BLACKWELL'S

VI[KAS

B W S H A N , MD

University of California, San F r a n c i ~ oClass nf 1991 , Srrim Editor, D i a ~ ~ l o s rRadiologist ic

VTSHAL PALL, MBBS

Governtnent Medical College, Chandigal-11, India, CIacs of 1996 Series Editor, 1. of Texas. I;alveston, Recident in Intrrnal Meclicine & : Preventive Medicine

TAO LE, MD
Universitv or Clalifornia,San Francisco. C l a ~ s 1996 of

A s m ZAMAN, MBBS
Kctv Drlhi, India

Blackwell
Science

CONTRIBUTORS
Robert Nason Universitv of fcxaq Medical Branch. C 1 x . ~ 2003 of
Kristen Lem Mygdal, MD

L'niwrsity o Kansas School o Medicine, R e ~ i d e tn in Radir~logy f f

Wpih Shah, PA-C
tltlive~ritv Texas M ~ d i c i l Rmnrh, C;alv~ston,Clxs o 2M0 of l r

Fadi Abu Shahin, MD Univemitv or Da~narcu~, Syria, Clasq nf 1 YO9

Hoang Nguyen, MD. MBA northwest en^ Llniversity, Class of 21301

Sonal Shah, MD Ross Univenitv, Class of 2000

B 2002 hy Blackrvell Science, lnc.

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Blackwell's Underground Clinical Wpettes: Pathophysiology m, 3e ISBN 0-63Y-1145554
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Bhuuhan, Vikas. Btaclnvell's underground clinical vignettes. Palhophpiolop / author, Vi'ikau Rhushan. - 3rd r d . p. :cm. - (Undergrof~nd clinical vignettes) k r . 4.of:PatIlophvsiologv / T ' i h EBl~tishan. 2nd ed. clYY9.15RN (kf3204551-3 (pbk.) 1. Phwinloffl. Patl~ologicat Case studies. 2. Phyacians - Licenser - United Status Examinatinn~ Study guides. [ D N L U 1 Clinical Mtdicinr - Case Repurt. 2. Clinical Medicine - ProM~ms and Excrciseq. WR 18.2 B573hb 20021 1. Ti&: Cndergzaund clinical vipettes. Fathnph?.;iology. 11. Fathoph!~inln~. 111. T~tle. Serieq. IV, RBI 1'3 .I3459 2002 FIG.O7'C17ttdc21

Notice 'l'hr ;IUI llors n rhis t,.rliirnr h:wr takrn c;lrt. ~hakthe ir~f?,~.rnatinn T contained herrin is accurate ant1 comptihlr with thcl .;ta~ltl;~rrlc genrnillv itcccplctl at tlir Iinw or pi1,licadon. Newrtliclcsc, i l is r l i l l i c ~ ~ l trnsure tn that :111I ~ infornlatiun given is entirclv acri~~.atc all tircumsrance~. P 1,r The pr~hlisl~er authors da not and ,ymranLcc the C O ~ I C I ~ol' this hook ant1 dicclaim anv liafiilitv, Ions. o r dar~lagc IS incrirrcd as a conneqrrence, directlv or intlirerlly, of the use arid :lpplic;ttion ot'nny r)f the rnnrrnts nf thiq vol~irne. n

Acknowledgments
Preface t o the 3rd Edition

xiii

How to Use This Book Abbreviations

xvi
xvii
Amyotrophic Lateral Sclerosis (ALS) Aphasia-Broca's C1 Spinal Cord Injury Cauda Equina Syndrome Cerebral Aneurysm Ctaniopharyngioma Creutzfeldt-Jakob Disease CVA, Capsular Infarct CVA,Hypertensive Epidural Bematoma Friedreich's Ataxia Glioblastorna Multiforme Guillain-Barre Syndrome He miballismus Huntington's Chorea Internuclear Ophthalmoplegia Kliiver-Bu y Syndrome Medulloblastoma
M6niGreS Disease Meningioma Metastatic Brain Tumor Migraine Multiple Sclerosis

!@! k" "

X

1
I c>

T'
ic

'I

!<
I
A

' 9

"" ' 9
1

I
x y

* ' 1

I.'
"
I

:>

Myasthenia Gravis Myotonic Dystrophy

Neuroblastorna Normal Pressure Hydrocephalus OLigodendrogboma Peripheral Neuropathy-Diabetic Pseudobulbar Palsy Subarachnoid Hemosrhage Subdural Hematoma Syringomyelia Temporal Arteritis (Giant Cell Arteritis) von Hippel-Lindau Disease Bre-ast-Cystosarcoma Phyllodes
S?+

!
1
)-

^"

A">'P

1'

+ ,

*" '

I
p, .
!I :

Breast-Fat

Necrosis

Breast-Fibrocystie Disease Breast -1nf ammatory Carcinoma Breast-Intraductal Papilloma Breast-Lobular Carcinoma Breast-Paget's Disease Breast Carcinoma Breast Fibroadenoma Cervical Carcinoma (In Situ) Choriacarcinoma Desmoid Tumor

Dysmenorrhea Endometrial Carcinoma

Endometriosis Hydatidiform Mole Menopause Ovarian Cancer Ovarian Cyst-Follicular Ovarian Teratorna Polycystic Ovarian Syndrome Primay Amenorrhea-Taarnefs Syndrome Uterine fibroids Uterine Leiomyosarcoma Vulvar Carcinoma Vulvar Le&oplakia Vulvar Malignant Melanoma Ectopic Pregnancy Postpartum Hemorrhage Postpartum Thrombophlebitis Sheehan's Syndrome Toxemia of Pregnancy-Pxeeclampsia Ewing's Sarcoma Hand-Dupuytren's Contracture Hip-Avascular Necrosis of Femaral Head Hip-SSipped Capital Femoral Epiphysis Knee-Anterior Cruciate ligament Injury Osteaarthri'cis Osteogenic Sarcoma Paget's Disease of Bone Septic Arthritis-Staphylococcal Spine-CervTcal Spondylosis Alcoholism Ankylosing Spondylitis Decompression Sickness Dermatomyositis

Orthopedics

Juvenile Rheumatoid Arthritis Mixed Connective Tissue Disorder Osteopetrosis Osteoporosis Polyarteritis Nodasa PolymyaIgia Hheumatica Polymyositis Progressive Systemic Sclerosis (Scleroderma) Pseudogout Raynaud's Disease Reiter's Syndrome Rheumatoid Arthritis Sjo gren's Syndrome Systemic Lupus Erythematosus (SLE)

Rheurnotology Toxicology

Wegener's Granulomatosis

Carbon Dioxide Narcosis

Thrcxighour the pi+od~~ctiont h i ~ of hook. we Iiave had the suppcll-1 ol rnan!tfrietlcls arid co1lea~uc.s. Special tfl;ink$ to mu- w ~ p port trarrz incl~~tliiig Gtipta. ; l t ~ r l ~ - e a AIXI~ Fellows, .Anastasia ;2nderuorl. Srishti C;itpra. Mona Pall. J o n a ~ h a n Kirsch nnd Chitag ,211ii11. p i o r c.oi~trihuiions ~li:l~ik FOIWP C;iatllli Le Nguyen. T a n u l Mar litrr; Alex Grimin. Sonia Snn to5 imrl Eli~:thettl ,Satlrlel-<.
Mi. h a \ r cr!juyc.d working with a t~o~.ld-claw i nter-national p ~ t h liut~iilg gro\lp a t Rlxrkwrll Science. inrlucIing Lanul-a DeYoung, Anlv Ni ~ tbt-ock, Liva Flanagan. Sha~vn t Girshrrgcr, Lurna Hind and Gclt-dun Tihhitts. Fur hclp wit11 .;ucuririz irr1age:e9 r . t h e h cntirc scries ~ v c also thank Lre Mar-tin. ki.;1uphc1-J o r l e ~ .Tina P a n i ~ / i n d Prtrr Ander.soii at tht5 L!ni~.er.sity of' Alnhania, the a ,L\rmed Fnrces Institute or Parholop, and m:my of our fcllo~v Rlack~rcll Scicncr a ~ ~ t h o i s .

For suhnii tting comments, corrections. cdiiir~g, proolreading. ant1 ;trsi$rat~rel c 1 . 1 ) all o f (he v i g n ~ t tr i~ l e i r all editions. ~ v c i ~~ t ~ collcctivcIy thank:
~\ct,rniovich,C:i~r.rllvr~ d%lexantler. liris .Ildrn. P I e n t ~ E. ,krva~l. Cynnran Bacolor, Natnlie k~rtcncva,Dcan Bar+rholomew,
%I;I

Det>ashisll Hel>rra.Sumir Rhntia, Sanjay Binrlta, I>a~~\lr Ilrinton, Jt~lianne Bt.n~vt~. ,2Pexat1rlrrI<ro\~,nie. 'Tanral-a.Callal~an, Darid C:anes, 1h-y;tn Caw?: h a r m Ca~ighev, I ~ h e Chen. Sanatl~an k r~ CEle~ig, ~ n n l d R Cheung, :lul-~~old Chin. Silnion Chiosca. Yv'oun C:ho, Sitrnriel Cl~ting, C:retrhen I:r)ii;ln~, \laclimit- Cnric. Christ npher C;n~grnve, Rnll.tlrl Cswa~i. LIT-c kin R. Cunningham, A. Scan Dallcy Rama Dandaln~tdi. Sunit Qas, Ryan i-brnando D;r\-c.J~ht? David. E m r n a n u d c l r 1;1 C ~ u zR o l x i - t Duhlello, , Navtieet llhillon. SIiattnila. Uissanaike, Daritl Donson. Adolr Etclicjiarav. N r a Euschio. Priscilla A. Frasc. Daricl Fr-cbnj-, i s l i n k C ~ I I I ~ PY<lh;~r~nes I; C1~l,l-ecg/iabhel; AniF Gehi, Tonv George. 1 ..M. Gninncn. Pat11F <;oval, Ales Gritnt~i, Rjeev Gtpta. Ahmad I-lalim, Sue I-Iall, Davicl IIx~sell~aclicl; Ta1m-a Ilcimrrt. Michelle Higlev, Dan Hoi 1. EricJ;icksr~n.Tirri J;~cLson, Stmdar Jayararnati. Pei-Ni+Jnne. Aal-rlian+loshi.Ki-7jni K.+Jtltla. Fail~azliap;~di. Seth I h p , liaron S. Ke~serheim, Sans Jdihan, .h~rlrcw Pin-wci KO. Francis k n g . Paul Knnitzkr: 1lT;t;lrrc-n S. Eirackuv. Benjanii11 H.S. 1,311. 1111i 1JC:;ISCP. t COII it= I ,ee, Srn~t .cp. Gt~iIlermo n I Lel~inar~n. Iicvin Lcung. Paul Lcvctt, M'arrcil L c ~ i n s o nEric Ley. FFi n Lin, . e

Pave1 L0hano.r; J. Mark Maddox, Aram hlardian, Sarnir Mehta, Gil MclmecI, Joe Messina, Rohert Mmca, hfichacl Murphy, Vivek

Vandkarni, Sim Nan>-an. Car,vell Nguyen, Linh Nguyen, Deanna Noblcsa. Craig Nodurrr, Georgr Norimi, Darin T. Okuda. Adam L. Palan re, Paul Pamphrus, Jin h a Park. Sunny Patr.1, Ricardo P i e t r n h m ~R i m L. h h ! , Aaqhita h n d e r i a , , hchan Reddy Beatriu Rcjg. Marilou Rrves,Jercmv Richmon, Tai Roe, Rick Roller, Rajiv Roy, Uiegn R ~ i i z, r l n t h o ~ ~ y Russell, Smi jay Sahgal. LT~rnilnala Sal-kar.John Schilling. lsahe!l Schmitt, Daren Schuhmi~chrr; Sonal Shah, Facli A b r ~ Shahin, Mae Sheikh~'lli. Edic Shen. Justin Smith,John Str~lak, .iIlian Su, Julie F Sur~rl;trar~~. Suri, Seth Swe~tscr, 2 ~ ~ ~ oTahvero, Merita Rita i nio Tan, Mark Tanaka, Eric Taylor,Jess Thonipsoil. lndi Tt-ehan, I<aymoi~d Tin-ncr, Okafo Uchenna, Eric U y p a n c o , Kicha Varma. John M7iiges. Alan Wang, Eunicc Wing, ~ZntlIy Weiss, Amy Williams. Rrian k-ang, I-Ian): Zaky, hrhraf Zaman and Da\;id Zipf.

For generously confsibrlzing images to the entire Lhdmpund Uinzrol L ' i p ~ t t rStep I serieq, we collectively thank the staff at IElarku,ell Scicncc in OxFn~-rl, Uoston, ;tncl Berlin as we11 as:
~.L~rorcl,Mede'cin~. J. [Oqney Mead: Rlackwrll Science Ltd, 1996.

Figures2.14,2.15,2.ltj,2.2T,2.2P,2..11,2.35.2.36.2.38,2.49. 2 . 6 5 ~2.65h, 2.65c, 2.103b, 2.1051>,3.20t>, 3.21, 8.27, 8.27b, . S.77h, 8.7'7~.I0.81h. I0.9hi, 12.283. 14.6, 14.16, 14.50,
Balln ister b, EEegg N, Gillcspie S. ~ I ~ / P I . ! ~ oIlzs~nse.2"Tditimn. I~,F Osnry Mead: Black~vcllS r i e i ~ c e I,crl, 2000. Fip~r.es 2.8, 3.4. 5.28, 18.10,'Clr:i.32, W5.6.

* C ~ ~ s c l ~ i e rHennessyTPJ, G r e e n l ~ ~ ~RM.RowlqDrZ, 12. i lgh

PA. CliniroI . I " r r r g ~ ~ . Osnev Mead: Elackwell Science Ltd. 1996. Figt~res 13.19, 18.22. 18.33.
Grace

Gillcspir SH, Ram fn t-rl R i l f d i c d ,~4;mhiolt!g Infkrfirm nl a nnrl Glmrr. (3~11ey Mead: Blachvrll Scjrnce I.rrl, 2000. Figures 20,23.

Ginshrrg L.. 1,~rflirrNotps on A;rrrmlogp, T"'I;:diiion.Osney Mearl: Rlackwel! Scicncc L d , 1!)99. Figures 12.3. 18.3, 18.3h.
Klliatt T, I-Iatings M, Dessel het-ger U. L.~rium I\lut~s on ~Werlirnl iMirrohinir~gy,3"'Eififio?r. s n q Mrad: Rlackwell Science Lrd, O 1997.Fiprscs2,5,7.8,9, 1 1 , 12, 14, 15, 16, 17,1I~,20,%,26, 27. 29, 30, 34.35.52.

Mehta ,a. Hoffbrand AV. Iic1mnotology~f G/nnw. 0 0 ~ n e v rt Mead: Rlackwe?l Science Ltd, 2000. F i ~ w e s =.I, 22.2,22.3.
Pleasc let us know if your name has heen missed or rnisqpeller3 and w t will he happy tu make the update i11 the next edition.

PREFACE TO THE 380 EDITION

We wcre very pleased with the overwhelmingly positive student feedback Tor the 2nd edition of our U ~ t d p ~ p ~ d n d Clinical TGp~tles series. M1elf over 100,000 copies of the UC?J honks are in print and have been used by students all over the world.
Over the Fast two years we have accumulated and incorporated over a thousand "updates"and improvements suggested by you,

our readers, including:

many additions of specific boards and wards testable content

ddetians of redundant and overtapping cases

reordering and reorganization of all cases i both series n
a n e w master index by case name in each Atlas

correction of a few bctual errors

diagno'is and treatment updates addition o f .%20 new cases i every hook n
and the addition of clinical exam photographs within UCVAnatomy

And most important o all, the third edition sets now include f two brand new COLOR ATLAS supplements, one for each Clinical Vignette rerim.

* The UW-Bnsir Sr.in,cp C'oEmAtlas f S f q 1) includes over 2.50

color plates, divided into gross pathology, microscopic pathology (histology), hematology, and microbiology (smears).
The UW-Cliinlral Science Colw Arlas (St$ 2) hnq over 1 23 colol-

plates, including patirnt images, clermatology, and funduv copy. Each atkas image is descriptiveIv captioned and linked to its corresponding Step I case, Step 2 case, and/or Step 2 Minicase.

How Atlas Links Work:

Step 2 Book Codes are: Step 1 Book Codes are: A = . X ~ ~ a ~ u n r v R5 = Rehauinr~l Scicrlct KC: = R i n c h e m i s ~ r ~ W t = k,Ticrohinlnp3Val. I > ! h~$ic~~ol>iolop-.I1 I= ! l'nl. 1 P 1 1 1 1 l \'<>I. I r'l = I'atlirrphvsir~lr~fiv. 11 Vnl. F3 = P : i l h c r p h ~ ~ i n l Val. I11 o~,

ER = lirnrrjienc? Medicine IhI1 = lnrrrnal MrrIirine. Vol 1 IM2 = lrutcrr~allqedicine, K ~ I . I1

\TI I = Neu l . u I u ~ O =O R / m R

I'lCD = Petli;itnr% SCR = S u r ~ c - r b PSI.'= F~vrli~arry M(: = \{iniCiqr

rl l : r IMP3-032A

Indicates Type of Image: M = I - I ~ m a t c ~ l o ~ 11 = blic rnt,icjlokq I'C; = Crrrs5 Patlinlrqv PM = hTrcrn.;copir P a t h n l r p

ER-0356,ER-035B
Indicates U C V l or UCY2 Series

* II'thr Casc n u m h e r (032, 03.5, ctc.) is rlcll fr)llnwed by a letter. thcn zhur-t- i s oitly one image. Othcmjse ,I, E, C . D inclica~e up to 4 itnages.

Bold Faced Links: I n or-rter to g v c you access to the largest number of inlages possible. wc have chosen to cross link the Step J a n d 2 series.

IT the link i s bdd-racer1 this inclicatcs tl~al[ h e link is direct (i.e.. Step 1 Gasc with [he Basic S ~ i e n c e Step 1 Atlas link).
Tf llle link is not halbfilccd tltix indica~es that the Tink is indirect (Stcp 1 c a w %it11 CEinical Science Step 2 AtIxq link or vice

\rema). 1t7eh a ~ alsu implemented a FPW v

request:
Each current and firtur-e eriitinn or o u r popular Rrvr ,.lirItnr t h IcSIViL,F S / P ~ (Appletoil & Langr/McC:rxu,-Hill) ancl Fzrst ~ I Aid/?). I ~ IP!$il/ll,E': Stqh 2 (Appleton X. Laiige/MrGmm,-Hill) honk will he Einkcd l o the curl-rspnnrling IICV case.
s t r n ~ ~ ~ ~ i lTl I ~11p0n your C ~ ~ P S

Wc eliminatetl LTCX -+ First Aid links as the). f i e q ~ i ~ n t l v hecotne out ol'datc, as thc Firsi Airi bunks are revired ycasly

T l ~ e : l l Atla<is also specially cl~signed quizringCnoforcaptions a m descriptive and d o riot give away thc case name clirec~~ly.
' i hope the updatcd UrnT\erie? will rcmaill a Mr
I I I I ~ ~ and wcll~ I P

integrated st~lclv roo1 that prorides conlpact cliniral corrclations to hasic ~ c i e n c ~ information. Tlte), are deqignecl to hc easv and li~n (comparatively) rn rrad. and I~elpfi~l h n h Eice~~sing for exam% n d thc wards. a
Clrt: irlvitr: yorlr corrections and sriggestinns for thc fourth edilint1 or hese hooks. Fur the fir<! S I I ~ I ~ ~ S F ~ O I ~ factual corof caol~ reccion or t ~ vipetre that is selectecl for incluhion in the ~ v fourth edition. yozl will reccivc a per5orlal acknc>wledgrnerit r l i

tlw revised l>r)r>k.1 C inu submit ovcr 20 high-quality corructions, additiorls 01- new ~igncttcs will alco consirler inviting you to we become a "Contrib~itor" the 'book of your choice. If vou arc on interestccl i r ~ becnrning a poteiltial ' ' C o n ~ i i h ~ ~ to r " n r ".4uthor" on a f i ~ i i LICV hook, 01- wo~-ki~lg i t l i team i l l developitlg t ~ ~ ~ OUT additional tlooks. plensc alsu e--lllaiI115 y011r C,Y/rcsume.
Fl.TF prcfrr ihat VOII s ~ r h i ncorrections or stiggertinns ria i~ rlectronic [nail to UCVteam@yahoo.com. Plea~c include "Llndergrotlnrt Vi~,mctte<" tlie s~~l?jec yorlr n~ttssage.H' you as t of dn not h a ~ accms 10 e-111ai1, the following mailing addresq: c uqe Black~vcll Fl~l~lisllfng, UC,lTEditors. 350 Main Street, httn: Maldun. M:"IO"E 48, USA.

HOW TO USE THIS BOOK

T h i s series was originally developed lo artdress rhe increasing number of clinical vignette questions on medical examinations, incl~lding LrSMLE Step 1 and Step 2. TI. is also deqiped to the supplement and cornpl~mei~t popular Frrrt Aid fm t h ~ the ITSh.IIx St? 1 (Applcton 8: L.angc/ McGraw Hill) and Firs/ Aid frlr I ~ IL~IVIJ: 2 (Appleton Lange/McGraw Idill). P St$

Each LlCV 1 hook uses a series of approximately 100 "supraprototypid" cases as a way to condense testable hcts and associations. The clinical vig-nettes in this series arc desitped to incorporate as many testable fact5 as possible into a cohesi\.e and memorablc clinical picture. The t+iflettr% represent compi>sitrs d ~ a w n from geneml and ~ p r c i a textbooks, l~ reference hnoks, thousands of USMLE stdc qr~cstiorls and the personal experience nf the authors and reviewers.

Al~hougheach case tends to present all the signs,symptoms, and diagnostic findings for a particular illness, patients generally will not present with such a "complete" picture either clinically or on a medical examination.Cases are not meant to simirlnle a potential real patient or an exan1 vipette. All the boldfaced "buzzwordsv are for learning purposes and are not necewarilv expected to he found in any one patient with the
disease. Definitions of' selected impnrtanr terms are placed within the vignettes in (S~ZZLL CAPS) in parentl~eses. Other parenthetical remarks often refer to the pathopl~y~iologv mechanism of or disease. The forrnat shnulrl also help s t ~ r r t e n ~ ~ to present learn cases succinctlv during oral "hullct" presentations on clinical roulrionr. The cases are meant to serve as a condenser1 review, n o t as a primary reference. The information provided in this book has heen prepared with a great dc-nl of tlloughr and caref ~ research. This book should not, hc~wever, considered as 1 he yolrr ole source of information. Corrections. suggestions and submissions of ncw cascs are c*ncouraged anrl will be acknowlrdged and incorporated when appropriate in r r ~ h ~editions. re

5-MA
ABGq

AB\m ACE ACTTI-1 ADI.1 AFP

t u
AInS ALL ALT AWL ANA

m s
ASD AS0 AST AV RE

5-aminosaliytic acid arterial hluod gases adriamvcin/blr.ornyci~~ /\incristine/dacarbazii~e angiutensin-converting enn;me arlrenocorticatropic hormone antidiuretic hormone alpha fetal protein aortic insufficiency acquired immunodeficiency s ~ d r n r n e acute lvrnphocy~ic leukemia alanine rransarn inase acute myelogennz~qleukemia antinuclear antibody a d d r respiratory distress syndronle atrial septa1 defeci anti-stseptolysin 0

aspartate transaminaqe arterioven 011s

barium enema

BP
RCIN
TAD

CALL4 CRC: CI-IF CK CI,L CML

cm3
CNS
COPD

CPK
CSF CT CTA CXR DIC DIP DIU DM DTR.; DXT

blood pressure bloud urea nitrogen coronary artery disease common acute lyrnphublastic leukemia. antigen complete blood coullt congestive heart failure cr~atine kinase chronic lymphocytic lelrkemia chronic rn).elogenous leukemia cytnrnegalovirus central nen70ussJIstem chronic obstn~csivepulrnonars disease creatine phnsphokinasr cerebrospinal fluid computed tomography cerebrovascular accident chest x-ray disseminated in tramscular coagulation distal interphaIangea1 diabetic ketoacidosis diabetes mcllitus deep tendon reflexes drep ven t ~i i th rnrnhosis s

EBY ECG

Epstein-Barr vil-ur

clcc trocardiok~aplly r~l~ocar-~lio~r;?pl~~~ qjrc~inn fixction e s o ~ ~ l ~ a g o j i n ~ t r oc'no~cvpy d~iorl cleccromvography EM[; cndowopic retrc>~prlc rhr)latrgc)pancrea~ography E RCP ESR ery01rncl;te ~ctlirr~en t;tlion 1 tr fin-retl rxpi1alol-y vcllun~e m'iT fine needle aspiratio11 FN.4 ETA-tZBS fl~iore.;ccnttrrpol~clrlal antihr~dv ahuo~-ption nC r : fbi-crrl vili11 rapacily g l n m e r ~ ~ lJiErrarion rare i~r CiFR grnvth horn~ollc GH pstrt)intcstiri;~l ra ~te colnnv GM-CSF g ~ ~ n u I a c~~~acrlc>phage stiinulating l ~ l c ~ ~ ~ ~ geni touriui~r\ CU hrpa~itis \inis A H .4v h~unan choriorlic goriadoti-rqlh it1 hrC; HEEAT head, e\fe.r. cam, HOSP. :111d tllro:lt 11 tunan i m r n ~ ~ n ( ~ c l r f i c i r n Ics~ vi FI lV human 1ri1koc)rrcr a i i ~ i ~ r t ~ IL T4 Iiiszoc o [ present illness I-IPI 11~;ll.t rate HR hi~rnan h i r s i ~ n i n ~ gloh~tlirr n ~ne tIKlG hcreditiir?l S ~ I I P ~ C ~ C V I O F ~ S 1-1 s irlrnrificalion nnrI rhief rnnrplaint ID/GG infi~lin-dc-pendent rlialnctcs 1ncl1itu'i TnnM immunoglahulin I?7 insulin-like growl h I:lrtor TGF it~tra~n~~~cular IM ju~qilar vcnous pr*c.ss~x~-c. .FT

Erhcl EF EC;D

LLTR 1,lIM LES 1J-r~ I,P I .v LXW

I,vt es

MCHC MCY MEN

kirlt~evu/ur~1er/hlacIrlct' Ixclate drl7vrlrogcna~e lower e s o p l ~ i ~ s esip lli i ~ l r t ~ r ~ I~\'L'I- fltnctio13 PSIS I~rrnhar pzinct~lre left ventricu'tar letr v ~ I rir~ilaln liyperti.nl~lly elertl-olvtes tnean corp~rscular ~ c m t ~ ~ l : l o ~ > i r ~ I conceniration Itlean ror.p~wc111ar r.r)?~ime rnriltiplc endocrine nrcl1d:rsi;l

MHC MI MOPP

MR NH1, WDDM
NYC3

NSAlI) P.4 PTP P RS

PE

tnanoclol-tal gatnmopathy nf undetermined signihrance tnaic~r I~istnronipatihili~y complex rnyocarclial inCarc~inn incchloretharnine/~~i~~cristi~~~ (Oncworin) / procarhazinr/prudnisvnc ma~netic resonance {imaging) non-H(~cljikin's lymph oma non-ins111 ii14epencleti t diabetes tnrlf i t us nil per us (nothing by mouth) nonsleroidal an ti-inflanlmutov dnlg

FFT.5 PMI PbIN PT PTCA PTI-I PTT PI! D RBC RPR

RR R!
RV R\W SRFT SMI>H SLE.
ST13

TFTs (PA TSlH TfBC

TIPS TPO 'TSH TTP U-A UGI LS T

postercranlerior proximal in tel-phaIa1igeal peripheral bloocl smear pliy~icalexam pulnionaw functio~r tests p o i u ~ maximal inteusitv of polyrnorp honuclear 1 ~ 1kocvte t prothrombin tinlu percntanrnus trandarninal ;itlgioplasly parath~.roicl hot-]none r~artial thrumhoplastin time pep1 ic lilcer disease red ldoocl cell rapid plasma reagin I-uspiratoryrate Keed-S~erttbrrg (cell) right ventrirular sight ventricular hypertropt~y small ho~vcl follo-rv-th l-ough ~ycndromeof iizippropriatr sucrction of ADI-I sy-stcmic ! E ~ ) ~ I Le l - y t h ~ n ~ a c o ~ u ~ S ~exu;tlly ~rai-i~mittcd diseasc thyroirl r~lttctiontests tissue plasmii~ogeii i r a ~ o r art Ihvr-oirl-sli~rlzilatingl o m o n c l total iron-binding capaciq uansjup~lar intnhepatir portoqstemic shunt thyroicl peroxirlase rhyroid-sti~nulating hurmonr thrombotic tflrom I2ocvtopenic puvut;l t11-inalysis upper GI ul trasouncl

VIIRI,

VS
\rT MBC MTIV

XR

Venereal nisexse Re~earchLaboratory rimI s i p s i~c~>tricular tachvcardia white hloorl cell Wow-Parkinson-U'hi te (syndmme ) x-ray

ID/CC h 49-vvaruld maIe immigrant who is a nativr of Guam ha? sern

three doctors in his country ancl tried d f i r e n t therapies for

marked. progressive weakness of his hands and arms, difficulty speaking.muscle wasting in both hands, and troul~lesonke involuntary rnusck conhactions (wscrc~lr ,~TIONS).
HPL

He has no history of sensory symptoms, hlaclder or howel dysfimction, fever, exanthem, dog bites. vaccinations, o r spinal o r cranial trauma.
Lawer motor neuron signs: bilateral wasting of hands, deep rendon reflexes absent in upper limhs, rntlscle weakness, Eascidations; tipper motor neuron signs: poqitive Bahinski's ~ i g nstiffness and spasticity of upper limhs: normal fundus, , sensorv system, and cranial nerves.

PE

Labs

LP: CSF normal. Slightly elevated CK; norrrlal TSH, T2,and T, levels; normal sertxnl calcium and glucose. EMG: partial innervacion with abnormal spontaneous activity in resting muscle and reduction in motor units under voluntary concrol.
CT/MR, brain: brain normal.
Nonspecific atrophy on muscIe biopsy.

Imaging
Micro hthalogy Treatment Discussion

Lartrgelv supportive; disease is proq-sessive and faul.

N s n known a q

Lou Gehrig's disease, amyotrophic lateral sclerosis (ALS) is a slowly progressive, generalized motor muscle paralvsis

:!

AMYOTROPHIC LATERAL SCLEROSIS (ALS)

ID/CC

A 60-year-old nlillc. presents rvit11 speech difficulties.

HPI

The patient devclclprd this difticuliy rc~llnwit~gleft-sided a stroke from wllich he is currentlv recovering. IIc is a diabetic whcl hits t l e e ~o n insulin Tor I 0 ?leai-s. and h c is alsr) a chronic i smoker.

PE

Spwcl~ lacks fluency; patirnt 1 1 : ~ rlif'ficult~~ finding certain words and rornerilnes produces wrong word; comprehension i well s
preserved, as i l T r Fiiglier mentill I*~tnrtinns; ahiljtv tn reprnt is 11rlte1- I hat] spn nlalienw ~peech; assnciatrd rucovcrin# 1-iahtsided aphasia noted: motor wcakr~rss righ~ of upper anrl lawer Iim hs with rxaggeratcci tlcup tclulon i.efexes a~tri right-~idecl Bahinski's rt.flcx.

Labs

Elrv;~tt.rl hloc~rlglllcosr: retnaitirl~l- tests ~ ~ o r n l a l . ol'

Imaging
Treatment

CT': infarct in region of left frontopanetal cortex.

Speech therapy in addific~u o phvuic I t herap? for qtrt~ke; t Inngtei.rr1 lo\+*-clor;e aqpirin. Thi~ p;~ticnr has, RI-1u ' s dyspha~ii~ (exl>l.e~~ive, nonfluen t) with an aswciated right l ~ e m i p l e ~ i'Tl-ie hrnii~ a. rlnmage causing this rtmrlitinn ir he1 i~verl 0 involve the dominant inferior frontal 1 g y m s tl31inr:i's AREA). i n contrast to patirrbls with M'rrnicke'q iiphasiil. piltie111s will1 Broca's nphasia have insight into thuir

Discussion

HPI

[ell wrlically so that his heact hit the gmund first. Despite the injury. 11cis corlscio~~s does not report any neurologic and deficit, only ever-e pain in his neck.

Me

PE
Imaging

No neurologic deficit found oil clinical exarnir~aticln

XR, cervical spine: burst fracture of atlas with ring broken into four pieces.
(JEETEWONFRACTURE)
0
C-I

<

Treatment

I r t heren LIT uns~ahle fractm-e requilir~g halo jacket

immobilization

and Fl~sionif n n n u n i o r ~ occnrs.

Discussion The most crjmmun mechanism of injury in patients with JeTfersnn fracture is axial luacling. Othus cervical spine injuries include atlantoaxial fracture dislocation, more frequcndy associatrcl 14th tle~~rolngic clefici t ; rliqplacernen t is conirnonl~ antcriur, and treatment consist5 ol' skull traction followed by immobilization.\I violent flexioncompression foi-ce may result in a sudden prolapse of h e nucIeus pulposus of the cer\-icaF disk into the vertebral canal, prorlucing quadriplegia: here an early decompression is rrq~~irm.

F"/

C 1 SPINAL CORD INJURY

ID/CC

A 70-yearslid male presents with dull, aching pain in both calves

after moderate exercise.

HPI

The svinptoms starter1 a few month5 ago, h.picallv developi~~g after the pa tier^^ wal k ~ 300 to 400 yarrls; symptoms were d relieved after a few minutes' rest or when the patient sat down and stooped Forward (pseudoclaudication) . In addition r the o pain, the patient has exprrie~iced ~ ~ l m h r ~inshis thighs. He r e s ha? had 1 1 0 sphincter disturbance but has had low back pain for many years.
Spii-tal exam reveals loss of lumbar lotdosis and reduced flexion and extension of lumbar spine; tone, power, and coordination in lowcr limbs norma[; reflexes in lower limbs symmetrical but reduced compared to upper limbs; plantar rcfleses flexor; peripheral arterial pulses present both at re91 and after exercise.
Lab parameters normal.

PE

Labs
Imaging

XR, lunihar pine: lumbar ~ponclylosis with masked osteophpe formation. CT, spinc: Itunbar spinal canaI stenosis confirmed.
Surgery requiring laminec~omy varin~is at 1eveIs.

Treatment
Discussion

A number of mcchallism~ ma?7 lead to lumbar canal stenosis, including osteoarthritis wilh hlrpertrophy of the fhcer joints, disk prolapse, surgerv,spondyfnli~t llesis, Paget's disease, neoplasia, and infectinn; any of thcsr conditions m:tv be st~perimposed on a congenitallv ~ ~ a n - o ~ v canal. The anternposterior diameter cpinal n l the cord is narrowed dliring extension, which tcnds to cornprnmise t h e hloorl supply of the cord. resulting in the develo p m r n t o ' symptoms; stooping forward docs the reverw and l

CAUDA EQUINA SYNDROME

ID/CC

A 40-year-old male complains of the 'Lworst headache of his Iife" and double vision.

HPI

He has been projectile vomiting. He has no history of fever or neck stiffn~ss.

Papilledema on funduscopic exam; right eye deviated IatexdIy and downward (due to right third crarlial nerve palsy); other cranial nerves normal: no mening~al signs noted; motor system examination normal.
Routine laboratory rests normal.
Angio, cerebral: posterior communicating (PCOM) artery aneurysm. CT, head: enhancing mass impinging over right third nerve.

PE

z m

? 0
0

F U

<

Labs

f rnaging

Treatment

Endovascular or new-osurgical clipping of anet~rysm. Congenital berry aneurysms are associated with polycystic kidney disease and arteriavenous malformation; tlzep may rupture (during sexual activity, weight lifting, straining) and cause subarachnoid hemorrhage.

Discussion

CEREBRAL ANEURYSM

ID/CC

A I 4vcar-olcl w l ~te male coines into the e m e r g e n c y room i hecxuse of projectile vomiting and a vevere headache.
HP ha\ a hislory of ~inexplaincdshort stahre and polyuria.

HPI

PE

Papilledema and optic disk swelling (cluc to incruasccl i~ltracranial pr.c.ssurch)on ii~r~dttwnpic esarn: con h~ston: visilal licId testing reveal5 biternporal hernianopia; no other fr~c;~l rlr~~r-ologic signs: no ileuroclltaneous n~arkel-5 meningeal signs. or

Imaging

S R , sklrll: enlarged sella turcica. CT/MR: enhancing. cystic, 111111 I ilohulat ~ ( supraqellar mass with ring calcification: 1 h~~dl-ocephaltlq (due t o oh~tructionof fommcn of Monro and aqucdr~ct Sylvivius). of
Cystic mas.;with concen~ric arras of'calcificatinn. Mixture of squamnris epjrlielial e l e ~ r l r n ~ I I I ~lelicate i ~ l l l a r ; r ~ ret s v o m i ~gliosis seen ;II per.iphrry;cho1este1-01-rich cwtic fluid. : Slu-gical removal; mdi otlierapy. C:rilitiophar~giclmais he most common supratentorial brain tumor in children and i s rrnhr-yologically cIeri~ccl from Rathke's pouch remnants. Ir is a cominon cause oTgrowth srtarclalion, rlinl~ccc~ insipidus (compression of pi tt~imry), biiemporal ltrrn ianrlpia (complrssion ol' optic rhiaqm). m d I~cadaclir (nhqtr~rrtive ~ ~ r i s n c c p h a l ~ ~shows ;I birnoclal age diqtritnttion h It s ) . with a scconrl peak in lhc fifth decnriu.

Gross Pathology

Micro Pathology

Treatment

Discussion

Atlas Link

n mPEP3-006 - I

FCI

CRANIOPHARYNGIOMA

ID/CC

A 62-year-old man is brought to his family doctor beca~~se of rapidly prop-essive loss of cognitive function (DEMENTM) and excessive somnolence.

MPI

Five Years ago, h e received a corneal mansplant. His wife slates that she ha? seen a definite change in his personality over the past year,
Dementia; myoclonic fascidations; normal frinduscopic exam; no other focal neurologic signs.

PE

Labs

LP: normal CSF profile. EEG: bursts of high-voltage slow-wave activity and slow background.
CT, head: ventricular enIargcmcut and cerrhral atrophy. MR, lxain: increawd signal itltrnsity in affected areas. PET, brain: areas of diminished glucose metabolisn~.

Imaging

Micro Pathology

Brain biopsy shows amyIoid depositio 11, spongiform degeneration, decrease in ner~ro~ls cerebral cortex, and assrocytir of proliferation; no inflammatory changcs sccn.
llsl,~ally fatal; vidarahine and amantarline are being tried.

Treatment
Discussian

A subacute spongiform cncephalopathy with a very long incubation period, Cruntzfeldt-Jakoh disaase is presumably caused b y a slow virus or prion and transtnitted via coi-neal transplants, dura
mater allografts,contanlinated cadaveric growth llnrmonu, or neurosurgical contamination. Lithium overdose mav mimic

signs and symptoms.

CREUTZFELDT-JAKOB DISEASE

ID/CC A 35-year-old woman known to have rheumatic mitral stenosis

awakens in the morning to find the right side of her body paralyzed.

HPI

The pasient also complains of palpitations. She has no history of fever. neck stiffness, vomiting. Iteadacl?c, o r transient ischemic attacks (TLAs).

PE

VS: na fever; irrrgularlv irregular pulse. PE: dcnse right-sided hemiplegia; brisk reflexes on right side; right-sided Babinski (FYITNSOR PL.Z~T.AR RESPOYS~)present: f tindtis normal; loud S1;
apical micldiaqtolic mnrmur ant1 opening snap.

tabs

ECG: prcsence or atrial fibrillation confirmed in addition to P-mi~rale. Blood culture sterile; roil tinr lab lest5 normal; riotting tinre. bleeding time, and PT normal.

Imaging

Echo: left atrial thromh~is. CT: scan perrol-med arter 24 hours reveals infarct in posterior limb of left internal capsule.
Start heparin after foI10w-up; therapv guidcd with PT,digoxin for management of a~rial fibrillation; valvuloplasty or valve rpplacemen t after resolution of Icfr atrial thrombus.

Treatment

Discussion

Mitrat stenosis with atrial fibrillation predisposes to thromboernholism.

CVA, CAPSULAR INFARCT

ID/CC

A 65-year-old white male develops sudden severe headache and

right-sided hemiplegia.

HPI

The patient is a h o w n hypertensive and takes his medication irregularly; he now has both urinary and f e d incontinence.
VS: severe hypertension (BP 210/ 180); no revel-. PE: dense right-sided hemiplegia; funduscopic exam reveals presence of papilledema in addition to hypertensive retinapathy; right-sided Babinski; eves deviated toward left; n o meningeal s i p s present.

PE

z 2"m

; o 0
0
G,

Labs

Routine labs normal; LP not done, since intracranial pressure {ICP) raised.

Imaging

C T , head: facd hemorrhage in left putamen region of basal g;inglia.

Autopsy: mass of bIood dissecting through parenchyma inlo deep structures of brain and ventricles.
Hvpertensive change., seen in adclitioil to putmenal hemorrhage; hyaline arteriolosclerosis:lipohyaliaosis; CharcotRouchard aneurysms.

Gross Pathology

Micro Pathology

Treatment

Supportivc management to reduce ICP and blood p r e m .

Discussion

Bleeding is niost often caused by hyperfension. In the preserlce of moderate to severe hypertension. d penetrating arterioles may rupture deep within the hrain, causing a hematoma that displace? hrain slructurps. Common sites are the putamen, thalamus, pons, and cerebellum.

CVA, HYPERTENSIVE

ID/CC:

h I!+=-ar-old remalr Olvmpic it~orsebackrider is brought into the emergeucv room with headache, codusion, weakness o f the left side o f her body, blurred *ion, and projectile vomiting.

HP3

Three Iinltrr ago, 411e hit thc right side nf her head when shc fell frnrn a horse during a training exercise.SIle lost consciousness For I minute ancl then appeared t havr recovered completely o before prewnting with the syrnptomatolop ((crrcrn ~ N ' F . R V ~ I . ) .
VS: RP mildly tlevatud; bradycardia. PE: papilledema: right~iderl mydriasis; dfel-ent pupillary reflex a h n o r m a l i ~ right on side; deviation of right eyeball outward and downward (RIGHT CN 1 1 PAWY); left-sidcd weakness; brisk reflexes on left side; 1 extensor plantar reresponse left side. on

PE

Imaging

CT/MR, head: r i ~ l trmpnra'al hone fracture; right-sided ~t

lens-shaped (convex) hyperdense extra-axial fluid collection.

Gross Pathotogy

CoIlection of blood hetween dura mater m skull wihrith mass i 3

effect.

Treatment
Discussion

Emrrgcn t surgical ctacuation.

The results of arterial bleeding (rupturc of middle meningeal artery) are ~~siially assnciated v5 th skull fracture. Classically. the . patient loses consciousness immediately aftel- Ileati irljur): but rejiains consciousness and remains asvnlptomatic for a vasiablr perinct of time before qmlptoms worsen.
IG-P3-010

Atlas Link

EPIDURAL HEMATOMA

ID/CC

A 19-ycnr-old male visit-5 his orthopediqt hecalrsc of a wide-based gait (ATAXIC: GAIT), congenitdl clubfoot. anrl abnormal lateral curvature of the spine (st:o~.rosrs).
For the past scvrral years, lie has l ~ a d increarring difficulty walking: the procefs l x g a n snhtly, hut Ile is now incapable of participating in sports. I-Te also complains of a proLgressive diminution of vision.
ITS: arrhythmia: tachycardia. PF.: hi131 concentric cot~traction era1 of\.isuill f:elds on viwal field testing; findings slrggestivc of retinitis pigmentom r ln retinal exam; scoIiosis of thoracic spinc; pcs c a n i s def(3rmity of righ ht foot; dirninish~cl sensatioil in stocking-glove distribution; proprioceptive sensory loss; areflexia; ataxia of li rn bq: Rabinski's sign: forceful pulse; prominentJVP;slutained apical impulse; S4 ( r l ~ to e ~ hypertrophic cardiunlyopathy).

HPI

m zW C
; 1 E

PE

5 2 b m

<

Labs

ECG: leCt ventriclrlar hypertrophy: invrsled T waves.

Imaging
Micro Pathology

Echo: evidence of'hypertrophic ol7strr1ctivc rcadiomvopathy.

Marked 105s of cells in posterior root ~ a n g l i a and degeneration of puripheml sensory Fibem: po5rerir)r atlrl lacel-al colun-ins of CNS a l w affected.

Treatment

En treatment available.
Tile mc)st cominon hel-edicary ataxia. Friedrcich's ataxia is an autosomal-recessivedisorder d ~ to a drfective gene on c chromusomc I),

Discussion

I DJCC

A 60-year-oldwhite male complains of headache that is worse in the mornirq along with occasional nausea and vomiting for
6 weeks.

HPI

One day prior to presentation, he had an isolated grand ma1

seizure.

PE

Bilateral papilledema: loss of recen r memory: brisk deep tendon reflexes on right side: Babinski on right side.
CT/blR: irregular enhancing leftsided mass with center; mass effect and surrounding edema.
necrotic

Imaging

Gross Pathology

Hemorrhagic and necrntic tumor mass in fillrating left parietal

lobe.
Micro Pathology

Biopsv reveals presence of anaplaqtic cells with pleomorphism and endothelial proliferation: foci of necrosis surroltnded bv palisading. Surgical resection: chemotherapy: radiotherapy.
Astrocytomas are graded accnrdin~ differentiation; the to highest grade (grade IV) i glioblastoma multiforme. It carries s a. poor prognosis.

Treatment
Discussion

Attas Links

PPEP3-012, PM-P2-012

GLIOBLASTOMA MULTIFORME

ID/CC

A SRyear-nld male visits his family doctor complaining of syrnmeb-ic muscle weakness that started diszally in his legs and ascended gradually, now involving the trunk and arms.

HPI

One week ago h e suffered frotn diarrhea and fever and was diagnosed wit11 and treated for UnmpyIobcart~r enteritis.
Syrnmebricd proxima1 muscle weakness and flaccidity in lower limbs; ahsen t deep tendon reflexes; normal sensory exam; normal cranial nerves.
Elevated gamma globulin. LP: increased CSF protein concentration without cellular increase: normal glucose. Nonreactive VDRL: decreased nerve conduction velocity indicative of demyelination on electrop hvsiologic studies.

PE

Labs

Treatment
Discussion

Flasmapheresis; intensive care and respiratory suppnrt. Gnillain-Barre syndrome i s a common causc of palyneuropathy in adr~lw that is usr~ally preceded by GI nr respiratory infection or by specific illnesses such as Epstein-Barr, Chmmfllohnrfm enteritis, and cytomegalovirt~si~lfection. Respiratory paralysis

GUILLAIN-BARRE
- -

SYNDROME

ID/CC

h 50-year-old male preseu FS to tllc eincrgcncy room with wild,

flinging movements of his left arm and leg.

HPI

H e has b ~ e n rliagnnxrrl with diabetes and hypertension but has taken his inrrlicarions nrllv i r r e g ~ ~ l a r l I-Ie i s also a chronic y. smoker.
L'nconunllecl, violcnt, rapid flinging movements of Ieft arm a c nF leg; remainder of neurc>logic exatrt nonnai.

PE

Labs
Imaging
Treatment

L.ab t m t s reveal ~Ievated l>looclg l u r o ~ ~ .

(T( d n n e at 48 hour=,):infa]-ct in r-ight subthalamic nucleu*.

Phcnothiuincs and d o p a m i n e antagonists such as sulpiridc and tr.lriibunazine mil)' ke o f l i ~ l p . I-IumihaPlismr~s ch;~racteri;.edby f o r c e f ~ l l ,flinging., and violent is inrwements. primarily ol' the proximal parts of thc. limbs of one .;irlr of I he body. rliat disappear cli~ring s1cc.p. The mosi cornmoil e t i n l o p i s that or a vnscdar rrTcnt thr contralater.al in srihthalamic ntlclc~~s: nther C~ZIISPS include an expancling artericvenouq rnalfi~i~ma~ I rail tna, nttnnr, and mu1ti pie sclcrusis. ion, tlosl cases resofve spnntaneouslv within 6 to 8 week$; howcber, $111-gervInay he inrlicatecI in caws nf in~ractahlr involuntary

Discussion

HEMIBALLISMUS

ID/CC

sZ 42-year-oldtnale presen r q with depression, pnor memory. and jerking movements of the limhs and fingers.

HPI:

His father died of a similar rnnilitiml in which thc symptoms progressively worsened, proceeding to demen ti3 t ~ ti1 h i 5 death n at the age o f 50.

PE

Chorea; psychiatric emluation rrveals cognitive impairment (i~~arrention poor concentration ~vichorit and memory loss) and depression; n o other focal neurolo.gic deficir Fo~~nd.

Imaging

MR, hmin: degeneration nf caudate n~~cleus. brain: cci-cbral CT, atrophv.

I,oss nf lxain mass wit11 striking atrophy nf caudate nuclel~s ~ d , a less stt-ikitlgly,plltarnen: secatldarp 105s of neurons in gIohus pallidus; cortical atrophy moqt commonlv occurs in Frontal lobe.

Gross Pathology

Micro Pathology

Degeneration oF piny GABAergic neurons in thc striaturn lcads to a net loss of inhihiton7 signals from the striaturn.

Treatment

No specific trP;Itmen 1 wailahle: stipportive and ytnptomarir treatment; genetic cnunseling with regard to ~ I ~ L I I - c offspring.

Disc~ssion Huntington's chorea is a n a~itosomal-dominant disease whocc gene Incus is nn clirotnasorne 4. I t is ratwed hy expansion of a trinlicleotide repeat (TAG) within t h c Huntington genu; cxpansion of the trinucluotidc repcat leads to greater frecjl~ency of disease in s~lccernh~e generarims (C;FNPI-IT:ANT~CIFJITIOV). h r T nnset arthe disease is typicallv hetween 30 atlcl50 years of age, pm,qressing to dealh tvi thin 15 to 20 years.

HUNTINGTON'S CHOREA

A 25-vear-old male is hrought to a neurologist with compIaints of inability to see on one side.
Two months ago he suffered right eye optic neuritis,hut his vision has s i ~ i f i c a n t l y improver1 since then, aIthough it is not completelv ~lormaE.

O n lateral Kaze it3 either direction, one eye does not adduct and the other has nyshgmus on abduction (finding characteristic of bilateral internuclear ophthalmoplegia) ; Cunduscopy reveals temporal pallor s f right disk (due to atrophy of papillomacular fibers); visual field testing reveals righr par-acentral scotnma: flexion of neck produces an elecbical sensation that runs down back and into legs ( I , P ~ E R M I ~ ' S SIGN: snggest? intramedulkary disease OF cervical cord).
Labs LP: specific increase in CSF IgG concentration. Aprose electrophoresis reveab oligoclonal hands in IgG region OF CSF. Evoked-potential ~tuclies visual, auditory. and somatosensory of pathways indicate impaired responses.

Imaging

MR, brain (T2W): investigation of choice: reveals multiple,

discrete, whfte-matter plaques.

Gross Pathology

Sharplv defined area5 of grav discolol-ation ( I S L ~ Q ~ E of white S) matter that occur particularly frequently around the ventricles and in the corpus caIlosum.

Micm Pathology

Activr plaques show evidence oC myelin breakdown, lipid-laden inncrophages, loss of oligodendrocytes, and relative preservation of xxons; ljmphocytes and mononuclear cells prominent at edges of plaq~ies. Beta-in trrfPron; immzine~uppression(corticosteroids. azathioprine. cyclnspnrine) , hi1t success has heen modest.
The following are suggestive of m1 tiple sderosis: (1 ) optic i1 neliritis, whose early s i p q include diminished visual acuity, central or paracentral scotoma. hyperernia and edema of t h v optic disk. and a defective pupillary reaction to light: (2) inter11uc1earophthalmoplegia ( d l ~ e dernvelination of the ~nedial to longit~~dinal Fasciculus); and ( 3 ) Lhermitte's sign.

Treatment

Discussion

INTERNUCLEAR OPHTHALMOPLEGIA

ID/CC

A 13-year-old male is brought to a physician hy his parents for an evaFuation of recen tlv ohserv~d overkddgence in sexual activities.

HPI

The parents also report that the patient's behavior has recently changed markedly from aggressive to extremely placid; directed questioning reveals that he has now started exploring things orally and has dev~loped voraciolls appetite. H e sufferer! from a herpes simplex encephalitis a Tetv months ago. There is n o history of prier psychiatric illness in the patient or in the family.
Patient is in exceIlent health and i s apparently unconcerned about his illness. displaying no reaction to parentq' complain t-7; when physician attempts to shake his hand. patient begins to orally explore it; on ~ e e i n g nurse in doctor's room, he starts to a

rn z " PP'

c
r o

c ' a <

PE

masturbate.

Imaging
Treatment

MR/CT, head: bilateral temporal lobe and arleygdala darnage.

No specific treatment available. Kli~ver-Bttcvsyndrumc is a syndrome of hyperphqia, hypersexuality, placidity, and hyperorality. In experimenta1 animals, it r e s ~ ~rrom bilateral removal of the amygdala; in humans, an LF l incomplete picture is generally seen secondary to extensive temporal lobe damage, as may occur during herpes simplex cncephaIitis or in degenerative or post-mumatic brain damage.

Discussion

ID/CC

A 6-year-ald maIr i s brought tu tile emrrgetlcy room wit11 acuteonset projectile vomiting, severe headache, and blurring of vision.
The parietlz reports unsteadiness of gait that has progressively wor~rned ovcr the past 2 months. He has no hiftnry of seizures, fcvc.~, r neck stiffness. o

MPI

PE

Papilledema: no meningeal signs; nyta5rnuc in all dirrctions of ga;.~: trlmcal ataxia; cranial nerves normal.
CBC: mild arlett~ia.

Labs
Imaging

CT/MR. bfi~irk: hornogeneo~ts, enhancing maw i cerebellar n vermis compressing and filling fourth ven trick; dilated third and lateral v e n ~ i c l e s (due 10oh<truc~ivu h?clrocephal~~~).
Soft. wuIl-circ~im~cr~he~l, liah t-gravisli mass o n cerebellar vcrmis.
Once intracranial Ilressurc (ICP) is controllrd. CSF on lumbar puncture shows malipan1 celIs: highly malignant tclrnnr charactrri;lc.d bv deupfv staining n ticlei w i t h want ~\~opla5111 arranged in pseudomsettes.

Gross Pathology Micro Pathology

Treatment

C:oriicosteroids for increased ICP; entire nc-urnis irradiation; surgical extil-pa tion; c hernothcrapy.
,4 common tumor. of childhood and thcr most prev;~lent brain tunlor in clrildrcn less than 7 years of age, medullohlastoma is clacsified a%a primilive r ~ e ~ t m e c t c ~ r l ~ rtilttlor (PNET). mal

Discussion

Atlas Link

I 1 - PPG-P3-018

h 40-vex-old man cnmplains that "the whole room seems to be spinning" (VERTIGO) whilc also experiencing ringing in the ears
(TINNITUS)

and nausea.

The p x t i ~ n also curnplaitls of a sense of fullness in his cars ancl t acids that his hearing has progressively diminished over the p;wt few years. TJis symptoms were initially unilateral but have now become bilateral. His ilInes5 has run a course o f remissions and relapses. I-Ic clenies any weak~lpss C the liml>f and Elas n o n I I ~ P I O T ~ of chardi~cIiargc trauma. or
PE

VS: normal. PE: anxious; neurologic exam normal: caloric teds Iilaterally nor~nal.
Pure-tone a ~ i d i o ~ n e treveals sensorineural bearing lass that is ry more markod for lower frequencies; Iolldness recl-~~itrnen t ~~rr.st.nt; short-increment senqi rivitr; index (S1ST)shows high scow; \QRL ncgativc.

Labs

Imaging

MRJLT, hcad: normal (perrormed to rule out intel-nal auditnl-v canal pathulugv) .
Gross distention of the endolymphatic system ( E N D C > L ~ V ~ ~ T ~ ' ~ C HYDROPS) .

Micro Pathology

Treatment

N o specific treatment; symptnmatic relief wit 11 vestibular suppressan$ diuretics, and low-soclium diet. Surgical intcrventiorr i~ ~ u n t ~ o v ~ r s i a l .
Mi.niPse1< n disease of t11c inner c a r characterizerl by acute is onset and recurrent attacks of vertigo; it is often associated with nalivea and vr~~niting together with diminished hearing and tinuitus. iUeho11g.11the exact etiology i s is~niknown. rnclolymphar ic liydrops (clue to uxccss of t n r l o l ~ m p h the scala media) in has been linked tn ohsisuction of resorption, rtefective
membrane exchange, and incrcasccl cnclol!mph inflow (secondary to alltrgy, vasomotor Fact nrs, o r retair~ed sodium ant1 water).

M ~ N I ~ R E ' DISEASE S

' 6O-yearold woman is seen with complaints nf having dirficulty 1 walking and lwo episodes of involuntary hand jerking
( PARTUL SEIZL~RF,).

Her attendant I-rvealsthat over the past few months her memory has deteriorated. She has slow mencation and urinary incontinence. She is not diabetic 01- I~yperrensive. Funduscopy reveals papilledema; tone of lower Iimhs i n c r ~ a s e d and strength rerIiiced (sP.~.%T~cK ~ P ~ R E S T S ) PA ; deep tendon reflexes exaggerated; hilateral Bahinski.
Labs Imaging
Rolltine laboratory
te-stq normal.

XR, s k ~ t l l hyperostosk of left parietal hone and sagittal suture. : CT (with contrast): left parietal pamqagittal tumor, MR (gadolinium):intense tumor enhancement; d d extension and invasioii into superior sagittal inu us.
Irregular. firm, gitty mass arising superficidly. indenting and compressing braEn but not invading it.

Gmss Pathology

Micro Pathology

Wherling pattern of meningotheliaI cells with regular, oval nuclei, indistinct cytoplasm, and psatnmoma bodies.
Surgical resection; radiation fur unresectahle cases.

Treatment Discussion

Meningioma iq a primary intmct.ania1 neoplasm arising from cells of the arachnoid granulations; it is chilracterixed Ilv slow p w t h , benign behavior, and expansile rather than inF1tra.tive growth. Common sites involved include the cerebra1 convexity, parasagirtal area (aq in this case), sphenoid wing, olfactory F o r w e , cerchellopontine angle. fomtnrn magnum. and spinal cord. The tumor is usually solitary, is more cc~mrnonin women, and is found in middle and later ages. Multiple rneningiomas mav he found in patients with neurofihromatosi~tvpe 2.

Atlas Links

ID/CC

A 59-year-old whitc female presrnts with a severe, dull retroorbital headache, vomiting, and diplopia.
She has smoked two packs of ciprettes a day for 22 years and has hcen diagnosed with lung crancer.

HPI

PE

VS: bradycardia; mild hypertension. PE: papilledema (due to

increased iritracranial pressure); right pupillary reflex abnormality i efferent pathway (due to right oculomotor nerve n palsv) .

Imaging

CT/MR: rouncl, discrete, ring-enhmchg lesion in right frontal lobe; surrounding vasogenic cdcma; shifting of midine structures to left (> I-cm shift considered severe).

Micro PathoIogy
Treatment

Biopw shnws small cell carcinoma.

Cansider ~urgicalreseciion; radiation therapy; dexarnethasone (to control in tracranial pressure).
BIood-borne brain metastases comrnonlv occur in patients with systemic malignancy. Common primary cancers that rcsult i n intrarranial metastasis are lung,breast, GI. and GU cancer and melanoma.

Discussion

Atlas Link

TTET3 PM-P3-021

METASTATIC B R A I N TUMOR

ID/CC

h 20-year-old woman complains of recurrent. throbbing headaches associated wit11 prr~foundnausea nnd light sensitivity.
the one side of her hcad. She also report5 seelng ''flashing lights" like I i g h ~ n i ~r~loving lg across Ilel- lielcl of visiot~. Stress, sleepIessness, and anviety ~ ~ s l ~ a l l y precipitate thest. Iieadaches. Her mother suffcrh horn migrairlc l~carlachcs(positive fiirnily hisror y ) .
Shr
tl;~.:

HPI

l ~ qirn ilar hearlac ties several d

limes each vcar sincc

o r l ~ e t hel- m e t ~ ~ t ~ - lp~riorls. or ral The 11cadachesO C C I I ~011

PE

YS: r~orrnal. PE: fr~riduscopic exam and vis~ialfield resting tlol-lnal: 11e11tc1logg.i~ rvarm ~~t,rmal.

Treatment

Prup11:-lactic thurapv wilt1 avoidance of precipitatin~ factors and rlt-\~g< as beta-l>lockers,~riryclfcnrlticiepl-ttwi~nrs,or calcil~m SU'CII cl ~ a i ~ nl1lockps5: ;ll>c~ri v t~ el i hvr.;~p\' r3~1ring acute attacks with NSAIDs, sumatriptan, crgotaminc, 01transnasal butorphanol .
Migraine heacl;~chej q the second most common callce of primary headache ( t h e mmt cornrllclrl in !lie ITnirerl State5 is tension heaclachc). 111 thc United Statcs, an cstin~ated 17%'of women and 6%of mcn arc affcctetl t ~ 111isdEsnl-iI~r, y The hfilditdie is ch;lra~teristic;llly preceded hy rl prodmme and i s episodic, grarl~iiiliri onset, usuaIly unilateral. and most conl111orllvi t l rhe temporal area. PI-ecipirati 1 : C a r t o n may ir~cludc 11 rtjerlwks,t'ns~ii~g. rmotional strcss, 3 r d foods cor~tai ning h-l-atnine, ~n.onr)~r~rli~trn gll~ianiate, t~iu-ites. nlTIlr m u s e is unknown bllt appears tn invnlve variations in crrcl~ral hloc,rl flow and .icrotoi~crgic pathways.

Discussion

IDJCC

PI 36-year-old white L'etnaIc p;1j75a n einergencv visit to 11cr

opE1~11alrnologist hecarwe of loss of central vision and pain on movement of her left eye ( d u u to optic neuritis): she also presents with scanning spucch and intention tremor in the hands.

HPI

Five years ago, she c r n i g a t ~ d rhr Unitecl Shtes l r s m Sweden. to She has hecn s~fFrring from recurrent paresthesias in the hands, arms. and Iegs; weakness in the l e and arms: vertigo; and ~ Madder urgency (mu1tiplc unrulatocl nerirologic yrripcntris). I ler Carnily doctor rolrl her he had "3iv~reria" and recorn-

5
c;1

PE

Dimirlishecl visual acuity; ccntral scotoma found on visual ficld charting; hyperemia and edema of lcft optic disk: defective afferctlt p ~ ~ p i l lt-eac~ion light in lefi y e ( . ~ . Z R ( ; V SGUNK a r ~ ~ to PcPIL); paresis of medial rect~ls mtrscle on lateral conjugate gaze but not on comer-gence (BIWTE~LV.1K'l'EKNtlr:LL~R I ~ H ' T I ~ A L M O P L E G;L ~ ) O mystagnus in a l ~ d u c t i i ~ g ; r l e c ~ ~ i csen~ation it nning down ey il. r back and into legs producecl t>y neck flexioi~( L F I E R M ~ X ' S SIGN): leg spssticitv and irlcseasecl ckcp tendon reflexes.
1.P: marked increase in CSF IgG concentration; prescrice of oligo.oclond bands in IgG region on CSF agzarose electrophoresis; CSF mthe~~wire normal. Ahnorma! visual, auditory, >and somatoseilsory evoked respoilses.

Labs

Imaging

MR, brain: multiple discrele high T2 signal ah tlnrmarities in perivcntricular and ot t1t.r whi t r ulatter al-ras (uspcciaTIy corpus
CalIosum).

Gross Pathology

Pathologic haIlnlark 01 disorder coilsists of distiilctive small gray plaques of dernyelination prrsrnt in I N S wllite nlartel-; oplic ne111,itis. Demvelination ancl glicrqiq; lipirl-laden mac~aphagt-s. Mainly suppol-tive;cor,tico.itc-roids:ACTI-I: azattlioprinc: cvclophorphamidr. MuItiplu scPcrosis is a n idiopatllic deinyelinating rlisorder whosc courw is market1 hv intermittent remissions and exacerbations.

Micro Pathology Treatment

Discussion

Atlas Link

'CIT1-l PG-P3-023

?!

MULTIPLE SCLEROSIS

ID/CC A 2,i-year-old Female has had marked weakness ancl drooping of

the eyelids ( P ~ S I S ) the evening T r the past 4 werks; she does in o not experience anv weakness in the morning follo~ving good a

night" sleep.
HPI

She has also been suffering from double vision (nr~~.cn*m)h e at end of rnch day.

PE

Ptosis developq on sustainrd elevation of eyelids: hysphonia develop5 as patient is asked t o narratc complainrs ;[ lengih; I wealmess of Forward flexion of head develops after repetitive resistance to force;patient could not maintain lzer lipper limb in ahdlicted posiriorr for more than a minute.
C I e a r a t improvement in strength with edrophonium administration. EMC;: progressive decrement in voltage during repetitive, low-frequency stimulation of motor nerve. Positive serum titer of antibodies to acetylcholine receptors.

Labs

Treatment

Xc~tylcholinestrra9e nhihitors (pyridostigmine) ; prednisone; i thymectamp; piasmapheresis.

Myixsthcnia p a r i s is an autoimmune disease that is due to the rlevelnpmenr of specific antibodies to one or more acetylcholine receptor subunits,reducing the availabilip of acetvlcholine

Discusion

ID/CC

An obese 35-year-old male goes to a clinic because of dktal muscle weakness i n both upper and lower Iimbs and gradual diminution of vision.

HPI

His faher suffered from a similar muscular weakness. The ~atient alsn suffers from mental retardation. 1

zm
C
70 0 r

PE Fmntal balding; typical facial wasting; bilateral cataracts; distal

muscle weakness in both upper and lower limbs; difficulty releasing grip after h audshake; percussion over tongue and thenar eminence reveals myotonia: mildly reduced deep tendon reflexes; normal sensory exam; moderately atrophic testicles: cqrdnovarus deformity of both feet.

a 0 <

Labs
Imaging
Micro bthotogy

Decreased plasma IgG. EMG: myopathic potentials; myotonia.

ECG: nonspecific ST-T changes.

Muscle biopy reveals iuternal nuclei (nuclei in center of the fiber rather than i periphery), type I fiber atrophy, and ring n fibers.

Treatment

Phen~loin: carbarnwepine; quinidine; procainamide; acetazw lamide; surgery required to correct Foot deformities.
The most cornman form of musc~~lar dystrophy among whites, myotonic dystrophy i q transmitted as an autosomal-dominant trait. It is associated with a genetic defect that encodes myotonh protein kiuase; the myotonic dystrophy gene locus has been

Discussion

MYOTONIC DYSTROPHY

ID/CC

A 5-year-old male is rrfkrred 10 ;I ~perialist his physician for hy cwli~;ltir)n arl abdominal mass and a rcccn tly no~iced nf IF ft-sided o r b i d proptosis.
His parcnts complain of cveigl~low, poor feeding. and a con tint uous lou,-grxlc fcvcr for the past few months.
PV.: mar-ked cachexia: Icft-sided orbital proplnsis and ecchvmores; large, smooth intra-abdomiaal mass palpable.

HPI

PE

Labs

XVarkcd elevation of urinary catecholamines and rnel;tholil~s var~illyltnai~d~lir (\%$A) n i ~ d acid honiovanillic acid (m'h).

Imaging

C , hdon~eii:intra-abdominal mass arising from and obliterating Ta left adrenal $and. N u t (hune w a n ) : rnrtarratic lvtic lesion in left ~~-11ira.11 rexitrn o f sk111l.
Sr~lirl, ro~xnrl saft-tumor mass o b l i t c r a t i ~ ~ g adrenal gland; left gray on cut surface ~ I l o ~ v i n g extenxivc hemorrhage a tic1 nprr-osis with c v s t formntion. Anaplastic, small, rcmnd-to-uval li yperclir-c~waric cells uvith scant cytoplasm in sheets aild at placcs fornriug Homer-Wright pseudorosettes; fmv ganglion ceIls wen: c l ~ c t r n l microscopy l reveal<pt-esrnce or neurosecretory granules. Srirfiical rc-st-ction:clnei~iotherapy with ~ c l o p h o s p h a m i c l e and ;idriarnvcin. h'currjhlastonia is a prirnarv malignant nenplasm that ariscs from irn~narut-e cellv nf thr adrenal meclulla and sccretet; c a t e c h n l a ~ n i n ei~ ;usually nrcul-5 in children under thc age of t .5 a n d presents with a n ahdomin;~lmas\. Ke~rrol>lasrornas Innst cornrr- only ni.lginar~in the adrenal glands hut m! also arise in a! the retmprrironpal sympathetic ganglia, pervis, neclc, r )l- posterior rr-ieciiasti~~um. Hutcl~inson'..;rle11r.c)l>laqtomapresent5 with uxlctlsi~r. skrill arirl orki~al melaqtaws that prnducc cxophtl~almos: rnctaqtases to lynlpll nodes. Iiver, lung, aiid hone are mmmon.

Gross Pathology

Micro Pathology

Treatment

Discussion

Atlas Links

IDjCC

A 60-vear-olcl male is seen l a ~ ~ e u r n l e g iror an evaluation of y sl deteriorating cognitive skills.

Over. the past Few wreks, the patient has sta~~ecl fled and has in urinary and b o ~ ~inron~inence. el

HPI

11itcl

PE

(:ogriitinn impairccl; irnpaircd ant hi il;xiion tvithnut eviclencc of primary motor; sensory, or cetehetlar r l y f ~ m c t i o n( G ~ ~ . L P R ~ Y ~ .dcep ~ P I I C I O ~ > reflexes in tart; pupils equal, rourid, and ; ~) react ivr lo light anrl arrommoclation; plant;trq l~ililterallyIlexor; h~nd.rls cloes not reveal ;my papilletfern;~.

z rn
C

0 r 0
t ,

<

Labs

Lr: nurmal opening pressure. I,ah parameters w tl~iririor~nal i lirnits.

CT: ventricular enlargement with relatively little cortical atrophy. N ~ z cc i s ~ e r n o ~ ~ a p persistent a c t i v i ~ radionuclide in lateral , hv: of vun~riclcs after 48 hours (characfcristic OF normal pressure hyclmcephal~~s). Tlisertir>nof a vcnri-ictiloperitonealslrunt.
P s ~ ~ ~ d n b ~ ~ t h x is- due to bilatcral dvsfunctiori of the cortit cohulhar t c z c ts. In acldirio~ilo clysphagia, ~lpsarthria. and 11yerart i ~ cgag r e f l ~ x e patients may ecxpcsicnce episodes of . ~. spnntxnroils crying o r Inlr~hter. most patic~lts, c a w e o f In the nn1.1nalpl-rssure hvclrocephalus is ncl t k r ~ o ~ m , l i n ~ ~ gittmay all l Fc~llo~u .;l~harachnoitl rl l~ernori.h;+ge tneningitis (5ometimcs otyears later). The value s f its carlv diagnosis lirs in the k ~ that~i i c is a treatable dementia.

Imaging

Treatment
Discussion

F*s"

NORMAL PRESSURE HYDROCEPHALUS

-

ID/CC

;4 ilGyear-old white

male complains t his inwrniqt of increaso ingI~. severe headaches upon awakening of a few monthq' duration: the headaches persist r hr.o~~gt~outafternoon and the
are mild in t h e evenings.

HPE

U%iIc in the doctor's office, the patient suffers a seizure and i s brought to the crncrgcncy room.

PE

Funduscopy reveals papilledema.

Normal.

Labs
Imaging

CT/MR: large frontal lobe mass with focal nodular calcifications,

Cross Pathology

Calcified qstic tumor with gelatinous consistency and areas oF necrosis and hemorrhage.
Tumor has few anapIadc features; regular cells aligned

Micro Pathology

smoothly; spherical ri~lctei with finely ~ r a n u l a chromatin, r calcifica~ions, and increased vascularip with areas of intrarumi~ralIrleeding.
Treatment
Discussion

SurgicaE resection/chemothcrapy with radiation.

LlsuaIIy l w grade hirt occasionally anaplastic. oligndeno drogIiomas resemble astl-ocytomas in most respects but grow mnre slowly and are mare sensitive to chemotherapy: calcification i s nored in 90% o f cases.

.*

OLIGODENDROGLIOMA

IO/CC

A 4Cyear-old maIe with a history of insulin-dependent diabetes meilitus (IDDM) presents w i t h tingling, numbness, b&g, and aching i the lower l e g and feet. n

HPI

The discomfort is paatict~larly promillent at night and is often relieved by walkmg, A hoop over the feet to prevent contact with bedclothes is often helpful. The patient takes i& n . irregmIarly.
L

: A

PE Mild weakness; mild distal sensory loss md loss of position and

vibration sense in both legs; hilateraIlv reducecl ankle and knee jerks.
Labs
Nerve conduction velociries slowed. EMG: features of denervation. EIuvated glycosylated hemoglobin levels indicate poor

g -<

GI

1~Iood sugar control.

Treatment

NSMDs and carbarnaxepine erfective in reducing cliscomfort; good glyccmic control to sIow progression or neuropathy.

Atlas l i n k

EEFTF MC-259

PERIPHERAL NEUROPATHY-DIABETIC

ID/CC

A 35-vear-old m,~lc h o has 1>rc11 w diagnowd wii h rn~lltiple sclerosis vivi ts his phvvician will1 aotnptainr5 01' difficulty swallowing (DYSFI-IAC.IA) a tld naqal rcpgiiation or Innd.
Six mon (11s ago

HPI

sz~l'fer~rl attack or retruhu1lx11an neuritiq: 2 inonthq go h e dcvclopcd spastic weakness of both lower
hp

limbs.

PE Sperch

is n1onc)torlotis, slliri-erl, and high-pitched ( " D C I N . ~ ~ Ut.c7s" ~ ~ z ~ ~ r r n rlrihhles from m o ~ ~ t cannot prc~tr-udt. n r.4); h; Tiis tonpe, which lies on the floor of the mouth and is small and spastic: pala1 a1 mr)veir~rnin al,~eut: jcrk exaggcratvrl; pal it-111 jaw is crr~olionallv labile.

Imaging Treatment

MR. I~rain:multiplc Coca1 wI~i~c-matter I ~ r s . plaq

Gen~1-al rnanagem~n of n i ~ ~ l t i p ~ec l c r o ~ ino spccific rreattnenl t l s; availnhl~.

(11. palsy hilnternl Discu~sTon Tkc common cau~cs pseit(lol~~~lt~ar inch~rle cel.'l>rovaucuI;~~. accicleil t s ilivnlving thr internal capsule, niolor ilt=rlrc>n disease. and n l r ~ l t i psrlel-o~is. l~

PSEUDOBULBAR PALSY

ID/CC

A 40-!car-old male dies ~ h n r t l v afltfr being brnught to the emrrgency room with the Lcrnost severe headache of his life."
His father died of chronic renal failure at the aKe of 45.

HPI

Imaging

CT, Ilcad: hyperdense blood in cistcrns and sulci.

Z '

Gross Pathology

Brain rc-veals staining or inferior surfices of hrainstetn, cerebell11111, ancl cercbral hernispt~~resl l 1 fresh blood duc to congeniwi ta1 herry anellrr:.;tn r.crpture:both kidneys reveat polycysric cllangu~: multiple cycrs ~ p r t ill Iivcr. i
In t hi? cace, hemnrrh age was c a i ~ ~ e d a r~rpti~sedtracranial 11y ill mneurysm in n paticn t with autosornal-dominantpoIycy~tic kidney disease. O t h e r causrs of .itibarachnoid hemorrhage inclurle AV maIformatinn and trauma.

m C

0 G1

,
0

<

Discussion

Atlas Link

q , J T T PG-P3-031

S U B A R A C H N O I D HEMORRHAGE

A ~O-~P~T--OICI Mack male complains of constant bifmntal headache and bIurred vision uf 3 wceks' dnration. He has had mild intermittent frontal headaches for the past 8 months a11d has become irritable; for the past month h e tias hecn extremefy drowsy and ofteti sleeps for :3O hours at a time. Ten months ago, he fell from a moving vehicle and lacerated his scalp.

HPI

PE

Kiateral papiflederna; dilated left pupil: right s m i hernipareplc sis: deep lendon reflexes nn right ~ i d e are hrisk; right-sided Babinski; tlo tneningral rigrl~ present.

Labs
Imaging

LP con trair~rlicatecF cIiw to raised irr I rarranial prexsure.

IT,head: hyperdense crescentic exka-axial fluid colle&ans (early); hypoclense fluid collection w t thick membranes (late). ih
Old blood encaqed in thick adherent h r n w membranes.
OIILY~ rnemhrane composed of panulation and fibrotis tissrie with hcmosiderin; inner membrane shows fibrouq tissue only.

Gross Pathology

Micro Pathology

Treatment

Surgical drai nagc

hematoma.

Disc~ssion S ~ i h d ~ ~ r a ! hcmatoma is a traumatic Iesion charartel-izect by acc~trnulalionofblor)d beween the ilura and arachnoid. It is ca~lsed laceration of the bridging veins and results in by displacement of the brain and possible c e r e b d herniations.
Atlas Link

[Tr;-TlZ PG-P3-032

SUBDURAL HEMATOMA

ID/CC

A .f&year-oId man is referred to a ne~irologist because af progressive anesthesia and weakness of both arms, occipital headaches, and a stiff gait.

HPI
PE

He has n o history of significant trauma

it1

thr past.
Z rn

No motor cleficits: lack of pain and temperature sensation i n both hands and arms {due spit~othalamictract involvernenl) but preserved position and tactile smsation {dorsal coltirnns unit~valved and pmprioceptive sensation sparttcl);t~nitnpairerl pain and temperature sensation below arms: thenar muscles of both hands atrophied; areflexia in both upper limbs; brisk deep tendon reflexes i11 both lower linths.

c
m
0

, , r

<

Labs
Imaging

Normal.
MR/CT, spine: cystic dilatation within c e n d cervical c o d .

ancl Gross %thology Spinal cord shaws central cavitation in longi~udinal. cleftlikc
Fashion.

Micro Pathology
Treatment

Hydromvelia is lined hv epet~clvmal tissue; q7ingomyeIia i s not.

Surgical shunting.

Discuss$on Syringomvelia mav he primary (associated with AmoW-Chjari malformation) or acquired (post-traumatic,pclqr it~flarnmamry, tumor-associated) .

SYRINGOMYELIA

ID/CC

A 7'9-year-oldwhite woman complaiiis or a throbbing, unilateral headache ~ h a l innst severe a r o ~ m d forclleatl aucl temples. is her

HPI

She has hacl rccurrenl bouts of fever over the past year atid also complait~r malaise and muscIe aches. She reports sonle of weight loss and occa'iiar~al vision problems in her right eve. She also reporis pain in her mandible when she i s eating (,W J I
C.L\C7DIC:4TTOK ) .

PE

T7S: f e ~ ' ~ r , nedlllar enlargement of temporal artery with PE: tenderness.

Labs

CRC: nnrnlal 14%C count; mild anemia. Markedly elevated FSR,

l~suallv 100 mm/hot~l: >

Gross 'Pathology

SwoIlen. cordlilie, ~egi~ientallv rloclular temporal artery.

Granulomatous in tlarn ina tory infilt~ilr rnedia and zidven~i uf ria on temporal artery biopsy: rl-agrnentatior~ in te t-nal elastic of lamina nith rnufritzucleatcd ~ ' 1 1 and fibrotic p;itches. ~

Micro Pathology
I

Treatment

Steroids should Ise starr~cl empirically hcfore hiopsv coilfirmation to prevent blindness.
Temporal arteriris i s the inoqt common vasculitis in the United Statcs; it frerll~rntlv coexists with polymyalgia rheumatics and carries a risk of ipsilareral blindness clue 50 thrombosis o f the ophthalmic artery. Diagnosis and treatment nrr based nn cliniraI grounds, since biopsy is positive i n only 60% nf cases.
i ._ PPH-P3-034 :l l

Discussion

Atlas Link

TEMPORAL A R T E R I T I S ( G I A N T CELL A R T E R I T I S )

lD/CC

hri 18-yea1--0lrl man present5 with headache, ataxia, and pmgessive Fosu of visiatl.

HPf

His father died of mrtaslaric bilateral renal cell crarcinoma at a rclativcly yo~mg age. Cerebellar ataxia; n p t a p u s : past-point in^ and inability to m z perform rapid alternating movements ( u ~ \ u ~ m c ~ c ~ o ~ m . s ~C ~ } ; , w 0 liu~rh~scnpir rxam rmeals prescncc of retinal hemangiornaq and , Q morlerale papillede~na(due to increased intmcranii~l pressure). GI

PE

<

Labs
Imaging

UA: normal (hemnluria may qignal rend cell carcinoma).

C,T/MR, head: cerebellar solid/cystic lesion with enhancing mural noduIe. CT, ~ ~ h d o m erenal, hepatic, and pancreatic n:

qs".
Gross Pathology Nemangioblastornas of ccrchcIIum and retina; iutnor occasionally located in medulla ur ccrvical spinal coi-d.

Treatment

Sltrgical removal oi' t unior; photocoap~lation treaunen t of for retinal lesions.

Discussien

I'on TIippcl-Lindi~ri disease is ;I rare autosomd-dorninant 1 3 e ~ t t = o ~ ~ t ~ i dvsplasia. The gene has lxrn linked to the I-af-1 t~tous oncogene on chromvsomc 3 arid has a variable penetrance and dclaycd uxpressir~r~.h e conditiotl is asqociated with renal cell T carcinoma tfia~ ofi e t ~ is mt~ltifocalor bilateral.

VON HIPPEL-LINDAU DISEASE

ID/CC A 45-year+oldHispanic feniale is brought tn the grmecologst for

a n evaluation of a gross difference in the size of her bras& of recell t origin.

HPI

Her medical history is unremarkable. Despite the recent itlcrcase in thc size of her right breast, she does not feel any pain and feels only a sensation o f fullness.

PE

Very large maw with firm, "wooden-log" consistency involring alrnost all o f right breast, tnaking it ~ M T ~ C the size of opposite P I~rena: mobile mass: appears well circumscribed; collateral bluish veins seen on skin along with striae; no peau rlbrange appearance; no nipple retraction, axillary lyrnphnrlenopatl. o r hcpalori~ugaly; opposite Ilreast normal.
US: largc, smooth m~iltilohulatedmass. Largt tumor with ntirnerotis cystic p a c e s nn cut section of strorna, producing recesses and lanpjtudinal openings and causing a leaflike (phyllodes) appearance.
Ahlmclance of normal-looking ducts, acini , and F t r n n m 14th no s i w s of cellular aryia and low mitotic index. Sirnplc excision.
:\ less common I~enigntllrnol- nf'hreast that is also known as fiant fihroadcnema, qstusarcoma phyllodes is a bulky tumor lhal, ill though u s ~ ~ a l i v benign histolngicallv, may recur following excisinrl anrl sr~~netinres l~nrlergoes a l i p a n t degeneration (5% m to 1 0 % ) .It rarely metastasizes to lymph node.;or rlistant sites.

Imaging Gross Pathology

Micro Pathology

Treatment
Discussion

Atlas Link

W a T l PM-P3-036

BREAST-CYSTOSARCOMA

PHYLLODES

ID/CC

,427-year-oldwomanwho isactivebtrainingforamarathon notes a painful lump in the upper outer quadrant of her right breast of' 2 davs' dura-rion.

HPI

She has no history of fever and no known family history of breast cancer.
Rehction af ovalying skin in upper outer quadrant oC right breast; indurated lesion the size of a lemon in same area; axilIarv lymph nodes not palpable.

'PE

Imaging

Manlrno: irregular mass with focal areas ofeggshell calcification.

Yellowish, fat? fluid an aspiration. Excisional biopsy shows localized area of granulation tissue within which are numerous lipid-laden macrophages subjacent to necrotic fat cells.

Gross Pathology
Micro PathoIogy

Treatment
Discussion

Nn ocher active management required.

Fat necrosis of the breast is a unilateral localized process associated with tmuma, breast biopy, reduction mammoplastv, and radiation. It is easily confused with cancer due to induration, fibrosis. clystrnphic calcification, and retraction of overlying skin: the key distinction is the presence of pain.

BREAST-FAT

NECROSIS

ID/CC
HPI

82-vuai.-old woman presenls with painful bilateral breast masses.

The pain i s cyclic in nature a n d increases in her premenstrual phaw, at which time the masses enlararge rapidly and then shrink. Slw F c c l ~ that both hrcasts arc ~lodulaiand is corioerr~ed a t h \lit= may ll;ivt' rancer,
Mildlv tcndcr mils< palpahlr ill tipper anti outer-quadrant of rigFi r ;uirI left I > ~ P ~ so h breasts nodular with multiple thick1 I; 1l ened areas; no change5 in overlying skin or nipple nuted (VS. breast canccr); n o axillary I ~ i n p h a d u ~ ~ o p a to t : ~ ~ d . f h) ~ Acpiration frnm hreast mass rereal? nonl>loodvfluid; mass &sap pears completely after aspiration.
Mamma: nodularitv and henip1 calcificatioiis, n o malipiat~t

PE

Labs

Imaging

tpar~lres.

Gross Pathology

C:ysis nf various 47es rarlging from

trlit-I-o~copic sevrra1 milto limetcrs s~~rraunclcrl drnse fil~rotic by tisst~c; contains clear or l ~ r < ~ ~ fl~ii~l. vri

Micro Pathology
Treatment
Discussion

PI-olifcration of x i n i in lobules ( s c ~ ~ ~ o~nr:riosrs). mr.

Kt=nss~lr.ance symptomatic management. ant1 Fihrncvsrir r l i s e a s ~ f r h e hi.ea$r i q cnnlmon in wr~rnenIwtween o the age? of 3-5a n d 55 and carries at1 it1cre:lsed risk nf ~ I I M S ~ V C brca~r cancer in patients with epithelial hypcrphsia and ahpia. Fi'ihrocvrt ir ci~ar~gc.; result from ho~-njonu mav imhi1l;lnc~~ with e i t h e r an excess of e~tsngen a rleficienr~ prt)gesteronc. or of

BREAST-FIBROCYSTIC

DISEASE

I'D/CC

h .59-vear-ald white female comes to her ramily docLor beca~ise of a presumed "jnfuc tion" in her right breast: she cornplait~s of pain and swelling.
Her history is unremarkable.

HPI
PE

VS: no fever or other systemic sign of infection. PE: right breast warm. rock-hard, and swellen with no areas of fluctuation; onethird of breast erythernatops with shinv o v e r I ~ i ~ g havi tlg skin peau d'orange appearance; painful to touch and pscsstire: several axillary lymph nodes enlarged and firm; qome coalescent.
Rolltine lab work normal.

Labs
Micro %thology

Large ~pheroidal cells and fine stroma infritrared 'by lymphoc y t e w n hreast skin biopsy; lymphalic blood ves~els occluded hy nlnlor cells.
Chemotherapy and radiotherapy, hormone therapy; poor prognosis.

Treatment

Discussion

Inflarnmatorv carcinoma OF thc breast is defined as hreast cancer with angiolymphatic spread: it i characterized by a nlaligs nant course wich earlv and widespread rnelaslases. Perform skin biopsy in patients dia<gnosed wit13 breast infection who do nnt respond promptly to an tihiotic treatment.
PTT'lT Pi-P3-039

Atlas Link

BREAST-INFLAMMATORY

CARCINOMA

ID/CC

X 35-year+Id female r~isheq the emergency room and waits to to sre a doctor hecati~e she i~ c ~ n c e r n e d about a bloody nipple discharge that she discovered this morning.

HPI
PE

She exercises, is very health conscious. and alwavs has ~ a f e sex.

Palparion arnund Iefi nipple reveals blood coming from one of the duct openings and a small, soft lump heneatlr areola; no breast masses o r axillary lymphadenopathy.

Imaging

Marnrno: negative. Ductographv: dilated ducr with intraluminal filling defect. Epi theliaI papillary growth with fibrotic cornponrlits, characieristically located within a lactiferous duct.
N o cellular a i nr anaplastic changes on spccirnen of kfoody wa discharge; only blood intermixed with foamy macrophages and hcnign ductal epi tllclium with fibrova~c~zlar core.
Surgical resection of lactiferous duct. Papilloma of the breast is a benign proliferatiori of ductal epithelial tissue and is, the most common cause of serous/ sang1"neous disc11arg.e.

Gross Path o l o a

Micro Pathology

Treatment

Discussion

BREAST-INTRADUCTAL

PAPILLOMA

A 4E-year-old w o r n presen 1s
breast.

a palpable mass in the left

The patient has been adrnitred to the hospital to obtain an excisional biopsy and for planning Eurther management. The patient's older sister recently died of metastatic breast cancer.
Idefthreast mass on palpation; nipples normally located without evidence of retraction; no evidence of axillary lymphadenopathy or hepatomegaly. Imaging

Mammo: frequently normal or an asymmetric density without deliilabte margins. Firm, white, irl-eplarlv shaped 3-cm mass was removed from each breast. Histologic sections reveal terminal lobulus distend~d interby mediate-sized cells with scant mitotic activity: neoplastic cells infiltrate the stroma with individual neoplastic culls in a single file (NIAN FII.E PAITEKV) that surrol~nds terminal lobule in a the target-appearing fashion.

Gross Pathology

Micro Pathology

Treatment

Modified radical mastectomy with axillarv lymph node sampling; radiotherapy; adjuvant chemotherapy if required. Frequent mammopnphic surveillance is needed owing to the high incidence of a second primary in the same or opposite
'breast.

Discussion

Infiltrating lobular carcinoma is the most common malignancy of the terminal lobule. I t accounts for 10%)tto 13% of all hreast cancers.

Atlas Link

BREAST-LOBULAR

CARCINOMA

ID/CC

h G8-yc:lr-old w h i t t woman

vivits her drrrnalologist because of a long-srandiug itching, ppainIess, scaling, and oozing erythemataus rash over her I-igh~ nipple.

HPI

Mrr- first menstrual period started at age 9. and she has never be~n married or had children; her menopause started at age 56.

PE

Nipple c>nright breast retracted and appears eczematous with redness, some edema. and desquamation; oozing of yellnwish ~xltc1aie:paitlless leF~ axillat-y lymphadenopathy: no hcpatnrnegaly o r lumps in oppositc l~rcast.
Ductal carcirloma wit11 exlension to overlying skin.
Charactrristic cell5 are scattered in he epidermis and are rnucin p c ~ ~ i t i v r have l a r ~ e and nuclei and abundant, pale-staining cytoplasm (P.M:ET's C:ELLS).

Gross Pathology
Micro Pathology

Treatment

MarIified raclical mastectomy with a x i l l a ~ lymph node dissec{inn and tarnosifen tlaerapv.

Discussion

Pagel's carcinoma is a scaly skin leqinn in the areola and nipple arking from ductal adenocarcinoma ~ i t h i n suharucllar excretory ducts and progressing uutrzrard.
I ' . PG-P3-042 "T r

Atlas Link

B R E A S T P A G E T ' S DISEASE

ID/CC
HPI

,I 52-yeat-ald urlrnar.rled white nulliparous female smokcr with early menarche prrrenrg with a painless lump in her right breaft.
Thc patient has a history of atypical hyperplaqia of the right I~reast. I-Icr mother died of breast cancer at 4(j years oTagc.

PE

r 3-rm. Fixed, hard, and nontender mass in upper outer quadZ d rant or right breast: retraction of overlying skin a ~ nipple; no nipple discharge: palpable axillary lymph nodes on right side.
R o u t i n e lab work rlorinal: nnrmal aIkzline phosphamc (no l~orie rne~aqiaws).

Labs

t ,

Imaging

Miammo: spicdated mass with architectr~ral distortion and multiple clustered pleomorphic rnicrocalcifications;skin th ickening and 1 . ~ rilrtini~. 1 CXR: n o evidence of mutastasis.
IIard. in-egltlar whitish mass wilh granules of calcification and focal yellow al-ras of 11ecrosis.Profounrl fibrosis with induration in sZrtlma (DESMOPLXSTI(: RT;A(TIO~').

< z
0 r 0

;
0

<

Gross Pathology

Micro Pathology

FNA large pleomnrphic cells arrxtlged in glaildq, cords, nests, and shcors in d e n ~ e fihrous stroma: mmor cells estrogen and progesterone receptor negative by Ilow q.tomelry. Core biopsy: anaplastic cells wi (11 Iligh mitotic index ronsistcnt with infiltratin3 dtictal arlenocarcinoma, riot otherwisr sprcifirrf .
Surgerv; tarno~ifen(For e~trogcn-rcccp~flr-positive tumors in prernenopa~~sal women); aclj~ivan che~nnthei-apy r with posrihle Ron? marrow tfi~~lxplan ration: radiotlirrapy. tnfiltratin~ ductal hreast carcitiow~ais rhe most common type of breast cancer. Approximately o n e in ninc women in the Unitecl States will develop breast cancer. Risk factors inclirde family h i s tory, early menarche, late menopawe, obesity, exogenous estrogens, atypical hyperplasia of breast, and breast cancer in the opposite breast.
PM-P3-043

Treatment

Discussion

Atlas Link

3 BREAST CARCINOMA

ID/CC

A 25year-old black female visits her family doclor for a painless right breast lump that she discovered on selfexamination; she is othel-wise a.symptomatic.

HFI
PE

Her medical history is unremarkahlc.

Small, encapsulated,well4efmed, rubbery, freely movable 3 c m maTs in right Iower quadrant of right breast; no overlying skin changes; no nipple retraction: no Iyn~phadenopathy:other breast nor-mal.
All rorltin~ work normal. lab

Labs

Imaging

Marnrno: oval low-densiq lesion with smoath margins; "popcorn calcifications" seen with dcgcneration. Solid mass; no areas of necrosis or hemorrhage (centrat myxoid degenerarion i older patienb). n
GlancluIar structures with ductal and stromal proliferation with no cellular atypia.

Gross Pathology

Micro Pathology

Treatment
Discussion

Surgical excision.
Fihroadenoma ir; the most common benign breast hunor in young women; it sometimes enlarges during pregnancy o r normal menstrual cvcles.
PEETE PM-P3-044

Atlas Link

BREAST FIBROADENOMA

ID/CC

'2'-velar-old female presents with an abnormal cervical Pap

smear.

HPI

S11r 1 1 ~ n o history of irregular menstrt ~ a bleeding. postcoital s l bleeding, intcrmcnstrual bleeding, or vaginal discharge. She tlclivcred her first baby at the age or 18 ancl l-lns had multiple sexual partners.

rmaging

Colposcopy reveals a sl~spicinus area Crow which a hiopfy is taken.

Micro Pathology

Biopsv xhcltvq loss nT nnrmal oricn tation uf srlua~nour rplls; atvpical cells qeen with winkled nuclei ar~rlperinuclear haIos involving fulI tl~ickncss squarrlous epitheli~lm; of bascrncnt
membrane intact.

4 3

-<

; Z

rn

r o

a
c3

Treatment
Discussion

Coile biopsy of area with regt~litr follow-up examinations. Ccrvical dvsplmia is a precursor of crrvical squamous ccll carciIzrlrna; ir i s as~nciatecl with infectjon with human papiuomavkus (HPV) types 16, 18, and 31.
PM-P3-045

<

Atlas Link

*
-

CERVICAL CARCINOMA (IN SITV)

-

ID/CC

A :'9-vear-olcl Vietnamese female \;isi~sh e r family doctor beon!we clf pr.r >tracictlnawsea, vaginal bleeding, dyspnea, and hemop-is.

HPI

Her 11 istorp t-ewalc one prcvious nnrnmal gestiitiorl and one spnntaneou~ abortir)tl as twll ar a dilataticli~ x~zd clttrttage 4 months ago for a hydatidiforrn mole.
Vaginal cr;amin:~lir)ri (1 I s p r c l ~IIIII i.c.vealsbluish-red vasa11ar wi l tumor and enlarged uterus; adnexa nnrl ovaries normal.

PE

Tabs
Imaging

Markedly elevated Fcruln arll-l ~ ~ r i n a lhCG levels. -y CXR: mllltiple metaqtatic nodules ( "c:\rut)~3 . k ~ ' set-UKD~WS r '
1 r5!oh-s).

Micro Pathology

Euagger;uerl 11-np a h l a ~ic (cvtntroplir~l~l~stic ~yncytiotr.clh ! and phr>I~lastic) tissric proli r ~ r ' i l l i c ~ will1 et~~lotiit't~~ial ll penetration: celltil;~r ;lt!.pin anrl h e m a t o g e ~ p h a t i 5prcacl. c
C h r m o ~ h ~ i a p fi~llow-11p v: with serial senurn hCG Ievel~. C:I~o~~ioca~-rir~oina i s ;I m;~ligtianr~ e ~ i . t t i ~ )tllnlor hat inay r~al clewlop d ~ ~ r i nnr~nal ng prcgnaurl: i ~ f t ~ l cxacuation o f hvtlatidiform molc. nr aftcr pre~icws sporat:tnrous ahor-tiorls.
U .EIJ PM-P3-046

Treatment

Discussion

Atlas Link

CHORIOCARCINOMA

I DJCC

A 5%year+ld Hispanic multipvida in her 20th week of pregnancy comes to the gynecologist's office complaining of a mas9 in her abdomen.

HPI

She i s pregnant far the fifth time. She has had no prior abortions or Gsections.

PE

ITS: RF normal. PE: n o edema; uterus currecl height for gestational age (at level of umbilicus) ; ill-defined, painless, nonmovable mrmss 5 cm Frrrm midline on rne.iogastriutn; skin riot red or warm: no exudate; no fluctuation; mass seems to disappear on contraction of rectus muscle.
Koutinc lab work on hlond, urine, and stool normal.

Labs

Imaging

CTJMR, nbdotnen: circumscribed mass.

Coarselv trahecula ted tilmor resembling scar t isslle; appears to arise From musmloaponeurotic waU.

Gross Pathology

Micro Pathalogy

Elongated, ~pinclie-shaped cells: tibrnl>lastic process; no evi~Ietlce atypical mitoses otl hiopslj. or
Surgical excision: r d i otherapy.
type nffibromatosis of the anterior abdominal wall in women. desinoid tumor is associated with previous trauma, multiple pregnancies. and Gnrdner's syndrome. 1t frequently recurs after excision.
t'i

Treatment

Discussion

""A

DESMOTD TUMOR

ID/CC

,4 t Gyear-old girl is seen with complaint5 of colicky lower abdominal pain together wi I 11 nausea m d vomiting aqsociated with the onset of menses.

HPI

She achievecf menarche at 14. and h e r initial cvcles were irregular lltit painless (dr~c o anovulatinn). She does nor complain of t any irregularity or excewive Ideeding arid has ncr ~~rinarv complaints o r diarrhea. Abdominal exam normal; p ~ e c o l o f i c cxatn reveals h l ~ ~ d stained pad; pelvic exam not performed d11uto inlac t hymcn; rerial exan1 normal.
Ko~rtine lab parameters normal.

PE

Labs Treatment

Symptomatic relief ui th prostaglandin qmthetase inhibitors w ~ c h as ~nefenamic acid or naproxen sudii~m; inlractahle syr1plorn.s may require suppression of ovldation using combined cstrogen/progcsterorte or progestogens.

Discussion

Ptimary dysmenorrhea is defined as painf1d periods I r which n o organic o r psychological cause can be found; the pail1 is calicky and uqually hegins shortly after or at the oilset of menses. I t is thought to he due to an increasc in the procluction nf pmstxglandins. leading co uterine vasoconstriction m d painl'i~lcontxactiorls. Occurring only during ovulatory cycles, primary dysnienol-rhea is most com~nonly u n d in women h under the age of 20.

DYSMENORRHEA

IDJCC

A 60-?car-old obese, nulliparous white female preqencr with interrnittcnt postmenopausal vagina! bleeding of 3 months' dr ira tion.

HPI

Shc- has a I~istol-y diabetes,hypertension, and infertility with of polycystic ovaries; menopause began at 56 years of age.
Utems
not enlarged on himan~tal paIpation.
I P F I rvithin ~

PE

Labs

CBC: rnilcl anemia. Stool and urine

normal limits.

Imaging
Gross Pathology

US. pelvis: thickening of endometrial qtripe.

Hysceroscopic biopsv performed wi I h rlilatation and curettage; fungating mass iisualized.

Adenacarclnama.

Micro Pathology

Treatment
Discwssian

Radiation ttiet-apv; hvstrr-eeclotny.

Enctometr-ial carcinoma is an estrogen-dependentcancer and i s an important differential d i a ~ g u s i s pn51tn"erlopausafbleeding. of

ENDOMETRIAL CARCINOMA
--

2'i-yeal-+ld white TcrnaEe is aclrnitted to the infertility clinic for evaluatinn or her inability to conceive: she also complai~ls of pain during intercourse ( D W ~ ~ ~ I K 1,h)~a11cl pain during menses EI N ( II\TMEUC)RRI TEA).

HPI

She i s nulligravida. She arlmiu to having rectal pain during menstruation: she also complains or having a11 abundant rnenstn~al period (M ENORKHAGIA OK HYPERMENORRHEA). Bluish spots in posterior fornix on vujnl-il \ p e c z ~ h ~rxam; on m I>imauu;~l exam, fixed, tender bilateral ovarian masses palpable d t irirlg rneu.;truation; induration in pouch o f Doudas with multiple small nodules palpable through posterior f'ilrnix.
Lapdi-uwcql-'): pelvis: o v a t-ies adhere tv htnad liqnment and show reuac tion ancl scarring in addition to endornefriomas, with tlerlse pei-itl~haland p~riovarian adhesions and thickening of uterosacraf ligaments. US, prlvis: rloi~specific ~ u t i c c enlat-gentent of'ovaries.
I\l-own iqh n o d ~ ~ l on titerosacral livments, ovarics, and pouch es of Douglas.
Litparuscopic biopw of affected arms shows ncld~tlrr consist to of r>therwisrnormal-looking. ii~~ictionir~g enrlomet rial glands. Oral contraceptives, progestogens. danazul, G n W . sur-gical rernoval/coag,rl~lazion lesions. of

PE

Imaging

Gross Pathology

Micro Pathology

Treatment

Discussion

Enclomerl-iosis r r f P r ~ endorn~rl-ial to tis~ue that is present outqidr the u t e r 1 1and produccs synpforns that varv with Ioci~lion. ~ 'iido~ne trial implari rs (endninetr~r ]inas or Lhchocolate cysts'') most frrquen tly involve both ovaries.

ID/CC

A 25-year-old Filipina in h e r 20th week of p r e p a n y prcscnts wirh vaginal bleeding but no pain.

HPI

Slle h a u I ~ c c n frcling inordinatdv nauseated and Isar suffered fi 1.i n ~ i n g her- ears. orrr in

PE 15 r n o r l ~ ~ aliyprl-tensinn (BP 150/95). Uterus large for ': te

gestational agc (three finger hreadrh~ above r~mhilicus)lowcr ; e r t r ~ m i e nonpitting edema. 2+

Labs

Markedly incseaqed PhCG. L A : proteinuria but 110 casts seen on micl-osctlpir.exain. F:Ievar I>lnncl tu-ic acid level. Ibl-yotvpe: ed rliploirl KX (completr mole): triploid LKY or XXX (partial
molc).

Imaging

IIS, pelvic;:complex "snowstorm" appearance and no fetal parts

in i~rerir~e c.:l\<ty.

Gross Pathology
Micro Pathology

Characrrr-istic appear-ance o f clusters of p p e s .

Choi.ionic villi marketllv rnlxl-gecl aiicl hyrlmpic rvitlr surrounding cvtn- and svnc7.tintropI~ot~lxstic tisrr~c pmlifcrarion and lack of ;~tlcquatc. vasc~tl;lrsupplv. T)jlatarion and suction curettngc, periorlic rletermination of :(; levels to irlenti l l clevelnpment nI' iiisasive mole or cAIiorinc;~~-cirio~~i:i.

Treatment

'D~SCUSS~OW A gcstat ional nct~plasm that nlav p r e s r ~ ~ r pai~lless as vapnal I>lcrding,pmeclampsia in the first triinrslrr, or hyperemesis
glaridarnm, hydxtirl i fhrrn rnnlp m;ly develop inro malignant

HYDATIDIFORM MOLE

ID/CC

A 53-year-old female complains of increasing fatigue, insomnia, and depression.

had episodes in whicll her face Fur the past (5 months she and neck have become hot and red ( I I ~ FLLSHES). She has been T amenomheic for the past 7 months: prior to hi.;, her meilstrual l~i=,torp nol-mal. was

HPI

PE

Thinning of the skin; him~tism; atrophic vaginal mucosa wit11

drcrensed sccrcticms.

Labs

Increased 24ho11r tlrinary gonadotropins (1,H anrl FSH).

Imaging
Treatment

XR, plain: asteoparosis of thoracoltunhar spine.

Estrogen replacement therapy brncficial.

Discussion

The estrrjgen deficiency srate prodl~cerl mrnopause has I>? shol-t-rati~e h o t flashes). rncdium-range (vaginal atrophy), and ( tong--range (ostropotoris) consequences that can he relieved or prrvrntrd b y eslrogen replacernun 1. C(1i-ninnn~ i d e effects in patierlls taking hormone t-eplacement tl-terapvii-tclitdcirregular I~lerdine;, eight gain, fluid relen~ioti, and eudumrtrial hyperpla~ia. Ncvcrtlic-lrss. pogtmenopausal hlccclir~g l ~ n ~helworkr-d s ~ d u p with i t 1 1 etirlom~~rial 11iopsv to rule out enrlomptrial cancrr.

MENOPAUSE

IQ/CC

A 56-year-old white nullipamus woman is referred for pvalnation of a pelvic mass founcl o n a routi~le plryl;ic;~l.

HPI

S l ~ reports increased Frequency of micturition and irregular e periods until they ceas~rl years ago. She has a history of breast 3
cancer in the distant past.

PE

b r g e cystic mass the qize oTa gr-ap~Frt~it right p e l r i ~ in that can hc fc.1t above the plibis qyrnphysis.
Cjh-125 levels elevated; LFTs normal.

Labs

Imaging

GT/US, pelvis: ?tic

pelvic mass arising out OF right ovary.

Gross Pathology
Micro Pathology

Partly sulid and partly cystic mass.

Papillary S ~ I - E I C ~ I I ~ C St~enplasric-ciliated r a l ~ i m n a r of cells;

psammoma bodies.

Treatment
Discussion

5111-gical sta<+nga11d rrsection: cl~einnrherapv.

common type nf p u c o l o g i c cancer; 111eserous type is most common and is often bilateral. Tt i< often arlvancerl at rlw time of rliagnosis (ompntal ma.ises, liver rnasscs, asrites) .
tnnst

Ovxian catlcer i s the third

?In

OVARIAN CANCER

ID/CC

X 20-vear-olcl Asian female visitq her family cloctor hecause o f chronic, intermittent left lower quadrant pain.

MPI

The pain is n o t accornpiniud 11v d ~ s p a r - e ~ ~mrnsir~zalirl-egunia, laritv, vaginal discharge, atlrtorninal rlistrnrinn, nausea, vomiting. r,l- diarrhea. I t i s not corrrlated ~ i t h menstrual periods. hcr
Left adnexal mass on himanual exam; ucerosacral ligaments 12orrnal; pouch uf DougIas 11ormal:McBurrie\.".;poin r nontender: no cviclrncc of ascitcs.

PE

Labs

Rouiii~e work on 12lond. urine, and stool normal; CA-125 %ah levels not elevated.

Imaging
Micro Pathology

US. pel~is: large (5-crn) simple cyst i left ovary. n

Vaginal smears lor cytohor~nor~al evaluation rcveal excessive estrogenic ctimulation and lack o f pro~eslarinnal erect.

Treatment

Follow-up bv ultrasound (sizahrc pcrcentagr disappear spon taneouslv) ; laparoscopic rcmo\al if prrsistcn t.
Follicular ovarian cvqt i s the most common cause of'owriai~

Discussion

O V A R I A N CYST-FOLLICULAR

X 25-ycar-old woman complains of loss of weight and intense righ~ lower ahrloinir~alpain and nausea that began wllen she ~~erlt <joggingverterdav afternoon.
Intermit tent episodes of similar pain have occ~irred over the pml several davs. She 1ias 1-egi11;lrn ~ n ~ t r u a I i ycles with average flow and no rlysmenorrhea and I ~ a d hcr last period 3 weeks ago.

1s mild hvpot~nsion; ': normal I-IR (IIR 90). PE: right lower
quadrant tenderness; pelvic exan1 rrveals tcnrler, lnohile &cm risht adnexal maqs antericrr lo ulerus.

Labs
Imaging

CBC: normal; pregnancy test ncgltivc.

327,I<t'R: i~-~-egular calcified mass it1 region nC right on-ar.;. US, pelvis: cystic tumor about 8 cm in diameter replaciilg the right
oval-v.

Gross Pathology

Cystic mass replacing the right ovary: thitl, fibrous wall with solid nodule at one aspect containing sebateo~ls material and mattcd hair: tooth strllctm-es also seen.
Malul-e tisqtle e l r r n e i ts representing all three germ cell layers ~ are prerenl, including gkin with aclnrxal strrlchlres, hone, cartilage, teelh. thyroid, F>mnchi.inteqtine, ailcl neural tissue.
Surgical resection curative. Pri~nar>, l>t.nigr~ teralonias nr dermnid cysts nriqit~ate fro111germ cells: tllmors are cystic and contain elements o all three germ f ceIl lavers. Complications of leraromas include ror-siot~, inf'ection. rupture lleaclir~g chemical perttonicis. iz~Cel-tilitv, to secrelion ni' thwoid h n r m o n e leading to hyperthvmidism ( S I ' I ~ L ~ ~ I A c n ; \ ~ 1 1 ) , and carcinoid s?ndromc duc to scroronin secretion; r;~relv, sclltarnolir cell carrinnma milv d~velnp a dermoid ryst. in

Micro Pathology

Treatment

Discussion

Atlas Link

OVARIAN TERATOMA

h 23-yem+ld married woman is seen wirh complaints of inability to conceive after a year 01' unprotected i n1~1-cowseI A - F E R T I L ~ } . (

Her last menstrual pcriod was ? months ago. and since tnenarI che she has only 4 t 5 periods each year ( O L I G O ~ ~ T ; Y O R I U < E ~ ) : o a pregnancy tcst at Ilorr~t. negative. She also complains of waq excessive facial hair. Her father was diabetic.
PE
Paticnt obese: excessive facial hair and malepattern hair distribution o n rest of body (I-ITRSLTSM} h ~ t110 virilizatiun ; pelvic t exam nc)rmal;secondary sexual cl~aractrrist well developed. ics
Elevated LW;decreased FSH atlrl loss of normal p~riodicity (1,I-T > FSI-I. 3: I ratio); serum testosterone and androstenedione clcvated: serum estradiol (total a n d Free) hithin normal limits in early and midfnllicular phases; pattern of secretion abnormal with no preovr~latory midluteal increase;TSH ar~rl or prolactin

Labs

levels normal.
Imaging

P'S, ~~-anm,;vagir~al resolution) : morpj~ologir (high features of polycystic ovaries (multiple peripheral follicles < 8 rnm in c1iarnett.r; prominell t echor!enw stroma) .
Ovaries urilarged with pearly-white capsule and multiple ~ S L S avpr-aging I cm in diamrter wirhin qrl-oma.

Gross Pathology

Micro Pathology

Cysts lined by gmnnulosa and theca cells. t h e lattcr luteiniyed; st 1-nmashows l~yperthecosis anrl lihro~is.

Treatment

Reduce eight: nvulation inductior~ h rlorniphcnc; laparcwir scrrpic ovarian diathermv or laser drilling irl cIrt~g-resistant cases; 1~11-dose billed contraceptive pill if curtrracrptiosi is desircd. corr~
Polvcvstic ovarian srnrlrome (Stein-Leventhal syndrome) is a clinical synclrome ot obesity, h i u t i s m , aad secondary amenorrhea or oligomcnorrhea with infertility due to anovulation, arcompanied hv multiple-follicle cyqts within hot11 o\aries. PCOS patient5 arc at incrAei\serl risk for breast and cndonwtrial rarcinomas (d11e ' otln~ppnsecl t LH stirnu1atiorl).

Discussion

Atlas Link

POLYCYSTIC O V A R I A N SYNDROME

ID/CC

h 17-!car-olrl w l l i t ~ Female visits her family phvaician b e r a ~ ~ s e

she has never had a menstrual period and lacks breast development.
HPI

(PRIMARY ARIFVORR~FEA)

She ha5 a histnry nf low birth weight and l?mphcdema

PE Short stature: In~v-setem-s; webbed neck: cubitl~s a l p ~ s ;low hairv

line: shield-likechest with widely spaced nipples; harsh systolic murmur heard on hack (rlucx 10 cui~rctat of' aorta): hymplasion
tir
t~ailq: s11o1-tFo~u-thmetacarpals: high-arct~ed pakite; absence

of pubic and d a r y hair:srn;~llclitoris ilnd pat galdc.

Labs

iltes~~q; ovaries

no!

High serum and urine FSH and 1.H; no Bars bodies on h~tcc;ll smear. L3rvot);pe:45,XO. US. pelvis: infantile sucak ovaries. Echo: bictispirl aortic valve.
Fikr-<Iic and a ~ r n p h i c t ovaries.

Imaging

Gross Pathology
Micro %thology

Xhwncc of fclllicIes in ot arics; normal twarian stronla replaced hv fibrous streaks.

Growth hormonc and androgens for increase in stature: suhsequuri t rstrogen thcrdpy to protect agai~lst osteoporosis.

Treatment

D ~ S C U S S ~ O R most common karvotype is 45.SO: less commonlv. m a The snicisixi. Tlrrnrr's s~rldrorrie associatrrl with fr-rqurnt skeletal, is renal ( I i n r s ~ ~ h o e kidney), and catdiovasc~rlaranomalies.

P R I M A R Y AMENORRHEA-TURNER'S

SYNDROME

ID/CC

A 39-yeawlcl black female prrsents will1 a several-tnnr~th-long historv of profuse menstruation IF~PFRMENC)RRII~-~} and frequent rnenstruaT periods (POI SMFVORRI I F 4).

HPI

Furr her- qu~qtion alco reir;ds painful periods (DWMEUORRI113) ing a n d irirl-easing urinary frecluency. She has a history of infertility and recurrent spontaneous abortions.
Enlarged, irregtlar uterus .on himanual palpation with swesal rria\';e.;nri po\t erior wall.
CBC/PBS: h\-poch~-omic, microcvtic anemia.

PE

Labs

Imaging
Gross Pathology

US. prlvi%: muItiple heterogeneous masses cliscorting uterus.

Occur in myonlctrium (95% ill-e intramural) and are round,

firm, and well cil-c~nnucribecl.

Micro Pathology In ttrli~cing bunclles o f unilbt-tn, clif'fer~ntiated.elongated mlrmth muscle cells with few mitows a n d no ar~;ipli~ria; maligrtant transfor-marioit rare.
Myomerrornv: hvsi ~rectnrny. TI~P most common tumor of the uterus ancl thc most common tumor in women, utcrinc fibroids are estrogendependent and commonly occrlr after 30 yc.;l~-so f age: ~ h c y lend t o regress aftcr

Treatment Discusdon

menopause.

*
> ,

UTERINE F I B R O I D S

ID/CC
HPI

woman \;isits l ~ epmecnlogist becatire of a foulr smelling, blood-tinged,purulent vaginal discharge.

A 60-!car-old

Shc has nevcr hrcn married and has never been prepant. She is l~vperrcnsivc and take5 oral I~vpoglvcemicagents for diahetes
inellitti%.

PE

VS: RP normal at present. PE: ovcrwight: fleshy, bulky?fzlripting tumor protrtiding fi-nm cervical ns rln vasinal speculum
exam.

Ernaging
Gross Pathology

CT/MR: Iarge, cnmplrx mass arising From

IIIP~I~S.

large. flerhv rumor- or rnynmetrium with areas or ~iecrc~sis and hcl-nclrrhage.

Micro Pathology

Rackground ol ~pindle-shaprd cells w i t h mom than I0 mitoses per high-power field o n hinpqv; many mitorrs h a w a h n a r m d nlr totic spindle. tZrIriamscin, progcstins, cornbinaliol~ chemotherapy.
: I higlllv aggressive malignant rumor or rn~wtletril~m. Ieiomvosarcoma of the utcrrls may arise in a leioin).t)rna or de t l n v t l . I t spreads I y contiguity, liematng-enously, attcl through

Treatment
Discussion

Ivmphatics.
Atlas Link

E T f T T PI-P3-059

UTERINE LEIOMYOSARCOMA

ID/CC

.A GFi=year+ld woman is rekr-red h-intraciahfe n~Imr i growth and pruritus.

F fc11 an ohsttuction i11 ~ h flow of her urinc. She was O c prostitute and wa'i ~re;~ttrl t e ~ ~ STBs. She i a chronic of ti)r s smoker.

HPI

Shc has ~
: I

PE

Cvnecologir exam reveal5 rxcoriation marks over \ ~ ~ l v a : exnphydc grnwth ariqing from leri labia niajora; left inguinal I phadenopnthv. y
C:vr;tc>scnpvreven15 lower l ~ r e ~ h r ~ l e n o c(clue to involvement a~ is Iw vulvar growth). Gross ~x~imination reveal5 firm, rxophyric growt 11.

tabs

Gmss Pathology

Micro Pathology

Micrnscnpir exam or piinch hiopsv sperimeai rcveals invasivc, welldifferen tiaterl s q ~ ~ a m o u s carcinoma with keratinuation. cell
C:onFir~n diagnosis: preoperative rarlintherxpv to shrink tumor rnilqs; radical i ~ ~ l v c c t o m y with lymph node rlis~ection.
\'11h.;~r cancer

Treatment

Discussion

is a cliscasc of oIder women with ;I mean ngc of associared with smoking. and i t 5 reccrlt increa~e in incidencc among younger women is associated wi tll papaw mavirus. Carcinoma in sit11 ( r ~ ~ l w r intraepithelial neoplasin. or
t i 0 veaw. I t
iq

XTN} arid qquarnolrs dysplasia ;11-t considcrrd prcclirsur Icqions.

VULVAR C A R C I N O M A

ID/CC

rZ 75-yuar-olcl white woman visi1.r her p c c o l o ~ s F r a routirxe tn checkup and i s round to have white spots r m her genitalia.

HPI

She c o t n p l a i n ~ oFslight outer vaginal itching bur rlenios any pc>$~n~eraopar~saI hlcr-ding. vaginal rlischar~e, drug intake. or

PE

Hypochromic rnacules 011 1al)ia majorn extcndir~g perincunl to and inner ~liighs patchy distrih~ltinl~ scale formation in IDESQLIAMA~C)N): skin thickened and rough ( F I I T I : R R E . ~ ~ T ~ T T C ) : no regional I~mphadenopathy; atrnpllic vaginitis on vaginal speculum exam.

Micro Pathology

Biopsy reveals hyperkeri-ltosisand Gl~rosis wirh thinning o f sqrialnous cpitlielium; lynphocvtic in fl;lmnlatory infiltra~ion, most prevalent n~rrounding t>toodvesseIs; no chronir inflamnra tory respclnse; 110 r i p s of malignant transfr~rnli~timn.
Biopsy; subsequent
treatment

nz 4 m r)
0

CI

<

Treatment

clcpenrlent on diagnosis.

Discussion

Vulvar leukoplakia is a clinical diagnosis that can be attributed to a varipty nf disorders t h a ~ pradricc while patchcs. Caures all may he h e n i p dirorc1~1-s such as ritiligo, as well as inflatntnatory conditions, prrmaIignant cor~rlii ions ( e . g . , d!rstrnpllies), or s q ~ ~ a t n cell~carcinon~;t. o~ s Always perform n biopsy.

*
La

VULVAR LEUKOPLAKIA

ID/CC

A 73-year-oldwoman i s brought to a gmecoIogist by her daughter, who became aware of a genital ulcer while helping her mother shower.

HPI

Hcr history reveals weight less and dyspnea together with h~pertcnsion and arthritis. Hat-d, nodular, h n m pigmented and ulcerated lesion on upper left labia rninora; no i ~ ~ gnali lynp hadenopathy: scattererl u cxupitant rides on chest auscl~ltation.

PE

Labs
Imaging

CDC/PBS: slight anemia.

CXR: multiple metastatic nodules. Biopsy reveals malignant melanoma cells with lymphocytic
reaction infiltrating into undrrlyi ng dermis; cells stain positive for SlIOO antigen and are negative for mucin.

Micro Pathology

Treatment

Surgery with regional lymph node dissection and adjutxnt

chemotherapy.

Discrrsdon

VlrIvar malignant melanoma is the second most common wEvar malignancy (the first is squamous cell carcinoma); r n e t a m ~ i s and prognosis depeild on the extent orvertical growth.

VULVAR MALIGNANT MELANOMA

ID/CC

A 25-year-old woman presents with amenorrhea of ti weeks' duration and pelvic pain over the past day.
She h= a history of va<@al spotting off and an for the past 2 weekq and has heen using an IUD for the past 3 years. She ha5 no history of vaginal discharge and no urinary symptoms, and her previous mensrrtial I~istory normal. Shr has had multiple is b c ~ ~ tof pelvic inflammatory disease. ts

HPI

PE

VS: BP normal. PE: p~lzllor; abdnrninal distention and decreased bowel sounds: cervical motion tenderness;uterus soft and slightly enlarged on prlvic exam; soft, tender, boggy mass in right adnexa and pouch of Douglas.
CDC: anemia. hCG levels lower than expected for this period of firstation: culdocenteds reveals presence of blood in cul-de-sac.
US, pelvis: no products of conception in uterine cavity; cloughnut-shaped mass in right adnexa; echogenic free fluid in

Labs

Imaging

culilc-sac.
Gross Pathology

Extm~~terine pregnancy, most commonly tubal.

Uterine curettage reveals presence of Arias-Stella reaction in the absence of villi.

Micro Pathology

Treatment

Laparoscopic linear salpingostomv and seamen tal rrsection; methotrexate,

Other risk factors for ectopic pregnancy itlclude previous tubal surgery,tuba1 ligation, endomeiriosis, previous ectopic pregnancy, and ovulation induction.

Discussion

ECTOPIC PREGNANCY

ID/CC

A 58-ycar-old grand mdtipara develops a marked drop in her blood pressure follo~ving uncontrolled bleeding immediately
aftcr delivery.

HPI
PE

She delivcl-cd twins at 35 weeks' gestation with polyhydramnios.

VS: hypotension; tachycardia, PE: anxious; pallr~r.;low central 2.enous pressure; uterus sort and flabby with inclistinct outlitle.

Labs

CBC: anemia; mildly decrpawrl hematocrit. Coag;lllationprofilr

normal.

Gross Pathology
Treatment

I!rerus grossly overdistended and flabby.

Fluid resuwitation; blood transfuvion; uterine massage; maintai ti contraction with an oxytocin infusiorl: ergotarnine fur ~~asoconstriction: folrn~l, if remove retained placenta; cllcck for cervical. vaginal, o -uterine 1acer.alions and uterine ruptrlre; r h ~ p o g a s ~ r artery ligation and/or- hysrel-ectorny if other ic mmsures Tail.
Primnry posrpartltm hemorrhagu (PPH) is defined as loss of 500 mL ar more of I,lood witllin 21 hours or a vaginal dcliverp { 1.000 rriT, af'let- a C-section) or anv atnount cjf h l e e t l i ~ ~ g is that surficient to produce a hernoclylamic compromise; primary causcs i11cl11cle urel-ine aton): rvhiiled placenta. and soft risque in jury. Faclors asrnriatccl with an iiicl-easecl risk of uterine atnny ;~ncl - r l a i n ~ rp l a c c n ~n d udr high multiparity, a maternal age r l i greater than 35 years, delivery after an antepsrrtum hemorrhage, multiple pregnancies, polyhydramnios, a past history of PPR, and coagulation disorders. Sheehan's synclt-eme-a cIinicaI svndromc uf hvpopi t l~itarisinseconcl;~ry isrhelnic pitltiitaty 10 necrosis-is ;I peculiar complicaticlt~of massive po.;tpar!mn

Discussion

POSTPARTUM HEMORRHAGE

ID/CC

A 28-ycnr-01d woman prcsents with wefling of hcr cntire left leg nf 1 cli+v's rlt~rat inn.

HPI

She d~lfvered nnl-ma1 full-term male baby 2 d r ~ y s a ago.

PE
Labs
Imaging

L t 4 leg erythemataus, warm, swollen, and tender.

Rrniliiie tcqn tinrni;~l;normal clotting profile.

US. Doppler: clot in left ftan~ural win. V e r ~ o ~ ~ . ; ~cnnfirmaphy: tui-y "5oTd staii(l;~rrl" hut tiv~aIlv required. not
[V hepnrin ancl monitoring of clotting time ;lucl P ele\ation m of limb: analgesicr; ancl soaks.

Treatment

Discussion

Phlepasia alba dolens (painful white leg) is due to iliofcmoral vcin il~rr~ii~bosis nucurring in Iatp pl-egnanrv find postpartum: it is relared ra compression by a gravid utens and hypercoagda-

POSTPARTUM THROMBOPHLEBITIS

Ill/CC

A 30-year-old woman presents with fhtigue. significant weight loss. and amenorrhea of 2 years' duration.
She had a baby 2 years ago and suffered significant postpartum bleeding. Shc bottle-fed her Rally because she was unable to lactate after delivers.
VS: hypotensinn (BP 8 5 / 6 0 ) . PE: skin tenling: fine wrinkling atnllnrl eyes ancl mouth; loss of ndllary ancl pubic hair.

HPI

'PE

Labs

Decreased levels o f trophic hormones @H, LH, ACTH, TSH, GH, prolactin):decreased levels uf target glai~d hormones IT,<, cortisol, estrogerls). TI.

Imaging

MR. pi tuital-v (usuallv before and after iiljection or gadohniuin DTPA) : abnormal signal in pit~utar.; grand.
Soft, pale, and liernort-hagic pituitary gland in carIy stages; shrunketi. fihnlus, and firm in latrr- stagus.
Mornlone rrplacemcn t: cortisol; levothyroxine (T,) ;
estrogen-11rogcstero~lr

Gmss Pathology

Treatment

replacement.

Discussion

Sheehatn's syndrome is mmt commonIy caused hv postpartum infarction of the pituitary. During pregnancy, the anturior pituitary grow4 to nearly twice i t 5 normal sizc. During delivery, 105s of hloocl or hypovolcmia decreases flow to the pituitary, inducing vasospasrn h a t lead.: to ischemic necrosis of the anterior pituitary. The posterior pi t z ~ i t a r y supplied by arterieq is and i u Illerefore mucll less susceptible to iscllcmia. Lnss of trophic hormoncs leads to atmphy of target organs. Ischerr~ic necrosis tnay also occur in males and in nonpregnanl f ~ ~ n a l e s (rraurna, sickle cell anclnia, dissemi~latecl travascular coagulain tion, \ 5 ~ s c ~ ~accider~ . lar ts)

SHEEHAN'S SYNDROME

ID/CC

ASfi~ear-r~ldwhiteprimi~~daat36weeksofgestationvisit~ her ohqtetrician for thr first time in her pregnancy complaining o l swollen legs and headache.
Her medical h isrory is unremarkable, and her pregnancy had apparrtl~ly developed w i t h no complications until the onset of her m p t e m s .
VS: hypertension (BP 170/110). PE: excessive weight gain (19 kg) ; fundusropir exam does not s11ow changes of llvpcrtensive retinnpathy 3 + pitting pedal edema: 1t pcriorhital edema; fundal heighl appropriatr: retal parts palpable; fetal heart solinds nortna!.

HPI

PE

Labs

CRC:/PBS: complete blood c o u t ~ ~ s coagulation profile and normal. Serum uric acid concentrations raised: mildly elevated AST and ALT; 9l-proteinuria. US, OR: single live Ferns; Le longitridinal: presentation cephalic; i normai biophysical profile; placental infarctions seen.
EndothtliaE cell s~velling with ohliteration of glorneruIar capillarv lumen on renal biopsy. Antihypertensive agen n; delivery of Fcnls and placenta, usually by Gsection.
Preeclampsia occtirs in 5% of all pregnancies; i t is most comnlon during the last trimester of a first prepanq. It is cllancterized by the triad of hypertension, proteinuria, and edema, Pro~ession eclampqia may occur with visual to disttirhances. seizlrres, and coma.

Imaging

Micro Pathology

Treatment

Discussion

IWCC

A 14-year+ld male is admitted to thc hospital complaining of pain and .welling in the Ieft leg.
T11c pain has heen prescnt Tor 2 months hut has become progressively worse over chat period. Thcrc i no llistory of s trxllmx o r infection.
VTS:mild fever. PE: tenderness ancl Fr~riform swelling over left fptnu~.

HPI

PE

Labs

Elevat~d ESR. K a r p t y x : translocation of the long arms of

chromosomes 11 and 22.

Imaging

XR, lcfl femur: lylic lesion in rned~~llary zoilc of rnidshaft with cortical deqtructinn and "onion-skin" appearance. CXR: no evidence of metastatic spread.
Large areas of' bone lvsis 21.5 tumnrs erode cancclfo~ts t~-aheculae nf long hones outward to cortex.
Biop~y I ~ o n c of rcvuals sheets of unifor-tn, small cells rescmbIing Iwr~phocytes; many place5 t urnnr cells surrollnd a central in clrai, area. forming a "pseudorosette.'Tell origin of tumor is
unhown.

Gross Pathology

Micro Pathology

Treatment

"Melt" tumor with radiotherapy: surgical resection: chew otherally: regular fnllow-up for recurrcncu.
Diaphysis of the long hones is the most common site of occurrence of Ewing's sarcoma. Five-year n~rvival 70% for locaIlv is rcscctahlc disrrasc bu t only 50% for those with advancer1
metastasis.

Discussion

1 EWING'S

SARCOMA

ID/CC

A 4 5 - y e a ~ l rwoman visits an orthopedist hecausc of an inability l

to extend her Ealu-th and fifth fingers.

HPI

She has a long-standing history of alcohol. abuse and has been to the emergency room sereral rimes for alcoholic gastritis.

PE

Mild icterus; [lalmar erythema; muscle wa~ting: malnol~rjshtnent; ahdome11rcvcals 2 + asci tic fluid (due to alcoholic liver clamage); fourth and frfrh fingers of right hand reveal flexion contractme wid1 nodular thickening and hick bands of tissue palpable ~rporl drawing examining finger-acrnsq palm.
Infiltration of pnlmal- Eawia with fibrous tissue and suhseq~~cnt contraction riefbrmiry.

Gross Pathology

Micro Pathelogy

Infil~t-ation pretendjnous fascia with rnvofihrohlasts with nf fih~osis pretend inn^^^ bandq. nf

Treatment

Surgcry (release of contractures and aclhesiot~q) rrequen tly ;

lct 1 rs. -cC 1

Discussion

Alsv callecl palinx fibromatosis, Dllpuyrren'~ contracture is

of 11n known etiology hut is associated with alcoholism and manual labor. It is associated with diabetes and anticcln~llsant medications.

CONTRACTU RE

I/[ D(

A 60-year-old woman is hrough t to the ostl~opedic clinic with compfaints of pain in the left hip and inability to bear weight on he lcft leg.
Thee years ago s l ~ r sustained a fracture or the neck OF the femur that was treated with intel-nal fixation. Slie is an alcoholic and has h e c n taking oral steroids for many yeiirs fur a ctironic

HPI

%kin ailment.
PE
1\11 movement? at left hip are restricted by pain: mrahle to hear weight on the limb.

Imaging

XR, left hip: increase in bone density of femoral head and coll a p ~ c l articular s ~ ~ r f a cdynamic hip screw in placr. MR. hip: n e; Inore sen5itivr.

Treatment

Total hip replacement arthropIa-ty significan tlv r ~ d n c e s morhiclirv.

Fracture of the neck o f the femur is the most common cause o f a avilwular necrc3vis of the femoral head: 01 PI. risk lactors iilclude rxccs~ivtr alcohol consumprion, strroid therapy. radiation [herapv, sickle cell anemia, and deep s p a diving (Caisson's disease). Normally blood i s supplied to the head by three routes: rhrntlgh vewels in the ligamen tlim teres, tliro~ngh capsular vessels rcflccted onto tllc femoral ncck, and thnmgh hranchcs of nr~trien1 v ~ s c e l s within the s~xl>sra~~ce hone. W ~ e n fracture of the the occurs. nutrient vessels arc necessarily severcd, capsular vessels are injured to varying degrees, and bIood supply is maintained only through the vessds i the ligamenturn tern. This ic a varin able q ~ ~ a n t i and is of'ten in~ufficien r e d r i n g in avasculruty t, necrosis of t I ~ c fcmoraI head.

Discussion

HIP-AVASCULAR

NECROSIS

OF FEMORAL HEAD

ID/CC

A 12-year-ald obese mde is brought to the horpital with canlplaints of sudden-onset pain of the left hip along with a limp. The pain is felt in the left groin and often radiates to rhe left thigh and k n e ~ .

HPI

PE

Left leg externally rotated and about 2 cm shorter; limited range of abduction and internal rotation; upon flexing left hip, knee is drawn toward left axilla.
XR, left hip (APview) : growth plate widened and irregular.
I-Iead was fixed with pins or screws to prevent further slipping.

Imaghg
Treatment

Discussion

Slipped femoral epiphyses affects youth 10 to 18 years old, with boys mare commonly af'fected than girls; affected children may hc overweight and in some cases have delayed sexual deveIop ment. represent^ a Salter-I-Iarristype I epiphyseal injury. Twenty-five percent are bilateral, of which 15% ta 20% occur sjmul~neously. Avascular necrosis of the femoral hrad and osteoarthritis mav arise a~ complications.

I C "r'

HIP-SLIPPED

CAPITAL FEMORAL EPIPHYSIS

--

- -

ID/CC

A 2.5-vear+ld Ce~nale athlete is hmright to rlie ER after she hurt her right knee.

HPI

She I-iad failen on a hyperextended right knee i h a ~ been unhas stahlc since the fall. Slw recall5 having heard a "popping" ronnd &; t the time o f the i n j ~ ~ r y
Kighr kneeexhihits~ffisionandpositiveanterior"rirawsi~" (!ilia can he p ~ ~ l l fol-ward on fc~mur rd with knee flexed) ; instahiliry o f right kncc.joint (drmons~raled moving uppri- end of hp [ilia fcrrwat-d o n I'cmur with knec flexed only I 0 ro 2 ) d e ~ c c s C 1L A ~ I - I ~ TES-v] ) . U~K

PE

Imaging

MR. knee: indistinct, I~etcrogenous ~ignal expectcd region of in rhc anterior cruciate ligamenr.
Surgical I-ecc~nsirucrion; pla5ter cylinder for 3 weeks followed by hainstring and quatlricepq e x e r c i s ~ ~ .

Treatment

Discussion

The ar~tel-io~cl-l~ciate ligament is torn by a. force dsivit~g e h upper cnd of thc tibia forward relative to the f e n ~ r ~ r hy 1lype1.or extension of the knee; [he posterior mlciate ligament is torn hy a Force driving the upper end of the tibia backward.

KNEE-ANTERIOR

CRUCIATE LIGAMENT INJURY

A 60-year-ld obese female is seen wi th corn plainh of gradually progressing stiffness and pain after use of the right knee.
Thc pain and stiff'ness are accornparlied by welling and deformity of the joint. She also reports difficulty walking aflcl limitatien of rnovenletl t.
Tenderness, pain, and cr-epitus of right k n r e on mnrion: firm m~cllii~g (ca~isrrl underlving bony proliferations) and joint hy cffi~sion; fix~rE rl~fol-mities: bony enla~,geme?lt a. varus ancl angulation, causing limiter1 motion at+joint;harlcls show bony swellings en distal interphalangedjoints (HEBERD~N'S E S ~ . VOU

Labs

Synovial fluid shows n o evidence of iliflammation; nosnlai

viscosir)r and rnucin clot tests: p r o r ~ i nglucose, and compleinent ,

Icvr*lsalso normal; serum rheumatoid fi~ctor raised. no1

Imaging

SR. right

knee {.Q and lateral views): narrowing of,join t space (medial > lateral) ; suhchondral I ~ o n e sclerosis: suhchondral r y t s and osrrophytus.

Cross Pathology

show eburnation of-joint uorface, rt?modelingof joint surface, osteophytes around lateral margins u ~clel-osis. of joint, s bchondral hone cysts, and hur~r
1,atr wages nf the discasr

Micro Pathology

Loss uf articiilar cartilage, bone resorption, and irregular and variable new hone and cartilage furn~:~tioil.
I'ain relid, impruvcrnent o f mohili!y, anrl correction of d c f o r m i ~ ; 1 replacement. join

Treatment

Discussion

O~teoarthritis. degenerative joint rliqeaqe. is characterized hv a t h c degcntra~ion articular cartilage and b y progressive of desu-uctinn ancl rcmrjdeIing OK the-joint struckrrrs. The condition affect<largr weight-hearing joints such as the knees, hips, and lutnhar a11rl cervical vertebrae: otf~eljnints commonly af tec ted are the PIP, DIP, and first carpometacarpal joints. It is more common in women, and irs incidence increases with age, partic~iZarls aft~r.55.

Atlas Links

P?@ OSTEOARTHRITIS

ID/CC A 12-year-old male presents with a swelling above thc right knee
and associated pain.
MPI

There is no history of trauma at the site of pain. There has been no discharse From thr swollen r q i o n and no fever.
Bony-hard, tender, roughly circular swelling ahove right knee (itl dsa femur); averlying skin Temperature normal; mechanical re.;triction of nlovement nf right knee.
Normal ESR elevated serum alkaline phosphatase (may be used xs marker of treatment response).

PE

Labs

Imaging

XR,plain: osteohlztstic bone lesion at distal end of femur with

characteristic "sunburst" or "onion-peelupel-io~teal reaction; pcriosteal elevation hl; rnetaphyseat tumor ( C o t ~ ~ ~ I TRMNGLE) . h"s

Gross Pathology

Firm. whitislt mass with osteoblastic hot2 P sclerosis originating from rnetaphysis adjacent to epiphyseal growth plate and invading through cortex, lifting up prt-instcum.
Bone biopsy shows multinuclcitted @ant cells, anaplastic cells with pleomorphism, and osteoid production with I'oci of sarcomatous degeneration.

Micro Pathology

Treatment

Stlrgical amputation; consider Iinlh salvage: radiotherapv, chemothe~-apv.

Osteoget~ic sarcoma is the mo7t common prirnarv malignant tomnr of hone (excluding mvrlnma and I ~ p h o m a;)it may he oqteohlasuc or osteo1;vzic. Pathologic fiactr~req may occur; pulmonary metastases are fseqlreut. These i~ increased an risk ~ + i t Paget's disease, prior t-adiation, and l-~eredjtarv h re tinohlastoma.

Discussion

Atlas Link

EEF'JT PM-F3-074

OSTEOGENIC SARCOMA

ID/CC A 70-year-old male inlmigrant from Etlgland presents with pain

in the right leg,producing an awkward gait, along with hilateral hearing loss.
HPI

Hc has also noted a progressive increase in his hat size.

PE

Slight bowing of right tibia; normal rectal exam;mixed conduci i w and sensorineural hearing loss confirmed b y audiometry; pliy~ical exam otherwise normal. Markedly elevated alkaline phosphatase; rnijdly elevated serum calciiim and phosphorus; normal serum transaminases; increased urinary excretion of hydroxyprohe.
XR, skull: scattered islands o f hone rysis {OSTEUI~OKQSIS CTRC:IIMSCRJP'~A); mixed thickening (hlastic) and lucency (lytic lesions) of bonc {COTTON-1~0~. E M C E ) . XR, leg (right side): APF bone soft with disorganized trahecufar pattern; bowed tibia.
Expansion of bonc cortex, hlastic h m ~ lesions, and bowing of e long bones (thick ivory bones).

Labs

Imaging

Gross Pathology

Micro 'Pathology

Mul tiplc cement lines with unminer-ali7ed nsteoid; indicative of excessive osteobIastic and osteoclastic actni ty.
Ostcotomirs: calcitonin: diphosphunate; mithramycin.
r;l

Treatment

Discussion

A condition of probable viral etiology, Paget's disease is charactetized by osteoclastic destruction of bone initially with excessive o<!eohlasticrepair, producing bone sclerosis. Mihen extensive, the resulting irlct-eased blood flow leads to high cardiac output congestive heart failure. Other compl isation? are pathologic fracture and osteosarcorna ( I % or patients)

o
VI

PAGET'S DISEASE OF BONE

f I

ID/CC

A 5-year-old male i s hrought to a physician with sudden-nset progressive ~evet-e pain, swelling, and rednew of-the right knee joint.

HPI

HP lias had a high-%grade fever tbr. the part 2 days. A few days ago IIP injured his right leg, and the injury subsequentIy became infected: Ilc is now r ~ n a b to~m o w his right Irg properly. l
L'S: fever. PE: infected ~vound r j g h ~ on leg; right knee red, swollen, and tender: all movements reqt ricted by pain.

PE

Labs

CRC: neutropMic leukocytosis wilh shift to thc left. Elevated ESR: p o v i a l fluid (obtained followingjoint aspiration) opaque and yellowish; joint effusion has WBC cormt > 50,000/ pL; Gram stain reveals gram-positive cocci in clusters: culture vields cnagulasp-pnsitivc Staphy Eococc~dsaureas.

Imaging

XR, right knee: early. soft tissue swelling and joint effusion: late, articular el-nsinns and reactive sclurusis. KLiC. g-;l!Iiurn scan: increased uptake by right kilcejoin I.
Broad-spectrum parented antibiotics initially. then speciFic a n t i biotic5 following culn~re srnsi titit)! rPporrs: if necessary joint may be opened. washed, and closed with a suctiorl drain and immohili7erl ~ i i ~ tsigns of inflammation subside. il Septic arthritis is caused by pyogenic organisms and is more cnnirnorl among children. especially males. S. rrivrruq is the most common cause; other urFrlisrns inclurle streptococci, gonococci, pi~cumococci, and Missuria mrne'n,gitirlr.~. Organisms reach the joint \-La hematogenoils ronte5 (most common; the primary rocus may IIP a pyoderma, throat infectio~~, d n ~ j tlqe, etc.), secondTV : ary to adjacent osteornyelitis, a r via penetrating wot~ntls, the or conditiot~may be iatrogenic.

Treatment

DTscussion

Atlas Links

E37E IMz-041~.IMZ-041B

9 SEPTIC

ARTW RITIS-STAPHY

LOCOCCAL

I D/CC

A 55-yrar-old woman presents with at1 achiig pain in the back of her neck, a feeling of stiffness, and a "grating" sensation upon
rnovmnent.

HPI

She ;11whas a historv OF a vague, ill-rlrfined, anrl ill-localized pain sprcadirlg OVPI- h e r shouldcr region. She cIocs nol contplain of ally noticeahlc motor weakness or sensory loss over any part of t h e hodv and has n o bladder or bowel campIaints.
N~rk slightly kyphotic; posterior cervical muscles tender; neck movements s l i g l ~ ~ rv s ~ c t e d extremes due to pain; audible le at crepitation on movement; diminished suphator and biceps reflex i the left upper limb: no motor or sensory loss n demon~tnhle.
XR, cervical spine (latcral view) : narrowing of intervertebral

PE

Imaging

disk space with formatian of osteophptes at vertebral matgins, especially anteriorly.

Treatment

There i~strotlg te~ldcncy a fur he symptoms of cervical spondylosis to subside spontancouslp. Trea~rnentincludcs analgesics, physiotherapy, and support of the neck by a closely fitting collar of plaster or plastic. Surgical intervention is I-rquiredtbl-patients who are unresponsive to conservative theixpv as well as for rhose with progressive inyelopathy or

Discussion

Degen~ratiw arthritis occurs predonlinantly in [he lowest three cervical jointr. T ~ changes first affect the central interverteP bra1joint5 and later affect the posterior intervertebral (facet) joint?. Osteophytes commonly encroach on the intervertebral Foramina, reducing t h e space rnr transmission or the cervical nriAves. If the rmtricted space i s f i ~ r t h e reduced by the traur matic edcma of h e contained soft tissues. manifestations of nerve pressure are likely to occur. Rarely. I h e spinal cord it.;elC may suffer darnage, prnducing a cer-viral myelopathy.

z
v ,

SPINE-CERVICAL

SPONDYLOSIS

ID/CC

A 32-v~artllclhomeless white male is brought to the emergency room hy all a m h ~ ~ l a n c e fnllowing convulsions that took place on thc street.
The patiell t is dirhevel ed and unshaven in l i i ~ appearance. A history; cannor be obtained llecnllse he is arnne and unable to respntld tr, qilesr icltls.

HPI

PE

Dehydration;jaundice; alcohol on breath; 2cm laceration on occipital area with no hleeding: semicomatose state with response to pain only: p~lpils equal: fine tremor in extl-emiries; p~lmar erythrnla; hepatomegaly.

Labs

CBC/PBS:maerocytic, hypochromic anemia. Elevated direct anrl inclirect bilin~bin; elevated AST and ALT; ASTJALTratio n1' 2 1 ; elevated alkaline phnsphataqe: elevated PT;low serum albumin: h ~ ~ ~ g l v c e m i a .
Fatty liver; tnicrorlodular cirrhosis; market1 gastritis: hl-onchiaI
aspiration.

CKISS Pathology

Micm Pathology

Hrpatacytex clistendcd with fat; hepatocellular necrosis: Mallory bodies (t~yaline); cytoplasmic vacuolkntion o f stem cells in hone marrow; mvofihl-illar necl-o~is: diffuse axnnal degeneration.
Vitami11.:(thiamine xlld folatc) : gIitcose: I-el~ydration; trear acute withdrawal and drliriunl tremens (DT?) with t>enzc>diazepines. Ncoholic DTs usuallv occur 2 to 5 clays aftcr cessation of drinking ant1 are rliaractel-i~ed sei;.ut-~s. P ~ ~ I S ~ agitation, Ilv ~ O I ~ S , disorientation, vist~al and tactile I~allucinations, and autonomic instabilitv. DT PI-ophjaxis cerisists of benzodixepi r-les and resl~xints prevent d a l n a ~ e patient and l 0th~1-S, have 10 to o DTF a rntlrtality rate or 15% if l ~ n t r e a t ~ r l .

Treatment

Discussion

ALCOHOLISM

A 24year-oId white male visits his family doctor complaining of low back pain and stiffness of the spine for almost 1 yeal; incl-easitlg in severity The back stjffnrss is eased h, a hor shower, and worsens afser ! prolonged inactivity. Stooped posture:loss of lumbar Inrdnqis and fixed kyphosis; tenderness over sacroiliac jointq; reducerl chest expansion; pl-ominent ahdomen.
Labs
Imaging
EEcvatrd ESR; negative rheumatoid tests; positive HLA-BZT.

XR, plain: sclerosis and blurring OF margins of sacroiliac joints; afikylosi~ and fusion of vertebrae ( U ~ iSPINE") in long-~ h ~ ~ ~ standing cascs.
Calcification nf intervertebral disks and longitudinal ligaments.
Similar to rhetlmatoid arthritis, but in different location and no rhcumatoicl nodules.

Gross Pathology
Micro Pathology

Treatment Discussion

Phvsical therapy; NS.4IDs; srilfasdazine.

Also called Marie-Striimpell disease and associaterl with HLA-B27, this inflan~matnrv arthritis with event~ial ankylosis of the spine is tvpicallv see11 in young mates; long-standing cases may present with iritis and aortic insufficiency. Ankylosing spondvlitis is also associat~rl with Reiter's svndrome ant1 inflammatory bowel disease.

ANKYLOSING SPONDYLITIS

ED/CC

A5.%~-e;~r~ldmaleisbroughtiilto thcu~nergei~cydepartiiirnt diaphoretic and ashen in appearance.

HP I

HP is ail experieizced pilnt who w7aq o n a crosscountry flight rrom New York to California. I-tis ascent was unrvuntful to ax1 altituclc of 30,000 feet above mean sea level in a n tlnpresst~l-ized airplane. Aftcr I hour at this c r ~ r i s i n ~ ~ u d e alti (and while on stipplemeillai oxygen). h e pilot noticed a gmd11al onset of weaknes and paresthesias of the right arm. These symptoms prc>gresqivelvworsened to involve both arms.
VS: normal. PE: disoriented and confused; normal cardiac and pulmc~n;u-y exam: ne~rvologic exam revealed severe flcxor wcaknes5 at the right elbow and wrist, numbness of the Forearm. and impiirecl fine motor control.

PE

Labs

(;RC/Lytes: nnrrnal. Xkntilation-pel-fi~sionscan ~ h o w c o \ r Q n nlisma~ch. XnGs (at room air): normal.

C:XR: mild increase in i11 tent itial mal- king^. Considel- head CTT scan if mental scalus changes do nnt improve with hyperbaric repressut-iZalion. MRI may he user111 for neurologic dccomprcs siorl icknrss (DCS) 10 locali~e area of i r ~ j ~ ~ r y .
Initial managemurlt with hydration, 100% oxygen delivery by
mask, and transfer t a hyperbaric chamher for compression o therapy. Serious cases of DCS tnav require i nr~~hatioil and prtmor agents. Compression therapy is the definitive treatInunt, anrl Ireattnent shnuld be qtarted as soon as possible.

Imaging

Treatment

Discussion

Decompression sickness,also reCerred to as Caisson's disease, c>cr.urs wl~en person is subjected lo a rapirl I-eduction in ambia ent pressure, resulting in the formation of dissolved I~ul>hles or nitr-ogen withi11body tissues ("he bends"). DCS car1 resull t'rom high-altitude exposure (i.c., a n unpressurized aircraft at > 29,000 I'eet altitude) or firom work in pressill-ized tunnels or caissorls, and is most cornmanly associatect with compressed-air (S(:URA) diving.

DECOMPRESSION SICKNESS

IDJCC

A 52-year-old white female complains to her fanlily rloctor of diffidty climbing steps for tllc past 6 mnnrhs and difficulty washir~aher hair Tor the past 2 weeks.
She stales that she docs not feel tired nr short of breath h l ~ that t her l~gs and arms " j ~ a s will not cooperate." S l ~ c t alro cotnplains aC intermittent fever. Periorhital edema with pl~rplish discoloratio11 ( r r ~ ~ r o n o ~ ~ R%~H) ; bntterfly rash on facc and neck; Raynaud's pher~o~nenon; scaling of skin with redness around knzzckles (C;OTTRC)Y'S I.ESIONT);proximal muscle weakness with tenderness in all four cxmcmi ties.

HPI

PE

Labs

CRC: mild leukoqtosis. Elevated serum CK; elevatetl aldola~e; elwated ESR: antinuclear antibodies (ANAS)presrnt, particularly against tRNA. EMG: spontaneous fthrillatirln.
Muwle edema progressing to tnuscle atrophy and fibrosis.
Musclc biopsy shows lymphocytic infiltration, primarily in a perivasc~tlarFashion hut also betwren muscle fibers on muscle l~inpsv: atrnphv and fibrosis seen.

Gross Pathology
Micro Pathology

Treatment
Discussion

Corticclsteroid~; mrthotrexate; azathioprine.

Dermatomyositis is a n idiopathic disorder primarily affecting c~lcler females: i~ is fi-cquently associated with malignancy (c.g., ovarian carcinoma).
IM"1051A, IM2-051B

Atlas Links

DERMATOMYOSITIS

ED/CC

A 10-year-nId male presents with a persistent low-*grade fever, skin rash, and painful swelling of both knees. Kc also complains of excessive fatiwe and significant anorexia. He has no history of sorc throat. pedal edema/orthopnca, noctl~rnal dyqpnea, or involuntary movemen tq.

HPI:

PE

1 : fever. PE: extensive ~rythematous % rnaculopapular rash; $eneraIizcd Iymphadenopathy; hepatosplcnomcgaly; arthritis or both knees; no suhcu taneous nodule^; no evidence of cardi tis: no Roth's spots on Funrluscopy; no petcchiae over skin or mucnsa; normal cardiov~scular exan.

Labs

CBC/FBS:leukocytosis; normoq-tic. iluri~lochrorr~ic anemia. Elevated ESR; blood cultures sterile; AS0 titers normal; throat
qwnh sterile: rhelunatoid factor negative: Teukncytosis with elevated proteins and markedly low glucose and complement fluid analysis. ECG: normal. levels on syno~ial

Imaging

XR, knees: effusion and soft tissue swelling. Echo: n o vcgctations or valvular disease.

Treatment
Discussion

NShIDs: corricosteroids.
Juvenile rheumatoid arthritis (JRA) r o r commonly affects the n rt kncc joint. Patient5 w i ~ h M s110uld undergo periodic ophthalJ molo9ic exams to carefully monitor for the onset of iridocyclitis, which can Tend to blindness.

>

JUVENILE RHEUMATOID ARTHRITIS

ID/CC

A 42-vcar-old wclman presrn 1s with dysphagia, butterfly rash, arthr;ilgias, myalgias. skin stiffness, mvplling of t h c fingers, proximal muscle weakness, and chronic pain in the finger joints.
She ha5 had these svnlptolns intrrmittentfy over the years. hut thrLvhave worsened over the past year.

HPT

RE

VS: BP normal. PE: er7jthematolls ra4h over face in biu~erflv

distribution: sclmdactyly; telangiectslrias in pt*ril~ngual areas; nonerosive arthritis af wrist and ankle joints; proximal: muscle weakness a~icltendernew: weaknew of twc-k n l ~ ~ s c l en o sensory s: Iow; tlnrmal rendotl reflexes; positive Rapnaud's phenomenon.

Labs

Elevated ESR: diffuse hypergammaglobulinemia; positive rheumatoid factor: higll titer nf'antir~\ic?ea~an tihodips ti peck led p a t t ~ r n sirondy positive test for antibody to RNP ); antigen (most v i c a l finding) ; anti-Smith antibody negative: anti-dsDNA an tihndy negative; normal complernen t levels; clcwted serum CPK luvrls; rnusclr biopsy and EMG suggestive of polymvc~~itis; normal RITs.
(Inrticoqteroidsand NSAlnq

Treatment
Discussion

Mixer[ con nett ive tiswe disorder (MCTTT)) inrlurles cllaracteristics nf one er more traditional connective h ~ diseases at the e same time. thus making it hard to lahcl as one or the other. T h r s t disorder.; incltidr ~ ~ ~ s i e rI~tpzis n i c rsythemat nsus.

A
,

- I

N I X E D CONNECTIVE TISSUE D I S O R D E R

IDJCC

A Fi&year+ld woman v i q i t q her phvsician complaining of severe l o w Ix~ck pait1 after a Call from h e r hccl.

HPI

Onsrt c ~ rnenopa~lse f was a1 48 w a n , Tkr patient i< not receiving hormone replacement therapy; she suffered a CrdIes' fracture lasr yrar rhal is in,il~~nitecl. nil-ected history reveal5 loss of height and a mild h~mchbaek deformity.

PE

Patient thin: kvphosis noted; percussioi~orer dnrsolumhar spine exqi risitelv t e i ~ ~ l erig11[ urisl s h o w n~alliniterl c ~lles' r: C fr-acf~~r-e.
Serum caIcium, phosphates. alkaliuc phosphat:~sc. xnd PTH within tlormal liini ts: densitorneby uscrl tt, qua11 ostropol-oris. tifv

Labs

Imaging

XR, dol-~olumhar spine: low orvertirnl height oT L4 vertebra (duc to collapse and comprcssion frarturr) and k!phosis. n E m (cl~ral-cncrgv x-rav absorptiomemy) : rtduccrl llrltle mass.

Gross Pathology

Thincortex;thmtrabecu~ae,re~cedinnumber.ren~~tin~in i~icreasetlmerl~illar-v spare: obvious rtxctl~re with Ilealing and deformity; collapse of vertebral bodies with kvphoscolioris.
Bonc hinpn: thin but nnrmallv formed corr ex and t r a b c c ~ ~ l a e ; normal calcification; trabcculac very slcndrr; microfracttires ant1 Ir.ac~ure he;~ling may he evidrt~ I.

Micro Pathology

Treatment

Hi~h-protein diet: calcium and vitamin D supplementation; androgens (ii~~;lholic effect oil h o ~ i e estrogens (.ihowri to halt ptngreqsive hone low) : exercise (weight hearing acrs as stirnr~lus boric Lormation): bracing of spinc to prtri7cnxfurthcr to f.mctu~-rs and rlefnr-mil? in a ae~w-ely rssieoporc~tic yline; bisphnc phorlate~ nrav he arlded, especially if liormone I-eplacemer~r r herapy is contraindicatcd.

I m C: 3

Di~cussion O~tcoporosir charactcrizcd hy a reduction of total skeletal is maw due to increased bone resorption (bone Formation is normal) wit11 qealer- 1 ~ of1lraheci~~i~r compac! 'bone: i t ~ thaii r e s ~ ~ lins a prerlispnsitinn to pathnlogir fracture. Cnmmnn t fracuitc sites arc the thoracic and ll~rnhar spine, distal forcarm, and prnxitnal I'Pn11tr.
Atlas Link

0 ~7

<

PGP3-085

OSTEOPOROSIS

ID/CC

A 4+ear+lrE male w i t h a history of Ilvpert~t~sinn develops s11dden abdominal pain ( d ~ w rnege~lreric to thrombosis) far 11ir)l-P severe than prim rpisodeq.
T h e pntien t had a pr~vinus episode of lrem;ltaria with periph13-711 edema [hat was diagnosed as g I o t t i ~ ~ ~ ~ ~ l n t i r pHer has a l~ itis. history or inte t-n~iten 1 (i-ver. n-lalaisc. nwalgia, arthralgia, and I olller vilgur svstrmic symptom^. Liverlo reticularis; ~ ~ ~ l x ~ l t a n e n u . ; or [orearms and finger nodulec pads; pairif 111, ~ l m p a n i c ;~bclomen; ~ ~ r p \ u - i c in lower legs; p .ipr)ts radial and pemneal nerve involvement ( M o h o n . E r RITIS bl L L,I I I ~ L I C X ) l . CRC: mai.ked n r t trophilic le~~kocytosis eosinophilia. ~ with Elrvarrcl ESR; presencc of HRsAg; positive P-ANCA.
:It~gic~, I-rrlal:m111tipIe small aneurpms rt11c1infarcts. Fihrii~oidI P C ~ O I i~.;?ina I inflammatory infil~rnleof lnrdia and adverl ti tia of sinall a n d rnerlillm-si~e vusscls in segmental fa512 inn, will1 t l ~ r ~ n ~ l ancl ipossi hle wnuurysm formation. ms ~

HPI

PE

Labs

Imaging

Gross Pathology

Micro Pathology

Segmental area5 of fibrhoid necrosis 1virI1 nel~tl-ophilic i n f i l ~ r ~ t i o n artena1 wall. of'

Stcruirls a n d other ixn1nunosuppres5ive agents, Polvarte t i t is t~otlow a type 111 hypersensitivity reaction characis tel-izrtl IJV mr~Iti+cm invc l l v r i ~ ~t. n u Renal involvement i s most cnmmon. h u otht-r psesentalions incliade pericar-clitis, myocar~ dial inhrctinn. retii~al ncclirsiu~r, and asthma.

Treatment Discussion

7 PPOLYARTERITIS NODOSA

XD/CC

A 70-year-old female i q seen with complair~ts f inabiJity to comb o her hair, put on her coat, and get up from her chair for the past 6 months. She complains of shoulder and pelvic area stiEfness and pain (er;peciallyduring morning hours), fcvrr, malaise, nnrl fa1 i ~ u e .

HPI

PE

VS: low-grade Cever. PI<:pallor; st if% cleliberate movements:

affec redjoin ta ~ l i o w rest1.i~ ~ mor.cmcIlt: muscle strength t d normal: rernainrlei- of p hysic;~lexam ncsrrnal.

Labs

CBC: nornochromic anemia. ESR markedly elevated: other aclli r-pllasr reactan LF such a.5 fibrinogen and a,-glollulin levels
increased.

Imaging Treatment

XR: normal.

Low-dose oral ~t~rnids: watch for drvclopment of @nt cell arteriti5. which threatens vision in ~ t p one-tl>irclaf patients. In
Polyrrl~.;llgia rl~emnzltica characteri~ed aching and morning is by stiffness in the shoulder and hip girdlc~, the psnximal rxtrettiitie>, the neck, atid the torso; t h r s p e c t r ~ ~of disease incl~tdes m giant cell arteriris. Mean age at onsct i~ 70; wnrnen are affected twice as orten as inerl. A strong association with HIADR4 has I~een h s e r ~ e dSnme casrs recur and some parirn ts becnine o . srernid rlependenr.

Discussion

POCYMYALGIA RHEUMATICA

1D/CC

A ST-vear+ld white Ccmale rninplains of increasing weakness for ~cvcrxl mnn t h ~ especi allv when climbing slain and corn bing her ,
hair.

HPI

She also corriplains of difficulty holding her neck upright. For t h e past few weeks. die has also t~ad difficulty swallowing.

PE

Atrophy of neck, shoulder, and thigh mmscles; motor weaknew in all proximal ml~scle groups; no sensory deficit; deep tendon reflexes reduced.
Markedly elevated s e m CPK levels; antinuclear antibodies (ANAS)demonstrable: elevated serum tr.ansaminasr.s and aldolasc. EMG: mal-krtll?~ increased insertional activity; polyphasic low-arnpli~t~cle motor rmit action pnteilti~ls with al~norinally low I - P C ~tInent. L~~

Labs

Gross Pathology
Micro Pathology

MurcTe eclema progressing to r n u s ~au-nphy and fibrosis. !~

Biopsv Srom rhigh niusclcs reveals inflammatory infiltrate i t i musclc, destr-uct inn of rnufcle fibers, and prrivascular infiltrate r)f'~nor~orluclu:~r rcsidual mnscle lil>ers fn~all. cells:
Highilosr gI~icocorticoirl~: niethotrexate; azathioprine.

Treatment

Discussion

b l y myocitis is frequcn tly seen as a paranenpla5tic m;lnifest;ltion of ovarian, breast, uter-ii~e, in testinn1 malignanq. An ~nssocior atetl r~eoplasm should a l ~ ~ i l he s n u ~ h t . ys

ID/CC

A 40-year-old white feinalc cornplrlirir or paleness and blllish discoloration of the hands, mainly upon exposure to cold, w i t h redness upon rewarming ( ~ R ~ N A II)HENORIE~\'OK)increasing -n's ; pain in thr knees, elbows, and I~ancls over seberal months; aiid recent difficulty swallowing solid food.
Shc alsn has masklike facies ~vi,uirh limited range o f expression. a

HPI

PE

Smooth, shiny, tight skin over face and fingers; erl~tna l hands n ancl reet; palpable wbcutantlous caIcinosir;;pigmentation and relangieclasias of race.
CBC: anernia. IJ\.pergammaglohuline~niil; ti-Scl-70 antibody; an posi the rhetunatoid faczor. PFTs: restrictive lung disease (fi hrosis) .

Labs

Imaging
Gross Pathology

UGI: low o f e~ophagcalr n o ~ i l idilated csophaglls. ~;

PuZrnonarv fibrosis wil h "I~oneycoml~" appearance: swellitlg of esophagra1 ~rrll; n a l a b s o ~ t i o n ~ svi~drome; cnlargeil kidneys with areas of'inl'arczion: myocardiris and pcriuarditis.
Dense fibrosis of collagen tissur of dennis with loss uf appendages and epidermal atrophy: intimal thickening of blond vesscls, pr-imarily in kidney k ~ u t also in GI tract and heart.

Micro Pathology

Treatment

Supportive: cdlcium channel blockers; clmeprazolc: cisapride:

penicillamine.

Discussion

Progressive systemic sclerosis (PSS) may he localized or-systemic o.;i (~isceral involve n-ienl) and rl present with calcit~ 5, r Raylm~rl's phenumen otl. esophageal ilir'nlvement. sclerr~dacrylr: and ielang-iectasia (CREST s m m ~ o m ) .

I XE
m

5 2

Atlas Links

m--lT7 PG-P2-089, PM-P3-089

KDTE MC-226

PROGRESSIVE SYSTEMIC SCLEROSIS (SCLERODERMA)

-

ID/CC
HPE
PE

X 60-vear-old woman presents with swelling and pain in the left

knee after undergoing a major surgical procedure.

She has no history of fever or trauma.
Luft kncc warm and crepitant ltpon movument, which is restricted and painful; positive patellin- tap irldica~eq e f f i ~ ~ i o n . an
Syno\-ialflitjcl fi-orn left knee sllows incr.eased leukocyre cnunt, pt-erlor~iinantly net~trophils; normal uric acid levels; birefringent crystals, Irrcjth free and within lcukocvtes; calcium pyrophosphate crvsials how a weakly positive birefi-ingence and are rhomboid

Labs

in shape.
Imaging
XR. lcfr knce: punctatc and linear calcification in articular cartilage (CHOM)ROCALCISUSIS).
Anti-inflammatory hug,including salirylates, phenylhutxone, iridomethacin. and glucocorticoids, arc ~ffcccivc varying tu rlegl-ees: join t aspiration ma!) help; triamcinoIone intra-alicul a d y f w reqiqtanr cases. r

Treatment

Discussion

The term "pseudogo~~t" rcfers to acute attacks of arthritis associated wit11 r he presence in llie sylov4al fluid of hirefringerent crystals, hot11 Iree and within Ieukncyte~. Pseudogout crystals show a weakly positive birefringence, whereas monosodium mate crystals show a strongly negative birefringence. Grncrally, pseudogour crystals are also sr~~hl>ier. ~ n c ~ r e and dismhoid tha11 umre crystals. Ratliogmphic r~idrr~cr calcinosis (presumably CPPD of crv~tals) cartilage and ocher suucntreq is often present. The in rypical pattern involves calcification in arric~ilm. cartilage, Rbrocartilage (meniscus nf the knee, pubic spmphysis, a n n ~ ~ l u s fihl-cSIN) svt~uvium, fibsou s capsules, tendons, and hursac. Common sites uf involvement include the knee, shnu tclel; wrist. elbow, F~;tr~d, ai~lrle. and

Atlas Link

PM-P3-090

3 '

PSEUDOGOUT

ID/CC

A 30-year-old \vIii~u female c o i n p l a i i ~ s 1I1itt the fingers of Imrh hanrls hecome pale n n exposure to cold.
At tirnc5. tIic pain is also prccipitatetl hv emotional stress. Slre is nor taking any rlrugs and rl.nt=q not ~uffer from any o t I ~ u s tIiaposrd ail~ncnt (e-g.,collagen varcnlar disease: thyroid, itclrt-lial, ur pituitary disraucs). Svrr~pto~n.; r-elicved when she are smiks her hailds in warm nqafer.
Peripheral ~ I I I F P S pal pahle; dipping patient's hands in coId water precipitated pain and resulted in development of digital blanching: rrlr.;lrmii~gT ; ~ ~ I S P ;~~ I O S ~anrl r ~ t h n of finger<. CV F r
Lahorntol-v test.? exc-111de all rauseq or secondary Rapaud's cliscncc ( c o l l a ~ c n vnscular d i ~ e a and ~ s ~ 't>lon~l rlvscrxsias) .

HPI

PE

Labs

Micro Pathology

Arrrrial wall ctlnnges

it1

advanrecl starc nCdisease.

Treatment

Protect hands anrl feer from expowre to cold: clnlg therapy with ii calcit~rn clinr~ncl blocker stlch aq rlikilipir~e a synpatholvtic or agrni s11ch as res~r-piiie gllanethirline. orPrimary EZ;ll;naucI'sphenomenon. or Riyna~rrl's disuase, is a \.dr;uxpasticclisr>rrle~; wllere;ts secu~i(l;~~-v Rayii;ii~d's pl~ennrnenon occilrs as a complitation of svste~nic rli5ease such as scIuroderma, swtcrnic lupus cr)rthtm;~tosus. and rclatc-ct i r n m ~ m o l o disorrlers. ~c M:orr~niare a f f r c ~ e r approxirnatrly i i v r times moi-P than men, l and the age at pl-esen tation i~ 11st1a1ly e t ~ r e e n and 10 vears. h Sfl Thc iingers arc irlr-oTvcd murc frrqltenrlv th;111 r l ~ c lees.

Discussion

RAYNAUD'S DISEASE

ID/CC

.A 'LS-~~ar-old pl-e7el-r with hilatcraI conjrmctivids, painful man t4 swelling of the right knee, bila~eralheel pain. ar~d painless ulcers on 'his penis.
Hr rvas diagnnwd and treaied for nongonocnccal uret hriris 1 week ago.
Bilateral coi~j~inctiviuis atl~eriolwith uveitis; circinate balaaitis; kerato-blmmorrhagieum on palms and soles; arthritis of' right knee and ankle.

HPE

PE

Labs

HU-R2'7 positive;s y n ovial fltiid rcvtals monocvtes with ptlagm neutropliils (W:ITER ,IS) : rheumatoid factor negative; (:EI rlrvatcd ESR.
mtosed

Imaging
Treatment

XR, ri~llr knee and ankle: presence of joint effusion.

NSAIDF are mainslav of thcrapv: treat chlainyclial urethri~is with clu~\~cycline.
K~irer'ssyndromc is an HL11-B2'7-associatedseronrgativc spondvloarthmpathy that is sccn ;rlmosc exclu~ively males arzd in il; associated with conj~lrlctivi~is, urethritis. arthritis, and heeI pain. The conclition has traditionally heell classified as an STD. Imt it ha<alsn occurred follo-rving regional enteritis with

Discussion

R E I T E RFS SYNDROME

A 47-year-~ld white female visitq her Camily doclor cnmplaining of' painfill p well in^ of lhe r i ~ knee. h ~ She has a history of chrunic pail1 along with morning stiffness in the hand joi nts laqting for a1 least 2 hours. Symmetrical deforming arthmpathy (ulnar d u d a tir>ri ; 4) tissue s ~ e l l i n q n d tcnderncss iri PI-oxi~nal terphalangral and a it1 met;~car-pophalangei~l (MCP) joint: warring of smaIl muscles of hand; flexion o MCP join t; hyperexi ension of proximal inlerC phnlnn~ral (PIP)joint and flexion of distal i~~lerphalangeal (DIP) joint (SM'AN-NFI:K ~ E I ; ~ ~ I I T Ye f f ' i t s i ~ ~ ~ ).; nil right knee with overlying skin redness ilild increased t e ~ ~ ~ l > e ~ -subcutanecnlg an~re:

Labs

Incrra~ed ESK: inci-eased protci~l iind white count in thc synovial fluicl: positive rheumatoid factor (IgM or IgA against I@) : pnsitivc antiutrcleai ant iborlies (ANAS); polyclcrnal g m m o p a t h y ; associil aed will1 HLA-DR4.

Imaging

SK,plain: n a r r o ~ i n g joint sparer: rirsion af,jnint ( 4 u ~ ; n o a s ) ; of demintlraljzation an tl l>nnerrosions; ,jnxta-t-artict nsteaporosis. 11ar
Rone ernsion with ankylosis; pericarrlitis, pleuritis; s ~ r b c ~ i ~ n e o u r nodufes with gcarlt~lo~na hrmation.

Gross Pathology

Micro Pathology

Plasma cell inLl tration of qmoiial mernhranes (suvorq~rs) wit11 rlertruction of articular cartilage, lendons, and ligarne~lts by thickened, infl arned synovial &sne (I~.L~NLIS)fibrosis. ;
Physical therap): thermal compresses, splints; NSAIDq; mcthutrcxate; gold: chlornquine: corticustcruids: other im~~~rinc>~nppr-essanrs: surgery.

Treatment

r
0 G l

Discussion

Rhet~matnirl arthritis is thc most common autotmml~ne disease. Cktllar involvement is sfen in 5%) o rases; neurologic involveo T ment of f l ~ carpal t l ~ n n e l t cat1 he a romplication.

<

Atlas Link

RHEUMATOID A R T H R I T I S

A 47-vear-olr! wnlnan visits h r r hcalth carc center complaining of dryness of the mouth (XF.ROSTC)MIA) ancl a gritty sensation i n her eyes with dryness ( X ~ K O I ~ W I - b u h 1 1 . 4 ) .
811~ has

been Iivpcrtrnsive for 20 years and liar su~t'fel-ed from long-standing rheumatoid arthritis, for which she has been treatcd wit11 NShIDs.

Filamentous keratitis with areas of denuded corneal epithelk~rn ( I < ~ ; . R A T O ~ : O ~ ~ ~ ; CS~CLI) on slir-lamp examinat ion with rose T~?T~S Fwngnl dyt staining o cornea; diminished tear famation as C meas~irerl strip of filter paper, with one end o f paper placed rjn inside 1owt.r evelid (Sc:tu~n{c~ ; parotid enlargement; ~17.s-r) excesnive!y clry mouth with ahundan t dell tal raries; charact~ristic w a ~ ~ - n e c k deformitieq of hands and 1111iar deviation (due to Inng-standing cleforming rhcurnatnicl arttll-iris).
Labs
Low saliva flow rates will1 lemon juice stimulation (< 0.5 mk/ min j : h ~ c r g a n ~ r n a g l o ~ ~ u F i npositive antibodies to IgG ~mia: dobdhw (RI r~,tYvl,4TOrn L\C;TO K) and antinuclear antihodies (ANA5). Sialc>griiphy (x-rays fnllowing cani~lrlationancl contrast injection of parotid ducts) : distortion of normal arlborization pattern. Nuc: impaired salivarp filr-icrion.

Imaging

Micro Pathology

Salivary and lacrimal glands show inflam malorv infiltration with T cclls, I3 cells, and plaljrn;~ culls, with predominance olCD4+ T rells; d u d obstruction with glandular acirlar tisst~catrophy with rat5 change.
Artificial tear preparations, increased and frequent oral intake of fluids. careful duntal hygiene, plaque ronrrxi programs. fli~oridc i~pplication.
Sjiigr-en'\ syndrome is dcfined as autoi tnnlune destl-tiction of salivary ancl lacri~nd glands; i r may he primary or associated with orher a ~ ~ l o i r n n ~ ~ r n e cliscases.

Treatment

Discussion

ID/CC

An I Ryar-nId white female prrscnts 14th a rnalar rash that i s uxaccrhatcd by sun r-xposuru ( ~ ~ w ' r o s ~ ~ s r m ~ r~ 1 1 with ;IS w ; ) as } arthl-alg-iaqand jaint stifnessinvoIving h e r ankles, wrists, and knee joints; she also complains o f decreaqecl risual acuity. anorexia. weight loss, malaiqe. and weakness.
She has a history of hcrnatl~ria and n u his~orv rlr-uzintake of prior to thc onsct of synplonls.

HPI

PE

VS: hyperrensio~i(RP If{)/ 14)O). PE: pallor: malar rash; pninfirl restricrion of movcnlent of ~ z ~ i sknrc. and ankle -joints; no t, c~hrious defurmiv; ruhitisl~r-xridates in cytoid I)orli~s nn f~ir~duscopic exarn.

Labs

CBC: Coom hq-positive anemia; neutropenia: thrornbocytopenia. Decreased C 1q, C2, C4; positive antinuclear antibodies (ANAs), anti-native DNA, and antism antibodies: positive I-E cells; falsepr~sitive VDRL clue to an~iphnspholipid antihorlies. LJA: proteinul-ia: RBCFanrl KBC casts.

Imaging

XR, plain: n o et-wive cli;i~~ges. Kchn: n n veg~tatinns seen on V~~IV~S (YS. endncardi tis) .
Serosiris; prriuarrliris: ple~~ritis; splenntnegalv; I~yperkeratotic, ~ r y t h e ~ n a t o plaqil~cs. us Thickening of hasemcnt n~rrnhrane l r l renal biopqy; mesangial c proliferatiori; thickened capillary walls, rreating "wireloop" appearance: clil'f~lse praliCcrative glomerr~lonephri i t n m ~ ~ n e tis: con~pl~x deposition in skin N ~ L I I lyniphocytic ii~filtration; vasculitis with fibr-inoid ~~ecrosis small artel-ips: almoqt any organ of

Gross Pathology

Micro Pathology

x C
U

Treatment

I-Iigh-rtos~ carticnsteroids for prolonged pcriotls; afternative rlr~igs: chloroquine; qclophosphamidf as trealrncnt for lupus nephritis. S s t c m i c Er~pus cryth cmatosr~s (SLF.) i s a type In hypersensitivity reaction.Trnrn~tt~e colnples v a s c ~itis ir the basic pathologic ~l lesion; can he drug-induced {e.g.,hydralazinc, pr-ocainamidu, isonia~idl..

cs r -< ~1

Discussion

Atlas Links

E m PM-P3-095

EEKE IMZ-056

@ I

SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

ID/CC

h $45-year-old white nvale cornplairls of cllronic nasal congeqtion a n d discharge over 111c pixst 5 months.

HPI

Ten ~ ; L J T F ago he d ~ v e l o p e d earache and cough along with an bloodv sputum production, dvspnea, musclc pain, rcd eyes, fever, and night swrat.;. IIr.ied-~~p cl-lls~s inuc~is congestive n a ~ a inucosa wit11 of in l shnllow ulcers and perroration of the nasal septum; sibilant rales disseminated in Iung- Gelds.

PE

Labs

CRC: mild anemia; noder rate leukoqeosis. UA: numerous RBCs; red cell casts and gt-anlllal- castq in 11rine. Positive cytoplasmic antineutrophilic antibodies (GAVCA) in serum.

Imaging

CXR: I~ila~ernl scar~er-ed small nodular den~itje5 with n o hilar

adenopat hy (w. sarcoid) .

Gross Pathology

Gmnl~lorna for-rr~a ill Ilings; vasczilir is and inflammation t ion involving upper reviraiory tract, lungs, peripheral arteries. and

kidneys.
Micro Pathology Focal r~ersotizirag vasculi tis i t~volving small vessels; grat11110rnas ant1 urusccntic glomcl-uloncphritis.
Immunosupprcssivc therapy with stcroids and cyclophos pliamicle.

Treatment

Discussion

Wegerit.~.\gra~irlulomatosis a systuniic autoimmlme vasmHtis is that cnn\ists of necrotizing vasc~~li~is necrot izing gra~lulw a11c1 mils of the lungs and airwavs, as well as a uecroti~ing glomerulius. C;:.Zh'Ch is Fern in lhe n~ajorily patients and serves as a of marker o disease activiry. C
PM-P3-096

Atlas Link

WEGENERrS GRANULOMATOSIS

lD/CC

,4 5X-vear-old mate complains of headache. a n x i ~ y~ h o r t n e s s . of breath. and incrcascd slerpin~ss (SO~W(II.EXI:E) u - l ~ i l ~ ' exp~Tic~lcing acute exacerbation OF COPD. kin

HPI

T h e paticnt is a chronic smoker and aIso complains of recent blurring of vision. He has a histoy of rpisorlic shortness or I~reilth, mucoicl cough, and occasional wl~eezing(consisteilt with prednrninantly bronchitic COPD) h u tin hiqtory nf neurologic ~ defiri t, previnns hypertension. or diabrtes.

PE

VS: ~arlivcarctia: tachxpnea: milcl ystolic hvpertensirrn; no frvcr. PE: anxious and in modcrate respiratrrry distrc~~; llsing accessorv musclcs of rcspiratior~ I 11 p l-nlonger1 expiration: wi mild central cyanosis and pallor; no clubbing: r x h e r n i r i ~ s warm: flapping tremor of hand (.IS ~'EKISIS) ; bounding pulses (dur to high volumc) ; F~induscopy reveals early papilledema: chcst hm-I-el-shapcd i t bilateral s h o ~ ~ canrl occa%onal rales; nu ~ h hi focal neurologic dcficits.

Labs

,ZRG.F:hypoxia, hypercapnia, and partially compensated respiratory acidosis. CBc: pulvcythelnia.

CXR (PA view): inct-eased hronchcxascular markings (dirty liing fields) .

Imaging

Treatment

Lowdose continuaus oxygen inhalation and, if required, mechanical ventilation to reverse acidosis; hrcxtd-spec~rurn an tibiotirs, broi~chodilators(ipratrcrpi~lttl I~roinirle anrl svmpathorn iineticq), and st~roids ~ ~ s e r lCOPD patierits. are in
Dyspnea and Ileadachc arc t11c cardinal svmptoms of hvpercap nix. F-Iypcrcapni~I F c ) prc)d1ices i varie~ of tlrllrologic ~ l y al>tlor~nali sv~nptoms ties: include somnolcnce. blr~r,rcd vision, rcstFcssness, and anxiety that can progress to tremors, asturixirj, drlirium, and coma. Supplemental nxygen s h n ~ h d uscd hc sparingly to avnirl inct-easing Pao,, which rcrnovcs the hypouic respiratory stimulris and leads ro respi rat al-y rlepression.

Discussion

C A R B O N D I O X I D E NARCOSIS