medicine
1441 Clifton Road
MELISSA AMICK
Atlanta, GA 30322
Department of Psychiatry and Human Behavior
USA
Brown University
amyalderson@gmail.com
Providence, RI 02912
USA
DANIEL N. ALLEN and
Department of Psychology Department of Medical Rehabilitation
University of Nevada Las Vegas Memorial Hospital of Rhode Island
Box 455030; 4505 Maryl and Parkway 111 Brewster Street
Las Vegas, NV 89154-5030 Pawtucket, RI 02860
USA USA
daniel.allen@unlv.edu Melissa_Amick@brown.edu
xviii List of Contributors
GLENN S. ASHKANAZI
STEVEN W. ANDERSON Department of Clinical and Health Psychology
University of Iowa Hospitals and Clinics University of Florida-College of Public Health and
0080-C RCP, 200 Hawkins Drive Health Professions
Iowa City, Iowa 52242 P.O. Box 100165
USA Gainesville, FL 32610-0165
steven-anderson@uiowa.edu USA
gashkana@phhp.ufl.edu
KEVIN M. ANTSHEL
STEPHANIE ASSURAS
Department of Psychiatry and Behavioral Sciences
Neuropsychology Program
Upstate Medical University
Queens College and The Graduate Center of the City
750 East Adams Street
University of New York
Syracuse, NY 13210
Flushing, NY 11367
USA
USA
and
stephassuras@hotmail.com
State University of New York - Upstate Medical
University
1752 Greenspoint Court Syracuse
JANE AUSTIN
Mount Pleasant, SC 29466
Department of Psychology
USA
William Paterson University
antshelk@upstate.edu
300 Pompton Road
Wayne, NJ 7470
USA
JENNIFER ANN NISKALA APPS austinj@wpunj.edu
Department of Psychiatry & Behavioral Medicine
Childrens Hospital of Wisconsin/Medical College of
Wisconsin BRADLEY AXELROD
9000 W Wisconsin Ave Ste B510 John D. Dingell VA Medical Center
Milwaukee, WI 53226 Psychology Section
USA 4646 John R Street
JApps@chw.org Detroit, MI 48201
USA
Bradley.Axelrod@va.gov
CAROL L. ARMSTRONG
The Childrens Hospital of Philadelphia GLEN P. AYLWARD
Neuro-Oncology/Neuropsychology SIU School of Medicine-Pediatrics
3535 Market Street, Ste. 1409-1410 P.O. Box 19658
Philadelphia, PA 19104 Springfield, IL 62794-9658
USA USA
armstrongc@email.chop.edu gaylward@siumed.edu
List of Contributors xix
THOMAS G. BURNS
CHARLES D. CALLAHAN
Neuropsychology
Memorial Medical Center
Childrens Healthcare of Atlanta
701 N. 1st Street
1001 Johnson Ferry Road NE
Springfield, IL 62781
Atlanta, GA 30342
USA
USA
Callahan.Chuck@mhsil.com
thomas.burns@choa.org
SHANE S. BUSH
BRUCE CAPLAN
Long Island Neuropsychology, P.C
Independent Practice
290 Hawkins Avenue, Suite B
564 M.O.B. East
Lake Ronkonkoma, NY 11779
100 E. Lancaster Ave.
USA
Wynnewood, PA 19096
drbush@gmail.com
USA
brcaplan@aol.com
TAMARA BUSHNIK
Rusk Institute for Rehabilitation Medicine
NYU Langone Medical Center NOELLE E. CARLOZZI
400 East 34th Street, RR115A Outcomes & Assessment Research Laboratory
New York, NY 10016 Kessler Foundation Research Center
USA 1199 Pleasant Valley Way
Tamara.Bushnik@nyumc.org West Orange, NJ 7052
USA
ncarlozzi@kesslerfoundation.org
MELISSA BUTTARO
Department of Psychiatry
Brown University, The Miriam Hospital
164 Summit Ave HELEN M. CARMINE
Providence, RI 2906 ReMed
USA Paoli, PA
MButtaro@lifespan.org USA
List of Contributors xxiii
WOON CHOW
Anatomy & Neurobiology
JENNIFER CASS Virginia Commonwealth University
Department of Pediatrics Box 980709
Nationwide Childrens Hospital and The Ohio State Richmond, VA
University USA
700 Childrens Drive wchow@vcu.edu
Columbus, OH 43205
USA
jennifer.cass@nationwidechildrens.org SHAWN E. CHRIST
University of Missouri
25 McAlester Hall
Columbia, MO 65211-2500
AMIRAM CATZ
USA
DEPARTMENT OF SPINAL
christse@missouri.edu
LOEWENSTEIN REHABILITATION HOSPITAL
278 ACHVZA STREET
RAANANA 43100
SEVERN B. CHURN
ISRAEL
Neurology
AND
Virginia Commonwealth University
TEL-AVIV UNIVERSITY
Box 980599, MCV Station
TEL-AVIV
Richmond, VA 23298-0599
ISRAEL
USA
amcatz@post.tau.ac.il
schurn@vcu.edu
URAINA CLARK
SANDY SUT IENG CHEANG The Warren Alpert Medical School of Brown University
University of Macau The Miriam Hospital
Department of Psychology Neuropsychology, The CORO Center, 3rd Floor
Av. Padre Tomas Pereira 1 Hoppin Street, Suite 317
Taipa, Macau SAR Providence, RI 2903
China USA
sandycheang@ymail.com UClark@lifespan.org
xxiv List of Contributors
RAY COLELLO
JOYCE A. CORSICA
Anatomy & Neurobiology
Department of Behavioral Sciences
Virginia Commonwealth University
Rush University Medical Center
Box 980709
1653 W. Congress Parkway
Richmond, VA
Chicago, IL 60612
USA
USA
rcolello@vcu.edu
Joyce_A_Corsica@rsh.net
GRACE COMBS
Applied Psychology and Counselor Education H. BRANCH COSLETT
Department of Psychology, FSL Department of Neurology
University of Northern Colorado University of Pennsylvania, HUP
McKee 248, Box 131 3400 Spruce Street
Greeley, CO 80631 Philadelphia, PA 19104
USA USA
graciecombs@gmail.com hbc@mail.med.upenn.edu
List of Contributors xxv
LAURA CRAMER-BERNESS
KRISTEN DAMS-OCONNOR
Department of psychology
Mount Sinai School of Medicine
William Paterson University
Department of Rehabilitation Medicine
300 Pompton Road
5 East 98th Street Rm B-14
Wayne, NJ 7470
New York, NY 10029-6574
USA
USA
bernessl@wpunj.edu
kristen.dams-oconnor@mountsinai.org
JUDY CREIGHTON
ANDREW S. DAVIS
Neuropsychology Program
Department of Educational Psychology
Queens College and The Graduate Center of the City
Ball State University
University of New York
Teachers College Room 524
Flushing, NY
Muncie, IN 47306
USA
USA
judybarry01@gmail.com
davis@bsu.edu
MOIRA C. DUX
LAUREN R. DOWELL
Rosalind Franklin School of Medicine
Laboratory for Neurocognitive and Imaging Research
University of Maryland Medical Center/Baltimore VA
Kennedy Krieger Institute
226 S. Ann Street
1750 E. Broadway, 3rd Floor
Baltimore, MD 21231
Baltimore, MD 21205
USA
USA
moira.dux@students.rosalindfranklin.edu
dowell@kennedykrieger.org
ALEKSEY DUMER
Queens College and The Graduate Center of the City ANGELA EASTVOLD
University of New York Department of Psychology
NSB A340 University of Utah
6530 Kissena Blvd. Salt Lake City, UT 84112-0251
Flushing, NY 11367 USA
USA angela.eastvold@psych.utah.edu
a.dumer@gmail.com
DAWN M. EHDE
MARY DUNKLE
Department of Rehabilitation Medicine
National Organization for Rare Disorders (NORD)
University of Washington
55 Kenosia Avenue, P.O. Box 1968
Seattle, WA 98195
Danbury, CT 06813-1968
USA
USA
ehde@u.washington.edu
mdunkle@rarediseases.org
DEBORAH A. FEIN
University of Connecticut
DANIEL ERIK EVERHART
406 Babbidge Raod, Unit 1020
Department of Psychology
Storrs, CT 06269-1020
Eastern Carolina University
USA
Rawl Bldg, East 5th Street
deborah.fein@uconn.edu
Greenville, NC 27858
USA
everhartd@ecu.edu
LEILANI FELICIANO
Department of Psychology
University of Colorado at Colorado Springs
NATHAN EWIGMAN Colorado Springs, CO
Department of Clinical and Health Psychology USA
University of Florida lfelicia@uccs.edu
Gainesville, FL 32611
USA
newigman@gmail.com WARREN L. FELTON
Neurology
Virginia Commonwealth University Medical Center
NATHALIE DE FABRIQUE Box 980599
Cook County Department of Corrections Richmond, VA
750 N. Dearborn Street, #1504 USA
Chicago, IL 60610 wfeltoniii@mcvh-vcu.edu
USA
ndefabrique@aol.com
ERIC M. FINE
University of California, San Diego
School of Medicine
JOSEPH E. FAIR
San Diego Veterans Affairs Healthcare System
Brigham Young University
1616 9th Ave. Apt. #23
2062 Dakota Ave
La Jolla, CA 92101
Provo, UT 84606
USA
USA
fine.eric@gmail.com
jfairmail@gmail.com
JESSICA FISH
JAELYN R. FARRIS Medical Research Council Cognition and Brain Sciences
Department of Psychology Unit
University of Notre Dame 15 Chaucer Road
Notre Dame, IN 46556 Cambridge, CB2 7EF
USA UK
jfarris@nd.edu jessica.fish@mrc-cbu.cam.ac.uk
List of Contributors xxix
BONNY J. FORREST
FAYE VAN DER FLUIT San Diego Center for Children
University of Wisconsin-Milwaukee 311 4th Avenue Suite 609
Department of Psychology, Gerland Hall San Diego, CA 92111
P.O. Box 413 USA
Milwaukee, WI 53201-0413 bforrest@centerforchildren.org
USA
vanderf2@uwm.edu MICHAEL A. FOX
Anatomy & Neurobiology
Virginia Commonwealth University Medical Center
JAMES R. FLYNN Box 980709
Department of Politics Richmond, VA
The University of Otago USA
P.O. Box 56 mafox@vcu.edu
Dunedin
New Zealand LISA M. FOX
jim.flynn@stonebow.otago.ac.nz Rusk Institute of Rehabilitative Medicine
NYU Langone Medical Center, Psychology Department
400 E. 34th Street
KRISTIN JOAN FLYNN PETERS New York, NY 10016
Department of Health Psychology USA
University of Missouri Health Care, School of Health lisa.fox@nyumc.org
Professions
One Hospital Dr., DC 116.88 LAURA L. FRAKEY
Columbia, MO 65212 Memorial Hospital of Rhode Island and Alpert Medical
USA School of Brown University
flynnpetersk@health.missouri.edu Pawtucket, RI
USA
lfrakey@gmail.com
NANCY S. FOLDI
Psychology Program ROBERT G. FRANK
Queens College and The Graduate Center of the City College of Public Health
University of New York Kent State University
65-30 Kissena Blvd P.O. Box 5190
Flushing, NY 11367 Kent, OH 44242-0001
USA USA
and rgfrank@kent.edu
xxx List of Contributors
GLEN GETZ
SHERRI GALLAGHER
Department of Psychiatry
Flagstaff Unified School District
Allegheny General Hospital
2910 N. Prescott Road
Four Allegheny Center
Flagstaff, AZ 86001
Pittsburgh, PA 15212
USA
USA
sherrigallagher@hotmail.com
ggetz@wpahs.org
ASSAWIN GONGVATANA
EMILIE GODWIN
Neuropsychology
Virginia Commonwealth University
Brown University
1223 East Marshall Street
The Miriam Hospital, Coro Bldg. 3-West
Richmond, VA 23298-0542
One Hoppin Street
USA
Providence, RI 02906
eegodwin@vcu.edu
USA
assawin@mac.com
GARY GOLDBERG
Virginia Commonwealth University School of Medicine/ DANIEL GOOD
Medical College of Virginia Brigham Young University
Richmond, VA 395 North 100 East
USA Provo, UT 84062
gary.goldberg.md@gmail.com USA
dag1978@hotmail.com
KIMBERLY A. GORGENS
DIANE CORDRY GOLDEN
Graduate School of Professional Psychology
Association of Assistive Technology Act Programs
University of Denver, MSC 4104
P.O. Box 32
2450 South Vine Street, MSC 4101
Delmar, NY 12054
Denver, CO 80208
USA
USA
dianegolden@sbcglobal.net
kgorgens@du.edu
MICHAEL R. GREHER
MARTIN HAHN
National Jewish Health and University of Colorado
Department of Biology
Denver School of Medicine
William Paterson University
Denver, CO
Wayne, NJ 7470
USA
USA
drgreher@comcast.net
HahnM@wpunj.edu
KATHRINE HAK
MAUREEN GRISSOM
Applied Psychology and Counselor Education
University of Missouri, Department of Health
University of Northern Colorado
Psychology MU Thompson Center for Autism and
McKee 248, Box 131
Neurodevelopmental Disorders
Greeley, CO 80631
300 Portland Street Suite 110
USA
Columbia, MO 65211
Kathrine.Hak@unco.edu
USA
grissommo@health.missouri.edu
MARLA J. HAMBERGER
New York Presbyterian
ELIZABETH STANNARD GROMISCH Columbia Comprehensive Epilepsy Center
Trinity College The Neurological Institute
1005 Smith Ridge Road Columbia University
Hartford, CT 6840 710 West 168 Street, 7th floor
USA New York, NY 10032
elizabeth.gromisch@trincoll.edu USA
mh61@columbia.edu
JANNA L. HARRIS
Hoglund Brain Imaging Center NATHAN HENNINGER
University of Kansas Medical Center Department of Pediatrics
3901 Rainbow Blvd. Mail Stop 1052 Nationwide Childrens Hospital
Kansas City, KS 66160 College of Medicine, Ohio State University
USA 700 Childrens Drive
jharris2@kumc.edu Columbus, OH 43205
USA
Nathan.Henninger@nationwidechildrens.org
ERIC S. HART
University of Missouri Center for Health Care Quality
MARY HIBBARD
Clinical Support and Education Building
Rusk Institute of Rehabilitation Medicine
Columbia, MO 65212
New York, NY 10016
USA
USA
harte@health.missouri.edu
mary.hibbard@mssm.edu
TRISHA HAY
YVONNE HINDES
Hoglund Brain Imaging Center
Division of Applied Psychology
University of Kansas Medical Center
Faculty of Education, University of Calgary
3901 Rainbow Blvd
2500 University Drive N.W
Kansas City, KS 66160
Calgary, AB T2N 1N4
USA
Canada
thay@kumc.edu
ylhindes@ucalgary.ca
ELISE K. HODGES
ROBERT L. HEILBRONNER Department of Psychiatry
Chicago Neuropsychology Group University of Michigan Health System
333 N. Michigan Avenue, #1801 Neuropsychology Division
Chicago, IL 60601 2101 Commonwealth, Suite C
USA Ann Arbor, MI 48105
rheilbronn@aol.com USA
r-heilbronner@northwestern.edu ekhodges@med.umich.edu
xxxiv List of Contributors
DAVID HULAC
KARIN F. HOTH
Division of Counseling and Psychology in Education
National Jewish Medical and Research Center
University of South Dakota
National Jewish Health
414 E. Clark Street
Denver, CO
Vermillion, SD 57069
USA
USA
psysocmed@njc.org
David.Hulac@usd.edu
EDWARD E. HUNTER
MARIANNE HRABOK
Department of Psychiatry and Behavioral Sciences
Department of Psychology
University of Kansas Medical Center
University of Victoria
3901 Rainbow Boulevard
P.O. Box 3050, STN CSC
Kansas City, KS 66160
Victoria, BC V8W 3P5
USA
Canada
ehunter@kumc.edu
mhrabok@uvic.ca
SCOTT J. HUNTER
LEESA V. HUANG Department of Psychiatry & Behavioral Neuroscience
Department of Psychology-0234 University of Chicago
California State University 5841 S Maryland Ave., MC 3077
400 West First Street Chicago, IL 60637
Chico, CA 95928-9924 USA
USA shunter@yoda.bsd.uchicago.edu
leesahuang@yahoo.com chgohunt@mac.com
KAREN HUX
DAWN H. HUBER Special Education and Communication Disorders
Pediatric Neuropsychological Services, LLC University of Nebraska Lincoln
1829 S. Kentwood, Suite 108 318N Barkley Memorial Center
Springfield, MO 65804 Lincoln, NE 68583-0738
USA USA
pnsllc@att.net khux1@unl.edu
List of Contributors xxxv
FARZIN IRANI
Psychiatry LISA A. JACOBSON
University of Pennsylvania Department of Neuropsychology
3400 Spruce street, 10 Gates Kennedy Krieger Institute
Philadelphia, PA 19104 Johns Hopkins University School of Medicine
USA 1750 East Fairmount Ave.
firani@upenn.edu Baltimore, MD 21231
USA
jacobson@kennedykrieger.org
CINDY B. IVANHOE
Neurorehabilitation Specialists
KELLY M. JANKE
Baylor College of Medicine
University of Wisconsin-Milwaukee
The Institute for Rehabilitation and Research
Department of Psychology
1333 Moursund Avenue, D110
P.O. Box 413
Houston, TX 77030
Milwaukee, WI 53201-0413
USA
USA
cbivanhoe@att.net
kmz@uwm.edu
GRANT L. IVERSON
NICHOLAS JASINSKI
Department of Psychiatry
Division of Neuropsychology
University of British Columbia
Henry Ford Health System
British Columbia Mental Health & Addictions
1 Ford Place
2255 Wesbrook Mall
Detroit, MI 48202
Vancouver, BC V6T 2A1
USA
Canada
NJasins1@HFHS.ORG
giverson@interchange.ubc.ca
BETH A. JERSKEY
COLLEEN E. JACKSON Department of Psychiatry and Human Behavior
Department of Psychology Alpert Medical School of Brown University
University of Connecticut Butler Hospital
406 Babbidge Road, Unit 1020 Blackstone Blvd.
Storrs, CT 6269 Providence, RI 2906
USA USA
colleen.jackson@uconn.edu Beth_Jerskey@brown.edu
ERIN JOYCE
MI-YEOUNG JO Pacific Graduate School of PsychologyStanford Doctor
Private Practice of Psychology Consortium
15353 Valerio Street Spinal Cord Injury Clinic
Van Nuys, CA 91406 VA Palo Alto Health Care System
USA 3801 Miranda Ave. (128)
myjo_9@yahoo.com Palo Alto, CA 94304
USA
EEJPsyD@Stanford.edu
SUSAN K. JOHNSON
Department of Psychology AARON N. JUNI
University of North Carolina at Charlotte Neuropsychology and Rehabilitation Psychology
9201 University City Blvd. Department of Physical Medicine & Rehabilitation
Charlotte, NC 28223-0001 The Johns Hopkins School of Medicine
USA 600 North Wolfe Street/Phipps 174
skjohnso@uncc.edu Baltimore, MD 21287
USA
ajuni1@jhmi.edu
JULENE K. JOHNSON
UCSF Epilepsy Center STEPHEN M. KANNE
University of California Thompson Center for Autism and Neurodevelopmental
400 Parnassus Avenue Disorders
San Francisco, CA 94143-0138 University of Missouri
USA 300 Portland, Suite 110
jjohnson@memory.ucsf.edu Columbia, MO 65211
USA
kannest@missouri.edu
JUDY A. JOHNSON
Pasadena Independent School District RICHARD F. KAPLAN
29731 Sullivan Oaks Drive Department of Psychiatry (MC-2103)
Pasadena, TX 77386 UConn Health Center
USA 263 Farmington Ave
jjohnson00708831@comcast.net Farmington, CT 06030-2103
USA
kaplan@psychiatry.uchc.edu
NANCY JOHNSON
Cognitive/Behavioral Neurology Center PAUL E. KAPLAN
Northwestern Feinburg School of Medicine Capitol Clinical Neuroscience
675 N. Street Clair, Galter 20-100 104 Summer Shade Court
Chicago, IL 60611 Folsom, CA 95630-1565
USA USA
johnson-n@northwestern.edu paulek_2000@yahoo.com
List of Contributors xxxvii
KIMBERLY A. KERNS
NARINDER KAPUR Department of Psychology
Neuropsychology Department University of Victoria
Addenbrookes Hospital Victoria, BC V8W 3P5
R3 Neurosciences, Box 83 Canada
Cambridge, CB2 0QQ kkerns@uvic.ca
UK
narinder.kapur@addenbrookes.nhs.uk
FARY KHAN
Department of Medicine
STELLA KARANTZOULIS University of Melbourne and the Royal Melbourne
Neuropsychology Program Hospital
NYU Langone Medical Center Bldg 21, Royal Park Campus
City University Parkville, VA, VIC 3152
New York, NY Australia
USA Fary.Khan@mh.org.au
skarantz@gmail.com
SO HYUN KIM
DOUGLAS I. KATZ University of Michigan Autism and Communication
Boston University School of Medicine Disorders Center (UMACC)
Braintree Rehabilitation Hospital 2236 East Hall
250 Pond Street Ann Arbor, MI 48109-0406
Boston, MA 2184 USA
USA sohkim@umich.edu
dkatz@bu.edu
TRICIA Z. KING
MICHAEL KAUFMAN Georgia State University
Department of Neurology Department of Psychology
Carolinas Medical Center 140 Decatur Street, Suite 1151
1010 Edgehill Road North Atlanta, GA 30303
Charlotte, NC 28207-1885 USA
USA tzking@gsu.edu
Michael.Kaufman@carolinashealthcare.org
BRAD KUROWSKI
ELIZABETH KOZORA
Cincinnati Childrens Hospital Medical Center
Department of Medicine
University of Pittsburgh
National Jewish Medical, and Research Center
Cincinnati, OH
National Jewish Health
USA
1400 Jackson Street
kurkowskiba@upmc.edu
Denver, CO 80208
USA
KozoraE@NJC.ORG
MATTHEW M. KURTZ
Department of Psychology
Wesleyan University
JOEL H. KRAMER
Judd Hall 314
UCSF Memory and Aging Center
Middletown, CT 6459
UCSF Med Ctr, 0984-8AC
USA
350 Parnassus Ave, Suite 706
mkurtz@wesleyan.edu
San Francisco, CA 94143
USA
jkramer@memory.ucsf.edu
MONICA KURYLO
Department of Rehabilitation Medicine
MATTHEW KRAYBILL University of Kansas Medical Center
Department of Psychology 3901 Rainbow Blvd
University of Utah Kansas City, KS 66160
Salt Lake City, UT 84112-0251 USA
USA mkurylo@kumc.edu
mkraybill@gmail.com
CHRISTINA KWASNICA
DENISE KRCH Barrow Neurological Institute
Kessler Foundation Research Center 222 W Thomas Road Ste 212
West Orange, NJ Phoenix, AZ 85013
USA USA
dkrch@kesslerfoundation.org Christina.Kwasnica@CHW.EDU
List of Contributors xxxix
SARAH K. LAGEMAN
Division of Neuropsychology and Behavioral Health THOMAS M. LAUDATE
Department of Rehabilitation Medicine Boston University
Emory University Brigham and Womens Hospital
1441 Clifton Road NE 648 Beacon Street, 2nd Floor
Atlanta, GA 30322 Boston, MA 02215-2013
USA USA
sarah.lageman@emoryhealthcare.org tlaudate@yahoo.com
HOYLE LEIGH
SOPHIE LEBRECHT
Department of Psychiatry
Brown University
University of California, San Francisco
Visual Neuroscience Laboratory
155 N. Fresno Street
Waterman Street
Fresno, CA 93701
Providence, RI 02903
USA
USA
Hoyle.leigh@ucsf.edu
Sophie_Lebrecht@brown.edu
KANGMIN D. LEE
TERRY LEVITT
Department of Neurosurgery
Independent Practice
Virginia Commonwealth University
1324 College Drive
Box 980631
Saskatoon, Saskatchewan S7N 0W5
Richmond, VA
Canada
USA
tlevitt@sasktel.net
klee2@mcvh-vcu.edu
CASSIE LINDSTROM
CATHERINE LORD
Dept of Psychology
Autism and Communication Disorders Center (UMACC)
UNC-Charlotte
University of Michigan
9201 University City Blvd
300 North Ingalls, 10th Floor
Charlotte, NC 28223
Ann Arbor, MI 48109-0406
USA
USA
cmlinds1@uncc.edu
celord@umich.edu
N. G. LOUISA and
Department of Rehabilitation Medicine Johns Hopkins University School of Medicine
Royal Melbourne Hospital Baltimore, MD 21205
Parkville, Victoria USA
Australia mahone@kennedykrieger.org
Louisa.Ng@mh.org.au
BRI MAKOFSKE
STEPHEN D. LUKE Applied Psychology and Counselor Education
National Dissemination Center for Children with University of Northern Colorado
Disabilities (NICHCY) McKee 248, Box 131
Washington, DC Greeley, CO 80631
USA USA
sluke@aed.org vonf5072@blue.unco.edu
AMIT MALHOTRA
JON G. LYON
Kaiser Permanente Medical Center
6344 Hillsandwood Road
280 West MacArthur Boulevard
Mazomanie, WI 53560
Oakland, CA 94611-5693
USA
USA
LyonBlanc@aol.com
amit.x.malhotra@kp.org
DONALD E. LYTLE
Department of Psychology PAUL MALLOY
California State University The Warren Alpert Medical School of Brown University
400 West First Street Butler Hospital
Chico, CA 95928-0234 345 Blackstone Blvd.
USA Providence, RI 2906
DLytle@csuchico.edu USA
PMalloy@Butler.org
ANNA MACKAY-BRANDT
Department of Psychiatry and Human Behavior WILLIAM VICTOR MALOY
Brown University Medical School The Virginia Institute of Pastoral Care
78 Dana Street 2000 Bremo Road, Suite 105
Providence, RI 2906 Richmond VA 23226
USA USA
anna.mackay@gmail.com wvm.vipcare@verizon.net
BERNICE A. MARCOPULOS
Department of Psychiatry and Neurobehavioral DAVID MCCABE
Sciences Queens College and The Graduate Center of the City
University of Virginia, Director, Neuropsychology Lab University of New York
Western State Hospital Department of Psychology
Box 2500 65-30 Kissena Blvd.
Charlottesville, VA 24402-2500 Flushing, NY 11367
USA USA
Bernice.Marcopulos@wsh.dmhmrsas.virginia.gov davidlmc@gmail.com
CHRISTINA R. MARMAROU
Neurosurgery REBECCA MCCARTNEY
Virginia Commonwealth University Emory University/Rehabilitation Medicine
Box 980631 1441 Clifton Road NE
Richmond, VA Atlanta, GA 30322
USA USA
crmarmar@vcu.edu beckygsu@aol.com
GUIDO MASCIALINO
Department of Rehab Medicine DALENE MCCLOSKEY
Mount Sinai School of Medicine Centennial Board of Cooperative Educational Services
5 East 98th Street 16473 Longs Peak Road
New York, NY 10029 Greeley, CO 80631
USA USA
Guido.Mascialino@mountsinai.org dalenemc@what-wire.com
MICAH O. MAZUREK
Thompson Center for Autism and Neurodevelopmental ERICA MCCONNELL
Disorders University of Northern Colorado
University of Missouri 2250 Ironton Street
300 Portland, Suite 110 Greeley, CO 80010
Columbia, MO 65211 USA
USA erica.mcconnell@hotmail.com
mazurekm@missouri.edu
DAVID E. MCINTOSH
Ball State University LINDA MCWHORTER
Department of Special Education, Teachers College Department of Psychology
Room 722 University of North Carolina at Charlotte
Muncie, IN 47306 9201 University City Blvd
USA North Carolina
demcintosh@bsu.edu Charlotte, NC 28223
USA
lmcwhor1@uncc.edu
MIECHELLE MCKELVEY
Department of Communication Disorders MARY-ELLEN MEADOWS
COE B141, University of Nebraska Kearney Division of Cognitive and Behavioral Neurology
Kearney, NE 68849 Brigham and Womens Hospital
USA 221 Longwood Ave
mckelveyml@unk.edu Boston, MA 2115
USA
mmeadows@partners.org
NICOLE C. R. MCLAUGHLIN
Butler Hospital
MICHAEL S. MEGA
Alpert Medical School of Brown University
Cognitive Assessment Clinic
345 Blackstone Blvd
Providence Brain Institute, Providence Health System
Providence, RI 02906
9427 SW Barnes Road, Suite 595
USA
Portland, OR 97225
nmclaughlin@butler.org
USA
michael.mega@providence.org
BRIAN T. MCMAHON
Department of Rehabilitation Counseling STEPHEN S. MEHARG
Virginia Commonwealth University Center for Memory and Learning
P.O. Box 980330 945 11th Ave Suite A
Richmond, VA 23298 Longview, WA 98632
USA USA
bmcbull@vcu.edu smeharg@cfmal.com
List of Contributors xlv
JO ANN PETRIE
RICK PARENTE
Brigham Young University
Department of Psychology
Provo, UT
Towson University
USA
Towson, MD
joann_petrie@cortex.byu.edu
USA
FParente@towson.edu
LEADELLE PHELPS
University at Buffalo, State University of New York
MATTHEW R. PARRY 427 Baldy Hall
Virginia Commonwealth University Buffalo, NY 14260
Richmond, VA USA
USA phelps@buffalo.edu
parrymr@gmail.com
KRISTIN D. PHILLIPS
JANET PATTERSON Medical College of Wisconsin-Milwaukee
Department of Communicartive Sciences and Disorders Department of Neurology, Division of
California State University Neuropsychology
East Bay 9200 W. Wisconsin ave
25800 Carlos Bee Blvd. Milwaukee, WI 53226
Hayward, CA 94542 USA
USA kphillips@mcw.edu
janet.patterson@csueastbay.edu
LINDA L. PHILLIPS
Anatomy & Neurobiology
SHELLEY PELLETIER
Virginia Commonwealth University
Board Certified in School Psychology
Box 980709
Shoreline Pediatric Neuropsychology Services, LLC
Richmond, VA
954 Middlesex Turnpike, A2
USA
Old Saybrook, CT 6475
llphilli@hsc.vcu.edu
USA
shelley.pelletier@us.army.mil
WADE PICKREN
Ryerson University
KENNETH PERRINE Department of Psychology, American Psychological
Northeast Regional Epilepsy Group Association
104-20 Queens Blvd., Apt. 10C 350 Victoria Street
Hackensack, NJ 11375 Toronto, ON ON M5B 2K3
USA Canada
krp2003@med.cornell.edu wpickren@psych.ryerson.ca
List of Contributors xlix
DONNA POLELLE
Department of Commication Sciences and Disorders ADELE S. RAADE
Saint Xavier University Adjunct Assistant Professor
3700 W 103rd Street Boston University Department of Speech
Chicago, IL 60655 Language, & Hearing Sciences
USA 635 Commonwealth Avenue
Polelle@sxu.edu Boston, MA 2215
USA
araade@comcast.net
MATTHEW R. POWELL
Clinical Neuropsychologist
Behavioral Medicine Center VANESSA L. RAMOS
Waukesha Memorial Hospital, Neuroscience Center Department of Psychology
721 American Avenue, Suite 501 Nationwide Childrens Hospital
Waukesha, WI 53188 700 Childrens Drive
USA Columbus, OH 43205
matthew.powell@phci.org USA
Vanessa.ramos@nationwidechildrens.org
TIFFANY L. POWELL
Department of Neurosurgery KATE D. RANDALL
Virginia Commonwealth University Psychology
Box 980631 Univesity of Victoria
Richmond, VA Victoria, BC
USA Canada
tpowell@mcvh-vcu.edu krandall@uvic.ca
l List of Contributors
DANIEL E. ROHE
Mayo Clinic LINDA ROWLEY
200 First Street Southwest Waisman Center Family Village
Rochester, MN 55905 University of Madison
USA 1500 Highland Avenue
rohe.daniel@mayo.edu Madison, WI 53705-2280
USA
rowley@waisman.wisc.edu
MARYELLEN ROMERO
Assistant Professor of Psychiatry
Department of Psychiatry and Neurology DONALD ROYALL
Tulane University Health Sciences Center The University of Texas Health Center at San Antonio
1440 Canal Street, TB-53 7703 Floyd Curl Dr, Mail Code 7792
New Orleans, LA 70112 San Antonio, TX 78229-3900
USA USA
mmcclai@tulane.edu royall@uthscsa.edu
ALEXANDRA RUDD-BARNARD
JON ROSE Rusk Institute of Rehabilitative Medicine Psychology
Spinal Cord Injury Clinic New York University Langone Medical Center
Veterans Affairs Palo Alto Healthcare System Service Psych InPat
3801 Miranda Ave. (128) 550 First Avenue
Palo Alto, CA 94304 New York, NY 10016
USA USA
Jonathon.Rose@VA.Gov Alexandra.Rudd@nyumc.org
lii List of Contributors
STEPHEN P. SALLOWAY
JULIA RUTENBERG Butler Hospital
Emory University/Rehabilitation Medicine Alpert Medical School of Brown University
Atlanta VAMC RR&D CoE 345 Blackstone Blvd
Atlanta, GA Providence, RI 2906
USA USA
jrutenbe@wellesley.edu Stephen_Salloway@brown.edu
JILLIAN SCHUH
MARLA SANZONE
Department of Psychology
Independent Practice, Loyola College of Maryland
University of Connecticut
104-A Annapolis Street
406 Babbidge Road, Unit 1020
Annapolis, MD 21401
Storrs, CT 6269
USA
USA
docmerri@yahoo.com
jillian.schuh@gmail.com
LYNN A. SCHAEFER
Department of Physical Medicine and Rehabilitation CHRISTIAN SCHUTTE
Nassau University Medical Center John D. Dingell VA Medical Center
2201 Hempstead Turnpike Psychology Section (11MHPS)
East Meadow, NY 11554 4646 John R
USA Detroit, MI 48201-1916
lschaefe@numc.edu USA
Christian.Schutte@va.gov
JUDITH A. SHECHTER
LAURA SHANK 100 East Lancaster Avenue
Rehabilitation Psychology and Neuropsychology Suite 564 East
Physical Medicine & Rehabilitation Wynnewood, PA 19096
University of Michigan USA
325 E. Eisenhower Parkway jshech564@aol.com
Ann Arbor, MI 48108
USA
laurasha@med.umich.edu TAMARA GOLDMAN SHER
Institute of Psychology
Illinois Institute of Technology
3105 S. Dearborn St.
CASEY R. SHANNON
Chicago, IL 60616
University of Northern Colorado
USA
Greeley, CO
sher@iit.edu
USA
laurasha@med.umich.edu
ELISABETH M. S. SHERMAN
Alberta Childrens Hospital
ANUJ SHARMA
University of Calgary
Virginia Commonwealth University School of Medicine
Calgary, AB
Richmond, VA
Canada
USA
Elisabeth.Sherman@calgaryhealthregion.ca
sharmaa@vcu.edu
CHERYL L. SHIGAKI
SALLY E. SHAYWITZ Department of Health Psychology
Department of Pediatrics University of Missouri
Yale University School of Medicine One Hospital Drive, DC046.46
P.O. Box 208064 Columbia, MO 65212
New Haven, CT 6520 USA
USA shigakic@health.missouri.edu
sally.shaywitz@yale.edu
GERALD SHOWALTER
BENNETT A. SHAYWITZ Department of Psychiatry and Neurobehavioral
Yale University School of Medicine Sciences
P.O. Box 208064 University of Virginia School of Medicine
New Haven, CT 6520 Charlottesville, VA 22908-0203
USA USA
bennett.shaywitz@yale.edu Gerald.Showalter@wwrc.virginia.gov
List of Contributors lv
LINDA SHUSTER
West Virginia University
P.O. Box 6122 MCKAY MOORE SOHLBERG
Morgantown, WV 26506 Communication Disorders and Sciences
USA University of Oregon
lshuster@wvu.edu 5284 University of Oregon
Eugene, OR 97403
USA
SUE ANN SISTO mckay@uoregon.edu
School of Health Technology and Management
Stony Brook University
1500 Stony Brook Road
Stony Brook, NY 11794-6018 SARA S. SPARROW
USA 94, Linsley Lake Road
sue.sisto@stonybrook.edu North Branford, CT 6171
USA
and
Yale University Child Study Center
BETH SLOMINE
230 South Frontage Road
707 North Broadway
New Haven, CT 06471
Baltimore, MD 21205
USA
USA
sara.sparrow@yale.edu
slomine@kennedykrieger.org
LAUREN STUTTS
Department of Clinical and Health Psychology
SUSAN STEFFANI
University of Florida
CCC-SLP
Gainesville, FL 32611
California State University, Chico, Department of
USA
Communication Sciences and Disorders
lstutts@phhp.ufl.edu
400 West 1st Street
Chico, CA 95929-330
USA
YANA SUCHY
ssteffani@csuchico.edu
Department of Psychology
University of Utah
380 S. 1530 E., Rm. 502
TARYN M. STEJSKAL
Salt Lake City, UT 84112-0251
Department of Physical Medicine and Rehabilitation
USA
Virginia Commonwealth University Medical Center
yana.suchy@psych.utah.edu
Virginia, VA
USA
thinktaryn@gmail.com JAMES F. SUMOWSKI
Neuropsychology and Neuroscience
Kessler Medical Rehabilitation Research and Education
WILLIAM STIERS Center
Johns Hopkins University School of Medicine Kessler Foundation Research Center
5601 Loch Raven Boulevard, Suite 406 1199 Pleasant Valley Way
Baltimore, MD 21239 West Orange, NJ 7052
USA USA
wstiers1@jhmi.edu jsumowski@kesslerfoundation.org
JOAN SWEARER
Department of Neurology CLAIRE THOMAS-DUCKWITZ
University of Massachusetts Medical School University of Northern Colorado
55 Lake Avenue North 1040 Blue Spruce Drive
Worcester, MA 01655 Greeley, CO 80538
USA USA
swearerj@ummhc.org clairethomasduckwitz@gmail.com
LAWRENCE H. SWEET
JENNIFER TINKER
Department of Psychiatry and Human Behavior
Department of Psychology
Brown University, Butler Hospital
Drexel University
345 Blackstone Blvd.
3315 Market Street, 14-308
Providence, RI 2906
Philadelphia, PA 19104
USA
USA
sweet@brown.edu
jrt38@drexel.edu
RUSSELL H. SWERDLOW
MICHELLE M. TIPTON-BURTON
University of Kansas School of Medicine
Physical Medicine and Rehabilitation
Landon Center on Aging, MS 2012
Santa Clara Valley Medical Center
3901 Rainbow Blvd
751 South Bascom Avenue
Kansas City, KS 66160
San Jose, CA 95128
USA
USA
rswerdlow@kumc.edu
michelle.tipton-burton@hhs.sccgov.org
MICHAEL J. TARR
Department of Cognitive and Linguistic Sciences and JEFFREY B. TITUS
Brain Science Program Pediatric Neuropsychologist
Brown University Washington University School of Medicine
Waterman Street St. Louis Childrens Hospital
Providence, RI 2903 One Childrens Place, 3S-32
USA St. Louis, MO 63110
Michael_Tarr@Brown.EDU USA
jbt0776@bjc.org
ELLA B. TEAGUE
Neuropsychology program TERI A. TODD
Queens College and The Graduate Center Department of Kinesiology
The City University of New York California State University, Chico
65-30 Kissena Blvd 400 West 1st Street
Flushing, NY 11367 Chico, CA 95929-330
USA USA
ellabjarta@gmail.com tatodd@csuchico.edu
lviii List of Contributors
NAM TRAN
Neurosurgery GARY TYE
Virginia Commonwealth University Medical Center Neurosurgery
Box 980631 Virginia Commonwealth University
Richmond, VA Box 980631
USA Richmond, VA
namdtran1@gmail.com USA
gtye@mcvh-vcu.edu
KATHERINE TREIBER
Santa Clara Valley Medical Center
Utah State University
KATHERINE TYSON
Logan, UT 84322
Department of Psychology
USA
University of Connecticut
and
406 Babbidge Road, Unit 1020
University of Massachusetts Medical School
Storrs, CT 6269
Worcester, MA
USA
USA
katherine.tyson@uconn.edu
katietreiber@yahoo.com
ANGELA K. TROYER
JAMIE VANNICE
Division of Psychology
Applied Psychology and Counselor Education
Baycrest Centre for Geriatric Care
University of Northern Colorado
3560 Bathurst Street
McKee 248, Box 131
Toronto, ON M6A 2E1
Greeley, CO 80631
Canada
USA
atroyer@baycrest.org
vannice215@gmail.com
THEODORE TSAOUSIDES
Department of Rehab Medicine
Brain Injury Research TODD VAN WIEREN
Mount Sinai School of Medicine Disability Support Services
5 East 98th Street, Room B-15 Indiana University of Pennsylvania
New york, NY 10029 Indiana, PA
USA USA
theodore.tsaousides@mountsinai.org toddvw@iup.edu
JOANN T. TSCHANZ
Utah State University REBECCA VAURIO
Center for Epidemiologic Studies Kennedy Krieger Institute
4450 Old Main Hill 707 North Broadway
Logan, UT 84322-4440 Baltimore, MD 21205
USA USA
joann.tschanz@usu.edu vaurio@kennedykrieger.org
List of Contributors lix
SCOTT VOTA
MIEKE VERFAELLIE Neurology
Memory Disorders Research Center 151A Virginia Commonwealth University
VA Boston Healthcare System and Bosten University Box 980599
School of Medicine Richmond, VA
150 South Huntington Ave USA
Boston, MA 2130 svota@mcvh-vcu.edu
USA
verf@bu.edu
GEORGE C. WAGNER
Department of Psychology
JEAN VETTEL Rutgers University
Brown University 152 Freylinghuysen Road
Campus Box 1978 Piscataway
Providence, RI 2912 New Brunswick, NJ 8854
USA USA
Jean_Vettel@brown.edu gcwagner@rci.rutgers.edu
NATHAN D. ZASLER
ANGELA YI
Concussion Care Centre of Virginia, Ltd.
Department of Rehab Medicine
3721 Westerre Parkway, Suite B
Mount Sinai School of Medicine
Richmond, Virginia 23233
5 East 98th Street
USA
New York, NY 10029
nzasler@cccv-ltd.com
USA
angela.yi@mountsinai.org
MICHELE L. ZACCARIO
Rusk Institute FADEL ZEIDAN
New York University Langone Medical Center Department of Psychology
Pace University UNC Charlotte
339 East 28th Street 9201 University City Blvd
New York, NY 10016 Charlotte, NC 28223
USA USA
michele.zaccario@nyumc.org fzeidan@uncc.edu
List of Contributors lxiii
504 Plan
3MS
M. J. H OLCOMB 1, DAVID E. M ACINTOSH 2
Modified Mini-Mental State Examination 1
Ball State University
Muncie, IN, USA
2
Ball State University
Muncie, IN, USA
5-HTP
L-Tryptophan Definition
Current Knowledge
6MWT
A 504 plan documents accommodations for qualified stu-
Six-Minute Walk Test dents which will allow them to have opportunities similar
Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan (eds.), Encyclopedia of Clinical Neuropsychology, DOI 10.1007/978-0-387-79948-3,
# Springer Science+Business Media LLC 2011
2 A AACN Practice Guidelines
References and Readings 2nd edition (ABS-RC: 2) and the Adaptive Behavior
A
Scales-School, 2nd edition (ABS-S: 2). Current versions
American Psychological Association. (2002). Criteria for practice guide- are a comprehensive compilation of the past versions.
line development and evaluation. American Psychologist, 57, These assessments seek to develop an estimate of adaptive
10481051.
behaviors in two scales defined with personal indepen-
American Psychological Association. (2002) Ethical principles of psy-
chologists and code of conduct. American Psychologist, 57,
dence and maladaptive behaviors in individuals with in-
10601073. tellectual disabilities. Items are rated with a yes/no
American Psychological Association. (2005). Determination and docu- response, on a 03 scale, or by frequency. Historically,
mentation of the need for practice guidelines. American Psychologist, the ABS-RC: 2 was used in institutions, but it is now
60, 976978.
also used in community settings, whereas the ABS-S:
Committee on Ethical Guidelines for Forensic Psychologists. (1991).
Specialty guidelines for forensic psychologists. Law and Human
2 was designed for use in school settings.
Behavior, 15, 655665. For both the ABS-RC: 2 and the ABS-S: 2, the
The AACN practice guidelines can be found on the AACNs Web site assessment can be administered by either of two
(www.theaacn.org) and are also published in the AACNs journal: approaches. In one method, the assessment is completed
The Clinical Neuropsychologist, 21, 209231.
by a professional or paraprofessional trained to use the
scales. In the second method, the assessment is adminis-
tered by someone familiar with the individual being
evaluated. Interpretation of results must be completed
AAMD ABS: 2 by an individual with formal training in psychometrics
and these scales.
AAMD Adaptive Behavior Scales The ABS-S: 2 enables an appraisal of an individuals
ability to cope with challenges they encounter in their
school, and aids in the diagnosis of intellectual disabilities
at ages 321. There are nine subscales in the first part of
the assessment, measuring personal independence and
AAMD Adaptive Behavior Scales responsibility of daily living: independent functioning,
physical development, economic activity, language devel-
C RISTA A. H OPP 1, I DA S UE B ARON 2
1 opment, numbers and time, prevocational/vocational ac-
Inova Fairfax Hospital for Children
2 tivity, self-direction, responsibility, and socialization. The
Inova Fairfax Hospital for Children
second part of the assessment, which addresses behavioral
Falls Church, VA, USA
domains, consists of seven subscales: social behavior, con-
formity, trustworthiness, stereotyped and hyperactive
behavior, self-abusive behavior, social engagement,
Synonyms and disturbing interpersonal behavior.
The ABS-S: 2 was normed on 2,074 students with
AAMD ABS: 2; AAMR ABS-RC: 2; AAMR ABS-S: 2
intellectual disabilities and 1,254 of their peers without
intellectual disabilities. Administration takes place in an
interview format with either a parent or teacher and may
Description vary from 20 min to 2 h, dependent on the rater. Scoring
is completed by hand. Raw scores are converted into
The American Association for Mental Deficiency Adap- percentiles, standard scores, and age equivalents for each
tive Behavior Scales (AAMD ABS) is a revised edition subdomain. Five factors can be derived: Personal self-
(1993) of the original assessments that were published in sufficiency, community self-sufficiency, personal social
1969. The American Association for Mental Retardation responsibility, social adjustment, and personal adjust-
(AAMR) (formerly known as the American Association ment. Percentiles, factor standard scores, and age equiva-
for Mental Deficiency) has changed its name to American lents are then reported based on factor scores.
Association on Intellectual and Developmental Disabilities The ABS-RC: 2 is also useful for the assessment of
(AAIDD). Therefore, intellectual disabilities have replaced personal development and social behavior in individuals
mental retardation as the terminology of choice. The with intellectual disabilities, but it has been developed for
behavior scales have been published in two versions, the individuals aged 1879. Like the ABS-S: 2, the assessment
Adaptive Behavior Scales-Residential and Community, has two parts, but there are more subscales in each part.
4 A AAMD Adaptive Behavior Scales
The first part has ten subscales: independent functioning, adaptive behavior as measured in Part II was not related
physical development, economic activity, language develop- to the Vineland Adaptive Behavior Scale and Adaptive
ment, numbers and time, domestic activity, prevocational/ Behavior Inventory (ABI), other measures of maladaptive
vocational activity, self-direction, responsibility, and sociali- behaviors.
zation. The second part contains eight subscales: social
behavior, conformity, trustworthiness, stereotyped and hy-
peractive behavior, sexual behavior, self-abusive behavior,
Clinical Uses
social engagement, and disturbing interpersonal behavior.
The ABS-RC: 2 was normed on a sample of 4,000 adults with
The ABS: 2 assesses the status of individuals with intellec-
intellectual disabilities, and administration times vary be-
tual disability, emotional maladjustment, autism, or de-
tween 15 and 40 min, depending on the informants knowl-
velopmental disability. It enables a professional to assess
edge of the individual being assessed. Raw scores are
strengths and weaknesses of an individual in adaptive
recorded and then converted to standard scores and percen-
areas, document progress, and assess the effectiveness of
tiles. The subscales yield the same five-factor scales as the
intervention/school programs. The manual cautions that
ABS-S: 2.
the examiner should interview a significant informant or
the instrument should be administered by that significant
informant. If an informant is unable to provide needed
Historical Background
information, then another informant needs to be inter-
viewed. Whereas the ABS is a standard assessment used in
The AAMD first published the ABS in 1969 in response to
determining adaptive and maladaptive behavior, its psy-
the definition of mental retardation that was enlarged in
chometric properties are limited, especially compared to
1959 to include adaptive behavior. The ABS-S: 2, first
other measures such as the Vineland Adaptive Behavior
published in 1969 by Nihira, Foster, Shellhaas, and
Scales.
Leland, was revised and standardized in 1974 by Lambert,
Whereas a strength of the ABS-S: 2 is that it was
Windmiller, and Cole and again in 1981 by Lambert and
normed on students with and without intellectual disabil-
Windmiller. The second and current edition was pub-
ities, the ABS-RC: 2s standard scores and percentile ranks
lished in 1993. The ABS-RC:2 were also first published
were not compared to individuals without intellectual
in 1969 by Nihira, Foster, Shellhaas, and Leland. It was
disabilities. Therefore, this assessment may not meet the
revised in 1974, and again in 1993. The goals of the
criteria to make a diagnosis of mental retardation accord-
revisions have been to improve the reliability of the inter-
ing to the AAMR requirements.
viewer in differentiating between individuals with intel-
lectual disabilities who are institutionalized and those
living in the community. Previously, these individuals
had been classified at different adaptive behavior levels
Cross References
according to the AAIDD.
Vineland Adaptive Behavior Scales
Psychometric Data
References and Readings
The authors of the ABS-S: 2 report three types of reli-
ability: internal consistency, stability, and interscorer. Aiken, L. (1996). Assessment of intellectual functioning. Switzerland:
Internal consistency is reported to range from 0.79 to Burkhauser.
Bracken, B., & Nagle, R. (2007). Psychoeducational assessment of preschool
0.98, while measures of stability range from 0.82 to 0.97.
children. New York: Routledge.
For Part I, interscorer reliability ranges from 0.95 to 0.98 Hogg, J., & Langa, A. (2005). Assessing Adults with Intellectual Disabil-
whereas it is 0.96 to 0.99 for Part II. Authors report ities. Malden, MA: Blackwell.
criterion validity in Part 1 moderately correlated with Lyman, W. (2008). Test review In N. Lambert, K. Nihira, & H. Lel (1993).
the ABS and the Vineland Adaptive Behavior Scales, AAMR Adaptive behavior scales: school. Assessment for Affective
Intervention, 33, 5557.
although Part II was not significantly related to either
Reynolds, C., & Fletcher-Janzen, E. (2007). Encyclopedia of special educa-
(Lyman, 2007). tion, a reference for the education of children, adolescents, and adults
The ABS-RC: 2 reports an internal consistency rang- with disabilities and other exceptional individuals (3rd ed., Vol. 1).
ing from 0.81 to 0.97. Concerning discriminant validity, Hoboken, NJ: Wiley.
Abbreviated Injury Scale A 5
Definition
Definition
Current Knowledge
This refers to an inability to walk. Abasia may be caused
by a variety of conditions including weakness, spasticity, The latest incarnation of the AIS score is the 2005
cerebellar incoordination, and movement disorders of revision. AIS is monitored by a scaling committee of
various types. the Association for the Advancement of Automotive
Cross References
Abbreviated Injury Scale. Table 1 AIS scores and their
Ataxia definition of injury severity
Spastic Gait AIS Score Injury
1 Minor
2 Moderate
3 Serious
ABAS-II 4 Severe
5 Critical
Adaptive Behavior Assessment System Second
6 Unsurvivable
Edition
6 A Ability Focused
Medicine and has been adopted by the American Associ- ablation is too destructive to neighboring tissues. Even
ation for the Surgery of Trauma since its publication in with sophisticated neurosurgical techniques, ablation of
the Journal of Trauma in 1985. any type in the nervous system may still produce unwanted
motor, sensory, or cognitive-behavioral impairments.
Synonyms
Abnormal Brain Growth
Resection
Microcephaly
Definition
are more common in JAE and occur in about 8090% of overall intelligence at least one standard deviation below
cases. Some patients with JAE also exhibit myoclonic the mean. Similar rates were found for VIQ and PIQ.
seizures, but they are typically mild and infrequent. While Their spoken language quotient (SLQ), as measured by
most patients with CAE become seizure-free in adoles- various versions of the Test of Language Development,
cence, seizure outcome in JAE is not well known. was average, but it was also lower than controls. A high
CAE is considered to be a benign childhood epilepsy percentage of children with CAE performed at least one
because of relatively good seizure control and functional standard deviation below the mean on language measures.
outcomes. Seizure control is less common in JAE, but In addition to finding a higher rate of cognitive
functional outcomes may be similar. Further research is limitations, Caplan et al. (2008) confirmed that children
needed to examine this. Tonicclonic seizures are believed with CAE also experience emotional and behavioral
to be a marker for poorer seizure outcome in both CAE comorbidities. Among the 69 children with CAE in their
and JAE. Functional outcomes in CAE are thought to be sample, 30% had a diagnosis of attention-deficit/hyperac-
most heavily influenced by psychosocial factors, such tivity disorder (ADHD), with 52% of those children diag-
as family adjustment, support systems, educational atti- nosed as ADHD-inattentive type. Moreover, about 29% of
tudes, and stigma toward the condition. Cognitive and/or their samples were diagnosed with a form of internalizing
behavioral side effects from antiepileptic drug (AED) psychopathology. Among those children, 75% were diag-
therapy may also limit outcomes. nosed with anxiety, 20% with depression, and 5% with
both anxiety and depression. After controlling for IQ and
demographic variables, children with CAE were found to
Neuropsychology and Psychology of have significantly higher ratings on scales of the Child
Absence Epilepsy Behavior Checklist (CBCL) that assess attention pro-
blems, somatic problems, social problems, withdrawal,
Cognitive functioning in CAE is traditionally considered and thought problems. The authors discovered that
benign, because children typically present with normal children with lower intelligence had greater social
intelligence and exhibit no significant impairments in problems, and females in the CAE sample were almost
functional outcomes. However, more recent research has six times more likely to be diagnosed with an anxiety
found evidence that patients with CAE are prone to disorder. In addition, children with CAE were more likely
having cognitive deficits and psychosocial problems, and to be diagnosed with ADHD or anxiety if they had more
they are more likely to receive special education services frequent seizures or a longer duration of illness.
and display low academic achievement. While patients
with poor seizure control exhibit the greatest difficulties,
cognitive and behavioral problems are also experienced by Evaluation
patients with good seizure control. Unfortunately, limited
information is known about cognitive and psychological Children and adolescents with CAE and JAE typically
functioning in JAE. present with no focal neurological abnormalities on
Patients with CAE do not have a characteristic examination. The presence of absence seizures is a defin-
cognitive profile. Cognitive difficulties have been reported ing feature of absence epilepsy, and hyperventilation or
in multiple domains, including attention, memory, and light stimulation can be highly effective at eliciting an
visual-spatial processing. A recent study by Caplan et al. event. In CAE, absence seizures occur multiple times per
(2008) revealed the presence of subtle cognitive impair- day, but, in JAE, they are more rare and may only occur
ments in children with CAE. When compared with con- once per day.
trols, they found that children with CAE (ages 6.711.2 Absence seizures can be either typical or atypical,
years) had significantly lower intelligence, as measured by and discrimination between the two types is usually
the Wechsler Intelligence Scale for Children Revised/Third done off of EEG findings. While typical absence seizures
Edition. While, as a group, children with CAE performed are characterized by clearly delineated episodes of activity
in the average range, they were below the performance of a arrest and impaired consciousness for less than 10 s,
control group. Similar differences were noted on verbal atypical absence seizures are associated with less abrupt
and visual intellectual tasks. The difference in perfor- onset and termination, and they may more commonly
mance IQ (PIQ) was less robust, but still significant, involve various semiological phenomena. Atypical
between children with CAE and controls. Among their absence seizures often last for more than 10 s and cannot
sample of 69 children with CAE, 27% demonstrated be elicited by hyperventilation or light stimulation. Tonic
Absence Seizure A 9
seizures are also frequently present in children with Ethosuximide has also been recommended and may be
A
atypical absence seizures. more appropriate for younger patients. In rare cases of
Typical absence seizures can be subdivided into simple more difficulty in controlling seizures, polytherapy may
and complex. Simple typical absence seizures constitute be needed. In patients with CAE, a seizure-free period of
about 90% of cases and may involve only minor motor 2 years is often recommended prior to discontinuation of
mannerisms (e.g., mild eyelid fluttering). Patients with therapy; however, this should be determined on a case-by-
complex typical absence seizures display more involve- case basis. Patients with JAE will require longer treatment
ment of motor features, such as automatisms or decreased and may continue on AEDs indefinitely. In adolescent
or increased muscle tone. Loss of consciousness may also patients, it is important to educate about the increased
be longer. risk of seizures with poor medication compliance, alcohol
Complex partial seizures can often mimic absence sei- consumption, or sleep deprivation.
zures, particularly when their expression is limited. Typical
absence seizures can be distinguished from complex partial
seizures because they are briefer, more frequent, and have no Cross References
post-ictal impairment. EEG characteristics and the presence
of various seizure types often distinguish atypical absence Petit Mal Seizure
seizures from complex partial epilepsy. Juvenile Myoclonic Epilepsy (JME)
When considering the presence of absence seizures, it
is important to consider whether the episodes can be
References and Readings
accounted for by variations in attention. This is especially
important when considering the high rate of attention
Aicardi, J. (1998). Diseases of the nervous system in childhood. London:
problems in children with epilepsy. Attempting to Mac Keith.
determine the degree of responsiveness during the epi- Berkovic, S. F., & Benbadis, S. (2001). Childhood and juvenile absence
sodes often helps with making the differential diagnosis; epilepsy. In E. Wyllie (Ed.), The treatment of epilepsy: Principles and
however, this can be difficult to determine when episodes practice (3rd ed. pp. 485490). Philadelphia, PA: Lippincott Williams
& Wilkins.
are very brief. Moreover, it is not uncommon for patients
Caplan, R., Siddarth, P., Stahl, L., Lanphier, E., Vona, P., Gurbani, S.,
to have both absence seizures and attention problems. Koh, S., Sankar, R., & Shields, W. D. (2008). Childhood absence
Therefore, a childs ability to respond during an episode epilepsy: Behavioral, cognitive, and linguistic comorbidities.
cannot be used to rule-out the presence of absence seizures. Epilepsia, 49(11), 18381846.
Sometimes a neuropsychological assessment can be helpful
in differentiating between absence seizures and episodes of
inattention. If the examiner has experience with absence
seizures, the neuropsychological assessment can provide Absence Seizure
multiple hours of one-on-one observation and interaction
that might provide opportunities to observe the episodes K ENNETH P ERRINE
and attempt to elicit responses. This can also be helpful if Northeast Regional Epilepsy Group
mental fatigue tends to elicit more events. Hackensack, NJ, USA
On EEG, absence seizures are characterized by parox- Weill-Cornell College of Medicine
ysmal bursts of high amplitude 34 Hz spike and slow New York, NY, USA
waves that are superimposed on a normal background.
The bursts vary in length (310 s), and the clinical absence
is time-locked to the burst period. This activity (clinical Synonyms
and electrographic) can be provoked during a routine
EEG recording using the hyperventilation activation Petit mal seizure; Psychomotor seizures
procedure.
Definition
Treatment
An absence (usually pronounced with a French accent as
Response to AED therapy in CAE and JAE is good, and ab-SAWNS) seizure is a type of generalized seizure
valproic acid is often considered the drug of first choice. caused by a large burst of electrical discharges that
10 A Abstract Reasoning
Ma, L., Phelps, E., Lerner, J. V., & Lerner, R. M. (2009). Academic
Academic Competency competence for adolescents who bully and who are bullied. The
Journal of Early Adolescence, 29(6), 862897.
Shapiro, E. S. (2008). From research to practice: promoting academic
T ODD VAN W IEREN
competence for underserved students. School Psychology Review,
Indiana University of Pennsylvania 37(1), 4651.
Indiana, PA, USA
Christian, K., Morrison, F. J., & Bryant, F. B. (1998). Predicting kinder- Alexic acalculia is the inability to read number and
garten academic skills: Interactions among child care, maternal A
correlations with the inability to read text. People with
education, and family literacy environments. Early Childhood
Research Quarterly, 13(3), 501521.
this type of acalculia may focus only on beginning digits
Shapiro, E. S. (2004). Academic skills problems: Direct assessment and (538 is read as 53). For those with alexic acalculia, mental
intervention (3rd ed.). New York: Guilford Press. calculation abilities exceed written calculation abilities
(Ardila & Rosselli, 2002).
Agraphic acalculia is the inability to write numbers.
Like aphasic acalculia, agraphic acalculia correlates with
Acalculia Brocas and Wernickes aphasia. In Brocas aphasia, acal-
culia deficits manifest as omissions, substitutions, and
N ATALIE WAHMHOFF, E LAINE C LARK order reversal. In Wernickes aphasia, difficulties are espe-
University of Utah cially evident when required to write quantities when they
Salt Lake City, UT, USA are orally dictated. Those with Wernickes aphasia also
tend to make paralexias and paragraphias (Ardila & Ros-
selli, 2002; Growth-Marnat, 2000).
Synonyms Frontal acalculia deficits occur in conjunction with
attention difficulties, perseveration, and impairment of
Acquired dyscalculia; Dyscalculia; Mathematics disability more complex math concepts (Dehaene, Cohen, & Chan-
geux, 1998). Difficulties are most apparent with multistep
operations, algorithms, and when planning is required.
Definition While complex concepts are difficult for patients with
frontal acalculia, more basic math concepts are usually
Acalculia, most simply, is the inability to perform mathe-
maintained (Ardila & Rosselli, 2002).
matical tasks. These difficulties can stem from other def-
Spatial acalculia impacts written mathematical tasks
icits or can exist independently. Acalculia deficits can be
more than mental math tasks. A difficulty with writing
global or selective and manifest in a wide variety of num-
numbers is quite apparent in these cases and manifest in
ber processing and calculation abilities.
several ways. Writing on only one side of the page, inability
to write numbers in a straight line, and general disorganiza-
Categorization tion are some of the deficits that impact math performance
(Basso, Burgio, & Caporali, 2000). Patients with spatial
Generally, authors have agreed on two major distinctions: acalculia often forget where to place remainders and car-
primary and secondary acalculia (Growth-Marnat, 2000). ried numbers, despite understanding the basic division
Primary acalculia occurs when mathematical deficits are and multiplication functions. Math procedure signs are
fundamental and are present independently of other defi- often undetected or switched (add instead of subtract).
cits. Deficits in primary acalculia include poor estimation,
number comparison abilities, and difficulty understanding
procedural rules and numerical signs. In primary acalculia, Epidemiology
these deficits will exist regardless of whether tasks are pre-
sented in an oral or written format (Adila & Rosselli, 2002). Acalculia can result from stroke, tumors, and trauma. It is
The secondary acalculias are due to primary deficits in also seen in patients with degenerative dementia (Ardila &
other areas. Aphasic acalculia occurs in patients with Wer- Rosselli, 2002).
nickes and Brocas aphasia. Patients with Brocas aphasia
have problems when translating word representations of
numbers (three hundred and forty-five) to their numeral Prognostic Factors and Outcomes
form (345). They may also read numbers with morpho-
logical errors (15 is read as 50) (Ardila & Rosselli, 2002; There is noted variability in prognosis for acalculia, ranging
Basso, Burgio, & Caporali, 2000). When the secondary from no recovery to full recovery. For primary acalculia,
acalculia stems from Wernickes aphasia, deficits are more improvement is limited. In the case of secondary acalculias,
severe. Reading and writing of numbers often have semantic recovery from the primary deficit, such as aphasia, alexia,
errors, and poor verbal memory often impacts the calcula- and agraphia, occur, the corresponding acalculia deficits
tion abilities of these patients (Grafman & Rickart, 2000). tend to improve as well.
14 A ACC
Neuropsychology and Psychology assessment. This will provide explicit areas to target dur-
of Acalculia ing rehabilitation (Grafman & Rickart, 2000). Long-term
rehabilitation programs should begin simply and progres-
Primary acalculia is associated with left posterior parietal sively work toward more complex tasks. With secondary
lesions. More specifically, damage to the left angular acalculia, focusing rehabilitation on the primary deficit
and supramarginal gyri occurs with primary acalculia may significantly improve the secondary acalculia deficits
(Grafman & Rickart, 2000). It is suggested that there are (Ardila & Rosselli, 2002).
separate neuropathways for rote number knowledge and
semantic number knowledge.
Neuroimaging techniques reveal that several brain Cross References
areas are active when performing calculations and also
that the pattern differs according to what type of calcula- Agraphia
tion is done (Dehaene, Cohen, & Changeux, 1998). This Alexia
occurs to the many abilities that calculation often Aphasia
requires, including verbal, spatial, executive functioning, Gerstmanns Syndrome
and memory. The areas most associated with calculation Spatial Dyscalculia
are the upper cortical surface and anterior aspect of the
left middle frontal gyrus, the bilateral supramarginal and
angular gyrus, the left dorsolateral prefrontal and premo- References and Readings
tor cortices, Brocas area, inferior parietal and left parietal
cortex, and the inferior occipitotempral regions (Ardila & Ardila, A., & Rosselli, M. (2002). Acalculia and dyscalculia. Neuropsychol-
Rosselli, 2002). ogy Review, 12, 179231.
It is important to keep in mind that damage to the Ardila, A., Matute, E., & Inozemtseva, O. (2003). Progressive agraphia,
right hemisphere and the frontal lobes also impact the acalculia, and anomia: a single-case report. Applied Neuropsychology,
10, 205214.
occurrence of acalculia, especially when it is a secondary
Basso, A., Burgio, F., & Caporali, A. (2000). Acalculia, aphasia, and spatial
acalculia. disorders in left and right brain-damaged patient. Cortex, 36,
265280.
Dehaene, S., Cohen, L., & Changeux, J. P. (1998). Neuronal network
Evaluation models of acalculia and prefrontal deficits. In R. W. Parks, D. S.
Levine, & D. L. Long (Eds.), Fundamentals of neural network model-
ing: neuropsychology and cognitive neuroscience (pp. 233255).
The arithmetic section of the Wide Range Achievement Cambridge, MA, USA: MIT.
Test (WRAT) has often been used to test operational skills. Grafman, J., & Rickart, T. (2000). Acalculia. In M. J. Farah & T. E.,
The Key Math, which is designed for children and adoles- Fienberg (Eds.), Patient based approaches to cognitive neurosciences:
cents, tests more targeted and specific abilities that are issues in clinical and cognitive neuropsychology. Cambridge,
Massachusetts: MIT.
suggested for an acalculia assessment (Grafman & Rickart,
Growth-Marnat, G. (Ed.). (2000). Neuropsychological assessment in clini-
2000). Many authors have suggested experimental bat- cal practice. New York: Wiley.
teries that target specific functions and include error Scruggs, T. E. & Mastropieri, M. A. (2000). Acalculia. In Encyclopedia of
analysis. These batteries often assess skills in the following special education (2nd ed., Vol. 1, p. 27). New York: Wiley.
areas: number recognition, number writing, number
transcoding, quantification, magnitude estimation, basic
arithmetic operations, calculation fact verification, multi-
column calculations, magnitude comparison, fractions,
algebra, and numeric knowledge. When possible, these
ACC
skills should be assessed in both written and oral form
Anterior Cingulate Cortex
(Ardila & Rosselli, 2002; Grafman & Rickart, 2000).
Treatment
Accelerated Hypertension
Some authors have suggested beginning rehabilitation
with an error analysis if it was not completed during the Hypertensive Encephalopathy
Accessory Cuneate Nucleus A 15
Definition Definition
Nucleus in the dorsolateral portion of the medulla In order to provide students with disabilities the free,
that receives sensory information likely from touch, pres- appropriate public education mandated by IDEA 2004
sure, and stretch receptors in the upper extremities. It and Section 504 of the Rehabilitation Act of 1973, changes
gives rise to the cuneocerebellar tract which enters the typically must be made to a childs educational curric-
cerebellum via the inferior cerebellar peduncle. The acces- ulum or environment. These accommodations include
sory cuneate nucleus is thought to be the equivalent of the changes in the method of presentation of material,
dorsal nucleus of Clarke in the lumbar, thoracic, and classroom seating location, availability of an interpreter
lower cervical cord which is the source of the dorsal for those with hearing impairment, response format,
spinocerebellar tract. These nuclei and tracts provide un- testing time allowed, setting, or other reasonable steps
conscious (as opposed to conscious) sensory feedback that do not significantly alter the content of educational
to the cerebellum in its regulation of individual muscles. material or the validity of tests. To be eligible to receive
Lesions of this nucleus might be expected to produce accommodations, students must be identified as having
cerebellar type symptoms of the ipsilateral upper extrem- a disability consistent with the guidelines presented in
ity (i.e., ataxia/incoordination of movement), but it is IDEA 2004 or Section 504 of the Rehabilitation Act of
relatively small and isolated lesions are likely to be 1973.
extremely rare. Accommodations may also be required in the work-
place under the Americans with Disabilities Act. These
could include installation of a ramp to permit wheelchair
access, flexible working hours, or provision of TTY
machines.
Accident Claims
Personal Injury Cross References
Accommodations
J ACOB T. LUTZ 1, DAVID E. M C I NTOSH 2
1
Bell State University
Accumbens Nucleus
Muncie, IN, USA
2 Nucleus Accumbens
Bell State University
Muncie, IN, USA
Current Knowledge
Definition
Applications
Acetylcholine has been identified as a neurotransmitter
substance since the mid-1920s. It is the neurotransmitter Dysfunction in the cholinergic system has been impli-
substance present at the neuromuscular junction and also cated in a number of clinical conditions including Alzhei-
innervates structures of the parasympathetic and sympa- mers disease (AD), diffuse Lewy body dementia (Londos,
thetic nervous systems (Feldman, Meyer, & Quenzer, 1997; Brun, Gustafson, & Passant, 2003), Huntingtons disease,
Iversen, Iversen, Bloom, & Roth, 2009). In the brain, and myasthenia gravis (Iversen et al., 2009). Recent work
cholinergic neurons have a wide distribution. Projections also suggests a reduction in cholinergic activity in Parkin-
emanate from the basal forebrain in the medial septal sons disease that may appear relatively early in the course
nucleus and terminate in the hippocampus and limbic of the condition (Shimada et al., 2009). Acetylchoinester-
cortex. Among other areas receiving cholinergic input are ase inhibitors are used in the palliative treatment of AD
the neocortex, olfactory bulbs, amygdala, neostriatum and myasthenia gravis. Cholinergic or anticholinergic
(caudate nucleus and putamen), the hypothalamus, and compounds are also used as a muscle relaxant for surgery,
various regions in the brain stem (Feldman et al., 1997). treatment of parkinsonism, glaucoma, urinary retention,
Acetylcholine is synthesized from the precursors Acetyl and in nonclinical applications such as insecticides in
CoA and choline in a chemical reaction involving the cata- agriculture and neurotoxins (and their antidotes) in war-
lytic enzyme, choline acetyltransferase (ChAT). The pres- fare (Feldman et al., 1997; Iversen et al., 2009). Much
ence of this enzyme has been used as a marker to locate research is being conducted to develop agents with greater
cholinergic neurons. Acetylcholine degradation (the prima- receptor subtype specificity to better address clinical
ry mode of removal from synapses) is accomplished by the conditions.
activity of a group of enzymes known as cholinesterases.
Acetylcholinesterase is the primary enzyme that breaks
down acetylcholine in the synapse. Thus, to enhance cho- Cross References
linergic function, a number of substances have been devel-
oped that inhibit the activity of this enzyme (Iversen Alzheimers Disease
et al., 2009). Anticholinesterase Inhibitors
Based on differences in the agonists that stimulate Cholinesterase Inhibitors
cholinergic receptors, two receptor subtypes have been
identified, nicotinic and muscarinic. Nicotinic receptors
are stimulated by nicotine, are excitatory, and show a References and Readings
rapid response to stimulation. Muscarinic receptors are
stimulated by muscarine, have either excitatory or inhibi- Feldman, R. S., Meyer, J. S., & Quenzer, L. F. (1997). Acetylcholine. In
tory effects, and show a slower response to stimulation. Principles of neuropsychoparhmacology (pp. 246249). Sunderland,
Further subtypes exist within the nicotinic and muscarin- MA: Sinauer Associates.
ic classes (Feldman et al., 1997; Iversen et al., 2009). Iversen, L. L., Iversen, S. D., Bloom, F. E., & Roth, R. H. (2009). Acetyl-
Acetylcholine is involved in a number of behavioral choline. In Introduction to neuropsychopharmacology (pp. 128149).
New York: Oxford University Press.
processes. As a neurotransmitter substance at the neuro- Londos, E., Brun, A., Gustafson, L., & Passant, U. (2003). Lewy body
muscular junction, it acts on motor neurons of the spinal dementia. Clinical challenges in diagnosis and management. In
cord and cranial motor nerve nuclei, playing an K. Iqbal & B. Winblad (Eds.), Alzheimers disease and related
18 A Acetylcholinergic System
disorders: Research advances (pp. 133142). Bucharest, Romania: (Bartels & Zeki, 2000), patients lose the ability to perceive
Ana Asian International Academy of Aging.
color, and therefore experience the world as varying
Shimada, H., Hirano, S., Shinotoh, H., Aotsuka, A., Sato, K., Tanaka, N.,
et al. (2009). Mapping of brain acetylcholinesterase alterations in
shades of gray. This disorder is termed cerebral achroma-
Lewy body disease by PET. Neurology, 73, 273278. topsia. The loss of color vision in these patients cannot be
explained by the photoreceptors typically damaged or
absent in patients with other types of color blindness.
Acetylcholinergic System
Categorization
Cholinergic System
Cerebral achromatopsia results from bilateral damage to
the V4/V4a region of the color center. If patients experi-
ence complete ablation of V4, they lose color vision in
Acetylcholinesterase Inhibitors their entire visual field. However, if patients experience
unilateral damage to V4, hemi-achromatopsia ensues,
Anticholinesterase Inhibitors where patients only lose color vision in the contralateral
Cholinesterase Inhibitors half of their visual field. In less extreme cases, known as
dyschromatopsia, patients lose the ability to perceive se-
lective colors and/or color constancy. These neuropsycho-
logical disorders, which are the result of damage to the
ACHE Inhibitors cerebral cortex, should not be confused with congenital
achromatopsia, which occurs as a malfunction of the cone
Anticholinesterase Inhibitors photoreceptors.
Epidemiology
AchEIs
Cerebral achromatopsia arises following brain damage to
Anticholinesterase Inhibitors V4/V4a located in the ventral medial region of the occipi-
Cholinesterase Inhibitors tal lobe, typically caused by a tumor, a hemorrhage, or
some sort of brain trauma. Due to the low incidence rate
of cerebral achromatopsia, it is difficult to provide a
reliable estimate of its prevalence. However, it seems safe
Achromatopsia to say that it is extremely rare. A review of the documen-
ted cases showed that of the 27 cases reported, 3 patients
S OPHIE L EBRECHT, M ICHAEL J. TARR recovered, 3 partially recovered, and 21 showed no recov-
Brown University ery (Bartels & Zeki, 2000).
Providence, RI, USA
Neuropsychology and Psychology of occur for up to a year. With regard to the treatment and
A
Achromatopsia diagnosis of cerebral achromatopsia, experimenters re-
port that some patients are not conscious of the absence
The region of damage in the visual field of achromotopsic of color vision. This phenomenon has been explained
patients, V4/V4a, is organized retinotopically; therefore, by the ablation of a color module leaving patients with-
damage to a particular region of V4 results in a loss of out even the concept of color post-lesion. This symp-
color vision at the corresponding location in the visual tom of achromatopsia should be noted when addressing
field. For example, if damage to V4 occurs in the left patients, because pushing a patient to describe a condi-
hemisphere, the patient will lose color vision in the right tion they are not aware of could be distressing for the
half of their visual field. Because V4 is located in the patient.
vicinity of the fusiform gyrus and the lingual gyrus,
known to process faces (Kanwisher et al., 1997), the co-
morbity between achromatopsia and prospoagnosia is
extremely high (Bouvier & Engel, 2006). In addition,
Cross References
patients with achromatopsia also have a higher incidence
Scotoma
of spatial and shape deficits. It has been noted that
patients with complete achromatopsia cannot even imag-
ine color, which means they cannot dream in color or use
color during mental imagery. This absence of color vision References and Readings
often leaves patients with no appetite for foods, which
appear gray, and no desire for intimacy, as flesh appears Bartels, A., & Zeki, S. (2000). The architecture of the color centre in the
gray. An insightful case study of a color-blind painter human visual brain: New results and a review. The European Journal
describes these experiences in detail (Sacks, 1995). of Neuroscience, 12(1), 172193.
Bouvier, S. E., & Engel, S. A. (2006). Behavioral deficits and cortical
damage loci in cerebral achromatopsia. Cerebral Cortex (New York,
N.Y.: 1991), 16(2), 183191.
Evaluation Kanwisher, N., McDermott, J., & Chun, M. M. (1997). The fusiform
face area: A module in human extrastriate cortex specialized
Cerebral achromatopsia can be diagnosed using a range of for face perception. Journal of Neuroscience, 17(11), 43024311.
color vision tests. The simplest test is an explicit color- Meadows, J. C. (1974). Disturbed perception of colors associated with
localized cerebral lesions. Brain: A Journal of Neurology, 97(4),
naming task that requires patients to name the color of 615632.
individual flash cards. The most common test for color Sacks, O. W. (1995). An anthropologist on mars: Seven paradoxical tales.
blindness is the Ishihara plates test. These plates contain New York: Vintage Books.
isoluminant colored dots of varying sizes that together Verrey, D. (1888). Hemiachromatopsie Droite Absolue. Conversation
create the perception of a number embedded in noise. Partielle De La Perception Lumineuse Et Des Formes. Ancien Kyste
Hemorrhagique De La Partie Inferieure Du Lobe Occipital Gauche.
In order to perceive the number, patients must be able to Archives dophtalmologie, 8, 289300.
distinguish between the different colored dots. Another Werner, J. S., & Chalupa, L. M. (2004). The visual neurosciences.
widely-used test is the Farnsworth-Maunsell 100 Hue test, Cambridge, MA: MIT Press.
in which patients are required to order colored caps based
on gradual shifts in hue from light to dark. Patients with
color blindness are unable to perform this task. Rarely, a
diagnosis is made using a Nagel Anomaloscope. This
apparatus is typically used to determine whether a patient ACoA
is a monochromat or a diachromat; however, some
experimenters/practitioners use it in the study of cerebral Anterior Communicating Artery
achromatopsia.
Treatment
Acoustic Aphasia
There is a period of spontaneous recovery for neurovisual
lesions, which typically lasts 3 months post-lesion, but can Pure Word Deafness
20 A Acoustic Neuroma
Acoustic Neuroma
E THAN M OITRA
Drexel University
Morgantown, WV, USA
Synonyms
Definition
Cross References
Radiosurgery
Radiotherapy
Acquired Achromatopsial
Achromatopsia
replicate, which is done in the nucleus of infected cells. 3. Transfusion of infected blood or other bodily incor-
HIV is a member of the lentivirus group of retroviruses poration of infected blood
which also includes simian immunodeficiency virus. Len- 4. A fetus or infant exposed to HIV before or during
tiviruses typically have longer incubation periods and birth or through breast feeding
greater genetic complexity than other retroviruses.
The natural progression of HIV infection can be divided
In 1985, a second strain of the virus was discovered,
into three stages: primary infection, clinical latency, and
which was designated as HIV-2. The original strain of the
symptomatic disease stage. The symptomatic disease
virus was designated as HIV-1. HIV-1 is much more
stage is further divided into early and late stages, with
common throughout the world, while HIV-2 is more
AIDS being equated with the late-symptomatic disease
common in certain parts of Africa alone. Also, HIV-2
stage. After a person is initially infected with HIV, a
appears to be milder than HIV-1, with a slower progres-
primary or acute infection stage commences, in which
sion to AIDS. Since its establishment in humans, HIV-1
HIV replicates up to ten billion copies of itself daily;
has undergone mutation of its genome and there are now
high levels of HIV in the blood or viraemia is evident.
three groups of HIV-1.
Approximately, 24 weeks after exposure, nearly 70% of
How HIV is transmitted tends to vary worldwide
those newly infected will experience an acute illness,
depending upon the geographic region. In the United
which has symptoms similar to influenza or mononucle-
States, approximately 45% of current cases of HIV infec-
osis, including fever, fatigue, muscle weakness, headache,
tion were obtained through malemale sexual contact
ocular pain, sensitivity to light, sore throat, diarrhea, and
(men who have sex with men or MSM), 22% were
lymphadenopathy. This illness is due to the temporary
through injecting drug users (IDU), and 5% were through
reduction of CD4+ T cells; it lasts for approximately
individuals who were both MSM and IDU. Approximate-
2 weeks and then resolves spontaneously. It is during
ly, 27% of cases were through malefemale sexual contact.
this stage that the individual typically first begins to
Transmission rates have been changing though, with new
produce antibodies to HIV, which is designated as
cases of infection in older white MSM decreasing. Trans-
seroconversion.
mission rates have been increasing in AfricanAmerican
Serological testing of blood can reliably detect HIV
and Latino MSM and younger white MSM due to
antibodies 26 months after seroconversion. Testing typi-
increases in high-risk sexual practices; approximately,
cally begins with an enzyme-linked immunosorbent assay
50% of new cases are in AfricanAmerican MSM. Rates
(ELISA) or test that looks for antibodies to HIV. A second
of transmission are also increasing in women, primarily
positive ELISA is needed in order to confirm the result.
due to heterosexual contact with MSM and IDU. In
This would then be followed by the Western Blot Proce-
Africa, transmission is primarily due to malefemale sex-
dure to confirm the presence of at least two specific HIV
ual contact. In Eastern Europe, transmission is primarily
antigen groups. A diagnosis of HIV infection is given after
in IDU or malefemale sexual contact. In Southeast
a positive Western Blot test follows two positive ELISA
Asia, transmission is primarily through contact with
tests. If HIV is confirmed, additional tests for plasma viral
commercial sex workers.
RNA (viral load) and CD4+ T cell counts are then typi-
cally completed, in order to assess the state of the immune
system and disease prognosis. Higher viral load counts
Natural History, Prognostic Factors, are typically related to faster disease progression. Lower
Outcomes CD4+ T cell counts are typically related to greater clinical
vulnerabilities.
HIV is not transmitted through casual contact, such as
After the acute illness disappears, the individual enters
touching. It can be transmitted when the bodily fluids of
the clinical latency stage in which symptoms are typically
infected individuals primarily blood, semen, vaginal
absent, other than possibly chronic lymphadenopathy.
fluid, or breast milk comes into contact with the blood-
This stage lasts an average of 10 years. During the clinical
stream or mucosal tissues of uninfected individuals.
latency stage, HIV continues to replicate and attack
Transmission can occur through:
CD4+ T cells, which in turn continues to counter attack.
1. Unprotected sexual contact (anal, vaginal, or oral) As the immune system becomes more compromised,
with an individual infected with HIV individuals eventually enter the early symptomatic disease
2. Sharing needles or syringes with HIV-infected stage, when a variety of symptoms begin to manifest,
individuals including lymphadenopathy, lack of energy, diarrhea,
Acquired Immunodeficiency Syndrome (AIDS) A 23
unintentional weight loss, chronic low-grade fever and Acquired Immunodeficiency Syndrome (AIDS). Table 2 HIV
A
sweats, frequent rashes or fungal infections, headaches, dementia symptoms
or short-term memory loss.
Behavioral difficulties
Finally, individuals enter the late stage of the symp-
Depression
tomatic disease stage or AIDS when the person has at least
Apathy, anhedonia, social withdrawal
one opportunistic infection or when the individuals
CD4+ T cell count is below 200 per mm3 of blood. The Personality changes, including spontaneous sudden and
most common opportunistic infections are PCP, Kaposis strong emotions
sarcoma, HIV wasting syndrome, and HIVencephalopathy Cognitive difficulties
(also known as dementia due to HIV disease or AIDS Confusion
dementia complex). Short-term memory lapses
Loss of concentration
Psychological and Neuropsychological Motor difficulties
Correlates of HIV Infection Lack of muscular coordination
Tremors
As HIV infection progresses, various psychological and Muscle weakness
neuropsychological complications involving both the cen- Loss of balance
tral as well as peripheral nervous systems can become
evident. During primary infection, reports of headaches
and aseptic meningitis are common. During the clinical
latency stage, an acute inflammatory demyelinating neu-
syndrome is characterized by behavioral, cognitive, and
ropathy (similar to Guillain-Barre syndrome; character-
motor declines and difficulties (Table 2). Initial symptoms
ized by progressive muscle weakness) can occasionally
typically manifest as cognitive difficulties (loss of concen-
develop. During the early symptomatic disease stage, pe-
tration and mild deficits in memory) with motor and
ripheral neuropathy is common. This is characterized by
behavioral difficulties frequently occurring. (This early
spontaneous pain (dysesthesia), pain due to light touches
stage is often labeled as HIV-associated minor cognitive
or changes in temperature (hyperesthesia), and weakness
motor disorder.) Later symptoms include partial paraly-
and wasting in arms/legs (distal atrophy).
sis, incontinence, and severe cognitive impairment. Death
It is during the late symptomatic disease stage or AIDS
usually occurs within 16 months after onset of severe
that most major neuropsychological complications devel-
symptoms. Individuals who are coinfected with hepatitis
op, and can include:
C or were IDU, typically display worse symptoms faster.
1. HIV encephalopathy (HIV dementia) As HIV-infected individuals live longer, it is estimated
2. Opportunistic infections that 5075% of all patients with AIDS will evidence
(a) Viral (Cytomegalovirus; Herpes Simplex I and II; some form of HIV dementia.
Herpes Zoster; JC virus, a polyomavirus or While HIV can be present in any part of the brain,
papovavirus which causes PML [progressive HIV is particularly common in the basal ganglia and
multifocal leukoencephalopathy]) central white matter (and to a lesser extent in neocortical
(b) Fungal/Protozoan (Toxoplasmosis, Cryptococcus, gray matter, the brainstem, and the cerebellum) in indi-
Candida, Mycobacterium) viduals not receiving antiretroviral therapy or highly
3. Lymphomas active antiretroviral therapy (HAART) (see below). In
(a) Primary central nervous system lymphomas individuals on HAART, there is evidence of greater
(b) Systemic (metastatic) lymphomas. (The most inflammation in the hippocampus and surrounding en-
common systemic lymphomas are: Hodgkins; torhinal and temporal cortex.
immunoblastic; Burkitts; and non-Hodgkins,
which is particularly prevalent.)
HIV encephalopathy is the term used to describe the Treatment
pathological features of encephalitis of the brain due to
HIV, while HIV dementia (also known as AIDS dementia While there is no cure or vaccine for HIV or AIDS at
complex) is used to describe the clinical syndrome. This this time, there are currently four different classes of
24 A Acquisition of Knowledge
antiretroviral drugs that interfere with the ability of HIV to UNAIDS. (2007). UNAIDS Annual Report 2007: Knowing your epidemic.
Retrieved June 15, 2008 from http://data.unaids.org/pub/Report/
replicate: reverse transcriptase inhibitors (nucleoside and
2008/jc1535_annual_report07_en.pdf
non-nucleoside types); protease inhibitors; entry/fusion Weeks, B. S., & Alcamo, I. E. (2006). AIDS: The biological basis (4th ed.).
inhibitors; and integrase inhibitors. In 1987, the US Food Sudbury, MA: Jones and Bartlett Publishers.
and Drug Administration (FDA) approved Azidothymi-
dine (AZT, also known as Zidovudine), the first nucleo-
side-reverse transcriptase inhibitor (NRTIs). Saquinavir,
the first protease inhibitor was approved in 1995.
Nevirapine, the first non-nucleoside-reverse transcrip- Acquisition of Knowledge
tase inhibitor was approved in 1996. Enfuvirtide, the
first fusion inhibitor was approved in 2003. Maraviroc, Learning
the first entry inhibitor, and Reltegravir, the first inte-
grase inhibitor, were approved in 2007.
In 1996, combination drug therapy or HAART
began. Three or more drugs are used in combination in
order to counter the development of drug resistance by Action Tremor
HIV. Strict adherence to medication intake schedules is
required. Not only is this schedule difficult to follow A NNA D E P OLD H OHLER 1, M ARCUS P ONCE DE LEON2
1
for many individuals, HAART often produces unpleasant Boston University Medical Center
and toxic side effects, including stomach problems and Boston, MA, USA
2
lipodystrophy. If followed correctly, HAART typically and William Beaumont Army Medical Center
drastically reduces viral load, often to undetectable levels El Paso, TX, USA
in the blood, which allows the immune system to re-
bound. Antiretroviral drug therapy and treatments for
opportunistic infections have greatly increased life expec- Synonyms
tancy of those with HIV infection, but due to the presence
of HIV in cells that remain out of reach of antiretroviral Intention Tremors
drugs, eradication of HIV from the human body is unat-
tainable at this time.
Definition
The former is called spatial akinesia (e.g., a hand that does with various body parts including the limbs, eyes, neck,
not move in left hemispace, but does move in right body- eyelids (e.g., keep your eyes closed for until I tell you to
centered hemispace) and the latter is called directional open them), jaw, and tongue. Patients can even demon-
akinesia (e.g., a horizontal gaze palsy where patients can- strate impersistence in activities such as walking. Like
not move their eyes to the left); (3) Stimulusresponse akinesia, it may also be directional (e.g., inability to main-
conditions: Some patients, such as those with Parkinsons tain leftward gaze) or hemispatial (inability to maintain
disease, are impaired in spontaneously initiating a move- dorsiflexion of the wrist in left space with the left arm, but
ment, but in response to a stimulus often have no trouble able to do so in right space).
initiating a movement. We call this endogenously evoked
akinesia (endo-evoked). Patients who fail to move to an
imperative stimulus but will move spontaneously we call
Defective Response Inhibition
exogenously evoked akinesia. A patient may have both exo-
evoked and endo-evoked akinesia, which we term mixed
Not all stimuli require a response and sometimes a re-
or global akinesia.
sponse might interfere with goal-oriented behavior. De-
fective response inhibition is defined as responding when
no response of that body part is required. It can be seen in
Hypokinesia a variety of body parts and might also be directional and
perhaps hemispatial.
A milder defect in the intentional motor (when) sys- There are several forms of defective response inhibi-
tems might not induce a total inability to initiate a re- tion. One means of testing for this disorder is to use the
sponse (i.e., akinesia), but rather these patients crossed response task. A blindfolded patient is instructed to
intentional deficit might be manifested by a delay in raise the hand opposite to that touched. Patients with
initiating a response. We call this delay hypokinesia. The defective response inhibition will often raise the touched
hypokinesias may also be subtyped into body part (e.g., hand first. This type of defective response inhibition may
limb or eyes) and action space (e.g., directional and be termed motor (limb or eye directional) response disinhi-
hemispatial). bition. These can be either contralesional or bilateral. The
eye directional defective response inhibition has also been
called a visual grasp. There are some patients, however,
Motor Extinction who have a perceptual disorder and when stimulated on
one side (e.g., left hand) feel that they were stimulated
Patients with sensory extinction are able to detect single on the other (e.g., right hand). This phenomenon is called
stimuli on either side of their body, but when presented allesthesia and it should not be confused with defective
with two stimuli one on each side of their body they crossed response inhibition.
remain unaware of contralesional stimuli. Motor extinc- Patients with defective response inhibition may also
tion is a form of akinesia or hypokinesia where a patient fail on the types of gono-go tasks described by Luria. For
who is without sensory extinction is asked to respond by example, the patient may be instructed to put up two
moving the hand (or hands) that was (were) touched. The fingers when the examiner puts up one finger and to put
examiner then delivers stimuli to the right, left, and both up no fingers if the examiner puts up two fingers. If the
hands and patients with motor extinction are aware that patient mimics the examiner such that when the examiner
both hands have been touched, but either fail to lift the puts up one finger, the patient puts up one finger and
contralesional hand to simultaneous stimuli or lift it after when the examiner puts up two fingers, the patient puts
a delay. up two fingers, the patient has echopraxia.
The inability to sustain a motor act or a series of motor acts When a patient incorrectly repeats a prior response
that are required to complete a goal is called motor imper- or when a patient continues to perform the same act
sistence. Like akinesia, impersistence can be associated when the goal of the act has been completed, it is called
Activa A 27
motor perseveration. In one type of motor perseveration, deficits, injuries, or diseases that injure the basal ganglia,
A
when the task requirements have changed the patient is the substantia nigra (e.g., Parkinsons disease), portions of
unable to switch to a different motor program and incor- the thalamus, as well as the white matter connections can
rectly repeats the movements. Luria (1965) calls this iner- also induce intentional deficits.
tia of program action and Sandson and Albert (1987) call
this recurrent perseveration. In the second type, the patient
continues to perform movements even though the task is Future Directions
completed. Luria (1965) called this efferent perseveration;
however, Sandson and Albert (1987) call this continuous Disorders of intention have received considerably less
perseveration. neuroscientific study than have disorders of sensory selec-
tive attention. There is a need for additional experimental
and clinical neuropsychological studies of these disorders.
Furthermore, assessment batteries are needed that will
Pathophysiology of Intentional facilitate the assessment of the subtypes of motor inten-
Disorders tion disturbances and which may provide additional
quantitative data for experimental analysis and norma-
Intentional motor disorders are often associated with bilat- tive comparison between patient groups and health
eral hemispheric lesions, but when these disorders are individuals.
caused by a unilateral hemispheric lesion they are more
commonly associated with right than left-hemisphere
lesions. The intentional disorders that have been reported Cross References
to be induced by primarily right-hemisphere lesions in-
clude akinesia (e.g., left-sided limbs, leftward arm move- Attention
ments, and even left horizontal gaze), hypokinesia (slowed Directional Hypokinesis
reaction times), motor impersistence of the left-sided Impersistence
limbs, left-sided gaze), and motor (continuous) persevera- Neglect Syndrome
tion. Many of the intentional defects associated with right-
hemisphere dysfunction, however, are not just limited to
the left limbs. For example, patients with a right-hemisphere
lesion are more often abulic, have slowed reaction times of References and Readings
their right hand, and have motor impersistence of eye
closure. These clinical studies suggest that the right hemi- Heilman, K. M., Valenstein, E, Rothi, L. J. G., & Watson, R. T. (2004).
Intentional motor disorders and apraxia. In W. G. Bradley,
sphere may be dominant for intentional control of the
R. B. Daroff, G. M. Fenichel, & J. Jankovic (Eds.), Neurology
motor systems. Studies with normal subjects provide in clinical practice: Principles of diagnosis and management.
further evidence for right-hemisphere intentional domi- (pp. 117130). Phila Penn: Butterworth Heineman.
nance. The anatomic and physiological basis for this domi- Heilman, K. M., Watson, R. T., & Valenstein, E. (2003). Neglect and
nance is not entirely understood. related disorders. In K. M. Heilman, & E. Valenstein (Eds.), Clinical
neuropsychology, (4th ed., pp. 296346). New York: Oxford Univer-
Studies of patients with focal lesions and studies of
sity Press.
monkeys suggest that the frontal lobes may play a critical Heilman, K. M. (2004). Intentional neglect. Frontiers in Bioscience, 9,
role in mediating intentional activity. The most important 694705.
areas of the frontal lobes appear to be the medial and Luria, A. R. (1965). Two kinds of motor perseveration in massive injury
lateral frontal lobes. The frontal cortex has strong projec- to the frontal lobes. Brain, 88, 110.
Sandson, J., & Albert, M. L. (1987). Varieties of perseveration. Neuropsy-
tions to the striatum. The lateral portion of the frontal
chologia, 22, 715732.
lobe projects to the caudate. The premotor cortex projects
to the putamen and the cingulate gyrus projects to the
ventral striatum. The striatum projects to the pars reticu-
laris of the substantia nigra and the globus pallidus. The
globus pallidus projects to specific thalamic nuclei and Activa
these thalamic nuclei project back to the frontal cortex.
Just as injury of the frontal lobes can induce intentional Deep Brain Stimulator (Parkinsons)
28 A Active Limb Activation
measure IADLs that are important for independent living. include the Direct Assessment of Functional Status, the
A
Some of the first of these measures were Lawton and Independent Living Scales, the Structured Assessment of
Brodys IADL Scale and the Disability Interview Schedule. Independent Living Skills, the Medication Management
Abilities Assessment, and many others.
The use of questionnaires administered either on paper
Current Knowledge or by interview allows the sampling of a large number of
behaviors in a short period of time. Self-report question-
ADLs are of interest across various health disciplines. Cur- naires may be appropriate for use with cognitively-normal
rent knowledge in this area is based on research conducted or mildly impaired populations. In the evaluation of
by psychologists, occupational therapists, nurses, psychia- dementia and other cognitive disorders, however, self-
trists, neurologists, and social workers, among others. reported abilities may be difficult to interpret because
of disease-related decreases in self-awareness. The use of
informant-based questionnaires avoids this limitation,
Relevance to Neuropsychology although informants can also be biased in their reports
and may not always be available. Nevertheless, this is one
For the neuropsychologist, an understanding of the of the most common methods for measuring IADLs, and
patients level of independence in ADLs, and in particular a large number of informant-based questionnaires exist,
IADLs, is of interest for several reasons. The diagnosis of such as the Lawton-Brody IADL Scale, the Bristol ADL
a number of cognitive and mental disorders requires an Scale, and the ADL questionnaire.
appraisal of the patients functional ability (American The choice of which particular method of assessment
Psychiatric Association, 2000). For example, impairment to be used will depend, in addition to practical considera-
in adaptive or functional ability is a diagnostic criterion for tions such as time, on the purpose of the assessment.
mental retardation and for schizophrenia. Impaired daily Real-word observations and performance-based measures
functioning is also required for the diagnosis of dementia provide information about what the person is capable
and is one of the defining differences between dementia (in of doing. Questionnaires, on the other hand, measure
which IADLs are impaired) and mild cognitive impairment what the individual is actually doing in his or her day-
(in which IADLs are intact or minimally affected). to-day life.
Increasingly, the evaluation of daily functioning is also
used to identify appropriate treatments for cognitive and
mental disorders. In particular, an important part of Future Directions
determining the effectiveness of behavioral or pharmaco-
logical interventions is measuring the impact of the inter- Although there are a large number of relevant instruments
vention on the patients daily functional ability, in that have been developed to assess ADLs, they vary in terms
addition to cognitive or affective outcomes. of how well their psychometric properties have been
characterized. Systematic literature reviews (e.g., Moore,
Palmer, Patterson, & Jeste, 2007; Sikkes, de Lange-de
Assessment of ADLs Klerk, Pijnenburg, Scheltens, & Uitdehaag, 2009) indicate
that, for many of these measures, there is a need for better
Assessment of ADLs can be accomplished in a number of theoretical justification of the content of the instrument,
ways. Real-world observation of the patient in his or her additional information about test validity and reliability,
own home provides relevant, objective information about indication of what constitutes a meaningful change
daily function. However, this method is obviously time over time, information about the relation between test
and labor intensive, and there are practical limits to the performance and actual real-world functioning, and the
number of behaviors that can be observed within a given development of comprehensive normative data.
time period. An alternative is the use of performance-based
measures, which require the patient to complete function-
al tasks such as preparing a meal, using the telephone, or Cross References
making personal financial transactions that are pre-
sented in a standardized way in the laboratory or clinic. Adaptive Behavior
A number of such instruments have been developed to Basic Activities of Daily Living (B-ADL)
measure single or multiple functional domains. Tests Functional Status
30 A Activities of Daily Living Questionnaire
Instrumental Activities of Daily Living (I-ADL) self-care activities; household care; employment and
Lawton-Brody iADL Scale recreation; shopping and money; travel; and communica-
tion. The informant rates the subjects competence in each
area according to a set of four descriptions of different
competence levels; scores range from 0 to 3 where higher
References and Readings scores indicate greater impairment. A fifth response
option, dont know/has never done is also available, and
American Psychiatric Association. (2000). Diagnostic and statistical
if this option is selected, the item is excluded from scoring.
manual of mental disorders (4th ed., text revision). Washington, DC:
Author. Scores from individual items are summed (with adjustment
Law, M., Baum, C., & Dunn, W. (Eds.). (2001). Measuring occupational for any items marked dont know/has never done) to form
performance: Supporting best practice in occupational therapy. subscale scores and then transformed to a percentage
Thorofare, NJ: Slack. impairment total score. Scores of 033% are classified as
Lawton, M. P., & Brody, E. M. (1969). Assessment of older people: Self-
no/mild impairment, those of 3466% as moderate
maintaining and instrumental activities of daily living. Gerontologist,
9, 179186. impairment, and those of 67100% as severe impairment.
McDowell, I., & Newell, C. (1996). Measuring health: A guide to rating
scales and questionnaires (2nd ed.). New York: Oxford.
Moore, D. J., Palmer, B. W., Patterson, T. L., & Jeste, D. V. (2007). A Historical Background
review of performance-based measures of functional living skills.
Journal of Psychiatric Research, 41, 97118.
Sikkes, S. A. M., de Lange-de Klerk, E. S. M., Pijnenburg, Y. A. L., The first reported use of the ADLQ was in a longitudinal
Scheltens, P., & Uitdehaag, B. M. J. (2009). A systematic review of study looking at cognitive test performance and daily
Instrumental Activities of Daily Living scales in dementia: room for functioning in patients with Alzheimers disease
improvement. Journal of Neurology, Neurosurgery, and Psychiatry, 80, (Locascio, Growdon, & Corkin, 1995). However, the
712.
development and psychometric properties of the measure
Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of
neuropsychological tests (3rd ed.). New York: Oxford. were first reported in Johnson, Barion, Rademaker,
Rehkemper, and Weintraub (2004). Since then, a Chinese
version has been developed and evaluated (ADLQ-CV;
Chu & Chung, 2008), and it has been used in several
studies involving people with non-Alzheimers dementia.
Activities of Daily Living
Questionnaire Psychometric Data
J ESSICA F ISH
Johnson et al. (2004) collected ADLQ data from the
Medical Research Council Cognition & Brain
primary caregivers of 140 people with dementia of various
Sciences Unit
types (Alzheimers disease, vascular/mixed, and fronto-
Cambridge, UK
temporal/primary progressive aphasia). The scale was
completed twice, with a 1 year interval between comple-
tions. Evidence of convergent validity was in the form of
Synonyms
correlations with global severity ratings (clinical dementia
rating r = 0.5 and 0.55 for first/second ratings, respec-
ADLQ
tively; MMSE r = 0.42 and 0.38 for first and second
ratings, respectively). Further evidence of its validity came
from the finding that scores declined significantly over the
Description year-long interval between testings, as would be expected
in people with degenerative conditions. A subgroup of
The activities of daily living questionnaire (ADLQ) was 28 participants took part in a testretest reliability study,
developed to measure the functional abilities of people with a 28 week interval between testings (mean 25.6
with dementia. It is an informant-rated questionnaire and days, SD 12.2). Correlations between first and second
should be completed by the patients primary caregiver. It ratings for the six subscales were high, between 0.86 and
consists of 28 items covering both basic and instrumental 0.92, with the exception of the employment subscale,
activities of daily living, organized into six subscales: which correlated at 0.65. Kappa scores for 25% of scale
Activity Restrictions, Limitations A 31
References and Readings require voluntariness and instead the actus reus is viewed
in light of the severity of the offense.
Greenwood, R. J., Barnes, M. P., McMillan, T. M., & Ward, C. D. (Eds.).
(2003). Handbook of neurological rehabilitation (2nd ed.). New York:
Psychology Press.
Mills, V. M., Cassidy, J. W., & Katz, D. I. (Eds.). (1997). Neurologic Cross References
rehabilitation: A guide to diagnosis, prognosis, and treatment planning.
Malden, MA: Blackwell Science. Insanity
Williamson, G. M. & Shaffer, D. R. (2000). The activity restriction model
Insanity Defense
of depressed affect: Antecedents and consequences of restricted normal
activities. In G. M. Williamson, D. R. Shaffer, & P. A. Parmelee (Eds.), Mens Rea
Physical illness and depression in older adults: A handbook of theory,
research, and practice. New York: Kluwer Academic/Plenum
Publishers.
References and Readings
Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (1997). Psycho-
logical evaluations for the courts: A handbook for mental health profes-
Activity Therapy sionals and lawyers. New York: Guilford.
Recreational Therapy
M OIRA C. D UX
University of Maryland Medical Center/Baltimore VA
Baltimore, MD, USA
Acute Brain Syndrome
Definition Metabolic Encephalopathy
Current Knowledge A
Acute Encephalopathy
Symptoms
Delirium
Toxic-Metabolic Encephalopathy ALL is characterized by the rapid proliferation of imma-
ture blood cells (lymphoblasts), which crowd out mature,
functional cells. It is associated with the enlargement of
lymphoid tissue in areas including the lymph nodes,
spleen, bone marrow, and lungs, and with increased lym-
Acute Febrile Polyneuritis phocytic cells circulating in blood and in various tissues
and organs. Persons afflicted will experience weakness and
GuillainBarre Syndrome
fatigue, anemia, unexplained fever and infections, weight
loss, or loss of appetite.
Treatment
Acute Inflammatory
Demyelinating The earlier the ALL is detected, the more effective is its
treatment. The goal is to induce a lasting remission,
Polyradiculoneuropathy (AIDP) considered to be a prevalence of less than 5% of
lymphoblasts in bone marrow. Advances made in the
GuillainBarre Syndrome
ability to match the genetic properties of the blast cells
to treatment options, in association with the availability
of new drugs and improvements made in bone marrow
and stem cell transplantation, have changed the prog-
Acute Lymphoblastic Leukemia nosis for ALL from a zero to a 75% survival rate over
the past 40 years.
J ACQUELINE L. C UNNINGHAM Most (if not all) patients with a childhood history of
The Childrens Hospital of Philadelphia ALL have brain atrophy. Whereas atrophy is associated
Philadelphia, PA, USA with treatment-effects of cranial irradiation therapy
and intrathecal chemotherapy (usually methotrexate), it
can also occur as a result of the condition, itself, rather
Synonyms than as an outcome of treatment, as it appears to cause
atrophy of the brain, which is not specific to certain brain
ALL tissues (Lucy Rorke, MD, personal communication).
Nonetheless, the strongest detrimental impacts on cog-
nition are attributable to treatment-effects and their
Definition damaging influence on the biological substrates of core
neurocognitive abilities, including executive functions
Acute lymphoblastic leukemia (ALL) is a form of cancer and information processing. Such impacts disrupt the
of the white blood cells (leukocytes). ALL is the most secondary abilities, i.e., those that are acquired and
common type of childhood leukemia, and is distingui- knowledge-based. The main approaches to alleviating
shed from chronic lymphoblastic leukemia (CLL) and neurocognitive effects of treatment include cognitive
acute myeloid (or myelogenous) leukemia, which are remediation, pharmacology, and ecological alterations
more prevalent in adults. in the classroom.
34 A Acute Myelogenous Leukemia
Cross References out mature, functional cells. In AML, the cell type is
granuloid, whose cancerous change disrupts its normal
Acute Myelogenous Leukemia ability to form red cells, some types of white cells, and
Leukemia platelets. Resulting symptoms are anemia, easy bruising
Neoplasms and bleeding, and disruption to the bodys ability to
resist infection. Impaired cognition and fatigue are
also strongly associated with AML. Whereas impairments
in these areas have been attributed to effects of chemo-
References and Readings therapy, recent research by Meyers, Albitar, and Estey
(2005) has identified differing cytokine levels present
Butler, R. W., & Mulhern, R. K. (2005). Neurocognitive interventions
for children and adolescents surviving cancer. Journal of Pediatric
prior to chemotherapy as also contributing to these
Psychology, 30, 6578. symptoms.
Crosley, C. J., Rorke, L. B., Evans, A., & Nigro, M. (1978). Central nervous
system lesions in childhood leukemia. Neurology, 28, 678685.
Prassopoulos, P., Carouras, D., Golfinopoulos, S., Evlogias, N.,
Theodoropoulos, V., & Panagiotou, J. (1996). Quantitative assess- Pathophysiology
ment of cerebral atrophy during and after treatment in children
with acute lymphoblastic leukemia. Investigational Radiology, 12,
749754.
The malignant cell in AML is the myeloblast, a mutated
Pui, Ching-Hon (2003). Treatment of acute leukemias: New directions for and immature cell in the granulocytic series, which under-
clinical research. New York: Humana Press. goes combinations with other mutations, to produce a
leukemic clone of cells. Because the process contributes to
much diversity and heterogeneity in cell differentiation, the
diagnosis of AML can be challenging. It remains important,
however, since the chromosomal structure of the leukemic
Acute Myelogenous Leukemia cells is the diseases most critical prognostic factor.
J ACQUELINE L. C UNNINGHAM
The Childrens Hospital of Philadelphia
Philadelphia, PA, USA Treatment
Current Knowledge
Cross References
Symptoms
Acute Lymphoblastic Leukemia
Acute forms of leukemia are characterized by the rapid Leukemia
proliferation of immature blood cells which rapidly crowd Neoplasms
Acute Respiratory Distress Syndrome A 35
Synonyms
Acute Myeloid Leukemia Adult respiratory distress syndrome; Respiratory distress
syndrome
Acute Myelogenous Leukemia
Definition
The 1994 AmericanEuropean Consensus Committee are the most common. This risk is independent of the
defines ARDS as the acute onset of bilateral infiltrates on risk from concurrent aspiration.
chest radiography, a partial pressure of arterial oxygen
Medical Treatment for ARDS:
(PaO2) to fraction of inspired oxygen (FIO2) ratio of less
than 200 mmHg and a pulmonary artery occlusion pres- People with ARDS require hospitalization and treat-
sure of less than 18, or the absence of clinical evidence of ment in an intensive care unit.
left arterial hypertension. There is no specific treatment for ARDS, but rather,
The mortality rate is approximately 3040%. Death treatment is primarily supportive using a mechanical
usually results from multi-system organ failure rather respirator and supplemental oxygen.
than lung failure alone. Diuretics can be given to eliminate fluid from the
Causes: A number of clinical conditions are associated lungs. However, fluids are often given via IV to pro-
with the development of ARDS. vide nutrition and prevent dehydration, but fluids
must be carefully monitored to avoid fluid accumula-
Sepsis and the systemic inflammatory response syn-
tion in the lungs.
drome (SIRS) are the most common conditions asso-
Antibiotic therapy may be administered to treat infec-
ciated with the development of ARDS.
tion, which is often the underlying cause of ARDS.
Severe traumatic injury (especially multiple fractures),
Corticosteroids may sometimes be given late in the
severe head injury, and pulmonary contusion are
process of ARDS or if the patient is in shock. If the
strongly associated with the development of ARDS. In
patient is in shock, drugs to counteract low blood
traumatic injury, factures of the long bones can cause
pressure caused by shock may be administered.
ARDS through fat embolism. In severe brain injury,
If the patient is experiencing anxiety, this can be
ARDS is thought to develop owing to a sudden dis-
treated with anti-anxiety medications.
charge of the sympathetic nervous system, which then
leads to acute pulmonary hypertension and injury to Respiratory therapists may see these patients to provide
the pulmonary capillary bed. In pulmonary contusions, inhaled drugs to decrease inflammation and provide re-
ARDS develops through direct trauma to the lung. spiratory comfort.
Multiple blood transfusions are an independent risk Because of the acute and medically serious nature of
factor for ARDS. The risk is independent of the reason ARDS, it would be unlikely for neuropsychological exam
for the transfusion or the coexistence of trauma. to be requested when a person is acutely ill with ARDS.
The incidence of ARDS increases with the number of Mortality with ARDS is 3040% and the person would
units of blood transfused. If the patient has pre-exist- typically be treated in an Intensive Care Unit. If the person
ing abnormal liver functioning or a coagulation ab- survives, outpatient neuropsychological evaluation could
normality, the risk is further increased. be requested and results may show memory deficits related
Near drowning can be another cause of ARDS. Devel- to the hypoxia as well as neuropsychological deficits
opment of ARDS is slightly more common with salt- related to the underlying medical cause for ARDS (e.g.,
water than with fresh-water. Aspiration leads to an severe TBI, near drowning, sepsis, medication overdose).
osmotic gradient that favors movement of water into
airspaces of the lung. Aspiration may be visible with
chest radiography, although the chest radiograph may Cross References
be normal early in the course of the disease.
Smoke inhalation is another possible cause of ARDS. Anoxia
Smoke inhalation causes lung tissue damage from direct Hypoxia
heat, toxic chemicals, and particulate matter carried
into the lung. Patients with smoke inhalation initially
may be asymptomatic, but patients with airway burns,
exposure to toxic fumes, or exposure to carbon monox- References and Readings
ide should be monitored closely for the development of
Bernard, G. R., Artigas, A., Brigham, K. L., Charlet, J., Falke, K., Hudson, L,
ARDS, even if the symptoms are initially absent.
Lamy M., Legall, J. R., Morris, A., & Spragg, R. (1994). Report of the
Overdoses of narcotics, tricyclic antidepressants, and American-European consensus conference on ARDS: Definitions,
other sedatives have been associated with the develop- mechanisms, relevant outcomes and clinical trial coordination. In-
ment of ARDS. Overdoses of tricyclic antidepressants tensive Care Medicine, 20, 225232.
Adaptive Behavior Assessment System Second Edition A 37
Adaptive Behavior Assessment System Second Edition. Table 1 Adaptive skills and three adaptive domains
Adaptive skills
Communication Speech, language, and listening skills needed for communication with other people, including vocabulary,
responding to questions, and conversation skills
Community use Skills needed for functioning in the community, including use of community resources, shopping skills,
and getting around in the community
Functional Basic reading, writing, mathematics, and other academic skills needed for daily, independent functioning,
academics including telling time, measurement, as well as writing notes and letters
Home living Skills needed for basic care of a home or living setting, including cleaning, straightening, property
maintenance and repairs, as well as food preparation and performing chores
Health and safety Skills needed for protection of health and to respond to illness and injury, including following safety rules,
using medicines, and showing caution
Leisure Skills needed for engaging in and planning leisure and recreational activities, including playing with
others, engaging in recreation at home, and following rules in games
Self-care Skills needed for personal care including eating, dressing, bathing, toileting, grooming, and hygiene
Self-direction Skills needed for independence, responsibility, and self-control, including starting and completing tasks,
keeping a schedule, following time limits, following directions, and making choices
Social Skills needed to interact socially and get along with other people, including having friends, showing and
recognizing emotions, assisting others, and using manners
Work Skills needed for successful functioning and holding a part-time or full-time job in a work setting, including
completing work tasks, working with supervisors, and following a work schedule
Motor skillsa Basic fine and gross motor skills needed for locomotion, manipulation of the environment, and the
development of more complex activities such as sports, including sitting, pulling up to a standing position,
walking, fine motor control, and kicking
Three domains and associated skill areas
Conceptual Includes communication, functional academics, self-direction, and health and safety skills
Practical Includes social skills and leisure skills
Social Includes self-care, home/school living, community use, health and safety, and work skills
a
Although fine and gross motor development is not included as one of the ten skills identified by the American Association on Intellectual and
Developmental Disabilities, it is included in some scales of adaptive behavior.
referred to as intellectual disabilities by AAIDD). The of support (10th ed.). Washington, DC: American Association on
Mental Retardation. A
ABAS-II is useful in this diagnosis as well as in interven-
American Psychiatric Association. (2000). Diagnostic and statistical man-
tion planning and monitoring for this and other ual of mental disorders (4th ed., text revision). Washington, DC:
disorders. American Association on Mental Retardation.
The ABAS-II also may assist in promoting an under- Burns, M. K. (2005). Review of the adaptive behavior assessment system
standing of the impact on a persons daily life activities of second edition. In R. Spies & B. Plake (Eds.), The sixteenth mental
measurements yearbook. Lincoln, NE: Buros Institute of Mental
other disorders (e.g., those often diagnosed first during
Measurements.
infancy or early childhood include autism, disorders of Ditterline, J., Banner, D., Oakland, T., & Becton, D. (2008). Adaptive
attention, communication, conduct, elimination, feeding behavior profiles of students with disabilities. Journal of Applied
and eating, learning, motor skills, and pervasive develop- School Psychology, 24, 191208.
mental disorders; Harman, Smith-Bonahue, & Oakland, Harman, J., Smith-Bonahue, T., & Oakland, T. (2009). Assessment of
adaptive behavior development in young children. In E. Mpofu &
2009; Oakland & Harrison, 2008). The ABAS-II is
T. Oakland (Eds.). Rehabilitation and Health Assessment: Applying
useful with children and adolescents who display disor- ICF Guidelines. New York: Springer.
ders including attention deficit/hyperactivity, acquired Harrison, P. & Oakland, T. (2000). Adaptive behavior assessment system.
brain injury, auditory or visual impairment, autism, San Antonio, TX: Harcourt Assessment.
developmental delays, emotional/behavioral disorders, Harrison, P. & Oakland, T. (2003). Adaptive behavior assessment system
second edition. San Antonio, TX: Harcourt Assessment.
learning disabilities, and physical impairments (Ditter-
Meikamp, J., & Suppa, C. H. (2005). Review of the adaptive behavior
line, Banner, Oakland, & Becton 2008; Harrison & assessment system second edition. In R. Spies & B. Plake (Eds.),
Oakland, 2003; Oakland & Harrison, 2008). The sixteenth mental measurements yearbook. Lincoln, NE: Buros
Adults diagnosed with such disorders as anxiety, acute Institute of Mental Measurements.
stress or adjustment disorder, bipolar disorder, depres- Mpofu, E. & Oakland, T. (2010). Assessment in rehabilitation and health.
Upper Saddle River, NJ: Merrill.
sion, mood disorders, psychosis, Parkinsons, postpartum
Oakland, T. & Harrison, P. (2008). Adaptive behavior assessment system-II:
depression, substance abuse, schizophrenia, and sleep Behavior assessment system-II: Clinical use and interpretation.
disturbance may display impairments in their functional New York: Elsevier
daily living skills. Older adults diagnosed with Alzhei- Olley, J. G., & Cox, A. (2008). Assessment of adaptive behavior in adult
mers type dementia and other cognitive and neuropsy- forensic cases: The use of the ABAS-II. In T. Oakland, & P. Harrison,
(Eds.), Adaptive behavior assessment system-II: Clinical use and inter-
chological disorders with late-life onset often display
pretation. Boston: Elsevier
impairments in their functional daily living skills. Al- Rust, J. O. & Wallace, M. A. (2004). Test review: Adaptive behavior
though data from the ABAS-II may not be crucial in the assessment system second edition. Journal of Psychoeducational
diagnosis of some of these disorders, ABAS-II data will Assessment, 22, 367373.
promote an understanding of their impact on daily living Wei, Y., Oakland, T., & Algina, J. (2008). Multigroup confirmatory factor
analysis for the parent form, ages 521, of the adaptive behavior
skills. The ABAS-II is used in the assessment of mental
assessment system-II. American Journal on Mental Retardation. 113,
retardation among death row inmates in light of the 2002 178186.
US Supreme Court Atkins decision (Olley & Cox, 2008).
Adaptive Functions
Cross References
Activities of Daily Living (ADL)
Activities of Daily Living
Activity Restrictions and Limitations
Adaptive Behavior ADD
Intellectual Disabilities
Attention Deficit, Hyperactivity Disorder
Minimal Brain Dysfunction
References
Adenoma
ADHD, Predominantly
E THAN M OITRA Hyperactive-impulsive Type
Drexel University
Morgantown, WV, USA Attention Deficit, Hyperactivity Disorder
Minimal Brain Dysfunction
Definition
A benign tumor of glandular origin. There are three types ADHD, Predominantly Inattentive
of adenomas: tubular (most common; tube-like struc- Type
ture), villous (least common; most likely to become can-
cerous; ruffled structure), and tubulovillous (blend of Attention Deficit, Hyperactivity Disorder
tubular and villous structures). Adenomas do not metas- Minimal Brain Dysfunction
tasize, though they can develop into malignancies known
as adenocarcinomas. The tumor may occur throughout
the endocrine system, including the pituitary gland.
Pituitary adenomas occur at a much higher incidence ADI-R
in adults than in children. Because their invasiveness is
local, they are almost always benign and can be difficult to Autism Diagnostic Interview, Revised
detect. There is the secreting and the nonsecreting type.
Clinical symptoms come from the endocrine dysfunction
or from mass effect, and include headaches, hypopituita-
rism, and visual loss (caused by compression in the optic
ADLQ
chiasm). Treatment of pituitary adenomas includes cor-
Activities of Daily Living Questionnaire
rection of electrolyte dysfunction, replacement of pitui-
tary hormones, surgical resection, and radiotherapy.
evidence consists of a witness testimonial, it must be Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In
J. Morgan, & J. Ricker (Eds.). Textbook of clinical neuropsychology. A
established that the witness is credible and that he/she
New York: Taylor & Francis.
has knowledge of that which he/she is declaring. For Jenkins v. United States, 307 F. 2d 637 (1962).
neuropsychologists, a central issue is the admissibility Kaufmann, P. M. (2008). Admissibility of neuropsychological evidence in
of ones data and opinions. Rules 401, 402, and criminal cases: Competency, insanity, culpability, and mitigation.
702705 from Article VII of the Federal Rules of Evi- In R. Denney, & J. Sullivan (Eds.). Clinical neuropsychology in the
criminal forensic setting. New York: Guilford.
dence (FRE) relate to Opinions & Expert Testimony.
Perhaps of most relevance to psychologists is rule
FRE 702 which states, If scientific, technical, or other
specialized knowledge will assist the trier of fact to
understand the evidence or to determine a fact in Admissibility of Psychological
issue, a witness qualified as an expert by knowledge, Evidence
skill, experience, training or education, may testify
thereto in the form of an opinion or otherwise. In Jenkins v. U.S. (1962)
other words, the expert should possess some form of
knowledge that a typical judge or juror would not be
expected to know or understand. Rule 703 states, The
facts or data in the particular case upon which an Admissibility of Psychological/
expert bases an opinion or inference may be those
perceived by or made known to the expert at or before Neuropsychological Evidence
the hearing. If of a type reasonably relied upon by
experts in the particular field in forming opinions or Baxter v. Temple (2005)
inferences upon the subject, the facts or data need not
be admissible in evidence in order for the opinion or
the inference to be admitted. Facts or data that are
otherwise inadmissible shall not be disclosed to the Adoption Studies
jury by the proponent of the opinion or inference
unless the court determines that their probative value R OHAN PALMER 1, M ARTIN H AHN 2
1
in assisting the jury to evaluate the experts opinion University of Colorado
substantially outweighs their prejudicial effect. Boulder, CO, USA
2
Several important cases have addressed the admissi- William Paterson University
bility of scientific testimony. In the case of Frye v. United Wayne, NJ, USA
States (1923), the Frye standard was established which
stated that: only scientific methods and concepts with
general acceptance within a particular field are admissi- Definition
ble. In the more recent case of Daubert v. Merrell Dow
(1993), it was determined that scientific testimony has to Adoption studies typically compare pairs of persons, e.g.,
abide by two criteria, the testimony must be: (a) scientifi- adopted child and adoptive mother or adopted child and
cally valid and (b) relevant to the case at hand. biological mother to assess genetic and environmental
influences on behavior.
families that share only genetic or environmental factors. References and Readings
Adoption creates two types of families. The genetic
family consists of pairs of genetically related individuals Bricker, J. B., Stallings, M. C., Corley, R. P., Wadsworth, S. J., Bryan, A.,
who do not share a common family environment (e.g., Timberlake, D. S., et al. (2006). Genetic and environmental
influences on age at sexual initiation in the Colorado adoption
biological parent and adopted-away child). The similar-
project. Behavior Genetics, 36, 820832.
ity between these pairs of relatives provides a direct Petrill, S. A., Plomin, R., DeFries, J. C., & Hewitt, J. K. (2003). Nature,
estimate of genetic effects on behaviors. The second nurture, and the transition to early adolescence. Oxford: Oxford
type family is the environmental family, which is University Press.
made up of pairs of individuals who are not genetically Plomin, R., Fulker, D. W., Corley, R., & DeFries, J. C. (1997). Nature,
nurture, and cognitive development from 1 to 16 years: A parent-
related but who share a common family environment (e.
offspring adoption study. Psychological Science, 8, 442447.
g., adoptive parent and adopted child). The similarity Young, S. E., Rhee, S. H., Stallings, M. C., Corley, R. P., & Hewitt, J. K.
between pairs of relatives from an environmental (2006). Genetic and environmental vulnerabilities underlying
family indicates the presence of environmental influ- adolescent substance use and problem use: General or specific?
ences on behavior. Adoption studies utilize either Behavior Genetics, 36, 603615.
parentoffspring pairs or sibling pairs. Because data
on biological parents and siblings of adoptees are
sometimes rare, comparison between genetic-plus-
environmental families (i.e., intact families) and adoptive
ADOS
families also provides evidence of genetic and environ-
Autism Diagnostic Observation Schedule
mental influences.
Definition
Cross References
Adrenocorticotropic hormone (ACTH) is produced by
Twin Studies the anterior pituitary gland and is a component of the
Advanced Progressive Matrices A 43
Historical Background
Adult Respiratory Distress The APM was designed in the 1940s to assess nonverbal
Syndrome abstract conceptualization skills of individuals for whom
the standard version was too easy; that is, those achiev-
Acute Respiratory Distress Syndrome ing a raw score of 50 or above on the SPM. For children
over 10 years of age with high intellectual functioning,
the APM may be the appropriate version to ensure an
adequate ceiling (Mills, Ablard, & Brody, 1993). For
additional information about the historical background
Advanced MS of the original test, please refer to the entry for Ravens
Progressive Matrices.
Secondary-Progressive Multiple Sclerosis
Psychometric Data
Advanced Progressive Matrices Norms for adolescents (ages 1216.5) and adults (1868+;
Sets I and II) for untimed (ages 1270+) and timed (ages
V ICTORIA M. L EAVITT 1728) versions are provided for North America (Raven,
Kessler Foundation Research Center Raven Court, 1998).The reliability of the test is considered
West Orange, NJ, USA good, with high internal consistency of APM Set II, and
split-half reliability coefficients varying between 0.83 and
0.87 (Strauss, Sherman, & Spreen, 2006). Set I, as it has
Synonyms only 12 items, yields lower figures. Reliability of the
original 48-item version was found to be high for adults
APM and children aged 11.5 years+ (>0.80); for younger
44 A Advocacy
Synonyms Advocate
Advocate; Support Advocacy
Affective Disorder A 45
Definition A
Adynamia
Affect is the display and experiencing of emotion. It
I RENE S HULOVA -P IRYATINSKY includes positive dimensions such as joy, interest, and
Butler Hospital contentment, as well as negative dimensions of emotion
Providence, RI, USA such as disgust, fear, and anger. Affect is a very rapid
response to internal (e.g., thoughts, memory) or external
stimuli (e.g., other people). It is different from mood,
Synonyms in that it is more momentary and observable by
others, whereas mood is longer-lasting and constitutes a
Asthenia symptom that patients may report (e.g., depression).
Affect can be observed from facial expression, gestures,
Definition posture, and speech (e.g., word choice, tone, rate).
Berrios, G. E. (2008). Classic text no. 76: Asthenia by A. Dechambre Affect Display
(1865). History of Psychiatry, 19(4), 490501.
Caplan, D. (1987). Neurolinguistics and linguistic aphasiology: An Affect
introduction. New York: Cambridge University Press.
Synonyms Synonyms
bipolar disorder, but more research is warranted. The have exaggerated responses to negative feedback, includ-
A
causes of depression are not fully understood, but appear ing rumination. Neuropsychological evaluations in de-
to involve the interaction of genetic and environmental pression and bipolar disorder are used frequently in
factors such as stress and disruptions in interpersonal research, as tests with broad clinical utility in the context
relationships. Thus, in addition to female gender, risk of assessing or treating affective disorders have not been
factors for depression include severe life stress such as widely disseminated.
traumatic events and loss of significant relationships. De-
pression is associated with shorter life expectancy from
suicide and other causes of death. For example, depres- Treatment
sion increases risk of cardiac disease, as well as risk of
mortality among individuals with cardiac disease. Depression is often treated with medication and/or psy-
chotherapy. A large number of medications are available
to treat depression, including selective serotonin reuptake
Neuropsychology and Psychology of inhibitors, which typically have relatively milder side
Affective Disorder effects and lower risks than older drugs such as mono-
amine oxidase inhibitors. Treatment of bipolar disorders
Depression is common in neurological conditions such as requires pharmacotherapy. In contrast to major depres-
stroke and traumatic brain injury (Robinson, 2006; sive disorder, bipolar cannot be successfully treated by
Rosenthal, Christensen, & Ross, 1998). Even without an psychotherapy alone.
obvious neurologic insult, individuals with alterations in
executive control, memory, and emotion regulation are at
increased risk for depression. Furthermore, individuals Cross References
with depression often show neuropsychological deficits
in the absence of neurological conditions. The neuropsy- Depressive Disorder
chological deficits specified in the diagnostic criteria for
depression include difficulty concentrating and making
decisions. Thus, depressed patients often exhibit deficits
in executive control, memory, and processing speed. For References and Readings
bipolar disorder, distractibility is typically present, as well
Allen, L. B., McHugh, R. K., & Barlow, D. H. (2008). Emotional disorders:
as impaired decision making reflected in the criterion
A unified protocol. In D. H. Barlow (Ed.), Clinical handbook of
relating to distractibility of excessive involvement in psychological disorders: A step-by-step treatment manual (4th ed.)
activities that present significant risk of negative conse- (pp. 216249). New York: Guilford Press.
quences. Current neuropsychological theories of depres- American Psychiatric Association (2000). Diagnostic and statistical man-
sion emphasize the frontal lobes and basal ganglia, ual of mental disorders (4th ed.). Text Revision. Washington, DC:
American Psychiatric Association.
including abnormalities in neural circuitry involving the
Chamberlain, S. R., & Sahakain, B. J. (2006). The neuropsychology of
prefrontal cortex, mesiotemporal cortex, striatum, amyg- mood disorders. Current Psychiatry Reports, 8, 458463.
dala, and thalamus (Chamberlain & Sahakain, 2006). Clark, L., Chamberlain, S. R., & Sahakian, B. J. (2009). Neurocognitive
These areas may also be implicated in bipolar disorder, mechanisms in depression: Implications for treatment. Annual
as they appear to underlie mood symptoms and treatment Review of Neuroscience, 32, 5774.
Robinson, R. (2006). The neuropsychiatry of stroke (2nd ed.). New York:
effects.
Cambridge University Press.
Rosenthal, M., Christensen, B., & Ross, T. (1998). Depression following
traumatic brain injury. Archives of Physical Medicine and
Rehabilitation, 79, 90103.
Evaluation
Cross References
Afferent
Lemniscal System
J OHN B IGBEE
Virginia Commonwealth University
Richmond, VA, USA References and Readings
Sensory
Age Decrements
Definition S ANDRA B ANKS
Allegheny General Hospital
Afferent is an anatomical term that indicates functional Pittsburgh, PA, USA
directionality. In nervous tissue, afferent is often used
synonymously with sensory information when it refers
to nerves carrying impulses from peripheral receptors Synonyms
toward the central nervous system. Afferent can also be
used in general to refer to any connection coming into a Age-associated cognitive decline
structure within the nervous system. The opposite direc-
tion of conduction is efferent.
Definition
Age-associated Cognitive Decline Aicardi, J., Chevrie, J.-J., & Baraton, J. (1987). Agenesis of the corpus
callosum. In P. J. Vinken, G. W. Bruyn, & H. L. Klawans (Eds.),
Handbook of clinical neurology (Vol.50, pp. 149173). New York:
Age Decrements Elsevier.
Mild Cognitive Impairment Marszal, E., Jamroz, E., Pilch, J., Kluczewska, E., Jablecka, H., &
Krawczyk, R. (2000). Agenesis of the corpus callosum: Clin-
ical description and etiology. Journal of Child Neurology, 15,
401405.
Zaidel, E., & Iacoboni, M. (2003). The parallel brain: The cogni-
tive neuroscience of the corpus callosum. Cambridge, MA: MIT
Age-Associated Memory Press.
Impairment (AAMI)
Benign Senescent Forgetfulness
Ageusia
M ARYELLEN R OMERO
Agenesis of Corpus Callosum Tulane University Health Sciences Center
New Orleans, LA, USA
J OHN E. M ENDOZA
Tulane University Medical Center
New Orleans, LA, USA Definition
Bogner, J. A., Corrigan, J. D., Stange, M., & Rabold, D. (1999). Reliability Current Knowledge
of the Agitated Behavior Scale. Journal of Head Trauma Rehabilitation,
14, 9196.
Corrigan, J. D. (1989). Development of a scale for assessment of agitation
There was no consensus on the definition of agitation
following traumatic brain injury. Journal of Clinical and Experimental within the greater health-care profession for many years.
Neuropsychology, 11, 261277. Clinicians in neuro-rehabilitation were using the term in
Corrigan, J. D. & Bogner J. A. (1994). Factor structure of the Agitated the early 1980s to describe a pattern of behavior observed
Behavior Scale. Journal of Clinical and Experimental Neuropsychology,
during recovery from traumatic brain injury. The devel-
16, 386392.
Corrigan, J. D. & Mysiw, W. J. (1988). Agitation following traumatic
opment of the Agitated Behavior Scale by Corrigan and
head injury: equivocal evidence for a discrete stage of cognitive associates in the late 1980s to measure this brain-injury-
recovery. Archives of Physical Medicine and Rehabiltation, 69, related behavior led to a more refined definition of the
487492. term. The term is not limited to just traumatic brain
Kadyan, V., Mysiw, W. J., Bogner, J. A., Corrigan, J. D., Fugate, L. P., &
injury as agitation can manifest in any setting in which
Clinchot, D. M. (2004). Gender differences in agitation after
traumatic brain injury. American Journal of Physical Medicine &
an individual experiences delirium and impaired cogni-
Rehabilitation, 83, 747752. tion (e.g., dementia).
The importance of the concept of agitation and its
measurement was vital to the establishment of the now
accepted viewpoint that recovery from agitation is pre-
ceded by improvement in cognition. Or conversely, inter-
Agitation ventions that decrease arousal and/or cognition can lead
to a worsening of agitation.
PAUL D. N EWMAN
Drake Center
Cincinnati, OH, USA Cross References
Agitated Behavior Scale
Synonyms Behavior Management
Deescalation
Posttraumatic agitation Dementia
Frustration Tolerance
Post-traumatic Confusional State
Definition Traumatic Brain Injury
Cross References A
Agnosia
Apperceptive Visual Agnosia
A NASTASIA R AYMER Associative Visual Agnosia
Old Dominion University Auditory Agnosia
Norfolk, VA, USA Pure Word Deafness
Tactile Agnosia
Visual Object Agnosia
Definition
Current Knowledge
Nonfluent Aphasia Loverso, F. L., Prescott, T. E., & Selinger, M. (1986). Cuing verbs:
A treatment strategy for aphasic adults. Journal of Rehabilitation A
Paragrammatism
Research, 25, 4760.
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Telegraphic Speech tion: Effects of targeted intervention. In M. J. Riddoch & G. Hum-
phreys (Eds.), Cognitive neuropsychology and cognitive rehabilitation.
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Saffran, E. M., Berndt, R. S., & Schwartz, M. F. (1989). The quantitative
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46, 30883100.
Friedmann, N., Wenkert-Olenik, D., & Gil, M. (2000). From theory to P ELAGIE M. B EESON 1,2 , S TEVEN Z. R APCSAK 1,2,3
1
practice: Treatment of agrammatic production in hebrew based on Department of Speech, Language, & Hearing Sciences
the tree pruning hypothesis. Journal of Neurolinguistics, 13, 250254. 2
Department of Neurology
Grodzinsky, Y. (1986). Language deficits and syntactic theory. Brain and 3
Southern VA Health Care System, The University of
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ence, 4(2), 5156.
Synonyms
Kolk, H. H. J., & Heeschen C. (1990). Adaptation symptoms and im-
pairment symptoms in Brocas aphasia. Aphasiology, 4, 221231. Written language disorders
56 A Agraphia
Agraphia is the term applied to acquired disorders of Agraphia is commonly observed following damage to the
spelling or writing caused by neurological damage in language-dominant left hemisphere. Although it is most
individuals with normal premorbid literacy skills. There frequently caused by stroke, agraphia can follow any kind
are several different agraphia profiles that variously result of focal damage to the brain regions critical for
from impairments of spelling knowledge, sound-to-letter implementing the various cognitive operations necessary
correspondences, letter-shape information, or motor for normal spelling and writing. Agraphia is also observed
control for handwriting. Although agraphia can occur in individuals with neurodegenerative disorders, including
in relative isolation, it often co-occurs with acquired those with primary progressive aphasia/semantic dementia
impairments of reading (alexia) and spoken language or Alzheimers disease. The specific agraphia profile reflects
(aphasia). the region of cortical damage or atrophy.
Categorization
Natural History, Prognostic Factors,
Several distinct forms of acquired agraphia occur that
Outcomes
reflect specific combinations of impaired and preserved
The prognosis for recovery from agraphia depends on the
spelling and writing abilities following damage to certain
etiology of the lesion and the extent of the underlying
brain regions. Spelling difficulties can result from damage
brain damage. Agraphia following stroke or traumatic
to central linguistic processes supported by the language-
brain injury tends to show some spontaneous recovery
dominant hemisphere in a manner analogous to acquired
in the first months after brain damage occurs, but residual
impairments of reading ( alexia). Agraphia can also
impairments often persist. Additional improvements may
result from disruption of peripheral processing compo-
be achieved with behavioral treatment directed toward
nents that guide the selection and production of appro-
strengthening the weakened cognitive processes that sup-
priate letter shapes.
port spelling or motor control for writing. In individuals
Common central agraphia syndromes
with neurodegenerative disorders, progressive worsening
Phonological agraphia refers to an impaired ability to of the spelling impairment is observed along with the
manipulate the sound system of the language gradual deterioration of other language and cognitive
(phonology) which manifests as a disproportionate functions.
difficulty with the spelling of nonwords (e.g., flig,
merber) compared with real words.
Deep agraphia is characterized by a marked im- Neuropsychology and Psychology
pairment of spelling ability for nonwords, as seen in of Agraphia
phonological agraphia, but with the additional hall-
mark feature of semantic errors (e.g., car for vehicle). Written words are typically produced in response to
Surface agraphia (also called lexical agraphia) is activation of a concept in the semantic system. The
characterized by relatively preserved ability to spell motivation to write a word may be driven by the desire
nonwords and regularly spelled words in the face of to convey a message, or in response to an auditory stimu-
marked impairment of spelling words with irregular lus, as in the context of writing a word to dictation. As
soundletter correspondences, such as choir. depicted in the cognitive model of single-word processing
in Fig. 1, the word meaning (semantics) and the
Common peripheral agraphia syndromes
phonological word form (phonology) both provide access
Allographic agraphia is an impairment of written to spelling knowledge (orthography). In literate adults,
spelling due to errors in letter selection. the spellings of familiar words are easily recalled as whole
Apraxic agraphia is an impairment of the selection words from ones spelling vocabulary (i.e., orthographic
and implementation of graphic motor programs lexicon). In contrast to this lexical approach, spellings can
necessary to move the hand to form letter shapes. be assembled on the basis of the knowledge of sound-to-
Micrographia is the production of abnormally small letter correspondences using a sublexical processing
letters due to defective control of the force, speed, and strategy as depicted in Fig. 1. A sublexical approach is
amplitude of handwriting movements. often employed when one is unsure about the spelling of a
Agraphia A 57
Semantics A
Phonology Orthography
Lexical
Words Words
Spoken word Written word
Sublexical
Auditory Visual
Phonemes Letters
analysis analysis
Speech Writing
Agraphia. Figure 1 A cognitive model indicating the component processes involved in spelling and writing
word, or when required to spell an unfamiliar word or a to damage to the sublexical route, while the better
nonword, such as glope. Spelling via soundletter corre- preserved real-word spelling by these patients reflects the
spondences is likely to yield correct responses for regularly residual functional capacity of the lexical and semantic
spelled words, such as drive, but over-reliance on the routes. There is evidence to suggest that phonological
sublexical route will result in phonologically plausible agraphia reflects a central impairment of phonological
errors for irregularly spelled words, such as kwire for processing ability that is also apparent on reading tasks;
choir. Thus, according to a dual-route model as depicted however, the spelling impairment is typically of greater
in Fig. 1, only the lexical route can deliver correct spellings severity due to the fact that spelling is a harder task than
for irregularly spelled words. The final stages of writing reading (Rapcsak et al., 2009). Although spelling accuracy
require translation of abstract spelling knowledge into for words (both regular and irregular) is better preserved
letter shapes and selection and implementation of the than spelling of nonwords, performance is often degraded
graphic motor programs for the appropriate handwriting to some extent relative to premorbid performance. Due to
movements. The various agraphia syndromes reflect the reliance on lexical processing with limited sublexical
specific impairments to these component processes input, real word spelling is typically influenced by lexical-
necessary for spelling and writing. semantic variables such as word frequency (high > low),
imageability (concrete > abstract), and grammatical class
(nouns > verbs > functors). Deep agraphia includes all of
Phonological/Deep Agraphia the characteristic features of phonological agraphia, but it
is distinguished from the latter by the production of
Phonological agraphia is characterized by difficulty in semantic errors (e.g., husband written as wife). In essence,
the generation of spellings on the basis of sound-to-letter deep agraphia can be considered a more severe form of
correspondences. This problem is particularly evident phonological agraphia.
during clinical evaluation when an individual is asked to Like phonological/deep alexia, phonological/deep
generate plausible spellings for nonwords. The dispropor- agraphia is typically encountered in patients with aphasia
tionate difficulty in spelling nonwords compared to syndromes characterized by phonological impairment in-
familiar words gives rise to an exaggerated lexicality effect cluding Brocas, conduction, and Wernickes aphasia. In
(Henry, Beeson, Stark, & Rapcsak, 2007; Rapcsak et al., such cases, there is damage to a network of perisylvian
2009). According to a dual-route model (Fig. 1), poor cortical regions involved in speech production/perception
nonword spelling in phonological agraphia is attributable and phonological processing including Brocas area,
58 A Agraphia
precentral gyrus, insula, Wernickes area, and supramar- representations as observed in individuals with semantic
ginal gyrus (Fig. 2). The contribution of these regions to dementia (Graham, Patterson, & Hodges, 2000). The
phonological processing skills is evident from lesion stud- reduction in the ability to process lexical-semantic
ies, but also in functional imaging studies of healthy information in such individuals results in overreliance
individuals when they perform a variety of written and on sublexical spelling procedures and regularization
spoken language tasks requiring phonological processing errors. As expected, it is not uncommon to observe
(Jobard et al., 2003; Vigneau et al., 2006; Rapcsak et al., co-occurance of surface alexia and agraphia in individuals
2009). In individuals with deep agraphia, the left hemi- with semantic dementia (Graham et al., 2000).
sphere damage tends to be more extensive than that Surface agraphia, like surface alexia, is typically
associated with phonological agraphia, and it has been associated with extrasylvian brain pathology (Fig. 2).
hypothesized that the right hemisphere may be responsi- Focal lesions that give rise to surface agraphia have been
ble for the characteristic deep agraphia profile (Rapcsak, documented in the left inferior occipito-temporal cortex
Beeson, & Rubens, 1991). (Rapcsak & Beeson, 2004). This region includes a portion
of the fusiform gyrus known as the visual word form area
that has been shown to be engaged in healthy adults
Surface Agraphia during reading (Cohen et al., 2002) and spelling tasks
(Beeson et al., 2003) and may represent the neural sub-
Surface agraphia is characterized by difficulty in spelling strate of the orthographic lexicon. Surface agraphia has
irregular words, which contain atypical sound-to-letter cor- also been described following focal damage to posterior
respondences. Regular words are spelled with significantly middle/inferior temporal gyrus and angular gyrus (Rapc-
better accuracy, thus yielding a regularity effect. Nonword sak & Beeson, 2002) and in patients with left anterior
spelling is relatively preserved. In a manner analogous to temporal lobe atrophy (Graham et al., 2000). In these
surface alexia, a dual-route theory attributes surface cases, the spelling deficit may reflect damage to a
agraphia to dysfunction of the lexical spelling route distributed extrasylvian cortical network involved in se-
(Fig. 1). Specifically, it has been suggested that the spelling mantic processing (Fig. 2).
disorder results from damage to the orthographic lexicon
(Rapcsak & Beeson, 2004). The loss of word-specific
orthographic knowledge prompts reliance on a sublexical Allographic Agraphia
phonemegrapheme conversion strategy that produces
phonologically plausible regularization errors on irregular Allographic agraphia refers to a disturbance of the ability
words, a finding that is most pronounced on low to activate or select appropriate letter shapes for the
frequency items (e.g., yot for yacht). Surface agraphia abstract orthographic representations generated by
may also result from damage to central semantic central spelling routes. This impairment of handwriting
Graphomotor control
Phonology
Semantics
Orthography
Beeson, P. M., & Rapcsak, S. Z. (2002). Clinical diagnosis and treatment (weight), or resistance to pressure, with difficulties in
of spelling disorders. In A. E. Hillis (Ed.), Handbook on adult lan-
perceiving size or shape is referred to as amorphogno-
guage disorders: Integrating cognitive neuropsychology, neurology, and
rehabilitation (pp. 101120). Philadelphia: Psychology Press.
sia. While perhaps seeming a bit artificial, according to
Beeson, P. M., & Rapcsak, S. Z. (2006). Treatment of alexia and agraphia. Bauer and Demery (2003), the distinction between ahy-
In J. H. Noseworthy (Ed.), Neurological Therapeutics: Principles and lognosia and amorphognosia apparently traces back to
Practice (2nd ed., pp. 30453060). London: Martin Dunitz. 1935 when a French neurologist, Delay, divided astereog-
Beeson, P. M., Rapcsak, S. Z., Plante, E., Chargualaf, J., Chung, A.,
nosis into two subtypes of deficits: amorphognosia, which
Johnson, S. C., et al. (2003). The neural substrates of writing: A
functional magnetic resonance imaging study. Aphasiology, 17,
was defined as a difficulty in recognizing the size or shape
647665. of an object by touch, and ahylognosia, which was de-
Cohen, L., Lehericy, S., Chochon, F., Lemer, C., Rivaud, S., & Dehaene, S. scribed as a failure to differentiate the molecular quali-
(2002). Language-specific tuning of visual cortex? Functional ties of an object, such as its density, weight, thermal
properties of the Visual Word Form Area. Brain, 125, 10541069.
conductivity, or roughness. Delay also defined a third
Graham, N. L., Patterson, K., & Hodges, J. R. (2000). The impact of
semantic memory impairment on spelling: evidence from semantic
type of astereognosis, tactile asymboly, which was char-
dementia. Neuropsychologia, 38, 143163. acterized as the inability to identify an object by touch
Henry, M. L. Beeson, P. M., Stark, A. J., & Rapcsak, S. Z. (2007). The role in the absence of amorphognosia and ahylognosia. These
of left perisylvian cortical regions in spelling. Brain and Language, same distinctions were followed by Critchley (1969)
100, 4452.
and continue to be used by more recent authors (Bauer
Jobard, G., Crivello, F., & Tzourio-Mazoyer, N. (2003). Evaluation of the
dual route theory of reading: A metaanalysis of 35 neuroimaging
& Demery, 2003). Hecaen and Albert (1978) in their
studies. NeuroImage, 20, 693712. book, Human Neuropsychology, attempted to explain
Kay, J., Lesser, R., & Coltheart, M. (1992). Psycholinguistic assessments these distinctions by suggesting that ahylognosia was
of language processing in Aphasia (PALPA). East Sussex, England: the loss of the capacity to differentiate structural
Lawrence Erlbaum Associates.
components of objects, resulted from impairment of
Rapcsak, S. Z., & Beeson, P. M. (2000). Agraphia. In L. J. G. Rothi, B.
Crosson, & S. Nadeau (Eds.), Aphasia and language: Theory and
intensity analyzers. By contrast, amorphognosia, was
practice (pp. 184220). New York: Guilford. thought to reflect the loss of the capacity to differentiate
Rapcsak, S. Z., & Beeson, P. M. (2004). The role of left posterior inferior forms, resulted from impairment of the analyzers of ex-
temporal cortex in spelling. Neurology, 62, 22212229. tent. Because determining any of these qualities requires
Rapcsak, S. Z., Beeson, P. M., Henry, M. L., Leyden, A., Kim, E. S.,
discriminatory judgments, in the absence of more ele-
Rising, K., et al. (2009). Phonological dyslexia and dysgraphia:
cognitive mechanisms and neural substrates. Cortex, 45(5), 575591.
mentary tactual defects, such disturbances suggest pathol-
Rapcsak, S. Z., Beeson, P. M., & Rubens, A. B. (1991). Writing with the ogy involving the somatosensory areas of the parietal
right hemisphere. Brain and Language, 41, 510530. lobe.
Tainturier, M.-J., & Rapp, B. (2001). The spelling process. In B. Rapp
(Ed.), The handbook of cognitive neuropsychology (pp. 233262).
Philadelphia: Psychology Press.
Vigneau, M., Beaucousin, V., Herve, P. Y., Duffau, H., Crivello, F.,
Houde, O., et al. (2006). Meta-analyzing left hemisphere language Cross References
areas: phonology, semantics, and sentence processing. NeuroImage,
30, 14141432. Amorphognosis
Astereognosis
Tactile Agnosia
Ahylognosia
J OHN E. M ENDOZA References and Readings
Tulane University Medical Center
New Orleans, LA, USA Bauer, R. M., & Demery, J. A. (2003). Agnosia. In K. Heilman, &
E. Valenstein (Eds.), Clinical Neuropsychology, (4th ed., pp.
236295). New York: Oxford University Press.
Critchley, M. (1969). The parietal lobes. New York: Hafner Publishing Co.
Definition Delay, J. (1935). Les astereognosis. Pathologie due Toucher, Clinque, Phy-
siologie, Topographie. Paris: Masson.
Inability to determine by touch alone certain physical Hecaen, H., & Albert, M. L. (1978). Human neuropsychology (Chapter 6,
properties of an object such as its texture, density Disorders of somesthesis and somatognosis). New York: Wiley.
Akelaitis, Andrew John Edward (A.J.) (19041955) A 61
Major Appointments
Synonyms
Dr. A.J. Akelaitis began his career as an assistant pro-
Restlessness fessor in the Department of Medicine, Division of
Psychiatry, at the University of Rochester School of
Medicine and Dentistry. At the same time, he also held
appointments at the clinics of the Strong Memorial
Definition and Rochester Municipal Hospitals in Rochester, New
York. He left these appointments to serve in the Navy
Akathisia is a syndrome characterized by unpleasant
during World War II. Following his service in the war,
sensations of inner restlessness that manifests itself with
Dr. Akelaitis worked as an Assistant Professor of Neu-
an inability to sit still or remain motionless.
rology at the New York Medical College and Assistant
Professor of Clinical Medicine in Neurology at Cor-
nell University Medical College. He also served as the
attending neuropsychiatrist at Mount Vernon (New
Current Knowledge
York) Hospital and on the staff of the Bellevue Hospi-
tal and the New York Hospital.
It is most often seen as a side effect of medications, mainly
neuroleptic antipsychotics. Patients may have difficulty
describing their symptoms, leading to a misdiagnosis of
Major Honors and Awards
anxiety and worsening of the condition upon treatment
with neuroleptic antipsychotic agents. Several medica-
Dr. Akelaitis was a Fellow of the American Psychiatric
tions have been used to treat the condition, including
Association. He was specialty certified by the Ameri-
benztropine and beta-blocking agents. Withdrawal of the
can Board of Psychiatry and Neurology and held
offending agent is often most effective. It may be seen with
membership appointments in the American Medical
Parkinsons disease.
Association, the New York State Medical Society, the
New York Society for Clinical Psychiatry, and the New
York Neurological Society.
Cross References
Parkinsons Disease
Landmark Clinical, Scientific, and
Tardive Dyskinesia
Professional Contributions
Dr. A.J. Akelaitis is best known for his observations of
patients who underwent sectioning of the corpus cal-
References and Readings losum (i.e., split-brain patients). Beginning in the
late 1930s, the neurosurgeon Dr. William P. van
Kumar, A., & Calne, D. (2004). Approach to the patient with a movement
Wagenen pioneered surgical sectioning of the corpus
disorder and overview of movement disorders. In R. L. Watts, & callosum for the treatment of intractable epilepsy
W. C. Koller (Eds.), Movement disorders (2nd ed., p. 9). New York: (Mathews, Linskey, & Binder, 2008). Dr. Akelaitis
McGraw-Hill. worked closely with Dr. van Wagenen and performed
62 A Akelaitis, Andrew John Edward (A.J.) (19041955)
pre and postoperative tests of cognitive and neurolog- Despite his contributions as one of the first indivi-
ical functioning on many of these individuals. Accord- duals to study neurological functioning following
ing to Akelaitis reports, patients who underwent callosotomy, Akelaitis has been criticized for employ-
callosotomy surgery largely did not show lasting ing insensitive or inadequate testing procedures.
changes in cognitive, intellectual, or motor function- However, reviews of his cases have confirmed that
ing, although their seizure activity was consistently his patients did exhibit what are now considered
alleviated. For nearly two decades, Akelaitis reports typical symptoms, although his explanations for
of largely normal functioning after callosotomy per- these manifestations, while consistent with much
petuated the generally accepted belief that sectioning of the research of the time, were often inadequate
the corpus callosum did not impact cognitive or (Sauerwein & Lassonde, 1996). In the 1950s and
motor functioning in humans. 1960s, researchers including Roger Sperry, Michael
Despite his reports of few neurological changes fol- Gazzaniga, Norman Geschwind, Edith Kaplan, and
lowing callosotomy, Akelaitis noted periodic cases Joseph Bogen began to publish articles involving cal-
with hemiplegia and praxic disturbances. He was losotomies in animals and humans, which contra-
slow, however, to include the sectioning of the corpus dicted many of Akelaitis findings. This sparked
callosum in his explanations for these changes; rather, renewed interest in the function of the corpus callo-
he attributed the symptoms to unintended operative sum and eventually earned Sperry the Nobel Prize
damage to adjacent cortical areas. In some cases, post- in 1981.
operative symptoms were seen as exacerbations of Through the course of his short career, Dr. Akelaitis
precallosotomy characteristics or were attributed to made significant contributions toward research on the
preexisting and/or postoperative psychological or be- corpus callosum and advanced the treatment of
havioral factors. Further, many of the symptoms ob- intractable epilepsy. He also published articles regard-
served by Akelaitis were transient and consequently ing the psychiatric aspects of myxedema (severe hypo-
not considered to be conclusively linked with callosal thyroidism), hereditary and vascular cerebral atrophy,
sectioning (Sauerwein & Lassonde, 1996). lead encephalopathy, acute demyelinating processes
Several factors most likely influenced Akelaitis reports (multiple sclerosis), and Picks disease.
of minimal neurological changes following callosotomy
surgery. First, the majority of patients Akelaitis observed
did not have complete callosotomies, nor were neuro-
Short Biography
surgical procedures well standardized at the time. Of the
28 patients he studied, only one third were reported to
Andrew John (A.J.) Akelaitis was born in Baltimore,
have undergone complete callosal sectioning, with the
Maryland, on July 11, 1904. He studied medicine
remainder nearly complete or partial sectioning
at Johns Hopkins University and received his M.D. in
(Bogen, 1995). The patients with only partially sec-
1929. In the early 1930s, he practiced clinical neurology
tioned callosal fibers undoubtedly continued to have
in Rochester, New York. He subsequently became an As-
interhemispheric transmission, thereby contributing
sistant Professor of Medicine at the University of
to Akelaitis findings of generally intact functioning.
Rochester School of Medicine and Dentistry. Dr. Akelaitis
Next, emerging research at the time reported no cogni-
joined the Navy during World War II where he served
tive changes following sectioning of the corpus callo-
with distinction at the rank of Commander. He married
sum. For example, Walter Dandy stated in 1936 that
the former Victoria Chesno. The couple had one son,
when the corpus callosum is split longitudinally. . . no
Andrew, and a daughter, Lillian. Akelaitis died at the
symptoms follow its division. This simple experiment at
New York Hospital on November 24, 1955 at the young
once disposes of the extravagant claims to the functions
age of 51.
of the corpus callosum (see Zaidel, Iacoboni, Zaidel, &
Bogen, 2003). Finally, Akelaitis lacked the technolo-
gies, such as the tachistoscope used by his successors,
to present stimuli to one visual field. Such technology Cross References
would possibly have given him insight into the spe-
cialization of the two hemispheres and interhemi- Corpus Callosum
spheric transfer of information via the corpus Epilepsy
callosum (Mathews, Linskey, & Binder, 2008). Split Brain
Akinetic Mutism A 63
Akinetic Mutism. Figure 1 Arrows show the left greater than right anterior cingulate lesions due to bilateral anterior cerebral
artery (ACA) ischemic stroke. Bilateral ACA lesions usually result in death due to loss of all limbic motivational input to prefrontal
cortex
profoundly apathetic, incontinent, and akinetic. They do area, and the oculomotor circuit, originating in the fron-
not initiate eating or drinking and if speech occurs, it is tal eye fields, are dedicated to motor function. The dorso-
restricted to terse responses. They seem awake, visually lateral prefrontal, lateral orbitofrontal, and anterior
tracking objects, but displaying no emotions even dur- cingulate circuits support executive cognitive functions,
ing painful circumstances, they remain indifferent. The personality, and motivation, respectively (Mega &
akinetic mute state also results from bilateral subcortical Cummings, 1994). Each of the five circuits has the same
paramedian diencephalic and midbrain lesions possibly member structures: the frontal lobe, striatum, globus
affecting the ascending reticular core, medial forebrain pallidus, substantia nigra, and thalamus. There is a
bundles, and isolated bilateral globus pallidus lesions. progressive spatial compaction of the circuits as they
travel through the basal ganglia. A lesion anywhere
along the path of a circuit will produce the same clinical
Categorization result but only in the globus pallidus interna are all the
frontal-subcortical circuits in such a compact spatial
When anterior cingulate lesions are bilateral, limbic, cogni- volume that a relatively small lesion can have profound
tive, and motor activation is disrupted producing profound effects.
akinetic mutism. Loss of ascending input from the reticular
core, due to bilateral lesions of the medial forebrain bundle,
may also produce akinetic mutism. Rarely are complete Epidemiology
bilateral lesions seen in humans, more frequently partial
circuit disruption results in a graded loss in motivation Akinetic mutism is exceedingly rare when permanent,
depending upon which circuit is damaged. since a bilateral lesion is necessary and usually results in
Five frontal-subcortical circuits have been named death. Unilateral anterior cerebral artery (ACA) strokes
according to their function or cortical site of origin: the are the usual cause of transient akinetic mutism, but ACA
motor circuit, originating in the supplementary motor strokes only make up 1% of all cerebral vascular lesions.
Akinetic Mutism A 65
Natural History, Prognostic Factors, skeletomotor effector region. When these two motor
A
and Outcomes regions are spared, motor activity will be normal but the
patient will demonstrate profound indifference, docility,
The natural history of akinetic mutism, when it arises and the loss of motivation to engage in a task. They can be
from a unilateral lesion, is usually a 2-week period of led by the examiner to engage in a task but will fail to self-
gradual improvement from the fully formed syndrome generate sustained directed attention. They lack cognitive
to near-complete recovery presumably enabled by contra- motivation.
lateral limbic activation gaining access to deafferented The role of the anterior cingulate as a cognitive effec-
networks. The outcome from bilateral lesions is usually tor is appreciated within the realm of language. Language,
death, given no ability for cross-hemispheric motivation. a cognitive function, is distinguished from the motor
Thus, prognosis will rely upon neuroimaging document- function of speech. Transcortical motor aphasia
ing the extent of the lesion. (TCMA) is the usual result of left anterior medial or
anterior dorsolateral prefrontal lesions. The classic syn-
drome of TCMA is initial mutism that resolves in days to
Neuropsychology and Psychology weeks, yielding a syndrome featuring delayed initiation of
brief utterances without impaired articulation, excellent
Extracingulate connections support a segregation of the repetition, inappropriate word selection, agrammatism,
cingulate into functional subregions (for a complete dis- and poor comprehension of complex syntax. Activation
cussion of these circuits, Cingulate Gyrus). Paralleling of dorsolateral prefrontal cortices enabling language and
the general distinction between posterior granular sensory speech arises from two sources: the anterior cingulate and
cortices and anterior agranular executive cortices, the the supplementary motor area (with the cingulate skele-
anterior cingulate can be considered an executive region tomotor region). When the executive prefrontal cortex
for affective motivation and cognition, while the posterior (areas 9, 10, and 46) is disrupted, cognitive language
cingulate, with its prominent granular layer IV receiving deficits are prominent (TCMA, type I); when motor
sensory input, is engaged in visuospatial and memory neurons in area 4, devoted to the speech apparatus, are
processing. The interconnections between the anterior disconnected from their activation, speech hesitancy
and posterior cingulate allow for regulatory control by and impoverished output ensues (TCMA, type II).
the anterior executive effector regions over posterior sen- These two functional realms are separable and can be
sory processing and reciprocal modulation of that regu- disconnected anywhere along two pathways. Direct dam-
latory input by the posterior cingulate. age to the supplementary motor area or its outflow to the
Three anterior effector regions include a visceral effec- motor cortex traveling in the anterior superior paraven-
tor region inferior to the genu of the corpus callosum tricular white matter will produce TCMA type II. Direct
encompassing area 25, the anterior subcallosal portions damage to the anterior cingulate, its outflow to areas 9,
of 24ab, and 32; a cognitive effector region that includes 10, and 46, or to the caudate via the subcallosal
most of the supracallosal area 24, and areas 24a0 b0 and fasciculus, just inferior to the frontal horn of the lateral
320 ; and a skeletomotor effector region within the depths of ventricle will disrupt frontal-subcortical circuits
the cingulate sulcus, that includes areas 24c0 /23c on the involved in motivation and executive cognitive function.
ventral bank, with 24c0 g and 6c on the dorsal bank. These The initial muteness has been described by a patient
three cingulate effector regions integrate ascending input after recovery from an anterior cingulate/supplementary
concerning the internal milieu of the organism with motor infarction as a loss of the will to reply to her
visceral motor systems, cognitive-attentional networks, examiners, because she had nothing to say, her mind
and skeletomotor centers to produce the affective motiva- was empty, and nothing mattered (Damasio & Van
tion necessary for the organisms engagement in the Hoesen, 1983).
environment. The loss of will to initiate a motor function results
Circumscribed lesions in humans are rarely confined from supplementary motor or cingulate skeletomotor
to one region of the cingulate. With an anterior lesion, the region damage, while poor initiation of a cognitive pro-
cognitive, skeletomotor, and visceral effector regions are cess results from lesions in supracallosal cingulate areas.
often affected. Bilateral lesions result in an akinetic mute Loss of emotional vigilance ranging from flattened affect
state. The loss of spontaneous motor activity results when to neglect can be produced by surgery in this region.
the lesion involves the supplementary motor area and the Anterior cingulate lesions in monkeys difficult subjects
66 A Akinetic Mutism
in which to evaluate subtle behavioral changes produce (FDG-PET) in frontal cortex has also resulted from
either no observable change or result in a transient pallidal lesions (Laplane, Levasseur, Pillon, Dubois,
stupor with ensuing lethargy, tameness, disturbed intra- Baulac, Mazoyer, et al., 1989) disconnecting their cortical
species social behavior, and decreased pain sensitivity targets. Yet, when pallidal lesions result from carbon
(Pribram & Fulton, 1954). Removal of the anterior cingu- monoxide poisoning, microscopic cortical lesions may
late (areas 24 and 32) in humans (cingulectomy) has been contribute to the functional imaging abnormalities.
employed as a treatment for epilepsy, psychiatric, and Ventral extension of a pallidal lesion appears to discon-
pain disorders. nect the anterior cingulate circuit, in nonhuman primates
The cingulum bundle has also been the site of surgical and humans (Mega & Cohenour, 1997), from limbic
lesions (cingulumotomy when only the bundle is trans- drive. Bilateral paramedian or anterior thalamic lesions
ected, or cingulotomy when cingulate cortex is also (Nagaratnam, Nagaratnam, Ng, & Diu, 2004), caudate
removed) to treat psychiatric and pain disorders. The cin- (Grunsfeld & Login, 2006), or putamen (Ure, Faccio,
gulum contains the efferents and afferents of the cingulate Videla, Caccuri, Giudice, Ollari, et al., 1998) lesions will
to the hippocampus, basal forebrain, amygdala, and all also disrupt the anterior cingulate frontal-subcortical
cortical areas, as well as fibers of passage between hippo- circuit.
campus and prefrontal cortex, and from the median raphe
to the dorsal hippocampus. Surgical ablation of the anterior
portion (sparing fibers relevant to memory function) is
most successful when treating aggression, extreme anxiety,
Evaluation
obsessivecompulsive behaviors, and severe pain. Psychotic
Evaluation of the patient suspected of suffering from
symptoms show only a temporary response. The only pro-
akinetic mutism is to first rule out other causes of possible
spective long-term follow-up of patients undergoing supra-
unresponsiveness. Documenting the response to first ver-
callosal anterior cingulotomy for the treatment of medically
bal stimuli, and then sensory stimuli, will provide evi-
refractory obsessivecompulsive disorder revealed a clear
dence for or against coma. Patients in coma will not
response in 28% and a partial response in 17% (Baer,
respond to internal (e.g., hunger) or external (e.g., pain)
Rauch, Ballantine, Martuza, Cosgrove, Cassem, et al.,
stimuli. All patients who survive the myriad of insults
1995). Including the subcallosal anterior cingulate/medial
producing coma will regain the sleep-wake cycle and will
orbital cortex may provide the best result in treating the
eventually open their eyes spontaneously. They are then
refractory obsessivecompulsive patient (Hay, Sachdev,
described as being in a persistent vegetative state. The
Cumming, Smith, Lee, Kitchener, et al., 1993) due to the
locked-in patient will blink to command and can be
elimination of the visceromotor aspects of the disorder.
taught to use blinking as a form of communication. The
Postsurgical personality changes are subtle after the acute
patient with akinetic mutism will respond to stimuli but
attentional disorder resolves. Although formal cognitive
will not initiate an unprovoked response. When any pa-
testing is unaltered, affect is flattened. Motivation for
tient with limited response is encountered, a brain imag-
previous enjoyments, such as reading, hobbies, and even
ing study is required in their evaluation.
spectator sports, is lost (Tow & Whitty, 1953); subtle
changes that reflect the loss of higher cognitive motivation.
The three anterior cingulate regions, by virtue of the
distinct functional systems they coordinate, are the con- Treatment
duits through which limbic motivation can activate feel-
ing, thought, and movement partial lesions produce Time is the best treatment for unilateral lesions producing
partial aspects of the akinetic mute state depending akinetic mutism since after the acute phase of the lesion
upon their location. (46 weeks) the patients usually recover limbic activation
Subcortical lesions can also produce the fully formed from unaffected regions. When subcortical lesions destroy
syndrome. Carbon monoxide poisoning with resultant ascending dopaminergic fibers in the medial forebrain
apathy and placidity was described in a patient with a bundle, patients may respond to dopaminergic
ventral pallidal lesion who also had hypoperfusion on agonist (Psarros, Zouros, & Coimbra, 2003), or paradoxi-
single photon emission computed tomography (SPECT) cally antagonists of the D2 receptor (Brefel-Courbon
predominately in the cingulate bilaterally (Mori, Yama- et al., 2007) and GABA activation (Spiegel, Casella,
shita, Takauchi, & Kondo, 1996). Hypometabolism on Callender, & Dhadwal, 2008), perhaps due to blocking
18
F-fluorodeoxyglucose positron emission tomography feedback-loop inhibition.
Alcohol Abuse A 67
Cross References A
Alcohol Abuse
Abulia
Amotivation N ATE E WIGMAN
Apathy University of Florida
Cingulate Gyrus Gainesville, FL, USA
Baer, L., Rauch, S. L., Ballantine, H. T., Martuza, R., Cosgrove, R., Alcoholism; Binge drinking; Excessive alcohol use
Cassem, E., et al. (1995). Cingulotomy for intractable obsessive-
compulsive disorder: prospective long-term follow-up of 18
patients. Archives of General Psychiatry, 52, 384392.
Brefel-Courbon, C., Payoux, P., Ory, F., Sommet, A., Slaoui, T., Raboyeau, Short Description or Definition
G., et al. (2007). Clinical and imaging evidence of zolpidem effect in
hypoxic encephalopathy. Annals of Neurology, 62(1), 102105. Alcohol abuse refers to a maladaptive pattern of alcohol
Damasio, A. R., & Van Hoesen, G. W. (1983). Focal lesions of the limbic
[use] leading to clinically significant impairment or
frontal lobe. In K. M. Heilman & P. Satz (Eds.), Neuropsychology of
human emotion (pp. 85110). New York: Guilford. distress. The DSM-IV Criteria for alcohol abuse are
Grunsfeld, A. A., & Login, I. S. (2006). Abulia following penetrating brain
injury during endoscopic sinus surgery with disruption of the ante-
rior cingulate circuit: case report. BMC Neurology, 6, 4.
Hay, P., Sachdev, P., Cumming, S., Smith, J. S., Lee, T., Kitchener, P., et al.
DSM-IV-TR Criteria for Alcohol Abuse
(1993). Treatment of obsessive-compulsive disorder by psychosur-
gery. Acta Psychiatrica Scandinavica, 87, 197207. 1. A maladaptive pattern of alcohol abuse leading to
Laplane, D., Levasseur, M., Pillon, B., Dubois, B., Baulac, M., Mazoyer, B., clinically significant impairment or distress, as mani-
et al. (1989). Obsessive-compulsive and other behavioural changes fested by one or more of the following, occurring
with bilateral basal ganglia lesions. Brain: A Journal of Neurology,
within a 12-month period:
112, 699725.
Mega, M. S., & Cohenour, R. C. (1997). Akinetic mutism: a disconnection Recurrent alcohol use resulting in failure to fulfill
of frontal-subcortical circuits. Neurology, Neuropsychology, and Be-
major role obligations at work, school, or home
havioral Neurology, 10, 254259.
Mega, M. S., & Cummings, J. L. (1994). Frontal subcortical circuits and (e.g., repeated absences or poor work perfor-
neuropsychiatric disorders. Journal of Neuropsychiatry and Clinical mance related to substance use; substance-related
Neurosciences, 6, 358370. absences, suspensions, or expulsions from school;
Mori, E., Yamashita, H., Takauchi, S., & Kondo, K. (1996). Isolated or neglect of children or household).
athymhormia following hypoxic bilateral pallidal lesions. Behavioral
Recurrent alcohol use in situations in which it is
Neurology, 9, 1723.
Nagaratnam, N., Nagaratnam, K., Ng, K., & Diu, P. (2004). Akinetic physically hazardous (e.g., driving an automobile
mutism following stroke. Journal of Clinical Neuroscience: Official or operating a machine).
Journal of the Neurosurgical Society of Australasia, 11(1), 2530. Recurrent alcohol-related legal problems (e.g.,
Pribram, K. H., & Fulton, J. F. (1954). An experimental critique of the arrests for alcohol-related disorderly conduct).
effects of anterior cingulate ablations in monkey. Brain: A Journal of
Continued alcohol use despite persistent or recur-
Neurology, 77, 3444.
Psarros, T., Zouros, A., & Coimbra, C. (2003). Bromocriptine-responsive rent social or interpersonal problems caused or
akinetic mutism following endoscopy for ventricular neurocysticer- exacerbated by the effects of the alcohol (e.g.,
cosis. Case report and review of the literature. Journal of Neurosur- arguments with spouse about consequences of
gery, 99(2), 397401. intoxication or physical fights).
Spiegel, D. R., Casella, D. P., Callender, D. M., & Dhadwal, N. (2008).
Treatment of akinetic mutism with intramuscular olanzapine: a case 2. These symptoms must never have met the criteria for
series. Journal of Neuropsychiatry and Clinical Neurosciences, 20(1), alcohol dependence.
9395.
Tow, P. M., & Whitty, C. W. M. (1953). Personality changes after opera- Although alcohol abuse is diagnosed primarily by ob-
tions of the cingulate gyrus in man. Journal of Neurology, Neurosur- served or reported impairment and distress related to
gery, and Psychiatry, 16, 186193.
alcohol use, the Dietary Guidelines for Americans recom-
Ure, J., Faccio, E., Videla, H., Caccuri, R., Giudice, F., Ollari, J., et al.
(1998). Akinetic mutism: a report of three cases. Acta Neurologica mends no more than one drink per day for women and
Scandinavica, 98(6), 439444. two drinks per day for men (USDA, 2005).
68 A Alcohol Abuse
Overall, the most consistently impaired neuro- Have you ever felt Guilty about drinking?
A
psychological domains include verbal and nonverbal Have you ever felt you needed a drink first thing in the
learning and perceptual-motor skills. More broadly, most morning (Eye-opener) to steady your nerves or to get
reviews conclude that abstraction-executive abilities are rid of a hangover?
impaired among alcohol abusers (Rourke & Grant, 2009).
Other brief assessments for alcohol abuse include the
Despite the consistency of these neuropsychological find-
POSIT and CRAFFT for adolescents (Knight, Sherritt,
ings, many of the samples from these studies are recently
Harris, Gates, & Chang, 2003), the Michigan Alcoholism
detoxified adults. Grant and Adams (2009) point out that
Screen Test (MAST) for adults (Magruder-Habib, Stevens,
neuropsychological recovery typically occurs following
& Alling, 1993), and the AUDIT-C for both adults and
the first year and perhaps more of detoxification.
adolescents (Bush et al., 1998). According to Fiellin, Reid,
Although the exact mechanisms of these neuropsy-
and OConnor (2000), the CAGE and the AUDIT are
chological deficits are not known, some of the major
the superior screening instruments in primary care set-
hypotheses attempting to explain these deficits have
tings compared with other alcohol abuse screeners
been (Chelune & Parker, 1981):
and other clinical methods. The CAGE is superior at
1. Chronic alcohol abuse results in premature aging of detecting diagnosable abuse and dependence and the
the brain. AUDIT is superior at detecting at risk and harmful drink-
2. Chronic alcohol abuse leads to global generalized CNS ing (Fiellin et al., 2000).
dysfunction.
3. Chronic alcohol abuse differentially disrupts the right
hemisphere of the brain.
4. Chronic alcohol abuse exerts its detrimental effect on Treatment
the anterior-basal regions of the brain.
5. Chronic alcohol abuse produces a generalized CNS Treatment ranges from support groups to rehabilitation
impairment that is particularly disruptive of the centers. Treatments of alcohol abuse appear to be largely
fronto-parietal association areas of the brain. psychosocial. In a systematic review, brief psychosocial
interventions among primary care patients were found
More recent neural hypotheses of the mechanisms of to be effective at reducing alcohol consumption (Kaner
neuropsychological deficits include reduced regional et al., 2007). Although well-known support groups such
blood flow to the frontal lobes, reduction in metabolites as Alcoholic Anonymous (AA) have been helpful to many
(e.g., NAA) that indicate lack of neuronal integrity, fron- people and likely constitute the most accessible form of
tal-striatal and cerebellar dysfunction manifesting as loss treatment, evidence has not supported AAs effectiveness
of dendritic arbor (Rourke & Grant, 2009). Grant and at reducing alcohol problems (Ferri, Amato, & Davoli,
Adams (2009) note that molecular mechanisms of the 2006). Medical treatments of alcohol abuse focus on
influence of chronic alcohol abuse on neuropsychological reducing craving. Naltrexone (Chick et al., 2000) and
functioning are largely unknown. Acomprosate (Garbutt, West, Carey, Lohr, & Crews,
1999) have been found to be effective at reducing craving.
Evaluation However, most medications are aimed at dependence, not
abuse symptoms.
A common screening tool for alcohol abuse is the CAGE
questionnaire (Ewing, 1984; see An even briefer CAGE
Questionnaire, Table B).The CAGE is highly effective
Cross References
at identifying problem drinkers among adults (Bernadt,
1982). Two yes responses on the CAGE indicate that the
Alcohol Brain Syndrome
respondent should be investigated further. The question-
Alcohol Dependence
naire asks the following questions:
Blood Alcohol Level
Have you ever felt you needed to Cut down on your Fetal Alcohol Syndrome
drinking? Michigan Alcoholism Screen Test
Have people Annoyed you by criticizing your Substance Abuse
drinking? Wernicke-Korsakoff s Syndrome
70 A Alcohol Addiction
References and Readings from the 2001 national household survey on drug abuse: Volume I.
Summary of national findings (Office of Applied Studies, NHSDA
Series H-17 ed.) (BKD461, SMA 023758). Washington, DC: U.S.
Bernadt, M. W. (1982). Comparison of questionnaire and laboratory tests
Government Printing Office. Retrieved March 14, 2009, from the
in the detection of excessive drinking and alcoholism. Lancet, 6,
World Wide Web: http://www.oas.samhsa.gov/nhsda/2k1nhsda/
325328.
vol1/Chapter3.htm
Chelune, G. J., & Parker, J. B. (1981). Neuropsychological deficits asso-
United States Department of Agriculture and United States Department
ciated with chronic alcohol abuse. Clinical Psychology Review, 1,
of Health and Human Services (USDA). (2005). Dietary guidelines for
181195.
Americans: Chap. 9 Alcoholic beverages (pp. 4346). Washington,
Chick, J., Anton, R., Checinski, K., Croop, R., Drummond, D. C.,
DC: US Government Printing Office.
Farmer, R. et al. (2000). A multicentre, randomized, double-blind,
Vaillant, G. E. (1995). The natural history of alcoholism revisited.
placebo-controlled trial of naltrexone in the treatment of alcohol
Cambridge, MA: Harvard University Press.
dependence or abuse. Alcohol, 35, 587593.
Ewing, J. A. (1984). Detecting alcoholism: The CAGE questionnaire.
JAMA, 252, 19051907.
Ferri, M. M. F., Amato L., & Davoli M. (2006). Alcoholics anonymous
and other 12-step programmes for alcohol dependence. Cochrane
Database of Systematic Reviews, Issue 3, Art. No.: CD005032. doi:
Alcohol Addiction
10.1002/14651858.CD005032.pub2
Fiellin, D. A., Reid, M. C., & OConnor, P. G. (2000). Screening for Alcoholism
alcohol problems in primary care: A systematic review. Archives of
Internal Medicine, 160(13), 19771989.
Garbutt, J. C., West, S. L., Carey, T. S., Lohr, K. N., & Crews, F. T. (1999).
Pharmacological treatment of alcohol dependence: A review of the
evidence. JAMA, 281, 13181325. Alcohol Amnesic Disorder
Grant, B. F., Dawson, D. A., Stinson, F. S., Chou, S. P., Dufour, M. C., &
Pickering, R. P. (2004). The 12-month prevalence and trends in DSM-IV Korsakoff s Syndrome
alcohol abuse and dependence: United States, 19911992 and 20012002.
Drug and Alcohol Dependence, 74, 223234.
Grant, I., Adams K. M. (Eds.) (2009). Neuropsychological assessment
of neuropsychiatric disorders (3rd ed., pp. 127158.). New York:
Oxford University Press.
Grant, B. F., Stinson, F. S., & Harford, T. C. (2001). Age at onset of alcohol
Alcohol Dependence
use and DSM-IV alcohol abuse and dependence: A 12-year follow-
up. Journal of Substance Abuse, 13, 493504. G LENN S. A SHKANAZI
Hasin, D. S., Van Rossem, R., McCloud, S., & Endicott, J. (1997). Alcohol University of Florida-College of Public Health and Health
dependence and abuse diagnoses: Validity in a community sample Professions
of heavy drinkers. Alcoholism, Clinical and Experimental Research, Gainesville, FL, USA
21, 213219.
Hawkins, J. D., Catalano, R. F., & Miller, J. Y. (1992). Risk and protective
factors for alcohol and other drug problems in adolescence and early
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Knight, J. R., Sherritt, L., Harris, S. K., Gates, E. C., & Chang, G. (2003). Alcoholism
Validity of brief alcohol screening tests among adolescents: A com-
parison of the AUDIT, POSIT, CAGE, and CRAFFT. Alcoholism,
Clinical and Experimental Research, 27, 6773.
Magruder-Habib, K., Stevens, H. A., & Alling, W. C. (1993). Relative Definition
performance of the MAST, VAST, and CAGE versus DSM-III-R
criteria for alcohol dependence. Journal of Clinical Epidemiology, As described in DSM-IV, alcohol dependence is a set of
46, 435441.
symptoms encompassing dysfunction in cognitive, behav-
Regier, D. A., Farmer, M. E., Rae, D. S., Locke, B. Z., Keith, S. J.,
Judd, L. L., et al. (1990). Comorbidity of mental disorders with
ioral, and physiological domains caused by continued
alcohol and other drug abuse. Results from the epidemiologic catch- alcohol use. A pattern of repeated alcohol ingestion exists,
ment area (ECA) study. JAMA, 264, 25112518. resulting in increasing amounts consumed in order to
Rourke, S. B., & Grant, I. (2009). The neurobehavioral correlates of obtain the desired effect (i.e., tolerance) and characteristic
alcoholism. In I. Grant & K. M. Adams (Eds.), Neuropsychological
symptoms if use is suddenly suspended (i.e., withdrawal).
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pp. 398454). New York: Oxford University Press.
There is a perceived loss of control over drinking, exhibited
U.S. Department of Health and Human Services. Substance Abuse and by repeated failed attempts to decrease or quit drinking.
Mental Health Services Administration(US DHHS). (2002). Results Individuals may spend increasing amounts of time
Alcoholic Brain Syndrome A 71
in drinking-related behaviors without being able to stop, alcohol, resulting in significant impairment on normal
A
despite being aware that drinking is causing, or exacerbat- brain functioning (APA Dictionary of Psychology, 2007).
ing, psychological or medical problems. Cognitive conse-
quences can include memory loss, difficulty performing
familiar tasks, poor or impaired judgment, and problems Categorization
with language.
As mentioned in the definition, alcoholic brain syndrome
encompasses several syndromes.
Cross References
1. Alcohol withdrawal delirium: A reversible condition
Alcohol Abuse that develops after cessation of chronic, extreme
Alcohol Dementia alcohol intake. Symptoms include disturbed con-
Alcoholic Brain Syndrome sciousness (e.g., disruption in attention/concentration),
Substance Abuse disruption in memory, orientation, and language be-
Substance Abuse Disorders yond what would be expected from typical alcohol
WernickeKorsakoff Syndrome withdrawal.
2. Alcohol-induced persisting dementia: A chronic condi-
tion that includes multiple cognitive deficits as
References and Readings a result of prolonged alcohol abuse. Cognitive areas
generally impaired include memory, speech, motor/
American Psychiatric Association (1994). Diagnostic and statistical man- sensory functions and executive functions. Global
ual of mental disorders (4th ed.). Washington, DC: American Psychi- impairment in intellectual functioning evolves grad-
atric Association.
ually over time.
3. Alcohol-induced persisting amnestic disorder: A persis-
tent disturbance in memory functioning caused by
chronic alcohol abuse. Memory impairment is severe
Alcoholic Amnestic Disorder enough to cause significant disturbance in occupa-
tional or social functioning.
Wernicke-Korsakoff Syndrome 4. Wernickes encephalopathy (WE): A syndrome resulting
from chronic alcoholism leading to nutritional defi-
ciency (i.e., Vitamin B1 [Thiamine] and characterized
by acute confusion, ataxia, sluggish pupillary reflexes,
Alcoholic Brain Syndrome and nystagmus and memory deficits). The syndrome
can result in coma or death. Lesions are centered in the
G LENN S. A SHKANAZI midbrain, cerebellum, and diencephalon.
University of Florida-College of Public Health 5. Korsakoff s syndrome: This condition often follows
and Health Professions episodes of WE. Thiamine deficiency, as a result of
Gainesville, FL, USA chronic, severe alcohol abuse, leads to a dense antero-
grade and retrograde amnesia. Patients with Korsak-
off s syndrome can store information for only a few
Synonyms seconds before they forget it. The resulting amnesia is
thought to be due to damage in the mammillary bod-
Alcoholic dementia; Alcoholic hallucinosis; Delirium ies, anterior or dorsomedial nuclei (or both) of the
tremens; Korsakoff s syndrome; WernickeKorsakoff thalamus.
syndrome
Another common feature is confabulation, in which
the patient recounts detailed and convincing memories
for events that have never happened.
Short Description or Definition
6. Alcohol-induced psychotic disorder: A condition involv-
Alcoholic brain syndrome is a collection of several syn- ing the presence of delusions and/or hallucinations
dromes associated with the acute or chronic use of due to the physiological effects of alcohol.
72 A Alcoholic Brain Syndrome
Treatment A
Alcoholic Dementia
The primary treatment option for patients experiencing
alcoholic brain syndrome is to stop drinking and remain Alcoholic Brain Syndrome
abstinent. Without additional alcohol exposure, the re-
covery from the delirium caused by alcohol is usually
good. This is obviously the first treatment to be utilized.
As mentioned above, thiamine deficiency is an important Alcoholic Hallucinosis
contributor to alcohol-related brain damage; therefore,
Vitamin B1 supplementation is necessary. Initially, the Alcoholic Brain Syndrome
vitamins can be given intravenously or intramuscularly
followed by oral administration. WE responds well to
high-dose vitamins, and such treatment can prevent the
occurrence of severe, chronic Korsakoffs syndrome. Sec- Alcoholic Polyneuropathy
ondarily, nutritional counseling to promote a vitamin-rich
and balanced diet is also part of this initial treatment pro- Korsakoff s Syndrome
tocol, especially for longer-term recovery and prevention.
Cross References
Alcoholic Psychosis
Alcoholism
Amnesia Korsakoff s Syndrome
Anterograde Amnesia
Dementia
Encephalopathy
Episodic Memory Alcoholism
Korsakoff s Syndrome
Organic Brain Syndrome G LENN S. A SHKANAZI
Retrograde Amnesia University of Florida-College of Public Health and Health
Semantic Memory Professions
Substance Abuse Gainesville, FL, USA
addiction and physiological withdrawal mechanisms, reported being current drinkers of alcohol (approximately
while for others, these are consequences of drinking. 109 million). That number drops to 44% when the age is
It is common for laypeople to equate any kind of 18 or older. Approximately 20% of persons aged 12 or older
excessive drinking with alcoholism. Those in the mental participated in binge drinking at least once in 30 days prior
health fields see that disorders related to alcohol use lie to the 2001 survey. Heavy drinking was reported by 5.7%
along a continuum of severity that may include physical of the 12 or older population (12.9 million). The highest
dependency/withdrawal (i.e., alcohol dependence) or may prevalence for both binge and heavy drinking was for those
involve impaired drinking habits that lead to health or in the 1825 age groups with the peak rate occurring at age
social problems/consequences but without dependency/ 21. Studies have found those who begin drinking at an
withdrawal (i.e., alcohol abuse). According to the APA earlier age are at higher risk to develop dependency. Those
Dictionary of Psychology, alcoholism is the popular term Americans who wait till age 21 are 4 times less likely to
for alcohol dependence. become dependent than those who begin drinking before
the age of 15 (i.e., 40% who start before age 15 develop
dependency on alcohol at some point in their lives). The
Historical Background
risk for developing dependency declines with age, as the
prevalence rate for alcoholism in those persons greater than
The term alcoholism was first used in 1849 by a physi-
65 years old is 3%.
cian, Magus Haas, to describe the systematic adverse
There are other nonage risk factors as well. Those with
effects of alcohol overconsumption. In the USA, it became
lower education and lower socioeconomic status are also
a popular term in the 1930s as a result of the growth of
at higher risk. There are also gender differences as men are
Alcoholics Anonymous (AA). Previously, society viewed
at minimum 2.5 times more likely to be defined as alco-
those who drank to excess as immoral, weak of character,
holic as women; however, the proportion of female alco-
and irresponsible. Societys response was punishment and
holics is increasing. White, non-Hispanic, individuals
removal of overconsumers from sober society to protect
are more likely to develop alcoholism than African-
the community. With the rise of AA, and their publication
Americans. The risk for Hispanics is generally the same
(i.e., the Big Book), the view of alcoholism changed
as Whites.
from character flaw to medical disease. AA viewed alco-
Alcoholism is estimated to be the third leading cause
holism as a physical allergy to alcohol accompanied by
of preventable death in the USA (after smoking and obe-
an obsession with drinking. This organization began to
sity). In the USA, 85,000 deaths are attributable to alcohol
dispel the previously held beliefs that alcoholics were
each year at a cost of $185 billion. The NIAAA estimates
unemployable, destitute, and isolated individuals by
that intoxication is present in 3060% of homicides, 22%
demonstrating that some highly respected people who
of suicides, 3350% of automobile accidents, 67% of
had been alcohol dependent had eventually overcome
drownings, and 7080% of fire-related deaths. More
their disorder and went on to lead productive lives.
than 50% of American adults have a close family member
who has or has had alcoholism. Approximately one in four
Epidemiology children younger than 18 in the USA is exposed to alcohol
abuse or alcohol dependence in their family.
The epidemiology of alcoholism can be confusing and Internationally, the World Health Organization esti-
contradictory, depending on the definition being utilized mates that there are 140 million people worldwide that are
and the measurement tool. The generally accepted overall alcohol dependent and they account for 3.5% of the total
rate of occurrence of alcoholism in the USA is 10%. The cases of disease worldwide, which is a higher rate than
U.S. National Longitudinal Alcohol Epidemiologic Study tobacco or illicit drugs.
concluded that alcoholism is prevalent in 20% of adult
hospital inpatients and in 17% of community-based
primary care practices. A 1985 U.S. National Hospital Current Knowledge
Survey found that 528,000 patients were discharged from
hospitals with a primary diagnosis of substance abuse, and Causes
for 81% (428,000), alcohol was the abused substance.
According to a 2001 survey conducted by the National There is no identifiable single cause of alcoholism. Scien-
Institute on Alcohol Abuse and Alcoholism (NIAAA) in tists believe that a myriad of factors play a role in the
the USA, approximately 48% of adults (aged 12 or older) development of alcoholism.
Alcoholism A 75
1. Genetics: Previous twin and adoption studies have more likely to be alcohol dependent. It is not an adequate
A
demonstrated that genes play an important role in measure by itself but can alert a health-care provider to
the development of alcoholism. Researchers found probe further. Another weakness is that it tends to be less
that identical twins (i.e., identical genes) have a higher reliable with populations with lower alcoholism rates
concordance rate for drinking behavior than fraternal (e.g., elderly) and does not identify hazardous drinking.
twins. Other studies have cast some doubt on these
2. Alcohol Use Disorders Identification Test (AUDIT):
twin studies by suggesting the environment of identi-
The AUDIT can detect both hazardous drinking
cal twins is more alike than fraternal twins, thus sug-
and alcohol abuse. It does not need to be administered
gesting a weakening of the argument in favor of genes.
face to face like the CAGE. It was developed by the
In the adoption studies, researchers found that World Health Organization and yields scores for
whether reared by biologic or adoptive parents, the sons consumption, dependency, and alcohol-related
of males with alcohol problems are 4 times more likely problems.
to have alcohol problems than sons of persons who are 3. Alcohol Dependence Data Questionnaire: More sensi-
not. In either case, epidemiologic studies indicate that tive than the CAGE and can distinguish abuse versus
alcoholism tends to run in families. Alcoholics are 6 times dependence.
more likely than nonalcoholics to have blood relatives
who are alcohol dependent. In summary, a persons
genetic makeup can predispose them to alcoholism Diagnosis
or not.
2. Peer influence: Social networks that include heavy Health-care providers most often use the Diagnostic Sta-
drinkers and alcohol abusers increase an individuals tistical Manual of the Mental Disorders, Fourth Edition,
risk for alcoholism. Text Revision (DSM-IV-TR) criterion for alcohol depen-
3. Cultural influence: Cultures that include well-estab- dence. The diagnosis requires three of the following
lished taboos against drunkenness and rules regarding criteria:
drinking have lower alcoholism rates than those who 1. Maladaptive pattern of the use leading to impairment/
do not. distress as manifested by three or more of the below
4. Psychiatric conditions: Certain psychiatric diagnoses occurring in the same 12-month period.
increase the risk of alcoholism. These include Tolerance
ADHD, panic disorder, schizophrenia, and antisocial Withdrawal
personality disorder. Drink more frequently or in larger amounts than
intended
Persistent desire to drink or unsuccessful efforts to
Screening cut down or control use
Great deal of time spent in acquiring/using alcohol
There are a variety of measures for alcoholism including or recovering from its effects
the following: Important social, occupational, or recreational
activities given up or reduced because of alcohol
1. CAGE: The CAGE is named for the four questions Drinking continues despite knowledge of persis-
asked of a patient before any questions regarding tent or recurrent physiological, or psychological
quantities drank are asked. problems caused or exacerbated by drinking
a Have you ever felt the need to Cut down on your
drinking?
b Have people Annoyed you by criticizing your
Treatment
drinking?
c Have you ever felt Guilty about drinking?
There are several well-accepted avenues of treatment.
d Have you ever felt you needed a drink in the morn-
ing to steady your nerves or get rid of a hangover? 1. Psychosocial: Studies have shown that simple, brief
(Eye-opener) interventions can be effective in those not severely
The CAGE has been extensively validated. Those who alcohol dependent. One of those getting an extensive
answer YES to two or more questions are 7 times trial has been Motivation Interviewing based on
76 A Alcoholism
Prochaskas Five Stages of Change Model. A summary afford a private hospital or private psychiatrist could
of the treatment approach is as follows: only find help in state hospitals, jails, or churches.
Precontemplation Patient expresses no interest or AA was the first self-directed approach toward treat-
need for change. The health-care professionals ment. The AA treatment model includes self-help
options are limited. They can point to discrepan- groups, utilizing psychological principles organized
cies between the patients goals and behavior and in small local community groups. The 12 steps of
recommend 2 weeks of abstinence. AA encourage confrontation of denial, admission of
Contemplation Patient expresses ambivalence or powerlessness over alcohol, and strives for people
skepticism about change. The provider should to atone for harm caused by their behavior while
work to influence them in direction of change, drinking. It encourages its members to live ethically
provide information about the dangers of alcohol with a reliance on a higher power. It is this sense
abuse, and recommend an abstinence trial. of AA as a religion that has led to nonreligious self-
Preparation Patient accepts need for change and help groups including rational recovery, LifeRing,
makes plans to accomplish changed drinking goal. and SOS.
Action Patient recognizes problem in drinking
behavior and takes observable steps to decrease
alcohol use. Professional reinforces decision for
change and may introduce self-help groups and/
Future Directions
or medications.
The following are areas needing continued study:
Maintenance Patient and professional work
together to maintain change and prevent relapse. 1. Genetic research current and future studies are look-
ing at individuals with a family history of alcoholism
to pinpoint the location of genes that influence vul-
2. Medications: The most common medications in the nerability to alcoholism. This line of study will assist
treatment of alcoholism are: in the early identification of individuals at risk and of
Disulfiram (Antabuse) Prevents the elimination new, gene-based treatment approaches.
of acetaldehyde, which is a by-product of alcohol 2. Treatment approaches The NIAAA has been funding
metabolism. Results in unpleasant side effects in a study called Project MATCH whose goal is to
persons still drinking including nausea, dizziness, identify variables important in predicting outcome
headache, flushing, vomiting, heart palpitations, based on patient characteristics and treatment design.
and sudden drop in blood pressure. Disulfiram 3. Medications Naltrexone was the first drug approved
needs to be taken daily to be effective. However, by the FDA in 45 years to help alcoholics stay sober
in at least one large clinical trial it did not increase following detoxification. More research is needed.
abstinence.
Naltrexone (ReVia) May work by blocking the
positive effects felt from drinking by blocking opi- Cross References
ate receptors in the brain thereby decreasing crav-
ing for alcohol. Clinical studies have found a Alcoholic Brain Syndrome
modest decrease in relapse (1220%). This drug Fetal Alcohol Syndrome
has an unknown cause of action. Korsakoff s Syndrome
Acamprosate (Campral) Used to maintain Michigan Alcoholism Screening Test
abstinence once alcoholics have stopped drinking. Motivational Interviewing
Thought to work by stabilizing the chemical Substance Abuse Disorders
balance in the brain. In clinical trials, the one Twin Studies
year abstinence rates have been 18% and 12% at WernickeKorsakoff s Syndrome
two years.
niaaa.nih.gov/publications/social/module2etiology&naturalhistory/
module2.html). Alexia A
National Institute on Alcohol Abuse and Alcoholism. Helping patients
who drink too much: A clinicians guide. URL Accessed on June 1,
S TEVEN Z. R APCSAK , P ELAGIE M. B EESON
2009 (http://www.niaaa.nih.gov/Publications/EducationTraining-
Materials/guide.htm). The University of Arizona
Schuckit, M. (2000). Drug and alcohol abuse: A clinical guide to Tucson, AZ, USA
diagnosis and treatment. New York, NY: Kluwer Academic/Plenum
Publishers.
U.S. Department of Health and Human Services and SAMHSAs National
Clearinghouse for Alcohol and Drug Information. Accessed URL
Short Description or Definition
on June 1, 2009 (http://ncadistore.samhsa.gov/catalog/facts.aspx?
topic=3). The term alexia is applied to acquired disorders of reading
produced by neurological injury in individuals with
normal premorbid literacy skills. Clinically, patients with
alexia have difficulty in recognizing, pronouncing, or
comprehending written words. Although alexia can
occur in relative isolation, it is more frequently encoun-
Alertness tered in the context of spoken language dysfunction or
aphasia. Most individuals with alexia have concommitant
C HRIS L OFTIS spelling impairment or agraphia, suggesting that reading
STG International and spelling rely on shared cognitive representations
Alexandria, VA, USA and neural substrates. Acquired alexia needs to be
distinguished from developmental dyslexia reflecting a
failure to attain normal reading skills.
Synonyms
Semantics
Phonology Orthography
Lexical
Words Words
Spoken word Written word
Sublexical
Auditory Visual
Phonemes Letters
analysis analysis
Speech Writing
Alexia. Figure 1 A cognitive model indicating the component processes involved in reading
the retrieval of the corresponding spoken word forms written word comprehension. However, whether semantic
from the phonological lexicon. The lexical route is mediation is also normally required for accurate oral
normally used to read familiar words and can support reading of familiar words is a topic of controversy
the processing of both regular words that have predictable (Coltheart et al., 2001; Plaut, McClelland, Seidenberg, &
spellingsound relationships (e.g., spring) and irregular Patterson, 1996; Woollams, Lambon Ralph, Plaut, &
words that contain atypical lettersound or grapheme Patterson, 2007). In summary, skilled reading depends
phoneme mappings (e.g., choir). By contrast, the sublex- on interactions between visual/orthographic processing,
ical route operates on units smaller than the whole word phonology, and semantics. Damage to these functional
and is thought to rely on the serial conversion of individ- domains or the disruption of the transfer of information
ual graphemes to the corresponding phonemes. The between the cognitive/brain systems that support these
sublexical route is essential for accurate reading of unfa- operations results in alexia.
miliar words or nonwords (e.g., nace) because these novel
items, by definition, do not have preexisting representa-
tions in the orthographic or phonological lexicon. The Epidemiology
sublexical route can also be used to generate plausible
pronunciations for regular words that strictly obey Alexia is commonly observed in right-handed individuals
spellingsound conversion rules. However, processing following damage to the language-dominant left hemi-
irregular words by the sublexical procedure results in sphere. Although it is most frequently caused by stroke,
regularization errors (e.g., have read to rhyme with alexia can follow any kind of focal injury (e.g., trauma,
save). Thus, according to dual-route theory, only the tumor) to the brain regions critical for implementing the
lexical reading route can deliver a correct response to various cognitive operations necessary for normal
irregular words. Note that the model depicted in Fig. 1 reading. Alexia is also often seen in the setting of neuro-
also includes an indirect route from orthography to pho- degenerative disorders, especially in patients with primary
nology via the semantic system. The activation of word progressive aphasia/semantic dementia or Alzheimers
meanings by this semantic reading route is critical for disease. In general, the specific alexia profile is determined
Alexia A 79
not so much by the etiology of the brain damage than by orthographic word forms and result in an abnormal
A
the location of the responsible lesions. word length effect in oral reading.
Pure alexia/letter-by-letter reading is most commonly
seen following left inferior occipito-temporal damage
Natural History, Prognostic Factors,
caused by posterior cerebral artery strokes. It has been
Outcomes
proposed that the critical lesions degrade or disrupt visual
input to the visual word-form area (VWFA) or directly
The prognosis for recovery from alexia depends both on
damage the VWFA itself (Cohen et al., 2003; Epelbaum
the etiology of the lesion and the extent of the underlying
et al., 2008). The VWFA is consistently activated in func-
brain damage. Alexia following stroke tends to show some
tional imaging studies of reading in normal individuals
spontaneous recovery over time, but patients with exten-
and has been localized to the mid-lateral portions of the
sive brain damage may never regain useful reading func-
left fusiform gyrus (BA37) (Cohen et al., 2002; Jobard,
tion and typically stop reading for pleasure. In individuals
Crivello, & Tzourio-Mazoyer, 2003) (Fig. 2). The VWFA
with neurodegenerative disorders, progressive worsening
receives converging input from bilateral posterior occipi-
of the reading impairment is observed along with the
tal areas (BA17,18/19) involved in visual feature analysis
gradual deterioration of other language and cognitive
and letter shape detection and it integrates this informa-
functions.
tion into larger perceptual units corresponding to whole
words (Fig. 2). Activation of the VWFA is sensitive to the
Neuropsychology and Psychology of orthographic familiarity of the letter string, consistent
Alexia with the notion that this cortical region may constitute
the neural substrate of the orthographic lexicon. The
Pure Alexia/Letter-By-Letter Reading orthographic codes computed by the VWFA are subse-
quently transmitted to cortical systems involved in the
In pure alexia, the rapid visual identification of familiar phonological and semantic components of reading
words that characterizes normal skilled reading is dis- (Fig. 3). Importantly, it has been shown that spelling
rupted. Reading is slow and laborious, often relying on a familiar words also activates the VWFA (Beeson et al.,
serial letter-naming strategy known as letter-by-letter 2003). These observations confirm the central role for the
reading. Typically, there is a monotonic increase in VWFA in orthographic processing and support the view
reading latencies as a function of the number of letters that the same orthographic lexical representations medi-
in the word, giving rise to an abnormal word length effect ate reading and spelling. Consistent with this hypothesis,
that is considered the hallmark feature of the syndrome. patients with damage to the VWFA are likely to show
Varying degrees of letter identification difficulty are pres- evidence of reading and spelling impairment attributable
ent and visual reading errors are common (e.g., chain to the loss of word-specific orthographic representations
charm). Collectively, these behavioral observations sug- (Rapcsak & Beeson, 2004).
gest that visual processing impairment plays a critical role
in the pathogenesis of pure alexia (Behrmann, Plaut, &
Nelson, 1998). Although the reading disorder may be Phonological/Deep Alexia
unaccompanied by significant aphasia or agraphia, many
patients with pure alexia demonstrate concommitant Phonological alexia is characterized by a disproportionate
anomia and spelling impairment (Rapcsak & Beeson, difficulty in processing nonwords compared with familiar
2004). Furthermore, patients often perform poorly on words, giving rise to an exaggerated lexicality effect in
nonreading tasks that require fine-grained visual discrim- reading (Crisp & Lambon Ralph, 2006; Patterson &
ination, suggesting that the reading impairment is part of Lambon Ralph, 1999; Rapcsak et al., 2009). Attempts to
a more general visual processing deficit (Behrmann et al., read nonwords often result in real word responses known
1998). Within the framework of the cognitive model as lexicalization errors (e.g., nace name). Although
depicted in Fig. 1, pure alexia is attributable to dysfunc- in phonological alexia reading of familiar words
tion at the visual feature analysis and/or letter identifica- (both regular and irregular) is relatively preserved,
tion stages of reading, or it may be produced by damage to performance is typically influenced by lexical-semantic
the orthographic lexicon. Damage to any of these visual variables including word frequency (high>low), image-
processing components would be expected to interfere ability (concrete>abstract), and grammatical class
with the rapid perceptual identification of familiar (nouns>verbs > functors). Deep alexia includes all the
80 A Alexia
Alexia. Figure 2 Location of the visual word form area (VWFA) (indicated by green circle) as determined by functional
neuroimaging studies of reading. This region receives input from bilateral posterior occipital visual areas (shown in purple).
Arrow indicates callosal transfer of information initially processed by right visual cortex
Phonology
Visual analysis
Semantics
Orthography
characteristic features of phonological alexia, but it insula, superior temporal gyrus/Wernickes area (BA22),
A
is distinguished from the latter by the production of and supramarginal gyrus (BA40) (Rapcsak et al., 2009).
prominent semantic reading errors (e.g., boy son) Consistent with the phonological deficit hypothesis, there
(Coltheart, Patterson, & Marshall, 1980). Although pho- is an excellent neuroanatomical correspondence between
nological and deep alexia were originally considered sepa- the location of the lesions that produce phonological
rate entities, there is now much evidence to suggest that alexia and the location of the perisylvian cortical areas
the difference between these syndromes is quantitative that show activation in normal individuals during a
rather than qualitative. Thus, phonological and deep alex- variety of written and spoken language tasks requiring
ia are more appropriately considered as points along a phonological processing (Jobard et al., 2003; Rapcsak
continuum, with the latter representing a more severe et al., 2009; Vigneau et al., 2006). As predicted by the
version of the former (Crisp & Lambon Ralph, 2006; continuum model, there is considerable overlap between
Rapcsak et al., 2009). the perisylvian lesion profiles of patients with phono-
Phonological alexia is typically encountered in logical and deep alexia, although the damage in deep
patients with aphasia syndromes characterized by phono- alexia tends to be more extensive. In fact, the massive
logical impairment (i.e., Brocas, conduction, Wernickes). destruction of left-hemisphere language areas in deep
Furthermore, it has been shown that most patients with alexia has lead to the hypothesis that reading perfor-
phonological alexia demonstrate prominent deficits and mance in these patients may be mediated by the intact
increased lexicality effects in spoken language tasks that right hemisphere (Coltheart et al., 1980).
require the manipulation and maintenance of sublexical
phonological information (e.g., repetition, rhyme judg-
ments, phoneme segmentation and blending), and also Surface Alexia
that such non-orthographic measures of phonological
ability correlate with and are predictive of reading perfor- In surface alexia the main difficulty involves reading
mance (Crisp & Lambon Ralph, 2006; Rapcsak et al., irregular words, especially when these items are of low
2009). These observations suggest that the written and frequency. Regular words of comparable frequency are
spoken language impairments in phonological alexia have processed more efficiently, and the discrepancy in perfor-
a common origin and are merely different manifestations mance between words with predictable versus atypical
of a central or modality-independent phonological deficit spellingsound relationships is reflected by an increased
(Crisp & Lambon Ralph, 2006; Patterson & Lambon regularity effect in reading. Nonword reading is typically
Ralph, 1999; Rapcsak et al., 2009). Consistent with this preserved. According to dual-route theory, surface alexia
view, the reading disorder in phonological alexia is usually is attributable to dysfunction of the lexical reading route
accompanied by a qualitatively similar spelling im- (Fig. 1). Specifically, it has been suggested that the reading
pairment (phonological agraphia) (Rapcsak et al., 2009). disorder in some cases may result from damage to the
According to dual-route models (Fig. 1), poor nonword orthographic lexicon (Coltheart et al., 2001; Patterson,
reading in phonological alexia is attributable to damage to Marshall, & Coltheart, 1985). Due to the loss of word-
the sublexical route, while the relatively preserved real specific orthographic knowledge, patients with this type
word reading performance of these patients reflects the of deficit will be forced to rely on a sublexical grapheme
residual functional capacity of the lexical and semantic phoneme conversion strategy that produces phonologi-
routes. The general phonological impairment observed in cally plausible regularization errors on irregular words.
the vast majority of patients suggests that the most com- Low-frequency irregular words are especially vulnerable
mon site of damage may be at the level of the phoneme because the activation of representations in the ortho-
units (with additional damage to the phonological lexicon graphic lexicon is normally modulated by word frequency
in more severe cases), as these phonological processing and the relative refractoriness of low-frequency items may
components are shared between written and spoken be further exaggerated by the brain damage. Consistent
language tasks. with the notion that reading and spelling rely on shared
Phonological alexia is most often associated with orthographic representations, patients with surface alexia
damage to a network of perisylvian cortical regions following damage to the orthographic lexicon show
involved in speech production/perception and phonolog- similar difficulty in spelling irregular words (surface
ical processing in general. Components of this distributed agraphia) (Patterson et al., 1985; Rapcsak & Beeson,
phonological system include posterior-inferior frontal 2004). Alternatively, surface alexia may result from dam-
gyrus/Brocas area (BA44/45), precentral gyrus (BA4/6), age to central semantic representations (Woollams et al.,
82 A Alexia
2007). Specifically, it has been proposed that accurate oral may reflect damage to the orthographic lexicon resulting
reading of low-frequency irregular words normally in the loss of word-specific orthographic knowledge. By
requires additional support from the semantic reading contrast, in patients with anterior temporal lobe lesions,
route and cannot be mediated efficiently by pathways and possibly also in patients with posterior temporo-
that rely on direct transcoding between orthographic parietal damage, surface alexia may be attributable to
and phonological representations (Plaut et al., 1996). the degradation of central semantic representations. The
With the degradation of semantic knowledge, the relative latter hypothesis is supported by functional imaging stud-
inadequacy of non-semantic reading routes is revealed ies of semantic processing in normal individuals that have
and manifests itself as surface alexia. Consistent with the shown activation of a large-scale left-hemisphere extra-
semantic deficit hypothesis, many patients with surface sylvian cortical network that included both anterior tem-
alexia perform poorly on verbal and nonverbal cognitive poral lobe and posterior temporo-parietal sites (Vigneau
tasks requiring semantic processing (e.g., picture naming, et al., 2006; Binder, Desai, Graves, & Conant, 2009)
verbal fluency, spoken word and picture comprehension). (Fig. 3).
Furthermore, the severity of the semantic impairment on
these nonreading tasks has been shown to correlate with
reading accuracy for low-frequency irregular words Evaluation
(Woollams et al., 2007). The proposed central semantic
deficit may also explain the frequent co-occurrence of In evaluating patients with alexia it is important to assess
surface alexia and surface agraphia (Graham, Patterson, the status of all the relevant component processes
& Hodges, 2000). involved in reading (Fig. 1). A comprehensive battery
In contrast to the strong association between perisyl- should include tests of letter and word recognition, as
vian damage and phonological alexia, surface alexia is well as measures of oral reading and reading comprehen-
typically encountered in the setting of extrasylvian brain sion. The evaluation should allow the clinician to identify
pathology. Although uncommon in patients with stroke, the nature of the functional impairment and to locate the
surface alexia has been described in individuals with left level of breakdown with reference to a cognitive model of
temporo-parietal lesions centered on posterior middle/ normal reading. It is equally important to document
inferior temporal gyrus and angular gyrus (BA20/21,37/ relatively spared reading abilities and the use of compen-
39), and also following inferior occipito-temporal lesions satory strategies by the patient, as this information may be
that involved the VWFA (Rapcsak & Beeson, 2004; Vanier helpful in planning treatment. The assessment of alexia is
& Caplan, 1985). As expected, patients with surface alexia best accomplished by the use of commercially available
following VWFA damage also showed evidence of visual reading batteries (e.g., Kay, Lesser, & Coltheart, 1992).
processing impairment and features of pure alexia/letter-
by-letter reading (Rapcsak & Beeson, 2004). A particularly
dramatic and pure form of surface alexia is consistently Treatment
observed in patients with semantic dementia (SD) (Wool-
lams et al., 2007). SD is a subtype of primary progressive A variety of behavioral treatment approaches have shown
aphasia/frontotemporal dementia in which the neurode- positive outcomes in the rehabilitation of alexia. In gen-
generative process has a predilection for left anterior and eral, treatment is directed toward strengthening the im-
inferolateral temporal cortex, including the temporal paired reading procedure/route or it encourages the use of
pole, middle/inferior temporal gyri, and anterior fusiform compensatory strategies to bypass the functional deficit
gyrus (BA38,20/21) (Galton et al., 2001; Mummery et al., (for a review, see Beeson & Rapcsak, 2006).
2000). Surface alexia has also been described in patients
with Alzheimers disease (Patterson, Graham, & Hodges,
1994) and is likely to reflect the frequent involvement of Cross References
left temporo-parietal cortex by the disease process. Al-
though distributed over a large anatomical area, the dis- Agraphia
parate extrasylvian lesion sites in surface alexia seem to Aphasia
have in common the potential for disrupting either lexical Dyslexia
orthographic or semantic processing. Specifically, in Phonological/Deep Agraphia
patients with VWFA involvement the reading disorder Surface Agraphia
Alexithymia A 83
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(2002). Language-specific tuning of visual cortex? Functional prop-
Woollams, A., Lambon Ralph, M. A., Plaut, D. C., & Patterson, K. (2007).
erties of the visual word form area. Brain, 125, 10541069.
SD-squared: On the association between semantic dementia and
Cohen, L., Martinaud, O., Lemer, C., Lehericy, S., Samson, Y., Obadia,
surface dyslexia. Psychological Review, 114, 316339.
M., et al. (2003). Visual word recognition in the left and right
hemispheres: Anatomical and functional correlates of peripheral
alexias. Cerebral Cortex, 13, 13131333.
Coltheart, M., Patterson, K., & Marshall, J. C. (1980). Deep dyslexia.
London: Routledge & Kegan Paul.
Coltheart, M., Rastle, K., Perry, C., Langdon, R., & Ziegler, J. (2001).
Alexia Without Agraphia
DRC: A dual route cascaded model of visual word recognition and
reading aloud. Psychological Review, 108, 204256. Alexia
Crisp, J., & Lambon Ralph, M. A. (2006). Unlocking the nature of the
phonological-deep dyslexia continuum: The keys to reading aloud
are in phonology ad semantics. Journal of Cognitive Neuroscience, 18,
348362.
Epelbaum, S., Pinel, P., Gaillard, R., Delmaire, C., Perrin, M., Dupont, S., Alexithymia
et al. (2008). Pure alexia as a disconnection syndrome: New diffusion
imaging evidence for an old concept. Cortex, 44, 962974.
Galton, C. J., Patterson, K., Graham, K., Lambon Ralph, M. A., Williams,
J OEL W. H UGHES
G., Antoun, N., et al. (2001). Differing patterns of temporal atrophy Kent State University
in Alzheimers disease and semantic dementia. Neurology, 57, Kent, OH, USA
216225.
Graham, N. L., Patterson, K., & Hodges, J. R. (2000). The impact of
semantic memory impairment on spelling: Evidence from semantic
dementia. Neuropsychologia, 38, 143163.
Definition
Jobard, G., Crivello, F., & Tzourio-Mazoyer, N. (2003). Evaluation of the
dual route theory of reading: A metaanalysis of 35 neuroimaging A deficit in apprehending, experiencing, and describing
studies. NeuroImage, 20, 693712. emotions, including difficulty in perceiving and under-
Kay, J., Lesser, R., & Coltheart, M. (1992). Psycholinguistic assessments of
standing the feelings of others. In particular, difficulty in
language processing in aphasia (PALPA). East Sussex, England: Lawr-
ence Erlbaum Associates.
distinguishing between emotions and bodily sensations
Mummery, C. J., Patterson, K., Price, C. J., Ashburner, J., Frackowiak, that indicate emotional arousal.
R. S. J., & Hodges, J. R. (2000). A voxel-based morphometry study of
semantic dementia: Relationship between temporal lobe atrophy and
semantic memory. Annals of Neurology, 47, 3645.
Patterson, K., Graham, N., & Hodges, J. R. (1994). Reading in dementia Current Knowledge
of the Alzheimer type: A preserved ability? Neuropsychology, 8,
835407.
The term alexithymia was coined by the late psychiatrist
Patterson, K., & Lambon Ralph, M. A. (1999). Selective disorders of
reading? Current Opinion in Neurobiology, 9, 235239.
Peter Sifneos to describe patients who could not find the
Patterson, K. E., Marshall, J. C., & Coltheart, M. (1985). Surface dyslexia: appropriate words to describe their emotional states.
Neuropsychological and cognitive studies of phonological reading. Literally meaning without words for emotions in
London: Lawrence Erlbaum. Sifneos native Greek, Alexithymia is a trait that overlaps
84 A Alice in Wonderland Syndrome
Categorization
Alice in Wonderland Syndrome Three variants of AHS have been described, each with
unique behavioral manifestations and neuroanatomical
Metamorphopsia correlations. These variants include the frontal, callosal,
and posterior forms.
Frontal Form
presentation corresponds to the topographical organiza- nondominant hand, they may experience difficulty with
A
tion of the PMC with control of lower limb movement the initiation of spontaneous speech while being able to
located more medially than the areas that control the follow verbal commands and repeat phrases without dif-
upper limb. The frontal variant is seen with involvement ficulty. These findings are consistent with a transcortical
of the medial aspect of the premotor cortex anterior to motor aphasia that affects spontaneous verbalization and
PMC including the supplementary motor area (SMA) and production of propositional language more than repeti-
anterior cingulate cortex (ACC). In functional activation tion and responsive language. Alternatively, this could be
studies, the medial frontal cortex has also been found to understood as an inability to initiate spontaneous verbal
activate spontaneously with complex purposeful move- output. The patient may thus be viewed as partially mute
ments and with internal imaging of voluntary movement, due to the relative akinesia seen with medial frontal cortex
suggesting that it may serve as a higher level system that injury.
modulates the activation of PMC in accordance
with volitional aspects of the performance. The readiness
potential that precedes an overt voluntary movement Callosal Form
by over 1,000 ms arises through activation of the ante-
romedial frontal cortex, suggesting that excitation of this Neuroanatomy
region precedes the appearance of the overt movement and The callosal variant is seen with an isolated lesion of the
activation of the PMC. Activation of the ACC is involved corpus callosum. The voluntary motor systems of the two
in intentional suppression of prepotent responses as tested hemispheres are isolated from each other due to lost
with the Stroop test. These areas may serve as a higher- interhemispheric communication. This variant has been
level system modulating the activation of PMC in described most frequently as a transient condition follow-
accordance with the volitional aspects of the performance. ing callosotomy. It may also be seen following infarction
or tumors selectively involving this structure.
Clinical Presentation
Behaviors seen frequently with the frontal variant in- Clinical Presentation
clude involuntary, visually driven reaching and grasping In the callosal variant of AHS, the appearance of
onto objects, an inability to voluntarily release these intermanual conflict or self-oppositional behaviors
objects, and utilization behavior in which the presence is the predominant feature. Grasping behaviors and exter-
of a frequently encountered object such as a comb or a nally driven reaching movements seen in the frontal vari-
toothbrush elicits behavior in which the object may be ant are notably less prominent. When there is a major
put to use independent of the social context. A grasp disconnection between the two hemispheres resulting
reflex to tactile stimulation is often present in the affect- from callosal injury, the language-linked dominant hemi-
ed hand. The patient may wake themselves up from sleep sphere agent that maintains its primary control over the
by grasping and pulling their own body parts. Patients contralateral dominant limb effectively loses its direct and
may show a prepotent tendency to be drawn toward linked control over the separate agent based in the
external objects. They also may demonstrate alien-asso- nondominant hemisphere (and, thus, the nondominant
ciated sexual self-stimulation or involuntary fondling of limb), which had been previously responsive and obedi-
anothers body, a great source of public embarrassment ent to the dominant agent. The possibility of purposeful
(Ong Hai and Odderson, 2000). Interestingly, while the action in the nondominant limb occurring outside of
patient clearly manifests purposeful involuntary coordi- the realm of influence of the dominant agent thus can
nated behaviors in the affected limb, when they attempt to occur. In the callosal variant, the problematic alien hand is
willfully move the limb, this is effortful and difficult. consistently the nondominant hand, while the dominant
Voluntary movement in the affected limb is often hypo- hand is the identified good controlled hand. The patient
kinetic and hypometric with greater activation of the axial may express frustration and bewilderment at the
and proximal limb muscles compared to the distal mus- conflicting and disruptive behavior of the alien hand
cles controlling the wrist and fingers, even though these whose motivations remain inaccessible to consciousness.
muscles are readily activated in the alien movements. There may be an attentional component that modulates
Generally, these alien behaviors appear in the hand con- the appearance of these episodes of self-oppositional
tralateral to the damaged hemisphere regardless of hemi- behavior since intermanual conflict is observed more
spheric dominance. When the dominant hemisphere is frequently when the patient is fatigued, stressed, or is
damaged, in addition to alien hand behavior in the engaged in effortful multitasking activity. Occasionally,
86 A Alien Hand Syndrome
rather than acting in a contradictory manner, the two from objects approaching the hand in distinct contrast to
hands are observed to be engaged in two different and the reaching and grasping behaviors that are seen in the
entirely unrelated activities as if being guided by frontal variant. The alien hand may assume a characteris-
completely separate and independent intentions. tic posture of fully extended digits with the palmar surface
In a dramatic example of this behavior, one patient retreating from environmental objects, an observation
was observed to initiate smoking a cigarette by pulling the that has been labeled an instinctive avoidance reaction
cigarette out of the package and placing it in her mouth by Denny-Brown and has also been referred to as the
with the controlled dominant hand followed by the alien parietal hand. At times, grasping behaviors can also be
nondominant hand, rather than beginning to light the observed with the posterior variant.
cigarette, suddenly reaching up, pulling it out of the her Alien hand behavior has also been reported in associ-
mouth, and throwing it across the room. Astonished, the ation with subcortical thalamic infarction. In addition to
patient reasoned that perhaps the alien hand was not in having been observed in the context of stroke, tumors and
favor of her smoking! surgical sectioning of the corpus callosum, alien hand
The callosal and frontal variants are often seen in behavior has been described in association with a number
combination with a corresponding overlap of observed of progressive neurodegenerative disorders including cor-
behaviors. For example, following cerebral infarction in ticobasal degeneration, multiple sclerosis, spongiform
the territory of the anterior cerebral artery, there may be encephalopathy, and Alzheimers disease. When AHS
ischemic injury to both the medial frontal lobe and the appears with these progressive encephalopathies, it is
corpus callosum. In this circumstance, there may be both usually accompanied by various forms of motor apraxia,
visually directed reaching and grasping alien behaviors along with multiple additional cognitive disturbances
in the limb contralateral to the area of injury as well as characteristic of the particular condition.
episodes of intermanual conflict. However, a clear differ-
entiation between apparent intermanual conflict due to
attempts to restrain alien behaviors associated with the Epidemiology
frontal variant (e.g., as in the case of self-grasping
described below), and true intermanual conflict, in While there are no epidemiologic studies of the occur-
which the two hands are directed toward independently rence of AHS variants in association with acquired brain
contradictory purposes, may be difficult to differentiate. damage, it can be assumed that this is a relatively rare but
striking manifestation of neurologic pathology.
upper limb that are directed by an internal action plan commissures linking the two cerebral hemispheres at the
A
and driven by an anticipatory model of future contingen- subcortical level. Thus, conscious human agency can be
cies. It presumably is also involved in activating with- thought of as emerging through the linked and coordinated
drawal movements that pull the limb back and away action of at least four major premotor systems, two in each
from external stimuli. It also functions to withhold action hemisphere. The overall general configuration of this heu-
directly responsive to surrounding objects through inhibi- ristic model is shown in Fig. 1.
tory influence over the PLPS. These two systems are pro- It is proposed that AHS, in its different variants de-
posed to be in a metastable balance through mutually scribed above, appears due to damage either to the corpus
inhibitory influence. Together, these two hemispheric callosum in the callosal variant (Fig. 2), the AMPS of
agency systems form an integrated intrahemispheric agency either hemisphere in the frontal variant (Figs. 3 and 4),
system. Furthermore, each intrahemispheric agency system or to the PLPS of either hemisphere in the posterior
has the capability of acting autonomously in its control over variant (Figs. 5 and 6).
the contralateral limb, although overall unitary control by a The common factor in these anomalous conditions is
conscious agent is maintained through interhemispheric the relative sparing of the PMC region controlling the
communication between these systems via the corpus contralesional alien hand, while the premotor regions
callosum at the cortical level and other interhemispheric involved in the intentional selection of action and the
Alien Hand Syndrome. Figure 1. Heuristic model for understanding alien hand syndrome (AHS).
Abbreviations: RH, Right Hemisphere; LH, Left Hemisphere; CC, Corpus Callosum; PMC, Primary Motor Cortex; AMPS,
Anteromedial Premotor System; PLPS, Posterolateral Premotor System.
This view is shown looking down from above the vertex with the face located at the top of the drawing and the back of the
head noted at the bottom of the drawing, the left side to the left and the right side to the right of the diagram. The open
bidirectional arrow between the AMPS and the PLPS indicates an interaction characterized by mutually interactive inhibition
creating a complementary metastable control of the contralateral hand. Solid arrows indicate facilitatory connections or
connections that maintain synchrony and coherence between the connected structures. Output from PMC is directed primarily
to the contralateral limb with some less potent ipsilateral projections illustrated by a dotted line. See text for further detail.
Note that the left hemisphere is stippled in the diagram designating this as the dominant hemisphere for most individuals in
correspondence with a dominant right hand
88 A Alien Hand Syndrome
Alien Hand Syndrome. Figure 2. The callosal variant of AHS. Theoretical explanatory model for the alien behaviors observed in
callosal damage. In this instance, there are findings consistent with callosal apraxia in addition to intermanual conflict associated
with the complete separation of the two intrahemispheric premotor intentional control systems. The limbs appear to be
operated by two relatively autonomous control systems. The intentional premotor system in the dominant hemisphere is linked
to the language system while that of the nondominant hemisphere is separated from it. The dominant hand is understood as
connected to self while the nondominant hand is not. The alien hand in this variant is the nondominant hand. This is indicated by
the stippled overlay on the left nondominant hand
inhibition of automatic behaviors in response to external conscious voluntary control. The patient may become
factors are impaired. fearful that they will be held accountable for consequences
A recent fMRI study of cortical activation patterns of an action of the alien hand over which they do not feel
associated with alien and non-alien movement has control. The patient may display auto-criticism com-
demonstrated that alien movement is in fact characterized plaining that the alien hand is not doing what it has been
by isolated activation of PMC without concomitant acti- told to do and is therefore characterized as disobedient,
vation of intrahemispheric premotor regions, while vol- wayward, or evil. They may even physically strike the
untary behavior includes the activation of PMC in concert alien hand with the controlled hand as a punishment
with activation of intrahemispheric premotor regions intended to discourage its wayward behavior, or constrain
(Assal, Schwartz, & Vuilleumier, 2007). the movement of the alien hand by grasping tightly onto it
with the controlled hand (self-grasping). They may
verbally address and instruct the hand as if it were an
Neuropsychology and Psychology unruly child acting autonomously and in need of correc-
of AHS tion. Conversely, they may respond to these contrary
actions with amusement.
The presence of AHS can cause the patient significant Given the predicament created, the patient may devel-
psychological distress as the hand seems to possess the op depersonalization and dissociate themselves from the
capability for acting autonomously, independent of their unintended actions of the hand. They often choose to
Alien Hand Syndrome A 89
Alien Hand Syndrome. Figure 3. The nondominant frontal Alien Hand Syndrome. Figure 5. The nondominant posterior
variant of AHS. Theoretical explanatory model for the alien variant of AHS. Theoretical explanatory model for the alien
behaviors observed in the frontal variant associated with behaviors observed in the posterior variant associated with
damage to the AMPS of the nondominant hemisphere. In this damage to the PLPS of the nondominant hemisphere. In this
case, the contralesional nondominant hand develops alien case, the contralesional nondominant hand develops alien
hand findings due to the release by disinhibition of the hand findings due to the release by disinhibition of behaviors
reaching and grasping behaviors driven from the dominant driven from the nondominant AMPS
PLPS
identify an external alien source for the voluntary con- evaluated for evidence of a grasp reflex with both tactile and
trol of the hand, or assign a distinct personality to the visual stimulation. The ability to release objects that have
hand as a way of seeking a satisfactory narrative to explain been grasped should also be assessed. Evaluation for callosal
this perplexing situation. apraxia and impairment of interhemispheric transfer of
From a psychological perspective, it is helpful to information should be included. When the posterior vari-
counsel the patient regarding the organic basis of their ant of AHS is suspected, a visual field assessment and
problem and provide assurance that there is a rational somatosensory examination of the affected limb should be
explanation for their concerns and that there is evidence completed as well as assessment for hemi-inattention. Evi-
that these problems can be treated and may gradually dence of a tendency to withdraw the limb from tactile and
improve over time. visual stimulation should also be elicited and noted.
In AHS, different regions of the brain are able to
command purposeful limb movements, without generat-
ing the conscious feeling of self-control over these move- Treatment
ments. There is thus a dissociation between the actual
execution of the physical movements of the limb and the There is no definitive specific treatment for AHS but a
process that produces an internal sense of voluntary con- number of different rehabilitative approaches have been
trol over the movements. This latter process, impaired in described. Furthermore, in the presence of unilateral
AHS, normally produces the conscious sensation that damage within a single cerebral hemisphere, there is
movement is being internally initiated and produced by often a gradual reduction in the frequency of alien beha-
an active self. Presumably, this process differentiates viors observed over time and a gradual restoration of
between re-afference (i.e., the return of kinesthetic sen- voluntary control over the affected hand. This suggests
sation from the self-generated active limb movement) that neuroplasticity in the bihemispheric and subcortical
and ex-afference (i.e., kinesthetic sensation generated brain systems involved in voluntary movement produc-
from an externally produced passive limb movement). tion can serve to reestablish functional connection be-
It may do this by giving rise to a parallel output signal tween the executive production process and the internal
from motor regions, a so-called efference copy. The self-generation and volitional registration process. Exactly
efference copy is then translated into a corollary dis- how this may occur is not well understood but could
charge, which conveys the expected re-afferent sensory involve a reorganization within residual elements of the
response from the commanded movement. The corollary intrahemispheric premotor systems both at the cortical
discharge can then be used in somatosensory cortex to and subcortical levels. In addition, some degree of ex-
distinguish re-afference from ex-afference and thus differ- panded participation of the intact ipsilateral hemisphere
entiate a self-produced active movement from a move- may be involved in the recovery process by extending
ment resulting from external forces. AHS may thus ipsilateral motor projections.
involve impaired production and transmission of either Different strategies can be used to reduce the interfer-
an efference copy or a corollary discharge signal. ence of the alien hand behavior in the ongoing coherent
controlled actions being performed by the patient. In the
frontal variant, an object such as a cane can be placed in
Evaluation the grip of the alien hand so that it does not reach out to
grasp onto other objects, thus impeding the patients
Evaluation of the patient with AHS involves careful forward progress during walking. In another approach,
observation of limb movement in various naturalistic voluntary control of the limb is developed by training the
contexts, along with reports from the patient regarding patient to perform a specific task with the alien limb, such
their sense of control over these movements. The relative as moving the alien hand to contact a specific object or a
dependence of movement on external context should be highly salient environmental target. Through training to
evaluated through assessment for utilization behaviors enhance volitional control, the patient can effectively
elicited by the presentation of external objects commonly override the alien behavior when it occurs. Recognizing
encountered in daily activities. A phenomenologic ap- that alien behaviors in the frontal variant are often sus-
proach to assessing and documenting the motor behavior tained by tactile input, another approach involves simul-
and linking it to introspective report from the patient is taneously muffling the actions of the alien hand and
essential. Not only should the verbal reports of the patient limiting sensory feedback by placing it in a restrictive
be noted, but also the associated affect. The limb should be cloak such as a specialized soft foam hand orthosis or,
Allele A 91
Current Knowledge
Allesthesia
Allokinesia is often associated with neglect syndromes,
J OHN E. M ENDOZA usually involving damage to the right hemisphere. It is
Tulane University Medical Center the motor counterpart of alloesthesia. Typically, a patient
New Orleans, LA, USA moves the right limb in response to a request to move
the left limb or moves towards the right, away from the
neglected side, when asked to move toward the neglected
Definition side. In animal models, the phenomena has been
associated with frontal, arcuate gyrus lesions (Heilman,
Misperception of the location of a stimulus. Although it Valenstein, Day, & Watson, 1995) and disconnections of
can occur in other modalities, it is most commonly frontal and posterior parietal cortices (Burcham, Corwin,
elicited by tactile stimulation and is often seen in the Stoll, & Reep, 1997).
presence of other symptoms of unilateral asomatog-
nosia. If a tactual stimulus is applied to the side of
the body contralateral to a hemispheric lesion, the alles-
thetic patient may perceive the nature of the stimulus Cross References
correctly but identify it as being applied to the compara-
ble area on the opposite (unaffected) side of the body. Allesthesia
In some instances the stimulus may be perceived as being Neglect Syndrome
on the same side of the body to which it was applied,
but displaced significantly from the point of the actual
stimulation (usually toward the midline). When present, References and Readings
this phenomenon likely results from post-rolandic
(parietal) lesions of the right rather than the left hemi- Burcham, K. J., Corwin, J. V., Stoll, M. L., & Reep, R. L. (1997).
sphere. More rarely it has been associated with brainstem Disconnection of medial agranular and posterior parietal cortex
lesions. produces multimodal neglect in rats. Behavioural Brain Research,
86(1), 4147.
Heilman, K. M., Valenstein, E., Day, A., & Watson, R. (1995). Frontal lobe
neglect in monkeys. Neurology, 45(6), 12051210.
Cross References
Asomatognosia
Alpha Rhythm
C INDY B. I VANHOE , N ATASHA K. E ADDY
Baylor College of Medicine
Allokinesia Houston, TX, USA
D OUGLAS I. K ATZ
Boston University School of Medicine Synonyms
Boston, MA, USA
Alpha waves; Bergers waves
Definition
Definition
This phenomenon refers to a motor response in
the wrong limb, contralateral to the requested side, Electromagnetic oscillations in the frequency range of
sometimes opposite to the direction requested. 812 Hz arising from synchronous and coherent electrical
Alprazolam A 93
J OHN C. C OURTNEY
Childrens Hospital of New Orleans
Current Knowledge New Orleans, LA, USA
Bragatti, J. A., De Moura Cordova, N., Rossato, R., & Bianchin, M. M. Benzodiazepine
(2007). Alpha coma and locked-in syndrome. Journal of Clinical
Neurophysiology, 24(3), 308.
Min, B. K., Busch, N. A., Debener, S., Kranczioch, C., Hansimayr, S., Proposed Mechanism(s) of Action
Engel, A. K., et al. (2007). The best of both worlds: Phase reset
of human EEG alpha activity and additive power contribute to
ERP generation. International Journal of Psychophysiology, 65(1), Binds to benzodiazepine receptors at the GABA-A ligand-
5868. gated channel, thus allowing for neuronal hyperpolariza-
tion. Benzodiazepines enhance the inhibitory action of
GABA via boosted chloride conductance.
Side Effects
Serious
Alpha-Synuclein Inclusions
Respiratory depression, hepatic dysfunction (rare), renal
Lewy Bodies dysfunction and blood dyscrasias, grand mal seizures
94 A ALS
Common
Altered Testing Procedures
Sedation, fatigue, depression, dizziness, memory pro-
blems, disinhibition, confusion, ataxia, slurred speech Modified Testing
Synonyms
ALS
Equivalent forms; Parallel forms
Anterolateral System
Definition
ALSFRS Alternate test forms are designed to avoid or reduce
content- or item-specific practice effects that are associated
Amyotrophic Lateral Sclerosis Functional Rating Scale with repeated administrations of the same neuropsycho-
logical test(s) (Benedict & Zgaljardic, 1998). Examination
of the manuals for many intellectual and neuropsycholog-
ical tests illustrate that practice effects are common,
ALSFRS-R especially over brief retest intervals (e.g., days or weeks).
Regarding test construction, alternate test forms should
Amyotrophic Lateral Sclerosis Functional Rating Scale include the same number of items, and the items should
be of equivalent difficulty. Moreover, the test instructions,
time limits, examples, and format should be identical to
the original instrument developed during standardiza-
Alterations tion, to reduce measurement error (Jackson, 2009). Of
course, measurement error can never be eliminated. For
Polymorphism example, content-sampling error and time-sampling error
inherent in all testretest paradigms are always
concerns in developing alternate test forms (Strauss,
Sherman, & Spreen, 2006). Additionally, alternate test
Altered forms cannot control other factors such as positive
carry-over effect (i.e., developing better test-taking stra-
Transgenic tegies), familiarity with the testing context (i.e., novelty
Alternate Test Forms A 95
effects), performance anxiety, and regression to the mean, provide alternate test forms. With the above caveats in
A
among others (Benedict & Zgaljardic, 1998; Busch, mind, alternate test forms can be useful in serial
Chelune, & Suchy, 2006; Salinsky, Storzbach, Dodrill, & neuropsychological evaluations.
Binder, 2001). This might, to some extent, explain why
some studies show that alternate test forms reduce or
eliminate practice effects, whereas other studies do not. Cross References
Short Biography
Alzheimer, Alois (18641915)
In the German municipality Marktbreit, Alois Alzheimer
K ATHERINE S. M C C LELLAN 1, A NNA B ACON M OORE 2 was born on June 14, 1864 to Eduard and Theresia
1
Atlanta Veterans Affairs Medical Center Alzheimer. Eduard, a Royal Notary, provided his family
Decatur, GA, USA with a comfortable upbringing. Although Alois had only
2
Emory University School of Medicine an older brother when he was born, six more siblings
Atlanta, GA, USA followed him. Alois spent the first four years of his
education at Catholic school in Marktbreit, until his
family left the area to find a new home with superior
Major Appointments educational opportunities for the children. The familys
chosen residence was in Aschaffenburg, and in 1874, Alois
Intern Mental Asylum at Frankfurt am Main, 1888 moved there in order to study at the Royal Humanistic
1895 Gymnasium. Alois completed his high-school degree in
Senior Physician Mental Asylum at Frankfurt am 1883 with excellent grades. He then decided to study
Main, 18951903 medicine because of his aptitude and fondness for the
Researcher Royal Psychiatric Clinic and District natural sciences, as well as a sense of duty to mankind.
Mental Asylum, Munich, 19031912 He enrolled at the Royal Friedrich Wilhelm Univer-
Assistant Professor Ludwig-Maximilian University, sity in Berlin for the 18831884 winter semester. In his
Munich, 19041912 psychiatry lecture there, he learned of John Conollys
Chief Physician Royal Psychiatric Clinic and District nonrestraint principle. Also called open treatment, the
Mental Asylum, Munich, 19061909 nonrestraint principle proposed the novel view that the
Professor of Psychiatry Psychiatry Clinic of Silesian mentally ill should be treated with a minimal amount of
Friedrich-Wilhelm University, Breslau, 19121915 physical constraint. Although Berlin was the medical
capital of Germany, Alois disliked Berlin and its
distance from his family. Therefore, he was transferred
Major Honors and Awards to the University of Wurzburg (Lower Franconia,
Germany), where his older brother was studying. As an
Extraordinary Professor, Ludwig-Maximilian Univer- aside, due to the influence of his older brother, Alois
sity (1909) joined and later held several officer positions in the
Geheimer Ministerialrat (Cabinet Councillor) (1915) Franconian Corps. His histology professor, Alfred von
Kolliker, gave him his first experience with microscopes
and staining techniques, which lead to his passion for
Landmark Clinical, Scientific, and forensic psychiatry. In the fall of the following year,
Professional Contributions Alois left to spend his winter semester at the Eberhard
Karls University of Tubingen. He returned in 1887 to the
Alois Alzheimer was both an excellent clinician and a Wurzburg Anatomical Institutes department of micros-
notable researcher. He is best remembered for being copy to write his doctoral thesis, On the Earwax
the first to definitively describe the symptoms and Glands. The intricate figures he presented in the
cerebral lesions of the disease now known as Alzhei- paper, as in all his papers, were proof of how scrupu-
mers Disease. Nonetheless, his contributions to sci- lously he conducted his research and clinical work. With
ence and medicine did not begin, nor do they end, the completion of his thesis, Alois Alzheimer received
there. He was one of the leaders of the movement to his doctor of medicine degree. He passed the state med-
implement the nonrestraint principle (explained ical examination and was awarded a license to practice
more fully below) in asylums. His neurohistological medicine in 1888.
work advanced the idea that psychiatric diseases were Shortly thereafter, he became a personal physician to a
biological in origin. And, through his roles as both mentally ill woman and traveled with her for five months.
doctor and scientist, he contributed to our under- Emil Sioli, the director of the Municipal Asylum for the
standing of a variety of conditions such as cerebral Insane and Epileptic in Frankfurt am Main had advertized
atherosclerosis, alcoholism, and general paresis. for an intern, specifically hoping for a competent doctor
Alzheimer, Alois (18641915) A 97
who was also adept with a microscope. Upon his return, reputations of all its doctors. But above all, in 1901,
A
the 24-year old Dr. Alzheimer was hired immediately. Alzheimer met the patient who would immortalize his
Dr. Franz Nissl also was hired as senior physician for the name: Auguste D. Auguste had been admitted to the
asylum. Nissl not only became one of Alzheimers closest asylum because of delusional and excessively forgetful
friends, but also taught him a powerful staining technique behavior. Although at admission she was disoriented,
for highlighting neuronal cell bodies (the Nissl stain), that anxious, and suspicious, over time she became unruly
helped Alzheimer achieve success in his histological stud- and disruptive. Alzheimer was particularly intrigued by
ies. Siolis main goal for the asylum was to fully employ her case for the duration of her stay in the hospital.
the nonrestraint principle. Alzheimer was particularly Alzheimers second child, Hans, was born in 1896,
skilled at gaining the trust of patients through conversa- and his third, Maria, was born in 1900. However, the
tion, and he often documented these conversations. lavish lifestyle he had lived with Cecilie ended when she
The dialogues often were central to diagnosing a patient, died in February 1901. Alzheimers sister, Elisabeth, took
and even more so to research. His talent in clinical inter- over his household. Though she was strict, she became an
viewing was such that clinicians who later read his notes integral part of the family. Without Cecilie, Alzheimer no
had sufficient information to evaluate his opinions and to longer had a reason to stay in Frankfurt. After his appli-
make their own diagnoses. Alzheimer drew on his micros- cation to be director of a regional asylum was rejected,
copy and forensic psychiatry training, to do histological he joined Nissl in Heidelberg in 1903 and went to work
investigations into the physical origins of psychiatric for Emil Kraepelin. The group he joined there was an
disorder. In Frankfurt, his topics of study included epi- international team of researchers. Later that same year,
lepsy, senile dementia, criminal minds, and a variety of Kraepelin was named director of the Royal Psychiatric
psychoses. He established himself as a well-rounded Clinic and the District Mental Asylum in Munich.
physician by publishing papers on a wide variety of topics. Alzheimer followed him, but was not paid in Munich
Aside from his duties as a physician and researcher, he also due to the lack of a position for him, and also his
appeared as an expert before courts and presented at desire to manage his own time. Despite his absence
many scientific meetings. While at Frankfurt, Alzheimer from Frankfurt, Alzheimer still received updates on
became an expert on general paresis, which later became Auguste D.
the subject of his postdoctoral thesis. By this point, Alzheimers thesis on general paresis
In Algeria, a personal physician who had been was finished, but because he moved twice in such a
traveling with a man suffering from general paresis sent short time, he had not yet turned it in. Alzheimer submit-
a telegram to Alzheimer in 1892 to request that he treat ted his postdoctoral thesis to the Ludwig-Maximilian
the worsening patient. Alzheimer obliged and went to University in Munich with the hopes of gaining associate
North Africa. He intended to bring the patient back to professorship. In it, he published not only his clinical
his hospital in Germany, but the patient died before dialogues, but also his postmortem histological findings.
reaching Germany, leaving his wife, Cecilie, a widow. With this paper, he asserted that histological exam-
Alzheimer and Cecilie became close friends, and eventu- inations could definitively show the presence of general
ally the widow asked him to marry her. They were married paresis. Until then, few doctors suspected that syphilis was
in April 1894 in the registry office of Frankfurt. Because a cause of general paresis, but shortly thereafter the link
Cecilie was Jewish, she had to convert to Catholicism between the two was found. His work was surpassed by
before the two could be married by the church in Febru- the discovery of a way to diagnose syphilis, without
ary 1895. On March 10, 1895, their first child, Gertrud, resorting to autopsies. In August 1904, he joined the
was born, and Dr. Nissl was chosen to be her godfather. universitys medical faculty.
But Nissl soon moved to work with Emil Kraepelin in Because of his experience at remodeling the Frankfurt
Heidelberg. Nissls departure created room for Alzheimer clinic, Alzheimer was fundamental in finishing the plans
to be promoted to senior physician within Siolis asylum. for the new Munich clinic. He furnished his anatomic
Also that year, to lessen the overcrowding of the main laboratory with the best equipment and the brightest
hospital, a new branch asylum opened. With this addi- students many of whom went on to make great con-
tion, Sioli and Alzheimer furthered their goal of fully tributions to science, including Ugo Cerletti electrical
implementing the nonrestraint principle by instituting shocks to generate convulsions, Hans Gerhard Creutzfeldt
duration baths rather than isolation. The asylum became and Alfons Jakob Creutzfeldt-Jakob disease, Frederic
known as a revolutionary clinic, and it elevated the Lewy Lewy bodies, and others. Alzheimer was made
98 A Alzheimer, Alois (18641915)
chief physician in 1906, a paid position, but also one that Though he returned to work, his health had not im-
took away much of his time in the laboratory. proved. This did not impede his ability to make signifi-
Two topics that consumed Alzheimer in Munich were cant contributions to science: in 1913 he found the
psychiatric symptoms resulting from pathological anato- syphilis pathogen in the central nervous system of a
my and classification of mental illnesses by etiology. The patient with general paresis.
latter faced much opposition from the scientific commu- After a long illness, Alois Alzheimer died on December
nity. Yet, the most opposition he ever faced was his pre- 19, 1915 from a heart condition and kidney failure.
sentation of the Auguste D. case. Auguste D. had always Though no one immediately took over his pursuit of an
fascinated Alzheimer. He had paid special attention to her, understanding of Alzheimers disease, people recom-
taking copious notes about their conversations. When he menced research on Alzheimers disease cases in the
moved away, he still received updates about her condition, 1950s. Studies of the disease began in earnest after Martin
which worsened progressively until her death. When Roths assertion in the 1960s that Alzheimers disease was
Auguste D. died in 1906, her files, brain, and spinal cord the most common cause of senile dementia. In the 1970s,
were sent to Munich. Alzheimer, along with his student Robert Katzman further propelled the surge of interest
Gaetano Perusini, immediately began examining the case. in Alzheimers disease by stating that it was one of the
In Tubingen, Alzheimer presented her case in a lecture most widespread diseases. Since then, the amount of re-
entitled On a Peculiar Severe Disease Process of the search on Alzheimers disease has increased exponentially,
Cerebral Cortex, in which he described the lesions (now resulting in multiple foundations and centers devoted
known to be neurofibullary tangles) that he believed solely to the disease that Alois Alzheimers colleagues con-
caused Augustes symptoms. Based on records from the sidered trivial.
time, his peers did not bother to ask questions, nor were
there any comments about the lecture in the minutes. He
later published the entire lecture, but still it received little Cross References
attention. He then tasked Perusini to find more patients,
similar to Auguste D. in the clinic. Perusini found four Alzheimers Dementia
cases and published an article entitled On Clinically and Alzheimers Disease
Histologically Peculiar Mental Illnesses in Advanced Age. Paresis
Another student of Alzheimers, Francesco Bonfiglio
found another case of presenile dementia, and also
published on the disease. Spurred by Bonfiglios paper, References and Readings
Kraepelin included a section on Alzheimers Disease, in
the 1910 edition of his text book Clinical Psychiatry. This Engstrom, E. (2007). Researching dementia in imperial Germany: Alois
Alzheimer and the economies of psychiatric practice. Culture, Medi-
publication is acknowledged as the origin of the term.
cine, and Psychiatry, 31, 405413.
Alzheimer himself never referred to it as Alzheimers Graeber, M., Kosel, S., Egensperger, R., Banati, R., Muller, U., Bise, K.,
Disease, though he had later publications on the disease. et al. (1997). Rediscovery of the case described by Alois Alzheimer in
Alzheimer decided to resign his post as chief physician in 1911: Historical and molecular genetic analysis. Neurogenetics,
order to devote more time to research, specifically 1, 73, 80.
Lage, J. (2006). 100 years of Alzheimers disease (19062006). Journal of
traveling to study epilepsy. Although he was no longer
Alzheimers Disease, 9, 1526.
employed by Kraepelin, Alzheimer undertook the respon- Maurer, K., & Maurer, U. (1998). Alzheimer: The life of a physician and the
sibilities of coeditor of Kraepelins Journal of Complete career of a disease. New York: Columbia University Press.
Neurology and Psychiatry. Morris, R., & Salmon, D. (2007). The centennial of Alzheimers disease
Recognition for Alzheimer and the disease carrying and the publication of Uber eine eigenartige Erkankung der
Hirnrinde by Alois Alzheimer. Cortex, 43, 821825.
his name began to spread. In 1912, the Silesian Friedrich-
Small, D., & Cappai, R. (2006). Alois Alzheimer and Alzheimers
Wilhelm University in Breslau asked him to join their disease: A centennial perspective. Journal of Neurochemistry, 99,
faculty as a full professor of psychiatry. During the move 708710.
to Breslau, Alzheimer fell ill, but nevertheless assumed his Snyder, P., & Pearn, A. (2007). Historical note on Darwins consideration
duties with vivacity. His patients and coworkers, includ- of early-onset dementia in older persons, thirty-six years
before Alzheimers initial case report. Alzheimers and Dementia,
ing Georg Stertz, Ottfried Forster, and Ludwig Mann,
3, 137142.
took notice of his kind, yet authoritative presence. In Zilka, N., & Novak, M. (2006). The tangled story of Alois Alzheimer.
1913, his health forced him to visit a private clinic. Bratisl Lek Listy, 107, 343345.
Alzheimers Dementia A 99
Epidemiology
Short Description or Definition
Prevalence and Incidence. AD is the most common cause of
One of the leading causes of dementia in late-life, dementia in late-life, accounting for 5070% of all cases
Alzheimers disease (AD), is a progressive neurodegenera- (Malaspina Corcoran, Schobel, & Hamilton, 2008).
tive disorder characterized by a gradual onset and pro- Current estimates suggest that 4.5 million individuals
gressive course, affecting memory and other cognitive suffer from AD in the US, and projections based on
domains. For a diagnosis, the cognitive impairments population trends suggest an increase to 13.2 million by
of AD must not occur exclusively in the context of a 2050 (U.S. Department of Health and Human Services,
delirium, and must be of sufficient severity to cause 2006). The overall prevalence of AD is about 56% in
impairment in social or occupational functioning. Diag- individuals aged 65 years or older in North America, and
noses of AD (Possible or Probable AD) are based on the doubles approximately every 5 years after the age of 60.
history and presentation of clinical symptoms, evidence Estimates suggest a prevalence of 1% at age 60, 16%
of cognitive impairment, and the exclusion of other between ages 80 to 85, and 26 to 45% for those above
causes of dementia such as stroke, metabolic disorders, age 85. Incidence rates also exhibit an age-related increase.
or other conditions that may account for the cognitive Studies report differing patterns of AD prevalence and
impairment. A diagnosis of Definite AD is based upon incidence at the upper end of the lifespan, with some
postmortem neuropathological analysis and is made reporting a plateau at very old ages (age 90 or 100;
when there are sufficient numbers of senile plaques and Mendez & Cummings, 2003).
neurofibrillary tangles in specific brain regions. Risk Factors. Increasing age is among the strongest
risk factor for AD. Other risk factors include the 4 allele
of the Apolipoprotein E (APOE) gene, positive family
Categorization history (also in sporadic AD), low education (possibly
due to less neural reserve), female gender (even after
AD may be categorized according to age of onset, accounting for differential survival), and history of head
family history, or presenting clinical features. Age cate- trauma and vascular factors such as high cholesterol and
gories distinguish between senile and pre-senile onset high blood pressure. Some risk factors occurring earlier in
(onset before age 65). Classifications based on family the lifespan affect AD risk. Studies suggest that high blood
history (familial AD vs. sporadic AD) distinguish AD pressure or high serum cholesterol in midlife increases the
forms that show high heritability. Familial AD is rare, risk of AD later in life. Although inconsistent, some stud-
generally of pre-senile onset, and has been associated ies report that treatment with antihypertensive medica-
with mutations in the APP gene on chromosome 21, tions or cholesterol lowing agents reduces the risk for AD
Presenilin 1 gene on chromosome 14, and Presenilin (Soininen, Kivipelto, Laakso, & Hiltunen, 2003). Recent
2 gene on chromosome 1 (Hardy, 2003). Its transmission studies have also examined the role of insulin resistance
resembles an autosomal dominant pattern (Morris & and diabetes in AD risk. Among potential protective
Nagy, 2004). factors, data from epidemiological studies suggest a lower
100 A Alzheimers Dementia
risk of AD among women receiving hormone replacement symptom onset range from 2 to 20 years. The mean
therapy. However, a large randomized clinical trial of survival has been reported as approximately 10 years,
estrogen and estrogen + progesterone in elderly women but some studies have reported considerably shorter du-
suggested an increase in all-cause dementia in those re- ration of 3 years. More rapid rate of disease progression
ceiving the combination hormone treatment. Thus, hor- has been associated with early, prominent language im-
mone therapy is not recommended for cognitive health pairment, frontal features, and extrapyramidal signs
(Malaspina et al., 2008). Other factors under active inves- (Mendez & Cummings, 2003).
tigation are diet, nutrients and nutrient supplements such
as antioxidant vitamins, omega 3 fatty acid, medications
such as non-steroidal anti-inflammatory agents, and life- Neuropsychology and Psychology
style practices such as physical activity and cognitive and of Alzheimers Dementia
social engagement.
Neuropsychological Deficits
Natural History, Prognostic Factors, The neuropsychology of AD follows the clinical progres-
Outcomes sion. In early stages, memory is almost always involved,
with specific deficits in learning new information. Remote
The clinical course of AD is usually one of a gradual onset memory such as memory for autobiographical or other
of symptoms with progressive decline. Many scientists knowledge-based systems (semantic memory) is relatively
believe the disease process starts in the brain decades unaffected. In early stages, standardized testing with
before overt symptoms emerge. A preclinical phase, char- word lists may reveal relative preservation of immediate
acterized primarily by episodic memory deficits, heralds or working memory, but impairment in delayed recall.
the onset of symptoms. This stage, also referred to as There is usually some benefit from cuing or recognition
mild cognitive impairment (MCI), lasts approximately procedures. With progression, cuing is no longer helpful,
13 years. Progression to dementia is characterized by and remote recall is affected. Implicit memory may be
increasing severity of cognitive impairment with severe relatively spared as patients show evidence of learning
memory deficits, visuospatial impairment, and other per- on priming and procedural motor tasks. Orientation to
ceptual disturbances. Language impairment begins with time and place is also affected in AD (Knopman & Selnes,
mild naming difficulties and circumlocutory speech, but 2003).
progresses to include comprehension deficits. Apraxia Language impairments progress from mild anomia
(difficulty performing learned motor tasks in the absence and word finding difficulties in early stages, to include
of impairment in primary motor or sensory functions) impairment in comprehension and writing. Errors in
and impaired executive functions and computational speech (paraphasias) become more common, and word
ability are also apparent. Behavioral changes are common substitutions become progressively less related to the tar-
with indifference, irritability, and sadness, progressing to get words. Repetition of speech may be relatively unaffect-
delusions and, in some individuals, more severe psychiat- ed until late in the disease course (Knopman & Selnes,
ric disturbances such as hallucinations and agitation. In 2003; Mendez & Cummings, 2003). Tests of verbal fluency
end stages, there is severe deterioration of all cognitive and confrontation naming are especially sensitive to early
functions, speech is generally unintelligible, and motor changes in language. Visuospatial disturbances may be
rigidity and urinary and fecal incontinence are present. subtle or nonexistent in the earliest stages of AD. In
Death may occur as the result of other causes such as moderate and severe stages, impairment may be evident
pneumonia or infections (Mendez & Cummings, 2003). on figure copying tasks or judgment of line orientation
On postmortem exam, the brain is characterized by (Knopman & Selnes, 2003). Figure 3 displays characteris-
generalized atrophy and sulcal and ventricular enlarge- tic examples of visuoconstructional impairment in four
ment. Figure 1a displays gross atrophy of an AD brain representative patients with AD.
compared with a brain from a cognitively normal elderly Impaired abstract reasoning, sustained attention,
individual. Figure 2 displays a coronal section of an AD planning, judgment, and problem solving may characterize
brain at the level of the hippocampus. impairment in executive functions. Deficits in executive
The duration of the entire disease course from MCI functions may be demonstrated on tests of verbal fluency,
to death is highly variable. Survival estimates from trailmaking, and set shifting. Tests such as the Rey Complex
Alzheimers Dementia A 101
Alzheimers Dementia. Figure 1 (a) and (b) display the brains from a cognitively normal elderly individual and an individual who
suffered from advanced AD, respectively. Note the severe atrophy apparent in the AD brain (Photo courtesy of Christine Hulette,
M.D., Bryan Alzheimer Disease Research Center, Duke University. Reproduced with permission from Elsevier Limited)
figure and clock drawing may also elicit impairment in apraxia may involve pantomiming the execution of a
executive functions with poor planning and execution of task. Anosognosia or an unawareness of disability is quite
the tasks. Deficits in working memory may be evident on common (Knopman & Selnes, 2003). Standardized assess-
tasks requiring mental manipulation or divided attention ment approaches are few. Some approaches rely on clini-
(Knopman & Selnes, 2003). cal observation, noting a discrepancy between self-report
Other neurocognitive aspects of AD include apraxia and of cognitive impairment and test performance, or a
anosognosia. In mild AD, deficits in praxis are not common discrepancy between caregiver and patient report of
but emerge later in the disease course. Assessment of impairment.
102 A Alzheimers Dementia
Alzheimers Dementia. Figure 2 Display of the atrophy in AD in this coronal section including the hippocampi. Note the dilated
lateral ventricles and loss of inferior temporal mass (Photo courtesy of Steven S. Chin, M.D., Ph.D., University of Utah Health
Sciences Center)
Alzheimers Dementia. Figure 3 Display of the visuoconstructional impairments in the drawings of four individuals with
Possible or Probable AD. The stimulus is the left-most figure
Alzheimers Dementia. Figure. 4. Seventy-four year old control subject with normal cognition. The top row shows normal brain
metabolic activity and the bottom row shows very few regions of hypometabolism. The areas of significant hypometabolism
indicated in the medial views are due to this individual having enlarged lateral ventricles relative to normative subjects.
Figures 46 These images are processed FDG-PET images obtained from elderly subjects. The images have been processed using
Neurostat sterotactic surface projections to illustrate the changes of the brain in Alzheimers disease. Subject scans are shown in
two rows in each figure, depicting projections onto six surfaces: R-lateral, L-lateral, R-medial, L-medial, Superior and Inferior. The
top row in each figure displays regional glucose metabolism with cooler colors (purple, blue) reflecting areas of
hypometabolism. The bottom row in each figure displays relative glucose metabolism for each participant as compared with a
normative sample of 27 cognitively normal elderly individuals. In this bottom series, the images display the statistical
significance, expressed as Z-scores, of the hypometabolism when compared to those of the normative sample. The brighter
colors (red, white) represent areas of significant hypometabolism and the cooler colors of blues and purples represent relatively
normal brain metabolism (All photographs courtesy of Norman L. Foster, M.D. and Angela Y. Wang, Ph.D., Center for Alzheimers
Care, Imaging and Research, University of Utah)
Alzheimers Dementia. Figure. 5. Sixty year old subject clinically diagnosed with MCI. The top row shows symmetric decreases
in metabolic activity in both hemispheres of the brain. Abnormalities are primarily in the parietal lobe (shown in the R-lateral and
L-lateral views) and the posterior cingulate cortex (shown in the R-medial and L-medial views), as seen in the green regions. The
bottom row confirms that these regions (green, yellow and red areas) are indeed significantly (Z-scores 2.5) hypometabolic.
This pattern is a distinguishing feature of AD seen in FDG-PET studies (All photographs courtesy of Norman L. Foster, M.D. and
Angela Y. Wang, Ph.D., Center for Alzheimers Care, Imaging and Research, University of Utah)
104 A Alzheimers Dementia
Alzheimers Dementia. Figure. 6. Seventy-two year old subject clinically diagnosed with AD. This subject shows an even greater
and more widely distributed decrease in glucose metabolism. Parietal and temporal lobes and posterior cingulate cortex (green
and blue region in the top row) are affected. The statistically significant changes in metabolic pattern (red and white regions in
the lower row) are much greater than the MCI case (All photographs courtesy of Norman L. Foster, M.D. and Angela Y. Wang, Ph.
D., Center for Alzheimers Care, Imaging and Research, University of Utah)
imaging is a more sensitive technique for detecting vomiting (Orgogozo, 2003). Memantine, an NMDA glu-
changes in brain function in early stages. Reduced glucose tamate receptor blocker, has been approved for use in
metabolism, usually in the temporoparietal and posteri- moderate and severe AD. This drug is believed to
or cingulate regions, is a consistent pattern in early AD. be effective by reducing neuronal excitotoxicity. Other
Figures 4 through 6 display the pattern of glucose hypo- treatments include the use of psychotropic medications
metabolism in MCI and AD compared with a cognitively (such as antidepressant and antipsychotic medications) to
normal elderly individual. Neuropsychological testing is address the behavioral or neuropsychiatric symptoms.
important to establish the degree of cognitive impairment Cognitive rehabilitation may be attempted early in the
and to identify patterns that may be suggestive of specific disease course while patients are still able to participate.
dementing illnesses. Additional tests such as sampling Psychoeducation, behavioral techniques, music therapy,
cerebrospinal fluid for tau and amyloid-B42 assays may and caregiver support and interventions are also impor-
be helpful as supplemental procedures in complex cases tant elements of clinical care.
(Mendez & Cummings, 2003).
Cross References
Treatment
Alois Alzheimer
Treatment for AD is palliative, with medications and thera- Aricept (Donepezil)
pies providing symptom management. Medications most Cholinesterase Inhibitors
commonly used are cholinesterase inhibitors that func- Dementia
tionally address the cholinergic deficit of AD by blocking Neurofibrillary Tangles
the activity of the acetylcholine degrading enzyme, acetyl- Senile Dementia
cholinesterase. These medications are modestly effective, Senile Plaques
and patients and families may observe an improvement in
some cognitive and behavioral symptoms. However, the
medications do not modify the trajectory of disease pro- References and Readings
gression. In general, cholinesterase inhibitors are well-
tolerated. The use of the first FDA-approved drug of this Grabowski, T. J., & Damasio, A. R. (2004). Definition, clinical features
and neuroanatomical basis of dementia. In M. M. Esiri, V. M.-Y.
class, tacrine, however, is rarely administered now because
Lee, & J. Q. Trojanowski (Eds.), The neuropathology of dementia
of risk of liver toxicity. Other medications include donepe- (2nd ed., pp. 133). Cambridge, UK: Cambridge University Press.
zil, rivastigmine, and galantamine. Side effects include Hardy, J. (2003). The genetics of Alzheimers disease. In K. Iqbal &
gastrointestinal symptoms such as diarrhea, nausea, and B. Winblad (Eds.), Alzheimers disease and related disorders: research
Alzheimers Disease A 105
advances (pp. 151153). Bucharest, Romania: Ana Asian Interna- progressive paranoia, delusional thinking, disorientation,
tional Academy of Aging. A
and poor memory. She was institutionalized for the last
Knopman, D., & Selnes, O. (2003). Neuropsychology of dementia.
In K. M. Heilman & E. Valensteins (Eds.), Clinical neuropsychology
3 years of her life. Upon her death, Alzheimer analyzed her
(4th ed., pp. 574616). New York: Oxford University Press. brain using a silver stain, and described both extracellular
Malaspina, D., Corcoran, C., Schobel, S., & Hamilton, S. P. (2008). and intracellular protein accumulations. The extracellular
Epidemiological and genetic aspects of neuropsychiatric disorders. protein accumulations were termed plaques and the
In S. C. Yudofsky & R. E. Hales (Eds.), Neuropsychiatry and beha-
intraneuronal protein accumulations were called tangles.
vioral neurosciences (5th ed., pp. 301362). Washington, DC:
American Psychiatric Association Press.
Alzheimer presented the results of this autopsy in 1906.
Mendez, M. F., & Cummings, J. L. (2003). Dementia a clinical approach Several other similar cases of relatively presenile (i.e.,
(3rd ed.). Philadelphia: Butterworth. arbitrarily defined as an onset prior to age 5565) clinical
Morris, J. H., & Nagy, Z. (2004). Alzheimers disease. In M. M. Esiri, dementia associated with plaques and tangles were noted
V. M.-Y. Lee, & J. Q. Trojanowski (Eds.), The neuropathology of
by Alzheimer and others over the next 4 years. In 1910,
dementia (2nd ed., pp. 161206). Cambridge, UK: Cambridge Uni-
versity Press.
Alzheimers departmental chair, Emil Kraepelin, pub-
Orgogozo, J.-M. (2003). Treatment of Alzheimers disease with cholines- lished a textbook covering the fields of neurology and
terase inhibitors. An update on currently used drugs. In K. Iqbal & psychiatry, and referred to patients with presenile demen-
B. Winblad (Eds.), Alzheimers disease and related disorders: tia, plaques, and tangles as having Alzheimers disease.
Research advances (pp. 663675). Bucharest, Romania: Ana Asian
Concurrently, other investigators, such as Oscar
International Academy of Aging.
Soininen, H., Kivipelto, M., Laakso, M., & Hiltunen, M. (2003). Genetics,
Fischer, also reported plaque presence in elderly demented
molecular epidemiology and cardiovascular risk factors of individuals. These individuals were older than those
Alzheimers disease. In K. Iqbal & B. Winblad (Eds.), Alzheimers with presenile dementia (i.e., generally older than age
disease and related disorders: Research advances (pp. 5362). 5565). As the commonality of progressive dementia in
Bucharest, Romania: Ana Asian International Academy of Aging.
the elderly was well recognized, the presence of plaques
U.S. Department of Health and Human Services. (2006). Journey
to discovery. 20052006 Progress report on Alzheimers disease.
in elderly demented individuals was felt to represent a
Washington, DC: U.S. Department of Health and Human Services. normal phenomenon. Such individuals were not diag-
nosed with Alzheimers disease. Instead, cognitive decline
in elderly adults was attributed to normal aging or other
poorly described conditions, such as hardening of the
Alzheimers Disease arteries. As a result, Alzheimers disease remained rela-
tively uncommon for a number of subsequent decades.
RUSSELL H. S WERDLOW, H EATHER A NDERSON In the 1960s, investigators began comparing elderly
J EFFREY M. B URNS demented subjects to those diagnosed with presenile
University of Kansas School of Medicine Alzheimers disease. Notable similarities were observed
Kansas City, KS, USA regarding the clinical course (chronic and progressive),
the clinical features (cognitive decline that featured evo-
lution of an amnestic state, followed by behavioral
Definition changes), and histopathology (plaques and tangles). By
the 1970s, the number of demented elderly was growing
A neurodegenerative disease of the brain characterized fast as demographic shifts in the aging population com-
clinically by insidious, chronic, and progressive cogni- bined with increased recognition of the syndrome. At this
tive decline, and histologically by cerebral accumula- point, the original definition of Alzheimers disease (as
tions of the proteins beta amyloid (plaques) and tau described by Alzheimer and named by Kraepelin) was
(tangles). expanded to account for all dementing individuals with
plaques and tangles, although some separation of these
groups was envisioned. Those meeting the original cri-
Historical Background teria of plaque and tangle dementia in presenile adults
were designated as having dementia of the Alzheimer
In 1902, a woman called Auguste D. came under the care type (DAT), while the previously unconsidered elderly
of Dr. Alois Alzheimer, then at the University of Frank- cases were designated as having senile dementia of the
furt. The patient manifested changes in behavior and Alzheimer type (SDAT). With increasing recognition of
cognition. Her clinical course was characterized by the problem, Alzheimers disease very quickly became
106 A Alzheimers Disease
incredibly common, as well as a Western civilization 40 and 42 amino acid-long variants of Ab predominate in
health priority. plaques, and are often designated Ab40 and Ab42. Ab42
In the USA, the 1980s saw the establishment of feder- seems to be particularly important to the formation of the
ally funded Alzheimers disease research centers, which amyloid plaques of Alzheimers disease, probably because
began to systematically study the clinical course of this this version of the protein is quite insoluble. When Ab
progressive dementia, mostly in the common SDAT form. accumulations begin to form in brain, they are not asso-
Academic research began to unravel the chemical make- ciated with disrupted cell elements and are called diffuse
up of plaques and tangles. Investigations into patterns and plaques. Another type of more evolved plaque can also be
causes of neurodegeneration were performed. This ad- found in Alzheimers disease patients, in which Ab
vancing knowledge enhanced the ability of clinicians to becomes condensed at the center of the plaque, and the
diagnose Alzheimers disease at increasingly subtle stages, vicinity of the plaque is associated with disrupted cell
as well as the ability to pharmacologically intervene to elements such as degenerating axons and dendrites. As
achieve partial, temporary symptomatic benefit in at least axons and dendrites are collectively called neurites, this
some individuals. type of plaque is called a neuritic plaque.
The tangles of Alzheimers disease are found primarily
in neurons. Under the microscope tangles have a fibrous
Current Knowledge quality to them, and hence tangles in Alzheimers disease
are referred to as neurofibrillary tangles. Neurofibrillary
Scientific Perspective tangles consist of a protein called tau. Normally, tau is
found in association with microtubules, which act as a
The plaques seen in persons with Alzheimers disease skeleton, or cytoskeleton supporting the cellular struc-
contain several aggregated proteins. The major constitu- ture. The function of tau appears to be the stabilization of
ent is a protein called amyloid beta (Ab). Beta is a these microtubules. Like many proteins, after its produc-
chemical term that specifies a certain pattern of protein tion tau is modified by the addition and subtraction of
folding. Amyloid is a general term that refers to proteins phosphate groups on certain amino acids, especially ser-
that give a particular appearance when exposed to a ine and threonine. During embryonic development, tau is
particular type of stain, Congo red. The beta amyloid, or heavily phosphorylated, but during youth and early adult-
Ab, found in the brains of Alzheimers disease patients hood this heavily phosphorylated pattern is rare if at all
derives from a particular protein called the amyloid pre- seen. In Alzheimers disease, though, tau again takes on
cursor protein (APP). a heavily phosphorylated pattern, which is felt to reflect
In the human brain, the APP is 695 amino acids long. an abnormal physiologic event and is referred to as
It is a transmembrane protein. One end (the carboxy end) tau hyperphosphorylation. Hyperphosphorylated tau
is found inside neurons, in the cytoplasm. The other end molecules begin to pair off, a process called dimeriza-
(the amino end) extends outside the cell. In between the tion. Hyperphosphorylated tau dimers, also called
cytoplasmic and extracellular portions is a stretch that paired helical filaments, are quite insoluble and begin
runs through the membrane. The normal function of to aggregate with each other. This aggregation, typically
APP is not well known. APP is digested by different visible extending from cell bodies into axons, comprises
enzymes, which cut the protein at different points. An the neurofibrillary tangle.
enzyme complex called the beta secretase (BACE) cuts As impressive as this advancing understanding of
APP in its extracellular portion. An enzyme or group of plaque and tangle composition is, recognizing what con-
enzymes referred to as the alpha secretase cuts APP in its stitutes these aggregations does not address why they
intramembrane segment. The gamma secretase cuts APP form. In this regard, genetic studies of DAT subjects who
twice, both times in its intramembrane segment. Both of inherit the disorder in an autosomal dominant fashion
the gamma secretase cuts occur closer to the carboxy end have had a large impact. Several hundred such families
of the APP than the alpha secretase cut. have been documented. In these families the disease
Different cutting combinations generate various APP affects about 50% of each generation, with typical onset
by-products. Cutting of an APP by beta and gamma occurring in the 3rd, 4th, 5th, or 6th decades. A small
secretases generates a 3843 amino acid stretch, and this number of these families have demonstrable mutations in
stretch tends to assume a beta folding conformation and the gene that encodes the APP. This gene is located on
has the features of an amyloid protein (i.e., birefringence chromosome 21, the same chromosome that is present in
under the microscope when stained with Congo red). The excess in Downs syndrome. Downs syndrome patients
Alzheimers Disease A 107
invariably accumulate Ab plaques in their 5th decade. with no other systemic or brain diseases that could
A
A somewhat larger number of these families have muta- account for the progressive cognitive decline. A diagnosis
tions in the gene that encodes a protein called presenilin 1. of definite Alzheimers disease can only be diagnosed at
This gene is found in chromosome 14. Presenilin 1 pro- autopsy by the presence of plaques and tangles (although
tein constitutes part of the gamma secretase complex. in some schemas tangles are not requisite) in an individ-
A smaller number of families have mutation of a related ual with a clinical history suggestive of dementia. The
gene on chromosome 1, which encodes a related protein, presence of plaques and tangles in typical brain regions
presenilin 2. Presenilin 2 can also participate in formation (mesial temporal, parietal, and inferior frontal structures)
of the gamma secretase. Mutations in the genes that is quite common in elderly persons with the clinical
encode APP, presenilin 1, and presenilin 2 all enhance syndrome of Alzheimers disease. As a result of the high
the production of Ab42. This has lent support to the prevalence of Alzheimers disease with advancing age (at
amyloid cascade hypothesis, which posits as Ab42 is least one commonly quoted study estimates approximate-
generated it begins to interfere with neuronal function, ly half of those over the age of 85 have it), the specificity of
kill neurons, and generate the other histologic features the clinical diagnosis is high. Recognition of how com-
seen in Alzheimers disease. While the logic underlying mon Alzheimers disease is in later life has also served to
this hypothesis is obvious, it is important to keep in mind enhance clinician awareness, thus improving sensitivity of
it assumes the very small subset of early-onset, autosomal the diagnosis. In the hands of an experienced physician,
dominant Alzheimers disease (which accounts for far less clinical diagnostic accuracy is excellent.
than 1% of those affected) have a similar if not identical Criteria originally designed to facilitate identification
etiology to the common sporadic, late-onset cases that of subjects for clinical trials have helped to standardize
constitute the vast majority. In those subjects, what initi- clinical diagnostic approaches. These criteria, such as
ates Ab42 production remains an open area of debate. those proposed by the National Institute of Neurologic,
Conceivably, population diversity in genes that contribute Communicative Disorders, and Stroke (NINCDS) and
to APP production or processing could cause Ab42 to the Alzheimers Disease and Related Disorders Associa-
appear. Environmental factors could lead to Ab42 forma- tion (ADRDA) in the 1980s emphasize the importance of
tion. Also, a variety of age-related factors promote Ab42 establishing that a progressive dementia exists in a patient.
formation. Two basic approaches are commonly used toward this
Other factors are recognized to play a role in Alzheimers end. One is to demonstrate a pattern of cognitive domain
disease, and where these factors fit into or what they tell us strengths and weaknesses that reliably suggest decline
about the etiologic hierarchy of the disease is unclear. One from a previous level of cognitive function has emerged.
factor relates to the APOE gene on chromosome 19. The For example, defective memory retention in the presence
APOE gene shows population variability due to the pres- of another defective cognitive domain (language, execu-
ence of two polymorphic positions. The common APOE tive function, visuospatial function, and praxis) in an
variants are the e2, e3, and e4 forms. The APOE e4 form elderly patient with cognitive complaints and an other-
is over represented in those with Alzheimers disease, wise unremarkable physical exam is strongly suggestive of
where it seems to move up the age of presentation in Alzheimers disease. The other approach focuses more on
those destined to develop the disorder. Mitochondrial defining the degree and nature of emerging declines in
function is also altered in Alzheimers disease, and these daily living activities. This latter technique focuses exten-
alterations are not limited to the brain. sively on collateral history obtained from family members
or friends of the patient.
The diagnosis is made primarily through clinical im-
Diagnostic Perspective pression, although that impression is influenced by a
small set of recommended laboratory and imaging tests.
Dementia is defined as cognitive decline that has ad- These tests are serologic (vitamin B12 level, thyroid func-
vanced to that point it interferes with activities of daily tion tests, electrolytes with renal and hepatic indices, and
living. While dementia has many different etiologies, a blood cell count) and structural (brain imaging by either
Alzheimers disease is the most common cause of demen- computed tomography or magnetic resonance imaging)
tia, accounting for 5060% of dementia verified by neu- in nature. As currently used, they mostly serve to rule out
ropathological examination of the brain at autopsy. The the presence of concomitant pathologies that can interfere
clinical diagnosis (i.e., diagnosis in life) of Alzheimers with cognition. Although this has contributed to the view
disease is made in patients who have progressive dementia that the Alzheimers disease diagnosis is one of exclusion,
108 A Alzheimers Disease
it should be noted that certain patterns of cognitive de- of action in Alzheimers disease has been questioned. In
cline elicited by clinical history or demonstrable by neu- any case, cohorts of patients with moderate or severe
ropsychological testing are so typical of Alzheimers Alzheimers disease, when randomized to memantine,
disease they can be used to support a diagnosis of inclu- perform better on measures of cognitive and functional
sion. It is important to note, though, that at the time of performance than do concurrent placebo treatment
this writing PET and APOE genotyping are not commonly groups. In severe Alzheimers disease, the magnitude of
used in the diagnosis of Alzheimers disease and cannot by observed benefit is similar to that obtained with donepe-
themselves establish a diagnosis of Alzheimers disease. zil. Memantine and donepezil have been studied in com-
bination with each other. Subjects with mini-mental state
exam scores of 514, who were already on donepezil, did
Treatment Perspective better as a group when memantine was added to their
treatment regimen than when placebo was added. De-
Although Alzheimers disease is currently neither revers- monstrable benefits in mild Alzheimers disease are lack-
ible nor curable, it is possible to treat its symptoms. The ing and thus the role of memantine in the mild stages of
first approved treatment for Alzheimers disease was tac- Alzheimers disease is not clear.
rine, a cholinesterase inhibitor. This drug increased levels A single study concluded high-dose vitamin E (2000 iu
of brain acetylcholine by antagonizing its synaptic degra- each day) might slightly slow decline in Alzheimers
dation. Increasing brain cholinergic tone was identified as disease patients. More recent general evidence, though,
a pharmacologic target because Alzheimers disease suggests taking more than 400 iu of vitamin E on a daily
patients show a profound loss of acetylcholine due to basis increases overall mortality. The marginality of any
degeneration of cholinergic neurons in the basal fore- vitamin E benefit, in conjunction with safety concerns, has
brain. Safer cholinesterase inhibitors (donepezil, rivastig- reduced enthusiasm for the use of vitamin E in Alzheimers
mine, and galantamine) have since superseded tacrine. In disease. Although a variety of other prescription medica-
addition to inhibiting acetylcholinesterase, rivastigmine tions (estrogens, statins), nonprescription medications
also inhibits buytrylcholinesterases that also hydrolyze (nonsteroidal anti-inflammatories), and nutraceuticals
acetylcholine, and galantamine is an allosteric modulator (gingko biloba) have been considered for the treatment
of acetylcholine nicotinic receptors. Each agent shows a of Alzheimers disease, published data to date on all other
similar overall degree of efficacy, although the individual treatment options has been at worst negative and at best
with Alzheimers disease may respond to or tolerate one insufficient to earn regulatory approval.
drug better than the other. Treatment cohorts followed for Other drug categories are commonly used to treat
12 weeks to 3 years indicate that as a group, those started targeted symptoms associated with Alzheimers disease.
on cholinesterase inhibitors tend to perform and appear For instance, antipsychotic medications are often used to
slightly improved compared to their immediate pretreat- treat agitated behavior. Some studies do show efficacy
ment baseline. This improvement appears detectable for in this regard, although other studies have argued the
612 months. By 12 months, though, treatment groups limited behavioral benefits antipsychotics may confer is
return to their pretreatment performance as ascertained canceled out by increased morbidity.
by cognitive testing, clinical impression, and caregiver
impression. Beyond 12 months, patients continuously
decline below their pretreatment baseline, although for Future Directions
at least the next several years patients appear to perform
better on cognitive testing than would otherwise be Scientific Perspective
expected. The clinical meaningfulness of this sustained
benefit has fueled considerable debate. Benefits have In the short term, considerable effort will be directed at
been observed on measures of cognitive ability, functional additional studies of Ab dynamics and homeostasis. Re-
ability, behavior, and caregiver stress. search will focus on the toxicities of different degrees of
At the time of this writing, memantine is the only Ab aggregation (especially oligomers, defined as short,
non-cholinesterase inhibitor specifically approved for soluble polymers of amyloid), cellular mechanisms of
the treatment of Alzheimers disease. Under in vitro con- Ab disposal, and tissue-level mechanisms of Ab disposal.
ditions, memantine blocks a cation channel associated Research over the longer term will need to address the
with the NMDA type of glutamate-activated ionotropic fact that the predominant etiologic hypothesis, the amy-
receptors. Whether or not this is its primary mechanism loid cascade hypothesis, cannot yet explain why Ab
Alzheimers Disease A 109
homeostasis changes in most of those affected or how Ab amyloid plaques and neurofibrillary tangles can be admi-
A
might give rise to other aspects of Alzheimers disease nistered intravenously, and the degree of brain ligand
pathology. It is possible the amyloid cascade hypothesis retention measured using PET. This approach can provide
will prove valid in those with early onset, autosomal an estimate of an individual patients plaque burden.
dominant Alzheimers disease caused by mutations of Development of techniques such as this will increasingly
the genes encoding APP, presenilin 1, and presenilin 2 pro- render the diagnosis of Alzheimers disease one of inclu-
teins, but not the late-onset cases (the vast majority). sion. Even so, this technology may, like others, turn out to
Disproving the amyloid cascade hypothesis in the serve best as an adjunct to the clinical diagnosis as op-
late-onset cases will likely require two events. First, inter- posed to the principal determinant of the diagnosis.
ventions that attempt to treat Alzheimers disease by The reason for this is that a substantial percentage of
targeting Ab will need to show absent or limited efficacy. nondemented individuals have relatively high plaque bur-
Second, other hypotheses better able to explain the overall dens. The significance of increased plaque burden in non-
Alzheimers clinical and pathological big picture will need demented individuals will need to be determined with
to demonstrate viability and durability. prospective long-term studies.
Because it will likely prove easier in the future to prevent None of the treatments approved for use in Alzheimers
neurodegeneration rather than reverse it, the ability to disease are approved for use in MCI, although available
render an early, accurate diagnosis is crucial. Also, the data argue cholinesterase inhibition (at least with done-
ability to treat the disease (either symptomatically or pezil) may provide a marginal benefit. Such a benefit
disease modifying) increases the importance of early di- would not be surprising, especially if MCI represents
agnosis. A confluence of neuropsychologic/clinical longi- very early Alzheimers disease in most people.
tudinal studies performed in conjunction with careful Over a decade of experience with symptomatic treat-
histopathologic correlation has already allowed a syn- ment has made it abundantly clear that disease-modifying
drome called mild cognitive impairment (MCI) to be de- treatments are required. Most current approaches toward
fined. MCI is known to represent a precursor of the disease modification are targeted to Ab homeostasis. In-
Alzheimer syndrome in the majority of those diagnosed hibition of its production (gamma secretase inhibitors
with it, and in more than half the MCI syndrome simply and modifiers), its targeted removal (active and passive
represents early Alzheimers disease. There is an emerging immunization approaches), prevention of its aggregation,
consensus that the line between normal age related cog- and enhancement of enzymatic degradation are all under
nitive decline and clinically excessive cognitive decline, at active pursuit. To date, a phase II Ab vaccination trial
least on an etiologic level, is a blurry one. Accordingly, by (AN1792) was halted when several of the subjects devel-
the time MCI is diagnosable in many individuals, substan- oped encephalitis. Other data obtained through this trial
tial irreversible brain change has occurred. Techniques and suggest the approach was successful in reducing cerebral
technologies for pushing the limits of the diagnosis to amyloid plaques. However, the most extensive published
stages that precede MCI are therefore needed. clinical data from AN1792 indicate that one year after
Most development toward this end focuses on the vaccination, the rate of cognitive decline was similar to
study of potential biomarkers. Biomarkers can be enti- (unchanged from or only very slightly reduced from) the
ties detectable in extractable tissues, such as blood or rate of decline shown by the placebo group of that trial.
cerebrospinal fluid (CSF). For example, CSF tau levels A phase III trial of tramiprosate, which retards Ab aggre-
increase in Alzheimers disease, while CSF Ab levels de- gation, was negative. A phase III trial of a gamma secretase
cline. When used in conjunction with fluorodeoxyglucose modifying agent (R-flurbiprofen) is underway. Phase III
PET, which shows the brains ability to consume glucose, trials of agents intended to humorally remove Ab are
investigators have been able to develop algorithms that scheduled.
predict future cognitive decline in elderly adults with If attacking Ab fails to meaningfully benefit Alzhei-
MCI, and even in individuals before they manifest cogni- mers disease patients, the validity of the amyloid cascade
tive complaints. hypothesis in late-onset, sporadic Alzheimers disease will
Biomarkers can also be demonstrated in vivo. For be called into question. If this happens, new models for
instance, ligands that bind amyloid plaques or both drug design will be needed. Currently, mice expressing a
110 A Alzheimers Disease
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Neurology, 42, 10971106.
other mutant human transgenes, serve as the gold
Klunk, W. E., Engler, H., Nordberg, A., Wang, Y., Blomqvist, G.,
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on Apolipoprotein E and Alzheimers Disease. The New England
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Stadlan, E. M. (1984). Clinical diagnosis of Alzheimers disease:
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distinction between Alzheimers disease and senile dementia: how National Institute on Aging, and Reagan Institute Working Group on
history can clarify nosology. Neurology, 36, 14971499. Diagnostic Criteria for the Neuropathological Assessment of Alzhei-
Blacker, D., & Tanzi, R. E. (1998). The genetics of Alzheimer disease: mers Disease. (1997). Consensus recommendations for the post-
current status and future prospects. Archives of Neurology, 55, mortem diagnosis of Alzheimers disease. Neurobiology of Aging, 18(4
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quantitative measures of dementia and of senile change in the Kokmen, E. (1999). Mild cognitive impairment: clinical characteri-
cerebral grey matter of elderly subjects. The British Journal of Psychi- zation and outcome. Archives of Neurology, 56, 303308.
atry, 114, 797811. Petersen, R. C., Thomas, R. G., Grundman, M, Bennett, D., Doody, R.,
Campion, D., Dumanchin, C., Hannequin, D., Dubois, B., Belliard, S., Ferris, S., et al. (2005) Vitamin E and donepezil for the treatment of
Puel, M., et al. (1999). Early-onset autosomal dominant Alzheimer mild cognitive impairment. The New England Journal of Medicine,
disease: prevalence, genetic heterogeneity, and mutation spectrum. 352, 23792388.
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Alzheimers Disease Cooperative Study ADL Scale A 111
month1month2 r = 0.94), and there was evidence of Galasko, D., Schmitt, F., Thomas, R., Jin, S., Bennett, D., & Ferris, S.
(2005). Detailed assessment of activities of daily living in moderate
convergent validity based upon the strong correlation
to severe Alzheimers disease. Journal of the International Neuropsy-
between ADCS-ADL-sev and other global impairment chological Society, 11, 446453.
measures (ADCS-ADL-sev MMSE r = 0.64; ADCS- Perneczky, R., Pohl, C., Sorg, C., Hartmann, J., Komossa, K.,
ADL-sev Severe Impairment Battery r = 0.71). The Alexopoulos, P., et al. (2006). Complex activities of daily living
mean score on first test was 25.4 (SD 12.7, maximum in mild cognitive impairment: Conceptual and diagnostic issues.
Age and Ageing, 35, 240245.
obtainable 54), with a mean decline of 5.6 points
(SD 7.5) over 6 months and 10.3 points (SD 10.3) over
12 months.
Perneczky et al. (2006) have found that the ADCS-
MCI-ADL scale can discriminate people with MCI from
control participants (a cut-off score of 52 gives sensitivity
Alzheimers Disease Co-operative
of 0.89 and specificity of 0.97). Study ADL Scale for Mild
Cognitive Impairment (ADCS-
ADL-MCI)
Clinical Uses
Alzheimers Disease Cooperative Study ADL Scale
The ADCS-ADL and its variants are the only ADL scales
designed with AD specifically in mind, and can provide a
fairly detailed assessment of competence in a variety of
ADLs. Galasko et al. (2005) state that the measure takes
too long to administer for it to be widely adopted in Alzheimers Disease Co-operative
clinical practice, but it would be useful in intervention
studies, and the ADL-sev in particular where the severity
Study ADL Scale for Severe
of the disorder may render measures such as the MMSE Impairment (ADCS-ADL-sev)
unsuitable due to floor effects. The careful selection of
items for the ADCS-ADL suggests that they are eminently Alzheimers Disease Cooperative Study ADL Scale
suitable for use in clinical trials. Perneczky et al. (2006)
found that even patients with a diagnosis of Mild
Cognitive Impairment exhibit deficits in instrumental
ADLs on the ADCS-ADL-MCI, and that scores can
successfully discriminate patients with MCI from healthy Amantadine
controls; as such, results from this scale may be useful in
forming an MCI diagnosis. Symmetril (Amantadine)
Cross References
Ambidexterity
Bristol Activities of Daily Living Scale
Disability Assessment for Dementia J OHN E. M ENDOZA
LawtonBrody iADL Scale Tulane University Medical Center
The Activities of Daily Living Questionnaire New Orleans, LA, USA
proficiencies or preferences are not uncommon, with men Pujol, J., Deus, J., Losilla, J. M., & Capdevila, A. (1999). Cerebral laterali-
zation of language in normal left-handed people studied by func- A
more frequently demonstrating such mixed preferences
tional MRI. Neurology, 52, 10381043.
than women. Tan (1988) found that approximately 66% Tan, U. (1988). The distribution of hand preference in normal men and
of the population was noted to express a strong right-hand- women. International Journal of Neuroscience, 41, 3555.
ed preference, while a little more than 3% were predomi-
nately left handed. The remaining 30% evidenced mixed
hand preferences. As noted elsewhere in this volume, hand-
edness is a common, but not the only measure of what is Ambiguous Personality
referred to as cerebral dominance. Another of the more Assessment
frequent indices of dominance is language, which is typically
organized primarily in the left hemisphere. While in the Projective Technique
majority of non-brain-injured individuals, the control
of both complex motor skills and language functions rest
within the left hemisphere, this may not always be the case,
particularly for those who are either left handed or ambi- American Academy of Clinical
dextrous. It has been shown that while right hemisphere Neuropsychology (AACN)
dominance for language is quite rare in right-handers, it
could approach 30% in strong left-handers. Individuals R EBECCA M C C ARTNEY
who are ambidextrous or whose parents are left handed Emory University/Rehabilitation Medicine
tend to fall somewhere in between these two groups with Atlanta, GA, USA
regard to the hemispheric localization of language. Further-
more, the localization of language may not be an all-or-none
phenomena. While one hemisphere may be more predomi- Membership
nant, language functions may be mediated to some extent by
both hemispheres. Individuals with mixed or anomalous American Academy of Clinical Neuropsychology
dominance, including those who were ambidextrous, tend (AACN) is an organization for psychologists who have
to have a greater incidence of at least some degree of bilateral achieved board certification in the specialty of Clinical
representation of language. In the event of unilateral strokes, Neuropsychology, under the American Board of Clinical
such individuals may evidence less severe residual aphasic Neuropsychology (ABCN). Membership in the Academy
deficits when compared to patients with strongly lateralized consists of three classes: Active, Senior, and Affiliate. Active
language when that hemisphere is affected. members are elected from among psychologists who have
been certified in clinical neuropsychology by the ABCN in
affiliation with the American Board of Professional Psy-
chology (ABPP). Senior members are elected from among
Cross References Active members who have been Academy members, for a
period of no less than the five preceding years, are age 65 or
Anomalous Dominance older or disabled, and are fully retired from the active
Dominance (Cerebral) practice of clinical neuropsychology. They continue to be
listed in the membership directory of the academy, and
they continue to receive any newsletters distributed to
Academy members. Senior members have no financial
References and Readings
obligations to the Academy and are allowed to continue
to subscribe to any journal available through the Academy.
Benson, D. F., & Geschwind, N. (1985). Aphasia and related disorders:
A clinical approach. In M. Mesulam (Ed.), Principles of behavioral At the time of this publication, there were 367 active senior
neurology (pp. 193238). Philadelphia: F.A. Davis Co. members in the United States and 20 members in Canada.
Knecht, S., Drager, B., Deppe, M., Bode, L., Lohmann, H., Floel, A., et al. Affiliate members are elected from among all others who are
(2000). Handedness and hemispheric language dominance in intellectually interested in the purposes of the Academy and
healthy humans. Brain, 123, 25122518.
wish to participate in its non-voting activities. All members
Pieniadz, J. M., Naeser, M. A., Koff, E., & Levine, H. L. (1983). CT scan
hemispheric asymmetry measurements in stroke cases with global are provided with a subscription to The Clinical Neuropsy-
aphasia: Atypical asymmetries associated with improved recovery. chologist, access to the AACN Clinical Discussion Email
Cortex, 19, 371391. List, and discounted fees to meetings and workshops.
114 A American Academy of Neurology (AAN)
through the Donald M. Palatucci Advocacy Leadership many other countries. Members include pediatricians,
Forum, and the Kenneth M. Viste, Jr., MD, Neurology pediatric medical subspecialists, and pediatric surgical
Public Policy Fellowship. Members also participate in the specialists. More than 34,000 members are board-certified
annual Neurology on the Hill visits to the USA Capitol in and called Fellows of the American Academy of Pediatrics
Washington, DC. The AANPAs BrainPAC political action (FAAP).
committee also is instrumental in representing neurol-
ogys interests on the federal level and supporting federal
legislators who support the profession and patients with
neurologic disorders.
Major Areas or Mission Statement
Cross References One of the AAPs major activities is to further the profes-
sional education of its members. Continuing education
Neuropsychiatry courses, annual scientific meetings, seminars, publica-
tions and statements from committees, councils, and sec-
tions form the basis of a continuing postgraduate
References and Readings
educational program.
More than 30 committees develop many of the AAPs
Visit the AAN online at www.aan.com.
positions and programs. Committees have interests as
varied as injury and poison prevention, disabled children,
sports medicine, nutrition, and child health financing.
American Academy of Pediatrics The AAP currently has six councils and 48 sections
consisting of more than 41,500 members with interest in
D EBBIE L INCHESKY specialized areas of pediatrics. This includes a section for
American Academy of Pediatrics resident physicians with more than 9,000 members. Sec-
Elk Grove Village, IL, USA tions and councils present educational programs for both
their members and the general membership of the AAP in
order to highlight current research and practical knowl-
Membership edge in their respective subspecialties.
The AAP publishes Pediatrics, its monthly scientific
The American Academy of Pediatrics (AAP) has approxi- journal; Pediatrics in Review, its continuing education
mately 60,000 members in the USA, Canada, Mexico, and journal; and its membership news magazine, AAP News.
American Board of Clinical Neuropsychology (ABCN) A 117
It also publishes manuals on such topics as infectious and oral examination. Because the diploma is based on
A
diseases and school health. In its public education efforts, peer review of clinical competency, the majority of ABCN
the AAP produces patient education brochures and a diplomates are active clinicians. However, many also en-
series of child care books written by AAP members. gage in clinical and basic science research, teaching, and a
The AAP executes original research in social, economic, wide range of other professional activities.
and behavioral areas and promotes funding of research. It The ABCN Board of Directors consists of 15 members
maintains a Washington, DC office to ensure that childrens elected by diplomates in good standing. The term of
health needs are taken into consideration as legislation and office is 5 years. Officers of the Board (President, Vice
public policy are developed. The AAPs state advocacy staff President, Secretary, Treasurer) are elected by the Board
provides assistance to chapters, promoting issues such as from among active elected directors. Elected Board mem-
child safety legislation and Medicaid policies that increase bers may serve no more than two consecutive terms.
access to care for low-income children. In addition to elected Board members, there is an exami-
nation chairperson, selected by the Board for a term of
5 years.
ABCN was initially established as an organization boards and recognition of special competencies of existing
solely charged with awarding diplomas to applicants board members. As a result, the Pediatric Special Interest
successfully demonstrating competency through the group was formed, and held the first meeting in 2009
examination process. In 1988, it became a membership during the AACN Conference.
organization and began charging dues so that resources Table 1 presents a timeline summary of major land-
for further development of the organization could be marks for ABCN.
built. This included creation and maintenance of a written
examination in consultation with Professional Examina-
tion Services (PES). After years of development and pilot Major Activities
testing to assure validity and reliability of the written
examination, in 1993, ABCN began to require that new ABCNs primary activities are developing, maintaining,
candidates pass the written examination prior to submit- and conducting the examination. The examination pro-
ting practice samples. The written examination is regular- cess consists of four distinct steps. First, the education and
ly reviewed for content updates to remove outdated items training experiences of the applicant are reviewed, initially
and assure that advances in clinical practice and knowl- at the ABPP central office, where the application is exam-
edge in the field are reflected in the examination. ined for graduate training, internship, and licensure sta-
The American Academy of Clinical Neuropsychology tus. Applications are then forwarded to ABCN for review
(AACN), an organization originally comprised of ABCN of advanced specialty training. Any practicing clinical
diplomates, was formed in 1996. ABPP had received legal neuropsychologist with a doctoral-level degree who pos-
advice that there was potential for conflict of interest in sesses a valid license to practice psychology is eligible to
the roles of credentialing bodies that also engaged in
advocacy. As a result, the academy was formed to fulfill
the advocacy and professional development role. AACN
has grown significantly and now includes an affiliate American Board of Clinical Neuropsychology (ABCN).
member category for neuropsychologists who have not Table 1 Timeline for major ABCN milestones
yet received their ABCN diploma, and for affiliated pro- 1981 ABCN incorporated in Minnesota
fessionals who are not neuropsychologists. Although
1983 First set of examinations completed
ABPP has recently received a different legal opinion that
1983 Formal affiliation between ABCN and ABPP
allowed member boards to once again merge with their
established
academies, ABCN and AACN have grown and function
1984 First ABCN/ABPP diplomates awarded
well in their complementary roles and at this time have no
plans to merge. 1988 ABCN bylaws revised to create membership
In 1997, a landmark conference regarding education organization
and training for clinical neuropsychologists was held in 1989 ABCN designated Specialty Council in Clinical
Houston (the Houston Conference on Specialty Educa- Neuropsychology by ABPP
tion and Training in Clinical Neuorpsychology). Attend- 1993 Written examination formally instituted
ing the conference were representatives from each of the 2002 AACN establishes mentoring program to promote
professional neuropsychology organizations, and the pro- board certification through ABCN
ceedings were published in 1998. In 2002, the ABCN 2002 ABCN votes to adopt Houston Conference guidelines
Board of Directors voted to adopt the Houston Confer- for eligibility for board certification, beginning in 2005
ence training guidelines as requisite training to be eligible 2002 Written examination updated to reflect Houston
for the ABCN diplomate. Candidates who received their Conference guidelines
degrees after January 1, 2005 are expected to have had 2004 500th ABCN diploma awarded
training and experience consistent with the guidelines laid 2004 BRAIN Website and Listserv group formed
out in the Houston Conference proceedings. 2005 Houston Conference education and training
In 2007, ABCN began to consider subspecialization requirements implemented
within the field, and address examination and recog- 2007 Committee to study subspecialization formed
nition of special competencies, such as pediatric neuro-
2009 700th diploma awarded
psychology. At that time, ABPP did not have a model for
2009 Pediatric Neuropsychology special interest group
subspecialization, and worked with ABCN to develop a
formed
framework to address issues such as overlap with other
American Board of Clinical Neuropsychology (ABCN) A 119
apply. Beginning in 2005, applicants for the ABCN presented with a brief description of a case, and is asked
A
diploma are expected to complete training consistent to inquire about background history, test data, and related
with the Houston Conference on Specialty Education medical information to arrive at a clinical diagnosis and
and Training in Clinical Neuropsychology. This includes conclusion. Along the way, the candidate may be asked
coursework in the areas outlined in the Houston Confer- about their rationale for test selection, their differential
ence, and completion of a formal 2-year postdoctoral diagnostic considerations, and how test results may
residency program in Clinical Neuropsychology. Howev- support or otherwise aid in diagnosis and treatment
er, recognizing that the field has evolved, applications planning. The professional and ethical portion of the
from candidates who obtained their graduate training examination is an opportunity for the candidate to dem-
prior to implementation of the Houston Conference stan- onstrate knowledge of important ethical considerations in
dards are evaluated according to the standards in place at the practice of neuropsychology, as well as discuss impor-
the time their degree was granted, provided they can tant issues for the field.
demonstrate that the pertinent requirements were met A comprehensive overview of the examination process
during their training (see www.theabcn.org for detailed was published in 2008 (Armstrong, et al., 2008).
requirement listings). Candidates who are respecializing Currently, ABCN conducts written examinations at
in neuropsychology, or who recently completed respecia- four major conferences each year:
lization programs, are expected to have education and
International Neuropsychology Society (INS North
training experiences consistent with the requirements in
American Meeting)
place at the time of their respecialization, not the date of
American Academy of Clinical Neuropsychology (AACN)
their original degree.
American Psychological Association (APA)
Once an applicants credentials have been reviewed
National Academy of Neuropsychology (NAN)
and accepted, the next step in the examination process is
a written examination. The written examination consists Oral examinations are conducted twice annually in
of 100 multiple-choice questions that cover a range of Chicago, Illinois, hosted by Rush University Medical Cen-
topics in neuropsychology. It is intended to evaluate the ter. One examination is conducted each autumn (usually
candidates breadth of knowledge and to assure that they late October, or early November) and the other in the
have the foundational knowledge necessary for competent spring (usually early May).
practice in clinical neuropsychology. It is administered The AACN holds an annual conference for continuing
at major conferences, including the AACN conference, education, professional development, and furthering the
National Academy of Neuropsychology (NAN) annual growth of the profession through advocacy. The meeting
meeting, International Neuropsychology Society (INS) is held annually in June.
North American Meeting, and the American Psychologi-
cal Association (APA) meeting. It was developed and is
maintained in association with PES.
Once a candidate has passed the written examination, Cross References
the next step is submission of a practice sample consisting
of two typical cases in the candidates practice. The prac- American Academy of Clinical Neuropsychology
tice samples are reviewed by at least three independent, (AACN)
board certified neuropsychologists. American Board of Professional Psychology (ABPP)
Following acceptance of the practice sample, the can- American Psychological Association (APA)
didate is invited to sit for the oral examination that con- International Neuropsychology Association (INS)
sists of three parts practice sample, fact-finding, and Meier, Manfred John (19292006)
ethics/professional development. The practice sample sec- National Academy of Neuropsychology (NAN)
tion of the orals provides the candidate an opportunity to
discuss their practice as applied to the specific cases they
submitted. The cases also serve as a starting point leading
References and Readings
to more in-depth discussion of differential diagnosis, and
general information about the nature of the disorder in
Armstrong, K., Beebe, D. W., Hilsabeck, R. C., & Kirkwood, M. W.
the case and related conditions. The fact-finding section (2008). Board certification in clinical neuropsychology: A guide to
of the oral examination is an opportunity for the candi- becoming ABPP/ABCN certified without sacrificing your sanity.
date to demonstrate clinical skills. The candidate is Oxford Press.
120 A American Board of Pediatric Neuropsychology
Bieliauskas, L. A., & Matthews, C. G. (1987). American Board of Clinical training in pediatric neuropsychology (from graduate
Neuropsychology: Policies and procedures. The Clinical Neuropsy-
school to continuing education), written examination, a
chologist, 1, 2128.
Bieliauskas, L. A., & Matthews, C. G. (1990). American Board of Clinical
practice sample submission, and an oral examination. The
Neuropsychology Update, 1990. The Clinical Neuropsychologist, 4, ABPdN does not have a grand fathering policy, and
337343. thus, all existing board members were required to com-
Bieliauskas, L. A., & Matthews, C. G. (1997). The American Board of plete all new phases of the examination process to ensure
Clinical Neuropsychology, 1996 update: Facts, data, and information
equality of standards among boarded members.
for potential candidates. The Clinical Neuropsychologist, 11, 222225.
Hannay, H. J., Bieliauskas, L., Crosson, B. A., Hammeke, T. A., Hamsher,
As of early 2010, 111 neuropsychologists have submit-
K. deS., & Koffler, S. (Eds.). (1998). Proceedings of the Houston ted applications to ABPdN and 75 members have passed
Conference on specialty education and training in clinical neuorp- the ABPdN examination process. At present, there are
sychology. Archives of Clinical Neuropsychology, 13, 157250. 57 active and five emeritus members of the board from
Ivnik, R. J., Haaland, K. Y., & Bieliauskas, L. A. (2000). American Board of
21 states, Canada, and Puerto Rico.
Clinical Neuropsychology special presentation. The Clinical Neurop-
sychologist, 14, 261268.
Report of the Division 40/INS Joint Task Force on Education, Accredita-
tion, and Credentialing (1984). Division 40 Newsletter, Vol. 2, no. 2,
Major Areas or Mission Statement
pp. 38.
Reports of the ins - division 40 task force on education, accreditation, and Board certification in pediatric neuropsychology serves to
credentialing (1987). The Clinical Neuropsychologist, 1(1), 2934. assist consumers by offering supportive evidence of the
Yeates, K. O., & Bieliauskas, L. A. (2004). The American Board of
competence of the pediatric neuropsychologists. The
Clinical Neuropsychology and American Academy of Clinical
Neuropsychology: Milestones past and present. The Clinical Neurop-
ABPdN is the only board certification organization with
sychologist, 18, 489493. the sole purpose of examining and certifying competence
in pediatric neuropsychology.
Landmark Contributions
American Board of Pediatric
Members of ABPdN practice in a variety of settings includ-
Neuropsychology ing universities, teaching hospitals, general hospitals, hos-
pital trauma centers, private practices, rehabilitation
P ETER D ODZIK facilities, stroke centers, memory disorder centers, group
American School of Professional Psychology-Schaumburg practices, and child development centers. Current members
Schaumburg, IL, USA hold academic affiliations at over 40 colleges and universi-
ties. Several members have developed tests commonly used
in the practice of pediatric and general neuropsychology.
Membership Member accomplishments include past president of APA
Division 40, current and past presidents of four State Psy-
The American Board of Pediatric Neuropsychology chology Boards, past president of National Academy of
(ABPdN) was developed in 1996 by a coalition of clinical Neuropsychology, past and present editor of Archives of
practitioners, representing institutions hiring pediatric Clinical Neuropsychology, past editor of Journal of School
neuropsychologists. The original group conceived the Psychology, and the owner/moderator of PEDS-NPSY, a
board to advance their belief that a unique interplay pediatric list-serve with over 1,600 members.
exists between neurodevelopmental issues and neuropsy-
chological assessment that requires special sets of exper-
tise not readily assessed by the then existing boarding Major Activities
entities. Following discussion with colleagues who were
members of medical practice and psychology boards, The ABPdN is the board-certifying arm of the American
the coalition elected to establish an independent certi- Academy of Pediatric Neuropsychology (AAPN), which is
fying authority. The examination process evolved into devoted to training and promotion of the field of pediat-
a comprehensive and multilevel process that includes a ric neuropsychology. The AAPN, in affiliation with the
written application including clinical case vignettes used American College of Professional Neuropsychology, holds
to determine decision-making strategies of the applicant, an annual conference each spring with topics related to
scope of practice and a thorough assessment of organized the field of pediatric neuropsychology.
American Board of Pediatric Neuropsychology A 121
reanalyzed, debated, approved, and then compiled into a ability to articulate the major findings and their rationale.
larger item pool for random selection by domain each year. Candidates discuss their rationale in such areas as:
A passing score of 70% is required. Each exam includes the
(1) Test selection (if applicable): psychometric proper-
following basic core areas:
ties, test validity/reliability, limitations for use, and
Psychometrics exclusion of all competing diagnoses.
Pediatric Neurosciences (2) Test interpretation (if applicable): alternate interpre-
Psychological and Neurological Development tations of findings, conflict resolution within the
Neuropsychological and Neurological Diagnostics data, discussion of strengths and weaknesses, and
Ethics and Legal Issues environmental and cultural factors.
Research Design Review for Clinical Application (3) Diagnostic conclusions: alternate diagnosis, ultimate
Intervention Techniques understanding of neuropathology, prognosis, pro-
Consultation and Supervisory Practices gression, lateralizing/localizing effects, pathognomic
signs, causality, environmental conditions, and
effects on neural development.
Oral Examination
(4) Recommendations and treatment planning: best
practices for treatment, availability, prognosis, fund-
This part of the examination process is comprised of a
ing, delivery options, cost/benefit analysis, iatrogenic
review of the candidates practice sample, the nature and
outcomes, parental compliance/agreement, and ethi-
application of neuropsychological knowledge to their cur-
cal issues.
rent practice, appreciation for ethical issues and obligations,
(5) Consultation and supervision (if applicable): best
and a review of the candidates views and philosophy on
practices for communication of data, delivery
pediatric neuropsychology. The oral examination also
options, supervisee needs/relationships, and rap-
includes a mock case review, in which the candidate is
port/therapeutic relationship.
given information about a fictional case, and they develop
and articulate their working hypothesis. The oral examina- This process is intended to be collegial and the examiners
tion is intended to be a collegial opportunity for the endeavor to be sensitive to the different and yet equally
reviewers to validate the candidates ability to think on viable approaches within pediatric neuropsychology. The
their feet and discern their preparation and readiness for purpose is to ascertain the Candidates logic and thought
board certification. processes and to allow them to demonstrate these skills.
The first portion of the oral examination permits the During the ethics segment, there is discussion of one or
examination team to consider the scope of the candidates two standardized vignettes, and the candidate is expected to
body of training and how they practice pediatric neuro- present relevant comments on the ethical dilemmas,
psychology (e.g., acute care, rehabilitation, outpatient, thoughtfully weighing them in the light of the APA ethics
assessment, and/or treatment) so that the fact-finding principles, professional practice standards, and relevant
and practice sample review can be conducted in the most statutes.
relevant fashion. This section is broken into two parts:
Part I: The examinee will explain their background.
Cross References
The examinee will provide a history of their educational
and professional background. Special consideration American Psychological Association (APA), Division 40
should be given to their pediatric neuropsychological National Academy of Neuropsychology (NAN)
training and background.
The examinee will explain their current role as a
pediatric neuropsychologist and the issues their typi- References and Readings
cal clientele present.
ASPPB National Register designation committee (2008). Retrieved
Part II: The examinee will demonstrate pertinent knowl- October 1, 2009 from http://www.nationalregister.org/designate_
edge of practical pediatric neuropsychology. stsearch.html
Beljan, P., Bos, J., Courtney, J., & Dodzik, P. (2006). Preparation guide for
The next segment of the oral examination allows the
examination and certification by the American Board of Pediatric
candidate to present the material in their practice sample Neuropsychology. Retrieved from http://abpdn.org/docs/study-
and to provide an overview of the history, evaluation pro- guide.pdf
cess, and outcome of the case. The examiners evaluate their For additional information please see the web site at www.abpdn.org.
American Board of Professional Psychology (ABPP) A 123
specialty board certification and serves as a gold standard For interested applicants, it contains application instruc-
for demonstration of specialty competency in professional tions as well as other helpful information. The organiza-
psychology. tion will publish its first book, Becoming Board Certified by
the American Board of Professional Psychology (ABPP) in
2009.
Landmark Contributions
who have a need for applied neuropsychological services, locations throughout the country. A workshop on the
a process whereby competent professional neuropsychol- ABN examination process is held at least once a year.
ogists can be identified. Individual candidate mentoring is offered throughout
To achieve the standards set forth by the ABN for the year.
competent professional practice of neuropsychology, the
following outcome objectives have been developed:
References and Readings
Validate the skills of clinical practitioners
Identify competent practitioners http://www.neuropsychologyboard.org/
Provide public information about professional Bennett, T. L., Horton, A. M., Jr. & Elliott, R. W. (1999). American Board
of Professional Neuropsychology (ABPN). Bulletin of the National
neuropsychology
Academy of Neuropsychology, 14, 79.
Document the maintenance of competence of profes- Elliott, R. W., & Horton, A. M., Jr. (1994). Philosophy of the American
sional neuropsychology practitioners with continuing Board of Professional Neuropsychology. Bulletin of the National
education requirements Academy of Neuropsychology, 11, 1415.
Provide individuals, organizations, and agencies who Elliott, R. W., & Horton, A. M., Jr. (1995). History and current status of
the American Board of Professional Neuropsychology. The Indepen-
use neuropsychology services with a referral directory
dent Practitioner, 15, 175177.
of ABN diplomates Goldstein, G. (2001). Board certification in clinical neuropsychology:
Some history, facts and opinions. Journal of Forensic Neuropsychol-
Recognition by ABN signifies to the public and to other
ogy, 2, 5765.
health professionals a high level of competency in applied Horton, A. M. Jr., Crown, B. M., & Reynolds, C. R. (2001). American
neuropsychology. The ABN does not ascribe to any spe- Board of Professional Neuropsychology: An Update-2001. Journal of
cific theoretical framework. While recognizing the impor- Forensic Neuropsychology, 2, 6778.
tance and contribution of a graduate education in
neuropsychology and subsequent specialty training, the
ABN believes that the critical element in the practice of
professional neuropsychology is the application of that American Board of Rehabilitation
training to client issues and needs. Psychology
DANIEL E. R OHE
Landmark Contributions Mayo Clinic
Rochester, Minnesota
Applied Neuropsychology, a peer reviewed edited
journal, is the official journal of the ABN.
Membership
Frank, R., Rosenthal, M., & Caplan, B. (Eds.). (2009). Handbook of In addition to the continuing education benefit, ACPN
rehabilitation psychology (2nd ed.). Washington, DC: American Psy- also has an official quarterly journal, Applied Neuropsy-
chological Association.
chology, which is dedicated to the presentation of
Nezu, C., Finch, A., Jr., & Simon, N. (Eds.). (2009). Becoming
board certified by the American board of professional psychology.
practitioner-based scholarly research.
New York, NY: Oxford University Press, Inc. Diplomates of the ABN who are in good standing are
automatically Fellows of ACPN and may use the acronym
FACPN on their signature line. Members of other neuro-
psychological organizations may also join the ACPN as
Affiliate members and receive a subscription to Applied
American College of Professional Neuropsychology, and participate in ACPN continuing
Neuropsychology (ACPN) education programs.
Membership
American Congress of
The American College of Professional Neuropsychology
Rehabilitation Medicine
(ACPN) is a membership organization formed on
September 1, 1995 that is composed of 350 (2009) Neu-
M ARCEL P. J. M. D IJKERS
ropsychologists who have doctoral degrees, are licensed
Mount Sinai School of Medicine
as psychologists, and have completed the Diplomate
New York, NY, USA
examination process.
Membership
Major Areas or Mission Statement
Membership is about 800, consisting of clinicians and
The academic arm of the American Board of Professional nonclinicians with an interest in medical rehabilitation
Neuropsychology (ABN) is the ACPN. The mission of the research, and training in medicine, psychology, occupa-
ACPN is to promote and provide the highest levels of tional and physical therapy, nursing, speech and language
services related to professional neuropsychology, for the pathology, political science, etc. Medical rehabilitation
benefit of the public and the profession. concerns restoration of function for individuals who as a
American Congress of Rehabilitation Medicine A 129
result of stroke, traumatic brain injury, spinal cord injury, 2008). Reflecting the ongoing differentiation between
A
amputation, and other disorders have impairments and radiologists and what (much later) would be called
activity limitations that are primarily physical in nature, physiatrists, the name was changed to American Congress
but often also include cognitive and behavioral deficits; it of Physical Therapy in 1925. To emphasize its link to
is to be distinguished from psychiatric rehabilitation, medicine rather than allied health, the organization
addictions rehabilitation, etc., although there is overlap renamed itself American Congress of Physical Medicine
in methods and sometimes clientele. Members share an in 1944.
interest in rehabilitation research, and the translation of While World War I had given rise to the development of
research-based knowledge into formats that are of use to rehabilitation, the involvement of physicians had been
medical rehabilitation clinicians. About 70 members are limited rehabilitation was centered on the vocational
located outside the USA, especially in Canada. rehabilitation of discharged servicemen. During and after
World War II, however, a number of physicians became
specialists in rehabilitation and started to apply methods
Mission Statement they had used with servicemen to the treatment of civilians
with amputations, spinal cord injury, stroke, and develop-
The mission of the American Congress of Rehabilitation mental disabilities such as cerebral palsy. To avoid the
Medicine is to enhance the lives of persons living with creation of a separate organization involving physicians
disabilities through a multidisciplinary approach to reha- with very similar interests and therapeutic regimens, a
bilitation, and to promote rehabilitation research and its shotgun marriage between physiatrists and rehabilita-
application in clinical practice (About ACRM, 2008). tion physicians was acknowledged in 1952 with expansion
The American Congress of Rehabilitation Medicine of the name of the organization to American Congress of
serves people with disabling conditions by promoting Physical Medicine and Rehabilitation (Zeiter, 1954).
rehabilitation research and facilitating information dis- In the 1960s, the Congress opened its membership to
semination and the transfer of technology. We value re- nonphysician rehabilitation professionals, first only those
habilitation research that promotes health, independence, holding a doctoral degree (1965), then also to nurses and
productivity, and quality of life for people with disabling therapists with an (earned) masters degree (Anonymous,
conditions. We are committed to research that is relevant 1998). To acknowledge the diminishing emphasis on
to consumers, educates providers to deliver best practices, physical medicine, the Congress changed its name again,
and supports advocacy efforts that ensure adequate public to American Congress of Rehabilitation Medicine, in
funding for our research endeavors (About ACRM, 1966. ACRM accepted rehabilitation professionals with a
2008). bachelors degree as members starting in 1986. The first
To develop and implement our vision, ACRM will nonphysician to become president of the organization
seek the involvement of rehabilitation professionals, in- took office in 1977; neuropsychologists who have served
cluding clinicians, senior level service managers, admin- as president include Leonard Diller, Mitchell Rosenthal,
istrators, educators, and researchers. We will call upon the and Wayne Gordon.
leaders in rehabilitation to identify current best practices In recent years, ACRM has redefined itself as an orga-
and best providers at all levels of care. We will disseminate nization focusing on rehabilitation science, with strong
this information to the field at our regional and national interest in both generating knowledge through research
meetings, through directed position papers, and in our and knowledge translation to bring research results to the
journal, Archives of Physical Medicine and Rehabilitation clinic in a format that practitioners can use (Hart, 1997;
(About ACRM, 2008). Heinemann, 2006; Wilkerson, 2004). It now is primarily
a group of creators, transmitters, and consumers of
research-based rehabilitation knowledge, both those
Landmark Contributions with clinical training (physicians, occupational and phys-
ical therapists, psychologists, etc.) and those without
The American Congress of Rehabilitation Medicine was (engineers, political scientists, etc.), bound by the convic-
established in 1923 as the American College of Radiology tion that collaboration of disciplines is the best way to
and Physiotherapy, a professional organization of physi- solve the problems inherent in disablement and the reha-
cians who had a clinical interest in diagnostic and thera- bilitation of persons with impairment, activity limita-
peutic radiology, as well as the therapeutic application of tions, and participation restrictions. The insignia of the
electricity and other physical therapies (About ACRM, organization still reflects ACRMs roots in physical
130 A American Congress of Rehabilitation Medicine
medicine, including the traditional symbols for the four grant programs supported by the National Institute on
elements: water, earth, fire, and air. Disability and Rehabilitation Research since 1987). There
also is considerable overlap between the membership of
the BI-ISIG and Divisions 22 (Rehabilitation Psychology)
and 40 (Clinical Neuropsychology) of the American Psy-
Major Activities
chological Association. The BI-ISIG publishes a newslet-
ter, Moving Ahead. Intense collaboration in research and
ACRM communicates with its members through its
clinical care occurs among the BI-ISIG members, who
scientific journal (the Archives of Physical Medicine and
have their own task forces and come together in an addi-
Rehabilitation APM&R), a newsletter (Rehabilitation
tional annual meeting.
Outlook) and weekly E-news, an electronic digest of
APM&R began in 1920 as the Journal of Radiology, the
time-sensitive news. An annual scientific meeting of 34
private property of a Dr. Albert A. Tyler (Cole, 1999). The
days, often held jointly with other scientific and profes-
journal changed its name to the Archives of Physical Ther-
sional organizations, brings together members and non-
apy, X-ray, Radium, in 1926; in 1930, Dr. Tyler gave the
members to discuss research findings, research methods,
journal to ACRM (then still named the American Con-
and issues relevant to the funding, implementation, and
gress of Physical Therapy) as a debt-free, unencumbered
dissemination of rehabilitation research.
gift. The later changes in the name of the journal parallel
A number of standing committees offer members an
the changes in the name of its owner. It became the
opportunity to work on issues of special interest. Current
Archives of Physical Therapy in 1938, the Archives of
committees include the International Committee (focus-
Physical Medicine in 1945; in 1953, the journal became
ing on the communications between US and foreign re-
the Archives of Physical Medicine and Rehabilitation, the
habilitation research specialists), the Clinical Practice
name it still has (Nelson, 1969). However, the content has
Committee (dealing with issues of evidence-based prac-
shifted gradually from emphasis on physical medicine,
tice and related matters), and the Involving Consumers in
with a fairly low research basis, to an accent on rehabilita-
Rehabilitation Research Committee. The Early Career
tion as carried out by all disciplines that play a role in
Committee aims to assist individuals new to rehabilita-
medical rehabilitation. It now is almost exclusively a re-
tion research in mastering the scientific, administrative,
search journal, with non-US contributions constituting
and personal aspects of a career in rehabilitation research.
over half the contents (Dijkers, 2009).
Over the years, a number of interdisciplinary special
The journal probably gives the best indication of the
interest groups (ISIGs) have existed under the aegis of
role of neuropsychology in rehabilitation settings, and of
ACRM; current groups include ISIGs focused on spinal
neuropsychologists in ACRM. The first paper with neu-
cord injury, stroke, the measurement of participation, and
ropsycholog* in its title or abstract was published in 1975.
traumatic brain injury.
Almost 200 have been published since, but they did not
The Brain Injury ISIG (BI-ISIG) grew out of the
become an annual presence until 1984. The number now
ACRM Head Injury Task Force, first called together in
averages ten a year. In scanning the contributions of
1979. The BI-ISIG, which attracts large numbers of psy-
neuropsychologists to APM&R, a number of characteristics
chologists and especially neuropsychologists, has played a
of neuropsychology in rehabilitation stand out:
crucial role in the development of services for individuals
with traumatic brain injury (TBI) in the United States. A Many of these papers are coauthored with representa-
definition of mild TBI often used in the literature emerged tives of other disciplines, especially physicians.
from the work of this group (American-Congress-of- Several straddle neuropsychology and rehabilitation
Rehabilitation-Medicine.-Head-Injury-Interdisciplinary- psychology, reflecting the fact that in many rehabilita-
Special-Interest-Group, 1993). The Journal of Head Trau- tion programs psychologists need to wear multiple
ma Rehabilitation (JHTR) was founded by a physician hats.
(Sheldon Berrol) and a psychologist (Mitchell Rosenthal) The focus, especially in recent years, is as much on
who were active in the BI-ISIG, as well as involved with treatment as on diagnosis, with cognitive rehabilita-
the fledgling National Head Trauma Foundation, now the tion for TBI and other diagnostic groups most
Brain Injury Association of America. There is significant prominent.
overlap between the BI-ISIG membership and both the A great variety of diagnostic groups have been studied,
Editorial Board of JHTR and the leadership of the TBI including those with peripheral vascular disease
Model Systems of Care (demonstration and research amputations, post-polio fatigue, multiple sclerosis,
American Psychological Association (APA) A 131
sickle-cell disease, progressive supranuclear palsy, Wilkerson, D. L. (2004). Individual, science, and society: ACRMs mis-
sion and the body politic. Archives of Physical Medicine and Rehabil- A
myotonic muscular dystrophy, and spinal cord injury.
itation, 85(4), 527530.
However, over the years and especially recently, stroke Zeiter, W. J. (1954). The history of the American Congress of Physical
and TBI have been the etiologies of disability that Medicine and Rehabilitation. Archives of Physical Medicine and
rehabilitation neuropsychologists have most often Rehabilitation, 35(11), 683688.
been concerned with.
While the American Congress of Rehabilitation Med-
icine is not an organization of psychologists, let alone
neuropsychologists, it is safe to say that it has played
American National Adult Reading
a key role in the development of neuropsychology for Test (ANART)
medical rehabilitation patients in the United States. In
the foreseeable future, it probably will continue to be the Weschlers Adult Reading test
forum in which these specialists, especially those who are
interested in research, interact with nurses, speech/lan-
guage pathologists, neuroscientists, and other specialties
that contribute to rehabilitation.
American Psychological
Association (APA)
WADE P ICKREN
References and Readings Ryerson University
Toronto, ON, Canada
About ACRM. (2008). Retrieved August 25, 2008, from http://www.acrm.
org/about/index.cfm
American-Congress-of-Rehabilitation-Medicine.-Head-Injury-Interdisci-
plinary-Special-Interest-Group. (1993). Definition of mild trau- Address and URL
matic brain injury. Journal of Head Trauma Rehabilitation, 8(3),
8687. 750 First Street NE, Washington, DC 20002-4242 (www.
Anonymous. (1998). Development of the American Congress of Rehabil- apa.org)
itation Medicine into a multidisciplinary professional society: Final
report of the Professional Development Committee, 19691972.
Archives of Physical Medicine and Rehabilitation, 79(12 Suppl. 2),
412. Membership
Cole, T. M. (1999). ACRM presidential address. In the clothing of chal-
lenge. American Congress of Rehabilitation Medicine. Archives of 150,000 as of 2010
Physical Medicine and Rehabilitation, 80(2), 127129.
Dijkers, M. P. (2009). International Collaboration and Communication
in Rehabilitation Research. Archives of Physical Medicine and
Rehabilitation, 90(5), 711716.
Major Areas or Mission Statement
Hart, K. A. (1997). Rehabilitation research: The new focus of the Ameri-
can Congress of Rehabilitation Medicine. Archives of Physical Medi- The mission of the APA is to advance the creation, com-
cine and Rehabilitation, 78(12), 12871289. munication, and application of psychological knowledge
Heinemann, A. W. (2006). ACRMs evolving mission: Opportunities to to benefit society and improve peoples lives.
promote rehabilitation research. Archives of Physical Medicine and
Rehabilitation, 87(2), 157159.
Kottke, F. J., & Knapp, M. E. (1988). The development of physiatry before
1950. Archives of Physical Medicine and Rehabilitation, 69 Spec No,
414.
Landmark Contributions
Krusen, F. H. (1969). Historical development in physical medicine and
rehabilitation during the last forty years. Walter J. Zeiter Lecture. The American Psychological Association (APA) was
Archives of Physical Medicine and Rehabilitation, 50(1), 15. founded in 1892 by a small group of men interested in
Nelson, P. A. (1969). History of the Archives A journal of ideas and what was called the new psychology. Its founding at this
ideals. Archives of Physical Medicine and Rehabilitation, 50(7),
particular time can best be understood as part of the large
367405.
Rusk, H. A. (1969). The growth and development of rehabilitation
number of changes occurring in the USA at that time.
medicine. Archives of Physical Medicine and Rehabilitation, 50(8), The emergence of a number of what are now standard
463466. academic disciplines, psychology, economics, political
132 A American Psychological Association (APA)
science, biochemistry, physiology, in the last 2 decades association that was much more broadly based than
of the nineteenth century was part of a reorganization of before the War and that was organized around an increas-
American knowledge production, reflecting a division of ingly diffuse conceptualization of psychology. Now, the
intellectual labor similar to the division of manufactory associations scope included professional practice and
labor. Like its fellow disciplines, the new psychology grew the promotion of human welfare, as well as the practice
and prospered as it responded to the needs of American of the science of psychology. This flexibility in scope has
society. remained to the present time, as new challenges and
Within the modern university system that emerged demands have arisen.
after the U.S. Civil War, the new disciplines quickly Psychology boomed after the end of World War II,
developed advanced degrees that provided credentials, with the greatest increase in membership coming between
which served to validate the disciplines members as 1945 and 1970. This was due to intense interest in the
experts in their special field. This occurred in parallel field, especially in the domains of clinical and applied
with the progressive movement in politics, which called psychology, among returning serviceman, many of
for a more efficient, less corrupt, social order. The whom saw the great need for better psychological services
synergism of these two developments, specialized exper- firsthand during the war. Institutional or structural fac-
tise and rationalized government, helped create the de- tors that facilitated this growth included the GI Bill, the
mand for trained personnel to fill the new professional new Veterans Administration Clinical Psychology training
niches created by the demands for a more efficient program, and the creation of the National Institute of
society. Psychology was one of the most successful of Mental Health. For the first time, psychology was a field,
the new disciplines to make itself useful for the social both science and practice, that was richly funded for
management of an increasingly complex and diversified training and research. This was, as one scholar termed it,
society. The Golden Age of Psychology. The rapid and incredible
In July 1892, G. Stanley Hall (18441924) met with a growth in APAs membership reflected this trends, as
small group of men to discuss the possibility of organizing membership grew 630% from 1945 to 1970, from 4,183
a psychological association. Although the details of the members (1945) to 30,839 (1970). By comparison, from
meeting are not known, the group elected 31 individuals, 1970 to 2000, APA membership grew to 88,500, with
including themselves, to membership, with Hall as the another 70,500 affiliates.
first President. The first meeting of the new American Part of what facilitated this growth was the new divi-
Psychological Association (APA) was held in December sional structure of the APA that grew out of the reorgani-
1892 at the University of Pennsylvania. The basic gover- zation plan during World War II. Now, members could
nance of the APA at this time was consisted of a small join a special interest group within APA and find other
council with an executive committee. This plan remained like-minded members. Of course, this also facilitated the
in effect until the reorganization of APA during World fractionation of psychology and pushed the field away
War II. from any sense of unity that it may have held prior to
Membership growth of the APA was modest over the the war. Nineteen divisions were approved in 1944, with
first 50 years of its existence. From 31 members in 1892, the two most numerous being clinical and personnel (now
there were 125 members in 1899, 308 in 1916, 530 in 1930, counseling). This reflected the sectional structure of the
and 664 in 1940. In 1926, a new class of nonvoting American Association of Applied Psychology (AAAP, f.
membership was formed, associate, and most of the 1937), which had emerged in 1937 as the chief rival to the
growth occurred in that class after 1926, so that there APA and had been the chief reason for the reorganization.
were 2,079 associate members in 1940. Many of these Because the Psychometric Society (Division 4) decided
associates were individuals doing practical or applied not to join and after Division 11, Abnormal Psychology
work in psychology and who also belonged to one of the and Psychotherapy, merged with Division 12, Clinical
applied associations that emerged in this time. Realizing Psychology, the number of active divisions was reduced
that the growth of applied psychology represented to 17. Growth in the number of divisions was slow until
a potential threat to its preeminence, the leaders of APA the 1960s, only three more were added, in part because
sought to reorganize the association during World War II. many of the older members, then in leadership positions,
Under this reorganization plan, the APA merged with were quite resistant to increasing the number of divisions.
other psychological organizations and created divisions The growth in the number of divisions since the 1960s has
to represent special fields of interest. There were initially been consistent, with 54 divisions now part of the APA
17 divisions (19 were proposed). The result was an structure. Many of the newer divisions reflect the growth
American Psychological Association (APA) A 133
of particular practice areas, for example, Division 50, the mid- to late 1980s, as tensions between those who
A
Addictions. However, there has also been growth in spe- wanted APA to remain a primarily scientific organization
cial interest areas that belie any simple science/practice and those who sought a greater emphasis by the associa-
dichotomy, for example, Society for the Psychology of tion on professional practice rose to a boil. A proposed
Women, Society for the History of Psychology, Interna- reorganization plan was defeated by a vote of the mem-
tional Psychology, Media Psychology, or the Study of Men bership and almost immediately a large group of dissident
and Masculinity. psychological scientists, including former APA Presidents,
left the APA to form what is now the Association for
Psychological Science (APS). Still, after a period of strug-
Major Activities gle, both organizations are strong, stable representatives
of psychology, with many psychologists belonging to both
The effect on APA governance of the divisional structure associations.
and the growth of state and provincial psychological One result of the split that led to the formation of
organizations has been marked. As mentioned, prior to APS is that professional interests have grown stronger
World War II, APAs governance structure was a small within APA. As the number of psychologists devoted to
council with an executive committee. After the reorga- professional practice grew and gained greater influence
nization and the end of the war, the Council of Repre- in the APA governance structure, a new unit was estab-
sentatives has grown in number to accommodate lished in the APA Central Office. The Office of Profes-
representation from each division and from state and sional Practice was created in the mid-1980s with a
provincial psychological associations, thus making gover- mandate to focus on applied practice activities, especial-
nance somewhat unwieldy. Various plans have been tried ly the promotion of health-care practice. To finance the
over the years to ensure a voice for each of the areas and expansion of activities, a special assessment was levied
interests groups in psychology on the council and it on psychologists licensed for health-care practice. With
remains a dynamic situation. One result of the growth this money, the office was able to engage in consultation,
of professional psychology, especially clinical and technical assistance, and legal and legislative assistance
counseling psychology, on governance has been the in- for professionals. The office also began to work closely
crease in the representation of professional interests, for with state associations to enhance practice issues and
example, licensing, specializations, etc., in the delibera- support efforts relevant to legislation in state legislatures.
tions of the council. At times, this has led to tension Within a few years, the range of activities led to the need
between the representatives of psychological science and to create the Practice Directorate within APA. Since that
those whose main commitment is to advancing time, the Practice Directorate has played the important
professional practice. In historical retrospect, it seems roles of handling all practice-related programs and has
clear that this tension was inherent in the reorganization been responsible for the coordination of practice efforts
of APA, as the association reflected developments in the in legal and legislative arenas. The special assessment and
field. the Practice Directorate represented a special moment
As a membership organization, APA has often been in APAs history in that they enhanced the power of
perceived as inadequately representing one or more clinical and professional practice both within and
its constituencies. It has been the case, more often than without APA.
not, that the resulting tension was resolved and the un- Even so, APA has maintained a commitment to the
happy parties remained within the association. However, promotion of psychological science. It publishes more
there have also been more serious disagreements that have than 40 peer-reviewed scientific journals. Internally, in
resulted in new organizations being formed. In the late the APA Central Office, this is represented by the Science
1950s, a group of experimental psychologists grew Directorate. Since the late 1980s, the Central Office has
unhappy with what they perceived as APAs drift from been reorganized to better represent the diverse constitu-
scientific psychology. By the end of 1959, this group encies of the membership. Beginning with the formation
formed the Psychonomic Society in order, they asserted, of the Practice Directorate in the late 1980s, other Direc-
to foster psychology as a science without a need to attend torates were formed in the hope that the interests of all the
to professional issues. The Psychonomic Society remains a membership would be better represented. As of 2009,
very viable and valuable organization of scientists to the there were the Practice, Education, Science, and Public
present moment; many of its members remained APA Interest Directorates. From a historical perspective, it is
members, as well. A more serious division occurred in too soon to determine whether this approach represents
134 A American Psychological Association (APA), Division 22
an advance for the association or a further balkanization Major Areas or Mission Statement
of the field.
APA remains the worlds largest membership organi- The Division of Rehabilitation Psychology works to unite
zation of psychologists. It has a fascinating past, marked psychologists and others interested in the prevention and
by growth, conflict, and increasing diversification. rehabilitation of disability and chronic illness. Rehabilita-
tion Psychology Practice is a specialty within the domain
of professional healthcare psychology, which applies psy-
Cross References chological knowledge and skills on behalf of individuals
with disabilities and chronic health conditions in order to
Advocacy; Entries 7786 (excluding 84); Entries maximize their health and welfare, independence and
376, 377 choice, functional abilities, and role participation. Such
American Psychological Association Division 22 disabilities include spinal cord injury, brain injury,
American Psychological Association Division 40 stroke, amputations, burns, work-related injuries, multi-
ple traumatic injuries, chronic pain, cancer, heart disease,
multiple sclerosis, neuromuscular disorders, AIDS, devel-
opmental disorders, psychiatric impairment, substance
References and Readings abuse, impairments in sensory functioning, and other
physical, mental and/or emotional impairments. The
Dewsbury, D. A. (1997). On the evolution of divisions. American Psychol- broad field of Rehabilitation Psychology also includes
ogist, 52, 733741.
rehabilitation program development and administration,
Evans, R. B., Sexton, V. S., & Cadwallader, T. C. (Eds.). (1992).
The American Psychological Association: A historical perspective.
research, teaching, public education and development of
Washington, DC: American Psychological Association. policies for injury prevention and health promotion, and
Fernberger, S. W. (1932). The American Psychological Association: advocacy for persons with disabilities and chronic health
A historical summary, 18921930. Psychological Bulletin, 29, 189. conditions.
Guthrie, R. V. (1998). Even the rat was white: A historical view of psychol-
ogy. Boston: Allyn and Bacon.
Pickren, W. E., & Schneider, S. F. (Eds.). (2005). Psychology and the
National Institute of Mental Health: A historical analysis of science, Landmark Contributions
practice, and policy. Washington, DC: APA Books.
1. Rehabilitation psychologists have worked in medical
settings as part of teams of healthcare professionals for
more than half a century, long before psychologists
were regularly involved in other healthcare settings.
American Psychological 2. Division 22 was established in 1958, one of the earlier
Association (APA), Division 22 divisions in APA.
3. Division 22 members conducted the initial research
W ILLIAM S TIERS on individual, interpersonal, and social changes
Johns Hopkins University School of Medicine related to changes in appearance and physical capacity,
Baltimore, MD, USA as well as the social psychology of stereotyping and
prejudice faced by persons with disability.
4. Division 22 members were among the pioneers help-
Membership ing psychology understand the world of work, how the
same can be affected by impairment and disability,
The American Psychological Association (APA) Division and issues about vocational rehabilitation.
22 Rehabilitation Psychology is composed of over 1,111 5. Rehabilitation psychologists have developed the
(2009) psychologists who provide clinical services (91%), principles of cognitive rehabilitation, and have served
teach (65%), conduct research (41%), manage rehabilita- as leaders in the federal model systems programs
tion programs (37%), and perform other activities too. for traumatic brain injury, spinal cord injury, and
They work in hospitals and clinics (40%), in university, burns.
college, medical school (27%), and other settings, and are 6. Board Certification in Rehabilitation Psychology was
also in independent practice (28%). established in 1997.
American Psychological Association (APA), Division 40 A 135
Major Activities the years from its initial one representative to the current
A
allotment of four seats. This trend coincides with Division
The journal Rehabilitation Psychology is published 40s increasing influence within APA and increasing recog-
quarterly by the APA. nition of neuropsychology as a clinical specialty.
Division 22, in conjunction with the American Board Eligibility for membership is based on the criteria re-
of Rehabilitation Psychology, holds an annual conference quired for Associate, Member, or Fellow status in the APA.
in the spring. Additional requirements include demonstrated interest
in the field of neuropsychology and its scientific develop-
ment, public dissemination, and/or clinical applications.
All members of the division have rights and privileges to
Cross References hold office and serve on division committees, vote in
regular elections, attend various meetings of the division,
American Psychological Association (APA) and receive publications of the division. Information for
Rehabilitation Psychology joining Division 40 can be obtained on the divisions web-
site at http://www.div40.org/membership.html.
APA statistics indicate that the majority of Division
40 members are women (55%). Ethnic minority members
References and Readings constitute 8% of the membership, consistent with larger
APA trends. Approximately, 80% of the division member-
American Psychological Association. (2008). A closer look at Division 22: ships have Ph.D. in clinical psychology or a related field.
A growing field meets the challenges of war. Monitor on Psychology,
Nearly half (42%) of the members work in independent
38(8), 5455.
Frank, R., Rosenthal, M., & Caplan, B. (Eds.). (2009). Handbook of settings. Most other members work in medical schools,
rehabilitation psychology (2nd ed.). Washington, DC: American Psy- hospitals, and university settings. Many combine their
chological Association. work in institutional and private-practice settings. Mem-
Larson, P., & Sachs, P. (2000). A history of Division 22. In D. A. Dewsbury bership surveys have indicated that psychologists in Divi-
(Ed.), Unification through division: Histories of the divisions of the
sion 40 spend a substantially larger amount of time
American Psychological Association (Vol. 5, pp. 3358). Washington,
DC: American Psychological Association. (>40%) in assessment activities than other APA members
(<15%). Approximately, one third of the members are
actively involved in research activities. Approximately,
40% are involved in clinical training.
American Psychological
Association (APA), Division 40 Major Areas or Mission Statement
W ILLIAM B. B ARR Division 40 was formed in 1980 with the mission of
New York University School of Medicine enhancing the understanding of brain-behavior relation-
New York, USA ships and the application of such knowledge to human
problems. Activities of the division encompass the areas
of science (e.g., presentations at the annual meeting of
Membership APA, awards for outstanding scientific contributions),
practice (e.g., Current Procedural Terminology CPT
The Division of Clinical Neuropsychology (Division 40) is billing codes, educational brochures for patients), edu-
one of 56 specialty divisions recognized by the American cation and training (e.g., neuropsychology graduate
Psychological Association (APA). Since its inception, it has student organization), and specialty public interest
become one of APAs largest and most active divisions. In its groups (e.g., women, minorities, geriatrics, rural, etc.).
nearly 30 years, membership has grown from 433 psychol- The division upholds APA bylaws and enacted its own
ogists to its current numbership of 5,315, which currently divisional bylaws in 1980, which were subsequently re-
makes it the second largest of all APA divisions behind only vised to their current form in 1997. Over the years, Divi-
the Independent Practice Division (Division 42). The divi- sion 40 has provided published guidelines on many aspects
sions representation to the APA council has grown over of neuropsychological practice and training while also
136 A American Psychological Association (APA), Division 40
fostering continued development of the science of neuro- Dr. Harold Goodglass with Dr. Gerald Goldstein serving
psychology through activity of its committees. The divi- as both the Secretary and Treasurer. The presidents of the
sion advances scientific knowledge in the field of division include many of the most prominent names in the
neuropsychology through its support of publication and field of neuropsychology (Table 1).
presentation of scientific papers at professional confer- One of the divisions earliest activities included work-
ences, including the APAs annual convention. ing with the INS Task Force on Education, Accreditation,
and Credentialing (TFEAC) in establishing guidelines for
doctoral, internship, and postdoctoral training in clinical
Landmark Contributions neuropsychology. Recommendations provided by that
group, calling for a combination of training experiences
Psychologists interested in the developing field of neuro- in psychology and the neurosciences, continues as the
psychology began participating on a regular basis at APA fields dominant model of training. The INS task force
meetings during the 1960s. The origins of Division 40 can was eventually discontinued as it became increasingly
be traced back to the development of the International evident that professional issues were becoming the domain
Neuropsychological Society (INS), which is known as the of Division 40. A listing of publications of other profes-
field of neuropsychologys first formal organization. Infor- sional guidelines and statements developed by Division
mal meetings of psychologists interested in neuropsycho- 40 committees and task forces are provided in Table 2.
logical issues were held at the annual APA meeting dating The purpose of these guidelines was to facilitate an adher-
back to 1965. The INS was formally organized in 1967 as ence to standards for professionals in the field of clinical
an outgrowth of these meetings with the goal of serving as neuropsychology with the ultimate goal of ensuring the
a scientific and educational organization. The need for quality of services provided to consumers.
formal representation in APA became increasingly appar- During the 1990s, a task force from Division 40 led
ent as professional issues regarding practice, education, by Manfred Meier successfully submitted a petition for
and training in neuropsychology began to emerge. Leaders clinical neuropsychology to become the first psycholog-
in the field, including Arthur Benton, Louis Costa and ical specialty recognized by the APAs Commission on
Manfred Meier, saw the need for the development of an Recognition of Specialties and Proficiencies in Profes-
organization to promote the growing specialty of clinical sional Psychology (CRSPP). Recognition of clinical
neuropsychology that was independent of INS and APAs neuropsychology as a specialty became official in 1997.
Division of Clinical Psychology (Division 12). The appli- This was followed by a set of activities, working in
cation to establish a Division of Clinical Neuropsychology conjunction with the National Academy of Neuropsy-
was submitted to APA and approved by its Council of chology (NAN), American Board of Clinical Neuro-
Representatives in September 1979. The formation of psychology (ABCN), American Academy of Clinical
Division 40 was made effective in January 1980, consistent Neuropsychology (AACN), and the Association of
with APA procedures. The divisions first President was Postdoctoral Programs in Clinical Neuropsychology
American Psychological Association (APA), Division 40. Table 1 Presidents of division 40 (clinical neuropsychology)
American Psychological Association (APA), Division 40. The division has four standing committees including
A
Table 2 Published guidelines from division 40 committees Membership, Fellowship, Elections, and Program Com-
and task forces mittees and four continuing committees consisting of the
Science Advisory, Education Advisory, Practice Advisory,
Year Activity
and Public Interest Advisory Committees. Special Com-
1987 Guidelines for Doctoral Training Programs in Clinical mittees, including Task Force Committees, can also be
Neuropsychology established by vote of the Executive Committee, when the
1987 Task Force Report on Computer-Assisted need arises. The Committee on APA Relations and the
Neuropsychological Evaluation Publications and Communications Committee are exam-
1988 Guidelines of Continuing Education in Clinical ples of these. The President, in consultation with the EC,
Neuropsychology appoints chairs of all divisional committees and task forces.
1989 Definition of a Clinical Neuropsychologist Summaries of divisional activities, minutes of executive
1989 Guidelines Regarding the Use of Nondoctoral committee meetings, and committee reports are published
Personnel in Clinical Neuropsychological Assessment biannually in Newsletter 40, the official division newsletter.
1991 Recommendations for Education and Training of Continued commitments to training have been demon-
Nondoctoral Personnel in Clinical Neuropsychology strated by the formation of the Division 40 Association for
1991 Guidelines for Computer-Assisted Neuropsychology Students in Training (ANST) and the
Neuropsychological Rehabilitation and Cognitive establishment of an Early Career Psychologists committee.
Remediation Committees and mentoring programs have been estab-
lished for women entering the field and for ethnic minority
members. Brochures describing an introduction to clinical
(APPCN) in developing an integrated model for spe- neuropsychology are available through the divisions Public
cialty training in clinical neuropsychology. Representa- Interest Advisory Committee (PIAC). The Practice Adviso-
tives from these organizations and various training ry Committee (PAC) provides monitoring of legislative
programs across the USA met in 1997 for what was activities and both local and national activities affecting
termed The Houston Conference on Specialty Training the practice of clinical neuropsychology. This committee is
in Clinical Neuropsychology. The conference led to the also responsible for interactions with government agencies
development and publication of a document describing such as the Centers for Medicare and Medicaid Services
an integrated model of education and training. Interac- (CMS). The PAC worked with other organization in estab-
tions between Division 40 and these other groups con- lishing a new set of CPT testing codes aimed at optimizing
tinue through an organization called the Clinical reimbursement for neuropsychological services. These
Neuropsychology Synarchy (CNS). codes were officially implemented in 2006.
The division has maintained its goal of integrating
science and practice. The Science Advisory Committee
Major Activities (SAC) continues in its role of producing scientific pro-
grams for the APAs annual convention. Studies on neu-
Officers of Division 40 include President, President-Elect, rologic syndromes, assessment, and developmental issues
Past President, Secretary, and Treasurer. These positions are among the topics most commonly presented in the
are elected by the general membership with the term of Division 40 program at the annual APA meeting. The SAC
President lasting 1-year and the roles of Secretary and also provides a number of awards for students and early
Treasurer lasting 3-years. The officers serve on an Execu- career psychologists establishing careers in neuropsycho-
tive Committee (EC) joined by various Division Commit- logical research. More recent SAC activities include inte-
tee Chairs, Divisional Representatives to APA Council, gration of neuropsychologys scientific activities with APA
and three Members-at-Large. Meetings of the EC are and government agencies such as the National Institutes
held twice yearly, with one of the meetings held at the of Health (NIH).
North American meeting of the INS in mid-winter and Division 40 does not publish or provide an official
the other coinciding with the APA convention in the journal. However, over the years, the division has main-
summer. Presidents of the division preside at meetings tained a close relationship with The Clinical Neuropsychol-
and serve as the Chairperson of the EC. Terms of office ogist (TCN), a journal focusing on clinical issues relevant
begin and end at the completion of the annual business to neuropsychologists. The journal has published a num-
meeting held during the summer. ber of statements and guidelines prepared by Division 40
138 A American Speech-Language-Hearing Association (ASHA)
task forces relevant to the practice of neuropsychology for 140,000 members and affiliates who are speech-language
and abstracts from Division 40s scientific program at pathologists, audiologists, and speech, language, and
APA. In 1989, TCN also began to publish regular listings hearing scientists in the USA and at the international level.
of training programs in neuropsychology. In 2006,
a user-interactive revision of the list was developed by
Major Areas or Mission Statement
the Education Advisory Committee (EAC) and trans-
ferred to the Division 40 web site. The listing currently
Vision: Making effective communication a human right,
includes 31 doctoral training programs, 42 internships,
accessible, and achievable for all.
and 78 sites offering postdoctoral residencies for specialty
training in clinical neuropsychology. The web site also
includes descriptions of other divisional activities and Mission
links to the divisions archival material.
Empowering and supporting speech-language patholo-
gists, audiologists, and speech, language, and hearing
Cross References scientists by:
American Academy of Clinical Neuropsychology (AACN) Advocating on behalf of persons with communication
American Psychological Association (APA) and related disorders
International Neuropsychological Society Advancing communication science
National Academy of Neuropsychology Promoting effective human communication
Adams, K. M., & Rourke, B. P. (Eds.) (1992). The TCN guide to professional ASHA has had several names during its 83-year history.
practice in clinical neuropsychology. Berwyn, PA: Swets & Zeitlinger. The first was the American Academy of Speech Correction
Costa, L. (1998). Professionalization in neuropsychology: The early years. (1925). The current name, The American Speech-Language-
The Clinical Neuropsychologist, 12, 17.
Hearing Association (ASHA), was adopted in 1978. ASHA
Meier, M. J. (1992). Modern clinical neuropsychology in historical per-
spective. American Psychologist, 47, 550558.
is the nations leading professional, credentialing, and
Meier, M. J. (2002). In search of knowledge and competence. In scientific organization for speech-language pathologists,
A. Y. Stringer, E. L. Cooley, & A-L. Christensen (Eds.), Pathways to audiologists, and speech/language/hearing scientists.
prominence in neuropsychology: Reflections of twentieth century ASHA has been the guardian of these professions for
pioneers. New York: Psychology Press.
over 75 years, initiating the development of national
Puente, A. E., & Marcotte, A. C. (2000). A history of Division 40 (clinical
neuropsychology). In D. A. Dewsbury (Ed.), Unification through
standards for each discipline and certifying professionals
division: Histories of the divisions of the American Psychological for 55 years.
Association, Volume V. Washington, DC: American Psychological ASHA began in 1925 at an informal meeting of the
Association Press. National Association of Teachers of Speech (NATS) in
Iowa City, IA, an organization of people working in the
areas of rhetoric, debate, and theater. Robert W. West was
the first president of the association from 1925 to 1928.
American Speech-Language- Its members were becoming increasingly interested in
Hearing Association (ASHA) speech correction and wanted to establish an organization
to promote scientific, organized work in the field of
L EMMIETTA M C N EILLY speech correction. Accordingly, in December of that
American Speech-Language-Hearing Association year, the American Academy of Speech Correction
Rockville, MD, USA ASHAs original predecessor was born.
ASHA has grown exponentially since its inception
from 25 members in 1925 to 140,000 in 2010. ASHA
Membership opened its first national office on January 1, 1958 in
Washington, DC. The association subsequently moved
The American Speech-Language-Hearing Association is four times, most recently settling in its current location
the professional, scientific, and credentialing association in Rockville, MD in 2008. ASHAs new national office is
American Speech-Language-Hearing Association Functional Assessment of Communication Skills for Adults A 139
a LEED certified green building the first nonprofit behaviors at the level of disability, based on direct obser-
A
companys building of that distinction in Maryland. vations by speech-language pathologists or significant
others who are familiar with the clients typical commu-
nication performance across the following domains:
Major Activities Social Communication; Communication of Basic Needs;
Reading, Writing, and Number Concepts; and Daily
Publications: The ASHA Leader; American Journal of Planning. Within each domain, specific functional beha-
Audiology; American Journal of Speech-Language viors are rated on a 7-point scale of independence, rang-
Pathology; Journal of Speech, Language, and Hearing ing from does the activity fully independently, through
Research; Language, Speech, and Hearing Services in five levels of does with varying degrees of assistance to
Schools; and Perspectives. does not perform the activity. For example, Social
Conferences: Annual convention and three niche confer- Communication concerns the ability to use names of
ences: Healthcare, Schools, and State Policy Workshop familiar people, exchange information on the telephone,
as well as several web events annually. answer yes/no question and follow directions, under-
stand facial expressions and tone of voice, comprehend
References and Readings nonliteral meaning, and understand TV and radio pro-
grams. Communication of Basic Needs assesses ability to
Interdisciplinary approaches to Brain Damage written by the joint com-
recognize familiar faces and voices, express feelings and
mittee http://www.asha.org/docs/html/PS199000093.html make known needs and wants, and respond in an emer-
Selected practice documents related to Adult Neurogenics are featured gency. Reading, Writing, and Number Concepts examine
in ASHAs Online Practice Policy documents. http://www.asha.org/ the ability to understand simple signs, use reference
academic/curriculum/slp-aneuro/deskref
materials, understand printed material and follow writ-
Structure and Function of an Interdisciplinary Team for Persons with
Acquired Brain Injury http://www.asha.org/docs/html/GL2007
ten directions, complete forms, write messages, and
00288.html make money transactions. Finally, Daily Planning eval-
Memory Assessment on an Interdisciplinary Rehabilitation Team: A uates the ability to tell time, sequence numbers for using
Theoretically Based Framework. http://ajslp.asha.org/cgi/content/full/ a telephone, maintain a schedule of appointments and
16/4/316?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=
use a calendar, and read a map. Each domain is rated
&fulltext=memory+assessment&searchid=1&FIRSTINDEX=0
&sortspec=relevance&resourcetype=HWCIT
globally on the basis of a Scale of Qualitative Dimensions
(i.e., adequacy, appropriateness, promptness, and com-
munication sharing). The measure yields domain and
dimension mean scores, overall scores, and profiles of
American Speech-Language- both Communication Independence and Qualitative
Dimensions.
Hearing Association Functional The ASHA-FACS includes:
Assessment of Communication
A 117-page manual
Skills for Adults A CD version to allow automatic tabulation of the
measures for recording incremental client assessments
C AROLE R OTH in MS Excel used in PC or Apple/Macintosh
Naval Medical Center A paper-and-pencil version with score summary and
San Diego, CA, USA profile forms that purchasers can copy
A rating key on a 5 x 7 card
An electronic index of ICD-9-CM codes
Synonyms
eligibility, and judging the value of care. Developed in independence scores had high interrater agreement
1995 by ASHA, it reflects the collaborative effort of (mean correlation = 0.95) as did overall scores (mean
more than 70 individuals, both ASHA members and correlation = 0.90). Intrarater reliability for communica-
related professionals. The first version of the measure, tion independence mean scores by assessment domain
the ASHA Functional Communication Measures (Frattali, ranged from 0.95 to 0.99 and intrarater reliability of
C.M.1998), was developed for use with both children and overall communication independence scores was 0.99.
adults. The FCMs consisted of 12 rating scales, each Intrarater reliability of qualitative dimension mean scores
representing a separate communication process and ranged from 0.94 to 0.99 and 0.99 for the overall qualita-
rated on an 8-point scale of independence. Development tive dimension scores.
of the FCMs was funded by the National Institute on The ASHA-FACS was moderately correlated with
Child Health and Human Development, National Insti- other measures of language and cognitive function as
tutes of Health. The FCMs were determined to be unsuit- demonstrated by external criterion measures used with
able for use with children, and as a result of other subjects with aphasia and cognitive-communication
limitations, a second group of experts specifically in impairments from traumatic brain injury. A significant
adult communication disorders edited the FCMs, pro- correlation of 0.76 (a = 0.05 level) was obtained be-
posed a multidimensional scoring system, and renamed tween Western Aphasia Battery (WAB) (Kertesz, 1982),
the instrument. Aphasia Quotients (AQs), and ASHA-FACS overall
Further revisions in 1992 included a reconceptualiza- scores. Statistically significant correlations were obtained
tion of the framework to measure at the level of disability, between ASHA-FACS domain scores and WAB subtest
consistent with the World Health Organizations Interna- scores, with the exception of correlations between WAB
tional Classification Scheme, resulting in the final title, fluency scores and reading and writing domain scores
the ASHA Functional Assessment of Communication from the ASHA-FACS. Correlations between the ASHA-
Skills for Adults (ASHA-FACS). The design of the FACS domain score and overall score and each of the
ASHA-FACS was based on a definition of functional Functional Independence Measure (FIM) scales (FIM
communication formulated in 1990 by an ASHA advisory 4.0; SUNY at Buffalo Research Foundation, 1993) were
group: the ability to receive or to convey a message, statistically significant (ranging from 0.42 to 0.82), with
regardless of the mode, to communicate effectively and the exception of the social interaction scale of the FIM.
independently in natural environments (cited in Frattali, External validation data for the subjects with cognitive-
C.M.1995). communication impairments ranged from 0.76 to 0.85
between the Scales of Cognitive Ability for Traumatic
Brain Injury (SCATBI) (Adamovich & Henderson,
Psychometric Data 1992) severity scores and the ASHA-FACS scores, and a
0.84 correlation between the ASHA-FACS overall scores
The usability, sensitivity, reliability, and validity of the with the SCATBI severity scores. These correlations were
ASHA-FACS were demonstrated through two separate all statistically significant at the a = 0.05 level. Statisti-
pilot tests and one field test. The first version was piloted cally significant correlations were found between ASHA-
in 1993 to determine the measures usability, resulting FACS domain and overall scores with the Rancho Los
in the development of a 7-point observational rating Amigos Levels of Cognitive Functioning (Hagen,
scale. A second pilot test confirmed the usability of the Malkmus, & Durham, 1979) (correlations ranged from
revised version, and acceptable levels of reliability and 0.64 to 0.83) and FIM scores (correlations ranged from
validity were found. A more sensitive scoring system for 0.50 to 0.80). Nonsignificant correlations were obtained
capturing qualitative information about the nature of a from SCATBI subtest scores and ASHA-FACS domain
clients functional communication led to the addition of scores obtained from the mild to moderately impaired
a second scoring feature, the 5-point Scale of Qualitative TBI group.
Dimensions. High internal consistency and social validity were
To establish interrater reliability, the ASHA-FACS reported. Internal consistency indicated that most item
was completed independently for 51 subjects by two scores covered the full 7-point rating scale, showed high
examiners within a 48-h period. Interrater reliability inter-item correlations between items within assessment
correlations on the seven assessment domain scores domains, were internally consistent with respect to as-
ranged from 0.72 to 0.92. Overall communication sessment domain, and that all items were measuring the
American Speech-Language-Hearing Association Functional Assessment of Communication Skills for Adults A 141
same underlying construct. The data indicated that all References and Readings A
domain scores correlated with overall ASHA-FACS
scores. Evaluation of social validity was accomplished Adamovich, B., & Henderson, J. (1992). Scales of cognitive ability for
by correlating overall ASHA-FACS scores with measures traumatic brain injury. Chicago: Riverside.
Davidson, B., & Worrall, L. (2002). The assessment of activity limitation
scored by family members or friends of subjects. These
in functional communication: Challenges and choices. In A. E. Hillis
measures included the Communicative Effectiveness (Ed.), The handbook of adult language disorders: Integrating cognitive
Index (CETI; Lomas et al., 1989) and a Rating of Overall neuropsychology, neurology, and rehabilitation (pp. 1934). New
Communication Effectiveness, a single overall index of each York: Psychology Press.
subjects communication effectiveness rated on a scale from Davidson, B., Worrall, L., & Hickson, L. (2003). Identifying the commu-
nication activities of older people with aphasia: Evidence from
1 (lowest) to 7 (highest). These data indicated high positive
naturalistic observation. Aphasiology, 17(3), 243264.
correlations between ASHA-FACS overall scores and Rat- Donovan, N. J., Rosenbek, J. C., Ketterson, T. U., & Velozo, C. A.
ings of Overall Communication Effectiveness by clinicians (2006). Adding meaning to measurement: Initial Rasch analysis of
(i.e., r = 0.81). ASHA-FACS overall scores did not correlate the ASHA FACS Social Communication Subtest. Aphasiology,
well with family members or friends Ratings of Overall 20(24), 362373.
Frattali, C. M. (Ed.) (1998). Measuring modality-specific behaviors,
Communication Effectiveness or CETI scores. CETI ratings
functional abilities, and quality of life. In Measuring outcomes in
were consistently higher than those measured using the speech-language pathology. (P. 203). New York: Thieme.
ASHA-FACS. Frattali, C. M., Thompson, C. K., Holland, A. L., Wohl, C. B., & Ferketic,
M. M. (1995). The American Speech-Language-Hearing Association
Functional Assessment of Communication Skills for Adults (ASHA
FACS). Rockville, MD: ASHA.
Clinical Uses Frattali, C. M., Thompson, C. M., Holland, A. L., Wohl, C. B., & Ferketic,
M. M. (1995). The FACS of life ASHA FACSa functional outcome
ASHA-FACS was designed for clinicians to rate functional measure for adults. ASHA, 37(4), 4046.
communication behaviors of adults with speech, lan- Hagen, C., Malkmus, D., & Durham, P. (1979). Levels of
guage, and cognitive-communication disorders resulting cognitive functioning. In Rehabilitation of the head-injured adult:
Comprehensive physical management (Appendix C., pp. 8789).
from left hemisphere stroke and from traumatic brain
Downey, CA: Professional Staff Association of Rancho Los Amigos
injury. Hospital.
In a review of the evidence leading to recommended Kagan, A., Simmons-Mackie, N., Rowland, A., Huijbregts, M., Shumway,
best practices for assessment of individuals with cogni- E., McEwen, S., Threats, T., & Sharp, S. (2008). Counting what
tive-communication disorders after TBI, the ASHA-FACS counts: A framework for capturing real-life outcomes of aphasia
intervention. Aphasiology, 22(3), 258280.
was one of a few standardized, norm-referenced tests that
Kertesz, A. (1982). Western Aphasia Battery. New York: Grune & Stratton.
met most established criteria for validity and reliability Lomas, J., Pickard, L., Bester, S., Elbard, H., Finlayson, A., & Zoghaib, C.
for use with this clinical population (Turkstra, Coelho, & (1989). The Communicative Effectiveness Index: Development
Ylvisaker, 2005). It was one of only four of the 31 tests and psychometric evaluation of a functional communication
reviewed that evaluated performance outside clinical set- measure for adults. Journal of Speech and Hearing Disorders, 54,
113124.
tings. It was unique in that it was based on research about
Ross, K. B., & Wertz, R. T. (2002). Relationships between language-based
daily communication needs in the target population and disability and quality of life in chronically aphasic adults. Aphasiol-
incorporated consumer feedback about ecological validity ogy, 16(8), 791800.
into the design. The research is rich in the many clinical State University of New York at Buffalo Research Foundation.
benefits of the ASHA-FACS. For example, this instrument (1993). Guide for use of the Uniform Data Set for Medical
Rehabilitation: Functional independence measure. Buffalo, NY:
has been used to measure communication disability rela-
Author.
tive to quality of life in chronically aphasic adults (Ross & Turkstra, L. S., Coelho, C., & Ylvisaker, M. (2005). The use of standar-
Wertz, 2002; Davidson, Worrall, & Hickson, 2003), to dized tests for individuals with cognitive-communication disorders.
evaluate the effectiveness of functionally based communi- Seminars in Speech and Language, 26(4), 215222.
cation therapy (Worrall & Yiu, 2000), and to evaluate real- Worrall, L., & Yiu, E. (2000). Effectiveness of functional communication
therapy by volunteers for people with aphasia following stroke.
life outcomes of aphasia interventions (Kagan et al.,
Aphasiology, 14(9), 911924.
2008). Using Rasch analysis of the ASHA-FACS Social Worrall, L., McCooey, R., Davidson, B., Larkins, B., & Hickson, L. (2002).
Communication Subtest (SCS), Donovan, Rosenbek, The validity of functional assessments of communication and the
Ketterson, & Velozo (2006) demonstrated that caregivers activity/participation components of the ICIDH-2: Do they reflect
were reliable respondents who could use the SCS to rate what really happens in real-life? Journal of Communication Disorders,
35(2), 107137.
therapy progress and functional outcomes.
142 A Americans with Disabilities Act of 1990
Elavil
Current Knowledge
Indication A
Amnesia
Depression
G INETTE L AFLECHE
Memory Disorders Research Center, Boston University
School of Medicine and VA Boston Healthcare System
Off Label Use Boston, MA, USA
Common
Additional Information
Drug Interaction Effects: http://www.drugs.com/drug_interactions.html
Drug Molecule Images: http://www.worldofmolecules.com/drugs/ Amnestic Disorder
Free Drug Online and PDA Software: www.epocrates.com
Gene-Based Estimate of Drug interactions: http://mhc.daytondcs. B ETH S PRINGATE
com:8080/cgi bin/ddiD4?ver=4&task=getDrugList
University of Connecticut
Pill Identification: http://www.drugs.com/pill_identification.html
Storrs, CT, USA
AML Synonyms
Basal forebrain amnesia typically results from vascu- famous individuals. The aspects of memory that remain
A
lar lesions or aneurysm surgery in the region of the intact in classic amnestic disorder patients (such as se-
anterior communicating artery. After basal forebrain mantic memory and motor skill learning) should also be
damage, patients may demonstrate extensive anterograde assessed.
amnesia (Bottger et al., 1998; Tidswell et al., 1995). The main differential diagnoses to consider include
Confabulation is common and may relate to the extent delirium and dementia. Delirium is defined by a distur-
of orbitofrontal involvement (Hashimoto, Tanaka, & bance in attention and consciousness, both of which are
Nakano, 2000), but it often subsides following the acute intact in amnestic disorders. Although dementias present
phase while the amnestic state remains. There is evidence similarly to amnestic disorders in that patients often pres-
that patients with basal forebrain amnesia benefit from ent with memory impairments, cognitive decline (rather
the presentation of cues to enhance recall (Osimani et al., than stability) occurs and impairments in other cognitive
2006). domains such as language or executive functions are
present.
Evaluation
Treatment
As amnestic disorders are defined by deficits in new
learning, memory is the cognitive domain that should Treatment of amnestic disorders is nonspecific and
be emphasized within a neuropsychological evaluation focused primarily on management of symptoms. Cogni-
that also includes assessment of other areas of cognitive tive rehabilitation and memory training programs, which
function such as orientation, attention, language, execu- emphasize the teaching of mnemonic strategies or the
tive functions, visuospatial skills, and psychological func- use of external memory aids such as note-taking or
tioning. Patients fitting the classic amnestic disorder audiotaping in order to enhance patients functioning in
profile will exhibit deficits in memory with generally daily life, have been used to improve memory in indivi-
intact cognition within other domains. duals with dementia and other disorders. Theoretically,
It is important to establish the specific nature of these programs would be useful for individuals with
patients memory impairments. Immediate memory amnestic disorders. However, without the ability for
span (typically assessed through tests such as Digit and patients to consciously recall they have learned these
Spatial Span from the Wechsler Memory Scales) should strategies and remember to implement them, these pro-
be within the normal range. Anterograde learning may grams are likely of little value for patients with amnestic
be assessed with measures such as list learning, story disorders.
learning, or figure memory. While patients will be able The use of pharmacologic agents to treat amnestic
to retain items and repeat them back as long as they disorders is not well studied, and large randomized con-
can keep them in memory, learning curves are typically trolled trials are lacking. In an open-label pilot study,
flat, and an intervening distractor task will cause items Benke et al. (2005) administered donepezil, a cholinester-
to be lost completely. The use of cues or yes/no recogni- ase inhibitor, to patients with a chronic amnestic syn-
tion format, which typically facilitates memory in drome from a ruptured and repaired aneurysm of the
most individuals, will not aid recall in these patients. anterior communicating artery, anterior cerebral, or
Explicit anterograde learning will be equally impaired pericallosal artery. Some measures of performance on a
regardless of the type of memory test (free recall, cued list-learning task improved significantly during the
recall, and recognition), stimulus material (e.g., words, 12-week medication administration period, suggesting
pictures, and sounds), and sensory modality through future double-blinded controlled studies would be useful
which information is acquired (e.g., visual, auditory, to more thoroughly examine the potential utility of
and somatosensory). cholinergic medications.
In addition, retrograde amnesia and memory for re- In addition, due to their memory impairment, patients
mote events can be examined in a qualitative manner are likely to experience impairments in their social and
by inquiring about autobiographical events as well as vocational activities and may also require supervised
memories that one can assume to be present in most living situations and a guardian for legal and medical
people from a given society such as pictures of concerns.
146 A Amnestic Syndromes
Cross References
Amnestic Syndromes
Amnesia
Amnestic Syndrome G INETTE L AFLECHE , M IEKE V ERFAELLIE
Dissociative Amnesia VA Boston Healthcare System and Boston University
Korsakoff s Syndrome School of Medicine
Temporal Lobectomy Boston, MA, USA
hippocampus and adjacent entorhinal, perirhinal and functioning are particularly common (Lim et al., 2004).
A
parahippocampal cortices, as well as the amygdala and In a minority of patients, anoxic injury leads to isolated
polar limbic cortices. Damage often extends to the lateral amnesia.
aspect of the temporal lobe, damaging the anterolateral Relatively selective developmental amnesia has been
aspect, the inferior aspect, or both. Anterior extension of documented in children and adolescents who experienced
damage into ventromedial areas such as the insular cortex an anoxic event shortly after birth. Gadian et al. (2000)
and the basal forebrain has also been documented. The reported on five cases, all of whom had selective bilateral
severity of memory impairment following HSE shows hippocampal atrophy. Neuropsychological results revealed
substantial variation that is directly proportional to the that all of the children performed poorly on tasks of epi-
extent of medial temporal lobe damage (Stefanacci, Buf- sodic memory, but attention, reasoning abilities, and vi-
falo, Schmolck, & Squire, 2000). Lesions are often asym- suospatial skills were intact. Strikingly, these children were
metrical, and this will define the clinical presentation. If able to acquire a considerable amount of new semantic
damage to the left temporal region is greater, verbal knowledge, as indicated by the fact that they were success-
memory problems dominate, whereas if right temporal fully able to attend mainstream schools. The relative pres-
damage is greater, nonverbal aspects of memory are pre- ervation of semantic learning in these children has been
dominantly impaired, such as memory for faces and ascribed to the integrity of subhippocampal cortical areas,
designs. Patients whose lesions extend into lateral tempo- including entorhinal and perirhinal cortices.
ral regions may also suffer from a severe retrograde am-
nesia that is thought to be due to damage to convergence
WernickeKorsakoff Syndrome
zones in anterior temporal areas. Damage primarily to
right anterior temporal regions is more likely to result in a
WernickeKorsakoff Syndrome.
loss of personal episodic memories (OConnor, Butters,
Miliotis, Eslinger, & Cermak, 1992), and that to the left
temporal cortex is associated with loss of semantic knowl- Cerebrovascular Accidents
edge (DeRenzi, Liotti, & Nichelli, 1987). Cases with un-
usual category-specific semantic impairments have also Bilateral posterior cerebral artery (PCA) infarction is a
been described, such as differential loss of knowledge of well-recognized cause of amnesia. Because the left and
concrete versus abstract concepts or animate versus inan- right PCA arise from the bifurcation of a common source,
imate concepts. strokes that occur upstream from the bifurcation can affect
the medial temporal lobes bilaterally, causing a dense
global amnesia. Neuroanatomical studies of patients with
Anoxia PCA infarction have revealed that lesions in the posterior
parahippocampus or the collateral isthmus (a pathway
Anoxic brain injury can result from any of a number of connecting the posterior parahippocampus to association
diverse etiologies including cardiac arrest, respiratory dis- cortex) are critical for the memory impairment (Von
tress, carbon monoxide poisoning, or drug overdose. Cramon, Hebel, & Schuri, 1988). When damage extends
These clinical conditions all diminish or cut off the supply posteriorly to include occipitotemporal cortices, deficits
of oxygen to the brain, either through reduced blood flow beyond amnesia are often seen.
or reduced blood oxygen saturation. The physiological Early in their clinical course, patients with PCA in-
consequences of such anoxic events are complex. Brain farction exhibit a global confusion that eventually resolves
areas particularly vulnerable to anoxic injury include the into an isolated amnestic syndrome or may be associated
hippocampus, basal ganglia, and watershed areas of the with additional neuropsychological deficits, such as visual
cerebral cortex. The clinical manifestations of anoxia are field defects, alexia, color agnosia, or anomia. The mem-
highly variable, but memory impairment is a common ory disturbance is characterized by a classic profile of
manifestation. A review of 58 studies of cerebral anoxia consolidation deficits in the context of normal working
showed that while damage to hippocampal structures was memory and normal intelligence. There may or may not
common, damage restricted to the hippocampus was seen be associated retrograde amnesia. Memory problems have
in only 18% of patients (Caine & Watson, 2000). Accord- also been described with unilateral, usually left, PCA
ingly, in a majority of patients, memory impairment infarction. In such cases, the memory impairment can
occurs in the context of generalized cognitive impairment. be transient or permanent, and is typically limited to
Significant changes in executive abilities and motor verbal material. Memory deficits in patients with right
148 A Amnestic Syndromes
PCA have been less well studied, but such examination is tests, particularly following a delay. This reflects a disrup-
complicated by the perceptual problems that frequently tion of strategic retrieval processes that allow access to
accompany right PCA infarction. information stored in memory. Deficient strategic mem-
Thalamic strokes can also lead to significant memory ory processes also contribute to poor encoding, and the
loss. Because the relevant thalamic centers are small and use of organizational strategies at encoding can enhance
adjacent to one another, it is difficult to establish associa- patients performance. A failure to adequately monitor
tions between site of damage and clinical deficits. A recent the outcome of memory search can also occur, and this
review (Van der Werf et al., 2000) suggests that damage to manifests as a tendency toward high level of false alarms
the mammillo-thalamic tract (MTT) invariably causes in recognition tests. In extreme cases, this can lead to
anterograde amnesia, and that no amnesia occurs in the impairment in recognition memory that exceeds that
absence of damage to the MTT. Medial dorsal lesions seen in recall. Other features linked to frontal dysfunction
cause a memory disturbance that is mild in comparison include impaired source memory and temporal tagging.
to the severe amnesia that arises when the lesion extends
to the MTT. Patients with thalamic amnesia exhibit exec-
utive dysfunction, increased sensitivity to interference, Evaluation
and variability in the persistence and extent of retrograde
amnesia. Although a primary focus of the assessment in amnesia is
on memory function, it is important to assess other cog-
nitive domains as well, including general intelligence,
ACoA Aneurysm attention, executive functions, language, semantic knowl-
edge, and visuospatial skills. Such a comprehensive ap-
Rupture of ACoA can result in a memory disorder that proach is required to distinguish whether a patient
ranges from mild to severe. The ACoA provides blood presents with a pure amnesic syndrome or with memory
supply to the basal forebrain, the anterior cingulate, the impairment in the context of more pervasive cognitive
anterior hypothalamus, the anterior columns of the for- difficulty. New learning abilities should be assessed by
nix, the anterior commissure, and the genu of the corpus measures of free recall, cued recall, and recognition, and
callosum. The pathological consequences of a ruptured should examine both immediate and delayed retention.
aneurysm may be a result of infarction directly, or sec- Information derived from specific aspects of perfor-
ondary to subarachnoid hemorrhage, vasospasm, and mance, such as the shape of the learning curve, compari-
hematoma formation. Because of the various neuropath- son of recall and recognition performance, and effects of
ological consequences, the clinical profiles associated with delay, all provide important pointers to the nature of the
ACoA aneurysm are more variable than those seen with memory breakdown (e.g. inefficiencies in encoding, re-
diencephalic or medial temporal lobe injuries, and the trieval, or consolidation) and may inform remediation.
impairments are often more global in nature (DeLuca & A variety of standardized tests are available to assess
Diamond, 1995). memory function, and the reader is referred to Lezak,
The acute phase of recovery following rupture and Howieson, & Loring, 2004, for specific examples. The
repair of ACoA aneurysm is characterized by a severe most commonly used standardized memory test is the
confusional state and a marked attentional disorder. As Wechsler Memory Scale-III or IV, which consists of a
the confusion resolves, memory problems become more series of subtests that probe various aspects of verbal
apparent. These can vary from mild impairments to se- and nonverbal memory in different formats. Assessment
vere amnesia. A temporally graded retrograde amnesia is of remote memory should cover knowledge of public
also frequently present. Other symptoms, including exec- events and people, personal facts, and autobiographical
utive dysfunction, confabulation, and poor insight, are events. Such assessment can be challenging, because there
likely to be part of the resulting clinical syndrome if the are few standardized measures available, and corrobora-
lesion extends to the medial frontal lobes. The clinical tion from a caregiver may be needed to establish the
outcome of patients with more extensive lesions is typi- accuracy of reported personal memories. With respect to
cally worse than that of patients with lesions limited to the general fund of knowledge, areas of assessment include
basal forebrain. knowledge of famous names and faces, public news
The amnesia associated with ACoA aneurysm has a events, and new vocabulary that has recently entered the
marked frontal dysexecutive component. Performance on language. Several structured interviews have been devel-
recognition tests is often better preserved than on recall oped to examine recollection of personal events and facts.
Amnestic Syndromes A 149
Amnion Rupture
Amotivational
Anencephaly
Apathy
Amorphognosis
J OHN E. M ENDOZA Amoxapine
Tulane University Medical Center
New Orleans, LA, USA J OHN C. C OURTNEY
Childrens Hospital of New Orleans
New Orleans, LA, USA
Definition
Amorphognosis is that aspect of tactile agnosia which Generic Name
refers specifically to deficits in the ability to appreciate
(identify) the external form of an object such as its shape, Amoxapine
size, or other contour features by tactual manipulation
alone. In the absence of more elementary somatosensory
disturbances resulting from either peripheral nerve or the
dorsal column system, such deficits suggest lesions in the Brand Name
contralateral postcentral gyrus of the parietal lobe or in its
adjacent association cortices. Ascendin
Cross References
Class
Ahylognosia
Astereognosis Tetracyclic antidepressant
Tactile Agnosia
Indication A
Amphetamine
Reactive depressive disorder, psychotic depression, and
depression accompanied by anxiety or agitation. J OA NN T. T SCHANZ 1, K ATHERINE T REIBER 1,2
1
Utah State University
Logan, UT, USA
2
University of Massachusetts Medical School
Off Label Use Worcester, MA, USA
enhancing the release and blocking the reuptake of the occur in amphetamine psychosis vs. schizophrenia;
monoamine neurotransmitters dopamine, norepineph- Iversen et al., 2009). As reported above, other negative
rine, and serotonin (Feldman et al., 1997; Iversen et al., effects of chronic amphetamine abuse include neurotoxic
2009). At high doses, the drug also inhibits the metabo- damage to neurotransmitter systems. Impairments in atten-
lism of catecholamines by the enzyme monoamine oxi- tion and memory have also been reported which may persist
dase. Chronic use of amphetamine has been associated even after a period of prolonged abstinence (Gouzoulis-
with damage to selective dopamine and serotonin Mayfrank & Daumann, 2009; Iversen et al., 2009).
neurons and receptors (Feldman et al., 1997; Gouzoulis-
Mayfrank & Daumann, 2009). Methamphetamine is also
a potent neurotoxin, although its toxic effects predomi- Future Directions
nantly involve the serotonergic system (Feldman et al.,
1997; Gouzoulis-Mayfrank & Daumann, 2009). The rein- Research into the psychoactive and behavioral effects of
forcing properties of amphetamine are hypothesized to amphetamine has helped advance knowledge of the psy-
reflect increased dopamine neurotransmission in the sub- chological role of several monoamine neurotransmitters
cortical structure, the nucleus accumbens. and their relevance to clinical conditions such as addic-
tion and schizophrenia and the neurochemistry underly-
ing some cognitive processes such as attention and
Historical Background and Clinical working memory. Future research will undoubtedly uti-
Relevance lize advances in technology to elucidate the neural struc-
tures and pathways associated with reward circuits
First introduced and marketed as a nasal or bronchial involved in addictions, examine the neuroplasticity of
decongestant in the 1930s, amphetamine was sought for the nervous system after chronic abuse, and clarify the
its psychoactive effects and as an appetite suppressant. It moderating role of genetics in the behavioral response to
was used in the military to enhance attention and coun- amphetamine and other compounds (Iversen et al., 2009).
teract the effects of sleep deprivation (Iversen et al., 2009;
Meyer & Quenzer, 2005). Amphetamine and its deriva-
tives have also been used for the treatment of narcolepsy, Cross References
attentional problems, and as a stimulant in the general
population (Meyer & Quenzer, 2005). D-Amphetamine
Over time, the addictive properties of amphetamine Dopamine
were realized, particularly of its potent derivatives. The
acute effects of amphetamine-based drugs are enhanced
by use of a rapid route of administration such as intrave-
References and Readings
nous injection. Following a short-term rush however, a
Feldman, R. S., Meyer, J. S., & Quenzer, L. F. (1997). Stimulants: Am-
period of restless agitation, depression, irritability, and
phetamine and cocaine. In Principles of neuropsychopharmacology
other negative symptoms ensues. Repeated, continuous (pp. 549568). Sunderland, MA: Sinauer Associates.
administrations are followed by a let down, with a pro- Gouzoulis-Mayfrank, E., & Daumann, J. (2009). Neurotoxicity of drugs
longed period of sleep. This alternating cycle, when re- of abuse-the case of methylenedioxyamphetamines (MDMA,
peated, results in a substantial physical toll on the body. ecstasy), and amphetamines. Dialogues in clinical Neuroscience, 11,
305317.
As with other drugs of abuse, dependence and tolerance
Iversen, L. L., Iversen, S. D., Bloom, F. E., & Roth, R. H. (2009). Psychos-
can develop with chronic use, leading to the administra- timulants. In Introduction to neuropsychopharmacology (pp. 447
tion of increasing doses to achieve the desired effects. 472). New York: Oxford University Press.
With sustained chronic use, negative effects may emerge. Meyer, J. S., & Quenzer, L. F. (2005). Psychomotor stimulants: Cocaine
These include repetitive, stereotyped behaviors as well as and the amphetamines. In Psychopharmacology: Drugs, the brain and
behavior (pp. 292300). Sunderland, MA: Sinauer Associates.
a psychotic syndrome consisting of hallucinations and
paranoid delusions. This syndrome, known as amphet-
amine psychosis is notably similar to the symptoms of
paranoid schizophrenia and has provided some support
for the dopamine hypothesis of schizophrenia. However, AMPS
qualitative differences between the two conditions also
exist (e.g., greater tendency for visual hallucinations to Assessment of Motor Process Skills
Amygdala A 153
Historical Background A
Amusia
The amygdala was originally described by Burdach in the
J OHN E. M ENDOZA late nineteenth century as an almond-shaped structure
Tulane University Medical Center situated deep in the anterior temporal lobe of the central
New Orleans, LA, USA nervous system. The amygdala was subsequently shown to
be important for the appropriate processing of emotional
information in nonhuman primates by Kluver and Bucy
Current Knowledge in the 1930s. This permitted McLean to include the amyg-
dala in the group of brain structures that make up the
Music involves both complex qualities such as familiar limbic system thought to be involved in processing
melodies, rhythm, or tempo, and more elementary of emotional information. Since then progress has
aspects such as discrimination of timbre, pitch, or continued toward understanding the role that the amyg-
tone. While lesions of the temporal lobes are fairly dala plays in processing and encoding emotional informa-
consistently implicated, the hemispheric localization of tion in the mammalian central nervous system.
lesions responsible for specific deficits has been more
controversial. Music, like language, is composed of indi-
vidual, temporally sequenced stimuli (musical notes, Current Knowledge
melodies, tunes), each capable of being analyzed with
regard to particular features such as pitch and timbre, The amygdala is an almond-shaped structure located in
functions that would appear to be more in keeping with the medial temporal lobe of mammals. However, the first
the suspected operations of the left hemisphere. By con- description of this almond-shaped structure only referred
trast, melodies may also be perceived as a gestalt, which to a portion of the amygdala called the basal nucleus.
is more characteristic of right hemisphere functions. Currently, the amygdala is described as a collection of
There is evidence that well-trained musicians come to different subnuclei or subareas, one of which is the basal
rely more heavily on the left hemisphere for processing nucleus. The nuclei have been grouped together based on
certain aspects of music when compared with non-musi- their phylogenetic similarities or similarities in their neu-
cians. However, the right hemisphere evidences superi- ronal elements. Older phylogenetic nuclei include the
ority for both the perception and expression of music in olfactory areas (i.e., cortical nucleus and nucleus of the
studies of non-musicians. Thus, the strategies by which olfactory tract) and the central and medial nuclei. More
various musical elements are approached, as well as the recent phylogenetic structures include areas similar to the
leading hemisphere in appreciating those elements, are neocortex such as the lateral, basal, and accessory basal
most likely determined in part by ones prior musical nuclei, which are collectively referred to as the basolateral
experience or training. In summary, while both the right region or complex. Based on similarities in their neuronal
and left hemispheres are apparently involved in the components, various nuclei of the amygdala have been
expression and perception or appreciation of music, the defined as neocortical-like nuclei (such as the basolateral
specific contributions of each are still somewhat of a complex) that consist of glutamatergic pyramidal-like neu-
mystery. rons or striatal-like nuclei (such as the central and medial
nuclei) that consist of GABAergic medium spiny neurons.
In humans, the amygdala is located under the uncus
of the limbic lobe at the anterior end of the hippocampus.
Amygdala It also merges with the periamygdaloid cortex and abuts
the putamen and tail of caudate nucleus. As a whole, the
R ORY M C Q UISTON amygdala receives diverse inputs from throughout the
Virginia Commonwealth University central nervous system. The basolateral complex receives
Richmond, VA, USA inputs encoding somatosensory, visual, auditory, gustato-
ry, olfactory, and visceral information from the dorsal
thalamus, prefrontal cortex, cingulate, parahippocampal
Synonyms gyrus, insular cortex, and sensory associational areas.
The central and medial nuclei receive inputs from olfac-
Amygdaloid body; Amygdaloid nucleus tory centers, hypothalamus (ventromedial and lateral),
154 A Amygdala
dorsomedial and medial nuclei of the thalamus, and vis- amygdala innervate the lateral nuclei. The lateral nuclei
ceral inputs from the parabrachial nuclei, solitary nucleus are the most dorsally located within the amygdala, medial
and periaqueductal gray of the brainstem. Outputs from to the piriform cortex, and underneath the striatum. The
the amygdala are equally diverse. They leave via two lateral nuclei receive associational inputs encoding a sin-
predominant pathways. The central nucleus contributes gle sensation (somatosensory, visual, auditory, gustatory,
to the stria terminalis where its efferents make connec- olfactory, or visceral). This is the first stage where sensory
tions with the hypothalamus (preoptic nuclei, ventrome- input is assigned emotional value and also where some
dial nucleus, anterior nucleus, and lateral hypothalamic emotional memories may be stored (however, the amyg-
areas), nucleus accumbens, septal nuclei, and rostral por- dala as a site for storing emotional memories remains a
tions of the caudate and putamen. However, the primary contentious issue). Although the lateral nuclei projects to
output of the amygdala is the ventral amygdalofugal multiple areas within and outside the amygdala, a major
pathway. Through this pathway, the basolateral complex output is the basal nuclei (located ventral to the lateral
sends inputs to the hypothalamus, septal nuclei, sustantia nuclei) where the initial sensory processing of the lateral
innominata, prefrontal, cingulate, insular, and inferior nuclei is integrated with inputs from highly processed
temporal cortices. Through the same pathway, the central areas including polymodal sensory areas and areas
nucleus projects diffusely in the brainstem innervating the involved in memory formation like the hippocampus.
dorsal vagus, raphe, locus coeruleus, parabrachial nuclei, The lateral and basal nuclei project medially to the central
and the periaqueductal gray. It is the interplay between the nucleus either directly or indirectly through intercalated
diverse afferents projecting to the amygdala, processing cells (intercalated cells separate the basolateral complex
within the amygdala, and the effect of the amygdala on its from the central and medial nuclei). The central nuclei
targets that contribute to the emotional assessment of send much of the processed emotional content from the
incoming sensory information and coordinated behavior- amygdala to the rest of the brain. Thus, the central nucle-
al responses. us is seen as the output region of the amygdala. The central
Most of what is known about human amygdala nucleus produces emotional responses through its effects
function comes from studies of patients with damage to on its various targets throughout the central nervous sys-
the amygdala. However, most damage in humans is not tem. For example, the central nucleus produces arousal
restricted to the amygdala alone and patients with damage through its innervation of modulatory systems in the
to larger areas of the medial temporal lobe have more brain stem that release norepinephrine, dopamine, seroto-
profound deficits. Nonetheless, patients with temporal nin, and acetylcholine. Its input to the periaqueductal gray
lobe damage including the amygdala display a number produces freezing, startle, analgesia, and cardiovascular
of emotional and inappropriate behavioral deficits. changes associated with fear. It also innervates the parabra-
These include impaired fear responses, hypersexuality, chial nucleus where it affects pain processing. Its inputs to
hyperorality, and hyperattention. These behaviors were the dorsal motor vagal nucleus controls parasympathetic
originally described by Kluver and Bucy in nonhuman nervous system function and it also affects vagal nerve
primates. function through its projection to the solitary nucleus.
Much of what is known about functional circuitry Finally, the central nuclei projects to the hypothalamus
within the amygdala and how it relates to encoding of where it controls the release of hormones and activates
emotion has been gleaned from studies in rodents. The the sympathetic nervous system.
amygdala can be divided into many subareas based on In summary, the amygdala is a complex group of
functional circuitry. Lateral to the amygdala is the piri- nuclei that receive diverse inputs from various regions of
form cortex, which encodes olfactory information. Olfac- the central nervous system to assess emotional value.
tory information from the piriform cortex, and other Similarly, after extensive processing, its outputs innervate
olfactory structures, projects to the most ventral and a diverse group of regions in the central nervous system to
lateral portion of the amygdala, the cortical nuclei. The exert its effect. The result is that the amygdala is involved
cortical nuclei in turn project medially to the ventrally in encoding fear, reward, aggression, sexual, maternal, and
located medial nuclei, which is a major output for olfac- ingestive behaviors. This results in effects on cognition,
tory information from the amygdala. However, less is attention, perception, and memory formation. Therefore,
known about the ventrally located olfactory associated it is not surprising that amygdala dysfunction has been
amygdala nuclei compared to the more dorsal multisen- associated with anxiety disorders such as posttraumatic
sory nuclei. The more dorsal nuclei receive information stress disorder, phobias and panic attacks, depression, and
from all sensory modalities. The major inputs to the schizophrenia.
Amyloid Plaques A 155
Future Directions A
Amygdaloid Nucleus
Most of what is known about emotional information
processing performed by the amygdala has been gleaned Amygdala
from studies of fear conditioning. However, the amygdala
also likely plays a role in the encoding of positive emo-
tions associated with rewarding stimuli. Currently, efforts Amyloid Plaques
are being made toward understanding the different types
of emotional values encoded in the amygdala. Also, it J OA NN T. T SCHANZ
remains somewhat contentious whether emotional mem- Utah State University
ory is actually stored by the amygdala. It is of great Logan, UT, USA
interest to determine where emotional memories are
stored in the amygdala (possibly the lateral nuclei) and
precisely what types of memories are being stored by the Synonyms
amygdala, that is, whether these memories are of con-
scious declarative forms or more procedural reflexive Diffuse plaques; Neuritic plaques; Senile plaques
forms. Understanding how the amygdala contributes to
the formation of different forms of emotional memory
will likely provide insights for the treatment of several
Definition
psychiatric illnesses such as posttraumatic stress disorder,
Amyloid plaques refer to an aggregation of beta amyloid
phobias, anxiety, and depression.
protein found in the extracellular space between neurons in
the brain. Amyloid plaques may be of diffuse, pre-amyloid
type, or neuritic, mature senile type. The latter is recog-
Cross References
nized as one of the neuropathological hallmarks of Alzhei-
mers disease (AD). Mature amyloid plaques are spherical
Efferent
in shape and consist of a central beta-amyloid core, fibril-
Insular Lobe
lary outward extensions, and surrounding dystrophic
Limbic System
neurites (elements of degenerating neurons). Unlike the
Locus Ceruleus
mature and senile plaques, diffuse plaques have an amor-
Midbrain Raphe
phous, irregular shape, and lack the surrounding neurites.
Neocortex
Striatum
Temporal Lobes Current Knowledge
Natural History, Prognostic Factors, toward echolalia, stereotypy of expression, and persevera-
A
Outcomes tion in ALS.
When deficits in memory are observed in ALS,
Incidence of ALS peaks in the 60s and drops rapidly they are more likely to be evident on immediate than
thereafter. A broad estimate of mortality is that 50% of delayed recall tasks. Some take this to implicate poorer
patients do not survive beyond 3 years from symptom executive control over encoding processes, whereas others
onset, but that some may survive 10 years or more. Three might invoke slowed information processing as an expla-
epidemiologic studies provide fairly consistent survival nation. The finding that patients can benefit dispropor-
data using time of diagnosis as the reference point tionately from the provision of recognition cues relative
(though diagnostic confirmation may lag onset by 1318 to free recall formats suggests that retrieval deficits might
months): 78% at 1 year, 56% at 2 years, and 32% at also be implicated, or that shallow levels of encoding are
4 years. Several factors are associated with poorer prog- sufficient to support recognition but not recall.
nosis: low-forced vital capacity, bulbar onset (often less Concerning behavioral changes, rating scales have
tolerant of forced ventilation), older age at onset, and revealed that as many as two thirds of persons with ALS
shorter interval between first symptom and presentation. show one or more of irritability, disinhibition, inflexibili-
Patients attending tertiary and specialized ALS clinics ty, restlessness, and apathy. Apathy and questionable or
tend to show longer survival and treatment with riluzole, poor social judgment are more likely to be observed in
on average, extends life by 3 months. Longer survival is patients with bulbar onset ALS. Surprisingly, although
seen in persons with only upper or lower motor neuron reactive depressive reactions may occur after diagnosis,
disease, though as noted, it is controversial whether var- major depression is quite rare among ALS patients (about
iants such as primary lateral sclerosis are ALS. 10%). Symptoms of depression are common, occurring in
about half of patients. Persons with ALS may in particular
experience hopelessness and end-of-life concerns. Patho-
Neuropsychology and Psychology logical laughing or crying, as seen in pseudobulbar syn-
of Amyotrophic Lateral Sclerosis dromes, should not be confused with depression.
Cross References
Synonyms
Assistive Technology
Cortical Motor Pathways ALSFRS; ALSFRS-R
Frontal Lobes
Frontal Temporal Dementia
Frontotemporal Lobar Degeneration Description
Speech
The Amyotrophic Lateral Sclerosis Functional Rating
Scale is a validated instrument designed to assess the
References and Readings functional status and the disease progression in patients
with amyotrophic lateral sclerosis (ALS). It is a tool that
Averill, A. J., Kasarskis, E. J., & Segerstrom, S. C. (2007). Psychological
can be used to monitor functional change in a patient
health in patients with amyotrophic lateral sclerosis. Amyotrophic
Lateral Sclerosis, 8, 243254.
over time. The ALSFRS is a 10-item functional inventory
Boeve, B. F. (2007). Links between frontotemporal lobar degeneration, which was devised for use in therapeutic trials in ALS.
corticobasal degeneration, progressive supranuclear palsy, and Each item is rated on a 04 scale, (with 0 being severely
Analysis of Variance A 159
impaired and 4 being normal) by the patient and/or hospital length of stay and survival time in ALS patients
A
caregiver, yielding a maximum score of 40 points. The treated with tracheostomy-intermittent positive-pressure
ALSFRS assesses the patients levels of self-sufficiency in ventilation. Through observation and interview the eva-
areas of self-feeding, grooming, ambulation and commu- luator assesses the following measures: speech, salivation,
nication, and swallowing. swallowing, handwriting, cutting food/handling utensils,
turning in bed and adjusting bed clothes, walking,
climbing stairs, and breathing.
Historical Background
The ALSFRS was developed because then current used Cross References
clinimetric scales being utilized at the time were contami-
nated with impairment measurements did not measure Amyotrophic Lateral Sclerosis
the broad range of disabilities that result from ALS, and
did not lend themselves to sub-score analysis that was
References and Readings
based entirely on disability components (Feinstein, 1987;
Louwerse et al., 1990; Streiner & Norman, 1989).
ALS CNTF Treatment Study (ACTS) Phase 111 Study Group. (1996).
The ALSFRS is a validated rating instrument for mon- The Amyotrophic Lateral Sclerosis Functional Rating Scale. Assess-
itoring the progression of disability in patients with ALS. ment of activities of daily living in patients with Amyotrophic
One weakness of the ALSFRS, as it was originally Lateral Sclerosis. Archives of Neurology, 53, 141147.
designed, was that it granted disproportionate weighting Cedarbaum, J. M., & Stambler, N. (1997). Performance of the
Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS)
to limb and bulbar, as compared to respiratory dysfunc-
in multicenter clinical trials. Journal of the Neurological Sciences,
tion. The ALS Functional Rating Scale Revised version 152(Suppl 1), S1S9.
that is also validated incorporates additional assessments Herndon, R. M. (2006). Handbook of neurologic rating scales (p. 96).
of dyspnea, orthopnea, and the need for ventilator New York: Demos Medical Publishing.
support. The Revised ALSFRS (ALSFRS-R) retains the Lo Coco, D., Marchese, S., La Bella, V., Piccoli, T., & Lo Coco, A. (2007).
The amyotrophic lateral sclerosis functional rating scale predicts
properties of the original scale and shows strong internal
survival time in amyotrophic lateral sclerosis patients on invasive
consistency and construct validity. mechanical ventilation. Chest, 132(1), 6469.
Psychometric Data
Analysis of Covariance
The ALSFRS was developed as an internally consistent,
reliable, and valid measure of disability in ALS patients ANCOVA/MANCOVA
as part of the Amyotrophic Lateral Sclerosis Ciliary Neu-
rotrophic Factor (ALS CNTF) Treatment Study (ACTS
Phase 111 Study Group, 1996). The ability of the ALSFRS
to be responsive to change in the clinical status of ALS Analysis of Variance
patients was evaluated cross-sectionally and prospectively
over time in phase 1 and phase 2 studies of CNTF in ALS. M ICHAEL D. F RANZEN
The ALSFRS has been validated both cross-sectionally Allegheny General Hospital
and longitudinally against muscle strength, the Schwab and Pittsburgh, PA, USA
England ADL rating scale, the Clinical Global Impression
of Change (CGIC) scale, and independent assessments of
patients functional status (Cedarbaum & Stambler, 1997). Synonyms
ANOVA
Clinical Uses
ANCOVA/MANCOVA
Anarchic Hand
M ICHAEL F RANZEN
Alien Hand Syndrome Allegheny Neuropsychiatric Institute
Pittsburgh, PA, USA
Anarthria
Synonyms
C AROLE R OTH
Naval Medical Center Analysis of covariance
San Diego, CA, USA
Definition
Synonyms
ANCOVA or analysis of covariance is a variant of the
Speechlessness ANOVA model in which the statistical effect of a
Anencephaly A 161
nuisance variable is removed mathematically from the Watson, P. (2009). Review of Analysis of variance and covariance: How to
choose and construct models for the life sciences. Psychological A
analysis in order to clarify the relations between the
Medicine, 39, 695696.
independent and the dependent variables. The optimal Wildt, A. R. & Ahtola, O. T. (1978). Analysis of covariance. Beverly Hills:
situation would be if the independent variable levels or Sage.
groups were not related to the nuisance variable. Howev-
er, if the nuisance variable is related to the dependent
variable and if the nuisance variable is systematically
represented among the independent variables, ANCOVA
may be used to partial out the statistical effect of the Anencephaly
nuisance variable or covariate. This is not a substitute for
removing the effect through experimental design. For E RIN D. B IGLER , J O A NN P ETRIE
example, level of education may be statistically related Brigham Young University
to performance on a memory test. If two groups of Provo, Utah, USA
depressed and nondepressed individuals differ systemati-
cally on the basis of their level of education, any
difference found with regard to performance on a Synonyms
memory test might be due to the different level of
education. By employing ANCOVA and using education Amnion rupture; Congenital defects; Exencephaly; Lack
level as the covariate, the researcher may have a of neural tube closure; MRI; Neural tube defects
clearer understanding of the relation between the
presence of depression and performance on the memory
test. Short Description or Definition
Although there are different mathematical methods
for conducting an ANCOVA including the use of multi- Using an in front of an anatomical descriptor signifies
ple regression (which see), ANCOVA under the general absence. Cephalic is Greek for head with encephalon
linear model provides a useful conceptualization of the specifically referring to the brain. Therefore, the term
underlying idea. We can think of calculating the regres- anencephaly is used to denote a congenital defect in the
sion between the covariate and the dependent variable development of the head, including the meninges, the
and then residualizing the influence of the covariate. cranium, and the scalp and, in particular, abnormal
Then an ANOVA can be conducted on the residual brain growth, with an almost completely diminished
values. In order to use ANCOVA, the data must satisfy prosencephalon (telencephalon diencephalon) or fore-
a few basic assumptions. There must be a linear relation brain and only rudimentary development of the brain
between the covariate and the dependent variable. The stem.
slope of the regression for each group or level of the
independent variable must be the same. The error term
should be normally distributed with a mean of zero. The
covariate should not be affected by the independent Categorization
variable.
Anencephaly results from the failure of closure of the
headend of the neural tube in early fetal development
(first 34 weeks) with subsequent neural tube defects
Cross References (NTD) including lack of formation of the brain, skull
and scalp. Loss of the forebrain includes loss of the two
Analysis of Variance (ANOVA)
cerebral hemispheres, the connecting corpus callosum,
neocortex, thalamus, hypothalamus and other structures
of the limbic system the amygdala, hippocampus, cau-
References and Readings date nucleus, ventricles, etc., and all of their connections
(Kolb & Whishaw, 2008). These structures comprise the
Belin, T. R., & Normand, S.-L. T. (2009). The role of
majority of human brain tissue and are required for
ANCOVA in analyzing experimental data. Psychiatric Annals, 39, almost all sensation perception and basic physiological
753759. functions including body temperature control, eating,
162 A Anencephaly
sleeping, and motor function, and cognition, language, Natural History, Prognostic Factors,
memory, emotion, thought processing, inhibition, deci- and Outcomes
sion making, and/or reasoning.
With the major portion of an infants brain being
undeveloped, particularly the cerebrum, and coupled
with the brain often being exposed in utero, the anence-
Epidemiology
phalic infant is frequently stillborn. An infant born alive
with anencephaly is, as a rule, blind, deaf, unconscious,
Anencephaly results from NTD (Cohen, 2002; Detrait
and may only reflexively respond. With only a basic brain
et al., 2005; Dias & Partington, 2004; Mitchell, 2005)
stem and a nonfunctioning cerebrum, prognosis is poor;
with approximately 1 in 1,000 births born with NTD;
anencephalic infants will never gain consciousness and
these may be associated with genetics, nutrition, environ-
will only have minimal reflex actions such as breathing.
ment, or a combination of all three. There is a known
There may be intermittent sound or touch responses;
higher prevalence of females born with anencephaly NTD
however, no further progress can be expected (see
as compared to males (James, 1980). Over the past 3
National Institute of Neurological Disorders and
decades, worldwide research has found an association
Stroke, 2010).
between prenatal folic acid deficits leading to folate defi-
ciencies (National Institutes of Health: Office of Dietary
Supplements, 2009) and NTD (see also Calvo & Biglieri, Neuropsychology and Psychology
2008; Kondo, Kamihira, & Ozawa, 2009; Wolff, Witkop, of Anencephaly
Miller, & Syed, 2009). While all the causes of open NTD
are not known, research indicates that daily consumption There is essentially no assessment that neuropsychological
of 4 mg/day of folic acid by women before and during testing can offer given the absence of cortical development
pregnancy brings about a 70% reduction in NTD (Centers in the anencephalic infant who does survive. Such chil-
for Disease Control and Prevention, 1991, 2008; Cornel & dren have reflexive function only (i.e., breathing and
Erickson, 1997; McLone, 2003; MRC Vitamin Study some responses to sound or touch can manifest) and
Research Group, 1991). will rarely survive longer than a few hours or days.
a b c
Anencephaly. Figure 1 Magnetic resonance imaging (MRI) findings of the head and neck 8 h after birth. (a) Sagittal
T1-weighted, (b) sagittal T2-weighted, and (c) coronal T1-weighted images show cranial schisis. The normal skin stops at the skull
base and encircles abnormally developed cerebral structures, the so-called area cerebrovasculosa (white arrows). Along the
border of the skull defect the skin seems to be in continuity with the superficial layer of the area cerebrovasculosa, probably the
pia mater (curved white arrow). The posterior fossa is funnel-shaped. A rudimentary brain stem (curved black arrows) and
primordium of cerebellum (small black arrows) are present. The cervical spine is normal (From Calzolari et al., 2004, With
permission)
Anencephaly A 163
Neuropsychologists should have an empathetic awareness Calzolari, F., Gambi, B., Garani, G., & Tamisari, L. (2004). Anencephaly:
MRI findings and pathogenetic theories. Pediatric Radiology, A
of this condition; they may be asked to consult with
34, 10121016.
parents and families about the nature of the infants Centers for Disease Control and Prevention (1991). Use of folic acid
deficits and the poor prognosis (Ashwal, 2005). for prevention of spina bifida and other neural tube defects
19831991. MMWR Morbidity and Mortality Weekly Report, 40,
513516.
Evaluation Centers for Disease Control and Prevention (2008). Prevalence of neural
tube defects and folic acid knowledge and consumption puerto
Although the pathogenesis of anencephaly is still not fully rico, 19962006. MMWR Morbidity and Mortality Weekly Report,
57, 1013.
understood, several studies suggest that exencephaly or the
Cohen, M. M., Jr. (2002). Malformations of the craniofacial region:
lack of skull growth or separation following NTD allows Evolutionary, embryonic, genetic, and clinical perspectives. American
the cerebral tissue to be exposed in utero causing damage Journal of Medical Genetics, 115, 245268.
from the amniotic fluid (Calzolari, Gambi, Garani, & Cook, R. J., Erdman, J. N., Hevia, M., & Dickens, B. M. (2008). Prenatal
Tamisari, 2004). As can be seen in Fig. 1, even though management of anencephaly. International Journal of Gynecology &
Obstetrics, 102, 304308.
there are other anomalies of physical development
Cornel, M. C., & Erickson, J. D. (1997). Comparison of national policies
associated with the presence of anencephaly, the most on periconceptional use of folic acid to prevent spina bifida and
dramatic is a failure of brain development. anencephaly (SBA). Teratology, 55, 134137.
Detrait, E. R., George, T. M., Etchevers, H. C., et al. (2005).
Human neural tube defects: Developmental biology, epidemiology,
Treatment and genetics. Neurotoxicology and Teratology, 27, 515524.
Dias, M. S., & Partington, M. (2004). Embryology of myelomeningocele
Ultimately, mortality rate is 100% with anencephaly. and anencephaly. Neurosurgical Focus, 16, E1.
Some anencephalic children do survive from hours to James, W. H. (1980). The sex ratios of anencephalics born to anencephalic-
days but rarely longer and in a persistent vegetative state prone women. Developmental Medicine and Child Neurology,
22, 618622.
(Payne & Taylor, 1997); thus, treatment is purely support-
Kolb, B., & Whishaw, I. Q. (2008). Fundamentals of human neuropsychol-
ive. The presence of a surviving infant with anencephaly ogy. New York: Worth Publishers.
raises numerous ethical questions about care, treatment, Kondo, A., Kamihira, O., & Ozawa, H. (2009). Neural tube defects:
and maintenance (Batavia, 2002; Cook, Erdman, Hevia, & Prevalence, etiology and prevention. International Journal of Urology,
Dickens, 2008; Obeidi, Russell, Higgins, & ODonoghue, 16, 4957.
McLone, D. G. (2003). The etiology of neural tube defects: The role of
2010), including the importance of continued research
folic acid. Childs Nervous System, 19, 537539.
for better ways to prevent and treat neurological birth Mitchell, L. E. (2005). Epidemiology of neural tube defects. American
defects. Journal of Medical Genetics. Part C. Seminars in Medical Genetics,
135C, 8894.
MRC Vitamin Study Research Group (1991). Prevention of neural tube
Cross References defects: Results of the medical research council vitamin study.
Lancet, 338, 131137.
Ethics in the Practice of Neuropsychology National Institute of Neurological Disorders and Stroke. (2010, January
14, 2010). NINDS anencephaly information page. National Insti-
Forebrain
tutes of Health: Reducing the burden of neurological disease Re-
National Institute of Neurological Disorders and Stroke trieved March 16, 2010, from http://www.ninds.nih.gov/disorders/
National Institutes of Health (NIH) anencephaly/anencephaly.htm
National Institutes of Health: Office of Dietary Supplements. (2009, 4/15/
2009). Dietary supplement fact sheet: Folate. Dietary Supplement
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supplements.info.nih.gov/factsheets/folate.asp
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Batavia, A. I. (2002). Disability versus futility in rationing health Payne, S. K., & Taylor, R. M. (1997). The persistent vegetative state and
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Calvo, E. B., & Biglieri, A. (2008). [impact of folic acid fortification on supplementation for the prevention of neural tube defects: An
womens nutritional status and on the prevalence of neural tube update of the evidence for the U.S. Preventive services task force.
defects]. Archives of Argentina Pediatrics, 106, 492498. Annals of Internal Medicine, 150, 632639.
164 A Aneurysm
Epidemiological Factors
Aneurysm. Table 1 Symptoms that may be associated with
Ruptured aneurysms, specifically the saccular type, are the ruptured and unruptured cerebral aneurysms (From Bonner &
most common cause of subarachnoid hemorrhage (SAH) Bonner, 1991)
after 20 years of age. This type of aneurysm accounts for
Ruptured
about 80% of nontraumatic aneurysms. aneurysms Unruptured aneurysms
Parenchymal Headache, nuchal rigidity
dissection
Natural History, Prognostic Factors, and Hyperfusion Neurologic deficit
Outcomes
Cerebral edema Drowsiness, confusion, focal
neurologic deficit
Unruptured aneurysms may be symptomatic and mani-
Cognitive Decerebrate rigidity/vegetative
fested as cranial nerve palsies. Ruptured cerebral aneur-
impairments disturbance possible
ysms can be associated with states of consciousness
Disturbances in Deep coma
ranging from lethargy to coma. Outcome depends on
personality
location and severity of bleeding. A sudden loss of
Angelman Syndrome A 165
Epidemiology
Exact prevalence rates of Angelman syndrome are
Angelman Syndrome unknown but have been estimated between 1/10,000 and
1/40,000 (Clayton-Smith & Laan, 2003). See Table 1 for
K RISTIN D. P HILLIPS 1, B ONITA P. K LEIN -TASMAN 2
1 estimates by subtype.
Medical College of Wisconsin
Milwaukee, WI, USA
2
University of Wisconsin-Milwaukee Natural History, Prognostic Factors,
Milwaukee, WI, USA and Outcomes
Genetic
mechanism Incidence Definition
De novo 70% Deletion on maternal
deletion chromosome region 15q1113
Uniparental 23% Both copies of chromosome 15
disomy are inherited from the father,
rather than one from each parent
Imprinting 25% Genes become inactivated as a
defect result of a disruption in genes
controlling the imprinting
process itself, or the imprinting
center
UBE3A 1015%
mutation
Unknown 1015%
(Cassidy, Dykens, & Williams, 2000; Clayton-Smith & Laan, 2003;
Williams, 2005)
fewer difficulties with motor and language skills than typically employed to identify genetic deletions, whereas
A
these individuals (Clayton-Smith & Laan, 2003). DNA-methylation testing can be used to detect uniparental
disomy or imprinting defects.
Neuropsychology and Psychology of
Angelman Syndrome
Treatment
Neuroanatomical findings have demonstrated anomalous
There is no cure for Angelman syndrome itself. Given
Sylvian fissures in individuals with Angelman syndrome
the high incidence of seizure disorder, management and
(Leonard et al., 1993), in addition to marked cerebellar
follow-up by a neurologist is usually necessary. Anticon-
atrophy (Jay, Becker, Chan, & Perry, 1991). Cognitive
vulsant medications have been utilized to manage sei-
functioning typically falls in the severe-to-profound
zures. Clonazepam, valproic acid, and phenobarbital
range of intellectual disability (Peters, Goddard-Finegold,
appear to be most effective in addressing seizures in
Beaudet, Madduri, Turcich, & Bacino, 2004). Similarly,
Angelman syndrome. Sleep difficulties have successfully
adaptive functioning is delayed, with a relative strength
been addressed through behavioral and pharmacological
in socialization and a relative weakness in motor skills
intervention.
(Peters et al., 2004). A primary feature of Angelman
Involvement in interventions such as occupational,
syndrome is limited expressive language, typically ranging
physical, and speech/language therapy is typically recom-
from no language to very few single words. There are
mended to address language and motor deficits. In addition
relative strengths in nonverbal communication and recep-
to speech/language therapy, alternative communication
tive language. Marked deficits occur in fine motor skills.
methods typically need to be explored. Special education
A happy temperament has been reported among
programming is also indicated in light of cognitive deficits.
individuals with Angelman syndrome, characterized by
Very few behavioral intervention studies have been con-
frequent smiling and laughter, which persists across the
ducted for individuals with Angelman syndrome. Behavior-
lifespan and is most evident in social interactions (e.g.,
al training has been used to increase communication and
Clayton-Smith, 2001; Clayton-Smith & Laan, 2003).
daily living skills.
Parents have rated their children with Angelman syn-
drome lower on irritability and lethargy, in comparison
to individuals with other developmental disabilities Cross References
(Summers & Feldman, 1999). A variety of behavioral
difficulties have been reported, the most common includ- Ataxia
ing inattention and hyperactivity (Clarke & Marston, Developmental Delay
2000; Summers, Allison, Lynch, & Sandler, 1995). Epilepsy
However, there is some indication that these behavioral Intellectual Disabilities
difficulties improve with age (Clayton-Smith, 2001). Microcephaly
Stereotyped behaviors, such as hand flapping, have been PraderWilli Syndrome
observed. In addition, individuals with Angelman syn- Seizure
drome often have an attraction to water and shiny objects. Syndrome
These latter findings have led to the conclusion that the
incidence of autism spectrum disorders is high in this
population; however, this may be overdiagnosed References and Readings
in Angelman syndrome given the severe-to-profound
intellectual disability. Sleep problems are common, in- Angelman Syndrome Foundation, Inc. (2009). Retrieved November 6,
cluding issues like falling asleep, staying asleep, and 2008, from http://www.angelman.org/
being easily roused from sleep. Feeding problems have Cassidy, S. B., Dykens, E., & Williams, C. A. (2000). Prader-Willi and
Angelman syndromes: Sister imprinted disorders. American Journal
also been reported.
of Medical Genetics, 97, 136146.
Clarke, D. J., & Marston, G. (2000). Problem behaviors associated with
15q- Angelman syndrome. American Journal on Mental Retardation,
Evaluation
105, 2531.
Clayton-Smith, J. (2001). Angelman syndrome: Evolution of the pheno-
Angelman syndrome is confirmed through genetic test- type in adolescents and adults. Developmental Medicine and Child
ing. Fluorescence in-situ hybridization (FISH) testing is Neurology, 43, 467480.
168 A Angiitis
Clayton-Smith, J., & Laan, L. (2003). Angelman syndrome: A review of the Definition
clinical and genetic aspects. Journal of Medical Genetics, 40, 8795.
Jay, V., Becker, L. E., Chan, F. W., & Perry, T. L. Sr. (1991). Puppet-like
syndrome of Angelman: a pathologic and neurochemical study.
Angiography refers to a set of procedures designed to
Neurology, 41, 416422. image the arterial circulation. As such, cerebral angio-
Leonard, C. M., Williams, C. A., Nicholls, R. D., Agee, O. F., Voeller, K. K., graphy refers specifically to the imaging of the cerebral
Honeyman, J. C., et al. (1993). Angelman and Prader-Willi arterial tree.
syndrome: A magnetic resonance imaging study of differences in
cerebral structure. American Journal of Medical Genetics, 46, 2633.
Levitas, A., Dykens, E., Finucane, B., & Kates, W. (2007). Behavioral
phenotype of genetic disorders. In R. Fletcher, E. Loschen, Current Knowledge
C. Stavrakaki, & M. First (Eds.), Diagnostic manual intellectual
disability: A textbook of diagnoses of mental disorders in persons with Historical background: Angiography was initially intro-
intellectual disability. Kingston, NY: NADD Press.
duced into medical practice in the late 1940s. Traditional
Peters, S. U., Goddard-Finegold, J., Beaudet, A. L., Madduri, N.,
Turcich, M., & Bacino, C. A. (2004). Cognitive and adaptive beha-
catheter angiography involves introduction of a catheter
vior profiles of children with Angelman syndrome. American Journal through a large peripheral artery (typically the femoral
of Medical Genetics, 128, 110113. artery), threading it to its desired location, and injection
Summers, J. A., Allison, D. B., Lynch, P. S., & Sandler, L. (1995). of radio-opaque contrast medium while obtaining radio-
Behaviour problems in Angelman syndrome. Journal of Intellectual
graphic images. The introduction of computerized tomo-
Disability Research, 39, 97106.
Summers, J. A., & Feldman, M. A. (1999). Distinctive pattern of
graphic angiography (CT angiography) in the 1970s
behavioral functioning in Angelman syndrome. American Journal allowed the administration of contrast material intrave-
on Mental Retardation, 104, 376384. nously rather than intra-arterially, since the reconstructed
Williams, C. A. (2005). Neurological aspects of the Angelman syndrome. tissue slices allow visualization of the contrast in the
Brain & Development, 27, 8894.
arteries or organs of interest. Magnetic resonance (MR)
Williams, C. A., Beaudet, A. L., Clayton-Smith, J., Knoll, J. H.,
Kyllerman, M., Laan, L. A., et al. (2006). Angelman syndrome
angiography was added to the diagnostic armamentarium
2005: Updated consensus for diagnostic criteria. American Journal in the early 1990s. MR digital subtraction angiography
of Medical Genetics, 140, 413418. can visualize the arterial circulation via detection of
moving water molecules in blood, without the attendant
risks of toxicity from the contrast medium or radiation
exposure (Fig. 1). It has the additional advantage of
Angiitis revealing abnormalities in the vessel wall, not merely
luminal filling defects. However, contrast agents designed
Vasculitis for visibility in MR scanning, and administered intrave-
nously, can further enhance visualization.
Psychometric data: The sensitivity, specificity, and pos-
itive predictive value of the various forms of angiography
Angio are dependent on the disorder under study and its preva-
lence in the sample being investigated. Overall, however,
Carotid Angiography less invasive forms of angiography have increasingly
supplanted the catheter-based methods, because of compa-
rable accuracy. In one recent porcine model, for example,
estimated degree of arterial narrowing did not differ
Angiography, Cerebral significantly between catheter and digital subtraction
imaging methods, and the correlation between methods
J OHN W HYTE was highly significant.
Moss Rehabilitation Research Institute Clinical uses: Angiography can be performed to
Albert Einstein Healthcare Network visualize occult vascular pathology such as unruptured
Elkins Park, PA, USA aneurisms, arteriovenous malformations, or the abnor-
mal vascular supply of tumors. It can also be performed
to localize the source of clinically significant bleeding, as
Synonyms in the case of ruptured aneurisms, or to locate the sites of
narrowing or occlusion by atherosclerotic plaque, throm-
(Cerebral) arteriography bus, arterial dissection, or external compression.
Angioma, Cavernous Angioma A 169
J ENNIFER T INKER
Drexel University
Philadelphia, PA, USA
Synonyms
Definition
Synonyms
Cross References A
Anomalous Dominance
Dominance (Cerebral)
J OHN E. M ENDOZA
Tulane University Medical Center
New Orleans, LA, USA References and Readings
word to express that meaning (Raymer & Rothi, 2008). Raymer, A. M., & Rothi, L. J. G. (2008). Cognitive neuropsychological
approaches to assessment and treatment: Impairments of lexical
These different steps in word finding engage different
comprehension and production. In R. Chapey (Ed.), Language in-
parts of the brain distributed throughout the left cere- tervention strategies in adult aphasia (5th ed., pp. 607631).
bral hemisphere. Therefore, when brain damage occurs, Baltimore: Lippincott, Williams & Wilkins.
anomia will accompany different types of aphasia, and
different types of anomic errors can arise. It is important
to note that anomia and anomic aphasia are not synony-
mous. Anomia is the primary symptom of anomic aphasia
Anomic Aphasia
and also can be observed in virtually all other forms of
A NASTASIA R AYMER
aphasia (e.g., Brocas aphasia, Wernickes aphasia), both as
Old Dominion University
initial and residual signs when other signs and symptoms
Norfolk, VA, USA
of aphasia have resolved.
When anomia occurs, a number of errors can be seen
(Goodglass, Kaplan, & Barresi, 2001). At times, the
moment of anomia leads to complete inability to retrieve
Definition
a word. Other times, an inappropriate word is retrieved,
Anomic aphasia is the language impairment that involves
also known as a paraphasia. Sometimes, the error word
only word finding difficulties or pure anomia in contrast
is somehow related to the intended word in meaning
to other forms of aphasia (Goodglass et al., 2001). Other
(semantic paraphasia, e.g., saying dog for cat) or sound
language modalities typically are intact, including auditory
characteristics (phonologic paraphasia, e.g., saying
comprehension of language, repetition of words and
crat for cat). Sometimes, the moment of word finding
sentences, and spontaneous generation of sentences.
difficulty is filled with a description of the intended word
or circumlocution (e.g., That thing that meows and has
whiskers. I cant think of the name.). In severe forms of Current Knowledge
anomia, neologisms may occur in which the uttered word
may not be recognizable at all (e.g., saying bilan for cat). Anomic aphasia is a form of language disorder associated
with acquired brain damage typically affecting the left
cerebral hemisphere (Raymer, 2005). Anomic aphasia
Cross References can be manifest as a difficulty in retrieving specific
intended words, often nouns, but sometimes verbs,
Circumlocution during the course of sentence generation. The grammati-
Confrontation Naming cal characteristics of the sentence remain intact. The
Paraphasia moments of word retrieval difficulty lead to long pauses,
Phonemic Paraphasia insertion of filler words, or selection of wrong words
Semantic Paraphasia (paraphasias) during conversation or other word retrieval
Word Finding activities, most commonly in tasks requiring individuals
to name pictures. Also common in anomic aphasia is
circumlocution, in which the speaker cannot think of
References and Readings the intended word and instead describes or provides
associated information about the word.
Biedermann, B., Ruh, N., Nickels, L., & Coltheart, M. (2008). Information When anomic aphasia occurs as a result of an acute
retrieval in tip of the tongue states: New data and methodological neurologic event (e.g., stroke), it can be accompanied by
advances. Journal of Psycholinguistic Research, 37, 171198.
pure alexia and difficulties with color identification
Goodglass, H., Kaplan, E., & Barresi, B. (2001). The assessment of aphasia
and related disorders (3rd ed.). Philadelphia: Lippincott, Williams, &
(Goodglass et al., 2001). Acutely, anomic aphasia has
Wilkins. been described following left temporal/occipital lesions
Goodglass, H., & Wingfield, A. (Eds.) (1997). Anomia: Neuroanatomical (e.g., area 37) and left thalamic lesions (Raymer, Moberg,
and cognitive correlates. San Diego: Academic Press. Crosson, Nadeau, & Rothi, 1997; Raymer, Foundas et al.,
Laine, M., & Martin, N. (2006). Anomia: Theoretical and clinical aspects.
1997). Anomic aphasia also can be seen chronically as
New York: Psychology Press.
Raymer, A. M. (2005). Naming and word retrieval problems. In
individuals recover from other forms of aphasia. In that
L.L. LaPointe (Ed.), Aphasia and related neurogenic language case, the accompanying symptoms and neural correlates
disorders (3rd ed., pp. 7286). New York: Thieme. of anomic aphasia vary.
Anosmia A 173
Epidemiology
References and Readings
Olfactory dysfunction is present in at least 1% of indivi-
Goodglass, H., Kaplan, E., & Barresi, B. (2001). The assessment of duals under the age of 65, with some estimates suggesting
aphasia and related disorders (3rd ed.). Philadelphia: Lippincott, total anosmia in as much as 5% of the population. Rates
Williams, & Wilkins. of impairment increase dramatically with age, with
Goodglass, H. & Wingfield, A. (Eds.) (1997). Anomia: Neuroanatomical
approximately 25% of older adults showing deficits in
and cognitive correlates. San Diego: Academic Press.
Laine, M. & Martin, N. (2006). Anomia: Theoretical and clinical aspects. olfaction (Murphy et al., 2002). In patients presenting to
New York: Psychology Press. chemosensory clinics, olfactory deficits are reported to be
Raymer, A. M. (2005). Naming and word retrieval problems. In related to disability and quality of life, though most
L. L. LaPointe (Ed.), Aphasia and related neurogenic language individuals with olfactory deficits are unaware of them.
disorders (3rd ed., pp. 7286). New York: Thieme.
It is well established that throughout the lifespan, women
Raymer, A. M., Foundas, A., Maher, L., Greenwald, M., Morris, M.,
Rothi, L. J. G. et al. (1997). Cognitive neuropsychological analysis show more acute olfactory abilities than men.
and neuroanatomic correlates in a case of acute anomia. Brain and
Language, 58, 137156.
Raymer, A. M., Moberg, P., Crosson, B., Nadeau, S., & Rothi, L. J. G. Causes
(1997). Lexical-semantic deficits in two patients with dominant
thalamic infarction. Neuropsychologia, 35, 211219.
The causes of olfactory impairments are typically
categorized as: (1) conductive/transport impairments,
(2) sensory/sensorineural deficits, or (3) central olfactory
neural impairment, though these categories are not
Anosmia mutually exclusive. The understanding of the potential
causes of olfactory deficits will be enhanced by a brief
H OLLY J AMES W ESTERVELT, N ICOLE C. R. M C L AUGHLIN review of the olfactory system, though it is noted that the
Alpert Medical School of Brown University olfactory pathways within the central nervous system
Providence, RI, USA (CNS) are not entirely agreed upon.
the primary olfactory cortex, which is primarily made up Anosmia. Table 1 Sampling of disorders associated with
of the piriform cortex. Other structures receiving direct olfactory deficits
input include the anterior olfactory nucleus, olfactory
Alcoholism/Korsakoffs
tubercle, amygdala, and rostral entorhinal cortex
syndrome Multiple sclerosis
(Gotfried & Zald, 2005). Projections from these primary
areas extend to secondary olfactory regions in the hippo- Alzheimers disease Multiple system atrophy
campus, hypothalamus, thalamus, amygdala, and agranular Amyotrophic lateral Parkinson dementia complex
insula, enabling encoding of odors into memory as well sclerosis of Guam
as emotional processing of specific odors (Gotfried & Corticobasal degeneration Parkinsons disease
Zald, 2005). There are also projections to the orbitofrontal Dementia with Lewy Progressive supranuclear
cortex (OFC), and it is believed that the OFC mediates bodies palsy
conscious perception of odors; lesions to this area often Diabetes mellitus REM sleep behavior disorder
lead to impaired olfactory abilities (Gotfried & Zald, Downs syndrome Restless leg syndrome
2005). In addition to the activation of the first cranial Frontotemporal dementia Schizophrenia
nerve, certain smells may also activate the trigeminal
Head injury Sjogrens syndrome
nerve (CNV), which mediates sensations associated with
certain odorants, including burning, cooling, irritation, Human immunodeficiency Syphilis
virus
or tickling sensations. Activation of the trigeminal nerve
may allow the detection of some odors, even in the Huntingtons disease Temporal lobe epilepsy
presence of primary olfactory impairments. Cranial Mild cognitive impairment Vascular dementia
nerve zero (nervus terminalis) may also play some role
in olfaction, though its function is poorly understood
in humans. loss. Given the vast number of disorders that have
shown olfactory deficits, theories have been postulated
Conductive/Transport Impairment that olfactory impairment may be a nonspecific marker
Olfactory impairment within this category arises from of CNS dysfunction. This is likely not the case, given
obstruction of nasal passages. Typical causes of obstruc- that the degree of deficit can differ widely among
tion include nasal inflammation, such as from allergies or disorders, there exists significant range of deficits
upper respiratory infection (URI), or other structural among patients within disorders, and the deficits can
interference, such as nasal polyps. URI is the most be unrelated to disease stage or magnitude of disease
common cause of smell loss, and is often transient. symptoms in some diseases but not others. Rather, it is
Permanent smell loss due to URI can occur, presumably probable that the presence and degree of olfactory
reflecting direct insult to the neuroepithelium, and involvement is related to the relative degree of structural
becomes more likely in older age. or biochemical damage to the specific regions of the
brain involved in olfactory transduction.
Sensorineural/Central Olfactory Neural
Impairment Neurodegenerative Diseases
Olfactory deficits within these categories arise from Interest in olfaction in neurodegenerative disorders began
damage to the neuroepithelium and/or impairment or most intensely in the 1980s, with a focus on Alzheimers
impingement of central olfactory structures from CNS disease (AD) and Parkinsons disease (PD). It was initially
disease. There are numerous congenital, endocrine, thought that these two disorders, which were often
neurological/neurodegenerative, nutritional/metabolic, thought of as the prototypical examples of cortical and
and psychiatric disorders that have been shown to be subcortical diseases, would share an early and notable
associated with olfactory deficits (for a review of these deficit. Olfactory deficits were then identified in a variety
causes, see Murphy, Doty, & Duncan, 2003). In addition, of neurodegenerative disorders, making olfactory loss a
injury, medications (for review see Doty & Bromley, nonspecific finding, though the degree of impairment
2004), environmental toxins (for review see Upadhyay & may be useful in distinguishing some disorders.
Holbrook, 2004), structural lesions, and medical/surgical The cause of olfactory deficits in neurodegenerative
interventions (for review see Murphy et al., 2003) can diseases is unknown (for a review of potential causes, see
affect neural functioning. The Table 1 provides a small Smutzer, Doty, Arnold, & Trojanowski, 2003). The deficits
sampling of disorders that can be associated with olfactory may be due at least in part to neurotransmitter system
Anosmia A 175
alterations, especially dopamine and acetylcholine. disease progression. Although group studies have shown
A
Damage to central processing areas is also a likely expla- consistent deficits in odor identification, it should be
nation, particularly involvement of the olfactory bulb and noted that the presence of deficits is not a universal
tracts, as relevant neuropathologic changes (e.g., neurofi- finding among patients with AD, making odor identifica-
brillary tangles, amyloid plaques, dystrophic neurites, tion tests imperfect screening instruments for the disorder.
Lewy bodies, and disproportionate neuronal loss) are Odor identification has also been studied recently in
often seen in these areas. Other relevant central proces- patients with mild cognitive impairment (MCI) and
sing areas (e.g., entorhinal cortex), however, also show cognitively intact older adults with and without genetic
neuropathologic changes, as may peripheral structures risk for future cognitive decline. Several longitudinal
(e.g., olfactory epithelium). studies have demonstrated that odor identification has a
strong relationship with memory performance, even in
Parkinsons Disease Olfactory impairment is a promi- healthy older adults performing within normal limits on
nent, common, and early feature of Parkinsons disease cognitive measures (Devanand et al., 2000; Wilson et al.,
(PD; see Doty, 2003a, b, for a review). The deficits tend to 2007). These studies also show that odor identification is a
be bilateral, and are more common than some of the unique and significant predictor of future cognitive
hallmark symptoms of PD, such as tremor. Olfactory decline above and beyond baseline memory performance,
deficits may be present before the motor symptoms as well as a good predictor of conversion to dementia in
become evident, and are apparent with both threshold patients with MCI. In cross-sectional studies of MCI
and identification tasks. The size of the effect is astoun- subtypes, patients with both amnestic and non-amnestic
ding (ranging from 1.17 to 12.15 in a meta-analysis; subtypes perform modestly worse than healthy older
Mesholam, Moberg, Mahr, & Doty, 1998), though the adults but better than patients with dementia (Devanand
majority of patients are not completely anosmic. Deficits et al., in press; Westervelt, Bruce, Coon, & Tremont,
do not appear to correlate with the extent of cognitive or 2008). In using olfactory performance to distinguish
motor involvement, do not respond to treatment, and do MCI subtypes, results are mixed, though when significant
not appear to be progressive over time. differences have been found between subtypes, the
magnitude of the difference is of questionable clinical
Other Parkinsonian Spectrum Disorders Other Parkinso- significance. Together, these studies suggest that when a
nian disorders, such as corticobasal degeneration (CBD), notable olfactory deficit is observed in patients with MCI,
multiple system atrophy, and progressive supranuclear there is substantial risk of future decline. However, odor
palsy, are also associated with olfactory deficits, though identification measures may not be particularly clinically
the impairments tend to be more mild than is seen in useful in early detection or early differential diagnosis for
PD (Doty, 2003a, b). These findings suggest that olfactory the modal patient.
functioning may be useful in distinguishing PD from
other parkinsonian disorders, though a more recent Dementia with Lewy Bodies Olfaction in dementia with
study of olfaction in CBD raises some question of Lewy bodies (DLB) was first described in a study that
potentially more notable deficit in this disorder than crudely measured anosmia with a brief detection task
was previously described (Pardini, Huey, Cavanagh, & (McShane et al., 2001). Forty percent of patients with
Grafman, 2009). DLB were found to be anosmic, in contrast with 16%
of patients with AD, and 6% of the healthy controls.
Alzheimers Disease and Mild Cognitive The presence of smell loss was not found to be associated
Impairment There has been fairly good consistency in with concurrent AD and DLB pathology on autopsy.
the literature for most of the studies examining olfaction Subsequent studies confirmed anosmia to be more
in Alzheimers disease (AD; see Doty, 2003a, b, for a common in DLB than in AD, with anosmia present in
review). The size of the effect is extremely large, ranging 5665% of patients with DLB (and some degree of smell
from 0.98 to 8.55 in a meta-analysis (Mesholam et al., loss in nearly 90%), but in only 1123% of AD patients
1998), though, as in PD, patients are typically not (Olichney et al., 2005; Westervelt, Stern, & Tremont,
completely anosmic. Odor identification deficits are al- 2003). Assessment of anosmia has been shown to improve
ways found; odor detection deficits are more inconsis- the sensitivity of diagnostic criteria for DLB, with mini-
tently demonstrated and may be a later symptom. The mal loss of specificity (Olichney et al., 2005). Combined,
odor identification deficit does not seem to be primarily these few studies raise the possibility that olfactory
due to a general cognitive deficit and deficits worsen with measures may be useful in distinguishing AD from DLB.
176 A Anosmia
Other Dementias Olfactory deficits have also been occurrence of any event associated with the deficit (e.g.,
described in other dementias, including recent, consistent head injury, illness). Fluctuations in symptoms may be
findings of smell deficits in frontotemporal dementia that most suggestive of obstructive causes, but need to be
are generally of the magnitude of deficits seen in AD distinguished from paroxysmal events. Medical history
(Luzzi et al., 2007; McLaughlin & Westervelt, 2008; should be carefully assessed, as multiple medical condi-
Pardini et al., 2009), and, in vascular dementia to a similar tions and medications may be associated with olfactory
or lesser extent to that seen in AD (Gray, Staples, Murren, alterations. Referral for an ENT evaluation may be war-
Dhariwal, & Bentham, 2001; Knupfer & Spiegel, 1986). ranted. Olfactory hallucinations, in particular, require
careful work-up as they may be indicative of seizure or
Head Injury tumor, and are less likely of primary psychiatric origin.
Olfactory loss is fairly common following head injury (for
review, see Costanza, DiNardo, & Reiter, 2003), with the
Classes of Assessment
incidence of anosmia ranging from approximately 5 to 60%.
These latter estimates represent the incidence among
There are three classes of olfactory assessment methods:
patients with severe head injury, though total anosmia
psychophysical, electrophysiological, and psychophysio-
can occur even after very mild injury. Partial or unilateral
logical, with psychophysical assessment being the most
loss may be less likely to be detected than total anosmia.
common and most clinically relevant.
Deficits may be caused by a variety of mechanisms, includ-
ing sinus/nasal injury, shearing of the olfactory nerve, or Psychophysiological
contusion/hemorrhage in central processing regions. In Psychophysiological assessment of olfactory abilities relies
regard to shear injuries, the axons of the olfactory receptor on the measurement of changes in the autonomic nervous
cells are particularly susceptible to injury as they pass system after presentation of an odorous stimuli, through
through the body ridges of the cribiform plate. Coup and such methods as heart rate and blood pressure. These
contra-coup forces are likely to result in anosmia, with methods are rarely used.
occipital blows most frequently causing smell loss.
Electrophysiological
Schizophrenia Electrophysiological assessments examine electrical
Olfactory deficits have been well-studied in schizophrenia activity generated in response to an odorant and are
(for review, see Doty, 2003a, b). Deficits have been shown primarily research tools. Electro-olfactograms (EOG)
to be of lesser magnitude than typically seen in AD and use electrodes placed on the human olfactory epithelium
PD, progress with disease duration, and are most asso- to directly assess olfactory abilities. Olfactory event-
ciated with negative symptoms of the disease. In patients related potentials (ERP) are recorded from the scalp
showing olfactory deficits, the impairments appear early surface, measuring electroencephalographic activity after
in the disease, perhaps in prodromal stages. There does presentation of brief, precisely defined odorous stimuli.
not appear to be any notable relationship with antipsy- For example, chemosensory ERPs can be obtained after
chotic medication use or cigarette smoking. Odor identi- stimulation of olfactory nerve (olfactory ERPs) or the
fication deficits correlate most strongly with measures of trigeminal nerve (somatosensory ERPs). Absence of
executive functioning in this population, rather than olfactory ERPs in presence of somatosensory ERPs
those of medial temporal lobe functioning. All aspects of suggests olfactory deficits. These measures are sensitive
olfaction appear to be impaired (i.e., identification, to age and gender effects. Chemosensory evoked poten-
threshold, discrimination, and memory). tials are unable to discern where the impairment is within
the olfactory pathway, but are considered among the only
objective ways of establishing smell loss.
Evaluation
Psychophysical
Clinical History Psychophysical methods are the most commonly used
assessment practices in both clinical and research settings.
Obtaining a detailed clinical history is critical in assessing In these techniques, stimuli are presented, and the patient or
olfactory deficits. Symptoms should be clearly defined, participant reports their perception (detection, discri-
and the clinician should attempt to determine the extent mination, identification); this category can be further
and duration of the perceived loss, as well as the sub-divided into threshold and suprathreshold tasks.
Anosmia A 177
Threshold Testing Odor Memory Test Odor memory test involve having the
A
Threshold testing is used to determine at what concen- individual smell an odor (or group of odors), and after a
tration a patient or participant can accurately detect the specified period of time, recognize the odor from a set of
presence of an odor. Two methods have been developed to distracters. Often, novel, non-descript odors are utilized
determine this threshold: the method of limits procedure to minimize the ability to label, and interference tasks are
and the single staircase procedure. In the method of limits introduced during delays to minimize rehearsal of the
procedure, a low concentration of a specific odor is odor labels/qualifiers.
presented, and the concentration is increased until it can
be detected. In the single staircase procedure, the concen-
tration is increased following trials in which the partici- Other Olfactory Assessment Tools
pant cannot detect the odor, and decreased following
correct detection. There are commercially available smell The Sniff Magnitude Test
threshold tests, for example, using felt-tipped pens and The sniff magnitude test is a recently developed clinical
squeeze bottles. Olfactometers can be used to present measure of olfaction based on the reflex-like reduction in
precise amounts of odorants through constant airflow. sniffing that occurs in response to detection of odors
However, many of these techniques can be cumbersome (especially unpleasant odors), but does not occur when
for clinical use. sniffing non-odorized air (Frank, Dulay, & Gestland,
2003). This response is observed in people with normal
Suprathreshold Tasks sense of smell, but is absent in those with anosmia. The
Suprathreshold tasks include rating scales/magnitude task involves having the patient sniff a canister that
estimation scales, odor identification tasks, and odor releases either a blank or an odor, while wearing a nasal
memory/recognition tasks. When using rating scales, the cannula connected to a device to measure the negative
participant rates the amount of the attribute perceived pressure created by the sniff. The test is quick to administer
(e.g., pleasantness); these may include category scales (about 5 min) and has minimal, if any, reliance on
(which category describes sensation) and line scales cognition, linguistic ability, and familiarity of odors.
(placement of mark on line with descriptors). When
using a magnitude estimation scales, a participant will Neuroimaging
assign a number to stimuli in relation to relative intensity. Imaging, particularly MRI, is clearly important for
identification of structural lesions that may be impinging
Odor Identification Tasks Odor identification tasks also on the olfactory system, or in assisting in diagnosis of
suprathreshold tasks, require participants to identify other neurologic disorders that may account for smell
odors, often by presenting scratch-and-sniff items, loss. CT is frequently used in identifying sinonasal disease.
tinctures in jars, or odorant-soaked tampons. These tasks MRI can also be useful in evaluating changes in olfactory
almost invariably include multiple choice options, as odor bulb volume due to viral, traumatic, or idiopathic olfac-
identification is otherwise extremely challenging even for tory dysfunction, with good relationship demonstrated
individuals with intact olfactory abilities. These tasks are between objective olfactometry (with chemosensory
easy to administer and the most frequent type of task used evoked potentials) and bulb volume. Functional scans,
in clinical settings, but can be somewhat costly depending in particular fMRI and PET, are also often used as research
on the task. The most widely used odor identification task tools in studying the functional organization of olfaction.
is the University of Pennsylvania Smell Identification These studies have shown involvement in the amygdala,
Task (UPSIT), which consists 40 micro-encapsulated piriform cortex, OFC, insula, anterior cingulate,
odorants presented in a 4-option, multiple choice format. thalamus, caudate, subiculum, upper pons, and cerebellar
Other, briefer measures include 12-item versions vermis, with different activation patterns depending on
(e.g., Cross-Cultural Smell Identification Test/Brief Smell the nature of the task (e.g., sniffing, smelling single odors,
Identification Test (BSIT), the BSIT-A designed especially discrimination, identification, etc.).
for AD, the BSIT-B designed especially for PD) and a
3-item screen (Pocket Smell Test). The UPSIT and BSIT
both have associated norms. Sniffin Sticks includes both a Treatment
threshold task and an odor identification task, and is
extensively normed in European samples (Hummell, Treatment is most promising in patients with smell loss
Kobal, Gudziol, & Mackay-Sim, 2007). associated with conduction problems. For example,
178 A Anosmia
antibiotic treatment, steroids, and allergy management Doty, R. L. (Ed.). (2003b). Handbook of olfaction and gustation (2nd ed.).
New York: Marcel Dekker.
may be helpful in reducing deficits associated with
Doty, R. L., & Bromley, S. M. (2004). Effects of drugs on olfaction and
inflammatory disease. Surgical removal of other obstruc- taste. Otolaryngologic Clinics of North America, 37, 12291254.
tions, such as nasal polyps, can also be effective in Frank, R. A., Dulay, M. F., & Gestland, R. C. (2003). Assessment of
restoring olfactory ability. In contrast, treatment of the Sniff Magnitude Test as a clinical test of olfactory function.
sensorineural/central neural problems is often less Physiology & Behavior, 78, 195204.
Gotfried, J. A., & Zald, D. H. (2005). On the scent of human olfactory
effective. Exceptions may include resection of tumors
orbitofrontal cortex: Meta-analysis and comparison to non-human
impinging on the olfactory system and, in some cases, primates. Brain Research Brain Research Review, 50, 287304.
resection of epileptogenic foci associated with olfactory Gray, A. J., Staples, V., Murren, K., Dhariwal, A., & Bentham, P. (2001).
seizures. Iatrogenic effects of medications are typically Olfactory identification is impaired in clinic-based patients
reversible with discontinuation of the medication and with vascular dementia and senile dementia of the Alzheimer type.
International Journal of Geriatric Psychiatry, 16, 513517.
eventual improvement in smell is expected after cessation
Hummell, T., Kobal, G., Gudziol, H., & Mackay-Sim, A. (2007).
of smoking. Recent work also suggests that olfactory Normative data for the sniffinsticks including tests of odor
training may improve olfaction in some patients identification, odor discrimination, and olfactory thresholds: an
(Hummel et al., 2009). Zinc or vitamin therapies are at upgrade based on a group of more than 3000 subjects. European
times prescribed to treat olfactory loss, but there is little Archives of Otorhinolaryngology, 264, 237243.
Hummel, T., Rissom, K., Reden, J., Hahner, A., Weidenbecher, M., &
evidence of benefit in the absence of associated defici-
Huttenbrink, K. B. (2009). Effects of olfactory training in patients
encies. Typically, the more severe and long-standing the with olfactory loss. Laryngoscope, 119, 496499.
smell loss, the less likely recovery is in sensorineural/ Knupfer, L., & Spiegel, R. (1986). Differences in olfactory test
central neural disorders. Especially for individuals performance between normal aged, Alzheimer and vascular type
who do not respond to treatment, education about the dementia individuals. International Journal of Geriatric Psychiatry,
1, 314.
safety implications of smell loss is important, given
Luzzi, S., Snowden, J. S., Neary, D., Coccia, M., Provinciali, L., & Lambon
concerns of the patients failure to detect hazardous Ralph, M. A. (2007). Distinct patterns of olfactory impairment in
odors (e.g., smoke) or spoiled food. Nutritional status Alzheimers disease, semantic dementia, frontotemporal dementia,
may also be compromised in patients with olfactory and corticobasal degeneration. Neuropsychologia, 45, 18231831.
deficits, and use of flavor enhancements in foods can be McLaughlin, N., & Westervelt, H. J. (2008). Odor identification deficits in
frontotemporal dementia: A preliminary study. Archives of Clinical
helpful in improving food intake (Schiffman, 2000).
Neuropsychology, 23, 119123.
McShane, R. H., Nagy, Z., Esiri, M. M., King, E., Joachim, C., Sullivan, N.,
et al. (2001). Anosmia in dementia is associated with Lewy
Cross References bodies rather than Alzheimers pathology. Journal of Neurology,
Neurosurgery, and Psychiatry, 70, 739743.
Mesholam, R. I., Moberg, P. H., Mahr, R. N., & Doty, R. L. (1998).
Cranial Nerves
Olfaction in neurodegenerative disease. A meta-analysis of olfactory
Olfaction functioning in Alzheimers and Parkinsons diseases. Archives of
Olfactory Bulb Neurology, 55, 8490.
Olfactory Tract Murphy, C., Doty, R. L., & Duncan, H. J. (2003). Clinical disorders of
olfaction. In R. L. Doty (Ed.), Handbook of olfaction and gustation
(2nd ed.). New York: Marcel Dekker.
Murphy, C., Schubert, C. R., Cruickshanks, K. J., Klein, B. E., Klein, R., &
References and Readings Nondahl, D. M. (2002). Prevalence of olfactory impairment in
older adults. Journal of the American Medical Association, 288,
Costanza, R. M., DiNardo, L. J., & Reiter, E. R. (2003). Head injury and 23072312.
olfaction. In R. L. Doty (Ed.), Handbook of olfaction and gustation Olichney, J. M., Murphy, C., Hofstetter, C. R., Foster, K., Hansen, L. A.,
(2nd ed.). New York: Marcel Dekker. Thal, L. J., et al. (2005). Anosmia is very common in the Lewy body
Devanand, D. P., Michaels-Marston, K. S., Liu, X., Pelton, G. H., variant of Alzheimers disease. Journal of Neurology, Neurosurgery,
Padilla, M., Marder, K., et al. (2000). Olfactory deficits in patients and Psychiatry, 76, 13421347.
with mild cognitive impairment predict Alzheimers disease at Pardini, M., Huey, E. D., Cavanagh, A. L., & Grafman, J. (2009). Olfactory
follow-up. American Journal of Psychiatry, 157, 13441405. function in corticobasal syndrome and frontotemporal dementia.
Devanand, D. P., Tabert, M. H., Cuasay, K., Manly, J. J., Schupf, N., Archives of Neurology, 66, 9296.
Brickman, A. M., et al. (in press). Olfactory identification deficits Schiffman, S. S. (2000). Intensification of sensory properties of food for
and MCI in a multi-ethnic elderly community sample. Neurobiology the elderly. Journal of Nutrition, 130, 92759305.
of Aging. Smutzer, G. S., Doty, R. L., Arnold, S. E., & Trojanowski, J. Q. (2003).
Doty, R. L. (2003a). Odor perception in neurodegenerative diseases. In Olfactory system neuropathology in Alzheimers disease Parkinsons
R. L. Doty (Ed.), Handbook of olfaction and gustation (2nd ed.). disease, and schizophrenia. In R. L. Doty (Ed.), Handbook of
New York: Marcel Dekker. olfaction and gustation (2nd ed.). New York: Marcel Dekker.
Anosognosia A 179
Upadhyay, U. D., & Holbrook, E. H. (2004). Olfactory loss as a result of day to the next. The more common hypotheses are that
toxic exposure. Otolaryngologic Clinics of North America, 37, A
the anosodiaphoria likely reflects the same type of neglect
11851207.
Westervelt, H. J., Bruce, J. M., Coon, W. G., & Tremont, G. (2008). Odor
or inattention that results in the original anosognosia,
identification in mild cognitive impairment subtypes. Journal of only less severe, is a result of a general emotional flatten-
Clinical and Experimental Neuropsychology, 30, 151156. ing or indifference that can follow right hemispheric
Westervelt, H. J., Stern, R. A., & Tremont, G. (2003). Odor identification lesions, or a combination of the two. (Heilman, Blonder,
deficits in diffuse Lewy body disease. Cognitive and Behavioral
Bowers, & Valenstein, 2003).
Neurology, 16, 9399.
Wilson, R. S., Schneider, J. A., Arnold, S. E., Tang, Y., Boyle, P. A., &
Bennett, D. A. (2007). Olfactory identification and incidence of mild
cognitive impairment in older age. Archives of General Psychiatry, Cross References
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Anosognosia
Denial (of Illness)
Anosodiaphoria
References and Readings
J OHN E. M ENDOZA
Tulane University Medical Center Adair, J. C., Schwartz, R. L., & Barrett, A. M. (2003). Anosognosia. In
K. Heilman & E. Valenstein (Eds.), Clinical neuropsychology (4th ed.,
New Orleans, LA, USA
pp. 185214). New York: Oxford University Press.
Critchley, M. (1969). The parietal lobes. New York: Hafner.
Heilman, K. M., Blonder, L. X., Bowers, D., & Valenstein, E. (2003).
Definition Emotional disorders associated with neurological diseases. In K. M.
Heilman & E. Valenstein (Eds.), Clinical neuropsychology (4th ed.,
pp. 447478). New York: Oxford University Press.
Anosodiaphoria is defined as the failure to fully appreciate
Prigatano, G. P., & Schacter, D. L. (Eds.), (1991). Awareness of deficit after
the significance of a neurological deficit as a result of a brain injury. New York: Oxford.
brain lesion.
appear to deny illness or be unaware of their disabilities. body (hemiparesis). Many of these patients will be un-
Seneca, the Stoic philosopher noted this about 2,000 aware of their weakness and when asked about the pres-
years ago, but the first modern description of a patient ence of weakness, they will deny this disability. Several,
with unawareness-denial was by von Monakow (1885). not mutually exclusive, mechanisms have been used to
Although there were other investigators who wrote about explain this phenomenon.
this striking disorder, it was Babinski (1914), who coined Psychological denial. Weinstein and Kahn (1955) who
the term anosognosia. This word comes from three roots: brought modern attention to this syndrome, posited
a = without, noso = disease, gnosis = knowledge. In that for many people having a stroke with weakness was
addition to describing patients who were unaware of a psychologically traumatic event, and the means by
their illness or disability, Babinski described other patients which some people deal with this trauma is to use
who appeared to be aware but remained unconcerned. He psychological denial. To test this hypothesis, Weinstein
called this disorder, anosodiaphoria. and Kahn studied patients who had anosognosia and
There are many forms of anosognosia and these found that even before their stroke these patients
forms are related to the nature of a patients disability. frequently used this denial defense mechanism.
When Babinski first used this term, the patients he Some investigators have noted that anosognosia for
described denied or were unaware of their hemiparesis. hemiplegia is more often associated with a left than right
Anton (1898) described patients who were unable to see hemiparesis. The psychological denial theory of anosog-
because they had destroyed their primary visual cortex, nosia cannot explain this asymmetry. Many patients with
but were unaware or denied their blindness. Patients left hemisphere injury, however, are aphasic and have
with Korsakoff s amnesic disorder are unaware of their problems with both the comprehension of questions
memory loss and aphasic patients such as those with (What is wrong with you? Are you weak?) as well as
Wernickes aphasia appear to be unaware of their jargon speakinganswering questions. Thus, Weinstein and
speech. Kahn thought what appeared to be a hemispheric asym-
metry was induced by a sampling bias.
Using selective hemispheric anesthesia (the Wada
Current Knowledge study) and questioning the patient after they recover
from anesthesia revealed that unawareness of the hemi-
Although of great academic interest, the presence of ano- plegia (anosognosia) was more common with the right
sognosia or anosodiaphoria has important medical impli- than left hemisphere anesthesia (Gilmore et al., 1992).
cations. For example, there are now treatments for stroke After the selective hemispheric anesthesia has worn off
that must be given within hours of the onset of symptoms. there is no aphasia or a need for psychological denial. The
The patients who are unaware of their disabilities or rightleft hemisphere asymmetries found were within
undervalue their importance might not seek immediate subjects, and thus premorbid personality can also not
medial attention. In addition, people who have disabilities account for this asymmetry. Although this study suggests
but are not aware of these disabilities might inadvertently that denial cannot entirely explain anosognosia for hemi-
injure themselves and/or others. Rehabilitation works plegia, denial might be used by many people to help deal
best, when patients are strongly motivated to get well. with diseases and disabilities.
When a person is either unconcerned or unaware of Failure of feedback. To know something is impaired, a
their disabilities, they are not motivated and unmotivated person requires feedback. Many investigators have sug-
patients are less likely to benefit from these treatments. gested that it is a failure of feedback, induced by either
They might even refuse to undergo rehabilitation and sensory loss (e.g., proprioception and hemianopia) or
they might not take their medications that can reduce inattention neglect, spatial or personal, that accounts for
their disability or possibly prevent further possible anosognosia of hemiplegia. That inattention neglect is
brain damage. more commonly associated with right hemisphere injury
might also account for the asymmetries of anosognosia.
Studies from our laboratory have revealed when
Possible Mechanisms of Anosognosia for undergoing selective right hemisphere anesthesia, during
Hemiplegia the time these patients demonstrate shoulder weakness
their shoulder proprioception is intact. To learn if this
Patients with hemispheric strokes often develop an inabil- disorder could be related to neglect, spatial or personal,
ity to use the arm-hand on the contralesional side of their we brought their hemiplegic left forelimb over to the right
Anosognosia A 181
side of their body and to their right visual field. To make Phantom movements. Limb amputation is often associated
A
certain subjects see their hand, we wrote a number on with a perception that the limb is still present and this
their hand and subjects were able to read these numbers. perception is thought to be related to the continued
Despite these strategies many, but not all, patients still presence of a brain representation of that missing phan-
denied weakness of that hand. Thus, a failure of feedback tom limb. When patients with a hemiparesis are asked to
can only explain anosognosia in some patients. In support move a limb, many often perceive that the paretic limb is
of this postulate, several investigators have reported dis- moving, and this phantom movement in combination
sociations between the presence of spatial neglect and with impaired feedback might account for anosognosia.
anosognosia. During selective hemispheric anesthesia (Wada test), we
Asomatognosia hypothesis. While patients with personal had blindfolded subjects with left hemiplegia attempt to
neglect might be unaware of the parts of their body, raise their paretic left arm and we then asked them to raise
patients with asomatognosia do not feel or claim that their right (non-paretic) arm to the same level as they
certain body parts belong to them. It has been posited perceived left arm. Some of the patients we tested did raise
that asomatognosia is caused by the alteration of the their right arm, suggesting that they had phantom move-
brains representation of the body, a body schema. Like ments, but we found no significant relationship between
spatial and personal neglect, asomatognosia is more com- phantom movements and anosognosia.
monly associated with right than left hemisphere lesions. Intentional motor disorder. Patients with right hemisphere
If patients with right hemisphere injury do not believe lesions often demonstrate contralesional limb akinesia
their left arm-hand belongs to them, they will not recog- also called motor neglect. Many of these patients do not
nize their own weakness. During right hemispheric anes- attempt to spontaneously move their akinetic arm and
thesia, the patients with left hemiplegia were shown their while less common some do not even attempt to move
left hand or someone elses left hand in a restricted view this arm to command. Limb akinesia can occur both with
box that projected to their right visual field. The patients and without a hemiplegia. Patients with limb akinesia
were asked if the hand they were viewing was their own or might not discover that they are weak because they do
another persons hand. We found that there were some not attempt to move this left arm. If they do not attempt
patients who had anosognosia who also had asomatogno- to move this arm, they will not experience a dissociation
sia, but only a small proportion. Thus, asomatognosia can between their expectations and performance, and it is this
also not fully account for this disorder. dissociation that alerts people that there is a problem.
Disconnection hypothesis. When a patient with a complete Providing external motivation such as suggestions or
callosal disconnection receives a stimulus to the left visual commands might entice patients to attempt a movement
field or on the left side of the body and is asked to tell the and with these commands some patients do discover their
examiner the nature of the stimulus, the left language weakness. Electromyographic studies have also provided
speech hemisphere often confabulates a response. evidence in support of this akinesia hypothesis.
Geschwind (1965) noted that large right hemisphere Summary. Based on the above discussion it appears that
lesions can both injure the right hemispheres cortex and several mechanisms might contribute to the presence of
intrahemispheric networks, as well as induce a interhemi- anosognosia for hemiplegia.
spheric disconnection. Thus, when asked about weakness,
the left hemisphere which is disconnected from the right
will confabulate a response I am not weak. The obser- Possible Mechanisms of Anosognosia for
vation mentioned above, where during the right hemi- Amnesia and Cortical Blindness
sphere anesthesia the patients left hand is brought over to
the right visual field and thus has access to the left lan- Damage to three interconnected brain networks can pro-
guagespeech dominant hemisphere, also tests this dis- duce amnesia, an impairment in the episodic memory
connection hypothesis. As mentioned, in few patients system: (1) the medial temporal lobe Papez circuit
when their arm could be visualized in the right visual (e.g., hippocampus, entorhinal and perirhinal cortex, for-
field left hemisphere, they did recognize their weakness. nix, the mammillary bodies, the mammillothalamic tract,
In these cases, we cannot be sure if their anosognosia was the anterior thalamus, and the retrosplenial cortex);
induced by a failure in feedback or a disconnection. Fu- (2) the dorsomedial thalamus; and (3) the basal forebrain
ture research will have to learn if these mechanisms can be (medial septal nucleus and the diagonal band of Broca),
dissociated. However, as mentioned above this procedure which provide acetylcholine to the hippocampus. Amne-
only helps a small minority of patients. sic patients with medial temporal lesions are often aware
182 A Anosognosia
of their disability and patients with damage to the basal suggesting that their unawareness might have been related
forebrain and to the medial thalamus are often unaware of to not being able to closely attend to their output. These
their memory deficit. aphasic patients might have focused their attention on
The reason for this dichotomy is not fully known, but what they were attempting to say rather than how they
the dorsomedial thalamic nucleus is heavily connected said it.
with the frontal lobes and damage to this dorsomedial
nucleus induces frontal dysfunction. Damage to the basal
forebrain is also often associated with frontal dysfunction.
Frontal lobe dysfunction is often associated with impaired
Future Directions
recall but not recognition, suggesting that the problem is
Anosognosia, the failure to recognize a disease or a dis-
not with the consolidation of memories, but rather re-
ability, might delay treatment, interfere with rehabilita-
trieval. The patients with amnesia from a thalamic or
tion, and put people in danger. Patients might be
basal forebrain injury, more often confabulate memories
anosognosic for a variety of neurological disorders such
than do those with medial temporal lobe damage. Since
as weakness, sensory loss, personal and spatial neglect,
these patients retrieve memories and have no means of
memory loss, and aphasia. There appears to be a variety
testing these memories veracity, they might be unaware
of mechanism that might account for anosognosia includ-
that their recall is incorrect and therefore they might be
ing psychological denial, impaired and false feedback,
unaware of their memory disorder.
alterations of the body schema, failures to test systems,
Blindness. Patients with Antons syndrome have blindness
and to initiate behaviors. Future research is needed. In
from damage to their primary visual cortex, usually from
addition to continuing to define and test possible
stroke. These patients often deny their blindness, confab-
mechanisms, effective treatments for these disorders are
ulate responses, and are unaware they are blind, anosog-
needed.
nosic. The reason why these patients are not aware of their
blindness is not known. We examined a patient with
Antons syndrome who had intact visual imagery. Perhaps
since these patients have intact visual imagery and cannot Cross References
receive visual input, this imagery is mistaken for online
input. Attention
Awareness
Consciousness
Possible Mechanisms for Unawareness Impaired Self-Awareness
of Aphasia
den infrakorticalen Opticuscentren und zum N. opticus. Archiv fur The CA1 cells of the hippocampus contain high
Psychiatrie und Nervenkrankheiten, 16, 151199. A
concentrations of glutamate and they are particularly
Weinstein, E. A., & Kahn, R. L. (1955). Denial of illness: Symbolic and
physiological aspects. Springfield, IL: Charles C. Thomas.
vulnerable to subnormal oxygenation levels. Therefore,
it appears that the action of glutamate on these cells is
the putative mechanism mediating cell death in this
region of the hippocampus and helps explain many
of the signs and symptoms associated with anoxia
Anosphrasia (Bonner & Bonner, 1991).
Anosmia
Signs and Symptoms
Categories
Anoxic Encepathopathy
The ACA can be divided into five segments A1A5,
Anoxia although it should be noted that some of the literature is
describing the A1 segment when referring to the ACA
(Sawada & Kazui, 1995).
Cross References
and posterior to the prefrontal cortex. The ACC was once severe depression and schizophrenia. While frontal lobot-
A
viewed as a single limbic structure, forming an important omy resulted in a reduction in agitation and other severe
part of the Papez circuit, though in reality analysis of its psychiatric symptoms, surgical removal of the frontal lobe
cytoarchitecture indicates that it consists of regions with caused severe cognitive dysfunction. Given that the orbital
different cell types. Its cell characteristics are agranular, frontal region was considered to be particularly important
and therefore are distinct from the cortex. for the control of impulses and emotional regulation,
The ACC encompasses several Broadmann areas, subsequent psychosurgical approaches typically restricted
including areas 24, 25, 32, and 33. The ACC wraps around ablation to these areas, often through leukotomy.
the corpus callosum, having the appearance of a collar Unfortunately, patients undergoing this procedure often
or belt. In fact, the term cingulum means belt in Latin. exhibited marked personality change, with flattening of
A large volume of the ventral ACC consists of Area 24, affect, apathy, and other undesirable effects. A third gen-
which merges with the posterior cingulate cortex eration of psychosurgical procedures ensued with efforts
(Area 23) along the posterior half of the corpus callosum. to target brain areas more selectively. The ACC was a
The division between the ACC and posterior cingulate is point of focus because of its association with both limbic
undifferentiated to a large extent, though these areas can areas as well as the frontal cortex. Beginning in the late
be separated based on the cortical layer IV in the posterior 1950s, cingulotomy was developed as an alternative to
cingulate. Anterior to Area 24 is the subgenual cortex frontal ablation. Early studies suggested that it had few
(Area 25), which may be considered to be distinct from adverse cognitive effects, and that it seemed helpful
other ACC areas. Anterior to this region is the dorsal for certain patients, particularly those with intractable
ACC, including areas 32 and 33. The midanterior section obsessivecompulsive symptoms, chronic pain, and
of the ACC is often termed midcingulate (mACC), while opiate dependence. There was also some evidence that it
the more posterior section is termed perigenual cingulate was helpful for patients with severe chronic depression,
(pACC). These areas have distinct cell characteristics, and though the basis for these effects may relate to reductions
there is strong evidence of functional differences across in emotional tension, obsessive thought processes, and
subareas of the ACC. other depression-associated problems.
Primary afferent input to the ACC is received via Literature on the psychosurgical effects of cingulotomy
axons from the midline and intralaminar thalamic nuclei, provided compelling evidence that the ACC plays a role
with the anterior nucleus receiving input from mamillary in human emotional experience and regulation. Further-
neurons, which in turn has projections from the subicu- more, there is also evidence that the ACC influences
lum. The ACC is associated with a large white-matter autonomic nervous system response, including heart
bundle, the cingulum, through which signals are trans- rate, blood pressure, and galvanic skin response, with
mitted to other limbic areas. As a paralimbic area, the these responses showing alterations in the rate of habitua-
ACC is a transition area between subcortical and limbic tion following cingulotomy (Cohen et al., 1995). Yet, most
structures, such as the amygdala and cortical areas, most early studies of the effects of cingulotomy suggested that
notably in the frontal lobes. The posterior ACC has heavy the ACC had little impact on intellectual ability or most
input from the amygdala, whereas the mid-ACC receives neuropsychological functions. Postsurgery patients tended
greater input from parietal areas. Connections between not to experience significant memory, language, or visual
the ACC and the mesial, ventral, and orbital frontal areas change. Subsequent controlled studies indicated that while
appear to be particularly important for emotional and these functions are largely spared following cingulotomy,
behavioral regulation. there are alterations in some attention-related functions,
most notably attentional focus, intention, and response
selection and control (Cohen et al., 2001). These changes
Function correspond with reductions in emotional tension and
distress, and also a tendency for reduced self-initiation
Current knowledge regarding the functions of the ACC of behavior (Cohen et al., 2001).
has its origins in the psychosurgical efforts of the mid- Recent experimental evidence suggests a functional
twentieth century. At that time, the role of the frontal dissociation between the posterior and middle ACC. The
lobes in emotion and behavioral control were recognized, mid-ACC plays a role in response selection and control,
and frontal lobotomy was experimented with as a means including intention and planning to act or to engage
of treating a variety of psychiatric conditions, including in cognitive operations. It has also been implicated in
186 A Anterior Cingulate System
processing new motor programs, working memory, and implicated in substance abuse, including opiate addiction
mismatch detection. In contrast, the posterior ACC and nicotine dependence. Citalopram binds to the sero-
appears to play a more direct role in emotional proces- tonin transporter at very high levels in the posterior ACC,
sing, though these areas are likely highly interconnected, which may account for the effects of this type of drug on
enabling the integration of emotional and attentional reducing mood, anxiety, and pain symptoms. There is
processes (Bush, Luu, & Posner, 2000). also evidence that functional response of the ACC varies
Interest in the functional significance of ACC as a function of risk-reward dynamics, appetitive state,
increased dramatically with the advent of functional neu- and motivation. Neuroimaging studies have begun to
roimaging methods. Activation of the ACC is evident point to its role in a variety of behavior problems, such
across a wide range of tasks. In fact, it is among the as obesity and inactivity.
most responsive areas of the brain on fMRI. This probably
reflects the fact that it plays an increased role when tasks
require motivation and drive to complete and where there Cross References
is demand for attentional effort and focus.
The ACC plays a significant role in response to the Apathy
conflict during cognitive tasks associated with decision Executive Function
making and the need to resolve competing or discrepant Intention
information (Botvinick et al., 1999). Some cognitive neu- Psychosurgery
roscientists argue that conflict monitoring is the primary
function of the ACC, though it seems likely that this
capacity is closely associated with the broader functions References and Readings
of regulation of drive, emotion, attention, and response
intention; and selection, initiation, and persistence Ballentine, H. T. Jr., Levey, B. A., Dagi, T. F., & Diriunas, I. B. (1977).
relative to situational demands. Neurosurgical treatment in psychiatry, pain, and epilepsy. In W. H.
Sweet, S. Obrador, & J. G. Martin-Rodriques (Eds.), Cingulotomy
for psychiatric illness: Report of 13 years experience (pp. 333353).
Illness Baltimore, MD: University Park Press.
Bush, G., Luu, P., & Posner, M. I. (2000). Cognitive and emotional
influences in anterior cingulate cortex. Trends in Cognitive Sciences,
Focal brain diseases affecting only the ACC are rare. 4(6), 215222.
However, the ACC is vulnerable to the effects of tumor, Botvinick, M., Nystrom, L. E., Fissell, K., Carter, C. S., & Cohen, J. D.
stroke, and other neurological conditions involving ante- (1999). Conflict monitoring versus selection-for-action in anterior
rior cortical infarction or mass action. Unilateral ablation cingulate cortex. Nature, 402(6758), 179181.
Cohen, R. A., Kaplan, R. F., Meadows, M. E., & Wilkinson, H. (1994).
of the ACC in laboratory studies of primates, and also
Habituation and sensitization of the orienting response
secondary to stroke, has been shown to produce hemi- following bilateral anterior cingulotomy. Neuropsychologia, 32(5),
neglect syndrome, providing further evidence that the 609617.
ACC plays an important role in attention. There is evi- Cohen, R. A., Kaplan, R. F., Zuffante, P., Moser, D. J., Jenkins, M. A.,
dence of ACC dysfunction secondary to atrophy asso- Salloway, S., et al. (1999). Alteration of intention and self-initiated
action associated with bilateral anterior cingulotomy. Journal of
ciated with neurodegenerative conditions, such as
Neuropsychiatry and Clinical Neurosciences, 11(4), 444453.
Alzheimers disease, which may contribute to symptoms Cohen, R. A., Paul, R., Zawacki, T. M., Moser, D. J., Sweet, L., &
of apathy and behavioral inertia in certain patients. How- Wilkinson, H. (2001). Emotional and personality changes following
ever, these changes are usually part of a much more global cingulotomy. Emotion, 1(1), 3850.
pattern of brain abnormality. Devinsky, O., Morrell, M. J., & Vogt, B. A. (1995). Contributions of
anterior cingulate cortex to behaviour. Brain, 118(Pt. 1), 279306.
The ACC plays a more obvious role in psychiatric
illness and also the range of normal behavior. Activation
of the ACC occurs in association with increased levels of
distress and emotional tension and anxiety. It also tends
to be associated with obsessive rumination and preoccu- Anterior Cingulate System
pation with internal states and signals, such as pain and
impulses to seek reward. Accordingly, the ACC has been Mesial Frontal System
Anterior Communicating Artery A 187
Synonyms Synonyms
Interhemispheric commissure Communicating artery; ACoA
Description
Definition
The anterior communicating artery (ACoA) intercon-
A relatively small commissure in the basal forebrain lying nects the two anterior cerebral arteries just rostral to the
above the optic chiasm and anterior to the main columns optic chiasm and resides at the anterior portion of the
of the fornix that connects homologous areas of the mid- Circle of Willis.
dle and inferior temporal gyri, including parts of the Ruptured ACoA aneurysms may impact a variety
olfactory cortices (Fig. 1). of neurologic, neuropsychological, and psychological
functions. This may, in part, be due to the fact that
the perforating branches of the ACoA supply the anteri-
or hypothalamus, mesial anterior commissure, lamina
termininalis, and areas implicated in executive function,
memory, and affect (e.g., fornix and basal forebrain, septal
nuclei, nucleus accumbens, diagonal band, and the medial
Acq Tm substantia innominata) (DeLuca & Diamond, 1995;
Sawada & Kazui, 1995). The profound memory disorders
that may be associated with ACoA aneurysm rupture do
not appear to directly involve neuroanatomic areas tradi-
tionally implicated in amnesia, which makes the ACoA
artery of both clinical and theoretical interest.
Etiology
ACoA aneurysms may develop as a result of trauma,
infections, degenerative diseases, or a congenital defect
(Parkin & Leng, 1993). Aneurysms often become symp-
tomatic as a result of subarachnoid hemorrhage (SAH)
following rupture (Riina, Lemole, & Spetzler, 2002). SAH
has an overall incidence of 10 to 16 per 100,000 and is a
major cause of mortality and morbidity (Clinchot,
Kaplan, Murray, & Pease, 1994).
Mechanisms
resulting in cerebral infarction and impairments in cogni- differences most notable on tests of executive and memo-
tion, personality, and functional activities (DeLuca & ry function. Relationships between recovery of executive
Diamond, 1995; McCormick, 1984). Damage to the function and temporal gradients in retrograde amnesia
basal forebrain region may help account for many of the have been reported, with improvements in executive func-
cognitive impairments that are observed in ACoA aneu- tion accompanied by parallel improvements in the severi-
rysm due to the fact that the basal forebrain region con- ty of retrograde amnesia. Improvement in the recall of
tains cholinergic neurons that project to the hippocampus complex visual-spatial information and an enhanced abil-
and amygdala, via the medial forebrain bundle to the ity to benefit from an executive learning strategy have also
entire cerebral cortex. Damage to this area would, there- been reported with little improvement on traditional
fore, particularly interfere with cholinergic activation of measures of memory or executive function (Diamond,
structures and circuits implicated in memory within the DeLuca, & Kelley, 1997a). Recovery from neuropsycho-
medial temporal lobe (Schnider & Landis, 1995). More- logical disturbances is generally poorer in patients with
over, vascular compromise of the perforating branches of ventral frontal lesion compared to those with basal fore-
the ACoA are believed to impact functional areas (e.g., brain and striate lesions.
executive function, memory, and affect) that are inner- Surgical outcome and prognosis following aneurysms
vated by these vascular branches. There is general agree- depend on multiple factors (e.g., initial clinical status,
ment in the literature suggesting that personality changes localization of aneurysm, age, and the morphological
following ACoA aneurysm are a result of frontal lobe characteristics of the aneurysm). Comparisons of clipping
dysfunction, particularly in the medio-basal zones along versus endovascular embolization procedures have shown
the distribution of the anterior cerebral artery. The sub- that, in a number of studies, clipped patients have more
callosal perforating artery has, in fact, been implicated in severe cognitive impairments than embolization patients
and may mediate personality changes and memory and that 33% of clipped patients had impairments in
impairments. memory and executive functioning (Chan, Ho, & Poon,
2002).
Generally, the severity of cognitive impairment has
Epidemiological Factors predictive value for functional status particularly with
respect to levels of required supervision at discharge
Rupture of cerebral aneurysms strikes at a mean age of
(Saciri & Kos, 2002).
50 years and accounts for 510% of all strokes (Dombovy,
Some work suggests that recovery of executive func-
Drew-Cates, & Serdans, 1998), and approximately
tion and not short- and long-term memory may, in fact,
8595% of all aneurysms develop at the anterior portion
be the best predictor of the ability to return to work
of the cerebral arterial supply, primarily at the Circle of
(DeLuca & Diamond, 1995).
Willis (Adams & Biller, 1992; Ropper, Brown, Adams, &
Victor, 2005). The ACoA is one of the most common sites
of cerebral aneurysm and is the most frequent site of
Neuropsychological and Psychological
cerebral infarct following aneurysm rupture (DeLuca &
Diamond, 1995; McCormick, 1984). About 3040% of
Outcomes
cerebral aneurysms affect the ACoA artery, and 90% of
Neuropsychological
cases are asymptomatic (Beeckmans, Vancoillie, & Michiels,
1998; Manconi, Paolino, Casetta, & Granieri, 2001) with
It is generally concluded that verbal intellectual skills,
various reports suggesting that the incidence of rupture is
language functions, visuo-spatial skills, and attention/
highest between 40 and 70 years of age (McCormick,
concentration are within normal limits or only mildly
1984; Sethi, Moore, Dervin, Clifton, & MacSweeney,
impaired, although complex concentration appears to be
2000) and that rupture occurs more frequently in females
reduced. An increased sensitivity to interference may be a
(i.e., 60% of cases) (Adams & Biller, 1992).
defining feature among ACoA amnesics and between
ACoA amnesics and diencephalic-mesial and temporal
Natural History, Prognostic Factors, and amnesics. More severe impairments are seen in delayed
Outcomes versus immediate memory and in executive function
(DeLuca & Diamond, 1995). Impairments in spatio-
With respect to impairment and chronicity, acute ACoA temporal discrimination appear similar to other popula-
patients are more impaired than chronic ones with tions with frontal lobe dysfunction (Schacter, 1987).
Anterior Communicating Artery A 189
Implicit memory involving data- and concept-driven re- statements or actions that involve distortions that are
A
trieval processes and behavioral and physiological indices unintentional (Moscovitch & Melo, 1997) with two dis-
(Diamond, Mayes, & Meudell, 1996) appears to be rela- tinct types of confabulation generally recognized in the
tively intact, although the evidence is sparse. Procedural literature, spontaneous and provoked (Kopelman, 1987).
memory on serial reaction time and mirror-reading tasks The key difference between provoked and spontaneous
also appears to be preserved. confabulation is that in spontaneous confabulation the
Spatial working memory in ACoA patients has been confabulation guides actions. Recovery from confabula-
reported to be impaired, and the impairment profile is tion appears to parallel improvement in temporal context
similar to patients with temporal lobe excisions. ACoA confusion, and recovery can occur in the absence of sig-
patients have displayed impairments in semantic memo- nificant improvement on traditional tests of memory and
ry, and difficulties to both the acquisition and recall of executive function.
verbal information showing little initial learning, a passive With respect to psychosocial outcomes, a significant
learning style, a flat learning slope, and impaired recogni- percentage of SAH survivors are left with cognitive,
tion discrimination, in addition to emitting a high num- emotional, and behavioral changes that can profoundly
ber of intrusions and false positives (Diamond, DeLuca, & impact their daily lives. Compared with controls,
Kelley, 1997b). ACoAs have shown impairments in infor- SAH patients display an increased incidence of mood
mation processing and autobiographical memory (espe- disturbance, cognitive impairment, and lower levels of
cially for events associated with context). ACoA amnesics independence, and participation on measures that reflect
(i.e., with putative basal forebrain damage) have exhibited social functioning. Levels of productive employment
impairments in delay eyeblink classical conditioning are generally reduced and many patients show clinically
(Myers et al., 2001), event-related potentials (ERPs), and significant posttraumatic stress symptomatology (see
in prospective remembering. Table 1 for a list of neuropsychological and psychological
impairments).
Psychological
Assessment and Treatment
ACoAs have displayed increased risk-taking on tasks in
which choices were associated with different magnitudes Given the wide range of impairments associated with
of reward and punishment. Confabulation is observed in ACoA aneurysm, it may be advisable for clinicians to
a subset of ACoA aneurysm patients and is manifested by use assessments that focus on those impairments that
Anterior Communicating Artery. Table 1 Neuropsychological and psychological impairments associated with ACoA aneurysm
are most salient and have the greatest impact on activities Cross References
of daily living (ADLs). Impairments in memory, executive
function, and attention/concentration as well as mood Activities of Daily Living (ADLs)
figure prominently following ACoA aneurysm rupture Amnesia
and should be part of routine assessment. For example, Aneurysm
assessments should examine set-shifting (e.g., Wisconsin Anterior Cerebral Artery
Card Sort Test (WCST) and Trails B), verbal and visual Confabulation
fluency (e.g., CFT/FAS and Design Fluency Test), verbal Executive Functioning
recall and recognition (e.g., California Verbal Learning Rey Complex Figure Test
Test (CVLT)), visual recall (e.g., ReyOsterreith Complex
Figure Test (ROCFT)), sustained attention (e.g., Cancel-
lation Test), information processing speed (e.g., n-back References and Readings
tasks), and impaired abstraction (e.g., Cognitive Estima-
tion Test (CET)). Adams, H. P., & Biller, J. (1992). Hemorrhagic intracranial vascular
In some cases, modification of existing assessment disease. In A. B. Baker & R. J. Joynt (Eds.), Clinical neurology
tools can be an effective way to enhance the assessment (vol. 2). Philadelphia: J. B. Lippincott.
process. For example, the ReyOsterrieth Organizational Beeckmans, K., Vancoillie, P., & Michiels, K. (1998). Neuropsychological
deficits in patients with an anterior communicating artery syn-
and Extended Memory (ROEM) test, which is a modifi-
drome: A multiple case study. Acta Neurologica Belgica, 98(3),
cation of the ROCFT, was reported to help identify 266278.
mechanisms underlying the nature of the impaired mem- Chan, A., Ho, S., & Poon, W. S. (2002). Neuropsychological sequelae of
ory in ACoA amnesics by using measures of recall and patients treated with microsurgical clipping or endovascular embo-
recognition (e.g., subunit recognition, spatial arrange- lization for anterior communicating artery aneurysm. European
Neurology, 47, 3744.
ment, and whole figure recognition). Moreover, encoding
Clinchot, D. M., Kaplan, P., Murray, D. M., & Pease, W. S. (1994).
and recall were improved by using an executive organiza- Cerebral aneurysms and arteriovenous malformations: Implications
tional strategy, in addition to identifying patients who for rehabilitation. Archives of Physical Medicine and Rehabilitation,
were more likely to benefit from such an intervention 75(12), 13421351.
(Diamond, DeLuca, & Kelly, 1997a; Prignatano & DeLuca, Cummings, J. L., & Trimble, M. R. (1995). A concise guide to neuropsychi-
atry and behavioral neurology. Washington, DC: American Psychiat-
1999).
ric Press.
Some work suggests that cognitive rehabilitation can DeLuca, J., & Diamond, B. J. (1995). Aneurysm of the anterior commu-
help increase compensatory strategies for attention and nicating artery: A review of neuroanatomical and neuropsychological
memory dysfunction and that rehabilitation can help sequelae. Journal of Clinical and Experimental Neuropsychology,
improve professional activities as well as ADLs with posi- 17(1), 100121.
Diamond, B. J., Mayes, A. R., & Meudell, P. (1996). Autonomic and
tive rehabilitation outcomes primarily associated with
recognition indices of aware and unaware memory in amnesics
changes in memory and attention. In a mixed sample of and healthy subjects. Cortex, 32, 439459.
SAH patients, a majority of survivors who receive inpa- Diamond, B. J., DeLuca, J., & Kelley, S. M. (1997a). Executive and
tient rehabilitation attain physical independence but memory impairment in patients with anterior communicating ar-
impairments in cognition and ADLs persist in upwards tery aneurysm. Brain and Cognition, 35, 340341.
Diamond, B. J., DeLuca, J., & Kelley, S. M. (1997b). Verbal learning in
of 40% of the patients (Dombovy, Drew-Cates, & Serdans,
anterior communicating artery aneurysm and multiple sclerosis
1998). Patients have generally shown impairments patients: Performance on the California verbal learning test. Applied
15 years poststroke, in visual short-term memory, reac- Neuropsychology. 4, 8998.
tion-time, verbal long-term memory, concentration, and Dombovy, M. L., Drew-Cates, J., & Serdans, R. (1998). Recovery and
language and information processing. Evaluation several rehabilitation following subarachnoid haemorrhage: Part II. Long-
term follow-up. Brain Injury, 12(10), 887894.
years after SAH associated with ACoA aneurysm rupture
Kopelman, M. D. (1987). Two types of confabulation. Journal of Neurolo-
has shown that cognitive problems negatively correlate gy, Neurosurgery and Psychiatry, 50(11), 14821487.
with the level of community integration and that impair- Manconi, M., Paolino, E., Casetta, I., & Granieri, E. (2001). Anosmia in a
ments in visual memory, verbal memory, and executive giant anterior communicating artery aneurysm. Archives of Neurolo-
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McCormick, W. F. (1984). Pathology and pathogenesis of intracranial
being characterized as having a good outcome, many
saccular aneurysms. Seminars in Neurology, 4(3), 291303.
ACoA patients continue to exhibit persistent cognitive Moscovitch, M., & Melo, B. (1997). Strategic retrieval and the frontal
impairments that negatively impact psychosocial func- lobes: Evidence from confabulation and amnesia. Neuropsychologia,
tioning (Ravnik et al., 2006). 35(7), 10171034.
Anterograde Amnesia A 191
Myers, C. E., DeLuca, J., Schultheis, M. T., Schnirman, G. M., Ermita, age 27, HM underwent bilateral resection of the medial
B. R., Diamond, B. J., Warren, S. G., & Gluck, M. (2001). Impaired A
temporal lobes for alleviation of refractory seizures, which
delay eyeblink classical conditioning in individuals with anterograde
amnesia resulting from anterior communicating artery aneurysm.
had become progressively more severe following a head
Behavioral Neuroscience, 115(3), 560570. injury he had suffered at age 9. The resection was success-
Parkin, A., & Leng R. C. (1993). Neuropsychology of the amnestic syn- ful in reducing his seizures but, unexpectedly, following
drome: Hove, U.K.: Lawrence Erlbaum. the treatment he was unable to remember his normal
Prignatano, G., & DeLuca, J. (1999). Methodological issues in research on
daily activities. For example, he could not recall eating
neuropsychological and intellectual assessment. In P. C. Kendall,
J. Butcher, & G. Holmbeck (Eds.), Handbook of research methods in
his meal within minutes of having finished it, and he
clinical psychology (pp. 241250). New York: Wiley. could not remember having had a conversation minutes
Ravnik, J., Starovasnik, B., Sesok, S., Pirtosek3, Z., Svigelj, V., Bunc, G., after it ended. He was unable to remember his regular
et al. (2006). Long-term cognitive deficits in patients with good caregivers, even though he could converse and interact
outcomes after aneurysmal subarachnoid hemorrhage from anterior
normally with them when they were present. These find-
communicating artery. Croat Medical Journal, 47, 253263.
Riina, H. A., Lemole, G. M., Jr., & Spetzler, R. F. (2002). Anterior
ings established that intact medial temporal lobes are
communicating artery aneurysms. Neurosurgery, 51(4), 993996. critical for normal memory function. HMs medial tem-
Ropper, A. H., Brown, R. H., Adams, R. D., & Victor, M. (2005). Adams & poral lobe resection had left him with a dense anterograde
Victors principles of neurology. New York: McGraw-Hill. amnesia, despite his having intact intelligence, attention,
Saciri, B. M., & Kos, N. (2002). Aneurysmal subarachnoid haemorrhage:
language function, and social skills. With respect to his
Outcomes of early rehabilitation after surgical repair of ruptured
intracranial aneurysms. Journal of Neurology Neurosurgery and Psy-
memory for the events that preceded his surgery, it was
chiatry, 72(3), 334337. initially thought that his retrograde amnesia ( Retro-
Sawada, T., & Kazui, S. (1995). Anterior cerebral artery. In J. Bogousslavsky grade Amnesia) was limited to approximately 2 years
& L. Caplan (Eds.), Stroke Syndromes (pp. 235246). Cambridge: prior to the operation, but more recent findings indicate
Cambridge University Press.
that he had a more extensive retrograde amnesia that
Schnider, A., & Landis, T. (1995). Memory loss. In J. Bogousslavsky &
L. Caplan (Eds.), Stroke syndromes (pp. 145150). Cambridge, MA:
extended to 11 years before the surgery. Subsequent neu-
Cambridge University Press. ropsychological studies of HM and other amnesic indivi-
Schacter, D. L. (1987). Implicit memory: History and current status. duals have further informed our current understanding of
Journal of Experimental Psychology: Learning, Memory and Cognition, both impaired and preserved memory function in amnesia
13, 501518.
(Corkin, 1984).
Sethi, H., Moore, A., Dervin, J., Clifton, A., & MacSweeney, J. E. (2000).
Hydrocephalus: Comparison of clipping and embolization in aneu-
rysm treatment. Journal of Neurosurgery. 92(6), 991994.
Neuropsychology of Anterograde
Amnesia
memory that do not require deliberate reference to a prior contribute to explicit memory; the first is recollection,
experience, often referred to as nondeclarative (or implic- the intentional, effortful process by which aspects of a past
it) memory, remain intact. episode are recovered. The second is familiarity, a sub-
Failure of declarative memory in amnesia can arise jective feeling that arises when information is processed
from a number of different etiologies. These include an- fluently and comes to mind easily. Whereas performance
oxia, herpes simplex encephalitis (HSE), anterior commu- on recall tasks depends on the ability to recollect contex-
nicating artery aneurysm (ACoA), WernickeKorsakoff tually appropriate information, performance on recogni-
syndrome (WKS), and stroke. The amnesia is a direct tion tasks can be supported by either recollection or
consequence of damage to the medial temporal lobes familiarity. Thus, the pattern of performance of patients
(i.e., HSE; anoxia), the midline diencephalon (i.e., WKS; with limited hippocampal lesions is thought to reflect
stroke), basal forebrain structures (i.e., ACoA), or some of impaired recollection, but preserved familiarity. Such a
the fiber tracts that link these regions. These amnesias pattern is consistent with findings from neuroimaging
are usually permanent. In contrast, in transient global am- and neurophysiological studies that suggest that the hip-
nesia (TGA) there is temporary dysfunction of memory- pocampus proper is critical for recollection, whereas
related brain structures including the hippocampal for- familiarity is supported by the perirhinal cortex. If the
mation and thalamus. Episodes of TGA typically last no damage is more extensive and extends beyond the hippo-
more than 24 h, after which the patients new-learning campus to include other medial temporal lobe structures
returns to normal, but a permanent amnesic gap remains such as the perirhinal cortex, then both recollection and
for the period of the attack ( Transient Global Amnesia). familiarity are affected, leading to striking impairments
The ability to remember newly encountered infor- on tests of recognition as well as recall.
mation depends on a number of stages, including the The degree of impairment in new semantic learning is
processing and representation of immediate experience also a function of the extent of the medial temporal lobe
(encoding), the transfer of that encoded information to lesion. Patients with injury limited to the hippocampus
long-term storage (consolidation), and its re-manifes- are able to acquire some new facts and concepts post-
tation in consciousness upon deliberate recall (retrieval) morbidly, although inefficiently, but patients with more
at a later time. Disruption of any one of these stages extensive medial lobe damage show minimal ability to do
could lead to anterograde amnesia. In patients with so (Verfaellie, 2000).
medial temporal or diencephalic lesions, encoding and In patients who suffered anoxia or a rupture of an
retrieval are thought to be relatively intact. Such aneurysm of the anterior communicating artery, frontal
patients perform normally on intelligence tests, and lobe impairments may be superimposed on the core amne-
on short-term memory tests, suggesting adequate sia ( Amnestic Syndromes). In such cases the antero-
encoding (Baddeley, 1995). Furthermore, impaired re- grade amnesia will be exacerbated by additional
trieval is unlikely to be the cause of their failed explicit impairments in encoding and retrieval. Executive functions
memory, because memories from many years ago can still such as planning, organizing, monitoring, and control of
be retrieved. Therefore, it is assumed that their impair- attention, all depend on the integrity of the frontal lobes.
ments reflect deficient consolidation. The medial tempo- Executive impairments will interfere with the ability to
ral lobes, through interactions with neocortical regions, mentally manipulate and organize information during de-
are thought to be critical for consolidation. They bind liberate encoding, and will also disrupt initiation and eval-
together into a coherent representation the different uation of memory search during effortful retrieval. The
aspects of an event that are neocortically represented latter can lead to unusually high levels of intrusions in
(Eichenbaum, 2006). recall, or false alarms in recognition, a phenomenon
Generally, the size of the causative brain lesion is known as enhanced susceptibility to false memory.
directly proportional to the density of the amnesia, but Despite such pervasive impairments in declarative
the specific location of the lesion will also impact on the memory, patients with anterograde amnesia show intact
nature of the memory impairment. For example, if the performance in a variety of forms of nondeclarative mem-
damage is limited to the hippocampal formation, perfor- ory. These include procedural learning (the acquisition of
mance on recall tasks is impaired, but performance on new skills or habits), eyeblink conditioning (learning to
recognition tasks can remain intact (Mayes, Holdstock, blink the eyes in response to a tone because of the repeat-
Isaac, Hunkin & Roberts, 2002). To account for these ed association of the tone with an air puff to the eye), and
findings, it has been suggested that two distinct processes repetition priming (improved accuracy or speed of
Anterograde Amnesia A 193
performance for stimuli to which an individual was re- and the choice among them should be informed by cog-
A
cently exposed) (Verfaellie & Keane, 2002). These forms of nitive factors such as premorbid abilities and skills as well
nondeclarative memory depend on neural circuits in the as post-morbid neuropsychological strengths and weak-
basal ganglia, cerebellum, or neocortex that remain spared nesses, including the severity of amnesia. Contributing
in amnesia (Squire, 1994). non-cognitive factors include premorbid lifestyle and
habits, and educational background. Contributing emo-
tional factors include insight and motivation, which are
Evaluation essential for any treatment choice, because the absence of
either will undermine rehabilitation efforts.
Anterograde amnesia refers to a severe and permanent Remediation of patients with severe amnesia relies
inability to learn new information in the presence of largely on those aspects of memory that are preserved,
otherwise normal intelligence, attention span, perception, such as procedural learning and priming. Techniques
reasoning, and language ability. The evaluation of antero- that capitalize on procedural learning use repetition to
grade amnesia must therefore, as a first step, include a drill skills and habits, ranging from essential activities
comprehensive neuropsychological work-up to determine of daily living to simple assembly tasks and cognitive
whether other areas of cognitive functioning are intact skills. Such skill learning is frequently involved when
and, if not, whether any deficits found contribute to the teaching a patient to use an external memory aid, such as
memory disorder. With regard to assessment of memory a memory notebook, calendar, diary, appointment book,
functioning itself, there are a variety of standardized tests or written reminders. The memory notebook is a preferred
available, and Lezak, Howieson, and Loring (2004) pro- compensatory instrument for amnesics because it is
vide a comprehensive review of the most commonly used divided into sections that are personally tailored to a
ones. Assessing performance on recall and recognition patients life (i.e., daily tasks, future plans, notes section,
tests is an essential component of the evaluation, because and so on). More sophisticated technology, in the form
their comparison can reveal the nature of the memory of computerized paging systems, electronic assistants,
processes that are affected. Both verbal and nonverbal alarms, and timers, is most useful for individuals who
memory should be examined, and memory should be had some proficiency in the use of such devices premor-
tested both shortly after learning and following a longer bidly. Learning to use such devices de novo may pose high
delay, to assess the rate of forgetting. Other factors of demands on working memory or episodic memory, which
diagnostic importance are a patients sensitivity to inter- is problematic for memory disordered patients. In such
ference and his or her ability to use organizational strate- instances, it is important to break the task down into
gies at encoding and retrieval. While a comprehensive small steps that can be practiced independently. Once
assessment of anterograde memory typically includes a the steps become automatized, they can then be gradually
variety of different tests, each developed for a specific integrated.
purpose, the use of a single standardized memory battery Other methods rely on preserved priming abilities
that evaluates all major aspects of new learning can (Verfaellie, 2000). One technique is the vanishing cues
provide a good overview of memory functioning. The technique, which has been used to teach amnesics
Wechsler Memory Scale-III (Wechsler, 1997) is probably computer-related vocabulary, business-related terms,
the most widely-used instrument for this purpose. In and novel concepts, through gradual reduction of cues
addition to indices of Immediate Memory and General that elicit correct answers. Another technique is errorless
(Delayed) Memory, it provides an index of Working learning. Error elimination requires explicit recollection
Memory, and, in patients with anterograde amnesia, a of the learning episode, and thus densely amnesic patients
split on the order of 20 points is to be expected between have great difficulty eliminating errors. Their perfor-
Working Memory and Immediate/General Memory. mance relies primarily on implicit memory, which typi-
cally leads to production of the strongest response. If that
response is incorrect, the error is likely to be further
Treatment strengthened across subsequent learning trials, thus inter-
fering with learning the correct response.
Rehabilitation interventions in amnesia aim at increasing For patients with milder memory impairments,
day-to-day functional adaptation and independence. strategies aimed at strengthening the impaired form of
A wide array of intervention techniques is available, memory are more appropriate. Such patients may
194 A Anterolateral System
brain. Because the nerve fibers making up the ALS cross Anticholinergic. Table 1 Anticholinergic medications
A
the midline within a few vertebral segments of where clinically used for the antimuscarinic effects
they enter the cord, lesions affecting the ALS will result
Medications for Oxybutynin (Ditropan),
in contralateral deficits. neurogenic bladder tolterodine (Detrol), trospium
including urge (Sanctura), solifenacin
incontinence, for (Vesicare), darifenacin
Cross References overactive bladder (Enablex)
Anticholinergic Benztropine (Cogentin),
Medial Lemniscus (Posterior Columns)
antiparkinsons medication trihexyphenidyl (Artane)
Antivertigo medication Meclizine (Antivert),
scopolamine (Transderm
References and Readings Scop)
Gastrointestinal Diphenoxylate/atropine
Mendoza, J. E., & Foundas, A. L. (2008). The somatosensory systems. In
antispasmodics (Lomotil), belladonna
J. E. Mendoza & A. L. Foundas (Eds.), Clinical neuroanatomy A
neurobehavioral approach (pp. 2347). New York: Springer.
medications (Donnatal)
Medications for Tiotropium (Spiriva),
bronchospasm ipratropium (Atrovent)
Anti-Anxiety Drugs
Anticholinergic. Table 2 Anticholinergic medications not
Anxiolytics primarily targeting the cholinergic receptors
Sedating Diphenhydramine (Benadryl),
antihistamines hydroxyzine (Vistaril), cyproheptadine
Anti-Anxiety Medications (Periactin)
Tricyclic Amoxaprine (Asendin), amytriptyline
Anxiolytics antidepressants (Elavil), desipramine (Norpramin),
imipramine (Tofranil), nortriptyline
(Pamelor)
Certain Clozapine (Clozeril), olanzapine
Anticholinergic antipsychotics (Zyprexia), risperidone (Risperdal)
Muscle relaxants Dantrolene (Dantrium), cyclobenzaprine
M ARY PAT M URPHY
(Flexeril)
MSN, CRRN
Paoli, PA, USA
blockers. The receptor subtypes affect the brain, salivary
glands, smooth muscle, and ciliary muscles of the eye.
Synonyms Categories of medications are clinically used for the
antimuscarinic effects and include medications for uri-
Anticholinergic medications
nary spasmodics and overactive bladder, anticholinergic
antiparkinsons agents, antivertigo medications, gastro-
Definition intestinal antispasmodics, mydriatic medications, and
medications for bronchospasm. Another group of med-
Anticholinergic agents alter the balance of neurotransmitters ications not primarily targeting the cholinergic receptors
in the central and peripheral nervous system inhibiting include sedating antihistamines, tricyclic antidepressants,
parasympathetic nerve impulses. Specifically, the agents muscle relaxants, some antipsychotics, antiarrythmics,
diminish acetylcholine and allow for the increase of and antiemetics. Neuropsychologists should be aware
dopamine. Anticholinergic medications are divided into of the medications their patients are taking and the
three categories based on their specific receptor targets in potential impact on neuropsychological test results. It
the nervous system and in other sites in the body: is necessary to differentiate between medication side-
antimuscarinic, ganglionic blockers, and neuromuscular effects and true consequences or neurologic disorder.
196 A Anticholinergic Medications
Side Effects
Anticholinesterase Inhibitors
Anticholinergic side effects can be caused by a wide range
of medications. Anticholinergic medications have periph- J OA NN T. T SCHANZ 1, K ATHERINE T REIBER 1,2
1
eral and central side effects including dry mouth, blurred Utah State University
vision, urinary retention or difficulty initiating voiding, Logan, UT, USA
2
constipation or bowel obstruction, decreased sweating, University of Massachusetts Medical School
increased heart rate, ataxia, increased body temperature, Worcester, MA, USA
agitation, confusion, delirium, memory impairment,
decreased attention, dizziness, and drowsiness.
Certain populations are at greater risk for adverse events Synonyms
related to anticholinergic medications. They include older
adults who already experience a decrease in acetylcholine Acetylcholinesterase inhibitors; ACHE inhibitors; AchEIs;
production; men with benign prostatic hypertrophy, Cholinesterase inhibitors
patients with glaucoma, and individuals with dementia
who are already taking cholinesterase inhibitors.
The elderly and patients with brain injury are often Definition
prescribed medications with anticholinergic properties to
address medical issues for bladder management, increased Anticholinesterase inhibitors are a class of substances that
muscle tone, and behavior (atypical antipsychotics). affect the cholinergic neurotransmitter system and are
There may be a cumulative effect of taking multiple often used for clinical purposes in the treatment of mem-
medications which act on the cholinergic system. ory disorders such as Alzheimers disease (AD). Nonclini-
Anticholinergic side effects in older adults include an cal uses include agricultural applications such as pesticides
increase in delirium, diminished ADLs, and decrease in and military applications such as the development of neu-
cognition (Fick et al., 2003; Han et al., 2001). rotoxins. Acetylcholine is normally released by the presyn-
patic neuron and activates receptors on the postsynaptic
cell. Acetylcholinesterase is the primary enzyme that breaks
Cross References down acetylcholine in the synaptic cleft. Cholinesterase
inhibitors block the activity of this enzyme, allowing the
Acetylcholine neurotransmitter substance to remain in the synaptic cleft
Dopamine longer to stimulate postsynaptic receptors.
Neurotransmitters
Current Knowledge
References and Readings
Clinical Indications
Fick, D. M., Cooper, J. W., Wade, W. E., Waller, J. L., Maclean, J. R., &
Beers, M. H. (2003). Updating the Beers criteria for potentially Cholinesterase inhibitors are often used in the treatment
inappropriate medication use in older adults: results of a US con-
of memory and other cognitive disorders. In AD, degener-
sensus panel of experts. Archives of Internal Medicine, 163(22),
27162724.
ation of brain cholinergic neurons has been associated
Han, L., McCusker, J., Cole, M., Abrahamowicz, M., Primeau, F., & Elie, with progressive cognitive deterioration. Because cholines-
M. (2001). Use of medications with anticholinergic effect predicts terase inhibitors do not reverse or stop the progressive
Anticoagulation A 197
circumstances when clinical and laboratory monitoring is monotherapy is the goal for the treatment of epilepsy,
feasible and when the patient does not have risk of falls, choosing medications targeting seizure control with
injuries, or other contraindications. fewest side effects. Monotherapy also makes it easier to
monitor side effects. Usually, if one drug fails, another
medication is trialed. If the initial AED fails, the physician
Cross References typically will wean this medication and try another first-
line drug. If monotherapy fails, polytherapy may be tried.
Atherosclerosis The physician will maximize the first-line dose and then
Central Venous Thrombosis add a second-line medication. General monitoring for
Cerebral Embolism AEDs includes the frequency and severity of seizures,
Heparin adverse events and side effects, and monitoring of plasma.
Thrombosis The chart below identifies FDA indications for commonly
Venous Thrombosis used AEDs.
Warfarin
elevating the seizure threshold and reducing the spread of hypontension, bradycardia, dysrhythmias, and cardiac
A
seizure activity in the brain. Phenobarbital may also in- changes, as well as venous irritation and
hibit calcium channels. thrombophlebitis.
Other adverse events/side effects include gingival
hyperplasia, hirstism, rash, hepatitis, megaloblastic
First-Line Medications anemia, thrombocytopenia, StevensJohnson syndrome,
systemic lupus Erythematosus, and folic acid deficiency.
Valproate (Depakote)
Drug interactions
Indication Drug interactions are many and include (but are not
Labeled indications include control of epilepsy (seizures limited to) chloramphenicol, dexamethasone, doxycycline,
disorders). As an AED, it can be used as monotherapy and furosemide, haloperidol, meperidine, methadone, oral
adjunctive treatment of tonicclonic, partial complex contraceptives, theophylline, and warfarin. Non-AEDs
seizures, and simple and complex absence seizures. It that effect phenytoin levels include alcohol, antacids, folic
can be used as an adjunctive treatment in patients who acid, rifampin, tube feedings, alcohol, cimetidine,
have multiple types of seizures. fluoxetine, imipramine, INH, omeprazole, propoxyphene,
sulfonamides, and trazadone.
Contraindications
The medication should be prescribed cautiously for Carbamazepine (Tegretol)
individuals with liver disease and urea cycle disorders
and for pregnant women. Indication
Carbamazepine is indicated as a first-line drug for use as
Adverse events/side effects an anticonvulsant for partial seizures, generalized tonic
Weight gain, thrombocytopenia, and elevated liver clonic, and mixed seizures, but not absence seizures. It is
enzymes may be dose related. When initially starting generally nonsedating within therapeutic range. It is also
the medication, patients may complain of nausea and indicated in the treatment of trigeminal neuralgia.
diarrhea. Hyperammonemia has been reported and may
be present despite normal liver function testing. In the Adverse events/side effects
elderly, there is a possible increase in somnolence. Adverse events associated with carbamazepine include
aplastic anemia and agranulocytosis. Pretreatment hema-
Drug interactions tology testing should be completed to obtain a baseline.
Medications that may increase valproate levels include The patient should be monitored and treatment should be
felbamate, rifampin, and chlorpromazine; medications that discontinued with hematology changes. StevensJohnson
valproate may affect include carbamazepine, amitriptyline, syndrome (an exfoliating dermatitis) has been reported.
nortriptyline, clonazepam, ethosuximide, lamotrigine, Carbamazepine has mild anticholinergic properties, so
phenobarbital, phenytoin, tolbutamide, and lorazepam. patients with intraocular eye pressure should be moni-
tored. Carbamazepine should not be used in pregnant
Phenytoin (Dilantin) women. Patients should be cautioned against drinking
alcohol.
Indication In the beginning of treatment, patients report side
Phenytoin is the oldest and one of the most effective effects including dizziness, drowsiness, nausea, and
medications in the treatment of a wide range of seizure vomiting.
types. The labeled use is for tonicclonic and partial
complex seizures. It is often used as a first-line drug Medications that affect carbamazepine plasma
choice for monotherapy. The usual dose is 300 to 400 levels
mg/day. An extended-release capsule allows for onetime a Drugs that increase plasma levels include cimetidine,
day dosing. The therapeutic range is 1020. danazol, macrolides, erythromycin, troleandomycin,
fluoxitine, nefazoned, loratadine, terfenadine, INH,
Adverse events/side effects propoxyphene, verapamil, grapedfruit juice, protease
Phenytoin can be administered intravenously. As a result, inhibitors, and valproate. Medications that decrease
specific adverse events/side effects can include carbamazepine plasma levels include cisplatin, felbamate,
200 A Anticonvulsants
Second-Line Medications
Barbiturates (Second Line)
Gabapentin (Neurontin)
Phenobarbital
Gabapentin is effective as an adjunctive therapy in the treat-
ment of partial seizures with and without generalization.
Indication
Labeled indications include control of epilepsy (seizures
disorders) and as a sedative/hypnotic medication for
Adverse events/side effects short-term treatment of insomnia. As an AED, it can be
Include dizziness, ataxia, weight gain, gi upset, somno-
used as monotherapy in the treatment of generalized
lence, and other symptoms of CNS depression.
(tonicclonic), simple, or partial complex seizures; for
myoclonic epilepsy; and for neonatal and febrile seizures
Drug interactions in children. It has also been prescribed for eclamptic
Antacids decrease their bioavailability. seizures during pregnancy.
Antidepressants A 201
Benzodiazepines
Definition
This class of medication is not typically used as first-line
medications. As a class, they can produce CNS depression Antidepressants are a class of medications that are
and behavioral changes. Other adverse reactions include used primarily in the treatment of clinically severe
202 A Antidepressants
mood or anxiety disorders. The majority of effective orthostatic hypotension, and weight gain. A concern with
antidepressants currently in use enhance neurotrans- this medication is the narrow therapeutic index, which
mission of serotonin and/or norepinephrine. General- raises the risk of death with overdose. SSRIs do not carry
ly, this is achieved by blocking the reuptake of the the same health concerns as MAOIs or TCAs. Common
neurotransmitter substance(s), inhibiting the enzymes side effects of SSRIs include nausea and sexual dysfunc-
responsible for its metabolism, or directly stimulating tion. A topic of much controversy is a possible increase in
the postsynaptic receptors (Iversen, Iversen, Bloom, & risk of suicidal ideation and behavior (see Current Knowl-
Roth, 2009). Several antidepressants are also used in edge). Side effects reported with mixed SSRINRIs (e.g.,
treating generalized anxiety disorder, panic disorder, and velaxafine), include headache, dry mouth, sedation, hyper-
obsessivecompulsive disorder (Bourin & Lambert, tension, and constipation (Iversen et al., 2009).
2002). Other conditions for which antidepressants have
demonstrated efficacy include eating disorders (Powers &
Bruty, 2009), neuropathic pain (OConnor & Dworkin, Current Knowledge
2009), stress incontinence, nocturnal eneuresis, ejaculatory
disorders (Michel, Ruhe, de Groot, Castro, & Oelke, 2006), Approximately 6070% of persons treated with antide-
migraine headaches, fibromyalgia (Stone, Viera, & Parman, pressants show a positive response. The lack of response in
2003), attention-deficit/hyperactivity disorder (Chung, 3040% of depressed individuals (at least to SSRIs) may
Suzuki, & McGough, 2002), smoking, insomnia, and pos- be due in part to the effects of genes. Variations in the
sibly pathological gambling (Grant & Grosz, 2004). serotonin transporter gene (often referred to as
There are several classes of antidepressant medica- 5-HTTLPR) modify the response of depressed persons
tions. Tricyclic antidepressants (TCAs) block the reuptake to SSRIs. Compared to those with a long (L) allele of
of monoaminergic neurotransmitters and monoamine this gene, persons with a short allele exhibit poorer
oxidase inhibitors (MAOIs) inhibit their metabolism. response to SSRI treatment. Variations in 5-HTTLPR
Other compounds are more selective in blocking the may also influence the experience of side effects
reuptake of specific neurotransmitters (selective serotonin (Horstmann & Binder, 2009).
reuptake inhibitors or SSRIs and noradrenergic reuptake The response rate to placebo in clinical trials of
inhibitors or NRIs). Compounds with dual serotonergic antidepressants is relatively high, ranging from 30 to
and noradrenergic actions have also been developed 50%. The placebo response is greater among individuals
(Iversen et al., 2009). with mild depressive symptoms, and recent meta-analyses
Regardless of the type of antidepressant, the com- of clinical trials of second-generation antidepressants
pounds are similar in their effectiveness and the time indicate significant treatment effects only among those
course of their effects. The lag between the initiation of with severe symptoms (Fournier et al., 2010; Kirsch
antidepressant treatment and the alleviation of symptoms et al., 2008).
generally takes 26 weeks for the maximal response. The Significant concerns of an increased risk of suicidal
delay in treatment response suggests that the therapeutic ideation and behavior (suicidality) have arisen over
effects may result from downstream events that reflect the use of new-generation antidepressants. The US Food
the brains adaptation to treatment (Iversen et al., 2009). and Drug Administration (FDA) has released several
Alternative treatments with a shorter treatment lag are advisories that antidepressant use may increase the risk
under active investigation (see Future Directions). of suicidality among children, adolescents, and young
Antidepressant medications differ in their profile of adults (http://www.fda.gov/NewsEvents/Newsroom/Press
side effects. First-generation MAOIs, which inhibit the Announcements/2007/ucm108905.htm). A meta-analysis
activity of both MAO-A and MAO-B, were known for of clinical trial data with SSRIs has confirmed a moderate
potentially serious side effects if patients also consumed increase in risk of suicidality among pediatric patients
foods containing tyramine (fermented products such as (Hammad, Laughren, & Racoosin, 2006). These observa-
wine or cheese). Potential effects included headache, tions are in contrast to epidemiological data that indicate
hypertension, cerebral hemorrhage, and death. Newer- reduced rates of completed suicides. Some have hypothes
generation MAOIs that act more selectively on MAO-A ized that the higher risk of suicidality with antidepressant
do not require the dietary restriction from tyramine- treatment likely occurs in a subset of high-risk patients
containing foods. Common side effects associated with with agitated major depression or unrecognized bipolar
TCAs include dry mouth, urinary retention, sedation, disorder (Rihmer & Akiskal, 2006).
Antihistamines A 203
Depression
Selective Serotonin Reuptake Inhibitors (SSRIs)
Serotonin Antihistamines
S TEPHANIE A. KOLAKOWSKY-H AYNER
Santa Clara Valley Medical Center,
References and Readings Rehabilitation Research Center
San Jose, CA, USA
Bourin, M., & Lambert, O. (2002). Pharmacotherapy of anxious disorders.
Human Psychopharmacology: Clinical Experimental, 17, 383400.
Chung, B., Suzuki, A. R., & McGough, J. J. (2002). New drugs for
treatment of attention-deficit/hyperactivity disorder. Expert Opinion Synonyms
Emerging Drugs, 7, 269276.
FDA Press Release. Extracted from http://www.fda.gov/NewsEvents/ Histamine antagonist; Inverse histamine agonists
Newsroom/PressAnnouncements/2007/ucm108905.htm on 1/21/2010
Fournier, J. C., DeRubeis, R. J., Hollon, S. D., Dimidjian, S., Amsterdam,
J. D., Shelton, R. C., et al. (2010). Antidepressant drug effects and
depression severity: A patient-level meta-analysis. JAMA, 303, 4753. Definition
Grant, J. E., & Grosz, R. (2004). Pharmacotherapy outcome in older
pathological gamblers: A preliminary investigation. Journal of Antihistamines are commonly used to treat allergies; H1
Geriatric Psychiatry and Neurology, 17, 912.
Hammad, T. A., Laughren, T., & Racoosin, J. (2006). Suicidality in
receptor inverse agonists typically reduce swelling and
pediatric patients treated with antidepressant drugs. Archives of vasodilation within the nasal area. H1 receptor antagonists
General Psychiatry, 63, 332339. include cetirizine, diphenhydramine also known as
204 A Antihypertensives
rapid control of hypertension and is recommended for Beta-blockers should not be discontinued abruptly but
A
patients with stages II and III hypertension. Triple-drug should be tapered over 14 days to prevent withdrawal
therapy may be required if the blood pressure control is which includes unstable angina, myocardial infarction,
not achieved. Some patients have resistant hypertension. and death.
A fourth line of medications may be required.
ACE Inhibitors
This class of antihypertensives inhibits ACE which
Classes of Antihypertensives converts angiotensin I to II a potent vasoconstrictor.
This is a first-line therapy for patients with diabetes and
Diuretics proteinuria. Mediations include Lotensin, Capoten,
Diuretics decrease blood pressure by causing diuresis, Vasotec, Monopril, Zestril, Univasc, Accupril, Altace, and
which results in decreased blood volume, cardiac output, Mavik.
and stroke volume. They fall into three categories: Side effects include cough, hypotension, hyperkalemia,
thiazides, loop diuretics, and potassium-sparing diuretics. rash, loss of taste, leukopenia, and neutropenia. They are
Thiazides onset of action occurs within 23 h. Their half- contraindicated in pregnancy and for patients with
life is 812 h allowing for once daily dosing. Trade names bilateral real artery stenosis.
include Hygroton, Hydrodiuril, Lozol, and Zaroxolyn.
Loop diuretics act in the loop of Henle in the kidney Calcium Channel Blockers
and are less effective in the long term. Their duration is Calcium channel blockers relax the cardiac and smooth
6 h. These agents are indicated with CHF or nephrotic muscle by blocking calcium channels that allow calcium
syndrome. Bumex, Edecrin, Lasix, and Demadex are trade into the cells. The result is vasodilation. They also
names. decrease the heart rate and slow cardiac conduction.
Potassium-sparing agents cause minimal diuresis Medications include Calan, Cardizem, Norvasc, Plendil,
and are relatively ineffective in lowering the blood pres- Procardia Cardene, Sular, and DynaCirc.
sure. The medications correct thiazide-induced potassium
and magnesium losses. Medication trade names include Side Effects
Midamor, Aldactone, and Dyrenium. Side effects include GI upset, edema, and hypotension.
Rare side effects include bradycardia, CHF, and AV
Adverse Events block. Other adverse effects include dizziness, headache,
Most complications occur related to dose and duration of shortness of breath, gingival hyperplasia, and edema.
use. Hypokalemia is a side effect, but can be managed
with potassium chloride or use of potassium-sparing Contraindications
agents. Calcium channel blockers should not be prescribed for
Acute gouty arthritis, muscle cramps, development of individuals with second- and third-degree heart block or
diabetes, nocturia or incontinence, and sun sensitivity left ventricular dysfunction.
have been noted as clinical side effects.
Other Classes of Antihypertensives
Beta-Blocking Agents Peripheral alpha1- receptors (Cardura, Minipress, and
Beta1-receptors are located in the heart and kidneys and Hytrin), central alpha2 (Clonidine, Aldomet, Tenex,
regulate heart rate and cardiac contractility. Beta2- and Wytension), direct vasodilators (Apresoline and
receptors regulate bronchodilation and vasodilation. Loniten), and adrenergic antagonists (Serpasil, Ismelin
Beta-blockers decrease blood pressure by blocking the and Hylorel) are the remaining categories of antihyper-
beta-receptors. Some beta-blockers are cardioselective tensives. They are mainly used as second- and third-line
that is, they do not block the beta2-receptors, therefore medications.
do not cause bronchoconstriction. These medications
include Lopressor, Kerlone, Tenormin, Sectral and Zebeta,
Corgard, Inderal, and Cartrol. Cross References
Side Effects Psychopharmacology
The most common side effects of beta-blockers are Stroke
fatigue, dizziness, bronchospasm, nausea, and vomiting. Transient Ischemic Attack (TIA)
206 A Antiplatelet Therapy
References and Readings attack and stroke in certain situations, for primary and
secondary prevention. This favorable effect is based on
August, P. (2003). Initial treatment of hypertension. New England Journal the ability of these agents to inhibit the chemicals that
of Medicine, 348, 610617. cause platelets to clump together initiating blood clot
Cranwell-Bruce, L. (2008). Antihypertensives. MEDSURG Nursing, 17(5),
formation.
337341.
Ernst, M., & Moser M. (2009). Use of diuretics in patients with
Aspirin is the prototypical antiplatelet agent. Other
hypertension. New England Journal of Medicine, 361, 21532164. currently available antiplatelet agents include ticlopidine
Houston, M. C., Pulliam Meador, B., & Moore Schipani, L. (2000). (Ticlid), clopidogrel (Plavix), and dipyridamole
Handbook of antihypertensive therapy (10th ed.). Philadelphia: (Persantine).
Hanley & Belfus.
Staessen, J., & Birkenhager, W. (2005). Evidence that new antihypertensives
are superior to older drugs. Lancet, 366(9489), 869871. Cross References
Atherosclerosis
Antiplatelet Therapy Cerebrovascular Disease
Coronary Disease
E LLIOT J. R OTH Ischemic Stroke
Northwestern University Myocardial Infarction
Chicago, IL, USA Peripheral Vascular Disease
Stent
Thrombosis
Definition
References and Readings
Antiplatelet therapy uses specific pharmacological agents
(antiplatelet agents) to inhibit the ability of platelets to
Tran, H., & Anand, S. S. (2004). Oral antiplatelet therapy in cerebrovas-
clump together to form blood clots, or thromboses, pri- cular disease, coronary artery disease, and peripheral arterial disease.
marily in arteries. It is commonly used in people with JAMA, 292, 18671874.
atherosclerosis (narrowing of the arteries).
groups of antipsychotics; Neuroleptics; Standard the serotonin, dopamine, and GABA neurotransmitter
A
antipsychotics systems. This multiple pathway approach may help with
the individualization and selection of the best agent based
on the individuals response.
Definition
cognition, decrease risk of tardive dyskinesias, and other Savitz, J., van der Merwe, L., Stein, D., Solms, M., & Ramesar, R. (2008).
Neuropsychological task performance in bipolar spectrum illness:
neurological changes resulting from these agents.
genetics, alcohol abuse, medication and childhood trauma. Bipolar
Newer atypical/novel antipsychotic agents have in- Disorders, 10(4), 479494.
cluded Risperidone, Zyprexa, Seroquel, Geodon, Abilify, Voruganti, L., & Awad, A. (2004). Neuroleptic dysphoria: Towards a new
and, most recently, Saphris. However, with these newer synthesis neuroleptic dysphoria. Psychopharmacology, 171(2),
atypical antipsychotics, other concerning side effects 121132.
Wozniak, J., Block, E., White, T., Jensen, J., & Schulz, S. (2008). Clinical
have been exposed including metabolic changes leading
and neurocognitive course in early-onset psychosis: a longitudinal
to alterations in carbohydrate and lipid metabolism, pos- study of adolescents with schizophrenia-spectrum disorders. Early
sible diabetes, and excessive weight gain. All of these Intervention in Psychiatry, 2(3), 169177.
newer agents require routine monitoring of weight,
blood sugar, and lipid profile studies to control the
potential adverse effects while achieving improvement in
both positive/negative symptoms of psychosis. Treatment
duration with these agents is individually maximized Antithrombotic Therapy
based on response to reduction in positive symptoms of
chronic thought disorders/psychosis. Shorter treatment Anticoagulation
durations may be possible in acute onset of delirium,
acute psychoses, or brief reactive psychosis.
Definition
Cross References
Anxiety is an unpleasant state characterized by affective,
Neuroleptics cognitive, and physiological elements such as fear, worry,
Psychopharmacology apprehension, and tension.
Psychotic Disorder Anxiety is similar to the emotion of fear, although the
function of chronic anxiety is often to avoid or mask true
fear through mechanisms of anxiety such as worry and
References and Readings anticipation of negative future outcomes. The physiologi-
cal manifestations of anxiety include increased blood
Ahmed, I. I., & Fuiji, D. (1998). Posttraumatic Psychosis. Seminars in
pressure, increased breathing rate (often shallow),
Clinical Neuropsychology, 3(1), 2333.
McDonald, B. C., Flashman, L. A., & Saykin, A. J. (2002). Executive increased heart rate, other cardiac symptoms (e.g., pain,
dysfunction following traumatic brain injury: neural substrates and skipped beats), gastrointestinal distress including nau-
treatment strategies. NeuroRehabilitation, 17(4), 333344. sea, stomach aches, increased motility of the gut, and
Meredith, C., Jaffe, C., Ang-Lee, K., & Saxon, A. (2005). Implications of diarrhea, generalized bodily distress such as fatigue and
chronic methamphetamine use: A literature review. Harvard Review
pain. Cognitively, anxiety is frequently characterized by an
of Psychiatry, 13(3), 141154.
Preston, J., ONeal, J. H., & Talaga, M. C. (2006). Child and adolescent overestimation of the probability of a negative future
clinical psychopharmacology made simple. Oakland, CA, US: New outcome and an exaggeration of the consequences of the
Harbinger Publications. negative outcome. For example, an anxious person may
Anxiolytics A 209
believe that it is likely that they will fail a test with habituation. Exposure can be in vivo or imaginal, and
A
catastrophic consequences. therapy frequently uses cognitive techniques to modify
Anxiety often occurs in response to external stressors. anxiety-generating cognitions. Anxiolytic medication is
It can be a normal reaction to stress, in which case anxiety also often prescribed.
can help coping behavior by focusing attention, mobiliz-
ing energy, and increasing goal-directed behavior.
However, anxiety can also be a reaction to internal (phys- Cross References
iological) cues or a generalized and pervasive mood with-
out identifiable precipitants. When anxiety is an excessive Anxiolytics
reaction, or present in the absence of any true challenges Beck Anxiety Inventory
or dangers, it is considered pathological. Individuals with
pathological levels of anxiety are typically high in trait
anxiety, which is a stable and enduring tendency to re- References and Readings
spond with anxiety to a wide variety of situations. Indi-
viduals high in trait anxiety are often also high in Allen, L. B., McHugh, R. K., & Barlow, D. H. (2008). Emotional disorders:
neuroticism. A unified protocol. In D. H. Barlow (Ed.), Clinical handbook of
psychological disorders: A step-by-step treatment manual (4th ed.,
pp. 216249). New York, NY: Guilford Press.
American Psychiatric Association (2000). Diagnostic and statistical
Historical Background manual of mental disorders (4th ed.), Text Revision. Washington,
DC: American Psychiatric Association.
Anxiety is basic to human experience and has been docu-
mented and treated since the beginning of recorded his-
tory. The relation between anxiety and health complaints
has been recognized since the seventeenth century, although Anxiolytics
psychiatric nosology did not become well developed
until the last century. A number of anxiety disorders S TEPHANIE A. KOLAKOWSKY-H AYNER
have been delineated in contemporary psychiatric writ- Santa Clara Valley Medical Center,
ings and are described in the most recent edition of the Rehabilitation Research Center
Diagnostic and Statistical Manual of Mental Disorders San Jose, CA, USA
published by the American Psychiatric Association.
Synonyms
Current Knowledge
Anti-anxiety drugs; Anti-anxiety medications
Although anxiety can be learned, it is thought to have a
biological basis in the amygdala and hippocampus. When
individuals are exposed to potentially dangerous or harm- Definition
ful stimuli, brain imaging often shows increased activity
in the amygdala accompanied by participant reports of Anxiolytics are prescription drugs used to reduce the
increased anxiety. Excessive anxiety can also compromise severity and extent of symptoms due to anxiety-related
performance on neuropsychological tests, especially by disorders. Often known as benzodiazepines, these drugs
interfering with attention and cognitive efficiency. are used to treat generalized anxiety disorder, panic attacks,
When suspected, the level of anxiety should be phobias, and other ongoing issues of excessive fear and
assessed. Anxiety is often measured using the Beck Anxi- dread. Medical illness often associated with high levels of
ety Inventory or Hamilton Anxiety Scale. They do not anxiety also includes brain injury, heart disease, and COPD.
diagnose anxiety disorders, but give a dimensional mea- There are six approved anxiolytics in the USA today includ-
sure of anxiety. ing the popular Diazepam (Valium), Lorazepam (Ativan),
Effective treatment of anxiety almost always involves and Alprazolam (Xanax). Anxiolytics are designed to im-
exposure to the feared stimulus. Treatments are based pact neurotransmitters in the amygdala by increasing
on the principles of classical conditioning, and the goal gamma-aminobutyric acid (GABA), an inhibitory neuro-
is to extinguish the fear response through exposure and transmitter that diminishes the fear response.
210 A Apallesthesia
measures (see below). Combining data from multiple it does appear as a nonspecific symptom for several other
A
studies, these authors report point prevalence rates of disorders. The merits of including apathy as a stand-alone
60.3% in Alzheimers disease, 46.7% in TBI, 60.3% in disorder in the upcoming DSM-V revision are currently
persons with focal frontal lesions, 33.8% in vascular de- being debated.
mentia, 34.7% poststroke, 22.2% in dementia with Lewy Prognostically, there is evidence to suggest that apathy
bodies, 29.8% in HIV, 20.5% in multiple sclerosis, and may be associated with more severe impairment and
53.3% in patients with major depression. Studies examin- negative outcomes. For example, a longitudinal study
ing apathy in other neurological conditions have found examining apathy in persons with Alzheimers disease
prevalence rates of 41% in CADASIL (Reyes et al., 2009), found that apathy at the baseline was associated with
90% in frontotemporal dementia, 91% in progressive faster cognitive and functional decline at follow-up
supranuclear palsy, 59% in Huntingtons disease, and (Starkstein et al., 2006) There is also some evidence that
33% in Parkinsons disease (Levy et al., 1998). Apathy is apathy may precede the development of Alzheimers dis-
also one of the most commonly observed neuropsychia- ease. One longitudinal study of patients with MCI found
tric symptoms in MCI (Apostolova and Cummings, that those patients who converted to Alzheimers disease
2008). had higher rates of apathetic symptomatology (91.7%)
While the above-described findings related to clinic- than those patients who did not convert (26.9%) (Robert
based samples, apathy has also been reported in a et al., 2006). Apathy has also been found to be significant-
community-based sample of older adults with prevalence ly associated with lower cognitive functioning and more
rates of 1.4% in cognitively normal elderly, 3.1% in mild severe motor symptoms in persons with Parkinsons dis-
cognitive syndrome, and 17.3% in dementia (Onyike ease (Pedersen et al., 2009). Apathetic symptomatology
et al., 2007). Apathy also appears to be quite common in has also been found to be negatively associated with
nursing home settings, with one study reporting a preva- functional improvement in rehabilitation settings after
lence rate of 84.1% (Wood et al., 2000). Apathy may also strokes (Hama et al., 2007) and increased risk for mortal-
appear as an adverse effect of some prescription drugs, ity in nursing home residents with dementia (van Dijk
including selective serotonin reuptake inhibitors (SSRIs) et al., 1994).
(Hoehn-Saric et al., 1990). Studies have also found that apathy is associated with
decreased performance of activities of daily living (ADLs)
in persons with stroke (Mayo et al., 2009; Starkstein et al.,
Natural History, Prognostic Factors, 1993), vascular dementia (Zawacki et al., 2002), fronto-
Outcomes temporal dementia (Kipps et al., 2009), dementia with
Lewy bodies (Ricci et al., 2009), and major depression
The word apathy comes from the Greek word apatheia, (Steffens et al., 1999). Alzheimers disease patients with
meaning, an absence of feeling. The Stoic philosophers apathy are more likely to be impaired on basic activities of
used this term to connote the total freedom from emo- daily living (dressing, bathing, toileting, transferring,
tions and passions which were thought to compromise walking, and eating) than nonapathetic Alzheimers dis-
rationality and the desired state of mental tranquility. ease patients, even when matched on degree of cognitive
However, over the centuries, the term apathy came to impairment (Albert et al., 1996; Stout et al., 2003). In
refer to a lack of reactivity and became viewed as patho- addition, apathy has been found to account for 27% of
logical rather than desirable. the variance in instrumental activities of daily living
While apathy can be observed as a symptom asso- scores (medication management, shopping, finances) in
ciated with a variety of psychiatric, neurological, and patients with Alzheimers disease (Boyle et al., 2003).
medical conditions, some authors have argued that apa- Finally, apathy does not only impact the patient. Due
thy, in some circumstances, may represent a neuropsy- to impairments in motivation, individuals with apathy
chiatric syndrome as well. Marin (1991) defined an apathy can require more support and management, which can,
syndrome as a loss of motivation which could not be in turn, result in increased caregiver burden and stress.
attributed to emotional distress, intellectual impairment, The caregivers of patients with Alzheimers disease-related
or a diminished level of consciousness. In contrast, apa- apathy have been shown to report significantly elevated
thy, as a symptom, was defined as a loss of motivation due levels of distress and perceived burden compared to those
to a disturbance of intellect, emotion, or level of con- who are caring for less apathetic patients with a similar
sciousness (Marin, 1991). Apathy is not considered an level of cognitive impairment (Kaufer et al., 1998). Care-
independent syndrome in the current DSM-IV, though giver distress secondary to neuropsychiatric symptoms,
212 A Apathy
including apathy, has been implicated in the eventual mood states, dysphoric versus emotionally indifferent, is
institutionalization of many patients with Alzheimers the most useful characteristic in making a differential
disease (Scott et al., 1997; Steele et al., 1990). diagnosis between apathy and depression. Apathy can be
thought of as a syndrome of primary motivational loss
and diminished emotional reactivity, while depression
reflects a syndrome of mood disturbance.
Neuropsychology and Psychology
The mechanisms of apathy are not fully understood,
of Apathy
though most theories suggest it involves disruption of the
frontal-subcortical neural circuit. This circuit begins with
In clinical practice and research, apathy is often mistaken
the anterior cingulate cortex, and continues to the ventral
for depression, though it is a distinct syndrome that can
striatum, the globus pallidus, and the thalamus, before
be distinguished from depression (Levy et al., 1998;
looping back to the anterior cingulate cortex. It has been
Marin, 1991; Starkstein et al., 2001). The syndromes of
hypothesized that neuropathological changes and altera-
depression and apathy share some symptoms (Table 1)
tions in regional chemistry, especially acetylcholine, do-
and may co-occur in that same individual, making diag-
pamine, and serotonin, in this circuit, are responsible for
nosis a challenging exercise. (Damsio and Van Hosen,
the clinical manifestation of apathy (David et al., 2008;
1983). For example, an apathetic demented patient who
Franceschi et al., 2005; Landes et al., 2001; Mega &
presents with fatigue, sleep disturbance, poor appetite and
Cummings, 1994). Apathy with impaired motivation
weight loss, poor concentration, and anhedonia, may be
and indifference has most strongly been associated with
diagnosed with a major depressive disorder even in the
damage to anterior cingulate cortex (ACC) (Damsio &
absence of dysphoria (Ishii et al., 2009). A number of
Van Hosen, 1983). In the most extreme cases, damage to
studies have found apathy to be correlated with high
the ACC results in akinetic mutism, and a complete loss of
scores on various depression measures (Rabkin et al.,
initiation and motivation. Single photon emission com-
2000; Ready et al., 2003; Starkstein et al., 2006). However,
puted tomography (SPECT) studies of patients with
this correlation may be due to the fact that many clinical
Alzheimers disease found that apathy was strongly and
measures of depression include questions assessing the
inversely correlated with right anterior cingulate activity
symptoms of both apathy and depression, which may
(Benoit et al., 1999) or with a bilateral reduction in
lead to misdiagnosis.
cingulate activity (Migneco et al., 2001).
Apathy may be distinguished from depression by the
Frontal regions have also been implicated in the man-
absence of dysphoric mood symptoms such as sadness,
ifestation of apathy. Neuroimaging studies have found
guilt, hopelessness, and helplessness. The difference in
apathy in AD patients to be correlated with hypoperfu-
sion in frontotemporal regions (Benoit et al., 1999; Craig
et al., 1996). In one study, apathetic stroke patients
Apathy. Table 1 Symptoms of apathy and depression
showed reduced regional cerebral blood flow in the right
Symptoms of Overlapping Symptoms of dorsolateral prefrontal cortex and the left frontotemporal
apathy symptoms depression regions (Okada et al., 1997). Subcortical regions may also
Loss of motivation Lack of interest in Dysphoria
be implicated in the presence of apathy. In one study,
and initiation events or activities apathy was seen in 80 stroke patients with lesions to
posterior limb of the internal capsule (Starkstein et al.,
Lack of persistence Lack of energy Hopelessness
1993). Apathy has also been observed with lesions to the
Diminished Psychomotor slowing Guilt
right hemisphere subcortical structures following TBI
emotional
reactivity
(Finset & Andersson, 2000).
Evidence from neuropsychological studies suggests
Reduced social Fatigue Pessimism
that apathy may be associated with cognitive impairment,
engagement
in particular, executive dysfunction. Apathetic patients
Poor insight Suicidal
with Alzheimers disease have been shown to have greater
ideation
executive functioning deficits, abilities thought to be
Loss of
mediated by the frontal lobes, than depressed patients
appetite
with Alzheimers disease (Kuzis et al., 1999). Another
Sleep
study found that apathetic patients with Alzheimers dis-
problems
ease showed significantly greater deficits on measures of
Apathy A 213
executive functioning, but performed similarly on other disinhibition, and executive dysfunction. This allows for
A
neuropsychological measures not dependent on executive an estimation of the extent to which current problem
function (McPherson et al., 2002). Apathy has also been behaviors represent a change from premorbid function-
associated with executive dysfunction in other clinical ing. T-scores greater than 65 are clinically significant. The
populations, including TBI (Andersson & Bergedalen, FrSBe has been shown to be reliable, valid, and sensitive to
2002), Parkinsons disease (Starkstein et al., 1992), pro- behavior change due to frontal lobe damage (Grace et al.,
gressive supranuclear palsy (Litvan et al., 1998), and HIV 1999), Alzheimers disease (Stout et al., 2003), TBI (Lane-
(Castellon et al., 2000). Brown & Tate, 2009), and a variety of other neurological
conditions.
The NPI is a structured interview conducted with
Evaluation an informant designed to assess for the presence of 12
neuropsychiatric symptoms, including apathy (Cum-
Formal assessment measures for apathy focus on those mings, 1997). A positive response to a screening question
symptoms of apathy that are distinct from depression. indicates the presence of the symptom and leads to fur-
The most commonly employed assessment instruments ther questions about the behavior and eventual ratings of
for apathy in clinical and research settings include the the symptom severity (mild, moderate, or severe) and the
Apathy Evaluation Scale (AES), the Neuropsychiatric amount of caregiver distress it causes. The Neuropsychia-
Inventory (NPI), and the Frontal Systems Behavior tric Inventory Questionnaire (NPI-Q) (Kaufer et al.,
Scale (FrSBe). Less commonly used but validated 2000) is a self-administered questionnaire completed by
measures include the Dementia Apathy Interview and a caregiver or informant that assesses for the presence of
Rating, the Lille Apathy Rating Scale, the Apathy Inven- the same 12 symptoms and asks for ratings of severity and
tory, the Behavior Rating Scale for Dementia, and the caregiver distress using the same rating scale as the NPI
Scale for the Assessment of Negative Symptoms in Alz- interview. Importantly, both of these versions of the NPI
heimers disease. Of note, while several of these measures include separate questions for depression and apathy. The
include self-report versions, these may fail to identify NPI asks caregivers to consider whether the behavior has
apathy in patients with reduced insight, and, therefore, been present for the past month. The NPI has been shown
informant measures may be more helpful in assessing for to have good reliability and validity; however, unlike the
apathy. other measures discussed, there is no recommended cut-
The AES comes in a clinician-administered version, off score for clinical significance. Of note, while the AES
an informant version, and a self-report version, all of and FrSBe provide more nuanced assessments of apathy,
which have been shown to have satisfactory reliability the NPI is the most widely reported measure of apathy
(Marin et al., 1991). The clinician-administered version reported in the literature. This is likely due to the fact that
(AES-C) of this measure is a semi-structured interview the NPI assesses for a wide array of neuropsychiatric
which includes 18 items and is focused on behavior that symptomatology, and is often used in intervention studies
has been present during the past month. Each item falls for a variety of conditions of which apathy may be one
into one of four categories (cognitive, behavior, emotion- symptom, but not a cardinal feature of a disorder.
al, or other) and is rated on a 4-point Likert scale, with
higher scores representing a greater degree of apathy.
A recent study examined the AES-C and found it to be Treatment
valid and reliable for identifying and quantifying apathy,
and found that using a cut-off score of 40.5 resulted in Nonpharmacologic interventions for apathy tend to focus
good sensitivity and moderate specificity (Clarke et al., on introducing new sources of interest and stimulation.
2007a; Clarke et al., 2007b). Pet therapy, art therapy, and physical therapies may be
The FrSBe (Grace & Malloy, 2001) was specifically useful in decreasing apathy, though the efficacies of these
designed to assess for behavioral changes associated with interventions have not been examined in a systematic
frontal lobe dysfunction and comes in a self-report and fashion with apathetic patients. Increasing opportunities
informant version. This questionnaire consists of 46 items for socialization and encouraging participation in social
and asks the respondent to rate the patients behavior on activities may also be helpful. Patients should be encour-
each item using a five-point Likert scale. Respondents are aged to be as functionally autonomous as possible. Senso-
asked to rate the patients behavior both before and after ry deficits and pain should be managed so that these do
the onset of illness or injury. Subscales assess apathy, not interfere with activities. Implementing exercise
214 A Apathy
programs and scheduled activities may also be beneficial commonly used to treat attention deficit/hyperactivity
in enhancing initiation and motivation. While there have disorder (AD/HD) and narcolepsy. These medications
been few studies on behavioral interventions specifically have also been used to treat apathy in Alzheimers disease,
for apathy, there is some evidence that behavioral therapy normal pressure hydrocephalus, Parkinsons disease,
may be helpful in reducing apathetic symptomatology. cerebrovascular accidents, and depression (Chatterjee &
One randomized controlled study comparing reminis- Fahn, 2002; Jansen et al., 2001; Keenan et al., 2005; Padala
cence therapy (a treatment modality designed to facili- et al., 2007b; Spiegel et al., 2009). However, most of
tate recall of experiences from the past to promote the evidence for the efficacy of these medications on
intrapersonal and interpersonal functioning) to a time apathy comes from case reports or case series. These
and attention control group (one-on-one time with an medications can also have negative side effects, including
activity therapist) found that apathy was reduced for both insomnia, loss of appetite, anxiety, and higher blood
groups of patients with dementia (Politis et al., 2004). pressure, which may deter their use with vulnerable popu-
Another study showed that individualized functional lations (Ishii et al., 2009). Other stimulating medications
and occupational training reduced apathy in patients such as modafinil (Padala et al., 2007a) and selegiline
with mild to moderate-stage dementia (Lam et al., (Newburn & Newburn, 2005) have been reported to
2010). Behavioral activation therapy (BA) is an interven- reduce apathy in case studies, however, further study is
tion which focuses on alleviating depression by increasing needed.
the individuals exposure to rewarding and reinforcing Reductions in apathy with the use of dopaminergic
stimuli by increasing activation and decreasing avoidance agents such as bromocriptine (Powell et al., 1996) and
behaviors (Dimidjian & Davis, 2009). This behavioral amantadine (Swanberg, 2007; van Reekum et al., 1995)
approach includes goal setting, activity scheduling, prob- have been reported in a few case studies, but no rando-
lem solving, and self-monitoring, to get patients to mized clinical trials have been conducted to date.
become more active and, thus, increase exposure to re- Apathetic-type symptoms and behavior may be seen in
ward, and positive reinforcement to combat depressive schizophrenic patients with negative symptoms. Atypical
symptomatology. It has been shown to be comparable antipsychotic medications such as risperidone, olanza-
to cognitive behavior therapy and pharmacotherapy pine, and clozapine have been shown to be helpful in
(paroxetine) in reducing depressive symptomatology in reducing negative symptoms in schizophrenia (van
placebo-controlled studies (Dimidjian et al., 2006; Reekum et al., 2005). However, none of the studies to
Sturmey, 2009). While this intervention has not been date has specifically examined apathy, and these medica-
examined in the treatment of apathy, its focus on tions can be associated with serious negative side effects
increased activity and exposure to pleasant, rewarding such as tardive dyskinesia, akathisia, extra pyramidal
experiences would appear to be particularly well-suited symptoms, and orthostatic hypotension.
to address the lack of interest, motivation, and anhedonia As previously noted, apathy is the most common
that characterize apathy. Future research may show this to neuropsychiatric symptom associated with Alzheimers
be a promising intervention for both depression and disease, and modest improvements in apathy have
apathy. been seen in patients with Alzheimers disease who are
Psychoeducation for families and caregivers can also treated with acetylcholinesterase inhibitor medications
be beneficial. Oftentimes, apathy is mischaracterized as a (Cummings, 2000; Mega et al., 1999). Currently, there
willful behavior (e.g., stubbornness or laziness) by care- are three acetylcholine inhibitor medications approved
givers who do not recognize that these behaviors are for use in the United States: donepezil, galantamine, and
related to neurological, psychiatric, and medical comor- rivastigmine. A recent meta-analysis identified 14 rando-
bidities. Educating families on the underlying causes for a mized, placebo-controlled trials of monotherapy with
patients low initiation and motivation may help lessen these medications in patients with Alzheimers disease
perceived caregiver burden and stress. that reported a behavioral outcome (Rodda et al., 2009).
Currently, there is no FDA-approved pharmacological Of these, only four were specifically designed to assess
intervention for apathy, however, many different medi- behavioral outcomes, and the rest used behavioral out-
cations, including acetylcholinesterase inhibitors, psy- comes as secondary measures. Overall, three of the 14
chostimulants, dopaminergic drugs, and atypical studies reviewed reported a statistically significant im-
antipsychotics, have been used off-label to treat apa- provement in the overall score on the Neuropsychiatric
thetic symptomatology. Methylphenidate and dextroam- Inventory, and only one found a significant reduction in
phetamine are psychostimulant medications that are apathy, specifically (Gauthier et al., 2002).
Apathy A 215
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van Dijk, P. T., Dippel, D. W., & Habbema, J. D. (1994). A behavioral Many systems have been proposed to classify aphasia
rating scale as a predictor for survival of demented nursing types (Kertesz, 1979). Each system represents a theoretical
home patients. Archives of Gerontology and Geriatrics, 18(2), perspective of aphasia and identifies aphasia types accord-
101113.
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van Reekum, R., Bayley, M., Garner, S., Burke, I. M., Fawcett, S., Hart, A.,
et al. (1995). N of 1 study: Amantadine for the amotivational
Classification systems can be dichotomous (e.g., fluent
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9(1), 4953. deficit), anatomically and behaviorally based (e.g., Boston
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Journal of Neuropsychiatry and Clinical Neurosciences, 17(1), 719.
aphasia), behaviorally based (e.g., Schuells system of
Wood, S., Cummings, J. L., Hsu, M. A., Barclay, T., Wheatley, M. V.,
Yarema, K. T., et al. (2000). The use of the neuropsychiatric invento-
multimodality, unidimensional impairment, such as
ry in nursing home residents. Characterization and measurement. aphasia with visual involvement), based on severity (e.g.,
American Journal of Geriatric Psychiatry, 8(1), 7583. mild, moderate, or severe), or follow a processing model
Zawacki, T. M., Grace, J., Paul, R., Moser, D. J., Ott, B. R., Gordon, N., (e.g., cognitive neuropsychological model of naming; Kay,
et al. (2002). Behavioral problems as predictors of functional abil-
Lesser, & Coltheart, 1996). Classification systems are use-
ities of vascular dementia patients. Journal of Neuropsychiatry and
Clinical Neurosciences, 14(3), 296302.
ful for a general understanding of an individuals
communication ability; however, controversy exists
regarding their clinical utility. Some individuals with
aphasia show symptoms that match more than one type
of aphasia and others show symptoms that do not fit into
Aphasia any of the classification categories. Studies examining
classification report 3570% success in classifying
J ANET PATTERSON participants as one aphasia type. Table 1 shows three
East Bay classification systems, with general characteristics of
Hayward, CA, USA each aphasia type.
Epidemiology
Short Description or Definition
Aphasia resulting from stroke occurs in approximately
Aphasia is an acquired communication disorder caused
80,000 people each year, affecting about 30% of indivi-
by brain damage, characterized by impairments of
duals who have a first-ever ischemic or hemorrhagic
language modalities; speaking, listening, reading and
stroke. Approximately, one million people in the United
writing; it is not the result of a sensory or motor deficit,
States are living with aphasia following stroke. Aphasia
a general intellectual deficit, confusion or a psychiatric
resulting from traumatic brain injury and other causes is
disorder (Hallowell & Chapey, 2008, p. 3). Aphasia is
difficult to estimate.
typically acquired suddenly as a result of a stroke and can
also appear following traumatic brain injury or other
neurological events such as tumor or disease. When Natural History, Prognostic Factors,
aphasia develops slowly over time and is the only Outcomes
behavioral symptom present, the diagnosis is typically
primary progressive aphasia (PPA). Aphasia is often Reports of language disorder following brain injury have
classified according to the appearance of a constellation existed for hundreds of years, initially primarily as case
218 A Aphasia
Aphasia. Table 1 Three examples of aphasia classification systems showing aphasia types and general characteristics
of each type
Dichotomous classification
Type Characteristics
Nonfluent aphasia Limited speech output
Effortful speech output
Content words retained; function words omitted
May or may not have articulation difficulties
Melodic contour altered
Fluent aphasia Approximates normal rate and sentence length
Content words omitted in severe fluent aphasia
Circumlocution present in mild fluent aphasia
Melodic contour preserved
Anatomical and behavioral classification
Type Characteristics
Brocas aphasia Nonfluent aphasia; expressive aphasia
Effortful output
Reduced phrase length and syntactic complexity; content words
usually preserved
Auditory comprehension may or may not be impaired
Impairments in reading, writing, naming, and repetition
Right hemiplegia often present
Wernickes aphasia Fluent aphasia; receptive aphasia
Auditory comprehension usually impaired
Impairments of reading, writing, naming, and repetition
Paraphasic errors
Melodic contour retained
Conduction aphasia Fluent aphasia
Auditory comprehension preserved
Impairment in repetition
Naming may be impaired
Error recognition typically preserved
Global aphasia Nonfluent aphasia
Impairments in auditory comprehension, reading writing, naming, and repetition
Limited functional communication often preserved
Anomic aphasia Fluent aphasia
Auditory and reading comprehension and repetition preserved
Word retrieval deficit
Transcortical motor aphasia Nonfluent aphasia
Auditory comprehension and naming may be impaired
Repetition preserved
Paraphasic errors and perseveration present
Aphasia A 219
reports. Paul Broca and Carl Wernicke in the late 1800s do not systematically influence prognosis (Pedersen,
presented clinical data relating behavioral and anatomical Jorgensen, Nakayama, Raaschou, & Olsen, 2004).
information, localizing language ability to the left hemi- Aphasia recovery occurs most rapidly immediately
sphere, and ultimately having their names adopted to following the brain injury, as the brain begins to heal
identify anatomical areas in the brain related to patterns itself. Studies have shown that recovery also continues
of language behavior. Current studies of persons with for years post stroke and treatment (Moss & Nicholas,
aphasia use neuroimaging techniques to further elucidate 2006). Outcome measures documenting change are
the behavioral and anatomical relationship. impairment-based (e.g., change in naming ability) or
Aphasia in the first few months after a stroke is the activity/participation-based (e.g., increased participation
acute stage and is often characterized by spontaneous in social activities), following the World Health Organiza-
recovery of language and communication deficits. In the tions International Classification of Functioning, Disabil-
chronic stage, an individual learns to live with aphasia and ity and Health (ICF; WHO, 2001). Some persons with
return to life activities. Prognosis for recovery is variable aphasia recover to near normal premorbid language and
and dependent upon both internal patient factors (e.g., communication performance while others remain severe-
severity of aphasia, type and extent of lesion, or concomi- ly aphasic. Almost every person has the potential for some
tant medical problems) and external factors (e.g., family level of functional communication, from being an inde-
support or communication interaction opportunities). pendent communicator in a variety of communication
Personal variables such as age, education, and gender interactions to being dependent upon an alternative or
220 A Aphasia
augmentative communication system or a conversational breadth and depth of an individuals support and
partner. communication networks (e.g., Blackstone & Berg,
2003). Several scales have been developed to screen for
depression. Some have a linguistic bias or rely on caregiver
Neuropsychology and Psychology report while others have been adapted to be aphasia
of Aphasia friendly and not depend exclusively on complex written
sentences. Three examples of instruments to examine
Cognitive neuropsychology has brought to aphasia depression are the Stroke Aphasia Depression Question-
evaluation and treatment a set of models of human naire (SADQ) (Lincoln, Sutcliffe, & Unsworth, 2000), the
cognitive mechanisms and processes thought to underlie Aphasia Depression Questionnaire (Benaim, Cailly,
language performance. An individuals performance on Perennou, & Pelissier, 2004) and the Visual Analog
several linguistic tasks is examined for patterns of Mood Scale (Stern, Arruda, Hooper Wolfner & Morey,
impaired and spared cognitive processes to infer the 1997). The SADQ while designed for persons with aphasia
cognitive architecture that underlies the performance. has a linguistic bias and is intended to rely on caregiver
For example, in a model of lexical processing, the linguistic report. The ADQ is a nine item tool used to assess post-
tasks might be lexical recognition (word/nonword stroke depression in persons who are hospitalized after
identification), auditory comprehension (pointing to a a stroke. The VAMS is an example of a non-linguistic
named word), and naming a picture (confrontation mood scale used for self-report of depressive symptoms.
naming). An individual who scores high on auditory
comprehension and reading words tasks but low on
confrontation naming may be inferred to show a deficit Evaluation
in phonological output lexicon but have an intact
semantic system and ability to use phonic skills to read Approaches to evaluation of aphasia vary with the
a word. That is, the individual may have intact semantic conceptualization of aphasia.
knowledge and be aware of the phonological form of a Some approaches take an impairment-based
word and be able to read it, but lack the phonological approach, viewing aphasia as a disorder of selected
skills to generate the verbal label. The performance abilities while others, such as the Life Participation
pattern serves to direct treatment to the impaired process- Approach to Aphasia (Chapey et al., 2008) take an activi-
es, using the spared processes as strengths. Cognitive ty/participation approach, viewing aphasia as a disrup-
neuropsychological models of language processing tion to communication and placing the person with
frequently used in aphasia assessment and treatment, aphasia and his or her family at the center of clinical
however, are not without criticism as being descriptive and decision-making activities. (Schuell, Jenkins & Jimenez-
not prescriptive, and requiring time-consuming assessment. Pabon, 1964) proposed a Stimulation-Facilitation model
In contrast to the deficit-specific models of cognitive based on auditory comprehension stimuli that are indi-
neuropsychology, the psychology of aphasia in assessment vidually adapted to persons with aphasia. Chapey et al.
and treatment recognizes the importance of an support a Cognitive Stimulation model, which views
individuals psychosocial state, quality of life, functional communication as a problem-solving and decision-
communication abilities, and communication network. making task. Following the World Health Organization
Tanner (2003) proposed an eclectic approach to examine ICF (2001), models of assessment and treatment typically
the psychology of aphasia from three perspectives: incorporate information at levels of impairment and ac-
effects of brain injury, psychological defenses and coping tivity/participation. Group treatment has gained popular-
styles, and responses to loss. This view speaks to the ity in recent years, recognizing the value of social
importance of an individuals premorbid personal connectedness (Avent, 1977; Kearns & Elman, 2008).
characteristics, their ability to adjust to change, and Lubinski (2008) discussed an environmental model, sug-
their external support network as they and their family gesting that clinicians consider physical and social envir-
learn to live with aphasia. Several models and tools exist onments of a person with aphasia to enhance treatment
to guide assessment and treatment in these areas. For effects. Finally, psychosocial models of intervention focus
example, quality-of-life scales ask questions about topics on integrating an individual into a communicating society
such as family support and general outlook on life (e.g., and promoting their participation in personally relevant
Communication-Related Quality of Life Scale; Cruice activities (Simmons-Mackie, 2008). Regardless of the ap-
et al., 2003). Social network diagrams illustrate the proach, in order to understand the linguistic and
Aphasia A 221
communicative abilities and needs of an individual, it is characterized by spontaneous recovery of language and
A
important to conduct an evaluation within a culturally communication deficits, while in the chronic stage of
sensitive framework. aphasia an individual learns to live with aphasia and
Three types of aphasia tests are commonly used to return to life activities. There are many well-validated,
assess language and communication abilities in persons effective techniques for aphasia rehabilitation, particular-
who have aphasia: screening tests (short assessments that ly for chronic aphasia. These range from general stimula-
may be administered at bedside), comprehensive aphasia tion approaches to treatments aimed at specific signs of
tests (batteries containing several subtests such as naming, aphasia, and are chosen according to the patients
reading, and writing), and tests of specific linguistic or individual needs, goals, aphasia characteristics, and
communicative function (e.g., syntactic function or etiology. For aphasia due to acute-onset causes (e.g.,
naming) (Patterson, 2008). In addition, assessment of vascular etiologies or trauma), therapy has been demon-
aphasia and its impact on a persons life includes testing strated to be effective both early after onset as well in the
cognitive abilities (e.g., memory), testing executive chronic stage. For aphasia due to progressive etiologies,
functioning (e.g., divided attention), observing a person therapy has been shown to be effective in maintaining
in activities of daily communication, and interviewing the functional communication and maximizing quality of
person with aphasia and family members about the communication life to the extent possible given the med-
impact of aphasia on life participation and functional ical diagnosis.
communication. Pharmacological intervention for aphasia may be
In aphasia assessment it is as important to determine undertaken for direct treatment of the language deficit
the presence or absence of aphasia, and presence of or administered to address a concomitant disorder, such
concomitant disorders, as well as to classify aphasia type as depression. Although research in this area is encouraging,
or describing aphasia symptoms. Examples of disorders to date no pharmacologic treatment has emerged
that may accompany aphasia but that are not aphasia are as consistently improving linguistic function without
apraxia of speech, dysarthria, dementia, memory im- adverse side effects (Greener, Enderby, & Whurr, 2001;
pairment, or psychiatric problems. These concomitant Murray & Clark, 2006; Troisi et al., 2002).
disorders will affect treatment planning and task selection. Treatment for aphasia historically focused primarily
Medical conditions, such as diabetes, cardiovascular dis- on restitution of function using impairment-based
ease, and any medications the patient takes may affect treatment techniques, with treatment targets such as
performance and should also be noted in the assessment word or sentence production, or writing. Examples of
report. these treatment techniques are Melodic Intonation
The goals of evaluation will vary depending upon Therapy, a semantic or phonologic cueing hierarchy,
factors such as severity of aphasia, age, and time post- and confrontation naming. More recently, treatment
onset. For example, an individual with mild aphasia who goals have expanded to include activity/participation
anticipates returning to work should have an assessment based treatments such as functional communication
that includes detailed information on linguistic proces- and group therapy. Examples of activity/participation
sing and a job task analysis to determine the linguistic treatment methods are book groups for persons with
requirements of the position. This information may be aphasia (with the linguistic level of the book modified
used to determine the individuals ability to return to a to be aphasia friendly), reciprocal scaffolding (e.g.,
job, to identify communication requirements of the job, Avent, Patterson, Lu, & Small, 2009), and supported
and to guide employment-related treatment. In contrast, conversation (e.g., Kagan, Black, Duchan, Simmons-
evaluation for an individual with severe aphasia and Mackie, & Square, 2001).
concomitant severe apraxia of speech may require an The four principles of evidence-based practice,
evaluation focused on functional communication current best practices, clinical expertise, client/patient
strategies to use with familiar communication partners values, and context of treatment, guide treatment
within a contained environment. planning. Clinical practice research and clinical trials
support the efficacy and effectiveness of aphasia therapy.
Systematic reviews, such as the one for constraint-induced
Treatment language therapy (Cherney, Patterson, Raymer, Frymark, &
Schooling, 2008), and meta-analyses (e.g., Robey, 1998)
The acute stage of aphasia is the first few months after a report the evidence from group studies and single-subject
stroke as the brain recovers from injury, and is often research studies for a specific treatment or aphasia
222 A Aphasia
therapy in general. Cherney and Robey (2008) and the References and Readings
Academy of Neurological Communication Disorders
and Sciences (ANCDS, 2008) present analyses of Academy of Neurological Communication Disorders and Sciences.
treatment effect sizes of aphasia treatment for specific (2008). Practice guidelines of the ANCDS: Evidence based practice
guidelines for the management of communication disorders in
treatment areas such as syntax and language
neurologically impaired individuals. http://www.ancds.org/index.
comprehension. php?option=com_content&view=article&id=9&Itemid=9. Accessed
3/29/10.
Avent, J. R. (1997). Manual of Cooperative Group Treatment for Aphasia.
Cross References New York: Elsevier.
Avent, J., Patterson, J., Lu, A., & Small, K. (2009). Reciprocal scaffolding
Agnosia treatment: A person with aphasia as clinical teacher. Aphasiology,
Agrammatism 23, 110119.
Benaim, C., Cailly, B., Perennou, D., & Pellissier, J. (2004). Validation of
Agraphia
the Aphasic Depression Rating Scale. Stroke, 35, 16921969.
American Speech-Language-Hearing Association (ASHA) Blackstone, S., & Hunt Berg, S. (2003). Social networks: A communication
Anarthria inventory for individuals with complex communication needs and their
Anomic Aphasia communication partners. Monterey, CA: Augmentative Communica-
Aphasia Tests tion Inc.
Chapey, R., Duchan, J. F., Elman, R. J., Garcia, L. J., Kagan, A., Lyon, J.,
Apraxia of Speech
et al. (2008). Life participation approach to aphasia: A statement of
Augmentative or Alternative Communication (AAC) values for the future. In R. Chapey (Ed.), Language intervention
Boston Diagnostic Aphasia Examination strategies in aphasia and related neurogenic communication disorders
Boston Naming Test (5th ed., pp. 279289). Philadelphia: Wolters Kluwer.
Brocas Aphasia Cherney, L. R., Patterson, J. P., Raymer, S. M., Frymark, T., &
Schooling, T. (2008). Evidence-based systematic review: Effects of
Carl, Wernicke
intensity of treatment and constraint-induced language therapy
Conduction Aphasia for individuals with stroke-induced aphasia. Journal of Speech-
Crossed Aphasia Language-Hearing Research, 51, 12821299.
Cue Cherney, L. R., & Robey, R. R. (2008). Aphasia treatment: recovery,
Dysarthria prognosis and clinical effectiveness. In R. Chapey (Ed.),
Language intervention strategies in aphasia and related neurogenic
Dysgraphia
communication disorders (5th ed., pp. 186202). Philadelphia:
Edith Kaplan Wolters Kluwer.
Evidence-Based Practice Cruice, M., Worral, L., Hickson, L., & Murison, R. (2003). Finding focus
Fluent Aphasia for quality of life with aphasia: Social and emotional health, and
Global Aphasia psychological well-being. Aphasiology, 17, 333353.
Davis, G. A. (2006). Aphasiology: Disorders and clinical practice.
Harold Goodglass
Englewood Cliffs, NJ: Prentice Hall.
Melodic Intonation Therapy Greener, J., Enderby, P., & Whurr, R. (2001). Pharmacological treatment for
Multilingual Aphasia Examination aphasia following stroke. Cochrane Database of Systematic Reviews.
Neurosensory Center Comprehensive Examination Issue 4. Art. No.: CD000424. doi: 10.1002/14651858.CD000424.
for Aphasia Hallowell, B., & Chapey, R. (2008). Introduction to language intervention
strategies in aphasia. In R. Chapey (Ed.), Language intervention
Paragrammatism
strategies in aphasia and related neurogenic communication disorders
Paraphasia (5th ed., pp. 319). Philadelphia: Wolters Kluwer.
Paul Broca Kagan, A., Black, S. E., Duchan, J. F., Simmons-Mackie, N., & Square, P.
Pragmatic Communication (2001). Training volunteers as conversational partners using
Progressive Aphasia Supported Conversation for Adults with aphasia (SCA):
A controlled trial. Journal of Speech-Language-Hearing Research,
Semantic Paraphasia
44, 624638.
SpeechLanguage Therapy Kay, J., Lesser, R., & Coltheart, M. (1996). Psycholinguistic assessments
Subcortical Aphasia of language processing in aphasia (PALPA): An introduction.
Telegraphic Speech Aphasiology, 10, 159215.
Transcortical Motor Aphasia Kearns, K., & Elman, R. (2008). Group therapy for aphasia: Theoretical
and practical considerations. In R. Chapey (Ed.), Language interven-
Transcortical Sensory Aphasia
tion strategies in aphasia and related neurogenic communication
Wernickes Aphasia disorders (5th ed., pp. 376398). Philadelphia: Wolters Kluwer.
Wernicke-Lichtheim Model of Aphasia Kertesz, A. (1979). Aphasia and associated disorders: Taxonomy localization
Western Aphasia Battery and recovery. New York: Grune & Stratton.
Aphasia Diagnostic Profiles A 223
Aphasia Diagnostic Profiles. Table 1 Aphasia diagnostic profiles: Nine subtests and five profiles
ADP Subtests
Subtest Description
Personal information Verbal response to questions
Writing Complete Patient Information Sheet
Reading Read items on Patient Information Sheet
Fluency Produce connected speech in three contexts
Naming Name familiar pictured items
Auditory language comprehension Answer questions word, sentence, and story levels
Repetition Repeat words and phrases
Elicited gestures Pretend to complete action
Singing Sing 3 familiar songs
ADP Profiles
Profile Description
Aphasia Classification Profile Identifies aphasia type (based on the Boston
classification system)
Aphasia Severity Profile Indicates specific strengths and weaknesses
Alternative Communication Profile Identifies patients strongest response modalities
and guides therapy
Error Profiles Identify the communicative value of a patients
responses
Behavioral Profile Indexes the patients overall emotional state during
testing
The ADP was first published in 1992 and since then has Three characteristics make the ADP a valuable clinical
been frequently used in clinical and research activities. assessment tool: the theoretical foundation and close re-
Numerous studies of aphasia treatment use the ADP as a lationship to the Boston aphasia classification system, the
measure of behavior change following intervention. structure of the test and clarity of the administration
manual, and the amount of administration and scoring
time required. It is also notable that both verbal and
Psychometric Data nonverbal modalities of communication are included in
the assessment. One limitation of the ADP is that it does
The ADP manual reported that it was standardized on 290 not examine any linguistic, psycholinguistic, or neuropsy-
adults with neurological impairments (222 potentially chological behavior in detail; additional tests in specific
aphasic adults) and 40 nonaphasic adults. The median areas would be required to obtain in-depth information as
age of these individuals was 70 years. The manual further part of an extensive diagnostic evaluation.
reported reliability coefficients (inter-item consistency)
for subtest raw scores that ranged from 0.73 (Behavioral
Score) to 0.96 (Repetition), with most of the coefficients Cross References
in the 0.90s. Testretest coefficients ranged from 0.64
(Elicited Gestures) to 0.91 (Information Units). The ADP Anomia
has a strong theoretical and psychometric foundation Anomic Aphasia
but has not been subjected to additional psychometric Aphasia
evaluation. Aphasia Tests
Aphasia Tests A 225
Historical Background
References and Readings
Aphasia has been assessed more or less systematically for
Helm-Estabrooks, N. (1992). Aphasia diagnostic profiles. Austin, TX: Pro many years. Clinical observation was the earliest method
Ed Inc.
of assessment, and the first standardized test was
World Health Organization. (2001). International classification of func-
tioning, disability and health. http://www.who.int/classifications/ published in 1926 by Henry Head. In the ensuing years,
icfbrowser/ several comprehensive aphasia tests and specific linguistic
tests appeared. Each comprehensive test is based upon a
theoretical model of aphasia, and although the tests
contain common subtests (e.g., sentence repetition), the
Aphasia Evaluation test results and aphasia diagnoses vary. For example, the
Minnesota Test for Differential Diagnosis of Aphasia
Aphasia Tests (Schuell, 1965) assesses language performance across
several modalities and rests upon Schuells theory of
aphasia as a unitary reduction in language across
Aphasia Tests modalities with or without accompanying perceptual or
motor deficits. In contrast, the Boston Diagnostic Aphasia
J ANET PATTERSON Examination (Goodglass, Kaplan, & Baressi, 2001) relates
California State University speech and language behavioral deficits to neurological
Hayward, CA, USA lesions. With yet a different perspective, Luria (1966)
proposed a comprehensive examination for aphasia
through nonstandardized observation of language perfor-
Synonyms mance in several modalities, but without specific subtests.
In recent years, several tests have emerged to assess
Aphasia assessment; Aphasia diagnosis; Aphasia evaluation specific language or communication functions in PWA.
For example, the ASHA-FACS (Frattali et al., 1995)
assesses functional communication skills such as partici-
Description pating in conversation, while the Reading Comprehension
Battery for Aphasia (LaPointe & Horner, 1998) evaluates
Tests of aphasia are used to diagnose the type and reading performance in several contexts, such as single
severity of aphasia and related disorders and to plan words and paragraphs.
intervention for the speech, language, and communica-
tion deficits demonstrated by persons who have aphasia
following brain injury (PWA). Three types of aphasia tests Psychometric Data
are commonly used to assess language and communica-
tion abilities in PWA: screening tests, comprehensive The availability of psychometric data for aphasia tests
aphasia tests, and tests of specific linguistic or ranges from prolific and well documented for some tests
226 A Aphasia Tests
to minimal or nonexistent for others, and the data Examination (Benton, Hamsher, Rey, & Sivan, 1994), and
appear in scholarly journals as well as in the test manuals. the Neurosensory Center Comprehensive Examination for
Spreen and Risser (2003) and Strauss, Sherman, and Aphasia (Spreen & Benton, 1977).
Spreen (2006) provide overviews of psychometric data
for many general aphasia tests and supplemental language
tests. Few studies, and none recently, compared psycho- Tests of Specific Linguistic or
metric data across tests. In evaluating a general or supple- Communication Function
mental test for aphasia, several factors should be
considered, including size and definition of the standar- Tests of specific functions provide detailed information
dization sample; reports of item, concurrent and predic- about a persons abilities in one area of linguistic
tive validity; test-retest, interrater and intrarater or communication ability and are particularly useful
reliability; report of raw score means, standard deviations, for persons who have severe or minimal aphasia and for
and ranges; information about test development, examiner whom comprehensive aphasia batteries would understate
qualifications, administration instructions, scoring, and communication strengths and weaknesses. Three
interpretation; and normative data. examples are the Revised Token Test (McNeil & Prescott,
Although it is difficult to judge which of the many 1978) for auditory comprehension, the Boston Naming
aphasia tests best meets all the factors mentioned above, Test (Goodglass, Kaplan & Weintraub, 2001) for
there are four tests that are frequently used in clinical oral naming, and the Psycholinguistic Assessments of
settings and have the most psychometric data published Language Processing in Aphasia (Kay, Lesser, & Coltheart,
about them: Boston Diagnostic Aphasia Examination, 1992).
Boston Naming Test, Token Test (and Revised Token Test),
and Western Aphasia Battery.
Tests of Cognitive-Communication Abilities
and Related Functions
Clinical Uses
Tests of cognitive-communicative abilities related to
Screening Tests for Aphasia language functions have been included as part of com-
prehensive aphasia batteries (e.g., the Ravens Progres-
Screening tests for aphasia are brief and may be adminis- sive Matrices [Raven, Raven, & Court, 1995] as part of the
tered at bedside. Their purpose is to rapidly determine Cortical Quotient in the WAB) or administered indepen-
the presence of aphasia or the need for further assessment. dently (e.g., Wechsler Memory Scale; Wechsler, 2009).
A screening test may be independent (e.g., Quick Assess-
ment for Aphasia; Tanner & Culbertson, 1999) or a short-
ened form of a comprehensive aphasia battery, such as the Tests of Functional Communication
Western Aphasia Battery (WAB; Kertesz, 2006).
Functional communication abilities in PWA are assessed
through observation or the use of specific tests. Func-
Comprehensive Aphasia Batteries tional communication includes verbal and nonverbal
methods of conveying information in activities of daily
A comprehensive aphasia battery is based on a theoretical living, such as reading signs, greeting individuals and
model of aphasia and contains several subtests. For participating in conversation. Functional communica-
example, the Boston Diagnostic Aphasia Examination tion assessed through observation can be contextually
(Goodglass et al., 2001) has 34 subtests and the perfor- bound, such as assessing conversation with familiar or
mance pattern is used to classify an individual with an unfamiliar partners. Tests of functional communication
aphasia type (e.g., Brocas aphasia). Although some are intended to simulate activities of daily living but
subtests of comprehensive aphasia batteries may appear typically are acontextual. Two examples of tests of
similar, the data obtained from each of the subtests and functional communication are the Communicative
the resulting aphasia diagnosis will vary according to the Activities of Daily Living 2 (Holland, A. L., Frattali, C.
theoretical model of aphasia which underlies the test. M. & Fromm, D. 1999) and the Assessment of Language-
Other comprehensive aphasia batteries are the Western Related Functional Activities (Baines, Heeringa, & Martin,
Aphaisa Battery (Kertesz, 2006), the Multilingual Aphasia 1999).
Aphonia A 227
Cross References Spreen, O., & Risser, A. H. (2003). Assessment of aphasia. Oxford: Oxford
A
University Press.
Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium
Activities of Daily Living of neuropsychological tests: Administration, norms and commentary
Aphasia (3rd ed.). Oxford: Oxford University Press.
Augmentative or Alternative Communication Wechsler, D. M. (2009) Wechsler Memory Scale - 4th Edition. San Antonio
Boston Diagnostic Aphasia Examination TX: Psychological Corporation.
Boston Naming Test
Luria, Alexander Romanivich (19021977)
Multilingual Aphasia Examination
Neurosensory Center Comprehensive Examination
for Aphasia Aphonia
Wechsler Memory Scales
Western Aphasia Battery LYN T URKSTRA
University of Wisconsin-Madison
Madison, WI, USA
References and Readings
Baines, K. A., Martin, K. W., & Heeringa, H. M. (1999). ALFA: Assessment Synonyms
of Language Related Functional Acclivities. Austin TX: Pro-Ed.
Benton, A. L., Hamsher, K. deS., Rey, G. J., & Sivan, A. B. (1994). Mutism
Multilingual Aphasia Examination (MAE-3). Lutz FL: Psychological
Assessment Resources Inc (PAR).
Davis, G. A. (2007). Aphasiology: Disorders and clinical practice (2nd ed.).
Boston: Pearson Allyn & Bacon.
Frattali, C. M., Thompson, C. K., Holland, A. L., Wohl, C. B., &
Definition
Ferketic, M. M. (1995). The American Speech-Language-Hearing As-
sociation Functional Assessment of Communication Skills in Adults. Mutism is the complete absence of voice, i.e., adduction
Rockville, MD: The American Speech-Language-Hearing Association. and vibration of the vocal folds is insufficient for vocal
Goodglass, H., Kaplan, E., & Weintraub, S. (2001). Boston Naming Test
production. Aphonia may be associated with vocal fold
(2nd ed.). Austin TX: ProEd.
Goodglass, H., Kaplan, E., & Baressi, B. (2001). Boston Diagnostic Aphasia
paralysis; trauma; severe cases of inflammation, edema,
Examination (3rd ed.). San Antonio, TX: Psychological Corporation. or scarring of the vocal folds; benign or malignant
Head, H. (1926). Aphasia and kindred disorders of speech. New York: diseases of the vocal folds that interfere with vocal
MacMillan. fold closure; neurologically based movement disorders
Holland, A. L., Frattali, C. M., & Fromm, D. (1999). Communicative
(e.g., spasmodic dysphonia); overuse of the voice; or
Activities of Daily Living - 2nd Edition. San Antonion: Psychological
Corporation.
somatoform disorders (e.g., in forms of elective mut-
Kay, J., Lesser, R., & Coltheart, M. (1992) Psycholinguistic Assessment of ism). Aphonia may be intermittent or episodic. For
Language Processes in Aphasia. London: Taylor & Francis Group. example, individuals with spasmodic dysphonia may
Kertesz, A. (2006). Western Aphasia Battery. New York: Grune & Stratton. have periodic, abnormal abduction or adduction of
LaPointe, L. L., & Horner, J. (1998). Reading Comprehension Battery for
the vocal folds that may be perceived as voice breaks.
Aphasia (RCBA2). San Antonio TX: Pearson.
Luria, A. R. (1966). Higher cortical functions in man. New York:
Individuals who stutter also may have periodic voice
Basic Books. breaks, in this case associated with tight adduction of
McNeil, M. R., & Prescott, T. E. (1978). Revised Token Test. Austin, the vocal folds.
TX: Pro-Ed. When voice loss is incomplete, or when vocal quality
Patterson, J. P. (2008). Assessment of language disorders in adults.
is affected without complete loss of voice (e.g., if the voice
In R. Chapey (Ed). Language intervention strategies in aphasia and
related neurogenic communication disorders (5th ed., pp. 64160).
is hoarse), it is referred to as dysphonia. Aphonia and
Baltimore: Wolters Kluwer. dysphonia refer specifically to abnormal sound output
Raven, J., Court, & Raven, J. C. (1995). Ravens Progressive Matrices. from the phonatory sound source (i.e., the larynx), and
San Antonio: The Psychological Corporation. should be distinguished from anarthria or dysarthria,
Schuell, H. (1965). Minnesota Test for Differential Diagnosis of Aphasia.
which are disorders of articulation, i.e., related to the
Minneapolis: University of Minnesota Press.
Spreen, O., & Benton, A. L. (1977). Neurosensory Center Comprehensive
movements of the tongue, lips, jaw, and soft palate.
Examination for Aphasia. Victoria BC: University of Victoria Accordingly, dysphonia or aphonia can occur indepen-
Neuropsychology Laboratory. dently from anarthria or dysarthria.
228 A APM
Cross References
Alzheimers Disease
APOE
Apolipoprotein E References and Readings
Plomin, R., Defries, J. C., Craig, I. W., & McGuffin, P. (2003). Behavioral
genetics in the postgenomic era. Washington DC: American Psycho-
Apolipoprotein E logical Association.
J OHN D E LUCA
Kessler Foundation Research Center
West Orange, NJ, USA Apoptosis
K ATHLEEN L. F UCHS
Definition University of Virginia Health System
Charlottesville, VA, USA
Apolipoprotein E (ApoE) is a polymorphic plasma glyco-
protein that transports cholesterol and other lipids, and
has been shown to be involved in the growth and repair of Synonyms
neurons. There is also some evidence to suggest that ApoE
is involved in lipid redistribution after demyelination. The Programmed cell death
ApoE protein is mapped to chromosome 19 and is poly-
morphic with three major isoforms, each of which trans-
lates into three alleles of the gene: ApoE-2, ApoE-3, and Definition
ApoE-4. ApoE-2 is associated with the genetic disorder
type III hyperlipoproteinemia. There is also some evi- Apoptosis is both a normal developmental process to
dence that this allele may serve as a protective role in the rid the body of overproduced cells as well as a sign of
development of Alzheimers disease (AD). ApoE-3 is pathology in mature neural systems. Apoptosis involves
found in approximately 64% of the population, and is activation of caspases proteins that cleave other pro-
considered as the neutral ApoE genotype. ApoE-4 has teins in order to inactivate or modulate them to trigger
Apperceptive Visual Agnosia A 229
Definition
Appalic Syndrome Inability or marked difficulty in visually identifying an
object or picture of an object as a result of impaired
D ONA E C L OCKE
perceptual abilities. In apperceptive agnosia, in addition
Mayo Clinic
to problems in the visual identification of an object,
Scottsdale, AZ, USA
patients show impairment in reproducing (e.g., by draw-
ing) the object or image and even matching the item to a
similar one within a visual array. This contrasts with
Synonyms
associative visual agnosia in which identification may
also be impaired but the patient can usually render a
Persistent vegetative state
reasonable representation (e.g., a drawing or graphomo-
tor copy) of the object that cannot be visually identified
Definition and can visually match it to a sample. Apperceptive visual
agnosia likely results from a defect in the secondary asso-
Appalic syndrome is an older term that has been replaced ciation areas of the visual cortex and is usually found in
by persistent vegetative state. The vegetative state is a patients who complain of general loss or reduction in
clinical condition of complete unawareness of the self and visual acuity.
the environment, accompanied by sleepwake cycles with
either complete or partial preservation of hypothalamic
and brain-stem autonomic functions. A thorough clinical Cross References
evaluation may be required to distinguish between persis-
tent vegetative state and other conditions, including Associative Visual Agnosia
coma, brain death, and locked-in syndrome.
DeRenzi, E., & Spinnler, H. (1966). Visual recognition in patients with Rationale or Underlying Theory
unilateral cerebral disease. Journal of Nervous and Mental Disease,
142, 513525.
DeRenzi, E., Scotti, G., & Spinnler, H. (1969). Perceptual and associative
From a behavioral systems perspective, behavioral devel-
disorders of visual recognition. Relationship to the side of the opment is a function of the reciprocal interaction of a
cerebral lesion. Neurology, 19, 634642. persons: (a) genetic-constitutional makeup, (b) history of
interactions, (c) current physiological conditions, (d) cur-
rent environmental conditions, and (e) behavioral dy-
namics or behavior change over time (Novak & Pelaez,
Applied Behavior Analysis 2004). Treatment providers should consider all these fac-
tors when developing behavioral programs for individuals
A NTHONY C UVO
with neuropsychological disorders. A major conceptual
Southern Illinois University
focus of ABA is to understand, explain, and control the
Carbondale, IL, USA
operant behavior of humans in their environment.
The most basic form of operant conditioning is the
probabilistic strengthening of a response by its reinforcing
Definition consequences and the weakening of a response by its
punishing consequences. For example, access to extra
Applied behavior analysis (ABA) is the science in which
computer time might reinforce the timely completion of
tactics derived from the principles of behavior are applied
academic work by students with attention deficit disorder,
to improve socially significant behavior and experimenta-
and loss of free play might punish their noncompliance.
tion is used to identify the variables responsible for the
In addition to control of behavior by its consequences,
improvement in behavior (Cooper, Heron, & Heward,
behavior can be evoked by stimuli that precede it. For
2007, p. 690).
example, a written or pictorial prompt might evoke a
medication taking response by a person with acquired
Historical Background head injury. The beneficial treatment effects and avoid-
ance of adverse effects by not taking the medication might
The most notable figure in ABA is B. F. Skinner whose increase the probability that the person will take it.
book, The Behavior of Organisms (1938), described his The probability that a response actually will occur at a
animal research on operant conditioning. Skinner given time can be influenced by contextual variables. For
explained how behavior operates on the environment example, severe symptoms of allergies on a particular day
and is a function of its environmental consequences. might increase the aversiveness of otherwise tolerable
Subsequently, Skinner explained the application of behav- academic task demands on a student with attention deficit
ioral principles and processes discovered in the animal disorder and increase the probability that the student will
laboratory to a utopian society (1948), human behavior engage in task escape behavior that day.
(1953), verbal behavior (1957), teaching (1968), and During the past decade, there has been a growing body
other issues related to ABA. of research on relational responding and relational frame
Research on the application of basic behavioral prin- theory (e.g., Hayes, Barnes-Holmes, & Roche, 2001;
ciples and processes to important societal concerns began Sidman, 1994) that has provided the conceptualization
to emerge in the middle of the twentieth century. The and supporting empirical data to account for a broader
Journal of Applied Behavior Analysis, the flagship journal range of phenomena relevant to ABA. For example, the
of the discipline, began publication in 1968 as an outlet theory explains how individuals who experience painful
for the emerging ABA research. In the initial issue of that medical procedures can develop a wide range of fears to
journal, the defining characteristics of ABA were identi- various persons, settings, and objects (Friman, 2007).
fied as being applied, behavioral, analytical, technological,
conceptually systematic, effective, and capable of produc-
ing generalizable outcomes (Baer, Wolf, & Risley, 1968). Goals and Objectives
Since then, ABA research has found a welcome home in
numerous professional journals in various disciplines. ABA treatment goals, regardless of neuropsychological
The Association for Behavior Analysis-International was population, can be broadly classified as efforts to promote
established in 1974 and is ABAs principal professional the acquisition, maintenance, fluency (i.e., rate), and
organization. generalization of adaptive behavior, as well as the
Applied Behavior Analysis A 231
reduction of challenging behavior. An individuals treat- people in their environment (e.g., staff, parents). Treat-
A
ment goals and objectives should be determined by an ment participants have been of diverse ages, diagnoses,
analysis of the persons behavioral excesses and deficits and severity of disability. Intervention has occurred in
based on their expectations in the environmental context both laboratory and natural settings for numerous adap-
(i.e., goals should be socially valid). tive and challenging behaviors to meet goals and objec-
The social validity of treatment goals can be deter- tives, such as those previously stated. The largest body of
mined more formally either by social comparison or sub- research and application can be found for persons with
jective evaluation techniques (Kazdin, 1977). The former developmental disabilities, especially intellectual disabili-
relies on considering an individuals behavior with respect ty, and more recently autism spectrum disorders. For
to that of an appropriate comparison group. For example, approximately 50 years, research and application for indi-
the classroom out-of-seat behavior of a child with hyper- viduals with intellectual disability has occurred across the
activity could be compared with that of his classmates lifespan, severity of the disability, behavioral topogra-
who serve as the social validation criterion. Does the phies, and in institutional and community settings. ABA
childs out-of-seat behavior fall unacceptably outside the research and applications to autism have been more lim-
range of his or her peers? Subjective evaluation relies on ited, but noteworthy, with children being the most fre-
the opinion of key persons in the environment as a social quent recipient of treatment. There is a much smaller, but
validation criterion. For example, the teacher might rate nevertheless important, body of ABA treatment demon-
the childs out-of-seat behavior daily with respect to its strations for individuals with other neuropsychological
acceptability. Is the childs out-of-seat behavior unaccept- disorders, including acquired head injury, Alzheimers
able in the opinion of the teacher? Treatment participants disease, attention deficit/hyperactivity disorder, cerebral
also could assist in setting their own goals as part of a self- palsy, dementia, epilepsy, learning disabilities, schizophre-
management program. nia, and Tourettes syndrome. ABA based treatments have
The specific behavioral topographies that are the goals much to offer these under-studied populations, their
of change might differ across individuals (e.g., by popula- families, and the staff who serve them. The breadth of
tion, type and severity of disability, setting). Treatment applicability of ABA-based treatments across clinical and
goals also can have commonality across different clinical nonclinical populations can be attributed to the generality
populations (e.g., rate of performing academic behavior by of the underlying principles and processes of the science
children with attention deficit/hyperactivity disorder and of behavior.
cerebral palsy; reduction of physically aggressive behavior
by individuals with acquired head injury and encephalitis).
Thus, practitioners should focus on understanding the Treatment Procedures
personcontext relationship when formulating treatment
goals, and not solely on a persons diagnosis. As previously stated, ABA is the science of behavior and
Individuals with various neuropsychological disorders not a treatment per se. Treatments typically include a
have had treatment goals related to specific target beha- number of components that are applications of the sci-
viors, such as: (a) acquired brain injury (aggression, vo- ence of behavior; however, claims about the efficacy of
cational behavior); (b) attention deficit/hyperactivity individual components cannot be made independently of
disorder (off-task, academic behavior); (c) autism (com- the whole treatment package. A common component of
munication, social skills); (d) cerebral palsy (conversa- ABA treatment packages is differential reinforcement (i.e.,
tion, walking); (e) dementia, including Alzheimers reinforcing the desired response and withholding rein-
disease (incontinence, wandering); (f) encephalitis (sexu- forcement or using a behavior reduction tactic for unde-
al and violent behavior); (g) epilepsy (diet compliance, sired responses). For example, the pathological tongue
seizure awareness); and (h) Tourettes syndrome (vocal thrust during mealtime of a 10-year old boy with mental
and motor tics). retardation and spastic cerebral palsy was treated by pre-
senting food when his tongue was in and pushing the
tongue back into his mouth with a spoon when he thrust
Treatment Participants out his tongue and expelled food (Thompson, Iwata, &
Poynter, 1979). Amount of attention was differentially
ABA has had wide application of its treatment procedures provided to control breath-holding for a 7-year old girl
to various clinical and nonclinical populations, including with mental retardation and Cornelia-de-Lange syn-
those with neuropsychological disorders, as well as key drome (Kern, Mauk, Marder, & Mace, 1995) and the
232 A Applied Behavior Analysis
bizarre vocalizations of an adult with schizophrenia involves performing a functional behavioral assessment
(Wilder, Masuda, OConner, & Baham, 2001). Noncon- to form a hypothesis regarding the cause of challenging
tingent attention (i.e., increasing attention overall with- behavior. For example, treatment for a persons self-inju-
out respect to a specific target behavior) has been used rious behavior should be developed based on an under-
effectively for reducing disruptive vocalizations by elderly standing of why that persons problem behavior occurs in
dementia patients (Buchanan & Fisher, 2002); noncontin- a given context. The specific self-injurious behavior might
gent escape (i.e., allowing escape from an activity regard- be reinforced by receiving social attention or tangible
less of behavior) has been used for treating aggression items from others, escape or avoidance from aversive
during bathroom routines for the same population stimuli (e.g., task demands, irritation from eczema), au-
(Baker, Hanley, & Mathews, 2006). tomatic reinforcement (e.g., sensory self-stimulation), or
To increase the likelihood that response consequences a combination of these consequences. Functional behav-
serve a reinforcing function, stimulus preference assess- ioral assessment procedures include various descriptive,
ments are formally conducted. For example, the relative indirect (e.g., interview, rating scales), and experimental
preference of 33 food items was assessed to help a 15-year methods. Treatment procedures are then derived from the
old girl with uncontrolled epilepsy maintain compliance hypothesized function of the problem behavior indicated
to a ketogenic diet (Amari, Grace, & Fisher, 1995). Highly by the functional behavioral assessment. For example, the
preferred foods were used to reinforce compliance to the inappropriate sexual behavior of a 9-year old boy with
diet. Allowing individuals to make choices among activ- acquired brain injury was determined to be reinforced by
ities to be performed is another tactic to promote desired social attention (Fyffe, Kahng, Fittro, & Russell, 2004).
behavior (e.g., on-task by individuals with traumatic Treatment derived from that hypothesis consisted of func-
brain injury) and reduce challenging behavior. tional communication training and withholding attention
Several behavioral components are important when for the inappropriate behavior.
teaching new behavior. Often, shaping by successive An example of a comprehensive treatment package with
approximations is required to teach behavior. For exam- multiple components is a study that evaluated the efficacy
ple, a 5-year old child with mental retardation and spina of training children with autism to pass the state mandated
bifida was taught the use of crutches by breaking the task vision screening when they started school (Simer & Cuvo,
down into a 10-step sequence (Horner, 1971). Prompts or 2009). The package included components to teach the
cues usually are required to evoke an unlearned response. visual discriminations required (i.e., preference assess-
Examples include physical prompts (e.g., physically guid- ment, choice making, match-to-sample discrimination
ing a child to use crutches), modeling (e.g., video demon- discrete trial training, transfer of stimulus control, differ-
strating a typically developing child undergoing a medical ential reinforcement), and additional components to re-
exam), visual prompts (e.g., picture or written memory duce escape/avoidance behavior (i.e., desensitization,
aides for adults with Alzheimers disease or acquired brain extinction, reinforcement of alternative behavior).
injury), and verbal instructions (i.e., telling people what Behavioral principles and processes can explain the
to do). ABA research has also demonstrated procedures to behavior of social units at all levels (e.g., individual, fami-
transfer control of responding from the prompt to natural ly, staff, organizations), and ABA interventions can be
cues (e.g., from the memory aide that prompts going to applied to each of these social levels. Treatment programs
the next activity to the time on the clock). are individualized with respect to frequency and duration,
Environmental arrangements are also helpful in with a general goal to assist the social unit achieve as
controlling behavior. A visual cloth barrier on an unsafe much independence as feasible. The distinguishing fea-
restricted area reduced entry to that area by dementia ture of ABA treatments is that they are based on an
patients who wandered (Feliciano, Vore, LeBlanc, & analysis of the function of behavior in its environmental
Baker, 2004). Furniture was rearranged to be more con- context, derived from the science of behavior (i.e., pri-
ducive to conversation, and mealtime routines were rear- marily operant conditioning), and assessed for efficacy at
ranged to improve behavior in dementia patients (Melin the level of individuals and not groups as a whole.
& Gotestam, 1981). Classroom environmental arrange-
ments of various types are standard practice to control
the behavior of children with autism. Efficacy Information
There is a considerable literature base on ABA
approaches to reduce the challenging behavior of persons The efficacy of ABA derived treatments most typically has
with neuropsychological disorders. Best practice today been established by small n single subject experimental
Applied Behavior Analysis A 233
designs that control for threats to internal validity. Exter- client, preferably to supplement objective observation by
A
nal validity is established by multiple systematic replica- others.
tions that expand the generality of the findings. The body Behavior should be analyzed at its most microscopic
of ABA treatment efficacy research has increased signifi- level (e.g., trial or session data for an individual) initially
cantly over the decades since the 1960s. There are sub- before aggregating across more macroscopic levels (e.g.,
stantial treatment efficacy and effectiveness data for basic trial blocks, days, persons). Most often, data are plotted in
behavioral processes and their application to persons with a figure (e.g., line graph) and analyzed visually. For re-
neuropsychological disorders. Thousands of studies have search purposes, data are typically collected in the context
demonstrated the efficacy of interventions that teach or of the requirements of a single subject experimental de-
strengthen behavior. Efficacy has been demonstrated for sign. For routine clinical practice, it is advisable to have at
procedures that involve basic reinforcement processes, least baseline and treatment data.
token reinforcement, self-management, shaping, chain- Since ABA-based treatment programs should address
ing, prompting, transfer of stimulus control, and other socially valid goals for an individual, outcome measure-
behavioral processes and techniques. Likewise, numerous ment largely relies on a consideration of whether treat-
studies have provided evidence for the efficacy of behavior ment effects have attained those goals. Most often, that
reduction procedures based on processes such as extinc- determination can be made by visual inspection of the
tion, punishment, response cost, timeout, and differential data. Behavior analysts are interested in clinically mean-
reinforcement of various types. During the past 25 years, ingful (i.e., visually obvious on a figure) results and not
the efficacy of deriving treatment hypotheses for challeng- less impressive improvement, even if it is unlikely to have
ing behavior from a functional behavioral assessment occurred by chance.
has been clearly demonstrated by a large number of The social or clinical significance of outcomes can be
systematic replications. measured more formally either by social comparison or
Although there is a substantial body of ABA efficacy subjective evaluation techniques (Kazdin, 1977) as previ-
research that demonstrates the generality of behavioral ously described. For example, does the out-of-seat behav-
principles and processes across target behaviors, popu- ior of a child with hyperactivity after treatment compare
lations, and settings, the amount of efficacy research favorably to that of his classmates who serve as the social
differs across specific populations with neuropsycholog- comparison criterion? Does the teacher who serves as the
ical disorders. An ABA approach to intervention has subjective evaluation criterion rate the childs out-of-seat
been best practice for decades for individuals with in- behavior as acceptable after treatment?
tellectual disability, especially for those with more se- Since the goal of ABA research and treatment is to
vere disability. Since the 1960s beginning with Lovaas produce socially significant outcomes for individuals and
seminal work, efficacy of an ABA approach to treat not for samples from a population, the role of statistical
young children with autism has been demonstrated analysis as a decision making tool is rather limited. De-
and earned support by the US Surgeon General (1999) scriptive statistics are sometimes used to supplement vi-
and others. A recent meta-analysis of behavioral treat- sual analysis and social validation. Descriptive statistics
ments for attention-deficit/hyperactivity disorder sup- can serve as an aide by summarizing certain key charac-
ported the efficacy of ABA for that population (Fabiano teristics of the data set (e.g., measures of central tendency,
et al., 2009). variability, trend, effect size). When inferential statistics
are used, they are employed to make inferences from a
sample of an individuals behavior (i.e., data collected) to
Outcome Measurement the universe of that individuals behavior, and not to
population parameters.
The primary method of measuring treatment outcomes in Another outcome evaluative criterion is theoretical
ABA is direct and systematic observation of the objective- significance. Are treatment outcomes consistent with the
ly defined behavioral topography of interest (e.g., its fre- behavioral conceptualizations that underlie treatment se-
quency, duration, intensity). That measurement could be lection for the target behaviors? For example, is behavior
either continuous (i.e., each event) or sampled from the reduction treated by extinction (i.e., withdrawing rein-
individuals universe of behavior (e.g., by interval or time forcement from a previously reinforced response) consis-
sampling recording). Observers should be reliability tent with what we know about extinction as a basic
trained and observe and record independently of each behavioral process? Because of the idiographic nature of
other. Some ABA programs include self-observation by a ABA treatment, standardized instruments based on
234 A Applied Behavior Analysis
normative data for measuring treatment outcome are References and Readings
generally eschewed in favor of individualized measure-
ment as previously described. Nevertheless, individualized Amari, A., Grace, N. C., & Fisher, W. W. (1995). Achieving and main-
rating scales, logs, and standardized tests are sometimes taining compliance with the ketogenic diet. Journal of Applied Be-
havior Analysis, 28, 341342.
used as secondary measures.
Baer, D. M., Wolf, M. M., & Risley, T. R. (1968). Some current dimensions
of applied behavior analysis. Journal of Applied Behavior Analysis,
1, 9197.
Qualifications of Treatment Providers Baker, J. C., Hanley, G. P., & Mathews, M. R. (2006). Staff-administered
functional analysis and treatment of aggression by elder with de-
mentia. Journal of Applied Behavior Analysis, 39, 469474.
The principal credential for applied behavior analysts is Buchanan, J. A., & Fisher, J. E. (2002). Functional assessment and non-
national certification that is administered by The Behav- contingent reinforcement in the treatment of disruptive vocalization
ior Analyst Certification Board, a nonprofit corporation. in elderly dementia patients. Journal of Applied Behavior Analysis, 35,
There are two levels of professional certification. The 99103.
Cooper, J. O., Heron, T. E., & Heward, W. L. (2007). Applied behavior
Board Certified Behavior Analyst (BCBA): (a) holds a
analysis (2nd ed.). Upper Saddle River, NJ: Pearson.
masters or doctoral degree, (b) has had the required Fabiano, G. A., Pelham, W. E. Jr., Coles, E. K., Gnagy, E. M., Chronis-
number of coursework hours in certain core content Tuscano, A., & OConnor, B. (2009). A meta-analysis of behavioral
areas of behavior analysis (i.e., ethics, behavioral princi- treatments for attention-deficit/hyperactivity disorder. Clinical Psy-
ples, behavioral assessment and selecting interventions, chology Review, 29, 129140.
Feliciano, L., Vore, J., LeBlanc, L. A., & Baker, J. C. (2004). Decreasing
experimental evaluation, behavioral measurement, and
entry into a restricted area using a visual barrier. Journal of Applied
behavioral change procedures), (c) has had the required Behavior Analysis, 37, 107110.
number of supervised clinical training hours, and (d) has Friman, P. C. (2007). The fear factor: A functional perspective on anxiety.
passed a written test. The Board Certified Assistant In P. Sturmey (Ed.), Functional analysis in clinical treatment
Behavior Analyst (BCABA) holds a Baccalaureate degree, (pp. 335355). Burlington, MA: Elsevier.
Fyffe, C. E., Kahng, S. W., Fittro, E., & Russell, D. (2004). Functional
has passed a written test, and has had similar coursework
analysis and treatment of inappropriate sexual behavior. Journal of
and supervised clinical experience as the BCBA but to Applied Behavior Analysis, 37, 401404.
a lesser degree. BCBAs and BCABAs can hold their Hayes, S. C., Barnes-Holmes, D., & Roche, B. (2001). Relational frame
required academic degrees in any discipline as long as theory. New York: Plenum.
they meet the other requirements. Psychologists can Horner, R. D. (1971). Establishing use of crutches by a mentally
retarded spina bifida child. Journal of Applied Behavior Analysis, 4,
be certified in Behavioral Psychology, with a concentra-
183189.
tion in ABA by the American Board of Behavioral Kazdin, A. E. (1977). Assessing the clinical or applied importance of
Psychology, an affiliate of the American Board of Profes- behavior change through social validation. Behavior Modification,
sional Psychology. 1, 427451.
Kern, L., Mauk, J. E., Marder, T. J., & Mace, F. C. (1995). Functional
analysis and intervention for breath holding. Journal of Applied
Behavior Analysis, 28, 339340.
Cross References Melin, L., & Gotestam, K. G. (1981). The effects of rearranging
ward routines on communication and eating behaviors of
psychogeriatric patients. Journal of Applied Behavior Analysis, 14,
Behavior Management
4751.
Behavior Modification Novak, G., & Pelaez, M. (2004). Child and adolescent development:
Behavioral Analysis A behavioral systems approach. Thousand Oaks, CA: Sage.
Behavioral Assessment Sidman, M. (1994). Equivalence relations: A research story. Boston:
Behavioral Therapy Authors Cooperative.
Simer, N., & Cuvo, A. J. (2009). Training vision screening behavior to
Behaviorism
children with developmental disabilities. Research in Autism Spec-
Conners Comprehensive Behavior Rating Scales trum Disorders, 3, 409420.
Errorless Learning Skinner, B. F. (1938). The behavior of organisms. New York: Appleton-
Extinction Century-Crofts.
Inter Rater Reliability Skinner, B. F. (1948). Walden two. New York: Macmillan.
Skinner, B. F. (1953). Science and human behavior. New York: Free Press.
Learning
Skinner, B. F. (1957). Verbal behavior. New York: Appleton-Century-
Stimulus Bound Behavior Crofts.
Stimulus Control Skinner, B. F. (1968). The technology of teaching. New York: Appleton-
Stimulus Generalization Century-Crofts.
Apraxia A 235
Thompson, G. A. Jr., Iwata, B. A., & Poynter, H. (1979). Operant control deficits or weakness, discoordination, movement disor-
of pathological tongue thrust in spastic cerebral palsy. Journal of A
ders, and sensory loss.
Applied Behavior Analysis, 12, 325333.
U. S. Department of Health and Human Services. (1999). Mental health:
Assessment: Assessment for apraxia involves asking a
A report of the surgeon general. Rockville, MD: Department of Health patient to carry out pantomimes of movements (e.g.,
and Human Services, Substance Abuse and Mental Health Services show me how you would salute . . . brush your teeth
Administration, Center for Mental Health Services, National Insti- with a toothbrush. . . blow out a candle). If the response
tute of Mental Health.
in not correct, the examiner may evaluate the patients
Wilder, D. A., Masuda, A., OConnor, C., & Baham, M. (2001). Brief
functional analysis and treatment of bizarre vocalizations in adult
response to imitation of the movement or ability to
with schizophrenia. Journal of Applied Behavior Analysis, 34, 6568. produce the movement using actual objects or tools.
The patient may also be tested for recognition of skilled
movements produced by the examiner.
Classification of Apraxia: Apraxias may be differentiated
Applied Behavioral Analysis by the body elements involved in the impaired movement,
using the terms limb apraxia, oral or buccofacial apraxia,
Behavior Modification trunk or axial apraxia. These disorders have different
neuroanatomical underpinnings and may occur separate-
ly in different individuals. For instance, patients with oral
apraxia may demonstrate normal function on tests of
Apprehension Span limb praxis.
Apraxia has been subclassifed into ideational apraxia,
Span of Apprehension ideomotor apraxia, and limb-kinetic apraxia (Liepmann,
1900). Ideational apraxia is a loss of the conception of a
gesture or skilled movement. In this form, the patient
does not seem to know what to do, and the motor activity
Apraxia is not facilitated by the use of actual objects. Ideomotor
apraxia affects the implementation of the movement,
D OUGLAS I. K ATZ producing spatial and timing errors. The patient seems
Boston University School of Medicine to know what to do but cannot carry the movement out
Braintree, MA, USA properly. Limb-kinetic apraxia is the inability to make
finely graded, precise limb movements. It has been
difficult to separate this form of apraxia from motor
Definition dysfunction related to elemental motor disturbance, and
it remains controversial that this is actually an apraxic
The inability to correctly carry out a learned, skilled disorder of learned skilled movement.
motor act despite the preserved capability of the Neuropathological localization: Apraxias are almost al-
sensorimotor system to produce the intended movement. ways associated with lesions in the dominant hemisphere.
The left parietal lobe is most often implicated in limb
apraxia in right-handers. Portions of the frontal lobes,
Current Knowledge including the supplementary motor area, have been
implicated in some forms of apraxia. Lesions in and
Apraxia is thought to involve a loss of representations or around Brocas area are most often implicated in cases
the inability to adequately access representations of of oral apraxia. Lesions affecting transmission of informa-
learned movements and motor skills in the damaged tion between the cerebral hemispheres, including lesions
brain. This may lead to a loss of recall of the concept or of the corpus callosum, may lead to apraxia of just the left
configuration of the movement, or the inability to limbs because of disconnection of movement engrams in
transform or implement the representational knowledge the left hemisphere from motor control areas in the right
of the movement into a well-coordinated, properly con- hemisphere (callosal apraxia).
figured, and sequenced gesture. The diagnosis of apraxia Other disorders: A number of disorders have labels that
requires the exclusion of cognitive and sensorimotor include the term apraxia but have no relationship with
impairments that may affect the ability to carry out the apraxia according to the usual definition. These include:
motor skill, such as arousal, attention, intention, language Dressing apraxia difficulty orienting clothes to the body,
236 A Apraxia of Speech
often associated with left neglect, and usually occurring (e.g., aphemia, phonemic disintegration, cortical dysar-
with superior parietal right hemisphere lesions; construc- thria, dyspraxia of speech). Currently, there are no
tional apraxia difficulty drawing or copying pictures or acceptable synonyms in use. The descriptor, stroke in-
designs; gait apraxia difficulty initiating or maintaining duced (SI), has occasionally been used to specify AOS
a normal gait pattern as can be seen in normal pressure resulting from stroke (e.g., SI-AOS).
hydrocephalus; apraxia of gaze difficulty directing eye
movements as seen in Balints syndrome. Apraxia of speech
is a disorder that affects the sequencing of sounds in Short Description or Definition
words and syllables. It can be developmental or acquired
and people with this disorder have difficulty coordinating AOS is a neurologic, motoric disorder of speech produc-
articulatory motor activities necessary for speech. It is tion that is characterized by slowed rate of speech, diffi-
controversial whether this represents an apraxia consis- culties in sound production, and disrupted prosody. AOS
tent with the definitions described above, or a subtype is not a disorder of language, although it rarely occurs
of language or elemental motor disorders affecting without aphasia. Consequently, there is no impairment of
articulatory sequencing. comprehension or production of language in pure AOS.
gyrus), parietal lobe, and insula (see Wambaugh and Unlike the dysarthrias, AOS is not associated with problems
A
Shuster, 2008 for a review). with muscle tone, weakness, reflexes, or sensory processing.
Little objective evidence exists concerning the natural
course of AOS, including the factors affecting prognosis.
Duffy (2005) indicates that mutism associated with AOS Evaluation
rarely lasts beyond 2 weeks unless other speech, language,
or cognitive deficits are also present. Diagnosis of AOS requires that the primary symptoms of
Treatment can be expected to result in the improvement reduced rate of speech, distorted sound production, and
in symptoms of AOS, even when AOS is chronic. Treatment disrupted prosody be present. Behaviors such as difficul-
guidelines for AOS have been developed, which provide ties with speech initiation or articulatory groping (see
effectiveness ratings for different types of treatment based above) may also be observed, but should not be used
on the existing published evidence (see below; Wambaugh, alone for purposes of differential diagnosis (Wambaugh
Duffy, McNeil, Robin, and Rogers, 2006a, 2006b). et al., 2006a). Screening for AOS typically involves eli-
citing a variety of speech samples and determining the
presence or absence of AOS symptoms. For example,
Neuropsychology and Psychology Duffy (2005) provides a tool that may be used to evaluate
of AOS speech production across tasks such as sound, monosyllabic
word, multisyllabic word, and sentence repetition; repeated
AOS is a neurogenic, motoric speech disorder that is word productions; alternate and sequential motion rates,
characterized by reduced rate of speech, disrupted pro- reading aloud; and connected speech production. The
duction of speech sounds, and disordered prosody. These Apraxia Battery for Adults second edition (Dabul, 2000)
symptoms may be accompanied by behaviors such as also provides tasks that may be used for examining speech
articulatory groping (silent and/or audible), speech initi- production in adults with suspected AOS. However, the
ation difficulties, increasing number of sound errors with criteria for diagnosis of AOS provided by this test will not
increasing word length or phonetic complexity, awareness differentiate AOS from phonemic paraphasia.
of speech errors, and motoric perseverations. Its severity
ranges from a total inability to speak to negligible speech
disruptions (McNeil, Robin, and Schmidt, 2009; Wam- Treatment
baugh et al., 2006a).
AOS is thought to be caused by difficulties in the Behavioral treatment has been demonstrated to have
process of converting correctly selected and ordered positive outcomes for persons with AOS. An AOS prac-
sounds into previously learned movement information tice guidelines report revealed four general approaches to
necessary for the implementation of intended speech AOS treatment: (1) articulatory-kinematic treatments
movements. That is, it is assumed that sounds are cor- (techniques focused on improving articulation of
rectly selected and sequenced at a linguistic level of pro- sounds), (2) rate/rhythm control treatments (therapies
cessing. However, there is difficulty in accessing stored involving manipulating rate of speech production or
movement plans/programs needed to articulate those imposing an external rhythm on speech), (3) alterna-
chosen sounds. These difficulties cause disruptions in tive/augmentative communication approaches (therapies
the selection, positioning, and movement timing of the involving training the use of methods/devices for supple-
articulators (e.g., tongue, lips, etc.). Consequently, sound menting or replacing speech), and (4) intersystemic fa-
productions may be inaccurate, transitions between cilitation/reorganization treatments (therapies utilizing a
sounds may be disrupted, and prosody may be abnormal. relatively intact system/modality such as singing or ges-
Sound durations as well as the intervals between sounds, turing to facilitate speech production) (Wambaugh et al.,
syllables, and words tend to be prolonged. Sound produc- 2006b). On the basis of objective evaluation of the existing
tion errors are relatively consistent in location and invari- evidence, the AOS guideline developers determined that
able in type across repeated productions (McNeil, Robin, articulatory-kinematic approaches were probably
and Schmidt, 2009). effective rate/rhythm control approaches and intersyste-
AOS typically occurs with aphasia and rarely occurs mic approaches were possibly effective; and AAC
without it. The symptoms of AOS, however, are not approaches had insufficient support to warrant a rating.
attributable to disruptions in language. AOS may also There are currently no published reports of restorative
occur with another motor speech disorder, dysarthria. neurological treatments applied specifically to AOS
238 A Apraxic Agraphia
(e.g., neuropharmaceutical treatment, electrical cortical speech used in addition to words to convey specific mean-
stimulation, transcranial magnetic stimulation), but ing and emotional information (Leon & Rodriguez, 2008;
such reports are likely to be available in the future. Wymer, Lindman, & Booksh, 2002). Aprosodia is tradi-
tionally characterized as linguistic or affective (Wymer
Cross References et al., 2002). Linguistic prosody aids meaning, e.g., convict
vs. convict or the dog and the cat in the cage are mine vs. the
Aphasia dog, and the cat in the cage, are mine allow unambiguous
Dysarthria discrimination of the semantic target. Affective prosody
Phonemic Paraphasia conveys attitude, e.g., incredulity, sadness, or anger, e.g.,
depending on the prosodic intonation, oh, yeah, Im just
great may be a sincere expression of a good feeling, or an
References and Readings equally sincere communication that the speaker is angry
or frustrated. Linguistic aprosodia is associated with both
Dabul, B. (2000). Apraxia battery for adults (2nd ed.). Austin, TX: Pro-ed.
Duffy, J. R. (2005). Motor speech disorders: Substrates, differential
left and right hemisphere lesions; affective aprosodia is
diagnosis, and management (2nd ed.). St. Louis, MO: Elsevier Mosby. more consistently associated with lesions of the right hemi-
McNeil, M. (2002). Apraxia of speech: From concept to clinic. Seminars sphere (Baum & Pell, 1999; Pell, 2006; Ross & Monnot,
in Speech and Language, 23(4), 221222. 2008). Additionally, the basal ganglia appear to play a key
McNeil, M. R., Robin, D. A., & Schmidt, R. A. (2009). Apraxia of speech:
role in processing and further distributing the meaning
Definition and differential diagnosis. In M. R. McNeil (Ed.), Clinical
management of sensorimotor speech disorders (2nd ed., pp. 249268).
associated with the prosodic characteristics (Cancelliere &
New York: Thieme. Kertesz, 1990; Pell & Leonard, 2003).
Wambaugh, J. L., & Shuster, L. I. (2008). The nature and management of Clinically, aprosodia is most often considered in terms
neuromotor speech disorders accompanying aphasia. In R. Chapey of whether receptive, expressive, or both aspects of pro-
(Ed.), Language intervention strategies in aphasia and related neuro-
sodic ability are diminished in an individual. However,
genic communication disorders (5th ed., pp. 10091042).
Philadelphia, PA: Lippincott Williams & Wilkins.
Ross and various colleagues have proposed a categoriza-
Wambaugh, J. L., Duffy, J. R., McNeil, M. R., Robin, D. A., & Rogers, M. tion system for the aprosodias, which is similar to that
(2006a). Treatment guidelines for acquired apraxia of speech: A used to categorize aphasia types. The system is based on a
synthesis and evaluation of the evidence. Journal of Medical Speech combination of deficit profile and site-of-lesion informa-
Language Pathology, 14(2), xvxxxiii.
tion: motor (in the area of the frontal operculum), sensory
Wambaugh, J. L., Duffy, J. R., McNeil, M. R., Robin, D. A., & Rogers, M.
(2006b). Treatment guidelines for acquired apraxia of speech: Treat-
(posterior temporal operculum), conduction (arcuate fas-
ment descriptions and recommendations. Journal of Medical Speech ciculus), transcortical (anterior or posterior watershed),
Language Pathology, 14(2), xxxvixvii. or global deficits (Ross, 1981, 2000; Ross & Monnot,
2008). For example, an individual with motor aprosodia
might not express affective or emotional prosody when
speaking; one with sensory aprosodia might not recognize
Apraxic Agraphia the affective meaning of prosodic signals in anothers
speech. These nondominant hemisphere anatomic corre-
Agraphia lates of prododic deficits do have modest empirical support
(see Ross & Monnot, 2008 for a summary of related inves-
tigations). Ross motor aprosodia should not be confused
with prosodic production impairments that arise from def-
Aprosodia icits in speech production due to dysarthria (Duffy, 2005).
Assessment of aprosodia is generally accomplished
K ATE K RIVAL through careful observation; however, the Florida Affect
Kent State University Battery (Bowers, Blonder, & Heilman, 1998) and the
Kent, OH, USA Aprosodia Battery (Ross, Thompson, & Yenkosky, 1997)
may be useful additions to standard testing regimes. Man-
agement of aprosodic impairments has received little at-
Definition tention in the literature; however, emerging evidence
suggests that behavioral therapies may have some effect
Aprosodia is a deficit in comprehending or expressing (Bornhofen & MacDonald, 2008; Leon et al., 2005; Rosen-
prosody, i.e., variations in pitch, loudness, or rhythm of bek et al., 2004). Future work that will focus on the design
Arachnoid Cyst A 239
and implementation of treatment for both expressive and Ross, E. D., & Monnot, M. (2008). Neurology of affective prosody and its
functionalanatomic organization in right hemisphere. Brain and A
receptive aprosodia is anticipated.
Language, 104(1), 5174.
Ross, E. D., Thompson, R. D., & Yenkosky, J. P. (1997). Lateralization of
affective prosody in brain and the callosal integration of hemispheric
Cross References language functions. Brain and Language, 56, 2754.
Wymer, J. H., Lindman, L. S., & Booksh, R. L. (2002). A neuropsycholog-
ical perspective of aprosody: Features, function, assessment and
Apraxia of Speech
treatment. Applied Neuropsychology, 9(1), 3747.
Dysarthria
Prosody
Arachnoid Cyst. Table 1 Frequency and distribution of intracranial arachnoid cysts (Rengachary, Watanabe, & Brackett, 1978)
Arachnoid Cyst. Figure 1 Classification of middle fossa arachnoid cysts. (a) bilateral type I middle fossa arachnoid cyst
demonstrating small size, biconvex appearance, and absence of mass effect; (b) type II middle fossa arachnoid cysts are
rectangular; (c) type III middle fossa arachnoid cyst are large and often have significant mass effect
Oberbauer, 1999). Midline posterior fossa arachnoid cysts structural anomalies such as agenesis of the corpus callo-
must not be confused with the DandyWalker complex. sum (occurring in 68% of patients), and a post fossa
The DandyWalker complex describes hypoplasia or agen- arachnoid cyst- like structure that is not a true arachnoid
esis of the cerebellar vermis, associated hydrocephalus, cyst (Wilkinson & Winston, 2008).
Arachnoid Cyst A 241
Arachnoid Cyst. Figure 2 Middle fossa arachnoid cyst both before (a) and after (b) surgery. Note the significant brain
re-expansion, an observation used to refute the suggestion of a congenital hypoplastic origin
242 A Arachnoid Cyst
Arachnoid Cyst. Figure 3 Foetal magnetic resonance image Arachnoid Cyst. Figure 4 Quadrigeminal Plate arachnoid cyst
demonstrating an interemispheric arachnoid cyst
Arachnoid Cyst. Figure 6 Appearance before (a) and during (b) endoscopic fenestration of a middle fossa arachnoid cyst
of 23 times/s (Hagebeuk, Kloet, Grotenhuis, & Peeters, suggesting that arachnoid cysts may suppress cognitive
2005). Cerebral convexity cysts often present with cranial and cortical function (Baroey Raeder, Helland, Hugdahl,
deformity and asymmetry alone. Quadrigeminal Plate & Wester, 2005; Wester & Hugdahl, 2003). However,
Cysts (Fig. 4) can present with obstructive hydrocephalus larger series do not support significant clinical improve-
or upward gaze palsies. Spinal arachnoid cysts usually ment after surgery in patients with severe developmental
present with myelopathy or nerve root compression (Di and behavior problems (Arai et al.; Levy, Wang, Aryan,
Rocco, 1990). Yoo, & Meltzer, 2003). In patients with developmental
delay, behavioral disorders, and an arachnoid cyst, or in
very young patients with large cysts, preoperative neu-
Neuropsychology and Psychology of ropsycological testing, EEG, and/or SPECT may have a
Arachnoid Cysts role in supporting surgical intervention (Wilkinson &
Winston).
Large arachnoid cysts may present with failure to reach
developmental milestones and psychomotor delay. Cases
of dementia in adults have been reported (Harsh, Evaluation
Edwards, & Wilson, 1986). Cerebellar signs may be mis-
diagnosed as motor delay. MFAC and convexity cysts can Diagnosis and follow-up are based on clinical evaluation
be associated with cognitive problems, developmental and imaging. Arachnoid cysts appear as cavities filled with
delay, behavioral disorders, and memory and attention fluid with the same characteristics as CSF, surrounded
deficits in up to 7.8% of patients (Arai, Sato, Wachi, by a thin wall that is not calcified and does not enhance
Okuda, & Takeda, 1996). Symptoms can be potentiated after contrast. Magnetic resonance imaging (MRI) is the
with antiepileptic drugs and sedatives. The relationship diagnostic tool of choice because of image resolution for the
between arachnoid cysts and developmental and behav- cyst and surrounding anatomy (Wilkinson & Winston,
ior problems is poorly understood. Functional magnetic 2008).
resonance imaging demonstrates no alterations in Blood
Oxygen Level Dependant responses (BOLD) yet Single
Photon Emission Computed Tomography (SPECT) Treatment
images can demonstrate reduced cerebral blood flow
and glucose metabolism even in the contralateral hemi- Indications
sphere (Wilkinson & Winston, 2008). Pre- and postop- Arachnoid cysts causing a focal neurological deficit, raised
erative cognitive testing, in small series, demonstrate ICP, or enlarging should be treated. The management of
improved performance in specific tasks after treatment, asymptomatic patients or patients with functional
244 A Arachnoid Cyst
symptoms, such as seizures and developmental delay, is Gosalakkal, J. A. (2002). Intracranial arachnoid cysts in children: A
review of pathogenesis, clinical features, and management. Pediatric
controversial. Asymptomatic cysts without mass effect
Neurology, 26(2), 9398.
may have a lower risk of bleeding if treated (Wilkinson Hagebeuk, E. E., Kloet, A., Grotenhuis, J. A., & Peeters, E. A. (2005).
& Winston, 2008; Parsch et al., 1997). Seizure control may Bobble-head doll syndrome successfully treated with an endoscopic
be improved, but a causal link is not always apparent. ventriculocystocisternostomy. Case report and review of the
Currently, treatment in patients with severe developmental literature. Journal of Neurosurgery, 103(3 Suppl), 253259.
Harsh, G. R., Edwards, M. S. B., & Wilson, C. B. (1986). Intracranial
delay does not appear to confer a significant functional
arachnoyd cysts in children. Journal of Neurosurgery, 64, 835842.
improvement (Arai et al., 1996; Levy et al., 2003). Jamjoom, Z. A., Okamoto, E., Jamjoom, A. H., al-Hajery, O., &
Abu-Melha, A. (1995). Bilateral arachnoid cysts in the Sylvian region
Surgical Options in female siblings with glutaric aciduria type I. Journal of Neurosur-
Three surgical options exist. These include shunt inser- gery, 82, 10781081.
Karabatsou, K., Hayhurst, C., Buxton, N., OBrien, D. F., & Mallucci, C. L.
tion, open exploration, or endoscopic fenestration (Lena
(2007). Endoscopic management of arachnoid cysts: An advancing
et al., 1996, Kaufman & Park, 2000). Commonly, a cysto- technique. Journal of Neurosurgery, 106(6 Suppl Pediatrics),
peritoneal shunt is placed, although revision rates due to 455462.
infection or system failure remain at 2040% at 8 years Kaufman, B. A., & Park, T. S. (2000). Treatment of arachnoid cysts. In
(Arai et al., 1996; Oberbauer et al., 1992). Cyst fenestra- D. G. McLane (Ed.), Pediatric neurosurgery Philadelphia, PA: WB
Saunders.
tion consists of opening a window in both the superficial
Kutlay, M., Colak, A., Demircan, N., & Akin, O. N. (1998). Iatrogenic
and deep walls of the cyst to allow communication be- arachnoid cyst with distinct clinical picture as a result of bone defect
tween the cyst and the subarachnoid space (Figs. 5 and 6). in the floor of the middle cranial fossa: Case report. Neurosurgery,
Both microsurgical and endoscopic approaches demon- 43(5), 12151218.
strate improvement in up to 90% of patients (Karabatsou Lena, G., Erdincler, P., Van Calenberg, F., Genitori, L., & Choux, M.
(1996). Arachnoid cysts of the middle cranial fossa in children. A
et al., 2007; Levy et al., 2003; Spacca et al., 2009). Endo-
review of 75 cases, 47 of which have been operated in a comparative
scopic fenestration is less invasive, and series suggest study between membranectomy with opening of cisterns and
reduced morbidity (Karabatsou et al., 2003; Spacca cystoperitoneal shunt. Neurochirurgie, 42(1), 2934.
et al., 2009). Treatment of an associated subdural hemor- Levy, M. L., Wang, M., Aryan, H. E., Yoo, K., & Meltzer, H. (2003).
rhage or hygroma may lead to resolution of the arachnoid Microsurgical keyhole approach for middle fossa arachnoid cyst
fenestration. Neurosurgery, 53(5), 11381145.
cyst (Parsch et al., 1997).
Miyajima, M., Arai, H., Okuda, O., Hishii, M., Nakanishi, H., & Sato, K.
(2000). Possible origin of suprasellar arachnoid cysts: Neuroimaging
and neurosurgical observations in nine cases. Journal of Neurosurgery,
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M ARTIN R. G RAF
Virginia Commonwealth University Medical Center
Richmond, VA, USA
Arachnoid Mater
Meninges Definition
Aricept
Side Effects
Donepezil
Serious
Aripirozole Physicians Desk Reference (62nd ed.). (2007). Montvale, NJ: Thomson
PDR.
Stahl, S. M. (2007). Essential psychopharmacology: The prescribers guide
(2nd ed.). New York, NY: Cambridge University Press.
Brand Name
demonstration by Moruzzi and Magoun (1949) shows perform cognitive challenges that are stressful or that
that the brain-stem reticular formation plays a key role require rapid response for adequate performance tend to
in generating brain activation as evident by electroen- result in increased fast wave EEG activity as well as
cephalography. This spurred subsequent neurophysiolog- autonomic nervous system responses such as increased
ical inquiry into the factors underlying arousal and their heart rate and respiration, metabolic activity, and a diver-
mediation of higher order cognitive functions. Early sion of blood from the gastrointestinal system to skeletal
efforts to understand the actions of neurotransmitters in muscle. These responses are associated with a readiness to
the brain were closely linked to the concept of arousal, respond, a key element of attention, the specific nature
based on the effects of norepinephrine on the sympathetic of this attention response depends on whether the task
nervous system response and wakefulness. Pribram and demands involve passive vigilance or more effortful
McGuinness (1975) posited dissociations between arousal, directed attention.
activation, and effort in the control of attention, an There is now strong evidence that multiple neural
important theoretical neuropsychological work that tied systems are involved in the maintenance, control, and
these processes to underlying brain mechanisms. Heilman allocation of arousal throughout the cortex. At least four
and Valensteins (1979) attention-arousal hypothesis neurotransmitter systems (acetylcholine, norepinephrine,
proposed that the arousal associated with reticular dopamine, and serotonin) mediate the energetic state of
activation interacted with a number of distinct cortical the brain, though a variety of peptides influence the
and subcortical systems involved in the control of neural response across specific brain systems. While hier-
attention, and that unilateral brain lesions across these archically the arousal originates in the brainstem, the
brain systems occur in patients with hemineglect nuclei of the hypothalamus play a critical role in the
syndrome. specificity of arousal relative to specific appetitive beha-
viors. For example, while damage to the reticular system
of the brain stem often results in coma, hypothalamic
Current Knowledge nuclei such as the suprachiasmatic nucleus exert control
over wakefulness, by maintaining circadian rhythm.
The concept of arousal has evolved substantially from its Furthermore, the arousal associated with drives such
original conception. Some neuroscientists argue that the as eating and sexual response are directly governed
concept has outlived its usefulness because the term by hypothalamic functions, with higher level limbic
arousal is used to refer to a broad range of different (e.g., amygdala) and cortical control.
behavioral and physiological phenomena with very The most obvious clinical manifestations of disor-
different underlying mechanisms. This has led to over- dered arousal occur in conjunction with delirium. How-
generalization of the construct. Yet, arousal continues to ever, factors that influence the level of arousal have direct
have an important role in neuropsychological theories. effects on performance, as described at the turn of the last
There is compelling evidence that brain stem and subcor- century in the YerkesDodson law. There are data from a
tical activity influences cortical activity and also auto- wide range of cognitive, behavioral, and neuropsycholo-
nomic response. At a behavioral and phenomenological gical studies demonstrating supporting the principle that
level, arousal provides a construct that links the more task performance varies as a function of arousal, with
primitive bioenergetic responses of the brain with higher optimal performance occurring at some intermediate level.
cortical functions. This is most obvious when considering Pathological states that cause lethargy usually reduce
levels of consciousness that can range from deep sleep attentional performance. Similarly, drugs, anxiety, or other
or coma to normal wakefulness to states of extreme factors that lead to excessive arousal also have detrimental
excitement, hyperactivity, or agitation. Altered arousal is consequences on performance. Disturbances of arousal are
a key feature of delirium that can occur due to transient commonly associated with both intoxication as well as
disruptions in brain function due to metabolic or drug withdrawal from drugs like alcohol and barbiturates,
influences. electrolyte imbalances, trauma to brain from closed
Stimulation of the sympathetic nervous system and an head injury, and various neurological disorders including
increase in the secretion of epinephrine and norepineph- encephalitis, tumors, advanced Alzheimers disease, and
rine increase the brain activity on EEG, along with stroke. Conditions that affect the brain stem, hypothalamus,
behavioral response toward greater wakefulness, where thalamus, limbic areas (e.g., amygdala), and frontal and
drugs like barbiturates and alcohol have the opposite temporal lobes are most likely to alter arousal, and there-
effect. The task that requires intense focused attention to by affect attention too.
Arteriovenous Malformation (AVM) A 249
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Ropper, A. H., Brown, R. H., Adams, R. D., & Victor, M. (2005). Adams &
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Victors principles of neurology. New York: McGraw-Hill. Articulation Disorder
Stein, B. M., & Wolpertson, S. M. (1980). Arteriovenous malformations Ataxia
of the brain. I: Current concepts and treatments. Archives of Neurol- Dysarthria
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Dystonia
Warlow, C. (2001). Stroke, transient ischemic attacks, and intracranial
venous thrombosis. In M. Donaghy (Ed.), Brains diseases of the
Phoneme
nervous system (pp. 775896). New York: Oxford University Press. Phonics
Websters new explorer medical dictionary (New Ed.). (2006). Springfield, Speech-Language Pathology
MA: Merriam-Webster.
Zhao, J., Wang, S., Li, J., Qi, W., Sui, D., & Zhao, Y. (2005). Clinical
characteristics and surgical results of patients with cerebral arterio-
venous malformations. Surgical Neurology, 63, 156161. References and Reading
Hulit, L. M., & Howard, M. R. (2005). Born to talk: an introduction to
speech and language development (4th ed.). Boston: Pearson A & B.
Plante, E., & Beeson, P. M. (2008). Communication and communication
Arteriovenous Malformations disorders: a clinical introduction (3rd ed.). Boston: Pearson A&B.
Vascular Malformations
Articulation Disorders
Arteritis PAMELA G. G ARN -N UNN
University of Akron
Vasculitis Akron, OH, USA
English as a second language are not considered to have an Articulation Disorders. Table 1
A
articulation disorder.
By age 3: p m h w b (emerging between ages 1 and 3)
By age k g d t f y (girls only) (emerging between ages
Categorization 3 : 2 and 3 )
By age 4: y (boys)
An articulation disorder is a type of speech sound disor- By age 5: s-blends (emerging between ages 3 and 5)
der. It is associated with a motoric inability to produce a By age v
speech sound or sounds (rather than a failure to acquire 5 :
the speech sound rules of a particular language) by the By age 6: sh ch j (girls)
expected normative age. ( Phonological Disorder.) Ar-
By age 7: sh ch j (boys) th (as in that)
ticulation and phonological disorders can co-occur.
By age 8: r s ng l z th (as in thumb) zh (as in measure)
Evaluation
ASEBA
Evaluation of articulation disorders is designed to
determine: Child Behavior Checklist
1. Existence of a problem
2. Nature of the problem (sounds in error, patterns
intelligibility)
3. Possible etiology(ies) of the problem, e.g., structural
ASHA-FACS
problem or neurological disorder
American Speech-Language-Hearing Association Func-
4. Probable course of treatment
tional Assessment of Communication Skills for Adults
5. Prognosis
To meet these goals, the following components should be
included in an evaluation for speech sound disorders:
1. Case history
2. Hearing screening
Ashworth Spasticity Scale (and
3. Oral mechanism evaluation Modified Version)
4. Phonemic sound-by-position tests
5. Language testing K ARI D UNNING
6. Other tests as appropriate University of Cincinnati
Cincinnati, OH, USA
Treatment
Synonyms
For patients with simple articulation disorders, a tradi-
tional, phonetic approach can be successful. Phonolog- AS; MAS
ical Disorders and Articulation Disorders for more
information on treatment.
Description
Cross References The Ashworth Scale (AS) and Modified Ashworth Scale
(MAS) measure spasticity. During the administration of
Apraxia
both AS (Ashworth, 1964) and MAS (Bohannon & Smith,
Articulation Disorder
1987), the examiner passively moves the joint being tested
Dysarthria
and rates the perceived level of resistance in the muscle
Phonological Disorder
groups opposing the movement. Both scales are single-
Phonology
item measures ranging from 0 to 4, where 0 indicates no
increase in muscle tone and 4 indicates that the affected
References and Readings part is rigid in flexion or extension. The AS is considered
an ordinal scale, whereas the MAS is considered a nominal
American Speech-Language-Hearing Association. (2007). Speech sound scale due to ambiguity created by the addition of the 1
disorders: Articulation and phonological disorders, from www.asha. grade between 1 and 2 (Pandyan, Johnson, Price, Curless,
org/public/speech/disorders/SpeechSoundDisorders. Barnes, & Rodgers, 1999).
American Speech-Language-Hearing Association. (2008). Incidence and
prevalence of communication disorders and hearing loss in children
(2008 Ed.), from www.asha.org/members/research/reports/children.
Historical Background
this addition may have decreased the reliability of the References and Readings A
MAS for heavier limbs (see below) (Ansari, Naghdi, Arab,
& Jalaie, 2008; Pandyan et al., 1999; Platz, Eickhof, Ansari, N. N., Naghdi, S., Arab, T. K., & Jalaie, S. (2008). The interrater
Nuyens, & Vuadens, 2005). and intrarater reliability of the Modified Ashworth Scale in the
assessment of muscle spasticity: Limb and muscle group effect.
NeuroRehabilitation, 23(3), 231237.
Psychometric Data Ashworth, B. (1964). Preliminary trial of carisoprodol in multiple sclero-
sis. Practitioner, 192, 540542.
Bohannon, R. W., & Smith, M. B. (1987). Interrater reliability of a
Inter- and intra-rater reliability of the AS and MAS show
modified Ashworth scale of muscle spasticity. Physical Therapy,
wide variation (Pandyan et al., 1999; Platz et al., 2005), 67(2), 206207.
which cannot be attributed to any one factor (Platz et al., Fleuren, J. F., Voerman, G. E., Erren-Wolters, C. V., et al. (2009). Stop
2005), although some evidence suggests that the inter- using the Ashworth Scale for the assessment of spasticity. Journal of
rater reliability of the MAS is lower for heavier limbs Neurology, Neurosurgery, and Psychiatry, 81(2), 4652.
Lance, J. W. (1980). Symposium synopsis. In R. G. Feldman, R. R. Young,
(Ansari et al., 2008; Pandyan et al., 1999; Platz et al.,
& W. P. Koella (Eds.), Spasticity: Disordered motor control (pp.
2005). Both scales have demonstrated responsiveness to 485494). Chicago, IL: Year Book Medical Publishers.
treatment (Platz et al., 2005). Pandyan, A. D., Gregoric, M., Barnes, M. P., et al. (2005). Spasticity:
Spasticity is characterized by an involuntary muscle Clinical perceptions, neurological realities and meaningful measure-
activity (Pandyan et al., 2005) and has been traditionally ment. Disability and Rehabilitation, 27(12), 26.
Pandyan, A., Johnson, G., Price, C., Curless, R., Barnes, M., & Rodgers, H.
defined as a velocity-dependent increase in muscle tone
(1999). A review of the properties and limitations of the Ashworth
due to a hyperactive stretch reflex (Lance, 1980). The and modified Ashworth Scales as measures of spasticity. Clinical
construct validity of the AS and MAS as spasticity assess- Rehabilitation, 13(5), 373383.
ments is inadequate because they do not address velocity Platz, T., Eickhof, C., Nuyens, G., & Vuadens, P. (2005). Clinical scales for
dependence. Rather, these scales measure passive resis- the assessment of spasticity, associated phenomena, and function:
A systematic review of the literature. Disability and Rehabilitation,
tance to movement (hypertonia), which is influenced by
27(12), 718.
spasticity but also altered by biomechanical factors unre-
lated to involuntary muscle activation (Fleuren, Voerman,
& Erren-Wolters, 2009; Pandyan et al., 1999; Platz et al.,
2005). Thus, AS and MAS scores are only moderately ASIA (American Spinal Injury
associated with reflexes (Platz et al., 2005) and electro-
myographic assessments (Fleuren et al., 2009; Pandyan Association) Exam
et al., 1999; Platz et al., 2005) and more strongly asso-
ciated with objective measures of resistance (Fleuren et al., ASIA Impairment Scale
2009; Pandyan et al., 1999; Platz et al., 2005).
administered guide to document neurological function addition, the ASIA Impairment Scale (AIS) grade classi-
after spinal cord injury (SCI) and is intended to be a fication, an indicator of injury completeness similar to
standard for measuring neurological outcomes in both the Frankel scale, is assigned based on this information.
clinical and research settings. Briefly, these standards AIS A denotes a complete injury with no sensory or
utilize a two-step process consisting of a specific neuro- motor function below the level of injury. Incomplete
logical examination followed by a classification proce- injuries are graded as AIS B if there is sensory but no
dure based on the results of the exam. The systematic motor function below the injury level, AIS C if there is
neurological examination assesses sensory and motor some motor sparing, AIS D for substantial motor
function of each spinal segmental level. Sensation of sparing, and AIS E for normal neurological examination
light touch and pinprick (PP) stimuli is scored as 0 for (Fig. 1).
absent, 1 for impaired, and 2 for normal. Motor func-
tion is scored on a scale of 0 for total paralysis to 5 for
normal strength. All 28 dermatomes are tested bilaterally Historical Background
for sensory function, and ten key muscles are tested
bilaterally for motor function, yielding sensory/light ISNCSCI has been used extensively in clinical practice
touch (LT) and sensory/pinprick (PP) summed scores and research since 1982. The standards and
ranging from 0 to 112 and motor summed scores rang- accompanying reference manual have undergone se-
ing from 0 to 100. The neurological level is assigned as quential revisions, most recently in 2000 and 2003,
the lowest level with normal neurological function. In respectively.
Comments:
REV 03/06
ASIA Impairment Scale. Figure 1 International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI)
Asomatognosia A 257
Psychometric Data A
Asomatognosia
Published studies have found total motor score ICCs from
0.98 to 0.99 for intra-rater reliability and 0.97 for inter- J OHN E. M ENDOZA
rater reliability. Total sensory scores intra-rater reliability Tulane University Medical Center
has ranged from 0.76 to 0.98, and 0.88 to 0.96 for inter- New Orleans, LA, USA
rater. One study reported agreement on individual
muscles with Kappas ranging from 0.3 to 0.89 for each
myotome, 0.02 to 0.83 for dermatome assessment Synonyms
using pinprick, and 0.17 to 1 when assessed using light
touch. Disturbance of body schema
Sensorimotor Assessment
Spinal Cord Injury
Current Knowledge
Hecaen, H., & Albert, M. L. (1978). Human neuropsychology (pp. 303 Categorization
330). New York: Wiley.
Heilman, K. M., Watson, R. T., & Valenstein, E. (2003). Neglect and Aspergers disorder is currently classified in the DSM-IV
related disorders. In K. Heilman & E. Valenstein (Eds.), Clinical
as one of five separate pervasive developmental disorders
neuropsychology (pp. 296346). New York: Oxford University Press.
Kortte, K. B., & Wegener, S. T. (2004). Denial of illness in medical (which also include autistic disorder, Retts disorder,
rehabilitation populations: Theory, research and definitions. Reha- childhood disintegrative disorder, and pervasive develop-
bilitation Psychology, 49, 187199. mental disorder NOS). Aspergers disorder is a fairly
Prigatano, G. P., & Schacter, D. L. (1991). Awareness of deficit after brain recent addition to the DSM, first appearing only in
injury: Clinical and theoretical issues. New York: Oxford University
the latest version, the DSM-IV (1994). According to the
Press.
DSM-IV, to meet criteria for Aspergers disorder, an
individual must demonstrate impairment in social
interaction (exhibiting at least two out of four possible
symptoms) and restricted and repetitive patterns of beha-
viors or interests (exhibiting at least one out of four
Asperger Syndrome possible symptoms). In addition, an individual must not
have a history of developmental delays in language,
Aspergers Disorder cognition, or adaptive functioning. Of note, the criteria
Aspergers Disorder A 259
for Aspergers disorder are identical to those for autistic a result, Wing (1981) published an influential paper
A
disorder (i.e., autism) in the areas of social impairment reintroducing Aspergers original ideas and arguing for
and restricted and repetitive behavior. However, autistic broadening the definition of autism to include Aspergers
disorder requires an additional criterion of impairment in disorder on the autism continuum. Eventually, a separate
communication (i.e., delays in language development, diagnosis of Aspergers disorder was added to the fourth
impairment in conversation, stereotyped language, or edition of the DSM (1994). Since that time, debate has
lack of pretend play). Additionally, there is not a require- continued as to whether or not Aspergers disorder should
ment in the criteria for autistic disorder that cognitive, remain a separate diagnosis from autism. The prevailing
language, and adaptive development fall within the current view is that Aspergers disorder and autism are
normal range in childhood (as is the case in Aspergers not distinctly different, and that Aspergers disorder
disorder) (American Psychiatric Association, 1994). may simply represent the milder end of the autism
spectrum. As a result, Aspergers disorder is often used
synonymously with the term high-functioning autism
Epidemiology (which typically refers to individuals meeting criteria for
Autistic Disorder whose IQ levels are above 70).
Prevalence estimates have varied widely from 0.3/1,000 With regard to developmental course, Aspergers
to 6/1,000 (see Mattila et al., 2007 for review). Based on a disorder is generally diagnosed much later than autistic
review of the literature, Fombonne (2003, 2005) esti- disorder, with an average age of diagnosis being 11 years
mated the prevalence rate for Aspergers disorder to be (possibly due to the lack of early developmental delays).
approximately 2/10,000. Such wide variations in prevalence It follows a continuous course throughout the lifespan,
rates are likely due to differences in diagnostic procedures although for some individuals symptoms remit as a result
and operational definitions used in each study. In fact, of early intervention (see Frith, 2004 for review). Research
recent rates from the same study varied from 1.6/1,000 into prognostic factors and outcome in Aspergers
to 2.9/1,000 depending on the specific criteria used for disorder is sparse, particularly since it has only been
diagnosis (Mattila et al., 2007). In terms of sex differences, recognized as an official diagnosis for little over a decade;
males are overrepresented in Aspergers disorders, with however, IQ and language ability have been found to be
an estimated sex ratio of 4:1 (see Schopler, Mesibov, & strong predictors of outcomes in autism spectrum
Kunce, 1998 for review). disorders in general.
Social impairment (2 or more) Restricted and repetitive behavior (1 or more) Lack of delays in
1. Impaired nonverbal behavior 1. Abnormal and intense preoccupation with Language
stereotyped or restricted interest
2. Impaired peer relationships 2. Inflexible and nonfunctional routines or rituals Cognitive development
3. Lack of seeking to share enjoyment, 3. Stereotyped and repetitive motor mannerisms Self-help skills
interests, or achievements
4. Lack of social or emotional reciprocity 4. Preoccupation with parts of objects Adaptive behavior
(other than social)
Curiosity about the
environment
Source: From Diagnostic and Statistical Manual of Mental Disorders, fourth edition, by American Psychiatric Association, 1994, Washington, DC
Aspergers disorder is also associated with cognitive great deal of overlap among these conditions, empirical
features that affect functioning outside the social findings have been equivocal. Some studies have found
domain. Studies have shown that individuals with visual-spatial impairments (with strengths in Verbal IQ)
Aspergers disorder have very uneven cognitive profiles. in Aspergers disorder, while others have not demonstrated
One explanation for this common finding is that these this pattern (see Klin et al., 2000). Further work with more
individuals have weak central coherence. That is, they stringent diagnostic criteria is needed in this area.
are more likely to process information as discrete units
rather than processing them as a unified whole. There is
some evidence that bottom-up processing occurs without Coexisting Conditions
accompanying top-down control. As a result, high levels
of details are perceived, while global information may be In addition to the core symptoms, Aspergers disorder
missed (see Frith, 2004). Studies have also shown may also be accompanied by co-occurring disorders.
consistent deficits in overall executive function among Studies have shown that a large percentage of children
individuals with Aspergers disorder (as is also the case with Aspergers disorder also exhibit problems with atten-
in autistic disorder). Specifically, poor performance has tion and impulse control (similar to those found in
been shown on both the Wisconsin Card Sorting Test and ADHD). However, the DSM-IV prevents an additional
the Tower of Hanoi. Particular deficits have been noted in diagnosis of ADHD when there is an existing pervasive
the ability to shift response set and in overall planning. developmental disorder diagnosis. In adolescence and
Consistent with this, individuals with Aspergers disorder adulthood, case studies indicate relatively high rates of
are often described as having difficulty adjusting to depression, anxiety, and bipolar disorder among indivi-
changes in routine or task demands, and as having a duals with Aspergers disorder (see Ghaziuddin, 2002 for
strong need for sameness (see Klin et al., 2000). review). Recent evidence has also demonstrated that indi-
Some studies, including Wings (1981) original de- viduals with Asperger disorder have significant adaptive
scription, have found significantly higher verbal IQ scores impairments as well (see Saulnier & Kim, 2007).
than performance IQ scores among individuals with
Aspergers disorder (the reverse of which is typically
found in autistic disorder). Motor clumsiness has also Evaluation
been observed among children with Aspergers disorder
since Hans Aspergers original paper, although it has Diagnostic assessment of Aspergers disorder is best con-
never been a part of the formal diagnostic criteria ducted using multiple methods and observers. Due to the
(see Frith, 1991). As a result, researchers have been complexity of the disorder, and its effects on broad areas
interested in potential similarities between Aspergers dis- of functioning, interdisciplinary assessment is recom-
order and nonverbal learning disorders (NVLD) or right mended. First, because Aspergers is a neurodevelopmen-
hemispheric dysfunction. These profiles are marked by tal disorder, parent report of early history and
relative strengths at rote verbal skills, with deficits in social development, as well as structured observations of current
understanding and motor coordination. While there is a behavior, are essential. Currently, the two gold-standard
Aspergers Disorder A 261
tools for diagnosis of autism spectrum disorders are based on the region, they may range from specialized
A
the Autism Diagnostic Interview Revised (ADI-R) and schools designed to serve students with Aspergers
the Autism Directed Observation Schedule (ADOS). disorder to modifications within general education class-
These are the most widely studied measures in the field, rooms. Some students may get benefit and support
and reliability and validity have been well established. The from paraprofessional aides in the classroom, while others
ADI-R is a comprehensive interview that assesses past and may require only slight academic modifications. Most
current functioning in the areas of communication, social students with Aspergers disorder benefit greatly from
interaction, and restricted or repetitive behavior. The communication interventions aimed at improving prag-
Autism Directed Observation Schedule (ADOS) is anoth- matic and social skills (see Klin et al., 2000; Mesibov et al.,
er diagnostic tool that allows for clinic-based observations 2001 for review).
across various structured activity- and conversation- Family support, parent training, and instruction on
based interactions. Despite their advantages, however, behavior management strategies can also be helpful when
neither tool was designed to measure Aspergers disorder disruptive behaviors accompany the clinical picture. For
specifically, or to differentiate between Aspergers disorder adolescents and adults, there are emerging data showing
and other PDDs. A number of other scales have been that both individual and group-based cognitive behavioral
developed to assess Aspergers disorder, but systematic therapy are promising in the treatment of co-occurring
research is lacking as of yet (see Matson & Boisjoli, 2008; symptoms of depression and anxiety. Individual work
Mesibov, Shea, & Adams, 2001 for review). with counsellors or mental health professionals could
In addition to assessing the core symptoms of Asper- also focus on social and communication skills training as
gers disorder, assessment should focus on cognitive, well as bolstering adaptive functioning. In addition, med-
adaptive, and communication skills. General measures of ications may be prescribed to treat associated symptoms
intelligence, such as the Wechsler Scales and the Stanford (particularly inattention, depression, and anxiety).
Binet Intelligence Scales are useful in assessing overall
functioning as well as particular strengths and weaknesses.
Additionally, it is helpful to include measures of visual- Cross References
spatial and visual-motor processing, particularly since
these areas are typically weaker in Aspergers disorder. Autistic Disorder
Given common deficits in executive function and atten- Nonverbal Learning Disabilities
tion, neuropsychological assessment of these functions is Pervasive Developmental Disorder NOS
recommended. Measures of social communication and
pragmatic language, and adaptive skills, also add informa-
tion to the clinical picture and help inform intervention References and Readings
recommendations (see Klin et al., 2000 for review).
American Psychiatric Association. (1994). Diagnostic and statistical
manual of mental disorders (4th ed.). Washington, DC: American
Treatment Psychiatric Publishing, Inc.
Fombonne, E. (2005). The changing epidemiology of autism. Journal of
Applied Research in Intellectual Disabilities, 18, 281294.
There is, as of yet, no available treatment that provides a
Fombonne, E., & Tidmarsh, L. (2003). Epidemiologic data on Asperger
cure for the core impairments of Aspergers disorder. disorder. Child and Adolescent Psychiatric Clinics of North America,
However, there are a number of interventions that target 12, 1521.
specific symptoms. In addressing social deficits in Frith, U. (Ed.). (1991). Autism and Asperger syndrome. Cambridge: Cam-
Aspergers disorder, there have been several promising bridge University Press.
Frith, U. (2004). Emanuel Miller lecture: Confusions and controversies
studies of social competence interventions among children
about Asperger syndrome. Journal of Child Psychology and
and adolescents with Aspergers disorder and autism. Such Psychiatry, 45, 672686.
interventions can be delivered in educational or outpatient Ghaziuddin, M. (2002). Asperger syndrome: Associated psychiatric and
clinic-based settings, and typically include cognitive and medical conditions. Focus on Autism and Other Developmental
behavioral components (including direct instruction, Disabilities, 17, 138144.
Klin, A., Volkmar, F. R., & Sparrow, S. (Eds.). (2000). Asperger syndrome.
modelling skills, and skills practice) (see Klin et al., 2000).
New York, NY: The Guilford Press.
Educationally, students with Aspergers disorder often Matson, J. L., & Boisjoli, J. A. (2008). Strategies for assessing Aspergers
benefit from modifications and supports provided syndrome: A critical review of data based methods. Research in
through special education. Although services vary widely Autism Spectrum Disorders, 2, 237248.
262 A Assessment of Consent
Mattila, M., Kielinen, M., Jussila, K., Linna, S., Bloigu, R., Ebeling, H., responsibilities, interpersonal relationships, community
et al. (2007). An epidemiological and diagnostic study of Asperger
life, education, employment, and recreation (classified as
syndrome according to four sets of diagnostic criteria. Journal of the
American Academy of Child & Adolescent Psychiatry, 46, 636646.
social roles). The long form includes 31 subsections, es-
McLaughlin-Cheng, E. (1998). Asperger syndrome and autism: A sentially covering the listed domains with a greater degree
literature review and meta-analysis. Focus on Autism and Other of specificity. Each item is rated on a 4-point level of
Developmental Disabilities, 13, 234245. accomplishment scale (with an additional option to
Mesibov, G. B., Shea, V., & Adams, L. W. (2001). Understanding Asperger
state not applicable), a 5-point level of satisfaction
syndrome and high-functioning autism. New York, NY: Kluwer
Academic/Plenum Publishers.
scale, as well as a rating regarding the type and level of
Saulnier, C. A., & Klin, A. (2007). Brief report: Social and communication assistance required (i.e., no assistance, assistive device,
abilities and disabilities in higher functioning individuals with adaptation, human assistance). A score for each item is
autism and Asperger syndrome. Journal of Autism and Developmental obtained with reference to a scoring template included in
Disorders, 37(4), 788793.
the manual, grading according to the level of difficulty and
Schopler, E., Mesibov, G. B., & Kunce, L. J. (Eds.). (1998). Asperger
syndrome or high-functioning autism? New York, NY: Plenum Press.
level of assistance. Item scores range from 0 (not accom-
Wing, L. (1981). Aspergers syndrome: A clinical account. Psychological plished) to 9 (performed with no difficulty and no assis-
Medicine, 11, 115130. tance), with mid-scale examples being 3 (performed with
difficulty and human assistance), and 6 (performed with
difficulty and technical aid or adaptation). Scores can then
be weighted by the number of applicable activities to
Assessment of Consent obtain domain-level scores, or a simple formula can be
used to obtain an overall score.
Informed Consent
Historical Background
Assessment of Life Habits (LIFE-H) Noreau, Fougeyrollas, and Tremblay (2005) stated that the
LIFE-H was developed to assess social participation in
J ESSICA F ISH people with disabilities, regardless of the nature of those
Medical Research Council Cognition & disabilities, and based upon the Disability Creation Process
Brain Sciences Unit model, which views handicap as the situational result of
Cambridge, UK the interaction of two causal dimensions: the characteristics
of the individual and those of the environment. Version
2.0 of the scale was developed following a content validity
Synonyms study that involved 12 experts in rehabilitation medicine
evaluating the scale (in terms of clarity and pertinence of
The abbreviation LIFE-H is consistent, but version num- content, classifications used in the measurement scales,
bers are often appended (e.g., LIFE-H 1.0, 2.0, 3.0, 3.1) etc.); modifications included reversing the scoring of the
accomplishment section such that higher scores reflected
the competence in the activity. Version 3.0 incorporated a
Description greater number of items within particular domains and
added additional filter questions to some sections (e.g., if
The Assessment of Life Habits (LIFE-H) is a self-report you are not currently employed, skip to section x). Version
measure of social participation of people with disabilities. 3.1 is a short form based upon version 3.0.
The original version of the scale consisted of 298 items;
later versions have reduced the number of items to 240
(version 3.0). Various short forms are also available (5577 Psychometric Data
items), with the most recent being the 77-item version 3.1.
The long form is said to take between 20 and 120 min to Fougeyrollas et al. (1998) reported that the LIFE-H v1.0
complete, and the short form, 2060 min. In the short demonstrated acceptable internal consistency in adults
form (version 3.1), items are organized into 12 categories: and children (Intra-class Correlation (ICCs) > 0.5 for
nutrition, fitness, personal care, communication, housing, each life habit), and good testretest reliability in children
mobility (classified as activities of regular living) and and adults with spinal cord injury (ICC children r = 0.73,
Assessment of Motor Process Skills A 263
and adults r = 0.74). Inter-rater reliability was examined and disabilities: Conceptual approach and assessment of handicap.
International Journal of Rehabilitation Research, 21(2), 127141. A
in a group of 20 stroke patients (Beaulieu et al., 1996;
Noreau, L., Desrosiers, J., Robichaud, L., Fougeyrollas, P., Rochette, A., &
Cited in Noreau et al., 2002), with ICCs for 6/12 accom- Viscogliosi, C. (March 18, 2004). Measuring social participation:
plishment ratings of life habits above 0.6, and 10/12 Reliability of the LIFE-H in older adults with disabilities. Disability &
satisfaction ratings for life habits above 0.6. Similar Rehabilitation, 26(6), 346352.
findings have been reported for inter-rater reliability of Noreau, L., Fougeyrollas, P., & Tremblay, J. (2005). The measure of life
habits (LIFE-H) users manual. RIPPH. http://www.ripph.qc.ca/?
LIFE-H scores for people with physical disabilities, with
rub2=4&rub=15&lang=en. Accessed 23 Apr 2010.
ICCs of r > 0.75 for 7/10 categories, and r = 0.89 for the Noreau, L., Fougeyrollas, P., & Vincent, C. (2002). The LIFE-H: Assess-
whole scale (Noreau et al., 2004). ment of the quality of social participation. Technology and Disability,
Several studies have examined the predictive validity of 14(3), 113118.
the LIFE-H. Desrosiers et al. (2003) presented evidence of Rochette, A., Desrosiers, J., & Noreau, L. (2001). Association between
personal and environmental factors and the occurrence of handicap
the convergent validity of the LIFE-H in the form of high
situations following a stroke. Disability & Rehabilitation, 23(13),
correlations with the Functional Autonomy Measurement 559569.
System (SMAF), and moderate correlations with the Func-
tional Independence Measure (FIM). Further, LIFE-H
scores were lower in stroke patients than neurologically
healthy controls. A comprehensive review of the psycho-
metric properties of the LIFE-H is available online (http://
Assessment of Motor Process
www.medicine.mcgill.ca/Strokengine-assess/module_lifeh_ Skills
indepth-en.html#section3).
K ELLI W ILLIAMS G ARY
Virginia Commonwealth University
Clinical Uses Richmond, VA, USA
pertain to sustaining performance, applying knowledge, tasks to the clients daily life needs. Therefore, the environ-
temporal organization, organizing space and objects, and ment should be naturalistic and approximate the conditions
adapting performance. Scores are based on observation of in which the client can comfortably perform tasks. Settings
the client from certified raters. Motor and process skills are for AMPS observation can vary based on the space available
rated simultaneously utilizing a 4-point ordinal criterion and can include fully equipped clinic kitchens, laundry
referenced rating scale with the highest score denoting com- rooms, outdoor, and the clients own room in the hospital
petent performance, followed by questionable, inefficient, or nursing home.
and markedly inefficient performance. AMPS computer The primary advantage of the AMPS is that it can be
scoring software converts ordinal raw scores of easy skill used with persons of virtually any age, diagnosis, or disabil-
items for persons of low ability and hard skill items for ity. However, the scope and breadth of evidence for AMPS
persons of high ability along a single common equal-interval use is limited in psychiatric, neurologic, and pediatric set-
linear scale (Fisher, 1994). tings. Geriatric settings have offered the most research evi-
dence for those with cognitive impairments and dementias,
followed by a sizable proportion of research for people with
Historical Background learning disabilities (Hitch, 2007).
The genesis of the AMPS is found in the psychiatric assess- Cross References
ment of clients with schizophrenia and depression in Hali-
fax, Canada (Fisher & Bernspang, 2007). In 1994, the basic Activities of Daily Living
idea was further developed and a specific tool was stan- Instrumental Activities of Daily Living
dardized by Anne G. Fisher, ScD, OTR and colleagues from Occupational Therapy
the Division of Occupational Therapy, Umea University in
Umea, Sweden. Currently, the AMPS is used in at least 20 References and Readings
countries. In 2006, the most recent version (sixth version)
was published to increase applicability across populations, Fisher, A. G. (1994). Development of a functional assessment that adjusts
diagnoses, disabilities, cultural background, nationality, ability measures for task simplicity and rater leniency. In M. Wilson
and age groups by adding additional tasks (Fisher, 2006). (Ed.), Objective measurement: Theory into practice (Vol 2, pp. 145
175). Norwood, NJ: Ablex.
Fisher, A. G. (2006). Assessment of motor and process skills. Vol. 1: Devel-
opment, standardization, and administration manual (6th ed.). Fort
Psychometric Data Collins, CO: Three Star.
Fisher, A. G., & Bernspang, B. (2007). Response to: A critique of the
The AMPS has robust psychometric properties. Interrater Assessment of Motor and Process Skills (AMPS) in mental health
and intrarater reliability are high with 95% of calibrator practice. Mental Health Occupational Therapy, 12, 1011. (Available
from http://www.ampsintl.com/documents/MHOT%20March%
raters demonstrating goodness-of-fit to the many-faceted
202007.pdf)
Rasch model. Testretest reliability is high on a diagnosti- Hitch, D. (2007). A reply from Danielle Hitch to the Fisher and Bern-
cally heterogeneous sample of older adults with r = 0.90 to spang response to: A critique of the Assessment of Motor and
0.91 for AMPS process scale and motor scale, respectively Process Skills (AMPS) in mental health practice. Mental Health
(Fisher, 2006). Studies have found good validity of the Occupational Therapy, 12, 14. (Available from http://www.ampsintl.
com/documents/MHOT%20March%202007.pdf)
AMPS when applied to groups of different racial, ethnic,
and cultural backgrounds, across gender, and with multi-
ple diagnoses.
Assisted Living
Clinical Uses J AY B EHEL
Rush University Medical Center
Fisher (2006) states: the AMPS provides occupational Chicago, IL, USA
therapy practitioners with a powerful and sensitive tool
that can assist with planning effective ADL interventions
and documenting change. Because of the AMPS unique Synonyms
and innovative design, occupational performance is
evaluated based on the familiarity and relevance of the Domiciliary care; Residential care
Assistive Technology A 265
Definition A
Assistive Technology
Assisted living is a care arrangement that provides
supervision and assistance to individuals who are unable D IANE C ORDRY G OLDEN
to live independently but do not require the level of care Association of Assistive Technology Act Programs
provided in conventional nursing homes. Delmar, NY, USA
Assisted living arrangements may take place in structured Assistive technology (AT) is a term used to refer to both
assisted living facilities, small group homes, or an indivi- AT devices and AT services. A formal, legal definition
duals own home or the home of a family member. These of AT devices and services was first published in the
arrangements have as their goal the preservation of a Technology-Related Assistance for Individuals with Dis-
degree of autonomy and privacy at home or in a home- abilities Act of 1988 as follows:
like setting. When sited in ones home, assistance may be " assistive technology device means any item, piece of
provided by a combination of paid caregivers, family equipment, or product system, whether acquired com-
members, and other paid or unpaid assistants to help mercially, modified, or customized, that is used to in-
with housekeeping, laundry, cooking, and transpor- crease, maintain, or improve functional capabilities of
tation. Assistance provided may include supervision for individuals with disabilities
safety, medication management, meal preparation, and
accompaniment and assistance during community-based " assistive technology service means any service that direct-
activities. Assisted living facilities may also offer social ly assists an individual with a disability in the selection,
activities and specialized services for individuals with acquisition, or use of an assistive technology device
cognitive impairment. Basic Activities of Daily Living AT devices include a vast array of items such as wheel-
(BADL) such as hands-on bathing, dressing, and feeding chairs, eyeglasses, hearing aids, Braille printers, electronic
are usually not considered as a part of an assisted living note-takers and organizers, augmentative communica-
arrangement as the consistent need for such basic care is tion systems, text-to-speech software, speech synthesizers,
often seen as an indication that nursing home or a home- adaptive keyboards, alternative pointing devices, voice
based parallel thereof is the more appropriate level of care. recognition software, aids for daily living, etc. AT services
Assisted living typically is not covered by private include evaluation/assessment services, selecting, fitting,
insurance or Medicare, and access to such care may be customizing, and repairing devices, delivering training
limited by an individuals finances. Moreover, there is a and technical assistance supports, and coordinating fund-
considerable variability in how care facilities, clinicians, ing and other necessary interventions to support device
and professional literature define and discuss assisted acquisition and use.
living. Consequently, the appropriate role of assisted liv- The definition of AT devices and services has remained
ing in the continuum of care remains unclear, and refine- unchanged through numerous reauthorizations of the
ment and redefinition of this role likely will be ongoing Assistive Technology Act and has been adopted in other
for some time. statutes, such as the Individuals with Disabilities Education
Act. The same definition has also been used in promulgat-
ing federal rules, such as the Electronic and Information
Cross References Technology Accessibility Standards developed pursuant to
Section 508 of the Rehabilitation Act (http://www.access-
Life Care Planning board.gov/sec508/standards.htm).
of AT. The history of hearing aids can be traced back to Goals and Objectives
Alexander Graham Bells pioneering work on develop-
ment of the telephone. Modern wheelchairs are patterned AT goals and objectives begin with a primary focus on
after the first folding, tubular steel wheelchair developed ameliorating and/or compensating for a specific function-
in the 1930s; while the first dedicated wheelchair (called al deficit. For example, electronic organizers can be used
an invalids chair) is thought to have been invented to address memory or information processing problems;
4 centuries ago for Phillip II of Spain. Some devices text-to-speech software can be used to address reading
were developed as AT and evolved into mainstream tech- deficits; augmentative communication systems can be
nology. For example, in 1948 the National Bureau of used to address communication limitations, etc. In most
Standards developed specifications for a low-cost reliable cases, secondary goals are also targeted for outcomes
talking-book machine for the blind that became the including increasing academic success, fostering gainful
tape recorder. Conversely, some items developed as main- employment, supporting independent community living,
stream technology became AT such as voice recognition decreasing inappropriate behaviors, etc. With expanding
software originally developed for dictation that is used by legal mandates for integration of individuals with
individuals with motor disabilities who are unable to use a disabilities into all societal settings (Individuals with
keyboard for computer access. Disabilities Education Act, Section 504 of the Rehabilita-
In recent years, technology use has become more tion Act, and the Americans with Disabilities Act), AT
commonplace for everyone. Similarly, AT use is now goals and objectives continue to expand into new
more frequent across the disability spectrum, addressing outcome areas.
deficits in hearing, vision, motor, social, organizational,
cognitive, speech, language, information processing, etc.
Especially critical today is the use of information technol- Treatment Participants
ogy (IT), including telecommunications. IT use is now
critical to success in education, employment, independent AT is an appropriate intervention option to consider
living, and community integration and AT is the interface when functional limitations are encountered. Candidacy
that makes IT accessible (http://www.albritton.us/AThis- for AT is not limited by age, disability diagnosis, or
tory.html). severity/combination of deficits. There are no prerequi-
sites for AT consideration and AT should not be relegated
to a last resort intervention after all other interventions
have been tried and abandoned.
Rationale or Underlying Theory AT can address a variety of human functions and is
frequently grouped into areas such as vision, hearing,
Today, AT intervention is rooted in the disability rights communication, daily living, computer access, learning/
movement and self-determination efforts of individuals cognition, environmental adaptations, mobility/seating/
with disabilities and their advocates. These initiatives positioning, vehicle modifications, and recreation/leisure.
helped to delineate the difference between the medical/ For almost all functional limitations, there is a range of AT
rehabilitation and independent living models of interven- intervention that can be considered as a treatment option
tion for individuals with disabilities. The medical model (Cook & Hussey, 2001).
identifies a physical or mental impairment or lack of
certain skills and treatment is delivered to remediate the
deficit(s). With the medical model the locus of the Treatment Procedures
problem lies with the individual and the goal is to fix
the individual in some way through professional Consideration for AT begins with assessment by a quali-
treatment. Under the independent living model the prob- fied team of individuals who are knowledgeable about the
lem is defined as a lack of supports and accommodations, individual, their strengths and limitations and the range
inaccessibility, and/or autonomy the problem lies with of potential AT options available to address the indivi-
the environment or the interaction with the environment, duals functional needs. Best practice includes conducting
rather than within the person. In this model, AT plays a structured device trials with various AT devices in the
major role in addressing/ameliorating interaction environment(s) in which the individual will be using the
difficulties, typically without overtly attempting to fix technology (e.g., home, school, work, community, etc.).
the disability itself (DeJong, 1979; Pelka, 1997). This allows for comparative analysis of different device
Assistive Technology A 267
features and functions to determine which best addresses and Do we understand the commitment? (DeRuyter,
A
the individuals needs. 1995; Trachtmann, 1994). In these articles, the authors
Once AT has been acquired for an individual, training postulated that stakeholders and AT providers must be
and support must be provided for the user, their family, prepared to show how their devices/services make a dif-
and other critical individuals such as teachers, therapists, ference in the lives of individuals who receive an AT
etc. More complex AT (computer-based software applica- intervention.
tions, assistive listening systems, augmentative communi- Today, outcome measurement is occurring in all AT
cation devices, etc.) frequently requires significant service areas (medicine, education, employment/voca-
investment of time and resources in initial programming, tional rehabilitation, and independent living) through a
fitting, and set-up, in addition to training on device use variety of interdisciplinary activities. Some are driven by
(Galvin & Scherer, 1996). policy needs, in particular, accountability for public dol-
lars spent on AT (e.g., Medicare, Medicaid, special educa-
tion, vocational rehabilitation, etc.) and justification for
Efficacy Information
private insurance expenditures on AT. Others are driven
by an overarching goal of quality service delivery and
Efficacy research on AT includes basic documentation of
continuous program improvement.
changes in functional skill areas (those the AT is intended
The most direct outcome measure for AT intervention
to address) and potential secondary improvements in
is demonstration of functional skills, independence, well-
academic, social, behavioral, and other areas. Much of
being, and quality of life. Administration of standardized
this efficacy is self-evident and is reported by the AT
assessments in the aided condition (using AT) can be
users themselves (Scherer, 1993).
useful in measuring outcomes in discreet skill areas (e.g.,
No discussion of AT efficacy would be complete with-
auditory discrimination, memory, expressive communi-
out addressing the issues of device abandonment and
cation, etc.). In addition, some global assessment of AT
cost/benefit. For many types of AT, consumer discontinu-
outcomes can be helpful such as the Psychosocial Impact
ing use of the device after acquisition has been a historic
of Assistive Devices Scales (PIADS) (Jutai & Day, 2002),
problem (Wessels, Dijcks, Soede, Gelderblom, & De
the Quebec User Evaluation of Satisfaction with Assistive
Witte, 2003). Factors shown to mitigate abandonment
Technology (QUEST) (Demers, Weiss-Lambrou, & Ska,
include active consumer and family involvement in the
1996), and similar instruments. A number of online
selection and implementation of AT and the relative ad-
resources are also available with extensive data on AT
vantage of AT within the array of intervention options
outcome tools and research such as the Adaptive Technol-
available (Alper & Raharinirina, 2006; Riemer-Reiss &
ogy Resource Center (http://atrc.utoronto.ca/index.php?
Wacker, 2000).
option=com_content&task=view&id=175&Itemid=69),
As technology continues to improve, the problem of
the Assistive Technology Outcomes Measurement System
device abandonment steadily abates. Today the greater chal-
(ATOMS) Project (www.uwm.edu/CHS/atoms), the Con-
lenge is in justifying the cost/benefit of AT to secure funding
sortium for Assistive Technology Outcomes Research
from private insurance, local, state, federal, and other fund-
(www.atoutcomes.org), and the Quality Indicators for
ing sources. Some types of AT, such as durable medical
Assistive Technology Services (www.qiat.org).
equipment, have a longer history of cost/benefit data in-
cluding prevention of secondary disabilities making funding
more readily available. Other types of AT, such as electronic
Qualifications of Treatment Providers
organizers used to remediate/compensate for cognitive lim-
itations, are relatively new with little cost/benefit data
AT intervention can be provided by an extensive list of
making funding difficult to secure (Gillette & DePompei,
professionals, usually specialists in the type of the AT
2004; Hart, Buchhofer, & Vaccaro, 2004).
provided. For example, hearing aids are typically provided
by audiologists or hearing instrument dispensers, eye-
Outcome Measurement glasses by optometrists and ophthalmologists, etc. How-
ever, as the AT becomes less prescriptive in nature, the
The field of AT outcomes is quite young with most pub- range of providers expands. Electronic note-takers and
lished work emerging in the 1990s. Two of the first organizers can be provided by a whole host of providers,
focused articles on evaluating AT outcomes posed the special educators, rehabilitation counselors, behavior
questions Are we ready to answer the tough questions? therapists, occupational therapists, AT practitioners, etc.
268 A Association Areas
Cross References
Major Areas or Mission Statement
Association Pathways
The mission of APPCN is to foster the provision of ad-
Heteromodal Cortex
vanced specialty education and training to promote the
Secondary Cortex
competencies that are necessary for practice in the specialty
Unimodal Cortex
of clinical neuropsychology (Boake, Yeates, & Donders,
2002).
References and Readings
Landmark Contributions
Kupermann, I. (1991). Localization of higher cognitive and affective
functions: The Association cortices. In E. R. Kandel, J. H. Schwartz, Formally incorporated in 1992, APPCN contributed to the
& T. M. Jessel (Eds.), Principles of neural science (3rd ed., pp. 823
Houston Conference, which established that completion
838). East Norwalk, CT: Appleton & Lange.
Mendoza, J. E., & Foundas, A. F. (2008). Clinical neuroanatomy: a behav-
of two years of formal postdoctoral residency training is a
ioral approach. New York: Springer. uniform requirement for entry into the professional prac-
Mesulam, M.-M. (2000). Principles of behavioral and cognitive neurology. tice of clinical neuropsychology (Hannay, Bieliauskas,
New York: Oxford University Press. Crosson, Hammeke, Hamsher, & Koffler, 1998). In more
Pandya, D. N., & Seltzer, B. (1982). Association areas of the cerebral
recent years, APPCN has been a key representative to inter-
cortex. Trends in Neurosciences, 5, 385390.
Pandya, D. N., & Yeterian, E. H. (2003). Cerebral cortex: architecture and
organizational groups like the Clinical Neuropsychology
connections. In Encyclopedia of the neurological sciences (pp. 594 Synarchy, and the Inter-Organizational Summit on Educa-
604). USA: Elsevier Science. tion and Training.
270 A Association for Postdoctoral Programs in Clinical Neuropsychology (APPCN)
Cross References A
Association Pathways
Commissures, Cerebral
J OHN E. M ENDOZA Projection Pathways
Tulane University Medical Center
New Orleans, LA, USA
Cross References
Arcuate Fasciculus
Association Pathways
Cerebral Cortex
Cingulum
U
Superior Longitudinal Fasciculus
U
SLF White Matter
AF arcuate fasciculus
IOF inf. occiptofrontal fasc. AF
SLF sup. longitudinal fasc. IOF
UF
U U-fibers
UF uncinate fasciculus
Associative Aphasia
Association Pathways. Figure 1 Conduction Aphasia
272 A Associative Memory
Astrocytoma Asymmetry A
Definition Synonyms
corresponding to Brocas area, has been shown to be more ability to use numbers is thought to be a function nor-
highly developed on the left side for most individuals. The mally carried out by the left hemisphere, the disturbance
gyri and sulci associated with the motor strip (Brod- of which following a lesion to the left hemisphere may be
manns area 4) are more prominent in the left hemisphere defined as acalculia (dyscalculia). However, most complex
of right-handers. Fairly consistent differences in the later- arithmetical operations also have a spatial component.
al fissure have been found, with the posterior ascending For example, precise alignment of rows and columns of
ramus of this sulcus making a more abrupt upward turn numbers is critical in mathematical operations, whether
in the right hemisphere as compared with the left. This completed mentally or on paper. These spatial relations
would suggest likely differences in the distribution of the can be disturbed following right-hemispheric lesions,
supramarginal and angular gyri in the inferior parietal resulting in what has been termed spatial dyscalculia.
lobules of the two hemispheres. Even on a more micro- It is known that the hemisphere contralateral to the
level, differences in the size and organization of individual hand being used to carry out some motor tasks is imme-
cells or cell columns have been identified in the two diately responsible for the execution of these movements.
hemispheres. However, the motor programs or engrams for overlearned
It is reasonable to speculate that some structural dif- motor skills are believed to reside in the left hemisphere,
ferences likely relate to functional differences between the certainly for the vast majority of right-handers, as well as
two hemispheres, particularly behaviors such as language many left-handers. Thus, any lesion that either directly
and handedness. However, functional asymmetries have interferes with those engrams or the ability of that infor-
either been demonstrated or are suspected well beyond mation to reach the premotor cortex of either hemisphere
those which can currently be explained by structural dif- can result in an impaired performance, especially if the
ferences. The following represent a sampling of some of individual is asked to demonstrate the action in the ab-
the functional differences that have been observed. sence of the actual object. This latter condition is referred
to as an ideomotor apraxia.
Perceptual abilities appear to be differentially
Functional Asymmetries distributed between the two hemispheres. It has already
been noted that the left hemisphere is normally the lead-
It has been well established that language expression ing hemisphere in interpreting verbal (semantic) infor-
and comprehension are normally mediated primarily, mation, while the right appears to be better adapted to
if not exclusively, by the left hemisphere, even among processing certain types of emotional cues. It seems that
left-handers. However, the right hemisphere has also the right hemisphere is also the more proficient in proces-
been shown to play an important role in communication. sing many types of visual-spatial or visual-gestalt infor-
Verbal communication is not just about using words in mation. Thus, the right hemisphere has been found to be
sentences or paragraphs; emotional tone or nuances of the generally superior in carrying out certain constructional
speaker often convey important meaning. In some com- tasks, making judgments regarding the orientation of
munications, such as those with a sarcastic intent, the real lines in space, in making discriminations regarding unfa-
message is carried by the tone rather than by the words, miliar faces, and in recognizing familiar tunes or environ-
which, if interpreted literally, might actually convey a very mental sounds. On the other hand, the left hemisphere
different message. The ability to use as well as interpret appears to be the leading hemisphere when it comes to
these emotional components of speech, known as proso- perception of certain aspects of ones own body. Problems
dy, is primarily mediated by the right hemisphere; damage of rightleft orientation and difficulty recognizing indi-
to this side of the brain may produce various forms of vidual fingers of ones hands (finger agnosia) are typically
aprosodia. With regard to using or interpreting the lan- associated with lesions of the left inferior parietal lobule.
guage of others, the right hemisphere is also believed to Functional MRI studies have demonstrated consistent
play an important role in identifying the central theme or activation of right hemisphere structures during tests of
point of the discourse of others and being able to stay on vigilance and directed attention. However, divided atten-
point when speaking or writing. It appears to be impor- tion tasks have been shown to selectively activate left
tant in appreciating verbal (as well as nonverbal) humor prefrontal cortex. PET imaging studies have demon-
and in detecting meaning from the differential inflections strated increased blood flow in the right prefrontal and
given to individual words in speech. superior parietal cortex during tasks requiring sustained
In addition to words, numbers also have their own attention, regardless of the type of stimulus (verbal, visu-
symbolic meaning. Hence, as might be expected, the al, etc.) or where it is introduced (left vs. right).
Ataxia A 277
Differences between the two hemispheres have also Davidson, R. J., & Hugdahl, K. (Eds.). (1996). Brain asymmetry. Cam-
bridge, MA: Bradford Books. A
been demonstrated in learning and memory tasks and
Gazzaniga, M. S. (2000). The new cognitive neurosciences. Boston: MIT
other cognitive domains. Of the two, the left hemisphere Press.
has been more strongly associated with learning verbal Gazzaniga, M. S., Ivry, R. B., & Mangun, G. R. (2002). Cognitive neuro-
information. While many studies have shown that the science: The biology of the mind. New York: W.W. Norton &
right hemisphere is perhaps better at learning certain Company.
Geschwind, N., & Levitsky, W. (1968). Left-right asymmetry in temporal
nonverbal or visual-spatial type information, the
speech region. Science, 161, 186187.
findings are generally less robust compared to the left Good, C. D., Johnsrude, I., Ashburner, J., Henson, R. N. A., Friston, K. J.,
hemisphere and verbal memory. One frequent explana- & Frackowiak, R. S. J. (2001). Cerebral asymmetry and the effects of
tion for this is that when faced with any memory task, sex and handedness on brain structure: A voxel-based morphometric
humans have a natural tendency to try to verbally encode analysis of 465 normal adult human brains. Neuroimage, 14,
685700.
the stimulus, thus bringing the left hemisphere into play.
Kinsbourne, M. (Ed.). (1978). Asymmetrical function of the brain. New
It has also been suggested that the two hemispheres York: Cambridge University Press.
play different roles in attention. The much more frequent Mendoza, J. E., & Foundas, A. L. (2008). Clinical neuroanatomy:
association of disorders such as unilateral neglect and A neurobehavioral approach. New York: Springer.
anosognosia with right hemispheric lesions have led to Ross, E. (2000). Affective prosody and the aprosodias. In M. Mesulam
(Ed.), Principles of behavioral and cognitive neurology. New York:
the hypothesis that while, as might be expected, the left
Oxford University Press.
hemisphere attends to the right side of space (both per- Walsh, K. (1994). Neuropsychology: A clinical approach. New York:
sonal and extrapersonal), the right hemisphere focuses on Churchill Livingstone.
both right and left space.
Finally, the association of the right hemisphere and
emotional expression would appear to go beyond the Ataxia
affective intonations of speech as described above. It is
commonly observed, both by health-care professionals as A NNA D E P OLD H OHLER 1, M ARCUS P ONCE DE LEON2
well as the spouses and other family members of persons 1
Boston University Medical Center
with brain injury that individuals with right hemisphere Boston, MA, USA
lesions often behave differently than those with left-sided 2
William Beaumont Army Medical Center
lesions. While the latter seem to remain emotionally at- El Paso, TX, USA
tached, even if that emotion is often one of anger, frustra-
tion, or sadness, right hemispherically damaged patients
are more likely to be described as apathetic, indifferent, Synonyms
emotionally flat, both in terms of their verbal and facial
expressions and their interpersonal relationships. Clumsiness
There are a number of different types of damage to the Cerebellar syndromes may be associated with cognitive
cerebellum. These range from fixed damage (e.g., stroke, slowing.
trauma, hypoxic injury) to chemical and metabolic, and Evaluation includes a detailed neurological examina-
degenerative. Cerebellar injury related to vitamin tion, magnetic resonance imaging, and laboratory inves-
deficiency (e.g., E, B12, and thiamine) may be reversible tigation for reversible or genetic causes.
and should be identified and treated. Metabolic diseases Treatment depends on the underlying insult.
such as Hartnups, Refsums, and the mitochondrial dis-
orders are less treatable. A deficiency of Coenzyme Q10
Cross References
has been described in patients with cerebellar ataxia, usu-
ally with childhood onset and often associated with sei-
Cerebellum
zures. The symptoms may respond to Coenzyme Q10
Dysdiadochokinesia
treatment. There are a number of hereditary cerebellar
ataxias. Most of the autosomal recessive and autosomal
dominant ataxias have no treatments. An exception is References and Readings
vitamin E deficiency, which is an autosomal recessive
disorder. It is due to a mutation in the gene coding for Gilman, S. (2004). Clinical features and treatment of cerebellar disorders.
the alpha tocopherol transfer protein located on the long In R. L. Watts & W. C. Koller (Eds.), Movement disorders (2nd ed.,
pp. 723736). New York: McGraw-Hill.
arm of chromosome 8. It is characterized by childhood
onset of ataxia, dysarthria, areflexia, proprioceptive defi-
cits, extensor plantar responses, and skeletal deformities.
The episodic ataxias, which are inherited in an Atheromatous Plaque
autosomal dominant fashion, may also have some
symptomatic treatment regimens. Episodic Ataxia Type 1 Atherosclerosis
is related to a chromosome 12 mutation in the potassi-
um channel gene. Clinically, the disease manifests with
episodes of ataxia lasting seconds to minutes. Patients
may also suffer from myokymia during and between
Atherosclerosis
attacks of ataxia. The ataxia may be induced by startle or
E LLIOT J. R OTH
exercise. Episodic Ataxia Type 2 is related to a defect
Northwestern University
located on chromosome 19. It is due to a mutation in a
Chicago, IL, USA
voltage-dependent calcium channel. Clinically, patients
present with nystagmus. Attacks last minutes to hours
and may be induced by a change in posture. Patients
may also complain of vertigo. As the disease progresses,
Synonyms
ataxia becomes permanent.
Arteriosclerotic vascular disease or ASVD; Atheromatous
plaque; Hardening of the arteries
Epidemiology
Definition
Ataxia is a common sign associated with inherited,
acquired, toxic, and traumatic events. Atherosclerosis is the progressive pathological process of
buildup of plaque inside the blood vessels, resulting in
blockage of blood flow through the vessels.
Natural History
The genetic syndromes that are associated with ataxia Current Knowledge
tend to be progressive. Individuals with static insults
such as strokes or trauma may show improvement in The plaque that causes atherosclerosis is comprised of
function over time. fatty substances, cholesterol, cells, calcium, and fibrin, a
Atkins v. Virginia A 279
stringy material found normally in the blood to help clot References and Readings A
the blood. The plaque formation process stimulates the
cells of the artery wall to produce substances that then Stary, H. C., Chandler, A. B., Dinsmore, R. E., Fuster, V., Glagov, S., Insull,
accumulate in the vessel wall. Fat builds up within W., et al. (1995). A definition of advanced types of atherosclerotic
lesions and a histological classification of atherosclerosis: A report
these cells and around them, and they form connective
from the Committee on Vascular Lesions of the Council on Arterio-
tissue and calcium. The artery wall thickens, the arterys sclerosis, American Heart Association. Circulation, 92, 13551374.
diameter is reduced, and blood flow and oxygen delivery
are decreased. Plaques can rupture or crack open, causing
the sudden formation of a blood clot (thrombosis).
Atherosclerosis can cause angina or myocardial infarction Atherosclerotic Dementia
if it blocks the blood flow in the coronary arteries that
supply the heart muscle, stroke if it blocks the carotid Vascular Dementia
arteries that supply the brain, kidney disease if it blocks
the renal arteries or gangrene possibly leading to amputa-
tion if it blocks the peripheral arteries that supply the
limbs. Atherosclerotic Heart Disease
Atherosclerosis may be asymptomatic for many years.
Risk factors for this disease have been well studied. It is Coronary Disease
thought that atherosclerosis is caused by a response to
damage to the endothelium from high cholesterol, high
blood pressure, and cigarette smoking. A person who has
all three of these risk factors is eight times more likely to Atherothrombotic Brain
develop atherosclerosis than is a person who has none. Infarction
Physical inactivity, diabetes, and obesity are also risk
factors for atherosclerosis. Heredity, advancing age, and Ischemic Stroke
racial background are less-significant risk factors.
Treatment options include lifestyle changes, use of
lipid-lowering and other drugs, angioplasty, and various
surgical procedures, depending on the location of the
plaque. Many lifestyle changes that prevent disease Athymia
progression also prevent the onset of the disease; these
include a low-fat, low-cholesterol diet, weight loss, exer- Abulia
cise, blood pressure control, diabetes management, and
smoking cessation.
Atkins v. Virginia
Cross References
R OBERT L. H EILBRONNER
Angioplasty Chicago Neuropsychology Group
Anticoagulation Chicago, IL, USA
Antiplatelet Therapy
Atherosclerosis
Cerebrovascular Disease Synonyms
Cholesterol
Coronary Disease Mental retardation defense
Ischemic Stroke
Myocardial Infarction
Peripheral Vascular Disease Historical Background
Stent
Thrombolysis Daryl Atkins and William Jones abducted Eric Nesbitt
Thrombosis from a convenience store and after finding only $60 in
280 A Atomoxetine
his wallet, Atkins and Jones used Nesbitts vehicle to drive were tainted. Thus, Atkins was set to be executed on
to an ATM and forced him to withdraw $200. Thereafter, 2 December 2005. However, the decision was recently
Atkins and Jones drove Nesbitt to an isolated location reversed again by the Virginia Supreme Court, as a result
where he was shot eight times and subsequently died. of state procedural grounds.
Atkins and Jones were quickly tracked down by police
and in custody, it was determined that Jones story claim-
ing that Atkins pulled the trigger was more coherent than Current Knowledge
Atkins story implicating Jones as the shooter. Thus,
Atkins was charged and convicted of abduction, armed Forensic psychological and neuropsychological assess-
robbery, and capital murder. This took place despite the ments of mentally retarded individuals being considered
results of an IQ test completed by a clinical psychologist, for the death penalty are highly important. Specifically,
in which Atkins score of 59 placed him in the mildly the U.S. Supreme Court did not specify the degree of
mentally retarded range. Nonetheless, he was sentenced mental retardation required to circumvent the death
to death. penalty and instead left such determinations up to the
The Supreme Court of Virginia was in agreement with discretion of the states. It is important to note that
the judgment of the trial court and the appeal was taken to sub-average intelligence alone does not warrant a label
the U.S. Supreme Court. In July of 2002, the U.S. Supreme of mental retardation. Impairments in adaptive function-
Court reversed the judgment of the trial court and the ing and evidence of mental retardation prior to the age of
Supreme Court of Virginia and referred the case back to 18 years are needed for a diagnostic determination of
the sentencing court to render a sentence other than the mental retardation.
death penalty. The U.S. Supreme Court ruled that
the punishment was excessive and thus prohibited by
the eighth Amendment as cruel and unusual if it is not Cross References
graduated and proportioned to the offense. An excessive
judgment is judged by current societal standards. Thus, Intellectual Disabilities
societys standards of decency, albeit subject to change, Intelligence
must prove that they are influenced by objective factors
to the maximum possible extent. Furthermore, it was
ruled that mental retardation does not preclude a persons References and Readings
capability to discriminate right from wrong, though
mental retardation does lead to a diminished capacities Atkins v. Virginia, 153 L. Ed 2d 335 (2002).
to process and comprehend information, reduces com- Cunnningham, M. D., & Goldstein, A. M. (2003). Sentencing determina-
tions in death penalty cases. In A. Goldstein (Ed.). Forensic psychology
munication abilities, and decreases ones ability to learn
(Vol 11). Handbook of psychology. New Jersey: Wiley.
from mistakes and experiences, reason logically, inhibit Denney R. L. (2005). Criminal responsibility and other criminal forensic
impulses, and understand the emotions and behaviors of issues. In G. Larrabee (Ed.). Forensic neuropsychology: a scientific
others. The U.S. Supreme Court concluded that mentally approach. New York: Oxford University Press.
retarded individuals are not exempt from criminal Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (2007). Psycho-
logical evaluations for the courts: A handbook for mental health
sanctions, though a decrease in personal culpability is
professionals and lawyers (3rd ed.). New York: Guilford Press.
warranted. Thus, due to the conclusions that the purposes
of retribution and deterrence are not accomplished in the
execution of mentally retarded individuals, coupled with
the increased risk that the death penalty will be imposed Atomoxetine
erroneously; the U.S. Supreme Court ruled that the eighth
Amendment precludes execution of mentally retarded J OHN C. C OURTNEY
persons. Childrens Hospital of New Orleans
Despite the courts ruling, in July of 2005 a Virginia New Orleans, LA, USA
jury determined that Atkins was intelligent enough to be
executed due to the fact that another IQ score had been
recorded at above 70. Moreover, the prosecution claimed Generic Name
that his poor performance in school was related to use of
alcohol and drugs and that earlier assessments of his IQ Atomoxetine
Atrophy A 281
Class
Serious
Common
Additional Information
Atrophy. Figure 1 Display of diffuse atrophy of the cerebral
hemispheres. Note the shrunken gyri and prominent, widened
Drug Interaction Effects: http://www.drugs.com/drug_interactions.html
Drug Molecule Images: http://www.worldofmolecules.com/drugs/ sulci (Photo courtesy of Steven S. Chin, M.D., Ph.D., University
Free Drug Online and PDA Software: www.epocrates.com of Utah Health Sciences Center)
282 A Attendant Care
circumscribed regional cell loss. With atrophy, there adequate training of an attendant, and financial issues
is also a corresponding loss of neural connections including paying a competitive wage to personal
(synapses). Features of atrophy include sulcal widening, attendants. Funding sources of attendant care may include
shrunken gyri, and enlarged ventricles. Focal atrophy may private resources such as: Health Insurance; Auto Insur-
occur as a result of trauma or cerebrovascular lesions, for ance; and Workers Compensation; or public resources
example. Generalized atrophy may occur with neurode- such as: Medicaid; Department of Vocational Rehabili-
generative conditions such as Alzheimers disease. Atro- tation; Department of Veterans Affairs; Crime Victims
phy may be viewed on gross inspection of the brain Compensation and/or other State-funded programs.
postmortem or antemortem with structural imaging tech-
niques such as MRI or CT scan. Figure 1 displays diffuse
brain atrophy of the cerebral hemispheres viewed from Cross References
the top.
Assisted Living
Definition
Attention
Attendant care involves the provision of services to assist
individuals with mental and/or physical disabilities in the R ONALD A. C OHEN
performance and/or conduct of activities of daily living in Brown University
order to maximize community inclusion and indepen- Providence, RI, USA
dent living. The intent of attendant care is to promote
independence, participation, and quality of life of the
individual while also preventing medical problems. Typi- Synonyms
cally, the individual receiving attendant services is unable
to perform such tasks independently, or may perform them Concentration; Focus; Vigilance
with great difficulty. Attendant services include, but are not
limited to activities such as: bathing, dressing, feeding,
toileting, transferring, mobility, cooking, cleaning, Definition
laundering, cognitive assistance and monitoring. Services
may also relate to sustaining health such as dispensing Cognitive processes that enable the selection of, focus on,
medications etc. and sustained processing of information. The object of
Attendant care may be provided by a family member attention can either be environmental stimuli actively
such as a spouse, partner, sibling or parent, or by a hired being processed by sensory systems, or associative infor-
employee. Typically, attendant care is provided by persons mation and response alternatives generated by ongoing
who have been trained to provide the service/s within the cognitive activity.
home and/or community. The independent living model
of attendant care contends that individuals with disabil-
ities should be empowered, to the highest degree possible, Historical Background
to recruit, screen and hire, train and terminate their
respective personal attendants, thereby ensuring Attention is subjectively self-evident to all people, and
self-determination and choice. Additional considerations terms that referred to attention-type experiences have
related to the provision of attendant care include where been described by philosophers through the ages. The
and how to locate and access quality service providers, concept of attention is strongly linked in the philosophy
Attention A 283
to the nature of consciousness, self-awareness, and most cognitive science. In particular, the application of infor-
A
theories of the mind. Accordingly, attention has been mation processing models and signal detection methods
the subject of psychological inquiry from the beginning of to the study of communication led to a resurgence of
this scientific discipline. The writings of William James research interest in attention. This is not surprising con-
captured this fact, as evident from this well-known ex- sidering the fact that signal detection and selection, the
cerpt from his Principles of Psychology (1898). basic elements of almost all theories of selective attention,
are also central to information and communication theo-
" Everyone knows what attention is. It is the taking posses-
ry. This approach emphasized the probabilistic nature of
sion by the mind, in clear and vivid form, of one out of what
information detection and selectivity, a conceptual depar-
seem several simultaneously possible objects or trains of
ture from earlier psychophysical methods used to study
thought. Focalization, concentration, of consciousness are
perception. The application of information processing
of its essence. It implies withdrawal from some things in
approaches to the study of attention was a logical step, as
order to deal effectively with others
one of the primary problems for any communication or
While written over 100 years ago, this description very information processing system is reducing the total
succinctly captures essential aspects of the phenomena of amount of incoming signal to manageable levels to enable
attention, and remains apropos even today. The under- subsequent processing of this information.
standing of cognitive, behavioral, and neuropsychological Broadbent (1958) proposed the first formal model of
bases, influences, and effects of attention have dramatically selective attention based on the information processing
evolved since the time of William James. Yet, the under- theory. He maintained that attention occurs because there
lying subjective and behavioral experiences characterized is an information bottleneck as the large quantity of
by James and the other psychologists of his time remain environmental information that is available during paral-
largely consistent with current phenomenology of atten- lel processing is subject to channel capacity limitations
tion. Different types of attention were described, such later in the stream of processing due to serial processing
as directed, divided, focused, sustained, selective, and constraints. Broadbent posited that the primary require-
volitional attention, many of which continue to be used ment for attention to occur was a filtering process (as
to describe the varieties of attentional experience. The shown in Fig. 1) that occurred soon after sensory registra-
primary limitation of these early efforts was the lack of tion and served to separate relevant from irrelevant signals
experimentation that would have enabled operationaliz- in order to enable meaningful information to be available
ing of these constructs and understanding of the processes for subsequent serial processing through limited capacity
underlying them. information channels. This model presumed a somewhat
Following the initial efforts of early psychologists to passive system by which this filtering occurred, with se-
study attention from perspectives of structuralism and lection driven by the salience of the stimuli themselves.
functionalism, a rather long period ensued dominated by However, the nature of this filtering process was not fully
behaviorism during which cognitive processes, like atten- operationalized in this initial model. In the years that
tion, were largely viewed as outside of the realm of empiri- followed, a number of variations on this model of atten-
cal psychological inquiry. Attention was considered to be a tion were proposed by other investigators working in the
construct that could be explained by more basic behavioral newly emerging field of cognitive psychology.
principles, such as discrimination learning, cue domi- Most notably, Treisman proposed an attenuation theo-
nance, anticipation, and expectation. Classical condition- ry of attention, which was similar to Broadbents model in
ing theory provided an essential framework for behavioral which he postulated a single process that occurred early in
analysis of attention, as the orienting response to novel the information processing stream, soon after sensory reg-
stimuli, and its subsequent habituation, provided the istration (Treisman & Gelade, 1980). According to attenua-
behavioral and neural building blocks for simple forms tion theory, selective attention requires distinguishing
of attention, in the absence of long-term memory forma- between messages on the basis of their physical character-
tion (i.e., conditioning). The concepts of motivation and istics, such as location, intensity, and pitch, as well as
drive which played a major role in neobehaviorism, also content. In this model of attention, stimuli naturally differ
helped to bridge attention theory and learning principles. in their threshold for activating awareness of a stimulus,
Information theory (Shannon & Weaver, 1949), which and the process of attention effectively decreases (i.e.,
evolved out of technological advances in radio communi- attenuates) the strength (e.g., loudness) of irrelevant sti-
cation and also radar detection during World War II, was a muli. This attenuation process was considered to occur in
major impetus for the subsequent re-emergence of conjunction with a feature integration process that enabled
284 A Attention
Sensory
Messages Filter Detector To memory
store
Attended
message
perceptual experience. This line of research was noteworthy linkages between arousal, activation, and effort in the
for the use of dichotic listening paradigms in which atten- control of attention (Pribram & McGuinness, 1979).
tion is divided between the two ears, and information must A large body of cognitive studies of attention followed
be selected from one of the two channels of input. this pioneering work. Several of these are particularly
The bottleneck models proposed by Broadbent and important in a historical context. Posner (1979) made
Treisman proposed that selection occurs at a very early an important distinction between overt and covert shifts
stage of processing soon after sensory registration, thereby of attention that occur in the context of visual selective
linking attention squarely with sensory selection. Essen- attention. Overt attention is characterized by the act of
tially, selective attention filters inhibits focus on informa- intentionally directing attention (i.e., looking) toward a
tion occurring in the unattended ear in dichotic listening stimulus, whereas covert attention occurs without intention
experiments before semantic analysis and other cognitive when focus is drawn to a particular stimulus or location,
processes have time to occur. Other investigators (e.g., typically as a result of cues or other types of information of
Deutch and Deutch (1963) argued that attention is strong- which the person may have little conscious awareness. Pos-
ly influenced by the response demands of a situation and ner also refined the use of chronometric methods
that it likely occurred at a later stage of processing, and that to demonstrate the costs associated with these attention
in reality both ears analyze incoming information seman- shifts. His research also made a distinction between two
tically, though response demands creates a bias toward primary processes, selection and focus, that were necessary
one ear over the other. This led to heated debates in the to account attentions intensity and spatial distribution.
1960s and 1970s over the location of the bottleneck. While Shiffrin and Schneider (1979) conducted seminal stud-
considerable experimental evidence indicated that early ies that distinguished automatic from controlled attention.
sensory selection occurs prior to a point in time when They varied the number of targets to be detected and the
semantic information has been processed, there is also consistency of target location based on either fixed or
other paradigms that demonstrate that in most situations variable memory demands. By creating greater variability
selection is greatly influenced by semantics and the re- in task characteristics, subjects could not rely on memory
sponse requirements that exist. to facilitate performance, which slowed their response
Subsequent researchers took this a step further by time. Under these conditions, automaticity was no longer
demonstrating that capacity limitations constrain atten- feasible. Besides demonstrating the distinction between
tion and the intensity of attentional focus that is possible automatic and controlled attention, these findings also
at any given point in time. Kahenmans (1983) capacity illustrated the relationship between attention and memory,
theory of attention proposed that peoples capacity for and set the stage for a long line of research examining
attentional focus is not static, but instead varies as a working memory in relationship to attention.
function of factors such as the reward characteristics of
the task, arousal level, and other biological determinants.
This theory of attention was extremely important in that Neuropsychological Models and
it brought to the forefront the fact that attention should Frameworks
not be conceptualized in purely mechanical terms as was
the case in early attention models based solely on infor- Over the past 2 decades, research efforts have been direct-
mation processing theory. Rather attention needed to be ed at organizing these varieties of attention into coherent
viewed in the context of the biological factors that drive it. frameworks. Furthermore, researchers have proposed
This helped to catalyze an emphasis on the study of neuropsychological models of attention that seek to char-
focused attention, a shift that coincided with information acterize the functional neuroanatomic systems involved
coming from psychophysiological studies that showed in attention, the processes for which these systems are
Attention A 285
responsible, and also how these functional brain areas efficient use of available attention capacity and the inten-
A
interact. The models described below are not meant to sity of focus. Similarly, some tasks that are weighted more
be an exhaustive review of the literature in this regard, but demand for sensory selective attention, while others place
rather highlights some of the key elements of current greater demand on response intention and selection. In
theoretical frameworks and the extent to which there is other words, while these four components need to be
consistency across models. accounted for in explaining attention across all situations,
Alan Mirsky provided one of the first neuropsycho- particular tasks may require minimal demands for sus-
logical frameworks to account for what he described tained attention, but intense demands on capacity and
as the elements of attention. This framework pro- focus. Validation efforts directed at this framework have
posed five elements of attention: (1) selection, (2) focus, shown the principal factors to be highly reliable, internally
(3) execute, (4) switch, and (5) sustain. This theoretical consistent, and valid with respect to their weighting relative
framework was derived from factor analyses of neuropsy- to specific brain disorders and conditions. For example,
chological test results obtained from a large sample from patients with attention-deficit disorder have greatest im-
his clinical practice. pairment on tasks requiring sustained attention, whereas
Cohen (1993) proposed a similar component process patients with diminished speed of processing have greatest
framework of attention that hypothesized four primary problems on tasks requiring capacity and focus. It is note-
components of attention (Fig. 2): (1) sensory selective worthy that the analyses conducted by both Mirsky and
attention; (2) response intention, selection, and control; Cohen yielded very similar factor structures and validity
(3) capacity-focus; and (4) sustained attention. A primary data, providing strong evidence that four to five primary
goal of Cohens model was to include components reflect- components processes exist that account for most varieties
ing similar levels of analysis. The components of this frame- of attention. These attentional component processes are
work were also derived from factor analysis of clinical described in greater detail below.
neuropsychological data with efforts made to retain only
the minimum number of factors necessary to account for
maximum variance in the data, with an effort to make Selective Attention
conservative interpretation of the component processes
associated with each factor. Each component was hypothe- A fundamental aspect of all attentional processes is that it is
sized to be a function of other more basic subcomponent selective. Attention enables the selective deployment of
processes. This model posits that these four components of cognitive resources for the processing of information from
attention are not completely orthogonal or functionally either the external environment or internal cognitive pro-
independent, but instead rather share common component cesses or associative representations. Attention also requires
subprocesses, processes depending on the task at hand. a shift from less salient information. Processes that enable
A simplified version of the model is shown. or facilitate the selection of salient information for further
In everyday situations, attention depends on the inter- cognitive processing are collectively referred to as selective
action of all four of these component processes. However, attention (Treisman, 1969; Triesman & Geffen, 1967).
for some tasks, the primary demand may be for sustained Individuals are constantly flooded with an infinite num-
attention or vigilance, whereas for another task it may be ber of signals from both outside and within. By reducing
the amount of information that will receive additional switching. Not only do these processes account for the
processing, attention constrains incoming information control of simple motor responses, they also provide the
to the individuals available capacity at a given point in foundation for more complex cognitive processes, such as
time, thereby keeping the level of information to be pro- planning, problem solving, and decision making, as well as
cessed at a manageable level. While selective attention is conceptual processes such as categorization, organization,
necessary and beneficial for cognitive function, there and abstraction. Executive control is strongly dependent on
are costs associated with selectively attending. By attend- the actions of prefrontal-subcortical systems. Executive
ing to a particular stimulus, the likelihood of detecting control is dependent on the ability of the system to act
other potentially relevant stimuli or choosing an alterna- with intention, to initiate responding, to inhibit responding
tive response strategy is reduced. Optimal selective atten- based on new information, and to efficiently shift from one
tion depends on the system being flexible and adaptive, response alternative to another in accordance with chang-
with the capacity to select and focus on certain stimuli, ing environmental demands.
but then to shift to other stimuli or cognitive processing
when task conditions change. Selective attention thereby
serves as a gating mechanism for the flow of information Focused Attention and Capacity Limitations
processing and the control of behavior.
Attention is also characterized by having intensity and by
the extent to which it is allocated in either a focal or
Response Selection and Control diffuse manner. The intensity of attentional focus is a
function of both situational and task demands and organ-
Attention has traditionally been viewed as a process closely ismic factors, such as motivation and drive. Focused at-
related to perceptual processing. It prepares the individual tention is constrained by capacity limitations (Kahneman,
for sensory intake, perceptual analysis, and integration with 1973) that limit the intensity of focus that is possible on a
other cognitive processes. Yet, there are many situations in moment-by-moment basis. Attentional capacity is influ-
which attention is not directed at incoming sensory infor- enced by both structural and energetic factors (Cohen,
mation, but rather selection response alternatives, and the 1993). Energetic capacity limitations tend to be state
control of responding once a selection has been made. Even dependent and reflect the changing energetic conditions
when a task primarily requires selective attention, there of the brain, including motivation, and the incentives to
are usually coexisting response demands. While sensory attend that are present in the situation. Structural factors
selection may be automatically elicited by the occurrence tend to be more stable and dependent on each persons
of salient external stimuli, more often than not the act intrinsic information processing capacity. Factors that in-
of attending is linked to a planned, goal-directed course of fluence structural capacity include the processing speed
action. In this regard, attention and responding are directed capacity that is a function of the integrity of neural trans-
to obtain information that will optimize behavior. mission, memory encoding, storage and retrieval limita-
The processes associated with response selection and tions, and temporalspatial processing dynamics that vary
control range from simple behavioral orienting, such as across people. Given these factors that limit attentional
turning ones head in response to a sound source to more capacity, focused attention varies relative to the cognitive
complex cognitive processes involving intention, planning, demands and type of information to be processed, and
and decision making. Response selection and control form situational incentive. Focused attention can occur relative
the basis for what humans typically experience as volitional to either sensory selective attention, or intention and
action. Before responding, individuals generate a large response selection, and in fact often involves the coordi-
number of response alternatives. These response alterna- nation of sensory and response selection. Such coordina-
tives are evaluated prior to making an actual motor re- tion is quite effortful (Pribram & McGuinness, 1975).
sponse, leading to response bias, that is, the probability of Arousal and activation vary as a function of the existing
selecting specific responses. demands for focused attention, with greater activation
The attentional processes involved in response selec- occurring when there is more utilization of available
tion and control are related to a broader class of cognitive capacity because of the need to focus.
processes, commonly referred to as executive functions
(Fuster, 1989; Luria, 1966). Several processes associated Automatic Versus Controlled Attention
with response generation underlie executive control: in- An important distinction exists between automatic and con-
tention, selection, initiation, inhibition, facilitation, and trolled attentional processing (Schneider & Shiffrin, 1977;
Attention A 287
Hasher & Zacks, 1979). Automaticity occurs most com- brightest students would encounter tremendous dif-
A
monly in the context of sensory selective attention, par- ficulties sustaining their focus for a lecture that lasted
ticularly for tasks requiring simple detection of a target 12 consecutive hours.
from a set of stimuli, and also on tests of attention span. Vigilance refers to sustained attention directed toward
With automaticity, there is usually relatively little demand specific targets, in which a state of readiness is required to
placed on attentional capacity, and often attention can detect and respond to stimuli occurring at variable and often
occur without much awareness or subjective effort (e.g., infrequent intervals (e.g., Colquhoun & Baddeley, 1967;
attending to other cars while driving on an empty high- Corcoran et al., 1977). Detecting rare targets with lengthy
way). Automaticity can be interfered with increase in size intervals between responses can be difficult. This type of
and complexity of the environment to be attended to. sustained attention is quite common in everyday life. For
Spatial selective attention is particularly well suited for example, a watchman may spend the entire night attend-
automatic attentional processing since visual information ing to the possibility of an intruder without this event ever
typically occurs in parallel with a vast array of information occurring. Attention to low-frequency events has different
reaching the brain almost instantaneously. Automaticity processing requirements than responding to high-
is more difficult to achieve for tasks that require sequen- frequency events and, for many people, is more difficult.
tial cognitive operations, though some degree of automa- Vigilance and sustained attention are under the influence
ticity may be attainable through practice. Controlled of sustained motivational level, boredom, and fatigue,
attention is typically required for tasks in which there which are sensitive to the dynamics of temporal tasks.
are working memory demands, or other requirement of
other cognitive processes, such as memory encoding and
retrieval, rapid processing speed, or executive control. The Current Clinical and Experimental
demand for focused intensity varies as a function of require- Evidence
ments for controlled attention that exist for a particular
task. Generally, response intention, selection, and control Neuropsychological Studies
are not very amenable to automatic attentional processing,
as behavioral responding typically requires complex motor Twenty years ago the clinical and experimental neuropsy-
sequencing with executive control demands. However, the chological literature on impairments of attention associated
fact people are able to perform certain tasks such as typing with neurological and psychiatric disorders affecting the
or playing a musical instrument with considerable automa- brain was quite limited. Much of the neuropsychological
ticity illustrates that automaticity is attainable for well- focus on attention was on the assessment of attention span
learned motor programs. in the context of psychometric analysis of performance on
tests such as digit span. This probably reflected the fact that
adequate attention was once viewed more as a necessary
Sustained Attention condition for other cognitive functions to occur optimally,
but not particularly important in its own right in consid-
Attention varies as a function of the temporal dynamics of erations of brainbehavior relationships. This attitude has
the task to be performed and the situation, and all humans changed dramatically, and attention is now widely regarded
experience some degree of performance variability, par- as a critical cognitive process that reflects not only the
ticularly when long periods of sustained performance interface between both the external environment and inter-
are required. Sustained attention refers to processes that nal cognitive functions, but also moment-by-moment in-
enable the maintenance of performance over time. Com- formation processing. A literature review conducted about
pared to other cognitive processes, such as language and 2 decades ago revealed less than 500 studies focused on the
visual perception, attention is inherently variable by ne- neuropsychology of attention. Recent literature reviews
cessity, as it must be responsive to changing stimulus suggest that this number is now over 40, 000. This increase
conditions, task demands, and motivational and energetic in interest in attention reflects the fact that: (1) attention
states. Problems with sustained attention commonly occur disturbances are one of the most common by-products
on tasks requiring attentional persistence for long dura- of brain, (2) attention is closely tied to the human experi-
tions when there are high levels of demand for effortful ences of consciousness, awareness, and cognitive control,
processing. All people have limits in their capacity for (3) major advances have occurred in the methodology for
sustained attention. Sitting in a 1-hour lecture is not a studying and assessing attention, and (4) for a number of
problem for most bright college students, but even the reasons, there has been an increased societal interest in
288 A Attention
attention disturbances, perhaps in part because of the in- Alzheimers and Neurodegenerative Dementias
tense information processing demands and pace of life that Attention disturbance is usually not described as a prima-
people now experience. ry feature of Alzheimers disease (AD) and histori-
Impairments of attention may be either specific or cally tended to be viewed as one cognitive function that
nonspecific. Specific impairments occur when only aspect was largely spared. This conclusion is misleading. While
of attention is affected. Often this occurs when impaired patients with early-stage AD typically do not show overt
attention is directly associated with a particular type of symptoms of severe inattention, they frequently have
cognitive operation, such as spatial processing. These marked difficulty with focused attention and executive
impairments are often associated with focal brain distur- control, particularly when tasks required controlled atten-
bances affecting specific cortical or subcortical systems tional processing. This reflects a distinction between per-
necessary for the cognitive operation. Nonspecific distur- formance on tests of simple and complex attention, as
bances of attention are much more common, often occur- conclusions about spared attention in AD have often been
ring due to disorders that affect arousal, motivation, or based on the observation of preserved attention span on
other factors that reduce attentional capacity, and as a tests such as digit span. Patients with early AD are often
result of more diffused nonlocalized brain disorders. Both usually alert, energetic, and able to maintain their general
types of attentional disturbance provide insights into the focus on the assessment process. Yet, most will have con-
cognitive processes of attention and the brain mechan- siderable difficulty on tasks requiring focused and divided
isms that underlie these processes. Though localized attention, suppression of interference (e.g., Stroop), in-
lesions provide the best vehicle for analysis of the role of formation processing speed and efficiency (e.g., Symbol
specific brain structures in attentional control, nonspecific Coding), and working memory. As the disease progresses,
attentional impairments illustrate the influence of meta- performance becomes impaired on most tasks requiring
bolic and neurotransmitter abnormalities on information effortful attentional processing. Pervasive disturbance
processing rate, arousal, and other energetic and structural eventually develops affecting all aspects of attention,
factors that may affect attentional capacity and focus. including self-awareness.
Attentional capacity is a direct function of level of con-
sciousness, making this an essential part of the clinical
assessment of attention. Levels of consciousness range
Multiple Sclerosis
Multiple sclerosis (MS), one of the most common neu-
from normal states of alertness and awareness to coma.
rologic in young adults, often affects learning, memory,
A brief summary of the attentional disturbances asso-
and executive control. Given the fact that the disease
ciated with several common neurological and psychiatric
affects the myelin of the white matter, deficits in these
conditions is provided below. For more detailed consider-
areas are often strongly associated with attentional
ation see Cohen (1993).
impairments and slowed inefficient information
processing. Attentional capacity is typically reduced
Stroke and Neglect Syndrome
with performance decrements usually evident under
Unilateral stroke affecting the nondominant cortex often
conditions of increased informational load. Fatigue is
causes hemi-neglect syndrome, perhaps the most well
the most common of all symptoms in MS and is asso-
known and dramatic form of attention disturbance. The
ciated not only with motor effort but also with attend-
defining feature of neglect syndrome is the failure to attend
ing to and performing cognitive tasks. Patients with MS
to, respond to, or be aware of stimuli on one side of space.
experience difficulty maintaining consistent effort on
Many variants of neglect syndrome may be observed clini-
tasks.
cally. Most patients with neglect exhibit impairments of
sensory selective attention, although some may have pri-
mary problems with hemi-spatial response selection and HIV
control. Experimental investigations have confirmed the Similar to MS, HIV-infected patients who have not
role of attention in hemi-neglect syndrome. Manipulation developed severe AIDS dementia typically show primary
of attentional parameters demonstrates that symptoms of impairments in the areas of psychomotor and informa-
hemi-neglect change as attentional demands are modified tion processing speed, focused and sustained attention,
(Kaplan et al., 1989). Regardless of which attentional pro- and executive functioning. This reflects the fact that when
cess is most affected, all patients with neglect syndrome not adequately treated, HIV tended to initially have great-
have a fundamental disorder involving the spatial distri- est effects on subcortical systems, including the basal
bution and allocation of attention. ganglia.
Attention A 289
Closed Head Injury irrelevant stimuli and thoughts has long been considered to
A
The most common effects of closed head trauma are diffuse be a major element of the disorder, which has been linked to
axonal damage due to shearing forces and frontal lobe dis- the dopamine system. Schizophrenics often encounter dif-
turbances. Consequently, attention and executive dysfunc- ficulties on tests of sensory selective attention because of
tion are among the most common associated cognitive their susceptibility to distraction. Slowed reaction time and
problems. Persistent distractibility, poor concentration, apa- processing speed also contribute to problems with atten-
thy, and fatigability are prominent sequelae. Deficits of arous- tional capacity, and both focused and sustained attention
al and poor performance on measures of selective, focused, (Nuechterlein, 1977).
divided and sustained attention, processing speed, and exec-
utive functioning tend to occur, which may contribute to Attention-Deficit Disorder (ADD)
associated learning and memory retrieval problems as well. A developmental disorder of attention, ADD is the most
widely recognized of all attention disturbance. Problems
Epilepsy with sustained attention and distractibility are key features
Transient changes in the level and quality of conscious- of the disorder, along with hyperactivity among a subset
ness, common in seizure disorder, typically cause marked of children. While there is general agreement regarding
alterations in attention around the time of the seizure. the existence of ADD, there continues to be considerable
During the time between seizures, patients with epilepsy debate about its manifestations and pathophysiology, par-
may have greater problems than healthy individuals ticularly in light of the fact that ADD tends to occur along
on tests of focused and divided attention. These deficits with other comorbid conditions.
appear to be related in part to slowed speed of processing
and its effects on attentional capacity. Pharmacological
effects associated with anticonvulsant therapy likely con- Primate Studies
tribute in part to these attentional effects.
Understanding of the neural substrates of attention was
greatly enhanced by the use of neurophysiological meth-
Metabolic Disturbances ods in primates. The value of these studies is that they
Factors that affect the metabolic function often cause provided directed recording of electrical activity from
delirium, or more subtle alterations in attention and brain areas implicated in attention both by past clinical
arousal. Accordingly, metabolic disturbance is one of the studies of patients with neurological disorders and also
most common reasons for transient alterations in atten- ablation studies involving laboratory animals. In the
tion among people without other neurological or psychi- 1970s, Robert Wurtz, Michael Goldberg and their collea-
atric illness. Metabolic disturbances that affect the brain gues (1982) began electrophysiological studies from the
can be the result of a wide variety of factors, including brain of monkeys trained on specific attention para-
drug effects and systemic illnesses, such as liver and kid- digms. The earliest of these studies showed that the
ney disease, and diabetes. superior colliculus exhibits increased firing rates during
visual attention, providing the first direct evidence of the
Psychiatric Disorders involvement of a neural area in this process. Subsequent-
Difficulties with focused and sustained attention are ex- ly, a large number of studies were conducted that showed
tremely common among patients with psychiatric disor- the contribution of other brain regions, particularly in
ders, including affective disorders (major depression and posterior visual areas to specific aspects of visual selec-
bipolar disturbance) and schizophrenia. Severe anxiety tive attention. This work both confirmed the role of areas
states can also interfere with attention. A strong relationship like the inferior parietal cortex that had been suspected
exists between expenditure of effort and performance on of being involved in visual selective attention based on
tests of attention and other demanding cognitive functions studies of hemi-neglect syndrome. Over time, there has
for patients with major affective disorders. Impairments been increasing emphasis on the role of frontal brain
tend to be somewhat proportional to severity of depression, systems in relationship to these posterior brain areas.
with performance improving when the depression resolves. There is now a large body of research on this topic,
Diminished attentional capacity is particularly evident on supporting to general conclusions about selective atten-
tasks that require psychomotor speed, attentional focus, tion: (1) Visual selective attention is controlled by mul-
and effortful demands for mental control. Abnormal atten- tiple interacting brain areas that comprise a functional
tion is also a central feature of schizophrenia, as filtering of system. (2) Selective attention involves not only
290 A Attention
posterior visual brain areas, but also frontalstriatal sys- and attention usually must be assessed within the context
tems that provide executive response control. Active of performance on tasks that load on one or more these
investigation continues using primate models with par- other domains. Attentional performance is often assessed
ticular emphasis on source analysis of how particular as derived measure obtained by comparing performance
types of neurons are tuned to optimize attention to across tasks that control for key attentional parameters
particular types of signals. This research has been instru- (e.g., targetdistractor ratio). Absolute performance often
mental in characterizing the functional brain systems provides less informative measures of performance incon-
governing attention in humans. sistencies in the assessment of attention. For example,
how performance varies as a function of time, spatial
characteristics, or memory load provides more informa-
Functional Neuroimaging tion about attentional dynamics than simply considering
total errors on a visual detection task. Since attention
Attention was one of the first cognitive functions to be is not the by-product of a unitary process, or a single
demonstrated through the use of functional imaging meth- sensory modality, it cannot be adequately assessed on the
ods, such as functional MRI and PET. The fact that atten- basis of findings from one specific test. For example,
tional parameters can be easily manipulated in the context conclusions based on digit span performance are mis-
of the scanner and that attention reflects the moment-by- guided. Attentional assessment requires a multifactorial
moment information processing of the brain makes it very approach. The specific attention measures used in an
conducive to study through functional brain imaging. evaluation depends on the overall level of functioning of
These efforts have largely confirmed the involvement of the particular patient. For patients with global cognitive
inferior parietal and frontal brain systems in attention, dysfunction, it may be difficult to use certain tasks that
with studies showing the relative contribution of specific require overly complex responses. For patients with rela-
areas in selective, focused, and sustained attention. This is tively high overall cognitive abilities, tasks should be cho-
a rapidly growing area of neuropsychological inquiry. To sen that require multiple component processes. If the
date, results of functional neuroimaging experiments have patient is able to perform well on these tasks, then severe
largely supported evidence from earlier cognitive, neuro- attention disturbance involving specific attentional com-
psychological, psychophysiological, and primate studies ponent processes can be ruled out. The Stroop and Trail-
with respect to the neural substrates of attention. Making tests are examples of tasks that require multiple
attentional processes. If impairments are found on such
tasks, then more extensive testing of specific component
Clinical Assessment Considerations processes can be conducted. When possible, efforts should
be made to use tasks that incorporate signal detection
Although an essential cognitive process, attention is more methods, even when not evaluating sensory selective
difficult to directly observe or measure than other cogni- attention per se. This methodology provides the best
tive functions like language, visual perception, or memory. means of accurately summarizing performance relative
Attention fluctuates in accordance with changes in task to all types of possible errors, and easily integrates with
demands and the processing capacity of the patient over response time measures.
time. Unlike other cognitive functions, performance may Attentional parameters that should be considered.
be quite different across different points in time, and it is A thorough assessment of attention should be based on
this variability that in fact defines attention. Attention is analysis of data from a comprehensive battery of atten-
often situation specific. This accounts for why some chil- tional tests that sample underlying component processes
dren with ADD perform well in a controlled laboratory (Cohen, 1993). Accordingly, tasks should be used that are
setting, despite reports of gross problems with inattention differentially sensitive to the following attentional para-
in school or the home. meters: (1) spatial characteristics, (2) temporal dynamics,
Unlike most other cognitive processes, attention pri- (3) memory demands, (4) processing speed requirements,
marily serves a facilitative function. Attention enhances (5) perceptual complexity, (6) demands for response
or inhibits perception, memory, motor output, and exec- sequencing and control, (7) cognitive complexity of the
utive functions, including problem solving. Yet, attention task, (8) effort required to complete task, and (9) task
is always measured as a function of performance on tasks salience, relevance, and reward value.
that also loads on one or more of these other cognitive While multifactor neuropsychological assessment
domains. Therefore, pure tests of attention do not exist, provides the best means of evaluating attentional
Attention A 291
impairments, a comprehensive attentional evaluation Colquhoun, W. P., & Baddeley, A. D. (1967). Influence of signal proba-
bility during pretraining on vigilance decrement. Journal of Experi- A
may not be feasible in everyday clinical practice, because
mental Psychology, 73, 153155.
of time constraints, the patients overall severity of cogni- Desimone, R., & Gross, C. G. (1979). Visual areas in the temporal cortex
tive impairment, or the fact that other cognitive functions of the macaque. Brain Research, 178, 363380.
must be assessed in greater detail because of the referral Deutsch, J. A., & Deutsch, D. (1963) Attention: Some theoretical con-
questions. Consequently, clinicians should be aware of the siderations. Psychological Review, 70, 8090.
Fuster, J. M. (l989). The prefrontal cortex: Anatomy, physiology, and neu-
information that can be obtained from different levels of
ropsychology of the frontal lobe. New York: Raven.
attentional assessment. A few primary tests of attention Goldberg, M. E., & Bushnell, M. D. (l981). Behavioral enhancement of
should be included in all neuropsychological evaluations. visual response in monkey cerebral cortex. II. Modulation in frontal
The continuous performance test (CPT) paradigm pro- eye fields specifically related to saccades. Journal of Neurophysiology,
vides an excellent measure for assessing sustained atten- 46, 783787.
Hasher, L., & Zacks, R. T. (1979). Automatic and effortful processes in
tion and other related indices. Tests of focused and
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selective attention are also now widely available. Several Heilman, K. M., Watson, R. T., & Valenstein, E. (l993). Neglect and
attention batteries have also been developed that may related disorders. In K. M. Heilman & E. Valenstein (Eds.), Clinical
facilitate the comprehensive assessment of the elements neuropsychology (3rd ed., pp. 279336). New York: Oxford Universi-
of attention. ty Press.
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Directional hypokinesia in neglect. Neurology, 2(33), 104.
Heilman, K. M., Watson, R. T., Valenstein, E., & Goldberg, M. E. (1988).
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Real-time functional brain-imaging methods will enhance York: Henry Holt.
Kahneman, D. (1973). Attention and effort. Englewood Cliffs, NJ:
the ability of neuropsychologists in the future to assess
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moment-by-moment variations in attention associated Kahneman, D., & Treisman, A. (1984). Changing views of attention and
with task performance. There continues to be the need automaticity. In R. Parasuraman & D. R. Davies (Eds.), Varieties of
to attentional batteries that are theoretically coherent and attention. New York: Academic.
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model of neglect. Journal of Neurology, Neurosurgery & Psychiatry,
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unilateral neglect. Archives of Neurology, 10, 304325.
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Directed Attention nation. In R. Parasuraman & D. R. Davies (Eds.), Varieties of atten-
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Habituation
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Psychological Review, 84, 166.
Cohen, R. A. (1993) Neuropsychology of Attention. New York: Plenum. Shannon, C. E., & Weaver, W. (1949). The mathematical theory of com-
Cohen, R. A., Meadows, M. E., Kaplan, R. F., & Wilkinson, H. (1994) munication. Urbana, Illinois: The University of Illinois Press.
Habituation and sensitization of the OR following bilateral cingulate Treisman, A., & Gelade, G. (1980). A feature-integration theory of atten-
damage. Neuropsychologia, 32(5), 609617. tion. Cognitive Psychology, 12, 97136.
292 A Attention Deficit Disorder
Verfaellie, M., Bowers, D., & Heilman, K. M. (1988). Attentional factors accounting for roughly half of the ADHD cases. ADHD-
in the occurrence of stimulus-response compatibility effects.
H/I is far less common, is most often observed in pre-
Neuropsychologia, 26, 435444.
Watson, R. T., Heilman, K. M., Cauthen, J. C., & King, F. A. (1973).
school and early elementary school aged children, and is
Neglect after cingulotomy. Neurology, 23, 10031007. probably just the earlier developmental stage to the C-
Wurtz, R. H., Goldberg, M. E., Robinson, D. L. (1982). Brain mechanisms type in many instances. In general, hyperactive-impulsive
of visual attention. Scientific American, 246(6), 124135. symptoms decline more steeply as children age (although
feelings of restlessness may persist), but inattentive symp-
toms remain relatively constant.
Children with ADHD-H/I and ADHD-C are at higher
Attention Deficit Disorder risk for conduct problems. Youth with ADHD-I are at
higher risk for learning disorders, anxiety and possibly
Attention Deficit, Hyperactivity Disorder depression. While some argue that ADHD-I is a distinct
disorder from ADHD-C and ADHD-HI, others have not
found consistent differences between the subtypes on
neuropsychological or laboratory measures. Though diag-
nosed as a categorical disorder, ADHD may actually rep-
Attention Deficit, Hyperactivity resent an extreme end along a normal continuum for
Disorder the traits of attention, inhibition and the regulation of
motor activity.
K EVIN M. A NTSHEL , R USSELL B ARKLEY
State University of New York - Upstate Medical University
NY, Mount Pleasant, SC, USA Epidemiology
conduct disorder such as lying, stealing, fighting and household and maintaining health are also consistently
A
otherwise violating the rights of others. Major depressive reported in adults with ADHD. For example, employer
disorder (2030%) and anxiety disorders (1030%) are ratings are lower for adults with ADHD and adults with
also relatively common co-morbid conditions in pediatric ADHD have more part-time employment and change
ADHD. jobs more often.
Longitudinal research following children with ADHD There are some data to suggest that ADHD is more
into adulthood suggests that 5086% of children with functionally impairing than most other outpatient psychi-
ADHD continue to show impairing symptoms as they atric disorders in these domains. While the relationship
age. The fact that some children do not continue to have between ADHD symptoms and impairment in children
an ADHD diagnosis may be due in part to the finding that with ADHD is modest (r = 0.3), these relationships may
ADHD symptoms decline as a function of age in typically be more robust in adults (r = 0.7).
developing populations. However, it may also simply reflect
that DSM symptoms and symptom thresholds may be
developmentally inappropriate and too restrictive, respec- Neuropsychology and Psychology of
tively, to be applied to outside of childhood. For example, ADHD
DSM-IV inattentive symptoms are more common in ado-
lescents than DSM-IV hyperactive/impulsive symptoms. A meta-analysis suggested that children with ADHD have
While this may infer that inattention persists at higher levels an IQ about nine points lower than typically developing
than hyperactivity/impulsivity, it may also simply reflect peers. Similar data have been reported in adults with
the developmental insensitivity of the DSM-IV symptoms. ADHD. Lower performance on the WAIS-III Arithmetic
Genetics also appear to be a large factor in those who and Digit Symbol may account for a substantial portion of
continue to demonstrate clinically significant ADHD the IQ differences noted between adults with ADHD and
post-childhood versus those whose symptoms are in re- community controls.
mission. For example, prevalence rates of ADHD among Controlled processing deficits are commonly observed
the relatives of children with persistent ADHD are signifi- in pediatric ADHD. Children with ADHD perform less well
cantly higher than rates in relatives of children with re- on laboratory tasks that assess vigilance, motoric inhibition,
mitted ADHD. In addition, a history of major depressive organization, planning, complex problem solving, and ver-
disorder before age 13 is a predictor of the syndromic bal learning and memory. Adults with ADHD are impaired
persistence of ADHD into adolescence as is having a on these same cognitive domains.
below average IQ. Both children and adults with ADHD perform less well
By definition, individuals with ADHD need to be on tasks that require vigilance, or the ability to sustain
functionally impaired in two or more domains of major attention. There is also some evidence that rare target
life activities. In children, academic, social and family paradigms (few targets, many non-targets) such as the
functioning domains are the most frequently impaired Gordon Diagnostic System appear to be more difficult
(MTA Collaborative Group, 1999). Educational impair- for adults with ADHD than those paradigms with higher
ments including academic underachievement and learning signal probabilities.
disabilities are well documented in the pediatric ADHD Response inhibition has been hypothesized to play a
literature. central role in pediatric ADHD. Continuous performance
In adolescents and young adults, academic impair- test (CPT) commission errors are a common laboratory
ments continue to persist; young adults with ADHD com- measure of this construct. Unlike attention deficits (which
pleted fewer years of education, with nearly 1/4 failing to seem to emerge more reliably in rare target CPTs), re-
complete high school. Others have similarly reported that sponse inhibition deficits emerge more reliably in higher
relatively few young adults with ADHD attempt college signal probabilities such as the Conners CPT. Several
(20%) and even fewer graduate (5%) from college. Com- studies have reported that adults with ADHD make
pared to children with ADHD who are followed into more errors of commission on high signal CPTs relative
adulthood, clinically diagnosed adults with ADHD appear to both clinical and community control participants.
to have higher intellectual levels, have graduated from high Executive functioning deficits are present in both pediat-
school and have at least attempted college. ric and adult ADHD. Thus, it is surprising that performance
In addition to educational impairments, impairments on one of the most well established tests of executive func-
in domains such as occupational, dating/marital relations, tioning, the Wisconsin Card Sorting Test is not impaired in
financial management, driving, child-rearing, managing a adults with ADHD. Multiple studies have failed to report a
294 A Attention Deficit, Hyperactivity Disorder
significant difference between adults with ADHD and com- which normative data are available. These include broad
munity controls on WCST categories completed and num- band questionnaires, such as the Behavioral Assessment
ber of errors, both perseverative and non-perseverative. System for Children 2nd edition or Child Behavior Che-
Verbal fluency is impaired in adult ADHD. The most cklist for screening the major dimensions of childhood
widely used verbal fluency task in adult ADHD popula- psychopathology (e.g. anxiety, depression, attention,
tions has been the Controlled Oral Word Association Test. hyperactivity, aggression, etc.). Narrow band question-
Multiple studies have reported significant differences be- naires specifically evaluate the symptoms of ADHD as set
tween community controls and adults with ADHD. forth in DSM-IV. Rating scales can reliably, validly and
Given the importance of attention and working mem- efficiently measure DSM-IV-based ADHD symptoms.
ory to memory encoding and storage, it is not surprising Some examples of instruments demonstrating appropriate
that adults with ADHD have been demonstrated to have psychometric properties with a strong normative base
memory deficits. They also appear to have more difficulty include the ADHD Rating Scale IVand the Conners Rating
managing auditory/verbal information relative to visual Scales 3rd edition.
information. Although differences emerge between adults A number of specific tests have been devised to provide
with ADHD and community controls on the WAIS-III objective measures of a subjects vigilance and impulse
Digit Span, the effect size of the differences are much control, such as the Gordon Diagnostic System, Conners
larger on the California Verbal Learning Test. For exam- Continuous Performance Test or the Test of Variables of
ple, adults with ADHD perform less well on overall rates Attention, among others. Research suggests, that these tests
of learning, recall, recognition and semantic clustering. are not especially accurate at classifying children as ADHD;
The weaker performance on the semantic clustering index while the presence of abnormal scores on such tests indi-
may indicate failure to adopt a strategy. cates the presence of a disorder in as many as 90% of
children who perform poorly, such scores cannot indicate
the specific disorder present. Moreover, the ecological va-
Evaluation lidity of these tests is low thus precluding the ability to
predict from the test scores how the child will function in
The American Academy of Child and Adolescent Psychia- more natural settings, such as home and school. These tests
try (Pliszka, 2007) has established guidelines for the are therefore not recommended for routine diagnostic eva-
assessment and treatment of ADHD. No neurological, luations of children with ADHD, although they may be
genetic, neuropsychological or behavioral tests have used in clinics specializing in ADHD as part of research or
sufficient positive and negative predictive power to accu- drug trials. More useful information is likely to be obtained
rately classify ADHD cases with sufficient success to recom- from the parent and teacher rating scales discussed above.
mend them for clinical diagnosis. Clinical diagnosis is based
largely on careful history taking, use of structured interviews
containing DSM-IV criteria for ADHD and related disor- Treatment
ders, and the expert knowledge of the clinician in the differ-
ential diagnosis among childhood mental disorders. Treatment for ADHD in children typically involves three
Paramount in the evaluative process is the time to listen to components: parent and child education and support,
parental concerns, probe for details concerning nature, classroom accommodations, and medication. Substantial
onset, and course, elaborate the specific impairments result- evidence exists to show that training parents in child
ing from these concerns, and place them in the larger behavior management skills can be of significant benefit
framework of the clinical taxonomy of mental disorders. in the reduction of parentchild conflict and improvement
The clinical interview is then supplemented with the use of in child success within the home (MTA Collaborative
parent and teacher behavior rating scales to assess develop- Group, 1999). The school setting frequently requires ad-
mental deviance of symptoms, screening of intelligence and justment to meet the special needs of the child with
academic achievement skills by standardized testing, brief ADHD. School interventions often include alterations to
observation of the child during unstructured and structured the curriculum and work load to better mesh with the
activities, contact with school personnel concerning class- limited attention, persistence, and disorganization of the
room functioning and compilation of prior school and child with ADHD; increases in sources of positive rein-
mental health records available on the child. forcement for work productivity; occasional use of imme-
Other sources of information essential for the diagnos- diate and systematic negative consequences for disruptive
tic process are behavioral rating scales or checklists on or inappropriate behavior; implementation of a daily
Attention Deficit, Hyperactivity Disorder A 295
school behavior report card (the ratings on which are What constitutes the psychosocial component, however,
A
linked to a home token economy). appears to be somewhat different in adult ADHD relative
The mainstay of treatment for many children with to pediatric ADHD. For example, neither cognitive behav-
ADHD is medication, frequently psychostimulants. Three ioral therapy (CBT) nor cognitive therapy has much re-
classes of medication appear to be useful for management of search support in pediatric ADHD. Nonetheless, there are
ADHD, these being: psychostimulants (methylphenidate, some data to suggest that CBT may be more efficacious in
amphetamines), noradrenergic reuptake inhibitors (ato- adults with ADHD. For example, in the adult ADHD
moxetine), and antihypertensive medications (clonidine, literature, there is some evidence that CBT is efficacious
guanfacine). for reducing functional impairments in adults concur-
Stimulant medications, especially extended release rently treated with stimulants.
formulations, are a front-line management strategy in
pediatric ADHD; approximately 70% of children with
ADHD will show an efficacious response to stimulant
Cross References
medications such as Methylphenidate or mixed-salt amphe-
tamines. The side effects of stimulants are fairly benign,
Americans with Disabilities Act
short-lived, dose related, and often managed through dose
Attention; Attention/Executive Functions
or timing adjustments, or by switching to a different deliv-
Atomoxetine; Behavior Assessment System for
ery system or stimulant.
Children (BASC)
Atomoxetine is a nonstimulant approved for man-
Cognitive Behavioral Therapy
agement of ADHD. Atomoxetine is an exclusive norad-
Conners 3rd edition
renergic reuptake inhibitor and is the first drug indicated
Continuous Performance tests
for ADHD that is not a Schedule II controlled substance
D-amphetamine
with low potential for abuse, making it more convenient
Executive Functioning
than the stimulants for sampling, prescribing, and titrat-
Individuals with Disabilities Education Act
ing. Clonidine and guanfacine are alpha2-noradrenergic
Learning Disability
agonists that have some effectiveness for the manage-
Metacognition
ment of hyperactive-impulsive ADHD symptomatology.
Methylphenidate
They are also considered off-label for treatment of
Section 504 of the Rehabilitation Act of 1973
ADHD as they have not as yet been specifically
Stimulants
approved by the FDA for treatment of ADHD. An extend-
Working Memory
ed release form of guanfacine received FDA approval
in 2009.
In adults, stimulant medications are effective in ap-
proximately 70% of individuals with ADHD. Atomoxe- References and Readings
tine may be particularly effective for adults with ADHD
and co-morbid depression or for those with a co-morbid Barkley, R. A. (2006). Attention deficit hyperactivity disorder:
substance use disorder. A handbook for diagnosis and treatment (3rd ed.). New York:
Guilford Press.
Managing psychiatric co-morbidity is a significant
Barkley, R. A., & Murphy, K. R. (2006). Attention deficit hyperactivity
component of pediatric ADHD. The same dictum appears disorder: A clinical workbook (3rd ed.). New York: Guilford.
central to managing ADHD in adults. While uncompli- MTA Collaborative Group. (1999). A 14-month randomized clinical
cated ADHD exists in about 25% of the adults with trial of treatment strategies for attention-deficit/hyperactivity
ADHD, most adults with ADHD have significant psychi- disorder. The MTA Cooperative Group. Multimodal treatment
study of children with ADHD. Archives of General Psychiatry, 56
atric co-morbidity that requires clinical attention and
(12), 10731086.
management. One aspect in which the psychiatric co- Nigg, J. (2006). What causes ADHD: Understanding what goes wrong and
morbidity is evident in treatment strategies is phar- why. New York: Guilford.
macotherapy. Although the evidence for the efficacy of The ADHD Report, a bimonthly newsletter for clinicians edited by
polypharmacy is limited at this time, multiple researchers Dr Barkley with contributions from leading clinicians and research-
ers. Guilford Publications, New York.
have asserted that polypharmacy may be more likely in
Pliszka, S. (2007). Practice parameter for the assessment and treatment of
adult ADHD than pediatric ADHD. children and adolescents with attention-deficit/hyperactivity disor-
Similar to pediatric ADHD, a psychosocial treatment der. Journal of the American Academy of Child and Adolescent Psychi-
component is typically recommended in adult ADHD. atry, 46(7), 894921.
296 A Attention Network Test (ANT)
is printed (e.g., red ink) when the word itself spells out a
Attention Network Test (ANT) different color (e.g., BLUE). Brain areas implicated in the
executive attention system include frontal midline regions
M ICHAEL S. WORDEN such as the anterior cingulate cortex and the lateral
Brown University prefrontal cortex. The neurotransmitter dopamine is
Providence, RI, USA important in the functioning of this network.
The Attention Network Task (ANT) was developed by
Jin Fan, Michael Posner, and colleagues at the Sackler
Attention is often subdivided by researchers into a Institute for Developmental Psychobiology. Using subtrac-
number of separate systems. Although there is certainly tive methodology, the ANT is designed to assess each of
some interaction between them, these systems play differ- these three attentional networks using a single reaction-
ent roles in terms of their effect on information processing time paradigm. The fundamental task of the participant is
and the control of behavior. Further, there is evidence that simple. On each trial, the participant looks at a small
different attentional systems are associated with different, fixation cross in the center of a computer screen and a
largely nonoverlapping brain regions and rely to a large small arrow, called the target, is briefly displayed either
extent on different neurotransmitter systems. One such above or below the fixation. The participant is required to
framework advanced by Michael Posner and colleagues respond by pressing one of two buttons as quickly and
defines three separate attention systems or networks: accurately as possible indicating whether the arrow is
alerting, orienting, and executive control. pointing to the left or right. On some presentations, the
The alerting system is responsible for helping the target is preceded by a briefly presented cue stimulus while
organism reach and maintain an alert state. This state, on other trials it is presented with no advanced warning.
which is separate from arousal, is characterized by a These cues are either predictive or non-predictive. Orient-
readiness to perceive and process incoming stimuli. The ing cues are presented either above or below the central
alerting system has been associated with superior fixation and indicate the location at which the upcoming
parietal, right frontal, and thalamic brain regions and arrow will be shown. Non-orienting cues are presented
the norepinephrine neurotransmitter system. either at the center of the screen or else both above and
The orienting system is responsible for selecting and below fixation simultaneously. Both types of cues indicate
giving preference to specific sensory information, often in that the target is about to appear but only orienting cues
terms of spatial location. Attentional orienting in space provide information regarding the location of the
may be done overtly by, for example, moving the head or impending target. Finally, in some cases, the target
covertly, that is, without moving the eyes or head. For arrow is flanked on either side by other stimuli. These
example, a football player might look down the field with flanking stimuli may be arrows pointing in the same
his eyes while attending covertly to the location and move- direction as the target (called congruent trials) or they
ments of other players in his peripheral vision. Attended may be arrows pointing in the opposite direction as the
items are generally processed faster and more accurately target (called incongruent trials).
than nonattended items. Brain areas that have been linked The efficiency of the three attention networks may be
to the orienting system include areas of the parietal cortex assessed independently for each participant by use of the
and the frontal eye fields, and the cholinergic neurotrans- subtractive method. Both reaction time and accuracy may
mitter system appears to play an important role. be examined. To assess the alerting network, scores from
The executive attention system is involved in monitor- trials in which no cue was presented are compared to
ing ones performance in the context of current task scores from trials in which there was a cue presented.
demands and providing control signals that help other The difference in mean reaction times between these two
systems adapt to changing contexts and conflicting trial types constitutes an efficiency score for the alerting
information. Executive attention is especially important network and is indicative of the extent to which the
for detecting and responding to situations in which there alerting network was able to use the information provided
is stimulusresponse conflict. Such conflict arises when by the cue to improve behavioral performance. In a
two or more stimuli or two or more aspects of the same similar manner, an efficiency score for the orienting
stimulus are associated with different behavioral network may be derived by subtracting mean scores
responses. A common example is the Stroop task in from trials with orienting cues from the scores for trials
which subjects are presented with printed words and with non-orienting cues. Both of these trial types include
they must name the color of the ink in which the word a cue so there should be no difference in terms of a
Attention Training A 297
contribution from the alerting network. The difference in Fan, J., McCandliss, B. D., Fossella, J., Flombaum, J. I., & Posner, M. I.
(2005). The activation of attentional networks. Neuroimage, 26(2), A
scores measures the degree to which the orienting system
471479.
could take advantage of the predictive cues to orient to a http://www.sacklerinstitute.org/users/jin.fan/
specific spatial location. In the case of the non-orienting Posner, M. I., & Rothbart, M. K. (2007). Research on attention networks
cues, the participant could not predict whether the target as a model for the integration of psychological science. Annual
would appear above or below the fixation point and Review of Psychology, 58, 123.
therefore could not improve performance by orienting
to one or the other spatial location. An efficiency score
is derived for the executive attention system by comparing
scores on trials with congruent flankers to trials with Attention Process Training
incongruent flankers. Subjects will tend to be slower and
less accurate for incongruent trials than for congruent Attention Training
trials, and the size of these differences indicates the extent
to which the individual is able to suppress conflicting
response tendencies.
A number of intriguing findings have come from Attention Span
studies that have utilized the ANT. Supporting the notion
that the three attention networks assessed by the ANT Span of Apprehension
constitute independent systems, a large-scale study of
over 200 individuals found that there was very little cor-
relation in efficiency scores among the three networks. In
other words, the particular score of an individual on any
Attention Training
one of the three attention networks does not tend to
predict that individuals scores on the other two networks.
M C K AY M OORE S OHLBERG
A high efficiency score for the alerting network, for exam-
University of Oregon
ple, does not suggest what ones scores are likely to be for
Eugene, OR, USA
either the orienting or executive attention networks.
Using electroencephalographic (EEG) recordings from
the surface of the scalp, it was found that each of these
Synonyms
three attention systems is associated with distinct patterns
of neural oscillations. A number of variations on the
Attention process training, direct attention training,
original ANT have been developed to address specific
process training
questions and for the study of special populations. For
example, child-friendly versions of the ANT that use
cartoon pictures of fish instead of arrows have been used
Definition
to study the development of attention systems.
Attentional deficits are a hallmark of many psychiatric
Attention training is based on the premise that attentional
and neurological disorders. The ANT has been used to
abilities can be improved by activating particular aspects
assess the relative impact of many disorders on the differ-
of attention through a stimulus drill approach. The
ent attention systems and to help distinguish between or
repeated stimulation of attentional systems via graded
establish subtypes of particular disorders. Among others,
attention exercises is hypothesized to facilitate changes
variations on the ANT have proven useful in the study
in attentional functioning. Most attention training
of attention deficit hyperactivity disorder, Alzheimers
programs assume that aspects of cognition can be isolated
disease, autism, borderline personality disorder, traumatic
and discretely targeted with training exercises.
brain injury, substance abuse, and schizophrenia.
Fan, J., McCandliss, B. D., Sommer, T., Raz, A., & Posner, M. I. (2002).
The aspects of attention that are trained vary widely
Testing the efficiency and independence of attentional networks. among interventions and frequently depend upon a
Journal of Cognitive Neuroscience, 14(3), 340347. theoretical model of attention. Attention models,
298 A Attentional Response Bias
regardless of their operational framework, appear to in- Cicerone, K. D., Dahlberg, C., Kamar, K., Langenbahn, D. M., Malec, J. F.,
Bergquist, T. F. et al. (2000). Evidence-based cognitive rehabilitation:
clude functions related to sustaining attention over time
Recommendations for clinical practice. Archives of Physical Medicine
(vigilance), capacity for information, shifting attention, & Rehabilitation, 81, 316321.
speed of processing, and screening out distractions. Some Galbiati, S., Recla, M., & Pastore, V. (2009). Attention remediation
attention efficacy studies evaluate attention interventions following traumatic brain injury in childhood and adolescence.
that focus on particular attention components such as Neuropsychology, 23(1), 4049.
Park, N. W., & Ingles, J. L. (2001). Effectiveness of attention rehabilitation
reaction time and sustained attention for visual informa-
after acquired brain injury: A meta-analysis. Neuropsychology, 15,
tion (e.g., Ponsford and Kinsella, 1988). Other efficacy 199210.
studies use attention training programs that include Park, N. W., Proulx, G., & Towers, W. M. (1999). Evaluation of the
hierarchical tasks to address a continuum of attention attention process training programme. Neuropsychological Rehabili-
components from basic sustained attention to more com- tation, 9, 135154.
Sohlberg, M. M., Avery, J., Kennedy, M., Ylvisaker, M., Coelho, C.,
plex mental control (e.g., Park, Proulx, & Towers, 1999;
Turkstra, L., & Yorkston, K. (2003). Practice guidelines for direct
Sohlberg, McLaughlin, Pavese, & Heidrich, 2001). attention training. Journal of Medical Speech Language Pathology,
Evidence supports the effectiveness of attention train- 11(3), xixxxxix.
ing beyond the effects of nonspecific cognitive stimulation Sohlberg, M. M., McLaughlin, K. A., Pavese, A., Heidrich, A., &
for patients with traumatic brain injury or stroke during Posner, M. (2001). Evaluation of attention process training and
brain injury education in persons with acquired brain injury. Journal
the postacute phase of recovery and rehabilitation (Butler,
of Clinical and Experimental Neuropsychology, 22, 656676.
2008; Cicerone et al., 2000). Evidence-based practice
guidelines for attention training were generated from
examination of the intervention research literature
(Sohlberg et al., 2003). Analysis of nine Class I and Class
II studies suggested that certain aspects of attention train- Attentional Response Bias
ing are helpful in improving attention performance in
some adults with traumatic brain injury. Treatment R ONALD A. C OHEN
parameters found to influence positive outcomes includ- Brown University
ed high frequency of attention training, combining Providence, RI, USA
attention training with metacognitive training (e.g, self
monitoring and strategy training), and individualizing
training to match the clients attention profile. The effects Definition
of attention training may be relatively small or task-
specific, and the research encourages clinicians to actively Attentional response bias refers to the tendency to re-
facilitate and monitor the impact of attention training on spond to the targets of attention on a particular task or
functional, everyday activities. in a given context. This tendency is often operationalized
using signal detection theory based on possible error types
that can occur; that is, either misses (errors of omission)
Cross References or false positive (errors of commission). The proportion
of these types of errors can be expressed as an index
Attention corrected relative to the total number of errors of all
Attention/Executive Functions types made (b: beta). A person who makes a much larger
Neuropsychological Rehabilitation number of errors of commission than errors of omission
Plasticity (misses) is exhibiting a response bias toward responding
Process Training by indicating they detected the target even when it was not
presented, which in many cases reflects excessive impul-
sivity or inhibitory control problems. Conversely, a ten-
dency to miss targets, but to rarely make false positive
References and Readings efforts, suggests that the patient may be trying hard to
never make an error, which in turn results in not respond-
Butler, R. W., Copeland, D. R., Fairclough, D. L., Mulhern, R. K.,
ing when they should. This is often observed in patients
Katz, E. R., Kazak, A. E. et al. (2008). A multicenter, randomized
clinical trial of a cognitive remediation program for childhood
who lack motivation, are depressed, or who experience
survivors of a pediatric malignancy. Journal of Consulting and problems because of slowed processing speed. In healthy
Clinical Psychology, 76(3), 367378. individuals, response bias can be influenced by
Atypical Teratoid/Rhabdoid Tumor (AT/RT) A 299
instructional set, reward, or other factors that shift the References and Readings A
likelihood of responding or not responding.
Larrabee, G. (2005). Forensic neuropsychology: A scientific approach.
New York: Oxford University Press.
Stern, B. H., & Brown, J. (2007). Litigating brain injuries. New York:
Cross References Thomson Reuters.
Atypical Antipsychotic
Attention-Deficit/Hyperactivity Neuroleptics
Disorder (ADHD)
Minimal Brain Dysfunction
Atypical Autism
Pervasive Developmental Disorder NOS
Attorney
M OIRA D UX
Rosalind Franklin School of Medicine
Baltimore, MD, USA Atypical Teratoid/Rhabdoid
Tumor (AT/RT)
Definition J ENNIFER T INKER
Drexel University
An attorney is defined as one who is legally appointed on Philadelphia, PA, USA
anothers behalf. An attorney-at-law is an individual who
has achieved the necessary educational requirements
(J.D.) and is licensed to practice law by the highest court Definition
of a state or some other form of jurisdiction. In civil cases
(e.g., personal injury, medical malpractice), there are Atypical Teratoid/Rhabdoid Tumor (AT/RT) is a rare,
plaintiff and defense attorneys. The plaintiff attorney highly malignant tumor of early childhood, most com-
represents the injured party (e.g., plaintiff) in an action monly diagnosed in infants who are less than 2 years. First
against the party they allege to be responsible for the described by Rorke and colleagues in 1987 (Lefkowitz,
damages; the defense attorney represents the defendant Rorke, & Packer, 1987), the AT/RT received its designation
(e.g., insurance company, hospital, and doctor). In crimi- because of its complex histological components. Progno-
nal matters, there are prosecution and defense attorneys. sis is extremely poor with a median survival of 611
The prosecuting attorney represents the party (e.g., Fed- months. Over half of AT/RTs identified are located within
eral, State, or local government) who has accused and the posterior fossa (brainstem, cerebellum, and pre-
wants to convict the offender of some type of criminal dominantly the cerebello-pontine angle) (Rorke, Packer,
action (e.g., murder, assault). The defense attorney repre- & Biegel, 1996). Clinical presentation varies largely by
sents the party (e.g., defendant) who has been accused of tumor location and size. Infants, in particular, may present
committing the crime. with nonspecific symptoms, including lethargy and
failure to thrive. Older children (>3 years of age) may
demonstrate more specific problems, including head tilt,
Cross References cranial nerve palsy, headache, and hemiplegia (Rorke &
Biegel, 2000). Often histologically confused with PNET/
Litigation medulloblastoma.
300 A Atypicals (antipsychotics)
References and Readings Heschls gyrus. The primary auditory cortex receives direct
A
input from the medial geniculate nuclei of the thalamus,
Bauer, R. M., & Demery, J. A. (2003). Agnosia. In K. Heilman & E. which it is thought to process auditory input at a very basic
Valenstein (Eds.), Clinical neuropsychology (4th ed., pp. 236295). level with little, if any, distinction between the right and left
New York: Oxford University Press.
hemispheres. The secondary auditory cortex (primarily
Brodmanns area 22) surrounds the primary cortex and,
for the most part, is located in the lateral portion of the
superior temporal gyrus. The posterior portion of this
Auditory and Visual Evoked secondary cortex in the left hemisphere constitutes
Potentials Wernickes area. These secondary cortices are thought to
be responsible for the further refinement of auditory
Event-Related Paradigms input, organizing it into meaningful or potentially mean-
ingful percepts. Lesions of Wernickes area (left hemi-
sphere) are associated with severe comprehension and
other language-related deficits, whereas comparable
Auditory Brainstem Response lesions in the right hemisphere may be associated with
difficulty recognizing or interpreting nonlanguage
Audiometry sounds. Such lesions in the right hemisphere might help
account for the inability of some patients to comprehend
Brainstem Auditory Evoked Responses or interpret the emotional tones or inflections in spoken
language, which may convey more meaning than the
actual words themselves (i.e., receptive aprosodia).
Auditory Cortex
J OHN E. M ENDOZA Auditory Discrimination
Tulane University Medical Center
New Orleans, LA, USA B ETH K UCZYNSKI 1, S TEPHANIE A. KOLAKOWSKY-H AYNER 2
1
University of California
Davis, CA, USA
2
Definition Santa Clara Valley Medical Center,
Rehabilitation Research Center
That portion of the cerebral cortex devoted exclusively to San Jose, CA, USA
the processing of input from the medial geniculate nuclei
(auditory information).
Synonyms
Current Knowledge Auditory processing
Auditory Pathway
From the Superior Olivary Nuclei to the
WOON C HOW Medial Geniculate Nuclei
Virginia Commonwealth University
Richmond, VA, USA Afferent fibers from the superior olivary nuclei merge
with other audition-associated ascending fibers and proj-
ect via the lateral lemniscus to the inferior colliculus. The
Definition inferior colliculus receives bilateral inputs from almost all
audition-related nuclei and acts as a nearly obligatory
The auditory neural pathway in the central nervous relay in the ascending auditory pathway. It is here that
system transmits and processes sound signals from the horizontally oriented and vertically oriented sound source
ear to the cortex. The configuration of the pathway is localization data is fully and finally integrated. Ascending
multisynaptic and bilaterally projecting. fibers from the inferior colliculus project ipsilaterally to
Auditory Processing A 303
drugs (e.g., aspirin), aftereffects of exposure to loud and/or recognize them at a later time. These tasks assess
noises, infections, or occasionally may represent the initial acquisition and retrieval components of memory, includ-
symptoms of a more serious condition such as a brain- ing encoding, learning characteristics, storage, consolida-
stem tumor. Unfortunately, treatment options for this tion over short- or long-time intervals, and subsequent
condition are quite limited. Having background noise, access to the information either by free retrieval or recog-
such as music, is often helpful. nition. The nature of the test composition, the instruc-
tions to the individual, and the scoring dictate what
conclusions are drawn from the task.
Cross References Auditory verbal learning tasks (AVLTs) are used in
both clinical and research settings, and are a hallmark of
Aphonia memory assessment. Recent versions of these tests include
Auditory Cortex the California Verbal Learning Test-II (CVLT-II; Delis,
Auditory Pathway Kaplan, Kramer, & Ober, 2000), Hopkins Verbal Learning
Cochlea Test-R (HVLT-R; Brandt & Benedict, 2001), World Health
Pure Word Deafness Organization/UCLA Auditory Verbal Learning Test
Tinnitus (WHO/UCLA AVLT; Maj et al., 1993), Rey Auditory
Weber Test Verbal Learning Test (RAVLT; Schmidt, 1996),
Neuropsychological Assessment Battery (NAB; Stern &
White, 2003), and List Learning in the Center to Establish
References and Readings Registry for Alzheimers Disease (CERAD; Morris et al.,
1989; Welsh, Butters, Hughes, Mohs, & Heyman, 1991).
Bradley, W. G., Daroff, R. B., Fenichel, G. M., & Jankovic, J. (2004). Other commonly used list-learning tasks (e.g., ADAS-
Neurology in clinical practice: Principles of diagnosis and management COG; Rosen, Mohs, & Davis, 1984), like the Free and
Fourth Edition. Philadelphia, PA: Butterworth-Heineman.
Cued Selective Reminder Task (FCSRT; Buschke, Sli-
Wilson-Pauwek, L., Akesson, E. J., Stewart, P. A., & Spacey, S. D. (2002).
Cranial nerves in health and disease. Hamilton, ONT: B.C. Decker,
winski, Kuslansky, Katz, Verghese, & Lipton, 2006; Gro-
Inc. ber, Merling, Heimlich, & Lipton, 1997), are not solely
auditory, adding visual words or picture presentations of
items.
List-learning tasks share many design features. The
Auditory Verbal Learning number of words in the to-be-learned list (e.g., 1216
items) has been designed to exceed the typical vigilance
J UDY C REIGHTON 1, H. A LLISON B ENDER 2 span of seven items and to stress learning demands. The
S TEPHANIE A SSURAS 1, J ENNIFER WOEHR 3 learning phase is the presentation of the list across several
J OAN C. B OROD 1,3 , N ANCY S. F OLDI 1,4 trials. Following each trial, the examinee is asked to recall
1
Queens College and The Graduate Center of the as many items as possible. Once the learning is complete,
City University of New York a short time interval elapses, usually including interfer-
Flushing, NY, USA ence tasks designed to prevent rehearsal of the list items.
2
New York University Langone Medical Center The examinee is then asked to recall items from the list,
New York, NY, USA constituting a short term recall. After another longer
3
Mount Sinai School of Medicine interval, again containing some distraction, a recall is
New York, NY, USA requested. These short- and long-term retrieval assess-
4
State University of New York, Stony Brook ments capture the examinees ability to store, consolidate,
School of Medicine and maintain information, as well as to retrieve it on
Mineola, NY, USA command. Tests can include a subsequent multiple- or
forced-choice task to facilitate access, capturing the items
that were encoded but could not be accessed on free
Description retrieval.
Despite commonalities, instruments vary in content
An auditory verbal learning task typically requires indivi- and administration. List construction on some tests (e.g.,
duals to hear a list of items, learn those items, and recall CVLT-II, HVLT-R, WHO/UCLA AVLT, and
Auditory Verbal Learning A 307
Direct translation of word lists into other languages verbal learning, providing detailed information about an
should be avoided, as words may have been selected to individuals capacity to acquire, consolidate, store, and
accommodate word frequency within a language or ap- retrieve information, thus revealing significant aspects of
propriateness within a culture. Some AVLTs are targeted the learning and retrieval processes. Learning character-
for certain populations using shorter versions and more istics, particularly primary and recency serial position
age-appropriate word lists (e.g., CVLT-C). Shorter effects (Foldi, Brickman, Schaefer, & Knutelska, 2003),
forms have also been adopted for more impaired indivi- are highly informative in differentiating clinical popula-
duals (e.g., CVLT-II, Short Form; Delis et al., 2000). tions and provide insight about how learning is occurring.
Verbal learning tests are used to evaluate memory A detailed step-by-step exploration of each individuals
performance over time. Testretest reliability using the learning process enables the clinician or researcher to
same version of the test has been evaluated for 1-month identify specific areas of vulnerability and can guide
as well as 1-year intervals. Many tests have adopted alter- intervention strategies.
nate or multiple forms. Whereas alternate versions can Auditory verbal learning tasks have been instrumental
reduce practice effects, there is evidence that these effects in assessing change in memory over time. The auditory
are not completely eliminated (Houx et al., 2002) due to verbal learning paradigm has been very sensitive in
procedural learning. Therefore, examiners must be very discriminating between healthy and memory-impaired
cognizant of practice effects. Even after 2 years, healthy groups. A vast literature demonstrates the sensitivity of
older adults showed practice effects on the CVLT-II (Blasi, these tests to the detection of prodromal Alzheimers
Zehnder, Berres, Taylor, Spiegel, & Monsch, 2009). disease.
Construct validity of adult and child versions of the
CVLT in assessing learning and memory has been exam-
ined in several studies. Attention span, learning efficiency, Cross References
delayed recall, and inaccurate recall represent one example
of a four-factor model that was identified by a factor California Verbal Learning Test-II
analysis in a sample of children with traumatic brain California Verbal Learning Test-II, Childrens Version
injury (Mottram & Donders, 2005). Variability across Consortium to Establish a Registry for Alzheimers
studies may be a result of methodological differences, Disease (CERAD)
demographic differences, severity of patient population, Hopkins Verbal Learning Test Revised
and/or time elapsed between disease onset and assessment. Memory
In terms of inter-test relationships, tests are not Neuropsychological Assessment Battery
interchangeable, as word lists and administrations vary Rey Auditory Verbal Learning Test
significantly. However, the RAVLT correlates moderately
with the CVLT-II. The CVLT-II is now incorporated into
the Wechsler Memory Scale IV as a valid component of References and Readings
the memory indices. The HVLT-R also shows a high
correlation with the CVLT-II, although it may be more Blasi, S., Zehnder, A. E., Berres, M., Taylor, K. I., Spiegel, R., &
appropriate for greater disease severity. Monsch, A. U. (2009). Norms for change in episodic memory as a
prerequisite for the diagnosis of mild cognitive impairment (MCI).
Neuropsychology, 23, 189200.
Boake, C. (2000). Edouard Claparede and the Auditory Verbal Learning
Clinical Uses Test. Journal of Clinical and Experimental Neuropsychology, 22,
286292.
Verbal learning tests are ubiquitous in assessments of Brandt, J. (1991). The Hopkins Verbal Learning Test: Development of a
new memory test with six equivalent forms. The Clinical Neuropsy-
memory-impaired populations. Amnesic disorders, de-
chologist, 5, 125142.
generative dementias (including Alzheimers, Parkinsons, Brandt, J., & Benedict, R. (2001). Hopkins Verbal Learning Test-Revised.
and Huntingtons disease), temporal lobe epilepsy, Lutz, FL: Psychological Assessment Resources.
traumatic brain injury, depression, and focal stroke are Buschke, H., Sliwinski, M. J., Kuslansky, G., Katz, M., Verghese, J., &
diseases that have not only benefited from these tests, but Lipton, R. B. (2006). Retention weighted recall improves dis-
crimination of Alzheimers disease. Journal of the International
also have promoted refinements and further development
Neuropsychological Society, 12, 436440.
to extend our knowledge of the neuropsychological Delis, D. C., Kaplan, E., Kramer, J. H., & Ober, B. A. (2000). California
underpinnings of memory function. These instruments Verbal Learning Test II (2nd ed.). San Antonio, TX: The Psycho-
evaluate both quantitative and procedural aspects of logical Corporation.
Augmentative and Alternative Communication (AAC) A 309
Delis, D. C., Kramer, J., Kaplan, E., & Ober, B. A. (1994). California Verbal
Learning Test Childrens Version. San Antonio, TX: Pearson. Augmentative and Alternative A
Delis, D. C., Kramer, J. H., Kaplan, E., & Ober, B. A. (1987). California
Verbal Learning Test: Adult Version. New York: The Psychological Communication (AAC)
Corporation.
Ebbinghaus, H. (1885). Memory: A contribution to experimental psychology D IANE C ORDRY G OLDEN
(H. A. Ruger & C. E. Bussenenues, Trans., 1913). New York: Teachers Association of Assistive Technology Act Programs
College, Columbia University.
Delmar, NY, USA
Foldi, N. S., Brickman, A. M., Schaefer, L. A., & Knutelska, M. E. (2003).
Distinct serial position profiles and neuropsychological measures
differentiate late life depression from normal aging and Alzheimers
disease. Psychiatry Research, 120, 7184. Definition
Grober, E., Merling, A., Heimlich, T., & Lipton, R. B. (1997). Free and
cued selective reminding and selective reminding in the elderly.
Augmentative and alternative communication (AAC) is a
Journal of Clinical and Experimental Neuropsychology, 19, 643654.
Houx, P. J., Shepherd, J., Blauw, G. J., Murphy, M. B., Ford, I., Bollen, E. L., set of procedures and processes by which an individuals
et al. (2002). Testing cognitive function in elderly populations: communication skills can be maximized for functional and
The PROSPER study. Journal of Neurology, Neurosurgery, and effective communication. AAC approaches supplement or
Psychiatry, 73, 385389. replace natural speech with aided options that incorporate
Maj, M., DElia, L., Satz, P., Janssen, R., Zaudig, M., Uchiyama, C., et al.
the use of some type of device ranging from simple picture
(1993). Evaluation of two new neuropsychological tests designed to
minimize cultural bias in the assessment of HIV-1 seropositive per- communication systems to complex speech generating
sons: AWHO study. Archives of Clinical Neuropsychology, 8, 123135. devices and/or unaided options that involve only the indi-
Morris, J. C., Heyman, A., Mohs, R. C., Hughes, J. P., van Belle, G., viduals body, such as sign language. AAC may be used to
Fillenbaum, G., et al. (1989). The Consortium to Establish a Registry augment understanding as well as written or oral expres-
for Alzheimers Disease (CERAD). Part I. Clinical and neuropsycho-
sion. A multimodal approach that includes both devices
logical assessment of Alzheimers disease. Neurology, 39, 11591165.
Mottram, L., & Donders, J. (2005). Construct validity of the California and unaided strategies may be most effective in meeting
Verbal Learning Test-Childrens Version (CVLT-C) after pediatric the individuals communication needs.
traumatic brain injury. Psychological Assessment, 17, 212217.
Ponton, M. O., Satz, P., Herrera, L., Ortiz, F., Urrutia, C. P., Young, R.,
et al. (1996). Normative data stratified by age and education for the Historical Background
Neuropsychological Screening Battery for Hispanics (NeSBHIS):
Initial report. Journal of the International Neuropsychological Society,
2, 96104. Prior to about 1970, AAC was not a widely accepted
Raaijmakers, J. G. W., & Shiffrin, R. M. (1981). Search of associative intervention technique and could even be described as
memory. Psychological Review, 88, 93134. contraindicated in the professional literature. At that
Rosen, W. G., Mohs, R. C., & Davis, K. L. (1984). A new rating scale for time, it was thought that the act of vocal production was
Alzheimers disease. American Journal of Psychiatry, 141, 13561364.
Schmidt, M. (1996). Rey Auditory Verbal Learning Test: A handbook. Los
a critical building block of human language development.
Angeles: Western Psychological Services. As a result, interventionists believed that providing an
Stern, R. A., & White, T. (2003). Neuropsychological Assessment Battery. alternative to speech production would deter speech
Lutz, FL: Psychologial Assessment Resources. (and thus language) development because the child
Welsh, K., Butters, N., Hughes, J., Mohs, R., & Heyman, A. (1991). would choose to use the easier alternative mechanism
Detection of abnormal memory decline in mild cases of Alzheimers
disease using CERAD neuropsychological measures. Archives of
(Bates, 1976; Fourcin, 1975).
Neurology, 48, 278281. In the late 1960s and in the 1970s, research evidence
indicated speech was actually a secondary component to
language function and that robust receptive and expres-
sive language could be developed without vocal produc-
Auditory-Sound Agnosia tion. In many cases, the use of alternatives to speech
production was found to actually support (and in no
Auditory Agnosia way deter) vocal production (Schlosser & Wendt, 2008;
Silverman, 1980; Zangari, Lloyd, & Vicker, 1994). In 1980,
the American Speech-Language-Hearing Association
(ASHA) established an Ad Hoc Committee on Commu-
Auditory-Verbal Agnosia nication Processes and Nonspeaking Persons that devel-
oped a position statement outlining the concept of AAC
Auditory Agnosia as a set of intervention techniques using a variety of
310 A Augmentative and Alternative Communication (AAC)
symbol sets and communication interaction behaviors. systems have been developed to meet this need with some
This became the framework for the field of AAC today. specifically focused on individuals with neuropsychological
At about the same time, new computer technologies disabilities such as autism (Glennen & DeCoste, 1997).
were exploding onto the scene creating previously un- While the core goal of AAC is to provide effective com-
imaginable opportunities for AAC device development. munication, related objectives can include decreasing
The Trace Research and Development Center, part of the problem behaviors and increasing successful education,
College of Engineering at the University of Wisconsin- employment, and community living outcomes.
Madison, was formed in 1971 to address the communica-
tion needs of people with severe disabilities who were
nonspeaking. The Center was an early leader and inno-
Treatment Participants
vator in the augmentative communication field and pio-
Early in the history of AAC, two misconceptions thrived
neered development of electronic communication aids in
regarding candidacy for AAC that a set of prerequisite skills
the 1970s and 1980s that became prototypes for the
(usually cognitive and motor) was required before AAC
speech generating devices (SGDs) of today (Vanderheiden,
could be considered and AAC should only be implemented
1978; Vanderheiden & Grilley, 1976).
after all traditional forms of speech therapy had failed. Both
have been proven unsubstantiated as many successful AAC
users have severe motor and/or cognitive impairments, and
Rationale or Underlying Theory
research has shown no justification for waiting to implement
AAC as it can support speech development (Shane & Bashir,
Today the widespread acceptance of AAC is a valid inter-
1980). As a result, candidacy for AAC is not limited by
vention option to develop viable means of effective com-
age, disability diagnosis or prerequisite cognitive or motor
munication for any individual with limited natural
skills. Individuals who can benefit from AAC may be of all
speech, as well as to enhance comprehension for those
ages, including infants and toddlers with disabilities, and
who are not hearing impaired, but who have difficulty
may have diagnoses including apraxia, dysarthria, apha-
understanding spoken language. In 1992, the National
sia, autism, ALS, cerebral palsy, multiple sclerosis, Parkin-
Joint Committee for the Communication needs of Per-
sons, mental retardation, etc.
sons with Severe Disabilities issued a Communication Bill
of Rights that unequivocally states, All persons, regard-
less of the extent or severity of their disabilities, have a Treatment Procedures
basic right to affect, through communication, the condi-
tions of their own existence. A set of 12 specific rights are The challenges for those providing intervention are main-
described in the Bill of Rights including, the right to have taining current knowledge of the vast array of available
access at all times to any needed augmentative and alter- AAC device options, appropriately matching the skill sets
native communication devices and other assistive devices of individuals with disabilities to available AAC systems,
and to have those devices in good working order. securing funding to acquire the system, improving com-
munication opportunities and environments, and
providing supports sufficient to ensure effective use of
Goals and Objectives the system.
Consideration for aided AAC intervention begins with
Most of the early history of AAC focused on individuals with assessment by a qualified team of individuals who are
neuromotor impairments that limited oral-motor skills knowledgeable about the individual and his other
such as cerebral palsy and amyotrophic lateral sclerosis. strengths and limitations especially in the areas of speech,
However, with the passage of landmark legislation such as language, and motor skills. One or more team members
the Education of Handicapped Childrens Act (P.L. 94142), should be knowledgeable about the range of potential
Section 504 of the Rehabilitation Act, and later the AAC alternatives available and those that are viable
Americans with Disabilities Act, individuals with all types options to meet the individuals communication needs.
of disabilities have become more integrated into education, Appropriate practice in assessment includes conducting
employment, community living, and society in general. This structured device trials with various AAC devices in the
change created a widespread need for individuals with environment(s) in which the individual will be using
all types of disabilities to have an effective, functional com- the technology (e.g., home, school, community, etc.).
munication system. More complex and efficient AAC This allows for comparative analysis of different device
Augmentative and Alternative Communication (AAC) A 311
features and functions to determine which best address Any number of standardized communication measures
A
the individuals functional communication needs. can be used to document communication efficacy using
The result of an AAC assessment is identification of the AAC system. In addition, the Adaptive Technology
the system features appropriate for an individual includ- Resource Center had identified a variety of outcome
ing specification of device input features (selection tech- measurement tools that focus on the functional efficiency
niques), message characteristics, and output features. and functional gains achieved through the use of assistive
Once an appropriate AAC system has been acquired, technologies including AAC, see http://atrc.utoronto.ca/
training and support for the user, their family, and others index.php?option = com_content&task = view&id =
(e.g., teachers, therapists, etc.) must be implemented. 178&Itemid = 69.
More complex AAC systems frequently require initial
programming and device setup as part of user support
services. Short and long-term communication goals
should be developed using the AAC system and therapy Qualifications of Treatment Providers
services implemented to support goal achievement
(Beukleman, Garrett, & Yorkston, 2007; Beukelman & AAC intervention is typically provided by speech-
Mirenda, 2005; www.aac-rerc.com). language pathologists (SLPs) who are licensed by states
as health care providers, educated at the graduate level
in the study of human communication, its develop-
Efficacy Information ment and its disorders. Medicare requires an SLP who
provides AAC assessments or treatment to hold the
Efficacy research on AAC ranges from observation of Certificate of Clinical Competence (CCC) in speech-
changes in functional communication skills to potential language pathology from the American Speech-
secondary improvements in academic, social, behavioral, Language-Hearing Association. In addition to SLPs,
and other areas. For individuals with limited or no some other types of professional providers may be
functional communication, an AAC system that delivers members of the intervention team providing AAC
basic communication ability can be deemed effective by services, especially in the educational environment, e.
self-verification of communication occurring (Fried- g., special educators, occupational therapists, assistive
Oken & Bersani, 2000). Beyond this basic gauge of AAC technology practioners, etc. (ASHA, 2002; ASHA, 2004;
efficacy, research has been done on a variety of specific ASHA, 2005).
outcomes such as decreasing problem behaviors through
the use of AAC (Vaughn & Horner, 1995), enhancing the
rate of AAC communication, (Venkatagiri, 1993), and
even nuances such as speech synthesizer intelligibility Cross References
(Mirenda & Beukelman, 1990) all in an effort to support
AAC efficacy. Articulation
Seminal work on AAC efficacy done by Ralf Schlosser Articulation Disorder
(2003) addressed a wide range of AAC efficacy issues Assistive Technology
including the role of AAC in facilitating or hindering Speech
natural speech development, literacy development in Speech/Communication Disabilities
AAC users, and the effects of speech output (use of speech Speech-Language Pathology
generating devices). In 2001, Medicare began coverage of Speech-Language Therapy
speech generating devices (SGDs) based on acceptance of Speech Sound Disorders
AAC efficacy research. Since then, a number of private
insurance carriers have followed suit and now cover
SGDs as do most state Medicaid programs.
References and Readings
American Speech-Language-Hearing Association. (1981). Position state-
Outcome Measurement ment on non-speech communication. ASHA, 23, 577581.
American Speech-Language-Hearing Association. (2002). Augmentative
The most direct outcome measure for AAC intervention is and alternative communication: Knowledge and skills for service delivery
demonstration of effective and efficient communication. [Knowledge and Skills]. Retrieved from www.asha.org/policy
312 A Aura
diagnosis of autism spectrum disorders. Journal of the American order to make judgments about social and communicative
Academy of Child and Adolescent Psychiatry, 45, 10941103. A
abilities as independent as possible from the effects of
Rutter, M., Le Couteur, A., & Lord, C. (2003). Autism diagnostic
interview-revised. Los Angeles: Western Psychological Services.
absolute level of language delay. Each module has its own
World Health Organization. (1992). The ICD-IO classification of mental protocol, which contains a schedule of activities designed
and behavioral disorders: Clinical descriptions and diagnostic guide- for use with children or adults at particular developmental
lines. Geneva: Author. and language levels. Recently, the Toddler Module of the
ADOS was developed for use in children between 12 and 30
months of age in addition to the original four modules
(Lord, Luyster, Gotham, & Guthrie, in press).
Module 1 is intended for children who do not use
Autism Diagnostic Observation spontaneous phrase speech consistently. It consists of 10
Schedule activities with 29 accompanying ratings. Module 2 is
intended for children with some flexible phrase speech
S O H YUN K IM , C ATHERINE L ORD who are not verbally fluent. It consists of 14 activities with
University of Michigan Autism and Communication 28 accompanying ratings. Module 3 provides 13 activities
Disorders Center (UMACC) and 28 ratings. It is intended for verbally fluent children
Ann Arbor, MI, USA for whom playing with toys is age-appropriate. The oper-
ational definition of verbal fluency is the spontaneous,
flexible use of sentences with multiple clauses that de-
Synonyms scribe logical connections within a sentence. It requires
the ability to talk about objects or events not immediately
ADOS present. Module 4 contains the socioemotional questions,
along with interview items about daily living and addi-
tional tasks. It is intended for verbally fluent adults and
Description for adolescents who are not interested in playing with toys
such as action figures (usually over 1216 years). This
The Autism Diagnostic Observation Schedule (ADOS; module consists of 1015 activities with 31 accompanying
Lord, Rutter, DiLavore, & Risi, 2001) is a semistructured, ratings. The difference between Modules 3 and 4 lies
standardized assessment of communication, social interac- primarily in whether information about social communi-
tion, and play or imaginative use of materials for individuals cation is acquired during play or through a conversational
who have been referred because of possible autism spectrum interview. It is important to note that adolescents or
disorders (ASD). As part of the schedule, planned social adults may feel uncomfortable when presented with the
occasions, referred to as presses (Lord, Rutter, Goode, & toys for young children that are available in modules 1
Heemsbergen, 1989) are created in which a range of social and 2; suggestions for modifying the earlier modules to be
initiations and responses is likely to appear. In the same appropriate for older children or adults who are less
way, communication opportunities are designed to elicit verbal are available from the authors. In addition to the
a range of interchanges. Play situations are included four modules, the Toddler Module is intended for chil-
to allow observation of a range of imaginative activities dren between 12 and 30 months of age who should have a
and social role-play. A variety of structured activities nonverbal age equivalent of at least 12 months and be
and materials, and less structured interactions, provide walking independently. It consists of 11 activities with 41
standard contexts within the ADOS in which the social, accompanying ratings (Lord et al., in press).
communicative, and other behaviors relevant to the The ADOS provides the diagnostic algorithms that are
understanding of ASD are observed. sets of rules that allow classification of autism or ASD.
The ADOS consists of four modules. Each module is Separate diagnostic algorithms for each module can be
appropriate for children and adults at different develop- generated using subsets of items in each module. Items
mental and language levels, ranging from no expressive or and the thresholds for the classification of autism and of
receptive use of words, to fluent, complex language in an ASD in the algorithms differ for each module. However,
adult. Only one module, lasting about 30 minutes, is admi- the general principles and procedures for computation are
nistered to any individual at a given point of time. In the the same across modules and similar to the DSM-IV
ADOS, the examiner uses the module that best matches the (American Psychiatric Association, 1993) and ICD-10
expressive language skills of the individual child or adult in (World Health Organization, 1992). The algorithms for
316 A Autism Diagnostic Observation Schedule
Module 1, 2, and 3 were recently revised from the previ- there was a need to extend the age and verbal limits of the
ous algorithms (Gotham, Risi, Pickles, & Lord, 2007). ADOS to be appropriate for younger and nonverbal chil-
Reflecting recent research, the revised algorithms now dren. The PL-ADOS was then created based on the grow-
consist of two new domains, Social Affect and Restricted, ing interest in using the instruments in clinical settings,
Repetitive Behaviors, combined to one score to which which addressed the concerns of parents and fit the abil-
thresholds are applied, resulting in generally improved ities of children functioning at infant and toddler levels.
predictive validity compared to the previous algorithms. As a result, it included more flexible, briefer activities and
The module 1 consists of no words and some words greater use of play materials for nonverbal young children
algorithms by language level. The module 2 includes that served as a downward extension for the ADOS, rather
Younger than 5 and Greater or Equal to 5 algorithms than a replacement. The PL-ADOS was effective in dis-
by chronological age. Module 3 includes a single algo- criminating 25-year-old-children with autism from chil-
rithm. All items appearing on the new algorithms con- dren with non-autism spectrum developmental delays
tribute to a single score with two classification thresholds, (DiLavore et al., 1995). However, it tended to be under-
one for autism and another for ASD. There are the inclusive for children with autism who had some expres-
Toddler Module algorithms for children between 12 and sive language. Thus, a tool was required to address the
30 months, who do not have phrase speech; once children needs of children who fell between the PL-ADOS and
have developed phrase speech, they should be adminis- ADOS in language skills. Furthermore, the ADOS con-
tered module 2. Since differential diagnosis can be chal- sisted primarily of activities intended for school-age chil-
lenging especially in toddlers, the toddler module dren. Additional or alternative tasks were needed for
algorithms generate range of concern (little-or-no con- adolescents and adults. The current edition of the ADOS
cern; mild-to-moderate concern; moderate-to-severe was designed in response to these factors. The current
concern) rather than strict classifications. ADOS differs from the preceding instruments in a way
Most of the ADOS pertains to behaviors that are rare that it is aimed at providing standard contexts for the
in individuals who do not have ASD and/or who do not observation of behavior for a broader developmental and
have profound intellectual disabilities. Thus, numerical age range of individuals suspected of having autism. Thus,
estimates of the typical scores of general population the current ADOS includes additional items developed for
have not been obtained. Scores from module 1 to 3 have verbally fluent, high-functioning adolescents and adults
now been calibrated for children with ASD to yield a as well as younger and nonverbal children.
standard severity score based on a large sample (see However, even though this updated version of the
below; Gotham, Pickles, & Lord, in press). ADOS did indeed extend the usefulness of the original
ADOS below a language level of 3 years, research has indi-
cated that it remained of limited value for children with
Historical Background nonverbal mental age below 16 months. Thus, a standar-
dized diagnostic measure applicable for infant and young
In its present form, the current WPS Edition of the ADOS toddlers was also needed for early identification (Gotham
(Lord et al., 2001) is a combination of two similar diag- et al., 2007). The recent development of the Toddler Mod-
nostic instruments: the 1989 version of the ADOS (Lord ule of the ADOS reflects this need for the measure to be
et al., 1989) and the Pre-Linguistic ADOS (PL-ADOS; applicable to very young children from 12 to 30 months.
DiLavore, Lord, and Rutter, 1995). The ADOS was first The original algorithms included two domains, social
introduced in the 1980s as a method of standardizing interaction and communication. Recently, Gotham et al.
direct observations of social behavior, communication, (2007) revised algorithms for module 1, 2, and 3, and
and play in children suspected of having autism. It was the existing social and communication domains were
intended to be administered to children between the ages merged, and the domain of Restricted Repetitive Beha-
of 5 and 12, who had expressive language skills at least at viors (RRB) was newly included. The revised algorithms
the 3-year-old level. It was proposed as a complementary resulted in increased specificity and sensitivity proving
instrument to the Autism Diagnostic Interview (ADI; Le increased diagnostic validity compared to the previous
Couteur et al., 1989), an investigator-based parent or algorithms. Furthermore, even though the inclusion of
caregiver interview that yielded a description of history, the RRB domain did not improve predictive value of the
as well as current functioning, in areas of development ADOS in differentiating individuals with autism from
related to autism. Because children under age five consti- those with pervasive developmental disorder not
tute the bulk of referrals for a first diagnosis of autism, otherwise specified (PDD-NOS; also referred as ASD), it
Autism Diagnostic Observation Schedule A 317
aided in distinguishing PDD-NOS from non-spectrum Psychometric properties for the Toddler Module were
A
cases. provided for a sample of 182 different participants. The
sensitivity of each algorithm ranged from 83% to 91%
and specificity from 86% to 94%. Inter-rater item and
Psychometric Data domain reliability was greater than .71, and inter-rater
algorithm reliability ranged from .60 to .90. Intraclass
Psychometric properties for the original ADOS were correlations ranged from .74 to .99 for all algorithm
provided for a carefully selected, blindly interviewed and domains and total scores (Lord et al., in press).
coded, sample of 223 children and adults with autistic
disorder (autism), pervasive developmental disorder
not otherwise specified (PDD-NOS) or non-spectrum Clinical Uses
(NS) diagnoses. Inter-rater reliability was assessed, with
mean multirater kappas of all items for each module rang- Use of the ADOS is related to the examiners clinical skills
ing from .65 to .78 and intraclass correlations above .82 for and experience with the instrument. Examiners need to be
all subdomain and domain scores. Test-retest reliability sufficiently familiar with the ratings and the activities so
varied by subdomain ranging from .59 to .82. In the origi- that they can focus their attention on observation of the
nal sample, the ADOS algorithms generally achieved 94% individual being assessed, rather than on administration
correct classification. The exceptions were the ASD versus of tasks. The examiner should have sufficient practice in
non-spectrum (NS) module 2 specificity of 87% and observation of ASD symptoms and scoring of the ADOS
module 3 sensitivity of 90%, and the PDD-NOS versus items, as well as in administering the activities. Examiners
NS Module 2 specificity of 88% and sensitivity of 89% are encouraged to attend workshops, use videotapes, or
and module 3 sensitivity of 80% (Lord et al., 2001). work with colleagues to obtain inter-rater reliability be-
Psychometric properties for the newly revised algo- fore administering the ADOS for clinical or research pur-
rithms were provided for a sample of 1,139 different poses (Lord et al., 2001). Examiners should note that the
participants. The revised algorithms resulted in increased Toddler Module and module 1 are always administered
specificity in classifying non-autism ASD in lower func- with parents or caregivers in the room, which provides an
tioning populations, evidenced by the 1231% increase in opportunity to show a parent, examples of behaviors that
specificity for children without any words (depending on define ASD, and get information from a parent about the
nonverbal mental age) and the modest gain in specificity validity of the childs behaviors during the testing session.
for older children who have not progressed beyond phrase Because the ADOS consists of codings made from a single
speech. During module 1, no words improved in each observation, it does not include information about histo-
diagnostic comparison (e.g., from the sensitivity of 19% ry or functioning in other contexts. This means that the
to 50% for children with nonverbal mental age of 15); ADOS alone cannot be used to make a complete standard
the specificity of both module 2 groups improved for diagnosis, but used in conjunction with other testing.
non-autism ASD versus NS (e.g., from 77% to 83% Lord and her colleagues suggested several strategies
for children greater or equal to 5). For autism versus that clinicians or researchers may take to measure how
non-spectrum and for ASD versus NS, the new and old behaviors of individual may have changed over time by
algorithms performed approximately equally well in using the ADOS item and domain scores (Lord et al.,
terms of sensitivity. For non-autism ASD versus non- 2000). If an individual has been administered the same
spectrum, sensitivity of the new algorithm was somewhat module more than once, raw scores on individual items
lower in module 1, no words (as was necessary to raise and on algorithm domains can be compared. If an indi-
specificity; e.g., 100% in old algorithm versus 97% in new vidual has changed modules, raw scores on items that
algorithm for children under nonverbal mental age under remain constant across modules (about two thirds of
15), but it showed improvement from the old algorithm each contiguous module) can be compared, yet compari-
in the higher-functioning modules 1 (AUT versus NS; son of raw domain scores is not meaningful. However, the
from 88% to 97%) and module 2 (ASD versus NS; from ADOS calibrated scores recently developed by Gotham
76% to 84%) cells. Inter-rater reliability on the ADOS was et al. (in press) can be used in this case to compare
monitored through joint administration and scoring by assessments across modules and time. The calibrated
two different examiners for at least 1 in 10 cases and, in scores have more uniform distributions across age- and
some cases, through scoring of videotapes. Agreement language-groups compared to raw totals, which make it
remained at greater than 85% (Gotham et al., 2007). possible to compare childrens scores longitudinally across
318 A Autism Spectrum Disorder
distinct algorithms. Thus, calibrated scores can be useful DiLavore, P., Lord, C., & Rutter, M. (1995). Pre-linguistic autism diag-
nostic observation schedule (PL-ADOS). Journal of Austism and
in clinical settings to test treatment responsiveness and
Developmental Disorders, 25, 355379.
other clinical outcomes in individuals with ASD. Gotham, K., Pickles, A., & Lord, C. (in press). Standardizing ADOS
In addition, it was suggested that more detailed scores for a measure of severity in autism spectrum disorders.
coding of communication samples or particular behaviors Journal of Autism and Developmental Disorders.
(e.g., pragmatics, sentence structure, gestures) may also be Gotham, K., Risi, S., Pickles, A., & Lord, C. (2007). The autism diagnostic
observation schedule: Revised algorithms for improved
carried out from videotapes of the ADOS. Other observa-
diagnostic validity. Journal of Autism and Developmental Disorders,
tional coding schemes that address specific aspects of 37, 613627.
behavior in more detail may also be applied using the Le Couteur, A., Rutter, M., Lord, C., Rios, P., Robertson, S.,
ADOS as a way of obtaining a discrete sample of behavior Holdgrafer, M., et al. (1989). Autism diagnostic interview: A
in standard contexts. Often, clinicians carrying out diag- semistructured interview for parents and caregivers of autistic per-
sons. Journal of Autism and Developmental Disorders, 19, 363387.
nostic assessments may wish to make programming
Lord, C. E., Luyster, R., Gotham, K., & Guthrie, W. J. (in press). Autism
suggestions for parents/caregivers, therapists, or teachers. diagnostic observation schedule toddler module. Los Angeles, CA:
Many of the activities and codes of the earlier modules Western Psychological Services.
have fairly straightforward implications both for how to Lord, C., Risi, S., Lambrecht, L., Cook, E. H., Leventhal, B. L., DiLavore,
teach an individual child and for the content of appropri- P., et al. (2000). The autism diagnostic observation schedulegeneric:
A standard measure of social and communication deficits associated
ate goals. For example, module 1 provides opportunities
with the spectrum of autism. Journal of Autism and Developmental
for children to make requests in a number of circum- Disorders, 30(3), 205223.
stances, including requests for action (i.e., the examiner Lord, C., Rutter, M., DiLavore, P. C., & Risi, S. (2001). Autism diagnostic
to blow a balloon), requests for food, requests to continue observation schedule. Los Angeles, CA: Western Psychological
a social game, and requests for an object or activation of Services.
Lord, C., Rutter, M., Goode, S., & Heemsbergen, J. (1989). Autism
that object (i.e., operating a bubble gun). Noting how
diagnostic observation schedule: A standardized observation of
children make requests and in what circumstances they communicative and social behavior. Journal of Autism and Develop-
are most easily able to communicate their interest or mental Disorders, 19(2), 185212.
needs, allows the clinician to create goals to teach new Lord, C., Rutter, M., & Le Couteur, A. L. (1994). Autism diagnostic
request behaviors and to help the children generalize interview-revised: A revised version of a diagnostic interview for
caregivers of individuals with possible pervasive developmental dis-
existing behaviors across contexts. Generating program-
orders. Journal of Autism and Developmental Disorders, 24(5),
ming goals from modules 3 and 4 may be somewhat more 659685.
complex, because fewer codes describe specific behaviors World Health Organization. (1992). The ICD-IO classification of mental
that may be usefully taught in a direct fashion. Realizing and behavioral disorders: Clinical descriptions and diagnostic
the degree to which adults with autism have limited guidelines. Geneva, Switzerland: Author.
insight into the nature of social relationships, or having
the opportunity to observe adolescents describing the
emotions of the main characters in a story, can be helpful
in representing the strengths they may have and
difficulties they experience in social interaction. Autism Spectrum Disorder
Pervasive Developmental Disorder NOS
Cross References
Autism Diagnostic Interview-Revised (ADI-R)
Autistic Disorder Autistic Disorder
Childhood Autism Rating Scales
Modified Checklist for Autism in Toddlers (M-CHAT) F RED R. VOLKMAR
also CHAT Yale University
New Haven, CT, USA
review a panel from the US National Research Council Kanner, L. (1943). Autistic disturbances of affective contact. Nervous
Child, 2, 217250. A
systematically evaluated ten treatment programs for youn-
Klin, A., Jones, W., Schultz, R., Volkmar, R., & Cohen, D. (2002). Visual
ger children with autism. Although differing in some fixation patterns during viewing of naturalistic social situations as
respects these programs shared many similarities including predictors of social competence in individuals with autism. Archives
intensive individualized programs and structured teach- of General Psychiatry, 59(9), 809816.
ing. Many, although not all, of these programs make ex- Klin, A., Jones, W., Schultz, R., & Volkmar, F. (2003). The enactive mind,
or from actions to cognition: Lessons from autism. Philosophical
tensive use of applied behavior analytic principles to teach
Transactions of the Royal Society of London, Series B: Biological
basic skills which can then be expanded. Increasing social Sciences, 358(1430), 345360.
and communication abilities are important goals. Psycho- Klin, A., Jones, W., Schultz, R., Volkmar, F., & Cohen, D. (2002). Defining
therapy is not a mainstay of treatment but is sometimes and quantifying the social phenotype in autism. American Journal of
used in older and more able individuals but is to be Psychiatry, 159, 895908.
Lord, C., & Venter, A. (1992). Outcome and follow-up studies of high-
problem-focused in nature.
functioning autistic individuals. In E. Schopler & G. B. Mesibov
Drug treatments can be helpful relative to certain symp- (Eds.), High-functioning individuals with autism current issues in
toms (e.g., agitation, stereotyped mannerisms) but do not autism (Vol. xviii, pp. 187199; 316). Plenum: NY.
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Washington, DC: National Academy Press.
Ozonoff, S., South, M., & Provencal, S. (2005). Executive functions.
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TEACCH during face discrimination among individuals with autism and
Vineland II Asperger syndrome. Archives of General Psychiatry, 57(4), 331340.
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Bachevalier, J. (1996). Brief report: Medial temporal lobe and autism: Journal of the American Academy of Child and Adolescent Psychiatry,
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322 A Autobiographical Memory
memories of the most recent two decades, and for people with her mother on Tuesdays, as opposed to remembering
A
over age 40, an increase in memories about adolescence a specific one of those Tuesday lunches.
and early adulthood (Wenzel & Rubin, 2005). This cueing Research also indicates that trauma and non-trauma
method is most commonly applied using the Autobio- memories differ substantially in clinical, but not in healthy
graphical Memory Test, first described in 1986 by populations (Dalgleish & Brewin, 2007). While involun-
Williams and Broadbent (Wenzel & Rubin, 2005). A second tary memory may be enhanced in some clinical popula-
open-ended method involves simply asking a participant tions, voluntary memory is often fragmented, incomplete,
about his or her life. A more structured approach, the and disorganized, particularly in people with a trauma
involuntary-memory-diary method, asks participants to history. PET and fMRI studies have suggested that the
keep a diary record of involuntary AMs as they occur. retrieval of trauma memories in PTSD patients is char-
Some other methods for delving into AM are more acterized by increased activity of limbic and paralimbic
closed-ended. The Autobiographical Memory Interview, areas, including the amygdala. Additionally, researchers
developed by Kopelman, Wilson, and Baddeley, was have found deactivation of the medial prefrontal areas
designed to be used with neuropsychological patients and Brocas area and decreased hippocampal activity in
(Wenzel & Rubin, 2003). The interview asks for specific PTSD patients when they processed emotional, rather
kinds of memories from specific time periods. The partic- than neutral material.
ipant is not provided a choice as to the types of memories In looking at depressed individuals, studies have
he or she will share. In the diary recall method, partici- suggested that cues reflecting personal characteristics
pants record events and subsequently receive a memory are more likely to promote a shift to processing of infor-
test for these events. Unlike the other methods, the diary mation within the long-term view of the self, increasing
recall method can provide some measure of the accuracy the likelihood that self-related semantic information will
of memories. A final method, the questionnaire method, be provided in response to cues on the Autobiographical
asks participants to report on a series of properties Memory Test. In a different, but related line of research,
of AMs; this method is often used in conjunction euthymic individuals with a history of depression and
with other approaches. Recently, Sutin and Robins patients with a borderline personality disorder retrieved
(2007) have developed The Memory Experiences Question- less specific AMs in response to cue words that matched
naire, a measure designed to assess a comprehensive range highly endorsed attitudes or schema. This finding suggests
of dimensions of AM. that an impaired retrieval of specific memories may be the
In developing methods to study such a multifaceted result of certain cues activating generic, higher-order
phenomenon as AM, researchers focus on several variables mental representations in people with both psychopathol-
of interest. These variables include: whether the memory is ogy histories and present diagnoses (Dalgleish & Brewin,
general or specific, latency to retrieve a memory, number of 2007; Hermans et al., 2006). Generally dysfunctional atti-
omissions (i.e., when a person does not present a specific tudes, whether in an individual exhibiting psychopathol-
personal memory for certain stimuli presented), age of mem- ogy or in a healthy individual, could play a part in an
ories, and affective tone. Studies have shown that the last of individuals inability to retrieve specific memories.
these variables, affective tone, can potentially differentiate
AMs of people with psychopathology from AMs of people
without psychopathology (Dalgleish & Brewin, 2007). Neuropsychology
Daselaar et al. (2008) have worked to map the time looking at schema activation and overgeneral AMs may
course of AM, including emotional and reliving aspects of be important to understanding why clinical populations
AM. Through functional magnetic resonance imaging remember AMs differently from nonclinical populations.
(fMRI), Daselaar et al. examined both initial accessing of Taking a closer look at the neuropsychology of AM
memories and subsequent elaboration of the memories. will be necessary as we find better ways to define AM
As a person first began to recall a personal memory, behaviorally. Correlating behavioral changes with the
hippocampal, retrosplenial, and medial and right prefron- brain changes we can see through imaging will be
tal cortex activation occurred (Daselaar et al., 2008). As a significant area for future study so that we develop a
participants rated memories, brain areas associated with clearer idea of what structures make up the AM circuitry
emotion and sensory function were activated, including the in the brain.
amygdala and hippocampus for initial emotion ratings,
and visual cortex and ventromedial and inferior prefrontal
cortex for reliving ratings. This line of research underlines Cross References
AMs dynamic involvement of multiple brain areas.
The visual, auditory, and olfactory systems all appear Declarative Memory
to be potential parts of the broad AM system. Research Episodic Memory
has demonstrated that visual imagery is central to AM, False Memory
especially when considering long-term visual memories Forgetting
(Greenberg & Rubin, 2003). The medial-temporal lobes Memory Impairment
and the frontal lobes have been implicated in case study Remote Memory
research examining visual imagerys role in AM. Auditory Working Memory
imagery may also be involved in AM, but so far studies
have not shown autobiographical amnesias to be related
specifically to damage of the auditory cortices. As with References and Readings
visual imagery, further research may provide more
information on this aspect of AM. Bluck, S. (Ed.). (2003). Autobiographical memory: Exploring its
functions in everyday life [Special issue]. Memory, 11.
In addition to its sensory dimensions, AM also seems
Dalgleish, T., & Brewin, C. R. (Eds.). (2007). Autobiographical memory
to be closely related to language. However, with only one and emotional disorder [Special issue]. Memory, 15(3).
exception, semantic dementia, AM impairment does not Daselaar, S. M., Rice, H. J., Greenberg, D. L., Cabeza, R., LaBar, K. S., &
seem to be related to language-related neuropsychological Rubin, D. C. (2008). The spatiotemporal dynamics of autobio-
impairments (Greenberg & Rubin, 2003). In semantic graphical memory: Neural correlates of recall, emotional intensity,
and reliving. Cerebral Cortex, 18, 217229.
dementia, patients display better recall for recent
Greenberg, D. L., & Rubin, D. C. (2003). The neuropsychology of
memories than for older ones. A patient with semantic autobiographical memory [Special issue]. Cortex, 39, 687728.
dementia experiences loss of AM, as well as a loss of the Hermans, D., Raes, F., Philippot, P., & Kremers, I. (Eds.). (2006).
ability to maintain semantic memories in storage. Autobiographical memory specificity and psychopathology. New York:
Psychology Press.
Levin, H. S., Benton, A. L., & Grossman, R. G. (1982). Neurobehavioral
consequences of closed head injury. New York: Oxford University
Future Directions Press.
Rubin, D. C. (Ed.). (1999). Remembering our past: Studies in autobio-
Future research on AM should concentrate on defining graphical memory. New York: Cambridge University Press.
and specifying AM both behaviorally and through Sutin, A. R., & Robins, R. W. (2007). Phenomenology of autobiographical
memories: The memory experiences questionnaire. Memory, 15(4),
neuropsychological techniques, including functional and
390411.
structural imaging. Continuing to consider and revise a Wenzel, A., & Rubin, D. C. (Eds.). (2005). Cognitive methods and their
conceptual model of AM, such as Pillemers, is important application to clinical research. Washington, DC: American Psycho-
to providing researchers a better, more definite way to logical Association.
conceptualize AM. Looking at AM cross-culturally may
help to determine whether this type of memory plays
distinct roles in different cultures and societies. Individual
differences in AM are also an important area for Autoimmune Disorders
study, given researchers focus on AM as important to
the formation of self. In research on psychopathology, Myasthenia Gravis
Automated Neuropsychological Assessment Metrics A 325
migraine headache (Roebuck-Spencer, Sun, Cernich, for accurate responses (MS), and the ratio of accuracy and
Farmer, & Bleiberg, 2007) and Alzheimers dementia speed or number of correct responses per minute
(Levinson, Reeves, Watson, & Harrison, 2005). Currently, (throughput score) (Jones et al., 2008).
in an effort to better identify the occurrence of traumatic
brain injury (TBI), the ANAM is also being used by the
US military to establish a baseline of neurocognitive Psychometrics
functioning prior to deployment for all Service members
(U.S. Army Medical Department, 2009). The subtests of the ANAM were selected from previously
Data from over nine studies suggest that varying established assessment instruments (e.g., Walter Reed Per-
combinations of ANAM subtest batteries are sensitive formance Assessment Battery, the Air Force Criterion
and specific in detecting neurocognitive change among Task Set, Navy Performance Evaluation Tests for Environ-
individuals with neurological disorders (Kane et al., mental Research) with research supporting their respec-
2007). Common uses include screening and triage, moni- tive sensitivity, reliability, and validity (Reeves et al.,
toring of disease progression, and detection of treatment 2007). As researchers have attempted to gather psycho-
and medication effects (Kane et al., 2007). metric data for the current ANAM, many have included
Other uses of the ANAM include evaluation of cogni- only subsets of the subtests in their studies rather than the
tive functioning in determining fitness for duty, neuro- entire ANAM battery.
toxicology, human factors engineering, and various fields Various combinations of ANAM subtests have been
of medicine such as aerospace, undersea, military opera- shown to be both sensitive and specific in detecting cog-
tions, and sports (Reeves et al., 2007). nitive changes in a number of neurological conditions
(Kane et al., 2007).
Through the process of multiple baseline administra-
Advantages tions, testretest reliabilities across several ANAM subtest
throughput scores ranged from 0.50 to 0.91 with 9 of the
The ANAM has been noted as an ideal instrument for 10 estimates <0.77(Short, Ivins, & Kane, manuscript in
assessing change in neurocognition (Roebuck-Spencer preparation).
et al., 2007). Through randomization of stimuli, practice Construct validity has been established between sev-
effects are minimized across numerous testing sessions eral of the commonly used ANAM subtests (e.g., Math,
(Roebuck-Spencer et al., 2007). Further, subtle changes Running Memory, Code Substitution Delayed Memory,
in response time can be more precisely detected as and Logical Reasoning) and more traditional measure of
compared to manual calculation of response time neurocognitive function such as Trail Making Test A&B,
(Roebuck-Spencer et al., 2007). Other advantages include Animal Naming, Controlled Oral Word Association Test,
time efficiency and cost-effectiveness, both of which are and the Digits Backward and Digit Symbol subtests of the
helpful when attempting to triage large numbers of WAIS-III (Short, Cernich, Wilken, & Kane, 2007).
patients (Kane et al., 2007).
Research findings indicate that varying combinations of The use of the ANAM in various fields of medicine and
the ANAM batteries are not as thorough as more extensive science continues to develop as evidenced by recent stud-
neurocognitive assessments. ies attempting to validate specified subsets of the ANAM
battery. Examples include validation of the ANAM-sports
medicine battery (ASMB) designed for surveillance and
Administration management of sports related concussions (Cernich,
Reeves, Sun, & Bleiberg, 2007), use of ANAM tests to
Administration time can range from a few minutes for a assess the effects of extreme environmental stressors
single subtest to 90 minutes for the entire battery (U.S. such as high altitude, toxins, and radiation exposure
Army Medical Department, 2009). Scoring for the ANAM (Lowe et al., 2007), and assessment of medication efficacy
is computer generated. Scores for ANAM subtests can be and potential medication side effects, specifically for
calculated in a variety of ways, including the percentage of CNS-active drugs (Wilken, Sullivan, Lewandowski, &
correct responses (accuracy score), mean response time Kane, 2007).
Automatic Language A 327
Cross References assessment metrics (ANAM): Issues and challenges. Archives of Clin-
A
ical Neuropsychology, 22, S79S87.
Short, P., Cernich, A., Wilken, J. A., & Kane, R. L. (2007). Initial construct
Cognitive Functioning validation of frequently employed ANAM measures through struc-
Concussion tural equation modeling. Archives of Clinical Neuropsychology, 22S,
Mild Brain Injury S63S77.
Traumatic Brain Injury Short, P., Ivins, B. J., & Kane, R. L. (manuscript in preparation). Reliabil-
ity characteristics of select Automated Neuropsychological Assess-
ment Metrics (ANAM) measures.
U.S. Army Medical Department (2009). Automated Neuropsychological
References and Readings Assessment Metrics (ANAM). www.medicine.army.mil/prr/anam.
html. Retrieved on 10 March 2009.
Vincent, A. S., Bleiberg, J., Yan, S., Ivins, B., Reeves, D. L., Schwab, K.,
Jones, W. P., Loe, S. A., Krach, K., Rager, R. Y., & Jones, H. M. (2008). et al. (2008). Reference data from the automated neuropsychological
Automated neuropsychological assessment metrics (ANAM) and assessment metrics for use in traumatic brain injury in an active duty
Woodcock-Johnson III tests of cognitive ability: A concurrent valid- military sample. Military Medicine, 173, 836849.
ity study. The Clinical Neuropsychologist, 22, 305320. Wilken, J. A., Sullivan, C. L., Lewandowski, A., & Kane, R. L. (2007). The
Kane, R. L., Roebuck-Spencer, T., Short, P., Kabat, M., & Wilken, J. use of ANAM to assess the side-effect profiles and efficacy of medi-
(2007). Identifying and monitoring cognitive deficits in clinical cation. Archives of Clinical Neuropsychology, 22S, S127S133.
populations using Automated Neuropsychological Assessment
Metrics (ANAM) tests. Archives of Clinical Neuropsychology, 22S,
S115S126.
Reeves, D. L., Winter, K. P., Bleiberg, J., & Kane, R. L. (2007). ANAM
genogram: Historical perspectives, description, and current endea- Automatic Behavior
vours. Archives of Clinical Neuropsychology, 22S, S15S37.
Roebuck-Spencer, T., Sun, W., Cernich, A. N., Farmer, K., & Bleiberg, J. Automatism
(2007). Assessing change with the automated neuropsychological
Stimulus-Bound Behavior
assessment metrics (ANAM): Issues and challenges. Archives of Clin-
ical Neuropsychology, 22, S79S87.
represent propositional language skill and should not be instead name the color of the stimulus. Reaction time
considered as a conscious attempt to participate in conver- tends to be slower when the color word and the color of
sational situations. Automatic language can be found in the stimulus are incongruent when compared to the time
severe aphasias and in many dementias. It may also occur to name the color of a series of Xs or a noncolor word.
in mental health problems such as schizophrenia.
Cross References
Cross References
Controlled Attention
Stereotypy Orienting Response
Reading Fluency
Stroop Effect
References and Readings
Automaticity
Automatism
A NNA M AC K AY-B RANDT
Brown University Medical School D OUGLAS I. K ATZ
Providence, RI, USA Boston University School of Medicine
Boston, MA, USA
Definition
Synonyms
A mental operation that proceeds without voluntary
control and without requiring capacity or processing Automatic behavior
resources.
Definition
Current Knowledge
This is a complex movement that occurs without
Automatic processes are usually found in the context of conscious awareness or purposeful intent.
stimulus information that is well integrated into the indi-
viduals memory through either a classical conditioning,
an overlearned behavior (e.g., reading), or an evolution- Current Knowledge
arily adaptive response (e.g., orienting response). The
Stroop effect (Stroop, 1935; MacLeod, 1992) is a good Automatisms may occur in the setting of complex-partial
example of the influence of automaticity on behavior. seizures. Typical simple movements include lip smacking,
Reading becomes automatic at a level of proficiency chewing, or finger rubbing. More complex automatisms
acquired by most school-aged children, such that it is include walking, running, undressing, and speaking. Emo-
out of an individuals control not read a presented word. tional expressions, such as laughing or crying, may also
This involuntary response is captured in the interference occur as automatisms. Automatisms may occur during
it produces when one attempts to ignore a color word and seizures or as post-ictal phenomena. Speech automatisms
Autonomic Nervous System A 329
Ebner, A., Dinner, D. S., Noachtar, S., & Luders, H. (1995). Automatisms
Insular cortex
with preserved responsiveness: A lateralizing sign in psychomotor
seizures. Neurology, 45(1), 6164.
Fenwick, P. (1990). Automatism, medicine and the law. Psychological
Medicine. Monograph Supplement, 17, 127. Paraventricular
Rasonyi, G., Fogarasi, A., Kelemen, A., Janszky, J., & Halasz, P. (2006). nucleus
Lateralizing value of postictal automatisms in temporal lobe
epilepsy. Epilepsy Research, 70(23), 239243. Central nucleus
of the amygdala
Lateral hypothalamic area
Parabrachial
region
Autonomic Nervous System
S COTT VOTA Nucleus of the
Virginia Commonwealth University tractus solitarius
Richmond, VA, USA
Nucleus ambiguus
Ventrolateral
Synonyms medulla
Internal regulation system; Involuntary nervous system; Autonomic Nervous System. Figure 1 Central components
Visceral nervous system of the autonomic nervous system
330 A Autonomic Nervous System
level of control over autonomic function. These intercon- gland (producing lacrimation) and the cerebral and cra-
nections ultimately descend to specific cells within the nial blood vessels (eliciting vasodilatation). Axons also
brainstem and spinal cord. The thoracolumbar outflow travel to the submandibular ganglion, providing secreto-
consists of fibers that arise in the intermediolateral cell motor and vasodilator inputs to the corresponding sali-
column of the thoracic and first two lumbar segments of vary glands. The inferior salivatory nucleus sends axons
the spinal cord. This is the origin of the sympathetic via the glossopharyngeal nerve to ultimately synapse on
division of the autonomic nervous system. The cranial the otic ganglion, stimulating parotid gland secretion.
outflow, arising from cranial nerve nuclei III, VII, IX, and Most preganglionic parasympathetic output from the
X, and the sacral outflow, arising from cell bodies in the brainstem is mediated by the vagus nerve, which receives
intermediate cell column of sacral segments 2 through 4, input from the dorsal motor nucleus of the vagus and the
form the parasympathetic division of the autonomic lateral portion of the nucleus ambiguus. The vagus nerve
nervous system. innervates the heart, respiratory tract, and the entire
gastrointestinal tract with the exception of the descending
colon and rectum.
Peripheral Component The sacral preganglionic output arises from neurons
of the sacral preganglionic nucleus located in the lateral
The peripheral part of the autonomic nervous system is gray matter of the sacral spinal cord. These axons travel to
then composed of sympathetic and parasympathetic the pelvic splanchnic nerves, which join the inferior hy-
pathways. These pathways arise from two distinct ana- pogastric plexus to innervate the descending colon, blad-
tomic portions of the brainstem and spinal cord. The der, and sexual organs. These outputs elicit contraction of
parasympathetic fibers arise from the craniosacral por- the bladder detrusor muscle and circular smooth muscle
tion, and the sympathetic fibers arise from the thoraco- of the rectum as well as regulating vasodilatation of the
lumbar region. Although anatomically separated, the two cavernous tissue of the penis required for erection.
parts are complementary in maintaining a balance in
the activities of many visceral structures and organs. The Sympathetic Nervous System
preganglionic neurons for both the parasympathetic and The sympathetic preganglionic neurons are primarily lo-
sympathetic divisions release acetylcholine, but the differ- cated in the intermediolateral nucleus in the thoracic and
ence lies in the postganglionic neurotransmitter release. upper lumbar regions of the spinal cord. The pregangli-
The parasympathetic postganglionic fibers release norepi- onic sympathetic axons exit through the ventral roots and
nephrine and epinephrine and thus are referred to as pass on to the corresponding spinal nerve to reach the
cholinergic. The sympathetic postganglionic fibers release paravertebral sympathetic chain. The majority of the pre-
norepinephrine and epinephrine and thus are classified as synaptic fibers branch and run rostrally or caudally along
adrenergic. The terminals of sympathetic fibers on sweat the sympathetic chain and synapse on the paravertebral
glands, however, do not follow this pattern and are cho- ganglia. The remaining fibers pass through the paraver-
linergic (see Figs. 2 and 3). tebral chain without synapsing. These form the splanch-
nic nerves which innervate prevertebral ganglia.
Parasympathetic Nervous System The paravertebral sympathetic ganglia are primarily a
Parasympathetic outputs arise from the preganglionic relay station for preganglionic inputs. They innervate
neurons located in the nuclei of the brainstem and sacral all tissues and organs except those in the abdomen, pelvis,
spinal cord. Preganglionic parasympathetic axons travel a and perineum. For example the superior cervical ganglion
long distance before eventually reaching their target gang- sends postganglionic axons to innervate the eye, facial
lia, which are typically close to, or within the target end sweat glands, salivary glands, pineal, thyroid, and para-
organ. thyroid glands. These outputs elicit pupil dilatation, con-
The cranial preganglionic parasympathetic nuclei traction of the Muller muscle of the eyelid, facial sweating,
(Edinger Westphal Nuclei) projects through the oculomo- and vasoconstriction in facial and cerebral circulation.
tor nerve. These preganglionic axons synapse on neurons The stellate ganglion, which receives preganglionic input
of the ciliary ganglion. The neurons innervate the iris and from the mid thoracic segment, sends postganglionic
ciliary muscles, eliciting pupillary constriction and ac- axons to innervate blood vessels and sweat glands in
commodation of the lens. The superior salivatory nucleus the upper limbs and trunk. These outputs produce either
located in the pons projects via the facial nerve to the vasoconstriction or vasodilatation in the skin and muscle,
sphenopalatine ganglion. This innervates the lacrimal sweating, or piloerection. Outputs from the stellate
Autonomic Nervous System A 331
Ciliary ganglion
A
Ciliary muscle
Sphenopalatine CN III
ganglion
Lacrimal gland
Submandibular CN VII
ganglion
Salivary glands
Parotid gland
CN IX
Otic ganglion
1
2
Larynx and trachea (CN X) 3
Lungs Vagus 4
Heart 5
6
Gastrointestinal tract 7
8
Abdominal organs
9
10
11
12
Large intestine 1
2
3
S24
Kidney
Bladder
Sex organs
ganglion also elicit cardiac acceleration and bronchodila- vasoconstriction, smooth muscle relaxation of the bladder
tion. The lumbar paravertebral ganglia subsequently and rectum, constriction of the internal sphincter of the
innervate the blood vessels and sweat glands in the lower bladder and rectum, and ejaculation.
limb.
The prevertebral ganglia are located anterior to the
abdominal aorta. Preganglionic input from the lower Diagnosing Autonomic Dysfunction
thoracic segments is carried by the splanchnic nerves to
the celiac and superior mesenteric ganglia and provides Medical History
postganglionic fibers to the celiac plexus that innervates As the autonomic nervous system innervates all organ
all abdominal viscera, with the exception of the descend- systems, a detailed medical history and physical examina-
ing colon. These outputs produce vasoconstriction and tion is paramount. This will help develop a proper differ-
inhibition of the gastrointestinal tract motility. Pregangli- ential diagnosis and laboratory evaluation. The goals of
onic axons from the lumbar spinal segments travel via the clinical evaluation are to identify the presence, loca-
the lumbar splanchnic nerves to synapse in the inferior tion, and time course of autonomic dysfunction. This will
mesenteric ganglion. These axons innervate the descend- help to determine which part(s) of the autonomic ner-
ing colon, rectum, bladder, and sexual organs eliciting vous system may be involved: sympathetic noradrenergic,
332 A Autonomic Nervous System
Ciliary muscle
Lacrimal gland
Salivary glands C1
Larynx and 1
T1
trachea 2
3
Lungs 4
5
Celiac
ganglion 6
Heart
7
Stomach 8
Small intestine 9
10
Adrenal medulla 11
12
1
Superior mesenteric 2
ganglion 3
Large intestine
L1
Kidney
Bladder
Inferior
Sex organs mesenteric
ganglion
sympathetic cholinergic, parasympathetic cholinergic, making note of acrocyanosis, pallor, mottling, diaphore-
or adrenomedullary. Specific questions should be asked sis, alopecia, or erythema should be performed. An
to determine if the patient may have symptoms of ortho- eye exam can also be valuable. Attention should be given
static hypotension, anhydrosis, weight change, constipa- to pupillary shape, size, and the response to light and
tion, sexual dysfunction, sialorrhea, or urinary retention. accommodation.
Questions related to aggravating and relieving factors
need to be considered. Examples include: relationship to Diagnostic Testing
meals; environmental temperature; and diurnal variation. A workup to uncover an etiology for autonomic dys-
A complete listing of all prescribed medications, as well as function should begin with routine serologic testing.
over the counter herbal and dietary supplements need to This includes serum electrolytes, glucose, hepatic function
be reviewed. tests, protein electropheresis, and a complete blood count.
Further serologic testing may include cortisol levels, para-
Examination neoplastic autoantibodies, and plasma catecholamines.
An exam should start with a general overview of the An EKG and echocardiogram should also be performed.
patient making note of facial expression, posture, and A variety of more specialized tests, both invasive and
height. Vital signs should be checked in the supine, seated, noninvasive, may also need to be considered. These in-
and standing positions. A thorough skin examination clude, but are not limited to the following: deep breathing
Autotopagnosia A 333
Carey, R., DePalma, G., Damianopoulos, E., Muller, C., & Huston, J.
(2004). The 5-HT1A receptor and behavioral stimulation in the rat:
References and Readings Effects of 8-OHDPAT on spontaneous and cocaine-induced behav-
ior. Psychopharmacology, 177(1/2), 4654.
Meltzer, H. (1980). Relevance of dopamine autoreceptors for psychiatry:
Benarroch, E. E., Westmoreland, B. F., Daube, J. R., Reagan, T. J., & Preclinical and clinical studies. Schizophrenia Bulletin, 6(3), 456475.
Sandok, B. A. (1999). Medical neurosciences: An approach to
anatomy, pathology, and physiology by systems and levels (4th ed.).
Philadelphia: Lippincott Williams and Wilkins.
Bradley, W. G., Daroff, R. B., Fenichel, G., & Jankovic, J. (2004). Neuro-
Autotopagnosia
logy in clinical practice (4th ed.). Boston: Butterworth-Heinemann.
Gilman, S., Newman, S. W., Manter, J. T., & Gatz, A. J. (2003). Manter J OHN E. M ENDOZA
and Gatzs essentials of clinical neuroanatomy and neurophysiology Tulane University Medical Center
(10th ed.). Philadelphia: F.A. Davis Company.
New Orleans, LA, USA
Netter, F. H. (1991). Nervous system, Part 1: Anatomy and physiology
(Vol. 1) (Ciba collection of medical illustrations). Summit, NJ:
Ciba-Geigy Corporation.
Ropper, A. H., & Brown, R. H. (2005). Adams and Victors principles of
Definition
neurology (8th ed.). New York: McGraw-Hill.
Rowland, L. P. (2005). Merritts neurology (11th ed.). Philadelphia: Disturbance of body schema involving the loss of ability to
Lippincott Williams and Wilkins. localize, recognize, or identify the specific parts of ones body.
334 A AVMs
While some reported cases exhibit impaired knowledge of Avolition is a severe problem with initiation, volitional, or
most body parts, autotopagnosia is most frequently man- willed action, and production of goal-directed behavior. It
ifested as difficulty in identifying or naming specific fingers may reflect a general lack of motivation and drive.
(finger agnosia), especially the three middle fingers. The Avolition is commonly seen as one of the negative
problem extends to identifying comparable body parts on symptoms in patients with schizophrenia, and is also
the examiner or graphic representations of body parts. The common in frontal lobe disorders affecting medial frontal
deficit usually involves both sides of the body, thus distin- systems.
guishing it from unilateral neglect. While not typically
classified as such, right-left disorientation likely reflects
another form or subtype of autotopagnosia. In this condi- Cross References
tion, patients are unable to reliably identify the right and left
sides of their bodies or those of others. Both finger agnosia Abulia
and right-left disorientation are frequently present at the Apathy
same time, particularly following lesions of the left angular Cingulate Gyrus
gyrus and may be a part of what has been defined as Gerst-
manns syndrome. The latter would also include deficits in
writing (agraphia) and arithmetical operations (acalculia).
References and Readings
Cross References
Foussias, G., & Remington, G. (2008). Negative symptoms in schizophre-
nia: Avolition and Occams Razor. Schizophrenia Bulletin Advance
Finger Agnosia Access 10.10.1093/schbul/sbn094.
Gerstmanns Syndrome Liddle, P. F. (1994). Volition and schizophrenia. In A. S. David &
Right-Left Disorientation J. C. Cutting (Eds.), The neuropsychology of schizophrenia
(pp. 3948). Hillside, UK: Lawrence Erlbaum.
Rummel, C., Kissling, W., & Leucht, S. (2005). Antidepressants as add-on
References and Readings treatment to antipsychotics for people with schizophrenia and pro-
nounced negative symptoms: A systematic review of randomized
Benton, A. L., & Sivan, A. B. (1993). Disturbances of the body schema. In trials. Schizophrenia Research, 80, 8597.
K. M. Heilman & E. Valenstein (Eds.), Clinical neuropsychology
(pp. 123140). New York: Oxford University Press.
Denberg, N. L., & Tranel, D. (2003). Acalculia and disturbances of the
body schema. In K. M. Heilman & E. Valenstein (Eds.), Clinical
neuropsychology (pp. 161184). New York: Oxford University Press.
Avonex
AVMs Beta-Interferons
Vascular Malformations
Avolition Awareness
I RENE P IRYATINSKY, PAUL M ALLOY Alertness
Butler Hospital and Alpert Medical School of Brown Consciousness
University
Providence, RI, USA
E DISON WONG
Center for Pain & Medical Rehab References and Readings
Fitchburg, MA, USA
Babinski, J. (1896). Sur le reflexe cutane plantaire dans certaines
affections organiques du systeme nerveux central. Comptes rendus
Synonyms des seances de la Societe de biologie et de ses filiales, 48, 207208.
Campbell, W. W. (2005). DeJongs the neurological examination (pp. 324,
331, 339). Philadelphia: Lippincott, Williams & Wilkins.
Long tract sign; Plantar reflex; Upper motor neuron sign Getz, C. (1985). Introduction to the motor systems. In E. R. Kandel &
J. H. Schwartz (Ed.), Principles of neural science (pp. 429442).
New York: Elsevier.
Definition Landau, W. M., & Clare, M. H. (1959). The plantar reflex in man, with
special reference to some conditions where the extensor response is
unexpectedly absent. Brain, 82, 321335.
The Babinksi reflex or sign is elicited by making contact
along the lateral side of the plantar foot with a blunt
implement and not causing pain, discomfort or injury
to the skin; the implement is run from the heel along a
curve to the metatarsal pads. There are three responses
possible:
Background Information
Extensor (positive or pathological): hallux (great toe) History (Medical, Social, Psychological)
extension and the other toes abduct
Flexor (negative or normal): all toes flex and the
foot everts
Indifferent: no response
Backward Digit Task
Current Knowledge Digit Span
Epidemiology
BADS
Aging may also affect balance. Approximately 40% of B
Behavioral Assessment of the Dysexecutive Syndrome people older than age 65 suffer falls each year. Vertigo is
the most common form of dizziness.
Balance Disorders
A NNA D E P OLD H OHLER 1, M ARCUS P ONCE DE LEON2 Evaluation
1
Boston University Medical Center
Boston, MA, USA The history and physical examination often lead to a diag-
2
William Beaumont Army Medical Center nosis. At times, laboratory tests and imaging are obtained for
El Paso, TX, USA confirmation or to rule out harmful diagnoses. If a reversible
cause is found and treated, significant recovery may occur.
However, if the balance problem is due to a permanent
Definition or progressive neurological deficit, the patient may need
training to manage their gait and balance difficulties.
Normal balance requires the integration of three sensory
systems: visual, vestibular (found in the inner ear), and
somatosensory (sensations from the skin, muscles, tendons, Treatment
and joints) in addition to muscle strength. When these
systems are impaired, individuals may experience episodes Physical therapy and vestibular rehabilitation may be
of spinning, light-headedness, trouble focusing their eyes, useful in appropriate cases. They may improve current
and/or poor balance or falls. functioning and potentially decrease the potential for
progression of deficits and complications from falls.
Categorization
Cross References
Balance may be affected by disturbances of strength in
the trunk or legs, sensation deficits, or difficulties with Ataxia
coordination. Multiple systems may be affected. A Parkinsons Disease
detailed history and neurological examination may help
detect the affected area. Balance may be impaired after a
focal event such as a stroke or may develop during the References and Readings
course of a neurodegenerative disease such as Parkinsons
disease. Medications and infections of the brain or inner Ackley, S., Newell Decker, T., & Limb, C. J. (2007). An essential guide to
ear may also contribute to balance difficulties. hearing and balance disorders. Psychology Press.
340 B Balint, R. (Rezso (Rudolf) Balint) (18741929)
index fingers, spaced far apart and held at various loca- one of the most common inhibitory neurotransmitter
tions across the patients visual field. The latter can be systems in the brain. Their behavioral effects include
tested in response to a person passing by or to a loud relaxation, drowsiness, and feelings of euphoria. However,
unexpected noise occurring in the periphery. their widespread effects also result in the depression of
reflexes and cardiovascular and respiratory functions,
particularly at higher doses (Feldman et al., 1997).
Treatment
The psychoactive effects of barbiturates increase
their risk for drug dependence and abuse. Symptoms of
Relatively little is known about treatment of patients with
tolerance and withdrawal develop with chronic use; how-
Balints syndrome. Rehabilitation often utilizes a func-
ever, greater tolerance develops to the psychoactive effects,
tional approach in which the patients strengths are used
but less so to the respiratory depressant effects of
to offset impairments. There is some evidence to suggest
barbiturates, resulting in an increased risk of a toxic
that cognitive and perceptual rehabilitation approaches
overdose (Feldman et al., 1997). Cross tolerance with
using verbal cues and organizational search strategies
other substances may also occur. For example, alcohol
can improve visual function and reaching abilities
use may also increase tolerance to barbiturates, further
(Perez, Tunkel, Lachmann, & Nagler, 1996).
increasing the risk of a toxic overdose.
Cross References
Current Knowledge
Hemiinattention
The use of barbiturates has declined significantly with the
Simultanagnosia
development of other anxiolytic and anticonvulsant
Visual Field Deficit
medications. Benzodiazepines, which are also anxiolytic
compounds that interact with the GABAA receptor
References and Readings (although a different site than barbiturates), have a larger
therapeutic window than barbiturates and have replaced
Perez, F. M., Tunkel, R. S., Lachmann, E. A., & Nagler, W. (1996). Balints their use as a safer alternative for the treatment of anxiety.
syndrome arising from bilateral posterior cortical atrophy or infarc-
tion: Rehabilitation strategies and their limitation. Disability and
Rehabilitation, 18, 300304. Cross References
Benzodiazepines
Gamma-Aminobutyric Acid (GABA)
Barbiturates
J OANN T. T SCHANZ 1, K ATHERINE T REIBER 1,2 References and Readings
1
Utah State University
Feldman, R. S., Meyer, J. S., & Quenzer, L. F. (1997). Sedative-hypnotic
Logan, UT, USA
2 and anxiolytic drugs. In Principles of neuropsychoparhmacology
University of Massachusetts Medical School (pp. 673729). Sunderland, MA: Sinauer.
Worcester, MA, USA
Historical Background Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (1997).
Psychological evaluations for the courts: A handbook for mental health
professionals and lawyers. New York: Guilford.
Thomas A. Barefoot burned down a bar and shot and Monahan, J. (1992). Mental disorder and violent behavior: Perceptions B
killed a police officer who was investigating the arson. and evidence. American Psychologist, 47, 511521.
Barefoot was convicted by the jury of capital murder of Monahan, J., & Steadman, H. J. (1994). Toward a rejuvenation of risk
a police officer. During the death penalty phase of the assessment research. In J. Monahan, & H. J. Steadman (Eds.),
Violence and mental disorder: Developments in risk assessment.
case, the state used psychiatric testimony to demonstrate
Chicago: University of Chicago Press.
that Barefoot posed a threat to society in the future. Mossman, D. (1994). Assessing predictors of violence: Being accurate
Specifically, the state had Drs. John Holbrook and James about accuracy. Journal of Consulting and Clinical Psychology,
Grigson review a hypothetical fact situation based on 62, 783792.
evidence from the case and asked each of the doctors if
the convicted individual would commit violent acts in the
future or would pose a threat to society. Both doctors
testified that the criminal would be a continued threat
to society. In fact, Dr. Grigson concluded that there was
Barona Index
a one hundred percent and absolute probability
G LEN E. G ETZ
that Barefoot would commit violent acts in the future
Allegheny General Hospital
and thus pose a continued threat to society. The judge
Pittsburgh, PA, USA
sentenced Thomas A. Barefoot to death. Barefoot
appealed the decision and in the Court of Criminal
Appeals raised several concerns about the way his trial
was handled, most notably with respect to the probability
Synonyms
that he would commit future violent acts. Barefoot argued
Premorbid intelligence regression model
that the psychiatrists testifying against him had not even
examined him and were making determinations based on
a hypothetical fact-based situation. Moreover, Barefoot
called into question the ability of psychiatrists to predict Definition
future dangerousness. The Court of Criminal Appeals
rejected all of Barefoots arguments and the U.S. Supreme Barona Index is a demographically based regression
Court rejected Barefoots suggestion that psychiatrists method to estimate premorbid intelligence in terms of
are not competent to make determinations regarding index scores on the Wechsler Adult Intelligence Scale-
dangerousness in future. The U.S. Supreme Court Revised (WAIS-R).
ruled that psychiatrists are no less reliable than laypersons
and that laypersons testimony of future dangerousness is
indeed permissible. The Court upheld that the use of Historical Background
hypothetical questions to establish future dangerousness
is just because such testimony is supported by the Federal Attempts have long been made to estimate premorbid
Rules of Evidence (FRE) that death penalty cases do not intellectual functioning. A frequent method in clinical
present special evidentiary problems. Furthermore, there practice is to estimate the level of premorbid cognitive
is evidence (e.g., Monahan, 1992; Monohan & Steadman, skill by subjectively considering aspects of the individuals
1994; Mossman, 1994) to suggest that mental health history such as education and occupation. Another com-
professionals do indeed predict violence significantly mon approach to estimate premorbid IQ is to use tests of
better than chance when relevant factors are included present ability, which are thought to be relatively resistant
in the determination. to change even during the phases of a psychiatric disorder
or those following a neurologically based disorder. A
variant is the best performance method in which the
References and Readings highest score obtained by an individual is assumed to be
the most likely premorbid level ( Best Performance
Denney, R. L. (2005). Criminal responsibility and other criminal forensic
Method). Research has been inconsistent as to the
issues. In G. Larrabee (Ed.), Forensic neuropsychology: A scientific effectiveness of this approach. In an attempt to reduce
approach. New York: Oxford University Press. the error in estimating intelligence based on current
344 B Barona Index
functioning and eliminate the subjectivity inherent in these equations resulted in less IQ variance and larger
clinical judgment, demographically based regression standard errors of estimate, cross validation studies were
equations were created to statistically predict intelligence successful.
test scores. A later method of combining demographic
information and current performance on IQ has also been
Current Knowledge
found to be relatively effective.
It is well established that demographic variables, such
Currently, premorbid estimation of IQ functioning
as education, social class and education, are correlated
includes the WAIS-III (Wechsler, 1997). Algorithms
with measured IQ. Wilson et al. (1978) created a regres-
derived from the WAIS-III with demographic variables
sion equation to predict WAIS IQ from demographic
have been developed by the Oklahoma Premorbid Intelli-
variables. They used regression modeling with WAIS Full
gence Estimate (OPIE-3). Four sets of algorithms using
Scale IQ, Verbal IQ, and Performance IQ as criteria and
between one and four WAIS-III subtests have been devised.
age, education, sex, race, and occupation as predictors.
Research consistently suggests that OPIE-3 methods are
With the development of the WAIS-Revised (WAIS-R),
reasonably effective in estimating premorbid intelligence.
further models were needed to estimate premorbid intel-
ligence. Baron, Reynolds, and Chastain (1984) generated
demographic equations for the estimation of premorbid Future Directions
WAIS-R IQ. Subsequently, research demonstrated suc-
cessful discrimination of neurologically based patients As we are on the brink of the release and utilization of
from non-neurologically based patients utilizing the the Wechsler Adult Intelligence Scale-IV, it is quite likely
WAIS-R. As demonstrated in Fig. 1, the predictor vari- that future regression models to estimate premorbid func-
ables incorporated into the model included those origi- tioning as indexed by scores on this test will be developed.
nally utilized by Wilson et al. (1978) as well as urban/rural It is necessary to continue to improve our methods of
residency, geographic location, and handedness. Although estimating premorbid abilities. Future models will most
Barona Index. Figure 1 Barona et al. (1984) regression formulas for pre-morbid IQ
Barthel Index B 345
likely consider other variables and/or include more spe- grooming, dressing, bowel and bladder continence, toilet-
cific criteria for the existing models. For example, the ing, transfers, mobility, and stair use. Items are weighted
expansion of technology along with fewer labor-based and scored according to their perceived importance. B
jobs will very likely influence the occupations used for Higher scores indicate better performance. In the most
the equation. As age expectancy increases, the role of age commonly used version, the maximum score of 100 indi-
on premorbid functioning will quite likely become a more cates full independence. Several versions of the Barthel
important variable as well. Index and their associated scoring methods exist. (Shah
et al. 1989) expanded the scoring categories to improve
the scale discriminability. Others have simplified the scor-
ing system, while incorporating additional categories,
Cross References
to sum to a maximum of 20 points.
Best Performance Method
Intelligence
Premorbid Estimate
Premorbid Functioning
Historical Background
The BI evolved over a 10-year period from the mid-1950s
until its publication in 1964. It was developed to per-
References and Readings mit nursing staff to assess the ability of patients with
neuro-muscular and musculoskeletal disorders to care
Barona, A., & Chastin, R. L. (1986). An improved estimate of premorbid for themselves. It was one of the first measures of activities
IQ for black and whites on the WAIS-R. International Journal of
of daily living (ADL) to be developed. Since its initial
Clinical Neuropsychology, 8, 169173.
Barona, A., Reynolds, C. R., & Chastain, R. (1984). A demographically
publication, it has been modified to both expand and
based index of premorbid intelligence for the WAIS-R. Journal of restrict the item scoring. The BI is widely used in rehabili-
Consulting and Clinical Psychology, 52, 885887. tation centers, despite subsequent investigations identify-
Schoenberg, M. R., Scott, J. G., Duff, K., & Adams, R. L. (2002). Estima- ing problems with the scaling and sum-scoring system.
tion of WAIS-III intelligence from combined performance and de-
The BI remains popular as it includes the key physical and
mographic variables: Development of the OPIE-3. The Clinical
Neuropsychologist, 16, 426438.
self-care items important for discharge planning and is
Wilson, R. S., Rosenbuam, G., Broan, G., Rourke, D., Whitman, D., & simple to use.
Grisell, J. (1978). An index of premorbid intelligence. Journal of Following the appearance of the BI, many other
Consulting and Clinical Psychology, 46, 15541555. indices of function have been developed, underlining the
importance of this type of tool in rehabilitation practice.
The BI and the Functional Independence Measure (FIM)
are the two most widely used measures of ADL in stroke
research. The BI tends to be used more frequently in
Barthel Index Europe while the FIM is more likely to be used in North
America.
G AVIN W ILLIAMS
Epworth Hospital
Melbourne, Victoria, Australia
Psychometric Data
Synonyms The original version of the BI was developed without the
investigation of content validity for item inclusion or
BI
validity of the scoring system. Many authors have ques-
tioned and subsequently suggested modifications to the
scoring system. Most recently, de Morton et al. (2008)
Description used Rasch analysis to investigate the validity of item
score summation for the BIs original and modified ver-
The Barthel Index (BI) measures ten functions that are sions. They found that score summation was not valid and
important for independent living feeding, bathing, although rescoring may improve the validity of the data
346 B Basal Forebrain
Cross References
Cross References
Functional Independence Measure (FIM)
Rivermead Mobility Index Anterior Communicating Artery
Organization. The striatal complex is composed of the GP (Haines, 2004). Three major fiber tracts are associated
neostriatum (caudate and putamen) and ventral striatum with the STN: the subthalamic fasciculus (STF), the ansa
(nucleus accumbens and olfactory tubercle) (Haines, lenticularis (AL), and the lenticular fasciculus (LF). The
2004). Embryologically the same, the caudate and puta- STF connects the STN and GP, crossing the internal cap-
men are separated by the internal capsule. Striosomes and sule; the AL connects the GPi and the thalamus and differs
matrix constitute a chemical and functional separation of from the STF in that it does not directly cross the internal
the striatal complex: striosomes are areas of low acetyl- capsule. Lastly, the LF crosses the internal capsule and
cholinesterase and high neuropeptide content, whereas ultimately joins the AL to form the thalamic fasciculus
matrix regions are rich in acetylcholinesterase (Bernacer, (or the H1 Field of Forel).
Prensa, & Gimenez-Amaya, 2007). This difference in Subthalamic function. The STN is thought to modu-
acetylcholinesterase content provides a convenient histo- late the entire circuitry of the basal ganglia (Hamani,
chemical differentiation between neostriatal regions Saint-Cyr, Fraser, Kaplitt, & Lozano, 2004).
(DiFiglia, Pasik, & Pasik, 1976). The GP consists of two segments: internal (medial,
Striatal function. The GABAergic cells of the striatum GPi) and external (lateral, GPe). The nucleus is bounded
project to the internal segment of GP and substantia nigra medially by the internal capsule, and laterally by
(striosomes project mainly to pars compacta; matrix pro- the putamen (Haines, 2004). Frequently, the term lenti-
jects mainly to pars reticulata). These nuclei also receive form nucleus is used to refer to the GP and putamen
substance-P and enkephalinergic input from the striatum together.
(Menguala, de las Herasb, Erroa, Lanciegoa, & Gimenez- Pallidal function. The internal segment tonically inhi-
Amaya, 1999). The striatum tonically inhibits its pallidal bits the ventroanterior and ventrolateral nuclei of the
and nigral targets. thalamus. The external segment tonically inhibits the
The striatum itself receives inhibitory GABAergic pro- STN and provides transient inhibition to the internal
jections from substantia nigra pars reticulata (Boyes & segment (DeLong & Wichmann, 2007). It is convenient
Bolam, 2007). Major excitatory input is found in gluta- to consider the GP as the gateway between the basal
matergic projections from thalamus (centromedian and ganglia and the thalamus. The thalamus, in turn, relays to
parafascicular nuclei) and cortex (several motor areas), as the motor areas of the cortex.
well as dopaminergic input from substantia nigra pars The basal ganglia have been described in terms of
compacta (Kubota et al., 1987). The latter dopaminergic functionally opposing direct and indirect pathways.
input terminates in both D1 and D2 dopamine receptor Broadly, the direct pathway promotes VA/VL thalamic
subtypes, an important determinant in excitation or inhi- relay to cortex, while the indirect pathway inhibits such
bition of striatal neurons (Surmeier, Ding, Day, Wang, & traffic. The following description of direct and indirect
Shen, 2007). pathways is a summary and integration of previous
The substantia nigra (SN) generally refers to two nu- sources.
clei, pars compacta and pars reticulata (SNpc, SNpr, Direct pathway. The VA/VL thalamic complex is under
respectively). The SN lies within the midbrain, caudal to tonic inhibition from both GPi and SNpr; transient inhi-
the crus cerebri and rostral to the red nucleus (Haines, bition of these nuclei is provided by the striatum. In this
2002). The SNpc contains dopaminergic neurons, while way, excitation of the striatum inhibits GPi output to the
the SNpr contains mostly GABAergic neurons. Intra- thalamus, and the net effect is disinhibition of the VA/VL
nigral connections serve as modulatory loops: GABAergic thalamic complex. The activation of striatal GABAergic
input to SNpc decreases dopaminergic activity within the projections to SNpr and GPi has two sources: cortical
pars compacta; dopaminergic input to SNpr decreases glutamatergic stimulation and nigral dopaminergic stim-
GABAergic activity (Boyes & Bolam, 2007; DeLong & ulation acting upon D1 striatal receptors. In this way, the
Wichmann, 2007). direct pathway is a case of thalamic disinhibition by
Nigral function. The pars reticulata provides tonic suppression of GPi activity.
inhibition of the thalamus, while the major function of Indirect pathway. If the direct pathway is considered as
the pars compacta is dopaminergic input to the striatum a suppression of GPi activity leading to disinhibition of
(Haines, 2002). the thalamus, the indirect pathway is described as sup-
The subthalamic nucleus (STN) is inferior to the thal- pression of the GPe leading to disinhibition of STN. Tonic
amus and medial to the GP; a biconvex-shaped structure, inhibition of STN comes from GPe (whereas tonic inhibi-
the STN is surrounded by dense bundles of myelinated tion of thalamus comes from GPi). The striatum serves to
fibers. The internal capsule separates the STN from the transiently inhibit GPe (as well as inhibit GPi as
Basal Ganglia B 349
previously described). The striatum contains both D1 and Corticostriatal: glutamatergic, from primary-, pre-,
D2 dopamine receptors. While the direct pathway uses D1 supplementary-, and cingulate-motor areas
receptors, the D2 subtype is the main striatal receptor of Nigrostriatal: dopaminergic from pars compacta; B
the indirect pathway. SNpc inhibits striatal output to GPe fibers terminate on two separate dopamine receptor
through these D2 receptors. types; also GABAergic from pars reticulata
In general terms, activity through the direct pathway
promotes thalamocortical activity by disinhibition of the
thalamus; the indirect pathway suppresses thalamocor- Efferent
tical activity. This opposing circuitry is thought to mod- Striatopallidal: GABAergic and substance-P projections to
ulate the net effect of the basal ganglia on thalamic internal segment; GABAergic and enkephalin projections
output. to external segment of globus pallidus
Striatonigral: striosomal GABAergic projections to
pars compacta; matrix GABAergic and enkephalinergic
Illness projections to pars reticulata
Efferent Thalamus
Subthalamopallidal and subthalamonigral (see Tremor
above)
Bergman, H., Feingold, A., Nini, A., Raz, A., Slovin, H., Abeles, M., et al.
Action Tremor
(1998). Physiological aspects of information processing in the basal
Afferent ganglia of normal and parkinsonian primates. Trends in Neurosci-
Assisted Living ence, 21(1), 3238.
Ataxia Bernacer, J., Prensa, L., & Gimenez-Amaya, J. M. (2007). Cholinergic
Bradykinesia interneurons are differentially distributed in the human striatum.
PLoS ONE, 2(11), e1174.
Caudate Nucleus
Bonelli, R. M., Wenning, G. K., & Kapfhammer, H. P. (2004). Hunting-
Cerebral Cortex tons disease: Present treatments and future therapeutic modalities.
Cholinesterase Inhibitors International Clinical Psychopharmacology, 19(2), 5162.
Chorea Boyes, J., & Bolam, J. P. (2007). Localization of GABA receptors in the
Cortical Motor Pathways basal ganglia. Progress in Brain Research, 160, 229243.
Brown, R., & Marsden, C. (1991). Dual task performance and processing
CorticalSubcortical Loop
resources in normal subjects and patients with Parkinsons disease.
Corticobasal Ganglionic Degeneration Brain, 114, 215231.
Corticobasilar Degeneration Brown, R., Soliveri, P., & Jahanshahi, M. (1998). Executive process in
Deep Brain Stimulator (Parkinsons) Parkinsons disease random number generation and response
Diencephalon suppression. Neuropyschologia, 36, 13551362.
Centonze, D., Bernardi, G., & Koch, G. (2007). Mechanisms of disease:
Dopamine Related Dyskinesia
Basic-research-driven investigations in humansthe case of hyperki-
Dystonia netic disorders. Nature Clinical Practical Neurology, 3(10), 572580.
Efferent Chang, H. T. (1988). Dopamine-acetylcholine interaction in the rat
Essential Tremor striatum: A dual-labeling immunocytochemical study. Brain Re-
Executive Functioning search Bulletin, 21, 295304.
DeLong, M. R., & Wichmann, T. (2007). Circuits and circuit disorders of
Gait Disorders
the basal ganglia. Archives of Neurology, 64, 2024.
Globus Pallidus DiFiglia, M., Pasik, P., & Pasik, T. (1976). A Golgi study of neuronal types
Huntingtons Disease in the neostriatum of monkeys. Brain Research, 114, 245256.
Internal Capsule Grahn, J., & Brett, M. (2007). Rhythm and beat perception in motor areas
Masked Facies of the brain. Journal of Cognitive Neuroscience, 19, 893906.
Grahn, J., & Brett, M. (2008). Impairment of beat-based rhythm discrim-
Mesolimbic Dopaminergic Projections
ination in Parkinsons disease. Cortex, 45, 5461.
Midbrain Haines, D. (2002). Fundamental neuroscience. New York: Churchill
Movement Disorders Livingstone.
Pallidotomy Haines, D. (2004). Neuroanatomy: An atlas of structures, sections, and
Pallidum systems. Philadelphia: Lippincott Williams & Wilkins.
Hamani, C., Saint-Cyr, J., Fraser, J., Kaplitt, M., & Lozano, A. (2004). The
Parkinson Plus Syndromes
subthalamic nucleus in the context of movement disorders. Brain,
Parkinsons Disease 127(Pt 1), 420.
Parkinsonian Movements Huntington Study Group (1996). Unified Huntingtons disease rating
Parkinsons Dementia scale: Reliability and consistency. Movement Disorders, 11, 136142.
Physiologic Tremor Johnson, T., Rosvold, H., & Mishkin, M. (1968). Projections from
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Postural Tremor
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Supplementary Motor Area ganglia functions: A review and proposal for a model of sensory
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Base Rate (Population) B 351
Administration
BASI
The tests can be administered individually or in groups,
Basic Achievement Skills Inventory timed or untimed; the comprehensive form takes about
2 h and the survey form takes about 50 min to complete.
The BASI is a newly developed test published in 2004. It students; (4) make placement decisions for ESL, GED, and
was developed with the assistance of teachers who wrote the program placement; (5) track academic progress, etc.
test items, which were then reviewed by curriculum experts. Specific applications in four settings are proposed in the B
Content was based on curriculum standards from The BASI Flash Demo (http://www.pearsonassessments.
Model Curriculum and Assessment Database (MCAD), a com/basidemo/basi.swf): (1) K-12 school/educational
database used by educators to align with district, state, and setting, (2) corrections setting for intake and evaluation of
national curriculum requirements and standards. offenders for placement in programs, (3) public safety for
employment screening, and (4) adult and child clinical
setting. Since BASI is a relatively new test, there are limited
research data to support its use across the wide variety of
Psychometric Data proposed settings. Literature search using PSYCINFO and
ERIC databases identifies one study (Griffith, 2006) using
Standardization is reportedly based on stratified random
the BASI with military students.
sampling to match closely with the US Census 2000.
Rhoades (2007) and Trevisan (2007) cautioned against
For the comprehensive form, a grade-appropriate sample
individual administration of the BASI due to the lack of
was stratified according to gender, race, parental educa-
standardization procedures. In adult and child clinical set-
tion, and region. Standardization of Form A was based on
tings, the BASI Comprehensive Form is recommended to
2,439 students tested during Fall 2002 and standardiza-
be used as a time- and cost-effective screening, providing an
tion of Form B was based on 2,130 students tested in
overview of achievement or alternative for individually
Spring 2003. The survey form included a school-age stan-
administered achievement test when detailed information
dardization sample of 2,518 students (aged 818) tested in
is not needed. The test is not recommended for diagnostic
school settings and an adult sample of 2,452 adults (aged
purposes but serves only as a screener to assist in the
1980) recruited in a variety of settings.
diagnostic process. There is no current support of its use
The test has good reliability. Buros Institute test
in neuropsychological testing. BASI appears best for cost-
reviewers (Rhoades, 2007; Trevisan, 2007) reported the
effective, group-based assessment and has been selected to
testretest stability, internal consistency, and alternate-
be used in educational and forensic settings such as pre- and
forms reliability to be fairly strong, with estimates ranging
posttesting under the Florida Juvenile Justice Common
from 0.54 to 0.96 for individual subtest scores and
Assessment Program. When more research data are avail-
0.670.98 for composite scores.
able, the potential use of the BASI can be reexamined.
Test validity is established through the Iowa Tests of
Basic Skills (ITBS), the Iowa Tests of Education Develop-
ment (ITED), the Tests of Adult Basic Education (TABE),
the Wechsler Individual Achievement Test Second Cross References
Edition (WIAT-II) and the Woodcock Johnson Psycho-
educational Battery III (WJ-III). A review of the data Academic Skills
(http://www.pearsonassessments.com/basi-correlation.
htm) indicates that intercorrelations between subtests are
supported; correlation coefficients range from low to References and Readings
high. Rhoades (2007) noted that correlations between
subtests with similar constructs are not particularly Griffith, R. (2006). An examination of factors influencing differences in
academic performance among active duty military students and
strong. Since some of the correlation studies were based
naval reserve officer training corps scholarship students in a univer-
on small sample size of students (around 40), unstable sity setting. Dissertation Abstracts International Section A, 67, 2071.
correlations are not unusual (Trevison, 2007). Retrieved from PsycINFO database.
Rhoades, E. K. (2007). Test review of the basic achievement skills inven-
tory. In K. F. Geisinger, R. A. Spies, J. F. Carlson, & B. S. Plake (Eds.),
The seventeenth mental measurements yearbook [Electronic Version].
Clinical Uses Retrieved February 17, 2009, from the Buros Institutes Test Reviews
Online website: http://unl.edu/buros
Trevisan, M. S. (2007). Test review of the basic achievement skills inven-
The author proposes that the comprehensive form provides
tory. In K. F. Geisinger, R. A. Spies, J. F. Carlson, & B. S. Plake (Eds.),
a complete evaluation of academic skills to (1) determine The seventeenth mental measurements yearbook [Electronic Version].
academic strengths and weaknesses; (2) screen for and assist Retrieved February 17, 2009, from the Buros Institutes Test Reviews
in diagnosing learning disabilities; (3) place for college Online website: http://unl.edu/buros
354 B Basilar Artery
Definition Definition
The basilar artery provides blood to the brain. This artery A battery approach to neuropsychological assessment is
and the two vertebral arteries comprise the vertebrobasilar the administration of multiple measures that cover a wide
system, which supplies blood to the posterior part of circle range of cognitive abilities to fully characterize an indivi-
of Willis and connects (anastomoses) with blood sup- duals neuropsychological strengths and weaknesses.
plied to the anterior part of the circle of Willis from the
carotid arteries. It arises from the confluence of the two
vertebral arteries, next to the lower brain stem, ascends Current Knowledge
parallel to the brain stem, and gives rise to the anterior
inferior cerebellar artery, which supplies part of the cere- The battery approach is predicated on the existence of a
bellum, some smaller branches that supply the brain stem, variety of instruments that have been empirically devel-
and the superior cerebellar artery. It finally divides into oped to measure myriad aspects of neuropsychological
the two posterior cerebral arteries (PCA). These supply function. Neuropsychological batteries generally contain
the upper brain stem, the occipital lobe, and the posterior a measure of general intellectual functioning or premor-
portion of the temporal lobes. bid functioning as well as assessments of basic neuropsy-
chological functions that may include attention, executive
Current Knowledge function, language, memory, visuospatial perception and
construction, and psychomotor function. Performance on
The clinical manifestations of basilar artery occlusion a test of general intellectual function serves as a context in
depend on the location of the occlusion, the extent of which performance across neuropsychological domains
thrombus, and the collateral flow. Normally, the blood can be considered. Selection of individual tests that com-
flows in an anterograde fashion from the vertebral arteries prise a neuropsychological battery is very likely to depend
to the basilar artery up to its terminal branches. This on the assessment setting, nature of the presenting prob-
pattern of flow may vary. If the proximal segment of the lem and differential diagnosis, and the theoretical orien-
basilar artery is occluded and the occlusion resulted from a tation of the clinician.
slowly progressive stenosis, collateralization occurs within One of the first battery approaches was what is com-
the cerebellum into the circumferential branches of the monly referred to today as the fixed battery. In the fixed
basilar artery. In addition, flow can be reversed from the battery approach, test selection is predetermined irrespec-
PCAs into the distal basilar artery. Thrombosis of the tive of the patients presenting problem. A comprehensive
basilar artery causes various clinical syndromes that result battery of tests is administered to all patients in the same
from brainstem ischemia, including cranial nerve dysfunc- standardized manner. Collection of collateral medical and
tion, difficulty in swallowing and breathing, and at its most social history is obtained following administration and
severe, locked-in syndrome. Basilar artery thrombosis is the scoring of the neuropsychological data to avoid response
most common cause of locked-in syndrome. Mortality rate bias on the part of the test administrator. Examples of this
of basilar artery occlusion is 70%, but this can be reduced psychometrically oriented, data-driven approach include
substantially through the use of antithrombotic agents. the LuriaNebraska Battery and the HalsteadReitan Bat-
tery. An advantage of the fixed battery approach is that it
Cross References facilitates comparison of test scores across patient groups
and assessment settings. Another advantage of the battery
Circle of Willis approach, when using standardized tests, is that this
Posterior Cerebral Artery approach facilitates the use of technicians in the adminis-
Vertebrobasilar System tration of the tests. This approach can also facilitate the
Battle Sign B 355
development of data banks for research purposes. A dis- flexible battery approach increased from 54% in 1989 to
advantage, however, is that it is often time-consuming, 70% in 1999. These data reflect the relative popularity of
cost-prohibitive, and may produce excessive testing ses- this approach, and suggest that it is likely to remain in B
sions that are poorly tolerated by patients (Mitrushina, favor in the coming years.
Boone, Razani, & DElia, 2005).
An alternative neuropsychological assessment method
is the flexible battery approach. In this hypothesis-driven Cross References
approach, initial test selection is guided by the patient
referral question, presenting problem, and the clinical Boston Process Approach
interview. A modest range of measures that survey a broad Fixed Battery
range of cognitive functions is specifically chosen to probe Flexible Battery
and characterize the patients presumed strengths and
weaknesses. Following this initial assessment, which is
sometimes referred to as a core or screening battery, References and Readings
the clinician will then select additional tests based on the
patients performance on the core battery and reported Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychologi-
cognitive concerns (Strauss, Sherman, & Spreen, 2006). cal assessment. Oxford: Oxford University Press.
Mitrushina, M., Boone, K. B., Razani, J., & DElia, L. F. (2005). Handbook
The flexible battery approach is more focused on each
of normative data for neuropsychological assessment. Oxford: Oxford
individual patients presenting problem and differential University Press.
diagnosis than the fixed battery approach. As a result, the Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of
total assessment period is restricted and may be more neuropsychological tests: Administration, norms, and commentary.
cost-effective. However, inherent in the flexible battery Oxford: Oxford University Press.
Sweet, J. J., Moberg, P. J., & Sucy, Y. (2000). Ten-year follow-up survey of
approach is the inconsistent administration of tests across
clinical neuropsychologists: Part 1. Practices and beliefs. The Clinical
patient groups. That is, not all of a neuropsychologists Neuropsychologist, 14, 1837.
patients will receive the same tests, thereby limiting com-
parisons of findings across patient groups or settings
(Mitrushina et al., 2005).
A variant of the flexible battery approach is the pro-
cess approach, also known as the Boston process approach Battle Fatigue
(Lezak, Howieson, & Loring, 2004). This method entails
emphasis on the more qualitative aspects of neuropsycho- Posttraumatic Stress Disorder
logical performance. When completing a task, patients are
closely observed for strategy formation and execution.
Atypical performances will be further probed by the clini-
cian with direct questioning or modified re-administra- Battle Sign
tion of the task to more fully examine the nature of
the behavioral dysfunction. This approach affords a B ETH R USH
more in-depth characterization of the patients neuropsy- Mayo Clinic
chological abilities. However, it has been criticized for its Jacksonville, FL, USA
lack of normative data and standards for the reliability
and validity of its methods (Strauss et al., 2006).
Synonyms
clinical symptom indicates the presence of a skull fracture. opinion that neuropsychologists were not competent to
A patient with this symptom may present with bloody offer expert testimony on brain malfunctions from motor
discharge of the ear. The bruise results from the force of vehicle accidents. The appellate court held that to exclude
impact, which forces the flow of blood out of the vascular such testimony on physical matters by psychologists
endothelium of a vessel into adjacent skin tissue. The would be to ignore present medical and psychological
battle sign may occur a few days following the onset of practice. Most states allow neuropsychological testimony
the skull fracture. about brain damage (Richardson & Adams, 1992) while
there is a greater diversity of opinion as to testimony
about causation.
Cross References
Current Knowledge
Depressed Skull Fracture
In Baxter v. Temple (2005), defense filed a motion in
limine to exclude the testimony of a neuropsychologist
References and Readings in a case of lead exposure as insufficiently unreliable
because opinions were based on results from a flexible
Victor, M., & Ropper, A. H. (2001). Principles of neurology (7th ed., neuropsychological test battery. The defense argued suc-
pp. 925953). New York: McGraw-Hill. cessfully that the neuropsychologists testimony should be
excluded because the Boston Approach had not been
subject to peer review and publication, has no known or
potential error rate, and is not generally accepted in the
Baxter v. Temple (2005) appropriate scientific literature. In other words, Daubert
factors were used by the trial judge to exclude expert
R OBERT L. H EILBRONNER neuropsychological evidence. Furthermore, the court
Chicago Neuropsychology Group made an important distinction between the roles of a
Chicago, IL, USA clinical provider and forensic examiner, emphasizing
that neuropsychologists in forensic practice must employ
objective methods that allow them to be unbiased truth
Synonyms seekers. The defendant motion in limine was granted.
Some (Reed, 1996) have argued that Daubert challenges
Admissibility of psychological/neuropsychological evidence of idiosyncratic (flexible) test combinations will eliminate
the use of flexible neuropsychological batteries in forensic
consulting. However, recent surveys of neuropsycholo-
gists show that the majority of neuropsychologist practi-
Historical Background tioners use a carefully constructed battery approach
specifically tailored to the patient/examinees specific
One of the first decisions to address the admissibility of
issues. In 2008, the New Hampshire Supreme Court
expert testimony by a psychologist or neuropsychologist
reviewed the neuropsychological literature, practices of
as to the existence of a brain injury or mental defect was
neuropsychologists, considered relevant Daubert stan-
Jenkins v. United States (1962). This was a criminal trial in
dards and various amicus briefs, and concluded that the
which the jury was instructed to disregard the testimony
exclusion of the neuropsychological testimony in Baxter v.
of the psychologists on the grounds that they could not
Temple (2005) was in error.
give a medical opinion as to mental disease or defect
because they did not have medical training. The appellate
court reversed the decision holding that the expert did not Cross References
need to be a medical practitioner. A later opinion, in
United States v. Riggleman (1969) supported the position Admissibility
that psychologists were not excluded from testifying Daubert v. Merrell Dow
about criminal sanity solely because they lacked medical Expert v. Treater Role
training. Simmons v. Mullins (1975) was an early appellate Federal Rules of Evidence
court decision that essentially reversed a trial court Kumho Tire v. Carmichael
Bayley Scales of Infant and Toddler Development B 357
References and Readings five domains: Cognitive (91 items), Language (49 recep-
tive and 48 expressive), Motor (66 fine motor and 72 gross
Chapple v. Ganger, 851 F. Suppl. 1481 (E. D. Wash, 1994). motor), Social-Emotional, and Adaptive. Like its prede- B
Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In cessors, the BSID-III is a modified power test. Assessment
J. Morgan & J. Ricker (Eds.), Textbook of clinical neuropsychology.
of the first three domains is accomplished by item admin-
New York: Taylor & Francis.
Greiffenstein, M. F., & Cohen, L. (2005). Neuropsychology and the law:
istration, while the latter two are completed using care-
Principles of productive attorneyneuropsychologists relations. giver response to a questionnaire. A Behavior Observation
In G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. Inventory is completed by both the examiner and the care-
New York: Oxford University Press. giver, and allows assessment of the childs behavior during
Jenkins v. United States (1962). 307 F. 2d 637.
testing and at home. The Language scale includes Receptive
Kaufmann, P. M. (2008). Admissibility of neuropsychological evidence in
criminal cases: Competency, insanity, culpability, and mitigation.
Communication and Expressive Communication subtests;
In R. Denney & J. Sullivan (Eds.), Clinical neuropsychology in the the Motor scale includes a Fine Motor and a Gross Motor
criminal forensic setting. New York: Guilford Press. subtest. The BSID-III Social-Emotional scale is an adapta-
Richardson, R. E. L., & Adams, R. L. (1992). Neuropsychologists as expert tion of the Greenspan Social-Emotional Growth Chart: A
witnesses: Issues of admissibility. The Clinical Neuropsychologist, 6,
Screening Questionnaire for Infants and Young Children
295308.
Simmons v. Mullins (Pa. Super Ct. 1975). 331 A2D 892, 897.
(Greenspan, 2004). The Adaptive Behavior scale is com-
United States v. Riggleman (4th Cir. 1969). 411 F.2d 1190. posed of items from the Parent/Primary Caregiver Form
of the Adaptive Behavior Assessment System-Second Edi-
tion (Harrison & Oakland, 2003). This scale measures
areas such as communication, community use, health
and safety, leisure, self-care, self-direction, functional
Bayley pre-academics, home living, social and motor, and yields
a General Adaptive Composite (GAC). Discrepancies
Bayley Scales of Infant and Toddler Development
between scaled scores can be reviewed to determine
whether the differences between subtests are statistically
significant.
G LEN P. AYLWARD The original BSID (Bayley, 1969) evolved from versions of
SIU School of Medicine-Pediatrics developmental tests that were administered to infants
Springfield, IL, USA enrolled in the landmark National Collaborative Perinatal
Project. It was considered the reference standard for the
assessment of infant development and administered to
Synonyms infants over the first 2 years. The BSID was theoretically
eclectic and borrowed from different research and test
Bayley; BSID-III instruments. The test contained three components: the
Mental Developmental Index (MDI), the Psychomotor
Developmental Index (PDI) (M = 100, SD = 16) and the
Description Infant Behavior Record, and was applicable from 2 to
30 months.
The Bayley Scales of Infant and Toddler Development- The BSID subsequently was revised into the BSID-II
Third Edition (BSID-III; 2006) is often considered to be (Bayley, 1993), this due in part to the upward drift
the reference standard for developmental assessment. It is of approximately 11 points on the MDI and 10 points
an individually administered test, applicable from 1 to on the PDI, reflecting the Flynn effect. Although the
42 months of age. The stated primary purpose of the mean remained the same, the SD was now 15. When
BSID-III is to identify children with developmental delay compared to the original BSID, the BSID-II scores
and to provide information for interventions. were 12 points lower on the MDI and 10 points lower
The BSID-III was normed on 1,700 children (divided on the PDI. The Behavior Rating Scale was devel-
into 17 age groups) and development was assessed across oped to enable the assessment of state, reactions to
358 B BBA
the environment, motivation, and interaction with peo- levels of development. Repeated administration can docu-
ple. The age range of the BSID-II was expanded to span ment the effects of an intervention program. However,
142 months. The instrument contained 22 item sets changes in test content and alteration of scales in conjunc-
and basal and ceiling rules that differed from the origi- tion with the Flynn effect and the more recent increase in
nal BSID. These rules were controversial because if mean scores (in comparison to the previous version) make
correction for prematurity was used to determine the longitudinal comparisons of scores difficult in individual
item set to begin administration, or if an earlier item children or cohorts. Extracting language items from the
set was employed because of developmental problems, cognitive scale also affects comparability with the MDI
scores tended to be somewhat lower, because the child found in previous versions. Conversely, the five domains
was not automatically given credit for passing the lower now allow the BSID-III to be more compatible with early
item set. The BSID-II was also criticized because it intervention requirements (IDEA; PL 108446, Part C). A
did not provide area scores compatible with IDEA criticism of the test is that it can take an exceptionally long
requirements for cognitive, motor, communication, so- time to administer and this can be very problematic when
cial, and adaptive function. testing young children. The BSID-III can be used in mul-
tidisciplinary clinics, NICU follow-up programs, or as a
follow-up evaluation after a child has been identified by
Psychometric Data the use of a screening test.
Clinical Uses
BCRS
Administration of the BSID-III provides quantitative and
qualitative data that give insight into the childs current Brief Cognitive Rating Scale
Beck Anxiety Inventory B 359
BDI-II
Historical Background
Beck Depression Inventory
The measure was developed to assess cognitive and psy-
chological symptoms of anxiety, with the goal of reliably
discriminating between anxiety- and depression-related
Bearing symptoms. An initial item pool (86 items) was compiled
from three existing scales (the Anxiety Checklist [Beck,
Orientation Steer, & Brown, 1985], Physicians Desk Reference Check-
list [Beck, 1978], and Situational Anxiety Checklist [Beck,
1982]). These measures contained items regarding the
wide range of symptoms reported by those with anxiety
disorders (Beck et al., 1988). A series of analyses were
Beck Anxiety Inventory conducted to reduce the item pool to 21 (Beck et al.,
1988). Initially, redundant items were eliminated. Succes-
L ISA A. B RENNER sive iterated principal factor analyses were used to achieve
VISN 19 MIRECC further item reduction (Beck et al., 1988). Remaining
Denver, CO, USA items were evaluated (validity and reliability analyses)
and results yielded a 21-item scale (Beck et al., 1988).
Synonyms
Psychometric Data
BAI
Work by Beck et al. (1988) suggests high internal consis-
tency (0.92) and test-retest reliability over 1 week, r(81) =
Description 0.75. The Inventory was shown to discriminate between
those with and without anxiety-related disorders. The BAI
The Beck Anxiety Inventory (BAI) (Beck, Epstein, Brown, & was also moderately correlated with the revised Hamilton
Steer, 1988; Beck & Steer, 1993) is a 21-item scale developed Anxiety Rating Scale, r(150) = 0.51. In addition, the
to assess the severity of anxiety symptoms. Respondents are validity of the BAI has been supported by findings
360 B Beck Anxiety Inventory
which suggest that the BAI and the Beck Depression note that scores should be interpreted based on normative
Inventory (BDI) (Beck, Rush, Shaw, & Emery, 1979) mea- data taking into account gender, age, and living situation.
sure different symptoms (Hewitt & Norton, 1993). In addition to its use with general clinical populations,
A number of factor-analytic studies have been the BAI has demonstrated utility in neuropsychological
conducted regarding the BAI and results have provided populations. The BAI has been used in clinical trials of
two- (cognitive and somatic components) (Hewitt and psychotropic interventions for depression following trau-
Norton, 1993), four- (subjective, neurophysiological, matic brain injury (TBI) (Ashman et al., 2009), and as a
autonomic, and panic) (Beck and Steer, 1991), five- (sub- measure of anxiety following TBI (Cantor et al., 2005).
jective fear, somatic nervousness, neurophysiological, Trahan, Ross, and Trahan (2001) used the BAI to study
muscular/motoric, and respiration) (Borden, Peterson, the relationship between self-reported post-concussive
& Jackson, 1991), six- (somatic distress, fear, autonomic and psychiatric symptoms in neurologically normal
hyperactivity, panic, nervousness, and motor tension) young adults and those recovering from mild TBI. They
(Morin, Landreville, Colecchi, McDonald, Stone, & found correlations between a composite of measure of
Ling, 1999) factor solutions. Hewitt and Norton (1993) post-concussive symptoms and the BAI. The BAI has
suggest that this range in number of identified factors also been used to measure anxiety following stroke
may, in part, be related to the inconsistency of specific (Moon, Kim, Kim, Won, & Kim 2004), though some
items composing the factors (somatic and cognitive) and research suggests that caution is warranted in its use
differences in factor-analytic approaches used. In psychi- with this population due to the potential overlap between
atric patients, Hewitt and Norton (1993) provided evi- somatosensory anxiety symptoms and neurological con-
dence to support that their two-factor structure was sequences of stroke (Schramke, Stowe, Ratcliff, Goldstein,
similar for men and women, despite the fact that & Condray, 1998).
women seemed to report more anxiety than men. Explor-
ing the psychometric properties of the BAI with older
adults, Morin et al.s six-factor solution included multi- Cross References
ple subjective, motoric, and physiological dimensions of
anxiety. A trend for older subjects to obtain slightly Anxiety
higher scores was also noted. Given the lack of consensus Beck Depression Inventory
regarding underlying factors for the BAI, use of the total
score, as recommended in its initial development (Beck &
Steer, 1993), remains the predominant approach for mea- References and Readings
suring anxiety with this instrument.
Ashman, T. A., Cantor, J. B., Gordon, W. A., Spielman, L., Flanagan, S.,
Ginsberg, A., et al. (2009). A randomized controlled trial of sertra-
line for the treatment of depression in persons with traumatic brain
Clinical Uses injury. Archives of Physical Medicine & Rehabilitation, 90(5),
733740.
The BAI appears to adequately measure anxiety-related Beck, A. T. (1978). PDR checklist. Philadelphia: University of Pennsylva-
symptoms, but may be more sensitive to physiological nia, Center for Cognitive Therapy.
Beck, A. T. (1982). Situational anxiety checklist (SAC). Philadelphia:
aspects of anxiety disorders (Ferguson, 2000). This is likely
University of Pennsylvania, Center for Cognitive Therapy.
in part related to the fact that items were selected to Beck, A. T., Epstein, N., Brown, G., & Steer, R. A. (1988). An inventory for
adequately discriminate between those with anxiety ver- measuring clinical anxiety: Psychometric properties. Journal of Con-
sus depression. As such, Ferguson (2000) notes that the sulting and Clinical Psychology, 56, 893897.
BAI may not be sensitive to detecting anxiety disorders Beck, A. T., Rush, A. J., Shaw, B. F., & Emery, G. (1979). Cognitive therapy
of depression: A treatment manual. New York: Guilford Press.
where psychophysiological arousal was not a prominent
Beck, A. T., & Steer, R. A. (1991). Relationship between the Beck Anxiety
feature, (p. 520). He also notes that the instrument has a Inventory and the Hamilton Anxiety Rating Scale with anxious
high level of face validity and, as such, respondent moti- outpatients. Journal of Anxiety Disorders, 5, 213223.
vation may impact reporting. In general clinical settings, Beck, A. T., & Steer, R. A. (1993). Beck Anxiety Inventory manual. San
the Inventory may be best used in conjunction with addi- Antonio, TX: Psychological Corporation.
Beck, A. T., Steer, R. A., & Brown, G. (1985). Beck anxiety checklist.
tional findings to diagnose anxiety-related disorders and
Unpublished manuscript, University of Pennsylvania.
assess treatment needs and responses. Borden, J. W., Peterson, D. R., & Jackson, E. A. (1991). The Beck Anxiety
Although it has been suggested the BAI is a valuable Inventory in nonclinical samples: Initial psychometric properties.
screen tool for older adults, Morin and colleagues (1999) Journal of Psychopathology and Behavioral Assessment, 13, 345356.
Beck Depression Inventory B 361
Cantor, J. B., Ashman, T. A., Schwartz, M. E., Gordon, W. A., Hibbard, Steer, 1987). A more substantial revision led to the devel-
M. R., Brown, M., et al. (2005). The role of self-discrepancy theory in
opment of the Beck Depression Inventory-II in 1996
understanding post-traumatic brain injury affective disorders: A
pilot study. Journal of Head Trauma Rehabilitation, 20(6), 527543.
(BDI-II; Beck, Steer, & Brown, 1996). B
Ferguson, R. (2000). Using the Beck Anxiety Inventory in primary care. The BDI-II is comprised of 21 individual items reflecting
In M. E. Maruish (Ed.), Handbook of psychological assessment in specific cognitive, affective, and physical symptoms of de-
primary care settings (pp. 509536). Mahwah, NJ: Lawrence Erlbaum pression. Each item includes four statements that vary in the
Associates, Inc.
description of symptom severity. Scores range from 0 to 3,
Hewitt, P. L., & Norton, G. R. (1993). The Beck Anxiety Inventory:
A psychometric analysis. Psychological Assessment, 5, 408412.
with a score of 3 indicating severe symptoms and a score of
Morin, C. M., Landreville, P., Colecchi, C., McDonald, K., Stone, J., & 0 indicating an absence of concern with that particular
Ling, W. (1999). The Beck Anxiety Inventory: Psychometric proper- aspect of depressive symptomatology (Lezak, Howieson,
ties with older adults. Journal of Clinical Geropsychology, 5, 1929. Loring, Hannay, & Fischer, 2004). The total score is the
Moon, Y. S., Kim, S. J., Kim, H. C., Won, M. H., & Kim, D. H. (2004).
sum of all endorsed statements. If more than one statement
Correlates of quality of life after stroke. Journal of the Neurological
Sciences, 224(12), 3741.
from a given item is chosen by the patient, only the statement
Osman, A., Hoffman, J., Barrios, F. X., Kopper, B. A., Breitenstein, J. L., & of greatest severity is scored. The maximum total score is 63.
Hahn, S. K. (2002). Factor structure, reliability, and validity of the As per the BDI-II Manual, the instrument requires
Beck Anxiety Inventory in adolescent psychiatric inpatients. Journal approximately 510 min to complete. This, of course,
of Clinical Psychology, 58(4), 443456.
may vary depending on the patient. The BDI-II is typically
Schramke, C. J., Stowe, R. M., Ratcliff, G., Goldstein, G., & Condray R.
(1998). Poststroke depression and anxiety: Different assessment
self-administered, but the examiner may read the items
methods result in variations in incidence and severity estimates. aloud to the patient if difficulties with reading or vision
Journal of Clinical & Experimental Neuropsychology, 20(5), 723737. are evident. The administration instructions differ
Trahan, D. E., Ross, C. E., & Trahan, S. L. (2001). Relationship among depending on the mode of administration (self or exam-
postconcussional-type symptoms, depression, and anxiety in neuro-
iner). The BDI-II instructs individuals to respond to the
logically normal young adults and victims of mild brain injury.
Archives of Clinical Neuropsychology, 16, 435445.
items based on how they felt during the past 2 weeks,
including today. The time frame was increased from 1
week in the original instrument to 2 weeks in the BDI-II
to remain consistent with the American Psychiatric Asso-
ciations Diagnostic and Statistical Manual on Mental
Beck Depression Inventory Disorders-Fourth Edition (American Psychiatric Associa-
tion, 1994) diagnostic criteria for clinical depression. The
D ENNIS J. Z GALJARDIC BDI-II is applicable to patients aged 1380 years.
Transitional Learning Center at Galveston
Galveston, TX, USA
Historical Background
Synonyms The original BDI was developed with the use of descriptors
provided by psychiatric patients (predominantly with de-
BDI; BDI-II pression). These descriptors were then consolidated and
adopted into 21 items, several of which differ from those
in the BDI-II. In an attempt to remove alternative wordings
and double negatives from the original BDI instrument,
Description Beck and colleagues (1979) developed the BDI-IA. Fifteen
of the 21 original items were modified. Moran and Lam-
The Beck Depression Inventory (BDI; Beck, Ward, Men- bert (1983) discovered that the BDI met only six of the
delson, Mock, & Erbaugh, 1961) is one of the most widely nine criteria for depression listed in the American Psychi-
used self-report inventories to assess depressive symptom atric Associations Diagnostic and Statistical Manual on
severity in adolescent and adult populations. It has been Mental Disorders-Third Edition (American Psychiatric
employed as a screening instrument in over 2,000 studies Association, 1980). Further, they reported that the BDI
(Richter, Werner, Heerlein, Kraus, & Sauer, 1998). An lacked items addressing psychomotor activity and agita-
amended version of the original 1961 instrument (BDI- tion and permitted only the endorsement of decreases
IA; Beck, Rush, Shaw, & Emery, 1979) was developed in (not increases) in appetite and sleep (which was inconsis-
1979, with publication of its manual in 1987 (Beck & tent with DSM-III criteria for clinical depression).
362 B Beck Depression Inventory
Further changes to the diagnostic criteria for clinical replicated Beck and colleagues (1996) two-factor solution
depression following the publications of the DSM-III-R in a sample of 414 undergraduates. However, in their
(1987) and-DSM-IV (1994) led to further revision of the model, the Pessimism and Loss of Interest in Sex
instrument. Changes implemented for what became the items significantly loaded onto the Cognitive-Affective
BDI-II included rewording of select statements, as well as factor. The internal consistency for both cognitive and
(1) introduction of the items of Agitation, Worthless- somatic factors were reported to be good (a = 0.87 and
ness, Concentration Difficulty, and Loss of Energy; (2) 0.74, respectively).
modifications to the Insomnia and Loss of Appetite
items to reflect both increases and decreases in sleep and
appetite; and (3) the removal of the Body Image Change, Clinical Uses
Work Difficulty, Weight Loss, and Somatic Preoccupa-
tion items. The BDI-II Manual designates the following raw score
classifications of depression severity: 13 = minimal;
1419 = mild; 2028 = moderate; 29 = severe. The
Psychometric Data instruments developers suggested that different cut-off
scores may be required depending on the characteristics
Standardization data for the BDI-II was obtained from 500 of the sample and the purpose for using the instrument.
psychiatric outpatients and 120 undergraduates. Internal Hence, on an individual basis, clinical judgment must be
consistency was high for each sample (a = 0.92 and 0.93, used, as the cut-off score ranges should be viewed as
respectively). These coefficient alphas are consistent with guidelines (Lezak et al., 2004). As per the BDI-II Manual,
those reported in independent study samples including: clinicians may give particular consideration to items
140 psychiatric outpatients (mean age = 37.6 years) 2 (Pessimism) and 9 (Suicidal Thoughts or Wishes) to
(a = 0.91; Beck, Steer, Ball, & Ranieri, 1996), 408 psychi- gauge suicide risk in a given psychiatric patient.
atric adolescents (age range = 1317 years) (a = 0.93; Given the burgeoning ethnic and cultural diversity of
Osman, Kopper, Barrios, Gutierrez, & Bagge, 2004), 414 psychiatric and neurological patient populations, there is an
nonclinical undergraduates (age range = 1739 years) ever-increasing need for linguistically and culturally sensi-
(a = 0.90; Storch, Roberti, & Roth, 2004), 414 nonclinical tive psychiatric inventories. Wiebe and Penley (2005) re-
high-school-aged students (age range = 1418 years) port on the psychometric properties of a Spanish
(a = 0.92; Osman, Barrios, Gutierrez, Williams, & Bailey, translation of the BDI-II using a sample of 895 undergrad-
2008), and 147 nonclinical community-dwelling older uates. They administered the English and/or Spanish ver-
adults (age range = 5990 years) (a = 0.86; Segal, Cool- sion of the BDI-II twice to each participant, with a 1-week
idge, Cahill, & ORiley, 2008). Test-retest reliability of the interval between administrations. The two versions of the
BDI-II was assessed in a sample of 26 psychiatric out- instrument were administered to English-only speaking or
patients (Beck et al., 1996), yielding a statistically signifi- bilingual participants. In their design, the monolingual
cant correlation coefficient (r = 0.93). participants (n = 539) received the English version of the
Beck et al. (1996) demonstrated the convergent and BDI-II at each time-point. One subset of the bilingual
discriminant validity of the BDI-II in 87 psychiatric out- participants (n = 355) were administered the Spanish ver-
patients by correlating BDI-II scores with scores from the sion of the BDI-II at each time-point, whereas the remain-
Hamilton Psychiatric Rating Scale for Depression (Hamil- ing bilingual subset (n = 254) were randomized in the
ton, 1960) and the Hamilton Rating Scale for Anxiety administration of the Spanish and English versions of the
(Hamilton, 1959). Here, the BDI-II was more positively BDI-II at each time-point. Internal consistencies for the
correlated with the Hamilton depression scale (r = 0.71) English only and Spanish only administration were good
than the Hamilton anxiety scale (r = 0.47). While asses- (a = 0.89 and 0.91, respectively). Test-retest reliabilities
sing the factor validity of the BDI-II, Beck and colleagues were statistically significant for each version across condi-
(1996) reported a two-factor solution using their outpa- tion (English only [r = 0.73], Spanish only [r = 0.86], and
tient standardization sample that yielded a Somatic- randomized administration [r = 0.76]). Confirmatory
Affective factor (12 items) and a Cognitive-Affective factor analyses revealed that their data from both English
factor (9 items). Items that did not significantly load onto and Spanish versions of the BDI-II demonstrated good
either factor included Pessimism and Loss of Interest fit with the factor solution reported in the BDI-II Manual.
in Sex. Similar findings were replicated using their stan- Cognitive complexity of a given instrument of depres-
dardization sample of undergraduates. Storch et al. (2004) sive symptom severity is important to consider, as such
Beck Depression Inventory B 363
measures may be administered to patient populations with (2009) stressed that, while the BDI-II can provide adequate
known cognitive impairment (e.g., Parkinsons disease means in assessing the severity of depression in patients
[Zgaljardic, Borod, Foldi, & Mattis, 2003] and Traumatic with TBI, the separate dimensions of depression need to B
Brain Injury [Draper & Ponsford, 2008]). Shumway, Sen- be addressed by considering all three score subtypes in
tell, Unick, and Bamberg (2004) demonstrated that three this patient population in order to avoid misattributing
versions of the BDI including the original instrument, the somatic symptoms of TBI to symptoms of depression.
BDI-II, and the BDI-Primary Care (BDI-PC; Beck, Guth, The BDI-II was developed to correspond with depres-
Steer, & Ball, 1997) had high overall complexity based on sive disorder criteria set forth by the DSM-IV. The reli-
the characteristics of (1) number of instructions, ques- ability and validity of the instrument have been
tions, and response options; (2) item length; (3) readabili- established across several studies including psychiatric
ty (grade level); and (4) linguistic problems (likely to result and neurological patients as well as nonclinical commu-
in memory overload). Nevertheless, good reliability and nity-dwelling individuals. This appears to extend to eth-
validity of the BDI-II have been demonstrated in patients nic and culturally diverse populations as well. The BDI-II
with significant cognitive impairment (Powell, 2003). is not intended to be used for the sole purpose of speci-
There has been concern that the somatic complaint fying a clinical diagnosis, but rather as an indicator of the
items included in the BDI-II may be confounded in select existence and severity of depressive symptoms (Beck et al.,
patients, such as the elderly or those with movement 1996). Further, that depression may occur concurrently
disorders (e.g., Parkinsons disease [PD]) (Lezak et al., with other psychiatric diagnoses (e.g., apathy) across pa-
2004). In these patient populations, physical sequelae tient populations also mandates caution when interpreting
might mimic somatic symptoms of depression, leading findings from the BDI-II.
to greater endorsement of somatic items on the BDI-II
and resulting in false classifications of depressive symp-
tomatology. Using the original BDI instrument, Levin,
Llabre, and Weiner (1988) reported that among patients
Cross References
with PD, somatic complaints were associated with the
Center for Epidemiologic Studies-Depression
depression and not the PD symptomatology (e.g., brady-
Geriatric Depression Scale
kinesia), suggesting that the BDI is a valid measure of
Hamilton Rating Scale for Depression
depression in this particular patient population. Internal
Self-Report Measures
consistency for the total score of the BDI was acceptable
Zung Self-Rating Depression Scale
(a = 0.88). When the scores for the somatic items were
removed from the total score, the internal consistency
remained relatively unchanged (a = 0.89). Green, Fel-
mingham, Baguley, Slewa-Youan, and Simpson (2001) References and Readings
administered the BDI to 117 patients with Traumatic
Brain Injury (TBI) 2 years after discharge from a residen- American Psychiatric Association (1980). Diagnostic and statistical man-
tial brain injury rehabilitation program. Internal consis- ual of mental disorders. Washington, DC: American Psychiatric
tency was high (a = 0.92). A principal component factor Association.
American Psychiatric Association (1987). Diagnostic and statistical man-
analysis using the BDI total score yielded a three-factor
ual of mental disorders (3rd ed., Rev.). Washington, DC: American
solution. The affective factor accounted for the largest Psychiatric Association.
percentage of variability, followed by negative attitudes American Psychiatric Association (1994). Diagnostic and statistical man-
toward the self and somatic disturbance factors. Their ual of mental disorders (4th ed.). Washington, DC: American Psychi-
findings were relatively consistent with the findings from atric Association.
Beck, A. T., Guth, D., Steer, R. A., & Ball, R. (1997). Screening for major
studies using non-TBI samples and suggest that BDI
depression disorders in medical inpatients with the Beck Depression
scores, at least for moderate levels of depression, did not Inventory for primary care. Behavior Research and Therapy, 35,
appear to be influenced by high incidences of somatic 785791.
complaints. Siegert, Walkey, and Turner-Stokes (2009) Beck, A. T., Rush, A. J., Shaw, B. F., & Emery, G. (1979). Cognitive therapy
reported on the existence of three-factor solution, yielding of depression. New York: Guilford Press.
Beck, A. T., & Steer, R. A. (1987). Manual for the Beck Depression
a depression factor (all 21 BDI-II items) as well as a
Inventory. San Antonio, TX: The Psychological Corporation.
somatic (11 items) and a cognitive/affective (8 items) Beck, A. T., Steer, R. A., Ball, R., & Ranieri, W. (1996). Comparison of
factor in a sample of patients with TBI. Internal consistency Beck Depression Inventories -IA and -II in psychiatric outpatients.
for item scores from each factor was strong. Siegert et al. Journal of Personality Assessment, 67(3), 588597.
364 B Beery Developmental Test of Visual-Motor Integration (VMI)
Beck, A. T., Steer, R. A., & Brown, G. K. (1996). Beck Depression Inventory
Manual (2nd ed.). San Antonio, TX: The Psychological Corporation. Beery Developmental Test of
Beck, A., Ward, C. H., Mendelson, l., Mock, J., & Erbaugh, J. (1961). An
inventory for measuring depression. Archives of General Psychiatry, Visual-Motor Integration (VMI)
4, 561571.
Draper, K., & Ponsford, J. (2008). Cognitive functioning ten years follow- K ELLY C ROTTY, I DA S UE B ARON
ing traumatic brain injury and rehabilitation. Neuropsychology, 22 Inova Fairfax Hospital for Children
(5), 618625.
Falls Church, VA, USA
Green, A., Felmingham, K., Baguley, I. J., Slewa-Younan, S., & Simpson, S.
(2001). The clinical utility of the Beck Depression Inventory after
traumatic brain injury. Brain Injury, 15(12), 10211028.
Hamilton, M. (1959). The assessment of anxiety states by rating. British Synonyms
Journal of Medical Psychology, 32, 5055.
Hamilton, M. (1960). A rating scale for depression. Journal of Neurology
Beery VMI; Developmental test of visual motor
and Neurosurgical Psychiatry, 23, 5662.
Levin, B. E., Llabre, M. M., & Weiner, W. J. (1988). Parkinsons disease integration
and depression: Psychometric properties of the Beck Depression
Inventory. Journal of Neurology, Neurosurgery & Psychiatry, 51(11),
14011404.
Lezak, M. D., Howieson, D. B., Loring, D. W., with Hannay, H. J., & Description
Fischer, J. S. (2004). Neuropsychological assessment (4th ed.).
New York: Oxford University Press. The BeeryBuktenica Developmental Test of Visual-
Moran, P. W., & Lambert, M. J. (1983). A review of current assessment
Motor Integration (VMI; Beery, Buktenica, & Beery,
tools for monitoring changes in depression. In J. S. Lambert, E. R.
Christensen, & S. DeJulio (Eds.), The assessment of psychotherapy
2004) is designed to identify deficits in visual-motor inte-
outcome (pp. 263303). New York: Wiley. gration, visual perception, and motor coordination. This
Osman, A., Barrios, F. X., Gutierrez, P. M., Williams, J. E., & Bailey, J. brief paper and pencil test requires the examinee to copy
(2008). Psychometric properties of the Beck Depression Inventory-II geometric designs of increasing complexity arranged in
in nonclinical adolescent samples. Journal of Clinical Psychology, 64
a developmental sequence. The 5th edition (2004) pro-
(1), 83102.
Osman, A., Kopper, B. A., Barrios, F., Gutierrez, P. M., & Bagge, C. L.
vides standardized normative data for individuals aged
(2004). Reliability and validity of the Beck Depression Inventory-II 218 years, and more recent norms have been established
with adolescent psychiatric inpatients. Psychological Assessment, 16 using 1,021 adults aged 19100 years. The VMI has a
(2), 120132. Full Form and a Short Form, both of which can be
Powell, R. (2003). Psychometric properties of the Beck Depression Inven-
administered to a group or individuals in approximately
tory and the Zung Self Rating Depression Scale in adults with mental
retardation. Mental Retardation, 41(2), 8895.
1015 min. The Short Form, for children aged 27 years,
Richter, P., Werner, J., Heerlein, A., Kraus, A., & Sauer, H. (1998). On the has 21 items and the Full Form extends to 30 items. Each
validity of the Beck Depression Inventory. A review. Psychopathology, reproduction of a geometric form is scored as 1 point if
31(3), 160168. correct and 0 if incorrect, with a discontinuation rule of
Segal, D. L., Coolidge, F. L., Cahill, B. S., & ORiley, A. A. (2008). Psycho-
three consecutive failures. Raw scores are converted to age-
metric properties of the Beck Depression Inventory II (BDI-II) among
community-dwelling older adults. Behavior Modification, 32(1), 320.
based standard scores, percentiles, and scales scores. Two
Shumway, M., Sentell, T., Unick, G., & Bamberg, W. (2004). Cognitive optional subtests use the same stimulus forms as the VMI,
complexity of self-administered depression measures. Journal of the visual perception test and the motor coordination test.
Affective Disorders, 83, 191198. These subtests are useful to compare visual-motor integra-
Siegert, R. J., Walkey, F. H., & Turner-Stokes, L. (2009). An examination
tion efficiency to pure visual or pure motor performance.
of the factor structure of the Beck Depression Inventory-II in a
neurorehabilitation inpatient sample. Journal of the International
Neuropsychological Society, 15(1), 142147.
Storch, E. A., Roberti, J. W., & Roth, D. A. (2004). Factor structure,
concurrent validity, and internal consistency of the Beck Depression
Historical Background
Inventory-Second Edition in a sample of college students. Depression
& Anxiety, 19(3), 187189. The Beery VMI, originally known as the Developmental
Wiebe, J. S., & Penley, J. A. (2005). A psychometric comparison of the Form Sequence, began to be developed in 1961. While
Beck Depression Inventory-II in English and Spanish. Psychological
other design-copying measures were available, they did
Assessment, 17(4), 481485.
Zgaljardic, D. J., Borod, J. C., Foldi, N. S., & Mattis, P. (2003). A review of
not comprise a sequence of designs that increased in
the cognitive and behavioral sequelae of Parkinsons disease: Rela- complexity or that reflected normal development. Keith
tionship to frontostriatal circuitry. Cognitive & Behavioral Neurology, Beery believed that visual-motor integration correlated
16(4), 193210. with academic achievement and wanted to construct a
Behavior B 365
measure of such skill development. He began with 72 and clinical interpretation. Even children who perform well
designs administered to 600 children in Illinois. Thirty on the visual-motor integration portion may have deficits
designs were selected and administered to another 600 in the subareas of visual perception and motor coordina- B
children, leading to the selection of the final 24 forms tion (Kulp & Sortor, 2003). In fact, visual perception spe-
administered today. cifically has been associated with math and reading ability
Beery VMI was published in 1967 by Keith Beery, (Sortor & Kulp, 2003). Research has also found a signifi-
Norman Buktenica, and Natasha Beery, with normative cant relation between visual-motor integration and aca-
data obtained in 1964 on 1,030 children. The Beery VMI demic achievement (Sortor & Kulp, 2003); however, these
has since been re-normed on more than 10,000 children. two areas are not exclusively correlated. Beery VMI is more
Updated editions of the Beery VMI were published in strongly correlated with chronological age than academic
1982 (2nd Edition), 1989 (3rd Edition), 1997 (4th Edi- achievement. Poor performance on the Beery VMI may or
tion), and 2004 (5th Edition). Each edition offered may not indicate academic achievement impairments.
updated norms. The most recent 5th Edition includes
standardized norms for children of 2 years of age and
provides visual-motor teaching methods from birth
Cross References
through early elementary school.
Beery Developmental Test of Visual-Motor Integration
(VMI)
Psychometric Data Bender-Gestalt, Second Edition
Rey-Osterrieth Complex Figure Test (ROCF)
The manual reports overall average reliabilities at .92 for
visual-motor integration, .91 for visual perception, and .90
for motor coordination. Beery VMI correlates .52 with the References and Readings
Wide Range Assessment of Visual Motor Abilities
(WRAVMA) drawing subtest and .75 with the Develop- Beery, K. E., Buktenica, N. A., & Beery, N. A. (2004). The Beery-Buktenica
mental Test of Visual Perception (DTVP-2) copying subtest. developmental test of visual-motor integration: Administration, scoring
Strong correlations between the Beery VMI and other visual and teaching manual. Minneapolis, MN: NCS Pearson.
motor assessments highlight the tests validity. Normative Kulp, M. T., & Sortor, J. M. (2003). Clinical value of the Beery visual-
motor integration supplemental test of visual perception and motor
scores are valid for both individual and group administra-
coordination. Optometry and Vision Science, 80(4), 312315.
tion, but it is recommended that those who perform below Malloy, P., Belanger, H., Hall, S., Aloia, M., & Salloway, S. (2003). Asses-
average be individually tested. It is also suggested that sing visuocontructional performance in AD, MCI and normal elder-
preschoolers be screened individually to maintain validity. ly using Beery visual-motor integration test. The Clinical
Neuropsychologist, 17(4), 544550.
Sortor, J. M., & Kulp, M. T. (2003). Are the results of the Beery-Buktenica
developmental test of visual motor integration and its subtests
Clinical Uses related to achievement test scores? Optometry and Vision Science,
80(11), 758763.
Key Features of the BASC-2 available and updated information on these versions
can be obtained by contacting Pearson Assessments
The BASC-2 has numerous features that make it a sophis- (www.pearsonassessments.com). Estimates from sales fig- B
ticated and reliable system of behavior assessment. The ures indicate the BASC-2 is one of the frequently, if not
BASC-2 assesses a wide range of distinctive dimensions. the most, administered individual psychological tests in
In addition to evaluating personality and behavioral pro- the public schools of the United States.
blems and emotional disturbance, the BASC-2 identifies
positive attributes that can be capitalized on in the treat-
ment process, attributes that are valuable but too often
ignored in clinical assessment.
Historical Background
The range of dimensions assessed helps in making
The original BASC (Reynolds & Kamphaus, 1994) was
differential diagnosis of specific categories of disorder,
published by American Guidance Service following
such as those denoted in the Diagnostic and Statistical
7 years of development work. The original BASC was
Manual of Mental Disorders (DSM-IV-TR; American
standardized for use with ages 2.5 through 18 years,
Psychiatric Association, 2000), as well as general cate-
and was rapidly adopted as the most frequently adminis-
gories of problems, such as those addressed by The Indi-
tered behavior scales in the schools in the United States.
viduals with Disabilities Education Act. The BASC-2 is
Versions were subsequently developed for international
highly relevant to federal regulations concerning the diag-
applications by TEA Ediciones in Spain. Clinical settings
nosis of severe emotional disturbance among children in
also began to adopt and use the BASC throughout North
the schools and also is sensitive enough to detect even
America as research demonstrated strong sensitivity and
mild behavior problems among children in other disability
specificity in diagnostic applications. The original BASC
categories, including learning disabilities and mental
has been used in more than 125 such research studies,
retardation.
many of which were large-scale, longitudinal analyses of
The BASC-2 allows information from multiple
both developmental psychopathology and treatment effi-
sources to be compared using instruments with overlap-
cacy. Once the BASC-2 was released in 2004, use grew
ping norms to help achieve reliable and accurate diag-
geometrically in all areas of practice.
noses. The software programs available for the BASC-2 will
also compare data from each of the BASC-2 components
and assess them for similarities and differences statistically
and graphically. Psychometric Data
Each BASC-2 component is designed for a specific
setting or type of respondent because some constructs The BASC-2 scales were designed to be highly interpret-
or behaviors are more important or measurable in some able and are built around clearly specified constructs with
settings than in others. This also gives clinicians a clear matching item content, developed through a balance of
view of the generalizability of the individuals behavior as theory and empirical data. Experienced clinicians wrote
well as the setting-specific nature of any behavior pro- the initial items and they were assigned initially to scales
blems that occur principally in one type of surrounding. using expert methods but refined iteratively using an
The BASC-2, PRS, SRP, and SDH are available in empirical approach based on the responses of nearly
Spanish as well as English and are also available on 50,000 individuals. Scales are consistent not only across
audio for nonreaders. The BASC-2 can be either hand sex and age levels but also between the teacher and parent
scored or computer scored (manual or scannable item forms. This provides a basis for consistent interpretation
entry). The BASC-2 Spanish version sold in the United of scales and for meaningful across-source and across-
States was developed simultaneously with the English time score comparisons. BASC-2 norms are based on
version and thus is far more than a translation, being a large, US Census Bureau representative samples and are
fully developed scale with yoked items (see Reynolds & differentiated according to the age, sex, and clinical status
Kamphaus, 2004, for a more detailed explanation). of the child. Clinicians have a choice of sex-based norms
The BASC-2 has been widely used and is available or combined-sex norms when deriving standard scores for
throughout the English-speaking world from Pearson the various subscales and composites. The BASC-2 covers
Assessments of Bloomington, MN and throughout the full age range, 2 through 21 years, of students in
the Spanish-speaking world from TEA Ediciones in preschool through high school settings, while maintaining
Madrid. Versions in several other languages are also developmental sensitivity and continuity of constructs.
368 B Behavior Assessment System for Children (BASC)
The BASC-2 also offers a version of the SRP for ages 18 Academic difficulties are frequently linked to behavior
through 25 for use in technical schools, colleges, and problems. Syndromes such as attention-deficit hyperactivity
universities. disorder (ADHD) and depression have known academic
The BASC-2 scales and composites have high inter- consequences; learning disabilities and mental retardation
nal consistency and testretest reliability. Most alpha are often associated with adjustment problems such as low
coefficients for the BASC-2 subscales and composites self-concept or anxiety. It is strongly suggested that every
exceed 0.80 and are sufficiently reliable for application child experiencing academic difficulties receive a behavioral
to diagnostic and treatment issues. Additionally, the assessment. Additionally, research demonstrates that good
BASC-2 offers various types of validity checks to help behavioral assessment of constructs such as attitude to
the clinician detect careless or untruthful responding, school, attitude to teachers, study skills, attention problems,
misunderstanding, or other threats to validity. The and adaptability, in tandem with cognitive assessment,
BASC-2 Manual demonstrates validity evidence for the improves the prediction of both school performance and
proposed applications of the BASC-2 scales that is exten- response to intervention.
sive and covers both theoretical and actuarial bases. The BASC-2 is also useful for assessing severe emo-
Correlations with numerous other rating scales and tional disturbance. The rating scales can help distinguish
self-reports are given as well as studies of a large number between children with conduct disorders or social malad-
of clinical groups. justment on the one hand and those with severe emotional
disturbance on the other, as called for by Federal laws.
With its various components, the BASC-2 can help assess
Clinical Uses all aspects of the federal definition of severe emotional
disturbance.
Clinical Diagnosis The BASC-2 may be particularly useful in designing
individual educational plans (IEPs) for emotionally dis-
The BASC-2 aids in the clinical diagnosis of disorders that turbed children. It allows the selection of target behaviors
are usually first apparent in childhood or adolescence. as well as clusters of behaviors to delineate syndromes that
It assesses a variety of symptoms that are noted in the are an important focus of the IEP.
DSM-IV-TR. The BASC-2 rating scales and the SOS were also
Because the components of the BASC-2 can be used designed for use at the preschool level to help develop
separately or in combination, the BASC-2 may be used family service plans (FSPs) for 4- and 5-year-olds with
easily in residential settings, in clinics, or by private prac- disabilities. The SDH is well suited to identifying the
titioners. The PRS and SDH can be completed by a parent service needs of families.
while the child is being evaluated by the practitioner, thus The BASC-2 is also useful in manifestation
reducing the practitioners time in the data collection determination.
process. The rating scales, the SRP, and the SOS can be Manifestation determination refers to a process
repeated on a regular basis to monitor a childs progress for determining the origin of behavior. The procedure is
and response to treatment. commonly encountered in special education and in 504
It is highly desirable that diagnosis be linked clearly to proceedings related to disciplinary actions or conduct
intervention. In this respect, treatment planning can also problems. Prior to the application of any adverse action
be facilitated by the BASC-2. Problem behaviors can be against a student with a disability, a multidisciplinary
delineated and targeted in a program leading to their team must determine that the behavior in question was
reduction. A similar strategy can be used with deficits in not a direct result of the students disability. The method
adaptive skills. is based on the premise that students with a disability
Differential diagnosis is becoming an increasingly im- should not be punished for behavior that is considered to
portant issue in school settings. This is partly because the be a manifestation of the disability. For example, suppose
complexity of many childrens problems requires an array a child with schizophrenia is experiencing auditory
of interventions that must be tailored to the individual hallucinations at school and tells a teacher that another
childs needs. Consequently, the BASC-2 is designed to be child is threatening to commit a violent act. Because these
sensitive to numerous presenting problems in the class- auditory experiences were a manifestation of the childs
room, including deficiencies in social skills, study skills, or disability, he or she should not be punished for reporting
other adaptive skills. them as actual threats.
Behavior Assessment System for Children (BASC) B 369
In most cases, the BASC-2 will prove helpful in the the admissibility of expert testimony based on test results.
manifestation determination process. The BASC has a Reynolds and Kamphaus (2002) provides examples of
long history of effectiveness in differentiating social mal- uses of the original BASC in forensic situations such as B
adjustment and conduct disorder from behavior asso- child custody evaluations, personal injury litigation, and
ciated with an emotional disturbance (Reynolds & juvenile certification. This manual contains considerable
Kamphaus, 2002). The BASC-2 content scales such as information on the reliability of BASC-2 scores and asso-
anger control, bullying, and executive functioning en- ciated standard errors of measurement, on the normative
hance its utility for such purposes. samples, and on validation studies, all of which are consid-
ered by judges in determining admissibility of testimony
based partially or wholly on test data. Also presented
Assessment of Individuals with Limitations here are additional crucial data on the ability of the
of Vision and Hearing BASC to measure child and adolescent psychopatholo-
gy and to discriminate among various diagnostic
The BASC-2 scales can be used to evaluate the behavioral groups, capabilities that also are included in the con-
and emotional status of children and adolescents with sideration of admissible evidence. The BASC is well
sensory impairments. The interpretation of BASC-2 test established in clinical environments such as schools,
scores for these individuals requires specialized training, child guidance centers, university clinics, and private
expertise, and supervised experience in working with practice settings in the United States and abroad. The
groups with sensory impairment. use of tests in a wide variety of settings is important in
establishing credibility and admissibility in various legal
proceedings.
When choosing instruments for forensic evaluations,
Program Evaluation it is also important for clinicians to evaluate the instru-
ments ability to detect dissimulation (Reynolds & Kam-
Repeated use of the BASC-2 TRS, PRS, SRP, and SOS can phaus, 2002). In court proceedings, individuals may have
aid in identifying a childs progress in specific programs. much to gain by appearing to have more or fewer pro-
Improvement in designated areas of behavior and in affec- blems than actually exist. Because nearly any behavioral or
tive states may be noted, and the strengths and weaknesses emotional problem or disorder can be minimized or exag-
of programs thus identified. The original BASC was shown gerated, objective methods are needed to determine
in a number of evaluation studies to be sensitive to the whether dissimulation has occurred. The BASC-2 has
effects of various intervention programs for young chil- scales designed and tested for the detection of dissimula-
dren (including the evaluation component of Head Starts tion in responding by parents, teachers, and children.
Project Mastery) and adolescents (e.g., the evaluation by In particular, the BASC-2 validity scales can identify exag-
the Civilian Health and Medical Program of the Uniformed gerated responding, minimization of problem reporting,
Services, or CHAMPUS, of the effectiveness of residential inconsistencies, random answering patterns, and other
treatment for adolescents). These and other applications of response methods that lead to inaccurate depictions of
the BASC in program evaluation are reviewed in Reynolds the childs or adolescents behavior. Because of the strength
and Kamphaus (2002). With the improved score reliabil- of its validity scales, its documented psychometric proper-
ities and broadened content coverage in both clinical and ties, and the validity of its application in behavior evalua-
adaptive domains, the BASC-2 is expected to surpass the tion and differential diagnosis, the BASC-2 provides
BASC in the evaluation of programs and of interventions support to educate professionals or those challenged in
at both the individual and program levels. legal proceedings regarding their diagnostic or placement
decisions.
Forensic Evaluation
The Behavioral and Emotional Screening
The BASC-2 is appropriate for use in legal or forensic System (BESS)
settings. According to several US Supreme Court rulings
of the 1990s, evidence of the psychometric properties of The BESS (Kamphaus & Reynolds, 2007, a member of
tests used in a forensic setting is crucial for determining the BASC-2 system or family of assessment devices), is a
370 B Behavior Assessment System for Children (BASC)
variant of the BASC-2 designed to identify risk for behav- and social skills. Each of these areas is assessed in detail
ioral or emotional problems and predict mental health (along with numerous others) on the assessment compo-
and educational outcomes. The BESS includes two parent nents of the BASC-2.
rating forms for ages 3 through 5 and 6 through 18, two Each chapter provides a theoretical framework,
teacher rating forms for the same age groups, and one annotated bibliography citing selections of the research
self-report form for ages 8 through 18. Each form of the literature, and the essential elements of each interven-
BESS is brief and, therefore, practical at less than 30 items tion followed by procedural steps and considerations
each and taking about 510 min of informant time and no for application. The BASC-2 Intervention Guide is a
prior training and coaching of the informant. comprehensive source book (472 pp.) of interventions.
Using the same item response formats as the BASC-2, The essential elements and the procedural steps are
each BESS form produces a single score indicating nor- designed for use by psychologists, counselors, or other
mal (T = 2060), elevated (6170), or extremely school professionals with mental health training (unlike
elevated risk (T = 71 or higher). Validity indexes are the assessment components of the BASC-2 which re-
provided for each form, and Spanish adaptations and quire more extensive training and supervised experience
translations for parent and teacher forms. Test scoring in clinical assessment and are considered to be Level C
software is designed for large-scale screening of entire products).
classes, grade levels, or school systems by incorporating Corresponding Classroom guides include evidence
scanned data entry of forms and sophisticated individual based interventions appropriate for classroom settings
and group reporting options, and a tracking report option organized by both externalizing and internalizing groups
for community monitoring of behavioral and emotional of behaviors and contain some sample lesson plans and
adjustment status. forms for common interventions and procedures. Parent
The BESS manual includes a detailed discussion of Tip Sheets present information about each type of prob-
development procedures and a separate chapter devoted lem for communication with parents or caregivers on how
to validity and reliability evidence collected to date. to help at home.
Articles by DiStefano and Kamphaus (2007) and Kam-
phaus et al. (2007) provide additional validity evidence
associated with pilot studies of the BESS. Virtually all reli-
ability coefficients exceed 0.90 and predictive validity stud- References and Readings
ies of behavioral, emotional, and educational outcomes
from 2 to 4 years in length are reported, as well as correla- American Psychiatric Association. (2000). Diagnostic and statistical man-
tions with numerous behavior rating scale measures of ual of mental disorders (4th ed., text revision). Washington, DC:
Author.
adjustment.
Current Population Survey (2001). United Stated Bureau of the Census,
The BESS manual also provides a detailed discussion Current Population Survey, Washington, DC: US Bureau of the
of promising screening practices, including the use of Census.
multiple gates and informants, desired age or grade DiStefano, C., & Kamphaus, R. W. (2007). Development and validation
groups for screening, and linking screening to early inter- of a behavioral screener for preschool-age children. Journal of Emo-
tional and Behavioral Disorders, 15, 93102.
vention aimed at preventing the onset of mental health
Kamphaus, R. W., & Reynolds, C. R. (2007). Behavior assessment system
disorders or unsuccessful educational outcomes. for children second edition (BASC-2): Behavioral and emotional
screening system (BESS). Bloomington, MN: Pearson.
Kamphaus, R. W., Thorpe, J. S., Winsor, A. P., Kroncke, A. P., Dowdy,
BASC-2 Intervention Guide for Emotional E. T., & VanDeventer, M. (2007). Development and predictive va-
lidity of a teacher screener for child behavioral and emotional
and Behavioral Problems
problems at school. Educational and Psychological Measurement, 6,
115.
The BASC-2 Intervention Guide, another component of Ramsay, M., Reynolds, C. R., & Kamphaus, R. W. (2002). Essentials of
the BASC-2, (Vannest, Reynolds, & Kamphaus, 2008) behavioral assessment. New York: Wiley.
provides an articulation of evidence-based practices for Reynolds, C. R., & Kamphaus, R. W. (1994). Behavior assessment system
for children. Bloomington, MN: Pearson Assessments.
each of 11 common types of emotional and behavioral
Reynolds, C. R., & Kamphaus, R. W. (2002). The clinicians guide to the
problems: aggression, conduct problems, hyperactivity, at- behavior assessment system for children. New York: Guilford.
tention problems, academic problems, anxiety, depression, Reynolds, C. R., & Kamphaus, R. W. (2004). Behavior assessment system
somatization, functional communication, adaptability, for children (2nd ed.). Bloomington, MN: Pearson Assessments.
Behavior Modification B 371
Definition Definition
Techniques used to control or modify an action or perfor- Behavior modification is the use of basic learning techni-
mance of a subject. This is a less-intensive version of behav- ques, such as conditioning, biofeedback, assertiveness
ior modification in which the goal is to develop, strengthen, training, positive or negative reinforcement, hypnosis, or
maintain, decrease, or eliminate behaviors in a planned or aversion therapy, to change unwanted individual or group
systematic way. Behavior management skills are particularly behavior. A technique, typically based on functional
important to enhance the probability that individuals, or assessment, used to reinforce adaptive behaviors while
groups, choose behaviors that are pro-social. Prosocial diminishing or extinguishing maladaptive behaviors.
behaviors are typically seen as personally fulfilling, produc- Seven characteristics of behavior modification, identified
tive, and socially acceptable. Persons surviving a traumatic by Martin and Pear (2007), include:
brain injury (TBI) often have behavioral disturbances such A strong emphasis on defining problems in terms of
as disinhibition and/or agitation. Due to learning impair- measurable behavior
ments as a result of their TBI, the traditional behavior Making environmental adjustments to improve
management approaches, which are based on learning the- functioning
ory principles, are modified. For example, behavior man- Precise methods and rationales
agement approaches with TBI survivors may focus more on Dynamic real-life application of techniques
stimulus control (e.g., controlling environmental cues) than Techniques grounded in learning and behavior theory
operant conditioning (e.g., recalling the contingency be- Scientific demonstration linking the imposed tech-
tween behaviors and the resulting consequences). nique with behavior change
Strong emphasis on accountability
Cross References
Cross References
Behavior Analysis
Behavior Modification Applied Behavior Analysis
Behavior Therapy Behavior Management
Behavioral Therapy Behavioral Analysis
Behavioral Assessment
Behavioral Therapy
References and Readings
Jacobs, H. E. (1993). Behavior analysis guidelines and brain injury rehabil- References and Readings
itation: People, principles and programs. Gaithersburg, MD: Aspen.
Karol, R. L. (2003). Neuropsychological intervention: The practical treat- Crum, C. (2004). Using a cognitive-behavioral modification strategy to
ment of severe behavioural dyscontrol after acquired brain injury. Boca increase on-task behavior of a student with a behavior disorder.
Raton, FL: CRC Press. Intervention in School & Clinic, 39(5), 305.
372 B Behavior Rating Inventory for Executive Functions
Hicinbothem, J., Gonsalves, S., & Lester, D. (2006). Body modification The BRIEF-P measures the behavioral manifestations of
and suicidal behavior. Death Studies, 30(4), 351.
executive function in preschool children aged 25. The
Lindsey, N., Reif, J., Bachand, A., & Seys, S. (2005). Behavior modifica-
tion following a diagnosis of hepatitis C infection. American Journal
BRIEF-P consists of a single form completed by parents
of Health Behavior, 29(6), 512519. and/or teachers/caregivers to rate the childs executive func-
Martin, G. L., & Pear, J. (2007). Behavior modification: What it is and how tions within the home and preschool settings. The question-
to do it (8th ed.). Upper Saddle River: Pearson Prentice Hall. naire consists of 63 items comprising five theoretically and
Ntinas, K. (2007). Behavior modification and the principle of normaliza-
empirically derived clinical scales measuring: Inhibit, Shift,
tion: clash or synthesis? Behavioral Interventions, 22(2), 165177.
Emotional Control, Working Memory, and Plan/Organize.
These scales form three factor-derived indexes: Inhibitory
Self-Control, Flexibility, and Emergent Metacognition and
one composite score the GEC. The BRIEF-P also includes
Behavior Rating Inventory for two validity scales Inconsistency and Negativity.
Executive Functions The BRIEF-SR is an 80-item adolescent self-report
behavior rating scale designed for older children and
G ERARD A. G IOIA 1, P ETER K. I SQUITH 2 adolescents, ages 1118 years with a fifth-grade or higher
1
Childrens National Medical Center reading ability, to complement the BRIEF Parent and
Rockville, MD, USA Teacher Forms. The 80 items yield information for eight
2
Dartmouth Medical School non-overlapping clinical scales measuring: Inhibit, Shift,
Lebanon, NH, USA Emotional Control, Monitor, Working Memory, Plan/
Organize, Organization of Materials, and Task Comple-
tion. The clinical scales form two broader indexes the
Synonyms Behavioral Regulation Index (BRI) and the Metacognition
Index (MI) and yield an overall summary score, the
BRIEF; BRIEF-A; BRIEF-P; BRIEF-SR GEC. The BRIEF-SR also includes the Negativity and
Inconsistency validity indexes.
The BRIEF-A measures the adults executive functions
Description in his or her everyday home and work environments. Two
formats are used a self-report and an informant report.
The Behavior Rating Inventory of Executive Function The Self-Report Form is designed to be completed by
(BRIEF) family of measures are rating scales completed adults 1890 years of age while the Informant Report
by parents and teachers of school children aged 518 years Form is administered to an adult who is familiar with
(BRIEF), by parents and caregivers of preschool children the rated individuals everyday functioning. The latter
aged 25 years (BRIEF-Preschool Version; BRIEF-P), by form can be used alone when the rated individual is
adolescents aged 1118 years (BRIEF-Self-Report Version; unable to complete the Self-Report Form or has limited
BRIEF-SR), or by adults aged 1890 years and their awareness of his or her own difficulties, or with the Self-
informants (BRIEF-Adult Version; BRIEF-A). Each Report Form to gain multiple perspectives on the indivi-
version facilitates assessment of the everyday behavioral duals functioning. The BRIEF-A is composed of 75 items
manifestations of executive dysfunction in the home, within nine nonoverlapping clinical scales measuring:
school, and/or work environments. Inhibit, Self-Monitor, Plan/Organize, Shift, Initiate, Task
The BRIEF consists of two forms, a parent question- Monitor, Emotional Control, Working Memory,
naire and a teacher questionnaire, designed to assess exec- Organization of Materials. The clinical scales form two
utive function behaviors of children and adolescents aged broader factor-based indexes: BRI and MI, and these
518 years in the home and school environments. It indexes form the overall summary score, the GEC. The
includes 86 items within eight non-overlapping clinical BRIEF-A also includes three validity scales Negativity,
scales measuring Inhibit, Shift, Emotional Control, Inconsistency, and Infrequency.
Initiate, Working Memory, Plan/Organize, Organization
of Materials, and Monitor. The BRIEF also includes two Historical Background
validity scales Inconsistency and Negativity. The eight
scales form two broader indexes based on the factor struc- Executive functions have been historically evaluated using
ture, Behavioral Regulation and Metacognition, as well as laboratory-based performance tests. While these types of
an overall score, the Global Executive Composite (GEC). measures offer the advantages of control over extraneous
Behavior Rating Inventory for Executive Functions B 373
variables and potential to fractionate and examine com- as well as the cerebellum. Furthermore, studies of indivi-
ponents of executive function separately such as planning duals with acquired focal lesions in non-frontal brain
versus working memory, they are limited in ecological regions such as the basal ganglia have provided further B
validity, or the ability to predict functioning in the support for a distributed circuitry model of executive
everyday environment. Fundamentally, executive func- functions. Damage to any given component of this
tions are necessary for organization of goal-directed be- circuitry may result in executive dysfunction.
havior in the everyday, real world, environment. Thus, The BRIEF was originally developed beginning in
in addition to assessing these functions with clinical 1994 by the authors following a commonly accepted
performance measures, it is important to also capture developmental model of executive function. The impetus
behavioral manifestations of executive function or dys- arose from frequent observations in clinical practice that
function. The BRIEF was developed to measure executive parent and teacher reports of a childs functioning in the
function through the assessment of individuals behavior everyday environment did not always, or even often, fit
in their everyday environments. Given the challenges of with the same childs test performance on putative
executive function assessment in the laboratory and executive function performance tests. Although originally
inherent limitations to applicability in the everyday intended for the authors own clinical and research use,
environment and to treatment, attention has increasingly the measure found acceptance initially in the pediatric
turned to alternative methods of evaluation that offer neuropsychology domain, and was published first in
enhanced ecological validity. Assessment methods that 2000, with subsequent versions published in the ensuing
reliably tap the individuals everyday executive problem- years. Since then, the several versions of the BRIEF have
solving in natural settings offer a complementary become widely used across the age spectrum and across
approach to clinical performance-based assessment. clinical, school, and research settings. Since publication, a
Executive function is generally viewed as a broad substantial body of literature has developed examining
umbrella term that encompasses a set of interrelated BRIEF profiles with a wide range of clinical groups
subdomains. Although authors vary in which functions (Strauss, Sherman, & Spreen, 2006).
are viewed as executive function domains, they typically
include: initiation of goal-directed behavior, inhibition of
competing actions or stimuli, planning and selection of Psychometric Data
relevant task goals, organization of behavior to solve
complex problems, flexible shifting of problem-solving BRIEF (Age 518 Years; Parent,
strategies when necessary, and monitoring and evaluation Teacher Forms)
of problem-solving behavior. In support of these
behaviors, working memory capacity plays a fundamental Standardization: Normative data are based on 1,419
role in holding information actively on-line in the parents and 720 teachers from rural, suburban, and urban
service of problem-solving, including planning and areas, reflecting 1999 US Census estimates for distributions
organization. Importantly, the executive functions are of SES, ethnicity, and gender. Separate normative tables,
not exclusive to cognition; emotional control is also including T scores, percentiles, and confidence intervals,
relevant to effective problem-solving activity and should are provided for four age groups for boys and girls sepa-
be considered in any definition. Historically, executive rately, with norms for both the parent and teacher forms.
functions have been closely associated with the integrity Clinical data are based on 212 parents and 120 teachers
of the frontal lobes of the brain. Much of the evidence rating children with developmental disorders or acquired
supporting a role for the frontal lobes in executive neurological disorders (e.g., learning disabilities, attention-
functions has come from studies of individuals with deficit/hyperactivity disorder (ADHD), traumatic brain
acquired focal damage to this region. More recently, injury (TBI), Tourettes syndrome, mental retardation,
studies using advanced brain imaging techniques such as epilepsy, and language disorders).
positron emission tomography (PET) and functional Reliability: High internal consistency (Cronbachs
magnetic resonance imaging (fMRI) have shown that alpha = 0.800.98 for both parent and teacher forms).
the frontal lobes play an essential role in executive func- Test retest reliability (Mean r = 0.82 for normative sample
tions. However, these same studies have also clearly shown parent ratings, Mean r = 0.88 for clinical sample teacher
that executive functions are not subserved by the frontal ratings). Inter-Observer (teacherparent) reliability
lobes alone, but rather by distributed neural circuitry that reflected in moderate correlations (Mean r = 0.32 for
includes other cortical regions and subcortical structures normative samples, Mean r = 0.34 for clinical samples).
374 B Behavior Rating Inventory for Executive Functions
Validity: Evidence of validity is demonstrated by 1118 years. The normative sample includes 1,118 parti-
several lines of evidence including high inter-rater cipants from a wide range of racial/ethnic backgrounds,
agreement for item-scale assignments by expert panel, educational backgrounds, as well as geographic regions
factor analytic studies, and structural equation modeling. that are matched to US Census data.
Convergent and divergent validity evidence is demon- Reliability: The BRIEF-SR scales demonstrate appro-
strated by convergence with scales of inattention and priate reliability. Internal consistency is high for the GEC
impulsivity and divergence of behavioral/emotional func- (a = 0.96) and moderate to high for the clinical scales
tioning from executive functioning using the ADHD-IV, (a = 0.720.96). Temporal stability is strong (r = 0.89 for
Behavior Assessment System for Children (BASC), CBCL, the GEC over a period of approximately 5 weeks), and
and CRS. Principal factor analysis of the BRIEF Parent there is strong inter-rater agreement for the GEC with
and Teacher Forms yielded a consistent 2-factor solution parent ratings on the BRIEF (r = 0.56). Teacher ratings on
(i.e., Behavioral Regulation, Metacognition) for norma- the BRIEF correlated less strongly with adolescent ratings
tive and clinical samples. Two of the Scales, Working on the BRIEF-SR (GEC r = 0.25) but were well within
Memory and Inhibit, are clinically useful in detecting expectations.
and predicting the diagnosis of ADHD. Validity: Principal factor analysis of the BRIEF-SR
yielded a 2-factor solution (i.e., Behavioral Regulation,
Metacognition) for normative and clinical samples. Corre-
BRIEF-Preschool (BRIEF-P; Age 25 Years; lational analyses with other self-report behavior rating
Parent, Caretaker Forms): scales (i.e., Child Behavior Checklist/Youth Self-Report
(CBSL/YSR), Behavior Assessment System for Children
Standardization: Normative data based on child ratings Self-Report of Personality (BASC-SRP), Child Health
from 460 parents and 302 teachers from urban, suburban, Questionnaire (CHQ), Profile of Mood States-Short Form
and rural areas, reflecting 1999 US Census estimates for (POMS-SF)) provide evidence of convergent and divergent
race/ethnicity, gender, socioeconomic status, and age. validity for the BRIEF-SR. Examination of BRIEF-SR pro-
Clinical samples included children in the following files in a variety of clinical groups provides further evidence
diagnostic/clinical groups: ADHD, low birth weight/ of validity based on clinical utility. BRIEF-SR ratings for
prematurity, language disorders, autism spectrum groups of adolescents with ADHD-I, ADHD-C, Insulin-
disorders, and a mixed clinical group. Dependent Diabetes Mellitus, Autism Spectrum Disorders,
Reliability: High internal consistency (a = 0.800.95 and Anxiety and Depressive Disorders showed different
for parent sample and a = 0.900.97 for teacher sample); patterns of scale elevations for each group compared to
testretest reliability (r = 0.780.90 for parents and matched control groups. Correlations between adolescent
0.640.94 for teachers); and modest correlations between and parent ratings for the clinical groups were strong,
parent and teacher ratings (r = 0.140.28). suggesting good agreement much of the time.
Validity: Convergent and discriminant validity
evidence established with other measures of inattention,
hyperactivity-impulsivity, depression, atypicality, anxiety, BRIEF-Adult (BRIEF-A; Self-Report, Informant
and somatic complaints (ADHD-IV-P, CBCL/15, Report)
BASCPRS). Factor analytic studies provide support for
a three-factor model of executive functioning embodied Standardization: The BRIEF-A was standardized and
by the three indexes in the parent and teacher normative validated for use with men and women from ages 18 to
groups, respectively. The Working Memory and the 90 years. The normative sample includes adults from a
Plan/Organize scales define the first component; the wide range of racial/ethnic backgrounds, educational
Shift and Emotional Control scales comprise the second backgrounds, as well as geographic regions that are
component; and the Inhibit and Emotional Control scales matched to US Census data.
define the third component. Reliability: The BRIEF-A has demonstrated multiple
lines of evidence for reliability. Internal consistency was
moderate to high for the Self-Report normative sample
BRIEF-Self-Report (BRIEF-SR): (a = 0.730.90 for clinical scales; 0.930.96 for indexes
and GEC) and high for the Informant Report normative
Standardization: The BRIEF-SR was standardized and sample (a = 0.800.93 for clinical scales; 0.950.98 for
validated for use with children and adolescents aged indexes and GEC). Using a mixed sample of clinical or
Behavior Rating Inventory for Executive Functions B 375
healthy adults who were seen for clinical evaluation or such as ADHD, TBI, lesions of the frontal lobes, Type 1
research study participation, internal consistency was Diabetes Mellitus, autism spectrum disorders, learning
high for the Self-Report Form (a = 0.800.94 for clinical disabilities, myelomeningocele and hydrocephalus, B
scales; 0.960.98 for indexes and GEC) and the Informant Tourettes syndrome, phenylketonuria, bipolar disorder,
Report Form (a = 0.850.95 for clinical scales; 0.960.98 obstructive sleep apnea, 22q11 deletion syndrome, galac-
for indexes and GEC). Test-retest correlations over a tosemia, sickle cell disease, and early focal frontal lesions.
4-week period across the clinical scales ranged from The BRIEF-Adult Version has been examined in indivi-
r = 0.820.93 for the Self-Report Form (n =0.50) and duals with Mild Cognitive Impairment, ADHD, Multiple
from r = 0.91 to 0.94 for the Informant Report Form Sclerosis, Alzheimers disease, epilepsy, and TBI. The
(n = 0.44). Correlations between Self-Report ratings and measures also show promise for veridicality, i.e., predict-
Informant Report ratings were moderate, ranging from ing behavior in the natural environment. The measures
r = 0.44 to 0.68 for the clinical scales and from 0.61 to 0.63 are increasingly being examined in relationship to other
for the indexes and the GEC. indications of everyday functioning. Correlational
Validity: The BRIEF-A exhibits multiple lines of analyses suggest strong, logical relationships between
validity evidence as an ecologically sensitive measure executive function and everyday behaviors such as impul-
of executive functioning in individuals with a range of sivity with aggression and working memory with atten-
conditions across a wide age range. In terms of convergent tion problems. Correlations with other real-world
validity evidence, the Self- and Informant Report Form of functioning such as adaptive functioning are reported in
the BRIEF-A scales, indexes, and GEC demonstrated individuals with developmental disabilities and scholastic
significant correlations in the expected direction with achievement. Teacher ratings of executive function on the
self- and informant reports on the Frontal Systems BRIEF predict student performance on high-stakes
Behavior Scale, Dysexecutive Questionnaire, and testing. While there are modest correlations between the
Cognitive Failures Questionnaire. Validity was further BRIEF and performance tests that tap aspects of executive
demonstrated via concurrent profiles of BRIEF-A scores functions, there are more intriguing relationships between
in clinical populations such as ADHD, multiple sclerosis, the BRIEF and biological markers such as genetic
epilepsy, mild cognitive impairment, and TBI. Validity conditions (e.g., 22q11 deletion syndrome), brain injuries
studies also are reported in the Professional Manual (e.g., frontal lobe lesions), metabolic conditions (e.g.,
comparing the BRIEF-A with the Clinical Assessment of phenylketonuria), and structural and functional imaging
Depression, the Geriatric Depression Scale, the Beck (e.g., frontal volume and activation). Finally, certain
Depression Inventory-II, and the State Trait Anxiety profiles of executive function in the everyday environ-
Inventory. Factor analysis of Self-Report Form data ment predict diagnosis of ADHD.
yielded a 2-factor solution (i.e., Behavioral Regulation, An understanding of the individuals profile of execu-
Metacognition) for normative and mixed clinical/healthy tive function strengths and weaknesses can lead to targeted
adult samples, accounting for 73 and 76% of the variance, pharmacological, behavioral, cognitive, or other therapeu-
respectively. Factor analysis of Informant Report Form tic interventions. Such strategies may be specifically tar-
data also yielded a similar 2-factor solution for the geted toward one area of executive functions such as
normative and mixed clinical/healthy adult samples, antecedent management for children with inhibitory con-
accounting for 81 and 78% of the variance, respectively. trol deficits, or may be more programmatic, such as the
comprehensive cognitive rehabilitation programs. The
BRIEF has potential uses for clinical treatment design,
Clinical Uses monitoring, and outcome measurement. Data from the
BRIEF can help the clinician focus on potentially problem-
Given the central importance of the executive functions to atic areas requiring further assessment. The same data may
the direction and control of dynamic real world behav- inform decisions about targets for treatment and types of
ior, the BRIEF was designed for a broad range of indivi- interventions based on the potential for ameliorating real-
duals with developmental, neurological, psychiatric, and world problems. Given that the BRIEF captures the patients
medical conditions. Deficits in various subdomains of the everyday functioning, the scales can suggest specific pro-
executive functions are central characteristics of many blems for which treatment goals and strategies can be
developmental and acquired neurological disorders across targeted. For example, an individual who is described as
the lifespan. Executive function deficits measured via the disinhibited in the everyday world might have treatments
BRIEF have been demonstrated in a variety of populations and supports targeted specifically toward boosting
376 B Behavior Therapy
inhibitory control or limiting opportunity for impulsive Gioia, G. A., Isquith, P. K., & Kenealy, L. (2008). Assessment of behavioral
aspects of executive function. In V. Anderson, R. Jacobs, & P. Anderson,
behavior. A child with difficulties shifting set might benefit
(Eds.), Executive functions and the frontal lobes: A life span approach.
from teaching and intervention strategies that incorporate Sussex, England: Psychology Press.
use of routines and schedules to reduce agitation and anxi- Gioia, G. A., Isquith, P. K., Kenworthy, L., & Barton, R. M. (2002). Profiles
ety when change is needed. of everyday executive function in acquired and developmental
Finally, behavioral assessment of executive function disorders. Child Neuropsychology, 8, 121137.
Isquith, P. K., Crawford, J. S., Espy, K. A., & Gioia, G. A. (2005). Assess-
can also contribute to treatment monitoring and eventual
ment of executive function in preschool children. Mental Retardation
outcome evaluation. Given the inherent difficulty in and Developmental Disability Research Review, 11, 209215.
administering performance measures of executive Malloy, P., & Grace, J. (2005). A review of rating scales for measuring
function in a repeated fashion, behaviorally anchored behavior change due to frontal systems damage. Cognitive and
measures may be more suited to such within-subjects Behavioral Neurology, 18, 1827.
Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). Behavior rating
methods. For example, a patient concerned about
inventory of executive function (BRIEF). A compendium of
attentional difficulties might reveal problems with inhibi- neuropsychological tests, administration, norms, and commentary
tory control and working memory on the BRIEF. After (3rd ed., pp. 10901099). New York: Oxford University Press.
appropriate interview and clinical diagnosis, treatment
methods might include medication and cognitive-behavior
therapy. To evaluate effectiveness of treatment, the measure
may be administered again after starting medication, and Behavior Therapy
again after a longer period to determine whether the effects
of treatment are maintained. Ratings can be provided by Behavior Modification
the individual themselves or an informant in their environ-
ment who has the opportunity to regularly observe their
behavior (e.g., parent, teacher, spouse). More frequent
monitoring might also be appropriate in some cases, such
Behavior/Behavioral Analysis
as for individuals who sustain a mild TBI, where full
Behavioral Assessment
neuropsychological evaluation may not be feasible or
appropriate at the time, but rapid, timely assessment of
functioning is important for determining when the
individual may return to normal activities. Behavior/Behavioral Observation
Behavioral Assessment
Cross References
In the Zoo Map Test, examinees are asked to show Shallice and Burgess (1991) developed the Six Elements
how they would visit a series of designated locations on a Test, which required examinees to carry out six tasks
map of a zoo, while following certain rules. In the high in a limited time frame without violating certain rules.
demand component, examinees incur a high number of It was tailored to a difficulty level that was consistent
errors by simply visiting the locations in the order given in with the high level of functioning of Shallices and
the instructions. In the low demand component, exam- Burgesss patients. Wilson et al. (1996) modified the Six
inees are simply required to follow the instructions to Elements Test, simplifying it for the more severely im-
produce an error-free performance. The goal of the test paired and less intellectually able patients who are often
is to assess spontaneous planning abilities. seen by neuropsychologists. These modifications evolved
The Modified Six Elements Test requires the completion into the BADS.
of three tasks (i.e., dictation, arithmetic, and picture nam-
ing), each of which is divided into parts A and B. Examinees
are required to attempt at least a part from each of the six Psychometric Data
subtests within a 10-min period and are instructed not to
complete both parts of the same task consecutively. The Multiple studies established the psychometric properties
goal of this component is to determine the examinees of the BADS. Wilson et al. (1996) found that inter-rater
ability to plan, organize, and monitor behavior. reliability was high, ranging from 0.88 to 1.00. Testretest
The Dysexecutive Questionnaire (DEQ) consists of reliability was also examined, with examinees generally
21 items that sample the range of problems commonly performing slightly, but not significantly, higher after the
associated with the Dysexecutive Syndrome. Four broad second administration. Correlations between the first
areas are sampled: emotional or personality changes, mo- and second test administrations were moderate, with the
tivational changes, behavioral changes, and cognitive exception of the Zoo Map Test, where virtually no corre-
changes. Items are scored on a 5-point (04) Likert lation was found. This lack of correlation was attributed
scale, ranging from Never to Very Often. Two versions to the presence of outliers and small sample size (n = 25).
are available, one completed by the examinee and another The testretest reliability of the BADS was similar in
by an informant. pattern to other tests of frontal lobe functioning admi-
nistered at the same time (e.g., Modified Card Sorting
Test; Nelson, 1976).
Historical Background The validity of the BADS was assessed across varied
populations. Bennett, Ong, and Ponsford (2005) investi-
The BADS is designed to evaluate patterns of deficits that gated the sensitivity of the BADS to executive dysfunc-
are typically subsumed under the functions of the frontal tion in a sample of 64 Australian patients who were
lobes. Rylander (1939) enumerated the deficits as involv- involved in motor vehicle or workplace accidents. All
ing disturbances in attention, increased distractibility, the patients experienced a loss of consciousness and
impaired ability to learn new tasks, and deficits contend- varying degrees of post-traumatic amnesia (PTA). The
ing with complex information. Shallice (1982) described authors concluded that scores derived from the BADS
this pattern of deficits as comprising impairment in atten- and other measures used in their study were only mod-
tional control, which he termed the supervisory system. erately useful in assessing executive dysfunction. In con-
Baddeley (1986) analogized the supervisory system to the trast, several studies found the BADS to discriminate
central executive component of working memory and between patients and control subjects. Krabbendam, de
suggested the term Dysexecutive Syndrome as a way of Vugt, Derix, and Jolles (1999) were able to discriminate
characterizing patients with this pattern of impairment. between schizophrenic patients and controls. Additional-
Such patients are most likely to present as impulsive, ly, Katz, Tadmor, Felzen, and Hartman-Maeir (2007) were
distractible, unable-to-use feedback to modify responses, able to discriminate between acute and chronic schizo-
and to behave inappropriately in social situations. phrenics, with the latter evidencing greater executive
The BADS was developed because patients who dysfunction. Verdejo-Garcia and Perez-Garcia (2007)
exhibited obvious impairments with executive function- examined the usefulness of the BADS in determining
ing in their day-to-day functioning often performed executive dysfunction in a Spanish sample of substance-
adequately on traditional tests of executive function- dependent examinees (SDI). They concluded that the
ing such as the Wisconsin Card Sorting Test or the BADS yielded greater effect sizes for differences between
Stroop Color Word Test. To address this discrepancy, SDI and controls than did traditional measures of
Behavioral Regulation B 379
Frontal Lobe
Behavioral Prothestics
References and Readings Cognitive Correctors
could be gradually acquired by rewarding approximations fear of heights, etc.). The phobic behavior can be defined
to the behavior. as avoidance of, or escape from, the phobic stimulus (e.g.,
escaping/running away from a spider or avoiding sit- B
uations involving public speaking). By escaping from
Current Knowledge the phobic stimulus patients can reduce their anxiety.
The behavior of escape/avoidance is reinforced since the
Behavioral therapy has been successfully used for a variety reduction of the anxiety is reinforcing for the individual
of problem behaviors including, but not limited to, chronic (negative reinforcement is a concept derived from operant
pain, substance abuse, depression, phobias, autism, obesity, conditioning). In SD, patients are gradually exposed to
managing stress, smoking cessation, anorexia, obsessive the phobic stimuli, allowing them to acclimate themselves
compulsive disorder, and attention-deficit/hyperactivity to it, until they are able to tolerate it. Patients create a
disorder. It has been extensively used in patients with hierarchy of fear steps that they must overcome to reach
developmental disabilities, severely disturbed psychotic the last step, the phobic stimulus. These hierarchies can be
patients, survivors of brain injury, and others where in- imaginable, pictures or actual exposure. Patients deal with
sight-oriented or cognitive therapies may not be effective. each successive step until the hierarchy is completed.
There are a myriad of methods involved including: Typically, patients are taught relaxation skills to control
fear responses during exposure to the hierarchy.
Systematic desensitization (SD)
Behavioral therapy treatment tends to be of shorter
Exposure and response prevention
duration than more traditional modes of psychotherapy
Contingency management
(e.g., psychodynamic). Initial sessions are dedicated to
Flooding
the explanation of the basic tenets of behavioral change
Modeling
(e.g., reinforcement, extinction, punishment, etc.). Once
Applied behavior analysis
established, a variety of techniques may be utilized
Operant conditioning
including:
Respondent conditioning
Role-playing therapist models desired behaviors or
Some of these techniques are used in everyday life. For
reactions.
example, parents and teachers place stars on a refrigerator
Skills training patient is taught new desired beha-
chart or bulletin board to reward desirable behavior by
viors to replace undesired ones for parenting, social
children. Some techniques involve accumulating points
situations, public speaking, etc.
for performing a desired behavior, points that can later be
Flooding form of systematic desensitization where
exchanged for some desirable reinforcer. These token
the patient is exposed directly to the feared stimulus
economies are a variation of operant conditioning and
to extinguish the fear response.
are used in a variety of settings. In addition, extinction of
Homework patients are to try out new behaviors
undesired behavior has penetrated the mainstream as seen
learned in therapy in real-life situations.
by the use of time out, a technique involving the re-
Conditioning application of reinforcement to in-
moval of a child from reinforcement, seen by the child as
crease a desired behavior or the removal of reinforce-
somewhat aversive, or punishing, with the hope of de-
ment to decrease an unwanted behavior (e.g., token
creasing the unwanted behavior.
economies).
Behavioral therapy is based on the concepts that (1)
Relaxation training used to help patients relieve
targeted behaviors can be modified by a variety of behav-
anxiety/tension. An important component of system-
ioral techniques and (2) that the newly acquired behaviors
atic desensitization.
will be more adaptive than the undesired ones. These
techniques tend to be empirical (data-driven) and observ- The use of behavioral therapy in the treatment of sur-
able. They do not rely for their effectiveness on any mental vivors of severe traumatic brain injury (TBI) can be
(cognitive) constructs like unconscious motivations. They problematic. Those who demonstrate severe behavioral
simply identify a behavior to change and change it rather dyscontrol as a result of their TBI also likely possess
than trying to understand why the individual was cognitive sequelae that hinder the successful therapeutic
performing that behavior. An example of one of these use of these techniques. Persons with severe memory
techniques is the use of SD. deficits may not be able to recall the behavior they
This technique is often used with people who have a performed to earn a reinforcer in a contingency manage-
specific phobia (e.g., fear of snakes, fear of closed spaces, ment system. Memory problems may also interfere
382 B Behavioral Variant Frontotemporal Dementia
with a survivors ability to recall that a particular behavior Kazdin, A., & Hersen, M. (1980). The current status of behavior therapy.
Behavior Modification, 4(3), 283302.
led to a particular consequence. Without this ability
Masters, J., Burish, T., Holton, S., & Rimm, D. (1987). Behavior therapy:
to recall contingencies, survivors are likely to not be able Techniques and empirical findings. San Diego: Harcourt Press
to make different choices (i.e., make behavior changes) Jovanovich.
for which behavior they exhibit in given situations. There- Wilson, K. (1997). Science and treatment development: Lessons from the
fore, behavior management strategies place special em- history of behavior therapy. Behavior Therapy, 28, 547558.
phasis on controlling environmental stimuli in order
to reduce problem behaviors (e.g., disinhibition and
agitation).
The goal of behavior therapy with moderate or mild Behavioral Variant
TBI survivors is to provide the patient with a behavioral
repertoire that they can use to solve daily life problems
Frontotemporal Dementia
as a result of cognitive deficits (i.e., compensatory ap-
Frontal Temporal Dementia
proaches). Critical behavioral therapy techniques utilized
Picks Disease
include self-monitoring, scheduling of activities, role-
playing, modeling, and contingency contracting.
understand the neural mechanisms of learning, a notable occurring around the mean and increasingly fewer obser-
example being Donald Hebbs seminal postulate that vations/scores occurring farther (above/below) from the
concordant firing in synaptically coupled neurons mean (68.26% of observations/scores fall within one B
increases the strength of the connection between the standard deviation of the mean; 95.44% fall within two
two neurons. This early postulate proved highly influen- standard deviations of the mean). A normal distribution
tial in subsequent research on the neural mechanisms of of observations is typical in large samples acting additively
learning. and independently, and is assumed in parametric statists
(e.g., t-tests, ANOVA). Standardized scores derived from
neuropsychological measures are based upon (assume)
Cross References normal distribution of the standardization sample, and
provide for direct comparison of performance between
Behavior Modification
different measures.
Cognitive Behavior Therapy
Learning
Cross References
References and Readings
Base Rate (Population)
Beggs, J. M., Brown, T. Y., Byrne, J. H., Crow, T., LeDoux, J. E., LeBar, K. Cut Off Scores, Cutting Scores
(1999). Learning and memory: Basic mechanisms. In M. J. Zigmond, Intelligence Quotient
F. E. Bloom, S. C. Landis, J. L. Roberts, & L. R. Squire (Eds.), Funda-
Mental Age
mental neuroscience (pp. 14111454). San Diego, CA: Academic Press.
Mills, J. A. (1998). Control: A history of behavioural psychology. New York: Percentiles
New York University Press. Standard Scores
ODonohue, W. T. (2001). The psychology of B.F. Skinner. Thousand Oaks,
CA: Sage.
Staddon, J. E. R. (2000). The new behaviorism: Mind, mechanism, and
society. Philadelphia, PA: Psychology Press. References and Readings
Bells Palsy
Bell Curve
T HESLEE J OY D E P IERO
E RICKA WODKA Boston University School of Medicine
Kennedy Krieger Institute Boston, MA, USA
Baltimore, MD, USA
Synonyms
Synonyms
Idiopathic facial paralysis
Gaussian distribution; Normal curve; Normal
distribution
Definition
Definition
Bells palsy is the acute onset of paralysis of the muscles
A normal distribution of observations/scores is shaped innervated by the facial nerve, not due to obvious causes
like a bell, with the majority of observations/scores such as trauma, stroke, or local infection.
384 B Belle Indifference
Current Knowledge Lyme disease can also cause Bells palsy. In endemic
areas, Lyme disease antibody tests should be done.
Anatomy Ramsay-Hunt syndrome refers to Bells palsy caused
by Varicella-Zoster (the virus that causes chicken-
The facial nerve innervates the muscles that control pox and shingles). The distinguishing characteristic of
the forehead and eyebrow, close the eyelids, and move Ramsay-Hunt is the presence of vesicles (small fluid filled
the cheeks and lips. It also supplies taste to the anterior blisters) in the eternal auditory canal or on the eternal ear.
two thirds of the tongue, and innervates the stapedius
muscle (a small muscle in the middle ear, connecting the
tympanic membrane to the stapes, that dampens excessive
vibration in the tympanic membrane due to loud noises). Treatment
Epidemiology
References and Readings
Incidence: 23/100,000 annually.
Cases in women and men are equal. Gilden, D. (2004). Clinical practice. Bells palsy. New England Journal of
Season: no seasonal preference. Medicine, 351, 13231331.
Age: occurs equally in all age groups. Hazin, R., Azizzadeh, B., & Bhatti, M. (2009). Medical and surgical
management of facial nerve palsy. Current Opinion in Opthalmology,
20(6), 440450.
Etiology
Epidemiology
Benign Epilepsy of Childhood
It is estimated that 1525% of school-aged children with
with Centrotemporal Spikes epilepsy meet diagnostic criteria for BRE. Males are be-
(BECTS) lieved to be more affected (3:2). Perinatal complications,
head trauma, central nervous system infections, or other
Benign Rolandic Epilepsy minor neurological insults have been associated with
about 10% of cases.
BRE is presumed to be genetically determined, with a
family history of seizures documented in 2132% of cases.
The electrographic abnormality seen in BRE is believed to
Benign Rolandic Epilepsy be the inherited trait and follows a transmission method of
autosomal dominance. The gene associated with the elec-
J EFFREY B. T ITUS 1,2 , R EBECCA K ANIVE 1, trographic trait is located on chromosome 15q14, though
M ICHAEL M ORRISSEY 1 the expression of the gene appears to be complex and likely
1
St. Louis Childrens Hospital affected by a multitude of genetic, environmental, and
St. Louis, MO, USA pathological factors. As such, only about 10% of children
2
Washington University School of Medicine with the electrographic trait exhibit clinical seizures.
St. Louis, MO, USA
Neuropsychology and Psychology of BRE While neurodevelopmental problems have been veri-
fied in the active phase of BRE, it is not yet clear whether
BRE has been traditionally thought to be a benign syn- these problems remit after resolution of the EEG abnorm- B
drome, resulting in few if any neurodevelopmental pro- alities. The evidence thus far would suggest no long-term
blems. However, more recent research has suggested that impairment, but this needs to be verified through longi-
BRE can be associated with some neurocognitive problems, tudinal studies.
particularly during the active phase of the syndrome.
Among the available research, children with BRE, as a
group, have been consistently demonstrated to have av- Evaluation
erage intelligence. Nevertheless, more extensive neuro-
psychological assessment has revealed evidence of at least The diagnosis of BRE can only be made when charac-
mild neurocognitive difficulties in up to 78% of children teristic electrographic abnormalities and seizures are
with BRE. Almost 56% are estimated to have neurocog- present concurrently. Individual seizures are brief (up to
nitive difficulties in more than one area. The types of 12 min), simple partial seizures that are typically senso-
reported difficulties experienced by this population are rimotor in expression. Oropharyngolaryngeal symptoms
varied, including problems with motor coordination, are common and are associated with unilateral numbness
visualmotor integration, spatial processing, language and motor features that result in gurgling and grunting
processing, memory, attention, and executive function- noises. Hemifacial motor movements often occur and
ing. However, absence of control groups, questionable consist of sudden clonic contractions of the lower lip.
quality of some cognitive measures, and variability in Ipsilateral tonic deviation of the mouth is also a common
the definition of a deficit in cognitive functioning among accompanying feature. Ictal speech arrest can occur in
the available studies has made it difficult to make gen- about 40% of cases, regardless of the side of seizure
eralizations about the presence of neurocognitive pro- focus. The neurological examination is typically normal.
blems in BRE. Eighty percent of children with BRE experience sei-
A 2005 study out of Australia identified the presence of zures during sleep or the waking period. About 75% of
cognitive deficits in a relatively large sample of children seizures occur in non-REM sleep, shortly after initiating
with BRE. These included problems with verbal memory sleep or shortly before waking. Secondary generalizing
and visual memory, with about 10% of children falling seizures occur in about 20% of cases and typically occur
below two standard deviations on the verbal memory later in the sleep cycle. Partial seizures have a tendency to
index of the wide range assessment of memory and occur at the beginning or end of the sleep cycle.
learning (WRAML). In contrast, only 5% were below two The characteristic EEG pattern of BRE involves frequent
standard deviations on the visual memory index. However, to occasional unilateral or bilateral centrotemporal (rolan-
33% were below two standard deviations on the Rey Figure dic) sharp and slow waves that are superimposed on a
Delay. Deficits in phonological processing were also noted normal EEG background. Interictal activity can be present
and were believed to correspond with reading and spelling while the patient is awake and is markedly activated during
problems in the sample (Northcutt et al., 2005). NREM sleep. The rolandic sharp and slow waves have a
A more recent study in 2008 also confirmed the pres- voltage dipole that is negative in the rolandic and sylvian
ence of academic problems in children with BRE. Specifi- fissures and positive in the frontal head regions.
cally, children with BRE were found to have normal
intelligence levels but higher rates of reading, writing, and
mathematics disorders than children in a control group. Treatment
Attention problems were not identified. Of note, the study
reported that children with BRE who had a younger age of BRE is associated with spontaneous remission of seizures,
seizure onset were more likely to have a learning disability, regardless of whether the patient is treated with an anti-
and children with BRE who had prolonged interictal EEG epileptic medication. The decision to treat a child with
abnormalities in sleep were significantly more likely to have BRE is often based upon the impact of the seizures on
a learning disability. It was concluded that children with quality of life. Because the seizures typically occur infre-
BRE who have seizure onset prior to 8 years of age and have quently and most commonly during sleep, many clini-
EEG abnormalities that persist for more than 1 year are at cians chose not to initiate treatment. If the events are
greater risk for developing learning disabilities (Piccinelli frequent and potentially frightening to the child or par-
et al., 2008). ent, treatment with an antiepileptic medication may be
388 B Benign Senescent Forgetfulness
Current Knowledge
Cross References Changes in cognitive functioning are prevalent in aging
populations. It has become clear that there is most
Carbamazepine likely a continuum between normal and abnormal men-
Gabapentin tal function in those individuals who will ultimately
Partial Seizure (Simple) develop dementia. Recent studies focusing on the char-
Valproate acterization of the earliest stages of cognitive im-
pairment have identified an intermediate period
between the cognitive changes of normal aging and
dementia (see Petersen, Stevens, Ganguli, Tangalos,
References and Readings
Cummings, & DeKosky, 2001). This transitional zone
has been described using a variety of terms, including
Aicardi, J. (1998). Diseases of the nervous system in childhood. London:
Mac Keith. benign senescent forgetfulness (BSF), age-associated
Ferrie, C. D., Nordli, D. R., & Panayiotopoulos, C. P. (2008). Benign focal memory impairment (AAMI), age-associated cognitive
epilepsies of childhood. In J. M. Pellock, B. F. D. Bourgeois, & decline (ACCD), cognitive impairment-no dementia
W. E. Dodson (Eds.), Pediatric epilepsy: Diagnosis and therapy (CIND), and, most recently, mild cognitive impairment
(3rd ed., pp. 335350). New York: Demos.
(MCI). AAMI differs from BSF in that it includes specific
Loiseau, P. (2001). Idiopathic and benign partial epilepsies. In E. Wyllie
(Ed.), The treatment of epilepsy: Principles and practice (3rd ed., memory test performance criteria of 1 SD below young-
pp. 475484). Philadelphia: Lippincott Williams & Wilkins. adult levels (see Larrabee & Crook, 1994). AACD expands
Northcutt, E., Connolly, A. M., Berroya, A., Sabaz, M., McIntyre, J., the definition to decrements in performance in other
Christie, J., et al. (2005). The neuropsychological and language cognitive domains. MCI further refined the definition to
profile of children with benign rolandic epilepsy. Epilepsia, 46,
include the presence of memory complaints, normal activ-
924930.
Piccinelli, P., Borgatti, R., Aldini, A., Bindelli, D., Ferri, M., Perna, S., et al. ities of daily living, normal global cognitive functioning,
(2008). Academic performance in children with rolandic epilepsy. but abnormal memory performance compared to age- and
Developmental Medicine and Child Neurology, 50, 353356. education-matched controls (see Smith & Rush, 2006).
The clinical concept of MCI is important because it is a
significant risk factor for dementia. While conversion
rates vary widely, most researchers estimate that indivi-
duals with MCI develop dementia at a rate of 1015% per
Benign Senescent Forgetfulness year, in contrast to the rate of 12% per year for age-
matched controls.
R ICHARD F. K APLAN
UConn Health Center
Farmington, CT, USA
Cross References
Synonyms
Age Decrements
Age-associated memory impairment (AAMI); Late-life Mild Cognitive Impairment
forgetfulness Normal Aging
Benton, Arthur (19092006) B 389
Kral, V. A. (1962). Senescent forgetfulness: Benign and malignant. Journal He received his B.A. and M.A. degrees from Oberlin B
of the Canadian Medical Association, 86, 257260. College, and completed his Ph.D. at Columbia University
Larrabee G. J., & Crook, T. H. (1994). Estimated prevalence of age-
in 1935 under the mentorship of Carney Landis, followed
associated memory impairment derived from standardized tests of
memory function. International Psychgeriatrics, 6, 95104.
by clinical training at the Payne Whitney Psychiatric
Petersen, R. C., Stevens, J. C., Ganguli, M., Tangalos, E. G., Cummings, Clinic of New York Hospital.
J. L., & DeKosky, S. T. (2001). Practice parameter: Early detection of
dementia: Mild cognitive impairment (an evidence-based review).
Report of the Quality Standards Subcommittee of the American
Academy of Neurology. Neurology, 56, 11331142. Major Appointments
Smith, G., & Rush, B. K. (2006). Normal aging and mild cognitive
impairment. In D. K. Attix & K. A. Welch-Bohmer (Eds.), Geriatric Dr. Benton volunteered for military service in the US
neuropsychology assessment and intervention (pp. 2756). New York:
Guilford.
Navy in 1941, and was commissioned as a lieutenant
in the medical department. His active duty ended in
1945, but he continued to serve in the US Navy Re-
serve for many years, eventually retiring at the rank of
captain. In 1946, he accepted a position in the Psy-
chology Department at the University of Louisville. In
1948, he became a professor at the University of Iowa,
Benton, Arthur (19092006) where he would remain for over 50 years. He initially
was appointed Professor and Director of Graduate
S TEVEN W. A NDERSON
Training in Clinical Psychology, and then accepted a
University of Iowa Hospitals and Clinics
joint appointment in the departments of Psychology
Iowa City, Iowa, USA
and Neurology in 1958. He officially retired in 1978,
but remained active in research, teaching, and other
professional activities for another 20 years.
Landmark Clinical, Scientific, and
Professional Contributions
Arthur Benton was one of the pioneering figures in Major Honors and Awards
clinical neuropsychology. Beginning in the 1940s, he
introduced and applied novel and objective assess- President, American Orthopsychiatric Association,
ment techniques that provided a basis for fundamen- 1965
tal brain-behavior studies in aphasia, visuospatial President, International Neuropsychological Society,
abilities, hemispheric specialization, and other 1970
cognitive processes. Through the development of Secretary-General, Research Group on Aphasia of the
standardized tasks that stressed specific abilities, to- World Federation of Neurology, 19711978
gether with collection of data from neurological Distinguished Professional Contribution Award,
patients and normal comparison subjects, he was American Psychological Association, 1978
able to bring increased reliability and sensitivity to Outstanding Scientific Contribution Award, Interna-
the mental status exam, helping to establish neuro- tional Neuropsychological Society, 1981
psychology as a valuable clinical entity. He devel- Samuel Torrey Orton Award, Orton Dyslexia Society,
oped a number of neuropsychological tests that have 1982
been in wide use in clinical and research settings Distinguished Service and Outstanding Contribution
worldwide for several decades, including the Visual Award, American Board of Professional Psychology,
Retention Test, Judgment of Line Orientation, 1985
Three-Dimensional Block Construction, and Facial Distinguished Clinical Neuropsychologist Award, Na-
Recognition. He advocated a flexible approach to tional Academy of Neuropsychology, 1989
clinical assessment, with the content and scope of Gold Medal Award for Life Achievement in the Appli-
testing determined by the referral question, context, cation of Psychology, American Psychological Foun-
and patient abilities. dation, 1992
390 B Benton, Arthur (19092006)
supplement the popular auditory digit span test in neuro- brain disease for the adult norms, and 236 children, aged
psychological evaluations (Benton, 1945). It was first pub- 613 years, enrolled in public schools in Iowa and
lished in 1946. Memory-for-designs tasks had appeared Wisconsin, for the child and adolescent norms. B
earlier in the century as part of larger intelligence tests,
but included only a few designs and did not have separate
normative data. As an addition to the digit span test, the Clinical Uses
BVRT was intended to provide a broader assessment of
short-term memory, and its format was selected for its As it recruits a number of different cognitive functions, the
resistance to emotional influence, employment of differ- BVRT is sensitive to many forms of brain damage
ent sensorimotor components (graphomotor versus audi- and disease; however, its ability to discriminate among
tory-vocal), and minimal examiner-subject interaction diagnoses is low (for a review, see Mitrushina et al.,
(freedom from interpersonal demands). The initial ver- 2005). An individuals global performance, quantified as
sion included seven cards and two parallel forms. A 1955 either the number correct score or error score, provides the
revision increased the number of designs and alternate best indicator of impairment. According to the manual,
forms, and added norms for children aged 816 years. measures of specific error types, such as omissions, perse-
Later editions included a design copy administration verations, and distortions, are not by themselves diagnos-
and updated norms. The most recent revision was tic, but may raise hypotheses for further testing. For
authored by Abigail Benton Sivan in 1992 and is avail- example, a high number of perseverative errors suggests
able from its publisher, Pearson Assessments (http:// possible frontal lobe damage, particularly if supported by
pearsonassess.com). other test and behavioral data. Omission of peripheral
figures may raise suspicion of brain damage and is most
frequently associated with left hemispatial neglect as a
Psychometric Data result of damage to right parietal lobe regions. In contrast,
global performance has not been found to consistently
Information on reliability and validity may be found in distinguish between patients with unilateral right and left
the manual. Test-retest reliability is 0.85. Alternate form brain damage. Though the BVRT is sensitive to the visuo-
reliability ranges from 0.79 to 0.84. There is evidence that spatial disturbance that is often observed in patients with
Form C is slightly less difficult than Forms D and E under right hemisphere damage, studies have shown that indivi-
Administration A. Correlations between immediate (Ad- duals with unilateral left hemisphere damage can exhibit
ministration A) and delayed (Administration D) recall are similarly poor results on Administration A (Vakil, Blach-
positive and range from 0.40 to 0.83, depending on the stein, Sheleff, & Grossman, 1989), as well as on copy and
combination of forms used. Construct validity has been multiple-choice administrations (Arena & Gainotti,
demonstrated through moderate correlations (0.460.62) 1978). This indicates that memory for the BVRT designs,
of the BVRT with nonverbal subtests from the Wechsler many of which can be verbalized, is mediated by both
Adult Intelligence Scales, including Block Design, Digit hemispheres. However, the presence of a delay interval
Symbol, and Object Assembly. may differentially affect verbally and visually encoded
Child and adolescent normative data are included for material and therefore help to discriminate between left-
Administrations A and C. The normative data for each and right-sided brain damage. Participants with right
method of administration are based on different standar- hemisphere damage achieved a lower total correct score
dization samples, and sample characteristics are provided on Administration D than on Administration A, whereas
for Administrations A, B, and C. (Normative data for individuals with left hemisphere damage had the opposite
Administration D are not included in the manual.) The pattern of performance, benefitting from the delay. In
standardization sample for Administration A is based on a contrast, scores from healthy participants did not differ
compilation of three separate studies totaling over 1,300 between the two administrations (Vakil et al., 1989).
participants, ranging in age from 869 years. (See manual Both copy and memory administrations are highly
for discussion of participant inclusion criteria for each of sensitive to early dementia, and may also help to identify
these studies.) The standardization sample for Adminis- individuals who are at risk for developing dementia in the
tration B is based on 103 medical inpatients and out- future. In one such study, participants with six or more
patients, aged 1660 years, with no evidence or history errors on Administration A were nearly twice as likely to
of brain disease. The standardization samples for Admin- develop Alzheimers Disease 1015 years later, when com-
istration C are 200 medical patients with no history of pared to participants who had fewer errors (Kawas et al.,
394 B Benton Visual Retention Test
doses (Stewart, 2005; Barker, Greenwood, Jackson, & Montplaisir, J., Hawa, R., Moller, H., Morin, C., Fortin, M., Matte, J.,
Reinish, L., & Shapiro, C. M. (2003). Zopiclone and zaleplon vs
Crowe, 2004). For these and other reasons, medications
benzodiazepiones in the treatment of insomnia: Canadian consensus
in this class are now more commonly used on a short- statement. Human Psychopharmacology: Clinical and Experimental, B
term rather than a long-term basis (Iversen et al., 2009). 18, 2938.
Ntais, C., Pakos, E., Kyzas, P., & Ioannidis, J. P. (2005). Benzodiazepines
for alcohol withdrawal. Cochrane Database of Systematic Reviews,
Current Knowledge 20, CD005063.
Barbiturates
GABA Historical Background
has determined that a gradient of fall risk exists over the Blum, L., & Korner-Bitensky, N. (2008). Usefulness of the berg balance
scale in stroke rehabilitation: A systematic review. Physical Therapy,
entire scale. A retrospective study of community-dwelling
88(5), 559566.
persons with stroke demonstrated that changing from 3 to Chou, C. Y., Chien, C. W., Hsueh, I. P., Sheu, C. F., Wang, C. H., & Hsieh,
4 for the standing on one leg item had a sensitivity of C. L. (2006). Developing a short form of the berg balance scale for
0.90 and a specificity of 0.50 for predicting the history of people with stroke. Physical Therapy, 86(2), 195204.
multiple falls. Internet Stroke Center (2007). Berg balance scale. http://www.strokecen-
ter.org/Trials/scales/berg.html. Accessed May 19, 2010.
Mao, H., Hsueh, I., Tang, P., Sheu, C., & Hsieh, C. (2002). Analysis and
comparison of the psychometric properties of three balance mea-
Clinical Uses sures for stroke patients. Stroke, 33(4), 10221027.
Muir, S. W., Berg, K., Chesworth, B., & Speechley, M. (2008). Use of the
The BBS is available online (Internet Stroke Center, 2007). berg balance scale for predicting multiple falls in community-dwelling
Administration requires 1020 min, a chair, a step, a elderly people: A prospective study. Physical Therapy, 88(4), 449459.
ruler, and a stopwatch. Balance ability is sometimes Stevenson, T. J. (2001). Detecting change in patients with stroke using the
berg balance scale. Australian Journal of Physiotherapy, 47, 2938.
grossly categorized as good, fair, or poor for score ranges Tyson, S. F., & Connell, L. A. (2009). How to measure balance in clinical
from 56 to 41, 4021 and 200, respectively. As stated practice. A systematic review of the psychometrics and clinical utility
above, a gradient of fall risk exists over the entire scale. of measures of balance activity for neurological conditions. Clinical
BBS scores are used when prescribing mobility aids and Rehabilitation, 23(9), 824840.
treatment interventions, identifying safe and unsafe activ-
ities, and to measure treatment effect. When assessing the
treatment effect for individual patients with stroke, a
score change of 6 points has been shown to represent Bergers Waves
real change, beyond measurement error, with 90%
confidence. Alpha Rhythm
Although originally designed to screen older adults for
fall risk, the BBS has subsequently been validated for
persons with stroke, multiple sclerosis, and Parkinsons
Disease. Best Performance Method
G LEN E. G ETZ
Cross References Allegheny General Hospital
Pittsburgh, PA, USA
Balance Disorders
Multiple Sclerosis
Parkinsons Disease Synonyms
Reliability
Sensitivity Cognitive potential
Specificity
Stroke
Definition
compared. The Best Performance Method assumes that one and Rebif (INF-b1a). These medications are adminis-
performance level exists for each persons cognitive abilities. tered via injection, and each has been shown to reduce the
A notable discrepancy between a patients best and other frequency of MS relapses, reduce MRI evidence of brain B
performances is indicative of neuropsychological impair- lesions, and possibly reduce disability progression.
ment. The Best Performance Method also assumes that
performance should be consistent across all areas of func- Cross References
tioning. For example, very superior intellectual and other
abilities would be expected from a patient who has earned a Multiple Sclerosis
doctoral degree in engineering. The method has been criti-
cized by some who believe that there is a high likelihood of
References and Readings
overestimating premorbid ability.
National Clinical Advisory Board of the National Multiple Sclerosis
Cross References Society. (2007). Disease management consensus statement. Retrieved
February 15, 2010, from http://www.nationalmssociety.org/down-
load.aspx?id=8
Deficit Measurement Noseworthy, J., Miller, D., & Compston, A. (2006). Disease-modifying
Premorbid Estimate treatments in multiple sclerosis. In A. Compston, C. Confavreux,
Premorbid Functioning H. Lassman, I. McDonald, D. Miller, J. Noseworthy, et al. (Eds.),
Premorbid Intelligence McAlpines multiple sclerosis (4th ed., pp. 729802). Philadelphia:
Elsevier.
Zhang, J., Hutton, G., & Zang, Y. (2002). A comparison of the mechan-
References and Readings isms of action of interferon beta and glatiramer acetate in the
treatment of multiple sclerosis. Clinical Therapeutics, 24, 19982021.
Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). The rationale
of deficit measurement. In Neuropsychological Assessment (4th ed.,
pp. 8699). New York: Oxford University Press.
Mortensen, E. L., Gade, A., & Reinisch, J. M. (1991). Best Performance Betaseron
Method in clinical neuropsychology. Journal of Clinical and
Experimental Neuropsychology, 13, 361371. Beta-Interferons
Definition
Definition
Interferon b is a disease-modifying drug currently indi-
cated for treatment of relapsing forms of multiple sclero- Beyond a reasonable doubt is the standard of proof
sis. Its mechanism of action is complex and is presumed required in most criminal cases within an adversarial
to inhibit immune system T-cell activation and migration system. Generally, the prosecution bears the burden of
into the central nervous system as well as modulate the proof and is required to prove their version of events to
action of some pro-inflammatory proteins (cytokines). this standard. This means that the proposition being
There are three FDA approved beta interferons available presented by the prosecution must be proven to the
in the US Avonex (INF-b1a), Betaseron (INF-b1b), extent that there is no reasonable doubt in the mind
400 B BG-II
Cross References
Definition
Burden of Proof
Clear and Convincing Evidence Faust, Ziskin, and Hiers (1991) and Wedding and Faust
Preponderance of the Evidence (1989) explain chief forms of bias related to clinical
Bicycle Drawing Test B 401
constructional ability. One study compared the Bicycle References and Readings
Drawing test to the BenderGestalt test (Greenberg,
Rodriguez & Sesta, 1994), and found bicycle drawing to Angenent, H. L. (1971). The development of thought structure among
be more sensitive to brain dysfunction in children with normal and mentally retarded children. Nederlands Tijdschrift voor
de Psychologie en haar Grensgebieden, 26(4), 215232.
visualspatial problems than the BenderGestalt test.
Greenberg, G. D., Rodriguez, N. M., & Sesta, J. J. (1994). Revised scoring,
Interestingly, improvements in bicycle drawing perfor- reliability, and validity investigations of Piagets Bicycle Drawing
mance have been described in studies of patients with Test. Assessment, 1(1), 89101.
Parkinsons disease, multiple sclerosis, and Tourettes syn- Hecaen, H., & Assal, G. (1970). A comparison of constructive deficits
drome (Sandyk, 1994; Sandyk, 1997a, 1997b), with rever- following right and left hemispheric lesions. Neuropsychologia, 8(3),
289303.
sal of spatial orientation in Parkinsons patients when they
Piaget, J. (1955). Perceptual and cognitive (or operational) structures in
received electromagnetic pulses to their brain (Sandyk, the development of the concept of space in the child. Acta Psycholo-
1998). The quality and complexity of the drawing pro- gica, 11, 4146.
duced by children and adults has also been linked to their Poppelreuter, W. (1990). Disturbances of lower and higher visual capacities
intellectual ability (Sharma, 1972). caused by occipital damage: with special reference to the psychopatho-
logical, pedagogical, industrial, and social implications. Oxford/
New York: Clarendon Press/Oxford University Press.
Sandyk, R. (1994). Reversal of a visuoconstructional deficit in
Parkinsons disease by application of external magnetic fields: A
Current Clinical Use report of five cases. International Journal of Neuroscience, 75(34),
213228.
Formal scoring systems exist for the Bicycle Drawing test Sandyk, R. (1997a). Progressive cognitive improvement in multiple scle-
(Greenberg et al., 1994) providing a means for deriving rosis from treatment with electromagnetic fields. International Jour-
quantitative results when using this test. Studies compar- nal of Neuroscience, 89(12), 3951.
Sandyk, R. (1997b). Reversal of a visuoconstructional disorder by weak
ing the drawings of amateurs with artists have also shown
electromagnetic fields in a child with Tourettes syndrome. Interna-
that observational, experimental, and neuropsychological tional Journal of Neuroscience, 90(34), 159167.
methods for scoring drawings can provide systemic differ- Sandyk, R. (1998). Reversal of the bicycle drawing direction in Parkin-
ences in cognitive skills among individuals (van Sommers, sons disease by AC pulsed electromagnetic fields. International Jour-
Lange-Kuttner & Thomas, 1995). Yet, most clinicians nal of Neuroscience, 95(34), 255269.
Sharma, T. R. (1972). Measuring intelligence through bicycle drawings.
currently use the Bicycle Drawing test in conjunction with
Indian Educational Review, 7(1), 130.
other constructional tests, including coping or freehand van Sommers, P. (1995). Observational, experimental and neu-
drawing tasks (e.g., cube, house), and examine results ropsychological studies of drawing. In C. Lange-Kuttner, &
qualitatively for gross spatial distortion, or omissions. G. V. Thomas (Eds.) Drawing and looking: Theoretical approaches
Other tests, such as copying of the Rey Complex Figure to pictorial representation in children (pp. 4461). Hertfordshire:
Harvester Wheatsheaf.
are now more widely used for visualmotor assessment.
Veiders, E. (1934). Analyse der Fahigkeit zum raumlichen Denken. Anal-
Yet, the bicycle drawing test is an easy-to-administer task ysis of the ability for spatial thinking. Psychotechnisches Zeitschrift,
that can yield valuable information about the visual- 9, 5260.
spatial and constructional abilities of patients. It can also
detect hemi-neglect syndrome, as some patients may omit
one side of the bicycle. IT can yield information about
additional information on intellectual development when
used in the assessment of children. Generally, it should Bilateral Acoustic
not be used as a stand-alone test, but should rather be Neurofibromatosis
used in conjunction with other tests of visual construc-
tional functioning. Neurofibromatosis Type 2
Cross References
stabilize during the post-acute phase (up to 5 months around. Translation without comprehension is a preserved
post-onset), language impairment becomes more directly ability to translate without an ability to comprehend the
related to site of lesion and damage to specific language meaning of either translation. Finally, spontaneous trans-
representations. lation is the involuntarily production of translations that
cannot be inhibited.
The heterogeneity of the bilingual population makes it
Clinical Variants and Recovery Patterns difficult to link language profiles and lesion site/size with
specific impairment and recovery patterns in individuals
Bilingual individuals with aphasia show great variability in with bilingual aphasia. However, a number of linguistic
impairment and recovery patterns. Paradis (2001), in factors in impairment and recovery patterns have been
a review of 132 cases of bilingual aphasia, found that identified. Naming and translation of cognate words
61% showed parallel recovery of their two languages, (that share sound and meaning in the two languages,
18% showed differential recovery of their two languages, e.g., lamp-lampara) is typically less impaired in bilingual
7% showed blended recovery, and 5% showed selective individuals with aphasia than naming and translation of
recovery (for similar distributions on a sample of 20 noncognate words (key llave, Goral, Levy, & Obler, 2006;
ItalianFriulian patients, see Fabbro, 2001). Reports of Kohnert, 2004; Roberts & Deslauriers, 1999). In general,
atypical and pragmatically inappropriate language swit- aspects of bilinguals languages that are more shared
ching behaviors in bilingual individuals with aphasia have are also more resistant to impairment (e.g., Kiran &
also appeared (Munoz, Marquardt, & Copeland, 1999). Tuctenhagen, 2005). Linguistic features that differ be-
Parallel impairment refers to aphasia of the same type tween languages (e.g., different grammatical systems)
and severity in both languages. The two languages are may result in differences in how symptoms of aphasia
impaired and recover simultaneously (relative to premor- are expressed even if the same underlying deficit exists.
bid language proficiency). Differential impairment refers For example, a morphologically rich language can theo-
to aphasia of the same type in both languages (e.g., fluent retically undergo greater morphological breakdown, and
vs. nonfluent) with crosslinguistic differences in severity morphological deficits may look more severe. Therefore,
levels. In contrast, differential aphasia refers to different cross-linguistic differences in the symptoms and recovery
aphasia symptoms in each language. Differential recovery patterns of bilingual aphasia frequently occur at points
refers to one language recovering better than the other where the two linguistic systems diverge.
(relative to premorbid levels). Blended impairment Another explanation for divergent recovery patterns
refers to the inappropriate combination of two or more in bilingual aphasia is the Cue Strength hypothesis (e.g.,
languages (e.g., the patient may lose the ability to discrim- Wulfeck, Bates, & Capasso, 1991). According to this hy-
inate between languages). Pathological mixing, character- pothesis, the linguistic importance of a grammatical
ized by inadvertent and uncontrolled language switches, is structure or the contribution it makes to the linguistic
typically associated with blended impairment. In contrast, message may account for crosslinguistic differences in
pathological fixation is an inability to switch languages. syntactic deficits, with more important cues being more
Antagonistic recovery refers to a pattern where one lan- likely to be preserved. For example, English-speaking
guage recovers first, and starts regressing when the other individuals with aphasia have been found to be more
language starts to recover. Alternating antagonism refers to sensitive to word-order errors during grammaticality
repetition of the antagonistic pattern, with the two lan- judgments while Italian-speaking individuals with apha-
guages alternating in availability (cycles may range from sia were more sensitive to morphological errors. Similarly,
hours to months). Selective impairment refers to aphasia differences in reading impairments have been found
in only one language, while the other language remains across languages with different orthographies. Readers
intact (relative to premorbid language proficiency). In are referred to a detailed review of current knowledge
addition, a variety of deficits have been identified in about bilingual aphasia by Lorenzen and Murray (2008).
bilingual aphasics ability to translate from one of their
languages to the other. An inability to translate is reflected
in bilinguals inability to translate either forward (from Assessment
their native language to their second language) or back-
ward (from their second language to their native lan- An important part of assessment in bilingual aphasia
guage). Paradoxical translation is an ability to translate consists of establishing premorbid proficiency levels as
from one language to the other, but not the other way accurately as possible and determining the nature and
Bilingual Aphasia B 405
extent of impairment in each language relative to these than the ability to retrieve known information. A clinician
premorbid proficiency levels. Self-reports, questionnaires may explain a new grammatical rule and test the clients
about the history of language use (e.g., code-switching), ability to generalize it. If the client generalizes the rule B
reports from family members or friends, and written or easily, then weak linguistic performance is likely due to
recorded samples of patients language abilities are typi- the influence of the non-target language or low profi-
cally used to establish premorbid proficiency levels. Once ciency in the target language, rather than aphasia.
premorbid proficiency levels have been established, it is
important to assess both of the patients languages in
Treatment
order to gauge their full linguistic capacity and impair-
ments across languages.
Assessment of both languages, together with the social
There are currently no assessment measures for bilin-
communication needs of the client, will inform choice
gual aphasia that meet all standards for measurement
of therapy language and specific therapy goals. A primary
validity. The bilingual aphasia test (BAT, Paradis, 1987)
goal in the treatment of bilingual individuals with aphasia
is the most comprehensive tool available and provides
is to maximally benefit both languages even if treatment
systematic ways to assess aphasia in many languages.
occurs in only one language.
Tasks on the BAT are equivalent in linguistic complexity
Treatment may be conducted to target both languages
across languages, and cover assessment of linguistic levels
directly. During bilingual treatment, language-switching
(phonological, lexical-semantic, morphological, syntac-
may be encouraged as a compensatory strategy to allow
tic), linguistic skills (comprehension, formulation, repeti-
the client to use his/her full linguistic capacity. Translation
tion, judgment, lexical access), and linguistic units (words,
may be used in a similar manner to aid lexical access. For
sentences, paragraphs). The BAT assumes that the test-
example, switch-back through translation (SBT) treat-
taker has pre-morbid proficiency in each language that is
ment (Ansaldo & Marcotte, 2007) is a procedure where
equivalent to at least 400 language-learning hours. The test
the client is cued to translate the word back into the other
is administered in each language on different days. Where
language whenever an inadvertent switch occurs.
it is not feasible to obtain all language versions of the BAT,
If resources are only available to treat in English, the
its principles may be followed during assessment.
speechlanguage pathologist may work to identify outside
Additional tasks that may be useful in examining
resources in helping to rehabilitate the clients other
bilingual individuals language impairments include the
languages. Such linguistic resources may include
type-token ratio in each language based on comparable
language-specific community groups, or guidance of fam-
language samples, number of verbs and grammatical
ily members. Crosslinguistic generalization is most likely
clauses per utterance, semantic acceptability, confabulation,
to occur when shared representations are targeted for
and total number of words or utterances within a set time
treatment, crosslinguistic associative links are used, or
window (fluency measures). Preservation of links between
similar cognitive processes are a focus of intervention
languages may be assessed by testing participants transla-
(e.g., reading in alphabetic languages). Current evidence
tion abilities from the native language to the second
suggests that treatment in the persons weaker language
language and vice versa. As part of cognitive assessment,
may generalize to his/her stronger language, especially
language switching behaviors may be examined. Patho-
when treatment targets are similar across languages
logical mixing can be distinguished from nonpathological
(Edmonds & Kiran, 2004). However, generalization from
mixing: For example, in nonpathological mixing, subject
the stronger to the weaker language is less likely. General-
and verb should be in the same language, and switches
ization across languages has been shown when cognate
should not occur on prepositions.
words are used in treatment (Kohnert, 2004), semantic
If, due to limitations in resources, assessment and
features are treated (Edmonds & Kiran, 2004, 2006), and
treatment are done only in English, the clinician may
general cognitive function is treated (Kohnert, 2004).
obtain information on the structure of the clients other
However, cross-linguistic generalization does not always
language in order to identify crosslinguistic influences in
occur (e.g., Galvez & Hinckley, 2003).
the clients English output. This may allow the clinician to
distinguish low premorbid proficiency in English from a
disorder. The dynamic assessment approach provides an Future Directions
alternative method for examining deficits in situations of
low premorbid language proficiency. Dynamic assessment In 2000, 18% of the population older than 5 years of age
focuses on the ability to learn new information, rather (47 million people) spoke a language other than English
406 B Bilingual Aphasia
at home, up from 14% in 1990 and 11% in 1980 (U.S. Abutalebi, J., & Green, D. (2007). Bilingual language production: The
neurocognition of language representation and control. Journal of
Census, 2000). As the bilingual population grows, with
Neurolinguistics, 20, 242275.
special growth in older adults, the need for accommoda- Ansaldo, A. I., & Marcotte, K. (2007). Language switching and mixing in
tion of bilingual individuals with aphasia will increase. the context of bilingual aphasia. In J. G. Centeno, L. K. Obler, & R. T.
Among Mexican Americans, stroke incidence is slightly Anderson (Eds.), Studying communication disorders in Spanish speak-
higher (1.63%) than in non-Latino white peers (1.36%), ers: Theoretical, research, and clinical aspects. Clevedon, UK: Multi-
lingual Matters.
and transient ischemic attacks are more frequent at youn-
Bialystok, E., Craik, F. I. M., Klein, R., & Viswanathan, M. (2004). Bilin-
ger ages (Lorenzen & Murray, 2008). The US Department gualism, aging, and cognitive control: Evidence from the Simon task.
of Health and Human Services found that Latino indivi- Psychology and Aging, 19, 290303.
duals were 33% less likely to receive necessary health- Bychowsky, Z. (1919). Concerning the restitution of language loss
care services, compared to non-Latino white peers subsequent to a cranial gunshot wound in a polyglot aphasic. In
M. Paradis (Ed.), Readings on aphasia in bilinguals and polyglots
(Lorenzen & Murray, 2008). With these changes in popu-
(pp. 130144). Montreal, QC: Didier.
lation dynamics, systematic evidence-based research on Chee, M. W., Caplan D., Soon, C. S., Sriram, N., Tan, E. W., Thiel, T., et al.
the efficacy of various treatment approaches for bilingual (1999). Processing of visually presented sentences in Mandarin and
aphasia has become increasingly necessary. English studied with fMRI. Neuron, 23, 127137.
One particularly promising avenue of research in Edmonds, L., & Kiran, S. (2004). Confrontation naming and semantic
relatedness judgments in Spanish/English bilinguals. Aphasiology, 18,
bilingual aphasia is in the area of cognitive control. Lan-
567579.
guage production requires cognitive control skills. These Edmonds, L., & Kiran, S. (2006). Effect of semantic naming treatment on
skills may be honed with bilingual experience, as bilin- crosslinguistic generalization in bilingual aphasia. Journal of Speech,
guals must suppress one language in favor of another Language, and Hearing Research, 49(4), 729748.
language every time they speak. Balanced bilinguals have Emmorey, K., Mehta, S., & Grabowski, T. (2007). The neural correlates of
sign versus word production. Neuroimage, 36, 202208.
been shown to have cognitive advantages over monolin-
Fabbro, F. (2001). The bilingual brain: Bilingual aphasia. Brain and
guals on nonlinguistic executive function tasks, especially Language, 79, 201210.
as they age (Bialystok, Craik, Klein, & Viswanathan, 2004; Galvez, A., & Hinckley, J. J. (2003). Transfer patterns of naming
Kave, Eyal, Shorek, & Cohen-Mansfield, 2008; Zied et al., treatment in a case of bilingual aphasia. Brain and Language, 87,
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Gesner, J. A. P. (1770). Sammlung von Beobachtungen aus der
system in bilinguals ability to appropriately maintain and
Arzneygelehrtheit und Naturkunde (pp. 17691776). Nordlingen:
switch between languages, it is important to examine the CG Beck.
integrity of the cognitive control system in bilingual indi- Golestani, N., Alario, F. X., Meriaux, S., Le Bihan, D., Dehaene, S., &
viduals with aphasia. It is also possible that cognitive- Pallier, C. (2006). Syntax production in bilinguals. Neuropsycholo-
control advantages may support language recovery and gia, 44(7), 10291040.
Goral, M., Levy, E. S., & Obler, L. K. (2006). Neurolinguistic aspects of
responsiveness to treatment in bilingual patients with
bilingualism. International Journal of Bilingualism, 6, 411440.
aphasia. Hernandez, A. E., Martinez, A., & Kohnert, K. (2000). In search of the
language switch: An fMRI study of picture naming in Spanish-
English bilinguals. Brain and Language, 73, 421431.
Indefrey, P. (2006). A meta-analysis of hemodynamic studies on first
Cross References and second language processing: Which suggested differences
can we trust and what do they mean? In M. Gullberg & P. Indefrey
(Eds.), The cognitive neuroscience of second language acquisition
Aphasia
(pp. 279304). Malden, MA: Blackwell.
Aphasia Tests Kave, G., Eyal, N., Shorek, A., & Cohen-Mansfield, J. (2008). Multilin-
Multilingual Aphasia Examination gualism and cognitive state in the oldest old. Psychology and Aging, 23,
SpeechLanguage Pathology 7078.
SpeechLanguage Therapy Kiran, S., & Tuctenhagen, J. (2005). Imageability effects in normal bilin-
gual adults and in aphasia: Evidence from naming to definition and
semantic priming tasks. Aphasiology, 19, 315325.
Klein, D., Milner, B., Zatorre, R. J., Zhao, V., & Nikelski, J. (1999).
Cerebral organization in bilinguals: A PET study of ChineseEnglish
References and Readings verb generation. NeuroReport, 10, 28412846.
Kohnert, K. (2004). Cognitive and cognate-based treatments for bilingual
Abutalebi, J., Brambati, S. M., Annoni, J. M., Moro, A., Cappa S. F., & aphasia: A case study. Brain and Language, 91, 294302.
Perani, D. (2007). The neural cost of the auditory perception of Lordat, J. (1843). Lecons tirees du cours de physiologie de lannee scolaire
language switches: An event-related fMRI study in bilinguals. Jour- 18421843. Journal de la Societe de medecine pratique de Montpellier,
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Binswangers Disease B 407
Current Knowledge healthy diet, exercising, and not smoking or drinking too
much alcohol. Controlling vascular risk factors can help
Neuropathology improve cognition and may even help prevent the devel-
opment of dementia (Roman, 2005).
Gross pathology of brain tissue affected by BD is charac-
terized by gyral atrophy and widening of the sulci result-
ing from the loss of cerebral white matter. Lateral Prognosis
ventricles are also typically enlarged. Lacunar infarctions
can be found in the white matter, pons, and basal ganglia BD is a progressive disease and there is currently no cure.
as well as occasionally in the cerebellum. Microscopic The course of BD can be variable and deterioration can
pathology of BD is marked by diffuse and patchy white occur suddenly or gradually and then progress in a step-
matter demyelination with areas of reactive gliosis and wise manner (Santamaria Ortiz and Knight, 1994).
decreased nerve fibers. The small arteries of the white
matter also show fibrous thickening, which is associated
with hypertension and cardiovascular disease. BD is be- Cross References
lieved to result from ischemic injury of frontal lobe white
matter and is distinct from the lacunar state of small vessel Leukoaraiosis
disease that also causes dementia but results from infarc-
tions in the basal ganglia and internal capsule.
References and Readings
Clinical Symptoms Babikian, V., & Ropper, A. H. (1987). Binswangers disease: a review.
Stroke, 18(1), 212.
BD typically has a slow, insidious onset that eventually Caplan, L. R. (1995). Binswangers disease revisited. Neurology, 45(4),
626633.
manifests in cognitive and motor dysfunctions related
Hachinski, V., Iadecola, C., Petersen, R. C., Breteler M. M., Nyenhuis,
to the disruption of subcortical neural circuits. Specifical- D. L., Black S. E. et al. (2006). National Institute of Neurological
ly, patients exhibit executive dysfunction (e.g., impaired Disorders and Stroke-Canadian Stroke Network vascular cognitive
initiation, inhibition, monitoring of goal-directed behav- impairment harmonization standards. Stroke, 37(9), 22202241.
ior, and verbal fluency), psychomotor slowing, inatten- Lezak, M. D., Howieson, D. B., & Loring, D. (2004). Neuropsychological
assessment (4th ed., p. 1016). New York: Oxford University Press.
tion, and short-term memory loss with poor retrieval
Olsen, C. G., & Clasen, M. E. (1998). Senile dementia of the Binswangers
but intact recognition (Roman, 2003). Other symptoms type. American Family Physician, 58(9), 20682074.
include changes in speech, an unsteady gait, postural Pantoni, L., & Garcia, J. H. (1995). The significance of cerebral white
instability, changes in personality or mood (including matter abnormalities 100 years after Binswangers report. A review.
apathy, irritability, and depression), as well as urinary Stroke, 26(7), 12931301.
Roman, G. C. (2003). Neurological aspects of vascular dementia:
incontinence (Babikian and Ropper, 1987; Caplan, 1995;
Basic concepts, diagnosis, and management, In P. A. Lichtenberg,
Lezak, Howieson, & Loring, 2004; Roman, 2003). D. L. Murman, & A. M. Mellow (Eds.), Handbook of dementia -
psychological, neurological, and psychiatric perspectives (pp. 149171).
Hoboken, NJ: John Wiley & Sons, Inc.
Treatment Roman, G. C. (2005). Vascular dementia prevention: a risk factor analy-
sis. Cerebrovascular Diseases, 20(Suppl. 2), 91100.
Santamaria Ortiz, J., & Knight, P. V. (1994). Review: Binswangers disease,
Treatment of BD is often targeted at specific symptoms. leukoaraiosis and dementia. Age Ageing, 23(1), 7581.
For example, medications such as donepezil and meman- Sink, K. M., Holden, K. F., & Yaffe, K. (2005). Pharmacological treatment
tine may be used to treat the cognitive symptoms asso- of neuropsychiatric symptoms of dementia: a review of the evidence.
ciated with BD. Individuals with depression may be Jama, 293(5), 596608.
treated with antidepressant medications (e.g., serotonin-
specific reuptake inhibitors (SSRIs) such as sertraline or
citalopram) and individuals with agitation or disruptive
behavior can be treated with atypical antipsychotic med-
ications such as risperidone or olanzapine (Sink, Holden, Biological Cycles
& Yaffe, 2005). Other treatment interventions are often
focused on reducing cardiovascular risk factors by eating a Circadian Rhythms
Biopsy B 409
Cross References
Definition
Acceleration Injury
An inclusive term to explore and describe the mechan- Deceleration Injury
ical and physical factors that result in traumatic brain Diffuse Axonal Injury
injury. Rotational Acceleration
Traumatic Brain Injury
Current Knowledge
References and Readings
Biomechanical injuries typically occur without the direct
impact of an outside object on the skull or brain, but Bayly, P. V., Cohen, T. S., Leister, E. P., Ajo, D., Leuthardt, E. C., Genin,
rather in the context of accelerationdeceleration injuries. G. M. (2005). Acceleration-induced deformation of the human
brain. Journal of Neurotrauma, 22, 845856.
High-speed situations such as motor vehicle accidents
Ommaya, A. K., Goldsmith, W., & Thibault, L. (2002). Biomechanics and
and sports provide mediums for these inertia-based inju- neuropathology of adult and pediatric head injury. British Journal of
ries. The structure of the skull includes sinuses and bony Neurosurgery, 16, 220242.
protective regions. Underlying brain tissue is held in sus-
pension underneath the skull not only by the meninges,
but also by a cushion of cerebral spinal fluid. Different
inertial forces such as linear acceleration, rotation of the
head, or massive vibration or air pressure changes in Biopsy
the environment can result in a wide range of potential
damage to these underlying substances. These disruptions B RAM G OLDSTEIN
may include skull fracture, linear acceleration injury, Hoag Hospital Cancer Center
rotational injury, and the effects of vibration of the skull Newport Beach, CA, USA
and brain against one another.
Superficial or deep lesions may result in parenchy-
mal injury depending on the type of mechanical force Definition
that occurred at the time of head trauma. Linear accel-
eration injuries are most often associated with superfi- A biopsy is a medical examination entailing the removal
cial brain injuries such as cerebral contusions, while of cellular tissue via a needle or surgical resection.
rotational injuries are most often associated with dis- In particular, an incisional or core biopsy involves a
ruptions to deep white matter tracts and projections, select sample of tissue, whereas an excisional biopsy
and centrally located brain structures and neural net- necessitates a larger specimen. The biopsy results are
works. Consequently, rotational injuries may be more typically evaluated microscopically by a pathologist, who
severe with regard to effects on cognition, motor skills, determines if a lesions pathology is benign or malignant.
and functional status. Concussion with or without loss Although histological confirmation of tumor diagnosis
of consciousness is also a consequence of biomechanical can be achieved, a biopsy sampling error can result if
forces and the subsequent effects on underlying brain the specific tissue section does not contain the most
tissue. representative cellular features. When the biopsy is
The biomechanics of injury differentially affect initial abnormal, the cell structure may be unusual and
and long-term recovery from acquired brain injury. Under- indicative of malignancy. However, further pathological
standing these different mechanical forces may help one to examination is often required to make a definitive
improve understanding of injury severity. Increased diagnosis.
410 B Biorhythms
Explosions may cause injury through four Typically, an individual is exposed to more than one
mechanisms: mechanism, making the contributions of one particular
mechanism difficult to separate from others. B
1. Primary Blast Injury
Approximately 60% of explosion-related injuries in
A primary injury is one sustained from exposure to combat lead to an acquired brain injury. As is the case
the shock/pressure waves initiated by the explosion. with other etiologies, the majority of traumatic brain
When explosive munitions are detonated, a shockwave injuries resulting from explosions are classified as mild
approaching a speed of 8000 meters/second is generated. in nature. Similar to civilian settings, mild traumatic
The waves generated from a blast can cause life-threaten- brain injury (mTBI) has not been consistently defined in
ing injuries when they strike an individual directly, or if the literature, which is a substantial limitation in making
they reflect off nearby surfaces and then come into con- meaningful comparisons between studies. Despite this,
tact with the person. The force generated is of such a definitions such as that proposed by the American Con-
magnitude that it often results in an instant fatality or in gress of Rehabilitation Medicine are coming into wider
trauma to multiple body systems. Body organs that are acceptance, and have largely been adopted by the military.
relatively solid or fluid-filled tend to sustain a lesser The ACRM definition of mTBI includes at least one
degree of injury than those that are gas-filled or have a symptom such as less than half an hour of loss of con-
gas-fluid interface, such as the tympanic membrane, sciousness, less than 24 hours of post-traumatic amnesia,
lung, and colon. Although not fully understood, research any retrograde or anterograde amnesia, mental status
suggests that the explosion may injure the central ner- changes immediately after injury, and transient/perma-
vous system directly, as in a concussion, but may also nent neurological impairments. The literature cautions
indirectly affect the brain. The latter case may occur when that a mild TBI from an explosion may not be equivalent
peripheral somatic areas are impacted by the blast, to mild TBI from other etiologies (e.g., motor vehicle
setting in motion events that ultimately impact the cen- accidents, sports injuries), as the former may affect the
tral nervous system (CNS), such as chemical/metabolic brain more diffusely and tend to involve trauma to other
cascades, physical sequelae (i.e., cerebral infarction organ systems, thereby complicating the patients clinical
caused by an air embolism), and/or kinetic events (e.g., presentation and recovery. However, studies have indi-
transfer of shock/pressure wave energy from the body, up cated that factors such as loss of consciousness and cog-
the vasculature into brain tissue). It has been postulated nitive deficits do not appear to significantly differ between
that the severity and number of the patients physical blast victims and those injured by other means, tentatively
wounds from primary blasts often overshadow symp- suggesting that knowledge gleaned from studying these
toms of traumatic brain injury, delaying diagnosis and other etiologies has at least some applicability to blast
treatment for these injuries. injury survivors.
Cognitive (e.g., memory, attention), somatic (e.g.,
2. Secondary Blast Injury
dizziness, headache, sleep initiation/maintenance difficul-
Secondary injuries occur when shrapnel, debris, or other ties), and emotional (e.g., irritability, nervousness) symp-
objects are caught up by the blast and propelled against/ toms are commonly seen initially. In civilian samples,
into an individual. Many of these injuries are therefore mild TBI symptoms appear to resolve quickly, with most
penetrating in nature. individuals showing rapid recovery within the first week.
However, over one third may continue to experience
3. Tertiary Blast Injury
significant post-concussion symptoms, and as many as
This type of injury is sustained when the person is caught 15% may continue to experience persistent symptoms
up and propelled by the blast wind that follows the initial after 12 months (Persistent Post-Concussion Syn-
shock wave, and is thrown against objects, structures, the drome). Unfortunately, post-concussion symptoms
ground, other individuals, etc., often resulting in blunt have not been consistently defined, and many point out
force wounds. that the constellation of symptoms present are vague and
lack the specificity needed to identify them as constituting
4. Quaternary Blast Injury
a true syndrome.
Quaternary blast injuries are those that arise from the This debate over whether the more chronic symptom
aftereffects of an explosion. Examples include being ex- constellation after mild TBI reflects a true neurological
posed to radiation, fire, chemicals, dust, or toxic sub- syndrome has particular relevance in blast injury, as the
stances that were precipitated by the explosive event. brief history of shell shock above illustrates. Those
412 B Blast Injury
favoring a neurological position cite animal models in helpful in identifying post-concussive symptoms and clar-
which direct and indirect exposure to primary blasts ifying the diagnostic picture, enabling education and
causes structural, chemical, and electrophysiological treatment efforts to proceed more quickly.
changes in the brain. Those weighing psychological fac- The profile of cognitive weaknesses resulting from
tors more heavily in terms of causation point to the mTBI blast injuries is quite variable. Slowed attention and in-
literature that indicates non-neurological factors, such as formation processing speed, motor slowness, and execu-
premorbid psychological coping resources and external tive and memory difficulties are common. As such,
stressors, appear to influence the development of concus- neuropsychological assessment should ideally be broad-
sion symptoms in some individuals. The fact that mTBI based, with all major cognitive domains sampled. As is the
symptoms overlap considerably with anxiety disorders case in sports concussions, the symptom picture for many
such as post-traumatic stress disorder is particularly note- individuals may evolve relatively rapidly, arguing for use
worthy, given the high incidence of PTSD in military of tests that have alternate forms (e.g., California Verbal
personnel who have experienced combat: Gaylord, Coo- Learning Test 2nd Edition, Hopkins Verbal Learning
per, Mercade, Kennedy, Yoder, and Holcomb (2008) Test-Revised). Tracking somatic symptoms (e.g., Post-
found that nearly 20% of military persons who incurred Concussion Scale-Revised) over time may also have utili-
blast and burn injuries were appropriate for both mild ty. Because of the high degree of PTSD and other affective
brain injury and PTSD diagnoses. Hoge, McGurk, Thom- disorders, a thorough psychological evaluation should
as, Cox, Engel, and Castro (2008) reported that approxi- always be performed (e.g., MMPI-2, PAI) and observation
mately 15% of soldiers surveyed after being returned for these symptoms should be an ongoing effort, not
home might meet criteria for both an mTBI and PTSD; simply one restricted to an initial evaluation. Given the
these servicemen and women were more likely to have high degree of lowered effort present in civilian mTBI
been exposed to a blast injury. In addition, their survey cases, effort testing (e.g., Test of Memory Malingering) is
indicated that the presence of affective distress might be often advocated, with the caveat that poor performances
the major factor in maintaining chronic health difficul- on these tests should not automatically be interpreted as
ties, including mTBI symptomatology. A compromise signs of intentional feigning of symptoms, but as a sign
position of sorts posits mTBI symptoms are likely neuro- that further investigation is warranted as to the cause of
logical in origin but are subsequently maintained by emo- the lowered effort.
tional/psychological factors, and that the presence of After a thorough diagnostic assessment has been per-
PTSD and similar affective disturbances can complicate formed, treatments generally have proceeded along the
healing from and coping with mTBI and vice versa. The lines advocated for mild brain injuries attributed to
fact that PTSD symptoms can arise long after the actual non-blast causes. Specifically, reassurance and education
trauma indicates that these emotional disturbances may regarding the nature and general recovery of cognitive and
influence a person at virtually any point in his or her brain other symptoms is delivered, and problematic symptoms
injury recovery. are treated with medication (e.g., analgesics for pain,
Treatment approaches of blast injuries have paralleled soporific medication for insomnia, antidepressants for
treatment efforts in mild TBI. Treatment begins with a affective symptoms) and other strategies (e.g., relaxation
thorough diagnostic assessment. The Armed Services have strategies for anxiety symptoms, psychotherapy for PTSD
made significant improvements in their endeavors to symptoms). Aggressive, evidence-based cognitive rehabil-
standardize comprehensive screening and interviewing itation efforts have been advocated and research programs
methods to identify service personnel who may have in this area have been proposed.
experienced an acquired brain injury, beginning on the
battlefield and continuing throughout the militarys med-
ical system. Efforts have also been made to carefully screen
the wounded for other symptoms (e.g., tinnitus) that Future Directions
place them at higher risk for having sustained a TBI in a
blast. Any assessment should include a thorough medical The research literature in blast injury is still in an early
evaluation and comprehensive interview that elicits his- stage of development. The following is a partial list of
torical information about past psychological treatment/ necessary future research efforts: clarifying definitions of
coping, substance use, and combat exposure. Neuropsy- mTBI and post-concussive symptom constellations; sepa-
chological evaluation as early as possible would also be ration of the effect of different blast mechanisms (e.g.,
Blessed Dementia Scale B 413
Batten, S., Beal, S., Bleiberg, J., et al. (2009). Defense Centers of Excellence
for Psychological Health and Traumatic Brain Injury and Defense
and Veterans Brain Injury Center consensus conference on cognitive Description
rehabilitation for mild traumatic brain injury. Washington, D.C.
Cernak, I., Wang, Z., Jiang, J., Bian, X., & Savic, J. (2001). Ultrastructural The Blessed Dementia Scale (DS) was developed in 1968
and functional characteristics of blast injury-induced neurotrauma.
by Blessed and colleagues in an attempt to quantify the
The Journal of Trauma, Injury, Infection, and Critical Care, 50,
695706.
degree of intellectual and personality deterioration
Gaylord, K., Cooper, D., Mercade J., Kennedy, J., Yoder, L., & Holcomb, J. (p. 799) in the elderly.
(2008). Incidence of posttraumatic stress disorder and mild trau- This rating scale consists of 22 items that reflect (1)
matic brain injury in burned service members: Preliminary report. changes in performance of everyday activities (8 items;
The Journal of Trauma, Injury, Infection, and Critical Care, 64,
e.g., using money and finding ones way), (2) changes in
S200S206.
Hoge, C., McGurk, D., Thomas, J., Cox, A., Engel, C., & Castro, C. (2008).
habits including self-care (3 items; i.e., eating, dressing
Mild traumatic brain injury in U.S. soldiers returning from Iraq. The and continence), and (3) changes in personality, interests,
New England Journal of Medicine, 358(5), 453463. and drives (11 items; e.g., evaluation of rigidity and
Jackson, G., Hamilton, N., & Tupler, L. (2008). Detecting traumatic brain affect). A close friend or relative is asked to provide
injury among veterans of operations enduring and Iraqi freedom.
these behavior ratings of the examinee over the past six
North Carolina Medical Journal, 69(1), 4347.
Jones, E., Fear, N., & Wessely, S. (2007). Shell shock and mild traumatic
months; when unavailable, medical records can be used.
brain injury: A historical review. American Journal of Psychiatry, 164, The DS is scored on a 028 point scale, where higher
16411645. numbers indicate a larger decrement in functional capac-
ity. On everyday activity items, a score of 1 is given for
total inability to perform a task, a score of is given for
partial, variable, or intermittent inability to perform
an activity, and a score of 0 is given if the patient is able
Bleeding to perform the task. The changes in habits section is
scored on a 4-point scale (i.e., 03), resulting in a stronger
Hemorrhage contribution to the total score. Personality changes
414 B Blessed Dementia Scale
are scored 1 if present or 0 if absent (Blessed, Tomlinson, Heyman, 1988). Additional analysis of the scale has indi-
& Roth, 1968; Blessed, Tomlinson, & Roth, 1988). A total cated that the items can be subdivided into four groups,
cut-off score of 4 out of 28 is typically used to differentiate each with its own score (Cognitive, items 17, score range
patients with dementia versus those without. Scores of 07; Personality Change, items 1217, score range 06;
49 indicate mild impairment, whereas scores of 10 or Apathy/Withdrawal, 18, 20, and 21, score range 03;
higher suggest moderate to severe impairment (Eastwood, Basic Self-Care, 911, score range 03), in order to aid
Lautenschlaegar, & Corbin, 1983). Stern, Mayeux, Sano, in interpretation (Stern, Hesdorffer, Sano, & Mayeux,
Hauser, and Bush (1987) have suggested 15 as the thresh- 1990).
old for moderate impairment.
The original DS also included a second section com-
prised of a brief battery of simple cognitive tasks, called Psychometric Data
the Information-Memory-Concentration Test (IMCT;
Blessed et al., 1968; Blessed et al., 1988). Similar to other In community-dwelling individuals, testretest reliabil-
brief mental status instruments, the IMCT incorporates ity after 4 weeks was r = 0.79; the first 11 items show
12 items of information/orientation, 11 items of long- marginal reliability (r = 0.68; Erkinjuntti et al., 1988).
term memory, a brief test for the 5-min recall of a persons Cole (1990) found an interrater reliability of r = 0.59
name and address, and three sequencing tasks requiring when comparing DS ratings by two independent raters
concentration (Blessed et al., 1968; Blessed et al., 1988). who each interviewed the caretakers of 47 dementia
This sub-component is typically no longer included in patients.
the DS. The initial study employing the DS showed that scores
increased as the presence of senile plaques increased
(r = 0.77; Blessed et al., 1968). Also, the DS showed
Historical Background discriminative validity in identifying senile dementia
patients compared with depressed, paraphrenia, delirious,
The original Dementia Scale (DS) evaluated informant- and physically ill patients (Blessed et al., 1968). Others
reported changes in behavior and daily functioning and have also noted that the DS is able to discriminate
also included cognitive tasks given to the patient. It was between dementia patients and community residents
developed by Blessed, Tomlinson, and Roth in 1968 in (Erkinjuntti et al., 1988; Lam et al., 1997). When a cut-
an attempt to compare the deterioration of intellect off of 4/28 was used, the DS was shown to have a sensitiv-
and personality with underlying brain neuropathology ity of 90% and a specificity of 84% (Erkinjuntti et al.,
(Blessed et al., 1968; Blessed et al., 1988). The Revised 1988). Moderate to high correlations have been reported
Dementia Scale (RDS) was introduced in 1988 and in- with other measures such as the CERAD Total score
cluded only items reflecting informant-rated changes (r = 0.40; Chandler et al., 2005), the Mini-Mental Status
in everyday activities and habits (items 1 through 11; Exam (r = 0.80; Hendrie et al., 1988), and the CAMDEX
Erkinjuntti, Hokkanen, Sulkava, & Palos, 1988). The sen- (r = 0.77; Hendrie et al., 1988). Additionally, Stern et al.
sitivity and specificity of the revised scale was higher than (1987) reported that disease progression can be moni-
that of the original DS, possibly due to lower dementia- tored using the DS; cognitive deficiencies affecting
specificity of the excluded items (i.e., changes in person- instrumental activities of daily living (e.g., handling
ality, interests, and drive; Lawson, Rodenburg, & Dykes, money, remembering short lists) were evident early
1977). However, the 4-week testretest reliability for the and worsened throughout the disease course, whereas
revised scale was lower (r = 0.68) than the original changes in basic self-care did not occur until 45 years
(r = 0.79), potentially due to the inclusion of fewer into the illness (Stern et al., 1990).
items (Erkinjuntti, et al., 1988). A cut-off of 1.5 on the RDS yields a sensitivity of
Items from the DS have been included in the standar- 93% and a specificity of 97% in discriminating between
dized interview with relatives that is part of the Cam- demented and non-demented subjects, regardless of
bridge Mental Disorders of the Elderly Examination level of dementia (Erkinjuntti et al., 1988). The RDS
(CAMDEX; Roth et al., 1986). Elements of this scale is also highly correlated with the Activities of Daily
have also been incorporated in the standardized battery Living Scale, the Instrumental Activities of Daily Living
of the Consortium to Establish a Registry for Alzheimers Scale, and the Functional Activities Questionnaire (Juva
Disease (CERAD; Morris, Mohs, Rogers, Fillenbaum, & et al., 1997).
Blessed Dementia Scale B 415
The DS appears minimally affected by demographic Chandler, M. J., Lacritz, L. H., Hynan, L. S., Barnard, H. D. Allen, G.,
Deschner, M., et al. (2005). A total score for the CERAD neuropsy-
factors. Age correlates moderately with DS scores
chological battery. Neurology, 65(1), 102106.
(r = 0.31), but when degree of dementia is taken into Cole, M. G. (1990). Interrater reliability of the Blessed Dementia Scale. B
account, age does not have a significant effect (Erkinjuniti Canadian Journal of Psychiatry, 35(4), 328330.
et al., 1988). Education appears unrelated to DS scores Eastwood, M. R., Lautenschlaegar, E., & Corbin, S. (1983). A comparison
(Erkinjuniti et al., 1988). AfricanAmerican patients score of clinical methods for assessing dementia. Journal of the American
Geriatrics Society, 31(6), 342347.
higher on the DS than white patients (Hargrove, Stoeklin,
Erkinjuntti, T., Hokkanen, L., Sulkava, R., & Palos, J. (1988). The Blessed
Haan, & Reed, 1998). The DS has been translated Dementia Scale as a screening test for dementia. International Jour-
and validated in Chinese, Korean, and Czech (Lam et al., nal of Geriatric Psychiatry, 3, 267273.
1997; Lee et al., 1999; Vajdickova, Kolibas, Heretik, & Folstein, M. F., Folstein, S. E., & McHugh, P. R. (1975). Mini Mental State:
Kosc, 1995). A practical method for grading the cognitive state of patients for the
clinician. Journal of Psychiatric Research, 12(3), 189198.
Hargrove, R., Stoeklin, M., Haan, M., & Reed, B. (1998). Clinical aspects
of Alzheimers disease in Black and White patients. Journal of the
Clinical Uses National Medical Association, 90, 7884.
Hendrie, H. C., Hall, K. S., Brittain, H. M., Austrom, M. G., Farlow, M.,
Parker, J., et al. (1988). The CAMDEX: A standardized instrument for
The DS offers a blend of items commonly found on the diagnosis of mental disorder in the elderly: A replication with a U.S.
mental status exams, activities of daily living scales, and Sample. Journal of the American Geriatric Society, 36(5), 402408.
instrumental activities of daily living scales. It is quick and Juva, K., Maela, M., Erkinjuntti, T., Sulkava, R., Yukoski, R., Valvanne, J.,
easy to administer, and additionally provides a quantifi- et al. (1997). Functional assessment scales in detecting dementia. Age
and ageing, 26(5), 393400.
cation of the degree of dementia severity. As such, it is
Lam, L. C. W., Chiu, H. F. K., Li, S. W., Chan, W. F., Chan, C. K. Y,
ideal for use by general practitioners and specialized Wong, M., et al. (1997). Screening for dementia: A preliminary study
medical and mental health care professionals to gauge on the validity of the Chinese version of the Blessed-Roth Dementia
initial status, as well as to track disease progression. Scale. International Psychogeriatrics, 9(1), 3946.
The DS may also provide more useful information in Lawson, J. S., Rodenburg, M., & Dykes, J. A. (1977). A dementia rating
scale for use with psychogeriatric patients. Journal of Gerontology, 32,
a clinical setting than the MMSE and other cognitive
153159.
assessment scales (Mant, Eyland, Pond, Saunders, & Lee, D. Y., Yoon, J. C., Lee, K. U., Jhoo, J. H., Kim, K. W., Lee, J. H., et al.
Chancellor, 1988) because it measures functional aspects (1999). Reliability and validity of the Korean version of Short Blessed
of dementia. Test (SBT-K) as a dementia screening instrument. Journal of the
Korean Neuropsychiatric Association, 38, 13651375.
Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychologi-
cal assessment (4th ed.). New York: Oxford.
Cross References Mant, A., Eyland, E. A. Pond, D. C., Saunders, N. A., & Chancellor, A. H. B.
(1988). Recognition of dementia in general practice: Comparison of
general practicioners opinion with assessments using the Mini-
Activities of Daily Living Scale
Mental State Examination and the Blessed Dementia Scale. Family
Alzheimers Disease Practice, 5(3), 184188.
CERAD Morris, J. C., Mohs, R. C., Rogers, H., Fillenbaum, G., & Heyman, A.
Clinical Dementia Rating (1988). Consortium to establish a registry for Alzheimers disease
Dementia (CERAD) clinical and neuropsychological assessment of Alzheimers
disease. Psychopharmacology Bulletin, 24(4), 641651.
Dementia Rating Scale
Roth, M., Tym, E., Mountjoy, C. Q., Huppert, F. A., Hendrie, H.,
Lawton-Brody Instrumental Activities of Daily Living Verma, S., et al. (1986). CAMDEX: A standardized instrument for
Scale the diagnosis of mental disorders in the elderly with special reference
to early detection of dementia. British Journal of Psychiatry, 149,
698709.
Stern, Y., Hesdorffer, D., Sano, M., & Mayeux, R. (1990). Measurement
References and Readings and prediction of functional capacity in Alzheimers disease.
Neurology, 40(1), 814.
Blessed, G., Tomlinson, B. E., & Roth, M. (1968). The association between Stern, Y., Mayeux, R., Sano, M., Hauser, W. A., & Bush, T. (1987).
quantitative measures of dementia and of senile changes in the Predictors of disease course in patients with probable Alzheimers
cerebral gray matter of elderly subjects. British Journal of Psychiatry, disease. Neurology, 37(10), 16491653.
114(512), 797811. Vajdickova, K., Kolibas, E., Heretik, A., & Kosc, M. (1995). Application of
Blessed, G., Tomlinson, B. E., & Roth, M. (1988). Blessed-Roth Dementia behavioural scale in the diagnosis of dementia of advanced age.
Scale (DS). Psychopharmacology Bulletin, 24(4), 705708. Ceska a Slovenska Psychiatrie, 91(1), 714.
416 B BlessedRoth DS
Categorization
There are two types of blindsight, termed Type I and Type II.
Patients with Type I blindsight report no conscious aware-
ness of stimuli presented in the damaged region of their
visual field, yet preserve the ability to detect stimuli pre-
sented there. Patients with Type II blindsight, report a faint
conscious perception of stimuli in the damaged region of
their visual field, yet preserve the ability to detect stimuli
with higher precision than their conscious perception.
Epidemiology
field contralateral to the hemisphere where the brain motion, and recent evidence suggests some patients
injury has occurred. For example, damage to the left can even differentiate between different wavelengths of
hemisphere of V1 results in impairment to the right color and form presented simultaneously in their scotoma B
visual field. Typically, because early visual brain areas (Trevethan, Sahraie & Weiskrantz, 2007). Primate studies
are retinotopically mapped, the extent of the damage support the claim that this unconscious perception is
to occipital lobe corresponds to the extent of the not subserved by islands of undamaged tissue. When
impairment in the visual field. For example, if an the striate cortex of primates is cortically ablated, like
entire hemispheres occipital lobe is ablated then the humans, they have no conscious perception of stimuli
entire contralateral visual field is damaged a condi- presented in their scotoma, but do retain unconscious
tion is termed hemianopia. Likewise, if a quarter of perception. The strongest neural evidence supporting
V1 is damaged (i.e., one half of one hemispheres the existence of blindsight comes from the identifica-
occipital lobe), a quarter of the contralateral visual tion subcortical connections from the Lateral Genicu-
field is damaged a condition is termed quadranopia. late Nucleus (LGN) directly to the extrastriate cortex.
Figure 2 illustrates the pattern of visual impairment and These pathways, unaffected by V1 damage, are potential
the corresponding scotoma that arises when only a small mediators for the unconscious visual perception ob-
region of occipital lobe is damaged. Although a patient served in blindsight. The identification of this pathway
with such a pattern of sparing and loss might experience has prompted a fascinating debate regarding the role
blindsight in such a restricted scotoma, the most interest- of V1 in the neural representation of consciousness.
ing cases of blindsight have been where patients have Scientists have posited that if there is perception, but
lost all conscious visual experience, that is, there is not conscious perception, without V1, then V1 must
injury covering early visual brain areas across both play a critical role as a neural correlate of conscious-
hemispheres. ness; this remains an active area of research (for review
Note that not all patients with scotomas experience see Tong, 2003).
blindsight. The functional definition is that despite an
absence of conscious perception, patients with blindsight
retain the ability to detect and discriminate stimuli pre- Evaluation
sented in their scotoma. For example, patients can localize
moving and stationary stimuli using saccades or pointing. Because blindsight patients experience no conscious
They can also discriminate line orientations, detect awareness of stimuli presented in their scotoma,
Right LGN
Optic radiation
Scotoma
Area of damage
Blindsight. Figure 2 The relationship between a region of damage in visual cortex (V1) and the corresponding impairment in the
visual field (Adapted from Bear, Connors, & Paradiso, 2006)
418 B Blitz-Nick-Salaam Krampfe
experimenters rely on a two-alternative forced-choice explanation cannot account for now-present circular
(2AFC) procedure to diagnose and evaluate the symp- form discrimination (Trevethan et al., 2007). One possi-
toms of the disorder. The 2AFC procedure, typically bility is that DBs improvement in form perception is the
used to assess behavioral performance in nonspeaking result of the large number of hours DB spent completing
primates, presents patients with a target stimulus, a experimental testing.
probe stimulus (matched to target), and a distractor One point to consider when diagnosing or treat-
stimulus (non-matched to target); the target can be pre- ing patients with blindsight is that Type I patients have
sented either prior to or simultaneously with the paired no conscious access to the stimuli presented in the sco-
probe and distractor. The patient must select either the toma. Consequently, experimenters should be cautious
probe or the distractor as matching the target. The patient in asking for specific answers when running a 2AFC task
is not allowed to respond, I dont know, so in this way in that this task may be distressing to a patient who
the task is a forced-choice. In a variant of this procedure experiences no conscious visual perception. That is,
used to assess blindsight, experimenters present an image they may find such a task irrelevant to their personal
both to the scotoma and the hemisphere of normal visual experience.
acuity then ask subjects to report whether the two stimuli
are the same or different (Weiskrantz, 1986). Given that
chance performance in these procedures is 50%, it is Cross References
interesting to note that patients estimate their success
rate on these tasks to be roughly 50%, but in reality Cortical Blindness
their success rate is closer to 90%, illustrating the discon- Hemianopia
nect between conscious and unconscious vision in blind- Scotoma
sight. Similar results have been observed using a Visual Field Deficit
somewhat different procedure designed to measure the
unconscious perception of visual motion in blindsight
patients: saccades tracking or pointing in which a moving
References and Readings
object is presented within the visual field of the scotoma
Bear, M. A., Connors, B. W., & Paradiso, M. A. (2006). Neuro-
and the patient is asked to track the object with their eyes
science exploring the brain. Baltimore, MD: Lippincott Williams &
or with their finger. Wilkins.
Dineen, J., & Keating, E. G. (1981). The primate visual system after
bilateral removal of striate cortex. Survival of complex pattern
Treatment vision. Experimental Brain Research. Experimentelle Hirnforschung.
Experimentation Cerebrale, 41(34), 338345.
Poppel, E., Held, R., & Frost, D. (1973). Residual visual function after
There is a period of spontaneous recovery for neurovi- brain wounds involving the central visual pathways in man. Letters to
sual lesions, typically up to 3 months post lesion, but has Nature, 243, 295296.
been reported to extend to up to a year. Following this Sahraie, A., Trevethan, C. T., MacLeod, M. J., Murray, A. D., Olson, J. A.,
period, active discrimination of stimuli presented in the & Weiskrantz, L. (2006). Increased sensitivity after repeated stimu-
lation of residual spatial channels in blindsight. Proceedings of the
scotoma seems to be the best strategy for improvement
National Academy of Sciences of the United States of America, 103(40),
in humans (Sahraie, Trevethan, MacLeod, Murray, 1497114976.
Olson, & Weiskrantz, 2006), and nonhuman primates Tong, F. (2003). Primary visual cortex and visual awareness. Nature
(Dineen & Keating, 1981). As such, blindsight patients Reviews. Neuroscience, 4(3), 219229.
that participate as experimental subjects sometimes show Trevethan, C. T., Sahraie, A., & Weiskrantz, L. (2007). Can blindsight be
superior to sighted-sight?. Cognition, 103(3), 491501.
large improvements in their visual discrimination abil-
Weiskrantz, L. (1986). Blindsight: A case study and its implications. Net
ities. For example patient DB, the first blindsight patient Library, USA: Oxford University Press.
studied extensively (Weiskrantz, 1986), was recently Werner, J. S., & Chalupa, L. M. (2004). The visual neurosciences.
retested, 30 years after his right striate cortex was surgi- Cambridge, MA: MIT Press.
cally removed during treatment of a nonmalignant ve-
nous tumor. Patient DB can now discriminate complex
circular forms presented in his scotoma, for example, he
can discriminate a circle from an oval. Previously the Blitz-Nick-Salaam Krampfe
ability to distinguish form was accounted for by DBs
ability to distinguish line orientations; however, this West Syndrome
Block Design B 419
of the multidimensional cognitive skills necessary for test performance. These are listed below and are part of
optimal performance on the BD test comes from two the WAIS-R-NI corpus (Kaplan et al., 1991).
sources: patients with cerebral disconnection (Geschwind,
1. Providing Additional Blocks Rather than constraining
1979; Kaplan, 1988); and patients with focal brain lesions.
the examinees performance by providing only 4 or 9
For example, patients who have undergone a commissur-
blocks as prescribed by the Wechsler test manual,
otomy (Geschwind, 1979; Kaplan, 1988) provided a
Kaplan et al. (1991) suggests presenting the patient
unique opportunity to study BD problem-solving strate-
with 9 blocks on all 4-block test items and 12 blocks
gies because these patients serve as their own controls.
on all 9-block test items. Attempting to construct
Since these patients have undergone resection of the corpus
designs with too few or too many blocks conveys
callosum and the anterior commissure, sensory informa-
additional information about possible spatial as well
tion cannot be transferred between the hemispheres. Illus-
as executive impairment.
trations provided by Geschwind (1979) and Kaplan
2. Flow Charting Documenting the patients perfor-
(1988) show that when commissurotomized patients use
mance with a flow chart is mandatory to the cogent
their right hand, that is, when BD constructions are guid-
analysis of BD test performance. Examples of the rich
ed by the left hemisphere with no input from the right
data which can be obtained with a flow chart are
hemisphere, the inherent 2 2 or 3 3 matrix is violated
illustrated by Kaplan et al. (1991, Figure 6). As de-
and there is a tendency for blocks to pile up on the right
scribed above, focal right-hemisphere lesioned
side of the design reflecting an inattention of left hemi-
patients tend to break the 2 2 or 3 3 grid config-
space suggestive right hemisphere dysfunction (Kaplan,
uration of the stimulus matrix and often produce
1988, Figures 12). Very different errors occur with com-
distorted responses. Kaplan et al. (1991) provides
missurotomized patients attempting BD using their
examples of BD constructions produced by right-an-
left hand, that is, when constructions are guided by the
terior and right-posterior lesioned patients in Figures
right hemisphere with no input from the left hemi-
6c and 6d, respectively (see p. 90). The 3 3 grid
sphere. Now, the 2 2 or 3 3 grid matrix is rarely
configuration is broken by both patients. However, the
violated. However, blocks tend to be rotated so that the
construction of a patient with a right-posterior lesion
internal details of individual blocks do not match
is measurably more distorted than the construction
the model. Thus, Geschwind (1979) and Kaplan (1988)
produced by a right-anterior lesioned patient suggest-
show that regardless of which hand is used commissur-
ing greater perceptual-spatial impairment. Thus, as
otomized patients produce zero point responses, but the
suggested many years ago by Kohs (1920, 1923) an
underlying brain-behavior relationships responsible for
analysis of BD moves provides important
these response strategies are very different. Kaplan,
information.
Palmer, Weinstein and Baker (1981) noted that similar
3. Errors Subtypes The WAIS-R-NI (Kaplan et al., 1991)
behavior occurs in patients with focal right and left hemi-
suggests a variety of error scores that supplement
sphere lesions. Patients with right-sided lesions often
the traditional total Wechsler scale score. The scoring
break the 2 2 or 3 3 matrix inherent in the stimulus
techniques described below are designed to supplement
resulting in highly distorted responses, blocks continue to
standardized scoring procedures and help in iden-
collect on the right side of hemi-space, and constructions
tifying underlying brain pathology.
are often initiated on the right-side with patients working
(a) Rotational errors scored when a blocks surface
from right to left. Patients with left-sided lesions respect
coloring is incorrect. This type of internal detail
the inherent grid configuration of the BD stimuli. These
error could be associated with a left hemisphere
patients often make single-block, rotational errors or mis-
lesion.
align internal details (Kaplan et al., 1991) with responses
(b) Broken configuration scored when the 2 2 or
initiated on the left side of hemi-space.
3 3 grid matrix of the design is violated. As
noted above, such errors are often seen in
patients with right hemisphere lesions.
Clinical Uses While rotational and broken configuration errors often
occur in patients with circumscribed stroke, patients
Kaplan and colleagues (Kaplan, 1988; Kaplan et al., 1991) with epilepsy (Zipf-Williams, 2000) or brain injury
have suggested a number of additional testing and scoring (Wilde et al., 2000) lateralized to one side of the brain
procedures to extract detailed information from the BD may also make these errors.
Block Design B 421
(c) Orientation errors scored when a block(s) is incor- have been debated. Joy et al. (2001) provided a compre-
rectly oriented, that is, when the final product or hensive evaluation of the reported age-related decline in
elements of the final product are shifted or misor- BD test performance and offered normative data for the B
iented about 30 degrees in relation to the model. clinical interpretation of BD in healthy older adults.
Spatial, perceptual, or executive problems might un- In addition to standard passfail scoring, these researchers
derlie this difficulty. also utilized proportional scoring methods as well as
(d) Perseverations scored when incorrect block pla- the supplemental measures detailed in the WAIS-R-NI.
cements persist either within or between successive Results confirmed a moderate negative correlation be-
BD trials. Gross perseverative behavior is often tween standard BD score and age (r = 0.455); however,
seen in patients with frontal lobe or frontal sys- the use of proportion scores, elimination of time con-
tems lesions. Less severe perseverative behavior straints, and termination of time-bonuses significantly
might occur in conjunction with rotational and reduced the documented age differences. These authors
broken configuration errors and may suggest dys- interpreted this finding as evidence for less severe age-
executive behavior associated with a specific brain related impairment in visuospatial and constructional
region. abilities in healthy older adults than traditional scoring
(e) Stimulus bound examples include instances when techniques suggest. In general, it is important to carefully
the examinee is drawn to build their construction consider the role of psychomotor slowing and error types
either right next to or under the stimulus booklet or when administering the standard block design test to
even pile blocks on top of the stimulus booklet. healthy older adults in order to avoid differently penaliz-
Less egregious, but no less important stimulus ing individuals based on age. Older adults diagnosed with
bound errors occur when patients are aware of but a neurodegenerative disorder exhibit different patterns of
unable to self-correct errors. errors depending upon their neuropsychological profile
(f) Response latency patients with bradyphrenia may and diagnosis. Stimulus bound errors, broken configura-
ultimately produce a correct construction and might tions, and psychornotor slowing are all more prevalent in
be able to self-correct errors but may complete a individuals diagnosed with a dementia relative healthy
correct design only after the time limit as prescribed older adult controls.
in the test manual has passed. Such behavior might be
associated with subcortical syndromes. However,
slow time to completion often occurs in patients
with alcohol abuse, brain injury, multiple sclerosis, Cross References
or epilepsy.
(g) Start position using a flow chart, documenting the Constructional Apraxia
start position of the first block also allows for exami- Visuoconstruction
nation of a preferred side, and can be indicative of
lateralized brain dysfunction (Akshoomoff-Haist
et al., 1989).
References and Readings
Joy, S., Fein, D., Kaplan, E., & Freedman, M. (2001). Quantifying quali-
tative features of block design performance among healthy older Blood Alcohol Level
adults. Archives of Clinical Neuropsychology, 16, 157170.
Kaplan, E. (1988). A process approach to neuropsychological assessment.
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2
Kaplan, E., Palmer, E. P., Weinstein, C., & Baker, E. (1981). Block Design: Cincinnati Childrens Hospital Medical Center
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Bergen, Norway.
Kaplan, E., Fein, D., Morris, R., & Delis, D. (1991). The WAIS-R as a
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Kohs, S. C. (1920). The block design tests. Journal of Experimental
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Kramer, J. H., Kaplan, E., Blusewicz, M. J., & Preston, K. A. (1991). Visual
hierarchical analysis of block design configural errors. Journal of Measure of alcohol in the blood.
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assessment (4th ed.). New York: Oxford University Press. Current Knowledge
Schorr, D., Bower, G. H., & Kiernan, R. (1982). Stimulus variables in the
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effects on block design: Qualitative performance features and g/dL). A level of 2030 mg/dL typically results from the
extended-time effects. Neuropsychology, 8, 9599. ingestion of one to two drinks. One drink corresponds to
Wilde, M. C., Boake, C., & Sherer, M. (2000). Wechsler Adult Intelligence
340 mL (12 oz.) of beer, 115 mL (4oz) of wine, and 43 mL
Scale-Revised Block Design broker configuration errors in
non-penetrating traumatic brain injury. Applied Neuropsychology,
(1.5 oz) of a shot. Blood alcohol levels as low as 2080 mg/
7, 208214. dL can lead to decreased inhibitions and decreased cogni-
Zip-Williams, E. M., Shear, P. K., Strongin, D., et al., (2000). Qualitative tive and motor performance, while levels of 300400 mg/
Block Design performance in epilepsy patients. Archives of Clinical dL can lead coma or death. Blood alcohol levels typically
Neuropsychology, 15, 149157.
correlate inversely with cognitive and motor performance
(i.e., as blood alcohol levels increase, cognitive and motor
performance decrease). Specifically, increased blood alco-
hol levels correlate with slower reaction time and inversely
correlate with frontal executive function. Additionally,
Blocqs Disease speed of cognitive performance recovers as alcohol levels
return to drug-free levels; however, accuracy may contin-
Astasia-Abasia
ue to remain impaired.
Cross References
Blood Alcohol Concentration Coma
Executive Function
Blood Alcohol Level Frontal Lobe
Schweizer, T. A., & Vogel-Sprott, M. (2008). Alcohol-impaired speed and There are four different types of Doppler US studies
accuracy of cognitive functions: A review of acute tolerance and
currently being utilized by physicians. Continuous wave
recover of cognitive performance. Experimental and Clinical Psycho-
pharmacology, 16(3), 340350.
Doppler is typically used at the bedside and only produces B
Harrisons Online Harrisons principles of internal medicine, 16th edn. sound from the transducer which the practitioner uses
Chapter 372: Alcohol and Alcoholism. to listen for blockage or stenosis of the vessel. Duplex
Doppler produces both a picture of the blood vessel and
a graph representing the speed and direction of blood flow
(hence the name duplex). Color Doppler uses a com-
puter to convert the Doppler sounds into colors and
Blood Clot
overlay those colors on an image of the blood vessel.
Power Doppler is more sensitive than color Doppler in
Hematoma
detecting blood flow. It combines the results given by
color Doppler with duplex Doppler. It is commonly
used to evaluate the flow of blood through vessels within
solid organs.
Blood Flow Studies Transcranial Doppler (TCD) is used to measure
the blood flow through the brains blood vessels. It is
C INDY B. I VANHOE , N ATASHA K. E ADDY becoming more widely used to evaluate for embolus,
Neurorehabilitation Specialists stenosis, vasospasm, and the risk of stroke.
Houston, TX, USA
Cross References
Synonyms Doppler Ultrasound
Regional Cerebral Blood Flow
Duplex/Doppler ultrasound (US); Vascular ultrasound Transcranial Doppler
Current Knowledge
Blood Oxygen Level-Dependent
Blood flow studies such as Doppler US uses a transducer
that sends high-frequency sound waves which bounce off J OHN W HYTE
of solid objects including red blood cells. The sound waves Moss Rehabilitation Research Institute
are reflected back to the transducer. Moving objects, Albert Einstein Healthcare Network
such as the red blood cells, cause a change in pitch of Elkins Park, PA, USA
the sound waves (also known as the Doppler effect).
These reflected waves are sent to and processed by a
computer which translates the waves into pictures or Synonyms
graphs. The images are representative of the flow of
blood through the vessel. BOLD
424 B Blood Oxygen Level-Dependent
Kida, I., & Hyder, F. (2006). Physiology of functional magnetic resonance resistance, and lack of pinocytic vesicles, provide limited
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lipophilic molecules less than 600 Da can passively diffuse
fMRI contrast mechanism. Magnetic Resonance Imaging, 22(10), through the BBB. This protects the brain from toxins,
15171531. microorganisms (i.e., bacteria) and peripheral neuro-
Nair, D. G. (2005). About being BOLD. Research Brain Research transmitters. This selective barrier can potentially limit
Reviews, 50(2), 229243.
entry of large substances required for normal brain func-
Stephan, K. E., Harrison, L. M., Penny, W. D., & Friston, K. J. (2004).
Biophysical models of fMRI responses. Current Opinion in Neuro-
tion, including insulin, amino acids, and glucose. In order
biology, 14(5), 629635. to circumvent this problem, the BBB has developed highly
Steward, C. A., Marsden, C. A., Prior, M. J., Morris, P. G., & Shah, Y. B. specialized transport mechanisms on both the luminal
(2005). Methodological considerations in rat brain BOLD contrast and albuminal membrane surfaces, such as Na-K-Cl
pharmacological MRI. Psychopharmacology, 180(4), 687704.
cotransporter, g-glutamyl transpeptidase (GGTP), and
the GLUT-1 glucose transporter. The protective BBB can
be at a disadvantage in that it prevents the entry of
pharmacologic agents that are often hydrophilic.
Blood Thinner Also unique to brain microvascular endothelium is
their intimate association with astrocytes, forming the
Warfarin (Coumadin)
glia limitans. Astrocytes are thought to participate in
the induction and maintenance of the endothelial BBB
phenotype. In vitro studies have shown that astrocytes
cocultured with endothelial cells can induce BBB pheno-
Blood-Brain Barrier typic features, including tight junctions and increased
electrical resistance. Astrocytic membranes and superna-
N AM T RAN
tant from astrocytic cultures share similar inductive
Virginia Commonwealth University Medical Center
properties. The mechanisms of this induction and the
Richmond, VA, USA
inductive factor(s) have yet to be fully elucidated.
In addition to its regulatory role, studies of the BBB
are beginning to emerge to demonstrate its function in
Synonyms
establishing a unique brain milieu. In vitro BBB models
have shown decreased tissue plasminogen activator and
Cerebral microvasculature
then anticoagulant protein thrombomodulin expression
and increased plasminogen activator inhibitor-1 expres-
Definition sion by brain endothelium compared with endothelium
from the periphery. These findings suggest a procoagulant
The blood-brain barrier (BBB) maintains brain homeo- environment in the brain that may predispose the brain
stasis by regulating the movement of compounds across to strokes.
the endothelium of cerebral capillaries.
Cross References
Current Knowledge
Neuroglia
BBB serves to maintain brain homeostasis by regulating
the influx and efflux of compounds to and from the brain.
The presence of a barrier was first documented by Paul Blood-Filled Dilatation
Ehrlich in the late nineteenth century. However, it was not
until the advent of the electron microscope that the Aneurysm
makeup of the BBB was begun to be understood. The
brain microvascular endothelium comprises the BBB. In
contrast to endothelium from other vascular beds, the Blue Spot
morphologic features unique to the brain microvascular
endothelium, such as tight junctions, increased electrical Locus Ceruleus
Body Schema B 427
Autotopagnosia
Cortical Blindness Boston Diagnostic Aphasia
Finger Agnosia Examination
Hemiinattention
Right-Left Disorientation C AROLE R OTH
Naval Medical Center
San Diego, CA, USA
References and Readings
BDAE oral apraxia task has been correlated with other Brocas Aphasia
articulation tasks (Sussman et al., 1986); correlations for Conduction Aphasia
the auditory comprehension measure on the BDAE and Praxis
the Token Test and with respective measures of the Porch Wernickes Aphasia
Index of Communicative Ability (PICA) have been
reported (Divenyl & Robinson, 1989). Brookshire and References and Readings
Nichols (1984) found the BDAE auditory comprehension
subtest did not predict auditory paragraph comprehen- Brookshire, R. H., & Nicholas, L. E. (1984). Comprehension of directly
sion of independent standardized material. and indirectly stated main ideas and details in discourse by brain-
Goodglass and Kaplan designed the BDAE to assess damaged and non-brain-damaged listeners. Brain and Language, 21,
various components of language function for the purpose 2136.
Crary, M. A., Wertz, R. T., & Deal, J. L. (1992). Classifying aphasias:
of discriminating among different patterns of CNS lesions
Cluster analysis of Western Aphasia Battery and Boston Diagnostic
indicative of types of aphasia. Studies to date have not Aphasia Examination. Aphasiology, 6, 2936.
determined decision rules for the diagnosis of individual Davidoff, M., & Katz, R. (1985). Automated telephone therapy for im-
subtypes of aphasia (Crary et al., 1992; Reinvang & proving comprehension in aphasic adults. Cognitive Rehabilitation,
Graves, 1975). 3, 2628.
Divenyl, P. L., & Robinson, A. J. (1989). Nonlinguistic auditory capabil-
Ecological validity of the BDAE for predicting prog-
ities in aphasia. Brain and Language, 37, 290326.
ress in speech therapy has been described by various Goodglass, H., & Kaplan, E. (1972). Boston Diagnostic Aphasia Examina-
authors (Davidoff & Katz, 1985; Helm-Estbrooks & tion (BDAE). Philadelphia: Lea & Febiger.
Ramsberger, 1986; Marshall & Neuberger, 1994). Goodglass, H., & Kaplan, E. (1983). The assessment of aphasia and related
disorders (2nd ed.). Philadelphia: Liea & Febiger.
Goodglass, H., Kaplan, E., & Barresi, B. (2001a). The assessment of aphasia
Clinical Uses and related disorders (3rd ed.). Philadelphia: Lippincott Williams &
Wilkins.
Goodglass, H., Kaplan, E., & Barresi, B. (2001b). Boston diagnostic aphasia
The BDAE is derived from samples of adult patients
examination (3rd ed.). Philadelphia: Lippincott Williams & Wilkins.
with stroke and therefore is most useful when assessing Helm-Estabrooks, N., & Ramsberger, G. (1986). Aphasia treatment de-
inpatient or outpatient adult populations with language livered by telephone. Archives of Physical Medicine and Rehabilita-
impairments resulting from strokes, but it can be used tion, 67, 5153.
effectively with persons who have sustained traumatic Marshall, R. C., & Neuburger, S. I. (1994). Verbal self-correction and
improvement in treated aphasia clients. Aphasiology, 8, 535547.
brain injury. The BDAE offers a comprehensive look at
Pineda, D. A., Rosselli, M., Ardila, A., Mejia, S. E., Romero, M. G., &
language function from a neuropsychological perspective. Perez, C. (2000). The Boston Diagnostic Aphasia Examination-
Complete administration of this battery requires approx- Spanish Version: The influence of demographic variables. Journal
imately 90 min. The Short Form requires approximately of the International Neuropsychological Society, 6, 802814.
4060 min. The BDAE is one of the most popular Reinvang, I., & Graves, R. (1975). A basic aphasia examination: Descrip-
tion with discussion of first results. Scandinavian Journal of Rehabil-
batteries for use by speechlanguage pathologists for eval-
itation Medicine, 7, 129135.
uation of aphasia and other neurologic language impair- Spreen, O., & Risser, A. H. (Eds.). (2003). Assessment of aphasia.
ments. In addition to its strength as a comprehensive New York: Oxford University Press.
assessment of language, the BDAE provides useful Sussman, H., Marquardt, T., Hutchinson, J., & MacNeilage, P. (1986).
instructions for observing and recording specific types of Compensatory articulation in Brocas aphasia. Brain and Language,
27, 5674.
error responses (e.g., paraphasia) found in individuals
with aphasia, reflecting what has been termed the Boston
school approach to aphasia classification. The detailed
examination of conversational and expository speech is an
Boston Naming Test
important and unique aspect of the BDAE and is well
C AROLE R OTH
described in the manual (Goodglass et al, 2001a).
Naval Medical Center
San Diego, CA, USA
Cross References
Anomic Aphasia Synonyms
Aphasia
Boston Naming Test BNT
Boston Naming Test B 431
Psychometric Data
Administration
Reliability, Validity, and Norms:
The Boston Naming Test assesses naming abilities of
children, aphasic adults, and normal adults. The drawings
Reliability
are shown to the examinee one at a time, and the exam-
inee is asked to name each of them. Familiarity decreases
Internal consistency for the 60-item form has been
as the test progresses. Following presentation of each
reported to range between 0.78 and 0.96. Reliability coef-
picture stimulus, two types of cues may be presented
ficients have been lower for the abbreviated versions; for
when there is an error response: a stimulus cue (de-
example, the Mack SF4 version ranges between 0.49 and
scriptive) and a phonemic cue. A stimulus cue is pre-
0.84. Testretest reliability is high over short intervals. For
sented when the subject clearly misperceives the picture or
longer time intervals, such as 1112 months, testretest
indicates a lack of recognition of the picture. A phonemic
reliability was marginal to high; for example, in a healthy,
cue is presented after each error response, including fol-
elderly Caucasian adult population, testretest reliability
lowing a stimulus cue. The subject is given up to 20 s to
ranged between 0.62 and 0.89 (Mitrushina & Satz, 1995);
respond following each stimulus presentation, including
and high retest reliability (0.92) in a normal or neurolog-
the cues. All responses are recorded as a correct re-
ically stable adult population (Dikmen et al., 1999).
sponse or as an error with the actual error response
recorded for later coding by type. Types of cues presented
are recorded as stimulus cue or phonemic cue; re- Validity
sponse latencies in seconds are also recorded. The total
correct score is the sum of the accurate responses pre- The BNT has been shown to correlate highly with other
sented spontaneously and following a stimulus cue. language-related measures; including the Visual Naming
432 B Boston Naming Test
Test of the Multilingual Aphasia Examination (Axelrod for 663 primarily Caucasian individuals older than 55
et al., 1994; Schefft et al., 2003); as well as with measures years of age, derived from the Mayo Older Americans
of intelligence; including the Verbal Comprehension Fac- Normative Studies (the MOANS projects). Raw scores
tor of the WAIS-R and the Standard Raven Progressive are converted to age-corrected scaled scores having a
Matrices in children aged 612 years (Storms et al., 2004). mean of 10 and a standard deviation (SD) of 3 (Strauss
Poor performance on the BNT has been described in et al., 2006). Additional studies have expanded the utility
subjects with neurologic disease; including: left- of the MOANS project by providing age- and IQ-adjusted
hemisphere and brainstem strokes, anoxia, multiple percentile equivalents of MOANS Age-adjusted BNT
sclerosis, Parkinsons disease, Alzheimers disease, and scores, for individuals over 55 years (Steinberg et al.,
closed head injuries. 2005); age- and education-adjusted normative data
based on African Americans from the Mayo Older African
American Normative studies (MOAANS) project (Lucas
Norms et al., 2005) (Strauss et al., 2006).
Data on BNT norms for children is limited. The test
The norms available in the test booklet are limited authors provide norms for ages 5 years 0 months (50)
to small groups of adults ranging in age between 18 and through 125, based on small groups of participants. The
79 (N = 178) and of children ranging in age between data were collected in 1987 and the normative data are
50 years and 125 years (N = 356). Information about believed to be largely from Caucasian boys and girls who
geographical region, ethnicity, or time reference for this were attending public and private schools and living with
normative data is not provided. middle class families in suburban or urban areas of
Cross-sectional studies suggest that age (Heaton et al., the northeastern United States. There is a gap in the
2004; Ivnik et al., 1996; MacKay et al., 2005; Mitrushina BNT norms for adolescents between the ages of 14 and
et al., 2005) and verbal intelligence affect the BNT scores 17 years.
(Killgore & Adams, 1999; Steinberg et al., 2005;
Tombaugh & Hubley, 1997). Gender has been reported
to be unrelated to BNT performance (Henderson et al.,
1998; Ivnik et al., 1996; Lucas et al. 2005; Riva et al., 2000). Clinical Uses
Other studies suggest men outperform women in older
samples, possibly because of male-biased items The BNT, a visual confrontation naming test, is recom-
(Randolph et al., 1999). Reading vocabulary is strongly mended as a supplement to the Boston Diagnostic Apha-
correlated with BNT performance (Graves & Carswell, sia Examination. It can be used to assess naming abilities
2003; Senior et al., 2001). Geographic region and ethnicity of children, aphasic adults, and typical adults, although
have been shown to affect performance (Heaton et al., there is limited and poorly described normative data, and
2004; Lucas et al., 2005). Linguistic background also no testretest reliability for children.
affects test scores according to Roberts et al. (2002).
In the literature can be found a number of normative
reports for adult English speakers (see pp. 905907,
Strauss, Sherman, & Spreen, 2006). For example, Heaton Cross References
et al. (2004) reviewed studies over a 25-year period and
presented age, gender, and educational norms for two Anomia
ethnicity groups: Caucasians and African Americans. Boston Diagnostic Aphasia Examination
Mitrushina et al. (2005) compiled data from 14 studies,
comprising a total of 1,684 educated participants with
above-average intelligence who were administered the
60-item version. Their data was presented in 5-year incre-
References and Readings
ments, ranging from ages 25 to 84 years. The data is
Axelrod, B. N., Ricker, J. H., & Cherry, S. A. (1994). Concurrent validity
considered to be similar to those provided by Kaplan
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for individuals with lower educational and intellectual Dikmen, S. S., Heaton, R. K., Grant, I., & Temkin, N. R. (1999). Test
levels. Ivnik et al. (1996) provided age-corrected norms retest reliability and practice effects of expanded HalsteadReitan
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neuropsychological test battery. Journal of the International Neuro- Roberts, P. M., Garcia, L. J., Desrochers, A., & Hernandez, D. (2002).
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Fastenau, P. S., Denburg, N. L., & Mauer, B. A. (1998). Parallel short Naming Test. Aphasiology, 16, 635645.
forms for the Boston Naming Test: Psychometric properties and Saxton, J., Ratcliff, G., Munro, C. A., Coffery, C. E., Becker, J. E., Fried, L., B
norms for older adults. Journal of Clinical and Experimental Neuro- & Kuller, L. (2000). Normative data on the Boston Naming Test and
psychology, 20, 828834. two equivalent 30-item short-forms. The Clinical Neuropsychologist,
Goodglass, H., Kaplan, E., & Barresi, B. (2000). The assessment of aphasia 14, 526534.
and related disorders (3rd ed.). Philadelphia: Lea & Febiger. Schefft, B. K., Testa, S. M., Dualy, M. F., Privitera, M. D., & Yeh, H. S.
Graves, R. E., Bezeau, S. C., Fogarty, J., & Blair, R. (2004). Boston Naming (2003). Preoperative assessment of confrontation naming ability and
Test Short Forms: A comparison of previous forms with new item intrictal paraphasia production in unilateral temporal lobe epilepsy.
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Heaton, R. K., Miller, S. W., Taylor, M. J., & Grant, I. (2004). Revised R. J. (2005). MAYOs Older Americans Normative Studies: Age- and
comprehensive norms for an expanded Halstead Reitan Battery: De- IQ-adjusted norms for the Boston Naming Test, the MAE Token
mographically adjusted neuropsychological norms for African Ameri- Test, and the Judgment of Line Orientation Test. The Clinical Neu-
can and Caucasian adults, Lutz, FL: PAR. ropsychologist, 19, 280328.
Henderson, L. W., Frank, E. W., Pigatt, T., Abramson, R. K., & Houston, Storms, G., Saerens, J., & De Deyn, P. P. (2004). Normative data for the
M. (1998). Race, gender and educational level effects on Boston Boston Naming Test in native Dutch-speaking Belgian children and
Naming Test scores. Aphasiology, 12, 901911. the relation with intelligence. Brain and Language, 91, 274281.
Ivnik, R. J., Malec, J. F., Smith, G. E., Tangalos, E. G., & Peterson, R. C. Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of
(1996). Neuropsychological test norms above age 55: COWAT, BNT, neuropsyschological tests: Administration, norms, and commentary
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The Clinical neuropsychologist, 10, 262278. Teng, E. L., Wimer, C., Roberts, E., Damasio, A. R., Eslinger, P. J.,
Kaplan, E., Goodglass, H., & Weintrab, S. (1983). The Boston naming Folstein, M. F., Tune, L. E., Whitehouse, P. J., Bardolph, E. L.,
test. Philadelphia: Lea & Febiger. Hui, H. C., & Henderson, V. W. (1989). Alzheimers dementia:
Killgore, W. D. S., & Adams, R. L. (1999). Prediction of Boston Naming Performance on parallel forms of the Dementia Assessment Battery.
Test performance form vocabulary scores: Preliminary guidelines for Journal of Clinical and Experimental Neuropsychology, 11, 899912.
interpretation. Perceptual and Motor Skills, 89, 327337. Tombaugh, T. N., & Hubley, A. (1997). The 60-item Boston Naming Test:
Lansing, A. E., Ivnik, R. J., Cullum, C. M., & Randolph, C. (1999). An Norms for cognitively intact adults aged 25 to 88 years. Journal of
empirically derived short form of the Boston Naming Test. Archives Clinical and Experimental Neuropsychology, 19, 922932.
of Clinical Neuropsychology, 14, 481487. Van Gorp, W., Satz, P., Kiersch, M., & Henry, R. (1986). Normative data
Lucas, J. A., Ivnik, R. J., Smith, G. E., Ferman, T. J., Willis, F. B., Petersen, on the Boston naming test for a group of normal older adults.
R. C., 7 Graff-Radford, N. R. (2005). Mayos Older African Amer- Journal of Clinical and Experimental Neuropsychology, 8, 702705.
icans Normative Studies: Norms for Boston Naming Test, Controlled Welch, L. W., Doineau, D., Johnson, S., & King, D. (2002). Educational
Oral Word Association, Category Fluency, Animal naming, Token and gender normative data for the Boston naming test in a group of
Test, WRAT-3 Reading, Trail Making Test, Stroop Test, and Judgment older adults. Brain and Language, 53(2), 260266.
of line Orientation. The Clinical Neuropsychologist, 19, 243269. Williams, B. W., Mack, W., & Henderson, V. W. (1989). Boston naming
Mack, W. J., Freed, D. M., Williams, B. W., & Henderson, V. W. (1992). test in Alzheimers disease. Neuropsychologiea, 27, 10731079.
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ease. Journal of Gerontology, 47, 164168.
MacKay, A., Connor, L. T., & Henderson, V. W. (2005). Dementia does
not explain correlation between age and scores on Boston Naming
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Boston Process Approach
Mitrushina, M., & Satz, P. (1995). Repeated testing of normal elderly with
the Boston Naming Test. Aging Clinical and Experimental Research, S HAHAL R OZENBLATT
7, 123127. Advanced Psychological Assessment, P.C.
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Smithtown, NY, USA
Handbook of normative data for neuropsychological assessment
(2nd ed.). New York: Oxford University Press.
Neils, J., Baris, J. M., Carter, C., Dellaira, A. L., Nordloh, S. H., & Weiler, E.
(1995). Effects of age, education, and living environment on Boston Synonyms
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BPA
Nicholas, L. E., Brookshire, R. H., MacLennan, D. L., Schumacher, J. G.,
& Porrazzo, S. A. (1988). The Boston naming test: Revised adminis-
tration and scoring procedures and normative information for non-
brain-damaged adults. Clinical Aphasiology, 18, 103115.
Description
Randolph, C., Lansing, A., Ivnick, R. J., Cullum, C. M., & Hermann, B. P.
(1999). Determinants of confrontation naming performance. Born out of the work of A. R. Luria (e.g., Higher Cortical
Archives of Clinical Neuropsychology, 14, 489496. Function in Man, 1966), the Boston Process Approach
434 B BPA
(BPA) to neuropsychological assessment is a method of provides a number of standard scores, enabling compar-
exploring the patients approach to a task and the process isons with a normative sample. In addition, they provide
involved in attaining a specific test score (Loring, 1999). the clinician with a way of understanding the patients
Its aim is to provide a more accurate characterization of approach to the task. For example, on the CVLT-II, wheth-
neuropsychological function and dysfunction and the er the examinee makes use of semantic categories (e.g.,
nervous system components involved than is achieved types of animals) to aid in recall of the word list is consid-
with a purely psychometric approach (Kaplan, 1988; ered and quantified.
Strauss, Sherman, & Spreen, 2006). Despite the growing popularity of the BPA, there
have been criticisms. As outlined by Strauss et al. (2006),
criticisms of the approach include insufficient norms, lim-
Current Knowledge ited information about reliability and validity, and pro-
blems with readministration due to nonstandard initial
According to Edith Kaplan (1988, 1990), the achieve- administration.
ment oriented approach to assessment is flawed, in that
it assumes that the scores obtained are reflective of an
underlying unitary mechanism. As an example, two indi-
viduals could arrive at a similar score via distinctly differ- Cross References
ent processes that are dependent on distinctly different
neural structures and/or pathways. The inherent loss of Hypothesis Testing Approach
data that occurs by focusing on composite or total scores
resulted in the development of an approach that focused
on how a specific result was obtained. This avenue of
investigation led to the Boston Process Approach (BPA). References and Readings
In addition to careful observation of the strategies used
during the completion of a task, the BPA emphasizes the Delis, D. C., Kramer, J. H., Kaplan, E., & Ober, B. A. (2000). California
importance of demographic variables (e.g., age, gender, verbal learning test. Adult version manual (2nd ed.). San Antonio,
socioeconomic status, education, and occupational TX: The Psychological Corporation.
status), medical history, and mental health history, be- Kaplan, E. (1988). A process approach to neuropsychological assessment.
In T. Boll, & B. K. Bryant (Eds.), Clinical neuropsychology and brain
cause each of these variables can influence a patients
function: Research, measurement, and practice. Washington, DC:
performance. American Psychological Association.
The BPA differs from the fixed and flexible battery Kaplan, E. (1990). The process approach to neuropsychological assess-
approaches to testing in that the final score is deempha- ment of psychiatric patients. Journal of Neuropsychiatry, 2, 7287.
sized; that is, whether a response is right or wrong is less Loring, D. W. (1999). INS dictionary of neuropsychology. New York:
Oxford University Press.
important than how it was attained (Kaplan, 1990). In
Luria, A. R. (1966). Higher cortical function in man. New York: Basic
addition, the test may be administered differently from the Books.
standardized approach, and additional measures may be Milberg, W. P., Hebben, N., & Kaplan, E. (1986). The Boston process
introduced to better understand the component processes approach. In I. Grant, & K. M. Adams (Eds.), Neuropsychological
that influence or are involved in the performance of a assessment of neuropsychiatric disorders. New York: Oxford
University Press.
particular task. Modified materials may also be used to
Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of
gain a better understanding of the errors or unusual neuropsychological tests: Administration, norms, and commentary
approaches that were noted on a task (Milberg, Hebben, (3rd ed.). New York: Oxford University Press.
Kaplan, 1986). Wechsler, D., Kaplan, E., Fein, D., Kramer, J., Morris, R., et al. (2004).
Although right or wrong answers are deemphasized in Wechsler intelligence scale for children integrated (4th ed.). San Antonio,
TX: Harcourt Assessment.
the BPA, it is essential for the qualitative observations to
be quantifiable and subjected to statistical analyses
(Kaplan, 1990). This approach contributed to the devel-
opment of a wide variety of measures including the
Wechsler Intelligence Scale for Children, 4th Edition
Integrated (Wechsler, Kaplan, Kramer, Morris, 2004) BPA
and California Verbal Learning Test, 2nd Edition (Delis,
Kramer, Kaplan, & Ober, 2000). Each of these measures Boston Process Approach
Brain Abscesses B 435
BPRS Bradykinesia
Brief Psychiatric Rating Scale A NNA D E P OLD H OHLER 1, M ARCUS P ONCE DE LEON2 B
1
Boston University Medical Center
Boston, MA, USA
2
William Beaumont Army Medical Center
Brachytherapy El Paso, TX, USA
B RAM G OLDSTEIN
Hoag Hospital Cancer Center Definition
Newport Beach, CA, USA
Bradykinesia is a slowness of movement. It is often seen
in parkinsonian individuals and is a cardinal feature of
Synonyms Parkinsons disease. It can be seen in movements of small
muscles when an individual is asked to rapidly open and
Internal radiation therapy close a hand, tap a finger, or move an arm back and forth
to grab an object. It can involve any limb in isolation, such
as decreased arm swing during gait evaluation, or the
Definition entire body at once, evident in the abnormal stillness of
a patient with Parkinsons disease. It may fluctuate during
Brachytherapy is a form of radiation therapy, and the day depending on fatigue and medication levels in the
often indicated for the treatment of specific, recurrent case of Parkinsons disease.
brain tumors and head or neck cancers. The procedure
involves the placement of radioactive (e.g., iridium-192,
palladium-103, or iodine-125) seeds inside or adjacent to
Cross References
a targeted lesion. The primary advantage of brachythera-
py is that the treatment allows for a higher radioactive
Parkinsons Disease
dose to be delivered to the tumor bed without damaging
the surrounding, healthy brain tissue (Sneed, Prados,
Phillips, Weaver, & Wara, 1992). In particular, High
Dose Rate brachytherapy utilizes catheters to mitigate References and Readings
exposure and accelerate the treatment time. Intracavitary
brachytherapy is another subtype that involves the use of Fahn, S., & Jankovic, J. (Eds.). (2007). Parkinsonism: Clinical features and
a balloon catheter which delivers localized radiation diagnosis. In Movement disorders (pp. 79100). Philadelphia:
Churchill Livingstone Elsevier.
therapy to the affected area. Following the completion of
radiotherapy, the radiation source and balloon catheter
are then removed. Brachytherapy is a safe procedure,
although reported side effects include infection, seizures,
and headaches. Brain Abscesses
M ICHAEL R. V ILLANUEVA , S USAN K. J OHNSON
Cross References University of North Carolina-Charlotte
Charlotte, NC, USA
Radiation Therapy
Symptoms
Brain Attack
Prognosis
Stroke
If brain abscesses are left untreated, death is the most
likely outcome. On the other hand, treatment can signifi-
cantly reduce the mortality rate to about 10 percent.
Earlier treatment predicts a better outcome, although
long-term neurological deficits may persist despite all Brain Commissures
intervention approaches (Kumar et al., 2004 and http://
www.nlm.nih.gov/medlineplus/ency/article/000783.htm). Commissures, Cerebral
Brain Death B 437
to appropriate community-based care. It is the only states to plan reforms in treatment, transportation,
Federal law that addresses the millions of Americans housing, education, and social support, in order to inte-
who suffer permanent disability as a result of traumatic grate brain injury survivors (among others) into the least B
brain injury. The law allowed the Health Resources and restrictive setting possible. To aid state agencies working
Services Administration (HRSA) to develop a grant toward compliance, the BIAA partnered with Indepen-
program for states to aid in the treatment of persons dent Living Research Utilization to provide regional train-
with TBI. The bill authorized appropriations to the Na- ing workshops regarding the content of the Olmstead
tional Institutes of Health (NIH) for TBI research and to decision, its intended effect, and potential strategies to
the Centers for Disease Control and Prevention (CDC) meet the community needs of individuals with cognitive
for a public information campaign regarding the conse- disabilities who might otherwise be wrongly placed in
quences and prevention of brain injury. The ability to institutional settings.
achieve successive appropriation bills has been due in The BIAA in coordination with the Brain Trauma
part to the work of the BIAA and others to persuade Foundation, the American Association of Neurological
approximately 100 members of Congress to join the Surgeons, and other professional contributors, developed
Congressional Brain Injury Task Force. The task force was and published Guidelines for the Management of Severe
established in 2001 to provide guidance to Congress and Brain Injury (2000). The BIAA was also involved in
other federal agencies regarding policies relevant to the care authoring Management and Prognosis of Penetrating
and treatment of those with traumatic brain injury. Brain Injury (Aarabi et al. 2001). These publications
In 1992, the BIAA was integral in shaping the Defense were created to provide up-to-date, evidence-based
and Veterans Head Injury Program, later renamed the guidelines and protocols to improve the outcome of TBI
Defense and Veterans Brain Injury Center (DVBIC). patients.
This organizations mission is to develop and provide As part of its ongoing legislation efforts to increase
advanced TBI evaluation and treatment for veterans as private insurance, Medicare, and TriCare coverage for
well as active duty military personnel and their depen- cognitive rehabilitation, the BIAA published Cognitive
dents. The program has lead to the development of six Rehabilitation: The Evidence, Funding, and Case for
additional Department of Defense and Veterans Affairs Advocacy of Brain Injury (Katz, Ashley, OShanick, and
treatment sites that offer specialized care for military Connors, 2006), which included ten recommendations to
survivors of brain injury. The DVBIC includes a number increase access and delivery of cognitive rehabilitation
of collaborating partners to provide medical care, clinical services across the nation.
research, and brain-injury education. It was named the In 1996, the BIAA founded the American Academy for
TBI operational component of the recently established the Certification of Brain Injury Specialists (AACBIS)
Defense Centers of Excellence for Psychological Health which to date has certified 2,800 members. The mission
and Traumatic Brain Injury. of AACBIS is to improve the care provided to individuals
In 1999, the US Supreme Court decided the case of with brain injury through enhanced education and train-
L.C. & E.W. vs. Olmstead. Justice Ruth Bader Ginsburg ing of their health care providers. Training is provided by
delivered the opinion of the Court, holding that under volunteers and based primarily upon The Essential Brain
Title II of the Americans with Disabilities Act states are Injury Guide (American Academy for the Certification of
required to place persons with mental disabilities in Brain Injury Specialists, 2007), which was also published
community settings rather than in institutions when the by the BIAA. Certification is granted to those with appro-
States treatment professionals have determined that com- priate work experience who have successfully completed
munity placement is appropriate, the transfer from the training and written examination.
institutional care to a less restrictive setting is not opposed
by the affected individual, and the placement can be
reasonably accommodated, taking into account the Major Activities
resources available to the State and the needs of others
with mental disabilities. Although the case was not spe- The BIAA has demonstrated a long-term commitment to
cific to brain-injury survivors, the ruling allowed the shaping public policy and partnering with governmental
BIAA and others to advocate for these individuals, many agencies. It has repeatedly worked to preserve and expand
of whom had been inappropriately placed in psychiatric rehabilitation options for persons with brain injury,
hospitals or other institutional settings that were not particularly Medicare and Medicaid beneficiaries. It has
designed for their unique needs. The ruling tasked the worked to secure federal funding for research and public
440 B Brain Plasticity
education. The BIAA remains active in disability advocacy Defense and Veterans Brain Injury Center. (n.d.). Defense and veterans
brain injury center: Home of defense and veterans head injury
and has provided consultation and assistance in develop-
program. Retrieved December 20, 2008, from http://www.dvbic.org
ing numerous legislative proposals that benefit those who Katz, D. I., Ashley, M. J., OShanick, G. J., & Connors, S. H. (2006).
have sustained brain injury. Encouraging private/public Cognitive rehabilitation: The evidence, funding, and case for advocacy
partnerships, particularly in the care of military service- of brain injury. McLean, VA: Brain Injury Association of America.
related brain injury, has been a crucial area of interven-
tion. Public policy initiatives that enhanced trauma care,
child abuse prevention, transportation safety, education,
and respite care were among the associations 2007 Brain Plasticity
accomplishments (G. Ayotte, personal communication,
November, 2008) J ENNIFER S UE K LEINER
The BIAA views brain injury prevention and aware- University of Arkansas for Medical Sciences
ness as a primary component of its mission. The associa- Little Rock, AR, USA
tion has distributed information kits, produced public
service announcements, and provided access to subject
matter experts for a number of media outlets. The BIAA Definition
also publishes TBI Challenge!, a quarterly newsletter with
a distribution that includes 25,000 households. The BIAA Plasticity refers to the brains ability to change its struc-
hosts/co-hosts a number of educational meetings and ture in response to development, the environment, or
conferences, maintains a comprehensive website, and injury.
publishes the National Directory of Brain Injury Rehabil-
itation Services to provide survivors and/or family mem- Current Knowledge
bers with helpful information about services in their
community. On an annual basis the BIAA responds to Brain plasticity, or neuroplasticity, refers to the brains
over 100,000 requests for assistance through either its ability to change in response to development, to the
national information call center or its website. Each environment (including learning), and in response to
March the BIAA circulates 10,000 information kits to injury or aging. While it was once conceptualized that
mark Brain Injury Awareness Month. once the brain ceases development, that it would then be
resistant to change, or in effect be static. Research over the
last several decades has demonstrated that the brain con-
Cross References tinues to be capable of change, or restructuring, through-
out the life span. While much research with respect to
Traumatic Brain Injury brain plasticity focuses on outcomes following injury,
brain plasticity also refers to developmental changes that
occur in the brain throughout the life span, including
References and Readings synaptic changes that occur in response to the acquisition
of new learning and memories. As such, plasticity is seen
Aarabi, B., Alden, T. D., Chestnut, R. M., Downs J. H., Ecklund, J. M.,
as an intrinsic property of the central nervous system in
Eisenberg, H. M., et al. (2001). Management and prognosis of
penetrating brain injury. Journal of Trauma, Injury, Infection and
that the brain is constantly restructuring and reorganizing
Critical Care, 51, S1S86. in response to new learning. Specifically, studies of
American Academy for the Certification of Brain Injury Specialists learning new behaviors, such as learning Braille, result in
(2007). The essential brain injury guide (4th ed.). McLean, VA: the rapid, but transient onset of cortical enlargement that
Brain Injury Association of America.
gives way to a more stable but less dramatic cortical
Brain Injury Association of America. (n.d.). Brain injury association USA
home page. Retrieved December 20, 2008, from http://www.biausa.
enlargement associated with plasticity.
org Research has demonstrated that a variety of factors
Brain Injury Association of America (2005). Academy of certified brain affect plasticity of neural reorganization and proliferation.
injury specialists (ACBIS). Retrieved December 20, 2008, from http:// One such environment includes the richness of the envi-
www.aacbis.net/index.html
ronment in which an organism is grown. Specifically,
Brain Trauma Foundation, American Association of Neurological
Surgeons, Joint Section on Neurotrauma and Critical Care (2000).
organisms that grow in a richly stimulating environment
Guidelines for the management of severe traumatic brain injury. in which a variety of experiences are encountered have
Journal of Neurotrauma, 17, 451627. greater plasticity than individuals who are reared in less-
Brain Reserve B 441
stimulating environments. Empirical experimentation in loss of a large area of the brain. The learning of alternative
humans and animals alike have demonstrated that the communication techniques, such as Braille, requires the
dendrite length as well as the density of synapses in adaptation of new behavior; research has demonstrated B
organisms with enriched motor and sensory environ- that individuals who have learned Braille have larger
ments surpass those raised in less-stimulating environ- sensory maps related to the finger pad used in reading
ments. However, these differences appear only to exist as compared to the contralateral equivalent or as com-
with early learning environments, as adolescents and pared to individuals who do not read Braille. Further-
adults show no such sensitivity to environmental factors. more, not only do blind individuals develop enlarged
Both gender and hormone differences also appear to play corresponding sensory maps, it has also been demon-
a role in neural plasticity, with specific respect to cortical strated that the occipital, or visual, cortex is subsequently
areas; for example, while males are more sensitive to recruited for tactile information processing, as well as
experience related to the visual cortex, females are more auditory information processing.
sensitive to development in the hippocampal area. The advancement of technology has furthered our
However, the concept of brain plasticity may be best understanding of the existence of, and mechanisms be-
understood by examining the processes by which the hind, neuroplasticity. The ability to specify and character-
brain changes in response to injury. Responses to injury ize brain function via visualization of glucose and oxygen
may result in the loss of a previously held behavior, release metabolism has allowed for exploration of mechanisms of
of a previously suppressed behavior, the assumption of a plasticity. Processes of functional neuroimaging, includ-
function by a neighboring neuronal network, or the de- ing positron emission tomography as well as functional
velopment of new behaviors (which may be adaptive or magnetic resonance imaging allows for indirect visualiza-
maladaptive). Physiologically, reorganization can occur tion of synaptic activity; experimentation involving tasks
by changing the balance between excitatory and inhibito- being performed while the brain is being imaged allows
ry synaptic and membrane responses as well as by for examination of synaptic changes. Magnetic resonance
strengthening or weakening synaptic connections. Brain spectroscopy is thought to be a promising technique that
plasticity can also involve the growth of new dendrites and allows for analysis of the connection between neurochem-
axons to form new synaptic connections. Research sug- ical changes and behaviors. Electroencephalography and
gests that the age of onset of the injury is critical in the magnetoencephalography allows to direct measurement
development of new connections, as long connections are of neuronal activity; however, it lacks structural specifici-
more difficult to form in the mature brain, whereas the ty. Transcranial magnetic stimulation also allows for di-
young brain may be more capable of forming long con- rect analysis of neural activity by temporarily suppressing
nections due to the existence of excess connectivity. brain regions, allowing for direct assessment of brain-
Changes in connectivity can occur through the strength- behavior relationships in conjunction with functional
ening or weakening of synaptic density or the rearrange- neuroimaging techniques.
ment of synaptic connections. While the concept of
neuroplasticity does at times result in recovery of adaptive References and Readings
behavioral changes, plasticity may also lead to unmasking
of previous suppressed and maladaptive behaviors as well Galaburda, A., & Pascual-Leone, A. (2003). Mechanisms of plasticity and
as the development of dysfunctional behaviors. behavior. In T. E. Feinberg & M. J. Farah (Eds.), Behavioral neurology
Brain plasticity may occur via multiple difference & neuropsychology (2nd ed., pp. 5770). New York, NY: McGraw-
Hill.
mechanisms. Perhaps the most common, and best under-
Kolb, B. (1995). Brain plasticity and behavior. Mahwah, NJ: Erlbaum.
stood, mechanism includes the expansion of a specified Kolb, B., Gibb, R., & Robinson, T. E. (2003). Brain plasticity and behavior.
area of circuitry or the recruitment of either a local or Current Directions in Psychological Science, 12(1), 15.
distal area of circuitry. Such reorganization of function is Kolb, B., & Whishaw, I. Q. (1998). Brain plasticity and behavior. Annual
a common post-injury response and occurs shortly fol- Review of Psychiatry, 49, 4364.
Nelson, C. A., & Luciana, M. (2001). Handbook of developmental cognitive
lowing the injury and continues to develop years follow-
neuroscience. Boston, MA: Massachusetts Institute of Technology.
ing the injury as the organism adapts. Remodeling or
reorganization occurs both at the cortical and subcortical
level, and can occur both within and between functional
modalities. For example, much literature exists examining Brain Reserve
the reorganization of neuronal circuitry in response to
blindness. Early onset blindness results in the functional Cognitive Reserve
442 B Brain Reserve Capacity
Current Knowledge
Brain Reserve Capacity
Research consistently demonstrates that the underlying
G LEN E. G ETZ neuropathology is not consistent with behavioral distur-
Allegheny General Hospital bance caused by dementia. Brain reserve capacity partially
Pittsburgh, PA, USA explains this phenomenon. It appears that symptomatic
behaviors are less likely to be prevalent in individuals
with greater brain reserve capacity. Research has also
Synonyms consistently found that cognitive reserve capacity, that is
the lifestyle approaches that encourage cognitive activity,
Global reserve; Reserve plays an important role in functional ability despite neu-
ropathological changes. It appears plausible that brain
reserve capacity, such as increased amount of neurons
Definition and neuronal connections, is at least in part due to beha-
viors that encourage cognitive reserve capacity, such as
Brain reserve capacity is the brains resilience to patho- education and occupation. The interplay of cognitive
logical damage or changes. The greater the brain reserve activity and brain reserve capacity is being carefully
capacity, the less likely an individual will demonstrate studied in an attempt to understand the relationship.
behavioral disturbance associated with a disease.
Future Directions
Historical Background Identifying factors that increase the likelihood of brain
reserve capacity has the possibility of being an invaluable
Research has attempted to understand the role of various tool toward improving the quality of elderly peoples
factors involved in cognitive decline. Frequent central lives and potentially reducing the risk of developing
nervous system disorders occur in the elderly, which Alzheimers disease. The National Institute of Aging and
increase the likelihood of cognitive decline. Age, in itself, other federally funded programs have invested millions of
is a factor known to alter cognitive functioning. Brain dollars to better understand the factors in improving
reserve capacity is the brains ability to effectively manage brain reserve capacity. Studies will continue to better
the increasing changes in normal aging and to cope understand factors that increase brain cells, synaptic
with pathological damage. connections, and other neurophysiological markers.
Post-mortem examination of elderly individuals The implementation and advancement of technology
provides evidence that that there is a discrepancy between will assist in providing a clearer understanding of these
the clinical manifestation of Alzheimers disease and the factors as well.
neuropathology of the disorder (Katzman et al., 1998).
Specifically, this early study found that a subset of
individuals whose brains were found to have a high
degree of pathology associated with Alzheimers disease
Cross References
did demonstrate minimal clinical symptoms associated
Alzheimers Disease
with the disease. Interestingly, the results from this study
Cognitive Reserve
suggested that the weight of the brains in this subset of
patients was higher. These patients were also found to
have more neurons. It was subsequently concluded that
perhaps these patients larger brains and their possession References and Readings
of more neurons were protective against dementia symp-
toms. While subsequent studies have been inconclusive, Katzman, R., Terry, R., DeTeresa, R., Brown, T., Davies, P., Fuld, P., et al.
many studies have suggested that head circumference, (1988). Clinical, pathological and neurochemical changes in demen-
tia: a subgroup with preserved mental status and numerous neocor-
brain volume, intracranial volume, and genetic influ-
tical plaques. Annals of Neurology, 23, 138144.
ences also play an important role in brain reserve Stern, Y. (2006). Cognitive Reserve, Theory and Application. Series Editor
capacity. L. Bieliauskas. New York, NY: Psychology Press.
Brain Tumor B 443
Hemodynamic Response B
Cross References
Edema
Intracranial Pressure
Brain Swelling
References and Readings
B ETH R USH
Mayo Clinic Marmarou, A. (2007). A review of progress in understanding the
Jacksonville, FL, USA pathophysiology and treatment of brain edema. Neurosurgery
Focus, 22, E1.
Definition
Brain Tumor
Expansion of the size of the brain that occurs following
head trauma and brain injury. E THAN M OITRA
Drexel University
Philadelphia, PA, USA
Current Knowledge
Current Knowledge
Brainstem Auditory Evoked BAER results are generally not affected by the effect of
Responses anesthesia and medications, or peripheral vestibular
pathology. BAER is sometimes used for prognostic
F LORA H AMMOND 1, LORI G RAFTON 2 purposes after brain injury, but its use is limited for this
1
Indiana University purpose. Complete absence of responses is considered an
Indianapolis, IN, USA ominous sign (Lew et al., 2007), and abnormal BAER may
2
Carolinas Rehabilitation confirm suspicion of brainstem injury, while normal
Charlotte, NC, USA BAER simply indicates preservation of the auditory
pathways through the brain. BAER does not reveal
information about damage that may have occurred
Synonyms elsewhere in the brain, and thus, normal BAER does not
necessarily predict good outcome (Lew et al., 2007;
Auditory brainstem responses (ABR); Auditory brainstem Zafonte, Hammond, & Peterson, 1996).
response audiometry; Auditory evoked response (AER);
Brainstem auditory evoked potentials (BAEP); BAER; Cross References
Brainstem response (BSR)
Evoked Potentials
Definition
References and Readings
Brainstem auditory evoked responses (BAER) test the
function of the auditory nerve and auditory pathways of Huszar, L. (2006). Clinical utility of evoked potentials. eMedicine.
the brain by measuring the electrophysiologic responses Retrieved July 9, 2007 from http://www.emedicine.com/neuro/
to repeated clicks presented to each ear. The response time topic69.htm
Brainstem Strokes B 445
Lew, H. L., Lee, E. H., Pan, S. S. L., & Chiang, J. Y. P. (2007). Electrophys- one of three anatomical locations within the brain stem.
iologic assessment techniques: evoked potentials and electroen-
Pontine brainstem gliomas are associated with the poorest
cephalography. In N. D. Zasler, D. I. Katz, R. D. Zafonte (Eds.),
Brain Injury Medicine (p. 158). New York: Demos.
prognosis for survival, while tectal and cervicomedullary B
Zafonte, R. D., Hammond, F. M., & Peterson, J. (1996). Predicting gliomas are associated with longer survival. Tectal brain-
outcome in the slow to respond traumatically brain-injured patient: stem gliomas are often associated with hydrocephalus as a
Acute and subacute parameters. NeuroRehabilitation, 6, 1932. result of compression of the fourth ventricle. Typical
manifestations of cervicomedullary tumors include dys-
phagia, unsteadiness, nasal speech, and sensory loss in the
face. Pontine brainstem gliomas are associated with cra-
nial nerve or long tract symptoms, including problems
Brainstem Glioma with the control of facial muscles, ocular movements, and
swallowing. Diffuse brainstem gliomas, once thought to
R OBERT R IDER be a single entity, are now thought to comprise a group of
Drexel University tumors with varying courses and outcomes. Brainstem
Philadelphia, PA, USA gliomas can also occur in the cervicomedullary junction,
pons, midbrain, and tectum; prognosis is worse and very
grim for diffuse brainstem gliomas. Diffuse brainstem
Synonyms gliomas do not typically enhance on MRI. They are not
responsive to radiotherapy, and treatment is usually lim-
Midbrain glioma; Pontine glioma ited to chemotherapy.
Brainstem Strokes
E LLIOT J. R OTH
Northwestern University
Chicago, IL, USA
Definition
A stroke that is caused by ischemia or hemorrhage in the
Brainstem Glioma. Figure 1 Picture credit: Michael Phillips midbrain, pons, or medulla is called a brainstem stroke.
and Peter C. Fisher There are many brainstem stroke clinical syndromes, the
446 B Breathing Tube
Basilar Artery The Brief Cognitive Rating Scale (BCRS; Reisberg & Ferris,
Cerebrovascular Disease 1988) is used to assess functional and cognitive abilities in
Lacunar Infarction both normal aging and progressive dementia. The BCRS
Locked-In Syndrome is part of the Global Deterioration Scale Staging System
Posterior Cerebral Artery (GDS; Reisberg, Ferris, de Leon, Crook, 1993) which is
Posterior Communicating Artery composed of three separate rating scales that include the
Pure Motor Stroke GDS, the Functional Assessment Staging (FAST; Reisberg,
Brief Psychiatric Rating Scale B 447
1988), and the BCRS. The BCRS provides objective rat- Mini-Mental State Examination
ings of a number of domains that include various cogni- Multi-Infarct Dementia
tive functions as well as functional abilities, mood, and B
behavior, and is made up of two parts. Part I includes
References and Readings
ratings for Concentration, Recent Memory, Remote
Memory, Orientation, and Functioning and Self Care,
Foster, J. R., Sclan, S., Welkowitz, J., Boksay, I., & Seeland, I. (1988).
while Part II allows for ratings of Speech and Language Psychiatric assessment in medical long-term care facilities: Reliabili-
Abilities, Motoric Capacities, Mood and Behavior, Praxis ty of commonly used rating scales. International Journal of Geriatric
Ability, Calculation Ability, and Feeding Capacity. Psychiatry, 3, 229233.
Each of the domains is rated on a 17 point scale that Ihl, R., Frolich, L., Dierks, T., Martin, E., & Maurer, K. (1992). Differential
validity of psychometric tests in dementia of the Alzheimer type.
ranges from normal (rating of 1) to profound im-
Psychiatry Research, 44, 93106.
pairment (rating of 7). For each domain, a behavioral Reisberg, B. (1988). Functional assessment staging (FAST). Psychophar-
anchor is provided for each point on the rating scale. The macology Bulletin, 24, 653659.
authors provided examples of questions that might be Reisberg, B., & Ferris, S. H. (1988). Brief cognitive rating scale (BCRS).
used to elicit information needed to complete the BCRS Psychopharmacology Bulletin, 24, 629636.
Reisberg, B., Ferris, S. H., de Leon, M. J., & Crook, T. (1988). Global
as well as guidelines for scoring each domain. Ratings are
deterioration scale (GDS). Psychopharmacology Bulletin, 24,
completed based on interviews with the patient and an 661663.
informant who is knowledgeable regarding the patients Reisberg, B., Ferris, S. H., Steinberg, G., Shulman, E., de Leon, M. J., &
day-to-day activities and functioning. Interviews may Sinaiko, E. (1989). Longitudinal study of dementia patients and aged
be conducted in person or over the telephone. The controls. In M. P. Lawton, & A. R. Herzog (Eds.), Special research
methods for gerontology (pp. 195231). Amityville, NY: Baywood.
BCRS has been translated into a number of languages
Reisberg, B., Sclan, S. G., de Leon, M. J., Kluger, A., Torossian, C., Shulman,
including Chinese, French, Polish, Spanish and Swedish, E., Steinberg, G., Monteiro, I., McRas, T., Mackell, J., & Ferris, S. H.
among others. (2007). The GDS staging system. In A. J. Rush (Ed.), Handbook of
psychiatric measures (pp. 431435). Washington, DC: American Psy-
chiatric Publishers.
Current Knowledge
assessing the following psychiatric symptoms: somatic con- (1988). Versions are also widely available online, includ-
cern, anxiety, emotional withdrawal, conceptual disorgani- ing at: http://www.public-health.uiowa.edu/icmha/out-
zation, guilt feelings, tension, mannerisms and posturing, reach/documents/BPRS_expanded.PDF.
grandiosity, depressive mood, hostility, suspiciousness, hal-
lucinatory behavior, motor retardation, uncooperativeness,
unusual thought content, blunted affect, excitement, and
Cross References
disorientation. The instrument takes approximately 510
min to complete, following an interview with the patient.
Affective Disorder
The clinician rates each item on a scale ranging from 1 (not
Alzheimers Dementia
present) to 7 (extremely severe). The inventory is geared
Alzheimers Disease
toward serious psychopathology. While the ratings on
Anxiety
individual items are meaningful, the BPRS can yield an
Clinical Interview
overall score, and sets of items can be grouped into
Dementia with Lewy Bodies
categories.
Parkinsons Dementia
Parkinsons Disease
Psychosis
Current Knowledge
Psychotic Disorder
Structured Clinical Interview for DSM-IV (SCID)
The BPRS has acceptable psychometric properties and
Traumatic Brain Injury
enjoys widespread clinical use. Several authors have devel-
oped versions of the BPRS with behavioral anchors to
improve reliability, or training methods to accomplish the
same (Ventura, Green, Shaner, & Liberman, 1993). Using a References and Readings
behaviorally anchored approach and no specific interview
format or rater training, Lachar et al. (2001) obtained Cummings, K. R., Raman, R., & Thai, L. (2007). Quetiapine for agitation
weighted kappa agreement between psychiatrists on the or psychosis in patients with dementia and parkinsonism. Neurology,
68, 13561363.
majority of items at above 0.75. Scores on the BPRS are
Devanand, D. P. (1998). A randomized, placebo-controlled dose-
highly correlated with those of other similar instruments comparision of a trial of haloperidol for psychosis and disruptive
although, as with all such clinician-rated scales, relation- behaviours in Alzheimers disease. American Journal of Psychiatry,
ships with external criteria are modest (Mortimer, 2007). 155, 15121520.
Initially developed for use with psychiatric populations, Dunayevich, E., Sethuraman, G., Enerson, M., Taylor, C. C., & Lin, D.
(2006). Characteristics of two alternative schizophrenia remission
the BPRS is one of the most common scales used to track
definitions: Relationship to clinical and quality of life outcomes.
outcomes in schizophrenia research (Dunayevich, Sethura- Schizophrenia Bulletin, 86, 300308.
man, Enerson, Taylor, & Lin, 2006). It has been used Lachar, D., Bailley, S. E., Rhoades, H. M., Espandas, A., Aponte, M.,
effectively with individuals having other psychiatric dis- Cowan, K. A., et al. (2001). New subscales for an anchored version
orders (e.g., bipolar disorder; Picardi et al., 2008), as well of the Brief Psychiatric Rating Scale: Construction, reliability and
validity in acute psychiatric admissions. Psychological Assessment, 13,
as those with neurological conditions having psychotic or
384395.
other psychiatric symptoms, including Alzheimers dis- Levin, H. S., High, W. M., Goethe, K. E., Sisson, R. A., Overall, J. E.,
ease, dementia with Lewy bodies, and Parkinsons disease Rhoades, H. M., Eisenberg, H. M., Kalisky, Z., & Gary, H. E. (1987).
(Cummings, Raman, & Thai, 2007; Devanand, 1998; Tar- The neurobehavioural rating scale: Assessment of the behavioural
iot, Profenno, & Ismail, 2004). It has also been used in a sequelae of head injury by the clinician. Journal of Neurology, Neuro-
surgery and Psychiatry, 50, 183193.
modified form with persons with traumatic brain injury
Mortimer, A. M. (2007). Symptom rating scales and outcome in schizo-
(Levin et al., 1987). In neuropsychological practice, the phrenia. British Journal of Psychiatry Supplement, 50, s7s14.
BPRS can enable the clinician to organize and quantify Overall, J. E., & Gorham, D. R. (1962). The brief psychiatric rating scale.
observations of psychotic symptoms or other seriously Psychological Reports, 10, 790812.
disordered behavior, both as part of an evaluation and Overall, J. E., & Gorham, D. R. (1988). The Brief Psychiatric Rating Scale
(BPRS): Recent developments in ascertainment and scaling. Psycho-
in tracking the course of the clinical condition over time.
pharmacology Bulletin, 24, 9799.
The BPRS is in the public domain, and may be found Picardi, A., Battisti, F., de Girolamo, G., Morosini, P., Norcio, B., Bracco,
in Overall & Gorham (1988). A behaviorally anchored R., et al. (2008). Symptom structure of acute mania: A factor study of
format may be found in Woemer, Mannuzza, and Kane the 24-item Brief Psychiatric Rating Scale in a national sample of
Brief Symptom Inventory B 449
patients hospitalized for a manic episode. Journal of Affective Dis- be hand- or computer-administered to individuals ages 13
orders, 108, 183189.
and older (BSI) or 18 and older (BSI-18). Respondents
Tariot, P. N., Profenno, L. A., & Ismail, M. S. (2004). Efficacy of atypical
antipsychotics in elderly patients with dementia. Journal of Clinical
rate the extent to which a specific problem has distressed B
Psychiatry, 65(Suppl. 11), 1115. them in the past 7 days (although evaluations over other
Ventura, J., Green, M. F., Shaner, A., & Liberman, R. P. (1993). time intervals may be specified), using a 5-point scale
Training and quality assurance on the Brief Psychiatric Rating (0 = not at all to 4 = extremely). Administration is
Scale. International Journal of Methods in Psychiatric Research, 3,
straightforward, taking 4 (BSI-18) to 810 min (BSI) to
221244.
Woerner, M. G., Mannuzza, S., & Kane, J. M. (1988). Anchoring the
complete.
BPRS. Psychopharmacology Bulletin, 24, 112117. Scores are determined by summing values for each
symptom dimension and then dividing by number of
items endorsed in the respective dimension. A GSI can
be calculated for either measure by adding the scores for
all subscales, as well as the additional items (in the BSI),
Brief Symptom Inventory and then dividing by number of responses. For the BSI
only, the PST is determined by counting the number of
J OSEPH F. R ATH , L ISA M. F OX items endorsed with a positive (nonzero) response, and
NYU Langone Medical Center the PSDI is derived by dividing the sum of the item
New York, NY, USA values by the PST. Raw scores can be converted to
standardized T scores, generating a profile that graphi-
cally illustrates a respondents current psychological
Synonyms symptom presentation. Interpretation of the BSI can
be done at three levels: global scores, primary symptom
BSI; BSI-18 dimensions, and discrete symptoms (Derogatis &
Melisaratos, 1983). Computer administration, scoring,
and interpretation programs are available for both
Description instruments.
Advantages of the BSI and the BSI-18 are that they can
The BSI (Derogatis & Melisaratos, 1983) is a shortened, be completed quickly and used for repeated assessments.
53-item version of the Symptom Checklist-90 (SCL-90; Also, both measures are reported to be sensitive to mild-
Derogatis, Lipman, & Covi, 1973) that measures to-severe psychological distress, making them useful with
emotional-behavioral functioning in nine dimensions: many populations. Limitations include typical concerns
somatization, obsessivecompulsive, interpersonal sensi- associated with self-report measures, such as possible
tivity, depression, anxiety, hostility, phobic anxiety, para- patient response bias and over or underreporting, as well
noid ideation, and psychoticism. Four additional items as limited utility with some medical populations (e.g.,
not specific to any one domain load on several different neurologic), given the paucity of acceptable norms and
dimensions. In addition, three global indices Global concerns that certain items on the BSI may be closely
Severity Index (GSI), Positive Symptom Total (PST), related to physical and cognitive symptoms (Slaughter,
and Positive Symptom Distress Index (PSDI), provide Johnstone, Petroski, & Flax, 1999).
more general assessment of psychological well-being.
The BSI-18 (Derogatis, 2000), an 18-item shortform of
the BSI intended as a screen for psychiatric disorders and
psychological distress, consists of three six-item subscales: Historical Background
somatization, depression, and anxiety. A GSI also can be
calculated. In response to the need for briefer evaluation tools rele-
Not intended for use as diagnostic tools, both instru- vant in a variety of assessment settings, including medical
ments are designed to identify psychological symptoms and industrial research studies, the BSI and BSI-18 were
in medical populations, psychiatric patients, and commu- derived from the SCL-90, a 90-item checklist that includes
nity non-patients, with separate norms for males and the same nine symptom dimensions and three global
females. Written at a sixth grade reading level and avail- indices as the BSI. The SCL-90, itself, was developed in
able in over 24 languages, these self-report measures can 1973 (Derogatis et al., 1973) and was derived from the
450 B Brief Symptom Inventory
Hopkins Symptom Checklist (Derogatis, Lipman, Rickels, Lindner, 1988) found the BSI to be a strong and reliable
Uhlenhuth, & Covi, 1974). The BSI was published in 1983 predictor of psychological distress; whereas, the BSI-18
(Derogatis & Melisaratos, 1983), and the BSI-18 followed has been demonstrated to predict levels of distress in
in 2000 (Derogatis, 2000). cancer patients (e.g., Recklitis, Parsons, Shih, Mertens, &
Robison, 2006).
Some concern has been raised about the factor struc-
Psychometric Data ture and discriminant validity of the BSI and BSI-18.
For example, Boulet and Boss (1991) found that BSI
The most recent BSI manual (Derogatis, 1993) provides subscales correlated with nonanalogous scales on the
normative data from four samples: (1) 1,002 heteroge- MMPI, suggesting poor discriminant validity. In terms of
neous psychiatric outpatients; (2) 914 adult non-patients; factor structure, moderate to high intercorrelations were
(3) 423 psychiatric inpatients; and (4) 2,408 adolescent found among BSI subscales, with one factor explaining
non-patients. The BSI-18 was normed on two samples: over 70% of the variance in a principal-components anal-
(1) 1,134 community adults and (2) 1,543 adult cancer ysis (Boulet & Boss, 1991). Several cross-cultural studies
patients. Additional norms have been developed for such of the BSI-18 (e.g., Asner-Self, Schreiber, & Marotta,
diverse samples as older adults (Hale, Cochran, & Hedge- 2006) found only the GSI (versus the three subscales) to
path, 1984), college students (Cochran & Hale, 1985), be a valid indicator of psychological distress. These
individuals with spinal cord injury (Heinrich, Tate, & researchers suggested that the BSI and BSI-18 assess the
Buckelew, 1994), British psychiatric outpatients (Ryan, degree, but not the exact nature of psychopathology,
2007) and community-dwelling adults (Francis, Rajan, & and therefore GSI scores should be considered the most
Turner, 1990), Israeli adolescents (Canetti, Shalev, & De- useful indicator of psychological distress derived from the
Nour, 1994), and others (see Derogatis, 1993). Internal measures.
consistency coefficients are strong for both the BSI (0.71
0.83) and BSI-18 (0.740.89). Testretest reliability also is
good, ranging from 0.68 to 0.91 (BSI) and 0.68 to 0.84 Clinical Uses
(BSI-18).
BSI and BSI-18 manuals cite a variety of studies sup- The BSI and BSI-18 are widely used measures of psycholog-
porting validity in a range of settings and populations, ical distress employed with a variety of populations includ-
including psychoneuroimmunology, psychopathology, ing inpatient and outpatient medical and psychiatric
pain assessment and management, HIV research, student patients, individuals receiving treatment for substance
mental health, and general clinical studies. For example, abuse, and college students. Individuals who are of extreme-
in symptomatic adults, convergent validity between the ly low intelligence, delirious, psychotic, or have motivation
BSI and the Minnesota Multiphasic Personality Inventory to distort their responses are not good candidates for either
(MMPI) was shown to be 0.300.72 (Derogatis & Meli- measure. Given factor-structure concerns noted above (e.g.,
saratos, 1983) for the Wiggins content scales (i.e., 13 scales Boulet & Boss, 1991), the instruments may be used most
tapping content areas such as social maladjustment and appropriately as screening tools to alert clinicians to ele-
authority conflict) and Tryon cluster scales (i.e., seven scales vated levels of psychological distress, rather than as diag-
assessing conceptual clusters such as social introversion and nostic indicators. According to the BSI manual
bodily symptoms). Convergent validity also has been (Derogatis, 1993), the measures are most useful in clini-
demonstrated between the BSI and several other scales in cal and research settings where time is a major limiting
predicting affective status among chronic pain patients variable.
(Kremer, Atkinson, & Ignelzi, 1982). High correlations Both measures can be used as onetime assessments or
were also found between the SCL-90 and the BSI (0.92 administered repeatedly to evaluate treatment efficacy or
0.96; Derogatis, 1993) and BSI-18 (0.910.96; Derogatis, trends over time. Both are reported to have been used
2000). In terms of predictive validity, the BSI has been successfully in primary care settings to assess significant
shown to be a good predictor of psychopathology in changes in psychological distress and symptoms in
several populations, including a community unipolar de- patients with medical problems. The measures can be
pression cohort (Amenson & Lewinsohn, 1981), drug- used in nonclinical populations (e.g., to assess caregiver
using adults (Buckner & Mandell, 1990), and the elderly distress) as well.
(Hale et al., 1984). Screening studies completed with Both the BSI and BSI-18 may be useful tools for
medical cohorts (e.g., Kuhn, Bell, Seligson, Laufer, & inclusion in neuropsychological assessments, given their
BRIEF-P B 451
brevity and utility for repeated administrations. The BSI- Cochran, C. D., & Hale, W. D. (1985). College student norms on the Brief
Symptom Inventory. Journal of Clinical Psychology, 41, 777779.
18 was reported to be a valid screening measure for the
Derogatis, L. R. (1993). Brief Symptom Inventory (BSI): Administration,
overall level of psychological distress in both inpatients scoring, and procedures manual (3rd ed.). Minneapolis, MN: National B
and outpatients with traumatic brain injury (Meachen, Computer Systems.
Hanks, Millis, & Rappaport, 2008). However, the obser- Derogatis, L. R. (2000). Brief Symptom Inventory-18 (BSI-18): Adminis-
vation that most items in the BSI obsessivecompulsive tration, scoring, and procedures manual. Minneapolis, MN: National
Computer Systems.
scale are more reflective of cognitive complaints (e.g.,
Derogatis, L. R., Lipman, R. S., & Covi, L. (1973). SCL-90: An outpatient
concentration and memory problems) than classic obses- psychiatric rating scale: Preliminary report. Psychopharmacology
sivecompulsive disorder traits (Slaughter et al., 1999) Bulletin, 9, 1328.
highlights an issue of particular concern to neuropsychol- Derogatis, L. R., Lipman, R. S., Rickels, K., Uhlenhuth, E. H., & Covi, L.
ogists. Given the overlap between many items and cogni- (1974). The Hopkins Symptom Checklist (HSCL): A self-report
inventory. Behavioral Science, 19, 115.
tive and physical symptoms, clinicians are urged to
Derogatis, L. R., & Melisaratos, N. (1983). The Brief Symptom Inventory:
interpret elevations cautiously and remain vigilant against An introductory report. Psychological Medicine, 13, 595605.
misusing scale names for diagnostic purposes. It is crucial Francis, V. M., Rajan, P., & Turner, N. (1990). British community norms
not to rely on psychiatric interpretations of elevated scales for the Brief Symptom Inventory. British Journal of Clinical Psychol-
in neurological patients who have no history of emotional ogy, 29, 115116.
Hale, W. D., Cochran, C. D., & Hedgepath, B. E. (1984). Norms for the
difficulties; instead, BSI and BSI-18 item responses
elderly on the Brief Symptom Inventory. Journal of Clinical and
might best be examined individually and used to guide Consulting Psychology, 52, 321322.
treatment. Heinrich, R. K., Tate, D. G., & Bucklew, S. P. (1994). Brief Symptom
Inventory norms for spinal cord injury. Rehabilitation Psychology, 39,
4956.
Kremer, E. F., Atkinson, J. H., & Ignelzi, R. J. (1982). Pain measurement:
Cross References The affective dimensional measure of the McGill Pain Questionnaire
with a cancer pain population. Pain, 12, 153163.
Beck Anxiety Inventory Kuhn, W. F., Bell, R. A., Seligson, D., Laufer, S. T., & Lindner, J. E. (1988).
Beck Depression Inventory The tip of the iceberg: Psychiatric consultations on an orthopaedic
Brief Psychiatric Rating Scale service. International Journal of Psychiatry in Medicine, 18,
375382.
Center for Epidemiologic Studies-Depression
Meachen, S. J., Hanks, R. A., Millis, S. R., & Rapport, L. J. (2008). The
Geriatric Depression Scale reliability and validity of the Brief Symptom Inventory-18 in persons
Hamilton Rating Scale of Depression with traumatic brain injury. Archives of Physical Medicine and Reha-
Millon Clinical Multiaxial Inventory bilitation, 89, 958965.
Minnesota Multiphasic Personality Inventory Recklitis, C. J., Parsons, S. K., Shih, M., Mertens, A., & Robison, L. L.
(2006). Factor structure of the Brief Symptom Inventory-18 in adult
Personality Assessment Inventory
survivors of childhood cancer: Results from the childhood cancer
Self-Report Measures survivor study. Psychological Assessment, 18, 2232.
Symptom Checklist-90-Revised Ryan, C. (2007). British outpatient norms for the Brief Symptom Inven-
Zung Self-Rating Depression Scale tory. Psychology and Psychotherapy: Theory, Research, and Practice,
80, 183191.
Slaughter, J., Johnstone, G., Petroski, G., & Flax, J. (1999). The usefulness
of the Brief Symptom Inventory in the neuropsychological evalua-
References and Readings tion of traumatic brain injury. Brain Injury, 13, 125130.
pharmaceutical and psychosocial interventions in people Woods, R. T., Bruce, E., Edwards, R. T., Hounsome, B., Keady, J.,
Moniz-Cook, E. D., et al. (2009). Reminiscence groups for people
with dementia. Recent examples include open-label and
with dementia and their family carers: Pragmatic eight-centre
controlled trials on the safety of aspirin (AD2000 Collab- randomised trial of joint reminiscence and maintenance versus B
orative Group, 2008) and neuroleptic treatments (Ballard usual treatment: A protocol. Trials, 10, 64.
et al., 2008) in people with AD, a comparison of cholines-
terase inhibitor and glutamate agonist treatment in mod-
erate-severe AD (Jones et al., 2009), and RCTs of
reminiscence therapy (Woods et al., 2009) and interper-
Bristol ADL Scale (BADLS)
sonal psychotherapy (Burns et al., 2005) for people with
Bristol Activities of Daily Living Scale
Azheimers disease and other dementias. Bucks and
Haworth (2002) have noted that completing the question-
naire may be in itself helpful for caregivers, as it can help
them to understand the effects of dementia in real-life Broadmann Area 17
terms.
Cuneus
Cross References
Major Appointments cancer cells can penetrate the venous and lymphatic
systems as they metastasize. In the early 1850s, he
Following the completion of his medical studies, performed the first experiment in Europe using hyp-
Broca served as a lecturer at the Faculty of Medicine notism as surgical anesthesia.
in Paris. He lectured on topics in anatomy and surgery Broca was a prolific writer. He wrote several medical
until a formal competition for a professorship at the classics early in his career, including an extensive
Faculty of Medicine opened in 1853. That year, at the treatise on brain aneurysms, Des aneurysms et de leur
age of 29, Broca successfully competed to achieve the traitment, published in 1856 and a memoir on cancer,
distinction of Professor Agrege and Chirurgien du Memoir sur lanatomie pathologique du cancer, pub-
Bureau Central (also known as Chirurgien des Hopi- lished in 1853.
taux or Surgeon of the Hospitals) at the Faculty of
Medicine. In 1867, he was selected to chair the patho-
logie externe. Broca received the distinction of a pro- Speech and Localization of Function
fessor of clinical surgery in 1868. That same year, he
was elected a member of the Academie de Medicine, Brocas most well-known observations were made in
and resigned his chair of the pathologie externe in 1861 and the several years that followed. During that
order to accept the chair of Clinical Surgery. He held time, there was considerable fervor regarding the
this position until his death. During his tenure as the plausibility of cortical localization of functions, as
chair of Clinical Surgery, Broca worked in several Franz Joseph Galls early nineteenth-century phrenol-
Parisian hospitals, including the Hopital St. Antoine, ogy had fallen out of favor. Gall, based on his observa-
the Pitie, the Hotel des Cliniques, and the Hopital tions of skull shape, placed the ability to speak and
Necker. recall words in the inferior aspect of the frontal lobes.
Jean Baptiste Bouillaud followed Galls localizationist
views and hypothesized that the anterior lobes of the
Major Honors and Awards brain contained a center for speech production.
Bouillaud even offered a prize to the first individual
Broca became widely recognized throughout France
who reported a case with loss of speech without lesion
for his work. In 1865, he was elected president of the
to the frontal lobes.
Paris Societe de Chirurgie. In 1868, he was appointed
In February 1861 at a meeting of the Societe dAnthro-
to the Academie de Medicine and named a member of
pologie de Paris, a heated debate ensued when Pierre
the French Legion of Honor. He was elected to a
Gratiolet, another well-respected scientist of the time,
permanent seat in the French Senate in 1880, just
proposed total brain volume as a meaningful correlate
before his death. Broca also received many posthu-
of intelligence, and indicated that the functions of all
mous honors, including the eponymous naming of
parts of the brain were essentially identical. These
Brocas area, Brocas aphasia, and the diagonal band of
views were vehemently opposed by Bouillaud and his
Broca.
student and son-in-law Simon Alexandre Ernest
Aubertin. Broca was present during these debates as
Landmark Clinical, Scientific, the secretary of the Societe dAnthropologie. As he
and Professional Contributions considered the views presented, Broca eventually
sided with Bouillaud and Aubertin and became less
Early Career willing to accept the idea that all parts of the cerebral
hemispheres function in the same way (Finger, 2000).
Although Broca is best known for his work on speech On April 12, 1861, a Monsieur Leborgne was admitted
and the localization of brain functions, he first estab- to Brocas surgical service at the Bicetre for cellulitis
lished his reputation as a physician and scientist using and gangrene of the right leg. Affected by epilepsy since
the microscope to study diseases (Finger, 2004). He childhood, Leborgne developed considerable limita-
described in detail the histology of articular cartilage tions in his ability to speak along with right hemipar-
(the type of cartilage that covers the end of bones), the esis at age 30. He could comprehend speech and
histology of Rickets, and demonstrated that muscular communicate using pantomime, but his speech output
dystrophy is primarily a disease of the muscles. Broca was limited to the monosyllabic phrase tan, which
was among the first to use a microscope to show that became his nickname.
Broca, Pierre Paul (18241880) B 455
Leborgne died due to complications from the cellulitis for reporting on the localization of language functions,
and gangrene on April 17, 1861. Broca quickly com- although there were others who may have preceded
pleted an autopsy and presented his preliminary find- him. In 1836, Marc Dax presented a work indicating B
ings at the meeting of the Societe dAnthropologie de that disturbances in language production were due to
Paris the following day. He reported atrophy of both lesions of the left hemisphere. Daxs work remained
hemispheres of Leborgnes brain, with extensive soft- largely unknown until his son Gustave Dax presented
ening of left-frontal areas originating from the third and published his deceased fathers work in the years
left-frontal convolution. He further examined after 1863 (see Buckingham, 2006 and Finger, 2000).
Leborgnes brain specimen and presented his in-
depth findings at the Societe in August 1861. His
Surgery
presentation included a description of Leborgnes ep-
ilepsy and physical difficulties, including right-sided
Broca focused heavily on the relation between the skull
paralysis and loss of speech, along with a description
and the brain. In June 1871, he treated a 38-year-old
of the growth of a lesion from the third left-frontal
laborer who was kicked in the left-frontal region of the
convolution to other areas of the brain. Over time, the
skull by a horse. There was no fracture; nevertheless, the
area of the third left-frontal convolution became
patient showed difficulties with speech production after
known as Brocas area as a result of this case.
approximately 1 month. He eventually lost his full ability
Later in 1861, Brocas surgical service was referred a
to express himself through speech and lapsed into a coma.
Monsieur Lelong who had fractured his left femur
Broca suspected an abscess in the area of the third frontal
after a fall. Lelong was an 84-year-old man who had
convolution and performed a craniotomy at this approxi-
been admitted to the Bicetre 8 years previously for
mate location based on his hypothesis. He successfully
senility. In the spring of 1860, Lelong had suddenly
drained the abscess, but the patient slipped back into
collapsed and fallen unconscious. Upon recovery, he
coma after approximately 11 h and died. Autopsy revealed
was able to produce only four French words: oui,
a left-sided, predominantly frontal, meningoencephalitis
non, toujours, and trios, though his pantomimes
(Jay, 2002). This surgery based on Brocas findings regard-
were nearly always correct (Lee, 1981). Lelong lived
ing the localization of speech functions is likely the first
for only 12 days after he was referred to Brocas ser-
practical application of the theory of cortical localization
vice. Following Lelongs death, Broca performed an
(Finger, 2004; Stone, 1991).
autopsy and found lesions in the second and third
frontal convolutions. Presenting the findings from the
case of Lelong to the Societe dAnthropologie de Paris Anthropology
in November 1861, Broca indicated that the findings
confirmed those from his study of Leborgne, and From approximately 1866 until his death, Broca focused
hypothesized Lelongs left-frontal lesion was due to the majority of his efforts on the advancement of anthro-
an old hemorrhage that had occurred at the time pology. Indeed, due to his interest in anthropology and
Lelong lost his speech in 1860 (Lee, 1981). the remains of early humans, he did not write any papers
Broca called the inability to produce language in the at all on speech and the brain after 1877 (Finger, 2000).
context of intact comprehension, as seen in the cases Brocas initial interest in anthropology was piqued after
of Leborgne and Lelong, aphemie (Armand Trous- serving on a commission examining excavations in the
seau subsequently renamed such disturbances apha- cemetery of the Celestins in 1847. The discovery of Nean-
sia in 1864). Broca published several additional cases derthal Man in 1856, the publishing of Charles Darwins
of aphemie with lesions to the left hemisphere. For controversial ideas in On the Origin of Species in 1859,
example, in 1863, he published a series of eight cases and the subsequent controversy on the origins of man
showing primarily left-frontal lesions with language furthered Brocas desire to study anthropology.
production deficits. In an 1865 manuscript, Broca Much of Brocas anthropological research focused on
firmly asserted that the left hemisphere is the domi- the comparative study of skulls and the cranium circum-
nant seat for language production. ference across ethnic groups. He devised various instru-
Brocas declaration that the left hemisphere is predom- ments, standardized techniques and methods to examine
inantly responsible for language is among the clearest the structure and topography of the brain based on mea-
and most dramatic examples of localization of neural surements from prehistoric craniums. He invented at least
functions. Broca continues to receive the primary credit 27 instruments to determine the relation between the
456 B Broca, Pierre Paul (18241880)
brain and skull, including a goniometer (instrument to several societies, schools, and laboratories. In 1848, he
measure angles), craniograph (instrument used to depict established a society of free-thinking individuals, many
the outline of the skull), and several stereographic instru- of whom were sympathetic to Charles Darwins contro-
ments (Cowie, 2000). versial theories. He started the anthropology laboratory at
In 1869, Broca published the first description of the the Ecole des Hautes Etudes in Paris in 1858, the Societe
Gibraltar skull. Discovered in 1848, the Gibraltar skull was dAnthropologie de Paris, the first-known anthropologi-
among the earliest skeletal remains identified as belonging cal Societe in the world, in 1859, the Revue dAnthropo-
to the early species of Homo sapiens neanderthlensis. Broca logie in 1872, and the Ecole dAnthropologie in Paris in
was also fascinated with the topic of neolithic trephina- 1876. Broca held leadership positions in many of these
tion, the process, whereby a hole is scraped or drilled into societies, including secretary of the Societe Anatomique,
the skull. His interest in trephination began in 1867 after general secretary of the Societe dAnthropologie de Paris,
he examined an Incan skull with cross-hatched cuts. He president of the Societe de Chirurgie, and director of the
hypothesized that the operations were performed to treat Ecole dAnthropologie.
internal maladies in children (Finger, 2004), and was Broca married Lugol Augustine, the daughter of
among the first to speculate that trephination was a ther- Dr. Jean Guillaume Auguste Lugol, who propagated the
apeutic practice that was survived postoperatively based use of iodine in the treatment of disease, on July 6, 1857.
on the signs of inflammation at the wound margins The couple had three children, one daughter and two
(Cowie, 2000). sons. Just as Broca followed in his fathers footsteps by
Broca was very active in the study of anthropology becoming a physician, his two sons succeeded him as well-
during the final years of his life. Indeed, he was known to respected medical scientists.
spend many hours per day in the Ecole dAnthropologie he Pierre Paul Broca died on July 9, 1880 in Paris at the
founded. During the last 2 decades of his life, Broca pub- age of 56. Autopsy showed that all organs were apparently
lished over 240 papers and monographs on anthropologi- sound, although some have speculated his cause of death
cal topics (Schiller, 1992), including a five-volume work to be heart disease (Finger, 2000, 2004). Following his
entitled, Memoires dAnthropologie, published in 1871. autopsy one of his students remarked, We shall probably
not be far from the truth in attributing the catastrophe to
cerebral exhaustion, arising from too protracted a course
Short Biography of severe intellectual exertion (Memoir of Paul Broca,
1881). He was buried at the Montparnasse Cemetery in
Pierre Paul Broca was born on June 28, 1824, in Sainte- Paris. In his life, Broca published over 500 books and
Foy-la-Grande, a small town near Bordeaux, France. He articles. His influence on ideas regarding cortical localiza-
was raised under the Calvinist Protestant tradition. His tion, speech, and anthropology endures today. Indeed,
maternal grandfather, in addition to serving as mayor of many scientists would agree that the foundations of mod-
Bordeaux during the French revolution, was a pastor. His ern neuropsychology and cognitive neuroscience were
mother, Annette Thomas, was the sister of a Protestant laid by Pierre Paul Broca (Dronkers et al., 2007).
minister. His father, Jean Pierre (known as Benjamin)
Broca, was a physician who served for several years as a Cross References
surgeon in the French Army and was present at the Battle
of Waterloo (Finger, 2000). Aphasia
Following his undergraduate education, Broca sought Brocas Aphasia
to study engineering at the Ecole Polytechnique in Paris Localization
(Schiller, 1992). The death of his only sibling, a sister Speech
named Leontine, in 1840, along with pressure from his Tan
parents to remain closer to home and follow his fathers Wernickes Aphasia
career path, led to his decision to change his course of
study to medicine in 1841.
References and Readings
Broca showed considerable interest in the scientific
societies that prevailed in Paris both during his study of
Buckingham, H. W. (2006). The Marc Dax (17701837)/Paul Broca
medicine and throughout his career. He joined the Societe (18241880) controversy over priority in science: Left hemisphere
Anatomique (Anatomical Society) in 1847 and the Societe specificity for seat of articulate language and for lesions that cause
de Chirurgie (Surgical Society) in 1849. He also founded aphemia. Clinical Linguistics & Phonetics, 20, 613619.
Brocas Aphasia B 457
Clower, W. T., & Finger, S. (2001). Discovering trepanation: The con- words and lacks functors, bound morphemes, and other
tributions of Paul Broca. Neurosurgery, 49, 14171425.
grammatical elements. Paraphasic errors are also present.
Cowie, S. E. (2000). A place in history: Paul Broca and cerebral localiza-
tion. Journal of Investigative Surgery, 13, 297298.
Reading and writing performance generally mirrors that B
Dronkers, N. F., Plaisant, O., Iba-Zizen, M. T., & Cabanis, E. A. (2007). of auditory comprehension and oral expression. Some
Paul Brocas historic cases: High resolution MR imaging of the brains individuals with Brocas aphasia have agrammatism, a
of Leborgne and Lelong. Brain, 130, 14321441. lack of grammatical structure in their extemporaneous
Finger, S. (1994). Origins of neuroscience. New York City: Oxford
or repeated output that is often associated with impaired
University Press.
Finger, S. (2000). Minds behind the brain. New York City: Oxford
comprehension of grammatical structures. Personality
University Press. and intelligence are typically intact, and, in general, non-
Finger, S. (2004). Paul Broca (18241880). Journal of Neurology, 251, linguistic cognitive functions are relatively preserved, but
769770. this is difficult to test the given role of language in cogni-
Greenblatt, S. H. (1970). Hughlings Jacksons first encounter with the
tive functions.
work of Paul Broca: The physiological and philosophical back-
ground. Bulletin of the History of Medicine, 44, 555570.
Grodzinsky, Y., & Amunts, K. (Eds.). (2006). Brocas region. New York
City: Oxford University Press. Categorization
Hothersall, D. (2004). History of psychology (4th ed.). New York City:
McGraw-Hill.
Brocas Aphasia is a type of aphasia that is characterized
Jay, V. (2002). Pierre Paul Broca. Archives of Pathology and Laboratory
Medicine, 126, 250251.
by speech that is effortful, sparse, and halting, with
Lee, D. A. (1981). Paul Broca and the history of aphasia: Roland P. impaired repetition and relatively intact language
Mackay Award Essay, 1980. Neurology, 31, 600602. comprehension.
Memoir of Paul Broca (1881). The Journal of the Anthropological Institute
of Great Britain and Ireland, 10, 242261.
Schiller, F. (1983). Paul Broca and the history of aphasia. Neurology,
33, 667.
Natural History, Prognostic Factors,
Schiller, F. (1992). Paul Broca: Founder of French anthropology, explorer of and Outcomes
the brain. New York: Oxford University Press.
Schreider, E. (1966). Brain weight correlations calculated from original The prognosis for recovery of functional communication
results of Paul Broca. American Journal of Physical Anthropology, 25,
in individuals with Brocas aphasia depends on the under-
153158.
Stone, J. L. (1991). Paul Broca and the first craniotomy based on cerebral
lying cause of the aphasia as well as factors such as the size
localization. Journal of Neurosurgery, 75, 154159. of the lesion and the patients age, premorbid language
skills, and comorbid health conditions. Individuals who
initially present with Brocas aphasia often evolve to a
clinical profile of anomic aphasia, with relatively good
Brocas Aphasia auditory and reading comprehension, and deficits pri-
marily in word-finding and the comprehension and pro-
LYN T URKSTRA duction of complex syntax.
University of Wisconsin-Madison
Madison, WI, USA
Neuropsychology and Psychology
of Brocas Aphasia
Synonyms
Brocas aphasia has been traditionally associated with
Anterior aphasia; Expressive aphasia; Motor aphasia lesions to Brodmanns areas 44 and 45 in the frontal
lobe of the dominant (typically left) hemisphere. Autopsy
data and neuroimaging studies, however, have shown
Short Description or Definition both the absence of Brocas aphasia in individuals with
lesions in this region and also the reverse (Yang, Zhao,
It is a type of aphasia that is characterized by speech that is Wang, Chen, & Zhang, 2008). The debate about the local-
effortful, sparse, and halting, and impaired repetition, ization of Brocas aphasia is part of a larger theoretical
with relatively intact language comprehension. The spo- discussion of the modularity of language and other cog-
ken output of individuals with Brocas aphasia often is nitive functions (for the treatment implications of this
described as telegraphic, as it contains primarily content debate, see discussion in Basso & Marangolo, 2000).
458 B Brocas Aphasia
Cross References
Evaluation
Aphasia
Aphasia is typically evaluated using a combination of Nonfluent Aphasia
standardized language tests and careful observation of Paraphasia
extemporaneous communication. Assessment of cogni- SpeechLanguage Therapy
tive functions such as attention, memory, and executive
functions is challenging in this group, given both the
verbal demands inherent in the structure of most neuro-
psychological tests and the complex interplay of language References and Readings
and other cognitive functions. Cognitive tests considered
Academic of Neurologic Coaammunication Disorders and Sciences.
to have relatively low language demands (e.g., the Cogni-
Evidence-based practice guidelines (Association). Retrieved October
tive Linguistic Quick Test, Helm-Estabrooks, 2001; or 1, 2008, from www.ancds.org
Ravens Standard Progressive Matrices, Raven 1938, in- Alexopoulos, G. S., Abrams, R. C., Young, R. C., & Shamoian, C. A.
cluded in the Western Aphasia Battery) are sometimes (1988). Cornell scale for depression in dementia. Biological Psychia-
used to test cognitive abilities other than language, with try, 23(3), 271284.
Basso, A., & Marangolo, P. (2000). Cognitive neuropsychological rehabili-
the caveat that language impairments are likely to influ-
tation: The emperors new clothes? Special issue: Cognitive neuropsy-
ence performance on these tests as well (Beeson, Bayles, chology and language rehabilitation. Neuropsychological Rehabilitation,
Rubens, & Kaszniak, 1993). 10(3), 219229.
The specific tests and measures used depend on the Beeson, P. B., Bayles, K. A., Rubens, A. B., & Kaszniak, A. W. (1993).
goals of the assessment (e.g., diagnosis vs. prediction of Memory impairment and executive control in individuals with
stroke-induced Aphasia. Brain and Language, 45, 253275.
functional performance vs. treatment planning), the time
Carson, A. J., MacHale, S., Allen, K., Lawrie, S. M., Dennis, M., House, A.,
post-onset (e.g., comprehensive test batteries are not et al. (2000). Depression after stroke and lesion location: A system-
appropriate in the context of acute stroke), and the atic review. Lancet, 356(9224), 122126.
patients clinical presentation. As lesions typically asso- Cherney, L. R., Patterson, J. P., Raymer, A., Frymark, T., & Schooling, T.
ciated with Brocas aphasia affect motor structures in the (2008). Evidence-based systematic review: Effects of intensity of
treatment and constraint-induced language therapy for individuals
frontal lobe, many patients with Brocas aphasia also have
with stroke-induced Aphasia. Journal of Speech, Language, and
apraxia of speech and hemiplegia or hemiparesis, which Hearing Research, 51(5), 12821299.
poses a particular challenge in assessment of language and Goodglass, H. (1993). Understanding aphasia. San Diego, CA: Academic
other cognitive functions. Press.
Bromocriptine B 459
Helm-Estabrooks, N. (2001). Cognitive linguistic quick test (1st ed.). San Current Knowledge
Antonio, TX: Psychological Corporation.
Kagan, A., Black, S., Duchan, J., Mackie, N., & Square, P. (2001). Training
volunteers as conversation partners using supported conversation
In some cases, the boundary identified by Brodmann is B
for adults with Aphasia (SCA): A controlled trial. Journal of Speech, also a functional boundary. For instance, primary visual
Language, and Hearing Research, 44, 624638. cortex is contained in Brodmanns area 17. Brodmanns
Manheim, L. M., Halper, A. S., & Cherney, L. (2009). Patient-reported area 18 is considered to be higher-order visual cortex.
changes in communication after computer-based script training for
Somatosensory functions are associated with Brodmanns
aphasia. Archives of Physical Medicine and Rehabilitation, 90(4),
623627.
areas 3, 1, and 2, with part of area 3 being recognized
Raven, J. C. (1938). Progressive matrices: A perceptual test of intelligence. as primary somatosensory cortex. Brodmanns areas 41
London: H.K. Lewis. and 42 are associated with audition (hearing). Primary
Thompson, C. K., & Shapiro, L. P. (2005). Treating agrammatic aphasia motor cortex (the output for motor commands) is asso-
within a linguistic framework: Treatment of underlying forms.
ciated with Brodmanns area 4, while premotor cortex
Aphasiology, 19(10/11), 10211036.
Townend, E., Brady, M., & McLaughlan, K. (2007). A systematic evalua-
(where the decision to move likely arises) is found in
tion of the adaptation of depression diagnostic methods for stroke Brodmanns area 6.
survivors who have aphasia. Stroke, 38(11), 30763083. A different interpretation of cytoarchitechtonic regions
Turner-Stokes, L., & Hassan, N. (2002). Depression after stroke: A review (107 areas) was published by Constantin von Economo and
of the evidence base to inform the development of an integrated care
Georg N. Koskinas in 1925.
pathway. Part 1: Diagnosis, frequency and impact. Clinical Rehabili-
tation, 16(3), 231247.
Yang, Z. H., Zhao, X. Q., Wang, C. X., Chen, H. Y., Zhang, Y. M. (2008).
Neuroanatomic correlation of the post-stroke aphasias studied with Cross References
imaging. Neurological Research, 30(4), 356360.
Auditory Cortex
Cerebral Cortex
Neocortex
Somatosensory Cortex
Brodmanns Area 17 Visual Cortex
Visual Cortex
References and Readings
Brodmann, K. (2005). Brodmanns: Localisation in the cerebral cortex.
(L. J. Carey Trans.). Berlin: Springer.
Brodmanns Areas of the Cortex Falk, D., & Gibson, K. R. (Eds.). (2008). Evolutionary anatomy of the
primate cerebral cortex. Cambridge: Cambridge University Press.
K IMBERLE M. J ACOBS
Virginia Commonwealth University
Richmond, VA, USA
Bromocriptine
Definition DAVID J. L IBON
Drexel University, College of Medicine
Brodmanns areas of the cortex refer to 52 regions of the Philadelphia, PA, USA
cerebral cortex that were identified in 1909 by German
Neurologist, Korbinian Brodmann, based on cytoarchi-
tectonic (cell size, spacing or packing density, and lami- Definition
nation) differences. Brodmanns areas are typically shown
on a map of the brain surface, but each region is conti- Bromocriptine is one of the group of medicines classified
nued through the depth of cerebral cortex. These regions as ergot alkaloids. Bromocriptine acts to block the release
were originally identified based on Nissl-stained sections of prolactin which is produced by the pituitary gland.
of human brain; however, Brodmann believed that they Bromocriptine is used to treat a variety of medical con-
applied to all mammals. ditions including problems with menstruation, infertility,
460 B BrownSequard Syndrome
Parkinsons disease, neuroleptic malignant syndrome, horn of the cord on the same side in which they entered.
and pituitary adenomas. When used in conjunction From there, their second-order neurons cross the midline
with diet, bromocriptine can also be used to treat type of the cord in the ventral while commissure and
2 diabetes. then ascend contralaterally as the ventral and lateral
spinothalamic tracts (anterolateral system).
Thus, a lesion that transects one-half of the spinal
References and Readings cord will cause motor symptoms on the same side of the
body as a result of severing the lateral corticospinal tract
Ropper, A. H., & Samuels, M. A. (2009). Adams and Victor principles of on that side. This will result in residual upper motor
neurology (9th ed.). New York: McGraw Hill. neuron type deficits below the level of the lesion (e.g.,
spastic paralysis, hyperreflexia, clonus, loss of superficial
reflexes, and a positive Babinski). As the lesion also affects
the ventral horns, lower motor neuron signs (flaccid pa-
ralysis, severe atrophy, hyporeflexia, and fasciculations)
BrownSequard Syndrome are potentially discernable at the level of the lesion.
Because the posterior columns are affected, the individual
J OHN E. M ENDOZA will also demonstrate a loss of proprioception, fine tactual
Tulane University Medical Center discrimination, and vibratory sense below the level of the
New Orleans, LA, USA lesion on that same side. Finally, as a result of a disruption
of the ascending spinothalamic tracts, there will be a loss
of pain and temperature. However, because these latter
Synonyms tracts represent sensory information that has crossed over
from the opposite side of the cord, the loss of pain and
Hemisection of spinal cord temperature will be contralateral to the lesion (and one of
the sides opposite to the motor and posterior column
symptoms). Because the fibers carrying signals for pain
Definition and temperature ascend and descend a couple of spinal
segments before decussating, the level of loss of pain and
BrownSequard syndrome is a neurological condition in temperature will be slightly below that of proprioception
which, as a result of a lesion affecting one half of the spinal and stereognosis.
cord, there is paralysis and loss of proprioception, vibra- A BrownSequard syndrome usually results from a
tion, and fine tactile discrimination on one side of the penetrating injury as might be found in a knife or bullet
body and loss of pain and temperature on the other. wound. Because such wounds lack anatomical precision,
exactly one half of the cord is rarely severed, but the term
is applied if the clinical picture generally matches that
Current Knowledge which was described above.
Side Effects
Bucco-facial Apraxia
Serious
Apraxia
Seizures, hypomania, induction of mania, and activation
of suicidal ideation (controversial)
Building-related Illness
Common
Sick Building Syndrome
Dry mouth, constipation, nausea, weight loss, insomnia,
dizziness, headache, agitation, tremor, abdominal pain,
tinnitus, and sweating
Bupropion
J OHN C. C OURTNEY 1, C RISTY A KINS 2 References and Readings
1
Childrens Hospital of New Orleans
New Orleans, LA, USA Physicians Desk Reference (62nd ed.). (2007). Montvale, NJ: Thomson
2
Mercy Family Center PDR.
Metarie, LA, USA Stahl, S. M. (2007). Essential psychopharmacology: the prescribers guide
(2nd ed.). New York, NY: Cambridge University Press.
Generic Name
Additional Information
Bupropion
Drug Interaction Effects: http://www.drugs.com/drug_interactions.html
Drug Molecule Images: http://www.worldofmolecules.com/drugs/
Brand Name Free Drug Online and PDA Software: www.epocrates.com
Gene-Based Estimate of Drug interactions: http://mhc.daytondcs.
com:8080/cgi bin/ddiD4?ver=4&task=getDrugList
Wellbutrin, Wellbutrin SR, Wellbutrin XL, Zyban Pill Identification: http://www.drugs.com/pill_identification.html
Class
indeed founded or true. There are three main types of Brand Name
standards of proof: beyond a reasonable doubt, clear and
convincing evidence, and a preponderance of the evi- Buspar B
dence. Artificial percentages have been associated with
each of these standards of proof with beyond reasonable
doubt coinciding with 9095% certainty, clear and Class
convincing evidence 75%, and a preponderance of the
evidence associated with just over 50%. Each of these Anxiolytic
standards is used during different inquiries in criminal
procedure (e.g., insanity defense, competency to stand
trial, and competency to be executed) and there are Proposed Mechanism(s) of Action
other standards used by appellate courts when reviewing
trial court records. Under the current Mental Penal Code Serotonin 1A partial agonist, thus diminishing the overall
Standard for Insanity, The defendant has the burden of serotonergic activity at those receptor sites
providing the defense of insanity by clear and convincing
evidence.
Indication
Cross References Anxiety disorders
Additional Information
Generic Name
Drug Interaction Effects: http://www.drugs.com/drug_interactions.html
Buspirone Drug Molecule Images: http://www.worldofmolecules.com/drugs/
464 B Butters, Nelson (19371995)
Free Drug Online and PDA Software: www.epocrates.com International Neuropsychological Society (Secretary-
Gene-Based Estimate of Drug interactions: http://mhc.daytondcs.
Treasurer 19741977; Board of Governors 19781981;
com:8080/cgi bin/ddiD4?ver=4&task=getDrugList
Pill Identification: http://www.drugs.com/pill_identification.html
Treasurer 19801983; President, 19841985)
National Academy of Neuropsychology (Fellow;
President 19921993)
American Psychological Society (Fellow, 1988)
Butters, Nelson (19371995) American Board of Clinical Neuropsychology
(Founding Fellow, Vice-President 19911993)
M ERYL A. B UTTERS , J AMES T. B ECKER Distinguished Clinical Neuropsychologist Award from
University of Pittsburgh School of Medicine the National Academy of Neuropsychology (1991)
Pittsburgh, PA, USA Meritorious Service Award from the Department of
Veteran Affairs (1993)
Distinguished Service Award from the American
Major Appointments Board of Professional Psychology (1993)
projections of the prefrontal cortex. In 1972, with their and extent of changes in cognition across patients
student Gary Van Hoesen, they reported on the afferent with different forms of brain damage, we advance
(incoming) projections to the entorhinal cortex around our understanding of the neuroanatomical basis of B
the hippocampus another brain region critical for the cognitive process in question, the organization of
memory functions. the process itself, and how these affect the individual
At the same time, Butters was working closely with patient. This concept became a theme sometimes
Donald Stein and Jeffrey Rosen on recovery of func- explicit, sometimes implicit that was woven into the
tion following frontal lobe lesions in nonhuman pri- fabric of Butters research career.
mates. They showed that if one removed sections of An early series of studies in the 1970s with Ina Samuels
the frontal lobes in monkeys in serial fashion, that is, exemplified this approach. Together they systematically
over a series of operations, the resulting deficit was examined visual and auditory short-term memory in
less than that exhibited by monkeys who experienced individuals with a variety of lesions. In the early 1970s
the same lesion, in one step. They concluded that cognitive psychologists were debating the relative mer-
these results demonstrated at least partial recovery of its of serial versus parallel processing in normal human
function. This finding in adult monkeys was impor- memory. Butters work with Laird Cermak on patients
tant because at the time, it was believed that if a brain with amnesic disorders was among the first to examine
lesion occurred after 12 months of age (in the monkey), this and other central topics of cognitive psychological
recovery was not possible. Patricia Goldman was research. The Butters and Cermak collaboration was
showing that infant macaques with brain lesions particularly fruitful because it directly influenced the
could fully recover some functions, but not others development of models of normal human memory
the data from the Rosen, Stein, and Butters series shed particularly the notion that memory is neither a serial
light on the question of whether, and by how much, nor parallel process, but rather that the type or intensity
older animals (and by extension, humans) could re- of the processing was what was critical (i.e., levels of
cover function after a brain lesion. processing argument espoused by Fergus Craik).
One of his earliest human studies (published with While in Boston, Butters and his colleagues made
Melvin Barton in 1970) on the role of the frontal and great strides in elucidating the neuropsychological
parietal lobes in concrete operations, shaped much of effects of alcoholism. The papers that emanated
Butters philosophy of neuropsychology and laid the from studies of chronic, non-amnestic alcoholics (in
groundwork for his career examining the brain struc- collaboration with Christopher Ryan, James Becker,
tures that mediate abnormal cognition. They took Kathleen Montgomery, and Barbara Jones) had a last-
concepts and methods that were popular in human ing influence on the way that the neuropsychology of
cognition at that time and used them to examine the alcoholism was viewed and studied. Butters work also
role of various brain structures in these cognitive pro- focused on the amnesic syndrome associated with
cesses. For example, humans with damage to the pari- chronic, severe alcoholism. With Laird Cermak, his
etal lobes have difficulty reversing or changing their systematic series of studies focused on the role of
behavior. That is, once taught a rule, they have difficul- interference and encoding in the short-term memory
ty deviating from that rule. What was unclear at the defects of patients with alcoholic Korsakoff s syn-
time was whether this deficit was a fundamental deficit drome. It is noteworthy that Butters developed
in reversal or a consequence of a deficit in retention personal relationships with many of these patients,
the ability to remember the old rule or learn the new venturing out to their homes (sometimes state hospi-
one. The results of these experiments not only in- tals or nursing homes) and repeatedly reevaluating
formed investigators on the nature of the behavioral them for different projects. This work was important
and cognitive changes that follow focal brain damage, not only because of what it revealed about both nor-
but lent insight into the cognitive processes themselves. mal and pathological memory, but also because it
Butters made exemplary use of what Hans-Lukas clearly demonstrated that a long-term consequence
Teuber referred to as the natural fracture lines of of alcoholism can be an amnestic disorder (a matter
behavior. That is, when a patient suffers a brain that was hotly debated at the time). Perhaps the most
injury, behavior and cognition fail neither completely enduring legacy of Butters and Cermaks work from
nor in a random manner. Rather, the breakdown this period was the 1980 publication of Alcoholic Kor-
occurs at points where different cognitive processes sakoff s Syndrome: An Information Processing Approach
intersect. By comparing and contrasting the nature to Amnesia. The title was not an accident they
466 B Butters, Nelson (19371995)
wanted to emphasize the role of neuropsychology in included Alzheimers disease, which he considered
analyzing information processing, and how this re- the prototypical cortical dementia. In San Diego
search informed our models of normal memory and his work with David Salmon, William Heindel, Wil-
memory systems. The publication of this text marked liam Beatty, Munro Cullum, Eric Granholm, Alexan-
the beginning of the modern era of cognitive der Troster, Agnes Chan, Andreas Monsch, and a host
neuroscience. of other students and colleagues focused on three
Another trend in Butters research during the middle- main areas, yielding a significant scientific contribu-
to-late 1970s was prompted by his desire to better tion in each.
understand retrograde amnesia, which had not yet Butters work with David Salmon and their colleagues
been studied in any systematic way, due in part, to demonstrated that the memory disorders of cortical
the lack of suitable test instruments. He collaborated and subcortical dementias are dissociable, with Alz-
with Marilyn Albert, who took on the task of develop- heimers disease characterized by poor consolidation
ing the Boston Retrograde Amnesia Battery, which and rapid forgetting due to limbic damage and Hun-
became the first carefully validated and well-normed tingtons disease by poor retrieval associated with
retrograde memory battery applicable to individuals fronto-striatal dysfunction. Butters work with trainee
of various ages. This productive collaboration, which Mark Bondi and others in his research group was
later included Jason Brandt and Donald Stuss, firmly among the first to show that the neuropsychological
established that the retrograde memory impairment characteristics of very early Alzheimers disease dif-
exhibited by Korsakoff s patients had a clear temporal fered from the benign cognitive changes of normal
gradient, suggesting that the neural substrates of aging and could therefore be used for the early and
memory change with the passage of time. That is, even pre-clinical detection of the disease, presaging
over time, episodic memories mediated by similar findings in HD. Finally, researchers were be-
the hippocampal circuitry are actively recalled, there- ginning to investigate the phenomenon of implicit
by losing their spatial and temporal markers, becom- memory, and Butters work with William Heindel,
ing part of semantic memory, stored elsewhere (likely David Salmon, Jane Paulsen, and others showed that
the temporalparietal association cortices). various forms of implicit memory could be dissociated
During his last years in Boston, Butters devoted in Alzheimers disease, Huntingtons disease, and Par-
considerable time to studying the cognitive effects of kinsons disease. These studies were the first to dis-
Huntingtons disease (HD), demonstrating a func- sociate priming and procedural learning in the brain,
tional dissociation between the locus of the lesions showing that procedural learning deficits were asso-
in HD (the basal ganglia) and those found in Korsak- ciated with basal ganglia dysfunction while priming
off s syndrome (the limbic system). This research deficits were linked with neocortical damage that
predated the identification of the HD gene and devel- occurs in Alzheimers disease. Any one of these con-
opment of a test to determine whether the offspring of tributions would have been impressive; together they
HD patients were destined to develop the disease. As represent a truly remarkable accomplishment.
part of the Center Without Walls, Butters worked In 1986 the American Psychological Association
with people at risk for HD (i.e., who had a parent (APA) acquired the journal Neuropsychology and But-
with the disease) to determine whether measures of ters was appointed Editor. APA tradition allows new
cognition administered earlier in life could predict editors to choose the colors of the journals binding
those who would ultimately develop the disease. and Butters chose a white background with (Boston)
In 1983 Butters accepted an offer to join the Depart- Celtic green for the text (he was a lifelong fan of his
ment of Psychiatry at the University of California- hometown basketball team). Butters goal for the
San Diego (UCSD), where he relished the opportunity journal was that it should become one of the journals
to develop a clinical service with neuropsychologists of record for basic, applied, and clinical research in
who conducted research as well as provided clinical neuropsychology. This will be another of his lasting
care. In addition to developing a first-rate psychology legacies.
service, Butters maintained his prolific research pub- Most important to Butters were his students his
lication rate. Soon after arriving in San Diego, UCSD academic children (and in some cases grandchildren)
was designated an Alzheimers Disease Research Cen- were almost as important to him as his biologic chil-
ter, which provided Butters with the opportunity to dren, Meryl, Paul, and Lisa. His attraction was inter-
expand his research on memory. His studies now national (e.g., John Hodges, Narinder Kapur, Matti
Butters, Nelson (19371995) B 467
Laine), and his mentees continue to lead the field (e. and scholar. A concussion sustained playing football left
g., Kathie Welsh-Bohmer, Terry Jernigan). him with lifelong anosmia, an irony not lost on him when
A narrative of Butters contributions to neuropsychol- he later published articles on the importance of assessing B
ogy would be woefully incomplete without a personal olfaction in brain-damaged patients. In addition to his
description. He possessed tremendous internal drive, obvious academic talent, he took great pride in his care-
and held both his students and colleagues to the high fully crafted image as a motor scooter-riding bad boy,
standards he applied to himself. Every Butters student emulating James Dean and Marlon Brando. Later in life,
carries distinct memories of the red ink and blunt he took great pleasure in recounting tales of his friendship
reviews accompanying their manuscript drafts. His with his classmate, the late social and political activist
academic rigor notwithstanding, Butters was univer- Abbie Hoffman. Butters particularly enjoyed retelling
sally known as an involved, supportive mentor and stories of drag racing against Hoffman from Worcester
colleague. His role extended to that of job-broker and to Boston, MA, and attending wild parties at Hoffmans
he devoted an inordinate amount of time and energy home. Somehow surviving high school, he went on to
to assisting and advising a vast array of students and attend the University of Chicago and Boston University.
colleagues about both professional and personal Butters was at first unsure of his professional goals.
matters. He was described as being biologically inca- His parents had pushed for him to become a physician or
pable of tolerating an unhappy student (a term that lawyer, but neither profession held much appeal. Public
he applied quite broadly); he had to help make things speaking came rather easily to him, and he was excited by
right (even if the student did not immediately appre- the idea of being a positive influence in the lives of college
ciate his wisdom). In his later years he took tremen- students. Therefore, during the second or third year of
dous pleasure in referring to himself as the college he decided to become a psychology teacher.
Godfather of Neuropsychology. Butters entered the doctoral program in psychology at
Butters was blunt, brash, and famously irreverent. Clark University in the fall of 1960. At the time Clark had
While his lifestyle was decidedly mainstream, he one of the leading psychology programs in the world. He
reveled in characterizing himself as something of a was awarded a Woodrow Wilson Fellowship for his first
rebel. He was unendingly curious about those who year of graduate school because of his commitment to
walked to the beat of a different drummer. His per- college teaching. During his second or third year in grad-
sonal role models included Marlon Brando, Lenny uate school, he ran across a research article by Mortimer
Bruce, and Woody Allen. He had a keen sense for Mishkin and H. Enger Rosvold about delayed alternation,
and appreciation of the absurd and ironic and found reversal learning, and the frontal lobes in monkeys.
humor in the darker side of life, though he never took Butters became fascinated with the concept that one could
himself too seriously. On more than one occasion he examine the neurological structures that underlie cognitive
was heard saying: If I hadnt become a neuropsychol- and psychological processes. At the time he was particularly
ogist, I wouldve been a comedian. At every APA and interested in what went on in the brain and what brain
INS meeting social hour, Butters could be found, structures played a part in the development of concrete or
beer in hand, listening to and recounting hilarious formal operations in the thinking processes of children.
stories about friends, colleagues, and family. Most of At the end of his third year of graduate school, Butters
the time, he played a leading role in these comedic wrote to Hal Rosvold, a researcher at the National Insti-
tales. In sum, Butters rare combination of intellectual tutes of Health (NIH), asking whether he would consider
creativity, drive, irreverence, and appreciation of taking him as a postdoctoral fellow. A visit to Rosvolds
satire allowed him to make his mark both in the lab led to a postdoctoral fellowship with Rosvold and
field of neuropsychology and in the hearts of his Mishkin from 1964 to 1966. His work at the NIH focused
contemporaries. on the roles of the septal nuclei, basal forebrain, and
caudate nucleus in reversal learning and delayed alterna-
tion performance. Butters was fulfilling his plan to spend
Short Biography a couple of years studying physiological psychology
and perhaps do a few studies on the neurological
Nelson Butters was born on May 7, 1937, in Boston, MA. bases of cognition before resuming the path toward his
He initially attended public school in Brookline, MA, and teaching career.
then Worcester Academy, a private boarding school, grad- Butters resumed his teaching path in 1966 by taking
uating first in his class. He was an accomplished athlete teaching positions at Wright State University and Antioch
468 B BVRT
Acknowledgment
We would like to thank Christopher Ryan and David BVRT
Salmon for very helpful comments on an earlier draft of
this chapter. Benton Visual Retention Test
C
progresses to transient ischemic attacks (TIA), and recur-
C/NC rent strokes. The migraine attacks are often reported to be
particularly long lasting or severe and may even include
Coma/Near Coma Scale symptoms of hemiplegia. The strokes are typically lacu-
nar, occurring in subcortical white matter or basal ganglia
and, in some cases, occur in the brain stem and spinal
cord. Recurrent subcortical infarcts lead to cognitive de-
CABG cline, pseudobulbar palsy, motor impairment, psychiatric
symptoms (most commonly depression), and ultimately
Coronary Artery Bypass Graft total motor impairment and subcortical vascular demen-
tia. Cognitive decline is most prominently seen as execu-
tive dysfunction, slowed processing speed, and reduced
attentional abilities. As the disease progresses, memory
Cadasil and other areas of cognitive functioning begin to decline.
The total amount of white matter involvement associated
A NGELA E ASTVOLD, YANA S UCHY
with the lacunar lesions and the degree of atrophy predict
University of Utah
severity of cognitive and motor impairment.
Salt Lake, UT, USA
Cause: CADASIL is caused by mutations or deletions in
the Notch3 gene on chromosome 19, which plays an
important role in cell differentiation during development.
Synonyms
While the pathogenetic mechanism has yet to be deter-
mined, the resulting pathophysiology includes thickening
Agnogenic medial arteriopathy; Chronic familial vascular
of arterial walls, accumulation of granular osmiophilic
encephalopathy; Familial Binswangers disease; Familial
material (GOM) in arterial walls (this distinguishes it
disorder with subcortical ischemic strokes, dementia, and
from arterial hypertension), gradual destruction of vascu-
leukoencephalopathy; Hereditary multi-infarct dementia
lar smooth muscle cells leading to fibrosis, progressive
thickening of arterial walls, and narrowing of the lumen
Short Description or Definition of cerebral arteries. This eventually causes thrombosis,
reduced blood flow, destruction of small- and medium-
Cerebral autosomal dominant arteriopathy with subcor- sized arteries, and consequently focal infarcts. MRI typi-
tical infarcts and leukoencephalopathy (CADASIL) is a cally reveals characteristic periventricular or white matter
rare autosomal dominant, adult-onset inherited cerebral hyperintensities, as well as infarcts in the basal ganglia and
vascular disease, characterized by migraines, recurrent brain stem. The cerebral cortex remains relatively intact.
transient ischemic attacks, and strokes leading to cogni- Diagnosis: Identification of a Notch3 mutation provides
tive decline and dementia. the most certain diagnosis, in addition to the presence of
GOM in arterial walls, which can be detected with a skin
biopsy. Positive MRI findings are often detected in even
Current Knowledge asymptomatic individuals. History of particularly severe
migraines may be the earliest indication.
Clinical Manifestation: The typical age of onset is Treatment and prognosis: Currently, there is no cure or
30500 years of age. The initial clinical manifestation disease-modifying therapy for CADASIL, and only symp-
often begins with migraines with aura, and slowly tom management and supportive care are available for
Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan (eds.), Encyclopedia of Clinical Neuropsychology, DOI 10.1007/978-0-387-79948-3,
# Springer Science+Business Media LLC 2011
470 C CAGE Questionnaire
affected individuals. Death typically occurs 1020 years to steady your nerves or to get rid of a hangover (eye-
after the onset of symptoms. Angiography and anticoagu- opener)? Individual item responses are scored in a binary
lants should be avoided as they may provoke cerebrovascu- fashion (0 = no and 1 = yes). The total score can
lar accidents. range from 0 to 4. A score of 2 or greater is typically
considered a positive finding, that is, an indication of
alcohol misuse.
Cross References
score at 2, pooled sensitivities across studies were 0.87 in Cross References
hospital inpatients, 0.71 in primary care patients, and 0.60
in ambulatory medical patients; specificities were 0.77, Alcohol Abuse
0.91, and 0.92, and positive predictive validities were Alcohol Dependence
0.57, 0.74, and 0.82, respectively. Aertgeerts et al. (2004) Alcoholic Brain Syndrome C
concluded that the CAGE is only of limited value as a Alcoholism
screening instrument using a cutoff score of 2 and Blood Alcohol Level
recommended that additional information about alcohol Fetal Alcohol Syndrome
use patterns be obtained from any patient who gives one Korsakoff s Syndrome
positive answer on the instrument. Michigan Alcoholism Screen Test
Dhalla and Kopec (2007) provided a more recent Substance Abuse
review of the CAGE validity literature, considering, in Substance Abuse Disorders
part, the sensitivity, specificity, and positive predictive WernickeKorsakoff s Syndrome
value associated with different cutoff scores. The authors
concluded that the CAGE has adequate validity in
References and Readings
screening medical and surgical inpatients, psychiatric
inpatients, and ambulatory medical patients for alcohol
Aertgeerts, B., Buntinx, F., & Kester, A. (2004). The value of the CAGE in
misuse and that use of a cutoff score of 2 affords the screening for alcohol abuse and alcohol dependence in general
best combination of sensitivity, specificity, and positive clinical populations: A diagnostic meta-analysis. Journal of Clinical
predictive value. At the same time, they cautioned that the Epidemiology, 57, 3039.
CAGE has not performed well with Caucasian women, American Psychiatric Association (1987). Diagnostic and statistical man-
ual of mental disorders (3rd rev. ed.). Washington, D.C.: American
prenatal women, and college students.
Psychiatric Press.
Fiellin, Carrington, and OConner (2000) also provide Bradley, K., Kiviahan, D., Bush, K., McDonell, M., & Fihn, S. (2001).
a noteworthy review of validity studies, concluding that in Variations on the CAGE alcohol screening questionnaire: Strengths
primary care settings, the CAGE questionnaire was more and limitations in VA general medical patients. Alcoholism: Clinical
effective in identifying patients with alcohol abuse or and Experimental Research, 25, 14721478.
Cherpitel, C. J. (2000). A brief screening instrument for problem drinking
dependency than in detecting patients with at risk,
in the emergency room: The RAPS4. Journal of Studies on Alcoholism,
hazardous, or harmful drinking. 61, 447449.
Dhalla, S., & Kopec, J. (2007). The CAGE questionnaire of alcohol
misuse: A review of reliability and validity studies. Clinical & Inves-
Clinical Uses tigative Medicine, 30, 3341.
Ewing, J. A. (1984). Detecting alcoholism: The CAGE questionnaire.
Journal of the American Medical Association, 252, 19051907.
The CAGE has enjoyed wide popularity in primary care Ewing, J. A., & Rouse, B. A. (1970). Identifying the hidden alcoholic.
settings as an alcohol-screening instrument for a number Presented at the 29th international congress on alcohol and drug
of reasons, as it is brief, easy to administer, and can be dependence, Sydney, Australia, February 3, 1970.
incorporated into more extensive interviews or question- Fiellin, D., Carrington, R., & OConner, P. (2000). Screening for alcohol
problems in primary care: A systematic review. Archives of Internal
naires (Bradley et al., 2001). However, support for its
Medicine, 160, 19771989.
validity across different populations has been mixed. It National Institute on Alcohol Abuse and Alcoholism (1995). The physicians
appears to perform best when used to screen for alcohol guide to helping patients with alcohol problems (NIH publication
abuse or dependence in clinical populations and less 95-3769). Bethesda: U.S. Department of Health and Human
well in detecting current hazardous drinking. Caution Services, Public Health Service, National Institutes of Health.
Pokorny, A., Miller, B., & Kaplan, H. (1972). The brief MAST: A short-
should be used especially when interpreting results for
ened version of the Michigan Alcoholism Screening Test. American
nonclinical respondents (i.e., general population) as well Journal of Psychiatry, 129, 342.
as for women and college students, as the CAGE has been Saunders, J. B., Aasland, O. G., Babor, T. F., De La Fuente, J. R., & Grant,
noted to under-perform in these populations. In addition, M. (1993). Development of the Alcohol Use Disorders Identification
as several authors have recommended, a positive finding Test (AUDIT): WHO collaborative project on early detection of
persons with harmful alcohol consumption-II. Addiction, 88,
on the CAGE, regardless of cutoff score level (i.e., 1
791804.
or 2), should be followed up by further examination Teitelbaum, L., & Carey, K. (2000). Temporal stability of alcohol
of alcohol consumption patterns, using established stan- screening measures in a psychiatric setting. Psychology of Addictive
dard criteria. Behaviors, 14, 401404.
472 C CalCAP
Illness
Structure
Cross References
This is the term for the primary visual cortex, V1, or
Calcarine Fissure
Brodmanns area 17. The area encompasses the medial
Calcarine Sulcus
surface and a small lateral surface of the occipital lobe,
Occipital Lobe
and is within the calcarine sulcus. It is located at the most
Scotoma
posterior portion of the cerebral hemisphere. The optic
Unimodal Cortex
radiations terminate in layer IV of the calcarine cortex.
Visual Cortex
Layer IV is rather thick and is subdivided into sublaminae.
The calcarine cortex is also sometimes referred to as
the striate cortex because of a strip of myelin (Stria of
Gennari) that can be visualized. The area is organized in References and Readings
a retinotopic fashion; that is, there is point-to-point
representation from the retina to the cortex. For instance, Netter, F. (1991). The CIBA collection of medical illustrations: Volume I
fibers in the right half of each retina (perceives information nervous system, part I: anatomy and physiology. West Caldwell, NJ:
in the left visual field) project to the right calcarine cortex, CIBA-Geigy.
and fibers in the left half of each retina (perceives informa-
tion in the right visual field) project to the left calcarine
cortex.
Calcarine Fissure
Function J OHN E. M ENDOZA
Tulane University Medical Center
From a functional perspective, the calcarine cortex is im- New Orleans, LA, USA
portant in determining orientation, spatial-frequencies,
and color properties of the visual stimulus. The infor-
mation is then projected to other areas in the occip- Synonyms
ital lobe for further visual analysis. This occurs via
transmission to the dorsal (where) and the ventral Calcarine sulcus
California Computerized Assessment Package C 473
California Computerized
Assessment Package
E RIC N. M ILLER C
UCLA Psychology Clinic
Los Angeles, CA, USA
Synonyms
CalCAP
Description
Calcarine Fissure. Figure 1 Mid-sagittal view of the brain
(MRI) showing calcarine fissure separating the cuneus and the The California Computerized Assessment Package (Cal-
lingual gyrus of the occipital lobe CAP; 1999) presents a series of brief reaction time tasks
designed to assess speeded information processing and
psychomotor functioning. The Standard CalCAP consists
Definition of 4 Simple and 6 GoNo Go reaction time subtests that
require 2025 min to complete. The individual reaction
The calcarine fissure is a deep sulcus located on the medial time measures were designed to assess a number of cog-
surface of the occipital lobe (see Fig. 1). The superior nitive domains, including speed of processing (reaction
(cuneus) and inferior (lingual gyrus) banks of this sulcus time), lexical discrimination (real word vs. nonsense
represent the primary cortical projection area for vision. word), rapid visual scanning, form discrimination and
Visual information that is first received in the upper matching, working memory (1-back and 2-back tasks),
portions of the retina is represented along the upper and sustained and divided attention. Each task includes a
banks of the fissure (cuneus), while that derived from practice session that must be completed with 100% accu-
the lower retina projects to its lower bank. Thus, lesions racy to proceed to the main 2-min procedure, a method
confined to the upper bank (cuneus) will result in con- that ensures that subjects understand the task instructions
tralateral inferior visual field defects, while damage to and that minimizes practice effects in longitudinal study
the lower bank (lingual gyrus) will produce upper field designs. Subjects are asked to focus on a display field and
deficits. Lesions destroying tissue along both the upper respond only to specific types of visual stimuli. For exam-
and lower banks may result in a contralateral homony- ple, in the form discrimination task, they are asked to
mous hemianopia. press a key only when two out of three non-nameable
figures are identical.
The core measures of reaction time, hits and false-
positives, and signal detection parameters allow investiga-
Cross References tors to measure accuracy and general speed of processing,
as well as relative differences among conceptually distinct
Cuneus decision tasks. Four iterations of a Simple reaction time
Lingual Gyrus routine at various points in the test allow direct measure-
Visual System ment of the subjects ability to maintain focus throughout
the 20-min testing procedure. The computer scores each
task using age- and education-specific norms derived from
641 men ranging from 21 to 58 years of age, with a mean
education of 16 years. Final scores are available immediate-
Calcarine Sulcus ly in tabular and graphical formats. Additional norms have
been derived for elementary school children (3rd, 5th, and
Calcarine Fissure 6th grades), women, and older individuals. The CalCAP
474 C California Computerized Assessment Package
also includes a 4-subtest language-independent abbreviated GoNo Go paradigms, the simple reaction time measures
battery weighted toward measures of speeded processing have relatively low testretest reliability (0.200.29), sug-
and working memory. These test batteries are well suited gesting that the psychomotor skills measured by the sim-
for collecting reliable information on psychomotor func- ple reaction time tasks vary considerably depending on
tioning in a brief period of time, and can be used effectively state variables such as mood, attention, fatigue, and time
for assessing changes over time. Stimulus materials are of day (Miller, 2008).
available in English, Spanish, French, Norwegian, Danish, CalCAP reaction time correlates most highly with age,
and Flemish. and, to a lesser extent, with years of education. Two
studies of gender effects on CalCAP reaction time have
shown no differences between men and women on any of
Historical Background the CalCAP indices (Berg, 1994). The psychometric prop-
erties of the CalCAP are unstable for 3rd graders, but
The CalCAP was originally developed by Dr. Eric Miller essentially the same as found in adults by the 5th grade
in the late 1980s as an automated substitute for (Budzinski, 1994).
traditional neuropsychological testing. The Standard
battery, developed in consultation with Dr. Paul Satz
and Dr. Ola Selnes, was designed to measure aspects of Clinical Uses
language and visuoperceptual skills, working memory,
speeded information processing, attention, learning and The CalCAP has been widely used in cross-sectional and
memory, visual scanning, and reaction time. While the longitudinal studies of HIV/AIDS (Miller et al., 1991;
reaction time tasks that comprise the Standard CalCAP Gonzalez et al., 2003), drug abuse (Chang et al., 2002;
battery do assess many of these areas of cognitive func- Volkow et al., 2001), depression (Stordal et al., 2004),
tioning, studies have shown that the tasks correlate only epilepsy (Hessen, Lossius, M. I., Reinvang, I., & Gjerstad,
modestly (0.20.4) with traditional neuropsychological 2008), traumatic brain injury (Waterloo, Ingebrigtsen, &
measures (Miller, Satz, & Visscher, 1991). Factor analyses Romner, 1997), and hyperbaric oxygen treatments
using the CalCAP and other neuropsychological test (Hjalmarsen, Waterloo, Dahl, Jorde, & Viitanen, 1999;
batteries show that the reaction time indices form two Van Hoof, Coomans, De Becker, De Meirleir, & Cluydts,
primary factors best characterized as simple reaction 2002). It is particularly sensitive to the psychomotor
time and decision speed. These factors are distinct changes seen in these disorders and, unlike most psycho-
from traditional neuropsychological measures and motor tasks, has minimal practice effects, making it par-
most researchers and clinicians now use the CalCAP ticularly appropriate for research paradigms such as
and other reaction time measures primarily as indices clinical trials and epidemiologic studies that require re-
of these types of speeded information processing and peated testing.
attention. The abbreviated version of the CalCAP specif- The cognitive functions assessed by the CalCAP pro-
ically excludes measures designed to look at visuoper- gram are best described as timed psychomotor skills re-
ceptual and language skills and focuses primarily on quiring focused or sustained attention. Impaired reaction
these core indices of reaction time, working memory, time across multiple measures is usually indicative of
and attention. generalized motor slowing or slowed information pro-
cessing. Impaired reaction time on specific measures,
particularly when coupled with scores outside of normal
Psychometric Data bounds on true-positive responding, is suggestive of a
more specific functional deficit, such as language skills
All the subtests in the CalCAP have high internal consis- (lexical discrimination task), visuoperceptual deficits
tency reliability (0.770.96). Six-month testretest reli- (form discrimination), or working memory (1-back and
ability for the GoNo Go paradigms is comparable with 2-back tasks).
levels seen for conventional neuropsychological proce- The standard CalCAP program classifies subjects as
dures (0.430.68), but there is less evidence of the practice outliers if they perform two standard deviations or
effects commonly seen with psychomotor measures. The lower on two or more of the tasks. Using these
reduced practice effects may be due to the requirement criteria, approximately 10% of subjects are classified as
that all subjects complete practice trials with 100% outliers. This base rate of 10% includes individuals with
accuracy before proceeding to the actual test. Unlike the premorbid conditions such as prior head injury, learning
California Verbal Learning Test (California Verbal Learning Test-II) C 475
disability, preexisting neurologic conditions, as well as Waterloo, K., Ingebrigtsen, T., & Romner, B. (1997). Neuropsychological
function in patients with increased serum levels of protein S-100
individuals who are simply on the low end of normal
after minor head injury. Acta Neurochirurgica, 139, 2632.
functioning.
Cross References
C
California Verbal Learning Test
Attention/Executive Functions (California Verbal Learning
Continuous Performance Tests
Information Processing Speed Test-II)
Speed of Information Processing
VisualMotor Function A NGELA Y I
Mount Sinai Medical Center
New York, NY, USA
References and Readings
Berg, D. (1994). Adults reaction time as affected by age and education level.
Synonyms
B.A. Honours Thesis supervised by Dr. Frank Spellacy, University of
Victoria. CVLT
Budzinski, L. M. (1994). An exploration of simple and choice reaction times
in children. B.A. Honours Thesis supervised by Dr. Frank Spellacy,
Victoria, B.C., University of Victoria.
Chang, L., Ernst, T., Speck, O., Petal, H., DeSilva, M., Leonido-Yee, M., Description
et al. (2002). Perfusion MRI and computerized cognitive tests
abnormalities in abstinent methamphetamine users. Psychiatry Re-
search, 114, 6579.
The California Verbal Learning Test-II (CVLT-II) is the
Gonzalez, R., Heaton, R. K., Moore, D. J., Letendre, S., Ellis, R. J., latest (2000) version of a widely used verbal learning and
Wolfson, T., et al. (2003). HIV Neurobehavioral Research Center memory test. The current version contains recalling
Group. Computerized reaction time battery versus a traditional and recognition of two lists of words over immediate
neuropsychological battery: detecting HIV-related impairments. and delayed trials. List A includes 16 words and requires
Journal of International Neuropsychological Society, 9, 6471.
Hessen, E., Lossius, M. I., Reinvang, I., & Gjerstad, L. (2008). Improve-
the examinee to recall the list over five trials. List B
ment in speeded cognitive processing after anti-epileptic drug with- (interference), which is also 16 words, is administered
drawal a controlled study in mono-therapy patients. Progress in after List A for one trial. Short-delay free recall and
Neurotherapeutics and Neuropsychopharmacology, 3, 199209. cued recall are administered after List B. A 20-min delay
Hjalmarsen, A., Waterloo, K., Dahl, A., Jorde, R., & Viitanen, M. (1999). follows the short-delay recalls, followed by nonverbal
Effect of long-term oxygen therapy on cognitive and neurological
dysfunction in chronic obstructive pulmonary disease. European
testing. Long-delay recall, long-delay-cued recall, and yes/
Neurology, 42, 2735. no-recognition trials of List A follow the 20-min delay.
Miller, E. N. (1999). CalCAP California Computerized Assessment Package A revision of the CVLT-II is the addition of a forced choice
Manual (2nd ed.). Los Angeles: Norland Software. recognition after a 10-min delay following the yes/no trial.
Miller, E. N. (2008). CalCAP California Computerized Assessment Pack- Another revision of the CVLT-II is the inclusion of a
age. Retrieved October 16, 2008 from http://www.calcaprt.com
Miller, E. N., Satz, P., & Visscher, B. (1991). Computerized and conven-
short form (nine words) and an alternate form. The short
tional neuropsychological assessment of HIV-1 infected homosexual form was created for the purpose of screening or for
men. Neurology, 41, 16081616. patients who may have more severe brain damage and
Stordal, K. I., Lundervold, A. J., Egeland, J., Mykletun, A., Asbjornsen, A., may feel overwhelmed by the long form. This short form
Landro, N. I., et al. (2004). Impairment across executive functions takes 15 min to administer and also has a 15-min delay.
in recurrent major depression. Nordic Journal of Psychiatry, 58,
4147.
The alternate form has been statistically equated with the
Van Hoof, E., Coomans, D., De Becker, O., De Meirleir, K., & Cluydts, R. standard form.
(2002). Hyperbaric oxygen therapy for chronic systemic infections in The CVLT-II was normed from a national standardi-
chronic fatigue syndrome. International Journal of Psychophysiology, zation sample of 1,087 of ages 1689, which was demo-
45, 8283. graphically matched to the US population. It takes 30 min
Volkow, N. D., Chang, L., Wang, G. J., Fowler, J. S., Leonido-Yee, M.,
Francesci, D., et al. (2001). Association of dopamine transporter
to administer and includes 30 min of delay. The test is
reduction with psychomotor impairment in methamphetamine administered with paper and pencil format, with the
abusers. American Journal of Psychiatry, 158, 377382. examiner recording the responses. The responses are
476 C California Verbal Learning Test Childrens Version
scored using the Comprehensive Scoring System, which of the literature of clinical populations from 1987 to
computes raw scores and standardized scores for over 1999, summarizing the key findings of CVLT perfor-
50 learning and memory variables. The raw scores can mance across various clinical populations, which include
be scored manually, although there are limits to how anterior temporal lobectomy, Korsakoff s syndrome,
much information can be derived. Alzheimers disease, Huntingtons disease, Parkinsons
The CVLT-childrens (CVLT-C) version can be adminis- disease, head injury, schizophrenia, depression and
tered to children aged 516 and can assist professionals in other affective disorders, chronic alcohol and drug
diagnosing memory impairment secondary to learning dis- abuse, posttraumatic stress disorder, systemic medical
abilities, attention-deficit disorder, mental retardation, and disease (HIV, chronic fatigue syndrome, Lyme disease,
other neurological disorders and psychiatric problems. The eosinophilia myaligia syndrome, cardiac transplant can-
administration is similar to the format of the CVLT-II, didates), insufficient effort, and predictor for everyday
although forced choice recognition is not included. functioning.
The first edition of the CVLT was originally published in Buschke Selective Reminding Test
1987 from the work of Delis, Kramer, Kaplan, and Ober Childrens Memory Scale
(1987). They created one of the first tests to incorporate Hopkins Verbal Learning Test
principles from cognitive science to measure learning and Rey Auditory Verbal Learning Test
memory. This test provided a measurement of not only Rivermead Behavioral Memory Test
what an individual remembers, but how they remember Selective Reminding Test
and what errors are made. This refuted previous beliefs Wechsler Abbreviated Scale of Intelligence
that memory dysfunction was limited to recall and Wechsler Memory Scale All Versions
recognition.
California Verbal Learning Test Childrens Version. Table 1 CVLT-C variable definitions
Variable
List A total Total number of words recalled across the five learning trials
List A1 Number of words recalled from the first trial
List A5 Number of words recalled from the fifth trial
List B Number of words recalled from the interference list (List B)
List A short-delay free Number of words recalled from List A immediately after the presentation of the interference list
recall (List B)
List A short-delay cued Number of words recalled from List A with semantic cueing
recall
List A long-delay free Number of words recalled from List A after a 20 min delay
recall
List A long-delay cued Number of words recalled from List A with semantic cueing after a 20 min delay
recall
Semantic clustering Number of consecutively recalled words from the same semantic category (i.e., consecutive words
from the same semantic category), which reflects the extent to which the subject has actively
imposed a semantic organization on the list of words
Serial clustering Number of consecutively recalled words in the same order as they were presented (i.e., a serial
cluster)
Primacy % Percentage of words correctly recalled from the beginning of List A (first four words)
Middle % Percentage of words correctly recalled from the middle of List A (middle seven words)
Recency % Percentage of words correctly recalled from the end of List A (last four words)
Learning slope Average number of new words acquired across the five List A immediate-recall trials (e.g., a score of 1
means that the examinee learned approximately one new word per trial)
Consistency % Percentage of target words recalled once on each of the four learning trials that were also recalled on
the very next trial; reflects ability to maintain a consistent learning strategy
Perseverations Total number of target words repeated within a trial (called repetitions on the CVLT-II; Delis et al.,
2000)
Free-recall intrusions Total number of extra-list intrusions on all the free-recall trials
Cued intrusions Total number of extra-list intrusions on all the cued-recall trials
Total intrusions Total number of extra-list intrusions made on free- and cued-recall trials
Recognition hits Total number of target words correctly identified on recognition testing as belonging to List A
(yes/no format)
Recognition Accuracy of distinguishing target from distracter words on recognition testing; calculated using
discriminability signal detection methods
False positives Total number of distractor words incorrectly identified as belonging to List A during recognition
testing
Response bias Tendency to favor yes or no responses on recognition testing (positive scores reflect a yes bias)
distinct patterns of memory dysfunction associated with neuroscience, and modeled in part after the Rey Auditory
various neurological and psychiatric populations. These Verbal Learning Test (Rey AVLT; Rey, 1964), the CVLT-C,
findings emphasize the multicomponential nature of like its adult counterpart ( California Verbal Learning
learning and memory, and, as such, the importance of Test (California Verbal Learning Test-II)), was developed
developing clinical memory tests that allow for the to facilitate the quantification of multiple learning and
evaluation of both qualitative and quantitative features memory parameters (Delis et al., 1987; Delis, Freeland,
of memory performance. Informed by principles of Kramer, & Kaplan, 1988; Delis et al., 1994; Delis et al.,
learning/memory from cognitive science and cognitive 2000).
California Verbal Learning Test Childrens Version C 479
Current Knowledge
Callosal Apraxia
The CANTAB includes two products: CANTABeclipse
Alien Hand Syndrome and CANTABelect. The CANTABeclipse is composed
Apraxia of 22 tests grouped in specific functional dimensions
measuring executive functioning, visual memory, atten-
tion, semantic/verbal memory, and decision making/
response control. A sixth functional category labeled in-
Callosal Disconnection Syndrome duction is comprised of two very short tests whose pur-
pose is to familiarize examinees with the general idea of
Split-Brain responding by touching the screen and to identify those
examinees who cannot reliably participate in the exami-
nation. Details of each subtest are described in Table 1.
The CANTAB offers users flexibility by providing subtests
that can be selected for administration based upon the
Cambridge Neuropsychological specific clinical question. All subtest stimuli are delivered
Testing Automated Battery nonverbally, although adequate receptive language func-
tions are necessary to understand task demands. The
M ARK A. S ANDBERG publisher regards the majority of CANTAB tests to be
Independent Practice independent of language and culture.
Smithtown, NY, USA CANTABelect is described as a bespoke service
geared toward conducting pharmaceutical clinical trials.
In this case, CCL configures a battery to meet the specific
Synonyms research needs and requirements of the investigator.
Research supporting the use of the CANTAB in the
CANTAB evaluation of brainbehavior relations is reasonably ex-
tensive, with many peer reviewed articles available for
review on the companys website www.cantab.com. Stud-
ies are available supporting the validity and utility of
Description the CANTAB in measuring the impact of pharmaco-
therapy on neuropsychological performance, and in the
The Cambridge Neuropsychological Testing Automated assessment of Attention Deficit Hyperactivity Disorder,
Battery (CANTAB) is a neuropsychological measure de- affective disorders, schizophrenia, senile dementia of the
veloped at the University of Cambridge and currently Alzheimers type and Parkinsons disease. Although consid-
marketed by Cambridge Cognition Limited (CCL). The erable research is available attesting to the usefulness of the
CANTAB is a theoretically derived instrument, predomi- CANTAB with adult populations, it has not been studied to
nantly measuring non-language functions. The current the same extent in the assessment of brain-behavior rela-
version is a Windows-based program operating on a PC tions and their developmental evolution among children.
platform utilizing interactive touch screen technology. More recent is the investigation of the CANTAB as
CANTAB tests are described as having sufficient sensitiv- a tool to assess the neuropsychological functioning of
ity to discern changes in cognitive functioning brought children. Luciana (2003) noted that because the format
about by CNS disorders and medications. and test items can be used in the assessment of all age
groups, the test allows for complex cognitive functions to
be measured from the time they developmentally emerge
Historical Background to the point where they begin to diminish. DeLuca and
colleagues (2003) studied the development of executive
Originally created in the late 1980s to diagnose dementia capacities during childhood and beyond. Using a sample
in elderly individuals (Fray et al., 1996), the CANTAB is of participants ranging in age from 8 to 64 years, they
used, and has been studied, across age groups and with found functional gains in the efficiency of working mem-
patients presenting with both psychiatric and neurologi- ory capacity, planning, and problem solving abilities be-
cal conditions. tween ages 15 and 19 and again from ages 2029 years. In
Cambridge Neuropsychological Testing Automated Battery C 481
contrast, the developmental vulnerability of executive cost will also be prohibitive to many private practitioners
skills was suggested by a decline in CANTAB performance and organizations.
among subjects in the 5064 year old sample (Luciana & Software requirements described by the publisher
Nelson, 2002). include Microsoft Windows 2000 or Windows XP
Reliability studies have been completed internally by operating systems, with minimal hardware requirements
Cambridge Cognition which described test-retest reli- being a PC with 800 MHz Pentium III processor, 256 MB
ability coefficients ranging from 0.40.87. Those findings of RAM, 100 MB free disk space, sound card and speakers,
are consistent with stability coefficients reported by CD-ROM drive, serial port and USB port. A touch screen is
Lowe and Rabbitt (1998). Luciana (2003) reported con- also required, which Cambridge Cognition offers to supply.
sistency coefficients ranging from .73 for a measure of
reaction time latency to .95 for performance on the self-
ordered search task. Future Directions
Limitations of CANTAB include its exclusion of
language based measures, thus leaving the examiner to Pharmaceutical companies have been using CANTAB
supplement the battery with additional tests to assess internationally in multi-site clinical trials for more than
relevant brain-behavior issues such as those involving 20 years. The company is in the process of developing a
laterality (e.g., auditory-verbal memory). CANTABs version of CANTAB for the health care market.
482 C Cancellation Tests
References and Readings individuals make few, if any, errors (Weintraub, 2000).
Standardized versions of cancellation tests, such as the
Elliott, R., McKenna, P. J., Robbins, T. W., & Sahakian, B. J. (1995). Ruff 2 & 7, show good testretest reliability. In addition,
Neuropsychological evidence for frontostriatal dysfunction in on the Ruff 2 & 7 younger adults performed better than
schizophrenia. Psychological Medicine, 25, 619630.
older adults and performances improved with higher
Luciana, M. (2003). Practitioner review: Computerized assessment of
neuropsychological function in children: Clinical and research appli-
levels of education. There were no gender effects
cations of the Cambridge Neuropsychological Testing Automated (Mitrushina, Boone, Razani, & DElia, 2005).
Battery (CANTAB). Journal of Child Psychology and Psychiatry,
44(5), 649663.
Luciana, M., & Nelson, C. A. (2002). Assessment of neuropsychological Clinical Uses
function through use of the Cambridge Neuropsychological Testing
Automated Battery: Performance in 4- to 12-year-old children. Cancellation tests have been shown to be sensitive in
Developmental Neuropsychology, 22(3), 595624.
detecting deficits in attention related to right hemisphere
Prouteau, A., Verdoux, H., Briand, C., Lesage, A., Lalonde, P., Nicole, L.,
Reinharz, D., & Stip, E. (2004). The crucial role of sustained
lesions, traumatic brain injuries, schizophrenia, and
attention in community functioning in outpatients with schizo- AIDS-related cognitive impairment. Cancellation tests
phrenia. Psychiatry Research, 129, 171177. are particularly useful in assessing visuospatial neglect.
Sweeney, J. A., Kmiec, J. A., & Kupfer, D. J. (2000). Neuropsychologic Unlike normal individuals who begin searching for target
impairments in bipolar and unipolar mood disorders on the
stimuli on the left side of the page and systematically
CANTAB neurocognitive battery. Biological Psychiatry, 48, 674685.
move rightward, neglect patients begin on the right
using a disorganized search pattern omitting many targets
on the left side of the page (Weintraub, 2000). By decreas-
Cancellation Tests ing the attentional demands of a letter cancellation test by
reducing the number of nontarget stimuli, right hemi-
R ICHARD F. K APLAN sphere damaged patients showed decreased left-sided ne-
UConn Health Center glect, suggesting that the neglect syndrome is the result of
Farmington, CT, USA an attentional bias (Kaplan et al., 1991).
bells).
Synonyms Definition
Future Directions
Synonyms
A relatively recent emphasis has been placed on the inter-
action of neural and cognitive limitations with factors Reduplication delusion; Reduplicative paramnesia
such as arousal, motivation, and effort.
Short Description or Definition
slightly in some physical characteristic from the found that the delusion actually had a 15% incidence in
genuine person, but the patient may have difficulty their sample of adult inpatients previously diagnosed as
verbalizing the precise nature of this difference. having schizophrenia. Several researchers have found
that about 25% of Alzheimer patients display delusions
involving misidentification of people (e.g., Mendez, C
Categorization Martin, Smyth, & Whitehouse, 1992). Large-scale
epidemiological studies are lacking to date.
Several variations of Capgras syndrome have been identi-
fied. Doppleganger or subjective doubles is the belief that
the patient himself has a double or impersonator
(Christodoulou, 1978). Fregoli syndrome is the belief that
Natural History, Prognostic Factors,
a person is capable of taking on the appearance of others,
Outcomes
while retaining his/her own psychological identity (Courbon
About 58% of Capgras patients who receive adequate
& Fail, 1927). Intermetamorphosis is the belief that people
neurodiagnostic workups are found to display primary
are changing in both physical and psychological identity
psychiatric disorder, uncomplicated by demonstrable
to become another person (Courbon & Tusques, 1932).
neurologic disease (Dohn & Crews, 1986). Although
Capgras syndrome must be distinguished from purely
psychological factors can be important in the production
perceptual or hallucinatory disturbances, and from
of delusions, a critical review of the literature by Malloy
generalized disturbance of cognition. That is, in order to
and Richardson (1994) demonstrated that delusions can
be properly and convincingly diagnosed as a delusion, the
also result from identifiable neurologic disease, from
disturbance must involve a mistaken belief (not merely a
generalized disturbances to focal lesions.
misperception) and must be persistent (not a transitory
They found that Capgras and its variants have been
effect of confusion). For example, in autoscopy, the patient
reported in association with a variety of systemic diseases
experiences a second self, as in the subjective doubles
and diffuse neurologic disorders. Systemic etiologies have
variant of Capgras. However, the phenomena differ in
included: Metabolic disturbances such as myxedema,
that the double is actually seen in autoscopy, rather than
pseudohypoparathyroidism, anemia, hepatic dysfunction,
believed to be active elsewhere, as in subjective doubles. The
B12 deficiency; intoxication and reactions to drugs inclu-
prosopagnosic person may fail to recognize his wife, where-
ding cocaine, chloroquine, disulfiram, digoxin, and
as the Capgras patient will insist that the present person is
lithium; cerebral infections such as encephalitis and
an imposter and that the real wife is somewhere else.
AIDS; subarachnoid hemorrhage; migraine; post-ECT
Patients in confusional states or dementia may express
confusion; minor head trauma; and degenerative dementia.
strange beliefs, but the beliefs typically change from hour
In terms of focal lesions such as tumors and stroke,
to hour and do not persist once the confusion resolves. Thus
that review demonstrated that the right hemisphere or
this problem should not be termed delusional.
bilateral lesions were invariably found on neuroimaging,
Patients with Capgras syndrome are usually described
with no exclusively left-hemisphere lesions. EEG and neu-
as forthcoming and cooperative. Although they insist that
rologic exam findings also implicated right-hemisphere
their delusional beliefs are true, they often admit to puzzle-
pathology. In terms of specific localization, 72% of cases
ment or bemusement regarding aspects of the delusion.
with CT or MRI scans had right frontal, temporal, or
Rather than escalate their defenses by becoming hostile,
frontotemporal involvement. Neuropsychological testing
they are more likely to confabulate an explanation. For
documented spatial, executive, and nonverbal memory
example, a patient with a delusion of duplication (Malloy,
problems, consistent with the right frontotemporal
1991) was asked how she could have two sets of children
localization on neuroimaging studies.
with identical names. She appeared momentarily puzzled
and then stated, My husband was in the Navy, and we
moved around a lot; it was hard to keep that straight.
Neuropsychology and Psychology of
Capgras Syndrome
Epidemiology
Capgras and its variants represent either underidentifica-
Dohn and Crews (1986) observed that Capgras syndrome tion or overidentification of the object of the delusion
was frequently overlooked in psychiatric patients and (Vie, 1930). Thus, in Capgras syndrome, the patient
486 C Capgras Syndrome
Careful clinical interview with both patient and family Reduplicative Paramnesia
members will help to elicit evidence of Capgras delusions.
In the course of family disputes and initial evaluations,
patients may learn to minimize or deny their delusions. References and Readings
Demented patients may forget instances of misidentifica-
tion. Hence, family informants may be extremely helpful. Alexander, M., Stuss, D. T., & Benson, D. F. (1979). Capgras syndrome:
The literature is replete with case reports positing a A reduplicative phenomenon. Neurology, 29, 334339.
American Psychiatric Association. (1987). Diagnostic and statistical
psychodynamic explanation for Capgras, but with no
manual of mental disorders-third edition-revised (DSM-III-R).
workup to rule out neurologic etiology. Since Capgras is Washington, DC: American Psychiatric Press.
commonly associated with neurologic illness; full workup Capgras, J., & Reboul-Lachaux, J. (1923). Lillusion des sosies dans
including neuroimaging and neuropsychological testing is un delire systematise. Bulletin de Societe Clinical Medicine Mentale,
essential. Neuropsychological testing often reveals deficits 11, 616.
Christodoulou, G. N. (1978). Syndrome of subjective doubles. The
in frontal/executive and visuospatial functions (Malloy &
American Journal of Psychiatry, 135, 249251.
Richardson, 1994). Facial recognition and memory Courbon, P., & Fail, G. (1927). Syndrome dillusion de Fregoli et
should be tested to rule out alternative explanations for schizophrenie. Bulletin de Societe Clinical Medicine Mentale, 88,
the patients problems, such as prosopagnosia. 214237.
Carbamazepine C 487
Serious
Common
Carbamazepine
Sedation, dizziness, confusion, headache, nausea, and
J OHN C. C OURTNEY vomiting.
Childrens Hospital of New Orleans
New Orleans, LA, USA
References and Readings
the bloodbrain barrier during the period of hypoxic Messier, L. D., & Myers, R. A. M. (1991). A neuropsychological screening
battery for emergency assessment of carbon-monoxide-poisoned
coma. Following apparent recovery from the acute expo-
patients. Journal of Clinical Psychology, 47(5), 675684.
sure, a delayed toxicity with abrupt onset may appear Prockop, L. D., & Chichkova, R. I. (2007). Carbon monoxide intoxica-
with symptoms of confusion together with motoric tion: an updated review. Journal of the Neurological Sciences, 262,
symptoms resembling Parkinsons disease (incoordina- 122130.
C
tion, muscular weakness, and muscular rigidity). These
neurological signs reflect extensive damage to the basal
ganglia and white matter of the brain with a demyelin-
ation that spares the neuronal axons. Neuroimaging Carboxyhemoglobinemia
scans will most likely appear normal 24 h after exposure;
lesions may begin to appear 2 weeks later. Functional Carbon Monoxide Poisoning
changes associated with these later-appearing lesions are
variable but attempts have been made to relate these to
the severity of the initial exposure. Acute exposure result-
ing in carboxyhemoglobin saturation of 25% or more will
result in later onset cognitive impairments in as many as Cardiac Ultrasound
50% of the patients. The cognitive deficits include agno-
sia, aphasia, and apraxia as well as impaired memory, Echocardiogram
impaired executive function, and a general decrease
in intellect. In addition, visuomotor performance dete-
rioriates. Finally, late-appearing changes in affect are
frequently reported as are obsessivecompulsive tenden- Career Counseling for Individuals
cies and anxiety (Hopkins & Woon, 2006). with Disabilities
Vocational Counseling
Treatment
Neurotoxins
Synonyms
References and Readings Angio
Current Knowledge
Cross References
The arch of the aorta gives off three branches: the
Angioma innominate, the left common carotid, and the left sub-
Glioma clavian. The innominate artery, also known as the bra-
Hemangioma chiocephalic artery, gives rise to the right subclavian
Hemiplegia artery and the right common carotid artery. The com-
mon carotid arteries bilaterally split into internal and
external carotid arteries. The right and left internal ca-
References and Readings rotid arteries, along with the vertebral arteries, branches
of the subclavian artery, are the major blood vessels
Dumolin, C. L., & Hart, H. R. (1986). Magnetic resonance angiography. supplying the brain.
Radiology, 161, 717. As plaque builds in the carotid artery, atherosclerosis,
Faro, S. H., Haselgrove, J. C., Wang, Z., et al. (1993). Carotid MR or hardening of arteries, develops. Carotid stenosis, or
angiography. Radiology, 189, 243.
narrowing of the carotid artery, may also develop and is
Love, B. B., & Biller, J. (2003). Neurovascular System. In C. G. Goetz (Ed.),
Textbook of clinical neurology (2nd ed.) (pp. 395424). Philadelphia: most common at the origin of the internal carotid artery
Saunders. or less commonly, at the distal common carotid artery.
Case Coordination C 491
If the stenosis is severe, it may result in decreased is inserted through the groin and is guided until it
perfusion of brain tissue, and consequently ischemia. reached the site of stenosis. After the location of the
Another mechanism by which ischemia can develop is catheter is confirmed, a balloon inflates and attaches a
by the development of a thrombus over the plaque. metal-mesh stent into the artery. The balloon is then
When this occurs, an embolus may break off and may deflated and the catheter is gently removed. However, C
result in the occlusion of a vessel distally. long-term studies have not proven this method to be
A stroke may develop if the brain is deprived of its superior to a CEA.
blood supply, resulting in a sudden loss of neurologic
function. However, transient ischemic attacks (TIA)
may be the diagnosis if symptoms, such as loss of sensory
or motor function in the extremities, inability to com- Cross References
prehend or initiate speech, or loss of vision in one
eye described a shade coming down over one eye (amau- Ischemia
rosis fugax), last for a few minutes to hours and Magnetic Resonance Angiography
resolve completely in twenty-four hours. Patients with Stroke
a history of stroke or TIA are at increased risk of a
subsequent stroke and should be further evaluated with
imaging studies. According to the North American
Symptomatic Carotid Endarterectomy Trial (NASCET),
References and Readings
patients with symptomatic carotid artery stenosis of
Chaturvedi, S., Bruno, A., Feasby, T., Holloway, R., Benavente, O.,
greater than 70% have a 26% risk of recurrent stroke Cohen, S. N. et al. (2005). Carotid endarterectomy an evidence-
over two years. based review: Report of the Therapeutics and Technology Assess-
The diagnosis of a TIA is made by carotid duplex ment Subcommittee of the American Academy of Neurology.
ultrasound. This type of imaging bounces high-frequency Neurology, 65, 794801.
Roffi, M., & Luscher, T. F. (2008). Management of carotid artery stenosis.
sound waves off blood vessels and the blood within the
Herz, 33, 490497.
lumen to determine blood flow and any abnormalities Rothwell, P. M., Eliasziw, M., Gutnikov, S. A., Fox, A. J., Taylor, D. W.,
within the vessels themselves. Other modalities useful in Mayberg, M. R. et al. (2003). Analysis of pooled data from the
the diagnosis of a TIA include computed tomography randomised controlled trials of endarterectomy for symptomatic
angiography (CTA), magnetic resonance angiography carotid stenosis. Lancet, 361, 107116.
(MRA), and angiography. In each of these, a contrast
dye, or gadolidium in the case of MRA, is injected into
the arteries. This helps identify any areas of poor blood
flow and determine the degree of stenosis.
If there is greater than 70% blockage of the carotid CARS
artery, a CEA is recommended if the patient is symptomatic
with a history of TIA or nondisabling stroke, or even if Childhood Autism Rating Scales
the patient is asymptomatic. However, a CEA should not
be performed during a stroke or TIA or if the patient
experiences a stroke that leaves him severely disabled.
If there is less than a 70% blockage and the patient
is asymptomatic, a CEA is not recommended. In this
case, the patient should be treated medically with a baby
CAS
aspirin daily. In addition, other risk factors that may cause
Cognitive Assessment System
further damage to the vessels, such as smoking, diabetes,
hypertension, and hypercholesterolemia, should be treated
medically. A CEA is only recommended for 5069% steno-
sis if the patient has had recurrent TIAs unresponsive to
medical management.
For patients that are high risk for the CEA, other Case Coordination
surgical interventions, such as angioplasty and stenting,
can be considered. During this procedure, a catheter Case Management
492 C Case Management
References and Readings that has become too difficult or is perceived as being too
difficult. Typically, persons experiencing catastrophic
Case Management Society of America (2002). CMSA standards of reactions are not fully aware of their increasing lability
practice. Little Rock, AR: Case Management Society of America. and cannot respond to logic and reason, but they
Chan, F., & Leahy, M. (2005). Health care and disability case management,
benefit from decreased demands and calm reassurance. C
(2nd ed.). Lake Zurich, IL: Vocational Consultants Press.
Huber, D. (2005). Disease management: A guide for case managers.
Catastrophic reactions were initially thought to be
St. Louis: Elsevier Saunders. associated only with dominant hemisphere damage and
Mullahy, C. (2004). The case managers handbook (3rd ed.). Sudbury, MA: accompanying language impairment, but they can follow
Jones and Bartlett. any neurological problem that interferes with adaptive
coping and executive functioning.
Catastrophic condition
Catatonic Behavior
Definition
S OLOMON K ALKSTEIN , FARZIN I RANI
This term coined by Goldstein (1948) describes acute University of Pennsylvania
distress, agitation, and disorganized behavior occurring Philadelphia, PA, USA
when overwhelmed by a situation with which the person
cannot cope, typically following brain injury or other
neurological impairment. Rapid and extreme anxiety, Synonyms
depression, and frustration can result when a person
becomes over-stimulated or overwhelmed with a task Catatonia
494 C Catatonic Behavior
subtype within schizophrenia persisted through early psychopharmacological studies. Neuroimaging studies have
and recent versions of the DSM. Only with the publica- not been conclusive and have revealed various cortical and
tion of DSM-IV was consideration given to catatonia subcortical brain regions to be associated with the illness, a
occurring within the context of other psychiatric and finding that may explain observed clinical differences
medical conditions. This was due to important articles in symptom presentation (Weder et al., 2008). Neuropsy- C
published in the 1970s, which established co-occurrence chological evaluation of catatonic psychiatric inpatients
of catatonia and mood disorders (Abrams & Taylor, as compared with a psychiatric control group revealed
1976) and emphasized the association between catatonia relatively poorer performance on measures of working
and neurological or general medical conditions (Gelen- memory and those visualspatial abilities related to right
berg, 1976). Several reports have also suggested the pres- parietal functioning. Additionally, significant differences
ence of a genetic form of catatonia not captured by between catatonic patients and healthy controls were
DSM-IV TR criteria (Taylor & Fink, 2003). Thus, the observed on attentional and executive tests associated with
conceptualization and classification of catatonia con- frontal functioning. These results suggest attentional-motor
tinues to evolve. and frontoparietal dysfunction in catatonia (Northoff,
An additional area of controversy involves categoriza- Nagel, Danos, Leschinger, Lerche, & Bogerts, 1999).
tion. Advocates have argued for DSM reclassification of
catatonia into a syndrome rather than a subtype of schizo-
phrenia. They claim that the current model fails to ade- Evaluation
quately recognize catatonia in other psychiatric illnesses
and limits treatment of catatonic behavior to protocols Although DSM-IV requires two of the six symptoms of
focusing on antipsychotic drugs. Fink and Taylor (2003, catatonic behavior listed previously in order to establish
2006) have therefore proposed a change in nomenclature, presence of catatonia, definitions of catatonia, as well as
suggesting that catatonia be reclassified separately from its basic signs and symptoms, are still the subject of
other syndromes, akin to delirium or dementia, with three debate and no authoritative set of criteria has been uni-
syndrome subtypes (nonmalignant, delirious, and malig- versally accepted (Caroff & Ungvari, 2007). Several rating
nant) and four specifiers (secondary to: mood disorders, scales have been developed to assess for the presence of
general medical conditions or toxic states, neurological dis- catatonia; however, the number of signs and symptoms
orders, or psychotic disorders). However, this reclassifica- that are included range from 10 to 40 for almost all
tion system remains controversial. instruments (Taylor & Fink, 2003). Several reviews have
Numerous risk factors for catatonia have been listed the most commonly listed signs and symptoms of
reported including history of perinatal infections, epilep- the syndrome as mutism, posturing, negativism, staring,
sy, medication effects, and frontal or basal ganglia dis- rigidity, echophenomena, stereotypes, grimacing, and
eases (Weder et al., 2008). Additionally, various aspects of perseveration (Weder et al., 2008). In terms of the num-
comorbid psychiatric conditions including age of onset ber of symptoms required to justify a diagnosis, mini-
and severity of symptoms are considered risk factors and mum thresholds vary among scales. While some scales
contribute significantly to prognosis. Although highly resemble the DSM-IV TR in requiring the presence of
variable, overall prognosis appears to be relatively better 2 or 3 symptoms out of a list of 11 or 12 (Lohr &
for recurrent catatonia and mood disorders with cat- Wisniewski, 1987; Rosebush, Hildebrand, Furlong, &
atonia as compared with catatonic schizophrenia. One Mazurek, 1990), other instruments assign greater weight
review study indicated that while acute treatment prog- to certain cardinal symptoms over other secondary symp-
nosis is excellent, long-term prognosis depends on the toms (Bush, Fink, Petrides, Dowling, & Francis, 1996).
underlying condition that elicited catatonia (Taylor & Other scales group symptoms by category (i.e., motor,
Fink, 2003). affective, and behavioral) and require a symptom from
each category in order to establish a diagnosis (Northoff
et al., 1999). Most authors accept a range from several
Neuropsychology and Psychology to 24-hours duration as being necessary in order to defin-
of Catatonic Behavior itively establish the presence of catatonia (Weder et al.,
2008). Attempts have been made to identify individual
Although the pathophysiology of catatonia remains poorly clusters within the catatonia syndrome based on dis-
understood, both the GABA and dopaminergic neurotrans- crete symptom-groups and duration of illness (acute/
mitter systems have been implicated in numerous chronic); however, further research in this area is
496 C Catecholamines
necessary before the existence of subtypes can be estab- Bush, G., Fink, M., Petrides, G., Dowling, F., & Francis, A. (1996).
Catatonia. I. Rating scale and standardized examination. Acta Psy-
lished (Weder et al., 2008).
chiatrica Scandanavica, 93, 129136.
Caroff, S. N., & Ungvari, G. S. (2007). Expanding horizons in catatonia
research. Psychiatric Annals, 37(1), 79.
Treatment Fink, M., & Taylor, M. A. (2003). Catatonia: A clinicians guide to diagnosis
and treatment. Cambridge: Cambridge University Press.
Fink, M., & Taylor, M. A. (2006). Catatonia: Subtype or syndrome in
Treatment for catatonic symptoms differs depending on DSM? American Journal of Psychiatry, 163, 18751876.
the underlying cause. Catatonia is currently viewed by Gelenberg, A. J. (1976). The catatonic syndrome. Lancet, 1(7973),
most clinicians from a predominantly biological frame- 13391341.
work (Penland, Weder, & Tampi, 2006). Generally, benzo- Lohr, J., & Wisniewski, A. A. (1987). Catatonia. In J. Lohr & A. A.
Wisniewski (Eds.), Movement disorders: A neuropsychiatric approach.
diazepines such as lorazepam are considered to be the first
New York: Guilford Press.
treatment of choice. If patients fail their initial trial, the Northoff, G., Nagel, D., Danos, P., Leschinger, A., Lerche, J., & Bogerts, B.
use of bilateral electroconvulsive therapy is usually con- (1999). Impairment in visual-spatial function in catatonia:
sidered. Although these are the best-studied treatments A neuropsychological investigation. Schizophrenia Research, 37,
of the syndrome, the efficacy of other approaches, such 133147.
Penland, H. R., Weder, N., & Tampi, R. R. (2006). The catatonic dilemma
as transcranial magnetic stimulation, continues to be
expanded. Annals of General Psychiatry, 5(14), 19.
explored. Catatonic schizophrenia may be treated by a Rosebush, P. I., Hildebrand, A. M., Furlong, B. G., & Mazurek, M. F.
variety of pharmacotherapeutic and psychotherapeutic (1990). Catatonic syndrome in a general psychiatric inpatient popu-
methods. Hospitalization may be necessary to protect lation: Frequency, clinical presentation, and response to lorazepam.
the patients safety and supportive psychotherapy and Journal of Clinical Psychiatry, 51, 357362.
Taylor, M. A., & Fink, M. (2003). Catatonia in psychiatric classification:
family education may help patients and their families
A home of its own. American Journal of Psychiatry, 160, 12331241.
adjust to problems created by the illness. Other support- Weder, N. D., Muralee, S., Penland, H., & Tampi, R. R. (2008). Catatonia:
ive services as sheltered workshops and special education A review. Annals of Clinical Psychiatry, 20(2), 97107.
may also be helpful. When catatonic symptoms are due to
a mood disorder, episodes may be treated with mood
stabilizers or antidepressant medications. Catatonic
symptoms caused by a medical disorder require correct Catecholamines
diagnosis of the underlying medical condition, followed
by appropriate treatment. For example, levodopa and M ARLA S ANZONE
amantadine (symmetrel) have shown some effectiveness Independent Practice
in reducing catatonic symptoms due to postencephalitic Annapolis, MD, USA
Parkinsons disease. Hospitalization and careful supervi-
sion of persons with catatonic symptoms may be neces-
sary to ensure that they do not harm themselves or others Synonyms
and to prevent malnutrition, exhaustion, or fever.
Adrenergic agonists; Direct- and indirect-acting adrenergic
receptor agonists; Dopamine agonists; Dopaminergic
Cross References agonists; Sympathomimetic amines/drugs/agents/
compounds
Delusion
Mood Disorder
Schizophrenia Definition
Catecholamines are sympathomimetic amines, a group involuntary movement disorder and tends to develop
of compounds including dopamine, epinephrine, and during middle adulthood. Anxiety can precipitate and
norepinephrine whose molecular structure is similar to exacerbate the amplitude of the tremor. It is generally
that of a larger class of neurotransmitters, the monoa- treated with beta blockers such low doses of propranolol
mines. The specific molecular structure of catecholamines and with lesser efficacy with Atenolol and anxiolytics C
is a benzene ring with two hydroxyl groups, an intermedi- such as alprazolam (Kaufman, 2007).
ate ethylamine to the side and an amine terminal group. Chemodectoma (nonchromaffin paraganglioma)
The chromaffin cells in the adrenal medulla and sympa- Benign chemoreceptor system tumors. The most common
thetic nervous system postganglionic fibers are the primary types are glomus jugulare tumor and carotid body tumor.
site of catecholamine production (Hoffman, 2004). Dopamine-b-hydroxalase Deficiency Severe orthostatic
During times of physiological and/or psychological hypotension of a congenital nature caused by the inability
stress neurons in the central and peripheral sympathetic to generate the enzyme, dopamine-b-hydroxalase.
nervous systems secrete catecholamines via hypothalamic Familial Paraganglioma Syndrome Genetic slow-
and adrenomedullary activation. Catecholamine toxicity, growing chromosomal (11q23) benign tumors primarily
also referred to as catecholamine dump or storm can of the head and neck. These unusual conditions are inher-
occur from trauma, causing over-stimulation, excessive ited from the father. Disfiguring facial swelling, hearing
production and circulation of catecholamines in the loss, tinnitus, pain, persistent cough, or other head/neck
central nervous system, and/or lesions of nuclei affecting anomalies as a function of cranial nerve damage from
the sympathetic nervous system in the brainstem. the tumor.
Catecholamines affect metabolic rate, temperature Neuroblastoma, produces catecholamines, and follow-
regulation, smooth muscle functions, cardiovascular and ing brain tumors is the second most common solid tumor
nervous systems. Epinephrine and norepinephrine auto- in childhood. Testing for the catecholamine metabolites,
nomically prepare the body for crisis such as cold, fatigue, vanillylmandelic acid and homovanillic acid, are
danger, and shock. Dopamine is produced as an interme- identified in the urine.
diate process in the synthesis of epinephrine and is critical Pheochromocytoma is a benign tumor that secretes
to the regulation of many neurological processes, includ- norepinephrine and epinephrine. It originates in
ing smooth fine and gross motor movements, energy sympathetic paraganglia or adrenal medulla.
regulation, motivation, executive functioning and formal Tetrahydrobiopterin deficiency is a genetic condition
thought regulation. Sympathomimetic drugs used to treat that results in the inability to manufacture the enzymes
conditions associated with these biological functions required to synthesize catecholamines. This leads to a defi-
mimic the actions of epinephrine, norepinephrine or do- ciency of the neurotransmitters, norepinephrine, epineph-
pamine (Sympathomimetic drug, 2008; Venes, Thomas, rine, and dopamine (Catecholamines, 2002; Myers, 2006).
Egan, Houska, 2001). Such drugs include those used to
treat a variety of abnormalities associated with central and
sympathetic nervous system processes (e.g., blood pressure, Mechanisms of Action
cardiac anomalies, premature labor, glaucoma, bronchitis,
asthma, emphysema, blood glucose metabolism, movement The adrenergic or sympathomimetic drugs act at
disturbances and psychosis). In addition, numerous disor- sympathetic postganglionic terminal by activating the
ders involve catecholamine disturbances of congenital, ge- catecholaminergic hormones, epinephrine (adrenaline),
netic, or familial origin such as dopamine-b-hydroxalase norepinephrine (noradrenaline), and/or dopamine in
deficiency, paraganglioma syndrome, and tetrahydrobiop- various ways. Some compounds have cross-reactivity
terin deficiency, but also those with unknown precipitating such that they act on catecholamines by more than one
mechanisms, including chemodectina, neuroblastoma, and mechanism of action (Sympathomimetic drug, 2008).
pheochromocytoma (Catecholamines, 2002). Directly activating adrenergic postsynaptic receptors
(Direct-Acting Adrenergic Agonists) include a-adrenergic
agonists and b-adrenergic agonists. There are five types:
Catecholamine-Related Conditions a1, a2, b1, b2, and b3. Adrenergic agonists stimulate one or
more of these receptor types. Adrenergic antagonists
Benign Familial Tremor/Essential Tremor is an inherited inhibit one or more of the actions of these receptors.
autosomal dominant condition suggestive of excessive Some agents have stimulating and blocking actions on
systemic adrenergic activity. It is the most common different receptors simultaneously. Receptor selectivity
498 C Catecholamines
Primary
neurotransmitter
actions Mechanism of action Examples of compounds Clinical uses
Alpha (a) Direct-acting alpha- Alpha (a1, a2) adrenergic Used to treat glaucoma by decreasing the
adrenergic adrenergic agonists agonists production of aqueous fluid; as vasopressors, nasal
agonists Promote decongestants and to dilate the pupil for eye
vasoconstriction, exams
mydriasis, body
tissue-building,
inhibition
of endogenous
testosterone release
a1 Agonists a1 Agonists Oxymetazoline, Used to treat anemias caused by deficient
stimulate phenylephrine production of red blood cells, aplastic anemia,
phospholipase C hypotension, shock, paroxysmal supraventricular
activity tachcardia
a2 Agonists a2 Agonists inhibit Clonidine (a2 and a2 Agonists reduce sympathetic nervous system
adenylyl cyclase imidazoline-I1 agonist) activation. They are used to treat opiate and
activity Guanfacine (affinity for alcohol withdrawal symptoms, as
preference for a2a antihypertensives, gestational hypertension, to
adrenoceptor) decrease peripheral resistance and as sedatives.
More recently they are being used to treat
Methyldopa is
neuropathic pain, sleep hyperhidrosis, and off-
approximately 50%
label, to counter the side effects of stimulant
absorbed from the gut,
medications
metabolized in the
intestines and liver and
alpha-methylnorepineprine,
its metabolite, stimulates
central a2- receptors
Mixed a, b1, Undetermined or Epinephrine, amidephrine, Mixed alpha- beta- agonists such as epinephrine
b2 agonists mixed alpha- beta- ergotamine, are used to treat anaphylaxis, acute asthmatic
stimulation norepinephrine, synephrine reactions, cardiac arrest, open-angle glaucoma
and nasal congestion. It stimulates alpha, beta1
and beta2 adrenergic receptors, increasing cardiac
output, dilating bronchials, skeletal muscle
vasulature, and pupils dilation due to constriction
of dilatory muscles
b Antagonists Direct-acting beta- Cardiac stimulants Beta blockers are used to treat heart block,
(beta blockers) adrenergic agonists ventricular tachcardia, cardiac arrest, asthma,
block the action of stimulate adenylyl chronic bronchitis, emphysema, hypovolemic and
epinephrine and cyclase activity and septic shock, low cardiac output or hypoperfusion,
norepinephrine open calcium congestive heart failure
channels
b1 Agonists Direct-acting b1- Albuterol, dobutamine Asthma, Chronic Obstructive Pulmonary Disease
adrenergic agonists (COPD), sinus congestion
Catecholamines C 499
Primary
neurotransmitter
actions Mechanism of action Examples of compounds Clinical uses
b2 Agonists Direct-acting b2- Metaproterenal, formoterol, A moderately selective b2 agonist. It acts on C
adrenergic agonists salmeterol, terbutaline smooth muscle in the lungs, uterus, and skeletal
stimulate muscle vasculature. Used to treat asthma, COPD,
postsynaptic premature labor
b2 receptors of the The enzyme intracellular adenyl cyclase catalyzes
intracellular enzyme, the conversion of ATP to cAMP. Elevated cAMP
adenylyl cyclase. levels are associated with bronchial
Closes calcium smooth muscle relaxation and regulation of mast
channels, relaxing cells activation
smooth muscle
Mixed b Isoproterenol (b1 and Cardiogenic shock, bradyarrhythmias, heart block,
antagonists b2 agonist) cardiac arrest, brochospasm associated with
anesthesia, asthma, chronic
bronchitis, emphysema, hypovolemic and
septicemic shock
COMT-inhibitors Indirectly inhibiting Entacapone, tolcapone Adjunctive Parkinsons treatment
epinephrine and
norepinephrine
metabolism
Dopamine Stimulate production Bromocriptine, Hypertensive crisis through vasodilation in
agonists and release at D1 apomorphine, fenoldopam coronary, renal, mesenteric and peripheral arteries
receptors Pramipexole (pre- and Pramipexole used to treat idiopathic parkinsonism
postsynaptic D2 and 3 signs. It stimulates caudate neurons
receptor agonism) by D3 agonism
Prolongs Levodopa (I-Dopa) Parkinsonianism Levodopas efficacy is as a
dopaminergic activity prodrug. It is a precursor of catecholamines
when combined with
COMT-inhibitors
and dopa
decarboxylase
inhibitors
(e.g., benserazide
or carbidopa)
Dopamine Increase Cocaine, Methylphenidate Cocaine used recreationally and highly subject to
Reuptake norepinephrine Amphetamines abuse. Ophthalmically used as local anesthetic.
Inhibitors (DRIs) release at central Amphetamines and methylphenidate are used
noradrenergic primarily to treat ADHD, narcolepsy, occasionally
neurons. Release of in geriatric depression, post-stroke and cancer
dopamine at to increase alertness and counteract
mesolimic areas in chemotherapy lethargy. All DRIs and stimulant-
high doses. acting drugs elevate mood, increase focusing
ability and physical energy in most, increase
respiration and heart rate, blood pressure and
decrease appetite. They can cause euphoria,
rebound depression and anxiety particularly
if over-used or used inappropriately to treat
depressive disorders
500 C Catecholamines
Primary
neurotransmitter
actions Mechanism of action Examples of compounds Clinical uses
Dopamine Blockade of Antipsychotic agents Schizophrenia, Bipolar I, mania
antagonists dopamine (more D2
than D1 receptor
affinity).
Moderate a
adrenergic and
histaminic
antagonism.
Monoamine Stimulate production Phenelzine (Nardil) Treatment resistant depression, atypical
Oxidase Inhibitors and release of depression, panic disorder
(MAOIs) catecholamines in
CNS
Chronic use Tranylcypromine (Parnate)
downregulates a2 or
b adrenergic and
serotonin receptors
Norepinephrine Prevent re-uptake of Most non-SSRI Atypical depression, dysthymia, anxiety, ADD, post
reuptake norepinephrine at antidepressants: Tricyclic traumatic stress disorder
inhibitors postsynaptic antidepressants (TCAs
receptors mixed catecholamine
stimulation and inhibition)
Atomoxetine (Strattera)
serotonin and
norepinephrine reuptake
inhibition Duloxetine
(Cymbalta) Venlafaxine
(Effexor) selective
norepinephrine reuptake
inhibition
Norepinephrine- Increase availability of Bupropion, pyrovalerone, ADHD, depression, chronic fatigue, obesity
dopamine monoamines, mazindol (also classified as a
reuptake inhibitor dopamine and TCA), phenethylamines
norepinephrine, due
to inhibiting re-
uptake, causing a
stimulating effect
Norepinephrine Stimulate production Mirtazapine (classified as a Depression, mixed anxiety-depression with sleep
releasers and release of tetracyclic and a disruption
catecholamines noradrenergic specific
serotonergic antidepressant
NaSSA)
Mianserin is a tetracyclic
antidepressant
Catecholamines C 501
Primary
neurotransmitter
actions Mechanism of action Examples of compounds Clinical uses
Stimulants Stimulate direct Amphetamine salts, ADD/ADHD increases alertness, concentration, C
production and methylphenidate, focus, attention, executive functions, endurance
release of dexamphetamine, mentally and physically. Diminishes verbal and
catecholamines, dextroamphetamine, physical impulsivity
particularly phentermine Narcolepsy to help maintain alertness
norepinephrine and
Major depressive disorder occasionally used to
dopamine from
augment antidepressant treatment
storage vesicles
Weight management phentermine is used to
suppress appetite
Also increase Schedule IV controlled substances in the US
norepinephrine and
dopamine levels via
reuptake inhibition
and binding to the
MAO protein
transporter
Stimulate production Modafinil, adrafinil Narcolepsy to maintain alertness, and counteract
and release of abnormal states that diminish alertness
catecholamines Night shift work to counteract fatigue
Over-the-counter Synthetic and plant- Ephedrine, To suppress appetite, increase alertness, enhance
stimulants based herbal pseudoephedrine, MDMA, athletic performance
preparations with cocaine, ephedra, Ma huang, Cocaine, a tropane derivative, is made from South
significant abuse guarana American coca bush leaves. It is occasionally used
potential thought to legally as an ophthalmic local anesthetic, but no
stimulate release of longer prescribed for its stimulant properties
catecholamines
Aminoff (2004); Hoffman (2004); Kaufman (2007); Katzung (2004); Psychoactive drug (2009); Stimulant (2009); Sympathomimetic drug (2008);
Williams and Turner (2008).
addresses strength of affinity toward a particular receptor norepinephrine. If the enzyme is inhibited, it prevents the
rather than absolute specific selectivity of one receptor only. removal of epinephrine and norepinephrine from the sys-
Direct-acting dopamine agonists are used to treat tem which induces sympathomimetic effects. Similarly,
hypertensive crisis and parkinsonian signs and symptoms. with COMT-Inhibitors, the enzyme catechol-O-methyl
Indirect-acting, norepinephrine transporter antagonists transferase (COMT) is an intracellular enzyme in the
such as ephedra, cocaine, and amphetamines. They block postsynaptic neuron that degrades epinephrine and nor-
and reverse the activity of the norepinephrine transporter epinephrine. This enzyme effectively deactivates them by
proteins. These proteins functionally terminate the effects of inducing S-adenosyl methionine (SAM) to add a methyl
norepinephrine and to a lesser degree, dopamine, by remov- group to the catecholamines. The COMT-Inhibitors
ing these neurotransmitters from the synapse and repacka- prevent this deactivation by blocking the breakdown/
ging them in their vesicles for later use (Katzung, 2004). reuptake process, and enabling the utility of the catecho-
Indirectly inhibiting epinephrine and norepinephrine lamines (Catechol-O-methyl transferase, 2009).
metabolism such as COMT-Inhibitors (e.g., entacapone Stimulating production and release of catecholamines as
and tolcapone) and MAOIs such as Parnate and Nardil do MAOIs and amphetamines. These agents are known to
(Aminoff, 2004) that raise the level of all the catecholamines increase cardiac output and blood pressure as a function of
in the blood. Monoamine oxidase (MAO) is the enzyme constricting peripheral blood vessels which increases the
primarily responsible for metabolism of epinephrine and force and rate of cardiac muscle contractions. They can
502 C Categorical Data
also lead to elevations in blood lipid levels due to fat Catecholamines. (2009b). In Merriam-Webster online dictionary.
Retrieved January 13, 2009, from http://www.merriam-webster.
catabolism and blood glucose levels due to glycogenolysis
com/dictionary/catecholamines
of skeletal and hepatic muscle (Sympathomimetic drug, Hoffman, B. B. (2004). Adrenoceptor-activating & other sympathomi-
2008). As discussed above, monoamine oxidase (MAO) is metic drugs. In B. Katzung (Ed.), Basic and clinical pharmacology
the enzyme primarily responsible for degrading catechola- (9th ed., pp. 122141). New York: Lange Medical Books/McGraw-
mines. It has a half-life of several minutes. MAOIs and Hill.
Katzung, B. G. (2004). Introduction to autonomic pharmacology.
psychoactive stimulants bind to the enzyme, inhibiting
In B. Katzung (Ed.), Basic and clinical pharmacology (9th ed.,
the break down of the catecholamines, and largely for this pp. 7593). New York: Lange Medical Books/McGraw-Hill.
reason the effects of amphetamines last longer than those of Kaufman, D. M. (2007). Clinical neurology for psychiatrists (6th ed.,
cocaine or crack. Amphetamines are among the adrenergic pp. 97, 190, 193, 430431). Philadelphia: Saunders Elsevier.
agents with dual mechanisms of action or cross-reactivity. Myers, T. (2006). Catecholamine (definition). In Myers, T. (Ed.),
Mosbys dictionary of medicine, nursing & health professions
The production of all the catecholamines, dopamine, epi-
(7th ed., pp. 323). Missouri: Mosby Elsevier.
nephrine, and norepinephrine is stimulated and the reup- Psychoactive drug. (2009). In Wikipedia. Retrieved January 16, 2009, from
take, particularly of norepinephrine, is inhibited. http://en.wikipedia.org/wiki/Psychoactive_drug
Stimulant. (2009). In Wikipedia. Retrieved January 15, 2009, from http://
en.wikipedia.org/wiki/Stimulant
Specific Compounds and Properties Sympathomimetic drug. (2008). In Wikipedia. Retrieved January 13,
2009, from http://en.wikipedia.org/wiki/Sympathomimetic
Venes, D., Thomas, C., Egan, E., & Houska, A. (2001). Catecholamine
All sympathomimetic amines can be conceptualized to be (definition), In D. Venes, et al. (Eds.), Tabers medical dictionary
part of a broad group of stimulant drugs due to their (19th ed., pp. 357). Philadelphia: FA Davis Company.
sympathetic nervous system and/or central nervous Williams, F. M., & Turner, T. J. (2008). Adrenergic pharmacology:
system activating properties. However, due to multiple Biochemistry & physiology of adrenergic function. In D. E. Golan,
A. H. Tashjian, E. J. Armstrong, & A. W. Armstrong (Eds.), Principles
mechanisms of action sympathomimetics are classified
of pharmacology: The pathophysiologic basis of drug therapy
accordingly and as a function of their use in the treatment (pp. 129139). Philadelphia: Lippincott Williams & Wilkins.
of specific conditions (Table 1).
Numerous studies have demonstrated the CTs ability related to perseveration, attention, and possibly other
to discriminate brain damaged from normal examinees, aspects of their performance on the CT.
with analyses indicating statistically significant discrimi-
nation at the 0.05 to 0.001 levels. However, utilizing only
the suggested cut-off score of 50 errors to separate groups
Cross References
has resulted in a false positive rate of as high as 18%. The
need to correct for age and education has been firmly
COWA
established, as these variables account for a significant
Ruff Figural Fluency
variance of CT error scores. Including these corrections
Tower of London
significantly reduces classification errors (Heaton, Grant,
Trail Making Test
& Matthews, 1991).
Wisconsin Card Sorting Test
The original intent of Halstead to utilize the CT as a
measure of frontal lobe functioning has not been vali-
dated by researchers. Also, the CT has been found to be
relatively ineffective in lateralizing damage to the brain. References and Readings
The test, as is the case with many neuropsychological
instruments, is significantly impacted by severe psychiat- Choca, J. P., Laastch, L., Wetzel, L., & Agresti, A. (1993). The
ric disturbance and inadequate effort. Halstead Category Test: A fifty year perspective. Paper presented at
the annual meeting of the American Psychological Association,
Toronto, Canada.
Halstead, W. C. (1940). Preliminary analysis of grouping behavior in
Clinical Uses patients with cerebral injury by the method of equivalent and non-
equivalent stimuli. American Journal of Psychiatry, 96, 12631294.
The category test is generally grouped in most textbooks Halstead, W. C., & Settlage, P. H. (1943). Grouping behavior of normal
persons and of persons with lesions of the brain. Archives of Neurol-
in the field with other measures of executive function-
ogy and Psychiatry, 49, 489503.
ing. This concept can be loosely defined as the ability to Heaton, R. K., Grant, I., & Matthews, C. G. (1991). Comprehensive norms
utilize feedback to organize and plan ones approach to for an expanded Halstead-Reitan Battery: Demographic corrections,
deal with a novel problem-solving task. Taking this defi- research findings, and clinical applications. Odessa, FL: Psychological
nition, the CT seems to qualify as a measure of this Assessment Resources.
Kupke, T. (1983). Effects of subject sex, examiner sex, and test apparatus
ability. In fact, clinicians who use the test typically in-
on Halstead Category and Tactual Performance Tests. Journal of
clude it in their battery of tests as an executive function- Consulting and Clinical Psychology, 51, 624626.
ing measure. This approach is supported by the test Minassian, A., Perry, W., Carlson, M., Pelham, M., & DeFilippis, N.
format and some research, but should not involve reli- (2003). The Category Test Perseveration, Loss of Set, and Memory
ance on the CT as the sole measure of executive func- scales: Three new scales and their relationship to executive function-
ing measures. Assessment, 10, 213221.
tioning. As previously discussed, the test does not
identify only frontal lobe dysfunction, but is sensitive
to any type of brain injury.
Unfortunately, the only validated and normed score
for the CT is the total error score. The original cut-off of
50 errors should not be used without also looking at age Caudate Nucleus
and education adjusted scores. The sex of the examinee
does not appear to have a significant effect on test per- S EEMA S HROFF
formance, and although norms are frequently broken Virginia Commonwealth University
down by sex, this demographic variable can largely be Richmond, VA, USA
ignored. The clinician can view CT performance as in-
dicative of the overall cortical integrity of his or her
patient. There is also some evidence that the test assesses Definition
fluidity of thinking and may be related to rehabilitation
outcome. The caudate nucleus is an arcuate mass deep into the
Ongoing research will hopefully provide the clinician cortical hemispheres forming a part of the basal ganglia.
more information concerning his or her patients abilities Apart from being involved in the smooth orchestration of
Caudate Nucleus C 505
motor actions, it has been recently implicated in other the direct pathway that increases motor activity. An
functions like learning, memory, emotion, and language. indirect pathway, involving the subthalamic nucleus,
decreases motor activity. The activity of these pathways
is further modulated by dopamine from the nigrostrial
Nomenclature tract and ACh from the interneurons. C
Striatum/Neostriatum = Caudate Nucleus (nucleus
caudatus) + Putamen Physiology and Pathophysiology
Corpus striatum (Dorsal division) = Caudate Nucleus +
Putamen + Globus Pallidus The caudate nucleus is mainly involved in motor planning
and execution of smooth movement. Degeneration of the
caudate is associated with dyskinesias and involuntary
Current Knowledge movements. Caudate pathology manifests in choreiform
movements (brisk, jerky, and purposeless), as seen in
Anatomy Sydenhams chorea (minute caudate hemorrhages and
capillary emboli post streptococcal infection in children)
The caudate nucleus has a globular head, tapering body, or Huntingtons disease (an adult onset degenerative he-
and down curving tail. The head lies in the floor and reditary disorder). Athetosis (slow, sinuous, and aimless
lateral wall of the anterior horn of the lateral ventricle, movements involving distal musculature) is also seen in
whereas the body runs in the floor. Medially, the caudate striatal pathology. Loss of nigrostriatal input in Parkin-
nucleus abuts the thalamus; in the floor of the ventricle, sons disease leads to akinesia and rigidity characteristic of
this junction forms a groove known as the sulcus the disease.
terminalis that lodges the stria terminalis of the choroids Recently, the caudate nucleus has been implicated in
plexus. The corpus callosum runs above the head and functions other than motor planning. Using functional
body. The caudate nucleus is separated from the lenti- imaging, Crinion et al. (2006), have shown activation of
form nucleus by the anterior limb of the internal capsule. the caudate nucleus during language processing in bilin-
gual persons. Initial degeneration in the caudate is
thought to be responsible for the dysphoria in early Hun-
Histology tingtons disease (Paradiso et al., 2008). The caudate nu-
cleus has also been theorized to envision positive
The caudate nucleus and putamen show similar histology. emotional events in the near future (DArgembeau et al.,
Small and large neurons (in a 20:1 ratio) with spherical or 2007) and provide complimentary information of the
ovoid dendritic fields populate the neostriatum making outcomes of actions (Lau & Glimcher, 2007).
connections within the nucleus or with the globus palli-
dus. Most small neurons contain GABA and either
enkephalin or substance P. Enkephalinergic neurons ex- Cross References
press D2 dopamine receptors whereas those with sub-
stance P have D1 receptors. Large neurons with spiny Globus Pallidus
dendrites contain acetylcholine esterase (AChE) and cho- Putamen
line acetyltransferase (CAT). Thalamus
Circuitry
References and Readings
Corticostriate, thalamostriate, and nigrostriate fibers
form the major input to the striatum. Cortical areas Crinion, J., Turner, R., Grogan, A., Hanakawa, T., Noppeney, U.,
Devlin, J. T., et al. (2006). Language control in the bilingual brain.
involved in motor planning and execution project to the
Science, 312(5779), 15371540.
caudate and putamen. Striatal neurons relay to the globus D Argembeau, A., Xue, G., Lu, ZL., Van der Linden, M., & Bechara, A.
pallidus that sends information to the thalamus (VA/VL), (2007). Neuroimage [Epub ahead of print].
which in turn feeds back to cortical motor areas forming Grays Anatomy (1995). (38th ed.). Pearson Professional Limited.
506 C Causal Modeling
CEA
Carotid Endarterectomy
Causal Modeling
Path Analysis
Ceiling Effect
S ANDRA B ANKS
Cavernoma Allegheny General Hospital
Pittsburgh, PA, USA
Angioma, Cavernous Angioma
Synonyms
Cavernous Hemangioma Ceiling level
least five more words would allow for an investigation of cut-off scores may be more appropriate with medical
that individuals upper limit of learning or memory populations.
capacity.
Historical Background C
Cross References
In 1971, the National Institute of Mental Health (NIMH)
Floor Effect began development of the CES-D to measure depressive
Testing the Limits symptoms in epidemiological research (Brantley,
Mehan, & Thomas, 2000). Selection of original compo-
nents of depressive symptomatolgy (e.g., depressed
mood, worthlessness) was based on factor analytic stud-
Ceiling Level ies and clinical literature (Radloff, 1977); specific items
were chosen from established measures. Following
Ceiling Effect minor revisions, items were added to the NIMH
structured interview and used in a large-scale study
(Brantley et al., 2000).
Follow-up studies support use of the CES-D with
diverse populations, including those with neurological
Center for Epidemiological compromise. Recent efforts have also focused on creating
StudiesDepression shorter versions of this measure (e.g., Iowa, Boston) to
facilitate use and decrease participant burden. Kohout
L ISA A. B RENNER and colleagues (1993) found that when assessing older
VISN 19 MIRECC adults (65 and older) shorter versions adequately evalu-
Denver, CO, USA ated the same symptom dimensions as the original mea-
sure. Findings from Carpenter et al. (1998), who pooled
data from 832 women (6 population), support use of the
Synonyms Iowa form over the Boston.
CES-D
Psychometric Data
Description Psychometric properties of the CES-D were initially
established with members of the general population
The Center for Epidemiological Studies Depression (household interview survey) and those receiving ser-
Scale (CES-D; Radloff, 1977) is a 20-item self-report vices in a psychiatric setting (Radloff, 1977). Validity
measure that assesses the presence and severity of depres- (content, construct, and criterion) was confirmed via
sive symptoms over the previous week. The CES-D has item choice and patterns of correlations with clinical
been used with adult and adolescents. Individuals com- ratings and alternate self-report measures. CES-D scores
pleting the Scale rate each item on a four-point Likert differentiated between psychiatric inpatients and mem-
scale ranging from 0 (rarely or none of the time) to 3 (most bers of the general population (Radloff, 1977): 70% of
or all of the time). Four items require reverse scoring. the patients versus 21% of the general population scored
Administration time is approximately 5 min. Responses above 16. Internal consistency, based on coefficient
are summed to obtain a total score of 060, with higher alpha and Spearman-Brown split-halves method, is gen-
scores indicating a greater frequency of symptoms. Scores erally high approximately 0.85 in the general population
of 16 or higher were suggested to identify subjects with and approximately 0.90 in the patient sample. Consistent
depressive illness (Radloff, 1977). Work by Pandya, Metz, with the expectation that shorter test-retest intervals
and Patten (2005) supports the use of this cut-off in those would produce higher correlations, test-retest reliabilities
with multiple sclerosis (MS). Earlier work by Zich, ranged from 0.32 (12 months) to 0.67 (4 weeks). Follow-
Attkisson, and Greenfield (1990) suggest that alternate up studies appear to support Radloff s (1977) original
508 C Center for Epidemiological StudiesDepression
Radloff, L. S. (1977). The CES-D Scale: A self-report depression scale for adults, and their families may require more than the
research in the general population. Applied Psychological Measure-
usual well child and/or adult, preventive care, and acute
ment, 1, 385401.
Rivera, P., Elliott, T. R., Berry, J. W., Grant, J. S., & Oswald, K. (2007).
illness interventions. It involves explicit changes in the
Predictors of caregiver depression among community-residing families roles of providers and office staff aimed at improving).
living with traumatic brain injury. Neurorehabilitation, 22(1), 38.
Zich, J. M., Attkisson, C. C., & Greenfield, T. K. (1990). Screening for
1. Access to needed services C
depression in primary care clinics: The CES-D and the BDI. Interna- 2. Communication with specialists, schools, and other
tional Journal of Psychiatry in Medicine, 20, 259277. resources
3. Outcomes for children and families
Landmark Contributions
Center for Medical Home
Improvement The story of the medical home extends from the 1935
Social Security Legislation Act that called for Maternal
W. C ARL C OOLEY, J EANNE W. M C A LLISTER and Child Health (MCH) Title V Programs to locate,
Crotched Mountain diagnose, and treat crippled children to todays emphasis
Concord, NH, USA on providing planned primary health care in the context
of the families and communities in which patients live.
Community-based care in a medical home is increasingly
Membership accepted as one of the means of achieving optimal out-
comes for children, youth, adults, and their families. The
The Center for Medical Home Improvement (CMHI) is a term medical home was coined in 1967 by the Council
nonprofit organization affiliated with Crotched Mountain. on Pediatric Practice, a subgroup of the American Acade-
(Crotched Mountain is a charitable organization employing my of Pediatrics, and has been described with a variety of
more than 900 people, whose mission is to serve individuals applications and definitions since then. The CMHI began
with disabilities and their families, embracing personal its efforts in 1993 with a capacity building endeavor for
choice and development, and building communities of pediatric practices serving children with special healthcare
mutual support. It provides specialized education, rehabili- needs. In 1997, the CMHI developed and implemented an
tation, community and residential support services for improvement model with multiple New Hampshire, Ver-
more than 2,000 individuals living in New England and mont, and Maine healthcare teams. These teams (physi-
New York.) It is founded in 1993 by Dr. Carl W. Cooley, cian leader and practice-based care coordinator)
Medical Director and Ms. Jeanne W. McAllister, R.N., M.S., partnered with patients and families to redesign primary
M.H.A., Director. Along with the founders, Dr. Cooley and care services. In 2003, the CMHI published the Medical
Ms. McAllister, the CMHI is staffed by Lora Council, M.D. Home Index (MHI) as a validated measurement to assess
(Quality Consultant), Lori Keehl-Markowitz, R.N., B.S.N. quality and monitor improvements in a primary care
(Program Manager), Leah Reed (Program Coordinator), practice (this tool is now available for adult care). Practice
and Kathleen Sherrieb, R.N., M.S., M.P.H. (Data Manage- improvement activities in multiple states have consistent-
ment Consultant). ly demonstrated sustained increases of greater than 30%
in MHI scores overall. A companion Medical Home Fam-
ily Index and Survey used by these practices has shown
Major Areas or Mission Statement significant improvement in clinical, functional, satisfac-
tion, and cost outcomes and an increase in the use of
The mission of the CMHI is to promote high-quality written and portable care plans. During 20012004, the
primary care in the medical home and secure health CMHI spread its model to ten additional practices in
policy changes critical to the future of primary care. The Vermont and New Hampshire and partnered with the
CMHI defines the medical home as a community-based National Initiative for Childrens Healthcare Quality
primary care setting which provides and coordinates high (NICHQ) in two national medical home learning collabora-
quality, planned, family-centered health promotion, acute tives. These efforts spread the CMHIs model to 20 state
illness care and chronic condition management (chronic Title V Programs, 40 primary care practices, and multiple
condition management acknowledges that children, family advocacy organizations. The CMHI and NICHQ
510 C Center for Medical Home Improvement
adapted the widely recognized Chronic Care Model as 2. Develop supports for primary care practices to im-
the Care Model for Child Health in a Medical Home. prove their medical homeness
A health resources and services administration 3. Align statewide efforts toward an investment in the
(HRSA) systems grant was awarded to the CMHI in future of primary care
2004 and a contract for a medical home outcomes re-
Following initial Council meetings, the New Hampshire
search study. Early findings from this research show that
Endowment for Health funded the CMHI to convene and
enhanced chronic condition management and care coor-
lead the New Hampshire Primary Care Task Force. This
dination, as outlined in the MHI, are associated with
group will issue consensus statements crafted to further
reductions in emergency room use, hospitalizations, and
detail the resources and supports necessary to develop and
visits to specialists. Starting in 2007, the CMHI Medical
provide relationship-centered primary care. The Task
Director, Dr. Carl Cooley began convening and chairing a
Force will comment on what they would like a future
Medical Home Work Group to design and develop a pilot
medical home pilot effort to look like in New Hampshire.
program to test a new process of primary care medical
The CMHI plans to develop and provide technical assis-
home-based, longitudinal care of children with genetic
tance to help primary care practices pass the National
conditions identified through newborn screening includ-
Committee for Quality Assurance (www.ncqa.org) medi-
ing the potential use of registries, planned care methods,
cal home recognition measure, a requirement to enter a
decision support mechanisms, comanagement with spe-
medical home pilot demonstration and receive enhanced
cialists, and application of family-and-patient-centered
payment. The CMHI is working with the New Hampshire
practices. This project is called the New England Genetics
Citizens Health Initiative and a public private multi-
Collaborative (NEGC) and is funding through the Uni-
payer and stakeholders group to craft New Hampshires
versity of New Hampshires Office of Sponsored Research.
pilot to stimulate a transformative process in primary care
A similar medical home project entitled Leadership Edu-
for the twenty-first century. In conclusion, the CMHI
cation in Autism Spectrum Disorders (LEASD) will begin
provides content expertise, education, and consultation
in the fall of 2008. The CMHI will work in partnership with
focused on medical home development to a variety of states,
Dartmouth Medical School (DMS) and the University of
centers, and initiatives including the American Academy of
New Hampshire Leadership in Education in Neurodeve-
Pediatrics, the commonwealth fund, various HRSA gran-
lopmental and Related Disabilities (LEND) program to
tees, and numerous state and regional programs.
assure that all children in New Hampshire are screened
for autism and spectrum disorders at 18 and 24 months to
assure early diagnosis and access to early intervention and
Major Activities
family-centered care for optimal development outcomes.
The providence for the medical home model has gained
2001: Medical Home Index (MHI), pediatric version, a
substantial momentum that in 2007, the American Acade-
validated measurement to assess quality and monitor
my of Pediatrics, American Academy of Family Physicians,
improvements in a primary care practice (available at
and the American College of Physicians as well as Family
www.medicalhomeimprovement.org). 2001: Developed
Voices and the National Association of Pediatric Nurse
a companion Medical Home Family Index and Survey
Practitioners endorsed the medical home as the model
(available at www.medicalhomeimprovement.org). 2001:
for twenty-first-century primary care (www.pcpcc.org).
Medical Home Improvement Tool Kit (available at www.
States are looking for better ways to effectively support
medicalhomeimprovement.org). 2003: Parent Partners
the healthcare needs of its citizens and the organizational
Creative Forces on Medical Home Improvement Teams
and operational needs of those providing health care.
(available at www.medicalhomeimprovement.org).
However, there is a looming crisis in primary care that
2006current: New Hampshire Council on the Future of
raises concerns about current and future capacity, recruit-
Primary Care Medical Home. 2007current: New Hamp-
ment, and reimbursement. In response to the current
shire Primary Care Task Force 2007: Medical Home Prac-
struggle inherent to primary care, the CMHI, in partner-
tice-Based Care Coordination, Workbook (available at
ship with New Hampshire Special Medical Services regu-
www.medicalhomeimprovement.org). 20072008: New
larly convenes the New Hampshire Council on the Future
England Genetics Collaborative (NEGC), Regional Genet-
of the Primary Care Medical Home with explicit goals to:
ics and Newborn Screening Collaborative. 2008: Leader-
1. Build and spread awareness of the medical home ship Education in Autism Spectrum Disorders (LEASD).
model of primary care 2008: Extra-Ordinary Care: Improving Your Medical
Center for Outcome Measurement in Brain Injury (COMBI) C 511
Home, Guide and Workbook (available at www.medical- measures for persons with brain injury. The measures
homeimprovement.org). included in the COMBI are commonly used in the field
2008: Medical Home Index (MHI), Adult version of brain injury rehabilitation and assessment. The
(available at www.medicalhomeimprovement.org). COMBI is a collaborative project of 16 brain injury
facilities or centers, with each center contributing infor- C
mation on one or more measures. For most supported
References and Readings
measures, there are syllabus and training information,
rating forms, background information on validity and
Cooley, W. C., McAllister, J. W., Sherrieb, K., & Clark, R. (2003).
The medical home index: Development and validation of a new
reliability, a reference list of published studies, and test-
practice-level measure of implementation of the medical home ing materials. A Frequently Asked Question (FAQ) sec-
model. Ambulatory Pediatrics, 3, 173180. tion is also included, compiled from past training
Cooley, W. C., & McAllister, J. W. (2004). Building medical homes:
Improvement strategies in primary care for children with special
health care needs. Pediatrics, 113, 14991506.
Dillon, A., & McAllister, J. W. (2006). Do you have a medical home? EP Center for Outcome Measurement in Brain Injury (COMBI).
Magazine (www.eparent.com), August, 2228.
Table 1 COMBI-featured scales/measures
Joint Principles of the Patient-Centered Medical Home (2007). http://
www.aafp.org/online/etc/medialib/aafp_org/documents/policy/fed/ Agitated Behavior Scale (ABS)
jointprinciplespcmh0207.Par.0001.File.dat/022107medicalhome.pdf
Apathy Evaluation Scale (AES)
McAllister, J. W., Pressler, E., & Cooley, W. C. (2007). Practice-based care
coordination: A medical home essential. Pediatrics, 120, e723e733. Awareness Questionnaire (AQ)
doi: 10.1542/peds.2006-1684. Cognitive Log (Cog-Log)
Takachand, M., Kaye, N., & Beesla, R. (2008). Strategies states can use to
Coma/Near Coma Scale (CNC)
support the infrastructure of medical homes. State Health Policy
Briefing. www.nashp.org/Files/shpbriefing_pcmhsupport.pdf. Coma Recovery Scale-Revised(CRS-R)
Confusion Assessment Protocol (CAP)
Community Integration Questionnaire (CIQ)
The Craig Handicap Assessment and Reporting Technique
Center for Outcome Measurement (CHART)
The Craig Handicap Assessment and Reporting Technique
in Brain Injury (COMBI) Short Form (CHART SF)
The Craig Hospital Inventory of Environmental Factors
J ERRY W RIGHT
(CHIEF)
Santa Clara Valley Medical Center
Disability Rating Scale (DRS)
San Jose, CA, USA
The Family Needs Questionnaire (FNQ)
Functional Assessment Measure (FAM)
Membership Functional Independence Measure (FIM)(TM)
Glasgow Outcome Scale (GOS)
The Center for Outcome Measurement in Brain Injury Extended Glasgow Outcome Scale (GOS-E)
(COMBI) is a collaborative project of 16 brain injury High Level Mobility Assessment Tool (HiMAT)
facilities or centers, most of them Traumatic Brain
Level of Cognitive Functioning Scale (LCFS)
Injury Model Systems (funded by the National Institute
Mayo Portland Adaptability Inventory (MPAI)
on Disability and Rehabilitation Research). The product is
an informational web site that is oriented primarily to- Mississippi Aphasia Screening Test (MAST)
wards clinicians and researchers, but is also freely avail- Neurobehavioral Functioning Inventory (NFI)
able to the general public. The Orientation Log (O-Log)
The Patient Competency Rating Scale (PCRS)
Participation Objective, Participation Subjective
Major Areas Satisfaction With Life Scale (SWLS)
Service Obstacle Scale (SOS)
The COMBI is an online resource for those needing
Supervision Rating Scale (SRS)
detailed information and support in regards to outcome
512 C Centers for Medicare and Medicaid Service
Major Activities
Major Areas
Currently, the COMBI has 28 featured scales, with 25
scales available for immediate download. Table 1 shows CMS is part of the Department of Health and Human
the COMBI-featured scales as of August 2008. Additional Services. The primary offices are not located in Washing-
information is available about measuring employment ton D.C. but in Baltimore. With a budget of approximate-
and substance abuse after brain injury. The COMBI web ly $650 billion, serving almost 90 million beneficiaries,
site also conducts testing and certification for the Disabil- CMS has a dominant role in American health care. The
ity Rating Scale (DRS). The COMBI web site (www.tbims. top position in CMS, the Administrator, is nominated by
org/combi) receives over 1,200 visitors per day, with well the President of the USA but must be confirmed by the
over one million visits in total. Senate. Most administrators have come to CMS from
academia or have been industry lobbyists. The position
of Administrator is complex and demanding. Nancy-Ann
DePearle, who served as Administrator at the end of the
Cross References Clinton administration, during one of the few periods in
which federal spending for CMS declined, noted that
Agitated Behavior Scale (ABS) the Administrator has . . .many bosses, including 535
Coma/Near Coma Scale (CNS) members of Congress, the White House, the Inspector
Coma Recovery Scale (CRS) General, the US Government Accountability Office
National Institute on Disability and Rehabilitation (GAO) (Health Affairs: W-5337).
Research (NIDRR) The Department of Health and Human Services,
Neurobehavioral Functioning Inventory (NFI) through CMS, issues regulations and policy clarifications
Orientation Log guiding the provision of services for Medicare benefici-
Rancho Los Amigos Scale aries. All psychologists who provide health-care services
Traumatic Brain Injury to Medicare recipients, particularly neuropsychologists,
Traumatic Brain Injury Model System interact with CMS. Each psychologist who provides ser-
vices to Medicare beneficiaries must obtain National Pro-
vider Identification (NPI) from CMS. Medicare fiscal
intermediaries oversee payment for Medicare services
References and Readings including the electronic fund transfer. Each state has a
fiscal intermediary overseeing Medicare Part A (inpatient
http://www.tbims.org/combi
services) and Part B (professional services). CMS regula-
Wright, J. M., Bushnik, T., & OHare, P. (2000). The center for outcome
measurement in brain injury (COMBI): An internet resource you
tions now guide the care of all Medicare beneficiaries. In
should know about. Journal of Head Trauma Rehabilitation, 15(8), addition, the size and impact of Medicare have made the
734738. agency a predictor of many coverage choices by private
Centigray C 513
insurers. When CMS issues proposed rules in the Federal Medicare program (health insurance for people aged 65
Register (the federal governments daily journal), all and over, younger people receiving social security disabil-
providers have the opportunity to comment. Final rules ity benefits, and persons with end-stage kidney disease)
that guide reimbursement by CMSs fiscal intermediaries as well as the Medicaid program (providing medical
are then issued. assistance from state and federal governments for eligible C
low-income persons). Although the federal government
regulates Medicaid, state governments actually operate
Landmark Contributions the program). HCFA also provided federal oversight for
quality control initiatives.
In July 1965, at the home of President Harry Truman, Between 1989 and 2000, the number of Medicare
President Lyndon B. Johnson signed the Social Security claims rose from 70% to more than 800 million claims.
Act of 1965. Titles XVIII and XIX of the Act fostered a new Despite this staggering volume, Medicares administrative
view of health care in the USA, responding to more than costs were only 2%. HCFA was viewed as having a bias
20 years of legislative initiatives aimed at creating an toward providers. In 2001, the Bush administration,
insurance program to cover hospitalization, health ser- believing that HCFA was perceived as bureaucratic, unre-
vices, and skilled nursing care for the elderly as well as sponsive, and biased toward physicians and hospitals,
coverage for low-income children. When implemented in sought a new image for the agency. In an effort to improve
1966, Medicare (created by Title XVIII) provided cover- the agencys image, the Bush administration changed the
age for more than 19 million elderly Americans. Medicaid name to the Centers for Medicare and Medicaid CMS
(created in Title XIX), covered almost 15 million people (Scully, 2005), heralding an effort to streamline the many
and was administered by the Social Rehabilitation administrative processes overseen by CMS. Despite the
Administration, an agency managing poverty and welfare new name, CMS continued the responsibilities of HCFA:
programs. Medicare, end-stage renal disease, quality, and coordina-
The inclusion of Medicare and Medicaid within the tion of Medicaid with the states.
Social Security Act reflected political compromises need-
ed for the passage of both programs. Wage and price
controls imposed during World War II prompted many
References and Readings
employers to offer health-care coverage. This de facto
DePearle, N. A. (2005). What does it take to run Medicare and Medicaid?
wage increase determined that health insurance became a Health Affairs, W-5337338.
common employer benefit, but one that excluded the Derzon, R. A. (2005). The Genesis fo HCFA. Health Affairs, W5326.
elderly and unemployed. By 1960, the inadequacy of Scully, T. A. (2005). Policy high oints: Medicare and medicaid in the New
health services for older Americans had become a political Millennium. Health Affairs, W5339.
issue. In order to secure enough votes to pass Medicare,
President Johnson created the concept of the medically
indigent thereby linking welfare to health care for the
elderly. Centigray
Medicare and Medicaid were administered separately
during the Johnson and Nixon administrations. Joseph B RAM G OLDSTEIN
Califano, Secretary of Health Education and Welfare Hoag Hospital Cancer Center
(HEW) under Jimmy Carter, believed that ineffective Newport Beach, CA, USA
oversight of health programs permitted spiraling health
costs and health-care spending inflation (Derzon, 2005).
Califano knew the separate administration of Medicare Synonyms
and Medicaid reflected the political compromises needed
to create coverage for the elderly and the poor through cGY
linking coverage for the elderly to welfare. The split ad-
ministration of Medicare and Medicaid, however, made Definition
the two mammoth programs less manageable. Califano
moved quickly, in secret and combined Medicare and Centigray (cGY) is the preferred measurement of
Medicaid, forming the Health Care Financing Admin- absorbed radiation and is equivalent to one-hundredth
istration (HCFA). HCFA provided oversight to the (10-2) of a gray, or 1 rad. The gray measures the deposited
514 C Central Auditory Processing Disorder
energy of radiation. The daily dose of radiation is also symptoms diagnosed by audiologists and/or speech and
referred to as a fraction, since each dose is a percentage of language specialists using the audiology code of ICD-9
the cumulative prescribed dose. The dose is given in code 388.4 (Abnormal Auditory Processes).
sublethal fractions, which protracts the dose to facilitate The American Speech-Language Hearing Association
the occurrence of repair kinetics. Hence, radiation oncol- (ASHA, 2005) defines CAPD as difficulties in the per-
ogists use fractions to take advantage of the differential ceptual processing of auditory information in the central
recovery rates for normal and neoplastic tissue, thereby nervous system (CNS). It presents in the form of poor
permitting repopulation of normal cells and inducing performance in one or more of the following abilities
radiosensitivity via increased oxygen to the remaining or skills: sound localization and lateralization, auditory
tumor cells. Fractionated dose radiotherapy enhances discrimination, auditory pattern recognition, temporal
the treatment efficacy by targeting the cancer cells aspects of audition (including temporal integration,
while mitigating the damage to healthy tissue. This is temporal discrimination/gap detection, temporal order-
determined using a therapeutic ratio, which compares ing, and temporal masking), auditory performance in
the damage to both cancerous and healthy cells (Potters, competing acoustic signals (including dichotic listen-
Timmerman, & Larson, 2005). Moreover, the biological ing), and auditory performance with degraded acoustic
effects on the relevant tissue vary according to the signals.
radiation type and intensity.
Categorization
Cross References
Neither CAPD nor SI is listed in the DSM-IVor DSM-IV-TR
Radiation Therapy manuals which provide diagnostic codes for psychologists,
psychiatrists, and speech and language specialists. When
References and Readings individuals from these fields assess problems that are
language based, they use language tests and assign diag-
Potters, L., Timmerman, R., & Larson, D. (2005). Stereotactic body
nostic codes associated with specific language disorders
radiation therapy. Journal of the American College of Radiology, 2, (e.g., ICD-9: 315.3, Developmental Speech or Language
676680. Disorder; ICD-9: 389.9, Unspecified Hearing Loss).
While the diagnosis of CAPD has a 50-year history,
there is much controversy about its legitimacy (Carneol,
2008). In part, this stems from its extensive comorbidity
Central Auditory Processing with a variety of impairments in attention/concentration,
Disorder language, memory, IQ, academic achievement, and other
behavioral/emotional disorders. Children with the CAPD
J UDITH A. S HECHTER 1, S ARAH J. L EINEN 2 diagnosis typically demonstrate poor listening skills,
1
Wynnewood, PA, USA difficulty filtering linguistic information from back-
2
Widener University ground noise, problems following oral instructions, poor
Chester, PA, USA auditory discrimination skills, distractibility/inattentive-
ness, and often need additional time to complete tasks
(Bloom & Hynd, 2008). In addition, there is no compel-
Synonyms ling research that reliably provides specific neurobiologi-
cal bases for CAPD when compared to findings that
Auditory perceptual disorder (APD); Auditory processing link specific brain regions with language-based learning
disorder (APD); CAPD; Sensory integration-C/APD disabilities and Attention Deficit Hyperactivity Disorder
(SI-C/APD) (Bloom & Hynd, 2008). Together, these factors have
called into question the validity of CAPD as a distinct
diagnosis.
Short Description or Definition It is important to understand the complicated neuro-
physiological constructs of hearing to better understand
Central Auditory Processing Disorder (CAPD) is not a the etiological basis of CAPD. That is, one must know
neuropsychological diagnosis. It refers to an amalgam of how sound travels from the outer ear through the middle
Central Auditory Processing Disorder C 515
and inner ear structures through the eighth auditory contribute to outcome trajectories (e.g., comorbid
nerve to Heschls gyrus in the left temporal lobe of the ADHD, LD) (Bloom & Hynd, 2008).
brain. At that point, the brain begins to recognize nerve
impulses as sound and then initiates interpretation of
these impulses as either speech or nonverbal auditory C
stimuli. These stimuli are then analyzed bilaterally by Neuropsychology and Psychology
auditory stimulus networks distributed within the brain of CAPD
(Carneol, 2008).
Some researchers have proposed CAPD subgroups The immense overlap of CAPD symptoms with other de-
such as Auditory Decoding Deficit (primary left auditory velopmental disorders such as ADHD continues to fuel
cortex dysfunction), Prosodic Deficit (nonprimary right debate among psychologists, neuropsychologists, audiolo-
auditory cortex and associated areas dysfunction), and gists, and speech-language pathologists over the existence of
Integration Deficit (corpus callosum dysfunction) (Bellis, this diagnosis. While a definition of CAPD as a unimodal
2003; Bellis & Ferre, 1999; Katz, 1992). These subtypes disorder is a useful tool in the conceptualization of the
may help clinicians design intervention strategies specific symptoms associated with it, the notion of CAPD as sepa-
to an individuals presenting problems However, these rate and distinct from language-based LD and ADHD has
models are theoretical in nature and not universally not been well documented in the literature.
agreed upon (ASHA, 2005; Jutras et al., 2007). In a sample of 30 913-year-olds, approximately 50%
of children diagnosed with CAPD also met diagnostic
criteria for ADHD (Riccio, Hynd, Cohen, Hall, & Molt,
1994). Based on ratings from audiologists and pediatri-
Epidemiology
cians, Chermak, Tucker, and Seikel (2002) identified
exclusive behavior sets characterizing CAPD and ADHD.
No truly authoritative population or prevalence studies
CAPD was not characterized by hyperactivity and
regarding CAPD are available (Castrogiovanni, 2008).
difficulties with impulse control.
Chermak and Musiek (1997) estimate a prevalence rate
Some individuals with CAPD may have difficulties
of 23% in children, with the disorder occurring twice as
with speech and language. Varying combinations of audi-
frequently in males than females. Cooper and Gates
tory processing deficits may be associated with different
(1991) estimate that the prevalence in older adults is
functional deficits in speech and language. Differential
1020%.
diagnosis between CAPD and language disorders can be
quite challenging due to shared symptomatology. For
example, a core deficit in phonological processing under-
Natural History, Prognostic Factors, lies dyslexia. Similarly, children with CAPD may have
and Outcomes difficulty discriminating and manipulating the phonetic
aspects of auditory input. It has been suggested that
The etiology of CAPD is unknown. CAPD may present as auditory processing problems in developmental dyslexia
a developmental or acquired disorder. The maturation of are specific to the encoding of speech, whereas CAPD is
the central auditory pathway may be delayed (Bamiou, characterized by a general dysfunction encoding all
Musiek, & Luxon, 2001). Prenatal or perinatal factors auditory stimuli.
(e.g., hyperbilirubenemia, anoxia or hypoxia, ototoxic Academic difficulties are often a characteristic of
drugs, Rh incompatibility, prematurity/low birth weight, children who carry a CAPD diagnosis (ASHA, 2005).
birth complications, maternal diabetes, or infections), ear They may have a difficult time encoding/learning spoken
infections, heavy metal exposure, cerebrovascular disor- information or information presented with background
ders, epilepsy, Lyme disease, and traumatic brain injury noise. They are more likely to have behavioral, emotional,
may be risk factors for the constellation of symptoms that and social difficulties secondary to poor communica-
characterize the CAPD diagnosis (Bamiou, Musiek, & tion skills (ASHA, 2005). The preceding, combined
Luxon, 2001; Musiek & Chermak, 2009; Riccio & with associated learning difficulties, can compromise
Hynd, 1996). self-esteem and contribute to emotional withdrawal, so-
It is not clear if problems in auditory processing matization, conduct disorders, depression, anxiety, and
resolve with age, if children are able to compensate for interpersonal problems (Riccio, Cohen, Garrison, &
auditory processing deficits, and what factors may Smith, 2005).
516 C Central Auditory Processing Disorder
Cooper, J., & Gates, G. (1991). Hearing in the elderly the Framingham several types of memory in the intact human mind.
cohort, 19831985: Part II. Prevalence of central auditory processing
Although philosophers have long been theorizing about
disorders. Ear and Hearing, 12, 304311.
Jutras, B., Loubert, M., Dupuis, J., Marcoux, C., Dumont, V., & Baril, M.
distinctions within memory, there has been experimental
(2007). Applicability of central auditory processing disorder models. evidence supporting the divisions of memory for only the
American Journal of Audiology, 16, 100106. past 3040 years (Baddeley, 1999). C
Katz, J. (1992). Classification of auditory processing disorders. In J. Katz,
N. Stecker, & D. Henderson (Eds.), Central auditory processing:
A transdisciplinary view (pp. 8191). Baltimore: Mosby-Yearbook.
Musiek, F., & Chermak, G. (2009). Diagnosis of (central) auditory
Historical Background
processing disorder in traumatic brain injury: Psychophysical
and electrophysiological approaches. Washington, DC: The ASHA
Leader. Retrieved from http://www.asha.org/publications/leader/ Baddeley and Hitch (1974) developed a theory of a WM
2009/091124/CAPD.htm. system that has become the dominant theory in cognitive
Riccio, C., Cohen, M., Garrison, T., & Smith, B. (2005). Auditory pro- psychology for the organization of WM (Fig. 1). Baddeleys
cessing measures: correlation with neuropsychological measures of
model addressed weaknesses of previous short-term
attention, memory, and behavior. Child Neuropsychology, 11,
363372.
memory (STM) models, including the failure to address
Riccio, C., & Hynd, G. (1996). Relationship between ADHD and central nonverbal processing. Baddeleys model, which has
auditory processing disorder: A review of the literature. School undergone changes throughout the years, is a theoretical
Psychology International, 17, 235252. concept and was not originally developed to directly cor-
Riccio, C., Hynd, G., Cohen, M., Hall, J., & Molt, L. (1994). Comorbidity
respond with specific neuroanatomical regions, though
of central auditory processing disorder and attention-deficit hyper-
activity disorder. Journal of the American Academy of Child and
research that attempts to do so has been completed. The
Adolescent Psychiatry, 33, 849847. original theory proposed the existence of three compo-
nents (Fig. 1). Baddeley envisioned this system as consist-
ing of a central executive, which is a limited capacity
subsection that controls certain subsystems. The central
executive controls slave systems, which are mainly used
Central Executive for the temporary storage of information. Information
that is not needed right now, but will soon be needed,
N ICOLE C. R. M C L AUGHLIN can be stored within these slave systems to free up the
Butler Hospital, central executive for other tasks. One of the slave systems
Alpert Medical School of Brown University is the articulatory or phonological loop, which is a verbal
Providence, RI, USA store. Subvocalization of verbal material creates a record
in a phonological buffer. The central executive then reads
the phonological buffer, and the cycle begins again. For
Definition most of this cycle, the central executive is not needed.
Another slave system is the visuospatial sketchpad, which
Working memory (WM) has been defined as the is used to rehearse visual or spatial materials. Manipulation
blackboard of the mind and the mental sketchpad of information within either the phonological loop or vi-
(Baddeley, 1986). It enables the online holding and mental suospatial sketchpad does not affect information being
manipulation of information. Human beings use WM retained in the other system; they have been shown to be
processes all the time. For example, WM is used to distinct systems in regard to interference effects (Brooks,
perform rapid mathematical functions in our heads and 1968). Another component, the episodic buffer
to understand the inherent meaning in speech and (described in detail below), was added in 2000 to address
writing. WM is also important for reasoning and problem some of the perceived weaknesses of the earlier model.
solving (Baddeley, 1999). Psychologists have posited The current multicomponent model describes fluid
Central Executive. Figure 1 Initial three-component model of working memory (WM) (Baddeley & Hitch, 1974)
518 C Central Executive
Central executive
Central Executive. Figure 2 Multicomponent model of working memory (WM) (Baddeley, 2000)
capabilities (such as temporary storage) and crystallized are prevented from rehearsing items by being required to
abilities that are involved in long-term knowledge (Fig. 2). state an irrelevant word (such as the. . .the. . .the), perfor-
Phonological Loop. The phonological loop is most mance declines. There also appears to be a word-length
similar to the earlier concept of STM (Baddeley, 1992) effect such that longer words are more difficult to remem-
and is the most studied component within this theory. ber, potentially because participants are subvocalizing
There are two components within the phonological loop: words, and thus, there is more time for information to
a phonological store and an articulatory rehearsal system. deteriorate (Baddeley, 2000).
The phonological store can temporarily hold acoustic People frequently use the phonological store in every-
material for 12 s and is involved in speech perception. day life, subvocalizing when counting and when reading.
The articulatory rehearsal system can maintain material Adult fluent readers use this component less than poor
by subvocal repetition and can take visually presented readers, or individuals learning to read, but show diffi-
material and register this material by subvocalization. culties picking up errors in written text when their sub-
There are several pieces of evidence that have supported vocalization capabilities are disrupted (Baddeley, 1999).
the presence of the phonological loop. The simplest piece The phonological store may also be important for new
of evidence is that it appears clear that our verbal store language acquisition as well as native language acquisition
holds only a limited amount of information. The phono- (Baddeley, 1999); some researchers have indicated that the
logical similarity effect indicates that sequences of items phonological loops primary purpose is for language
with similar phonological sounds are more difficult to acquisition (Baddeley, Gathercole, & Papagno, 1998).
remember than those with disparate sounds (e.g., mad A deficit in phonological STM appears to stem from a
can cap man is more difficult than pen day cow bar rig) defective phonological store. Articulatory rehearsal appears
(Baddeley, 1966b). In addition, it demonstrates the ten- to be defective in aphasic patients with dyspraxia, as they
dency for participant errors to be phonologically similar are unable to carry out the speech motor codes needed for
to the correct item (i.e., F for S and B for G). Similarity of articulation (Waters, Rochon, & Caplan, 1992). Dysarthric
meaning does not seem to be important for the phono- patients do not appear to have a deficit in articulatory
logical store (Baddeley, 1966b), whereas long-term stor- rehearsal, likely because their deficits are peripheral, not
age is affected by similarity of meaning, but not sound central (Baddeley & Wilson, 1985). Neuroanatomically,
(Baddeley, 1966a). The irrelevant speech effect shows that based upon lesion studies, the inferior parietal cortex
exposure to irrelevant speech either at the same time or appears to be related to the temporary phonological store,
directly after stimuli material can disrupt immediate re- whereas the articulatory rehearsal system uses brain areas
call of the stimulus; meaningless noise does not disrupt necessary for speech production, such as Brocas area and/
stimuli material. This effect is the same for phonologically or the supplementary motor area (Muller & Knight, 2006).
similar or dissimilar items (Salame & Baddeley, 1987). Visuospatial Sketchpad. Similar to the function of the
Preventing rehearsal (articulatory suppression) further phonological loop for verbal material, the visuospatial
decreases performance (Baddeley, 1999); when participants sketchpad allows for the maintenance of temporary
Central Executive C 519
representations of visuospatial information. The visuo- override the CS when the CS fails or no known schema
spatial sketchpad is involved in such tasks as visual imag- exists (Shallice, 1982).
ery and mental rotation. Evidence for this system comes Additional attentional processes that have been ascribed
from the finding that visuospatial immediate memory can to the central executive include focusing, dividing, and
be disrupted by visual tracking, but not by verbal coding switching attention, as well as serving as the interface be- C
(Baddeley, 1999). There seems to be separate subsystems tween the subsystems described above and long-term mem-
involved in the maintenance of visual information (shape, ory (Baddeley, 2001). With regard to focusing attention,
color) and spatial information, independent of the central participants in a chess game were impaired by interference
executive (Klauer & Zhao, 2004). In addition, there also from a random digit generation task; this was thought to
appears to be a dissociation of this system into two com- place a heavy load on the central executive (Robbins et al.,
ponents, a passive store that maintains information, and 1996). With regard to dividing attention, participants
a more active device that manipulates it (Bruyer & with Alzheimers disease who have long-term memory and
Scailquin, 1998). attentional deficits are impaired on dual-task paradigms,
The visuospatial sketchpad appears to be principally yet are able to do the same tasks individually (MacPherson
represented within the right hemisphere (Baddeley, 2000). et al., 2007). There is a question as to whether switching is
Within this component, as noted above, past research has primarily an executive process, though there also appears
indicated that there is a neuroanatomical differentiation to be a strong relationship between the phonological loop
between object and spatial information. Using evidence and switching.
from the parietal and temporal visual streams, it is likely Research has indicated that the prefrontal cortex,
that the parietal lobe (dorsal stream) processes spatial especially the dorsolateral prefrontal region, is critical
information (i.e., where the relation of objects across for executive functioning (Collette et al., 1999; DEsposito
coordinates) while the temporal lobe (ventral stream) et al., 1995; Owen, Evans, & Petrides, 1996; Salmon et al.,
processes nonspatial (i.e., what as in a visual image or 1996). However, more recent studies have indicated that,
object information); this subdivision also possibly although the PFC appears essential for executive function-
extends into dorsal and ventral regions of the prefrontal ing, other brain regions may also be important for these
cortex, and these regions are most relevant for short-term, functions (Baddeley & Wilson, 1988; Baker et al., 1996;
or working memory processes (Goldman-Rakic & Leung, Berman et al., 1995; Nagahama et al., 1996).
2002). Episodic Buffer. As mentioned above, the episodic buff-
Central Executive. The central executive has been er was developed in response to a need for a component to
described as a homunculus, or little man who makes relate between working and long-term memory (Baddeley,
decisions as to how the slave systems should be used. It is a 2000). The episodic buffer is envisioned as a temporary
limited capacity attentional system that is in charge of the storage system controlled by the central executive, as well
phonological loop and visuospatial sketchpad (Baddeley, as an interface between multiple systems. The central
1999). Although the central executive may be considered executive can retrieve information from the store, and
the most important component of this model, given the potentially manipulate or modify it. There were multiple
apparent complexity of this system, it is difficult to investi- weaknesses to the initial three-component theory that this
gate. Baddeley (1986) coined the term dysexecutive syn- multicomponent model has attempted to address. For
drome (DES) to describe dysfunctions of the central example, with articulatory suppression (interference of
executive. encoding of numbers) of the phonological loop, short-
Baddeley has compared the central executive to Norman term span becomes shorter, but does not completely dis-
and Shallices ideas of the supervisory attentional system appear (Baddeley, 1984). In addition, participants can
(SAS; Shallice, 1982), which is used in planning, decision- remember more in sentences or in paragraphs than
making, novel situations, and difficult situations. Cont- would be anticipated solely based upon the phonological
ention scheduling (CS) is a process that chooses one store. There appears to be a distinction between immedi-
response and inhibits another, in a crude and fast way. ate recall and delayed prose recall, with disparate subsys-
CS modulates the selection of an action schema when it is tems implicated in each (Wilson & Baddeley, 1988). The
routine, or unconscious, selecting the schema with the episodic buffer appears to be preserved in patients with
strongest triggers. The SAS is a higher level system that impaired LTM (Baddeley, 2000), and plays an important
is slow and flexible. It is utilized when a selection is role in sending information to and retrieving information
more complex, and adjusts the activation level of the from LTM. Although there is minimal research specifically
action schemas. Therefore, the SAS can actually examining the neuroanatomical basis of this component,
520 C Central Executive
it appears likely that the frontal lobes are involved in Berman, K. F., Ostrem, J. L., Randolph, C., Gold, J., Goldberg, T. E.,
Coppola, R., et al. (1995). Physiological activation of a cortical
these processes. Specifically, there appears to be greater
network during performance of the Wisconsin card sorting test: A
right frontal activation for integration of information positron emission tomography study. Neuropsychologia, 33(8),
(Prabhakaran, Narayanan, Zhao, & Gabrieli, 2000). 10271046.
Baddeleys theory provided a framework for the Brooks, L. R. (1968). Spatial and verbal components of the act of recall.
initial study of working memory processes, addressing Canadian Journal of Psychology, 22, 349368.
Bruyer, R., & Scailquin, J. C. (1998). The visuospatial sketchpad for
weaknesses of earlier models of STM that did not suffi-
mental images: Testing the multicomponent model of working
ciently account for many important pieces of information. memory. Acta Psychologica (Amsterdam), 98(1), 1736.
Although there have been additions to the model since its Collette, F., Salmon, E., Van der Linden, M., Chicherio, C., Belleville, S.,
initial conception, Baddeleys ideas continue to be relevant Degueldre, C., et al. (1999). Regional brain activity during tasks
today, and this work has informed, and continues to in- devoted to the central executive of working memory. Cognitive
Brain Research, 7(3), 411417.
form, multiple avenues of research within the field.
DEsposito, M., Detre, J. A., Alsop, D. C., Shin, R. K., Atlas, S., &
Grossman, M. (1995). The neural basis of the central executive
system of working memory. Nature, 378(6554), 279281.
Cross References Goldman-Rakic, P. S., & Leung, H. (2002). Functional architecture of the
dorsolateral prefrontal cortex in monkeys and humans. In D. T. S. R.
T. Knight (Ed.), Principles of frontal lobe function (pp. 8595). New
Attention/Executive Functions
York: Oxford University Press.
Controlled Attention Klauer, K. C., & Zhao, Z. (2004). Double dissociations in visual and
Dysexecutive Syndrome spatial short-term memory. Journal of Experimental Psychology: Gen-
Working Memory eral, 133(3), 355381.
MacPherson, S. E., Della Sala, S., Logie, R. H., & Wilcock, G. K. (2007).
Specific AD impairment in concurrent performance of two memory
tasks. Cortex, 43(7), 858865.
References and Readings Muller, N. G., & Knight, R. T. (2006). The functional neuroanatomy of
working memory: Contributions of human brain lesion studies.
Baddeley, A. D. (1966a). The influence of acoustic and semantic similarity Neuroscience, 139(1), 5158.
on long-term memory for word sequences. Quarterly Journal of Nagahama, Y., Fukuyama, H., Yamauchi, H., Matsuzaki, S., Konishi, J.,
Experimental Psychology, 18(4), 302309. Shibasaki, H., et al. (1996). Cerebral activation during performance
Baddeley, A. D. (1966b). Short-term memory for word sequences as a of a card sorting test. Brain, 119(Pt 5), 16671675.
function of acoustic, semantic and formal similarity. Quarterly Jour- Owen, A. M., Evans, A. C., & Petrides, M. (1996). Evidence for a two-
nal of Experimental Psychology, 18(4), 362365. stage model of spatial working memory processing within the lateral
Baddeley, A. D. (1984). Exploring the articulatory loop. Quarterly Journal frontal cortex: A positron emission tomography study. Cerebral
of Experimental Psychology, 36, 233252. Cortex, 6(1), 3138.
Baddeley, A. (1986). Working memory. Oxford: Clarendon. Prabhakaran, V., Narayanan, K., Zhao, Z., & Gabrieli, J. D. (2000).
Baddeley, A. (1992). Working memory. Science, 255(5044), 556559. Integration of diverse information in working memory within the
Baddeley, A. D. (1999). Essentials of human memory. East Sussex: frontal lobe. Nature Neuroscience, 3(1), 8590.
Psychology Press. Repovs, G., & Baddeley, A. (2006). The multi-component model
Baddeley, A. (2000). The episodic buffer: A new component of working of working memory: Explorations in experimental cognitive psy-
memory? Trends in Cognitive Science, 4(11), 417423. chology. Neuroscience, 139(1), 521.
Baddeley, A. D. (2001). Is working memory still working? American Robbins, T. W., Anderson, E. J., Barker, D. R., Bradley, A. C., Fearny-
Psychologist, 56(11), 851864. hough, C., Henson, R., et al. (1996). Working memory in chess.
Baddeley, A. D., & Hitch, G. (1974). Working memory. In G. A. Bower Memory & Cognition, 24(1), 8393.
(Ed.), Recent advances in learning and motivation. New York: Salame, P., & Baddeley, A. (1987). Noise, unattended speech and short-
Academic. term memory. Ergonomics, 30(8), 11851194.
Baddeley, A. D., & Wilson, B. (1985). Phonological coding and short- Salmon, E., Van der Linden, M., Collette, F., Delfiore, G., Maquet, P.,
term memory in patients without speech. Journal of Memory and Degueldre, C., et al. (1996). Regional brain activity during working
Language, 24, 490502. memory tasks. Brain, 119(Pt 5), 16171625.
Baddeley, A., & Wilson, B. (1988). Frontal amnesia and the dysexecutive Shallice, T. (1982). Specific impairments of planning. Philosophical Trans-
syndrome. Brain and Cognition, 7(2), 212230. actions of the Royal Society of London. Series B, Biological Sciences, 298
Baddeley, A., Gathercole, S., & Papagno, C. (1998). The phonological (1089), 199209.
loop as a language learning device. Psychological Review, 105(1), Waters, G. F., Rochon, E., & Caplan, D. (1992). The role of high-level
158173. speech planning in rehearsal: Evidence from patients with apraxia of
Baker, S. C., Rogers, R. D., Owen, A. M., Frith, C. D., Dolan, R. J., speech. Journal of Memory and Language, 31, 5473.
Frackowiak, R. S., et al. (1996). Neural systems engaged by planning: Wilson, B., & Baddeley, A. (1988). Semantic, episodic, and autobiograph-
A pet study of the tower of London task. Neuropsychologia, 34(6), ical memory in a postmeningitic amnesic patient. Brain and Cogni-
515526. tion, 8(1), 3146.
Cerebellar Cognitive Affective Syndrome C 521
Ferro, J. M., Canhao, P., Bousser, M. G., Stam, J., & Barinagarrementeria,
Definition F. (2005). Cerebral vein and dural sinus thrombosis in elderly
patients. Stroke, 36, 19271932.
Central venous thrombosis is a rare form of stroke Stam, J. (2005). Thrombosis of the cerebral veins and sinuses.
that results from thrombosis (blood clot) of the veins in New England Journal of Medicine, 352(17), 17911798.
the dura mater that surround and drain blood from
the brain.
Salvati, M., Cervoni, L., Raco, A., & Delfini, R. (2001). Spontaneous
Cerebellar Hemorrhage cerebellar hemorrhage: Clinical remarks on 50 cases. Surgical
Neurology, 55, 156161.
E LLIOT J. R OTH
Northwestern University
Chicago, IL, USA
Cerebellar Mutism
Synonyms J ACQUELINE L. C UNNINGHAM
The Childrens Hospital of Philadelphia
Posterior fossa hemorrhage Philadelphia, PA, USA
Definition
Definition
A cerebellar hemorrhage is a bleeding into the cerebellum,
A transient speech disorder typically associated with re-
the portion of the brain located posteriorly, that controls
section of cerebellar tumors, particularly medullo-
balance, coordination, and related functions.
blastomas involving the cerebellar vermis. Cerebellar
mutism can also result from stroke in the cerebrovascular
Current Knowledge distribution, affecting the cerebellar peduncles and brain-
stem. Cerebellar mutism forms part of a syndrome of
It is estimated that 10% of all intracerebral hemorrhages, or deficits, known as the posterior fossa syndrome (PFS).
about 12% of all strokes, are cerebellar hemorrhages. It
can be caused by high blood pressure, heavy alcohol con-
sumption, cocaine use, anticoagulant use, clotting disor- Current Knowledge
ders, cerebral vascular abnormalities such as arteriovenous
malformations and aneurysms, and cerebral amyloid Symptoms
angiopathy. Approximately two-thirds are thought to result
from hypertension. Symptoms of the hemorrhage include As part of PFS, cerebellar mutism is associated with de-
headaches, especially at the posterior and inferior area of creased or absent speech, irritability, hypotonia, ataxia,
the skull, nausea and emesis, stiff neck, dizziness and verti- and the inability to coordinate voluntary movements,
go, blurred or double vision, balance and coordination including the volitional motor aspects of speech. Mutism
deficits, speech difficulty, and altered consciousness. The occurs within the first week of surgery (or cerebrovascular
onset of symptoms is generally abrupt and dramatic. This event) and its duration may be a matter of days or weeks.
is a medical emergency, requiring immediate neurosurgical It is expected to resolve within 4 months. However, even
attention. It is diagnosed using CTor MRI scanning. Treat- with the return of functional speech, the quality of voca-
ment relies on reducing intracranial pressure and surgically lizations may lack normalcy in being hypernasal, mono-
removing the hemorrhage as quickly as possible. tone, high pitched, slowed, and/or sparse.
Cross References
Pathophysiology
Hemorrhagic Stroke
Intracerebral Hemorrhage Information obtained from single photon emission
Intracranial Hemorrhage computerized tomography (SPECT) studies have sup-
ported the theory that cerebellar mutism results from
the effects of decreased cerebral and cerebellar blood
References and Readings
flow upon cell functioning in particular brain pathways.
These cause disruption in the cerebellar modulation
Kirollos, R. W., Tyagi, A. K., Ross, S. A., van Hille, P. T., &
Marks, P. V. (2001). Management of spontaneous cerebellar hema-
of neural circuits that link prefrontal, posterior parietal,
tomas: A prospective treatment protocol. Neurosurgery, 49, superior temporal, and limbic cortices with the
13781386. cerebellum.
Cerebellum C 523
To thalamus and red nucleus cells. Golgi cells are intermediary cells located in the
Corticopontine granular layer. They receive stimulatory input (GABA)
fibers Superior cerebellar from the granular layer to inhibit granule cells.
peduncle
Cerebellum
Nuclei
Pons Purkinje cells can transmit inhibitory signals to the deep
To vestib. nuclei of the cerebellum. The most lateral nucleus,
nuclei
Pontine dentate, receives its input from the OCT and PCT and
Dentate
mossy fibers Interposed
carries planning information from the posterior parietal
Fastigial area. The interpositus nucleus (globose and emboliform)
Middle receives its Purkinje cell input from the OCT and PCT
cerebellar fibers carrying information from primary motor cortex
peduncle (area 4), DSCT, and CCT. Medially located fastigial
Inferior cerebellar nucleus receives input from the DSCT and CCT. Vestibu-
peducncle lar nuclei receive proprioceptive input from the spinal
Climbing fibers Proprioceptive cord and medullary olive.
from inferior olive information from
spinocerebellar tract
(mossy fibers) Fibers
Climbing fibers go to all parts of the cerebellum. They are
Cerebellum. Figure 1
not restricted to a particular zone. A climbing fiber sends
a collateral synapse to the deep cerebellar nuclei, which is
excitatory. The climbing fiber then climbs up, synapsing
Histology on the dendrites of the Purkinje cell. Each Purkinje cell
receives input from only one climbing fiber axon, but
Cortex each climbing fiber axon can split to innervate several
The cerebellar cortex consists of three layers from outer- Purkinje cells. The climbing fiber-Purkinje cell synapses
most to innermost: molecular, Purkinje (pyriform), and are excitatory. The OCT terminates directly on Purkinje
granular layers. The molecular layer consists mainly of cells, by-passes granule cells, and causes complex spikes in
neuropil and is the site of synapses. The majority of cells Purkinje cells.
in this layer are stellate and basket cells in addition to a Mossy fibers are the axons of DSCT, CCT, vestibulo-
few neurons. Purkinje cell layer consists of a single layer of cerebellar (VCT) and PCT carrying input to the cerebellar
large (25 micrometer) pear-shaped neurons. It is consid- cortex. They terminate and excite granule cells. Each
ered the largest cells in the nervous system. The granular mossy fiber branches profusely in the white matter. Each
layer is dense with 37 million neurons per cubic mm, Purkinje cell receives input from approximately >20,000
consisting of small cells with a granular cytoplasm. mossy fibers and only one climbing fiber.
Beneath the cortex lies white matter that forms the core Parallel fibers are the long axons of granule cells that
of the foliae. pass dorsally through the granule and Purkinje cell layers
to reach the molecular layer of the cerebellar cortex,
Cells where they bifurcate and run parallel to the long axis of
The cerebellum is formed of several complex cells. Stellate the folium. Parallel fibers excite a row of Purkinje cells
cells project local inhibitory output (taurine). Basket cells and in addition to a few basket cells that in turn will
also project local inhibitory output (GABA). They feed inhibit distant Purkinje cells outside the field of
forward inhibition on Purkinje cells. Purkinje cells are excitation.
stimulatory, transmitting impulses from the cerebellar The aminergic fibers originate in the Raphe nuclei and
cortex via efferent pathways. They project inhibitory out possess serotonergic input, modulating the granule and
(GABA). Granule cells have axons that run parallel to the molecular layers. This category of fibers also includes
longitudinal axis of the lobes. Granule cell excite by way of those originating from the locus ceruleus possessing
glutamate. Granule cells are only excitatory in the cere- noradrenergic input and terminating in all three cortical
bellar cortex, terminating on Golgi, basket, and Purkinje layers.
Cerebral Amyloid Angiopathy C 525
The cerebellum receives input from all areas of the Albus, J. S. (1971). A theory of cerebellar function. Mathematical
central and peripheral nervous systems. Continuous Biosciences, 10, 2561.
Miller, N., & Newman, N. (2005). Walsh and Hoyts clinical neuro-
flow of information from the spinal centers and cortical C
ophthalmology (6th ed.). Philadelphia, Pennsylvania: Lippincott
areas are integrated in the cerebellar cortex. The cere- Williams and Wilkins.
bellar output then guides the precision of different cere- Ramnani, N. (2006). The primate cortico-cerebellar system: anatomy and
bral functions namely equilibrium, planned voluntary function Nature Reviews Neuroscience, 7, 511522.
movements, and muscle tone. The cerebellar modula- Ropper, A., & Brown, R. (2005). Adams and Victors principles of
neurology (11th ed.). USA: McGraw-Hill Inc.
tion of motor control is executed through its inhibitory
Strick, P. L., Dum, R. P., & Fiez, J. A. (2009). Cerebellum and nonmotor
output to the motor cortex and the descending motor function. Annual Review Neuroscience, 32, 413434.
tracts. Guided by visual, proprioceptive, and vestibular
spinal input, the cerebellum compares the intended
force needed to execute a planned voluntary movement
with the appropriate muscle power needed to execute it. Cerebral Amyloid Angiopathy
It then modulates the tone of the agonist and antagonist
muscles through inhibitory input to the motor cortex, E LLIOT J. R OTH
the pyramidal and extrapyramidal tracts, aiming at the Northwestern University
execution in a precise manner. While this voluntary Chicago, IL, USA
movement is executed, maintenance of equilibrium re-
gardless of movement or body position is achieved
through the cerebellar output to the antigravity muscles Definition
and the vestibular centers. Eye movements are also
maintained during body movement via extensive con- Cerebral amyloid angiopathy is a syndrome characterized
nections with the occulomotor nuclei in the brain stem. by recurrent spontaneous lobar cerebral hemorrhages of
More recent data has shown that the cerebellum is various sizes and in various locations. Each hemorrhage
involved in cognitive, behavioral, and emotional proces- may be asymptomatic or may cause all of the symptoms
sing, including executive control, attention, working of lobar hemorrhages resulting from increased intracranial
memory, learning, language, pain, and emotion (Strick pressure, including severe headache, seizure, stiff neck, and
et al., 2009). vomiting; altered consciousness; paralysis or weakness and
sensory loss; cognitive and language dysfunction, often
Lesions leading to dementia after multiple episodes.
Damage to the cerebellar center or to either its inflow
or outflow tracts leads to loss of cerebellar fine tuning
Current Knowledge
modulation on different cerebral functions. Vestibulocer-
ebellar lesions can result in disequilibrium, nystagmus,
The pathological process that causes this disease is the depo-
abnormal gait, and recurrent falls. Truncal ataxia and
sition of a protein, beta-amyloid, in the walls of the arteries of
scanning speech are seen with spinocerebellar lesions.
the brain. Interestingly, this protein is identical to the one
Patients with corticocerebellar lesions can display signs
found in high quantities in the brains of patients with Alz-
of dysmetria, asynergia (decomposition of the voluntary
heimers disease. The incidence of cerebral amyloid angio-
movement), hypotonia, dysdiadochokinesia, and inten-
pathy is difficult to estimate, but is known to increase with
tion tremors.
advancing age. It is thought to account for 15% of all intra-
cerebral hemorrhages in patients over 60 years and up to
Cross References one-half of lobar intracerebral hemorrhages in patients older
than 70, totaling about 20 per 100,000 per year in that group.
Ataxia Diagnosis is usually made based on the clinical presentation
Dysdiadochokinesia and imaging of recurrent spontaneous lobar hemorrhages,
Glutamate with no other predisposing problems, usually associated with
Nystagmus progressive decline in function, and most often associated
Proprioception with dementia. The recurrences can occur simultaneously,
526 C Cerebral Angiitis
clustered in time, or separated by years. The definitive normal, but magnetic resonance imaging of the brain is
diagnosis, based on pathological findings, is most typically abnormal in more than 90% of patients. However, the
made postmortem. Treatment is usually supportive, consist- pattern of abnormal findings is not specific. Cerebrospi-
ing of observation, symptom relief, and rehabilitation. nal fluid analysis usually reveals elevations in total protein
level or white blood cell count. Angiography has a low
sensitivity and low specificity. Treatment usually includes
Cross References cyclophosphamide and prednisone.
Hemorrhagic Stroke
Lobar Hemorrhage
Cross References
Synonyms
Current Knowledge
Cerebral Blood Flow
Angiitis that is confined to the brain is relatively uncom-
mon, and is called primary angiitis of the central nervous E LLIOT J. R OTH
system (PACNS), isolated CNS vasculitis, primary CNS Northwestern University
vasculitis, or granulomatous angiitis of the nervous sys- Chicago, IL, USA
tem. It usually affects small- and medium-sized cerebral
blood vessels, but does not involve blood vessels outside
of the CNS. Headache and encephalopathy are the most Synonyms
frequent symptoms. Stroke occurs in about 20% of
patients. Blood tests reflecting inflammation are usually Cerebral autoregulation; Cerebral perfusion pressure
Cerebral Cortex C 527
Definition
Cerebral Cortex
Cerebral blood flow is the amount of blood that goes
through the arterial tree in the brain in a given amount K IMBERLE M. J ACOBS
of time. Virginia Commonwealth University
Richmond, VA, USA
C
Current Knowledge
Cerebral Cavernous Malformation Structurally, the cerebral cortex can be divided into four
(CCM) lobes named after the overlying cranial bones: frontal,
parietal, temporal, and occipital. Prominent sulci define
Angioma, Cavernous Angioma the borders of these lobes. Primary functions of the lobes
528 C Cerebral Cortex
Cerebral Dominance
Cerebral Embolism
Hemispheric Specialization
E LLIOT J. R OTH
Northwestern University
Chicago, IL, USA
Cerebral Edema
Synonyms
G ARY T YE 1, J OHN B ROWN 2
1
Virginia Commonwealth University Embolic stroke
Richmond, VA, USA
2
Medical College of Georgia
Augusta, GA, USA Definition
A cerebral embolism is a blood clot (thrombus) that starts
Synonyms from the heart or blood vessel where the clot originates
and stops in an artery that leads to or rests within the
Cytotoxic edema; Vasogenic edema brain. The result is occlusion of the vessel and obstruction
of the flow of oxygen and blood to the brain tissue
supplied by that artery.
Definition
Current Knowledge
Cerebral edema is an increase in the water content of the
brain that leads to brain swelling. It may be divided into Cerebral embolisms cause about 1520% of all strokes and
two broad categories: vasogenic and cytotoxic. about one-quarter of all ischemic strokes. It occurs most
Cerebral Palsy C 531
Anticoagulation
Echocardiogram Cerebral Leukencephalopathy
Infarction
Ischemic Stroke Periventricular Leukomalacia
Myocardial Infarction
Thrombosis
Warfarin
Cerebral Malformation
References and Readings Arteriovenous Malformation (AVM)
Short Description or Definition birth raises the risk of CP, with a recent study reporting a
diagnosis of cerebral palsy among 20% of children born at
As defined by the International Workshop on the Definition or before 27 weeks of gestation versus diagnosis of 56%
and Classification of Cerebral Palsy, Cerebral Palsy (CP) is: of children born between 28 and 31 weeks (Ancel et al.,
2006). Reviewers note that overall rates of CP are climb-
" a group of permanent disorders of the development of
ing, while rates of CP among full-term infants have
movement and posture, causing activity limitation, that
remained stable (1.1/1,000). This suggests that increases
are attributed to non-progressive disturbances that oc-
are largely due to greater survival of preterm infants
curred in the developing fetal or infant brain. The motor
(Mukherjee & Gaebler-Spira, 2007).
disorders of cerebral palsy are often accompanied by
disturbances of sensation, perception, cognition, commu-
nication, and behavior, by epilepsy, and by secondary Natural History, Prognostic Factors,
musculoskeletal problems (Rosenbaum et al., 2007). and Outcomes
Type Description
Spastic cerebral palsy Increased muscle tone with movement
Spastic quadriplegia Spasticity of upper and lower limbs
Spastic diplegia Greater involvement of the legs than the arms
Spastic hemiparesis Greater involvement of one side of the body (more often the right); greater impairment
of the arm than the leg
Extrapyramidal cerebral palsy Involuntary and abnormal muscle movements: dystonia (fluctuating tone and abnormal
body postures) and/or athetoid movements (writhing movements in the extremities)
Hypotonic cerebral palsy Persistent, low muscle tone
Mixed and atypical cerebral palsy Mixture of spasticity and extrapyramidal symptoms
Cerebral Palsy C 533
Domain Symptoms
Secondary muscular and Delayed development of adaptive motor skills
orthopedic symptoms Gait abnormalities C
Oral-motor difficulties and problems with speaking and drooling
Contracture (shortening of the muscle)
Bone deformities (e.g., hip subluxation/dislocation)
Scoliosis
Osteoporosis
Reduced limb growth
Neurologic symptoms Seizure disorder
Sensory Visual problems
Homonomous hemianopsia (in spastic hemiplegia)
Strabismus
Nystagmus
Visual sequela related to prematurity
Tactile/perceptual deficits
Stereognosis
Poor two-point discrimination (in spastic hemiplegia)
Neglect of affected side of the body (in spastic hemiplegia)
Hearing loss
Feeding/gastrointestinal Dysphagia and aspiration
Malnutrition requiring gastrostomy
Gastroesophageal reflux
Constipation
Incontinence or difficulty voiding
Dental Malocclusion
Poor tooth enamel
Pain and fatigue Pain associated with primary (e.g., spasticity and contractures) and secondary
(e.g., constipation) disease processes
Pain related to medical procedures
Fatigue secondary to poor mobility
fine motor deficits can limit participation in traditional disability (e.g., attractiveness, social interaction, athletics,
tests (Fennell & Dikel, 2001). Little is known about cog- academics) (Miyahara & Cratty, 2004). Adolescent girls
nitive functioning among children with CP who do not may be at particularly increased risk for low self-concept
have a diagnosis of mental retardation, reports of deficits (Shields, Murdoch, Loy, Dodd, & Taylor, 2006). Studies of
in learning (specifically arithmetic), visual-perceptual Quality of Life (QOL) suggest lower QOL for children
processing (particularly in spastic diplegia), working with CP, particularly in areas associated with CP and its
memory, and executive functioning are emerging (Blon- sequella (e.g., academics, social interaction) and most
dis, 2004; Jenks et al., 2007). notably for children with quadriplegia/more severe CP
The empirical literature regarding mental health in (Livingston, Rosenbaum, Russell, & Palisano, 2007).
children with CP is sparse. Overall, studies of children Transition to adulthood is understudied, but issues re-
with physical disabilities suggest that difficulties with garding reduced involvement in age-appropriate social
adjustment are atypical, with the exception of poor self- activities and roles, employment, and leisure activities
concept in areas directly impacted by the physical are of concern (Liptak, 2008).
534 C Cerebral Palsy
with hemiplegic cerebral palsy. Cochrane Database of Systematic the mean arterial pressure (MAP) and the intracranial
Reviews (2), CD004149.
pressure (ICP).
Houlihan, C. M., ODonnell, M., Conaway, M., & Stevenson, R. D.
(2004). Bodily pain and health-related quality of life in children
with cerebral palsy. Developmental Medicine and Child Neurology,
46(5), 305310.
Jenks, K. M., de Moor, J., van Lieshout, E. C. D. M., Maathuis, K. G. B.,
Cross References C
Keus, I., & Gorter, J. W. (2007). The effect of cerebral palsy on
arithmetic accuracy is mediated by working memory, intelligence, Cerebral Blood Flow
early numeracy, and instruction time. Developmental Neuropsycholo- Intracranial Pressure
gy, 32, 861879.
Liptak, G. S. (2008). Health and well being of adults with cerebral palsy.
Current Opinion in Neurology, 21, 136142.
Livingston, M. H., Rosenbaum, P. L., Russell, D. J., & Palisano, R. J. References and Readings
(2007). Quality of life among adolescents with cerebral palsy: What
does the literature tell us? Developmental Medicine and Child Neu-
Diringer, M. N., & Axelrod, Y. (2007). Hemodynamic manipulation
rology, 49(3), 225231.
in the neuro-intensive care unit: cerebral perfusion pressure therapy
Menkes, J. H., & Sarnat, H. B. (2000). Perinatal asphyxia and trauma.
in head injury and hemodynamic augmentation for cerebral vaso-
In J. H. Menkes & H. B. Sarnat (Eds.), Child neurology (6th ed.,
spasm. Current Opinion in Critical Care, 13(2), 156162.
pp. 401466). Philadelphia: Lippincott Williams & Wilkins.
Wright, W. L. (2007). Multimodal monitoring in the ICU: when could
Miyahara, M., & Cratty, B. J. (2004). Psychosocial functions of children
it be useful? Journal of the Neurological Sciences, 261(12), 1015.
and adolescents with movement disorders. In D. Dewey & D. E.
Tupper (Eds.), Developmental motor disorders: A neuropsychological
perspective (pp. 427442). New York: Guilford.
Mukherjee, S., & Gaebler-Spira, D. J. (2007). Cerebral palsy. In R. L.
Braddom (Ed.), Physical medicine and rehabilitation (3rd ed.,
pp. 12431267). Philadelphia: Elsevier. Cerebral Seizures
Rosenbaum, P., Paneth, N., Leviton, A., Goldstein, M., Bax, M., Damiano,
D., et al. (2007). A report: The definition and classification
Epilepsy
of cerebral palsy April 2006. Developmental Medicine and Child
Neurology Supplementum, 109, 814.
Shields, N., Murdoch, A., Loy, Y., Dodd, K. J., & Taylor, N. F. (2006).
A systematic review of the self-concept of children with cerebral
palsy compared with children without disability. Developmental
Medicine and Child Neurology, 48, 151157. Cerebral Thrombophlebitis
Tupper, D. E. (2007). Management of children with disorders of motor
control. In S. J. Hunter & J. Donders (Eds.), Pediatric neuropsycho- Central Venous Thrombosis
logical intervention: A critical review of science and practice
(pp. 338365). Cambridge, UK: Cambridge University Press.
Van der Burg, J. J. W., Didden, R., Jongerius, P. H., & Rotteveel, J. J.
(2007). Behavioral treatment of drooling: A methodological critique
of the literature with clinical guidelines and suggestions for future
research. Behavior Modification, 31, 573594. Cerebral Vasculitis
Cerebral Angiitis
Cerebral Perfusion Pressure
G ARY T YE 1, J OHN B ROWN 2
1
Virginia Commonwealth University
Cerebral Venous Thrombosis
Richmond, VA, USA
2
Medical College of Georgia
Central Venous Thrombosis
Augusta, GA, USA
Definition
Cerebral Ventricles
Cerebral perfusion pressure (CPP) is the net pressure of
flow of blood to the brain, which is the difference between Ventricles
536 C Cerebrovascular Accident (CVA)
Multi-infarct Dementia
Cross References
Atherosclerosis
Cerebral Angiitis
Cerebrovascular Disease Cerebral Embolism
Dissection
E LLIOT J. R OTH Hemorrhagic Stroke
Northwestern University Infarction
Chicago, IL, USA Intracerebral Hemorrhage
Ischemic Stroke
Stroke
Definition Subarachnoid Hemorrhage
Thrombosis
Cerebrovascular disease refers to the group of conditions Transcranial Doppler Ultrasonography
characterized by disease of the blood vessels that supply Vascular Dementia
blood to the brain. It can occur in the blood vessels that Vascular Malformation
lead to the brain or in the blood vessels inside the brain. Vasculitis
While it usually presents with symptoms of a stroke (the
group of clinical manifestations of cerebrovascular disease),
it can be asymptomatic, in which case it is usually detected
either by physical examination or selected imaging References and Readings
techniques.
The term cerebrovascular accident (or CVA) is Wolf, P. A., & Grotta, J. C. (2000). Cerebrovascular disease. Circulation.
incorrect and should be avoided, as there is nothing 102, IV-75.
accidental about a stroke. The term cerebrovascular
disease is a more general term than is stroke, because
cerebrovascular disease includes asymptomatic or sub-
clinical disease, in addition to the clinically manifest
strokes.
Most cerebrovascular disease is obstructive in nature,
Cerebrum Surface
caused by atherosclerotic plaques that line the blood
Cerebral Cortex
vessel walls and block the blood flow. If the blockage is
only partial, and is not severe enough to impair brain
function, then the disease remains asymptomatic. How-
ever, if the obstruction is severe enough to reduce blood
supply to the extent that brain injury occurs, then symp-
toms suggesting a stroke ensue. If these symptoms are CES-D
temporary and completely reversed, the phenomenon is
known as a transient ischemic attack. If the brain Center for Epidemiological StudiesDepression
Chapple v. Ganger C 537
Cross References
CGY
Daubert v. Merrell Dow
Centigray
Chandler Exterminators v. Morris Chandler Exterminators Inc. v. Morris, 200 Ga. App. 816 (1992).
Schudel v. General Electric, 120 F. 3d 991 (1995).
(1992)
R OBERT L. H EILBRONNER
Chicago Neuropsychology Group
Chicago, IL, USA Chapple v. Ganger
R OBERT L. H EILBRONNER
Definition Chicago Neuropsychology Group
Chicago, IL, USA
The testimony of neuropsychologists is commonly
challenged by defense attorneys in cases relating to infer-
ences of subtle brain changes associated with neurotoxic Historical Background
brain injury. In the case of Schudel v. General Electric
(1995), plaintiffs accepted neuropsychological evidence In the cause of Daubert v. Merrell Dow (1993), it was ruled
for brain damage caused by organic solvents and poly- that for scientific testimony to be admissible, it has to be:
chlorinated biphenyls (PCBs). However, the federal (a) scientifically valid, and (b) relevant to the case at
appeals court from the Ninth Circuit ruled that neuro- hand. The court provided a list of guidelines intended
psychological testimony is limited only to damages and to aid in the determination of scientific validity (e.g.,
cannot determine physical causation. The court opined peer reviewed, fasifiability, acceptable error rate, etc.).
that determination of causation is relegated to medical The Daubert ruling along with subsequent related rulings
doctors (MDs) or left to the discretion of the jury to (e.g., General Electric v. Joiner, 1997, Kumho Tire v.
make connections between neurocognitive deficits pre- Carmichael, 1999), generated significant debate among
sented and exposure to toxins. In the case of Chandler psychologists and neuropsychologists, and many other
Exterminators v. Morris (1992), the Georgia Supreme disciplines. Specifically, Reed (1996) viewed the Daubert
Court ruled in favor of the trial courts decision to ruling to necessitate the utilization of commercially avail-
prohibit neuropsychological testimony that proposed a able fixed batteries only, such as the HalsteidReitan Bat-
link between neurotoxicants and impaired neuropsycho- tery. However, most neuropsychologists employ a flexible
logical test scores. In response to this case, Georgia legis- battery approach; thus, contradicting Reeds assertions
lature wrote a new law permitting neuropsychologists to implying that most neuropsychologists would not be suit-
provide testimony related to causation of brain injuries ed for involvement in forensic work. In support of his
in Georgia. Challenges to specific neuropsychological conclusion, Reed referenced the case of Chapple v. Ganger
tests and test batteries occur with some degree of fre- (1998), a brain injury claim. Review of the judges written
quency, and they should be taken seriously by all parties decision in Chapple v. Ganger outlined that all
538 C Charles Bonnet Syndrome
general lack of awareness of the syndrome in the medi- Charles Bonnet Syndrome. Table 2
cal community, as well as patients reluctance to dis-
Factors Favoring the Recurrence of Hallucinations
close their hallucinatory symptoms for fear of being
labeled psychotic or demented (Plummer et al., 2007). Dimly lit conditions
One estimate suggests that the prevalence of complex States of drowsiness C
visual hallucinations in patients with visual impairment Physical and social isolation
is between 11% and 15% (Menon et al., 2003).
2. Age of onset: CBS may occur at any age, but it is more
common in the elderly. Average age of onset tends to be
in the 70s and 80s (Plummer et al., 2007). The
increased prevalence of CBS in older adults is likely Charles Bonnet Syndrome. Table 3
related to the greater incidence of sudden visual loss
Factors that May Help Relieve Hallucinations
and/or isolation in this age group (Menon et al., 2003).
Rapid blinking
Sustained eye closure
Natural History, Prognostic Factors, Diversionary activities
Outcomes Limiting exposure to dim lighting
Walking away
Historical background: Charles Bonnet syndrome is
Looking at or approaching images
named after the eminent Swiss philosopher and naturalist
who first described this phenomenon in 1760 (Hedges,
2007). In a book entitled Essai Analytique sur les Faculties
de LAme [Analytical Essays Concerning the Faculties of
the Mind], Charles Bonnet described how his cognitively It has been argued that CBS is more commonly asso-
intact, 89-year-old grandfather with failing eyesight began ciated with higher degrees of visual impairment, and with
to experience well-formed visual hallucinations, which he bilateral as opposed to unilateral ocular pathology
was aware were not actually physically present. Interest- (Menon et al., 2003). It has also been suggested that it is
ingly, Charles Bonnet began to have similar experiences not the specific lesion site or the severity of impairment,
later in his own life. At the age of 22, he began to experi- but rather the rate of development of the visual im-
ence severe eye pain and progressively worsening loss of pairment that best predicts CBS (Plummer et al., 2007).
vision that made it difficult for him to use a microscope, That is, hallucinations may be more common in the
and he ultimately turned to more abstract philosophical context of sudden or unexpected decrease in visual func-
pursuits and theoretical questions in biology. In his re- tion (Menon et al., 2003).
tirement, he experienced formed visual hallucinations Outcomes: The course of CBS can be unpredictable.
associated with many of the common attributes of the While the onset is generally sudden, it may also be gradual
syndrome that shares his name, including blindness, in- (Menon et al., 2003). Hallucinations can last from seconds
tact cognition, and occurrence in quiet and reflective to hours, or even days. Clustering of episodes across days
settings. Another native of Geneva, George de Morsier, or weeks is not unusual. Three patterns of the syndrome
proposed in 1967 that visual hallucinations in older men have been described (Menon et al., 2003). The episodic
without mental deficiency be designated the syndrome of pattern, characterized by hallucinations that happen over
Charles Bonnet. a period of days to months and then permanently cease, is
Current thinking/prognostic factors: The question of reportedly the least common. In the periodic pattern,
whether visual impairment is necessary for the develop- phases of hallucinatory activity alternate with phases of
ment of CBS has been the matter of debate. Some argue remission. The continuous pattern, as its name suggests, is
that CBS is almost invariably associated with impaired characterized by unremitting hallucinations (i.e., no hal-
vision, and it may occur whenever sensory input to the lucination-free intervals) (Menon et al., 2003). Overall,
brain is decreased sufficiently to allow release phenomena. the duration of CBS can extend from days to years, and
Other researchers report that visual dysfunction, though spontaneous recurrence after a symptom-free interval is
common, is not mandatory for diagnosis, and note that possible. Interestingly, some sufferers have reported per-
CBS has been found in individuals with intact vision manent remissions of CBS in conjunction with ongoing
(Terao & Collinson, 2000). visual decline (Plummer et al., 2007).
540 C Charles Bonnet Syndrome
Synonyms
Current Knowledge EDTA therapy
Whiteneck, G., Brooks, C. A., Charlifue, S., Gerhart, K. A., Mellick, D.,
Categorization
Overholser, D., et al. (1998). Guide for use of the CHART: Craig
handicap assessment and reporting technique. www.craighospital. While Chelation therapy has been used for treating lead
org/Research/CHART December 28, 2009. poisoning and vascular occlusive disease, oral chelation ther-
apy with the a-ketohydroxypyridine chelator 1,2-dimethyl-
3-hydroxypyrid-4-one (L1, INN/BAN: deferiprone) has
also been used in iron- and aluminum-overloaded patients.
A number of iron(III) chelators have also shown antimalar-
CHART-SF ial activity in vitro with the proposed mechanism of activ-
ity involving the withholding of iron from metabolic
CHART Short Form pathways of the intra-erythrocytic parasite and the forma-
Craig Handicap Assessment and Reporting Technique tion of toxic complexes with iron.
Chemical AIDS C 543
Evaluation
Adverse Side Effects
Studies examining the efficacy of Chelation therapy have
shown mixed results. In one authoritative systematic re-
In one systematic review, adverse effects were character-
view, it was stated that proponents of chelation therapy
ized as rare but cases of hypocalcemia and a single case of
adhere to pathophysiological models of arteriosclerosis,
increased creatinine was noted in a patient on the EDTA
which are inconsistent with current knowledge and prac-
intervention (Seely, Wu & Mills, 2005). Moreover, it was
tice (Ernst et al., 2001). In a systematic review of chelation
emphasized that if the treatment is used in lieu of proven
therapy in the treatment of malaria, it was concluded that
therapies, indirect harm to the patient could result. In
when used via oral administration, its efficacy and low
another systematic review, it was reported that EDTA
cost make it more accessible than desferrioxamine for the
treatment may be associated with life-threatening adverse
majority of patients needing iron chelation (Mabeza et al.,
effects, such as hypocalcemia and severe kidney damage,
1999).
in addition to prolonged bleeding and respiratory distress
(Ernst et al., 2001).
Treatment and Mechanisms
Adjuvant chemotherapy may also reduce the proba- the cell attempts to divide and before repair occurs
bility of tumor resistance to drug therapy in the event of (Chabner & Longo, 2004).
disease recurrence (Chabner & Longo, 2004). Further- The probability of the intended effect on the targeted
more, adjuvant chemotherapy is effective at destroying molecules reflects the appropriate concentration of drugs,
residual cancer cells that have spread to distal parts of amount or dose and timing of drug administration. Drug C
the body (i.e., metastasis), particularly because rapidly absorption, distribution, and penetration are also signifi-
proliferating lesions are very amenable to treatment. cant factors inherent in the efficacy of chemotherapy.
Palliative chemotherapy is indicated when the curative The precise drug dosage can be complicated because if
potential is very low and the primary goals are to decrease the amount is too low, it may be ineffective against the
the patients tumor burden and prolong life expectancy. tumor. Conversely, if the dosage is too high, patients may
suffer from excessive toxicity.
Since chemotherapy damages healthy cells during the
Current Knowledge therapeutic process, the treatment is associated with
several harmful side effects, such as myelosuppression.
Recent neuropsychological research has indicated that For example, bone marrow, which produces white blood
chemotherapy can also adversely impact cognitive cells, red blood cells, and blood platelets, can be damaged
functioning, both short-term and delayed. In particular, during chemotherapy treatment. In particular, white
neuropsychological research studies have provided blood cells and platelets frequently drop transiently after
evidence discussing the impact of chemotherapy on atten- chemotherapy, so that patients are at increased risk for
tion, memory, and concentration (Armstrong, Gyato, infection and bleeding during and after chemotherapy.
Awadalla, Lustig, & Tochner, 2004). Consequently, many Many chemotherapy patients also suffer from nausea
of these chemotherapy-induced cognitive impairments and vomiting because the drugs irritate the stomach lining
can significantly impair patients daily activities, such as and bowel. Certain chemotherapy drugs also cause alope-
working, being involved in a committed relationship, and cia or hair loss. This condition results from the chemo-
attending to personal responsibilities. Research has therapy agent adversely affecting the growth of hair cells,
suggested that many of these impairments are temporary causing them to become brittle and eventually break.
but some may be more long-term, or even permanent. Several chemotherapies also result in anorexia, severe
The cognitive effects are not uniform, and the severity loss of appetite, and significant weight loss. Fatigue, diar-
appears to reflect a higher concentration and/or larger rhea, and constipation are also very common side effects
dose of chemotherapy. from cancer and chemotherapy. Additionally, specific
Most of the different types of chemotherapy drugs chemotherapy agents can cause stomatitis, a condition
damage or interfere with the replication of DNA and/or that results in sores manifesting inside the mouth or throat.
RNA, and are used to treat several malignancies, particu- Chemotherapy is most often given intravenously,
larly brain tumors, lymphomas, and leukemias. Some of involving a thin needle, which is inserted into a patients
the chemotherapies include: alkylating agents (such as vein on the hand or lower arm. Intravenous chemotherapy
cisplatin, carboplatin, cyclophosphamide, and temozolo- can also be delivered through catheters, ports, and pumps.
mide) to treat brain tumors, lymphomas, and leukemias; The treatment is frequently given in cycles that reflect
nitrosoureas (e.g., carmustine and lomustine) to treat brain specified treatment periods which are alternated with
tumors and lymphomas; antimetabolites to treat leukemias rest periods. This is necessary because patients require
(such as methotrexate); anthracycline and related drugs substantial relief to permit the body to recuperate, build
(e.g., doxorubicin), which have toxic effects on the heart; healthy new cells, and restore strength.
topoisomerase inhibitors (such as topotecan, irinotecan, Treatment regimens may be given daily, weekly,
and etoposide); mitotic inhibitors (e.g., vinblastine and or monthly and are based upon a drugs efficacy or
vincristine), which can cause peripheral nerve damage; toxicity. Chemotherapy is usually administered before
and corticosteroid hormones, which can be used to kill or (neo-adjuvant) surgery or post (adjuvant) surgery.
slow the growth of cancer cells. There are other che- Neo-adjuvant chemotherapy is intended to decrease the
motherapies that are excluded from these categories, such primary tumors size. This potentially mitigates the harm-
as L-asparaginase, -hydroxyurea, and -thalidomide. The ful effects of surgery or radiotherapy and enhances the
specific manner in which chemotherapy achieves the efficacy of chemotherapy.
intended effect is contingent upon the particular drug(s) Adjuvant chemotherapy is employed when there is
employed. However, cytotoxicity usually occurs when scant evidence of residual disease, but there is an increased
546 C Chief Sensory Nucleus of V
risk of cancer recurrence. Adjuvant chemotherapy may cuneatus and gracilis in the medulla, which mediate simi-
also reduce the probability of tumor resistance to drug lar input from the trunk and extremities. It gives rise to
therapy in the event of disease recurrence (Chabner & trigeminothalamic fibers, which terminate in the ventral
Longo, 2004). Furthermore, adjuvant chemotherapy is posterior medial nucleus of the thalamus.
effective at destroying residual cancer cells that have
spread to distal parts of the body (i.e., metastasis), partic-
ularly since rapidly proliferating lesions are very amenable Current Knowledge
to treatment. Palliative chemotherapy is indicated when
the curative potential is very low and the primary goals Because of its size and density, it is rare for brainstem
are to decrease the patients tumor burden and prolong lesions to be isolated to a single nucleus or pathway.
life expectancy. Theoretically, lesions which involve this nucleus might
most readily be distinguished on a routine neurological
exam by changes (asymmetries) in two-point discrimina-
Cross References tion on the ipsilateral face. In practice, however, such
lesions are likely to involve other brainstem nuclei and
Systemic Therapy pathways, including the adjacent motor nucleus of V,
spinal trigeminal tract and/or nucleus, spinal thalamic
tracts, lateral portions of the medial lemniscus, and mid-
dle cerebellar peduncles resulting in ipsilateral muscle
References and Readings weakness of the jaw muscles, ipsilateral changes in pain
and temperature in the face and diminished or abolished
Armstrong, C. L., Gyato, K., Awadalla, A. W., Lustig, R., & Tochner, Z. A. corneal reflex, contralateral loss of pain and temperature
(2004). A critical review of the clinical effects of therapeutic irradia-
in the extremities, diminished or loss of proprioception,
tion damage to the brain: the roots of controversy. Neuropsychology
Review, 14, 6586. stereognosis, and vibration in the contralateral extremities
Chabner, B. A., & Longo, D. L. (2004). Cancer chemotherapy and biother- (leg > arm), and ipsilateral cerebellar signs.
apy: Principles and practice. Hagerstown, MD: Lippincott, Williams &
Wilkins.
References and Readings
Synonyms
Definition
ASEBA; CBCL
Nucleus responsible for proprioceptive feedback from the
muscles of facial expression, stereognosis or fine tactual
discrimination, and vibratory sensations from the face. Description
Located in the dorsolateral pons just medial to the middle
cerebellar peduncle and inferior to the superior cerebellar The Achenbach System of Empirically Based Assessment
peduncle, it is the functional equivalent of the nuclei (ASEBA) comprises a family of forms for rating
Child Behavior Checklist C 547
behavioral/emotional problems and adaptive characteris- interviewer who administers the SCICA to 6- to 18-year-
tics. For ages 1 to 90 years, developmentally appropri- olds and the Test Observation Form (TOF), which test
ate forms are designed to be completed by collaterals who examiners use to rate the behavior observed during the
know the person who is being assessed. These forms administration of individual ability and achievement tests
include versions of the Child Behavior Checklist to 2- to 18-year-olds. Table 1 summarizes the ASEBA C
(CBCL), completed by parent figures for 1- to 5-year- forms, ages covered, who completes the forms, and refer-
olds and for 6- to 18-year-olds; the Caregiver-Teacher Re- ences to manuals for each form.
port Form (C-TRF) for ages 15, completed by daycare
providers and preschool teachers; the Teachers Report
Form (TRF) for ages 618, completed by teachers and Normed Profiles
other school personnel; the Adult Behavior Checklist
(ABCL) for ages 1859, completed by spouses, partners, Scores obtained from all ASEBA forms are displayed on
family members, friends, therapists, and other collaterals; profiles in relation to norms that are based on distribu-
and the Older Adult Behavior Checklist (OABCL) for ages tions of scale scores obtained by large samples of peers.
60 and older, completed by caregivers as well as by For the collateral and self-report forms for ages 1 to
collaterals. 90 years, norms are based on a US national probability
The ASEBA also includes parallel forms completed by sample of people who had not received mental health or
the people being assessed, including the Youth Self-Report substance abuse services in the preceding 12 months. For
(YSR) for ages 1118, the Adult Self-Report (ASR) for the CBCL/618, TRF, and YSR, norms are provided for
ages 1859, and the Older Adult Self-Report (OASR) for many cultures in addition to the USA, as detailed later
ages 60 and older. The collateral and self-report forms (Achenbach & Rescorla, 2007a, b). Multicultural norms
assess functioning in everyday contexts over periods of for the CBCL/15 and C-TRF were released in 2010. For
26 months. the DOF, SCICA, and TOF, norms are based on ratings of
In addition to the collateral and self-report forms, children observed in the contexts for which these instru-
other ASEBA forms are designed for rating behavior ob- ments are designed.
served in specific situations. These forms include the Each profile displays an individuals scale scores in
Direct Observation Form (DOF), which is completed by terms of standard scores (T scores) and percentiles based
observers who rate two or more 10-min samples of chil- on the normative sample of that individuals peers, as
drens behavior observed in classrooms and other group rated by a particular type of informant (e.g., parent,
settings; the Semistructured Clinical Interview for Chil- teacher, self). The profiles also display demarcations be-
dren and Adolescents (SCICA), which provides an inter- tween the normal range, borderline clinical range, and
view protocol and a rating form completed by the clinical range on each scale. Figure 1 illustrates a profile
70 L
R
E 65
N
60 O
55 R
M
50
Anxious/ Withdrawn/ Somatic Social Thought Attention Rule-breaking Aggressive A
L
depressed depressed complaints problems problems problems behavior behavior
Total score 12 12 6 14 14 9 5 14
T score 70-C 83-C 64 80-C 77-C 63 56 67-B
Percentile >97 >97 92 >97 >97 90 73 96
1 14.Cries 2 5.EnjoysLittle 2 47.Nightm ares 2 11.Dependent 2 9.MindOff 0 1.ActsYoung 1 2.Alcohol 2 3.Argues
0 29.Fears 2 42.PreferAlone 1 51.Dizzy 2 12.Lonely 0 18.HarmSelf 0 4.FailsToFinish 0 26.NoGuilt 1 16.Mean
0 30.FearSchool 2 65.WontTalk 1 54.Tired 1 25.NotGetAlong 1 40.HearsThings 2 8.Concentrate 0 28.BreaksRules 0 19.DemAtten
0 31.FearDoBad 2 69.Secretive 0 56a.Aches 0 27.Jealous 2 46.Twitch 0 10.SitStill 0 39.BadFriends 1 20.DestroyOwn
0 32.Perfect 0 75.Shy 1 56b.Headaches 2 34.OutToGet 0 58.PicksSkin 2 13.Confused 0 43.LieCheat 0 21.DestroyOther
2 33.Unloved 0 102.LacksEnergy 0 56c.Nausea 2 36.GetsHurt 2 66.RepeatsActs 1 17.Daydream 0 63.PreferOlder 1 22.DisobeyHome
2 35.Worthless 2 103.Sad 0 56d.EyeProb 1 38.Teased 0 70.SeesThings 1 41.Impulsive 0 67.RunAway 1 23.DisobeySchl
2 45.Nervous 2 111.Withdrawn 1 56e.SkinProb 2 48.NotLiked 2 76.SleepsLess 1 61.PoorSchool 1 72.SetsFires 1 37.Fights
0 50.Fearful 0 56f.Stomach 1 62.Clumsy 0 83.StoresUp 2 78.Inattentive 0 81.StealsHome 0 57.Attacks
0 52.Guilty 0 56g.Vomit 1 64.PreferYoung 1 84.StrangeBehav 0 82.StealsOther 0 68.Screams
1 71.SelfConsc 0 79.SpeechProb 2 85.StrangeIdeas 1 90.Swears 1 86.Stubborn
2 91.ThinkSuic 2 100.SleepProb 1 96.ThinksSex 2 87.MoodChang
2 112.Worries 0 99.Tobacco 2 89.Suspicious
0 101.Truant 0 94.Teases
1 105.UsesDrugs 2 95.Temper
0 97.Threaten
0 104.Loud
Copyright 2001 T.M. Achenbach B = Borderline clinical range; C = Clinical range Broken lines = Borderline clinical range
Child Behavior Checklist. Figure 1 Syndrome profile scored from the Youth Self-Report completed by 15-year-old Wayne Webster (From Achenbach & Rescorla, 2001, p. 33)
Child Behavior Checklist C 549
of syndrome scales scored from the YSR completed by McConaughy, & Howell, 1987). Consequently, profes-
15-year-old Wayne Webster (not his real name). sionals who work with children recognize the need to
obtain reports from multiple informants. Meta-analyses
of correlations between collateral and self-reports of
Scales on Which ASEBA Instruments adult psychopathology have also revealed only modest C
Are Scored correlations that argue for using multi-informant data to
assess adults (Achenbach, Krukowski, Dumenci, &
ASEBA problem items are scored on scales for syn- Ivanova, 2005).
dromes derived empirically via exploratory factor ana- Because each informant may provide valid and useful
lyses (EFAs) and confirmatory factor analyses (CFAs). information that differs from what other informants
These empirically derived syndromes reflect patterns of provide, data from multiple informants should be com-
problems found to co-occur in ratings by each kind of pared. Software for scoring ASEBA forms facilitates cross-
informant. informant comparisons by printing scores obtained from
In addition to the syndrome scales, each form is parallel forms on parallel profiles. In addition, it prints
scored on DSM-oriented scales constructed by having side-by-side comparisons of ratings by up to eight infor-
experts from many cultures select ASEBA problem items mants on all problem items that have counterparts on
that are very consistent with particular diagnostic cate- forms completed by different informants. It also prints Q
gories of the American Psychiatric Associations (1994) correlations that measure the degree of agreement be-
Diagnostic and Statistical Manual-Fourth Edition (DSM- tween each pair of informants and compares them with
IV). Like the syndrome scales, the DSM-oriented scales Q correlations between pairs of informants in large refer-
are displayed on profiles in terms of T scores, percentiles, ence samples.
and normal, borderline clinical, and clinical ranges. Most An especially useful kind of comparison between
forms are also scored on scales comprising critical items informants reports is illustrated in Fig. 2. This is a com-
that are of particular concern to clinicians. parison between syndromes scored from the YSR com-
The collateral and self-report forms are additionally pleted by Wayne Webster, CBCLs completed by Waynes
scored on scales for favorable characteristics, such parents, and TRFs completed by three of Waynes tea-
as competence, adaptive functioning, and personal chers. For each syndrome, such as the Anxious/Depressed
strengths. The particular items and scales are geared to syndrome shown in the upper left-hand corner, the bars
the developmental level of people being assessed and to reflect the magnitude of standard scores (T scores)
the informants knowledge of people being assessed. For obtained from ratings by each kind of informant. Because
example, parents of 6- to 18-year-olds provide data re- the T scores are based on ratings by each kind of infor-
garding their childrens involvement in sports, nonsports mant for a normative sample of children, the height of the
activities, organizations, jobs and chores, friendships, and bar indicates the level of the problems reported by a
relationships with parents, siblings, and peers. Teachers particular kind of informant compared to problems
provide data on childrens academic performance and reported by that kind of informant for a normative sam-
adaptive characteristics at school. For adults, data are ple of children. For example, the leftmost bars indicate
requested regarding friendships, relations with spouse or that the Anxious/Depressed syndrome scores obtained
partner, children, job, and enrolment in educational pro- from CBCL ratings by Waynes parents are above the top
grams. Only the items relevant to the adult being assessed broken line compared to parents CBCL ratings of a
are scored. For example, adults who lack a spouse or normative sample of adolescent boys. As scores above
partner, children, job, or enrolment in educational pro- the top broken line are in the clinical range, the CBCL
grams are not scored on those items. Adult forms also bars indicate that Waynes parents reported more pro-
have normed scales for substance use. blems of this syndrome than were reported by parents of
97% of boys in the normative sample.
Cross-Informant Comparisons
Multicultural Norms
Meta-analyses have revealed that correlations between
parent, teacher, and self-reports of childrens problems Norms obtained in one society may not be generalizable
are typically only low to moderate (Achenbach, to other societies. To determine the degree of
550
Cross-informant comparison - CBCL/TRF/YSR Syndrome scale T Scores (2001 version)
C
ID: 2301251405 Name: Wayne webster Gender: Male Birth date: 03/03/1986 Comparison date: 04/13/2001
Form Eval ID Age Informant name Relationship Date Form Eval ID Age Informant name Relationship Date
CBC1 001 15 Alice N. Webster Biological mother 04/04/2001 TRF5 005 15 Carmen Hernandez Classroom teacher {F} 04/11/2001
CBC2 002 15 Ralph F. Webster Biological father 04/05/2001 TRF6 006 15 Charles Dwyer Classroom teacher {M} 04/12/2001
YSR3 003 15 Self Self 04/08/2001
TRF4 004 15 George jackson Classroom teacher {M} 04/10/2001
90
Child Behavior Checklist
80
70
60
50
72C 72C 70C 68B 61 63 82C 70C 83C 79B 74C 74C 58 61 64 50 58 62
CBC1 CBC2 YSR3 TRF4 TRF5 TRF6 CBC1 CBC2 YSR3 TRF4 TRF5 TRF6 CBC1 CBC2 YSR3 TRF4 TRF5 TRF6
90
80
70
60
50
69B 69B 80C 70C 65B 69B 69B 66B 77C 70C 60 66B 71C 76C 63 68B 65B 64
CBC1 CBC2 YSR3 TRF4 TRF5 TRF6 CBC1 CBC2 YSR3 TRF4 TRF5 TRF6 CBC1 CBC2 YSR3 TRF4 TRF5 TRF6
70
60
50
57 62 56 63 53 53 73C 73C 67B 82C 65B 67B
CBC1 CBC2 YSR3 TRF4 TRF5 TRF6 CBC1 CBC2 YSR3 TRF4 TRF5 TRF6
Child Behavior Checklist. Figure 2 Cross-informant comparisons of syndrome scores for Wayne Webster (From Achenbach & Rescorla, 2001, p. 39)
Child Behavior Checklist C 551
generalizability across societies, the same assessment instruments, theory, and applications, as well as directions
instruments must be administered to large representative in which the ASEBA is now moving. Translations are
samples of people in different societies. This has been available in 85 languages. Over 6,500 publications by
done with ASEBA instruments in many societies. CFAs some 9,000 authors report use of the ASEBA in 80 cultural
of CBCL, TRF, and YSR data from many societies support groups and societies (Berube & Achenbach, 2010). ASEBA C
the generalizability of syndromes that were initially instruments are available in paper and Internet-based
derived from US samples. Comparisons of scale scores electronic versions in many countries around the world
show that the distributions of CBCL, TRF, and YSR scores for practical assessment in clinical, educational, forensic,
in many societies approximate those obtained in the USA. and other services, as well as for research on countless
However, some societies have substantially lower or topics, such as genetics, medical conditions, outcome
higher mean scores. To take account of societal differences evaluations, epidemiology, development, diagnosis, and
in scale scores, separate sets of norms have been con- multicultural comparisons. Because the ASEBAs concep-
structed for the societies obtaining relatively low scores, tual framework is open ended and generative, it continues
societies obtaining intermediate scores, and societies to advance in multiple directions (Achenbach, 2009).
obtaining relatively high scores. Because parent, teacher,
and self-ratings often yield different scores, the multicul-
tural CBCL, TRF, and YSR norms were constructed sepa- Psychometric Data
rately. For some societies, problem scores obtained from
one kind of informant are relatively low, while scores Table 2 summarizes psychometric data for all ASEBA
obtained from another kind of informant are intermedi- instruments in terms of mean alphas, test-retest reliability,
ate or high. For example, CBCL and TRF problem scores and the percentage of variance in ASEBA scale scores
obtained from Japanese parents and teachers are in the accounted for by clinical referral status, after partialing
low range, whereas YSR scores obtained from self-ratings out demographic effects. Many additional psychometric
by Japanese youths are in the intermediate range. findings including goodness of fit obtained from CFAs
To enable practitioners and researchers to compare in diverse samples are reported in ASEBA manuals and
CBCL, TRF, and YSR scores with culturally appropriate in refereed publications listed by Berube and Achenbach
norms, the scoring software provides options for displaying (2010).
problem scale scores in relation to norms for low-scoring,
intermediate-scoring, and high-scoring societies. For ex-
ample, CBCL and TRF scores for a Japanese youth would Clinical Uses
typically be displayed in relation to norms for low-scoring
societies. However, the youths YSR scores would be dis- ASEBA instruments have numerous clinical uses. Berube
played in relation to norms for intermediate-scoring socie- and Achenbach (2010) list publications reporting use of
ties. If the Japanese youth lived in the USA and attended an the ASEBA in relation to over 150 medical conditions.
American school, the TRF scores would be displayed in Some 600 publications report use of the ASEBA for eval-
relation to US norms for teachers ratings. If the youths uating treatments and outcomes for many kinds of psy-
parents were well acculturated to the USA, the CBCL scores chopathology and other problems.
could be displayed in relation to US norms and also in ASEBA instruments can be used at many stages of
relation to Japanese norms to see whether the scores were clinical processes, including screening to identify needs
clinically deviant according to either set of norms. for help, documentation of problems and adaptive func-
tioning for use in clinical referrals, and intake assessment
on which to base treatment decisions. During the course
Historical Background of treatment, ASEBA instruments are useful for determin-
ing whether goals are being met. Following the treatment,
The ASEBA stems from Achenbachs (1966) factor- ASEBA instruments can be readministered to evaluate
analytic derivation of syndromes of child and adolescent outcomes and subsequent functioning. At any point,
psychopathology. Since then, over 4 decades of research ASEBA instruments can be used to assess behavioral/
and practical experience have produced ASEBA instru- emotional concomitants of neuropsychological and med-
ments for ages 1 to 90 years. Achenbach (2009) docu- ical disorders. The availability of similar ASEBA instru-
ments the historical development of ASEBA research, ments for children and adults facilitates family
552 C Child Behavior Checklist
assessment, as well as close coordination between inter- Achenbach, T. M., & Rescorla, L. A. (2003). Manual for the ASEBA adult
forms & profiles. Burlington, VT: University of Vermont, Research
ventions for parents and their children.
Center for Children, Youth, and Families.
Achenbach, T. M., & Rescorla, L. A. (2007a). Multicultural supplement to
the manual for the ASEBA school-age forms & profiles. Burlington,
References and Readings VT: University of Vermont Research Center for Children, Youth, and
Families.
Achenbach, T. M. (1966). The classification of childrens psychiatric symp- Achenbach, T. M., & Rescorla, L. A. (2007b). Multicultural understanding
toms: A factor-analytic study. Psychological Monographs, 80, (No. 615). of child and adolescent psychopathology: Implications for mental health
Achenbach, T. M. (2009). The Achenbach system of empirically based assessment. New York: Guilford Press.
assessment (ASEBA): Development, findings, theory, and applications. Achenbach, T. M., & Reescorla, L. A. (2010). Multicultural guide to the
Burlington, VT: University of Vermont, Research Center for manual for the ASEBA preschool forms & profilies. Burlington, Ver-
Children, Youth, and Families. mont, University of Vermont Research Center for Children, Youth,
Achenbach, T. M., Krukowski, R. A., Dumenci, L., & Ivanova, M. Y. and Families.
(2005). Assessment of adult psychopathology: Meta-analyses and American Psychiatric Association (1994). Diagnostic and statistical man-
implications of cross-informant correlations. Psychological Bulletin, ual of mental disorders (4th ed.). Washington, DC: Author.
131, 361382. Berube, R. L., & Achenbach, T. M. (2010). Bibliography of published
Achenbach, T. M., McConaughy, S. H., & Howell, C. T. (1987). Child/ studies using the Achenbach system of empirically based assessment
adolescent behavioral and emotional problems: Implications of (ASEBA). Burlington, VT: University of Vermont, Research Center
cross-informant correlations for situational specificity. Psychological for Children, Youth, and Families.
Bulletin, 101, 213232. McConaughy, S. H., & Achenbach, T. M. (2001). Manual for the semi-
Achenbach, T. M., Newhouse, P. A., & Rescorla, L. A. (2004). Manual for structured clinical interview for children and adolescents (2nd ed.).
the ASEBA older adult forms & profiles. Burlington, VT: University of Burlington, VT: University of Vermont, Research Center for Chil-
Vermont, Research Center for Children, Youth, and Families. dren, Youth, and Families.
Achenbach, T. M., & Rescorla, L. A. (2000). Manual for the ASEBA McConaughy, S. H., & Achenbach, T. M. (2004). Manual for the test
preschool forms & profiles. Burlington, VT: University of Vermont, observation form for ages 218. Burlington, VT: University of
Research Center for Children, Youth, and Families. Vermont, Research Center for Children, Youth, and Families.
Achenbach, T. M., & Rescorla, L. A. (2001). Manual for the ASEBA school- McConaughy, S. H., & Achenbach, T. M. (2009). Manual for the ASEBA
age forms & profiles. Burlington, VT: University of Vermont, direct observation form for ages 611. Burlington, VT: University of
Research Center for Children, Youth, and Families. Vermont, Research Center for Children, Youth, and Families.
Childhood Autism Rating Scales C 553
sensory and stereotypy construct, and (4) an activity Rating Scale (CARS). Journal of Autism and Developmental Disorders,
10, 91103.
level and consistency of intellectual response compo-
Schopler, E., Reichler, R. J., & Renner B. (1988). The Childhood Autism
nent, accounted for 57% of the variance in ratings. Rating Scale (CARS). Los Angeles, CA: Western Psychological
Services.
Clinical Uses
Cross References
each subtest. The last subtest on both levels requires the deficits or speech/language difficulties (Boll, 1993). The
child to remember and reapply the conceptual rules from CCT can provide insights regarding a childs cognitive
previous subtests. abilities and learning strategies in terms of difficulties in
The CCT was normed on a stratified representative memory or shifting between conceptual ideas (MacNeil
sample of 920 children in 12 age groups ranging from Horton, 1996). However, a poor score on the CCT does C
5 years to 16 years, 11 months. Administration requires not necessarily indicate a neurologically based disorder;
approximately 1520 min. The raw score is the total rather it indicates a disruption in mental processing
number of errors (CCT Total), which is converted into (Boll, 1993).
an age-normed T-score (M = 50, SD = 10). The CCT is Studies of the CCTs sensitivity to brain dysfunction
easy to administer and score. indicate that the measure is not consistently sensitive to
structural brain damage or neurodevelopment disorders
in children (Bello, Allen, & Mayfield, 2008; Donders,
Historical Background 1996). Results from studies examining the sensitivity of
the CCT to various forms of brain dysfunction suggest
The CCT was developed in an effort to provide an efficient that the CCT assesses multiple dimensions of problem
and well-normed childrens version of the HCT, which is solving, rather than a general construct of abstraction
well documented to be sensitive to cerebral impairment (Nesbit-Greene & Donders, 2002), which has led some
(Reiten & Wolfson, 1992), and the previous childrens to caution against relying on the total error score as the
versions of the Category Test (Reiten & Wolfson, 1993). sole index of brain dysfunction (Allen, Knatz, & Mayfield,
Concerns about lengthy administration times and expen- 2006; Bello et al., 2008). The use of the CCT with brain-
sive, bulky equipment led to the development of various injured children may be hampered by the fact that the
short forms of these tests (e.g., Short Category Test Book- overall T-score is based on six different subtests that differ
let Format), with the most comprehensive of these efforts in their sensitivity to the severity of the injury (Nesbit-
resulting in the CCT (Boll, 1993). The CCT and California Greene & Donders, 2002). Another problem with using the
Verbal Learning Test-Childrens Version (CVLT-C) were CCT with brain-injured children is that it is untimed and
standardized and normed on the same population. children are provided with corrective feedback throughout
the test administration, and these features may compensate
for any deficits in processing speed or executive function
Psychometric Data that the child may be experiencing (Donders & Nesbit-
Greene, 2004). Overall, studies investigating the use of the
Median internal consistency reliability for the CCT Total CCT with brain-injured children suggest that caution is
score is 0.88 for Level 1 and 0.86 for Level 2, with average needed in interpreting the results, and that it should be
standard errors of measurement of 3.46 and 3.74, respec- supplemented with psychometric data from additional
tively (Boll, 1993). The separate age-level values and neuropsychological tests (Donders & Nesbit-Greene,
averaged coefficients indicate that the CCT possesses a 2004; Moore, Donders, & Thompson, 2004).
high degree of internal consistency across ages. The
manual reports a variety of studies demonstrating that
the CCT consistently and significantly correlates with Cross References
other measures of achievement or cognitive ability, such
as the WISC-R and CVLT-C. Concept Learning
Delis-Kaplan Executive Function System
Halstead-Reitan Neuropsychological Test Battery
Clinical Uses Memory (Including Memory Impairment)
Nonverbal Learning Disabilities
The CCT is a widely used test of the ability to solve Problem Solving
problems by developing and modifying strategies of
responding to various visual designs and patterns References and Readings
(Nesbit-Greene & Donders, 2002). The CCT does not
require demonstration of acquired skills, ability, or Allen, D. N., Knatz, D. T., & Mayfield, J. (2006). Validity of the childrens
knowledge, and eliminates potential confounding vari- category test-level 1 in a clinical sample of heterogeneous forms of
ables because it can be used with children with motor brain dysfunction. Archives of Clinical Neuropsychology, 21, 711720.
556 C Childrens Memory Scale
Bello, D. T., Allen, D. A., & Mayfield, J. (2008). Sensitivity of the childrens of both immediate and delayed (30 min) recall. Each
category test level 2 to brain dysfunction. Archives of Clinical
Auditory/Verbal subtest also provides a measure of recogni-
Neuropsychology, 23, 329339.
Boll, T. (1993). Childrens category test. San Antonio, TX: Psychological
tion recall.
Corporation. After administration of the core subtests (Table 1), the
Donders, J. (1996). Validity of short forms of the intermediate Halstead examiner can derive eight index scores: Attention/Con-
category test in children with traumatic brain injury. Archives of centration, Verbal Immediate, Verbal Delayed, Delayed
Clinical Neuropsychology, 11, 131137.
Recognition, Visual Immediate, Visual Delayed, Learning,
Donders, J., & Nesbit-Greene, K. (2004). Predictors of neuropsychologi-
cal test performance after pediatric traumatic brain injury.
and General Memory (Fig. 1). The learning Index is
Assessment, 11(4), 275284. derived using subtest scores from the Auditory/Verbal
MacNeil Horton, A. (1996). Book and test reviews. Archives of Clinical (Word Pairs) and the Visual/Nonverbal (Dot Locations)
Neuropsychology, 11, 171173. domains. The General Memory Index is a measure of
Moore, B. A., Donders, J., & Thompson, E. H. (2004). Validity of the
global memory functioning and is generated using both
childrens category test-level 1 after pediatric traumatic brain injury.
Archives of Neuropsychology, 19, 19.
the immediate and delayed memory indexes from the
Nesbit-Greene, K., & Donders, J. (2002). Latent structure of the childrens Auditory/Verbal and Visual/Nonverbal domains.
category test after pediatric traumatic head injury. Journal of Clinical The immediate portion of the core battery takes
and Experimental Neuropsychology, 24(2), 194199. approximately 3040 min to administer, with an additional
Reiten, R. M., & Wolfson, D. (1992). Neuropsychological evaluation of
1020 min required for administration of the delayed
older children. South Tucson, AZ: Neuropsychology Press.
Reiten, R. M., & Wolfson, D. (1993). The Halstead-Reitan neuropsycho-
recognition sections. Two record forms are provided for
logical test battery: Theory and clinical interpretation (2nd ed.). South students of 58 and 916 years. Scoring tables in the
Tucson, AZ: Neuropsychology Press. manual or computer software allow for conversion of raw
scores to scaled scores (mean = 10; SD = 3) at the individ-
ual subtest level and standard scores (mean = 100; SD = 15)
at the index level. Table 1 provides a brief description of
the core subtests.
Childrens Memory Scale
M ORRIS J. C OHEN
Medical College of Georgia Historical Background
Augusta, Georgia, USA
Development of the CMS began in 1985 to provide clin-
icians with a comprehensive, well-standardized, individu-
Synonyms ally administered instrument that would assess the
important processes involved in learning and memory
CMS within the pediatric population. Prior to this, traditional
psychological evaluation of children with neurological and
neurodevelopmental disorders included tests of intelli-
gence, achievement, and behavior/emotional functioning
Description with little if any attention paid to the childs ability to learn
and remember new information. This was the case despite
The Childrens Memory Scale (CMS), published in 1997,
the fact that most referrals were in some way related to the
provides a comprehensive assessment of learning and mem-
students inability to learn and remember school-related
ory in children and adolescents of ages 5 through 16 years.
content. As a result, the CMS was developed with five goals
The CMS is individually administered and designed to be
in mind:
used as part of a standard psychological or neuropsycho-
logical evaluation. It assesses declarative learning and mem- 1. The development of an instrument that was consistent
ory functions across three domains: Auditory/Verbal, with current theoretical models of learning and memory.
Visual/Nonverbal, and Attention/Concentration (working 2. The development of an instrument that was sensitive
memory). Each domain contains two core subtests and to developmental changes over time.
one supplemental subtest. The subtests comprising the 3. To evaluate the relationship between memory and
Attention/Concentration domain provide measures of at- intelligence and provide the clinician with a mecha-
tention and working memory. Each subtest in the Auditory/ nism to meaningfully evaluate discrepancies between
Verbal and Visual/Nonverbal domains provide measures IQ and learning/memory performance.
Childrens Memory Scale C 557
Childrens Memory Scale. Table 1 Description of CMS core index and subtest components
Core
Core Index Subtest Subtest Description
Verbal Memory Indexes Stories Two stories (age-dependent 58-, 912-, and 1316-year olds) are read.
(Immediate, Delayed, and Immediately after presentation of each story, the child is asked to repeat as
C
Recognition) much of the story as can be remembered. In the delayed portion, the child
retells the stories and then answers questions about the stories (recognition
recall).
Word Pairs A list of 10 or 14 (age-dependent 58-year olds; 916-year olds) related and
unrelated word pairs are read; thereafter the stem is read and the child recalls the
associate. Three learning trials are administered followed by a free recall. In the
delayed portion, the child is asked to recall the word pairs spontaneously
followed by a recognition section.
Visual/Non-Verbal Memory Dot The child is shown an array of dots (blue) located within a rectangle in a stimulus
Indexes (Immediate and Locations book. This page is removed and the client is asked to replicate the spatial location
Delayed) of the dots by placing chips on a 3 4 or 4 4 rectangular grid (depending on
age 6 dots for 58-year olds; 8 for dots 916-year olds). Three learning trials are
administered followed by presentation of a distractor array (red dots) after which
an immediate recall trial is presented. In the delayed portion, the child is asked to
reproduce the original blue dot array.
Faces The child is presented with a series of 12 (58-year olds) or 16 (916-year olds)
pictured human faces one at a time. In the immediate and delayed recall sections,
the child is asked to identify the stimulus faces from a different set of foils (36 or
48 colored photos).
Attention/Concentration Numbers A digit span forwards and backwards task (similar to the WISC-III subtest).
Index Sequences The client is asked to mentally sequence or manipulate information as quickly as
possible. The 12 items include such tasks as reciting numbers, days of the week
and months in forward and reverse order, and counting by 2s, 4s, and 6s. Scoring
is based upon accuracy and speed.
Stories 2* Verbal
Word pairs 2* Delayed
Word lists 2**
Delayed
Recognition
Learning
Numbers* Attention/
Attention/concentration Sequences* Concentration
Picture locations**
Note: *Core subtest; **Supplemental subtest
expectancy. With the release of the WISC-IV in 2004, a With respect to validity, the results of confirmatory
smaller correlation study was conducted involving 126 chil- factor analysis yielded a three-factor solution consisting of
dren of ages 616 years (Drozdick, Holdnack, Rolfhus, & Auditory/Verbal memory, Visual/Nonverbal memory,
Weiss, 2005). The authors provide tables which allow for and Attention/Concentration. The manual also provides
ability-memory discrepancy analysis using the predicted concurrent validity studies with different measures of
actual or simple difference methods and base rates. intelligence and achievement, which consistently show a
The CMS manual provides both split-half and test- moderate positive correlation. The CMS correlations with
retest reliability coefficients for the index scores and subt- measures of executive functioning and language were low
ests. For the indexes, average split-half reliability estimates to moderate. Finally, with respect to other measures of
ranged from .76 to .91. Average split-half reliability esti- memory, moderate to high correlation were obtained
mates for the subtests ranged from .71 to .91. Test-retest across the indexes of the CMS and Wechsler Memory
reliability (mean re-test interval 59.6 days) was assessed ScaleIII, and low to moderate correlations were obtained
across three age bands using a sample of 125 students. across the indexes of the CMS and the Wide Range As-
Reliability estimates ranged from .29 to .89 for the indexes. sessment of Learning and Memory. More recently, perfor-
Due to the nature of memory assessment and psychometric mance on both the CMS and WRAML-2 was reported as
considerations (restriction of range), decision consistency low to moderate in the latter tests Examiners Manual
test-retest coefficients were also calculated. These ranged (Sheslow & Adams, 2003).
from .61 to .93 for the indexes and from .71 to .93 for the
core subtests. Results indicated a general practice effect of
up to one standard deviation, similar to what was obtained Clinical Uses
on the WISC-III. Inter-rater reliability was also assessed on
subtests requiring subjective scoring (e.g., stories) and was As previously stated, the CMS was designed to be used as
found to be high. part of a standard psychological or neuropsychological
Chlordiazepoxide C 559
Cross References
Cross References
Memory
Wechsler Memory Scale Correlation Coefficients
Wide Range Assessment of Memory and Learning
Cohen, M. J. (1992). Auditory/verbal and visual/spatial memory in chil- Castellan, N. J. Jr. (1965). On the partitioning of contingency tables.
dren with complex partial epilepsy of temporal lobe origin. Brain Psychological Bulletin, 64, 330338.
and Cognition, 20, 315326.
Cohen, M. J. (1997). Childrens memory scale. Administration manual.
San Antonio, Texas: The Psychological Corporation.
Cohen, M. J., Holmes, G. L., Campbell, R., Smith, J. R., & Flanigan, H. F.
(1990). Memory performance following unilateral electrical stimu-
lation of the hippocampus in a child with right temporal lobe
Chlordiazepoxide
epilepsy. Journal of Epilepsy, 3, 115122.
Drozdick, L. W., Holdnack, J., Rolfus, E., & Weiss, L. (2005). Technical J OHN C. C OURTNEY
report #5 WISC-IV and childrens memory scale. San Antonio, Texas: Childrens Hospital of New Orleans
Harcourt Assessment. New Orleans, LA, USA
Kibby, M. Y., & Cohen, M. J. (2008). Memory functioning in children
with reading disorders and/or attention-deficit/hyperactivity disor-
der: A clinical investigation of their working memory and long-term
memory functioning. Child Neuropsychology, 14, 525546. Generic Name
Riccio, C. A., Cash, D. L., & Cohen, M. J. (2007). Learning and memory
performance of children with specific language impairment (SLI). Chlordiazepoxide
Applied Neuropsychology, 14, 255261.
Riccio, C. A., Garland, B. H., & Cohen, M. J. (2007). Relations between
the test of variables of attention (TOVA) and the childrens memory
scale (CMS). Journal of Attention Disorders, 11, 167171.
Sheslow, D., & Adams, W. (2003). Wide range assessment of memory
Brand Name
and learning -2. Administration manual. Wilmington, DE: Jastak
Associates. Librium, Librax
560 C Chlorpromazine
Class
Chlorpromazine
Anxiolytic
J OHN C. C OURTNEY 1, C RISTY A KINS 2
1
Childrens Hospital of New Orleans
New Orleans, LA, USA
Proposed Mechanism(s) of Action 2
Mercy Family Center
Metarie, LA, USA
Chlordiazepoxide binds to the GABA-A ligand-gated
chloride channel complex and, thereby, enhances the in-
hibitory effects of GABA. Generic Name
Chlorpromazine
Indication
Bipolar disorder
Additional Information
Side Effects
Drug Interaction Effects: http://www.drugs.com/drug_interactions.html
Drug Molecule Images: http://www.worldofmolecules.com/drugs/
Free Drug Online and PDA Software: www.epocrates.com
Serious
Gene-Based Estimate of Drug interactions: http://mhc.daytondcs.
com:8080/cgi bin/ddiD4?ver=4&task=getDrugList Neuroleptic malignant syndrome, jaundice, agranulocy-
Pill Identification: http://www.drugs.com/pill_identification.html tosis, seizures
Cholesterol C 561
Cross References
Synonyms
Atherosclerosis
Fats; Lipids Cerebrovascular Disease
Coronary Disease
Ischemic Stroke
Definition Myocardial Infarction
Peripheral Vascular Disease
Cholesterol is a naturally occurring lipid found in the Stent
bloodstream and in cells throughout the body. It is an Thrombosis
important component of cell membranes and some hor-
mones. However, hypercholesterolemia, or high blood References and Readings
concentrations of cholesterol, is a major risk factor for
heart disease and stroke. Fletcher, B., Berra, K., Ades, P., Braun, L. T., Burke, L. E., Durstine, J. L.
et al. (2002). Managing abnormal blood lipids: A collaborative
approach. Circulation, 112, 31843209.
Current Knowledge National Cholesterol Education Program Expert Panel on Detection,
Evaluation, and Treatment of High Blood Cholesterol in Adults
(Adult Treatment Panel III): Third Report of the National Choles-
Cholesterol does not typically exist on its own in the blood; terol Education Program (NCEP) Expert Panel on Detection, Evalu-
it is transported to and from cells by lipoprotein molecules. ation, and Treatment of High Blood Cholesterol in Adults (Adult
Low-density lipoprotein (LDL) is the major carrier of Treatment Panel III). (2002). Circulation, 106, 31433421.
562 C Cholinergic System
working memory and the attentional processes required agent is currently being assessed as a treatment for schizo-
for error detection (see Sarter, Gehring, & Kozak, 2006 for phrenia (Lameh et al., 2007).
review). Emotional processing is also intricately related to
the cholinergic system; additionally, cholinergic inputs
to the frontoparietal cortex may influence how attention Future Directions C
is directed toward emotional information (Bentley, Vuilleu-
mier, Thiel, Driver, & Dolan, 2003). As would be expected Future research focused on the interaction between the
from a system influencing such a variety of cognitive cholinergic system and other neurotransmitters (e.g., nor-
functions, decreased cholinergic tone, associated with epinephrine, dopamine, and serotonin), and neuromodu-
Alzheimers disease, results in impaired cognitive perfor- lators (e.g., substance P) will be important in
mance broadly extending to memory, attention, and understanding the complexities that exist within the
executive functioning (Terry & Buccafusco, 2003). brain and body regarding the communication of chemical
signals. Further development of biomarkers (e.g., behav-
ioral, electrophysiological, and neuroimaging) associated
Pharmacological Agents Acting on the with cholinergic decline will allow for improved identifi-
Cholinergic System cation of disorders associated with the cholinergic system.
A continued focus on the role of the cholinergic system in
Pharmacological agents impact the cholinergic system in Alzheimers disease, as well as the development of phar-
a number of different ways. At the receptor level, nicotinic maceutical interventions targeting the cholinergic system
acetylcholine agonists stimulate cholinergic activity large- in the treatment of this disease (e.g., alpha-7 subunit
ly in peripheral acetylcholine neurons. However, nicotine, nicotinic agonists), remains critical, particularly with the
a nicotinic acetylcholine agonist acts at nicotinic receptors continued rise in the elderly population. Finally, research
in the central nervous system. Nicotinic acetylcholine on the role of the cholinergic system in a number of
antagonists acting on cells in the central nervous system psychological disorders, including stress, affective and
are rarely used in clinical practice. panic disorders, and schizophrenia, will continue to high-
Pilocarpine is an example of a muscarinic acetylcho- light the broad implications on mental health that this
line agonist that acts on the parasympathetic nervous system has.
system. While pilocarpine is not used clinically to treat
central nervous system disorders, it has a range of side
effects, including excessive sweating and salivation, which Cross References
are related to its action on muscarinic receptors in the
parasympathetic nervous system. Muscarinic acetylcho- Alzheimers Disease
line antagonists, such as scopolamine and atropine, block Cholinesterase Inhibitors
muscarinic receptors. Their anticholinergic effects on the Memory
parasympathetic nervous system result in side effects such Memory Impairment
as dry mouth, constipation, and tachycardia. Mild Cognitive Impairment
Acetylcholinesterase inhibitors, such as tacrine and
donepezil, block the breakdown of acetylcholine in the
synaptic cleft. The result is sustained levels of acetylcho- References and Readings
line in the synapse that are capable of transmitting
chemical information to other cells. Acetylcholinester- Bentley, P., Vuilleumier, P., Thiel, C., Driver, J., & Dolan, R. (2003).
ase inhibitors are commonly used in the treatment of Cholinergic enhancement modulates neural correlates of selective
attention and emotional processing. Neuroimage, 20, 5870.
Alzheimers disease, as well as Lewy Body dementia.
Bodick, N. C., Offen, W. W., Levey, A. I., Cutler, N. R., Gauthier, S. G.,
Presently, there is a developing focus on the choliner- Satlin, A., et al. (1997). Effects of xanomeline, a selective muscarinic
gic system in the treatment of schizophrenia. Xanomeline, receptor agonist, on cognitive function and behavioral symptoms in
an M1/M4 muscarinic acetylcholine agonist that was Alzheimer disease. Archives of Neurology, 54, 465473.
initially assessed as a possible treatment for Alzheimers Lameh, J., Burstein, E. S., Taylor, E., Weiner, D. M., Vanover, K. E., &
Bonhaus, D. W. (2007). Pharmacology of N-desmethylclozapine.
disease, has shown efficacy in treating psychotic
Pharmacology & Therapeutics, 115, 223231.
symptoms (Bodick et al., 1997). In addition, N-des- Sarter, M., Givens, B., & Bruno, J. P. (2001). The cognitive neuroscience of
methylclozapine (norclozapine, NDMC), a metabolite of sustained attention: Where top-down meets bottom-up. Brain Re-
the antipsychotic clozapine, is a partial M1 agonist. This search Reviews, 35, 146160.
564 C Cholinesterase Inhibitors
Sarter, M., Gehring, W. J., & Kozak, R. (2006). More attention must be
paid: the neurobiology of attentional effort. Brain Research Reviews, Chorea
51, 145160.
Terry, A., & Buccafusco, J. (2003). The cholinergic hypothesis of age
A NNA D E P OLD H OHLER 1, M ARCUS P ONCE DE LEON2
and Alzheimers disease-related cognitive deficits: recent challenges 1
and their implications for novel drug development. Journal of Phar- Boston University Medical Center
macology and Experimental Therapeutics, 306, 821827. Boston, MA, USA
2
William Beaumont Army Medical Center
El Paso, TX, USA
Cholinesterase Inhibitors
Synonyms
J OA NN T. T SCHANZ
Utah State University Dance-like
Logan, UT, USA
Synonyms Definition
Acetylcholinesterase inhibitors; AchEIs; Anticholinester-
Chorea is characterized by brief, irregular muscle
ase inhibitors; CHEIs
contractions that are not repetitive or rhythmic,
but appear to flow from one muscle to the next.
Definition They may appear as dance-like movements of the
limbs, trunk, or head. Typical movements include facial
Cholinesterase inhibitors are a class of medications used in grimacing, shoulder adduction, and finger extension
the treatment of Alzheimers disease (AD) that targets the and contractions. They can be associated with snakelike
cholinergic neurotransmitter system. The treatment is writhing movements of the hands or feet known as
based on the observation of a cholinergic deficiency in athetosis.
AD. Cholinesterase inhibitors block the activity of acetyl-
cholinesterase, the primary enzyme that breaks down ace-
tylcholine, and allows the neurotransmitter substance to
remain in the synaptic cleft longer in order to stimulate Current Knowledge
postsynaptic receptors. These drugs do not prevent the
further degeneration of cholinergic neurons. They are mod- Chorea is a feature of Huntingtons disease, and may be
estly effective in the short-term improvement of attention, present with rheumatic fever. It can be seen as a side effect
concentration, memory, and some behavioural symptoms, of the medication levodopa or the dopamine agonists and
but are not thought to alter the progression of the disease. may result from metabolic disorders, endocrine disorders,
and vascular incidents.
Cross References
A NTHONY Y. S TRINGER
Emory University Education and Training C
Atlanta, GA, USA
19541955 Visiting research fellow at Radcliffe
University
Landmark Clinical, Scientific, and 1956 Master of Arts in Psychology, University of
Professional Contributions Copenhagen, Denmark
1957 Doctor of Philosophy, University of Copenha-
In 1966, Anne-Lise Christensen reviewed a newly gen, Denmark
published English translation of Aleksandr Lurias
Christensen began attending the University of Copenha-
(1966) Higher Cortical Functions in Man and instantly
gen in 1945, shortly after the liberation of Denmark from
realized its value in the clinical bedside examination of
the Nazi occupation. She writes about the overwhelming
cognitive function in neurological patients. Luria
experience of freedom and peace . . . [when] the borders
provided a comprehensive theory of the brain organi-
were opened, and we were full of hope and expectations
zation, from which cognitive tasks could be developed
for the future (Christensen, 2002, p. 119). Her studies
to measure various cortical functions. However, Lur-
were interrupted for 6 years by marriage and mother-
ias scholarly presentation required some adaptation
hood, but she returned to the university in 1952, dividing
before it could be practically applied by clinicians.
her time between literature and psychology. Christensen
Christensen began to translate Lurias methods into
was accepted at Radcliffe University in 1954, where she was
her native Danish as well as adapt them for use in the
exposed to the work of Talcott Parsons, Gordon Allport,
clinic. Responding to Lurias invitation, Christensen
Gardner Lindzey, and George Mandler. She cites, Jerome S.
visited him in Moscow and presented her Danish
Bruner, however, as her greatest influence. In his work,
translation. Luria labeled it a vulgarization of his
Christensen was exposed to New Look Psychology
method, but added he had always wanted someone
which took the methods of the psychophysics laboratory
to do this. He encouraged Christensen to do an En-
and applied them to everyday perceptual experience.
glish translation. Five years later, Christensen pre-
Christensen returned to Denmark in 1955 and
sented the first English draft to Luria on her second
completed her doctoral studies at the University of
visit to Moscow. Luria made edits and provided a
Copenhagen in 1957. Bruner continued to influence
paper for Christensen to translate and include in the
Christensens career for more than a decade later when
final draft. Christensen published Lurias Neuropsy-
he insisted she introduced herself to Luria at a 1969
chological Investigation (LNI) in 1975, including a
conference in London. Impressed with her attempts at
textbook, manual, and stimulus cards.
using his method in Denmark, Luria invited Christensen
The LNI introduced a qualitative approach to neuro-
to visit him in Moscow, but a sudden heart attack pre-
psychological examination, which contrasted with the
vented his participation in the conference. Nonetheless,
predominant quantitative batteries used in the USA.
an official invitation arrived in Denmark, and Christensen
The LNI is one of the few theory-based assessment
made her visit to the Bourdenko Neurosurgical University
approaches in neuropsychology and has been lauded
Institute to work with Luria in September 1970 (Chris-
for its flexible administration, brevity, and qualitative
tensen, 2002). This began the collaboration between
focus (Kolb & Wishaw, 1990). It has also been criti-
the two, which resulted in the introduction of the LNI
cized for its lack of norms and insensitivity to mild
to the West.
impairment (Lezak, 1983). Though Christensen de-
clined to collaborate, Charles Golden and colleagues
(Golden, Purisch, & Hammeke, 1979) in the USA Major Appointments
attempted to standardize the administration of the
LNI and establish a normative base for interpretation. University Hospital of Aarhus (Psychiatry and Neuro-
This unfortunately violated the original qualitative surgery Departments), Jutland, Denmark, 19591968
focus of the LNI and generated considerable contro- University Hospital of Aarhus (Head of Clinical Psy-
versy and criticism. Nonetheless, Christensens original chological Department), Jutland, Denmark, 19691981
566 C Chromosome
Cross References defined by the Centers for Disease Control (CDC) study
group (Holmes et al., 1988); they named the condition
Deoxyribonucleic Acid (DNA) Chronic Fatigue Syndrome (CFS). In subsequent years,
Gene case definitions for CFS were created in Australia, Great
Britain (Sharpe et al., 1991; Wessely, 1995), and Canada C
(Carruthers et al., 2003), and a pediatric case definition
for ME/CFS was published in 2006 (Jason et al., 2006).
Chronic Bronchitis Currently, the most common case definition used in the
United States for the clinical diagnosis of CFS is a version
Chronic Obstructive Pulmonary Disease of the original 1988 CDC case definition revised in 1994
by an International CFS Study Group (Fukuda, Straus,
Hickie, Sharpe, Dobbins, & Komaroff, 1994).
The diagnosis of CFS cannot be made if the following
are present and could account for the presence of persis-
Chronic Familial Vascular tent fatigue: (1) permanent medical exclusions include
Encephalopathy organ failure, chronic infections, rheumatic and chronic
inflammatory diseases, major neurologic diseases requir-
Cadasil ing systemic treatment, major endocrine diseases, and
primary sleep disorders; (2) temporary medical exclu-
sions include treatable conditions that require evaluation
over time to determine the extent to which they contrib-
Chronic Fatigue Immune ute to the fatiguing illness such as conditions discovered
at onset or initial evaluation (e.g., effects of medications,
Deficiency Syndrome (CFIDS) sleep deprivation, untreated hypothyroidism, untreated
or unstable diabetes mellitus, active infection), conditions
Chronic Fatigue Syndrome that resolve (e.g., pregnancy), cardiac conditions, and
morbid obesity specified as BMI > 45; 3) permanent
psychiatric exclusions include lifetime diagnoses of bipo-
lar affective disorders, schizophrenia of any subtype, de-
Chronic Fatigue Syndrome lusional disorders of any subtype, dementias of any
subtype, organic brain disorders, and alcohol or substance
G UDRUN L ANGE abuse within 2 years before onset of the fatiguing illness,
UMDNJ-New Jersey Medical School any past or current diagnosis of major depressive disorder
Newark, NJ with psychotic or melancholic features, anorexia nervosa,
or bulimia.
If none of the exclusions apply, using the 1994 case
Synonyms definition, CFS is diagnosed based on the following cri-
teria: (1) fatigue is of new or definite onset, not due to
Chronic Fatigue Immune Deficiency Syndrome (CFIDS); exertion, not relieved by rest, and must result in substan-
Medically unexplained symptoms (MUS); Myalgic en- tial reductions in previous levels of educational, occupa-
cephalitis (ME); Post-infectious fatigue syndrome tional, social, or personal activities, (2) fatigue is of at least
(PFIS). Nomenclature used by patients or healthcare pro- 6 months duration, and (3) concurrent with at least four
viders is generally based on their etiologic perspective of the following eight symptoms that could not have
predated the onset of fatigue: impairment in short-term
memory or concentration severe enough to result in
Short Description or Definition substantial reductions in previous levels of educational,
occupational, social, or personal activities, painful lymph
Chronic fatigue syndrome is a complex illness defined by nodes in front or back of the neck or under the arms, sore
unexplained disabling fatigue and a combination of non- throat, muscle pain, pain in more than one joint without
specific accompanying symptoms that can have sudden or accompanying redness or swelling, headaches of a new
gradual onset. The diagnosis of the condition was initially type, unrefreshing sleep, postexertional malaise lasting
568 C Chronic Fatigue Syndrome
more than 24 h. These symptoms are nonspecific and dysfunction, cortisol dysregulation, small white matter
variable in both nature and severity over time. They lesions in the frontal regions of the brain, orthostatic
were selected on the basis of consensus clinical opinion and cognitive dysfunction. Most abnormalities are found
and were not identified empirically. in CFS patients who do not suffer from comorbid psychi-
atric disorder, most commonly depression. Treatments,
especially cognitive behavioral and graded exercise treat-
Categorization ments, enhance the prognosis for improvement. If un-
treated, complete recovery from CFS is rare.
Only a small percentage of patients complaining of fatigue
will be categorized as having CFS. Most patients either have
prolonged fatigue, defined as self-reported, persistent fa- Neuropsychology and Psychology of
tigue of 1 month or longer, or chronic fatigue defined as Chronic Fatigue Syndrome
self-reported persistent or relapsing fatigue of 6 or more
consecutive months (Fukuda et al., 1994). Other conditions CFS patients typically complain of difficulties with concen-
of unexplained etiology with similar symptom profiles tration, memory, and thinking, yet neuropsychological
are often comorbid with CFS and can include Fibromyalgia testing does not generally confirm the reported cognitive
Syndrome (FMS), Temporo-Mandibular Syndrome dysfunction. Available data suggest that the main cognitive
(TMS), Irritable Bowel Syndrome (IBS), Multiple Chemi- deficit in individuals with CFS is slowed information pro-
cal Sensitivity (MCS), Gulf War Syndrome (GWS), Major cessing, which can affect memory as well as executive
Depressive Disorder (MDD), and anxiety disorders. function. Depression is a very common comorbid condi-
tion (Tiersky, Johnson, Lange, Natelson, & DeLuca, 1997).
Epidemiology Neuroimaging data increasingly provide evidence for de-
creased cerebral blood flow and functional activation of
In the United States, CFS occurs in up to about 0.5% of brain areas suggesting increased cognitive effort (Lange,
the general population. It is most commonly found in Wang, Deluca, & Natelson, 1998; Lange et al., 2005).
middle-aged women and is most common in Latinos,
followed by African Americans, and Whites. The illness
affects women (predominantly between the ages of 40 Evaluation
and 59) more often than men (Reyes et al., 2003). In
general, the expression of the syndrome is not gender- A neuropsychological testing battery for individuals with
specific (Buchwald, Pearlman, Kith, & Schmaling, 1994). CFS should include measures of overall current and pre-
Chronic Fatigue Syndrome can also occur in children and morbid cognitive function (i.e., Wechsler Test of Adult
adolescents. While gender distribution is similar to that of Reading, Wechsler Adult Intelligence Scale III/IV), simple
adults, prevalence rates are significantly lower. and complex attention as well as information processing
and working memory (i.e., Continuous Performance
Test, Gordon, Trails, Paced Auditory Serial Addition
Natural History, Prognostic Factors, Test), executive function (i.e., subtests of DelisKaplan
Outcomes Executive Function System including verbal fluency,
towers test; Wisconsin Card Sort Test), memory
Disorders with similar symptom profiles have been des- (i.e., Wechsler Memory Scale III/IV, California Verbal
cribed for at least two centuries and have been known Learning Test II, Rey Osterrieth Complex Figure Test),
under a variety of names including neurasthenia, language function (i.e., Boston Naming Test), visual
Akureyri disease, Epstein Barr Syndrome, and chronic perceptual function (i.e., Judgment of Line Orientation,
mononucleosis. Although many hypotheses exist about Hooper), and motor function (i.e., grip strength, finger
the causes for CFS, the etiology of the condition is still tapping, pegboard). It is also recommended to test the
unclear. Some believe that CFS is a latent form of depres- level of motivation and effort expanded during neuropsy-
sion and anxiety disorder, while others view the syndrome chological testing as well as emotional functioning to
as a sleep disorder, attribute it to endocrine dysfunction improve interpretability of test results.
or abnormalities within the central nervous system When an individual is diagnosed with CFS, it may be
(CNS). Abnormalities identified in individuals with desirable to evaluate the intensity and severity of symp-
CFS include HypothalamicPituitaryAdrenal Axis toms associated with CFS using self-report
Chronic Fatigue Syndrome C 569
questionnaires. To assess fatigue intensity, several mea- Dietrich, & Oxman, 2000). Medication management
sures have been used including the Chalder Fatigue Scale of fatigue is in its infancy; presently, there is no FDA
and the Krupp Fatigue Scale; both have acceptable psy- approved drug for fatigue. Over the counter medications
chometric properties. The Chalder Fatigue Scale is a 14- offer a first line of therapeutic possibilities. Stronger med-
item instrument with a 4-choice format and separates ications, including stimulants and antidepressants, re- C
mental and physical fatigue (Chalder et al., 1993). The quire a physicians prescription. In general, medications
Krupp Fatigue Severity Scale includes nine items rated on used to treat depression, anxiety, and pain are very often
7-point scales and is sensitive to different aspects and used for the pharmacologic treatment of CFS. It is impor-
gradations of fatigue severity. Most items in the Krupp tant to recognize that fatigue is not solely due to disease
scale are related to behavioral consequences of fatigue processes, but can occur as an indirect consequence of
(Krupp, LaRocca, Muir-Nash, & Steinberg, 1989). The decreased physical activity and conditioning. Graded aer-
Checklist Individual Strength (CIS) is a 20-item inventory obic exercise training is a safe and effective treatment for
with four subscales commonly used to measure fatigue CFS and has been shown to improve quality of life. The
severity, concentration, reduced motivation, and physical primary goal of exercise is to avoid the spiral of decon-
activity. The CIS focuses on fatigue over the preceding ditioning that is common in most fatiguing diseases.
2 weeks (Vercoulen, Swanink, Fennis, Galama, Van der
Meer, & Bleijenberg, 1994). Another commonly used in-
strument to measure fatigue severity is the Multidimen- Cross References
sional Fatigue Inventory, also a 20-item questionnaire
providing scores for severity of general fatigue, physical Epstein Barr Syndrome
fatigue, mental fatigue, as well as reduced motivation and Gulf War Syndrome
activity (Smets, Garssen, Bonke, & De Haes, 1995). Neurasthenia
Another symptom that might warrant additional in- Unexplained Illness
vestigation is pain. Five of the eight CFS-defining symp-
toms reflect pain (headaches of a new type, pattern, or
severity, muscle pain, and multi-joint pain without
References and Readings
swelling or redness, sore throat, tender cervical/axillary
lymph nodes). In many cases, pain may be the primary
Buchwald, D., Pearlman, T., Kith, P., & Schmaling, K. (1994). Gender
determinant of disability for some individuals with CFS. differences in patients with chronic fatigue syndrome. Journal of
The McGill Pain Questionnaire (MPQ) is a well-validated General International Medicine, 9, 397401.
questionnaire that can be used to further characterize Chalder, T., Berelowitz, G., Pawlikowska, T., Watts, L., Wessely, S., Wright,
pain or follow the course of pain in CFS (Melzack, 1975). D., et al. (1993). Development of a fatigue scale. Journal of Psychoso-
matic Research, 37, 147153.
The MPQ includes four components: (1) a human figure
Carruthers, B. M., Jain, A. K., De Meirleir, K. L., Peterson, D. L., Klimas,
drawing on which patients are asked to mark the location N. G., Lerner, A. M. et al. (2003). Myalgic Encephalomyelitis/
of their pain; (2) a series of adjectives divided into groups Chronic Fatigue Syndrome: clinical Working Case Definition,
from which patients identify their experience by circling Deagnostic and Treatment Protocols. Journal of Chronic Fatigue
word descriptors; (3) questions about prior pain experi- Syndrome, 11, 7115.
DeLuca, J. (2008). Fatigue as a window to the brain. London, UK: MIT
ence, pain location, and information on the use of pain
Press.
medication; and (4) a present pain intensity index. Fukuda, K., Straus, S. E., Hickie, I., Sharpe, M. C., Dobbins, J. G., &
Komaroff, A. (1994). The chronic fatigue syndrome: a comprehensive
approach to its definition and study. International Chronic Fatigue
Treatment Syndrome Study Group. Annals of Internal Medicine, 121, 953959.
Holmes, G. P., Kaplan, J. E., Gantz, N. M., Komaroff, A. L., Schonberger,
L. B., Straus, S. E., et al. (1988). Chronic fatigue syndrome: a working
Effective treatment needs to be tailored to each individual case definition. Annals of Internal Medicine, 108, 387389.
diagnosed with CFS and often consists of a combination Jason, L. A., Bell, D. S., Rowe, M. D., van Hoof, E. L. S. J. K., Lapp, C.,
of behavioral, pharmacological, and physical interven- et al. (2006). A pediatric case definition for myalgic encephalomyeli-
tions. Behavior modification or cognitive restructuring tis and chronic fatigue syndrome. Journal of Chronic Fatigue Syn-
drome, 13, 144.
are two cognitive behavioral therapy (CBT) approaches
Kroenke, K., Taylor-Vaisey, A., Dietrich, A. J., & Oxman, T. E. (2000).
used to treat CFS. Using this modality, reductions in Interventions to improve provider diagnosis and treatment of men-
the effect of fatigue on functional ability and quality of tal disorders in primary care. A critical review of the literature.
life have been shown in CFS (Kroenke, Taylor-Vaisey, Psychosomatics, 41, 3952.
570 C Chronic Multisymptom Illnesses
Krupp, L. B., LaRocca, N. G., Muir-Nash, J., & Steinberg, A. D. (1989). Short Description or Definition
The fatigue severity scale. Application to patients with multiple
sclerosis and systemic lupus erythematosus. Archives of Neurology,
46, 11211123.
The Global Initiative for Chronic Obstructive Lung Disease
Lange, G., Steffener, J., Cook, D. B., Bly, B. M., Christodoulou, C., Liu, (GOLD) Workshop defines COPD as a disease state char-
W. C., et al. (2005). Objective evidence of cognitive complaints in acterized by airflow limitation that is not fully reversible.
Chronic Fatigue Syndrome: a BOLD fMRI study of verbal working The airflow limitation is usually both progressive and asso-
memory. Neuroimage, 26, 513524.
ciated with an abnormal inflammatory response of the
Lange, G., Wang, S., Deluca, J., & Natelson, B. H. (1998). Neuroimaging
in chronic fatigue syndrome. American Journal of Medicine, 105,
lungs to noxious particles or gases. The GOLD committee
50S53S. was organized by the World Health Organization (WHO)
Melzack, R. (1975). The McGill pain questionnaire: major properties and and the US National Heart Lung and Blood Institute
scoring methods. Pain, 1, 277299. (NHLBI). Additional information about definitions, diag-
Reyes, M., Nisenbaum, R., Hoaglin, D. C., Unger, E. R., Emmons, C.,
nosis, treatment, and research can be found at www.gold
Randall, B., et al. (2003). Prevalence and incidence of chronic fatigue
syndrome in Wichita, Kansas. Archives of Internal Medicine, 163,
copd.com.
15301536.
Sharpe, M. C., Archard, L. C., Banatvala, J. E., Borysiewicz, L. K., Clare,
A. W., David, A., et al. (1991). A reportchronic fatigue syndrome:
guidelines for research. Journal of the Royal Society of Medicine, 84,
Categorization
118121.
Smets, E. M., Garssen, B., Bonke, B., & De Haes, J. C. (1995). The
Multidimensional Fatigue Inventory (MFI) psychometric qualities COPD has traditionally been defined by severity of airflow
of an instrument to assess fatigue. Journal of Psychosomatic Research, limitation. Indeed, the GOLD COPD classification system
39, 315325. relies on measures of airflow limitation to stratify COPD
Tiersky, L. A., Johnson, S. K., Lange, G., Natelson, B. H., & DeLuca, J.
severity (i.e., Forced Expiratory Volume in 1 Second (FEV1)
(1997). Neuropsychology of chronic fatigue syndrome: a critical
review. Journal of Clinical and Experimental Neuropsychology, 19,
and Forced Expiratory Volume in 1 Second/Forced Vital
560586. Capacity(FEV1/FVC ratio)). This staging system is cur-
Vercoulen, J. H., Swanink, C. M., Fennis, J. F., Galama, J. M., Van der rently the primary determinant of treatment guidelines.
Meer, J. W., & Bleijenberg, G. (1994). Dimensional assessment of Heterogenity in the pathophysiology underlying COPD
chronic fatigue syndrome. Journal of Psychosomatic Research, 38,
has been increasingly recognized over the past decade
383392.
Wessely, S. (1995). The epidemiology of chronic fatigue syndrome.
including chronic bronchitis, peripheral airways disease,
Epidemiological Review, 17, 139151. and emphysema (Friedlander, Lynch, Dyar, & Bowler,
2007). At times, asthma has also been included in group-
ing of patients with COPD.
Natural History, Prognostic Factors, in daily activity, increased disease severity, and impaired
Outcomes health status (Hopkins & Bigler, 2001; McSweeny &
Labuhn, 1996).
Risk factors for COPD include both environmental and
host-related variables. Cigarette smoke has been identified C
as the most important external risk factor, followed by pipe
Evaluation
and cigar smoke, occupational dusts and chemicals, history
of severe childhood respiratory infections, HIV infection,
Neuropsychological evaluation for cognitive problems
outdoor pollution, and IV drug use. Host-related factors
associated with COPD typically involves measurement of
include airways hyper-responsiveness, genetic factors, se-
attention, learning and memory, reasoning and executive
vere hereditary alpha1-antitrypsin deficiency, low birth
functioning skills, visuomotor speed, and visuoperceptual
weight, and maternal cigarette smoking during gestation
function. Psychological evaluation typically involves mea-
(Anthonisen & Manfred, 2004).
sures of depression, anxiety, and health-related quality
of life.
Neuropsychology and Psychology of
COPD
Treatment
Empirical studies have documented neuropsychological
(i.e., cognitive) deficits in patients with chronic airway Research to date suggests that a variety of therapeuticap-
obstruction and COPD. Two of the largest studies proaches utilized in COPD patients (including oxygen
conducted in the 1980s were the Nocturnal Oxygen therapy, comprehensive rehabilitation programs, and
Therapy Trial (NOTT) and the Intermittent Positive surgical techniques) improve psychological and cognitive
Pressure Breathing Trial (Prigatano & Levin, 1988). The functioning. Long-term (greater than 6 months) use of
pattern and extent of cognitive dysfunction reported oxygen therapy improves cognitive performance in
in COPD vary across patients and appear to be associated COPD, probably due to direct effects of improved oxygen
with disease severity. In COPD patients with moderate delivery to the central nervous system (Prigatano & Levin,
to severe hypoxemia, deficits have been identified in sim- 1988). Early studies reported improvement in visual
ple motor movement and overall strength, perceptual- memory, verbal memory, and motor speed among the
motor integration, abstract reasoning, attention to COPD subjects following 6 months of continuous oxygen
auditory stimuli, learning and memory, and language therapy. Large multisite studies have also demonstrated
skills. In patients with mild hypoxemia, impairments in benefits of oxygen for cognitive function in COPD.
higher cerebral functioning include abstract reasoning, For example, in the Nocturnal Oxygen Therapy Trial,
auditory and visual attention, verbal and nonverbal COPD patients receiving continuous oxygen therapy for
learning and recall, and reasoning and motor skills 12 months experienced greater improvements in cognitive
(Kozora et al., 2008). There is some evidence that cogni- performance than did patients receiving only nocturnal
tive impairment has an independent impact on patients oxygen therapy.
daily functioning, medical regimen adherence, and quali- There is also evidence suggesting that comprehensive
ty of life, although results have been mixed (McSweeny & multidisciplinary rehabilitation programs can improve
Labuhn, 1996). cognitive functioning and psychological status in emphy-
Psychological changes and emotional distress have also sema patients. Comprehensive rehabilitation programs
been noted in COPD patients. To date, depression and for treatment of COPD are well established and typically
anxiety are the most commonly observed psychological include assessment, education, instruction on respiration,
problems in COPD (Hynninen, Breitve, Wiborg, Pallesen, & psychosocial support, and exercise training with the
Nordhus, 2005) with estimates of the prevalence of dep- goal of restoring patients to the highest level of indepen-
ression ranging from approximately 25% to 50%. Some of dent function (Make, 2004). As noted in a recent review
the discrepancies in prevalence estimates across studies article (Kozora et al., 2008), Emery and colleagues first
may be related to differences in the method used to assess reported in 1991 improved complex attention in COPD
depression. In addition to emotional distress, multiple patients following a 30-day exercise rehabilitation pro-
studies have demonstrated poor quality of life in patients gram that included instructional/educational compo-
with COPD, which has been associated with restrictions nents, psychosocial counseling, and stress reduction. In a
572 C Chronic Organic Brain Syndrome
subsequent study published in 1998, they reported im- J. B. Karlinsky, & T. E. King (Eds.), Baums textbook of pulmonary
diseases (7th ed.). Philadelphia: Lippincott, Williams & Wilkins.
provement in verbal fluency and reduction in symptoms
Friedlander, A. L., Lynch, D., Dyar, L. A., & Bowler, R. P. (2007). Pheno-
of anxiety and depression in a group participating in types of chronic obstructive pulmonary disease. COPD: Journal of
exercise, stress reduction, and education programs when Chronic Obstructive Pulmonary Disease, 4(4), 355384.
compared to a control group participating in stress reduc- Hopkins, R. O., & Bigler, E. D. (2001). Pulmonary disorders. In R. E.
tion and education treatment only. This finding high- Tarter, M. Butters, & S. R. Beers (Eds.), Medical neuropsychology
(2nd ed., pp. 247266). New York: Kluwer/Plenum.
lighted the utility of the exercise component toward
Hynninen, K. M., Breitve, M. H., Wiborg, A. B., Pallesen, S., & Nordhus,
improved cognitive and psychological functions. In I. H. (2005). Psychological characteristics of patients with chronic
2002, Kozora and colleagues also reported improvement obstructive pulmonary disease: A review. Journal of Psychosomatic
in visual attention and semantic fluency, among COPD Research, 59(6), 429443.
patients following a 3-week comprehensive rehabilitation Kozora, E., Emery, C., Kaplan, R. M., Wamboldt, F. S., Zhang, L., & Make,
B. J. (2008). Cognitive and psychological issues in emphysema.
program when compared to untreated COPD and
Proceedings of the ATS, 5(4), 556560.
healthy control subjects similar in age, education, and Make, B. (2004). Pulmonary rehabilitation. In J. D. Crapo, J. Glassroth,
gender. This program included exercise, educational, in- J. B. Karlinsky, & T. E. King (Eds.), Baums textbook of pulmonary
structional, and psychosocial components. In addition to diseases (7th ed., pp. 289307). Philadelphia: Lippincott Williams
cognitive changes, significant improvement in depressive and Wilkins.
McSweeny, A. J., & Labuhn, K. T. (1996). The relationship of neuropsy-
symptoms was reported. Together, these studies indicate
chological functioning to health-related quality of life in systemic
improved cognitive performance and psychological medical disease: The example of chronic obstructive pulmonary
status following comprehensive rehabilitation with an disease. In I. Grant & K. M. Adams (Eds.), Neuropsychological assess-
exercise component. Many studies have also reported ment of neuropsychiatric disorders (pp. 577602). New York: Oxford
increased quality of life following comprehensive reha- University Press.
Prigatano, G. P., & Levin, D. C. (1988). Pulmonary system. In R. E. Tarter,
bilitation (Make, 2004).
D. H. Van Theiel, & K. L. Edwards (Eds.), Medical neuropsychology
Finally, studies have suggested some improvement in (pp. 1126). New York: Plenum.
cognition for moderate to severe COPD patients follow-
ing lung volume reduction surgery (LVRS). In an ancillary
study of the National Emphysema Treatment Trial
(NETT), Kozora and colleagues (Kozora et al., 2008)
examined neuropsychological and psychological func-
Chronic Organic Brain Syndrome
tioning of patients receiving LVRS when compared to
Organic Brain Syndrome
patients receiving only medical therapy (MT). The LVRS
group showed significant improvement compared to the
MT group at 6 months on a measure of psychomotor
speed, delayed recall for verbal information, and trends
toward improved sequential thinking, and psychomotor Chronic Progressive Multiple
speed and naming to confrontation. LVRS patients Sclerosis
also experienced significant reduction in depression at
6 months, as reflected in total Beck Depression Inventory Primary Progressive Multiple Sclerosis
score. There was no direct evidence that improved cogni-
tion in LVRS was related to improved physical capacity
(workload and 6 min walk) or pulmonary function.
Chronic Widespread Pain
Cross References Disorder
Fibromyalgia
Anoxia
centers to produce the affective motivation necessary for nucleus of the vagus, and the nucleus accumbens/olfacto-
the organisms engagement in the environment. The pos- ry tubercle of the ventral striatum.
terior sensory processing region (areas 23ab and 29/30) Brain areas that have reciprocal connections with the
assists in the memory and processing of environmental visceral effector region, except the claustrum that supplies
stimuli targeted as relevant to the organism based on their auditory input, also influence visceral function when sti-
motivational valence. The following sections will explore mulated. This results from their amygdalar connections
the connections of these four cingulate regions (Figs. 2 that convey the visceral state of the organism to paralimbic
and 3) and their function as observed from electrical areas. The orbitofrontal cortex mediates empathic, civil,
stimulation, imaging, and clinical data. and socially appropriate behavior. Rostral auditory associ-
ation cortex in the superior temporal area also provides
auditory information to the visceral effector region. No
Function visual information has direct access to the subcallosal area.
The dorsolateral prefrontal lobe, functioning as an
Visceral effector region. Major reciprocal connections with executive processor, provides nonreciprocal input
the visceral effector region are with the basal and accesso- from areas 9 and 46 to the subcallosal anterior cingulate
ry basal amygdala, medial orbitofrontal cortex areas 11, region. Executive functions permit an organized behav-
12, and 13, anterior superior temporal pole area 38, and ioral response to solve a complex problem. This includes
the anterior ventral claustrum. Major nonreciprocal pro- the activation of remote memories, self-direction, and
jections from the visceral effector region target the para- independence from environmental contingencies, shifting
sympathetic nucleus of the solitary tract, the sympathetic and maintaining behavioral sets appropriately, generating
thoracic intermediolateral column, the dorsal motor motor programs, and using verbal skills to guide behavior.
Dorsolateral prefrontal efferents into the subcallosal cin-
gulate provide feedback inhibition on the basic drives of
hunger, aggression, and reproductive urges. Table 1 for a
summary of these anatomic connections.
Subcallosal cingulate processing modulates visceral
output from brain-stem sympathetic and parasympathetic
centers. Access to the basal ganglia, via the ventral striatum,
allows processing of the internal milieu of the organism to
influence the skeletal motor system as well. This visceral
motor network encompasses the bulk of the orbitofrontal-
centered paralimbic belt dedicated to assessing the emo-
Cingulate Gyrus. Figure 2 Cytoarchitectonic divisions of the tional valence of objects based upon internal motivating
cingulate and adjacent areas drives. Patients with lesions in this area are often
disinhibited with irritability, lability, tactlessness, and fatu- reticular activating system and its control over arousal.
ous euphoria. Patients act upon visceral drives without Table 2 for a summary of these anatomic connections.
regard to social decorum. Skeletomotor effector region. Major reciprocal connections
Cognitive effector region. Major reciprocal connections with the skeletomotor effector region are with the primary
with the cognitive effector region are with the basal amyg- and supplementary/premotor motor cortex areas 4 and 6, C
dala, prefrontal areas 8, 9, 10, and 46, caudal orbitofrontal prefrontal areas 8, 9, and 46, parietal areas 1, 2, 3a, 5, and
area 12, inferior temporal pole area 38, rostral insula, 7b, and caudal insula. Major nonreciprocal projections
anterior parahippocampal areas 35 and 36, and the ante- from the skeletomotor area target the lateral putamen,
rior medial claustrum. Areas reciprocally connected to the spinal cord, ventromedial parvocellular division of the red
cognitive effector region share a general similarity with nucleus, and the ventrolateral pontine gray matter.
the subcallosal anterior region underscoring their com- Primary motor cortex has very limited input. The me-
mon membership in the orbitofrontal paralimbic belt. dial supplementary, lateral premotor, and cingulate skeletal
The cognitive effector region is more developed in its motor regions are the only forebrain inputs to the primary
cytoarchitecture, than the subcallosal cingulate, and thus motor cortex. The skeletomotor region in the banks of the
has stronger connections with more phylogenetically re- cingulate sulcus conveys limbic influence to the medial
cent neocortex of dorsolateral prefrontal areas 8, 9, 10, supplementary, lateral premotor, and primary motor cor-
and 46 devoted to executive function. The amygdala pro- tex. Frontal eye fields in area 8 also share reciprocal connec-
vides internal affective input to the supracallosal anterior tions with the skeletal motor effector region. Areas 9 and 46
cingulate. The distribution of amygdala efferents deline- of the dorsolateral prefrontal cortex contribute executive
ates the dorsal boundary of the cingulate as a functional input to the limbic motor system. Thus, executive and
system. Auditory input arises from the anterior medial limbic systems gain access to primary motor area 4 indi-
claustrum as well as a minor link with the auditory asso- rectly. The cingulate skeletal motor region receives the
ciation area of the superior temporal gyrus. The rostral greatest outflow from executive prefrontal cortex than
insula and anterior parahippocampal areas provide addi- all other motor cortices underscoring its influence over
tional reciprocal connections with the cognitive effector goal-directed behavior. Sensory-motor parietal areas 1, 2,
region not associated with the subcallosal region. Rostral 3a, and 5 also have reciprocal connections with the skeletal
insular cortex is a transitional paralimbic region that motor center within the banks of the cingulate sulcus. The
integrates visceral alimentary input with olfactory and rostral parietal area 7b has a strong relationship with the
gustatory afferents. Connections with the anterior para- premotor cortex, while the granular cortex of the caudal
hippocampal areas 35 and 36 allow the supracallosal cin-
gulate to influence multimodal sensory afferents entering
the hippocampus. Cingulate Gyrus. Table 2 Major reciprocal connections and
Major nonreciprocal projections of the supracallosal nonreciprocal targets for the cognitive effector region of the
anterior cingulate include the auditory association cortex cingulate cortex
of anterior superior temporal area 22, allowing the cognitive
effector region to influence language and the access of Reciprocal connections
semantic stores. The posterior parietal area 7a and the Basal amygdale
dorsomedial head of the caudate are also targets. Parietal Prefrontal areas 8, 9, 10, and 46
area 7a is the sensory component in the extrapersonal Caudal orbitofrontal cortex area 12
attentional network linked with the dorsolateral prefrontal Inferior temporal pole area 38
executive system. The head of the caudate is also a target Rostral insula
of this executive prefrontal cortex. Cingulate input to the
Anterior parahippocampal areas 35 and 36
caudate assists in the initiation of vocalization behavior as
Anterior medial claustrum
well as executive function. Emotional vocalizations occur-
ring during stimulation in monkeys requires intact cingu- Nonreciprocal targets
late efferents to the periaqueductal gray that produce Anterior superior temporal area 22
similar behaviors when stimulated. The most caudal amyg- Parietal area 7a
dalar projections to 24c, extending into anterior 24c, inner- Dorsomedial head and body of caudate
vate a face representation region that may have direct
Periaqueductal gray
connections with the facial nucleus in the pons. Efferents
Dorsomedial pontine gray matter
to the dorsomedial pons provide cingulate influence on the
576 C Cingulate Gyrus
insula is a somatosensory limbic region. More corticospinal The posterior granular sensory cortices are distin-
neurons are found in the cingulate than are found in the guished from the anterior agranular executive cortices.
supplementary motor cortex, and the cingulate has about The posterior cingulate, with its prominent granular
40% of the amount found in primary motor cortex. Table 3 layer IV, is dedicated to visuospatial and memory proces-
for a summary of these anatomic connections. sing. Major reciprocal connections are with the dorsal
Sensory processing region. Major reciprocal connections visual system of the inferior parietal lobe dedicated to
with the sensory processing region are with caudal parie- spatial processing and with the frontal eye fields in area
tal area 7a, frontal eye fields area 8, posterior perirhinal 8. Reciprocal connections with lateral prefrontal area 46
area 35, presubiculum, posterior parahippocampal area allow an interaction between executive and sensory/mne-
36, prefrontal area 46, and the ventral caudal claustrum. monic processing, which may mediate perceptual work-
Major nonreciprocal projections from the sensory proces- ing memory tasks. Posterior parahippocampal and
sing region target the dorsal caudate, posterior superior perirhinal areas 36 and 35, as well as the presubiculum,
temporal gyrus area 22, and orbitofrontal area 11. These are reciprocally connected to the sensory processing re-
regions are shown in Table 4. gion of the posterior cingulate. These connections modu-
late the multimodal efferents entering the entorhinal layer
III cells that form the perforant pathway into the hippo-
Cingulate Gyrus. Table 3 Major reciprocal connections and campus. Feedback from these areas to the cingulate pro-
nonreciprocal targets for the skeletomotor effector region of vides highly processed sensory information, and the
the cingulate cortex ventral visual system involved in feature analysis can
Reciprocal connections influence the posterior cingulate through these connec-
Primary motor area 4 tions. Although in cats dorsal caudal claustrum is related
Supplementary motor area 6 to visual processing, while the ventral caudal claustrum
receives auditory input, it is possible that in primates
Prefrontal areas 8, 9, and 46
visual information may reach the posterior cingulate via
Parietal areas 1, 2, 3a, 5, and 7b
the ventral caudal claustrum.
Caudal insula The nonreciprocal targets of the posterior sensory
Nonreciprocal targets processing region include the dorsal caudate which also
Lateral putamen receives input from area 7a of the caudal inferior parietal
Spinal cord lobe. This shared connection between the dorsal head of
the caudate and the dorsal visual system of area 7a sup-
Red nucleus
ports the role of the posterior cingulate in visual atten-
Ventrolateral pontine gray matter
tion. Output to posterior superior temporal area 22 will
influence auditory association cortex. Limited efferents to
the rostral portion of area 11 provide the only overlap
with the orbitofrontal-centered belt.
Cingulate Gyrus. Table 4 Major reciprocal connections and Electrical stimulation of the cingulate. Electrical stimula-
nonreciprocal targets for the sensory processing region of the tion studies of the cingulate in humans and animals are
cingulate cortex difficult to interpret because differing techniques have
Reciprocal connections been used in these investigations. With varying intensity,
Caudal parietal area 7 time course, and location of stimulation, it is not
surprising that a spectrum of results is noted. Despite
Frontal eye fields area 8
technical variations, stimulation of the anterior cingulate
Prefrontal area 46
in humans regularly produces visceral motor and affective
Posterior parahippocampal areas 35 and 36 changes, speech alterations, and automatic motor beha-
Presubiculum viors (Meyer, McElhaney, Martin, & McGraw, 1973). In
Ventral caudal claustrum contrast to inhibitory responses elicited by the stimula-
Nonreciprocal targets tion of primary motor cortex, which cannot be controlled,
Dorsal caudate
respiratory arrest from cingulate stimulation can be over-
come volitionally (Penfield & Jasper, 1954). Automatic
Posterior superior temporal area 22
behaviors noted include unilateral and bilateral move-
Orbitofrontal area 11
ments and repetitive tic like movements of the hands,
Cingulate Gyrus C 577
lips, or tongue. These movements can also be consciously cingulate skeletomotor region is also activated (Grafton,
suppressed; implicating the cingulate as an unconscious Woods, & Tyszka, 1994).
effector supports its role in the pathophysiology of
obsessivecompulsive disorder (behaviors that respond
well to cingulotomy). Fear, pleasure, agitation, euphoria, Illness C
and a sense of well being affective phenomena also
common after limbic stimulation have been reported Structural and functional abnormalities. Seizures originat-
(Meyer, McElhaney, Martin, & McGraw, 1973). Involun- ing in the anterior cingulate can alter visceral activity,
tary vocalizations and speech arrests are less common in produce involuntary skeletomotor output, result in dis-
humans than in animals with stimulation of areas 32, 24, turbed attention, and cause interictal behavior abnormal-
and the rostral part of 25 (Vogt & Barbas, 1988). ities. The severity and specific abnormality will depend
Functional activation of the cingulate. In functional activa- upon the location of the seizure focus and ensuing dam-
tion studies, the cognitive effector region of the anterior age that affects interictal brain function. A diverse assort-
cingulate is activated when sustained attention to novel ment of atonic, absence, speech arrest, autonomic, and
tasks is required. Tasks spanning motor, language, mem- complex partial seizures with secondary generalization
ory, and visuospatial paradigms all produce supracallosal have been described. Inaccessibility of the medial hemi-
anterior cingulate activation. When memory encoding is sphere to surface electrode recording is the greatest obsta-
combined with a motor task demanding sustained cle to the elucidation of cingulate seizures.
divided attention (Fletcher et al., 1995), only the anterior In a study involving 36 cases (Mazars, 1970), depth
cingulate is activated due to the sustained vigilance electrodes revealed near-instantaneous bilateral spread to
demanded by dividing effort between the two tasks. the frontal poles when the focus was in the anterior
When motivation to master a task is no longer required, cingulate; posterior foci spread to the contralateral cingu-
and accurate performance of a task becomes routine, the late within seconds, followed by involvement of the con-
anterior cingulate returns to a baseline activity level vexities with generalized tonicclonic seizures. Emotional
(Raichle et al., 1994). The acquisition of novel cognitive stress often precipitated the seizures. Psychoses and
strategies requires the dynamic vigilance of the supra- episodic rage were common interictal behavioral abnorm-
callosal cingulate, but with practice the motivation re- alities that responded to the removal of the anterior cin-
quired to entrain new cognitive networks to a novel task gulate and occasionally the frontal pole as well.
is no longer necessary. A distinction between motivation Consciousness may be altered and automatisms can be
and attention is important. A task is still attended to and voluntarily inhibited or integrated with ongoing move-
completed correctly after the motivating influence of the ments (Geier et al., 1977).
supracallosal cingulate has initiated the acquisition of an An 11-year-old girl who initially had atonic seizures at
efficient cognitive routine. Through the activation of the age 30 months was reported to develop complex partial
anterior supracallosal cingulate limbic motivation directs seizures with blinking, lip smacking, automatisms, and
the selection of the best cognitive strategy among many humming (Levin & Duchowny, 1991). An obsessive
competing contingencies. Thus, activation studies using compulsive disorder developed over a 5-year period, and
varied tasks consistently activate the cognitive effector by age 8, she became preoccupied with Satan and her
region in normals motivated to succeed in whatever task personal hygiene. Seizure focus, recorded from depth
is given them. The contribution to an extrapersonal at- electrodes, was in the right anterior cingulate. The
tentional network involving direct links between the patients behavioral abnormalities responded well to a
anterior cingulate, dorsolateral executive frontal area, 4-cm ablation of the right anterior cingulate.
and the inferior parietal lobule provided by the cogni- Another case of a right anterior cingulate focus with
tive effector region is the motivation to engage in a cogni- accompanying behavioral abnormalities has been de-
tive challenge. scribed (Devinsky, Morrell, & Vogt, 1995). One year
Functional imaging has also confirmed the role of the after mild head trauma, a 42-year-old male developed,
skeletomotor effector region in the preparation of motor over a 15-year period, sociopathic behavior and complex
output and motor learning. When a motor task is only partial seizures unresponsive to medical treatment. Sei-
imagined, the cingulate cortex inferior and anterior to the zures were usually nocturnal and frequent (1020 per
supplementary motor area (dorsal bank of the cingulate night), with stereotypic motor output: facial contortions,
sulcus) shows significant activation. During the acquisi- tongue thrusting, a strangulated yell, flexion of the neck
tion of procedural learning in a rotory pursuit task, the and trunk, bilateral extremity extension, and thrashing
578 C Cingulate Gyrus
with preserved consciousness. Occasionally, generali- lesions, possibly affecting the ascending reticular core. Fail-
zation to tonicclonic seizures developed with loss of ure of response inhibition on go-no-go tests is the major
consciousness. neuropsychological deficit in the patient with an anterior
Irritability, disinhibition, and sexual deviancy were medial frontal damage. The loss of spontaneous motor
behavioral complications in a police officer who was dis- activity results when the lesion involves the supplementary
missed from the force because of brutality and the use of motor area and the skeletomotor effector region. When
confiscated drugs. Surface and sphenoidal electroenceph- these two motor regions are spared, motor activity will be
alogram showed rhythmic bifrontal theta. Magnetic reso- normal but the patient will demonstrate profound indif-
nance imaging (MRI) and [18F]-fluorodeoxyglucose ference, docility, and the loss of motivation to engage in a
positron emission tomography (FDG-PET) were essen- task. They can be led by the examiner to engage in a task
tially normal, but depth electrodes revealed a right cingu- but will fail to self-generate sustained directed attention.
late focus which spreads to the ipsilateral orbitofrontal They lack cognitive motivation.
area and contralateral anterior cingulate in 300 ms. Re- The role of the anterior cingulate as a cognitive effec-
section of the right cingulate and anterior corpus callo- tor is appreciated within the realm of language. Language,
sum relieved 90% of the spells with only brief axial flexion a cognitive function, is distinguished from the motor
being the residual seizures. The behavioral abnormalities function of speech. Transcortical motor aphasia
were reported to improve with the patient married and (TCMA) is the usual result of left anterior medial or
employed as a fast-food restaurant manager. anterior dorsolateral prefrontal lesions. The classic syn-
Both stimulation and seizure activity can discharge drome of TCMA is initial mutism that resolves in days to
the functional centers of the cingulate to produce a vis- weeks, yielding a syndrome featuring delayed initiation of
ceral effect, a cognitive or behavioral change, and a speech brief utterances without impaired articulation, excellent
or motor output. Appreciating the functional centers repetition, inappropriate word selection, agrammatism,
within the cingulate assists the interpretation of the and poor comprehension of complex syntax. Activation
signs and symptoms exhibited when it discharges. The of dorsolateral prefrontal cortices enabling language and
interictal behavioral abnormalities of anterior cingulate speech arises from two sources: the anterior cingulate and
epilepsy reflects the dysfunction of limbic networks the supplementary motor area (with the cingulate skele-
which, if affecting infracallosal and orbitofrontal cortex, tomotor effector region). When the executive prefrontal
will result in visceral motor disturbances and disinhibi- cortex (areas 9, 10, and 46) is disrupted, cognitive lan-
tion with socially inappropriate behavior. Obsessive guage deficits are prominent (TCMA, type I); when
compulsive features may occur with dysfunction of the motor neurons in area 4, devoted to the speech apparatus,
cognitive component of the supracallosal cingulate. This are disconnected from their activation, speech hesitancy
abnormal dynamic vigilance exerted by the cognitive and impoverished output ensues (TCMA, type II). These
effector region in obsessivecompulsive disorders can two functional realms are separable and can be discon-
occur from a well-circumscribed seizure focus in this nected anywhere along two pathways. Direct damage to
region (Levin & Duchowny, 1991) and is relieved by the supplementary motor area or its efferent pathway to the
surgical ablation of this region or its outflow (Tow & motor cortex traveling in the anterior superior paraventri-
Whitty, 1953). cular white matter will produce TCMA type II. Direct
Focal lesions and syndromes. Well-circumscribed lesions in damage to the anterior cingulate, its outflow to areas 9,
humans are rarely confined to one region of the cingulate. 10, and 46, or to the caudate via the subcallosal fasciculus,
With an anterior lesion, both the cognitive, skeletomotor just inferior to the frontal horn of the lateral ventricle will
and visceral effector regions are often affected. Bilateral disrupt frontal-subcortical circuits involved in motivation
lesions result in an akinetic mute state ( Akinetic Mut- and executive cognitive functions. The initial muteness has
ism). Patients are profoundly apathetic. Rarely moving, been described by a patient after recovering from an anteri-
and incontinent, they eat and drink only when fed, and if or cingulate/supplementary motor infarction as a loss of the
speech occurs it is limited to monosyllabic responses to will to reply to her examiners, because she had nothing
questioning. Patients appear awake with eyes tracking to say, her mind was empty, and nothing mattered
objects. Displaying no emotions, even when in pain, ( Akinetic Mutism).
patients show complete indifference to their circumstance. The cingulum bundle has also been the site of surgical
Transient akinetic mutism with similar features occurs with lesions (cingulumotomy, or cingulotomy when cingulate
unilateral lesions. The akinetic mute state can also result cortex is also removed) to treat psychiatric and pain dis-
from bilateral paramedian diencephalic and midbrain orders. The cingulum contains the efferents and afferents
Cingulate Gyrus C 579
of the cingulate to the hippocampus, basal forebrain, and the hippocampus during encoding and may also be
amygdala, and all cortical areas, as well as fibers of passage important in the storage of long-term information.
between hippocampus and prefrontal cortex, and from Synthesizing cytoarchitectonic refinements, nonhu-
the median raphe to the dorsal hippocampus. Surgical man primate tracer studies, clinical-behavioral correla-
ablation of the anterior portion (sparing fibers relevant tion data, and functional neuroimaging results have C
to memory function) is most successful when treating been refined but have not significantly added to the
aggression, extreme anxiety, obsessivecompulsive beha- basic description of cingulate function offered by Papez:
viors, and severe pain. Psychotic symptoms show only a
" It is thus evident that the afferent pathways from the recep-
temporary response. The three anterior cingulate regions,
tor organs split at the thalamic level into three routes, each
by virtue of the distinct functional systems they access, are
conducting a stream of impulses of special importance. One
the conduits through which limbic motivation can acti-
route conducts impulses through the dorsal thalamus and
vate feeling, thought, and movement.
the internal capsule to the corpus striatum. This route
Lesions of the posterior cingulate disrupt memory func-
represents the stream of movement. The second conducts
tion in animals and humans. The closing link in the circuit
impulses from the thalamus through the internal capsule to
of Papez, from the anterior thalamic efferents traveling
the lateral cerebral cortex. This route represents the stream
through the posterior cingulum to areas 32 and 29/30, is
of thought. The third conducts a set of concomitant
the cingulate projection sent to the presubiculum. Anterior
impulses through the ventral thalamus to the hypothala-
cingulotomy will not disrupt this memory circuit but rarely
mus and by way of the mamillary body and the anterior
pathologic lesions will extend into, and beyond, the poste-
thalamic nuclei to the gyrus cinguli, in the medial wall of the
rior cingulate. If the lesion extends inferior to the splenium
cerebral hemisphere. This route represents the stream of
of the corpus callosum, it may also disrupt the fornix, thus
feeling. In this way, the sensory excitations which reach the
disconnecting the efferents from the hippocampus to the
lateral cortex through the internal capsule receive their
diencephalon. If the lesion extends posteriorly, it may dam-
emotional coloring from the concurrent processes of hypo-
age the supracommissural portion of the hippocampus
thalamic origin which irradiate them from the gyrus cinguli.
the gyrus fasciolaris and the fasciola cinerea. A large left-
(Papez, 1937)
sided lesion that extended beyond the posterior cingulate
into the fornix and supracommissural hippocampus after
the surgical repair of an arteriovenous malformation
resulted in a persistent amnesia (Cramon & Schuri, 1992). References and Readings
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gery & Psychiatry, 16, 186193. resonance imaging and neuropsychological performance in amnestic
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Watson, R. T. (1987). Retrosplenial amnesia. Brain, 110, 16311646. Psychiatry, 77(10), 11222112.
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cingulate vocalization region in the rhesus monkey. In J. D. Newman (2004). Fiber tract-based atlas of human white matter anatomy.
(Eds.), The physiological control of mammalian vocalization (pp. Radiology, 23(1), 7787.
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Cingulum
CIQ
I RENE P IRYATINSKY
Butler Hospital and Alpert Medical School of Brown Community Integration Questionnaire
University
Providence, RI, USA
Circadian Clock
Definition
Circadian Rhythms
The cingulum is a collection of white matter fibers project-
ing from the cingulate gyrus to the entorhinal cortex in the
brain, allowing for communication between components
of the limbic system. The cingulum connects the medial
temporal lobe with the posterior cingulate gyrus. Diffusion
Circadian Rhythms
tensor imaging studies have reported cingulum bundle
B RUCE J. D IAMOND
disruption in mild cognitive impairment and Alzheimers
William Paterson University
disease. In addition, some research indicates that in Alzhei-
Wayne, NJ, USA
mers disease, the posterior cingulate cortex hypofunction
is due to the indirect effect of the degeneration of cingulum
fibers secondary to medial temporal lobe atrophy.
Synonyms
Cross References Biological cycles; Biorhythms; Circadian clock
Cingulate Gyrus
Short Description or Definition
References and Readings
The pervasive characteristics of living organisms are
Catani, M., Howard, R. J., Pajevic, S., & Jones, D. K. (2002). Virtual
rhythmic biological and behavioral changes, which are
in vivo interactive dissection of white matter fasciculi in the human expressed at varying levels of organization ranging from
brain. NeuroImage, 17(1), 7794. basic cellular to the highly complex level. These rhythms
Circadian Rhythms C 581
are generally referred to as circadian rhythms and they Developmental factors may also mediate some of the
organize a variety of behaviors, including sleep, which is variations in circadian rhythms. For example, the aging
characterized by a 90-min Rapid Eye Movement (REM) process is often accompanied by changes in circadian
cycle and the wakefulnesssleep cycle, which is organized rhythms that may affect sleep and place older adults at a
around an approximate 24-h cycle (Carlson, 1999). higher risk for sleep disturbance (Lee-Chiong, 2005). C
intraindividual and interindividual basis. At a certain Quigg, M. (2006). Circadian rhythms: Problems with the body clock.
In N. F. Watson & B. V. Vaughn (Eds.), Clinicians guide to sleep
point, these deviations may be considered clinical abnor-
disorders (pp. 277304). Philadelphia: Taylor & Francis.
malities (i.e., seasonal affective disorder). In fact, seasonal Waterhouse, J. M., & DeCoursey, P. J. (2004a). Human circadian organi-
affective disorder has been treated using phototherapy, zation. In J. C. Dunlap, J. J. Loros, & P. J. DeCoursey (Eds.),
which involves the use of light boxes, whereby a patient Chronobiology: Biological timekeeping (pp. 291323). Sunderland,
is exposed to light each winter morning or evening and MA: Sinauer.
Waterhouse, J. M., & DeCoursey, P. J. (2004b). The relevance of circadian
the circadian rhythm becomes entrained in ways that
rhythms for human welfare. In J. C. Dunlap, J. J. Loros, &
induce circadian phase shifts (Kolb & Wishaw, 2005). P. J. DeCoursey (Eds.), Chronobiology: Biological timekeeping
Individuals who make substantial changes to their sleep (pp. 325356). Sunderland, MA: Sinauer.
wake cycles during night work or after trans-meridian
travel could be helped using a chronobiological treatment.
The treatment would involve exposure to appropriate
circadian synchronizers and basing therapy on phase re-
sponse curves for light and melatonin (Waterhouse & Circle of Friends
DeCoursey, 2004a). By making accommodations in the
timing of work-related activities (Hasher et al., 2005), Circles of Support
performance efficiency can be improved and fatigue
decreased, thus enhancing health and safety (Costa, 1999).
And in treating patients with bipolar disorder, psychoso-
cial therapies should identify areas of vulnerability includ- Circle of Willis
ing unhealthy circadian rhythms (Newman, 2006).
E LLIOT J. R OTH
Northwestern University
Cross References
Chicago, IL, USA
Fatigue
Sleep Disturbance
Definition
References and Readings The circle of Willis is the anatomical name given to
the formation of arteries at the base of the brain that
Carlson, N. R. (1999). Foundations of Physiological Psychology (4th edn.).
contribute the overwhelming majority of blood supply
Boston: Allyn & Bacon. to the brain.
Clark, A. V. (Ed.). (2005). Causes, role and influence of mood states.
Hauppauge, NY: Nova Biomedical Books.
Costa, G. (1999). Fatigue and biological rhythms. In D. J. Garland, J. A.
Wise, & D. V. Hopkin (Eds.), Handbook of aviation human factors Current Knowledge
(pp. 235255). Mahwah, NJ: Lawrence Erlbaum.
Hasher, L., Goldstein, D., & May, C. P. (2005). Its about time: Circadian
rhythms, memory, and aging. In C. & Izawa N. Ohta (Eds.), Human The circle of Willis is formed by the connections between
learning and memory: Advances in theory and application: The 4th the predominantly horizontal branches that derive from
Tsukuba international conference on memory (pp. 199217). the middle cerebral arteries anteriorly and from the
Mahwah, NJ: Lawrence Erlbaum. basilar artery posteriorly. The right and left middle cere-
Johnson, C. H., Elliot, J., & Foster, R. (2004). Fundamental properties of
bral arteries each give off an anterior cerebral artery
circadian rhythms. In J. C. Dunlap, J. J. Loros, & P. J. DeCoursey
(Eds.), Chronobiology: Biological timekeeping (pp. 67105). Sunder- (forming the anterolateral borders of the circle of Willis),
land, MA: Sinauer Associates. which goes forward to supply blood to the frontal lobe.
Kolb, B., & Wishaw, I. Q. (2005). An introduction to brain and behavior. These anterior cerebral arteries are connected to each
New York: W. H. Freeman. other by the anterior communicating artery, which
Lee-Chiong, T. L. (2005). Sleep: A comprehensive handbook. Hoboken, NJ:
forms the front of the circle. Posteriorly, the basilar
Wiley.
Newman, C. F. (2006). Bipolar disorder. In F. Andrasik (Ed.), Compre- artery bifurcates into the right and left posterior cerebral
hensive handbook of personality and psychopathology: Vol. 2: Adult arteries, which supply the occipital and posterior tem-
psychopathology (pp. 244261). Hoboken, NJ: Wiley. poral lobes and the cerebellum, forming the posterior
Circles of Support C 583
border of the circle. Each posterior cerebral artery is personal goals. It is one of many tools addressing life
connected to the middle cerebral artery on its planning from a functional or strategic assessment ap-
corresponding side by a posterior communicating artery, proach known as person-centered planning. Person-
forming the posterolateral borders of the circle. There centered planning replaces more traditional assessment
are several clinical implications of the circle pattern approaches associated with the medical model of services. C
of these arteries. Perhaps most importantly, because Circles of support originated in Canada and have experi-
of the interconnectedness of the arteries that result enced widespread use in North America. Circles view
from this circle format, if one of the main arteries is people as individuals and assist them to attain self-
occluded, the distal smaller arteries that it supplies determination focusing upon empowerment and
can potentially receive blood from the other arteries not dependence of the individual. It is capacity oriented
that make up the circle, a phenomenon known as and identifies strengths, preferences, likes, and dislikes of
collateral circulation. This helps to prevent cerebral the individual. The circle will also identify support needs
ischemia and stroke. The circle of Willis also is a in order to achieve a particular goal.
common site for cerebral aneurysms, with the greatest The focus person leads the process and decides who
numbers involving the anterior communicating artery, will participate in the circle and the direction which the
posterior communicating arteries, and middle cerebral planning will take. Typically, a facilitator is selected
arteries. from within the circle to help energize the group. The
first circle is the circle of intimacy and includes
the people most intimate in the focus persons life. The
Cross References second circle, the circle of friendship, includes good
friends and close relatives. The third circle, the circle
Anterior Cerebral Artery of participation includes the people and organizations
Anterior Communicating Artery the focus person is involved with. The fourth circle is the
Basilar Artery circle of exchange and includes those that are paid to
Internal Carotid Artery be in the focus persons life. Members are not paid to be
Middle Cerebral Artery involved in a circle of support, but are involved in the
Posterior Cerebral Artery focus persons life in some capacity. Members use their
Posterior Communicating Artery skills, knowledge, and networks to help the focus person
Vertebrobasilar System accomplish goals. The circle develops and monitors
the plan, making sure that it is current with the wishes
of the focus person. It is as ongoing process that is
dynamic.
Circles of Support
A MY J. A RMSTRONG Cross References
Virginia Commonwealth University
Richmond, VA, USA Inclusion
Medical Model
Synonyms
References and Readings
Circle of friends
Falvey, M. A., Forest, M., Pearpoint, J., & Rosenberg, R. (1994). All my
lifes a circle. Using the tools: Circles, MAPs and PATH. Toronto,
Definition Canada: Inclusion Press.
OBrien, C. L., & OBrien, J. (2000). The origins of person-centered
planning: A community of practice perspective. Retrieved from
A circle of support is a group of people that forms a http://thechp.syr.edu/PCP_History.pdf
community around a specific individual (focus person) OBrien, J., & Lyle OBrien, C., (Eds.). (1998). A little book about person
with significant disabilities to assist him or her to achieve centered planning. Toronto: Inclusion Press.
584 C Circumduction
Brand Name
Additional Information
Celexa
Drug Interaction Effects: http://www.drugs.com/drug_interactions.html
Drug Molecule Images: http://www.worldofmolecules.com/drugs/
Free Drug Online and PDA Software: www.epocrates.com
Class Gene-Based Estimate of Drug interactions: http://mhc.daytondcs.
com:8080/cgi bin/ddiD4?ver=4&task=getDrugList
Selective Serotonin Reuptake Inhibitor Pill Identification: http://www.drugs.com/pill_identification.html
impairments due to cognitive deficits rather than to phys- that uses a total score generated from the sum of boxes
ical disability. Based solely on clinical information (CDR-SB) has gained popularity to quantify progression
obtained from the patient and informant, and without of AD in longitudinal clinical trials of experimental thera-
reference to psychometric performance, a box score de- pies (e.g., Petersen et al., 2005).
scribing the level of impairment is generated for each
domain. The box score ranges from 0 to 3, representing
none to severe impairment. Using a scoring algo- Psychometric Data
rithm, one of five possible stages is then derived from
the individual box scores as follows: CDR = 0 (no demen- Interrater Reliability
tia); CDR = 0.5 (questionable dementia); CDR = 1 (mild
dementia); CDR = 2 (moderate dementia); CDR = 3 (se- Most of the psychometric studies on the CDR have
vere dementia). Although not part of the original proto- focused on interrater reliability in multicenter clinical
col, CDR = 4 (profound) and CDR = 5 (terminal) can also trials. These studies have concluded that experience
be used to classify the later stages of dementia. An alter- using the CDR increases reliability estimates, although
native method that generates a total score (range 018) adequately trained inexperienced raters may also demon-
from the sum of boxes (CDR-SB) has also been used for strate a high level of agreement (kappa = 0.83 or higher;
quantification purposes in longitudinal studies (e.g., Schafer et al., 2004; Tractenberg, Schafer, & Morris, 2001).
Cortes et al., 2008). The CDR takes about 90 min to The CDR also shows good reliability among raters of
administer. various qualifications. There were no major differences in
The CDR has been used in clinical practice and mul- reliability among physicians, nurses, PhDs, social workers,
ticenter clinical trials, as well as in cross-cultural dementia psychometrists, or research assistant raters (85% for
studies around the world. The CDR protocol is available non-MDs and 82% for MDs; Oremus, Perrault, Demers,
in over 60 languages and dialects, including English, & Wolfson, 2000). Kappas between physician raters range
French, Spanish, Italian, Portuguese, Dutch, Czech, from 0.75 to 0.94 for the six individual domain scores and
Bulgarian, Russian, Tagalog, Afrikaans, Greek, German, CDR-SB score. Kappas between nurses, or between nurses
Hebrew, Indian dialects, Chinese dialects, Japanese, and and physicians, range from 0.66 to 0.77 (Oremus et al.,
Korean. These translations can be downloaded free of cost 2000).
on the CDR Web site (http://alzheimer.wustl.edu/cdr/
default.htm).
An online training video on the use of the CDR is Construct Validity
available free for individual users, and takes about 89 h to
complete. Detailed scoring algorithms, including tie-break Evidence for construct validity of the CDR appears solid.
rules, and an online scoring worksheet are available on the In the original study, the CDR had strong correlations
CDR Web site. with the Blessed Dementia Scale (BDS; r = 0.74) and the
Pfeiffer Short Portable Mental Status Questionnaire
(SPMSQ; r = 0.84) among individuals with CDR ratings
Historical Background between no dementia and very mild dementia (Hughes
et al., 1982). Correlations with various cognitive measures
The CDR was originally developed at the Washington ranged from small to large in community-dwelling sam-
University School of Medicine in 1979 to evaluate the ples (Folsteins Mini-Mental State Examination = 0.33;
progression of AD (Hughes et al., 1982). The original BDS = 0.74; SPMSQ = 0.84). Similar correlations were
protocol has evolved somewhat over the years, with box found with neuropsychological measures such as the
descriptors updated to sharpen the distinction between CERAD Boston Naming Test, list learning, and verbal
severity levels within each domain, and new scoring rules fluency (Oremus et al., 2000).
added to resolve scoring ambiguity (Morris, 1993). Alter- In terms of neuropathology, CDR = 0.5 (a proxy for
native scoring methods have also been suggested to im- mild cognitive impairment; MCI) has been associated
prove scoring accuracy (Gelb & St. Laurent, 1993). An with multiple pathological signs including those related
extension of this scale to include CDR = 4 (profound) and to AD, Lewy body dementia, or vascular dementia, as
CDR = 5 (terminal) to classify the later stages of dementia well as nonspecific pathology (Saito & Murayama,
(Dooneief, Marder, Tang, & Stern, 1996) among nursing 2007). Further, increased microglia activation, an inflam-
home elderly has been proposed. As well, another method matory biomarker of AD, was seen with advancing CDR
Clinical Dementia Rating C 589
stages (Xiang, Haroutunian, Ho, Purohit, & Pasinetti, populations (Lim, Chin, Lam, Lim, & Sahadevan, 2005;
2006). A negative association was also found between the Senanarong, Chen, & Orgogozo, 2006), although empiri-
CDR-SB score and glucose metabolism in the right poste- cal validation of various translations is needed.
rior cingulate gyrus (Perneczky, Hartmann, Grimmer, The CDR presents several advantages over psychomet-
Drzezga, & Kurz, 2007). ric tests. First, it is an assessment option for patients who C
are illiterate and/or have limited English language profi-
ciency. Moreover, it can also be used in the presence of
aphasia, a condition common among patients with
Predictive Validity
dementing disorders. Lastly, the administration of the
CDR does not require a standardized set of instructions,
There is evidence that individuals with higher CDR-SB
but is dependent upon a set of guidelines. As such, the
scores are more likely to develop dementia in the future
CDR is easily adapted for cross-cultural use (e.g., Lim
(Lynch et al., 2006). In addition, CDR scores predict
et al., 2005). A disadvantage of the CDR is that it is
survival in individuals with suspected dementia. Using
somewhat lengthy to administer.
survival as outcome, the median survival was 1 year for
CDR = 5, 2 years for CDR = 4, 2.5 years for CDR = 3,
3 years for CDR = 2, and 3.5 years for CDR = 1 (Dooneief
Cross References
et al., 1996). Use of the CDR as a screening tool for
dementia revealed a sensitivity of 92% and specificity of
Alzheimers Disease
94% for mild dementia in a community sample of adults
CERAD
older than age 75 (Juva et al., 1995).
Dementia
Clinical Uses
References and Readings
The original focus of the CDR was to assess community-
dwelling older adults, since its anchor points probe for Cortes, F., Nourhashemi, F., Guerin, O., Cantet, C., Gillette-Guyonnet, S.,
examples of ones engagement with the home and com- Andrieu, S., et al. (2008). Prognosis of Alzheimers disease today: A
two-year prospective study in 686 patients from the REAL-FR study.
munity. However, it has also been adapted for use in
Alzheimers and Dementia, 4(1), 2229.
chronic long-term care facilities (Marin et al., 2001). Dooneief, G., Marder, K., Tang, M. X., & Stern, Y. (1996). The Clinical
The global CDR is widely used primarily for the staging Dementia Rating scale: community-based validation of profound
of AD. It has also been used to stage other dementing and terminal stages. Neurology, 46, 17461749.
disorders such as Parkinsons disease and frontotemporal Gelb, D. J., & St. Laurent, R. T. (1993). Alternative calculation of the
global clinical dementia rating. Alzheimer Disease & Associated
dementia. In recent years, CDR = 0.5 has been used to
Disorders, 7(4), 20211.
characterize MCI; however, there is evidence that a large Hughes, C. P., Berg, L., Danziger, W. L., Coben, L. A., & Martin, R. L.
proportion (29.7%) of individuals at CDR = 0.5 also meet (1982). A new clinical scale for the staging of dementia. British
ICD-10 criteria for mild dementia (Lynch et al., 2006). Journal of Psychiatry, 140, 566572.
In terms of longitudinal studies of AD progression, Juva, K., Sulkava, R., Erkinjuntti, T., & Ylikoski, R. (1995). Usefulness of
the Clinical Dementia Rating scale in screening for dementia. Inter-
both the global CDR and the CDR-SB appear to be
national Psychogeriatrics, 7(1), 1724.
useful for tracking cognitive changes over a 23 year Lim, W. S., Chin, J. J., Lam, C. K., Lim, P. P. J., & Sahadevan, S. (2005).
period (e.g., Cortes et al., 2008; Meguro et al., 2004). Clinical Dementia Rating: experience of a multi-racial Asian
Of the two scoring methods, the CDR-SB is more population. Alzheimer Disease and Associated Disorders, 19(3),
commonly used in clinical drug trials because it was 135142.
Lynch, C., Walsh, C., Blanco, A., Moran, M., Coen, R., Walsh, J., et al.
found to be sensitive to changes within 12 months follow-
(2006). The clinical dementia rating sum of box score in
ing baseline measurement in donepezil drug trials, where- mild dementia. Dementia and Geriatric Cognitive Disorders, 21(1),
as the global CDR was not (e.g., Petersen et al., 2005). 4043.
There is also evidence that the CDR-SB is more useful Marin, D., Flynn, S., Mare, M., Lantz, M., Hsu, M., Laurans, M., et al.
than the global CDR in distinguishing mild cognitive (2001). Reliability and validity of a chronic care facility adaptation of
the Clinical Dementia Rating scale. International Journal of Geriatric
deficits from dementia (Lynch et al., 2006).
Psychiatry, 16(8), 745750.
In addition to western populations, use of the CDR Meguro, K., Shimada, M., Yamaguchi, S., Sano, I., Inagaki, H.,
has also been accepted as an appropriate comprehensive Matsushita, M., et al. (2004). Neuropsychosocial features of very
measure for studies of dementia patients in Asian mild Alzheimers Disease (CDR 0.5) and progression to dementia
590 C Clinical Depression
Current Knowledge
Clinical Extender There are three types of clinical interview, reflecting
the degree to which the content and questions are scripted:
Psychometrician structured, semi-structured, and unstructured. A struc-
tured interview (e.g., the Structured Clinical Interview for
DSM-IV-Clinical Version [SCID-I]; First, Gibbon, Spitzer,
& Williams, 2001), like any standardized assessment tool,
gathers specific data that allows clinicians to make com-
Clinical Importance parisons between client and normative group function.
Criticisms of structured clinical interviews include a frus-
Clinical Significance tration with lengthy questionnaires and the missed
Clinical Neuropsychology C 591
Clinical neuropsychology is recognized as a specialty intended to measure what he termed biological intelli-
by APA, the American Board of Professional Psychology, gence. The influence of Galtons statistical methods is
and the Canadian Psychological Association. Though seen in Halsteads factor analysis of his test battery. Hal-
there is no agreed upon date for the emergence of the stead proposed a four-factor theory of biological intelli-
field, clinical neuropsychology began to be recognized as a gence in his 1947 book Brain and Intelligence. Halsteads
distinct professional discipline following a 1948 sympo- book was severely criticized by contemporary scholars,
sium at the APA annual meeting appropriately entitled and ultimately had little influence on theories of intelli-
Neuropsychology. In this symposium, Hans-Lukas gence (Hartman, 1991). Halsteads test battery, however,
Teuber described procedures he and Morris Bender devel- did prove highly influential. Ralph Reitan, a student of
oped to study the behavioral effects of penetrating missile Halstead, established a laboratory at the University of
wounds to the brain (Benton, 1987). Indiana Medical Center in 1950. Over the course of sever-
Clinical neuropsychology had many forerunners. In al decades, Reitan expanded upon Halsteads initial bat-
the 1880s, Sir Francis Galton opened a laboratory in tery, adding procedures for detecting aphasia and sensory
London where for a few pennies, people could take tests perceptual impairments and for comparing performances
of visual acuity, reaction time, and various psychophysical of the two sides of the body. Reitan developed adaptations
abilities. A cousin of Charles Darwin, Galtons ideas about of the original adult battery for children and adolescents,
eugenics have made him a historically controversial fig- collected normative data, and used discriminant function
ure. Nonetheless, factor analytic statistical methods grew analysis to validate the ability of the various batteries to
out of his work and became critical for the development discriminate brain damaged from neurologically healthy
and validation of mental ability tests. His work was put to individuals. The HalsteadReitan Neuropsychological
practical use in early twentieth-century France when psy- Test Battery set a standard of excellence for test develop-
chologists Alfred Binet and Theodore Simon developed ment in neuropsychology and for a time was the most
intellectual tests to identify and place children in need of widely used approach in clinical neuropsychological
special education. World War I also stimulated interest in assessment.
his work in the USA, as the military sought tests that Reitans students were also prolific (Reed, 1985). They
could efficiently identify the strengths and weaknesses of include Hallgrim Klve who studied with Reitan in the
large numbers of military recruits. As many as one million 1950s and then established a laboratory at the University
soldiers underwent mental ability testing during World of Wisconsin. Another student, Homer Reed, directed the
War I (Hartman, 1991). Neuropsychology Laboratory at the New England Medical
Just after World War I, the German American psychol- Center in Boston where he concentrated on use of the
ogist Shepherd Ivory Franz (1919) published detailed battery with pediatric patients. Phillip Rennick, who did
descriptions of tests of tactile sensation, motor coordina- fellowship training with Reitan, went on to establish a
tion, praxis, language, attention, memory, visuospatial laboratory at the former (now defunct) Lafayette Clinic
perception, reasoning, and intelligence. By 1924, hand in Detroit, and developed a repeatable neuropsychological
dynamometers, finger tapping keys, motor steadiness battery for situations in which serial testing is needed.
tests, form perception boards, and tests of color vision, Just as World War I provided an impetus for the
vibration sense, attention, and memory were available development of psychological ability tests, World War II
(Hartman, 1991). Though these tests were quickly stimulated the development of neuropsychological assess-
adopted by researchers (see Neuropsychology, Science of), ment and treatment methods because of the large number
clinical application came a few decades later. Franz advo- of veterans who returned having survived penetrating
cated for their clinical use in a series of lectures to the missile wounds to the brain. Reitans early work involved
Government Hospital for the Insane, starting in 1910 the examination of brain-injured World War II veterans.
(Hartman, 1991) and in 1904 was instrumental in estab- This population also provided the impetus for Hans-
lishing a psychological laboratory at Mclean Hospital in Lukas Teubers work in the USA and Aleksandr Romano-
Boston. This was the first such laboratory in a hospital vich Lurias work in Russia.
setting in the USA Teuber immigrated from Germany and served as a
In 1935, the experimental psychologist Ward Halstead noncommissioned naval officer before establishing the
opened a laboratory at the University of Chicago for the Psychophysiological Laboratory with the neurologist
psychological study of neurology and neurosurgery Morris Bender at New York University (Weinstein,
patients. Adopting techniques used to test animals in 1985). Besides being the focal point for numerous seminal
ablation studies, Halstead designed a series of tests studies, Teubers laboratory was the incubator for many
Clinical Neuropsychology C 593
neuropsychologists who went on to make important con- neuropsychology practitioners and researchers. Dr.
tributions including Joseph Altman, Lila Ghent, Rita Kaplan and her associates in Boston developed the pro-
Rudel, Josephine Semmes, and Sidney Weinstein. cess-approach to neuropsychological assessment, recog-
Meanwhile, working with Russian World War II veterans nizing that tests are complex and multifactorial and that
at the Burdenko Neurosurgical Institute in Moscow, Luria patients can take different paths to the same test score. C
developed a richly qualitative approach to neuropsycho- According to this approach, only by systematically analyz-
logical assessment that was in stark contrast to the quan- ing the process(es) by which patients arrive at their
titative and normatively based test batteries that were in responses, often by parsing a test into fine-grained com-
use in the USA Luria also was an exponent of neuropsy- ponents, can a neuropsychologist truly understand what
chological approaches to rehabilitation and to use of aspect of brain functioning is compromised.
pharmacologic agents to enhance recovery of function Activity was not confined to North America. Clinical
(Gualtieri, 1988). neuropsychology got its South American start when C.
Besides having cognitive disorders, many injured Mendilaharsu and S. Acevedo de Mendilaharsu established
World War II veterans were aphasic, sparking neuropsy- the first South American neuropsychological laboratory
chological interest in language and the brain. Kurt in 1958 at the Neurological Institute in Montevideo,
Goldsteins ( Goldstein, Kurt) book Language and Lan- Uruguay (Ardila, 1990). Despite sometimes challenging
guage Disorders, published in 1948, combined his theories economic conditions, South American neuropsycholo-
about abstracting ability with neurologys classic anato- gists conducted investigations of constructional ability,
mico-clinical syndrome approach to aphasia. The num- dementia, and language. The field spread to Mexico,
bers of aphasic veterans available for study along with Peru, Columbia, Chile, Argentina, Brazil, Honduras,
Goldsteins influential book attracted experimental neu- Nicaragua, and elsewhere.
ropsychologists and psycholinguists to the study of lan- Clinical neuropsychology as a profession has be-
guage disorders (Goodglass, 1985). Aphasia research come increasingly organized since its inception. The
centers began to appear in the USA, among them being International Neuropsychological Society (INS), the
a laboratory established by Arthur Benton. National Academy of Neuropsychology (NAN), and
In 1948, the year of Teubers seminal APA presenta- the APA Division of Clinical Neuropsychology (Divi-
tion, Arthur Benton accepted an appointment as Profes- sion 40) are the most well-known professional neuro-
sor of Psychology at the University of Iowa, and by 1950 psychological organizations within the USA. Founded
established a neuropsychological testing unit at the Uni- in 1967, INS has a membership that exceeds 3,500.
versity of Iowa Hospitals (Hamsher, 1985). There, Benton NAN, formed in 1974, includes more than 3,000 mem-
and his students and colleagues conducted normative bers. Division 40 of the American Psychological Associ-
studies, examining the effects of age, gender, and educa- ation, incorporated in 1980, is the most recent of these
tion. They also developed a variety of tests for use in professional organizations, with a membership of over
studying what had previously been vaguely defined clini- 4,000 neuropsychologists. Although smaller in member-
cal disorders such as the Gerstmann syndrome ship, neuropsychologists have formed similar profes-
( Gerstmann Syndrome). Over the next 2 decades, Ben- sional organizations throughout Europe, Asia, South
tons laboratory was home to numerous neuropsychology America, Australia, and Africa (see, for example, Jodzio,
pioneers including Max Fogel, Donald Shankweiler, Kerry 1998; Nihashi, 1998; Preilowski, 1997).
deS. Hamsher, Nils Varney, Scott Lindgren, Otfried Formal courses and organized programs of instruc-
Spreen, and Harvey Levin (Hamsher, 1985). Bentons tion in clinical neuropsychology began to appear in the
laboratory conducted important studies of aphasia using 1960s, with one of the first offered in the Biological
control group designs, psychological test construction Psychology Doctoral Program at the University of
methods, statistical analysis, and psycholinguistic theory. Oklahoma Health Sciences Center (Parsons, 1991). Qual-
The behavioral neurologist Norman Geschwind, along ity and content of instruction varied from program to
with his colleagues Davis Howes and Harold Goodglass, program as they multiplied in the 1970s. In 1979,
established an aphasiology center at the Boston Veterans Dr. Manfred Meier initiated an effort to establish stan-
Administration Hospital, continuing the more rigorous dards of training and competence for neuropsychologists
neuropsychological approach to language disorders. The in the USA. This effort culminated first in the establish-
Boston VA became a major center for neuropsychological ment of the American Board of Clinical Neuropsychology
training and research with Dr. Edith Kaplan famously (ABCN) in 1981. A year later, the American Board of
serving there as mother to a generation of Professional Neuropsychology (ABN) was established
594 C Clinical Neuropsychology
patients need to resist the temptation to be pulled to the patients are presented with a blank page and asked to
numbers 10 and 11. Thus, clock setting to ten after draw a clock with numbers and set the hands for ten
eleven tends to elicit a variety of stimulus-bound errors. after eleven, (3) a pre-drawn clock face where patients
In a survey of neuropsychologists and neurologists are asked to put in all the numbers and set the hands for
described by Freedman, Leach, Kaplan, Shulman, and 20 after 8, (4) a clock reading test with hands, but without
Delis (1994), other commonly used clock settings include numbers where patients are asked to identify a specified
20 after 8 and 3 oclock. time, and (5) a clock reading test with numbers and hands
where patients are, again, asked for the specified time. For
clock drawing to command and copy, behavior related to
Psychometric Data the drawing of the clock face, the numbers, and hands,
and the representation of the clock hands originating
Some scoring guidelines for the CDT can be found as part from the center of the clock are each scored using a
of the Boston Diagnostic Aphasia Examination supple- 13-point scoring system. The pre-drawn clock face condi-
mentary language tests (BDAE; Goodglass & Kaplan, tion is scored using an 11-point scoring system. Thus, for
1972, 1983; Goodglass, Kaplan, & Baresi, 2001). In this portion of the test scores have a 037 point range.
the original BDAE (with the rakishly purple cover [see Each clock reading subtest contains six test stimuli. Per-
Hollands forward to the third edition of the BDAE, formance on the three clock drawing test conditions are
2001]), the CDT test was one of several tests believed to combined with a score measuring the copy of a complex
be sensitive to parietal lobe injury. As described in the figure for a combined age-corrected scale score. The two
BDAE corpus, clock drawing is one of six figures where clock reading subtests are scored separately (range 06).
patients are asked to draw to command and copy (i.e., a For these two tests, age-related percentile cut scores are
clock with hands set for 10 after 11, a daisy, an elephant, provided.
a red cross, a three-dimensional cube, and a house). For Freedman, Leach, Kaplan, Shulman, and Delis (1994)
the clock drawing portion of the test, a three-point described a very comprehensive clock scoring system
scoring system was described awarding a point for an using a variety of clock drawing conditions and clock
approximately circular clock face, symmetry of number settings. Normative data was collected and grouped by
placement, and correctness of numbers. No scoring for decade from age 20 to 80. Separate scales were devel-
the representation of the clock hands was suggested. oped to assess the drawing of the clock face, the drawing
Normative data for the entire figure drawing test of the numbers, the presence and drawing of the clock
(range 013 for the separate command and copy test hands, and the degree to which the clock hands emanated
conditions) is provided. Separate normative data for from the center of the clock face. Base rates for a wide
the CDT is not included. Additional normative informa- range of clock drawing behavior are provided. These data
tion is provided by Borod, Goodglass, and Kaplan show that certain errors occur more frequently with age.
(1980). In this report, norms are also provided for two For example, for clock setting using ten after eleven, the
additional clock assessment procedures clock setting representation of the clock hands tends to be differentially
with numbers and clock setting without numbers. In affected by age.
both tests, the patient is presented with pre-drawn clock As noted above, a wide number of clock drawing
faces with and without numbers and are asked to draw the procedures have been reported (Lezak, Howison, & Lor-
hands to read 1:00, 3:00, 9:15, and 7:30. Performance is ing, 2004); however, most researchers follow Kaplans
assessed using a 12-point scoring system. Borod and col- (1988, 1990) suggestion and ask patients to set the
leagues (1980) described both age and educational effects hands for ten after eleven (Freedman et al., 1994). It is
for this clock assessment procedure. Other researchers important to understand that many CDT scoring proce-
have commented on the effects of education on clock dures are essentially atheoretical, that is, the administra-
drawing test performance (Marcopulos, McLean, & tion and scoring procedures were devised with an
Giuliano, 1997). eye toward sensitivity to brain damage or neurological
The Kaplan-Baycrest Neuropsychological Survey insult rather than to assess for deficits involving specific
(KBNS; Leach et al., 2000) contains a comprehensive cognitive constructs. Recent research, particularly in
clock test consisting of five parts: (1) clock drawing to using the CDT as part of a dementia evaluation, suggests
command where patients are asked to draw the face that the command and copy conditions are related to
of clock put in all the numbers and set the hands for different underlying cognitive mechanisms (Cosentino,
10 after 11, (2) a clock drawing to copy condition where Jefferson, Chute, Kaplan, & Libon, 2004; Libon, Swenson,
Clock Drawing C 599
Barnoski, & Sands, 1993; Libon, Malamut, Swenson, & in the command condition may be due to a deficit in
Cloud, 1996; Rouleau, Salmon, Butters, Kennedy, & visuospatial memory. Tranel, Rudrauf, Vianna, and
McGuire, 1992). Equally important, different results are Damasio (2008) administered the CDT to a large group
obtained depending on test instruction (see Cosentino of focal lesioned patients. Imaging studies found errors on
et al., 2004 for a review). These considerations are criti- the CDT were associated with right parietal (supramargi- C
cally important when the task at hand is to differentiate nal gyrus) and left inferior frontal-parietal opercular
between say, dementia subtypes. brain damage. These researchers also noted that visuospa-
tial errors were predominant in patients with right hemi-
sphere damage, whereas time-setting errors were
Clinical Uses predominant in patients with left hemisphere lesions.
Dementia As noted above, since the late 1980s, there
Focal lesions Clock drawing has not been extensively has been a plethora of research demonstrating the value
studied in non-dementia, focal lesioned patients. None- of the clock drawing test as both a screening test for
theless, specific patterns of deficits can be associated with dementia as well as a means of investigating cognitive
specific neurologic lesions. Freedman et al. (1994) and constructs that may differentiate between dementia sub-
Kaplan (1988, 1990) provide some instructive exemplars. types (see Cosentino et al., 2004 for a review). Rouleau
For example, patients with left posterior brain lesion et al. (1992, 1996) examined patients with Alzheimers
resulting in a Wernickes aphasia often present with lan- disease (VaD) and Huntingtons disease (HD) adminis-
guage comprehension deficits. While these patients may tering a clock drawing test to command and copy with
demonstrate general understanding of the clock drawing hands set for ten after eleven. An analysis of errors proved
instructions, numbers may be omitted entirely with hatch effective in differentiating between dementia subtypes.
marks used as substitutes (Freedman et al., 1994). Patients AD patients made more conceptual errors while HD
with a left anterior lesion presenting with a Brocas apha- patients produced more graphomotor errors. These
sia often have difficulty in understanding functor words authors speculated that semantic knowledge deficits
such as to and after. These patients, therefore, may be might underlie the deficits produced on the CDT by AD
apt to draw the clock hands pointing to the numbers 10 patients whereas executive dysfunction might underlie the
and 11. Further assessment is required to see if this kind errors produced by HD patients. When the command and
of error is caused by a language-related deficit or repre- copy conditions were compared, AD, but not HD patients
sents an executive deficit. Patients with left hemisphere improved from the command to copy test conditions.
lesions might initiate their drawing on the left side of the Rouleaus research was the impetus for a series of studies
clock, that is, on the side contralateral to their intact right conducted by Libon and colleagues (Cosentino et al., 2004;
hemisphere. Thus, numbers may be written correctly but Libon et al., 1993, 1996) that examined differences on
in a counterclockwise direction (Freedman et al., 1994). the CDT between patients with AD and vascular dementia
Interesting dissociations can be found in clock draw- (VaD). In their original study, Libon et al. (1993) found
ings to command versus copy in focal lesion patients. no difference in errors between AD and VaD patients in
Kaplan (1990) provides several instructive examples. An the command condition. However, similar to Rouleau,
analysis of clock drawings produced by a patient with a AD patients improved, that is, made fewer errors than
right parietal lesion demonstrates differential impairment VaD patients in the copy condition. These findings
in the copy versus the command test conditions. In the were replicated in a second study (Libon et al., 1996),
copy test condition, many numbers were omitted on the that is, AD patients generally improved from the com-
left side of the drawing. The clock drawing to command mand to copy test conditions compared to VaD patients.
did not demonstrate this behavior and was generally Cosentino et al. (2004) grouped dementia patients diag-
intact compared to the copy test condition. Kaplan nosed clinically with either AD or VaD on the basis of
(1988) demonstrated the opposite profile in a patient MRI white matter alterations (MRI-WMA). These groups
with a right temporal lesion. Here, there was differential were compared to dementia patients with Parkinsons
impairment in the command condition. For this patient, disease (PD). Patients presenting with minimal to mild
the clock drawing to copy was generally intact. For the MRI-WMA continued to improve from the command to
right parietal lesioned patient, the differential impairment copy test conditions, that is, produce fewer errors in the
in the copy condition likely reflected a deficit involving copy versus the condition test conditions, compared to
visually mediated neglect of left hemi-space. For the left patients with moderate to severe MRI-WMA and PD
temporal lobe patient, the visuospatial impairment seen patients. Errors produced the copy condition were
600 C C-Log
correlated with poor performance on executive tests. Libon, D. J., Malamut, B. L., Swenson, R., & Cloud, B. S. (1996). Further
analyses of clock drawings among demented and non-demented
Errors produced in the command condition were corre-
subjects. Archives of Clinical Neuropsychology, 11, 193211.
lated with overall dementia severity and tests related to Libon, D. J., Swenson, R., Barnoski, E., & Sands, L. P. (1993). Clock
semantic knowledge. Cahn-Weiner (2003) correlated CDT drawing as an assessment tool for dementia. Archives of Clinical
performance with MRI measures of atrophy and found Neuropsychology, 8, 405416.
that impaired CDT performance was attributable to im- Marcopulos, B. A., McLean, A., & Giuliano, A. J. (1997). Cognitive
impairment or inadequate norms: A study of healthy, rural, older adults
pairment in multiple cognitive domains but was primarily
with limited education. The Clinical Neuropsychologist, 11, 111131.
related to volume loss involving the right temporal cortex. Mayer-Gross, W. (1935). Some observations on apraxia. Proceeding of the
Taken as a whole, this research suggests that different Royal Society of Medicine, 28, 12031212.
cognitive constructs underlie impaired clock drawing in McFie, J., & Zangwill, O. L., (1960). Visuo-constructive disabilities asso-
patient with cortical versus subcortical dementia. ciated with lesions of the left cerebral hemisphere. Brain, 83,
243260.
Rouleau, I., Salmon, D. P., & Butters, N. (1996). Longitudinal analysis of
Cross References clock drawing in Alzheimers disease patients. Brain and Cognition,
31, 1734.
Rouleau, I., Salmon, D. P., Butters, N., Kennedy, C., & McGuire, K.
Constructional Apraxia (1992). Quantitative and qualitative analyses of clock drawings in
Alzheimers and Huntingtons disease. Brain and Cognition, 18, 7087.
Rubin, L. A., Barr, W. B., & Burton, L. A. (2005). Assessment practices of
References and Readings clinical neuropsychologists in the United States and Canada: A
survey of INS, NAN, and APA Division 40 members. Archives of
Benton, A., & Tranel, D. (1993). Visuoperceptual, visuospatial, and Clinical Neuropsychology, 20, 3365.
visuoconstructional disorders. In K. M. Heilman & E. Valenstien, Tranel, D., Rudrauf, D., Vianna, E. P. M., & Damasio, H. (2008). Does the
(Eds.), Clinical neuropsychology (3rd ed.). New York: Oxford clock drawing test have focal Neuroanatomical correlates? Neuropsy-
University Press. chology, 22, 553562.
Borod, J. C., Goodglass, H., & Kaplan, E. (1980). Normative data on the Tuokko, H., Hadjustavropoulos, T., Miller, J. A., & Beattie, B. L. (1992).
Boston diagnostic aphasia examination, parietal lobe battery, and the The clock test: A sensitive measure to differentiate normal elderly
Boston Naming Test. Journal of Clinical Neuropsychology, 2, 209216. from those with Alzheimer disease. Journal of the American Geriatrics
Cahn-Weiner, D. A., Williams, K., Grace, J., Tremont, G., Westervelt, H., Society, 40, 579584.
& Stern, R. A. (2003). Discrimination of dementia with lewy bodies Van der Horst, L. (1934). Constructional apraxia: Osychological views on
from Alzheimer disease and Parkinson disease using the clock draw- the conception of space. Journal of Nervous and Mental Disease, 80,
ing test. Cognitive and Behavioral Neurology, 16, 8592. 645650.
Cosentino, S., Jefferson, A. J., Chute, D. L., Kaplan, E., & Libon, D. L.
(2004). Clock drawing errors in dementia: Neuropsychological and
Neuroanatomic considerations. Cognitive and Behavioral Neurology,
17, 7483. C-Log
Freedman, M., Leach, L., Kaplan, E., Shulman, K. I., & Delis, D. C. (1994).
Clock drawing: A neuropsychological analysis. New York: Oxford
University Press.
Cognitive-Log
Goodglass, H., & Kaplan, E. (1972). Assessment of aphasia and related
disorders (1st ed.). Philadelphia, PA: Lea and Febiger.
Goodglass, H., & Kaplan, E. (1983). Assessment of aphasia and related
disorders (2nd ed.). Philadelphia, PA: Lea and Febiger. Clomipramine
Goodglass, H., Kaplan, E., & Baresi, B. (2001). Assessment of aphasia and
related disorders (3rd ed.). Philadelphia, PA: Lippincott, Williams. J OHN C. C OURTNEY
Head, H. (1926). Aphasia and kindred disorders of speech. New York:
Childrens Hospital of New Orleans
Macmillan.
Kaplan, E. (1988). A process approach to neuropsychological assessment. New Orleans, LA, USA
In T. Boll & B. K. Bryant (Eds.), Clinical neuropsychology and brain
function: Research, measurement, and practice. Washington, DC:
American Psychological Association. Generic Name
Kaplan, E. (1990). The process approach to neuropsychological assess-
ment of psychiatric patients. Journal of Neuropsychiatry and the
Clinical Neurosciences, 2, 7287. Clomipramine
Leach, L., Kaplan, E., Rewilak, D., Richards, B., & Proulx, G.-B. (2000).
The kaplan baycrest neurocognitive assessment. San Antonio, TX: The
Psychological Corp.
Brand Name
Lezak, M., Howison, D. B., & Loring, D. W. (2004). Neuropsychological
assessment (4th ed.). New York: Oxford University Press. Anafranil
Clonazepam C 601
Serious
Class
Paralytic ileus, hyperthermia, lowered seizure threshold
Anxiolytic
and rare seizures, orthostatic hypotension, sudden death,
arrhythmias, tachycardia, QTc prolongation, increased
intraocular pressure, hepatic failure, extrapyramidal
Proposed Mechanism(s) of Action
symptoms, mania, and suicidal ideation
Binds to benzodiazepine receptors at the GABA-A ligand-
gated channel, thus allowing for neuronal hyperpolariza-
Common tion. Benzodiazepines enhance the inhibitory action of
GABA via boosted chloride conductance.
Blurred vision, constipation, increased appetite, urinary
retention, dry mouth, nausea, diarrhea, heartburn,
strange taste in mouth, weight gain, fatigue, weakness, Indication
dizziness, headache, anxiety, nervousness, restlessness, se-
dation, sexual dysfunction, sweating Panic disorders (with or without agoraphobia), Lennox
Gastaut syndrome, akinetic seizures, myoclonic seizure,
absence seizures
References and Readings
Off Label Use
Physicians Desk Reference (62nd ed.). (2007). Montvale, NJ: Thomson
PDR.
Stahl, S. M. (2007). Essential psychopharmacology: The prescribers guide Atonic seizures and other anxiety disorders, acute psycho-
(2nd ed.). New York, NY: Cambridge University Press. sis, insomnia
602 C Clonidine
Hypertension
Common
Serious
Additional Information
Sinus bradycardia, atrioventricular block during with-
Drug Interaction Effects: http://www.drugs.com/drug_interactions.html
drawal, hypertensive, encephalopathy, cerebrovascular
Drug Molecule Images: http://www.worldofmolecules.com/drugs/
Free Drug Online and PDA Software: www.epocrates.com
accidents, and death
Gene-Based Estimate of Drug interactions: http://mhc.daytondcs.
com:8080/cgi bin/ddiD4?ver=4&task=getDrugList
Pill Identification: http://www.drugs.com/pill_identification.html Common
Generic Name Physicians Desk Reference (62nd ed.). (2007). Montvale, NJ: Thomson
PDR.
Stahl, S. M. (2007). Essential psychopharmacology: The prescribers guide
Clonidine
(2nd ed.). New York, NY: Cambridge University Press.
Brand Name
Additional Information
Duraclon, Catapres, Catapres-TTS, Clorpres
Drug Inter action Effects: http://www.drugs.com/drug_interactions.html
Drug Molecule Images: http://www.worldofmolecules.com/drugs/
Free Drug Online and PDA Software: www.epocrates.com
Class Gene-Based Estimate of Drug interactions: http://mhc.daytondcs.
com:8080/cgi bin/ddiD4?ver=4&task=getDrugList
Antihypertensive Pill Identification: http://www.drugs.com/pill_identification.html
Closure C 603
Common
Clorazepate
Sedation, fatigue, depression, dizziness, memory pro-
J OHN C. C OURTNEY blems, dysinhibition, confusion, ataxia, and slurred
Childrens Hospital of New Orleans speech.
New Orleans, LA, USA
C
Brand Name
Synonyms
Indication
Visual integration; Visual synthesis
Anxiety Disorder, symptoms of anxiety, and acute alcohol
withdrawal.
Definition
Off Label Use Visual closure refers to the ability to perceive and recog-
nize objects, shapers, features, or symbols from incom-
Partial seizures (as an adjunct). plete or degraded visual stimuli. It reflects the capacity of
humans to fill in missing information from incomplete
sensory input to achieve a meaningful percept.
Side Effects
functions (i.e., perception and cognition) are holistic impairments on tests such as the Gollin Figures and
consisting of analog processes that occur in a parallel Mooney Closure tests. This may reflect the fact that impair-
manner and are self-organizing. This led to the well- ments on this type of task tend to be embedded in other
known conclusion regarding perception, and cognition visual perception deficits that are more striking. However,
more generally, that the whole is greater than the sum it is also clear that closure paradigms have not been system-
of its parts. Based on this framework, all perceptual atically implemented into standard neuropsychological
processes act to achieve optimal organization and recon- batteries using modern computerized methods, so that
ciliation with the objects that are being perceived. A definitive conclusions regarding impairments of closure
critical principle driving Gestalt perception is pragnanz secondary to localized and global brain disorders cannot
(law of conciseness), which maintains that people orga- be reached at this point.
nize their experience in an orderly, symmetric, and simple
manner when possible. Visual closure was one of the
five laws of pragnanz, with others including the laws Cross References
of similarity, proximity, symmetry continuity, and com-
mon fate. Gollin Figures
While each of these laws has potential value in ac- Hooper Visual Organization Test
counting for elements of visual integration, the law of Simultanagnosia
visual closure seems to have had the most direct impact,
particularly with respect to clinical neuropsychology.
Early clinical studies of the effects of posterior cortical
lesions on visual perception indicated that certain patients
References and Readings
had difficulty in simultaneously processing all the ele-
Ellis, W. D. (1938). A source book of Gestalt psychology. New York:
ments of their visual sensorium to achieve a unified
Harcourt, Brace & World.
percept, a syndrome that was labeled simultagnosia Foreman, N. (1991). Correlates of performance on the Gollin and
(Poppelreuter, 1990). Mooney tests of visual closure. Journal of General Psychology, 118
(1), 1320.
Harlow, R. F. (1938). Philosophys contribution to Gestalt psychology.
Journal of Psychology: Interdisciplinary and Applied, 5, 185200.
Current Knowledge Holmes, D. S. (1968). Search for closure in a visually perceived pattern.
Psychological Bulletin, 70(5), 296312.
The idea that visual perception occurs as a function by- Jones, E. C., & Dennis, M. E. (1972). Perceptual closure as a function of
product of active self-organizing processes is now widely gap size. Perceptual and Motor Skills, 35(1), 126.
accepted by most visual scientists, though many would Kohler, W. (1929). Gestalt psychology. New York: H. Liveright.
Mooney, C. M., & Ferguson, G. A. (1951). A new closure test. Canadian
reject a pure holistic view. Instead, visual perception and
Journal of Psychology/Revue Canadienne de Psychologie, 5(3),
higher-order visual processes tend to be conceptualized as 129133.
the by-product of computational processes carried out by Poppelreuter, W. (1990). Disturbances of lower and higher visual capacities
modular neural networks responsible for specific opera- caused by occipital damage: with special reference to the psychopatho-
tions. Visual closure is thought to result from such pro- logical, pedagogical, industrial, and social implications. Oxford/New
York: Clarendon Press/Oxford University Press.
cesses occurring in extra-striatal systems found primarily
in the parietal cortex. Psychometric studies of closure
have tended to employ tests such as the Gollin Figures
and Mooney test (Foreman, 1991; Holmes, 1968; Jones &
Dennis, 1972; Mooney & Ferguson, 1951). In healthy Clot Buster
adults, the ability to recognize line drawings that have
been degraded has been shown to be a function of the Recombinant Tissue Plasminogen Activator
size of gaps in the drawing (Jones & Dennis, 1972), which
in turn reflects the amount of missing information. Per-
formance on closure tests has been shown to not be
strongly associated with visual search performance, sug-
gesting that these are distinct visual processes (Foreman, Clot Busting
1991). While tests of closure have existed for over 40 years,
there are relatively few studies demonstrating consistent Thrombolysis
Clustering C 605
Side Effects
Clotting
Serious
Thrombosis
Agranulocytosis, neuroleptic malignant syndrome, sei- C
zures, pulmonary embolism, myocarditis, hyperglycemia.
Clozapine Common
J OHN C. C OURTNEY 1, C RISTY A KINS 2 Increased risk for diabetes, sweating, and increased
1 salivation.
Childrens Hospital of New Orleans
New Orleans, LA, USA
2
Mercy Family Center References and Readings
Metarie, LA, USA
Physicians Desk Reference (62nd ed.). (2007). Montvale, NJ: Thomson
PDR.
Generic Name Stahl, S. M. (2007). Essential psychopharmacology: The prescribers guide
(2nd ed.). New York, NY: Cambridge University Press.
Clozapine
Additional Information
Class
Indication
Clustering
Schizophrenia (treatment-resistant), reduction of suicidal
M ICHAEL D. F RANZEN
behavior.
Allegheny General Hospital
Pittsburgh, PA, USA
References and Readings waves in the cochlea are transduced into bioelectrical
nerve impulses. The acoustic division of the eighth cranial
Brunton, L. B., Lazo, J. S., & Parker, K. L. (Eds.). (2005). Goodman & nerve has its cell bodies in the spiral ganglion of the
Gilmans the pharmacological basis of therapeutics (11th ed.). cochlea.
New York: McGraw Hill.
Stahl, S. M. (2008). Stahls essential psychopharmacology: Neuroscientific C
basis and practical applications. New York: Cambridge University
Press.
Cross References
Auditory System
CMS
References and Readings
Childrens Memory Scale
Ropper, A. H., & Brown, R. J. (2005). Deafness, dizziness, and disorders
of equilibrium. In Adams and Victors principles of neurology.
New York: McGraw Hill.
CNC
Coma/Near Coma Scale
Cochlear Nerve
Vestibulocochlear Nerve
CNS Lupus
Lupus Cerebritis
Cochlear Nuclei (Dorsal
and Ventral)
J OHN E. M ENDOZA
Cochlea Tulane University Medical Center
New Orleans, LA, USA
K ERRY D ONNELLY
University at Buffalo/SUNY
Buffalo, NY, USA
Definition
Cochlear nuclei is the nuclei that receive first-order
Definition auditory input from the organ of Corti in the cochlea of
the inner ear.
The cochlea, a small conical structure, is the part of the
inner ear that converts mechanical energy (vibrations)
into nerve impulses sent to the brain. It is also known as
the organ of hearing. The word cochlea is a Latin word Current Knowledge
derived from the Greek kokhlos, which refers to the land
snail. A coiled tube, the cochlea winds around a central The cochlear nuclei are divided into a dorsal and a
axis, forming the anterior part of the labyrinth. It contains ventral group. The dorsal cochlear nuclei give rise to
the organ of Corti, which includes the hair cells that the dorsal acoustic stria, which immediately cross the
constitute the primary mechanisms by which pressure midline and contribute fibers that ascend contralaterally
608 C Coding
offers several possible categories for diagnosing individuals (b) Undifferentiated Somatoform Disorder requires
with excessive cognitive symptoms (e.g., Malingering and one or more physical complaints, with no reference
Conversion Disorder); however, shortcomings of these con- made to cognitive difficulties.
ditions have been noted in the literature. In particular, (c) Conversion Disorder requires one or more symp-
Malingering has been the subject of considerable debate toms or deficits affecting voluntary motor or sensory C
and criticism, especially with regards to the objectivity with function [emphasis added], without mention of
which clinicians can assess if feigned symptoms were inten- cognitive or memory difficulties among the specific
tionally or unintentionally produced. Improvements have criteria.
been made in establishing criteria for this condition (e.g., (d) Pain Disorder requires only excessive pain
Slick et al., 1999), but clinicians nevertheless often remain symptoms.
reluctant to use any diagnosis that requires them to make (e) Somatoform Disorder NOS could conceivably in-
judgments about intentionality of symptom exaggeration. clude individuals with predominantly excessive cog-
Clinicians often face three general problems in trying nitive symptoms; however, soma denotes physical
to use existing DSM-IV categories to classify individuals rather than cognitive problems, and the list of exam-
with excessive cognitive symptoms. These problems in- ple cases provided in the DSM-IV for this catchall
clude (a) lack of a diagnostic category that adequately category makes no reference to excessive cognitive
targets the specific features of this relatively common symptoms.
condition, (b) the use of criteria that require the clinician (f) Dissociative Amnesia requires one specific type of
to make judgments about internal states that are exceed- cognitive problem, namely, an inability to recall
ingly difficult to evaluate in an objective manner (e.g., important personal information, usually of a trau-
intentional versus unintentional production of excessive matic or stressful nature [emphasis added]. Howev-
symptoms), and (c) difficulties in determining the relative er, individuals presenting with excessive cognitive
role that external incentive and sick-role factors may play symptoms do so in a myriad of ways (Bush et al.,
in the symptom production. 2005; Delis & Jacobson, 2000; Larrabee, 2003). Some
Symptom Specificity. The existing DSM-IV cate- people endorse problems in all cognitive domains
gories addressing excessive symptomatology can be queried, including attention, language, math, visual-
divided into two general types: symptom-specific versus spatial functions, higher-level executive functions,
symptom-nonspecific conditions. Symptom-specific con- new learning and memory, and remote recall of im-
ditions are those that require amplification of only certain portant personal information. In contrast, other
types of symptoms. The DSM-IV offers a relatively small individuals endorse difficulties in only one or a few
number of symptom-specific categories, which fall only specific cognitive skills (e.g., short-term memory and
among the somatoform disorders (e.g., Somatization Dis- concentration), while denying problems in other cog-
order and Conversion Disorder) and dissociative disor- nitive domains, including recall of important person-
ders (e.g., Dissociative Amnesia and Dissociative Fugue). al information. In fact, cases of isolated difficulty in
In addition, the DSM-IV offers two symptom-nonspecific remembering important autobiographical informa-
conditions, Malingering and Factitious Disorder, which tion are relatively rare, illustrating the limited utility
are discussed in the next section. of this diagnostic category for the vast majority of
A major problem in trying to subsume individuals cases with excessive cognitive symptoms.
with excessive cognitive complaints or invalid test perfor- (g) Dissociative Fugue not only requires one specific
mances into one of the symptom-specific diagnoses is that cognitive difficulty (inability to recall some or all
the cognitive symptoms of many of these cases simply fail of ones past), but carries the added stipulation that
to fit adequately in these categories. Following are expla- this difficulty must surface in the context of a sud-
nations of this problem for each of the symptom-specific den, unexpected, travel away from home or ones
categories provided in the DSM-IV: customary place of daily activities [emphasis
added]. These cases are extremely rare among indi-
(a) Somatization Disorder requires at least four pain
viduals presenting with excessive cognitive symp-
symptoms, two gastrointestinal symptoms, one sex-
toms, thereby precluding the use of this category
ual symptom, and one pseudoneurological symp-
for almost all cases.
tom. However, many individuals who present with
(h) Dissociative Identity Disorder is thought to occur
primarily excessive cognitive symptoms have few if
in individuals with multiple personalities in which
any physical complaints (Larrabee, 2005).
they exhibit an inability to recall important
610 C Cogniform Disorder
information about one or more personality states goal-directed motivation. An individual may be both
when they are in a different personality state. How- conscious of producing feigned behavior (e.g., is capable
ever, cases of multiple personalities are relatively of admitting to self and others that he or she is simulating
rare, particularly in clinicalneuropsychological symptoms) and motivated to do so for some type of
practice, and thus this diagnosis is seldom applicable personal gain; these features would meet criteria for a
to individuals with excessive cognitive symptoms. DSM-IV diagnosis of Malingering. However, someone
(i) Dissociative Disorder NOS is another catchall cate- may be largely unconscious of the feigned behavior (e.g.,
gory that, conceivably, could encompass individuals has convinced himself or herself that the excessive symp-
with excessive cognitive complaints. However, the toms are real), yet the feigned behavior may still arise due
tenor of this category is for individuals who exhibit to a specific, goal-directed purpose. For example, it was
an inability to recall personal information that was of noted during World War II that some soldiers, when faced
a traumatic or stressful nature, thereby greatly limit- with the prospect of entering the frontlines of battle,
ing the utility of this category for most cases of would develop psychogenic paralysis (what would now
excessive cognitive symptoms. be diagnosed as Conversion Disorder given that the symp-
tom amplification occurred primarily in the motor do-
Taken together, the aforementioned nine symptom-spe-
main). These individuals often appeared to truly believe
cific categories either fail to include cognitive complaints,
they were paralyzed, thereby suggesting an unconscious
target only highly specific, relatively rare types of cogni-
(conversion) process. However, their exaggerated behav-
tive problems, or require that other, qualitatively different
ior (paralysis) was clearly goal-directed, because it was
symptoms or conditions also be present (e.g., extensive
manifested in the context of an external incentive (avoid-
physical symptoms for Somatization Disorder). For these
ance of danger). In these cases, the conscious component of
reasons, these diagnoses generally fail to capture the vast
intentionality may have been absent, but the goal-directed
majority of individuals presenting with excessive cogni-
motivational component for producing the symptom was
tive symptoms.
likely present.
Intentionality. Another difficulty in using existing
In neuropsychological practice, the same type of disso-
DSM-IV categories has to do with required criteria related
ciation may occur in which individuals may produce exces-
to intentional/unintentional or voluntary/involuntary
sive cognitive symptoms in reaction to an external incentive
control over the production of the excessive complaints
(e.g., litigation), thereby suggesting goal-directed motiva-
or symptoms. For example, a key required criterion for
tion for the symptom production. However, these indivi-
the two symptom-nonspecific categories Malingering
duals may have nevertheless convinced themselves that their
and Factitious Disorder is that the clinician must deter-
symptoms are real, thereby suggesting a lack of a conscious
mine if the excessive symptoms were generated in an
component to the symptom production. Thus, for these
intentional or volitional manner. The problem here is
individuals, only certain components of intentionality may
that this criterion reflects a causative internal state that,
be present, with the lack of conscious awareness calling
for the majority of cases, is difficult if not impossible to assess
into question whether they would adequately meet the
in an objective manner. That is, the degree to which a person
required criteria for a diagnosis of Malingering. Another
may be exhibiting excessive symptoms or behaviors in an
complicating factor in the assessment of intentionality is
intentional, voluntary, or conscious manner versus an un-
that conscious awareness likely exists on a continuum, with
intentional, involuntary, or unconscious manner represents
individuals varying from being fully conscious, to semicon-
an untestable diagnostic hypothesis for many cases (see also
scious, to largely unconscious of the production of the
Slick et al., 1999). A clinician may have a hunch about
feigned behavior. Although an operational definition of
whether an individuals excessive complaints or symp-
intentionality is beyond the scope of this chapter, the im-
toms were under the voluntary or involuntary control of
portant point here is that intentionality of symptom pro-
the person, but usually these impressions are not substan-
duction not only refers to an elusive internal state, but
tiated by objective data, such as a disclosure or confession
it likely has component features that exist on a continuum
made by the individual to a clinician or other uninvolved,
(e.g., levels of conscious awareness), thereby making this
reliable third party.
construct exceedingly difficult for clinicians to assess in an
Another difficulty in this area of diagnosis is that
objective manner. Consequently, many clinicians are reluc-
intentionality is likely multifactorial in nature. For exam-
tant to use diagnoses such as Malingering, Factitious Disor-
ple, there may be at least two key components of inten-
der, and Conversion Disorder at least in part because of
tionality that can be dissociated: conscious awareness and
difficulty in objectively assessing the presence or absence
Cogniform Disorder C 611
of intentionality in the generation of the excessive As another example, some individuals may begin to
symptom. feign symptoms intentionally and consciously in reaction
External Incentive. A third difficulty in using existing to an external incentive (e.g., a lawsuit). However,
DSM-IV categories to diagnose individuals with excessive these individuals may gradually, and perhaps uncon-
cognitive symptoms is related to another required criteri- sciously, assume a progressively worsening sick role due C
on for the two symptom-nonspecific categories Malin- to (a) a prolongation in obtaining the external incentive
gering and Factitious Disorder regarding the presence or (e.g., caused by delays in the lawsuit); and (b) increased
absence of external incentive in the production of the skepticism and questioning on the part of family mem-
symptoms. Specifically, external incentive is a required bers, coworkers, or health providers about the authentici-
inclusionary criterion for Malingering and required exclu- ty of the individuals complaints. This prolonged scrutiny
sionary criterion for Factitious Disorder. (If there is an may be overwhelming to these individuals, compelling
absence of external incentive, then the clinician must them to adopt the sick role and exhibit illness behavior
make a further determination of whether or not an indi- in widespread areas of their lives, to the point where they
vidual has adopted the sick role in order to diagnose may even convince themselves of the authenticity of their
Factitious Disorders). However, the criterion of external symptoms. In other words, while the DSM-IV treats
incentive carries its own inherent difficulties for clinicians external incentive and sick role as mutually exclusive diag-
to identify when considering these diagnoses. First, for nostic criteria for differentiating Malingering and Facti-
many cases, practitioners may not have access to sufficient tious Disorder, in reality, as is the case for most psychiatric
background information about a persons life to be able to conditions, they may co-occur in varying degrees (Slick
assess if external incentives are operative in the case. That et al., 1999).
is, a practitioner may be unaware that a patient has or is Given these limitations in the DSM-IV, the following
planning to apply for disability or to initiate a civil lawsuit two diagnostic categories were proposed by Delis and
in the future, or has committed a crime and fears that he Wetter (2007) to encompass cases of excessive cognitive
or she may soon be apprehended. This lack of knowledge complaints or poor (invalid) test performances in the
about possible covert sources of external incentives makes absence of sufficient evidence of intentionality of symp-
it difficult to utilize the diagnoses of Malingering or tom production to warrant a diagnosis of Malingering.
Factitious Disorder for a number of cases, especially
given that such information is a required criterion rather
than an optional one for these categories. Neuropsychology of Cogniform Disorder
Second, as currently written, the DSM-IV criteria do
not allow for the possibility that a comorbidity may occur The essential feature of Cogniform Disorder is a pattern of
between the adoption of the sick role and the presence cognitive complaints or low scores on psychometric cog-
of external incentives (see also Slick et al., 1999). nitive tests that are considered to be excessive because they
For example, some individuals may gradually develop cannot be fully explained by a neurological disorder, by
into a progressively worsening sick role without the pres- another mental disorder that is associated with CNS dys-
ence of external incentives. However, after a period of function (e.g., schizophrenia), by a general medical con-
time, these individuals may present as so disabled that dition known to affect CNS function (e.g., renal disease),
they begin to receive disability payments, without neces- by the direct effects of a substance (e.g., opioid medica-
sarily having actively sought out such compensation. The tions), or by other factors known to affect cognitive func-
financial gain, however, likely buttresses and propagates tioning (e.g., developmental learning disorder, insomnia,
the continuation of the sick role. According to the and normal aging process). If the cognitive complaints or
DSM-IV, these individuals would have started out as poor test performances occur in the presence of a known
having Factitious Disorder, but as soon as the external neurological or mental disorder or any other factor
incentive was initiated and became a reinforcing factor, known to affect CNS function (e.g., medication), the
the diagnosis of Factitious Disorder would be called into cognitive symptoms are in excess of what would be
question (again, because external incentive is a required expected from the history, physical examination, labora-
exclusionary criterion for this condition). However, for tory tests, or psychometric validity testing. Findings from
these cases, the predominant causative factor for the ex- the clinical interview or psychometric testing of cognitive
cessive symptomatology may still be the adoption of the functions do not substantiate the degree of cognitive
sick role, with the external incentive playing a secondary complaints or symptoms because of the presence of at
or supportive role in the continuation of the symptoms. least two of the following features:
612 C Cogniform Disorder
(a) Cognitive complaints or poor test performances that reporting significant cognitive complaints and dysfunction
are rare for patients with documented mild to mod- in their daily lives.
erate generalized brain damage (e.g., loss of remote The primary distinguishing feature between Cogniform
autobiographical memories and inability to perform Disorder and Cogniform Condition (see below) concerns
overlearned verbal skills like reading, spelling, or the degree to which the individual presents as cognitively
simple math) impaired in widespread areas of his or her life. Specifically, a
(b) Inconsistencies between the individuals excessive diagnosis of Cogniform Disorder should be made if there is
cognitive complaints or poor test performances and reasonable evidence that the individual exhibits excessive
the relatively mild nature of the injury or illness as cognitive symptoms in most if not all areas of his or her life
documented in the medical records and seemingly at all times, thereby suggesting a conversion-
(c) Inconsistencies between the individuals excessive like adoption of the sick role manifested primarily as cogni-
cognitive complaints or poor test performances and tive dysfunction. In addition, in Cogniform Disorder, the
observed behavior degree of claimed disability in performing activities of daily
(d) Delayed onset of excessive cognitive complaints or living will often parallel the individuals complaints of cog-
symptoms after an injury and/or significant worsen- nitive dysfunction and poor (invalid) cognitive test perfor-
ing of symptoms over time without an adequate mance. For example, the individual not only obtains
explanation for the decline in functioning (e.g., severely deficient (and likely invalid) scores on tests of
subsequent neurological complications) visual-motor and visual-spatial functioning, but he or she
(e) Significant inconsistencies in cognitive test scores or also ceases to drive a vehicle because of the perceived cogni-
profiles across repeat evaluations tive problems. In many ways, Cogniform Disorder is analo-
(f) Patterns of cognitive test scores within an examina- gous to the somatoform condition of Conversion Disorder,
tion that are rare for brain-damaged patients but with the excessive symptoms manifested primarily in
(g) Significant inconsistencies in cognitive complaints or terms of cognitive dysfunction rather than deficits affecting
symptoms over time primarily motor or sensory functions (e.g., nonepileptic
(h) Evidence of insufficient test-taking effort or exagger- seizures). For this reason, Cogniform Disorder should be
ation on tests designed specifically to assess validity considered as a new subtype of the somatoform disorders.
of cognitive performance
(j) Evidence of insufficient test-taking effort or exagger-
ation on specific measures obtained from standard
Neuropsychology of Cogniform
ability tests that have been empirically found to assess
Condition
validity of cognitive performance
The essential features of Cogniform Condition are the
Considerable individual differences are found in the per- same as those of Cogniform Disorder in every respect,
formances of people with this condition on psychometric with the exception of the degree to which the individual
tests of cognitive skills (Larrabee, 2003; Slick et al., 1999). exhibits cognitive dysfunction in widespread areas of his
Some individuals obtain markedly low scores on most or her everyday life. That is, in Cogniform Condition,
cognitive tests administered; these individuals are often there is (a) a lack of reasonable evidence that the individ-
less sophisticated about medical and psychological condi- ual presents as cognitively dysfunctional in many areas of
tions and more blatant in their symptom amplification. his or her life, and (b) evidence of significant inconsis-
Other people may obtain low and invalid scores on only a tencies between the individuals excessive cognitive com-
few tests administered (e.g., memory tasks); these indivi- plaints or poor test performances in an evaluation and his
duals may be more subtle in their symptom exaggeration or her higher level of everyday functioning. For example,
and, as a result, more difficult to identify. Occasionally, an an individual may obtain severely deficient (and likely
individual may perform within expected ranges on most invalid) scores on tests of visual-motor and visual-spatial
cognitive tests administered, including cognitive validity functioning and yet continues to drive a vehicle without
tests, and yet continue to complain of extensive cognitive apparent difficulty. In other words, in Cogniform Condi-
problems and dysfunction in their daily lives. These indivi- tion, the individual is not given a diagnosis of disorder,
duals may have learned from other sources (e.g., Internet; because there is a lack of reasonable evidence that the
attorney coaching) that neuropsychological tests are capa- individual is acting out the sick role of being cognitively
ble of detecting poor test-taking effort, and consequently dysfunctional in widespread areas of his or her life despite
exert adequate effort on psychometric tests despite presenting to the clinician in a manner that suggests that
Cogniform Disorder C 613
he or she should be markedly impaired in everyday As proposed here, a diagnosis of Cogniform Disorder or
functioning. Cogniform Condition does not exclude the possibility of
intentional production of the excessive symptoms; rather,
these categories imply only that there is insufficient evidence
Cogniform Disorder and Condition Versus at the time of the assessment to formulate a diagnosis of C
Malingering: Similarities and Differences intentionality and therefore Malingering. Indeed, an ad-
vantage of having diagnostic categories such as Cogni-
Cogniform Disorder, Cogniform Condition, and Malin- form Disorder and Cogniform Condition is that they
gering (when manifested in the form of cognitive dysfunc- allow the clinician to label the cognitive symptoms as
tion) are similar in that the individual may present with excessive using more neutral terms that avoid the accusa-
excessive cognitive complaints or exhibit evidence of tory implications of Malingering when there is a lack of
inadequate effort and exaggeration on formal neuropsy- clear evidence to make that diagnosis. In addition, as
chological testing. However, a diagnosis of Cogniform discussed above, intentionality is likely multifactorial in
Disorder or Cogniform Condition should not be made if nature and is comprised of at least two key components:
there is reasonable evidence that the excessive cognitive conscious awareness and goal-directed motivation. The
symptoms are produced in an intentional or volitional individual who has convinced himself or herself that the
manner, in which case a diagnosis of Malingering may be feigned behavior is real may not be fully or even partially
warranted. As noted above, however, this determination conscious of his or her symptom amplification, but this
can be difficult to make for many cases due to inherent person may nevertheless have developed the symptoms in
problems in objectively assessing the internal state of the reaction to the presence of external or interpersonal
intentionality of simulated behavior. For this reason, it is incentives for personal gain. The categories Cogniform
likely that many cases of excessive cognitive symptoms Disorder and Cogniform Condition allow the clinician
would receive the more neutral diagnosis of Cogniform to acknowledge the presence of incentives that may
Condition, and possibly a diagnosis of Cogniform Disor- have played a significant role in the goal-directed motiva-
der if the individual exhibits cognitively dysfunctional tion for the excessive symptomatology without having to
behavior in widespread areas of his or her life. However, make the difficult determination of whether the individu-
when evidence emerges that implicates at least a conscious al is conscious or unconscious of these dynamics.
component in the production of the excessive cognitive
symptoms, then a diagnosis of Malingering (or Malin-
gered Neuropsychological Dysfunction; Slick et al., 1999)
References and Readings
may be warranted. Following are different examples of
Binder, L. M. (1993). Assessment of malingering after mild head trauma
evidence that can be supportive of a diagnosis of with the Portland Digit Recognition Test. Journal of Clinical and
Malingering: Experimental Neuropsychology, 15, 170182.
Binder, L. M., Storzbach, D., Anger, W. K., Campbell, K. A., & Rohlman,
(a) On psychometric testing, an individual obtains an D. S. (1999). Subjective cognitive complaints, affective distress, and
accuracy score on a forced-choice recognition memory objective cognitive performance in Persian Gulf War Veterans.
test that falls significantly below a chance level. Such a Archives of Clinical Neuropsychology, 14, 531536.
score provides empirical evidence that the individual Bush, S. S., Ruff, R. M., Troster, A. I., Barth, J. T., Koffler, S. P., & Pliskin,
N. H. (2005). Symptom validity assessment: Practice issues and
correctly remembered the right answers above a
medical necessity. Archives of Clinical Neuropsychology, 20, 419426.
chance level and used this knowledge to frequently Delis, D. C., & Jacobson, M. (2000). Neuropsychological testing. Encyclo-
select the wrong answer (Larrabee, 2003; Millis, 1992). pedia of Psychology. New York: American Psychological Association/
(b) A person who is involved in two separate personal- Oxford University Press.
injury lawsuits for different accidents complains of Delis, D. C., Kramer, J. H., Kaplan, E., & Ober, B. A. (2000). The
California verbal learning test (2nd ed.). San Antonio: The Psycho-
one set of symptoms and injuries to doctors asso-
logical Corporation.
ciated with one lawsuit and different symptoms and Delis, D. C., & Wetter, S. R. (2007). Cogniform disorder and cogniform
injuries to other doctors associated with the second condition: Proposed diagnoses for excessive cognitive symptoms.
lawsuit. Such selective reporting of symptoms that Archives of Clinical Neuropsychology, 22, 589604.
correspond to the different lawsuits suggests a con- Frederick, R. I. (1997). Validity indicator profile. Minnetonka: NCS
Assessments.
scious component to the symptom amplification.
Gervais, R. O., Russell, A. S., Green, P., Allen, L. M., Ferrari, R., & Pieschl,
(c) An individual confesses to intentionally per- S. D. (2001). Effort testing in patients with fibromyalgia and disabil-
forming poorly when taking cognitive tests. ity incentives. Journal of Rheumatology, 28, 18921899.
614 C Cognistat
Green, P., Rohling, M. L., Lees-Haley, P. R., & Allen, L. M. (2001). Effort for Level of Consciousness is also provided. The various
has a greater effect on test scores than severe brain injury in com-
subscales are modeled after more extensive and well-vali-
pensation claimants. Brain Injury, 15, 10451060.
Hom, J., & Denney, R. L. (2002). Detection of response bias in forensic
dated neuropsychological tests but in an abbreviated from.
neuropsychology. Journal of Forensic Neuropsychology, 2, 1166. For example, Attention is assessed using digit repetition
Iverson, G. L., & Binder, L. M. (2000). Detecting exaggeration and similar to that used on the Wechsler scales. Unlike other
malingering in neuropsychological assessment. The Journal of Head screening procedures that yield a single summary score,
Trauma Rehabilitation, 15, 829858.
Cognistat is designed to yield a score for each domain and
Iverson, G. L., & Franzen, M. D. (1996). Using multiple objective memory
procedures to detect stimulated malingering. Journal of Clinical and
thus produce a differentiated profile of cognitive abilities.
Experimental Neuropsychology, 18, 3851. Cognistat also employs an adaptive testing approach (re-
Larrabee, G. J. (2003). Detection of malingering using atypical perfor- ferred to as a screen and metric approach) to decrease the
mance patterns on standard neuropsychological tests. The Clinical time spent in administration. In this approach, an item that
Neuropsychologist, 17, 410425.
is of average difficulty is first administered for each subtest.
Larrabee, G. J. (2005). Assessment of malingering. In G. L. Larrabee (Ed.),
Forensic Neuropsychology. New York: Oxford University Press.
If that item is passed, no other items are administered from
Millis, S. R. (1992). The recognition memory test in the detection of that subtest, but if it is failed, additional easier items are
malingered and exaggerated memory deficits. The Clinical Neurop- administered. The raw scores for each subscale are then
sychologist, 6, 406414. plotted on a standard profile form, and performance is
Millis, S. R., Putnam, S. H., Adams, K. M., & Ricker, J. H. (1995). The
classified as being in the average range or as indicative of
California verbal learning test in the detection of incomplete effort.
Psychological Assessment, 7, 463471.
mild, moderate, or severe impairment.
Mittenberg, W., Patton, C., Canyock, E. M., & Condit, D. C. (2002). Base
rates of malingering and symptom exaggeration. Journal of Clinical
and Experimental Neuropsychology, 24, 10941102.
Slick, D. J., Sherman, E. M. S., & Iverson, G. L. (1999). Diagnostic criteria
Current Knowledge
for malingered neurocognitive dysfunction: Proposed standards for
clinical practice and research. The Clinical Neuropsychologist, 13, Because extensive validity and normative information
545561. were not available when Cognistat was originally pub-
Sweet, J. J. (1999). Malingering: Differential diagnosis. In J. J. Sweet (Ed.),
lished, a number of studies have subsequently examined
Forensic Neuropsychology. Lisse: Swets and Zeitlinger.
Tombaugh, T. N. (1996). Test of memory malingering. New York: Multi
its sensitivity to brain damage as well as the influence
Health Systems. of demographic variables on test performance. Research
revealed that the Cognistat may be more sensitive to brain
damage than the Mini Mental State Exam, Cognitive Ca-
pacity Screening Examination, and Mattis Dementia Rating
Cognistat Scale (e.g., Drane et al., 2003). Cognistat has also been
found to be sensitive to a variety of neurological and
DANIEL N. A LLEN
psychiatric disorders, and to age-related changes
University of Nevada Las Vegas
in cognitive abilities (for brief review see Doninger,
Las Vegas, NV, USA
Ehde, Bode, Knight, Bombardier, & Heinemann, 2006).
Correlations between its various subtests and neuropsycho-
logical measures of similar abilities provide evidence for its
Synonyms construct validity (Nabors, Millis, & Rosenthal, 1997).
The Cognistat battery also has limitations. For exam-
Neurobehavioral cognitive status examination (NCSE)
ple, performance is influenced by demographic variables,
including age and education. Although the original valida-
Description tion study of the Cognistat demonstrated no differences in
performance between age groups (Kiernan, Mueller,
The Cognistat test battery (Kiernan, Mueller, & Langston, Langston, & Van Dyke, 1987), subsequent investigations
1995), formerly called the Neurobehavioral Cognitive found that increased age is associated with poorer perfor-
Status Examination, is a screening tool designed to as- mance on the Construction, Memory, Similarities, Atten-
sess a number of different cognitive domains including tion, and Calculation domains, with Construction and
Orientation, Attention, Language Abilities (Comprehen- Memory appearing to be the most consistently impacted
sion, Repetition, Naming), Construction, Memory, Calcu- by age (Drane & Osato, 1997). Additionally, years of
lations, and Reasoning (Similarities, Judgment). A rating education and low levels of educational attainment are
Cognitive Affective Syndrome C 615
Definition
Cross References
First described by Schmahmann and Sherman (1997),
Mattis Dementia Rating Scale (DRS)
cerebellar cognitive affective syndrome (CAS) refers to
Mini Mental State Exam (MMSE)
a cluster of impairments involving higher-order cognitive
processes and affective functioning. Symptoms tend to
References and Readings cluster in executive dysfunction, including problems
with planning, set shifting, verbal fluency, abstract
Doninger, N. A., Ehde, D. M., Bode, R. K., Knight, K., Bombardier, C. H., & reasoning, perseveration, attentional dysregulation, hy-
Heinemann, A. W. (2006). Measurement properties of the neurobe- peractivity, impulsivity and disinhibition, and deficits in
havioral cognitive status examination (Cognistat) in traumatic brain working memory. However, symptoms may also include
injury rehabilitation. Rehabilitation Psychology, 51(4), 281288.
Drane, D. L., & Osato, S. S. (1997). Using the neurobehavioral cognitive
visuospatial disorders, expressive language disorders,
status examination as a screening measure for older adults. Archive of affective abnormalities, difficulties with visuospatial orga-
Clinical Neuropsychology, 12(2), 139143. nization, visual memory, logical sequencing, and blunted
Drane, D. L., Yuspeh, R. L., Huthwaite, J. S., Klingler, L. K., Foster, L. M., or inappropriate affect (Schmahmann & Sherman, 1997).
Mrazik, M., & Axelrod, B. N. (2003). Healthy older adult perfor-
mance on modified version of the Cognistat (NCSE): Demographic
issues and preliminary normative data. Journal of Clinical and Current Knowledge
Experimental Neuropsychology, 25(1), 133144.
Kiernan, R. J., Mueller, J., & Langston, J. W. (1995). Cognistat (Neurobe- Causes and Correlates of CAS
havioral Cognitive Status Examination). Lutz, FL: Psychological
Assessment Resources.
Kiernan, R. J., Mueller, J., Langston, J. W., & Van Dyke, C. (1987). The The co-occurrence of these cognitive and affective symp-
Neurobehavioral Cognitive Status Examination: A brief but quanti- toms arises from the disruption of neuroanatomical cir-
tative approach to cognitive assessment. Annals of Internal Medicine, cuits connecting the cerebellum with frontal, parietal,
107(4), 481485. temporal, and limbic cortices. Damage to these connec-
Macaulay, C., Battista, M., Lebby, P. C., & Mueller, J. (2003). Geriatric
performance on the Neurobehavioral Cognitive Status Examination
tions can occur in association with cerebellar infarct
(Cognistat) what is normal? Archives of Clinical Neuropsychology, 18, (Schmahmann and Sherman, 1997), cerebellar atrophy
463471. associated with severe alcoholism (Fitzpatrick, et al.,
Nabors, N. A., Millis, S. R., & Rosenthal, M. (1997). Use of the Neuro- 2008), cerebellar tumor or tumor resection (Levihson,
behavioral Cognitive Status Examination (Cognistat) in traumatic
et al., 2000; Konczak, 2005), trauma, neurodegenerative
brain injury. Journal of Head Trauma Rehabilitation, 12(3), 7984.
Schrimsher, G. W., Parker, J. D., & Burke, R. S. (2007). Relation between
disorders, or cerebellitis. Affective symptoms have been
cognitive testing performance and pattern of substance use in males associated with damage to the cerebellar vermis (Levihson,
at treatment entry. Clinical Neuropsychologist, 21(3), 498510. et al., 1997). Lesions of the anterior lobe of the cerebellum
616 C Cognitive Archives
tend to produce only minor changes in executive and The Planning scale consists of: matching numbers,
visualspatial functions. Children with a cognitive affective planned codes, and planned connections.
syndrome can also have autistic characteristics, and diag-
1. Matching numbers The individual is asked to
nosis of autism can be confounded by cerebellar lesions.
locate and underline a pair of matching numbers.
The task begins with 1 digit and progressively moves
References and Readings into 7 digit numbers.
2. Planned codes A client is requested to complete a
Schmahmann, J., & Sherman, J. (1998). The cerebellar cognitive affective series of boxes according to a corresponding code
syndrome. Brain, 121, 561579. provided at the beginning of each item.
Schmahmann, J., Weilburg, J. D., Sherman, J. B., & Janet, C. (2007). The
3. Planned connections This subtest is similar to
neuropsychiatry of the cerebellum - insights from the clinic. Cere-
bellum, 6(3), 254267.
the original trail making task. In this subtest, both
numerical and alphabetical sequences are employed.
The Attention scale encompasses: number detection,
expressive attention, and receptive attention.
Cognitive Archives 1. Number detection This subtest consists of rows of
numbers with both target and distracter stimuli.
Cognitive Correctors
At the top of each item page, a key is printed with
the target numbers. Children are instructed to under-
line only the target specified.
Cognitive Assessment System 2. Expressive attention Children aged 57 are to
identify the size of an assortment of animals, in spite
L EESA V. H UANG of the size depicted on the page. For children aged
California State University 817, this subtest is similar to the Stroop Test.
Chico, CA, USA Color words are presented in a different colors of ink
(e.g., the word red might be in blue ink) and the
children are requested to name the color of the ink.
Synonyms 3. Receptive attention First, a series of pictures or words
are presented in which the client must identify and
CAS underline identical stimuli. Second, children are
requested to recognize and identify two items that
Description share a common characteristic. This subtest is omitted
for the Basic Battery.
The DasNaglieri Cognitive Assessment System (CAS;
The Simultaneous Processing scale includes: nonverbal
Naglieri & Das, 1997a, 1997b) is a cognitive assessment
matrices, verbalspatial relations, and figure memory.
to assess children aged 5 years, 0 months to 17 years,
11 months. Individual administration time is approximate- 1. Nonverbal matrices A variety of pictures with
ly 1 h. The CAS is arranged in three separate, yet interrelat- geometrical shapes or patterns are shown to the
ed levels of scores: individual subtests, PASS (Planning, student. The student needs to select one option that is
Attention, Simultaneous, and Successive) composite scales, consistent with the presented relationship or pattern.
and a Full Scale quotient. Twelve subtests comprise the CAS 2. Verbal spatial relations The individual receives
and each subtest generates a scaled score (M = 10; SD = 3). auditory information and determines which picture
The Standard Battery utilizes 12 subtests with three subt- best represents the verbal description given. Presented
ests per PASS process, while the Basic Battery includes in a multiple-choice format, the series of pictures
eight subtests, two subtests for each PASS process. Each of allows the student to demonstrate understanding of
the four PASS composite scores (M = 100; SD = 15) is a logical, grammatical, and spatial information.
combination of the subtests included in each respective 3. Figure memory A client is required to trace geometric
process. Finally, the Full Scale Score (M = 100; SD = 15) is design previously observed, which is embedded within
the aggregate total of the four PASS cognitive processes a larger and more intricate geometrical design. This is
scales, which are equally weighed. omitted from the Basic Battery.
Cognitive Assessment System C 617
The Successive Processing scale consists of: word series, position to a collective whole (Naglieri, 2005). Successive
sentence repetition, sentence questions, and speech rate. Processing is described as the unidirectional, consecutive
organization of stimuli (Naglieri & Das, 2005).
1. Word series Individuals are instructed to repeat a
series of commonly used words in the same consecutive
order given. The difficulty level increases as the word C
list starts with two words and ends with nine words.
Psychometric Data
2. Sentence repetition This subtest demands that
The standardization sample of 2,200 children is represen-
individuals repeat sentences that gradually become
tative of the US population on nine criteria (Naglieri &
longer. The sentences in this subtest utilized color
Das, 1997b). The internal consistency of the CAS subtests
words to reduce the contextual meaning and possible
range from 0.75 to 0.89, with a median reliability of 0.82.
interference with simultaneous processing. The score
The Standard Battery had average reliabilities of 0.88
is based on the total number of correctly repeated
(Planning), 0.88 (Attention), 0.93 (Simultaneous), and
items.
0.93 (Successive). The reliability coefficient range for the
3. Speech rate This subtest is administered only to
Full Scale score was 0.950.97.
children aged 57. Children are verbally presented
The theoretical premise of the CAS was constructed
with a series of three word combinations and are
on a four-factor model; however, factor analyses
requested to repeat each combination as quickly and
generated empirical support for both three- and four-
as many times as possible within 30 s.
factor models depending upon the age category. Further
4. Sentence questions Administered to children aged 817
research has provided support for the CAS as a measure of
instead of speech rate. In an extension of the sentence
g. Weak correlations were found between the CAS
repetition task, this subtest requires that children re-
Standard Battery Full Scale and PASS scales (ranging
spond to a question about a nonsensical sentence.
from 0.37 to 0.67) with the Wechsler Intelligence Scale
for Children-third edition (WISC-III; Wechsler, 1991).
Historical Background Predictive validity was moderately established with cluster
and subtest scores from the WoodcockJohnson Psycho-
The CAS is one of few cognitive processing instruments Educational Battery-Revised Tests of Achievement
which incorporate a neuropsychological foundation. The (Woodcock & Johnson, 1989).
theoretical basis of the CAS is an extension of Alexander S.
Lurias work relating to the brains three functional units
Clinical Uses
(Naglieri, 1999, 2005). It was modified and refined by Das,
Naglieri, and Kirby into four processing components:
The purpose of the CAS is to provide an analysis of an
Planning, Attention, Simultaneous, and Successive Pro-
individuals cognitive abilities through the measurement
cessing, otherwise known as PASS, to explain differences
of the PASS processes (Naglieri, 2005). The authors
in cognitive processing of children (Das & Naglieri, 2001).
suggested that the CAS is a valuable alternative tool
The Planning subtests require individuals to engage in
to the traditional Wechsler or StanfordBinet scales,
a problem-solving sequence to complete novel tasks
when assessing individuals who may have attention-
(Naglieri, 1999). The development, selection, application,
deficit/hyperactivity disorders (ADHD), LD, mental
and evaluation of strategies are crucial to the success of
retardation, traumatic brain injury, serious emotional
performance (Naglieri & Das, 1997b). Subtests in the
disturbance, giftedness, and planning problems (Naglieri
Attention scale require a combination of three com-
& Das, 1997b). Furthermore, possessing an under-
ponents: focused, selective, and sustained attention
standing of an individuals PASS profile will provide
(Naglieri, 2005). Focused attention involves the act of
essential information for the selection and evaluation of
attending to presented stimuli in the environment.
instructional recommendations (Das & Naglieri, 2001;
Selective attention is the concentration of attention to
Naglieri & Das, 1997b).
chosen stimuli while disregarding nonessential or com-
peting stimuli. Sustained attention is the differential
effort (over time, especially) an individual applies to- References and Readings
ward task completion. Simultaneous Processing subtests
require the ability of an individual to incorporate and Das, J. P., & Naglieri, J. A. (2001). The Das-Naglieri cognitive assessment
comprehend unconnected entities and its relation/ system in theory and practice. In J. J. W. Andrews, D. H. Saklofske, &
618 C Cognitive Assessors
Treatment Procedures
Cognitive Behavioral Couples
Therapy A CBCT approach to treatment with both relationship
distressed and health impaired couples focuses on three
TAMARA G OLDMAN S HER factors: behavioral factors, affective/emotional factors,
Illinois Institute of Technology and cognitive factors. The behavioral component
Chicago, IL, USA includes increasing positive behaviors such as spending
more time together and decreasing negative behaviors
such as criticizing or nagging. In addition, because com-
Synonyms munication problems are the most commonly reported
presenting complaint of distressed couples, the behavior-
Behavioral marital therapy; CBCT; Cognitive behavioral al aspects of the treatment also typically involve a skills-
marital therapy; Couples therapy; Marital therapy oriented approach to communication change where the
Cognitive Behavioral Therapy C 619
value and skills of working together to solve a problem References and Readings
are the foci.
Affect is a focus of therapy insomuch as it is an Baucom, D. H., Shoham, V., Mueser, K. T., Daiuto, A. D., & Stickle, T.
indicator of significant relationship distress and for its (1998). Empirically supported couple and family interventions
for marital distress and adult mental health problems. Journal of
ability to direct the therapist in exploring links between C
Consulting and Clinical Psychology, 66, 5388.
the emotions of the partners and their behaviors. Affect Epstein, N. B., & Baucom, D. H. (2002). Enhanced cognitive-behavioral
can be approached with a skills approach in helping therapy for couples. Washington, DC: American Psychological
partners learn to express their own and listen to the Association.
other persons emotions and by linking specific emotions Epstein, N. H., Baucom, D. H., & Daiuto, A. (1997). Cognitive-behavioral
couples therapy. In W. K. Halford, & H. J. Markman (Eds.), Clinical
to specific relationship issues.
handbook of marriage and couples intervention (pp. 415449). West
The third factor in CBCT is cognition. As Epstein et al. Sussex, England: Wiley.
(1997) note, the importance of cognitive factors in Halford, W. K., & Markman, H. J. (Eds.). (1997). Clinical handbook of
relationship functioning lies in the fact that objectively marriage and couples intervention. New York: Wiley.
observable behavioral events are often subjectively experi- Keefe, F. J., Caldwell, D. S., Baucom, D. H., Salley, A., Robinson, E.,
Timmons, K., et al. (1996). Spouse-assisted coping skills training
enced quite differently by the partners. The therapist
in the management of osteoarthritic knee pain. Arthritis Care and
works to uncover underling cognitive factors shaping the Research, 9(4), 279291.
behavior and affect of the partners in order to increase Kiecolt-Glaser, J. K., & Newton, T. L. (2001). Marriage and health: His
understanding and promote behavioral change. and hers. Psychological Bulletin, 127(4), 472503.
Schmaling, K., & Sher, T. G. (2000). The psychology of couples and illness:
Theory, research, and practice. Washington, D.C.: American Psycho-
logical Association.
Efficacy Information Shadish, W. R., & Baldwin, S. A. (2003). Meta-analysis of MFT inter-
ventions. Journal of marital and family therapy, 29, 547570.
CBCT, and its predecessor, Behavioral Marital Therapy Shadish, W. R., & Baldwin, S. A. (2005). Effects of Behavioral Marital
(BMT) have been one of the most researched forms of Therapy: A Meta-analysis of randomized controlled trials. Journal of
couples therapy (Shadish & Baldwin, 2003, 2005). Results Consulting and Clinical Psychology, 73(1), 614.
Wilson, S. E. (2001). Socioeconomic status and the prevalence of health
of efficacy trials repeatedly demonstrate that those who
problems among married couples in later midlife. American Journal
receive either CBCT or BMT report less distress than those of Public Health, 91(1), 131135.
who receive no treatment and that this finding remains
not only for couples presenting with general marital
distress, but also for depression, agoraphobia, and alcohol
abuse (Baucom, Shoham, Mueser, Daiuto, & Stickle,
1998). Based upon recent meta-analyses of studies using Cognitive Behavioral Marital
CBCT, Shadish and Baldwin (2003, 2005) reported an Therapy
overall mean effect size ranging from 0.59 to 0.84 for
couples therapy generally, with no differential effective- Cognitive Behavioral Couples Therapy
ness across theoretical orientation found.
Qualifications of Providers
Cognitive Behavioral Therapy
CBCT can be conducted by a variety of treatment
providers, specifically trained in its use with various TARYN M. S TEJSKAL
populations. This includes neuropsychologists, clinical Virginia Commonwealth University Medical Center
psychologists, marriage and family therapists, counselors, Richmond, VA, USA
social workers, and clergy.
Definition
Cross References
Cognitive behavioral therapy (CBT) is a theoretical
Behaviorism framework based on the premise that a persons
Cognitive Behavior Therapy cognitions influence their emotions and behavior. CBT
620 C Cognitive Behavioral Therapy
provides considerable utility in addressing a variety of alleviation. To reduce the emotional experience of frustra-
common emotional consequences of neurological disor- tion, a neuropsychologist working with a brain-injured
ders including anxiety and depression, as well as behavior patient would encourage the patient to appreciate the
modification for brain injury survivors. gains made since the injury. In order to avoid unrealistic
expectations for post injury functioning that could create
frustration; the patient would be cautioned against com-
Historical Background paring present capabilities to preinjury functioning.
negative behavior (Epstein & Baucom, 2002). For exam- encouraging healthy compartmentalization of emotion
ple, family members may respond angrily toward a pa- (Epstein & Baucom, 2002). In cases of neurological diag-
tient who acts aggressively after a brain injury. Over time nosis or injury, emotions may either be exacerbated or
negative reciprocity pervades relationships, invading minimized. To enhance the expression of emotions, thera-
cognitions and emotions such that family members pists may ask probing questions to bring awareness to C
make global negative attributions about another persons emotional experience such as: What happens to you
intentions and behavior (Epstein & Baucom, 2002). when. . .What is it like for you when. . .How do you feel
An important aspect of CBT is skills-based training; as you listen to your son expressing his experience. . .
meant to enhance positive behavior and decrease negative (Epstein & Baucom, 2002). In this way, individuals and
behavior. In an instance where a person receives a diagnosis family members can be encouraged to share their emo-
of schizophrenia, a CBT clinician may intervene with a tional experience in the context of a safer therapeutic
family to teach communication skills. Empirical literature environment.
has discussed the detrimental impact of expressed emotion
(EE) in families with a schizophrenic family member.
Further, the family members may be overwhelmed and Efficacy Information
have many questions, concerns, and emotions they need
to express surrounding the diagnosis. Communication CBT has been empirically validated for the treatment of
skills training teaches families how to communicate more many disorders including anxiety (Barlow, OBrien, &
productively with one another about complex issues and Last, 1984), sexual dysfunction (Baucom, Shoham,
reduce detrimental patterns of interaction such as EE. Mueser, Daiuto, & Stickle, 1998), depression (Beach,
Sadeen, & OLeary, 1990), bipolar disorder, schizophre-
nia, and bulimia nervosa (Baucom et al., 1998). CBT is
Cognition-Focused Interventions often used in conjunction with medication for a variety of
mental health concerns. In addition, CBT effectively
The manner in which a person cognitively ascribes mean- enhances coping skills for adults with chronic illness
ing to behavior is an important factor in CBT. Therapy (Rybarczyk, DeMarco, DeLaCruz, Lapidos, & Fortner,
often focuses on reassessing and amending these 2001) and caregivers (Gallagher-Thompson, Lovett,
cognitions. Areas of inquiry often include attributions, Rose, McKibbin, Coon, et al., 2000).
expectancies, assumptions, standards, and beliefs. In Recently, advances in computer software have given
order to evaluate and modify existing cognitions, clinicians rise to computerized versions of CBT. Though computer-
intervene using guided discovery during which clients are ized cognitive behavioral therapy (CCBT) is not meant t o
asked to identify and evaluate their cognitions (Epstein & replace face-to-face therapy, it does provide an additional
Baucom, 2002). When a patient is depressed about a treatment option. CCBT allows clients to participate in
neurological diagnosis or injury, clinicians may intervene therapy when there is a paucity of available therapists,
at the cognitive level challenging catastrophic thinking the associated costs are prohibitive, or the prospect of
(e.g., I will never get better). A therapist may focus speaking to someone face-to-face seems off-putting. In
decreasing negative self talk (e.g., I am worthless the way 2006, the United Kingdoms National Institute of Health
I am now) by encouraging the patient to keep a journal of and Clinical Excellence (NICE) provided guidelines
thoughts occurred and the impact of the thoughts on the recommending CCBT as a result of randomized
patients mood (Epstein & Baucom, 2002). controlled trials for mild to moderate depression and
anxiety (NICE, 2006).
Emotion-Focused Interventions
Outcome Measurement
Interventions within the realm of emotions may range
from expanding minimized emotional experience to con- Many clinicians used standardized instruments to assess
taining heightened emotional experience. Clinicians may the presence and severity of a variety of neuropsychologi-
draw on a variety of strategies to access, heighten, or limit cal diagnoses. With regard to depression, clinicians may
emotional experience including normalizing emotional use self-report inventories such as the Beck Depression
responses, metaphor, acceptance of emotional expression, Inventory (BDI II; Beck, Steer, & Brown, 1996) Finally, the
enhancing tolerance for distressing emotions, and Beck Anxiety Inventory (BAI: Kabacoff, Segal, Hersen, &
622 C Cognitive Behaviorism
Van Hasselt, 1997) has shown considerable utility in Inventory and the State-Trait Anxiety Inventory with older adult
psychiatric outpatients. Journal of Anxiety Disorders, 11(1), 3347.
diagnosing anxiety and identifying the severity of the
Lazarus, A. A. (1971). Behavior therapy and beyond. New York: McGraw-
anxiety symptoms. Hill.
Lazarus R. S., & Folkman, S. (1984). Stress, appraisal and coping.
New York: Springer.
Qualifications of Treatment Providers Leahy, R. L. (Ed.) (1997). Practicing cognitive therapy: A guide to inter-
ventions. Northvale, NJ: Jason Aronson.
National Institute of Health and Clinical Intervention (NICE). (2006).
CBT can be used by a variety of treatment providers such Depression and anxiety computerized cognitive behavioral therapy
as neuropsychologists, clinical psychologists, marriage (CCBT). Retrieved on July 17, 2007 at http://guidance.nice.org.uk/
and family therapists (MFTs), counselors, and social TA97
workers. Treatment providers using CBT should have OFarrell, T. J., Choquette, K. A., Cutter, H. S. G., Brown, E. D., &
appropriate clinical training in the model. Further, McCourt, W. F. (1993). Behavioral marital therapy with and without
additional couples relapse prevention sessions for alcoholics and
clinical providers learning to use CBTas a conceptual frame- their wives. Journal of Studies on Alcohol, 54, 652666.
work to guide therapy should seek supervision from an Rybarczyk, B., DeMarco, G., DeLaCruz, M., Lapidos, S., & Fortner, B.
experienced individual trained in the model of CBT. (2001). A classroom mind-body wellness intervention for older
adults with chronic illness: Comparing immediate and one year
benefits. Behavioral Medicine, 27, 1527.
Cross References
Behaviorism
Behavior Modification Cognitive Behaviorism
Psychotherapy
Behaviorism
Barlow, D. H., OBrien, G. T., & Last, C. G. (1984). Couples treatment of Cognitive Control
agoraphobia. Behavior Therapy, 15, 4158.
Baucom, D. H., Shoham, V., Mueser, K. T., Daiuto, A. D., & Stickle, T. R.
(1998). Empirically supported couples and family therapies for
Controlled Attention
adult problems. Journal of Consulting and Clinical Psychology, 66,
5388.
Beach, S. R. H., Sadeen, E. E., & OLeary, K. D. (1990). Depression in
marriage: A model for etiology and treatment. New York: Guilford
Press.
Cognitive Correctors
Beck, A. T., Rush, A. J., Shaw, B. F., & Emery, G. (1979). Cognitive therapy
of depression. New York: Guilford. R ICK PARENTE
Beck, A. T., Steer, R. A., & Brown, G. K. (1996). Manual for the Beck Towson University
Depression Inventory-II. San Antonio, TX: Psychological
Towson, MD, USA
Corporation.
Beck, J. S. (1995). Cognitive therapy: Basics and beyond. New York:
Guilford.
Dattilio, F. M., & Freeman, A. (Eds.). (2003). Cognitive-behavioral strate- Synonyms
gies in crisis intervention (3rd ed.). New York: Guilford.
Ellis, A. (1975). A new guide to rational living. Englewood Cliffs: Prentice
Behavioral memory aids; Behavioral prothestics; Cogni-
Hall.
Epstein, N. B., & Baucom, D. H. (2002). Enhanced cognitive-behavioral
tive archives; Cognitive assessors; Cognitive monitors;
therapy for couples. Washington: American Psychological Association. Cognitive orthotics; Cognitive robots; Cognitive trainers;
Gallagher-Thompson, D., Lovett, S., Rose, J., McKibbin, C., Coon, D., External aids; Prosthetic devices
Futterman, A., & Thompson, L.W. (2000). Impact of psycho-
educational interventions on distressed family caregivers. Journal of
Clinical Geropsychology, 6, 91110.
Definition
Heider, F. (1958). The psychology of interpersonal relations. New York:
Wiley.
Kabacoff, R. I., Segal, D. L., Hersen, M., & Van Hasselt, V. B. (1997). A device, external to the mind, that enhances or replaces
Psychometric properties and diagnostic utility of the Beck Anxiety a memory or cognitive function.
Cognitive Functioning C 623
fine motor dexterity. Different cognitive functions are sup- radio or television nearby. Divided attention allows us to
ported by distinct cortical and subcortical brain regions. pay attention to two or more tasks simultaneously. At the
Disruption of neural processes in these brain regions can top of the hierarchy is alternating attention, which is the
result in a range of cognitive deficits and syndromes. most complex form of attention that involves shifting of
attention from one task to another.
Many neuropsychological measures have been
Historical Background designed to evaluate attention. One such measure is the
digit span test. This task assesses basic attention capacity
In the 1600s, Descartes, a philosopher, was one of the first through repetition of series of numbers of increasing
scholars to establish the idea that the brain controls be- length (e.g., 1-2-3-etc). Continuous performance tests
havior. In the late 1700s, Franz Gall, a forefather of phre- are computerized assessment tools that evaluate attention
nology (the study of behavior based on the size and shape capacity over an extended time period of 1015 min.
of the skull) helped identify that different parts of the Higher level attention skills can be examined using tests
brain regulate distinct aspects of thought, personality, and involving mental arithmetic, more complex mental arith-
behavior. Later on in the 1800s and 1900s,Wilder Penfield, metic, connecting numbers and letters in alternating
Hughlings Jackson, Paul Broca, and Carl Wernicke, to sequence (e.g., 1-A-2-B-3-etc), and resequencing of
name only a few researchers who contributed significantly numbers and letters in numeric and alphabetic order
to the field of neuropsychology, used epilepsy and lesion (e.g., transform 8-K-2 to 2-8-K).
models to delineate distinct neuroanatomical correlates of Attention problems can be seen across the lifespan.
cognitive and motor functions. More recently, functional In children, poor concentration, distractibility, and trou-
(e.g., fMRI, PET) and structural (e.g., MRI, CT) neuro- ble regulating behavior can be related to an Attention
imaging techniques have provided an even more well Deficit Disorder, a diagnosis that is made when pervasive
defined understanding of brain-behavior relationships. attention difficulties are demonstrated prior to age seven
years and are observable in at least two different environ-
ments. However, attention difficulties in children also
Current Knowledge may develop secondary to anxiety, language disorders,
or neurological disorders such as epilepsy. In adults, at-
Attention tention deficits may manifest secondary to a variety of
neurological and medical conditions, including sub-
Attention refers to the ability to concentrate on informa- arachnoid hemorrhage, epilepsy, dementia, head injury,
tion that is heard and seen in the environment in both diabetes, and hypothyroidism. Stress, depression, and med-
the presence and absence of distractions. It also allows ication side effects often contribute to attention problems
us to concentrate on two things at once, such as balancing in adults, as well.
a checkbook while talking on the phone. It is regulated
by the frontal lobes, although pathways involving the
pons, parietal lobe, and thalamus are involved in the Mental Processing Speed
mediation of attention, as well. Dysfunction along these
pathways can contribute to various types of attention Mental processing speed, a term used synonymously with
problems. reaction time, refers to the speed at which an individual
Attention is hierarchically organized, and disruption thinks and completes activities. It is largely regulated by
of the most basic attention skills leads to disruption of the frontal lobes and subcortical regions, and it has global
more complex attention abilities. Focused attention is the effects on cognition. That is, if mental processing speed is
most rudimentary level of attention that permits us to poor slowness also is observed in areas of attention, lan-
concentrate or be vigilant to something in the environ- guage, and spatial processing abilities. This mental slow-
ment for a very brief time period. Sustained attention ing is referred to as bradyphrenia.
refers to maintenance of concentration for minutes or Reaction time can be evaluated in several ways. Mea-
hours. Selective attention requires even greater attention sures requiring manual transcription of numbers and
capacity that allows us to attend to a particular task while shapes are very sensitive to bradyphrenia. Other tasks
filtering out irrelevant, background information. For ex- may involve rapid reading of color names and naming
ample, this form of attention is used when we read the of colors, timed symbol search, and rapid generation of
newspaper and are not distracted by noises such as the words.
Cognitive Functioning C 625
Current Knowledge
(e.g., age, education, gender, culture), injury-related (e.g., rapid recovery than diffuse injury (Sohlberg & Mateer,
time since injury, extent, and severity of injury), and 2001).
psychological characteristics (e.g., therapeutic alliance,
comorbid psychological disorders, awareness).
Psychological Factors C
Demographic Variables Therapeutic Alliance: CR should be an interactive partner-
ship between the client, their significant others, and the
Age : Younger adults show better levels of recovery therapist. Cultivating a relationship characterized by
than older adults (Teuber, 1975 as cited by Sohlberg & attentiveness, respect, trust, commitment, and rapport is
Mateer, 2001). ABI in older adults may be complicated a critical component of CR. Open communication and
by a number of factors, including the superimposition involvement of the client and family in goal setting can
of effects of ABI on declining cognitive abilities (Richards, also enhance engagement in rehabilitation (Sohlberg &
2000 as cited by Sohlberg & Mateer, 2001), and psycho- Mateer, 2001).
social difficulties more prevalent in the population, Comorbid Psychological Disorders: Depression and
including reduced levels of social support and financial anxiety are frequently associated with brain injury (e.g.,
resources (Goleburn & Golden, 2001). However, it Anson & Ponsford, 2006). These can impede CR and
has also been suggested that older adults often have a adjustment following injury due to their propensity
greater degree of stability, coping skills, fewer life to decrease motivation and contribute to a feeling of
demands, and effective compensatory techniques, which hopelessness (Sohlberg & Mateer, 2001).
may be helpful to promoting recovery (Sohlberg & Awareness: Lack of awareness can occur following
Mateer, 2001). brain injury, and has been associated with poor self-
Education and Intelligence: Premorbid intelligence and regulation, disengagement in CR programs (Allen &
education are significantly related to recovery and adjust- Ruff, 1990), and poorer outcome.
ment (Anson & Ponsford, 2006). Although these factors provide important informa-
Gender: Some research suggests that women have tion related to the pattern and degree of recovery follow-
better recovery following left hemisphere lesions than ing brain injury, it should also be noted that much more
men (Kimura, 1983), and circulating sex hormones have research is needed to better identify therapy factors and
also been shown to have neuroprotective effects (e.g., client characteristics that optimize clinical outcomes of
Roof, Duvdevani, & Stein, 1993). CR (Cicerone et al., 2005).
Culture: Culture influences beliefs regarding the
nature and cause of loss, service utilization, degree of
personal responsibility for health, role of family, and Treatment Procedures
many other facets of psychological and behavioral
functioning relevant to recovery and participation in CR Examples of domains that have been a focus of CR
(Sohlberg & Mateer, 2001). include: attention, memory, language, visuoperceptual
difficulties, executive functions, and socioemotional
and behavioral disturbances. CR encompasses a range
Injury Related Variables of interventions. These can be broadly divided into
two types of techniques. The first are those that aim to
Time since Injury: Spontaneous recovery typically occurs restore or enhance function, by targeting the underlying
at a faster rate immediately following brain injury, partic- impairment (Glisky & Glisky, 2002; Sohlberg & Mateer,
ularly within the first 6 months, with significant recovery 2001). For example, Attention Process Training (APT) is a
also occurring up to 2 years following injury (Sohlberg & theoretically-driven program that contends that attention
Mateer, 2001). However, it is important to note that can be improved through repeated activation of atten-
compensatory techniques can be implemented and tional systems (Sohlberg & Mateer, 1987, 2001). APT
underlying motor and cognitive skills improved years consists of a group of hierarchically organized tasks
after injury (e.g., Shaw et al., 2005). that exercise different components of attention (e.g.,
Extent and Severity of Injury: Relatively mild injuries sustained, selective, alternating, divided attention). In
are associated with faster recovery rate and better CR of memory deficits, restorative/generalized memory
outcomes. Focal injuries are often associated with more approaches aim to improve specific memory systems
630 C Cognitive Rehabilitation
across tasks and contexts (e.g., prospective memory train- compared to a number of alternate treatment conditions,
ing, Raskin & Sohlberg, 1996 as cited by Sohlberg & with no comparison demonstrating a benefit for an
Mateer, 2001). Various approaches to executive function alternate treatment condition.
rehabilitation provide practice in executive skills (e.g., Although implementation of CR programs results in
planning) and guiding behavior through self-talk positive change, a number of methodological problems
(e.g., self instructional training, Cicerone & Giacino, 1992). have been identified in the CR literature, including but
The second category of CR interventions is compen- not limited to:
satory techniques, which aim to compensate for, or bypass
1. Variability in client characteristics and treatment
deficits (Sohlberg & Mateer, 2001; Wilson & Zangwill,
settings
2003). These include environmental supports (e.g.,
2. Insufficient description of samples
organization of physical space, manipulation of physio-
3. Small sample sizes
logical factors such as sleep, nutrition etc.) and external aids
4. Inadequate description of interventions
(e.g., calendars, pagers, checklists, etc.; Manly, Ward, &
5. A lack of standardized treatment protocols and
Robertson, 2002; Wilson & Zangwill, 2003). Compen-
treatment approaches (including type of intervention,
satory techniques can be helpful in managing diverse
length, and intensity)
types of cognitive difficulties (Sohlberg & Mateer, 2001).
6. Lack of appropriate control conditions (e.g., no
A third approach involves the use of specialized
treatment or alternate treatment)
approaches to teaching and stabilizing new behaviors
7. Lack of uniform outcome measures
and knowledge in people with memory difficulties.
These include instructional techniques such as errorless Evidence-based standards of CR are frequently
learning, in which mistakes are minimized (Wilson, identified as important in advancing the field of CR, both
Baddeley, Evans, & Shiel, 1994), the method of vanishing in terms of quality of treatment and for fiscal support at
cues (Glisky & Glisky, 2002) and traditional behavioral an organizational level (e.g., Sohlberg & Mateer, 2001).
shaping and training techniques. Cicerone et al. (2005) reviewed evidence for CR inter-
Psychosocial support or psychotherapy (e.g., sup- ventions used with TBI and stroke populations. A number
ported listening, brain injury education, relaxation training) of interventions with strong empirical support were
can also be an integral part of a rehabilitation program, identified. Strategy training (e.g., APT or Time Pressure
depending on the needs of the client (Sohlberg & Mateer, Management) was effective during postacute rehabilita-
2001). Computer programs can be used as an adjunct tion for TBI. A number of visuospatial rehabilitation
to CR, but should not be the sole form of CR (Cicerone techniques were empirically supported for improving ne-
et al., 2005). It has been recommended that computer glect following right hemisphere stroke (e.g., visuospatial
CR programs be focused, structured, monitored, and rehabilitation, scanning training). Specific gestural or
ecologically valid. A successful rehabilitation program strategy training was found to be effective in the treatment
typically involves a combination of interventions, speci- of apraxia following left hemisphere stroke. A number of
fically tailored to the individuals level of disability language interventions were shown to have empirical
and personal goals (Manly et al., 2002; Solhberg & support, including cognitive linguistic therapies,
Mateer, 2001). pragmatic conversational skills, interventions for specific
The duration and frequency of CR varies widely (e.g., language impairments (e.g., reading comprehension), and
Geusgens, Winken, van Heugten, Jolles, & van den adjunctive computer-based interventions. Strong empiri-
Heuvel, 2007; Kurtz & Nichols, 2007). CR has been cal support was shown for memory strategy training (e.g.,
delivered both on an individual and on a group basis. visual imagery and external aids, such as memory
Significant others (e.g., family) are viewed as an integral notebooks). Some empirical support was found for self-
part of treatment (Sohlberg & Mateer, 2001). instruction and self-monitoring interventions for
executive functions. Empirical support was found for
comprehensive-holistic CR programs, which address
Efficacy Information multiple aspects of impairment.
Interventions that meet criteria for inclusion as an
Cicerone et al. (2005) examined differential treatment empirically supported treatment do not represent an
effects of CR compared to alternate treatment conditions exhaustive list of CR interventions that may be effective,
in 46 class I studies with TBI and stroke populations. but reflect the empirical status of the field of CR research
Their review suggested a clear differential benefit of CR and practice. A successful rehabilitation program typically
Cognitive Rehabilitation C 631
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3858). London: The Guilford Press. training program. Journal of Clinical and Experimental Neuropsy-
Chambless, D. L., & Ollendick, T. H. (2001). Empirically supported chology, 19, 117130.
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Review of Psychology, 52, 685716. integrated neuropsychological approach. New York: Guilford Press.
Cicerone, K. D., Dahlberg, C., Malec, J. F., Langenbahn, D. M., Wilson, B. A., Baddeley, A. D., Evans, J. J., & Shiel, A. (1994). Errorless
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Cicerone, K. D., & Giacino, J. T. (1992). Remediation of executive tation: Theory and practice. Exton, PA: Psychology Press.
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Clare, L. (2003). Cognitive training and cognitive rehabilitation for
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Synonyms
schizophrenia: A review of recent advances. Current Psychiatry
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Laatsch, L., Harrington, D., Hotz, G., Marcantuoro, J., Mozzoni, M. P., &
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behavioral rehabilitation treatment studies in children with ac-
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Cognitive reserve is a concept often used to describe how
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interventions: Clinical research and practice (pp. 80104). London:
individual differences mediate the clinical expression of
The Guilford Press. brain damage. In this context, some individuals may cope
Manly, T., Ward, S., & Robertson, I. (2002). The rehabilitation of atten- better than others and function within relatively normal
tion. In P. J. Eslinger (Ed.), Neuropsychological interventions: Clinical limits, despite the presence of neuropathology.
research and practice (pp. 105136). London: The Guilford Press.
OBrien, A., Chiaravalloti, N. D., Govereover, Y., & DeLuca, J. (2008).
Evidenced based cognitive rehabilitation for persons with multiple
sclerosis: A review of the literature. Archives of Physical Medicine and Historical Background
Rehabilitation, 89, 761769.
Ponsford, J. (2004). Introduction. In J. Ponsford (Ed.), Cognitive and
behavioral rehabilitation: From neurobiology to clinical practice
Historically, one of the earliest observations of cognitive
(pp. 16). London: The Guilford Press. reserve was described in a study that found characteristic
Roof, R. L., Duvdevani, R., & Stein, D. G. (1993). Gender influences senile plaques and neurofibrillary tangles commonly asso-
outcome of brain injury: Progesterone plays a protective role. Brain ciated with Alzheimers pathology present in healthy, cog-
Research, 607, 333336.
nitively unimpaired elderly (Blessed, Tomlinson, & Roth,
Shaw, S. E., Morris, D. M., Uswatte, G., McKay, S., Meythaler, J. M., &
Taub, E. (2005). Constraint-induced movement therapy for recovery
1968). Similar observations between brain pathology and
of upper-limb function following traumatic brain injury. Journal of performance variability frequently have been described in
Rehabilitation Research and Development, 42, 769778. the extant literature.
Cognitive Stimulation C 633
the presence or absence of comorbid illnesses, and of the cerebrum may cause difficulty with pragmatics,
the nature of specific individual variables such as age, context-sensitive semantics, and expressive affective pros-
gender, and psychological state. In the absence of con- ody, obscuring indications of mood and compromising
founding circumstances, improvement of CCD is antici- the ability to communicate successfully in social situa-
pated when caused by stroke, excisable tumor, remitting tions. Egocentrism may impair recognition of these pro- C
disease, or traumatic brain injury. When caused by pro- blems and reduce insight into the communication needs
gressive debilitating conditions such as non-excisable of others. Deficits in vigilance, sustained and selective
tumors or dementing diseases, CCD will worsen over attention, and/or in attention switching can cause salient
time. CCD of greater initial severity has a poorer prog- information to be missed, and reduce the sequential
nosis than CCD of mild or moderate initial severity, or simultaneous processing of information from multiple
although in the case of recovery from TBI, the degree sources. Difficulties drawing inferences, extracting themes
of functionality at hospital discharge may be more pre- and topics in discourse, interpreting nonliteral language,
dictive than the initial severity of injury (Testa, Malec, and/or reading affective and prosodic cues for mean-
Moessner, & Brown, 2005). The presence of co-occurring ing during conversational exchanges may be present
illnesses may compromise the speed and extent of recov- (Ylvisaker et al., 2008). A tangential communication
ery from CCD at any severity level; CCDs arising from style often emerges, where excessive, vaguely relevant
traumatic injuries causing diffuse brain damage, for ex- details are inserted inappropriately into narratives and
ample, are likely to be accompanied by paralysis, motor discourse (Myers & Blake, 2008).
speech disorders, and injuries to vital organs. Damage to the right parietal association cortex and to
Recovery from CCD differs by etiology. Re- the right parietal-temporal-occipital (PTO) cortex can
covery after stroke is usually most rapid in the first lead to contralateral inattention, impeding reading,
3 months post onset. Recovery from thrombo-embolic writing, and listening for stimuli in left hemispace.
stroke may continue after 6 months post onset, where- Lesions in PTO cortex can also cause visual-spatial per-
as recovery from hemorrhagic stroke may plateau at the ception and recognition deficits (including topographical
6-month point. Functional improvement in cognitive- and geographical agnosias), which can impair navigation
communication abilities after severe traumatic brain in- in familiar environments if verbal mediation strategies are
jury is generally slower at the outset when compared not used to compensate. Damage to secondary (superior
with stroke and typically proceeds in a stairstep fashion temporal) association cortex may reduce the efficiency of
over months or years. Recovery from mild stroke or auditory language processing if stimuli are complex or if
brain injury often seems rapid in comparison. Many they must be processed quickly. Damage may also impair
individuals with mild brain injuries appear to recover the interpretation of affective prosody when produced by
quickly (Ylvisaker et al., 2008), but as many as 1520% others. Lesions to prefrontal, parietal, and temporal cor-
suffer from persistent fatigue and reduced information tex have been associated with anosognosia, the failure to
processing speed for years after injury. Frequently unrec- recognize the existence or presence of illness and a prob-
ognized and untreated, these deficits cause lifelong cogni- lem that can reduce compliance with treatment activities
tive challenges that threaten social adjustment and (Myers, 2001; Myers & Blake, 2008; Tomkins, 1995).
successful community reentry. In contrast to stroke and When damage to the cerebral cortex is bilateral, def-
TBI, recovery from dementing illness is not expected. Early icits across multiple systems may interact to impair com-
stages of cognitive decline in DAT are accompanied by munication to different degrees. Bilateral cortical
forgetfulness, word-finding difficulties, and changes in so- damage, for example, can cause impairments in the self-
cial pragmatics; mid stages are characterized by increased regulation of communicative behaviors that range from
memory loss, anomia, and social withdrawal; and late failure to organize discourse efficiently to failure to in-
stages are associated with loss of most useable cognitive hibit inappropriate utterances and actions. It can also
and physical functions (Bayles & Tomoeda, 2007). diminish the ability to focus attention and memory so
as to make them useful during conversational exchanges
(Ylvisaker et al., 2008). However, if bilateral damage
Neuropsychology and Psychology of involves subcortical hippocampal structures, then funda-
Cognitive-Communication Disorder mental disruptions of declarative (semantic, episodic, and
lexical) and explicit memory may occur. Hippocampal
Damage to the prefrontal and frontal association regions damage/deterioration is common with brain injury/
of the right (or nonlanguage-dominant) hemisphere dementing disease, and it can lead to a host of
636 C Cognitive-Communication Disorder
impairments ranging from difficulty learning new infor- 5. The ability to discriminate relevant from irrelevant
mation to the presence of a severe, unremitting amnesia. detail and integrate disparate parts into a coherent
Bilateral damage to lower brain stem reticular activat- whole can be evaluated with scene description tasks.
ing circuits can severely compromise arousal, alertness, 6. Cognitive flexibility and functional problem-solving
and awareness. This can lead to brief losses of conscious- abilities can be assessed with tasks that require the
ness or to intractable coma. When caused by dementing generation of multiple strategies for achieving a goal
disease, coma is most likely followed by death. However, and that require repairs of failed communicative inter-
recovery from traumatic coma frequently leads to return changes with others (Myers, 1999).
of function through increasing levels of responsivity,
Standardized tests used to assess cognitive-communication
communication, orientation, self-regulation, and cogni-
functions subsequent to right hemisphere impairment in-
tive integration. A common sequela of traumatic brain
clude the Mini-Inventory of Right Brain Injury (Pimental &
injury is a period of post-traumatic amnesia (PTA), that
Kingsbury, 1989), The Rehabilitation Institute of Chicago
is, inability to form new memories of events happening
Clinical Management of Right Hemisphere Dysfunction
after brain injury with disorientation to time, place, or
(Halper, Cherney, & Burns, 1996), and The Burns Brief
person. In individuals who are verbal, confabulation may
Inventory of Communication and Cognition (Burns, 1997).
be present until disorientation and confusion diminish.
While these instruments will elicit symptoms of CCD
As cognitive functioning improves, individuals with brain
associated with right hemisphere damage, findings must
injuries will benefit from environmental structure and
be interpreted with a view toward evaluating impair-
external direction to support increasingly purposive, flex-
ments of underlying neuropsychological processes, for it
ible, and goal-oriented behavior.
is here that treatment will be most profitably directed
(Myers, 1999).
Standardized tests used to assess CCD after TBI in-
Evaluation
clude the American Speech-Language-Hearing Association
Functional Assessment of Communication Skills in Adults
Evaluation of a suspected CCD requires examination of
(Frattali, Thompson, Holland, Wohl, & Ferketic, 1995),
cognition as it affects and interacts with skills of speech
the Behavioral Rating Inventory of Executive Function
planning and execution, language comprehension and
(Roth, Isquith, & Gioia, 2005), the Brief Test of Head
production, and pragmatic/discourse aspects of commu-
Injury (Helm-Estabrooks & Hotz, 1991), the Ross Infor-
nication in everyday social contexts (Turkstra, Cohelo, &
mation Processing Assessment-2 (Ross-Swain, 1996), and
Ylvisaker, 2005).
the Scales of Cognitive Ability for Traumatic Brain Injury
Tests of cognitive-communication skills are used to
(Adamonovich & Henderson, 1992). The Glasgow Coma
evaluate the effects that deficits in attention, orientation,
Scale (Jennett & Teasdale, 1981) and the Rancho Los
perception, memory, organization, and executive func-
Amigos Levels of Cognitive Function Scale (Hagen,
tions can have on communication. Examples of tasks
Malkmus, Durham, & Bowman, 1979) or its revisions,
frequently included in formal and informal assessments
including a version adapted for children under 14 years of
are provided for illustration:
age (Blosser & DePompei, 2003), are frequently used to
1. Attention to left hemispace is frequently tested with assess consciousness and track recovery of cognitive func-
line-bisection, cancellation, and drawing tasks as well tions after coma.
as with more complex reading, writing, and listening Informal situational assessments of cognition and
tasks that require individuals to process communica- communication may yield valuable information that can-
tion stimuli from both right and left sides of body not be obtained from formal standardized tests (Blosser &
midline. DePompei, 2003; Turkstra et al., 2005). This naturalistic
2. Inferencing abilities are tested by asking patients to approach to assessment has been termed, functional,
interpret humor, to recognize indirect requests for collaborative, context-sensitive, hypothesis-testing assess-
actions, and to follow the themes of conversations. ment, and it should be conducted with the purpose of
3. Orientation is assessed by asking patients to respond identifying situational variables that can be manipulated
to questions about time, place, and person. to improve the successful participation of injured indivi-
4. Memory for facts, events, and procedures is evaluated duals as they operate within their everyday environments
with yes/no questions, narratives, and performance of (Ylvisaker et al., 2008). Best-practice assessment proce-
familiar routines. dures for individuals who have suffered brain injuries
Cognitive-Communication Disorder C 637
should, therefore, include (1) completion of formal test- functional merits (p. 209), improving fundamental cog-
ing in specific skill domains and (2) completion of obser- nitive processes will have the greatest automatic generali-
vational checklists (or quality of life inventories) where zation to the many untrained tasks where those processes
children (or adults) may be evaluated in natural environ- are needed.
ments (Blosser & DePompei, 2003; Turkstra et al., 2005). Generalization may be more difficult to achieve when C
This type of assessment might include administration of CCD is associated with diffuse brain injury versus focal
the Functional Assessment of Verbal Reasoning and Execu- stroke. Rehabilitation approaches that are more contextu-
tive Strategies test (MacDonald, 2005), a tool that was alized (implemented in natural settings) and more func-
developed for evaluation of cognitive-communication tion oriented (designed to support activities of daily
skills specifically related to reading, writing, and living) than traditional methods are ideal for promoting
reasoning (Turkstra et al., 2005), and the Quality of Com- rapid skill mastery and transfer. Ylvisaker et al. (2008)
munication Life Scale (Paul et al., 2005). suggest that treatment strategies designed to compensate
Assessment of CCD in dementia employs standar- for impaired executive functions and self-regulation abil-
dized screening tests, severity staging instruments, and ities will most often lead to successful rehabilitation after
comprehensive assessment batteries (Hopper & Bayles, brain injury. They advocate practicing essential cognitive-
2008). Screening tests include the Story Retelling Subtest communication tasks (such as conversing with family
of the Arizona Battery for Communication Disorders members or taking notes during lectures) within support-
of Dementia (Bayles & Tomoeda, 1993) and the FAS ive real-world environments, where stimuli likely to trig-
Verbal Fluency Test (Borkowski, Benton, & Spreen, ger errors have been removed and where the use of
1967). Tests for estimating the severity of cognitive de- external aids is encouraged.
cline include the Mini-Mental State Examination Intervention for CCD in DAT emphasizes manage-
(Folstein, Folstein, & McHugh (1975)) and the Global ment rather than rehabilitation. Goals are designed
Deterioration Scale (Reisberg, Ferris, deLeon, & to help individuals maintain functional competence for
Crook (1982)). Comprehensive assessment batteries as long as possible. Decontextualized errorless learning
include the Arizona Battery for Communication Disorders activities that drill attention and memory can bolster the
of Dementia (Bayles & Tomoeda, 1993) and the Function- encoding and retrieval of factual information in early
al Linguistic Communication Inventory (Bayles & stages of dementia. Intervention tasks that employ exter-
Tomoeda, 1994), the latter being most useful for indivi- nal memory aids that recruit long-term (remote) mem-
duals with severe dementia (Hopper & Bayles, 2008). ory (such as reminiscing about past events) and that
Assessment findings can help families work with SLPs to draw on procedural memory for familiar routines
understand and compensate for the symptoms of cogni- (such as describing how to get home) can help promote
tive decline. social communication and safety while capitalizing
on those aspects of cognition that are available in mid-
stage dementia. Tangible sensory stimuli (including dolls
and stuffed animals) can help support communication
Treatment and reduce agitation as dementia advances (Bourgeois,
1990, 1991, 1992; Hoerster, Hickey, & Bourgeois, 2001;
Treatment for CCD has traditionally been decontextua-
Hopper & Bayles, 2008).
lized (implemented in rehabilitation settings or at
bedside) and deficit oriented (designed to improve/
support impaired cognitive-communication processes).
Conventional pencil/paper or computer tasks are used to Cross References
stimulate underlying cognitive processes (e.g., attention,
memory) with the expectation that as they improve, so Attention
will the related functional skills (e.g., readiness to listen to Brain Injury
speakers taking turns, remembering a set of instructions Cognitive Functioning
given by ones boss). Myers (1999) advocates the use Cognitive Processing
of decontextualized treatments to facilitate cognitive- Cognitive Rehabilitation
communication skills in individuals who have acquired Dementia
CCD from right hemisphere stroke. She argues that al- Executive Functioning
though the value of any treatment approach rests on its Functional Neuroanatomy
638 C Cognitive-Communication Impairment
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Mild Brain Injury
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Cognitive-Log C 639
controlling for demographics and injury severity. The Cog- Lee, D., LoGalbo, A. P., Banos, J. H., & Novack, T. A. (2004). Prediction of
cognitive abilities one year following TBI based on cognitive screen-
Log was also significantly correlated with the results of the
ing during rehabilitation. Rehabilitation Psychology, 49, 167171.
Mini Mental State Examination, a well-known cognitive Penna, S., & Novack, T. (2007). Further validation of the orientation and
screening test (r = 0.75, P < 0.001). Cognitive Log relative to the mini mental status exam. Archives of
Physical Medicine and Rehabilitation, 88, 13601361.
Clinical Uses
Individuals without known neurological insult received Collaborative Care
average total Cog-Log scores of 28 ( 2), and mean
individual item scores were greater than or equal to Family-Centered Care
2.4. Age, education, and gender did not predict total or
individual item scores (p > 0.05). Stepwise discriminant
analyses on a sample of 82 persons with brain injury and
82 normal controls matched for age, education, and gen-
der revealed that a cut-off score of 25 correctly classified Collagen Vascular Disease
88.4% of individuals in their respective groups.
The Cog-Log is a companion instrument to the Ori- E LLIOT J. R OTH
entation Log (O-Log). Generally, the Cog-Log is not Northwestern University
administered until a score of at least 15 is achieved on Chicago, IL, USA
the O-Log, indicating that the person is responding and
able to respond correctly to some orientation questions.
Administering the Cog-Log prior to this point has not Synonyms
proven fruitful. The Cog-Log can be administered every
day, but typically three times a week is sufficient to moni- Connective tissue disease
tor progress or detect deterioration.
Efficiency and ease of assessment were considered
when choosing items; tasks requiring additional stimuli Definition
(e.g., block construction) or extended administration
times were not included. The Cog-Log was designed for Collagen vascular diseases are a group of conditions that are
flexible administration to patients with severe cognitive characterized by malfunction of the tendons, bones, and
and behavioral disturbances. Administration time ranges connective tissue, that are supported by collagen. Their
from 7 to 10 min for confused patients, but can be as short pathogenesis is autoimmune in nature.
as 5 min for those who perform well.
Current Knowledge
Cross References The most common collagen vascular disorders include
rheumatoid arthritis, systemic lupus erythematosus,
Cognitive Functioning
scleroderma, and dermatomyositis. Others include poly-
Galveston Orientation and Amnesia Test
myositis, polyarteritis nodosa, ankylosing spondylitis, and
Mini Mental State Examination
a number of vasculopathies. These diseases are frequently
Traumatic Brain Injury
associated with diffuse inflammatory changes, abnormal
immunity. Vascular abnormalities that result from these
conditions serve as frequent causes of various types of
References and Readings vasculitis. Common features include arthritis, skin
changes, eye changes, pericarditis, pleuritis, myocarditis,
Alderson, A. L., & Novack, T. A. (2003). Reliable serial measurement of nephritis, and vasculitis of the brain, peripheral nerves, or
cognitive processes in rehabilitation: The Cognitive-Log. Archives of
extremities. They also may have a variety of hematological
Physical Medicine and Rehabilitation, 84, 668672.
Center on Outcome Measurement in Brain Injury (COMBI). www.tbims. changes causing clotting or bleeding, and a number of
org/combi. Accessed May 26, 2009. abnormal circulating blood proteins.
Color Agnosia C 641
The cause of most of these diseases is unknown. color perception (i.e., retaining the ability to match
Hereditary factors and deficiencies, autoimmunity, envi- colors or to identify the numbers on the Ishihara plates).
ronmental antigens, infections, allergies, and antigen- They also have difficulty matching colors, either verbally
antibody complexes in various combinations are probably or visually, to familiar colored objects (e.g., identifying
involved. the color normally associated with cherries, lettuce, C
or bananas).
Relatively rare, pure color agnosia must be distin-
Cross References guished from other disturbances of color perception
and color naming (color anomia). In color blindness,
Cerebral Angiitis the individual is unable to perceive or distinguish either
Lupus Cerebritis certain colors or possibly all color. In the latter case,
Vasculitis the world is seen in shades of black and white. While
color blindness is usually congenital, it can also be ac-
quired, a condition known as central achromatopsia.
References and Readings The latter is a perceptual deficit thought to result
from lesions in the visual cortices (e.g., lingual gyrus
Klippel, J. H., Stone, J. H., Crofford, L. J., & White, P. H. (Eds.). (2008).
and the occipitotemporal (fusiform) gyrus). In this dis-
Primer on the rheumatic diseases (13th ed.). New York: Springer. order, the patient may have difficulty verbally naming a
visually presented color, pointing to a color named by
the examiner, or simply matching or sorting colored
objects to others of a similar hue, yet still be able
to indicate (name) the colors normally associated with
Collapsed Lung common objects (e.g., the colors of the outside, inside,
and seeds of a watermelon). In a milder form of this
Pneumothorax condition (dyschromatopsia), colors are described as
dull, washed out, or faded. In color anomia, the
problem is not one of perceptions, but of language.
The patient can perceive and match colors, but has diffi-
Colliculi culty naming specific colors or pointing to colors named
by the examiner.
Inferior Colliculi In the few published cases, lesions associated with
color agnosia tend to occur in the left or bilateral occipi-
totemporal area.
Color Agnosia
J OHN E. M ENDOZA Cross References
Tulane University Medical Center
New Orleans, LA, USA Achromatopsia
Color Anomia
Definition
Literally, a loss of previous color knowledge. References and Readings
Bauer, R. M., & Demery, J. A. (2003). Agnosia. In K. Heilman & E.
Current Knowledge Valenstein (Eds.), Clinical neuropsychology (4th ed., pp. 236295).
New York: Oxford University Press.
Tranel, D. (2003). Disorders of color processing. In T. E. Feinberg & M. J.
In pure color agnosia, patients have difficulty naming Farah (Eds.), Behavioral neurology and neuropsychology (pp. 243
or pointing to named colors, despite relatively preserved 256). New York: McGraw-Hill.
642 C Color Anomia
Definition
Current Knowledge
Clinical Uses
Description
The CPM was designed for use with children, older
The colored progressive matrices (CPM) are an alter- people, for anthropological studies, and for clinical
nate form of the Ravens progressive matrices (RPM) work. Its format makes it valuable for individuals who
that was published in the 1940s. Shorter and simpler cannot understand English; people with physical disabil-
than the original, this version was designed for younger ities, aphasias, cerebral palsy, or deafness; and people with
children (ages 511 years), the elderly (over 65 years), below normal intelligence. The colored backgrounds were
and people with moderate or severe learning difficul- introduced to attract the patients attention; the test
ties. As such, it also tends to be used more frequently in can be administered in the form of illustrations in a
research protocols, although it is important to note booklet or as boards with moveable pieces. Patients with
that the CPM and the RPM are not interchangeable, left hemisphere damage perform better on the colored
nor may derived scores from the two tests be inter- matrices than on the standard form (as described in
preted the same. CPM contains 36 items, grouped into Lezak, Howieson, & Loring, 2004). This is likely attribut-
three sets (A, Ab, B) of 12 items each: A and B from the able to the fact that while only one-fifth of RPM items test
original version, with the addition of set Ab. Most visuospatial skills almost exclusively, fully one-third of
items are presented on a colored background to make items on the CPM are predominantly visuospatial, with
the test visually stimulating for participants; the bright other items involving more problem-solving. Also likely
background does not seem to detract from the clarity due to the visuospatial task demands, individuals with
of the stimuli. As the last few items in set B are exactly Lewy body dementia tend to have more difficulty on the
the same as they appear in the standard version, an CPM than Alzheimers patients with similar levels of
examinee who succeeds on these may go on to Sets C, dementia.
644 C Coma
Cross References rating, and include the Glasgow Coma Scale (Teasdale &
Jennett, 1976) and the FOUR Score (Wijdicks, Bamlet,
Advanced Progressive Matrices Maramattom, Manno, & McClelland, 2005).
Ravens Progressive Matrices
Standard Progressive Matrices
Prognosis
References and Readings Mortality rates for patients in coma vary with etiology,
but commonly reach or exceed 50%. Early prognostic
Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsycho- variables of poorer outcome include lower Glasgow
logical assessment (4th ed.). New York: Oxford University Press. Coma Scale scores (Teasdale & Jennett, 1976), increased
Raven, J. C. (1938, 1996). Progressive Matrices: A perceptual test of
intelligence. Oxford: Oxford Psychologists Press Ltd.
age, absent pupillary responses, systolic blood pres-
Raven, J., Raven, J. C., & Court, J. H. (1998). Raven manual: Section 2. sure <90 mm Hg, and computed tomography abnormal-
Colored progressive matrices. Oxford: Oxford Psychologists Press Ltd. ities, including compression, effacement, or blood within
Raven, J., Raven, J. C., & Court, J. H. (2000). Raven manual research the basal cisterns, or extensive traumatic subarachnoid
supplement 3: American norms, neuropsychological applications. hemorrhage (Posner et al., 2007).
Oxford: Oxford Psychologists Press Ltd.
Raven, J., Raven, J. C., & Court, J. H. (2003). Manual for Ravens
progressive matrices and vocabulary scales. Section 1: General
overview. San Antonio, TX: Harcourt Assessment. Cross References
Strauss, E., Sherman, E. M. S., & Spreen, O. (Eds.). (2006). A compendium
of neuropsychological tests (3rd ed.). New York: Oxford University Decerebrate Posturing
Press.
Decorticate Posturing
Glasgow Coma Scale
Loss of Consciousness
Minimally Conscious State
Coma Stupor
Vegetative State
J ACOB K EAN
Indiana University School of Medicine
Indianapolis, Indiana, USA References and Readings
Posner, J. B., Saper, C. B., Schiff, N. D., & Plum, F. (2007). Plum and
Definition Posners diagnosis of stupor and coma. New York: Oxford University
Press.
Coma is a state of unconsciousness in which the patient is Teasdale, G., & Jennett, B. (1976). Assessment and prognosis of coma
incapable of being awake, and is unarousable, even with after head injury. Acta Neurochirurgica, 34, 4555.
Wijdicks, E. F. M., Bamlet, W. R., Maramattom, B. V., Manno, E. M., &
vigorous stimulation. Coma is usually the result of dis- McClelland, R. L. (2005). Validation of a new coma scale: The FOUR
ease or injury, and rarely lasts for more than 4 weeks. score. Annals of Neurology, 58, 585593.
While comatose, a patient may respond to painful stimuli
but lack the ability to demonstrate localized response or
defensive movements (Posner, Saper, Schiff, & Plum,
2007).
Coma Recovery Scale
Current Knowledge M ARK A. S ANDBERG
Independent Practice
Diagnosis Smithtown, NY, USA
Definition Kalmar, K., & Giacino, J. (2005). The JFK coma recovery scale-revised.
Neuropsychological Rehabilitation, 15(34), 454460.
The CRS-R, including administration and scoring information and rating
The Coma Recovery Scale (CRS-R) is a 23-item instru- forms, is available as a PDF file at www.tbims.org/combi/crs/index.
ment used to assist with differential diagnosis, prognostic html.
assessment, and treatment planning with patients having C
disorders of consciousness.
Current Knowledge
Coma Vigile
The Coma Recovery Scale was initially developed by
Vegetative State (Persistent)
Giacino and colleagues in 1991 and thereafter was revised
in 2004 as the JFK Coma Recovery Scale-Revised (Giacino,
Kalmar, & Whyte, 2004). The scale was developed with the
goal of helping to identify the neurobehavioral character-
istics of persons diagnosed with disorders of conscious- Coma/Near Coma Scale
ness, thus allowing for increased prognostic clarity and
refinement of treatment methods. In its revised form, M ARK A. S ANDBERG
the CRS-R is composed of 23 items, which coalesce into Independent Practice
six subscales addressing auditory, visual, motor, oromo- Smithtown, NY, USA
tor, communication, and arousal functions. Each subscale
is composed of hierarchically arranged items associated
with the brain stem, subcortical, and cortical processes. Synonyms
Items receiving higher scores are more likely to reflect
cognitively mediated activity. The test syllabus offers be- C/NC; CNC
havioral criteria, which provide the user with operationa-
lized characteristics used to determine whether a specific
response to sensory stimuli has been demonstrated by the
Description
patient. A rating form is included as part of the syllabus,
which allows the user to perform serial assessments.
The Coma/Near Coma (CNC) Scale was designed to
Based on the 2004 study by Giacino, Kalmar and Whyte,
measure small changes in neurobehavioral status among
interrater and testretest reliability were described as high
patients who have sustained severe brain injuries and
for CRS-R total scores, although some systematic scoring
whose clinical functioning is consistent with vegetative
differences between raters were found on the visual and
or near-vegetative states. The CNC Scale is useful when
oromotor/verbal subscales. The CRS-R has been translated
reliable, valid, and economical assessment of patients func-
into Spanish, Italian, German, French, Dutch, and Norwe-
tioning is required so as to substantiate clinical change or
gian. The CRS-R offers a reliable method for measuring the
lack thereof in patients whose functioning is at markedly
trajectory of consciousness following severe brain injury,
low levels. The CNC Scale expands the levels of the
and in assisting in the differential diagnosis of patients in a
Disability Rating Scale (DRS). It has five levels and is
minimally conscious state from those in a vegetative state.
composed of 11 items. The items assess dysfunction in
the sensory and perceptual dimensions and describe the
severity of primitive response deficits.
Cross References
of the CNC Scale is recommended whenever the DRS accuracy. Following this training, it is noted that while
score is greater than 21 (Extremely Severe Disability). single ratings can be used, a minimum of two indepen-
The scoring form also notes that in cases in which DRS dent ratings per patient is encouraged for purposes of
scores are < 21, CRC Scale ratings should be conducted promoting reliability (Rappaport, 2000). It is also recom-
monthly in conjunction with the DRS. mended that rating be taken at approximately the same
time each day whenever possible.
The CNC Scale includes 11 items representing 8 res-
Current Knowledge ponse dimensions (see Table 1). Each item includes
3 score options (0, 2, 4); 4 referring to no responsivity
As described by Rappaport, Dougherty, and Kelting (1992) to sensory stimulation. The CNC Total Score is com-
and as detailed by the Center for Outcome Measurement puted by adding the ratings (0, 2, 4) from each of the
in Traumatic Brain Injury (COMBI; www.tbims.org/ 11 items and dividing that score by the number of items
combi/cnc/index.html), interrater reliability calculated at rated. Categorical descriptions are offered to coincide
three time periods was 0.97. The lowest interrater inter- with the Total Score attained (i.e., No Coma through
correlation of 0.86 was found to exist on item 8 (nasal Extreme Coma).
swab). The internal consistency as determined using
alpha coefficients were 0.43, 0.65, and 0.65 for CNC Scale
scores taken at 1, 8, and 16 weeks post injury, respectively Cross References
(Rappaport, Dougherty, & Kelting, 1992).
A rank order correlation between CNC Scale and DRS Coma Recovery Scale
scores was 0.69 (p < 0.02). The correlation between CNC Dementia Rating Scale
Scale ratings and the prominence of sensory (auditory,
visual, and somatosensory) abnormality as determined
through evoked potential studies was 0.52 (p < 0.05) References and Readings
(Rappaport, Dougherty, & Kelting, 1992).
Discussion regarding training in the use of the CNC Rappaport, M. (2000). The coma/near coma scale. The center for outcome
Scale is offered by COMBI (www.tbims.org/combi/cnc/ measurement in brain injury. Accessed 1/6/08 from www.tbims.org/
cnctat.html). It is recommended that proper use of combi/cnc.
the instrument follows a period during which evaluations Rappaport, M. (2005). The disability rating scale and coma/near coma
scales in evaluating severe head injury. Neuropsychological Rehabili-
are performed simultaneously with several raters indepen-
tation, 15(3/4), 442453.
dently offering judgments for each item. Interrater com- Rappaport, M., Dougherty, A., & Kelting, D. L. (1992). Evaluation of
parisons and discussion concerning the reasons for making coma and vegetative states. Archives of Physical Medicine and Reha-
specific judgments are encouraged to refine observational bilitation, 73, 628634.
Commission on Accreditation of Rehabilitation Facilities (CARF) C 647
(3) all people should be empowered to exercise informed accreditation for rehabilitation organizations providing
choice. services to workers with occupational disabilities.
In support of its mission, CARF has identified numer- CARF has also expanded the types of services for
ous purposes that are the focus of the agency, including which it provides accreditation. In September 1997,
the following (retrieved June 29, 2010, from http://www. CARF was awarded a contract by the Department of
carf.org/About/Mission/). Health and Human Services, Substance Abuse and Mental
Health Services Administration for the development and
To develop and maintain current, field-driven stan-
implementation of opioid treatment program accredi-
dards that improve the value and responsiveness of
tation. After successful development of the program
the programs and services delivered to people in need
accreditation, CARF was recognized in November 2001
of rehabilitation and other life enhancement services.
by the Substance Abuse and Mental Health Services
To recognize organizations that achieve accreditation
Administration (SAMHSA)/Center for Substance Abuse
through a consultative peer-review process and dem-
Treatment (CSAT) as an approved accrediting organiza-
onstrate their commitment to the continuous improve-
tion for opioid treatment programs. As noted previously,
ment of their programs and services with a focus on the
CARF acquired the Continuing Care Accreditation
needs and outcomes of the persons served.
Commission (CCAC) in 2003. Acquisition of the CCAC
To conduct accreditation research emphasizing out-
promoted CARFs vision of being an independent re-
comes measurement and management, and to provide
source that identifies high-quality care providers for indi-
information on common program strengths as well as
viduals of all ages, including children and older adults.
areas needing improvement.
More recently, in February 2007, CARF began accrediting
To provide consultation, education, training, and
suppliers of certain durable medical equipment, prosthet-
publications that support organizations in achieving
ics, orthotics, and supplies (DMEPOS) after the Centers
and maintaining accreditation of their programs and
for Medicare & Medicaid Services (CMS) approved CARF
services.
as a national authority for DMEPOS suppliers.
To provide information and education to persons
Over the decades, CARF has steadily grown and
served and other stakeholders on the value of
expanded its accreditation services to serve individuals in
accreditation.
other countries. In 1969, CARF accredited its first program
To seek input and to be responsive to persons served
in Canada and the first program in Europe was accredited
and other stakeholders.
in August 1996. CARF Canada was incorporated in Sep-
CARF also collaborates with partners who share CARF tember 2002 in Edmonton, Alberta. Further expansion of
vision and goals, while remaining politically neutral. CARF services included accreditation of the first program in
partners include professional associations, advocacy groups, South America in December 2005. The value of CARF
governmental agencies, or individuals who are committed accreditation is extensive and includes benefits such as
to enhancing the lives of persons who receive services. business improvement, risk management, funding access,
positive visibility, accountability, peer network, in addi-
tion to other benefits detailed on the CARF web site.
Landmark Contributions
One of the key developments in the history of CARF was Major Activities
the passing of a resolution by the Council of State Admin-
istrators of Vocational Rehabilitation in April 1970, which CARF accreditation outcomes for CARF and CARF
urged state agencies to support a CARF accreditation Canada consist of a three-year accreditation, a one-year
requirement for all organizations providing rehabilitation accreditation, provisional accreditation, nonaccreditation,
services. As CARFs services became increasingly recog- and preliminary accreditation. CARF-CCAC accreditation
nized by organizations and agencies, additional support of outcomes include a five-year accreditation and nonaccre-
CARF accreditation services has occurred. In November ditation. CARF currently has close to 1,500 surveyors
1974, Goodwill Industries of America recognized CARF throughout the North and South America and Europe.
as the accrediting organization for all Goodwill organiza- The steps to accreditation involve a year or more of
tions. Another resolution, adopted in November 1980 by preparation prior to the site survey and ongoing quality
the Association of Trial Lawyers of America, urged state improvement following the survey. CARF designates a
workers compensation agencies to require CARF CARF Resource Specialist to organizations seeking
Commission on Accreditation of Rehabilitation Facilities (CARF) C 649
accreditation to provide guidance and technical assistance its accreditation areas (Behavioral Health, Employment
regarding the accreditation process. Other resources are and Community Support, and Medical Rehabilitation).
also available to facilitate the preparation process, includ- Then in 1996, CARF published the first edition of the
ing standards manuals for the various programs and Accreditation Sourcebook. A significant contribution to
services an organization can seek accreditation for and accreditation services occurred in 1998, when CARF C
the CARF Accreditation Sourcebook, which explains the rewrote its standards to be unidimensional as part of its
accreditation process. CARF accreditation requires that an commitment to implementing the Standards Confor-
organization must implement and use the standards in its mance Rating System. Additional standards were pub-
programs for at least 6 months prior to the survey. During lished in 1999, including standard manuals for Adult
this time, an organization conducts a self-study and eval- Day Services Programs and the Veterans Health Adminis-
uation of conformance to the standards using the stan- tration Comprehensive Blind Rehabilitation Services.
dards manual in conjunction with the survey preparation Moreover, that same year, CARF extended its practice of
questions. Then the organization submits the Intent to disclosing information to the public regarding an organi-
Survey, which includes detailed information about lead- zations survey report from just the Medical Rehabilitation
ership, programs, and services that the organization is Standards Manual to also include the Behavioral Health
seeking to accredit and the service delivery location(s). Standards Manual and the Adult Day Services Standards
CARF selects a survey team by matching program or Manual. CARFs public information policy was extended
administrative expertise and relevant field experience to include all areas later in 2003. Standards manuals for
with the organizations unique requirements. During the assisted living programs were published in 2000, followed
survey, the survey team determines the organizations by the Child and Youth Services Standards Manual in 2005,
conformance to all applicable standards on site through the Aging Services Standards Manual in 2006, and Stan-
the observation of services, interviews with persons served dards for Dementia Care in 2006. In addition to these
and other stakeholders, and review of documentation. publications, CARF has continued to implement new
The survey team shares findings related to the standards standards in many of its areas of accreditation.
with the organization at an exit conference before the CARF has implemented new electronic programs
team leaves the site and this information is also submitted designed to support and further develop its mission. In
to CARF. CARF then renders the accreditation decision 2007, CARF released the uSPEQ (pronounced you speak;
and a written survey report approximately 68 weeks after http://www.uspeq.org/) Consumer Experience Survey, fol-
the survey. Organizations have 90 days after notification lowed by the uSPEQ Employee Climate Survey. Both survey
of an accreditation award to fulfill an accreditation condi- tools are designed to assist organizations with their well-
tion by submitting to CARF a Quality Improvement Plan being and performance improvement. CARF launched its
(QIP) outlining actions that have been or will be imple- extranet Customer Connect (http://customerconnect.carf.
mented in response to the recommendations made in the org) for accredited organizations and those seeking accred-
survey report. CARF maintains communication with itation to manage their individual contact information,
organizations throughout the tenure of accreditation. view up-to-date information about their organizations
CARF has published numerous standard manuals surveys, and access information about the accreditation
which have identified program evaluation standards, result- process. Most recently in 2008, CARFs ASPIRE to Excel-
ing in improved services and programs provided to clients lence quality framework has been implemented in all
receiving rehabilitation services. The first publication of a standards manuals. This framework provides a logical,
new section of standards manuals for Rehabilitation Facil- action-oriented approach to ensure that organizational
ities in November 1973 became a springboard for future purpose, planning, and activity result in desired outcomes.
CARF contributions to program evaluation. In January Each year, CARF sponsors numerous in-person and
1986, standards for two new program areas (Respite Pro- web-based seminars to help providers maintain confor-
grams and Alcoholism and Drug Abuse Treatment Pro- mance to the CARF standards. The in-person seminars are
grams) were published in the 1986 Standards Manual for held in cities across the United States, Canada, and Europe.
Organizations Serving People with Disabilities. The 1988
Standards Manual for Organizations Serving People with
Disabilities included new standards in areas of Post-Acute Cross References
Brain Injury Programs and Community Mental Health
Programs. CARF published a separate volume of its 1995 Americans with Disabilities Act of 1990
Standards Manual and Interpretive Guidelines for each of Individuals with Disabilities Education Act
650 C Commissural Fibers
Commissural Fibers
Commissures, Cerebral
Commissurotomy
C HRISTINA K WASNICA
Barrow Neurological Institute
Commissural Magna Phoenix, AZ, USA
Corpus Callosum
Synonyms
Split-brain
Commissures, Cerebral
M ARTIN R. G RAF Definition
Virginia Commonwealth University Medical Center
Richmond, VA, USA The term referring to the medical procedure in which
interconnecting fibers between the cerebral hemispheres
are lesioned. The anatomic location includes either
Synonyms or both anterior and posterior fibers. When posterior
fibers are resected, connections to the hippocampus may
Brain commissures; Commissural fibers also be affected.
Community Integration Questionnaire C 651
Current Knowledge key social and interpersonal bonds (staying close and
connected with individuals who matter most in daily
The neurosurgical technique is based on the premise life). Thus, effective communication depends as much
that when stimuli enters the brain it is rapidly communi- on nonverbal behaviors such as facial expression, touch,
cated via the corpus callosum to the other hemisphere. In and vocal intonation as on the exchange of words. C
refractory epilepsy, severing the corpus callosum Acquired deficits to communication ability can occur in
may prevent the spread of electrical activity between hemi- the absence of a notable loss in intelligence or nonlinguistic
spheres and generalization of the seizure activity. This cognitive functions, as in aphasia, dysarthria, dysphonia/
procedure prevents the communication of the two hemi- aphonia, and apraxia of speech.
spheres thus resulting in a clinical scenario of a split
brain. Clinical disconnection syndromes in these patients
have been studied across a large number of neurocognitive
Cross References
tasks. These surgical lesions have allowed for the study of
Language
sensory perceptual processes and lateralization. Deficits
Speech
have been seen in the area of perception, attention, memo-
ry, language, and reasoning. In studies of these patients, it
has become clear that the left hemisphere has limitations in
perceptual functions while the right hemisphere has more Communication Disorders
striking limitations in cognitive functions. The split brain
studies have also led to theories of consciousness and a Speech/Communication Disabilities
neurocognitive framework for the human experience.
Communicating Artery
Community Integration
Anterior Communicating Artery
Questionnaire
M ARCEL D IJKERS
Communication Ability Mount Sinai School of Medicine
New York, NY, USA
J ON G. LYON
Mazomanie, WI, USA
Synonyms
Definition CIQ
Participation. The CIQ consists of 15 items relevant to resulting instrument, sometimes designated the CIQ-R,
home integration (H) (living), social integration (S) dropped one item and reassigned two to different sub-
(loving), and productive activities (P) (working). It is scales (Sander et al., 1999). The CIQ-R has been used
scored to provide subtotals for each of these domains, as infrequently. In 2002, The TBIMS dropped the CIQ, and
well as a total community integration score. The basis for replaced it (in 2007) with the Participation Assessment
scoring is primarily frequency of performing activities or with Recombined Tools (PART).
roles, with secondary weight given to whether or not
activities are done jointly with others and the nature
of these other persons (for example, with/without dis- Psychometric Data
ability). The CIQ can be completed by either the index
person or a proxy in 1015 min. The optimal method Psychometric information for the CIQ is spread out over
of data collection is an in-person interview, but a number of sources. In addition to the three papers by
telephone interviewing is quite common, and the Trau- Willer et al. (Willer, Linn, et al., 1994; Willer, Ottenbacher,
matic Brain Injury Model Systems (TBIMS) have also et al., 1994; Willer, Rosenthal, et al., 1993) key texts
utilized self-administered CIQs. No formal training are those by Corrigan and Deming (1995), Sander et al.
and credentialing process for the administration of the (1999); Sander et al. (1997) and Tepper, Beatty and
CIQ exists. DeJong (1996). Dijkers critically summarized psychomet-
ric information available through 1997 (Dijkers, 1997),
and some later sources are given in Reistetter and Abreus
Historical Background 2005 article (Reistetter and Abreu, 2005).
Reliability. Results of reliability studies have been
The CIQ was developed in the early 1990s by Barry Willer, mixed. Based on the (Pearson) correlations reported in
Ph.D. and a group of professionals and consumers to the earliest study, the interrater reliability of the CIQ
provide a measure of community integration after trau- appears to be in the acceptable range. However, the
matic brain injury (TBI) that could be used in the TBIMS intraclass correlation coefficient (ICC), a more appropri-
research program (Willer, Linn, & Allen, 1994; Willer, ate measure, resulted in much lower numbers, according
Ottenbacher, & Coad, 1994; Willer, Rosenthal, Kreutzer, to a later investigation, especially for the Home (H)
Gordon, & Rempel, 1993). They used the following design dimension. More recent research also suggests that in
criteria: brevity; suitable for use in in-person or telephone home integration there is the greatest discrepancy
interviews with the person with TBI or a proxy; focus on between reports by subjects with TBI and those by their
behaviors rather than feeling states; no biases resulting proxies. In the latter study, the person with TBI tended
from age, gender, or socioeconomic status; sensitive to a to report higher values than the proxy for all three
wide variety of living situations; and value neutral. The components. Subscale ICCs ranged from 0.43 (H) to
instrument has been translated (not always using formal 0.81 (P) fairly low values.
backtranslation methods) into French, Spanish, Japanese, For the CIQ total score, three out of four studies have
Korean, Dutch, Swedish, and Norwegian, among others. reported internal consistency levels that exceed the
It is the most extensively used measure of community criterion of a coefficient alpha above 0.80. However, the
integration/ participation in research on TBI, but has corresponding values for the S and P dimensions
also been applied in investigations of other types of are much lower, especially for the latter quite likely
brain injury (including stroke and brain tumors), spinal due to the fact that alpha is based on only two variables,
cord injury, burns, trauma in general, mobility disabil- which are very dissimilar from one another: work/study/
ities, and post-polio syndrome. The most common appli- volunteering, and travel.
cations are in program evaluation and the study of the Distribution issues. Corrigan and Deming noted
natural history of TBI and other impairments, but the negatively skewed distributions for the premorbid data
CIQ has also been used in clinical trials of interventions to for CIQ total, positive skews for H for all four samples,
improve post-injury functioning, and in construct valida- negative skews for all samples for the S dimension, and
tion studies of newly proposed participation measures. negative skew for P for the TBI-premorbid sample, but
Because the assignment of items to subscales by Willer positive skew for the three others. Various kurtosis pro-
et al. was based on a correlation matrix calculated for a blems were also noted. They recommended that the P
small sample, Sander et al. performed a factor analysis subscale not be used independently from its contribution
involving all CIQ items for a much larger sample. The to the CIQ total score. Distribution problems were also
Community Integration Questionnaire C 653
noted by Willer et al. and Sander et al., among others. use of the CIQ is freely given but should be requested, by
Unless these can be resolved through transformations, contacting him at the Center for Research on Community
nonparametric statistics need to be used in order to deal Integration at the Ontario Brain Injury Association,
with the nonnormal distributions. 3550 Schmon Parkway, Thorold, Ont L2V 4Y6, Canada,
Validity. No formal content or face validity studies of email: willer@vaxxine.com C
the CIQ have been done, but it was developed utilizing
a panel consisting of both consumers and professionals
with expertise in TBI outcome studies. Cross References
CIQ subscales and total score have been found to
correlate with subscales and total score on CHART CHART Short Form
(another measure of community integration), impairment Community Re-entry
and disability, time since injury, CIM (a measure of subjec- Craig Handicap Assessment and Report Technique
tive community integration), and subjective quality of life, Frenchay Activity Index
according to multiple reports. Most researchers find Glasgow Outcome Scale
negative correlations between CIQ and its subtotals (except Glasgow Outcome Scale Extended
sometimes H) and age. Females tend to have higher CIQ Impact on Participation and Autonomy Questionnaire
scores for total, H and S than males, but lower P scores. Instrumental Activities of Daily Living (I-ADL)
Sensitivity. The available research shows that the CIQ LawtonBrody IADL scale
can validly distinguish between persons with TBI and LIFE-H
nondisabled people. Persons with TBI are less integrated Outcome, Outcome Measurement
along all dimensions than nondisabled comparison Participation Measure for Post Acute Care (PM-PAC)
groups in most research. In one study, CIQ scores distin- Participation Objective Participation Subjective
guished between three groups of persons with TBI living Reintegration to Normal Living Index
in settings differentiated by supervision/support level: Social and Occupational Functioning Scales (SOFAS)
independent in the community, in the community with Traumatic Brain Injury Model System
some (natural) support, and in an institution such as a
nursing home, rehabilitation facility, etc. Willer et al.
reported gain in CIQ (sub)scores for people with TBI References and Readings
receiving residential rehabilitation services between the
second and third anniversary of injury, while a control Corrigan, J. D., & Deming, R. (1995). Psychometric characteristics of the
group who received no or minimal home-based services community integration questionnaire: Replication and extension.
did not show a gain. Corrigan et al. found trends toward Journal of Head Trauma Rehabilitation, 10, 4153.
Dijkers, M. (1997). Measuring the long-term outcomes of traumatic
improvement in all three subscales and total score in a
brain injury: A review of the community integration questionnaire.
cross-sectional study covering injury anniversaries one Journal of Head Trauma Rehabilitation, 12, 7491.
through four. However, in another investigation, a very Reistetter, T. A., & Abreu, B. C. (2005). Appraising evidence on
small increase from the first to the second anniversary of community integration following brain injury: A systematic review.
injury was reported for a group that in large majority did Occupational Therapy International, 12, 196217.
Sander, A. M., Seel, R. T., Kreutzer, J. S., Hall, K. M., High, W. M., Jr., &
not receive rehabilitation services any longer.
Rosenthal, M. (1997). Agreement between persons with traumatic
brain injury and their relatives regarding psychosocial outcome
using the community integration questionnaire. American Journal
Clinical Uses of Physical Medicine and Rehabilitation, 78, 353357.
Sander, A. M., Fuchs, K. L., High, W. M., Jr., Hall, K. M., Kreutzer, J. S., &
In several TBI community integration programs, the CIQ Rosenthal, M. (1999). The community integration questionnaire
revisited: An assessment of factor structure and validity.
is used to monitor patients progress. However, due to the
American Journal of Physical Medicine and Rehabilitation, 80,
lack of norms and the dependence of community 13031308.
functioning on the environment, the clinical applicability Tepper, S., Beatty, P., & DeJong, G. (1996). Outcomes in traumatic brain
of the CIQ is very much limited. injury: Self-report versus report of significant others. Brain Injury,
10, 575581.
Willer, B., Linn, R., & Allen, K. (1994). Community integration
Note and barriers to integration for individuals with brain injury. In
Barry Willer Ph.D., who was the principal investigator in M. A. J. Finlayson & S. H. Garner (Eds.). Brain injury rehabilitation:
developing the CIQ, holds the copyright. Permission for clinical considerations. Baltimore: Williams and Wilkins.
654 C Community Re-entry
Willer, B., Ottenbacher, K. J., & Coad, M. L. (1994). The community Cross References
integration questionnaire. A comparative examination. American
Journal of Physical Medicine and Rehabilitation, 73, 103111.
Willer, B., Rosenthal, M., Kreutzer, J. S., Gordon, W. A., & Rempel, R.
Quality of Life
(1993). Assessment of community integration following rehabili-
tation for traumatic brain injury. Journal of Head Trauma
Rehabilitation, 8, 7587. References and Readings
Definition
Community-based rehabilitation attempts to en- into well-perfused organs. Muscle, fat, and bone are
hance the quality of life for individuals with disabilities saturated last. These tissues can serve as storage depots
and their families. This includes meeting basic needs, for substances, contributing to the half-life of a drug and
but also going beyond to promote inclusion and partici- potentially to interaction effects days or weeks after a drug
pation. Community-based rehabilitation typically has been discontinued. C
involves several domains of functioning such as health, The body may be inappropriately be treated as a single
education, work, socialization, and ones sense of entity or compartment. In a single-compartment model,
empowerment. Reaching a point of social acceptance in drugs are theoretically presumed to diffuse through tis-
the 1970s and 1980s, the original mission of community- sues and the differences in absorption and retention of a
based rehabilitation was to offer individuals with dis- drug are not treated as significant. This single-compart-
abilities access to programming and supports in their ment model is sufficient in many situations, but is less
own home communities using local resources. Further, useful when drugs may be preferentially retained in one
community-based rehabilitation ostensibly attempts to type of tissue. Dosage predictions fail if they do not
optimize the provision of rehabilitative services, equal- account for the gradual ambient leaching of the drug
ize opportunity, reduce poverty, and promote full social from fat or bone into general circulation.
inclusion. The instances in which a multi-compartment model
is most relevant are for individuals who are medically ill,
have unusually high or low body fat, are pregnant, have
an abnormality in pH or illnesses which impact the liver
References and Readings or kidneys (and thus profoundly alter the pharmacoki-
netics of drug metabolism). Differences in the propor-
Church, J., Saunders, D., Wanke, M., & Pong, R. (1995). Organizational
models in community based health care: A review of the literature.
tion of fatty tissue can influence dosing decisions since
Health Promotion and Programs Branch,Ottawa: Health Canada. mg/kg may be less appropriate for drugs that are less fat-
World Health Organization, (2004). CBR: A strategy for rehabilitation, soluble. One common example of this reduced alcohol
equalization of opportunities, poverty reduction and social inclu- tolerance in women since fatty tissues do not readily
sion of people with disabilities: Joint position paper/International
absorb alcohol.
Labour Organization, United Nations Educational, Scientific and
Cultural Organization and the World Health Organization.
Another form of compartmentalization is ion
trapping, in which the pH alters the ease with which a
drug is transferred into or out of a particular tissue. For
example, neonatal blood is typically more acidic than
adult blood, making it more difficult for slightly alkaline
drugs to diffuse back into maternal circulation. Similarly,
Comorbid the GI tract is more acidic than blood plasma. Weakly
acidic drugs diffuse more readily to blood than weakly
Dual Diagnosis alkaline drugs.
Cross References
pH
Compartmentalization Pharmacokinetics
N ADIA W EBB
Childrens Hospital of New Orleans
New Orleans, LA, USA References and Readings
Brunton, L. B., Lazo, J. S., & Parker, K. L. (Eds.). (2005). Goodman &
Definition Gilmans the pharmacological basis of therapeutics (11th ed.).
New York: McGraw Hill.
Stahl, S. M. (2008). Stahls essential psychopharmacology: Neuroscientific
Drugs do not saturate tissues within the body at the same basis and practical applications. New York: Cambridge University
rate. They are quickly absorbed into blood plasma and Press.
656 C Compensation Neurosis
Definition
Current Knowledge
Compensation neurosis is a condition in which
symptoms are associated with a real or presumed dis- The goal of education is to help students acquire skills and
ability (possibly exaggerated) that may bring financial master concepts that will be useful in daily living.
compensation. This type of neurosis is believed to be Generally, two approaches are used when instruction
motivated by the desire for, and hope of, monetary or fails to help students learn. These include compensatory
interpersonal gain. Many factors are involved in com- education approaches and/or remedial education
pensation neurosis, such as the psychological factors approaches ( Remedial Education Approach). Compen-
before and after the presumed injury, the additional satory educational approaches require teachers to present
effects on the quality of life, and the possible influences material in a different format, providing students an
of legal or insurance processes. The term is often used alternative way to master a particular concept and dem-
in litigation and seems to be quite controversial in onstrate knowledge. They are used when students lack the
psychiatry. ability to acquire a certain skill or concept. For example, a
deaf student who is unable to speak may be taught sign
language as an alternate form of communication; or, a
student with a math calculation disability may be taught
Cross References to use a calculator. When students fail to make progress in
a certain skill area, evidence-based strategies and methods
Fake Bad Scale must be employed to help the student attain expected
Malingering learning outcomes. In these cases, education must be
adjusted by offering different activities that facilitate the
same results. This approach in isolation does not repre-
sent best practice, and in keeping with the educational
and neuro-developmental literature, practitioners should
References and Readings offer both remedial and compensatory approaches,
depending on students specific academic needs. Educa-
Brown, J. T. (October 1996). Compensation neurosis rides again: tors must be careful to distinguish between the education-
A practitioners guide to defending PTSD claims
al content in which a student struggles (e.g., reading or
(post traumatic stress disorder). Defense Counsel Journal, 63(4),
467482.
math), and the method in which a particular concept may
Rogers, R. (2008). Clinical assessment of malingering and deception (3rd be successfully integrated. Offering a compensatory ap-
Ed.). New York: Guilford Press. proach should be a last resort on the educational
Compensatory Strategies C 657
continuum, and should be supported by a comprehensive Examples of behavioral strategies would include repeating
neuro-psychological evaluation. phrases during social interactions to ensure accurate pro-
cessing of conversation, or associating words with images
to enhance recall.
Cross References C
Academic Competency
Current Knowledge
Academic Skills
Compensatory Education Approach
Compensatory strategies have shown efficacy in rando-
Remedial Education Approach
mized, controlled studies for improving the ability to
Strength-Based Education Approach
remember to complete activities in the future (prospective
memory) in neurologically impaired people with cogni-
tive difficulties. For example, Wilson, Emslie, Quirk, and
References and Readings Evans (2001) used an external pager in 143 people be-
tween 8 and 83 with a range of neurological illnesses or
DAmato, R. C., & Rothlisberg, B. A. (1996). How education should trauma. Results revealed that more than 80% of clients
respond to students with traumatic brain injury. Journal of Learning
improved in carrying out self-care activities and meeting
Disabilities, 29(6), 670683.
DAmato, R. C., Rothlisberg, B. A., & Work, P. L. H. (1999). Neuropsy-
appointments, as well as other life skills, relative to the
chological assessment for intervention. In C. R. Reynolds, & T. B. baseline period, and effects were maintained seven weeks
Gutkin (Eds.), The handbook of school psychology (pp. 452475). after return of the pager. Environmental modifications,
New York: Wiley. including use of signs, removal of distracting environ-
Gaddes, W. H., & Edgell, D. (1994). Learning disabilities and brain
mental stimuli, and checklists have been used effectively
function: A neuropsychological approach (3rd ed.). New York:
Springer.
in randomized, controlled trials to enhance adaptive func-
tioning and quality of life in people with severe psychiatric
illnesses as well (Velligan, Prihoda, Ritch, Maples, Bow-
Thomas, & Dassori, 2002).
Compensatory Strategies
M ATTHEW M. K URTZ Cross References
Wesleyan University
Middletown, CT, USA Cognitive Rehabilitation
Environmental Modifications
Mnemonic Techniques
Definition Neuropsychological Rehabilitation
Prosthetic Memory Aids
Compensatory strategies are environmental modifica-
tions or behavioral strategies designed to bypass persistent
impairment in attention, memory, executive-function,
References and Readings
and/or other cognitive skills as a means to achieve desired
rehabilitation goals. Environmental modifications could
Velligan, D. I., Prihoda, T. J., Ritch, J. L., Maples, N., Bow-Thomas, C., &
include the use of external aids or modifying the setting in Dassori, A. (2002). A randomized, single-blind pilot study of com-
which activities take place. The use of an alphanumeric pensatory strategies in schizophrenia outpatients. Schizophrenia Bul-
pager and a checklist for a person with memory and letin, 28, 283292.
executive-function deficits to ensure completion of daily Wilson, B. A. (2008). Neuropsychological rehabilitation. Annual Review
of Clinical Psychology, 4, 141162.
tasks at specific times would be an example of external
Wilson, B. A., Emslie, H. C., Quirk, K., & Evans, J. J. (2001). Reducing
aids. Working in a distraction-free room to enhance con- everyday memory and planning problems by means of a paging
centration skills in a person with symptoms of disinhibi- system: a randomised control crossover study. Journal of Neurology,
tion would be an example of modifying an environment. Neurosurgery and Psychiatry, 70, 477482.
658 C Competence to Testify
Current Knowledge
Temporal Lobe
Temporal Lobe Epilepsy Computed Tomography
R OBIN S EKERAK
Waikato District Health Board
References and Readings Hamilton, New Zealand
Historical Background
Computer-Based Test
Interpretation CARB was designed and published by Conder, Allen, and
Cox (1992) to provide a computerized tool for adminis-
Test Interpretations: Computer Based tration of the forced-choice, digit recognition tasks that
were gaining popularity in assessment of effort during
neuropsychological testing. Hiscock and Hiscock (1989)
had described the use of forced-choice methods in detect-
Computerized Assessment of ing malingering, and Binder (1990) had also studied
Response Bias this method of assessing effort. The authors of CARB
elected to name the test with the phrase response bias
DAVID R. C OX (as opposed to malingering or effort) to clarify that the
Chapel Hill, NC, USA test was not diagnosing malingering, but measuring a
bias in the response.
Several versions of CARB have been published and
Synonyms studied. The original version (Conder et al., 1992) utilized
three distinct trials of 37 items each. Each item includes
CARB presentation of a five digit number that is to be remem-
bered followed by a short delay during which the examin-
ee is to count silently backward from 20. The examinee
Description then selects the presented number from two choices the
target and a foil. The groups of trials differ from one
The Computerized Assessment of Response Bias (CARB) another in that the delay between stimulus presentation
is a computer-based tool for evaluating effort. The CARB and forced-choice presentation increases from 3 to 6 and
is one of many measures characterized as symptom valid- 9 s, respectively. Initial studies of this version of CARB
ity tests (SVT), indices of response bias used to assess determined that performance at or below 89% correct
possible malingering, poor effort, or exaggeration of defi- falls more than two standard deviations below the sample
cit. The test is a computerized version of the digit recog- of individuals with documented brain injury (Conder
nition paradigm in a forced choice format, akin to the et al., 1992); performance below 90% was therefore
procedures described by Hiscock and Hiscock (1989) and deemed to be biased.
Binder (1990). Frequently, statistical determination of Subsequent versions of CARB include CARB-97
below chance performance (based on the binomial theo- (Allen, Conder, Green, & Cox, 1997) and another revi-
rem) has been used with tests such as these to identify sion referenced in Green and Iverson (2001). CARB-97
persons showing poor effort. This method requires an added auditory feedback to the original version such that
extremely low score to reach a statistically significant correct responses are followed by a pleasant tone and
level. However, as the CARB is an easy test, it allows the incorrect responses are followed by an unpleasant tone.
clinician to detect inadequate effort using above-chance The third revision shortened the delays between stimulus
cutoff scores (Millis, 2008). The test has been studied with presentation and forced choice presentation to 1.5, 2.5,
a wide variety of populations, including persons with and 3.5 s. Each CARB version has been utilized in pub-
head injury, chronic fatigue syndrome, musculoskeletal lished studies, although the original version may continue
injuries, pain disorders, and psychiatric or emotional dis- to be the most widely used (Larrabee, 2007).
turbances such as depression. Published studies have in-
cluded both adults and children. Administration time can
vary depending on the protocol used; however, it typically Psychometric Data
can be completed in no more than 1015 min. CARB
performance results include percent correct for each As with many, if not most, symptom validity tests the
group of trials as well as the overall percent correct. Also nature of CARB presentation of a relatively easy task
reported are the response latency for each item, average that may be perceived to be much more difficult than it
latency per group of trials, and overall, as well as other, actually is leads to a distribution of scores that is highly
information. An interpretive summary can also be skewed toward the upper end. Individuals who put forth
provided, if desired. effort on the test get all, or nearly all, of the items correct.
Computerized Assessment of Response Bias C 663
This appears to be true regardless of the presence or appropriate in any clinical population. The concept of
absence of brain injury or other cognitive deficits. CARB the binomial theorem and evaluation of performance
was originally validated on a small sample (8) of indivi- against chance levels is a useful and reasonably valid
duals who had documented severe brain injury. That measure. Clearly, however, there is support for the use of
group performed at 98.6% (SD = 3.6) correct (Conder CARB with cutoff scores at above-chance levels in assess- C
et al., 1992). Internal consistency across items is therefore ment of those groups of whom scientific studies have been
also quite high among individuals putting forth adequate published.
effort; biased responding with suboptimal effort is pre-
sumed, by the nature of the construct, to be more variable.
Individuals who perform at a passing level on CARB Cross References
yielded 96.697.6% accuracy across the blocks of trials,
whereas those who performed at a non-passing level Dissimulation
yielded accuracy scores ranging from 62.4 to 74.6% Effort
(Allen et al., 1997; Allen, Richards, Green, Iverson, & Forensic Neuropsychologist
Conder, 1998). An accuracy difference of 10% or more Forensic Neuropsychology
between any two blocks of the test correlates highly Forensic Psychology
with poor (non-passing) scores on CARB (Allen et al., Hiscock Forced-Choice Test
1997). Testretest reliability (1-week interval) is quite Malingering
high (r = 0.97) (de Armas, 1996). Portland Digit Recognition Test
Response Bias
Rey 15-Item Test
Clinical Uses Symptom Validity Assessment
Word Memory Test
Clinically, CARB is used to assess response bias and level
of effort and assist in the detection of symptom exaggera-
tion and/or malingering. Published studies have primarily References and Readings
involved populations of individuals alleging brain injury
Allen, L. M., Conder, R. L., Green, P., & Cox, D. R. (1997). CARB 97
via closed head trauma, although other diagnostic group-
manual for the computerized assessment of response bias. Durham:
ings have also been examined. These include chronic CogniSyst.
fatigue syndrome, fibromyalgia, stroke, chronic pain, Allen, L. M., Iverson, G. L., & Green, P. (2003). Computerized assessment
multiple sclerosis, brain aneurysm, and brain tumor of response bias in forensic neuropsychology. Journal of Forensic
(Allen, Iverson, & Green, 2003). Neuropsychology, 3(2), 205225.
Allen, L. M., Richards, P. M., Green, W. P., Iverson, G. L., & Conder, R. L.
Additional studies have used samples of persons with
(1998). Performance patterns on the computerized assessment
brain injury, alleging brain injury, or with alleging other of response bias in 1752 compensation cases (Abstract). Archives of
disorders that may affect cognition. The variable of seek- Clinical Neuropsychology, 13, 1516.
ing versus not seeking compensation as a result of an Binder, L. M. (1990). Malingering following minor head trauma. The
injury or condition is a significant one in the realm of Clinical Neuropsychologist, 4, 2536.
Conder, R., Allen, L., & Cox, D. (1992). Manual for the computerized
assessing effort. Lending support to the notion that sec-
assessment of response bias. Durham: CogniSyst.
ondary gain can be a major factor in these cases. Gervais, de Armas, A. (1996). Detection of malingering in forensic psychological
Green, Allen, and Iverson (2001) reported that 100% of evaluations. The Forensic Examiner, JulyAugust, 2628.
the individuals with fibromyalgia and rheumatoid arthri- Flaro, L., Green, P., & Allen, L. M., III. (2000). Symptom validity test
tis who were not seeking compensation performed in a results with children: CARB and WMT. Archives of Clinical Neuro-
psychology, 15(8), 840.
fashion that indicated adequate effort. Age does not ap-
Gervais, R., Green, P., Allen, L., & Iverson, G. (2001). Effects of coaching
pear to be a significant factor influencing performance on on symptom validity testing in chronic pain patients presenting for
CARB. Flaro, Green, and Allen (2000) reported on chil- disability assessments. Journal of Forensic Neuropsychology, 2(2),
dren performing at or above normal adult performance 119.
levels on CARB. Allen et al. (2003) summarized a number Green, P., & Iverson, G. L. (2001). Validation of the computerized assess-
ment of response bias in litigating patients with head injuries. The
of studies providing validation for the utility of CARB
Clinical Neuropsychologist, 15, 492497.
with a variety of diagnostic groupings. Hiscock, M., & Hiscock, C. K. (1989). Refining the forced-choice method
CARB appears to be an appropriate measure of effort for the detection of malingering. Journal of Clinical and Experimental
across a very large age range, and would in theory be Neuropsychology, 11, 967974.
664 C Computerized Axial Tomography
Larrabee, G. J. (2007). Assessment of malingered neuropsychological defi- Hypothesis testing is a persons ability to try out several
cits. New York: Oxford University Press.
possible concepts, solutions, or rules in a systematic way
Millis, S. R. (2008). Assessment of incomplete effort and malingering in
the neuropsychological examination. In Morgan, & Ricker (Eds.),
to determine which is the most useful. Cognitive psychol-
Textbook of clinical neuropsychology. New York: Taylor & Francis. ogists define concept learning as the development of
prototypes, schemas, attributions, or exemplars (Lamberts &
Shanks, 1997). Prototypes are learned typical representa-
tions of the concept. For example, a compact car is small,
gets good gas mileage, and is a Toyota. Schemas are scripts
Computerized Axial Tomography that are learnt for behaving or for evaluating behavior.
For example, an introduction usually involves a greeting,
Computed Tomography a smile, and a handshake. Attributions are collections of
behavior adjectives and adverbs that define the notion of a
person, place, or behavior. For example, Republicans are
white, rich, and drive Hummers. Exemplars are collections
Concentration of examples of concepts that are stored in memory. For
example, observing people at the mall defines the concept
Attention of an everyday person. Humans use several different
strategies to form concepts. Conservative focus involves
gradual manipulation of one aspect of a complex concept
at a time until the concept clarifies. Focus gambling tests
several possible hypotheses at the same time instead of
Concept Learning one at a time. Scanning involves forming global hypoth-
eses about a concept, then modifying them in accordance
R ICK PARENTE with rewards and punishments. Trial and error is the
Towson University tendency to just keep trying until something works.
Towson, MD, USA Aspects of the task that improve concept acquisition
include an orderly arrangement of the items that the
person must use to form the concept. A compact display
Synonyms in which all the aspects of the concept are present also
improves acquisition. Sequential problems that present a
Hypotheses; Rules consistent concept also improve concept acquisition. The
saliency, novelty, and complexity of the concept, and
whether feedback is provided affect how quickly a person
Definition learns it (Hunt & Ellis, 2003).
Current Knowledge
Concussion Epidemiology
M ATTHEW R. P OWELL , M ICHAEL A. M C C REA Current epidemiology statistics likely grossly underesti-
Neuroscience Center mate the true incidence and prevalence of concussion
Waukesha, WI, USA because of imprecise surveillance systems (e.g., many
individuals with concussive injury never seek medical
care and those who do seek care are frequently not hospi-
Synonyms talized), and because current estimates are clouded by
variable research methodologies and inconsistent defini-
Mild traumatic brain injury; Sport-related concussion tions of concussion (McCrea, 2008; National Center for
Injury Prevention and Control, 2003). As discussed in
greater detail under MTBI entry, best estimates would
Definition suggest an incidence of at least 100/100,000 and perhaps
as high as 500/100,0000 population depending on study
A concussion refers to a trauma-induced alteration in methodologies (McCrea, 2008).
neurological function. Giza and Hovda (2001) and
Hovda et al. (1990) discuss the abnormal neurometabolic
processes associated with concussion. Early clinical signs
Recovery and Outcome Post Concussion
of concussion include alteration of mental status or be-
havior, and can be accompanied by other symptoms out-
Concussions fall under the general rubric of MTBI, and are
lined below.
frequently graded by severity (e.g., grade 13 concussion).
More significant concussive injury is associated with great-
er length of altered consciousness, LOC, or amnesia, al-
Diagnosis though concussive injury often occurs without observed
LOC or measurable amnesia. Generally speaking, acute
Diagnosis of concussion is made based on identifying neurologic, behavioral, or cognitive symptoms begin
an event with adequate biomechanical force to cause a acutely and resolve within 710 days, with a small percent-
concussive injury and by examining acute injury severity age of individuals exhibiting symptoms beyond 3 months
indicators. Frequent events associated with a direct trauma (Belanger & Vanderploeg, 2005; Iverson, 2005; McCrea,
or blow to the head to cause concussion include a fall, 2008). Providers should expect a relatively rapid and full
motor vehicle accident, or sports collision. In addition to a recovery for patients following a single concussion. There
direct blow to the head, a concussion can result from rapid is no apparent difference in outcome across the various
acceleration, deceleration, rotational or percussive forces grades of concussion, though there are reports of longer
that affect brain tissue. Injury severity indicators frequent- recovery periods for complicated cases, such as those
ly used to diagnose concussion include alteration of with intracranial abnormalities identified via imaging
consciousness (confusion), loss of consciousness (LOC), (Williams, Levin & Eisenberg, 1990).
666 C Conditioned Stimulus
language comprehension, but significantly impaired rep- utterances (stereotypies). This is consistent with the
etition. Utterance length is normal or increased, and finding that the arcuate fasciculus is spared in many
speech has normal prosody and grammar and is produced patients with conduction aphasia (Quigg et al., 2006).
with normal effort. There is a reduction in content words, By contrast to the localization or modular view of
paraphasic errors are common, and oral reading is Geschwind and others, researchers in connectionist theo- C
impaired. ry have conceptualized language as the product of a
distributed cortical network that can be disrupted in
various ways to produce similar phenotypes. The advent
of functional imaging resurrected the notion of dis-
Categorization connection syndromes and more modular notions of lan-
guage (Catani & Ffytche, 2005), and the current view is
Conduction aphasia is differentiated from other types of somewhere in between a modular and connectionist
fluent aphasia (Wernickes aphasia, transcortical sensory perspective.
aphasia, and anomic aphasia) by the disproportionate
impairment in repetition relative to comprehension and
spontaneous production. It is differentiated from Wer-
nickes aphasia in particular by the patients awareness of Evaluation
his or her paraphasic errors.
The signs and symptoms of conduction aphasia will
be revealed only with testing of auditory and reading
comprehension, spontaneous language (e.g., asking the
Natural History, Prognostic Factors, patient to describe a picture), and repetition, so that
Outcomes the relative performance in these modalities can be
compared.
The prognosis for recovery of functional communication
in individual with conduction aphasia depends on the
underlying cause of the aphasia as well as factors such as
the size of lesion and the patients age, premorbid lan- Treatment
guage skills, and comorbid health conditions. Individuals
who initially present with conduction aphasia often There is no specific treatment for conduction aphasia,
evolve to a clinical profile of anomic aphasia, with rela- beyond therapies that are appropriate for aphasia in gen-
tively good auditory and reading comprehension, and eral ( Aphasia).
deficits primarily in word-finding and the comprehension
and production of complex syntax.
Cross References
Aphasia
Neuropsychology and Psychology of
Nonfluent Aphasia
Conduction Aphasia
Conduction aphasia historically has been attributed to the
interruption of white matter pathways, notably the arcuate References and Readings
fasciculus (part of the superior longitudinal fasciculus),
that connect posterior and anterior cortical structures Catani, M, & Ffytche D. H. (2005). The rises and falls of disconnection
involved in language comprehension and production. syndromes. Brain, 128(Pt 10), 22242239.
Thus, conduction aphasia historically was referred to as Geschwind, N. (1965). Disconnexion syndromes in animals and man. II.
a disconnection syndrome (Geschwind, 1965). Lesion Brain 88, 585644.
Goodglass, H. (1993). Understanding Aphasia. San Diego, CA: Academic
and imaging studies have revealed, however, that conduc-
Press.
tion aphasia may result from lesions in a variety of loca- Quigg, M., Geldmacher, D. S., & Elias, W. J. (2006). Conduction aphasia
tions, and that arcuate fasciculus lesions are instead as a function of the dominant posterior perisylvian cortex: Report of
implicated in the production of repetitive, stereotypic two cases. Journal of Neurosurgery 104, 845848.
668 C Confabulation
are unable to accurately monitor the source of their 4. Three-factor cognitive-neuropsychological theory.
memories which results in errors and confusion. Metcalf et al. (2007) propose a 3-factor combined
According to this view, patients who confabulate can- model that hypothesizes interplay between an execu-
not accurately identify where or when events occur. tive control retrieval deficit, an evaluation deficit, and
They may also be unable to differentiate between a persons individual emotional/motivational biases. C
whether a representation is real or not due to Specifically, according to this view confabulatory
impairments in being able to access qualitative symptoms and content are the result of a failure of
characteristics of the representation such as details executive control that causes an impaired search and
about perceptual, contextual, affective, and semantic selection of appropriate memories from the autobio-
information. Furthermore, secondary evaluation graphical episodic memory store. Confabulators are
deficits may also lead to the over-inclusive acceptance purportedly unable to critically evaluate material from
of memories. their autobiographical or general semantic store and
2. Temporal context theory. Dalla Barba, Mantovan, accept confabulations as real memories. Further,
Cappelletti, and Denes (1998) have suggested that the personal biases may be involved in the preferential
primary impairment in confabulation is a difficulty selection of memories that are emotionally biased.
with temporal consciousness. According to this
view, patients who confabulate are confused about the
temporal order of information retrieved from memory. Evaluation
While knowledge of time is preserved and patients are
aware of a past, present, and future, they are unable to Since Korsakoff s initial description in 1955 of pseudo-
make correct temporal judgments about their mem- reminiscences in alcoholic patients with amnesia who
ories, resulting in sequencing errors. Schnider (2003) made up fictitious stories about events that did not
has also argued that there is confusion between pres- occur, there has been considerable variability in how con-
ently relevant and irrelevant memories, resulting in a fabulation is defined, identified, assessed, and understood.
failure to suppress activated but presently irrelevant For instance, it remains debated whether confabulations
memory traces. Neuroanatomically, Schnider et al. are readily differentiated from delusions. Some have ar-
have linked confabulation with the posterior orbito- gued that confabulations are not different from delusions
frontal cortex and the anterior limbic structures di- since both are held firmly over time. Others have viewed
rectly connected with it. Schnider argues that the confabulations, delusions, hallucinations, and insight as
adaptation of thought and behavior to ongoing reality part of a continuum. Still others have argued that delu-
is mediated by the anterior limbic system which acts sions and confabulations differ since confabulations tend
by suppressing activated memory traces that do not to be associated with partially valid memories that can be
pertain to ongoing reality. In patients who confabu- traced to real events. Further debate ensues about whether
late, this monitoring of ongoing reality in thought confabulations are deliberate attempts to compensate for
goes awry and appears to be related to the brains memory gaps due to embarrassment or whether they
reward system. involve source monitoring, temporal context, or retrieval
3. Retrieval theory. This view has been primarily promoted difficulties that result in an implausible or fictional out-
by Moscovitch and Melo (1997) who argue that put. Confabulation, particularly following ACoA aneur-
deficient strategic retrieval processes and monitoring ysms has also been viewed as representing differences in
deficits influence confabulation. Specifically, confabu- degree and not kind. Other important variables identi-
lation is thought to be the result of a faulty strategic fied in the understanding of confabulation have included
retrieval search and inaccurate ordering and making the distinction between an unaware or
placement of memories in context. According to this aware process, premorbid personality factors, the
view, strategic retrieval is dependent upon executive need for coexistence of amnesia, differentiation from
processes and is self-initiated and goal-directed, while an acute confusional state, disconnection syndrome,
associative retrieval is automatic and independent of and whether indifference/apathy or deceit/lying is
executive processes. Confabulators are thought to involved (DeLuca, 2000). There has been further varia-
show deficiencies in strategic retrieval, influenced by bility in decisions on how to label an individual as a
deficiencies in the neocortical/prefrontal/hippocampal confabulator. Some have used the term based on clinical
network hypothesized to be involved in strategic observations of spontaneous or provoked production of
retrieval. fabricated stories following brain injury. Other researchers
670 C Confabulation
Treatment
Schnider, A., Ptak, R., von Daniken, C., & Remonda, L. (2000). Recovery References and Readings
from spontaneous confabulations parallels recovery of temporal
confusion in memory. Neurology, 55(1), 7483.
American Psychological Association. (2002). Ethical principles of psychol-
Victor, M., & Ropper, A. H. (Eds.). (2001). Principles of neurology
ogists and code of conduct. American Psychologist, 57, 10601073.
(7th ed.). New York: The McGraw-Hill Companies, Inc.
Committee on Ethical Guidelines for Forensic Psychologists (1991).
Specialty guidelines for forensic psychologists. Law and Human C
Behavior, 15, 655665.
Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (2007).
Confidentiality Psychological evaluations for the courts (3rd ed.). New York: Guilford.
R OBERT L. H EILBRONNER
Chicago Neuropsychology Group
Chicago, IL, USA
Confirmatory Bias
M OLLY E. Z IMMERMAN
Albert Einstein College of Medicine
Definition
Bronx, NY, USA
Confidentiality has been defined as containing informa-
tion whose unauthorized disclosure could be prejudicial to
the national interest. In psychology, it is one of the most
Definition
important components of the Ethical Principles of Psychol-
Confirmatory bias occurs when a clinician preferentially
ogists and Code of Conduct (2002) and the Specialty
accepts or seeks evidence that confirms an initial hypoth-
Guidelines for Forensic Psychologists (1991). The bound-
esis at the expense of thorough consideration of emerging
aries of confidentiality vary based on the setting, that is,
contradictory evidence (Garb, 2003). For example, a cli-
whether it is in the clinical versus forensic realm and
nician may formulate an initial impression that a client
whether it is in the civil versus criminal realm. In treatment
has dementia based on a referral question. During the
settings, clinicians consider confidentiality of paramount
clinical interview, the clinician may focus on questions
importance and they are reluctant to disclose information
relating to memory and change in activities of daily living
obtained from a client even when there are explicit legal or
while failing to ask questions that specifically pertain
countervailing ethical mandates to do so (such as when a
to differential diagnoses, such as major depressive
patient may harm another). Such a position is unrealistic in
disorder. In neuropsychological settings, this generally
the forensic context because the results of forensic evalua-
unintentional bias may impair judgment, hamper
tions (be it civil or criminal) are routinely disclosed to third
decision-making, produce false reports, and negatively
parties. In both clinical and forensic contexts, psychologists
impact the assessment approach and interpretation of
provide examinees with adequate informed consent
findings. To address this bias, the clinician should system-
prior to engaging in the examination. In the forensic con-
atically review all available data within a hypothesis-
text, it is the duty of forensic psychologists to inform clients
testing framework in an effort to remain as impartial as
of their legal rights regarding the purpose of an evaluation,
possible (Weiner & Greene, 2007).
the anticipated forensic service, the nature of procedures to
be utilized, the limits of confidentiality, who has retained
the examiner for the evaluation, who the report may be References and Readings
directed to, and that anything which is said during the
examination could be included in a report or come up Garb, H. N. (2003). Clinical judgment and mechanical prediction. In
during deposition or trial testimony. Some jurisdictions I. B. Weiner, D. K. Freedheim, M. Gallagher, J. A. Schinka, & R. J.
do not provide for confidentiality of mental health evalua- Nelson (Eds.), Handbook of psychology (pp. 2742). New York: Wiley.
Weiner, I. B., & Green, R. L. (2007). Handbook of personality assessment.
tions. For example, when an evaluation is conducted as a
New York: Wiley.
result of direct court order, confidentiality is not provided.
Word finding
Confounding Variables
J AMES H. B ANOS
Definition
University of Alabama at Birmingham
Confrontation naming involves the selection of a specific
Birmingham, AL, USA
label corresponding to a viewed stimulus, usually a
picture, of a viewed object or action. Confrontation
naming also refers to a type of task used in assessment
Synonyms
when problems with naming are of concern.
Confound; Extraneous variable; Lurking variable
Current Knowledge
Definition
Confrontation naming tasks often are incorporated as part
A confounding variable is an extraneous variable that
of clinical language testing to detect impairments of word-
obscures the true relation between two other variables or
finding abilities in individuals with various types of neuro-
groups of interest. In experimental research designs, a con-
logic impairments typically affecting the left hemisphere of
founding variable often presents as an unintended
the brain (Spreen & Risser, 2003). Although word finding
or undesirable systematic difference between groups
takes place during the course of sentence generation in
(the independent variable) that is also systematically related
conversational speech, it is most often tested clinically in
to the outcome of interest (dependent variable). It hinders
confrontation naming tasks where the vocabulary tested is
the ability to infer a causal relation between the variables
constrained to known, identified target words. Therefore,
and can lead to misattributing a causal effect to the inde-
word-finding functions are at times referred to as naming
pendent variable (a threat to internal validity). Potential
abilities (e.g., Chialant, Costa, & Caramazza, 2002).
confounding variables are most effectively addressed during
The most common published test of confrontation
study design (e.g., via random assignment, case control
naming is the Boston Naming Test (Kaplan, Goodglass,
matching), but may be addressed to some extent during
& Weintraub, 2001); other published confrontation
statistical analysis (e.g., handled as a covariate).
naming tests also exist for use in children and adults
with word-finding difficulties (An Object and Action
Cross References Naming Battery, Druks, & Masterson, 2000; Test of
Adolescent/Adult Word Finding, German, 1990). Con-
Analysis of Covariance (ANCOVA)
frontation naming tests assess the ability to retrieve
Error, Sources of
different types of words, including pictures of objects to
test noun retrieval, or pictures of actions to test verb
References and Readings retrieval. Although most tests of confrontation naming
test stimuli from a variety of semantic categories, some
Kazdin, A. E. (Ed.). (2003). Methodological issues & strategies in clinical
tests can differentiate pictures according to the semantic
research (3rd ed.). Washington, DC: American Psychological
Association.
category to which they belong, including pictures from a
Pearl, J. (2009). Causality: Models, reasoning, and inference. Cambridge: variety of natural categories such as animals, fruits, and
Cambridge University Press. vegetables, and man-made categories such as furniture,
Congestive Heart Failure C 673
References and Readings Common causes of CHF include coronary artery disease,
myocardial infarction, hypertension, cardiac valve dis-
Chialant, D., Costa, A., & Caramazza, A. (2002). Models of naming. ease, cardiomyopathy (heart muscle disease), congenital
In A. E. Hillis (Ed.), The handbook of adult language disorders heart disease, and endocarditis (heart infection). In CHF,
(pp. 123142). New York: Psychology Press. the heart continues to pump, but exercise and activity
Druks, J., & Masterson, J. (2000). An object and action naming battery.
cause shortness of breath (dyspnea on exertion or
Hove, UK: Psychology Press.
German, D. J. (1990). Test of adolescent/adult word finding. Austin, TX: DOE), fatigue, weakness, light-headedness, or syncope.
Pro-Ed. Because the heart is not pumping the blood completely
Goodglass, H., Kaplan, E., & Barresi, B. (2001). The assessment of aphasia and effectively, the blood backs up into the heart
and related disorders (3rd ed.). Philadelphia, PA: Lippincott, chambers, and ultimately into the venous system. This
Williams & Wilkins.
causes congestion of the tissues, and edema in the legs
Kaplan, E., Goodglass, H., & Weintraub, S. (2001). The Boston naming
test. Philadelphia: Lippincott, Williams & Wilkins. and internal organs, including the lungs (pulmonary
Laine, M., & Martin, N. (2006). Anomia: Theoretical and clinical aspects. edema), often resulting in shortness of breath, especial-
New York: Psychology Press. ly when lying supine (orthopnea). Electrocardiogram
Spreen, O., & Risser, A. H. (2003). Assessment of aphasia. Oxford: Oxford and certain blood tests are usually abnormal, and chest
University Press.
x-ray shows congestion of the lungs. Echocardiography
Tranel, D., Adolphs, R., Damasio, H., & Damasio, A. R. (2001). A neural
basis for the retrieval of words for actions. Cognitive Neuropsychology, is noninvasive and highly revealing, and has the potential
18(7), 655670. to provide a quantitative measure of severity of heart
failure as indicated by the proportion of blood in the left
ventricle that can be pumped out by the muscle (ejec-
tion fraction).
Congenital Defects CHF is a chronic condition with acute exacerbations
during which patients may experience moderate or signif-
Anencephaly icant distress. Chronic congestive heart failure can be
managed using a combination of interventions that help
the patient to compensate for the problems caused by
CHF. Acute pulmonary edema is a medical emergency.
Congenital Hypothryroidism Treatment of acute CHF episodes usually consists of rest,
salt reduction, identification, and removal of the precipi-
Hypothyroidism
tating factor (such as infection, cardiac arrhythmia, or
other cause), and the use of select medications that help
to improve the pumping ability (contractility) of the
Congestive Heart Failure heart muscle and the capacity of the blood vessels to
provide blood supply to other organs. These medications
E LLIOT J. R OTH include angiotensin-converting enzyme inhibitors, beta-
Northwestern University blockers, digoxin, diuretics (water pills), and vasodilators.
Chicago, IL, USA At times, valve disease warrants valve replacement sur-
gery, and extremely severe heart damage may require
cardiac transplantation to prevent death. Usually, mild
Synonyms to moderate congestive heart failure can be treated with
medications, adjustments of exercise and activity levels,
Heart failure and medical supervision.
674 C Conjugate Gaze
Cross References can result, which can then cause diplopia. The mechan-
isms for horizontal eye movements are better understood
Coronary Disease than vertical eye movements. As individuals age, the abil-
Echocardiogram ity to look upward tends to decline. Cerebral structures
control when and where the eyes move, and the brain
stem structures control how they move.
References and Readings The centers for lateral conjugate gaze are in the frontal
and occipital cortices. In the frontal lobe, this area is in the
Jessup, M., Abraham, W. T., Casey, D. E., Feldman, A. M., Francis, G. S., posterior aspect of the frontal lobes, referred to as the
Ganiats, T. G., et al. (2009). Focused update: ACCF/AHA guidelines frontal eye fields. This area is close to the motor strip.
for the diagnosis and management of heart failure in adults: A report The function of the frontal centers is to control voluntary
of the American College of Cardiology Foundation/American Heart
conjugate eye movements to the opposite side. The frontal
Association Task Force on Practice Guidelines, Developed in collab-
oration with the International Society for Heart and Lung Trans-
eye fields (FEF) receive inputs from peristriate, parietal
plantation. Circulation, 119, 19772016. and superior temporal cortex, medial pulvinar, and the
dorsomedial nucleus of the thalamus. Stimulation of FEF
results in contralateral saccades. In strokes that affect this
area, one may see an eye deviation toward the side of the
lesion and away from the paralyzed limb. This usually
Conjugate Gaze occurs only in the acute phase of an infarct. At some
point, the patient may be unable to move the eyes away
M ARY-E LLEN M EADOWS from the lesion on command. However, they may be able
Brigham and Womens Hospital to follow an object to the opposite side if the occipital lobe
Boston, MA, USA center is not damaged. The occipital lobe centers for
lateral conjugate gaze control eye movement when an
individual is following an object to the opposite side.
Synonyms Lesions of the occipital lobe that control lateral conjugate
gaze are less common than lesions of the frontal centers.
Eye movements; Versional movements Conjugate gaze can be disrupted by stroke or trauma,
depending on the location of the damage. For instance, an
intracerebral hemorrhage in the caudate nucleus or puta-
Definition men will cause conjugate deviation of eye movements to the
side of the lesion. Pineal tumors, which can press upon the
Conjugate gaze is the ability of the eyes to work together midbrain, can cause paralysis of upward gaze. Deeper dam-
or in unison. It refers to the motion of both eyes in the age can affect downward saccades. Given the extensive
same direction at the same time. The eyes can look later- anatomy of control of the visual system, damage or dys-
ally (left/right), upward, or downward. Disorders in con- function along any of the nuclei or tracts integral to eye
jugate gaze refer to the inability to look in a certain movements can result in abnormalities. Eye movements
direction with both eyes. utilize the basal ganglia and cerebellum in their planning
and coordination. Patients with basal ganglia disorders may
have involuntary, small, or slow eye movements. Patients
Current Knowledge with parkinsonian symptoms may have a restriction of
upgaze. In patients with progressive supranuclear palsy, a
Conjugate gaze is mediated in the brain stem by the cardinal feature is the restriction of upgaze, and these
medial longitudinal fasciculus, which is a nerve tract patients initially complain of difficulties with reading. The
that connects the abducens, trochlear, and oculomotor flocculus of the cerebellum is important for suppression of
nuclei. These nuclei, in turn, are responsible for the mus- vestibular reflex and for smooth pursuit movements. Parts
cles that control eye movements. The left pontine center of the vermis help to coordinate saccades, and damage can
connects with the right frontal center for conjugate gaze result in dysmetric saccades. Patients with long-standing
to the left, and the right pontine center connects with the frontal lobe lesions cannot inhibit inappropriate saccades
left frontal center for conjugate gaze to the right. If extrao- from a fixation to a peripheral, visually attractive stimulus
cular muscles are not working properly, dysconjugate gaze that appears suddenly. Frontal seizures with a focus in or
Conners 3rd Edition (Conners 3; Conners 2008) C 675
near one eye field may manifest by turning of the eye and from the individual. These are critical for binocular single
head away from the side of the lesion. vision and depth perception.
Evoked eye movements (dolls eye test or oculocepha-
lic reflex) and the caloric response can be used in the
examination of an unresponsive patient. However, if a Cross References C
patient is in a comatose state due to drug intoxication
or hypothermia, these tests may show no response. The Dysconjugate Gaze
assessment is as follows: Lateral Gaze Palsy
Dolls eye test This test can be used in either the Saccadic Eye Movements
comatose or conscious patient. Hold the patients eyes
open and rapidly move/rotate the head to one side and
hold it there. If the brain stem reflexes are intact, the eyes References and Readings
will move conjugately in the direction opposite to the head
rotation. If the injury is in the brain stem, the eyes do not Lavin, P. J. M., & Weissman, B. (2000). Neuro-Opthalmology. In W. G.
Bradley, R. B. Daroff, G. M. Fenichel, & C. D. Marsden (Eds.),
move. If a patient is conscious and can follow commands,
Neurology in clinical practice: Principles of diagnosis and management.
have them fixate on an object. This test should not be used Boston, MA: Butterworth-Heinemann.
in a patient who has possible cervical injuries. Marshall, K. G. (2006). Cranial nerve III, IV and VI: Disorders of conju-
Caloric test This test can be done in the comatose gate Gaze. Patient Care Canada, 17, 5157.
patient. The patients ear canal is irrigated with 20 ml of Ross, R. T. (1999). How to examine the nervous system (3rd ed.). Stamford,
CT: Appleton and Lange.
ice-cold water. The eyes should move toward the ear that
is irrigated. If the patients eyes do not move, the lesion is
in the brain stem. This test should not be used in a patient
who has possible cervical injuries, or who has blood in the
ear canal or a perforated eardrum. Connectionist Model of Aphasia
Conjugate eye movements allow the eyes to get an
image onto the fovea and keep it there. Fast movements WernickeLichtheim Model of Aphasia
or saccades allow images onto the fovea and slower move-
ments keep them there. Smooth pursuit movements com-
pensate for target movement. There are several ocular
motor systems: Connective Tissue Disease
Saccadic system moves the eyes rapidly (up to 800 /s)
in order to fixate on new targets in the visual fields. These Collagen Vascular Disease
can be voluntary or a response to a verbal command.
Reflex saccades can also occur to stimuli that are threat-
ening or to a sound in the periphery.
Pursuit system enables the eyes to track slowly moving
targets (approximately 70 /s) so the image is stable on the Conners 3rd Edition (Conners 3;
fovea. Conners 2008)
Vestibular eye movement subsystem maintains a stable
image on the retina during head movements. The vesti- C. K EITH C ONNERS 1, J ENNI P ITKANEN 2, S ARA R. R ZEPA 2
1
bulo ocular reflex maintains the eyes in the same direction Duke University Medical School
in space during head movements. This is controlled by the Durham, North Carolina, USA
2
semicircular canals, which respond to rotational accelera- Multi-Health Systems Inc.
tion of the head. Toronto, Ontario, Canada
Optokinetic system complements the vestibular eye
movement system. It uses reference points in the environ-
ment to maintain orientation. This system uses fixation Description
and pursuit in humans.
Vergence system enables the eyes to move dysconju- The Conners 3rd Edition (Conners 3; Conners, 2008), the
gately (converge and diverge) in the horizontal axis to latest version of the Conners Rating Scales, is a thorough
maintain fixation on a moving target toward or away assessment of attention deficit/hyperactivity disorder
676 C Conners 3rd Edition (Conners 3; Conners 2008)
(ADHD) and its most commonly associated problems (including all three ADHD subtypes: ADHD predomi-
and disorders in school-aged youth. The Conners 3 is a nantly inattentive type, ADHD predominantly hyperac-
multi-informant assessment with forms for parents, tive-impulsive type, and ADHD combined type),
teachers, and youth. Parent and teacher ratings can be disruptive behavior disorders, learning disorders, anxiety
obtained about youth aged 618 years, and youth aged disorders, major depressive disorder, bipolar disorder, and
818 years can complete the self-report. pervasive developmental disorders.
The assessment features multiple content scales that
assess ADHD-related concerns such as inattention and
hyperactivity as well as related problems in executive func- Historical Background
tioning, learning, aggression, and peer/family relations.
Symptoms of distinct diagnoses can be assessed with scales The first versions of the Conners Parent and Teacher
that link directly to the Diagnostic and Statistical Manual Rating Scales were developed at the Harriet Lane Clinic
of Mental Disorders (DSM-IV-TR; American Psychiatric of the John Hopkins Hospital in the 1960s during
Association, 2000) symptomatic criteria for ADHD, con- Dr. Leon Eisenbergs controlled studies of psychotherapy
duct disorder (CD), and oppositional defiant disorder and medications. The purpose of the original scales was to
(ODD). Anxiety and Depression Screener items are also serve as the basis for a detailed interview about a childs
included in order to provide coverage of two internalizing problems. The first version of the parent scale contained
problems frequently associated with ADHD. The Conners items grouped in terms of problems (e.g., sleep, eating,
3 also provides two index scores: the Conners 3 ADHD temper, friendships, school). The teacher scale included
Index and the Conners 3 Global Index. The assessment items related to functioning within the classroom setting
also includes validity scales (positive impression, negative (e.g., group participation, attitude towards authority).
impression, and inconsistency index), severe conduct crit- The earliest research studies on the scales supported the
ical items, impairment items (home, school, and social research properties of the scales. For example, the very
settings), and open-ended additional questions (addition- first study on the teacher scales (Conners, 1969) demon-
al concerns, strengths). strated adequate test-retest reliability. In addition, good
The Conners 3 offers full-length, short, and index form sensitivity to drug treatment and nondrug therapy effects
options. The full-length forms convey the most detailed were established.
information of all the forms. The short forms are useful The scales were first formally published in 1989 as the
when the administration of the full-length version is not Conners Rating Scales (CRSTM, Conners, 1989, 1990) and
possible or practical (e.g., when multiple administrations offered a standard format with normative data, detailed
over time are required). The index forms are useful in information about the psychometric properties and the
screening and treatment monitoring situations. Adminis- proper use of the scales, and the hand-scorable
tration requires approximately 20 min for the full-length QuikScoreTM form allowing for easy administration
forms, 10 min for the short forms, and approximately 5 and scoring. The CRS was later revised (CRS-R; Conners,
min for the index forms. Raw scores are converted to age- 1997) to offer a self-report component and scales
and gender-based standard scores (including T-scores and linked to the Diagnostic and Statistical Manual of Mental
percentiles). Results from the DSM-IV-TR symptom Disorders Fourth Edition (American Psychiatric Associ-
scales are also reported in terms of symptom counts, ation, 1994) criteria for ADHD. The Conners 3, the latest
that is, whether or not the minimum symptom require- revision of the Conners Rating Scales, incorporates the
ments set by the DSM-IV-TR have been met. The Conners key features of its predecessors with updated normative
3 ADHD Index produces a probability score, which indi- data and psychometric properties, an age range specific
cates whether the youths scores are more like youth with to the assessment of school-aged children, and an
ADHD, or with those from the general population. increased focus on the assessment of ADHD, associated
The Conners 3 normative sample consists of 3,400 features, and the disorders most commonly comorbid
assessments (1,200 parent, 1,200 teacher, and 1,000 with ADHD.
self-report assessments) including 50 boys and 50 girls
from each age (618 years for the parent and teacher
report, 818 years for the self-report). The racial/ethnic Psychometric Data
distribution of this sample closely matches the US
population. Approximately 2,100 clinical cases were also Results of reliability analyses revealed that the Conners 3
collected from youth with following diagnoses: ADHD forms have high levels of internal consistency, with
Conners 3rd Edition (Conners 3; Conners 2008) C 677
Conners 3rd Edition (Conners 3; Conners 2008). Table 1 Overview of Correlations between the Conners 3 and Other Measures
Conners Comprehensive Behavior Rating Scale. Table 1 Overview of correlations between the Conners CBRS
and other measures
membership effects (as determined with partial Z2) were Conners, C. K. (2008). Conners 3rd Edition manual. Toronto, Ontario,
Canada: Multi-Health Systems.
moderate to large, on average, accounting for 14.5% of
Individuals with Disabilities Education Improvement Act of 2004
the variance in scores. In terms of the classification (IDEA), Pub. L. No. 108446, 118 Stat. 2647 (2004). [Amending
accuracy of the scores (as determined by a series of 20 U.S.C. 1400 et seq.].
discriminant function analyses), the mean overall correct
classification rate was 78% across all forms.
The authors report that the psychometric properties of Conners, C. K. (1989). Conners Rating Scale Manual. New York: Multi-
the revised scale are adequate as demonstrated by good Health Systems, Inc.
Conners, C. K., Erhardt, D., & Sparrows, E. (1999). Conners Adult ADHD
internal reliability coefficients, high testretest reliability, C
Rating Scales (CAARS). North Tonawanda, NY: Multi-Health
effective discriminatory power, and predictive and struc- Systems, Inc.
tural validity. Various researchers have suggested three Conners, C., Sitarenois, G., Parker, J. D., & Epstein, J. N. (1998).
main areas for the application of Conners scales: as a The revised Conners Parent Rating Scale (CPRS-R): factor struc-
general screening tool for the detection of problematic ture, reliability, and criterion validity. Journal of Abnormal Child
Psychology, 26, 257268.
behaviors in children, as a complimentary tool for clarify-
Conners, C., Sitarenois, G., Parker, J. D., & Epstein, J. N. (1998). Revision
ing a specific diagnosis, and as a measurement tool for the and restandardization of the Conners Teacher Rating Scale (CTRS-R):
assessment of treatment results. Factor structure, reliability, and criterion validity. Journal of Abnor-
mal Child Psychology, 26, 279291.
Clinical Uses
The Conners scales are designed to assess symptoms as
reported by various informants (e.g., parents, teachers,
self-report for adolescents) using corresponding factor
Conners Continuous
structures. There are also short and long forms that vary Performance Test (CPT)
from approximately 20 to 80 items. The Conners ratings
have also been compared across cultures, including (for C. K EITH C ONNERS 1, G ILL S ITARENIOS 2
1
example) Italian, British, Chinese, Brazilian, and New Duke University Medical School
Zealand cultures and, consequently, translated into a num- Durham, NC, USA
2
ber of different languages, including Sudanese Arabic, Multi Health Systems Inc.
Turkish, and Hindi. Toronto, ON, Canada
The Conners Adult ADHD Rating Scale (CAARS) was
more recently developed to assess ADHD symptomato-
logy in adults. The rating scale was designed to be com- Description
pleted by respondents about themselves or about a
significant other. Factor analyses used to select items and The Conners Continuous Performance Test - 2 (CPT-2;
determine the structure of the CAARS scale were Conners, 2000) is a computer administered test that is
based on data from a derivation sample consisting of designed to assess problems with attention. The Con-
839 normal adults and from a clinical sample consisting ners CPT-2 presents 360 stimuli trials (i.e., individual
of 167 adult. Four major dimensions were reported: three letters) on the screen, with 1, 2, or 4 s between the
corresponded to the core features of ADHD seen in chil- presentation of letters (ISI: Inter-Stimulus Interval).
dren and the fourth, a secondary consequence of ADHD: The 360 trials are divided into 18 blocks of 20 trials
(1) Inattention/Executive Functioning, (2) Hyperactivity/ each. The ISIs are counterbalanced across these blocks.
Restlessness, (3) Impulsivity/Emotional Lability, and (4) Respondents are instructed to press the spacebar or the
Problems with Self-Concept. Internal reliability coefficients appropriate key on the mouse for any letter that
for the CAARS scales for four different age groups (18 years appears, except the letter X. The CPT takes fourteen
plus) were reported to be excellent (coefficient alphas min to administer excluding the recommended practice
ranging from 0.86 to 0.92 for males and females) and test. Over the duration of the test, 324 non-X stimuli
testretest reliability over a month was felt to be strong (i.e., targets) appear, and the letter X (nontarget)
(0.800.91). appears 36 times. One of the primary advantages of
this paradigm is the high proportion of targets that
appear, yielding a larger pool of responses for generat-
Cross References ing the statistical output. Many statistics are computed
including omission errors (i.e., failing to respond to a
Conners 3rd Edition (Conners 3; Conners 2008) target letter), commission errors (i.e., responding to a
Conners Comprehensive Behavior Rating Scales nontarget), hit reaction time (the average speed of
682 C Conners Continuous Performance Test (CPT)
correct presses to target letters), hit reaction time stan- was initially released as a DOS application (Conners
dard error (which assesses the consistency of responses CPT 3.0; Conners, 1994), and later renormed and
to targets), detectability (the ability to discriminate converted into a windows application (Conners, 2000).
between targets and nontargets), response style
(assesses speed vs accuracy response tendencies), per-
severations (reaction times less than 100 ms), hit reac-
tion time by block (change in reaction time as the test Psychometric Data
progresses), standard error by block (change in re-
sponse consistency as the test progresses), reaction Reliability. Split-half reliability results were computed for
time by ISI (change in mean reaction time across the original standardization sample (Conners, 1994,
ISI), and standard error by ISI (change in response 2000). The mean split-half reliability was 0.83 (range
consistency across ISI). These statistics are converted 0.66 to 0.95). Test-retest reliability is reported in Conners
to T-scores and can be interpreted in terms of various (2000) and is based on a small sample (n = 23). Test-retest
aspects of attention including inattention, impulsivity, correlation was high for the confidence index (r = 0.89),
and vigilance. The CPT-2 also provides a confidence and moderate to high for omissions, commissions,
index probability (expressed as a percentage out of 100) Hit Reaction Time, Hit Reaction Time Standard Error,
of the results being from a clinical as opposed to a non- Variability, Detectability, and Response Style (range
clinical case. r = 0.43 to r = 0.84). The Block change and ISI change
T-scores are computed using General Population (not statistics were less consistent across test administrations
pre-identified with a clinical condition) norms (Conners, (r = 0.05 to r = 0.51).
2000) obtained for children and adults aged 6 years and Validity. Validation of the CPT-2 focused on its two
above. The test is based on a total of 1,920 normative primary clinical uses: to help identify clinical problems
cases, out of which 52.8% of the cases were female, 47.0% with attention or potential neurological impairments and
were White, 27.0% Black, and 21.4% of other ethnic to monitor treatment effects. Conners (2000) compares
origin. Demographic information for age is shown in means from the General Population normative data
Table 1. T-scores are computed based on the age and (n = 1483), and ADHD cases (n = 271) in an ANCOVA
gender of the respondent. Data was also collected for controlling for age and gender for 617 year olds.
378 ADHD cases, and 223 neurologically impaired cases Performance of the ADHD group was significantly worse
(e.g., post-concussive, other organic brain syndrome, on the CPT-2 for all of the measures except commissions
dementia). where no significant difference was found. Discriminant
Function Analysis was also used to examine classification
accuracy for the CPT-2. For 617 year olds, sensitivity is
Historical Background reported at 83% and specificity at 82%. Similar analyses
were conducted on the adult samples. In addition to
The acronym CPT was first introduced by Rosvold, General Population data (n = 437), ADHD data
Mirsky, Sarason, Bransome, and Beck (1956) who used a (n = 107), neurologically impaired data was also available
particular version to detect attention lapses in subjects (n = 223). Performance of the ADHD group and
with petit mal epilepsy. In early CPTs such as the one neurologically impaired group was significantly worse
they used, subjects were required to press a key when a than the General Population group for all of the CPT-
target letter (e.g., X) appeared or when the target 2 measures. The neurologically impaired group also
letter appeared preceded by another letter (e.g., AX). scored significantly worse than the ADHD group on
While the CPT was being explored in clinical settings, several measures. They made significantly more omission
Mackworth (1957) initiated basic vigilance studies. errors, had significantly slower reaction times, had more
By changing basic test parameters, different CPT variable reaction times, and their standard error was
paradigms could be constructed. The CPT-2 paradigm affected more by letter presentation speed (ISI).
Conners Continuous Performance Test (CPT). Table 1 Age distribution of CPT-2 Normative Sample
Age 67 89 1011 1213 1415 1617 1834 3554 55+
n 88 283 369 258 261 224 237 146 54
Consciousness C 683
feed-forward organization. Another major impetus for con- In contrast to AD, patients with certain other degenera-
sciousness becoming an acceptable topic for scientific in- tive conditions often show general preservation of con-
quiry was advances in the field of neuroscience itself. sciousness despite having marked impairments of other
Functional imaging methods have made it easier to measure functions. For example, patients with severe Parkinsons
brain responses associated with subjective cognitive states, disease typically have major motor symptoms, cognitive C
providing evidence that these states have valid neurobiolog- control problems, including executive and attention dys-
ical underpinnings. Furthermore, growing recognition function. They may also show blunting of emotional re-
of the brains complexity has played an important role. sponse and altered emotional functioning. Yet, they are
There is now general consensus that cognition involves often painfully aware of their deficits, and have greater
interactions among large networks of cortical neurons, preservation of sense of self than the AD patient. Similarly,
which communicate across larger functional systems in an patients with Multiple Sclerosis (MS) sometimes exhibit
integrated manner. This perspective enabled cognitive neu- pseudobulbar affect. They are affectively labile with dramat-
roscientists to shift away from having to view consciousness ic shift in the outward expression of emotion, but yet they
as a unitary process sitting on top of the pyramid of cogni- report not feeling the emotion that corresponds to this
tive functions. expression. Despite these problems with affective and be-
Several lines of research and clinical evidence have had havioral control, patients with MS typically are very aware
particular bearing on current knowledge regarding the of their deficits and may feel locked in because of their
neuropsychology of consciousness. The effect of various symptoms.
types of drugs, most notably hallucinogens, opiates, and Alterations in self-awareness and consciousness also
alcohol on conscious experience provides one of the most occur as a result of damage to specific brain regions. This
clear cut illustrations of the fact that even among healthy fact is significant since it suggests that certain functional
people consciousness is not a static phenomenon. Ones anatomic systems play an important role in the experience
sense of reality, identify and self-awareness can be dra- of consciousness, though probably not as a function of
matically altered by drugs such as LSD, presumably any one of these systems alone. Subcortical lesions affect-
by altering the flow of information processing. Case stud- ing the mid-brain, brain stem and thalamus often cause
ies have consistently shown that disruption of self-aware- dramatic alterations in level of consciousness. The reticu-
ness as a function of damage to specific brain regions. lar system which generates ascending activation to cortical
Furthermore, clinicians working with patients experien- areas plays a well established role in this phenomenon,
cing neurodegenerative dementias have long been aware with damage to this system contributing to problems with
of dramatic impoverishment in the quality of conscious arousal and alertness. Damage to these areas frequently
experience as the disease progresses, corresponding underlies coma, and the electrical activity measured by
with the extent of cortical atrophy. Among patients with EEG is amplified by generators in these systems. Persistent
Alzheimers disease (AD) and other neuronal dementias, a vegetative state is a clinical illustration of this, as these
loss of self is one of the hallmark features of the end- unfortunate patients exhibit impaired higher cortical
stage condition. In fact, it is when AD patients lose their functions, have persevered circadian sleep-wake cycles
ability to respond to people who are familiar and mean- and autonomic function. Compared to healthy people
ingful to them, and when they lose awareness of their own who are awake, patients with this syndrome have im-
identify, their personal memories, and the nature of their paired connectivity between brainstem and thalamic
cognitive problems that families typically experience areas and the cortex. This results in a reduced cortical
greatest despair. In fact, it is often this change, along activation, with generalized slowing consistent with coma.
with an inability for self-care that leads to nursing home These subcortical areas are clearly essential for maintain-
placement rather than the amnestic disturbance per se. ing normal consciousness, since disconnecting them from
The loss of identity in AD patients usually corresponds cortical areas impairs alertness, attention, and other cog-
with personality changes and profound impoverishment nitive functions linked to consciousness. The activation
in abstraction and associative ability. While other cogni- and arousal associated with this functional anatomic sys-
tive functions like language are often also severely tem makes an essential contribution to is a necessary part
impaired at this point, it may be difficult to fully appreci- of the maintaining consciousness, thought this activation
ate these changes, though the quality of emptiness and is not sufficient to account for the full dynamic of con-
loss of self that may ultimately be the characteristic that sciousness (i.e., self-awareness and sense of identity).
best defines severe dementia rather than impairment in a The thalamus appears to play an important role in
particular cognitive domain. consciousness, but as a convergence site of reticular
686 C Consciousness
activation from lower brain systems and because of its There is strong evidence from studies of neglect syn-
role in integrating this activation with cortical input. drome that medial frontal and anterior cingulate cortical
Bilateral ablation of the central medial zone of the intra- areas play a major role in intention (Cohen, 1993). The
laminar nucleus of the thalamus produces coma and intent and plan for action seems to be an important
persistent vegetative states. This thalamic area is affected element of consciousness, as this capacity is linked to
by general anaesthetics and drugs that cause sedation, the experience of drive and spontaneity. An illustration
suggesting that it is necessary for anti-psychotic drugs. of this is seen in patients who undergo cingulotomy, as
This evidence suggests that a functioning thalamus is their primary long-term neurocognitive deficit involves
necessary, but not sufficient, for human consciousness, diminished performance on tests requiring intention, and
though again this area alone is not sufficient to account also reduced spontaneity, initiative, and creative impulse.
for all aspects of consciousness. Cortical and limbic func- It is also noteworthy that syndromes that result in altera-
tions are necessary elements of normal consciousness. tions of consciousness that involve delusions and a frac-
Patients with bilateral occipital damage who exhibit tured sense of reality, such as reduplicative para-amnesia
blind sight are clearly experiencing some alteration in and Capgrass syndrome, tend to occur among patients with
consciousness and self-awareness. Yet, this disturbance frontal lobe damage, often involving the non-dominant
may be relative focal and limited to vision. For these hemisphere. In cases of reduplicative paramnesia, patients
individuals, altered consciousness has not affected all may feel with certainty that while the house in which they
aspects of self-awareness and identity. Damage to other are living looks identical to their real home, that it is in fact a
cortical areas may also produce specific impairments of replica of their home. When queried these patients usually
awareness and self-consciousness. For example, the ne- acknowledge the logical implausibility of their belief, but
glect syndrome commonly associated with right inferior state that they are certain because of some feeling they have,
parietal damage often results in anosognosia and im- suggesting a relationship between emotional response and
paired awareness of what is being neglected. For some of the processing of these frontal systems. Primate studies
these patients, the alteration in awareness and conscious- support the role of the frontal cortex in this regard, and
ness extends beyond the limits of the spatial disturbance led Crick and Koch (1995) to propose that consciousness is
that is observed. Yet, typically patients with these lesions largely dependent on the resonance of thalamo-cortical
do not lose their sense of self completely. They may show systems acting in a recursive manner.
diminished ability for self-reflect, but usually show abil- The role of memory and its underlying functional
ities in this regard as long as not tied to the types of spatial neuroanatomic systems raises a number of interesting
processing that is affected. issues for consciousness. Patients like HM, who have
The frontal cortex seems to have be the cortical area suffered bilateral damage to hippocampal or paralimbic
with greatest influence on consciousness, self-regulation areas, lose their ability to form new episodic memories.
and self-referential processes. The famous case of Phineus Yet, they typically retain retrograde memory for events
Gage, the railroad worker who suffered severe frontal that happened earlier in their life, which enables them to
damage when a spike penetrated his brain provided his- retain some sense of personal identity based on these
torical illustration of this. Following his injury Gage recollections. Yet, they have lost the ability to update
became indifferent to social consequences and showed these memories or to adapt these memories to new cir-
little self-regard for his own behavior (Harlow, 1848). cumstances. Clearly this results in an alteration of con-
While it is impossible to accurately evaluate the nature sciousness, though they may still report knowing who
of change in his subjective sense of consciousness and self- they are or what they think about certain topics.
awareness based on the historical record, it seems clear In addition to the contribution of specific cortical
from clinical descriptions that his self-awareness was dra- and subcortical areas to the experience of consciousness,
matically altered. In modern case study, Eslinger and his there is a rich history of research on brain laterality,
colleagues (1986) have demonstrated impaired empathy specifically the effects of disconnection between the left
associated with frontal lobe disturbance, which affects and right hemisphere of the brain. The corpus callosum
social reasoning, judgment and perspective taking. Inter- provides the white matter linkage between the hemi-
estingly, this disorder of empathy also involves problems spheres. Roger Sperry observed remarkable alterations
with moral reasoning, a notion consistent with early phil- in consciousness and awareness among patients who
osophical ideas regarding consciousness, which as dis- underwent surgical severing of the corpus callosum
cussed earlier conceptualized consciousness as tied to (split brain) for treatment of intractable seizures. When
mans capacity for moral knowledge. the two hemispheres are no longer able to communicate,
Consciousness C 687
each processes and interprets information presented to it likely play a significant role, by linking emotional valence
in different ways. For example, the left hemisphere is to particular associative information. In interaction with
typically more involved in language processing, and cortical areas, these limbic areas enable amplification of
therefore tended to be aware of verbal elements of infor- affective signals, and also their regulation, providing for
mation during tasks compared to the right hemisphere. the intensity and richness of emotional affective experi- C
Because of a lack of integration between the cortical ence. (5) Some type of mechanism that enables temporal
hemispheres, it is possible to dissociate elements of con- binding of associative experiences in an integrated way
sciousness tied to each side of the brain. Yet, following likely occurs that creates the feeling of a unified conscious
this procedure, it was only under experimentally con- experience and sense of single identity, I.
trolled conditions that major dissociations occurred. Some of the distinctions that exist between theories of
Post-surgically, people still experienced a single identity consciousness relate to the emphasis that is placed on
in everyday experience, suggesting that splitting the two particular cognitive functions. Some recent investigators
hemispheres does not completely disrupt the sense of a have emphasized the role of posterior brain systems
unified consciousness. involved in visual processing to a greater extent. A num-
ber of theories emphasize the role of frontal brain systems.
For example, one of the first neuroscientific models of
Neuropsychological Models of consciousness proposed by Shalice (1978) viewed con-
Consciousness sciousness as a byproduct of dominant action systems
that govern the stream of thought processes. As discussed
Consideration of the neuropsychological literature to date previously, many models have focused on frontal systems
has established the foundations for the cognitive neuro- with varying degrees of emphasis on particular processes.
science of consciousness. Over the past 15 years, theoreti- Damasios model of consciousness emphasizes the critical
cal models have been developed to explain how the role of emotional processing systems, as well as the so-
brain creates mind and consciousness. These models matic or visceral activity of the body as a whole.
include contributions from philosophers (Dennett, 1991; Perhaps the greatest distinction between theories of
Churchland, 2005), neuropsychologists/behavioral neu- consciousness stems from the extent to which it is
rologists (Cohen, 1993; Damasio, 1995, 2000) and cogni- viewed as distinct from the physical processes that com-
tive neuroscientists (Shallice, 1978; Eccles, 1994; Crick prise, or essentially indistinguishable from these physical
and Koch, 1995; McClelland et al., 1997; Grossberg, processes, as Dennett argues. Furthermore, some of these
1999). It is beyond the current scope to review each of models take more of a top-down approach, whereas others
their models. However, there are several features common view consciousness from a bottom-up perspective, as an
to each, as well as a few fundamental distinctions between emergent byproduct of all of the more basic associative
these models, as summarized below. processes that underlie cognition. The PDP approach of
Most current models of consciousness take the per- McClelland and Rummelhart (1986, 1997), and other neu-
spective that consciousness is not localized to a single rocomputational theories (e.g., Grossberg, 1999) take a
brain region, but rather is an emergent function of multi- more bottom-up approach and tend to view conscious-
ple systems acting in an integrated fashion. (1) Almost all ness as the sum total of these distributed processes. De-
researchers and theorists agree that lower brain areas (e.g., spite these differences, there seems to be little
reticular system) that energize the brain and lead to alter- disagreement that consciousness is a complex cognitive
ing levels of activation are a necessary requirement for phenomenon that results from the interaction of multiple
consciousness, but not sufficient to explain the phenome- brain systems and processes, which in an integrated co-
non. (2) The thalamus seems to play and essential role, herent manner provides for the sense of self, universally
particularly as a nexus for this activation and for integra- experienced by healthy humans.
tion and gating of cortical information. (3) Frontal
regions appear to play several important roles, including
the generation and sustaining of intention, switching and Future Directions
gating of information, and the enabling of feedback, and
feed-forward mechanisms that enable reprocessing of cer- The cognitive and neurobiological bases of consciousness
tain associative information and feedback control for remain major frontiers in neuropsychology. Clinicians
gating of this information as it flows from posterior evaluating patients following acute brain injury routinely
cortical systems. (4) Limbic areas, such as the amygdala, assess level of impaired consciousness is (i.e., the degree of
688 C Consolidation
coma, stupor or lethargy), though this level of analysis Crick, F., & Koch, C. (1990). A framework for consciousness. Nature
Neuroscience, 6(2), 119126.
does not fully capture the richness of normal conscious
Damasio, A. R. (1995). Consciousness. Knowing how, knowing where.
experience. None the less, clinical research is likely to yield Nature, 375, 106107.
greater precision in the assessment of levels of Damasio, A. R. (2000). The feeling of what happens: Body and emotion in
consciousness. the making of consciousness. New York: Vintage.
Some progress has been made in understanding how Damasio, H., Grabowski, T., Frank, R., et al. (1994). The return of
Phineas Gage: Clues about the brain from the skull of a famous
consciousness arises as a by-product of other cognitive
patient. Science, 264, 11021105.
functions, most notably emotional experience, attention Dennett, D. (1991). Consciousness explained. Boston: Little & Company.
and executive functioning. Computation neuroscience Eccles, J. C. (1994). How the self controls its brain. Berlin: Springer.
has offered a perspective for explaining consciousness as Eslinger, P. J. (1998). Neurological and neuropsychological bases of em-
an emergent property of complex neural systems. Yet, the pathy. European Neurology, 39, 193199.
Grossberg, S. (1999). The link between brain learning, attention, and
measurement of the qualitative experience that comprises
consciousness. Consciousness and Cognition, 8, 144.
consciousness is still far from becoming part of main- Harlow, J. M. (1848). Passage of an iron rod through the head. Boston
stream neuropsychological assessment, or from being Medical and Surgical Journal, 39, 389393.
fully operationalized as a cognitive construct or neuropsy- Harlow, J. M. (1868). Recovery from the passage of an iron bar through
chological domain. Greatest progress has been made with the head. Publications of the Massachusetts Medical Society, 2,
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respect to the study of anosognosia, and recent efforts to
McClelland , J. L., Cohen, J. D., & Schooler, J. W. (1997). The neural basis
better understand the relationship between subjective ex- of consciousness and explicit memory: Reflections on Kihlstrom, Man-
perience of cognitive impairments and also cognitive and dler and Rumelhart. Scientific approaches to consciousness (pp. 499
emotional experience and neuropsychological perfor- 509). Lawrence Erlbaum Associates.
mance. This seems to be a fertile area of continued inves- Rumelhart, D. E., McClelland, J. E., & the PDP Research Group (1986).
Parallel distributed processing: Explorations in the miccrostructure
tigation. Functional imaging approaches have provided
of cognition, Vol. 1: Foundations. Cambridge, MA: Bradford/MIT
considerable insight into the neural bases of conscious- Press.
ness and likely will continue to be on the cutting edge of Shallice, T. (1978). The dominant action system: An information proces-
these efforts, since they provide a means for observing sing approach to consciousness. In K. S. Pope & J. L. Singer (Eds.),
brain response associated with subjective experience. Evi- The stream of consciousness: Scientific investigations into the flow of
experience. New York: Plenum.
dence of the attentional default network that emerged
Wundt, W. (1902). Outlines of psychology (Trans., 2nd ed.). Oxford.
from functional imaging research illustrates the type of Engelmann:
insight about the neural bases of consciousness that can
be achieved from these approaches.
Cross References
Consolidation
Anosognosia
Attention E LIZABETH S TANNARD G ROMISCH
Capgras Syndrome Trinity College
Minimally Conscious State Hartford, CT, USA
Parallel Distributed Processing
Reduplicative Paramnesia
Reticular Activating System Synonyms
Split-Brain
Memory consolidation
Sterpenich, V. et al. (2009). Sleep promotes the neural reorganization of nition (delayed) conditions, and a constructional praxis
remote emotional memory. The Journal of Neuroscience, 29(16),
test that requires examinees to copy four line drawings of
51435152.
Takashima, A., et al. (2005). Declarative memory consolidation in
increasing complexity (Rosen, Mohs, & Davis, 1984).
humans: A prospective functional magnetic resonance imaging Administration time for the battery is 2030 min for
study. Proceedings of the national academy of sciences of the United persons with AD; persons without AD may complete the
States of America, Vol. 103, no. 3, 756761. battery in less time (Morris et al., 1989). As may be
Tronson, N. C., & Taylor, J. R. (2007). Molecular mechanisms of memory
expected given the brevity of the battery and the range
reconsolidation. Nature Reviews Neuroscience, 8, 262275.
Weingartner, H., & Parker, E. S., (Eds.). (1984). Memory consolidation:
of cognitive ability of the target populations, floor and
psychobiology of cognition. Hillsdale, NJ: Lawrence Erlbaum ceiling effects have been noted for individuals with later
Associates. stage AD and for individuals without AD, respectively
(Morris et al., 1989). Normative data are available based
on the CERAD program control sample (Welsh et al.,
1994), as well as from studies of specific demographic
groups (see Clinical Uses).
Consortium to Establish a
Registry on Alzheimers Disease
Historical Background
S USAN VANDERHILL 1, E STHER S TRAUSS 1
E LISABETH M. S. S HERMAN 2
1 The Consortium to Establish a Registry for Alzheimers
University of Victoria
Disease (CERAD) was first funded by the National Insti-
Victoria, BC, Canada
2 tute on Aging in 1986 in response to a US Congressional
University of Calgary
mandate to collect epidemiological data concerning the
Calgary, AB, Canada
incidence of AD. A nationwide consortium of 24 univer-
sity medical centers (the CERAD program) developed,
standardized, and evaluated methods for clinical, neuro-
Synonyms psychological, neuropathologic, and neuroimaging
assessments of AD. Data from the study population, in-
CERAD
cluding 1,094 patients with AD and 463 controls evalu-
ated annually between 1987 and 1996, are available on
CD-ROM for research purposes (CERAD, Box 3203,
Description Duke University Medical Center, Durham, NC 27710).
As the bulk of the CERAD data collection took place
The Consortium to Establish a Registry for Alzheimers
before the widespread use of cholinesterase inhibitor
Disease (CERAD) neuropsychological battery was
treatment for AD, these data provide a rich source of
designed to provide an efficient, standardized method to
information as to the natural history of AD. A special
evaluate cognitive functioning in individuals with Alzhei-
issue of Neurology (49, suppl. 3) published in 1997 com-
mers disease (AD). Originally employed in a large-scale
memorated the tenth anniversary of the CERAD program
longitudinal research program, tests were selected to pro-
and reviewed study design, implementation, and key find-
vide coverage of the primary cognitive deficits associated
ings from wide-ranging investigations of AD.
with AD (e.g., memory, language, praxis, and intellectual
status) and to have a range of difficulty appropriate for
use through much of the course of the disease (Morris
et al., 1989). Individual tests within the battery include Psychometric Data
well-known, commonly used measures such as the Mini-
Mental State Exam (MMSE; Folstein, Folstein, & Psychometric data for the CERAD neuropsychological
McHugh, 1975), a 15-item modified Boston Naming battery reported for participants enrolled over the first
Test (Kaplan, Goodglass, & Weintraub, 1978), and an 19 months of the study indicated good interrater reliabil-
Animal Naming verbal fluency test (Isaacs & Kennie, ity across all tests, with intraclass correlation coefficients
1973). Also included are a ten-item word list memory ranging from 0.92 to 1.0 (Morris et al., 1989). One
test, with free recall (immediate and delayed) and recog- month testretest data showed variable reliability
Consortium to Establish a Registry on Alzheimers Disease C 691
(rs from 0.36 to 0.90), with lower correlation coefficients impact test performance. Normative data for patients
for measures whose variance may have been constrained with AD have also been reported, in addition to annual
by ceiling effects (Morris et al., 1989). The efficacy of rates of change on CERAD test scores over a 4-year period
CERAD battery measures (individually and in combina- (Morris et al., 1993).
tion) for detecting AD and for staging dementia severity The CERAD battery has been extensively translated. C
has been evaluated using discriminant function models Normative data for the CERAD are available for a wide
(Welsh, Butters, Hughes, Mohs, & Heyman, 1991; Welsh, range of racial/cultural/demographic groups (Bertolucci
Butters, Hughes, Mohs, & Heyman, 1992). The word list et al., 2001; Fillenbaum, Heyman, Huber, Ganguli, &
delayed recall measure was shown to most effectively Unverzagt, 2001; Fillenbaum et al., 2005; Ganguli et al.,
differentiate those with AD from controls (correctly 1991; Ganguli et al., 1996; Guruje et al., 1995; Whyte et al.,
identifying 94% of controls and 86% of mild AD 2005). Beeri et al. (2006) provide normative data for
patients), although the ability of this measure to distin- individuals over the age of 85.
guish dementia severity groups was limited due to floor Methods for deriving a single summary score for the
effects. For those with impaired scores on delayed recall, CERAD battery (excluding the MMSE) and normative
performance on the naming measure was shown to mod- data for this summary score for unimpaired, mild cogni-
estly increase the discrimination of those with mild from tive impairment (MCI), and AD groups have been pub-
moderate AD (improvement in overall accuracy from lished (Chandler et al., 2005). This total score was
65 to 71%). Verbal Fluency and Constructional Praxis reportedly more consistent at differentiating these three
performances were shown to increase discrimination of groups from one another compared to the MMSE total
those with moderate from severe AD (improvement in score, which showed limited ability to distinguish patients
overall accuracy from 66 to 81%). Within a cognitively with MCI from unimpaired individuals, and the word list
impaired sample, the CERAD battery has also been found delayed recall score, which showed limited ability to dis-
to distinguish those with AD from those with schizophrenia tinguish MCI from AD.
(Davidson et al., 1996). Clinicalpathological correlations Some authors have described an overreliance of the
have shown association between CERAD neuropsycho- CERAD battery on using verbal modalities of testing, at
logical performance and AD pathology on autopsy the expense of other relevant information. To address this
(Hulette et al., 1998). Data from the CERAD program issue, a visual memory measure based on recall of the
have been used to identify predictors of time to institu- Constructional Praxis test items has been proposed. Ad-
tionalization in patients with AD, but information from ministration instructions and psychometric properties of
the CERAD neuropsychological battery (with the excep- a delayed recall and recognition condition of this measure
tion of the MMSE) was not considered in this study have been reported (Spangenberg, Henderson, & Wagner,
(Heyman, Peterson, Fillenbaum, & Pieper, 1997). 1997).
In sum, the brevity of the CERAD neuropsychological
battery, combined with the vast associated research base,
make the CERAD a useful tool for assessment of cognitive
Clinical Uses function in cases of suspected dementia where factors
such as fatigue, motivation, or incapacity prevent a more
The chief objective of the CERAD neuropsychology task extensive neuropsychological examination of cognitive
force was to develop a brief, reliable battery of neuropsy- capacities (Welsh et al., 1994). Assessment in specialized
chological tests that would be widely used by researchers memory clinics, however, would typically employ a more
and clinicians to assess the cognitive status of patients extensive neuropsychological battery.
with AD at entry and during long-term annual follow-
up (Welsh-Bohmer & Mohs, 1997). The CERAD neuro-
psychological battery has been widely used in research Cross References
studies, including those completed at many of the NIH-
sponsored Alzheimers Disease Centers. Normative data Alzheimers Disease
based on 413 CERAD control subjects aged 5089 have Blessed Dementia Screen
been published to facilitate clinical application (Welsh Clinical Dementia Rating Scale (CDR)
et al., 1994). These data are stratified by age, education, Mattis Dementia Rating Scale (DRS)
and gender, as these factors were shown to differentially Mini-Mental State Exam (MMSE)
692 C Constraint Induced Therapy
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dinal assessment of probable Alzheimers disease. Neurology, 43(12),
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Morris, J. C., Heyman, A., Mohs, R. C., Hughes, J. P., van Belle, G.,
et al. (2006). Age, gender, and education norms on the CERAD
Fillenbaum, G., et al. (1989). The Consortium to Establish a Registry
neuropsychological battery in the oldest old. Neurology, 67(6),
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Bertolucci, P. H., Okamoto, I. H., Brucki, S. M., Siviero, M. O., Toniolo
Rosen, W. G., Mohs, R. C., & Davis, K. L. (1984). A new rating scale for
Neto, J., & Ramos, L. R. (2001). Applicability of the CERAD Neuro-
Alzheimers disease. The American Journal of Psychiatry, 141(11),
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Spangenberg, K. B., Henderson, S., & Wagner, M. T. (1997). Validity of a
Chandler, M. J., Lacritz, L. H., Hynan, L. S., Barnard, H. D., Allen, G.,
recall and recognition condition to assess visual memory in the
Deschner, M., et al. (2005). A total score for the CERAD Neuropsy-
CERAD battery. Applied Neuropsychology, 4(3), 154159.
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Welsh, K. A., Butters, N., Hughes, J., Mohs, R., & Heyman, A. (1991).
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Detection of abnormal memory decline in mild cases of Alzheimers
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F. W. (2001). Performance of elderly African American and
(1992). Detection and staging of dementia in Alzheimers disease.
White community residents on the CERAD Neuropsychological
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Borenstein, A. R., Foley, D. J., et al. (2005). Performance on the
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R. N., & Weiner, M. F. (2005). Performance of elderly Native Amer-
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R., et al. (1996). Cognitive test performance in a community-based
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Constraint Induced Therapy
et al. (1991). Effects of age, gender, and education on cognitive
tests in a rural elderly community sample: Norms from the Mono- M ARIANNE H RABOK , K IMBERLY A. K ERNS
ngahela Valley Independent Elders Survey. Neuroepidemiology, 10(1), University of Victoria
4252. Victoria, BC, Canada
Guruje, O., Unverzargt, F. W., Osuntokun, B. O., Hendrie, H. C.,
Baiyewu, O., Ogunniyi, A., et al. (1995). The CERAD Neuropsycho-
logical Test Battery: Norms from a Yoruba-speaking Nigerian sam-
ple. West African Journal Of Medicine, 14(1), 2933. Definition
Heyman, A., Peterson, B., Fillenbaum, G., & Pieper, C. (1997). Predictors
of time to institutionalization of patients with Alzheimers disease: Constraint induced therapy (CIT) typically refers to a
the CERAD experience, part XVII. Neurology, 48(5), 13041309.
therapeutic intervention involving restraint of a non-
Hulette, C. M., Welsh-Bohmer, K. A., Murray, M. G., Saunders, A. M.,
Mash, D. C., & McIntyre, L. M. (1998). Neuropathological and
affected upper extremity combined with intensive practice
neuropsychological changes in normal aging: Evidence for pre- with the affected upper extremity (e.g., Alberts, Butler, &
clinical Alzheimer disease in cognitively normal individuals. Journal Wolf, 2004; Charles & Gordon, 2005; Taub & Uswatte,
of Neuropathology and Experimental Neurology, 57(12), 11681174. 2005, 2006).
Isaacs, B., & Kennie, A. T. (1973). The Set test as an aid to the detection of
dementia in old people. The British Journal of Psychiatry: The Journal
of Mental Science, 123(575), 467470.
Kaplan, E., Goodglass, H., & Weintraub, S. (1978). The Boston Naming
Historical Background
Test. Boston, MA: Veterans Administration Medical Center.
Morris, J. C., Edland, S., Clark, C., Galasko, D., Koss, E., Mohs, R., et al. CIT has its origins in primate unilateral deafferentation
(1993). The Consortium to Establish a Registry for Alzheimers studies, where it was observed that animals avoided use
Constraint Induced Therapy C 693
of their deafferented limb unless the intact limb was to rehabilitation strategies that focus on developing com-
restrained (e.g., Taub & Uswatte, 2005, 2006). Concepts pensatory techniques, CIT aims to optimize and develop
from CIT were then applied to humans in single case residual functions (Lillie & Mateer, 2006). Although CIT
studies, case series, and small group studies, primarily has been shown to result in significant gains in motor
with people who had sustained hemiplegia following stroke activity, it should not be expected to restore or normalize C
(Taub et al., 1993). The Extremity Constraint Induced movements to pre-injury levels (Taub & Uswatte, 2005).
Treatment (EXCITE) study is a randomized clinical trial
involving over 200 individuals enrolled at 39 months
post-stroke, using uniform CIT methods and measure- Treatment Participants
ment at six testing sites in the USA (Nichols-Larsen,
Clark, Zeringue, Greenspan, & Blanton, 2005; Winstein CIT has been applied in a variety of populations, includ-
et al., 2003; Wolf et al., 2006). ing upper limb hemiparesis following stroke, traumatic
brain injury, and children with hemiplegia (Charles &
Gordon, 2005; Lillie & Mateer, 2006; Shaw et al., 2005).
Rationale or Underlying Theory Research has suggested significant improvement in a
number of populations, including those with chronic
CITreflects the integration of a wide variety of research fields,
TBI (identified as > 1 year post-TBI; Shaw et al., 2005)
including neuroplasticity, motor learning, and behavioral
and both subacute and chronic stroke (identified as <
therapy (Sterr, Szameitat, Shen, & Freivogel, 2006). Two
1 year post-stroke and > 1 year post-stroke, respectively;
mechanisms are typically proposed as the basis for
Page, Levine, & Leonard, 2005).
increased use of affected extremities in CIT: overcoming
Adherence to treatment and level of residual motor
learned nonuse, and induction of use-dependant cortical
function prior to CIT is positively related to treatment
organization (Morris & Taub, 2001).
gains (Shaw et al., 2005; Taub & Uswatte, 2005). Cognitive
Learned nonuse is said to occur following brain injury
impairment is linked to less improvement (Morris et al.,
because unsuccessful motor attempts with the affected
2006). An inherent assumption in CIT is that a residual
extremity leads to punishment (e.g., pain, incoordination,
level of motor function remains in the affected extremity,
failure to meet the goal of the movement). Compensatory
which enables a degree of positive reinforcement for its
behavior also occurs when use of the non-affected extrem-
use (Taub & Uswatte, 2006). A minimal level of residual
ity is positively reinforced.
motor function is often specified as an inclusion criterion
CIT aims to counter-condition learned nonuse. For
in research studies of CIT, as is a lack of general cognitive
example, in animals, the functional limb is restrained and
impairment (e.g., Nichols-Larsen et al., 2005).
the animals complete forced, repetitive movements with
Due to the higher degree of neural plasticity early in
the affected limb. This results in an alteration in the
development, it has been suggested that children may
contingencies of reinforcement, such that compensatory
benefit from CIT to an even greater extent than adults
use of the non-affected limb is no longer reinforced, and
(e.g., Taub & Crago, 1995 as cited by Charles & Gordon,
use of the affected limb is reinforced.
2005). Specific modifications have been made to make
Use-dependant cortical reorganization is a second
CIT more amenable to childhood populations, including
primary mechanism thought to underlie CIT. Neuroim-
conducting CIT in the home environment and practice
aging and transcranial magnetic stimulation studies of the
sessions in the context of play (DeLuca, Echols, Law, &
brain prior to and following CIT have suggested cortical
Ramey, 2005).
reorganization around the infarct site, and recruitment of
a large portion of neurons adjacent to those originally
involved in the control of the limb (Dong, Dobkin, Cen,
Wu, & Winstein, 2006; Morris & Taub, 2001; Park, Butler, Treatment Procedures
Cavalheiro, Alberts, & Wolf, 2004).
CIT is generally administered as a package treatment,
incorporating restraint of the unaffected limb, and
Goals and Objectives structured practice (e.g., shaping and repetitive practice)
with the affected limb. A transfer package involving
A major goal of CIT is to increase the use of the affected limb behavioral techniques used to promote transfer of gains
in real world environments (Sterr et al., 2006). In contrast from the laboratory to daily life is emphasized in CIT
694 C Constraint Induced Therapy
(Taub & Uswatte, 2005). Structured practice with the measured in a laboratory setting, and it is therefore
affected arm involves everyday functional tasks (Alberts recommended that these dimensions be assessed separately
et al., 2004), such as eating lunch, throwing a ball, etc. (Uswatte & Taub, 2005).
(Glover, Mateer, Yoell, & Speed, 2002). The Wolf Motor Function Test is a frequently used
Conventional CIT treatment aims for restraint of the measure of laboratory motor performance (e.g., Uswatte
unaffected limb for 90% of waking hours, and structured & Taub, 2005). Functional outcome is most often studied
practice 68 h per day (Kaplon, Prettyma, Kushi, & through use of Motor Activity Log (MAL), a semistruc-
Winstein, 2007). The treatment period for restraint and tured interview of extremity use in real-life settings (e.g.,
structured practice is 5 days per week, for 23 consecutive Winstein et al., 2003).
weeks with a 1:1 therapistpatient ratio.
The intensive nature of CIT has prompted concerns
regarding its applicability to clinical settings, due to insti-
Qualifications of Treatment Providers
tutional and clinical factors. For example, facilities may
lack the space, therapist time, and associated financial
Physiotherapists most frequently provide services. Occu-
resources to administer CIT (Levine & Page, 2004).
pational therapists have also been identified as providing
Concerns have also been raised regarding attrition rates,
services (e.g., Brogardh, 2006). Specialized training in
compliance, and health care coverage (Levine & Page,
CIT is recommended (DeLuca et al., 2005; Winstein
2004; Page & Levine, 2003).
et al., 2003).
Because these factors can impede service delivery and
the aims of CIT, modifications of treatment protocols
have been introduced. For example, group delivered CIT
(Brogardh, 2006), automatized delivery of CIT with less Cross References
therapist involvement (AutoCITE; Lum, Uswatte, Taub,
Hardin, & Mark, 2006; Taub, Lum, Hardin, Mark, & Brain Plasticity
Uswatte, 2005), and fewer hours of restraint and shorter Cognitive Rehabilitation
practice sessions over longer periods (Levine & Page, Head Injury
2004; Page & Levine, 2003; Page, Sisto, Johnston, & Hemiparesis
Levine, 2002) have all resulted in significant treatment Hemiplegia
gains, often comparable to the gains of conventional Physical Therapy
CIT (e.g., Levine & Page, 2004; Lum et al., 2006). Current Rehabilitation Psychology
research suggests that particularly important features are Traumatic Brain Injury
prolonged rehearsal on specific tasks involving the affect-
ed limb and shaping in effort to improve everyday activity
(Pomeroy & Tallis, 2002). References and Readings
Alberts, J. L., Butler, A. J., & Wolf, S. L. (2004). The effects of constraint-
induced therapy on precision grip: A preliminary study. Neuroreh-
Efficacy Information abilitation and Neural Repair, 18, 250258.
Brogardh, C. (2006). Constraint-induced movement therapy in patients
CIT has been shown to significantly improve performance with stroke: A pilot study on effects of small group training and of
extended mitt use. Clinical Rehabilitation, 20, 218227.
on laboratory tests of motor ability and functional out-
Charles, J., & Gordon, A. M. (2005). A critical review of constraint-
come measures. The effect sizes for functional outcome induced movement therapy and forced use in children with hemiplegia.
measures tend to be very large in magnitude (Taub & Neural Plasticity, 12, 245261.
Uswatte, 2005). Effect sizes on laboratory tests tend to DeLuca, S. C., Echols, K., Law, C. R., & Ramey, S. L. (2005). Intensive
be more variable (as reviewed by Lillie & Mateer, 2006). pediatric constraint-induced therapy for children with cerebral
palsy: Randomized, controlled, crossover trial. Journal of Child
Neurology, 21, 931938.
Dong, Y., Dobkin, B. H., Cen, S. Y., Wu, A. D., & Winstein, C. J. (2006).
Motor cortex activation during treatment may predict therapeutic
Outcome Measurement gains in paretic hand function after stroke. Stroke, 37, 15521555.
Glover, J. E., Mateer, C. A., Yoell, C., & Speed, S. (2002). The effectiveness
There can be significant discrepancies between real of constraint induced movement therapy in two young children with
world spontaneous functional use and motor ability as hemiplegia. Pediatric Rehabilitation, 5, 125131.
Constructional Apraxia C 695
Kaplon, R. T., Prettyma, M. G., Kushi, C. L., & Winstein, C. J. (2007). Six constraint-induced movement therapy. Rehabilitation Psychology,
hours in the laboratory: A quantification of practice time during 50, 3442.
constraint-induced therapy (CIT). Clinical Rehabilitation, 21, Winstein, C. J., Miller, J. P., Blanton, S., Taub, E., Uswatte, G., Morris, D.,
950958. et al. (2003). Methods for a multisite randomized trial to investigate
Lillie, R., & Mateer, C. A. (2006). Constraint-based therapies as a pro- the effects of constraint-induced movement therapy in improving
posed model for cognitive rehabilitation. Journal of Head Trauma
Rehabilitation, 21, 119130.
upper extremity function among adults recovering from a
cerebrovascular stroke. Neurorehabilitation and Neural Repair, 17,
C
Levine, P., & Page, S. J. (2004). Modified constraint-induced therapy: 137152.
A promising restorative outpatient therapy. Topics in Stroke Rehabil- Wolf, S. L., Winstein, C. J., Miller, J. P., Taub, E., Uswatte, G., Morris, D.,
itation, 11, 110. et al. (2006). Effects of constraint-induced movement therapy on
Lum, P. S., Uswatte, G., Taub, E., Hardin, P., & Mark, V. W. (2006). upper extremity function 3 to 9 months after stroke: The EXCITE
A telerehabilitiation approach to delivery of constraint-induced randomized clinical trial. JAMA, 296, 20952103.
movement therapy. Journal of Rehabilitation Research and Develop-
ment, 43, 391400.
Morris, D. M., Shaw, S. E., Mark, V. W., Uswatte, G., Barman, J., &
Taub, E. (2006). The influence of neuropsychological characteristics
on the use of CI therapy in persons with traumatic brain injury.
Neurorehabilitation, 21, 131137. Constructional Apraxia
Morris, D. M., & Taub, E. (2001). Constraint-induced therapy approach
to restoring function after neurological injury. Topics in Stroke A MY K. B YERLEY, A NDREW S. DAVIS
Rehabilitation, 8, 1630. Ball State University
Nichols-Larsen, D. S., Clark, P. C., Zeringue, A., Greenspan, A., &
Muncie, Indiana, USA
Blanton, S. (2005). Factors influencing stroke survivors quality of
life during subacute recovery. Stroke, 36, 14801484.
Page, S., & Levine, P. (2003). Forced use after TBI: Promoting plasticity
and function through practice. Brain Injury, 17, 675684. Synonyms
Page, S. J., Levine, P., & Leonard, A. C. (2005). Modified constraint-
induced therapy in acute stroke: A randomized controlled pilot
Constructional dyspraxia
study. Neurorehabilitation and Neural Repair, 19, 27.
Page, S. J., Sisto, S., Johnston, M. V., & Levine, P. (2002). Modified
constraint-induced therapy after subacute stroke: A preliminary
study. Neurorehabiliation and Neural Repair, 16, 290295. Short Description or Definition
Park, S. W., Butler, A. J., Cavalheiro, V., Alberts, J. L., & Wolf, S. I. (2004).
Changes in serial optical topography and TMS during task perfor-
mance after constraint-induced movement therapy in stroke: A case
Constructional apraxia is an inability to reproduce pat-
study. American Society of Neurorehabiliation, 18, 95105. terns or join component parts into a whole. This condition
Pomeroy, V., & Tallis, R. (2002). Neurological rehabilitation: A science is assessed through observation of a patient completing
struggling to come of age. Physiotherapy Research International, activities such as drawing, copying, or building three-
7, 7689.
dimensional objects (Lezak, Howieson, & Loring, 2004).
Shaw, S. E., Morris, D. M., Uswatte, G., McKay, S., Meythaler, J. M., &
Taub, E. (2005). Constraint-induced movement therapy for recovery
Impairments in processing spatial forms observed in con-
of upper-limb function following traumatic brain injury. Journal of structional apraxia can occur in the absence of apraxia of
Rehabilitation Research and Development, 42, 769778. singular motor movements (Benton, 1969).
Sterr, A., Szameitat, A., Shen, S., & Freivogel, S. (2006). Application of
CIT concepts in the clinical environment: Hurdles, practicalities, and
clinical benefits. Cognitive Behavioral Neurology, 19, 4853.
Taub, E., Lum, P. S., Hardin, P., Mark, V. W., & Uswatte, G. (2005).
Categorization
AutoCITE: Automated delivery of CIT with reduced effort by
therapists. Stroke, 36, 13011304. Recent studies suggest qualitatively different types of con-
Taub, E., Miller, N. E., Novack, T. A., Cook, E. W. III, Fleming, W. C., structional apraxia determined by the location of brain
Nepomuceno, C. S., et al. (1993). Technique to improve chronic
insult. In general, patients with right hemisphere im-
motor deficit after stroke. Archives of Physical Medicine and Rehabil-
itation, 74, 347354.
pairment make more coordinate type errors (e.g., distance
Taub, E., & Uswatte, G. (2005). Use of CI therapy for improving motor and angular distortions), whereas those with left hemi-
ability after chronic CNS damage: A development prefigured by Paul spheric impairment tend to commit categorical errors
Bach-Y-Rita. Journal of Integrative Neuroscience, 4, 465477. (e.g., position exchanges and pattern reversals) (Laeng,
Taub, E., & Uswatte, G. (2006). Constraint-induced movement therapy:
2006). Patients with right hemispheric impairment gener-
Answers and questions after two decades of research. Neurorehabil-
itation, 21, 9395.
ally experience difficulties with the overall gestalt of the
Uswatte, G., & Taub, E. (2005). Implications of the learned non-use constructional task. Their approach to the task may ap-
formulation for measuring rehabilitation outcomes: Lessons from pear to be more fragmented and disjointed, not coming
696 C Constructional Apraxia
together as a whole. Constructions created by patients treatment, while others may have symptoms spontane-
with right hemisphere impairment may make drawing ously diminish. For other patients, symptoms may be
that are sparse or may be significantly distorted with permanent. Further research for prognostic factors of
regard to perspective or proportion. Some patients with constructional apraxia is warranted.
right hemisphere injury may create overdetailed or repet-
itive drawings, while others may draw elaborate pictures
that are missing important components. Additionally, Neuropsychology and Psychology of
patients with right hemispheric lesions frequently dem- Constructional Apraxia
onstrate left-sided visual inattention and include more
details on construction tasks. Symptoms associated with constructional apraxia fre-
In contrast, those with left hemisphere impairment quently indicate deficits associated with right parietal
may be able to construct the overall concept and propor- dysfunction, or the non-dominant hemisphere and
tions accurately, and their constructions may also be global cognitive processing impairment. However, re-
symmetrical. However, they often tend to generate draw- cent research has shown involvement of both hemi-
ings with fewer details (McFie & Zangwill, 1960). Patients spheres, namely the parietal lobes, in constructional
with left hemisphere impairment may perform construc- apraxia. Furthermore, neuroimaging research has
tion tasks better when given a model rather than working implicated the ventral premotor area, posterior part
on command. They tend to focus on overall shape rather of the inferior temporal sulcus, and occipital cortex
than specific details. Overall, construction deficits tend to in constructional deficits (Makuuchi, Kaminaga, &
be more common when the lesion is located in the poste- Sugishita, 2003; Nielson, Cummings, & Cotman, 1996).
rior portion of the brain rather than the anterior areas. Constructional apraxia should not be conceptualized as a
Furthermore, while patients with cortical lesions make the unitary syndrome caused by discrete lesions (Guerin, Ska,
same types of constructional errors as patients with sub- & Belleville, 1999), but as resulting from impairment in
cortical lesions, those with subcortical impairment appear lateralized perceptual processing of spatial abilities
to have more significant impairment. (Laeng, 2006). Constructional apraxia manifests more
frequently in patients with lesions in the non-dominant
hemisphere (De Renzi, 1997). Consequently, construc-
Epidemiology tional apraxia often co-occurs with deficits in visual-spa-
tial perception (Benton, 1982), although both conditions
A review of literature revealed no known reports of preva- may exist independently. Therefore, the presentation of
lence of constructional apraxia. In fact, constructional constructional apraxia differs among patients; some
apraxia is generally included as a subcategory of apraxia, struggle with copying figures, whereas others demonstrate
which is listed as a rare disease by the office of rare difficulty constructing designs with blocks (Lezak et al.,
diseases (ORD) of the National Institutes of Health 2004).
(NIH). Therefore, apraxia is known to affect less than
200,000 people in the population of the USA. Thus, as a
subcategory of apraxia, constructional apraxia affects even Evaluation
fewer individuals. Common causes of constructional aprax-
ia include dementia (e.g., Alzheimers Disease) and stroke, The utility of constructional apraxia as an estimate of
which are two of the most frequent neurological diseases. global cognitive functioning has a well-established his-
tory in neurology, neuropsychology, and rehabilitative
settings. Tasks commonly utilized to assess construc-
Natural History, Prognostic Factors, and tional apraxia include copying, drawing, or construct-
Outcomes ing simple figures, such as clocks or crosses. These
efficient, brief, and easy-to-administer construction
Constructional apraxia is not in itself a disease but a tasks are attractive to clinicians for use with patients
symptom of another neurological illness. Therefore, prog- with impaired test-taking skills. The tasks used to
nosis of the condition is related to the prognosis of the assess constructional apraxia have proven to be re-
underlying neurological condition. Prognosis for indivi- markably sensitive to global neurological impairment
duals with constructional apraxia varies and has not been because of the involvement of various cognitive pro-
well studied. Some patients may improve via continued cessing domains necessary for completing construction
Content-Referenced Testing C 697
tasks. Construction tasks can require complex cogni- Benton, A. L. (2009). Constructional apraxia: Some unanswered ques-
tions. In A. L. Benton (Ed.), Brain & behavior: Research in clinical
tive processes, including visual-spatial and visuoper-
neuropsychology (pp. 129141). Chicago: Aldine.
ceptual abilities, receptive language skills, numerical De Renzi, E. (1997). Visuospatial and construction disorders. In T. E.
knowledge, graphomotor skills, and intact executive Feinberg, & M. J. Farah (Eds.), Behavioral neurology and neuropsy-
functioning (Freedman et al., 1994; Lezak et al., 2004; chology (pp. 297304). New York: McGraw-Hill.
C
Shulman, 2000). The sensitivity of construction tasks to Fischer, J. S., & Loring, D. W. (2004). Construction. In M. D. Lezak,
D. B. Howieson, & D. W. Loring (Eds.), Neuropsychological assess-
global cognitive impairment renders these tasks advanta-
ment (4th ed., pp. 531568). New York: Oxford University Press.
geous in the assessment of patients with known or Freedman, M., Leach, L., Kaplan, E., Winocur, G., Shulman, K., & Delis,
suspected neurological impairment, including those D. C. (1994). Clock drawing: A neuropsychological analysis. New York:
with Alzheimers Disease, Huntingtons Disease, Parkin- Oxford University Press.
sons Disease, aphasia, seizures, CVAs, and TBIs. As an Guerin, F., Ska, B., & Belleville, S. (1999). Cognitive processing of drawing
abilities. Brain and Cognition, 40:464478.
example, construction tasks have been shown to be useful
Laeng, B. (2006). Constructional apraxia after left or right unilateral
as screening tools and markers for disease progression stroke. Neuropsychologia, 44:15951606.
with Alzheimers patients. Furthermore, the presence of Lezak, M. D., Howieson, D. B., & Loring, D. W. (2004). Neuropsychologi-
constructional apraxia early in the course of Alzheimers cal Assessment (4th ed.). New York: Oxford University Press.
Disease has been shown to be a predictor of accelerated Makuuchi, M., Kaminaga, T., & Sugishita, M. (2003). Both parietal lobes
are involved in drawing: A functional MRI study and implications
cognitive decline (Smith, Esiri, Barnetson, King, & Nagy,
for constructional apraxia. Brain Research, 16:338347.
2001). McFie, J., & Zangwill, O. L. (1960). Visual construction disabilities
associated with lesions of the left cerebral hemisphere. Brain,
83:243260.
Treatment Nielson, K., Cummings, B., & Cotman, C. (1996). Constructional apraxia
in Alzheimers disease correlates with neuritic neuropathology in
occipital cortex. Brain Research, 741:284293.
While physical and occupational therapies may modestly Shulman, K. L. (2000). Clock-drawing: Is it the ideal cognitive screening
improve the functioning of an individual with construc- test? International Journal of Geriatric Psychiatry, 15:548561.
tional apraxia, no specific medical treatment has been Smith, M., Esiri, M., Barnetson, L., King, E., & Nagy, Z. (2001). Con-
found to be effective with significantly improving con- structional apraxia in Alzheimers disease: Association with occipital
lobe pathology and accelerated cognitive decline. Dementia and
structional deficits. Furthermore, medications that target
Geriatric Cognitive Disorders, 12:281288.
the slowing of dementia do not appear to help with
constructional apraxia. The most beneficial approach for
treatment of individuals with constructional apraxia
involves ensuring their environments are safe. For exam-
ple, it is important to arrange furniture in the home in a
way that the patient can navigate safely. Constructional Dyspraxia
Constructional Apraxia
Cross References
Apraxia
Dementia
Stroke
Visual-Motor Function Content Validity
Test Validity
References and Readings
Contingency Table
C HRISTIAN S CHUTTE , B RADLEY A XELROD In this example, hit rate would be calculated by adding
John D. Dingell VA Medical Center the number of true negatives and false negatives and then
Detroit, MI, USA dividing them by the number of total decisions ((TP +
FN)/total decisions). This allows for a relatively simple
way to assess the relationships between categorical
Synonyms variables.
A concrete example would be a group of 100 patients,
2 2 Table 40 of whom have a true diagnosis of traumatic brain injury
(TBI) who are administered a brief test to diagnose the
presence of the disorder. By comparing the obtained scores
to a cutting score, 55 patients are tagged as having the
Definition disorder and 45 patients as not having the disorder. Using
this information, a contingency table to find hit rate can
A contingency table is generally a representation of cate- quickly be defined, sensitivity, and specificity, as well as
gorical data in a tabular format, such as a 2 2 table, positive and negative predictive power.
though the table can have three or more variables. One can then calculate hit rate ((TP + FN)/total
decisions) or (35 + 5)/100 = .4, sensitivity (TP/(TP +
FN)) or 35/(35 + 5) = .875, specificity (TN/(TN + FP))
Current Knowledge or 45/(45 + 15) = .75, positive predictive power (TP/(TP +
FP)) or 35/(35 + 15) = .70, and negative predictive power
There are row variables on the horizontal axis and column (TN/(TN + FN)) or 45/(45 + 5) = .90. In verbal form, hit
variables on the vertical axis. It represents mutually exclu- rate is a proportion of the number of correctly identified
sive variables. Good examples of contingency tables are TBI patients and incorrectly ruled out TBI patients divided
hit rate, sensitivity, and specificity, as well as positive and by the total number of patients (40% in this case) or
negative predictive power. In the case of hit rates to assess sensitivity is computed by the percentage of people who
the number of correct classification decisions that result actually have the disorder who were appropriately detected
from the use of a particular test or measure, one would by the test (87.5% in this case).
enter the number of true positives, true negatives, false More complex contingency tables can be used, such as
positives, and false negatives into a contingency table like 3 2, 3 3, or more, though relationships between
the one below. variables are less clear owing to difficulties with interac-
tion effects. More complex contingency tables are often
analyzed with more complex statistical procedures, such
as log-linear analysis.
Synonyms
Psychometric Data
CPT
In its original form, the CPT provided measures of accu-
racy and response bias over the course of a fixed time
Description period using signal detection methodology. Although the
tests may vary in terms of length and type of stimulus
An attention paradigm that has evolved into a class used, the underlying paradigm is the same across versions
of neuropsychological tests used to assess sustained of the test. Patients are presented with series of letters
attention. There is not a single continuous performance (typically) or other stimuli on a screen, and are told to
test (CPT) test, as a number of commercially available and push a response key only when they see the target
research CPT tasks exist and have been published in the stimulus. They are instructed not to respond when they
neuropsychological literature. The common characteristic see any other stimuli. The letter x has often been used
of all CPT tests is that they involve sequential presentation as the target in CPT paradigms, with the task to respond
of stimuli, usually letters or numbers, over an extended to this letter while ignoring other letters that flash before
period of time. The task demand is to attend and respond them. Several common variations of the standard CPT
to particular target stimuli, while ignoring other stimuli paradigm exist, in particular, a conditional task, in which
that serve as nontarget distractors. the patient must only respond to the target letter when
another stimulus occurs immediately before it (e.g., A-X),
which increases the difficulty of the task.
Historical Background In recent years, a variety of tests based on computer-
ized CPT paradigms have been developed, with varying
Early efforts by psychologists to assess attention in the degree of sensitivity and specificity to clinical disorders of
context of intellectual or other cognitive testing typically attention. At the present time, the most widely used
relied on tests such as digit span, which provided a commercially available CPT tests are versions by Conners,
useful measure of attentional focus and span, but did not Epstein, Angold, and Klaric (2003) and the Test of
address other important elements of attention, such as the Variables of Attention (TOVA), though a variety of others
patients ability to selectively attend to information or to exist, such as VIGIL (1990) and the d2 Test of Attention
sustain attention (Cohen, 1993). Sustained attention was (Brickenkamp, 1992). Typically, a CPT is included as part
particularly difficult to assess using traditional paper-and- of a more comprehensive battery of tests of attention and
pencil tests, as it required the measurement of signal detec- executive functioning.
tion performance over extended periods of time. Psycholo- Given that the CPT paradigm is based on signal detec-
gists typically relied on behavioral observation or analysis of tion theory and method, there are certain indices common
patterns of inconsistency in test performance over time to to all versions of the test; correct responses, errors of
derive evidence of sustained attention problems. The devel- omission (misses), errors of commission (false positives),
opment of the tachistoscope for rapid presentation of visual and response time (RT). Correct responses are based on the
stimuli with controlled timing provided a means of circum- sum of the number of times the patient correctly responds
venting this problem. Mirsky et al. (1956) described the to the target and correctly avoids responding to distractors.
continuous performance paradigm and provided research Omission errors (misses) are errors involving a failure to
data supporting its sensitivity in detecting brain damage. respond to the target, while commission errors (false posi-
The advent and widespread availability of computer tech- tives) are errors involving a response to nontargets. Errors
nology in the decades that followed led to more widespread of commission reflect a failure to inhibit responding. High
experimentation with this paradigm and it eventually being omission rates indicate that either the patient is not
700 C Continuous Performance Tests
focusing adequately on the stimuli or that their processing of consistency across versions of the tests, particularly
speed is slow and they are unable to respond rapidly with respect to basic parameters such as the duration of
enough. CPT tests usually also provide a measure of the interstimulus interval (ISI), the total duration of the
mean response time (RT), which reflects the processing task, and the task demand. For example, a task requiring
speed of the patient during the task. Higher rates of correct detection of a single letter is much easier than a condi-
detections indicate better attention performance. tional paradigm requiring detection of a letter sequence
From these primary measures, signal detection indices (A-X). Furthermore, the duration of a typical CPT test
are usually derived based on a comparison of error types. can range from several minutes to over 20 min. Versions
Based on the total number of errors of commission with a long ISI tend to be relatively easy to perform, but
and omission on the CPT, a discrimination index (d0 ) is are tedious, with behavioral challenge arising from the
calculated which provides a measure of accuracy based on monotony of the task, which over a long test period is
standardized scores (z-scores) from normative samples. A really a test of vigilance. In contrast, versions that have a
measure of response bias (b) is also usually derived based short ISI with conditional task demands or other char-
on the difference in standard scores for each type of error. acteristics that increase difficulty tend to be more sensi-
Response bias indices provide a better way of interpreting tive to sustained attention in the context of greater
tendencies to make one type of error or the other because information processing demand and requirement for
they account for the total number of errors of each type. focused attention. Adaptive-rate continuous perfor-
Receiver operator characteristics (ROC) metrics can also mance methods provide a means of circumventing this
be derived, which provide a way of interpreting the issue. For example, the adaptive-rate continuous perfor-
tendency to make errors of each type as a function of mance test (ARCPT; Cohen, 1993) adjusts its ISI over the
the signal detection parameters of the task, such as course of the test based on accuracy of response to
percentage of targets to nontargets. General equations compensate for the slow speed of processing. The final
for d0 and b are shown below. ISI that is maintained by the end of the test provides a
strong measure of capacity limitations related to proces-
d0 zmisses zfalse positives sing speed deficits. Also the ARCPT provides separate
vigilance decrement and inconsistency indices to enable
b zmisses zfalse positives=z total errors assessment of the temporal dynamics of performance
over 10 blocks of time over the duration of the test.
In the past, many versions of the CPT provided only these The ARCPT measures attentional performance on a
basic indices, which limited their usefulness in rapid and challenging task which is less subject to bore-
characterizing problems with sustained attention. While dom. Therefore, the ARCPT provides enables the assess-
d0 and b provide excellent measures of detection accuracy, ment of sustained attention during a cognitively
response deposition, and overall attention performance, demanding task compared to standard CPT paradigms.
they do not directly provide measures of performance Also, because the ISI adjusts to shorter durations when a
over time. Recent versions of the Conners and other CPT patient is performing well, the test can typically be com-
tests tend to now include a measure of temporal variability pleted in about 10 min. The ARCPT also provides several
in performance. While there are a variety of ways that the metrics that enable assessment of temporal inconsisten-
temporal nature of performance can be determined, two cies in performance.
general types of measures exist: (1) performance decrement Despite the fact that CPT tests vary in a variety of
and (2) performance inconsistency. Performance decre- ways, many of the commonly used measures (e.g., Con-
ment provides a measure of the change in performance ners CPT) report good testretest reliability in healthy
between the beginning and end of the test. In theory, if a adults. CPT performance tends to correlate well with
person is failing to sustain attention, their performance performance on other information processing-based
should worsen the longer they stay on task. However, measures and performance on speeded tests of attention
often this measure is not impaired except when there is a and executive function, such as the Stroop and trail-
severe problem with sustained attention and fatigue after making test. Validation studies conducted with the
several minutes of effort. Performance inconsistency ARCPT indicate strong reliability (r = .95) for accuracy
indices provide an alternative temporal measure based on of performance (d0 ) across samples and strong validity as
variance across time, as opposed to linear decrement. indicated by the sensitivity of CPT indices to measures of
A primary problem in interpreting the meaning and structural and functional brain and systemic physiological
clinical significance of CPT findings stems from the lack disturbances as discussed in greater detail below.
Contraindication C 701
Definition
Cross References
A contraindication is a circumstance, condition, symptom,
Attention Deficit Disorder (ADD) or factor that increases the risk associated with a medical
Signal Detection procedure, drug, or treatment. A contraindication refers to
702 C Contrecoup Injury
any intervention considered inappropriate or inadvisable results from accelerationdeceleration events during
based upon unique factors of the situation such as potential which the force impacting the head causes the brain to
harmful interactions between drugs or medical conditions slam into the skull on the opposite side of the blow. Motor
that renders an individual vulnerable if implemented. vehicle accidents, falls, sports injuries, and physical
A contraindication may be absolute or relative. Absolute assaults with blunt objects frequently result in contrecoup
contraindications are those which are inadvisable without injuries. Skull characteristics make the most probable
exception or qualification. They are either permanently sites of injury in the frontal and temporal lobes, as tips
recommended against, or temporarily until the disqualifying of the skull can more easily be forced into the under-
condition is remediated. The use of the atypical antipsychot- lying brain tissue in the frontal and temporal lobes.
ic medication, clozapine carries a risk of agranulocytosis, a Neuropsychological evaluation can help to identify cog-
sever low white blood cell disorder condition. Clozopine nitive impairments arising from both the primary and
would be an absolute contraindication in an individual secondary sites of injury.
with a history of bone marrow suppression
Relative contraindications refer to circumstances in
which procedures or treatments are considered in com- Cross References
parative terms. They are contingent upon a risk/benefit
analysis of the relevant factors. The proportionate value of Acceleration Injury
an intervention is compared with its potential for negative Biomechanics of Injury
consequences. An example of a relative contraindication Cortical Contusion
would be the use of an anti-convulsant/mood stabilizing Traumatic Brain Injury
medication in the ongoing treatment of a pregnant
woman with severe bipolar mania and suicidality, but
whose has been asymptomatic only while on lithium. References and Readings
Cross References Graham, D. I., Saatman, K. E., Marklund, N., Copnte, V., Morales, D.,
Royo, N., & McIntosh, T. K. (2006). The neuropathology of trauma.
In R. W. Evans (Eds.), Neurology and trauma (2nd ed., pp. 4594).
Extrapyramidal Side Effects New York: Oxford University Press.
Iatrogenic
Signs
Synonyms
Contrecoup Injury
Central executive; Cognitive control; Supervisory atten-
B ETH R USH tional system
Mayo Clinic
Jacksonville, FL, USA
Definition
specific administration instructions). Several tests of pho- more difficult, while results for the F-A-S form appeared
nemic fluency exist, some of which are part of larger test more variable. In addition, COWAT scores have been
batteries (e.g., the MAE or the Neurosensory Center Com- correlated with neuropsychological measures such as
prehensive Examination for Aphasia; Spreen & Benton, reading tests and IQ tests.
1977) and others that can be administered independently
(e.g., the F-A-S Test).
Verbal fluency is a cognitive function that facilitates Clinical Uses
information retrieval from memory. Successful retrieval
requires executive control over cognitive process such as Scoring for the COWAT and other verbal fluency tests is
selective attention, mental set shifting, internal response straightforward. The examiner writes each word as it is
generation, and self-monitoring. Tests of verbal fluency produced by the individual. The transcript is reviewed
evaluate an individuals ability to retrieve specific informa- and inadmissible words (i.e., repetitions, proper names,
tion within restricted search parameters (Lezak, Howieson, or slang) are eliminated. The test score is the total number
Loring, Hannay, & Fischer, 2004). The two most common of different words produced for all three letters (see
parameters are semantic fluency and phonemic fluency. Strauss et al., 2006 and Benton et al., 1994 for specific
administration instructions).
Supplementary scoring measures for the COWAT and
Historical Background other phonemic fluency tests provide additional informa-
tion in clinical diagnosis and treatment. Supplementary
Borkowski, Benton, and Spreen (1967) were early propo-
scorning measures are error analysis, and cluster and
nents of systematically examining word fluency in persons
switching analysis (see Table 1). In error analysis, the
with brain damage. They recognized that so-called word
examiner notes any observable pattern of production of
fluency tasks had been used in neuropsychological inves-
errors that suggests a loosening of executive control over
tigation of patients with brain damage and undertook two
cognitive processes that would result in errors. For exam-
studies of the task. The first established the relations
ple, a pattern of multiple repetitions of previous responses
between word fluency and various English letters; the
suggests perseveration and inefficient self-monitoring.
second examined word fluency data for persons with
Error patterns provide qualitative performance data and
brain damage and control patients. They presented nor-
may appear as common patterns such as repetition of a
mative data for the first study and comparative data for
word, or idiosyncratic patterns. Clustering and switching
the second, clearly supporting their hypotheses of the
analyses evaluate the depth of the searchable knowledge
utility of word fluency assessment.
base (clusters) and the cognitive flexibility within and
Since publication of these data, word fluency tasks,
across categories (switching) (Troyer, Moscovitcvh, &
and the COWAT in particular, have been investigated in
Winocur, 1977). An example of an efficient search strategy
detail. Spreen and Risser (2003) suggest that this assess-
would be identifying a cluster or subcategory within the
ment tool in its various forms has been one of the most
category (e.g., words that begin with cr in the COWAT
frequently and thoroughly investigated neuropsychologi-
task of naming words that begin with C) and producing as
cal assessment measures for unimpaired and neurologi-
many items in that category as possible and then switch-
cally impaired persons. A search of electronic databases
ing clusters (e.g., to words beginning with cl). Clusters
confirms this suggestion.
are scored by counting the number of clusters and calcu-
lating the mean cluster size; switches are scored by count-
Psychometric Data ing the number of transitions between clusters. Rules for
scoring cluster size and number of switches appear in
Psychometric data for the COWAT and other phonemic Troyer et al. (1997) and normative data for healthy adults
fluency tests, as well as other verbal fluency tasks (e.g., appear in Troyer (2000).
semantic fluency) are readily available. Norms have been Scores from the COWAT are useful in evaluation of
published for children and adults of varying ages, levels of persons with neurogenic communication disorders, such
education, ethnic diversity, and geographical diversity as aphasia following stroke, traumatic brain injury, and
(Loonstra, Tarlow, & Sellers, 2001; Strauss et al., 2006). dementia. Studies have included COWAT in the diagnos-
Some differences have been noted between test forms, tic batteries given to several patient populations and also
most notably, between the COWAT and F-A-S Test in treatment studies as measures of behavioral change.
(Barry, Bates, & Labouvie, 2008); the CFL form appeared The utility of the COWAT in identifying the nature
Controlled Oral Word Association Test C 705
Controlled Oral Word Association Test. Table 1 An example of F-A-S Test results and cluster and switch scoring for a person
with aphasia
Switches = 7
FAS-Test: A apple, aardvark, alpaca, Clusters = 5 (mean size = 1)
ammonia, arsenic apple
aardvark
Words = 5 alpaca
ammonia
arsenic
Switches = 4
FAS-Test: S substantial, sum, subtraction, Clusters = 5 (mean size = 1.8)
stuck, structure, symbol, sympathy, stroke, sixty substantial, sum, subtraction
stuck, structure
Words = 9 symbol, sympathy
stroke
sixty
Switches = 4
Total Words = 23 Clusters = 18
Mean size = 1.27
Switches = 15
and severity of performance deficits in clinical popula- Boston Diagnostic Aphasia Examination
tions has been supported; however, conflicting findings Circumlocution
have been reported. Typically, the total number of ac- Clustering
ceptable responses is the reported test result; however, Cognitive-Communication Disorder
increasingly cluster and switching scores are reported as Cognitive Functioning
well. The COWAT is valuable in detecting cognitive Cognitive Processing
dysfunction, but it requires further study before defini- Cue
tive conclusions are possible with regard to performance Cued Recall
patterns that can be linked with specific neurogenic Error Recognition and Correction
behavioral deficits. Free Recall
Mayos Older American Normative Studies (MOANS)
National Adult Reading Test
Cross References Phonemic Cue
Semantic Cue
Aphasia Semantic Fluency
Aphasia Tests Verbal Mediation
Benton, Arthur Western Aphasia Battery
706 C Contusion (Cerebral)
Definition
Contusion (Cerebral)
Coping is responding to environmental stimuli, events,
Cortical Contusion and circumstances for the purpose of minimizing or
managing stress, solving problems, and modulating phys-
iological and emotional responses. Coping is often paired
with stress (as the latter elicits the former) in what has
Conventional Antipsychotics become the stress and coping literature associated with
Richard Lazarus and Susan Folkman. Stress responses
Antipsychotics typically involve appraising the stimulus or event, which
begins the process of assigning value (e.g., distressing)
and determining responses (e.g., fight, flight, freeze).
Coping is a process that follows stress appraisals, and
Conversation Analysis coping responses seek to manage stress with cognitive,
physiological, and behavioral responses. Various coping
Discourse Assessment strategies have been categorized, such as appraisal-
focused, problem-focused, or emotion-focused coping.
Current Knowledge
References and Readings
Atherosclerosis, which is the process by which cholesterol
and fat deposits adhere to the inside walls of blood vessels, Hansson, G. K. (2005). Inflammation, atherosclerosis, and coronary
creates plaques that block the blood supply through blood artery disease. New England Journal of Medicine, 352, 16851695.
Virmani, R., Kolodgie, F. D., Burke, A. P., Farb, A., & Schwartz, S. T.
vessels. When this narrowing or occlusive process blocks the
(2000). Lessons from sudden coronary death: a comprehensive mor-
coronary artery, the accompanying obstruction to blood phological classification scheme for atherosclerotic lesions. Arterio-
flow reduces the supply of oxygen and nutrients to the sclerosis, Thrombosis, and Vascular Biology, 20, 12621275.
heart muscle, creating ischemia that causes chest pain (an-
gina pectoris), or death of the cells of the heart (myocar-
dial infarction). At times, it can cause other problems such
as arrhythmias (abnormal heart rhythm) or congestive heart
failure, in which the heart is unable to pump sufficient blood
to the remainder of the body. CAD is the most common type
Coronary Heart Disease
of heart disease and the leading cause of death in the USA.
Coronary Disease
Risk factors for CAD have been well studied; these include
smoking, diabetes, hypertension, hyperlipidemia, obesity,
sedentary lifestyle, excessive alcohol intake, and family his-
tory. Although chest pain is the most common symptom,
dyspnea, palpitations, diaphoresis, nausea, radiation of the
pain to the neck and left arm, and no symptoms at all, also Corporis Callosi
are possible. The diagnosis relies on electrocardiogram,
blood levels of cardiac enzymes, echocardiography, select Corpus Callosum
Correction Factor C 709
Ganglion
Gazzaniga, M. S. (1939 )
Current Knowledge Sperry, Roger Wolcott (19131994)
Split-brain
The corpus callosum is the largest commissure of the
adult brain that provides a bridge for the passing of
information from one cerebral hemisphere to the other References and Readings
by 200300 million myelinated and unmyelinated axons.
The size of the corpus callosum varies greatly but is Barr, M., & Kiernan, J. (1983). The human nervous system An anatomical
generally larger in females than in males. In the human, viewpoint (4th ed.). Philadelphia: Harper and Row.
the corpus callosum begins development around the 11th Gazzaniga, M. S. (2005). Forty-five years of split-brain research and still
going strong. Nature Reviews Neuroscience, 6(8), 653659.
week of gestation and continues through adolescence.
Haines, D. (2006). Fundamental neuroscience for basic and clinical appli-
Initially, the corpus callosum is composed of astrocytic cations (3rd ed.). Philadelphia: Churchill Livingstone Elsevier.
processes, which serve as conduits for growing axons Kandel, E., Schwartz, J. H., & Jessel, T. M. Principles of neural science
extending to the contralateral hemisphere. This inter- (4th ed.). New York: McGraw-Hill.
hemispheric commissure lies beneath the cortex at the Paul, L. K., Brown, W. S., Adolphs, R., Tyszka, J. M., Richards, L. J.,
Mukherjee, P., et al. (2007). Agenesis of the corpus callosum:
bottom of the cerebral longitudinal fissure. It forms
Genetic, developmental and functional aspects of connectivity.
much of the roof of the lateral ventricles and is composed Nature Reviews Neuroscience, 8(4), 287299.
of four parts: the rostrum, the genu, the body (also known
as the trunk), and the splenium. Each portion of the
corpus callosum is responsible for connecting symmetri-
cal regions of the two hemispheres with the rostrum and
genu connecting portions of the prefrontal and premotor Corpus Mamillare
cotices, the body interconnecting the premotor, motor,
supplementary motor, and the posterior parietal cortices, Mammillary Bodies
while the splenium connects portions of the temporal,
occipital, and parietal lobes. Although being the largest
white matter tract of the brain, the corpus callosum is not
essential for life. The complete or partial absence of the Correction Factor
corpus callosum is known as agenesis of the corpus callo-
sum. This condition is rare, and estimates of incidence K Scale
710 C Correlation Coefficients
Historical Background 6
y
4
The correlation, or co-relation, coefficient is typically
attributed to Karl Pearson who developed the formalized
2
idea of correlation during the mid- to late 1800s. However,
the beginning of the idea of correlation may have 0
come from Sir Francis Galton, cousin of Charles Darwin.
0 2 4 6 8 10
Galton worked on genetic heritability of sweet peas. x
Through his work on heritability, he developed the
If data are positively related, such that as one variable
beginnings of regression and correlation. Pearson, who
increases in value so does the other, then one would be
worked in Galtons lab and was later his biographer, attrib-
able to draw a line that would approximately represent
uted the development of the regression slope to Galton.
the data. The below example illustrates an ideal relation-
Pearson then generalized Galtons work into the Pearson
ship that rarely occurs in research of a 1 correlation
Product Moment Correlation (PPMC), or moment
such that for every one unit that x goes up, y goes up one
meaning the average of a set of products. The PPMC is
unit.
often identified as r, which Galton originally used to
denote regression and Pearson later used as notation
for correlation. In 1896, Pearson published an article in 30
which he credited Bravais for developing the rudiments of
the correlation formula. In this work published in the 25
Philosophical Transactions of the Royal Society of London,
Pearson showed that the optimum values for the regres- 20
y
read this text. Simply because the light is on while you are
4 reading does not mean that light is causing you to read,
they are certainly related, as there will always be some
light source when you read, but one does not cause the
2 other. A determination of causation requires adequate
0 2 4
x
6 8 10 experimental design.
There are certain important components that underlie
The above examples show linear relationships between
most correlations. Correlations are most meaningful
variables, which is an assumption of most correlation
when certain criteria are met, and different correlation
coefficients. An examination of scatter plots can also
coefficients are affected differently by failure to meet
show nonlinear relationships, which are not well repre-
different assumptions. The Pearson product moment
sented by standard correlation coefficients, such as the
correlation coefficient has the most stringent set of
PPMC, but can be shown with other methods, such as
assumptions. These include:
eta. The below example has a near 0 Pearson r, but the
variables are clearly related nonlinearly. 1. The data are interval: The level of measurement must
at least be interval as opposed to nominal or ordinal,
25 though this can be managed if it is violated.
2. The data must have a linear relationship: Most correla-
20 tions assume that the data are linearly related, as op-
posed to nonlinear. To the degree which the data are
15
better explained by a nonlinear relationship correlation
y
10
coefficients will underestimate the relationship. Prior to
completing a correlation analysis this can be examined
5 by looking at a scatter plot, as discussed above.
3. The variables have similar and normal underlying
0 distributions (more formally in a bivariate correlation,
0 5 10 15 20 such as the Pearson r): To the degree that the
x
712 C Correlation Coefficients
distributions of the different variables differ, the cor- also other methods that are more robust to violations of
relation will attenuate the relationship between them. the above assumptions, such as Spearmans rho or eta in
For example, if correlating a variable that is normally the case of a nonlinear relationship. The correlation
distributed, like height, with one that is highly skewed coefficient is considered a measure of effect size, which is
like ability to walk. This is done when correlating a a measure of how different two distributions are. By
continuous variable with a dichotomous variable or convention a correlation of .1 is small, .3 moderate and
when correlating interval data with ordinal. If this .5 large (Cohen, 1988), at least in the behavioral sciences
assumption is violated to a significant degree then that can expect weaker relationships in general due to
rank order correlation may be used, such as the the complexity of the subject matter. For example, the
Spearmans rho or other nonparametric correlations. correlation between IQ and academic achievement is
4. The data must have homoscedasticity: The error, or approximately .55 (Griffenstein & Baker, 2003), but one
random variance in X and Y, contained in the data, is would expect no effect for a correlation between IQ and
assumed to be equal along the entire distribution. If, height. The correlation coefficient can also be squared to
for example, one portion of the distribution has a give the coefficient of determination, which is the amount
much larger random variance, then the correlation of variance in one variable that is accounted for by
may overrepresent the relationship because it would another. For example, if one had a correlation of .55
underrepresent the amount of variance that is between IQ and academic achievement then the coeffi-
attributable to error. If data are heteroscedastic, then cient of determination (r 2) would be .30, which means
the distribution will not be normal and the correlation that approximately 30% of the variance in academic
may misrepresent the data. achievement is accounted for by IQ.
5. The data has no or limited outliers: Correlation is
1. PPMC or r: The PPMS is an association of two
sensitive to outliers. Some forms of correlation are
continuous variables that shows the degree of linear
more robust to a violation of this assumption than
relationship between them. It can be calculated using
others, but normally correlation is a representation of
raw scores, deviation scores, or by using a covariance
deviations of mean values. Thus, to the degree that the
formula. A common method is to calculate the
mean is affected by outliers then the correlation will be
standard score (or z score) and sum the products of
also. If there are many outliers, or few that are large,
the two variables and divide them by the degrees of
this problem can be managed through use of a
freedom (n 1) or S xy=n S 1.
Spearman rho, which ranks data.
2. Point-biserial or rpb: This is a special case of the PPMC
6. There is limited measurement error present in the
for use when there is a continuous variable, such as
sample: It is assumed that the measurement is relative-
height, and a dichotomous nominal variable, such as
ly free from or has limited error. In other words, if one
gender. The point-biserial correlation is for naturally
has an invalid test that only measures error then
dichotomous variables, such as gender, not artificially
the correlation will be near zero, as the data will be
dichotomized variables, such as taking a naturally
random and show little relationship. It is rare in
continuous distribution, such as intelligence, and
behavioral research to be free from error, but limiting
making it into high and low intelligence.
it is an important aspect of experimental control.
3. Phi: This is a special case of the PPMC for use when
7. The data have adequate variance: In order to
both variables are dichotomous and nominal. Note that
adequately assess relationships among variables there
as with rpb this is for naturally dichotomous variables,
must be variance among the variables. In general, the
such as gender, not artificially dichotomized variables.
more variance the higher the correlation will be.
4. Biserial or rb: This is for use when there is one contin-
To the extent that these criteria are violated, which they uous variable, such as height, and a dichotomized
often are, the correlation coefficient is more or less im- variable, such as high and low intelligence. So, the
pacted depending on the type of correlation. biserial correlation measures the relationship between
There are several different methods calculating basic X and Y as if Y were not artificially dichotomized. This
correlation coefficients depending on the type and char- is similar to the point-biserial, but the formula is
acteristics of the data being analyzed. There are several designed to replace some of the variance that is lost.
different types of correlation that depend on the level of One of the important components listed above is that
measurement (nominal, ordinal, interval, ratio) being the data have adequate variance. However, when a
made. Most are special cases of the Pearson r. There are variable is dichotomized much of the variance is lost.
Cortical Blindness C 713
pathways. The underlying pathophysiological mecha- currently unclear whether recovery of visual capacity in
nism involves direct destruction and/or de-afferentation cortical blindness is mediated by spared neural tissue
of primary visual cortex (striate cortex, Brodmann area within primary visual cortex, or whether it reflects the
17, or V1). The term Antons syndrome is applied to strengthening and increasing utilization of alternative
patients with cortical blindness who demonstrate explic- visual pathways to extrastriate cortical regions that bypass
it denial or unawareness (anosognosia) of their visual the damaged geniculostriate system, or both. Anosogno-
impairment. sia for visual loss in patients with Antons syndrome also
tends to diminish over time, often parallel to the resolu-
tion of the visual impairment, but partial defects of aware-
ness are not uncommon. In cases of transient cortical
Categorization
blindness due to RPLS, full recovery of vision can occur
within a few days along with complete resolution of the
Although no universally agreed upon classification system
characteristic neuroradiological abnormalities.
exists, patients with cortical blindness differ in terms of the
severity of the visual loss, the presence/absence of spared
visual abilities (conscious or unconscious), the degree of
awareness of the deficit, and the extent of functional recov-
ery. There are also variations across cases with respect to the
Neuropsychology and Psychology of
capacity to generate internal visual representations using
Cortical Blindness
mental imagery and the susceptibility to experience abnor-
In severe cases of cortical blindness, all forms of conscious
mal visual sensations/hallucinations. The precise neurobio-
visual perception may be abolished. However, this type
logical mechanisms underlying these individual differences
of total visual loss is relatively uncommon and many
in clinical presentation remain to be determined.
patients retain at least some rudimentary visual awareness
of motion or light. Visual form discrimination is pro-
foundly impaired and objects, faces, or written words can-
Epidemiology not be identified. Visuospatial orientation is also severely
compromised and patients may repeatedly bump into
Cortical blindness is a relatively rare condition, typically objects when attempting to navigate in their environment.
caused by bilateral occipital strokes in the territory of the Although individuals with cortical blindness do not
posterior cerebral arteries. Other less common etiologies have normal conscious awareness of visual events, they
include anoxic brain damage, carbon monoxide poison- can sometimes demonstrate surprising ability to respond
ing, head trauma, and occipital lobe tumors. Transient to stimuli presented within the blind portions of their
cortical blindness can be seen in the context of the revers- visual fields. Specifically, patients may be able to detect,
ible posterior leukoencephalopathy syndrome (RPLS) locate, and discriminate visual stimuli that they claim not
related to hypertensive encephalopathy, the use of chemo- to see. Residual visual capacity within the cortically blind
therapeutic and immunosuppressant drugs, or the injec- field in the absence of conscious awareness has been
tion of radiological contrast agents during cerebral referred to as blindsight (Weiskrantz, 1986; Stoerig &
angiography. Cowey, 1997, 2007; Stoerig, 2006). The fact that blindsight
can be observed in patients with extensive destruction or
de-afferentation of primary visual cortex suggests that the
Natural History, Prognostic Factors, preserved visual abilities of these individuals are mediated
Outcomes by neural pathways from retina to extrastriate visual cor-
tex that bypass the damaged geniculostriate system. There
Although some recovery of visual function is observed in are in fact a number of alternative non-geniculostriate
most cases of cortical blindness, patients with neuroim- pathways capable of transmitting visual information to a
aging evidence of extensive structural damage to occipital variety of temporo-parietal extrastriate cortical areas via
cortex typically do not regain full normal vision. Residual subcortical relay nuclei in the midbrain and diencephalon
visual field defects are common, and cortical blindness (Weiskrantz, 1986; Stoerig & Cowey, 1997, 2007; Stoerig,
may evolve into visual agnosia characterized by a persis- 2006). The specific visual functions retained in blindsight
tent inability to recognize objects, faces, or words despite may depend on which of these multiple parallel visual
the return of more elementary visual functions. It is pathways are available to patients. For instance, the
Cortical Blindness C 715
residual capacity to detect motion and to locate and visual imagery may provide faulty input to an otherwise
manually grasp targets presented in the blind field may intact monitor. The loss of normal visual input following
depend on projections from the retina to the superior damage to primary visual cortex may in fact give rise to
colliculus, with additional connections to the pulvinar frequent release hallucinations in patients with cortical
and to middle temporal (MT/V5) and dorsal parietal blindness due to increased cortical excitability in de-affer- C
cortex (Danckert & Rossetti, 2005). Other alternative ented extrastriate areas. Furthermore, since visual percep-
pathways from retina to ventral extrastriate cortex or tion and imagery are mutually inhibitory under normal
amygdala might be involved in mediating unconscious circumstances, the absence of bottom-up activation by
visual form discrimination (Trevethan, Sahraie, & Wei- geniculostriate afferent signals may be accompanied by a
skrantz, 2007) and implicit recognition of emotional fa- relative enhancement of visual imagery mediated by unop-
cial expressions (Morris, DeGelder, Weiskrantz, & Dolan, posed top-down activation of sensory representations in
2001; Pegna, Khateb, Lazeyras, & Seghier, 2005). Func- extrastriate cortex. Patients may deny blindness because
tional imaging studies in patients with blindsight are con- they continue to experience internally generated visual sen-
sistent with the notion that unconscious processing of sations and may mistake these for veridical perceptions
visual information depends on non-geniculostriate visual resulting from retinal stimulation by external visual events.
pathways. Specifically, these investigations have demon- To understand anosognosia for visual loss, it is useful
strated activation in midbrain/thalamic nuclei, extrastriate to briefly consider what is currently known about
cortical areas, and the amygdala in the absence of concomi- the neural correlates of normal visual awareness. In this
tant activity in the lesioned primary visual cortex during context, it is important to emphasize that conscious
visual stimulation of the blind field (Sahraie, Weiskrantz, awareness of visual events normally entails the capacity
Barbur, Simmons, Williams, & Brammer, 1997; Stoerig, to acknowledge, describe, reflect upon, and make appro-
2006; Morris et al., 2001; Pegna et al., 2005). priate cognitive judgments about ongoing visual ex-
Patients with cortical blindness may demonstrate un- periences (Weiskrantz, 1997; Block, 2005; Dehaene,
awareness of their profound visual impairment. This Changeux, Naccache, Sackur, & Sergent, 2006). Thus,
striking clinical condition is referred to as Antons syn- awareness and the ability to provide introspective report
drome. Anosognosia for visual loss can take several dif- or commentary about the quality, content, and veridi-
ferent forms. In extreme cases, patients emphatically deny cality of visual perceptions are closely related functions.
being blind and produce confabulatory responses when Although localized neural activity in cortical visual areas
questioned about their visual abilities. Other patients (striate and extrastriate cortex) is obviously necessary for
acknowledge a change in their vision but they typically visual information to reach this commentary stage of
offer a variety of excuses to explain the difficulty, attribut- conscious awareness, it is by itself not sufficient (Wei-
ing it to poor lighting conditions or to a problem skrantz, 1997; Rees, Kreiman, & Koch, 2002; Dehaene
with their eyeglasses. A number of theories have been et al., 2006). In particular, evidence from recent neuroim-
advanced to explain anosognosia for blindness (Bisiach aging studies suggests that conscious vision requires the
& Geminiani, 1991; Heilman, 1991; Celesia, Brigell, & additional recruitment of dorsolateral prefrontal and pa-
Vaphiades, 1997; Adair, Schwartz, & Barrett, 2003). For rietal cortical regions implicated in visual attention and
instance, it has been proposed that normal visual aware- working memory (Rees, Kreiman, & Koch, 2002; Dehaene
ness depends on a hypothetical monitor located in extra- et al., 2006). Prefrontal cortex is also involved in mediat-
striate visual association areas that receives afferent input ing strategic cognitive operations necessary for critically
from primary visual cortex (Heilman, 1991). In addition evaluating and interpreting the meaning and signific-
to evaluating activity within the visual areas of the brain, ance of visual experiences in order to determine the
the monitor also sends efferent information to cortical most appropriate behavioral response.
language areas enabling subjects to verbally comment on Activation of frontal and parietal regions by input
their visual experiences. Lesions that disrupt the input from cortical visual areas is also likely to play an essential
and/or output connections of the visual monitor, or pro- role in awareness of abnormal visual function. Specifi-
duce damage to the monitor itself, result in anosognosia cally, damage to primary visual cortex and extrastriate
for visual impairment that may include verbal denial of areas may give rise to visual field defects and/or domain-
the deficit and the production of confabulatory responses specific impairments in processing distinct visual stimu-
by the disconnected language areas. It has also been sug- lus attributes (e.g., form, color, motion). If the same lesions
gested that in cases of cortical blindness, internally gener- also interfere with the bottom-up activation of the fronto-
ated visual experiences in the form of hallucinations or parietal cortical network that normally enables conscious
716 C Cortical Blindness
awareness and introspective report about visual experi- movement, or identity of objects presented in their blind
ences, anosognosia for the visual impairment ensues. De- field using a forced-choice response method (e.g., was it a
fective awareness of visual function may also result from key or a coin?). Better than chance performance on these
direct damage to the putative fronto-parietal network that types of tasks is taken to be indicative of blindsight. Alter-
provides the critical top-down attentional amplification natively, patients can be asked to try to reach for and grasp
required for perceptual information processed within objects in their blind field. Regardless of the testing method
visual cortical modules to enter consciousness, or it may used, it is important to establish after each trial whether
be produced by lesions that disrupt feedforward/feedback the patient had any conscious awareness of the visual
connections between fronto-parietal cortex and visual stimulus. Visual imagery can be tested by asking the sub-
processing areas. In summary, conscious awareness of jects to answer questions about the visual attributes of
both normal and abnormal vision requires dynamic re- familiar objects or animals (e.g., do polar bears have long
ciprocal interactions between cortical visual areas and or short tails?). Patients should be questioned about ab-
specialized regions within frontal and parietal cortex. normal visual perceptions and hallucinations. Unaware-
Lesions that disrupt the spatial distribution, intensity, ness of deficit may be revealed by spontaneous comments
or timing of activation across the different functional or behavior, but patients should also be specifically asked
components of this large-scale neural system may give to describe the quality and content of their visual experi-
rise to a variety of clinical conditions characterized by ences. The severity of anosognosia can be quantified by
unawareness of preserved or impaired visual processing, simple rating scales (Bisiach & Geminiani, 1991; Celesia,
including blindsight, visual neglect, and anosognosia for Brigell, & Vaphiades, 1997).
blindness. Structural neuroimaging (CT/MRI) studies in
patients with cortical blindness due to stroke typically
reveal extensive bilateral infarctions involving primary
Evaluation visual cortex and underlying white matter, often with
evidence of lesion extension into adjacent extrastriate
Neuro-ophthalmological examination in cortical blind- visual association areas (Brodmann areas 18/19, 37)
ness confirms the normal fundoscopic appearance of the (Figure 1). SPECT/PET scans frequently demonstrate
retinas and optic nerves. The pupillary light response is blood flow/metabolic abnormalities that extend beyond
characteristically preserved, as the retinal fibers that me- the boundaries of the lesions seen on CT/MRI, providing
diate this reflex leave the optic tract prior to the origin of evidence that the cortical visual areas that appear spared
the geniculostriate pathways. Reflexive blinking to threat- by structural imaging studies are in fact functionally
ening visual gestures is usually absent, and in the majority compromised. In patients with transient cortical blind-
of cases optokinetic nystagmus cannot be elicited by ness due to RPLS, neuroimaging studies have demon-
moving a striped object in front of the patients eyes. strated reversible bilateral subcortical white matter
The dissociation between preserved pupillary light re- abnormalities in posterior occipito-temporo-parietal
sponse and absent optokinetic nystagmus can have diag- regions attributable to vasogenic edema.
nostic utility in distinguishing patients with cortical
blindness from patients with severe peripheral visual loss
due to bilateral eye or optic nerve pathology in whom
both responses are lost, and also from individuals with Treatment
psychogenic blindness in whom both responses are
retained. A number of behavioral approaches have been tried with
Clinical evaluation of visual function in patients with varying degrees of success to restore visual function in the
cortical blindness should include tests of visual acuity, cortically blind field and/or help patients learn alternative
determination of light and motion perception in different strategies to compensate for their visual impairment
sectors of the visual field, tests of spatial localization, as (Kerkhoff, 2000). Attempts to increase awareness of the
well as assessments of color perception, object and face visual deficit in patients with anosognosia constitute an
recognition, and reading ability. Studying blindsight usu- important component of the treatment program. It has
ally requires the use of specialized testing equipment in an been shown that blindsight performance can be improved
experimental laboratory environment. However, in the by training, and the use of these techniques may aid the
clinical setting blindsight can be tested by requiring recovery of vision in individuals with cortical blindness
patients to guess the presence/absence, location, (Stoerig, 2006; Sahraie et al., 2006).
Cortical Blindness C 717
Cortical Blindness. Figure 1 Diffusion-weighted MRI scan in a patient with cortical blindness following bilateral posterior
cerebral artery strokes. Note massive destruction of primary visual cortex (Brodmann area 17), with lesion extension into ventral
extrastriate visual association areas (Brodmann areas 18,19,37). This individual demonstrated complete denial of blindness
(anosognosia), consistent with Antons syndrome
Cross References
Cortical Color Blindness
Contrecoup Injury
Achromatopsia Edema
Focal Lesion, Contusion
Intracranial Pressure
B ETH R USH Bigler, E. D. (2001). The lesions in traumatic brain injury: Implications
Mayo Clinic for clinical neurophysiology. Archives of Clinical Neuropsychology,
16, 95131.
Jacksonville, FL, USA
Graham, D. I., Saatman, K. E., Marklund, N., Conte, V., Morales, D.,
Royo, N., & McIntosh, T. K. (2006). The neuropathology of trauma.
In R. W. Evans (Eds.), Neurology and Trauma (2nd ed., pp. 4594).
Synonyms New York: Oxford University Press.
Cortical Deafness
Definition
Pure Word Deafness
Cortical contusions are bruises on the brain tissue that
form from the small blood vessel leaks (veins and arteries
covering the parenchymal tissue), or a series of microhe-
morrhages following trauma. Trauma is usually the result
of physical blows to the head such as those sustained in a Cortical Lewy Body Disease
motor vehicle accident, direct blow to the head from (CLBD)
assault, or significant sports-related injuries. Veins and
arteries on the surface of the brain are damaged, which Dementia with Lewy Bodies
results in bleeding and bruising. When the blood vessel is
torn, blood escapes from the vessel at a rate that is faster
than the blood that can be absorbed by the brain. Conse-
quently, cortical contusions commonly result in edema
and increased intracranial pressure.
Cortical Magnification
R ONALD A. C OHEN
Current Knowledge Brown University
Providence, RI, USA
Second to diffuse axonal injury, cortical contusion is the
most common type of intra-axial lesion following brain
trauma. By radiologic definition, a cortical contusion Definition
must involve some portion of the superficial gray matter.
Because gray matter has more vasculature than white Cortical magnification refers to the fact that the number
matter, most cortical contusions are hemorrhagic, where- of neurons in the visual cortex responsible for processing
as diffuse axonal injuries are rarely hemorrhagic. The the visual stimulus of a given size varies as a function of
frontal and temporal lobes are the most common sites the location of the stimulus in the visual field. Stimuli
of cortical contusions. When present, cortical contusions occurring in the center of the visual field that have been
are usually found bilaterally. Compared to diffuse axonal detected in the fovea of retina are processed by a very large
injury lesions, cortical contusions are much less likely to number of neurons in the primary visual cortex of
involve an initial presentation of coma or altered loss of the occipital lobe, though these neurons handle only a
consciousness. very small region of the central visual field. Conversely,
Cortical Mapping C 719
stimuli detected in the peripheral visual field tend to be specific informational characteristics (e.g., shape, color,
processed by a much smaller number of neurons in the texture, position, movement), neurons with greatest sen-
primary visual cortex. sitivity to these dimensions vary as a function of cortical
magnification factors and a tradeoff of size of the recep-
tive field and the number of neurons dedicated to each C
Current Knowledge part of the field.
surface for the purpose of identifying areas critical for is based on negative responses, thorough mapping of
sensory, motor or language function. This procedure is language cortex requires administration of tasks assessing
utilized when brain surgery involves the removal or a range of language functions.
disruption of potentially functional cortical areas. Sites Depending on the location of the area identified and
identified via cortical mapping are typically spared the nature of the response elicited by stimulation, it is
from resection, with the goal of preserving function generally held that removal of a positive sensory, motor,
postoperatively. or language site identified by stimulation will result in
Stimulation is applied using a bipolar stimulator, usu- impaired function postoperatively. Of the few clinical
ally via pairs of adjacent subdural electrodes. The procedure series published, results suggest that postoperative
can be conducted intra-operatively in a conscious patient function is best preserved when the resection margin
before resection of brain tissue, or extra-operatively, if sub- exceeds 1 cm from the functional site. On the other
dural electrodes have been implanted, most commonly in hand, there is some evidence that certain sites identified
pharmacologically resistant epilepsy patients who require by stimulation can be removed without concern of
intracranial EEG monitoring to delineate the region of postoperative decline. These include motor sites identi-
seizure onset (Fig. 1). The identification of sensory and fied in the supplementary motor area, tongue, and lower
motor sites is based on stimulation-evoked positive face areas (due to their bilateral representation), and
responses, such as a subjective sensation (e.g., tingling) possibly, language sites identified in the basal temporal
or an observable movement (e.g., muscle twitch). In con- region, although this is somewhat controversial.
trast, stimulation of language cortex elicits no experiential For motor and sensory mapping, the duration of elec-
or observable response in an inactive patient. Rather, trical stimulation is typically 2 s, whereas language
cortical language mapping relies on negative responses, mapping typically requires 48 s of stimulation, depending
in that the patient must be engaged in a language task on the particular task under assessment. The level of stim-
and stimulation of language cortex will disrupt task ulation administered ranges from 1 mA to a maximum of
performance. Thus, for language mapping, stimulation 17 mA, with motor and sensory cortex typically utilizing
produces a discrete, reversible lesion, theoretically en- <10 mA and language cortex typically requiring >10 mA.
abling the examiner to observe the functional conse- Under normal circumstances, cortical stimulation causes
quences of damage to the site(s) stimulated. Stimulation neither pain nor discomfort. One risk of stimulation, how-
of frontal language cortex typically produces speech ar- ever, is the evocation of a seizure, particularly in patients
rest, whereas stimulation of posterior (temporal/parietal) with epilepsy, due to a likely lower seizure threshold in
language cortex typically elicits comprehension, naming epileptogenic areas. To minimize the probability of a stim-
or reading difficulties. Because cortical language mapping ulation induced seizure, EEG is monitored on an ongoing
Cortical Mapping. Figure 1 Implanted subdural electrode grid used for EEG recording and electrical stimulation mapping
Cortical Motor Pathways C 721
basis, and the stimulation intensity is lowered when location of posterior temporal and parietal language
abnormal discharges are associated with stimulation. Nev- sites appears to vary more among individuals, although
ertheless, benzodiazepines are typically kept close at hand this might merely reflect alterations in the distribution of
for IV administration for instances when a seizure occurs language sites in patients with epileptogenic cortex in the
and fails to resolve rapidly on its own. temporal region. C
Historical Background
Clinical Uses
Alteration of function via cortical stimulation in both
One of the main challenges with brain surgery involving
animals and humans dates back to the mid 1800s. The
cortical regions is to remove a sufficient amount of
procedure came into clinical use in the early twentieth
pathological tissue without removing areas critical for
century in association with surgical resection of epilepto-
function. Cortical mapping is used to identify cortical
genic cortex in patients with pharmacologically refractory
regions critical for function, in order to spare these areas
epilepsy. Initially used to identify sensory and motor
from resection or protect them from damage during
cortex, Wilder Penfield and colleagues pioneered the tech-
surgical procedures. Cortical mapping is typically
nique in the 1950s for use in the identification of language
performed when there is concern that resective brain
cortex. Stimulation-based language mapping was further
surgery (e.g., tumor resection, epilepsy surgery) might
refined by George Ojemann and colleagues who essential-
impinge upon, or possibly overlap with cortical regions
ly established the current clinical standards. In addition to
necessary for function.
its clinical utility, investigators have used the opportunity
provided by clinical stimulation to investigate structure-
function relations. These studies build upon the lesion
model, contributing more precise information regarding Cross References
functional localization due to both the controlled setting,
and the smaller, more discrete lesions induced Epilepsy
temporarily by stimulation than that typically found Functional Imaging
with naturally occurring lesions. Localization
supranuclear palsy, no clear genetic etiology has been retrieval deficits. Remote memory has been little studied
identified. Dementia and other cognitive and behavioral in CBD, but the pattern of poor recall but intact recogni-
abnormalities were thought to be rare in CBD until the tion on remote memory tasks suggests a retrieval deficit.
last decade, but it is now appreciated that the frequency of Visuospatial impairments have been observed.
neurobehavioral abnormalities observed as a presenting With respect to emotional and neuropsychiatric C
problem or during the course of the condition is quite issues, depression is common in CBD (73% of patients),
high. It might be that the inconsistent incidence and though apathy (40%), irritability (20%), and agitation
prevalence estimates of cognitive impairment in CBD (20%) also occur with frequency.
are a function of whether patients were drawn from
movement disorder, dementia, or psychiatry clinics.
Evaluation
Natural History, Prognostic Factors, The selection of specific neuropsychological tests in CBD,
Outcomes like any other condition, should be guided by the referral
questions and the patients ability to cooperate and meet
Disease onset is usually in the sixth decade of life, and task demands. However, tests of executive function (e.g.,
mean time to death from diagnosis is about 7 years. planning, abstraction, and cognitive flexibility), praxis,
visuospatial functions, attention, learning and memory,
and word retrieval should be employed. Symptom
Neuropsychology and Psychology of inventories relating to apathy, depression, and frontal
Corticobasal Degeneration behavior syndromes, such as the Neuropsychiatric
Inventory, Hamilton depression scale, and Frontal
CBD involves an asymmetric apraxia, most often of the Systems Behavior Scale can be helpful in characterizing
ideomotor type, but ideational and limb kinetic apraxias neuropsychiatric features of CBD.
also occur. Thus, patients most often have difficulty
demonstrating the use of tools. Poor drawing (construc-
tional apraxia) is also commonly seen. Language distur- Treatment
bance occurs early or during progression of the syndrome,
and the aphasia is most often non-fluent (about 56% of Some cases may show transient improvement in parkin-
cases) or anomic (30%). The pattern of performance on sonian features with levodopa treatment; dopamine ago-
language tests in patients with the traditional CBD pre- nists are generally even less helpful than levodopa.
sentation is somewhat inconsistent across studies, but Tremor, if present, may be alleviated by benzodiazepines.
phonological impairments may be an important feature. Antidepressants with anticholinergic profiles are to be
Performance on verbal fluency tests, especially lexical or avoided given possible adverse cognitive side effects, but
phonemic fluency tests, is usually impaired either due to selective serotonin reuptake inhibitors may be helpful in
the executive demands of those tasks or aphasia. Perfor- treating depression. Speech therapy is helpful in treating
mance on semantic memory tasks such as conceptual dysphagia.
matching and visual confrontation naming and expressive
vocabulary is relatively preserved and impaired in a mi-
nority of patients, although some studies have reported Cross References
considerable impairment on semantic tasks early in the
disease. When naming is impaired, disproportionate Basal Ganglia
benefit is derived from cuing suggesting a retrieval rather Corticobasal Degeneration
than semantic memory deficit. Comprehension is typical- Frontal Lobes
ly preserved early, but comprehension of grammatically Frontal Temporal Dementia
complex material declines with disease progression. Frontotemporal Lobar Degeneration
Executive dysfunction, as indicated by poor perfor- Gait Disorders
mance on tasks such as the card sorting tasks and the Movement Disorders
Trailmaking test is common. Episodic memory impair- Parkinson Plus Syndromes
ments in CBD are relatively mild early in the course of the Parkinsons Disease
condition and appear to involve both encoding and Parkinsonian Movement
726 C Cortico-Basal Ganglia Loop
Striatum
Tauopathy Corticoliberin
Corticotropin-Releasing Hormone
References and Readings
Belfor, N., Amici, S., Boxer, A. L., Kramer, J. H., Gorno-Tempini, M. L.,
Rosen, H. J., et al. (2006). Clinical and neuropsychological features
of corticobasal degeneration. Mechanisms of Ageing and Develop-
ment, 127, 203207.
Boeve, B. F. (2007). Parkinson-related dementias. Neurologic Clinics, 25,
Corticospinal Tract
761781.
Geda, Y. E., Boeve, B. F., Negash, S., Graff-Radford, N. R., Knopman, Cortical Motor Pathways
D. S., Parisi, J. E., et al. (2007). Neuropsychiatric features in 36
pathologically confirmed cases of corticobasal degeneration. Journal
of Neuropsychiatry and Clinical Neurosciences, 19, 7780.
Graham, N. L., Bak, T. H., & Hodges, J. R. (2003). Corticobasal degener-
ation as a cognitive disorder. Movement Disorders, 18, 12241232.
Kertesz, A. (2003). Pick complex: An integrative approach to frontotem- Corticotropin-Releasing Factor
poral dementia: Primary progressive aphasia, corticobasal degenera-
tion, and progressive supranuclear palsy. The Neurologist, 9, 311317.
(CRF)
Lang, A. E. (2003). Corticobasal degeneration: Selected developments.
Movement Disorders, 18(Suppl. 6), S5156. Corticotropin-Releasing Hormone
Litvan, I., Cummings, J. L., & Mega, M. (1998). Neuropsychiatric features
of corticobasal degeneration. Journal of Neurology, Neurosurgery, and
Psychiatry, 65, 717721.
Murray, R., Neumann, M., Forman, M. S., Farmer, J., Massimo, L.,
Rice, A., et al. (2007). Cognitive and motor assessment in autopsy-
proven corticobasal degeneration. Neurology, 68, 12741283. Corticotropin-Releasing Hormone
Sha, S., Hou, C., Viskontas, I. V., & Miller, B. L. (2006). Are frontotem-
poral lobar degeneration, progressive supranuclear palsy and corti- B ETH K UCZYNSKI 1, S TEPHANIE A. KOLAKOWSKY-H AYNER 2
cobasal degeneration distinct diseases? Nature Clinical Practice 1
University of California
Neurology, 2, 658665.
Davis, CA, USA
Troster, A. I., & Fields, J. A. (2008). Parkinsons disease, progressive 2
supranuclear palsy, corticobasal degeneration, and related disorders Santa Clara Valley Medical Center,
of the frontostriatal system. In J. E. Morgan & J. H. Ricker (Eds.), Rehabilitation Research Center
Textbook of clinical neuropsychology (pp. 536577). New York: Psy- San Jose, CA, USA
chology Press.
Synonyms
Cortico-Basal Ganglia Loop
Corticoliberin; Corticotropin-releasing factor (CRF)
CorticalSubcortical Loop
Definition
Corticobasal Syndrome
Corticotropin-releasing hormone (CRH) is a hormone
Corticobasal Degeneration that is primarily produced by the hypothalamus and is
involved in the stress response. It is released from the
paraventricular nucleus of the hypothalamus with the
Corticodentatonigral primary action within the anterior lobe of the pituitary
to initiate the release of adrenocorticotropic hormone
Degeneration with Neuronal (ACTH). CRH (41 amino acids long) is derived from a
Achromasia 191 amino acid preprohormone. Other areas of CRH
synthesis include peripheral tissues, and it is highly
Corticobasal Degeneration expressed in the placenta.
CPT C 727
Cross References
Counterindicant
Hormone
Contraindication
C
References and Readings
CPM
Coumadin Raven Matrices
Warfarin (Coumadin)
CPRS
Counseling
Conners Rating Scales
Psychotherapy
(WHO, 1980), was later revised as the International factors included in this subscale include household com-
Classification of Functioning, Disability and Health position, romantic involvement; the number of relatives,
(ICF) (WHO, 2001). The domain of handicap was business associates, and friends with whom regular writ-
reconceptualized and changed to participation. The ten or oral contract is maintained, and the frequency of
migration away from the use of handicap toward the initiating conversations with strangers. C
more widespread use of participation is evident in Economic Self-Sufficiency is the individuals ability to
literature published since 2001. According to the WHO, sustain customary socioeconomic activity and indepen-
handicap (participation) describes the total effects and dence. This dimension is defined as the remaining dispos-
interplay of all the consequences of disability: social, able household family income after nonreimbursed
economic, cultural, and environmental. medical expenses have been excluded.
Each CHART dimension of handicap is characterized
by directly observable qualities which lend themselves to
easy quantification. While an infinite number of factors Psychometric Data
might have been included, to keep the instrument to a
practical length the following dimensions have been Initial calibration of the CHART scoring system was based
operationalized based on the WHO definitions. on administration of the instrument to 88 able-bodied
Physical Independence is the individuals ability to individuals and 100 persons with SCI. Once the norms
sustain a customarily effective independent existence. had been established, two studies were conducted to
The major component of this subscale is the number of assess the psychometric properties. CHART showed high
hours per day someone is needed to provide routine or testretest reliability 0.93 for the total score and from
occasional assistance (whether paid or unpaid). Indivi- 0.80 to 0.95 for the subscales. The correlation of subject
duals are viewed as somewhat less handicapped if they proxy scores was 0.83 for the total CHART score. Rasch
take primary responsibility for instructing and directing analysis established that CHART is a well-calibrated linear
people who are providing assistance to them. scale, with a good fit of both items and persons to its data
Cognitive Independence is the individuals ability to (Whiteneck et al., 1992).These studies established the
sustain a customary level of independence without the CHART as a reliable and valid instrument, as well as a
need for supervision. The factors included in this subscale well-calibrated linear scale (Whiteneck et al.; Dijkers,
reflect the amount of hours that a person needs supervi- 1991). A decade later, subsequent testing on a group of
sion both inside and outside the home, as well as the 236 persons with SCI reported similar results testretest
amount of difficulty an individual has in remembering, correlations of 0.87 and subjectproxy correlations of 0.85
communicating, and managing money. were reported (Whiteneck, Brooks, & Mellick, 1997).
Mobility is the individuals ability to move about The Revised CHART which included the Cognitive
effectively in his/her surroundings and is demonstrated Independence subscale was tested on 1,110 persons in
by the hours per day out of bed, days per week out of the six impairment categories SCI, traumatic brain injury,
house, nights per year spent away from home, accessibility stroke, multiple sclerosis, amputation, and burn
of the home, and transportation utilization. (Whiteneck , Brooks et al., 1997). Results indicated that
Occupation is the individuals ability to occupy time in the cognitive subscale of the CHART was reliable and
the manner customary to that persons sex, age, and enhanced the appropriateness of the CHART in assessing
culture. The time spent in various activities is used to handicap among persons having cognitive impairments
measure this dimension. The relative value society places (Mellick et al., 1999). Participant-proxy agreement across
on different activities is used to weight the time in each the six disability groups provided evidence in support of
category. Although there was a potential for subjective the inclusion of proxy data for persons with various types
bias based on value judgments in developing the scale in of disabilities (Cusick et al., 2001)
this dimension, priority has been give to gainful employ- In an effort to reduce the number of items in the
ment, schooling, and active homemaking and mainte- CHART, a short form was developed. A multidimensional
nance, and this prioritization has been supported by analysis was performed which showed that fewer variables
validity and reliability testing. Other elements documen- were needed to obtain CHART scores. Regression analyses
ted include volunteer work, recreational pursuits, and were performed on each subscale with the dependent mea-
self-improvement activities. sure being the scale score and the variables contributing to
Social Integration is the individuals ability to partici- the subscale acting as the predictor variables. All CHART
pate in and maintain customary social relationships. The subscale scores could be reduced by fewer questions to reach
730 C Craig Handicap Assessment and Reporting Technique (CHART) Short Form
90% explained variance except Economic Self-Sufficiency, Mellick, D., Walker, N., Brooks, C. A., & Whiteneck, G. (1999). Incorpor-
ating the cognitive independence domain into CHART. Journal of
which using the main variables could only explain 45%.
Rehabilitation Outcome Measures, 3(3), 1221.
For additional information about the development, Segal, M. E., & Schall, R. R. (1995). Assessing handicap of stroke
testing, and scoring procedures for the CHART and survivors. A validation study of the Craig handicap assessment and
CHART-SF please consult the Guide for use of the reporting technique. American Journal of Physical Medicine &
CHART: Craig Handicap Assessment and Reporting Rehabilitation, 74, 276286.
Walker, N., Mellick, D., Brooks, C. A., Whiteneck, G. G. (2003).
Technique at www.craighospital.org/Research/CHART.
Measuring participation across impairment groups using the Craig
handicap assessment and reporting technique. American Journal of
Physical Medicine and Rehabilitation, 82(12), 936941.
Clinical Uses Whiteneck, G., Brooks, C. A., Charlifue, S., Gerhart, K. A., Mellick, D.,
Overholser, D., et al. (1998). Guide for use of the CHART: Craig
handicap assessment and reporting technique. www.craighospital.
The CHART is a useful tool to measure handicap
org/Research/CHART December 29, 2009.
(participation limitations) in populations with injury or Whiteneck, G. G., Brooks, C. A., & Mellick, D. C. (1997). Handicap
chronic illness with or without rehabilitation interven- assessment Final report. Rehabilitation research and training center
tion. The CHART is designed as an interview tool, on functional assessment and evaluation of rehabilitation outcomes.
which can be administered face-to-face or by telephone. Buffalo, NY: State University of New York.
Whiteneck, G. G., Charlifue, S. W., Gerhart, K. A., Overhosler, J. D., &
Each item on the instrument has been carefully and con-
Richardson, G. N. (1992). Quantifying handicap: A new measure of
cisely worded to minimize ambiguity of interpretation. It long-term rehabilitation outcomes. Archives of Physical Medicine and
is possible to use the instrument as a mailed question- Rehabilitation, 73, 519526.
naire, although some valuable data potentially would be Whiteneck, G. G., Fougeyrolles, P., & Gerhart, K. A. (1997). Elaborating
lost in the absence of interaction with an interviewer the model of disablement. In M. Fuhrer (Ed.), Assessing medical
rehabilitation practices: The promise of outcomes research. Baltimore:
providing consistent prompts. There is no set time period
Paul H. Brooks Publishing Co.
for administering the CHART; however, it is recom- World Health Organization. (1980). International classification of impair-
mended that multiple measurements be taken over the ments, disabilities and handicaps: A manual of classification relating to
course of a persons lifetime to assess changes with adap- the consequences of disease. Geneva: World Health Organization.
tation to the disability and to gain insight into changes in World Health Organization. (2001). International classification of func-
tioning, disability and health. Geneva: World Health Organization.
participation which may occur over time. CHART has
been demonstrated to be a reliable measure of societal
participation limitations in a variety of populations in-
cluding people with physical and/or cognitive functional
limitations.
Craig Handicap Assessment and
Reporting Technique (CHART)
Cross References Short Form
CHART Short Form
CHART Short Form
Functional Deficits/symptoms
Number Name componenta Function of dysfunction
I Olfactory N. Special sensory Olfaction (smell) Hyposmia and anosmia
II Optic N. Special sensory Vision Anopsia
III Oculomotor N. Somatic motor Extraocular muscles (superior, inferior, and Diplopia and ptosis
medial rectus muscles)
Visceral motor Ciliary and pupillary constrictor muscles Pupil dilation
IV Trochlear N. Somatic motor Extraocular muscle (superior oblique muscle) Diplopia
V Trigeminal N. Somatic sensory Face, mouth/jaw, teeth, sinuses, meninges, Trigeminal neuralgia
oral and nasal mucosa
Branchial motor Muscles of mastication Asymmetric chewing
VI Abducens N. Somatic motor Extraocular muscle (lateral rectus muscle) Diplopia and medial
deviation of eye
VII Facial N. Special sensory Taste (anterior 2/3 of tongue) Loss of taste
Somatic sensory Skin on ear and tympanic membrane
Branchial motor Muscles of facial expression Facial paralysis and Bells
palsy
Visceral motor All salivary glands (excluding the parotid
gland) and lacrimal glands
VIII Vestibulocochlear N. Special sensory Audition and balance Deafness, tinnitus, and
vertigo
IX Glossopharyngeal N. Special sensory Taste (posterior 1/3 of tongue) Loss of taste
Somatic sensory Skin on ear, tympanic membrane, posterior
1/3 of tongue, and tonsillar fossa
Branchial motor Stylopharyngeus muscle
Visceral motor Parotid gland
Visceral sensory Carotid body and carotid sinus Blood pressure
regulation deficits
X Vagus N. Special sensory Taste (epiglottis) Loss of taste
General sensory Skin on external ear
Branchial motor Muscles of the larynx and pharynx Dysphagia and
dysphonia
Visceral motor Glands, smooth and cardiac muscle in the
neck, thorax, and abdomen
Visceral sensory Pharynx, larynx, and thoracic and abdominal
viscera
XI Spinal Accessory N. Somatic motor Trapezius and sternocleidomastoid muscles Shoulder drop and
weakened neck rotation
XII Hypoglossal N. Somatic motor Muscles of the tongue Tongue deviation
a
Alternative names for the functional components include following: General somatic efferent (GSE), somatic motor; general visceral efferent
(GVE), visceral motor; special visceral efferent (SVE), branchial motor; general somatic afferent (GSA), somatic sensory; general visceral afferent
(GVA), visceral sensory; special somatic afferent (SSA) and special visceral afferent (SVA), special sensory.
pharyngeal arches and the fact that branchial motor nu- expression (innervated by CN VII), the pharyngeal and
clei are located in distinct brainstem columns (immedi- laryngeal muscles (innervated by CNs IX, X and the
ately adjacent and lateral to the somatic motor nuclei). cranial portion of CN XI), and the muscles of mastication
Muscles of branchial origin include the muscles of facial (innervated by CN V).
Cranial Nerves C 733
Visceral motor fibers provide autonomic (parasympa- relating to olfaction (CN I), vision (CN II), audition (CN
thetic) innervation to the head, neck, thoracic, and ab- VIII), balance (CN VIII), and taste (CNs VII, IX, and X).
dominal viscera, where they control glandular secretions This special sensory designation is an all-encompassing
and smooth and cardiac muscle contraction. The motor classification in that it does not distinguish between the
neurons that regulate parasympathetic visceral motor somatic senses (vision, audition, and balance) and the C
processes are typically positioned immediately lateral to more visceral sensations (olfaction and taste).
the branchial motor nuclei column in the brainstem. Intracranial Courses. Cranial nerves must traverse
Visceral motor fibers in CN III transmit parasympathetic through foramina (small holes) within the skull in order
innervation to structures in the eye that regulate pupil to navigate the path between the brain and the various
constriction and lens accommodation. CN VII regulates structures to which they provide innervation. Often, cra-
the secretion of tears (via the lacrimal glands) and salivary nial nerves will travel through these foramina together in
gland secretions (along with CN IX). CN X conveys vis- groups as they exit the cranium. For example, CNs IX, X,
ceral motor innervation to glandular tissue, smooth and and XI pass through the jugular foramen on their descent
cardiac muscles of the gastrointestinal, pulmonary, and to structures in the neck, thoracic, and abdominal cav-
cardiovascular systems. CN X has the most extensive ities; while CNs III, IV, and VI all traverse the superior
distribution of the cranial nerves, with its innervation orbital fissure to enter into the orbit to innervate the
spanning structures within the head and neck down to extraocular muscles. Knowledge of the origins of cranial
the thoracic and abdominal regions. nerves (i.e., brain stem nuclei), their intracranial course
Sensory Cranial Nerves. The remaining three func- and the cranial foramina through which they pass is
tional classifications of cranial nerve fibers convey sensory crucial to any neurological exam, as the diagnosis of
information and include somatic sensory, visceral sen- dysfunction of specific nerves can help to pinpoint the
sory, and special sensory fibers. As previously mentioned, site of and provide valuable information about damage or
the brainstem nuclei associated with these sensory fibers injury to the brain. For example, one of the early symp-
are located more laterally in the brainstem relative to toms of a pituitary adenoma is impaired vision, which
motor nuclei and are arranged in longitudinal columns results from the close proximity between the pituitary
according to functional components (from lateral to me- gland and fibers of CN II, which can become compressed
dial: special sensory, somatic sensory, and visceral sensory as a result of the tumor bulk. Similarly, CN VI has a very
nuclei). Somatic sensory fibers carry information from long intracranial course and, due to its emergence near
exteroreceptors and proprioceptors in the skin, muscles, the bottom of the brain and its course through the cav-
tendons, and joints of the head and neck, mediating the ernous sinus, it is often the first cranial nerve to be
perception of pain, temperature, touch, and propriocep- affected in the case of elevated intracranial pressure, com-
tion. CN V is the major somatic sensory nerve of the head, mon symptoms of which include painful eye movement
mediating cutaneous and proprioceptive sensation from and blurred or double vision (diplopia). Unlike the ma-
the skin, muscles, and joints in the face, mouth, and jaw as jority of cranial nerves, which exit from the ventral surface
well as sensory innervation of the teeth, oral and nasal of the brainstem, CN IV exits the midbrain dorsally and
mucosa, sinuses, and meninges. CN IX also transmits wraps around the lateral surface of the brainstem to enter
somatic sensory information from a portion of the oral the orbit. Due to this long peripheral course around
mucosa (the posterior third of the tongue and tonsillar the brainstem, CN IV is particularly susceptible to head
fossa) and, together with CNs VII and X, mediates sensa- trauma, where damage to this nerve is manifested by
tion of the skin on the outer ear. diplopia or blurred vision. Such examples demonstrate
In contrast to somatic sensory fibers, visceral sensory the manner in which cranial nerve dysfunction can pro-
fibers receive sensory input from receptive endings in vide insight into the various pathological situations that
visceral structures, such as walls of blood vessels or of can occur in the brain.
the digestive tract. Congruent with its expansive distribu- Cranial Nerve Dysfunction. Cranial nerve dysfunction
tion, CN X mediates the majority of visceral sensation in is not uncommon and can result from a variety of under-
the pharynx, larynx, thoracic, and abdominal cavities. CN lying pathologies, ranging from brain trauma to various
IX transmits visceral sensory information from the carotid forms of neurological disease. Common cranial nerve
sinus and carotid body, important structures in the regula- dysfunctions/disorders include Trigeminal Neuralgia
tion of blood pressure and respiration. (CN V), Bells Palsy (CN VII), Ramsay Hunt Syndrome
The final category of sensory cranial nerve fibers is the (CN VII), acoustic neuroma (CN VIII), Glossopharyngeal
special sensory fibers, which convey sensory information Neuralgia (CN IX), eye movement-related cranial nerve
734 C Cranial Pneumocyst
Cross References
Synonyms
Acoustic Neuroma
Anosmia Decompressive craniectomy
Auditory Pathway
Auditory System
Autonomic Nervous System Definition
Bells Palsy
Deaf/Hearing Impairment Craniectomy or decompressive craniectomy is a surgical
Diplopia procedure in which a section of the skull is removed
Dysphagia and not immediately replaced (Hutchinson, Timofeev, &
Dysphonia Kirkpatrick, 2007; Aarabi, Hesdorffer, Ahn, Aresco,
Medulla Scalea, & Eisenberg, 2006). This procedure is most fre-
Midbrain quently used when increased intracranial pressure follow-
Neurologic Examination ing traumatic brain injury does not respond to other less
Olfaction aggressive interventions. Following brain trauma, the
Olfactory Bulb brain may expand within the skull. The resulting
Optic Nerve increased intracranial pressure can compromise brain
Optic Neuropathy function, particularly in the brain stem. Compression of
Pons the brain stem can compromise its basic life support
Ptosis functions, that is, cardiac and respiratory regulation, cre-
Pupillary Light Response ating a life-threatening situation. By removing part of the
Taste skull, the swelling brain is provided room to expand,
Tinnitus reducing intracranial pressure and pressure on the brain-
Trochlear Nerve stem. Although the section of the skull that is removed
Vestibulocochlear Nerve in a craniectomy is not immediately replaced, the bone
Visual Field Deficit removed may be stored and replaced at a later date when
Visual System brain swelling is reduced and stable. Artificial materials
Vertigo may also be used to replace the removed skull. In a
craniotomy, a section of the skull is removed and replaced
as part of the initial surgical procedure. Craniotomy is
more frequently performed as part of surgical interven-
References and Readings tion for disorders such as brain tumor or arteriovenous
malformation. However, a craniectomy may be preferred
Kandel, E. R., Schwartz, J. H., & Jessell, T. M. (2000). Principles of neural
in such cases if the condition appears to be associated with
science (4th ed.). New York: McGraw-Hill.
Shaw, J. P. (1992). A history of the enumeration of cranial nerves by brain swelling. Craniectomy may have no advantage over
European and British Anatomists from the time of Galen to 1895, craniotomy in long-term outcome after severe brain
with comments on nomenclature. Clinical Anatomy, 5(6), 466484. injury (Woertgen, Rothoerl, Schebesch, & Albert, 2006).
Cranioplasty C 735
Cross References C
Brain Swelling
Craniotomy
Aarabi, B., Hesdorffer, D., Ahn, E., Aresco, C., Scalea, T. M., &
Eisenberg, H. M. (2006). Outcome following decompressive craniect-
omy for malignant swelling due to severe head injury. Journal of
Neurosurgery, 104, 469479.
Hutchinson, P., Timofeev, I., & Kirkpatrick, P. (2007). Surgery for brain
edema. Neurosurgical Focus, 22(5), E14.
Jiang, J. Y., Xu, W., Li, W. P., Xu, W. H., Zhang, J., Bao, Y. H., et al. (2005).
Efficacy of standard trauma craniectomy for refractory intracranial
hypertension with severe traumatic brain injury: A multicenter, Craniopharyngioma. Figure 1 Courtesy Michael Fisher, MD,
prospective, randomized controlled study. Journal of Neurotrauma, Peter C. Phillips, MD. The Childrens Hospital of Philadelphia
22(6), 623628.
Woertgen, C., Rothoerl, R. D., Schebesch, K. M., & Albert, R. (2006).
Comparison of craniotomy and craniectomy in patients with acute References and Readings
subdural haematoma. Journal of Clinical Neuroscience, 13(7),
718721. Fahlbusch, R., Honegger, J., Paulus, W., Huk, W., & Buchfelder, M.
(1999). Surgical treatment of craniopharyngiomas: experience with
168 patients. Journal of Neurosurgery, 90, 237250.
Craniopharyngioma
Cranioplasty
E THAN M OITRA
Drexel University J ACINTA M C E LLIGOTT
Morgantown, WV, USA National Rehabilitation Hospital
Dun Laoghaire Co., Dublin, Ireland
Definition
Definition
Craniopharyngioma is a slow-growing, extra-axial,
epithelial-squamous, calcified cystic tumor. It occupies Cranioplasty or replacement of bone flap or prosthesis is a
the suprasellar/sellar region and shows benign histology surgical procedure usually performed to fill in, or replace
but malignant behavior, as it may invade surrounding a defect in the skull following a craniectomy or removal
areas and recur after the treatment (Fahlbusch, Honegger, of a bone flap (Fig. 1).
Paulus, Huk, & Buchfelder, 1999). Craniopharyngiomas
may develop embryogenetically, arising from remnants
of the craniopharyngeal duct and/or Rathke cleft or meta- Further Reading
plastically because of residual squamous epithelium. The
most common presenting symptoms are endocrine dys- Cranioplasty can be performed using the patients own
function, headache, and visual disturbances. Craniophar- bone flap, which has been frozen or stored in the patients
yngiomas are treated with surgery or surgery followed by abdomen, or multiple materials such as titanium mesh,
radiotherapy. hydoxyapatite, and polymethylmethacrylate can be used
736 C Craniospinal Irradiation
Craniospinal Irradiation
Craniospinal Radiotherapy
Craniospinal Radiotherapy
as alternative to create a cranioplasty flap, where necessary
computerized techniques may be used to generate a J ACQUELINE L. C UNNINGHAM
custom made cranioplasty (Long, 2007). Childrens Hospital of Philadelphia
The decision to perform and the timing of the cranio- Philadelphia, PA, USA
plasty to replace the skull defect following craniectomy
can be variable. Originally considered to be largely a
cosmetic rather than a therapeutic procedure, cranio- Synonyms
plasty may be now be performed to prevent late neuro-
logical complications associated and identified with Craniospinal irradiation; CSI
craniectomy including the so called syndrome of
trephined (Dujovny, Agner, & Aviles, 1999; Long,
2007). Characteristics of this syndrome include headache, Definition
dizziness, irritability, epilepsy, discomfort, and psychiatric
symptoms, and in addition, the skin overlying the skull Craniospinal radiotherapy is an irradiation that is
defect may become indented (Dujovny, Aviles, Fernandez, & directed at the whole brain and length of the spinal axis,
Charbel, 1997). The pathophysiology of the the including the meninges, as part of the cancer treatment to
syndrome of the trephined is unknown, however, a control malignant cells. It serves as a radical (curative)
number of factors have been implicated including antineoplastic therapy, as a prophylaxis against a neo-
atmospheric pressure, cerebral blood flow, and cerebro- plasms involvement with the central nervous system, or
spinal fluid changes (Dujovny et al., 1997). Neurological as a palliative recourse when cure is impossible. Craniosp-
and functional improvements have been shown to inal irradiation (CSI) is technically challenging, and is
improve following cranioplasty in this syndrome, for used with computed tomography (CT) simulation and
example, in 1945 Gardner described the improvement in multimodality MRI registration to define a large target
symptoms in some patients following cranioplasty with volume, which spares healthy tissues, and assures exact
tantalum (Dujovny et al., 1997; Gardner, 1945). reproducibility of treatment from day-to-day.
MRI evidence of the craniospinal radiation injury to
the brain has been seen in lHermittes sign (a side effect of
References and Readings
radiotherapy on the spinal cord, experienced as shock
sensations), telangiectasia (dilated capillaries), white mat-
Dujovny, M., Agner, C., & Aviles, A. (1999). Syndrome of the trephined:
Theory and facts. Critical Reviews in Neurosurgery: CR, 9(5), 271278.
ter changes, basal ganglia change, necrosis, and cerebral
Dujovny, M., Aviles, A., Fernandez, P., & Charbel, F. T. (1997). Cranio- atrophy. Although the differential sensitivity of specific
plasty: Cosmetic or therapeutic? Surgical Neurology, 47, 238241. brain regions to radiotherapy has not been determined,
Craniotomy C 737
factors relating to total dose, dose per fraction, and inter- Definition
val between fractions have been identified as important
variables influencing the brains response to radiation. Neurosurgical procedure involving the opening of the
Present research focuses on the development of treatment skull as a means of decreasing intracranial pressure
protocols based on the efficacy in tumor control while and/or for purposes of removal of a mass lesion. C
using the least dose of craniospinal radiotherapy
(1,800 cGy), often in conjunction with chemotherapy, as
the efficacy of CSI dose reduction in ameliorating neuro- Current Knowledge
endocrine and neurocognitive sequelae remains unclear.
Today, in contrast to the much higher doses used in the Craniotomy as a treatment for increased ICP from a mass
past decades, CSI doses of 2,400 and 3,600 cGy (with daily lesion has its foundation early in the history of neurosur-
fractions of 150 or 180 cGy) are standard. Studies contin- gery. Decompressive craniotomy (DC) initially was intro-
ue to evaluate how low a dose will remain effective in a duced to lower the intracranial pressure (ICP) in patients
risk-adapted setting. Controversy also exists on the with inoperable tumors and in managing uncontrolled
expression of radiation effects on specific neurocognitive ICP after traumatic brain injury (Brit & Hamilton,
domains. Attention and memory are known to bear a 1978). In recent years, DC has been recommended as an
vulnerability to neurotoxicity, but issues of individual alternative treatment for space occupying acute hemi-
differences, including premorbid and disease-related risk spheric infarction with or without massive medically
factors, are expected to influence neuropsychological uncontrolled brain edema (Schwab, 1998). During the
outcomes. acute period following cerebral infarction, neurologic
decline is often attributed to surrounding edema. Apart
from relieving the mass effect, restoration of the micro-
Cross References circulation around the infarcted area is the target of DC.
The management of increased intracranial pressure is a
Radiation Injury common clinical scenario in neurosurgery. Strategies for
Radiation Oncology the management of ICP fall into two general categories:
Radiotherapy to reduce the volume of the intracranial compartment
(medical management) and to remove the mechanical
constraints imposed by the cranial vault (surgical).
In patients who sustain a severe non-penetrating
References and Readings head injury, overall 2545% require a craniotomy for
evacuation of a hemorrhagic mass lesion, including epi-
Armstrong, C. L., Gyato, K., Awadalla, A. W., Lustig, R., & Tochner, Z. A.
(2004). A critical review of the clinical effects of therapeutic irradia-
dural, subdural, and intracerebral hematomas (Miller,
tion damage to the brain: The roots of controversy. Neuropsychology 1981). There is little debate in the surgical management
Review, 14, 6586. of a rapidly deteriorating patient with a focal neurological
Brady, L. W., Heilmann, H. P., Molls, M., & Schlegel, W. (2006). New deficit and neuroimaging findings of an expanding intra-
techniques in radiation oncology. New York: Springer.
cranial hematoma associated with significant mass effect
and midline shift. For less obvious situations controversy
remains given the lack of class I and II data to support any
treatment standard. Complete removal of a brain tumor
without inflicting neurological deficits is a desirable end
Craniotomy result in neurosurgical practice. Craniotomy was tailored
to encompass tumor plus adjacent areas presumed to
E DUARDO L OPEZ contain eloquent cortex. Magnetic brain stimulation or
Johnson Rehabilitation Institute intraoperative cortical stimulation can be used to guide
Edison, NJ, USA resection of functional cortex.
DC remains a controversial procedure in spite of a
number of studies published in the literature on its use
Synonyms in the treatment of intracranial hypertension secondary
to malignant cerebral edema, traumatic brain injury,
Craniectomy; Trephination aneurysmal subarachnoid hemorrhage, central venous
738 C Cretinism
thrombosis, encephalitis, intracerebral hematoma, and conditions are characterized pathologically by neuronal
metabolic encephalopathies. It has been shown to be loss, spongiform change, and astrocytic gliosis. Cell loss
effective in reduction of ICP refractory to medical therapy can be seen microscopically as multiple perforations to
but evidence supporting an improvement in clinical out- the brain tissue creating the characteristic sponge-like
come has not been conclusive. appearance. Prion diseases are caused by infectious
agents, which are abnormal self-replicating forms of a
normal brain protein, prion protein.
Cross References
Temporal Lobectomy
Categorization and Epidemiology
CreutzfeldtJakob Disease (CJD) may be sporadic (that is
References and Readings develop spontaneously without apparent cause), familial
(inherited), or acquired (transmitted by infection).
Brit, R., & Hamilton, R., (1978). Large decompressive craniotomy in the
treatment of acute subdural hematoma. Neurosurgery, 2(3), 195200.
CJD occurs worldwide with a mean annual incidence
Bullock, R., Chestnut, R., Ghajar, J., Gordon, D., Hartl, R., Newell, D. W., of approximately 12 cases per million population
et al. (2006). Surgical management of subdural hematoma. (Ladogana et al., 2005). Except for variant and iatrogenic
Neurosurgery, 58(3), S216S224. CJDs, which are in decline, the disease has a relatively
Chen, C., Smith, E., Ogilvy, C., Carter, B. S., (2006). Decompressive
stable incidence with no convincing evidence of geo-
craniectomy: Physiological rationale, clinical indications, and
surgical considerations. 5, 7080.
graphical clustering (although there are regions with an
increased incidence of familial cases). The gender inci-
dence is equal.
Historical Background
also possible for a person-to-person spread to occur, as has Variant CJD presents with a psychiatric syndrome,
been seen in four cases of blood transfusion-associated including depression and anxiety, for about 6 months
vCJD infection (most recent incidence figures are available before there is a progressive neurological and cognitive
from http://www.cjd.ed.ac.uk). decline as in sCJD, although chorea and dystonia occur as
well as myoclonus. The mean survival is 14 months, and C
vCJD affects a younger age group, with the mean age
Natural History and Prognosis being 29 years at death.
Approximate
duration of Early clinical
Form Age at onset illness features Neuropsychological findings Exceptions
Sporadic 90% of cases 4 months (65% of Neurological signs Areas of early cognitive deficit Rare cases occur
between 50 cases survive <6 including cerebellar vary in this heterogeneous younger than 50.
and 80 years, months from ataxia, cognitive group, but there is often marked Young cases may
mean 65 years symptom onset). impairment (global impairment by the time of a present with some
dementia or referral for testing. Most cases psychiatric
specific deficits in show rapid progression to a symptoms
earlier stages), wide-ranging dementia initially, similar to
followed by including verbal and nonverbal the vCJD
myoclonus, rigidity, memory, executive dysfunction, presentation, and
and rapid and nominal skills. Poor memory may have longer
deterioration to loss and/or attention are often early disease duration.
of speech, features, but there may be 19% sCJD cases
voluntary occasional cases where initial have a disease
movement, and deficits reflect dysphasia or visual duration >12
awareness. disturbance. months
Heidenhain form:
very focal visual
difficulties for
weeks/months
before other
cognitive
symptoms.
Look for: Rapid cognitive decline Brownell
over multiple test sessions. Oppenheimer
Fluctuations in responsiveness form: pure
and distractibility. cerebellar
syndrome for
Intrusion errors.
several weeks or
Verbal and motor perseveration. months before
cognitive decline
Familial Dependent on Dependent on the Dependent on fCJD cases may demonstrate less Some cases can
the specific specific mutation, mutation, may severe/rapid cognitive decline at have an illness
mutation of but on average present in a similar the early stages of a longer duration of years.
the prion 25 years fashion to sCJD or disease course than sCJD or vCJD Fatal Familial
protein gene, with predominant cases. A single case in 2000 Insomnia: early
but most often sleep and showed specific isolated deficits sleep disturbance
between the autonomic in delayed verbal memory and and autonomic
ages of 30 and disturbance or word finding prior to global dysfunction is
50 years. cerebellar ataxia involvement. One study suggests prominent
(see Exceptions, that naming ability may be
Gerstmann
right). Deterioration preserved in some cases in
Straussler
is usually slower comparison to sCJD and vCJD.
syndrome:
than in vCJD or Look for: Family history of CJD or
progressive
sCJD with a longer other (possibly misclassified)
cerebellar ataxia
disease course. neurological disease
Creutzfeldt-Jakob Disease C 741
Approximate
duration of Early clinical
Form Age at onset illness features Neuropsychological findings Exceptions
Iatrogenic Dependent on Following dura Human growth Dependent on mode of C
age at mater graft similar hormone infection: transmission; human growth
exposure. to sCJD, With progressive hormone patients present with
Incubation human pituitary cerebellar ataxia with relatively preserved
period hormones 1218 syndrome, delayed cognitive function until later
following months onset of dementia stages, while intracerebral cases
intracerebral Human dura mater present with broad-ranging
exposure is infection: rapidly dementia with rapid
1946 months, progressive deterioration as seen in sCJD.
extending to dementia, similar to Look for: Rapid cognitive decline
many years or sCJD over multiple test sessions for
decades with intracerebral exposure cases.
peripheral History of relevant exposure to
exposure. differentiate from sCJD.
Variant Median age 28 Median 14 Most commonly, Measurable impairments on tests In 15%,
(range 1274) months initial presentation of both verbal and nonverbal neurological
is psychiatric memory, executive function, symptoms
disturbance speed of attention, and nominal precede
including skills have characterized psychiatric.
depression, descriptions of published cases.
agitation, and Language, verbal reasoning, and
behavioral changes, visuoperceptual skills may be less
although in some frequently impaired, although
cases cognitive this may reflect an earlier disease
changes may be stage at the time of testing. One
the first sign of study suggests possible
abnormality. preserved ability in some
A delay of months components of visuoperception
is possible before in comparison to patients
distinct suffering from sCJD or fCJD.
neurological signs, Global involvement follows with
although cognitive rapid disease progression.
changes may be Look for: Fluctuating attention
found earlier in the and effort during testing.
disease course. Cognitive impairment more
Sensory symptoms profound than expected for
such as pain or odd depression or including areas of
sensation in limbs deficit unusual for psychiatric
or face may be disorders.
reported. Ataxia, Significant cognitive decline over
myoclonus, and follow-up assessment sessions.
significant
cognitive
impairment (such
as memory)
develop as the
disease progresses
Sources of information for this table are referenced under References and Readings.
742 C Creutzfeldt-Jakob Disease
Phenotype MRI EEG CSF 1433 Tonsil biopsy Blood test History
Sporadic Important in In 6080% of A positive 14-3-3 Negative At codon 129
(sCJD) excluding other cases, generalized CSF immunoassay of the prion
conditions. High bi- or triphasic is strongly protein gene:
signal on FLAIR or periodic sharp supportive of a 70%
DWI sequences in wave complexes diagnosis of sCJD methionine
the caudate and at 1/s. May not in the appropriate homozygous
putamen in about appear until later clinical context
70% of cases stages of the i.e., rapidly
disease progressive
dementia. Positive
in 90% of cases of
sCJD
Familial Some cases Characteristic Positive less Negative Analysis of Positive family
(fCJD) similar to sCJD periodic pattern is frequently than the prion history of CJD in
less frequently sCJD protein gene about 30% of
seen than in sCJD. for mutations cases
Iatrogenic Similar to sporadic Characteristic Positive in a Negative Mainly Relevant
CJD periodic pattern is proportion of homozygous exposure risk
less frequently cases methionine such as
seen than in sCJD. or valine treatment with
cadaveric
derived human
growth hormone
or human dura
mater graft
Variant Characteristic Normal or Positive in 50% of As vCJD, unlike All tested
(vCJD) high signal in the nonspecifically cases, but does the other CJD cases
posterior thalamic abnormal. not distinguish phenotypes, methionine
region (the Characteristic from sCJD involves the homozygous
pulvinar sign) in periodic pattern lymphoreticular
over 90% of cases in two cases late system, abnormal
on FLAIR or DWI in clinical course. protein may be
sequences. found in the
biopsy of tonsil
tissue in about
90% of cases.
Adapted from The National CreutzfeldtJakob Disease Surveillance Unit website (http://www.cjd.ed.ac.uk/investigations.htm).
indication of whether the degree or pattern of impairment professionals in supporting individuals and their families
seen in a patient could indicate CJD. through a period of acceptance, adjustment, loss, and
ultimately grief. Provision might also be made for the
counselling of healthcare staff involved in these distressing
Evaluation cases. C
Neuropsychological testing may occur prior to, in parallel
with, or following medical investigations and may provide
support for a diagnosis of CJD or prompt the clinician to
Cross References
instigate more extensive investigation than would usually
Prion Disease
be undertaken in patients presenting, for example, with
Spongiform Encephalopathy
primarily psychiatric complaints as in vCJD. A number of
further investigations may be used in the diagnosis of CJD
(Table 3).
References and Readings
Definition
Cross References
Crisis Intervention Approaches
Stress Management
Critical incident stress management (CISM; Mitchell &
Everly, 1993) is an approach to crisis intervention that has
garnered considerable attention since its initial develop- References and Readings
ment in the 1980s. A key component of CISM is the critical
incident stress debriefing (CISD), a group session held Mitchell, J. T., & Everly, G. S. (1993). Critical incident stress debriefing: An
shortly after an incident. Led by CISM-trained facilitators, operations manual for the prevention of traumatic stress among emer-
gency services and disaster workers. Ellicott City, MD: Chevron.
the CISD session allows participants to share their experi-
National Child Traumatic Stress Network and National Center for PTSD
ences of the incident, and the leaders provide psychoedu- (2006). Psychological first aid: Field operations guide, (2nd ed.). Re-
cation about stress reactions and recommended coping trieved March 5, 2010, from http://www.ncptsd.va.gov/ncmain/
strategies. In the 1990s, a controversy developed surround- ncdocs/manuals/PFA_V2.pdf.
ing CISM, as research evidence mounted that failed to National Institute of Mental Health (2002). Mental health and mass
violence: Evidence-based early psychological intervention for victims/
show its effectiveness. A National Institute of Mental
survivors of mass violence. A workshop to reach consensus on best
Health (NIMH) report (2002) suggested that stand-alone practices. NIH Publication No. 02-5138, Washington, D.C.: U.S.
CISD does not consistently prevent post-traumatic disor- Government Printing Office.
ders, and that some individuals, such as those with high Roberts, A. R. (2005). Crisis intervention handbook: Assessment, treatment,
arousal, may be put at heightened risk for adverse out- and research. (3rd. ed.). New York: Oxford University Press.
comes as a result of CISD-type interventions. Thus, man-
datory participation in group debriefing sessions is
considered very questionable.
An alternative to CISM is psychological first aid
(PFA; National Child Traumatic Stress Network and
Criterion Validity
National Center for PTSD, 2006). PFA is an evidence-
Test Validity
informed, pragmatic approach that targets acute stress reac-
tions and the immediate needs of persons exposed to a
critical incident (NIMH, 2002). The goals of PFA include
enhancement of safety (both objective and subjective), re-
duction of stress-related symptoms, restoration of rest and Criterion-Referenced Testing
sleep, linkage with resources, and facilitation of social
support. Domain Referenced Test Interpretation
Cross-Examination C 747
Direct Examination
CS-PFP10
References and Readings
Physical Functional Performance
Brodsky, S. L. (2004). Coping with cross-examination and other pathways
to effective testimony. Washington, DC: American Psychological
Association.
(1975). Federal rules of evidence for United States courts and magistrates.
St. Paul, MN: West Publishing.
Greiffenstein, M. F., & Cohen, L. (2005). Neuropsychology and the law: CT
Principles of productive attorney neuropsychologist relations. In
G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach. Category Test
New York: Oxford University Press.
Computed Tomography
CRS
CT Scan
Coma Recovery Scale
Conners Rating Scales Computed Tomography
Cue C 749
If correct response
(a) Say correct or right
(b) Present Cue #2
If incorrect response
(a) Present Cue #4
Cue. Figure 1 An example of a cueing hierarchy for oral naming using semantic and phonological cues
750 C Cue Dominance
to be dominant over color and to interfere with attention from free recall in that a cue or word is presented that is
to and naming of the actual color that is being presented related to the information being remembered. This aides
underlies the Stroop effect. Researchers and clinicians in the process of memory retrieval. Some examples of
studying or assessing attentional processes need to ac- cued recalls are the names of the categories in which
count for both the inherent and acquired cue dominance words were originally grouped or the presentation of C
of stimuli being used in the context of particular tasks. related words. For instance, in remembering the word
feather, the word bird may be used as a cued recall.
Cross References
Cued Recall
California Verbal Learning Test (California Verbal
M ARGARET M OULT Learning Test-II)
Institute of Living Hartford Cue
CT, USA Free Recall
Ivanoiu, A., Adam, S., Van der Linden, M., Salmon, E., Juillerat, A. C., Recent studies emphasize that the deconstruction of
Mulligan, et al. (2005). Memory evaluation with a new cued recall
race is critical for improved clinical care of ethnically di-
test in patients with mild cognitive impairment and Alzheimers
disease. Journal of Neurology, 252, 4755.
verse populations. The construct has been identified as a
Vogel, A., Morentsen, L., Gade, A., & Waldemar, G. (2007). The category proxy for more meaningful but complex variables such as
cued recall tests in very mild Alzheimers disease: Discriminative acculturation and indicators of quality of education, which
validity and correlation with semantic memory functions. European account for significant proportions of racial and ethnic
Journal of Neurology, 14, 102108.
differences in neuropsychological test scores (Romero
et al., 2009, p. 765). However, increasing emphasis has
been placed on the importance of the clinician knowing
when to apply race- and ethnic-based norms, as their use
can actually reduce detection of neuropsychological im-
Cultural Diversity in pairment. The complexity of this issue was demonstrated
Neuropsychology in one of the Mayos Older African American Normative
Studies (MOAANS) project studies. In this study, adjust-
S ARAH K. L AGEMAN ment of test scores for reading level surprisingly reduced
Emory University detection of cognitive impairment in African Americans.
Atlanta, GA, USA Lucas and colleagues (2005) suggest that their findings
indicate that cohort differences in reading level may be
more reflective of individual differences in cognitive abil-
Definition ity rather than contextual differences such as educational
backgrounds. While race may be a very efficient and
The term cultural diversity generally refers to the differ- parsimonious variable for clinicians to use at times, the
ences in defining cultural features that exist between confounding of causes and effects of these variables pre-
people (or within a given population), such as language, sents a significant challenge. The Diversity Summit parti-
dress, and traditions, as well as more abstract concepts cipants unanimously agreed that guidelines for
involving significant variations in societal organization neuropsychological practice among ethic and racial mino-
and interaction styles between individuals and environ- rities are needed to further improve clinical service for
ments. With respect to neuropsychology, the term ethic and racial minorities.
encompasses the racial and ethnic diversity among neu- In an effort to initiate the development of practical
ropsychologists themselves and the populations they in- guidelines regarding the use of demographic corrections,
teract with, as well as issues related to the influences of clinical criteria have been developed. As detailed in the
race and ethnicity on neuropsychological evaluations and Diversity Summit proceedings, demographic corrections
treatments. are useful to identify and characterize acquired neurocog-
nitive impairments in adults who (1) are natives of the
country of assessment, (2) developed normally, (3) received
Current Knowledge mainstream education, and (4) speak English as their first
language (for the U.S. norms). Demographic corrections
Multicultural competency is a developing area of focus are sometimes useful to identify and characterize acquired
within the field of neuropsychology. As the ethnic and neurocognitive impairments in (1) teenagers or young
racial diversity of the United States increases, so do efforts adults who have not completed their education, (2) adults
to provide neuropsychological services to ethnic minori- who may have had a mild developmental disorder, or (3)
ties. Thus, clinical and research studies have begun to anyone with a linguistic, cultural, or educational back-
examine how cultural variables influence performance ground not well represented in the normative subject
on neuropsychological measures. While the past decade sample. Demographic corrections are not recommended
has witnessed some publications providing test norms for when the clinician is asked (1) to characterize absolute
specific racial and ethnic groups, the use of racial and levels of functioning, (2) to identify or characterize possi-
ethnic norms for neuropsychological measures remains a ble acquired impairment in capital punishment cases or
contentious issue. Readers are encouraged to examine the when determining qualifications for special services,
summary of the 2008 Diversity Summit for additional or (3) in cases of possible acquired impairment in indivi-
details (Romero et al., 2009). Highlights of the summit duals who have developmental disability, (4) to character-
and recent literature are provided below. ize acquired cognitive impairment in individuals who
Cultural Diversity in Neuropsychology C 753
have major background differences from the normative importance of training in cross-cultural neuropsychology
sample, (5) to predict future performance in employment will become increasingly important as the Spanish-
or academic settings, and (6) for employment selection speaking Latino population in the United States grows.
decisions. In addition to these clinical guidelines, empha- In keeping with the demographic changes in the
sis is being increasingly placed on correctly using proba- United States, training programs and national organiza- C
bilistic statistics to predict group membership (i.e., tions are brainstorming ways to promote recruitment and
diagnosis) for individuals. Readers are encouraged to retention of students with diverse racial and ethnic back-
review recent publications regarding likelihood ratios, grounds in the study of neuropsychology. Emphasis has
which express the risk associated with certain test scores been placed on student exposure to neuropsychology
(Smith, Ivnik, & Lucas, 2008). earlier in academic careers, mentoring with minority
While additional study of the deconstruction of race is and nonminority faculty who are sensitive to diversity
needed, development of ethnic group norms itself is a issues and financial support of minority students. The
challenging and complex task. Test publishers are increas- study of cultural diversity itself has gained support and
ingly making efforts to include important background many licensing bureaus require documentation of diver-
variables in normative samples. However, the cost of sity training in graduate school for licensure. Numerous
recruiting and assessing large cohorts is prohibitive for organizations, including the American Psychological
individual centers and researchers and is often even a Association (APA) Division 40, the International Neuro-
challenge for larger test publishing companies. While psychological Society (INS), and the National Academy of
inclusion of demographic variables in research articles is Neuropsychology (NAN), promote awareness of diversity
an important piece to further develop this area, federal issues and foster collaborations among clinicians and
and foundation-funded grants may be necessary to sup- researchers interested in diversity issues. Readers are
port multicenter partnerships, ideally suited to study encouraged to review current guidelines, including the
large, diverse cohorts. APA Ethical Principles (2002) and the APA Multicultural
Despite the development of racially and ethnically Guidelines (2002) for further details.
diverse norms, including the widely used Heaton norms Diversity in neuropsychology has become of increasing
for African American and Caucasian adults, the almost interest and importance; however, measures to improve
endless variety possible with regard to individuals language, diagnostic assessment of ethnic minorities may not fully
culture, and education backgrounds limits the application address the issue of improving clinical care of ethnic mino-
of demographically corrected norms for every possible clin- rities, given limited health care access of non-English-
ical situation (Heaton, Miller, Taylor, & Grant, 2004). Local speaking patients. Access to care and other factors affecting
norms may be helpful for clinicians working with popu- ethnic minority patient outcomes have increasingly been
lation groups with less common background variables; examined in the field of neuropsychology. Readers are
however, integration of test scores with a persons individ- encouraged to review two issues of the Journal of Head
ual context is ultimately critical for formulation of an Trauma Rehabilitation, dedicated to U.S. and international
accurate diagnosis. In clinical practice, other issues re- cultural issues in the rehabilitation of survivors of traumatic
garding assessment of ethnic, linguistic, and cultural head injury (Cultural Issues, 2007 and International
minorities are also present. The use of translated tests, Programs and Perspectives, 2007). Articles in these issues
interpreters, and bilingual psychometrists are all current highlight complex relationships between functional out-
methods for evaluating non-English-speaking patients. comes, disability severity, return to work, and utilization
However, the shear complexity of language fluency is of professional psychological services in ethnic minority
important to consider. Language fluency of an individual groups. The international issue describes models of care
can vary widely with regard to (1) age of acquisition, (2) used in the context of various health care systems as well
proficiency, (3) manner of learning, (4) amount of lan- as international perspectives on research and develop-
guage exposure, (5) predominant mode of bilingual inter- ment in the field of traumatic brain injury rehabilitation.
action, and (6) language structure variables (i.e., alphabetic The study of health disparities among ethnic minority
versus logographic languages and phonemic versus non- populations is a complex, but critical field of research.
phonemic status). Currently, the use of translated tests and Multiple dimensions of disparity exist, including many of
official interpreters or bilingual psychometrists are pre- the variables discussed regarding the deconstruction of
ferred over using family members as interpreters and site race such as ethnicity, education, income, occupation,
translation, which refers to the practice of quickly translat- and geography. Continuous study of these variables is
ing a test with no standardization procedures. The critical to improve clinical care for ethnic minorities.
754 C Cultural Sensitivity
European-American culture. This document states that Heaton, R. K., Miller, S. W., Taylor, M. J., & Grant, I. (2004). Revised
comprehensive norms for an expanded Halstead-Reitan Battery: demo-
all individuals are in a social, political, and economic
graphically adjusted neuropsychological norms for African American and
context. Therefore, it is increasingly important for psy- Caucasian Adults. Lutz, FL: Psychological Assessment Resources Inc.
chologists to be aware of the specific needs of individuals Nagra, A., Skeel, R. L., & Sbrage, T. P. (2007). A pilot investigation on the
based on their ethnic/racial heritage and social group effects of stress on neuropsychological performance in Asian-Indians
C
identity. Guidelines relating to research direct psycho- in the United States. Cultural Diversity & Ethnic Minority Issues, 13,
5463.
logists to recognize the importance of conducting cul-
Smith, G. E., Ivnik, R. J., & Jucan, J. (2008). Assessment techniques:
ture-centered and ethical psychological research among tests, test batteries, norms and methodological approaches. In
persons from ethnic, linguistic, and racial minority J. E. Morgan, & J. H. Ricker (Eds.), Textbook of clinical neuropsycho-
backgrounds. logy (pp. 3857). New York: Taylor & Francis.
A second area of cultural sensitivity that directly
relates to neuropsychology is the impact of culture on
testing and on normative data. It is important that an
individual examinees test performance be related to indi-
viduals of similar backgrounds. For example, the norma- Culturally Reduced Test
tive group of an elderly, well-educated African American
man should closely match his background. Without close Culture Fair Test
matching of cultural, demographic, and educational
background, unacceptable problems with misclassifica-
tion of normal test results as abnormal can result in
misdiagnosis of the patient. There is evidence that exam-
iners belonging to a culture different from that of the
examinee can elicit some anxiety, which may have a dele-
Culture Fair Test
terious effect on test scores on more demanding cognitive
G LEN E. G ETZ
tasks (Nagra, Skeel, & Sbrage, 2007). Additionally, cultural
Allegheny General Hospital
background, demographics and educational history, can
Pittsburgh, PA, USA
have a sizeable impact on normative data. Smith, Ivnik
and Jucan (2008) discuss the development of norms spe-
cifically for older adults on commonly used neuropsy-
Synonyms
chological tests. They subsequently developed more
specific norms for older African Americans, as it was
Culture free test; Culturally reduced test
found that culture had a significant impact on normative
data. Heaton, Miller, Taylor and Grant (2004) have pub-
lished norms that take into account age, education, and
cultural background. This area of cultural sensitivity plays Definition
an important role in neuropsychology in terms of diag-
nosis and test evaluation. Culture fair test is a test that is equally fair to all cultural
groups. Fairness is related to a lack of bias in the inter-
pretation or use of a test to classify or diagnose. In a
Cross References culture fair test, the validity of the interpretation is
similar across different cultural groups. It is unlikely
Cultural Diversity that any test can entirely eliminate the influence of
Normative Data learning and cultural experience, given that the test con-
tent, language, directions, and validity criteria are cul-
turally bound. However, avoiding culturally loaded
References and Readings items, items that are found to be unfair to certain groups
of people, increases the likelihood of it being a culturally
American Psychological Association. (2002). Ethical principals of psychol-
fair test. Culturally loaded items, such as those that
ogists and code of conduct.
American Psychological Association. (2002). Guidelines on multicultural
utilize pictures or general information that are differen-
education, training, research, practice and organizational change for tially prevalent for certain cultures, decrease the likeli-
psychologists. hood of a culturally fair test.
756 C Culture Free Test
Synonyms
Illness
Broadmann area 17
Damage to the cuneus would typically occur among
patients experiencing focal brain lesions. Most often
such lesions occur secondary to cerebral infarction
Structure (stroke) typically involving the posterior cerebral circula-
tion, though neurosurgery to remove neoplasm may re-
The term cuneus comes from the Latin term for wedge, sult in focal damage to the cuneus as well. Some atypical
which reflects the shape of this occipital brain area. The neurodegenerative diseases are also known to cause tissue
cuneus is a wedge-shaped cortical area located in the loss in this occipital area. The effects of focal damage to
medial occipital gyri, superior to the calcarine fissure the cuneus are known primarily from experimental abla-
and posterior to the parietaloccipital fissure. The cuneus tion in primate studies and to some extent from single-
Cuneus C 757
tral
Cen
us
sulc Paracentr
al
eu
s lobule Su
n p fr
cu ont
ae al g
Pr Cing
ulate yru
s
C
Pa Cingulate gyrus sul
cus
ri
eto
-oc
m
callosu
Cuneus Corpus
cip
.
nix
For
Fi
Fissure
ss
ur
ine
y
Isthmus tor
fac
r
rol
lca
Lingual gyrus Pa ea
Ca
Hippo Uncus a r
camp
al gyr
Fusif us
Infr orm
Inf. T temp. gyrus
emp.
Gyru
s sulcus
Cuneus. Figure 1 Sagital illutration showing the cuneus in the posterior cortex
Etiology
CRH
Exogenous CS refers to iatrogenic CS resulting from chron-
ic GC therapy. GC, which have potent anti-inflammatory Pituitary
and immunologic actions, are widely used for the treat-
ment of various diseases such as inflammatory bowel
disease, asthma, rheumatoid arthritis, and organ transplan- ACTH
tation. Thus, exogenous CS is the most common form of
CS. Endogenous CS may be caused by dysregulation at
Adrenal gland
various levels of the hypothalamicpituitaryadrenal axis
(HPA), resulting in cortisol overproduction. The regulation
of cortisol synthesis and secretion is mediated by the hypo- Cortisol
thalamus, the pituitary, and the adrenal glands. Corticotro-
pin (ACTH) is synthesized and secreted by corticotrophs of Cushings Syndrome. Figure 1 Schematic representation of
the pituitary which are mainly regulated by two hypotha- the hypothalamuspituitary axis. Normally cortisol negatively
lamic hormones, corticotropin-releasing hormone (CRH), regulates corticotropin-releasing hormone (CRH) and ACTH
and arginine-vasopressin. Pituitary ACTH is secreted in the secretion
Cushings Syndrome C 759
Cushings Syndrome. Table 1 Classification of the etiologies and cognition, has increased considerably in the recent
of endogenous Cushings syndrome (CS) years. McEwen, Weiss, and Schwartz (1968) demonstrated
the presence of glucocorticoid receptors (GR) in the rat
ACTH-dependent CS (80% of cases)
brain. Loss of brain volume was documented in patients
Pituitary adenoma (Cushings disease) (80%)
with endogenous and exogenous CS (Bourdeau et al., C
Ectopic ACTH or corticotropin-releasing hormone (CRH)
2002). In addition, cerebral magnetic resonance spectros-
secretion from nonpituitary neoplasms (20%)
copy revealed abnormalities of cerebral metabolism in
ACTH-independent CS (20% of cases) patients with CS (Khiat et al., 1999).
Adrenal adenoma (60%) Psychiatric disturbances occur in patients with both
Adrenal carcinoma (40%) exogenous and endogenous CS. Interestingly, patients
Bilateral ACTH-independent macronodular adrenal with endogenous depression frequently have high corti-
hyperplasia (rare) sol levels, and present HPA axis dysregulation, disclosed
Bilateral micronodular adrenal hyperplasia (primary by abnormal suppression on the dexamethasone test.
pigmented nodular adrenocortical disease and However, diurnal rhythm is usually maintained in de-
nonpigmented adrenal hyperplasia) (rare) pressed patients, and they do not develop physical signs
of CS. CS is also associated with an increased prevalence
of cognitive impairments, particularly of attention,
learning, and memory. Patients with CS represent a
identified. During the investigation, it is important to natural model that illustrates the interactions between
evaluate patients for other illnesses, drugs, alcohol, or hypercortisolism and brain function. The following sec-
depression. All these conditions may lead to misinterpre- tion focuses on the psychiatric and neuropsychological
tation of the endocrine test results. The overnight 1-mg anomalies in CS.
dexamethasone suppression test, a screening tool for the
diagnosis of CS, consists of administering 1 mg of dexa-
methasone at bedtime (23:00 h) with measurement of Psychiatric Disorders Associated with CS
plasma cortisol the following morning (08:0009:00 h).
Normal subjects should suppress plasma cortisol to less Depression is a frequent feature of CS. In fact, several
than 50 nmol/L. Measurement of free cortisol in 24- reports indicate that more than 50% of CS patients pres-
h urine collection is useful to confirm the diagnosis; ent severe depressive symptoms that reach the threshold
values fourfold greater than the upper limit of normal of a major depressive disorder. Haskett (1985) observed a
are highly suggestive of CS. More recently, late-night lifetime history of psychiatric symptoms and signs in
salivary cortisol has proven useful in the diagnosis of CS patients with proven CS. He found that the majority of
(Nieman et al., 2008). Once a diagnosis of CS is con- them experienced psychiatric disturbances that closely
firmed, the next step is to establish the etiology. Plasma resembled typical syndromes, with episodes of major
ACTH will distinguish between ACTH-dependent and - affective disorder (endogenous depression, mania, or
independent CS. ACTH values lower than 1.1 pmol/L hypomania). Moreover, Haskett (1985) noted that there
indicate ACTH-independent CS, and CT imaging of the were no significant differences between pituitary-
adrenal glands should be performed (Newell-Price et al., dependent and -independent forms of CS in the occur-
2006). ACTH values above 3.3 pmol/L suggest ACTH- rence of major depression. His observations were later
dependent CS, and further endocrine investigations confirmed by independent investigations (Kelly, 1996;
should be undertaken, including magnetic resonance im- Sonino, Fava, Belluardo, Girelli, & Boscaro, 1993). Using
aging of the pituitary glands and other tests that are DSM-III criteria (American Psychiatric Association,
beyond the scope of this chapter. 1980), Hudson, Hudson, Griffing, Melby, and Pope
(1987) reported a high lifetime diagnosis of mood
disorders in CS patients, of whom 14% were assessed to
Psychiatric Diseases and have major affective disorder. Loosen, Chambliss, DeBold,
Neuropsychological Disturbances Shelton, and Orth (1992) recognized major depressive
Associated with CS disorder in 68% of adult patients with CS. In addition,
this disorder was frequently associated with anxiety
Understanding the importance and complexity of adrenal disorder (generalized anxiety disorder and/or panic dis-
hormones impacting cerebral function, particularly affect order), indicating a syndrome of anxious depression in
760 C Cushings Syndrome
patients with active CS (Loosen et al., 1992). Focusing on reported generalized impairment of cognitive functions
Cushings disease, Sonino, Fava, Raffi, Boscaro, and Fallo (decreased concentration and memory, perceptual distor-
(1998) found major depression in more than half of their tions). More recently, Starkman, Gebarski, Berent, and
patients studied. Moreover, major depression was signifi- Schteingart (1992) observed moderate-to-severe deficits
cantly correlated with older age, female gender, higher in a wide variety of language and nonverbal subtests
pretreatment urinary cortisol levels, and more severe among more than two-thirds of their CS patients.
clinical conditions. Kelly, Kelly, and Faragher (1996) Difficulties with reasoning ability, comprehension, and
reported that some patients with CS were diagnosed to processing of new information also were found. Deficits
have depression and were more depressed than patients in these areas of cognition were confirmed in a recent
with other pituitary tumors. Concentrating on the controlled study of 19 CS patients (Forget, Lacroix,
question of whether depression in CS more consistently Somma, & Cohen, 2000). In fact, memory was the most
resembles any of the clinical subcategories, Dorn et al. studied function in CS because the emphasis was on the
(1995) established that patients with active endogenous hippocampus which is rich in GR. However, the distribu-
CS exhibited significant psychopathology, expressed pri- tion of GR in many areas of the cerebral cortex suggests
marily by atypical depression. This concept of atypical that extrahippocampic sites could also be the target of
depression refers to the presence of weight gain or cortisol, and generalized impairment of cognitive func-
increased appetite, fatigue, hypersomnia, leaden paralysis, tions after chronic hypercortisolism may be related to this
and longstanding interpersonal rejection sensitivity wide dispersion of GR.
(American Psychiatric Association, 1994).
In summary:
Treatment
1. More than 50% of patients with CS report severe
depressive symptoms
The goal of CS treatment is to correct the hypersecretion
2. There are no significant differences between ACTH-
of adrenal hormones. In most cases, tumor-specific
dependent and -independent forms of CS in the
surgery will be performed. In Cushings disease, pituitary
occurrence of major depression
tumors should be resected, and in adrenal CS adrenalec-
3. Major depressive disorders are commonly associated
tomy performed. In cases of ectopic ACTH secretion
with anxiety disorders
secondary to benign tumors, the latter should be
4. Major depression is significantly correlated with
removed; but in most cases, there are metastatic malig-
older age, female gender, higher pretreatment urinary
nant tumors. If surgery is unsuccessful, drugs that block
cortisol levels, and more severe clinical conditions
steroid synthesis may be administrated to achieve
5. A high prevalence of atypical depression features is
eucortisolism (Biller et al., 2008; Stewart, 2008).
seen in CS
Neuropsychological disorders have been detected in about The reversibility of brain volume loss after the correction
two-thirds of patients with CS. The first such investiga- of hypercortisolism in patients affected by CS was previ-
tion evaluated unselected CS patients on the Michigan ously demonstrated (Bourdeau et al., 2002). Similarly,
Neuropsychological Test Battery (Whelan Schteingart, treatment of hypercortisolism is often efficacious in
Starkman, & Smith, 1980). This test battery included decreasing the depressive components of illness. Signifi-
standardized and objective measures with a broad range cant improvement in depressive symptoms was observed
of discrete language, verbal and nonverbal reasoning, when patients with CS were assessed by psychometric
auditory and visual memory, and sensory and motor methods before and after the treatment of their endo-
functions. The study revealed diffuse bilateral cerebral crine condition, (Dorn et al., 1997; Forget, Lacroix, &
dysfunction in CS with impairment in nonverbal, visual Cohen, 2002; Kelly et al., 1996; Starkman, Giordani,
ideational, visual-memory, and spatialconstructional Gebarski, & Schteingart, 2006; Starkman, Schteingart, &
abilities. Starkman, Schteingart, and Schork (1981) Schork, 1986). In a longitudinal study of depressive and
Cushings Syndrome C 761
Rubin, S., & Roessler, R. (2008). Foundations of the vocational rehabilita- to assess the disorder must be reliably able to measure the
tion process. Austin, TX: Pro-Ed.
particular abilities, taking into consideration the span
Szymanski, E. M., & Parker, R. M. (2003). Work and disability: Issues and
strategies in career development and job placement (2nd ed.). Austin,
of the standard error, to place an education-altering
TX: Pro-Ed. label on that child. If such standards are not followed,
Wagner, C. C., Armstrong, A. J., Fraser, R. T., Vandergoot, D., & Thomas, then clinicians run the risk of basing a decision on a C
D. F. (2006). Evidence-based employment practices in vocational false-positive (the discovery of a limitation when none
rehabilitation. In K. J. Hagglund & A. W. Heinemann (Eds.), Applied
exists in actuality) or a false-negative (the missing of
disability and rehabilitation research. New York: Springer.
Wehman, P., Inge, K. J., Revell, G., & Brooke, V. (2007). Real work for real
a limitation when one truly exists).
pay: Inclusive employment for people with disabilities. Baltimore, MD: To consider an example, a cutoff score may be the
Paul H. Brookes. score differentiating between intact performance and im-
pairment on a particular test. To speak in terms of diag-
nosing cognitive impairment, in a normal distribution of
scores, a cutoff score would be any score below 95% which
is obtained by the intact group within a particular stan-
Cut Scores dardization sample.
Clinicians may adjust cutoff scores because of individ-
Cut Off Scores, Cutting Scores ual or cultural variables that render the reliability of a
test nonapplicable to the individual or sample being
assessed. Otherwise, the clinician could run the risk of
pathologizing an individual who does not truly exhibit
the pathology.
Synonyms
Cut scores
CVA
Definition Stroke
CVLT
focus was on the relation between the brain and lan- Short Biography
guage, but his interests expanded to include memory,
perception, and autism in the 1980s. By the late 1980s, Antonio Damasio was born in Lisbon, Portugal in 1944.
his research focused on how brain injuries can teach He attended the University of Lisbon Medical School,
about how concepts and categories are stored in the and received his M.D. in 1969 followed by his Ph.D. in
brain. He proposed a neural architecture for learning 1974 from the University of Lisbon. In 1967, he studied
and recall based on convergence/divergence of signals with Norman Geschwind at the Aphasia Research
from critical nodes called convergencedivergence Center (Boston, MA) and later returned to America in
zones. Throughout his career, he has focused primar- 1975 when he became an Assistant Professor at the
ily on the study of how brain damage affects psycho- University of Iowa, earning the distinction of Full
logical functioning, thereby inferring how the healthy Professor in 1980. His association with the Salk Institute
brain operates. began in the 1980s. He remained at the University of
Damasios largest influence has been his theories on Iowa until 2005 when he moved to the University of
emotion and decision-making. Before 1995, the Southern California as the director of the Brain and
study of judgment and decision-making rarely or Creativity Institute, and Professor of Psychology,
tangentially considered how emotion could influ- Neuroscience, and Neurology.
ence behavior. In Descartes Error, Damasio advanced
the hypothesis that not only could emotion aid
reason in decision-making, but reason itself evolved
Cross References
from emotion and the two remain critically
interconnected. Central to Damasios theory is the
Geschwind, Norman (19261984)
Somatic Marker Hypothesis, which proposes that,
when making decisions, options are marked either
negatively or positively, consciously or unconscious-
ly, thus deterring an individual from making a nega- References and Readings
tive choice or, on the contrary, promoting a positive
choice. Damasio, A. R. (1989a). Time-locked multiregional retroactivation:
In the years since this revolutionary idea, numerous A systems level proposal for the neural substrates of recall and
recognition. Cognition, 33, 2562.
studies have lent support to the idea that emotion and Damasio, A. R. (1989b). The brain binds entities and events by multire-
reason are interdependent. These studies have includ- gional activation from convergence zones. Neural Computation, 1,
ed patients with ventromedial prefrontal damage who 123132.
are unable to monitor visceral states and, as a result, Damasio, A. R. (1996). The somatic marker hypothesis and the possible
functions of the prefrontal cortex. Transactions of the Royal Society
make poor decisions. The Somatic Marker Hypothesis,
(London), 351, 14131420.
however, has not been immune to criticism. Recently, Damasio, A. R. (1999, 2000). The feeling of what happens: Body and
these criticisms have focused on the extensive reliance emotion in the making of consciousness. New York: Harcourt.
of the hypothesis on the Iowa Gambling Task and Damasio, A. R. (2003). Looking for Spinoza: Joy, sorrow and the feeling
sometimes-conflicting evidence from other labora- brain. New York: Harcourt.
Damasio, A. R. (2005). Descartes error: 10th anniversary edition, with a
tories regarding the mediational role of the ventrome-
new author preface. New York: Penguin Books.
dial prefrontal cortices. Damasio, A. R., Grabowski, T. J., Bechara, A., Damasio, H., Ponto, L. L.
Since the publication of Descartes Error, Damasio has B., Parvizi, J., et al. (2000). Subcortical and cortical brain activity
extended his work on emotion to include research and during the feeling of self-generated emotions. Nature Neuroscience,
philosophy on the nature of feelings, morality, and 3, 10491056.
Damasio, A. R., & Meyer, K. (2008a). Behind the looking-glass. Nature,
consciousness. He has proposed that, in addition to
454, 167168.
mapping representations of the external world and Damasio, A. R., & Meyer, K. (2008b). Consciousness: An overview of the
internal states, regions such as the posterior cingulate phenomenon and of its possible neural basis. In S. Laureys &
cortex and related cortices (the postero-medial corti- G. Tononi (Eds.), The neurology of consciousness. Oxford: Elsevier.
ces) create a second-order representation which Dunn, B. D., Dalgleish, T., & Lawrence, A. D. (2006). The somatic marker
hypothesis: A critical evaluation. Neuroscience and Biobehavioral
allows an organism to develop a sense of self. These
Reviews, 30, 239271.
studies have begun to form a bridge between neuro- Gluck, A. (2007). Damasios error and descartes truth: An inquiry into
psychology and philosophy, posing testable hypoth- consciousness, metaphysics, and epistemology. Scranton, PA: Universi-
eses regarding metacognition. ty of Scranton Press.
Daubert v. Merrell Dow Pharmaceuticals (1993) D 769
Major Honors and Awards De Renzis initial studies on aphasia were conducted in
collaboration with Luigi A. Vignolo and took Gesch-
President of the European Brain and Behavior Society winds model into consideration. Vignolo had com-
(19731974) pleted a thesis on aphasia and had considerable
Carlo Riquier Award (University of Pavia, 1988) training in language and its disorders. De Renzi and
Honorary Degree in Psychology (University La Vignolo garnered the assistance of a local mime group
Sapienza of Rome, 1993) to study the potential role of nonverbal information in D
Member of the Academia Europea (1994) the aphasics comprehension deficit (De Renzi, 1998).
Honorary Member of the American Neurological The group presented short sketches miming everyday
Association (1995) actions (e.g., hammering a nail). In support of their
Honorary Member of the Academy of Neurology original impairment hypothesis, De Renzi and Vig-
(1995) nolo found that aphasics demonstrated significant
Honorary Member of the British Neuropsychological comprehension deficits. For the Seventh International
Society (1996) Congress of Neurology, they submitted a presentation
Professor Emeritus (University of Modena, 2002) about the aphasics impairment hypothesis including
Member by Election, Academy of Aphasia their recent findings about comprehension dysfunc-
Member of Research Group on Aphasia of the World tion. Arthur L. Benton reviewed their manuscript,
Federation of Neurology providing encouragement and prompting them to
Member of International Neuropsychological Symposia contemplate the behavior of normal subjects. This
first contact with Benton soon evolved into a lifelong
mentorship and friendship for De Renzi. Benton not
Landmark Clinical, Scientific, and only shared knowledge and presented lectures, but
Professional Contributions also pushed De Renzi to develop strong methodologi-
cal discipline during his time in Iowa City as a Fullb-
Ennio De Renzis fame stems from his heartfelt devo- right Professor. Bentons influence became evident
tion to establishing neuropsychology as a scientific during the 1960s and 1970s when De Renzi began to
discipline in Italy. One of the most influential postwar shift his focus from examining small groups of select-
neuropsychologists (Milner, 1997), De Renzi contrib- ed patients to studying unselected clinical cases.
uted greatly to the understanding of a broad range of Vignolo and De Renzi, together with other members of
neurological phenomena, including aphasia, apraxia, an interdisciplinary group which originally included
agnosia, topographical amnesia, and disorders of lan- such well-known scientists as Henry Hecaen, Hans Teu-
guage, spatial cognition, and memory. His seminal ber, and Clemens Faust, met to discuss various neuro-
works in these areas, particularly hemispheric special- logical phenomena, namely aphasia, constructional
ization and neuropsychological deficits in patients apraxia, alexia, cerebral dominance, effects of unilateral
with localized brain lesions, were ahead of his time. versus bilateral lesions, and disorders of time sense and
His insatiable exploration into unchartered neurolog- body schema. In 1962, the efforts of De Renzi, Vignolo,
ical and psychological territories spanned more than Brenda Milner, Klaus Poeck, and Oliver Zangwill
40 years and prompted further neuropsychological renewed the group that formally adopted the name of
research. the International Neuropsychological Symposium
During his early years studying neurology and psychi- (Stringer & Cooley, 2002). Also in 1962, De Renzi
atry, De Renzi worked closely with Professor Gildo and Vignolo collaborated on a paper about the
Gastaldi from the University of Pavia at two psychiat- Token Test, which has been one of De Renzis most
ric and two neurological wards. Gastaldi ultimately influential works.
asked De Renzi to join his group in the Department of During the early 1960s, establishing research proto-
Nervous and Mental Disease at the University of cols was challenging in light of scarce grant funding.
Modena. A few years later when Gastaldi accepted a De Renzi put forth great effort to assemble a group of
new position and moved to Milan to become the colleagues willing to devote countless hours in ex-
Chair of Nervous and Mental Disease, De Renzi fol- change for name recognition on papers. This group
lowed in Gastaldis footsteps, subsequently deciding came to be known as the Milan Group and consisted
to put aside his interest in psychiatry and devote of Vignolo, Pietro Faglioni, Hans Spinnler, and Anna
himself entirely to neurology. Basso. Piero Faglioni, an Italian neurologist with a
772 D De Renzi, Ennio (1924 )
passion and expertise for statistical analysis, played a 1969, De Renzi was called to hold the Chair of Ner-
principal role in influencing De Renzis approach to vous and Mental Diseases by the School of Medicine
experimental design. De Renzis application of clinical at the University of Trieste.
cases and appropriate methodology fostered inferen- Following his tenure in Milan and Gastaldis death in
tial thinking about his patients with unilateral hemi- 1973, De Renzi accepted a position as Chair of Ner-
spheric damage and consequently prompted vous and Mental Diseases in Modena. He acted as
examination of the interplay between specialization/ chief of a department with 70 neurological beds and
asymmetry and anteriorposterior lesion location. 40 psychiatric beds. In 1982, De Renzi published his
In 1964, one year after Henry Hecaen established the influential book Disorders of Space Exploration and
journal Neuropsychologia, De Renzi founded Cortex. Cognition, a book that remains a cornerstone in the
Cortex was created as an international journal devoted field of neuropsychology, with particularly rich insight
not only to the anatomy of the central nervous system, into perceptual and visuomotor disorders. He elo-
but more so to a focus on cognition and the relation quently and perceptively combined pertinent animal
between the central nervous system and mentation. Of research with detailed discussions of human neuropsy-
particular interest was the behavior of patients, includ- chological research in his book.
ing children, with acquired brain lesions versus normal Over the span of his 50-year devotion to neuropsy-
controls. In addition, the journal sought to provide chology, De Renzi has published on a broad range of
insight into the use of functional neuroimaging tech- topics. He distinguished four levels in the processing of
niques to record brain activity. De Renzi noted that the spatial information, including exploration, percep-
only way to subject ones research to a broader audi- tion, memory, and conceptualization. He described
ence and to contribute to the field was to publish in the phenomena of constructional apraxia, hand and
international journals rather than burying (it) in eye forced deviation, and disorders of space percep-
Italian journals (2006). With the birth of this journal, tion. De Renzi and his group examined hemispatial
De Renzi embarked on a challenging mission to ac- neglect and developed a purely acoustic test for audi-
quire not only the English language, but also a specific tory neglect. He also directed attention toward agno-
manner in which to report statistical data. The first sia. Along with Faglioni and Spinnler, De Renzi
editorial board of the journal included numerous devised a strategy to tackle recognition disorders by
prominent intellectual critics of the time, namely, Ar- investigating each hemispheres contribution to visual
thur Benton, George Ettlinger, Norman Geschwind, information processing. As technology advanced, De
Harold Goodglass, and Klaus Poeck. Gastaldi, one of Renzi utilized computed tomography scans to enhance
De Renzis early teachers, was the editor in chief. When his research. From prosopagnosia and Balints syn-
Gastaldi died in 1973, De Renzi assumed the role of drome to memory disorders and frontal signs, De
editor in chief and actively held this position until Renzis extensive research agenda contributed much
2000 when Sergio Della Sala succeeded him. to the field of neuropsychology as it is know today. In
During the 1960s, the European Brain and Behavior fact, he more recently examined the anatomy of dis-
Society was formed, whose aim was to provide an orders in depth perception (Turnbull, Driver, &
opportunity for international scientists to assemble to McCarthy, 2004) and focused on tactile agnosia as
share interests and beliefs about the biological founda- his final area of research.
tions of behavior. De Renzi actively participated in the De Renzi has considered himself primarily as a clinician
preliminary discussions to create the society and was who engages in research and teaching not only for self-
nominated as president for 19711973. In 1969, the edification, but also to shed light on the patients con-
Italian Society of Neurologys invitation to De Renzi ditions and optimal interventions. De Renzi believed
to present about hemispheric dominance represented that the pure essence of neuropsychology dwells in the
a momentous shift in the field. At the meeting, De Renzi interface of the patients treatment, research protocols,
reviewed his findings from the previous 6 years of and teaching. I always felt the opportunity to shift
research on a wide range of neuropsychological topics. from patient examinations to research and teaching,
At this time, neuropsychology seemed to be gaining in far from causing loss of concentration and dissipation
popularity as young students became impressed with of energy, is an asset because it keeps your mind open
neuropsychology, and an increasing number of psycho- and inquisitive on a wide range of practical and theo-
logical and neurophysiological departments were retical aspects of reality and can be immensely gratify-
emerging around the world (De Renzi, 1998). In ing (De Renzi, 1998).
Deaf D 773
Short Biography stage of his life. He commented that he sees his life as
positive and gratifying in that his interest and curiosity
Ennio De Renzi was born in the agricultural hub of about culture have enriched every day on earth, and he
Cremona, Italy on December 18, 1924. The atmosphere has led both the family and career life he has desired (De
in Italy during the first 20 years of his life was filled Renzi, 1998).
with fascist propaganda and devoid of any freedoms or
liberties. As such, De Renzi noted it was repressive, D
untruthful, and boring (De Renzi, 1998). His maternal Cross References
grandfather played a large role during De Renzis early
years, prompting him to devour as many books as Agnosia
possible. As a voracious reader during his school Anomic Aphasia
years, De Renzi learned to read literature in the original Apraxia
languages of French and German. Fortunately, De Arthur L. Benton
Renzis book knowledge flourished at a time when he Balints Syndrome
was required to relocate to 14 different cities during the Hemispatial Neglect
first 17 years of his life due to his fathers military Hemispheric Specialization
career. Unfortunately, he had significant difficulties Prosopagnosia
establishing strong interpersonal relationships with
classmates and teachers.
Interestingly, De Renzi had no interest in the
References and Readings
sciences during secondary school. So, he decided to
De Renzi, E. (1996). Balint-Holmes syndrome. In C. Code (Ed.), Classic
study law at the university. Patriotic propaganda
cases in neuropsychology (pp. 123144). New York, NY: Psychology.
permeated the political climate at that time while De Renzi, E. (1998). Ennio De Renzi. In L. R. Squire (Ed.), The history of
underneath the country was struggling. De Renzi noted neuroscience in autobiography (Vol. 5, pp. 227269). The Society for
that after the Nazi occupation of Italy and his attempt to Neuroscience. http://www.sfn.org/skins/main/pdf/history_of_neur-
locate a niche in the free part of the country, he enlisted oscience/hon_vol_5/c5.pdf. Retrieved 19 June 2009.
De Renzi, E. (1999). Agnosia. In G. Denes & L. Pizzamiglio (Eds.),
as a private and served alongside his father as a colonel
Handbook of clinical and experimental neuropsychology (pp. 371408).
in the Italian army (1998). Even though he had already New York, NY: Psychology.
passed seven law examinations, De Renzi was drawn to De Renzi, E. (2000). Prosopagnosia. In M. J. Farah & T. E. Feinberg
medicine, particularly during wartime turmoil. After (Eds.), Patient-based approaches to cognitive neuroscience (pp. 8595).
reading a book about brain functioning, he became Cambridge, MA: MIT.
De Renzi, E., Cavalleri, F., & Facchini, S. (1996). Imitation and utilization
fascinated with the workings of the mind and decided
behavior. Journal of Neurology, Neurosurgery, and Psychiatry, 61,
to abandon law, take his medical entrance exams, and 396400.
forge ahead into the field of medicine. In 1945, he De Renzi, E., Lucchelli, F., Muggia, S., & Spinnler, H. (1997). Is memory
accepted a position at the Ghislieri College in Pavia, loss without anatomical damage tantamount to a psychogenic deficit?
Italy. This institution was one of the few that offered The case of pure retrograde amnesia. Neuropsychologia, 35(6), 781794.
Milner, A. D. (1997). Ennio De Renzi. In N. Sheehy, A. J. Chapman,
deserving students from less-affluent families the oppor-
& W. Conroy (Eds.), Biographical dictionary of psychology
tunity for a university education free of charge. And (pp. 137138). New York, NY: Routledge.
so began his odyssey into the field of neurology and Stringer, A. Y., & Cooley, E. L. (2002). Neuropsychology: a twentieth-
ultimately neuropsychology. century science. In A. Y. Stringer, E. L. Cooley, & A.-L. Christenson
From his early childhood years, De Renzi sought to (Eds.), Pathways to prominence in neuropsychology: reflections of
twentieth- century pioneers (pp. 326). New York, NY: Psychology.
broaden his cultural horizons. As a youngster, he avidly
Tesak, J., & Code, C. (2008). Milestones in the history of aphasia: Theories
read literature from various countries. During college, and protagonists. New York, NY: Psychology.
he desired to interact with friends from different regions Turnbull, O. H., Driver, J., & McCarthy, R. A. (2004). 2D but not 3D:
of the world. He established the international journal, Pictorial-depth deficits in a case of visual agnosia. Cortex, 40, 723738.
Cortex, to facilitate international communication and
dissemination of knowledge. De Renzi firmly believed
that visiting foreign cities and spending time with collea-
gues around the world were added bonuses to his profes- Deaf
sion. He gleaned intimate knowledge about other places
and expanded his cultural horizons throughout every Deaf/Hearing Impairment
774 D Deaf/Hearing Impairment
Categorization
Natural History, Prognostic Factors,
Hearing is commonly defined according to where the Outcomes
problem occurs and the degree of hearing loss. Conduc-
tive hearing impairment refers to problems occurring in The impact of hearing loss varies widely depending on the
the outer or middle ear, which are commonly medically or cause, level, and type of hearing impairment and the age of
surgically treatable (i.e., otitis media). Sensorineural onset. Onset prior to the development of speech and language
hearing impairment typically refers to a failure in the is associated with greater difficulties with language acquisi-
cochleas ability to translate mechanical energy produced tion, academic achievement, and social and occupational
by the middle ear into neural impulses sent to the eigth functioning. Research indicates that early identification of
nerve. Mixed hearing loss refers to simultaneous occur- hearing loss and intervention yields significant benefits on
rence of both difficulties. Conductive and sensorineural language, academics, and social-emotional development
hearing losses are differentiated from various types of (Calderon & Naidu, 2000; Yoshinaga-Itano, Sedey, Coulter,
central hearing loss, often referred to as cortical deafness, & Mehl, 1998). With the adoption of newborn infant
which refers to impairment in the ability to interpret hearing screening procedures, many children are identi-
sounds despite adequate functional mechanisms for fied with hearing loss in the first months of life, compared
hearing (e.g., pure word deafness, auditory agnosia). to an average age of 18 months prior to universal screen-
Degree of hearing loss is typically defined by ones ings (Mertens, Sass-Lehrer, & Scott-Olson, 2000).
ability to perceive sounds of different frequencies Full access to language has a significant impact on
(measured by hertz or cycles per second) and at different predicted outcomes. Research has indicated that reading
Deaf/Hearing Impairment D 775
levels of deaf and hard of hearing high school graduates lobes are activated in individuals using sign language and
have traditionally hovered around the 4th grade level those relying on spoken language. However, in native sign
while deaf undergraduate college students with deaf par- language users, there is additional activation in the right-
ents read on average at 11th grade level (Hauser, 2001; hemisphere superior temporal and inferior parietal
Traxler, 2000). Recognizing the importance of communi- regions (Newman, Bavelier, Corina, Jezzard, & Neville,
cation, many families struggle to determine the most 2002). Other subtle differences in cognitive organization
appropriate method for augmenting hearing and have been found among individuals with hearing loss D
providing access to communication whether in English who prefer to communicate using sign language, including
and/or through a signed language. No single solution has improved peripheral visual attention, mental rotation,
emerged as panacea for all individuals with hearing loss. image generation, and face processing (for review, see Hau-
Regardless of communication modality, positive parent ser, Wills, & Isquith, 2006; Marscharck & Hauser, 2009).
child interaction has been identified as a predictor of the Despite these subtle differences, research has indicated
childs linguistic development (Calderon & Naidu, 2000; that when assessed nonverbally by an evaluator familiar
Pressman, Pipp-Siegel, Yoshinaga-Itano, & Deas, 1999). with their preferred communication mode, individuals
Research indicating that over 90% of children who are who are deaf and hard of hearing perform similarly to
deaf have parents who are hearing highlights the common hearing peers on standardized, nonverbally administered
struggle experienced by many families to find a suitable intelligence tests (Braden, 1994; Maller, 2003).
communication system (Mitchell & Karchmer, 2002). Considerable controversy exists regarding whether
Struggles with literacy secondary to limited access to there exists a psychology of deafness, that is, whether
spoken English often translate into career challenges. Un- there are certain traits that are uniquely associated with
fortunately, compared to hearing peers, unemployment individuals who are deaf or hard of hearing (Paul &
rates are higher for individuals who are deaf and hard of Jackson, 1993). Others suggest that this viewpoint repre-
hearing and large numbers receive Supplemental Security sents a culturally biased perspective (Lane, 1988). Some
Disability Insurance (SSDI) (Danek & Busby, 1999). researchers have suggested that there may be indirect
However, the employment challenges of individuals with developmental consequences of growing up with hearing
hearing loss are not significantly different from other loss in a world that relies heavily on auditory input
individuals with disabilities. For example, there is no (Hauser et al., 2006). Compared to same-age hearing
difference in the probability of employment between peers, individuals who are deaf and hard of hearing may
youth with learning disabilities and those with hearing be more likely to be exposed to risk factors that can
loss (Wagner, Newman, Cameto, Garza, & Levine, 2005). contribute to developmental and behavioral challenges.
Notably, these group means may overshadow the satisfac- These factors may include academic difficulties, low self-
tory quality of life attained by many individuals who are esteem, inconsistent discipline, and sexual/physical abuse
deaf and hard of hearing, many of whom attain successful (Black & Glickman, 2005; Simeonsson, Wax, & White,
careers in areas such as education, psychology, neuropsy- 2001). Although children with hearing loss develop
chology, medicine, and law. Advances in technology and attachments to caregivers similarly to their peers, the
legal precedent established through the Americans with diagnosis of a childs hearing loss may impact parents
Disability Act continue to broaden opportunities avail- attachment to their child and contribute to difficult
able to individuals who are deaf and hard of hearing. parentchild interactions (Meadow-Orlans, 1990).
Children who have not developed adequate language
and communication skills regardless of modality are
Neuropsychology and Psychology more likely to demonstrate difficulties with self-regula-
of Deafness/Hearing Loss tion and social skills (Rhine-Kahlback, 2004). Findings of
greater prevalence of psychopathology among individuals
Neuropsychologists should be cognizant that many causes with hearing loss are controversial and may reflect meth-
of hearing loss, particularly nongenetic causes, are com- odological flaws. Greater rates of psychopathology may
monly associated with other neurological factors that may not be attributable to hearing loss per se, but rather
have a severe impact on functioning. When isolated from may reflect characteristics of dysfunctional families such
comorbid neurological conditions, hearing loss has been as those commonly associated with greater rates of psy-
shown to impact cognitive organization but not intellec- chopathology among individuals who do not have
tual functioning (Braden, 1994). fMRI studies have indi- hearing loss (Powers, Elliott, Patterson, & Shaw, 1995).
cated that similar regions in the left frontal and temporal Limited communication skills in any modality are
776 D Deaf/Hearing Impairment
associated with poor psychosocial functioning and may neuropsychological evaluations with individuals from
significantly limit access to therapeutic intervention (Black this unique population. Neuropsychologists are strongly
& Glickman, 2005). Clinicians who are not skilled in encouraged to consult with and refer to clinicians trained
working with deaf clients may be more likely to provide in deafness.
a narrow range of diagnoses to clients who are deaf and
may over diagnose psychotic disorders. In contrast, when
assessed by deafness mental health specialists, lower rates Treatment
of psychosis and a greater range of less severe psychiatric
problems are reported (Black & Glickman, 2009). Treatment varies depending on the cause and nature of
the hearing loss as well as ones cultural perspective. No
one treatment approach has been demonstrated to be
Evaluation effective for all individuals who are deaf or hard of
hearing. Conductive hearing losses are often treated with
Hearing loss is typically identified through audiological antibiotics or minor surgeries, such as insertion of
assessment which may include behavioral, electroacous- pressure-equalization tubes. Individuals with sensorineu-
tic, and electrophysiological measures. Immittance audio- ral hearing loss may benefit from use of hearing aids or a
metry identifies outer and middle ear function. cochlear implant (CI) (refer to Pisoni et al., 2008 for a
Audiological evaluation also involves obtaining a pure- review of factors impacting outcomes of CI users). Reha-
tone audiogram which depicts an individuals hearing bilitation may include use of an amplification system in
sensitivity at varying frequencies and intensities. New- addition to language stimulation programs, use of assis-
born infant hearing screening procedures, which allow tive devices, auditory training, and speech therapy. Envi-
for early diagnosis of hearing loss, may include otoacous- ronmental accommodations (e.g., preferential seating),
tic emissions screening (OAE) and auditory brain stem use of adaptive technology to improve access (e.g., flash-
response screening (ABR). ing light systems), and communication repair strategy
Numerous factors impact the validity and reliability of training are also essential.
neuropsychological assessment of individuals who are The mental health needs of individuals who are deaf
deaf and hard of hearing. Misdiagnoses of mental retar- and hard of hearing traditionally have been underserved.
dation, learning problems, and psychopathology are com- Referral to a mental health professional trained in deaf-
mon when assessed by individuals not appropriately ness, preferably one with fluency in the clients preferred
trained to form clinical diagnoses based on an under- communication mode, reflects best practice. Indirect
standing of the dynamic interplay between deafness and communication through an interpreter during therapy
its associated causes, language functioning and accessibil- may negatively impact the therapeutic process by inter-
ity, and family dynamics. Factors complicating the assess- fering with the patienttherapist relationship, increasing
ment process include the match between the evaluator the likelihood of miscommunication, and interfering with
and clients preferred communication modality, selection the therapists ability to reliably assess the clients mental
of assessments demonstrated to be valid and reliable with status (Leigh, Corbett, Gutman, & Morere, 1996; Sussman
clients who are deaf, use of interpreters, and interpreta- & Brauer, 1999). When interpreters must be used, it is
tion of test results. Translation into a different communi- preferable to use a consistent, certified interpreter.
cation modality or use of other accommodations may
alter an assessments underlying construct and its psycho-
metric properties, therefore invalidating the results. As an Cross References
example, one of the most commonly used assessments of
intelligence, the Wechsler Intelligence Scale for Children Auditory Cortex
III, has been demonstrated to measure the construct of Auditory System
intelligence among children with hearing loss differently
than same-age hearing peers at the factor and item levels
(Maller & Ferron, 1997). Nonverbal assessment tools References and Readings
which do not rely heavily on verbal content reflect best
practice when assessing individuals with hearing loss American Speech-Language-Hearing Association (2009). The prevalence
(Braden, 1994; Maller, 2003). Specialized training is re- and incidence of hearing loss in children. Retrieved June 11, 2009,
quired to adequately conduct psychological and from http://www.asha.org/public/hearing/disorders/children.htm
DeanWoodcock Neuropsychological Assessment System D 777
Black, P., & Glickman, N. (2005). Language dysfluency in the deaf additional disabilities. Journal of Childhood Communication Disor-
inpatient population. JADARA, 39(1), 303321. ders, 17, 1519.
Black, P., & Glickman, N. (2009). Language and learning challenges in Pressman, L., Pipp-Siegel, S., Yoshinaga-Itano, C., & Deas, A. (1999). The
the deaf psychiatric population. In Cognitive-behavioral therapy relation of sensitivity to child expressive language gain in deaf and
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Synonyms
Powers, A. R., Elliott, R. N., Patterson, D., & Shaw, S. (1995). Family
environment and deaf and hard of hearing students with mild DeanWoodcock; DW; DWNB
778 D DeanWoodcock Neuropsychological Assessment System
Cross References
Death Aggravating Factors
Atkins v. Virginia
Brain Death Mitigating Factors
Cross References
Deceleration Injury
Acceleration Injury
B ETH R USH Biomechanics of Injury
Mayo Clinic Diffuse Axonal Injury
Jacksonville, FL, USA
Accelerationdeceleration injury Barth, J. T., Freeman, J. R., Broshek, D. K., & Varney, R. N. (2001).
Acceleration-deceleration sport-related concussion: The gravity of
it all. Journal of Athletic Training, 36(3), 253256.
Meythaler, J. M., et al. (2001). Archives of Physical Medicine and Rehabili-
Definition tation, 82, 14611471.
Olvey, S. E., et al. (2004). Neurosurgery, 54, 672677.
Deceleration injury is a traumatic injury to the brain,
typically following an acceleration injury to the brain in
a high-speed situation such as a motor vehicle accident or
high-impact sports. Deceleration injuries typically result
in additive damage to an acceleration injury after Decerebrate Posturing
the speed and inertia of momentum is obstructed. In
this situation, the brain that is moving forward in the J ACOB K EAN
direction of inertia falls backward in the opposite direc- Indiana University School of Medicine
tion, which may result in further compression of the Indianapolis, Indiana, USA
underlying brain tissue and white matter connections.
Synonyms
Current Knowledge
Extensor posturing
Similar to acceleration injuries, primary injury in decel-
eration injury results in bruising, hemorrhage, and
shearing of the underlying tensile strength of white mat-
ter connections deep within the brain. Secondary injury Definition
may occur hours or even days after the inciting traumatic
event. Secondary effects of injury can include decreased Decerebrate posturing is a pattern of movement com-
cerebral blood flow, edema, hemorrhage, increased in- monly produced by extensive forebrain lesions including
tracranial pressure, and biochemical changes that may the subcortex. It is characterized by rigidity, extension of
cause excitotoxicity and more extensive damage to the the arms with wrists pronated, extension of the legs,
surrounding brain structures and their associated downward pointing of the toes, and sometimes arching
connections. of the spine. Decerebrate posturing may be partial or
Acceleration and deceleration injuries are not uncom- asymmetric. Decerebrate (or extensor) posturing is
mon in high-impact sports such as football and hockey. distinguished from decorticate (or flexor) posturing by
Neuropsychologists are now often involved in baseline the extension of the arms. Decerebrate posturing is
cognitive screens for professional sports players and in generally the result of massive and bilateral forebrain
subsequent cognitive evaluations following a traumatic lesions or other damage that includes upper brainstem
event in which a concussion or traumatic brain injury and sometimes the rostral pons. Common causes include
is suspected. Professional sports organizations strongly cerebral infarction (stroke), intracranial hemorrhage, pri-
endorse the use of helmets in such sports activities to mary brain tumor, secondary brain tumor, traumatic
reduce the amount of free range of motion in the head head injury, increased intracranial pressure from any
that can potentially cause a more severe acceleration cause, brainstem tumor, and metabolic or hepatic
deceleration injury. encephalopathies.
Declarative Memory D 781
J ILLIAN S CHUH
University of Connecticut procedures remains largely intact. One famous patient,
Storrs, CT, USA H.M., described by Brenda Milner and William Beecher
Scoville in a classic 1957 paper, underwent surgery to
reduce seizures caused by intractable epilepsy. After the
Synonyms bilateral removal of his hippocampus and medial tempo-
ral lobes, H.M. suffered profound anterograde amnesia.
Explicit memory He was no longer able to store new declarative memories
and had a limited ability to retrieve previously stored
memories (Corkin, 1968; Milner, 1965). However, his
Definition ability to learn new skills (procedural memory) appeared
largely unaffected. For example, in a task of mirror draw-
Declarative memory refers to long-term memory, which ing, H.M.s accuracy improved with each testing session.
stores information that can be consciously discussed or However, H.M. was unable to recognize the details of the
declared. Declarative memory, which is the form of testing environment, and thought each session to be
memory as viewed by most lay people, involves the novel, as though he were encountering the testing materi-
deliberate encoding of information for objects, events, als for the first time (Milner, 1965; Scoville & Milner,
or facts. One of several distinct memory systems (Lezak, 1957). H.M.s case provides an example of anatomic dis-
Howieson, & Loring, 2004), declarative memory is often sociation, in which tasks that differ in their declarative
contrasted with non-declarative or procedural memory, versus procedural memory demands are associated with
which refers to the unconscious encoding of procedures, distinct neuroanatomical structures. This anatomic disso-
routines, or patterned information for learning skills or ciation suggests that declarative and procedural tasks may
processes. Declarative memory is further divided into rely on distinct memory storage and retrieval systems in
episodic, the memory for experiences localized in time the brain.
and space, and semantic, the memory for learned
knowledge that is not time locked to a specific event;
e.g., rote memorization of a historical fact or the letters Current Knowledge
of the alphabet (Fig. 1).
Since the time of H.M., many studies have examined
memory in a variety of neurological presentations. In
Historical Background particular, the consolidation of declarative memory has
been an area of interest and debate. While several memory
The theory of multiple memory systems largely began functions have been identified, there is controversy over
with the distinction between declarative and procedural the stages involved in the formation of declarative mem-
memory. This distinction is observed in patients with ories (Parkin & Rapp, 2001). For example, there is long-
anterograde amnesia, who have an inability to store new standing debate as to whether short term memory is a
declarative memories, while memory for skills or distinct function or one element in the consolidation
782 D Declarative Memory
process (Fuster, 2003; McGeoch, 1932). Many studies The consolidation of long-term memories is thought
suggest that there are three stages that are clinically useful to occur through a process known as long-term poten-
(Loring, 1999): sensory memory, short term/immediate tiation (LTP; Tranel & Damasio, 2002). LTP, lasting
memory, and long term/secondary memory (Fig. 2). anywhere from hours to months, is the simultaneous
In the first stage, lasting several seconds at the most, stimulation of two or more neurons that results in an
information is held in a sensory store, while extraneous increased synaptic strength, improving communication
stimuli in the environment are filtered out (Crowder, between the synapses (Cooke & Bliss, 2006). This stimu-
Surprenant, & Kazdin, 2000). The information either lation of a neural network primes the system for learning.
quickly decays or is further processed as short-term mem- Long-term memory formation occurs when continued stim-
ory if there is rehearsal or additional support for its ulation increases synaptic strength, allowing the learning to
salience. Short-term memory, known as immediate mem- be retained. Memory storage does not occur at particular
ory, serves as a temporary store. Lasting from roughly 30 s sites in the brain, but rather results from the contribution of
to several minutes (Lezak et al., 2004), this storage allows many cortical networks (Markowitsch, Tulving, & Craik,
for the integration of relevant perceptual information. If 2000). Several areas of the brain have been identified as
the memory trace is not processed for long-term consoli- particularly critical in declarative memory consolidation.
dation, the memory quickly dissipates. For example, upon These include the hippocampus, midline diencephalon,
receiving the new phone number of a friend, you may medial temporal lobe, mammillary bodies, thalamus, for-
remember it for the several minutes before dialing. nix, and frontal lobes (Parkin & Rapp, 2001).
However, it may be difficult to recall that number several
hours later. The number is retained while it is relevant,
but after a short period of time this memory dissipates. Future Directions
Memory traces can be retained for up to several hours
through rehearsal (the engagement of repetitive mental While it is clinically useful to organize the process of
processes; Brown, Craik, & Tulving, 2000). If this number declarative memory formation into distinct stages, these
was important to remember, you might repeat it several stages may be functionally interdependent. For example,
times or use a mnemonic device (a word or phrase recent research suggests that short-term memory may
representing the numbers) to enhance your memory. simply be the early part of the consolidation process in
The process of rehearsal increases the chance of that memory formation (Fuster, 2003). Additionally, the dis-
information being retained or encoded in long-term tinction between episodic and semantic memory can also
memory. However, it does not guarantee long term be fuzzy. If you learn that Nouakchott is the capital of
storage, as conscious rehearsal can only be maintained Mauritania, tomorrow you are likely to remember the
temporarily. Rehearsal techniques often imply that long- event of learning it (e.g., reading this article, where you
term memory formation happens through effortful were when reading, what happened before and after, etc.),
attention. However, memories may also form through making it an episodic memory. However, what happens if
incidental learning, which requires little conscious effort. tomorrow, a radio program has a feature on Mauritania?
If you repeatedly hear this fact, the episodic memory will
become a semantic memory. Conversely, if you have the
semantic knowledge that Nouakchott is the capital of
Sensory memory
(milliseconds to several seconds)
Mauritania, and then help your friend with a geography
project on Mauritania, you now have an episodic memory
Repetition
involving a semantic memory. It is likely these compo-
Short term/ Immediate
nents are not separate memory systems, but rather two
Memory
(thirty seconds to several minutes) processes that are closely intertwined (Willingham, 2004).
Even the distinction between declarative and procedural
memory can become blurred. Some researchers speculate
Long term/ Secondary
Memory that there is a developmental progression during learning
(hours to years)
upon which knowledge moves from more implicit
representations to more declarative representations as
Memory decay/
Forgetting the learner matures (Alibali & Koedinger, 2000).
Research continues to address these relationships, and it
Declarative Memory. Figure 2 Stages of declarative memory is likely that a multidisciplinary approach will further the
Decorticate Posturing D 783
knowledge of declarative memory. Brain imaging studies are Fuster, J. M. (2003). Cortex and mind: Unifying cognition. New York:
Oxford University Press.
improving the understanding of the anatomical networks
Lezak, M. D., Howieson, D., & Loring, D. (2004). Neuropsychological
involved in declarative memory processes. Such an under- assessment (4th ed.). New York: Oxford University Press.
standing will guide the examination of declarative memory Loring, D. W. (1999). INS dictionary of neuropsychology. New York:
formation at the cellular level, so that we will not only be able Oxford University Press.
to understand the anatomical areas important for declara- Markowitsch, H. J., Tulving, E., & Craik, F. I. M. (2000). Neuroanatomy
of memory. The Oxford handbook of memory (pp. 465484).
tive memory, but have better insight into the formation of D
New York: Oxford University Press.
declarative memory. Additionally, computational modeling McGeoch, J. A. (1932). Forgetting and the law of disuse. Psychological
can shed insight into how one memory processes may inter- Review, 39(4), 352370.
act with another. Finally, much of the research on declarative Milner, B. (1965). Memory disturbance after bilateral hippocampal
memory in humans has focused on typical adults or patients lesions. In P. Milner & S. Glickman (Eds.), Cognitive processes and
the brain. Princeton: Van Nostrand.
with lesions occurring later in life (as in the cases of studies
Ofen, N., Kao, Y. C., Sokol-Hessner, P., Kim, H., Whitfield-Gabrieli, S., &
of anterograde amnesia). While this has given immense Gabrieli, J. D. (2007). Development of the declarative memory
knowledge of the declarative memory system, relatively little system in the human brain. Nature Neuroscience, 10(9), 11981205.
empirical work has examined the development of this sys- Parkin, A. J., & Rapp, B. (2001). The structure and mechanisms of
tem. One imaging study by Ofen et al. (2007) comparing memory. The handbook of cognitive neuropsychology: What deficits
reveal about the human mind (pp. 399422). New York: Psychology
children to adults found increased activation in the pre-
Press.
frontal cortex with increasing age. Additionally, activation Scoville, W. B., & Milner, B. (1957). Loss of recent memory after bilateral
in the prefrontal cortex correlated with developmental hippocampal lesions. Journal of Neurology, Neurosurgery, and Psychi-
gains in the ability to remember details of an experience. atry, 20(1), 1121.
Further examination of developmental processes will not Tranel, D., & Damasio, A. R. (2002). Neurobiological foundations of
human memory. In A. D. Baddeley, B. A. Wilson, & F. N. Watts
only provide insight on the maturational trajectory of
(Eds.), Handbook of memory disorders. (2nd ed., pp. 2750). Oxford:
declarative memory, but also help further the understand- John Wiley & Sons.
ing of the relationship between those memory systems Willingham, D. T. (2004). Cognition: The thinking animal (2nd ed.).
which appear seemingly distinct. Upper Saddle River, NJ: Pearson/Prentice Hall.
Cross References
Current Knowledge
Cross References
DBS was first performed in 1987 to treat tremor of
Parkinsons disease and essential tremor. The exact
Decerebrate Posturing
mechanism of action for DBS is unknown, though it is
likely related to the inhibition/blockade of excitatory
membrane potentials. In Parkinsons disease, leads are
References and Readings usually implanted in the subthalamic nucleus and inter-
nal part of the globus pallidus (GPi). The procedure is
Posner, J. B., Saper, C. B., Schiff, N. D., & Plum, F. (2007). Plum and performed with stereotactic localization of the selected
Posners diagnosis of stupor and coma. New York: Oxford University nuclei and can be performed in one or two surgeries
Press.
depending on regional preferences and standard of
care. DBS generally allows for reduction in the need for
medications for the symptoms of Parkinsons disease.
Motor fluctuations and tremor are most affected by
DBS. Improvements in tremor, rigidity, and bradykine-
De-efferented State sia are sustained. DBS is considered a reversible
procedure.
Locked-In Syndrome
Cross References
Parkinsons Disease
Deep Brain Stimulation (DBS) Tremor
De-escalation D
Synonyms
DAWN E. B OUMAN
Drake Center, Default mode network; Task-negative network
Cincinnati, OH, USA
Definition
Definition
The default network is a system of brain regions that is
De-escalation is the reduction in the intensity of out-of- active when there are no external cognitive demands. The
control, agitated behavior and the prevention of further greatest brain activity is observed in this system during
behavioral crisis. Following a traumatic brain injury or unstructured rest, and the least activity is observed during
other neurological disorder, including stroke or brain tasks that require concerted external focus. The existence
tumor, some people become more easily confused, over- and core location of the default network are widely ac-
whelmed, threatened, fearful, uncertain, impulsive, and cepted; however, its function and subsystems remain
less able to control their behavior, resulting in increasing under investigation. The default network is distributed
levels of verbal and physical aggression. De-escalation bilaterally and is comprised of at least two core midline
techniques including decreasing stimulation, offering regions, the posterior cingulate cortex and medial frontal
calm reassurance, redirecting, and reorienting can gyrus, plus the inferior parietal lobule and areas in the
decrease agitation and lessen the intensity of a behavioral medial and lateral temporal lobe.
outburst.
Historical Background
functional neuroimaging research. An early meta-analysis uncontrolled baseline processing. The research focus very
of visual processing generated the first major interest in quickly transitioned from explaining observed deactivation
deactivation patterns in 1997. Schulman and colleagues patterns, to mapping the default network, and finally to the
found common deactivation patterns across studies that larger implications for cognitive neuroscience and neuro-
administered different visual tasks. These deactivations psychiatry. Although core regions have been well described
corresponded to regions now known as the default net- and empirically supported, the full anatomical extent and
work. In 2001, Raichle and colleagues first labeled this specific functions of this network remain under investiga-
baseline activity a default mode of brain function in a tion. Future work will likely be aimed at narrowing and
series of influential papers that substantially increased re- integrating the broad cognitive functions that have been
search interest in this system. attributed to the default network and its subsystems, for
example, the theory that the default network serves as an
imagery simulator for creative thinking. These and other
cognitive constructs associated with the default network
Current Knowledge
will require reliable and valid operational definitions that
are feasible to use in functional neuroimaging environ-
Investigation of default network activity has revealed con-
ments. Integration of the default network with other
sistent inverse relationships with a wide variety of exter-
brain systems such as the task-positive network and atten-
nally focused tasks that range from visual processing
tional systems is also likely to be a major goal of future
to more complex working memory tasks. Consistent pos-
research. Investigation of the role of the default network in
itive relationships have been observed with several inter-
neuropsychiatric disorders is providing novel findings and
nal thought processes such as stimulus independent
a new framework within which to interpret them. Clinical
thoughts, daydreaming, mind wandering, imagining an-
research on the default network remains limited; however,
other persons perspective, imagining the future, self-
this direction is very likely to grow rapidly.
referential thought, and retrieving memories. At rest
and during externally focused cognitive challenges, de-
fault network activity has been found to be inversely References and Readings
correlated with another set of brain regions that are
recruited during externally focused goal-directed proces- Buckner, R. L., Andrews-Hanna, J. R., & Schacter, D. L. (2008). The
sing (i.e., task-positive network). Therefore, the default brains default network: Anatomy, function, and relevance to disease.
network may represent one component of an opponent Annals of the New York Academy of Sciences, 1124, 138.
process system that is responsible for introspection, while Fox, M. D., Snyder, A. Z., Vincent, J. L., Corbetta, M., Van Essen, D. C., &
the task-positive network may be related to processing Raichle, M. E. (2005). The human brain is intrinsically organized
into dynamic, anticorrelated functional networks. Proceedings of the
with external attentional focus. It has been suggested National Academy of Sciences USA, 102(27), 96739678.
that the general function of the default network is flexi- Raichle, M. E., MacLeod, A. M., Snyder, A. Z., Powers, W. J., Gusnard,
ble mental simulation. Cognitive subprocesses may also D. A., et al. (2001). A default mode of brain function. Proceedings of
be mapped onto different subsystems of the default the National Academy of Sciences USA, 98, 676682.
network. For instance, areas in the medial temporal Shulman, G. L., Fiez, J. A., Corbetta, M., Buckner, R. L., Miezin, F. M.,
et al. (1997). Common blood flow changes across visual tasks: II.:
lobes appear to be recruited during memory contribu- Decreases in cerebral cortex. Journal of Cognitive Neurosciences, 9,
tions to default processing, while increased activity in 648663.
the medial frontal cortices has been associated with
evaluation of self-relevant situations. Altered default net-
work activity has been reported among older adults and
several clinical populations, including patients with Defective Visual Localization
autism, Alzheimers disease, depression, anxiety disor-
ders, and schizophrenia. Optic Ataxia
Future Directions
Defensiveness
There has been rapid growth in research on the default
network since it was first recognized as more than simply K Scale
Dejerine, Joseph Jules (18491917) D 787
Cross References
Deficit Measurement
Best Performance Method
S ANDRA B ANKS Individual Comparison Standards
Allegheny General Hospital National Adult Reading Test
Pittsburgh, PA, USA Premorbid Functioning
Wechsler Test of Adult Reading D
Definition
References and Readings
Deficit measurement is the measurement of impairment
Knapp, S. J., & VandeCreek, L. D. (2006). Assessment. Washington, DC:
when an examinees performance is significantly below an
American Psychological Association.
actual or estimated previous level of functioning. Lezak, M. D., Howieson, D. B., Loring, D. W., Hannay, H. J., &
Fischer, J. S. (2004). Neuropsychological Assessment. New York:
Oxford University Press.
Snyder, P. J., Nussbaum, P. D., & Robins, D. L. (2006). Clinical neuro-
Current Knowledge psychology: A pocket handbook for assessment. Washington, DC:
American Psychological Association.
In neuropsychology, deficit measurement can be achieved
by assessing cognitive functioning as separate domains or
as a composite of domains. For instance, cognitive deficits
in a particular ability domain, such as list-learning ability, Degenerative
can be measured via the administration of a list-learning
task during which the examinee must recall a list of words Atrophy
both immediately and after a specified time delay. If the
examinee performs poorly, the score on this subtest pro-
vides information about deficits associated with the Deglutition Disorder
examinees list-learning ability. In the case of using com-
posite scores, several subtests are combined to generate a Dysphagia
composite score that represents performance in a general
domain of functioning, such as auditory memory. This
could include several separate aspects of functioning, such
as list-learning, narrative memory, encoding of abstract
Dejerine, Joseph Jules (1849
concepts, etc. 1917)
The level of deficit is determined by comparing the
examinees subtest or composite score with that of the J ACKIE L. M ICKLEWRIGHT, T RICIA Z. K ING
normative group (the group of individuals in the popula- Georgia State University
tion who have been examined with the same test) or with Atlanta, GA, USA
the examinees previous level of functioning. Deficit mea-
surement thus highlights the concept of normality,
given that a particular level of performance to represent Education and Training
a deficit when the level of performance falls below the
level most commonly observed (also called the criterion 1868 and 1870: Academie de Geneve (Bachelors of
level) in the population at large is considered. Deficit Science in Biology and Comparative Anatomy)
measurement is often captured indirectly, by estimating 1875: Hospital de la Pitie (Internship)
the examinees premorbid ability level, ideally by clinical 1879: Faculty of Medicine of Paris (Doctor of Medicine)
interview with the examinee and others, behavior rating
scales, and premorbid test results, and comparing the Major Appointments
examinees present performance with this estimate.
More direct comparison can be challenging because ob- Head of Clinic, Hospital Bicetre (18791886)
jective measures of an examinees previous level of func- Professor of Neurology and Chief Consultant,
tioning may be unavailable. Hospital Bicetre (18871894)
788 D Dejerine, Joseph Jules (18491917)
Professor of Neurology, Salpetriere (18951911) Charcot was planning to block his candidacy; Dejerine
Clinical Chair in Diseases of the Nervous System, confronted Charcot and demanded an explanation. After
Salpetriere (19111917) a heated discussion, Jean Martin Charcot conceded and
promised Dejerine his vote. Dejrine was appointed Profes-
sor Agrege and Chief Consultant for Hospital Bicetre in
Major Honors and Awards 1887. During his 8-year tenure he began his seminal work
on cerebral localization, outlining the somatotopy of pyra-
Vice-President Societe de Biologie (1895) midal tracks, ascending sensory tracts, and thalamo-corti-
Founding member of the French Neurological Society cal connections. Dejerine also published widely on
(1899) acquired disorders of spoken and written language. His
Moxon Gold Medal of the Royal College of Physicians first publication on writing disorders in aphasia patients
of London (1914) (1891) provided an outline of the neural architecture
necessary for reading, writing, and pronunciation. He
went on to provide detailed clinical and organic descrip-
Landmark Clinical, Scientific, and tions of progressive aphasia and pure alexia. Dejerine was
Professional Contributions the first to demonstrate that pure alexia resulted from the
disconnection of the angular gyrus from both visual
French neurologist J. Jules Dejerine earned acclaim for cortices.
his localizationist approach to the study of the ner- In 1895, Dejerine joined the faculty of the famed
vous system by combining the anatomical approach of Salpetriere as a Professor of Neurology. In the years to
Charcot with the experimental approach of Vulpian. follow, his collaborations yielded numerous publications
Dejerines early research focused on the pathophysiol- and clinical descriptions of disorders of the nervous
ogy of sensation and acquired disorders of spoken and system, including peripheral and central ataxia, sensory
written language. His body of work yielded detailed parietal syndrome, and thalamic syndrome. His body of
clinical and anatomical descriptions of a number of work culminated in the publication of Anatomie des
cognitive disorders, including pure alexia, aphasia, centres nerveux in 1895 and Semiologie des affections du
tactile agnosia, and thalamic syndrome. systeme nerveux in 1914. Akin to many of the French
neurologists before him, Dejerine developed an interest
in the therapeutic treatment of mental disorders. Dejerine
Short Biography believed that rational and theoretical aspects of psycho-
therapy are secondary to the emotional elements; he
J. Jules Dejerine was born into a modest family in 1849, in emphasized nonspecific contributors to therapeutic
Geneva, Switzerland. As a young man during the Franco- change such as support, empathy, and understanding.
Prussian war, Dejerine cared for wounded soldiers in a In 1915, he published. The psychoneuroses and their
Geneva hospital. After earning degrees in biology and com- treatment by psychotherapy.
parative anatomy from the Academie de Geneve, he depart- In addition to his prolific research career, Dejerine
ed for Paris in 1871 to pursue medical studies. Under the served as a founding member of the French Neurological
mentorship of Edme Felix Alfred Vulpian at Hospital de la Society. He also was actively involved with the Academie
Pitie, Dejerine published numerous papers on pathologies de Medicine and Societe de Biologie, which elected him
of the peripheral nervous system. In 1879, after completing Vice-President in 1895. Dejerines final contributions
his dissertation on acute ascending paralysis, Dejerine came during the First World War when he worked
received a Doctor of Medicine degree from the Faculty of endlessly to care for the neurologically wounded in a
Medicine of Paris. In 1888, Dejerine found both a partner military hospital. After many years of service, Dejerine
and collaborator when he wed American Augusta Marie died of uremia in 1917 at the age of 67.
Klumpke, the first female medical intern in Paris.
Dejerines hard-working reputation preceded him, and
upon completion of his degree, he was offered the Head of Cross References
Clinic position under Professor Alfred Hardy at the Hospi-
tal Bicetre in Paris. He remained in this position until 1886 Agraphia
when he was nominated Professor Agrege. Shortly follow- Alexia
ing his nomination Dejerine learned that Jean Martin DejerineRoussy Syndrome
Delayed Response Tasks D 789
Synonyms
Delayed Prompting
Thalamic pain syndrome
Errorless Learning
Definition
an example of a hyperactive delirium; however, the condi- days. It may be preceded by a subsyndromal period of
tion, along with the withdrawal caused by other sedatives, more subtle cognitive deficits. The level of confusion and
may represent a distinct condition with unique pathophys- degree of arousal generally fluctuates throughout the day.
iology. Hypoactive delirium, although also common is The following vignette illustrates a typical presentation of
under recognized given its subtler phenomenology, and it delirium:
is sometimes called quiet delirium. That the hyperactive An 82-year-old man was transferred from a nursing
and hypoactive states can co-occur in a mixed state (which home to a general medical hospital for a change in mental
may be more common than either state alone) lends sup- status. Usually quiet and pleasant, he became increasingly
port to the idea that the two types are not distinct entities, suspicious and annoyed at the nursing home staff. He had a
but rather two expressions of the same syndrome. history of Alzheimers disease, which was considered moder-
It should be noted that in the neurology literature, the ate in severity. On admission to the hospital, he was lethar-
word delirium is sometimes used to describe only the gic and slept for most of the morning. Later in the day, he
hyperactive type, and the hypoactive category is referred was seen by the attending physician; at that time, he was
to terms such as acute encephalopathy. However, as awake, alert, and only mildly confused and the physician
discussed above, there is little evidence to suggest that questioned the appropriateness of the patients admission to
the two categories represent meaningful distinctions, the hospital. However, later that evening the patient became
and in the psychiatric literature, delirium refers to argumentative with staff, demanding to leave the hospital.
both the hyperactive and hypoactive types. He seemed to believe he was in fact locked up in a prison,
Delirium is also occasionally categorized by the site in and worried that people were trying to break in through his
which it takes place: thus, the term Intensive Care Unit window in order to harm him. He was given haloperidol,
(ICU) Psychosis is sometimes used in the medical litera- which helped calm him; however, he continued to be para-
ture to describe a particularly troublesome example of a noid and confused. On medical evaluation, a urinary tract
hyperactive delirium seen in the intensive care setting. infection was discovered, and appropriate antibiotics were
Again, such categorizations are of little heuristic or diag- initiated. His periods of belligerence slowly decreased over
nostic value and there is no reason to suspect that the the next several days, and within a week, although still more
delirium occurring in that setting differs from that seen confused than normal, he was able to return to his nursing
elsewhere. home.
This vignette illustrates several typical aspects of de-
lirium, most notably the fluctuating course, which can
Epidemiology
confuse the treating team. The patients dementia can be
seen as a predisposing factor, while the infection was the
Delirium is likely the most common syndrome affecting
likely precipitant. Although treating the precipitants is the
patients in general hospitals. Accurate prevalence figures
mainstay of treatment, it is typical that cognitive improve-
are difficult to obtain given the methodological limita-
ment lags behind treatment of the underlying medical
tions of the various investigations into the disorder. Epi-
condition.
demiological and clinical studies suggest that 1020% of
The prognosis and outcome of delirium is under-
all hospitalized adults, 3040% of elderly hospitalized
standably variable, and dependent on the actual preci-
patients and up to 80% of patients in intensive care
pitants. Although much literature including the
units experience delirium at some time during their hos-
DSM-IV-Text Revision (DSM-IV-TR) (American
pitalization. Delirium is most likely underreported in any
Psychiatric Association, 2000) describes delirium as a
setting; the hypoactive type in particular is often not
transient and reversible state, more recent work suggests
noticed by clinical teams, or misdiagnosed as another
that there are often permanent cognitive deficits following
disorder. The development of validated instruments
an episode of delirium. The syndrome is a poor prognos-
designed to detect delirium (see below) has improved
tic indicator that is associated with high morbidity and
the detection of delirium.
mortality. That this is so may simply reflect the many
serious illnesses than can precipitate a delirium, however
Natural History, Prognostic Factors, some investigators have suggested that the syndrome itself
Outcomes can have a deleterious effect on the course of an illness,
perhaps through the damaging effects of the abnormal
By definition, delirium is an acute syndrome, meaning neurochemical activity that occurs during a delirious
that it comes on rapidly, often in the course of hours to episode.
Delirium D 793
Neuropsychology and Psychology Rating Scale (Trzepacz, Mittal, Torres, Kanary, Norton,
of Delirium & Jimerson, 2001), the Memorial Delirium Assessment
Scale (Breitbart et al., 1997), and the Confusion Assess-
Delirium is a neuropsychological syndrome that can ment Method (Inouye, VanDyck, Alessi, Balkin, Siegal, &
affect any area of cognitive functioning. Its effect on the Horwitz, 1990).
most primitive of functions (attention, concentration, Many disorders can mimic the symptoms of delirium,
and arousal) clearly has effects on more complex func- and the differential diagnosis can be difficult in the acute D
tions such as abstract thinking, judgment, and insight. setting. One of the more challenging distinctions is be-
Decision-making capacity is generally impaired, and tween delirium and the dementias. Although both are
patients (and their families) are encouraged to postpone defined by their cognitive effects, patients with dementia
important decisions until the delirium can be treated. generally have an intact level of consciousness, and the
Many delirious patients, once recovered, do not re- course of dementia (particularly the common ones such
member the episode. In one study, of those who were able as Alzheimers disease) is more chronic and less fluctuat-
to recall it, at least half experienced their delirious episode ing. However, the two disorders frequently co-occur, and
as highly distressing (Breitbart, Gibson, & Tremblay, comorbid delirium including subsyndromal delirium
2002). This is true even for those patients who appeared should be suspected in cases where patients with dementia
calm or sedated at the time they were delirious. have perceptual disturbances or sudden changes in their
mental state (Meagher & Trzepacz, 2007).
Both delirious and psychotic patients can have hallu-
Evaluation cinations and delusions; however, patients with primary
psychotic disorders such as schizophrenia tend to experi-
The provocative behavioral effects of delirium (such as ence their symptoms in the setting of a clear sensorium:
agitated and violent behavior) usually bring patients to they feel alert, awake, and aware of their surroundings.
clinical attention. When these symptoms are lacking, de- Schizophrenic patients generally lack the profound con-
lirium is often missed; thus, the most important part of fusion and general cognitive deficits seen with delirious
the evaluation is maintaining a high level of suspicion for patients. In addition, the delusions and hallucinations
delirium in the clinical setting. Delirium should be sus- experienced in schizophrenia tend to be more elaborate
pected in individuals who have factors that predispose and fixed than with delirious patients.
them to the syndrome. These include preexisting cogni- Delirium, particularly hypoactive delirium, can be
tive disorders, such as any dementia, substance use dis- mistaken for depression, and the psychosomatic medicine
orders (particularly alcoholism), neurological disorders, literature suggests that this misdiagnosis is common in
and the presence of any collection of chronic medical the general hospital setting. Although the two share some
disorders and/or the use of multiple therapeutic medica- features such as impairments in concentration and atten-
tions. Psychiatric disorders alone, apart from the demen- tion, depression has a more gradual onset and a less
tias and substance use disorders do not significantly fluctuating course. Delirious patients may look sad at
increase the risk of a delirium. times, even tearful, however their mood tends to be
When delirium is suspected, the evaluation should more labile than with depressed patients.
focus on the cognitive examination. Evidence of impaired Similarly, mania, particularly severe mania can mimic
attention, disorientation to date and place, and gross the confusion and agitation associated with delirium.
confusion all make delirium a likely explanation. General Again, the course can help distinguish the two, with
cognitive testing such as that used for the Mini-Mental mania usually having a history of a more progressive course
State Exam can show evidence of the gross cognitive leading to the current symptoms. In addition, although
impairment found in delirious patients; however, the severely manic patients can be confused, they tend not to
test is not specific for delirium. It can be useful to include have the gross cognitive changes seen with delirium.
tasks that focus on the attentional deficits seen in deliri- In cases where the clinical exam cannot distinguish
um, and such as Clock Drawing and the Trail Making Test. between delirium and other psychiatric disorders, an
These tests, although limited in scope have the advantage electroencephalography (EEG) may help: the EEG shows
of being simple to use and require little training. diffuse slowing, whereas the EEG is normal in most other
A number of tests specifically designed to detect delir- psychiatric disorders.
ium have been developed; these require specific training Once convinced of the presence of a delirium, the
in their use, and include such tests as the Delirium major evaluative task becomes one of discovering the
794 D Delirium
underlying etiology. Essentially, any process that can cause simple exercises, and sleep hygiene protocols. Most of
a disruption in the normal functioning of the brain can these behavioral strategies have limited support in the liter-
cause a delirium. The most common causes are infections ature, and many have never been tested in the acute setting;
and the toxic effects of drugs and other substances. There however, they have anecdotal support, face validity, and are
are a number of useful mnemonics meant to help one unlikely to harm the patient. There is some methodologi-
remember the potential precipitants: one popular mne- cally sound data to support behavioral interventions for the
monic is the acronym I WATCH DEATH (a potent prevention of delirium in predisposed individuals (Inouye
reminder of the morbidity associated with delirium): et al., 1999) as well as for the efficacy of staff education to
Infections recognize delirium early (Lundstrom, Edlund, Karlsson,
Withdrawal (Alcohol and other substances) Brannstrom, Bucht, & Gustafson, 2005).
Acute Metabolic At times, symptomatic pharmacological treatment of
Trauma delirium becomes necessary. Such treatment is usually
Central nervous system pathology initiated when the behavioral manifestations of delirium,
Hypoxia particularly aggressive or violent behaviors, make it im-
Deficiencies (vitamin, other nutritional) practical or dangerous to wait for the natural improve-
Endocrinopathies ment that will follow the successful treatment of the
Acute vascular disorders underlying precipitants. Alternately, no clear underlying
Toxins/drugs disorder may be found, and yet palliative care may be
Heavy metals indicated to relieve the delirious patients distress.
It is apt to consider these as precipitants than Most commonly, sedating medications are given to
causes. In reality, the cause is usually multifactorial, calm the delirious patient. Most of the evidence for such
combining predisposing factors with acute precipitants treatment is anecdotal or derived from uncontrolled stud-
that upset an already tenuous cognitive balance. It can ies. Historically, the most common medications used in
be argued that neither is a direct cause, but that the the acute setting were narcotics, benzodiazepines, and
combination initiates a cascade of events in the patients antipsychotics. Narcotics are rarely used now except in
brain. A number of experts have used the term acute cases where pain or dyspnea is thought to play a role in
brain failure to describe delirium: the term is meant to the patients general distress. Benzodiazepines are still
imply an analogy to heart failure, in which an already commonly used, however should not be considered a
impaired organ can suddenly and rapidly malfunction first-line medication given their capacity to confuse
after the onset of a seemingly minor insult. patients further. Although this side effect is sometimes
labeled as a paradoxical effect of the drug, it is in fact a
common side effect of all sedative-hypnotic medications.
Treatment Antipsychotics are most commonly used, both typi-
cal antipsychotics such as haloperidol, and the atypical
The most important step in the treatment of delirium is agents such as risperidone, olanzapine, quetiapine, and
recognition of the syndrome. Once recognized, treatment newer agents. All antipsychotics seem to carry the benefit
primarily focuses on discovering and treating the under- of having a calming effect without causing the level of
lying precipitants, and the particular treatment is specific sedation and confusion seen with benzodiazepines. It is
to the medical or neurological disorder. thought that the antipsychotic effect of these agents can
A number of behavioral strategies have been suggested help organize a patients thoughts; this latter supposition is
to minimize the confusion of the delirious patient. These not as well supported, and seems counter to the under-
strategies include indirect environmental interventions, for standing of the pharmacodynamic effects of a group of
example introducing lighting variations (or windows) into drugs that often take weeks to treat psychosis. Anecdotally,
intensive care settings to help orient patients to the time of however, patients do seem less confused and psychotic
day, and the minimization of unnecessary noise. Objects after only a short term of antipsychotics; it may simply
from the patients home may be placed in a hospital room to be that patients can organize their thoughts better once
increase the familiarity of the surroundings or family mem- they are calmer.
bers may be encouraged to remain with the patient when- Many of the side effects that limit the use of antipsy-
ever possible. Direct behavioral interventions include chotics, such as parkinsonian symptoms or tardive dyski-
frequent reorientation of the patient, minimization of nesia, are less relevant in the acute care setting. When
restraints, use of sensory aids (eyeglasses, hearing aids) using antipsychotics, once should watch for the rare but
Delirium D 795
U.S. Food and Drug Administration. (2005). FDA public health advisory: The single-score method is particularly problematic for
Deaths with antipsychotics in elderly patients with behavioral distur-
executive-function tests because these tasks typically
bances. Rockville, MD: U.S. Food and Drug Administration.
require both more fundamental cognitive skills (e.g.,
language, visuoperception, and fine motor skills)
and higher-level executive functions for successful perfor-
mance. The D-KEFS was developed to provide measures
Delirium Tremens that assess (a) both fundamental and higher-level skills in
order to evaluate if a patients poor performance on a task
Alcoholic Brain Syndrome is due to an executive-function deficit or to an im-
pairment in a more fundamental cognitive skill and (b)
strategies and error types in order to more precisely char-
acterize the nature of the executive dysfunction.
DelisKaplan Executive Another objective of the D-KEFS was the development
of test-design features that enhanced the instruments
Functioning System sensitivity to mild executive dysfunction or mild frontal-
lobe injury. For instance, switching conditions were added
E RIC M. F INE 1, D EAN C. D ELIS 2 to several traditional executive-function tasks that previ-
1
San Diego, CA, USA ously did not require cognitive flexibility. As an example,
2
University of California, San Diego, School of Medicine in addition to the three standard Stroop conditions
La Jolla, CA, USA (color naming, word reading, and inhibition; Stroop
Color Word Test, Adult), a new Stroop condition was
developed for the D-KEFS Color-Word Interference Test
Synonyms that requires examinees to switch back and forth between
naming the dissonant ink color and reading the dissonant
D-KEFS word. Cognitive switching, or the ability to abandon a
previous response in order to generate a novel or compli-
mentary response (Delis et al., 2001a), is considered one
Description of the hallmarks of executive functions and is particularly
dependent on the integrity of frontal-lobe functioning. In
The DelisKaplan Executive Function system (D-KEFS) is addition, certain D-KEFS tests were designed to contain
the first battery of tests designed exclusively for the assess- capture stimuli that pull for concrete or stimulus-
ment of executive functions in children and adults that bound responding in patients who are vulnerable to this
has been normed on a large national sample representa- tendency due to frontal-lobe injury.
tive of the demographic characteristics of the U.S. popu-
lation (Delis, Kaplan, & Kramer, 2001a). Key objectives in
the development of D-KEFS measures were to provide Descriptions of the D-KEFS Tests
psychologists a comprehensive tool for assessing a wide
array of executive functions, including cognitive flexibili- The D-KEFS consists of nine tests measuring a wide spec-
ty, problem-solving, conceptual reasoning, inhibition, trum of verbal and nonverbal executive functions. These
multitasking, and nonverbal and verbal creativity. tests were either (a) modifications of existing clinical tests of
The D-KEFS can be distinguished from most other executive functions (e.g., Stroop Color-Word Interference
executive-function tests by its embrace of the cognitive Test and Trail-Making Test) in order to increase their sensi-
process approach in which multiple measures are gener- tivity to frontal-lobe dysfunction, (b) or modifications of
ated to isolate the mechanism of a patients poor score on tasks used in past experimental studies of executive func-
a particular task (Delis, Kramer, Kaplan, & Ober, 2000; tions but that had not been developed into standardized
Kaplan, Fein, Kramer, Delis, & Morris, 1999). With the clinical instruments, or (c) relatively new tests that Delis
exception of the Wisconsin Card Sorting Test (Heaton, and colleagues developed.
Chelune, Talley, Kay, & Curtiss, 1993), the majority of
existing clinical measures of higher-level cognitive func- D-KEFS Trail-Making Test
tions yield a single achievement score for each task (e.g., The D-KEFS Trail-Making Test is a modification of the
the Category Test and the traditional Trail-Making Test). traditional Trail-Making Test that was first developed by
DelisKaplan Executive Functioning System D 797
Partington (Partington & Leiter, 1949), modified by army and empty dots. The switching condition, which is novel
psychologists, and then included in the HalsteadReitan to the D-KEFS, has been found to be particularly sensi-
battery. It is one of the most commonly used neuropsy- tive to frontal-lobe dysfunction (e.g., Cato, Delis, Ailds-
chological measures for assessing executive functions. kov, & Bigler, 2004; Kramer et al., 2007).
While the traditional version offers two conditions Part
A (number sequencing) and Part B (numberletter D-KEFS Color-Word Interference Test
switching) the D-KEFS version offers five conditions: The D-KEFS Color-Word Interference Test is a variant of D
visual scanning, motor speed, number sequencing, letter the Stroop Test (Stroop, 1935; Stroop Color Word Test,
sequencing, and numberletter switching. The four base- Adult), a commonly used measure of response inhibition.
line conditions enable the clinician to assess empirically if The D-KEFS version includes two baseline conditions
a patients poor performance on the switching condition is naming of color squares (Condition 1) and reading of
due to a deficit in cognitive flexibility or to impairment in color words printed in black ink (Condition 2) and the
one or more of the underlying component skills needed to traditional interference condition (Condition 3) in which
perform the switching task (i.e., motor speed, visual scan- the examinee must inhibit reading the words in order to
ning, number sequencing, or letter sequencing). name the incongruent ink colors in which these words are
printed in. The D-KEFS version differs from other Stroop
D-KEFS Verbal Fluency Test tasks by the inclusion of a fourth condition that requires
Verbal fluency tasks are among the most commonly admi- the examinee to switch back and forth between naming
nistered neuropsychological measures ( Verbal Fluency). the dissonant ink colors and reading the conflicting
The D-KEFS Verbal Fluency Test is a timed measure that words. This condition has been shown to be particularly
includes (a) a letter fluency condition that requires exam- sensitive to frontal-lobe dysfunction (e.g., Cato et al.,
inees to generate as many words as possible that start with a 2004).
particular letter, (b) a category fluency condition that
requires examinees to generate as many words as possible D-KEFS Sorting Test
from designated semantic categories, and (c) a category The D-KEFS Sorting Test (formerly called the California
switching condition that requires examinees to alternate Card Sorting Test; Delis, Squire, Bihrle, & Massman,
between generating words from two different semantic cate- 1992) was designed to provide a standardized measure of
gories. In general, patients with predominately frontal-lobe conceptualreasoning skills. Specifically, Condition 1, free
damage tend to have more difficulty on the letter fluency sorting, requires examinees to sort cards according to eight
task relative to the category fluency task, whereas patients possible target rules, including five primarily perceptual or
with early Alzheimers disease often show the opposite nonverbal rules (e.g., straight versus curved outer edges),
pattern due to a breakdown in semantic knowledge and three primarily verbal rules (e.g., clothing versus body
(Delis et al., 2001b). parts). In Condition 2 (sort recognition), the examiner
sorts the same sets of cards into two groups according to
D-KEFS Design Fluency Test the eight target sorts and, after each sort, asks the examinee
Design fluency measures, developed as a nonverbal analog to identify and describe the correct rules used to generate
of verbal fluency measures (Jones-Gotman & Milner, the sort. Performance is evaluated both in terms of the
1977; Design Fluency), require examinees to produce total number of correct target concepts reflected in the
as many different designs as possible that meet a particu- examinees sorts, as well as the accuracy and level of ab-
lar criterion (e.g., the designs must always contain four straction of the examinees sort descriptions. This measure
lines) within a time interval. The D-KEFS Design Fluency has been found to be sensitive to frontal-lobe dysfunction
Test is a modified version of traditional procedures. For (e.g., Huey et al., 2009; Fine et al., 2009).
each condition, the examinee is presented rows of boxes
that contain an array of dots and is instructed to draw as D-KEFS Twenty Questions Test
many different designs as possible using only four straight The D-KEFS Twenty Questions Test is a modification of a
lines. The three conditions of the test vary in difficulty, popular, informal game played by children and adults, and
with Condition 1 (filled dots) assessing basic design assesses categorical processing, hypothesis testing, and
fluency, Condition 2 (empty dots only) requiring exam- concept formation. For this task, the examinee is presented
inees to inhibit connecting the filled dots while connect- with a stimulus page depicting pictures of 30 common
ing only the empty dots, and Condition 3 (switching) objects. The examinee tries to ask the fewest number of
requiring examinees to switch between connecting filled yes/no questions in order to identify the unknown target
798 D DelisKaplan Executive Functioning System
object. Multiple process measures are scored, including clearly displayed to the examiner in the stimulus booklet
level of abstract thinking, error types, and response stra- and the response form.
tegies (e.g., verbal versus nonverbal). This test has been Each D-KEFS test is a stand-alone instrument that
found to be sensitive to focal frontal lesions (Baldo, Delis, can be administered individually or with other D-KEFS
Wilkins, & Shimamura, 2004). tests. For all of the primary measures and many of the
optional measures, the raw scores are converted to scaled
D-KEFS Word Context Test scores, with a mean of 10 and a standard deviation of 3, for
The D-KEFS Word Context Test is an adaptation of a test each of the following 16 age groups: 8, 9, 10, 11, 12, 13, 14,
developed originally for studying how children acquire 15, 1619, 2029, 3039, 4049, 5059, 6069, 7079, and
word meanings (Werner & Kaplan, 1952). The examinee 8089. Raw scores for several of the optional measures
is asked to discover the meaning of mystery words based have limited ranges in normative (nonclinical) popula-
on clues given in sentences. For each mystery word, the tions, and therefore they are corrected using cumulative
examinee is shown five sentences (clues) that assist with percentile ranks for each of the 16 age groups. The D-KEFS
decoding the meaning of the words. This test is a measure scoring software is available, which automatically com-
of deductive reasoning and verbal abstract thinking and putes many of the scoring formulas and converts raw
patients with focal frontal lesion exhibit deficits on this scores into standardized scores. Most traditional execu-
measure (Keil, Baldo, Kaplan, Kramer, & Delis, 2005). tive-function tests do not provide alternate forms, which
can be problematic for repeat examinations. However, the
D-KEFS Towers Test D-KEFS provides alternate forms for three of the tests that
The D-KEFS Towers Test is a modified version of other are particularly prone to practice effects (i.e., Sorting Test,
tower tasks that have been used in experimental studies. Twenty Questions Test, and Verbal Fluency Test).
The examinee is asked to build target towers by moving
two to five disks of different sizes across three pegs in as
few moves as possible while adhering to two rules: (a) Normative Sample
move only one disk at a time and (b) never placing a
larger disk over a smaller disk. The D-KEFS Tower Test The D-KEFS was standardized on a nationally representa-
assesses several aspects of executive functioning, including tive, stratified sample of 1750 children, adolescents, and
spatial planning, rule violations, and inhibition. This test adults, aged 889 years. Stratification was based on age,
has been found to be sensitive to focal frontal lesions sex, race/ethnicity, years of education, and geographic re-
(Yochim, Baldo, Kane, & Delis, 2009). gion. The 2000 U.S. census figures were used as target values
for the composition of the D-KEFS normative sample.
D-KEFS Proverb Test There were 75 175 people in each of the 16 age groups.
The D-KEFS Proverb Test was modeled after a prov- The D-KEFS sample was comprised of approximately equal
erb interpretation measure developed in the 1950s by proportions of men and women for most of the age groups;
Gorham (1956). For the D-KEFS version, an examinee is however, the older age groups had more women than men,
asked to interpret proverbs in two conditions: (a) free which is consistent with the census data. The D-KEFS
inquiry where the examinee generates his or her own sample was divided into five major educational groups
interpretations of the proverbs and (b) multiple choice used by the U.S. Census: less than or equal to 8 years,
where the examinee selects the best interpretation from 911 years, 12 years, 1315 years, and greater than or
among four alternatives. This task measures metaphorical equal to 16 years. For examinees between the ages of
thinking and provides a means for comparing generation 8 and 19, the mean parental education was substituted.
versus comprehension of abstract verbal information.
This test has been found to be sensitive to frontal-lobe
Historical Background
dysfunction (McDonald, Delis, Norman, Tecoma, &
Iragui-Madozi, 2008)
Most of the D-KEFS tests were developed in the late 1980s
and early 1990s by Delis and colleagues, with the entire set
Administration and Scoring of D-KEFS instruments assembled in 1994. The prelimi-
nary measures underwent a try-out study of approxi-
Instructions for administration are given in the stimulus mately 300 normative subjects and 50 mixed neurological
booklet or record form. The required materials, discon- patients, and modifications were made to improve the
tinued rules, time limits, and standardized prompts are reliability and validity of the measures.
DelisKaplan Executive Functioning System D 799
Kiang, Light, Prugh, Coulson, Braff, & Kutas, 2007), and Kaplan executive function system sorting test. Journal of Clinical and
Experimental Neuropsychology, 31(5), 624631.
in children with heavy prenatal alcohol exposure (Matt-
Floyd, R. G., McCormack, A. C., Ingram, E. L., Davis, A. E., Bergeron, R.,
son, Goodman, Caine, Delis, & Riley, 1999; Schonfield, & Hamilton, G. (2006). Relations between the Woodcock-Johnson
Mattson, Lang, Delis, & Riley, 2001). III clinical clusters and measures of executive functions from the
Delis-Kaplan executive function system. Journal of Psychoeducational
Assessment, 24, 303317.
Cross References Gorham, D. R. (1956). Use of the proverbs test for differentiating schizo-
phrenics from normals. Journal of Consulting Psychology, 20(6),
435440.
California Verbal Learning Test (California Verbal Heaton, R. K., Chelune, G. J., Talley, J. L., Kay, G. G., & Curtiss, G. (1993).
Learning Test-II) Wisconsin card sorting test manual - Revised and expanded. Odessa,
Category Test FL: Psychological Assessment Resources.
Design Fluency Test Homack, S., Lee, D., & Riccio, C. A. (2005). Delis-Kaplan executive
function system (test review). Journal of Clinical and Experimental
Executive Functioning
Neuropsychology, 27, 599609.
Frontal Lobe Syndrome Huey, E. D., Goveia, E. N., Paviol, S., Pardini, M., Krueger, F.,
Stroop Color Word Test, Adult Zamboni, G., et al. (2009). Executive dysfunction in frontotemporal
Trail Making Test dementia and corticobasal syndrome. Neurology, 72(5), 453459.
Verbal Fluency Jefferson, A. L., Poppas, A., Paul, R. H., & Cohen, R. A. (2007). Systemic
hypoperfusion is associated with executive dysfunction in geriatric
Wisconsin Card Sorting Test
cardiac patients. Neurobiology of Aging, 28, 477483.
Jones-Gotman, M., & Milner, B. (1977). Design fluency: The invention of
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Delusion D 801
temporal lobe epilepsy: Utility of a multi-level design fluency test. beliefs are sustained despite incontrovertible evidence to
Neuropsychology, 19, 806813.
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Psychiatric disorders including psychotic disorders
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patients with frontal lobe epilepsy and temporal lobe epilepsy: rela- disorder), mood disorders (bipolar disorder and de- D
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Parmenter, B. A., Zivadinov, R., Kerenyi, L., Gavett, R., Weinstock-
Guttman, B., Dwyer, M. G., et al. (2007). Validity of the Wisconsin
Neurodegenerative disorders such as Alzheimers dis-
Card sorting and Delis-Kaplan executive function system (D-KEFS) ease, frontotemporal dementia, dementia with Lewy
sorting tests in multiple sclerosis. Journal of Clinical and Experimen- bodies, Huntingtons disease, basal ganglia calcifica-
tal Neuropsychology, 29, 215223. tion, and multiple sclerosis.
Partington, J. E., & Leiter, R. G. (1949). Partingtons pathway test. The Other central nervous system disorders including
Psychological Service Center Bulletin, 1, 920.
Reitan, R. M., & Wolfson, D. (1993). Halstead-Reitan neuropsychological
brain tumors, particularly temporal lobe and deep
battery. Tuscon, AZ: Neuropsychology Press. hemispheric tumors, epilepsy, subdural hematomas,
Schonfield, A. M., Mattson, S. N., Lang, A. R., Delis, D. C., & Riley, E. P. anoxic brain injuries, and fat embolism.
(2001). Verbal and nonverbal fluency in children with heavy prenatal Metabolic conditions including hypercalcemia, hypo-
alcohol exposure. Journal of Studies on Alcohol, 62, 239246.
natremia, hypoglycemia, uremia, hepatic encephalop-
Stroop, J. R. (1935). Studies of interference in serial verbal reaction.
Journal of Experimental Psychology, 18, 643662.
athy, and porphyria.
Werner, H., & Kaplan, E. (1952). The acquisition of word meanings: A Vascular conditions such as atherosclerotic vascular
developmental study. Monographs of the Society for Research in Child disease, especially when associated with diffuse, tem-
Development,15 (Serial No. 51). United Kingdom: Blackwell poroparietal or subcortical lesions, hypertensive en-
Publishing.
cephalopathy, subarachnoid hemorrhage, and
Yochim, B., Baldo, J., Nelson, A., & Delis, D. C. (2007). D-KEFS Trail
Making Test performance in patients with lateral prefrontal cortex
temporal arteritis.
lesions. Journal of the International Neuropsychological Society, 13, Infectious diseases such as human immuno-
704709. deficiency virus/acquired immune deficiency syn-
Yochim, B. P., Baldo, J. V., Kane, K. D., & Delis, D. C. (2009). D-KEFS drome, CreutzfeldtJakob disease, syphilis, malaria,
Tower Test performance in patients with lateral prefrontal cortex
and acute viral encephalitis.
lesion: The importance of error monitoring. Journal of Clinical and
Experimental Neuropsychology, 31(6), 658663.
Endocrinopathies such as Addison disease, Cushing
syndrome, hyperthyroidism or hypothyroidism, and
panhypopituitarism.
Vitamin deficiencies including vitamin B-12 deficiency,
Delusion folate deficiency, thiamine deficiency, and niacin
deficiency.
A NTHONY C. RUOCCO 1, FARZIN I RANI 2 Medications including adrenocorticotropic hor-
1 mones, anabolic steroids, corticosteroids, cimetidine,
University of Illinois at Chicago
Chicago, IL, USA antibiotics (cephalosporins, penicillin), disulfiram,
2 and anticholinergic agents.
University of Pennsylvania
Philadelphia, PA, USA Toxins such as mercury, arsenic, manganese, and
thallium.
Synonyms Categorization
Psychotic misconception
Delusions are usually categorized according to the follow-
ing themes:
Description Other delusions may involve themes of guilt, jealousy,
or religion. Delusions may also be classified as bizarre or
A delusion is an erroneous belief typically associated with non-bizarre. Bizarre delusions are implausible beliefs,
misinterpretation of perceptions or experiences. These which are incomprehensible and are not associated with
802 D Delusion
typical life experiences (e.g., person believes tiny robots psychotic features, as they have a longer time to recovery
are recording his or her conversations by installing micro- and are more likely to be treated with antipsychotic med-
phones in his or her teeth). Bizarre delusions are asso- ication (Maj, Pirozzi, Magliano, Fiorillo, & Bartoli, 2007).
ciated with schizophrenia and its spectrum disorders.
Non-bizarre delusions are plausible, understandable, and
consistent with a persons daily experiences (e.g., person Neuropsychology of Delusion
believes his or her spouse is unfaithful). Delusions can
be systematized or organized around a common theme. A delusion is a symptom of psychosis, which may be
Delusions may be congruent with the persons mood (e.g., present in a variety of neuropsychiatric disorders, including
person believes he or she can walk on water during the delirium, dementia, brain injury, and psychiatric disorders.
course of a manic episode) or the content of the delusion One prominent theory of the neuropathogenesis of delu-
can also be incongruent with the persons mood (e.g., sions and hallucinations in schizophrenia is a reactive
person believes he or she is dead but is not depressed). synaptic regeneration in brain regions, such as the frontal
cortex, which receive degenerating temporal lobe connec-
tions (Stevens, 1992). These connections might result in
Epidemiology spontaneous, stimulus-independent retrieval of episodic
memories that resemble delusions. Positive symptoms of
A World Health Organization survey of first-contact inci- schizophrenia, including delusions, share minimal rela-
dence of schizophrenia in 10 countries found the incidence tions with neuropsychological function, whereas cogni-
of referential and persecutory delusions to be the most tive deficit is more commonly associated with negative
common (Sartorius et al., 1986). Delusional thinking symptoms (e.g., deficiencies in emotional responsiveness,
may be quasidimensional in nature (i.e., both continuous spontaneous speech, and volition) (OLeary et al., 2002).
and dichotomous), ranging from overvalued ideas on one Several brain structures have been associated with delu-
hand to frank psychosis on the other. The prevalence of sions in a variety of neuropsychiatric disorders. Delusions
delusional beliefs in nonclinical samples is estimated at in Alzheimers disease are correlated with lower gray mat-
between 1 and 3 percent, which may vary depending on ter density in the left frontal lobe, right frontoparietal
the content of the delusion and the demographic charac- cortex, and left claustrum (Bruen, McGeown, Shanks, &
teristics of the sample (for a review, see Freeman, 2007). Venneri, 2008). Lower prefrontal cortex volumes are
related to delusions in older adults with depression
(Kim et al., 1999). In patients with schizophrenia, de-
Natural History, Prognostic Factors, lusional symptoms have been associated with smaller
Outcomes right planum temporale volumes (Yamasaki et al., 2007).
Delusion. Table 1
Theme Description
Persecutory Belief of being tormented, followed, tricked, spied on, or ridiculed
Referential Belief that certain gestures, comments, passages from books, newspapers, song lyrics, or other
environmental cues are specifically directed at the patient
Grandiose Belief about exaggerated power, knowledge, importance, or a special relationship to a deity or famous person D
Somatic Belief that there is a change or disturbance in ones body appearance or functioning
Control Belief that ones feelings, impulses, thoughts, or actions are under the control of some external force
Thought Belief that ones thoughts are audible to others
broadcasting
Thought insertion Belief that thoughts which are not ones own have been inserted into his or her mind
Thought Belief that ones thoughts have been removed from his or her mind
withdrawal
Erotomanic Belief that another person is in love with him or her
schizophrenia in different cultures. A preliminary report on the initial 1. Multiple cognitive deficits are present, including the
evaluation phase of the WHO Collaborative Study on determinants of
following:
outcome of severe mental disorders. Psychological Medicine, 16,
909928.
(a) Memory impairment (new learning or recall)
Stevens, J. R. (1992). Abnormal reinnervation as a basis for schizophre- (b) One or more of aphasia, apraxia, agnosia, or
nia: A hypothesis. Archives of General Psychiatry, 49, 238243. executive dysfunction
Yamasaki, S., Yamasue, H., Abe, O., Yamada, H., Iwanami, A., et al. 2. Cognitive deficits significantly impair social or occu-
(2007). Reduced planum temporale volume and delusional beha-
pational functioning and reflect a significant decline
viour in patients with schizophrenia. European Archives of Psychiatry
and Clinical Neuroscience, 257, 318324.
from a previous level of higher functioning
3. Cognitive deficits are not exclusively present during a
delirium
4. Cognitive deficits cannot be better attributed to
another Axis I disorder, such as depression or schizo-
Dementia phrenia (Rabin, Wishart, Fields, & Saykin, 2006)
While the etiology of every type of dementia is not
K ATHRYN V. PAPP
fully understood, there are a wide variety of causes
The University of Connecticut
including vascular changes, inherited genes, vitamin
Storrs, CT, USA
deficiencies, prions, and viruses. The causes of demen-
tia may not be mutually exclusive, with a number of
pathologies contributing to a patients symptoms. Mul-
Synonyms
tiple types of dementia have been described, which
roughly fall into two groups that exemplify the initial
Senility
area of degeneration: Cortical versus subcortical.
Dementias are more easily distinguished from one an-
other in early stages but become increasingly difficult
Short Description or Definition
to differentiate as pathology becomes more global. The
time period from diagnosis to death involves interindi-
Dementia is characterized by a progressive decline in mul-
vidual variation and variation based on the dementias
tiple domains of cognitive functioning which ultimately
etiology. Mortality can be as short as 2 months after
interferes with independent daily living and results in a
symptom onset in AIDS dementia and Creutzfeldt
shortened lifespan. It is not a disease or a disorder in itself
Jakob disease and as long as 20 years in Alzheimers
but a descriptive symptom complex with a number of
disease (AD).
potential underlying pathologies and/or traumas which
result in deficits in multiple domains. It is largely a diagno-
sis of the elderly, but certain forms such as Multiple sclero- Categorization
sis and Huntingtons disease can appear in middle age.
Variations such as CreutzfeldtJakob disease and head Dementia can be broadly characterized as Cortical (origi-
trauma, which results in a nonprogressive dementia, can nating in cortical areas; characterized by intellectual and
occur at any age. In progressive dementia, early symptoms memory dysfunction, aphasia, agnosia, and apraxia) ver-
can include memory complaints (forgetting names and sus Subcortical (originating in frontosubcortical systems
appointments) and difficulty in learning new material. As such as brainstem, thalamus, and basal ganglia; initial
dementia progresses, complaints may include difficulties motor dysfunction and cognitive slowing, with language
with expressive and receptive language, impairments in and memory the last faculties affected). These distinctions
executive function (poor judgment, difficulty planning), are not necessarily mutually exclusive and there is much
an inability to remember already learned information overlap (Table 1).
(such as names of family members), and changes in per- Until approximately 1015 years ago, dementia was
sonality. Dementia patients ultimately require full nursing largely subdivided into nonspecific atrophy, Parkinsons
care in the last stages of disease; they suffer from inconti- disease, dementia with Lewy bodies, AD, and Picks
nence, motor inability, echolalia, paraolia, and the return of disease. Since then, an explosion in both research and
primitive reflexes. A common cause of death is infection. imaging techniques as well as a more thorough study of
The general characteristics of dementias (as outlined in the cognitive deficits earlier in the disease course has
detail for specific dementias in the DSM-IV-TR) are: shown that these categorizations may oversimplify the
Dementia D 805
differential etiology and neuropathology of dementia. Ott, Breteler, Bots, & Slooter, 1997); high stress (Pope,
Further research may parse out further categories and Shue, & Beck, 2003); exposure to aluminum, copper, and
stages of dementia in the future. iron in the water supply (Christen, 2000); and occupa-
tional exposure to fumigants and/or defoliants (Tyas,
Manfreda, Strain, & Montgomery, 2001).
Epidemiology Several protective factors have also been explored,
with varying degrees of empirical support, including
AD is the most common form of dementia with estimates high educational attainment (Ott, Van Rossum, Van
that account for 5080% of dementia cases. Other com- Harskamp, Van de Mheen, Hofman, & Breteler, 1999),
mon forms include Vascular dementia, which accounts strong social networks (Fratiglioni, Paillard-Borg, &
for 15% of cases, and dementia with Lewy Bodies which, Winbald, 2004), participation in leisure activities
accounts for 20% of all incidence (McKeith, Galasko, (Scarmeas, Levy, Tang, Manly, & Stern, 2001; Verghese,
Kosaka, Perry, Dickson, & Hansen, 1996). Less prevalent Lipton, Katz, Hall, Derby, & Kuslansky, 2003), exercise
are Parkinsons disease, progressive supranuclear palsy (Colcombe & Kramer, 2003), the use of nonsteroidal anti-
(PSP), Picks disease, frontotemporal dementia, normal inflammatory drugs, the use of antioxidants (Fotuhi et al.,
pressure hydrocephalus, and Wernicke-Korsakoff s syn- 2008), and diets with high levels of vitamins B6, B12, and
drome. Relatively rare dementias are prion diseases such folate (Tyas et al., 2001).
as CreutzfeldtJakob disease which occurs in one in one
million (Kapur, Abbott, Lowman, & Will, 2003).
Vascular dementia is more common in Japan versus Natural History, Prognostic Factors,
AD although the overall dementia rates are equal (Otsuka, Outcomes
1994; Seno, Ishino, Inagaki, Iijima, Kaku, & Inata, 1999).
It is unclear if this difference occurs because of different Dementia was long considered a natural part of aging
diagnostic trends or environmental and/or gene pool dif- (Berchtold & Cotman, 1998). The term was first used in
ferences. AD is particularly prevalent in Israeli Arabs a clinical context by Pinel and Esquirol in the eighteenth
(Wertman, Brodsky, King, Bentur, & Chekhmir, 2007) century (Berchtold & Cotman; Cummings & Benson,
and less prevalent in China (Liu, Guo, Zhou, & Xia, 1992); however, the link between neuropathology and
2003) and India (Ferri et al., 2005). dementia was not made until 1907 by Alois Alzheimer.
The prevalence of dementia in developed countries is A date considered as the beginning of the modern study of
1.5% in people aged 65. The incidence of dementia dementia is 1968 which marks the publication by Blessed
increases dramatically with age, doubling every 5 years et al. of an article linking cognition with histopathology.
after age 65 (Kukull & Ganguli, 2000). The largest risk The general prognosis for dementia is poor, although
factors are increasing age and familial history. Those if caught early, WernickeKorsakoff s dementia, Normal
diagnosed with mild cognitive impairment (MCI) or Pressure Hydrocephalous, and Wilsons dementia can be
Downs syndrome and those with a history of traumatic treated with relative success. The disease course from
brain injury, stroke, or migraine are at greater risk. A diagnosis to death is quite variable with Creutzfeldt
variety of lifestyle risk factors have been studied and Jakob disease, resulting in death within a few weeks for
variably implicated, including vascular symptoms asso- some patients; Lewy body dementia averaging 3 years to
ciated with hypertension, obesity, and diabetes (Hofman, mortality; and AD from 5 to 20 years.
806 D Dementia
Neuropsychology and Psychology of (Kelley & Petersen, 2007). Petersen et al. (2001) divided
Dementia MCI into categories to reflect a subgroup of patients with
episodic memory complaints who convert to AD at a
Differential Diagnosis greater rate: Amnestic (primary memory complaint) ver-
sus nonamnestic (complaints can include language, atten-
Memory impairment is necessary for a diagnosis of tion/executive function, and visuospatial skills). Those
dementia; however, it must be accompanied by an diagnosed with Amnestic MCI (MCIa) progress to AD D
impairment in at least one more cognitive domain, to be at a rate of 1015% per year in comparison with a rate of
distinguished from Amnestic Disorder. It must also be 12% per year in healthy elderly (Petersen et al).
distinguished from Delirium which can include deficits Presentations typical of cortical and subcortical
in multiple cognitive domains including memory; dementias are outlined in Table 2.
however, delirium primarily presents with decreased The ability to distinguish between dementias is greatest
awareness and altered consciousness. Delirium is also at the early stages of the disease. Late stages of dementia
characterized by abrupt onset and fluctuating course present similarly as deterioration becomes profuse. These
whereas dementia onset is usually insipid and progressive. mid- and late-stage dementia symptoms can include per-
Depression is comorbid in the early stages of a number of sonality changes with delusions of persecution, comabtive-
dementias, but because Depression itself can present as ness, agitation (especially at night which is termed
cognitive impairment, it must be ruled out as the primary sundowning effect), gait disturbances, and delirium.
diagnosis. Depression can also be a predictor of later
dementia; following the cognitive changes of an individu-
al will enable the practitioner to make the most accurate Evaluation
diagnosis (Cummings, 2003).
Dementia must also be distinguished from the cogni- The goals of a neuropsychological evaluation for dementia
tive changes (such as lowered speed of processing and are obtaining baseline measures of functioning to provide a
changes in attention) which are seen in normal aging. benchmark of change over time, determining areas of
Recent research has also outlined the state between nor- strength and weakness, assisting in making a diagnosis
mal aging and dementia. The criteria for diagnosis of MCI and providing treatment recommendations, and determin-
include (1) cognitive complaint (usually memory), pref- ing the validity of a prior diagnosis or the effectiveness of a
erably corroborated by an informant; (2) cognitive im- treatment/intervention. Diagnosis of dementia involves a
pairment (usually memory), for age and education; (3) detailed patient history, a clinical exam, and radiology to
essentially normal general cognitive function; (4) largely detect structural or electrical abnormalities in the brain.
preserved activities of daily living and (5) no dementia The clinical exam covers the following domains: (1) general
awareness and cooperativeness; (2) function of cranial status is equivocal. Side effects include mild psychotic
nerves; (3) motor function; (4) reflexes; (5) sensory func- symptoms such as visual hallucinations, paranoid delu-
tion; and (6) mental status. sions, vivid dreams, confusional states, and dyskinesias.
Selegiline, a monoamine oxidase (MAO) inhibitor has
been used to extend the efficacy of L-Dopa (Nutt, Ham-
Treatment
merstad, & Gancher, 1992). Anticholinergic medications
have been used to treat motor effects, but they tend
Cortical Dementia
to have adverse effects on some executive functions
(Glatt & Koller, 1992). Deep brain stimulation or lesion-
Current treatments for cortical dementias are symptom-
ing in the globus pallidus, subthalamic nucleus, or ventral
atic. Cholinesterase inhibitors are used to treat AD and
intermediate thalamic nucleus has been used in patients
Lewy body dementia as they enhance cholinergic function
with no sign of cognitive symptoms and difficulty in
early in disease course (often up to 4 years). However, this
managing medication (Eskandar, Cosgrove, & Sinobu,
class of drug confers less benefit on cognition as choliner-
2001). When successful, these treatments result in im-
gic cell death increases throughout the disease progres-
proved motor function as well as increased psychomotor
sion. Currently used anticholinesterase inhibitors include
speed and working memory (Pillon, Ardouin, & Damier,
Reminyl (galantamine), Aricept (Donepezil), and Exelon
2000).
(rivastigmine). Side effects include gastrointestinal diffi-
Treatment options for Huntingtons are limited to
culties, particularly anorexia, nausea, and vomiting, and
palliative care. Neuroleptic drugs have been used to relieve
serious side effects which occur in 1% of patients are
choreic movements, however, they tend to promote
gastrointestinal ulceration, gastrointestinal hemorrhage,
Parkinson-like symptoms (Lerner & Whitehouse, 2002).
and mild pancreatitis. Also reported are diarrhea, vivid
PSP does not respond to dopamanergic or anticholin-
dreams, cramps, and dizziness. Cholinesterase inhibitors
ergic drugs despite its shared features with Parkinsons
may decrease heart rate and increase shortness of breath
disease (Kompoliti, Goetz, & Litvan et al., 1998). Anti-
in patients with asthma. Patients who respond with im-
depressants have been used to treat its emotional
provement in both cognitive and behavioral symptoms,
symptoms.
while taking a cholinesterase inhibitor are more likely to
have dementia with Lewy bodies rather than AD
(McKeith, 2002). Disease-modifying drugs, some of Cross References
which are in human trials, continue to be investigated
(Cummings, Vinters, Cole, & Khachaturian, 1998). Alzheimers Dementia
Patients with comorbid depression are also treated with Alzheimers Disease
antidepressants, and those with psychotic symptoms such Subcortical Dementia
as frequent hallucinations and delusions are treated with Vascular Dementia
antipsychotic agents. In the earlier stages of dementia,
patients might benefit from supportive therapy or group
therapy as well as environmental adjustments; however, as References and Readings
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Hansen, L. A. (1996). Consensus guidelines for the clinical and
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Dementia of Frontal Lobe Type
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Pillon, B., Ardouin, C., & Damier, P. (2000). Neuropsychological changes Frontal Temporal Dementia
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Neurology, 55, 411418. Picks Disease
810 D Dementia Rating Scale-2
Historical Background
Dementia Rating Scale-2
The original version of the DRS was developed by Mattis in
S TEPHANIE G RIFFITHS 1, E LISABETH M. S. S HERMAN 2 the early to mid-1970s so that clinicians and researchers
E STHER S TRAUSS 3 could evaluate patients who suffered from organic mental
1
Simon Fraser University syndromes. Although the initial descriptions of the de-
Burnaby, British Columbia, Canada mentia rating scale included relatively small samples of
2
University of Calgary cognitive impaired patients, considerable psychometric ev-
Calgary, Alberta, Canada idence supporting its utility as a dementia-screening tool
3
University of Victoria has accumulated in the intervening decades (e.g., Strauss
Victoria, British Columbia, Canada et al., 2006). The test revision (DRS-2) has not changed
any of the original DRS items, but the clarity of scoring
instructions has improved and extensive new normative
Synonyms data are available. An alternate version (DRS-2: Alternate
Form) with a new item content has also been developed
DRS-2; Mattis dementia rating scale (Schmidt & Mattis, 2004) for use in serial assessments.
Psychometric Data
Description
Estimates of internal consistency (Cronbachs alpha) vary
The Dementia Rating Scale-2 (DRS-2) is the most recent by subscale, with total score, construction, conceptualiza-
edition (2001) of a battery-style assessment of mental tion, and memory falling above 0.70, attention falling
status in older patients with suspected dementia. The above 0.65, and initiation/perseveration at approximately
DRS-2 consists of 24 brief subtests whose scores are com- 0.45 (Smith et al., 1994). The estimated alternate form
bined into five subscales of attention, initiation/persever- reliability for the DRS-2 total score falls above 0.80, and
ation, construction, conceptualization, and memory (for ranges from 0.66 (initiation/perseveration) to 0.80 (Mem-
details, see Strauss, Sherman & Spreen, 2006). In addition ory) for subscale scores (Schmidt et al., 2005). Practice
to quantifying impairment in these specific cognitive effects appear to be reduced when the alternate form
domains, the sum of all five subscale scores (out of a is given. Numerous studies report strong correlations
possible total of 144 points) yields an estimate of overall between DRS total scores and other mental status evalua-
dementia severity. As subtest items are arranged and tions, such as the Mini Mental State Exam (MMSE),
administered in order of decreasing difficulty, patients and the DRS has demonstrated adequate sensitivity and
who successfully complete the initial items on any sub- specificity for cognitive impairment in a variety of sam-
scale can be given credit for adequate performance on the ples (Strauss et al., 2006). However, the validity of specific
entire subscale. subscales is not as consistently supported (especially for
Historically, clinical cutoff scores were used to construction, attention, and initiation/ perseveration)
identify individuals with cognitive impairment. More and subscale scores should be interpreted with caution.
recently, normative data for the DRS were collected
through the Mayo Older Americans Normative Study Clinical Uses
(MOANS; Lucas et al., 1998). As the DRS-2 items and
subscales are identical to those of the DRS, the MOANS Like the original version, the DRS-2 provides screening
normative data can be used to calculate age-corrected information regarding mental status. As such, it does not
scaled scores (M = 10, SD = 3) and percentile rank provide a comprehensive evaluation of cognitive func-
equivalents for subscale and total scores (see also Strauss tioning and, if impairment is evident, patients should be
et al., 2006). Administration time for the entire battery referred for a full neuropsychological evaluation. The
ranges from 10 to 15 minutes for healthy older individuals DRS-2 has the advantage of removing floor effects for
to 3045 minutes for those with dementia. This instru- some lower functioning patients who would be difficult
ment facilitates comprehensive screening of mental status to test using other cognitive batteries. A corresponding
in patients whose cognitive impairments preclude the use disadvantage is its potential insensitivity to cognitive def-
of more demanding tests. icits, or ceiling effects, in high-functioning individuals.
Dementia with Lewy Bodies D 811
As a screening instrument, the DRS accurately differ- Jurica, P. J., Leitten, C. L., & Mattis, S. (2001). Dementia Rating Scale-2.
Odessa, FL: Psychological Assessment Resources.
entiates individuals with probable Alzheimers dementia
Knox, M. R., Lacritz, L. H., Chandler, M. J., & Cullum, C. M. (2003).
from cognitively normal elderly adults, and is capable of Association between Dementia Rating Scale performance and neu-
detecting both early signs of dementia (Knox et al., 2003) rocognitive domains in Alzheimers disease. The Clinical Neuropsy-
and cognitive impairment in low-functioning samples chologist, 17(2), 216219.
(Das et al., 1995). The DRS also allows clinicians to assess Lucas, J. A., Ivnik, R. J., Smith, G. E., Bohac, D. L., Tangalos, E. G.,
Kokmen, E., Graff-Radford, N. R., & Petersen, R. C. (1998). Norma-
the initial severity of cognitive impairment in the elderly D
tive data for the Mattis Dementia Rating Scale. Journal of Clinical
and, particularly with its alternate version, can be used to and Experimental Neuropsychology, 20(4), 536547.
track the progression of dementia over time. There is Plehn, K., Marcopulos, B. A., & McLain, C. A. (2004). The relationship
evidence that patterns of performance on the subscales between neuropsychological test performance, social functioning,
of the DRS permit clinicians to discriminate between and instrumental activities of daily living in a sample of rural older
adults. The Clinical Neuropsychologist, 18(1), 101113.
common forms of dementia such as AD, Huntingtons
Rilling, L. M., Lucas, J. A., Ivnik, R. J., Smith, G. E., Willis, F. B., Ferman,
disease (HD), Parkinsons disease (PD), and vascular de- T. J., Petersen, R. C., & Graff-Radford, N. R. (2005). Mayos Older
mentia (e.g., Cahn-Weiner et al., 2002). Further, DRS African American Normative Studies: Norms for the Mattis Demen-
total scores appear to predict adaptive functioning in tia Rating Scale. The Clinical Neuropsychologist, 19(2), 229242.
community-dwelling elderly (e.g., Plehn et al., 2004) Schmidt, K., & Mattis, S. (2004). Dementia Rating Scale-2: Alternate form.
Lutz, FL: Psychological Assessment Resources.
and are related to objective indicators (MRI) of cortical
Schmidt, K. S., Mattis, P. J., Adams, J., & Nestor, P. (2005). Alternate-form
atrophy (Fama et al., 1997). reliability of the Dementia Rating Scale-2. Archives of Clinical Neu-
The MOANS DRS-2 normative data has been criti- ropsychology, 20(4), 435441.
cized for over-sampling highly educated Caucasians living Smith, G. E., Ivnik, R. J., Malec, J. F., Kokmen, E., Tangalos, E. G., &
in urban areas, thus, underestimating the abilities of indi- Petersen, R. C. (1994). Psychometric properties of the Mattis
Dementia Rating Scale. Assessment, 1(2), 123131.
viduals with lower education and socioeconomic status or
Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of
of minority ethnicities. To address this concern, new age- neuropsychological tests. New York: Oxford University Press.
and education-corrected data from a large group of
AfricanAmerican community volunteers (Mayo African
American Normative Studies, MOAANS) have been
provided by Rilling and colleagues (2005). Like the Dementia Scale (DS)
MOANS data, these DRS norms may be extended to
the DRS-2. Blessed Dementia Scale
Cross References
Dementia Syndrome of
Alzheimers Dementia
Frontal Temporal Dementia
Depression
Mental Status Examination
Subcortical Dementia
Vascular Dementia
Dementia with Lewy Bodies. Table 1 Revised criteria for the clinical diagnosis of probable or possible DLB (Adapted from
McKeith et al., 2005)
Suggestive
Central features Core features features Supportive features
Cognitive Progressive dementia Fluctuating cognition
with impairments in with pronounced
(a) memory (b) attention changes in attention
(c) executive functioning and alertness
(d) visuospatial ability
Psychiatric Recurrent, detailed Neuroleptic (a) Nonvisual hallucinations
visual hallucinations sensitivity (b) delusions (c) depression
Behavioral/ Parkinsonism REM sleep (a) Autonomic dysfunction
somatic disturbance (e.g., orthostatic hypotension, urinary
incontinence) (b) repeated falls and
syncope (c) transient, unexplained
loss of consciousness
Neuroimaging SPECT/PET: Low (a) CT/MRI: Relative preservation of
dopamine medial temporal lobe (b) SPECT/PET:
transporter Generalized low uptake with reduced
uptake in basal occipital activity (c) EEG: Prominent
ganglia slow wave activity with temporal lobe
transient sharp waves (d) MIBG
myocardial scintigraphy: Abnormally
low uptake
Note: Central features are essential for DLB diagnosis. Two or more core features are needed for probable DLB diagnosis, and one for possible DLB
diagnosis. One or more suggestive feature in addition to one or more core feature are needed for probable DLB diagnosis, and one or more
suggestive features with no core feature is sufficient for possible DLB diagnosis. Suggestive features are commonly present but not proven to have
diagnostic specificity
Dementia with Lewy Bodies D 813
the early 1970s, the term incidental Lewy body disease whether it lies on the same disease spectrum as PDD. The
began to be used. Clinical and pathological features of current guidelines regarding clinical diagnosis consider
demented patients with Lewy bodies were subsequently whether cognitive or motor symptoms are present first
detailed by a number of Japanese neuropathologists. By (McKeith et al., 2005). If cognitive symptoms appear prior
the late 1980s, it came to be recognized that DLB occurred to, or concurrently with, motor symptoms, then a diag-
much more commonly than previously suspected, as it was nosis of DLB can be made.
reported to be the second most common type of dementia Although there is also considerable similarity between D
among the elderly. With advances in histological techni- the cognitive symptoms and clinical course of DLB and
ques, LBs and Lewy neurites were identified outside of the AD, retrospective clinical assessments of pathologically
substantia nigra, including limbic and cortical areas. In confirmed DLB have revealed distinct cognitive and psy-
October of 1995, an international workshop in Newcastle, chiatric features of the disorder, with the presence of visual
England was organized to establish clinical diagnostic cri- hallucinations and visuospatial or constructional dysfunc-
teria and pathological protocols for DLB, the results of tion suggesting DLB. There is increasing consensus that the
which were published by McKeith et al. (1996). These DLB syndrome can be identified and can be differentiated
criteria were reviewed again at a 1999 international work- from other dementia syndromes in routine clinical prac-
shop (McKeith, Perry and Perry, 1999) and then most tice, prior to pathological confirmation (Lennox & Lowe,
recently revised in 2005 (see above) (McKeith et al., 2005). 1996). Differentiating between brainstem predominant,
limbic and diffuse neocortical LB pathology may also be
relevant to the clinical syndrome (McKeith et al., 2005).
Current Knowledge
A description of DLB symptoms can be found in Table 1. The original neuropathologic criteria for DLB only re-
In addition to the more typical signs of dementia, com- quired the presence of LBs somewhere in the brain of a
plex visual hallucinations accompanied by a fluctuating patient who was clinically diagnosed with dementia
course are often viewed as the tell tale indicators of DLB (McKeith et al., 1996). Other coexistent pathological fea-
(McKeith et al., 2005). Hallucinations are often accompa- tures such as amyloid plaques, neurofibrillary tangles, and
nied by delusions, anxiety, and behavioral disturbances. neuronal loss were neither inclusive nor exclusive for a
Additionally, patients with DLB often suffer from sleep diagnosis of DLB. Although this liberal definition had the
disturbances and a disruption of the day/night cycle, advantage of including many cases, the development of
which may be the result of vivid, frightening dreams that more sensitive methods for detecting LBs has lead to
occur during REM sleep. It is not uncommon for patients many neuropathologically positive cases being identified
to appear to act out their dreams by vocalizing or violently as DLB even without the clinical syndrome. The most
moving around in bed. Therefore, when assessing for DLB, recent consensus guidelines regarding neuropathological
screening questions that address sleep issues should always criteria for DLB recommend a semiquantitative grading
be utilized. With respect to the extrapyramidal motor scale (e.g., mild, moderate, severe, and very severe) for
features, patients with DLB may exhibit similar, but some- lesion density, as opposed to simply identifying the pres-
what more severe, difficulties as compared to patients with ence or absence of LB pathology (McKeith et al., 2005).
PD. These include action tremor (resting tremor is less The density of LB pathology can be highly variable, and
common in DLB), slowness of movement, postural insta- LBs can be frequently found in brainstem nuclei, as well as
bility, gait difficulty, facial immobility, and muscle rigidity. various limbic, and neocortical regions.
However, the assessment of motor features can be compli-
cated by the presence of cognitive impairment. Fluctua-
tions may be one of the most difficult clinical symptoms to Treatment
assess and inter-rater reliability is often low.
Because DLB appears to have motor symptoms, cog- The clinical management of patients with DLB can be
nitive profiles, neuropathological features, and a clinical complex and includes (a) efforts to detect and appropri-
course that are comparable to those of Parkinson disease ately diagnose cognitive impairment early on in the dis-
dementia (PDD), there is an ongoing debate in the cur- ease process, (b) assessment and management of
rent literature about whether DLB is a distinct entity or neuropsychiatric and/or behavioral symptoms, (c)
814 D Demographically-Adjusted T Score
treatment of the movement disorder, and (d) monitoring Lennox, G., & Lowe, J. (1996). The clinical diagnosis and misdignosis of
Lewy body dementia, In R. Perry, I. McKeith, & E. Perry (Eds.),
and management of autonomic dysfunction and sleep
Dementia with Lewy bodies: Clinical, pathological, and treatment
disorder. Non-pharmacologic interventions have not issues (pp. 920). Cambridge, UK: Cambridge University Press.
been systematically evaluated, but may include strategies McKeith, I. G., Dickson, D. W., Lowe, J., Emre, M., OBrien, J. T.,
that increase social interactions and increase levels of Feldman, H. et al. (2005). Diagnosis and management of dementia
arousal as a way of reducing the presence and impact of with Lewy bodies: third report of the DLB Consortium. Neurology,
65(12), 18631872.
visual hallucinations (McKeith et al., 2005). Pharmaco-
McKeith, I. G., Galasko, D., Kosaka, K., Perry, E. K., Dickson, D. W.,
logic treatments such as Levodopa can be used to help Hansen, L. A. et al. (1996). Consensus guidelines for the clinical and
alleviate the parkinsonian motor disorder associated with pathologic diagnosis of dementia with Lewy bodies (DLB): report of
DLB. However, dopaminergic medications may exacer- the consortium on DLB international workshop. Neurology, 47(5),
bate psychiatric symptoms, and as such should be started 11131124.
McKeith, I. G., Perry, E. K., & Perry, R. H. (1999). Report of the second
at low doses and increased only gradually (McKeith et al.,
dementia with Lewy body international workshop: diagnosis and
2005). Other pharmacologic interventions may include treatment. Consortium on Dementia with Lewy Bodies. Neurology,
cholinesterase inhibitors or atypical antipsychotic medi- 53(5), 902905.
cations. Cholinesterase inhibitors may specifically be use-
ful for the fluctuating cognitive impairments that are
associated with DLB, and may improve global functioning
as well as activities of daily living. Although selective Demographically-Adjusted T
serotonin reuptake inhibitors (SSRIs) or serotonin-nor-
epinephrine reuptake inhibitors (SNRIs) have been
Score
reported as the preferred pharmacologic intervention for
T Scores
depression in DLB, there have been no systematic studies
of the treatment of depression in patients with this
disorder.
Demyelination
Future Directions K ATHLEEN L. F UCHS
University of Virginia Health System
Efforts to find new ways of treating DLB have begun to Charlottesville, VA, USA
focus on early detection in hopes of finding ways of
potentially slowing the progression of the disease. For
example, practical biomarkers or disease modifying stra- Definition
tegies that target alpha-synuclein deposition would be
significant developments that hopefully will occur in the Demyelination is the process in which myelin, the protec-
near future (Goldmann Gross, Siderowf, & Hurtig, 2008). tive protein and lipid sheath around a nerve fiber, is
broken down. This impacts the efficiency of nerve con-
duction and leaves the axon vulnerable to damage and
Cross References degeneration. In multiple sclerosis, demyelination in the
central nervous system is presumed to result from im-
Alzheimers Disease
mune system activation of inflammatory processes that
Lewy Bodies (Alpha-Synuclein Inclusions)
attack myelin and inhibit repair (remyelination) by
Metabolic Encephalopathy
oligodendrocytes. Axonal damage and loss are thought
Parkinsons Dementia
to produce some of the symptoms of multiple sclerosis,
Parkinsons Disease
especially in myelin-rich fibers such as the optic nerve.
Compston, A., Lassmann, H., & Smith, K. (2006). The neurobiology American Psychiatric Association (1994). Diagnostic and statistical
of multiple sclerosis. In A. Compston et al. (Eds.), McAlpines manual of mental disorders (4th edn.). Washington, DC: American
multiple sclerosis (4th ed., pp. 449490). Philadelphia, PA: Elsevier. Psychiatric Association.
Caplan, B. (2010). Rehabilitation psychology and neuropsychology with
stroke survivors. In R. G. Frank, M. Rosenthal, & B. Caplan (Eds.),
Handbook of rehabilitation psychology (2nd edn., pp. 6394). D
Washington DC: American Psychological Association.
Prigatano, G. P., & Klonoff, P. S. (1998). A clinicians rating scale for
evaluating impaired self-awareness and denial of disability after
brain injury. The Clinical Neuropsychologist, 12(1), 5667.
Denial
DAWN E. B OUMAN
Drake Center Dentate Gyrus
Cincinnati, OH, USA
S EVERN B. C HURN
Virginia Commonwealth University
Definition Richmond, VA, USA
DNA Structure
Base pairs
Adenine Thymine D
Guanine Cytosine
Sugar phosphate
backbone
Deoxyribonucleic Acid (DNA). Figure 1 Structure of DNA. (This figure is the property of the US National Library of
Medicine (2009))
Young, 2001; Konradi et al., 2004; Kuromitsu et al., 2001), U.S. National Library of Medicine. Structure of DNA. Accessible at:
http://ghr.nlm.nih.gov/handbook/basics/dna. Accessed 23 Sep 2009.
as well as the risk for schizophrenia (Tkachev et al., 2003).
attorneys) to question fact or expert witnesses to make cerebral blood vessels. Individuals with depressed skull
decisions regarding the testimony to be presented at the fracture may develop raccoon eyes, or a battle sign that
trial. During a deposition, both attorneys and a court tips the clinician off about the presence of a skull fracture.
recorder are present, but no judge or jury is in attendance. Depressed skull fracture is common after blunt trauma to
Everything asked and answered during the deposition is the head (i.e., direct blow with a hammer or any other
transcribed by a court reporter. hard object).
There are essentially two types of depositions: Individuals with depressed skull fracture are at the
discovery deposition and trial deposition. A discovery increased risk of developing brain infection, as the brain
deposition is held by the opposing counsel and is tissue may be directly exposed to the outside environ-
intended to extract information about an experts ment. As such, emergent management of the skull frac-
opinions, refine the dispute by focusing on the most ture, with removal of bone fragment and appropriate
relevant issues, and to gain a sense of the expert witness dressing of the skull wound, is pursued, typically with
demeanor. A trial deposition (de bene esse) serves to surgical intervention.
preserve an expert witness testimony if he/she is not
able to appear at trial. A trial deposition and live trial
testimony consist of two parts: direct examination and Cross References
cross-examination. For further details on direct and cross-
examinations please see each respective entry. Battle Sign
Raccoon Eyes
Cross References Skull Fracture
Cross-Examination
Direct Examination References and Readings
Braakman, R. (1972). Depressed skull fracture: Data, treatment, and
References and Readings follow-up in 225 consecutive cases. Journal of Neurology, Neurosur-
gery, & Psychiatry, 35, 395402.
Greiffenstein, M. F. (2009). Basics of forensic neuropsychology. In Graham, D. I., Saatman, K. E., Marklund, N., Conte, V., Morales, D.,
J. Morgan, & J. Ricker (Eds.), Textbook of clinical neuropsychology. Royo, N., & McIntosh, T. K. (2006). The neuropathology of trauma.
New York: Psychology Press. In R. W. Evans (Eds.), Neurology and trauma (2nd ed., pp. 4594).
Melton, G. B., Petrila, J., Poythress, N. G., & Slobogin, C. (2007). New York: Oxford University Press.
Psychological evaluations for the courts (3rd ed.). New York: Guilford Victor, M., & Ropper, A. H. (2001). Principles of neurology (7th ed., pp.
Press. 925953). New York: McGraw-Hill.
Definition
Depression Equivalent
Depressed skull fracture is a fracture or break of the
cranial bone that results in depression of the bone frag- Masked Depression
ment into the underlying brain tissue.
Current Knowledge
Depression Without a Depression
This may result in bruising because of compression of the
underlying brain tissue, or disruption to the underlying Masked Depression
Depressive Pseudodementia D 819
Treatment
Depressive Disorder
Treatment of depression includes pharmacological and
R OBERT G. F RANK psychotherapeutic interventions. A wide range of antide-
Kent State University pressant medications thought to modify the level of neu-
Kent, OH, USA rotransmitters in the postsynaptic cleft. In particular, the
neurotransmitters serotonin, norepinephrine, and doma- D
nine have been identified as associated with depression.
Synonyms Combined treatment providing psychopharmacological
antidepressants and psychotherapy has been shown to be
Affective disorder; Depression; Emotional disorder; effective (Thase et al., 1997).
Mood disorder In the National Comorbidity Study (Kessler et al.,
2003), 51.65 of those with 12 month in major depressive
episode treatment was adequate in only 41.9% of the
Short Description or Definition
cases, resulting in only 21.7% of those with major depres-
sive disorder being treated adequately.
A depressive disorder refers to the presence of sad or
irritable mood that lasts for more than 2 weeks. In addi-
tion, the individual must experience lack or increase in
appetite with decrease or increase in weight, insomnia or References and Readings
hypersomnia, feelings of worthlessness, despair, suicidal
ideation or intent, guilt, and impaired social and occupa- American Psychiatric Association (1994). Diagnostic and statistical man-
ual of mental disorders (4th ed.). Washington, DC: American Psychi-
tional functioning. In the USA in DSM-IV, depressive
atric Association.
disorders are divided by severity and duration into American Psychiatric Association (2000). Diagnostic and statistical man-
major depressive episode and dysthymic disorder. ual of mental disorders (4th ed., text revision). Washington, DC:
American Psychiatric Association.
Kessler, R. C., Berglund, P., Demler, O., Jin, R., Koretz, D., Merikangas,
Epidemiology K. R., et al. (2003). The epidemiology of major depressive disorder:
Results from the National Comorbidity Survey Replication (NCS-
R). The Journal of the American Medical Association, 289, 30953105.
The National Comorbidity Study (Kessler et al., 1994)
Kessler, R. G., McGonagle, K. A., Zhao, S., Nelson, C. B., Hughes, M.,
reported a prevalence of 17.1% for depressive disorders. Eshleman, S., et al. (1994). Lifetime and 12 month prevalence of
Males were less affected (12.7%) than females (21.3%). DSM-III-R psychiatric disorders in the United States: Results from
Dysthymic disorder is less common, affecting 4.8% the National Comorbidity Study. Archives of General Psychiatry, 51,
of men, 8% of women, and 6.4% of the population 819.
Thase, M. E., Greenhouse, J. B., Frank, E., Reynolds, C. F., Pilkonis, P. A.,
overall. A more recent replication of the National Comor-
Hurley, K., et al. (1997). Treatment of major depression with psy-
bidity Study focusing on major depressive disorder chotherapy or psychotherapy-pharmcotherapy combinations.
(Kesser et al., 2003) found a month prevalence of 6.6% Archives of General Psychiatry, 54(11), 10091015.
of the population, or about 13.114.2 million adult
Americans. The majority (59%) of individuals with
major depressive episode had significant or very severe
role impairment.
Depressive Disorder Not
Otherwise Specified
Evaluation
Minor Depressive Disorder
The assessment and treatment of depressive disorders
relies upon clinical evaluation or self-report instruments.
Many self-report inventories allow patients to assess their
own symptoms. Also, structured clinical interviews based
on DSM-IV exist. The majority of these tools focus on Depressive Pseudodementia
major depressive episode, though some provide a blended
rating that does not equate to DSM-IV diagnoses. Pseudodementia
820 D Dermatome
Definition
Dermatome
Dermatome is the area of the skin supplied by a spinal
J OHN E. M ENDOZA sensory nerve root. Since the nerve roots come in pairs,
Tulane University Medical Center the dermatome on one side of the body is a mirror image
New Orleans, LA, USA of the other (see Fig. 1). The numbers in figure refer to the
level at which the nerve roots enter the spinal vertebrae.
Dermatome. Figure 1 Approximate distribution of the segments of the skin represented by the various dorsal roots of the
spinal cord
Design Fluency Test D 821
for nameable errors and designs that are drawn with the
wrong number of lines. Moreover, Strauss, Sherman, and Desipramine
Spreen, (2006) concluded: . . .the norms for older adults
are based on a very small sample of individuals and J OHN C. C OURTNEY
should be used with considerable caution. Thus, the Childrens Hospital of New Orleans
DFT is a sensitive measure, but it is difficult to score New Orleans, LA, USA
and the available normative data for adults are
suboptimal.
Generic Name
prolongation, hepatic failure, mania, and potential for thought to originate in multipotential cells found in
activation of suicidal ideation. the neural plate in the embryonic stage of human
development. In patients younger than 2 years, the des-
moplastic ganglioglioma presents as a large, cystic, often
Common dura-attached mass. Although it is usually of low grade,
the desmoplastic ganglioglioma can be slow-growing,
Blurred vision, constipation, urinary retention, increased infiltrating, and become fatal in its progression. Surgery D
appetite, dry mouth, diarrhea, heartburn, weight gain, is the treatment of choice, with no complimentary
fatigue, weakness, dizziness, anxiety, sexual dysfunction, treatment needed in cases of complete tumor resection.
sweating, rash, and itching.
Cross References
References and Readings
Brain Tumor
Ganglioglioma
Physicians Desk Reference (62nd ed.). (2007). Montvale, NJ: Thomson
PDR. Glioma
Stahl, S. M. (2007). Essential psychopharmacology: The prescribers guide Neoplasms
(2nd ed.). New York, NY: Cambridge University Press.
supplied the Union with certain information pertaining NLRB decisions (NLRB v. Pfizer, 1985; NLRB v. U.S. Postal
to petitioners employee psychological aptitude testing Service, 1994) uniformly recognize that discovery of psy-
program under which certain unit employees had been chological tests is restricted under Detroit Edison v. NLRB
rejected for certain job openings because of their failure to and essentially amounts to an implied federal common
receive acceptable test scores. However, petitioner re- law psychologist nondisclosure privilege. However, in
fused to release the actual test questions, the actual em- another case (Chiperas v. Rubin, 1998), the Court did
ployee answer sheets, and the scores linked with the not agree that a psychologist had no standing to quash a
names of the employees who received them, maintaining subpoena (for records) issued to someone who is not
that complete confidentiality of these materials was nec- a party to the action, unless that party (e.g., the psychol-
essary to insure the future integrity of the tests and to ogist) claims some personal right or privilege with regards
protect the examinees privacy interests. Petitioner offered to the documents sought. In other words, if the psychol-
to turn over the scores of any employee who signed a ogist does not assert a privilege, then there is no basis to
waiver releasing petitioners psychologist from his pledge object to the release of psychological test materials.
of confidentiality, but the Union declined to seek such Kaufmann (2009) provides a comprehensive review
releases. In unfair labor practice proceedings against of the issues surrounding psychological and neuropsy-
petitioner based on the Unions charge that petitioner chological test security and recommended practices and
had violated its duty to bargain collectively under 8(a)(5) legal arguments against disclosure of test materials.
of the National Labor Relations Act by refusing to pro- Some of the recommended practices include: (1) infor-
vide relevant information needed by the Union for the mal negotiation and agreement among the parties, (2)
proper performance of its duties as the employees bar- file a motion to quash the subpoena, (3) file a motion to
gaining representative the NLRB contended that all intervene as a right, and (4) consider contempt citations
the requested items were relevant to the grievance and carefully. Legal arguments include: (1) assertion of psy-
ordered petitioner to turn over all of the materials directly chological nondisclosure privilege based on state and
to the Union, subject to certain restrictions on the Unions federal law and (2) assertion of intellectual property
use of the information. The Board rejected petitioners restrictions and contractual obligations. Other proce-
request that, in order to preserve test secrecy, the tests dures include: (1) move for protective orders and (2)
and answer sheets be turned over to a psychologist select- seeking an in camera review. This elaborates upon previ-
ed by the Union. The Board and the Court of Appeals, in ous position papers from neuropsychological organiza-
its decision enforcing the Boards order, rejected peti- tions (NAN, 2000; 2003; AACN, 2003; 2007) related to
tioners claim that employee privacy and the professional issues of test security and disclosure of tests and raw
obligations of petitioners industrial psychologists should test data.
outweigh the Unions request for the employee-linked
scores. In its decision, the High Court held that the
NLRB abused its discretion when it ordered the consult- Cross References
ing I/O psychologists to release to the union standardized
test questions, answers, and results from psychological Confidentiality
aptitude tests. In Camera Review
Privilege
Current Knowledge
References and Readings
According to Kaufmann (2009), there are no U.S.
Supreme Court holdings directly addressing whether or
American Academy of Clinical Neuropsychology (2003). Official position
not psychologists must release test materials to nonpsy- of the American Academy of Clinical Neuropsychology on ethical
chologists or may refuse to release those materials. How- complaints made against clinical neuropsychologists during adver-
ever, in Detroit Edison v. NLRB, the Court spoke of the sarial proceedings. The Clinical Neuropsychologist, 17, 443445.
public policy of test security for standardized psychologi- American Academy of Clinical Neuropsychology (2007). AACN practice
guidelines for neuropsychological assessment and consultation. The
cal instruments. The Court determined that the right
Clinical Neuropsychologist, 21, 209231.
of the psychologists to refuse release of test materials American Psychological Association (2002). Ethical principles of
superseded the rights of a Union to discovery of such psychologists and code of conduct. American Psychologist, 57,
information. Additional federal appellate cases and 10601073.
Developmental Delay D 825
Bush, S. S., & Martin, T. A. (2006). The ethical and clinical practice of Current Knowledge
disclosing raw test data: Addressing the ongoing debate. Applied
Neuropsychology, 13, 125136.
Detroit Edison Co. v. NLRB, 440 U.S. 301, (U.S. 1979).
A developmental delay in a specific domain may occur in
Freides, D. (1993). Proposed standard of professional practice: Neuro- isolation or in conjunction with delays in other domains. To
psychological reports display all quantitative data. The Clinical Neu- recognize a delay and to select an appropriate intervention,
ropsychologist, 7, 234235. clinicians must first have an understanding of developmen-
Grote, C. (2005). Ethical practice of forensic neuropsychology. In
tal norms (Schroeder & Gordon, 2002). It is important to D
G. Larrabee (Ed.), Forensic neuropsychology: A scientific approach.
New York: Oxford University Press.
consider that patients may present with a developmental
Jaffe v. Redmond, 518 U.S. 1 (1996). delay as their primary or secondary area of concern, and
Kaufman, P. M. (2009). Protecting raw data and psychological tests from clinicians should be able to evaluate and recognize such a
wrongful disclosure: A primer on the law and other persuasive delay in both contexts. This is especially important in med-
strategies. The Clinical Neuropsychologist, 23, 11301159.
ical settings where a developmental delay may be secondary
National Academy of Neuropsychology (2000). Test security: Official
statement of the National Academy of Neuropsychology. Archives
to a medical condition (Stone, MacLean, & Hogan, 1995).
of Clinical Neuropsychology, 15, 383386. Assessing for the presence of developmental delays is critical
National Academy of Neuropsychology Policy and Planning Committee in making accurate diagnoses of developmental disorders
(2003). Test security: An update. Official statement of the National and formulating subsequent treatment plans. The following
Academy of Neuropsychology. See http://www.nanonline.org/NAN/
chart provides examples of developmental disorders, devel-
Files/PAIC/PDFs/NANTestSecurityUpdate.pdf.
NLRB v. Pfizer, Inc., 763 F.2d 887 (7th Cir., 1985).
opmental delays associated with them, and the age para-
NLRB v. U.S. Postal Service, 17 F. 3d 1434 (4th Cir., 1994). meters based on the DSM-IV-TR criteria (American
Psychiatric Association, 2000).
To better understand the role of developmental delays
within the context of a developmental disorder, a closer look
at a specific diagnosis is warranted. For example, as Table 1
Developmental Coordination depicts, developmental delays in the intellectual and
Disorder
Developmental Delay. Table 1
Soft Signs Developmental Developmental
disorder delays observed Age of onset
Mental Intellectual Prior to age 18
Retardation Adaptive
(MR)
Developmental Delay
Autistic Disorder Social Prior to age 3
L AURA C RAMER-B ERNESS Emotional
William Paterson University Communication/
Wayne, NJ, USA Language
Aspergers Social No age specified
Disorder Emotional
Synonyms Retts Disorder Social Typical development
Communication/ followed by onset of
Developmental retardation Language delays that may begin
as early as 5 months of
Physical
age
Motor
Definition Childhood Social Typical development
Disintegrative Emotional until at least age 2 and
Developmental delay is the failure to achieve certain Disorder loss of skills prior to
Communication/
developmental milestones at the appropriate age. Such Language
age 10
delays may occur in one or more of the following areas:
Physical
intellectual, emotional, social, motor, physical, language/
Motor
communication, and adaptive skills.
826 D Developmental Dyslexia
adaptive domains are necessary for a diagnosis of mental Short Description or Definition
retardation. As currently defined in the DSM-IV-TR
(American Psychiatric Association, 2000), an intellectual Developmental Gerstmann syndrome (Gerstmanns syn-
quotient (IQ) of less than 70 indicates an intellectual drome) is a rare disorder that has been infrequently de-
delay. The criteria for delayed adaptive skills are a signifi- scribed in the literature. The descriptions focus on four
cant impairment in two or more of the following areas: classic deficits: difficulty writing (dysgraphia or agraph-
communication, self-care/home living, social/interper- ia), difficulty with arithmetic (dyscalculia or acalculia), an
sonal skills, use of community resources, self-direction, inability to distinguish left from right, and difficulty
functional academic skills, work, leisure, health, and safe- identifying fingers (finger agnosia). Constructional dys-
ty. If this combination of developmental delays is present praxia, an inability to copy simple figures, is often includ-
prior to the age of 18, then a diagnosis of mental retarda- ed as a fifth symptom. The cause is not known, but over
tion may be made. It is important to note that there may the last 10 years, studies using advanced imaging techni-
be an overlap in certain diagnoses. For instance, a patient ques have reported the deficits to be associated with
may be diagnosed with mental retardation and autistic posterior inferior parietal lesions located on the left side
disorder. This comorbidity of disorders would then need of the brain (Miller & Hynd, 2004).
to be considered in how to best address the developmental
delays associated with each.
Characterization
Cross References
Fewer than 25 cases have been reported in the literature
Disability and, in the last 15 years, fewer than five appear to have
Intellectual Disability been described. As a result, there is not yet a consensus
about how to define the syndrome.
References and Readings It was first described as a discrete syndrome by Kins-
bourne and Warrington (Kinsbourne & Warrington,
American Psychiatric Association (2000). Diagnostic and statistical man- 1963) who reported on seven children. All presented
ual of mental disorders (4th ed., text revision). Washington, DC: with left-right confusion, finger agnosia, and construc-
American Psychiatric Association. tional dyspraxia, and about half also presented with dys-
Schroeder, C. S., & Gordon, B. N. (2002). Assessment & treatment of
calculia and dysgraphia. As that description suggests,
childhood problems (2nd ed.). New York: Guilford.
Stone, W. L., MacLean, W. E., & Hogan, K. L. (1995). Autism and mental there is some disagreement in the literature as to whether
retardation. In M. C. Roberts (Ed.), Handbook of pediatric psychology all four classic deficits must occur for a diagnosis to be
(pp. 655675). New York: Guilford Press. reached. (A similar lack of consensus applies to adult cases
as well.) At least one author has theorized that the disor-
der may present in a partial form if a child has simply
Developmental Dyslexia learned to compensate to some degree in a deficit area of
the syndrome (PeBenito, 1987).
Dyslexia
Epidemiology
Developmental Gerstmann
Syndrome No epidemiological studies of the syndrome have been
reported and some authors have stated that there is no
B ONNY J. F ORREST clear basis for considering it to be a unique syndrome
San Diego Center for Children (Miller & Hynd, 2004).
San Diego, CA, USA
Current Knowledge
Carroll, B. J., Martin, F. I., & Davies, B. (1968). Resistance to suppression Narcolepsy and depression (treatment-resistant).
by dexamethasone of plasma 11-O.H.C.S. levels in severe depressive
illness. British Medical Journal, 3, 285287.
Fountoulakis, K. N., Gonda, X., Rihmer, Z., Fokas, C., & Iacovides, A.
(2008). Revisiting the Dexamethasone Suppression Test in unipolar Side Effects
major depression: an exploratory study. Annals of General Psychiatry, D
7, 2231.
Serious
Generic Name
Additional Information
Dexmethylphenidate
Drug Interaction Effects: http://www.drugs.com/drug_interactions.html
Drug Molecule Images: http://www.worldofmolecules.com/drugs/
Brand Name Free Drug Online and PDA Software: www.epocrates.com
Gene-Based Estimate of Drug interactions: http://mhc.daytondcs.
Focalin, Focalin XR com:8080/cgi bin/ddiD4?ver = 4&task = getDrugList
Pill Identification: http://www.drugs.com/pill_identification.html
Class
Stimulant
Dextroamphetamine
Proposed Mechanism(s) of Action Amphetamine
D-Amphetamine
Dexmethylphenidate facilitates the release and blocks the
reuptake of both norepinephrine and dopamine.
Indication DFT
Attention-deficit hyperactivity disorder. Design Fluency Test
830 D Diabetes Mellitus
found that Type 2 diabetics had smaller volume gray References and Readings
matter, larger lateral ventricle volume, and a larger white
matter lesion volume than those in the controls. This Anderson, R. J., Clouse, R. E., Freedland, K. E., & Lustman, P. J. (2001).
same research group found that cognitive functioning, The prevalence of comorbid depression in adults with diabetes:
A meta-analysis. Diabetes Care, 24, 10691078.
particularly attention/executive functioning and informa-
Awad, N., Gagnon, M., & Messier, C. (2004). The relationship
tion processing speed, was inversely related to white mat- between impaired glucose tolerance, Type 2 diabetes, and cognitive
ter lesions, brain atrophy, and the presence of infarcts function. Journal of Clinical and Experimental Neuropsychology, 26, D
(Manschot et al., 2006). 10441080.
In addition to these neuropsychological issues, it is not Brands, A. M. A., Biessels, G. J., de Haan, E. H. F., Kappelle, L. J., &
Kessels, R. P. C. (2005). The effects of Type 1 diabetes on cognitive
surprising that diabetes is associated with higher levels of
performance: A meta-analysis. Diabetes Care, 28, 726735.
psychological distress than in controls (Ryan, 2001). For Brands, A. M. A., van den Berg, E., Manschot, S. M., Biessels, G. J.,
instance, in a meta-analysis, Anderson, Clouse, Freedland, Kappelle, L. J., de Haan, E. H. F., & Kessels, R. P. C. (2007). A detailed
and Lustman (2001) found that prevalence rates of depres- profile of cognitive dysfunction and its relation to psychological
sion varied widely across studies (360%, but with most distress in patients with type 2 diabetes mellitus. Journal of the
International Neuropsychological Society, 13, 288297.
studies around 20%), and that Type 1 and Type 2 diabetics
Desrocher, M., & Rovet, J. (2004). Neurocognitive correlates of Type I
were twice as likely to be depressed as the controls. The diabetes mellitus in childhood. Child Neuropsychology, 10, 3652.
prevalence of comorbid depression was higher in women Jacobson, A. M., Musen, G., Ryan, C. M., Silvers, N., Cleary, P,
than in men, in clinical versus community samples, and Waberski, B., Burwod, A., Weinger, K., Bayless, M., Dahms, W.,
when assessed via self-report versus standardized diagnos- Harth, J., & The Diabetes Control and Complications Trial/Epide-
miology of Diabetes Interventions and Complications Study Re-
tic interviews. When depression has been evaluated in
search Group. (2007). Long-term effect of diabetes and its
diabetes populations, Watari et al. (2006) found that de- treatment on cognitive function. The New England Journal of Medi-
pressed Type 2 diabetics performed more poorly than cine, 356, 18421852.
non-depressed diabetes and controls in cognitive domains Jongen, C., van der Grond, J. V. D., Kappelle, L. J., Biessels, G. J.,
of attention and executive functioning. In contrast, Viergever, M. A., & Pluim, J. P. W. (2007). Automated measurement
of brain and white matter lesion volume in type 2 diabetes mellitus.
Brands et al. (2007) found no relationship between self-
Diabetologia, 50, 15091516.
report measures of psychological distress and cognition in Manschot, S. M., Brands, A. M. A., van der Grond, J., Kessels, R. P. C.,
individuals with type 2 diabetes. Algra, A., Kappelle, L. J., & Biessels, G. J. (2006). Brain magnetic
resonance imaging correlates of impaired cognition in patients with
type 2 diabetes. Diabetes, 55, 11061113.
Ryan, C. M. (2001). Neurobehavioral disturbances associated with dis-
Evaluation orders of the pancreas. In R. E. Tarter, M. Butters, & S. R. Beers
(Eds.), Medical neuropsychology (2nd ed., pp. 127162). New York:
A variety of relatively minor cognitive deficits can be asso- Kluwer Academic/Plenum.
Watari, K., Letamendi, A., Elderkin-Thompson, V., Haroon, E., Miller, J.,
ciated with diabetes and, as such, any neuropsychological
Darwin, C., & Kumer, A. (2006). Cognitive function in adults with
measure used in this population should be quite sensitive to type 2 diabetes and major depression. Archives of Clinical Neuropsy-
cognitive impairment. Domains to be assessed by the chology, 21, 787796.
neuropsychologist should include, at minimum, verbal
memory and learning, processing speed, attention, set-
shifting, and motor functioning.
Cross References
Diaschisis Cerebral Blood Flow
Ischemic Penumbra
E LLIOT J. R OTH Ischemic Stroke
Northwestern University
Chicago, IL, USA
References and Readings
Definition Finger, S., Koehler, P. J., & Jagell, C. (2004). The Monakow concept of
diaschisis: Origins and perspectives. Archives of Neurology, 61,
Diaschisis is a sudden loss of function in a portion of the 283288.
brain connected to, but at a remote distance away from, Infeld, B., Davis, S. M., Lichtenstein, M., Mitchell, P. J., & Hopper, J. L.
(1995). Crossed cerebellar diaschisis and brain recovery after stroke.
a damaged area.
Stroke, 26, 9095.
Komaba, Y., Mishina, M., Utsumi, K., Katayama, Y., Kobayashi, S., &
Mori, O. (2004). Crossed cerebellar diaschisis in patients with
Current Knowledge cortical infarction: Logistic regression analysis to control for con-
founding effects. Stroke, 35, 472476.
The site of the area that has been injured by an acute focal
disturbance such as stroke or penetrating brain injury,
and the site of the diaschisis are connected to each other
by neurons. The loss of the damaged structure disrupts
the function of the remaining intact systems and causes
Diazepam
a physiological imbalance. These changes are most
J OHN C. C OURTNEY
pronounced during the first few days following cerebral
Childrens Hospital of New Orleans
infarction or injury. Some function may be restored with
New Orleans, LA, USA
a gradual readjustment of the otherwise intact but
suppressed areas.
Diaschisis can be classified according to the connecting
neuronal fibers involved. When the connecting fibers are
Generic Name
intra-hemispheric, the phenomenon of ipsilateral thalamic
Diazepam
or subcortical-cortical diaschisis may be seen; when they
are interhemispheric, there is transcallosal diaschisis, and if
they are cerebellar, the diaschisis is of the contralateral Brand Name
cerebellum or crossed cerebellar diaschisis. Ipsilateral tha-
lamic and crossed cerebellar diaschisis are frequently ob- Valium, Diastat
served, but have no clinical significance.
Reversal of the effects of the subcortical-cortical and
transcallosal diaschisis might explain the neuropsychologi- Class
cal and functional neuroimaging changes observed over the
first few months after the cerebrovascular incident. Anxiolytic
Dichotic Listening D 833
advantage) for spoken words (digits). Kimura (1961a, b, patients with complete hemispherectomy are able to pro-
1967) observed that patients with damage to the left cess auditory material presented monaurally but are
temporal lobe performed more poorly than patients impaired under dichotic presentation. Specifically, these
with right temporal lobe damage as well as adult control patients are able (7489% accuracy) to detect dichotic
participants. These findings were expected as patients information in the ear contralateral to the hemispherec-
with damage to the left hemisphere should have more tomy but accuracy was extremely poor (312%) for the
difficulty performing language-based tasks relative to the ear ipsilateral to the lesion (de Bode, Sininger, Healy,
other groups. Surprisingly, all groups demonstrated better Mathern, & Zaidel, 2007).
detection for information presented to the right relative to An attentional model has also been proposed to ex-
the left ear. The REA was initially unexpected because each plain the REA observed on dichotic word listening tasks.
ear projects to the contralateral and ipsilateral hemi- Kinsbourne (1975) proposed that a REA occurs because
sphere. Based on these redundant connections, each ear attention is shifted toward the contralateral side of space
should have access to the language center. Kimura rea- following engagement of a hemisphere. For example, a
soned that a REA occurred because the ipsilateral pathway REA occurs during dichotic word listening because the
is suppressed during dichotic but not monaural stimuli left hemisphere is engaged for processing verbal material,
presentation. According to her model, in patients with and the activation of the left hemisphere leads to a shift of
speech localized to the left hemisphere, information from attention toward information presented to the right side
the left ear is sent to the right hemisphere and then of space (see Springer & Deutsch, 1985 for review). In
through the cerebral commissures before gaining access support of the attentional model, a double dissociation
to the left hemisphere for processing of the verbal mate- was found in dichotic listening depending upon the type
rial. By contrast, the right ear projects to the left hemi- of material (verbal or melodic) that had to be remem-
sphere (without the additional step of crossing over the bered across trials. That is, a REA was found when parti-
cerebral commissure) that results in a REA (see Kimura, cipants had to remember a sentence across dichotic trials
1967 for review). Further support for Kimuras model of and a modest LEA (as measured by reaction time) oc-
dichotic listening comes from work done with patients curred when subjects compared melodies presented just
who have undergone the intracarotid sodium amytal pro- before and immediately following dichotic syllable pairs
cedure (Wada test) to determine which hemisphere med- (Morais & Landercy, 1977). According to the attentional
iates language. Unlike patients with language lateralized model, activation of the right hemisphere during melodic
to the left hemisphere with the typical REA, patients who processing would focus attention to the left side of space
have language lateralized to the right hemisphere showed creating the LEA.
a left ear advantage (LEA, Kimura, 1961b, 1967).
Data from patients with complete disconnection of
the hemispheres via commissurotomy have also been Psychometric Data
cited as support for Kimuras model. Split-brain patients
are able to identify spoken words presented monaurally, Strauss et al. (2006) report normative data for children
indicating that the ipsilateral pathway is intact in the (age 2 through grade 6) as well as mean scores for right-
absence of competing information. However, under dich- handed adults. The authors caution that the adult norms
otic presentation, an exaggerated REA occurs. In fact, are based upon university students and therefore are only
seven out of ten patients with a commissurotomy detected applicable for younger adults. Normative data for older
no information presented to the left ear (Milner, Taylor, & adults is not available for this particular task. There are
Sperry, 1968), and this extinction of information was also published means and standard deviations for adults rang-
described in two patients with complete disconnection of ing in age from 16 to 79, split into decades (Meyers,
the hemispheres (Springer & Gazzaniga, 1975). According Roberts, Bayless, Volkert, & Evitts, 2002) using a similar
to Kimuras model, the extinction of information occurs task with more word pairs (Dichotic Word Listening Test,
because information presented to the left ear is projected Roberts et al., 1994). Generally, normative data for dich-
to the disconnected right hemisphere. The right hemi- otic listening tasks are not presented separately for men
sphere is not specialized to process this type of verbal and women, as it is thought that gender does not signifi-
material, and this information cannot be projected to cantly influence dichotic listening performance. A recent
the left hemisphere because the corpus callosum is cut. meta-analysis of 12 studies involving a total of 3,822
Consequently, split-brain patients are not able to report subjects concluded that there were no significant differ-
hearing information presented to the left ear. Similarly, ences between sexes in terms of dichotic listening
Dichotic Listening D 835
performance (Sommer, Aleman, Somers, Boks, & Kahn, and psychiatric populations. Patients with multiple scle-
2008). rosis (MS) also show abnormal dichotic listening perfor-
Testretest reliability for dichotic listening word pairs mance. For example, relative to control participants,
is variable. Hiscock and colleagues (2000) report a reli- patients with MS have been observed to demonstrate a
ability of 0.45 with a 1 week interval between testing. This greater REA in unforced and right ear forced attention
was conducted with a sample of 340 right- and left-hand- conditions. By contrast, in the left ear forced attention
ed college students. Reliability did improve when more condition, the control group demonstrated the expected D
trials were added (0.76). Testretest reliability has been LEA, while the MS group persisted in demonstrating a
reportedly higher (0.760.92) when testing was per- REA (Gadea et al., 2002). Patients with other neurode-
formed in the same day with a smaller sample of epileptic generative diseases can also show an exaggerated REA
patients (Strauss, Gaddes, & Wada, 1987). relative to a neurologically normal comparison group.
According to Kimuras model, the ear advantage on This has been observed in patients with very mild and
dichotic word listening tasks should indicate which hemi- mild Alzheimers disease (Duchek & Balota, 2005;
sphere is dominant for language. Test validity data is Gootjes et al., 2006, Claus & Mohr, 1996). Additionally,
derived from patients who have completed both the dich- patients with Alzheimers disease and Huntingtons dis-
otic listening task and undergone the Wada test to deter- ease have been found to demonstrate a REA even under
mine which hemisphere mediates language. Hugdahl and left ear forced attention conditions, whereas demented
colleagues (1997) reported a REA for 8/10 participants and non-demented patients with Parkinsons disease are
who demonstrated left hemisphere language dominance, able to shift attention to the left ear and show the
whereas all three patients with right hemisphere language expected LEA (Claus & Mohr, 1996). Dichotic listening
dominance showed a LEA. Similarly, in a larger sample of has also been examined in patients with depression and
epileptic patients, a REA was found in the patient group schizophrenia, disorders often associated with right
with left hemisphere language dominance, whereas hemisphere dysfunction. An exaggerated REA in de-
patients with right hemisphere language dominance pressed individuals has been associated with response
reported words presented to the left ear more accurately to antidepressant treatment (see, Bruder et al., 1999 for
than patients with left or bilateral hemisphere language review), and patients with schizophrenia also show a
dominance (Strauss et al., 1987). These findings are also REA even under left ear forced attention conditions
consistent with the original studies reported by Kimura (Hugdahl et al., 2003).
(1961b, 1967) described above. High concordance has
also been found for fused dichotic word listening and Cross References
lateralized fMRI activation during a verb generation task
performed by children with epilepsy. All eight patients Auditory Cortex
with greater left than right hemisphere activation during Auditory Pathway
the verb generation task showed a REA, whereas bilateral Auditory Processing
activation was associated with a REA in three of the Auditory System
patients and no ear advantage in the other three (Fer- Intracarotid Sodium Amobarbital Test
nandes, Smith, Logan, Crawley, & McAndrews, 2006). As Split-Brain
seen above, dichotic listening performance is not always Wada Test
predictive of the hemisphere that mediates language. A
significant factor to consider is that patients who undergo
Wada testing have epilepsy or other brain abnormalities, References and Readings
which can effect brain organization and the lateralization
Bruder, G. E., Tenke, C. E., Stewart, J. W., McGrath, P. J., & Quitkin, F. M.
of different cognitive abilities.
(1999). Predictors of therapeutic response to treatments for depres-
sion: A review of electrophysiologic and dichotic listening studies.
CNS Spectrums, 4, 3036.
Clinical Uses de Bode, S., Sininger, Y., Healy, E. W., Mathern, G. W., & Zaidel, E.
(2007). Dichotic listening after cerebral hemispherectomy: Method-
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(see Historical Background and Psychometric Data). Parkinsons, and Huntingtons diseases. Acta Neurologica Scandina-
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836 D Diencephalic Seizures
Duchek, J. M., & Balota D. A. (2005). Failure to control prepotent path- Strauss, E., Gaddes, W. H., & Wada, J. (1987). Performance on a free-
ways in early stage dementia of the Alzheimers type: Evidence from recall verbal dichotic listening task and cerebral dominance deter-
dichotic listening. Neuropsychology, 19, 687695. mined by the carotid amytal test. Neuropsychologia, 25, 747753.
Fernandes, M. A., Smith, M. L., Logan, W., Crawley, A., & McAndrews, Strauss, E., Sherman, E. M. S., & Spreen, O. (2006). A compendium of
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dichotic listening and fMRI activation in frontal and temporal
lobes in children with epilepsy. Brain and Language, 96, 106114.
Gadea, M., Marti-Bonmat, L., Arana, E., Espert, R., Casanova, V., &
Pascual, A. (2002). Dichotic listening and corpus callosum magnetic
resonance imaging in relapsing-remitting multiple sclerosis with
emphasis on sex differences. Neuropsychology, 16, 275281.
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Gootjes, L., Bouma, A., Van Strien, J. W., Van Schijndel, R., Barkhof, F., &
Scheltens, P. (2006). Corpus callosum size correlates with asymmet- Hemodynamic Response
ric performance on a dichotic listening task in healthy aging but not
in Alzheimers disease. Neuropsychologia, 44, 208217.
Hayden, S., & Spellacy, F. (1978). Dichotic word listening test. Neuropsy-
chology Laboratory, Department of Psychology, University of
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Hiscock, M., Cole, L. C., Benthall, J. G., Carlson, V. L., & Ricketts, J. M.
(2000). Toward solving the inferential problem in laterality research:
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L INDA L. P HILLIPS
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Society, 6, 539547. Richmond, VA, USA
Hugdahl, K., Carlsson, G., Uvebrant, P., & Lundervold, A. J. (1997).
Dichotic-listening performance and intracarotid injections of amo-
barbital in children and adolescents. Preoperative and postoperative
comparisons. Archive of Neurology, 54, 14941500.
Synonyms
Hugdahl, K., Rund, B. R., Lund, A., Asbjrnsen, A., Egeland, J.,
Landr, N. I., et al. (2003). Attentional and executive dysfunctions Epithalamus-pineal gland, habenular nuclei; Subthalamus-
in schizophrenia and depression: evidence from dichotic listening Subthalamic nucleus, zona incerta
performance. Biological Psychiatry, 53, 609616.
Kimura, D. (1961a). Some effects of temporal-lobe damage on auditory
perception. Canadian Journal of Psychology, 15, 156165.
Kimura, D. (1961b). Cerebral dominance of the perception of verbal
Structure
stimuli. Canadian Journal of Psychology, 15, 166171.
Kimura, D. (1967). Functional asymmetry of the brain in dichotic listen- The diencephalon can be divided into four regions:
ing. Cortex, 3, 163178. epithalamus, thalamus, subthalamus, and hypothalamus.
Kinsbourne, M. (1975). The mechanism of hemispheric control of the The epithalamus constitutes a small area of the dienceph-
lateral gradient of attention. In P. M. A. Rabbitt, & S. Dornic (Eds.),
Attention and performance V (pp. 8197). London: Academic Press.
alon, located dorsally above the thalamus. It contains the
Meyers, J. E., Roberts, R. J., Bayless, J. D., Volkert, K., & Evitts, P. E. pineal, a lobular midline structure, just rostral to the
(2002). Dichotic listening: Expanded norms and clinical application. superior colliculi. Historically thought to be the seat of
Archives of Clinical Neuropsychology, 17, 7990. the soul, neurons in this single midline structure are
Milner, B., Taylor, L., & Sperry, R. W. (1968). Lateralized suppression called pinealocytes, which secrete melatonin and are
of dichotically presented digits after commissural section in man.
Science, 161, 184186.
linked with the retina through a circuitous path via the
Morais, J., & Landercy, M. (1977). Listening to speech while retaining sympathetic chain. At the base of the pineal, a stalk con-
music: What happens to the right-ear advantage? Brain and Lan- nects the structure to the remainder of the diencephalon.
guage, 4, 295308. The bilateral habenula is the other component of the
Roberts, M. A., Persinger, M. A., Grote, C., Evertowski, L. M., Springer, epithalamus, positioned rostral to the pineal and posteri-
J. A., & Tuten, T., et al. (1994). The dichotic word listening test:
Preliminary observations in American and Canadian samples.
or commissure. The two habenular nuclei are joined by a
Applied Neuropsychology, 1, 4556. commissure, and receive input from the dorsal thalamus
Sommer, I. E., Aleman, A., Somers, M., Boks, M. P., & Kahn, R. S. (2008). via the stria medullaris fibers. Output from the habenula
Sex differences in handedness, asymmetry of the planum tempor- targets the midbrain reticular formation.
ale and functional language lateralization. Brain Research, 1206, The largest portion of the diencephalon is the dorsal
7688.
Springer, S. P., & Deutsch, G. (1985). Left brain, right brain (Rev. ed.).
thalamus. It extends rostrally to the interventricular fora-
New York: W.H. Freeman. men, superiorally to the transverse cerebral fissure and the
Springer, S. P., & Gazzaniga, M. S. (1975). Dichotic testing of partial and floor of the lateral ventricles, inferiorly to the hypotha-
complete split brain subjects. Neuropsychologia, 13, 341346. lamic sulcus, and posteriorly it overlaps the midbrain.
Diencephalon D 837
Topographically, the thalamus is divided into nuclear the pituitary. The hypothalamus has three sets of nuclear
groups, designated medial or lateral based upon their groups based upon their rostrocaudal position. As with
relationship to an interposed sheet of axons called the the dorsal thalamus, both medial and lateral areas are
internal medullary lamina. Largely rostral to this lamina identified. The anterior group, arranged above the optic
is an anterior set of nuclei, collectively grouped into one chiasm, includes medially the preoptic, paraventricular,
entity and bordering the interventricular foramen. The anterior, and supraoptic nuclei. Laterally, an anterior pre-
medial division of the dorsal thalamus contains a single optic group is also identified and a broadly dispersed D
dorsomedial nucleus. Laterally, the nuclei are divided into lateral nucleus. In dorsoventral alignment with the tuber
dorsal and ventral layers. Dorsally, they include lateral cinereum (pituitary stalk) is the tuberal group of nuclei.
dorsal, lateral posterior, and pulvinar groups. Ventrally, a These include dorsomedial, ventromedial, and arcuate
more complex arrangement exists forming the ventral nuclei in the medial area. In the lateral tuberal zone, the
anterior and lateral nuclei, with the posterior group having rostral lateral nucleus extends more caudally, accompa-
a posteriolateral and posteriomedial region. More laterally nied by the lateral tuberal nuclei and tuberomammillary
arranged are the geniculate bodies, made up of a medial nuclei. Finally, the posterior nuclear group of the hypo-
auditory and lateral visual nuclear group. In one region the thalamus is formed primarily of the mammillary bodies
internal medullary lamina splits to surround two centrally and the posterior nuclear groups. Laterally, this region
embedded nuclei, the centromedian and parafascicular contains the most caudal extension of the lateral nucleus.
groups. Finally, the thalamus has two more thinly The hypothalamic nuclei are traversed by major fiber
distributed nuclear groups: the thalamic reticular nucleus bundles, including the fornix, stria terminalis, median
surrounding portions of the thalamic perimeter and the forebrain bundle, dorsal longitudinal fasciculus, and
midline nuclei, which are essentially a sheet of cells that the two major mammillary body connections, the mam-
extend from the periaqueductal gray zone to cover the millothalamic tract and mammillotegmental tract. These
midline surface of the thalamus. Major thalamic input is pathways interconnect the hypothalamic nuclei and
derived from ascending sensory and motor pathways, as represent their major input (e.g., fornix stria terminalis,
well as limbic inputs, information that is processed within median forebrain bundle)/output (e.g., mammillothala-
the thalamus. A subset of this input is routed as relay mic, mammillotegmental tracts, median forebrain
connections to specific areas of functional cortex or to bundle, and dorsal longitudinal fasciculus) routes to in-
more broadly integrative regions of association cortex. terface with other brain regions. A unique relationship
Fiber routes for input include the mammillothalamic exists between the hypothalamus and the pituitary.
tract, fornix, medial lemniscus, central tegmental tract, Through two different mechanisms, the hypothalamus
inferior colliculi, optic tract, and the white matter tracts interacts with the pituitary gland: either via direct axonal
of the prefrontal, parietal, occipital, and temporal lobes. In projections to the posterior pituitary (neurohypophysis)
this way, the thalamus acts as a gateway to influence output or through the secretion of regulatory factors at a portal
from a wide range of cortical and subcortical circuits. vein interface in the pituitary stalk (adenohypophysis).
A part of the midbrain gray matter is wedged rostrally
between the hypothalamus and internal capsule to form
the subthalamus. It lies inferior to the dorsal thalamus and Function
contains nuclear extensions from the midbrain red nucle-
us and substantia nigra as they project to the thalamus. The primary function associated with the epithalamus is
Specific cellular groups in the subthalamus include the regulation of circadian rhythm through the pineal gland.
subthalamic nucleus and the zona incerta. The subthala- The pineal is an endocrine gland, secreting high levels of
mic group is the largest and has extensive connections melatonin hormone during darkness. Melatonin provides
with the basal ganglia. Dorsal to the subthalamic nucleus a rhythm for setting the sleepwake cycle, and can affect
is the thinner zona incerta, which represents an extension gonadotrophic hormone production as a function of day-
of midbrain reticular formation. light period. It is thought that hormonally stimulated
Most ventral in the diencephalon is the hypothalamus. reproductive behavior may be optimized by linking longer
It extends along the descending portion of the third day length with sexual cycles. Although the specific func-
ventricle, rostrally to the lamina terminalis, superiorly to tion of the habenular nucleus is not fully understood, it
the hypothalamic sulcus at its border with the dorsal receives input from the stria medullaris and projects to the
thalamus; inferiorly it forms the most ventral diencepha- midbrain reticular formation, potentially mediating lim-
lic tissue, a portion extending into the posterior lobe of bic influence on brainstem output.
838 D Diencephalon
The thalamus is generally thought to function as a diencephalon. In the epithalamus, the primary target of
gateway for information transfer to the cerebral cortex. pathology is the pineal gland. Both tumor and cyst for-
The physiological firing of thalamic neurons can occur in mation can occur in the pineal. Tumors often alter the
either a tonic or bursting mode. Tonic firing permits production and release of melatonin, affecting sleepwake
point-to-point information transfer, where signals from cycle patterns and, in some cases, are correlated with
the lower levels of the CNS can pass directly to the cortex. ocular dysfunction. Since melatonin also has gonadotro-
Thalamic neurons also have a burst mode of signaling, phic effects, shifts in its production may change reproduc-
which conveys longer, rhythmic waves of depolarization, tive behavior. Non-neoplastic parenchymal cysts can
critical to functional patterns of activation such as those occur within the pineal. Although a rare occurrence,
that occur during sleep/wake cycles. Different thalamic these may reach a size that compresses the adjacent cere-
nuclear groups target specific brain regions to affect bral aqueduct and tectal plate. Such compression could
motor coordination (cortex, basal ganglia, and cerebel- restrict CSF flow, with the potential for producing
lum), autonomic integration (midbrain), hormonal regu- hydrocephaly.
lation (hypothalamus), hearing, vision, cognition, and Within the dorsal thalamus, pathology is usually
emotion (auditory, visual, and limbic cortices). linked to neuronal loss, astrocytosis, or myelin break-
Of all the diencephalic divisions, least is known about down. These can occur due to metabolic dysfunction, as
subthalamic function. The subthalamic nucleus is in WernickeKorsakoff syndrome or Parkinsons disease,
interconnected with the basal ganglia, and is part of the in the prion-induced plaque pathology of Creutzfeldt
indirect pathway between that region and the cerebral Jakob disease and sporadic fatal insomnia, or with the
cortex. At this position, the subthalamic nucleus may act characteristic spongy white matter condition generated in
to regulate inhibitory output from the basal ganglia. KearnsSayre syndrome. With the loss of neurons and
Much less is known about the zona incerta, its function axonal degeneration, abnormal thalamocortical integra-
most likely related to its continuity with the midbrain tion affects motor function and may generate spasticity,
reticular formation. epileptic seizure activity, or dementia.
Hypothalamic function is mediated by two types of Functional change in the subthalamus is thought to
interface: reciprocal connections with regions providing contribute to the muscular rigidity of Parkinsons disease.
its major afferent pathways (thalamus, septum, and brain- In that case, over activity of neurons in the subthalamic
stem), which influence the behavioral output directed by nucleus and the related globus pallidus leads to increased
those regions, and projections to brainstem autonomic inhibition of the thalamus, with reduced cortical motor
nuclei (midbrain, medulla, and spinal cord), which di- activation. Focal vascular lesions of the subthalamus can
rectly regulate visceral functions such as circadian cycles, also produce the violent involuntary limb movement seen
osmoregulation, appetite, body temperature, and fight/ with hemiballismus.
flight response. These systemic responses are also influ- Pathology in the hypothalamus/pituitary is most
enced by hypothalamic connections to the limbic system extensively described, due to the significant role of these
(via the mammillothalamic tract, fornix, and stria termi- regions in autonomic nervous system regulation and their
nalis). Two other major functions of the hypothalamus are interface with a variety of peripheral organs. For example,
the regulation of growth and reproductive cycles, con- lesions within specific hypothalamic nuclei controlling vis-
trolled through broad feedback loops, utilizing receptor- ceral functions of appetite, thirst, sleepwake cycle, regula-
mediated monitoring in both the hypothalamus and the tion of body temperature, or emotional state often result in
affected peripheral organs. Growth and reproduction are extreme shifts within the normal range of these functions.
particularly dependent upon interaction with the pituitary Many of the human responses to such hypothalamic
lobes, either via secretion of hypothalamic-generated lesions can be replicated in animal models. Functional
hormones within the lobe (neurohypophysis), or release disorders of the hypothalamus/pituitary axis can also be
of hypothalamic regulatory factors into the hypothalamic driven by abnormal metabolism. One of the best-known
hypophyseal portal system for control of pituitary hor- examples is thiamine deficiency, where microvascular
mone production (adenohypophysis). lesions occur in the mammillary bodies. These lesions
underlie WernickeKorsakoff syndrome encephalopathy,
Illness which presents as a variety of ophthalmic abnormalities
and cognitive deficits. A wide range of tumors, including
Illness resulting from structural and metabolic pathology gliomas and adenomas, are also common in the hypothal-
has been documented for all subregions of the amus and pituitary. They selectively affect the secretion of
Differential Ability Scales (DAS and DAS-II) D 839
age-matched peers. This flexibility in test administration recall of digits backward, phonological processing, and
allows for the assessment of children based on ability rapid naming.
rather than age.
Administration time for the core battery is 2565 min
for Early Years, and 4565 min for School-Age. Diagnostic Psychometric Data
subtests average an additional 1530 min of testing time.
Each subtest offers predetermined starting points based The manual states that the internal reliability for GCA is 0.90
on the childs age that eliminates items likely to be too for lower-level preschool, 0.94 for upper-level preschool, and
easy or too difficult for a child. Discontinuation rules are .95 for school-age. Internal reliabilities of subtests vary be-
based on decision points, stopping points, and basal and tween 0.70 and 0.95. Testretest reliability was reported to be
ceiling guidelines. Raw scores are converted to T-scores quite strong with GCA score reliability ranging from 0.89 to
that are then converted into standard scores. Verbal, non- 0.94. The DAS handbook includes a number of studies
verbal, and spatial cluster scores can be derived from reporting consistently high correlations of the DAS GCA
subtest standard scores. score with overall composite scores of other cognitive bat-
The DAS-II manual suggests that qualifications to teries, such as the Wechsler Preschool and Primary Scale
administer the test battery include formal graduate or of Intelligence, StanfordBinet, WoodcockJohnson, and
professional training in psychological assessment and in- Wechsler Intelligence Scale for Children.
terpretation of results. A trained technician can adminis-
ter and score the battery, but should not be involved in the
Clinical Uses
interpretation.
The DAS-II is designed to provide an understanding of
a childs definable strengths and weaknesses within a wide
Historical Background range of cognitive abilities. The DAS-II helps determine
why a child is not learning and targets the specific nature
The original DAS (1990) was designed as a revision and
of the problem. Interpretation of the DAS-II allows for
extension of the British Ability Scales (BAS), published
intervention strategies to be identified and implemented.
in 1979. The BAS is a cognitive ability battery devel-
Results can assist in placement decisions for educational
oped to meet the need of British psychologists and
settings, and provide useful clinical information for neu-
educators for an assessment similar to the Wechsler
ropsychological evaluation and research purposes.
Intelligence Scale for Children (WISC), but valid for
The DAS-II is not considered an intelligence test.
British culture and standardized on a British popula-
The aim of the DAS-II is to provide a summary of mean-
tion. The BAS also aimed to resolve weaknesses of the
ingful and distinct subtest scores, with IQ assessment as a
available ability and IQ tests such as ethnic and social
secondary function. Interpretation of the DAS-II is
class bias within measures and minimal focus on
intended to identify specific strengths and weaknesses.
diagnostic subtests. A further aim was to incorporate
A unique aspect of the DAS-II is that the diagnostic
findings of emerging research of early cognitive devel-
subtests are fairly independent of composite scores.
opment that were not measured by existing ability-test
Therefore, these subtests are capable of providing unique
batteries.
information beyond that provided by composite scores
The development of the DAS began in 1984 by obtain-
(Elliott, 2007).
ing reviews, opinions, and feedback of the BASs perceived
Minimal research is currently available on the DAS-II
strengths and weaknesses from educators and clinical
because of its recent publication date; however, the origi-
psychologists. Based on this feedback, six subtests were
nal DAS has proven useful in differentiating children with
dropped and four subtests were added. Other revisions
learning disability from control children as well as
included reconstruction of existing subtests to increase
providing distinct profiles for various subtypes of learning
efficiency and reliability. Standardization of the DAS
disability (McIntosh & Gridley, 1993).
was performed during 19871989 in 70 cities across the
USA.
The DAS-II was published in 2007, and offers new and Cross References
updated artwork and materials, revised subtests, and
updated standardized norms. Four new subtests were Kaufman Assessment Battery for Children (K-ABC)
added to the second edition: recall of sequential order, NEPSY-2nd Edition
Diffuse Axonal Injury D 841
Diffuse Astrocytoma
Differential Diagnosis Fibrillary Astrocytoma
S ANDRA B ANKS
Allegheny General Hospital
Pittsburgh, PA, USA
Diffuse Axonal Injury
Definition B ETH R USH
Mayo Clinic
Differential diagnosis refers to the process of differentiat- Jacksonville, FL, USA
ing one diagnosis from another, and in turn, providing
the most fitting diagnosis based on an individuals pre-
sentation. The Diagnostic and Statistical Manual of Synonyms
Mental Disorders, for instance, has historically defined
diagnoses from a categorical perspective, with many DAI
diagnoses having features in common with one another.
This requires careful consideration of all features of
an individuals presentation and the fine details of the
Definition
diagnosis itself.
One of the major types of traumatic brain injury that
results from accelerationdeceleration effects rather than
direct impact on the brain. Typically, the injury is the
Current Knowledge result of high-speed situations such as motor vehicle
accidents or violent shaking of the head from side to
In neuropsychology, the process of providing the most side. The forceful motion of injury disturbs the delicate
fitting diagnosis for a patient involves assessment of the underlying white matter tracts of the brain that are re-
possible causes of the patients current condition as well as sponsible for connecting the functional areas controlling
a consideration of characteristics of the cognitive and neu- motor skills, cognitive skills, language skills, and behavior.
robehavioral presentation. This process must take into Because each neural cell or vessel has a fixed length and is
842 D Diffuse Axonal Injury
held in position along its path by other cells, there is simple terms, DWI makes it possible to see the disruption
limited elasticity in any cell or vessel. Thus, acceleration of cortical and subcortical connections throughout the
deceleration forces can disrupt cells and vessels despite an brain that may appear normal on a structural image
absence of direct trauma to the cell or vessel. Damage may of the brain such as a standard brain MRI or head CT.
result from stretching, twisting, or rotation. Diffuse axonal With DWI techniques, the understanding of DAI and the
injury (DAI) is more likely to occur with lateral movements implications of such damage have exploded. For example,
of the brain and least likely to occur with sagittal move- DWI images can now demonstrate structural abnormal-
ments of the brain. ities in traumatic brain injury patients even when DAI
cannot be visualized using standard imaging techniques
(Hou et al., 2007). DWI images have also been used as
Historical Background prognostic indicators to determine the length of coma
following traumatic brain injury (Zheng et al., 2007).
The first detailed pathological and histological account of Cognitive studies show that the greater the white matter
diffuse white matter damage following traumatic brain damage in diffuse axonal injury, the greater the cognitive
injury occurred in 1956 (Strich, 1956). In 1982, the first deficits observed in traumatic brain injury survivors
paper using the term diffuse axonal injury was pub- (Kraus et al., 2007). These improved methods for study-
lished (Gennarelli et al., 1982). Since that time, the idea of ing DAI will most likely expand the knowledge of the
DAI has further evolved and it is now clear that axons are heterogeneity of clinical presentation and outcome in
not affected in isolation, but rather there is injury to the the so-called mild traumatic brain injury, suggesting that
functional areas of the brain that are disconnected from mild traumatic brain injury may not be mild at all.
one another as a result of axonal damage. There is also DAI disturbs white matter tracts that are responsible
secondary damage to brain areas resulting from biochem- for efficient and coordinated communication between
ical changes and edema that evolve following disruption functional areas of the brain. Consequently, patients
of the axons. It has been suggested that diffuse axonal with DAI undergoing neuropsychological evaluation typ-
injury is therefore a misnomer and that it may be more ically demonstrate diffuse cognitive impairment most
appropriate to describe this type of injury as diffuse brain prominently characterized by markedly slowed cognitive
injury, a term that would include DAI. processing speed, poor attention, and difficulties with
executive functions like frontal lobe connections to
other functional brain areas are disturbed.
Current Knowledge
Bigler, E. D. (2001). Distinguished Neuropsychologist Award Lecture Diffusion tensor imaging (DTI) is a magnetic resonance
1999. The lesion(s) in traumatic brain injury: Implications for imaging (MRI) technique that reveals the integrity of white
clinical neuropsychology. Archives of Clinical Neuropsychology, 16,
matter tracts that link regions of the brain to each other.
95131.
Gennarelli, T. A., Thibault, L. E., Adams, J. H., Graham, D. I., Thompson,
DTI exploits the characteristic that water molecules
C. J., & Marcincin, R. P. (1982). Diffuse axonal injury and traumatic are in constant motion. In regions such as the ventricles, D
coma in the primate. Annals of Neurology, 12, 564574. which offer little physical constraint, movement occurs
Hou, D. J., Tong, K. A., Ashwal, S., Oyoyo, U., Joo, E., Shutter, L., & randomly in every direction. In contrast, water molecules
Obenaus, A. (2007). Diffusion-weighted magnetic resonance imag-
in white matter fibers are constrained by the physical
ing improves outcome prediction in adult traumatic brain injury.
Journal of Neurotrauma, 24, 15581569.
boundaries of the axon sheath, which cause greater
Kraus, M. F., Susmaras, T., Caughlin, B. P., Walker, C. J., Sweeney, J. A., & movement along the long axis of the fiber than across it.
Little, D. M. (2007). White matter integrity and cognition in chronic The diffusion properties of water molecules are
traumatic brain injury: A diffusion-tensor imaging study. Brain, 130, studied within and between three dimensional elements
25082519.
called voxels. The technique is called diffusion tensor
Strich, S. J. (1956). Diffuse degeneration of the cerebral white matter
in severe dementia following head injury. Journal of Neurology,
imaging because a tensor, a mathematical description of
Neurosurgery, and Psychiatry, 19, 163185. the orientation and magnitude of diffusion, is computed
Zheng, W. B., Liu, G. R., Li, L. P., & Wu, R. H. (2007). Prediction of for each voxel. Images acquired in different planes will
recovery from a post-traumatic coma state by diffusion-weighted highlight different white matter tracts or provide different
imaging (DWI) in patients with diffuse axonal injury. Neuroradiology,
views of them. Within a uniformly oriented tract,
49, 271279.
anything that disrupts the regular structure of white
matter, such as loss of the protective myelin sheath or
deterioration of the axons, might allow the water mole-
Diffuse Cerebral Gliomatosis cules to move more freely, resulting in an altered image.
Gliomatosis Cerebri
Current Knowledge
DTI has demonstrated white matter abnormalities in
Diffuse Lewy Body Disease Alzheimers disease, schizophrenia, AIDS, depression,
(DLBD) and normal aging. DTI has also been used to examine
brain white matter microstructural integrity in alcoholism
Dementia with Lewy Bodies and in the corticospinal tracts after stroke. It can reveal
microstructural abnormalities in white matter, such as
myelin loss, enlargement of microtubules, and degrada-
tion of membranes, even when that region appears
Diffuse Plaques normal on structural MRI.
In the area of traumatic brain injury, there is early
Amyloid Plaques evidence to suggest that DTI may be of value in assessing
diffuse axonal injury, which is typically difficult to image
with other available techniques.
Diffusion Tensor Imaging
Cross References
S USAN L ADLEY-OB RIEN
Denver Health Medical Center Magnetic Resonance Imaging
Denver, CO, USA
References and Readings
Synonyms Bigler, E. D. (2007). Neuroimaging correlates of functional outcome.
In N. D. Zasler (Ed.), Brain injury medicine (pp. 202). New York:
DTI Demos.
844 D Diffusion-Weighted Imaging
Rosenbloom, M. (2003). Using magnetic resonance imaging and infarction core, which usually progresses to full infarc-
diffusion tensor imaging to assess brain damage in alcoholics.
tion unless there is early reperfusion. The initial DWI
Alcohol Research and Health, 27, 146.
Xu, J., et al. (2007). Diffuse axonal injury in severe traumatic brain injury
lesion volume and ADC values correlate with infarction
visualized using high-resolution diffusion tensor imaging. Journal of volume and with neurologic assessment tests. ADC values
Neurotrauma, 24(5), 75365. may be useful in differentiating tissue destined to infarct
from that potentially salvageable with reperfusion thera-
py. With improvements in MR software and hardware,
diffusion MR will continue to improve the management
Diffusion-Weighted Imaging of patients with acute stroke.
E LLIOT J. R OTH
Northwestern University Cross References
Chicago, IL, USA
Ischemic Penumbra
Perfusion-Weighted Imaging
Synonyms
Historical Background magical number seven, plus or minus two: Some limits on
our capacity for processing information, the basic para-
The term digit span encompasses several important digm of span as a psychological method had already been
constructs and names of tests designed to measure these examined.
constructs. First and foremost digit span, along with The second and more frequently used meaning of
reaction time, may be viewed as one of the two original the term digit span is as the name of psychological
paradigms used by experimental psychologists to investi- tests to assess working memory and/or the ability to D
gate cognition. The origins of digit span as a psychological retain and keep in mind a circumscribed amount of
construct date from the work Gottfried Leibniz information for a short period of time (Wechsler, 1997).
(16461716). Leibniz suggested that individuals have a As a psychological test, digit span and other techniques
finite capacity to prospectively process or hold in mind to measure span of apperception have long history.
the information from the environment. He termed this James McKeen Cattell (18601944; 1890), who worked
capacity the span of apperception. (Much of the historical in Wilhelm Wundts laboratory in Leipzig, included a
information reviewed in this essay is drawn from JTE test of span for consonants in his compendium of mental
Richardsons paper: Measurements of short-term mem- tests. (After being fired from Columbia University for his
ory: A historical review. Cortex, 43, 635650, 2007.) opposition to World War I, Cattell, along with Robert
In the nineteenth century, Herman Ebbinghaus Woodward and Edward Lee Thorndike, founded the
(18501909; 1885/1964 cited in Richardson, 2007) was Psychological Corporation in 1921 in New York City. In
the first cognitive scientist to show how span could be 1939, Woodwards former student, David Wechsler, pub-
used as an experimental paradigm to investigate memory lished the first version of the intelligence test that has so
and learning. In America, Oliver Wendell Homes pater very much influenced the science and practice of psychol-
(18091894; 1871 cited in Richardson, 2007) made ogy.) In addition, span was included as part of the intelli-
cogent observations on the parameters of digit span as a gence tests devised by Binet and Simon (1905; cited in
method to assess span of apperception, i.e., in uttering Richardson, 2007). In this version, up to three trials
distinctly a series of unconnected numbers or letters were administered for each digit sequence. Terman
before a succession of careful listeners, I have been (1916) also included a digit span test in the Stanford
surprised to find how generally they break down, in trying revision of the BinetSimon scales, but in Termans ver-
to repeat them, between seven and ten figures or letters sion, digit sequences both forward and backward were
(Holmes, 1871, cited in Richardson, 2007). (Oliver Wen- administered.
dell Holmes, Sr., M.D., a resident of Cambridge,
Massachusetts, was a very influential nineteenth century
physician. In addition to his achievements as Dean of
Harvard Medical School, Dr. Holmes popularized the use Psychometric Data: Digits Forward/
of the stethoscope as medical instrument. Equally impres- Digits Backward
sive were Dr. Holmes literary achievements. Admired by
Edgar Allan Poe, Dr. Holmes was a member of an illustri- From the beginning, a digit span subtest has been at least
ous group of Boston literati and could count as his friends included in virtually all Wechsler intelligence and memory
such individuals as Ralph Waldo Emerson and Henry scales for both children and adults. Wechsler combined
Wadsworth Longfellow. Known for his essays and poetry, performance on the digits forward and digits backward
Dr. Holmes was a founder of the Atlantic Monthly tasks into a single score on the basis of psychometric
magazine. In addition to his other achievements Dr. considerations. However, clinical observations have long
Holmes is credited for naming the city of Boston The demonstrated the value of treating each procedure as a
Hub of the Universe. Holmes son, Oliver Wendell Holmes, separate test as combining forward and backward test
Jr., was a well known jurist and was appointed Associate performance potentially obscures the value of each test
Justice to the Supreme Court by President Theodore Roo- condition to measure related, but separate psychological
sevelt.) Several years later, Ebbinghaus made a similar constructs.
observation commenting, the question can be asked, A lively debate has centered on the cognitive con-
what number or syllables can be correctly recited after structs that underlie successful performance for both
only one reading. . . for me the number is usually seven, digits forward and backward test conditions. Indeed,
(1885, cited in Richardson, 2007). Thus, more than 80 Kaplan and colleagues (1991) tend to view the digits
years before George Millers seminal paper (1956) The forward test condition as a means for measuring span or
846 D Digit Span
how many bits of information can be held in mind, where- where the examinee is asked to repeat increasing spans of
as the digits backwards test condition is viewed as a test of digits in the order they were presented; and digits back-
mental manipulation. In this context, the observations of wards, where the examinee is asked to repeat increasing
Kaplan and colleagues (1991) are consistent with the con- spans of digits in reverse order. In both test conditions,
structs put forth by Baddeley and Hitch (1974) regarding two trials are administered for each span length. Each
working memory. Other researchers have made similar condition is discontinued when the examinee fails both
observations. For example, Ramsey and Reynolds (1995) trials for a given span. The raw score is the sum of trials
and Reynolds (1997) studied large samples of children correctly repeated. This raw score is converted into an
and adolescents using a protocol of tests requiring the age-corrected scale score. Developed as part of the origi-
repetition of sequences of digits and letters both forwards nal Bellevue Intelligence Tests (Wechsler, 1939), Wechsler
and backwards. An analysis of forward and backward test writes there is perhaps no test that has been so universally
scores using principal component analysis found that used in scales of intelligence as that of Memory Span for
forward and backward span loaded on different factors. Digits (Wechsler, 1944). Wechsler acknowledges that
These researchers therefore concluded that the forward low scores on the Memory Span for Digits Test are
and backward span should not be combined and reflect frequently associated with attention deficits (Wechsler,
distinct underlying cognitive processes. 1944). He further states that the problems with attention
Weinberg, Diller, Gerstman, and Schulman (1972) and concentration as measured with his Memory Span for
studied patients with visual scanning deficits associated Digits Test are often associated with differentially poor
with right hemisphere pathology and noted differential repetition for digits backward, writing this deficiency is
impairment on digits backwards versus digits forward. often referred to by psychologists as a lack of mental
These researchers advanced the notion that repeating digits control (Wechsler, 1944). Wechslers use of the term
backward may rely on a capacity to form and keep in mind mental control is consistent with what is now understood
a visual image of the digit sequence. Successful perfor- as working memory. Thus, although Wechsler combined
mance on digits backward may be viewed in terms of the forward and backward digit test performance, he was
ability to keep this visual image in mind while peeling certainly cognizant that his combined scale score could
numbers off in reverse order. Rudel and Denckla (1974) be fractionated to measure a variety of cognitive
retrospectively studied digit forward and backwards test functions.
performance in children with learning disabilities. Children Kaplan and colleagues (1991) have suggested a num-
with evidence of left hemisphere dysfunction produced ber of additional administration and scoring techniques
particularly reduced scores on digits forward. Children to complement the traditional administration of the
with evidence of right hemisphere dysfunction appeared Wechsler Digit Span Test. First, Kaplan and colleagues
disadvantaged when asked to repeat digits backward. Rudel (1991) suggest the investigator administer test trials
and Denckla (1974) believed that their data suggested the until the examinee fails to repeat the exact number of
successful performance for digits forward is governed by a digits that were administered, rather than discontinuing
left hemisphere-mediated cognitive construct involving after failure within a pair of trials within a particular span,
auditory storage/ rehearsal while successful performance regardless of whether the examinees response is correct or
for digit backwards is governed by a right hemisphere not. By following this procedure, particularly for the
mediated construct involving visual imagery operations. digits forward test condition, one is able to assess the
The value of separating forward from backwards test con- patients span of apperception, or the number of bits of
ditions is acknowledged in the latest version of Wechslers information the patient is able to keep in mind. Moreover,
IQ test (WAIS-IV) wherein separate scale scores are now for some brain damaged patients where attention, con-
provided for digits forward and backward. centration, and working memory may be compromised
or fluctuate it is possible that despite, say, failing both
5-span test trials, a patient could be successful on one or
Clinical Uses more 6-span test trials or on even longer span lengths.
Kaplan (personal communication) has also said that a
Wechslers Digit Span Task better scoring procedure for the Digit Span Test would
be to tally the number of digits correctly recalled, rather
This task is most often associated with the corpus of than the number of successful trials. (Dr. Kaplan is well
intelligence tests authored by David Wechsler. Wechslers known for saying: If they want numbers, give them
Digit Span subtest comprises of two parts digits forward, numbers that are meaningful.) The interpretation of a
Digit Span D 847
juxtaposition of two digits in the middle of a trial could be 5-digit span lengths for a total of 21 trials. All 4 and
very different compared to instances of omissions or 5-span trials were constructed so that contiguous
obvious perseverations. The idea of tallying the number numbers were placed in strategic positions. For example,
of digits correctly recalled was the impetus for the in 4-span trials contiguous numbers were placed in either
creation of The Backwards Digits Test (BDT; Lamar & the first and third or second and fourth digit positions,
colleagues, 2007, 2008) described below. e.g., 5269 or 1493. In 5-span trials contiguous numbers
Kaplan (1988) and Kaplan and colleagues (1991) also were placed in the three middle digits positions, e.g., D
call the attention to the variety of errors patients make on 16579. Three-span test trials were not constructed in this
the digit span test. fashion because of the primacy and recency effects. This
Omission Refers to responses characterized by a miss- placement strategy was devised to examine the capacity of
ing digit. On the digits forwards task, an omission error patients to resist the temptation to produce capture errors
occurs if a four-digit response of 6-8-2-1 is given to the or to erroneously group contiguous numbers together,
five-digit stimulus 6-8-2-5-1. Interpretation of omission e.g., 15679 incorrectly reported backward as 96751.
errors typically considers the length of the number se- The BDT is administered using standardized Wechsler
quence: whether this kind of error occurs on shorter (4- procedures except that there is no discontinuation.
span) versus longer (7 or 8-span) digit sequences. All 21 trials are administered. The two major indices
Addition Refers to responses characterized by added derived from this test are percent of digits recalled in
digits. On the digits forward task, an addition error ANY ORDER and percent of digits recalled in SERIAL
occurs if a five-digit response for 4-7-2-3-1 is given to the ORDER.
four-digit stimulus 4-7-2-3. One type of addition error is Percent Correct ANY-ORDER This score reflects the
an automatized addition error. An automatized addition sum total of digits correctly recalled regardless of their
error occurs when the added number is sequential, i.e., serial position, divided by the total possible correct, mul-
7-4-5-3 repeated as 7-3-4-5-6. Automatized addition errors tiplied by 100, [(total number of correct digits ANY-
suggest an executive control or frontal system problem. ORDER)/ (total possible correct)] 100. By eliminating
Perseveration A perseveration refers to a number the importance of serial position, this measure is believed
duplication error. Perseveration errors occur within to reflect less complex aspects of working memory char-
and/or between trials. When noting a perseveration, one acterized mainly by short term or immediate storage and
should observe whether the perseverated responses were rehearsal mechanisms.
captured within or between test trials. Percent Correct SERIAL-ORDER This score reflects
Substitution A substitution error refers to a unique the total number of digits correctly recalled in exact
number that is contained in the digit response string serial position, divided by the total possible correct,
that replaces an original number and does not change multiplied by 100, [(total number correct digits SERI-
the original digit string length. This differs from the AL-ORDER)/ (total possible correct)] 100. This mea-
addition errors described previously, because addition sure is believed to assess the more executively demanding
errors change the digit string length. This is coded when aspects of working memory that are associated with
any digit is given instead of a digit contain on the mental manipulation, such as disengagement and tem-
stimulus. poral reordering.
Sequence Error A sequence error refers to a correctly In addition to ANY and SERIAL ORDER recall, the
repeated digit that is simply misordered, but does not BDT affords an opportunity to examine for capture errors
change the number string length. or the tendency of patients to erroneously group contigu-
Capture Error A type of sequence error that is ous numbers together. Two major types of capture errors
characterized by the grouping of contiguous numbers are scored (see Kaplan, Fein, Morris, & Delis, 1991).
together. Within Trial Capture Errors This error is coded on
4 and 5-span trials when the capture error is confined
to digits within the trial at hand, i.e., 14933491;
The Backwards Digit Test (BDT) 1657995671.
Between Trial Capture Errors This error is coded on
Lamar and colleagues (2007, 2008) devised The Backward 3, 4 and 5-span test trials when participants pull down or
Digits Test (BDT), a digit backward paradigm designed incorporate a digit or digits from an immediately preced-
specifically to assess for working memory deficits in ing test trial. In a larger sense, both types of capture errors
dementia. This test consists of seven trials of 3, 4, and could be viewed as perseverations.
848 D Digit Span
Lamar and colleagues (2007) studied two groups of References and Readings
dementia patients where the onset of the illness was insid-
ious and the overall severity of the dementia was mild. Baddeley, A. D., & Hitch, G. J. (1974). Working memory. In (Ed.),
The severity of periventricular and deep white disease was G. H. Bower, The psychology of learning and motivation: Advances
in research and theory (vol. 8). New York: Academic Press.
measured using MRI scans, and patients were categorized
Cattell, J. M. (1890). Mental tests and measurements. Mind, 15, 373381.
as presenting with either mild versus moderate/severe Cosentino, S., Jefferson, A. J., Chute, D. L., Kaplan, E., & Libon, D. L.
white matter disease. No difference was found for ANY (2004). Clock drawing errors in dementia: Neuropsychological and
ORDER recall, suggesting that span, storage, and rehearsal neuroanatomic considerations. Cognitive and Behavioral Neurology,
mechanisms did not differ between these dementia 17, 7483.
Ebbinghaus, H. (1964). Memory: A contribution to experimental psycho-
groups. However, significant differences were found for
logy (Ruger HA and Bussenius CE, Trans) (Original work published
SERIAL ORDER recall suggesting differential working in 1885). New York: Dover.
memory impairment for dementia patients who had Holmes, O. W. (1871). Mechanism in thought and morals: An address
significant subcortical white matter disease, a finding delivered before the Phi Beta Kappa Society of Harvard University, June
well documented in the literature (see Lamar et al., in 29, 1870, with notes and afterthoughts. Boston: Osgood.
Kaplan, E. (1988). A process approach to neuropsychological assessment.
press; Libon, Price, Garrett, & Giovannetti, 2004 for
In T. Boll, & B. R. Bryant (Eds.), Clinical neuropsychology and brain
reviews). A series of step-wise regression analyses found function: Research, measurement, and practice: Master lectures.
that ANY ORDER recall was primarily related to perfor- Washington, DC: The American Psychological Association.
mance on the MMSE or dementia severity. By contrast, Kaplan, E., Fein, D., Morris, R., & Delis, D. (1991). The WAIS-R as a
SERIAL RECALL was related to performance on wide neuropsychological instrument. San Antonio, TX: The Psychological
Corporation.
number of other executive tests including letter fluency,
Lamar, M., Catani, M., Heilman, K. M., & Libon, D. J. (2008). The impact
The Boston Revision of the Wechsler Memory Scale Men- of region-specific leukoaraiosis on working memory deficits in de-
tal Control Test (Lamar et al., 2002), and clock drawing mentia. Neuropsychologia, 46, 25972601.
errors (Cosentino, Jefferson, Chute, Kaplan, & Libon, Lamar, M., Price, C., Davis, K. L., Kaplan, E., & Libon, D. J. (2002).
2004). In a follow-up study, Lamar and colleagues Capacity to maintain mental set in dementia. Neuropsychologia, 40,
435445.
(2008) examined regional MRI white disease and perfor-
Lamar, M., Price, C. C., Libon, D. J., Penney, D. L., Kaplan, E., Grossman,
mance on ANY and SERIAL order recall and found that M., et al. (2007). Alterations in working memory as a function of
reduced SERIAL ORDER recall was related to left inferior leukoaraiosis in dementia. Neuropsychologia, 2007; 45, 245254.
parietal white matter disease. These data are consistent Libon, D. J., Price, C., Garrett, K. D., & Giovannetti, T. (2004). From
with ideas put forth by Rudel and Denckla (1974), sug- Binswangers disease to Leukoaraiosis: What we have learned about
subcortical vascular dementia. The Clinical Neuropsychologist
gesting that a visual imagery mechanism may underlie
Vascular Dementia Special Edition, 18, 83100.
successful backward digit span performance. The impor- Miller, G. A. (1956). The magical number seven, plus or minus two: Some
tance of number sequencing is acknowledged in the latest limits on our capacity for processing information. Psychological
version of Wechslers IQ test (WAIS-IV) wherein a sepa- Review, 63, 8197.
rate score is now calculated for the longest digit string Ramsey, M. C., & Reynolds, C. R. (1995). Separate digits tests: A brief
history, a literature review, and a reexamination of the factor
correctly sequenced.
structure of the Test of Memory and Learning (TOMAL). Neuropsy-
Digit span is one of the original paradigms used chology Review, 5, 151171.
to investigate cognitive psychology. Attention to Reynolds, C. R. (1997). Forward and backward memory span should not
error type, i.e., the act of focusing on the process be combined for clinical analysis. Archives of Clinical Neuropsy-
by which the scaled score is obtained (Kaplan, 1988), chology, 12, 2940.
Richardson, J. T. E. (2007). Measurements of short-term memory:
particularly where errors occur and differential perfor-
A historical review. Cortex, 43, 635650.
mance on forward versus backward recall, can yield a Rudel, R. G., & Denckla, M. B. (1974). Relation of forward and backward
wealth of information about cognitive disorders in digit repetition to neurological impairment in children with learning
brain-injured patients. After more than a century, a disabilities. Neuropsychologia, 12, 109118.
seemingly simple task of digit repetition remains an Terman, L. M. (1916). The measurement of intelligence: An explanation of
and a complete guide for the use of the Stanford revision and extension
important construct in the understanding and assessment
of the Binet-Simon intelligence scale. Boston: Houghton Mifflin.
of cognition. Wechsler, D. (1939). The measurement and appraisal of adult intelligence
(1st ed.). Baltimore: Williams and Wilkins Corporation.
Wechsler, D. (1944). The measurement and appraisal of adult intelligence
Cross References (3rd ed.). Baltimore: Williams and Wilkins Corporation.
Wechsler, D. (1997). The Wechsler adult intelligence scale-III. San Antonio,
Serial Recall TX: Psychological Corporation.
Digit Symbol Substitution Test D 849
Weinberg, J., Diller, L., Gerstman, L., & Schulman, L. (1972). Digit corresponding geometric symbol. The examinee is then
span in right and left hemiplegics. Journal of Clinical Psychology,
shown a series of boxes containing numbers in the top
28, 361.
boxes, and blank boxes below them. After a short practice
trial, they are then asked to copy the corresponding
geometric symbol under each number. The raw score is
the number of correct items completed within the pre-
Digit Span Test scribed time limit. D
The Symbol Digit Modalities Test (SDMT; Smith,
Digit Span
1982): This test is very similar to Wechslers DS test,
except that rather than transposing symbols to numbers,
the examinee is asked to transpose numbers to symbols.
An advantage of the SDMT is that there are both written
Digit Supraspan and oral administrations. Smith (1984) suggests the
administration of both the written and oral versions of
Serial Digit Learning the test, with the written version being administered first.
In addition, the SDMT provides the additional advantage
of allowing the examinee to write familiar numbers (19)
rather than geometric symbols (Lezak, Howieson, &
Digit Symbol Substitution Test Loring, 2004). Smith (1984) reported greater sensitivity
of the SDMT compared to the DS subtest in detecting
B RIANNE M AGOUIRK B ETTCHER 1, DAVID J. L IBON 2 brain damage.
E DITH K APLAN 3, R OD S WENSON 4, DANA L. P ENNEY 5 Both the DS and SDMT are well known to be highly
1
Temple University sensitive to the presence of cognitive impairment (see
Philadelphia, PA, USA Lezak et al. (2004) for a review). The general sensitivity
2
Drexel University College of Medicine of these tests stems from the fact that multiple cognitive
Philadelphia, PA, USA skills are necessary for optimum performance. For
3
Suffolk University example, at a minimum the examinee must be able to
Boston, MA, USA produce written output (graphomotor skills); visually
4
University of North Dakota Medical School track back and forth from the test key to the test form
Fargo, ND, USA (visual scanning); and locate the test items (visuospatial
5
The Lahey Clinic Burlington ability). Performance improves to the extent the examinee
MA, USA learns the symbol/number pairs without referring to
the test key for each test item (incidental memory). In
addition to norms provided in the Wechsler test manuals,
Synonyms additional adult norms for DS can be found in DElia,
Boone, and Mitrushina (1995); Heaton, Grant, and
Coding; Digit symbol subtest Matthews (2004); and Ivnick, Malec, and Smith, (1992).
DS performance is highly sensitive to the effects of age
(Lezak et al., 2004). Wechsler (1981) reported low correla-
Description tions between DS and many of his other subtests. Since
the addition of the Symbol Search subtest to the Wechsler
The Digit Symbol substitution test (DS): This is a subtest corpus, factor analysis suggests that DS and Symbol
from the corpus of intelligence tests authored by David Search comprise a general processing speed factor.
Wechsler. Since Wechslers publication of the Bellevue In order to disambiguate the specific brain-behavior
Intelligence Scale (BIS; Wechsler, 1939), the basic format relationships that underlie impaired performance on the
and concept of this test has changed very little. The test is DS test, Kaplan and colleagues (Kaplan, 1988; Kaplan,
timed. Various versions from the Wechsler corpus allow Fein, Morris, & Delis, 1991) have developed additional
90 or 120 s. The test requires the examinee to transcribe a test procedures (see WAIS-R-NI; Kaplan et al., 1991) as
unique geometric symbol with its corresponding Arabic described below.
number. The examinee is initially shown a key containing Symbol Copy Test Condition: The symbol copy test
the numbers from 1 to 9. Under each number there is a condition of DS test involves the direct copying of
850 D Digit Symbol Substitution Test
geometric symbols without the burden of visual scanning indicating that the tests share 50% of variance. This fur-
or incidental memory. The purpose of this measure is ther reiterated the integral role of processing speed in DS
to assess the contribution of both motor speed and performance. Joy, Kaplan, and Fein (2004) also examined
graphomotor ability to DS performance. It is also data (n = 1167) from the WAIS-III/WMS-III standardiza-
suggested that the examiner keep track of the output tion sample for individuals who completed at least one DS
produced in each 30 s epoch (Kaplan et al., 1991). Kaplan supplemental procedure. Consistent with previous find-
et al. (1991) noted that the output produced by some ings, results suggested that speed (i.e., Symbol Copy)
patients, particularly with subcortical disease, may decline accounted for approximately 50% of the variance in DS.
on the latter portion of the test. Using the incidental memory learning indices, memory
Incidental Learning: This test condition involves two accounted for only 57% of DS variance. However, per-
procedures. After the DS test is concluded the examinee formance on the pairing and free recall tests provided
may be asked to recall and write the symbols that were incremental explanatory power, authenticating a second-
coupled with each number (pairing). This is followed by ary (albeit small) role of memory in DS performance.
asking the examinee to recall and write as many of the Contributory role of incidental memory: The incidental
symbols as possible without any visual cues (free recall). memory measures (pairing and free recall) have been
Part of the rationale for this procedure is that, although previously used as supplementary indices of memory
examinees are not instructed to memorize the number/ without extensive research regarding their construct
symbol pairs, some degree of incidental learning can validity and without normative data. Joy, Kaplan, and
reasonably be expected to occur. Fein (2003) examined the performance of WAIS-III/