m146 PDF

Neuroimaging
A Teaching File
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Neuroimaging
A Teaching File
Matthew F. Omojola, MD, FRCPC

Professor, Radiology and Neurological Sciences
Division of Neuroradiology
Department of Radiology
University of Nebraska Medical Center
Omaha, Nebraska
Mauricio Castillo, MD, FACR

Professor of Radiology
Chief, Division of Neuroradiology
University of North Carolina
Chapel Hill, North Carolina
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Library of Congress Cataloging-in-Publication Data
Neuroimaging (Omojola)
Neuroimaging : a teaching file / [edited by] Matthew F. Omojola, Mauricio Castillo.
p. ; cm.
Includes bibliographical references and index.
ISBN 978-1-4511-7328-4
I. Omojola, Matthew F., editor. II. Castillo, Mauricio., editor. III. Title.
[DNLM: 1. NeuroimagingCase Reports. 2. Central Nervous SystempathologyCase
Reports. 3. Central Nervous System DiseasesdiagnosisCase Reports. WL 141.5.N47]
RC386.6.D52
616.8'04754dc23
2014023085
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accepted practices. However, the authors, editors, and publisher are not responsible for errors or
omissions or for any consequences from application of the information in this book and make no
warranty, expressed or implied, with respect to the currency, completeness, or accuracy of the
contents of the publication. Application of this information in a particular situation remains the
professional responsibility of the practitioner; the clinical treatments described and recommended
may not be considered absolute and universal recommendations.
The authors, editors, and publisher have exerted every effort to ensure that drug selection and
dosage set forth in this text are in accordance with the current recommendations and practice at the
time of publication. However, in view of ongoing research, changes in government regulations,
and the constant flow of information relating to drug therapy and drug reactions, the reader is urged
to check the package insert for each drug for any change in indications and dosage and for added
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practice.
LWW.com
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For Omoniyi, Olufemi, Ayokunle, Oluwadamilola, Temitope, and Opeyemi; the best children
in the world.
MFO
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C on t r ib utor s
Stephen Bagg, MD Diana F. Florescu, MD

Fellow in Neuroradiology Associate Professor
Division of Neuroradiology Department of Medicine
Department of Radiology Transplant Infectious Diseases Program
University of North Carolina University of Nebraska Medical Center
Chapel Hill, North Carolina Omaha, Nebraska
Mauricio Castillo, MD, FACR J. Gibson, MD

Professor of Radiology Fellow in Neuroradiology
Chief, Division of Neuroradiology Division of Neuroradiology
Department of Radiology Department of Radiology
University of North Carolina University of North Carolina
Chapel Hill, North Carolina Chapel Hill, North Carolina
Alin Chirindel, MD Bryan S. Jeun, MD

Fellow, Russell H Morgan Department of Radiology Fellow in Neuroradiology
and Radiological Science, Department of Diagnostic Radiology
Johns Hopkins Medical Institutions Yale University School of Medicine
Baltimore, Maryland New Haven, Connecticut
Karen Ragland Cole, MD, MPH, MBA Michele H. Johnson, MD

Vice Director, Pediatric Radiology Associate Professor
Long Beach Memorial Hospital Department of Diagnostic Radiology
Clinical Professor Radiology Yale University School of Medicine
Department Neuroradiology New Haven, Connecticut
University of Southern California
Keck School of Medicine Syed A. Jaffar Kazmi, MD
Long Beach, California Assistant Professor
Departments of Pathology and Microbiology
John M. Collins, MD, PhD University of Nebraska Medical Center
Assistant Professor of Radiology Omaha, Nebraska
The University of Chicago Medicine Matthew Kruse, MD
Chicago, Illinois Resident, Russell H Morgan Department of Radiology
and Radiological Science,
Felipe Espinoza, MD Johns Hopkins Medical Institutions
Fellow in Neuroradiology Baltimore, Maryland
Division of Neuroradiology
Department of Radiology Miguel Lemus, MD
University of North Carolina Staff Radiologist
Chapel Hill, North Carolina Department of Radiology
Hospital University of Bellvitge
lHospitalet de Llobregat,
Pierre Fayad, MD, FAHA, FAA
Spain
Reynolds Centennial Professor of Neurology
Department of Neurological Sciences
Director, UNMC-CU Neurology Residency Program Ajay Malhotra, MD
Director, Stroke Center Assistant Professor
The Nebraska Medical Center Department of Diagnostic Radiology
University of Nebraska Medical Center (UNMC) Yale University School of Medicine
Omaha, Nebraska New Haven, Connecticut
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Contributors vii
Robert D. Messina, MD Rathan Subramaniam, MD

Assistant Professor Visiting Associate Professor of Radiology
Department of Diagnostic Radiology Russell H Morgan Department of Radiology and
Yale University School of Medicine Radiological Sciences
New Haven, Connecticut Johns Hopkins University School of Medicine
Adjunct Associate Professor of Radiology
Frank J. Minja, MD Boston University School of Medicine
Assistant Professor Boston, Massachusetts
Department of Diagnostic Radiology
Yale University School of Medicine
New Haven, Connecticut Matthew L. White, MD
Professor of Radiology
Matthew F. Omojola, MD, FRCPC Division Chief Neuroradiology
Professor, Radiology and Neurological Sciences University of Nebraska Medical Center
Division of Neuroradiology Omaha, Nebraska
University of Nebraska Medical Center
Omaha, Nebraska David Yousem, MD
Professor of Radiology and Radiological Science
Russell H Morgan Department of Radiology
Ray Peeples, MD and Radiological Science,
Fellow in Neuroradiology Johns Hopkins Medical Institutions
Division of Neuroradiology Baltimore, Maryland
University of North Carolina
Chapel Hill, North Carolina Rana K. Zabad, MD
Assistant Professor and Director
Sofia Pina, MD Multiple Sclerosis Program
Staff Neuroradiologist Department of Neurological Sciences
Department of Neuroradiology University of Nebraska Medical Center
Hospital Santo Antnio CHP Omaha, Nebraska
Porto, Portugal
Colin S. Poon, MD, PhD, FRCPC Elcin Zan, MD

Staff Radiologist Fellow, Russell H Morgan Department of Radiology
Department of Radiology and Radiological Science,
Orillia Soldiers Memorial Hospital Johns Hopkins Medical Institutions
Orillia, Ontario. Canada Baltimore, Maryland
Adjunct Assistant Professor of Radiology
Yale University School of Medicine
New Haven, Connecticut Vahe M. Zohrabian, MD
Assistant Professor
Department of Diagnostic Radiology
Noelia Silva Priegue, MD Yale University School of Medicine
Staff Neuroradiologist New Haven, Connecticut
Hospital Povisa
Vigo, Spain
William B. Zucconi, DO
Samer Salhab, MD Assistant Professor
Fellow in Neuroradiology Department of Diagnostic Radiology
Department of Diagnostic Radiology Yale University School of Medicine
Yale University School of Medicine New Haven, Connecticut
New Haven, Connecticut
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P ub l i she r s F or e wor d
Teaching files are one of the hallmarks of education in radiology. When there was a need for a compre-
hensive series to provide the resident and practicing radiologist with the kind of personal consultation
with the experts normally found only in the setting of a teaching hospital, Wolters Kluwer was proud to
have created a series that answers this need.
Actual cases have been culled from extensive teaching files in major medical centers. The discus-
sions presented mimic those performed on a daily basis between residents and faculty members in all
radiology departments.
This series is designed so that each case can be studied as an unknown. A consistent format is used to
present each case. A brief clinical history is given, followed by several images. Then, relevant findings,
differential diagnosis, and final diagnosis are given, followed by a discussion of the case. The authors
thereby guide the reader through the interpretation of each case.
Last year we made additional changes to the series. Cases have been randomized to better prepare
the reader for the challenges of the clinical setting. In addition, to answer the growing demand for
electronic content, we have included more cases online, which has left us, in turn, able to offer a more
cost-effective product.
We hope that this series will continue to be a trusted teaching tool for radiologists at any stage of
training or practice and that it will also be a benefit to clinicians whose patients undergo these imaging
studies.
The Publisher
viii
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P RE FAC E
I have always maintained a teaching file from my days as a radiology resident. My teaching materials
always came from these files which are updated from time to time. It is therefore a great privilege to be
asked to lead a group of great contributors to put together some of these cases that we share with you
here in a teaching file format. The presentation follows the new format of the teaching file in the Wolt-
ers Kluwer series, incorporating the relevant imaging findings with the clinical information in a case-
based format. The Question-and-Answer segments, the Reporting Responsibilities, and the What the
Treating Physician Needs to Know segments were the most difficult for me to write. I actually enjoyed
doing them. These sections have turned out to be as informative as the Discussion section. I have
tried to use the ACR communication guide as a guide in composing the Reporting Responsibilities.
However, communication continues to evolve in clinical practice with the emergence of the electronic
medical record. Individual practice should decide what is feasible with regard to the communication
practice. Where possible we have included the specific clinical scenario leading to the diagnosis in
each case so that the path to diagnosis would be clear. While this is a guide, the path to diagnosis may
not necessarily be the same in your own practice. This may change from time to time depending on the
available clinical information. We have collaborated with our colleagues from the clinical services and
pathology where possible in some of the chapters. I believe that we get the best result through interdis-
ciplinary collaborations in the management of our patients.
This title should be useful to residents in radiology and the busy general radiologists who want to
quickly look up a case similar to a problem case they may have. In this regard, we have elected to pres-
ent each case discussion starting with the imaging findings and discussion of the differential diagnosis
to be closely followed by the pathology, clinical presentation, and treatment where necessary so that
our readers would be able to first compare the imaging findings with their cases before diving into the
clinical findings. I also think this book will be useful to the neurology and psychiatry residents doing
elective rotations in neuroimaging. Students doing observership in neuroimaging and medical students
doing neuroscience rotation may also find this book useful. I have been privileged to mentor many of
these residents and students. We have included some cases on normal anatomy. We have also covered
how advanced imaging could be helpful in the differential diagnosis in relevant situations. We have
been as comprehensive as possible in each case considering the constraint of space. I think we have
covered most of the common cases in neuroimaging practice. Several cases with several imaging fea-
tures are discussed in several places resulting in some form of duplication which I consider necessary
in order to do justice to the more common imaging features of such cases.
Matthew F. Omojola
ix
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AC KN OWL E DGME NTS
This book is the work of several contributors. I am therefore grateful to my contributors for making this
book a reality. I would like to thank Dr. Justin Tran for reading through the section on traumatic brain
lesions and for his suggestions. Dr. Najib Murr read through the section on hippocampal malrotation
and made suggestions.
My special thanks to my colleagues in the department of Neurological Sciences at the University of
Nebraska Medical Center for their support throughout my time at the UNMC. They have contributed
immensely to my longevity at this institution. I would like to thank the many Neurology and Psychiatry
residents who took electives with me for the impetus to be a part of this project. I am grateful to all
our colleagues, who took care of the patients presented here, for their contributions to the well-being
of our patients.
I want to remember the late Jonathan Pine, with whom I started out this project at LWW, for his
kindness, support, and dedication to duty while he was under tremendous burden, which I was not
aware off until the very end. Id also like to thank Charley Mitchell, who originated the project but left
LWW before we could actualize it. Charley was very supportive of this project, and I thank him for his
comforting words during the initial break in the project. This project could not have been completed
without the support of many people at LWW, including Amy Dinkel and her group. I learned a lot from
my development editor, Mary Beth Murphy.
Deborah Klein sought and pulled most of the references for this book. Drs. Terri Love and Lisa
Wheelock supplied some images on posterior fossa congenital lesions, some of which I used in this
book. I am also grateful to many other colleagues who contributed images.
I owe a debt of gratitude to my co-editor Dr. Mauricio Castillo, my mentor and a great friend with-
out whom this work could not have been done. Mauricio has served our profession and specialty with
distinction in so many ways. Those of us who have benefited from his knowledge, leadership and
wisdom should be thankful for knowing him. I would like to thank Allan Fox, MD for my foundation
in Neuroradiology. I am indebted to my children, Omoniyi, Olufemi, Ayokunle, Oluwadamilola,
Temitope and Opeyemi; my cheerleaders for their love, constant encouragement and support. I dont
know what I could have done without them! I am grateful to my wife, Jumoke, for her support.
Matthew F. Omojola
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Case
1 CLINICAL HISTORY 65-year-old female with history of breast cancer.
Figure 1-1 Figure 1-2
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2 Case 1
FINDINGS Figure 1-1. Axial post-contrast T1WI through Vasogenic edema results from a breakdown of the normal
the corona radiata/centrum semiovale. There is a ring-enhanc- blood-brain barrier (BBB) and tight junctions in the arte-
ing mass in the left frontoparietal junction with surrounding rial walls. Proteins and fluid that are normally intravascu-
white matter (WM) hypointensity (star). Figures 1-2 and 1-3. lar penetrate into the cerebral parenchyma and extracellular
Axial T2WI and FLAIR through the mass. The mass is sur- space through the disrupted BBB. The extravascular fluid
rounded by confluent WM hyperintensity (star) extending into primarily affects WM and spreads along WM fiber tracts;
the corona radiata/centrum semiovale and the subcortical WM only rarely does it affect GM. The most common causes of
in a finger-like fashion into the gyri sparing the cortical gray vasogenic edema are tumor, infection, trauma, and inflam-
matter (GM) (arrows). The intervening sulci are effaced by mation. Vasogenic edema generally takes time to develop,
mass effect. Figure 1-4. Axial ADC map through the lesion. in contrast to cytotoxic edema that develops rapidly and
There is confluent hyperintensity (star) of the WM around typically involves both WM and GM. Management consists
the mass (star) indicative of increased diffusivity and lack of of treatment of the underlying pathology. Primary manage-
restricted diffusion. ment of the edema may include osmotherapy with the use of
diuretics, hypertonic saline, glycerol, and mannitol to reduce
DIFFERENTIAL DIAGNOSISVasogenic edema, cyto- fluid load by osmotic diuresis, corticosteroids that suppress
toxic edema, encephalitis, primary malignancy, metastasis, the expression of the edema-producing factor vascular endo-
toxoplasmosis, and abscess. thelial growth factor (VEGF) and somehow tighten the BBB
and in the extreme, surgical decompression to relieve the
DIAGNOSIS Vasogenic edema secondary to brain metastasis. compression of the underlying brain.
DISCUSSION Both CT and MRI are capable of demon- Questions for Further Thought
strating the features of vasogenic edema. Typically on CT, 1. Why do brain gliomas result in vasogenic edema?
vasogenic edema manifests as area of confluent hypoden- 2. Can vasogenic edema and cytotoxic edema coexist?
sity relative to normal brain parenchyma. It predominantly
involves the WM and spares the GM even when it involves
Reporting Responsibilities
the deep structures. It insinuates into the subcortical WM
Direct reporting is essential. Vasogenic edema is usually
and produces mass effect with effacement of the sulci.
an indication of an acute or subacute condition requiring
Within the deep structures, it involves the internal, exter-
immediate resolution. Describe the location of abnor-
nal, and extreme capsules without involvement of the basal
mality and any associated important findings such cause
ganglia or the thalami. Mass effect on these structures may
of the edema if present, mass effect, herniation, and
be present. On MRI, vasogenic edema manifests as areas
hydrocephalus. Present reasonable differential diagnosis
of confluent hypointensity on T1WI with corresponding
and recommend further workup or follow-up studies, if
confluent hyperintensity on FLAIR and T2WI (although
deemed necessary.
not to the degree of normal cerebrospinal fluid [CSF]
hyperintensity). The typical finger-like insinuations into
the subcortical WM are better demonstrated on the FLAIR What the Treating Physician Needs to Know
with suppression of the CSF hyperintensity. Additionally, Relevant differential diagnosis and the most likely cause of
vasogenic edema is either iso- or hypointense on DWI the vasogenic edema
with corresponding hyperintensity on ADC maps (in con- What further workup is needed to evaluate the abnormality?
trast to cytotoxic edema that is hyperintense on DWI but Degree of mass effect produced by the edema
hypointense on ADC maprestricted diffusion). It does
not restrict diffusion. Invariably, there is diffuse mass effect Answers
depending on the size of the edema. The underlying cause 1. It is believed that gliomas produce VEGF that is involved
of the edema may be visible as in this case heremetastatic in angiogenesis. Newly formed arteries are not normal
breast cancer. Causes of vasogenic edema include metas- and lack tight junctions between their endothelial cells
tases, primary malignant or high-grade brain tumors such leading to escape of fluids into the extracellular space.
as glioblastoma (GB), sarcomas, and oligodendroglioma Additionally, other factors such as the hypoxia-inducible
(ODG), and inflammatory lesions or infections such as factor and the vascular permeability factor also increase
tumefactive demyelination and abscesses. In some forms of this process.
encephalitis, the vasogenic edema may be the manifestation 2. Yes. Vasogenic and cytotoxic edema can coexist in sev-
of the disease. It could be present around subacute contu- eral situations, such as PRES, venous thrombosis, trauma,
sions and hematomas. hypoxic ischemic encephalopathy, and large infarction.

Case
2 CLINICAL HISTORY 83-year-old male with double vision.
FINDINGS Figure 2-1. Axial T2WI through the sella DISCUSSION The imaging appearance of metastatic dis-
turcica. There is a slightly hyperintense (to normal brain) ease to the skull base or calvarium is highly variable and
mass, involving the sella/suprasellar region and right cav- dependent on the primary histology. T2 signal ranges from
ernous sinus (arrows). Figures 2-2 and 2-3. Axial and sagit- hypointense to hyperintense, texture ranges from solid to
tal post-contrast T1WI, respectively. The mass is essentially cystic, and enhancement may be brisk and homogeneous to
nonenhancing and separate from the laterally displaced patchy, mild or nonexistent. In our patient, a history of pros-
mildly enhancing pituitary gland (arrows). Figure 2-4. Axial tate cancer was known, which greatly helped in the diagnosis.
DWI through the mass. The tumor is hyperintense which Without such information, only a general differential diagno-
aids in the visual detection of the lesion and suggests hyper- sis can be offered, though in any elderly patient with a new
cellularity. mass, even in the absence of a known primary cancer, metasta-
sis will be high on the list of differential diagnoses. Skull base
DIFFERENTIAL DIAGNOSIS Meningioma, macroadenoma, metastasis can occur at any age in both genders. Presentation is
metastasis, chordoma. usually due to the location with visual disturbance the present-
ing symptoms in this case. Treatment depends on the primary
DIAGNOSIS Prostate cancer metastasis. tumor response to chemotherapy or radiation therapy.

4 Case 2
Question for Further Thought What the Treating Physician Needs to Know
1. Which imaging features are helpful in limiting the differ- Lesions of the bony skull, and particularly the skull base and
ential diagnosis for parasellar/clivus lesions? parasellar region, can be hard to identify and hard to diagnose
Regardless of imaging features, with a history of cancer,
Reporting Responsibilities metastatic disease will be very high on the differential
This tumor deserves direct reporting. The first challenge is consideration. Even without a known primary cancer, and
to identify the presence of a lesion, which can be difficult particularly in older patients, metastasis will still be high
for small tumors in the anatomically complex parasellar on the list
region or for small tumors in the wide expanse of the cal- Location, size, and complications or invaded structures
varium. Use all imaging sequences, MRI and CT, to best
advantage. DWIs can be very helpful in this regard as small Answer
lesions may be conspicuously hyperintense on DWI and 1. Taking this case as an example, lack of enhancement argues
quite obvious given the typical lack of signal seen every- against meningioma. Distinct separation from the pituitary
where outside the brain parenchyma. Describe involve- and lack of enhancement render a pituitary macroadenoma
ment of the cavernous sinuses, Meckel caves, impingement less likely. Chordomas are typically very T2 hyperintense,
of the optic apparatus, and any vascular encasement. These and most enhance, unlike the present tumor. Prostate can-
findings will often greatly influence the treatment options cer, on the other hand, is well known to be blastic which
available to a patient. accounts for the T2 hypointensity seen in this case.

Case
3 CLINICAL HISTORY Child presenting with chronic and progressive movement
disorder characterized by tremors and dystonia.
FINDINGS Figure 3-1. Axial T2WI through the basal gan- bilateral symmetrical focal hyperdensity medially within
glia. There is bilateral focal symmetrical hyperintensity in the globus pallidus. All the differentials could produce
the medial globus pallidi (due to gliosis and spongiosis) sur- similar findings without the hypointense rim but with
rounded by thin hypointensity (due to iron and other metal presence of other parenchymal changes.
deposition) (arrows) compatible with the eye of the tiger PKAN is a very rare autosomal recessive disorder and is
sign. Figure 3-2. Corresponding FLAIR image shows similar the most common form of the neurodegeneration with brain
but perhaps less obvious findings. iron accumulation (NBIA) disorders. PKAN is characterized
by a mutation in the PANK 2 gene. There are two subtypes of
DIFFERENTIAL DIAGNOSISMultiple system atrophy, the disease: classic PKAN with onset in childhood and rapid
cortical basal ganglionic degeneration, multiple sclerosis, progression and atypical PKAN with a later onset and slow
neurofibromatosis, human immunodeficiency virus demen- progression. There is no cure and most patients die within
tia, Freidrich ataxia, carbon monoxide poisoning, PKAN. 10years of onset of the disease.
DIAGNOSIS Pantothenate kinase-associated neurodegen- Questions for Further Thought

eration (PKAN). 1. What is the pathophysiology underlying the signal abnor-
malities?
DISCUSSION MRI combined with clinical features 2. What are the main characteristics of NBIA disorders?
indicate the need for gene testing. The eye of the tiger
sign is virtually a pathognomonic finding of PKAN and Reporting Responsibilities
occurs even in the presymptomatic stages, and the central It is a chronic disease. Routine reporting is sufficient.
T2-weighted hyperintensity may accentuate with time or, Recognize the eye of the tiger sign. Evaluate carefully the
in some cases, vanish on serial imaging. Usually there are signal intensity of basal ganglia, taking into account varia-
no other brain parenchyma signal abnormalities except for tions of imaging findings reported in the literature and the
hypointensity of substantia nigra reflecting iron accumu- field strength especially as they refer to the normal degrees
lation in end stages of the disease. CT may show small of iron deposition in those locations.
5

6 Case 3
What the Treating Physician Needs to Know of hypointensity on T2WIs. The process underlying the
The diagnosis is confirmed by genetic testing central T2 hyperintensity is probably related to vacuoliza-
Although the eye of the tiger is pathognomonic, it could be tion and spongiosis.
confused with other entities 2. NBIA refers to a group of neurodegenerative disorders,
Absence of white matter (WM) separates PKAN from its characterized by extrapyramidal signs and mental deterio-
mimics ration associated with iron deposition in the basal ganglia.
MRI may substantiate the clinical suspicion leading the Except for neuroferritinopathy all subtypes are autosomal
way to molecular genetic confirmatory testing recessive. PKAN, neuroaxonal dystrophy, infantile neu-
roaxonal dystrophy, fatty acid hydroxylase-associated
Answers neurodegeneration, aceruloplasminemia, neuroferritinop-
1. PKAN deficiency leads to accumulation of neurotoxic athy, Kufor-Rakeb syndrome, Woodhouse-Sakati syn-
compounds containing cysteine that cause tissue damage drome, and static encephalopathy of childhood with
with iron deposition corresponding to the peripheral area neurodegeneration in adulthood are all related disorders.

Case
4 CLINICAL HISTORY Infant with a large head.

Courtesy of Terri Love MD. Courtesy of Terri Love MD.
extends down to the ventricular CSF, indicating corpus cal-

losal agenesis. There is no septum pellucidum.
DIFFERENTIAL DIAGNOSISChiari III malformation

(CIIIM), occipital cephalocele.
DIAGNOSIS CIIIM.
DISCUSSION Cardinal imaging findings in CIIIM are the

occipital/suboccipital cephalocele which could contain a
combination of dysplastic cerebellum, brainstem, upper cer-
vical spinal cord, and varying amount of CSF and meninges.
The posterior fossa is usually small with a low-lying torcula.
The fourth ventricle may not be visualized, and when it is
visualized it is distorted and tubular. The medulla is kinked,
and the tectum is beaked. Supratentorial findings are similar
to CIIM. Dysgenetic corpus callosum, large massa interme-
dia, cortical malformations, hypoplastic falx, and hydro-
Figure 4-3 cephalus are the predominant findings. The hydrocephalus
Courtesy of Terri Love MD.
may not necessarily be present initially but postoperatively.
Some of these babies may have microcephaly. Bony changes
FINDINGS Figure 4-1. Axial T2WI through foramen mag- could include concave clivus and posterior petrosal surface,
num. Large foramen magnum with a posterior defect (star) posterior occipital and upper cervical spine posterior ele-
for the large occipitocervical predominantly cerebrospinal ment defects, patulous foramen magnum, and lacunar skull.
fluid (CSF)-filled meningocele (arrows). Figure 4-2. Axial Syringohydromyelia, additional low spinal dysraphism, and
FLAIR through the brainstem. There is inferior descent of tethered cord have all been reported in CIIIM. MRI is the
the dilated lateral ventricles compressing and deforming the most appropriate technique for comprehensive evaluation
brainstem which is compressed against the concave clivus of CIIIM. The cephalocele contents and brain parenchymal
and petrosal surface (arrows). Figure 4-3. Axial FLAIR malformations are best delineated by MRI. CT may show
through the lateral ventricles. There is severe dilatation the bony changes optimally. In utero diagnosis is made by
of the lateral ventricles. Anterior interhemispheric fissure US or fetal MRI.
7

8 Case 4
CIIM is the least common of the Chiari malformations What the Treating Physician Needs to Know
and the most lethal with very poor prognosis. There is a male In utero diagnosis is usually made by US or fetal MRI
preponderance. CIIIM usually presents with a large occipital or Is there hydrocephalus? Shunting is the optimal treatment
suboccipital mass due to the occipital-cervical myelomeningo- for that
cele/cephalocele. Hindbrain symptoms and signs could include Are there other associated anomalies?
respiratory difficulty, apnea, swallowing difficulty, paraparesis, CT could suggest the diagnosis, but MRI is best for evalu-
and spasticity. Seizures and developmental delay are common, ation of brain parenchymal malformations. CT may be suf-
but a very small percentage could be asymptomatic. Treatment ficient for postoperative follow-up
consists of surgical removal of the cephalocele with dural repair Entire spine should be evaluated by MRI to exclude addi-
and shunting of the hydrocephalus. Prognosis is very poor indeed. tional spinal anomalies such as syringohydromyelia, addi-
Question for Further Thought tional dysraphism, and tethered cord
1. How do you differentiate CIIIM from occipital cephalocele?
Answer
Reporting Responsibilities 1. Isolated occipital cephalocele does not have the constel-
The occipital cephalocele is usually clinically evident, and lation of posterior fossa and supratentorial anomalies that
routine reporting is sufficient. CIIIM must be differentiated are usually associated with the CIIIM. Occipital cepha-
from isolated occipital cephalocele. Hence categorization locele can also occur in the context of syndromes such
of other associated malformations becomes very important. as in Meckel-Gruber syndrome and Goldenhar-Gorlin
Evaluation of the entire spine is always necessary. syndrome.

Case 5 CLINICAL HISTORY Patient presented with long-standing hypopituitarism and recent
decreased visual acuity.

10 Case 5
best reflects the tumors aggressiveness, being almost cer-

tain when more than 60% of the internal carotid artery
(ICA) is encased by the tumor. There is a high probability
of cavernous sinus involvement when the venous compart-
ment is obliterated or when the lateral limit of the cavern-
ous ICA is surpassed by the tumor. On the other hand,
there is a low probability of cavernous sinus invasion
when less than 25% of the intracavernous ICA is encased
by the tumor.
PAs show various initial presentations, imaging appear-
ances, and biologic behavior. Although most PAs remain
within the sella turcica and have a benign behavior, few
may have an aggressive clinical course with progressive
growth, resistance to medical treatment, invasion of the
adjacent structures (sphenoid bone, cavernous sinus, supra-
sellar region, sphenoid sinus, and nasopharynx), and recur-
rence after surgical resection or even rarely progression to
pituitary carcinoma, which is defined by the World Health
Organization (WHO) as a PA with metastases. The mecha-
nisms underlying this aggressive behavior, despite mostly
benign histopathological features, have not yet been fully
defined. Nevertheless, aside from the atypical morphologic
characteristics and invasive behavior, the WHO classifi-
Figure 5-5 cation includes other features suggestive of an aggressive
behavior, such as elevated mitotic index, a 3% Ki-67 index,
and extensive nuclear staining for p53. Lately, other crite-
FINDINGS Figure 5-1. Sagittal post-contrast T1WI. ria of aggressiveness and malignancy have been described,
There is a large inhomogeneous sellar mass with suprasel- including overexpression of endocan in endothelial cells
lar extension and invasion of the sphenoid sinus (arrow). (related to angiogenesis), a reduction in the E-cadherin/
Figure 5-2. Two months later, a post-contrast axial T1WI catenin expression (in prolactinomas associated with
shows bilateral cerebellar masses (arrows) that are metas- changes in cell-to-cell adhesion and cellular migration), the
tases consistent with metastatic pituitary carcinoma. Figure presence of dysregulated genes (as the overexpression of
5-3. Axial NCCT in a companion case of invasive pituitary the hst gene), and DNA abnormalities (basically accumula-
adenoma (PA) shows destruction of the central bony skull tions of multiple allelic deletions).
base (arrows). Figure 5-4. Sagittal post-contrast T1WI in The mass was confirmed by biopsy, revealing a pituitary
the companion case. There is a large enhancing sellar mass carcinoma. At the 2-month follow-up, the patient had devel-
with invasion of the sphenoid sinus and extension into the oped ataxia and an abnormal gait. Therapy involves surgi-
posterior nasal cavity (arrows). Figure 5-5. Coronal post- cal resection to reduce the tumor and decompress the region
contrast T1WI shows invasion of both cavernous sinuses by prior to adjuvant chemo- and/or radiotherapy.
the sellar mass (arrows).
Questions for Further Thought
DIFFERENTIAL DIAGNOSISMeningioma, metastasis, 1. What gender is more affected and which type of secret-
clival chordoma, chondrosarcoma, plasmacytoma, malignant ing adenomas are pituitary carcinomas most frequently
macroadenoma. related to?
2. Based on imaging, what is the stage or stages that may
DIAGNOSIS Malignant macroadenoma. represent invasion by PA?
DISCUSSION MRI is the best imaging technique to Reporting Responsibilities

evaluate malignant PA and depict the tumoral extensions. Direct reporting is essential in malignant tumors. Describe
MRI shows an isointense mass on T1WI with a variable the extension of the invasive PA and involvement of the cav-
intensity on T2WI and heterogeneous enhancement that ernous sinus, suprasellar structures (such as the optic path-
invades the central skull base. CT is superior in depicting way), and bone. Detect the possibility of craniospinal or
intratumoral calcifications, expansion of the sella, and dis- systemic metastases, which would point to a pituitary carci-
ruption of the sella floor. Invasion of the cavernous sinuses noma with a worst prognosis than invasive macroadenomas.

Case 5 11
What the Treating Physician Needs to Know tumors and are more frequently seen in male patients
Extent of the tumor and invasion of neighboring structures with pituitary macroadenomas that are resistant to
treatment.
2. On the imaging classification, stage III corresponds to a
Answers macroadenoma (>1 cm) with enlargement and invasion
1. Pituitary carcinomas are mostly related to adrenocorti- of the floor of the sella or suprasellar extension, while
cotropic hormone (ACTH) or prolactin (PRL)-secreting stage IV defines destruction of the sella.

Case
6 CLINICAL HISTORY 28-year-old female with stroke.

12

Case 6 13
hyperintensity on FLAIR and T2WI. MRA or CTA may be

useful in evaluating the vascular structures. Both the CTA
and the MRA in this patient showed extensive vasculopathy
both intracranially and extracranially.
Infarcts of the CC are rare. They are more common in
the splenium than the body and anteriorly presumably due
to higher frequency of posterior circulation infarcts com-
pared to ACA territory infarct. They are more common in
the elderly particularly patients with diabetes and hyperten-
sion due to diffuse atherosclerotic changes. Other causes
include thromboembolic phenomenon, atherothrombotic
phenomenon, vasculitis, vasculopathy, Susac syndrome, and
watershed phenomenon. Clinical presentation may include
confusion, neuropsychiatric symptoms, and specific syn-
Figure 6-5 dromes of alien hand syndrome or disconnection syndrome,
paraparesis, and gait disturbances.
Question for Further Thought

FINDINGS Figure 6-1. Axial DWI through the body of
corpus callosum (CC). There is diffuse hyperintensity through 1. Where does the vascular supply to the CC come from?
the CC and cingulum on the right (arrow). Figures 6-2 and 6-3.
ADC maps through the genu (Figure 6-2) and body (Figure Reporting Responsibilities
6-3) of the CC. There is low ADC in the genu and through Acute infarct deserves direct reporting. Chronic infarct on
the entire body of the CC and cingulum on the right (arrows). the other hand requires only routine reporting. There may
Figure 6-4. Axial T2 FLAIR through the body of CC. There be other associated abnormalities such as WM or gray mat-
is diffuse hyperintensity in the CC on the right (arrow). ter (GM) changes elsewhere in the brain particularly in focal
Multifocal bilateral white matter (WM) hyperintensities and infarcts of the CC that may suggest the underlying pathol-
a left parietal old infarct are also present. Figure 6-5. Coronal ogy. These should also be reported so as to be able to reach a
T2WI through the CC about 9 months later. There is atro- comprehensive diagnosis.
phy and hyperintensity of the CC on the right side (arrows).
Associated right parasagittal frontal lobe chronic infarct and What the Treating Physician Needs to Know
widespread bilateral WM changes are present. There is ex Location of infarct; to determine possible cause and arte-
vacuo dilatation of the right lateral ventricle. rial territory involved
Other associated infarcts or lesions
DIFFERENTIAL DIAGNOSIS CC infarct, toxic leukoen- Demyelination may be a difficult entity to distinguish from
cephalopathy (LE), anterior cerebral artery territory infarct. infarct in the CC
DIAGNOSIS CC infarct (right anterior cerebral artery Answer

[ACA] territory infarct). 1. The rarity of CC infarct is due in part to its extensive col-
lateral arterial vascular network deriving from three main
DISCUSSION Depending on the cause, CC infarct could arterial trunks. The cranial (anterior) segment of the CC is
be focal or diffuse. Like all infarcts, it restricts diffusion supplied by the subcallosal and medial callosal arteries from
(hyperintense on DWI and hypointense on ADC maps) the anterior communicating artery. The pericallosal artery
in the acute phase with local mass effect but no contrast which is a branch of the ACA is most often the main vascu-
enhancement. The appearance mimics toxic LE. The infarct lar supply to the body of CC. The caudal aspect (splenium)
here is large with associated parasagittal right frontal lobe of the CC receives supply from the posterior pericallosal
infarct. Focal mass-like and contrast-enhancing infarct could artery, a branch of the posterior cerebral artery. The vessels
resemble a neoplasm or a demyelinating lesion. Presence give penetrating branches that arise almost perpendicular
of other changes such as WM lesions, large arterial territo- to the parent vessels which perhaps protect the CC from
rial infarcts, and the clinical history may help in sorting out thromboembolic processes. The bulk of the supply is from
the differential diagnoses. In the chronic phase, CC infarct the ACA. Isolated ACA territory infarcts are rare account-
shows atrophy, ex vacuo dilation of the ventricle along with ing for a very small percentage of all intracranial infarcts.

Case
7 CLINICAL HISTORY Adult with sensorineural hearing loss.
FINDINGS Figure 7-1. Axial T2WI through the cerebello- DIFFERENTIAL DIAGNOSISCochleitis/labyrinthitis,
pontine angles (CPAs). The normal T2 signal hyperintensity cochlear schwannoma.
of the fluid (perilymph and endolymph) in the middle turn of
the left cochlea has been replaced (arrow) (note the normal DIAGNOSIS Intralabyrinthine (cochlear) schwannoma
cochlea on the right, open arrowhead). Figure 7-2. Axial (ILS).
post-contrast T1WI through same level. There is avid corre-
sponding enhancement (arrow). Figures 7-3 and 7-4. Axial DISCUSSION A high index of suspicion and a high-
T2WI and corresponding post-contrast T1WI through the resolution MRI are required to make the diagnosis of ILS.
internal auditory canal (IAC), respectively, in a companion ILS originates within the cochlea. It is hyperintense on unen-
case. There is avidly contrast-enhancing isointense tumor hanced T1WI and enhances avidly following intravenous
filling the right IAC and protruding into the CPA cistern gadolinium administration. It is sharply circumscribed and
with extension into the cochlea (transmodiolar typearrow hypointense on thin heavily T2W cisternogram. A recent
in Figure 7-3) and vestibule (transmacular typearrowhead classification system is based on the anatomic region(s) of
in Figure 7-3). tumor involvement. The intracochlear type is the most
14

Case 7 15
common, where tumor is confined to the turns in the cochlea. loss, vertigo, tinnitus, and subjective ear fullnessmimicking
The intravestibular type has tumor in the vestibule with many neurootologic diseases.
or without extension into the semicircular canals, whereas
vestibulocochlear ILS involves both compartments of Question for Further Thought
the inner ear, but not the IAC or middle ear space. Tumors 1. What are the management options for these tumors?
that grow between the IAC and inner ear are transmodio- When is surgery indicated?
lar if they grow across the modiolus to or from the cochlea.
A transmacular tumor crosses the macula cribrosa and
involves the vestibule and IAC. The rarest of all is the
Direct reporting is necessary as in any tumor. Anatomic
transotic schwannoma, which spans the IAC and inner and
extent of disease must be described, including the IAC and
middle ear compartments. Our companion case (Figures 7-3
CPA components if present and any corresponding mass
and 7-4) shows tumor filling the IAC and protruding into
effect on the brainstem.
the CPA cistern with both transmodiolar and transmacular
growth patterns.
The main differential for our case is inflammatory inner What the Treating Physician Needs to Know
ear disease or labyrinthitis. MRI findings in inflammatory Anatomic compartments involved (cochlea, vestibule,
disease may be subtle (if present at all) and require careful IAC, middle ear)
attention to the signal properties of the inner ear fluid and any Follow-up recommendations: serial MR surveillance for
corresponding enhancement. The normal T2 fluid hyperinten- schwannoma and for cases of indeterminate enhancement/
sity in acute labyrinthitis is usually normal, and the degree of labyrinthitis
enhancement may be mild but is commonly diffuse within the Follow-up MRI and/or concurrent CT in select cases
labyrinth. In contrast, the pronounced T2 fluid signal replace- (complicated infection, bone involvement, labyrinthitis
ment and the corresponding intense contrast enhancement ossificans)
seen in cases of ILS are quite focal. CT is virtually always
normal in acute cases of labyrinthitis unless caused by gross Answer
middle ear disease (tympanogenic labyrinthitis) or temporal 1. In patients with serviceable hearing, these slow-growing
bone fracture. MRI of hemorrhagic or posttraumatic laby- tumors may be observed with periodic MRI. Clinical
rinthitis may demonstrate inherent T1 hyperintensity of the factors supporting surgical intervention include intrac-
inner ear fluid. If chronic, labyrinthine fibrosis and varying table vestibular symptoms, demonstrable tumor growth,
degrees of ossification (labyrinthitis ossificans) may develop, tumor presence in the IAC/CPA cistern and absence of
in which case CT is useful in confirming the diagnosis. serviceable hearing. Hearing preservation is not possi-
Purely ILSs are rare, benign tumors arising from the ble after surgical removal of an intralabyrinthine tumor.
Schwann cells lining the terminal ends of the cochlear and Poor surgical candidates have been treated with conven-
vestibular nerves. The clinical presentation is nonspecific tional external beam radiation therapy or stereotactic
and variable and may include unilateral sensorineural hearing radiosurgery.

Case
8 CLINICAL HISTORY 49-year-old right-handed female presented with imbalance,
followed by inability to void and limping with the left leg.
16

Case 8 17
FINDINGS Figure 8-1. Sagittal short tau inversion recov- for chickenpox and rabies. NMO remains the most common
ery (STIR) cervical spine MRI demonstrating extensive etiology of LETM. LETM can be present at any age and
continuous spinal cord hyperintensity from C1 to T5. The has no gender preference. It is associated with severe neu-
thoracic spine study (not shown) showed inferior extension rologic deficits which could be acute or insidious in onset.
of the lesion into the thoracic spinal cord. Figure 8-2. Sagittal Cerebrospinal fluid (CSF) analysis including cell count and
post-contrast T1WI at the same time showing extensive protein, an autoimmune screen, and specialized tests such
patchy contrast enhancement throughout the lesion (arrows). as serum and CSF culture, serology, and polymerase chain
Figures 8-3 and 8-4. Sagittal T2WI and post-contrast sagit- reaction assist in identifying the cause of the lesion. As her
tal T1WI, respectively, 8 weeks later showing healing of the spinal cord lesion extended over more than three vertebrae,
extensive lesion with only a small residual spinal cord lesion she was tested for NMO or aquaporin 4 (AQP4) antibody
with contrast enhancement anteriorly at the foramen mag- which was positive.
num level (arrows).
DIFFERENTIAL DIAGNOSIS Transverse myelitis, hyper- 1. What is high-risk syndrome (HRS)?
tensive myelopathy, ependymoma, astrocytoma. 2. What is the natural history of HRS?
DIAGNOSIS Longitudinally extensive transverse myelitis

(LETM).
This is usually an emergent situation and direct reporting is
important. The extent and pattern of T2 hyperintensity along
DISCUSSION LETM refers to extensive inflammation of
with the pattern of contrast enhancement should be reported.
the spinal cord extending over three or more vertebral seg-
Presence of leptomeningeal enhancement may be useful
ments and is located centrally within the spinal cord. MRI
in narrowing the differential. Changes in follow-up study
is the test of choice in evaluation of suspected LETM. MRI
should be documented as this may also help in the differen-
of the spinal cord usually demonstrates T2 hyperintensity
tial diagnosis. Brain MRI should be suggested, and if orbital
with or without contrast enhancement over an extended por-
symptoms are present, orbital MRI may be useful. MRA
tion of the spinal cord usually more than three vertebral lev-
looking for vascular lesions may be necessary.
els. T1WI hypointensity is common in LETM. There may
be associated swelling of the spinal cord in the acute phase.
Intramedullary tumors particularly ependymomas and astro- What the Treating Physician Needs to Know
cytomas may mimic LETM and often appear hyperintense on Location, extent, and number of lesions if more than one
T2WI but tend to be slow growing and often present insidi- What other imaging tests could be useful: MRA, brain
ously and may be associated with widening of the spinal MRI, imaging other levels of the spinal cord
canal. Long segment subacute spinal cord infarcts or hyper- Is lumbar puncture (LP) feasible as this is one of the ways
tensive myelopathy may be difficult to differentiate from to investigate LETM?
LETM. The presence of leptomeningeal enhancement (as Any change on follow-up? This might further narrow the
in neurosarcoidosis or Lyme disease), osseous remodeling list of differential diagnoses
such as spinal canal expansion and scalloping of vertebral
bodies (ependymoma and astrocytoma), and the presence of Answers
brain and orbital abnormalities can further help refine the 1. HRS refers to a monophasic episode of LETM or optic
diagnosis. It is therefore important to image the whole cen- neuritis (ON) with positive NMO or AQP4 antibody.
tral nervous system (CNS) in any case of suspected LETM. 2. Considering the high specificity of NMO-IgG and the
There are several causes for LETM but by far the com- similarity between the HRS presentation and recovery
monest is inflammation/demyelination and can be seen in and NMO groups, HRS can be categorized as an NMO
isolation or in the context of neuromyelitis optica (NMO), spectrum disorder (NMOSD). Small case series of HRS of
autoimmune diseases such as systemic lupus erythematosus ON and TM show that HRS patients are likely to develop
(SLE), Sjgren syndrome, antiphospholipid syndrome, NMO or recurrent attacks of ON or myelitis. Some patients
Behet, neurosarcoidosis, postinfectious myelitis and ADEM, with NMO-IgG LETM may have abnormal visual evoked
paraneoplastic as well as a complication of Lyme disease, potentials. Conversely, some NMO-IgG ON patients may
syphilis, measles, and some vaccinations, particularly those have a small spinal cord T2-hyperintense lesion.

Case
9 CLINICAL HISTORY 77-year-old female presenting with left facial droop and slurred speech and
left hemiplegia of a few hours duration. CT showed vague right middle cerebral artery (MCA) terri-
tory hypodensity. She had rtPA before transfer.

18

Case 9 19
FINDINGS Figure 9-1. Axial ADC map through the level which could all mimic acute infarct. PCE is considered due
of the thalami. There is a right temporoparietal cortical low to contrast leakage through the disrupted bloodbrain bar-
ADC within the territory of the inferior division of the right rier. Thrombolytics are known to cause or exacerbate blood
MCA (arrows) consistent with the history of acute infarct. brain barrier leakage. PCE should be differentiated from
There is very minimal subcortical change. Figure 9-2. Axial other entities that could produce T1 hyperintensity such as
FLAIR through same region. There is right insula ribbon cortical laminar necrosis or hemorrhagic infarct by excluding
effacement (arrows) along with mainly cortical hyperinten- hyperintensity on the non-contrast T1WI. The GRE obtained
sity within the right MCA inferior division territory. There is during this initial MRI and the follow-up CT in this case
minimal subcortical hyperintensity. Figure 9-3. Axial post- excluded significant hemorrhagic conversion, and the CT
contrast T1WI. There is patchy gyriform contrast enhance- shows increasing mass effect as expected.
ment (arrows) through the lesion. Non-contrast T1WI at the
same time (not shown) revealed hypointensity within the Question for Further Thought
infarct. Figure 9-4. Axial NCCT 24 hours later. There is a 1. How is contrast-enhancing infarct differentiated from its
diffuse hypodensity (arrows) through the lesion with local mimics?
mass effect and subtle midline shift to the left. There is no
significant hemorrhage. Reporting Responsibilities
Acute infarcts are emergencies requiring direct reporting to
DIFFERENTIAL DIAGNOSISAcute infarct, subacute the referring physician. Presence of hemorrhage, mass effect
infarct, encephalitis, arteriovenous malformation (AVM). particularly midline shifts, or cerebrospinal fluid (CSF)
pathway obstruction should be reported. The differential of
DIAGNOSIS Acute right MCA territory infarct. contrast-enhancing infarcts should be excluded in the report
if possible. Otherwise a short follow-up evaluation of the
DISCUSSION The hallmark of acute infarct on MRI is an temporal profile of the enhancement and the infarct may be
area of restricted diffusion that is confined to a vascular ter- necessary to be absolutely sure that one is not dealing with
ritory in this case the inferior division of the right MCA. The encephalitis, tumor, or vascular malformation; most of these
restricted diffusion occurs within less than 1 hour following do not restrict diffusion.
the ictus while FLAIR and T2 changes take a couple hours
to develop. DWI more accurately and promptly demonstrates
What the Treating Physician Needs to Know
acute infarct compared with CT. Initially, mass effect may
be slight, developing over the first week because of increas- Location and size
ing vasogenic edema on top of the initial cytotoxic edema Presence of significant mass effect, hemorrhage, hernia-
before stabilizing. Obliteration of the insula ribbon is one tions, or hydrocephalus
of the first recognizable mass effects produced by an MCA The necessity for vascular imaging. Presence of vascular
territory infarct and a popular sign of an acute MCA terri- dissection, stenosis, occlusion, or irregularity as seen in
tory infarct on either CT or MRI. Over the next few weeks, vasculitis or vasculopathy may dictate the management
the mass effect recedes. Three types of contrast enhancement approach. CTA or MRA of the head and neck may be
pattern have been described in the acute infarct. Intravascular appropriate and is often carried out as part of a compre-
contrast enhancement supposedly due to slow flow occurs in hensive imaging protocol of the stroke patient
about 75% of acute cortical infarcts. Deep infarcts tend not
to show intravascular enhancement. Meningeal enhancement Answer
occurs in about a third of the patients presumably due to 1. The mimics include neoplasm, vascular malformation,
meningeal inflammation/congestion over the area of infarct. and encephalitis. The history is very important with an
Parenchymal contrast enhancement (PCE) occurs in up to acute onset in acute infarct and a rather insidious onset
26% of acute infarcts within the first 24 hours in one series. in the mimics. The mimics tend not to restrict diffusion
This figure rises to 100% by the end of the first week (sub- and do not conform to a vascular territory. Otherwise a
acute infarcts) and gradually declines over the next 4 weeks. short follow-up evaluation of the temporal profile of the
The gradual decline tends to exclude the differential diagno- enhancement may be necessary to establish the natural
ses of inflammatory, neoplastic, or vascular malformations history.

Case
10 CLINICAL HISTORY 54-year-old male with ataxia.
FINDINGS Figure 10-1. Axial FLAIR through the bra- Be wary of other possibilities, however, as other etiologies
chium pontis. There is a hyperintense lesion within the left including tumor and demyelinating disease can also restrict
middle cerebellar peduncle (arrow). Figure 10-2. Axial DWI diffusion.
through the lesion. The lesion restricts diffusion (arrow).
DIFFERENTIAL DIAGNOSISInfarction, demyelinating Infarction should always be considered in the context of an
disease, metastasis, tumor, infection. isolated lesion with restricted diffusion
In the brainstem and cerebellum, the vascular distribution
DIAGNOSIS Infarction. is less familiar than supratentorially and is subject to vari-
ability. As such, a lesion may not seem at first glance to
DISCUSSION The lesion shown above was the only lesion correspond to a vascular distribution. Ischemic lesions in
to be seen in an otherwise unremarkable brain. While the the brainstem often respect the midline which may be a
fact that the lesion is solitary does not necessarily exclude valuable clue
many items from a potentially long differential, it does tend
to favor ischemia, particularly given the hyperintensity on Answer
DWI. Demyelinating disease usually, but not always, pres- 1. The midbrain is supplied by branches of the posterior
ents with multiple lesions, as does metastatic disease, and cerebral artery (PCA), and the pons is supplied by per-
infection. This lesion could certainly represent a tumor, even forating vessels off the basilar artery. The upper half
with restricted diffusion, as some tumors do indeed restrict of the cerebellum, including the superior cerebellar
diffusion. However, in this case, the clinical context was peduncle, and half of the middle cerebellar peduncle,
compatible with an ischemic event, and MRA (not shown) is supplied by the superior cerebellar artery (SCA). The
revealed abrupt termination of a branch of the left posterior lower half of the cerebellum, including the lower half of
inferior cerebellar artery (PICA) adjacent to the site of stroke. the middle cerebellar peduncle, is supplied through the
PICA. The anterior inferior cerebellar artery (AICA)
Question for Further Thought supplies the petrosal surface of the cerebellum, a por-
1. What are the vessels responsible for supplying the brain- tion of the lateral inferior cerebellar cortex.
stem and cerebellum?
Direct reporting is necessary whenever a stroke is suspected.
Suggest ischemic infarct when an area of restricted diffu-
sion is found correlating to a known vascular distribution.
20
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Case
11 CLINICAL HISTORY 37-year-old male with seizures.
FINDINGS Figures 11-1 and 11-2. Axial DWI with cor- cranial fossa and suprasellar region (arrows). Figure 11-3.
responding ADC map through the posterior fossa. There Axial T2WI through the posterior fossa. There is a right
is a heterogeneous DWI hyperintense mass which is isoin- CPA, prepontine cistern, suprasellar and adjacent right
tense (to brain) on ADC map in the right cerebellopontine mesiotemporal hyperintense mass with mass effect on
angle (CPA). The mass extends into the right middle surrounding structures (arrows). The intensity is that of
21
73284_Online-case011_p021-023.indd 21 16/09/14 7:57 AM

22 Case 11
reach a large size with significant mass effect on surround-

ing structures but absolutely no parenchymal edema or
contrast enhancement. White epidermoid which is a rarer
form of epidermoid cyst has unique imaging characteristics
appearing hyperintense on T1WI and hypointense on T2WI,
thus resembling dermoid.
Epidermoid cyst is usually a well-circumscribed lobulated
non-contrast-enhancing hypodense (CSF density) mass on
CT. However, minimal rim enhancement along the periph-
ery has been demonstrated in approximately 25% to 35% of
cases. Just as in the MRI, there is no surrounding edema.
Secondary dystrophic calcification is a rare occurrence in
about 10% and is thought to be caused by peritumoral leak-
age of cyst contents. Hyperdense cyst, the so-called white
epidermoid, is uncommon.
Epidermoid cyst is rare and typically asymptomatic until
the third to fifth decades of life with presentation at a mean
age of 40 years. Clinical presentation depends on the loca-
Figure 11-5 tion of the lesion, extension, and mass effect on adjacent
vital structures. Presentation may include mild headaches,
seizures, and rare fatal events. Trigeminal neuralgia, hemi-
facial spasm, tinnitus, gait ataxia, diplopia, and dysphagia
CSF. Figure 11-4. Axial FLAIR through the suprasellar
are some of the presenting symptoms of posterior fossa epi-
cistern. There is a right mesiotemporal/prepontine/upper
dermoid. Pineal region lesions may cause hydrocephalus
right CPA somewhat heterogeneous hypointense mass.
and Parinaud syndrome. Epidermoid cyst is a congenital
There is deformity of and mass effect on the brainstem
anomaly thought to arise from misplaced inclusions of ecto-
and right temporal lobe with compression of the right
dermal squamous epithelial remnants trapped during neural
temporal horn (arrow). Figure 11-5. Right parasagittal
tube closure and separated from the ectoderm between the
third and fifth weeks of intrauterine development. During
post-contrast T1WI. There is a non-contrast-enhancing
lobulated hypointense right CPA and suprasellar mass
this same period of embryogenesis, the otic and optic vesi-
(arrows) compressing the right cerebellum, brachium
cles are also being formed, and it is believed that migration
pontis, and midbrain.
along these or other developing neurovascular structures
accounts for the lateral placement seen in most epidermoid
DIFFERENTIAL DIAGNOSISArachnoid cyst, epider-
cysts. It is speculated that inclusions occurring prior to
moid cyst, dermoid cyst.
the third week of embryologic development may result in
intraventricular and intracerebral lesions, as this coincides
DIAGNOSIS Epidermoid cyst.
with formation of the primary cerebral vesicle. The cyst is
lined with keratinizing stratified squamous epithelium. As
DISCUSSION Epidermoid cyst or pearl tumor is best
desquamation occurs, the cystic cavity fills with epithelial
imaged by MRI. This tumor is subarachnoid in location. It is
cells consisting predominantly of keratin in concentric lay-
most frequently located in the CPA. Location in or extension
ers, water, and cholesterol from cell membrane degradation,
into the prepontine and suprasellar cisterns and the middle
giving the tumor its pearly appearance.
cranial fossa is not uncommon. It could be found in the pineal
Treatment is gross total surgical resection which gen-
region or within the ventricles and anywhere intracranially.
erally carries a good prognosis. The potential for damage
It generally follows CSF intensity on spin echo sequences
to cranial nerves and adjacent critical structures during
appearing hypointense on T1WI and hyperintense on T2WI.
aggressive resection exists. Aseptic meningitis due to rem-
However, this presentation has been shown to be somewhat
nant cyst material is a known complication of surgery.
variable depending on the cyst contents with some degree
of signal heterogeneity. It could be slightly hyperintense
on FLAIR. Epidermoid cyst is usually heterogeneously Question for Further Thought
hyperintense on DWI with ADC map approaching that of 1. Why is white epidermoid different from the regular epi-
the brain parenchyma. This distinguishes it from arachnoid dermoid cyst?
cyst which has CSF intensity pattern on DWI/ADC map.
Dermoid on the other hand is well circumscribed mainly Reporting Responsibilities
midline to parasagittal in location with T1 hyperintensity Although this is a benign process, the significant mass effect
and heterogeneous T2 hypointensity. Epidermoid cyst could requires direct reporting. Presence of hydrocephalus makes

Case 11 23
direct reporting more urgent. The location and extent of the Answer
mass should be described in detail. 1. White epidermoid is hyperdense on CT and hyperintense
on T1WI; changes thought to be due to a relatively high
protein concentration and large fraction of albumin. It is
What the Treating Physician Needs to Know hypointense on T2WI probably on the basis of the higher
Location and extent of the mass viscosity of the cyst content. Its lipid content is also
Presence of significant mass effect or hydrocephalus believed to be different from typical epidermoid cyst.

Case
12 CLINICAL HISTORY 12-year-old female with complex partial seizures.
FINDINGS Figures 12-1 and 12-2. Axial T2WI and GRE DISCUSSION FCDT occurs when the top layer of the brain
through the frontal lobes demonstrate expanded right frontal does not form properly and is one of the common causes
gyrus with ill-defined hyperintensity in the subcortical white of epilepsy. It is one form of cortical dysplasia with many
matter (WM) extending in a flame-shaped fashion into the other types described. MRI may be negative in as many as
deep WM in Figure 12-1 (arrows). Figure 12-3 and 12-4. 33% of FCDT. MRI best characterizes the appearance which
Axial NCCT and corresponding coronal T2WI through the may be nonspecific. There is GM and WM involvement; T2
frontal lobes in a companion case demonstrate a right frontal hyperintensity in subcortical WM location, extending/taper-
lobe subcortical hypodensity on CT and corresponding sub- ing in a flame-shaped fashion to the ventricle, overlying cor-
cortical hyperintensity on the coronal T2WI (arrows). tical GM thickening with cortical expansion and abnormal
sulcal pattern. There may be a focal mass effect. There is
DIFFERENTIAL DIAGNOSISGliosis, polymicrogyria, usually no contrast enhancement or restricted diffusion. The
tuberous sclerosis, low-grade tumor, focal cortical dysplasia. CT shows a subcortical hypodensity with overlying cortical
thickening. FDG-PET is shown to be a sensitive technique in
DIAGNOSIS Focal cortical dysplasia (balloon cell focal identifying areas of cortical dysplasia with FDG-PET detect-
cortical dysplasia of Taylor [FCDT]). ing interictal hypometabolism localized to areas of cortical
24

Case 12 25
dysplasia in approximately 75% of patients. Gliosis tends to dysplasia. Should these fail to control the seizures, surgery
be associated with volume loss with similar intensity pattern may be a viable option. Completeness of resection of the
to FCDT. Low-grade tumors may show a rounder or a more dysplastic tissue is felt to be one critical factor in determin-
diffuse configuration, usually noncontrast enhancing with ing the outcome of surgery.
local mass effect. Polymicrogyria show rather small gyri
with shallow sulci. Questions for Further Thought
FCD is a heterogeneous group of congenital migrational 1. Can imaging differentiate subcortical tuber of tuberous
abnormality characterized by the presence of abnormal neu- sclerosis complex (TSC) from cortical dysplasia?
rons and glial cells within a localized region of the brain. The 2. Is IV contrast imaging or DWI helpful in distinguishing
various classifications of cortical dysplasia have not gener- between low-grade tumor and dysplasia?
ally been satisfactory. Non-Taylor FCD has been classified
into two types: architectural and cytoarchitectural dyspla- Reporting Responsibilities
sias. The most recent classification recognizes three types Routine reporting is sufficient in this indolent lesion. Size,
of FCD. In type I the MRI may be normal or with a small location, number if more than one and signal abnormality of
focus in the temporal lobe. Seizures may not develop until dysplastic tissue should be described.
adulthood. Type II is a more severe form of cortical dyspla-
sia seen in children and can involve the temporal but mainly What the Treating Physician Needs to Know
the frontal lobes of the brain. FCDT falls into this category. Use of a combination of anatomic and functional neuro-
Type III includes any of types I and II with associated lesions imaging studies increases accuracy in locating the lesion
elsewhere in the brain that may include mesiotemporal scle-
rosis, vascular malformation, and tumor. This type may also Answers
be due to some form of brain injury early in life. FCD may 1. The presence of periventricular lesions particularly calci-
cause severe refractory epilepsy with the visible imaging fications may point in the direction of TSC. The subcor-
lesion significantly smaller than the seizure-generating area. tical T2 hyperintensity are similar in both entities. The
There are no clinical characteristics that differentiate FCD histology of subcortical tubers and FCD could be similar.
from neoplasms. The histologic correlate of the lesion con- 2. No. Contrast imaging or DWI does not help differenti-
sists of large, bizarre, disoriented neurons and the presence ate tumor from FCDT. Both are non-contrast-enhancing
of balloon cells in the subcortical WM and cortex, histologic and do not restrict diffusion. The flame-shaped tapering
pattern very similar to tuberous sclerosis. Anticonvulsants toward the ventricle and frontal lobe location of FCDT
will typically be the first-line of treatment offered for cortical appear unique and may help in separating both entities.

Case
13 CLINICAL HISTORY 25-year-old male who presented with left retro-orbital
pain followed by multiple punctate visual scotomas 5 days later.
FINDINGS Figure 13-1. Coronal MR T1WI of the orbits. and contrast enhancement. These are best visualized on the
There is subtle asymmetry of the optic nerves (arrows). T2WI, short tau inversion recovery (STIR), and post-con-
Figure 13-2. Coronal T2WI with fat suppression of the orbits trast fat-suppressed T1WI. MRI findings are nonspecific
through the same level as in Figure 13-1. There is hyper- and cannot distinguish inflammatory, ischemic, infec-
intensity of the left optic nerve with effacement of the sur- tious, or neoplastic optic neuropathy. Neuromyelitis optica
rounding cerebrospinal fluid (CSF) (transverse arrow). The (NMO)-related ON tends to be posterior with more chias-
right optic nerve is surrounded by hyperintense CSF within matic involvement and contrast enhancement. They are also
the sheath (vertical arrow). Figure 13-3. Coronal fat sat post- more likely to be bilateral. The history and brain and spine
contrast T1WI through the orbits. There is contrast enhance- imaging findings are important in the differential diagno-
ment of the left optic nerve (arrow). Figure 13-4. Coronal sis. However, large nodular enhancement of the optic nerve
T2WI through the corpus callosum. There are two corpus suggests neoplasm.
callosum hyperintense foci (arrows). ON should be the first differential diagnosis in a young
patient with acute to subacute visual loss associated with
DIFFERENTIAL DIAGNOSIS Optic neuritis (ON), optic pain. The visual loss could be variable in severity from
neuropathy, optic sheath meningioma, optic nerve glioma. dyschromatopsia (mainly red desaturation) to severe visual
loss. Pain is present in 90% of the cases. The diagnosis of
DIAGNOSIS Optic neuritis (ON) acute. typical ON is clinical. MR is useful in those atypical cases
to avoid misdiagnosis and particularly to exclude compres-
DISCUSSION CT does not ordinarily do justice to evalu- sive lesions. Two-thirds of cases of ON are retrobulbar and
ation of the soft tissues of the orbits. The MRI findings in therefore the fundoscopy could be normal. Optic pallor,
acute ON include optic nerve swelling, T2 hyperintensity, however, might develop 4 to 6 weeks later and the relative
26

Case 13 27
afferent pupillary defect (RAPD) may disappear. Visually therefore are considered for patients who require faster
evoked potentials are useful for detection of subclini- recovery, such as monocular patients, patients with severe
cal ON but not for established ones. CSF analysis should bilateral visual loss, or those with occupations requiring
be reserved for atypical ON, bilateral cases, systemic or normal visual acuity (VA). Bilateral lesions are more com-
infectious ones. Multiple sclerosis (MS) is thought to be mon in NMO
the leading cause of ON where it could be the CIS in about Location and extent of lesion. Long segment and cana-
50% of cases. It is also a part of the NMO spectrum. The licular segment involvement appear to be associated with
differential diagnosis is broad and includes other optic severe visual loss and a poor prognosis for visual recov-
neuropathies seen in the context of sarcoidosis, chronic ery. Posterior involvement appears to be more common
relapsing inflammatory ON (CRION), connective tissue in NMO
disease, and vasculitis. Any associated brain lesions to suggest MS, NMO, sar-
coidosis, etc.?
Questions for Further Thought Any compressive lesions?
1. What is the natural history of ON?
2. What is the risk of developing MS following the first Answers
attack of ON? 1. Visual recovery occurs spontaneously around 2 to 3 weeks
in 80% of the affected eye, stabilizes over months, and
Reporting Responsibilities continues to improve up to 1 year.
Presence of ON could follow normal reporting practice as 2. MRI of the brain has a prognostic role in terms of defin-
long as there is no compressive disease. MR just confirms the ing the risk of a patient for a second MS-defining episode.
clinical impression. Since ON is a component of the NMO As a rule of thumb the presence of more than one ovoid
spectrum disorder, presence of myelopathic signs should lesion increases the risk of developing MS. Other than
prompt evaluation of the spinal cord. the MRI findings, male gender, the presence of papillitis
(i.e., anterior involvement rather than retrobulbar), severe
What the Treating Physician Needs to Know vision loss (no light perception), lack of pain, retinal exu-
Is it unilateral or bilateral? High-dose corticosteroids dates, and peripapillary hemorrhages are associated with
improve short-term but not long-term visual recovery and a lower risk for developing MS.

Case 14 CLINICAL HISTORY 28-year-old female who presented with 6-month history of
vertigo followed by headache, left arm and leg numbness, and left lower homonymous
quadrantanopia.
28

Case 14 29
Figure 14-5
FINDINGS Figures 14-1 and 14-2. Axial DWI with ADC

map through the corpus callosum (CC) and bilateral forceps
major showing confluent asymmetric bilateral hyperinten-
Figure 14-6
sity larger on the right than left crossing the splenium of the
CC. There is involvement of the subcortical U fibers on the
right (vertical arrows in Figure 14-2). Some areas of sub-
tle restricted diffusion are seen within the splenium of CC reported in demyelination. The definition of tumefactive
(transverse arrows) in Figure 14-2. Figure 14-3. Axial FLAIR demyelination is not consistent in the literature and may
through the same level showing splenium and bilateral for- refer to various combinations of the following: WM location,
ceps major confluent hyperintensity surrounding isoin- large size (>2 cm), little mass effect or edema, and/or typical
tense core extending into the subcortical region on the right enhancement patterns (nodular, patchy, thin open or incom-
(arrows). There is no significant mass effect. Figures14-1 to plete ring, heterogeneous). The butterfly pattern fits any of
14-3 show enlargement of the splenium. Figure 14-4. Axial the listed differential diagnoses. Butterfly glioma tends to
MRI perfusion relative Cerebral Blood volume (rCBV) be necrotic and has a thick irregular ring contrast enhance-
map through the lesion showing low blood volume in the ment with surrounding large vasogenic edema and increased
splenium and bilateral forceps major (arrows). Figure 14-5. blood flow and volume on perfusion studies. Lymphoma on
Post-contrast coronal T1WI through the splenium showing the other hand is highly cellular and may show restricted dif-
patchy nodular contrast enhancement through the splenium fusion and avid contrast enhancement. Necrosis is not a com-
and lateral to the right trigone (arrows). Figure 14-6. Axial ponent of lymphoma except in the setting of HIV. It has been
DTI color orientation map through the splenium showing suggested that NCCT may be able to differentiate TDL from
disruption of fiber tracts with reduced FA in the splenium, GB and lymphoma on the basis of attenuation pattern on CT;
bilateral forceps major, and the right posterior corona radiata lymphoma is usually hyperdense.
(arrows). Cerebrospinal fluid (CSF) analysis prior to biopsy
showed minimal increase in protein and an increase in IgG
DIFFERENTIAL DIAGNOSISDemyelinating process, synthesis rate. There were no pleocytosis or oligoclonal
lymphoma, butterfly glioma. bands. Bacterial, viral, and fungal studies were negative.
MRI of the cervical and thoracic spine was normal. The
DIAGNOSIS Tumefactive demyelinating lesion (TDL). diagnosis was made by stereotactic brain biopsy through
the right parietal lesion. TDL of the central nervous sys-
DISCUSSION MR findings of demyelination include tem (CNS) can be solitary or multiple. In the presence of
white matter (WM) T2 hyperintensity anywhere in the brain preexisting demyelinating lesions such as multiple scle-
including the CC, minimal surrounding edema and mass rosis (MS) the diagnosis may be easy. Dissemination in
effect, nodular, patchy, incomplete ring, and arc patterns time and space is necessary to convert to diagnosis of MS.
of contrast enhancement on post-contrast T1WI. However, Scrutinization of the history and prior imaging if available
contrary to popular belief, TDL can be associated with mass becomes important in this regard. TDL has been described
effect and edema. Diffusion restriction could be subtle but rarely in association with astrocytoma. Solitary TDL has
not common and usually indicates acute lesions. Low rela- preceded the appearance of lymphoma, and therefore, lon-
tive blood volume and flow on perfusion studies have been gitudinal monitoring of single TDL is warranted.

30 Case 14
Question for Further Thought Other imaging studies that may narrow the list of differen-
1. What other lesion could be confused with the histology tial diagnosis include MR spectroscopy, MRI of the spinal
ofTDL? cord, and MR of the orbits in the presence of visual distur-
bance to exclude other forms of demyelination
Direct reporting is required of a mass lesion. In the presence Answer
of a single solitary intracranial lesion suspicious for demy- 1. TDL may be misinterpreted as a neoplasm given its
elination, imaging of the spinal cord should be recommended hypercellular nature and the frequent presence of atypical
to exclude other lesions that could confirm presence of a reactive astrocytes and mitotic figures.
demyelinating process elsewhere.

Is this a single lesion or are there other lesions that may
suggest MS?

Case
15 CLINICAL HISTORY 12-year-old female with cough and headache.
FINDINGS Figure 15-1. Axial T2WI through the infe- collection (arrow), indicating high cellularity within the col-
rior frontal lobes. There is hyperintense fluid opacification lection and suggestive of an empyema. Figure 15-3. Axial
within both frontal sinuses (transverse arrows) with a small post-contrast T1WI with fat saturation through the frontal
left frontal lenticular-shaped T2 hyperintense extraaxial col- lobes, performed 2 days after Figures 15-1 and 15-2. There
lection (vertical arrow). Figure 15-2. Axial DWI demon- is a much larger frontal epidural collection with thick dural
strates restricted diffusion within the left frontal extraaxial enhancement and displacement (arrows). Note how the
31

32 Case 15
frontal epidural collection displaces the anterior falx poste- therapy and/or surgical management. Therefore the spaces
riorly, confirming epidural rather than subdural location of immediately adjacent to opacified paranasal sinuses should be
the collection. Figure 15-4. Axial post-contrast T1WI with carefully scrutinized for spread of infection. Frontal sinus dis-
fat saturation through the ethmoid sinuses, performed at the ease can spread into the epidural space and through the frontal
same time. There is mucosal thickening and enhancement in bone into the extracranial subgaleal space. Frontal bone and
the ethmoid air cells. There is a left orbital medial extraconal extracranial space involvement is also known by the eponym
subperiosteal enhancement suggesting phlegmon (arrow). Potts puffy tumor; referring to an acute presentation with a
There is mild left orbital proptosis. fluctuant frontal region mass, a subperiosteal abscess, devel-
oping as an extension of adjacent frontal bone osteomyelitis.
DIFFERENTIAL DIAGNOSIS Sinusitis with complication, Our patient returned 2 days later with a much larger fron-
leukemic or lymphomatous infiltration, epidural abscess. tal epidural empyema and a new subperiosteal abscess in
the left medial postseptal orbit; despite early detection and
DIAGNOSIS Frontal/ethmoid sinusitis complicated by epi- prompt initiation of antibiotic therapy. The frontal epidural
dural empyema and left orbital subperiosteal abscess. empyema required surgical evacuation and a prolonged
course of intravenous antibiotics. Our patients aggressive
DISCUSSION Both CT and MRI can demonstrate epidural clinical course underscores the need to maintain a high index
abscess or empyema. However, the superiority of MRI lies of suspicion for complications of paranasal sinus disease.
in succinct characterization of the fluid as an abscess on the
basis of the DWI restricted diffusion. The location of the col- Question for Further Thought
lection between the dura and the cranial vault or skull base 1. What is another important complication of paranasal
makes it epidural. There is stripping and displacement of the sinus disease?
dura away from the inner table and the extent of the collec-
tion is limited by the dural attachment at the sutures. The Reporting Responsibilities
collection is hypodense on CT with dural enhancement. It Direct reporting is essential. A contrast-enhanced MRI
is hypointense on T1WI and hyperintense on T2WI. There examination should be considered once an epidural abscess
is intense enhancement of the dura. Significant mass effect collection is suspected in order to completely delineate the
could occur with herniation of the brain. The surrounding extent and to look for subtle extension into the brain paren-
parenchymal changes of edema and compression if any are chyma or dural venous sinuses.
also well demonstrated by MRI. Our patient developed a
frontal epidural abscess collection, which while subtle on the What the Treating Physician Needs to Know
T2WI is well depicted on the DWI. DWI can be extremely Extent of the collection and location
helpful in confirming the hypercellular nature of extraaxial Potential structures at risk or complications
fluid collections, such as might be seen with blood products Associated pathology such as possible sources of the
or epidermoid tumors. Epidural empyema could be single infection
or multifocal. Organisms reach the epidural space by direct
invasion as in this case, through the bloodstream or by direct Answer
inoculation at surgery. The potential differentials enhance in 1. Dural venous sinus thrombosis. The frontal sinus venous
a solid homogeneous fashion. drainage is by valveless diploic veins of Galen. Frontal
An important imaging goal in the setting of acute sinus dis- sinus infection can therefore spread by retrograde throm-
ease, besides the detection of inflammatory change within the bophlebitis into the superior sagittal sinus via these
paranasal sinuses, is to detect the potential complications of diploic veins of Galen. Similarly, mastoiditis can be com-
the disease. It can be argued that detection of complications of plicated by thrombosis of the ipsilateral transverse/sig-
sinusitis is even more important than confirming the presence moid sinuses. MR venography (MRV) with intravenous
of inflammatory change, which is typically self-limiting in contrast would be the examination of choice to exclude
the vast majority of patients. Early detection of complications venous sinus thrombosis. Cavernous sinus thrombosis can
allows for expedient administration of appropriate antibiotic complicate sphenoid sinusitis.

Case
16 CLINICAL HISTORY 31-year-old female with multiple skin nodules
referred to establish care for brain lesions.
FINDINGS Figure 16-1. Axial T2 FLAIR through the fora-

men magnum. There is an ovoid hyperintense mass pos-
teriorly and to the right at the junction of the medulla and
spinal cord (arrows). This mass did not enhance with con-
trast. Figure 16-2. Axial T2WI through the upper pons. There
is a transverse basis pontis hyperintense lesion (arrow) that
did not enhance. Figure 16-3. Axial FLAIR through the lat-
eral ventricles. There are focal hyperintense lesions in the
left corona radiata (transverse arrow) and right splenium
(vertical arrow). Both lesions did not enhance with contrast.
Figure 16-4. Coronal post-contrast T1WI through the chiasm.
There is a nonenhancing isointense thickening of the chiasm
(arrow) consistent with optic pathway glioma (OPG). There
is ex vacuo dilatation of the right frontal horn with atrophy
Figure 16-5 of the right caudate nucleus due to an old cerebrovascular
33

34 Case 16
accident (CVA). Figure 16-5. Coronal post-contrast T1WI e legantly demonstrated by CT. The hallmark of NF2 is bilat-
through the occipital lobes. There are two contrast-enhancing eral vestibular schwannomas.
left scalp masses (arrows) consistent with neurofibromas. NF1 is an autosomal dominant neurocutaneous disorder
This patient had an astrocytoma (not shown), removed from occurring in about 1:3,000 births. It is caused by a muta-
the cerebellum. tion in NF1 gene (neurofibromin), a tumor suppressor gene
located on chromosome 17q11.2. Its clinical presentation
DIFFERENTIAL DIAGNOSISNeurofibromatosis type 1 is protean and includes subcutaneous nodules, Lisch nod-
(NF1), Neurofibromatosis type 2 (NF2). ules, caf-au-lait spots, kyphoscoliosis, bone dysplasia,
macrocranium, cognitive deficits, OPG, and astrocytomas.
DIAGNOSIS Neurofibromatosis type 1 (NF1). Diagnosis is made on the basis of set criteria. Because of the
large number of lesions and unpredictability of their growth,
DISCUSSION Intracranial abnormalities are present in about it is recommended that only symptomatic lesions be treated.
15% of NF1 population. The most common lesions are white
matter (WM) T2 hyperintensity and astrocytomas. These Question for Further Thought
are most suitably imaged by contrast-enhanced MRI. WM 1. Is there a place for surveillance imaging in NF1?
lesions are multifocal poorly defined non-contrast-enhancing
areas of T2 hyperintensity usually located in the brainstem,
cerebellum, basal ganglia, corona radiata, and the splenium
Routine reporting is sufficient. However, presence of hydro-
of the corpus callosum (CC). They are more common in chil-
cephalus, CVA, and other acute changes may necessitate
dren than adults; they tend to grow over a period of time
direct reporting. There should be complete categorization of
and subsequently disappear. These lesions are generally not
all tumors and WM changes for cross-clinical referencing.
related to tumors or edema and have been described as areas
of myelin vacuolation. DTI has shown widespread decreased
fractional anisotropy and significant increased ADC in NF1 What the Treating Physician Needs to Know
WM suggesting reductions in WM integrity across the entire Complete tabulation of all lesions to determine which one
brain. The astrocytomas are commonly present in the optic is symptomatic and treatable
pathways (OPG), the cerebellum, and elsewhere in the brain. Only lesions that are symptomatic are recommended for
They are usually low-grade pilocytic astrocytomas, usually treatment
mixed intensity masses with variable contrast enhancement Progression of lesions is unpredictable and may have salu-
and mass effect. The contrast enhancement and mass effect tatory growth
differentiate these tumors from the WM T2 hyperintensities.
However, non-contrast-enhancing astrocytomas that are Answer
small and homogeneous in intensity may be difficult to 1. Surveillance imaging in NF1 is controversial. The usual
differentiate from WM lesions. Anaplastic astrocytomas
reason for surveillance imaging is to discover lesions
and glioblastoma (GB) are not common. Large and strategi- before they become symptomatic. Discovering new
cally placed astrocytomas may obstruct cerebrospinal fluid tumors does not necessarily translate into treatment in
pathways resulting in hydrocephalus. Other cranial lesions NF1. It is recommended that only symptomatic tumors
include internal auditory canal (IAC) neurofibroma, menin- be treated. There is also the added cost, anxiety, and
gioma, stenosis of intracranial internal carotid artery (ICA), invasiveness of some procedures particularly in children
absent greater wing of the sphenoid (the bare orbit sign), requiring sedation or anesthesia for imaging of lesions
and bone defect in the mastoid region. The bone changes are that may not affect the management of these patients.

Case 17 CLINICAL HISTORY 38-year-old female with headaches and hypopituitarism.
DIFFERENTIAL DIAGNOSISRathke cleft cyst, cystic

pituitary adenoma, craniopharyngioma.
DIAGNOSIS Rathke cleft cyst.
DISCUSSION Typical MRI findings of Rathke cleft cyst

include a nonenhancing lesion in the mid-aspect of the
pituitary gland. About 50% are hyperintense and 50% are
hypointense on pre-contrast T1WI secondary to differences
in the mucinous component of the fluid. Characteristic find-
ings include a nonenhancing mural nodule within the cyst
and multiple small cysts within the cyst on T2 imaging. They
rarely arise in the pituitary stalk.
Rathke cleft cyst (aka pars intermedia cyst) is a result of
failure of regression of Rathke cleft, which is the embryo-
logic separation between the adenohypophysis and the neu-
rohypophysis. Rathke cleft is thought to be a remnant of the
superior extension of the stomodeum, which is the rostral ter-
Figure 17-3 mination of the aerodigestive tract in the fetus. Rathke cleft
cysts are relatively common, found in up to 33% of patients
at autopsy. They are generally asymptomatic and found inci-
FINDINGS Figure 17-1. Sagittal T1WI. There is an intra- dentally. Occasionally, they can become large enough to
sellar mass that is isointense with the brain (arrow). The produce symptoms secondary to mass effect with the most
hyperintense neurohypophysis is not present. Figure 17-2. common symptoms being headaches, visual field changes,
Coronal post-contrast T1WI through the sella turcica. The and pituitary dysfunction. Treatment is generally expectant
mass does not enhance but its periphery does (arrow) prob- with surgical excision reserved for symptomatic cases.
ably due to surrounding normal pituitary tissue. Figure 17-3.
Axial T2WI through the sella. There is a posterior mural Questions for Further Thought
nodule (arrow) that is typical of this entity and helps differ- 1. What is the embryologic function of Rathke cleft?
entiate it from other c ystic pituitary masses. 2. What is the significance of the nonenhancing nodule?
35

36 Case 17
Reporting Responsibilities Answers

Routine reporting is sufficient unless it is large and com- 1. The adenohypophysis forms from the ventral portion of
pressive. Describe the abnormality and its extent. Advise the Rathke cleft. The remaining portion of the cleft forms
clinician of lack of requirement of follow-up imaging in the pars intermedia that normally regresses.
asymptomatic cases once the diagnosis is achieved. 2. The nonenhancing nodule has been described as a mucin
clump on pathologic evaluation. This corresponds with the
What the Treating Physician Needs to Know MRI findings of a nonenhancing nodule that demonstrates
Relevant differential diagnosis T1 hyperintensity on pre- contrast imaging.
Relative benignity of Rathke cleft cyst

Case
18 CLINICAL HISTORY 63-year-old female with prosthetic cardiac valve with
right-sided weakness.
FINDINGS Figure 18-1. Axial NCCT through the temporal There is very minimal if any diminution in cerebral blood
lobes before CT perfusion (CTP). There is normal appear- volume (CBV) in the left temporal lobe (star) compared
ance of the temporal lobes without infarct. Figure 18-2. Axial with the right. Figure 18-3. Axial CTP blood flow color map
CTP blood volume color map through the temporal lobes. through the temporal lobes. There is significant diminution
37

38 Case 18
ischemia results in focal brain death, it is termed cerebral

infarction which is permanent and irreversible.
CBV is measured as the amount of blood in milliliters
per 100 g of brain tissue. CBF is measured as the amount
of blood in milliliters flowing through 100 g of brain tissue
per minute while the MTT is the time it takes a quantity of
blood to pass through a volume of brain and is measured
in seconds. The quantification of the parameters varies
depending on the equipment and software, and there is no
standardized threshold for guiding therapy. There are con-
flicting figures in the literature. Presented here are some fig-
ures available in the literature, and they are for guidance
only. Values are generally higher in the gray matter (GM)
than white matter (WM) because of the higher demand of
the GM for energy. The higher end of the values are there-
Figure 18-5 fore for the GM. Normal mean CBV is 2 to 4 mL/100 g.
Total volume of blood to the brain is 800 mL/min (15%
to 20% cardiac output) and the normal CBF is 21 to 65
in the left temporal lobe cerebral blood flow (CBF) (star) mL/100 g/min. Infarct core CBF threshold is set at 4.8 to
compared with the right. Figure 18-4. Axial CTP mean 8.4 mL/100 g/min while tissue infarction threshold is 17 to
transit time (MTT) color map through the temporal lobes. 18 mL/100 g/min and the salvageable or ischemic penum-
There is prolonged MTT (star) through the left temporal bra threshold is 14.1 to 35 mL/100 g/min. MTT increase
lobe compared with the right. Figure 18-5. MIP CTA of higher than 145% has been suggested to define the penum-
the head. There is a filling defect in the left middle cerebral bra or salvageable tissue. It is easier to use the color maps
artery (MCA) bifurcation (arrow), a saddle embolus. There when quickly looking for a mismatch. Reduced CBV and
is minimal attenuation or reduced caliber of some left MCA prolonged MTT indicate infarction. When the size of the
branches compared with the right suggesting diminished reduced CBV is equal to the size of the prolonged MTT, the
blood flow. so-called matched CBV and MTT, this defines the infarct
core, and usually there is no benefit from intervention in
DIFFERENTIAL DIAGNOSIS Acute infarct left MCA ter- this situation. When the size of the reduced CBV is smaller
ritory, ischemia left MCA territory. than prolonged MTT, there is a CBV/MTT mismatch which
defines the presence of a penumbra or salvageable brain, and
DIAGNOSIS Ischemia without infarct left MCA territory. this may benefit from intervention. When there is normal
CBV with prolonged MTT as in our patient, this defines
DISCUSSION CTP could be useful in evaluating the an ischemic region of the brain and suggests reversibility,
patient presenting with an ischemic event. CTP could be and the patient may benefit from appropriate intervention.
useful in a situation where the structural imaging (NCCT) That intervention in this patient could be thrombectomy or
is equivocal or normal to identify the presence of disturbed thrombolysis.
blood supply to a particular portion of the brain and deter-
mine corrective action. CTP may help identify patients most
likely to benefit from intervention. However, the interpreta-
tion of the CTP information is rather complex and could be 1. If there is total occlusion of the MCA bifurcation, in what
challenging. There is no standardization of software or mea- other way can blood get to the MCA territory to prevent
surements. We will deal with some fundamental information or reduce the size of infarction?
obtainable from the CPT and how that information could
be useful in triaging the stroke patient. We will not get into Reporting Responsibilities
the complexity of performing the CTP or extensive debate As is often said, time is brain! This situation demands direct
about the numbers. The basic parameters usually measured reporting so decision could be made to address reversibility
by CTP are CBF, CBV, and MTT. These are calculated from of the ischemia.
the source data of the CTP by deconvolution analysis. To
discuss these parameters as they apply to the stroke patient,
some basic definitions may be necessary. Cerebral ischemia What the Treating Physician Needs to Know
defines inadequate blood supply or oxygenation to the brain. Location and size of the ischemic region as measured on
This could be due to poor perfusion because of low or no the CBF and MTT
blood flow to the brain. This could be reversible. When The pattern of mismatch

Case 18 39
Answer vessels from the anterior cerebral artery (ACA) and pos-
1. In this situation, there is reduced blood flow as measured terior cerebral artery (PCA) or other congenital collater-
by CTP. However, with a normal or near normal CBV, als may supply blood to the MCA territory. There may
blood is getting into the MCA territory somehow. The already be augmentation of the blood flow in this case,
main avenue for blood to get behind an occluded vessel we cannot be sure.
is by collateral circulation. In this case leptomeningeal

Case
19 CLINICAL HISTORY Young patient with hypotonia and developmental delay.

Courtesy of M. Castillo MD. Courtesy of M. Castillo MD.

Courtesy of M. Castillo MD. Courtesy of M. Castillo MD.
FINDINGS Figure 19-1. Axial T2WI through the lower nodulus. There is lack of midline fissure in the single cerebel-
cerebellum. There is a single cerebellum without a midline lar hemisphere. Figure 19-3. Coronal T2WI through the cer-
fissure. The folia are oriented transversely, and there is con- ebellum. There is transverse orientation of the folia and WM
tinuity of the cerebellar white matter (WM) across the mid- (arrow). The cerebellum is pear shaped. Figure 19-4. Sagittal
line (arrows). There is no distinct vermis. Figure 19-2. Axial T1WI demonstrates what appears to represent the fastigium
T2WI through the fourth ventricle. There is fusion of bilat- (transverse arrow). The cerebellar hemisphere bulges infe-
eral middle cerebellar peduncles and dentate nuclei around riorly obliterating the cisterna magna (vertical arrow). The
the deformed fourth ventricle (arrows). There is no o bvious vermis is not visualized. There is normal appearance of
40

Case 19 41
supratentorial midline structures in this instance. There is no etiology is unknown and is generally ascribed to dorsoven-
hydrocephalus. The aqueduct is thin but otherwise normal. tral patterning defect between the 8 and 16 weeks of gesta-
tion. The clinical outcome varies depending on the severity
DIFFERENTIAL DIAGNOSIS N/A. and could be compatible with long life and incidentally dis-
covered in adulthood.
DIAGNOSIS Rhombencephalosynapsis (RES).
DISCUSSION The hallmarks of RES at imaging are a 1. Is RES strictly a hindbrain malformation?
lack of central fissuring in the cerebellum with formation of
a single cerebellum, transverse orientation of the folia with Reporting Responsibilities
continuity of white matter across the midline, a diamond- Routine reporting is sufficient unless there is unexpected
shaped (deformed) fourth ventricle with fusion of the cer- hydrocephalus. RES could easily be missed on CT evalua-
ebellar peduncles and dentate nuclei around it. The vermis tion of congenital hydrocephalus. These patients should at
is not visualized except in mild cases. The single cerebellum least have the benefit of MR evaluation, and this should be
is continuous at its base and bulges inferiorly to obliterate recommended. Detailed scrutiny of supratentorial structures
the cisterna magna. The cerebellar volume is preserved to should be undertaken in any situation where the imaging is
slightly decreased. Fusion of the colliculi could be pres- positive for RES.
ent. Some associated supratentorial anomalies may include
hydrocephalus which occurs in about 50% usually due to What the Treating Physician Needs to Know
aqueductal stenosis, deficiency or hypoplasia of the septum
Babies with congenital hydrocephalus should be evaluated
pellucidum, and dysgenesis of the corpus callosum and other
for the presence of RES
midline anomalies, none of which is present in this case.
RES cannot be adequately evaluated by CT. CT is there-
MR rather than CT offers the complete way to evaluate this
anomaly. fore not sufficient for evaluation of congenital hydroceph-
RES is a rare congenital anomaly of the hindbrain. It is alus. MR is the examination of choice
usually sporadic, but syndromic forms exist. Clinically, these In utero diagnosis of RES can be made by fetal MR
patients are generally hypotonic with developmental delay.
There is no gender preference. RES has been found in the Answer
context of two other syndromes; Gomez-Lopez-Hernandez 1. No. There is associated mid- and forebrain malformations
(GLH) syndrome where there is temporal parietal alopecia, that suggest that there is a domino effect of some form.
dysmorphic features, trigeminal anesthesia and turricephaly, These malformations include tectal fusion, mammillary
and the VACTERL anomaly which consists of vertebral body, corpus callosal, septum pellucidum and anterior
anomalies, anal atresia, cardiovascular anomalies, trachea commissural anomalies, absence of olfactory bulb, holo-
esophageal fistula, renal anomalies, and limb defects. The prosencephaly, and septooptic dysplasia to mention a few.

Case
20 CLINICAL HISTORY Adult with sensorineural hearing loss.
Figure 20-2
Figure 20-1
FINDINGS Figure 20-1. NCCT temporal bone. There is a DIAGNOSIS Vestibular schwannoma (VS).
smoothly marginated expansion of the right porus acusticus
and internal auditory canal (IAC) as compared with the nor- DISCUSSION NCCT best demonstrates the bony changes
mal canal on the left side. Note the blunting of the posterior of VS. MRI is the examination of choice for evaluation
lip (arrow). Figure 20-2. Axial post-contrast volumetric T1 of the total geography of the lesion. T2WI demonstrates
gradient sequence. There is an avidly enhancing lesion fill- replacement of the normal hyperintense CSF signal within
ing the majority of the IAC, laterally sparing the fundus with the IAC by VS (Figure 20-3). Like schwannomas elsewhere
demonstration of a small cerebrospinal fluid (CSF) fundal it enhances strongly with gadolinium contrast agents. Most
cap (arrow). There is no suggestion of intralabyrinthine are slowly growing and primarily extend medially into the
tumor extension. Medially, the mass protrudes slightly into CPA cistern from its origin within the IAC. VS may demon-
the cerebellopontine angle (CPA) cistern. Figure 20-3. Axial strate small foci of hypointensity (intratumoral hemorrhage)
T2 cisternographic sequence shows that the tumor arises from on SWI. Larger tumors may be associated with intratumoral
the more posterior, vestibular nerve at the level of the IAC, cysts or occasionally adjacent arachnoid cysts. In contrast,
displacing the cranial nerve (CN) VII/VIII complex (arrow- CPA meningiomas are less commonly intracanalicular, may
head) anteriorly within the canal. Figure 20-4. Companion have calcifications on CT, and rarely have intratumoral hem-
case: axial post-contrast T1WI. There is mixed solid and orrhage. A companion case, Figure 20-4, illustrates mixed
cystic vestibular schwannoma (VS) with the predominately solid and cystic VS with the predominately solid, enhancing
solid, enhancing component within the IAC extending into component within the IAC and the anterior aspect of the CPA
the anterior aspect of the CPA cistern (arrow). cistern. Posteriorly, this tumor has a peripherally enhancing,
multilocular cystic component that contributes tomass effect
DIFFERENTIAL DIAGNOSIS VS, CPA and/or intracana- on the middle cerebellar peduncle, pons, fourth ventricle,
licular meningioma, facial nerve schwannoma. and cerebellum. Less commonly, a lateral, intralabyrinthine
42

Case 20 43
growth pattern is observed. Very rarely, it can grow into the Reporting Responsibilities
middle ear space. Direct reporting to the referring physician is necessary.
VS arises from the Schwann cells surrounding the supe- Tumor size, extent of growth, and mass effect on adja-
rior and inferior vestibular nerves (components of CN VIII), cent critical structures are of primary importance in direct-
which course posteriorly within the IAC. It typically origi- ing treatment. Significant mass effect (on the brainstem
nates laterally within the canal in the region of Scarpa gan- and fourth ventricle) and the presence of edema may indi-
glion (which has the largest population of Schwann cells), cate impending herniation or hydrocephalus and should be
rather than from the more medial glialschwann junction urgently reported to the referring physician. When possible,
(Obersteiner-Redlich zone) as was once thought. The term the radiologist should comment on the apparent nerve of ori-
acoustic neuroma is a misnomer, as these tumors are not gin. Laterally, the fundus of the IAC and the inner ear should
neuromas, and they rarely arise from the cochlear nerve that be described in the report, and medially, the CPA cistern and
is positioned within the anteroinferior quadrant of the IAC any mass effect on the brainstem, cerebellum, and CN V
(before it coalesces medially with the superior and infe- must be reported. Cystic changes, calcifications, and intratu-
rior vestibular components of CN VIII in the CPA cistern). moral hemorrhage may influence surgical resectability.
Recall that the facial nerve is positioned in the anterosuperior
quadrant of the IAC and that a characteristic labyrinthine What the Treating Physician Needs to Know
tail of enhancement is seen with facial nerve schwannoma. Tumor size, medial to lateral extent, and related mass
As with other benign CPA lesions, tumor location and effect
size determine the clinical presentation. Mass effect on the Need for adjunctive imaging with CT, MRI without or
CN VII/VIII complex within the IAC will initially lead to with contrast
recurrent episodes of vertigo and tinnitus with the later onset Complications such as edema, mass effect, hydrocephalus,
of unilateral sensorineural hearing loss. With increasing size, and herniations if present
facial nerve dysfunction may result, and CPA mass effect
can even cause a trigeminal neuropathy, with loss of the cor- Answer
neal reflex as an early manifestation. In patients undergoing 1. When surgery is indicated, a translabyrinthine approach is
workup for unilateral sensorineural hearing loss, high-reso- reserved for those cases where significant hearing loss has
lution, non-contrast T2-weighted MRI has been proposed as already occurred, as any residual hearing will be sacri-
sufficient for screening purposes. A potential limitation with ficed. The translabyrinthine approach allows direct access
this approach may be reduced sensitivity to intralabyrinthine to the CPA cistern, IAC fundus, and facial nerve and is
pathology, inflammatory lesions, and central nervous system appropriate for tumors of all sizes. When hearing preser-
(CNS) pathology. vation is a goal, a middle cranial fossa (tumors <2 cm),
or suboccipital surgical approach is used. Radiosurgery
Question for Further Thought (e.g., Gamma Knife, Cyberknife) is being utilized with
1. Which factors influence the surgical approach? increasing frequency as initial therapy for smaller tumors.

Case
21 CLINICAL HISTORY 4-year-old male with intractable headache and visual
disturbance.
Figure 21-1
Courtesy of K. Salzman, MD. Figure 21-2
Courtesy of K. Salzman, MD.

Courtesy of K. Salzman, MD. Courtesy of K. Salzman, MD.
FINDINGS Figure 21-1. Coronal non-contrast T1WI anteriorly. There is no clear separation between the tumor
through the trigone/occipital horns. There is a large isointense and the anterior and lateral walls of the trigone suggestive
mass within the left trigone and occipital horn (star). There is of infiltration. Figure 21-4. Axial post-contrast T1WI. The
blurring of the superior and lateral margins of the left trigone mass is avidly contrast enhancing and lobulated with clearly
(arrows). Figure 21-2. Coronal T2WI through same level as defined margins. There is however possible infiltration of the
in Figure 21-1. The left lateral ventricular mass is hetero- anterolateral walls where the parenchymal vasogenic edema
geneously hyperintense with surrounding parenchymal vaso- exists. There is local mass effect.
genic edema superiorly and laterally (arrows). Figure 21-3.
Axial T2WI. There are hypointense areas within the poste- DIFFERENTIAL DIAGNOSIS Choroid plexus carcinoma
rior aspect of the mass (star) suggesting either calcifications (CPC), choroid plexus papilloma (CPP), intraventricular
or blood products. There is surrounding vasogenic edema meningioma, metastatic carcinoma.
44

Case 21 45
DIAGNOSIS Choroid plexus carcinoma (CPC) WHO III. Li-Fraumeni syndrome, rhabdoid predisposition syndrome,
and germline INI1 mutation to name a few. CPC grows rap-
DISCUSSION CPC on MRI is usually a large intraven- idly with a rather poor prognosis. It is recommended that the
tricular mass with heterogeneous intensity pattern on T2WI tumor be totally removed with gross total resection achiev-
and GRE because of the presence of calcifications and able in only 40% to 50%. There is a 5-year survival rate of
hemorrhage. It is usually isointense on T1WI, has irregular 40%. Adjuvant treatment with chemotherapy and/or radia-
lobulated margins with surrounding parenchymal brain infil- tion remains controversial.
tration, perilesional edema, and avid contrast enhancement.
Hydrocephalus is common, and distant subarachnoid space Question for Further Thought
enhancing masses indicate tumor seeding which may occur 1. Is preoperative craniospinal imaging necessary in CPC?
in about two-thirds of these patient at diagnosis. Systemic
metastases have also been reported in long survivors. These Reporting Responsibilities
features are rare in choroid plexus papilloma (CPP) or Like all tumors, direct reporting is indicated. Presence of
meningioma. Metastatic tumors tend to be multiple, but soli- hydrocephalus and CSF seeding make direct reporting more
tary lesions can occur. About 50% of CPC are found in the urgent. The location, number, size, and general geography of
lateral ventricles, 40% in the fourth ventricle, and 5% in the the lesion along with presence or otherwise of CSF dissemi-
third ventricle with the remaining 5% involving more than nation should be reported. Craniospinal MRI may be invalu-
one ventricle. Calcifications and hemorrhages impart hetero- able for exclusion of seeding and should be recommended.
geneity to the hyperdense mass on NCCT. Areas of necrosis
are hypodense on NCCT. It shows avid contrast enhance- What the Treating Physician Needs to Know
ment and infiltration of ventricular wall with surrounding Location, number if more than one, size of lesion, and
hypodense edema. It is a highly vascular tumor. presence of distant subarachnoid space contrast enhancing
CPC is a WHO III tumor with about 80% occurring in lesions
children where it constitutes between 20% and 40% of all Hydrocephalus if present
choroid plexus tumors and almost all occurring in children Need to evaluate the remainder of the central nervous sys-
between 2 and 4 years. CPC is generally more common in tem (CNS) to exclude CSF dissemination
males with a male to female ratio of 1:1 in the lateral ventri- If follow-up, presence of residual tumor or metastases
cles and 3:2 in the fourth ventricle. Because of the propensity
to obstruct the cerebrospinal fluid (CSF) pathway, symptoms Answer
and signs of hydrocephalus are the most common presenting 1. Yes. At the time of diagnosis, it is estimated that over
symptoms. Infants may present with large heads. Headache, two-thirds of the cases already have CSF dissemination
vomiting, and visual problems due to raised intracranial of tumor. It is therefore essential to obtain MRI of the cra-
pressure are common. Familiar CPC occurs in families with niospinal axis to exclude distant metastasis so as to better
germline TP53 mutations, clustering of cancer suggestive of plan how to proceed with management.

Case
22 CLINICAL HISTORY 2-year-old male liver and small bowel transplant patient
with deviated gaze and stiffness.

46

Case 22 47
FINDINGS Figures 22-1 and 22-2. Axial DWI with corre- Clinical presentations of watershed infarcts vary con-
sponding ADC map through the centrum semiovale. There siderably depending on the location and severity. Syncope,
is almost symmetrical bilateral centrum semiovale irregu- hypotension, episodic fluctuating or progressive weakness,
lar linear (rosary-like or string-of-beads pattern) restricted hemiparesis, aphasia, mental status changes, and coma are
diffusionhyperintense on DWI with hypointensity on
some of the features. Internal watershed infarcts are more
ADC map (arrows). Figures 22-3 and 22-4. Axial DWI with likely to have poorer outcome compared with external water-
corresponding FLAIR through the basal ganglia. There are shed infarcts due to the inability of internal border zones to
bilateral patchy and wedge areas of hyperintensity in bilat- form new collaterals.
eral parieto-occipital junctions and around the trigones (pos-
terior arrows), left lentiform nucleus (line arrow), right head Question for Further Thought
of caudate nucleus (right vertical arrow), and left frontal lobe 1. What is the pathophysiology of watershed infarct?
at the anterior cerebral artery (ACA) and middle cerebral
artery (MCA) junction (anterior left arrow). Reporting Responsibilities
Direct reporting is always necessary in acute and subacute
DIFFERENTIAL DIAGNOSISWatershed infarcts, sep- infarcts. Physiologic studies such as perfusion CT or MRI
tic emboli, posterior reversible encephalopathy syndrome and vascular studies such as MRA or CTA may be recom-
(PRES). mended for evaluation of pattern of ischemic changes and
vascular stenosis.
DIAGNOSIS Bilateral watershed infarcts.
DISCUSSION MRI offers the best technique to image the The extent and location of lesions may be useful in direct-
parenchymal changes of watershed infarcts. MRA may elu- ing patient management
cidate on the arterial changes. The hallmark of watershed Presence of hemorrhagic components and significant mass
infarcts (or border zones infarcts) at imaging is the junc- effect
tional location of infarcts between two neighboring vascu- MRI and CTA are useful adjuncts to the diagnostic workup
lar territories, and the restricted diffusion is consistent with Perfusion studies either CT or MR perfusion can help in
acuity. Watershed infarcts could be classified into two dis- determining the degree of hemodynamic compromise
tinct types. The external or cortical watershed zones include Cardiac evaluation is always important to exclude cardiac
cortical gray matter (GM) and underlying white matter contributing factors
(WM) between the large vascular territories of the ACA
and MCA in the paramedian frontal lobes, MCA and poste- Answer
rior cerebral artery (PCA) in the parieto-occipital junction, 1. The pathophysiology and causative mechanism of water-
and MCA and PCA in the posterior temporal parietal junc- shed infarct is complex and fraught with controversies.
tion. These are usually wedge-shaped lesions. The internal Suffice to say that two underlying factors could act alone
watershed zones include the deep GM (thalamus and basal or in combination to cause each or both external and
ganglia) and the WM of the corona radiata/centrum semi- internal watershed infarcts. These are hypoperfusion and
ovale where smaller branches of the internal carotid artery embolic phenomena. There is a consistent association
(ICA), ACA, MCA, and PCA arborize. Similar changes between hemodynamic compromise and internal border
could occur centrally in the cerebellum. These lesions con- zone infarcts, and embolic phenomenon and cortical or
stitute about 10% of all infarcts. Depending on the vulner- external watershed infarcts. Global event of cardiac ori-
ability of individual shared territories, watershed infarcts gin can produce both hypoperfusion and embolic phe-
could be unilateral or bilateral, supratentorial, and/or nomenon, while local events such as arterial stenosis,
infratentorial or may be segmental involving posterior more dissection, and thrombotic events can also produce hypo-
than anterior or vice versa. CT often shows hypodensities in perfusion and embolic phenomenon. Global hypoperfu-
these areas but acuity could be difficult to determine. Septic sion is more likely to cause widespread lesions, while
emboli or embolic phenomenon in general could resemble unilateral vascular stenosis is more likely to have unilat-
watershed infarcts, while PRES could show almost similar eral effect. The presence of internal and external water-
distribution of lesions, but restricted diffusion is usually not shed infarcts in this patient underscores the complexity of
a feature of uncomplicated PRES. the pathogenesis.

Case
23 CLINICAL HISTORY Acute onset of impairment of consciousness.
Figure 23-2
Figure 23-1
FINDINGS Figure 23-1. Axial FLAIR image through the

thalami. There is bilateral almost symmetrical medial thalamic
hyperintensity (arrows). Figures 23-2 and 23-3. Corresponding
axial DWI and ADC map, respectively, through the thalami.
The lesions restrict diffusionhyperintense on DWI with low
ADCconsistent with acute infarctions.
DIFFERENTIAL DIAGNOSISBasilar artery thrombosis,

deep cerebral venous sinus thrombosis, artery of Percheron
infarctions.
DIAGNOSIS Bilateral paramedian thalamic infarcts from

occlusion of the artery of Percheron.
DISCUSSION Acute arterial infarctions of both inferior and

medial thalami most often result from occlusion of the ros-
tral basilar artery, which also commonly affect the midbrain
and portions of the temporal and occipital lobes (supplied by
the posterior cerebral artery) or the cerebellum (supplied by
branches of the vertebrobasilar arterial system).
One of the rare anatomical variants of the posterior cir-
culation is the artery of Percheron (Table 38-1), an uncom-
mon single dominant thalamoperforating artery arising from
either of the P1 segments, which when occluded causes
Figure 23-3 characteristic bilaterally symmetric paramedian thalamic
48

Case 23 49
Table 38-1 The Vascularization of the Thalamus Is Rich, Table 38-2 There Are Four Distinct Patterns of Artery
Being Fed by Multiple Perforating Arteries of Percheron Infarctions, Which Reflect the Known
Paramedian Artery Variations
Thalamic Territories Fed By Origin In
Anterior Polar arteries PcomA Bilateral paramedian thalamic with rostral 43%
midbrain
Paramedian Thalamoperforating P1 segment
arteries of the poste- Bilateral paramedian thalamic without 38%
rior cerebral midbrain
artery (PCA) Bilateral paramedian and anterior thalamic 14%
Inferolateral Thalamogeniculate P2 segment with midbrain
arteries of the PCA Bilateral paramedian and anterior thalamic 5%
Posterior Posterior choroidal P2 segment without midbrain
arteries of the PCA From Lazzaro NA, Wright B, Castillo M, et al. Artery of Percheron
infarction: imaging patterns and clinical spectrum. Am J Neuroradiol.
From Lazzaro NA, Wright B, Castillo M, et al. Artery of Percheron 2010;31:12831289.
infarction: imaging patterns and clinical spectrum. Am J Neuroradiol.
2010;31:12831289.
infarcts, revealing hyperintense T2-FLAIR and restricted like those referred in differential diagnosis section, which
diffusion, with or without rostral midbrain involvement. would imply a different clinical approach
The midbrain involvement occurs probably when the supe- Occlusion of the artery of Percheron is generally treated
rior
mesencephalic artery shares a common origin with systemically, but occlusion of the basilar artery, even its
the paramedian thalamic artery, determining a distinctive tip, may need interventional treatment
imaging finding that is a V-shaped hyperintensity on axial
FLAIR and DWI along the pial surface of the midbrain in Answers
the interpeduncular fossa. 1. Bithalamic involvement generally causes agitation,
obtundation or coma, and also memory dysfunctions
Questions for Further Thought and various types of ocular and behavioral changes.
1. What are the clinical features at presentation and their Occlusion of the artery of Percheron presents typically
anatomical correlation? as a triad of altered mental status (variable severity),
2. Is occlusion of the artery of Percheron perceived on MRI vertical gaze palsy (mesencephalic involvement), and
as a unique pattern? amnesia (variable anterior polar territory involvement).
Depending on which parts of the midbrain are affected
Reporting Responsibilities (i.e., interpeduncular nucleus, decussation of the supe-
Direct reporting is necessary for these acute infarcts. rior cerebellar peduncles, medial part of the red nucleus,
Describe the extent of signal intensity abnormalities, nucleus of cranial nerve III, and anterior part of the
referring to their specific locations within the thalami or periaqueductal gray matter), the neurologic signs may
midbrain, documenting a defined arterial territory of the encompass other oculomotor disturbances, hemiplegia,
paramedian or anterior thalamic arterial zones. It is neces- cerebellar ataxia, and movement disorders.
sary to exclude thrombosis of the basilar artery or the deep 2. The imaging pattern of artery of Percheron infarctions
cerebral venous sinuses as these may require aggressive typically involves the bilateral median thalami with
and different treatment than isolated occlusion of the artery variable involvement of the midbrain (see Table 38-2).
of Percheron.

Exact localization of the infarct, excluding the anatomi-
cal appearance, other causes for the signal abnormalities,

Case
24 CLINICAL HISTORY Elderly patient with worsening diplopia and hypesthe-
sia on his left cheek. Physical examination showed involvement of ipsilateral
cranial nerves III, IV, and VI.
Figure 24-2
Figure 24-1
to hyperdense round lesion that expands the cavernous
sinus and enhances significantly sometimes with curvilin-
ear calcifications and bone remodeling of the sella turcica.
On MRI, the aneurysm may appear as a rounded flow void
attached to a contiguous arterial flow void and associated
pulsation artifacts propagating in the phase-encoding direc-
tion. When thrombosed, aneurysms are generally hetero-
geneous in both T1WI and T2WI, and hemoglobin and
calcifications determine other features. When CTA or MRA
are not conclusive, conventional catheter angiography is the
imaging modality of choice and may be accompanied by
endovascular treatment. The CTA is the conclusive proof
Figure 24-3
of the aneurysm in this case and differentiates it from the
other differentials.
FINDINGS Figure 24-1. Axial NCCT through the sella. Parasellar aneurysms may originate from either or both
There is a left parasellar hyperdense ovoid lesion that occu- the intracavernous and supraclinoid portions of the ICA. The
pies and bulges the left cavernous sinus (arrow). Figures 24-2 supraclinoid portion of the ICA is the most common site
and 24-3. Axial and sagittal reconstructed views, respec- (about 35%) of all intracranial aneurysms. As these aneu-
tively, from CTA in the same patient. There is an intensely rysms usually arise at the branching site or at curves in the
enhancing saccular aneurysm of the left cavernous internal supraclinoid ICA, they tend to occur at the origin of the oph-
carotid artery (ICA) (arrows). thalmic artery (upper surface), superior hypophyseal artery
(medial wall), posterior communicating artery (posterior
DIFFERENTIAL DIAGNOSIS Pituitary adenoma, menin- wall), anterior choroidal artery (posterior wall), and apex
gioma, cavernous sinus wall hemangioma, metastases, of the ICA bifurcation into the anterior and middle cere-
schwannoma, parasellar aneurysm. bral arteries. However, although intracavernous aneurysms
account for less than 3% of all intracranial aneurysms, they
DIAGNOSIS Parasellar aneurysm. produce about 25% of all cavernous sinus syndromes. When
aneurysms occupy the anterior sector of the cavernous sinus,
DISCUSSION Imaging studies show a large variation they affect the first branch of the trigeminal nerve and the
in the appearance of a parasellar aneurysm that depends third cranial nerve sometimes associated with involvement
on the presence of mural calcifications and/or thrombosis of the fourth and sixth cranial nerves. When the posterior
within the aneurysm. CT often shows a parasellar isodense sector is occupied, the aneurysm compresses the second and
50

Case 24 51
third divisions of the trigeminal nerve. Involvement of these variations. Around the sella, the exclusion of an aneurysm as
cranial nerves results in clinical presentations, which include the cause of the abnormality seen is perhaps one of the most
constant hypesthesia or hyperalgesia of the face, diplopia, important roles a radiologist can play.
and ptosis. Moreover, large aneurysms may compromise
the homolateral optic nerve causing atrophy and subsequent What the Treating Physician Needs to Know
vision loss. Exact location of the aneurysm and relationship to the par-
ent vessel
Question for Further Thought Establish the aneurysms relationship with surrounding
1. Why is the pupillary reflex first affected by compression structures
caused by aneurysms? Presence of a bleed or in this case a fistula with the cavern-
ous sinus if any
Direct reporting is essential in view of potential for an aneu- Answer
rysmal rupture which is unpredictable. Describe the aneu- 1. Contrary to ischemic causes of cranial nerve III palsies,
rysms characteristics, which include at least the origin, when compressed by aneurysms, both the superficially
shape, size, angle, direction, neck, time of filling, relation located pupillomotor fibers are first affected. Ischemic
with neighboring vessels, and characteristics of the parent neuropathies such as those seen in diabetes affect all func-
vessel, signs of rupture or complications, and anatomical tions of the nerve.

Case
25 CLINICAL HISTORY 65-year-old female with sudden collapse.
Figure 25-1
Figure 25-2
Figure 25-4
a round 1.4-cm relatively hypointense structure (transverse

arrow) indicating probable source of the hemorrhage
basilar tip aneurysm. There is dilation of the temporal horns
(vertical arrows) consistent with acute hydrocephalus.
Figure 25-2. Axial NCCT through the third ventricle. There
Figure 25-3 is hyperdensity with the third and lateral ventricles consis-
tent with intraventricular hemorrhage (IVH) (arrows). Figure
FINDINGS Figure 25-1. Axial NCCT through the supra- 25-3. CTA 3D volume-rendering coronal posterior view of
sellar cistern. There is diffuse subarachnoid space hyper- the basilar artery. There is a 1.5-cm round basilar summit
density consistent with subarachnoid hemorrhage (SAH). aneurysm pointing superiorly and tilted to the right (trans-
Posteriorly within the SAH in the suprasellar cistern, there is verse arrow). The neck measures 5 mm. The origins of the
52

Case 25 53
posterior cerebral artery (PCA) are well visualized below intracranial pressure due to hydrocephalus. Management
the aneurysm neck (vertical arrows). Figure 25-4. Coronal options are better now than previously and include both sur-
MIP CTA. This confirms the smooth outline of the aneurysm gical and endovascular choices.
(vertical arrow) and the tilt to the right with clear visualiza-
tion of the origins of the bilateral PCA below the aneurysm Question for Further Thought
neck (transverse arrows). 1. What are the risk factors for intracranial aneurysms?
DIFFERENTIAL DIAGNOSIS N/A. Reporting Responsibilities

Ruptured aneurysm with SAH is an emergency and should
DIAGNOSIS Basilar artery summit aneurysm (BAA) immediately be reported directly to the referring physician.
withSAH. Presence of other aneurysms, if visualized, should be reported.
Following CTA or MRA, appropriate measurements of the
DISCUSSION BAA forms about 5% of intracranial sac- aneurysm neck, dome to neck, maximum dimension, direc-
cular aneurysms. It presents as a relatively hypodense filling tion of projection, and surrounding structures all should be
defect posteriorly within the SAH in the suprasellar cistern categorized. Complications such as parenchymal hematoma,
in this case. Small ones may actually not be visible within hydrocephalus, cerebral vasospasm, and ischemic changes if
the SAH. BAA usually projects as outpouch from the sum- present should also be reported.
mit of the basilar artery on CTA or MRA with variable tilts
but mostly pointing superiorly. Both CTA and MRA are What the Treating Physician Needs to Know
equally effective in demonstrating BAA. An unruptured Location, number, dimensions, direction of projection of
BAA may be seen as a focal mild hyperdensity posteriorly the aneurysm, or aneurysms
in the suprasellar cistern on NCCT or a focal round signal If more than one aneurysm, it may be necessary to suggest
void on MRI. It usually contrast enhances like any other which aneurysm has ruptured
vascular structure unless it is thrombosed. BAA has been The extent of SAH, complications such as hydrocephalus,
known to embed in the hypothalamus, thalamus, and the parenchymal hematoma, IVH, infarcts, or cerebral vaso-
midbrain or project into the third ventricle. Large BAA may spasm if present on CTA or MRA
calcify, present with mass effect with surrounding edema in
the surrounding brain parenchyma. The neck could incorpo- Answer
rate the origins of the PCA. It is always important to locate 1. There are several acquired and congenital risk factors for
the posterior communicating arteries just in case treatment intracranial aneurysms. Aneurysms are more common in
leads to sacrifice of the origins of the P1. Partially throm- women aged over 50. They are more prevalent in some
bosed aneurysm tends to be irregular with laminated inten- lifestyle choices such as smoking cigarette, cocaine use,
sity pattern at MRI. When BAA ruptures, the hemorrhage possibly alcohol abuse, and oral contraceptive pill use.
is mostly within the suprasellar cistern, posterior fossa Some underlying diseases such as hypercholesterolemia
cerebrospinal fluid (CSF) spaces, and adjacent brain paren- and hypertension are suspect while there is a definite link
chyma for penetrating aneurysm. Diffusion of the SAH into with vascular wall infection and head trauma. However,
the sylvian fissures and convexity sulci via the lateral fis- the main associations are with congenital situations
sures can occur as in this case. Intraventricular extension of relating to abnormal vascular wall formation such as
the hemorrhage is always a possibility. Complications may autosomal dominant polycystic kidney disease, neurofi-
include cerebral vasospasm, ischemic changes, brain swell- bromatosis type 1 (NF1), coarctation of the aorta, Ehlers-
ing, and hydrocephalus. Danlos disease, 1-antitrypsin deficiency, cystic fibrosis,
Most BAA like most intracranial aneurysms are asymp- tuberous sclerosis, and fibromuscular dysplasia to name a
tomatic until rupture. Clinical presentations of unruptured few. Aneurysms are also common in people with family
BAA when symptomatic include headache, visual dis- history of aneurysms, familial aneurysms, and history of
turbances, focal neurologic deficits, and signs of raised prior SAH.

Case
26 CLINICAL HISTORY 15-year-old female who first developed vertigo and imbalance
with complete improvement. Six weeks later she presented with lower extremity numb-
ness and weakness, bowel and bladder dysfunction.
Figure 26-4
lesions of varying shapes and sizes abutting the callososeptal

interface (vertical arrows). There are also multiple cervical
spinal cord hyperintense lesions (transverse arrows). Figure
26-4. Parasagittal FLAIR showing multiple ovoid periven-
tricular and deep WM lesions (so-called Dawson fingers,
Figure 26-3 vertical arrows). There is an occipital subcortical lesion
(transverse arrow). Figure 26-5. Axial FLAIR through the
upper corona radiata showing multiple subcortical lesions
FINDINGS Figure 26-1. Axial MR T2WI through the lower (arrows). Multiple periventricular and deep white matter
brainstem. There are multifocal brainstem, bilateral brachium (WM) lesions are also demonstrated. Lesions are of varying
pontis, and cerebellar hyperintense lesions (arrows). Figure shapes and sizes. Figure 26-6. Axial T1WI through the cen-
26-2. Axial T2WI through the trigones of the lateral ven- trum semiovale. There are deep WM multifocal hypointense
tricles. There are bilateral peritrigonal hyperintense lesions lesions, the so-called T1-weighted black holes (transverse
(arrows). Figure 26-3. Sagittal FLAIR through the corpus arrows). Figure 26-7. Axial post-contrast T1WI through the
callosum showing multifocal corpus callosal hyperintense same level. There are multiple contrast-enhancing lesions
54

Case 26 55
DISCUSSION MS is the most common demyelinating

inflammatory disease involving the WM of the central ner-
vous system (CNS). It remains a clinical diagnosis supported
by MRI and laboratory findings. MRI is the examination of
choice for the evaluation of the suspected MS patient present-
ing with the so-called clinically isolated syndrome (CIS) in
whom MRI lesions suggestive of demyelination are found in
about 50% to 70%. Typical MRI lesions are T2 hyperintense
WM lesions of varying shapes and sizes found in the periven-
tricular, deep, and subcortical WM of the cerebrum, brain-
stem, brachium pontis, and cerebellum. Presence of lesions
in the corpus callosum (CC) and the spinal cord lends strong
support to the diagnosis of MS. CC lesions typical of MS
abut the callososeptal interface; pattern not seen in vasculitis
or sarcoidosis or Susac syndrome. Most of these lesions are
round, ovoid, flame shaped, linear, or punctate. Large lesions
more than 2 cm in size are classified as tumefactive. Smudgy
or confluent WM T2 hyperintensity is also found in the MS
brain. MR spectroscopy and DTI have shown abnormality in
the apparent normal WM in MS patients. Increasingly, deep
Figure 26-7
and cortical gray matter (GM) involvement is being reported.
GM lesions are more common in ADEM. This case illustrates
the various patterns of contrast enhancement in MS, which
includes the nodular type in about 68%; incomplete thin ring,
which is almost pathognomonic for MS in about 23%; and arc
of the incomplete ring (transverse arrow), punctate (vertical and other patterns in about 9%. Leptomeningeal e nhancement
arrow), and a small arc enhancement (line transverse arrow) is generally absent in MS but present in its mimics. Contrast
in the left centrum semiovale. enhancement is the hallmark of active/acute lesion. Up
to 80% of contrast-enhancing lesions have T1-weighted
DIFFERENTIAL DIAGNOSISMultiple sclerosis (MS), hypointensity, so-called black holes. Less than 40% of the
vasculitis, sarcoidosis, Susac syndrome, acute disseminated black holes become permanent. The number of black holes
encephalomyelitis (ADEM). correlates well with the degree of d isability. Steroid treatment
may modify the pattern of contrast enhancement. Contrast
DIAGNOSIS Multiple sclerosis (MS). enhancement usually resolves within 6 weeks.

56 Case 26
MS is a disease of the temperate climate and is more com- What the Treating Physician Needs to Know
mon in women than men. Pediatric involvement is present in Location, number, pattern, and contrast enhancement of
about 5% of MS population. Its presentation or exacerbation lesions on initial MRI
may include features such as optic neuritis in as many as Presence of new lesions, both contrast and non contrast
50%, transverse myelitis, brainstem symptoms such as ver- enhancing, and their location on follow-up studies
tigo, diplopia, imbalance, Bells palsy, and less frequently Pattern of volume loss and presence of black holes on
focal neurologic deficit. These lesions may wax and wane. T1WI
Supportive laboratory findings include cerebrospinal fluid Criteria for diagnosis of MS keep changing; hence, a full
(CSF) and vestibular evoked potential (VEP) findings. CSF description of positive and negative findings is always
studies in this patient revealed no pleocytosis or abnormal helpful to the referring physician
protein but an increase in IgG index and synthesis and posi-
tive oligoclonal bands. The presence of oligoclonal bands in
the CSF is not diagnostic of MS but supportive of demyelin- Answer
ation. A comprehensive blood workup was done to rule out 1. MS is characterized clinically into four categories.
MS mimics in this patient. Relapsingremitting MS (RRMS) is characterized by
relapses and remissions and more common in women
Question for Further Thought than men (3:1). Secondary progressive MS (SPMS) is
1. What are the various types of MS? characterized by secondary slow progression of RRMS
with and without superimposed relapses. Primary pro-
Reporting Responsibilities gressive MS (PPMS) is characterized by at least 1
Routine reporting is sufficient. An ideal MRI report for MS year of progression of spastic mono- or paraparesis or
should contain the number of lesions unless they are too ataxia. Progressive relapsing MS (PRMS) is character-
numerous to count, the size of the lesions, the shape of the ized by at least 1 year of progression of the neurologic
lesions, the borders of the lesions, and the pattern of enhance- deficit with superimposed relapses. The diagnosis of
ment and presence or absence of leptomeningeal enhance- relapsing type of MS is done by fulfilling the criteria of
ment. Periventricular lesions are to be reserved for the dissemination in time (DIT) and dissemination in space
ones clearly abutting the ventricles. Location of the infraten- (DIS). The presence of enhancing and non-enhancing
torial lesions should be described. Presence of black holes lesions on the same MRI is enough to fulfill the crite-
or at least a description that the T2 lesions are associated ria for DIT. The presence of at least one lesion in two
with T1 hypointensities should be acknowledged. The term of the four typical MRI locations for demyelination
such as nonspecific should be avoided in the context of is enough to fulfill the criterion of DIS. These typical
clear abnormalities in the spinal cord. The degree of v olume locations are periventricular, juxtacortical, infratento-
loss should be reported. rial, and spinal cord.

Case
27 CLINICAL HISTORY 55-year-old female with headache.
FINDINGS Figure 27-1. Axial NCCT through the sella tur- or thrombus, and can be peripherally calcified, which is a
cica. There is a well-circumscribed homogeneously hyper- very characteristic and helpful finding. On MRI, aneurysms
dense mass in the right parasellar region (arrow). Figure 27-2. can be homogeneously enhancing with signal voids on
Axial T2WI through the sella. The mass is conspicuously T1WI and T2WI, reflecting a patent lumen. Alternately, the
hypointense. Figure 27-3. Axial post-contrast T1WI. There is aneurysm sac may be partially thrombosed, as in this case,
only a peripheral rim of enhancement (arrow). Figure 27-4. showing variable T1 and T2 intensity patterns, and partial or
DSA PA right internal carotid injection. The mass opacifies rim enhancement. Dedicated vascular imaging, either CTA/
with contrast and arises from the cavernous segment of the MRA or conventional angiogram, will confirm the diagnosis
right internal carotid artery (ICA) (arrow). by showing contiguity of the lesion with the adjacent ICA
lumen.
DIFFERENTIAL DIAGNOSIS Aneurysm, cavernous sinus
thrombosis, meningioma, metastasis. Question for Further Thought
1. Why does the aneurysm in this case appear to fill solidly
DIAGNOSIS Cavernous ICA aneurysm, partially throm- with contrast on conventional angiogram, while on MRI
bosed. it shows only thin peripheral enhancement?
DISCUSSION ICA aneurysm is an important differential Reporting Responsibilities

consideration for any sella/parasellar mass. On CT, aneu- All aneurysms deserve direct reporting. It is difficult to
rysms are often hyperdense, reflecting either flowing blood predict when they will rupture. Maintain a high level of
57

58 Case 27
suspicion for aneurysm whenever a sella/parasellar mass- CTA/MRA or conventional angiography are confirmatory
like lesion is found. Describe the cross-sectional size, loca- Size with neck and dome-to-neck measurements
tion, and caliber of the neck of the aneurysm if possible. For Presence of complications such as hemorrhage, spasm,
paraclinoid aneurysms, attempt to distinguish between intra- and mass effect
dural (distal to the optic strut) and extradural (proximal to the
optic strut) locations. Intradural aneurysms are by definition Answer
subarachnoid and more prone to spontaneous rupture than 1. Conventional angiography (DSA) suffers from the draw-
are extradural aneurysms. backs inherent to all planar or 2D forms of imaging in that
depth information is lost and that in-line structures are
What the Treating Physician Needs to Know superimposed on the final image. In this case, therefore, a
Aneurysms of the parasellar region are not uncommon and thin layer of contrast migrating around the margins of the
can usually be diagnosed with moderate certainty on stan- thrombus in spherical fashion is radiologically indistin-
dard cross-sectional imaging guishable from a solidly filling patent aneurysm.

Case
28 CLINICAL HISTORY 33-year-old HIV-positive patient presenting with a
2-day history of fevers, altered mental state, and meningismus.
59

60 Case 28
FINDINGS Figure 28-1. Axial FLAIR through the basal gan- arteries but may also be seen in periventricular white mat-
glia. There is bilateral symmetrical basal ganglia hyperinten- ter (WM), thalamus, midbrain, and medial cerebellum. MRI
sity (arrows). Mild hydrocephalus is present along with other is the modality of choice in the investigation of the patient
parenchymal lesions. Figure 28-2. Corresponding post-contrast with suspected cryptococcosis. In the perivascular spaces
T1WI. There are nodular and ring enhancement in the basal the fungus forms cyst-like conglomerates filled with mucoid
ganglia bilaterally (arrows) with smaller ones elsewhere in the gelatinous material produced by the capsule of the organ-
cerebral hemispheres. Figure 28-3. In the same patient, axial isms which tend to restrict diffusion and are hyperintense
T2WI through the posterior fossa shows dilated and hyperin- on T2WI. Their location around the basal ganglia is sugges-
tense perivascular spaces in the region of the dentate nuclei tive of cryptococcosis. Enhancement of the cysts as well as
(arrows) of the cerebellum, suggesting cryptococcal involve- leptomeningeal enhancement is seen in immunocompetent
ment. Figure 28-4. Parasagittal post-contrast T1WI in a dif- patients who are able to produce an immunologic reaction
ferent patient shows multiple brain parenchyma-enhancing or in cryptococcocal-IRIS (immune reconstitution inflam-
nodules (including the cerebellum) and thick leptomeningeal matory syndrome)-related disease. Involvement of the brain
enhancement (arrows). Figure 28-5. Axial post-contrast T1WI parenchyma is characterized by areas of high T2/FLAIR
(companion patient) through the centrum semiovale shows thin signal, restricted diffusion, and parenchymal enhancement
leptomeningeal enhancement (arrows). Figure 28-6. Axial post- the so-called cryptococcomas which are more common in
contrast T1WI shows ependymal enhancement (arrows) in the patients with normal or near-normal immune system.
trigones of the lateral ventricles and a nodular lesion in the head In the HIV-positive patients cryptococcal infection is
of the right caudate nucleus (vertical arrow). frequently seen in association with IRIS and can result in
higher mortality rate. Leptomeningeal enhancement can be
DIFFERENTIAL DIAGNOSISTuberculosis, cryptococ- associated with communicating hydrocephalus. In instances
cosis, toxoplasmosis, pyogenic abscesses, and lymphoma. of cryptococcal-IRIS, patients seem to have higher organism
burden in the CSF which might be associated with elevated
DIAGNOSIS Cryptococcosis. intracranial pressure and hydrocephalus by either increased
production of mucoid material or greater reactivity to cryp-
DISCUSSION The infection often starts as meningitis tococcal antigens. Ventriculitis can also occur leading to
(along the base of the skull), although parenchymal involve- contrast enhancement of the walls of the ventricles and even
ment is also found, such as cryptococcomas. Enlarged involvement of the choroid plexus. Recent studies with DTI
Virchow-Robin spaces occur mainly around lenticulostriate depict WM microstructural changes apparently not seen by

Case 28 61
routine MRI although prospective studies are still needed. Reporting Responsibilities
Perilesional edema is common with TB and toxoplasmosis. This is an emergency deserving of direct reporting. The
Lymphoma tends to be isointense to hypointense on T2WI extent of disease, presence of raised intracranial pressure,
with homogeneous contrast enhancement in the immuno- and hydrocephalus should be mentioned. Recognition of the
competent. Ring enhancement is common in the immuno- pattern of the cysts in the basal ganglia, contrast enhance-
compromised. Pyogenic abscesses are round with smooth ment, raising the possibility of cryptococcal-IRIS in immu-
ring enhancement and large perilesional edema. nocompromised patients is important.
Cryptococcus neoformans is a fungus, found in mammal
and bird feces, transmitted to humans through the respira- What the Treating Physician Needs to Know
tory route and may infect the human central nervous sys- Specific features of cryptococcosis to support the diagnosis
tem (CNS). It is infrequently seen in immunocompetent Presence of hydrocephalus and raised intracranial pressure
individuals and occurs predominantly in immunocompro-
mised patients most often HIV-positive individuals. Most Answers
common symptom is headache, usually associated with 1. Lumbar puncture (LP) shows increased cerebrospinal
meningeal signs, confusion, seizures, blurred vision, and fluid (CSF) pressure and mild-to-moderate leukocytosis,
sometimes focal neurologic deficits, mild fever, and nuchal decreased glucose, and elevated protein levels. The India ink
rigidity. test is specific, demonstrating the fungus in CSF. The level
of antigen titer correlates with the severity of the disease.
Questions for Further Thought 2. Hydrocephalus is one of the factors contributing to the
1. Which other complementary investigations are important high mortality of this disease usually treated with repeated
for the diagnosis? LP or emergent ventriculostomy. Concomitant combined
2. How is the hydrocephalus associated with cryptococcosis therapy with antifungal medications is necessary and also
managed? HARRT when appropriate.

Case
29 CLINICAL HISTORY 8-year-old male with malignant astrocytoma of the corpus
callosum treated with combination therapy.
Figure29-1 Figure29-2
62

Case 29 63
familiar with the effects of these on images. Some of these

may mimic abnormalities, some are abnormalities, and some
are just life support instruments casting artifacts. Surgical
intervention usually starts with a craniotomy (a cranial vault
bone flap raised to allow entry and replaced at the end of
the intervention) or craniectomy (a bone flap raised and not
replaced or may be replaced at a later date). The margins of
these flaps are seen as defects in the cranial vault. The crani-
ectomy could produce a sunken effect on the scalp. Because
of foreign bodies at the margins of the craniotomy, it is eas-
ier to identify on the GRE sequence where the susceptibility
effect is greatest. These margins are usually easy to see on
CT bone windows.
If there is hydrocephalus or one is expected, a ventricu-
lar drain or a VP shunt may be placed. The drain or shunt
should pass through the lateral ventricle for it to function
properly. Shunt complications may include malfunction such
as overdrainage or inadequate drainage which could result in
Figure29-5 slit ventricles or hydrocephalus, respectively, shunt infection
with ventriculitis and ependymal contrast enhancement with
intraventricular debris, extraaxial collections particularly
FINDINGS Figure 29-1. Axial treatment planning post-con- with overdrainage and intraventricular hematoma (IVH) dur-
trast T1WI through the lateral ventricles. There is a large ing insertion or revision of the shunt. The valve of the shunt
partially cystic and solid mass (WHO IV) astrocytoma (star) may produce signal void artifacts peripherally on the cranial
of the corpus callosum bulging into the lateral ventricles. vault particularly on the GRE and DWI, and sulcal hyperin-
Figure29-2. Axial NCCT through the corona radiata about tensities on FLAIR images.
2years into his treatment which included surgical removal of Confluent WM T2 hyperintensity or leukoencephalopa-
the tumor via bilateral parietal craniotomies, radiation treat- thy is a fairly common complication of radiation treatment
ment, and chemotherapy. There are bilateral ventriculoperito- and chemotherapy. In the acute phase, this probably relates
neal (VP) shunts (transverse arrows), multifocal subcortical to edema, while on the long term, there are demyelination,
calcifications (vertical arrows), and bilateral periventricular axonal loss, spongiosis, gliosis, and vasculopathy. Decreased
smudgy white matter (WM) hypodensities (stars). Figure N-acetyl aspartate (NAA) has been demonstrated on MR
29-3. Axial FLAIR through the corona radiata. There is bilat- spectroscopy within the WM hyperintensity. Brain necro-
eral confluent WM hyperintensity (stars). The left parasagit- sis presenting with contrast enhancement could mimic neo-
tal parietal surgical cavity (vertical arrow) represents part of plasm. MRI perfusion study may be useful for distinguishing
the surgical tract to the original tumor. There are hyperinten- radiation necrosis (low to poor relative Cerebral Blood
sities in the left parietal sulci (transverse arrow) produced by Volume [rCBV]) from tumor (high rCBV). Brain volume
the VP shunt susceptibility artifact. There is thickening of the loss with widening of the sulci and enlargement of the ven-
dura (chevrons). The craniotomy flap is present posteriorly tricles is not uncommon following treatment. Punctate GRE
(line arrows). Figure 29-4. Axial GRE through the centrum hypointensities within the WM may represent calcifications,
semiovale. There are bilateral large signal void artifacts from microhemorrhage, or telangiectasia. CT usually differenti-
the VP shunts (stars). There are multiple punctate hypointen- ates calcifications (dense on CT) from microhemorrhages
sities in bilateral centrum semiovale WM (transverse arrows) and telangiectasia (usually not seen on CT). Mineralizing
consistent with telangiectasia or microhemorrhages or calci- angiopathy resulting in calcifications is a common compli-
fications. Figure 29-5. Coronal post-contrast T1WI through cation of tumor treatment. All these changes begin as early
the frontal lobes. There is smooth pachymeningeal enhance- as within 3 months of completion of treatment. Mild pachy-
ment (arrows) which is circumferential around the cerebral meningeal enhancement may be seen following surgery, and
and cerebellar hemispheres. this could disappear subsequently. Avid and robust pachy-
meningeal enhancement may suggest intracranial hypoten-
DIFFERENTIAL DIAGNOSIS N/A. sion particularly if there is associated hind brain sagging or
pseudo Chiari I. Nodular pachymeningeal configuration may
DIAGNOSIS Treatment-related changes. indicate tumor seeding. Some of these changes may or may
not be symptomatic.
DISCUSSION This case illustrates some of the changes
that can occur following intervention in the management Question for Further Thought
of intracranial pathology. Multimodal interventions happen 1. Which is the best imaging technique for the evaluation of
very frequently in clinical management, and we have to be treatment changes?

64 Case 29
Reporting Responsibilities Answer

Significant changes following brain tumor treatment should 1. While CT may be able to demonstrate all the changes
be reported directly. Complications of shunts usually require discussed here, MRI offers the best imaging technique
immediate attention. New tumors or new areas of contrast to evaluate posttreatment changes. If the consideration
enhancement usually require further evaluation with DWI if is whether a shunt or EVD is functioning or not, CT is
one has not been obtained, perfusion studies, and MR spec- adequate. Parenchymal changes, however, are best evalu-
troscopy. ated by MRI particularly with other available procedures
such as MR spectroscopy, perfusion, and the multiplanar
What the Treating Physician Needs to Know capability of MRI.
What is new? Where and how severe?
Are life support tubes doing what they should be doing?
What other investigations should be done to clarify the
new findings?

Case
30 CLINICAL HISTORY 10-year-old male, history withheld.
FINDINGS Figures 30-1 and 2. Axial and coronal T2WI one encounters multiple bilateral lesions in the brachium
through the brachium pontis, respectively. There is hyperinten- pontis, one can only offer a differential, and a fairly lengthy
sity in the bilateral middle cerebellar peduncles (brachium pontis) one at that.
(arrows). The pons is also significantly abnormal as well.
DIFFERENTIAL DIAGNOSIS Demyelinating disease, neu- 1. Histologically, what are the WM lesions of neurofibroma-
rodegenerative disease, viral encephalitis, tumor, small vessel tosis type 1?
ischemia, phakomatosis.
DIAGNOSIS Phakomatosis (neurofibromatosis type 1). Direct reporting is necessary in view of the long list of dif-
ferentials, some of which may require urgent treatment.
DISCUSSION The brachium pontis or middle cerebellar Describe the distribution of lesions and make an attempt to
peduncles are thick arms of white matter (WM) pathways identify any other distinguishing features elsewhere in the
connecting the cerebellum to the pons and to the rest of brain which might narrow the differential to a more manage-
the brain. As predominantly white matter structures, they able length.
are susceptible to any and all lesions known to affect the
WM of the brain. This includes demyelinating disease (mul- What the Treating Physician Needs to Know
tiple sclerosis [MS], acute disseminated encephalomyeli- The differential diagnosis for bilateral brachium pontis
tis [ADEM], progressive multifocal leukoencephalopathy lesions is very large and, with few exceptions, cannot be
[PML]), infiltrating tumor, metastases, ischemic change, much reduced by imaging features alone
infection, the phakomatoses, toxic and metabolic dis-
ease including osmotic demyelination. In addition, certain Answer
degenerative conditions specific to this region such as olivo- 1. The WM lesions of neurofibromatosis, sometimes referred
ponto-cerebellar atrophy may be seen. A few of these condi- to as unidentified bright objects (UBOs), probably rep-
tions do have some more characteristic findings which may resent some combination of disordered, hamartomatous
help in narrowing the differential. Largely, though, when cells, and areas of vacuolization.
65

Case
31 CLINICAL HISTORY 59-year-old male with decreased level of consciousness,
weight loss, persistent low-grade fever, and fatigue.
66

Case 31 67
FINDINGS Figures 31-1 and 31-2. Axial DWI with cor- i nfections and fungal lesions as well as primary or metastatic
responding ADC map. There is ring-restricted diffusion in neoplasms from which it could be difficult to differentiate.
the splenium of the corpus callosum (CC) (arrows). There Clinical presentation is usually nonspecific; mental sta-
is surrounding increased diffusion consistent with vaso- tus changes, fever, neurologic deficit, and weight loss.
genic edema. Figure 31-3. Axial FLAIR through the sple- CSF evaluation usually shows elevated white cells with
nium of the CC. There is smudgy hyperintensity through lymphocytic predominance, high protein, and low glu-
the splenium of the CC surrounding a hyperintense ring cose. Initial bronchoalveolar lavage stain was negative for
with hypointense core (vertical arrow). There is an addi- AFB in this patient. Diagnosis was made by brain biopsy.
tional ring hyperintensity in the adjacent left occipital lobe Bronchoalveolar lavage culture was positive for Acid Fast
with a hypointense core (posterior transverse arrow) and in Bacilli (AFB) after 6 weeks. The patient improved on qua-
the parafalcine left frontal lobe (anterior transverse arrow). druple antituberculous regimen.
Figure 31-4. Axial post-contrast T1WI through the sple-
nium just superior to Figure 31-3. There are two adjacent Questions for Further Thought
ring contrast enhancing lesions in the splenium on the left 1. Is TB still common in the US?
with hypo/isointense core (arrow) and other ring-enhancing 2. For how long has TB been known?
cortical lesions elsewhere in the brain (transverse arrows). 3. What is the great white plague?
These also have isointense core.
DIFFERENTIAL DIAGNOSIS Tuberculomas (TBs), metas Direct reporting is essential in view of the serious differen-
tasis, pyogenic abscess, fungal infection, neurocysticercosis. tials needing urgent attention.
DIAGNOSIS CC TB. What the Treating Physician Needs to Know

Location and number of lesions in the CC and elsewhere
DISCUSSION The typical feature of TB, the so-called tar- in the brain
get sign, comprises central calcification with a ring-like area Neurotuberculosis protean manifestations make the dis-
of enhancement after injection of contrast. Neurotuberculosis ease a great mimicker similar to neurosyphilis, neurosar-
presents as meningitis, granulomata, tuberculous abscess, or coidosis, and neuroborreliosis (Lyme disease)
spinal tuberculous arachnoiditis. Vasculitic changes have Isolated butterfly TB of the CC has been mistaken for
been reported resulting in infarcts although they are more malignant gliomas, and the diagnosis of CC TB was made
commonly found at autopsy. Parenchymal TBs are usually by biopsy
located in the frontoparietal lobes, in the basal ganglia, and
rarely in the CC. When they are present in the CC, they are Answers
usually part of a more widespread parenchymal involve- 1. TB resurgence peak in the United States in 1992. The
ment. TBs may be noncaseating, caseating with a solid cen- number of TB cases reported annually has decreased.
ter or caseating with a necrotic center. Noncaseating TBs According to CDC statistics, two-thirds of the TB cases
are usually hypointense on T1WI and hyperintense on T2WI occur in foreign-born persons. The incidence is highest
with homogeneous enhancement. Caseating TB with a solid in the Asian and lowest in the white population. Other at-
center has a relative hypointense or isointense core with sur- risk populations include the homeless, IV drug users, and
rounding hyperintense rim or ring on FLAIR and T2WI with persons with AIDS.
a rim/ring contrast enhancement. Necrotic caseating lesions 2. TB has been recognized since 1500 bc. Hippocrates pro-
have hyperintense core with hypointense rim on T2WI with vided the first clinical description of TB albeit he thought
rim enhancement. The rim diffusion restriction shown in it was hereditary. Aristotle believed correctly however
Figure 31-2 does not conform to a pyogenic abscess where that TB was contagious.
the core usually restricts diffusion. The core of an abscess 3. The great white plague refers to the TB epidemic in
is also usually hyperintense on FLAIR and T2WI. The dif- Europe that started in the 17th century and lasted for
ferential diagnosis for TB includes other granulomatous 200 years!

Case 32 CLINICAL HISTORY 68-year-old male undergoing restaging for non-small cell
carcinoma of the lung.
68

Case 32 69
FINDINGS Figures 32-1 to 32-3. Axial T2WI, non-contrast The most common metastasis to the choroid plexus is
T1WI, and post-contrast T1WI through the lateral ventricles, from renal cell carcinoma. Tumors of origin have included
respectively. There is a small left lateral ventricle round T1 lung carcinoma, melanoma, and colon carcinoma. Metastases
and T2 isointense (to white matter [WM]) avidly contrast- are seen most commonly in adults, although they have also
enhancing choroid plexus mass (arrows). There is also a been found in children with extracranial childhood tumors.
punctate metastasis in the right parasagittal parietal lobe When they form part of multifocal brain metastases, the pre-
(vertical arrow in Figure 32-3). Numerous new punctate sentation could be mute. Headache or symptoms and signs
contrast-enhancing metastases are present in other areas of of hydrocephalus are present in obstructive lesions. Visual
the brain (not shown). Figure 32-4. Axial post-contrast T1WI symptoms may arise as a result of periventricular invasion
through same level 7 months before. There is no significant and edema. Treatment includes surgical removal, radiation
mass in the choroid plexus. treatment, or chemotherapy. Isolated choroid plexus metasta-
ses have been known to be indolent for a long period.
DIFFERENTIAL DIAGNOSIS Intraventricular meningi-
oma, colloid cyst, xanthogranuloma, choroid plexus metastasis. Question for Further Thought
1. What are the common metastatic tumors to the brain?
DIAGNOSIS Metastasis non-small cell lung carcinoma.
DISCUSSION Metastasis to the choroid plexus is rare. It
Direct reporting is essential. Location, number, other paren-
could be isolated but most often associated with multifo-
chymal metastases, hydrocephalus or hemorrhagic, and/or
cal metastases to the brain. It is more common in the lateral
periventricular complications are all worth reporting.
ventricular trigones than elsewhere. It usually presents as
an avid contrast-enhancing mass of varying sizes on CT or
MRI. Signal pattern is variable on T1WI and T2WI. NCCT
often shows a hypodense to isodense mass. It may be compli- Location, number, and complications
cated by hemorrhage and hydrocephalus. Presence of hem- Is it safe to perform lumbar puncture for CSF sampling?
orrhage may alter the density pattern on CT and intensity
pattern on MRI. Third ventricular choroid plexus metastases Answer
have been reported to mimic a colloid cyst except that the 1. The most common sources of metastases to the choroid
metastases showed vivid contrast enhancement. Ependymal plexus are kidney, lung, and colon. There are a few reports
contrast enhancement has been reported with choroid plexus of thyroid, melanoma, stomach, breast, bladder, and lym-
metastases indicating ependymal spread. Ventricular wall phoma. The sources in children are leukemia, lymphoma,
invasion with peritumoral edema has also been reported and sarcoma. 11% of brain metastases have no known ori-
mimicking intraventricular meningioma, carcinoma, or xan- gins at the time of diagnosis. Despite the very high blood
thogranuloma. Choroid plexus metastasis could be difficult flow to the choroid plexus, it is very rareto find choroid
to diagnose when they are small, mimicking normal contrast- plexus metastases accounting for less than 5% of intra-
enhancing choroid plexus. Comparison with prior study and cranial metastases in autopsy series and less than 1% of
index of suspicion helps in catching this very difficult lesion. clinically evident cerebral metastases.

Case
33 CLINICAL HISTORY Chronic alcoholic patient presenting with a subacute but
progressive encephalopathy and ophthalmoplegia.
the hypothalamus (arrows) and dorsal midbrain. Figure 33-3.

Axial FLAIR through the midbrain. There is periaqueductal
hyperintensity (arrow).
DIFFERENTIAL DIAGNOSISTop of the basilar syn-

drome, deep venous system thrombosis, neuromyelitis
optica, viral encephalitis, Wernicke encephalopathy (WE),
acute disseminated encephalomyelitis, Creutzfeldt-Jacob
disease, mitochondrial disorder, and toxic-related changes
(metronidazole).
DIAGNOSIS Wernicke encephalopathy (WE).
DISCUSSION There are some typical MRI findings in

WE, crucial for early diagnosis. Usually there is a sym-
metric hyperintense signal on T2WI in the medial thalami,
periventricular area of third ventricle, mammillary bodies,
tectal plate, periaqueductal region, and dorsal medulla. Some
lesions may enhance. Atypical findings are described more
frequently in nonalcoholic patients and include symmetric
T2-weighted hyperintensity in the cerebellum, cerebral cor-
tex, and cranial nerves nuclei.
Figure 33-3 WE is a clinical neurologic entity characterized by con-
sciousness impairment, ocular dysfunction, and ataxia
FINDINGS Figure 33-1. Axial FLAIR through the third ven- caused by thiamine deficiency (vitamin B1). If left untreated,
tricle. There is bilateral symmetrical hyperintensity (arrows) it may evolve to Korsakoff psychosis or even death; thus it
along the walls of the third ventricle. Figure 33-2. Axial is considered a medical emergency requiring prompt treat-
FLAIR slightly below Figure 33-1. There is hyperintensity in ment (intravenous thiamine). It is frequently associated with
70

Case 33 71
chronic alcohol abuse due to malnutrition, but in 50% of What the Treating Physician Needs to Know
cases it can occur in nonalcoholic patients (due to imbal- An MRI is needed in an emergency setting to correlate
anced diet, gastrointestinal surgery, prolonged vomiting, with the patients neurologic status and laboratory results
chemotherapy, and systemic diseases). to contribute to an accurate and early diagnosis
Questions for Further Thought Answers

1. What is the pathophysiology of thiamine deficiency? 1. Thiamine is a coenzyme found in food that is involved
2. Are there any imaging features that distinguish WE in in carbohydrate metabolism and functions as an osmotic
alcoholic from nonalcoholic patients? gradient regulator. Therefore, its deficiency causes cellu-
lar swelling and bloodbrain barrier disruption.
Reporting Responsibilities 2. Large studies reveal enhancement of medial thalami
Direct reporting is important as WE is considered a medi- and mammillary bodies to be more frequent in alcoholic
cal emergency. Verify imaging findings that document WE patients, whereas involvement of infratentorial struc-
having in mind the typical and atypical findings described tures (atypical findings) is more frequent in nonalcoholic
in the literature. patients.

Case
34 CLINICAL HISTORY 71-year-old male with a history of nausea, vertigo, veer-
ing to the right, and right Horner syndrome.
FINDINGS Figures 34-1 and 34-2. Axial DWI through the (of Wallenberg). DWI usually in most cases reveals a dor-
medulla. There is a right posterolateral medulla small area solateral medullary hyperintensity or restricted diffusion
of restricted diffusion (arrows). Figure 34-3. Axial DWI which could extend into the adjacent restiform body (inferior
through the restiform body. There is focal restricted diffusion cerebellar peduncle). Other associated lesions may include
in the right restiform body (anterior arrow) with punctate pontine, brachium pontis, and cerebellar small or patchy
areas of restricted diffusion in the right cerebellum (poste- infarcts. The small infarcts may be very faint hyperintensity
rior arrow). Figure 34-4. Axial T2WI through the medulla. on FLAIR and T2WI. Vascular studies such as MRA, CTA,
There is a small right lateral medullary hyperintensity (trans- or DSA demonstrate more often vertebral artery (67%) rather
verse arrow). Signal void is missing within the right vertebral than isolated posterior inferior cerebellar artery (PICA) (10%)
artery (vertical arrow). Figure 34-5. Contrast-enhanced MRA occlusion or stenosis. Presence of vascular lesions may be
of the neck. There is tapered occlusion of the right distal ver- useful in excluding the other differential diagnoses. Glioma
tebral artery (V2) consistent with dissection. is usually mass-like and may not necessarily restrict diffusion.
LMI results in the lateral medullary syndrome or
DIFFERENTIAL DIAGNOSIS Demyelination, lateral medul Wallenberg syndrome described in 1895 by the man whose
lary acute infarct, glioma. name it bears. The history is usually of a sudden onset
of symptoms in most cases with a small percentage with
DIAGNOSIS Right lateral medullary infarct (LMI). insidious onset. It is more common in males than females
with age range from the third decade to the ninth. The
DISCUSSION CT is often not effective in the evaluation underlying pathology is an infarction of the lateral medulla
of LMI. The small size of the infarct coupled with beam- which could be due to atherothrombosis, embolus, small
hardening artifact render most lesions invisible on CT. MRI vessel disease, or dissection. Risk factors include hyper-
particularly DWI is the modality of choice when a patient tension, diabetes mellitus, current cigarette smoking, and
present with the acute classical lateral medullary syndrome atrial fibrillation. The triad of ipsilateral Horner syndrome
72

Case 34 73
(meiosis, ptosis, and anhydriosis), ipsilateral ataxia, and

contralateral hypalgesia is virtually diagnostic of LMI.
Other presentations include vertigo, headache, dysphagia,
dysarthria, hoarseness, hiccups, nystagmus, facial paraly-
sis, and numbness and visual disturbance among others.
Prognosis is generally good with very few deaths reported
in the acute phase.

1. What fibers are disrupted by LMI?
Direct reporting is essential in any case of acute infarct. The
angiographic findings may affect how the patient is managed.

Extent and locations of the infarcts
Associated vascular abnormality. In this case dissection
but embolic and thrombotic causes are possible
Presence of hemorrhage
Answer
1. There is disruption of the spinothalamic tract. The conse-
quence of this is loss of pain and temperature sensation to
the opposite side of the body. The damage to the cerebel-
lum or the restiform body (inferior cerebellar peduncle)
can cause ataxia. There is also damage to the hypothala-
mospinal fibers which disrupts the sympathetic nervous
Figure 34-5 system relay causing Horner syndrome.

Case
35 CLINICAL HISTORY 13-year-old female with dystonia and contractions of the
right hand.
FINDINGS Figure 35-1. NCCT through the thalamus. mass effect. Figure35-2. Axial T2WI through the thalamus.
There is a heterogeneous mass in the left thalamus (verti- There is a collection of tubular signal voids in the left thala-
cal arrow) with tubular hyperdensities extending into a large mus (vertical arrow) extending into a large tortuous signal
tortuous midline hyperdense tubular structureenlarged void in the midline consistent with a large internal cerebral
internal cerebral vein (transverse arrow). There is local vein and great vein of Galen (transverse arrows). Areas of
74

Case 35 75
hyperintensity are seen surrounding the tubular signal voids DSA confirmed in this case that most of the afferent supply
in the left thalamus. These areas possibly represent gliosis. was from the left thalamoperforators. Strategically placed
Figure35-3. Axial 3D TOF MRA source image through the AVM could produce mass effect and hydrocephalus. The
thalamus. There is a collection of serpentine tubular struc- presence of a parenchymal nidus indicates an AVM draining
tures in the left thalamus. Large tortuous internal cerebral into the vein of Galen rather than VGAM.
vein and vein of Galen are visualized (arrows). Figure 35-4. AVMs are rare and considered congenital but de novo
Volume-rendered 3D TOF MRA best projection. There is a AVMs are reported. They have been described in all age
left thalamic tangle of vessels (the nidus) with many large groups. Common presentations include headache, numbness,
feeding arteries from the enlarged left P-Com artery and the neurologic deficit, intracranial hemorrhages (subarachnoid
left posterior cerebral artery (PCA) (arrows). hemorrhage [SAH], intracranial hemorrhage [ICH], and intra-
ventricular hemorrhage [IVH]), mental status changes, and
DIFFERENTIAL DIAGNOSIS Arteriovenous malformation coma. The risk of hemorrhage depends on the hemodynamic
(AVM), vein of Galen aneurysmal malformation (VGAM). of the AVM, its location, and the presence of prior hemor-
rhage. This risk is estimated at 1% to 4% per year. In rare sit-
DIAGNOSIS Left thalamic AVM. uations, AVMs could thrombose spontaneously. Multimodal
treatment is usually advocated. This includes endovascular
DISCUSSION Both MRI and CT are complementary in treatment, radiosurgery, and surgical intervention.
the evaluation of AVM. MRI, however, presents compre-
hensive topographical and parenchymal details unmatched Question for Further Thought
by CT. CT, however, is better at distinguishing between 1. What are the complications of AVM?
calcifications and hemorrhage. CTA and MRA are equally
effective in evaluating the afferent, nidus, and efferent vas-
cular patterns of the AVM. The classic AVM has afferent
Direct reporting is necessary in this circumstance as it is
vessels or arteries that are usually larger than other arter-
unpredictable when AVM will rupture. Location, number if
ies in the vicinity. There is a nidus that contains a tangle of
more than one, origin and number of feeders, number and ter-
small vessels that are hyperdense on CT and show signal
mination of efferent veins, associated parenchymal changes
voids on T2WI, some of which could dilate into aneurys-
including new or old hemorrhage, gliosis, volume loss, mass
mal proportion. T2 hyperintense areas present within the
effect, calcifications, and hydrocephalus should be reported.
nidus probably represent gliosis. Calcifications and old and
new hemorrhages could be present. Calcification and acute
hemorrhage are usually hyperdense on CT but could be dif- What the Treating Physician Needs to Know
ferentiated by measuring the Hounsfield unit. Both may be Location, size, and number if more than one
hypointense on T2WI and GRE. Surrounding volume loss Pattern of afferent and efferent vessels regarding origins,
due to steal phenomenon or edema due to recent bleed could number, stenosis, and aneurysms
be visible. The draining or efferent veins are also usually Evidence of prior hemorrhage or new hemorrhage
larger than other veins in the vicinity. The afferent and effer- Presence of steal phenomenon, gliosis, atrophy, calcifica-
ent vessels could be single or multiple. Accelerated ath- tion, mass effect, hydrocephalus, etc
erosclerotic changes on the afferent vessels could result in
irregularities, stenosis, or aneurysmal formation that could Answer
alter the flow dynamics of the AVM. The details of the vas- 1. Complications of AVM include accelerated atherosclero-
culature are well demonstrated by CTA or MRA. DSA now sis on afferent arteries, hemorrhage, mass effect, volume
plays a role in endovascular treatment of AVM, with the pri- loss, hydrocephalus, dementia, and high cardiac output
mary diagnosis being made by CT/CTA and MRI/MRA. The failure.

Case
36 CLINICAL HISTORY 59-year-old female experienced weakness in her left leg
and left arm after hitting her head on the car.
76

Case 36 77
mass on all sequences with irregular, ring, and heterogeneous

contrast enhancement. GS may not always show a dural tail
despite its dural abutment. However, a variety of appearances
have been reported including diffusely infiltrating tumors and
ill-defined borders. Multifocal tumor is rare, and GS tends
to have more extracranial and intraaxial metastases than GB
which occurs in up to 30%. It may be difficult to distinguish
GS from meningioma or other extraaxial masses such as
dural metastases, lymphoma, or neurosarcoidosis because of
its dural attachment. The heterogeneity and pattern of con-
trast enhancement may resemble GB or metastases but dis-
tinguish it from abscess or lymphoma except in HIV patients
where lymphoma could show heterogeneous enhancement.
Other cortical-based enhancing tumors that could be con-
sidered would be pleomorphic xanthoastrocytoma (PXA),
dysembryoplastic neuroepithelial tumor (DNET), pilocytic
Figure 36-5
astrocytoma (PA), ganglioglioma (GG), and oligodendro-
glioma. The thickened irregular rind of enhancement of this
mass virtually excludes these tumors which are in general
FINDINGS Figure 36-1. Axial T2 FLAIR. There is a right
associated with mild or no edema.
parasagittal parietal cortical mass with dural abutment.
GS is a WHO IV rare tumor that presents in a broad range
Confluent hyperintensity is present anteriorly and laterally to
of ages (13 to 74 years) with a somewhat more common pre-
the mass most consistent with vasogenic edema or infiltrat-
sentation in the sixth and seventh decades. There is a male
ing tumor (vertical arrow). The mass is heterogeneous but
predominance. Clinical features depend on location. GS is a
mostly hyperintense with some inner rind of hypointensity
mixed tumor of glial and mesenchymal components which
(transverse arrow). Figure 36-2. Axial T2WI is very similar
could be primary or secondary. The sarcoma part is com-
to the T2 FLAIR image. There is cerebrospinal fluid (CSF)
posed of fascicles of spindle cells topographically related to
hyperintensity anteriorly and laterally within the mass con-
blood vessels. Mesenchymal element is usually fibroblastic
sistent with area of necrosis versus cyst formation (arrow).
but could be cartilage, bone, smooth muscle, etc. It forms
Figure36-3. Axial DWI through the mass. There is mild
about 3% of GBs.
hyperintensity in the medial and posterior rim of the mass
that corresponds with minimally elevated to normal ADC
values. The T2 hyperintensity anterior to the mass has mark-
edly elevated ADC values (arrow). The findings correspond 1. Is there a survival difference between GS and GB?
to a cystic necrotic mass with greater solid component pos- 2. Where are most metastatic lesions from GS found?
teriorly and medially with a rind of debris. Gradient echo
image that is not shown was unremarkable for calcifications Reporting Responsibilities
or blood product. Figure 36-4. Axial post-contrast T1WI. Every tumor deserves direct reporting. Location is important
There is an irregular rind of peripheral enhancement that is in articulating the differential and helping determine accessi-
rather intense anteriorly and laterally (arrow). The adjacent bility for surgery. Presence of leptomeningeal enhancement
dura/falx is abutted, thickened, and enhancing. Figure 36-5. may indicate metastases.
Photomicrograph shows proliferation of atypical spindle
cells arranged in a fascicular growth pattern (H&E stain). What the Treating Physician Needs to Know
Location. Proximity to the superior sagittal sinus in this
DIFFERENTIAL DIAGNOSISMeningioma, hemangio case may affect surgical plan
pericytoma, cortical gliomas, metastasis, abscess, lym- Other possibilities. A short list of differential may help in
phoma, glioblastoma (GB), gliosarcoma (GS). pursuing other options
Are there metastases?
DIAGNOSIS Gliosarcoma (GS). Is lumbar puncture (LP) safe when there is leptomeningeal
enhancement?
DISCUSSION GSs are supratentorial cortical tumors with
a majority located in the temporal lobes with some reports of Answers
higher incidence in the frontal lobes. They are located more 1. Prognosis is dismal with survival less than 15 months just
peripherally with dural abutment than GB. There is frequently as bad as GB. However, GS metastasizes more frequently
prominent edema and on CT can appear hyperdense mimicking than GB. O6-methylguanine-DNA-methyltransferase
meningioma. The enhancement can be uneven, and thick- (MGMT) methylation and IDH1 mutations are rare in GS
walled rim-like or ring enhancement and intratumoral strip indicating worse prognosis.
enhancement may be present. MRI shows heterogeneous 2. Both intracranial and extracranial in up to 30%.

Case
37 CLINICAL HISTORY 30-year-old female with craniopharyngioma and
multiple craniotomies.
78

Case 37 79
FINDINGS Figures 37-1 and 37-2. Axial NCCT soft tissue treatment-related abnormalities such as leukoencephalopa-
and bone windows, respectively, close to the vertex. There thy, infections either abscess or meningitis. In one particular
is a small chamber of an Ommaya reservoir assembly over series infection rate was 15%. Complication rates vary from
the anterior parietal bone (arrow). There is evidence of mul- 10% to 36%. Death has occurred as a result of malposition,
tiple prior craniotomies. Figure 37-3. Axial NCCT through malfunction, and other complications.
the level of the third ventricle. The tip of the Ommaya The Ommaya reservoir assembly has been used to aspi-
catheter is in a right suprasellar cyst embedded in the right rate craniopharyngioma cysts and deliver bleomycin into
thalamus (arrow). There is substantial right frontal cystic such cysts, antibiotic administration, treatment of lym-
encephalomalacia (star) from prior surgery with overlying phoma, leukemia, leptomeningeal tumors and metastases,
craniotomy. Figure 37-4. Axial NCCT through the lateral and intraventricular morphine injection in palliative care.
ventricles. There is a second Ommaya reservoir assembly Usually the catheter is placed within the cyst, tumor, or in
with the tip in a partially calcified right suprasylvian cys- the ventricles. Various techniques have been used for place-
tic mass (arrows). There is bifrontal encephalomalacia from ment of Ommaya reservoir. These include stereotaxy either
prior surgery (stars). with a frame or frameless navigation, endoscopy, ultrasound
guidance, and fluoroscopy.
DIFFERENTIAL DIAGNOSISVentricculoperitoneal shunt,
Ommaya reservoir. Question for Further Thought
1. What is an Ommaya reservoir?
DIAGNOSIS Ommaya reservoirs for treatment of intracra-
nial craniopharyngioma cysts. Reporting Responsibilities
The position of the reservoir is usually checked before use.
DISCUSSION The Ommaya reservoir is a versatile tool Post insertion CT has been recommended as an appropriate
for treatment of intracranial diseases that requires inter- tool to do this. Precise location of the tip of the reservoir
mittent aspiration or delivery of drugs and chemotherapy. should be reported directly to the physician. Complications
NCCT or MR usually demonstrates a subcutaneously should also be appropriately reported.
located chamber or reservoir under the scalp in a position
advantaged enough for the intended target. There is no
connection to the peritoneal cavity. The reservoir is con-
nected to a catheter that extends into the target. It is always Location of the tip or tips of the catheter if more than one
necessary to localize the tip within the intended target to Complications if any
avoid inadvertent injection or infusion of medication into
the wrong target. These targets may include cysts in and Answer
around the brain, tumors, and the ventricles. It is always 1. The Ommaya reservoir was invented in 1963 by Ayub K.
necessary to know the intended target. Complications Ommaya, a Pakistani neurosurgeon. It consists of a cath-
that could be observable at imaging include malposition eter that is attached to an implanted reservoir under the
of the catheter, intracranial hemorrhages both parenchy- scalp. The system could be either assembled at the time of
mal and extraaxial (subdural hematoma and hygromas), surgery or preassembled.

Case
38 CLINICAL HISTORY 52-year-old female with prior multiple craniotomies
and ventriculoperitoneal shunts on follow-up for seizure disorder.
80

Case 38 81
FINDINGS Figure 38-1. Axial NCCT through the centrum the fatty woven bone is usually hyperintense on MRI. There
semiovale. There is a mixed density right frontal extraaxial is invariably avid contrast enhancement of the dura and
collection (right transverse arrow). There is an area of focal the membrane. The pattern could be mistaken for calvarial
hyperdensity (calcification/ossification) at the anterior end of masses. If the woven bone pattern is confined to the frontal
the collection (vertical arrow). Left-sided dural bony excres- lobes, it may be mistaken for hyperostosis frontalis interna
cences are present (transverse left arrow). Figure 38-2. Axial in the appropriate age group.
NCCT bone window setting through the lateral ventricles. CCSDH is a very rare condition that is more common
There is a left anterior transfrontal and a right posterior trans- in children than in adults. Because of its rarity, the inci-
parietal ventriculoperitoneal (VP) shunts (vertical arrows). dence is not definitely known but said to occur in up to
Ossified excrescences are present along the inner table 10% of CSDH. It has been reported in association with
bilaterally with ossification of the falx (transverse arrows). chronic ventriculoperitoneal shunts, postmeningitic sub-
Figures 38-3 to 38-5. Axial T1WI, T2WI, and FLAIR, dural effusions, and late complications of head injuries
respectively, through the inferior lateral ventricles. There is a in atrophic brains. The pathogenesis is not known and
large biconvex heterogeneous right frontal convexity extra- calcification may take 3 to 12 months to develop, while
axial mass compressing the brain under the right frontopa- ossification takes much longer. The membrane could cal-
rietal craniotomy consistent with a large organized chronic cify in an eggshell fashion or the entire collection could
subdural hematoma (CSDH) (transverse arrows). There are ossify. Presentation is much varied from asymptomatic to
multiple hyperintense bony excrescences on all sequences symptoms and signs of raised intracranial pressure, sei-
along the inner table bilaterally (vertical arrows). Figure zures, neurologic deficit, decreased level of consciousness,
38-6. Coronal post-contrast fat sat T1WI through the level calvarial masses, and cortical hemorrhage. Asymptomatic
of the cavernous sinuses. There is thickened pachymenin- cases do not require treatment unless there is significant
geal contrast enhancement (vertical arrows) around the brain compression of the brain. Symptomatic cases have been
and surrounding the convexity calcified extraaxial masses. surgically resected.
A double layer is present along the left inner table surround-
ing the fat-suppressed extraaxial calcified SDH (transverse Question for Further Thought
arrows). These changes have remained stable for 8 years of 1. What is armored brain?
follow-up.
DIFFERENTIAL DIAGNOSISCalvarial mass, chronic Unless there are complications such as cortical hemor-
calcified/ossified subdural hematoma (CCSDH), hyperosto- rhage, brain edema, or herniations, routine reporting is suf-
sis frontalis interna. ficient. Complications should be communicated urgently and
directly. Circumferential calcification tightly adherent to the
DIAGNOSIS Chronic calcified/ossified subdural hema- brain should be reported as this could pose a potential prob-
toma (CCSDH). lem if surgical excision is envisaged.
DISCUSSION The CCSDH may present as a thin What the Treating Physician Needs to Know
hyperdense (calcified) shell surrounding the crescentic or Location and size and effect on the underlying brain
biconvex CSDH or as irregular sheaths of extraaxial hyper- Presence of armored brain may be a contraindication if
densities of varying sizes and shapes which could be contin- surgery is contemplated
uous or fragmentary on CT. There may be varying degrees
of brain compression depending on the size and location.
Woven bone configuration is not uncommon as shown in Answer
Figure 38-2. The MRI changes parallel the CT changes with 1. An armored brain is a form of CCSDH where there is cir-
extraaxial masses presenting as heterogeneous, hypoin- cumferential calcification of the subdural membrane encas-
tense, or hyperintense structures depending on whether the ing the brain with tight adhesion to the arachnoid membrane
content is organized blood, calcification, or woven bone on and the brain. Attempt to remove such a CCSDH surgically
all sequences. The calcification could be hypointense, while may result in damage to the u nderlying brain.

Case
39 CLINICAL HISTORY 62-year-old male on treatment for multiple myeloma.
Abdominal biopsy showed amyloid deposits; now presenting with altered mental
status.
FINDINGS Figure 39-1. Axial T2WI through the surface of the cerebral hemispheres (transverse arrows).
medulla. There is superficial thin hypointense coating of Multifocal hypointense in some cases heterogeneous
the surface of the medulla and cerebellum (arrows). Figure masses are present in the brain parenchyma consistent
39-2. Axial GRE through the inferior pons. There is a thin with multifocal hemorrhages. There are fluid blood levels
hypointensity coating of the surface of the pons, the brain the bilateral lateral ventricles (vertical arrows). There is
chium pontis bilaterally, and the cerebellum posteriorly a left frontotemporal subdural collection.
(arrows). There are multifocal hypointense lesions in the
cerebellum and brainstem thought to represent hemor- DIFFERENTIAL DIAGNOSIS Multifocal hematoma, muti-
rhagic foci due to cerebral amyloid angiopathy (CAA). focal cerebral cavernous malformations, superficial sidero-
Figures 39-3 and 39-4. Axial GRE through the upper pons sis, intraventricular hemorrhage.
and the thalami, respectively. There is an extensive super-
ficial hypointense coating of the brainstem, around the DIAGNOSIS Superficial siderosis (SS) due to multifocal
superior vermis and cerebellar folia, sylvian fissures, and hematoma.
82

Case 39 83
DISCUSSION The primary reason for presenting this case of age of onset mostly occurring in people above 40 years.
is the superficial siderosis due to recurrent bleed from amy- There appears to be a presymptomatic phase of the disease.
loid angiopathy. SS of the CNS is best demonstrated by GRE The neurologic manifestations include sensorineural hear-
sequence and T2WI. The hallmark is a very thin superficial ing loss, gait ataxia, dementia, pyramidal signs, and bladder
hypointense coating of the brain on T2WI and GRE. The disturbance. A clinical history of subarachnoid hemor-
finding is pathognomonic for this entity. It is a disease of rhage may be present. The source of the bleeding is found
the brain resulting from chronic iron or hemosiderin deposi- in 54% of cases. Dural pathology including cerebrospinal
tion on the surface (subpial layers of the brain and spinal fluid (CSF) cavity lesions (meningoceles or pseudomenin-
cord) of the CNS caused by repeated and persistent hemor- goceles) has been reported in 47% and vascular tumors in
rhage into the subarachnoid space. The posterior fossa par- 35%. SS is not a final diagnosis but an important finding
ticularly the superior vermis and cerebellum, the brainstem, indicating a remote or recurrent intracranial bleed into the
cranial nerves, and the base of the brain are preferentially subarachnoid space. Finding the cause of the bleed may
affected. GRE images are more likely to show SS than any lead to an effective surgical treatment, but this is always
other sequence because of its higher sensitivity for hemo- a challenge.
siderin deposition, and this is even more effectively dem-
onstrated on 3 T magnet because of the higher magnetic Question for Further Thought
susceptibility effects of blood degradation products such 1. Why is sensorineural hearing loss such a prominent part
as ferritin and hemosiderin at that field strength. Cerebellar of the clinical features of SS?
volume loss and hydrocephalus may be present in some of
these patients. Hypointense coating on the cranial nerves Reporting Responsibilities
particularly CN VIII may be visible. The reasons for the This is a chronic disease, and unless there is an acute lesion
chronic repeated hemorrhage may be visible on the MRI as such as aneurysm, AVM, parenchymal hematoma, masses
in this case with multifocal hemorrhages. Other causes of SS that is reportable directly, routine reporting is sufficient.
may include aneurysms, cerebral cavernous malformation Recommendation of spinal investigation may be prudent in
(CCM), repeated trauma, neoplasms, hemorrhagic infarcts, the absence of definite intracranial cause.
spinal and cerebral arteriovenous malformation (AVM) and
postoperatively from repeated shunts. There has also been
association of SS with intracranial hypovolaemia.
Fluid-filled collections of obscure origins particularly Location of SS
in the spinal canal are also seen in a number of these Associated pathology or complications, if present
cases. These are usually associated with pachymeningeal Recommendations for finding the cause
enhancement. Associated atrophy of cerebellar hemi-
spheres and vermis and leptomeningeal thickening with Answer
enhancement may be present. Despite extensive evalua- 1. Sensorineural hearing loss features in about 95% of SS
tion which may include cerebral and spinal CTA, MRA and may be the first symptom often accompanied by
and DSA, and CT myelogram, the cause of the bleeding tinnitus. The vulnerability of the eighth cranial nerve
may not be found. may be explained by the greater length of hemosid-
SS is a rare debilitating disease that is more common erin deposition resulting in demyelination, gliosis, and
in men than in women with a 3:1 ratio with a wide range neuronal loss.

Case
40 CLINICAL HISTORY 12-month-old with a nodule at the site of the anterior
fontanel.
FINDINGS Figure 40-1. Sagittal T1WI. There is a well- Question for Further Thought
circumscribed T1 hypointense subgaleal mass over the ante- 1. Can a dermoid be differentiated from an epidermoid by
rior fontanel (arrow). Figure 40-2. Coronal T2WI through imaging?
the mass. The mass is hyperintense (arrow). The lesion did
not restrict diffusion nor contrast enhance (not shown). Reporting Responsibilities
Routine reporting is sufficient for this benign lesion.
DIFFERENTIAL DIAGNOSISSebaceous cyst, epider- Recognize the benign nature of the lesion. A definite imag-
moid cyst, dermoid cyst. ing diagnosis is not necessary as both epidermoids and der-
moids in the subgaleal space are surgical lesions with a good
DIAGNOSIS Subgaleal dermoid cyst. prognosis.
DISCUSSION Congenital inclusion cysts occur as a result What the Treating Physician Needs to Know
of imperfect partitioning of ectodermal elements, particu- Subgaleal inclusion cysts are benign lesions which arise
larly involving sites of neural tube closure, during embryonic from mild anomalies of neural tube closure
development. Most commonly, these present as intracranial These lesions are easily treated at surgery with good out-
lesions, but when extracranial, they arise in the subgaleal comes. They do not imply the presence of other more sig-
space. Histologically, two types are recognized. Those cysts nificant neural tube anomalies
composed exclusively of epidermal lineages are referred to Location and size
as epidermoid. These are fluid density on CT, hyperintense
on T2WI, and generally isointense on T1WI. They contain Answer
epidermal cells and keratinous debris. Dermoids, by contrast, 1. Classically, dermoids are found at the midline, whether
contain epidermal as well as dermal elements including hair intracranially or extracranially. In the case of a sub-
follicles, sebaceous, and apocrine apparatus. On CT, der- galeal dermoid, the region of the anterior fontanel is a
moids can be less dense than fluid, reflecting the presence favored location. Subgaleal dermoids may also occur off-
of fatty sebaceous secretions. For the same reason, they may midline. Dermoids may have a fatty internal consistency,
be hyperintense on T1WI, though this is not obligatory. On which can help to distinguish them from epidermoids.
T2WI, variable and heterogeneous signal is typical. Neither Epidermoids, on the other hand, can show restricted dif-
epidermoid nor dermoid cysts enhance centrally, though fusion, perhaps reflecting the presence of ordered layers
either may show mild rim enhancement. of keratin filament.
84

Case
41 CLINICAL HISTORY 28-year-old right-handed female with initial presentation of
right-sided occipital and neck pain, forgetfulness, and subsequent decreased visual
acuity and left hemianopsia.
FINDINGS Figure 41-1. Right parasagittal T1WI MRI of post-contrast T1WI. There is a 3.3 cm 2 cm irregu-
the brain showing a heterogeneous hypointense lesion in lar thick crinkled avid enhancement corresponding to the
the right occipito-temporo-parietal white matter (WM) with middle layer in Figure 41-3 surrounding an irregular iso/
very minimal mass effect (arrow). Figure 41-2. Axial T2WI hypointense core. The peripheral portion is isointense with
through the mass. The mass (arrow) is lateral to the right the surrounding brain. The axial post-contrast images (not
trigone and occipital horn showing shades of hyperinten- shown) show some discontinuity in the enhancing portion.
sity from central almost cerebrospinal fluid (CSF) intensity There were a few non-contrast-enhancing periventricular
to less hyperintense variegated heterogeneous periphery. focal T2 hyperintensity elsewhere in the bilateral cerebral
There is no significant mass effect. Figure 41-3. Coronal WM (not shown).
FLAIR through the lesion showing a three-layer pattern of
isointense irregular core with surrounding irregular crin- DIFFERENTIAL DIAGNOSIS Tumefactive multiple scle-
kled hyperintensity and a periphery of smudgy medium rosis (TMS), astrocytoma, granuloma, progressive multifo-
hyperintensity presumably edema. Figure 41-4. Coronal cal leucoencephalopathy (PML).
85

86 Case 41
DIAGNOSIS TMS. to steroid therapy with resolution of the lesions and their
mass effect. However, in this patient, despite initial treat-
DISCUSSION TMS as opposed to tumefactive demyelin- ment with intravenous corticosteroid and subsequent oral
ating lesion (TDL) occurs in the contest of multiple sclero- steroid therapy, the lesion was unrelenting and showed pro-
sis (MS) in which case the diagnosis of MS is established gression with new lesions elsewhere in the brain on several
with the dissemination in time and space criteria as well as follow-up MRI.
other criteria satisfied. A tumefactive demyelination could
also progress to MS after satisfying the usual criteria. TMS Question for Further Thought
is described as large well-demarcated mass-like WM lesions 1. What other lesions can be confused or coexist with TMS?
larger than 2 cm with little mass effect and edema. The other
MRI characteristics include hypointensity on T1WI and Reporting Responsibilities
hyperintensity on FLAIR and T2WI with a variety of con- Since the diagnosis is almost always in doubt, direct report-
trast-enhancing patterns including incomplete ring, arc, or ing is appropriate. Hint of high-grade malignancy should
nodular contrast enhancement as seen in other MS plaques. be stressed. Advanced imaging such as perfusion and MR
The central nonenhancing core of the incomplete ring is spectroscopy (MRS) may be helpful in this regard and can
thought to represent a more chronic phase of the demyelin- be recommended. Presence of leptomeningeal enhancement
ation. TMS lesions are usually single but multiple lesions are may suggest alternative diagnosis.
reported. The size makes confusion with other neoplastic/
inflammatory lesions such as glioblastoma (GB), lymphoma,
abscess, and PML possible. However, the pattern of enhance-
ment and surrounding edema and the location tend to cast Location is important regarding planning for biopsy
doubt on the differentials. There may be areas of restricted Is there any significant mass effect? Is lumbar puncture
diffusion and decreased perfusion, thus separating TMS (LP) safe?
from some of its mimics. Dilated vascular structures within Presence of other WM lesions that might suggest MS
tumefactive lesions have been reported in T2-echoplanar MR Any leptomeningeal enhancement?
perfusion. Balo concentric sclerosis, another form of demye-
linating process, usually presents with many concentric rings Answer
of alternating zones of demyelinated and myelinated WM 1. TMS like other TDLs has been described rarely in associ-
somewhat dissimilar to this present case. ation with astrocytoma. Solitary TMS lesion has preceded
TMS is more common in women and because of its the appearance of lymphoma and therefore longitudinal
mass-like configuration may present with symptoms of monitoring of single TDL is warranted. Histologic diag-
mass lesions such as headache, focal neurologic deficit, and nosis of TDL may be misinterpreted as a neoplasm given
aphasia. Her CSF was positive for oligoclonal bands along its hypercellular nature and the frequent presence of atyp-
with high glucose. Serology was negative for multiple other ical reactive astrocytes and mitotic figures. These features
diseases. These lesions are supposed to respond very rapidly pose a potential trap for the pathologist.

Case
42 CLINICAL HISTORY 71-year-old female noted to have frequent falls for the past
3 years, recently suffered apraxia, poor spatial orientation, visual hallucinations,
orthostatic hypotension, and cogwheel rigidity in the left arm.
Figure 42-1
FINDINGS Figure 42-1. F-18 FDG-PET. Top two rows: noted in Parkinson disease. Decreased dopamine transporter
brain surface project hypermetabolic maps. Bottom two uptake on 123I-FP-CIT (DAT scan) SPECT or occipital
rows: hypometabolic maps. There is markedly decreased hypoperfusion PET has the greatest reliability compared
metabolism (yellow areas) in bilateral frontal lobes and mod- with other imaging modalities and is listed as one of the sug-
erately decreased metabolism (blue areas) in occipital, tem- gestive features of the diagnostic criteria. Parietal lobe hypo-
poral, and parietal lobes. The occipital lobe hypometabolism perfusion is commonly found in both AD and DLB.
is characteristic of Lewy body disease. DLB is the second most common cause of neurodegen-
erative dementia after AD. The most striking symptoms are
DIFFERENTIAL DIAGNOSISAlzheimer disease (AD), progressive cognitive decline with fluctuations, visual hal-
Dementia with Lewy bodies (DLBs). Parkinson disease with lucinations, and parkinsonism. The age of onset is usually
dementia (PDD), normal pressure hydrocephalus (NPH), later than 50.
Parkinson-plus syndromes (progressive supranuclear palsy, Pathologically Lewy bodies are the intracellular alpha-
multisystem atrophy, corticobasal degeneration). synuclein protein aggregates. The relationship of DLB
and PDD is unclear. Practically, when dementia antedates
DIAGNOSIS Dementia with Lewy bodies (DLBs). parkinsonism the diagnosis is DLB and vice versa. There
is no imaging pearl differentiating these two entities.
DISCUSSION On structural imaging with MRI, hip- Pathologically Lewy bodies predominate in the brainstem in
pocampal atrophy is less severe compared with AD and the PDD, whereas in the cerebral cortex in DLB, probably
medial temporal lobe structures are relatively preserved. underlying the preceding cognitive decline. Neuronal loss in
PET 11-carbon-labeled Pittsburgh compound-B (11C-PIB) the substantia nigra is less severe in DLB.
detects -amyloid accumulation widely in brain in DLB just The clinical distinction between DLB and AD is a chal-
as AD. The only clue may be that a much lesser accumulation lenge and -amyloid accumulation is a known feature in
87

88 Case 42
DLB. Alzheimer-type clinical findings usually coexist but do h ydrocephalus, cerebrovascular disease, intracranial mass,
not fulfill the criteria for AD diagnosis. Despite a similar level and fluid collection. In cases of brain atrophy the involved
of dementia severity, DLB selectively involves visual asso- site should be addressed.
ciation areas and subcortical structures. Visual hallucination
is one of the major clinical features which manifests as brain What the Treating Physician Needs to Know
hypometabolism in occipital lobe on FDG-PET. Despite the diverse clinical presentation, differentiation
from NPH can be challenging. That is usually when the
Question for Further Thought treating physician orders an imaging study
1. Is there an early diagnostic tool for DLB?
Answer
Reporting Responsibility 1. Imaging is an excellent tool to assist in the diagnosis of
Routine reporting is sufficient as the diagnosis is clini- DLB ruling out other potential causes of dementia, unfor-
cal. Patients should carefully be evaluated for evidence of tunately there is no early diagnostic tool available.

Case
43 CLINICAL HISTORY 48-year-old male with headaches.
FINDINGS Figures 43-1 and 43-2. Axial FLAIR and corre- DIAGNOSIS Tectal glioma.
sponding T2WI respectively through the midbrain. There is
a 1.2-cm round hyperintense dorsal midbrain mass (arrows) DISCUSSION Tectal glioma is one of the most benign
encompassing the cerebral aqueduct. Apart from a mild bulge focal brainstem gliomas and very rare in adults. MRI is the
posteriorly, there is no mass effect. Figures 43-3 and 43-4. most appropriate modality for the evaluation of tectal glioma.
Sagittal pre- and post-contrast T1WI through the mass. The The mass is usually located in the tectum and may either
mass is hypointense and does not enhance (arrows). There is wrap around the aqueduct or compress the aqueduct. It
a posterior bulge of the colliculi with apparent obliteration bulges into the quadrigeminal cistern or presents as an
of the aqueduct. exophytic mass. Lesion size could be as large as 5 cm.
It is mostly isointense with a minority hypointense or
DIFFERENTIAL DIAGNOSIS Tectal glioma, pineal mass, hyperintense on T1WI and mostly hyperintense on FLAIR
midbrain cyst, neurofibromatosis type 1 (NF1). and T2WI. It is well circumscribed and rarely contrast
89

90 Case 43
enhances. However, posttreatment contrast enhancement and resection. It is considered an indolent tumor which rarely
is present in 50% following radiosurgery. Calcifications progresses requiring no more than CSF diversion in view of
and cysts may be present. In its critical location, tectal its excellent prognosis.
glioma causes hydrocephalus by obstructing the aque-
duct. Hydrocephalus could be detected in all cases. CT Question for Further Thought
may not adequately show lesions in this location and 1. In what way is tectal glioma different from other brain-
could be normal in tectal glioma with the hydrocephalus stem gliomas?
ascribed to other causes such as aqueductal stenosis. Other
differentials may include pineal region mass, NF1, and Reporting Responsibilities
neuroepithelial cyst. Direct reporting is essential. Presence of hydrocephalus
It is a WHO I/II low-grade astrocytoma. The pathology makes direct reporting mandatory.
may include pilocytic astrocytoma, hamartoma, fibrillary
astrocytoma, or rarely higher grade astrocytomas. It has a What the Treating Physician Needs to Know
mean age at presentation of 10 years. It is very rare in adults Pattern of lesion
forming 8% of adult brainstem gliomas (1.2% to 2.5% of all Presence of hydrocephalus
adult central nervous system [CNS] tumors). It is more com-
mon in men than women. Presenting symptoms include head- Answer
ache, visual problems, and ataxia with balance p roblems. In 1. It is focal and confined to the tectal plate. It usually shows
about a quarter of the patients, the tumor is detected inciden- no infiltrative tendency like the intrinsic diffuse brainstem
tally. Treatment options include cerebrospinal fluid (CSF) glioma. It rarely progresses and should require no more
diversion for the hydrocephalus, radiation, chemotherapy, than CSF diversion for treatment.

Case
44 CLINICAL HISTORY 10-year-old male with headache.
FINDINGS Figure 44-1. Axial NCCT through the poste- DIFFERENTIAL DIAGNOSIS Medulloblastoma, ependy-
rior fossa. There is a heterogeneous midline posterior fossa moma, choroid plexus papilloma.
mass with scattered calcifications (arrows). Figure 44-2.
Sagittal T1WI. The mass is within the fourth ventricle, DIAGNOSIS Ependymoma.
with extension of tumor through the midline foramen of
Magendie (arrow). Notice stretching of the corpus callo- DISCUSSION Ependymoma is a tumor derived from
sum, indicating hydrocephalus (star). Figure 44-3. Axial ependymal rest cells. Most are infratentorial and within the
T2WI through the mass. The lesion is heterogeneous with fourth ventricle. They are usually well defined and show
some cystic foci. Figure 44-4. Axial post-contrast T1WI very heterogeneous characteristics on both CT and MRI,
through the mass. There are areas of enhancement (arrows) reflecting the presence of tumoral hemorrhage or cysts and
within the mass. calcifications. Supratentorial ependymomas tend to exhibit
91

92 Case 44
more cystic component than their posterior fossa counter- Reporting Responsibilities
part. Enhancement is variable, but most do enhance to some Direct reporting is necessary in this obstructive neoplasm. The
degree. There is usually very little or no perilesional edema. hydrocephalus should be treated promptly. Identify the tumor
Classically, ependymoma is described as a plastic tumor in as being largely intraventricular as opposed to paraventricu-
that it can insinuate itself and extrude, almost like toothpaste, lar, which can help to limit the differential. Note any associ-
through small spaces such as the foramina of Luschka and ated hydrocephalus as this may need to be addressed urgently.
Magendie. When present, this is a very helpful imaging sign Presence of leptomeningeal tumors should be mentioned.
to discriminate it from the other posterior fossa masses listed
in the differential diagnosis. What the Treating Physician Needs to Know
Ependymomas are designated WHO II and III tumors, Ependymoma is one of several tumors arising within the
and there is no definite imaging way to separate WHO posterior fossa, often in children or young adults
II lesion from the anaplastic tumor except that grade III Imaging findings can be suggestive of the diagnosis
lesions are more likely to contrast enhance. The histologic There is overlap between the appearances of posterior
criteria are also very uncertain. The difference is in their fossa tumors, and therefore, a differential diagnosis should
biologic and clinical behaviors. The anaplastic lesion grows always be given
rather more rapidly causing raised intracranial pressure Presence of complications such as herniations, CSF dis-
early in the disease. SV40 virus strain has been identified in semination, and hydrocephalus
ependymomas, raising the possibility of an association with
this infection. Ependymomas have no gender preference. Answer
Posterior fossa ependymomas are more common in children 1. Ependymal rest cells can be found anywhere in the peri-
than adults and invariably present with features of hydro- ventricular region and even somewhat removed from
cephalus and raised intracranial pressure such as headache, the ventricle. On rare occasions, ependymomas may
nausea, and vomiting. Macrocrania is a feature in children even appear to be entirely parenchymal. These are usu-
under the age of 2 years. Children with posterior fossa ally supratentorial in location. More typically, posterior
ependymomas fare worse than their adult counterpart prin- fossa ependymomas are often described as arising from
cipally because of the location of their tumors in the poste- the floor of the fourth ventricle. This results in a tumor
rior fossa and increased incidence of anaplasia. Incomplete with a sharp interface along the ventricle roof. On the
resection and cerebrospinal fluid (CSF) disseminations are other hand, medulloblastoma, which would be high on
poor prognostic factors. the differential whenever ependymoma is considered, is
described as arising from the fourth ventricle roof, giving
Question for Further Thought a sharp interface with the ventricular floor.
1. Where, precisely, is the layer of cells from which an epen-
dymoma arises?

Case
45 CLINICAL HISTORY 35-year-old male with intermittent headaches.
93

94 Case 45
FINDINGS Figures 45-1 and 45-2. Axial DWI with cor- etastases is present in about 33% of anaplastic ependymo-
m
responding ADC map through the lateral ventricles. There mas at diagnosis. CSF spread is also much more common
is a mixed solid cystic mass occupying the left trigone in young children and with infratentorial tumors than with
with restricted diffusion in the solid component (transverse supratentorial tumors. CT usually shows a solid cystic mass
arrows). The ADC map shows surrounding parenchymal with avid contrast enhancement. Calcification is present in
finger-like hyperintensity consistent with vasogenic edema about 50% which with hemorrhage result in heterogeneous
(vertical arrow in Figure 45-2). Figure 45-3. Axial T2WI. hyperdensity of the tumor.
The solid component of the mass is heterogeneous but Both low-grade and high-grade ependymomas have sim-
mostly isointense with gray matter (GM) with tiny hyperin- ilar clinical presentation except that the anaplastic tumors
tense areas (transverse arrow). The cystic components have tend to develop more rapidly resulting in raised intracra-
cerebrospinal fluid (CSF) intensity (vertical arrow) and pos- nial pressure early in the disease. Large head, headache,
teriorly to the mass is the hyperintense vasogenic edema. dizziness, ataxia, and vomiting are common in children
Figure 45-4. Axial post-contrast T1WI. There is avid contrast and posterior fossa lesions. Neurologic deficit, seizures,
enhancement of the solid component with mild heterogene- and features of raised intracranial pressure are common in
ity. A rim of contrast enhancement surrounds the entire mass supratentorial ependymomas. There is no gender prefer-
including the cystic components. ence. Anaplastic changes are more common in childhood
diseases and posterior fossa location. Anaplastic ependy-
DIFFERENTIAL DIAGNOSIS Ependymoma, choroid ple momas are WHO III tumors which show increased cellular-
xus carcinoma, subependymoma. ity, brisk mitotic activities, and microvascular proliferation.
Treatment usually consists of surgical resection with radia-
DIAGNOSIS Anaplastic ependymoma WHO III. tion treatment for anaplastic ependymomas. Craniospinal
radiation is recommended for known CSF seeding. Surgical
DISCUSSION Ependymoma is a tumor of children and resection is recommended for WHO II ependymomas with
young adult growing from the ventricular wall or the central the option of radiation treatment for incomplete resection
spinal cord canal. Anaplastic ependymoma is a histologic and CSF seeding.
diagnosis, and imaging is not able to differentiate the differ-
ent grades of ependymoma. Supratentorial ependymomas Question for Further Thought
form 30% of intracranial ependymomas; 70% of supra- 1. Is craniospinal MRI necessary in the evaluation of epen-
tentorial ependymomas are parenchymal extraventricular dymoma?
in origin. The tumor tends to be well circumscribed but
occasionally frankly invasive, most often showing necrotic, Reporting Responsibilities
cystic, and solid areas particularly supratentorial ependy- Direct reporting is important in ependymomas. Location,
moma. It restricts diffusion due to its high cellularity, but presence of hydrocephalus, and leptomeningeal enhance-
restricted diffusion is not present in all ependymomas. The ment are important with regard to prognosis.
solid component may show some heterogeneity presenting
as hypointense to isointense to hyperintense on T2WI and What the Treating Physician Needs to Know
hyperintense on FLAIR. On T1WI, the solid component is Location
mostly hypointense. Calcification or hemorrhage produces Presence of CSF seeding
some degree of signal heterogeneity but usually hypointense Presence of hydrocephalus
on all sequences. Cystic areas may follow CSF intensity on
all sequences with some showing hyperintensity on FLAIR Answer
due to high protein content. The tumor usually shows het- 1. Yes. Indicators of adverse outcome at the time of initial
erogeneous but avid contrast enhancement. Perfusion MRI diagnosis include young age below 3 years, anaplastic
generally demonstrates markedly elevated cerebral blood histologic features, incomplete surgical resection, and
volume which may not return to baseline due to leaky cap- evidence of CSF seeding. Presence of tumor spread via
illaries. Hydrocephalus is usually a feature of intraven- the CSF will alter the management strategies of ependy-
tricular ependymomas particularly posterior fossa lesions. moma. It is therefore necessary that craniospinal MRI be
Presence of leptomeningeal enhancement, smooth or nodu- obtained at the time of initial diagnosis to exclude CSF
lar, is indicative of CSF seeding. Positive CSF cytology for seeding.

Case
46 CLINICAL HISTORY 55-year-old male with a history of diabetes, hyperten-
sion, dyslipidemia, and current smoker presenting with left-sided weakness and
expressive aphasia.
FINDINGS Figures 46-1 and 46-2. Axial DWI and FLAIR the splenium (chevron) in Figure 46-3. Images of the mid-
MRI through the brachium pontes. There are central areas brain (not shown) revealed similar changes. Figure 46-5.
of diffusion restriction within T2 hyperintensity in bilateral Axial FLAIR images through the corona radiata. There is
brachium pontes extending into adjacent pons and cerebel- almost bilateral symmetrical coronal radiata periventricu-
lum bilaterally (transverse arrows). The nodulus also shows lar (transverse arrows) and occipital subcortical (vertical
tiny restricted diffusion within a larger T2 hyperintensity arrows) hyperintensity. Figure 46-6. Axial FLAIR through
(vertical arrows). Figures 46-3 and 46-4. Axial DWI and the centrum semiovale. There is bilateral multifocal sub-
T2WI through the thalami. There are bilateral thalamocortical frontoparietal white matter (WM) hyperintensity.
capsular and posterior lentiform nuclei areas of diffusion There were no areas of abnormal contrast enhancement
restriction within larger areas of T2 hyperintensity possibly on the initial and follow-up images. Follow-up GRE (not
surrounding edema (arrows). There are smaller lesions in shown) 4days later through the pons showed petechial
the bilateral subcortical occipital lobes (vertical arrows) and hemorrhages.
95

96 Case 46
DIFFERENTIAL DIAGNOSIS Posterior reversible enceph vaccination within 2 to 4 weeks. It can occur at any age
alopathy syndrome (PRES), acute disseminated encepha- but is more common in childhood. ADEM presents as an
lomyelitis (ADEM), osmotic myelinolysis (OM), multiple acute prodromal stage of fever, headache, malaise, nausea
sclerosis (MS). and vomiting followed by encephalopathy, and multifo-
cal symptoms. Although ADEM is thought to be a mono-
DIAGNOSIS ADEM. phasic disease, recurrent or multiphasic forms have been
described. Recurrent ADEM refers to recurrence of the
DISCUSSION MRI is very important for the diagnosis of initial symptoms 3 or more months after the first ADEM
ADEM. CT may be normal or show nonspecific hypoden- event, with the same localization by history, examination,
sities. Contrast enhancement could be variable. Imaging and imaging. Multiphasic ADEM refers to the occurrence
findings could be significantly delayed. The abnormalities of a new clinical event meeting the criteria for ADEM,
are best identified on FLAIR and T2-weighted images. The 3 or more months after the first event and involving new
lesions are typically large, confluent, and poorly demarcated anatomic areas by history, examination, and imaging.
T2 hyperintensities with corresponding T1 hypointensities. Older studies reported a 10% mortality rate with ADEM,
Lesions are bilateral but asymmetric involving the subcorti- although this number could be less. Acute hemorrhagic
cal and central WM and gray matter (GM) of the cerebrum leukoencephalitis (AHLE) and acute necrotizing hemor-
and cerebellum, brainstem, and brachium pontes. Spinal cord rhagic leukoencephalitis (ANHLE) are considered extreme
involvement could be long similar to longitudinal extensive variants of ADEM. The autopsy findings in this case were
transverse myelitis (LETM) or focal and present in up to 30% those of ANHLE. Death is common within a week from
of ADEM population. The basal ganglia and thalami are fre- brain edema, although early and aggressive treatment with
quently involved in a symmetrical fashion which would be steroids, plasma exchange, and IVIG might increase the
unusual in MS and PRES. The periventricular area is also chance of a favorable outcome.
involved but the typical ovoid lesions characteristic of MS are
not present. Corpus callosum involvement is usually smudgy
and not as well defined as in MS. Gadolinium enhancement
is variable in presence and shape: linear, incomplete or com- 1. Is spine involvement useful in differentiating ADEM
plete ring enhancement, nodular, or gyriform. Hemorrhage from its mimics?
is not a typical feature of ADEM but occurs in its variants.
Hemorrhage is best demonstrated on the GRE. The lesions Reporting Responsibilities
with respect to their WM and subcortical location and signal This is an acute situation requiring direct reporting.
pattern could mimic PRES. However, deep GM involvement Location of lesions helps differentiate ADEM from MS
is uncommon in PRES. Large central pontine lesion with at imaging. Brain swelling, herniations, or hydrocephalus
peripheral sparing is the hallmark of OM. Associated deep should be noted.
GM lesions occur in extrapontine OM.
ADEM is an acute autoimmune-mediated inflammatory What the Treating Physician Needs to Know
demyelinating disease that typically follows an infection or Location of lesions. Any complications?

Case 46 97
Is it safe to perform lumbar puncture (LP)? Any complica- Is spinal MRI useful in evaluation of ADEM? Postinfectious
tions that might prevent LP? myelitis or cerebellitis represent focal forms of ADEM.
Any changes on follow-up that may be useful in exclud-
ing the mimics or in further defining ADEM? Any new Answer
symptoms or signs clinically or by MRI within the first 3 1. Spinal involvement is useful in differentiating ADEM
months of the inciting ADEM event are considered as part from PRES and vasculitis but may not be helpful in
of the same event. excluding MS except in the presence of encephalopathy
which is present in ADEM but not in MS.

Case
47 CLINICAL HISTORY 70-year-old female, healthy otherwise, presenting with rapidly
progressive dementia for the last 2 to 3 months and language difficulties. Upon admission
to the hospital, she was nonverbal, became progressively ataxic, and elicited myoclonus.
FINDINGS Figures 47-1 and 47-2. Axial DWI, and cor- the descending order of frontal, parietal, and temporal lobes.
responding FLAIR at the level of basal ganglia. Diffusion The involved areas do not show enhancement. Sporadic CJD
restriction and increased FLAIR signal are noted in the dominates in the cerebral cortex. It predominantly involves
bilateral corpus striatum (transverse arrows) and thalamus the gray matter (GM) (cortex), basal ganglia (striatum, cau-
(vertical arrows). Two characteristic signspulvinar sign date and putamen more than globus pallidus), and thalamus
(symmetric involvement of pulvinar, the posterior nuclei of in the descending order. Primary sensorimotor cortex is
thalamus) and hockey stick sign (symmetric involvement relatively spared until late stage and cortical involvement is
of pulvinar and dorsomedial thalamus)are present (vertical asymmetric. PET and SPECT are nonspecific but adjunctive
arrows). There is also involvement of the insula cortex. for diagnosis of early stages, with demonstration of regional
glucose hypometabolism and decreased uptake of tracer and
DIFFERENTIAL DIAGNOSISAcute hypoxic ischemic absolute values on rCBV.
encephalopathy (HIE), encephalitis, other causes of dementia CJD is a fatal, rapidly progressive neurodegenerative
(Alzheimer disease, frontotemporal dementia, multi-infarct disorder caused by prions. The clinical presentation is non-
dementia, corticobasal degeneration), Leigh syndrome, specific and is assessed under the umbrella of dementia syn-
Wilson disease, Creutzfeldt-Jakob disease. dromes. The definite diagnosis is obtained via brain biopsy.
Three subtypes exist as sporadic (85%), familial (15%), and
DIAGNOSIS Creutzfeldt-Jakob disease (CJD), variant iatrogenic/infectious (<1%, includes variant form). The spo-
form. radic form is the most common and, as suggested by WHO in
2009, the diagnosis comprises combinations of myoclonus,
DISCUSSION Typical MRI findings of variant form are visual or cerebellar signs, pyramidal/extrapyramidal signs,
DWI-ADC abnormality and T2/FLAIR hyperintensity of akinetic mutism, and diagnostic tests such as typical electro-
the striatum (caudate and/or putamen), thalamus, and cortex encephalography (EEG) (periodic sharp wave complexes),
(cerebral and/or cerebellar). Cerebral cortex is involved in positive 14-3-3 or tau protein cerebrospinal fluid (CSF)
98

Case 47 99
assay and MRI findings. NECT is usually normal, besides What the Treating Physician Needs to Know
brain atrophy on successive imaging. The differential diagnosis of CJD is varied, with each of
the diseases having diverse treatment options. Early and
Question for Further Thought accurate diagnosis is crucial for best treatment options
1. What is the most challenging differential diagnosis in NECT of the head is often nonspecific, when there is clini-
CJD? cal concern, brain MRI should promptly be obtained if
there is no contraindication
Reporting Responsibility
Direct reporting is essential in this transmissible disease. Answer
MRI findings can be subtle, but given high fatality of the 1. Differentiation from acute HIE may sometimes be diffi-
disease, early diagnosis is essential to salvage the residual cult given the specific cerebral/cerebellar cortical involve-
functional brain parenchyma with newly developed potential ment in the variant form. subacute symptom onset, lack
therapeutic agents as well as prompt offer of adequate pal- of edema, and mass effect should steer away from HIE.
liative care.

Case
48 CLINICAL HISTORY 60-year-old male with endocrine dysfunction.
FINDINGS Figure 48-1. Sagittal non-contrast T1WI. There asymptomatic individuals managed expectantly and surgical
is a well-defined 1-cm mass in the expected location of the resection reserved for symptomatic patients. Recurrence is
pituitary stalk (arrow). It is isointense with the brain. The nor- common in patients managed surgically secondary to the
mal hyperintense neurohypophysis is not seen. Figure48-2. extensive vascular supply and surrounding structures mak-
Coronal post-contrast T1WI through the mass. There is avid ing complete resection difficult.
enhancement of the mass. There is mild impression on the
chiasm (arrow). Questions for Further Thought
1. What is a pituicyte?
DIFFERENTIAL DIAGNOSISPituicytoma, pituitary ade- 2. What other types of tumors arise in the neurohypophysis?
noma, optic nerve glioma, meningioma, lymphocytic hypoph-
ysitis, pituitary metastasis, neurosarcoidosis. Reporting Responsibilities
Routine reporting is sufficient in this indolent tumor unless
DIAGNOSIS Pituicytoma. there is compression of critical structures or discovered
unexpectedly. Describe the abnormalities and their extent.
DISCUSSION Pituicytoma is a rare tumor arising from Suggest further evaluation with angiographic imaging to
pituicytes in the neurohypophysis or the pituitary stalk. MRI evaluate vascularity of the tumor.
is the examination of choice for the evaluation of pituicy-
toma. Typical MRI findings include an avidly enhancing What the Treating Physician Needs to Know
mass in the posterior pituitary gland or the stalk with an
Relevant differential diagnosis
often absent posterior hyperintense neurohypophysis on
non-contrast T1WI. The tumor is isointense to hyperintense Potential for surgical complications secondary to vascular-
on T2WI. On angiography, pituicytomas demonstrate a ity of the tumor
prominent tumor blush as a result of their rich capillary net-
work. It could be difficult to differentiate pituicytoma from Answers
its differentials as most lesions in this location are usually 1. A pituicyte is a specialized glial cell that arises from the
well defined and contrast enhancing, perhaps with the excep- ependymal cell lineage. Pituicytes are the predominant
tion of hamartoma. Meningioma is dural based with dural cell type in the neurohypophysis and assist in the storage
tail visible. and release of hormones.
Affected individuals typically present in the fifth decade 2. Tumors arising from the neurohypophysis are much
with endocrine dysfunction and/or visual field deficits from lesscommon than those arising from the adenohypoph-
mass effect on the optic chiasm. The tumor may also be an ysis with the most common being adenoma. Additional
incidental finding in an asymptomatic patient. Pathologically, tumors arising from the neurohypophysis include metas-
pituicytoma is a WHO I fibrillary astrocytoma that is typi- tases, choristoma, hamartoma, craniopharyngioma, ger-
cally indolent. Treatment is dependent upon symptoms with minoma, ganglioglioma, teratoma, and schwannoma.
100
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Case
49 CLINICAL HISTORY 28-year-old female with postpartum headache. She was
readmitted 4 days later following seizure at home.
101

102 Case 49
Figure 49-6
DISCUSSION The most important diagnostic workup

for PRES is imaging. NCCT shows diffuse hypodense
areas in subcortical and cortical bilateral temporal occipital
and parietal lobes. MRI lesions of PRES appear as bilat-
eral cortical and/or subcortical iso- or hypointensities on
T1WI and hyperintensities on T2WI and FLAIR predomi-
nantly posteriorly in the temporal, occipital, and parietal
Figure 49-5 lobes. Bilateral frontal lobes particularly superiorly are also
affected as in this patient. The parietal or occipital lobes
are involved in 98% of cases, frontal lobes (68%), the tem-
poral lobes (40%), and the cerebellar hemispheres (30%).
FINDINGS Figures 49-1. Axial DWI through the upper A bilateral and symmetrical appearance is highly typical.
centrum semiovale showing patchy bilateral almost sym- DWI generally show increase diffusion consistent with
metrical frontoparietal cortical hyperintensity with cor- vasogenic edema. Occasional areas of restricted diffusion
responding ADC hyperintensity in Figure 49-2 (arrows). indicate ischemic changes. The lack of restricted diffusion
Figures49-3 and 49-4. Axial FLAIR and T2WI, respec- in the classical areas tends to exclude multifocal water-
tively, through same level confirm cortical patchy hyperin- shed infarcts. Hemorrhages, ischemic lesions, and contrast
tensity with mild effacement of sulci in the involved areas enhancement are unusual but do occur in a small percent-
consistent with cortical swelling (arrows). Subcortical U age of patients. Perfusion imaging may show a reduction
fibers are intact. Figure 49-5. Axial FLAIR through the in relative Cerebral Blood Volume (rCBV) and Cerebral
lateral ventricles. There is almost symmetrical cortical Blood Flow (CBF) consistent with hypoperfusion. MR
ribbon multifocal hyperintensity in bilateral frontal and spectroscopy may show increase choline and creatine peaks
parieto-occipital lobes (arrows). Temporal lobes (not shown) with decreased N-acetyl aspartate (NAA). MRA and MRV
are also involved. Figure 49-6. Axial FLAIR through the are normal, thus excluding vasculopathy and venous sinus
cerebellum. There is patchy bilateral almost symmetrical thrombosis. However, there has been some angiographic
cerebellar hyperintensity (arrows). Subtle changes are also demonstration of vascular irregularities and string-of-bead
present in the bilateral brachium pontes and tegmentum. appearance that may suggest some form of vasculopathy.
Follow-up MRI 4 months after these images demonstrated The clinical presentation of PRES includes head-
complete resolution of the lesions. ache, confusion, visual impairment, nausea, vomiting, and
seizures. Other findings include status epilepticus, focal
DIFFERENTIAL DIAGNOSIS Posterior reversible neurologic deficits, cerebellar syndrome, and coma. PRES
encephalopathy syndrome (PRES), multifocal watershed triggers include arterial hypertension in the classic setting of
subacute infarcts, multifocal cortical edema, acute demy- hypertensive encephalopathy, eclampsia or pre-eclampsia,
elinating encephalomyelitis (ADEM), cerebral venous sinus chemotherapy agents such as cisplatin and methotrexate,
thrombosis. immunosuppressant such as cyclosporine and tacrolimus in
organ transplant patients, septicemia and severe infections,
DIAGNOSIS Posterior reversible encephalopathy syndrome chronic renal failure and dialysis, autoimmune diseases such
(PRES). as systemic lupus erythematosus (SLE), scleroderma, and

Case 49 103
Wegeners granulomatosis. Moderately to severely elevated 2. What is the relationship between PRES and NMO anti-
blood pressure is reported in 75% of patients. bodies?
The pathophysiology of PRES is poorly understood. These
include (1) a breakdown of cerebral autoregulation due to a Reporting Responsibilities
rapid rise in blood pressure leading to disruption of the blood PRES is an emergency and requires direct reporting of find-
brain barrier, (2) endothelial dysfunction due to circulating ings to the referring physician to allow prompt withdrawal or
toxins which is more pertinent for triggers such as cytotoxic treatment of any known triggers.
and immunosuppressive drugs, sepsis, and autoimmune dis-
ease and pre-eclampsia, and (3) focal vasospasm leading to
decreased blood flow and ischemia with resultant edema.
Usually all blood tests are normal. Cerebrospinal fluid (CSF) MRI is the definitive imaging investigation of PRES
may be normal or have a slightly raised protein level. Abnormal Atypical imaging features include involvement of the
blood tests when they occur are nonspecific for PRES and may basal ganglia, deep white matter and splenium of the cor-
reflect underlying pathology. Laboratory evidence of endothe- pus callosum, isolated brainstem lesions, and unilateral
lial injury with thrombocytopenia, red cell fragmentation with involvement. Hydrocephalus can complicate swelling in
schistocyte formation and increased lactate dehydrogenase the posterior fossa
may be present especially in pre-eclampsia/eclampsia.
Management usually consists of removal and/or treat- Answers
ment of triggers, airways support, and seizure control. 1. The predominance of posterior involvement is explained
Complete recovery is the rule unless there are complications. on the basis of poor sympathetic innervation of these
Complications may include infarcts, hydrocephalus, and lobes and the posterior fossa structures.
unabated seizures. Epilepsy has been reported as a long-term 2. The increased occurrence of PRES in patients with
complication in a few cases. Recurrent forms of PRES have Neuromyelitis optica (NMO) and positive aquaporine-4
been reported in about 10% of cases. (AQP4) water channel autoantibodies have led to hypoth-
esis that an alteration in water flux due to AQP4 autoim-
Questions for Further Thought munity may predispose to PRES. There is no proof of
1. What is the reason of the parieto-occipital predilection of this yet.
PRES on imaging?

50
CLINICAL HISTORY 28-year-old right-handed male with history of attention
Case deficit and hyperactivity disorder (ADHD), learning and hearing problems, h istory
of exercise intolerance who presented numerous times to the hospital for recurrent
seizures.
104

Case 50 105
FINDINGS Figure 50-1. Axial MRI FLAIR through the MELAS is a mitochondrial disease characterized by the
occipital lobes. There is a left medial occipital lobe smudgy presence of myopathy, encephalopathy, lactic acidosis,
hyperintensity mainly cortical/subcortical in location stroke-like episodes, and seizures. It generally presents in
(arrows) at the time of initial presentation with seizures. Two late childhood but could present at any age. Associated
months later, he suffered another set of seizures. Figure50-2. morbidities include diabetes, endocrinopathies, cardiac
An axial FLAIR image from that time showing a right medial abnormalities, short stature, deafness, and neuropsychi-
occipital lobe cortical/subcortical smudgy hyperintensity and atric manifestations. The clinical findings are protean
sulcal effacement (arrows). There is resolution of initial left because of the ubiquitous presence of defective mito-
occipital hyperintensity. Figure 50-3. Axial DWI through the chondria in the cells. The pathophysiology of the disease
level of the thalamus 2 years after Figure 50-2 following new is not very well known. The mitochondria generate ade-
seizures. There is circumferential left hemispheric cortical/ nosine triphosphate (ATP) by oxidative phosphorylation
subcortical area of restricted diffusion (arrows) and smudgy (OXPHOS). Defects in OXPHOS lead to abnormal cellu-
hyperintensity with sulcal effacement and mass effect on lar metabolism (ATP production) in MELAS. The serum
FLAIR (not shown). There is subtle mild midline shift from lactate and Creatine phosphokinase (CPK) levels are gen-
left to right. Nine months later he returned with new episodes erally high as was the case in this patient. Cerebrospinal
of seizures. Figure 50-4. Axial FLAIR through the occipital fluid (CSF) protein is increased. The diagnosis is usually
lobes demonstrates a new right parietal, temporal, and occipi- confirmed by mitochondrial DNA analysis or by identify-
tal cortical/subcortical smudgy T2 hyperintensity with sul- ing the ragged red fibers with trichrome stain in muscle
cal effacement (arrows). There is restricted diffusion in these biopsy if the mitochondrial DNA testing is negative as was
regions. There is residual hyperintensity and volume loss done in this case. EM demonstrated an increase in number
(dilated left lateral ventricle and sulci) in the left hemisphere. and size of mitochondria.
DIFFERENTIAL DIAGNOSIS Multifocal seizure changes, Questions for Further Thought

multiple ischemic infarcts, mitochondrial myopathy, enceph- 1. What is the cause of morbidity and mortality in MELAS?
alopathy, lactic acidosis, and stroke-like episodes (MELAS), 2. What is the treatment for MELAS?
Leigh disease (LD).
DIAGNOSIS Mitochondrial myopathy, encephalopathy,
Direct reporting is necessary particularly with restricted dif-
lactic acidosis, and stroke-like episodes (MELAS).
fusion suggesting infarction. MRA could exclude occlusive
vascular disease. Significant midline shift should be reported
DISCUSSION MRI findings of MELAS are not spe-
since a lumbar puncture (LP) may be in the workup. The
cific. There are predominant cortical and subcortical areas
multifocal, fluctuating, and transient nature of these abnor-
of restricted diffusion and T2 hyperintensity in the parietal
malities on repetitive MRI should alert the radiologist to the
and occipital lobes; smudgy but could be well defined with
diagnosis.
features resembling cortical edema or laminar necrosis. The
changes tend to cross vascular territories which along with
sparing of deep white matter differentiate MELAS from What the Treating Physician Needs to Know
ischemic infarcts. Bilateral basal ganglia T2 hyperintensi- Location and size and the possibility of infarction
ties and calcifications make MELAS indistinguishable from Is LP feasible? Significant mass effect may contraindicate
LD. However, brainstem involvement which is unusual LP
in MELAS is a part of LD. Prolonged seizure activities What can advanced imaging tell us? Nothing specific!
(which this patient had) could result in extensive cortical
hyperintensity and sulcal effacement and indistinguishable Answers
from MELAS. Contrast enhancement has been reported in 1. MELAS shortens life expectancy. Morbidity and mor-
MELAS. Subsequently, encephalomalacia and volume loss tality are due to the encephalomyopathy, stroke-like
could be found on follow-up MRI in the affected areas. The episodes followed by hemiplegia and hemianopia, and
multifocal, fluctuating, predominantly posterior location clinically may progress to dementia, debilitating myop-
and transient nature of these abnormalities should suggest athy and intractable seizures. Cardiomyopathy, severe
the diagnosis. Proton MRS might be useful but not specific arrhythmias, aortic dissection, and renal failure contribute
in MELAS showing a decreased N-acetyl aspartate (NAA) to morbidity.
and increased lactate peak. One-third of the patients may not 2. There is no proven treatment for MELAS. Treatment is
show the lactate peak! Conversely an abnormal lactate peak supportive and do not change the natural outcome of the
might be present in unaffected family members. disease.

Case
51 CLINICAL HISTORY 45-year-old female with right facial numbness for years.
FINDINGS Figure 51-1. Axial NCCT through the petrous of foramen magnum. There is chronic smooth bony remodel-
apices, brain window. There is a well-marginated isodense ing of the right petrous apex and adjoining clivus (arrows).
mass (with streak artifacts) centered on the right petrous apex Figure 51-3. Axial T2WI through the mass. There is a hetero-
bulging into the middle cranial fossa and posterior fossa right geneous intensity pattern within the well-defined mass. There
cerebellopontine angle (CPA) (arrows) with mass effect on is no surrounding edema. Figure 51-4. Axial T1WI through
surrounding structures. Figure 51-2. Axial NCCT at the level the mass. The mass is homogeneously hyperintense (arrow).
106

Case 51 107
DIFFERENTIAL DIAGNOSISPetrous apex mucocele, imaging features of cholesterol granuloma so as not to mis-
congenital cholesteatoma, cholesterol granuloma. take it for infection or neoplasm.
DIAGNOSIS Cholesterol granuloma. What the Treating Physician Needs to Know

Size and extent of lesion
DISCUSSION CT usually shows an expansile sharply Hyperintensity on pre-contrast T1WIs clinches the diag-
demarcated erosion of the petrous apex and adjoining clivus nosis and usually precludes the need for further workup
by usually an isodense or hypodense non-contrast-enhancing or biopsy
mass. The mixture of cholesterol crystals and blood deg- Asymptomatic cases may be observed
radation products result in a very characteristic T1- and
As with other petrous apex pathology, cholesterol granu-
T2-weighted hyperintensity on MRI. Heterogeneity of the
loma may elicit a characteristic set of symptoms, including
intensity may be a feature on T2WI however. This lesion
headache, facial pain, and CN V and VI deficits
does not enhance.
Cholesterol granuloma is a benign and frequently asymp- Answer
tomatic reaction to chronic hemorrhage within an aer-
1. Two theories exist. The older, classic theory proposes
ated petrous apex. Granuloma formation results in chronic
that negative pressure develops in the pneumatized
expansile remodeling of the petrous apex. When it is suf-
petrous apex secondary to obstruction of the air cell out-
ficiently large as to abut the brain, it merely displaces but
flow pathway during bouts of otitis media. Repetitive
does not infiltrate. Lesions can become quite large and may
episodes of negative pressure cause small mucosal ves-
elicit symptoms due to encroachment on the adjacent brain
sels to rupture with collection of blood product in the
parenchyma and surrounding neurovascular bundles.
air cells. This blood then undergoes anaerobic degrada-
tion producing cholesterol crystals which in turn incite a
Question for Further Thought granulomatous reaction. A newer theory, quickly gaining
1. How does a cholesterol granuloma form? acceptance, suggests that aggressive pneumatization of
the petrous apex during development may leave areas of
Reporting Responsibilities marrow exposed to the air cells. This uncovered marrow
Direct reporting is necessary to ensure adequate understand- would be susceptible to repeated hemorrhage, from which
ing of what this mass represents. Identify the characteristic point granuloma formation ensues as in the classic theory.

Case
52 CLINICAL HISTORY 61-year-old male with headache. He was an outpatient
at the time of the CT!
FINDINGS Figure 52-1. Axial NCCT through the posterior lateral ventricles in a companion patient with SAH. There is
fossa. There is subarachnoid space (SAS) hyperdensity in bilateral scattered sulcal hyperintensity (arrows).
the posterior and middle cranial fossae (arrow). Figure 52-2.
Axial T2WI through the suprasellar cistern. There is diffuse DIFFERENTIAL DIAGNOSIS N/A.
hypointensity in the SAS (arrow). Figure52-3. Axial GRE
through the suprasellar cistern. There is diffuse hypointen- DIAGNOSIS Acute subarachnoid hemorrhage (ASAH).
sity/signal void in the suprasellar cistern and lateral fissures
(arrows). Figure52-4. Axial non-contrast T1WI through the DISCUSSION The classical NCCT sign of ASAH is sub-
suprasellar cistern. There is diffuse isointensity in the supra- arachnoid space hyperdensity. NCCT is the gold standard
sellar cistern (arrow). Figure 52-5. Axial FLAIR through the for the diagnosis of ASAH with a sensitivity of up to 100%
108

Case 52 109
(Figure52-5). The thinking used to be that NASAH is a benign

process compared to aneurysmal SAH. Complications in a
recent cohort of NASAH include early hydrocephalus 25%,
late hydrocephalus 13%, vasospasm 4%, infarction 2%, and
death 3%. Follow-up angiograms usually show aneurysm in
about 24%. It is therefore essential that a repeat angiogram,
CTA, MRA, or DSA be performed. There is controversy as
to how soon the follow-up angiogram should be performed
but usually within 4 weeks. If that is negative, then another
follow-up between 2 and 6 months may be desirable.

1. What is the cause of NASAH?
This is an acute situation requiring direct reporting. The
extent of the hemorrhage and recommendation for angio-
gram should be mentioned. Visible cause such as venous
Figure 52-5
thrombosis, arteriovenous malformation (AVM), cerebral
cavernous malformation (CCM), or dural arteriovenous fis-
within the first 6 hours. The location and size of the hem- tula (DAVF) on the MRI should be mentioned.
orrhage may influence the sensitivity of CT. About 10% of
SAH may not be visible on CT after 24 hours. There has been What the Treating Physician Needs to Know
an emphasis on FLAIR MRI sulcal hyperintensity for dem- Location of hemorrhage
onstrating this group of CT-negative SAH. As seen in this Other associated findings such as intraventricular hemor-
case, acute SAH could be visible on other MRI sequences. rhage (IVH), hydrocephalus, parenchymal hemorrhage, or
There is SAS isointensity on T1WI, hypointensity on T2WI, focal clot that may suggest location of aneurysm, paren-
and hyperintensity on FLAIR. The location of the SAH is chymal changes that may point in the direction of a cause
predominantly posterior fossa in this case with some diffu- of the hemorrhage
sion into the convexity sulci. The logical follow-up evalua- Recommendation for finding out the cause of the SAH:
tion of SAH is either CTA or MRA, both of which are equally CTA or MRA
effective in uncovering the aneurysmal origins of most non-
traumatic SAH. If either of this fails to show the cause, DSA Answer
is the next logical examination. 1. Repeat angiograms show aneurysm in about 24% of
Initial CTA and DSA were negative for aneurysm in this NASAH. These aneurysms may not be visible initially
patient. A follow-up CTA showed extensive vasospasm but because of their size, presence of spasm, or complex anat-
no aneurysm. MRI showed multifocal infarcts. A follow-up omy of their locations. Other possible causes may include
DSA did not reveal any aneurysm. This therefore is one form occult AVM, CCM, telangiectasia, coagulopathy, venous
of nonaneurysmal subarachnoid hemorrhage (NASAH). thrombosis, pituitary apoplexy, arterial dissections, spinal
NASAH forms about 15% of all nontraumatic SAH. The AVM, and DAVF. DSA in NASAH should always include
location of the hemorrhage is predominantly posterior fossa external carotid injections to exclude DAVF. However,
and around the midbrain into the suprasellar region in this the cause of the hemorrhage is never found in the major-
case with some convexity diffusion in the companion patient ity of NASAH.

Case
53 CLINICAL HISTORY 65-year-old female with acute onset of left-sided
hemiparesis.
110

Case 53 111
the hallmark of cytotoxic edema. The most common cause

of restricted diffusion and cytotoxic edema is hyperacute/
acute ischemic infarct. Restricted diffusion is evident within
the first 30 minutes of the ictus. The changes are replicated
on the spin echo sequences of T1WI as diffuse hypointen-
sity and on the FLAIR and T2WI as hyperintensity. These
changes may take up to 2 hours to manifest on the spin echo
MRI sequences and about 6 hours to see the correspond-
ing hypodensity on NCCT. Hence, the DWI is crucial to
making an early diagnosis of hyperacute infarction. It has
been well said that not everything that shines on DWI is
infarct. Subacute or chronic infarcts or chronic demyelinat-
ing lesions may present as hyperintensity on DWI but nor-
mal or hyperintensity on ADC map. As such subacute and
chronic infarcts do not restrict diffusion. The ADC map
is crucial to making that decision. This scenario is known
as T2 shine through. Some vasogenic edema may fall into
this category. Vasogenic edema does not restrict diffusion.
It is essential to have the matching hypointensity on the
ADC map to call it restricted diffusion. Hypoxic ischemic
encephalopathy, seizures, and mitochondrial enzymopathies
produce restricted diffusion. There are also other entities that
restrict diffusion without cytotoxic edema! These include
hypercellular tumors such as lymphoma, some areas within
Figure 53-5 high-grade tumors such as glioblastoma (GB), some acute
demyelinating lesions, toxins such as methotrexate toxicity,
sporadic Creutzfeldt-Jakob disease (CJD), abscesses (core)
and ventriculitis, and acute hemorrhage. These lesions do not
FINDINGS Figures 53-1 and 53-2. Axial DWI with cor-
follow vascular territories like infarcts do and some of them
responding ADC map through the level of the basal gan-
enhance following contrast administration.
glia. There is diffuse cortical and subcortical hyperintensity
In contrast to vasogenic edema, the bloodbrain bar-
on the DWI with matching hypointensity on the ADC map
rier (BBB) remains intact in cytotoxic edema. In cytotoxic
(stars) in the right temporoparietal lobes with involvement
edema of acute infarction, there is disruption of the normal
of the right operculum and subinsula region. There is efface-
Na/K pump within the cell membrane due to a global energy
ment of the sulci in the region involved consistent with mass
failure. This results in relative increased Na within the cell,
effect. Figures 53-3 to 53-5. Axial T2WI, FLAIR, and T1WI,
and through osmosis increased intracellular water. This is
respectively, through the area. There is confluent hyperinten-
reflected as edema in both the GM and WM, in contrast to
sity involving the gray matter (GM) and white matter (WM)
only WM involvement in vasogenic edema. Since this results
of the region on the FLAIR and T2WI with corresponding
in reduced diffusivity or motion of the water molecules in
hypointensity on T1WI (stars). Mass effect is manifested by
both the extracellular space (less water and less space due
effacement of the sulci and mild compression of the right
to adjacent swollen cells) and the intracellular space (inside
lateral ventricle with subtle midline shift.
the cells organelles create barriers to movement of water)
the affected regions show hypointensity on the ADC maps.
DIFFERENTIAL DIAGNOSIS Vasogenic edema, cytotoxic
In infarcts, this onset is rapid and the low ADC values can
edema.
last for 10 to 15 days. The clinical presentation of cyto-
toxic edema is that of underlying infarct: focal neurologic
DIAGNOSIS Cytotoxic edema secondary to right middle
deficit either sensory or motor or both and if the infarction
cerebral artery territory acute infarction.
is sufficiently large, alteration of mental status, and coma.
Treatment is directed to the cause of the edema.
DISCUSSION Imaging of cytotoxic edema is best accom-
plished by MRI. The various sequences contribute some
information to the diagnosis but the most important sequence Questions for Further Thought
is the DWI with its ADC maps. The classical changes are 1. Is restricted diffusion synonymous with cytotoxic edema?
matching hyperintensity on the DWI and hypointensity on 2. What is more likely to be reversible? Cytotoxic or vaso-
the ADC map consistent with restricted diffusion. This is genic edema?

112 Case 53
Reporting Responsibilities What further evaluation is necessary to elucidate on the

Direct reporting is essential as all causes of cytotoxic edema cause of restricted diffusion?
should be treated promptly. In the setting of infarct, describe
location, size, stage, vascular territory, and associated abnor- Answers
malities such as hemorrhage and herniation. If appropriate, 1. No. Several processes other than cytotoxic edema (as
suggest most likely etiology of infarct and further workup seen in infarcts) can result in restricted diffusion or simu-
needed. late restricted diffusion such as abscesses due to hyper-
cellularity and high protein content of pus, highly cellular
What the Treating Physician Needs to Know tumors (i.e., lymphoma, medulloblastoma), vacuolation
In the setting of infarct, the territory, size, and stage of the as in CJD and mitochondrial enzymopathy, intramyelinic
infarct edema in demyelination, and blood products.
Presence of hemorrhage may affect how the infarct is 2. Cytotoxic edema is generally thought to be irreversible
treated due to permanent cellular damage. In contrast, vasogenic
If the restricted diffusion is not infarct suggest what you edema is more likely to be reversible because it is due to
think it is a breakdown in the BBB and not the cell itself.

Case
54 CLINICAL HISTORY 58-year-old patient presents with long-standing exophthalmos
and decreased visual acuity of the left eye, left ophthalmoplegia (involvement of the III,
IV, and VI cranial nerves), and hypopituitarism.
Figure 54-2
Figure 54-1
FINDINGS Figure 54-1. Axial NCCT through the sella tur- sinus opacification and osteoneogenesis. Figure54-3. Sagittal
cica. There is a mild-to-moderate hyperdense mass occupying non-contrast T1WI shows a large isointense homogeneous
the left parasellar region with linear calcifications in its lateral sella mass that erodes the clivus and extends posteriorly
margin (arrow). Figure 54-2. Coronal bone window NCCT into the interpeduncular and pre/pontine cisterns (arrows).
through the anterior clinoid processes. There is hyperostosis Figure54-4. Axial post-contrast T1WI through the mass.
especially of the left anterior clinoid process and greater wing There is an avidly enhancing mass centered in the left para-
of the sphenoid bone (vertical arrows) and lytic areas in the left sellar region and extends to the sella turcica. The mass com-
sphenoid sinus wall (transverse arrow). There is a left s phenoid presses the left temporal lobe laterally (transverse arrow) and
113

114 Case 54
in the sellar region after pituitary adenomas. Meningiomas

may arise from the suprasellar (tuberculum sella, anterior
clinoid processes, planum sphenoidale, upper clivus, and
diaphragm sella), parasellar (cavernous sinus), or intrasellar
regions (diaphragm sella).
The clinical presentation depends on the meningio-
mas location and extension and is generally related to the
involvement of the surrounding cranial nerves and vascular
structures. Intrasellar meningiomas may mimic a nonfunc-
tioning adenoma, presenting with visual symptoms without
pituitary dysfunction. Parasellar meningiomas can cause uni-
lateral loss of vision and diplopia as they affect the ipsilateral
optic nerve and the different cranial nerves that innervate
the extrinsic ocular muscles (III, IV, and VI), a constella-
tion of findings referred as the superior orbital fissure syn-
drome. As parasellar meningiomas invade the orbit through
the superior orbital fissure and obstruct the orbital draining
venous system or the cavernous sinus, exophthalmos appears.
Furthermore, extension toward the cavernous sinus, Meckel
cave, or the sella turcica may result in a cavernous sinus syn-
Figure 54-5 drome and all branches of the trigeminal nerve (CNV) are
involved. Suprasellar meningiomas commonly cause mon-
ocular vision loss that evolves to a binocular impairment as
the brainstem posteriorly (vertical arrow). The left internal the asymmetric and slow growing lesion displaces the optic
carotid artery (ICA) is narrowed and encased by the mass nerve or chiasm. Other associated symptoms include head-
(triangle). There is a left orbital proptosis. Figure54-5. aches, seizures, and mental status changes.
Coronal post-contrast T1WI through the sella. There is inva-
sion of both cavernous sinuses and the sella by the mass. Question for Further Thought
There is a left middle cranial fossa floor dural tail (arrow).
1. What syndrome is associated with meningiomas of the
tuberculum sella and is characterized by ipsilateral optic
DIFFERENTIAL DIAGNOSIS Parasellar schwannoma, pitu
atrophy and contralateral papilledema?
itary adenoma, chordoma, meningeal metastasis, neurosarcoid,
Rosai-Dorfman disease, meningioma, lymphoma.
Direct reporting is essential in view of the neurovascular and
DIAGNOSIS Parasellar meningioma.
critical structures involved. Evaluate caliber of ICA since
unlike other sellar and parasellar lesions, the ICA can be nar-
DISCUSSION On CT, meningiomas typically appear as
rowed when encased by meningiomas. Describe extension
extraaxial homogeneously hyperdense masses with avid
and involvement of the cavernous sinus, suprasellar cistern,
contrast enhancement with occasional intramural calcifica-
optic canal, or Meckel cave. Since there is a high prevalence
tions, and hyperostosis of the adjacent bone. Expansion of
of multiple meningiomas, it is prudent to exclude multiple
the underlying sphenoid sinus (pneumosinus dilatans) is not
meningiomas.
uncommon. Hyperostosis of the sella floor is typical of para-
sellar meningioma. On MRI, they are hypointense to isoin-
tense to gray matter (GM) on both T1WI and T2WI. They
show rounded dural margins and cerebrospinal fluid (CSF) Location and extension of the lesion and certainty of the
clefts around them. An important imaging feature is a linear diagnosis
dural tail of enhancement suggesting their extraaxial origin. Base of skull meningiomas may benefit from preoperative
They are homogeneously solid tumors but may occasion- embolization
ally contain areas of necrosis, scars, cysts, and calcifications.
Post-contrast CT and MRI reveal intense homogeneous Answer
enhancement. Angiography shows a dense tumor blush. 1. The Foster Kennedy syndrome is a rare condition asso-
Most of the differentials can mimic meningioma. Metastasis ciated with frontobasal intracranial lesions such as cra-
rarely gives rise to hyperostosis. Lymphoma tends to restrict niopharyngioma, meningioma, pituitary adenoma, and
diffusion because of its hypercellularity. sphenoid wing masses such as meningiomas. It consists
Meningiomas are mostly benign slow-growing WHO I of unilateral or ipsilateral optic atrophy produced by
tumors that originate from dural surfaces presumably from direct pressure on the optic nerve by the mass, contralat-
arachnoid cap cells and are the most common primary non- eral papilledema secondary to raised intracranial pressure,
glial intracranial tumor and the second most frequent lesion central scotoma, and anosmia.

Case
55 CLINICAL HISTORY 59-year-old male with headache.
FINDINGS Figure 55-1. NCCT through the dorsum sella. h yperdensity seen on the NCCT. Figure 55-4. Digital sub-
There is asymmetrically increased density along the left traction angiography (DSA). Left internal maxillary artery
petroclinoid ligament and anterior tentorium edge (trans- injection. The DAVF is supplied by the middle meningeal
verse arrows). There is a more focal appearing hyper- artery with early shunting into the varix (arrow) and drain-
density slightly lateral and posterior to the left ambient age into the vein of Galen and straight sinus. Tiny menin-
cistern (vertical arrow) associated with a medial tubular geal feeders are seen corresponding to the CTA fi ndings in
density overlying the midbrain. Figures 55-2 and 55-3. Figure 55-3.
CT angiography with axial and sagittal MIP reconstruc-
tions. There are numerous small caliber vascular structures DIFFERENTIAL DIAGNOSIS Dural arteriovenous fistula
extending from the left petroclinoid ligament posteriorly (DAVF)/malformation. Pial arteriovenous malformation
along the left medial edge of the tentorium (short white (AVM), aneurysm.
arrows). There is a large venous varix (long white arrow)
adjacent to the left midbrain and pons corresponding to the DIAGNOSIS DAVF/malformation.
115

116 Case 55
DISCUSSION On NCCT, DAVF may be suspected on transverse or sigmoid sinus may result in pulsatile tinnitus,
the basis of enlarged extraaxial hyperdensities suggesting and patients with carotid cavernous DAVFs may present
a vascular origin or the presence of transosseous calvarial with pulsatile exophthalmos, chemosis, and cranial nerve III,
vascular channels on bone window CT (such as an enlarged IV, and/or VI neuropathy.
foramen spinosum containing an enlarged middle menin- Based on clinical course, DAVFs can be divided into
geal artery). NCCT is useful to rule out subarachnoid hem- benign and aggressive types, with the latter demonstrat-
orrhage and may also demonstrate cerebral edema, both ing retrograde venous drainage. The most commonly used
potential complications of DAVF. Enhanced CT and CTA DAVF classification system is the Borden classification
demonstrate abnormal intracranial arterial and venous struc- that divides DAVFs into three types: (1) venous drain-
tures without a parenchymal nidus. In our case, note that the age directly into dural venous sinus or meningeal vein,
abnormal vascularity involves the tentorium (extraaxial) and (2)venous drainage directly into dural venous sinus with
shows a varix with mass effect upon the brainstem. CTA cortical venous reflux, and (3) venous drainage directly into
can demonstrate stenotic, partially thrombosed or occluded subarachnoid veins (cortical venous reflux only). Cortical
dural venous sinuses, often associated with collateral (abnor- venous reflux, reversal of flow in cortical veins with venous
mal venous channels and varices). When abnormal cortical hypertension, cerebral edema, and risk of parenchymal
venous drainage of the fistula is present (cortical venous hemorrhage is the most ominous finding that must not be
reflux), one may identify a plethora of abnormal vascular missed on imaging.
channels, transcortical in location without clear organization
or parenchymal nidus, an ominous finding. On MRI, one Questions for Further Thought
may also note arterialized, dilated cortical veins without a 1. What are the most diagnostic cross-sectional imaging fea-
parenchymal nidus and abnormalities of the dural sinuses on tures of DAVF?
T1- and T2-weighted sequences with or without flow voids. 2. What is the choice of therapy for DAVFs?
Angiography remains the gold standard for diagnosis and
treatment planning, demonstrating multiple dural arterial Reporting Responsibilities
feeders (most commonly dural/transosseous branches from Direct reporting is essential. Timely diagnosis is important;
external carotid artery), arterial inflow into parallel venous as findings of a thrombosed sinus, prominent varices, abnor-
channels, thrombosis, and flow-reversal involving dural mally dilated cortical veins, and cerebral edema relate to
sinuses/cortical veins. patients who are at risk of (fatal) hemorrhage may require
DAVFs are vascular lesions that are characterized by emergent intervention. CT/CTA and MR/MRA may be nor-
numerous tiny fistulous connections between branches of mal with small DAVFs, and DSA should be recommended
dural arteries with dural veins, cortical veins, or a venous for confirmation, especially if given the clinical history of
sinus. Representing 10% to 15% of all cerebral vascular objective pulsatile tinnitus.
malformations with arteriovenous shunting, DAVFs are usu-
ally acquired and idiopathic, although there may be ante- What the Treating Physician Needs to Know
cedent trauma or dural venous sinus thrombosis. They can
Location and suspected pattern/route of venous drainage
occur anywhere within the intracranial dura, although they
are most common at the transverse, sigmoid, and cavern- Ominous findings: sinus thrombosis, intracranial hemor-
ous sinuses. Abnormal microvascular fistulous connections rhage, cerebral edema, venous infarction, dilated cortical
within the walls of the involved dural venous sinus results in veins
arteriovenous shunting and arterialization of the sinus may
ultimately interfere with normal parenchymal venous drain- Answers
age (due to venous hypertension in the involved sinus). This 1. Abnormal vascular structures along dural surfaces with-
relative obstruction to normal venous drainage may lead to out parenchymal nidus, associated findings include dural
parenchymal venous hypertension and cerebral edema due to venous sinus thrombosis, transosseous venous channels,
retrograde venous drainage from the sinus into dilated corti- arterialized venous structures on MRA, dural arteriove-
cal veins, predisposing a patient to intracranial hemorrhage. nous shunting on DSA.
Another mechanism that may promote DAVF evolution is 2. A conservative approach to the treatment of DAVF
pathologic activation of neoangiogenesis by growth factors without cortical venous reflux (Borden type 1) includes
such as vascular endothelial growth factor (VEGF) and basic observation and Gamma Knife Radiosurgery alone.
fibroblast growth factor (bFGF). Spontaneous resolution has occasionally been reported.
The symptoms of DAVFs vary depending on location For the more dangerous Borden type 2 and 3 DAVF,
and route of venous drainage. Patients may be completely transvenous or transarterial endovascular approaches
asymptomatic or may present with bruit, headache, or even have been employed, alone or in combination with open
fatal hemorrhage. A petrous region DAVF draining into the surgery or Gamma Knife Radiosurgery.

Case
56 CLINICAL HISTORY Young adult presenting with headaches, decreased vision
acuity, and hypopituitarism.
in the suprasellar region probably due to blood/protein in the

fluid. Figure 56-3. Post-contrast sagittal T1WI. The mass is
multiloculated (star) with enhancement of the cyst walls and
septations with compression of the third ventricle and the
midbrain (vertical arrow). The sella turcica is normal (trans-
verse arrow), indicating suprasellar location of the mass.
DIFFERENTIAL DIAGNOSIS Rathke cleft cyst, Craniopha

ryngioma (CP), suprasellar arachnoid cyst, hypothalamic
astrocytoma, pituitary adenoma, dermoid or epidermoid
tumors, thrombosed aneurysm, and germ cell tumors.
DIAGNOSIS Craniopharyngioma (CP).
DISCUSSION Most CPs are located in the suprasellar

region causing optic atrophy and/or increased intracranial
Figure 56-3 pressure as they protrude into the third ventricle and exert
mass effect on the chiasm and hypothalamus. On CT and
MRI, CPs show typical heterogeneous cystic and solid-
FINDINGS Figure 56-1. Axial NCCT through the suprasel- enhancing components. Cystic components are seen predom-
lar region. There is a suprasellar predominantly cystic mass inantly in children and solid ones in adults. Calcifications
with central calcifications (transverse arrow). There is dila- occur in up to 80% of tumors and are more common in the
tion of the trigones (vertical arrow) consistent with hydro- adamantinomatous subtype. On MRI, the solid parts are usu-
cephalus. Figure 56-2. Axial FLAIR through the suprasellar ally isointense to hypointense on T1WI and hyperintense
region. There is a hyperintense multicystic lobulated mass on T2WI. The cystic parts of adamantinomatous tumors are
117

118 Case 56
hyperintense on both T1WI and T2WI corresponding to a Questions for Further Thought
highly proteinaceous fluid. Edema along the proximal aspect 1. What are the two imaging hallmarks of CP?
of the optic radiations is typical and is thought to occur due to 2. What is a sign affecting the optic nerve that is specific
obstruction of the perivascular spaces in that location. Rathke forCP?
cleft cyst is usually unilocular (could have multiple internal
cysts) with a mural nodule but does not calcify nor contrast
enhance. Arachnoid cyst follows cerebrospinal fluid (CSF) Reporting Responsibilities
density or intensity pattern unless there has been the rare Direct reporting is essential particularly with hydrocephalus
hemorrhage into it. Arachnoid cyst does not calcify. Pituitary and because of its effect on the visual pathways. Describe
adenoma originates in the sella turcica. As such, enlargement the extent of the mass (pre- or retrochiasmatic) and rela-
of the sella is present in pituitary adenoma. Enhancing septa- tion with other neighboring vascular and nervous structures.
tions would be unusual in thrombosed aneurysm. Dermoids Assess the relationship of the tumor to the arteries and pitu-
and epidermoid tumors do not contrast enhance. itary gland. There should be a comment on the presence or
CPs are slow-growing WHO I tumor that is locally absence of hydrocephalus.
aggressive. It originates from squamous epithelial cell rests
of Rathke pouch. There are two main subtypes: adamantino-
matous and squamous papillary, both with different age of
presentation, tumor location, imaging features, and histologic Total geography of the lesion to assess the possibility of a
characteristics. The squamous papillary subtype is essentially complete surgical resection
seen in older adults, while the adamantinomatous occurs Presence of hydrocephalus
in children. In children, the clinical presentation includes CPs have been known to extend to the nasopharynx and
growth and sexual development retardation, obesity, and the nasal passages. Exclude lesions in these locations
hydrocephalus. Raised intracranial pressure, visual changes, Is it mostly cystic or solid?
and hormonal changes are the usual presentations in adults.
Treatment is usually surgical. Complete resection is impos-
sible in most cases and may carry a high morbidity as neigh- Answers
boring vascular and neural structures may be injured during 1. The two major imaging hallmarks of CPs are calcifica-
surgery. The management strategy is based on surgical resections and cystic components.
tion followed by radiotherapy. Cystic CPs have been treated 2. Edema spreading along the optic tract is said to be a spe-
with ommaya reservoir system with cyst aspiration, with or cific sign of a CP but can occasionally be seen with large
without injection of sclerosing agent with excellent result. pituitary macroadenomas.

Case
57 CLINICAL HISTORY 48-year-old male with traumatic brain injury.
119

120 Case 57
FINDINGS Figure 57-1. Axial NCCT through inferior fron- and seizures. Surgical intervention may be indicated if there
tal lobes. There is a 1.5-cm round hyperdensity surrounded is significant mass effect or herniations depending on other
by a hypodense halo in the left frontal lobe just posterior variables. Patient outcome depends very much on the age
and superior to the left orbital roof (arrow) consistent with of the patient and admission Glasgow Coma Scale (GCS)
hematoma. There are contusions in the right frontal and left score; the higher the GCS score, the better the outcome.
temporal lobes. Figure 57-2. Axial NCCT through the frontal
lobes 6 months after Figure 57-1. The area of hematoma is Question for Further Thought
now represented by a well-defined hypodensity (encepha- 1. What is responsible for the delayed and expanding TICH
lomalacia) significantly smaller than the original lesion in traumatic brain injury?
(arrow). Figures 57-3 and 57-4. Axial NCCT 24 hours apart
in another patient. There are new and expanding hematomas Reporting Responsibilities
(arrows in Figure 57-4) on follow-up. There is significant TICH is an emergency requiring direct reporting. Location and
brain swelling, mass effect, and herniation. number if more than one should be categorized. Associated
complications such as swelling, compartmental shifts or
DIFFERENTIAL DIAGNOSIS N/A. herniations, and other associated primary injuries should be
enumerated. Significant changes on follow-upshould also be
DIAGNOSIS Traumatic intracerebral hematoma (TICH). communicated directly.
DISCUSSION TICH is a hyperdense mass on CT. TICH is What the Treating Physician Needs to Know
usually superficial and more common in the frontal and tem- Location and number if more than one
poral lobes than elsewhere. They are rarely deep seated. A Other primary injuries such as contusions, extraaxial hem-
hypodense halo of edema is usually present. The hematoma orrhages, and intraventricular hemorrhages
and surrounding edema frequently increase in size over the Complications and secondary injuries such as brain swell-
following few days. New foci or so-called delayed hematoma ing, infarcts, herniations, hydrocephalus, expansion, and
may develop up to 15 days after the initial injury. Presence of new hematomas on follow-up
mass effect depends on the size of the hematoma and its sur-
rounding edema. While CT is usually the method of choice for Answer
evaluation of acute TICH, MRI is more sensitive in the sub- 1. The incidence of delayed traumatic intracerebral hema-
acute and chronic stages of the hematomas and could show toma (DTICH) is less than 10% in most series. DTICH
many more smaller hematomas using various sequences. The can be superficial or deep and may occur up to 15 days
MRI presentation depends on the age of the hematomas. following the initial insult. It may or may not be heralded
TICH is due to shearing or rapid deceleration injury, by new symptoms or deterioration of clinical status. It
resulting in rupture of superficial/subcortical blood ves- has been observed to occur in patients with or without
sels with extravasation of blood into the brain parenchyma. decompressive surgery. Several theories for the forma-
A large hematoma can cause secondary brain damage by tion of new and expanded hematoma include presence
compression and displacement or herniation of surround- of existing injury such as necrosis and vascular injury
ing structures. TICH is invariably associated with other with subsequent coagulopathy, presence of infarction
primary intracranial injuries. Follow-up imaging is usu- with secondary hemorrhagic conversion, loss of local
ally necessaryfor assessment of the status of the hematoma autoregulation resulting in coalescing small hemor-
particularly when the patient is not doing well. The natu- rhages, and venous congestions resulting in blood exu-
ral history is that of a gradual resorption and transforma- dation among others. Secondary hypoxic insult is more
tion from hyperdensity to hypodensity. The end result is a common in this subset of patients. DTICH is therefore
much smaller focal encephalomalacia and volume loss while regarded as an epiphenomenon likely to represent hem-
smaller hematomas may completely disappear. The clinical orrhage into an existing traumatized area and could be
presentation depends on size, number, and other associated ascribed to local hypoxia and hypercapnia following
injuries. It could include headaches, focal neurologic deficit, the vascular damage.

Case
58 CLINICAL HISTORY 35-year-old female with paraesthesia in bilateral
upper extremities.
Figure 58-4
FINDINGS Figure 58-1. Sagittal MR T1WI. There is a

very small posterior fossa with a very small cerebellum
extending through wide foramen magnum into the upper
cervical spinal canal (transverse thick arrows). The ver-
tical thin arrow points to the low position of the torcula.
There is a pointed (beaked) tectum (thin transverse arrow).
The fourth ventricle is tubular and continuous inferiorly
Figure 58-3 into the upper spinal canal with no visible fastigium (star).
121

122 Case 58
ventricle without a visible fastigium, wrapping of the cer-

ebellum around the brainstem resulting in anteroposterior
orientation of cerebellar folia, beaking of the tectum with
flattening of basis pontis, and bundling of the vermis as it
pushes through the tentorial incisura. The posterior petrosal
surface is concave. The sagittal and axial T1WI and T2WI
demonstrate these abnormalities very well. The supratento-
rial abnormalities include hydrocephalus in over 90% and
corpus callosum dysgenesis in over 60%. Deficient falx with
interdigitation of cerebral hemispheres across the midline,
sulcal, and cortical malformations such as hypoplasia, het-
erotopia, pachygyria, and polymicrogyria; colpocephaly;
and large massa intermedia are common. Symptomatic
hydrocephalus is considered a complication of repair of the
associated myelomeningocele (MM). Once the hydrocepha-
lus is shunted, there could be dilatation of midline subarach-
noid spaces around the incisura. Lacunar skull is always a
feature of CIIM in babies and is best demonstrated by CT.
The spinal malformations are best evaluated by spinal MRI
and include open dysraphism with MM in virtually 100% of
patients, tethering of the spinal cord and syringohydromyelia
Figure 58-5
in over 50%. Changes of CIIM are conveniently evaluated in
utero by US and MRI. This may allow in utero intervention
which has been shown to improve outcome in these children.
Thebody/splenium of the corpus callosum is severely atro- CIM is not associated with the constellation of supratentorial
phic (chevron). Parasagittal cerebral dysgenesis is present changes and MM and usually, apart from cerebellar tonsil-
(right left arrow). The massa intermedia (heart) is large. lar ectopia, there is no significant cerebellar or brain stem
Figure 58-2. Axial T2WI through the small posterior fossa. abnormality. Lack of cerebellar fusion excludes RES, while
There is anteroposterior orientation of the cerebellar folia occipital bone defect with protruding cerebellum is always
(transverse arrows). There is concavity of posterior petro- present in OC.
sal surface. Figure 58-3. Axial FLAIR through the lateral These children are born with open spinal MM. It is more
ventricles. There is dysgenesis of the parasagittal occipi- common in females than males. CIIM may present with
tal lobes and dilation of the posterior interhemispheric fis- lower limb paralysis, developmental delay, hindbrain symp-
sure (star). There is scalloping of the ventricular walls with toms of cranial nerve palsies, and respiratory problems.
periventricular hyperintensities due to leukomalacia (trans- Spasticity and bladder dysfunction are subsequent prob-
verse arrows). There is bilateral cortical malformations, a lems. Incidence of CIIM is decreasing due to prophylactic
mixture of pachygyria and polymicrogyria. Figure58-4. folate therapy during pregnancy. Folate deficiency and use of
Axial T2WI through midbrain. Beaked tectum protrudes anticonvulsants during pregnancy have been linked to open
into dilated quadrigeminal cistern (star). Figure58-5. spinal dysraphism. It is suggested that constant leak of CSF
Sagittal cervical spine T2WI. There is inferior descent from open MM result in lack of intracranial CSF space dis-
of the medulla, the fourth ventricle, and the cerebellum tention crucial to brain and cranial vault development. This
through the large foramen magnum into the upper cervical lack of distention results in developmental domino effect
spine (thin arrows). There is a cervical thoracic syringomy- leading to hypoplasia, maldevelopment, and malposition
elia from about C3C4 disc level to the visualized upper of various posterior fossa and supratentorial structures. In
thoracic spine (transverse thick arrows). utero or early postnatal repair of the MM has been shown to
improve outcome.
DIFFERENTIAL DIAGNOSISChiari II malformation
(CIIM), CIM, occipital cephalocele (OC), rhombencephalo- Questions for Further Thought
synapsis (RES). 1. Is there a genetic basis for CIIM?
2. Is there a screening method for detection of fetal CIIM?
DIAGNOSIS Chiari II malformation (CIIM).
DISCUSSION The focus of the malformation is in the pos- Discovery of CIIM either on fetal US/MRI or in postnatal
terior fossa and the changes include a small posterior fossa imaging requires direct reporting for prompt treatment which
with low attachment of the torcula and transverse sinuses, has been linked to improved outcome. Evaluation of CIIM
descent of the medulla, fourth ventricle and the cerebellum is incomplete without evaluation of entire craniospinal axis,
into the upper cervical spinal canal, elongated tubular fourth and this should be recommended.

Case 58 123
What the Treating Physician Needs to Know Answers

Complete head and spine MRI is crucial for initial evalu- 1. Yes. Mutation of methylene-tetra-hydrofolate reduc-
ation of CIIM and subsequent evaluation with any new tase (MTHFR) gene is common in neural tube closure
symptoms anomaly. Prophylactic folate therapy during pregnancy is
CT may be enough for follow-up of shunted hydro- known to reduce this risk.
cephalus 2. Yes. Maternal serum and amniocentesis testing for ele-
Other associated anomalies as detailed above vated -fetoprotein and fetal US.

Case
59 CLINICAL HISTORY 66-year-old female woke up from sleep with left lower
extremity weakness.
FINDINGS Figure 59-1. Axial NCCT through the vertex. through the vertex. There is an ovoid hypointense (blooming)
There is a 1.5 cm 1.2 cm subcortical right frontal para- mass in the right frontal premotor cortex (arrow). Figure59-3.
sagittal premotor well-defined hyperdensity with surround- Axial GRE through the upper pons. There are multifocal
ing hypointense halo consistent with acute hemorrhage with punctate hypointensities in the pons and bilateral cerebral
surrounding edema (arrow). Figure 59-2. Axial MR GRE hemispheres (arrows) consistent with microhemorrhages.
124

Case 59 125
Figure 59-4. Axial FLAIR through the centrum semiovale. familial cases occurring about 10 years earlier. It is more
There is bilateral almost symmetrical confluent WM hyper- common in Alzheimer disease (AD). CAA-related hema-
intensity (arrows). tomas may be asymptomatic but when large could pres-
ent with focal neurologic deficits, mental status changes,
DIFFERENTIAL DIAGNOSIS Cerebral cavernous malfor- headache, and coma. Definitive diagnosis of CAA can
mation (CCM), hypertensive intracerebral hematoma (ICH), only be made by histology. However, four clinical grades
amyloid angiopathy-related ICH, hemorrhagic metastasis. of CAA have been proposed. These are definitive CAA,
probable CAA with pathology, probable CAA and possible
DIAGNOSIS Right frontal lobar hematoma with microhe- CAA depending on available clinical and pathologic evi-
morrhages. dence. PET imaging with beta-amyloid-binding Pittsburgh
Compound B (11C PiB) has improved the clinical detec-
DISCUSSION NCCT offers the fastest and most efficient tion of CAA in a noninvasive manner. CAA is character-
way to image acute hematoma which is usually hyperdense. ized by progressive deposition of amyloid beta-protein in
The microhemorrhages, however, are not seen on CT. It medium- to small-sized arteries and penetrating arterioles.
usually requires MRI particularly the GRE to demonstrate The histologic hallmark includes severely disrupted vas-
these punctate to 1-cm hypointense foci. Hence MRI fur- cular architecture, double barreling, microaneurysm for-
ther characterizes the pattern of the hematoma. In this age mation, fibrinoid necrosis, and evidence of perivascular
group presence of a primary lobar hematoma always raises hemorrhage. There may be an association between aspirin
the suspicion of cerebral amyloid angiopathy (CAA). CAA- and statin use and CAA-related ICH recurrence. There is
related lobar hematoma represents between 10% and 34% no known treatment for CAA.
of primary ICH and tends to be lobar in location, due to
the involvement of superficial cortical and leptomeningeal Question for Further Thought
vessels. They are often recurrent or multiple simultaneous 1. What is the pathologic basis of the GRE hypointensity
hematomas. CAA hematomas are most frequent in the occip- associated with microhemorrhage?
ital lobe, followed by frontal, temporal, or parietal lobes,
respectively. Spontaneous bleeding due to CAA can also be
small and asymptomatic and is one of the causes of remote
Acute hemorrhage requires direct reporting. All complica-
microhemorrhage.
tions and associated findings should be reported.
Differential diagnosis of microhemorrhages includes
chronic hypertension and coagulopathy. Location of the
microhemorrhages may shed more light upon possible cause What the Treating Physician Needs to Know
of the hematoma. Since CAA is rarely found in the basal Location and size
ganglia, thalamus, brainstem, and cerebellum, presence of Associated findings and complications
microhemorrhages in these locations may suggest chronic There are no pathognomonic findings of CAA on imaging
hypertension. Symptomatic hypertensive hemorrhages are
more common in these locations. Hemorrhagic infarcts due Answer
to venous sinus thrombosis may be lobar in location but could 1. The histology of these punctate to small GRE hypointen-
occur in the thalamus. Hemorrhagic metastases are usually sity indicates previous extravasation of blood and is related
juxtacortical in location with surrounding edema. CCMs to bleeding-prone microangiopathy of different origins.
Zabramski 4 are usually multiple punctate blooming dots on Hypertension, CAA, lacunar infarcts, WM hyperinten-
GRE occurring anywhere in the brain. There is usually no sur- sities, hyperlipidemia, diabetes mellitus, endstage renal
rounding edema or mass effect unless there has been a recent disease, cardiac transplant, and warfarin use have all been
bleed. Larger CCM may show popcorn pattern on T1WI associated with microhemorrhages. Microhemorrhages in
and T2WI. the brainstem, cerebellum, and the basal ganglia/thalamus
There are no specific clinical presentations of CAA. are associated with chronic hypertension while lobar sub-
It is mostly sporadic with a small percentage of familial cortical microhemorrhages are associated with amyloid
cases. Itis common in the elderly usually above 55 with the angiopathy.

Case
60 CLINICAL HISTORY 50-year-old male with a history of alcohol abuse and
malnutrition presenting with respiratory failure and pneumonia.
FINDINGS Figures 60-1 and 60-2. Two contiguous axial DIAGNOSIS Osmotic demyelination syndrome (ODS)/
T2WI through the pons showing mild smudgy hyperintensity central pontine myelinolysis (CPM).
in the basis pontis. Lesion is very subtle (arrows). Figures
60-3 and 60-4. Follow-up images 8 days later reveal a rather DISCUSSION ODS refers to either CPM or/and extra-
prominent round central pontine hyperintensity with fluffy pontine myelinolysis (EPM) that can occur in isolation as
outline and peripheral sparing (arrows). There were no other in this instance or together. About 50% of ODS is strictly
significant abnormalities elsewhere other than mild white pontine in location (CPM). Characteristic MRI changes of
matter changes and volume loss. There was no restricted dif- CPM may not be apparent for the first 2 weeks of the ill-
fusion or abnormal contrast enhancement. ness. Typical changes of CPM on standard MRI are iso- to
hypointense on T1WI and T2-hyperintense centrally in the
DIFFERENTIAL DIAGNOSISOsmotic demyelination pons with peripheral sparing as in this case. The lesion gener-
syndrome (ODS)/central pontine myelinolysis (CPM), ally does not enhance with contrast. Diffusion restriction in
chronic small vessel ischemic disease, encephalitis, brainstem the pons has been reported in CPM. The differential diagno-
posterior reversible encephalopathy syndrome (PRES). ses are easily excluded by the clinical and temporal profiles
126

Case 60 127
of this lesion. Improvement in pontine T2 hyperintensity Reporting Responsibilities

on follow-up imaging usually lags behind clinical improve- ODS is a clinical diagnosis and imaging helps to confirm
ment. Subsequently, the residual lesion is small and centrally the diagnosis. Sometimes it may not be expected clinically;
located in the pons and can persist for years. DTI shows hence, direct reporting to referring physician is appropriate.
superiority in detecting early changes and prior to the appear- A negative or positive imaging finding should not signifi-
ance of classical changes described above. DTI often shows cantly alter the treatment that is supportive. Routine report-
decreased fractional anisotropy (FA) and increased mean ing will suffice in most cases.
diffusivity (MD) values indicating destruction of fiber tracts
from demyelination and gliosis. Complications such as brain What the Treating Physician Needs to Know
swelling, infarction, and hemorrhage have been reported. A negative imaging study does not exclude ODS. There is a
Brain volume loss could be a long-term effect of ODS. lag between clinical presentation and imaging appearance
Clinically, ODS is typically biphasic, presenting with The classical teaching is that ODS is associated with
encephalopathy followed by improvement. A combination rapid correction of hyponatremia (>8 mmol/L per day).
of clinical signs and symptoms might follow 2 or 3 days Nevertheless, ODS has been reported in the context of a
later including quadriparesis, dysarthria, dysphagia, com- slow correction of hyponatremia (<5 mmol/L)
plete locked-in syndrome, hallucinations, akinetic mutism, Is neoplasm excluded where there is enlargement of the
seizures, and coma. Disorders associated with ODS include pons in the initial study?
hypo- and hypernatremia, hypokalemia, acute and chronic
alcoholism, alcohol withdrawal, malnutrition, anorexia ner- Answers
vosa, prolonged diuretic use, psychogenic polydipsia, burns, 1. EPM tends to occur in the following locations: white mat-
liver cirrhosis, post liver transplant, post pituitary surgery, ter of centrum semiovale; internal, external, and extreme
cyclosporine use, post urologic/gynecologic surgery (s/p gly- capsules; corpus callosum; subcortical areas; basal gan-
cine infusion), and pancreatitis. Our patient had many of these glia; thalamus; mammillary bodies; midbrain; cerebel-
predisposing risk factors. Histologically, ODS is character- lum; hippocampus; and even spinal cord.
ized by intramyelinic edema, breakdown of the bloodbrain 2. There is no known treatment for ODS regardless of eti-
barrier, and oligodendrocyte degeneration. This process might ology. Proposed treatments in the literature include rein-
perpetuate the formation of antibodies against the myelin, duction of hyponatremia, plasmapheresis (PP), IVIG,
thus the suggested benefit from using immunotherapy for the and corticosteroids. There is no clear evidence as to why
treatment of the condition. Up to 28% of patients will recover PP and IVIG may help reverse the neurologic sequelae
completely with over half left with permanent neurologic of CPM. However, it has been suggested that the ini-
changes and the remainder dying of the disease. Absence of tial osmotic stress results in the release of myelinotoxic
EPM usually results in a better outcome. products that can lead to demyelination. It is possible that
therapeutic PP may reduce the volume of these toxins and
Questions for Further Thought result in clinical improvement. IVIG was shown in mice
1. What are the extrapontine areas associated with EPM? to reverse demyelination of the spinal cord and resulted in
2. What is the treatment of ODS? promotion of myelin repair.

Case 61 CLINICAL HISTORY 35-year old male with a slowly progressive neurologic disor-
der characterized by tremor, abnormal gait, slow speech, and lately some trouble swal-
lowing. His father had similar symptoms and died of an unknown disease.
FINDINGS Figure 61-1. Axial NCCT through the basal Patients with the very rare Fahr disease present with a
ganglia. There are significant bilateral almost symmetrical progressive encephalopathy characterized by movement
calcifications in the basal ganglia, thalami, frontal white mat- disorders, gait disturbances, tremors and other abnormal
ter, and occipital cortex. There is no atrophy of the brain. movements, headache, vertigo, syncope, and seizures and
Figure 61-2. Axial NCCT in a different patient. There is may also show psychiatric abnormalities particularly in the
lesser degree of bilateral symmetrical calcifications in the advanced stage of the disease. Rarely, the patients may be
basal ganglia, thalami, and subcortical frontal white matter. asymptomatic. They may have a family history of the dis-
order, and symptoms generally start between 30 and 50
DIFFERENTIAL DIAGNOSIS Hypoparathyroidism, pseu- years of age. Abnormalities in chromosomes 14, 8, and 2
dohypoparathryroidism, pseudopseudohypoparathyroidism, have been suggested but remain unproven. The biologic
hypothyroidism, physiologic calcifications, Fahr disease abnormality is probably related to abnormal metabolism of
(bilateral striopallidodentate calcinosis). phosphate transport leading to high calcium levels. Once
calcification begins in the brain, it is progressive and may
DIAGNOSIS Fahr disease (bilateral striopallidodentate also affect the cerebellum. Anatomically, these calcifica-
calcinosis). tions occur intimately associated with arteries and not veins.
Diffuse cerebral atrophy is common. The diagnosis is basi-
DISCUSSION CT of the head shows extensive and dense cally one of exclusion.
calcifications in the lentiform nuclei and heads of the caudate
nuclei. Calcifications are also seen in the frontal white mat- Questions for Further Thought
ter, posterior thalami, and occipital lobes. MRI shows GRE 1. How common is it for extrapyramidal disorders to result
hypointensities in these areas of calcifications. All the differ- in dementia?
ential diagnosis present with similar imaging findings except 2. What other findings can help you make the diagnosis of a
that physiologic calcifications tend to be relatively smaller systemic metabolic calcium disorder in the face of cere-
and confined to the globus pallidus. bral calcifications?
128

Case 61 129

This is a chronic disease, and routine reporting is sufficient. 1. Dementia is a very common long-term sequela of all
Identify features that may lead to the suspicion of a generalized extrapyramidal (movement) disorders, and most patients
disorder of calcium metabolism. Offer a differential diagnosis. show progressive cognitive decline until they eventually
reach a vegetative state.
What the Treating Physician Needs to Know 2. Presence of extracranial calcifications such as sialoliths,
Are calcifications of the physiologic type and thus not ocular calcifications, extreme calcification of the pinnae,
important or are they pathologic and probably secondary and skin calcifications all point to a systemic calcium
to a disease? disorder.
Are the hyperdensities truly calcifications or could they be
related to other lesions such as hematomas?

Case
62 CLINICAL HISTORY 65-year-old female with ataxia.
FINDINGS Figures 62-1 and 62-2. Sagittal T1WI and Question for Further Thought
axial T2WI through the cerebellum respectively. There 1. Can cerebellar atrophy be distinguished from cerebellar
is prominence of the pericerebellar cerebrospinal fluid hypoplasia?
(CSF) spaces with significant thinning of the cerebel-
lar folia and widening of intervening CSF spacesfis- Reporting Responsibilities
sures (arrow). There is normal appearing brainstem. The Routine reporting is sufficient as this is not an acute situation.
remainder of the brain (partially shown in Figure 62-1) is First, identify the presence of cerebellar atrophy. This can be dif-
relatively normal in morphology. ficult as many cases are subtle. As a general rule, the cerebellum
should be quite full, even in older individuals, such that the fis-
DIFFERENTIAL DIAGNOSIS Normal aging, drug-related sures which separate the folia are difficult to visualize. Second,
atrophy, alcohol-related atrophy, cerebellar ataxia syndrome, pay close attention to the morphology of adjacent structures
paraneoplastic disease. including the brainstem, medulla, and upper spinal cord.
DIAGNOSIS Cerebellar ataxia syndrome (inherited). What the Treating Physician Needs to Know
Isolated cerebellar atrophy carries a broad differential diag-
DISCUSSION Many potential causes exist for cerebel- nosis including toxic, metabolic, iatrogenic, and inherited
lar atrophy. The present case could represent any of the conditions
etiologies listed in the differential. Many other entities Precise diagnosis is beyond the realm of imaging in most
also exist, and in the pediatric age group, the list expands cases
significantly with a host of rare inherited disorders. The Other relevant changes elsewhere in the brain
distinction is usually made on the basis of clinical his-
tory, examination, and laboratory testing. Imaging can Answer
be of some help in narrowing the differential, as certain 1. Cerebellar atrophy implies loss of tissue. This results in
entities present with relatively more specific patterns of widening of the fissures relative to the folia. The pos-
abnormality. Atrophy of the cerebellum is the common terior fossa is usually normal in size, and the cerebel-
denominator among all, with variable involvement of lum should continue to fill it, albeit less completely.
other structures such as the brainstem and spinal cord. Cerebellar hypoplasia implies a small but tightly packed
This pattern recognition approach can be used to separate cerebellum with otherwise normal shape and morphol-
isolated cerebellar atrophy, which may be due to drugs, ogy. The posterior fossa may be small itself, or it may be
toxins, or inherited cerebellar ataxia (as in the present normally sized and largely empty. In actual practice, it
case) from other entities such as spinocerebellar ataxia or can be quite difficult or impossible to distinguish atrophy
multiple system atrophy. from hypoplasia.
130

Case
63 CLINICAL HISTORY 4-month-old with failure to thrive for the last 2 weeks.
Figure 63-4
lobes and splaying with compression of the cerebral pedun-

cles and midbrain posteriorly (arrows). Figure 63-2. Axial
T2WI through the mass. The mass is homogenously hyper-
Figure 63-3 intense. The lines seen at the lateral aspect of the mass are
the bilateral A1s being elevated (arrows). Figure 63-3. Axial
ADC map through the mass. There is an elevated ADC value
FINDINGS Figure 63-1. Axial T1WI through the suprasel- throughout the mass. Figure 63-4. Coronal post- contrast
lar region. There is a large hypointense mass filling the supra- T1WI through the mass. The mass (star) enhances relatively
sellar cistern with compression of the frontal and temporal homogenously throughout. Figure 63-5. Photomicrograph
131

132 Case 63
it can be difficult to tell exactly where they are arising. A

meningioma or any highly cellular mass, such as lymphoma,
are usually isointense to hypointense on T2WI. The high cel-
lularity of these lesions usually results in lower ADC values
or restricted diffusion, and this lesion has elevated ADC val-
ues. A meningioma may present with a dural tail which is
absent in this case. Lack of sella enlargement excludes pitu-
itary macroadenoma.
PMA, a WHO II astrocytoma, is a tumor usually seen in
children and young adult. PMA is a localized glioma with
piloid cells and myxoid background. A characteristic find-
ing is perivascular arrangement of its cells. Rosenthal fibers,
eosinophilic granular bodies, and microcysts are usually
not seen. Necrosis and parenchymal infiltration are more
common. PMA often occurs in the hypothalamic and chias-
mic region, but they can occur in any part of the CNS. The
Figure 63-5
lesions may have lobar presentation with a propensity for the
temporal lobes. Secondary to the predominant location in
the third ventricular region, many of the cases present with
shows radial perivascular arrangement of piloid glial cells hydrocephalus and symptoms of increased intracranial pres-
(H&E stain). sure (headache, nausea, vomiting). Other symptoms include
failure to thrive, confusion, ophthalmoplegia, facial weak-
DIFFERENTIAL DIAGNOSIS Glioma, meningioma, lym- ness, extremity weakness/hemiplegia, and seizure when
phoma, pituitary adenoma, craniopharyngioma, pilomyxoid cortical. PMAs are associated with long survival even when
astrocytoma (PMA), pilocytic astrocytoma (PA). partially resected. PMA has a higher rate of recurrence and
CSF dissemination compared with PA.
DIAGNOSIS PMA.
DISCUSSION The suprasellar region is the commonest
1. Do PMAs need more aggressive therapy?
location of PMA and occurs in about two-thirds of the cases.
PMA may occur elsewhere in the CNS. This case is that of
a solid suprasellar PMA as are most of these tumors show-
Direct reporting is essential for all tumors. The presence of
ing homogeneous hypointensity on T1WI and hyperintensity
hydrocephalus must be immediately relayed to the referring
on T2WI with homogeneous contrast enhancement. Mixed
physician. Growth rate in a follow-up evaluation should be
intensity pattern with heterogeneous contrast enhancement
reported. Presence of leptomeningeal disease may be an indi-
may occur in a mixed solid and cystic tumor. There is gener-
cation of CSF dissemination. All encased vascular structures
ally elevated ADC. GRE may demonstrate hemorrhage in
should prompt further evaluation by MRA or CTA.
up to 20%, and calcification is very rare. PA shares a lot of
features in common with PMA from which it may be dif-
ficult to differentiate. PMAs compared with PAs have been What the Treating Physician Needs to Know
found to have more solid nonenhancing areas, higher ADC, Location
and higher T2WI signal intensity. The higher ADC and Presence of mass effect or hydrocephalus
T2WI signal intensity likely reflects the myxoid component. Presence of leptomeningeal disease may prompt cranio-
A more heterogeneous lesion particularly with hemorrhage spinal axis evaluation
may be difficult to distinguish from craniopharyngioma.
Other suprasellar tumors such as glioma, lymphoma, menin- Answer
gioma, and suprasellar extension of macroadenoma may 1. PMA requires a more aggressive treatment. Recurrence
pose a difficult diagnostic challenge. When lesions are large rate is higher, and overall survival is shorter than in PA.

Case
64 CLINICAL HISTORY 2-year-old with increasing ataxia, irritability, and new onset
vomiting.
FINDINGS Figure 64-1. Sagittal non-contrast T1WI. There component in front of the pons (vertical arrow) is not enhanc-
is a large multilobulated solid cystic mass in the suprasellar ing. Figure 64-3. Coronal post-contrast T1WI. The superior
cistern. There is marked mass effect and displacement of the extension of the lesion to the foramina of Monro (trans-
floor of the third ventricle (vertical arrow), posterior inferior verse arrow) and the dilation of the lateral ventricles (star)
frontal lobes (transverse arrow), midbrain and pons (star). are demonstrated. The sella turcica is compressed (vertical
Thelesion is heterogeneous hypo- to isointense. Figure 64-2. arrow). Figure 64-4. Axial T2WI. The cystic component is
Sagittal post-contrast T1WI. Thin (thin arrow) and thick noted to be hyperintense to cerebrospinal fluid (CSF) (star),
(thick anterior arrow) walls of enhancement are present. The and the more solid component is only mildly hyperintense
133

134 Case 64
(arrow). There is mild surrounding edema. No evidence of (NF1) are identified, then the diagnosis of a PA becomes
calcifications was found on the MRI. very likely. PAs occur mostly in the first two decades
of life although the age of presentation can extend to late
DIFFERENTIAL DIAGNOSISOptic nerve glioma, cra- adulthood. PA occurs equally in males and females. PAs
niopharyngioma, meningioma, metastatic tumor, mac- often present with symptoms of hydrocephalus due to CSF
roadenoma, aneurysm, sarcoid, tuberculoma, pilocytic pathway obstruction but can also present with symptoms
astrocytoma (PA). related to local mass effect or invasion. These symptoms
include loss of vision, pituitary hypofunction, diencephalic
DIAGNOSIS PA of optic pathway. syndrome, cranial nerve dysfunction with cavernous sinus
involvement, and/or proptosis. PA is the most benign of all
DISCUSSION PA has a strong propensity to present as a
astrocytic tumors and the most common glioma in children
complex cystic and solid mass but can be mainly cystic or
1 to 19 years forming about 5% to 6% of all gliomas. PA is
solid. Calcification and hemorrhage can occur (24% and 31%,
a relatively circumscribed tumor composed of compact cel-
respectively), but some series have yielded no calcifications.
lular areas consisting of elongated and fibrillated cells that
The cystic component is iso- to slightly hyperintense to CSF
alternate with loose myxoid, microcyst-rich areas. Nuclei
on T1WI, incompletely suppressed on FLAIR, and hyperin-
contain bland chromatin. Mitoses are none to rare. Brightly
tense to CSF on T2WI. The solid component is iso- to hypoin-
eosinophilic Rosenthal fibers are common in compact areas,
tense on T1WI and iso- to hyperintense on T2WI. The solid
whereas eosinophilic granular bodies (EGBs) can be seen in
component and cyst wall have variable contrast enhancement
either region. Necrosis and glomeruloid vascular hyperpla-
from none to avid enhancement. Mass effect is usual with
sia can be seen and should not be confused with high-grade
very little or no surrounding edema. PA in the optic pathway
glioma. It is a WHO I tumor with a good chance of cure
such as this may not always be well-defined like elsewhere
following complete removal.
but can be infiltrating, spreading along the optic tracts and
nerves. MRS may show a high choline and creatine peak
with reduced N-acetyl aspartate (NAA) in the solid compo- Question for Further Thought
nent. Perfusion MRI usually shows reduced relative cerebral 1. What are the factors associated with event-free survival?
blood volume (rCBV) and cerebral blood flow (CBF) in the
solid portion. The CT findings follow the MR morphology. Reporting Responsibilities
PA is isodense to hypodense without contrast. It may show Presence of hydrocephalus or herniation deserves direct
hyperdense calcification or hemorrhage and variable contrast reporting to the referring physician. Although PA is a WHO
enhancement of a mural nodule or solid component within I tumor, presence of leptomeningial enhancement may sug-
a nonenhancing cyst. Cyst wall may enhance. The majority gest the occasional but rare tumor dissemination, and evalu-
of PA occur in the posterior fossa with a reported incidence ation of the craniospinal axis may be warranted. A vascular
of 75% with the remainder split between the supra/parasellar mass such as aneurysm should not be confused with a tumor.
region as in this case and supratentorial lobar regions. The If in doubt suggest MRA or CTA.
supratentorial lobar location is more common in adults and
occurs more in the temporal and parietal lobes. What the Treating Physician Needs to Know
The absence of calcifications makes craniopharyngioma
There is a strong association of PA with NF1. If this has
unlikely. The lack of skull base thickening, marrow infiltra- not already been established, it may be necessary to look
tion, dural tail, and overall complex morphology excludes for this association
meningioma. This lesion or any lesion is unlikely to be a Is the tumor well circumscribed or infiltrating? Any com-
pituitary adenoma if the sella is unremarkable. The lack of plications?
flow artifact, lack of blood clot, and presence of solid and Location and size on both initial and follow-up recording
cystic areas rule out a giant aneurysm. Sarcoid is excluded by any tumor growth
the complex solid enhancing and nonenhancing components.
One may expect associated leptomeningeal and cranial nerve
enhancement (especially CN V and VII) in sarcoidosis. Answer
Metastatic disease or glioblastoma presents in this location 1. Four pathologic features (necrosis, oligodendroglioma-
rarely, and the features could be similar, but more associated like features, vascular hyalinization, and calcification)
edema would be expected. With metastatic disease solid show a significant correlation with decreased event-free
nonenhancing tumor would not be present. survival. Also, PAs involving the optic pathway are asso-
Patients with neurofibromatosis type 1 have a propensity ciated with worse event-free survival compared with
for developing PA. If stigmata of neurofibromatosis type 1 those arising in other locations.

Case
65 CLINICAL HISTORY Acute right-sided weakness following a left middle
cerebral artery (MCA) aneurysm clipping.
FINDINGS Figure 65-1. Axial NCCT through the tem- a left temporal scalp soft tissue swelling from recent sur-
poral lobes. There is a left temporal lobe diffuse hypoden- gery (arrow). Figure 65-2. Axial CT perfusion (CTP) blood
sity (star) with mass effect and subtle left uncal herniation flow color map through the temporal lobes. There is signifi-
consistent with malignant acute ischemic infarct. There is cantly diminished blood flow in the left temporal lobe (star).
135

136 Case 65
Figure65-3. Axial CTP blood volume color map through the 65 mL/100 g/min. Infarct core CBF threshold is set as 4.8
temporal lobes. There is diminished left temporal lobe blood to 8.4 mL/100 g/min while tissue infarction threshold is 17
volume (star) of same size as the flow deficit. Figure 65-4. to 18 mL/100 g/min, and the salvageable or ischemic pen-
CTP mean transit time (MTT) color map through the tem- umbra threshold is 14.1 to 35 mL/100 g/min. MTT increase
poral lobes. There is prolonged MTT (star) of similar size higher than 145% has been suggested to define the penumbra
to the blood flow and volume deficits. These are matching or salvageable tissue. It is, however, easier to use the color
perfusion maps consistent with infarction without significant maps when quickly looking for a mismatch. Reduced CBV
area of salvageable penumbra. and prolonged MTT indicate infarction. When the size of
the reduced CBV is equal to the size of the prolonged MTT
DIFFERENTIAL DIAGNOSIS N/A. as in our case here, the so-called matched CBV and MTT,
this defines the infarct core, and usually there is no benefit
DIAGNOSIS CTP of left MCA territory ischemic infarct. from intervention. However, when the size of the reduced
CBV is smaller than the size of prolonged MTT, there is a
DISCUSSION In this situation, there is an established CBV/MTT mismatch which defines the presence of a pen-
infarct following the clipping of a left MCA aneurysm. CTP umbra or salvageable brain around the infarct core, and this
could be useful to determine whether there is a salvageable may benefit from intervention. When there is normal or near
area surrounding the infarct core, known as the ischemic normal CBV with prolonged MTT, this defines an ischemic
penumbra. The penumbra could benefit from some form of region of the brain, and this suggests reversibility, and the
aggressive treatment. We will deal with some fundamental patient may benefit from appropriate intervention.
information obtainable from the CTP and how that informa-
tion could be useful in triaging the stroke patient. We will Question for Further Thought
not get into the complexity of performing the CTP or exten- 1. Is there any reason to prefer CTP over MR perfusion?
sive debate about the measured parameters. The interpre-
tation of the CTP information is rather complex and could
be challenging. There is no standardization of software and
Direct reporting is essential in acute ischemic infarcts.
quantification of the parameters and how the parameters could
Presence of salvageable region makes reporting more urgent.
guide therapy. The parameters usually measured by CTP are
the cerebral blood flow (CBF), cerebral blood volume (CBV),
and MTT and time to peak (TTP). These are calculated from What the Treating Physician Needs to Know
the source data of the CTP by deconvolution analysis. To dis- Size and location of lesion
cuss these parameters as they apply to the stroke patient, some Presence or absence of a penumbra
basic definitions may be necessary. Cerebral ischemia indi-
cates inadequate blood supply or oxygenation to the brain or a Answer
region of it due to poor perfusion as a result of low or no blood 1. For the stroke patient, CTP may be a better test. NCCT
flow. This could be reversible. When ischemia results in focal continues to be the first-line examination for the stroke
brain death, it is termed cerebral ischemic infarct which is per- patient. It is readily available, faster, cheaper, and timely
manent and irreversible. This is what we have in this patient. and gives essential information regarding presence of
CBF is measured as the amount of blood in milliliters an ischemic infarct and its size, hemorrhagic stroke,
flowing through 100 g of brain tissue per minute. CBV is or a stroke mimic such as a tumor. These are facts that
measured as the amount of blood in milliliters per 100 g of make NCCT appealing regarding the window of oppor-
brain tissue. MTT is the time it takes for a quantity of blood tunity to intervene in the acute stroke treatment protocol.
to pass through a volume of brain and is measured in seconds. Adding CTP to NCCT takes possibly an additional 15 to
The quantification of the parameters varies depending on the 20minutes for data acquisition and processing. The down-
equipment and software. There are conflicting figures in the side of CT is always the radiation exposure! MR with MR
literature regarding the various measurements. Presented perfusion is cumbersome for this kind of patients and may
here are some figures available in the literature, and they are not yield the desired result quickly enough to enable acute
for guidance only. Values are generally higher in the gray stroke management. On the other hand, MR perfusion in
matter (GM) than white matter (WM) because of the higher tumors tends to be better in view of the better tissue char-
demand of the GM for energy, the higher end of the values are acterization required for evaluation of brain tumors and
therefore for the GM. Normal mean CBV is 2 to 4mL/100g. the added advantages of the various MR sequences and
Total blood flow to the brain is 800 mL/min (15% to 20% multiplanar capability. Time may not necessarily be brain
cardiac output), and the normal CBF is calculated at 21 to in tumor evaluation!

Case
66 CLINICAL HISTORY 50-year-old female with subacute headache and acute
onset of bilateral lower extremity weakness (right greater than left).
137

138 Case 66
DIAGNOSIS Idiopathic granulomatous pachymeningitis

complicated by secondary sagittal sinus thrombosis, venous
congestion, and venous infarction.
DISCUSSION MRI is the examination of choice for evalu-

ation of pachymeningitis which primarily affects the dura
arachnoid. There is usually dural thickening which could
be focal or extend over a sizable portion particularly of the
falx and tentorium. The thickened dura is isointense on pre-
contrast T1WI. Post-contrast T1WI shows enhancement of
the convexity dura. Where the meningeal dura (as opposed
to the periosteal dura) folds on itself to form the falx and the
tentorium, there is usually a central hypointensity separating
the enhancing dura, forming a triple-layered structure with
dural enhancement enclosing the hypointensity as seen in
Figures 66-4 and 66-5. At other times there is apparent fusion
of the two leaves of dura. There may be associated nodularity
or focal thickening or leptomeningeal thick enhancement as
seen in Figure 66-4. On T2WI, the dura is hypointense with
peripheral hyperintensity. CT is not as sensitive as MRI, but
it generally replicates the MRI findings of enhancing triple-
Figure 66-5 layered falx and tentorium. Convexity dural enhancement may
blend with overlying bone density on CT. The thickened dura
is hyperdense without contrast. Imaging is much better in the
detection of complications or sequelae of extensive pachyme-
ningeal deposits, such as hydrocephalus, venous sinus throm-
FINDINGS Figure 66-1. Axial FLAIR image through the bosis, and venous infarction as demonstrated in this case.
centrum semiovale. There is bilateral subcortical white The thrombosis of the superior sagittal sinus and bilat-
matter signal hyperintensity within the centrum semiovale eral cerebral venous infarction are secondary to the exten-
(arrowheads). There is a suggestion of increased signal sive dural inflammation. The dura may normally enhance,
intensity in the posterior aspect of the sagittal sinus. There most notably over the upper convexities. This physiologic
is also focally hyperintense FLAIR signal involving the cor- phenomenon is most conspicuous at 3T MR, particularly
tex of the medial surface of the left hemisphere (paracentral with isovolumetric post-contrast gradient T1 techniques. It is
lobule*) that shows corresponding diffusion restriction on smooth and usually no thicker than a couple of millimeters.
axial DWI in Figure 66-2 (arrows). Note the small, medial Reactive, diffuse dural thickening and enhancement may be
contralateral focus of diffusion restriction (Note: the DWI observed following intracranial surgery. Most of the differ-
signal hyperintensity along the right convexity is artifactual). entials tend to present nodular characteristics or mass-like
Figure 66-3. Sagittal 2D TOF MR venography MIP image lesions that tend to separate them from the idiopathic hyper-
shows absence of flow-related enhancement along the mid trophic pachymeningitis but not from each other. Intracranial
and posterior aspect of the superior sagittal sinus (arrow- hypotension on the other hand presents a rather diffuse
heads). Figures 66-4 and 66-5. Coronal post-contrast T1WI smooth pachymeningeal enhancement with sagging of the
anteriorly and posteriorly, respectively. There is abnormal mid- and hindbrain.
pachymeningeal thickening and enhancement (small arrow- Pachymeningitis is a rare disorder with diverse etiology.
heads) in addition to sulcal leptomeningeal (pia-arachnoid) Two major forms are identified: the primary or idiopathic
enhancement along the medial surface of the left cerebral hypertrophic pachymeningitis when the cause cannot be
hemisphere (small arrows). found as in our case and the secondary pachymeningitis
when a definable association can be made. Early reports of
DIFFERENTIAL DIAGNOSISDural and leptomeningeal pachymeningitis were in association with tuberculosis and
metatastasis (breast, lymphoma), leukemia, en plaque and syphilis, but there are several other associations. Its patho-
lymphoplasmacyte-rich meningiomas (LRM), neurosar- genesis remains speculative but presumed to be due to auto-
coidosis, Wegener granulomatosis, tuberculous meningitis, immune phenomenon or a result of direct infiltrative process.
noninfectious inflammatory meningitis (i.e., rheumatoid Imaging has very low specificity regarding the cause or eti-
pachymeningitis), bacterial and fungal meningitis, hypertro- ology of pachymeningitis. It has multiple clinical presenta-
phic pachymeningitis, idiopathic granulomatous meningitis, tions with headache and craniopathies being some of them.
intracranial hypotension. An extensive clinical workup including cerebrospinal fluid

Case 66 139
(CSF) analysis, dural, leptomeningeal, and brain biopsy may puncture and blood work is required to exclude infection
be required, and a specific cause is not always identified. and malignancy
In this case, the pathologic specimens showed nonspecific
inflammatory process with granulomatous features. Answers
1. The dura or pachymeninges is the thick, fibrous outer layer
Questions for Further Thought of the meninges that consists of two layers (outer perios-
1. How does the anatomy of the dura contribute to the teal and inner meningeal) in the intracranial compartment,
changes seen on imaging? unlike the single layer in the spinal canal. The two intracra-
2. What is the nature of the relationship between lum- nial dural layers are closely applied along the inner table
bar puncture and diffuse intracranial dural thickening/ of the skull, separating to form the dural venous sinuses.
enhancement on MRI? Hence inflammation of the dura can lead to inflamma-
tion of the venous sinuses resulting in sinus thrombosis.
Reporting Responsibilities The inner layer is redundant and folds into the interhemi-
The finding of dural venous sinus thrombosis, with or with- spheric fissure as the falx cerebri and similarly forms the
out venous congestion, venous infarction, or parenchymal roof of the posterior fossa as the tentorium cerebelli. It
hemorrhage is a clinical emergency requiring prompt report- also forms the small falx cerebelli along the midline of the
ing and urgent treatment to prevent life-threatening conse- posterior fossa, between the cerebellar hemispheres. The
quences. Secondary hydrocephalus and any focal mass effect two layers of this fold enclose a loose layer which forms
or brain edema should also be reported emergently. the nonenhancing portion of the triple layer on contrast-
enhanced T1WI.
What the Treating Physician Needs to Know 2. Diffuse intracranial dural enhancement is rare following
Extent/distribution of disease uncomplicated lumbar puncture. Intracranial hypotension
Complications of pachymeningitis (venous sinus involve- either spontaneous or following a lumbar puncture or wet
ment, venous congestion/edema/infarction), mass effect, tap (inadvertent subarachnoid needle placement dur-
leptomeningeal component, hydrocephalus ing epidural anesthesia) shows characteristically smooth,
The cause of the thrombosis is usually not evident on thin, and diffuse dural enhancement which resolves once
imaging. Additional workup including expeditious lumbar the leak is treated.

Case
67 CLINICAL HISTORY 36-year-old male with 3-month history of headaches found
unresponsive in his car.
140

Case 67 141
FINDINGS Figure 67-1. Axial NCCT through the lateral the confluent changes. There is usually associated volume
ventricles shows dilatation of the lateral ventricles (stars) loss unlike the effacement of the sulci in hydrocephalus.
and absence of cortical sulci. There is subtle periventricular Periventricular lymphoma generally contrast enhances and
hypodensity (arrows). Figures 67-2 to 67-4. Corresponding show reduced diffusion.
axial T2WI and FLAIR through various levels of the lateral The pathogenesis of transependymal CSF flow is based
ventricles show the dilated ventricles (star) and confluent on disruption of the tight uninterrupted ependymal lining of
periventricular hyperintensity (arrows) which is better dem- the ventricles in the setting of raised intraventricular pres-
onstrated on the FLAIR images due to cerebrospinal fluid sure. CSF is forced across the ependymal lining into the
(CSF) suppression. This confluent periventricular hyperin- interstitium of the periventricular WM; hence, the continu-
tensity in the setting of hydrocephalus is known as periven- ity of the periventricular changes with the ependymal wall.
tricular edema or transependymal CSF flow. This represents a compensatory mechanism aimed at help-
ing the reabsorption of CSF. The fluid composition in this
DIFFERENTIAL DIAGNOSIS Small-vessel ischemic dis- periventricular edema is identical to CSF, with similar ionic
ease (leukoaraiosis), cerebral volume loss, normal pressure concentrations and negligible protein levels (as opposed to
hydrocephalus, communicating hydrocephalus, noncommu- vasogenic edema). There are various causes of acute raised
nicating hydrocephalus, periventricular lymphoma, ependy- intraventricular pressure resulting in transependymal CSF
mitis granularis. flow, which may include obstructing masses, meningitis,
subarachnoid hemorrhage, and normal pressure hydro-
DIAGNOSIS Transependymal CSF flow or edema due to cephalus. Transependymal CSF flow is seen most com-
obstructive hydrocephalus. monly with acute (decompensated) ventricular obstruction,
which usually occurs at the level of the cerebral aqueduct.
DISCUSSION The interest in this case is in the transep- It is observed far less frequently in the setting of commu-
endymal CSF flow. Transependymal CSF flow is known nicating hydrocephalus. Contrast should be administered to
by several names; periventricular edema, interstitial edema, all patients presenting with acute hydrocephalus to exclude
hydrocephalic edema, and transependymal CSF permeation. underlying lesions. Caution has been advised when placing
The hallmark of transependymal CSF flow is the confluent these patients for long periods of time in the MR unit as they
periventricular T2 hyperintensity that may initially appear may acutely decompensate.
around the frontal horns, trigone, and occipital horns of the
lateral ventricles that eventually spreads to surround the Question for Further Thought
entire lateral ventricles. It may extend from the ependymal 1. What additional sequences might aid in evaluation of the
walls to the subcortical white matter (WM) in continuity cerebral aqueduct?
in very severe cases. It is almost invariably associated
with acute decompensated hydrocephalus with ventricular
enlargement. It is better appreciated on FLAIR images due
Direct reporting is essential. Acute hydrocephalus is an
to the suppression of the CSF. On CT it appears as confluent
emergency and this finding as well as any identifiable cause
periventricular hypodensity. DWI and ADC may be normal
must be reported to the requesting physician.
to increased diffusivity. DTI may show decreased fractional
anisotropy (FA). There is generally no contrast enhancement
unless there is superimposed ventriculitis or the hydroceph- What the Treating Physician Needs to Know
alus is caused by ventriculitis in which case there may be Is there an identifiable cause of ventricular obstruction
ependymal linear contrast enhancement along with intraven- (aqueductal stenosis and intraventricular/midbrain mass)?
tricular debris. Ependymitis granularis is a normal variant. Are additional sequences needed?
There is a small triangular cap of T2 hyperintensity around
the frontal horns. This is due to regionally decreased myelin, Answer
increased extracellular fluid, or focal breakdown of the 1. 3D high-resolution T2 sequences (CISS, GRASS, SPACE,
ependymal lining with gliosis. Leukoaraiosis and other WM etc.) can be utilized to evaluate for stenoses or small webs
diseases, on the other hand, tend to be discontinuous; even especially at the level of the aqueduct of Sylvius. Phase
when they are confluent, they tend to show some sparing of contrast cine MRI has also been used to evaluate the aque-
the periventricular walls or evidence of viable WM within duct in the setting of hydrocephalus.

Case
68 CLINICAL HISTORY 44-year-old male with a known history of Von Hippel-Lindau
(VHL) disease.
Figure 68-2
Figure 68-1
FINDINGS Figure 68-1. Axial T2-weighted MRI through Figure 68-4. Axial T2WI through the suprasellar region. The
the lower cerebellum. There are two small hyperintense cer- mass is heterogeneous but predominantly isointense. There
ebellar lesions: adjacent to the fourth ventricle on the right are some vascular signal voids superiorly to the left of the
(transverse arrow) and posterior/peripheral on the left (ver- lesion (arrow). Figure 68-5. Coronal post-contrast T1WI
tical arrow). There is no surrounding edema. Figure 68-2. through the mass. The mass has avid contrast e nhancement.
Post-contrast axial T1WI through inferior cerebellum. Figure68-6. Axial post-contrast T1WI just superiorly to the
There is a spiculated contrast enhancement of the left cer- mass. There are tubular contrast-enhancing structures super-
ebellar lesion (arrow). Figure 68-3. Sagittal T1WI. There is olateral to the left of the lesion consistent with blood vessels
a well-circumscribed isointense suprasellar mass (arrow). (arrow).
142

Case 68 143
DIFFERENTIAL DIAGNOSISMetastases, multiple heman- This patient has been followed up at our institution for
gioblastomas, astrocytoma. about 6 years with very little or no change in the CNS masses.
CNS symptoms are usually due to obstruction to CSF flow
DIAGNOSIS Multiple hemangioblastomas in a patient resulting in hydrocephalus. Small lesions situated away from
with VHL disease. CSF pathways may be asymptomatic.
This patient has multiple visceral lesions, which include
DISCUSSION Hemangioblastomas are rare tumors that bilateral phthisis bulbi, renal cell carcinoma, splenic masses,
can occur sporadically as well as in familial forms. They pancreatic masses, testicular masses, and a lung nodule.
are usually located in the cerebellum in up to 72% of VHL VHL disease is an autosomal dominant inherited disease
disease. They are very rare in the suprasellar region. Spinal with protean manifestations. It is caused by a mutation of
cord hemangioblastomas are more common than supraten- a tumor suppressor gene located in chromosome 3p25-26.
torial lesions. MRI is the modality of choice for evaluation There are four phenotypes of the disease.
and screening of the central nervous system (CNS) lesions The clinical manifestations include cerebellar, retinal, and
in VHL disease. Hemangioblastomas in VHL disease tend spinal cord hemangioblastomas, endolymphatic sac tumors,
to be multiple, occurring in the young with dismal prog- pancreatic and renal cysts, renal carcinomas, tumors of the
nosis. Sporadic hemangioblastomas tend to occur in the pancreas and epididymis, pheochromocytoma/paragan-
older population. These tumors could be cystic mostly glioma of the adrenal gland/paraganglia, and cysts in other
with contrast-enhancing mural nodule mimicking pilocytic organs. Most of these lesions are treatable. Family members
astrocytoma in the young. Solid, hemorrhagic, or mixed who are carriers of the gene require monitoring and screen-
tumors are usually very vascular with tubular vascular sig- ing both clinically and by imaging. Patients with known dis-
nal voids demonstrable at MRI. These vascular structures ease also undergo annual clinical and multimodal imaging
are also demonstrated at angiography. The cystic portion screening. Yearly lifetime screening is recommended and
of the tumor has cerebrospinal fluid (CSF) intensity on all begins after the age of 10 years.
sequences. Hemorrhagic tumors are usually heterogeneous.
Solid tumors are usually isointense on FLAIR, T1WI, and Question for Further Thought
T2WI with avid contrast enhancement. Lack of surround- 1. How is the clinical diagnosis of VHL disease made?
ing edema tends to distinguish these multiple tumors from
metastases. Other findings not shown include heteroge- Reporting Responsibilities
neous vitreous humor in deformed globes consistent with Direct reporting is essential. Presence of brain hemangioblas-
phthisis bulbi presumably due to retinal hemorrhage from tomas should prompt evaluation of the spine for spinal heman-
retinal hemangioblastomas. gioblastomas. MRI of the entire spine is recommended.

144 Case 68
What the Treating Physician Needs to Know Answer

The number and location of the hemangioblastomas 1. The clinical diagnosis of VHL is based on three diagnos-
If a patient is being investigated for the first time, multiple tic criteria: multiple CNS hemangioblastomas, one CNS
hemangioblastomas point in the direction of VHL disease hemangioblastoma along with other systemic manifesta-
and the necessary testing and surveillance should be put tions of the disease, and a family history with any mani-
in place festation of the disease.
Location of lesions allows correlation with symptoms to
determine which of the multiple lesions to treat

Case
69 CLINICAL HISTORY 39-year-old female with strange sensations in her epigastric
area radiating to her right shoulder. No frank weakness but some difficulty utilizing
her right hand.
145

146 Case 69
ependymomas, we suggest that these tumors have elevated

perfusion as seen in this case. MR spectroscopy demon-
strates elevated choline and reduced NAA as is seen with
many tumors. The ADC is elevated in ependymoma but not
as high as in pilocytic astrocytoma. The mild surrounding
edema and the pattern of enhancement are distinct from the
thick irregular ring enhancement associated with a metasta-
sis or glioblastoma. Edema is not a prominent component
of cortical ependymoma. Other cortical tumors such as PA,
dysembryoplastic neuroepithelial tumor (DNET), pleomor-
phic xanthoastrocytoma (PXA), GG, and oligodendroglioma
could be considerations. Since cortical ependymoma is often
present with a cyst and nodular appearance, this helps to dif-
ferentiate it from ordinary WHOII astrocytoma and oligo-
dendroglioma.
Cortical ependymoma is a rare tumor occurring more
Figure 69-5
commonly in young adults often in the late second to early
fourth decades. The tumor commonly presents with seizures
because of its cortical location. Ependymoma is composed
FINDINGS Figure 69-1. Left parasagittal non-contrast 3D of monomorphic cells with round to oval nuclei with bland
T1WI. There is a well-defined posterior left frontal hypoin- chromatin. It forms perivascular pseudorosettes and epen-
tense mass with mild vasogenic edema anteriorly (arrow). dymal canals. Mitoses, necrosis, and vascular endothelial
There is area of irregular isointensity within the lesion. Figure proliferation are present in the anaplastic lesion. Surgical
69-2. Axial post-contrast T1WI through the mass. There is an resection is often curative for the WHO II ependymoma,
avid contrast-enhancing portion laterally (arrow) with the rest and subtotal resection often results in worse outcome.
showing a cystic appearance. Figure69-3. Axial ADC map WHO III ependymoma is known to recur locally despite
through lesion. There is an irregular, mild, lowADC within radiation therapy.
the solid component with the rest showing high ADC values.
There is edema anteriorly to the mass (arrow). Figure 69-4. Questions for Further Thought
Axial relative Cerebral Blood Volume (rCBV) map through 1. Should postoperative radiation therapy be utilized?
the mass. There is elevated rCBV in the lateral component of 2. Should there be continued posttreatment surveillance?
the mass (arrow). Figure 69-5. Photomicrograph shows tumor
composed of monomorphic cells with mildly hyperchromatic Reporting Responsibilities
nuclei. Central canal-like structures are present (H&E stain). Tumors deserve direct reporting so that treatment could
begin in earnest. Location is important. Tumors located in
DIFFERENTIAL DIAGNOSIS eloquent areas may require functional imaging evaluation to
Metastasis, glioblastoma, astrocytoma, ganglioglioma (GG), determine resectability. Tumor growth should be reported in
pilocytic astrocytoma (PA), cortical ependymoma. follow-up studies.
DIAGNOSIS
Cortical ependymoma.
Location will determine resectability
DISCUSSION Presence of significant mass effect or herniations
Ependymoma is often cystic or multicystic with less com- Rate of growth on surveillance or follow-up study
mon presentation as a solid tumor. Cortical ependymoma Ependymoma recurrences are mostly local, and repeated
tends to enhance, and the enhancement has been described surgery is often possible
as having a popcorn appearance. Calcifications and hem-
orrhage occur within the tumor but have been reported to Answers
be variably present at imaging ranging from uncommon to 1. There are limited data on utilizing radiotherapy in adult
common. On CT ependymoma is predominantly hypodense ependymoma. It is potentially not helpful in WHO II
to isodense. T2WI demonstrates hyperintensity, and T1WI lesions, whereas in WHO III lesions it may be useful
is more heterogeneously isointense to hypointense. When to add.
present the calcification is hyperdense on CT and is hypoin- 2. Surveillance imaging is justified to detect early evidence
tense on MRI sequences. Extrapolating data from the pedi- of recurrence that might allow reoperation or more effect
atric population with a high percentage of infratentorial salvage therapies.

Case
70 CLINICAL HISTORY This child was accidentally left in the garage inside a running
car, doors closed, while the mother went to answer a long telephone call. She came out
and found the child unconscious.
FINDINGS Figure 70-1. Axial MR T2WI through the

basal ganglia. There is bilateral symmetrical globus pallidus
hyperintensity (arrows). Figures 70-2 and 70-3. Axial DWI
with corresponding ADC map through the basal ganglia.
There is bilateral symmetrical restricted diffusion in the glo-
bus pallidus (arrows).
DIFFERENTIAL DIAGNOSIS Wilson disease, Creutzfeld-

Jakob disease, Japanese encephalitis, Carbon monoxide
(CO) poisoning and Leigh syndrome and other mitochondri-
opathies.
DIAGNOSIS Carbon monoxide (CO) poisoning.
DISCUSSION CO is the commonest lethal poison world-

wide, resulting frequently in neurologic injury despite treat-
ment. NCCT shows symmetric hypodensities in the globi
pallidi but diffuse hypodensity of the cerebral WM can also
be seen. On MRI the globi pallidi can appear hyperintense or
hypointense on T1WI (due to necrosis or hemorrhage), hyper-
intense on T2WI, have restricted diffusion and occasionally
patchy or peripheral enhancement of the necrotic areas.
The chronic changes could present as bilateral globus pal-
Figure 70-3 lidus calcifications, diffuse brain atrophy, and cerebral white
147

148 Case 70
matter (WM) demyelination. The putamina and thalami are Question for Further Thought
sometimes also involved in CO poisoning, but less frequently 1. Through which mechanism does CO poisoning causes the
than the globus pallidus. The changes could resemble Wilson late brain changes?
disease and hypoxic-ischemic encephalopathy (HIE).
CO has a higher affinity than oxygen for the hemoglobin Reporting Responsibilities
protein and when inhaled it produces carboxyhemoglobin Direct reporting is necessary in this acute situation. A short
which causes acute tissue anoxia-hypoxia as it precludes the list of differentials should be given.
proper oxygen transport by erythrocytes. Symptoms may vary
from headaches and nausea to mild cognitive defects, motor
dysfunction, visual disturbances, loss of consciousness, and
coma. Acute brain injury patterns include diffuse hypoxia- The extent of the lesions
ischemia, cortical and basal ganglia injuries particularly of Fewer lesions are associated with better functional outcome
the globus pallidus. Damage and necrosis of the globus pal-
lidus occurs immediately and only rarely produces parkin- Answer
sonism since the nigrostriatal pathway is partially preserved. 1. CO also inhibits the mitochondrial electron transport
There is no treatment, and management includes sup- enzyme chain and activates polymorphonuclear leuko-
portive measures. The use of hyperbaric oxygen therapy is cytes which cause brain lipid peroxidation leading to the
controversial. late effects of poisoning.

Case
71 CLINICAL HISTORY 26-year-old female with dizziness and giddiness.
FINDINGS Figure 71-1. sagittal T1WI through the fourth Supratentorial structures are generally normal and are best
ventricle. There is inferior descent of the pointed cerebel- evaluated by mutiplanar and multisequence MRI. Cine phase-
lar tonsils (arrow) below the foramen magnum. The fourth contrast MR demonstrates decreased cerebrospinal fluid
ventricle (star) is in normal position. There is a subtle kink- (CSF) flow within the foramen magnum as well as abnor-
ing at the junction of the medulla and the spinal cord (chev- mal CSF pulsation around the brainstem. There is usually no
ron). Midline supratentorial structures are normal without hydrocephalus. The pointed cerebellar tonsils differentiate
obvious hydrocephalus. Figure 71-2. An axial FLAIR CIM from some forms of idiopathic intracranial hypertension
through the level of the foramen magnum. There is oblitera- where there are low-lying cerebellar tonsils with rounded
tion of the cisterna magna by the cerebellar tonsillar ectopia margin. There is always an intracranial mass or mass effect
(arrows). in transforaminal herniation. Low-lying cerebellar tonsils are
usually less than 5 mm below the foramen magnum.
DIFFERENTIAL DIAGNOSISChiari I malformation CIM is one of three forms of Chiari malformations of
(CIM), idiopathic intracranial hypertension, low-lying ton- the posterior fossa. CIIM and CIIIM are more severe hind-
sils, transforaminal herniation. brain malformations and are associated with many other
congenital anomalies both in the spine and in the brain.
DIAGNOSIS Chiari I malformation (CIM). CIVM is a controversial entity that some prefer to call
severe cerebellar hypoplasia. A large number of cerebellar
DISCUSSION The diagnosis of CIM is predicated on tonsillar ectopia is discovered incidentally. Up to 50% of
the presence of a peg-like or pointed inferior descent of patients labeled as CIM are asymptomatic. Patients with
the cerebellar tonsils below the foramen magnum by at cerebellar tonsillar ectopia longer than 5 mm and syringo-
least 5 mm. Other associated findings include oblique ton- myelia are more likely to be symptomatic. The commonest
sillar folia on T2WI, a kink at the junction of the medulla symptoms include those generally associated with hind-
and the spinal cord, and normal position or slight descent brain malformations such as suboccipital headache, neck
of the fourth ventricle with or without syringohydromyelia. pain, vertigo, gait, and ocular disturbances. Patients with
Syringohydromyelia is present in up to 75% of the CIM pop- syringohydromyelia could present with dissociative sen-
ulation. CT shows a crowded foramen magnum with efface- sory symptoms and neuropathic joint. CIM occurs in all
ment of the cisterna magna and normal appearance of the age groups and slightly more common in female than male.
fourth ventricle. MRI is the modality of choice for making There was a 12% positive family history in a published
the diagnosis. The sagittal T1WI is best for demonstrating cohort of 364 symptomatic patients. Skull base dysplasia
the pattern of the cerebellar tonsillar descent. Axial T2WI and scoliosis are present in a significant proportion of these
or FLAIR demonstrates the crowded foramen magnum and patients. The natural history is not well known, and treat-
obliteration of the cisterna magna. ment is controversial but consists mainly of suboccipital
149

150 Case 71
decompression with or without duroplasty to allow normal What the Treating Physician Needs to Know
CSF flow. CIM is usually an incidental finding on cranial or cervical
spine MRI
Question for Further Thought Further imaging evaluation in the appropriate clinical set-
1. How do you differentiate CIM from CIIM? ting such as cine phase-contrast MRI to study the CSF
dynamic at the foramen magnum and cervical thoracic
Reporting Responsibilities spine MRI to exclude syringohydromyelia are appropriate
There is no immediate risk associated with CIM. Routine before instituting management
reporting is usually sufficient. The description of the findings
should include pertinent negatives such as absence of signifi- Answer
cant supratentorial abnormalities, hydrocephalus, or intra- 1. CIIM is usually associated with a small posterior fossa,
cranial masses, to help distinguish this entity from the other elongated or effaced fourth ventricle, tectal beaking, cere-
forms of Chiari malformations and mimics. Suboccipital bellar wrap around the brainstem, some supratentorial dys-
decompression does not cure the headache in idiopathic genetic changes, and hydrocephalus. Myelomeningocele
intracranial hypertension, a distinct differential diagnosis is always present in CIIM, and the incidence of syringo-
that invariably requires a ventriculoperitoneal shunt. So it is myelia is almost 100%.
important to make that distinction.

Case
72 CLINICAL HISTORY 54-year-old female presenting with headache and
right facial nerve weakness.
FINDINGS Figure 72-1. Axial T2WI through the cer- Figure 72-3. Axialpost-contrast T1WI reveals a broad-based
ebellopontine angle (CPA). There is a mass at the right mass with intense, homogeneous enhancement with a dural
CPA with a cleft of cerebrospinal fluid (CSF) separating it tail extending into the right internal auditory canal (IAC)
from the brainstem (arrow), confirming its extraaxial loca- (arrow). Figure 72-4. Coronal post-contrast T1WI confirms
tion. The adjacent brainstem is compressed and displaced the dural-based lesion along the tentorium cerebelli (arrow).
to the left, demonstrating mild edema. The mass is ovoid,
sharply circumscribed, and hypointense. Figure 72-2. Axial DIFFERENTIAL DIAGNOSISVestibular schwannoma,
3D volumetric heavily T2WI. This better demonstrates the dural metastasis, hemangiopericytoma, meningioma and
relationship of the mass to the adjacent neural and vascu- epidermoid.
lar structures including cranial nerves VII and VIII (verti-
cal arrow) and the basilar artery (BA) (transverse arrow). DIAGNOSIS CPA meningioma.
151

152 Case 72
DISCUSSION Meningioma is a well-circumscribed, Questions for Further Thought

extraaxial mass that is typically hyperdense or isodense 1. What is the choice of therapy?
on CT, often with hyperostosis on bone window images. 2. Is the dural tail malignant infiltration?
Meningioma is isointense to hypointense on T2WI and usu-
ally shows intense, homogeneous enhancement. A broad Reporting Responsibilities
dural base is common, with a dural tail more commonly Findings of significant edema and mass effect upon the brain-
seen with meningioma than other dural-based lesions. Dural stem and/or compression of neural or vascular structures
enhancement can extend into the IAC as in this case but usu- must be urgently reported. Images should be scrutinized for
ally does not widen the IAC or blunt the bony margin of the presence of multiple dural-based lesions that may sug-
the porus acusticus. In contrast, vestibular schwannoma fre- gest neurofibromatosis type II or meningiomatosis.
quently widens the IAC and tends to present an acute angle
to the petrous bone. Larger schwannomas are often hetero- What the Treating Physician Needs to Know
geneous and show areas of hemorrhage and cyst formation,
Location and size
less commonly seen in meningioma. A dural tail is uncom-
Relationship to adjacent neural and vascular structures
monly seen with schwannomas. Dural metastases are often
including cranial nerves V, VII, and VIII, BA, or the ante-
nodular and multiple and have a more aggressive appear-
rior inferior cerebellar artery loop within the IAC
ance than meningioma, often with edema in the adjacent
Extension of the mass anteriorly into Meckel cave, inferi-
brain. Epidermoid tumor demonstrates restricted diffusion,
orly into the jugular fossa or extension to the level of the
encases rather than displaces adjacent structures, and rarely
foramen magnum
enhances. Hemangiopericytoma is an aggressive lesion often
with bone erosion and destruction rather than hyperostosis. It
may present extracalvarial component. Answers
Meningioma is most commonly benign and WHO I. 1. Surgery is the treatment of choice, when possible.
Atypical and/or malignant meningiomas, although rare, Stereotactic radiosurgery is considered for surgically
typically show more aggressive imaging features. CPA inoperable lesions or following incomplete resection.
meningioma is the second most common CPA tumor after 2. The dural tail usually represents hypervascular dural
vestibular schwannoma (which accounts for 75% to 80% reaction rather than actual tumoral infiltration. The dural
of CPA masses). Meningioma occurs in middle age and is tail sign is not specific for meningioma. It does not indi-
more common in female. Meningioma often remains asymp- cate malignancy.
tomatic until large and presents with mass effect on adjacent
neural or vascular structures.

Case
73 CLINICAL HISTORY 49-year-old male with a known history of developmental
delay, prior cerebrovascular accident, and pulmonary sarcoidosis with a 3-day
history of encephalopathy.
Figure 73-4
Figure 73-3
FINDINGS Figure 73-1. Axial FLAIR through the mid- the sella. There is thickening and contrast enhancement of
brain. There is thickening of the third cranial nerves bilaterally the chiasm and the infundibulum (vertical arrow). There is
(arrows). Left brainstem atrophy is due to Wallerian degen- bilateral Meckel caves and cavernous sinuses enhancement
eration from left middle cerebral artery (MCA) territory old (transverse arrows). There is a large left MCA territory
infarct. There is thickening of the trigeminal nerves as well encephalomalacia due to old cerebrovascular accident (line
(not shown). Figure 73-2. Axial post-contrast T1WI through arrows).
the brachium pontis. There is thickening and enhancement of
the bilateral trigeminal nerves (arrows). Figure 73-3. Axial DIFFERENTIAL DIAGNOSISMeningitis, sarcoidosis,
post-contrast T1WI through the midbrain. There is thicken- carcinomatosis, lymphoma, Langerhan cell histiocytosis.
ing and contrast enhancement of bilateral third cranial nerves
(arrows). Figure 73-4. Coronal post-contrast T1WI through DIAGNOSIS Neurosarcoidosis (NS).
153

154 Case 73
DISCUSSION MRI is the modality of choice in the evalu- the diagnosis is based on the documentation of systemic
ation of NS, but the imaging manifestations of NS are non- sarcoidosis in the absence of other neurologic diseases to
specific with a long list of differential diagnosis. About 82% explain the symptomatology.
of the patients with NS have positive MR findings. MRI may
detect subclinical NS, but a normal MRI does not exclude the Question for Further Thought
presence of NS particularly in patients with cranial neuropa- 1. Is high-dose steroid effective treatment for cranial nerve
thies only or undergoing corticosteroid treatment. Cranial involvement in NS and do MR findings regress after
nerve involvement is present in up to 50% of patients with treatment?
NS. The more common abnormalities detected by MRI are
nonenhancing periventricular white matter lesions and men- Reporting Responsibilities
ingeal enhancement. Leptomeningeal enhancement is more Direct reporting is necessary in view of the acute encepha-
common in the suprasellar/basal region and to a lesser degree lopathy and the rather serious differential diagnosis.
in the frontal basal region. Other abnormalities are hydro-
cephalus, enhancing brain parenchymal lesions, dural mass
lesions, and spinal cord involvement. Contrast-enhancing
dural masses may simulate meningioma, lymphoma, or Pattern, location, and extent of lesions. A biopsy may be
metastases. Infiltration with contrast enhancement of the needed to confirm diagnosis
pituitary gland, optic chiasm, and hypothalamus is typical Complication of disease such as hydrocephalus
and present in 18% of patients. Lymphoma, Langerhan cell Regression or progression of lesions on follow-up studies
histiocytosis, and carcinomatosis can produce similar find-
ings. However, 50% of the patients with symptoms referable Answer
to the pituitary axis may not have abnormal findings on rou- 1. Most patients show response to steroid treatment. Clinical
tine contrast-enhanced MRI. and imaging changes of cranial nerve involvement fre-
Clinical CNS involvement occurs in 5% of patients quently do not correlate. About 40% of cranial nerve
with systemic sarcoidosis but is found in up to 14% to deficits seen at clinical examination may not be correlated
27% of autopsies. NS primarily develops in the lepto- at imaging. In contrast, 44% of cranial nerves with MR
meninges and results in disruption of the leptomeningeal evidence of sarcoidosis have no symptoms referable to
bloodbrain barrier, resulting in leptomeningeal enhance- the involved cranial nerves. Dysconjugate eye gaze was
ment. This also allows the granulomatous inflammatory the only cranial nerve abnormality detected in this patient.
process to enter the brain parenchyma along the perivas- Clinical resolution often does not imply imaging resolu-
cular spaces. Perivascular involvement and vasculitis may tion. Patients with optic nerve involvement often have
produce cerebral ischemia. NS is a great mimicker, and residual symptoms or no response to treatment.

Case
74 CLINICAL HISTORY Child with caf au lait spots suggesting neurofibromatosis
type1 (NF1) presenting with progressive left nonpulsatile proptosis and decreased
visual acuity on that side.
FINDINGS Figure 74-1. Axial T2WI through the orbits.

There is expansion and hyperintensity of the left optic
nerve with increased fluid under the dural sheath (arrow).
The globe is proptotic and flat posteriorly. Figure 74-2.
Axial post-contrast fat-suppressed T1WI through the
orbits. There is enhancement of the optic nerve compo-
nent (thin arrow) with a central hypointense nonenhancing
portion. There is similar enhancing pattern in the chiasm
(thick arrow). Figure 74-3. Axial T2WI in a companion
patient. There is mild enlargement of both optic nerves
(left greater than right) and no abnormal signal inten-
sity. Figure 74-4. Axial post-contrast T1WI in the same
patient. There is no contrast enhancement in either optic
nerve. Figure 74-5. Axial FLAIR image through suprasel-
lar cistern in the same patient. There is hyperintensity and
Figure 74-5 lobulation of the optic chiasm (arrow).
155

156 Case 74
DIFFERENTIAL DIAGNOSIS Optic neuritis, Optic path- of vision for as long as possible. OPG demonstrate variable
way glioma (OPG) perioptic nerve schwannoma, optic clinical and imaging progression; hence, the treatment is
nerve sheath meningioma, idiopathic orbital inflammatory individualized. Nevertheless, treatment is based primarily on
pseudotumor, and sarcoidosis. a patients progressive functional loss and not on imaging-
evident growth. Chemotherapy with carboplatin, and vincris-
DIAGNOSIS Optic pathway glioma (OPG). tine is the first-line treatment strategy for all patients who
have imaging and/or clinical progression. Surgical excision
DISCUSSION Diagnosis of OPG is based on MRI, and is only rarely indicated in confined tumors that have radio-
biopsy is only considered rarely as a means for a definitive logic progression. Radiotherapy has proven to be helpful in
diagnosis. CT demonstrates well-demarcated and fusiform recurrent and progressive disease.
enlargement of the nerve, often with a tortuous or kinked
appearance as well as enlargement of the optic canal. On Questions for Further Thought
MRI, these gliomas are hypo-isointense on T1WI, hyper- 1. What other central nervous system (CNS) findings can be
intense on T2WI and show variable contrast enhancement. found in a patient with NF1?
The intensity of contrast enhancement may vary from 2. Is there a classification for OPGs?
study to study and has little if any correlation with growth
or malignant transformation and is typically seen in NF1 Reporting Responsibilities
patients. Optic neuritis usually shows minimal optic nerve Direct reporting is necessary in OPG. Describe the extent
swelling with T2 hyperintensity and contrast enhancement. of the lesion and explain any changes in size with respect to
Schwannoma and meningioma could be focal or fusiform prior studies. Recognize other CNS imaging findings that are
and show avid contrast enhancement. Sarcoidosis and orbital associated with NF1.
inflammatory pseudotumor may also involve the extraocular
muscles and lacrimal glands. What the Treating Physician Needs to Know
OPGs are neoplasms derived from astrocytes that are part
Is there a confined disease that would benefit from radical
of the precortical visual pathway usually found within the
surgery?
optic nerves, but these tumors can occur anywhere along the
optic pathway. OPGs typically affect young children with a The evolution of the disease (stability, progression, or
peak incidence between 2 and 8 years of age. OPGs repre- regression if treated) on follow-up
sent about 3% to 5% of childhood brain tumors and gener-
ally have a more benign histology usually WHO I juvenile Answers
pilocytic astrocytomas and diffuse fibrillary astrocytomas in 1. Other CNS findings in NF1 include T2WI focal areas of
this population. Rarely, they occur in adults and tend to have hyperintensity in the basal ganglia, brainstem, and cere-
a more malignant histology, WHO III or IV anaplastic astro- bellum (so-called areas of dysplastic or vacuolar myelin),
cytomas or glioblastoma multiforme. Children diagnosed other CNS tumors (juvenile pilocytic astrocytoma or dif-
with OPGs may have NF1 (about 30%), and when bilateral fuse brainstem glioma), progressive sphenoid wing dys-
it is almost pathognomonic for NF1. Patients with OPG may plasia, lambdoid suture defects, and dural calcification at
be asymptomatic or may present with ophthalmologic com- vertex, moyamoya phenomenon, and buphthalmos.
plaints that vary depending on location and include decreased 2. Yes, the Dogde and modified Dodge classification (DC and
visual acuity, color vision loss, visual field defects, relative MDC, respectively), which consists of stage Alimited
afferent pupil defects, strabismus, nystagmus, proptosis, and to one or both optic nerves, stage Binvolves the chiasm,
optic disc pallor. and stage Cextension toward the posterior pathways,
The management of OPG although controversial among hypothalamic involvement, leptomeningeal dissemina-
neuro-ophthalmologists has as a main goal the preservation tion, or other NF1 findings.

Case 75 CLINICAL HISTORY 3-month-old male with progressive encephalopathy, weakness,
difficulty breathing, and cardiomyopathy.
FINDINGS Figure 75-1. Axial T2WI. There are bilateral that all metabolites are low and presence of large inverted
symmetrical hyperintense lentiform nuclei which appear lactate doublet peak from a voxel obtained in the basal gan-
swollen. There is high signal and atrophy of the occipi- glia.
tal white matter (WM) and cortical sulci prominence. Mitochondrial disorders, also known as respirator chain
Figure75-2. MRS at TE = 135 ms obtained at the level of disorders, are relatively common (1:10,000 live births). Most
the left basal ganglia. There are reduced overall concentra- involve several organs including but not limited to the brain,
tions of choline (Cho), creatine (Cr), and N-acetyl aspartate heart, skeletal muscles, kidneys, and liver. Some mitochondri-
(NAA). The inverted peak of lactate is clearly seen (arrow). opathies present in newborns while others later in life when the
energy demands overwhelm an already compromised energy
DIFFERENTIAL DIAGNOSISDiffuse anoxia (including pathway, thus presenting as exercise intolerance. One of the
inhalation of toxic gases), hypoglycemia, organic acidurias, most common is MELAS which predominantly affects the
acute necrotizing encephalitis (predominantly thalamic deep gray matter structures but may also result in cortical and
involvement), hemolytic uremic syndrome, and thrombotic subcortical infarctions. In MELAS, MRS shows low metabo-
thrombocytopenic purpura mitochondrial encephalopathy lites and lactate even in the normal appearing brain. Another
with lactic acidosis and strokes (MELAS). relatively common disorder is Kearns Sayre syndrome which
presents with predominantly ophthalmoplegia, and thus, MRI
DIAGNOSIS Mitochondrial encephalopathy with lactic aci- shows pronounced abnormalities in the dorsal midbrain but
dosis and strokes (MELAS). also throughout the white matter. Leigh syndrome is caused by
at least four different enzymatic defects, but on MRI their phe-
DISCUSSION MR T2WI and FLAIR image show high notypic manifestations are similar (basal ganglia, brainstem
signal and swelling of the basal ganglia, thalami, and the [especially the cranial nerve nuclei], and territorial infarctions)
midbrain. The WM may also show high signal intensity, and and do not permit their differentiation. Alper disease which is
these areas may have restricted diffusion on ADC maps. As accompanied by liver cirrhosis and Menkes kinky hair syn-
in this patient, cortical atrophy may be present. MRS shows drome are also considered as mitochondrial disorders. In the
157

158 Case 75
former, the brain abnormalities tend to be occipital, while in What the Treating Physician Needs to Know
the latter there is diffuse white matter involvement, brain atro- Extent of the disease and involvement of critical areas
phy, and very tortuous intracranial arteries. Confirmation of all such as motor, visual, and auditory cortex
mitochondriopathies is a genetic one. Extent of brainstem involvement which may explain respi-
ratory and swallowing difficulties
1. Why is the basal ganglia predominantly affected? Answers
2. How can one be sure that one is looking at lactate on MR 1. In any systemic disorder leading to energy failure, the
spectroscopy? basal ganglia and thalami are first affected because their
metabolic rate is higher than that of the rest of the brain.
Reporting Responsibilities Other regions with high metabolic rates such as the motor
Routine reporting is sufficient unless there is evidence of cortex, visual cortex, and brainstem cranial nerve nuclei
restricted diffusion. Suggest the diagnosis when the basal are also acutely affected.
ganglia, thalamic or cerebral infarctions occur and suggest 2. Lactate is located at 1.3 to 1.4 parts per million in the
obtaining MRS to document the presence of lactate. You may spectra and has the typical doublet peak configuration.
suggest the type of mitochondrial disease according to the It resonates below the baseline at TE of 135 to 145 ms and
site of predominant involvement. above the baseline at all other echo times.

Case
76 CLINICAL HISTORY 45-year-old female with a history of anoxic
encephalopathy.
159

160 Case 76
stage. In the setting of HIE, deep GM is usually affected.

CT also shows cortical hypodensity in the affected area in
the acute setting. Subsequently hyperdense gyri have been
demonstrated in the chronic stage which may reflect calcifi-
cations or hemorrhagic conversion. Vascular imaging, CTA
or MRA, may be useful for evaluation of the vascular struc-
tures particularly in focal CLN. MRS may show presence of
lactate and lactic acid in the affected areas.
CLN is a sign of profound anoxic damage. It is found in
all ages and has no gender predilection. It most commonly
occurs in the setting of HIE. The most common cause of HIE
in the adult are cardiac arrest and respiratory failure. CLN is
known to occur as a complication of traumatic brain injury,
Figure 76-5 posterior reversible encephalopathy syndrome (PRES), sta-
tus epilepticus, hypoglycemia, mitochondrial disorders,
electrocution, strangulation, near drowning, toxins, menin-
FINDINGS Figure 76-1. Axial DWI through the basal gan-
goencephalitis, and ischemic infarcts. The GM is hypermet-
glia. There is diffuse cortical hyperintensity (arrows) along
abolic and is preferentially affected by global ischemia or
with similar changes in the caudate and putamen bilaterally
hypoxic changes. CLN is a sign of selective vulnerability of
resulting in pronounced whitegray matter (WMGM) dif-
the cortical GM by HIE. The third cortical layer is the most
ferentiation. Figure 76-2. Corresponding T2WI. The cortical
vulnerable, followed by the fifth or sixth, with the second and
GM and the basal ganglia are hyperintense with pronounced
fourth being relatively more resistant to ischemic necrosis
WMGM differentiation. Convexity sulci are effaced due
from hypoperfusion. Patient may recover from focal involve-
to swelling of the cortical tissue. Figure 76-3. Axial T1WI
ment, but rarely is recovery from global CLN possible, and
without contrast through the frontal lobes in another patient
the prognosis is generally poor with devastating and severe
who had an anterior communicating artery aneurysm repair.
neurologic deficits, including severe memory loss, personal-
There is bilateral thin cortical curvilinear hyperintensity
ity changes, permanent vegetative state, or death. There is no
(arrows) covering the hypointense thickened cortical GM in
known cure, and treatment is supportive.
the bilateral frontal lobes. Figure 76-4. Axial FLAIR through
same level as Figure 76-3. There is bilateral curvilinear
hyperintensity over the frontal lobes (arrows) with frontal
cortical GM thickening and hyperintensity. Figure 76-5. 1. What is responsible for the cortical thin hyperintensity on
Axial GRE through the frontal lobes. There is a pencil-thin the T1WI?
linear hypointensity over the frontal lobes (arrows).
DIFFERENTIAL DIAGNOSISCortical laminar necrosis Direct reporting is essential in view of the acute nature of the
(CLN), hypoxic ischemic encephalopathy (HIE), hemor- disease. Other associated changes that may give a clue to the
rhagic conversion of ischemic infarcts, encephalitis, cortical underlying process should be reported.
calcification.
DIAGNOSIS CLN in hypoxic ischemic encephalopathy (HIE). Location and extent of the changes
This is a sign of profound hypoxia from which there is
DISCUSSION The most efficient way to image acute CLN rarely a good recovery
or HIE for that matter is by MRI. DWI in the acute setting
shows diffuse cortical GM hyperintensity with correspond- Answer
ing hypointensity on ADC consistent with restricted diffu- 1. There is no consensus regarding the cause of T1 hyper-
sion. Areas of restricted diffusion correlate anatomically intensity in CLN. The underlying pathology of CLN at
with areas of infarction at histology. There is corresponding autopsy has been consistent with necrotic debris through-
T2 hyperintensity of the GM. There is swelling of the GM out the entire cortex without hemorrhage or calcification.
with effacement of the convexity sulci. There is usually no However, some have demonstrated the presence of min-
contrast enhancement in the acute stage but may occur in eralization such as calcifications or traces of iron in CLN,
the subacute CLN. In the chronic stage, there is a thin cor- while others have described pan-necrosis with fat-laden
tical curvilinear hyperintensity on T1WI and FLAIR over macrophages in the affected regions. GRE imaging has
the cortical GM. The cortical T1 hyperintensity may mimic also showed that cortical curvilinear hyperintense lesions
cortical hemorrhage or mineralization. In this case, there is on T1WI did not represent hemorrhage in most cases.
a thin curvilinear hypointensity over the frontal lobes on SWI has shown that a very small proportion of CLN had
GRE. Brain swelling has receded, and there may be under- dotted hemorrhage in the region of T1 hyperintensity, but
lying encephalomalacia and volume loss in the chronic most areas of CLN showed no hemorrhage on SWI.

Case
77 CLINICAL HISTORY 67-year-old female involved in a motor vehicle
collision.
FINDINGS Figures 77-1 and 77-2. Contiguous axial NCCT infarct. There is unilateral right uncal herniation (UH). There
through the suprasellar cistern. There is effacement of the is deformity of the right side of the suprasellar star confi
suprasellar cistern (star) by medially projecting bilateral guration and compression of the right perimesencephalic
unci. There is side-to-side compression of the brainstem cistern and midbrain by the shifted right uncus (arrow in
and obliteration of the perimesencephalic cisterns (arrows). Figure 77-3). Figure 77-4 demonstrates the overflow of the
There are bilateral frontal lobe contusions and bilateral right uncus over the free edge of the right tentorium behind
fronto-temporal fossa extraaxial hemorrhages. the dorsum sella and anterolateral to the upper pons (arrow).
Figures 77-3 and 77-4. Contiguous axial MRI FLAIR
through the suprasellar cistern in a different patient with DIFFERENTIAL DIAGNOSIS Bilateral UH, transtentorial
right middle cerebral artery (MCA) territory acute ischemic herniation (TTH).
161

162 Case 77
DIAGNOSIS UH. fatal. Milder form as in Figures 77-3 and 77-4 may not have
such a drastic impact but should be carefully monitored.
DISCUSSION UH could be difficult to identify in the early
stage on CT but easily recognized on MRI because of MRIs Question for Further Thought
ability to demonstrate structures in this region well without 1. What are the boundaries of the six-pointed star suprasellar
significant artifacts. In the acute phase of trauma, the CT is cistern?
usually what is available and reasonable. There is partial or
complete effacement of the suprasellar cistern depending on
whether the herniation is unilateral or bilateral. In a unilat-
This is an acute situation requiring direct reporting. The
eral UH, there is flattening of the affected side of the pointed
degree of shift, other associated herniations such as subfal-
suprasellar star and perimesencephalic cistern, compression
cine herniation (SFH), TTH, and the cause of the herniation
of the cerebral peduncle, and displacement of the brainstem
should be documented. Other injuries and complications if
to the contralateral side with widening of the ipsilateral cer-
there are any should also be reported.
ebellopontine angle (CPA) cistern. The herniating uncus
may subsequently project posteriorly to the dorsum sella and
anterolateral to the pons. In a bilateral setting, the midbrain,
upper pons, and perimesencephalic cisterns are flattened Degree of herniation and laterality or if bilateral
from side to side with anteroposterior elongation. UH is due Associated primary or secondary injuries
to displacement by a low temporal fossa mass, swelling, or Complications if present
extraaxial collection. However, UH is always a component of
TTH due to a more cephalad parenchymal or extraaxial mass. Answer
The clinical consequences of a unilateral UH are ipsilat- 1. The boundaries of the suprasellar cistern are as follows:
eral hemiparesis due to Kernohan notch (deformity created the cerebral peduncles posteriorly enclosing the inter-
by compression of contralateral midbrain against the contra- peduncular cistern in the midline (posterior point of the
lateral tentorium) and compression of the ipsilateral third cra- star), the bilateral temporal lobes unci laterally separated
nial nerve producing the classical dilated nonreactive pupil. from the cerebral peduncles by the perimesencephalic
Complications could include compression of the posterior cisterns (posterolateral points of the star), and the pos-
cerebral artery (PCA), superior cerebellar artery (SCA), terior aspect of the gyrus rectus and orbital gyrus form
and the anterior choroidal arteries resulting in infarctions the anterior boundary on either side enclosing the inter-
in the territories of these vessels. These complications are, hemispheric fissure in the midline (the anterior point of
however, greatest with TTH. Duret hemorrhage, brainstem the star) with the lateral fissures separating the frontal and
edema, venous congestion, and respiratory center failure are temporal lobes forming the anterolateral points of the star
complications of severe UH and TTH. UH could be rapidly on either side.

Case
78 CLINICAL HISTORY 52-year-old female was admitted with mental status changes.
FINDINGS Figures 78-1 to 78-4. Axial MR DWI and and centrum semiovale, respectively, showing smudgy
corresponding ADC maps through the corona radiata T2 hyperintensity in bilateral corona radiata and centrum
(Figures78-1 and 78-2) and the centrum semiovale semiovale. MRI done 5 days before this set (not shown)
(Figures78-3 and 78-4) demonstrating bilateral mainly did not demonstrate any significant WM diffusion restric-
posterior smudgy or confluent white matter (WM) tion. There was no contrast enhancement.
restricted diffusion in the posterior corona radiata and
smudgy/confluent restricted diffusion throughout the DIFFERENTIAL DIAGNOSIS Toxic leukoencephalopathy,
entire WM of the bilateral centrum semiovale (arrows). delayed WM hypoxic ischemic encephalopathy (HIE) (delayed
There is sparing of the subcortical U fibers. Figures78-5 posthypoxic leukoencephalopathy, DPHL), leukodystrophy,
and 78-6. Axial FLAIR images through the corona radiata acute disseminated encephalomyelitis (ADEM).
163

164 Case 78
DIAGNOSIS Delayed posthypoxic leukoencephalopathy It is well known that anoxic or hypoxic injury produces
(DPHL). acute neurologic deficits with changes visible in the deep or
cortical GM. GM is very susceptible to hypoxia. It is less
DISCUSSION The classical imaging findings of DPHL well known that severe neurologic consequences may be
are bilateral symmetrical WM confluent restricted diffu- delayed for days or weeks after the injury and that the cor-
sion with similar T2 hyperintensity in the cerebral hemi- responding imaging findings may be delayed even later and
spheres days or weeks following a hypoxic event. Initial confined to the WM. The temporal separation between the
imaging at the time of initial insult is often normal. injury and the neuropsychiatric consequences and/or imag-
Depending on the cause of the initial insult, cerebellar and ing findings presents a diagnostic challenge. It has been
internal capsular lesions may also be present particularly reported that 3% of victims of acute CO intoxication have
in heroin-related hypoxic changes known as chasing the delayed neurologic sequelae 2 to 40 days (mean 2.4 days)
dragon. The deep or superficial gray matter (GM) is usu- after the initial injury. The delay is caused by the selective
ally spared but imaging changes in these structures have necrosis of myelin-producing glia cells in the border zones
been reported. The WM changes are reversible along with of the WM. Underlying pathology may include WM vacu-
excellent clinical recovery over the long term. It may be olation, gliosis, and spongiform changes.
difficult to exclude the differential diagnoses at imag- The rarity of this condition seems to suggest unidentified
ing without the benefit of the history. The syndrome of individual susceptibilities to hypoxic neuronal injury. An
delayed neurologic deterioration with cerebral demyelin- extensive workup for inflammatory, infectious, paraneoplas-
ation has been reported in the setting of carbon monoxide tic, vascular, metabolic, and inherited leukodystrophies was
(CO) poisoning. The basal ganglia are usually involved in undertaken and yielded no positive answer in this patient.
CO poisoning. However, this syndrome is now more com- Increased myelin basic protein (MBP) in the cerebrospinal
monly seen in the context of drug overdose with heroin or fluid (CSF) and abnormal imaging were the only abnor-
benzodiazepine and in other situations that lead to severe malities found. Management is generally supportive and the
hypoxia. long-term prognosis is good.
A month prior to her current admission this patient was
found unresponsive. She was found to be in cardiogenic Questions for Further Thought
shock with shocked liver and acute renal failure. She was 1. Is DPHL always preceded by unresponsiveness?
treated, improved close to her baseline, and was sent home. 2. What is the difference between HIE and DPHL?
She reportedly started feeding dirt to the family pet and wore
her underwear on the outside of her pants. She was not able Reporting Responsibilities
to ambulate without support as she was weak and unsteady. The restricted diffusion demands prompt and direct report-
She was readmitted 2 weeks following her initial discharge ing. The predominant WM location of lesions should evoke
with mental status change. Neuropsychiatric behavior is a ischemic or toxic leukoencephalopathy. High index of suspi-
prominent feature of DPHL. Seizure may also be a feature cion is necessary in this rather uncommon presentation of a
of this disorder. common problem.

Case 78 165

Absence of complications such as brain swelling and her- 1. DPHL is always preceded by unresponsiveness except in
niations cases associated with CO poisoning.
CSF evaluation is necessary and LP is safe in the absence 2. DPHL is a rare complication of HIE occurring in 2.75%
of mass effect of patients with CO poisoning. While HIE mostly affect
Most patients improve within 3 to 6 months. The likeli- GM, DPHL is mostly a WM disease. It is not well under-
hood of recovery is inversely related to the patients age stood why only a small subset of HIE develop DPHL.

Case
79 CLINICAL HISTORY 68-year-old male with gastric carcinoma and new-onset
blurring of vision and myelopathy.
Figure 79-1
Figure 79-2
Figure 79-3
FINDINGS Figure 79-1. Axial post-contrast T1WI through

the cerebellopontine angles (CPAs). There is nodular con-
trast enhancement around the bilateral CN VII and VIII both
in the cisternal and in the intracanalicular segments (verti-
cal arrows). There is similar but smaller nodular enhance-
ment along the right CN VI (transverse arrow). Figure 79-2.
Axial post-contrast T1WI through the trigeminal nerves.
There is nodular but somewhat smooth enhancement along
the bilateral trigeminal nerves (transverse arrows). There
is also smudgy contrast enhancement around the cerebel-
lar folia (vertical arrow). Figure 79-3. Axial post-contrast
T1WI through the interpeduncular cistern. There is nodu-
lar and linear contrast enhancement on the surface of the
cerebral peduncles (transverse arrows). Figure 79-4. Post-
contrast sagittal T1WI through the cervical spine. There is
fine nodular enhancement of the surface of the spinal cord
and brainstem (arrows). FLAIR images (not shown) dem-
onstrated hyperintensity along CN VII and VIII and sur-
rounding the superior cerebellar folia. Other noncontrast
images did not show any significant abnormality other than
mild ventriculomegaly.
DIFFERENTIAL DIAGNOSISLeptomeningeal metasta-

ses (LM), sarcoidosis, drop metastases, lymphoma.
DIAGNOSIS Leptomeningeal metastases (LM).
DISCUSSION MRI is the examination of choice in the

evaluation for LM. Findings that constitute LM include
nodular or diffuse leptomeningeal enhancement, nodular or
diffuse thickening, and enhancement of cranial nerves as in Figure 79-4
166

Case 79 167
this patient, ependymal enhancement, subarachnoid space The common central nervous system (CNS) malignancies
(SAS) FLAIR hyperintensity, leptomeningeal thickening seeding the CSF spaces include medulloblastoma, ependy-
or nodules on non-contrast studies, and hydrocephalus. The moma, glioblastoma (GB), germinoma, and pineoblastoma.
pattern of enhancement may vary from thin or thick sheets LM risk rises with longer cancer survival and is usually a
of contrast enhancement to miliary and large masses. The late complication of cancer occurring in about 5% of cancer
tumor may seed the leptomeninges diffusely or multifocally. patients often accompanying systemic relapse of the disease.
The hydrocephalus could be on the basis of cerebrospinal Treatment is very ineffective prolonging life by mere weeks.
fluid (CSF) pathway obstruction or impaired CSF absorption
at the arachnoid granulations by cellular debris or high CSF Question for Further Thought
protein resulting in high viscosity of the CSF. Some of these 1. Does steroid use have any effect on detection of LM?
findings are shared with other nonneoplastic inflammatory
entities in the SAS from which LM must be distinguished Reporting Responsibilities
for appropriate management. The best imaging modality to Direct reporting is required. Improved survival follows
make that diagnosis is contrast-enhanced MRI of the brain prompt and immediate treatment. Location and pattern
with a sensitivity of 59% in cytologically proven CSF seed- of LM as well as complications such as ventriculomegaly
ing compared with 41% for contrast-enhanced FLAIR and should be reported. Associate parenchymal metastases may
12% for unenhanced FLAIR. The yield of unenhanced MRI alter the pattern of treatment.
and CT is very poor indeed. The ultimate proof of LM is by
cytology of the CSF. What the Treating Physician Needs to Know
The CSF cytology on the first lumbar puncture (LP) in
Pattern of LM
this patient following the MRI was positive for metastatic
adenocarcinoma. It also showed high-protein, low-glucose, Is it safe to perform LP? LP may not be safe if there are
and numerous white cells. LM is a difficult diagnosis to make associated masses that can result in brain shift during LP
with the yield of neoplastic cells in the CSF rising from 45%
to 61% in the first sample to 72% to 88% in the third sample. Answer
The prognosis is very poor indeed with the median survival 1. Steroid is known to seal off the bloodbrain barrier
for patients with LM ranging from 8 to 16 weeks despite (BBB) preventing contrast enhancement of otherwise
the best available treatment. Hematologic malignancies can contrast-enhancing tumors. It has been found at least in
result in LM in up to 24% of patients. The most common one study that steroid has a paradoxical effect on LM;
solid tumors causing LM are breast cancer, lung cancer, more contrast-enhancing LM are detected in patients on
and melanoma with incidences ranging between 5% and steroids compared with those not on steroid. The reason
23%. Stomach and pancreatic cancers seed less frequently. for this is not clear!

Case
80 CLINICAL HISTORY 59-year-old female with sudden onset of left-sided weakness.
168

Case 80 169
FINDINGS Figure 80-1. Axial NCCT through the basal mass typical of acute hemorrhage. Fluid levels can occur
ganglia. There are two hyperdense masses in the right basal in the hyperacute stage or if a bleed occurs into an exist-
ganglia, one in the right globus pallidus (transverse arrow) ing mass. Acute bleed could be hypodense in the anemic
and the larger lateral one straddles the putamen and external patient since the density is due to the hemoglobin protein.
capsule (vertical arrow). Both communicate superiorly (not Presence of mass effect depends on the size of the hema-
shown). There is effacement of the right frontal horn due toma. The basal ganglia are one of the classical locations of
to mass effect. Figure 80-2. Axial DWI through the basal hypertensive hematoma. Other locations include the brain-
ganglia. There is heterogeneous area of restricted diffusion stem, thalamus, and the cerebellum. However, up to 20%
in the right basal ganglia. Figure 80-3. Axial T1WI through of hypertensive hematoma occurs elsewhere in the brain
the basal ganglia. The right basal ganglia mass is mainly outside these primary areas. MRI is useful for further char-
isointense with thin surrounding hypointense halo. There is acterization of the hemorrhage and to visualize presence
effacement of the right sylvian fissure (arrow). Figure 80-4. of associated lesions such as microhemorrhages, lacunar
Axial GRE through the basal ganglia. There is heteroge- infarcts, and white matter (WM) changes that may not have
neous blooming of the mass (arrow). Figures 80-5 and 80-6. been well characterized by CT. The MRI pattern of hema-
Axial T2WI and FLAIR through the mass. The mass has toma depends on the age of the hematoma primarily due to
similar heterogeneous pattern of mixed hyperintensity and the state of the hemoglobin. In this case, MRI was obtained
isointensity with local mass effect and midline shift. There within 24 hours of the ictus, and the pattern is somewhere
is mild surrounding hyperintensity consistent with vaso- between the hyperacute and acute stages. The typical
genic edema (arrows). hyperacute hematoma contains intracellular oxyhemoglo-
bin which has diamagnetic property and is hyperintense
DIFFERENTIAL DIAGNOSIS N/A. on T2WI and hypointense on T1WI. The acute hematoma
contains intracellular deoxyhemoglobin with paramagnetic
DIAGNOSIS Acute hypertensive right basal ganglia property. It is hypointense on T2WI and GRE and isoin-
hematoma. tense on T1WI. Usually in the hyperacute/acute stages,
surrounding edema and mass effect begin to develop. If the
DISCUSSION CT remains the fastest and the most hematoma is large enough, it may result in herniations and
efficient way to image acute bleed. The NCCT obtained dissect into the ventricles. Hydrocephalus is a complica-
within hours of the ictus shows a homogeneous hyperdense tion of intraventricular hemorrhage (IVH).

170 Case 80
Chronic hypertension is the most common cause of pri- Reporting Responsibilities

mary nontraumatic intracerebral hematoma (PICH) with the Direct reporting is essential. Complications such as mass
location mostly central in the brain. Other causes of PICH effect, herniations, IVH, or subarachnoid hemorrhage (SAH)
include amyloid angiopathy, mostly subcortical in location. should be reported.
Use of street drugs such as cocaine, amphetamines, and other
stimulants can cause intracerebral hematoma (ICH). The What the Treating Physician Needs to Know
classic clinical presentation of acute hemorrhage is that of Location and size of hematoma
acute neurologic deficits, mental status changes, and coma. Complications
This patient has a history of hypertension, hyperlipidemia, Associated lesions such as WM changes, microhemor-
coronary artery disease, and diabetes mellitus type 2. The rhages and their location, and other suspicious signs of
basis for hypertensive bleed remains debated. It is generally secondary intracerebral hematoma (SICH)
believed that chronic hypertension results in intimal hyper- Further evaluation by MRA or CTA in the appropriate situ-
plasia and hyalinization of the end or penetrating arteries ation
in the brainstem, cerebellum, thalamus, and basal ganglia.
These hyalinized vessels become narrow predisposing to Answer
necrosis and formation of small pseudoaneurysms known 1. Early subacute ICH contains methemoglobin within the
as Charcot-Bouchard aneurysms. These aneurysms rupture red cells resulting in hypointensity on T2WI and GRE
giving rise to hemorrhage. The hemorrhage is interstitial in and hyperintensity on T1WI. The mass effect is persis-
location splitting tissue; hence, the appearance is more often tent. The late subacute stage has extracellular methemo-
ovoid and linear than round. The prognosis depends on size globin resulting in hyperintensity on all sequences. It may
and location with brainstem lesions generally fairing the begin to show peripheral contrast enhancement. Chronic
worst. hematoma contains mostly ferritin, and hemosiderin
which are isointense to hypointense on all sequences. The
Question for Further Thought edema and mass effect have resolved with a central cleft
1. What are the MRI changes in subacute and chronic mainly containing cerebrospinal fluid (CSF) or residual
hematoma? methemoglobin.

Case
81 CLINICAL HISTORY 4-month-old child with microcephaly, severe irritability,
and diminished deep tendon reflexes.
FINDINGS Figure 81-1. Axial T2WI through the corona hyperintensity (arrows). Figure 81-3. Parasagittal T1WI
radiata shows symmetrical bilateral deep hemispheric white in another companion case shows enlarged optic nerve
matter (WM) hyperintensity (stars). Figure 81-2. Axial (arrow). Figure 81-4. Axial T2WI in an end-stage patient.
T2WI through the cerebellum in a companion case. There There is marked cerebral volume loss and hyperintense
is bilateral medial cerebellar (dentate nuclei) symmetrical atrophic WM.
171

172 Case 81
DIFFERENTIAL DIAGNOSISAdrenoleukodystrophy present between the third and sixth months of life, and most
(ALD), metachromatic leukodystrophy, maple syrup urine die within the first few years of life. There is no cure for the
disease, Tay-Sachs, Krabbe disease. disease.
DIAGNOSIS Krabbe disease (globoid cell leukody Questions for Further Thought
strophy). 1. How is the definitive diagnosis of Krabbe disease made?
2. How can DTI be helpful in evaluation of these patients?
DISCUSSION NCCT classically demonstrates hyper-
density in the bilateral thalami and occasionally within the Reporting Responsibilities
caudate nuclei, dentate nuclei, and corona radiata. MRI find- Direct reporting may be appreciated because of the non-
ings are nonspecific with periventricular, pyramidal tracts specific nature of the findings and the rapidly fatal outcome
and medial cerebellar dentate nuclei T2-hyperintensity. of the disease. Describe the MR or CT findings and give a
Thalamic T2 hypointensity may be present. Splenium of differential diagnosis. Exclude other focal brain lesions that
the corpus callosum involvement can be seen in the late- could account for the patients symptoms.
onset variant of the disease. There is no associated enhance-
ment within the brain parenchyma, although interestingly,
the cranial (including the optic chiasm) and spinal nerves
may be hypertrophied and can enhance. Late stages of the Are there any findings that may help to narrow the differ-
disease demonstrate volume loss, similar to that seen in late ential diagnosis?
stages of other dysmyelinating diseases. ALD classically Is there any further imaging that may be helpful?
affects peritrigonal WM and occurs in a much older popula-
tion of children. Answers
Krabbe disease is a rare autosomal recessive lysosomal 1. Leukocyte or skin fibroblast B-galactosidase assay.
disorder secondary to galactocerebroside B-galactosidase 2. DTI demonstrates more abnormalities than T2 images
enzyme deficiency, which results in a deficiency of an and provides a quantitative measurement of severity of
important component of the myelin sheath. Psychosine accu- the disease process. This also allows for an optimal way
mulates in excess and is toxic to the brain. Most patients to monitor treatment effects.

Case
82 CLINICAL HISTORY 28-year-old female with no significant past medical
history presented to the Emergency Room with a 3-week history of severe headache
and lethargy.
Figure 82-2
Figure 82-1
With permission from Applied Neurology UBM Medica. Case previously
published in Appl Neurol. 2007;3:3537.
Figure 82-4
thin non-contrast-enhancing wall and apparently communi-

cates with the right frontal horn (arrow). There is midline
shift from right to left as demonstrated in Figure 82-1. Figure
82-3. Axial FLAIR image through the pons showing a right
Figure 82-3 perimesencephalic or pontine cyst (possibly on the surface
of the pons) with surrounding somewhat thick T2 hyperin-
FINDINGS Figures 82-1 and 82-2. Axial and sagittal tensity (arrow) presumably arachnoid inflammation and/or
post-contrast T1WI, respectively, through the frontal lobe pontine gliosis or edema. Figure 82-4. Axial FLAIR through
showing a huge multiloculated cerebrospinal fluid (CSF) the giant cyst in the right frontal lobe showing internal septa-
intensity cyst in the right frontal lobe with compression of tions (arrows), midline shift from right to left, surrounding
the right lateral ventricle. The cyst is surrounded by a very confluent T2 hyperintensity anteromedially and posteriorly
173

174 Case 82
(line arrows) consistent with edema or gliosis. There is also NCC is an infection caused by the larval stage of the tape-
confluent T2 hyperintensity around the dilated left lateral worm Taenia solium. This infestation is the most common
ventricle frontal and occipital horns (chevrons) consistent parasitic disease of the human CNS and the most common
with transependymal CSF permeation. Lineal hyperintensi- cause of acquired seizures in Central and South America. In
ties are present in the subarachnoid spaces consistent with the United States, NCC is usually diagnosed in immigrant
meningitis. populations from endemic areas. The vesicular stage of the
disease is inactive. The three stages of degeneration include
DIFFERENTIAL DIAGNOSIS Neurocysticercosis (NCC), the colloidal, nodular/granular, and calcified nodular.
neuroepithelial cyst, porencephalic cyst, giant perivascular Seizures are the most common presentation of NCC (78.8%).
space, hydatid cyst. Other presentations include headaches, focal neurologic def-
icits, meningitis, gait abnormalities, visual changes, altered
DIAGNOSIS Neurocysticercosis (NCC) giant vesicular cyst. mental state, and cranial nerve palsies. Intraventricular cyst
could present with intermittent obstruction. The diagnosis of
DISCUSSION Giant parenchymal NCC vesicle is a rare NCC is based on a combination of epidemiologic data, signs
presentation of the vesicular stage of NCC. The imaging fea- and symptoms, serologic test (ELISA), brain imaging, and
tures of the vesicular stage of NCC consist of a cyst with a pathology (if available). Treatment consists of a combination
tiny eccentric calcification representing the scolex. On CT of antiparasitic drugs, treatment of raised intracranial pres-
the cyst is usually small, 5 to 20 mm in size or a cluster of sure (ICP), and surgery for obstructive symptoms.
smaller cysts, of CSF density and the calcification is hyper-
dense. MRI usually shows a small cyst of CSF intensity on all Question for Further Thought
sequences with an eccentric tiny focal hypointensity on GRE 1. Should MRI of the spine be performed in patients diag-
and T2WI or hyperintensity on DWI and FLAIR represent- nosed with basal subarachnoid neurocysticercosis?
ing the scolex. There is usually no surrounding edema, brain
reaction, or contrast enhancement. Large parenchymal cysts
more than 5 cm in size have been reported. They may have
Direct reporting is essential in this rather large mass.
significant mass effect and surrounding edema with smooth
Herniation and hydrocephalus make reporting more urgent.
ring enhancement. Large cysts up to 12 cm are usual in the
Other imaging findings to look out for include infarcts due to
racemose NCC. The cyst or cluster of cysts are within the
arteritis, leptomeningeal enhancement raising the possibility
subarachnoid space most frequently in the sylvian fissures
of arachnoiditis, and presence of intraventricular cysts.
or around the rostral brainstem insinuating along the brain
surface with compression of the brain parenchyma. There
is usually no surrounding edema or contrast enhancement.
Hydrocephalus could be a feature due to meningitis, arach- Location and number of cyst or cysts. Presence of other
noiditis, and CSF pathway obstruction. This cyst resembles stages of the disease in other parts of the brain
a racemose NCC which often can grow into parenchymal Presence of hydrocephalus
form. The scolex calcification is not usually visualized in the Leptomeningeal changes suggesting meningitis or arach-
racemose cyst. Extension of leptomeningeal inflammation noiditis
along the perivascular spaces could result in vasculopathy Presence of infarction that may suggest vasculopathy
and ischemic infarcts. Hydatid cyst in the appropriate geo-
graphical region is an obvious consideration that may not be Answer
possible to differentiate by imaging. A porencephalic cyst 1. Patients with basal subarachnoid NCC have common
should not cause hydrocephalus or meningitis. There could involvement of the spinal subarachnoid space. MRI of the
be a thin gliosis surrounding a neuroepithelial cyst and large spine should be performed early after basal subarachnoid
perivascular space. NCC is diagnosed to exclude spinal disease.

Case
83 CLINICAL HISTORY 61-year-old male presenting with memory problems.
Figure 83-1
FINDINGS Figure 83-1. Top row: Axial FLAIR MRI. up the degree of brain atrophy. There are no early CT or MRI
There is mild bilateral temporoparietal volume loss. Second, findings of AD. Functional MRI shows diminished intensity
third, and fourth rows: Axial and surface projection maps and/or delayed activation in the prefrontal cortex and medial
F-18 FDG PET. There is bilateral posterior temporal and temporal lobe, which are primary circuits of learning and
parietal hypometabolism (right greater than left). Posterior memory. The magnitude of FDG temporoparietal metabolic
temporal and parietal hypometabolism with preservation of deficits on FDG-PET correlate well with cognitive impair-
precentral gyrus metabolism is a characteristic feature of ment. Basal ganglia, thalamus, cerebellum, and primary
Alzheimer disease (AD). sensorimotor cortex are usually spared. A normal -amyloid
PET imaging with preserved graywhite differentiation
DIFFERENTIAL DIAGNOSISVascular dementia, fron- excludes the possibility of dementia due to AD. Patients with
totemporal dementia, Lewy body dementia, corticobasal mild cognitive impairment and a positive -amyloid PET
degeneration, normal pressure hydrocephalus. imaging have greater chance of progressing to AD (50% to
60%) than those with a negative amyloid PET study (less
DIAGNOSIS AD. than 4% to 7%). However, amyloid PET study can be posi-
tive even in elderly patients without cognitive difficulties.
DISCUSSION AD is a progressive neurodegenerative AD insidiously starts at the temporal lobe entorhinal
disorder that manifests with gradual deterioration in cogni- cortex and gradually progresses to hippocampus and neo-
tion, behavior, and motor function. It is the most common cortex followed by association areas. Therefore, the earliest
dementia in the elderly followed by vascular and frontotem- affected abilities are learning and short-term memory. Then
poral dementias. The definitive diagnosis is obtained through cognitive loss is enhanced with loss of orientation, long-term
brain biopsy showing accumulation of extracellular senile memory, and personality. The inevitable outcome is behav-
-amyloid plaques and intracellular neurofibrillary tangles ioral changes (e.g., hallucinations), loss of language, visuo-
formed by tau proteins. spatial skills, and eventually motor function. There is no cure
The conventional CT and MRI are a part of the demen- available, but slowing the disease progression or prevention
tia workup primarily to rule out vascular dementia, normal in case of early diagnosis is extensively investigated. Thus,
pressure hydrocephalus, or secondary causes such as intra- early diagnosis is crucial as atrophy translates into irrevers-
cranial mass. Another common use of imaging is following ible neuronal death. The functional imaging techniques
175

176 Case 83
-amyloid PET imaging sought to offer early recognition of What the Treating Physician Needs to Know
the disease, potentially to slow devastating outcomes with A negative -amyloid PET imaging study is valuable to
antiamyloid therapies. exclude AD
A positive study in patients with mild cognitive impair-
Question for Further Thought ment has prognostic implications
1. What is the best follow-up imaging study for evaluating The FDG PET is valuable in disease monitoring of AD
AD progression? and in the diagnosis of frontotemporal dementia and Lewy
body dementia as the spatial pattern of FDG hypometabo-
Reporting Responsibilities lism is characteristic
Routine reporting is sufficient unless there are acute findings.
The diagnosis of AD is clinical. Primary benefit of structural Answer
imaging (CT and MRI) is to rule out intracranial mass or 1. -amyloid PET imaging is valuable in the early diagnosis
acute infarct in an elderly patient. Secondary benefits are or exclusion of AD. FDG PET/CT and MRI changes cor-
adjunctive providing areas of focal cerebral atrophy to sup- relate best with disease progression and can be used for
port the diagnosis. evaluating patients with AD in follow-up.

Case
84 CLINICAL HISTORY 41-year-old female with occasional headache and visual
problems.
177

178 Case 84
FINDINGS Figure 84-1. Coronal post-contrast T1WI not hormonally active; however, growth hormone and thyro-
through the sella turcica. There is a 2.5-cm enhancing sella/ tropin-secreting pituitary tumors are often very large.
suprasellar mass (arrows) without cavernous sinus exten- Presenting symptoms may be related to mass effect on the
sion. The mass compresses the optic chiasm that cannot be optic chiasm, cavernous sinuses, and hydrocephalus. Many
identified. Figure 84-2. Sagittal post-contrast T1WI. There is macroadenomas, however, are discovered incidentally.
expanded sella turcica. The mass is compressing the anterior Treatment is variable and includes surgical resection, medi-
aspect of the third ventricle (arrow). There is no hydrocephalus. cal therapy, and radiation.
Figure 84-3. Axial post-contrast T1WI through the sella in a
companion case. There is a large sella-enhancing mass extend- Questions for Further Thought
ing into the right cavernous sinus, encasing the internal carotid 1. Can macroadenomas metastasize?
artery (arrow) and compressing the right temporal lobe. There 2. What defines cavernous sinus invasion by the tumor?
is posterior extension compressing the pons (vertical arrow).
Figure 84-4. Corresponding ADC map shows that the mass
(star) has low ADC (restricted diffusion) due to high cellularity.
Routine report is sufficient unless it is large enough to invade
and compress surrounding structures. Describe the mass,
DIFFERENTIAL DIAGNOSISPituitary macroadenoma,
size, location, and extent. Describe the mass effect on adja-
Rathke cleft cyst, meningioma, craniopharyngioma, aneu-
cent structures, including the optic chiasm and optic nerves.
rysm, metastasis.
Also, describe whether there is cavernous sinus invasion and
the patency of the carotid arteries.
DIAGNOSIS Pituitary macroadenoma.
DISCUSSION Pituitary macroadenomas are WHO I tumors
that are by definition greater than 10 mm in size, to distin- Presence of extension into adjacent structures such as the
guish them from microadenomas that are less than 10 mm in cavernous sinus and clivus
size. These tumors often have a characteristic snow man Size and extent of the mass
appearance, with a waist at the level of the diaphragma sella
that tends to restrict tumor growth at this level. The masses Answers
are often heterogeneous on MRI and enhance after contrast 1. Metastasis is extremely rare but has been reported par-
administration due to their rich blood supply. On T2WI, they ticularly in malignant adenomas. Conversely, other
tend to be isointense with brain and may contain multiple tumors may metastasize to the gland. These patients
small cystic (hyperintense) regions. On DWI, they are hyper- often have acute onset of symptoms including panhypo-
intense reflecting their high cellularity. Compression ofthe pituitarism.
optic chiasm and invasion of the cavernous sinuses arenot 2. Tumor encasing more than two-thirds of the cavernous
uncommon and extension into the sphenoid sinus may be segment of the carotid artery likely has cavernous sinus
present in large lesions. Many of these tumors are generally invasion.

Case
85 CLINICAL HISTORY 70-year-old male with smoldering multiple myeloma
developed mental status changes. Rule out meningitis.
179

180 Case 85
FINDINGS Figure 85-1. Axial FLAIR through the vertex. lumbar puncture (LP) to exclude raised intracranial pressure
There is diffuse sulcal hyperintensity over bilateral cerebral (RIP) and mimics of meningitis. Unfortunately, while RIP
hemispheres (arrows point to some of the hyperintense sulci). is considered a contraindication to LP, normal CT scan does
The sulci appear prominent on T2WI (not shown). Figure 85-2. not completely exclude RIP in patients with BM. Imaging is
Axial post-contrast T1WI through same levels as Figure 85-1. not needed in most patients with meningitis as it is often nor-
There is bilateral sulcal effacement with very little or no lep- mal. It may, however, be necessary in patients not respond-
tomeningeal enhancement (arrows). Figure85-3. Axial DWI ing to treatment as expected or in those suspected to have
through the trigone 2 weeks following antimicrobial treatment. complications. The causative organisms reach the central
There is a tiny right trigonal restricted diffusion (arrow) com- nervous system (CNS) by inhalation, via the bloodstream,
patible with ventricular debris and ventriculitis. Figure 85-4. by direct extension from surrounding paranasal sinuses and
Axial FLAIR at the same time as Figure 85-3. There is residual mastoid infections or by direct inoculation during inter-
right frontal superior sulcus hyperintensity (arrow) with reso- vention. The common organisms in the immunocompetent
lution of sulcal hyperintensity and effacement elsewhere. are Streptococcus pneumoniae and Neisseria meningitidis
accounting for about 80% of cases, while S. pneumoniae,
DIFFERENTIAL DIAGNOSISMeningitis, subarachnoid Listeria monocytogenes, and gram-negative bacilli are com-
hemorrhage (SAH),ventriculitis. mon in the immunosuppressed. Group B streptococci and
coliform bacilli are responsible for most cases in neonates.
DIAGNOSIS Bacterial meningitis (BM). The CSF is this patient was described as very cloudy with
evidence of acute inflammation. S. pneumoniae was isolated
DISCUSSION MRI is the most sensitive imaging modal- from the blood, and he responded to a combination of antimi-
ity for evaluation of BM. Contrast-enhanced MRI is nor- crobials. The follow-up MRI showed resolving sulcal hyper-
mal in about 50%. It should therefore be emphasized that intensity and evidence of complicating ventriculitis.
normal imaging does not exclude BM. In those with posi-
tive imaging, findings include sulcal FLAIR hyperinten- Question for Further Thought
sity as in this patient, generally believed to be due to the 1. In what ways could DWI be useful in evaluation of men-
high protein content of the cerebrospinal fluid (CSF) in ingitis?
BM, leptomeningeal enhancement due to inflammation,
subcortical edema, and brain swelling. Complications Reporting Responsibilities
such as hydrocephalus, vasculopathy, cerebritis, abscess, Findings compatible with meningitis or its complications
ischemic infarcts, venous sinus thrombosis, ventriculi- should be directly reported.
tis, and subdural collections either empyema or hygroma
could easily be detected by imaging. FLAIR sulcal hyper-
intensity is not unique to BM and may be seen in SAH,
supplemental oxygen administration, propofol administra- Is there RIP? Is it safe to perform LP?
tion, and leptomeningeal metastases. The history may be Are there complications? If so what are they?
useful in excluding these situations. The pattern of lepto- On follow-up studies, is there response to treatment or are
meningeal enhancement in BM may not be distinguishable there new findings or progression of complications?
from some normal physiologic situations. CT is capable
of showing the complications but not as effective as MRI. Answer
Neuroimaging can identify conditions that may predis- 1. The most common complication of meningitis is hydro-
pose to BM such as skull base fractures, paranasal sinus cephalus. This could easily be established by CT or rou-
or mastoid infections, and congenital anomalies and moni- tine MRI. However abscess, subdural empyema, and
tor response of the complications to treatment. MRA and ventriculitis require DWI for characterization. These
MRV are useful in evaluation of the vascular complica- three complications usually show restricted diffusion
tions such as vasculopathy, infarctions, and venous sinus (hyperintense on DWI and hypointense on ADC map) due
thrombosis. to cellular debris, thus making it possible to distinguish
BM is a severe and often lethal neurologic illness with subdural empyema from subdural hygroma, abscess from
about 30% to 50% of survivors having permanent neuro- tumors, and ventriculitis from hemorrhage. Hemorrhage
logic disability. BM can occur in all ages and both genders. may sometimes produce heterogeneous restricted diffu-
The diagnosis of BM is based on clinical and CSF findings. sion. DWI can also be used to monitor response of these
However, imaging particularly CT may be requested before complications to treatment.

Case
86 CLINICAL HISTORY 75-year-old male with right vision loss.
Figure 86-4
FINDINGS Figure 86-1. Axial NCCT through the occipi-

tal lobes. There is a large left occipital hypodensity (arrows)
abutting the tentorium medially. Figure 86-2. Axial DWI
through the occipital lobes. There is a large hyperintensity
(low ADC not shown) consistent with area of restricted dif-
Figure 86-3 fusion in the left occipital lobe. Figure 86-3. Axial T2WI
181

182 Case 86
through the occipital lobes. There is a large left occipital lobe limited neurologic deficit not qualifying for thrombolytic ther-
hyperintensity extending into the posteromedial left tempo- apy. However, clinical presentations may include vision loss
ral lobe (arrows). Figure 86-4. 3D TOF MRA. There is a loss usually hemianopsia, limb weakness, and sensory deficits.
of signal in the left posterior cerebral artery (PCA) at the P2
P3 junction (vertical arrow). Distally the left PCA is severely Question for Further Thought
attenuated (transverse arrow). 1. Why does the vessel remain open in some cases of
infarction?
DIFFERENTIAL DIAGNOSIS N/A.
DIAGNOSIS Left PCA territory acute infarct.
This is an acute situation demanding direct reporting to the
referring physician. As in all acute or subacute infarcts, pres-
DISCUSSION Ischemic infarct is hypodense on NCCT.
ence of hemorrhage, brain swelling, or herniations should be
This may not be very visible within the first 6 hours particu-
reported. Accompanying vascular changes on MRA should
larly if it is small. The acute and subacute phase results in
also be enumerated as this may affect management choices.
mass effect which depends on the size of the infarct. Chronic
infarct usually results in volume loss. The corresponding
hyperintensity on the DWI is consistent with acute/subacute
infarct. The location of the PCA infarct depends on the loca- Size and location of infarct
tion of the occlusion. Proximal occlusion could involve all Complications such as hemorrhagic conversion or
PCA territory which includes the occipital lobe, posterome- herniations
dial temporal lobe, the splenium of the corpus callosum, the Vascular changes
midbrain, and thalamus. The MRA shows a P2P3 junction
severe stenosis; hence, the occipital and the mesiotemporal Answer
lobes are affected. 1. An embolus may block an artery and results in infarc-
Infarctions of the PCA territory are not very common tion. With elevated blood pressure or thrombolysis, the
accounting for 9.6% of cerebral infarctions in a particu- embolus breaks up and disperses. Infarcts could also be
lar series. Lacunar infarction was the most frequent stroke caused by a low flow state without arterial occlusion as
subtype (34.5%) followed by atherothrombotic infarction in watershed infarcts. Intramural hemorrhage in dissec-
(29.3%) and cardioembolic infarction (21.6%). PCA infarction could retract resulting in recanalization of occluded
tion occurs less frequently and more often is associated with artery.

Case
87 CLINICAL HISTORY 62-year-old female with subarachnoid hemorrhage and
left frontal parenchymal hematoma.
DISCUSSION The reason for showing this case is to dem-

onstrate the persistent trigeminal artery (PTA). The PTA can
be seen on MRI, CT, MRA and CTA, and DSA as an arterial
connection between the cavernous ICA and the mid-basilar
artery. There could be some variation in the location of the
PTA arising from the precavernous ICA to as high as the
proximal dural ring of the ICA. It could give hypophyseal
and meningeal branches. Usually the basilar artery proximal
to the PTA is hypoplastic. The corresponding vertebral arter-
ies are also small in size. In its course, the PTA could be in
close proximity to the third, fourth, fifth, and sixth cranial
nerves that can be compressed by the vessel. PTA is known
to wander into the sella turcica perforating the dorsum sella
before anastomosing with the basilar artery. This is known
as the medial type PTA which could have surgical impli-
Figure 87-3 cations during transphenoidal hypophysectomy. The other
type is the lateral type that runs laterally to the sella turcica.
The presence of posterior communicating arteries is vari-
FINDINGS Figure 87-1. Oblique 3D volume-rendering able. There is associated intracranial aneurysm in between
CTA of the left ICA. There is a large vessel (vertical arrow) 3% and 16% which is not very different from the general
joining the left ICA to the mid-basilar artery. The surface population. The other clinical implications include impedi-
nodularity is due to calcifications. The proximal basilar artery ment to endovascular treatment of basilar tip aneurysm and
is not visualized (congenitally hypoplastic) while the distal posterior fossa vascular malformation since the vertebrobas-
basilar artery is robust (transverse arrow). Figure87-2. Axial ilar system proximal to the anastomosis is generally hypo-
oblique MIP of the same vessel (arrow). There are multiple plastic. PTA variants represent precavernous ICA branches
calcific foci on it. Figure 87-3. 3D volume rendering of the that do not connect to the basilar artery.
anterior circulation in the same patient. There is an anterior PTA is a rare remnant of the embryonic circulatory sys-
communicating artery aneurysm (arrow). tem that anastomoses the cavernous ICA with the middle
or distal portion of the basilar artery. It represents the most
DIFFERENTIAL DIAGNOSIS N/A. common of the persistent primitive collaterals between the
anterior and posterior circulation. The others are the persis-
DIAGNOSIS Persistent trigeminal artery (PTA). tent otic, hypoglossal, and proatlantal arteries.
183

184 Case 87
Question for Further Thought Answer

1. What is the origin of PTA? 1. PTA is a fetal arterial connection between the ICA and
the developing basilar artery. It appears at about the
Reporting Responsibilities 28 to 29 days of embryonic development along with
Routine reporting is sufficient unless there is associated the hypoglossal and otic arteries mainly to supply the
reason for a direct reporting such as the aneurysm in this developing hindbrain before the proper formation of the
instance. The hypoplastic proximal basilar artery is worth basilar artery. These vessels hang around for about 4 to
commenting upon not to be mistaken for severe atheroscle- 8 days before disappearing as the basilar artery forms.
rotic disease. Persistence of these vessels particularly the PTA inhibits
the proper development of the basilar artery, hence there
What the Treating Physician Needs to Know is hypoplasia of the basilar artery in about 75% of PTA.
Other associated abnormalities
This is a congenital anastomosis between the anterior and
posterior circulation and does not require any treatment or
follow-up imaging

Case 88 CLINICAL HISTORY 11-year-old female diagnosed with neurofibromatosis type 2
(NF2) has a growing intraventricular mass.
Figure 88-4
Figure 88-3
FINDINGS Figure 88-1. Axial FLAIR through the h yperintensity extending into the white matter (WM) con-
trigones. There is a homogeneous isointense (to gray mat- sistent with parenchymal vasogenic edema (arrow). Figure
ter [GM]) mass within the right trigone measuring 3.8 cm 88-2. Coronal T2WI through the mass. Mass (star) is mildly
2.6 cm at the widest point (star). There is periventricular heterogeneous but isointense to GM. Peripherally superi-
185

186 Case 88
orly and medially the mass is as hyperintense as the adja- Menigiomas are designated WHO I tumors with very few
cent periventricular hyperintense vasogenic edema (arrow). histologic subtypes atypical WHO II or anaplastic WHOIII.
Figure88-3. Post-contrast T1WI. There is homogeneous NF2-associated meningiomas have a higher mitotic index
contrast enhancement of the smooth marginated mass (star). and a more aggressive clinical behavior than sporadic menin-
Figure 88-4. DTI color directional map. There is mass effect giomas. All histologic subtypes of meningiomas have been
on the right inferior fronto-occipital fasciculus and inferior reported within the ventricles. They are more common in
longitudinal fasciculus (transverse arrows) which have been adults than in children except in NF2. There is no gender
compressed laterally. There is also posterior displacement of preference unlike hemispheric menigiomas that are more
the fibers in the right splenium with disorganization of the common in females. Clinical presentation is usually not
right forceps major (vertical arrow). specific and may include headache, mental status changes,
focal neurologic deficit, vertigo, and gait disturbance.
DIFFERENTIAL DIAGNOSIS Choroid plexus carcinoma Intraventricular menigiomas tend to grow very large before
(CPC), choroid plexus papilloma (CPP), intraventricular presenting because of the vague symptoms unless it is situ-
meningioma, ependymoma. ated where it could obstruct the CSF pathways. Total exci-
sion by piecemeal reduction in tumor volume with ultrasonic
DIAGNOSIS Intraventricular meningioma. aspiration has been advocated as providing a safe surgical
treatment and providing cure.
DISCUSSION Like most meningiomas, intraventricu-
lar meningioma has unique imaging features on MRI. It is Question for Further Thought
mostly isointense to GM on T1WI and isointense to hyper- 1. Apart from meningioma, what other intracranial tumors
intense on T2WI, usually smooth marginated and avidly occur in NF2?
contrast enhancing. Calcifications impart some heterogeneity
to the signal pattern with multifocal hypointensities on T2WI.
Meningiomas generally exhibit local mass effect dilating the
Direct reporting is desirable. CSF pathway obstruction
ventricle and compressing adjacent structures but rarely pro-
makes direct reporting more urgent. Early treatment of intra-
ducing perilesional edema as in this case. DTI may show
ventricular meningioma is very important to achieve favor-
attenuation of surrounding fiber tracts and reduced Fractional
able outcome. Location, size, and number if more than one
Anisotropy (FA). The trigone is the most common location of
should be reported. Presence of periventricular edema which
intraventricular meningioma. About 77.8% of intraventricu-
might suggest an aggressive tumor or confuse location of
lar meningiomas occur in the lateral ventricles closely fol-
tumor should be mentioned.
lowed by the third ventricle in 15.6% and the fourth ventricle
in 6.6%. On NCCT meningiomas are isodense to slightly
hyperdense with avid contrast enhancement following con-
trast administration. Angiogram usually shows increased Location and size of tumor
vascularity and tumor blush with most of the supply com- Effect on surrounding structures
ing from the posterior choroidal artery and some from the Presence of hydrocephalus
anterior choroidal artery. CPP is usually lobulated, more het- Differential diagnosis
erogeneous on T2WI with flow voids, and more frequently
associated with hydrocephalus even when it is not obstructing Answer
the CSF pathways. CPC in more poorly marginated with a 1. Bilateral vestibular schwannomas are the hallmark of
tendency to infiltrate ventricular walls and cause perilesional NF2. Other tumors include schwannomas of the trigemi-
edema. Ependymomas are generally more heterogeneous in nal nerves, and gliomas which include ependymomas and
signal characteristics and avidly contrast enhancing. astrocytomas (mostly pilocytic). Other nontumoral intra-
Intraventricular meningioma is rare accounting for cranial lesions may include menigioangiomatosis and
between 0.5% and 3% of all intracranial meningiomas. cerebral calcifications.

Case
89 CLINICAL HISTORY 27-year-old male with neurofibromatosis and increasing
symptom; suspect subarachnoid hemorrhage.
FINDINGS Figure 89-1. Sagittal non-contrast T1WI. There superior cerebellar arteries (vertical arrows). The draining
is a coil of signal voids (vertical arrow) within the fourth veins extend into the great vein of Galen (transverse arrow)
ventricle with tubular signal voids extending into the cisterna and along the straight sinus into the region of torcula (chev-
vena magna and along the straight sinus (transverse arrows). ron). Figure 89-4. Lateral DSA of a vertebral artery injec-
Figure 89-2. Axial MIP CTA through the posterior fossa. tion. The arterial feeders (vertical arrows) to the nidus (star)
There is a collection of large vessels reminiscent of a bag are hypertrophied superior cerebellar arteries with drainage
of worms, the nidus, measuring about 5 cm in longest into the vein of Galen/straight sinus and some veins heading
dimension in the midline posterior fossa at the expected loca- into the torcula (transverse arrows).
tion of the fourth ventricle (midline vertical arrow). There
is a large draining vein extending to the torcula on the right DIFFERENTIAL DIAGNOSISArteriovenous malformation
(chevron) with multiple smaller veins along the tentorium (AVM), dural arteriovenous fistula (DAVF) vascular tumor.
(transverse arrows). Figure 89-3. Sagittal MIP CTA. The
arterial feeders are from the basilar arteryhypertrophied DIAGNOSIS AVM of fourth ventricle.
187

188 Case 89
DISCUSSION CT and MRI are complementary techniques Question for Further Thought
for the evaluation of AVM. AVM is an abnormal connection 1. Is there a relationship between neurofibromatosis type 1
between the artery and vein across a tangle of intermediary (NF1) and AVM?
vessels known as the nidus. An unruptured AVM is usually
mildly hyperdense on CT. It may have defined or feathery
borders with calcifications and surrounding parenchymal vol-
Direct reporting is essential particularly if it is unexpected.
ume loss or mass effect as the case may be. There is always
Significant interval changes on follow-up should also be
contrast enhancement that may demonstrate the arterial feed-
directly reported. AVMs can grow. The afferent and effer-
ers, the nidus, and venous drainage. Hemorrhage, hyper-
ent vessels should be carefully evaluated and recorded, and
dense mass lesion with mass effect, is usually present in the
the location of the nidus should be described. Presence of
ruptured AVM. CTA is usually preferred over MRA for the
aneurysms on the feeders, within the nidus, and on the effer-
demonstration of the vascular components of the AVM. MRI
ent veins should be carefully sought. Stenosis of the feeders
is best for evaluation of the complex. Parenchymal changes
or the draining veins could have implications on treatment
may include mass effect due to the nidus, hematoma, and
and natural history of the disease. AVM may spontaneously
edema; volume loss due to arterial steal phenomenon and
thrombose in a small percentage of patients.
gliosis. The arterial feeders and the draining veins could be
single or multiple and are usually dilated, ectatic signal voids
that may have aneurysms or stenosis. The nidus is a coil of What the Treating Physician Needs to Know
tubular signal voids usually within the parenchymal or epen- Size and location of the nidus
dymal as in this case; leptomeningeal location is not unknown Feeders and draining veins; number, morphology, sources
and is best demonstrated by T2WI. DSA is the definitive test of arterial feeders, and destinations of the veins
for comprehensive evaluation of the vascular components as Whether high or low flow; depends on size and how
each of the feeders could be separately injected to demon- quickly the veins fill on DSA
strate their individual contributions to the nidus and may also Presence of surrounding hemorrhage, volume loss, gliosis,
offer therapeutic options. DAVF usually has no nidus; this hydrocephalus, etc.
differentiates it from an AVM. Hypervascular tumors tend to
be more mass-like with salt and pepper pattern rather than
large signal voids. Thrombosed AVM however may resem- Answer
ble a neoplasm in the absence of the vascular components. 1. There is an underlying vasculopathy in patients with NF1
AVMs are considered congenital but de novo occurrence that has been termed NF1 vasculopathy, and it results in
is increasingly reported. They are a rare cause of neurologic a wide spectrum of vascular lesions. The prevalence of
deficits particularly if they rupture. The cause of AVM is vascular lesions in large clinical series is 0.4% to 6.4%.
unknown. It is mostly supratentorial in location and rare in These are mostly arterial stenoses and aneurysms in extra-
the posterior fossa. It has a prevalence of between 15 and cranial locations particularly in the aorta, renal, and mes-
18 per 100,000 but postmortem series report higher figures. enteric arteries with renal artery stenosis being the most
Most AVMs are discovered in association with primary common. The basis for the vasculopathy is uncertain but
intracranial hemorrhagesparenchymal, subarachnoid, or probably due to deficiency in neurofibromin in NF1 caus-
intraventricular with hemorrhages occurring in as many as ing proliferation of neural elements within the vessel wall
72% of hospital-based AVM series. Factors associated with or intimal compression by neural tumors. More often,
hemorrhagic presentation include deep venous drainage, sin- the histologic feature is fibromuscular dysplasia with a
gle draining vein, venous stenosis, high feeding arterial mean predominance of intimal thickening. These lesions occur
pressure, small nidus size, intranidal aneurysm, and venous more commonly in the young. Carotid, vertebral, or cere-
reflux. These are signs that are easily visible at imaging if bral artery lesions were seen in 19% of a particular series
we look for them. Other clinical presentations include head- of NF1 patients. These are commonly aneurysms, occur-
aches, seizures, and neurologic deficit. The crude annual risk ring in the third decade of life, and more often in women
for the first occurrence of a hemorrhage from an unruptured than men. Head and neck vascular lesions include cervi-
AVM is about 2% with a long-term crude annual case fatal- cal vertebral AVM presumably due to contained rupture
ity rate between 1% and 1.5%. Treatment options for AVM of vertebral artery aneurysms, spontaneous subclavian
include surgery, endovascular embolization, and radiosur- artery rupture, AVM of the face, and intracranial aneu-
gery, usually in combination. rysms and AVMs.

Case
90 CLINICAL HISTORY 66-year-old female post cardiac arrest. Pupils fixed and
dilated. Rule out anoxic brain injury.
189

190 Case 90
FINDINGS Figure 90-1. Axial NCCT through the tento- later findings are usually associated with poor prognosis.
rial incisura. There is bilateral cerebral hemispheric patchy Subsequently, in the chronic stage there is profound volume
hypodensities (arrows) with crowding of the tentorial inci- loss that correlates well with persistent vegetative state. MRI
sura and effacement of the suprasellar cistern and poste- is more sensitive for early detection of changes of ASHIE
rior compression of the upper pons by the superior vermis. and DWI could show lesions within a few hours following
Figure90-2. Axial NCCT through the inferior basal ganglia. the injury.
There are patchy bilateral basal ganglia cortical/subcortical HIE affects all ages. Risk factors tend to vary with age.
frontal lobes hypodensities (arrows) Figure 90-3. Axial In the adult population, the common risk factors are cardio-
NCCT through the superior basal ganglia. There are bilateral pulmonary arrest, drowning, hypoperfusion states, stran-
symmetrical caudate nuclei and lentiform nuclei hypoden- gulation, hanging, carbon monoxide poisoning, and drug
sities (arrows). Multifocal patchy bilateral subcortical overdose. Clinical presentations depend on the duration
hypodensities are also present (vertical arrows). Figure 90-4. and severity of the injury and could include confusion, sei-
Axial NCCT through the centrum semiovale. There are mul- zures, stroke-like changes, mental status changes, and coma.
tifocal cortical/subcortical almost symmetrical hypodensities Management is mostly supportive, and the prognosis is poor.
in bilateral cerebral hemispheres (arrows). There is global
poor whitegray matter (WMGM) differentiation with Question for Further Thought
effacement of convexity sulci. 1. Why is the GM more susceptible to ischemia and hypoxia
than the WM?
DIAGNOSIS Acute subacute hypoxic ischemic encepha-
This is an emergency requiring direct reporting. The entire
lopathy (ASHIE). (CT changes)
pattern of involvement should be enumerated.
DISCUSSION ASHIE findings in the adult depend on the
severity of the injury. The immediate CT could be normal; What the Treating Physician Needs to Know
this does not exclude hypoxic ischemic encephalopathy Full description of the pattern of HIE
(HIE). Classical changes include bilateral symmetrical basal Complications such as hemorrhage, herniations, or hydro-
ganglia, thalamic and hippocampal hypodensities, multifo- cephalus
cal cortical/subcortical hypodensities, diffuse poor WMGM A normal CT does not exclude HIE. It may take up to
differentiation, sulcal effacement, and in the most severe 3days for the changes to manifest. MRI shows changes
situation, central herniation. Lesions could be distributed in earlier than CT, and MRI should be obtained if there is
watershed zones. Other less common findings could include equivocal CT changes
WMGM reversal sign with WM hyperdensity, white cer-
ebellar sign where the cerebellum appears white in contrast Answer
to the hypodense cerebral hemispheres, subarachnoid hem- 1. The brunt of HIE is borne by the GM because it is meta-
orrhage presenting mostly as cortical sulcal hyperdensi- bolically more active than the WM. It contains a large
ties, pseudosubarachnoid hemorrhage (hyperdense vessels) number of synapses which exhibit glutamate excitotoxic-
within the basal cisterns and the tentorium due to stagna- ity as a consequence of hypoxia and ischemia. These GM
tion of flow within the vessels, hydrocephalus, and dif- areas include the basal ganglia, thalami, hippocampus,
fuse brain hypodensity due to brain swelling/edema. These cerebral cortex, and the cerebellum.

Case
91 CLINICAL HISTORY 48-year-old female with metastatic infiltrating ductal
breast carcinoma to the T12 vertebral body, staging examination.
FINDINGS Figure 91-1. Axial post-contrast T1WI sagittal reformatted post-contrast 3D T1WI through the
through the temporal lobes. There is a homogenously frontal lobes, respectively. There is a concurrent dural-
enhancing extraaxial mass within the right middle cra- based enhancing mass with dural tails at the left frontal
nial fossa (arrow). Figure 91-2. Cerebral blood volume convexity with contiguous osseous and extracranial com-
(CBV) map from dynamic susceptibility contrast MR ponents (arrows).
perfusion. There is markedly elevated relative Cerebral
Blood Volume (rCBV) within the right middle cranial DIFFERENTIAL DIAGNOSISMeningioma, lymphoma,
fossa mass (arrow). Figures 91-3 and 91-4. Coronal and hemangiopericytoma, metastases.
191

192 Case 91
DIAGNOSIS Dural metastatic breast cancer. aggressive neoplasms such as lymphoma and hemangio-
pericytoma could have arisen within the short time frame
DISCUSSION Dural metastasis shares the same features of less than 4 months. However, the concurrent left frontal
with most dural-based extraaxial aggressive lesions; extradural-based lesion with contiguous osseous and extracranial
axial compression of the brain, avid contrast enhancement, components overwhelmingly confirms that the right middle
dural tail, and overlying bone destruction and in this case cranial fossa lesion is a metastasis rather than a meningioma,
transcalvarial subgaleal extension of the tumor in the fron- in this patient with known metastatic breast cancer.
tal component. Hemorrhagic masses most likely will present
with GRE blooming. There may or may not be infiltration Questions for Further Thought
of the underlying brain. Meningioma is the most com- 1. What are the four most common dural-based metastatic
mon extraaxial homogenously enhancing mass in an adult lesions in adults?
patient and statistically would be the likely diagnosis for a 2. What is the single most common dural-based metastatic
right middle cranial fossa extraaxial mass in this patient age lesion in children?
group. The key to this case is the patients history of meta-
static breast cancer and previous brain imaging (4 months Reporting Responsibilities
prior) which did not depict a right middle cranial fossa mass. Direct reporting is essential in every metastatic disease.
Meningiomas are slowly growing extraaxial lesions, unless Presence of an additional enhancing dural-based lesion
they degenerate into a higher grade, which typically occurs with osseous and extracranial extension confirms metastatic
with a preexisting meningioma. MR perfusion imaging has disease and effectively excludes meningioma from the dif-
been suggested to help differentiate between dural-based ferential diagnosis.
metastasis and meningioma, whereby meningioma showed
significantly more elevated rCBV compared to dural-based
metastases. Interestingly, our case demonstrates markedly
elevated rCBV. Therefore, MR perfusion was not helpful in Location, number, and size
excluding the possibility of a meningioma in our case. Comparison to prior imaging
Additional imaging with CT would likely have demon-
strated permeative change in the right greater sphenoid wing Answers
with dural-based metastatic disease, rather than the hyper- 1. Breast, prostate, lung, and melanoma.
ostosis typically seen with meningioma. Certainly more 2. Neuroblastoma.

Case
92 CLINICAL HISTORY Young patient presenting with a chronic and very slowly
progressive left cranial nerve III palsy.
DISCUSSION Parasellar schwannomas are well-

circumscribed fusiform or rounded lesions on CT and MRI
that follow the expected course of a cranial nerve or its
branches, enlarge the skull base foramina, and show an avid
enhancement after contrast. Schwannomas may show a cen-
tral area of lesser contrast enhancement (the target sign).
Occasionally, they may contain fluid levels. On CT, they
appear as isodense to hypodense lesions, while on MRI T1WI
they are isointense to hypointense and usually hyperintense
on T2WI. Depending on cystic-necrotic and intratumoral
hemorrhagic component, schwannomas may appear hetero-
geneous with variable contrast enhancement patterns. They
generally lack dural tails since they are not dural based and
that distinguishes them from meningiomas. Lymphoma and
inflammatory pseudotumor are not as well circumscribed as
schwannomas. Perineural tumor spread tends to involve a
Figure 92-3 long segment of the nerve.
Schwannomas are benign WHO I neoplasms of nerve
sheaths that are composed entirely of well-differentiated
FINDINGS Figure 92-1. Coronal post-contrast T1WI shows Schwann cells. In the parasellar region they may arise from
an enhancing small mass (arrow) in the expected course of the nerve plexus located on the medial wall of the pituitary
the left oculomotor nerve in the superolateral left cavernous fossa, perivascular nerve cells, autonomic vasomotor nerve
sinus. Figure 92-2. Axial post-contrast T1WI shows the ante- plexus, or any of the cranial nerves passing through the cav-
rior location of the mass (arrow) within the left cavernous ernous sinus, including the third, fourth as in the companion
sinus. Figure 92-3. Coronal T2WI through the cavernous case, maxillary, and ophthalmic branches of the trigeminal
sinuses in a companion case. There is a well-defined round nerve, and the sixth cranial nerves Parasellar schwannoma
hypointense mass (arrow) in the mid-left cavernous sinus. tends to arise from cranial nerves III and V, this last one
being the second most common site of intracranial schwan-
DIFFERENTIAL DIAGNOSISMeningioma, metastasis noma and representing about one-third of primary trigeminal
(including perineural spread), lymphoma, schwannoma, and Meckel cave tumors.
inflammatory pseudotumor. Depending on the cranial nerves involved, symptoms
could include facial numbness, pain, paresthesia, as well as a
DIAGNOSIS Cavernous sinus schwannoma (oculomotor decreased visual acuity or visual field defects that generally
nerve). relate to the tumors suprasellar extension. Interestingly,
193

194 Case 92
about one-half of the patients exhibit hypopituitarism with structures. Describe the cranial nerve from where the para-
a modest hyperprolactinemia, possibly explained by the sellar schwannoma arises and the extent of the tumor and
compression of the pituitary stalk. Treatment depends on any compression it produces. Make sure the lesion is not an
the clinical symptoms and consists of surgery and different aneurysm. If in doubt, recommend CTA or MRA.
forms of radiotherapy. Small asymptomatic schwannomas
may require only follow-up to assess growth. What the Treating Physician Needs to Know
Probable origin and extent of the schwannoma
Relation with surrounding sella and parasellar structures
1. What is the most common cranial nerve affected by so that proper treatment can be administered
schwannoma?

Routine reporting is sufficient for this indolent tumor unless 1. The most common intracranial schwannoma originates in
it has grown significantly large and compresses critical the vestibular division of cranial nerve VIII.

Case
93 CLINICAL HISTORY 71-year-old female with a history of recent postural
hypotension, dry mouth, resting tremor, and urinary retention.
FINDINGS Figures 93-1 and 93-2. Axial T2WI and FLAIR, parietal lobe volume loss precede the others. 11C-Raclopride
respectively, through the pons. There is hot cross bun sign at PET shows decreased postsynaptic D2 receptor density in
the pons. It is correlated with selective loss of myelinated putamen. F-DOPA PET shows decreased putaminal F-DOPA
transverse pontocerebellar fibers and neurons in the pontine influx constants (lower activity in posterior putamen).
raphe. There is widening of the posterior fossa CSF spaces MSA like its Parkinson-plus counterparts (progressive
consistent with volume loss. supranuclear palsy and corticobasal degeneration) is a pro-
gressive, sporadic, adult-onset neurodegenerative disease.
DIFFERENTIAL DIAGNOSIS Parkinson-plus syndromes Neuropathologically the characteristic finding is diffuse
(progressive supranuclear palsy, corticobasal degeneration), central nervous system (CNS) alpha-synuclein-positive glial
spinocerebellar ataxia, multiple system atrophy (MSA) cytoplasmic inclusions. The trigger is unknown. The affected
hereditary olivopontocerebellar atrophy. population is usually over 50 years of age. Multiple system
refers to various combinations of extrapyramidal, pyramidal,
DIAGNOSIS Multiple system atrophy (MSA)cerebellar cerebellar, and autonomic involvement; particular involve-
type. ment of one of the systems may be predominant.
Orthostatic hypotension is often the earliest and most
DISCUSSION Two imaging subtypes are described: debilitating symptom. Autonomic dysfunction ultimately in
MSA-C (cerebellar type) with preceding olivopontocere 97% of the patients manifests with genitourinary dysfunc-
bellar degeneration (inferior olivary nucleus, pons, and tion being the most frequent in women, and early erectile
cerebellum) and MSA-P (parkinsonian type) with preced- dysfunction in men. MSA progresses rapidly, and motor
ing striatonigral degeneration (substantia nigra, putamen, impairment by cerebellar or corticospinal tract dysfunction
caudate nucleus, and globus pallidus). MSA-C findings are develops within 1 year of onset. The devastating outcome is
described in the figures. MSA-P shows decreased signal in wheelchair bound in less than 5 years in 50% of the patients.
the dorsolateral putamen surrounded by thin rim of hyperin- The median survival is less than 10 years. There is no effec-
tensity on T2WI. Another finding is enlarged space between tive therapy other than palliative care.
putamen and external capsule giving a slit-like signal void
on T2WI. Pontine tegmentum and corticospinal tracts are Question for Further Thought
relatively preserved. Pontine volume loss, inferior olivary 1. What is the preferred modality of imaging?
atrophy, cerebellar and superior cerebellar peduncle atrophy
are the concomitant findings. Reporting Responsibilities
FDG-PET shows hypometabolism in the effected puta- Routine reporting is sufficient. Hot cross bun sign is typical
men, cerebellum, and even the motor cortex in the advanced of MSA, but sometimes there may be vague findings such as
stage. Eventually, in both types, motor cortex becomes atro- pontine and symmetric cerebellar volume loss without abnor-
phic. In terms of cerebral cortical involvement, frontal and mal T2-weighted signal or only posterior lateral putaminal
195

196 Case 93
T2-weighted hyperintensity. These findings should at least The hypertrophic olivary degeneration which may lead to
raise the possibility of Parkinson-plus syndromes. atrophy of inferior olivary nucleus and crossed cerebellum
is another entity that should be kept in mind. In asymmet-
What the Treating Physician Needs to Know ric involvement of inferior olivary nucleus and cerebel-
lum, the inciting event should be sought on brain MRI
MSA-C clinical presentation may be vague and indistinc-
tive from nonfamilial adult-onset cerebellar degeneration
secondary to Hashimoto thyroiditis, lithium intoxication, Answer
prolonged phenytoin/phenobarbital use, paraneoplastic 1. MRI is the modality of choice. Nuclear medicine is
syndromes, or nutritional adjunctive in cases of vague MRI and clinical findings.

Case
94 CLINICAL HISTORY Young female presenting with seizures.

Courtesy of Annette Douglas, MD. Courtesy of Annette Douglas, MD.
Figure 94-3
Figure 94-4
Courtesy of Annette Douglas, MD.
Courtesy of Annette Douglas, MD.
197

198 Case 94
FINDINGS Figure 94-1. Axial NCCT through centrum parts of the world such as in Latin America. Other present-
semiovale. There is a small right frontal lateral cortical cyst ing symptoms could include headache, neurologic deficit,
with an eccentric tiny calcification (transverse arrow). There and mental status changes. Diagnosis is usually confirmed
is a subtle hypodensity on its medial aspect. A small cortical by ELISA or electroimmunotransfer blot assay (EITB) with
calcification is present in the left parasagittal parietal lobe a specificity of about 100%. NCC is treated with antiparasitic
(vertical arrow). Figure 94-2. Axial FLAIR MRI through the medication albendazole with praziquantel as an alternative
same level. There is a tiny hypointense ring with a central along with antiseizure and anti-inflammatory therapy. Intense
hyperintensity surrounded by a well-defined lobulated hyper- inflammation is usually associated with dying parasite.
intensity (arrow) consistent with vasogenic edema. There is
mild effacement of local sulci. Figures 94-3 and 94-4. Axial Question for Further Thought
pre- and post-contrast T1WI through same level. There is a 1. Where can you find NCC in the CNS?
smooth ring contrast enhancement within the hypointense
lesion (arrow).
Colloidal stage of cysticercosis is an emergent state that
DIFFERENTIAL DIAGNOSISPleomorphic xanthoastro-
should be reported directly to the referring physician.
cytoma (PXA), neurocysticercosis (NCC), metastasis, cere-
Location, number, presence of mass effect, and the amount
bral cavernous malformation (CCM).
of edema should all be noted. Presence of other stages of the
disease and complications should be reported.
DIAGNOSIS NCC vesicular/colloidal.
DISCUSSION This is a transition between the vesicular
and colloidal stage of NCC involution. The tiny calcifica- Location of lesions
tion is the scolex usually present in the nonreactive vesicu- Presence of other stages of the disease
lar stage. The surrounding edema and contrast enhancement Presence of complications such as hydrocephalus or
are consistent with host reaction associated with colloidal meningitis
degeneration, the most reactive stage of the four stages of
NCC and represents the so-called acute encephalitic phase. Answer
There is an intense reaction of the host to leakage of the cys- 1. NCC could be found in the parenchymal, subarachnoid,
ticercus antigen as it degenerates, resulting in breakdown of and intraventricular locations. Parenchymal lesions
the bloodbrain barrier with surrounding contrast enhance- undergo the four-stage degeneration. There is controversy
ment and edema. There could be a single cyst or multiple regarding location of so-called parenchymal cysts. A large
cysts. The entire brain could be riddled with similar lesions. autopsy series found that most of these cysts are situated in
MRI offers the best characterization of the lesions and their the crypts of the sulci or in perivascular spaces rather than
surrounding reactions. In this patient there is a second small in the brain parenchyma. The literature, however, main-
calcified left parietal focus consistent with the calcified final tains that parenchymal disease exists. Intraventricular and
stage of the involution. Perilesional edema is uncommon in subarachnoid NCC does not undergo the stages of degen-
CCM unless they have recently bled. Multiplicity of lesions eration. They remain cystic, can rupture, or obstruct the
is uncommon in PXA. Meningeal attachment and scalloping CSF pathway. Intraventricular cyst can be demonstrated
of inner table are common in PXA. Metastases rarely show to shift position within the ventricular system on MRI.
calcifications. Subarachnoid cysts could be the most difficult to diag-
Cysticercosis is the most common cause of epilepsy in the nose. If they are large enough, they could be confused
world. It is most prevalent in the poor countries of the world with arachnoid cyst. Large basal cistern and perisylvian
but increasingly diagnosed in the developed world as a result cysts also known as racemose NCC tend to have multiple
of globalization. The seizures are due to the intense inflamma- cysts that are associated with intense reaction and contrast
tion resulting in perilesional edema associated with the colloi- enhancement and can result in arachnoiditis with conse-
dal and granular nodular stages but seizures have been known quent communicating hydrocephalus. Spinal involvement
to occur also in the calcified nodular stage. The calcified is not uncommon but should be suspected in basal dis-
lesions are common in asymptomatic patients in the endemic ease.

Case
95 CLINICAL HISTORY 83-year-old female brought to the ER with mild altered
mental status.
FINDINGS Figure 95-1. Axial NCCT through the basal gan-

glia. There is a poorly defined hypodensity in the posterior limb
of the right internal capsule/anterior thalamus. Figure95-2.
Axial FLAIR MRI through the basal ganglia. There is an ovoid
hyperintense lesion (arrow) in the posterior limb of the right
internal capsule. Figure 95-3. Corresponding DWI. There is
focal restricted diffusion of the internal capsular lesion.
DIFFERENTIAL DIAGNOSISEnlarged Virchow-Robin

spaces, cerebral autosomal dominant arteriopathy with sub-
cortical infarcts and leukoencephalopathy (CADASIL),
lacunar infarct, cryptococcus, and neurocysticercosis.
DIAGNOSIS Lacunar infarct (LI) basal ganglia.
DISCUSSION CT is relatively insensitive in detecting

acute LI, and even when recognized it may be difficult to
distinguish from a chronic lesion. Only MR DWI has the
sensitivity to detect acute ischemia within the first few hours
of onset. On CT, LI is seen as a small and well-defined
hypodense area, while on MRI it appears as hypointense
T1WI and hyperintense T2WI focal lesion. Other findings
such as silent cerebral infarcts, leukoaraiosis, and micro-
bleeds may be present. LI is defined as small subcortical
Figure 95-3 infarct (2 to 20 mm in diameter) believed to be caused by
199

200 Case 95
the occlusion of a single perforating cerebral artery and is Reporting Responsibilities

usually found in the basal ganglia, thalamus, internal cap- Acute LI deserves direct reporting as an emergency. There
sule, corona radiata, cerebellum, and brainstem. is a need to exclude intracerebral hematoma or cortical
Risk factors for LI include increasing older age, male infarcts that can mimic any of the lacunar syndromes.
gender, hypertension, diabetes, smoking, previous transient Suggestion for further evaluation of the vessels should
ischemic attack (TIA), and probably ischemic heart disease. be made. Presence of accompanying other signs sugges-
Asymptomatic LI is much more frequent than symptomatic tive of small vessel disease (atherosclerosis) should be
ones, but its existence is associated with a higher risk of sub- mentioned.
sequent vascular events, cognitive decline, and dementia.
When symptomatic, LI could present as a pure motor stroke,
a pure sensory stroke, a sensorimotor stroke, ataxic hemipa-
resis, and dysarthria-clumsy hand syndrome. About one-third Location of imaging findings to correlate the clinical
of LI progress and worsen 24 to 48 hours after onset and have deficits
a poorer outcome. Early clinical predictors of progression Lacunar syndromes and LI progression are clinical diagno-
include female gender, age 75 years, hypertension, diabetes, sis as there may not be imaging correlates of these changes
hypertriglyceridemia, occlusive lesion in the parent artery, a Are there other entities mimicking LI?
pure motor stroke, severity of motor deficits on admission,
and lesions located in the corona radiata and pons. Answer
Management in the acute setting is based on correct- 1. CADASIL is a genetic small vessel disease that should
ing systemic hemodynamic alterations, clotting disorders, be suspected in young patients with migraines, a family
improving cerebral perfusion, and controlling the inflamma- history of stroke or dementia of early onset, and extensive
tory response. However, there are no guidelines advising on white matter changes on imaging studies, predominantly
how to proceed to prevent or treat LI progression. in the anterior temporal lobes and subinsular white matter
tracts.
1. When should CADASIL be kept in mind?

Case
96 CLINICAL HISTORY 63-year-old female with metastatic breast cancer.
FINDINGS Figure 96-1. Axial T2WI through the medulla. lesion should then be made, with attention to the ipsilateral
There is hyperintensity within the right medullary olive central tegmental pathway (red nucleus to olivary nucleus) or
with minimal swelling (white arrow). Figure 96-2. Axial within the contralateral dentate nucleus or superior cerebellar
post-contrast T1WI. The lesion does not show pathologic peduncle. Histologically, deafferentation of olivary nucleus
enhancement to suggest the presence of a metastatic lesion. input results in neuronal loss and a proliferation of glia which
However, more superiorly, at the level of the contralateral may give the affected olive a hypertrophic appearance.
superior cerebellar peduncle, there is a small enhancing
focus compatible with metastasis (white arrow). Question for Further Thought
1. Can HOD be detected clinically?
DIFFERENTIAL DIAGNOSISMetastatic disease, isch-
emia, demyelination, hypertrophic olivary degeneration Reporting Responsibilities
(HOD). Direct reporting is necessary. HOD is a rare condition and
should be kept in mind whenever nonspecific signal change
DIAGNOSIS Hypertrophic olivary degeneration (HOD). is seen in the medullary olive unexplained by something
more common (multiple sclerosis [MS], stroke, etc.). HOD
DISCUSSION HOD results from an insult that interrupts may be suggested when a potential causative lesion is found
some component of the dentato-rubro-olivary pathway. This in the dentato-rubro-olivary pathway.
pathway, also referred to as the triangle of Guillain-Mollaret,
represents a synaptic network connecting the ipsilateral
red nucleus via the ipsilateral central tegmental tract to the
ipsilateral inferior olivary nucleus within the medulla. The HOD is a rare entity and likely often missed or misdiag-
contralateral dentate nucleus also contributes input to this nosed as some more common brainstem pathology
network, to the ipsilateral red nucleus via the contralateral
superior cerebellar peduncle, and to the ipsilateral olivary Answer
nucleus via the inferior cerebellar peduncles. HOD may be 1. On clinical examination, HOD will present as palatal
suspected when increased T2 signal with or without hypertro- tremor or myoclonus. This finding, subsequent to a known
phy is seen in the medullary olive. A search for the offending brainstem insult, is reasonably specific for the diagnosis.
201

Case
97 CLINICAL HISTORY 38-year-old female with headaches for a number of years that
have increased in frequency and severity over the last several months. She also has
intermittent numbness and tingling in her upper extremities.
FINDINGS Figure 97-1. Axial FLAIR through centrum to the calcification within the white matter (WM) of the right
semiovale. There is a hyperintense mainly cortical mass centrum semiovale. Figure 97-2. Axial post-contrast T1WI
in the parafalcine right frontoparietal lobes. At the lateral through the mass. There is no enhancement of the hypoin-
aspect a thin hypointense line is present that is consistent tense mass. Figure 97-3. Axial GRE through the mass.
with calcification (arrow). There is vasogenic edema lateral There is focal hypointensity in the mass laterally (arrow)
202

Case 97 203
in ODs tend to be significantly higher than that in astro-

cytomas. The median normalized ADC (nADC) has been
found to help separate low-grade OD from low-grade astro-
cytomas with low-grade astrocytomas having higher nADC
values than ODs.
ODs account for 5% to 6% of all gliomas and can occur
at any age, but the majority occurs in adults between 40 and
45years of age. These tumors are rare in children. The tumors
tend to present more frequently in the frontal lobes but can
occur throughout the cerebral hemispheres. Clinically OD
presents with seizures in about two-thirds of the patients.
Headache, neurologic deficit, and cognitive and mental sta-
tus changes are other ways OD could present. ODs are rare in
the brainstem, cerebellar hemispheres, or spinal cord. These
are infiltrating tumors with uniform cell density. Typical his-
tologic features include monomorphic cells with artifactual
Figure 97-5
perinuclear halos (fried egg), nuclei with delicate chroma-
tin and small nucleoli, minigemistocytes (cell with eccentric
nucleus and abundant eosinophilic cytoplasm), microcysts,
microcalcifications, prominent delicate capillary network
corresponding to the region of hypointense signal on the
(chicken wire), perineuronal satellitosis, and perivascular
axial FLAIR. Figure 97-4. ADC map through mass. There
and subpial accumulation. Mitoses are few, and there is no
is predominantly elevated ADC value, but there is a rim of
necrosis or vascular endothelial proliferation. IDH1 muta-
low ADC surrounding the calcification. This could indicate
tions and codeletion of 1p/19q are favorable prognostic fac-
area of increased cellularity or an artifact from the calcifica-
tors when present in OD. Patients with OD have a longer
tion. Figure 97-5. Photomicrograph shows tumor composed
survival than similar grade astrocytomas. The treatment
of monomorphic round to oval cells with characteristic peri-
includes chemotherapy and surgical resection. Malignant
nuclear halos (fried-egg appearance). A delicate capillary
progression may occur but takes a long time.
network (chicken wire capillaries) and psammomatous cal-
cification are also present.
DIFFERENTIAL DIAGNOSIS Astrocytoma, oligodendro- 1. What genetic changes are important to be aware of for OD
glioma (OD), metastatic lesion, ischemic stroke with hemor- and how does current genetic information about OD relate
rhagic transformation. to treatment response?
DIAGNOSIS OD WHO II. Reporting Responsibilities

Direct reporting is necessary particularly if there is signifi-
DISCUSSION ODs are mostly solid supratentorial masses cant mass effect, herniation, and/or hemorrhage. Location
originating from the gray matter with smooth well-demar- of mass in eloquent areas may pose surgical challenge and
cated margins. They are generally hypodense to heteroge- should be mentioned. Presence of enhancement and edema
neous on CT. OD more commonly calcifies than similar may indicate higher-grade OD. Growth rate on follow-up
grade astrocytomas. There is usually local mass effect. evaluation may be important.
MRI best demonstrates the location of the lesion and the
surrounding WM extension. OD is hypointense on T1WI What the Treating Physician Needs to Know
within the cortical or deep gray matter (GM) and hyper- Location and number if more than one lesion
intense on FLAIR and T2WI. This case demonstrates If a large lesion, is there any significant herniation?
no enhancement as is generally seen in low-grade OD. Would advance imaging be useful in further characteriza-
Presence of surrounding edema is variable but is usually tion?
from none to very little. GRE may show focal hypointensity
consistent with calcifications. ODs have a great tendency Answer
to calcify and using s usceptibility-weighted MRI calcifica- 1. Isocitrate dehydrogenase (IDH) mutations are present
tions have been detected in approximately 80% of cases. in a majority of OD. IDH1 is more common than IDH2
Metastatic and astrocytic brain lesions rarely present with mutations and is correlated with 1p and 19q status in ODs.
calcifications. Spectroscopy of OD demonstrates elevated Both IDH and 1p and 19q loss are related to favorable out-
choline (Cho), decreased creatine (Cr), and decreased NAA. come in OD. O6-methylguanine-DNA-methyltransferase
The Cho/Cr ratio has been demonstrated to be significantly (MGMT) gene promoter methylation is common in pedi-
higher in WHO III compared to WHO II OD. The rCBVmax atric and young adult OD.

Case
98 CLINICAL HISTORY 15-year-old male with T-cell lymphoblastic lymphoma. Three
months after initiation of methotrexate treatment, the patient developed a headache with
a progressive right-sided brachiofacial weakness and dysarthria.
204

Case 98 205
FINDINGS Figure 98-1. Axial ADC map through the sple- and there are different theories. MTX competitively inhibits
nium of corpus callosum. There is focal restricted diffu- the enzyme dihydrofolate reductase (DHFR) that is required
sion of the splenium (arrow). Figure 98-2. Axial ADC map for the synthesis of tetrahydrofolate, an important precursor
through the corona radiata. There are bilateral symmetrical for purine base synthesis. The likely depletion of brain folate
white matter (WM) areas of restricted diffusion (arrows). stores as a result of this interaction may be responsible for
Figures 98-3 and 98-4. Axial T2WI through the splenium the neurotoxicity. MTX also promotes release of adenosine
and the corona radiata, respectively. There are confluent from fibroblasts and endothelial cells. High levels of adenos-
hyperintensity in the splenium and bilateral WM (arrows). ine dilate blood vessels and promote release of neurotrans-
mitters that may slow the rate of neuron discharge and may
DIFFERENTIAL DIAGNOSIS Toxic encephalopathy, meth- play a part in the pathophysiology of MTX neurotoxicity.
otrexate leukoencephalopathy (MTX LE), posterior reversible Increased concentration of homocysteine, an excitotoxic
encephalopathy syndrome (PRES), delayed post-hypoxic leu- neurotransmitter, is also present. Increased homocysteine is
koencephalopathy (DPHL) hypoglycemia, hypoxic ischemic thought to cause small vessel vasculopathy which conceiv-
encephalopathy (HIE). ably could promote the changes seen on MRI. The pathology
of MTX LE includes variable changes of demyelination, loss
DIAGNOSIS MTX LE. of oligodendroglia, focal or diffuse areas of WM necrosis,
mineralizing microangiopathy, and glial damage that could
DISCUSSION The MRI findings in MTX LE include bilat- be widespread throughout the CNS. Management of MTX
eral symmetrical diffusion restriction in the cerebral WM LE has included high-dose folinic acid, aminophylline, and
mostly in the corona radiata and centrum semiovale with leucovorin with mixed result. MTX was stopped in this
corresponding hyperintensity on FLAIR and T2WI. The patient and he improved.
T2 hyperintensity may lag behind the DWI changes. Basal
ganglia involvement has also been reported. WM lesions are Question for Further Thought
usually smudgy and diffuse and occur more frequently than 1. Is MTX the only chemotherapeutic agent known to
basal ganglia lesions. Basal ganglia lesions could resemble cause LE?
changes of HIE. Unilateral lesions and lesions isolated to
the corpus callosum or specific areas (anterior or posterior) Reporting Responsibilities
of the centrum semiovale (CSO) have been reported. There Restricted diffusion usually indicates cytotoxic edema sug-
is usually no mass effect and contrast enhancement has not gesting infarction. The history is important in arriving at the
been a feature. Lack of cortical and subcortical involvement diagnosis. Direct reporting is necessary so that prompt man-
differentiates MTX LE from PRES. DPHL could resemble agement could begin. Although the entity is called MTX LE,
MTX LE. These DWI changes are transient and resolu- gray matter could be affected and this may explain the pres-
tion of lesions occurs in a mater of days or weeks. Clinical ence of choreoathetosis in some patients. Presence of gray
improvement is the norm. Persistence of small residual T2 matter involvement may mimic HIE.
changes has been reported as late as 39 months following
the event. Reintroduction of MTX may be associated with
recurrence in up to 56%. Lower MTX dosage might prevent
recurrence. Is there evidence of true ischemia?
MTX is an important component in the treatment of acute MTX LE has been seen with all forms of MTX administra-
lymphocytic leukemia (ALL). Time of occurrence of MTX tion and at different dosages. The risk, however, is higher
LE from the time of MTX administration is variable and most with high-dose treatment, intrathecal administration, and
of the reported cases have been within 1 or 2 weeks following cranial irradiation
administration of MTX but could be as long as 127 months. The clinical recovery of MTX LE (days) is faster than the
The clinical presentation of MTX LE includes headache, MRI recovery (months)
mental status changes, stroke-like symptoms, choreoatheto-
sis, and seizures. Symptoms may fluctuate. Patients are gen- Answer
erally adolescents with no gender preference. The incidence 1. No. Other chemotherapeutic agents used in combination
of acute neurotoxicity associated with treatment of ALL with have been associated with LE and these include vincristine,
a variety of drugs including MTX is about 5% to 18%. The melphalan, cyclophosphamide, BCNU, adriamycin, pro-
pathophysiology of MTX toxicity is not definitely known carbazine, bleomycine, cisplatin, and nitrogen mustards.

Case
99 CLINICAL HISTORY 63-year-old male with liver transplantation 4 years prior was
admitted for mental status changes and headache.
FINDINGS Figure 99-1. Axial NCCT of the head. There (not shown). Figure 99-3. Coronal non-contrast T1WI
is a small focus of hyperdensity (blood) in the anterior left through the level of the sphenoid sinus. There is hyperintense
suprasellar cistern in the vicinity of the left internal carotid sphenoid sinus opacification with surrounding hypointense
artery (ICA) (arrow), and the rest of the suprasellar cistern material (vertical arrow). The sella turcica and suprasellar
is isodense to brain presumably due to inflammatory tissue. cerebrospinal fluid (CSF) are isointense with the brain (star)
Figure 99-2. DWI through the level of the basal ganglia. consistent with granulation/inflammatory tissue replacing
There is restricted diffusion in the left basal ganglia and thal- the suprasellar CSF. There is patchy left basal ganglia hyper-
amus (arrow) and elsewhere in the left cerebral h emisphere intensity consistent with hemorrhagic infarcts (transverse
206

Case99 207
arrow). Figure 99-4. Axial T2WI through the sphenoid sinus. to contiguous spread of an infection resulting in proximal
There is mixed signal intensity within the sphenoid sinus MA, bloodstream infection, or septic embolization result-
(vertical arrow), either blood or fungus infection. Figure ing in distal MA. Bacteria (Streptococci spp., Staphylococci
99-5. Coronal post-contrast T1WI through the levels of the spp.), fungi (Aspergillus spp., Candida spp., Coccidioides,
suprasellar cistern and basal ganglia. There is diffuse contrast agents of mucormycosis), parasites, and viruses have been
enhancement extending from the sphenoid sinus through the implicated as etiologic agents. CTA and MRA are equally
sella turcica and suprasellar cistern (inflammation) into the effective in demonstrating MA.
left basal ganglia (subacute infarct). There is a defect in the MA accounts for 0.7% to 6.5% of all intracranial aneu-
floor of the sella turcica (vertical arrow) suggesting route of rysms. Infective endocarditis, cavernous sinus thrombo-
infection to the cranial cavity. There is a target-like hypoin- phlebitis, sinusitis, otitis media, mastoiditis, meningitis, and
tensity in the region of the left ICA terminus suggesting an orbital cellulitis are the common causes of MA. Intracranial
aneurysm (transverse arrow). Figure 99-6. 3D TOF MRA. MAs are usually silent. The patient might develop signs and
There is a lobulated aneurysm of the left ICA terminus with symptoms later in the course of the disease due to aneurysm
occlusion of the left anterior cerebral artery (ACA). enlargement, SAH, or ICH. The most common symptoms are
fever, headache, seizures, focal neurologic deficits, or men-
DIFFERENTIAL DIAGNOSIS subarachnoid hemorrhage, tal status changes. Unruptured MAs carry a 30% mortality,
fungal infection, left internal carotid aneurysm, basal men- while rupture is associated with much higher mortality up
ingitis. to 80%. These patients require long-term antibiotic therapy.
Aggressive treatment is recommended in the immunocom-
DIAGNOSIS Mycotic aneurysm (MA) of the left internal promised patient. There are specific indications for endovas-
carotid terminus due to transdural fungal invasion from the cular and surgical treatment of MA. Left nasal endoscopy
sphenoid sinus. with left total ethmoidectomy and left sphenoidotomy was
performed in this patient revealing acute and chronic inflam-
DISCUSSION This left ICA MA is due to transdural spread mation with fungal hyphae suggestive for Aspergillus. The
of sphenoid sinus Aspergillus spp. infection via the sella tur- patient was treated with oral and intravenous antifungal
cica into the suprasellar cistern. Like every aneurysm, there medication.
is an outpouch from the left ICA terminus. This developed
over a period of weeks. The suprasellar subarachnoid hemor- Question for Further Thought
rhage (SAH) was due to rupture of the aneurysm with sub- 1. How do we monitor MA to assess the response to treat-
sequent intraventricular hemorrhage (IVH) (not shown). Left ment?
hemispheric infarcts are a product of distal embolization and/
or occlusion of small vessels due to the infection. SAH, intra- Reporting Responsibilities
cerebral hemorrhage (ICH), and IVH are complications of a MA is an emergent condition deserving of direct reporting.
ruptured aneurysm. MA represents an abnormal focal arterial The number and locations of other aneurysms should be
dilatation due to degeneration of the arterial wall secondary noted. Presence of infarcts, SAH, IVH, ICH, distal v ascular

208 Case99
occlusions, and arterial spasms should be documented. Presence of inciting pathology such as sinus infection or
Hydrocephalus may require emergent ventricular drainage underlying cardiac disease
and should be directly communicated.
Answer
What the Treating Physician Needs to Know 1. CTA and MRA are equally effective in monitoring the
Location, number, and size of aneurysms on initial and progress of MA. CTA appears to be more effective for
follow-up imaging small distal MA. There is no consensus as to whether this
Complications such as SAH, ICH, infarcts, hydrocephalus should be done weekly or monthly.
on initial and follow-up study. These will affect manage-
ment decisions

Case
100 CLINICAL HISTORY 32-year-old male presenting with headache.
FINDINGS Figure 100-1. Sagittal T1-weighted brain displaced, there are low-lying cerebellar tonsils (black arrow)
MRI demonstrating midline structures. There is sagging and enlargement of the pituitary gland (long white arrow).
of the splenium of the corpus callosum depressing the Figures 100-2 and 100-3. Axial FLAIR and coronal T2WI,
internal cerebral veins/vein of Galen junction (short white respectively. There is diffuse T2-hyperintense subdural fluid
arrow). Although the brainstem does not seem particularly bilaterally involving the supratentorial (white arrows) and
209

210 Case 100
2 mm, less intense than normal cavernous sinus enhance-

ment, discontinuous, and more prominent at the convexity.
In meningitis, pial-subarachnoid enhancement is greater than
dura-arachnoid enhancement that characterizes intracranial
hypotension. Idiopathic hypertrophic pachymeningitis is
characterized by localized or less often diffuse dural thicken-
ing more than 75% dural surface of unknown cause and may
be difficult to differentiate from SIH, although the headaches
are not usually orthostatic, there may be bony invasion, and
sagging of the midbrain is not usually apparent.
Typically affecting young/middle-aged adult females, SIH
is a syndrome of reduced cerebrospinal fluid (CSF) pressure
clinically characterized by orthostatic headaches that occur or
worsen with upright posture. Most cases of SIH result from
a slow, persistent CSF leak, commonly after lumbar punc-
ture, surgery, or trauma. At times, an occult CSF leak may
develop spontaneously in the presence of a microstructurally
weakened dura and/or arachnoid diverticula. Medical causes
of SIH include severe dehydration, diabetic coma, uremia, and
systemic illness. Pathophysiologically, CSF and blood vol-
Figure 100-5 ume are inversely related according to the Monro-Kellie doc-
trine, such that reduced CSF volume, and consequently low
CSF pressure, lead to compensatory dilatation of the vascular
infratentorial compartments (figure 3 inferior white arrows). spaces, mostly of the dural venous sinuses due to higher com-
Figures 100-4 and 100-5. Axial and coronal post-contrast pliance on the venous side. By definition, CSF opening pres-
T1WI. There is diffuse, intense, smooth pachymeningeal sure is low (less than 6 mm H20) or may be unobtainable (dry
enhancement (white arrows). tap), and CSF analysis may be within normal limits or may
reveal increased protein, lymphocytic pleiocytosis, or xantho-
DIFFERENTIAL DIAGNOSIS Meningitis, meningeal chromia. The natural history and prognosis for this condition
metastases, idiopathic hypertrophic pachymeningitis sponta- is spontaneous resolution over a period of weeks to months,
neous intracranial hypotension (SIH). and treatment options for the associated headaches are similar
to those for post-lumbar puncture headaches, including bed
DIAGNOSIS Spontaneous intracranial hypotension (SIH). rest, analgesics, hydration, oral caffeine, epidural blood patch,
and epidural saline infusion.
DISCUSSION The classic imaging quartet for the diag-
nosis of SIH is as follows: (1) downward displacement of the Question for Further Thought
brain through the incisura, (2) diffuse smooth dural thick- 1. What imaging protocols are utilized in the delineation of
ening/enhancement, (3) distension of veins/dural sinuses, the precise site of CSF leak?
and (4) subdural hematomas/hygromas. Unenhanced CT is
relatively insensitive, although it may detect bilateral sub- Reporting Responsibilities
dural fluid collections, thickened dura, or effacement of Direct reporting is essential, and attempts should be made
the suprasellar cistern. Sagittal T1WI may show sagging to obtain as detailed a medical history as possible prior to
midbrain displaced below the level of the dorsum sella, interpretation, inquiring about recent lumbar puncture, sur-
sagging of the splenium of the corpus callosum, com- gery, or trauma. Timely diagnosis is important, as patients
pression of the pons against the clivus, inferior descent of who are encephalopathic may require emergent intrathecal
the cerebellar tonsils, and an apparently enlarged pituitary saline infusion. Large subdural hematomas may cause mass
gland. Axial MRI images may reveal small, distorted lat- effect and acute deterioration. Albeit rare, coma and death
eral ventricles with medial displacement of the atria, as well from severe intracranial herniation are possible.
as increased fluid around the optic nerves. T2-weighted/
FLAIR images demonstrate thickened, hyperintense dura, What the Treating Physician Needs to Know
and subdural fluid hygromas/hematomas. Most importantly, Presence and severity of brain descent
post-contrast T1WI reveal diffuse, intense pachymeningeal Suspected site of CSF leak
(dura-arachnoid) enhancement that is smooth, not nodular
or lumpy bumpy as would be seen with leptomeningeal Answer
metastases. The enhancement involves both the supratento- 1. Although many CSF leaks remain occult on all types of
rial and infratentorial compartments and may extend into the spinal imaging studies, CT slices obtained from the skull
CPA or IAC. Normal dural enhancement is thin less than base through the entire spinal axis after CT myelography

Case 100 211
is the protocol of choice in delineating the precise site less precise than CT myelography, is considerably lower
of epidural contrast extravasation. Digital myelography in radiation exposure. RNC may be used to directly show
may demonstrate active extravasation at the site of leak. a CSF leak by focal accumulation of radioactivity outside
Remember that lumbar puncture performed for myelogra- the subarachnoid space. Indirect signs on RNC include
phy may lead to a CSF leak. In order to avoid the radiation poor migration of radiotracer over the convexities, rapid
exposure incurred by obtaining a CT myelogram of the radiotracer washout, and early appearance of radioactivity
entire spine, radionuclide cisternography (RNC), although in the urinary system.

Case 101 CLINICAL HISTORY 15-year-old female first developed vertigo and imbal-
ance with complete recovery. Six weeks later she presented with lower extrem-
ity numbness and weakness, bowel and bladder dysfunction.
same level as in Figure 101-1. Some lesions show black holes

(arrows). Figure 101-3. Sagittal T2 FLAIR. There are mul-
tiple callosalseptal interface hyperintense lesions (arrows).
DIFFERENTIAL DIAGNOSISToxic metabolic encepha-

lopathy (medication induced, cocaine, inhaled opiates,
chemoradiation, Marchiafava-Bignami), inflammatory demy-
elination (acute disseminated encephalomyelitis [ADEM]
and Neuromyelitis optica [NMO]), systemic lupus erythema-
tosus (SLE) Susac syndrome (SS) Multiple sclerosis (MS).
DIAGNOSIS Multiple sclerosis (MS).
DISCUSSION The interest in this case is in the corpus cal-

losum lesions. Corpus callosal involvement in MS has been
Figure 101-3 recognized for decades owing to the high number of myelin-
ated fibers and is associated with cognitive loss. Callosal
demyelinating lesions are usually multiple, small, nodular,
FINDINGS Figure 101-1. Axial FLAIR through the corona and characteristically T2 hyperintense involving callosal
radiata. There are multifocal hyperintense lesions of variable septal interface and arise from the inferior aspect of the CC.
shapes and sizes in the periventricular, deep, and subcortical This is distinctive from SS where the lesions are usually round
white matter (WM) in the bilateral corona radiata (transverse (snowball) and affect the central fibers. Infarcts and other
arrows), some smudgy hyperintensities around the occipi- inflammatory and vascular lesions tend to stay centrally in
tal horns. There is a left splenium callosalseptal interface the CC. Toxic lesions have a tendency to affect the splenium
lesion (vertical arrow). Figure 101-2. Axial T1WI through and genu and may disappear on follow-up studies. Presence
212

Case 101 213
of ovoid pericallosal lesions or Dawson fingers along the CC evaluation is not complete without sagittal FLAIR
large periventricular collecting veins tends to support the or T2WI because CC lesions could be easily missed on
diagnosis of MS. These are attributed to so-called perivenular axial cuts
inflammation. MS plaques in general have a p redilection for
perivenular spaces as the blood flow is known to be slowest in
that compartment allowing the extravasation of inflammatory Answers
cells through the bloodbrain barrier. There are several pat- 1. Dawson was a Scottish pathologist who described the
terns of lesion contrast enhancement in MS, with the incom- pericallosal lesions.
plete or open ring being the most specific for MS. 2. CC lesions are present in other demyelinating syndromes
MS affects all ages but more commonly in the young with such as ADEM and NMO. The literature does not report
female preponderance. Clinical manifestations include numb- differentiating elements between CC involvement in MS
ness, visual disturbances, vertigo, focal neurologic deficits; and ADEM.
symptoms and signs that wax and wane. Diagnosis is made on 3. Most recently some differences have been reported with
set criteria that recognize dissemination in time and space and CC involvement in MS and NMO. CC is frequently
involvement of multiple compartments including supratento- affected in NMO contrary to the widespread belief that
rial, infratentorial, spinal cord and the eyes. It usually evolves brain lesions are uncommon in NMO. The frequency of
into a chronic disease with subsequent disability and cognitive these lesions parallels the AQP4 water channel distribu-
dysfunction. Treatment usually consists of immunotherapy. tion in the lower surface of the CC. Diffuse and heteroge-
neous cystic lesions with blurred margins in the splenium
Questions for Further Thought of the CC are more frequent in patients with NMO than
1. Who was Dawson? MS and are frequently visualized on axial MRI of the
2. How can CC lesions in MS be differentiated from ADEM? brain, while a sagittal FLAIR or T2 is most often required
3. How can CC lesions in MS be differentiated from NMO? to visualize the lesions of MS. The heterogeneity of NMO
CC lesions in the acute stage has been referred to as mar-
Reporting Responsibilities bled appearance with a hyperintense rim and hypointense
Direct reporting is essential. It is suggested that the earlier core on T2WI. Similar to MS lesions, CC lesions in NMO
the treatment for MS is started, the more favorable the out- might disappear or become hypointense. Previous histo-
come. Lesion number and location and presence of contrast pathologic studies in NMO revealed extensive necrotic
enhancement should be noted. Contrast enhancement usually cavitary brain lesions associated with the loss of AQP4
denotes acute and active lesions. immunoreactivity. The presence of cystic lesions in the
CC of NMO patients is therefore not unexpected.
CC lesions are not pathognomonic of any specific disease,
but their configuration and location might provide a clue
to the diagnosis

Case
102 CLINICAL HISTORY 22-year-old male with mental status changes.
Figure102-3
Figure102-4
FINDINGS Figure 102-1. Axial DWI through the mid- (arrows). Inferior extension of the lesion to the medulla is
brain. There is hyperintensity posteriorly in the midbrain present (not shown). Figure 102-6. Axial FLAIR through
(arrow). ADC map (not shown) did not show low ADC. the pons 4 months later. There is minimal residual hyper-
Figures 102-2 and 102-3. Axial DWI with corresponding intensity in the tegmentum (arrow). There is no contrast
ADC map through the basal ganglia. There are multifocal enhancement on the initial and follow-up images in any of
areas of restricted diffusion (arrows) in the left caudate the areas involved.
nucleus, right insula, and operculum. Figures 102-4 and
102-5. Axial T2WI through the pons and midbrain, respec- DIFFERENTIAL DIAGNOSIS Multifocal infarcts, demy-
tively. There is confluent hyperintensity in the pontine teg- elinating process, encephalitis, brainstem glioma, osmotic
mentum extending into midbrain tegmentum and tectum demyelination.
214

Case 102 215
Figure102-6
Figure102-5
DIAGNOSIS Brainstem encephalitis (presumed Bickerstaff (MFS), Guillain-Barr syndrome (GBS), and acute demy-
brainstem encephalitis [BBE]). elinating encephalomyelitis (ADEM). There is usually a pre-
ceding infection resulting in a prodromal stage. Treatment is
DISCUSSION BBE is a rare autoimmune brainstem by corticosteroids, airways management, intravenous immu-
encephalitis of unknown etiology. The MRI changes of BBE noglobulin, and plasmapheresis.
which is present in about 30% of the patients are a longitu-
dinal confluent T2 hyperintensity in the brainstem extending Question for Further Thought
from the medulla to the midbrain, usually without restricted 1. Which are the organisms implicated in the prodromal
diffusion or contrast enhancement, but some expansion of the phase of BBE?
brainstem. It has been described by some as vasogenic edema,
while some have reported areas of restricted diffusion in some Reporting Responsibilities
cases. In most of the images published, the tegmentum has Direct reporting is indicated in view of the differential and
been the region most affected. Concomitant involvement of the necessity for aggressive management in this otherwise
the cerebellum and supratentorial structures has been reported. curable illness.
A negative MRI does not exclude the diagnosis if the clinical
criteria are met. The diffuse nature of the changes and lack of What the Treating Physician Needs to Know
restricted diffusion exclude brainstem infarct. It may be diffi- Extent of disease
cult to completely exclude other demyelinating processes. The Reasonable differential diagnosis
wide age range does not fit with brainstem glioma. Osmotic
myelinolysis usually spares the periphery and is mostly con-
fined to the pons. Usually there is complete recovery with Answer
resolution of the signal changes in BBE as in this case. 1. BBE has been reported following infection with several
The clinical criteria for BBE include ataxia with bilat- organisms including Mycoplasma pneumoniae, cytomeg-
eral ophthalmoplegia. There is usually disturbed conscious- alovirus, Haemophilus influenzae, and Campylobacter
ness, bulbar palsy, pupillary changes, facial weakness, and jejuni. The exact pathogenesis is unknown. BBE is asso-
hyperreflexia. Tetraparesis could be present in about 50% of ciated with the presence of antiganglioside antibody,
the patients. The diagnosis is supported by the presence of (anti-GQ1b), which is also present in other diseases
anti-GQ1b antibodies and an abnormal brain MRI. However, with similar prodromal phase such as MFS, ADEM,
absence of these does not exclude the diagnosis. There is and GBS indicating similar pathogenetic mechanism of
clinical overlap between BBE, Miller Fisher syndrome these diseases.

Case
103 CLINICAL HISTORY 91-year-old male fell 2 days earlier and now has a
change in mental status.
FINDINGS Figure 103-1. Axial NCCT through the tento- layering on the tentorium and along the falx. The cerebral
rial incisura. There is a 1.5-cm thick crescentic hyperdense hemisphere is displaced away from the inner table of the
right temporaloccipital extraaxial collection displacing the skull or falx with compression and effacement of the sulci.
right temporal and occipital lobes from the inner skull table Medium- and large-sized ASDH tend to displace midline
consistent with acute subdural hematoma (ASDH) (trans- structures, resulting in subfalcine, uncal, and transtento-
verse arrows). There is hyperdense subdural hematoma (SDH) rial herniations. Massive transtentorial herniation could
around the right tentorium (triangle). There is crowding of produce Duret hemorrhages of the brainstem. The ipsilat-
the incisura with effacement of the cerebrospinal fluid (CSF) eral lateral ventricle is usually compressed and obliterated
spaces. The right temporal horn is obliterated. The left temporal along with the basal cisterns. Compression of the third ven-
horn is severely dilated with periventricular hypodensity (verti- tricle and the foramen of Monro tend to create obstruction
cal arrow) consistent with transependymal CSF permeation due to CSF flow from the contralateral lateral ventricle result-
to the acute hydrocephalus. Figure 103-2. Axial NCCT through ing in acute hydrocephalus. Acute periventricular edema
the suprasellar cistern. There is obliteration of the suprasellar may surround the dilated ventricle. The contralateral con-
(star), interpeduncular, perimesencephalic, and quadrigemi- vexity sulci may be effaced by the increased pressure in the
nal cisterns by a right uncal and transtentorial herniation. The lateral ventricle. Compression of ipsilateral cortical veins
left trigone is massively dilated (vertical arrow). There is acute may result in venous congestion and edema of the cerebral
hemorrhage (hyperdensity) in the right quadrigeminal cistern/ white matter particularly in the subcortical regions. The
midbrain (chevron). The calcified pineal gland is displaced other complications of the herniations may include infarc-
inferiorly and to the leftmidline shift (transverse arrow). tions in the vascular territories of the posterior cerebral
artery (PCA), superior cerebellar artery (SCA), anterior
DIFFERENTIAL DIAGNOSIS N/A. choroidal artery, and anterior cerebral artery (ACA) due to
compression of these arteries.
DIAGNOSIS Acute subdural hematoma (ASDH) with There is occasional hypodense or isodense ASDH, which
complications. has been explained on the basis of severe anemia or dilution
of the ASDH by CSF that has entered the subdural space
DISCUSSION The hallmark of ASDH on CT is the through an arachnoid tear. This is actually a rare phenom-
crescentic hyperdense extraaxial collection. The collec- enon. Active bleeding into the ASDH may result in a mixed
tion can freely distribute around the cerebral hemisphere, density hematoma. This is present about 40% of the time.
216

Case 103 217
ASDH is more common supratentorially than infratentori- Reporting Responsibilities

ally. SDH is generally believed to be due to rupture of corti- This is an emergency and should be directly reported. Size,
cal veins as they cross the potential subdural space caused location, complications such as herniations, infarcts, hydro-
by minor or severe abnormal brain rotations or skull frac- cephalus and presence of active bleed should be reported.
tures. It has also been postulated that arterial bleed could Significant changes on follow-up should also be directly
cause ASDH. Such bleed tends to be large and rapidly pro- reported. Presence of other primary and secondary brain inju-
gressive. SDH may be associated with other brain parenchy- ries should be mentioned.
mal injuries or traumatic subarachnoid hemorrhage. Other
causes of SDH include coagulopathies, surgical trauma, What the Treating Physician Needs to Know
ventriculoperitoneal shunts, and neoplastic lesions of the Location, size, complications, other primary and second-
dura or arachnoid. It has no gender predilection and occurs ary injuries
in all ages. Presentation may include headache, focal neuro- Usefulness of other imaging modalities in defining clinical
logic deficit, seizures, changes in mental status, and coma. outcome
The management of ASDH depends on several factors Significant changes on follow-up
that include the Glasgow Coma Scale (GCS) score, neu-
rologic state of the patient, the size and acuity of the SDH Answers
along with degree of midline shift, associated primary inju- 1. MRI is not essential in making the diagnosis of ASDH.
ries and herniations on CT, clinical condition of the patient at The patients clinical condition usually does not permit
presentation and over time, as well as the age of the patient. MRI evaluation in the acute phase. In the long term, how-
Small ASDH in a clinically stable patient could be conser- ever, MRI could be useful in evaluation of inappropriate
vatively managed. Symptomatic lesions are generally surgi- response to management such as stagnant or deteriorating
cally treated. Outcome generally depends on the GCS score clinical state. MRI could better define the extent of contu-
at presentation; the lower the score, the higher the mortal- sions, infarctions, or DAI that may have been missed or
ity. ASDH thicker than 1 cm, producing more than 5 mm of not visualized on CT. These could help in defining prog-
midline shift, the presence of transtentorial herniation, and nosis in such cases. MRI could also be useful in evalu-
elevated intracranial pressure (ICP) are all associated with ation of SDH due to underlying meningeal pathologies
significant morbidity and mortality. such as neoplasm or vascular malformations.
2. The role of MRI has been described above. CTA or DSA
Questions for Further Thought could be useful in defining bleeding vessels and such
1. Is MRI essential in the management of ASDH? bleeding points could be treated via the endovascular
2. Are there other imaging modalities that could be useful in route. Perfusion studies may be able to define abnormal
the management of SDH? blood flow in the brain. Such perfusion abnormalities
have been associated with poor outcome in SDH.

Case
104 CLINICAL HISTORY 11-month-old male with lack of progression in develop
mental milestone and an enlarging head circumference.
FINDINGS Figure 104-1. Axial T2WI through the basal

ganglia. There is diffuse white matter (WM) hyperintensity
with involvement of the globus pallidus and anterior aspect
of the cerebral peduncles (arrows). Figure 104-2. Coronal
T2WI through the basal ganglia. There is hyperintensity in
the WM and bilateral globus pallidus (arrows). Figure 104-3.
Axial T1 inversion recovery image through the basal ganglia.
There is marked hypointensity throughout the WM.
DIFFERENTIAL DIAGNOSISPelizaeus-Merzbacher
disease, Alexander disease, Canavan disease mucopolysac-
charidoses, megalencephalic leukodystrophy with cysts.
DIAGNOSIS Canavan disease.
DISCUSSION Both CT and MRI are capable of demon-

strating the imaging findings of Canavan. As usual MRI is
the examination of choice in view of its superior capabilities.
CT and MR show confluent WM disease, hypodense on CT
and hyperintense on T2WI. Lesions may spare the external
and internal capsules, the corpus callosum, and the deep cer-
ebellar WM. Atrophic changes are common and vary from
mild to severe. These changes are usually nonspecific but
Figure 104-3 posterior structures are more clearly affected than frontal
218

Case 104 219
lobes in contradiction to Alexander disease that involves the Reporting Responsibilities

frontal lobes predominantly, the other WM disease present- Routine reporting is sufficient as the diagnosis is laboratory
ing with macrocephaly like Canavan. The most common based and imaging is only supportive. The accumulation of
abnormalities found in patients with m ucopolysaccharidoses NAA is disproportionately greater in Canavan disease than
are cystic lesions that correspond to enlarged Virchow-Robin in any other central nervous system (CNS) condition; there-
perivascular spaces and are linear and oriented along the fore, spectroscopy is useful for making the diagnosis and
medullary vessels mostly in the parieto-occipital WM. should be recommended.
Subcortical cysts are common with megalencephalic leuko-
dystrophy. MR spectroscopy shows elevated N-acetylaspartic What the Treating Physician Needs to Know
acid (NAA) peak in Canavan disease, perhaps the only dis- Canavan disease may affect any ethnic group but has a
ease known to do this. Confirmation of the diagnosis of high prevalence among Ashkenazi Jews (1 in 40 is a
Canavan disease relies primarily on biochemical findings. carrier)
Canavan disease is a rare severe progressive autosomal Genetic counseling is recommended for people who want
recessive spongiform leukoencephalopathy that leads to death to have children and have a family history of Canavan
in the first decade of life. The histologic spongy degeneration disease
of the brain was first described by Canavan in 1931 but not Counseling should be considered if both parents are of
recognized as a specific entity until 1949 by van Bogaert and Ashkenazi Jewish descent. For this group, DNA testing
Bertrand. Prior to 1988 the diagnosis could only be confirmed can almost always tell whether one or both parents are
by brain biopsy or at autopsy. Since then, the basic defect carriers
in Canavan disease was determined to be a deficiency in the
NAA degrading aspartoacylase enzyme. This allowed for the Answers
development of a laboratory diagnosis by analyzing the blood 1. Urine NAA is greatly elevated in patients with Canavan
or urine for NAA. In 1994, the ASPA gene responsible for disease which allows for a convenient, noninvasive
production of aspartoacylase was localized to chromosome laboratory test.
17, which led to the identification of many mutations result- 2. Because the underlying deficit in Canavan disease is
ing in the disease. Although the precise cellular mechanism the absence of a single enzyme as the result of a single
is not well understood, the lack of breakdown of NAA and its gene mutation, there have been clinical trials involv-
marked subsequent accumulation is believed to interfere with ing virus gene transfer containing recombinant DNA of
normal myelin production in the brain. the missing gene. Gene therapy showed a decrease in
elevated NAA in the brain and slowed progression of
Questions for Further Thought brain volume loss. Improvement in seizure frequency
1. What is the laboratory test used to confirm Canavan disease? and stabilization of overall clinical status were also
2. Is gene therapy for Canavan an effective treatment? observed.

Case
105 CLINICAL HISTORY Panel of normal intracranial venous anatomy.
Figure 105-1
Figure 105-2
Figure 105-3
Figure 105-4

220

Case 105 221
FINDINGS All MRV images are contrast-enhanced brain

MRV, MIP, or volume rendered in appropriate projections
to show the sinuses.
Figure 105-1. Sagittal MIP. The superior sagittal sinus
(SSS) (vertical arrows) drains the cortical veins into the tor-
cula (star). The largest of the cortical veins is the vein of
Trolard or the superior anastomotic vein (transverse arrows)
connecting the SSS to the middle cerebral vein of Sylvius.
The deep structures are drained by the paired internal cere-
bral veins (ICVs) (line arrows). The ICVs are joined by the
basal veins of Rosenthal (pentagon) to form the great vein
of Galen (GVG) (arrowhead). The GVG is connected to the
torcula by the straight sinus (SS) (chevron). On this sagittal
MIP, the bilateral transverse and sigmoid sinuses are super-
imposed in the posterior fossa (curved arrow). The occipi-
tal sinus is a midline structure (triangle) draining into the
torcula. Figure 105-2. MRV submentovertical view (SMV)
MIP. The midline structures of the SSS and the ICVs are
superimposed in the midline (transverse arrows). The
Figure 105-9 bilateral transverse sinuses (TVSs) are peripherally placed

222 Case 105
p osteriorly against the occipital bones (vertical arrows). The The larger sinuses of the SSS, the transverse/sigmoid
TVS drains via the sigmoid sinus on either side (chevrons) sinuses, the ICVs, the GVG, and the SSs are seen in all
into the internal jugular veins (IJV). Figure 105-3. Oblique normal situations while the ISS is present in 46% to 52%.
sagittal MRV MIP in another patient. There is a bifid torcula. The occipital sinus is present in 10% to 18%. The veins of
The SSS preferentially drains into the right TVS (transverse Labbe and basal veins of Rosenthal are present in over 90%.
arrow). The SS preferentially drains into the left TVS (ver- Thelocation of the anastomotic vein of Labbe that connects
tical arrow). The vein of Labbe is shown on the right side the middle cerebral vein to the TVS is variable. The anasto-
(chevron) draining into the lateral aspect of the TVS. Figure motic vein of Trolard is inconstantly visualized and present
105-4. The inferior sagittal sinus (ISS) (arrows) drains into in less than 40%. There is great variability in the visualization
the SS. Figure 105-5. Coronal view from behind. The cav- of the smaller veins. The right TVS is dominant in 54%to
ernous sinuses (transverse arrows) on either side of the sella 59%, the left in 25% to 36% while the two are co-dominant
turcica and the retroclival sinus complex (vertical arrows) in 8% to 16%. The SSS preferentially drains into the right
are demonstrated. Figure 105-6. Volume-rendered 3D of TVS in 52%, the left TVS in 30%, and the torcula in 18%.
the right inferior petrosal sinus emptying into the right sig- The SS preferentially drains into the right TVS in 20%, the
moid jugular junction (arrows). Figure 105-7. Axial GRE left in 34%, and the torcula in 46%.
through the SSS. The SSS is smooth walled and hyperin-
tense (arrows). Acute thrombus usually shows GRE bloom- Question for Further Thought
ing. Figure 105-8. Axial T2WI through the SSS. The SSS is 1. What are the imaging findings in acute sinus thrombosis?
usually a smooth-walled flow void (arrows). SSS is hyperin-
tense in acute thrombosis. Figure 105-9. Volume-rendered Reporting Responsibilities
3D contrast-enhanced MRV. There is duplication or large Unless there is thrombosis, reporting is routine. Detail of
fenestration of the SSS posteriorly (transverse arrows) with what sinuses are visualized, obstructed, hypoplastic, or not
each side draining into their respective TVS with a cross-link visualized should be recorded. Usually MRI of the brain is
(vertical arrow) representing the torcula. obtained at the same time. Relevant changes on the MRI
should be tied to the findings on the MRV. Presence of new
DIFFERENTIAL DIAGNOSIS N/A. collaterals should be noted.
DIAGNOSIS Normal cranial venous sinuses by MRV. What the Treating Physician Needs to Know
List of veins and sinuses visualized and explanation for
DISCUSSION Three-dimensional contrast-enhanced those not seen or those of irregular size
MRV of the brain is now judged to be the best imaging tech- Extent of abnormalities discovered
nique for evaluating the cranial venous sinuses. The presence Parenchymal changes if any
of artifacts is substantially reduced and the full extent of the
vessels is visualized. The non-contrast images are notorious Answer
for flow gaps within the sinuses mimicking absence or throm- 1. Acutely thrombosed venous sinuses tend to show hyper-
bosis. Of course, the MRV should be interpreted in conjunction intensity on FLAIR and T2WI, iso- to hyperintensity on
with the MRI, which should show the usual signal void in the T1WI, and blooming on GRE. Acutely thrombosed sinus
venous sinuses on T2WI and hyperintensity on GRE. Presence is usually large and may be smooth walled or irregular.
of signal within the sinuses on T2WI and blooming on GRE MRV will usually show absence, diminished, and irregu-
should raise suspicion of thrombosis. On T1WI it is possible lar size of the sinus depending on the degree of throm-
for slow flow to exhibit iso or hyperintensity within the large bosis. Collateral veins may form and cortical veins may
sinuses of the SSS and the transverse sigmoid complex. equally be thrombosed and poorly visualized.

Case
106 CLINICAL HISTORY Patient with red and swollen left eye.
FINDINGS Figure 106-1. Coronal NCCT through the DISCUSSION CCF implies an abnormal communica-
orbits. There is enlargement of the left superior ophthalmic tion between the cavernous sinus and some element of
vein relative to the right (arrows). Figure 106-2. DSA left the carotid vasculature, either the ICA itself or a branch
internal carotid artery (ICA) PA (posterior-anterior) view. of the ICA. CT and MRI show enlarged cavernous sinus
There is abnormal early and dense opacification of the left with corresponding enlargement of the superior ophthal-
cavernous sinus with early opacification of the contralateral mic vein. The findings could be bilateral in high-flow fis-
right cavernous sinus (black arrow) and the bilateral jugular tulas with cross communication to the contralateral side.
veins (white arrows). Figure 106-3. Coronal T2WI through Proptosis with orbital fat stranding and enlarged extra-
the orbits in a companion case with incidental findings at ocular muscles may be present. Brain parenchymal and/or
imaging for unrelated complaint. There is mild prominence subarachnoid hemorrhage from ruptured engorged cortical
of the right superior ophthalmic vein (arrows). Figure 106-4. veins are occasionally present. Retroclival and pterygoid
Time of flight MRA source images level of the cavernous venous plexus enhancement could be present. CT and MR
sinus in the same patient as figure 106-3. There is asymmet- angiography may be diagnostic in showing abnormal early
ric flow-related signal in the right cavernous sinus. opacification or abnormal flow in the cavernous sinus and
prominence of the cavernous sinus outflow pathways. DSA
DIFFERENTIAL DIAGNOSISCavernous sinus thrombo- is diagnostic and will reveal the source arteries and full
sis, carotid cavernous fistula (CCF), cavernous ICA aneurysm. pattern of venous outflow.
CCFs are rare and are classified as being either high flow
DIAGNOSIS Both patients have CCF. (direct) or low flow (indirect). High-flow CCFs are typically
223

224 Case 106
posttraumatic or secondary to rupture of a cavernous ICA of one or both cavernous sinuses is seen or when the superior
aneurysm. Low-flow CCFs arise from small dural arteriove- ophthalmic veins are larger than normal.
nous (AV) fistulas involving the cavernous sinus and menin-
geal branches of the external carotid artery (ECA) and ICA. What the Treating Physician Needs to Know
Standard imaging can suggest the diagnosis when abnormal CCF is an infrequent but potentially serious condition as
arterial flow into the cavernous sinus results in expansion of sequelae include loss of vision, intracerebral hemorrhage,
the sinus and its outflow pathways (such as the superior oph- subarachnoid hemorrhage, and venous thrombosis
thalmic vein). Is it a direct or indirect fistula? This will affect how it is
The most common cause of a direct CCF is trauma, usu- treated
ally high flow and most commonly occurs in the young but Are there any complications?
could be found in all ages and both gender. Indirect or dural All arterial feeders and venous outflows should be
CCF on the other hand tends to occur in elderly women and is delineated
usually low flow. Complications of CCF include vision loss
due to central retinal vein occlusion or secondary glaucoma, Answer
epistaxis that could be fatal, subarachnoid hemorrhage, and 1. All direct or high-flow CCFs should be treated. These
intracerebral hemorrhage due to retrograde venous drainage. are usually of acute onset and result in striking clinical
findings including orbital swelling, chemosis, bruit,
Question for Further Thought bulging red eye, and loss of vision. Endovascular embo-
1. When and how should CCFs be treated? lization is the preferred approach. For low-flow CCFs,
particularly asymptomatic or incidental lesions, the
Reporting Responsibilities need for treatment should be evaluated on an individual
Direct reporting is required because of the progressive visual basis. A mild CCF may never produce symptoms, and
complications or fatal hemorrhages if left untreated. Suspect the available treatments are not without substantial risk
CCF and suggest dedicated imaging when abnormal bulging themselves.

Case
107 CLINICAL HISTORY 59-year-old female on chronic immunosuppression with
mycophenolate mofetil and tacrolimus for kidney and pancreas transplant per-
formed 7months prior was admitted with decreased level of consciousness.
FINDINGS Figure 107-1. Axial DWI MRI through the arrows) within the left frontal lobe lesion. There is sur-
frontal lobes. There is a left frontal lobe well-circumscribed rounding confluent hyperintensity consistent with vasogenic
hyperintense mass (arrows) consistent with restricted diffu- edema (vertical arrow) and mass effect on surrounding struc-
sion. A similar but smaller lesion is present inferiorly in the tures manifested by effacement of convexity sulci. There
right frontal lobe (not shown). Figure 107-2. Axial FLAIR is vasogenic edema in the right frontal lobe surrounding
through the same level. There is alternating (six) layers of dif- the second lesion (not shown). Figure 107-3. Axial T2WI
ferent intensity pattern (concentric target pattern) (transverse through the left frontal lobe mass. There is a central high
225

226 Case 107
intensity core (star) with a surrounding multi-layer irregular serum levels of calcineurin inhibitors (tacrolimus, cyclo-
wall; isointense to brain around the core with an outer layer sporine), and cytomegalovirus disease within the preceding
of thin hyperintensity (transverse arrows) before the edema 6 months. Pulmonary disease is the predominant clinical
(vertical arrow). Figure 107-4. Post-contrast coronal T1WI presentation (40%). Nocardia seems to have tropism for
through the left frontal mass. There is a thick irregular mural neural tissue, central nervous system (CNS) being the most
contrast enhancement (arrow). Axial non-contrast T1WI (not common site of infection. Clinical presentation includes
shown) demonstrated a hypointense mass surrounded by a headache, mental status changes, focal neurologic deficits,
thin mildly hyperintense wall and another coat of isointense and seizures. Definitive diagnosis is made by bacteriologic
capsule before the vasogenic edema. culture following surgical evacuation of the abscesses.
Nocardia meningitis is not common and can present with
DIFFERENTIAL DIAGNOSIS Abscess (pyogenic), toxo- or without associated brain abscess. Treatment is usually
plasmosis, aspergillosis, glioblastoma (GB), infarcts. prolonged with a combination of antibiotics including tri-
methoprim/methoxazole as the drug of choice. Prognosis
DIAGNOSIS Nocardiosis brain abscess. depends on the site of involvement, extent of the disease,
and immune status of the host. Cure rate in patients with
DISCUSSION Nocardial brain abscess could be single or brain involvement is approximately 40% to 50%. Prognosis
multiple. MRI best depicts the geography of the lesion. The is worse in the immunocompromised and patients with mul-
hallmark of an abscess is a round area of restricted diffusion tiple abscesses.
with surrounding edema and mass effect. The restricted dif-
fusion is ascribed to the cellularity of the pus. The typical Question for Further Thought
pyogenic abscess has a smooth thin contrast-enhancing ring 1. When should MRI or CT of the brain be repeated after the
surrounding the pus. In this case however, the ring enhance- initial diagnosis?
ment is thick, somewhat crenated, and irregular, and there
is a multilayer wall within and surrounding the center of the Reporting Responsibilities
abscess on FLAIR and T2WI, indicating presumably several An abscess of any type is an emergency requiring direct
layers of different tissues within the mass, hence their dif- reporting. Location, number, and size will determine man-
ferent intensities. There is a large amount of surrounding agement strategy. Complications such as herniations should
vasogenic edema. The irregularity of the enhancing wall be reported. Leptomeningeal enhancement may indicate
may suggest something more sinister such as GB. However, presence of meningitis.
the homogeneous restricted diffusion and the target pattern
are unusual of GB. Aspergillosis on the other hand tends to What the Treating Physician Needs to Know
show heterogeneous DWI and T2 pattern with a variable pat-
Location and size. It is recommended that abscesses
tern of contrast enhancement with intracavitary projections.
smaller than 2 cm in the presence of other extra-CNS
Infarcts are usually confined to a vascular territory with ring
infection could be watched if patient is stable. Abscesses
enhancement being unusual.
larger than 2.5 cm should be excised or drained. Outcome
Clinical presentation of nocardiosis can be acute with
seems better with excision
rapid progression or insidious with subacute or chronic
evolution. Nocardia species, aerobic filamentous branching If a lumbar puncture (LP) is necessary is it safe? Significant
Gram-positive bacteria, are ubiquitous in the environment mass effect or herniation contraindicates LP
causing human infections by direct inoculation of the skin Brain imaging is always recommended in patients with
or by inhalation. The disease is rare in the United States, evidence of systemic Nocardia infection. CNS involve-
affecting both immunocompetent and immunocompro- ment has been reported in up to 44% of cases with sys-
mised patients. The frequency of nocardiosis among solid temic nocardiosis
organ transplant recipients varies from 0% to 20%, and
among allogeneic bone marrow recipients the rate is 0.3% Answer
to 1.7%. Risk factors include preexisting immunocom- 1. The imaging should be repeated every 2 weeks until the
promised status (diabetes, alcoholism, AIDS, solid organ lesion is stable in size and imaging should be repeated
transplantation), chronic pulmonary disorders, graft-versus- if there is clinical deterioration. Drug treatment usually
host disease, administration of high doses of steroids, high continues for at least 1 year.

Case 108 CLINICAL HISTORY Liver transplant patient presenting with a rapidly
progressive encephalopathy and seizures.
227

228 Case 108
FINDINGS Figure 108-1. Axial NCCT through the basal common extranodal involvement. Isolated involvement of
ganglia. There are multiple patchy hypodense areas in the the CNS is rare. According to the WHO revised classification,
white matter (WM) (arrows). Figure 108-2. Axial FLAIR there are four categories: early lesions, polymorphic PTLD,
through the corona radiata. There are large hyperintense areas monomorphic PTLD, and classic Hodgkin lymphoma-type
in bilateral cerebral WM with local mass effect. Figure108-3. PTLD. Clinically patients present with headache, motor
Corresponding axial post-contrast T1WI. There are multiple deficits, ataxia, aphasia, and seizures. Management often
areas of enhancement mostly nodular and ring with surround- requires stopping immunosuppression. Prognosis is very
ing edema (arrows). Figure 108-4. Axial NCCT in a different poor indeed with mortality approaching 90% for those not
patient shows a large right parieto-occipital acute hemor- responding to cessation of immunosuppression.
rhage with an internal fluid level (arrow) as a complication
of intracerebral posttransplant lymphoproliferative disorder Questions for Further Thought
(PTLD). 1. Are there risk factors for PTLD of the CNS?
2. Which is the best diagnostic approach?
DIFFERENTIAL DIAGNOSISAbscess, toxoplasmosis,
and other neoplastic processes (other central nervous sys- Reporting Responsibilities
tem [CNS] lymphomas), posttransplant lymphoproliferative This is an emergency in view of the mimickers requiring
disorder. direct reporting. Consider the possibility of PTLD in immu-
nosuppressed and posttransplanted patients where you sus-
DIAGNOSIS Posttransplant lymphoproliferative disorder pect infectious etiologies.
(PTLD).
DISCUSSION MRI offers the best imaging assessment of
PTLD. The most common neuroimaging findings are multifo- The extent and location of the lesions in order to program
cal disease with predominance for periventricular/basal gan- a biopsy
glia regions, but also meningeal/ependymal and infratentorial
involvement can occur. The pattern is very similar to AIDS- Answers
related lymphoma, usually iso- to hypointense onT1WI and 1. Yes, some factors increase the risk of PTLD such as trans-
heterogeneously hypo- to hyperintense on T2WI with hetero- plantation type (lung and intestine are more commonly
geneous patchy, nodular, or ring enhancement. Surrounding associated), type and intensity of immunosuppression
edema is common resulting in diffuse WM hyperintensity (cyclosporine and tacrolimus), quantity of lymphoid tis-
on FLAIR and T2WI. CT often shows patchy hypodensities sue within the transplanted graft, and EBV infection and/
with similar pattern of contrast enhancement as in MRI. It or exposure.
could be difficult to distinguish from opportunistic infections 2. As imaging and cerebrospinal fluid (CSF) abnormalities
such as tuberculosis (TB) or toxoplasmosis. Biopsy is often are nonspecific, and only a few patients have abnormal
required for definitive diagnosis. cytology and flow cytometry results, biopsy has been
PTLD is a complication of solid organ transplantation or proven useful for a definite diagnosis. There is no optimal
hematopoietic stem cell transplantation, with higher inci- treatment for PTLD of the CNS. Without treatment the
dence in children and a known association with Epstein-Barr outcome is poor but with treatment survival times as long
virus (EBV). Typically, the lesions are B cell in origin, with as 4 years have been reported.

Case
109 CLINICAL HISTORY 21-year-old female with dizziness, ataxia, and
left-sided hearing loss (HL).
Figure 109-3
FINDINGS Figures 109-1 and 109-2. Axial DWI with

corresponding ADC map through the body of corpus callo-
sum (CC). There are multiple lesions in the CC and white
matter (WM) of the corona radiata showing increased dif-
fusion (arrows). Figure 109-3. Sagittal T2 FLAIR. There
are
multiple CC hyperintense lesions without significant Figure 109-4
229

230 Case 109
callosalseptal interface signal changes. The large central central arterioles of the CC probably due to some form of
lesion (arrow) is consistent with the so-called snowball vasculopathy. Diagnosis is usually confirmed by retinal fluo-
lesion. Figure 109-4. Axial T2 FLAIR through the lateral rescein angiographic demonstration of branch retinal artery
ventricle. There are multiple hyperintense lesions in the genu occlusion (BRAO). An aggressive treatment regime is advo-
and splenium of the CC (arrows). cated. This consists mainly of immunosuppressive drugs
with high-dose corticosteroid as the mainstay. Additional
DIFFERENTIAL DIAGNOSISToxic m etabolic therapies such as intravenous immunoglobulin, mycophe-
encephalopathy (medication induced, cocaine, inhaled
nolate mofetil, and cyclophosphamide may be necessary.
opiates, chemoradiation, Marchiafava-Bignami), inflammatory Rituximab is the newest therapy to consider. Disease is,
demyelination (multiple sclerosis [MS], acute dissemi- however, usually self-limiting. Complications may include
nated encephalomyelitis [ADEM], and neuromyelitis optica blindness, dementia, and deafness.
[NMO]), vasculopathic and vasculitic stroke (venous stroke,
systemic lupus erythematosus (SLE), Susac syndrome (SS).
DIAGNOSIS Susac syndrome (SS). 1. Are snowballs, icicles, and spokes pathognomonic of SS?
DISCUSSION The focus of this discussion is on CC Reporting Responsibilities

involvement in SS. CC lesions are often seen in SS and Direct reporting is necessary in view of the differential diag-
always in the encephalopathic form. These lesions are best nosis which may include MS. Most SS patients are usually
visualized on sagittal T2 FLAIR as hyperintense lesions and initially mistaken for MS because of similarities of imaging
sagittal T1 as hypointense lesions. The central portion of the findings. Hence identification of specific CC lesions may
CC is affected, and these are large round T2 hyperintense save the patient from prolonged misdiagnosis.
lesions referred to as snowballs which eventually evolve
into pathognomonic central callosal holes. Central callo-
sal holes occur only in SS, and not in MS or ADEM. The
Specific location and shape (snowball) of lesions in the CC
roof of the callosum is also frequently affected: T2 hyper-
distinguishes SS from other mimics!
intense lesions here assume icicle and spoke configura-
If there is a clinical suspicion of SS or when demyelination
tions. Thecallosalseptal (ventricular or perivenular) surface
or SS is in the differential diagnosis, the request should
where MS plaques arise is infrequently involved, and only
include sagittal T2 FLAIR as it is not routinely performed
when there are also centrally located lesions. Lesions may
and could be crucial to the diagnosis
contrast enhance in the subacute phase. There is usually no
surrounding significant edema. The deep gray matter may
be involved in about 70% and leptomeninges in about 33%. Answer
Presence of leptomeningeal enhancement is crucial in further 1. The authors who first published SS contended that the
differentiating this from MS. diagnosis can be made with certainty in an encephalo-
The clinical diagnosis depends on the triad of sensori- pathic patient when the above pathognomonic corpus cal-
neural HL, visual disturbance, and encephalopathy. Itis losal findings are present, even in the absence of BRAO
more common in women than men and affects all ages. and HL. Furthermore, the findings of these lesions in the
Pathologically, SS lesions are microinfarcts affecting the CC should spur a search for BRAO and HL.

Case
110 CLINICAL HISTORY Newborn female with in utero diagnosis of
holoprosencephaly (HPE).
at formation of anterior interhemispheric fissure (vertical

arrow). Figure 110-3. Axial T1WI through the lateral ven-
tricles. This again confirms the univentricle without septum
pellucidum or corpus callosum. There is a wide posterior
notch possibly interhemispheric fissure (arrow).
DIFFERENTIAL DIAGNOSIS Hydrocephalus, HPE, lissen-

cephaly, hydranencephaly.
DIAGNOSIS HPE with lissencephaly.
DISCUSSION Combined HPE and lissencephaly is a rare

malformation that combines lack of midline brain formation
with migrational abnormality of a smooth cerebral surface
without sulcation. Both entities occurring separately or com-
bined can be adequately imaged by CT or MRI. But MRI
gives better tissue characterization than CT and offers mul-
tiplanar capability to image the midline abnormalities prop-
erly. These images demonstrate the presence of a univentricle
with surrounding horseshoe brain, lack of formation of sep-
tum pellucidum or identifiable corpus callosum, and fusion of
Figure 110-3 the thalami. A rudimentary interhemispheric fissure appears
anteriorly and posteriorly. Four types of HPE are recognized:
alobar, semilobar, lobar, and middle interhemispheric vari-
FINDINGS Figure 110-1. Coronal T2WI through the mid- ant. The pattern of the HPE in this case resembles semilo-
brain. There is a thin horseshoe brain mantle (vertical bar (incomplete) HPE where the hemispheres are variably
arrows) surrounding an enlarged single ventricle (star). There separated, such that dysgenetic falx cerebri and incomplete
is a band of hyperintense white matter (WM) underneath the interhemispheric fissure are present in frontal and occipi-
smooth cortical gray matter (GM) without significant sul- tal regions as manifested by the wide anterior and occipital
cation. The thalami are fused (transverse arrows). Figure notches in this patient. Sometimes, the presentation of HPE
110-2. Axial T2WI through the thalami. There is fusion of does not neatly fall into any of the categories but somewhere
the thalami (star) and the frontal lobes. There is an attempt within the spectrum. It has been suggested that instead of
231

232 Case 110
strict categorization, we should be thinking of a spectral dis- What the Treating Physician Needs to Know
order or a continuum of nonseparation of the forebrain where The pattern and type of the malformation
some patients fall within border zones of the four types. The
smooth brain surface and lack of sulcation is consistent with Answer
lissencephaly. HPE and lissencephaly are very rare congeni- 1. SHH is one of three proteins in the mammalian signal-
tal anomalies, and a combination of both in the same patient ing pathway family called hedgehog, the others are desert
is very rare indeed. hedgehog (DHH) and Indian hedgehog (IHH). The SHH
In utero diagnosis of HPE was made by amniocentesis gene provides instructions for making the SHH protein
with finding of sonic hedgehog gene (SHH gene) mutation which functions as a chemical signal that is essential for
and a positive family history. In utero sonogram showed embryonic development of the central nervous system
the lack of midline formation with microcephaly, hypothe- (CNS), eyes, limbs, and many other parts of the body.
lorism, flattened nose, and low set ears. These changes could SHH plays a role in cell growth, cell specialization, and
be seen as early as 6 weeks of age when the forebrain cleav- the patterning of the body. It is necessary for the develop-
age is supposed to be completed. These patients present with ment of the forebrain just as it is important for the forma-
midline facial deformities that may include a single eye in tion of digits in the limbs. It helps establish the midline
the forehead, hypo- or hypertelorism, single nose, and cleft that separates the right and left halves of the forebrain, the
deformities of the lips and palate. The prognosis is grim. prerequisite for formation of the cerebral hemispheres. It
is also involved in formation and separation of the two
Question for Further Thought eyes. Disordered eye formation prevents other facial
1. What is SHH gene? structures from taking their normal positions with a dom-
ino effect on the nose, palate, and lips. Mutation or aber-
Reporting Responsibilities rations of this protein results in lack of forebrain cleavage
Routine reporting is sufficient unless there is an acute find- and formation of midline structures of the face otherwise
ing. The dilated univentricle may resemble hydrocephalus. known as HPE.

Case
111 CLINICAL HISTORY 45-year-old male with headache and dizziness.
T1WI through the mass. The solid nodule (arrow) demon-

strates intense enhancement, but there is a lack of enhance-
ment of the cyst wall. Figure 111-3. Sagittal post-contrast
T1WI in a companion case demonstrates multiple heman-
gioblastomas (arrows) in the posterior fossa, cervical and
thoracic spine, diagnostic of von Hippel-Lindau syndrome.
DIFFERENTIAL DIAGNOSISHemangioblastoma, pilo-

cytic astrocytoma, glioblastoma, metastasis.
DIAGNOSIS Hemangioblastoma.
DISCUSSION Hemangioblastoma is most commonly

located in the cerebellum, brainstem, or spinal cord.
The majority (90%) arises in the posterior fossa, with
supratentorial lesions usually limited to patients with von
Hippel-Lindau syndrome. The tumor is usually well demar-
cated, but vasogenic edema may be present. The classic
appearance of a cystic mass with mural nodule abutting the
pial surface constitutes about 60% of the cases; as much as
40% of the cases manifest as purely solid tumors. There is usu-
ally very little or no surrounding edema. Hemangioblastoma
is often highly vascular, and hemorrhage may be present.
Prominent feeding arteries and draining veins may be visible
and seen as flow voids on MRI. These vessels can be well
demonstrated on CTA. On angiography, prolonged vascular
blush and arterial-venous shunting are commonly present.
Some tumors may benefit from preoperative embolization.
Figure 111-3 Patient age helps to differentiate hemangioblastoma
from pilocytic astrocytoma, with the former seen primarily
FINDINGS Figure 111-1. Axial T2WI through the poste- in adults and the latter seen in pediatric patients. When the
rior fossa. There is a mixed cystic and solid tumor in the right diagnosis of hemangioblastoma is considered in younger
cerebellar hemisphere. A solid nodule (arrow) abuts the pial and pediatric patients or when multiple tumors are pres-
surface of the cerebellum. Figure 111-2. Axial post-contrast ent, von Hippel-Lindau syndrome needs to be considered.
233

234 Case 111
Glioblastoma usually appears more heterogeneous and Reporting Responsibilities

infiltrative compared to hemangioblastoma. There is usu- Direct reporting is essential for all tumors. Assess the degree
ally substantial vasogenic edema surrounding glioblastoma. of mass effect. It is always necessary to evaluate for vascu-
Although hemangioblastoma is the most common primary larity and hemorrhage and to exclude additional or multiple
posterior fossa tumor in adults, metastasis is far more com- lesions. Additional spine evaluation is necessary to exclude
mon statistically and needs to be excluded. multiple lesions.
Most cases of hemangioblastoma are sporadic and are
seen in adults older than 30 years. However, approximately What the Treating Physician Needs to Know
25% of cases arise in patients with von Hippel-Lindau syn- Are there additional lesions in the neuroaxis which will
drome. Patients with von Hippel-Lindau syndrome often suggest von Hippel-Lindau syndrome and warrant investi-
present at younger age (usually over 15 years) and have mul- gation with abdominal imaging?
tiple tumors. Hemangioblastoma is a WHO I tumor. Surgical Spine evaluation is required to exclude these additional
treatment usually requires en bloc resection, as piecemeal lesions
resection can result in excessive hemorrhage.

1. What is the association between patients with hemangio- 1. Upregulation of erythropoietin may be seen in patients
blastoma and polycythemia? with hemangioblastoma, resulting in polycythemia.

Case
112 CLINICAL HISTORY 43-year-old male with increasing headache and
mental status changes associated with photophobia and phonophobia,
and nausea and vomiting.
Figure 112-2
Figure 112-1
Figure 112-4
FINDINGS Figure 112-1. Axial T2 FLAIR through the

Figure 112-3 trigones. There are multiple serpentine signal voids (arrows)
in the left occipital lobe subarachnoid spaces. Figure 112-
2. Axial T2WI through the lower pons. There are multiple
serpentine signal voids within the subarachnoid spaces
(arrows). Figure 112-3. Axial post-contrast T1WI through
the lower pons. There are multiple contrast-enhancing lep-
tomeningeal vessels in the posterior fossa (arrows). The left
transverse sinus is enlarged and enhanced (vertical arrows).
Figure 112-4. 3D TOF MRA of the head. There are multiple
arterial branches (arrows) from the left occipital artery, left
middle meningeal artery, and a left internal carotid artery
(ICA) tentorial artery feeding a left occipital fistulae in the
left transverse sigmoid junction (double arrows). There are
multiple draining veins. Figure 112-5. Coronal MIP contrast-
enhanced MRV of the head. The left sigmoid sinus and inter-
nal jugular veins are not visualized. The right sigmoid sinus
and internal jugular vein (arrows) and the bilateral transverse
Figure 112-5 sinuses are visualized.
235

236 Case 112
DIFFERENTIAL DIAGNOSIS Dural arteriovenous fistula Clinical presentation of DAVF depends on its location.
(DAVF), leptomeningeal angiomatosis, arteriovenous mal- Posteriorly located DAVF may present with tinnitus, head-
formation (AVM). ache, mental status changes, focal neurologic deficit, cog-
nitive decline, and trigeminal neuralgia. Anteriorly placed
DIAGNOSIS Dural arteriovenous fistula (DAVF). DAVF may present with orbital proptosis and venous con-
gestion, ophthalmoplegia, and retro-orbital pain. DAVF is
DISCUSSION DAVF is a direct dural arterial and venous probably due to cerebral venous sinus thrombosis or outflow
communication without an intervening nidus or brain paren- obstruction with venous hypertension, resulting in patho-
chymal malformation. The vascular components consist of logic rendition of normal physiologic arteriovenous (AV)
arterial feeders usually of external carotid artery (ECA) or shunts or neoangiogenesis with formation of de novo fis-
meningeal origins and venous components that could be tulae. Endovascular treatment, radiosurgery, and surgical
venous sinus, meningeal, or cortical/leptomeningeal in loca- options are available.
tion depending on whether the DAVF is benign or malignant.
Imaging findings depend on which of these vascular compo- Question for Further Thought
nents and the parenchymal complications that are present. 1. What is the basis of clinical classification of DAVF?
MRI offers the most comprehensive method of evaluation
of DAVF by depicting not only the vascular components Reporting Responsibilities
but also the parenchymal and extraaxial complications. Direct reporting is essential in DAVF. Presence of paren-
Presence of too many cortical or dural signal voids or ves- chymal, subarachnoid, and occasionally intraventricular
sels particularly around the sinuses or within the subarach- hemorrhages should be acknowledged. Parenchymal edema,
noid spaces on FLAIR or T2WI should raise the suspicion leptomeningeal vascular engorgement, and transmedul-
of DAVF. Malignant DAVF could be complicated by paren- lary prominent veins are indicative of venous hypertension
chymal or subarachnoid hemorrhages, venous hypertension requiring prompt management. It is necessary to search for
manifested by cortical/leptomeningeal venous drainage, occluded venous sinuses.
engorged transmedullary veins, and other changes of raised
intracranial pressure such as parenchymal edema, perioptic What the Treating Physician Needs to Know
cerebrospinal fluid (CSF) space enlargement, and/or superior Location of fistula, arterial feeders, draining veins, and
ophthalmic veins enlargement. Multiple contrast-enhancing parenchymal or subarachnoid changes
large dural and leptomeningeal arteries and cortical veins Venous sinus occlusions
are present. It may be difficult to see small low-flow DAVF. Complete delineation may require DSA
Thrombosis of cortical veins or venous sinuses is associated
with DAVF. CTA and MRA and particularly time-resolved Answer
MRA are capable of demonstrating the vascular components. 1. The more widely used classification is the Boyden clas-
Venous sinus occlusions and/or enlargement are demon- sification, which divides DAVF into benign or malignant
strable by MRV. These changes are more common around depending on the venous drainage pattern that determines
the cavernous sinuses, transverse/sigmoid junction, and the severity of the disease. Boyden I is benign with dural
the convexities. DSA is, however, the definitive method of sinus drainage and no reflux into the cortical veins.
comprehensive characterization of DAVF. A parenchymal/ Boyden II is malignant with drainage into the sinuses with
cortical nidus or bag of worms best seen on FLAIR and cortical venous reflux. Boyden III is also malignant with
T2WI is usually present in leptomeningeal angiomatosis direct drainage into cortical veins. The Cognard classifi-
and AVM. cation is more complex and has seven types.

Case
113 CLINICAL HISTORY 1-year-old child with nystagmus and seizures.
237

238 Case 113
FINDINGS Figure 113-1. Axial T2WI through the basal and presentation and include the classic, connatal (most
ganglia. There is diffusely hyperintense white matter (WM) severe), transitional, and adult forms. The category and
(stars), indicating lack of mature myelin at 1 year of age. severity of the disease is related to the abnormality in the
Figure 113-2. Corresponding axial T1WI in the same patient. PLP gene. There is heterogeneity with a broad spectrum in
There is normal hyperintense myelin only at the posterior the clinical presentation of the disease. The most common
limbs of the internal capsules and along the optic radiations presentations include nystagmus, ataxia, seizures, spastic-
(arrows). Figures 113-3 and 113-4. Axial T2WI with cor- ity, and involuntary movements. Clinically, PMD is often
responding T1WI through the basal ganglia, respectively, misdiagnosed as cerebral palsy because the classic PMD
in a companion case. There is diffuse hyperintense myelin is characterized by childhood-onset spastic paraplegia,
on T2WI (stars) and lack of normal hyperintense myelin on mild cognitive impairment, ataxia, athetosis, and periph-
T1WI throughout the brain. eral neuropathy. There is no treatment and management is
supportive.
DIFFERENTIAL DIAGNOSISPelizaeus-Merzbacher dis
ease (PMD), metachromatic leukodystrophy, Lowe syndrome, Questions for Further Thought
Salla disease, trichothiodystrophy. 1. What causes the tigroid myelination pattern?
2. What is the difference between demyelinating and dys-
DIAGNOSIS PMD. myelinating disorders?
DISCUSSION PMD is a rare X-linked dysmyelinating Reporting Responsibilities

disorder with clinical and molecular heterogeneity linked to Routine reporting is sufficient in this chronic disease.
deletion, mutations, or duplication of the proteolipid protein Describe the abnormalities and their extent. Suggest the
(PLP1) gene locus at Xq22. MRI typically reveals diffuse presence of a metabolic disorder so that appropriate labo-
T2 hyperintensity throughout the WM both infra- and supra- ratory tests can be ordered. Suggest further evaluation with
tentorially, extending to involve the subcortical U fibers, MR spectroscopy in an attempt to elucidate the metabolic
consistent with abnormal myelination. The tigroid myelina- defect and better map the extent of the disease.
tion pattern can sometimes be seen in mild cases. Atrophy
and decreased WM volume may also occur. Diffuse or focal What the Treating Physician Needs to Know
decrease in N-acetylaspartic acid (NAA) peak consistent with
Extent and pattern of WM disease
neuronal damage, and mildly elevated choline and creatine
peaks suggestive of astrocytic changes have been observed Relevant differential diagnosis including dysmyelinating
in PMD. CT reveals diffuse hypodensity of the WM. Diffuse and demyelinating disorders
WM dysmyelination is common in trichothiodystrophy, a
genetic disorder of sulfur deficiency that usually presents Answers
with brittle hair. Salla disease is a very rare genetic free sialic 1. The tigroid pattern is common to a host of WM diseases
acid storage disorder with supratentorial WM lesions with- and mostly occurs in the mild form of PMD. Islands of
out cerebellar WM involvement. The volume loss, however, normal myelin in the setting of diffuse dysmyelination or
tends to be global with involvement of the cerebellum. demyelination contribute to the tigroid pattern.
The PLP is the most abundant protein in the central 2. Demyelinating disorders cause destruction of normally
nervous system (CNS) myelin. There are four differ- formed myelin. Dysmyelinating disorders are the result
ent categories of PMD which differ in disease severity of abnormal myelin formation.

Case
114 CLINICAL HISTORY 3-year-old male with vertigo.
FINDINGS Figure 114-1. Axial post-contrast T1WI through CCM, there may be hemorrhagic product from the CCM. In
the pons. There is a branching tubular structure emanating isolation, DVAs rarely hemorrhage, but it does occur, par-
from the 4th ventricular region crossing the right middle cer- ticularly if the large draining vein thromboses.
ebellar peduncle (arrow). The surrounding brain is normal in
appearance. Figure 114-2. 3D volume-rendered MRV, view- Question for Further Thought
ing the posterior fossa from a bottom-left vantage, shows the 1. How are DVAs treated?
full extent of the lesion, as it originates in the right cerebellum
(black arrow), coursing medially and superiorly to join the
deep venous system at the vein of Galen (white arrow).
Routine reporting is sufficient. When large, DVAs should
certainly be described, and an associated CCM or other vas-
DIFFERENTIAL DIAGNOSIS Developmental venous anom
cular malformation should be sought. Small DVAs, how-
aly (DVA), arteriovenous malformation (AVM), capillary tel-
ever, are very common and warrant no special treatment or
angiectasia.
follow-up. If mentioned at all, the presence of such lesions
may be safely buried in the body of the report.
DIAGNOSIS DVA.
DISCUSSION DVA is a congenital anomalous venous
structure which is able to function normally as a vein and DVAs are extremely common anomalous structures
which drains normal brain parenchyma. These are very com- which, even when large, are almost always of no clinical
mon incidental lesions. They are almost always asymptom- significance
atic and of no clinical consequence. The classic imaging They require no special imaging or follow-up unless the
appearance is that of a network of small enhancing feed- morphology is unusual and diagnosis is uncertain
ing vessels which converge onto a larger draining vessel,
and from there, to a deep cerebral vein or dural sinus. The Answer
morphology is likened to the Medusa-head. DVAs may 1. Asymptomatic DVAs do not require treatment. They are
be occult on pre-contrast CT and MRI, but they are usually anomalous veins which function normally to drain nor-
quite evident on post-contrast images. They may be asso- mal brain parenchyma. To remove them would induce
ciated with other vascular anomalies, including cerebral venous ischemia and even infarction within the drained
cavernous malformations (CCMs). When associated with a territory.
239

Case
115 CLINICAL HISTORY 25-year-old male with seizure activity on Sunday
morning following a Saturday night out of heavy drinking.
FINDINGS Figures 115-1 and 115-2.Axial contiguous (arrow in Figure115-2). Figure 115-3. Axial DWI through
NCCT through the level of the corona radiata. There is a the mass. There are three areas of increased diffusion within
cortical-based suprasylvian right frontal lobe mass with a the mass. Figure 115-4. Axial T2 FLAIR. The mass is well
hyperdense lateral component and a hypodense medial com- circumscribed and hyperintense with a small area of profound
ponent. There is scalloping of the adjacent inner table (arrow hypointensity anterolaterally (arrow). There is no surround-
in Figure 115-1) and a medial peripheral focal calcification ing edema. Figure 115-5. Axial GRE. There is an area of
240

Case 115 241
blooming anterolaterally corresponding to the hypointensity of the perfusion and diffusion data. PXA can be nonenhanc-
on FLAIR and hyperdensity on CT most probably a focus of ing and may not have elevated perfusion. The diffusion and
hemorrhage or calcification (vertical arrow). The scalloping perfusion data for PXA are very limited. Differential diag-
of the inner table is again demonstrated (transverse arrow). nosis usually includes other cortical- or dural-based tumors
Figure 115-6. Coronal post-contrast T1WI through the mass. such as ganglioglioma, oligodendroglioma, DNET, lobar
There is an inferolateral nodular contrast enhancement with PA, and meningioma. A cystic component is unusual in a
an inferomedial rim enhancement (arrow). There is remodel- meningioma which generally occurs in the older population.
ing of the calvarium lateral to the mass. Meningeal enhancement and bone remodeling are uncom-
mon in the other differentials. DNET generally shows a
DIFFERENTIAL DIAGNOSISGanglioglioma, pilocytic bubbly pattern.
astrocytomas (PAs), dysembryoplastic neuroepithelial tumor PXA is a rare WHO II neoplasm constituting less than 1%
(DNET), meningioma, oligodendroglioma, astrocytoma, of all astrocytic tumors. It usually occurs in the second and
pleomorphic xanthoastrocytoma (PXA). third decades of life but could be seen in younger population
and in older adults. PXA occurs approximately equally in
DIAGNOSIS PXA. men and women. It is a slow-growing tumor with the patients
presenting with a long history of seizures or symptoms of
DISCUSSION PXA is usually a cortical-based supratento- raised intracranial pressure (such as headaches or nausea and
rial well-circumscribed solid/cystic lesion with variable sur- vomiting). PXA usually demonstrates a superficial compact
rounding edema abutting the meninges and scalloping the component and an infiltrating component resembling diffuse
adjacent calvarium. Deep-seated PXA does occur. PXA is astrocytoma. The typical histologic
features include cel-
more common in the temporal lobes but does occur in the lular pleomorphism, xanthomatous change (vacuolization
frontal and parietal lobes. Posterior fossa and spinal PXA are of tumor cells), thick sclerotic vessels, eosinophilic granu-
rare. The solid part or mural nodule is hypodense to isodense lar bodies (EGB), perivascular lymphocytes, and abundant
on CT with hypointensity to isointensity on T1WI and mild reticulin. Mitoses are usually absent or rare if detected at all.
hyperintensity on T2WI/FLAIR. The cystic component It has a favorable prognosis following gross total resection.
shows cerebrospinal fluid (CSF) intensity on MRI and CSF
density on CT. It may contain calcification which is hyper- Question for Further Thought
dense on CT in over a third of the cases. It may also contain
1. What is the preferred treatment and how successful is
areas of hemorrhage which is hyperdense on CT and hypoin-
treatment?
tense on T2WI/FLAIR and GRE. There is heterogeneous
contrast enhancement with a nodular solid portion and the
cystic portion showing rim or ring enhancement. Meningeal Reporting Responsibilities
enhancement and the presence of a dural tail are not uncom- Like any other tumor, PXA deserves direct reporting. PXAs
mon. Calcification or hemorrhage in PXA obscures analysis can present with hydrocephalus and mass effect, and these

242 Case 115
factors should be immediately communicated. Location is Answer

important for treatment purposes. 1. Surgical resection results in a high cure rate of 70% to
80% at 5 years. These tumors do have a higher recurrence
What the Treating Physician Needs to Know rate and higher transformation to anaplastic grade than
Location and size. These will affect surgical planning other low-grade astrocytomas. Acting swiftly likely helps
Presence of CSF obstruction particularly by deep-seated to avoid anaplastic transformation.
tumor

Case
116 CLINICAL HISTORY 40-year-old female with right-sided weakness.
FINDINGS Figure 116-1. Axial T2WI through the suprasel- DIFFERENTIAL DIAGNOSIS N/A.
lar cistern. There is extensive a network of serpentine signal
voids within the suprasellar and perimesencephalic cisterns DIAGNOSIS Moyamoya disease (MMD).
(arrows) consistent with multiple collaterals. The anterior
cerebral artery (ACA) and middle cerebral artery (MCA) are DISCUSSION The classical finding in MMD is the bilat-
not visualized. Figures 116-2 and 116-3. Axial and coronal eral supraclinoid ICA occlusion with preservation of the
post-contrast T1WI through the lateral ventricles, respec- posterior circulation. To compensate for the occlusions,
tively. There is extensive network of contrast enhancement there is development of a rich network of collaterals at the
within the subarachnoid spaces (transverse arrows) and trans- base of the brain, within the basal ganglia and thalami, in
medullary (vertical arrows). Figure 116-4. 3D TOF MRA. the subarachnoid spaces and transmedullary within the cere-
There is bilateral supraclinoid internal carotid artery (ICA) bral WM seen as multiple serpentine network of vessels,
occlusion at the level of the ophthalmic arteries (arrow). the leptomeningeal enhancement, and transmedullary streaky
ACA and MCA are not seen bilaterally. There is preservation contrast enhancement on CT and MRI as demonstrated in
of the posterior circulation. this case. Prominent sulcal linear hyperintensity on FLAIR
243

244 Case 116
which represents leptomeningeal collaterals is known as seen in association with moyamoya is the morning glory
ivy sign. The classical DSA findings in MMD are those of disk, an enlargement of the optic disk with concomitant
intracranial ICA occlusions with prominent collaterals at the retinovascular anomalies. Six grades of MMD have been
base of brain likened to something hazy, like a puff of ciga- recognized. There is no known cure, but revascularization
rette smoke, which, in Japanese, translates into moyamoya. surgery has been promising.
Transdural collaterals from extracranial circulation are usu-
ally present. The posterior circulation is usually intact. MRA Question for Further Thought
and CTA demonstrate the occlusion of the ICA but may not 1. What is moyamoya syndrome?
demonstrate the puff of smoke as reliably as DSA. The
occlusion or in some cases severe stenosis could be uni-
lateral with eventual bilaterality of lesions in most cases.
Direct reporting is necessary in view of the vascular occlu-
Complications include subarachnoid and parenchymal cere-
sions. Complications when present should be categorized.
bral hemorrhage (hyperdense on CT) due to rupture of the
fragile collaterals. Ischemic infarcts, hypodense parenchy-
mal lesions on CT, and hyperintense DWI and FLAIR/T2WI What the Treating Physician Needs to Know
lesions on MRI are also common due to the arterial occlu- The pattern of intracranial arterial occlusions
sions. Aneurysms are also common. Presence or otherwise of subarachnoid and parenchymal
There are two peaks for the occurrence of MMD at hemorrhages
age of 5 years and in the mid-40s. It is more common
in women than men. MMD is more common in Asian Answer
population but has been described in people of all races. 1. Patients with supraclinoid ICA occlusion and associated
Down syndrome, sickle cell disease, and cranial radia- risk factors (Down syndrome, sickle cell disease, and
tion particularly for pituitary and parapituitary lesions cranial radiation) are said to have moyamoya syndrome
present special risk factors. Headache, transient ischemic while those without associated risk factors are regarded as
attack (TIA), seizures, movement disorders, mental status having moyamoya disease. Those with unilateral findings
changes, and focal neurologic deficits are some of its pre- have the moyamoya syndrome, even if they do not have
senting features. An ophthalmologic finding occasionally other associated risk factors.

Case
117 CLINICAL HISTORY 59-year-old female with altered mental status
and history of recent brain bleed.
FINDINGS Figure 117-1. NCCT through the centrum semi-

ovale. There are bilateral almost symmetrical extraaxial cres-
centic hypodense collections with effacement of the bilateral
convexity sulci consistent with chronic bilateral subdural
hematomas (SDHs). But there is a not so subtle fluid-fluid
level posteriorly within the SDHs (arrows). Figure 117-2.
NCCT through the same level about 6 weeks later. There is
a mildly hyperdense material mostly posteriorly within the
bilateral SDH mixed in on the left and lineal/wavy on the right
(arrows) indicating interval rebleed. The left SDH is larger
with mild effacement of the right SDH. Figure 117-3. NCCT
a month later. Further diffuse iso/hyperdensity of the left SDH
(arrow) which has increased in size with almost complete
effacement of the right SDH. The pattern is very suggestive
of a subacute SDH in view of its isodensity with the brain.
DIAGNOSIS Acute on chronic subdural hematoma (SDH).
DISCUSSION The initial CT scan showed a small fluid-

fluid level (hematocrit effect) within the bilateral hypodense
SDHs. The history was clear about a prior brain bleed. A
chronic SDH is an encapsulated extraaxial collection usu-
ally hypodense sometime reaching CSF density on CT due
Figure 117-3 to degradation of the blood. It could remain crescentic in
245

246 Case 117
shape but biconvex septated chronic SDH is not uncommon. Question for Further Thought
The membrane becomes thickened, hyperdense on CT, and 1. What are the other imaging findings in chronic SDH?
vascularized showing contrast enhancement. The new hem-
orrhage or re-bleed is usually hyperdense on CT. It may layer Reporting Responsibilities
on the membrane or the inner table, could be nodular in con- Any fresh hemorrhage is an emergency and should be
figuration, or create a hematocrit effect. The isodensity of the promptly reported to the referring physician. The size and
SDH in Figure 117-3 is reminiscent of a subacute SDH. The any growth of the SDH in comparison with prior study
overall pattern here suggests a fresh bleed into a hypodense should be documented. Herniations if present should be
chronic SDH. This occurs in up to 1 in 10 chronic SDH. communicated.
Follow-up over several months showed increasing size of the
left SDH and hyperdensity of the bilateral SDH consistent What the Treating Physician Needs to Know
with ongoing bleeding. The initial CT showed balanced
Location and size
bilateral collections with effacement of the cerebral sulci
Complications particularly herniation and subcortical
and no significant midline shift or transtentorial herniation.
edema
Subsequently, the left SDH enlarged and effaced the right
Growth or retraction of the SDH on follow-up study
SDH. Over time the bilateral SDH resorbed without surgery.
Acute on chronic SDH is more common in the elderly.
The underlying reason for this SDH behavior was not dis- Answer
covered in this patient. Reasons for a rebleed might include 1. CT or MRI may show loculation and multiple septations
rupture of the neovasculature in the subdural membrane, in the chronic SDH. The content may attain cerebrospinal
repeat head injury, coagulopathy from any cause, underly- fluid (CSF) density on CT or intensity on MRI. High pro-
ing meningeal neoplastic involvement, and shunt operations tein containing collection could be hyperintense on T1WI.
with unstable intracranial pressure. MRI could be useful in Multiple calcified subdural plaques are not uncommon
better defining the integrity of the dura particularly looking and these may cause mass effect on the cerebral hemi-
for dural pathology. If the clinical situation is stable, the spheres. Pachymeningeal contrast enhancement on both
SDH may be closely monitored and as happened in this case CT and MRI may be present.
may eventually resolve. Presence of significant herniation
may be an indication for surgery.

Case
118 CLINICAL HISTORY 57-year-old male presented 5 days after an injury to
his left hand with persistent pain and erythema of the hand and newly devel-
oped fever, generalized weakness, and progressive mental status changes.
Figure 118-1
Figure 118-2

247

248 Case 118
Figure 118-5
Figure 118-6
hemorrhages were present. Figure 118-5. Axial FLAIR

through the centrum semiovale at 3 weeks follow-up. There
is progression of a right frontal lobe and a left parietal lobe
cortical/subcortical hyperintensities (arrows) consistent
with infarcts. Figures 118-6 and 118-7. Axial and right para-
sagittal post-contrast T1WI, respectively. There are multi-
focal thin ring-enhancing lesions consistent with multifocal
abscesses. There are daughter abscesses in the right frontal
(transverse arrow in Figure 118-7) and left parietal lobes
(vertical arrow in Figure 118-6). There is mild surrounding
edema. Mild leptomeningeal enhancement is present in the
right perisylvian region (transverse arrow in Figure 118-6).
Imaging of the lumbar spine (not shown) demonstrated L2-3
discitis with paravertebral, epidural, and paraspinous soft
tissue changes.
Figure 118-7
DIFFERENTIAL DIAGNOSISEmbolic infarcts, multifo-
cal abscesses, septic emboli (SE), metastases.
FINDINGS Figures 118-1 to 118-3. Representative axial
DWI through various levels of the brain. Figure 118-1 shows DIAGNOSIS Septic emboli (SE) with multiple abscesses.
multifocal small right cerebellar restricted diffusion (arrow).
Figure 118-2 shows a right peritrigonal (vertical arrow) and DISCUSSION SE to the brain can present in one of two
a left parieto-occipital junction (transverse arrow) areas of major ways. Single or multifocal small infarcts in white
restricted diffusion. Figure 118-3 shows a small right frontal gray matter junctions or in end arteries of the deep gray
cortical/subcortical restricted diffusion (arrow). Figure 118- matter, brainstem, or cerebellum. These infarcts are diffu-
4. Axial FLAIR through trigone shows a left parieto-occip- sion restricting and T2 hyperintense. Over time, they evolve
ital junction T2 hyperintensity corresponding to the lesion into abscesses that can enlarge and remain hyperintense on
in Figure 118-2. There are many more lesions on DWI and DWI with low ADC and typical thin ring enhancement with
FLAIR and T2WI than presented here. There was no sig- surrounding vasogenic edema. There may be hemorrhagic
nificant contrast enhancement at presentation, but punctate components. SE can also present with mycotic aneurysms

Case 118 249
(MAs) in peripheral locationswhitegray matter junction and MA. Hewas treated with vancomycin. There were mul-
which could be hyperdense on CT and avidly contrast enhance tiple infarcts/abscesses in other organs in this patient.
with surrounding edema. MA rupture produces parenchyma
hematoma or subarachnoid and/or intraventricular hemor- Question for Further Thought
rhage depending on its location. CTA more frequently than 1. What is the imaging test of choice for the evaluation of
MRA is useful in confirming the peripheral aneurysms. The the patient suspected of SE?
multifocal infarcts of SE may not be distinguishable from
other multifocal infarcts. The ring enhancement may suggest Reporting Responsibilities
multiple metastases. Large amount of edema and significant Infarcts and abscesses are emergent problems that should
irregularity of the ring enhancement are in favor of metas- be reported directly. Complications such as meningitis, ven-
tases. Metastases, however, rarely restrict diffusion while triculitis, hemorrhage, and hydrocephalus or brain hernia-
infarcts and abscesses do. MRS usually shows elevated lip- tions should be documented. Multifocal infarcts with hints
ids, lactate, and amino acids in abscesses. of hemorrhage should suggest SE.
SE to the brain are hematogenous in origin and the abscesses
tend to be multiple and multiloculated. Echocardiogram in
this patient showed 8 mm 6 mm mobile echogenic mass
attached to the noncoronary cusp of the aortic valve consis- Location and number of lesions
tent with vegetation (infective endocarditis). Serial blood Initial images may not show typical abscess or MA
cultures were positive for Staphylococcus aureus (MRSA). Presence of complications
Infective endocarditis is complicated by septic embolization Lesion could and does progress despite effective antibiotic
in 20% to 50% of cases, with up to 65% of embolic events treatment
involving the central nervous system (CNS). The majority CT could be used to guide abscess aspiration
(90%) of CNS emboli lodge in the distribution of the mid-
dle cerebral artery. The highest risk of embolization is with Answer
mitral valve vegetation, staphylococcus infection, during the 1. MRI is more sensitive than CT scan. MRI can detect early
first 2 to 3 weeks of antibiotic therapy, and if the vegetation changes of infarcts, cerebritis, brain edema, and ven-
increases in size during therapy. Neurologic presentation triculitis and can better visualize small satellite lesions.
of SE includes headache, mental status changes, ischemic However, stereotactic aspiration of the abscesses is usu-
stroke, intracerebral hemorrhage, meningitis, brain abscess, ally CT guided.

Case
119 CLINICAL HISTORY Newborn presenting with hypotonia, seizures, and
a high forehead.
FINDINGS Figure 119-1. Axial T2WI through the basal frontal and temporal regions and particularly the Sylvian fis-
ganglia. There is lack of clear visualization of the internal sures may be prominent, extending too far superiorly and
capsules and possible fusion of all of the deep gray matter medially, and lined by thickened dysplastic cortex. The cor-
(GM) nuclei (stars). There is diffuse hyperintensity through- pus callosum may be dysplastic.
out the white matter (WM). Figure 119-2. Axial T2WI Zellweger syndrome (aka cerebrohepatorenal syndrome
through the corona radiata superiorly to Figure 119-1. There of Zellweger) is an autosomal recessive disease that causes
is dysplastic cortex (arrow) outlining both Sylvian fissures. complete absence of peroxisomes leading to multiple meta-
Extensive WM hyperintensity is present. (Case courtesy of bolic disturbances. Affected individuals typically present
Z. Patay, Memphis, TN.) shortly after birth with dysmorphic facies (high forehead,
hypoplastic supraorbital ridges, epicanthal folds, midface
DIFFERENTIAL DIAGNOSISZellweger syndrome, bilat hypoplasia, and large fontanels) and hypotonia. Associated
eral perisylvian syndrome, congenital cytomegalovirus (CMV), abnormalities include glaucoma, cataracts, retinal pigment
infantile Refsum disease. degeneration, hepatomegaly, renal cortical cysts, and stip-
pled chondral calcifications (chondrodysplasia punctata).
DIAGNOSIS Zellweger syndrome. The prognosis is poor with most affected individuals expir-
ing within 3 months.
DISCUSSION Like all WM diseases, MRI is the exami-
nation of choice for the evaluation of Zellweger syndrome. Questions for Further Thought
Axial T1WI and T2WI show hypointensity and hyperinten- 1. What are the expected ages for complete WM myelina-
sity, respectively, in the WM of the cerebral hemispheres tion on MRI?
compatible with hypomyelination or lack of normal myelin- 2. Briefly, what is the normal myelination pattern of the
ation. Additionally, the internal capsules are not well seen developing brain?
and the lentiform nuclei may appear fused with the thalami
or caudate nuclei heads. This represents a persistence of the Reporting Responsibilities
primitive basal ganglionic eminence. Germinolytic cysts Direct reporting may be essential in view of the rapidly fatal
may be present at the caudothalamic notch region (not seen outcome. Describe the abnormalities and their extent. The
in this patient). Polymicrogyria may be visible in the anterior combination of germinolytic cysts and cortical dysplasias in
250

Case 119 251
the Sylvian regions should suggest the diagnosis. Advise fur- Answers
ther evaluation with imaging of the abdomen to evaluate the 1. Myelination completes at 1 year of age on T1WI and at
liver and kidneys. 2 years of age on T2WI. However, the periatrial WM
also known as the terminal zones of myelination may
What the Treating Physician Needs to Know remain immature until 20 years of age and occasionally
Relevant differential diagnosis never finish myelinating.
Associated findings elsewhere in the body and which is the 2. Myelination typically progresses from inferior to supe-
best way to image them rior, medial to lateral, and posterior to anterior.

Case
120 CLINICAL HISTORY 38-year-old male with a family history of Huntington dis-
ease presenting with choreiform movements which have progressively gotten worse.
the frontal horns of the lateral ventricles (arrows). There is

no contrast enhancement.
DIFFERENTIAL DIAGNOSISHuntington disease, neu-

roacanthocytosis syndromes (McLeod, chorea-acanthocyto-
sis), frontotemporal dementia, and Alzheimer disease.
DIAGNOSIS Huntington disease.
DISCUSSION MRI shows characteristic atrophy involving

the head of the caudate nucleus and the putamen. Caudate
atrophy results in the loss of the bulge of the inferior lat-
eral borders of the frontal horns with enlargement of frontal
horns of the lateral ventricles and flattening of their lateral
contour giving them a box-like configuration. The brain
is diffusely atrophic with white matter (WM) volume loss
affecting both frontal lobes. DWI reveals increased ADC
in the caudate nucleus, putamen, and WM which correlates
with disease severity. Signal intensity abnormalities consist-
ing of slight T2 hyperintensity in the atrophic neostriatum
Figure 120-3 correlates with greater motor and cognitive impairments and
usually seen in the juvenile form. Proton MRS shows low
levels of N-acetyl aspartate (NAA) and creatine and pres-
FINDINGS Figure 120-1. Axial T2WI through the caudate ence of lactate peak in the basal ganglia as well as in the
nucleus. There is outward bowing of the lateral walls of the cortex of symptomatic patients. PET imaging shows abnor-
frontal horns with nonvisualization of the heads of the cau- mal neostriatal hypometabolism, especially in the caudate
date nuclei (arrows). There is hyperintensity of the putamina nuclei. Caudate atrophy is characteristic of neuroacantho-
(vertical arrows). Figure 120-2. Corresponding FLAIR image cytosis syndromes (McLeod, chorea-acanthocytosis) as in
shows similar findings (arrows). Figure 120-3. Coronal Huntington disease. However, mild and generalized cortical
post-contrast T1WI shows the box-like configuration of and cerebellar atrophy and increased signal intensity lesions
252

Case 120 253
in the cerebral hemispheric WM on MRI are also common. Questions for Further Thought
Frontotemporal dementia and Alzheimer disease in later 1. Which are the potential imaging biomarkers for detection
stages show predominance of brain atrophy, but atrophy in of Huntington disease?
Huntington disease occurs in younger patients and affects 2. How can inheritance influence the severity and prognosis
predominantly the caudate nuclei. of the disease?
Huntington disease is an autosomal dominant neurode-
generative disorder caused by a CAG trinucleotide repeat Reporting Responsibilities
expansion that lengthens a glutamine segment in the novel This is a chronic disease, and routine reporting is in order.
Huntington protein. The gene responsible for Huntington Identifying the typical pattern of the disease in an adequate
disease has been mapped to chromosome 4p16:3. The muta- clinical context helps to rule out other frequent causes of
tion leads to a selective neuronal loss, astrogliosis, and gross atrophy which can involve the striatum.
atrophy affecting the neostriatum, particularly the paraven-
tricular portion of the caudate and putamen. Marked neuro-
nal loss can also be seen in deep layers of the cerebral cortex,
globus pallidus, thalamus, subthalamic nucleus, substan- No single imaging technique is sufficient for diagnosis of
tia nigra, and cerebellum, which may demonstrate varying Huntington disease, imaging findings are nonspecific and
degrees of atrophy depending on the pathologic grade. overlap with other conditions including aging; hence, the
Clinically, Huntington disease manifests with a triad of diagnosis must be confirmed by genetic tests
abnormal movement, emotional problems, and cognitive Imaging plays a complementary role and is especially
abnormalities which begin insidiously and progress over valuable for monitoring the disease progression
many years until the death of the individual. Movement
abnormalities include disturbances of both involuntary and Answers
voluntary movements. Choreathetosis is an early sign pres- 1. Potential neuroimaging biomarkers for detection of early
ent in over 90% of individuals. With advancing disease the neuronal dysfunction include MRS. Putaminal NAA and
chorea is superseded by bradykinesia, rigidity, and dys- myoinositol are promising potential biomarkers of dis-
tonia. There is global and progressive decline in cognitive ease progression with low NAA and increased myoino-
capacity leading to dementia. Depression with suicide risk sitol levels seen in presymptomatic and early Huntington
is the most common psychiatric symptoms. Individuals also disease. Functional MRI has proven to be sensitive to
develop significant personality changes, affective psychosis, changes in striatal function long before the emergence of
or schizophrenic psychosis. Onset of symptoms is between motor symptoms showing decreased caudate activity and
35 and 45 years of age resulting in death after 10 to 20 years. a compensatory increase in activity of the supplementary
The juvenile form onset occurs in the first to second decades motor area and anterior cingulate during performance of a
of life with survival of less than 15 years. This form accounts cognitive task.
for 5% to 10% of Huntington cases, and its clinical presenta- 2. Inheritance via the father can lead to earlier onset through
tion differs from the adult disease. There is severe mental succeeding generations, a phenomenon termed anticipa-
deterioration, prominent motor and cerebellar symptoms, tion, and as a result most patients with juvenile Huntington
speech and language delay, and a rapid decline. disease inherit the disease from their fathers.

Case
121 CLINICAL HISTORY 58-year-old male with stroke status post rtPA.
Figure 121-4
shown) consistent with acute/subacute ischemic infarct in the

right middle cerebral artery (MCA) inferior division terri-
tory. Figure 121-2. Corresponding axial FLAIR. There is a
smudgy hyperintensity in the right MCA territory posteriorly
Figure 121-3 (arrow). Figure 121-3. Axial FLAIR through the cavernous
sinuses. The usual signal void in the right internal carotid
artery (ICA) is absent replaced by isointensity (arrow) con-
FINDINGS Figure 121-1. Axial DWI through the lateral sistent with occlusion of the right ICA. Figure 121-4. Axial
ventricles. There is a large cortical subcortical posterior right proton density (PD) fat sat through the skull base. There is
frontal hyperintensity (arrow), which extends into the right enlargement of and hyperintensity in the right ICA (arrow)
parietal lobe on other slices. This region has low ADC (not consistent with mural or intraluminal clot. Figure 121-5. 3D
254

Case 121 255
Figure 121-5
TOF MRA of the head. The right ICA is not visualized. The
right anterior cerebral artery and proximal middle cerebral
artery (MCA) (arrows) opacify via collateral opacification
of the supraclinoid ICA. Distal right MCA branches are not
visualized. Figure 121-6. Contrast-enhanced MRA of the
neck. There is tapered occlusion of the right ICA proximally
(arrow).
Figure 121-6
DIAGNOSIS Right MCA territory infarct due to right ICA The specific advantages of CTA are the visualization of
dissection. atherosclerotic calcifications, identification of more intimal
flaps, high-grade stenosis, and soft tissue changes. Treatment
DISCUSSION Acute infarct mostly present as area of depends on prevailing circumstances and includes anticoagu-
restricted diffusion on DWI; there is hyperintensity in the lation, angioplasty, or stenting. There is very little surgical
affected territory with corresponding low ADC. There are role in the management of dissection.
several potential mechanisms for an infarct, which may
include atherothrombosis, embolic phenomenon, dissection, Question for Further Thought
vasculopathy/vasculitis, and low flow states. How the patient
1. What are the causes of arterial and specifically ICA
is managed depends on the cause of the stroke. MRA of the
dissection?
head and neck are usually necessary to unravel the cause
or causes of infarcts. In this case, the MRA shows tapered
occlusion of the right ICA just beyond the origin, a feature
Direct reporting is required in acute ischemic changes. MR
that is most consistent with dissection. Angiographic demon-
stroke protocol should include MRA of the head and neck for
stration of ICA dissection may include focal stenosis, string-
evaluation of causes of the stroke.
of-beads pattern, tapered or stump occlusion, and presence of
intimal flap or double lumen. Aneurysms either fusiform or
pseudoaneurysm is present in 5% to 13%. Mural or luminal
hyperintensity particularly on T2WI or PD fat sat sequence Location and extent of the infarct, presence of hemor-
consistent with hematoma is present in 48%. Hemorrhages, rhage, mass effect, and herniation
subarachnoid or parenchymal, occur mainly in vertebral and Volume measurement may be demanded before rtPA
intracranial arterial dissections. CTA and MRA are equally administration
effective in the evaluation of arterial dissections in the neck. Location and pattern of dissection

256 Case 121
Answer autosomal dominant polycystic kidney disease, and pseu-

1. Cervical arterial dissection could be spontaneous or trau- doxanthoma elasticum. Traumatic causes include motor
matic. Spontaneous dissection could be idiopathic and vehicle collision, neck manipulation, exotic dancing, blunt
tends to occur in fibromuscular dysplasia, connective tissue injury, knife injury, gunshot injury. Infection could also
disorders such as Ehlers-Danlos syndrome and Marfan syn- produce dissection of the carotids.
drome, genetic disorders such as osteogenesis imperfecta,

Case
122 CLINICAL HISTORY 52-year-old female with coiled basilar tip
aneurysm.
FINDINGS Figure 122-1. Lateral CT scout view of the the T2WI. There is no surrounding artifact. Figure 122-5.
skull. There is a metallic density posterosuperiorly to the Axial 3D TOF MRA source image through the coil. There
dorsum sella (arrow) consistent with aneurysm coil. Figure is a round hypointense mass without artifacts within the
122-2. Axial MIP CTA through the level of the coil. There interpeduncular cistern (arrow). Figure 122-6. Source image
are extensive streak artifacts (arrow) at the site of the coil pre- CE 3D TOF MRA below the level of the aneurysm coil.
venting assessment of completeness of the aneurysm coiling There is a contrast-enhancing pouch (arrow) below the coil at
and other structures in the vicinity. The NCCT (not shown) the aneurysm neck region. Figure 122-7. MIP 3D TOF MRA
shows similar streak artifacts. Figures 122-3 and 122-4. without contrast. There is an obvious irregular pouch (arrow)
Axial MR GRE and T2WI through the coil within the inter- at the aneurysm neck consistent with residual aneurysm.
peduncular cistern. There is a round hypointense mass within
the interpeduncular cistern (arrows) on both theGREand DIFFERENTIAL DIAGNOSIS N/A.
257

258 Case 122
of aneurysm coiling due to the significant beam hardening

artifacts from the metallic coil that obscure not only the
aneurysm region but also the surrounding structures. The
gold standard used for follow-up of aneurysm treatment
used to be DSA. DSA is invasive, costly, and has compli-
cations. There is a body of evidence supporting the use of
contrast-enhanced TOF MRA (CE MRA) for the follow-
up of coiled aneurysms.
CE MRA has become the standard in most places for
evaluation of coiled aneurysms. The coil is usually dark
(hypointense) on all sequences and on the source images of
the MRA. They are virtually not visible on the MIP images.
However, additional stenting tends to introduce artifacts
within the stented vessels. The completeness of aneurysm
Figure 122-7 coiling falls into three categories. Class 1 is complete oblit-
eration. Class 2 is residual aneurysm neck, while class 3 is
recanalization of the aneurysm. Up to 50% of coiled aneu-
DIAGNOSIS Aneurysm coiling with residual aneurysm rysm may fall into class 1, while less than 50% fall into
neck. classes 2 and 3 combined. The reasons for incompleteness
of aneurysm coiling include prior rupture, large aneurysm
DISCUSSION Endovascular treatment of aneurysms size, wide neck, and large neck size. When evaluating these
with coils has increased significantly over the past images, it is usually good to have a checklist. The items
years following the publication of the findings of the on the checklist may include completeness of coiling, new
International Subarachnoid Aneurysm Trial (ISAT) that hemorrhage, mass effect from the coil, complications such as
showed favorable outcome for endovascular aneurysm hydrocephalus (due to subarachnoid hemorrhage [SAH] or
coiling over clipping. However, there is a high rate of mass effect from the coil), ischemic infarcts, cerebrovascular
recurrence or recanalization of coiled aneurysm com- spasm, and presence of new aneurysms or growth of other
pared with clipping. It is always necessary to follow up uncoiled aneurysms.
coiled or clipped aneurysms to determine the degree of
obliteration of the aneurysm. As shown in these images, Question for Further Thought
CT/CTA is usually not good for evaluating completeness 1. What is the aneurysm coil made of?

Case 122 259

Direct reporting is essential once a new, residual neck or 1. Coils can either be detached, pushed, or injected into
recanalized aneurysm is found. Complications such as the aneurysm and are made of various materials or com-
infarcts, hemorrhage, mass effect, hydrocephalus, or spasm bination of materials. The initial detachable coils were
should also be sought and reported. made of platinum less than hair strand in thickness.
Subsequent ones have polymer or hydrogel coating.
What the Treating Physician Needs to Know Some liquid coils are based on ethylene vinyl alcohol
Completeness of aneurysm coilingabsence of residual (EVOH). Pipeline devices are made of flexible mesh tube
neck and recanalization made of metal alloy. There are other forms of bioactive
Complications if any coated coils.

Case
123 CLINICAL HISTORY 1-year-old female with congenital hydrocephalus.
FINDINGS Figure 123-1. Axial NCCT through the lat- absence of the septum pellucidum with a well-formed corpus
eral ventricles. There is absence of the septum pellucidum callosum (arrow). Figure 123-4. Coronal T2WI through the
creating a univentricle (star). There is thickening and
suprasellar cistern. There is enlarged empty suprasellar cis-
smoothening of bilateral perisylvian gyri consistent with cor- tern (star) due to hypoplasia of the chiasm and infundibulum/
tical malformation (transverse arrows). There is a right-sided pituitary gland. The septum pellucidum is absent. The floor
closed lip schizencephaly (vertical arrows). Figure 123-2. of the frontal horns is pointing inferiorly (arrows).
Coronal NCCT through the suprasellar cistern. The chiasm is
thin (inferior vertical arrow) with cerebrospinal fluid (CSF) DIFFERENTIAL DIAGNOSISOptic infundibulum dys-
in the sella turcica. There is inferior pointing of the floor plasia (OID), septooptic dysplasia (SOD), lobar holoprosen-
ofthe frontal horns (transverse arrows). There is flattening cephaly, Kallmann syndrome.
of the roof of the frontal horns (superior vertical arrow).
Figure 123-3. Axial T2WI in a companion patient. There is DIAGNOSIS Septooptic dysplasia (SOD).
260

Case 123 261
DISCUSSION Both CT and MRI are capable of dem- delay, visceral anomalies, and neurologic deficits are com-
onstrating the changes of SOD. The septum pellucidum is mon. Management is mainly supportive and pituitary hor-
absent resulting in one rather than two lateral ventricles. mone replacement.
Coronal images demonstrate flattening of the ventricu-
lar roof and floor of the frontal horns pointing inferiorly. Question for Further Thought
The fornix is fused and low-lying. The optic pathways 1. What is the pathogenesis of SOD?
are hypoplastic along with the infundibulum and pituitary
gland resulting in empty large suprasellar cistern. There Reporting Responsibilities
may be ectopic placement of the neurohypophysis. Other Routine reporting is sufficient. A full description of all asso-
associated changes could include a variety of migrational ciated anomalies will be necessary to determine prognosis.
and cortical malformational disorders such as heterotopias,
schizencephaly, polymicrogyria in SOD plus. It may What the Treating Physician Needs to Know
be difficult to differentiate from lobar holoprosencephaly. Full description of findings
Absence of the olfactory apparatus may suggest Kallmann Other associated anomalies
syndrome. Isolated absence of septum pellucidum may be
found in otherwise normal people during neuroimaging for Answer
other reasons. 1. Embryologically, the disease is a disorder of midline pros-
SOD was described by de Morsier in 1956 as a rare encephalic development, sometimes placed within the
congenital disorder with abnormalities of the optic nerves family of holoprosencephaly. There is secondary degener-
and tracts and absence of the septum pellucidum. It is 80% ation of the optic nerve fibers due to cerebral lesions. Risk
female predominant with a prevalence of 6.3 per 100,000. factors include in utero infection especially cytomegalovi-
Nearly two-thirds of these patients have hypothalamic-pitu- rus (CMV), young maternal age, drug and alcohol abuse
itary dysfunction. Patient may present with hypopituitarism, and a disruption of blood flow to certain areas of the brain
hypotonia, and blindness, and the diagnosis is made clini- during critical development. Most SOD are sporadic but
cally when there is midline brain defect, optic nerve hypo- underlying genetic defect in the SOX, OTX2, and HESX1
plasia, and pituitary dysfunction. Seizures, developmental genes have been found in a very small percentage.

Case
124 CLINICAL HISTORY 19-year-old female with weight loss, slurred
speech, behavioral changes, hypersomnolence and confusion.
FINDINGS Figures 124-1 and 124-2. Axial FLAIR through the sylvian fissures. There are numerous tiny nodu-
through the posterior fossa and T2-weighted MRI through lar contrast-enhancing lesions throughout the brainstem,
the level of the basal ganglia. There are multifocal smudgy cerebellum, and cerebral hemispheres mainly in cortical and
cortical/subcortical hyperintensity widespread in the brain- subcortical locations (arrows point to representative lesions).
stem, cerebellum, and bilateral cerebral hemispheres (arrows Multiple areas of leptomeningial enhancement are also dem-
point to representative lesions) with some central gray mat- onstrated in Figure124-4 (vertical arrows).
ter
components with features consistent with vasogenic
edema. Figures 124-3 and 124-4. Axial post-contrast T1WI DIFFERENTIAL DIAGNOSIS Miliary tuberculosis (TB),
through the posterior fossa and coronal post-contrast T1WI metastases, neurocysticercosis, fungal granulomas.
262

Case 124 263
DIAGNOSIS Miliary TB of the brain. with echocardiogram were negative in this patient. Bacterial
blood cultures remained negative. The brain biopsy proved
DISCUSSION MRI is the modality of choice for evalu- positive on acid fast bacilli (AFB) stain and culture for
ating brain military TB. The vasogenic edema surrounding Mycobacterium tuberculosis. She was started on therapy
the lesions present as multifocal smudgy hyperintensity on with ethambutol, isoniazid, pyrazinamide, and rifampin;
FLAIR and T2WI mostly in the subcortical locations in both anti-seizure medication was added for prevention of sei-
infra- and supratentorial regions. Non-contrast T1WI shows zures. Over the next week, her symptoms improved, and was
smudgy hypointensities. Post-contrast T1WI shows nodular discharged home to complete the treatment.
homogeneous enhancement usually less than 3mm in diam-
eter within the edema throughout the brain. Invariably there Question for Further Thought
is associated leptomeningeal enhancement. Lesions are too 1. How often do we have to repeat the MRI of the brain
small to characterize on DWI. Follow-up MRI showed sig- during treatment?
nificant progression in number and size of lesions on follow
up. Tuberculous lesions may also undergo paradoxical reac- Reporting Responsibilities
tion to treatment, becoming more prominent earlier on during Presence of miliary lesions or multifocal lesions for that mat-
treatment before they eventually gradually regress. Lesions ter should be directly reported to enable urgent evaluation of
are located in the cortical and subcortical regions or where underlying pathology. TB should be suggested in the differ-
end arteries are such as the brainstem or basal ganglia due to ential of miliary lesions of the brain.
their hematogenous origin. They are usually tiny and monot-
onous rarely varying in size. Lesions that are close together What the Treating Physician Needs to Know
may coalesce into bigger lesions. The monotony of the lesion Miliary TB of the brain is usually associated with tuber-
size is important in differentiating these lesions from their culous meningitis. Presence of leptomeningeal lesions
mimics. Presence of leptomeningial enhancement adds to indicates meningitis
the confidence of the diagnosis of tuberculous meningoen- Treatment for TB should be initiated based on clinical
cephalitis. In contrast, metastases have varying sizes mostly suspicion in the presence of miliary lesions
ring enhancing with extraordinary amount of surrounding Is it safe to perform lumbar puncture (LP)? Yes, as long as
vasogenic edema. The granular nodular neurocysticercosis there is no mass effect
also tends to vary in size and may show some calcifications.
However, treated miliary TB may also show calcifications. Answer
Fungal granulomas may show a military pattern. Presence 1. Once the diagnosis is made, follow-up imaging depends
of paramagnetic materials in the walls of fungal granulomas on patients response to treatment. Clinical deteriora-
may result in GRE hypo/heterogeneous intensity. tion is usually an indication for imaging. If the patient
Clinical presentation of miliary TB is usually insidious is improving, the imaging should not be repeated. Once
presenting with weight loss, fever, weakness, confusion, drug treatment is completed, imaging is always necessary
and neurologic deficit. Initial abdominal and chest CT along to confirm the status of the lesions.

Case
125 CLINICAL HISTORY 2-year-old female with posterior head mass.
FINDINGS Figures 125-1 and 125-2. Axial and sagittal asymptomatic, and detected incidentally on imaging for
CTA images. There is a defect in the right retromastoid some other reason.
occipital bone. An intensely enhancing structure passes
through this defect to join the sigmoid sinus (arrow). Question for Further Thought
1. Is there an association between sinus pericranii and other
intracranial vascular anomalies?
DIFFERENTIAL DIAGNOSIS AV malformation, AV fistula,
sinus pericranii. Reporting Responsibilities
Direct reporting is essential to prevent inadvertent nee-
DIAGNOSIS Sinus pericranii. dling or intervention. Describe the location and nature of
the anomalously communicating veins. Search the brain for
DISCUSSION Sinus pericranii is an anomalous communi- other venous anomalies.
cation between a scalp vein and a dural venous sinus. The
most typical arrangement is communication between a varix,
or dilated vein, in the scalp and the superior sagittal sinus Sinus pericranii is, in most cases, an asymptomatic and
via an enlarged transcalvarial emissary vein. Drainage to incidentally detected anomalous communication between
the other dural sinuses, as in this case, is less common. On the extracranial and intracranial venous systems
noncontrast CT, the calvarial defect may be the only hint of When small, these are often left alone
this entity. MRI will better reveal the likely vascular nature Sinus pericranii is associated with a small risk of hemor-
of the lesion. Contrast studies and CTA confirm the diagno- rhage, and so, when these are large or of concern to the
sis. DSA can also be obtained for confirmation and mapping patient, they can be surgically treated without difficulty
prior to surgery.
These are very rare almost always congenital lesions, Answer
though posttraumatic versions have been reported. On clini- 1. Sinus pericranii is often found in conjunction with other
cal examination, the patient may present with a soft, reduc- venous anomalies, such as developmental venous anoma-
ible scalp mass possibly causing a bluish discoloration of the lies, venous malformations, and aberrant development of
overlying skin. Most of the time, sinus pericranii are small, the dural sinuses.
264

Case
126 CLINICAL HISTORY 29-year-old female with a history of severe
refractory headache and fatigue.
Figure 126-3
FINDINGS Figures 126-1 and 126-2. Axial NCCT through

the suprasellar and third ventricular levels, respectively.
There are multiple hypodense masses in the right sylvian
fissure, anterior interhemispheric fissure on Figure 126-1
and in the region of the genu of corpus callosum (CC) in
Figure 126-2 (arrows) with HU of 105 characteristic of Figure 126-4
265

266 Case 126
lipoma. There is a linear hyperdensity on the right side of and generally homogeneously hypointense to isointense on
the fat in Figure 126-2 consistent with calcification. Figure GRE and fat-saturated pulse. This distinguishes it from der-
126-3. Sagittal MRI T1WI. There is a large hyperintensity moid or teratoma. It does not contrast enhance. The most
in the anterior interhemispheric fissure (vertical arrow) and common locations are along the midline brain 45% within
at the location of the genu of the CC (transverse arrow). The the interhemispheric fissure and CC, 25% quadrigeminal,
CC is absent. Figures 126-4 and 126-5. Axial FLAIR and and superior cerebellar cisterns with the rest distributed else-
T2WI, respectively, through the level of the lateral ventricles. where within the subarachnoid and intraventricular spaces.
There is a thick anterior interhemispheric hyperintensity and Lipomas can be grouped into two distinct types based
a triangular-shaped hyperintensity between the tapered and on imaging: tubulonodular and curvilinear. Tubulonodular
displaced frontal horns of the lateral ventricles in the location lipomas are the more common variety. They are rounded
of the absent CC (arrows). Figure 126-6. Axial post-contrast or lobular and usually measure 2 cm or more in thickness.
fat sat T1WI through the lateral ventricles. The fat is hypoin- The anteriorly situated tubulonodular lipomas are associ-
tense on this fat sat image (arrow) without enhancement. ated with extensive callosal and midline anomalies while the
posteriorly located ones have less severe associations. The
DIFFERENTIAL DIAGNOSISMultifocal lipomas, pneu- tubulonodular variety can extend into the choroid plexus and
mocephalus, dermoid cyst, intracranial teratoma, multifocal the lateral ventricles. Curvilinear lipomas are usually thin,
hemorrhages. elongated, and curvilinear mainly along the corpus callosal
margin. They usually measure less than 1 cm in thickness
DIAGNOSIS Lipomas. and are more posteriorly situated. They are associated with
very mild congenital changes.
DISCUSSION Intracranial lipoma is a rare congenital Intracranial lipomas are usually asymptomatic anomalies
lesion characterized by the presence of fatty deposits in inap- and an occasional finding in patients undergoing imaging for
propriate places in the central nervous system (CNS). NCCT unrelated reasons. Most patients with sylvian fissure lipomas
usually shows a hypointense linear or nodular mass measur- (5%) tend to have seizures however. Lipomas associated
ing in the range of 100 HU as opposed to pneumocephalus with more severe congenital anomalies like CC agenesis may
from which it must be distinguished which usually measures present with seizures, psychomotor delay, headache, and
in the 1,000 HU. It usually deforms or mildly displaces its behavioral disturbances. Aneurysms and vascular malforma-
surrounding structures which could also be dysplastic or dys- tions have been reported in association with lipomas. The
genetic as in this case. Calcification is commonly associated lipomas by themselves are not likely symptomatic but the
with lipoma. Lipoma has unique MRI features. It is homoge- associated frontofacial abnormalities are probably respon-
neously hyperintense on all spin echo sequences and FLAIR sible for the symptoms. Lipomas are generally not treated.

Case 126 267
Question for Further Thought Curvilinear pericallosal lipomas can mimic inferior sagit-
1. What is the embryologic origin of intracranial lipoma? tal sinus and deep cerebral vein thrombus
Answer
1. Lipomas are considered congenital entities and not
Routine reporting is sufficient. Associated congenital anom-
tumors. They rarely grow. There are several theories
alies, mass effect, or the occasional hydrocephalus in quadri-
of their embryologic origins ranging from mesodermal
geminal cistern lesions are worth reporting.
inclusion due to dysraphism, hyperplasia of normal lep-
tomeningeal fat cells, heterotopia of displaced dermal
What the Treating Physician Needs to Know anlage, or as abnormal differentiation of embryologic
Location and number and associated congenital malforma- meninx primitiva. This last hypothesis seems the most
tions if any widely accepted; hence most intracranial lipomas are
Sylvian fissure lipomas almost invariably associated with located in the subarachnoid space in which location blood
seizures vessels and cranial nerves can run through them.

Case
127 CLINICAL HISTORY 52-year-old male presenting with headache
and acute onset of seizure.
FINDINGS Figure 127-1. Axial T2WI through the pari- there is T1 hyperintensity suggestive of hemorrhage (arrow).
etal lobes. There is a right parietal heterogeneous extraaxial There is no adjacent calvarial hyperostosis. Figure 127-3.
mass posteromedially abutting the falx cerebri and convexity Axial post-contrast T1WI. There is heterogeneous but avid
dura. There are prominent flow voids within (vertical arrow). enhancement with a small dural tail laterally (arrow). The
There is surrounding vasogenic edema particularly anteri- patient underwent complete resection of the lesion and post-
orly (transverse arrow) with local mass effect. Figure 127-2. operative baseline study (not shown) revealed no residual
Sagittal T1WI through the mass. The mass is heterogeneous abnormal enhancement. Figure 127-4. Axial post-contrast
with areas of isointensity and hypointensity. Posteriorly, T1WI 3 years later. There is enhancing nodular mass with
268

Case 127 269
dural tail within the surgical bed consistent with tumor recur- metastasis being distinctly unusual. Adult tumors are often
rence (arrows). multilobulated and large at presentation. They arise from
primitive mesenchymal cells, pericytes of Zimmerman,
DIFFERENTIAL DIAGNOSISMeningioma, hemangio- which are modified smooth muscle contractile cells sur-
pericytoma (HPC), dural metastases, lymphoma, neurosar- rounding capillaries. They were previously called angio-
coidosis, gliosarcoma, solitary fibrous tumor. blastic meningiomas but were reclassified in the 1993
WHO classification. These are distinctive mesenchymal
DIAGNOSIS Hemangiopericytoma (HPC). neoplasms that may be WHO II or III (anaplastic). HPCs
are highly cellular tumors with prominent mitotic activity
DISCUSSION HPC is a rare highly malignant extraaxial (median Ki-67 index of 5% to 10%). However, histologic
tumor which typically arises from the dura and is usually features are not predictive of outcome.
supratentorial in location. It may arise along the cerebral The mean age of detection is 35 to 45 years, and they
convexities, or along the falx, tentorium, or dural sinuses. often present with headaches, focal neurologic deficits, or
Most tumors have a broad-based dural attachment but can seizures. HPC eventually metastasizes extracranially, pre-
have a narrow dural attachment in up to a third of cases. dominantly to bone, lung, liver, and adrenals.
HPC is highly cellular and appears hyperdense on CT.
There is marked, often heterogeneous enhancement. HPCs, Questions for Further Thought
unlike meningiomas, are not associated with calcifications 1. What is the choice of therapy?
or hyperostosis. Bone erosion is present in more than half of 2. What is the natural history and prognosis?
the patients and is best evaluated by CT. Transcalvarial sub-
galeal extension through destroyed calvarium is common. A Reporting Responsibilities
dural tail can be seen in up to 50% of cases. These tumors Direct reporting is necessary in view of its malignant nature.
may invade the brain parenchyma and are often associated HPCs are highly cellular and vascular extraaxial tumors that
with peritumoral edema and mass effect. MRI often shows can bleed extensively during surgery. Presence of increased
isointense mass with internal flow voids on T1WI and T2WI. tumoral vascularity, enlarged vessels, cerebral edema, and
A small proportion of the tumors show heterogeneous inten- bone destruction should be reported.
sity due to necrosis or cyst formation. HPC is isointense to
slightly hyperintense on DWI and exhibits heterogeneous What the Treating Physician Needs to Know
contrast enhancement. MR spectroscopy shows absent
Extent of lesion, bony erosion, invasion of dural sinuses,
N-acetyl aspartate (NAA), markedly increased choline
and cranial nerves
with elevated myoinositol (using short TE). Nuclear medi-
Presence of extracranial metastases, commonly liver,
cine bone scan can be helpful for detection of extracranial
lungs, lymph nodes, and bones
metastasis. DSA demonstrates an early and prolonged, dense
Preoperative embolization helps prevent excessive bleed-
tumor stain with extensive arteriovenous shunting. Although
ing during surgery
the vascular supply is principally dural, these tumors often
parasitize local pial vascular supply depending on the loca-
tion of the tumor. Answers
HPC is considered a variant of solitary fibrous tumor from 1. Surgical resection with radiation therapy or radiosur-
which it may be difficult to differentiate. The lack of calcifi- gery for residual or recurrent disease are the treatments
cation and hyperostosis and the presence of flow voids, bone of choice. Preoperative embolization maybe helpful.
destruction and transcalvarial extension tend to differentiate Radiosurgery is an effective alternative to surgical resec-
this tumor from meningioma. Although lymphoma and neu- tion in locally recurrent tumors.
rosarcoidosis tend to be multifocal or diffusely dural based, 2. HPCs frequently recur locally in 50% to 90% of cases.
calvarial involvement is uncommon. Gliosarcomas are rare Extracranial metastases can be seen in up to 30% of cases,
glial tumors with dural involvement, often appearing more and the presence of extracranial metastases significantly
infiltrative indicating the parenchymal origin. worsens prognosis. Both local recurrence and distant
HPC rarely occurs intracranially in infants but have metastases can occur many years after initial diagnosis,
a more benign course with local recurrence and distant so long-term follow-up is mandatory.

Case
128 CLINICAL HISTORY Panel of fenestrated vessels.
FINDINGS Figure 128-1. 3D TOF MRA volume-rendering DIAGNOSIS Arterial fenestrations (AFs).
submento-vertical (SMV) view. The medial half of the left
A1 has two separate segments (arrows). The posterior seg- DISCUSSION AFs are congenital anomalies that result in
ment is joined to the anterior communicating artery while the formation of two separate segments along the course of the
anterior segment is joined to the left A2. The pattern is con- artery. AF has become more commonly demonstrated in the
sistent with fenestration of the left A1. Figure 128-2. Oblique era of cross-sectional imaging of CTA and MRA. CTA and
3D TOF MRA MIP of the neck. There is fenestration of MRA usually demonstrate a vessel splitting into two separate
the right distal vertebral artery (arrow) at V3V4 junction. segments along the course of the artery with the two segments
Figure 128-3. Coronal 3D volume rendering of CTA of the uniting distally. Fenestration is more common along the ver-
neck. There is fenestration of the proximal basilar artery tebralbasilar system particularly in the vertebral artery at
(arrow). Figure 128-4. Coronal MIP CTA of the head. There the C1C2 level or the V3V4 junction but AF can occur
is fenestration of the basilar artery terminus (arrow) involv- anywhere within the intracranial circulation as demonstrated
ing the origin of the bilateral P1. In all the four fenestrations, in this panel. The separate segments or limbs usually have
there is asymmetry of the size of the two fenestrated limbs. asymmetric calibers, and the length of fenestration is variable
from pin hole separation to short or long segments. It has been
DIFFERENTIAL DIAGNOSISArterial duplication, arte- noted that the histological composition of the walls of fenes-
rial fenestration. trated arteries are very variable and also asymmetric with one
270

Case 128 271
segment lacking in adequate muscular wall development with Reporting Responsibilities

irregular or absent elastic fiber development in the other. Both Routine reporting is sufficient. Location, number, and other
segments are enclosed in same adventitia. Fenestration should associated anomalies should be reported. This becomes par-
be differentiated from duplication. Fenestration is a split along ticularly important if interventional procedure is planned so
the arterial course while duplication implies double origins as to make the right choice of equipment and access to the
of the arterial structure with different adventitia and perhaps abnormality being treated.
similar normal muscular wall development.
AF is usually an incidental discovery on CTA, MRA, or What the Treating Physician Needs to Know
DSA. AF has been reported in association with aneurysms, Location, size, and associated anomalies if any
arteriovenous malformation (AVM), and arterial dissection.
It has been suggested that the abnormal histologic features of Answer
AF may predispose it to significant pathologic consequences. 1. There is a possibility that the presence of AF may influ-
ence the choice of access route or equipment during inter-
Question for Further Thought ventional procedures since these vessels are intrinsically
1. Are there clinical situations where the presence of AF smaller than their parent vessels. Knowledge of the pres-
may affect the decision-making process? ence of AF may therefore be useful in this regard.

Case 129 CLINICAL HISTORY 27-year-old male with nocturnal spells.
FINDINGS Figure 129-1. Axial T2WI through the lateral lateral ventricles with well-formed subcortical white matter
ventricles. The lateral ventricles are widely separated and (WM) tract (Probst bundle) and cortical gray matter (GM)
parallel with bilateral tapering of the frontal horns (vertical separating the lateral ventricles from the interhemispheric fis-
arrows) and dilated occipital horns (colpocephaly) (trans- sure. The septum pellucidum and corpus callosum (CC) are
verse arrows). The interhemispheric fissure separates the two absent. Figure 129-2. Axial DTI Fractional Anisotropy (FA)
272

Case 129 273
map through the lateral ventricles. The WM tract around the are best viewed on sagittal MRI although its relationship to
colpocephaly is thin suggesting hypoplasia (arrows). WM the cerebral hemispheres is best shown on coronal images.
tracts do not cross the midline. Probst bundles are medial DTI/tractography demonstrates the midline connectivity of
to the ventricles (star). Figure 129-3. Coronal T2 FLAIR the hemispheres through the CC. The CC is a densely packed
through the frontal horns. There is wide separation of the WM structure and therefore has the expected hypointensity
tapered frontal horns (vertical arrows). WM Probst bundle on T1WI and T2WI after the age of 24 months. Up until that
project medially to the frontal horns (transverse arrows). The age, MRI shows that myelination is more advanced in the
interhemispheric fissure extends down to the third ventricu- posterior part of the CC when compared to the anterior region.
lar roof (hatched arrow). Figure 129-4. Sagittal T1WI. There CC dysgenesis can be partial or complete (or agenesis).
is radiating cortical bundles, so-called spoke wheel gyri, in
the midline (transverse arrows). The CC and the cingulate Questions for Further Thought
gyrus are missing. The internal cerebral vein lies in the infe- 1. What is the etiology of CC dysgenesis or agenesis?
rior aspect of the interhemispheric fissure, the expected loca- 2. What are the clinical findings associated with CC dysgen-
tion on the velum of the third ventricle (vertical arrow). esis/agenesis?

Routine reporting is sufficient. Degree of dysgenesis and
DIAGNOSIS Agenesis of corpus callosum (ACC). other associated anomalies should be described.
DISCUSSION MRI is superior to CT in demonstrating the
features of ACC because of its multiplanar and tissue charac-
terization capabilities. Axial MRI demonstrates parallel lat- Pattern of CC dysgenesis
eral ventricles separated by brain tissue bundle on either side ACC can be isolated or associated with other brain abnor-
of the interhemispheric fissure. There is no midline crossing malities
of the WM. Coronal and axial MRI and DTI demonstrate As many brain structures form concurrently, other brain
the longitudinal callosal bundles of Probst. They represent malformations and syndromes such as interhemispheric
nondecussated callosal fibers that deviate at the interhemi- cysts, pericallosal lipomas, lissencephaly, polymicrogy-
spheric fissure to run along the medial borders of the lateral ria and pachygyria, neuronal heterotopias, Dandy-Walker
ventricles instead of crossing the midline. Coronal images malformation, midline facial malformation, and syntelen-
demonstrate widely separated anterior horns of the lateral cephaly (fusion of the dorsal part of the brain) might be
ventricles with the interhemispheric fissure extending unto present
the roof of the third ventricle (trident sign) or in some cases Dysgenesis of the CC can be screened for in utero using
unto a midline cyst resembling a Viking helmet or mouse ultrasound or MRI
head. ACC is typically accompanied by a characteristic dil-
atation of occipital horns or colpocephaly. The sagittal image Answers
shows radiating brain folds on either side of the midline. The 1. CC dysgenesis can be sporadic or result from genetic
cingulate gyrus is not visualized. DTI confirms lack of mid- (chromosomal abnormalities such as trisomies 8 and 18
line connectivity. and genetic syndromes such as Andermann and Aicardi),
The CC develops between 8 and 20 weeks of gestation infectious (prenatal rubella, toxoplasmosis, CMV, influ-
and is made of four parts with the rostrum anteriorly fol- enza), vascular, or toxic (fetal alcohol syndrome) causes.
lowed by the genu, body, and splenium in that order. The 2. The clinical presentation of CC dysgenesis is very broad
development of the CC is thought to progress craniocaudally, and ranges from none in two-thirds to three-quarters of the
with the exception of the most anterior part, the rostrum, that cases to borderline/moderate and severe disability with
develops later. Some studies suggest that the development learning disability, mental retardation, seizures, and psy-
of the CC starts with the formation of the anterior body and chiatric disorders. Most importantly, the prognosis does
progresses bidirectionally. The four components of the CC not seem to be different in isolated and complex cases.

Case
130 CLINICAL HISTORY 55-year-old male with a history of alcohol
abuse. He was found unconscious on the street and brought to the hospital
by the emergency services.

274

Case 130 275
FINDINGS Figure 130-1. Axial NCCT is normal. methanol. The clinical presentations may vary, and generally
Figure130-2. Axial T2WI. There are bilateral hyperin- there is a latent period of 12 to 24 hours before symptoms due
tense globus pallidus lesions (arrows). Figure 130-3. Axial to the time required for methanol to be metabolized by the
FLAIR shows bilateral lentiform nuclei hypointensities liver into formaldehyde and formic acid, both of which are
(arrows). Figure 130-4. Axial GRE demonstrates bilateral potent harmful metabolites that result in severe metabolic aci-
symmetrical hyperintensity (arrows) with surrounding min- dosis with high anion gap. The optic nerves and the putamina
eralization (hypointensities) in the medial lentiform nuclei. are the most vulnerable sites, and visual complaints that are
due to acute optic nerve demyelination or necrosis are usu-
DIFFERENTIAL DIAGNOSISHypertensive intracranial ally the first symptoms. Early neurologic symptoms include
hemorrhage, anoxic infarcts, methanol intoxication, carbon headaches and seizures and later permanent neurologic dys-
monoxide poisoning, extrapontine osmotic demyelination function, coma, and death. Nausea, vomiting, and abdominal
syndrome, and Wilson disease. pain are common initial symptoms, while respiratory arrest is
a later symptom (6 to 36 hours after the intoxication).
DIAGNOSIS Methanol intoxication. Management involves initial supportive care and pre-
venting the progression of lesions with the administration of
DISCUSSION Typical imaging findings of methanol intox- ethanol or fomepizole.
ication are bilateral hemorrhagic putaminal necrosis, clearly
seen on both CT and MRI and reported in about 7% to 14% Question for Further Thought
of patients and always associated with a poor prognosis. CT 1. Why are the putamina the most affected structures by
findings in early stages may also include hyperdense lesions methanol intoxication?
representing hemorrhage or hypodense areas corresponding
to cytotoxic edema in both putamina that later evolve to cys- Reporting Responsibilities
tic necrosis if the patient survives. Acutely, optic nerves may This is an acute event and requires direct reporting.
also be swollen and show contrast enhancement. On MRI, Identifying the pattern of bilateral putaminal hemorrhagic
putaminal lesions display variable signal intensity on T1WI involvement as a probable methanol intoxication is impor-
(depending on the age of the hemorrhage), hyperintensity tant so that proper treatment is administered.
on T2WI (as well as in other basal ganglia and contiguous
white matter [WM]), restricted diffusion on DWI and vari- What the Treating Physician Needs to Know
able contrast enhancement (no enhancement to rim or strong
An immediate diagnosis to establish treatment
enhancement). Edema and/or necrosis of other structures
such as the hippocampi, cerebellum, or fronto-occipital sub- The extent of the central nervous system (CNS) involve-
cortical WM may be detected, usually with preservation of ment to determine prognosis
the subcortical U fibers.
Methanol is a clear colorless liquid with a weak alcoholic Answer
odor, widely used in cleaning products, antifreeze (for cars), 1. Putamina are the more vulnerable structures to the toxic
and industrial solvents. Acute intoxication occurs after the effects of methanol in the CNS because of their high
consumption of adulterated alcoholic beverage or following oxygen and glucose consumption and poor venous drain-
the accidental or suicidal ingestion of a product containing age that exposes them to the toxic effects of formic acid.

Case
131 CLINICAL HISTORY 46-year-old female with history of diabetes mellitus,
end-stage renal disease, on hemodialysis, was found down and unresponsive
with seizures and fever.
FINDINGS Figure 131-1. Axial DWI through the level of the trigones. There is blooming within the trigones (arrows)
the frontal horns of the lateral ventricles. There is a left fron- consistent with hemorrhage. Figure 131-4. Axial FLAIR
tal white matter (WM) and a left peritrigonal focal restricted through the trigones. There is hyperintensity within the con-
diffusion consistent with acute infarcts (vertical arrows). vexity subarachnoid spaces (transverse arrows) suggesting
There is subtle multi-focal diffusion restriction in the sple- meningitis or subarachnoid hemorrhage (SAH). There is
nium centrally (line arrow) and within the trigones and bilateral peritrigonal confluent hyperintensity consistent with
subarachnoid spaces. Figure 131-2. Axial DWI through the edema (vertical arrows) Figure 131-5. Coronal post-contrast
level of the centrum semiovale showing bilateral deep WM T1WI through the trigones. There is bilateral ependymal
restricted diffusion (stars). Figure 131-3. Axial GRE through enhancement (ventriculitis) in the trigones surrounding the
276

Case 131 277
shows widespread WM changes consistent with chronic

ischemic changes. Arterial cerebrovascular complications
including vasculitis resulting in vessel wall irregularities,
arterial narrowing and/or dilatations, and occlusions of
distal vascular branches could be seen on DSA and CTA.
Sulcal FLAIR hyperintensity and leptomeningeal enhance-
ment in this situation are due to the meningitis. SAH should
be considered particularly with GRE blooming (hemor-
rhage) within the lateral ventricles. SAH and parenchymal
hemorrhages are known complications of S. pneumoniae
meningoencephalitis. Hydrocephalus and subdural effu-
sions are other common complications.
The magnitude of the CNS insult depends on the host
immune response and the neurovirulence of the pathogen.
Infecting organisms display differences in neurotropism,
explaining differences in the areas affected, clinical pre-
sentation, and imaging findings. Clinical presentation could
include febrile illness, headache, photophobia, stiff neck,
cognitive dysfunction, behavioral changes, focal neurologic
deficits, and seizures. Diagnosis is usually made on CSF
Figure 131-5 evaluation and blood and CSF culture including sensitivity
to decide appropriate antibiotic treatment.
intraventricular hemorrhage (vertical arrows). There is lep- Question for Further Thought
tomeningial enhancement diffusely but more prominent 1. What investigation (MRI vs. CT scan) should be ordered
around the superior vermis with encysted fluid collections in a patient suspected of meningoencephalitis?
(transverse arrows). There is a focal nodular right parietal
parasagittal contrast enhancement (line arrow). A few other Reporting Responsibilities
parenchymal ring enhancements (not shown) were present. This is an acute situation requiring direct reporting. Compli
Bilateral parietal convexity cerebrospinal fluid (CSF) isoin- cations such as cerebritis, abscess, hydrocephalus, effusions,
tense small effusions enclosed by leptomeningial enhance- herniations, and cerebral vein thrombosis when present
ment (chevrons) are also present. should be recorded. Presence of invasive sinusitis should
be mentioned. Development of complications on follow-up
DIFFERENTIAL DIAGNOSIS Meningoencephalitis, SAH should be communicated directly.
with complications.
DIAGNOSIS Meningoencephalitis (Streptococcus pneu- Are there any specific signs that could point in the direc-
moniae). tion of specific meningoencephalitis?
Are there complications?
DISCUSSION S. pneumoniae is a common cause of Is it safe to perform a lumbar puncture (LP)? Yes if there
bacterial meningitis, but less frequently associated with is no significant swelling or mass effect
encephalitis. Most of the cases of meningoencephalitis with Imaging obtained very early in the course of the disease
S. pneumoniae have been described in the pediatric popu- can be normal. Suspicion and history are key to successful
lation but can occur in adults. S. pneumoniae meningoen- diagnosis
cephalitis presents with imaging changes of meningitis, Other imaging methods to confirm the diagnosis? CTA
vasculitis with arterial thrombosis, and/or septic cortical and DSA may show the vascular complications
thrombophlebitis leading to ischemia and hemorrhages.
Central nervous system (CNS) parenchymal injury in the
early course of the disease are seen as areas of restricted dif- Answer
fusion and T2 hyperintensities both in the gray matter (GM) 1. MRI is more sensitive and specific than CT scan and
and mostly WM reflecting areas of ischemia due to necro- should be the study of choice for the evaluation of patients
tizing vasculitis and/or thrombosis. Follow-up in survivors suspected of meningoencephalitis.

Case
132 CLINICAL HISTORY 7-month-old baby supposedly fell out of stroller!
278

Case 132 279
FINDINGS Figure 132-1. Axial NCCT through the lateral of the causes of the IVH. Dependent debris of ventriculitis
ventricles. There is bilateral intraventricular hyperdensity usually restricts diffusion and is not hypointense on GRE.
with bloodcerebrospinal fluid (CSF) levels in the trigones Ventricular wall enhancement may be present which is
(arrows). There is diffuse brain hypodensity with no white uncommon in acute IVH.
gray matter differentiation and effacement of convexity sulci IVH occurs in all ages and could be primary or second-
consistent with brain edema and swelling. Figure132-2. ary. There is a long list of causes which include trauma,
Axial T2WI through the lateral ventricles. There are fluid ruptured aneurysms, ruptured AVM, ruptured intracerebral
fluid levels in the trigones (arrows) with hypointense depen- hematoma (ICH), hypertension, coagulopathy, meningo-
dent collections. Figure 132-3. Axial GRE through the lateral encephalitis, moyamoya, and intraventricular hemorrhagic
ventricles. There is bilateral intraventricular hypointensity metastases. About 45% of spontaneous ICHs and 25% of
with fluidfluid levels in the trigones (arrows). Figure 132-4. aneurysmal subarachnoid hemorrhages (SAHs) extend into
Axial FLAIR through the lateral ventricles. There is bilateral the ventricles. Symptoms may include headache, confusion,
intraventricular hypointensity with fluidfluid levels in the and drowsiness. Presence of hydrocephalus is a sign of poor
trigones (arrows). The dependent hemorrhage in the trigones prognosis. Treatment consists of managing the underlying
is slightly hyperintense compared with the CSF in the third pathology to avoid rebleed and managing raised intracranial
ventricle. There is visible sulcal hyperintensities consistent pressure (ICP). Fibrinolysis in combination with extraven-
with subarachnoid hemorrhage. tricular drainage shows promise as a technique to reduce
intraventricular clot volume and to manage the concomitant
DIFFERENTIAL DIAGNOSISVentriculitis, intraventric- complications of IVH. Prognosis is poor with mortality in
ular hemorrhage (IVH). the range of 50% to 80%.
DIAGNOSIS Acute intraventricular hemorrhage (acute Question for Further Thought

IVH) in acute hypoxic-ischemic encephalopathy. 1. What are the complications of IVH?
DISCUSSION Demonstration of subtle IVH is impor- Reporting Responsibilities

tant. The modality of choice for demonstrating acute IVH This is an acute situation requiring direct reporting.
is NCCT. Usually acute hemorrhage is hyperdense on CT Associated ICH or SAH as well as visible underlying pathol-
measuring between 60 and 100 HU. Depending on the vol- ogy should be reported. Presence of hydrocephalus and
ume of the hemorrhage, it could form a bloodCSF level raised ICP makes reporting more urgent.
which depends on the different densities of blood compo-
nent and CSF or fill the entire ventricle as a hyperdense clot.
Hydrocephalus is a common complication due to foraminal
or ductal obstruction. The source of the hemorrhage may not Size of hemorrhage and location
be evident on the CT. In this particular situation it is assumed Causes of IVH if present on the images
that this was due to coagulopathy in a case of child abuse Is there hydrocephalus and raised ICP?
as there were subarachnoid, subdural, and subgaleal hemor- Recommendation for the workup of the causes of the IVH
rhages in addition to the IVH. Contrast-enhanced CT or CTA
may elucidate on other causes of the hemorrhage such as Answer
arteriovenous malformation (AVM), ruptured aneurysm, and 1. Acute complications of IVH visible at imaging include
vasculitis in the appropriate clinical situation. Parenchymal acute obstructive hydrocephalus, periventricular edema,
hematomas may rupture into the lateral ventricles. IVH could reduced cerebral perfusion, raised ICP, brain swelling
be a complication of surgical intervention as in intraventricu- with resulting mass effect. Other complications may
lar catheter placement or neurosurgical operations. include damage to the thalamus and reticular activating
MR usually shows intraventricular hypointensity on all system resulting in decreased level of consciousness and
sequences in acute IVH. The GRE best depicts acute IVH as coma, permanent occlusion of the arachnoid granulations
dark signal within the ventricles. The FLAIR usually shows by blood breakdown products result in poor CSF absorp-
hypointense blood which is slightly brighter than CSF. As tion and subsequent communicating hydrocephalus, and
on the CT, a bloodCSF level is common within the lateral the inflammatory reaction caused by blood products may
ventricles, and a clot cast within the ventricles is not uncom- affect long-term cognitive function. All these result in
mon in large hemorrhages particularly within the third and significant morbidity and increased mortality, hence the
fourth ventricles. MRA may also be utilized in the evaluation necessity for aggressive management of IVH.

Case
133 CLINICAL HISTORY 71-year-old male suddenly became bradycardic,
hypoxic, and unresponsive following esophagoscopy to dislodge impacted meat.
280

Case 133 281
Figure 133-6
DISCUSSION The hallmark of CAGE on NCCT is

hypodense gas bubbles within the brain parenchymal mea-
sured in this instance 450 HU. The HU measurement dif-
ferentiates gas from fat. There are also gas bubbles outlining
the right hemispheric sulci presumably within leptomenin-
geal vessels or subarachnoid space (SAS). There appears
to be a preponderant involvement of the right cerebral
hemisphere by CAGE in the literature. These gas bubbles
Figure 133-5
are usually visible within a very short time after the occur-
rence. They tend to disappear within 24 hours at which time
the affected cerebral hemisphere becomes hypodense with
FINDINGS Figure 133-1. Axial NCCT through the cen- mass effect consistent with cerebral swelling and infarc-
trum semiovale. There are multiple gas bubbles measuring tions. DWI demonstrates gyriform cortical ribbon hyper-
up to 450 HU in the right cerebral parenchyma and within intensity consistent with cortical laminar necrosis with
the right convexity sulci (arrows). Fewer similar gas bubbles underlying WM edema visualized on FLAIR and T2WI.
are present in the left frontal lobe. Figure 133-2. Axial fol- Diffuse mass effect is present. Contrast enhancement both
low-up NCCT within 24 hours. There is resolution of the gas within the sulci and brain parenchyma is in keeping with
bubbles. There is diffuse right cerebral hemisphere hypoden- bloodbrain barrier breakdown.
sity with no gray matter (GM)white matter (WM) differ- CAGE is a rare complication of endoscopy and has been
entiation. There is effacement of the right convexity sulci. reported following upper gastrointestinal (GI) endoscopy
Similar but less prominent hypodensity is present in the left of various types. CAGE has variable clinical presentations
cerebral hemisphere (arrow). Figure 133-3. Axial DWI a which include altered mental status, confusion, headache,
few hours following Figure 133-2. There is extensive right dizziness, seizures, visual field defect, and coma during
hemispheric cortical ribbon hyperintensity consistent with or shortly after the procedure. Autopsy report has demon-
cortical laminar necrosis (arrows). There is underlying WM strated edematous brain with changes of diffuse hypoxic
smudgy hyperintensity. Similar but patchy changes are pres- ischemic damage at microscopy. Brain herniations have
ent in the left hemisphere. Figure 133-4. Axial FLAIR. There also been reported. There is no evidence-based treatment
is smudgy right hemispheric hyperintensity with patchy since very few cases have been reported. The recommended
changes on the left. Figure 133-5. Axial post-contrast T1WI. treatment is administration of hyperbaric oxygen (HBO2).
There is extensive right hemispheric linear sulcal and patchy This patient had six HBO2 therapy treatments but did not
parenchymal contrast enhancement with similar but less regain consciousness.
prominent changes on the left (arrows). Figure 133-6. 3D
TOF MRA. There are more prominent right MCA territory Question for Further Thought
branches (arrows) compared to the left suggesting increased 1. How does air get into the cerebral arterial circulation dur-
right hemispheric perfusion. ing endoscopy?
DIFFERENTIAL DIAGNOSIS Gas embolus, fat embolus. Reporting Responsibilities

This is an acute event that requires direct reporting. Immediate
DIAGNOSIS Cerebral arterial gas embolism (CAGE). HBO2 administration has been suggested as life-saving.

282 Case 133
What the Treating Physician Needs to Know into the right atrium. Presence of intraatria shunt allows
Location of the air bubbles gas into the arterial circulation and onward propagation
Presence or otherwise of brain swelling or herniations to the brain and other organs. It is also suggested that in
the absence of intraatrial shunt, overwhelming amount of
Answer pulmonary venous gas in excess of 30 mL could result in
1. Gas entry into the circulation requires an open vessel systemic arterial gas embolism. Echocardiogram did not
and a pressure gradient. The presence of severed veins reveal intraatrial shunt in this patient. A patent foramen
at the site of the procedure coupled with high pressure ovale is present in 33% of the normal population until the
gas insufflation allows gas to enter the venous circulation third decade and in 27% in all age groups.

Case
FINDINGS Figure 134-1. Axial T2WI through cavernous characteristically hypointense on T2WI, again reflecting
sinuses. There is a mass, slightly hyperintense to brain, strad- high cellularity, which can help to distinguish it from other
dling the suprasellar space and the middle cranial fossa on tumors. Regardless of modality, enhancement is usually
the left (arrows). Figures 134-2 to 134-4. Axial, coronal, and homogeneous and intense. Underlying bone invasion and
sagittal post-contrast T1WI, respectively. The mass is homo- sclerosis and thickening are common. Most meningiomas
geneously enhancing. The mass encases and slightly narrows are slow growing and show benign behavior. Metastasis
the left internal carotid artery (arrow in Figure 134-3). The are often multiple and show significant aggressive behav-
mass extends into the sella but remains separate from the ior such as invasion of underlying brain or overlying
flattened pituitary (white arrow in Figure 134-4). Notice the bone. It may be difficult to distinguish one from the other.
additional midline lesion (black arrow in Figure 134-4). Sarcoidosis could easily mimic meningioma. Associated
leptomeningeal or parenchymal lesions may help in differ-
DIFFERENTIAL DIAGNOSIS Meningioma, pituitary mac entiating sarcoidosis from meningioma. Intrasellar location
roadenoma, metastasis, sarcoidosis. and widening of the sella turcica are more in favor of pitu-
itary macroadenoma.
DIAGNOSIS Meningioma. Meningioma is a very common extraaxial mass which can
be found anywhere within the calvarium. The tumor derives
DISCUSSION On CT, meningioma is often homoge- from arachnoid meningothelial cap cells and usually demon-
neous and slightly hyperdense, reflecting high cellular- strates a broad dural attachment, sometimes tapering gradu-
ity. Calcifications may be present. On MRI, the tumor is ally at the margins to form a so-called dural tail. Common
283

284 Case 134
locations include the falx, the cerebral convexities, anterior tumor and any associated mass effect or compromise to adja-
skull base, suprasellar region, cavernous sinus, and cerebel- cent normal structures.
lopontine angle. On rare occasions, they may be intraven-
tricular, in which case they are usually located in the lateral What the Treating Physician Needs to Know
ventricular atrium. Meningioma can arise in all age groups,
Meningiomas are very common tumors in adult patients,
though it is especially common in middle-aged women. Skull
which can be diagnosed in most cases with reasonable
base meningiomas are particularly difficult to treat. Both sur-
confidence on imaging alone
gical resection and radiation therapy are treatment options
Location and size
and may be combined.
Complications such as vascular encasement, osseous inva-
sion, mass effect, and brain edema
1. Are all meningiomas benign?
2. What is the appropriate management when a meningioma Answers
is detected? 1. Most meningiomas are benign, slow-growing tumors that
produce symptoms through mass effect. More aggressive
Reporting Responsibilities atypical and even malignant variants do exist, which
Direct reporting is essential when tumor is in critical areas on imaging tend to show invasion of the brain or skull,
such as the skull base, invading the bone or causing sig- recurrence after surgery, and even metastasis.
nificant brain edema and mass effect. Make as confident an 2. Asymptomatic lesions can be followed on imaging. For
imaging diagnosis as possible based on the presence of the symptomatic lesions, surgery is curative. If the tumor is
fairly characteristic features. Describe the location of the surgically inaccessible, radiation therapy can be beneficial.

Case
135 CLINICAL HISTORY 59-year-old male native of Mexico was admitted
with decreased level of consciousness, weight loss of approximately 40
pounds, and persistent low-grade fever, night sweats, and fatigue.
285

286 Case 135
FINDINGS Figures 135-1 and 135-2. Axial FLAIR and DISCUSSION Tuberculomata and TB brain abscesses are
post-contrast T1WI MRI through the splenium of the cor- space-occupying lesions 0.41 cm and over 1 cm in size,
pus callosum showing multiple ring-enhancing small lesions respectively. CT of TB granulomata may show nonspe-
measuring about 1 cm or less in size in the left parasagittal cific hypodensities, while the larger abscesses may show a
frontal lobe, left splenium, and in bilateral occipital lobes smooth isodense ring surrounding a hypodense core. These
(arrows). Lesions are surrounded by very minimal hyperin- lesions tend to show smooth ring enhancement follow-
tensity on the FLAIR except in areas where they coalesce as ing contrast administration. TB shows restricted diffusion
in the splenium where the surrounding T2 hyperintensity is on DWI particularly the larger abscesses with the smaller
large. Lesions are mostly cortical and/or subcortical in loca- lesions being poorly defined. There is a hypointense smooth
tion. Figures 135-3, 135-4, 135-5, and 135-6. Axial DWI with ring with a central hyperintense core on T2WI. The rim
corresponding ADC map, T2WI, and post-contrast T1WI tends to be hyperintense on FLAIR with smooth enhance-
through the superior centrum semiovale. There are more ment following contrast administration. Presence of lepto-
multifocal cortical subcortical lesions with central diffusion meningeal enhancement suggests meningitis particularly at
restriction in bilateral frontal lobes. The two posterior left the base of the brain and the large fissures. Tuberculomata
frontal lobe lesions (transverse arrows) show smooth thick and TB abscesses could be single or multiple, but more often
ring enhancement in Figure 135-6 with surrounding vaso- the tuberculomata are multiple. There is usually consider-
genic edema (vertical arrows in figures 5 and 6). The larger able vasogenic edema surrounding the larger abscesses. The
left posterior frontal lobe lesion measures about 1.6cm with larger abscesses often show a crenated appearance with a
a hyperintense core and hypointense rim on the T2WI (Figure heterogeneous core on T2WI. The crenated rim may distin-
135-5 transverse arrow). There are many more lesions in the guish TB from pyogenic abscess where the ring is almost
brainstem, cerebellum, and cerebrum (not shown). There is always smooth. Metastatic rings are usually irregular and
very minimal leptomeningial enhancement. generally do not restrict diffusion centrally. Calcifications
may be present in neurocysticercosis with minimal edema.
DIFFERENTIAL DIAGNOSISTuberculomata/abscess, met Toxoplasmosis tends to show the concentric target pattern on
astases, neurocysticercosis, chronic pyogenic abscesses, T2WI and the eccentric target sign on post-contrast images.
toxoplasmosis. Clinical presentation is usually with fever or low-grade
temperature, malaise, headache, nausea, vomiting, espe-
DIAGNOSIS Tuberculomata and tuberculous (TB) abscess. cially in cases of meningitis. Seizures, focal neurologic

Case 135 287
deficits, and altered mental status are other symptoms. e mphasized as these usually indicate vascular complication
CSF evaluation usually shows elevated white cells with that is more common with meningitis.
lymphocytic predominance, high protein, and low glucose.
Diagnosis was made by brain biopsy and drainage of the What the Treating Physician Needs to Know
large lesion. Bronchoalveolar lavage culture was positive Number and location of lesions to allow decision regard-
for acid fast bacilli (AFB) after 6 weeks. Corticosteroid ing management choices
treatment in addition to quadruple drug regimen (isonia- Complications such as hydrocephalus or herniations that
zid (INH) + pyrazinamide (PZA) + ethambutol (EMB) may require surgical intervention
+ rifampicin (RIF)) usually reduces tuberculoma size and Is lumbar puncture (LP) safe? Significant herniation may
perilesional edema leading to symptomatic improvement preclude an LP
and seizure control. Surgical therapy for intracranial tuber- Patients started on anti-TB medication can have a paradox-
culoma is considered only when a positive diagnosis is not ical reaction with worsening of the symptoms and expan-
possible by other means, medical therapy fails, or when sion of the tuberculoma
decompression is necessary.
Answer
Question for Further Thought 1. Mycobacterium tuberculosis is the second cause of death
1. How common is tuberculoma and TB brain abscess? worldwide, accounting for approximately 9 m illion deaths
each year. In the United States, the majority of cases are
Reporting Responsibilities diagnosed in the homeless, intravenous drug abusers,
Direct reporting is always necessary if an abscess or multi- patients with AIDS, foreign-born persons (especially from
focal lesions are present. Presence of herniation, significant countries with high-prevalence of TB), prison inmates,
edema, leptomeningeal enhancement, and hydrocephalus migrant farm workers, and alcoholics. Intracranial tuber-
should be reported as these may influence management culomas develop in up to 1% of patients with active tuber-
decisions. If bland infarcts are present they should be
culosis and up to 28% of those with TB meningitis.

Case
136 CLINICAL HISTORY Patient with leukemia and sinusitis presenting with
acute proptosis and periorbital chemosis and bilateral cavernous sinus
syndrome with multiple cranial nerve palsies.
DISCUSSION Contrast-enhanced CT and MRI in the

venous phase are the preferred imaging of choice, as the cav-
ernous sinus and its venous tributaries cannot be properly
evaluated otherwise. Even though findings on a non-contrast
CT are often subtle, an asymmetric or expanded hyperdense
cavernous sinus with a convex lateral wall, and an associ-
ated dilated superior ophthalmic vein, are suggestive of
CST. Contrast-enhanced MRI shows multiple nonenhanc-
ing defects in the normally enhancing cavernous sinus. The
ipsilateral tentorial leaf may appear thick and show contrast
enhancement. On CTV or MRV, the abnormal and irregular
nonenhancing filling defects in the sinuses and tributaries
correlate with the thrombi. The diagnosis may be challeng-
ing and at times impossible to be definite. Almost all the
Figure 136-3 differentials tend to contrast enhance even when they form
filling defects.
CST can have a septic or aseptic etiology, with an infec-
FINDINGS Figure 136-1. Coronal post-contrast T1WI with tious origin being much more common than the posttrau-
fat suppression shows that both cavernous sinuses contain matic or postsurgical ones. Septic causes include sinusitis,
multiple nonenhancing foci (arrows). Figure 136-2. Axial otitis media, odontogenic conditions, and skin infections on
fat-suppressed post-contrast T1WI shows again the cav- the mid-face area, all of them involving the cavernous sinus
ernous sinuses are expanded and contain nonenhancing by direct extension or spread of infection through draining
foci (arrows). Figure 136-3. Axial T2WI through the orbits veins such as the ophthalmic veins and the emissary veins
shows hyperintense filling defects expanding both superior that connect to the pterygoid plexus. Microorganisms that
ophthalmic veins (arrows). The soft tissues of the eyelids are have been reported to produce CST vary greatly depending
hyperintense and swollen. on the source of infection, but Staphylococcus aureus is by
far the most common one.
DIFFERENTIAL DIAGNOSIS Cavernous sinus neo- The usual clinical presentation is related to the source
plasms (schwannoma, meningioma, lymphoma, and metas- of infection and includes fever, tachycardia, hypotension,
tasis), cavernous sinus thrombosis (CST), infections or confusion, and even coma. Headache with a unilateral fron-
inflammatory processes (Tolosa-Hunt syndrome, sarcoid- totemporal or retrobulbar distribution and nuchal rigidity
osis, and Wegener granulomatosis), cavernous carotid fistu- generally mean intracranial or meningeal extension and these
las, and internal carotid aneurysms. conditions are reported in about one-third of patients. Visual
impairment can result from congestion of the ocular venous
DIAGNOSIS Cavernous sinus thrombosis (CST). drainage (chemosis, periorbital edema, and proptosis) or be
288

Case 136 289
secondary to involvement of the cranial nerves that innervate congestion affecting the ophthalmic structures and cerebral
the extraocular muscles all of them contained in the cavern- parenchyma, which include swelling, edema, hemorrhage,
ous sinus. Other signs or symptoms include hypo- or hyper- and/or infarct.
esthesia of the second and third divisions of the trigeminal
cranial nerve and pituitary dysfunction. What the Treating Physician Needs to Know
Confirmation of their suspected clinical diagnosis
Questions for Further Thought If the source of infection is discernible on the images
1. Is CST treatment surgical or medical? Any complications caused by venous congestion
2. What are the current figures concerning CSTs morbidity
and mortality? Answers
1. Management is based on a surgical approach on the pri-
Reporting Responsibilities mary source of infection and the affected surrounding
Direct reporting is essential in this often fatal condition. The tissues, as well as an associated aggressive medical treat-
earlier treatment is started, the better the outcome! Make the ment which includes antibiotics, corticosteroids, and anti-
diagnosis so that anticoagulation can be started. Make sure coagulants.
the contralateral sinus is patent. Describe the probable source 2. Even though diagnosis and treatment have improved in
of infection. Report the extent of CST, the hemodynamic recent decades, CST still has a high mortality and morbid-
changes on the draining veins, and the probable venous ity (30% to 50%).

Case
137 CLINICAL HISTORY 2-year-old female with progressive difficulty walking,
deterioration of coordination, and speech disturbances.
FINDINGS Figure 137-1. Axial DWI through the splenium

of corpus callosum. There is hyperintensity of the sple-
nium (arrow). The ADC map (not shown) is consistent with
restricted diffusion. Figures 137-2 and 137-3. Axial T2WI
through the splenium and centrum semiovale, respectively.
There is symmetric confluent white matter (WM) (sometimes
called butterfly) hyperintensity (stars) extending from the
ventricular walls to the subcortical WM. The splenium and
genu of the corpus callosum (arrows) are involved. Linear
tubular and punctate hypointensities are present within the
confluent WM changes consistent with the so-called tigroid
pattern.
DIFFERENTIAL DIAGNOSISPelizaeus-Merzbacher,
TORCH, periventricular leukomalacia, metachromatic leu-
kodystrophy (MLD), Krabbe disease.
DIAGNOSIS Metachromatic leukodystrophy (MLD).
DISCUSSION MLD is a devastating demyelinating dis-

ease due to lysosomal storage disorder resulting in the accu-
mulation of galactosylceramide sulfatide, a lipid component
of myelin with a characteristic metachromatic histologic
staining. The most common form of MLD (50% to 80%) is
Figure 137-3 the late infantile form. Typical MRI findings in this form of
290

Case 137 291
the disease include symmetric confluent T2 hyperintensity in or sooner. The adult form usually manifests as a psychiatric
the corona radiata and the centrum semiovale. There is pos- disorder or progressive dementia after the age of 16 years.
terior predominance and sparing of the subcortical U fibers. There is no cure for MLD. Care is mostly supportive.
There is occasional cerebellar WM involvement in late stages
of the disease. Various portions of the corpus callosum, Question for Further Thought
the internal capsules, and the claustrum may be involved. 1. What is the appropriate workup for patients with sus-
Multiple linear and punctate radiating hypointensities, so- pected MLD?
called tigroid pattern, are usually present within the confluent
WM hyperintensities. There is usually no contrast enhance- Reporting Responsibility
ment. An MRI severity scoring system has been proposed to Although there is no cure for this disease and it is not an acute
provide a measure of brain involvement in this disease. situation, a direct report may be appreciated so that appropri-
MLD is a rare disease directly caused by a deficiency ate genetic testing to confirm the diagnosis may begin.
of the enzyme arylsulfatase A (ASA) and is characterized
by enzyme activity that is less than 10% of human controls What the Treating Physician Needs to Know
leading to buildup of sulfatides in many tissues of the body This is a heritable disorder. The families of patients with
and in the brain eventually destroying the myelin sheath. The suspected MLD should be referred for genetic counseling
ARSA gene responsible for ASA is located on the long arm Progression of disease on follow-up
of chromosome 22. It has autosomal recessive inheritance.
More than 110 mutations have been identified in the ARSA Answer
gene resulting in MLD. 1. Urine and blood samples are required for a formal diag-
Three forms of the disease are recognized: late infan- nosis of MLD (an MRI is not definitive in diagnosing
tile MLD with symptoms usually beginning by ages 1 to the disease because the appearance may overlap with
2 years, juvenile MLD with symptoms usually beginning many other conditions). The blood ASA enzyme levels
between ages 4 and 12 years, adult (and late-stage juvenile are measured first, followed by urine sulfatide levels. An
MLD) with symptoms occurring between age 14 and adult- increased amount of sulfatide in the urine indicates that
hood (over age 16), but may begin as late as the 40s or 50s. the ASA enzyme is not breaking down sulfatides, which
Symptoms include muscle wasting and weakness, muscle usually suggests MLD. A low level of ASA enzyme in the
rigidity, developmental delays, progressive loss of vision blood is not a definitive test for MLD because these same
leading to blindness, convulsions, impaired swallowing, low levels of ASA are also found in a condition called
paralysis, and dementia and eventually coma. Untreated, ASA pseudo-deficiency prevalent in up to 5% of people
most children with this form of MLD die by the age of 5 with European ancestry.

Case
FINDINGS Figure 138-1. Axial NCCT through the vertex.

There is hyperdensity within the right central sulcus (large
arrow) consistent with subarachnoid hemorrhage (SAH).
There is similar but smudgy hyperdensity along the right
superior frontal sulcus (smaller anterior arrow). There is a
right parietal thin acute subdural hematoma (SDH) (chev-
ron) and a left parietal subgaleal hematoma (vertical arrow).
Figure 138-2. Axial MRI FLAIR through the vertex 6 days
after Figure 138-1. There are multiple sulcal hyperintensi-
ties in bilateral cerebral convexities (arrows) consistent with
SAH. Figure 138-3. Axial GRE through the same level as in
Figure 138-2. There are bilateral sulcal hypointensities cor-
responding to the SAH locations in Figure 138-2 (arrows).
Bilateral subgaleal collections have grown in the interval.
DIFFERENTIAL DIAGNOSIS
SAH, cortical vein
thrombosis.
DIAGNOSIS Traumatic subarachnoid hemorrhage (TSAH).
DISCUSSION TSAH is best evaluated by CT in the acute

stage. The typical CT findings are multifocal hyperdensity
Figure 138-3 within the sulci and subarachnoid space (SAS). Large volume
292

Case 138 293
SAH is usually a diffuse hyperdensity within the SAS fol- Question for Further Thought
lowing the brain surface pattern and not difficult to diagnose. 1. How is TSAH different than aneurysmal SAH?
TSAH are more common in the convexities and the fissures.
Subtle changes could include apparent effacement of cortical
sulci either focally or diffusely. This may happen with small
TSAH or any SAH for that matter requires direct reporting.
volume SAH or diluted SAH. Over time the SAH is diluted
Location, size, and associated injuries such as contusions,
by cerebrospinal fluid (CSF) and it may be difficult to identify
hematomas, DAI, IVH, herniations, and extraaxial hemor-
on CT. Traces of hemorrhage could be present in the inter-
rhages should be reported. Locations that may suggest preex-
peduncular, perimesencephalic, and quadrigeminal cisterns as
isting disease such as aneurysms and vascular malformations
well as along the falx. As demonstrated here, the MRI in the
may benefit from MRI/MRA. Complications on follow-up
subacute and chronic setting is very sensitive to SAH even
such as increase in size, IVH, hydrocephalus, and ischemia
long after it could not be visualized on CT. MRI tends to dem-
should also be directly reported.
onstrate hemorrhages that are not visualized on the initial CT.
The diamagnetic and paramagnetic materials in degenerated
blood in the SAS are bright on FLAIR and hypointense on What the Treating Physician Needs to Know
GRE and T2WI and usually appear to restrict diffusion on Location and size
DWI. Differential diagnosis of FLAIR sulcal hyperintensities Associated injuries
may include meningitis or collateral vessels in the appropriate Complications
clinical setting. New imaging techniques such as SWI have MRI and MRA may be useful in the follow-up to establish
been shown to demonstrate more hemorrhages than seen on associated secondary injuries such as ischemia, IVH, and
CT. MRI is also useful in eliminating cortical vein thrombosis. SAH not visualized on initial CT
TSAH is fairly common occurring in up to 53% of head
injuries. Isolated TSAH may present with headaches and sei- Answer
zures. But TSAH is usually part of a more diffuse and severe
1. Location of the hemorrhage is key to differentiating
traumatic brain injury (TBI) where presentation may include
TSAH from aneurysmal SAH. TSAH is mostly convexity
loss of consciousness, focal neurologic deficit, and/or coma.
and fissural in location. There may be a small amount of
Brain contusions, brain hematoma, intraventricular hematoma
blood in the basal cisterns. Usually there are associated
(IVH), diffuse axonal injury (DAI), and extraaxial hemor-
primary injuries. IVH may occur in both. Cortical vas-
rhages are usually present in the same patient. Most of these
cular malformations or cortical vein thrombosis can also
patients present with a low Glasgow Coma Scale (GCS) score.
cause convexity SAH. Aneurysmal SAH is mostly basal
Patients are generally older. TSAH tends to be more common
in location with secondary diffusion to the convexities.
where the SAS is wide as in infants and elderly. It is due to
There usually are no other associated injuries unless the
traumatic rupture of leptomeningeal vessels and is associated
aneurysmal rupture occurs in the setting that can produce
with parenchymal contusions, hematomas, and SDH. It has
injuries such as rupture followed by a fall or a motor vehi-
been argued that TSAH either represents a severe form of TBI
cle collision. Suspicious location for aneurysmal SAH
or has direct deleterious effect on the brain by causing arte-
or suggestion of underlying pathologies warrants further
rial spasm, ischemia, and hydrocephalus. TSAH is generally
evaluation by MRI/MRA or CTA.
regarded as a marker of adverse outcome with a high rate of
morbidity and mortality. There is no known treatment.

Case
139 CLINICAL HISTORY Patient 1 (3-year-old male) and patient 2
(6-year-old male) both complain of headache and vomiting.
FINDINGS Figure 139-1. Axial post-contrast MR T1WI Choroid plexus carcinoma generally behaves more
through the posterior fossa. There is an intensely enhancing aggressively. Necrosis, cystic change, hemorrhage, invasion
rather knobbly or lumpy fourth ventricular mass surrounded of normal brain parenchyma, and brain edema are more com-
by a thin rim of CSF. Figure 139-2. Axial post-contrast monly seen with choroid plexus carcinoma. However, cho-
T1WI through the lateral ventricles in a different patient. roid plexus carcinoma can be indistinguishable from choroid
There is a similar rather lumpy avidly contrast-enhancing plexus papilloma on imaging.
mass with surrounding CSF within the right ventricular
atrium. Question for Further Thought
1. What is the prognosis for a patient with choroid plexus
DIFFERENTIAL DIAGNOSIS Choroid plexus papilloma, papilloma?
choroid plexus carcinoma, ependymoma, meningioma,
medulloblastoma. Reporting Responsibilities
Direct reporting of this tumor is essential. Note the very
DIAGNOSIS Choroid plexus papilloma. characteristic features of this intraventricular tumor. This
is one of the few occasions in brain tumor imaging when a
DISCUSSION In both cases, the tumor demonstrates a single entity may reasonably be offered as opposed to a dif-
very striking frond-like morphology. Notice also the severe ferential diagnosis.
hydrocephalus in patient 2, even though the tumor is not
located at a site likely to cause obstruction to the bilateral What the Treating Physician Needs to Know
lateral ventricles. Choroid plexus papilloma, in its typical Choroid plexus papilloma is one of the most common
form, is a tumor with benign behavior (WHO I), almost brain tumors encountered in young children
always found in young children. They arise wherever cho- The typical imaging features are very characteristic and
roid plexus normally exists, including the lateral ventricles lead to a confident imaging diagnosis
(excepting the frontal horns), the roof of the third ven- The hydrocephalus in patient 2 is probably due to overpro-
tricle, the fourth ventricle, and the foramina of Luschka. duction of CSF
Histologically, a choroid plexus papilloma is indistinguish-
able from normal choroid plexus. On gross inspection, Answer
the tumor is composed of tightly packed papillary projec- 1. Patients with typical choroid plexus papilloma (WHO I)
tions, resembling a head of cauliflower. An almost identi- have an excellent prognosis with 100% survival when
cal appearance is seen on MRI where the tumor shows a treated with definitive surgery. On rare occasions, more
distinctly papillary or frond-like morphology. The lesion aggressive histologic types can arise. The atypical papil-
is usually hyperintense on T2WI, shows internal vascular loma (WHO II) shows a tendency to recur after resection,
flow voids, and enhances intensely. Hydrocephalus is a but the survival is still nearly 100%. Choroid plexus car-
common associated finding, which usually reflects over- cinoma (WHO III), on the other hand, behaves in a more
production of cerebrospinal fluid (CSF) by the tumor rather malignant fashion with the potential for brain invasion
than obstruction. and dissemination. Prognosis for carcinoma is poor.
294

Case
140 CLINICAL HISTORY 51-year-old female with a 1-week history of
headache. She was brought into the emergency room after developing a new
severe headache with loss of consciousness.
Figure 140-2
are bilateral irregular, multilobulated middle cerebral

artery (MCA) bifurcation aneurysms with nipples (arrows).
The right MCA aneurysm has many tits and the neck incor-
porates the origins of all branches. There is irregularity and
multifocal constriction and dilation of the bilateral anterior
cerebral artery (ACA), MCA, and posterior cerebral artery
(PCA) (line arrows) consistent with vasospasm. Figure
140-3. Axial MIP CTA of the head. The two irregular
lobulated MCA aneurysms are again demonstrated (large
Figure 140-1 arrows) with irregularity of the arteries (line arrows) con-
sistent with vasospasm.
DIAGNOSIS MCA aneurysms (bilateral) with subarach-

noid hemorrhage and cerebral vasospasm (CVS).
DISCUSSION This patient highlights four important

points about aneurysm rupture: how to identify a ruptured
aneurysm in the presence of multiple aneurysms, the concept
of silent rupture, cerebrovascular spasm, and brain swelling
as complications. Multiple aneurysms are present in over
30% of patients with aneurysms. These are more common
in women than men in a ratio of 5:1. It is therefore important
to scrutinize the entire study for additional a neurysms once
one is found. It is always necessary to determine which aneu-
rysm has ruptured once multiple aneurysms are discovered
in a patient with subarachnoid hemorrhage (SAH). Since the
Figure 140-3
ruptured aneurysm will be treated first and urgently to pre-
vent further bleeding, it is imperative to identify the one that
ruptured. Helpful indicators pointing to a ruptured aneurysm
FINDINGS Figure 140-1. Axial NCCT through the syl- include (a) closeness to the highest concentration of SAH or
vian fissures. There is a rounded hyperdensity with periph- parenchymal hematoma; (b) larger aneurysm size; (c) aneu-
eral extensions in the left sylvian fissure (arrow) consistent rysmal wall irregularity such as lobulation, daughter aneu-
with hemorrhage. There is effacement of the convexity rysm, nipples, or tits; (d) local vasospasm; (e)serial change
sulci and basal cisterns, suggesting brain swelling and cen- in contour; and (f) obvious contrast extravasation during
tral herniation. Figure 140-2. Three-dimensional volume DSA or CTA. When there are multiple aneurysms, the ante-
rendering CTA head submentovertical (SMV) view. There rior communicating artery aneurysm is the most likely to
295

296 Case 140
have ruptured with the MCA aneurysm the least likely with hematoma, and hydrocephalus should all be carefully tabu-
all other sites somewhere in between. In this case, the con- lated. Other associated lesions coincidental or causative such
centration of SAH is in the left sylvian fissure, suggesting the as AVM and tumors should be reported. A statement should
left MCA aneurysm is the recent rupture. Both aneurysms be inserted, suggesting the aneurysm that has ruptured.
are about the same size and are both irregular in outline, sug-
gesting that both have probably ruptured. The irregularity of What the Treating Physician Needs to Know
the vessels is diffuse consistent with diffuse spasm indicating Location and number of aneurysms and SAH
at least a subacute rupture presumably from the right or both Which aneurysm has ruptured?
aneurysms since spasm tends to occur 3 to 5 days follow- Complications if any
ing SAH. Silent rupture or so-called warning leak may occur What imaging techniques best answer questions regarding
in about 50% of patients before the obviously symptomatic evaluation of primary and secondary lesions
one. The headache of the warning leak is not as severe as
the usual worse headache of my life that patient with SAH Answer
presents with. There may have been a warning leak a week 1. CVS is now the leading cause of poor outcome and death
before the present presentation in this patient. The efface- affecting about 20% of patients who survive the initial
ment of the sulci and basal cisterns is most probably due to ictus of SAH. The real reason for CVS remains a mystery!
brain swelling in the absence of hydrocephalus. This is pos- It is thought that the primary offending agents are blood
sibly related to ischemic complications of the CVS. products within the subarachnoid space. These products
probably set up a cascade of activities driven by several
Question for Further Thought chemicals acting together to cause CVS. These processes
1. What is the pathogenesis of vasospasm in SAH? may include calcium-dependent and calcium-independent
vasoconstriction, effects of chemicals such as superox-
Reporting Responsibilities ide free radicals, endothelins, nitrous oxide, and protein
This is an acute situation requiring direct reporting. The kinase. A disruption of vascular tone autoregulation, endo-
number of aneurysms, locations of aneurysms and SAH, thelial proliferation, and apoptosis along with inflamma-
direction of projection, aneurysm size with measurements, tory cascades have all been implicated in the development
and relationship to vascular branches and surrounding struc- of CVS. Treatment options include nimodipine, hyperten-
tures should be reported. Complications such as vasospasm, sive hypervolemic therapy, and balloon angioplasty along
brain swelling, infarction, parenchymal or other extraaxial with management of the ischemic complications.

Case
FINDINGS Figure 141-1. Axial NCCT through fourth ven- line cyst (star). Cerebellum is rotated and compressed later-
tricle. Large fourth ventricle ballooned into a large dorsal ally (arrows). There is effacement of the subarachnoid space.
posterior fossa cyst (star) with remodeling of the occipi- Figure 141-4. Coronal T2WI through the fourth ventricle.
tal bone. The cerebellum is compressed anterolaterally The fourth ventricle opens into a dorsal midline cyst (arrow).
(arrows). The third (chevron) and lateral ventricles (verti- Cerebellum is compressed laterally. The lateral ventricles are
cal arrows) are severely dilated. Figure 141-2. Sagittal MR dilated (stars).
T1WI through the fourth ventricle. There is a large posterior
fossa cyst (star) compressing the brainstem and displacing DIFFERENTIAL DIAGNOSISDandy-Walker malforma-
the torcula upward (arrow). The cyst extends through the tion (DWM), mega cisterna magna (MCM), posterior fossa
foramen magnum (transverse arrow). The third and lateral arachnoid cyst (PFAC).
ventricles are dilated. Figure 141-3. Axial T2WI through the
fourth ventricle. The fourth ventricle opens into a dorsal mid- DIAGNOSIS Dandy-Walker malformation (DWM).
297

298 Case 141
DISCUSSION The imaging findings of DWM include a be high in mild cases however. In utero diagnosis could be
large posterior fossa, a dilated fourth ventricle, with no rec- made by US and fetal MRI. The clinical outcome depends
ognizable fastigium, that communicates with a large dorsal on the severity of the malformation and associated changes
posterior fossa cyst, small or hypoplastic cerebellum dis- elsewhere in the brain. Mild DWM is compatible with long
placed and compressed anterolaterally by the large dorsal life, while a severe form with hydrocephalus requires either
cyst, superiorly displaced tentorium, and high anchoring of endoscopic third ventriculostomy, ventriculoperitoneal
the torcula and transverse sinuses also known as torcular- shunt alone, or in combination with cyst shunting.
lambdoid inversion. The brainstem is usually normal but
compressed against the clivus. Hydrocephalus occurs in over Question for Further Thought
80% of these patients. There may be a variety of corpus cal- 1. Is there a genetic basis for DWM?
losum and cortical malformations. The severity of DWM
varies from mild to severe depending on the size of the cyst Reporting Responsibilities
and accompanying complications. The diagnosis can easily Unless there is complicating hydrocephalus, routine reporting
be made on CT as in this case, but MRI offers the best oppor- is sufficient. Complicating hydrocephalus should be reported
tunity to demonstrate all the components of the malforma- directly to the referring physician. If the initial study was a
tion. MCM does not usually communicate with the fourth CT scan, patient may benefit from full categorization of the
ventricle other than via the foramen of Magendie and is usu- associated anomalies particularly the cortical malformations
ally retrocerebellar with veins and falx traversing it and vari- and dysgenetic corpus callosum by MRI. MRI should there-
able scalloping of the occipital bone. Retrocerebellar PFAC fore be recommended.
on the other hand usually has no veins and falx traversing
it and also does not communicate with the fourth ventricle. What the Treating Physician Needs to Know
Hydrocephalus is not a feature of MCM and PFAC. Presence of hydrocephalus
DWM is a posterior fossa malformation that has over DWM is one of the common hindbrain malformations, and
the years been described in many ways: Dandy-Walker the prognosis is good if it is mild
cyst, Dandy-Walker spectrum, Dandy-Walker variant, Associated malformations that may affect prognosis
and Dandy-Walker continuum. Dandy-Walker variant has Supratentorial anomalies could be present in up to two-
largely disappeared from recent writings because of its thirds of the patients with DWM. MRI is the imaging
ambiguous and imprecise definition. The spectrum and con- modality of choice for evaluation of these anomalies
tinuum address the degree of variability and severity. Infant
with DWM usually presents with a large head with mini- Answer
mal cerebellar signs. The underlying pathology mirrors the 1. Three genes are associated with DWM. FOXC1 is
imaging findings. Failure of proper development or atresia involved in mesenchymal-neuroepithelial signaling and
of the fourth ventricular foramina is linked to the ballooning only expressed in the cerebellum. ZIC1 and ZIC4 are
of the fourth ventricle with formation of a retrocerebellar involved in determination of cerebellar size and pat-
cyst. The high position of the torcula is explained on the tern of folia formation. Deficiencies in these three genes
basis of earlier development of the malformation before the have been linked to a variety of hindbrain malformations
torcula is properly anchored. The torcula does not have to including DWM.

Case
142 CLINICAL HISTORY 12-year-old male trauma victim.
299

300 Case 142
FINDINGS Figure 142-1. Axial NCCT through the lateral well demonstrated by in utero MRI. MR is more sensitive in
ventricles. There are bilateral suprasylvian coronal clefts lined detecting small cleft, the GM lining the lips, and the associ-
by cortical gray matter (GM) (arrows). The clefts extend from ated migrational malformations. The ventricle wall may be
the subarachnoid space to the lateral ventricular walls. This is tented pointing to the defect. The other associated findings
consistent with closed lip schizencephaly. The ventricles are include absent septum pellucidum in about two-thirds of the
abnormally shaped with nonvisualization of the septum pel- cases, absent or focal dysplasia or agenesis of the corpus
lucidum. Figure 142-2. Axial T2WI through the lateral ven- callosum, migrational malformations, Dandy-Walker mal-
tricles in a companion patient. There is a wide cleft through formations, and septooptic dysplasia. The separated thalami
the right cerebral hemisphere linking the wide open lateral differentiate this entity from lobar holoprosencephaly. A
ventricle to the subarachnoid space. The margin of the cleft porencephalic cyst is usually lined by gliotic tissue rather
is lined by cortical GM (arrows). This is an open lip schizen- than GM. Severe bilateral schizencephaly may be difficult
cephaly. The splenium of the corpus callosum and the septum to differentiate from hydranencephaly. Arachnoid cyst is an
pellucidum are absent. Figures 142-3 and 142-4. Axial NCCT extraaxial CSF containing mass that does not usually com-
through the lateral ventricles in another companion patient as municate with the ventricles.
a baby (Figure 142-3) and as a teenager (Figure142-4). There Schizencephaly is an uncommon disorder of neuronal
is a left frontal open lip schizencephaly lined by GM (vertical migration. The clinical presentation depends on the sever-
arrows) with overlying cranial vault remodeling (transverse ity of the malformation. Bilateral large lesions are the worst
arrows). The septum pellucidum is absent. with severe CNS impairment. Presentations include seizures,
developmental delay, hemiparesis, quadriplegia, and mental
DIFFERENTIAL DIAGNOSIS Porencephaly, holoprosen- retardation. Treatment is mainly supportive.
cephaly, hydranencephaly, arachnoid cyst, schizencephaly.
DIAGNOSIS Schizencephaly. 1. In what way does in utero diagnosis contribute to man-
agement of schizencephaly?
strating the changes of schizencephaly, which is basically Reporting Responsibilities
a cerebral hemisphere cleft that connects the subarachnoid Routine reporting is sufficient. Associated anomalies should
space with the ventricle. This cleft is lined by GM. There are be itemized for prognostication purpose.
basically two types: the closed lip (type I) and the open lip
(type II). In the closed lip, the GM-lined lips are in contact
with each other as in Figure 142-1. The lips are widely sepa-
rated with cerebrospinal fluid (CSF) in between in typeII Severity of malformation and other associated findings
as in Figures 142-2 to 142-4. In utero diagnosis of schizen- This is not hydrocephalus but may coexist with hydro-
cephaly is possible by both ultrasound and MRI. The closed cephalus
lip could pose a challenge during in utero diagnosis. In utero
ultrasound of the open lip schizencephaly presents a wedge- Answer
shaped defect with echogenic cortex lining the cleft. The 1. There is no cure for schizencephaly. Prenatal diagnosis is
defect could be unilateral or bilateral, of varying sizes from able to define the severity of the malformation. This could
small to very large and extends from the pial surface to the be useful in prognostication. This will help in manage-
ventricle wall. The septum pellucidum is usually absent and ment of the pregnancy and counseling the parents regard-
the thalami are separate in most cases. These changes are also ing real expectations once the child is born.

Case
FINDINGS Figure 143-1. Skull film PA view. There are extraosseous and osseous locations, though bony disease
innumerable lucent lesions throughout the calvarium (arrows is most common. On plain films, these can be seen as scat-
point to some of them). Figure 143-2. Axial NCCT through tered, well-defined lucent lesions without sclerotic margins.
the vault. There are corresponding multiple hypodense cra- Where one or two lesions are recognized on plain films, there
nial vault lesions, some well-defined, and all without sclero- are probably many others not yet visible as it takes the loss
sis. Figure 143-3. Coronal reconstruction of CECT centered of about 50% of the bone substance to manifest as lucency
on one lesion with a prominent extraosseous subgaleal soft on plain films. CT is of course more sensitive in this regard
tissue component (arrow). Figure 143-4. Axial post-contrast showing multifocal cranial vault or skull base hypodense
T1WI. There are multiple contrast-enhancing scattered intra- lesions which enhance following contrast administration.
diploic lesions (arrows point to some of them). MRI is the test of choice to detect subtle lesions. They appear
as areas of T1 and T2 intermediate intensity marrow replace-
DIFFERENTIAL DIAGNOSISPaget disease, metastases, ment with enhancement. Myeloma can produce soft tissue
multiple myeloma, lymphoma. mass of considerable size.
Middle-age and older adults are typically affected, and
DIAGNOSIS Multiple myeloma. median survival with therapy is on the order of several years.
Histologically, multiple myeloma presents as small mono-
DISCUSSION Multiple myeloma is a disseminated malig- clonal populations of plasma cells found throughout the
nancy of plasma cells. Disease deposits can be found in both skeletal system.
301

302 Case 143
Questions for Further Thought Imaging findings are suggestive, but diagnosis relies on
1. What is the most appropriate imaging study to follow laboratory and pathology assessment
multiple myeloma? Distribution of lesions and complications such as sig-
2. How does multiple myeloma relate to plasmacytoma? nificant soft tissue masses, mass effect, and foraminal
compression
Direct reporting is essential in any malignant lesion. Describe Answers
the distribution of disease, and assess for any unusual mani- 1. Despite the lower sensitivity, plain films are most often
festation or complications. These might include extraosseous used in the follow-up of multiple myeloma. CT and MRI
disease, fractures, and encroachment on critical structures or are preferred to better assess cranial and spinal disease.
foramina by soft tissue tumor. Understand that while the imag- PET has a limited role, and bone scintigraphy has no role
ing findings of multiple myeloma are suggestive of the diagno- as these lesions are cold on bone scan.
sis, they are not specific. In the absence of histologically proven 2. Whereas multiple myeloma is a disease with multiple
disease, a differential must be given which would include other lesions, plasmacytoma is a single site of plasma cell tumor.
malignant etiologies, and depending on the appearance, even Histologically, they are the same. Plasmacytoma carries a
some benign etiologies such as hyperparathyroidism. slightly better prognosis than disseminated myeloma.

Multiple myeloma is a common neoplastic bony disease
of older adults

Case
144 CLINICAL HISTORY 36-year-old male with history of chronic neck pain
and smoking developed right homonymous hemianopsia. He had two recent
chiropractic neck manipulations.
FINDINGS Figure 144-1. Volume-rendering 3D CTA of the DIAGNOSIS Duplication of right vertebral artery with
neck in oblique projection. There are two origins to the right pseudoaneurysm.
vertebral artery from the right subclavian artery (white verti-
cal arrow).The two limbs of the duplicated vertebral artery DISCUSSION Duplication of the vertebral artery is a rare
join at C4 level. The limb with proximal origin runs extraspi- anatomic variant resulting in double origins of the verte-
nally within the carotid sheath just behind the right common bral artery emanating from the subclavian artery on either
carotid artery (CCA), while the distal limb behaved nor- side or in the case of a duplicated left vertebral artery; the
mally entering the right C6 foramen transversarium. There proximal origin could also arise from the aortic arch. The
is a small outpouch from the distal aspect of the proximal limb from the proximal origin usually runs in the carotid
extraspinal limb of the duplication just before the two limbs sheath as in this case and stays extraspinal for a variable
join at C4 (transverse black arrow) consistent with a pseudo- length before finally uniting with the limb from the distal
aneurysm. The left vertebral artery originates directly from origin which usually follows the normal vertebral artery
the aortic arch (chevron). There is also fenestration of the course entering the foramen transversarium at C6 or C7.
proximal basilar artery (not shown). Figure144-2. Oblique The point of union of the duplicated limbs is variable. The
projection MIP contrast-enhanced MRA of the neck. This outpouch from the proximal limb of the duplication here is
confirms the duplicated proximal right vertebral artery (ver- clearly a pseudoaneurysm suspected to be traumatic as a
tical black arrow) and the pseudoaneurysm distally in the consequence of chiropractic manipulation. There has been
proximal limb (transverse white arrow). MRI (not shown) reports of associated anomalies with duplicated vertebral
demonstrated acute infarctions in the left occipital lobe, left artery and these include the presence of aneurysm and arte-
medial thalamus, and right parietal lobe suggesting embolic rial fenestrations distally and intracranially, arterial dissec-
phenomenon. tion and in one case a kink at one of the origins suspected
to be responsible for dizziness in one patient. The vulner-
DIFFERENTIAL DIAGNOSIS N/A. ability of the duplicated limbs has been debated in the
303

304 Case 144
literature. It is suspected that there may be no abnormality Reporting Responsibilities

in the histologic composition of the wall of the duplicated The infarctions in the brain and the pseudoaneurysm are
segments but the extraspinal course of one of the limbs acute situations and should be reported promptly. Presence
may predispose it to injury. However, traumatic or spon- of other congenital or acquired anomalies should be reported.
taneous pseudoaneurysms have been known to occur in the
regular vertebral artery and therefore may not be due to What the Treating Physician Needs to Know
the extraspinal course of the duplicated limb. Interestingly Locations of the dual origins and point of union of the
the left vertebral artery originates from the aortic arch in duplicated vertebral artery
this patient and there is fenestration of the proximal basilar Location, measurements, and direction of projection of the
artery as well. pseudoaneurysm
Pain and cerebrovascular events are the two common Other associated anomalies if any
presentations of cerebrovascular arterial dissection/pseudoa-
neurysm as in this case. Management of such a case follows Answer
the regular management protocol for pseudoaneurysm. The 1. Duplication is applied to double origins of vessels while
duplicated limbs are usually smaller than their parent artery fenestration is applied to a normal-appearing vessel that
and the flow dynamics may also be different than the rou- splits up and then unites along its course before getting to its
tine. These situations may complicate the choice and route of final destination. There has been some report of histologic
endovascular treatment if such becomes necessary. differences and abnormalities in the composition of the
walls of fenestrated segments. It is expected that the dupli-
Question for Further Thought cated segments or limbs may not have any abnormality or
1. How is duplication different from fenestration? asymmetry in the histologic composition of their walls.

Case
145 CLINICAL HISTORY 38-year-old male with headache and dizziness.
FINDINGS Figure 145-1. Axial GRE through the lateral (arrows). Two left frontal lobe lesions show associated cor-
ventricles. There is a left lateral ventricular subependymal tical thickening of intermediate intensity. Figure 145-3.
round hypointensity (arrow). Figure 145-2. Axial FLAIR Axial post-contrast T1WI through the level of the centrum
image through the level of the centrum semiovale showing semiovale. There is a left subcortical flame-shaped contrast
multifocal radial white matter hyperintense bands radiating enhancement (arrow). Figure 145-4. Axial NCCT through
from the subcortical regions in bilateral cerebral hemispheres the lateral ventricles. There are bilateral subependymal
305

306 Case 145
Tubers tend to be isointense to hypointense on T1WI. About

10% of cortical/subcortical tubers may enhance following
intravenous contrast administration. There is a higher inci-
dence of contrast enhancement in subependymal giant cell
astrocytoma (SGCA) which occurs in about 1.7% to 26%
of patients with TSC. SGCAs are located in the caudotha-
lamic grove region, where they could obstruct the foramen of
Monro causing hydrocephalus. The calcifications without the
subcortical changes may resemble congenital toxoplasmosis.
Other abnormalities such as hippocampal abnormalities
of msiotemporal sclerosis (MTS) and hippocampal mal-
rotation (HIMAL) have been found in a subset of patients
with TSC. DTI has been useful in demonstrating additional
lesions in white matter (WM) tracts that appear visually nor-
mal, indicating a rather more extensive brain involvement
that may suggest not only abnormal myelination but may
correlate with the extent of the lesion load. CT demonstrates
the unmistakable ependymal calcifications which, how-
ever, could mimic congenital toxoplasmosis calcifications.
However, the associated subcortical tubers and the clinical
presentations tend to give the diagnosis a way.
Figure 145-5

1. What are the clinical manifestations of TSC?
hyperdensities consistent with calcifications (arrows).
Figure145-5. Axial NCCT through the left centrum semi- Reporting Responsibilities
ovale. There is a thick linear left frontal subcortical hyperin- Routine reporting is sufficient as this represents chronic
tensity consistent with a subcortical tuber (arrow). changes. However, presence of SGCA with hydrocephalus
should trigger direct reporting.
DIFFERENTIAL DIAGNOSISToxoplasmosis, tuberous
sclerosis complex (TSC). What the Treating Physician Needs to Know
Location of calcifications and tubers
DIAGNOSIS Tuberous sclerosis complex (TSC). Presence of complications such as SGCA and hydrocephalus
DISCUSSION MRI is the method of choice in the compre- Answer

hensive evaluation of the brain in TSC. The findings here 1. TSC is an autosomal dominant neurocutaneous disorder.
represent the classical MRI finding in tuberous sclerosis. The Neurologic manifestations include seizures, develop-
GRE is the best MRI sequence for demonstrating the hypoin- mental delays, and neuropsychiatric behaviors. There are
tense subependymal calcifications. Subependymal tubers diagnostic criteria formulated by the National Tuberous
are iso- to hyperintense on other sequences and are seen as Sclerosis Association. There are three categories of TSC:
mild protrusions into the lateral ventricles. Hemorrhages definite, probable, and possible TSC. The common fea-
may behave in a similar fashion but the other findings of tures, however, are the cutaneous lesions of shagreen
subcortical tubers on FLAIR and T2WI suggest otherwise. patch, facial and ungual fibromas, and hypomelanotic
The subcortical tubers are best demonstrated on the FLAIR macules. Central nervous system (CNS) changes include
and T2WI as multifocal hyperintensities of varying shapes subependymal nodules and cortical/subcortical tubers
and sizes mostly as subcortical bands radiating to the white and SGCA. Multiple retinal nodular hamartomas, car-
matter. Cortical tubers present as cortical thickening and are diac rhabdomyosarcomas, pulmonary changes, and renal
slightly hyperintense compared with the normal cortical gray angiomyolipoma are other findings. A family history is
matter on FLAIR and T2WI. There is usually no mass effect also important and genetic testing allows for the DNA
or surrounding edema. This differentiates tubers from lesions sequencing for TSC1 and TSC2 mutations in up to 80%
such as multifocal subcortical metastases or hemorrhages. of affected individuals.

Case
146 CLINICAL HISTORY Normal supratentorial DTI color directional maps.
IOFF (Green)
ILF (Green)
Sagittal stratum = IOFF + ILF + OR + SLF
Figure 146-1 Midbrain level
(IOFF)
Anterior commissure
ILF
Optic radiation Geniculocalcarine tract
Figure 146-2 Thalamic level
307

308 Case 146
Genu IC (Yellow/red)
Anterior limb IC (Green)
Genu IC (Green)
External capsule (different colors)
Posterior limb internal capsule (Blue)
Splenium (Yellow/red)
Figure 146-3 Basal ganglia level
SOFF (Green)
Body of corpus callosum (red)
Corona radiata (Blue)
SLF (Green) (contains the arcuate fasciculus)
Figure 146-4 Body of corpus callosum level

Case 146 309
Cingulum (Green)
Figure 146-5 Cingulum level above corpus callosum
FINDINGS All images are axial DTI color directional maps. right direction), while lateral segments are yellow or mixed
The color hue indicates direction of fiber tracts: green indi- color in view of the different directions of the fiber tracts.
cates anteroposterior direction; blue indicates craniocaudal or Figure 146-4. Level of the body of corpus callosum. The
superoinferior direction; red indicates left to right direction. anteroposteriorly directed SOFF and the SLF (arcuate) are
Figure 146-1. Level of the midbrain. Inferior occipito- green. The right to left fibers of the body of corpus callosum
frontal fasciculus (IOFF) connects the occipital to frontal is red. The corona radiata is blue because of its superoinfe-
lobe, and inferior longitudinal (occipitotemporal) fasciculus riorly directed fibers. Figure 146-5. Level above the body of
(ILF) connects the occipital lobes to the temporal lobes. The corpus callosum. The fibers of the cingulum are green; they
sagittal stratum is a large complex white matter (WM) tract are anteroposteriorly directed.
combination of all the fibers that end up in the occipital lobe,
and these include IOFF, ILF, the optic radiation, superior DIFFERENTIAL DIAGNOSIS N/A.
longitudinal fasciculus (SLF) (connects frontal lobe cortex
to temporal, parietal, and occipital cortex) and the superior DIAGNOSIS Normal DTI WM directional maps.
occipitofrontal fasciculus (SOFF). Figure 146-2. Level of
the thalamus. Visible tracts include IOFF, ILF, and the optic DISCUSSION Since we used DTI directional maps in
radiation (geniculocalcarine). These fibers merge into the some of the cases in this book, we thought it is appropriate to
sagittal stratum. The anterior commissure is red at the level look at the normal anatomy of the WM fiber tracts so we can
of lamina terminalis. Figure 146-3. Level of the basal gan- understand their normal anatomical orientation. The detail of
glia. The anterior limb of the internal capsule (IC) is green the technique of DTI is beyond the scope of this book. WM
because of the anteroposteriorly directed frontopontine and fibers are classified into three categories: the association,
thalamocortical tracts. The posterior limb of the IC is blue the projection, and the commissural fibers. The Association
because of the superoinferiorly directed corticospinal, cor- fibers connect cortical areas in each hemisphere. These are
ticospontine, and corticobulbar tracts traveling down the the inferior and superior occipitofrontal fasciculi, superior
centrum semiovale and corona radiata. The external capsule and inferior longitudinal fasciculi, the cingulum and the
color varies from green posteriorly to blue anteriorly because uncinate (frontotemporal) fibers. The projection fibers con-
of the direction of the various components. The splenium and nect cortical areas to the spinal cord, cerebellum, brainstem,
genu of the corpus callosum have a red hue centrally (left to and central gray matter (GM). These include corticospinal,

310 Case 146
corticobulbar, corticopontine, and geniculocalcarine fibers. What the Treating Physician Needs to Know
The commissural fibers connect similar cortical areas in the Relationship of pathology to fiber tracts; are the fiber tracts
two hemispheres, and these include the corpus callosum and or cortical tissues displaced, disrupted, or infiltrated?
the anterior commissure. Additional DTI tractography may be indicated along with
functional MRI
1. What are the uses of DTI? Answer
1. Uses include determination of infiltration, disruption, dis-
Reporting Responsibilities placement, or edema of the tracts by pathology such as
These maps cannot be interpreted meaningfully without the tumors, infarcts, demyelination, infection, etc. DTI could
structural imaging studies, and the DTI tractography maps be useful in monitoring response to therapy and guidance
may also be useful. Displacement, disordered FA, and infil- during surgery to prevent or minimize damage to normal
tration should be reported. tissue or to steer clear of vulnerable tracts.

Case
147 CLINICAL HISTORY This patient (like many others with these lesions) presented
after cardiorespiratory arrest and prolonged resuscitation. He was unconscious with
only brainstem reflexes and signs of increased intracranial pressure.
311

312 Case 147
hypoxic-ischemic-related changes. On CT, basal ganglia

hypodensity is one of the first findings to become obvi-
ous, and other findings include cortical GM hypodensity,
loss of normal GMWM differentiation, and effacement
of convexity cerebrospinal fluid (CSF) spaces due to brain
swelling. During the early subacute phase MRI can reveal
swollen basal ganglia with subtle hyperintensity on T2WI,
and this signal abnormality persists into the chronic phase.
Proton MRS can demonstrate a lactate peak or a high
glutamineglutamate peak. In the late subacute period
the findings of post anoxic leukoencephalopathy are pres-
ent with WM T2 hyperintensity which may also show
restricted diffusion. Finally, in the chronic stage diffuse
atrophy is seen.
Nonperinatal HIE is mainly caused by cardiac arrest or
cerebrovascular disease in adults and drowning or asphyxi-
ation in the pediatric population. In the majority of patients
the clinical findings are usually evident and correlate well
with imaging abnormalities. This depends on the severity
of the insult, the age of the patient, the type and timing of
imaging studies. HIE involving the basal ganglia is usually
secondary to hypoperfusion of the basal ganglia. Global HIE
results from cardiorespiratory failure and involve basal gan-
glia, the hippocampi, and the cerebral cortex initially. When
the cortex is compromised, the motor strip and calcarine
regions tend to be affected first. Generally, GM structures
Figure 147-5
tend to be more vulnerable to HIE because of their high
basal metabolism and concentration of excitatory substances
FINDINGS Figure 147-1. Axial NCCT through the basal like glutamate normally found at the postsynaptic gaps. The
ganglia. There are bilateral symmetrical hypodense lesions prognosis is grave with the patient remaining in vegetative
in the head of the caudate nuclei (CN) and lentiform nuclei state if he/she survives.
(arrows). Figure 147-2. Axial NCCT through the corona
radiata. The CN hypodensity extends into the bodies of the Questions for Further Thought
CN (arrows). There is effacement of the cortical sulci and 1. What is the clinical picture after a basal ganglia/internal
poor hemispheric graywhite matter (GMWM) differentia- capsule injury?
tion. Figure 147-3. Axial T2WI through the basal ganglia. 2. Name three imaging HIE signs or findings related to a
There is symmetrical bilateral corpus striatum hyperinten- poor outcome?
sity (vertical arrows). There is also bilateral diffuse cortical 3. What basal ganglia structures are included in the Alberta
cerebral hyperintensity and effacement of convexity sulci Stroke Program Early CT Score (ASPECTS)?
(transverse arrows). There is hyperintensity of posterome-
dial bilateral thalami (arrowheads). Figures 147-4 and 147-5.
Axial DWI and ADC map through the basal ganglia. There
Direct reporting is essential in this acute setting. It is impor-
are bilateral symmetrical basal ganglia, thalamic and cortical
tant to exclude intracranial hemorrhage, identify changes
GM diffusion restriction.
secondary to HIE, and describe signs of accompanying ele-
vated intracranial pressure, such as midline shift or findings
DIFFERENTIAL DIAGNOSIS Carbon monoxide poison-
of significant herniation, all of which have a bearing on the
ing, methanol poisoning, hypoxic-ischemic encephalopa-
prognosis.
thy (HIE) Wilson disease (early stage), hyperammonemia
(acute hepatic decompensation), hypoglycemia, and osmotic
demyelination. What the Treating Physician Needs to Know
In the acute setting, physicians need to exclude HIE mim-
DIAGNOSIS Hypoxic-ischemic encephalopathy (HIE). ickers such as intracranial hematomas
Extent of the HIE
DISCUSSION Although CT is usually the initial imag- Probable compromised and salvageable surrounding
ing study used to evaluate HIE in the acute setting, diffu- parenchyma
sion-weighted MRI is the technique that shows very early Later extension of the injury and probable complications

Case 147 313
Answers easuring the relative HU, is associated with a vegeta-

m
1. Patients with basal ganglia/internal capsule injury are tive state if less than 1.22 and to brain death if lower than
more likely to have hypotonia, flaccid paralysis, persis- 1.18to 1.19.
tent impaired balance, and ambulation performance. 3. The caudate nucleus, the lentiform nucleus, and the
2. Pseudo-subarachnoid hemorrhage sign, reversal sign, nearby posterior limb of the internal capsule are included
and white cerebellar sign are all related to a poor out- as middle cerebral artery territory regions defined by the
come. The ratio of GM/WM differentiation, obtained by ASPECTS.

Case
148 CLINICAL HISTORY Young traveling sales female found wandering in
the local airport and was brought to the emergency room.

314

Case 148 315
Figure 148-6
DISCUSSION DTE lesions consist of multifocal patchy

hypodense lesions on CT with or without contrast enhance-
ment. There are multifocal T2 hyperintense masses of varying
sizes in the thalamus, basal ganglia, cerebellum, and cortical
and subcortical cerebral regions on MRI. Usually there is no
Figure 148-5
restriction of diffusion and mass effect is minimal. Contrast
enhancement is usually none to minimal. Hemorrhagic com-
plications have been reported and may be the initial presenting
FINDINGS Figure 148-1. Axial NCCT through the basal imaging findings. There is associated leptomeningeal enhance-
ganglia. There are multifocal patchy hypodensities in bilat- ment. It could be difficult excluding ADEM. However, lep-
eral thalami, basal ganglia, frontal, and peritrigonal white tomeningeal enhancement is not a usual feature of ADEM.
matter (WM). Similar hypodensity is present in the cer- Metastases are contrast-enhancing lesions with inordinate
ebellum (arrow). Figures 148-2 and 148-3. Axial DWI and amount of edema with significant mass effect. PCNSL shows
ADC map through basal ganglia. There are multiple bilat- ring contrast enhancement in the AIDS patient, restricts dif-
eral thalamic/basal ganglia (arrows), subcortical/cortical fusion and usually T2 hypointense because of its cellularity.
frontal, and posterior temporal hyperintense (non-diffusion DTE is a rapidly fatal disease caused by the parasite
restricting) masses of varying sizes with minimal local mass Toxoplasma gondii. DTE is associated with toxoplasmosis of
effect. Figures 148-4 and 148-5. Axial FLAIR through the other organs such as retinochoroiditis, pneumonia, and mul-
posterior fossa (Figure 148-4) and through the bilateral basal tifocal organ system failure. Disseminated infection, more
ganglia (Figure 148-5). There are multiple small to large common in immunocompromised individuals (patients with
fairly well-defined irregularly marginated somewhat smudgy HIV infection or transplant recipients), is a rare event but
hyperintense masses in the brainstem, cerebellum, thalami, in most cases results in a fatal outcome. It was the AIDS-
basal ganglia, and bilateral frontal and temporoparietal corti- defining condition in this patient who was subsequently
cal subcortical regions. Figure 148-6. Coronal post contrast discovered to be HIV positive and to have toxoplasma car-
T1WI through the basal ganglia. This demonstrates one of diomyopathy. Most patients present with symptoms and
the very few rather thick smooth ring contrast-enhancing signs of encephalopathy/encephalitis such as confusion,
lesions in the left thalamus (transverse arrow). It contains a lethargy, mental status changes, fever, headache, or focal
tiny cleft of isointensity. There is also evidence of leptomen- neurologic deficit. Seizure may be a feature. The majority
ingeal enhancement (vertical arrows). of clinicians rely initially on an empiric diagnosis of toxo-
plasma encephalitis (based on serology Toxoplasma DNA by
DIFFERENTIAL DIAGNOSISMeningoencephalitis, acute polymerase chain reaction (PCR) and imaging), which can
disseminated encephalomyelitis (ADEM), disseminated toxo be confirmed on the basis of clinical and imaging improve-
plasmosis, metastases, primary CNS lymphoma (PCNSL). ment with specific anti-T. gondii therapy in the absence of a
likely alternative diagnosis. Brain biopsy is mainly reserved
DIAGNOSIS Disseminated toxoplasmosis encephalitis (DTE) for patients who have negative serology or fail to respond to
in HIV. specific antitoxoplasmosis therapy.

316 Case 148
Question for Further Thought Is lumbar puncture (LP) safe? Presence of significant mass
1. What is the treatment of choice? effect may contraindicate LP
If lymphoma is a consideration, steroid may need to be
Reporting Responsibilities withheld prior to biopsy
This is an emergent condition requiring direct reporting.
Significant mass effect should be reported to prevent inad-
vertent LP in brain herniation. Answer
1. Usually a combination of pyrimethamine and sulfadiazine
What the Treating Physician Needs to Know is very effective against the protozoa. The treatment is
Location of surgically reachable lesion if biopsy becomes administered 1 to 2 weeks beyond the resolution of clini-
necessary cal signs and symptoms.

Case
149 CLINICAL HISTORY 59-year-old male presented with headache.
317

318 Case 149
Figure 149-5
FINDINGS Figure 149-1. Axial T1WI through the corona

radiata. There are bilateral almost symmetrical crescentic
extraaxial hypointense collectionsalmost cerebrospinal Figure 149-6
fluid (CSF) intensity (arrows). There is bilateral convexity
sulcal effacement. Figure 149-2. Axial FLAIR through the
same level. The bilateral extraaxial collections are hyper- thickening and avid enhancement of the outer layer of the
intense, higher than CSF intensity (arrows). Figure 149-3. encapsulating membrane (pachymeninges laterally) in this
Axial T2WI through the same level. The collections are case with very little or no enhancement of the medial mem-
hyperintense (about CSF intensity) with some effacement of brane. CSDH generally do not restrict diffusion. Presence of
convexity sulci but no midline shift. Figure 149-4. Axial GRE diffusion restriction within it suggests a rebleed and or empy-
through the collections. There is blooming (arrows) within ema. MRI may also demonstrate underlying brain parenchy-
the medial membrane of the predominantly hyperintense mal changes or subarachnoid hemorrhage (SAH) related to
crescentic collections. This may suggest early calcification the original injury. SDG almost always follow CSF intensity
or acute blood product. Figure 149-5. Coronal post-con- on all sequences.
trast T1WI through the midbrain. There is bilateral smooth CSDH is generally hypodense on NCCT with membrane
pachymeningeal enhancement surrounding the hypointense enhancement following intravenous contrast administration.
extraaxial collections (arrows). There is no significant medial Depending on the size of the CSDH, there may be varying
membrane enhancement. Figure 149-6. Axial NCCT through degrees of sulcal effacement and midline shift. Bilateral bal-
the lateral ventricles. There are bilateral crescentic extraaxial anced collections as in this case rarely result in midline shift.
hypodense collectionsjust slightly higher than CSF den- However, bilateral large collections may result in central
sity (arrows). herniation. Sulcal effacement is always present. Organized
CSDH may become biconvex in shape with internal septa-
DIFFERENTIAL DIAGNOSISChronic subdural hema- tions and loculation. Some may even calcify as described
toma (CSDH), subdural hygroma (SDG). elsewhere in this book.
CSDH occurs in all ages and has no gender preference. It
DIAGNOSIS Chronic subdural hematoma (CSDH). is more common in chronic alcoholics and the elderly and has
been reported in dural metastases. Barring a rebleed which may
DISCUSSION Findings of CSDH on MRI depend on the occur in up to 10% of CSDHs, the collection will eventually
age of the hematoma. Most frequently the shape remains resorb leaving behind either no trace of underlying changes,
crescentic. The collection is hypointense to slightly hyperin- a thickened membrane, or in some cases a calcified plaque.
tense to CSF on T1WI, hyperintense to CSF on FLAIR, and Follow-up CT about 6 months later in this patient showed com-
mostly isointense or hyperintense to CSF on GRE and T2WI. plete resolution of the collections without any intervention.
GRE in this case shows some blooming of the medial mem-
brane suggesting either hemosiderin or calcifications within Question for Further Thought
the membrane. It may also represent acute bleed. There is 1. Is MRI essential for the diagnosis of CSDH?

Case 149 319
Reporting Responsibilities If a rebleed is present are there complications such as

Although this may not be an acute situation, direct reporting hydrocephalus, underlying brain edema, or herniations?
is necessary particularly because it was unexpected. Direct Is there underlying meningeal pathology such as metas-
reporting becomes even more crucial if there is herniation or tases?
significant mass effect and because of the potential to rebleed.
Other associated injuries discovered should be reported. Answer
1. Not really. CT is sufficient if there is no complication. A
What the Treating Physician Needs to Know rebleed or poor neurologic state despite adequate manage-
Location, size, structural displacements, or herniations and ment may require MRI to exclude underlying meningeal
presence of other injuries lesions or unsuspected brain parenchymal lesions such
Is the evolution of the SDH as expected or is there a as diffuse axonal injury (DAI) or other cerebrovascular
rebleed on follow-up? lesions.

Case
150 CLINICAL HISTORY 21-year-old female with dizziness, ataxia,
and left-sided hearing loss.
FINDINGS Figures 150-1 and 150-2. Axial DWI and and splenium of the CC (arrows) with no significant cal-
FLAIR MRI through the corpus callosum (CC). There lososeptal interface hyperintensity. The so-called snowball
are multifocal CC, bilateral periventricular and subcorti- lesion (vertical arrow) is a large round focal hyperintensity
cal hyperintense lesions with subtle lesions in the anterior in the CC. Figure150-4. Axial T2WI through the brachium
thalamus bilaterally, and right cingulate cortex (arrows). pontis. There is a medial left brachium pontis focal hyper-
Figure150-3. Sagittal FLAIR MRI through the CC show- intensity adjacent to the fourth ventricle (transverse arrow)
ing multiple round hyperintense lesions in the genu, body, and a small lesion anteriorly in the basis pontis on the right
320

Case 150 321
(vertical arrow). There were no obvious areas of restricted diagnosis. SS is confirmed by demonstrating branch retinal
diffusion on these images. Post-contrast images (not shown) artery occlusion (BRAO) by fluorescein angiogram. BRAO
did not reveal any abnormal contrast enhancement. is usually not painful in contrast to optic neuritis in MS. The
SNHL is sometimes associated with vertigo, tinnitus, and
DIFFERENTIAL DIAGNOSIS Susac syndrome (SS), vas- nystagmus. The treatment of SS involves treatment of exac-
culitis, multiple sclerosis (MS), acute disseminated encepha- erbations with high-dose corticosteroids and IVIG. Long-
lomyelitis (ADEM). term immunosuppressants may be required. The disease
tends to stabilize within 2 to 4 years with varying degrees of
DIAGNOSIS Susac syndrome (SS). residual impairment.
DISCUSSION Imaging findings in SS consist of multifocal Question for Further Thought

T2 hyperintensities in both white matter (WM) and the deep 1. Is a brain biopsy necessary to make the diagnosis of SS?
and cortical gray matter (GM) of the brain. The involvement
of the corona radiata and centrum semiovale and the CC is Reporting Responsibilities
present in all patients and the lesions are best seen on the This is usually not an acute situation and routine report-
FLAIR. Brachium pontis, brainstem, and cerebellar hemi- ing may be sufficient. In the presence of MRI findings
spheres are involved in between 30% and 52% of patients. suggestive of MS in a patient with encephalopathy, SS
Lesions in the CC preferentially affect the central region should be in the differential. Leptomeningeal enhance-
and are round resulting in snowball lesions, in contrast to ment and basal ganglia involvement are uncommon in MS
the callososeptal interface lesions found in MS. Dawson and should be highlighted. Imaging of the spinal cord is
fingers, the ovoid periventricular (perivenular) lesions char- clinically warranted.
acteristic of MS, are not common in SS. The deep GM of the
basal ganglia and thalami is affected (similar to ADEM and
vasculitis but in contrast to MS) in up to 70% of patients.
Contrast-enhancing lesions in both GM and WM could be Detailed description of lesions
seen in up to 70% of affected population. A very small per- Presence of leptomeningeal enhancement may prompt
centage of the lesions may restrict diffusion. Leptomeningeal CSF evaluation
enhancement is present in about 30% of SS patients and dis- CSF may occasionally show elevated IgG index or synthe-
tinguishes SS from MS or ADEM where leptomeningeal sis rate with oligoclonal band
enhancement does not often occur. An extensive blood and SS is an uncommon disorder and may not be on the radar
cerebrospinal fluid (CSF) workup is required to rule out other of the radiologist who is not privy to all the information.
vascular, neoplastic, toxic, and infectious diseases. A report of possible MS on the MRI in an encephalopathic
The etiology of SS is not definitely known but it is patient should result in clinical evaluation for SS
believed to be a microangiopathy with microinfarcts possi-
bly immune mediated and multisystemic in presentation. It Answer
is characterized by the triad of microangiopathy of the brain, 1. In the presence of the clinical triad of encephalopathy,
eye, and ear, resulting in encephalopathy, severe headache, hearing loss, and visual impairment, a brain biopsy is not
behavioral changes, mental status changes, and confusion warranted. In comatose patients who cannot cooperate
along with visual impairment and sensorineural hearing loss with an eye or ear examination, a biopsy may be indi-
(SNHL). It is more common in women than men and more cated to tailor treatment and differentiate SS from ADEM
commonly occurs between the third and sixth decades of life. or other forms of vasculopathy. Biopsy tends to show
All the three elements of the syndrome may not be present perivascular inflammation and microinfarctions without
initially; hence, imaging may be the pointer to the correct vessel wall necrosis.

Case
151 CLINICAL HISTORY 62-year-old female with clipped right middle
cerebral artery (MCA) bifurcation aneurysm for follow-up.
Figure 151-3
Figure 151-4
322

Case 151 323
subarachnoid, cerebral vasospasm, infarcts, and hydrocepha-

lus. CT, CTA, and MRA will resolve most of these problems.
Acute hemorrhage is better evaluated by CT. Parenchymal
infarcts are better evaluated by MRI. As shown in these
images, CTA and MRA are not adequate for evaluation of
completeness of clipped aneurysms due to significant arti-
facts from the metal. However, if the question is presence of
complications such as cerebral vasospasm in the aftermath
of a clipped aneurysm or evaluation of development of new
aneurysm elsewhere, CTA or MRA continues to be the first-
Figure 151-7 line examinations for these indications, but there is a general
preference for CTA. The 3D volume rendering offers the
best CTA image, and it does demonstrate continuity of ves-
sel and resolution of some of the artifacts, but question still
remains about complete visualization of the clipped aneu-
FINDINGS Figure 151-1. Lateral scout skull view for
rysm. In the literature, CTA has been judged to be superior to
NCCT. The metallic clips in the paraclinoid region (trans-
MRA for the evaluation of clipped cerebral aneurysms par-
verse arrow) represent the aneurysm clips. The craniotomy
ticularly in follow-up of aneurysm remnants, and stenosis or
bolts are present superiorly and anteriorly (vertical arrows).
cerebral vasospasm. DSA continues to be recommended for
Figure 151-2. Axial CTA through the clips. There are signifi-
comprehensive evaluation of the postsurgical clipped cere-
cant streak artifacts through the clipped aneurysm rendering
bral aneurysms to resolve any cases of diagnostic uncertainty
evaluation of completeness of clipping impossible. Figure
on noninvasive imaging.
151-3. 3D CTA volume rendering of the aneurysm and clip.
This offers the best way to show continuity of the vessel
and the clipped aneurysm (arrow). But there is still question
about how well the aneurysm has been obliterated. Figures 1. What is cerebral aneurysm clip made of?
151-4 and 151-5. Axial T2WI and 3D TOF MRA source
image, respectively, through the aneurysm clips. The clips Reporting Responsibilities
cast a large susceptibility hypointensity (arrows) on all MR Direct reporting is essential if there is an acute or negative
sequences. This artifact is largest on the GRE (not shown) finding. If completeness of aneurysm clipping cannot be
and 3D TOF images. Figures 151-6 and 151-7. MIP images determined, DSA should be the next logical step.
of the 3D TOF MRA and CE MRA of the head, respectively.
The only sign of the aneurysm clip is lack of visualization of What the Treating Physician Needs to Know
the region of the clipping (arrows) such that the clipped aneu- Is there any residual aneurysm or recanalization?
rysm and its surrounding vessels cannot be seen or assessed. Is your technique adequate for evaluation of completeness
of aneurysm coiling?
Answer
DIAGNOSIS Effect of aneurysm clips on vascular structures 1. The old aneurysm clips were made of ferromagnetic mate-
with different imaging modalities. rials, hence the reluctance to image these with MRI. Death
from torque and displacement of a ferromagnetic clip in
DISCUSSION Some aneurysms continue to be clipped an MR unit has been reported. Recently made aneurysm
despite the popularity of aneurysm coiling. There are specific clips are either cobalt or titanium alloys that may contain
indications for aneurysm clipping. There are several reasons parts of titanium, nickel, molybdenum, chrome, alumi-
to want follow-up imaging on the postoperative aneurysm. num, vanadium, iron, and cobalt in various combinations
Evaluation for completeness of clipping is probably the most as composed by various manufacturers. Titanium clips
important. There may be unexplained clinical deteriora- show less artifact and image distortion on CT or MRI, and
tion. This may be due to new bleed either parenchymal or they show no translational attraction in MR unit.

Case
152 CLINICAL HISTORY 24-year-old female with a 3-day history of right-
sided Jacksonian seizures.
FINDINGS ated effacement of left frontal sulci. Figure 152-2. Axial

Figure 152-1. Axial FLAIR through the vertex. There GRE through the superior sagittal sinus (SSS). There is
are thick curvilinear hyperintense foci (arrows) in the enlarged blooming within the anterior SSS (arrow) and
posterior left frontal parasagittal convexity with associ- within the left vein of Trolard. Figure 152-3. Sagittal
324

Case 152 325
p ost-contrast T1WI. There is iso- to hypointense signal Sinus thrombosis is an uncommon disease of adults. It is
with peripheral enhancement in the anterior SSS (arrows). more common in women than men. Sinus thrombosis affects
There is homogeneous opacification of the SSS posteri- the SSS and the transverse sinuses in the majority of patients
orly. Figure 152-4. 3D contrast-enhanced MRV. There is (70%). Risk factors include pregnancy, oral contraceptive
irregular severe narrowing of the anterior SSS (arrows) use, prothrombotic state/blood dyscrasias, dehydration, intra-
with smooth contrast opacification of the patent poste- cranial hypotension, infection, and malignancy. Presenting
rior SSS. There are multiple irregular collaterals over the symptoms and signs could be nonspecific but the common
frontal lobes. ones are headache in over 95%, seizures, focal neurologic
deficit, papilledema, decreased level of consciousness, and
DIFFERENTIAL DIAGNOSISSubarachnoid hemorrhage coma. The syndrome of benign intracranial hypertension
(SAH), cortical infarcts, SSS thrombosis. (BIH) (headache, visual disturbances, and papilledema) is
common. Treatment is usually anticoagulation and endovas-
DIAGNOSIS SSS thrombosis (acute). cular thrombolysis when deemed appropriate.
DISCUSSION MRI and contrast-enhanced MRV are the Question for Further Thought
examinations of choice for comprehensive evaluation of 1. How do you differentiate congenital hypoplasia of cerebral
SSS or other cerebral venous sinus thrombosis. The MRI venous sinus from chronic thrombosis?
demonstrates signal within the sinuses; ordinarily there
should be signal void due to flowing blood. Acute throm- Reporting Responsibilities
bosis tends to produce isointensity within the swollen Direct reporting is required. This is an acute situation. Pattern
sinuses on T1WI, which may become hyperintense over of occlusion and corresponding parenchymal hemorrhagic
time. FLAIR and T2WI show iso/hyperintensity within infarcts as well as mass effect should be reported. Presence
the sinuses. There is blooming within the sinuses and the of SAH should be mentioned.
associated cortical veins on GRE and SWI. Parenchymal
changes such as cortical subcortical white matter (WM)
hemorrhagic venous infarction are demonstrated on DWI
and ADC maps as areas of heterogeneous restricted diffu- Extent of thrombosis including all the venous sinuses
sion with the appropriate blooming on GRE. The hyper- involved
intensity of vasogenic edema is well demonstrated on the Pattern of infarction and complications such as SAH, mass
FLAIR and T2WI. SAH if present could produce sulcal effect, and herniations
hyperintensity on FLAIR. Post-contrast T1WI shows The diagnosis could be made on MRI but MRV confirms
peripheral enhancement of the clot. Parenchymal enhance- the diagnosis
ment may also be present. MRV demonstrates occlu- Progress of recanalization or progression of thrombosis on
sion, irregularity, or filling defects within the thrombosed follow-up
sinus along with collaterals, which are generally irregular Patients with suspected benign intracranial hypertension
superficial or transmedullary vessels. (BIH) should be evaluated for venous sinus thrombosis
NCCT may demonstrate sinus hyperdensity in about
20% of cases. The so-called empty delta sign refers to filling Answer
defect (clot) with peripheral enhancement within the sinus 1. It could be difficult differentiating a hypoplastic sinus
on contrast-enhanced axial CT. Hemorrhagic infarcts and from chronic thrombosis. Hypoplastic venous sinus is
SAH are usually hyperdense. The accuracy of CTA in dem- usually small to nonexistent with smooth outline and
onstrating the luminal filling defects and irregularities of the no filling defects or irregularities. There are usually no
sinuses has been assessed as similar to contrast MRV. DSA irregular collaterals. One of the transverse sinuses is the
is now rarely done for diagnosis and obtained if endovascu- most likely to be hypoplastic with preferential drainage
lar treatment is contemplated. through the dominant sinus.

Case
153 CLINICAL HISTORY 12-year-old male presenting with headache.
FINDINGS Figure 153-1. Axial T2WI through the FLAIR through the mass shows complete signal suppres-
posterior fossa at the level of cerebellopontine angles sion in the lesion, which is isointense to cerebrospinal
(CPAs). There is a well-circumscribed hyperintense fluid (CSF). Figure 153-3. ADC image shows high ADC
extraaxial mass at the right CPA (star) causing mild mass without diffusion restriction. Figure 153-4. Axial post-
effect upon the adjacent medulla. There is smooth ero- contrast T1WI shows no appreciable enhancement in the
sion of the petrosal surface (arrow). Figure 153-2. Axial lesion.
326

Case 153 327
DIFFERENTIAL DIAGNOSIS Epidermoid cyst, neuroen- indications. Most arachnoid cysts do not enlarge over
teric cyst, arachnoid cyst, parasitic cyst, cystic neoplasm, that time and require no treatment. However, when they
is, schwannoma, meningioma, or metastasis. become large, they can compress adjacent neural and/or
vascular structures and present with intracranial hyper-
DIAGNOSIS CPA arachnoid cyst. tension, seizures, or focal neurologic deficits. Rarely sud-
den expansion occurs due to intralesional hemorrhage or
DISCUSSION Uncomplicated arachnoid cysts have a secondary infection. Subdural hemorrhages in association
thin, imperceptible wall and appear as smoothly marginated, with arachnoid cysts are rarely reported.
extraaxial masses. The cyst is isodense to CSF on CT with
noncalcified, nonenhancing walls. The internal contents fol- Question for Further Thought
low CSF intensity on all MRI pulse sequences, demonstrat- 1. What is the most successful choice for therapy?
ing complete signal suppression on FLAIR without restricted
diffusion. These characteristic findings help to differenti-
ate arachnoid cysts from epidermoids, which are semisolid
Routine reporting is sufficient. Arachnoid cysts are usually
lesions with restricted diffusion that do not suppress on
incidental findings and are not treated surgically unless caus-
FLAIR. Unlike epidermoids that tend to insinuate into cister-
ing mass effect. Uncomplicated cysts have typical imaging
nal structures and engulf adjacent neurovascular structures,
features. If the imaging findings are diagnostic, one should
arachnoid cysts tend to displace them. CT may show expans-
avoid offering distracting differential diagnosis that may
ile remodeling of the petrous bone, a testament to their slowly
result in more aggressive management. Complicating fea-
growing and long-standing presence, especially in children.
tures such as hemorrhage, extreme mass effect, ventricu-
Cystic neoplasms involving the CPA tend to have at least
lar trapping, abnormal enhancement, or significant change
some tumoral enhancement with thick walls and possible
between studies should be immediately communicated to the
calcifications. Uncommonly, meningiomas and vestibular
referring clinician.
schwannomas may produce secondary arachnoid cysts due to
CSF trapping. Neuroenteric cysts can occur in the prepontine
cistern and in the midline and often contains proteinaceous What the Treating Physician Needs to Know
contents (hyperintense on T1WI). Although arachnoid cysts Location and size
typically do not communicate with subarachnoid space, CT Relationship to adjacent neural and vascular structures
cisternography or phase-contrast cine MR can be helpful to including cranial nerves V, VII, and VIII, basilar artery,
document any communication prior to surgery. vertebral artery, anterior inferior cerebellar artery (AICA),
Arachnoid cysts are benign congenital intra-arachnoid or posterior inferior cerebellar artery (PICA) loops
collections of CSF. The common locations are the middle Evidence of complications including infection, hemor-
cranial fossa, over the cerebral convexities, in the retrocere- rhage, and subdural hemorrhage
bellar cistern, and the perimesencephalic cistern. About 10%
to 20% occur in the posterior fossa, and the CPA is a common Answer
site. Most remain confined to the CPA with rare extensions 1. Most cases are asymptomatic and require no treatment.
into the IAC or extension dorsally adjacent to the brainstem. Surgery is reserved for cases where symptoms are clearly
Arachnoid cysts may be congenital or acquired. Congenital linked directly to the anatomic location of the arachnoid
cysts are thought to result from altered CSF dynamics in cyst. Endoscopic cyst decompression via fenestration is
utero. Acquired arachnoid cysts or loculations result from least invasive and preferred initial approach. It may be
arachnoid adhesions and trapping of CSF, frequently in asso- helpful to document communication with subarachnoid
ciation with extraaxial neoplasms or trauma. space prior to cyst fenestration. Radical cyst removal is
Arachnoid cysts are usually asymptomatic and are challenging due to the numerous neurovascular structures
incidentally discovered on imaging performed for other in the CPA.

Case
154 CLINICAL HISTORY Adult female on follow-up imaging for
multifocal cerebrovascular events.
FINDINGS Figure 154-1. Axial DWI through the trigones. a very thin rim of contrast enhancement (transverse arrow)
There is an ovoid hyperintense mass replacing the glomus presumably due to displaced choroid tissue. The right cho-
of the left choroid plexus (arrow). Figure 154-2. ADC map roid plexus is avidly contrast enhancing as usual (vertical
through the trigones. There is intermediate hypointensity arrow).
in the mass suggesting mildly restricted diffusion within
the trigonal mass (arrow). Figure 154-3. T2WI through the DIFFERENTIAL DIAGNOSIS Xanthogranulomatous degen
trigone. The choroid plexus mass is hyperintense (arrow) eration of the choroid plexus (XGDC), choroid plexus cyst,
hardly distinguishable from CSF. There are some linear infarction of the choroid plexus, epidermoid tumor, intraven-
hypointensity crisscrossing the mass. The right normal tricular neurocysticercosis.
choroid plexus is hypointense (vertical arrow). Figure 154-
4. Coronal post-contrast T1WI through the trigones. The DIAGNOSIS Xanthogranulomatous degeneration of the
left choroid plexus mass has similar intensity to CSF with choroid plexus (XGDC).
328

Case 154 329
DISCUSSION XGDC is very common. It is also referred crystals, and blood product ingested by mononuclear cells to
to as choroid plexus cyst in some publications. It is distinct produce the xanthomatous response. It is usually asymptom-
from CSF containing cysts described elsewhere in this book. atic, and it is usually not treated. Obstructive lesions particu-
It is usually located in the trigones of the lateral ventricle larly in the third ventricle could be surgically removed.
but may be seen in the other ventricles. It is bilateral in two-
thirds of the cases and usually less than 1 cm in size but Question for Further Thought
could assume enormous size. It mostly follows CSF inten- 1. What is the relationship between XGDC and antenatal
sity on spin echo sequences. It is hyperintense on DWI with choroid plexus cyst?
intermediate hypointensity on ADC map and should not be
confused with acute choroid plexus infarction which should
be extremely hypointense on ADC map. There is usually no
This is a benign lesion of no clinical significance and should
contrast enhancement with the T1WI displaying CSF inten-
not be mistaken for acute infarct of the choroid plexus.
sity. Calcification surrounding the cyst may impart irregu-
Routine reporting is sufficient. Strategically located XGDC
lar hypointensity on GRE and T2WI. NCCT shows a CSF
in the third ventricle may cause obstruction and hydrocepha-
density choroid plexus mass which may or may not have
lus. Presence of hydrocephalus demands direct reporting.
surrounding hyperdense fragmentary shell of calcification.
Occasionally XGDC could be slightly hyperdense on CT.
There is usually no contrast enhancement. It may mimic epi-
dermoid tumor. Lack of contrast enhancement excludes the This is a benign lesion that should be left alone
usual choroid plexus tumors. True choroid plexus cyst (CSF Is there hydrocephalus? That may require treatment
containing) has CSF intensity on DWI. Neurocysticercosis
may not necessarily attach to the choroid plexus, could be Answer
multifocal within the ventricles, and change location with 1. There is no relationship between the two. Antenatal cho-
different head position. roid plexus cyst is usually small and has CSF echogenicity
XGDC is an incidental benign lesion occurring in all ages at US. It usually regresses after birth. It has been found to
but mostly in adults without gender discrimination. It is com- be more common in fetuses with trisomy 18 and other
posed of desquamated cellular debris with cholesterol, lipid congenital lesions.

Case
155 CLINICAL HISTORY 41-year-old male fell from a height. He was
stuporous on arrival.
FINDINGS Figure 155-1. Axial NCCT through the basal DISCUSSION SFH is the most common brain herniation.
ganglia. There is a left hemispheric mixed density (hyper- The cause of SFH in this patient is the large left hemispheric
acute) subdural hematoma (SDH) (transverse arrows). There hyperacute SDH. SFH is easily and readily imaged by CT.
is also some subarachnoid hemorrhage (SAH) beneath the There is usually a unilateral hemispheric mass, extraaxial
SDH. The midline structures such as the septum pellucidum, mass, or collection of bilateral hemispheric masses or col-
third ventricle, and the calcified pineal gland (vertical arrows) lection with a dominant side. This pushes the midline struc-
have moved to the right of midline. The third and left lateral tures of the septum pellucidum, third ventricle, pineal gland,
ventricles are compressed. Convexity sulci are effaced. There internal cerebral veins (ICVs), to the contralateral side away
is mild dilation of the right lateral ventricle (chevron). Figure from the dominant mass. A high convexity mass/collection
155-2. Axial NCCT through the corona radiata. This demon- may also push down on the ipsilateral cingulate gyrus, which
strates how to measure the shift; the septum pellucidum (verti- subsequently slides medially under the falx to the contralat-
cal arrow) represents the midline structure measured against eral side. These structures could therefore be found on the
a line connecting the anterior and posterior attachment of the contralateral side of the midline. There is usually compres-
falx. The falx itself may bend away from the side of the mass sion or effacement of the ipsilateral convexity sulci, lateral
or collection (transverse arrows point to the SDH). There is ventricle, and the third ventricle. A few complications can
dilation of the right lateral ventricle (chevron). occur. Obstruction at the level of the third ventricle and the
foramina of Monro prevents cerebrospinal fluid (CSF) from
DIFFERENTIAL DIAGNOSIS N/A. exiting the contralateral lateral ventricle resulting in trapping
or hydrocephalus of that ventricle. This could be associated
DIAGNOSIS Subfalcine herniation (SFH) due to acute with periventricular edema. Compression or wedging of the
subdural hematoma. ACA branches against the inferior edge of the falx by the
330

Case 155 331
herniating brain could produce stenosis orocclusion of the What the Treating Physician Needs to Know
pericallosal or callosomarginal arteries resulting in ischemic Size of shift measured in millimeters or centimeters
infarction in the ipsilateral ACA territory. If these vessels Offending mass or collection; location and size
rupture, SAH could occur. Compression of the ICV, vein of Other primary injuries such as fractures, epidural hema-
Galen, or the straight sinus could result in deep venous sys- toma (EDH), SDH, hygromas, contusions, hematoma, and
tem obstruction with venous congestion, further raised intra- diffuse axonal injury (DAI)
cranial pressure, and venous infarction. Although MRI is Complications and other associated herniations
not necessary to demonstrate SFH, it has a capacity to show
early complications of SFH. Answer
Massive SFH may also be associated with transtentorial
1. Paradoxical herniation is the phenomenon of SFH follow-
and ipsilateral uncal herniations. Patients usually show dete-
ing brain decompressive surgery without evidence of raised
rioration in clinical status. If the brainstem is compressed,
intracranial pressure or any extraaxial lesion that could
Duret hemorrhages may occur and cardiac and respira-
account for the herniation. Paradoxical herniation can occur
tory functions may be affected. Significant shift that alters
following a lumbar puncture, upright position, CSF leak,
patients clinical status may be an indication for decompres-
or dehydration in the presence of a large craniectomy. The
sive surgery.
explanation is that gravity on the decompressed brain creates
a negative pressure gradient between atmospheric and intra-
Question for Further Thought cranial pressure under these circumstances and this allows
1. What is paradoxical brain herniation? the brain to be sucked into the low-pressure infratentorial
compartment and through the foramen magnum. The clini-
Reporting Responsibilities cal condition tends to deteriorate suddenly. Focal neurologic
SFH is an emergency requiring direct reporting. The size deficit and mental status changes occur. The patient may
is usually measured in relation to displacement of midline become unarousable and comatose. It could be fatal. These
structures away from the midline. It is important that com- changes could also be delayed a few days after a lumbar
parison measurement should be done at about the same puncture (LP). The CT scan usually demonstrates a mid-
level. Whatever is causing the herniation should be docu- line shift or SFH with a sunken brain or a concavity of the
mented. Other primary intracranial injuries should be cat- brain at the site of craniectomy. Recommended treatment has
egorized. Complications such as infarcts, trapping of the included the Trendelenburg position, hydration, blood patch,
contralateral lateral ventricle, and significant changes on and early cranioplasty with varying degrees of success. It is
follow-up should be reported directly as well. There may be recommended that an LP be withheld in a patient who has a
other associated herniations in massive midline shifts such large craniectomy. If, however, an LP becomes necessary,
as uncal/transtentorial herniations that are all reportable. then it is done with the patient in the Trendelenburg position.

Case
156 CLINICAL HISTORY 6-year-old male with seizures, spastic quadriple-
gia, cortical blindness, nystagmus, and developmental delay.
FINDINGS Figure 156-1. Sagittal T1WI through the Figure 156-4. Coronal T2WI through the trigone. There is
posterior fossa. Small- to normal-sized posterior fossa. left colpocephaly with bilateral periventricular heteroto-
There is significantly reduced size of the cerebellum and pia (chevrons). Remnant of cerebellum/vermis dangles in
vermis with a small remnant (chevron) and no recogniz- the posterior fossa large CSF space (vertical arrow). There
able cerebellar hemispheres. The fourth ventricle opens is asymmetry of periventricular white matter (WM) with
posteriorly inferiorly into a large cerebrospinal fluid (CSF) thickening of cortical tissue (star).
space. There is a fastigium (arrow). The tectum is thick-
ened. The pons is small. The third ventricle (star) is large. DIFFERENTIAL DIAGNOSISCerebellar hypoplasia, Chiari
The splenium is not formed. Figure 156-2. Left parasagit- IV malformation (CIVM), Dandy-Walker malformation (DWM).
tal T1WI. There is an oblique cleft through the occipital
lobe (arrows). Figure 156-3. Coronal FLAIR through the DIAGNOSIS Cerebellar hypoplasia/CIVM.
hippocampus. Asymmetric orientation and thickened mal-
formed hippocampus (arrows). The right head of caudate DISCUSSION CIVM is a controversial entity. Some
is small compared with the left (transverse arrows). There consider CIVM and extreme cerebellar hypoplasia as
is asymmetry of the cerebellar remnants (vertical arrows). one and the same entity. The typical imaging findings are
332

Case 156 333
extremely small or absent cerebellum within an otherwise Question for Further Thought
CSF-filled normal- to small-sized posterior fossa, small 1. Are spine and spinal cord lesions part of the CIVM?
pons with absence of other features of CIIM or CIIIM.
In this particular case there is evidence of supratentorial Reporting Responsibilities
abnormality such as agenesis of the splenium, nodular het- Routine reporting is sufficient. Hydrocephalus is usually not
erotopia, colpocephaly, and occipital cleft. DWM usually a part of this entity.
has a large posterior fossa with high-riding tentorium and
torcula. The fastigium is usually absent, and the fourth What the Treating Physician Needs to Know
ventricle opens into the posterior fossa cyst in the midline. MR offers the best way to evaluate these patients
The cerebellum is present but small. Brainstem may be Sometimes it may be difficult to differentiate this entity
small to normal. from DWM
Cerebellar hypoplasia/CIVM is extremely rare, making it This entity is compatible with long life. However associ-
difficult to understand the natural history of the syndrome. ated abnormalities elsewhere as discovered in this case
Ataxia and developmental delay are some of the presenting may complicate the clinical picture
features. Incidental cases in adults who are being investigated
for mild neurologic symptoms have been reported. There Answer
are a host of associated congenital syndromes such as Very 1. No. Changes associated with CIM, CIIM, and CIIIM are
Low Density Lipoprotein Receptor (VLDLR)-associated usually not present in CIVM. Hence this entity is con-
cerebellar hypoplasia, Walker Warburg syndrome, Williams sidered not to belong to the Chiari malformation group.
syndrome, and the ataxia telangiectasia syndrome. The prog- However, isolated cervicomedullary abnormalities have
nosis is poor, and there is no known treatment. been reported in cerebellar hypoplasia.

Case
157 CLINICAL HISTORY 48-year-old female with headache, visual disturbance
flashing lights, occasional confusion, word finding difficulty, and seizures. She
is newly diagnosed with lung cancer on chemotherapy which included cisplatin.
Figure 157-2
Figure 157-1
Figure 157-3 Figure 157-4 (2 months later)
334

Case 157 335
FINDINGS Figure 157-1. Axial T2 FLAIR through the to imaging. Treatment is usually by withdrawal or reducing
lateral ventricles. There is bilateral parasagittal almost sym- the dose of the triggering medication which in this case is
metrical parieto-occipital subcortical patchy hyperinten- suspected to be cisplatin.
sities. There is a collection of hyperintense signal in the
splenium of the CC on the right (arrow). Figure 157-2. Axial Questions for Further Thought
T2 FLAIR through the high corona radiata. There is bilateral 1. Is hypertension (HTN) always a triggering factor for
almost symmetrical parasagittal parietal subcortical hyperin- PRES?
tensities (arrows). Additional left periventricular hyperinten- 2. What is the pathophysiology of PRES?
sities anteriorly. Figure 157-3. Coronal post-contrast T1WI
through the splenium of the CC. There is focal contrast Reporting Responsibilities
enhancement of the splenium on the right side (arrow) corre- Direct reporting is important in any situation where PRES is
sponding to the areas of FLAIR hyperintensity. Figure 157-4. suspected. Prompt withdrawal of the trigger prevents perma-
Axial T2 FLAIR through the splenium 2 months after the ini- nent damage.
tial study. There is resolution of the splenium and occipital/
parietal lesions. What the Treating Physician Needs to Know
Comprehensive categorization of all lesions and complica-
DIFFERENTIAL DIAGNOSISPosterior reversible ence tions which may include hemorrhage, mass effect, hernia-
phalopathy syndrome (PRES), acute disseminated encepha- tions, and hydrocephalus
lomyelitis (ADEM), metastases, lymphoma, cerebral venous PRES can occur in patients with complex systemic condi-
thrombosis, gliomatosis cerebri, multiple watershed infarcts. tions
The prognosis of PRES is usually benign if promptly rec-
DIAGNOSIS PRES of CC. ognized and treated. Atypical radiologic findings including
CC involvement should be recognized to avoid a delayed
DISCUSSION The specific interest in this case is PRES diagnosis
involvement of the CC. PRES can present with a variety
of imaging findings on CT and MRI. The typical lesions in Answers
PRES include bilateral symmetrical pattern of cortical/sub- 1. Acute HTN is commonly associated with PRES, but it is
cortical T2 hyperintensity in the parieto-occipital lobes that always neither sufficient nor necessary for PRES to occur.
may extend into the posterior temporal and frontal lobes in The severity of HTN in PRES cases is variable and not
a patchy and sometimes confluent fashion. There is usually extreme.
no restricted diffusion in the uncomplicated cases. However, 2. The pathophysiology of PRES remains incompletely under-
atypical distributions of T2 hyperintensity have been reported, stood. The most common theory is that acute HTN exceeds
and these include a unilateral pattern, involvement of the cer- the autoregulatory capacity of the cerebral vasculature
ebellum, brainstem, basal ganglia, and focal lesions of the resulting in hyperperfusion. The consequent breakdown
CC. CC involvement by PRES is usually in the splenium and of the bloodbrain barrier (BBB) leads to the formation
is present in about 10% of PRES population. Isolated CC of vasogenic edema. This theory likely explains the cases
PRES is unusual and could be easily confused with other dis- of PRES seen in association with hypertensive crisis and
eases such as lymphoma, toxic leukoencephalopathy (LE), most cases of eclampsia. PRES in patients without severe
infarct and demyelinating lesions. It usually exists as a part acute HTN (e.g., in cases of sepsis, organ transplantation,
of more widespread PRES lesions. Hemorrhage is increas- and in some patients receiving calcineurin inhibitors, such
ingly being recognized in PRES and does not exclude the as cyclosporine and tacrolimus and other chemotherapies)
diagnosis. Restricted diffusion is another atypical finding in may require other explanations. Endothelial damage and
PRES which should not preclude the diagnosis of PRES in BBB dysfunction is an attractive theory in the pathogen-
the appropriate clinical context. The location of lesions, pat- esis of this kind of PRES. The reported association of
tern of poor or no contrast enhancement, lack of restricted PRES with neuromyelitis optica spectrum disorders raises
diffusion allow convenient exclusion of the differential diag- the possibility of autoimmune-mediated disruption of the
noses. These changes may be seen as subcortical or cortical endothelium of aquaporin-4 water channels as a predis-
hypodensities on NCCT. posing condition for PRES. The association of PRES with
Clinical presentation consists of visual disturbance, con- bevacizumab, a monoclonal antibody against vascular
fusion, seizures, and headache. Recognizing that this may endothelial growth factor (VEGF) that inhibits angiogen-
represent PRES in the appropriate clinical setting should lead esis, is another supportive argument for that theory.

Case
158 CLINICAL HISTORY 50-year-old female with multiple sclerosis (MS) on
treatment with natalizumab after failed high-dose interferon-. She presented to
receive her 35th dose and reported a month history of headache and memory loss.
Figure 158-1
Figure 158-2
FINDINGS Figures158-1 and 158-2. Right parasagittal and Follow-up axial FLAIR at about 9 weeks. There is significant
axial FLAIR MRI through the corona radiata, respectively. progression of lesion in the right frontal lobe with extension
Baseline multifocal white matter (WM) hyperintensities of through the corpus callosum into the left frontal lobe (arrow).
MS. Figures 158-3 and 158-4. Axial and right parasagittal There is no significant mass effect. Figure 158-6. Axial non-
FLAIR, respectively, through the corona radiata at the time contrast T1WI through the level of the centrum semiovale.
of presentation with headache and memory loss. These show There is thin cortical gray matter (GM) T1 hyperintensity on
a large confluent right frontal mainly subcortical T2 hyperin- the lateral border of the lesion (arrows). Figure158-7. Post-
tense lesion without mass effect (arrows). Lesion was hypoin- contrast axial T1WI at this time. There is irregular patchy rim
tense on T1WI and did not contrast enhance. Figure158-5. contrast enhancement (CE) (arrows).
336

Case 158 337
best sequence for visualization of this lesion. CE has been

reported in natalizumab-induced PML while PML in HIV
may not show CE. CE is subtle, patchy, heterogeneous, or
linear and does not necessarily conform to the size or shape
of the PML lesion. More frequently CE is an indication
of immune reconstitution inflammatory syndrome (IRIS).
PML may affect the deep GM of the thalamus and basal
ganglia resembling ADEM. Infratentorial PML has prefer-
ences for the brachium pontis and the pons. Peripheral dif-
fusion restriction, T1 hyperintensity, and CE are all signs of
IRIS. One thing unique about PML is that it could be very
large with very little or no mass effect. DTI would show
decreased fractional anisotropy (FA) and WM architectural
disorganization. MRS usually demonstrates low N-acetyl
aspartate (NAA), high mIns, and high choline peaks. The
location, lack of typical CE, and size tend to differentiate
Figure 158-7 this entity from the underlying MS or tumefactive lesion.
Encephalitis tends to create mass effect.
PML is caused by a polyoma virus (John Cunningham
virus or JCV). An initial subclinical infection with JCV
DIFFERENTIAL DIAGNOSIS Progressive multifocal leu- is relatively common. A large segment of the population
koencephalopathy (PML), progression of MS, HIV encepha- harbors the virus. However, the virus only causes PML in
lopathy, encephalomyelitis. people with impaired immunity (lymphomas, HIV, and most
recently in patients on immunomodulators such as natali-
DIAGNOSIS PML due to natalizumab (n-PML). zumab, efalizumab, and rituximab). Clinical manifestations
of PML include progressive hemiparesis, clumsiness of
DISCUSSION PML is a monofocal or multifocal mostly limbs, disturbances of speech or vision, changes in think-
subcortical confluent lesion. It is hypointense on T1WI and ing, memory, confusion, and personality changes. Currently,
hyperintense on FLAIR and T2WI. FLAIR offers the single the confirmation of the diagnosis of n-PML relies on finding

338 Case 158
the DNA virus in the cerebrospinal fluid (CSF) using poly- What the Treating Physician Needs to Know
merase chain reaction (PCR) technique. Location, number, and size of lesions
Presence of CE that may suggest IRIS
Question for Further Thought Is lumbar puncture (LP) safe?
A negative MRI does not exclude PML. Clinical suspicion
1. What is the differential diagnosis of a worsening patient
should be followed by CSF evaluation using PCR technique
with n-PML after discontinuation of natalizumab?
Answer
Reporting Responsibilities 1. The differential diagnosis includes progression of PML,
Direct reporting is essential for prompt withdrawal of IRIS, or a rebound of MS. In IRIS, MRI shows a progres-
offending medication. Diagnosis of PML can be tricky if sive increase in lesion size, bilateral involvement, deep
lesion is small and in deep WM blending with underlying GM and brainstem involvement, and understandably CE.
MS. Suspicion is the key to the diagnosis. One can see pre-contrast hyperintense rim on T1WIs.

Case
159 CLINICAL HISTORY 1-year-old male with developing head mass.
FINDINGS Figure 159-1. Coronal T2WI through poste- DIAGNOSIS IH.

rior frontal bone. There is a left frontal scalp mass, mildly
hyperintense to brain, with dark foci suggesting flow voids DISCUSSION IH in the head is mostly cutaneous in loca-
(arrow). Figure 159-2. Axial T1WI through the lesion. The tion. It presents on CT as a well-defined soft tissue mass
mass is isointense to brain (arrow). Figure 159-3. Axial CTA confined to the scalp that enhances moderately to strongly.
source image. There is diffuse enhancement of the mass. The It may displace but usually does not invade structures.
underlying bone appears intact. Figure 159-4. Volume ren- MRI is the examination of choice for evaluation of the total
dered CTA. The lesion vascular supply is from branches of geography of IH. MRI tends to show isointense mass with
the external carotid artery (ECA) (arrows). flow voids and avid contrast enhancement on all spin echo
sequences. Ultrasound has been used to differentiate IH from
DIFFERENTIAL DIAGNOSISCongenital hemangioma other cutaneous cystic lesions or lymph nodes. It is limited
(CH), infantile hemangioma (IH), vascular malformation, in its ability to map the extent of the lesion. It could grow to
hemangioendothelioma. a rather large size.
339

340 Case 159
IH is a true neoplasm of vascular endothelial origin as Reporting Responsibilities

opposed to a vascular malformation. Up to 10% of Caucasian Direct reporting is essential for all tumors. Describe the size
infants are affected. The tumor may arise anywhere, but the and extent of the lesion. Describe any potential complications,
head and neck regions are most common. When superficial, for example, compression of a critical structure (e.g., the optic
the tumor appears as a bluish-red, noncompressible mass nerve) or airway effacement when present.
with the surface texture of a strawberry. The classic IH is
small or undetectable at birth and develops rapidly during
the first year of life. The tumor demonstrates a two-phase
life cycle. Initially, during the proliferative phase, the tumor IH is a common benign lesion which is best left alone
grows rapidly and is highly vascularized. Histologically, Definitive therapy should be considered when mass effect
endothelial cell hyperplasia predominates. During the threatens critical structures or when skin ulceration is seen
subsequent involutive phase, the endothelial cells thin with Extent and other associations such as in PHACES
deposition of fibrofatty material. Complete or near-complete syndromeposterior fossa malformation, hemangioma,

regression is the norm within 5 to 7 years. IH is contrasted arterial anomalies, cardiac anomalies/aortic coarctation,
to CH, a histologically similar lesion but with a different eye abnormalities, and sternal defects
natural history. CHs are present and fully formed at birth.
By clinical examination and imaging, they appear similar to Answer
IH. CHs follow two possible life cycles. Some will undergo 1. Hemangioma of bone (vascular malformation, may be
rapid involution with complete regression in 1 to 2 years. capillary or cavernous based on size of vessels); cavern-
Others are noninvoluting and not only persist with time but ous hemangioma of the brain (vascular hamartoma prone
actually grow as the child grows. Pharmacotherapy with cor- to intralesional hemorrhage); IH; CH; hepatic cavern-
ticosteroids and propranolol appears to be a safe and effec- ous hemangioma (benign network of vascular channels);
tive treatment when treatment is deemed necessary. Laser hemangioendothelioma (malignant vascular tumor of the
surgery and surgical excision are other treatment options. skin and organs).

1. How many clinically distinct hemangiomas are there in
the body?

Case
160 CLINICAL HISTORY 54-year-old male with a 3-day history of headache,
speech difficulty, and a right facial droop.
341

342 Case 160
FINDINGS Figure 160-1. Axial NCCT through the frontal or progression from WHO II ODG. Rare complications of
lobes. There is a large left frontal lobe intraaxial mass with ODG include leptomeningeal oligodendrogliomatosis, cere-
an anterior hyperdense nodule (transverse arrow). There is brospinal fluid (CSF) drop metastases, and systemic metas-
a larger posterior cystic component with a hyperdense fluid tases. The imaging features could sometimes mimic diffuse
component and level (vertical arrows). The hyperdense areas astrocytoma, arteriovenous malformation (AVM), infection,
are consistent with hemorrhage. There is substantial mass glioblastoma (GB), or even meningioma.
effect with subfalcine herniation to the right (line arrow). ODGs are the second most common primary brain tumors
Figure 160-2. Axial FLAIR through the centrum semiovale. in adult with a male to female ratio of 1.1:1. The peak inci-
The anterior nodule is predominantly hypointense with sur- dence is at 40 to 45 years. It could be indolent and large
rounding heterogeneous hyperintensity. The posterior cystic before discovery. It is assumed in this instance that the
cavity is hypointense with surrounding hyperintensity hemorrhage produced the neurologic deficits. Other pre-
edema (arrow). Figures 160-3 and 160-4. Axial pre- and senting features include seizures and headache. Lesions in
post-contrast T1WI. The anterior nodule is hypointense pre- eloquent areas may present with specific neurologic deficits.
contrast with heterogeneous contrast enhancement (arrows). ODG with 1p and/or 19q deletion are sensitive to vincristine
The cystic posterior component does not enhance with (PVC), temozolomide, radiation, or combined chemoradia-
contrast. tion. Prognosis is closely linked to the 1p and 19q deletion
with survival time up to 7 years.
DIFFERENTIAL DIAGNOSIS Secondary intracerebral hema
toma (SICH), hemorrhage into an abscess, hemorrhage into a Question for Further Thought
tumor. 1. What is responsible for the hemorrhage in ODG?
DIAGNOSIS Hemorrhage into a WHO III anaplastic oligo- Reporting Responsibilities

dendroglioma (ODG). Direct reporting is necessary in tumors particularly with
hemorrhage and mass effect.
DISCUSSION ODG is a gray matter (GM)-based tumor
that could be a low-grade WHO II or a high-grade WHO III
(anaplastic). It is usually a cortical/subcortical supratentorial
mass mostly frontal lobe single hemispheric lesion with rare Size, location, mass effect, and other complications
multifocality. Rare infratentorial or spinal location has been Presence of leptomeningeal disease which may alter the
described. It is usually a hypodense non-contrast enhancing management protocol
mass on CT with or without hyperdense areas of calcification
and rare hemorrhage and cystic change. The anaplastic ODG Answer
is more likely to show hemorrhage, cystic change, and sur- 1. ODG is a richly vascularized tumor with the capillary net-
rounding hypodense edema. ODG is usually a cortical-based work described as chicken-wire pattern with a tendency
hypointense well-defined mass on T1WI, hyperintense on for intratumoral hemorrhages. It has been suggested that
T2WI with some heterogeneity in the presence of calcifica- the anaplastic progression of ODG may be due to the pres-
tions or hemorrhage. The calcifications and hemorrhage are ence of small zones of vascular endothelial growth fac-
hypointense on GRE. Surrounding edema, cystic change, and tor (VEGF)-expressing cells which induce early vascular
contrast enhancement suggest anaplastic ODG. The contrast- proliferation and subsequent accelerated angiogenesis
enhancing area may show elevated relative Cerebral Blood related to areas of necrosis and hemorrhage. Hemorrhage
Volume (rCBV). Anaplastic ODG WHO III may be primary and necrosis are more common in anaplastic ODG.

Case
161 CLINICAL HISTORY Newborn male with large mass at vertex.
343

344 Case 161
FINDINGS Figure 161-1. Sagittal T1WI through the defect in the bone (arrows) with a mushroom appearing
vertex mass. Brain tissue along with meninges and cere- cephalocele superiorly to it.
brospinal fluid (CSF) are seen protruding through a defect
in the frontoparietal junction. The mass measures 8.5 cm DIFFERENTIAL DIAGNOSIS N/A.
7.1 cm 5.3 cm. There is a linear hyperintense struc-
ture extending from the suprasellar cistern into the mass DIAGNOSIS Vertex cephalocele.
(anterior arrow) possibly a vascular pedicle. It appears as
if the midline structuresventricles, corpus callosum (pos- DISCUSSION The hallmark of a cephalocele is the pres-
terior arrow)have been flushed up through the defect. ence of intracranial contents within an extracranial sac.
Figure161-2. Coronal T2WI through the mass. There is The contents of the sac protrude through a congenital bone
dysgenetic brain, CSF, and meninges up in themass. The defect. Parietal vertex cephalocele forms about 5% to 10%
large CSF space (star) probably represents the ventricles. of all cephaloceles. The content is usually dysgenetic brain,
The falx or a vascular pedicle (transverse arrow) extends vascular structures, CSF space, and the sac itself could be
into the mass. There are multiple heterotopic gray matter various combinations of the meninges. Duplication, split,
(GM) throughout the brain within the intracranial cavity or fenestration of the SSS is a common anomaly in vertex
(vertical arrows). Figure 161-3. Axial T2WI through the cephaloceles. Persistent falcine sinus is also common. There
suprasellar cistern. There are two rows of GM separated by is verticalization of most midline structures such as the ten-
white matter (WM) in bilateral cerebral hemispheres con- torium, the vein of Galen, the fourth ventricle, and the aque-
sistent with bilateral subcortical band heterotopia (arrows). duct as they head into the cephalocele.
The cortical GM shows rudimentary sulci suggesting poly- Hydrocephalus is common. Other congenital anomalies
microgyria (lateral arrows). Figure161-4. Coronal T2WI may include migrational disorders such as heterotopias and
through the posterior lip of the defect. There is possible cortical malformations (dysplasia, polymicrogyria, lissen-
split superior sagittal sinus (SSS) (transverse arrows) with cephaly). Midline malformations may also be present, and
the left sinus extending into the cephalocele. A vascular these may include corpus callosal dysgenesis, absence of the
lake presumably a large dilated vein is present on the right falx, and holoprosencephaly with fusion of the thalami. The
side in the cephalocele (vertical arrow). The posterior fossa prognosis is always grim in the presence of these anoma-
structures are also flushed up the tentorial incisura (star). lies. Management consists of surgical repair and supportive
Figure 161-5. Axial NCCT through the tentorium. There treatment.
is crowding of the tentorial incisura (star) with effacement There is a type of parietal vertex cephalocele known as
of all CSF spaces intracranially. Figure161-6. Reformatted atrophic parietal cephalocele. There is a very small defect
coronal NCCT through the mass. This demonstrates the through which protrudes a tiny to small amount of meninges

Case 161 345
with or without brain tissue and CSF. Venous anomalies are What the Treating Physician Needs to Know
also associated with this form of parietal cephalocele. Contents of the cephalocele sac and the location of the
neck
Question for Further Thought Changes in the intracranial cavity; are there other dysge-
1. What is the best way to evaluate the associated vascular netic changes within the brain?
anomalies? The nature of the vascular structures

Routine reporting is sufficient. The neonatologist is well 1. MRA and MRV have been found very useful in the evalua-
aware of the diagnosis. The expectations are that we dis- tion of the vascular anomalies associated with vertex ceph-
cuss the various anomalies and particularly the vascular alocele. It is important before surgery to completely map
changes. out the vascular structures to prevent tragic consequences.

Case
162 CLINICAL HISTORY 75-year-old male with new onset left-sided leg and foot
weakness.
346

Case 162 347
temporal lobe or occipital lobe. CT demonstrates a well-

defined hypodense mass. Mass is usually hypointense on
T1WI and hyperintense on T2WI and FLAIR. There is a
solid or irregular rim enhancement and extensive surround-
ing edema. The lesion in this instance demonstrates relative
restricted diffusion with elevated rCBV consistent with a
cellular high-grade tumor. Anatomic imaging features are
similar to a solitary metastasis from which it could be dif-
ficult to differentiate. GG, cortical ependymoma, lobar PA,
and PXA could have a similar appearance since all of these
lesions can present as a solidly enhancing mass rather than
the more standard cystic and nodular mass. However, these
other cortical/subcortical masses tend to have less sur-
rounding edema. Enhancement is more unusual in dysem-
bryoplastic neuroepithelial tumor (DNET) which is often
multicystic/bubbly in nature. Low-grade astrocytomas and
oligodendrogliomas would in general not enhance, and sur-
rounding edema is usually absent. Higher-grade anaplastic
astrocytomas and oligodendrogliomas may have surround-
ing edema. Glioblastoma could present as a solid mass with
microscopic necrosis that is not visible at imaging but will
Figure 162-5 usually present with a thick irregular rim of enhancement.
GCA occurs over a broad age range but in general occur
in adults older than 50 years with a male predominance.
FINDINGS Figure 162-1. Axial non-contrast T1WI through Presentation depends on location with seizures and focal neu-
the cerebral convexities. There is a round right posterior fron- rologic deficits as common presentations. GCA at pathology
tal lobe parasagittal small well-circumscribed mass (transverse can be confused with reactive conditions such as multiple
arrow) in the premotor area, mildly heterogeneous but isoin- sclerosis, progressive multifocal leukoencephalopathy, and
tense compared to surrounding expanded hypointense white infarction secondary to the granular cells resembling mac-
matter (WM) that is consistent with edema (vertical arrow). rophages. Most patients with GCA die within 1 year. The
Figure162-2. Corresponding axial T2WI. The mass is mildly aggressive behavior is opposite of the benign nature of gran-
heterogeneous and slightly hypointense (arrow) compared to ular cell tumors in other locations in the body. Follow-up
the surrounding hyperintense edema. The right superior fron- after excision in this patient revealed a new anterior right
tal gyrus is expanded, and adjacent sulci are partially effaced. frontal lobe mass with similar characteristics.
Figure 162-3. Coronal post-contrast T1WI. There is a single
mildly heterogeneous contrast-enhancing, sharply margin-
ated, subcortical mass in the posterior parasagittal right fron-
tal lobe. There is surrounding hypointensity consistent with 1. What is the choice of therapy?
edema or infiltrating tumor. Figure 162-4. Axial blood volume 2. Are there any common genetic alterations?
perfusion map through the mass. There is significant increase
in the relative cerebral blood volume (rCBV) (arrow). Reporting Responsibilities
Figure 162-5. ADC map through the mass. The mass has rela- Direct reporting is essential. Presence of significant edema
tive restricted diffusion peripherally compared to the adjacent and mass effect makes reporting more urgent. Presence of
brain. This likely indicates hypercellularity in the mass and tumors or enhancement elsewhere may suggest multifocal
the surrounding elevated ADC values indicate that most of the lesions or cerebrospinal fluid (CSF) seeding.
surrounding abnormality is likely vasogenic edema.
DIFFERENTIAL DIAGNOSIS Metastasis, anaplastic astro Location and size
cytoma, anaplastic oligodendroglioma, ganglioglioma (GG), Presence of significant mass effect or herniation
pilocytic astrocytoma (PA), pleomorphic xanthoastrocytoma Place of advanced imaging to further characterize the mass
(PXA), lobar ependymoma.
Answers
DIAGNOSIS Granular cell astrocytoma (GCA). 1. Surgical excision plus postoperative chemotherapy or
radiotherapy is the treatment of choice for most patients
DISCUSSION GCA is a rare WHO IV astrocytoma. The with GCA.
tumors almost all occur in the cerebral hemispheres. More 2. Loss of 9p and 10q are present in almost all cases and may
tend to occur in the parietal or frontal lobes than in the help explain the aggressive behavior.

Case
163 CLINICAL HISTORY 24-year-old female originally from Peru was
brought to the Emergency Room by her friends after they noticed seizure
activity followed by loss of consciousness.
348

Case 163 349
DIFFERENTIAL DIAGNOSISCalcified neurocysticerco-

sis (CN), embolic calcifications, dystrophic calcifications,
miliary brain metastasis, toxoplasmosis.
DIAGNOSIS Calcified neurocysticercosis (CN).
DISCUSSION CN is the final stage of the four stages of

involution of central nervous system (CNS) cysticerco-
sis. It is considered an inactive stage of the disease but is
known to be associated with seizures. Like most cysticercosis
lesions, the calcifications are found in the whitegray mat-
ter junction at imaging as demonstrated in these images. It
has been argued using histopathologic technique that these
calcifications are actually located in the deep sulci or in peri-
vascular spaces surrounding perforating vessels and therefore
technically these so-called parenchymal lesions are subarach-
noid in location. CT depicts these calcifications as hyper-
dense with no obvious surrounding parenchymal changes.
They are small to punctate in size and generally numerous.
Contiguous calcifications could coalesce. The location and
pattern on CT are unmistakable. CN is mostly hypointense on
all MR sequences and may have a surrounding halo of hyper-
intensity on T2WI, FLAIR, and GRE suggestive of gliosis
or edema as demonstrated in most of the images presented
here. Calcified granular stage could have mild peripheral
Figure 163-5 contrast enhancement while the late lesions generally do not
show contrast enhancement. Both CT and MR particularly
GRE sequences are equally effective in demonstrating these
lesions. Toxoplasmosis, treated tuberculosis, cytomegalovi-
rus, and rubella congenital infections show a pattern of cal-
FINDINGS Figures 163-1 and 163-2. Axial NCCT through cifications that tends to be larger, irregular, periventricular,
two different levels of the brain showing multiple small round and of varying shapes. Embolic calcifications are usually
hyperdense lesions, calcifications, mostly in cortical/subcor- leptomeningial in location. Metastases rarely calcify, tend to
tical locations of bilateral cerebral hemispheres (arrows in grow, contrast enhance, and have perilesional edema.
Figure 163-1). Two punctate calcifications are located in the In endemic region, CN have been reported in 10% to 20%
right lentiform nucleus (vertical arrows in Figure 163-2). A of asymptomatic individuals. CN is more prevalent than via-
tiny cortical right thalamic calcification is demonstrated in ble cysts, especially in symptomatic patients. The prevalence
Figure 163-2 (transverse arrow). There is no obvious sur- of calcified lesions ranges from 9% to 83% in selected popula-
rounding edema or contrast enhancement on post-contrast tions with seizures. Edema around calcified lesions (presum-
images. Figure 163-3. Axial DWI through the level of the ably late nodular granular stage) is a common finding (50%)
thalamus. There are two punctate hypointense lesions in the and might be associated with seizures or focal neurological
right frontal graywhite matter junction laterally with sur- deficits. This patient provided a history of neurocysticercosis
rounding zone of hyperintensity (arrows). The two lesions diagnosed in childhood, and a history of seizure disorder diag-
are represented by focal hypointense lesions with surround- nosed when she was about 9 or 10 years of age. She has been
ing hyperintense halo (possibly gliosis or edema) on the axial on treatment with carbamazepine, and the seizures were well
GRE image through same level in Figure 163-4 (transverse controlled. After several years of treatment, she discontinued
arrows). A few other similar lesions are demonstrated in the the treatment. She did well until this episode. Seizures rep-
right lentiform nucleus and left operculum (vertical arrows). resent the most common presentation of neurocysticercosis
Bilateral parietal cortical hypointense lesions are less well (NCC) (78.8%), especially if the lesions are localized in the
visualized on the axial T2WI (Figure 163-5) as they are of parenchyma. The diagnosis of NCC is based on a combination
almost same signal intensity with the brain (arrows). A lesion of epidemiological data, signs and symptoms, serological tests
at the junction of the body of the right caudate nucleus and ETIB or ELISA, brain imaging, and pathology (if available).
the right corona radiata is hypointense with surrounding The patient was started on medication with good response.
hyperintense halo possibly gliosis (chevron). An additional
isointense lesion with hyperintense halo is in the right frontal Question for Further Thought
lobe laterally (vertical arrow). 1. What are the four stages of neurocysticercosis involution?

350 Case 163

In patients with seizures, direct notification is important to 1. Neurocysticercosis is a consequence of ingestion of the
direct further investigation of findings. In asymptomatic egg of tapeworm Taenia solium. It is a fecal oral con-
patients, routine reporting will suffice. Presence of other tamination with CNS involvement almost 100%. The
stages of NCC should be noted. involution starts with the vesicular stage; cyst with thin
wall, an eccentric tiny calcified scolex with no surround-
What the Treating Physician Needs to Know ing abnormality on CT or MRI. The colloidal stage is the
Location of lesions for proper correlation with site of sei- most reactive of the stages with contracted lesion showing
zure as determined by other clinical parameters contrast enhancement and surrounding edema. The nodu-
Other stages of neurocysticercosis that may be present lar granular stage is much smaller with receding edema
Presence of contrast enhancement that may suggest active and contrast enhancement. The nodular calcified stage is
stage of the disease small usually without edema or contrast enhancement.

Case
164 CLINICAL HISTORY 32-year-old male with dizziness and
lightheadedness.

351

352 Case 164
Figure 164-5
Figure 164-6
FINDINGS Figure 164-1. Axial NCCT through the pos- shows a hyperdense mass with contrast enhancement.
terior fossa. There is a large hyperdense mass (vertical Calcification and cysts may be present. Medulloblastomas
arrow) laterally on the right in the posterior fossa. There is are almost always iso-hypointense on T1WI and hyperin-
hypodensity medially to the mass (transverse arrow) con- tense on T2WI and often show contrast enhancement. There
sistent with edema. Figure 164-2. Axial T2WI through the is reduced diffusion because of the hypercellularity of the
mass. The mass is mildly heterogeneously hyperintense and tumor. Intratumoral cysts, necrosis, and calcification are not
exophytic with widening of the right cerebellopontine angle uncommon. A striated pattern similar to Lhermitte-Duclos
(CPA) and normal-appearing right internal auditory canal tumor has been reported. There is surrounding edema and
(IAC) (chevron). There is compression of the right brachium frequent invasion of the brainstem. Presence of leptomenin-
pontis (vertical arrow). There is edema medially (transverse geal enhancement is indicative of cerebrospinal fluid (CSF)
arrow). Figure 164-3. Axial FLAIR through the mass. The dissemination. Just like in children, evaluation of the neu-
round mass is mildly hyperintense (star) with surrounding roaxis is necessary to exclude metastases. Hydrocephalus is
edema medially and compression of the fourth ventricle common, virtually in all patients, due to fourth ventricular
(single arrow) and brainstem. There is scalloping of the right obstruction. The exophytic location in the right petrosal sur-
petrosal surface (lateral 2 arrows over the petrous bone). face with widening of the CPA raises concern for menin-
Figure164-4. Sagittal post-contrast T1WI through the mass. gioma. The right IAC is normal thus excluding a vestibular
There is diffuse contrast enhancement of the mass (arrow). schwannoma. High-grade astrocytoma remains a possibility.
Figures 164-5 and 164-6. Axial DWI and ADC map through Medulloblastoma is a WHO IV highly malignant primi-
the mass. The mass is hyperintense on DWI and hypointense tive neuroepithelial tumor which accounts for 15% to 30% of
on ADC map consistent with restricted diffusion (arrows). pediatrics intracranial tumors but less than 1% of adult brain
tumors. It is a highly cellular tumor composed of small round
DIFFERENTIAL DIAGNOSIS Schwannoma, medulloblas- blue cells. There are four histologic types; imaging cannot
toma, astrocytoma, medulloblastoma, meningioma. differentiate between the various histologic types. Clinical
presentations include nausea and vomiting, headache, trun-
DIAGNOSIS Adult medulloblastoma. cal and limb ataxia, and papilledema. There is a male pre-
dominance with most occurring between 21 and 40years
DISCUSSION Adult medulloblastoma is nothing like of age. By the time these tumors are discovered, they have
the same tumor in children except in the histologic details spread invariably through the neuroaxis and occasionally
and posterior fossa location. The tumor is more likely to outside the central nervous system (CNS).
be lateral than midline. In this particular case, the tumor is
exophytic with extrinsic compression of the right brachium Question for Further Thought
pontis and rotation of the brainstem. The scalloping of the 1. What are the treatment options for adult medulloblastoma?
petrosal surface suggests a slow-growing tumor. Both CT
and MRI show the specific pattern of the tumor, but MRI Reporting Responsibilities
is the modality of choice for the evaluation because of its Direct reporting is essential for a large tumor totally unex-
great tissue characterization ability, multiplanar capability, pected. Presence of hydrocephalus and leptomeningeal
and ability to completely assess the neuroaxis. CT usually enhancement should be reported.

Case 164 353
What the Treating Physician Needs to Know survival of 10.6 years. Some childhood prognostic fac-
Size and location of mass and its effect on surrounding tors such as age at diagnosis, M stage, and extent of
structures resection have not been found to be predictive in adults.
Evidence of CSF seeding or extracranial spread Positive predictive factors in adults include higher post-
Is there hydrocephalus? operative functional status, absence of hydrocephalus and
ventriculoperitoneal (VP) shunt, and overexpression of
Mouse double minute 2 homolog (MDM2). Achievement
Answer of these survival trends relies on surgical removal of the
1. In one of the largest survival study, the 2-, 5-, and 10-year tumor and radiation to the entire neuroaxis. The role of
survival rates for adults with medulloblastoma were chemotherapy in adult medulloblastoma is not as well
79.9%, 64.9%, and 52.1%, respectively, with a median established as in children.

Case
165 CLINICAL HISTORY 47-year-old right-handed female initially
presented with painful visual loss in the left eye.
Figure 165-2
Figure 165-1
FINDINGS Figures 165-1. Axial MR T2WI through the

corona radiata. There are multifocal small hyperintense
lesions mainly in the deep white matter of bilateral cerebral
hemispheres (arrows). Figure 165-2. Axial post-contrast
T1WI through the middle cranial fossae. There is a small
left medial temporal focal contrast enhancement (transverse
arrow). There is mild leptomeningeal enhancement around
the brainstem (vertical arrow). Figure 165-3. Axial non-
contrast T1WI through the orbits. There is thickened hypoin-
tense left optic nerve and sheath (arrow). Figure 165-4. Axial
post-contrast T1WI through the lateral ventricles. There is
multifocal leptomeningeal and pachymeningeal enhance-
Figure 165-5 ment (arrows). Figure 165-5. Coronal post-contrast T1WI
354

Case 165 355
axis, cerebral white matter (WM), and the corpus callosum.

Lesions are usually isointense to hypointense on T1WI with
multifocal T2 hyperintensities; nonspecific small-to-large
lesions with surrounding edema mimicking tumors or tume-
factive demyelinating process sometimes. Periventricular
lesions are usually confused with MS. A substantial per-
centage will contrast enhance in an avid nodular fashion.
Leptomeningeal and pachymeningeal lesions could be
hypointense on T2WI depending on the degree of fibrous
material present. These lesions could be solitary, fragmen-
tary, or diffuse with effacement and avid enhancement of
the sulci and perivascular spaces mimicking meningitis,
Lyme disease, lymphoma, etc. Large nodular meningeal
masses with avid contrast enhancement may mimic neo-
plasm, metastasis, meningioma, or Langerhans cell histio-
cytosis. These changes are usually absent in MS. Contrast
enhancement of cranial nerves is common. Communicating
or obstructive hydrocephalus may occur. Orbital involve-
ment includes thickening and contrast-enhancing optic
nerves and sheath mimicking optic neuritis, other forms
of optic neuropathy, glioma, metastases, or meningioma.
Orbital inflammatory disease, lacrimal adenitis, chorio-
retinitis, and retinal periphlebitis are other forms of orbital
involvement.
Clinical presentations depend on the anatomical structures
involved and could be nonspecific and mimic several other
entities such as MS, optic neuropathy, hypothalamicpituitary
dysfunction, ataxia, or neurologic deficit. Asymptomatic NS
has been reported. The estimated frequency of NS is 5% but
Figure 165-6 postmortem diagnosis might increase this figure to 10%. The
commonly accepted criteria for the diagnosis of NS include
with fat suppression of the orbits. The left optic nerve and a clinical picture compatible with NS, exclusion of other
sheath show enlargement and enhancement (transverse neurologic diseases, and histologic confirmation of disease
arrow). Figures 165-6. Sagittal post-contrast T1WI, through elsewhere or by biopsy.
the foramen magnum. There is thickened contrast-enhancing The diagnosis was made in our patient following cauda
dural plague (pachymeninges) posteriorly at the cranioverte- equina biopsy prompted by an abnormal spinal MRI
bral junction (arrow) projecting into the cerebrospinal fluid obtained for walking difficulty. NS in general carries a poor
(CSF) space. prognosis. Most patients are treated with steroids. Plasma
exchange has also been used for acute exacerbations.
DIFFERENTIAL DIAGNOSISMultiple sclerosis (MS), Immunosuppressants and immunodulatory agents are used
sarcoidosis, Langerhans cell histiocytosis, meningioma, neu- for their steroid-sparing effect.
rofibromatosis type 1 (NF1).
DIAGNOSIS Neurosarcoidosis (NS). 1. What is the Kveim test?
DISCUSSION Sarcoidosis is a multisystem noncaseat-

ing granulomatous disease of unknown etiology, which has
NS rarely present as emergency. Routine reporting is suffi-
a propensity for the lungs and relatively rarely affects the
cient. Acute optic neuropathy, hydrocephalus, cord compres-
central nervous system (CNS). NS involves every aspect of
sion, and tumor-like presentations deserve direct reporting to
the CNS, including the brain parenchyma, cranial nerves,
the referring physician.
spinal cord, leptomeninges and pachymeninges, orbits,
and the cauda equina. Its manifestations can mimic several
other CNS disorders, hence difficulty in the diagnosis. MRI What the Treating Physician Needs to Know
is the examination of choice for evaluating NS although CT Location and number of lesions. Biopsy may become
changes correlates well with the MRI findings. In the brain, necessary
NS has a propensity for the hypothalamusinfundibulum Any complications such as masses or hydrocephalus?

356 Case 165
Is lumbar puncture (LP) safe? Leptomeningeal enhancement spleen, lymph node, or other tissue from a confirmed case
may be clue to diagnosis resulting in CSF examination of sarcoidosis, is injected intradermally. A positive test
is characterized by the formation of a papule at the injec-
Answer tion site within 4 to 6 weeks. Microscopic examination
1. The Kveim skin test is a less invasive test, and if posi- of the papule exhibits nonnecrotizing granulomas and the
tive, is considered to be diagnostic of sarcoidosis or NS. absence of foreign material. Test sensitivity is about 80%.
The test material, a suspension of granuloma-containing False positive rate is about 1% to 2%.

Case
166 CLINICAL HISTORY 17-year-old male with headache, nausea,
and vomiting.
357

358 Case 166
FINDINGS Figures 166-1 and 166-2. Axial pre- and andT2WI. Contrast enhancement has been described as
post-contrast CT, respectively, through the pineal region. varying from mild to intense and anywhere in between.
There is a 2-cm well-circumscribed contrast-enhancing The size of the tumor can vary between a few millimeters
hyperdense mass in the pineal region (arrow). A tiny cal to about 3.8 cm. There is usually no hemorrhage or calcifi
cification is present posteriorly in the mass. There are three cation. Presence of calcification, cysts, or fat may indicate
other similar lesions in the suprasellar cistern (not shown), a teratoma. Germinomas are usually midline in location in
along the left tentorium (vertical arrow) and in the inferior and around the third ventricle, with the pineal region being
fourth ventricle (not shown). There is hydrocephalus (stars). the most common location followed by the suprasellar
Figure 166-3. Axial T2WI through the pineal region. There region and up and down the ventricular system including the
is a well-circumscribed isointense mass (to gray matter thalami and basal ganglia. It could occur synchronously in
[GM]) with three tiny hyperintense foci internally in the or metastasize to multiple centers such as the pineal gland,
pineal region (arrow). There is surrounding hyperintensity neurohypophyseal axis, bilateral basal ganglia, and thalamic
in the thalami suggesting edema. There is hydrocephalus. regions or diffuse periventricular location with intramedul
Figures 166-4 and 166-5. Pre- and post-contrast sagittal lary and intrasellar variants. Metastases are often necrotic
T1WI through the pineal mass. The masses are isointense and T2 hyperintense. It may be difficult to differentiate from
to hypointense with avid contrast enhancement in the pineal pineocytoma and pineoblastoma. The pineal region could be
region (vertical arrow), hypothalamus (transverse arrow), affected in trilateral retinoblastoma mostly in children under
and at the obex (chevron). The stars are within dilated 3 years of age.
third and lateral ventricles consistent with hydrocephalus. Germinoma is the commonest tumor of the pineal region
Figure166-6. Post treatment sagittal post-contrast T1WI constituting about 35% of all pineal region tumors. It is more
through same level as in Figure 166-5. The masses are no common in males than females and occurs in all ages but
longer visualized. generally at a lower age than other pineal cell tumors and
usually in peripubertal males and young adults. The trans
DIFFERENTIAL DIAGNOSIS Germinoma, retinoblastoma, phenoidal biopsy of the hypothalamic mass in this patient
metastases, pineocytoma, pineoblastoma. yielded pure germinoma, the most common central ner
vous system (CNS) germ cell tumor. The histology is that
DIAGNOSIS Germinoma. of undifferentiated cell that resemble primordial germinal
element. It could be mistaken for sarcoidosis or tuberculo
DISCUSSION A recent publication claims there were no sis if granulomatous response predominates. Presentation of
definitive imaging characteristics that distinguished pineal germinoma varies with its location and size. Hydrocephalus
cell tumors from germinomas except that germinomas is common with pineal location resulting in raised intracra
showed higher ADC values, and the patients were younger nial pressure with headache, nausea, and vomiting predomi
than patients with pineal cell tumors. Germinomas are nating. Neurohypophyseal axis tumor may present with
usually hyperdense on CT and isointense on all sequences visual problems because of compression of the chiasm, and
with minimal variation of the intensity pattern on T1WI hypopituitarism and diabetes insipidus due to pituitary and

Case 166 359
hypothalamic failure. It is a very radiosensitive tumor with a What the Treating Physician Needs to Know
very high cure rate. Cerebrospinal fluid (CSF) diversion may Number and location of lesions
be indicated in the initial treatment of the tumor. Presence of CSF pathway obstruction
Presence of CSF seeding
The necessity for entire neuroaxis evaluation
1. What are some of the hormonal expressions of this tumor? Answer
1. Germinomas could express various oncoproteins. Such
Reporting Responsibilities oncoproteins include beta-human chorionic gonadotropin
Direct reporting is essential not only because of the hydro (HCG) and alpha-feto proteins, both of which were not
cephalus but also because early intervention can prolong elevated in this patient. Others include human placental
life and cure. It is important to tabulate the number and lactogen (HPL), placental alkaline phosphatase (PLAP),
location of lesions. Complete evaluation of the neuroaxis and other cytokeratins. Measurement of these hormones
is important to exclude metastases or other synchronous may assist in the correct diagnosis and monitoring thera
germinomas. peutic response of this tumor.

Case
167 CLINICAL HISTORY 21-year-old male with intractable seizures,
headache, glaucoma, and diabetes mellitus.
360

Case 167 361
Figure 167-6
ipsilateral leptomeningeal angiomatosis (LA) with lack of

normal cortical venous drainage, and congenital glaucoma.
Figure 167-5 CT and MRI are complementary tools to confirm the diag-
nosis of SWS. CT demonstrates the typical tramline cortical
or gyriform calcification in the location of the LA. This is
FINDINGS Figure 167-1. Axial NCCT through the occipi- most frequent in the frontoparietal lobes. However, involve-
tal lobes. There are bilateral occipital cortical calcifications ment of other cerebral lobes and bilateral LA as in this
(arrows) extending to the posterior right temporal lobe. patient hasbeen reported. Atrophy of the involved brain is
Figure167-2. Axial NCCT through the frontoparietal convex- typical, resulting in dilated sulci. Imaging, particularly MRI,
ity. There are right frontotemporal gyriform (tramline) calcifi- also shows other associated lesions such as enlargement of
cations (vertical arrow). Some faint left frontoparietal cortical the choroid plexus and leptomeningeal contrast enhance-
calcifications are present (transverse arrows). Figure167-3. ment in both supratentorial and infratentorial regions.
Axial GRE through the occipital lobes. There is bilateral Leptomeningeal enhancement has been described in all
occipital cortical hypointensity/blooming in the areas of cal- lobes of the cerebral hemispheres, while posterior fossa lep-
cifications seen in Figure 167-1. There is a small focus of left tomeningeal enhancement is rare. These changes are more
temporal lobe hypointensity (arrow). Figure 167-4. Axial GRE optimally demonstrated by contrast-enhanced MRI as clearly
through the frontoparietal convexities. Right frontoparietal seen in this patient. Physiologic imaging using MR perfu-
multifocal cortical hypointensity/blooming is present. There sion, SWI, MR spectroscopy, SPECT, and FDG-PET has
is dilatation of the right central sulcus (arrow) consistent with demonstrated underlying vascular and metabolic abnormali-
volume loss. Figure 167-5. Axial post-contrast T1WI through ties in these patients. These abnormalities include delay of
the corona radiata. There are prominent transmedullary veins venous contrast clearance on MR perfusion, transmedullary
coursing through the corona radiata bilaterally (arrows). venous collaterals on SWI, elevated choline at MR spectros-
Figure 167-6. Coronal post-contrast T1WI through the occipi- copy, hypoperfusion on SPECT, and hypometabolism on
tal lobes. There is extensive bilateral occipital parietal thick PET in the areas of cortical LA. DTI has shown abnormal
leptomeningeal enhancement (arrows). white matter (WM) structural integrity in these patients.
SWS was originally described in 1879 as an association
DIFFERENTIAL DIAGNOSIS N/A. of facial PWS, glaucoma, and seizures with the imaging find-
ings of pial angiomatosis described subsequently. The PWS
DIAGNOSIS Sturge-Weber syndrome (SWS). is usually located more frequently in the V1 and V2 distribu-
tion but involvement of V3 distribution has been reported.
DISCUSSION SWS otherwise known as encephalotrige Other clinical presentation may include hemiparesis, hemi-
minal angiomatosis is a neurocutaneous syndrome charac- anopsia, headache, glaucoma, mental/developmental retar-
terized by facial port wine stain (PWS) or nevus flammeus, dation, or cognitive function impairment. The etiology of

362 Case 167
this disease is unknown. The underlying pathogenesis is LA What the Treating Physician Needs to Know
and impaired superficial venous drainage with subsequent Location and pattern of lesions to allow for proper catego-
transmedullary drainage that seems insufficient. This results rization of the disease
in impaired cortical perfusion and ischemia with astrogliosis, Other imaging modalities that can shed light on
atrophy, and perivascular calcifications. It is a progressive pathogenesis
disease. It has been proposed that early recognition may lead Complications such as new hemorrhages
to aggressive and/or prophylactic management that may pre-
vent progression of disease particularly seizures and cogni- Answer
tive function impairment. 1. Yes. There is significant heterogeneity in the clinical pre-
sentation of the disease. All the changes may not be pres-
Question for Further Thought ent at the onset or even subsequently. It is a progressive
1. Is there known variation in the clinical presentation disease with both imaging and clinical features changing
ofSWS? over time. Three types of SWS are recognized. Type I
have both facial PWS and LA and may include glaucoma.
Reporting Responsibilities Type II usually has facial angioma alone with no CNS
Routine reporting may be sufficient. However, in the pres- involvement but may have glaucoma. The type III is an
ence of acute seizure disorder, it may be prudent to directly isolated LA without glaucoma or PWS. Imaging plays
report the findings to the referring physician so that appropri- a crucial role in early confirmation of the syndrome and
ate management decision could be made. sorting out the different types.

Case
168 CLINICAL HISTORY Adult with headache.
Figure 168-1
Figure 168-2
A B
Figure 168-3
FINDINGS Figure 168-1. Sagittal T2WI. There is abnor- (arrowheads) and scalp components (arrows) of the mass
mal, lobular isointense extraaxial tissue along the inner (confirmed on ADC, not shown). Figure 168-3. Axial post-
table of the skull (arrowheads). There is infiltration of contrast T1WI. There is diffuse enhancement of the extra-
the bony calvarium with contiguous involvement of the axial and extracranial components of the mass along with
deep scalp layers (arrows). Figure 168-2. Axial trace permeative destruction of the intervening frontoparietal
DWI. There is restricted diffusion within the intracranial calvarium (arrows).
363

364 Case 168
DIFFERENTIAL DIAGNOSIS Subdural empyema/abscess large B-cell lymphoma (DLBCL); often aggressive, neces-
with calvarial osteomyelitis and scalp infection, dural/calvar- sitating expedient and accurate diagnosis. Lymphoma may
ial lymphoma, dural/calvarial metastasis, aggressive menin- also arise in the dura as primary dural lymphoma (PDL),
gioma, plasmacytoma. which is a different entityusually a low grade, small cell
lymphocytic lymphoma with a more indolent course and bet-
DIAGNOSIS Secondary intracranial lymphoma. ter prognosis. It is most commonly a marginal zone B-cell
tumor, usually seen in the gastrointestinal tract where it is
DISCUSSION The imaging appearance of a lymphoma- referred to as MALT (mucosa-associated lymphoid tissue)
tous mass reflects the high cellularity of the tumor, usually lymphoma. PDL may secondarily involve the leptomeninges.
appearing hyperdense on NCCT, relatively hypointense on Clinical symptoms of CNS lymphoma are nonspecific and
T2WI and often restricting diffusion on DWI. There is typi- may be nonfocal and include cognitive dysfunction, person-
cally avid, homogeneous enhancement following contrast ality changes, and disorientation. More focal symptoms such
administration. The appearance may be even more hetero- as brainstem and cerebellar signs or cranial nerve dysfunc-
geneous in the immunocompromised patient. Because it is tion may relate to mass effect or leptomeningeal disease. The
dural based, dural tails are present. Within the calvarium, initial evaluation of patients with CNS lymphoma routinely
lymphoma typically exhibits a permeative growth pattern includes ophthalmologic examination and CSF analysis
with little cortical destruction, limiting the utility of radio (including cytology, PCR, and flow cytometry). Successful
graphy and CT for definitive diagnosis. Secondary dural treatment relies on an accurate histologic diagnosis with the
involvement may occur in cases arising in or metastasiz- exception of emergent therapies to manage intracranial mass
ing to bone. Contrast-enhanced MRI is critical for complete effect or intracranial hypertension.
disease mapping, in all of its forms (as multifocal disease
is not uncommon), and for biopsy planning when needed. Question for Further Thought
In our case, calvarial/meningeal metastasis is a reasonable 1. How would one differentiate primary from secondary
differential, which is most commonly secondary to prostate CNS/intracranial lymphoma?
or breast cancer, where diffusion restriction is less common.
Plasmacytoma often mimics the CT and MR features
of lymphoma but is uncommonly limited to the dura.
For secondary intracranial lymphoma, complete disease
Meningioma is rarely this aggressive but may occasionally
mapping is required with description of multifocal dural/
be indistinguishable from lymphoma limited to the dura.
skull disease, parenchymal involvement, and leptomeningeal
Hemangiopericytoma could be confused with this lesion. A
disease. Any mass effect, brain edema, or accompanying
mature subdural abscess would not enhance homogeneously.
dural venous sinus occlusion must be reported emergently.
Lymphoma is a challenging disease with protean manifes-
tations. Recognizing the many forms and manifestations of
intracranial lymphoma is critical to the understanding of the What the Treating Physician Needs to Know
disease and to the ability to make the diagnosis with imaging. Extent, number, and location of disease
Primary CNS lymphoma (PCNSL) is a subtype of extrano- Features necessitating emergent treatment (significant
dal non-Hodgkin lymphoma (NHL) which, by definition, is mass effect/herniation, dural venous sinus occlusion)
confined to the brain, leptomeninges, and eyes or occasion- Presence of leptomeningeal disease
ally to the spinal cord. It usually manifests within the deep
and periventricular tissues of the cerebrum. Secondary CNS Answer
lymphoma (SCNSL) more commonly affects the leptomen- 1. At the time of diagnosis, the presence of systemic disease
inges, dura, calvarium and scalp as in our case but may also confirms a diagnosis of SCNSL. Imaging of the chest,
present with intraaxial mass lesions. It is present in up to 5% abdomen, pelvis, and testes with bone marrow biopsy
of patients with aggressive systemic disease. Both primary is required. Secondary disease is most commonly dural,
and secondary forms are almost always histologically diffuse osseous, and leptomeningeal.

Case
FINDINGS Figure 169-1. NCCT through the inferior frontal

lobes. There is extraaxial crescentic large cerebrospinal fluid
(CSF) density space measuring about 1 cm thick over both
frontal lobes with effacement of underlying sulci (vertical
arrows). There is associated left frontal opercula hematoma
(transverse arrow) and IVH in left trigone (posterior left arrow).
Figures169-2 and 169-3. Axial FLAIR and T2WI through the
frontal lobes respectively. The bifrontal extraaxial collections
have CSF intensity on all sequences (arrows). The left frontal
opercula hematoma and IVH are again visualized.
DIFFERENTIAL DIAGNOSISAtrophy or volume loss,

subdural hygroma (SDG), chronic subdural hematoma
(CSDH).
DIAGNOSIS Traumatic subdural hygroma (TSDG).
DISCUSSION TSDG is defined as crescentic collection of

CSF, xanthochromic, or slightly hemorrhagic fluid in the sub-
dural space at least 3 mm thick following trauma. This may
be preceded by what has been described as a subdural effu-
sion (SDE) usually same fluid less than 3 mm thick in 50%
of SDG population. Some literature treats SDG and SDE as
the same entity. It has also been known as subdural hydroma
Figure 169-3 or subdural fluid collection. The fluid is usually hypodense
365

366 Case 169
or CSF density on CT with CSF intensity pattern on MRI. Question for Further Thought
There is effacement of the underlying sulci and depending 1. What is the pathogenesis of TSDG?
on its size and lateralization could be associated with midline
shift. It is unilateral in 65% and bilateral but asymmetric in
35% of one particular series. SDG could be detected imme-
Presence of any extraaxial collections including SDG fol-
diately following minor or severe head trauma or delayed.
lowing trauma requires direct reporting. Other associated
Association with ventriculomegaly occurs in about a third
traumatic findings should be enumerated. Presence of mass
of the patients. It is most commonly supratentorial and fron-
effect or ventricular dilatation should be reported. Follow-up
tal but posterior fossa occurrence has been reported. SDG
imaging changes in size, associated CSDH, or associated
is considered benign but could progress significantly over
ventriculomegaly are reportable.
time resulting in significant mass effect. TSDG is found in
about 6% of traumatic head injuries. Brain contusions, dif-
fuse axonal injury, subdural hematoma, fractures, and sub-
arachnoid hemorrhage or other forms of intracranial injuries Size and location
occur in about 45.8% as in this patient. Differentiating this Growth on follow-up imaging
entity from atrophy in the elderly could be difficult but the Presence of mass effect, ventriculomegaly, or CSDH
presence of sulcal effacement is key to the differentiation.
Asmall proportion of SDG evolve into CSDH. This evolu- Answer
tion has not been fully explained. The enhancing membrane 1. The basic explanation for TSDG is the presence of an
of CSDH differentiates it from SDG. arachnoid tear, allowing CSF to accumulate in the sub-
SDG is more common in men and occurs in all age groups. dural space. Presence of a flap valve provides a one-way
Apart from trauma, SDG could be found as a complication mechanism that allows continuing accumulation of CSF.
of meningitis, craniotomy, shunt, subarachnoid hemorrhage, A second explanation is that the fluid is an effusion due to
or arachnoid cyst. Presentation depends on other associated increased permeability of the traumatized vessels rather
traumatic findings. A stand-alone SDG could present with than CSF. Yet another explanation particularly in those
headache, seizures, and altered mental status. It is usually associated with ventriculomegaly is that of impaired CSF
considered benign, and treatment is conservative but opera- absorption resulting in some form of external hydroceph-
tive treatment may include craniotomy or CSF diversion if alus. These are usually the patients that may require CSF
associated with significant growth or ventriculomegaly. diversion.

Case
170 CLINICAL HISTORY 59-year-old right-handed female developed
constant hiccups associated with nausea and vomiting that lasted for
2 weeks from which she completely recovered. Three months later, she
developed ear pain followed by progressive numbness of her arms,
torso, and legs. Ultimately she lost control of her bladder and became
severely quadriparetic.
Hyperintensity extends to the medulla. Figure 170-2. Sagittal

post-contrast T1WI through the cervical spine. There is
smudgy contrast enhancement within the lesion from the
level of the foramen magnum to C3 (arrows), short of the
inferior extent of the lesion on STIR. Lesion is isointense
without contrast (not shown). Figure 170-3. Coronal post-
contrast T1WI through the level of the third ventricle. There
is a poorly marginated round contrast-enhancing lesion
in parasagittal right posterior frontal centrum semiovale
(arrow). There were a few other white matter lesions else-
where (not shown).
DIFFERENTIAL DIAGNOSISLongitudinally extensive

transverse myelitis (LETM), neuromyelitis optica (NMO),
multiple sclerosis (MS), astrocytoma, ependymoma.
Figure 170-3
DIAGNOSIS Neuromyelitis optica (NMO).

FINDINGS Figure 170-1. Sagittal short tau inversion
recovery (STIR) cervical spine MRI. There is enlargement DISCUSSION Classical imaging findings of NMO include
of the cervical spinal cord with smudgy hyperintensity from optic neuritis (ON) and long segment spinal cord lesion usu-
the level of the foramen magnum to the C4 level (arrows). ally longer than 3 vertebral lengthsLETM. MRI is the
367

368 Case 170
imaging method of choice as in all other demyelinating pro- was identified as the target of NMO-IgG, hence the name
cesses. The affected optic nerve is enlarged and hyperintense AQP4 antibody. AQP4 is diffusely found in the brain but
on T2WI and avidly contrast enhances in the acute stage. abundant in the optic nerve and spinal cord. The new criteria
The affected part of the spinal cord is long, swollen, and T2 for diagnosis of NMO now include ON, acute myelitis, and
hyperintense as demonstrated in this case. Acute lesions are the presence of at least two of three supportive criteria such
usually contrast enhancing. Severe ON with LETM with- as LETM, brain MRI not diagnostic of MS, and NMO or
out or with minimal brain involvement tends to distinguish AQP4-IgG seropositivity.
NMO from MS. The occurrence of brain lesions in NMO
was thought to be infrequent. Brain lesions when present are Question for Further Thought
usually non-contrast-enhancing nonspecific T2 hyperintense 1.
What is neuromyelitis optica spectrum disorders
lesions that could be found in periventricular locations in the (NMOSDs)?
cerebrum or cerebellum, corpus callosum, corticospinal tract
(mostly in Koreans), brainstem within the diencephalon, and
around the cerebral aqueduct and could be contiguous with
Direct reporting is necessary in any case of spinal mass. The
spinal cord lesions. Brain lesion enhancement when present
location here may predispose to respiratory failure. In the
is cloud-like, referring to multiple patches of enhancement
presence of LETM, NMO should be included in the differ-
with blurred margins. Brain lesions have been found in about
ential diagnosis. Brain evaluation should be recommended
70% of Japanese NMO population. Well-defined nodular
as a follow-up evaluation if the initial study was spine imag-
enhancement is rarely found. MR spectroscopy has found
ing. Spinal leptomeningeal enhancement should be reported
diffuse white matter damage to be absent in NMO but pres-
when present.
ent in MS. Astrocytoma and ependymoma are always a con-
sideration of enlarged hyperintense spinal cord. However,
the temporal profile of spinal cord neoplasm is different in What the Treating Physician Needs to Know
that there is no improvement and LETM can significantly Location and extent of lesion or lesions
improve as demonstrated elsewhere in this text. ON is not a Presence of leptomeningeal enhancement may help in dif-
feature of spinal neoplasm. ferential diagnosis of LETM
NMO is a monophasic or relapsingremitting inflam- Is LP feasible?
matory demyelinating disorder that clinically presents with If ON is present, is it bilateral? Patients with bilateral and
severe visual impairment and myelitis. Relapsing disease is simultaneous ON, poor visual recovery from ON, recur-
more frequent in women (3:1 female to male ratio). Lumbar rent ON, and concurrent autoimmune disease should be
puncture (LP) in this patient showed pleocytosis, increased screened for NMO antibodies if they lack the typical
protein (1.5times normal) but no oligoclonal bands. Extensive changes of MS on head MRI
serological workup was negative except for positive anti- Improvement or progression on follow-up study?
ribonucleoprotein (nRNP) antibodies usually present in mixed
connective tissue disease. She subsequently presented with Answer
right ON, which was treated with high-dose steroids with sig- 1. The availability of AQP4 antibody assay has allowed
nificant recovery. Her NMO-IgG testing was subsequently the identification of cases with a diversity of central ner-
positive. This case shows the difficulty in confirming the diag- vous system (CNS) lesions beyond the classical NMO
nosis of NMO. phenotype leading to NMOSD. NMOSD encompasses
NMO-IgG is a circulating autoantibody that is pres- definitive NMO, as well as limited forms, such as AQP4-
ent inpatients with NMO but absent in those with MS. IgG-positive LETM, or recurrent or bilateral AQP4-IgG-
The astrocyte water channel protein aquaporin 4 (AQP4) positive ON and Asian opticospinal MS.

Case 171 CLINICAL HISTORY 14-month-old female with hypotonic cerebral palsy.
FINDINGS Figure 171-1. Axial DWI. There is a large isoin- The mass is hyperintense with myriads punctate and linear
tense mass occupying the trigone and body of the right lat- hypointense areas. Figure 171-5. Post-contrast axial T1WI.
eral ventricle (star). Figure 171-2. Right parasagittal T1WI. There is avid contrast enhancement with lobulation of the
The large intraventricular mass is hypointense (star) to sur- outline of the mass. Some punctate and short linear hypoin-
rounding white matter (WM). Figure 171-3. Axial FLAIR. tensities are within the mass.
The right intraventricular mass is isointense (star). There is
mild asymmetry of the lateral ventricles with mild displace- DIFFERENTIAL DIAGNOSIS Choroid plexus carci-
ment of the septum pellucidum to the left. There is no hydro- noma (CPC), choroid plexus meningioma, choroid plexus
cephalus. Figure 171-4. Coronal T2WI through the trigones. papilloma (CPP).
369

370 Case 171
CPP usually presents with symptoms and signs of raised

intracranial pressure such as headache, nausea, and vomiting.
Enlargement of the head is common in infants. CPP arises
from the epithelium of the choroid plexus. The pathology mir-
rors normal choroid plexus architecture, usually a cauliflower,
well-defined mass which may adhere to the ventricular wall
but no infiltration. Increased vascularity, cysts, xanthomatous
degeneration, hemorrhage, and calcifications may be present.
Surgical removal is usually curative with a very low recur-
rence rate. Rare transformation to CPC has been reported, and
dissemination into the CSF spaces is not unknown.

1. What is the etiology of CPP?
2. What is atypical CPP?
Figure 171-5 Direct reporting is necessary because this is a tumor and
may present with hydrocephalus, and early treatment may
be curative. Location, presence of hydrocephalus, and lepto-
DIAGNOSIS Choroid plexus papilloma (CPP) WHO I. meningeal enhancement should be reported.
DISCUSSION CPP is usually a well-marginated intraven- What the Treating Physician Needs to Know
tricular mass sometimes with irregular mildly lobulated out- Location, number if more than one, presence of hydro-
line. CPP generally does not restrict diffusion, is isointense to cephalus, leptomeningeal enhancement
hypointense on T1WI, isointense to hyperintense on FLAIR
and T2WI, and avidly contrast enhancing. The punctate and Answers
linear hypointense areas on T2WI reflect vascular signal voids 1. It has been postulated that the simian virus 40 (SV40)
of the rich vasculature, hemorrhage, and/or calcifications. plays a role in the etiology of CPP. SV40 DNA sequences
Hydrocephalus is common usually due to overproduction of were found in populations exposed to SV40-contaminated
CSF or CSF pathway obstruction. CPP could also occur in polio vaccine but not in those who received noncontami-
the cerebellopontine angles with extraventricular and cystic nated polio vaccine. It is also determined that the incidence
CPP being very rare. CSF dissemination is a well-known of CPP in both populations is the same, suggesting that
complication resulting in nodular leptomeningeal enhance- SV40 may therefore not play a causal role in the develop-
ment and CSF pathway obstruction. The CT usually shows ment of CPP but a bystander infection due to an enabling
avidly contrast enhancing iso- to hyperdense intraventricular microenvironment that allows replication of the virus in
mass with lobulated margin. Calcification and hemorrhage the tumor. CPP has also been reported in the rhabdoid
impart some degree of heterogeneous hyperdensity to the predisposition syndrome and as part of the Aicardi syn-
mass. Hydrocephalus may also be present on CT. About drome; a triad of infantile spasm, CC agenesis, and cho-
50% of CPP occur in the lateral ventricles, 5% in the third, rioretinal lacunae that occur almost exclusively in female
and 40% in the fourth ventricle, with 5% multi-ventricular. as an X-linked dominant syndrome. Multi-ventricular
It usually does not infiltrate the ventricular walls or produce CPP occurs in about 1% of Aicardi syndrome.
periventricular edema like meningioma and CPC. 2. Atypical CPP represents about 15% of all choroid plexus
CPP is a WHO I tumor of children and along with other neoplasm. It is a CPP with increased mitotic activity with
choroid plexus tumors constitutes about 10% to 20% of all a malignancy status higher than CPP WHO I but lower
brain tumors in the first year of life but less than 1% of all than CPC WHO III. It is a histologic grading with no dif-
brain tumors. ferentiating imaging findings from CPP.

Case
172 CLINICAL HISTORY 62-year-old female status post fall with laceration
to occiput.
FINDINGS Figures 172-1 and 172-2. Axial NCCT brain DIFFERENTIAL DIAGNOSIS Meningioma, dural metas-
and sagittal MIP reformatted images, respectively, from a tasis, granulomatous processes such as sarcoidosis and tuber-
head and neck CTA. There is a partially calcified (periphe culosis (TB), primary bone neoplasm, that is, osteogenic
rally) extraaxial dural-based lesion arising from the posterior sarcoma, chondrosarcoma.
aspect of the foramen magnum (arrows) compressing the
upper spinal cord. Figures 172-3 and 172-4. Sagittal pre- and DIAGNOSIS Foramen magnum meningioma.
post-contrast T1WI, respectively. There is a homogeneously
contrast-enhancing (Figure 172-4) isointense (Figure 172-1) DISCUSSION Classical imaging findings of foramen mag-
dural-based lesion at the posterior aspect of the foramen num meningioma on CT and MRI are a homogeneously
magnum (vertical arrows) extending inferiorly to C2 level contrast-enhancing dural-based mass usually isodense on

with anterior displacement and compression of the adjacent NCCT and isointense on all MRI sequences, with a dural
medulla and upper cervical cord. There is a short dural tail tail, which is a linear enhancement of the dura beyond the
inferiorly in Figure 172-4 (transverse arrow). mass lesion. Meningiomas often calcify (hyperdense on CT
371

372 Case 172
and hypointense on MRI sequences particularly GRE) and can 2. Could meningioma be a part of the multiple inherited
be associated with hyperostosis of the adjacent bone, particu- schwannomas, meningiomas, and ependymomas (MISME)
larly at the skull base. Compression of the medulla, cerebel- triad?
lum, spinal cord, and the vertebral arteries is not uncommon
with foramen magnum meningioma. A heavily calcified dural- Reporting Responsibilities
based mass favors the diagnosis of meningioma over dural The ordering physician should be directly informed if
metastatic disease. Chondrosarcoma and osteosarcoma are impending neurologic compromise is seen or suspected
usually calcifying lesions. Sarcoidosis and TB are great mimics from a large meningioma at the foramen magnum causing
of meningioma, and usually there are other lesions elsewhere brainstem or cervical cord compression/edema. This is par-
to support these diagnoses. The finding of a dural tail is clas- ticularly important when the detection of the meningioma on
sic for meningioma, but can be seen with other dural-based imaging is incidental to the reason for the examination and
neoplastic or inflammatory lesions. Typical meningiomas the referring physician is not suspecting the diagnosis.
are often very slowly growing but can encase adjacent struc-
tures. Malignant meningiomas may invade adjacent structures
including the dural venous sinuses, the calvarium, and occa-
sionally the brain and result in vasogenic edema. Location, size, and mass effect upon adjacent structures
Meningiomas have a slightly greater female predomi- Presence of calcifications, cysts, or hemorrhage that may
nance and is a tumor of the adult population. Progesterone impact surgical approach and resectability
receptors have been identified in meningiomas, and these The main point of dural attachment (anterior, anterolateral,
tumors have been shown to increase in size during preg- lateral, posterolateral, or posterior) should be described
nancy. Prior radiation exposure (such as whole brain irra- when possible and whether the tumor is predominantly
diation) has been identified as a predisposing factor for the above or below the foramen magnum
development of meningiomas with a common radiation-
induced tumor latency of approximately 20 to 35 years. It Answers
is also common in neurofibromatosis. Genomic analysis of 1. While meningiomas are common intracranial tumors,
meningiomas suggests that there are distinct meningioma it is important to note that only 2% to 3% of meningio-
subtypes based on genetic mutations, and as a result avenues mas occur at the foramen magnum. Meningiomas could
for targeted therapeutics may be possible. Both surgical and also be multiple particularly in the inherited syndromes
Gamma Knife approaches have been advocated as provid- and with history of remote radiation therapy/exposure.
ing favorable treatment options. Regardless of the surgical Metastasis in the setting of known cancer should be
approach, complete resection of the tumor at the first attempt considered when the lesion is atypical or aggressive in
has been advocated. appearance. Dural metastasis from breast carcinoma can
mimic meningioma.
Questions for Further Thought 2. Meningiomas are part of the MISME syndrome and are
1. How common are meningiomas of the foramen mag- usually associated with patients who have neurofibroma-
num and how does the prevalence affect the differential tosis type 2 (NF2). Only 10% of patients with multiple
diagnosis? meningiomas have NF2 however.

Case
173 CLINICAL HISTORY 71-year-old male presented with confusion.


373

374 Case 173

FINDINGS Figures 173-1 and 173-2. Axial ADC map and Subependymomas are usually mildly hyperintense on T2WI.
corresponding DWI through the lateral ventricles. There is a Hemorrhage and calcification is occasional and hypointense
smooth nodular mass within the right frontal horn showing on T2WI and GRE. There is usually no perilesional edema.
no evidence of restricted diffusion (arrows). Figures 173-3 The tumor could extend from one ventricle to the adjacent
and 173-4. Axial FLAIR and T2WI, respectively, through ventricle. However, supratentorial subependymomas may be
the lateral ventricles. There is a smooth marginated mildly periventricular in location and have cystic components. MR
hyperintense right ventricular nodular mass with a tiny focal differentiation of subependymomas from other gliomas is
hypointensity posteromedially on the T2WI (arrow) (focal provided most reliably by the location and morphology of the
calcification or hemorrhage?). There is no evidence of perile- tumor and not by differences in signal intensity. However,
sional edema or mural attachment. Figures 173-5 and 173-6. ependymomas tend to be heterogeneous in character both in
Axial pre- and post-contrast T1WI, respectively. The nodule signal and in contrast enhancement pattern, while the other
is hypointense to surrounding white matter (WM) and does differentials are usually contrast enhancing with the possi-
not contrast enhance. There is a tiny focal hyperintensity pos- bility of perilesional edema. SEGCA is usually associated
teromedially corresponding to the T2 hypointensity (arrows). with ventricular wall calcification. CT of subependymoma
tends to show contrast-enhancing isodense nodular intraven-
DIFFERENTIAL DIAGNOSISEpendymoma, subepen- tricular mass. Symptomatic lesions are generally obstructive
dymoma, metastasis, subependymal giant cell astrocytoma leading to hydrocephalus. Spinal cord subependymomas are
(SEGCA). usually eccentric in location.
Subependymomas are slow growing, rare, low-grade WHO
DIAGNOSIS Subependymoma (incidental). I tumor usually located in the fourth and lateral ventricles;
about 50% to 60% of cases are in the fourth ventricle. They
DISCUSSION Subependymomas are usually solid, intra- are more common in men than in women usually in middle
ventricular tumors with relatively homogeneous signal inten- aged and elderly. Most are incidental and asymptomatic.
sities and often multiple not exceeding 2 cm in size. They Symptoms usually arise as a result of obstructive hydrocepha-
are generally hypointense to isointense to surrounding WM lus and may include headaches, nausea, visual disturbance,
on T1WI, and most do not contrast enhance. When they do, and loss of balance. The pathologic features consist of a
enhancement is usually heterogeneous and mild to moderate. matrix of dense fibrillary material containing scattered cluster

Case 173 375
of uniform cells with oval nuclei with perivascular pseudoro- What the Treating Physician Needs to Know
sette formation. There is usually absence of mitosis, atypia, Location, number, and size
and abnormal vascularization. Rare familial cases have been Non-contrast-enhancing intraventricular tumor is more
reported. Radical surgery is usually curative when possible. likely to be a subependymoma rather than anything else,
Radiation therapy may be used if the tumor progresses or and the diagnosis should be suggested to avail the patient
recurs. A shunt may be needed to treat hydrocephalus. the opportunity for radical surgical removal which is usu-
ally curative
Question for Further Thought Presence of hydrocephalus
1. What is the cell of origin of subependymoma?
Answer
Reporting Responsibilities 1. The cell of origin is debatable and include subependymal
All tumors deserve direct reporting. Presence of hydrocepha- glia (most likely), astrocytes of the subependymal plate,
lus makes direct reporting more urgent. ependymal cells, and a mixture of both.

Case
174 CLINICAL HISTORY Newborn female with prenatal diagnosis of vein of
Galen malformation (VGM) now presents with high-output cardiac failure.
FINDINGS Figure 174-1. Sagittal Doppler cranial ultra- (transverse arrow) and torcula/SSS (vertical arrow) are seen
sound. There is a large 3.5-cm vascular structure posteri- posteriorly to the aneurysm. Figure 174-3. Coronal T2WI
orly to the third ventricle and splenium of corpus callosum through the aneurysm. There is superior displacement of the
(appropriate position of the vein of Galen) (star) with two splenium of the corpus callosum (vertical arrow). Multiple
large feeding vessels demonstrated: a pericallosal artery round and tubular signal voids representing large affer-
and a posterior cerebral artery (arrows). Figure 174-2. Axial ent arteries (arrows) are seen surrounding the aneurysm.
FLAIR through the third ventricle. There is a 3.2 cm 3.1 Figure174-4. 3D TOF MRA of the head Submentovertical
cm signal void posteriorly to the thalami and third ventricle (SMV) view. There is direct connection of all major intra-
with mass effect consistent with the vein of Galen aneu- cranial vessels (arrows), anterior cerebral artery (ACA),
rysmal malformation (VGAM) (star). Large draining sinus posterior cerebral artery (PCA), and middle cerebral artery
376

Case 174 377
(MCA) to the aneurysmal malformation. Huge bilateral output may flow through the malformation. Thus, VGAM
transverse sinuses (chevrons) emanate from the torcula. produces a steal phenomenon, resulting in brain volume loss,
encephalomalacia, neurologic deficit, and calcifications.
DIFFERENTIAL DIAGNOSIS N/A. Congested superficial veins develop and these are prone
to rupture. Both parenchymal and intraventricular hemor-
DIAGNOSIS VGAM. rhages are features of VGAM. Hydrocephalus as a feature of
VGAM is fairly common due to obstruction of the aqueduct.
Definitive treatment of VGAM is mostly by endovascular
DISCUSSION The hallmark of the VGAM is a dilated
route with significant improvement in survival compared
persistent median prosencephalic vein of Markowski in the
with the pre-endovascular therapy time. Radiosurgery has
usual location of the vein of Galen behind the third ventricle
also been utilized.
and the splenium of the corpus callosum. There is usually no
visible vascular nidus. This is seen on prenatal and postnatal
Doppler ultrasound as a vascular pouch with very rich vascu-
lar connections. NCCT shows a hyperdense mass with mass 1. What is the embryologic origin of VGAM?
effect on surrounding structures, which contrast enhances
avidly following contrast administration. MRI particularly Reporting Responsibilities
FLAIR and T2WI shows a large smooth signal void fed by This deserves a direct reporting if the find is unsuspected.
a collection of tubular signal voids draining into the torcula. A confirmation of a suspected VGAM also deserves direct
It does enhance avidly with significant artifacts due to the reporting. Size of the malformation, afferent arteries, effer-
turbulent flow. The complications that can be demonstrated ent veins, and complications should be enumerated. DSA is
on CT and MRI include mass effect, parenchymal and intra- usually necessary to complete the evaluation of VGAM.
ventricular hemorrhages, calcifications, volume loss, gliosis,
encephalomalacia, and hydrocephalus. CTA and MRA are What the Treating Physician Needs to Know
capable of demonstrating the total geography of the lesion. Size of malformation, afferent arteries, and the efferent
CTA, however, may be challenging due to the fast flow. The veins/sinuses
mural type usually shows multiple identifiable feeders to the Associated parenchymal or extraaxial changes
pouch, while the choroidal type may show poorly defined Follow up changes. Some of these malformations have
numerous smaller feeders in a maze configuration anteriorly been known to thrombose spontaneously
to the aneurysm. VGAM should be distinguished from aneu- Complications following treatment
rysmal dilation of the vein of Galen caused by large arterio-
venous malformation (AVM) or dural fistula draining into Answer
the true vein of Galen. A definite nidus is present in an AVM 1. It is generally believed that VGAM forms between the
unlike VGAM. 6and 11 weeks of gestation following the development of
VGAM is frequently diagnosed in utero by ultrasound the Circle of Willis (COW). It is an arteriovenous fistula
as in this case. It may present in the neonatal period or in of the median prosencephalic vein (the precursor of the
infancy usually with high-output cardiac failure or develop- vein of Galen) and the primitive choroidal arteries. This
mental delay. Less severe low-flow VGAM may present later prevents the regression of this precursor vein and there-
in life. The mural type appears to be less severe compared fore formation of the proper vein of Galen and straight
with the choroidal type. Hence, most of the early present- sinus. It also retards proper brain development. Efferent
ing VGAMs are the choroidal type, while the late presenting flow is usually through a falcine sinus or other anomalous
VGAMs are mostly mural type. Up to 80% of the cardiac veins or sinuses into the SSS.

Case
175 CLINICAL HISTORY 18-year-old male presenting with two episodes
of seizures.
surrounding smudgy hyperintensity posteromedially (trans-

verse arrow) that is consistent with edema. There is moder-
ate mass effect with effacement of the overlying gyri and a
moderate amount of medial shift of the uncus. Figure 175-2.
Axial post-contrast T1WI. This shows the anterior cystic
component with enhancing thick posterolateral solid compo-
nent (arrow). Figure 175-3. Photomicrograph shows tumor
composed of haphazardly arranged ganglion/ganglioid cells
in a neuropil-rich stroma. Few eosinophilic granular bodies
(EGBs) are also noted. Glial cells are not prominent in this
area (H&E stain).
DIFFERENTIAL DIAGNOSIS Ganglioglioma (GG), dysem

bryoplastic neuroepithelial tumor (DNET), pleomorphic
xanthoastrocytoma (PXA), metastatic lesion, tumefactive
demyelination, oligodendroglioma, astrocytoma.
Figure 175-3
DIAGNOSIS Ganglioglioma (GG).
FINDINGS Figure 175-1. Axial T2WI through the tempo-
ral lobes. There is a 4-cm relatively sharply circumscribed DISCUSSION The temporal lobe is consistently found to
mass in the left temporal lobe. The anterior cystic compo- be the predominant lobe in which GG presents followed in a
nent has cerebrospinal fluid (CSF) hyperintensity with the distant second by the frontal lobe. The masses are well cir-
solid posterior component showing a slightly less hyper- cumscribed with varying degrees of cystic and solid com-
intense signal character (vertical arrow). There is minimal ponents. A cystic component is present in around 60% of
378

Case 175 379
cases. GG can present as completely cystic or a solid non- in eloquent areas or very large. GG is composed of haphaz-
infiltrating mass in a minority of cases. There may be asso- ardly arranged ganglion cells and glial cells. Ganglion cells
ciated calvarial scalloping related to slow growth of this could be small (ganglioid cell) or large with basophilic Nissl
mainly cortical lesion. On CT, GGs could be predominantly substance. Bi- or multinucleated forms are usually seen. The
hypodense with fewer cases being reported as hyperdense glial component could be of astrocytic (pilocytic or fibrillary)
and isodense. Hyperdensity is often related to the presence or oligodendroglial phenotype. Mitoses are none to rare but
of calcifications. GGs are usually hypointense on T1WI and similar to pilocytic astrocytoma. Vascular proliferation is not
hyperintense on T2WI. Calcification resulting in a bloom- uncommon. Other characteristic histologic features include
ing intensity on GRE is relatively common and has been EGBs, perivascular lymphocytes, and calcospherites. GGs
reported in about 40% to 50%. Enhancement is common in have been associated with DNET, cortical dysplasia, oligo-
GGs occurring in up to 95%, and GGs often have associated dendroglioma, and tanycytic ependymoma. Multifocal GGs
edema. The enhancement pattern is variable including solid have been reported.
strong enhancement, peripheral enhancement, focal nodular
enhancement, and marginal rim enhancement. GGs have Question for Further Thought
been found to have moderately elevated relative Cerebral 1. What is the standard therapy?
Blood Volume (rCBV) within the solid component. This
is significantly higher than that in low-grade gliomas 2.14 Reporting Responsibilities
1.67. High choline peaks are found in general in GGs. While GG is not an acute situation, like every tumor, direct
Choline peaks have been found elevated compared with reporting is important to enable prompt treatment. Location
N-acetyl aspartate (NAA) peaks and variable compared with is crucial to whether the mass could be completely excised
creatine. Calvarial scalloping is not a feature of oligoden- for seizure-free survival. Lesions in eloquent areas may
droglioma or astrocytoma. However, low-grade oligoden- benefit from functional imaging. Growth rate on follow-up
drogliomas can have higher perfusion (rCBV) values that evaluation should be documented.
might overlap the rCBV of GG. Presence of calcifications,
pattern of surrounding edema and contrast enhancement, and
cortical location distinguish GG from demyelinating lesions,
metastases, abscesses, and astrocytoma. DNET and PXA are Location and number if more than one
two other cortical lesions that may be difficult to differentiate Usefulness of functional imaging for tumors in eloquent
from GG. DNET often has a microcystic/bubbly appearance, areas
while PXA may show associated dural involvement. Presence of other associated lesions such as developmental
GGs are a low-grade WHO I tumors that arise in chil- venous anomaly, cortical dysplasia, DNET. These may affect
dren and young adults with an average age of presentation how tumor is treated
around 20 years. GG has equal male-to-female ratio. The
vast majority present with protracted medical refractory epi- Answer
lepsy. Focal neurologic deficits or symptoms of increased 1. Prompt recognition and complete resection are associated
intracranial pressure are present when tumors are located with a high epilepsy cure rate.

Case
176 CLINICAL HISTORY Newborn male with severe hydrocephalus.
FINDINGS Figure 176-1. Axial T2WI through the lateral brain surface again visualized. Figures 176-3 and 176-4.
ventricles. There is dilatation of the lateral ventricles (stars) Axial and coronal T2WI through the lateral ventricles
consistent with hydrocephalus. There is smooth brain sur- following shunting of the hydrocephalus. Convexity sulci
face (no sulci visible) with shallow sylvian fissures (arrows) have appeared (arrows); rather shallow with small gyri. There
and hourglass configuration of the cerebral hemispheres. is hypoplasia of the periventricular white matter (WM).
Figure 176-2. Coronal T2WI through the sylvian fissures. Figure 176-5. Axial NCCT through the lateral ventricles
Hydrocephalus with shallow sylvian fissures and smooth in a companion case. There is hour glass deformity of the
380

Case 176 381
Lissencephaly, also known as smooth brain, is a het-

erogeneous group of disorders of cortical formation charac-
terized by absent or hypoplastic sulci. Two major types are
recognized: the type I or classic lissencephaly and the type
II or cobblestone lissencephaly (congenital muscular dystro-
phy). Each of the types has several subtypes. The genetic
subtypes of the classic lissencephaly are LIS1 gene with
equal malefemale ratio with mostly parieto-occipital agyria,
doublecortin gene usually seen in female with mainly frontal
lobes gyral abnormality, and the ARX gene characterized by
frontal pachygyria and parietooccipital agyria. The subtypes
of the cobblestone lissencephaly are the Walker-Warburg
syndrome characterized by diffuse cobblestone lissenceph-
aly, unmyelinated WM, muscular dystrophy, ocular abnor-
mality, progressive macrocephaly with hydrocephalus and
posterior cephalocele; the Fukuyama congenital muscular
dystrophy characterized by congenital muscular dystrophy,
frontal polymicrogyria with parietooccipital cobblestone
lissencephaly and chromosome 9q31-33 abnormality; the
muscleeyebrain disease due to abnormality of chromo-
some 1p32-p34 presenting with muscular dystrophy, mild
Figure 176-5
ocular abnormality, developmental delay, and mild poor
sulcation of the frontal lobes with hypomyelination of the
WM. Clinical presentation includes developmental delay,
seizures, and hypotonia.
cerebral hemispheres with thick smooth cortical gray mat- Genetic testing in the anchor case was negative for
ter (GM) and no visible sulci consistent with lissencephaly/ Walker-Warburg syndrome and for muscular dystrophy.
pachygyria spectrum. There is hypoplasia of the WM par- Thelesson to learn from this case is that hydrocephalus
ticularly in the parietooccipital lobes (arrows). could completely efface all sulci resulting in a false-positive
lissencephaly.
DIFFERENTIAL DIAGNOSIS Lissencephaly, hydroceph-
alus, subcortical band heterotopia. Question for Further Thought
1. What are the causes of lissencephaly?
DIAGNOSIS Hydrocephalus presenting as lissencephaly.
DISCUSSION The initial consideration for the anchor Hydrocephalus warrants direct reporting. Otherwise, routine
images was lissencephaly with hydrocephalus perhaps the reporting is sufficient.
Walker-Warburg type where hydrocephalus is a component.
The hourglass configuration of the cerebral hemispheres, the
shallow sylvian fissures, the smooth brain surface, the appar-
ently very thin cortical GM, and the lack of sulcation s uggest The severity and locations of the brain malformations
lissencephaly. Following shunting of the hydrocephalus, Other associated changes
there is appearance of rather small gyri with shallow sulci,
not quite the cobblestone expected in Walker-Warburg? Answer
There is also significant hypoplasia of the WM. The compa 1. There is genetic underpinning to the various forms of lis-
nion case shows a smooth brain surface, hourglass configura- sencephaly. Viral infection in utero and/or insufficient
tion of the cerebral hemispheres with a rather thick cortical blood supply to the fetal brain early in pregnancy have
GM consistent with pachygyria lissencephaly spectrum. been implicated.

Case
177 CLINICAL HISTORY Motor vehicle accident with anterior and central skull
base fractures 1 year ago. Patient is presenting with persistent headaches now.
FINDINGS Figure 177-1. Axial CTA image, bone window sinuses (star). There appears to be a tenuous tract through
through the ethmoid. There is comminuted ethmoidal frac- the planum on the left (transverse arrow). Figures 177-3 and
ture. Additionally, there is a fracture through the sphenoid 177-4. Axial heavily T2WI fast imaging employing steady
roof at the skull base with pneumocephalus in the region state acquisition (FIESTA) and FLAIR, respectively, in a
of the suprasellar cistern (arrows). Figure 177-2. Coronal companion case. There is hyperintense collection anterior to
T2WI through the planum sphenoidale 1 year later. There is the left middle cranial fossa (star) through a defect in the
encephalomalacia and gliosis of the bilateral subfrontal lobes dura and the left greater wing of sphenoid. Fluid collection
(vertical arrows). Additionally, there is hyperintense opaci- abuts and compresses the left lateral rectus (arrows). The
fication in the region of the posterior ethmoid and sphenoid fluid suppresses on FLAIR in Figure 177-4.
382

Case 177 383
DIFFERENTIAL DIAGNOSISGrowing skull fractures, A growing skull fracture (leptomeningeal cyst) is a rare
pseudomeningocele, leptomeningeal cyst, sinus retention but well-documented complication of craniofacial trauma
cyst and infection. less commonly seen following fractures of the skull base.
A well-defined fracture of the skull base in association with
DIAGNOSIS Posttraumatic cerebrospinal fluid (CSF) traumatic dural injury results in exposure of the fracture to the
collection (pseudomeningocele). pulsations of the cerebrospinal fluid within the subarachnoid
space. Pulsatile pressure begins to gradually widen the frac-
DISCUSSION High-resolution CT at the level of skull ture line permitting the dura/meninges to prolapse through
base is highly sensitive for demonstrating skull base frac- the defect. The content may be slightly hyperintense on T1WI
tures acutely, and the lack of healing, bony resorption or reflecting slightly more proteinaceous contents within the
growing fractures may be identified on follow-up imaging. leptomeningeal cyst as compared with a pseudomeningocele.
Advanced MR techniques such as T2 FIESTA, FLAIR, With infection, abscess will present with single or mul-
T1, and T2 sequences can be extremely helpful in determin- tiple fluid pockets with poorly defined margins associated
ing the extent of posttraumatic fluid collections and often- with overlying soft tissue swelling and edema. The exact
times permit identification of the direct communication with constellation of findings is related to the path of spread of the
the subarachnoid space (as shown in our companion case). infection. The fluid collections associated with infection do
The fluid collection in pseudomeningoceles should be the not follow the imaging characteristics of CSF.
same as the CSF within the ventricles and the subarachnoid
space on all MRI sequences. Questions for Further Thought
For definitive documentation of communication of the 1. What is the natural history of this disease?
fluid collection with the subarachnoid space and identifi- 2. What treatment options are there?
cation of the exact point of communication, radionuclide
cisternography or CT cisternography is required. For radio-
nuclide cisternography, indium is instilled into the thecal sac
Direct reporting is essential in view of the possible com-
via lumbar puncture and the patient tipped head down on
plications. Location and density or signal intensity of the
the fluoroscopy table in order to advance the radionuclide
fluid collections and their proximity to the location of the
into the intracranial subarachnoid space. Imaging is obtained
skull base fracture and the presence of complications such
immediately and delayed in order to define the location of
as pneumocephalus, empyema, or other infection must be
the communication. Nasal and external auditory canal pled-
urgently reported.
gets may be employed and counted for radioactivity when
active CSF leak is suspected. For CT cisternography, myelo-
graphic nonionic iodinated contrast is instilled into the thecal
sac via lumbar puncture and the patient tipped head down on Location, size, and relationship to adjacent structures
the fluoroscopy table in order to advance the contrast into the Presence/absence of complications as assessed by imaging
intracranial subarachnoid space. Direct coronal CT imaging Usefulness of cisternography for identification of the tract
is extremely useful in the depiction of contrast leaking into
the dependent fluid collection in the setting of a skull base Answers
fracture. 1. Increasing fracture diastasis over time may lead to prolapse
Asymptomatic or minimally symptomatic cases of post- of meninges and meningocele formation. Progressive
traumatic fluid collection without active CSF leak may be neurologic deficits may develop from low CSF pressure
identified and subacutely treated surgically. When active states and from infection in cases of active CSF leak.
leak is present, the increased risk of meningitis, empyema, 2. All of these complications of skull base fractures are
or infectious complications warrants emergent surgical treated surgically with the addition of antibiotic therapy
treatment. in those patients with active CSF leak and/or meningitis.

Case
178 CLINICAL HISTORY Newborn with occipital mass. Two CT images
from posterior fossa evaluation.
FINDINGS Figures 178-1 and 178-2. NCCT through two include corpus callosum dysgenesis and gray matter hetero-
levels of the posterior fossa. There is protrusion of the cere topia. Features of CIIIM could be present, but isolated OC
bellum, cerebrospinal fluid (CSF), and covering meninges usually does not have the array of supratentorial changes
through a large occipital bone defect (arrows). This produces seen in CIIIM. In utero diagnosis could be made by US and
a large bulge in the suboccipital region. The fourth ventricle fetal MRI. This could be useful in planning the delivery of
is effaced. these babies.
OC is usually present at birth and could undergo rapid
DIFFERENTIAL DIAGNOSIS Occipital cephalocele (OC), increase in size in the postnatal period. Other presenting
Chiari III malformation (CIIIM). features include developmental delay, hindbrain symptoms,
and paraparesis. OC forms about 75% of all cephaloceles
DIAGNOSIS Occipital cephalocele (OC). in the Western hemisphere compared with frontobasal pre-
dominance in Asia and Africa. There is female predomi-
DISCUSSION OC is a protrusion of posterior brain struc- nance 2:1 but up to 70% in one series. The prognosis of
tures through an occipital bone defect. The content of the OC depends on the size, the contents of the sac, presence of
cephalocele depends on the location above or below the hydrocephalus, and other associated congenital anomalies. It
torcula. This may include the cerebellum, brainstem, fourth is recommended that early surgery be performed to remove
ventricle, occipital lobes, lateral ventricle, CSF, and obvi- the cephalocele and repair the bone defect. Hydrocephalus
ously overlying meninges. CT demonstrates a variable-sized is usually treated by shunting. Pathologic evaluation of the
defect in the occipital bone and the sac content of brain and contents usually shows dysgenetic brain tissue that is not
CSF. The detail of the content is not usually well delineated adequately demonstrated at imaging.
by CT. These are better delineated by MRI. Microcephaly
is a common finding in these patients. Hydrocephalus is
more frequently encountered postoperatively. Supratentorial Question for Further Thought
associated abnormalities are present in about 50%, and these 1. What are the goals of imaging in OC?
384

Case 178 385
Reporting Responsibilities Differentiating the isolated OC from CIIIM could be diffi

Routine reporting is generally sufficient, but presence of cult. The management is probably not too different
hydrocephalus should be reported directly. For prognosis MRI may not be able to define the pattern of dysgenesis of
purposes, all associated abnormalities should be defined. Of the brain content of the OC sac
cause MRI and MRV should be recommended in this regard.
It may also be necessary to study the arterial anatomy by Answer
MRA. 1. To define the bone defect, the sac content, the vascular
structures, and other associated anomalies.
MRI is superior to other imaging modalities
MRV may be necessary for proper evaluation of the
venous sinuses that could form part of the sac content

Case 179 CLINICAL HISTORY 47-year-old right-handed female presenting with
seizures. These are follow-up images after subtotal resection of original lesion.
Figure 179-1
Figure 179-2
The solid c omponents are hyperintense and project into the

right corona radiata (arrow), while the cystic components
showCSF hypointensity. Figure 179-3. Axial T2WI through
the corona radiata. The solid components are somewhat hyper-
intense but brighter than surrounding white matter (WM).
Figure179-4. Axial DTI color directional map through the
CC. There is disruption of the red CC fibers (vertical arrows)
and the adjacent right superior fronto-occipital fasciculus and
the superior internal capsule (transverse arrows).
DIFFERENTIAL DIAGNOSISGlioblastoma (GB), lym-

phoma, tumefactive or toxic demyelination, oligodendroglioma.
Figure 179-3 DIAGNOSIS CC oligodendroglioma II (ODG II) recurrent.
DISCUSSION CT and MRI are complementary techniques

FINDINGS Figure 179-1. Sagittal T1WI through the corpus for imaging ODG. Tumor calcification is better defined on
callosum (CC). There is a mixed cystic anteriorly (cerebro- CT scans than on MRI, but MRI is till the examination of
spinal fluid [CSF] intensity) and solid hypointense (posteri- choice. Conventional MRI of the brain is crucial in the diag-
orly) mass in the midbody of the CC (arrow) projecting to nosis, staging, treatment, and follow-up of brain tumors.
the septum pellucidum inferiorly. Post-contrast T1WI (not ODGs are relatively hypointense on T1WI and hyperin-
shown) demonstrated very minimal contrast enhancement. tense on T2WI. Peritumoral edema, uncommon with ODG,
Figure 179-2. Axial FLAIR through the corona radiata. is depicted as hyperintensity on T2WI and FLAIR. Mixed
386

Case 179 387
of the CC and the contralateral hemisphere. The patient had a

repeat stereotactic biopsy confirming ODG II. Fluorescence
In Situ Hybridization analysis (FISH) analysis on the tissue
demonstrated co-deletion of 1p/19q. As the lesion was not
amenable to resection, she was offered chemotherapy with
temozolomide and radiation.

1. What is the treatment of ODG?
2. What is the survival rate of patients with ODG?
Direct reporting is essential in a recurrent tumor. Changes
in the original location of tumor should be mentioned, while
changes of aggressive behavior, crossing CC and infiltrating
the corona radiata, should be reported.

Extent and progression of tumor along with possible com-
plications of herniation or hydrocephalus
Although this was an ODG II, the imaging behavior of
invasion and crossing the CC and involvement of the con-
tralateral hemisphere suggest anaplastic ODG; hence, the
treatment recommendation is that for aggressive ODG
Tumors with 1p/19q co-deletion benefit particularly from
the addition of procarbazine/lomustine and CCNU are the
Figure 179-4 same/vincristine (PCV) chemotherapy to radiation therapy.
Side effects of PCV is, however, very significant and prob-
ably translate into reduced quality of life for long-term sur-
vivors. Therefore, temozolomide which has less severe side
cystic and solid regions could be present. Hemorrhage and effects is replacing PCV in the treatment of these tumors.
calcifications may be present in the mass depicted as areas of The long-term benefit of temozolomide is unknown
blooming on GRE. Post-contrast MRI is always performed
as it has a prognostication value; completely resecting Answers
enhancing tissue (more common in ODG III) independently 1. The treatment of an ODG should be individualized
improves outcome irrespective of histologic grade or genetic depending on the presence or absence of symptoms, loca-
status. Physiologic MRI techniques such as perfusion, diffu- tion, and biologic aggressiveness of the tumor, extent of
sion, and functional MRI add information to the microscopic possible surgical resection, histopathology, and degree
architecture of the tumor but might not necessarily differen- of anaplasia. Treatment options vary from conservative
tiate between the different types of tumors or the differential treatment with serial imaging studies and no intervention
diagnosis. to aggressive multimodal treatment including surgical
Primary ODG of the CC is rare. Initial imaging of the resection, radiotherapy, and chemotherapy.
brain showed a right frontoparietal tumor (images not 2. Overall, as many as 75% of patients with nonanaplastic
shown). She had a subtotal resection, and histology was that tumors have a median reported survival duration of 6 to
of ODG II. Follow-up MRI showed an increase in the size of 10 years. For those with anaplastic ODGs, median sur-
the residual lesion, with the latest MRI showing infiltration vival is about 3 to 4 years.

Case
180 CLINICAL HISTORY 55-year-old male with altered mental status.
FINDINGS Figure 180-1. Axial NCCT through the pons. hematoma, location of the insult, and effectiveness of therapy
There is hyperdense material in the pons (arrow). There is to prevent further hemorrhage. Intraventricular extension of
mild expansion of the pons. Figure 180-2. Susceptibility blood worsens the prognosis and may lead to hydrocephalus.
MRI. There is corresponding hypointense signal charac
teristic of blood with expansion of the pons. Question for Further Thought
1. What is the appropriate imaging management for a patient
DIFFERENTIAL DIAGNOSIS Hypertensive hemorrhage, with spontaneous primary intracerebral hemorrhage
hemorrhagic tumor, hemorrhagic vascular lesion, amyloid (PICH)?
angiopathy.
DIAGNOSIS Hypertensive brainstem hemorrhage. Direct reporting is necessary in all acute hemorrhage. Identify
the hemorrhage, describe the size and location, and describe
DISCUSSION Hypertensive intracerebral hemorrhage any associated intraventricular or subarachnoid extension.
refers to parenchymal hemorrhage secondary to rupture of a Presence of hydrocephalus due to either mass effect or intra-
small vessel previously damaged by long-standing or poorly ventricular hematoma (IVH) should be mentioned.
controlled hypertension. Non-contrast CT is in most cases
sufficient to identify the hematoma which is hyperdense.
The degree of brainstem swelling depends on the size of the
hematoma, and this progresses for the next few days. MRI Hypertensive hemorrhage is a fairly common sequela to
may give additional information regarding the age of the long-standing or poorly controlled hypertension
blood, and the likelihood of an underlying lesion particularly Mortality is high, particularly with large bleeds and with
when it is done with contrast. GRE images may demon- intraventricular extension
strate presence of microhemorrhages elsewhere in the brain. Imaging is critical to exclude an underlying lesion
Contrast enhancement may help differentiate underlying
neoplasm from primary intracerebral hemorrhage (PICH). Answer
Amyloid angiopathy is rare in the brainstem. 1. All patients with PICH deserve MRI and noninvasive vas-
Histologically, affected vessels demonstrate atheroscle- cular imaging (CTA or MRA) to exclude an underlying
rosis and fibrinoid necrosis which weakens their walls pre- tumor or vascular lesion. For patients over 45 year of age,
disposing to rupture. Common locations include, in order of with documented hypertension, and with hemorrhage
decreasing frequency: putamen and external capsule, thala- in a typical location, no further imaging investigation
mus, pons and posterior fossa, and cerebral lobes. Patients is required. In patients younger than 45, with no known
often present with sudden loss of consciousness. There may history of hypertension, or with an unusual pattern of
be respiratory issues requiring intubation due to the location hemorrhage, conventional angiography is recommended
of the hemorrhage. Prognosis is dependent on the size of the as a final step to evaluate for an occult vascular lesion.
388

Case
181 CLINICAL HISTORY 45-year-old male with increasing headaches.
New right-sided weakness.
Figure 181-2
Figure 181-1
Figure 181-4
FINDINGS Figure 181-1. Axial NCCT through the corona

radiata. There are four small well-circumscribed hyper-
dense masses (arrows) with surrounding hypodense edema
in juxtacortical locations in the two hemispheres. The right
parasagittal mass shows a hyperdense periphery surround-
ing a hypodense core. There were many other lesions with
Figure 181-3 similar configuration elsewhere in the brain (not shown).
389

390 Case 181
metastases, meningoencephalitis, cerebral cavernous mal-

formation (CCM), arteriovenous malformation (AVM), sep-
tic emboli, aneurysms and usually has contrast enhancement
and perilesional edema. HM could be single or multiple
and are usually hyperdense on CT with surrounding vaso-
genic edema. MRI is more sensitive than CT for the evalu-
ation of metastases in general revealing two to three times
more metastases than CT. It is the preferred examination of
choice. HM are typically hyperintense on T1WI with some
heterogeneity and may show areas of nodular or rim contrast
enhancement. They are T2 hyperintense with GRE bloom-
ing. Necrotic masses may show fluid levels. Presence of a
hypodense core on CT and target sign on MRI in this patient
should raise suspicion of a SICH most importantly neoplas-
tic. However, nonhemorrhagic melanoma to the brain tends
to appear hyperintense on T1WI and hypointense/isointense
on FLAIR and T2WI similar to neurocutaneous melanosis.
Hyperintensity on T1, FLAIR, and T2WI in such a tumor
Figure 181-5 is in favor of hemorrhage. Calcification is a feature of
neurocysticercosis.
Differential diagnosis of multifocal HM includes mela-
Figure181-2. Axial non-contrast T1WI through inferior noma, choriocarcinoma, renal and thyroid carcinomas. They
posterior fossa. There is a right cerebellar peripherally placed are uncommon but do occur in breast, lung and colorectal
target lesion with alternating rings of hyperintensity and carcinomas, and rarely in atrial myxoma. Advanced imaging
hypointensity (arrow). There were many lesions with simi- such as perfusion, MRS, or DTI do not appear to be able to
lar configuration elsewhere in the brain. Figure181-3. Axial differentiate between the various types of metastases.
T2WI through the largest lesion in the right peritrigonal HM represent less than 15% of all metastases. Their clini-
region. The mass is hyperintense with eccentric anterolat- cal presentation could be insidious. Headache, seizures, and
eral target configuration. There is a hypointense thin smooth focal neurologic deficits are some of their features. Presence
rim and surrounding hyperintense vasogenic edema. Other of brain metastases almost always alters the treatment para-
T2 lesions show a variegated intensity pattern. The tri- digm of the patient. Single metastasis could be surgically
gone is compressed. Figure181-4. Axial GRE through the removed. Multiple brain metastases invariably require multi-
centrum semiovale. Two lesions (arrows) with variegated modality treatment. The prognosis is generally poor.
mostly hypointense signal with hypointense rims and sur-
rounding edema are present, one in each posterior frontal Question for Further Thought
lobes in juxtacortical locations. Figure 181-5. Coronal post-
1. Is it possible to differentiate melanotic and amelanotic
contrast T1WI through the frontal lobe masses. Since the
melanoma metastases to the brain?
lesions were T1 hyperintense, it is difficult to visualize con-
trast enhancement. Some blurring of the hypointense ring of
the target is, however, noted. Reporting Responsibilities
Direct reporting is necessary for tumors and hemorrhages.
DIFFERENTIAL DIAGNOSISNeurocutaneous melano- Presence of significant mass effect and herniations should
sis, melanoma, multifocal primary intracerebral hematoma be highlighted.
(PICH), multifocal hemorrhagic metastases (HM), multifo-
cal neurocysticercosis. What the Treating Physician Needs to Know
Location, number, and size of lesions. Patient may require
DIAGNOSIS Multifocal HM (melanoma). biopsy for diagnosis
Degree of edema, mass effect, and any herniations
DISCUSSION There is a history of prior surgical removal It is difficult to distinguish between the various types of
of melanoma in the back in this patient. HM at imaging
Multifocal brain parenchymal hemorrhages can be pri-
mary (PICH) or secondary (SICH). PICH could be due to Answer
hypertension, amyloid angiopathy, and coagulopathy and 1. Melanoma metastases exhibit a variable MR pattern that
are usually hyperdense on CT with minimal perilesional is dependent on several factors. The melanotic mela-
edema and thin perilesional ring enhancement in the sub- noma contains melanin pigment which exhibits paramag-
acute phase. SICH could be due to high-grade brain tumors, netic characteristics and results in shortening of T1 and

Case 181 391
T2 relaxation times. The typical melanotic melanoma is like most other metastases and hyperintense or isointense
hyperintense in relation to cortex on T1WI, hypointense on T2WI and FLAIR. Contrast enhancement is variable
in relation to cortex on T2WI, and isointense or hyperin- but mostly avid. However, the classical findings may not
tense in relation to cortex on FLAIR. The typical amela- be present with a significant crossover of signal patterns
notic melanoma is hypointense or isointense on T1WI in the two types.

Case
182 CLINICAL HISTORY 37-year-old female with acute impairment of
mental status.
Figure 182-2
Figure 182-1
FINDINGS Figure 182-1. Axial T2WI through the thalami. areas of hypointensity in T2WI documenting hemorrhage.
There is bilateral thalamic hyperintensity with hypointense Venograms both 3D TOF and phase contrast show absent
focal areas within it due to hemorrhage and mass effect flow-related enhancement in the deep venous system includ-
(arrows). Figure 182-2. 3D TOF MRV showing absent signal ing the internal cerebral veins, vein of Galen, and straight
in internal cerebral veins, vein of Galen and straight sinus. sinus. The anterior portion of the superior longitudinal sinus
may also be involved.
DIFFERENTIAL DIAGNOSISTumor with invasion or Patients clinically present with rapid neurologic deteriora-
compression of dural venous sinuses, deep venous sinus tion, although some may show abulia, executive deficits, and
thrombosis, polycythemia or dehydration (reveal hyperdense amnesia. The deep cerebral venous system comprises inter-
blood in patent dural sinuses), hypoplasia/aplasia of dural nal cerebral vein, vein of Galen, and straight sinus. DVST
sinuses, intrasinus arachnoid granulation. occurs in 16% of patients with cerebral venous thrombosis,
and it increases the risk of mortality, although recanalization
DIAGNOSIS Bithalamic hemorrhagic infarcts caused by rates are higher in deep cerebral veins and cavernous sinus
deep venous sinus thrombosis. compared with others dural sinuses.
For screening purposes the unenhanced CT is the method
DISCUSSION Bilateral thalamic hemorrhagic infarct of choice; however, if there is high clinical suspicion, con-
should raise suspicion of underlying deep venous sys- trast-enhanced CT should be performed and complemented
tem thrombosis (DVST), which may also affect the basal by noninvasive venographic studies. CT venography has
ganglia. Patients may show swollen thalami with areas of proved better diagnostic accuracy compared with MR venog-
hyperintensity on T2WI suggestive of edema and central raphy using TOF or phase contrast techniques.
392

Case 182 393
Table 182-1 Methods of Evaluating Cerebral Venous Sinus Thrombosis

Technique Findings Pitfalls
NCCT Dense clot sign 10%30% negative
CECT Empty delta sign (might not be seen in DVST); False negatives if dense thrombus
falx and tentorium enhancement due to venous Fenestrated dural sinus mimics thrombus
stasis and hyperemia 10%30% negative
CT venography Filling defect
MRI Bright thrombus on T1WI, in early subacute
phase, due to methemoglobin (MHb), high signal
on T2/FLAIR and blooming on T2* WI
MRV with contrast Peripheral enhancement of veins TOF false positives from in-plane flow and
false negatives due to MHb; phase contrast
sensitive to artifacts and turbulent flow;
post-contrast false negatives due to MHb
Questions for Further Thought Answers

1. Which is the best diagnostic imaging method to detect 1. Conventional MRI, when available, in combination
DVST? with CT venography is the appropriate method to assess
2. Are there risk factors for DVST? DVST, although each institution may define their best
protocol for these situations. See table 182-1.
Reporting Responsibilities 2. Hyperhomocysteinemia, hypercoagulable state in preg-
Direct reporting is essential in this acute event requiring nancy and puerperium, use of oral contraceptives, vascu-
urgent treatment. Description of imaging findings on paren- litis, and infections.
chymal structures and deep venous dural system.

Nature of imaging abnormalities affecting the thalami and
the presence and extent of DVST

Case
183 CLINICAL HISTORY 3-year-old male presenting with delayed growth
and found to have hypopituitarism.
FINDINGS Figures 183-1 and 183-2. Coronal and sagit- two stalks and thickening of the tuber cinereum on both
tal non-contrast T1WI, respectively. There is a hyperin- T1WI and T2WI. CT shows a small sella turcica in EPL and
tense nodular small mass (neurohypophysis) (arrows) at a wide sella turcica in DPG, as well as other osseous skull
the expected location of the junction of the infundibulum base abnormalities. A small lipoma could mimic ANH but
to thehypothalamus just behind and inferiorly to the chi- the constellation of associated congenital findings should
asm. The infundibulum is absent. The adenohypophysis exclude the differentials.
is barely visible. The normal intrasellar hyperintense ANH is probably a result of abnormal interaction
posterior pituitary lobe is not seen. between the Rathke pouch (ectoderm) and the diencephalic
neuroectoderm caused by genetic mutations. The causes of
DIFFERENTIAL DIAGNOSIS Suprasellar lipoma, Rathke EPL include trauma (particularly at birth) and destruction
cleft cyst, basilar artery tip aneurysm (especially if partially of the stalk by ischemia. When the stalk is nonfunctioning,
or completely thrombosed), small craniopharyngioma, aber- hormones produced in the hypothalamus cannot reach the
rant neurohypophysis (ANH). posterior intrasellar lobe reservoir and may accumulate any-
where along the downward path of migration. Because these
DIAGNOSIS Aberrant neurohypophysis (ANH). hormones are lipid rich, the normal positioned and EPLs
are hyperintense on T1 sequences. When there is EPL, the
DISCUSSION ANH refers to a rare congenital or acquired adenohypophysis is small due to lack of stimulation by
anomaly of the hypothalamic and pituitary axis whereby hypothalamic-releasing factors that can no longer use the
the neurohypophysis is located in places other than pos- stalk to migrate inferiorly. Thus, patients tend to present
teriorly in the sella turcica. ANH include mainly ectopic with hormonal abnormalities related to the anterior lobe
posterior lobe (EPL) and duplicated pituitary gland (DPG), while posterior lobe hormones continue to be secreted by
both found as incidental findings but sometimes found in the EPL into the pituitary portal system. On the other hand,
patients with pituitary deficiency (particularly growth hor- DPG is commonly related to duplication of the stalk and of
mone deficiency). EPL can be seen on the undersurface of the sella, and sometimes with hypothalamic dysmorphism
the median eminence of the hypothalamus or at the infe- such as fusion of mammillary bodies and tuber cinereum.
rior end of the truncated pituitary stalk. MRI is the imag- All the cases of DPGs have shown accompanying abnor-
ing modality of choice. The neurohypophysis is normally malities of the face or brain especially midline clefting
hyperintense on T1WI. EPL therefore could be seen as a syndrome or associated with midline brain malformations,
hyperintense structure on T1WI located at the median emi- pituitary dwarfism, and Kallmann syndrome. Although
nence in the floor of the third ventricle or along a truncated there is no treatment for these hypothalamic and pituitary
pituitary stalk since the pituitary stalk is usually absent or anomalies, hormonal supplementary treatment is adminis-
thin in this situation. In contrast, DPG usually demonstrates tered when associated with pituitary dysfunction.
394

Case 183 395

1. What midline anomalies have been associated with EPLs 1. EPLs have been associated with hamartomas of the tuber
and DPGs? cinereum, septo-optic dysplasia, Chiari I malformation,
lobar holoprosencephaly, olfactory bulb anomalies, agenesis
Reporting Responsibilities of the corpus callosum, persistent craniopharyngeal canal,
Routine reporting is sufficient. Other associated midline and cerebellar vermian dysplasia. DPGs have been associ-
anomalies depending on the type of ANH should be recorded. ated with hypertelorism, cleft palate, mouth and tongue
dysmorphism, persistence of the craniopharyngeal canal,
midline clival defects, choanal atresia, and ectopic adenohy-
What the Treating Physician Needs to Know pophyseal, and hamartomatous pharyngeal masses among
Identifiable hypothalamicpituitary axis abnormalities that other disorders.
can explain the pituitary dysfunction that occurs in these
patients
Presence of other accompanying midline anomalies

Case
184 CLINICAL HISTORY 51-year-old male 10 years post kidney and
pancreas transplant with homonymous hemianopsia.
FINDINGS Figure 184-1. Axial DWI through the parietal Sagittal post-contrast T1WI. There is a thick smooth ring
lobes. There is a focus of hyperintensity (restricted diffu- enhancement of the lesion. The ring is attenuated inferopos-
sion) in the right parietal lobe (vertical arrow). Figure 184-2. teriorly (arrow). Edema extends into the occipital lobe infe-
Axial T2WI through mass. There is an ovoid right parietal riorly. Figure 184-5. Axial GRE through the mass. The mass
isointense (with white matter [WM]) rim surrounding a shows a thick hypointense rim (arrow) surrounding a small
core of mild hyperintensity (transverse arrow). There is sur- hyperintense core with surrounding edema. Figure 184-6.
rounding hyperintense vasogenic edema (vertical arrow). Photomicrograph shows large lymphoid cells with atypi-
Figure184-3. Axial T1WI through the mass. The mass is cal hyperchromatic nuclei; some with prominent nucleoli
hypo/isointense (arrow) with surrounding hypointense (monomorphic posttransplant lymphoproliferative disease
vasogenic edema. There is local mass effect. Figure 184-4. [PTLD]/diffuse large B-cell lymphoma) (H&E stain).
396

Case 184 397
DIFFERENTIAL DIAGNOSIS PTLD, glioblastoma (GB), for Epstein-Barr virus (EBV) is highly suggestive of the
lymphoma, metastasis, demyelination, infection, neurosar- diagnosis, but biopsies are often obtained. The CSF PCR
coidosis. for EBV can be positive even when PCR on the peripheral
blood is negative. PTLD could be polymorphic or mono-
DIAGNOSIS PTLD. morphic, depending on its constituent cells phenotype.
Polymorphic PTLD shows the full range of B-cell matura-
DISCUSSION Central nervous system (CNS) PTLD is tion. Monomorphic PTLD is composed of monomorphic
a rare disorder and has many similar imaging character- large cell with basophilic cytoplasm and prominent nucle-
istics to CNS lymphoma. The majority of patients tend to oli. The majority of PTLDs fall into the category of diffuse
have multifocal disease. The imaging appearance is often large B-cell lymphoma.
suggestive of lymphoma, particularly given the clinical The survival is quite variable, and treatment almost
history, and the majority of the lesions are supratentorial, invariably involves decreasing or withdrawal of immunosup-
periventricular, or in the basal ganglia region. This one pressive therapy in the first instance. Mortality is very high in
happens to be lobar in location. On CT the mass is isodense nonresponders to immunosuppressive therapy modification.
to hyperdense because of its hypercellularity. PTLD is This mass was surgically excised.
hypointense on T1WI and hypointense to hyperintense
on T2WI. The lesion often has restricted diffusion related Questions for Further Thought
to hypercellularity. The presence of hemorrhage can also 1. What is the drug of choice in the treatment of CNS PTLD?
contribute to restricted diffusion, but hemorrhage is an 2. Is brain irradiation useful?
unusual feature of CNS PTLD. Edema often surrounds the
lesion. CNS PTLD often presents with ring enhancement Reporting Responsibilities
resembling AIDS-related lymphoma. Ring enhancement is Direct reporting is important in view of the life-threatening
not associated with CNS lymphoma in immunocompetent nature of the disease. Significant mass effect or herniation
patients. Associated or isolated leptomeningeal multifocal makes reporting more urgent. Location is important particu-
contrast-enhancing lesions may be present. There is limited larly regarding biopsy. Leptomeningeal disease when pres-
literature on perfusion imaging in CNS PTLD. Differential ent may aid diagnosis through lumbar puncture (LP).
diagnosis may include metastasis, GB, toxoplasmosis,
demyelination, or abscesses. GB is always a consideration What the Treating Physician Needs to Know
in any ring-enhancing brain lesion with surrounding edema. Location is important for planning biopsy to differentiate
GB is, however, more irregular. this entity from infection or lymphoma
CNS PTLD is a very rare tumor due to immunosuppres- Is it safe to perform a LP to aid diagnosis? Presence of sig-
sion and occurs several years following transplantation, nificant mass effect and herniation may contraindicate LP
mainly solid and bone marrow transplants. It tends to occur Could this represent an abscess? In the posttransplant
more frequently in children than adults with no gender pref- period, infection is always a possibility particularly if the
erence. Clinical presentation depends on location. Positive lesion restricts diffusion and has ring enhancement. Early
cerebrospinal fluid (CSF) polymerase chain reaction (PCR) treatment is very important for survival in abscess

398 Case 184
Answers patients given the side effects of methotrexate particu-

1. There is limited clinical experience treating CNS larly its renal toxicity that is of concern in renal trans-
PTLD due to its rarity. Rituximab, an anti-CD20 plant patients.
monoclonal antibody either alone or in combination 2. Brain irradiation has been shown to be an effective
with anthracycline-based chemotherapy has been used. treatment for CNS PTLD. However, there are concerns
Methotrexate alone or in combination with rituximab related to delayed effects on CNS function in longer-term
can be utilized successfully, particularly in young fit survivors.

Case
185 CLINICAL HISTORY Normal MRI anatomy of the brain.
Third ventricle with nodular mammilary body

inferiorly to it
Medial surface of the cerebral hemisphere
Body of corpus callosum with star in the genu
Splenium of corpus callosum
Tectal plate superior and inferior colliculi posterior

to aqueduct of Sylvius in midbrain
4th ventricle with cerebellum behind it
Medulla with pons (triangle) superiorly
Pituitary gland in sella turcica
Figure 185-1
Sphenoid sinus
Internal carotid artery
Basilar artery
Internal ear structures vestibule, cochlea and SCC
Brachium pontis middle cerebellar peduncle
Cerebellum
4th ventricle inferior
Pons
Figure 185-2
399

400 Case 185
Anterior interhemispheric fissure with gyrus rectus on either side
Orbital gyrus
Left MCA in lateral fissure
Suprasellar cistern with chiasm, infundibulum and

mamillary body projecting into it
Interpeduncular cistern between the cerebral peduncles
Aqueduct of Sylvius
Right temporal lobe
Occipital lobe
Figure 185-3
Genu of corpus callosum
Anterior falx with frontal lobes on either side
Frontal lobe WM; forceps minor
Head of caudate nucleus hugging frontal horn
Lentiform nucleus, putamen + globus pallidus
Third ventricle with thalamus on either side
Trigone or atrium of lateral ventricle
Splenium corpus callosum
Occipital lobe
Figure 185-4

Case 185 401
Frontal lobe WM in corona radiata
Body of lateral ventricle with coronal radiata

(star) lateral to it
Parieto occipital WM, forceps major
Figure 185-5
Frontal lobe
Cingulum
Centrum semiovale
Parietal lobe
Figure 185-6

402 Case 185
FINDINGS Images through different levels of the brain show- third, and fourth. The basal ganglia are composed of the cau-
ing the structures as marked. Figure 185-1. Midline T1WI sag- date nucleus, putamen, globus pallidus, and claustrum. The
ittal. Figure 185-2. Axial T2WI through the pons. Figure185-3. internal, external, and extreme capsules separate the struc-
Axial T2WI through the midbrtain. Figure185-4. Axial T2WI tures of the basal ganglia and thalami.
through the thalami and basal ganglia. Figure 185-5. Axial
T2WI through the corona radiate. Figure 185-6. Axial T2WI Question for Further Thought
through the centrum semiovale. 1. Are anatomical structures the same from individual to
individual?
DIAGNOSIS Normal MRI anatomy of the brain.
Appropriate localization is important when we see an abnor-
mality; hence, correct anatomical localization is important.
DISCUSSION The anatomy of the brain at imaging is
important and could be very challenging particularly for
beginners. It is important that pathology is properly located What the Treating Physician Wants to Know
to explain the clinical symptoms and signs. The various Where is the lesion?
MRI sequences show similar structures with different levels
of clarity. The T1WI, T2WI, and FLAIR in that order tend Answer
to show the anatomy better than other sequences. Midline 1. Most normal structures are the same with minor varia-
structures are demonstrated better on the sagittal images. We tions. Some of these variations could be due to congeni-
obtain specific planes to demonstrate specific structures such tal reasons such as they may particularly affect vascular
as coronal images for hippocampal structures. The major ana- structures, both arteries and veins, and the ventricles.
tomical landmarks have been demonstrated here. Vascular Rotation of images may render some structures more
structures and white matter (WM) tracts on DTI have been prominent and asymmetric. Disease processes more than
discussed elsewhere in this book. Some major structures are anything else render significant variations in size, contour,
highlighted here. There are three cerebellar peduncles; infe- and location of structures. Think of these reasons when
rior, middle, and superior. There are 4 ventricles; two lateral, structures appear significantly different from normal.

Case
186 CLINICAL HISTORY 35-year-old male with new onset seizures.
Figure 186-2
Figure 186-1
Figure 186-4
internal bony septations. The content is hypodense on brain

Figure 186-3 window (not shown). Figure 186-2. Axial T2WI through the
mass. The mass is homogeneously hyperintense except for
the septations and the rim which are hypointense (arrows).
The content follows cerebrospinal fluid (CSF) intensity on
all sequences including DWI and ADC maps. There is no
FINDINGS Figure 186-1. Axial cranial NCCT through significant abnormality or edema of the surrounding brain.
the middle cranial fossa bone window setting. There is a Effusion of the right mastoid air cells is present (not shown).
5 cm 4.5 cm thin rim expansile mass in the right temporal Figure 186-3. Sagittal pre-contrast T1WI through the mass. It
fossa (star) arising from the petrous bone. It has irregular is a well-defined hypointense extraaxial mass displacing the
403

404 Case 186
adjacent temporal lobe. There is a thin hypointense dark rim ABC of the temporal bone is uncommon with mostly sin-
(arrows). Figure 186-4. Coronal post-contrast T1WI through gle case reports. ABC is generally an expansile benign non-
the mass. There is a somewhat shaggy mural contrast enhance- neoplastic mass affecting flat bones and the vertebrae and the
ment. Internal septations show similar contrast enhancement metaphysis of long bones. It is composed of multiple blood
pattern (arrows). filled cavities, hence the fluid levels in most of these lesions.
It occurs usually within the first three decades of life with
DIFFERENTIAL DIAGNOSIS Giant cell tumor, aneurys- most of them occurring under the age of 20 years. The tumor
mal bone cyst (ABC), chordoma, fibrous dysplasia. was surgically removed and the histology showed secondary
ABC within underlying fibrous dysplasia.
DIAGNOSIS Aneurysmal bone cyst (ABC) within fibrous
dysplasia.
DISCUSSION The imaging pattern of petrous/temporal 1. How common is secondary ABC?
bone ABC is that of ABC elsewhere; a well-defined cystic
multiloculated expansile mass with thin wall and multiple Reporting Responsibilities
internal septations. It characteristically has a bubbly appear- Direct reporting is necessary in view of the size and loca-
ance produced by multiple locules and diverticula. The wall tion of the lesion. Effect on surrounding structures such as
is usually hypointense (dark) on MRI. It has been described middle and inner ear and the brain should be identified and
as having heterogeneous internal content and fluid levels due reported.
to blood degradation product. In our case, there is no sig-
nificant fluid level, and internal content is mainly hyperin- What the Treating Physician Needs to Know
tense on T2WI and hypointense on T1WI. The CT shows a
Location, size, and effect on surrounding structures
very thin bony rim and septa. Giant cell tumor can resemble
ABC, complete with fluid levels and loculations. Fluid lev-
els have also been described in fibrous dysplasia, malignant Answer
fibrous histiocytoma, bone cyst, chondrosarcoma, and osteo- 1. Most ABCs are primary but up to one-third are second-
sarcoma. Chordoma is usually a midline tumor rather more ary within underlying tumors such as chondroblastoma,
heterogeneous with solid components. fibrous dysplasia, osteosarcoma, and giant cell tumor.

Case
187 CLINICAL HISTORY 54-year-old male with AIDS on antiretrovirals
presenting with tremors, jerking movement of limbs, and dropping objects.
405

406 Case 187
FINDINGS Figure 187-1. Axial FLAIR through the tem- subcortical and not necessarily bilateral. PML may contrast
poral lobes. There is bilateral anterior temporal lobes white enhance with a T1WI cortical hyperintensity in immune
matter (WM) hyperintensity (arrows). Figure 187-2. Axial reconstitution inflammatory syndrome (IRIS). Chronic
T2WI through the basal ganglia. There is confluent bilat- small vessel ischemic changes are usually monotonous
eral symmetrical WM hyperintensity around the frontal and small WM changes which may be confluent in severe cases.
occipital horns extending from the ventricular walls to the Binswanger disease tends to be globally confluent and not
subcortical regions (arrows). The frontal WM hyperintensity necessarily frontal predominant.
extend across the genu and anterior body of the corpus callo- HIVE usually presents with a combination of cognitive
sum (vertical arrow). This T2WI captures the degree of brain deficit, movement disorders such as tremors, gait instabil-
volume loss which is mild in this case. Figures 187-3 and ity and weakness, depressive symptoms, and behavioral
187-4. Axial FLAIR through the corona radiata and centrum changes. HIVE is the result of direct HIV infection of the
semiovale, respectively. There is predominant symmetrical brain macrophages and/or microglial cells. On autopsy of
bilateral frontal lobes confluent WM hyperintensity extend- AIDS patients with HIVE, demyelination, microglial nod-
ing from ventricular wall to subcortical regions (transverse ules, multinucleated giant cells, and perivascular infiltration
arrows). The lesions are more subcortical, smudgy, and not are described. Diagnosis is based on neuropsychological
as confluent in the parietal and occipital WM with relative testing of suspected individuals and exclusion of alternate
sparing of the deep WM (vertical arrows). It is noted that conditions. Cerebrospinal fluid (CSF) studies are helpful
there is no mass effect, and the post-contrast images (not inthe diagnosis and excluding mimics such as cryptococco-
shown) do not show areas of contrast enhancement. sis, toxoplasmosis, PML, or syphilis. Highly active antiret-
roviral therapy (HAART) is the primary treatment of choice,
DIFFERENTIAL DIAGNOSIS Chronic small vessel isch- and its benefit is well documented.
emic changes, progressive multifocal leukoencephalopa-
thy (PML), HIV encephalopathy (HIVE), leukodystrophy, Question for Further Thought
Binswangers disease. 1. What are the risk factors for HIVE?
DIAGNOSIS HIVE.
Routine reporting is sufficient in this case. It should be clearly
DISCUSSION Imaging features of HIVE consist of
distinguished from opportunistic infections that are common
diffuse, confluent, or smudgy WM changes that are pre-
in patients with AIDS, and most of such lesions tend to con-
dominantly frontal with involvement of the genu of the
trast enhance in either the parenchyma or meninges.
corpus callosum but occur elsewhere in the corona radiata
and centrum semiovale. These lesions are hypointense on
T1WI and hyperintense on FLAIR and T2WI. They gen- What the Treating Physician Needs to Know
erally extend from ventricular walls to subcortical WM Pattern of WM lesions and degree of volume loss
in the frontal lobes and around the occipital horns but are Other associated abnormalities
predominantly subcortical elsewhere. They are more often Exclusion of opportunistic infections and neoplastic
symmetrical without mass effect or contrast enhancement. changes
Unilateral lesions have been reported. Basal ganglia calci-
fications are seen in adults but more common in children. Answer
There is varying degrees of brain volume loss affecting Risk factors for HIVE include older age at seroconversion,
both cortical and subcortical regions resulting in enlarge- female gender, duration of HIV infection, presence of a prior
ment of the ventricles and sulci. Caudate nucleus atrophy is AIDS-defining diagnosis, low CD4+, and low nadir CD4
common. MRS shows low N-acetyl aspartate (NAA) peak count. These risk factors suggest that severe and prolonged
with elevated myoinositol not only in the WM but also immunosuppression may have long-lasting effects on neuro-
in the GM. Presence of lactate peak has also been noted, psychiatric performance regardless of subsequent viral sup-
and this disappears following successful treatment of the pression. Other factors such as high plasma HIV-RNA load,
encephalopathy. CT shows corresponding non-contrast- anemia, low weight, presence of hepatitis C virus, and sub-
enhancing confluent hypodensity in similar locations as in stance abuse are associated with increase in the prevalence of
MRI along with global brain volume loss. PML is usually neurocognitive deficits and dementia.

Case
188 CLINICAL HISTORY 46-year-old female with superior sagittal sinus
thrombosis.
Figure 188-1
Figure 188-2
Figure 188-3
FINDINGS Figure 188-1. Sagittal contrast-enhanced MRV of

the head. There is occlusion of major portion and irregularities
of the superior sagittal sinus (SSS) consistent with SSS throm-
bosis (transverse arrows). There is extensive collateral venous
drainage in the frontal region (vertical arrows). Figure188-2.
Axial FLAIR through the centrum semiovale. There are multi-
focal right frontoparietal subcortical hemorrhages (transverse
arrows) with local mass effect. Right posterior frontal sulcal
hyperintensity suggests subarachnoid hemorrhage (SAH) or
leptomeningeal collateral (vertical arrow). Figure 188-3. Axial
post-contrast T1WI through the lateral ventricles. There are Figure 188-4
407

408 Case 188
Figure 188-6
classical ivy sign seen on FLAIR images or arterial collat-

erals seen on post-contrast T1WI in ischemic infarcts and
chronic arterial occlusions such as in moyamoya disease.
Leptomeningeal venous collaterals are equally important.
Leptomeningeal venous collaterals are seen in occlusive
venous diseases particularly venous sinus thrombosis and
Figure 188-5 dural arteriovenous malformations (DAVF). They can also
occur in Sturge-Weber syndrome (SWS) where there is deep
venous occlusion and venous collaterals. MRI and MRV
multiple contrast-enhancing leptomeningeal vessels (trans- offer the best noninvasive means of imaging these collat-
verse arrows) extending from the cortex through the white erals. T1WI and FLAIR images may show apparent sulcal
matter (WM) to the large right ependymal/thalamostriate effacement or hyperintensity. Post-contrast T1WI demon-
vein (vertical arrow). Figure 188-4. Axial post-contrast T1WI strates prominent cortical veins along with transmedullary
close up of the right peritrigonal region. There are multiple veins draining into the ependymal or deep venous sinuses.
temporo-occipital transmedullary veins from the cortex to the They could be confused with perivenular medullary inflam-
ependyma (vertical arrows). There is prominent diploic vas- mation described in some inflammatory diseases. In some
cular enhancement in the right temporal and parietal bones cases, focal changes may resemble DVA! SWI can also
(transverse arrows). Figure 188-5. Axial post-contrast T1WI demonstrate dilated cortical and deep medullary collateral
through the centrum semiovale 2months after the event. There veins as irregular multiple tubular signal voids. They may
are prominent right frontoparietal sulcal and medullary veins be associated with dural and intraosseous or diploic venous
(vertical arrows). There are also multiple right dural, diploic, channel enlargement as in this case. These collaterals form
and galeal vascular contrast enhancements (transverse arrows) an alternative route of venous drainage. DAVF may be com-
consistent with collateral veins. Figure188-6. Contrast- plicated by venous reflux and dilatation and collateraliza-
enhanced MRV about 2 months following initial ictus (patient tion of the cortical veins that may predispose the patient to
is on Coumadin). There is recanalization of the SSS (verti- intracranial hemorrhage. Hence parenchymal hemorrhage
cal arrows), but the collaterals anteriorly have become more is a complication of leptomeningeal venous collaterals and
prominent (transverse arrows). MRA of the head (not shown) engorgement.
continues to be normal. The aim of imaging DAVF is not only to identify the arte-
rial feeders and the site of the fistula but also to identify the
DIFFERENTIAL DIAGNOSIS N/A. pattern and direction of venous drainage of the fistula. The
findings may have implications on the strategy for the man-
DIAGNOSIS Leptomeningial venous collaterals due to agement of DAVF as interruption of these leptomeningeal
SSS thrombosis. collaterals could form one way of treating some selected
DAVF.
DISCUSSION This case is specifically presented to show-
case the leptomeningeal collateral veins. What comes to Question for Further Thought
mind when you discuss leptomeningeal collaterals is the 1. What are the other changes in SWS?

Case 188 409

Any of the conditions associated with leptomeningeal venous 1. SWS (a neurocutaneous syndrome) could present with
collateral formation requires direct reporting; they are lesions a mix of facial port-wine nevus, ipsilateral brain corti-
requiring prompt attention. Recommendations for MRV and cal calcifications, hemiparesis, seizures, vascular eye
DSA if necessary should be made. If the leptomeningeal cal- changes, and glaucoma. Imaging features may include
cifications expected in SWS are not evident on MRI, then CT leptomeningeal angiomatosis, cortical and pial calcifica-
should be recommended. tions, deep venous obstruction, and enlargement of the
choroid plexus.
Associated findings such as venous sinus thrombosis,
DAVF, or suspicion of SWS
Other parenchymal changes such as hemorrhages
Recommendations for further evaluation: MRV, CT, DSA

Case
189 CLINICAL HISTORY 40-year-old male involved in trauma.
410

Case 189 411
lipoma is usually intimately associated with the choroid

plexus. In our first patient the location is higher than that
of a normal choroid plexus but the associated calcifications
suggest an ectopic location of the choroid plexus, and it
enhanced in continuity with the rest of the choroid plexus.
Calcification may be present in the vicinity if a lipoma. Fat
or lipoma has a unique presentation on MRI. It is hyperin-
tense on FLAIR, T1WI, and T2WI. However, chemical shift
artifact can create a hypointense signal on T2WI. It is usually
isointense to gray matter (GM) with the fat saturation pulse
and usually without contrast enhancement. It is hypointense
on GRE. Teratoma and dermoid may contain fat along with
other tissues, but these are usually well-encapsulated masses
that may contain different kinds of tissues usually with a
heterogeneous density on CT and T1 hyperintensity with T2
heterogeneity at MRI. Melanosis is usually hyperintense on
T1WI and hypointense on T2WI. Air bubble is hypointense
on all MR sequences.
IVL is usually an incidentaloma and of no clinical sig-
nificance. Most IVLs are tiny to small, but a left lateral ven-
tricular lipoma measuring up to 3.5 cm has been implicated
Figure 189-5
as a cause of acute hydrocephalus leading to sudden death.
Intracranial lipomas have been regarded as malformations
rather than tumors. They arise as abnormal persistence of
meninx primitiva during development of the subarachnoid
FINDINGS Figure 189-1. Axial NCCT through the superior
space; hence, most of these lipomas are located in the sub-
lateral ventricles. There is a small fat hypodensity surrounded
arachnoid space. Since most of these are incidental and
by calcification superiorly in the left lateral ventricle. The
asymptomatic, there is usually no need for treatment.
fat measures 62 HU compared with 1,018 HU in the air
containing sphenoid sinus. Figures 189-2 and 189-3. Axial
FLAIR and T1WI, respectively, on follow-up MRI demon-
strate a small round hyperintensity in the location of the fat 1. Can intracranial lipoma be symptomatic?
(arrow). Figure 189-4. Axial GRE through same level. There
is a focal hypointensity in the location of the fat (arrow). Reporting Responsibilities
Figure 189-5. Coronal NCCT through the third ventricle in IVL is a benign nontumoral entity and requires only routine
another patient. There is an ovoid homogeneous fat hypoden- reporting. Location and size as well as differentiating them
sity within the third ventricle (arrow) measuring 125 HU. from their mimics are very important.
DIFFERENTIAL DIAGNOSIS Dermoid, lipoma, teratoma, What the Treating Physician Needs to Know
air bubble, melanosis. IVL is a benign entity requiring no treatment since they are
asymptomatic
DIAGNOSIS Intraventricular lipoma (IVL).
Answer
DISCUSSION IVL is reported as a rare entity found in all 1. Intracranial lipoma is generally not symptomatic by
the ventricles but more commonly in the lateral ventricles. itself but the associated congenital anomalies with which
The hallmark of IVL on CT is the extreme low attenuation intracranial lipomas are found may provoke symptoms.
resembling air bubble from which it must be differentiated Most of these anomalies consist of midline or sylvian
particularly in patients with history of trauma. Lipomas usu- fissure malformations, dysplasias, dysgenesis or agen-
ally measure less than 100 HU while air measures in the esis of the tissues and hence can result in developmental
region of 1,000 HU. This always helps in differentiating a delays, psychomotor retardation, cranial nerve defects,
lipoma from pneumocephalus. Within the lateral ventricle, and seizures.

Case
190 CLINICAL HISTORY 28-year-old female with gait abnormality and vision
loss in right eye.
FINDINGS Figure 190-1. Sagittal MRI T1WI. There is a DIFFERENTIAL DIAGNOSISJoubert syndrome (JS),
high-riding fourth ventricle (star) at the junction of the mid- cerebellar vermian atrophy or hypoplasia, pontocerebellar
brain and the pons with obvious cerebellar vermis volume atrophy.
loss (arrow). The superior fourth ventricular velum is almost
horizontal. Figure 190-2. Axial T2WI through the superior DIAGNOSIS JS and Joubert syndrome-related disorder
cerebellar peduncle demonstrates the molar tooth sign or (JSRD).
malformation (MTS or MTM) (arrows). Figure 190-3. Axial
T2WI inferior to the MTS. There is batwing appearance of DISCUSSION The hallmark of the diagnosis of JS or
the inferior fourth ventricle (arrows). Figure 190-4. DTI JSRD at imaging is the MTS or MTM. This is represented by
color directional map through MTS. There is thickening and large or thickened superior cerebellar peduncles projecting
horizontal disposition of the superior cerebellar peduncles horizontally behind the pons/midbrain with deepening of the
(arrows). interpeduncular fossa resulting in a molar tooth configuration
412

Case 190 413
on CT or MRI as in Figure 190-2. The other findings include there is no treatment. These patients are cognitively impaired
a high-riding fourth ventricle at the junction of the pons and and have problem coping.
midbrain. The velum of the fourth ventricle is almost hori-
zontally directed. The nodulus and dentate nucleus could Question for Further Thought
be heterotopic. There is a batwing or triangular appearance 1. Is the MTS pathognomonic for JS?
of the inferior fourth ventricle on the axial images as in
Figure 190-3. The dentate nucleus has been shown to be lat- Reporting Responsibilities
erally displaced. Interpeduncular heterotopia and hamartoma This is not an acute disorder, and as such routine reporting is
of the tuber cinerium have been reported in association with sufficient. Recognizing the MTS is important in placing the
JSRD. DTI has been useful in demonstrating the underly- patient in a definitive disease category that should lead to the
ing pathogenesis of the structural imaging findings. These necessary genetic testing to confirm which of the five muta-
include horizontally directed superior cerebellar peduncles tions may be responsible for the disease.
with lack of decussation of the fibers, lack of decussation of
the corticospinal tracts in the medulla, and laterally located
deep cerebellar nuclei. Other abnormalities reported in this
disorder include occipital cephaloceles, Dandy-Walker mal- Is there a definite MTS? This would exclude other differ-
formation, corpus callosal changes, and white matter (WM) ential diagnoses
T2 hyperintensities. MTS is absent in the other differential Are there other central nervous system (CNS) abnormali-
diagnoses. ties outside the posterior fossa? These may suggest the
JS is a rare complex malformation of the hind/midbrain degree of severity
inherited in an autosomal recessive mode with an estimated How can the other systemic changes be evaluated? US and/
prevalence of 1 in 100,000. There are at least five identified or CT may be useful for evaluation of the renal, hepatic,
chromosomal abnormalities associated with the JS spectrum and skeletal abnormalities and neuroophthalmologic eval-
responsible for about 50% of known JS population. The uation for the ocular changes
clinical presentations include irregular breathing in infancy,
ocular apraxia, hypotonia, cognitive impairment, and ataxia. Answer
Imaging plays an important role in the diagnosis, and the 1. MTS is found throughout the spectrum of JS disorders
MTS is the characteristic finding on axial CT or MRI. The known as JSRDs. JSRDs are classified into six phenotypic
underlying pathology is that of vermis aplasia or hypoplasia subgroups: pure JS, JS with ocular defect, JS with renal
with thickening of the horizontally directed superior cerebel- defect, JS with oculorenal defects, JS with hepatic defect,
lar peduncles associated with lack of decussation of its fibers. and JS with orofaciodigital defects. With the exception of
The corticospinal tracts in the caudal medulla also show lack rare X-linked recessive cases, JSRDs follow autosomal
of decussation. Other systemic changes include endocrine recessive inheritance and are genetically heterogeneous.
abnormalities, renal disease, ocular colobomas and retinal Mutations in at least five genes (TMEM216, CEP290,
changes, occipital cephalocele, hepatic fibrosis, polydactyly, TMEM67, RPGRIP1L, and CC2D2A) are known to be
and oral hamartomas. The prognosis is generally poor as responsible for the anomalies in this spectrum.

Case
191 CLINICAL HISTORY 64-year-old female with transient left leg
weakness and shaking.
414

Case 191 415
FINDINGS Figure 191-1. Axial NCCT through the basal in favor of amyloid angiopathy. Associated leptomeningeal
ganglia. There is mild prominence of the lateral ventricles enhancement may suggest vasculitis in which case acute and
and sulci compatible with mild global brain volume loss. chronic lesions may coexist.
Multifocal bilateral almost symmetrical periventricular LA is a frequent finding on CT or MRI of the elderly
and subcortical white matter (WM) smudgy hypodensities above 65 with age range from 48 to 97. LA has no significant
(arrows) are present mainly in the frontal lobes. Figure191-2. gender preference. Several studies have linked LA to sys-
Axial NCCT through the lateral ventricles. There are multifo- temic hypertension, advanced age, lacunar infarcts, micro-
cal poorly marginated hypodensities in bilateral deep and sub- hemorrhages, and cerebral atrophy. Yet others have declared
cortical WM (arrows point to some of them). Figures191-3 LA a feature of the small vessel cerebrovascular patholo-
and 191-4. Axial FLAIR MRI through the corona radiata and gies that lead to stroke, including hypertensive arteriopathy,
centrum semiovale, respectively. There are multiple mostly cerebral amyloid angiopathy, and CADASIL and has been
punctate to small bilateral discreet WM hyperintensities found in healthy elderly subjects, in cognitive dysfunction
mostly in the deep and subcortical regions (transverse arrows) in the nondemented elderly patient, in Binswanger disease,
with some smudgy hyperintensities around the occipital and Alzheimer disease, vascular dementia, and stroke. It is usu-
frontal horns (vertical arrows). ally not a disease but an indication of a process that should
be unearthed and treated if possible.
DIFFERENTIAL DIAGNOSIS Demyelinating lesions,
vasculitis, chronic small vessel ischemic changes, leukoara- Question for Further Thought
iosis, and migraine. 1. What is the pathogenesis of LA?
DIAGNOSIS Multifocal WM chronic small vessel isch- Reporting Responsibilities

emic changesleukoaraiosis. Routine reporting is sufficient. The distribution of lesions
could help in directing further evaluation of the basic
DISCUSSION Multifocal WM changes in the elderly are problems.
usually reported as non-specific in most radiology reports;
yet the location and pattern could be useful in directing
further evaluation of these patients. Typical leukoaraiosis
lesions are small, usually round, but shape could vary, deep, Pattern of distribution of lesions
and subcortical multifocal hypodensities within the corona Presence or otherwise of other lesions such as brainstem
radiata and centrum semiovale on NCCT. The outline is hyperintensities, ischemic infarcts, or hemorrhage
often irregular and not sharp. When they are periventricular, It is not a disease but a pointer to an underlying problem
they are found as smudgy hypodensities around the frontal Further evaluation by CTA, MRA, and perfusion studies
and occipital horns of the lateral ventricles. The corpus cal- (CT, MR, or SPECT) may be useful
losum is not usually involved, and they show no mass effect.
The MRI shows corresponding lesions as hyperintense on Answer
FLAIR and T2WI. They could be monotonous rarely varying 1. The pathogenesis is controversial and probably multifac-
in size except when they are confluent, extending from ven- torial. The pathologic basis of LA is somewhat protean
tricular wall to subcortical U fibers. There is usually associ- including demyelination, gliosis, necrosis, and cavitation
ated volume loss. They do not contrast enhance. There are considered due to atherosclerosis of small or large vessels.
other lesions that could mimic leukoaraiosis. Location is key Similar pathologic features are found in arteriosclerotic
to the underlying pathology of some of these rather nonspe- vascular encephalopathy or Binswanger disease. It is not
cific lesions. They could become somewhat specific when unusual for watershed infarcts of the WM to subsequently
they involve certain locations. Periventricular lesions in the present as WM hyperintensities on follow-up MRI. It is
corona radiata in the young could suggest demyelinating pro- increasingly likely that LA may be caused by not only
cess particularly when they are ovoid or flame shaped with poor perfusion of the WM due to hyalinized thickened
variable sizes and involve the corpus callosum. Involvement and stenosed small vessels as originally thought but also
of the external capsules and anterior temporal lobes particu- possibly venous outflow impairment and microinfarc-
larly in the younger patient in the 40s may suggest cerebral tions as a result of occlusion of these same small arteries.
autosomal dominant arteriopathy with subcortical infarcts Embolic phenomenon as a cause of these occlusions has
and leukoencephalopathy (CADASIL). Additional lesions also been proposed. Presence of LA may be a major pre-
in the brainstem may point in the direction of hypertensive dictor of future ischemic strokes in both large and small
arteriopathy while lack of lesions in the brainstem may be vessel strokes.

Case
192 CLINICAL HISTORY 40-year-old female with no past medical history
presenting with headaches.
FINDINGS Figure 192-1. Axial post-contrast T1WI through mass (m) extending from the region of the right Meckel
the inferior posterior fossa. There are multifocal meningeal cave posterolaterally along the right tentorial edge (arrow).
enhancements bilaterally along the cerebellum and brainstem Figure 192-3. Coronal post-contrast T1WI through the mass.
with irregular enhancement in the right near the Meckel cave The mass (m) extends from the Meckel cave to the cerebel-
(arrow). Figure 192-2. Axial post-contrast T1WI through lopontine angle (CPA), as well as scattered leptomeningeal
the pons. There is a lobulated, enhancing dural-based enhancing foci predominantly around the brainstem and upper
416

Case 192 417
cervical cord. Figure 192-4. Post-contrast coronal T1WI Reporting Responsibilities

through the pituitary fossa. There is enhancement and As with most intracranial lesions, location and acuity of
thickening of the infundibulum and undersurface of the optic the lesion(s) and potential complications generally dictate
chiasm (arrows) in addition to the mass (m) extension into the urgency of relaying findings. Dural-based neurosarcoid
the right cavernous sinus. lesions may obstruct the arachnoid granulation or ventricular
outlets causing hydrocephalus. Other lesions may cause sig-
DIFFERENTIAL DIAGNOSIS Neurosarcoidosis, lymphoma, nificant mass effect on vital structures warranting urgent com-
leptomeningeal carcinomatosis, metastatic disease, and infec- munication of findings. While in many cases routine reporting
tious etiologies. is sufficient, a phone call to the referring clinician with an
unexpected finding is usually helpful and appreciated.
DIAGNOSIS Neurosarcoidosis.
DISCUSSION Sarcoidosis is a multisystem inflamma- Diagnosis of neurosarcoidosis is based on documentation
tory disease characterized by noncaseating granulomas. of systemic sarcoidosis in the absence of other neurologic
Neurosarcoidosis has been reported in up to 5% of patients disease
with sarcoidosis (with higher percentages seen in postmor- Neurologic manifestations are frequently the presenting
tem series). Imaging findings typically consist of pachy- symptoms but may be nonspecific
meningeal/dural masses, leptomeningeal involvement, Neurosarcoidosis is usually included in the differential
enhancing brain parenchymal lesions, and cranial nerves diagnosis when an abnormality involving the leptomenin-
thickening and enhancement. Approximately 1/3 to 1/2 of ges and/or dura is detected particularly in the setting of
the lesions tend to involve the dura. Approximately 1/3 of systemic sarcoidosis
the lesions tend to involve the leptomeninges, with the cra- Imaging is important in the clinical evaluation of sarcoid-
nial nerves, optic nerves, and brain parenchyma (hypothala- osis for both diagnosis and follow-up to assess response to
mus/infundibulum) the next most common location. The treatment and to guide the next best course of action
remaining occur in the spine, most commonly dural-based. Early diagnosis of neurosarcoidosis is important because
The dural-based and parenchymal lesions of neurosarcoid of its associated high morbidity and mortality. Recognition
typically enhance homogeneously on post-contrast T1WI of the constellation of findings of neurosarcoidosis in asso-
and have a predilection for involvement of the skull base. ciation with systemic sarcoidosis may help to avoid addi-
Contrast-enhanced MRI including multiplanar sequences tional invasive testing and biopsy
and fat saturation is the best imaging tool for diagnosis of
neurosarcoidosis. Answer
1. Look for a recent prior chest X-ray or chest CT if one has
Question for Further Thought been performed. Chest abnormalities, predominantly hilar
1. What other imaging would help in confirming your suspi- adenopathy, can be seen in greater than 90% of patients
cion of neurosarcoidosis? with systemic sarcoidosis and neurosarcoidosis.

Case
193 CLINICAL HISTORY Child with acute onset of influenza-like symptoms
and encephalopathy.
FINDINGS Figure 193-1. Axial FLAIR image through the Lesions are usually hyperintense on T2-weighted and FLAIR
thalami. There are asymmetric hyperintensities of both len- images and may have restricted diffusion without mass effect.
tiform nuclei and thalami (arrows). Also note the high sig- Contrast enhancement or sulcal hyperintensities on FLAIR
nal in posterior subarachnoid spaces. Figure 193-2. Axial reflect meningeal involvement. Patients who present with
FLAIR image cephalad to Figure 193-1. There is bilateral flaccid paralysis show spinal cord lesions. Often the signal
caudate nuclei hyperintensities (arrows). There is minimal abnormalities in basal ganglia and thalami are bilateral and
white matter (WM) changes posterior to the occipital horns. symmetric. MS rarely affects gray matter (GM). CJD often
show cortical ribbon DWI hyperintensity. West Nile could
DIFFERENTIAL DIAGNOSIS Japanese encephalitis, mimic any of the other differentials.
West Nile virus encephalitis, Creutzfeldt-Jakob disease West Nile virus is a flavivirus and a member of the
(CJD), bacterial meningitis, acute poliomyelitis, Guillain- Japanese encephalitis virus serocomplex transmitted to
Barr syndrome, and multiple sclerosis (MS). humans by mosquitoes. It is endemic in Africa and Middle
East, and large outbreaks have occurred in several countries
DIAGNOSIS West Nile virus encephalitis. (it is increasingly encountered in the United States). The
central nervous system may be affected through possible
DISCUSSION CT is invariably normal. MRI is abnormal hematogenous spread, manifesting as aseptic meningitis,
in 50% of patients with involvement of the basal ganglia, encephalitis, acute flaccid paralysis, or Guillain-Barr syn-
thalami, mesial temporal lobe, midbrain, pons, and cerebel- drome although this happens only in less than 20% of infected
lum, and some articles also show white matter abnormalities. patients. The majority of infections are asymptomatic, and
418

Case 193 419
20% to 30% of patients present with mild fever. A definite What the Treating Physician Needs to Know
diagnosis is made showing West Nile virus-specific anti- The pattern of abnormalities found on MRI may help make
bodies in CSF. Treatment is symptomatic and supportive. the correct diagnosis and predict prognosis
A normal MRI does not exclude the diagnosis of West
Question for Further Thought Nile encephalitis. A normal MRI is well correlated with
1. What is the prognosis of West Nile virus encephalitis? good outcome

Direct reporting is necessary in this acute infection. 1. Patients with normal brain MRI or signal abnormalities
Description of the extent of the lesions and pattern of only seen on DWI (that usually revert) have a better out-
enhancement which are suspicious for the diagnosis of West come. Patients with MR abnormalities in other sequences
Nile virus encephalitis is important. have a high risk of mortality.

Case
194 CLINICAL HISTORY 22-year-old female with possible stroke. There is a
history of connective tissue disease of unknown type.
FINDINGS Figures 194-1 and 194-2. 3D volume ren- arrows) with small out-pouches of the petrous ICAs -
dering with corresponding MIP, respectively at differ- pseudo aneurysms (line arrows). There is also twisted
ent rotations, of contrast-enhanced MRA of the neck. wire appearance of the branches of the bilateral middle
There is fusiform dilatation of the aortic arch extending cerebral arteries (MCA) and anterior cerebral arteries
into the origins of all the great vessels, and bilaterally (ACA) (vertical arrows). Figure 194-4. Axial T2 pro-
into the common carotid arteries and proximal internal ton density (PD) fat sat through the skull base. There
carotid arteries (ICAs). Figure 194-3. MIP of 3D TOF is crescentic mural hyperintensity surrounding the flow
non-contrast MRA of the head. There is fusiform dilata- void in the bilateral ICAs (arrows). Figure 194-5. Axial
tion of bilateral petrous ICAs and right A1 (transverse DWI through the cingulate gyrus. There is right frontal
420

Case 194 421
the form of ischemic changes or infarcts are demonstrated

as signal abnormalities on the MRI; in this case, a right dis-
tal ACA territory infarct. There were also multifocal white
matter (WM) changes consistent with multifocal small
infarcts or ischemic changes. CTA and DSA are also use-
ful in demonstrating various aspects of the abnormalities.
These changes could be found in the listed differentials.
The clinical information will serve as a guide to the correct
diagnosis.
Fusiform or cylindrical aneurysms intracranially or extra-
cranially are rare. Intracranially, they are more common in
the posterior circulation. The causes may include arterial dis-
section, atherosclerosis, and disorders of collagen and elastin
metabolism, infections, and very rarely neoplastic invasion
of the arterial wall. The underlying pathology is usually
internal elastic lamina degeneration and fissuring with myx-
oid changes or altered blood within the wall of the vessel
depending on the cause. Serpentine channel forms as disease
extends longitudinally, combined with varying degrees of
intramural thrombosis. The cervical vessels in this patient
did not demonstrate mural thrombosis. Multifocal arterial
aneurysms in different parts of the body were present in
this patient. The underlying diagnosis was connective tissue
disease of unknown type. Known connective tissue or col-
Figure 194-5
lagen disease responsible for fusiform aneurysms includes
lupus, Marfan syndrome, Ehlers-Danlos, and unknown fac-
parasagittal restricted diffusion in the right ACA terri- tors. Vascular involvement in NF1 could produce similar
tory consistent with acute infarct (arrow). This was due findings. Rupture could result in subarachnoid and or paren-
to distal occlusion of right A2 (not shown). chymal hemorrhage. Mass effect on critical structures could
produce focal neurologic deficit.
DIFFERENTIAL DIAGNOSIS Fusiform aneurysm due to
vasculopathy, vasculitis, Marfan syndrome, Ehlers-Danlos, Question for Further Thought
neurofibromatosis type 1 (NF1), arterial dissection. 1. What are the treatment options?
DIAGNOSIS Cylindrical and fusiform aneurysm due to Reporting Responsibilities

vasculopathy of unknown origin. Direct reporting is necessary in view of possible complication
of rupture and the presence of acute ischemic changes. The
DISCUSSION Cylindrical or fusiform aneurysm is a location, extent, and associated parenchymal abnormalities
circumferential arterial dilatation resulting from patho- should be tabulated. Presence of underlying pathology if
logic involvement of the entire artery; if a long segment known should also be reported.
of the artery is involved it is termed cylindrical, and if a
short segment is involved it is termed fusiform. MRI with What the Treating Physician Needs to Know
MRA offers the best method of showing the changes of Location, extent, and number of lesions
fusiform or cylindrical aneurysm detailing the pattern of Associated intracranial changes (infarcts, parenchy-
dilatation, presence or absence of mural thrombus, associ- mal or subarachnoid hemorrhage, and mass effects if
ated brain parenchymal changes, and other related bone or present)
dural changes. There is usually elongation and dilatation of Underlying cause may not be determinable at imaging
the vessels involved, best demonstrated by MRA or CTA.
Axial PD fat sat may show presence of hyperintense mural Answer
thrombus or hemorrhage. Associated pseudoaneurysm may 1. Multifocal extensive changes as in this case could be difficult
present as an outpouch. Distal vascular irregularities such to manage. Conservative management is recommended with
as the twisted wire appearance in this case indicate distal only symptomatic or critical lesions treated. Focal lesions
vasculopathic changes. Distal occlusive changes can also could be wrapped, clipped, coiled, stented, or resected as
be demonstrated. The consequences of the vasculopathy in judged appropriate with or without bypass options.

Case
195 CLINICAL HISTORY Elderly female found unconscious at home.
Figure 195-4
FINDINGS Figure 195-1. Axial DWI through the upper

pons. There are bilateral large almost symmetrical tem-
porooccipital hyperintensities (stars). Left basis pontis,
right tegmentum, and bilateral superior vermis/cerebel-
Figure 195-3 lum demonstrate confluent hyperintensities. The ADC map
422

Case 195 423
(not shown) demonstrates corresponding hypointensities mesiotemporal lobe involvement may lead to impaired mem-
consistent with restricted diffusion and acute infarcts. ory and abulia. Visual field and function defects are due to
Figure195-2. Axial DWI through the midbrain. There are occipital lobes involvement. Agitated delirium at onset can
similar bilateral symmetrical temporo-occipital and conflu- occur with dominant occipitotemporal infarcts. The fatality
ent midbrain (star) hyperintensities consistent with acute rate in basilar artery occlusion is generally very high, 40%
infarcts. Figure195-3. Axial DWI through the thalami. to 86%. Basilar artery occlusion lends itself to intraarterial
There are symmetrical bilateral thalamic (stars) and occipi- thrombolysis which is said to improve the outcome reducing
tal acute infarcts. Figure 195-4. Normal CTA MIP of top the fatality to below 39%.
of the basilar major branches in a normal companion case.
Basilar summit (chevron), posterior cerebral artery (PCA) Question for Further Thought
(vertical arrows), superior cerebellar artery (SCA), dupli- 1. How does the vascular anatomy explain the clinical pre-
cated in this case (transverse arrows). sentation of top of the basilar syndrome?

Direct reporting is essential in acute infarcts. The extent of
DIAGNOSIS Top of the basilar syndrome. the distribution of infarcts and the presence of significant
mass effect and hemorrhage should be reported. CTA and
DISCUSSION CT and MRI are equally effective in demon- MRA are useful for evaluating the vessels.
strating the changes of top of the basilar syndrome. MRI par-
ticularly DWI demonstrates the acuity and arterial territories What the Treating Physician Needs to Know
of lesions better than CT. The basis of the imaging changes Extent and location of the infarctions
is occlusion of the rostral portion of the basilar artery and Vascular findings if MRA or CTA has been obtained
in effect all the branches emanating from the basilar artery Presence of cerebrospinal fluid (CSF) pathway obstruction
summit which include bilateral PCA, bilateral SCA, bilateral
thalamoperforators, peduncular branches, and depending on Answer
the extent of the inferior extension of the occlusion, pon- 1. The basilar artery summit branches as demonstrated in
tine branches of the basilar artery. Imaging reveals infarcts figure 195-4 serve many anatomical regions that help
involving the bilateral temporal lobes posteriorly and medi- to explain the symptomatology of this syndrome. The
ally, bilateral occipital lobes, bilateral superior cerebellum PCA in its P1 segment gives origins to several vessels.
and superior vermis, pons, midbrain, and bilateral thalami. A The thalamoperforators supply the medial thalamus
hyperdense basilar artery may be visible on CT. The MRA (memoryamnesia and confabulatory symptoms): The
or CTA may show occlusion of the basilar summit and its peduncular branches which supply the median and para-
rostral branches but recanalization is common after occlu- median midbrain such as the reticular formation (dis-
sion being present in up to 62%. There may be a variation turbance of consciousness), CN III nucleus (extraocular
in the imaging presentation of this syndrome depending on muscle movement disorders and pupillary changes), and
the pattern of collateral circulation or anatomical variation at the cerebral peduncles (corticospinal tractsmotor path-
the basilar artery summit particularly variations of the pos- waysweakness). The P2 segment gives origins to the
terior communicating arteries and PCAs. Top of the basilar thalamogeniculate arteries supplying the lateral thala-
syndrome could present as bilateral paramedian midbrain mus (sensory), the posterior choroidal artery supplying
infarcts, or there could be asymmetric presentation of the the geniculate bodies (hearing and visual), the pulvinar
infarctions in the PCA territories depending on the origins and dorsolateral thalamus (integration of visual, motor,
of the PCAs. and limbic), and the posterior limb of internal capsule
Top of the basilar syndrome is a manifestation of occlu- (motorweakness or paresis of upper limb). Distal
sion of the rostral basilar artery. There may be a clini- branches of the PCA supply the occipital lobes (visual)
cal prodromal stage with transient ischemic attacks due to and the medial temporal lobe (memory). The SCAs
thrombosis, but acute cardioembolic event may not present supply the cerebellum (motor coordination and equilib-
with significant warning signs. Clinical presentation often riumataxia). Perforating branches of the upper basilar
includes disorders of consciousness, hypersomnolence, artery supply the pons (reticular activating system and
changes involving the oculomotor and pupillomotor func- cranial nerves nucleiunconsciousness, dysarthria, and
tions with fixed and dilated pupils if the third nerve nucleus craniopathies). Hypothalamic supply disruption via the
is affected, behavioral changes, ataxia, and psychiatric posterior communicating artery or the thalamoperforators
symptoms such as vivid hallucinations. Thalamic and/or may lead to behavioral changes and endocrinopathies.

Case
196 CLINICAL HISTORY 4-year-old female with lethargy.
424

Case 196 425
as WHO II fibrillary astrocytoma which could transform into

WHO III or IV. The MRI findings are those of infiltrating
expanding brainstem mass that is diffusely hyperintense on
T2WI with poorly defined boundaries. It is hypointense on
T1WI and does not contrast enhance. Presence of hemor-
rhage, calcification, and necrosis may herald transformation
to a higher grade. This tumor was radiated. It is not certain
whether the contrast enhancement is related to radiation
effect or progression to a higher grade. The FLAIR gener-
ally shows a hyperintense mass. It does not restrict diffusion
consistent with hypocellularity and edema. The tumor usu-
ally encases the basilar artery and could produce exophytic
components. Leptomeningeal enhancement may indicate
CSF seeding.
The second type is more focal and less infiltrating and
usually indolent for many years. This includes tectal glioma
confined to the tectum; tegmental glioma confined to the cen-
tral brainstem and the dorsal exophytic glioma all of which
are WHO I or pilocytic astrocytoma. These tumors are all
hyperintense on FLAIR and T2WI and hypointense on T1WI
Figure 196-5
and do not enhance with contrast.
All the differentials are legitimate and somewhat treat-
able and should be excluded. Osmotic myelinolysis mainly
FINDINGS Figure 196-1. Axial NCCT through the pons. affects the central portion of the pons and spares the periph-
There is expanded hypodense pons obliterating all the sur- ery. Extrapontine components could be present. It should be
rounding cerebrospinal fluid (CSF) spaces including the considered in the appropriate clinical setting. Acute or sub-
fourth ventricle and wrapping around the basilar artery acute demyelination may contrast enhance and may affect
(arrow). Figure 196-2. Axial FLAIR through the pons. There other parts of the brain particularly the brachium pontis.
is expansion of the pons with striated hypointense central por- Brainstem encephalitis could be focal or diffuse. It has been
tion and surrounding hyperintense periphery. There is a well- suggested that MR spectroscopy could be useful in differen-
circumscribed hypointense component anteriorly and to the tiating DIBSGs from the mimics. BSG accounts for 10% to
left of the basilar artery (arrow). There is effacement of the 20% of all central nervous system (CNS) tumors in children.
cerebellopontine angles (CPAs) and the prepontine cistern Clinical presentation includes headache, nausea and vom-
with compression of the fourth ventricle (stars). Figure 196-3. iting, lethargy, long tract signs, and cranial nerve palsies.
Axial T2WI through the pons. The mass is homogeneously Radiation is the standard of care. However, this tumor is not
hyperintense encasing the basilar artery (arrow). Figure 196- amenable to treatment, and the prognosis is very poor; the
4. Post-contrast sagittal T1WI. There are two components to worst prognosis of all childhood brain tumor.
the non-contrast-enhancing hypointense mass, a posterior and
an anterior component separated by an isointense S- shaped Question for Further Thought
tissue (arrow). Figure 196-5. Coronal post-contrast T1WI 1 1. BSG is known to be a tumor of children, does it occur in
year following the other images. She now has progressive long adults?
tract signs and cranial nerves palsy. There is irregular contrast
enhancement centrally with peripheral thick linear enhance- Reporting Responsibilities
ment along with increase in the size of the mass. Direct reporting is warranted. This is a neoplasm, and there is
CSF space compression and obstruction. Presence of hydro-
DIFFERENTIAL DIAGNOSISOsmotic demyelination, cephalus makes direct reporting more urgent.
brainstem encephalitis, demyelination, brainstem glioma.
DIAGNOSIS Brainstem glioma (BSG), diffuse intrinsic Location and size with areas of involvement
brainstem glioma (DIBSG). Complications such as hydrocephalus
Are there less fulminant differentials to consider?
DISCUSSION BSG is mostly a tumor of children. There
are basically two types. The DIBSG (60% to 80% of all Answer
BSG) which this one represents is a diffuse infiltrating brain- 1. Yes, BSG occurs in adults but is less common (1.5% to
stem tumor extending up and down the brainstem. It is gener- 2.5% of all intracranial tumors in adults) with a rather
ally hypodense on CT and does not contrast enhance unless benign clinical behavior. The diffuse intrinsic form has a
it has transformed to a higher grade tumor. It begins usually better prognosis in adults than in children.

Case
197 CLINICAL HISTORY 62-year-old male presenting with memory
problems, depression, and daytime sleepiness.
Figure 197-4
Figure 197-3
426

Case 197 427
FINDINGS Figures 197-1. Top row. Axial F-18 FDG-PET dementia (SD) and progressive nonfluent aphasia (PNFA).
images through the frontotemporal lobes. There is asymmet- 25% to 40% of FTD is familial. It is more common in the
ric mild to moderate decrease in FDG uptake in the frontal age range of 45 to 64 years. Usually there is lack of inhibi-
lobes (right greater than left) (arrows) Figure 197-2. The tion in the frontal lobes; thus, FTD patients tend to struggle
fused PET/CT and Figure 197-3 NCCT, show mild to mod- with binge eating and compulsive behaviors. There is clini-
erate bilateral frontal volume loss (arrows). Figures 197-4 cal overlap between FTD, corticobasal degeneration, and
and 197-5. Right parasagittal F-18 FDG-PET and fused progressive supranuclear palsy.
PET/CT, respectively. There is decrease in FDG uptake in Pathologically a very heterogeneous group of genes are
the right lateral temporal lobe (arrows) with normal FDG involved. Tau protein (hyperphosphorylated microtubular
uptake in the parietal and occipital lobes. Figure 197-6. protein) is the major constituents of the cellular inclusions
Right parasagittal NCCT. There is enlarged frontal horn responsible for the disease.
(star) consistent with volume loss.
DIFFERENTIAL DIAGNOSISAlzheimer disease (AD), 1. Does the asymmetric frontal or temporal FDG hypome-
Lewy body dementia, corticobasal degeneration, frontotem- tabolism or volume loss exclude FTD?
poral dementia, progressive supranuclear palsy.
DIAGNOSIS Frontotemporal dementia (FTD). Routine reporting is sufficient as the diagnosis is clinical
unless there is an acute finding that exclude the disease.
DISCUSSION FTD, formerly known as Pick disease, is a Intracranial masses and acute territorial infarct should be
neurodegenerative disorder with behavioral and language ruled out. The degree of frontotemporal volume loss should
deficits preceding memory deficits. Frontal lobes are usually be addressed specifically in the longitudinal follow-up.
affected before the temporal lobes. FDG-PET is the most use-
ful imaging modality in the diagnosis of FTD, demonstrating What the Treating Physician Needs to Know
decreased uptake in frontal and temporal cortices. FDG-PET
Any intracranial coexisting pathologies such as mass,
helps to differentiate it from other dementing syndromes such
infection, or acute infract should be excluded
as AD or Lewy body dementia. The role of structural imaging
Advanced AD can demonstrate FDG hypometabolism and
is supportive to principally exclude any intracranial mass or
volume loss in the frontotemporal cortices
acute infarcts. Volumetric brain MRI studies show the preced-
ing frontal lobe volume loss compared with normal controls
and also the other lobes of the same individuals cerebrum. Answer
Three clinical syndromes of FTD are described: behav- 1. The asymmetric distribution of frontal and temporal FDG
ioral variant FTD and two language variants, semantic hypometabolism or volume loss does not exclude FTD.

Case
198 CLINICAL HISTORY 49-year-old female with a history of 20 years of
medically resistant complex partial seizures and auras.
FINDINGS Figure 198-1. Coronal FLAIR through the hip- The underlying pathology is neuronal loss and chronic
pocampus. There is asymmetry of the hippocampus. The fibrillary gliosis usually restricted to CA1 region of hippo-
right hippocampus is smaller than the left and hyperintense campus, the hippocampal pyramidal cell layer just next to
(arrow). Figure 198-2. Coronal T1WI through the hippocam- the temporal horn.
pus. Subtle decreased volume with flattened surface of the However, the spectrum is dispersed between initial injury
right hippocampus (arrow). to the hippocampus where there is no architectural change
and the final neuronal loss with gliosis. Hence, up to 30% of
DIFFERENTIAL DIAGNOSISStatus epilepticus, mesial the focal epileptogenic cases may not be shown on MRI until
temporal sclerosis, choroidal fissure cyst, low-grade primary the late gliosis stage. On the contrary, the pathology may
glioma, dysembryoplastic neuroepithelial tumor (DNET), be bilateral and totally unsuspected. At this point, although
posterior cerebral artery (PCA), or anterior choroideal artery controversial, the earliest finding is the signal alteration on
(AChoA) infarct, hippocampal malrotation. T2WI with confirmatory 18F-fluorodeoxyglucose-positron
emission tomography (FDG-PET) or FMZ-PET to show
DIAGNOSIS Mesial temporal sclerosis (MTS). ipsilateral hypometabolism. The contribution of MR spec-
troscopy is limited given the fact that both hippocampi may
DISCUSSION MTS also commonly referred to as hip- demonstrate various degrees of decreased N-acetyl aspartate
pocampal sclerosis is the most common pathology in drug- (NAA) secondary to neuronal loss.
resistant mesial temporal lobe epilepsy. MRI is the imaging Surgery is the available treatment option, and postsurgi-
technique of choice for evaluation of MTS. A routine MRI cal seizure freedom is better obtained in MRI or FDG-PET-
seizure protocol should include coronal thin section T2WI positive patients compared with negative ones.
and or FLAIR, quantitative volumetric 3D T1 and post-
contrast T1WI sequences; all perpendicularly aligned to Question for Further Thought
the long axis of hippocampus. The checklist includes hip- 1. What is the most common cause of drug-resistant
pocampal hyperintensity on T2 and FLAIR, volume loss and mesial temporal lobe epilepsy in adults and in pediatric
loss of internal architecture on 3D T1 SPGR, temporal horn population?
enlargement on T2WI on the affected side, diminution in
size of the column of fornix on the affected side, and finally Reporting Responsibilities
lack of enhancement on post-contrast T1WI. When these five Routine reporting is sufficient. Dual pathology in MTS cases
findings exist with the proper clinical context and abnormal is a known phenomenon. Detailed assessment of the brain
EEG, the diagnosis is straight forward. Low-grade gliomas should be obtained focusing on the most common coexist-
may not necessarily involve the hippocampus but the entire ing vascular malformations, low-grade cerebral tumors,
mesiotemporal lobe. Cysts and infarcts are hypointense on and cortical development abnormalities. Bilateral MTS
T1WI. Encephalitis such as limbic or herpes simplex virus should always be kept in mind, and special attention should
(HSV) tends to affect other parts of the temporal lobes and be paid before declaring the brain MRI scan as normal.
may show contrast enhancement or hemorrhagic focus. Hippocampus as a part of neocortex is already hyperintense
428

Case 198 429
in kids and should not be confused with MTS. DWI may be Answer
able to rule out any acute injury to the hippocampus such as 1. MTS is a long-term outcome from a remote injury to the
PCA or AChoA infarct. hippocampus. Therefore it is expected to be seen in adults
rather than the pediatric population. The most common
What the Treating Physician Needs to Know causes of drug-resistant temporal lobe epilepsy is MTS
The laterality or bilaterality of the MTS in adults and tumors or cortical dysplasia in the pediatric
Concomitant pathologies, particularly congenital lesions population.
or inflammatory lesions that may cause mesiotemporal
hyperintensity particularly neuronal migrational problems,
glioma, and encephalitis
Presence of volume loss elsewhere in the temporal lobes

Case
199 CLINICAL HISTORY 33-year-old female seen to establish care for an
ongoing brain tumor.
FINDINGS Figure 199-1. Axial T2WI through the poste- between the cerebellum and the petrosal surface consistent
rior fossa. There is a well-circumscribed 5.4 cm 3.8 cm with a cyst. Supratentorially (not shown) there are multiple
exophytic left cerebellar mass insinuating itself into the left flame-shaped subcortical T2 hyperintensities radiating and
cerebellopontine angle (CPA) (arrows), compressing the left tapering unto the ventricular walls consistent with focal corti-
brachium pontis medially and mildly rotating the brainstem. cal dysplasia (FCD). Figures 199-2 and 199-3. Axial FLAIR
The fourth ventricle is compressed. Mass is composed of a and T1WI through the mass. There are alternating hypoisoin-
lacy and striped pattern of alternating isointensity and hyper- tense and isointense stripes through the mass. The hypoin-
intensity. There is a large extraaxial cyst on the right (star) tense areas correspond to the hyperintensities on T2WI
430

Case 199 431
consistent with cystic areas, while the isointense areas are on MR spectroscopy. Adult-onset medulloblastoma is usually
the hypertrophied cerebellar folia. Figures 199-4 and 199-5. eccentric in location and may not show contrast enhancement.
Axial ADC map and DWI through the mass. There is repli- A striped pattern similar to LDD has been reported.
cation of the striped pattern in a predominantly hyperintense CS is a very rare disorder characterized by multiple ham-
mass on both DWI and ADC map. There is no restricted dif- artomas of the three germ cell layers inherited in an autoso-
fusion. Figure 199-6. Left parasagittal post-contrast T1WI. mal dominant fashion. Adult-onset LDD is pathognomonic
There are a few linear enhancing structures consistent with for the syndrome. LDD is a benign (WHO I) cerebellar mass
enlarged interfolial veins (arrows). regarded as a malformation/hamartoma composed of dis-
torted enlarged cerebellar folia which are filled by ganglionic
DIFFERENTIAL DIAGNOSISAdult-onset medulloblas cells. There is hypertrophy and coarse gyral pattern of the
toma, dysplastic gangliocytoma of the cerebellum (Lhermitte- affected area of the cerebellum. This could be monofocal or
Duclos disease [LDD]). occasionally multifocal. LDD grows very slowly. The com-
mon denominator between adult-onset LDD and CS is the
DIAGNOSIS Dysplastic gangliocytoma of the cerebellum presence of germline PTEN mutations. There is a propensity
or LDD (pathognomonic for Cowden syndrome [CS]). for development of hamartomas particularly trichilemmoma
and malignant lesions of the skin, thyroid, breast, endome-
DISCUSSION Adult-onset dysplastic gangliocytoma of trium, and gastrointestinal tract. Our patient had several
the cerebellum otherwise known as Lhermitte-Duclos dis- tumors removed in the past. These include a left cerebel-
ease (LDD) is pathognomonic for CS. The MRI features of lar tumor possibly precursor of the present mass, a bladder
LDD consist of enlargement and distorted architecture of tumor, a thigh tumor, and a right breast tumor. CS has no
the involved cerebellum with a typical striped appearance gender preference and occurs in all ages with the average
of enlarged folia interspersed with small cystic areas. On all age at diagnosis of 34 years. PTEN mutations are generally
sequences, the cystic areas follow cerebrospinal fluid (CSF) absent in children with LDD.
intensity, while the striped folia are isointense to cerebellar
tissue. DWI and ADC maps show diffuse hyperintensity in a Question for Further Thought
striped manner. There is usually no contrast enhancement, but 1. What are the presenting features of CS?
prominent interfolial veins are known to opacify with contrast.
Mass effect may be pronounced depending on the size and the Reporting Responsibilities
fourth ventricle could be compressed resulting in hydrocepha- Direct reporting is essential. LDD is not a malignant lesion,
lus. Other imaging features include macrocephaly, heterotopic but there is a propensity for development of malignant
gray matter (GM), and cortical dysplasia. Areas of increased lesions elsewhere. Presence of hydrocephalus makes direct
rCBV within the lesion have been correlated to regions of reporting urgent. It is important to document location and
FDG-hypermetabolism and high thallium (201-Tl) uptake. number of lesions if more than one. Other associated intra-
Elevated level of lactate and decreased levels of myoinosi- cranial lesions such as FCD or heterotopic GM should be
tol, choline, and N-acetyl-aspartate have been demonstrated communicated.

432 Case 199

Location and number of lesions 1. LDD may precede the appearance of the full syndrome.
Other associated findings or complications LDD may present with cerebellar signs, signs of raised
Need to monitor development of other hamartomas and intracranial pressure, and cranial nerve palsies. Multiple
malignant lesions elsewhere trichilemmoma lesions are pathognomonic. Other ham-
artomas and malignant lesions as described above may
present subsequently.

Case 200 CLINICAL HISTORY 48-year-old male with headache.
433

434 Case 200
FINDINGS Figure 200-1. Axial DWI through the lateral ven- tumor blush or very vascular tumor. Most of the differential
tricles. There is a 4.7 cm 3.3 cm ovoid cystic solid mass diagnosis could mimic neurocytoma. They are also cystic
in the left lateral ventricle with local mass effect on the lat- and solid with calcifications or hemorrhagic components.
eral ventricular walls. The solid component is hyperintense Neurocytoma is designated a WHO II neuroepithelial
(arrow). The corresponding ADC map (not shown) shows tumor mostly occurring in adolescent and young adults with
hypointense solid component consistent with restricted a mean age of 29 years with about 70% presenting between
or reduced diffusion. There is mild ventricular dilatation. 20 and 40 years. It has no gender preference. Headache and
Figure 200-2. T2WI through the lateral ventricles. The solid visual disturbance are the prevalent presenting symptoms
components (arrow) are isointense with cortical gray mat- with papilledema and ataxia as the most frequent present-
ter (GM) somewhat slightly higher than white matter (WM) ing signs. Neurocytomas account for between 0.25% and
intensity. The cystic portion has cerebrospinal fluid (CSF) 0.5% of all intracranial tumors. Tumor is usually composed
intensity (star). Figure 200-3. Axial FLAIR. The solid areas of uniform round cells within a fibrillary matrix with focal
are mildly hyperintense. Figure 200-4. Axial post-contrast glial fibrillary acidic protein (GFAP) reactivity in less than
T1WI. There is no contrast enhancement. Mass has isoin- half the cases. Treatment is by surgical removal with che-
tense solid areas with hypointense (or CSF intensity) cystic motherapy and radiation treatment reserved for incomplete
components (arrow). resection and recurrence.
DIFFERENTIAL DIAGNOSISOligodendroglioma, pilocytic Question for Further Thought

astrocytoma, giant cell astrocytoma, ependymoma, central 1. Are EVNs different from intraventricular neurocytomas?
neurocytoma.
DIAGNOSIS Central liponeurocytoma. Direct reporting is necessary because this is a tumor. Size,
location, and number if more than one and the presence of
DISCUSSION Central neurocytoma is a rare intraventricu- hydrocephalus should be reported.
lar predominantly lateral ventricular mass, but extraventric-
ular neurocytoma (EVN) is not unknown. Third and fourth What the Treating Physician Needs to Know
ventricular neurocytomas are rare. It is a cystic/solid mass Location, size, and number if more than one
in 85% of the cases, inhomogeneously isointense on T1WI Presence of hydrocephalus
with hypointense areas representing vessels (62%) or calci- Leptomeningeal enhancement that may suggest metastases
fications (69%) or hemorrhagic product. It is iso- or hyperin- Is there a need for craniospinal axis imaging? This is
tense on T2WI with mild-to-moderate contrast enhancement. always necessary in all high-grade intraventricular tumors
Cystic degeneration is common and is hypointense or CSF
intensity on all sequences. Neurocytoma may show sig- Answer
nificantly increased choline, decreased N-acetyl aspar- 1. EVNs are similar but show some variation in the biologic
tate (NAA), and creatine at MRS with lower Normalized and histologic features compared to the intraventricular
apparent diffusion coefficient (NADC) values; findings that lesions. There could be some imaging differences arising
may resemble high-grade glioma. Hydrocephalus may be a from disruption of the bloodbrain barrier resulting in per-
feature depending on its location and usually due to fora- ilesional edema in hemispheric lesions, but not all hemi-
men of Monro obstruction. Periventricular CSF permeation spheric lesions show surrounding edema. Hemispheric
may be present in such a situation. The presentation here is lesions are also usually well defined with cystic degen-
therefore somewhat different from the majority of neuro- eration, calcifications, hemorrhage, and varying degrees
cytomas. Our tumor shows mild restricted diffusion but no of contrast enhancement and could mimic a high-grade
contrast enhancement. NCCT shows iso- to slightly hyper- tumor such as high-grade astrocytoma, Primitive neuro-
dense mass with mild-to-moderate heterogeneous contrast ectodermal tumor (PNET), or oligodendroglioma. EVNs
enhancement in most neurocytomas. About 51% show are also more frequently associated with poorer outcome
hyperdensity consistent with calcifications. DSA may show compared to their intraventricular counterparts.

Case
201 CLINICAL HISTORY Adult with conductive hearing loss.
LS TS
LSC LSC
PG
MS
OW
FINDINGS Figures 201-1 and 201-2. Axial contiguous

NCCT of the left temporal bone. There is a smooth expan-
sion of the facial nerve canal primarily involving the proxi-
mal tympanic segment (arrow TS). The labyrinthine (or
fallopian) segment (arrow LS) is also expanded. The soft
tissue component in the epitympanum (*) contacts and dis-
places the ossicles laterally. The otic capsule and ossicles are
intact. Figures 201-3 and 201-4. Coronal and sagittal NCCT
through the tympanic cavity, respectively. The enlarged
facial nerve is passing under the lateral semicircular canal
(LSC) and filling the oval window (OW) niche on the coro-
nal (Figure 201-3) and sagittal (Figure 201-4) CT. The sagit-
tal image also shows contiguous expansion of the posterior
genu (arrow PG) and proximal mastoid segment (MS) of the
Figure 201-5
facial nerve canal. Figure 201-5. Axial post-contrast T1WI
435

436 Case 201
MRI through the mass. There is avid enhancement of the encasing bony structures as our case demonstrates. This is
lesion in the anterior genu region. a supportive, distinguishing feature from more aggressive
lesions such as glomus tumor. When asymptomatic, they are
DIFFERENTIAL DIAGNOSIS Facial nerve schwannoma, observed with monitoring for tumor growth and loss of facial
glomus tympanicum tumor, perineural spread of tumor, nerve function. When symptomatic, a translabyrinthine sur-
physiologic facial nerve enhancement, hemangioma, greater gical resection and cable nerve graft are usually attempted,
superficial petrosal nerve (GSPN) schwannoma. although symptomatic relief has been described with surgi-
cal decompression of the nerve alone.
DIAGNOSIS Facial nerve schwannoma.
DISCUSSION CT and MRI are complementary in the 1. What other segments of the facial nerve may be involved?
evaluation of facial nerve schwannoma. Contrast-enhanced 2. What imaging features distinguish GSPN schwannoma
CT is of limited value, and contrast administration should from facial nerve schwannoma?
be reserved for MRI that will show an avidly enhancing
tubular mass. The bone erosion and expansion are well Reporting Responsibilities
demonstrated by NCCT. The lesion usually enhances Direct reporting is essential both when the tumor is discov-
homogeneous on post-contrast T1WI and is hyperintense on ered unexpectedly and in the symptomatic patient. Both CT
T2WI and rarely exceeds 1 cm in cross-sectional diameter. and MRI should be recommended as complementary studies.
Occasionally, intratumoral cysts may form. The tumors CT will demonstrate bone and ossicular chain involvement,
margins may appear more irregular along the MS second- whereas MRI is more sensitive in detecting the full extent
ary to tumor expansion into adjacent air cells. This nerve of the lesion (IAC, CPA, extracranial segments). When pos-
sheath tumor may grow along any portion of cranial nerve sible, differentiate from vestibular schwannoma if the tumor
VII, but unlike its vestibular counterpart, is rarely isolated is primarily isolated to the cistern or IAC.
to the internal auditory canal (IAC). It will often grow in a
discontinuous fashion along the nerve with skip lesions
in between. It commonly extends over a few segments of
the nerve as in our case where it involves the labyrinthine, Segments of the nerve involved, including CPA, IAC, and
geniculate, tympanic, and proximal mastoid segments. any extracranial/parotid extension
When it does involve the IAC, it may present like a ves- Relationship to middle ear structures, particularly the
tibular schwannoma with sensorineural hearing loss, tinni- ossicles
tus, and/or vestibular symptoms and may occasionally grow
into the cerebellopontine angle (CPA) cistern. Interestingly, Answers
until the tumor is large, it rarely results in facial nerve dys- 1. Rarely, the tumor will extend into or be isolated to the
function that is often incomplete. As in our case, tumor of extracranial segments and intraparotid branches of the
the TS in the middle ear may result in conductive hearing nerve. Here, whenever possible, be careful to differentiate
loss. Glomus tympanicum has a very distinctive bluish color from perineural spread of a parotid neoplasm. Perineural
at otoscopy, and the epicenter is in the tympanic cavity but spread is classically attributed to adenoid cystic carci-
could erode into the jugular foramen and adjacent structures. noma but may also be seen in lymphoma, squamous cell
Hemangioma on the other hand may show some calcifica- carcinoma, and melanoma.
tion. Bone destruction is not usually present in physiologic 2. A schwannoma of the GSPN (carrying parasympathetic
facial nerve enhancement. fibers) will grow anteromedially, enlarging its bony canal
Facial nerve schwannomas are histologically identical toward the vidian canal. Here, it joins the deep petrosal
to vestibular schwannomas (named after Theodor A. H. nerve (sympathetic fibers) forming the vidian nerve. It
Schwann [1810 to 1882]). They are typically slow-growing may present as a contiguous mass within the adjacent
tumors, allowing for remodeling and expansion of the middle cranial fossa.

Case
202 CLINICAL HISTORY 23-year-old female with bladder problems and
urinary frequency.
FINDINGS Figure 202-1. Axial FLAIR through the infe- DISCUSSION PA is a low-grade tumor that has a ten-
rior temporal lobes. There is a well-circumscribed hyperin- dency to be supratentorial and lobar in adults. They occur
tense right temporal lobe mass (arrow). There is no edema. more in the temporal and parietal lobes while opticochi-
Figure202-2. Coronal post-contrast T1WI through the asmic lesions (more common in children) are unusual in
mass. There is mildly heterogeneous solid enhancement adults. The cerebellum is the second most common loca-
(arrow). Figure 202-3. Axial ADC map through the mass. tion in adults. The tumors are often solid in about 50%
There is high diffusion with a central normal focus (arrow). with variable contrast enhancement from strong to only
Figure202-4. Photomicrograph shows compact cellular minimal. Large cystic tumors with nodules (21%) or mixed
(upper right) and loose myxoid (lower left) areas. Cells are cystic and solid enhancement (29%) occur frequently. CT
elongated in compact areas (H&E stain). shows a well-circumscribed hypodense mass with hetero-
geneous enhancement with very little or no surrounding
DIFFERENTIAL DIAGNOSISStroke, abscess, demyelin edema. The cystic component shows cerebrospinal fluid
ation, pilocytic astrocytoma (PA), ganglioglioma, pleomorphic (CSF) density. MRI shows hypointense or isointense solid
xanthoastrocytoma (PXA), dysembryoplastic neu roepithelial component on T1WI, generally hyperintense on FLAIR
tumor (DNET), oligodendroglioma. and T2WI with avid homogeneous or heterogeneous con-
trast enhancement. The cystic component tends to follow
DIAGNOSIS Pilocytic astrocytoma (PA) lobar. CSF intensity on all sequences but may not completely sup-
437

438 Case 202
press on FLAIR. There is little or no surrounding edema. either region. Necrosis and glomeruloid vascular hyperpla-
Hemorrhage or calcification is uncommon. Perfusion is sia can be seen and should not be confused with high-grade
generally not elevated but could be mildly increased, and glioma. Cure is achieved by total resection.
there is no restricted diffusion. MRS may show elevated
choline and creatine and lactate with low N-acetyl aspartate Questions for Further Thought
(NAA) confusing this low-grade tumor with malignancy. 1. What is the preferred treatment for PA in the adult?
Lack of calcification differentiates lobar PA from oligo- 2. Can malignant change occur in long-standing lobar PA?
dendrogliomas and PXA. The rounded appearance and lack
of diffusion restriction make ischemia very unlikely. Solid Reporting Responsibilities
enhancement and lack of centrally restricted diffusion dif- All tumors deserve direct reporting. Presence of significant
ferentiate this from an abscess. The mass was erroneously mass effect or obstruction to CSF pathways makes direct
read out as a potential demyelinating plaque despite the reporting more urgent. Changes in character and size of the
fact that the location is a little bit unusual, and the enhance- tumor on follow-up should be stressed.
ment was solid rather than a partial ring. Calcifications are
more common in ganglioglioma, but the location is perfect. What the Treating Physician Needs to Know
DNET could be difficult to differentiate from this tumor Location of lesion is important for surgical decision
in this location. PXA is often cortical based with a cyst making
and a nodule of enhancement that abuts the leptomeninges Are there any significant interval changes on follow-up?
with associated leptomeningeal enhancement and/or corti- Any significant mass effect or obstruction to CSF
cal remodeling. pathways?
Presentation depends on location and varies from sei-
zures, headache, and neurologic deficit. PAs are well- Answers
circumscribed WHO I astrocytomas composed of compact 1. Complete and aggressive surgical resection results in
cellular areas consisting of elongated and fibrillated cells that much better outcome. Stereotactic radiosurgery may be
alternate with loose myxoid, microcyst-rich areas. Nuclei beneficial for unresectable PA. A standardized adjuvant
contain bland chromatin. Mitoses are none to rare. Brightly treatment protocol for unresectable PA does not exist.
eosinophilic Rosenthal fibers are common in compact areas, 2. Malignant change may rarely occur in long-standing
whereas eosinophilic granular bodies (EGBs) can be seen in lobar PA.

Case
203 CLINICAL HISTORY 75-year-old male complaining of dizziness.
FINDINGS Figure 203-1. Axial NCCT of the brain through appear normal with no evidence of spasm. Figures 203-3 and
the supra sella cistern. There is a 1-cm pear-shaped hyper- 203-4. Coronal and axial MIP CTA, respectively. The rela-
dense structure in the interhemispheric fissure separating the tionship of the aneurysm neck (line arrow) to the other vascu-
posterior aspect of the bilateral gyrus rectus (arrow). This lar structures is defined. The right A1 is missing presumably
structure is about the same size as it was 8 years earlier when congenital, a normal variant. Normal left A1 (arrow).
it was first noticed on NCCT. It was suggested that it could
represent an aneurysm. There was no hemorrhage on the pre- Differential Diagnosis N/A.
vious or on the present CT. Figure 203-2. 3D volume-ren-
dered CTA submentovertical (SMV) view. There is a 1.2-cm DIAGNOSIS Anterior communicating artery aneurysm
lobulated outpouch consistent with an aneurysm (arrow) of (A-Com-A).
the anterior communicating artery emanating from the junc-
tion of the left A1, and bilateral A2s and absence of right A1 DISCUSSION This is a medium-sized A-Com-A. Saccular
segment. Aneurysm is pointing superiorly and anteriorly sep- aneurysms are classified as small under 5 mm, medium 5 to
arating the two A2s. It has a clearly defined neck that mea- 15 mm, large 15 to 25 mm, and giant 25 to 50 mm. A-Com-A
sures about 4.5 mm. There is a right-sided lobule or daughter is a common saccular aneurysm that represents roughly about
aneurysm pointing to the right. The other vascular structures 30% to 35% of intracranial saccular aneurysms. Unruptured
439

440 Case 203
aneurysm on NCCT could evade detection. However, pres- difficult to know when an aneurysm would rupture. It is
ence of hyperdense focus in the basal subarachnoid space also important to suggest further confirmation by CTA or
should raise the suspicion for an aneurysm as in this situation. MRA which was eventually done in this case. CTA mea-
Rupture of A-Com-A usually results in concentration of blood surements should include the aneurysm neck, dome to
in the anterior suprasellar cistern or anterior interhemispheric neck, and maximum measurement. Direction of projection
fissure. This is hyperdense on NCCT. If the rupture point is of aneurysm and changes in the surrounding vessels should
against any of the frontal lobes, a frontal lobe parenchymal also be reported.
hematoma should indicate the location of the aneurysm. A
hypodense or calcified filling defect within the subarachnoid What the Treating Physician Needs to Know
hemorrhage (SAH) in the anterior suprasellar cistern or in
Size and location of aneurysm and number if more than
the interhemispheric fissure could also suggest the location
one
of the ruptured A-Com-A. One of the interesting features of
Evidence of previous bleed or complication such as hydro-
this aneurysm is the presence of lobulation, tit, and daughter
cephalus and encephalomalacia
aneurysm usually associated with prior rupture. There are no
Measurements, direction of projection, presence of adjoin-
symptoms and signs of prior rupture in this patient. Congenital
ing vascular and bony structures, compressions of sur-
absence or hypoplasia of A1 segment of the anterior cerebral
rounding structures and numbers if more than one
artery (ACA) is a common congenital variant. It could fac-
tor into how this patient is ultimately managed knowing that
bilateral A2 derive their blood supply from a single A1. Answer
A-Com-A may be clinically silent like the majority of 1. The management of an unruptured and asymptomatic
intracranial aneurysms. Clinical presentation depends on aneurysm is a controversial one. It has been suggested
the size, compression of surrounding structures, or rupture. that this decision should be individualized. The preva-
A-Com-A has been known to present with headache, visual lence of unruptured aneurysm in the population is about
problems, dementia, endocrine problems, hyposmia, and sei- 2.3% which is significantly higher than the prevalence of
zures and to cause loss of consciousness and focal neurologic SAH suggesting that most aneurysms do not rupture. It is
deficit usually associated with a rupture. suggested from recent data that aneurysms of similar size
that never hemorrhaged have a rupture rate of 2.6%. Other
Question for Further Thought factors to consider when deciding on the management of
1. Should the aneurysm have been investigated and treated an unruptured aneurysm include patients level of toler-
when it was initially suggested or discovered on NCCT ance of anxiety and depression that goes with watchful
8years earlier? waiting or the so-called time bomb effect of living with an
aneurysm, frequency of follow-up and reliability of such
Reporting Responsibilities assessment techniques, and the choice and acceptance of
Although there was no SAH on the initial or follow-up CT, associated morbidity of both investigation techniques and
it is still necessary to directly report this aneurysm. It is treatment options.

Case
204 CLINICAL HISTORY 65-year-old female with cognitive decline,
depression, and history of TIAs.
441

442 Case 204
FINDINGS Figure 204-1. Axial NCCT through the corona These are believed to result in arteriolar luminal narrowing
radiata. There are patchy periventricular white matter (WM) and ischemia with subsequent myelin pallor and rarefaction.
hypodensities (arrows) and a mild degree of global brain vol- Although the basis is assumed to be arterial in nature, some
ume loss. Figures 204-2 and 204-3. Axial T2WI and FLAIR authors think that venous abnormalities may be responsible
respectively through the corona radiata. There is mild to for it. Small amounts of leukoaraiosis have no clinical cor-
moderate volume loss. There are both patchy and confluent relates, but significant changes are associated with cogni-
WM hyperintensities in bilateral corona radiata (arrows). tive declines. Increased incidence of leukoaraiosis is seen
Figure 204-4. Corresponding axial DWI shows no acute in hypertensive and especially diabetic patients supporting
infarctions. the explanation that the changes are due to arterial disease.
Patients with extensive leukoaraiosis are also at risk for cere-
DIFFERENTIAL DIAGNOSIS Leukoaraiosis, cerebral bral infarctions.
atherosclerosis, amyloid angiopathy, transependymal cere-
brospinal fluid (CSF) flow, radiation-induced damage, Questions for Further Thought
cerebral autosomal dominant arteriopathy with subcortical 1. What is the association of these findings with stroke?
infarcts and leukoencephalopathy (CADASIL). 2. Which sequence can be utilized to evaluate for microhe-
morrhages? Which of the two listed differential diagnoses
DIAGNOSIS Leukoaraiosis. can show microhemorrhages?
DISCUSSION Leukoaraiosis, used synonymously with
chronic small vessel ischemia and WM changes, is a
Routine reporting is sufficient. Describe the distribution and
slowly progressive degenerative process involving the WM
extent of density or signal abnormality and comment on vol-
commonly seen in older adults. CT often shows patchy
ume loss, lobar involvement, severity, and whether patchy or
poorly defined hypodensities in the WM of the corona radi-
confluent. Exclude other causes of patient symptoms such as
ata and centrum semiovale. They are usually monotonous in
acute hydrocephalus or acute infarctions.
shape and configuration as opposed to variation in the shape
of WM lesions in demyelinating process. They are mostly
deep WM in location, but strict periventricular and subcorti- What the Treating Physician Needs to Know
cal lesions are often present. These lesions may be conflu- Whether or not the patient has an acute ischemic event
ent particularly around the frontal and occipital horns. MRI, Whether or not the findings have progressed, if compari-
particularly the FLAIR sequence, is better at fully bringing son studies are available
these lesions to light showing them as areas of hyperintensi- Presence of hydrocephalus or other causes to explain the
ties without mass effect. There is invariable association of symptoms
volume loss which could be predominantly central resulting Presence of microhemorrhage
in ventriculomegaly. They do not contrast enhance and do
not restrict diffusion. Cystic areas within these lesions are Answers
considered due to chronic lacunar infarcts. Confluent lesions 1. WM disease and stroke are associated to a certain extent
could mimic amyloid angiopathy and Binswanger disease. by their shared risk factors. Progressive WM disease
Inferior extension into the capsules and anterior temporal increases the patients risk of ischemic stroke, especially
lobes involvement may suggest CADASIL. In the appropri- lacunar strokes. Several studies show on average a three-
ate clinical setting, radiation-related leukoencephalopathy fold increase in stroke incidence in patients with leuko-
could resemble confluent leukoaraiosis. Periventricular CSF araiosis. The changes of leukoaraiosis are not associated
flow is usually located around the frontal and occipital horns with restricted diffusion on ADC maps as acute infarc-
initially and is associated with hydrocephalus. tions are.
The pathogenesis and pathology of leukoaraiosis is 2. Susceptibility-weighted imaging (SWI). Both amyloid
incompletely understood. Endothelial damage and hyaline angiopathy and radiation-induced WM damage can show
degeneration with thickening of the vessel wall is observed. microhemorrhages.

Case
205 CLINICAL HISTORY 62-year-old female for stroke evaluation.
443

444 Case 205
Figure 205-6
Figure 205-5
FINDINGS Figure 205-1 Axial post-contrast CT. There is composed of a very thin arachnoid membrane. The immedi-
a right frontal extraaxial cerebrospinal fluid (CSF) density ate surrounding brain is usually cortical GM which could be
collection with compression of the right frontal lobe slightly thickened and flattened due to mass effect. PC on the
(vertical arrow). There is mild thinning and remodel- other hand is surrounded by gliotic tissue since it is a result of
ing of the overlying right frontal bone (transverse arrow). parenchymal destruction. PC also communicates freely with
Figures 205-2 to 205-4. Axial DWI, FLAIR, and T2WI, the CSF space. The majority of ACs are noncommunicating,
respectively, through the lesion. The collection follows CSF while a small percentage may communicate via a ball valve
intensity on all sequences. There is compression of the fron- mechanism which could be demonstrated by CT cisternogram
tal lobe (vertical arrows). Thinning of the right frontal bone or phase contrast cine MR. Epidermoid tumor follows CSF
is again demonstrated (transverse arrows). Figures 205-5 intensity on all spin echo sequences but restricts diffusion
and 205-6. Axial NCCT and axial T2W MRI, respectively, which distinguishes it from AC. However, some ACs may
through the right middle cranial fossa in a 3-year-old boy. contain proteinaceous fluid or become hemorrhagic making
These demonstrate an arachnoid cyst (AC) in a more typical differentiation difficult. Hemorrhage into ACs could be spon-
location in the right middle cranial fossa. There is smooth taneous, traumatic, or due to a ruptured aneurysm. Racemose
compression of the frontal and temporal lobes (arrows) with neurocysticercosis is usually multiloculated with septations.
overlying right temporal bone remodeling and thinning seen Pilocytic astrocytoma and hemangioblastoma are parenchy-
on the CT. In these two cases like in all cases, the mass is mal lesions with solid mostly contrast-enhancing components.
extraaxial to cortical gray matter (GM). AC accounts for less than 1% of all intracranial masses
and is widely accepted as developmental anomalies in which
DIFFERENTIAL DIAGNOSIS AC, epidermoid cyst, neuro- splitting or duplication of the primitive arachnoid mem-
cysticercosis, porencephalic cyst (PC), pilocytic astrocytoma, brane allows subarachnoid fluid collection. It is a common
hemangioblastoma. benign lesion occurring in the CNS both within the intra-
cranial compartment (most common) and within the spinal
DIAGNOSIS Arachnoid cyst (AC). canal. Common intracranial locations include the middle cra-
nial fossa/sylvian fissure (50% to 60%), suprasellar (10%),
DISCUSSION Both CT and MRI are capable of demon- quadrigeminal plate cistern (10%), cerebellopontine angle
strating the changes of AC, but MRI is the preferred method (5% to 10%), supracerebellar cistern (<5%), and cisterna
of choice. AC is an extra axial CSF containing mass located magna (<5%).
in the subarachnoid space compressing the underlying brain Most cases begin during infancy; however, onset may
with remodeling and thinning of the overlying calvarium. be delayed until adolescence. Although the vast major-
Itis homogeneously CSF hypodense on CT and follows CSF ity is sporadic, they are seen with increased frequency in
intensity on all MRI sequences. There is usually no vascular mucopolysaccharidoses. The majority of ACs is small and
structure within it, and the wall can rarely be identified being asymptomatic. Headache and seizures are the most common

Case 205 445
symptoms. When symptoms occur, they are usually the Answers

result of gradual enlargement resulting in mass effect. This 1. No. Most ACs are incidental, asymptomatic, and do not
results in either direct neurologic dysfunction, or distortion cause seizures. However, since ACs are more common
of normal CSF pathways resulting in obstructive hydroceph- in the middle cranial fossa than elsewhere, there is the
alus. Surgical treatment when necessary includes surgical suggestion that underlying temporal lobe compression,
excision, surgical fenestration, or cyst shunting into the CSF dysplasia, or hypogenesis could lead to seizures.
space or peritoneum. 2. It has been suggested that symptomatic ACs are usually
the growing or enlarging ACs and that these symptom-
Questions for Further Thought atic ACs tend to communicate with the arachnoid space
1. Does AC cause seizure? via a ball valve mechanism that allows CSF to enter but
2. Is there any difference between communicating and not exit. CT cisternography and/or phase contrast cine
noncommunicating AC? MR have been used to demonstrate presence of such
communication. There is a consensus that symptomatic
Reporting Responsibilities cysts causing seizures, hydrocephalus, focal neurologic
This is a benign usually incidental finding and requires rou- deficits, or raised intracranial pressure may benefit from
tine reporting. It is important to report the size, presence of surgical management and demonstration of communi-

bone remodeling, and associated hydrocephalus if any. cation between the ACs and the subarachnoid space is
important in the preoperative evaluation.
Size and location
Presence of adjacent compressed or dysplastic tissue
Communicating or noncommunicating in symptomatic
lesions at CT cisternogram or phase contrast cine MR

Case
206 CLINICAL HISTORY 28-year-old female with headache and new-onset
diplopia.
FINDINGS Figure 206-1. Axial T2WI through the petrous Reporting Responsibilities
apices. There is hyperintensity of the right petrous apex (arrow). Direct reporting is essential in this infection. First, recog-
Figure 206-2. Axial post-contrast T1WI. There is subtle nize and report any asymmetric opacification of the aer-
enhancement of the bone and surrounding soft tissues (arrow). ated petrous apex. This may be the only abnormality on
an otherwise normal head CT. When petrous apicitis is
DIFFERENTIAL DIAGNOSIS Trapped fluid, petrous api- suspected, recommend MRI follow-up. On MRI, assess the
citis, cholesterol granuloma, cholesteatoma, tumor. adjacent skull base for extension of osteomyelitis, exclude
the presence of epidural spread of infection, and verify that
DIAGNOSIS Petrous apicitis. the nearby arterial and venous flow voids are present indi-
cating patency.
DISCUSSION CT findings of petrous apicitis include loss
of aeration or trabecular pattern with hypodensity of the
petrous apex with variable contrast enhancement. The MR
T1WI often shows a hypointense petrous apex with contrast Petrous apicitis is analogous to, and often coexists with,
enhancement. Lesion is hyperintense on FLAIR and T2WI otomastoiditis
and may show some expansion of the petrous apex. Antibiotic therapy will usually be sufficient for treatment,
Petrous apicitis refers to a phlegmonous, infected col- but if diagnosis is delayed, severe complications as dis-
lection involving the petrous apex. Aeration of the petrous cussed above can develop
apex is a precondition for the development of this disease. The constellation of facial pain and lateral rectus dysfunc-
Petrous apicitis may be seen in isolation or with concomi- tion is highly suggestive of a petrous apex or skull base
tant infection of the middle ear and mastoids. As both cranial process and should prompt imaging evaluation
nerves (CN) V and VI may be affected by inflammation in The extent of the disease and complications
the petrous apex, the constellation of facial pain (CN V) and
lateral rectus palsy (CN VI) leading to diplopia, referred to as Answer
Gradenigo syndrome, is highly suggestive of the diagnosis. 1. Petrous apex opacification without enhancement or mar-
As the disease progresses, loss of the bony trabeculae in the row edema, and particularly in the absence of symptoms,
apex and cortical erosion are expected findings, to which CT is suggestive of trapped fluid. A cholesterol granuloma
is very sensitive. Infection can extend beyond the petrous will appear markedly and homogeneously T1 hyperin-
apex resulting in osteomyelitis of the skull base, particularly tense. Cholesteatoma will appear as a smooth, expansile
the adjacent clivus, epidural abscess, and venous thrombosis. mass without inflammatory change and may restrict on
diffusion imaging. With tumor, look for solid enhance-
Question for Further Thought ment and invasion of the adjacent structures.
1. How does one differentiate petrous apicitis from the other
entities in the differential?
446

Case
207 CLINICAL HISTORY 69-year-old female fell in the shower and became
unresponsive.
447

448 Case 207
FINDINGS Figure 207-1. Axial NCCT through the tento- brain such as the midbrain, hypothalamus, and chiasm are
rial incisura. The upper pons/midbrain (star) is deformed, inferiorly displaced obliterating the suprasellar cistern. All
slightly hypodense, and displaced to the right compressed CSF spaces within the tentorial incisura such as the perimes-
against the right tentorium (Kernohan notch [KN]). There is encephalic, quadrigeminal, and interpeduncular cisterns are
effacement of the surrounding interpeduncular, prepontine, obliterated. The brainstem is deformed and elongated in the
perimesencephalic, and quadrigeminal cisterns. The tento- anteroposterior direction. Continuous pressure on the brain-
rium is dense due to hemorrhage (transverse arrows). There stem produces edema, kinking of the basilar artery summit,
is medial displacement of the left uncus behind the dorsum and shearing of the brainstem perforating arteries causing
sella consistent with uncal herniation (UH) (black verti- linear DH within the brainstem.
cal arrow). The right temporal horn is dilated and trapped The pathogenesis of DH remains controversial. They
(chevron). The cause of the herniation is a large left hyper- are considered arterial in origin. There may be venous
acute subdural hematoma (SDH) (left lateral transverse congestion because of the rostrocaudal compression of the
arrow). Figure 207-2. Axial NCCT through the suprasellar brainstem. Conceivably, this could produce venous engorge-
cistern. There is complete effacement of the suprasellar and ment, thrombosis, and infarction with hemorrhages in the
interpeduncular cisterns (star), perimesencephalic cisterns, brainstem. Differential diagnosis of DH includes direct and
and the quadrigeminal cistern by descending brain tissue shearing traumatic brainstem hemorrhages, hypertensive
transtentorial herniation (central herniation). Structures hemorrhage, and hemorrhage due to vascular malformations.
at the base of the brain such as cerebral peduncles, hypo- DHs are linear in configuration and directed in an antero-
thalamus, chiasm, and the uncus cannot be differentiated. posterior direction within the brainstem particularly upper
The right trigone (vertical arrow) is dilated. The left hemi- pons and midbrain. However, DHs could be round, ovoid, or
spheric SDH (transverse arrows) is larger. Figure 207-3. irregular in configuration. They are more common ventrally
Coronal reformatted NCCT through the tentorial incisura. and laterally. The incidence of DH varies widely between
The tentorium is demonstrated by the vertical arrows. There autopsy and imaging series being present in up to 60% of
is effacement of the cerebrospinal fluid (CSF) space around traumatic brain injury autopsies and up to 10% of imaging
the tentorial incisura due to descent of supratentorial struc- series. Presence of DH is a very bad sign with high mortality.
tures (star). There are multiple hyperdense foci in the brain- The outcome in survivors is dismal.
stem (line arrow). These are the Duret hemorrhages (DHs). Other complications of DTTH include infarctions in the
The third ventricle is effaced and displaced to the right. contralateral posterior cerebral artery, anterior choroidal
There is midline shift subfalcine herniation (SFH) from left artery, and superior cerebellar artery territories due to infe-
to right (transverse arrows) by the left hemispheric SDH rior displacement and/or pinning of these vessels against
(chevron). Figure 207-4. Axial NCCT through the pons. the contralateral tentorium. Hypothalamic and basal gan-
There is elongation of the pons in an anteroposterior direc- glia infarcts are due to compression of the lenticulostriate
tion with effacement of surrounding CSF spaces. The DHs and perforating arteries at the base of the brain. Obstructive
(arrows) are shown as thick linear hyperdensities within the hydrocephalus of the contralateral lateral ventricle is due to
deformed pons. There is hyperdense hemorrhage along the obstruction at the level of the third ventricle and foramen
left tentorium. of Monro produced by the SFH. The KN may result in ipsi-
lateral hemiplegia. Third nerves palsy due to compression
within the interpeduncular cistern results in the classical
dilated and nonreactive pupils of rostrocaudal herniation.
These patients are usually obtunded. The eventual outcome
DIAGNOSIS Descending transtentorial herniation (DTTH)
if the herniation is not resolved is compromise of the respira-
featuring left UH, left subfalcine herniation (SFH), DH.
tory center and death.
DISCUSSION CT is the method of choice for demonstrat-
ing acute herniations and hemorrhages in traumatic head
injuries. The clinical environment in acute trauma may be 1. What is central herniation?
very unstable, thus preventing the use of MRI. Herniations
and DH are secondary traumatic injuries. DTTH is the Reporting Responsibilities
second most common herniation of the brain after SFH. This is an emergency requiring direct reporting. The magni-
Supratentorial masses, parenchymal or extraaxial, push down tude of all primary and secondary injuries should be catego-
on the cerebral hemisphere. Structures move inferiorly and rized. Any significant changes on follow-up should also be
medially under the falx resulting in SFH. The mesiotempo- directly reported.
ral lobe flows over the suprasellar cistern creating UH. The
uncus subsequently is displaced posteriorly and inferiorly What the Treating Physician Needs to Know
behind the dorsum sella. The brainstem is displaced laterally All primary injuries. These may include contusions, hema-
against the free edge of the contralateral tentorium creating tomas, extraaxial collections, brain swelling, and sub-
the deformity known as KN. Structures at the base of the arachnoid and intraventricular hemorrhages

Case 207 449
All secondary injuries. These may include herniations, to bihemispheric pathology pushing down on the central
hydrocephalus, infarctions, DH, and brain swelling brain structures through the tentorial incisura. This may
CT is the best imaging modality in the acute phase of sig- involve bilateral UH compressing the midbrain from
nificant trauma for both initial and follow-up evaluation. either side resulting in anteroposterior elongation of the
MRI may be used as a problem-solving tool midbrain and pons. There is also associated rostrocaudal
pressure on the central structures such as the midbrain,
Answer hypothalamus, and pons. It has been described in the
1. The term central herniation is not popular. It has been context of brain swelling from any cause, bihemispheric
used to describe a form of transtentorial herniation due extraaxial collections, and central base of brain masses.

Case
208 CLINICAL HISTORY 2-week-old male with increasing head circumference.
FINDINGS Figure 208-1. The initial imaging study was a DIFFERENTIAL DIAGNOSISMedulloblastoma, glioblas-
head sonogram. This shows a large hyperechoic mass in the toma, atypical teratoid rhabdoid tumor (ATRT), ependymoma.
posterior fossa (arrows). Figure 208-2. Axial NCCT through
the posterior fossa. The mass is hyperdense and well cir- DIAGNOSIS ATRT.
cumscribed (arrows). There is hydrocephalus with transep-
endymal cerebrospinal fluid (CSF) flow (vertical arrows). DISCUSSION ATRT is an aggressive, WHO IV tumor
Figure 208-3. Axial T2WI through the posterior fossa. The most commonly found in infants and children under 3 years.
mass is heterogeneously hypointense with mild surround- The posterior fossa is the most common site of disease, but
ing hyperintensityedema (arrows). Figure208-4. Sagittal it can occur elsewhere. Up to 20% of patients present with
post-contrast T1WI. The mass shows mild patchy enhance- leptomeningeal spread at diagnosis. Imaging features are
ment (arrows). There is compression of the brainstem. similar to those of the more common medulloblastoma, with
450

Case 208 451
which ATRT shares quite a few features. On CT, the tumor aggressive-appearing tumor in young children under 3 years
is hyperdense reflecting both hemorrhage and a composition of age, particularly when medulloblastoma would also be
of densely packed small cells. Heterogeneous intensity is appropriate. Look for evidence of leptomeningeal spread as
expected on T1WI and T2WI with evidence of cysts, hem- this finding significantly impacts survival. If none is seen in
orrhage, and enhancement. Leptomeningeal enhancement is the brain, suggest imaging of the remainder of the neuroaxis
indicative of CSF spread of the tumor. (also appropriate for medulloblastoma).
Clinical presentation includes macrocrania because of
hydrocephalus, vomiting, failure to thrive, and visual symp- What the Treating Physician Needs to Know
toms with headache in the older children. There is a male ATRT is an aggressive primitive tumor found in children
predominance. Evaluation of the entire neuroaxis is always mostly under 3 years of age
necessary to exclude CSF seeding. The prognosis is poor. Presence of leptomeningeal spread at diagnosis is not rare
Longer survival is recorded in children older than 3 years and significantly impacts survival
presumably because of the use of intensive therapies. Presence of complications such as hydrocephalus
Spinal imaging is required to exclude CSF spread
1. Can ATRT be differentiated from medulloblastoma by Answer
imaging? 1. No. By imaging, ATRT and medulloblastoma in the
posterior fossa are identical. They are even to some
Reporting Responsibilities degree similar histologically as they both contain small
Direct reporting is essential in this obstructing tumor with primitive-type cells. The age may be a helpful clue as
hydrocephalus. Include ATRT in the differential for an ATRT is rare in children older than 3 years.

Case
209 CLINICAL HISTORY 77-year-old male with a 2-year history of progressive
dementia, urinary incontinence, gait instability, and increased frequency of falls.
FINDINGS Figure 209-1. Axial T2WI through lateral ven-

tricles. There is ventricular enlargement out of proportion
to sulcal dilation. There is bilateral frontal and occipital
periventricular caps (arrows). Figure 209-2. Axial FLAIR
through the body of the lateral ventricle. There is confluent
periventricular hyperintensity over the frontal and occipital
regions suggestive of transependymal cerebrospinal fluid
(CSF) flow (arrows). Figure 209-3. Axial NCCT through the
lateral ventricles following ventriculoperitoneal (VP) shunt-
ing. There is normalized caliber of the ventricles with reso-
lution of periventricular caps and interval appearance of thin
subdural collections (arrows). Figure 209-4. Pre-operative
In-111 diethylene triamine pentaacetic acid (DTPA) cister-
nography. There is persistent radiotracer uptake in the lat-
eral ventricles (star) at 24 hours.
DIFFERENTIAL DIAGNOSIS Normal aging brain,

Alzheimer disease, vascular dementia, sporadic subcortical
arteriosclerotic encephalopathy, Parkinson disease, normal
pressure hydrocephalus (NPH).
DIAGNOSIS NPH.
DISCUSSION NPH is defined as idiopathic communicat-

ing hydrocephalus where the degree of the triventricular dila-
Figure 209-3 tation is out of proportion to degree of cortical sulcal dilation
452

Case 209 453
Figure 209-4
under normal CSF pressure. The fourth ventricle is usually Question for Further Thought
spared. Conventional MRI demonstrates disproportional 1. Does the degree of ventricular enlargement correlate with
ventricular enlargement compared with sulcal dilatation. symptoms?
There may be confluent periventricular T2 hyperintensity,
indicating transependymal CSF flow and/or edema second- Reporting Responsibilities
ary to impaired venous flow and/or periventricular arterial Direct reporting may be necessary particularly if this entity
ischemia. Biphasic flow at the cerebral aqueduct on cine is not clinically suspected. The lack of WM T2 hyperinten-
phasecontrast MRI is well correlated with good response sities in a mild-to-moderate triventricular enlargement may
to shunting. Periventricular and deep white matter (WM) be secondary to normal aging. If that is the case and diag-
T2 hyperintense lesions which may be due to small vessel nosis is indeterminate, radionuclide cisternography with
ischemic changes are signs of poor outcome after ventricular In-111 DTPA should be performed.
shunting. The diagnostic indicators of NPH are flow void in
cerebral aqueduct on MRI and prominent ventricular activ- What the Treating Physician Needs to Know
ity at 24 hours on In-111 DTPA cisternography. Aqueductal
NPH is a surgically treatable condition, whereas Alzheimer
flow void sign reflects increased uncompensated CSF flow.
disease or vascular dementia is not
Thinning of the corpus callosum is always present.
If dementia is the preceding symptom compared with gait
Presence of significant WM infarctions could point
disturbance or lack of urinary incontinence, other dement-
in the direction of vascular dementia as centrally pre-
ing syndromes should be suspected
dominant atrophy may produce similar disproportionate
Alzheimer disease should be favored where hippocampal
enlargement of the ventricles. The disproportionate ven-
or medial temporal volume loss is the most significant
tricular enlargement rules out normal aging process and
finding. Disproportional dilatation of the parahippocampal
Parkinson disease.
fissure is characteristic for atrophy seen in Alzheimer dis-
NPH is a treatable cause of dementia, and imaging is
ease but not in NPH
adjunctive to determine the candidates who will benefit
Vascular dementia should be favored if multiple infarcts
from ventricular decompression. The typical presentation is
are striking especially in the deep WM and the basal g anglia
the Hakim triad of gait apraxia, urinary incontinence, and
dementia. Lumbar puncture (LP) was performed in this
patient with the CSF opening pressure at 18 cm H2O. LP Answer
may be used as a therapeutic trial of VP shunting. Patients 1. To our knowledge the acuity of hydrocephalus correlates
who respond to large-volume tap are generally supposed to with symptoms instead of degree of hydrocephalus. The
benefit from VP shunting. degree of hydrocephalus is a better sign of cortical atrophy.

Case
210 CLINICAL HISTORY 43-year-old male presenting with strange
behavior and speech difficulty for 2 days, fever two or three times in
the past 6months with chronic cough.
454

Case 210 455
FINDINGS Figures 210-1 and 210-2. Axial contiguous i ntravenous drug use, poor dental hygiene, or recent dental pro-
NCCT through the sylvian fissures. There is a large area cedures. Echocardiogram in this patient revealed mitral valve
of hyperdensity (acute hemorrhage) (arrows) straddling the regurgitation with vegetation. Such vegetation breaks off and
left sylvian fissure involving the left operculum and insula occludes distal cerebral arteries resulting in septic infarcts and
region. There is a peripheral hypodense halo of edema with the bacteria/fungus/virus from septic emboli escape through
local mass effect. Figure 210-3. Axial MIP CTA of the head the vasa vasorum and cause severe inflammation of the adven-
through the same level. There is a small pear-shaped con- titia and weakens the vessel wall. The arterial pulsation against
trast collection within the left perisylvian hematoma (verti- the weakened vessel wall eventually results in aneurysm for-
cal arrow). There is straightening of the left M2 (transverse mation and enlargement. PMA generally requires prolonged
arrow) due to mass effect. Figure 210-4. 3D surface shaded antibiotic treatment for at least 6weeks with or without
CTA. There is a pear-shaped aneurysm along one of the M3 aggressive neurosurgical management. However, intervention
branches (arrow). has been shown to improve mortality.
DIFFERENTIAL DIAGNOSIS Primary intracerebral hema Question for Further Thought

toma (PICH), secondary intracerebral hematoma (SICH). 1. What are the treatment options for PMA?
DIAGNOSIS SICH due to peripheral mycotic aneurysm Reporting Responsibilities

(PMA) with intracerebral and subarachnoid hemorrhage. PMA and its complications are emergent situations requiring
direct reporting. Because PMA could be multiple in over a
DISCUSSION PMA is a consequence of distal embolic third of cases, efforts should be made to exclude additional
infection or septic emboli mostly from endocarditis. PMA lesions. Scrutiny of the CTA or MRA should be thorough.
could be single or multiple. They are usually silent until Serial DSA has been recommended in one major report to
they rupture and produce subarachnoid hemorrhage (SAH) monitor progress of these lesions.
or intracerebral hematoma (ICH) as in this instance.
Unruptured PMA may present with mass effect. Common What the Treating Physician Needs to Know
imaging presentation is usually peripheral or lobar ICH or
How many are there? The locations are important for sur-
SAH which is hyperdense on CT. Mass effect depends on
gical planning
the size of the parenchymal hematoma and septic infarcts.
What are the associated complications, SAH, ICH, intra-
The infarct is hypodense on CT. A peripheral hematoma in
ventricular hematoma (IVH), infarcts, or is the aneurysm
a young individual deserves further evaluation to exclude
unruptured?
treatable causes such as aneurysm or vascular malformation.
CTA is preferred over MRA, but both could be effective in
CTA or MRI/MRA identifies the underlying aneurysm as an
the diagnosis and follow-up of PMA. Aneurysms less than
outpouch of a small vessel.
3 mm are less likely to be adequately evaluated by MRA.
About 65% of patients with PMA have infective endocar-
DSA has equally been recommended. DSA is not without
ditis (IE). About 60% of PMA in one series had ruptured at
its complications
presentation and 73% of these had IE. MCA territory is the
commonest location of PMAs. The age range for PMA is from
35 to 55 years with a slight male predominance. Fever, splinter Answer
hemorrhages, focal neurologic deficits, headache, and mental 1. Complete resolution of PMA has been achieved in about
status changes are some of the presenting clinical features. 30% of cases on antibiotics alone. Endovascular treat-
The common organisms involved are Staphylococcus aureus ment options include coil, stent, and glue. Neurosurgical
and Streptococcus species. Underlying favorable environ- options include clipping, excision, coagulation, and
ments include cardiac valvular disease, immunosuppression, external carotid-internal carotid (EC-IC) bypass.

Case
211 CLINICAL HISTORY 56-year-old male with delirium and aphasia a few
weeks following liver transplant.

456

Case 211 457
DISCUSSION MRI is the examination of choice for

the evaluation of patients suspected to have ODS. In this
particular instance, there is a combination of CPM and
EPM. This combination occurs in about 30% of ODS. CT
if obtained may demonstrate hypodensity centrally in the
pons, while the changes elsewhere may not be apparent.
Typical MR changes are central large pontine T2 hyper-
intensity sparing the peripheral fibers and bilateral sym-
metrical T2 hyperintensities in the MCPs, internal and
external and extreme capsules, corpus callosum, subcor-
tical areas, basal ganglia, thalamus, mammillary bod-
ies, midbrain, cerebellum, and hippocampus. In the pons,
sparing of the corticospinal tract is not uncommon result-
ing in what resembles a panda face pattern. When the
corpus callosum is involved the central fibers are gener-
ally the ones affected as in this case. Changes have been
reported in the spinal cord. Interestingly, in this particular
case, the EPM show restricted diffusion, while the CPM
did not restrict diffusion. Restriction of diffusion has been
Figure 211-7 reported in all sites. DTI tends to show disruption of fiber
tracts. Some instances of peripheral contrast enhancement
have been reported. Long-term outcome ifthe patient sur-
FINDINGS Figures 211-1 and 211-2. Axial MR DWI and vives is shrinking of the lesions with total disappearance
corresponding ADC map through the brachium pontis. There in some regions such as the corpus callosum, white matter
is bilateral symmetrical ovoid area of diffusion restriction (WM), and the basal ganglia. The clinical setting is crucial
in the middle cerebellar peduncles (MCPs) (arrows). Figure in excluding the differential diagnoses. The basal ganglia
211-3. The corresponding axial FLAIR. There is hyperin- and sometimes cortical involvement may mimic HIE. Lack
tensity not only in the bilateral MCP but also in the pons of the usual prodromal syndromes excludes ADEM.
with a thin rim of normal pontine intensity (arrows). Figure ODS is a rare disorder that has a slight male prepon-
211-4. Axial DWI through the inferior basal ganglia. There derance. ODS is a known complication of orthotopic liver
is bilateral symmetrical restricted diffusion in the external transplant and occurs in up to 15% of this population. This is
capsules (chevrons), bilateral subthalamic regions (trans- due to the major electrolyte disturbances that often compli-
verse arrows), and posterior limb of internal capsules (ver- cate liver transplant. Disturbances associated with ODS in
tical arrows). Figures 211-5. Axial DWI and ADC map, the liver transplant setting include hyponatremia and hyper-
respectively, through the basal ganglia. There is symmetri- natremia, hypokalemia, cyclosporine use, and pancreatitis.
cal restricted diffusion in the posterior limb of the internal The underlying pathology is myelin loss with neuron and
capsules (transverse arrows) and in the splenium of the cor- axon sparing associated with swelling. There is usually no
pus callosum (vertical arrows). There is subtle hyperinten- inflammation associated. ODS is typically biphasic clini-
sity in the head of caudate nucleus bilaterally. Figures 211-6 cally and the patient might present with encephalopathy fol-
and 211-7. Axial FLAIR through the basal ganglia region lowed by improvement. A combination of clinical signs and
and the splenium level, respectively. There are symmetrical symptoms might follow 2 or 3 days later that may include
hyperintensities in the bilateral external capsules (transverse quadriparesis with dysarthria and dysphagia to a com-
arrows) and posterior limbs of the internal capsules (vertical plete locked-in syndrome, hallucinations, akinetic mutism,
arrows) in Figure 211-6 and splenium of corpus callosum seizures, and coma.
(arrow) in Figure 211-7. Note the sparing of the peripheral
fibers in the corpus callosum. All the affected structures
show mild enlargement.
1. How is CPM treated?
DIFFERENTIAL DIAGNOSISOsmotic demyelination
syndrome (ODS): central pontine myelinolysis (CPM) and Reporting Responsibilities
extrapontine myelinolysis (EPM), hypoxic ischemic encepha- Direct reporting is appropriate in any situation where there is
lopathy (HIE), acute disseminated encephalomyelitis (ADEM). restricted diffusion and the differential diagnostic possibili-
ties may include hypoxic ischemic change, infection, and/or
DIAGNOSIS ODS CPM and EPM. inflammation.

458 Case 211

Presence of significant mass effect 1. There is no known treatment for ODS regardless of
A negative imaging study does not exclude ODS. There is etiology. Proposed treatments in the literature include
a lag between clinical presentation and imaging appearance reinduction of hyponatremia, plasmapheresis (PP),
The classical teaching is that ODS is associated with rapid IVIG, and corticosteroids. These treatments have not
correction of hyponatremia. Nevertheless, ODS has been proven useful.
reported in the context of slow correction of hyponatremia

Case
212 CLINICAL HISTORY 22-year-old male with history of cystic fibrosis and
seizure disorder presents with the worst headache of his life. Imaging was
obtained to rule out subarachnoid hemorrhage or aneurysm.
Figure 212-1
FINDINGS The initial NCCT was normal. Figure 212-1.

Sagittal CTA MIP. There are multiple segmental vascu-
lar irregularities involving multiple territories (arrows).
Figure212-2. Axial MIP through the centrum semiovale.
There is extensive vascular irregularitysignificant bifron- Figure 212-2
tal arterial attenuation and beading (transverse arrows) with
some dilated vessels posteriorly (vertical arrows).
DIFFERENTIAL DIAGNOSISVasculitis, vasculopathy, been reported in all ages but appears to peak in the fifth
vasospasm. decade. The syndrome is characterized by severe thunderclap
headaches with features suggestive of subarachnoid hemor-
DIAGNOSIS Reversible cerebral vasoconstriction syndrome rhage, with or without other symptoms such as focal neuro-
(RCVS). logic deficits. Laboratory investigations including erythrocyte
sedimentation rate (ESR) and lumbar puncture (LP) are usu-
DISCUSSION The imaging findings in RCVS are usually ally normal or minimally abnormal with minimally raised
nonspecific. Initial imaging could be normal in up to 55% cell count or elevated protein in the cerebrospinal fluid (CSF)
of the patients and becoming subsequently abnormal. MRI without any evidence to support vasculitis. RCVS is presum-
or CT could be abnormal in 12% to 81% with abnormalities ably due to a transient disturbance in the control of cerebro-
consisting of focal mainly convexity subarachnoid hemor- vascular tone presumably due to vasoactive substances. The
rhages, parenchymal hemorrhage, ischemic infarcts mainly disease is self-limited and the symptoms tend to resolve spon-
in watershed locations, posterior reversible encephalopathy taneously if there are no complications. The vascular imaging
syndrome, and brain edema. MRA and/or CTA tend to dem- changes tend to resolve within 3 months. Persistent headache
onstrate changes that suggest vasculitisvascular irregu- may require a more aggressive treatment with nimodipine or
larities, string of beads pattern, alternating constriction, and corticosteroids. Death has been reported in a few cases.
vasodilation of vessels. The initial angiogram could be nor-
mal and repeat angiogram may show resolution of lesions. Question for Further Thought
Based on the angiographic findings, the differential should 1. How do you differentiate vasculitis from RCVS?
include vasculitis, vasculopathy, and vasospasm. Occasional
aneurysm and vascular dissections have been reported. Reporting Responsibilities
RCVS is increasingly recognized. It is more common Direct reporting is necessary. The clinical concern is usually
in women than men. Generally, it is presumed that there for subarachnoid hemorrhage. Findings such as convexity
is a trigger. It is common in the postpartum period, during subarachnoid hemorrhage, ischemic infarcts, parenchymal
stressful periods, physical and emotional exertion, and has hemorrhage, and brain swelling should be reported if present.
459

460 Case 212

Location of lesions and associated parenchymal changes 1. It could be difficult to distinguish between the two enti-
or complications ties. Helpful hints to the diagnosis of RCVS include
Imaging may not be able to differentiate vasculitis from thunderclap headache, various patterns of cerebrovas-
vasculopathy or RCVS; the findings are similar cular events, normal or near-normal CSF, and ESR.
Changes of RCVS may not be present on initial imag- There is usually reversal of the vascular irregularities on
ing and may take up to 2 weeks to appear. It is there- angiography without treatment. Vasculitis or angiitis,
fore necessary to repeat the imaging if symptoms persist. on the other hand, has an insidious onset of the head-
Maximum vasoconstriction could take up to 16 days to ache; cerebrovascular changes are usually of ischemic
occur after the ictus. Reversal of angiographic findings infarcts, CSF changes of inflammatory reaction, and an
takes up to 12 weeks elevated ESR.

Case
213 CLINICAL HISTORY 5-year-old female presenting with generalized seizure
activity and apnea with a Glasgow Coma Scale (GCS) of 3 on admission.

461

462 Case 213
FINDINGS Figure 213-1. Axial NCCT through the upper reported in CD. The posterior fossa may be small or normal
cerebellum shows abnormal and irregular shape of the fourth in size. There may be other associated changes supratentori-
ventricle with poor definition of the velum (posterior arrow). ally such as gray matter heterotopia and midline malforma-
The interpeduncular cistern could be seen anteriorly (ante- tions. Absence of molar tooth sign (MTS) excludes JS and
rior arrow) suggesting a high-riding fourth ventricle. Figure JSRD. The age of this patient and degree of brainstem vol-
213-2. Sagittal post-contrast MRI T1WI shows widening of ume loss exclude olivopontocerebellar atrophy.
the fourth ventricle with the top of the fourth ventricle at Like all hindbrain malformations, the presentation of CD
the level of the interpeduncular cistern (transverse arrow). could be complex depending on other associated anoma-
The superior velum is slightly flat (vertical arrow). Figure lies. CD generally manifests in infancy or early childhood.
213-3. Axial T2WI through the mid-fourth ventricle dem- Developmental delay, hypotonia, facial deformities, and
onstrates widening of the fourth ventricle with a large nod- respiratory and coordination issues tend to dominate the
ule posteriorly. There is widening of the prepontine and clinical presentation. Seizures are not uncommon. CD has
cerebellopontine cisterns with a left pericerebellar cerebro- been reported in association with other hindbrain and wide-
spinal fluid (CSF) space widening. The cerebellar folia are spread brain malformations, congenital muscular dystrophy,
obliquely directed and slightly asymmetric (arrows). Small congenital infections, alcohol-related disorders, and trisomy
but otherwise normal cerebellar peduncles and pons are syndromes. The management and the natural outcome tend
present. Figure 213-4. Axial T2WI through the upper pos- to depend on the associations.
terior fossa showing abnormal foliation with irregular cleft
through the superior cerebellum (vertical arrow). There is Question for Further Thought
bilateral gray matter heterotopia supratentorially (transverse 1. Is high-riding fourth ventricle (i.e., at the ponto-mesence-
arrows). Figure 213-5. Coronal T2WI through the trigones. phalic junction) pathognomonic for JS or JSRD?
There is hypoplasia of the falx with focal interdigitation
of the cerebral hemispheres across the midline (transverse Reporting Responsibilities
arrows). Gray matter nodular heterotopia is present (chev- Routine reporting is sufficient in this instance unless these
rons). Small left cerebellum with surrounding enlarged CSF changes are unexpected. Presence of CD requires fur-
space (inferior left transverse arrow). There is vermis hypo- ther search for other abnormalities elsewhere in the brain.
plasia. Figure213-6. DTI color orientation map through the Conversely, supratentorial congenital malformations should
posterior fossa. There is asymmetry and disorganization of instigate scrutinization of posterior fossa for associated
the fiber tracts (arrows). cerebellar lesions.
DIFFERENTIAL DIAGNOSIS Cerebellar dysplasia (CD),

olivo-ponto-cerebellar atrophy, Joubert syndrome (JS), and
Joubert syndrome-related disorder (JSRD). MR is the examination of choice for posterior fossa evalu-
ation. Some of these findings may be unexpected
DIAGNOSIS Cerebellar dysplasia (CD). Apart from known posterior fossa syndromes, CD is non-
specific, and its clinical presentation could be variable
DISCUSSION Findings in unclassified CD are those of A large number of genetic mutations are responsible for
abnormal formation of cerebellar tissue which may include various forms of cerebellar malformations in association
abnormal sulcal and folia orientation, lack of proper mid- with midbrain and forebrain anomalies. Genetic testing
line formation with interdigitation of cerebellar folia across may not be out of place in these children
the midline, abnormal widening of the CSF spaces includ-
ing clefts and malposition or irregular outline of the fourth Answer
ventricle and disorganized fiber tracts. DTI confirms disor- 1. High-riding fourth ventricle is a known component of the
ganization and asymmetry of the fiber tracts. Heterotopic JSRD but can occur in some other posterior fossa malfor-
gray matter, pachygyria, and polymicrogyria have all been mations. The MTS is the pathognomonic sign for JSRD.

Case
214 CLINICAL HISTORY 7-year-old male who had been developing normally
started 12 months before with hearing loss which progressed to visual and
coordination problems followed by an overall decline of intellectual and motor
milestones.
FINDINGS Figure 214-1. Axial T2WI through the trigones. contrast-enhanced MRI. However, on CT, the affected WM
There is bilateral symmetrical peritrigonal confluent white is hypodense and generally located in the posterior aspects
matter (WM) hyperintensity extending from the ventricular of the brain surrounding the trigones. MRI especially T2WI
walls to the subcortical U fibers (stars) crossing the midline and FLAIR show confluent symmetrical hyperintensity of
through the splenium of corpus callosum. Figure 214-2. the peritrigonal WM linking up across the splenium. The
Coronal post-contrast T1WI through the trigones. There is affected cerebral WM typically has three different zones.
bilateral symmetrical centrally affected WM hypointense The central or inner zone appears moderately hypointense
with peripheral wavy contrast enhancement following the on T1WI and markedly hyperintense on T2WI. This zone
pattern of the subcortical U fibers (arrows). corresponds to irreversible gliosis and scarring. The inter-
mediate zone represents active inflammation and breakdown
DIFFERENTIAL DIAGNOSISKrabbe disease, meta- in the bloodbrain barrier. This zone may appear isointense
chromatic leukodystrophy (MLD), Alexander disease, or slightly hypointense on T2WI and readily enhances in a
Adrenoleukodystrophy (ALD), Canavan disease. wavy pattern after contrast administration. The peripheral
or outer zone represents the leading edge of active demy-
DIAGNOSIS Adrenoleukodystrophy (ALD). elination. It appears moderately hyperintense on T2WI and
demonstrates no enhancement. MR spectroscopy obtained
DISCUSSION ALD describes several closely related from the enhancing region shows elevated choline reflect-
inherited disorders that affect the metabolism of certain lip- ing active inflammation, low N-acetyl aspartate (NAA), and
ids resulting in the accumulation of very long chain fatty lipids. When performed in the nonenhancing region, most
acids (VLCFAs) in the affected tissues, including the myelin metabolites are low, and lipids and lactate are abundant.
of the nervous system, the Leydig cells of the testes, and The abnormalities initially affect the periventricular WM
the adrenal cortex. There is, however, no clear genotype with eventual spread to the subcortical regions. Symmetric
phenotype correlation. Imaging of ALD is best achieved by abnormal areas of T2 hyperintensity along the descending
463

464 Case 214
pyramidal tract may be present. Atypical cases with unilat- are also at risk for adrenal insufficiency. Lastly, the adre-
eral or predominantly frontal lobe involvement may occur. nal gland failure or Addison type shows signs of adrenal
The brain retains its volume until late in the disease. The insufficiency such as increased skin pigmentation, decreased
abnormalities may extend inferiorly to involve the midbrain. appetite, vomiting, weight loss, loss of muscle mass, muscle
During the latter stages of the disease, the affected WM may weakness, and possible coma.
show calcifications. Krabbe and MLD are global affection
of the centrum semiovale and corona radiata, while ALD Question for Further Thought
is confined posteriorly. Canavan tends to affect posterior 1. In a male child with WM changes on MR and the clini-
corona radiata and centrum predominantly, while Alexander cal suspicion of ALD what are some appropriate tests for
favors the frontal WM. further workup and diagnosis?
ALD is a genetic metabolic disorder inherited in an
X-linked pattern. Therefore, the majority of affected individ- Reporting Responsibilities
uals are male as they are homozygous for the affected gene. Direct reporting is important because dietary intervention
Women carriers can have milder forms of the disease. It has may halt progression of the disease. The Loes score is a
an incidence of 1:20,000 people with no ethnic predilection. severity rating of the abnormalities in the brain found on
Three subtypes of the disease are recognized: the childhood MRI. It ranges from 0 to 34, based on a point system derived
cerebral form which appears in mid-childhood ages 4 to 8 from the location and extent of disease and the presence of
years, adrenomyelopathy occurs in men in their twenties or atrophy in the brain, either localized to specific points or gen-
later, and impaired adrenal function, called Addison disease erally throughout the brain. A Loes score of 0.5 or less is
or Addison-like phenotype in adult. The typical clinical pre- classified as normal, while a Loes score of 14 or greater is
sentation of the childhood cerebral form is that of a hither to considered as severe.
healthy young boy, 4 to 8 years old, with new-onset emotional
instability and disruptive behavior at school. Other symptoms
may include adrenal insufficiency, decreased fine motor con-
trol (deterioration of handwriting), decreased muscle tone, Genetic counseling is recommended for prospective
aphasia, hearing loss, strabismus, visual disturbances, diffi- parents with a family history of ALD
culty swallowing, and possible seizures. This is a progressive Prenatal diagnosis from chorionic villus sampling is
disease that leads to a persistent vegetative state anywhere available
from 2 to 10 years after onset of neurologic symptoms.
The adrenomyelopathy form presents with difficulty Answer
controlling urination, muscle weakness or leg stiffness, and 1. Blood tests can detect VLCFA or these may also be found
decreased mentation and visual memory. These patients in skin biopsies.

Case
215 CLINICAL HISTORY 21-year-old female with infertility.
FINDINGS Figure 215-1. Coronal dynamic post-contrast DIFFERENTIAL DIAGNOSISPituitary microadenoma,

T1WI. There is a 6-mm nonenhancing mass (arrow) in the Rathke cleft cyst, pars intermedia cyst.
right side of the pituitary gland. Figure 215-2. Delayed
static high-resolution corresponding post-contrast T1WI in DIAGNOSIS Pituitary microadenoma.
the same patient also shows the adenoma. Figure 215-3.
Coronal 90-second dynamic post-contrast coronal T1WI in DISCUSSION Pituitary microadenomas usually mani-
a companion case shows a right-sided adenoma (arrow). fest on imaging as intrasellar masses measuring less than
Figure215-4. Coronal post-contrast delayed T1WI. The 10 mm in diameter that enhance at a slower pace than the
tumor is less conspicuous due to enhancement that makes normal pituitary gland since the majority of them have an
it more difficult to differentiate from the adjacent gland. indirect blood supply compared with the more direct blood
This case shows the advantage of using dynamic imaging supply to the normal gland. The majority of these lesions
for evaluation of microadenomas. are located in the adenohypophysis and demonstrate a
465

466 Case 215
round or ovoid morphology. Since most microadenomas the cavernous sinuses. Treatment of symptomatic micro-
are prolactin-producing and most lactotrophs are located in adenomas includes both medical and surgical options.
the lateral aspects of the gland, most adenomas occur there.
MR is superior for detection of microadenomas and detects Questions for Further Thought
an additional 10% when compared with those detected by 1. What distinguishes a microadenoma from a macroad-
CT. Adding dynamic post-contrast images increases MRI enoma?
sensitivity even more particularly in detecting very small 2. What is the concept behind dynamic contrast-enhanced
adenomas such as those responsible for Cushing disease. On imaging to diagnose adenomas?
T2WI, microadenomas are generally bright but their signal
may vary. Bleeding into an adenoma is not rare and when it Reporting Responsibilities
results in symptoms of increased intracranial pressure and Routine reporting is sufficient. Describe the size and location
cranial neuropathies it is referred to as pituitary apoplexy. of the mass and level of confidence of the diagnosis. Even
Many patients with adenomas present with endocrine though an MRI may be ordered specifically to evaluate the
abnormalities, most commonly infertility, galactorrhea, pituitary, make sure to evaluate the remainder of the brain for
and amenorrhea in women and impotence in men. The additional possible abnormalities.
other common microadenomas are the adrenocorticotropic-
producing (which are generally small and difficult to iden- What the Treating Physician Needs to Know
tify) and growth hormone-producing ones. Others, such as Size, location, and imaging characteristics of the mass
those related to thyroid-stimulating and follicle-stimulating Degree of confidence in diagnosis as this may affect
hormones, are rare. Null cell adenomas tend to be asymp- treatment
tomatic except when large and resulting in mass effect and
are probably at least as common as prolactin-producing Answers
tumors. Rathke cleft cysts and pars intermedia cysts do not 1. A microadenoma measures less than 10 mm and a mac-
contrast enhance and could mimic microadenoma. roadenoma is larger than 10 mm.
There is a strong correlation between elevated pro- 2. Microadenomas are believed to enhance more slowly than
lactin levels and the presence of an adenoma but some normal pituitary tissue; therefore, multiphase T1 coronal
microadenomas are discovered incidentally. If the serum imaging after administration of contrast may highlight the dif-
prolactin level is higher than 200 ng/mL, an adenoma is ference in signal intensity between the more slowly enhanc-
almost always present. Serum levels above 1000 ng/mL ing microadenoma and the surrounding normal pituitary at
generally indicate an aggressive tumor that has invaded some point in time, which increases diagnostic accuracy.

Case
216 CLINICAL HISTORY 19-year-old female liver transplant recipient was
admitted for seizures. Three weeks prior to admission, she was treated with
high-dose steroids for rejection.
FINDINGS Figures 216-1 and 216-2. Axial DWI and cor- the areas of restricted diffusion on the DWI. Figure 216-4.
responding ADC map through the occipital lobes. There Axial non-contrast T1WI through the occipital lobes. There
is a large irregular left occipital lobe mass with thick ring is a large left occipital hypointensity. There is bilateral
restricted diffusion (arrows) surrounding a small core of globus pallidus T1 hyperintensity (arrows) consistent with
increased diffusion. There is a surrounding rim of high dif- effect of parenteral hyperalimentation. Figures216-5 and
fusion on the ADC map considered to represent vasogenic 216-6. Axial T2WI and FLAIR, respectively, through two
edema. Figure 216-3. Axial GRE through the mass. There is a different levels of the left occipital mass. There is a lobu-
thick irregular rind of heterogeneous hypointensity (arrows) lated hypointense mass with surrounding vasogenic edema
(possibly hemorrhage or fungal hyphae) corresponding to and local mass effect with local sulcal effacement. We could
467

468 Case 216
not administer contrast because of renal failure. NCCT of abscesses or neoplasm. However, this differentiation could
the head (not shown) obtained before the MRI showed a be difficult. Biopsy is generally recommended for diagnosis.
large left occipital heterogeneous mass. There was no obvi- Clinical symptoms are not sensitive diagnostic tools for
ous acute hemorrhage. IAB. Patients can present with fever, focal neurologic deficit,
There was a second smaller but similar lesion in the left seizures, mental status changes, and headache. Concomitant
parietal lobe (not shown). pulmonary infection is common as in this case, but cases of
isolated cerebral aspergillosis have been reported. IAB has
DIFFERENTIAL DIAGNOSIS Hematoma, pyogenic abscess, been reported in up to 15% of the solid organ transplant
tuberculous abscess, fungal abscess. recipients and up to 11% of the allogeneic stem cell recipi-
ents, with mortality rates ranging from 65% to 92%. Brain
DIAGNOSIS Invasive aspergillosis of the brain (IAB). involvement is usually hematogenous from pulmonary inva-
sive aspergillosis as in this patient. Direct transdural invasion
DISCUSSION Imaging pattern of IAB in the immuno- from surrounding paranasal sinus infection is discussed else-
compromised patient is usually that of solitary or multifo- where in this book. The basic pathology is that of infective
cal rapidly progressive brain parenchymal masses mostly vasculopathy due to angioinvasive nature of the Aspergillus
hemorrhagic infarcts within the deep gray matter and the organism resulting in endothelial proliferation and vascular
subcortical/cortical areas of the brain. Masses are of varying thrombosis with hemorrhagic infarcts or coagulative necro-
sizes and generally hypodense on CT with variable areas of sis, diffuse cerebritis, with or without colonization/abscess,
peripheral hyperdensity consistent with hemorrhage. MRI ventriculitis, and meningitis or meningeal masses. Early
features include hypointense lesions with areas of peripheral diagnosis of this infection, before dissemination to the brain,
hyperintensity on T1WI, hyperintense center with hypoin- is the key to a successful treatment.
tense rim on T2WI with GRE hypointensity peripherally Open brain biopsy in this patient showed necrotizing
suggestive of blood products and fungal hyphae. The pres- inflammatory lesions with fungal septate hyphae compatible
ence of blood or hyphae presents a heterogeneous DWI with Aspergillus spp. Brain biopsies and bronchoalveolar
intensity pattern. There is surrounding vasogenic edema. culture grew Aspergillus nidulans.
Contrast enhancement is variable ranging from none to thin
patchy crenated peripheral enhancement or multifocal ring Question for Further Thought
enhancement. Ventriculitis and meningitis are common 1. Is there any laboratory test for the diagnosis of invasive
and may not necessarily contrast enhance in the immuno- aspergillosis?
compromised. Diffuse parenchymal disseminated infection
resulting in brain swelling or edema or cerebritis with or Reporting Responsibilities
without petechial hemorrhages is rare but reported. A pattern The very many imaging patterns of IAB make the spe-
of venous infarct has also been described. Hydrocephalus cific diagnosis difficult. Any combination of the patterns
is the usual complication of meningitis or ventriculitis. The described earlier points in the direction of invasive infection
presence of blood products or mineralization and heteroge- which deserves direct reporting for purposes of early further
neous DWI pattern differentiate IAB from other forms of evaluation for diagnostic purposes and treatment.

Case 216 469
What the Treating Physician Needs to Know The treatment should be initiated based on suspicion
Specific location of lesions reachable for biopsy will help in the presence of significant risk factors for invasive
the treating physician aspergillosis
Is it safe to perform lumbar puncture (LP)? Significant
mass effect with herniation may preclude LP Answer
Presence of invasive aspergillosis of the lung in the con- 1. Aspergillus galactomannan assay can be useful for the
text of brain masses should prompt a link between both diagnosis of invasive aspergillosis in solid organ transplan-
lesions tation. The test has a better sensitivity (67% to 100%) and
IAB in an immunocompromised host has a very poor prognosis specificity (91% to 98%) if performed on bronchoalveolar
(almost 100% mortality) because of its angioinvasive lavage (BAL) specimens. False-positive galactomannan
widespread thrombotic pathophysiology tests have been reported.

Case
217 CLINICAL HISTORY Adult with severe headache.
FINDINGS Figures 217-1 and 217-2. Coronal and sagittal Multiple punctate similar hyperintensities are present in
reformatted NCCT through the planum, respectively. There the subarachnoid spaces (arrows). Figure 217-4. Axial fat
is a fat density (star), extraaxial left supraclinoid mass with saturation T1WI through the mass. There is suppression of
rim calcifications. In addition, there are scattered fat droplets the hyperintense signal within the left mass. The scattered
throughout the subarachnoid spaces (arrows). Figure 217-3. hyperintense foci within the subarachnoid spaces bilaterally
Axial T1WI through the mass. There is a hyperintense left on the T1WI suppress on the fat sat T1WI as well, confirm-
supraclinoid lesion with hypointense peripheral signal. ing the fatty consistency.
470

Case 217 471
DIFFERENTIAL DIAGNOSIS Dermoid cyst, epidermoid sulci, and ventricles has been described as pathognomonic
cyst, craniopharyngioma, teratoma, lipoma. for diagnosis of a ruptured dermoid cyst.
DIAGNOSIS Ruptured dermoid cyst. Question for Further Thought

1. What should the clinician do if an uncomplicated dermoid
DISCUSSION Dermoid cysts are benign congenital cysts cyst is reported?
containing dermal elements including hair follicles, seba-
ceous glands, and sweat glands. They are typically midline, Reporting Responsibilities
unilocular, and well-circumscribed fat density/intensity Routine reporting is sufficient in most cases of small uncom-
masses without perilesional edema or associated enhance- plicated dermoid cysts. However, in the setting of larger
ment on post-contrast CT or MRI. When intracranial, they tumors, or those associated with clinical and/or imaging
are more commonly found in the suprasellar and parasellar findings of rupture, that is, chemical meningitis, acute hydro-
regions than in the posterior fossa. On CT, they are typically cephalus, or fat droplets within the subarachnoid space, such
low density, with negative Hounsfield unit attenuation, but critical findings should be called urgently to the referring
can rarely be heterogeneous or hyperdense, depending on clinician.
the constitution of the elements of the cyst. Approximately
20% of dermoids are associated with capsular calcification.
On MRI, dermoids are T1 hyperintense and suppress on fat
saturation sequences. On T2WI, the appearance can be more Although relatively rare, even a small dermoid cyst can
variable ranging from hyperintense to hypointense and are spontaneously rupture. The risk of rupture increases with
often heterogeneous. The imaging behavior resembles that increasing size of the dermoid. Rupture can lead to sig-
of lipoma. Epidermoid tends to restrict diffusion and usually nificant morbidity and mortality due to chemical meningi-
eccentric in location. Craniopharyngioma and teratoma are tis, leading to hydrocephalus, seizure, coma, vasospasm,
usually heterogeneous in intensity with elements of calcifica- infarction, and/or death
tion and contrast enhancement. Rarely, dermoid cyst can undergo malignant transforma-
Dermoid cysts are rare, comprising less than 0.5% of pri- tion into squamous cell carcinoma, even years after surgi-
mary intracranial tumors (4 to 9 times less common than the cal resection
more laterally located epidermoid cysts). Clinical presenta-
tion is often in the third decade of life and may be associ- Answer
ated with a long history of vague symptoms predominated 1. In addition to the rare spontaneous rupture, head trauma
by headache. Presentation can also include seizures, focal and inadvertent spillage during surgical resection can
neurologic deficits, and episodes of aseptic (chemical) men- result in chemical meningitis with equivalent results.
ingitis, although these are usually associated with dermoid While treatment is complete surgical resection, some
rupture. Once rupture occurs, fat droplets can disseminate have opted for a wait-and-see approach depending on
throughout the CSF and cause extensive chemical meningi- size, location/accessibility of the lesion, and the presence
tis and demonstrate leptomeningeal enhancement on imag- of associated complications from mass effect upon criti-
ing. Of note, the finding of fat droplets within the cisterns, cal structures.

Case
218 CLINICAL HISTORY 45-year-old female involved in a motor vehicle
collision with sudden deterioration of level of consciousness.
FINDINGS Figure 218-1. Axial NCCT through the level DISCUSSION This case illustrates how quickly subdural
of the frontal horns at about 8 AM when she was brought hematoma (SDH) can increase in size particularly if there is
into the emergency room. There are bilateral extraaxial cres- active bleeding. The CT finding of HSDH is usually a new
centic collections larger and of mixed density (hypodense crescentic extraaxial mixed density (hypo-iso-hyperdense)
and hyperdense), on the left (arrows) and thinner iso- to collection. The newness distinguishes this from a rebleed
hyperdense on the right (chevrons). There is a mild midline into an existing CSDH. The initial CT (Figure 218-1) shows
shift to the right (star). Figure 218-2. Axial NCCT through a hyperacute subdural on the left with a left-to-right mid-
the same level as in Figure 218-1 about 3 hours later at about line shift. Within the following 3 hours, a larger hyperacute
11 AM when the patients condition deteriorated. There is collection has developed on the right side with reversal of
now a larger mixed density (iso-hypo-hyperdense) cres- the midline shift. The hypodense component is said to be
centic extraaxial collection surrounding the right cerebral due to fresh blood and unretracted clot while the hyperdense
hemisphere (transverse arrows). The left extraaxial collec- component represents the retracted clot generally seen in
tion is now partially effaced. There is now a midline shift to acute SDH. Theother explanation for the hypodense por-
the left (star). There is also dilatation (acute hydrocephalus) tion of the collection is the presence of an arachnoid tear that
of the left trigone (vertical arrow) consistent with a trapped allows cerebrospinal fluid (CSF) to enter the subdural space.
left lateral ventricle due to effacement and obstruction at the The mixed density of the left SDH in Figure 218-1 could
level of the foramen of Monro/third ventricle. be confused with a fresh hemorrhage into an existing CSDH
(recurrent SDH).
DIFFERENTIAL DIAGNOSIS Hyperacute subdural hema The history is key usually in these situations and in any
toma (HSDH), recurrent bleed into chronic subdural hema- case the presence of hyperdense SDH indicates at least an
toma (CSDH). acute component that should be taken seriously. The other
important findings here are the larger reversal of midline
DIAGNOSIS Hyperacute subdural hematoma (HSDH) with shift from right to left between the two images and the
active bleed. development of acute left lateral ventricular hydrocephalus
472

Case 218 473
in Figure 218-2 due to the obstruction at the level of the fora- Question for Further Thought
men of Monro or the third ventricle. 1. What is the treatment of choice in this situation?
HSDH is found in about 40% of acute SDH population.
It could be of any size but has a propensity to grow. SDH is Reporting Responsibilities
generally believed to be due to rupture of cortical veins as This is an emergency requiring direct reporting to the refer-
they cross the potential subdural space caused by minor or ring physician. Other primary injuries that are present should
severe abnormal brain rotations or skull fractures. SDH may be reported along with complications such as hydrocephalus
be associated with brain parenchymal injuries or traumatic and herniations.
subarachnoid hemorrhage. Other causes of SDH include
coagulopathies, surgical trauma, ventriculoperitoneal shunts,
and neoplastic lesions of the dura or arachnoid. It has no gen-
der predilection and occurs in all ages. At surgery, HSDH is Acuity of the collection
a reddish purplish gel under a bulging dura representing the Location and size
retracted clot while the unretracted clot flows like unclotted Severity of midline shift, other herniations, and ventricu-
blood. Points of active bleed were found in 11 of 13 patients lar trapping. The midline shift is usually measured so that
with extraaxial hyperacute hemorrhages at surgery in one comparison with follow-up could easily be appreciated
series. Presentation and management depends on the size and
consequences. Smaller collections could be closely moni- Answer
tored clinically and by imaging while large lesions with her- 1. Some measures to reduce edema is usual and of course
niations and/or hydrocephalus are usually surgically treated. immediate surgical evacuation of the HSDH.

Case
219 CLINICAL HISTORY A young female, 3 days postpartum, suddenly
developed headaches and hypopituitarism.
DIAGNOSIS Lymphocytic hypophysitis (LYH).
DISCUSSION A definite diagnosis of LYH is only

achieved with a tissue biopsy; a presumptive diagnosis is
accepted based on clinical, laboratory, and imaging stud-
ies. CT and MRI studies show large symmetrical pituitary
masses with homogeneous and intense contrast enhance-
ment, a thickened and nondisplaced pituitary stalk, dural
enhancement, and sometimes extension into the suprasel-
lar region or cavernous sinuses. On MRI a loss of pos-
terior pituitary T1 hyperintensity may be seen, and an
empty sella can be found at late chronic stages of the
disease. The lesion can be somewhat hypointense on T2
sequences and sometimes show only peripheral contrast
enhancement.
Figure 219-3 Hypophysitis may be classified by its anatomic distribu-
tion and whether it is a primary or secondary finding. LYH is
a rare autoimmune chronic idiopathic inflammation, result-
ing from an infiltration of lymphocytes and plasma cells,
FINDINGS Figure 219-1. Sagittal post-contrast T1WI shows which can be limited to the adenohypophysis (more frequent
a peripherally enhancing lesion in the pituitary gland (verti- presentation), the infundibulum, and neurohypophysis, and
cal arrow) with thickened and enhancing stalk (transverse even the entire hypophysis. It is often seen in pregnant and
arrow). Figure 219-2. Coronal post-contrast T1WI through postpartum women who develop diabetes insipidus, hypo-
the sella turcica shows the lesion extending into the suprasel- pituitarism, and headaches or visual impairment, these last
lar cistern and touching the chiasm (arrow). Figure219-3. symptoms probably related to the compression and inflam-
Corresponding coronal T2WI. There is hypointensity of the mation of neighboring neural structures. Concurrent auto-
periphery of the lesion (arrows) enclosing a hyperintense immune conditions are reported in about one-third of LYH
core. Lack of cavernous sinus involvement and mass effect cases, most of them being autoimmune thyroid disorders
on the optic chiasm are better seen on this sequence. such as Hashimoto thyroiditis, Graves disease, and other
subacute thyroiditis.
DIFFERENTIAL DIAGNOSIS Pituitary hyperplasia, Management is usually based on hormone replacement
pitu
itary adenomas, suprasellar tumors (germinoma or and steroids, and in specific nonresponsive cases, other
astrocytoma), inflammatory pseudotumor, sarcoidosis, and immunosuppressive treatments and even stereotactic radio-
histiocytosis. therapy have been employed.
474

Case 219 475
Questions for Further Thought Exclude other probable causes of a pituitary dysfunction
1. Name other similar pathological inflammatory processes as pituitary apoplexy, other primary hypophysitis (sarcoid
that affect the orbit and cavernous sinus. or histiocytosis), or tumoral disease (suprasellar, sellar, or
2. Are there other types of primary pituitary hypophysitis? parasellar)

Direct reporting is essential because of its clinical features 1. LYH is pathologically similar to orbital pseudotumor and
and implications. Try to exclude other causes of a secondary Tolosa-Hunt syndrome.
hypophysitis such as suprasellar germinoma or craniopha- 2. Other types of primary pituitary hyphophysitis are granu-
ryngioma. Evaluate associated findings involving the sella lomatous, xanthomatous, mixed forms (lymphogranulo-
turcica and parasellar structures such as cavernous sinus matous and xanthogranulomatous), necrotizing, and IgG4
enlargement or meningeal thickening. plasmacytic hypophysitis.

Pattern of pituitary involvement (adenohypophysitis, infun-
dibuloneurohypophysitis, or infundibulopanhypophysitis)
that would support and correlate the clinical presentation

Case
FINDINGS Figure 220-1. Bone scintigraphy. There is three phases, each with a distinct imaging appearance. The
abnormal tracer uptake in the calvarium, skull base, and pel- lytic phase, characterized by predominant osteolytic activity,
vis (arrows). Figure 220-2. NCCT head bone window. There results in lucent lesions on plain film and CT. In the skull,
are cortical thickening, abnormal bone matrix, and patchy these can be large and geographic, often referred to as osteo-
sclerosis (hyperdensity) (arrows). Figure 220-3. Axial T2WI porosis circumscripta. A mixed phase follows which reflects
through the cerebral hemispheres. There are diffuse thicken- osteoblastic repair. Cortical and trabecular thickening are typi-
ing and heterogeneous marrow intensity of the cranial vault cal of this stage. Finally, a blastic phase ensues, with devel-
(arrows). Figure 220-4. Axial post-contrast T1WI through opment of sclerotic foci, indicating an excessive osteoblastic
the cranial vault. There are marrow replacement, expansion response. In the skull, this can result in the pathognomonic
of the diploic space, and patchy enhancement (arrows). cotton-wool appearance whereby fluffy-appearing sclerotic
lesions develop in areas of prior osteoporosis circumscripta
DIFFERENTIAL DIAGNOSISPaget disease, metastases, resulting in a mixed-density pattern on CT. The diploic space
fibrous dysplasia. of the skull may widen with considerable deformity of the
bony anatomy. MRI T1WI shows areas of hypointensity
DIAGNOSIS Paget disease. within thickened cranial vault or skull base with heterogeneous
contrast enhancement. It is often heterogeneous intensity on
DISCUSSION Paget disease is a common metabolic con- T2WI. Complications include bony weakening with the poten-
dition of the bones which leads to excessive remodeling and tial for fracture, accelerated arthritis, neurologic symptoms
deformity. Any bone can be involved, though the skull, spine, due to narrowing of the skull base and spinal foramina, and
and pelvis are common sites. The disease progresses through rarely, sarcomatous transformation and giant cell formation.
476

Case 220 477
Paget is a disease of the elderly with a slight male pre- n arrowing. Recognize the characteristic appearance of Paget
ponderance. It is uncommon in people of African and Asian disease so as to distinguish it from other more ominous or
descent. Paget is due to replacement of bone marrow and malignant conditions.
bone thickening by fibrovascular tissue which subsequently
progresses to calcification and sclerosis. Clinical presenta- What the Treating Physician Needs to Know
tion when symptomatic may include macrocrania, asym- Paget disease is a common benign condition of the bones
metric deformity of the skull, cranial neuropathy, and pain which, in most cases, can be diagnosed with certainty
particularly when complicated by fracture, giant cell tumor based on characteristic imaging features
formation, or sarcomatous degeneration. There is usu- Are there any complications?
ally elevated serum alkaline phosphatase. Treatment is by What is the extent of the disease?
bisphosphonates.
Answers
Questions for Further Thought 1. Plain films and/or bone scintigraphy are usually adequate
1. What sort of imaging is most appropriate in the diagnosis to make the diagnosis. When the imaging features are not
and follow-up of Paget disease? sufficiently specific, for example, with an incidental skull
2. How would sarcomatous transformation appear on abnormality on head CT, consider imaging the pelvis and
imaging? long bones. These are frequent sites of concomitant dis-
ease where the features may be more typical.
Reporting Responsibilities 2. Sarcomatous transformation should be highly suspected
Routine reporting is sufficient unless there are complica- when areas of new lytic change develop in Pagetoid bone,
tions such as fractures, sarcomatous change, and foraminal especially if a soft tissue process can be detected.

Case
221 CLINICAL HISTORY 26-year-old female fell ill at work with left-sided
predominantly lower extremity weakness, nausea, and vomiting.
FINDINGS Figure 221-1. Axial DWI through bilateral cen- h emisphere sparing the posterior aspect which is supplied by
trum semiovale. There is a parasagittal right frontoparietal the posterior cerebral artery. The location of this infarct is
hyperintensity with low ADC (not shown) consistent with consistent with ACA territorial infarct. The corpus callosum
acute/subacute ischemic infarct. Figure 221-2. Axial T2WI with the exception of the splenium is also supplied by the
through the same level. There is a confluent mainly cortical ACA. The recurrent artery of Heubner, a branch of the ACA,
right frontoparietal parasagittal hyperintensity (transverse supplies part of the basal ganglia. Acute stroke imaging is
arrows). Multifocal bilateral centrum semiovale hyperinten- not complete without evaluation of the vascular system. This
sities are present (vertical arrows). was indeed done in this patient, and the MRA showed occlu-
sion of the right A2 along with extensive vascular changes
DIFFERENTIAL DIAGNOSIS N/A. consistent with vasculopathy. With the availability of acute
stroke therapies, physiologic imaging to evaluate salvage-
DIAGNOSIS Right anterior cerebral artery (ACA) territory able area or the penumbra is increasingly performed with
acute/subacute ischemic infarct. MR or CT perfusion.
The ACA territory ischemic infarct is rare and is the
DISCUSSION The classical DWI finding of acute isch- least common of the three major cerebral territorial isch-
emic infarct is the restricted diffusion which is hyperin- emic infarcts representing 1.3% of patients with ischemic
tense on DWI with hypointensity on ADC. MRI particularly strokes in one particular series. The main risk factors of ACA
DWI is the ideal modality of choice for initial evaluation of infarcts include hypertension, diabetes mellitus, hypercholes-
stroke. Nationwide, CT continues to be the examination of terolemia, cigarette smoking, atrial fibrillation, and myocar-
choice because of its ready availability, fast and immedi- dial infarct; not too different from other territorial infarcts.
ate exclusion of stroke mimics and hemorrhage that allow This patient had extensive vasculopathy of unknown cause
prompt acute thrombolytic therapy. The location of the affecting both large and small vessels. Additional findings
hyperintensity in this patient indicates the vascular territory of extensive white matter (WM) changes were present in
involved. The ACA supplies the parasagittal frontal lobe Figure 221-2. Clinical presentation of ACA territorial infarct
virtually involving the entire medial surface of the cerebral includes mutism, confusion, and hemiparesis predominantly
478

Case 221 479
involving the lower limb. Management of ischemic infarcts Underlying vascular pathology may determine manage-
includes acute therapies such as recombinant tissue plasmin- ment strategy. CTA or MRA is useful for evaluation of the
ogen activator (rtPA), endovascular therapies, and rehabilita- vascular system
tion with preventive measures to combat the risk factors. The
outcome of major stokes is usually poor. Answer
1. The DWI hyperintensity with accompanying hypointen-
Question for Further Thought sity on ADC is consistent with restricted diffusion. Water
1. What is the physiologic explanation of the DWI hyperin- molecules undergo Brownian motion (diffusion) in nor-
tensity? mal physiologic state. Diffusion is free as in cerebrospinal
fluid (CSF) and somewhat restricted by cell membrane,
Reporting Responsibilities macromolecules, and fibers within cellular structures;
Direct reporting is essential in acute cerebrovascular event. in all directions (isotropic) or in restricted directions
Early treatment in some cases could affect the outcome. (anisotropic). Onset of acute infarction results in failure
Presence of mass effect and hemorrhage should be mentioned. of the cell membrane ion pump, and excess sodium enters
the cell. This results in a net movement of water from
What the Treating Physician Needs to Know the extracellular to intracellular compartment with cyto-
Location and size of infarct toxic edema or cellular swelling. This further restriction
Complications such as hemorrhage, mass effect, and of water molecular diffusion is captured in DWI as hyper-
herniations intensity with corresponding hypointensity on ADC map.

222
CLINICAL HISTORY 71-year-old female with long-standing progressive
Case history of mild cognitive impairment, slurred speech, frequent falls,

motor weakness, tremor, downward gaze palsy with subsequent complete
ophthalmoplegia, and dementia.
DIAGNOSIS Progressive supranuclear palsy (PSP).
DISCUSSION MRI is the preferred imaging modality for

evaluation of PSP. The classical MRI finding is that of the
penguin or hummingbird sign. There is atrophy of mid-
brains tegmentum with upper anterior surface concavity (thin
sharp beak and head of the hummingbird), relatively preserved
but still appreciable volume loss of the pons (body of the hum-
mingbird) and cerebellum (wings of the hummingbird).
PSP is a neurodegenerative disorder evaluated within
Parkinson-plus syndromes, characterized by supranuclear
ophthalmoplegia primarily affecting vertical gaze, postural
instability secondary to dystonic rigidity of the neck and upper
trunk, mild dementia, and pseudobulbar palsy. Pseudobulbar
Figure 222-3 palsy presents with inability to control swallowing and chew-
ing, slurred speech, and inappropriate emotional outbursts.
It is an upper motor neuron dysfunction of brainstem upper
FINDINGS Figure 222-1. Sagittal T1 MPRAGE. There is motor nuclei and resultant corticobulbar neuronal degenera-
moderate midbrain/tegmental volume loss (vertical arrow), tion. Its pathologic substrate is neuronal loss and gliosis with
thinned tectal plate (posterior transverse arrow), mild pontine atrophy of the midbrain, subthalamic nucleus, and thalamus.
volume loss (flattening of the basis pontis-chevron) known There may be pallidum and frontal lobe atrophy. PSP is the
as hummingbird sign. Figure 222-2. Axial MPRAGE second most common neurodegenerative cause of parkinson-
through the midbrain. There is widening of the interpedun- ism. However, patients do not respond to dopamine or anti-
cular cistern (vertical arrow) and concave appearance of the cholinergic therapy as the Parkinson disease.
lateral midbrain (transverse arrows). Figure 222-3. Coronal
MPRAGE through the brainstem. There is loss of volume of Question for Further Thought
the superior cerebellar peduncle (arrows). 1. What is the impact on differentiating PSP from Parkinson
disease?
DIFFERENTIAL DIAGNOSISParkinson disease, Par
kinson variant of multi system atrophy, dementia with Lewy Reporting Responsibilities
bodies, corticobasal degeneration, progressive supranuclear Routine reporting is sufficient. The diagnosis is clinical
palsy (PSP). with imaging support. Secondary findings such as midbrain
480

Case 222 481
infarcts, masses, or MRI signal alterations with subtle mid- Answer

brain tegmental atrophy may mislead or delay the diagnosis 1. Imaging clues should be carefully evaluated given the
of PSP. fact that it is critical differentiating parkinsonism-plus
syndromes from Parkinson disease. In parkinsonism-plus
What the Treating Physician Needs to Know syndromes such as PSP, life expectancy is much lower
Imaging is exclusionary; concomitant intracranial patholo- and treatments such as levodopa and deep brain stimula-
gies should be carefully evaluated tion are usually ineffective.

Case
223 CLINICAL HISTORY 58-year-old female who had a severe headache
and collapsed at home.
FINDINGS Figure 223-1. Axial MRI FLAIR through the is an ovoid contrast-enhancing focus measuring 10.4 mm
suprasellar cistern. There is diffuse hyperintensity through- 6.24mm on the left in the suprasellar cistern. Figure 223-3.
out the visualized SASsuprasellar cisterns (anterior verti- 3D TOF MRA source image through the suprasellar cistern.
cal arrows), bilateral sylvian fissures (transverse arrows), and There is an ovoid outpouch measuring 11.6 mm from the neck
the quadrigeminal/ambient cisterns (posterior vertical arrow) to the dome and 3.25 mm at the neck from the left internal
consistent with acute subarachnoid hemorrhage (SAH). A CT carotid artery (ICA) projecting posteriorly between the basilar
scan done earlier on in the day shows basal and convexity artery and the left superior cerebellar artery (arrow) consistent
SAH and IVH (intraventricular hemorrhage). Figure 223-2. with an aneurysm at the level of the posterior communicat-
Post-contrast T1WI through the suprasellar cistern. There ing artery (P-Com). Figure 223-4. 3D TOF MRA 3D volume
482

Case 223 483
rendering. The aneurysm (arrows) is pear shaped and projects p rojection, and its relationship to the surrounding structures
posteriorly from the left ICA. should be provided. Presence or otherwise of complications
such as IVH, hydrocephalus, cerebral vasospasm, and brain
DIFFERENTIAL DIAGNOSISICA anterior choroidal swelling or ischemic lesions should be mentioned.
artery aneurysm, posterior communicating artery aneurysm
(P-Com-A). What the Treating Physician Needs to Know
Extent of SAH
DIAGNOSIS P-Com-A. Location and direction of projection and the relevant mea-
surements of the aneurysm. If more than one aneurysm,
DISCUSSION P-Com-A represents about 30% to 35% of which one has bled?
all intracranial saccular aneurysms. P-Com-A arises from Presence of complications
the ICA at the level of the P-Com. It is an outpouch from
the ICA usually projecting posteriorly but could project lat- Answers
erally, inferiorly, or superiorly. A large P-Com-A could be 1. The essence of a grading system is to provide uniformity
seen as a focal hyperdensity posteriorly within the suprasel- in measuring the severity of SAH and its effect on the out-
lar cistern on CT. As in this case, it could present as a con- come of treatment. As expected, a good grade results in a
trast-enhancing mass in the suprasellar cistern with the MRA good outcome, while a bad grade result in bad outcome.
demonstrating the neck, dome, and projection of the aneu- There are several clinical grading systems. The two most
rysm. Its relationship to the surrounding structures is well frequently used grading systems are the World Federation
demonstrated. Smaller aneurysms may not be visualized on of Neurological Surgeons (WFNS) grading system pro-
CT and MRI until the CTA or MRA is done. The aneurysm posed in 1998 and the Hunt and Hess grading system
usually arises from the region of the neck of the P-Com when proposed in 1968. The WFNS has five grades predicated
it is present. An infundibulum of the P-Com should be dif- on the Glasgow Coma Scale (GCS) and motor deficit.
ferentiated from an aneurysm. An infundibulum is a funnel- Grade1 is GCS score 15 without motor deficit. Grade 2
shaped dilation of the origin of the P-Com less than 3 mm in is GCS score 13 to 14 without motor deficit. Grade 3 is
diameter with the artery emanating as the stem of the funnel. GCS score 13 to 14, with motor deficit. Grade 4 is GCS
CTA and MRA are equally effective in imaging aneurysm score7 to 12, with or without motor deficit. Grade 5 is
3mm or larger. The source images combined with maximum GCS score3 to 6, with or without motor deficit.
intensity projection or volume-rendered images particularly The Hunt and Hess grading system has six grades
in the sagittal plane best depict the P-Com-A to best advan- based on surgical risk and outcome assessment at pre-
tage. Positive identification of the anterior choroidal artery sentation. Grade 0 is unruptured aneurysm without
and the P-Com is necessary in determining the ICA level of symptoms and has a mortality rate of 0%. Grade 1 is
origin of the aneurysm. Parenchymal hematoma, vasospasm, asymptomatic or mild headache and slight nuchal rigid-
ischemic lesions, brain swelling, IVH, and hydrocephalus ity and has a mortality rate of 1%. Grade 2 is moderate-
are known complications. to-severe headache, stiff neck, without neurologic deficit
P-Com-A is more common in women than men with a except cranial nerve palsy, and has a mortality rate of
female to male ratio of 4:1. It occurs in all ages from the 5%. Grade 3 is drowsy or confused, mild focal neuro-
second decade upward but more commonly in midlife and logic deficit, and has a mortality rate of 19%. Grade 4 is
the elderly. Like all aneurysms, most P-Com-A are silent. stupor, moderate, or severe hemiparesis and has a mor-
Symptomatic P-Com-A may present with headache, vision tality rate of 42%. Grade 5 is deep coma, decerebrate
loss, third nerve palsy, and SAH and associated symptoms rigidity, and has a mortality rate of 77%. The grade is
and signs of raised intracranial pressure. Third nerve palsy advanced one level for the presence of serious systemic
occurs in up to 60% of P-Com-A and could present before, disease (hypertension, diabetes, severe arteriosclerosis,
during, and after SAH. Appropriate grading provides the risk and chronic pulmonary disease) or vasospasm on angi-
assessment during initial presentation. Treatment options ography.
include direct surgical clipping and endovascular coiling. 2. The Fisher scale is a CT grading system proposed in 1980
predicated on vasospasm risk based on the amount of
Questions for Further Thought SAH on the initial CT. It has four grades. Group 1: no
1. How is SAH graded clinically? blood detected. Group 2: diffuse deposition or thin layer
2. How is SAH graded by CT? with all vertical layers of blood (in interhemispheric fis-
sure, insular cistern, or ambient cistern) less than 1 mm
Reporting Responsibilities thick. Group 3: localized clots and/or vertical layer of
Like all aneurysms and SAH, direct reporting is neces- blood 1 mm or more in thickness. Group 4: intracerebral
sary. The usual measurements of the aneurysm, direction of or intraventricular clots with diffuse or no SAH.

Case
224 CLINICAL HISTORY 61-year-old female with headache, nausea, and
visual complaints. Visual field examination revealed a partial left inferior
quadrantanopsia.
484

Case 224 485
or lactate with elevated choline (Cho) decrease N-acetyl-l-

aspartate (NAA) and lower creatine (Cr). An abscess tends
to restrict diffusion centrally with smooth thin ring enhance-
ment. Only a minority of metastatic lesions and GB will
have centrally restricted diffusion. Metastasis on the other
hand with irregular ring contrast enhancement and sur-
rounding edema may be difficult to distinguish from GB.
Multifocal metastasis could mimic multifocal GB which
occurs in less than 10% of cases. The enhancement pattern
of tumefactive demyelination is that of incomplete or open
ring enhancement with limited surrounding edema and low
rCBV. Lymphoma is usually a solid-enhancing lesion and
is often adjacent to CSF spaces. An open ring appearance in
immunocompetent patients with a thick rind can occur rarely
in lymphoma. Lymphoma may show restricted diffusion due
to its high cellularity and by perfusion demonstrates contrast
Figure 224-5
leakage rather than elevatedrCBV.
GB, a WHO IV astrocytoma, is the most common pri-
mary intraparenchymal brain tumor. GB can be seen in all
FINDINGS Figure 224-1. Axial FLAIR through the age groups but has a peak incidence in the seventh and eighth
trigones. There are two masses (vertical arrows) with het- decades and rarely occurs in children. There is a slightly
erogeneous intensity in the right parietal occipital lobes greater occurrence in men. The location of GB determines
with surrounding edema and mass effect on the right trigone the mode of presentation. Headache, neurologic deficit, con-
(transverse arrow). Figure 224-2. Axial ADC map through fusion, and visual impairment are but some of the presenta-
the masses. There is low ADC in the masses (arrows). tions. GB demonstrates variable cytology, ranging from cells
Figure 224-3. Axial post-contrast T1WI. There is a thick with clear astrocytic quality to undifferentiated, largely glial
irregular ring contrast enhancement with a hypointense core fibrillary acidic protein (GFAP)-negative cells. Vascular
in each of the lesions (arrows). Figure 224-4. Axial perfu- endothelial proliferation or necrosis, with or without pseu-
sion relative Cerebral Blood Volume (rCBV) map. There is dopalisading, is prerequisite for the diagnosis. Common GB
peripheral increased blood volume in the masses (arrows). variant includes small-cell and giant-cell types. Less com-
Figure 224-5. Low-power photomicrograph shows mark- mon variant includes epithelioid, rhabdoid, and granular
edly cellular tumor with pseudopalisaded necrosis and vas- cells. Chromosomal abnormalities are quite common and
cular endothelial proliferation (H&E stain). include loss of chromosome 10 and less frequently gain of
chromosome 7. Molecular abnormalities include deletions
DIFFERENTIAL DIAGNOSIS Glioblastoma (GB), abscess, and mutations of p16 and Phosphatase and Tensin Homolog
metastasis, tumefactive demyelination, lymphoma. (PTEN), Loss Of Heterozygosity (LOH) of 17p, p53 muta-
tions and amplification of Mouse double minute 2 homolog
DIAGNOSIS Glioblastoma (GB). (MDM2) and Epidermal Growth Factor Receptor (EGFR).
Treatment may include multimodality platform of a com-
DISCUSSION GB tends to be hypodense on NCCT with bination of surgery, chemotherapy, and radiation therapy.
heterogeneous mostly irregular contrast ring enhancement Unfavorable prognostic factors include older age, poor
and surrounding hypodense vasogenic edema. MRI is the Karnofsky performance scores, and incomplete resections at
imaging modality of choice for evaluation and follow-up of surgery. The expected survival is short with a minority of
the patient with GB. GB is a necrotic mass with a variable patients surviving a year after diagnosis.
size and surrounding vasogenic edema. GB is usually isoin-
tense to hypointense on T1WI. The solid component could
be heterogeneous in signal intensity on FLAIR and T2WI, Questions for Further Thought
while the necrotic portion is hyperintense. There is varying 1. How does O6-methylguanine-DNA-methyltransferase
amount of surrounding T2 hyperintensity infiltrating the sub- (MGMT) methylation relate to survival in GB?
cortical WM or adjacent GM which could represent a com- 2. When does pseudoprogression usually occur in patients
bination of vasogenic edema and infiltrating tumor. Areas of treated with radiation therapy and temozolamide?
susceptibility changes on GRE suggest either blood products
or calcification. Hemorrhage is present in less than 10% and Reporting Responsibilities
calcification is infrequent. Post-contrast T1WI shows irregu- Direct reporting is often necessary in tumors. Presence of
lar somewhat nodular ring contrast enhancement. The solid significant mass effect or herniations makes direct reporting
component of GB in general demonstrates elevated rCBV more urgent. Location and number of lesions should be tabu-
and elevated ADC values except where hypercellularity lated as this may affect how the patient is treated. Presence
exists. MR spectroscopy demonstrates elevated lipids and/ and extent of infiltrative edema or lesion in the contralateral

486 Case 224
hemisphere is important to mention. Functional MRI may be Answers

useful before resection if lesion is situated in eloquent areas. 1. MGMT methylation in GB has been associated in mul-
tiple studies with increased survival, and these patients
What the Treating Physician Needs to Know benefit more from temozolamide. IDH1 mutations are
Location and number of lesions associated with significantly longer survival.
Possibility of benign differential diagnosis and how to dif- 2. Pseudoprogression occurs most frequently in the first
ferentiate them 3 months after radiation therapy and concurrent temo-
Significant mass effect or herniations zolamide. Pseudoprogression occurs very frequently in
Presence of infiltrative lesions in the other hemisphere patients with MGMT promoter methylated tumors.

Case
225 CLINICAL HISTORY Newborn with facial deformity and suspected raised
intracranial pressure.
FINDINGS Figure 225-1. NCCT through the thalami. alformations ranging from cyclopia with one eye in the
m
There is fusion of the thalami (arrows) and frontal lobes. The middle of the forehead, varying degrees of hypothelorism,
falx and interhemispheric fissure are absent. There is tremen- presence of proboscis, deformity of the nose, and midline
dous dilation of the lateral ventricles. Figure 225-2. Axial clefts. Microcephaly is the norm and presence of enlarged
NCCT through the body of the lateral ventricles. There is a head in alobar HPE may indicate hydrocephalus or a dorsal
single dilated lateral ventricle covered by a band of brain tis- cyst as in this case. Midline structures are usually well formed
sue anteriorly due to fusion of the two hemispheres without in hydrocephalus. In hydranencephaly the falx cerebri and
formation of midline structures such as the corpus callosum, interhemispheric fissure are present, but the frontal lobes and
interhemispheric fissure, the falx, and the septum pellu- most of the cerebral hemispheres are not developed. There is
cidum. A large dorsal cyst (star) is present. separation of the thalami and rudimentary posterior cerebral
hemispheres. Porencephaly is due to a destructive cyst that
DIFFERENTIAL DIAGNOSIS Holoprosencephaly (HPE), communicates with the cerebrospinal fluid (CSF) space.
hydranencephaly, severe hydrocephalus, porencephaly. HPE occurs soon after conception at about 5-week fetal
life. It has a prevalence of 1 in 10,000 to 1 in 20,000 births.
DIAGNOSIS Alobar HPE. The most severe cases are either spontaneously aborted or
die before birth; hence, its true prevalence is higher in the
DISCUSSION Both CT and MRI can demonstrate the first trimester. The cause of HPE is unknown but has asso-
changes of HPE, but MRI is the modality of choice in view ciation with environmental factors, some medications such
of its multiplanar capability and the many sequences that as aspirin, sporadic chromosomal abnormalities, mutations,
can demonstrate the various structures succinctly. HPE is a and syndromic disorders. HPE is inherited with a familial
spectrum of anomalies characterized by lack of formation risk of 5% to 15%. There are several HPE genes that can be
of midline structures with variable interhemispheric fusion. tested for. HPE is associated with trisomy 13 and 18. Clinical
Perinatal ultrasonography and/or MRI can be used to iden- presentation could range from very mild to severe. Severe
tify most cases of the severe forms of the malformation in seizure disorders, pituitary and hypothalamic dysfunctions,
utero. Findings in alobar HPE include lack of division of the autonomic dysfunction, and other life-threatening condi-
bilateral cerebral hemispheres, forming a horseshoe around tions may sometimes be associated with HPE. The severity
a single ventricle, absent interhemispheric fissure, falx, sep- of associated mental or developmental delay depends on the
tum pellucidum and corpus callosum, fusion of the thalami, severity of HPE. Genetic counseling and risk assessment
and absent olfactory bulb. Other findings may include facial depend on the determination of the specific cause of HPE in
487

488 Case 225
an individual. There is no known cure. Treatment is symp- Answers

tomatic and supportive. 1. HPE appears in around the fourth to fifth week of pregnancy
during the stage of ventral induction with nonformation of
Questions for Further Thought individual hemispheres. The severe ones could easily be
1. How early can HPE be diagnosed in utero? identified by ultrasound thereafter. Milder forms may
2. How is HPE classified? require MRI. Once diagnosed, appropriate counseling can
be performed. This may aid management of the pregnancy.
2. HPE exists in a spectrum. It is subdivided into (1) alo-
bar HPE which is complete absence of midline forebrain
Routine reporting is sufficient unless there is associated
division resulting in a monoventricle and fused cerebral
hydrocephalus or dorsal cyst resulting in raised intracranial
hemispheres, lack of formation of the falx, and micro-
pressure. It is important to be able to identify the mimics.
cephaly, (2) semilobar HPE which is the intermediate
Your checklist should include presence of a univentricle,
form with partial separation of the hemispheres posteri-
absence of the corpus callosum and septum pellucidum, falx
orly but essentially a monoventricle. It is also invariably
cerebri, olfactory bulbs, and basal ganglia and hemispheric
associated with microcephaly, (3) lobar HPE which is the
fusion.
mildest form of HPE with separation of the hemispheres
except rostrally. The frontal horns are fused. Septum pel-
What the Treating Physician Needs to Know lucidum is absent, and there is variable formation of the
Extent of hemispheric and deep gray nuclei fusion which corpus callosum. The patient is usually normocephalic,
coincides with severity of mental or developmental (4) middle interhemispheric HPE or syntelencephaly
deficiency where the anterior and posterior cerebral hemispheres
Presence of dorsal cyst or hydrocephalus are separated with fusion of the midsection of the brain
Presence of migrational malformations around the motor and sensory strips (perirolandic fusion).

Case
226 CLINICAL HISTORY 1-day-old baby with in-utero diagnosis of a complex
left temporal lobe mass.
Figure 226-3
Figure 226-4
FINDINGS Case previously published BJR 2006; 79: lobe (transverse arrows). There is mild mass effect on
e140-e144. the midbrain. This tangle of blood vessels drains into the
Figure 226-1. Axial FLAIR through the midbrain. There large torcula (vertical arrow). There is hyperintense brain
is a large collection of tubular signal voids measuring parenchyma interspersed within the mass presumably glio-
about 5cm in maximum dimension in the left temporal sis. Figure 226-2. Axial T1WI through the temporal lobes.
489

490 Case 226
There is a large, ovoid, and heterogeneous left temporal and venous outflow occlusion, embolic phenomenon from
lobe well-defined mass (transverse arrows). The bilateral arterial feeders or aneurysms, and hypercoagulable states.
transverse sinuses are large (vertical arrows). Figures 226-3. There is no evidence of significant encephalomalacia in this
Follow-up axial T2WI through the midbrain at age of patient to suggest underlying significant prior hematoma,
4months. There is complete disappearance of the left tem- and there was no significant clinical event between the two
poral lobe mass with widening of the cerebrospinal fluid studies to suggest seizure activities. The presence of a left
(CSF) spaces (black stars) surrounding the left temporal frontal subdural collection may suggest a prior subdural
lobe consistent with volume loss. Figure226-4. Axial T2WI hematoma, but it may well represent an effusion complicat-
through the lateral ventricles. There is a crescentic hyperin- ing the volume loss.
tense left frontal extraaxial collection (black arrow).
DIFFERENTIAL DIAGNOSIS Dural arteriovenous fistula 1. Can a thrombosed AVM reopen?
(DAVF), arteriovenous malformation (AVM), glioma.
DIAGNOSIS Spontaneous thrombosis of AVM. Discovery of an AVM and follow-up evaluation findings
deserve direct reporting because of possible catastrophic
DISCUSSION An AVM is composed of a tangle of ves- complications. Spontaneous thrombosis which occurs in 1%
sels called the nidus with one or more arterial feeders and to 3% of all AVMs is a rarity and deserves immediate report-
one or more venous outflow. The nidus is what differenti- ing to prevent unnecessary invasive studies.
ates an AVM from the other vascular malformations par-
ticularly DAVF. One of the less well-known aspects of What the Treating Physician Needs to Know
AVM is why a small percentage can disappear completely Location, size, arterial feeders, venous outflow, and asso-
without a trace. AVM is described as congenital, and we are ciated parenchymal changes
not sure whether they grow once formed but spontaneous Any complications following the spontaneous thrombosis
regression is a well-known rare feature. Clinical presenta-
tions of AVM include headache, seizures, focal neurologic Answer
deficit, and altered mental status when complicated by hem- 1. Yes, there has been a reported recanalization of a previously
orrhage. The reasons for complete thrombosis are not well asymptomatic thrombosed AVM. Because of this, it is rec-
understood. These have included seizure activity, parenchy- ommended that such thrombosed AVM should be followed
mal and subarachnoid hemorrhages, arterial feeder stenosis, up for at least 3 years to ensure permanent thrombosis.

Case
227 CLINICAL HISTORY Patient with known liver insufficiency who presents
to the emergency department with a 1-week history of progressive diffuse
encephalopathy.
FINDINGS Figure 227-1. Axial FLAIR through the tempo-

ral lobes. There is bilateral symmetrical mesiotemporal lobe
hyperintensity (arrows). Figure 227-2. Axial FLAIR through
the sylvian fissures. There is bilateral symmetrical insular
cortex hyperintensity (arrows). Figure 227-3. Axial T1WI
through the basal ganglia. There is bilateral globus pal-
lidus hyperintensity (arrows) often seen in chronic hepatic
encephalopathy (HE).
DIFFERENTIAL DIAGNOSIS Hypoxic-ischemic enceph-

alopathy, Wilson disease, cholestatic disorders, paren-
teral hyperalimentation, viral encephalitis, and sporadic
Creutzfeldt-Jacob disease (CJD), HE.
DIAGNOSIS he.
DISCUSSION Imaging findings of HE correlate directly

with clinical severity. In the chronic stage MRI demonstrates
bilateral and symmetrical T1-weighted hyperintensity in the
globus pallidus, adenohypophysis, and mesencephalon. Acute
HE manifests as T2 hyperintensity in the cortex with sparing
of the perirolandic and occipital regions. The hippocampi are
commonly affected. White matter (WM) abnormalities are
not uncommon and may also resolve or diminish in size when
Figure 227-3 the acute period is over. Proton MRS reveals an increase in
491

492 Case 227
the glutamine and glutamate peaks and a reduction in the that do not subside after treatment. Minimal HE is generally
myoinositol and choline peaks. After the liver dysfunction or subclincal.
when precipitating factor has been corrected, the MRS abnor-
malities are the first to disappear, while the pallidal hyperin- Question for Further Thought
tensities on T1WI tend to disappear 3 months to 1 year later. 1. What is the West Haven criteria in HE?
The T1-weighted pallidal hyperintensity may be present in
parenteral hyperalimentation or Wilson disease but would be Reporting Responsibilities
unusual in the other differential diagnoses. The mesiotempo- This diagnosis requires direct reporting particularly in view
ral and insular changes are present in limbic and Herpes sim- of the differential diagnostic possibilities. Cerebral edema
plex virus (HSV) encephalitis. DWI cortical hyperintensities and signs of increased intracranial pressure are present in the
are often present in sporadic CJD and in HIE. episodic HE. Describe the normalization of the imaging find-
HE represents a wide continuum of temporal and revers- ings on the follow-up of treated patients.
ible neuropshychiatric and motor disorders that develop in
the setting of hepatic failure or porto-systemic shunting.
The manifestations extend from mild cognitive impairment
to coma and from rigidity to extrapyramidal syndrome. Generally the diagnosis of HE is based on clinical findings,
Neurotoxic substances such as manganese and ammonia, so physicians need to exclude other neurologic diseases
which are normally metabolized by the liver accumulate in like an underlying evolving vascular dementia or acute
blood when there is liver dysfunction and cross the blood viral encephalitis
brain barrier. The presence of these substances produces Is it safe to perform a lumbar puncture (LP)? The diagno-
intracellular edema, selective neuronal loss, and reactive sis of encephalitis may be aided by LP
gliosis predominantly in the globus pallidus but also in the
substantia nigra reticulata and neostriatum. HE can be classi- Answer
fied as episodic, chronic, or minimal. Episodic HE develops 1. The West Haven criteria is a four grade semi quantitative
in a short period of time and varies in severity. Chronic HE grading of mental status, contemplating the level of auton-
is a more regular and less severe process that can also present omy, changes in consciousness, dependence to therapy,
as relapsing acute episodes or as persistent manifestations intellectual function, and behavior.

Case
228 CLINICAL HISTORY Young African-American female presenting
with hypopituitarism and visual difficulties.
p ost-contrast T1WI in the companion case. There is homoge-

neous enhancement of the enlarged pituitary gland (arrow).
DIFFERENTIAL DIAGNOSISMetastases, meningitis,

meningioma, tuberculosis, lymphoma, leukemia, sarcoid-
osis, and histiocytosis. When the brain is affected, mul-
tiple sclerosis, Lyme disease, and vasculitis should be
considered.
DIAGNOSIS Pituitary axis neurosarcoidosis.
DISCUSSION In the central nervous system (CNS)

and spine, sarcoidosis typically displays a thickening and
enhancement of the leptomeninges, particularly at the skull
base, even though sarcoidosis may involve bone, dura
mater, nerve roots, leptomeninges, and parenchyma. When
affecting the sella region, leptomeningeal thickening and
enhancement on contrast-enhanced T1WI are seen around
the hypothalamus and pituitary infundibulum. Involvement
of only the pituitary gland has been rarely reported, with
nonspecific findings of a cystic-enhancing sellar mass with
Figure 228-3 extension toward the suprasellar space. In the brain, white
matter (WM) lesions that are nonspecific but may simu-
late multiple sclerosis (MS) and show variable contrast
FINDINGS Figure 228-1. Sagittal MR post-contrast T1WI. enhancement may be seen.
There is diffusely enhancing infiltrative lesion in the pitu- Sarcoidosis is a multisystem granulomatous disease of
itary gland, stalk, and suprasellar region (arrow). Figure 228- unknown cause, more frequent in females, African-Americans,
2. Sagittal non-contrast T1WI in a companion case. There is and Northern Europeans, affecting the CNS in up to one-
an enlarged pituitary gland (arrow). Figure 228-3. Coronal fourth of the patients with the systemic disease. Although
493

494 Case 228
s ymptomatic and isolated CNS involvement is fairly uncom- Question for Further Thought
mon, imaging evidence of intracranial disease is relatively 1. What syndrome characterizes an acute presentation of
frequent (about10%) in patients with systemic disease. sarcoidosis?
Headaches, seizures, signs of meningeal irritation, cranial
nerve deficits especially facial nerve paralysis and vision loss, Reporting Responsibilities
as well as other signs and symptoms similar to those of mul- Routine reporting is sufficient in this nonacute situation.
tiple sclerosis such as weakness, paresis, paresthesia, diplopia, Describe and identify pituitary and associated CNS abnor-
and dysarthria are frequent. A clinical picture of endocrine malities that could steer the differential diagnosis toward
dysfunction is seen when the hypothalamus or pituitary gland sarcoidosis.
is affected, including any combination of obesity, psychosis,
diabetes insipidus, amenorrhea, hypoglycemia, or other sig- What the Treating Physician Needs to Know
nificant pituitary insufficiency. The definite diagnosis is made Location of lesion and extent of disease
with a biopsy, but in many this procedure is not possible and Other associated parenchymal or extraaxial findings such
in absence of histologic proof of systemic disease, the prob- as WM lesions and patchy or leptomeningeal enhancement
able diagnosis is reached by two or more imaging and clinical elsewhere
findings (chest radiograph, gallium scan, and elevated serum Compression of critical structures
angiotensin-converting enzyme levels).
Management is not well established, but corticosteroids Answer
and other immunosuppressants like methotrexate are usually 1. Lfgren syndrome is an acute presentation of sarcoidosis
administered to treat symptomatic patients. CNS and spinal that occurs in 9% to 34% of patients, which consists in
involvement carries a poor prognosis. arthritis, erythema nodosum, and bilateral hilar adenopathy.

Case
229 CLINICAL HISTORY 14-year-old female with 1-year history of
worsening headache.
Figure 229-1
Figure 229-2
FINDINGS Figures 229-1 and 229-2. Axial and reformat- DIFFERENTIAL DIAGNOSISOsteoblastic metastasis,
ted sagittal NCCT through the orbits. There is expansion and fibrous dysplasia (FD), Paget disease, intraosseous menin-
sclerotic (ground-glass) appearance of the left greater wing gioma.
of sphenoid bone (stars). There is abrupt sharp transition
between the lesion and surrounding bone (arrows). There DIAGNOSIS Fibrous dysplasia (FD).
is mild encroachment on the left orbit with mild proptosis.
Figures229-3 and 229-4. Axial T2WI and T1WI, respec- DISCUSSION The classical imaging findings of FD are
tively, through the lesion. There is homogeneous hypoin- best depicted on the NCCT. Monostotic and polyostotic
tensity of expanded thickened left greater wing of sphenoid forms of the disease exist. The cranial vault and skull base
bone (stars). There is compression of the left lateral rectus are affected in up to 25% of cases, with the monostotic
and narrowing of the left orbital apex. Figure 229-5. Axial being more common. There is expansion of the involved
post-contrast T1WI through the lesion. There is almost bone. It has a sclerotic, most often homogeneous ground-
homogeneous contrast enhancement of the lesion with two glass, appearance both on CT and on plain radiograph. Some
tiny poorly enhancing foci (arrows). hypodense foci could be present. The margins are sharply
495

496 Case 229
syndrome has a predilection for girls. Most are monostotic with

up to 25% polyostotic and found at m ultiplesites. Theunder-
lying pathology is abnormal formation of fibro-osseous tis-
sue resulting in admixture of woven bone and fibrous tissue
and expansion of the bone. The imaging heterogeneity may
be due to elements of fat, cystic degeneration, and calcifica-
tions within the lesion. Clinical presentations depend on loca-
tion. Orbital involvement may result in proptosis. Skull base
involvement may lead to craniopathies. Facial or skull defor-
mity is common in large lesions, while hearing loss and facial
weakness occur in temporal bone involvement. It is generally
painless, but craniopathies may produce pain. There is no spe-
cific treatment. Surgical excision or curettage may result in
recurrence.
Figure 229-5 Question for Further Thought

1. Is there a genetic or syndromic form of FD?
demarcated from surrounding bone. It varies in size rang-
ing from a few centimeters to large lesion that may encom-
Routine reporting is sufficient in most cases in this benign
pass the calvarium and skull base. FD is most common in
bone lesion. However, distortion or compression of criti-
the ethmoid bones, frontal bones, skull base, maxilla, and
cal structures and foramina as in this case requires direct
zygomatic arch but can affect any cranial bones. It is also
reporting.
found all over the skeleton. Itis deforming and can encroach
on structures and foramina, thereby impinging cranial
nerves. MRI usually demonstrates hypointense lesion on all
sequences, sometimes with focal areas of hyperintensity on Location, extent of lesion, and number if more than one
FLAIR. It enhances avidly homogeneously, but heterogene- Presence of critical structures compression or foraminal
ity is not uncommon. When it affects the frontal or sphenoid deformity
bone, FD could encroach on the orbits causing proptosis Certainty of diagnosis
and deform the skull base foramina and fissures impinging
on cranial nerves. FD is glucose avid on FDG-PET. The Answer
sharp margins tend to distinguish FD from its mimics such 1. FD is associated with mutation in the GNAS1 gene,
as osteoblastic metastases, intraosseous meningioma, and which controls skeleton-forming mesenchymal tis-
Paget disease. The cotton-wool appearance of the bone is sue. The McCune-Albright syndrome is polyostotic FD
typical of Paget. The pagetoid FD may resemble Paget! affecting mainly young girls in their first decade of life
Apart from the pagetoid-type FD, the other two types of FD with endocrinopathy resulting in precocious puberty, and
described are the cystic and sclerotic types. skin hyperpigmentation otherwise known as caf-au-lait
FD is a rare disease of the young usually presenting under spots which could be confused with skin changes of
30 years. It has no gender preference; but the McCune-Albright neurofibromatosis.

Case
230 CLINICAL HISTORY 43-year-old female with posttraumatic coma.
FINDINGS Figures 230-1 and 230-2. Axial contiguous space. Pneumocephalus could be due to traumatic disrup-
NCCT through the inferior frontal lobes. There are bilateral tion of the skull, following neurosurgical procedures such
frontal extraaxial hypodensities (air collections) (stars) with as evacuation of subdural hematoma (SDH), external ven-
compression of the frontal lobes. The frontal lobes peak tricular drain placement, tumor removal, or skull base sur-
into the air collections with a midline separation of the fron- gery. Spontaneous tension pneumocephalus has also been
tal lobes (white arrows). There is airfluid level laterally in reported. The Mount Fuji sign is usually not present in non-
the collections (black arrows). Intraventricular hemorrhage tension pneumocephalus. Nontension pneumocephalus is
due to diffuse axonal injury (DAI) is present in the trigones common and associated with surgery, fractures through the
in Figure 230-2. mastoids or paranasal sinuses, and iatrogenic through intra-
venous lines. Sometimes, the source of the pneumocepha-
DIFFERENTIAL DIAGNOSIS N/A. lus may not be found. Nontension pneumocephalus resolves
within the first week in up to 85% of cases.
DIAGNOSIS Pneumocephalus (tension pneumocephalus). Patients with tension pneumocephalus, however, may
present with headache, neurologic deficit, and decreased
DISCUSSION Presence of a large subdural collection of level of consciousness. The correct diagnosis of tension
air over the frontal lobes with compression and separation pneumocephalus has to be made on the basis of not only
of the frontal lobes as in this case has been described as the imaging but also clinical presentation. Treatment is prompt
Mount Fuji sign. It is regarded as a sign of tension pneumo- decompression by a variety of ways. The outcome is usually
cephalus. It is assumed that the air collection is under pres- very good.
sure, hence the capacity to compress and separate the frontal
lobes resulting in the peaking of the frontal lobes into the Question for Further Thought
air collections. A ball-valve mechanism is invoked as the 1. What is the pathogenesis of pneumocephalus from iatro-
mechanism allowing air to get in but not exit the subdural genic intravenous injection of air?
497

498 Case 230

Tension pneumocephalus is an emergency and should be 1. Iatrogenic intravenous pneumocephalus is due to cepha-
reported directly to the referring physician. Presence of lad flow of air via peripherally inserted venous catheter
underlying pathology such as fractures, paranasal sinus dis- through the internal/external jugular veins to the intra-
ease, and evidence of recent surgery should be reported. cranial venous sinuses under certain clinical situations
such as valsalva maneuvers as in coughing, stenotic or
What the Treating Physician Needs to Know occluded brachiocephalic vein, partially obstructed supe-
Amount of air if it could be estimated along with the rior vena cava (SVC), or low flow state as in heart failure.
degree of mass effect Such air bubbles are found in the cavernous sinuses, in
Source of the air if it can be deduced and around the sella turcica, in the superior ophthalmic
Associated primary or secondary injuries veins, behind the clivus, and in the petrosal sinuses.

Case
231 CLINICAL HISTORY 46-year-old male with 4 months history of right eye
vision loss and ptosis. A left groin mass biopsy showed non-Hodgkin diffuse
large B-cell lymphoma (DLBCL).
Figure 231-4
Figure 231-3 FINDINGS Figure 231-1. Coronal T2WI through the chi-
asm. There is enlargement and mild hyperintensity of the
optic chiasm on the right (vertical arrow) which extends on
the other images (not shown) along the right optic nerve into
the optic foramen. There is hypointense thickening of the
right cavernous sinus (transverse arrow). Figure 231-2. Axial
T1WI through the suprasellar cistern. There is hypointensity
of the right uncus extending into the right superior orbital
fissure (arrows). Figure 231-3. Axial DWI through the supra-
sellar cistern. There is mild restricted diffusion in the nodu-
lar right third cranial nerve (arrow). The mass compresses
the right uncus laterally. Figure 231-4. Coronal post-contrast
T1WI through the anterior sella turcica. There is avid nodu-
lar contrast enhancement of the right optic nerve (vertical
arrow) which on the other images (not shown) extends pos-
Figure 231-5 teriorly to the chiasm and anteriorly to the right orbital apex.
499

500 Case 231
There is also avid contrast enhancement of the enlarged right diseases. Single-nodular or multiple-nodular cranial nerves
cavernous sinus (transverse arrow). Figure 231-5. Axial post- involvement may be reminiscent of multiple schwannomas.
contrast T1WI through the suprasellar cistern. There is avid Calvarial, skull base, and dural masses may not be distin-
contrast enhancement of the right anterior clinoid process guishable from other metastases or aggressive meningio-
and adjacent right optic foramen and superior orbital fissure mas. SCNSL is more common in the elderly. Headache is
(anterior transverse arrow). There is avid nodular enhance- present in 30% to 40% of patients. Other presentations may
ment of the right third cranial nerve (posterior transverse include cranial nerve dysfunction as in this patient, insidi-
arrow). There is mass effect on the right temporal lobe. ous nonspecific neurologic symptoms, and encephalopathy.
The cerebrospinal fluid (CSF) examination in this patient
DIFFERENTIAL DIAGNOSISSarcoidosis, lymphoma, was negative for malignant cells which is not unusual as it
metastases, schwannomatosis. may take three CSF examinations to yield a positive result.
However, there was a dramatic response to chemotherapy
DIAGNOSIS DLBCL with central nervous system (CNS) with substantial reduction in size and pattern of contrast
involvement. enhancement of these lesions.
DISCUSSION MRI is the imaging modality of choice in Question for Further Thought
the evaluation of the patient with possible secondary lym- 1. In what ways is PCNSL different from SCNSL?
phoma. CT is significantly less sensitive. This case was
staged as IVB non-Hodgkin DLBCL with CNS involvement;
otherwise known as secondary CNS lymphoma (SCNSL).
Direct reporting is necessary. The prognosis of SCNSL is
There is secondary CNS involvement in about 5% to 9%
dismal. Early treatment may prolong life. Lesions may
of systemic non-Hodgkin lymphoma (NHL). This tends to
mimic other leptomeningeal diseases. Index of suspicion is
occur early at a median of 5 to 12 months after the primary
always necessary, and the diagnosis may not be difficult in a
diagnosis of NHL. About 80% of these are due to DLBCL.
patient with known systemic lymphoma.
This is usually in the form of leptomeningeal and ependymal
disease in about two-thirds of cases. Parenchymal lesions are
uncommon and, when present, typically result from second-
ary involvement from the leptomeninges via infiltration of Location of lesions; they are generally multifocal
the perivascular spaces. Parenchymal masses are hypoin- Complications such as hydrocephalus
tense on T1WI and hypo- to hyperintense on T2WI. The
pattern of leptomeningeal disease may include superficial Answer
cerebral masses, nodular continuous or multifocal enhance- 1. PCNSL usually presents as parenchymal masses which
ment of the ependyma and leptomeninges and/or cranial could be nodular or infiltrative, isodense to hyperdense
nerves. Optic nerve sheath involvement is rare, and multiple on CT and hypointense to isointense on T2WI with avid
cranial nerves nodular or smooth enhancement tends to occur contrast enhancement in the immunocompetent and ring
as late complications. Diffusion restriction in lymphoma is enhancement in the immunocompromised. They are usu-
attributed to its high cellularity. MRS tends to demonstrate ally adjacent to the ventricles particularly in the basal
elevated lipid peaks and high creatine/choline ratio with DTI ganglia or cerebellum and also close to the subarachnoid
showing decreased fractional anisotropy (FA) similar to pri- spaces. Leptomeningeal disease is uncommon and usu-
mary CNS lymphoma (PCNSL). Other findings in SCNSL ally secondary to the parenchymal lesions. SCNSL on the
may include hydrocephalus, contrast-enhancing calvarial other hand is primarily leptomeningeal, dural, and or cal-
and dural thickening and masses, cavernous sinus and pitu- varial/skull base with minimal parenchymal involvement
itary infiltrations, and scalp masses. usually via infiltration of the perivascular spaces. It could
Differential diagnosis may include sarcoidosis, tuber- be difficult to differentiate one from the other however at
culosis, vasculitis, or other inflammatory leptomeningeal imaging.

Case
232 CLINICAL HISTORY 53-year-old female with headaches.
Figure 232-4
Figure 232-3
FINDINGS Figure 232-1. Axial GRE through the inferior component (subacute hemorrhage) superiorly and anteri-
pons. There is a 1.7-cm minimally irregular mass slightly orly (arrow in Figure 232-4), both with a thin hypointense
to the right in the basis pontis (arrow). Mass has a very rim. Figure232-5. Axial post-contrast T1WI through the
thick dark (blooming hemosiderin) rim with a heteroge- mass. There is a thin peripheral rim enhancement posteri-
neous core. Figure 232-2. Axial T2WI through the mass. orly (transverse arrow) associated with a contrast-enhancing
The irregular dark rim is thinner with the heterogeneous DVA in the left cerebellum (vertical arrow).
core slightly wider compared with the GRE. There is no
surrounding edema or mass effect. Figures 232-3 and 232-4. DIFFERENTIAL DIAGNOSISHematoma, cerebral cav-
Axial non-contrast T1WI through the mass at two contigu- ernous malformation (CCM), developmental venous anom-
ous levels. There are two components to the mass. There aly (DVA), hemorrhagic tumor.
is a heterogeneous component (popcorn pattern) inferiorly
and posteriorly (arrow in Figure 232-3) and a hyperintense DIAGNOSIS Braistem CCM with DVA.
501

502 Case 232
children. It could be sporadic and familial. The familial type

is usually multiple and more common in people of Mexican
descent. There are three genes associated with the familial
CCMCCM1, CCM2, and CCM3. Acquired CCM follow-
ing brain radiation is well known. The pathology is that of
a bag of blood-filled caverns with surrounding hemosiderin
and no intervening brain parenchyma. Clinical presentations
include seizures, headache, and focal neurologic deficit.
Most small lesions are asymptomatic. Hemorrhage is the
most common complication. Repeated hemorrhage into the
lesion is common often leading to lesional growth. Surgical
removal is the treatment of choice for symptomatic CCM in
easy-to-reach areas. Radiosurgery has also been used.

1. How is CCM classified?
Figure 232-5 Reporting Responsibilities

Direct reporting is necessary. Presence of hemorrhage,
surrounding edema, and contrast enhancement should be
reported as these may indicate an acute or growing lesion.
DISCUSSION CT may miss small CCM or lesions in dif- Growth or enlargement on follow-up should also be reported.
ficult to evaluate areasbrainstem and peripheral temporal
lobes. Larger lesions are well-circumscribed hyperdense What the Treating Physician Needs to Know
lesions with no surrounding edema or mass effect unless Location and number of lesions
there has been a recent hemorrhage within it. Calcifications Complications if any
may be present. CCM could be single or multiple. MRI Growth pattern on follow-up
offers the best imaging evaluation of CCM. Depending on Presence of DVA
the type, the T1WI may show hyperintensity with surround- Asymptomatic lesions do not require treatment or follow-up
ing hypointense rim or the so-called popcorn ball consist-
ing of heterogeneous intensity with surrounding hypointense Answer
rim. The T2WI show similar changes as T1WI, but the rim 1. A classification for CCM based on pathologic and MRI
is always thicker. There is usually no surrounding edema or characteristics is known as the Zabramski classifica-
mass effect. The hypointense rim blooms on GRE. Small to tion. The type I lesion has hyperintense core on T1WI
punctate lesions are better demonstrated on the GRE which and hyperintense or hypointense core and surrounding
tends to show many more lesions. SWI on the other hand hypointense rim on T2WI with the pathology correlate
defines may more lesions in the familial CCM than GRE. representing subacute blood. The type II has mixed sig-
Contrast enhancement is variable from none to moder- nal core on T1WI and T2WI with surrounding hypoin-
ate. CCM could coexist with DVA in which case the DVA tense rim corresponding to hemorrhagic cavities and
contrast enhances as seen in this case. This association is thrombosis of mixed ages. The type III is isointense
more common in the sporadic type than the familial type. or hypointense on T1WI and T2WI with a hypointense
Contrast-enhanced study is imperative for demonstration of rim, and hypointense with blooming on GRE and corre-
the DVA in view of the risk of venous infarct if inadvertently lates with resolved hemorrhage with hemosiderin within
surgically removed with symptomatic CCM. Angiograms and around the lesions. The type IV is poorly or not
are usually negative in CCM. Hemorrhagic tumor tends to seen on T1WI and T2WI with punctate hypointensity
have surrounding edema. on GRE corresponding to capillary telangiectasia. This
CCM occurs in all ages but more commonly in the elderly. demonstrates a close relationship with the familial form
It is a common cause of parenchymal brain hemorrhage in of CCM.

Case
233 CLINICAL HISTORY 76-year-old male with dysarthria and bradypsychia
upon awakening. There is a history of hypertension currently treated with an
Angiotensin Converting Enzyme (ACE) inhibitor.
FINDINGS Figure 233-1. Axial NCCT through the basal

ganglia. There is an ovoid right lentiform nucleus/external
capsular well-defined homogeneous hyperdensity (arrow)
without significant mass effect on adjacent structures or
midline shift. Findings consistent with acute hematoma.
Figure 233-2. Axial T2WI through the basal ganglia. There
is an ovoid lamellated heterogeneous mass in the right len-
tiform nucleus/external capsule (arrow). The outside ring of
hyperintensity is due to perilesional edema. Figure 233-3.
Axial GRE through the basal ganglia. There is a homo-
geneous hypointense mass in the right lentiform nucleus/
external capsule (arrow). There is a surrounding thin rim of
hyperintensity (edema).
DIFFERENTIAL DIAGNOSIS Hematoma due to vascular

malformations, deep venous thrombosis, hemorrhagic tumors
including metastasis, amyloid angiopathy, anticoagulation,
and drug abuse (typically methamphetamine and cocaine).
DIAGNOSIS Hypertensive basal ganglia hematoma (HBGH).
DISCUSSION CT is usually the initial study in the evalu-

ation of a suspected hematoma. HBGH is seen as a round
or ovoid homogeneous hyperdense mass surrounded by
Figure 233-3 hypodense vasogenic edema and sometimes associated with
503

504 Case 233
leukoaraiosis and lacunar infarcts. Although their typical Management is based on supportive measures like nor-
locations make one immediately suspicious of hypertension malization of blood pressure and correction of coagulopathy
as the cause, atypical locations and patients under 55 years if any. Surgical evacuation has a limited role and in basal
may deserve evaluation with MRI, CTA, or other vascular ganglia hematoma is indicated mainly in patients with large
studies to exclude underlying lesion. Presence of one or more hematomas or lobar clots within 1 cm of the cortical surface.
spot signs (tiny dense dots of contrast enhancement inside the
hematoma) following contrast administration indicate active Questions for Further Thought
bleeding and portray a poor prognosis. On MRI, hematomas 1. What are the risk factors for hematoma growth in the ini-
are mostly heterogeneous with a variable signal intensity tial CT?
that depends on the age of the blood. In the acute phase, 2. Name other clinical risk factors associated with hema-
most hematomas are mostly composed of deoxyhemoglobin toma growth.
which is isointense on T1WI sequences and very dark on
T2WI. GRE and SWI are useful to demonstrate the existence Reporting Responsibilities
of other microhemorrhages that indicate prior hemorrhages Any form of intracranial acute hematoma is an emergency
related to hypertension. Imaging follow-up is essential as deserving of direct reporting. It is important to describe the
one-third of intraparenchymal hematomas can expand more size and extent of the hematoma in the basal ganglia, as
than twice their size in the first few hours, implying a poor well as its relation to the ventricle and the cortical surface.
outcome. Atypical hematomas deserve a contrast-enhanced Suspicion for underlying causes and complications such
study to rule out an underlying lesion. Extension into the as intraventricular hematoma (IVH), subarachnoid hemor-
ventricles is predominantly seen with large hematomas or rhage, presence of raised intracranial pressure, mass effect,
thalamic bleeds and may produce obstructive hydrocephalus. or herniations should be addressed.
Spontaneous HBGH are usually caused by long-term
hypertension and tend to occur in the basal ganglia (40%), What the Treating Physician Needs to Know
thalami (35%), cerebral hemispheres (15%), cerebellum (5% Size and extension of the hematoma
to 10%), and pons (1% to 5%). Hypertension causes a reac- Are there complications?
tive hyperplasia of the arteriolar smooth muscle cells which Is the hematoma atypical enough to suggest an underlying
is later replaced by collagen predisposing these arteries to lesion? If there is a need for further evaluation, what other
occlusions and Charcot-Bouchard aneurysms or ectasia. modalities might be useful for this?
Patients tend to be older than 45 years and have a history of
chronic hypertension. The presentation is variable depend- Answers
ing on the epicenter in the caudate or lentiform nuclei. 1. Findings that suggest hematoma growth on the initial CT
Caudate HBGH usually ruptures into the ventricles and may include large hematoma volume on the initial CT and the
mimic subarachnoid hemorrhage symptoms which may be presence of focal contrast extravasation or the so-called
severe if the anterior limb of the internal capsule is involved. spot sign on the contrast-enhanced study or CTA.
Putaminal HBGH causes sensory and motor deficits as the 2. Oral anticoagulant use, hyperglycemia, renal failure, low
corticospinal tract and the posterior thalamocortical projec- serum cholesterol, platelet dysfunction, and persistently
tions in the posterior limb of the internal capsule may be increased blood pressure are factors that predispose to
compromised. growth of HBGH.

v
Case 234 CLINICAL HISTORY 76-year-old patient with a history of bilateral

blurred vision of several months now has worsening bilateral diplopia and
left pulsatile tinnitus. The right eye is red.
FINDINGS Figure 234-1. Axial NCCT through the cav- the left internal carotid artery (ICA). Figure 234-6. Lateral
ernous sinuses. There is a subtle outward bulge of the DSA of selective left external carotid artery (ECA). There
left cavernous sinus (arrow). Figure 234-2. Axial FLAIR is ECA feeders from the left middle meningeal artery and
through the orbits. There is an enlarged and tortuous left sphenopalatine artery (arrows).
superior ophthalmic vein (arrow). Figure 234-3. 3D TOF
MRA. There is visualization of an enlarged left superior DIFFERENTIAL DIAGNOSISTolosa-Hunt syndrome,
ophthalmic vein (arrow) similar to the arterial structures. cavernous sinus thrombosis, carotid cavernous sinus fistula,
Figure 234-4. Coronal T2WI shows an additional flow thyroid ophthalmopathy, pseudotumor or retrobulbar masses
void in the left cavernous sinus (arrow). Figure 234-5. (such as metastases).
Anteroposterior DSA. There is early opacification of the
cavernous sinuses (arrows) during a selective injection of DIAGNOSIS Carotid-cavernous sinus fistula (CCF).
505

506 Case 234
DISCUSSION CCF is an abnormal vascular communica- high-pressure arterial blood into a low-pressure venous sys-
tion between the ICA or ECA and the cavernous sinus. CT tem, elevating the cavernous sinus pressure and impeding
findings correlate with the underlying physiopathology of the venous drainage of nearby structures, particularly those
CCF and include enlargement and tortuosity of the superior of the orbit. This explains the presenting triad of pulsatile
ophthalmic vein, enlargement of the ipsilateral cavernous exophthalmos, bruit, and conjunctival chemosis. Visual
sinus, thickening of the extraocular muscles and periorbital complaints may be due to retinal ischemia and may indicate
soft tissues, and proptosis. MRI replicates the CT findings the need for urgent treatment. Less common presentations
in addition to orbital edema and abnormal flow voids in the like intracerebral or subarachnoid hemorrhage are due to
affected cavernous sinus. When a high-flow fistula results intracranial venous hypertension and also indicate that the
in retrograde flow into the cortical veins, dilatation of these fistula must be rapidly treated.
veins, leptomeningeal thickening, and signs of venous con- The main objective of CCF treatment is to occlude the
gestion are present and may lead to cerebral edema and hem- fistula while preserving the normal flow of blood through
orrhagic infarctions. Susceptibility weighted imaging clearly the ICA. There are many treatment options. Conservative
shows the multiple, enlarged, and tortuous abnormal cortical management includes manual compression of the ipsilat-
veins. Both CTA and MRA are capable of demonstrating the eral ICA. Endovascular treatment includes either transarte-
additional vessels or signal voids in the cavernous sinuses rial or transvenous embolization, and placement of covered
and enlargement of arterialized superior ophthalmic vein. or flow-diverting stents. Surgical intervention may include
DSA is essential for confirmation of the diagnosis, classify- suturing, clipping, or trapping the fistula, packing the cav-
ing the fistula, and delineating the venous drainage pathways. ernous sinus to occlude the fistula, sealing the fistula with
Enlarged obstructed or thrombosed superior ophthalmic vein fascia and glue, ligating the ICA, or a combination of these
along with filling defects could be present in cavernous sinus procedures.
thrombosis. Enlarged vascular structures are not usual fea-
tures of the other differential. Questions for Further Thought
CCFs can be classified based on etiology (traumatic 1. What other entities are associated with CCFs?
or spontaneous), rate of flow (high versus low flow), the 2. In what group of patients are indirect CCFs often seen?
anatomy of the fistula (direct or indirect), and number of
fistulas (single or multiple). In 1985, Barrow et al. defined Reporting Responsibilities
four types of CCF (types AD). Type A is direct and high- Direct reporting is essential so that urgent treatment might
flow communication between the ICA and the cavernous begin. Identify the imaging signs resulting from high pres-
sinus, and the rest (BD) are indirect and low-flow shunts sure in the cavernous sinus, mainly in the orbital structures
that develop from meningeal branches of either the ICA and in the draining venous structures. Identify enlarged cor-
or the ECA. Type A is the most frequent of all, appear- tical veins that indicate a need for rapid treatment. Describe
ing mainly in young males with a history of a recent head signs of cerebral edema or infarction that do not follow an
trauma. In these cases, the main concern is the acute flow of arterial territory.

Case 234 507

A high degree of clinical suspicion is needed, especially in 1. Fibromuscular dysplasia, Ehlers-Danlos syndrome, and
the emergency setting pseudoxanthoma elasticum.
Absence of compressive lesions such as masses and hema- 2. Indirect CCFs are generally seen in postmenopausal
toma responsible for the proptosis or orbital vascular con- female patients with subtle clinical symptoms related to
gestion them.

Case
235 CLINICAL HISTORY 21-year-old femalet with amnesia, fluctuating
unresponsiveness, crying, hand tapping, and tongue protrusion during
speech.
FINDINGS Figure 235-1. Axial post-contrast CT through

the temporal lobes. There is a vague left temporal lobe
hypodensity with a possible vague ring enhancement in the
left parasellar region (arrow). Figure 235-2. Axial FLAIR
MR through the temporal lobes. There is a confluent medial
left temporal lobe hyperintensity with lateral peripheral
finger-like projections consistent with vasogenic edema
(arrow). Figure 235-3. Axial post-contrast T1WI through the
temporal lobes. There is a 1.5-cm thick ring enhancement
in the medial left temporal lobe just lateral to the left cav-
ernous sinus (arrow). MRA obtained subsequently showed
no aneurysm.
DIFFERENTIAL DIAGNOSIS Ganglioglioma, high-grade

astrocytoma, abscess.
DIAGNOSIS Langerhans cell histiocytosis (LCH).
DISCUSSION LCH rarely affects the central nervous sys-

tem (CNS) alone. It is a multisystem disorder that has pro-
Figure 235-3 tean manifestations with CNS involvement that is mostly
508

Case 235 509
secondary in about 16%. CT and MRI play complementary Immunohistochemistry showed numerous CD68-positive
roles in the evaluation of the multitude of lesions of LCH. cells that were also positive for S100 and CD1a. Electron
This is a case of rare tumorous involvement of the brain microscopy showed sheets of large cells with slightly lobu-
parenchyma which more commonly affects the temporal lated nuclei with prominent nuclear clumping and nucleoli.
lobes where it could be confused with ganglioglioma as it Abundant cytoplasm was noted in some cells. Within the
was in this patient. The more common manifestations are cytoplasm there were numerous mitochondria, moderate
in the hypothalamicpituitary axis in about 50% of CNS amount of rough endoplasmic reticulum, prominent Golgi,
involvement and in the pineal region where the lesion pres- and in most cells numerous Birbeck granules in the rod form.
ents as avidly contrast enhancing masses which could be The findings are consistent with LCH. Clinical presentation
cystic in the pineal region. They are generally isointense of brain parenchymal involvement depends on location of
to hypointense on T1WI and isointense to hyperintense on lesions and may include diabetes insipidus, pituitary hor-
T2WI. Differential diagnosis could include sarcoidosis, mone dysfunction, hydrocephalus, seizures, and psychiatric
astrocytoma, germ cell tumor, lymphoma, and metasta- disorders.
ses. Other manifestations include T2 hypointense choroid
plexus masses in 6% and meningeal involvement in 29%. Question for Further Thought
Neurodegenerative gray matter changes of bilateral sym- 1. What other histiocytic lesions could affect the brain?
metrical lesions of the cerebellum, brainstem, and thalami
and cerebellar atrophy are the second most common brain Reporting Responsibilities
manifestations of LCH. These are of variable signal inten- Mass lesions require direct reporting. Location of lesion is
sity on T1- and T2-weighted MRI. White matter lesions in important for management planning. Definitive diagnosis of
about 36% may include dilated perivascular spaces, multifo- LCH is an enigma requiring correlation of finding from vari-
cal nonspecific T2 hyperintensity, and leukoencephalopathic ous parts of the brain and body and subsequently biopsy.
pattern. Craniofacial bone involvement occurs in up to 80%
and calvarial lytic lesions in up to 54%. Spontaneous regres-
sion of lesions has been reported.
LCH is a proliferative histiocytic disorder of unknown Location is always important for management decision
cause most commonly affecting children and young adults. Is this an isolated lesion or are there other lesions that
Surgical resection of this lesion was undertaken. Pathology could help nail the diagnosis?
described a maroon-brown tissue with the microscopy Are there other imaging techniques that could help in the
showing an intense inflammatory infiltrate consisting of diagnosis: FDG-PET, MRS; and how useful are they?
mononuclear chronic inflammatory cells with numerous
eosinophils, clusters of macrophages, and perivascular Answer
cuffs of lymphocytes. Stains for microorganisms (acid-fast 1. Erdheim-Chester and Rosai-Dorfman disease are other
bacilli, Gomori Methenamine Silver Stain (GMS), Periodic histiocytic lesions that could mimic CNS involvement of
Acid-Schiff (PAS), and Gram stain) were all negative. LCH. These are generally diseases of adults.

Case

510

Case 236 511
FINDINGS Figures 236-1 and 236-2. Axial contiguous or associated with other brain parenchymal injuries. Infarcts
NCCT through the inferior frontal lobes. There is a right fron- are usually wedge-shaped hypodensities within a vascular
tal lobe hypodensity just above the right orbital roof (vertical territory and may also become hemorrhagic.
arrows) consistent with contusion. There is a small lateral Brain contusions are relatively common. They are present
irregular heterogeneous area consistent with the pepper- in up to 43% of closed head injuries. Because of the associated
and-salt pattern of hemorrhagic contusion (black arrows). In injuries, the presentation could be complex and could include
the left frontal lobe, there is a round hyperdensity surrounded headaches, focal neurologic deficit, seizures, symptom and
by a hypodense halo above the left orbital roof (transverse signs of raised intracranial pressure, and mental status changes.
arrows) consistent with hematoma. There is a smaller mixed They are more common in men than women. The pathology
density cortical lesion in the left temporal lobe (chevrons) is that of brain necrosis, edema, and subpial hemorrhages.
consistent with hemorrhagic contusion. Figures236-3 and Management is usually supportive. However, large contu-
236-4. Contiguous NCCT through the inferior frontal lobes 6 sions, contusions associated with multicompartmental injuries,
months after Figures 236-1 and 236-2. There is bifrontal and older age group, and moderate-to-severe head injuries may
left temporal sharply demarcated hypodensities in areas of benefit from aggressive medical and surgical treatment. The
previous contusions and hematoma (arrows) consistent with outcome depends on these other primary or secondary injuries,
encephalomalacia. There is generalized widening of the sub- and favorable outcome indicators are younger age and good
arachnoid spaces indicating volume loss. Glasgow Coma Scale (GCS) scores at presentation.
DIFFERENTIAL DIAGNOSIS Contusions, infarcts. Question for Further Thought

1. What is the role of MRI in the evaluation of contusion?
DIAGNOSIS Multifocal hemorrhagic brain contusions.
DISCUSSION Cerebral contusions could be nonhemor- This is an emergency requiring direct reporting. Location,
rhagic or hemorrhagic. Nonhemorrhagic contusions are number, and other associated injuries should be defined.
generally hypodense on CT. The outline is usually poorly Growth, new lesions, and/or stability on follow-up imaging
defined and can vary in size from punctate to very large. should be reported.
Hemorrhagic contusions tend to be heterogeneous or mixed
density; the salt-and-pepper appearance has been used to What the Treating Physician Needs to Know
describe it. Contusions are commonly multiple superficial Location, number, and associated injuries
injuries due to direct impact of the brain against the rough or Significant changes on follow-up imaging
protuberant bones of the cranial vault in closed head injury. Deterioration in clinical status of the patient may indicate
Hence, they are more common in the frontal lobes, particu- enlarging or presence of new contusions or other injuries.
larly over the rough orbital roofs, along the greater wings Follow-up imaging is usually required
of the sphenoid involving the frontotemporal lobes, and
posteriorly in the temporal lobes around the petrous ridges. Answer
Contusions can also occur as coup and contrecoup injuries 1. MRI may not be very useful in the initial evaluation of
in acceleration and deceleration head injuries. They may contusions because of the clinical instability at this stage
occur as part of multicompartmental injuries. Contusions but very effective during the subacute and chronic stages
evolve over days becoming larger with increasing edema of the injury particularly if patient is not responding to
until stability. As such many more and larger contusions treatment as expected. MRI may show many more lesions
could be identified on follow-up CT. Mass effect resolves than CT. Both bland and hemorrhagic contusions usu-
within 2weeks and depending on their size could persist ally restrict diffusion and depending on the age and the
as permanent hypodense areas or encephalomalacia as seen amount of blood could be T1 and T2 hypo/hyperintense
on the follow-up images in this patient. Associated brain with mass effect depending on their size. Hemorrhagic
volume loss is usually present when the contusions are large components are hypointense on GRE and SWI.

Case
237 CLINICAL HISTORY 3-year-old male with progressive right-sided
hearing loss.
FINDINGS Figure 237-1. Axial NCCT of the right tem- the bony vestibular aqueduct, although there is no reliable
poral bone. There is mild enlargement of the right ves- imaging correlate. A normal endolymphatic sac in the fovea
tibular aqueduct, measuring approximately 1.3 mm (long is barely visible as a fluid-filled hyperintense structure on
white arrow). The left vestibular aqueduct is not enlarged. T2WI. The diagnosis on axial thin-section CT of the tempo-
Figure 237-2. Axial T2WI through the temporal bones. There ral bone is made by measuring the midpoint of the distal limb
is an enlarged, elongated T2 hyperintense structure (isoin- in the bony vestibular aqueduct halfway between the bend at
tense to endolymph of adjacent inner ear structures) along the proximal limb and the fovea. A measurement greater than
the posterior margin of the right temporal bone consistent 1.5 mm is defined as abnormal vestibular aqueduct enlarge-
with an enlarged endolymphatic sac (long white arrow). The ment, although more recent studies have reported a midpoint
diameter of the right endolymphatic duct/sac is larger than measurement greater than 1 mm and opercular measurement
the adjacent semicircular canal (short white arrow). The nor- that is equal to or larger than 2 mm. As a general rule, a ves-
mal left endolymphatic duct/sac is not visible on MRI, an tibular aqueduct diameter that exceeds that of the adjacent
expected finding. posterior semicircular canal or facial nerve canal is consid-
ered enlarged. CT may also demonstrate associated cochlear
DIFFERENTIAL DIAGNOSISPendred syndrome, endo- dysplasias and vestibular anomalies, the most common being
lymphatic sac tumor, enlarged endolymphatic sac. modiolar deficiency (or absence). Although thin-section CT
of the temporal bone is adequate to demonstrate enlargement
DIAGNOSIS Enlarged endolymphatic sac syndrome. of the vestibular aqueduct, it cannot show the membranous
labyrinth and thus is less sensitive for the detection of associ-
DISCUSSION On CT, the normal bony vestibular aque- ated cochlear anomalies. Enlargement of the endolymphatic
duct, which contains the endolymphatic duct, has an inverted sac may occur in the presence of a normal-sized vestibular
J or hockey stick appearance. The short limb arises along aqueduct on CT. High-resolution T2WI is the superior imag-
the anteromedial wall of the vestibule and courses postero- ing tool, even in less-experienced hands, allowing for obvi-
medially and parallel to the common crus, descends postero- ous detection of an elongated fluid-filled T2 hyperintense
laterally through the petrous pyramid, and terminates along endolymphatic sac. Precise measurements on axial T2WI are
the posterior temporal bone. On MRI, the normal endolym- still made at the midpoint between the bend at the proximal
phatic duct is seen as the union of the utricular and saccular limb of the endolymphatic duct and the posterior margin of
ducts and courses through the bony vestibular aqueduct to the temporal bone. Endolymphatic sac tumor presents as a
terminate as a blind-ending endolymphatic sac in the fovea highly vascular retrolabyrinthine mass centered in the fovea
of the posterior temporal bone, a small recess between lay- of the endolymphatic sac and demonstrates calcification, het-
ers of dura and adjacent to the sigmoid sinus. The transi- erogeneous MRI signal and enhancement, and permeative
tion between the endolymphatic duct and sac occurs within bony destruction.
512

Case 237 513
Enlargement of the endolymphatic duct and sac is rec- vestibular anomalies, such as enlargement of the membra-
ognized as the most common congenital anomaly of the nous vestibule, gross vestibular dysplasias, and semicircular
inner ear on imaging and is one of the most common mor- canal dysplasias.
phologic findings associated with congenital sensorineural
hearing loss. Embryologically described as arrested fetal What the Treating Physician Needs to Know
development of the inner ear due to a nonspecific insult, the Laterality
trigger of sensorineural hearing loss in this condition may Associated cochlear and vestibular anomalies
be mild head trauma to an already fragile or structurally
deficient cochlea. Answers
1. Most cases are sporadic, although a familial autosomal
Questions for Further Thought recessive inheritance pattern has been reported. Pendred
1. Are there any associated genetic alterations? syndrome, an autosomal recessive syndrome seen in
2. What is the choice of therapy? 15% of patients with enlarged vestibular aqueduct due
to SLC26A4 Pendrin gene mutation on chromosome7,
Reporting Responsibilities presents with dyshormonogenic goiter and modiolar
Routine reporting is sufficient, as this is not an acute disorder deficiency.
unless it is unexpected. Enlargement of the endolymphatic 2. There is no accepted treatment, although physicians may
sac may occur in the presence of a normal-sized vestibular recommend avoidance of contact sports or other activi-
aqueduct on CT, and as such, high-resolution MRI should be ties that may result in head trauma and promote hearing
pursued in the appropriate clinical context. It is important to loss in this condition. Surgical occlusion and oblitera-
have a systematic approach in evaluating temporal bone CT, tion of the endolymphatic sac has had limited success.
as the diagnosis is easily missed if not carefully scrutinized. Fortunately, cochlear implantation has been shown to sig-
It is necessary to report associated cochlear a nomalies, nificantly increase quality of life for those with profound
cochlear dysmorphisms, scalar asymmetries, as well as bilateral SNHL.

Case
238 CLINICAL HISTORY 60-year-old female with severe acute head injury.
FINDINGS Figure 238-1. Axial NCCT through the fora- cerebellum. Figure 238-3. Axial NCCT through the upper
men magnum. There is complete effacement of the cisterna pons. There is swelling and mild anteroposterior elonga-
magna and the cerebrospinal fluid (CSF) space surrounding tion of the pons. There is swelling of the cerebellum and
the upper spinal cord (arrow). Figure 238-2. Axial NCCT visualized supratentorial brain with obliteration of all CSF
through the inferior posterior fossa. There is complete spaces. There are hemorrhages in the pons (transverse white
obliteration of the posterior fossa CSF spaces including the arrows) and around the tentorium. In the right temporal lobe,
fourth ventricle, the cerebellopontine angle (CPA) cisterns, pneumocephalus is present (vertical white arrow). There
and the pericerebellar spaces by the swollen brainstem and is also a right temporal extraaxial acute hemorrhage and
514

Case 238 515
p neumocephalus (black arrow). A transtentorial herniation of the herniation and accompanying injuries in the setting
is present in this patient (not shown). Figure 238-4. Sagittal of trauma.
MRI T1WI through the foramen magnum in a different
patient with a posterior fossa mass. There is inferior cerebel- Question for Further Thought
lar descent through the foramen magnum (vertical arrow). 1. In what other conditions could you have foramen mag-
There is compression of the brainstem and the medulla is num herniation?
partially squeezed through the foramen magnum (transverse
arrow).
This is an acute condition and should be reported directly to
the referring physician. The extent of displacement may not
be visible on CT but measurable on MRI. The reasons for the
DIAGNOSIS Foramen magnum herniation.
herniation should be described and other associated patholo-
gies categorized.
DISCUSSION Foramen magnum herniation is the pro-
trusion of the posterior fossa structures through the fora-
men magnum. The classical foramen magnum herniation is What the Treating Physician Needs to Know
the Chiari malformation, where the cerebellar tonsils and Extent of displacement of the tonsils and other structures
inferior posterior fossa structures are projecting below the if possible. MRI does this better than CT but may not be
foramen magnum. Traumatic foramen magnum herniation available in the acute trauma phase
is usually due to mass effect or swelling of posterior fossa Other displaced structures such as the medulla and the
structures, or continuation of transtentorial herniation push- fourth ventricle
ing down on posterior fossa structures through the fora- Presence of mass, swelling, or other primary and second-
men magnum. The diagnosis could be challenging on axial ary injuries intracranially
CT. CT usually shows crowding of the foramen magnum
with obliteration of the CSF spaces particularly the cisterna Answer
magna and inferior posterior fossa. If it is possible to refor- 1. Foramen magnum herniation can occur in intracranial hypo-
mat the CT in the sagittal direction, the obliteration of the tension or in any situation where there is spinal CSF leak.
cisterna magna becomes readily visible. The best demon- The abnormally low spinal canal pressure allows the ton-
stration, however, is by MRI. The axial images would show sils and some other posterior fossa structures to be sucked
the cerebellar tonsils posteriorly in the foramen magnum through the foramen magnum into the upper spinal canal.
and obliteration of the cisterna magna. Sagittal images show Similar displacement can occur in paradoxical herniation due
the descent of posterior fossa structures to best advantage to negative pressure gradient in patients with decompressive
with the inferior extent of the tonsils visible in the cervical craniectomy. Benign idiopathic intracranial hypertension can
spinal canal posteriorly to the spinal cord. MRI may not be mimic Chiari malformation with displacement of the tonsils
feasible in the acute phase of trauma. The diagnosis is sug- into the spinal canal. It is generally advised that a lumbar
gested on the basis of the CT findings. Hydrocephalus may puncture be withheld in patients with any form of herniation
be a feature. Clinical presentation depends on the severity or in those situations known to predispose to herniation.

Case
239 CLINICAL HISTORY 46-year-old male with a mass on the head.
FINDINGS Figures 239-1 and 239-2. Axial NCCT bone Question for Further Thought
and soft tissue windows, respectively, through the parietal 1. Typical, atypical, and aggressive hemangiomas, what is
bones. There is a soft tissue mass of the right parietal cal- the difference?
varium (arrows) with through and through cortical erosion
with beveled margin. Reporting Responsibilities
Routine reporting is sufficient since most of these are asymp-
DIFFERENTIAL DIAGNOSIS Metastatic disease, myeloma, tomatic and incidental. Large lesions, however, may require
lymphoma, eosinophilic granuloma, hemangioma. direct reporting for proper management. Recognize the
benign nature of the lesion. Many of these lesions may have
DIAGNOSIS Right parietal bone hemangioma. nonspecific appearance on MRI. When in doubt, suggest CT
follow-up, as the classic patterns of lucency noted above will
DISCUSSION Hemangioma of the bone is a benign lesion often help to exclude other possibilities.
of disordered vessels, not a tumor. On CT, hemangioma
presents as focal lucency within a bone. Occasionally, an
internal texture may be discerned, for example, a honeycomb
or spoke-wheel pattern in the skull, or a striated, corduroy Hemangioma of the bone is most often an occult, incidental
pattern in the spine. On MRI, hemangiomas are enhanc- finding of no significance
ing lesions that are hyperintense of T2WI and isointense to In rare cases, large or atypical lesions may require tissue
hyperintense on T1WI depending on the amount of fat pres- sampling
ent. Cranial vault hemangiomas are usually small and result
in cortical thinning and diploic widening, often with greater Answer
effect on the outer table rather than the inner table. The large 1. These terms are most often used in relation to spine
and aggressive appearing lesion in the above case is unusual. lesions. Atypical hemangioma will be hyperintense
This lesion could mimic any of the lesions in the list of dif- on T1WI (reflecting fatty material) and T2WI (reflect-
ferential diagnoses. Tissue sampling will be necessary for ing slow flow vessels in addition to fatty material).
the correct diagnosis. This appearance is highly specific and should lead to a
Histologic classification of hemangiomas depends on the confident diagnosis. An atypical hemangioma remains
histologic type of vessels present. Capillary hemangioma, hyperintense on T2WI but lacks T1 hyperintensity due to
commonly found in the spine, shows numerous small cali- scarcity of fat. This is a nonspecific appearance and can
ber vessels, often oriented in parallel. Cavernous heman- be a diagnostic dilemma as metastatic disease or other
gioma is composed of fewer but larger vessels along with marrow-replacing processes may present similarly. An
fibrous septa and is more common in the skull. Mixed capil- aggressive hemangioma will appear as in the case above,
lary and cavernous varieties also exist. Regardless of the with substantial bony deformation and often an extraosse-
site, these lesions are most often asymptomatic and detected ous soft tissue component. Tissue sampling is necessary
incidentally. in these cases to exclude a malignant etiology.
516

Case
240 CLINICAL HISTORY 29-year-old male involved in a motor vehicle
collision.
FINDINGS Figure 240-1. Axial NCCT through the frontal

lobes. There is a right frontal convexity extraaxial biconvex
hyperdensity measuring 1.1 cm in maximum thickness dis-
placing the brain medially from the inner table (arrow). There
is overlying subgaleal hematoma and swelling of the scalp.
Figure 240-2. Axial NCCT bone algorithm blood window
level 1.25 mm reconstruction at a level inferior to Figure
240-1. This emphasizes the biconvex nature of the hyper-
density. There is a hairline fracture of the overlying frontal
bone (not shown). Figure 240-3. Axial NCCT in a differ-
ent patient. This demonstrates a very large biconvex mixed
density but mostly hyperdense right frontal acute extraaxial
hematoma with significant mass effect on the right frontal
lobe. The mixed density suggests active bleeding.
DIFFERENTIAL DIAGNOSIS Acute subdural hematoma

(ASDH), acute epidural hematoma (AEDH).
DIAGNOSIS Acute epidural hematoma (AEDH).
DISCUSSION AEDH is a biconvex hyperdense extraaxial

collection on CT displacing the brain from the inner table. It
derives its biconvex configuration from its location between
the calvarium and the periosteal dura, which limits its spread
to the tight sutural attachment of the periosteal dura. Hence,
Figure 240-3 it is said that epidural hematoma (EDH) does not cross the
517

518 Case 240
suture line. In a small percentage of patients, this may not monitoring. EDH enlargement occurs frequently and early
always be true! EDH at the convexity crosses the midline between 8 and 36 hours. It has been recommended that a
superiorly to the superior sagittal sinus. There is associated repeat CT be obtained within 36 hours or sooner depend-
fracture in up to 95% of the cases. A fracture does not have to ing on the condition of the patient. Operative management
be present in a minority of patients and particularly in children. is generally recommended for EDH larger than 1.5 cm.
Mass effect is always present. Large EDH may lead to brain Endovascular embolization of the bleeding artery seems to
herniations. Figure 240-3 illustrates how large AEDH could be a feasible option to stop rehemorrhage and enlargement
get and the significant mass effect it could exert on surround- of the EDH.
ing structures. Other forms of brain injuries could be present
compounding the management issues in these patients. CT Question for Further Thought
provides a very rapid way to evaluate this condition like in all 1. What is the pathogenesis of AEDH?
cases of acute injuries to the head and is recommended for all
patients with moderate (Glasgow Coma Scale [GCS] of 9 to
12), severe (GCS of 8 or less) head injury, and for mild (GCS
Direct reporting is required. Location and size of EDH along
of 12 to 15) head injury under certain circumstances. Large
with the pattern of mass effect and herniations if any should
hematomas are easily detectable, while tiny ones may need
be clearly communicated. Heterogeneous or mixed density
window level adjustment to a blood window level. MRI
AEDH may indicate ongoing bleed requiring closer atten-
particularly DWI, FLAIR, and GRE sequences could be use-
tion. Other injuries should be enumerated. Any significant
ful when necessary but may not be appropriate in an unstable
changes on follow-up should also be communicated directly.
clinical environment. EDH is more common supratentorially
in the temporoparietal regions where the large middle men-
ingeal artery and its branches could be disrupted by fractures. What the Treating Physician Needs to Know
It is found all over the cranial cavity including the poste- Location and size and number if more than one
rior fossa. It is bilateral in up to 10% of adult EDH with the Presence of a fracture, hyperacute blood, mass effect, or
second lesion on the contrecoup side. ASDH, however, is herniations
usually crescentic in configuration extending along the con- Significant changes on follow-up
vexity and parafalcine surfaces of the brain. However, it may
be difficult to differentiate ASDH from AEDH particularly Answer
in small volume collections. Chronic subdural hematoma 1. EDH is due to a direct force on the calvarium, resulting
(SDH) may become biconvex and loculated because of fibrin in a fracture and laceration of meningeal arteries. Blood
and septal formation within the collection. accumulates between the calvarium and the periosteal
EDH could be found in up to 4% of all head injuries. It dura. The arterial nature of the bleed leads to rapid and
is more common in men than women. It is not as common continuing accumulation of blood that does not cross
in infants and the elderly. Patients usually present with the suture lines because of the dural attachment at the sutures.
so-called lucid interval between an initial loss of conscious- This results in a biconvex configuration of the hematoma
ness at the site of trauma and subsequent loss of conscious- and local mass effect. Large hematoma may lead to her-
ness that may be fatal. Lucid interval occurs in about 33% niations. EDH is more common in areas rich in arterial
of EDH. More common presentations include headache, sei- meningeal supply such as along the middle meningeal
zures, nausea, vomiting, focal neurologic deficit, and mental artery and its branches in the frontotemporal regions.
status changes. Posterior fossa EDH could be rapidly fatal Venous bleed when it occurs may result in delayed pre-
due to progressive accumulation of blood in the tight space sentation and smaller EDH volume. A fracture does not
with compression of the brainstem. Management in small have to be present in children to cause EDH because of
EDH could be conservative with close clinical and imaging the plasticity of the childs cranial vault.

Case
241 CLINICAL HISTORY 41-year-old female, without significant past medical
history with a 3-day history of generalized malaise, nausea, headache, lethargy,
and fever.
FINDINGS Figure 241-1. Axial NCCT of the head through Axial DWI through the trigones. There is bilateral intra-
the thalami. There is a well-defined small hypodensity in ventricular restricted diffusion with fluid-fluid levels in the
the left thalamus (arrow). There are fluid-fluid levels in the trigones consistent with cellular debris (arrows). Figure
trigones bilaterally consistent with debris (vertical arrows). 241-5. Axial FLAIR through the third ventricle. There is
There is ventriculomegaly. Effacement of convexity sub- a left thalamic well-circumscribed hyperintensity (trans-
arachnoid spaces is present due to raised intracranial verse arrow) corresponding to the changes seen in Figures
pressure. Figures 241-2 and 241-3. Corresponding axial 241-1 to 241-3. There are fluid-fluid levels in the trigones
DWI and ADC map through the thalami. There is a left (vertical arrows). The precipitate is hyperintense. There
thalamic focal restricted diffusion (arrows) with surround- is hydrocephalus with periventricular edema. Figure 241-
ing small edema consistent with an abscess. Figure241-4. 6. Post-contrast coronal T1WI. There is a left thalamic
519

520 Case 241
smooth ring enhancement opening superiorly into the left tuberculous and fungal abscesses and the presence of vari-
lateral ventricle (arrow), representing the point of rupture ous amino acids only in pyogenic abscesses. Tumoral cavi-
of the abscess. ties generally do not restrict diffusion centrally, but lipids
and lactate peaks could be present on MRS. Abscesses close
DIFFERENTIAL DIAGNOSIS Pyogenic abscess, tubercu- to the ventricles could rupture into the ventricle producing
lous abscess, fungal abscess, necrotic tumor. ventriculitis. In up to 30% of adult cases and up to 90% of
neonatal cases, ventriculitis and meningitis could complicate
DIAGNOSIS Left thalamic pyogenic abscess with ven- cerebral abscess. Hydrocephalus is a known complication of
triculitis. meningitis and ventriculitis. The cellular debris within the
ventricles usually restricts diffusion. There may or may not
DISCUSSION A brain pyogenic abscess is typically a be ependymal enhancement. Follow-up MRI in this patient
well-defined cystic lesion with surrounding hypodense vaso- eventually showed ependymal enhancement and subdural
genic edema on NCCT. Following contrast administration, empyema.
there is usually a smooth ring enhancement separating the Pyogenic brain abscess is usually hematogenous in origin.
cystic lesion from the surrounding edema. The mass effect Direct inoculation of the brain is always a possibility during
depends on the size of the abscess and its surrounding surgical interventions. The common presentations include
edema. Most pyogenic abscesses are located in the white headache, fever, focal neurologic deficit, and altered men-
gray matter junction. MRI is the preferred examination for tal status. Definitive diagnosis requires CSF examination or
evaluating this condition. An abscess is round with central stereotactic guided drainage with culture. The CSF is usually
restricted diffusion and surrounding vasogenic edema. It cloudy or turbid with pleocytosis, low glucose, and high pro-
is hypointense on T1WI and hyperintense on T2WI. There tein. Treatment is with appropriate antibiotics. CSF diver-
is a smooth thin ring contrast enhancement separating the sion may be necessary for the treatment of hydrocephalus
abscess from the edema. There is a mass effect, and the and ventriculitis.
degree of herniation depends on the size and the amount of
surrounding edema. An abscess is more frequently supra-
tentorial in location distributed almost evenly between all
cerebral lobes. It is solitary in over 50% of cases. It could 1. How safe is a lumbar puncture (LP) in the presence of a
be difficult to differentiate a pyogenic abscess from a fun- significant mass effect from an abscess?
gal or tuberculous abscess. Fungal abscess tends to have a
rather irregular thick contrast-enhancing wall with internal Reporting Responsibilities
projections, while the tuberculous abscess wall is smooth, An abscess is an emergency requiring direct reporting.
multilobulated, or crenated. Fungal abscess may show inter- Presence of hydrocephalus or ventriculitis or significant
nal hypointensity or heterogeneity on spin echo sequences mass effect such as subfalcine, transtentorial, uncal, or
with peripheral restricted diffusion. MRS has been shown transforaminal herniation should be appropriately stated and
to demonstrate presence of lipid and lactate in pyogenic, communicated as such.

Case 241 521

MRI is preferable to CT in the evaluation of the patient 1. LP could be detrimental in the presence of intracranial
suspected of a brain abscess mass effect or raised intracranial pressure resulting in
Location and number if more than one herniation as it may worsen the herniation. In this case,
Is LP safe? Significant mass effect may contraindicate LP CSF was obtained through an external ventricular drain,
It could be impossible to determine the offending organ- while the abscess was stereotactically drained under CT
ism by imaging guidance.

Case
242 CLINICAL HISTORY 60-year-old male with mental status changes.
522

Case 242 523
also be involved. Hydrocephalus is not common. Other less

common areas of involvement include the eye and the spi-
nal cord. PCNSL appears hyperdense relative to the brain
with surrounding hypodense edema on NCCT. It usually
enhances avidly following contrast administration. PCNSL
is hypointense on T1WI and isointense to hyperintense on
T2WI. Perilesional edema is frequently present although
disproportionate to the lesion size. Enhancement is mostly
homogeneous and strong. Closed and occasionally open-ring
contrast enhancement is possible in immunosuppressed pop-
ulation due to necrosis. The notch sign refers to an abnor-
mally deep depression at the tumor margin on post-contrast
images. Diffusion restriction is common in PCNSL. This is
explained on the basis of high tumor cellularity. Diffusion
restriction is also present in acute ischemic stroke, the central
necrosis of brain abscesses, the solid portion of high-grade
gliomas, and some metastases. However, PCNSL lesions
Figure 242-5
often have more restricted diffusion and lower ADC values
than high-grade gliomas and metastases. Because PCNSL is
glucose and thallium avid, PET and thallium scans can help
in differentiating PCNSL for its mimics.
FINDINGS Figures 242-1 and 242-2. Axial DWI through
PCNSL is a rare but lethal disease with a peak incidence
the splenium and body of corpus callosum (CC), respec-
in the sixth and seventh decades in the immunocompetent
tively. There are smudgy hyperintensities in the splenium
and fourth decade in the immunosuppressed. It has a slight
(vertical arrow in Figure 242-1) and body of CC (transverse
male preponderance. Clinical presentations include focal
arrow in Figure 242-2). These areas show low ADC. Similar
neurologic deficit, mental status changes, seizures, and neu-
smudgy hyperintensities are present in bilateral periven-
ropsychiatric behavior. The diagnosis was made by brain
tricular white matter (WM). Figure 242-3. Axial FLAIR
biopsy in this patient. Lumbar puncture (LP) with CSF
through the genu of CC. There is smudgy hyperintensity
cytology could be useful in making a diagnosis but is much
extending from the right frontal WM to the left through the
more rewarding in secondary CNS lymphoma (SCNSL)
genu of CC (arrow) the so-called butterfly lesion. Figure
where the tumor is mostly leptomeningeal. Prognosis is
242-4. Axial FLAIR through the body of CC. There is a
dismal without treatment. Chemoradiation may prolong life
transverse band of smudgy hyperintensity through the mid
significantly.
body of the CC (vertical arrow). Similar smudgy periven-
tricular hyperintensities are present in the right corona radi-
ata with the right frontal lesion showing an iso/hypointense
core. Figure242-5. Coronal post-contrast T1WI through the 1. How common is leptomeningeal enhancement in PCNSL?
splenium. There is a band of contrast enhancement through 2. What is the ghost-and-mimicry tumor?
the splenium (vertical arrow) and in the right corona radi-
ata (transverse arrow). Similarly there is smudgy contrast Reporting Responsibilities
enhancement across the body of CC (not shown). Direct reporting is necessary. The multifocality of lesions
and a wide range of differential mandate direct reporting.
DIFFERENTIAL DIAGNOSIS Acute disseminated enceph
alomyelitis (ADEM), multiple sclerosis (MS), lymphoma, but- What the Treating Physician Needs to Know
terfly glioma. Location of lesions in CC for surgical planning
A bulky infiltration of the CC that is not accompanied by
DIAGNOSIS Primary CNS lymphoma (PCNSL) with CC necrosis is suggestive of PCNSL
involvement. The incidence of PCNSL has been on the rise in immuno-
competent individuals. A high index of suspicion is there-
DISCUSSION PCNSL presents as a monofocal or multifo- fore in order in this population
cal disease with the monofocal type predominating. Lesions Conversely with the use of highly active antiretroviral
are present predominantly in the cerebral hemispheres, in therapies (HAART), the incidence of PCNSL in HIV
particular the frontal lobes, followed by the CC and basal patients has declined
ganglia. Butterfly lesions extending from one hemispheric
WM to the other across the midline are typically found in Answers
the splenium or genu of the CC. This pattern is shared with 1. PCNSL is predominantly a brain parenchymal disease. The
other lesions such as tumefactive demyelination, astrocy- sensitivity of MRI for the detection of leptomeningeal
toma, and ODG. The cerebellum and posterior fossa can disease in PCNSL is estimated to be around 20% or less.

524 Case 242
Therefore, the exclusion of leptomeningeal seeding in 2. Ghost-and-mimicry tumor is PCNSL that was initially
PCNSL is not always possible with imaging methods confused with an alternative diagnosis by biopsy but
alone. CSF analysis remains indispensable and even then resolved with steroid treatment and worsened again lead-
requires serial CSF studies to increase the yield of positive ing to a second biopsy with a definitive diagnosis. This
findings. SCNSL on the other hand is predominantly lep- designation underlines two important features of PCNSL:
tomeningeal with perivascular brain parenchymal involve- the likelihood of an alternative diagnosis such as ADEM
ment and significant positive CSF yield. and steroid sensitivity.

Case
243 CLINICAL HISTORY 58-year-old female experienced brief inability
to name items, inability to name her grandchildren, or recite the months
of the year.
525

526 Case 243
enhancement pattern of this lesion may suggest a subacute

left MCA territory infarct. Gyriform cortical enhancement
would be expected in a late subacute infarct of this size.
The mixed pattern of ADC values would also be unusual
for an ischemic stroke, but acute on chronic ischemia can
produce a mixed pattern. Bacterial encephalitis should be
associated with meningitis with diffuse meningeal and corti-
cal enhancement. Viral encephalitis particularly HSV 1 is
centered in the medial temporal lobe usually with evidence
of restricted diffusion but patchy hemorrhage and contrast
enhancement may be present. PML is a subcortical lesion in
the immunocompromised which can have areas of restricted
diffusion, and enhancement may be present in Immune
reconstitution inflammatory syndrome (IRIS).
AA tends to occur in older patients than WHO II astro-
cytoma predominantly occurring in the fifth to sixth decades
Figure 243-5
but do occur in a wide age range with almost equal male
to female ratio. Clinical presentation of AA depends on its
location. Frontal lobe lesions may present with behavioral
changes. Speech impediment, vision impairment, motor and
FINDINGS Figures 243-1 and 243-2. Axial DWI and cor-
sensory changes are some of the neurologic deficits experi-
responding ADC map through the sylvian region. There is
enced by these patients. Seizure is perhaps the commonest
a left insula/subinsula minimal opercula diffuse hyperin-
presenting symptoms. AA is more hypercellular and dem-
tensity on DWI with a small focal area of diffusion restric-
onstrates more nuclear pleomorphism and hyperchromasia,
tion in the insula on ADC map (arrow). Figure 243-3. Axial
in contrast to grade II astrocytoma. Mitoses are usually seen
FLAIR through the lesion. There is diffuse hyperintensity
easily. Neither vascular endothelial proliferation nor necro-
in the left insula and surrounding region with apparently
sis is a feature of AA. Most (75% to 80%) of the diffuse
well-defined margin except anteriorly (arrow). There is no
(grades II and III) astrocytomas demonstrate mutations of
significant mass effect. Figure 243-4. Coronal post-con-
the isocitrate dehydrogenase (1 or 2) gene. TP53 mutation
trast T1WI through the lesion. There is patchy lineal pre-
is also common. AA invariably progresses to glioblastoma
dominant peripheral enhancement (arrows). Figure 243-5.
within a short period. Poor prognostic factors include older
Photomicrograph shows hypercellular tumor with atypical
age at onset, small cell morphology, and epidermal growth
hyperchromatic nuclei and several mitoses.
factor receptor (EGFR) amplification within the tumor.
DIFFERENTIAL DIAGNOSIS Subacute enhancing MCA
infarct, encephalitis (especially HSV), progressive multifo-
cal leukoencephalopathy (PML), anaplastic astrocytoma 1. What abnormalities are detected in the peritumoral area
(AA) WHO III, astrocytoma WHO II, changes due to seizure. by spectroscopy and diffusion tensor imaging?
DIAGNOSIS Anaplastic astrocytoma (AA). Reporting Responsibilities

All tumors deserve direct reporting. Presence of significant
DISCUSSION AA is a WHO III astrocytoma. It presents mass effect and herniations makes reporting more urgent.
as a focal diffuse hypodensity on CT with patchy contrast The location of the mass is important with regard to treat-
enhancement. MRI usually shows an infiltrating hypoin- ment options. Lesions located in eloquent areas may benefit
tense lesion on T1WI that is poorly defined with enlarge- from functional imaging, and this should be recommended.
ment of infiltrated structures. AA is diffusely hyperintense Alteration in size and change in pattern of contrast enhance-
on FLAIR and T2WI with patchy contrast enhancement ment on follow-up may indicate transformation to GB.
on post-contrast T1WI. AAs have a greater tendency to Presence of leptomeningeal enhancement may suggest cere-
enhance than WHO II astrocytomas. Astrocytomas tend brospinal fluid (CSF) seeding.
to be WM based, but this case demonstrates that they can
have gray matter involvement. Higher-grade astrocytomas What the Treating Physician Needs to Know
may have areas of restricted diffusion that can be related Location. This is important for treatment purposes
to areas of hypercellularity. The ADC values of low-grade Whether this may represent primary or secondary AA if
gliomas tend to be elevated compared with higher-grade possible. Comparison with prior studies becomes impor-
gliomas. ADC values in glioblastoma (GB) are generally tant in this regard
lower than in AAs. ADC values are also found to be a use- What advanced imaging studies may be useful for further
ful biomarker to indicate survival. Thelocation and contrast definition

Case 243 527
Answer most of these tracts are disarranged. Low FA ratios in the

1. Peritumoral MRS demonstrates elevated Cho/Cr and tumor center are consistent with a high degree of disorga-
Cho/NAA ratios in high-grade tumors. The periphery nization of myelinated fiber tracts in both low-grade and
of low-grade gliomas contains a considerable amount of high-grade gliomas.
preserved fiber tracts. In high-grade gliomas, however,

Case
244 CLINICAL HISTORY Normal variants of cervical arterial anatomy.

528

Case 244 529
FINDINGS All figures are contrast-enhanced MIP or vol-

ume-rendering neck MRA in different projections to best
demonstrate the anatomy. Figure 244-1. Normal three-vessel
origins from the aortic arch. From right to left are the bra-
chiocephalic trunk, which divides into the right subclavian
artery and the right common carotid artery (RCCA), the
left common carotid artery (LCCA), and the left subclavian
artery. The vertebral arteries (transverse arrows) originate
from the subclavian arteries. The common carotid artery
(CCA) divides distally into the internal carotid artery (ICA)
and the external carotid artery (ECA) at about C4 level. This
is the most common aortic arch configuration encountered.
Figure244-2. There is a common origin of the brachioce-
phalic trunk and the LCCAthe so-called bovine origin
(arrow) which is different from the real bovine configura-
tion. This is a common anomaly. The true bovine configura-
tion has a single trunk from the aortic arch that gives rise to
all the neck and head vessels. The LCCA (line arrow) arises
as a branch of the brachiocephalic trunk in this case. The left
subclavian artery has a separate origin (transverse arrow).
The chevrons point to the CCA bifurcations. Figure 244-3.
Four-vessel origins from the aortic arch. From right to left
are the brachiocephalic trunk, the LCCA, the left vertebral
artery (originating directly from the aortic arch) (arrow), and
the left subclavian artery. Figure 244-4. There is aberrant
origin of the right subclavian artery (vertical black arrow)
from the aortic arch distal to the origin of the left subcla-
Figure 244-7 vian artery. It then courses behind the esophagus to reach

530 Case 244
the right side. The right vertebral artery originates from for arterial stenosis and occlusions. There are also congenital
the aberrant right subclavian artery (chevron). There is a collateral anastomoses of the carotid and the vertebrobasilar
common origin of the RCCA and LCCA (transverse white systems; the persistent trigeminal artery, the persistent otic
arrow). The left subclavian artery originates normally from artery, the persistent hypoglossal artery, and the persistent
the aortic arch. Figure 244-5. Each great vessel originates proatlantal artery. The brain is probably the most demand-
separately from the aortic arch. These are from right to left, ing organ in the body representing about 2% (3 lb) of total
RCCA, right subclavian, LCCA, and left subclavian. There body weight yet it receives about 15% (750 mL) of the total
is no brachiocephalic trunk. The vertebral arteries emanate cardiac output. Perhaps this is due to the important functions
from their respective subclavian arteries. Figure 244-6. This of the brain. This blood supply could be drastically reduced
is a variant of the aberrant right subclavian artery with the or affected by both congenital and acquired anomalies of the
right vertebral artery arising from the RCCA (arrow). There aortic arch and of the individual vessels with significant con-
are separate origins of the RCCA and LCCA from the aortic sequences to the brain and the body in general.
arch (vertical arrows). Figure 244-7. There is duplication of
the origin of the right vertebral artery (transverse arrows) Question for Further Thought
from the right subclavian artery and the two duplicated limbs 1. What are the common acquired abnormalities of the great
join distally (chevron). neck vessels?

Routine reporting of the neck vascular imaging is suffi-
DIAGNOSIS Normal variant vascular anatomy of the ori- cient. In the case of significant arterial disease due to ath-
gins of the great vessels from the aortic arch. erosclerotic stenosis, fibromuscular dysplasia, dissection,
pseudoaneurysms, and arterial blowout, additional direct
DISCUSSION The embryology of the aortic arch is com- communication with the referring physician becomes essen-
plex and will not be described here. More than 20 aortic arch tial. However, all anomalies should be carefully categorized
variants have been described. This panel demonstrates some and communicated accordingly.
of the normal variant anatomy of the origins of the great ves-
sels from the aortic arch. The commonest encountered pattern What the Treating Physician Needs to Know
of the aortic arch branches is in Figure 244-1, where there are The pattern of the congenital variants
three vessels originating from the aortic arch. This is found Other associated anomalies
in between 75% and 93% of the population depending on the
ethnic or racial population studied. The blood supply to the Answer
brain is through four major vessels, the bilateral ICA form- 1. The common acquired anomalies of the great vessels
ing the anterior circulation and the bilateral vertebral arteries of the neck are related to degenerative, inflammatory,
joining to form the basilar artery and the posterior circulation. and traumatic changes. The predominant degenerative
In about 10% of the population one vertebral artery termi- change is atherosclerotic in origin, resulting mostly in
nates in posterior inferior cerebellar artery (PICA) and do not luminal narrowing, mural calcifications, artery-to-artery
make any significant contribution to the basilar artery. The embolism, occlusion, or fusiform aneurysm formation.
extracranial circulation and the blood supply to the meninges Inflammatory lesions include vasculitis, vasculopathy,
are provided by the bilateral ECA. The ICA and ECA are the blowout lesions, and mycotic aneurysms. Traumatic
two branches of the CCA and they both anastomose at the lesions include dissections resulting in stenosis, pseudoa-
skull base. These anastomoses could be useful compensating neurysms, occlusions, or arteriovenous fistula formation.

Case
245 CLINICAL HISTORY 19-year-old male with moyamoya syndrome
(proximal internal carotid artery [ICA] occlusion).
FINDINGS Figure 245-1. Axial FLAIR image through the Question for Further Thought
cerebrum. There is an old left hemispheric watershed infarct 1. Can crossed cerebellar diaschisis be predicted in the acute
(arrows) with left hemispheric atrophy. Figure 245-2. Axial phase of a cerebral infarct?
FLAIR through the cerebellum. There is atrophy and smudgy
hyperintensity within the right cerebellar hemisphere, right Reporting Responsibilities
brachium pontis, and pons (arrows). This is a chronic situation, and routine reporting is sufficient.
When cerebellar atrophy coexists with chronic ischemia in
DIFFERENTIAL DIAGNOSIS Crossed cerebellar atrophy the contralateral cerebrum, crossed cerebellar diaschisis and
and diaschisis, primary cerebellar infarct, chronic infection/ atrophy should be suggested rather than invoking a separate
inflammation of the cerebellum. cerebellar insult.
DIAGNOSIS Crossed cerebellar atrophy and diaschisis. What the Treating Physician Needs to Know
Crossed cerebellar atrophy is an occasional secondary
DISCUSSION Crossed cerebellar atrophy and diaschisis finding following a substantial infarct to the contralateral
results from a substantial insult to the cerebrum, usually cerebral hemisphere
ischemic and usually in the MCA distribution. This results
in loss of neuronal input to the cortico-ponto-cerebellar tract Answer
and deafferentation injury to the contralateral cerebellar 1. In the acute phase, the cerebellar hemisphere contralateral
hemisphere. This results in atrophy of the affected cerebral to an acute cerebral infarct may show abnormal perfusion
hemisphere, and over time, the affected cerebellum evolves parameters (decreased blood flow, increased transit time)
in a similar way to the infarcted tissue ultimately leading to which are predictive of developing crossed cerebellar
atrophy. diaschisis and atrophy in the long term.
531

Case
246 CLINICAL HISTORY 52-year-old male with long-standing left cranial
nerve XII palsy.
Table 246-1 Differential Considerations for Hypoglossal

Nerve Palsy
Anatomic Segments
of the Hypoglossal
Nerve Differential
Intraaxial Glioma, demyelinating disease,
segment cerebral infarction, intracranial
hemorrhage
Cisternal Basilar artery dolichoectasia,
segment meningioma
Skull base segment Metastasis, nasopharyngeal
carcinoma, paraganglioma, nerve
sheath tumor
Figure 246-3
Extracranial segment Metastasis, carotid artery
dissection, carcinoma, infection
FINDINGS Permission to use images by Matthew White DIFFERENTIAL DIAGNOSISThe differential consider-
MD. Figure 246-1. Axial T2WI through the hypoglossal ations for hypoglossal nerve palsy are dependent on the loca-
canal. There is a heterogeneous T2 hyperintense mass tion of the lesion along the course of the hypoglossal nerve.
situated anterolaterally with respect to the left aspect See table 246-1.
of the medulla within the premedullary cistern (arrows)
that extends into the widened hypoglossal canal and ret- DIAGNOSIS Hypoglossal schwannoma.
rostyloid space (RSS) along cranial nerve (CN) XII.
Figures 246-2 and 246-3. Coronal and axial post-contrast DISCUSSION CT and MRI are both useful imaging modal-
T1WI, respectively. There is an avidly contrast-enhanc- ities for the evaluation of hypoglossal nerve dysfunction. The
ing dumbbell-shaped mass (star) in the left hypoglossal strength of CT lies in its ability to characterize a mass in rela-
canal. Single- and double-headed arrows in Figure 246-3 tion to the osseous anatomy, whereas the advantage of MRI
illustrate the normal right and expanded left hypoglossal lies in its far superior soft tissue contrast resolution. On CT,
canals, respectively. schwannomas are often isodense to brain parenchyma unless
532

Case 246 533
containing cystic areas. Schwannoma within the hypoglossal these tumors as intradural, dumbbell-shaped (demonstrating
canal may show smooth widening of the canal with preser- both an intracranial and an extracranial component), extra-
vation of the overlying cortex. For small schwannoma, CT cranial, or peripheral.
may be inadequate in characterizing its true intracranial and
extracranial extents. MRI typically demonstrates schwan- Questions for Further Thought
noma to be T1 isointense to hypointense, and mildly T2 1. How common is CN XII involvement in patients with
hyperintense without associated restricted diffusion. These neurofibromatosis type II (NF2), aka MISME syndrome
tumors enhance heterogeneously after contrast administra- (Multiple Inherited Schwannomas, Meningiomas, and
tion on both CT and MRI. Ependymomas)?
Intracranial schwannomas are benign neoplasms that arise 2. What are the treatment options for skull base hypoglossal
from cranial nerves and account for 8% of all intracranial schwannomas?
tumors. When arising from the hypoglossal nerve, symptoms
manifest as ipsilateral tongue paralysis, fasiculations, devia- Reporting Responsibilities
tion, and atrophy. Patients can also present with a suboc- Hypoglossal schwannoma involving the cisternal segment can
cipital headache, vomiting, or papilledema from increased exert mass effect on the brainstem and cerebellum resulting in
intracranial pressure due to mass effect on the brainstem and intracranial hypertension, hydrocephalus, and cerebral hernia-
cerebellum. Cranial neuropathies involving CN VII through tion, requiring urgent reporting of these critical findings.
XI may arise from adjacent compression. Histologically,
these tumors are composed of Antoni A- and Antoni B-type What the Treating Physician Needs to Know
cells, the former of which consist of densely packed, elon- Location, size, extent of tumor, and relationship to adja-
gated spindle cells, with the latter demonstrating a myxoid cent structures
consistency. Presence/absence of mass effect and cerebral herniation
The hypoglossal nerve provides motor innervation to
the intrinsic and extrinsic muscles of the tongue as well Answers
as contributing to the superior root of the ansa cervicalis, 1. Hypoglossal nerve involvement is exceptionally rare. Of
which innervates the infrahyoid strap muscles. The hypo- patients afflicted with NF2, 95% will develop vestibular
glossal nucleus is located in the medulla oblongata at the schwannomas. The next most common cranial nerve to be
level of the floor of the fourth ventricle with fibers coursing affected is the trigeminal nerve.
anterolaterally to exit the medulla at the preolivary sulcus 2. Combination of transcondylartranstubercular approach
(intraaxial segment). Nerve fibers coalesce, forming the surgical resection, stereotactic radiation, with or without
hypoglossal nerve, which travel to the entrance of the bony sural nerve grafting in patients with hypoglossal nerve
hypoglossal canal that is formed by the jugular tubercle resection.
and occipital condyle (cisternal segment). The hypoglossal
nerve subsequently travels through and exits the hypoglos- ACKNOWLEDGMENTS
sal canal at the skull base (skull base segment), after which Images courtesy of Drs. Pavani Adapa1, Arash Kamali2,
it enters the nasopharyngeal carotid space (extracranial Matthew F. Omojola1, Matthew L. White1, and Mark D.
segment). It courses anteroinferiorly and exits the carotid Keiper1. This case was originally presented as part of an edu-
space in between the internal carotid artery and jugular vein cational exhibit at the Radiologic Society of North America
at the level of the posterior belly of the digastric muscle to (RSNA) 2013 annual meeting (December 1 to 6, Chicago, IL).
the floor of the mouth. Knowledge of this detailed anatomy
is essential in describing the precise location and extent of 1
Department of Radiology, University of Nebraska Medical Center,
hypoglossal schwannomas which is necessary for surgical Omaha, NE.
planning. One surgical grading scale proposes categorizing 2
University of Medicine and Dentistry of New Jersey, Camden, NJ.

Case
247 CLINICAL HISTORY 74-year-old female found unconscious.
FINDINGS Figure 247-1. Axial NCCT through the supra- region of the circle of Willis and the lateral fissures diffus-
sellar cistern. There is diffuse hyperdensity consistent with ing into the perimesencephalic/quadrigeminal cisterns and
acute hemorrhage with some filling defects in the suprasel- up into the convexity sulci and interhemispheric fissure.
lar cistern (transverse arrows) extending into the anterior Predominant posterior fossa ASAH could be due to aneu-
interhemispheric fissure (chevron), lateral fissure on either rysm or nonaneurysmal perimesencephalic hemorrhage.
sides (vertical arrows), and around the upper pons (curved ASAH due to trauma, tumor, and coagulopathy could be
arrows). Figure 247-2. Axial NCCT through the level of the located anywhere intracranially. ASAH due to cortical
trigones. There is extension of the hyperdense hemorrhage vein and venous sinus thrombosis is mostly convexity in
into the anterior interhemispheric fissure (transverse arrows). location. Visibility of ASAH on CT depends on the size
There are fluid levels in the trigones (vertical arrows) consis- and location. The sensitivity of CT in assessing aneurys-
tent with intraventricular hemorrhage (IVH). mal ASAH has been assessed at 100% if it is done within
6hours of the ictus. However, a sizable percentage may not
DIFFERENTIAL DIAGNOSIS N/A. be visible on CT for one reason or the other after 6hours. In
this group, the subarachnoid spaces may present as effaced
DIAGNOSIS Acute subarachnoid hemorrhage (ASAH). or isodense with the brain. Some hemorrhages may also
have escaped into the ventricles resulting in subtle fluid
DISCUSSION ASAH is typically hyperdense within the fluid levels of IVH.
subarachnoid spaces on CT. The location depends on the The current standard of care is to perform lumbar punc-
cause. Causes include but not limited to ruptured saccular ture (LP) looking for red cells and xanthochromia in the
aneurysm, nonaneurysmal perimesencephalic hemorrhage, CSF. There is currency regarding performing CTA instead
trauma, ruptured arteriovenous malformation (AVM) or of LP since CTA following a negative CT has greater than
arteriovenous (AV) fistula, vasculopathy, cortical vein and 99% probability of detecting the causative aneurysms. MRI
sinus thrombosis, coagulopathy, extension of parenchymal may also be useful in this situation particularly if the his-
hematoma, and extraaxial tumors. Saccular aneurysms are tory is strongly suggestive of ASAH. Patients describe such
responsible for about 80% of nontraumatic ASAH and are symptoms as the worst headache of their lives; the thun-
usually located along the vertebro-basilar system and in the derclap headache. If an aneurysm is found, a decision is then
534

Case 247 535
made about how best to treat it. About 10% of nontraumatic What the Treating Physician Needs to Know
subarachnoid hemorrhage (SAH) is due to the nonaneurys- Location, size, complications, and other associations
mal perimesencephalic hemorrhage. Is it safe to perform LP if the initial imaging is negative?
How do we best find out the cause of the hemorrhage?
1. How do you investigate the causes of SAH? Answer
1. MRI of the brain without and with contrast may be impor-
Reporting Responsibilities tant when the SAH is not detected on CT. Hyperintense
This is an emergent situation requiring direct reporting. The SAS on FLAIR, fluidfluid level in the ventricles on GRE
location is important and may suggest the cause of the hem- along with leptomeningeal enhancement may suggest
orrhage. Complications such as IVH, hydrocephalus, infarcts SAH. MRI may also show AVM, tumors, or thrombosed
due to spasms, and other associations such as masses, paren- veins/sinuses in the appropriate situation. Vascular causes
chymal hematoma, cortical and sinus thrombosis, visible of SAH are best demonstrated by CTA, MRA, or MRV.
AVM, or fistulas should be reported. Suggestions for further DSA is usually reserved as a problem solving tool and for
workup are always necessary. endovascular treatment.

Case
248 CLINICAL HISTORY 24-year-old male complains of left arm activity
where he has tension and is unable to move his arm for a few seconds to a
few minutes. These episodes have become more frequent.
536

Case 248 537
this instance. AO is heterogeneously hyperintense on FLAIR

and T2WI. There is a variable mass effect and surrounding
edema. Post-contrast T1WI demonstrates patchy enhance-
ment. Ring enhancement is unusual and thought to repre-
sent a poor prognosis. MRS shows significantly high Cho/
Cr ratio while perfusion shows a high rCBV. Lower ADC
values have been found to be predictive of higher-grade AO.
The parasagittal frontal location in this case may be con-
fused with anterior cerebral artery (ACA) territory ischemic
infarct. However patchy and nonuniform restricted diffusion
suggests otherwise. Metastatic lesions are usually necrotic
with ring contrast enhancement and considerable surround-
ing edema. Abscess usually restricts diffusion centrally,
has a rim of elevated diffusion, and has a smooth thin ring
enhancement within the surrounding edema. It is unusual to
Figure 248-5
see hemorrhage or calcifications within areas involved by
status epilepticus. This mass must be differentiated from a
group of tumors that are well circumscribed gliomas, and
this includes DNETs, ganglioglioma, PXA, and lobar pilo-
FINDINGS Figure 248-1. Axial FLAIR MRI through the cytic astrocytomas. All these tumors usually present in a
level of the corpus callosum. There is a mildly heteroge- younger adult population. DNET is usually a bubbly cortical
neous hyperintense mass in the medial right frontal lobe tumor while PXA is cortical based and may have a dural tail
that is diffusely infiltrating the cortex as well as the adjacent and scallops the inner table.
white matter (WM) and corpus callosum. It is effacing the AO is a designated WHO grade III tumor usually presents
adjacent sulci and the right lateral ventricle with minimal in the fifth decade. It may develop as a primary tumor or a
right to left midline shift. Figure 248-2. Axial post-con- progression from lower-grade OD. AO is rare in the pedi-
trast T1WI through the mass. There are multifocal areas of atric population. AO is cellular and mitotically active and
enhancement within the mass (arrows). Figure 248-3. Axial usually demonstrates microvascular proliferation. Necrosis
GRE through the mass. There is a small area of hypoin- may be present. The survival of AO is significantly less than
tensity (arrow) anterolaterally within the mass consistent low-grade OD. Increase in tumor size within 6 months is pre-
with calcification or hemorrhage. Figure 248-4. Axial ADC dictive of poor outcome. Completely resecting enhancing tis-
map through the mass. There is relative restricted diffusion sue independently improves outcome. This finding supports
(arrows) in the mid and posterior aspect of the mass involv- aggressive resection and may impact treatment planning for
ing WM with elevated ADC values predominantly anteri- patients with AO. Other clinical features that indicate poor
orly and at the periphery posterior of the mass involving prognosis are older age, the presence of focal neurologic def-
the GM. Figure 248-5. Photomicrograph shows oligoden- icits at presentation, postoperative Karnofsky performance
droglioma with slight nuclear pleomorphism and atypia and score (KPS) < 80, and partial resection.
several mitoses (H&E stain).
DIFFERENTIAL DIAGNOSISOligodendroglioma, infarct, 1. What are the important genetic characteristics of AO?
metastatic disease, infection, status epilepticus, dysembryo-
plastic neuroepithelial tumor (DNET), ganglioglioma, pleo- Reporting Responsibilities
morphic xanthoastrocytoma (PXA). Direct reporting is important in the presence of a large or
confusing lesion. Presence of significant hemorrhage or her-
DIAGNOSIS Anaplastic oligodendroglioma (AO WHO III) niations makes direct reporting more urgent. Differentiating
AO from its various mimics may be a challenge. Advanced
DISCUSSION AO is predominantly frontal lobe in loca- imaging may be confusing and as shown is rarely useful in
tion and very rare in the posterior fossa. AO is a cortically making definitive pronouncement. The occasional leptomen-
based tumor showing considerable heterogeneity of its den- ingeal oligodendrogliomatosis has been reported as a rare
sity on CT due to its various solid, cystic, hemorrhagic, and complication and is worth reporting if present.
calcified components. The cysts are hypodense on CT while
the calcifications are hyperdense. Mass is heterogeneous and What the Treating Physician Needs to Know
hypointense on T1WI except where blood products may pro- Location and size of contrast enhancement which may
duce T1 hyperintensity. Areas of hemorrhage or calcifica- influence operative technique
tions are usually hypointense on GRE. There may be areas Are there findings indicating a need for lumbar puncture
of restricted diffusion within the lesion as demonstrated in such as leptomeningeal disease?

538 Case 248
Is craniospinal MRI necessary if there is leptomeningeal in AO is strongly correlated with 1p and 19q codeletion.
enhancement? IDH1 mutations also correlate with 1p/19q deletion and
Are there significant herniations? MGMT promoter methylation. Loss of heterozygosity of
How useful is advanced imaging in confirming the diagnosis? 10q likely leads to shortened survival.
Answer
1. Codeletion of 1p and 19q predicts increased chemosensi-
tivity and a better prognosis for AO. O6-methylguanine-
DNA-methyltransferase (MGMT) promoter methylation

Case
249 CLINICAL HISTORY 2-year-old male being evaluated for hydrocephalus.
FINDINGS Figure 249-1. Axial T2WI through the thalami. the centrum semiovale, respectively. Only small parafalcine
The frontal lobes are absent and replaced by cerebrospinal strips of frontal parietal lobes are present bilaterally (arrows)
fluid (CSF) (stars). There is a slit third ventricle between the with the supratentorial space occupied by CSF. The remnants
thalami (transverse arrow). The superior vermis and cerebel- of the frontal lobes display rounded margins. It is impossible
lum are normal (chevrons). Figures 249-2 and 249-3. Axial to identify convexity brain mantle. Large signal void artifact
T2WI through the expected levels of the lateral ventricles and posteriorly on the right in Figures 249-1 and 249-2 is from
539

540 Case 249
ventriculoperitoneal (VP) shunt valve. Figure249-4. Coronal on the basis of a destructive process involving the cerebral
T2WI through the middle cranial fossae. Only rudimentary hemispheres probably due to bilateral ICA occlusions and
medial temporal lobes are visualized (arrows). Posterior subsequent destruction of the cerebral hemispheres. The
fossa structures are present and grossly normal (stars). The posterior circulation is intact hence posterior fossa struc-
supratentorial space is filled with CSF except for small knob- tures and the occipital lobes are normal. These children pres-
bing of frontal and parietal lobes around the falx cerebri. ent with large heads that easily transluminate. They perish
mostly within the first year of life. Attempt is made to control
DIFFERENTIAL DIAGNOSISHydrocephalus, schizen- the macrocephaly by shunting.
cephaly, hydranencephaly, alobar holoprosencephaly.
DIAGNOSIS Hydranencephaly. 1. Is it definite that early in utero occlusion of ICA causes
hydranencephaly?
strating all the features of hydranencephaly. There is essen- Reporting Responsibilities
tially absent cerebral hemispheres except for small amount Direct reporting may be necessary, although prenatal diag-
of brain tissue along the falx cerebri and posteriorly in nosis may have been made. In any case, it is important to be
the region of the occipital lobes. The entire supratentorial able to distinguish this entity from its mimics.
region is full of CSF without identifiable convexity brain
mantle. The falx cerebri and interhemispheric fissure are
always present. The posterior fossa structures are intact.
The thalami are present but the basal ganglia could be rudi- Certainty of the diagnosis and exclusion of the mimics
mentary. There is macrocephaly. Angiographic images in Absence of cortical brain mantle distinguishes hydranen-
this population usually show severely hypoplastic supracli- cephaly from hydrocephalus; hydrocephalus is treatable,
noid internal carotid arteries (ICAs). Even in severe hydro- while hydranencephaly is not
cephalus, it is possible to identify thin cerebral mantle on
MRI. Large open lip schizencephaly is lined by cortical Answer
gray matter (GM). In hydranencephaly, the margin of the 1. No, it is not definite but is the most plausible explanation
rudimentary brain lining the CSF is gliotic. The midline on the basis of experimental occlusion of ICA in mon-
structures of the falx and interhemispheric fissure are not key fetuses resulting in hydranencephalic changes. Other
formed in alobar holoprosencephaly. There is usually a theories of etiopathogenesis of hydranencephaly include
brain mantle particularly anteriorly. congenital infections such as toxoplasmosis and viral
Hydranencephaly is a rare congenital abnormality that infections resulting in destruction of the cerebral hemi-
is diagnosable in utero. The diagnosis can be made by US. spheres, maternal exposure to toxins such as carbon mon-
It is usually sporadic but has been reported in association oxide and butane gas resulting in fetal hypoxic ischemic
with Fowler syndrome, trisomy 13, renal aplastic dysplasia, changes and subsequent brain liquefaction, thromboplastic
and polyvalvular developmental heart defect. It has no gen- material from a deceased monochorionic twin resulting in
der or racial preferences. The pathogenesis is believed to be brain destruction, and an extreme form of leukomalacia.

Case
250 CLINICAL HISTORY 21-month-old male with bilateral mixed hearing
loss.
FINDINGS Figure 250-1. Lateral skull X-ray. There is a the calvarial defect in Figure 250-1), encasing, and narrow-
well-circumscribed, lytic lesion of the parietal calvarium ing the confluence of venous sinuses (arrow). Figure 250-5.
with no marginal sclerosis (anterior arrow). There is a second Axial FLAIR through the cerebellum demonstrates T2 hyper-
more geographic lesion near the torcula (posterior arrow). intense foci involving the cerebellar dentate nuclei bilaterally
Figure 250-2. Coronal T2WI through the parietal lesion. The (arrows).
lesion shows beveled margins with involvement of the outer
table and sparing of the inner table (arrow). Figure 250-3. DIFFERENTIAL DIAGNOSIS Metastatic disease, infection,
Coronal post-contrast image shows the bony replacement by epidermoid, dermoid, leptomeningeal cyst, mastoiditis, rhab-
mildly enhancing soft tissue (arrow). Figure 250-4. Sagittal domyosarcoma, granulomatous disease, that is, sarcoidosis.
post-contrast T1WI demonstrates enhancing soft tissue (cir-
cle) with bony destruction at the torcula, (corresponding to DIAGNOSIS Langerhans cell histiocytosis (LCH) of the bone.
541

542 Case 250
Figure 250-5
DISCUSSION LCH, formerly known as histiocytosis X, is of cases, most often in patients with multisystem disease.
a systemic disease that typically manifests in the first decade The common sites of CNS disease include:
of life. There is a wide spectrum of presentation, from the 1. Skullmost commonly the temporal bones, calvarium,
mild solitary eosinophilic granuloma of bone in adolescent and orbits
and young adults to more severe, disseminated forms as in 2. Dura
Hand-Schuller-Christian disease and Letterer-Siwe disease 3. Hypothalamus and pituitary infundibulum
(10% of LCH cases). CT is helpful to assess the lytic lesions 4. Bony vertebral column
of the calvarium, temporal bones, and orbits. The calvarial 5. Cerebral and cerebellar parenchyma
lesions are classic, sharply marginated lytic lesions with bev- 6. Spinal cord
eled margins and lack of sclerosis. The temporal and orbital Symptoms depend on the site(s) of involvement. For
lesions may demonstrate geographic bone destruction with example, osseous lesions may present as a calvarial mass,
soft tissue mass. Mastoid disease is often bilateral. MRI, temporal bone lesions may lead to hearing loss and chronic
due to its superior contrast resolution, is better at detecting drainage from ear, or involvement of the bony orbit may
parenchymal involvement, nodular thickening of the menin- result in proptosis. Headaches may reflect dural infiltration
ges, pituitary stalk thickening, and soft tissue masses associ- and diabetes insipidus (DI) is common in patients with hypo-
ated with destructive bony lesions. Involvement of the dural thalamic and pituitary infundibular involvement. Cerebellar
venous sinuses, as in our case, is better depicted by MRI. involvement often causes ataxia and dysmetria, while cere-
Identification of lytic bony lesions and/or other sites of central bral lesions reflect the functional areas involved.
nervous system (CNS) involvement in children should raise Hand-Schuller-Christian disease (15% of LCH cases) is
concern for LCH. Metastatic disease is uncommon in younger the chronic, disseminated form that presents in younger chil-
children, especially in the absence of a known primary. dren (1 to 5 years of age) with the classic triad of DI, exoph-
Patients with infection and sepsis tend to be much sicker with thalmos, and destructive bone lesions. Letterer-Siwe disease
more perilesional edema than is seen with LCH. Sarcoidosis (10% of LCH cases) is the acute disseminated form that pres-
and other granulomatous diseases are less common in chil- ents in infants with fever, anemia, rash, hepatosplenomegaly,
dren and present in older patients with predominantly dural- and thrombocytopenia.
based/meningeal disease; bony lesions are uncommon.
The exact etiology of LCH is unknown but may be due
to immune system dysfunction rather than a truly neoplastic Questions for Further Thought
process. There is proliferation of antigen-presenting d endritic 1. What is the choice of therapy?
cells of marrow origin. The CNS is involved in 10% to 50% 2. What is the natural history and prognosis?

Case 250 543

Direct reporting is important in view of the aggressiveness 1. Choice of therapy depends on symptoms, location, and
of LCH and some rather life-threatening differentials. It is extent of disease. Solitary eosinophilic granulomas have
important to identify and report the different CNS sites that the best prognosis and may spontaneously resolve. The
can be involved with histiocytosis and document solitary options for treatment include observation, excision/
versus multifocal disease due to the impact on prognosis. curettage of bony lesions, sclerotherapy, and radiation
For temporal bone and mastoid disease, differentiation from with or without chemotherapy is reserved for multifo-
mastoiditis and rhabdomyosarcoma is important. cal, malignant forms. Patients with DI may need supple-
mental vasopressin in addition to chemotherapy and/or
What the Treating Physician Needs to Know radiation.
Extent of CNS involvement including solitary/multifocal 2. The natural history and prognosis vary, depending on the
disease, calvarial lesions, mastoid/orbital disease, pituitary age at presentation and extent of involvement. Solitary
stalk thickening, brain parenchymal involvement, verte- disease has the best prognosis. Morbidity and mortality
bral lesions, as well as cord involvement are higher with multifocal and systemic disease. Prognosis
Evidence of response of CNS lesions to treatment, espe- remains unpredictable, as it may assume a more aggres-
cially to radiation and chemotherapy sive form with time.

Case
251 CLINICAL HISTORY Adult male sustained a motor vehicle collision.
FINDINGS Figure 251-1. Axial NCCT through the body of hemorrhagic conversion of ischemic infarct, AVM or aneu-
corpus callosum (CC). There is a focal hyperdensity anteriorly rysm rupture, surgical intervention such as catheter place-
to the right in the body of the CC (arrow). Figure 251-2. Axial ment and hemorrhagic metastasis. Initial evolution is that
NCCT through the same level about 1 month later. There is of development of surrounding edema and local mass effect
persistent but smaller hyperdensity in same location. with subsequent resolution which can be slow taking up to
several weeks. Depending on the size, the imaging outcome
DIFFERENTIAL DIAGNOSIS Hematoma, calcification. could be a tiny residual hyperdensity, a hypodense focus, or
complete disappearance without a trace or suggestion of a
DIAGNOSIS Corpus callosum hematoma (CCH). prior hematoma in the vicinity. Resolved large hematoma
may result in focal atrophy.
DISCUSSION CCHs are easily recognizable on NCCT. It could be difficult distinguishing a small hemorrhage
They are usually hyperdense, and in the acute phase there from calcification. HU measurement is one way, but there is
may be no surrounding hypodensity or edema. The large an overlap where the calcification may not be dense enough
ones are usually not mistakable. The small hematomas are to measure above 100 HU. Most hemorrhages will measure
the difficult ones to identify. They are usually without signif- less than 100 HU (active bleed is about 130 HU). A short fol-
icant mass effect or associated intraventricular hemorrhage. low-up will usually solve the problem. Hemorrhage evolves
In the context of trauma, presence of CCH denotes a grade 2 while calcification is static.
diffuse axonal injury (DAI). It is more common in the pos- Clinical presentation of CCH is variable and nonspecific.
terior body and splenium with anterior location suggesting This could include loss of consciousness, focal neurologic
a more severe injury. It could be delayed with the initial CT deficit or coma if DAI is severe. Behavioral changes have
showing a normal appearance. Brain swelling, other paren- also been reported in isolated CCH. Treatment is usually
chymal and extraaxial hematomas, and herniations could be supportive.
present depending on the severity of the injury. Traumatic
CCH could be due to laceration by the rigid falx and/or Question for Further Thought
rotational shearing force. However, causes of isolated CCH 1. Is there a place for MRI in the evaluation of CC traumatic
mayalso include spontaneous hemorrhage, coagulopathy, hemorrhage?
544

Case 251 545

Direct reporting is necessary in all hemorrhages. The size, loca- 1. MRI is most suited to evaluation of CCH particularly
tion, and associated injuries elsewhere intracranially which because of its multiplanar capability and superior tissue
could include, intraventricular hemorrhage (IVH), subarachnoid characterization. CC hematoma is present in about 47%
hemorrhage (SAH), extraaxial hematomas, and other shearing of nonfatal traumatic brain injury. In the acute stage, both
petechial hemorrhages and contusions should be documented. DWI and GRE are very effective in visualizing CCH. It is
usually heterogeneous on DWI and hypointense on GRE.
What the Treating Physician Needs to Know Subsequently, all other sequences reveal the hemorrhage
Size and location in variable intensity signals depending on the age of the
Associated injuries and complications hematoma. Other parenchymal hemorrhages not seen on
Traumatic CCH denotes a grade 2 DAI; a rather serious CT are more likely to be visualized on MRI.
head injury
MRI may demonstrate many more contusion and hemor-
rhagic foci

Case
252 CLINICAL HISTORY Infant born with lumbar myelomeningocele.
Figure 252-2
Figure 252-1
FINDINGS Figure 252-1. Axial NCCT through the poste-

rior fossa at the level of the fourth ventricle. There is efface-
ment of the fourth ventricle. There is bilateral cerebellar
tongues (arrows) wrapping around the brainstem. Figure
252-2. Axial NCCT through the midbrain. There is mild
tectal beaking (arrowhead) and bundling of the cerebellum
(transverse arrows) as it pushes up through the incisura.
There is scalloping of the inner table. Figure 252-3. Axial
NCCT through the level of the centrum semiovale showing
deficiency of the falx with interdigitation of the cerebral gyri
across the midline (arrows). Mild ventriculomegaly is pres-
ent (stars).
DIFFERENTIAL DIAGNOSISRhombencephalosynapsis
(RES), Chiari II malformation (CIIM), cerebellar mass.
DIAGNOSIS Chiari II malformation (CIIM).
DISCUSSION While MRI is the modality of choice for

evaluation of CIIM, the diagnosis could easily be made on
CT. Changes demonstrable on CT include a small posterior
Figure 252-3
546

Case 252 547
fossa, deformity with narrowing of the fourth ventricle, cer- should be recommended. Complete craniospinal evaluation
ebellar wrap around the brainstem, beaking of the tectum, by MRI is necessary to document other anomalies.
and towering of the cerebellum as it pushes through the
tentorial incisura. Crowding of the foramen magnum would What the Treating Physician Needs to Know
indicate descent of posterior fossa structures. Supratentorial CT is not sufficient for evaluation of the patient with CIIM.
abnormalities include dysgenesis of the falx with cerebral Gross changes are demonstrable. Associated parenchymal
tissue interdigitating across the midline, ventriculomegaly, changes are best demonstrated by MRI
scalloping of the inner table. Lack of continuity of cerebel- Bone changes are clearly visualized and better demon-
lar white matter and folia across the midline exclude RES, strated on CT however
while preservation of white gray matter interface excludes Presence of hydrocephalus may be an indication for cere-
cerebellar mass. The small fourth ventricle, the tectal brospinal fluid (CSF) diversion
beaking, the cerebellar tongues around the brainstem, and
crowding of the incisura are definitive enough for the diag- Answer
nosis of CIIM. Thoracic or lumbar myelomeningocele is
1. Cranial vault changes include the lacunar skull with scal-
always present.
loping and thinning of the inner table with focal deficien-
cies and multiple ridges. These changes are supposedly
Question for Further Thought due to bone dysgenesis rather than raised intracranial
1. What are the cranial vault and skull base bony changes pressure, and they gradually disappear as the child grows
inCIIM? and may be replaced by vault hyperostosis as a result of
craniosynostosis following hydrocephalus shunting. The
Reporting Responsibilities posterior petrosal surface and the clivus tend to show mild
Routine reporting is sufficient unless there is hydrocephalus. concavity, while the foramen magnum is always gaping
MRI remains the best way to evaluate this anomaly and it to allow the descent of posterior fossa structures.

Case
253 CLINICAL HISTORY 18-year-old female with seizures.
Figure 253-1
Figure 253-2
FINDINGS Figure 253-1. Sagittal T1WI through the level hyperintensity suggesting mild transependymal cerebrospi-
of the aqueduct. There is dilation of the third and lateral ven- nal fluid (CSF) flow. Figure 253-3. Coronal T2WI through
tricles (stars). The aqueduct is closed (arrow). The fourth the aqueduct. There is dilation of the third and lateral ventri-
ventricle is normal (chevron). Figure 253-2. Axial FLAIR cles. The aqueduct is barely visible (transverse arrow). There
through the body of the lateral ventricles. There is dilation of is a right parasagittal parietal burr hole with underlying
the lateral ventricles (stars). There is very thin p eriventricular encephalomalacia (vertical arrows) from prior intervention.
548

Case 253 549
Figure 253-4. Axial post-contrast T1WI through the aque- evaluation of aqueductal stenosis. This usually reveals no
duct. There is no mass or abnormal contrast enhancement in flow within the duct. Clinical presentations usually includes
the brainstem or around the aqueduct. headache, visual problems, and vomiting. These patients are
usually young with smaller ventricular size. However the
DIFFERENTIAL DIAGNOSISObstructive hydrocepha- elderly patient with aqueductal stenosis usually presents with
lus, communicating hydrocephalus, volume loss. normal pressure hydrocephalus (NPH) symptoms of memory
loss, ataxia, and incontinence with invariably larger size of
DIAGNOSIS Hydrocephalus due to aqueductal stenosis. the ventricles. Treatment options include endoscopic third
ventriculostomy (ETV), ventriculoperitoneal shunt, and sur-
DISCUSSION Both CT and MRI are useful tools in the gical resection of tumors when feasible.
evaluation of hydrocephalus. MRI does a better job in the
initial evaluation because of its multiplanar capability which Question for Further Thought
would allow better visualization of midline structures. The 1. How does the ETV work?
excellent tissue characterization ability of MRI also lends
itself particularly in the brainstem where significant artifacts Reporting Responsibilities
tend to occur on CT. CT is however sufficient for follow- Direct reporting is essential. Negative changes on follow-up
up of hydrocephalus once the diagnosis has been made and should also be called directly to the physician.
treatment initiated. Hydrocephalus is abnormal dilation of
the ventricles. It may be obstructive or communicating. The
level of obstruction is visible most of the time at imaging.
Intraventricular obstruction could be within the ventricles, Is it obstructive or communicating hydrocephalus
at the foramina of Monro, and at the aqueduct of Sylvius. Level of obstruction and cause of obstruction if visible
Obstruction could be congenital, inflammatory, neoplas- Adverse changes on follow-up which may include extra-
tic, or due to extrinsic compression of the CSF pathways. axial collections, hemorrhage, overshunting, or nonfunc-
Extraventricular obstructions are generally not visualized; tioning of the shunt
such cases where the entire ventricular system is dilated is
known as communicating hydrocephalus. Volume loss is Answer
usually associated with dilated subarachnoid spaces which 1. ETV is a form of CSF diversion that has become the
is not present in this case. mode of treatment for proven aqueductal stenosis even
Aqueductal stenosis could be due to intraventricular hem- for those who have positive CSF flow in the aqueduct
orrhage, infection with ventriculitis, neoplastic particularly by cine phasecontrast MR flow study. For such patients
tectal, midbrain or pineal region mass with compression with positive flow study to qualify for ETV, they must
of the aqueduct, idiopathic and/or congenital due to a web, show disproportionate enlargement of the third and lat-
aqueductal forking, or fibrosis. Structural neuroimaging with eral ventricles compared with the fourth ventricle. The
MRI could uncover many of the causes, but the idiopathic third ventricular floor is approached through transven-
cause remains an enigma. Some cases of idiopathic aque- tricular endoscopy, and a communication is created to
ductal stenosis have turned out to be due to tectal glioma the suprasellar or basal cistern through the third ventricu-
particularly when the initial evaluations have been done by lar floor. This provides a diversion of the CSF from the
CT. Sagittal cine phase MR flow study is very useful in the aqueduct.

Case
254 CLINICAL HISTORY Newborn with facial abnormality on clinical
examination.
FINDINGS Figure 254-1. Coronal T2WI through the Figure254-4. Axial CECT through the orbits. There is a thin
orbits. There is a cystic mass within the right orbit, medial communication (white arrow) between the orbital cyst and
to the globe, and medial rectus muscle (arrow). Figures the nasal abnormality.
254-2 and 254-3. Sagittal T2WI and axial FLAIR, respec-
tively, through the orbits. There is abnormal protrusion DIFFERENTIAL DIAGNOSIS Mucocele, dermoid/epider-
of brain parenchyma into the nasofrontal region (arrows). moid, dacryocystocele, meningocele.
550

Case 254 551
DIAGNOSIS Orbital meningocele with nasofrontal menin- Question for Further Thought
goencephalocele. 1. How do you differentiate a skull base meningocele from
other common skull base cystic lesions?
DISCUSSION Cephalocele defines any abnormal protru-
sion of intracranial contents through cranial vault or skull Reporting Responsibilities
base defects and is of several types. A cephalocele contain- Direct reporting is essential as the complications could be
ing meninges and cerebrospinal fluid (CSF) alone is called a catastrophic if left untreated. Verify the cystic nature of the
meningocele. A cephalocele containing CSF and brain tissue lesion and accurately describe the location as well as asso-
is called a meningoencephalocele, while an atretic cephalo- ciated mass effect. Make an effort to identify the site of
cele contains only meninges and degenerated brain tissue the bony defect. Thin-section CT may be required as the
without CSF. Typical CT changes of a skull base meningo- site of communication with the intracranial space can be
cele include CSF density structure protruding through the extremely small.
skull base or the orbitofacial region, identifiable defect in the
adjacent bone, and displacement of normal structures such as What the Treating Physician Needs to Know
the globe and extraocular muscles as in this case. CT findings
Meningoceles are benign and usually easily corrected.
of meningoencephalocele on the other hand tend to include
However, when congenital, they are often associated with
some brain material with or without CSF as in the nasofron-
an underlying syndrome or condition which may be far
tal component of the present cephalocele. 3D bone recon-
more problematic
struction may define the bony defect perfectly. Similarly,
Location and size
meningocele follows CSF intensity on all MRI sequences
Content if more than CSF
within the abnormal protrusion of the meninges. Brain mate-
rial is present in meningoencephalocele.
Cephaloceles can be congenital or posttraumatic/post- Answer
surgical in nature. Typical locations include the anterior 1. Mucoceles are typically associated with a paranasal
skull base, orbitofrontal, occipital, and parietal regions. sinus and show evidence of chronic bony remodeling/
Ameningocele may be occult and asymptomatic or may expansion. An epidermoid may be suspected based on
produce symptoms related to mass effect. As with any static characteristic high signal on DWI. Dermoids may show
fluid collection, meningoceles are susceptible to infection. characteristics of fatty material on CT and MRI. A menin-
Meningoceles are benign and usually easy to correct at sur- gocele should follow CSF characteristics on any imaging
gery. Meningoencephalocele on the other hand may be a sequence. If a bony defect can be identified, the diagnosis
little more complex. When congenital, however, cephalo- should be offered with confidence.
celes may herald the presence of a more serious underlying
syndrome or condition, as in the case presented here.

Case
255 CLINICAL HISTORY 49-year-old female on follow-up for lesion
discovered incidentally.
Figure 255-4
FINDINGS Figure 255-1. Axial NCCT through the frontal

horns of the lateral ventricles. There is a round smooth-walled
hyperdense 1.2-cm mass at the level of foramina of Monro
(arrow). There is no hydrocephalus. Figure 255-2. Axial
T2WI through the mass. There is a smooth, round relatively
Figure 255-3 isointense (to gray matter [GM]) mass occupying the region
552

Case 255 553
of foramina of Monro (arrow). Figure 255-3. Axial FLAIR. Symptomatic colloid cyst may present with sudden col-
There is a homogeneous hyperintense mass at the foramina lapse and death due to acute obstructive hydrocephalus.
of Monro. Figure 255-4. Coronal post-contrast T1WI through Headache that tends to be positional is a common presenta-
the mass. The mass is isointense without contrast enhance- tion. Most are found in the third to fifth decades of life with
ment. There is a thin rim of contrast enhancement considered few cases described in the pediatric population. There are
to be within vascular structures or the choroid plexus. Mass choices when it comes to treatment. These include watchful
hangs down from the septum pellucidum. waiting, cerebrospinal fluid (CSF) diversion surgery, open
surgical removal, or simple endoscopic and stereotactic
DIFFERENTIAL DIAGNOSIS Colloid cyst, choroid plexus aspiration of the cyst. The imaging pattern has been cor-
papilloma (CPP), choroid plexus carcinoma (CPC), pilocytic related with the ease of percutaneous aspiration. The hyper-
astrocytoma, subependymal giant cell astrocytoma (SGCA). dense lesions on CT and hypointense lesions on T2WI are
usually difficult cysts to aspirate because of the high viscos-
DIAGNOSIS Colloid cyst of the third ventricle. ity of the cyst contents. Complete resection is curative but
could leave 30% of the patients with persistent hydrocepha-
DISCUSSION Colloid cysts are hyperdense on NCCT in lus. Surgical complications may include cyst recurrence,
about 70% with the rest isodense or hypodense. They are epilepsy, venous infarctions, and memory problems from
located at the level of foramina of Monro appearing to hang fornix injury.
down from the septum pellucidum, with a size of a few milli-
meters to 4 cm. Colloid cysts usually do not contrast enhance. Question for Further Thought
Peripheral thin rim enhancement is generally ascribed to 1. What is the content of the colloid cyst?
vascular or choroid plexus enhancement. Hydrocephalus
may or may not be a feature. The MRI features are variable.
Colloid cysts could be isointense, hypointense, or hyperin-
Direct reporting is necessary in colloid cyst since it could
tense on T1WI. Most are isointense to hypointense on T2WI
cause acute hydrocephalus. Presence of acute hydrocepha-
and hyperintense on FLAIR. They generally do not contrast
lus makes direct reporting more urgent. The density pattern
enhance. Contrast-enhancing mass in similar location should
on CT and intensity pattern on MR should be accurately
suggest alternate diagnosis such as xanthogranuloma or cho-
described as this could influence management options.
roid plexus metastasis. Hydrocephalus may or may not be a
feature. CPP, CPC, SGCA, and pilocytic astrocytoma all tend
to contrast enhance and occur remotely from the foramen of What the Treating Physician Needs to Know
Monro and generally grows to enormous size before detection. Size and location
Colloid cyst of the third ventricle is a rare intracranial Density or intensity pattern depending on the imaging
mass accounting for about 1% of all intracranial neoplasm technique
and 15% to 20% of intraventricular masses. The origin of Presence or otherwise of hydrocephalus
colloid cyst is a topic of debate with most regarding it as
originating from the neuroepithelium. There is also a sug- Answer
gestion that it may have origin similar to Rathke cleft cyst 1. It is usually assumed that there is mucin or colloid within
and that both entities represent the same lesion in different the cyst. Presence of cholesterol, cellular debris, and
locations. Generally there is a single layer of columnar epi- hemorrhage within the cyst has been reported. Chemical
thelium containing mucinous material. Most are discovered irritation by the cyst contents may lead to xanthogranulo-
by chance and are asymptomatic. matous reaction in the ventricular wall.

Case
256 CLINICAL HISTORY 52-year-old female undergoing treatment for
metastatic melanoma with dementia-like symptoms, including personality
changes and cognitive dysfunction.
FINDINGS Figure 256-1. Axial non-contrast CT through

the corona radiata/centrum semiovale. There are diffuse
hypodensities in bilateral cerebral white matter (WM) (star).
Figures 256-2 and 256-3. Axial T2WI and corresponding
FLAIR, respectively, through the corona radiata. There is
confluent WM hyperintensity extending from the ventricular
wall to the subcortical WM (star). There is minimal cortical
volume loss.
DIFFERENTIAL DIAGNOSISLeukoaraiosis, radiation-

induced leukoencephalopathy (RIL).
DIAGNOSIS RIL.
DISCUSSION This is a case of RIL 1 year after whole-

brain irradiation. The history is important in making the
diagnosis. CT and MRI are both capable of showing the
changes of RIL but MRI is superior in accurately visualizing
subtle WM changes and the extent of overt changes. These
changes could be acute or delayed for several years. Imaging
changes range from localized or diffuse WM hypodensity
on CT or localized and diffuse T2 hyperintensity on MRI
to frank necrotic changes with ring, and irregular contrast
enhancement that may not be distinguishable at imaging
Figure 256-3 from the original tumor. Focal necrosis has the CT and MR
554

Case 256 555
appearance of a mass lesion with surrounding edema and Reporting Responsibilities

raised intracranial pressure. The confluent WM changes Direct reporting is essential when any new changes are seen
generally do not contrast enhance. SWI may show punctate on follow-up imaging in the oncology population. Any acute
or irregular hypointensities considered to represent either or focal finding such as stroke or focal necrosis and/or evi-
microhemorrhages or telangiectasia. Mineralizing microan- dence of tumor recurrence should be reported. Presence of
giopathy is common in patients receiving whole-brain irra- brain swelling or mass effect should be mentioned.
diation generally accompanied by chemotherapy, especially
via intrathecal delivery. These brush-like calcifications are What the Treating Physician Needs to Know
generally seen at the graywhite matter junctions or in the Acute findings to explain clinical presentation apart from
basal ganglia and are discussed elsewhere in this book. RIL (stroke, disease progression/recurrence)
Radiation injury following whole-brain radiation therapy Suggestion for further evaluation such as perfusion and
can occur acutely after treatment or several years later. The MR spectroscopy particularly in necrotic lesions
acute form reflects edema and typically resolves with ste-
roids. RIL is the delayed form of injury, occurring years after Answers
treatment and is a result of injury to the microvasculature. 1. Microhemorrhages, mineralizing angiopathy, radiation-
The pathologic correlates include demyelination, coagulation induced cavernous angioma, and/or capillary telangiec-
necrosis, vascular sclerosis, and vasculitis. It has also been tasia.
found that the risk of leukoencephalopathy in patients treated 2. Yes. Patients with underlying leukoaraiosis are at higher
with radiosurgery alone for brain metastases is significantly risk for developing RIL.
lower than that for patients treated with whole-brain radia-
tion. RIL can be clinically silent but can also cause confusion,
personality change, memory impairment, and overt dementia.

1. What are the possible causes of signal loss demonstrated
on SWI?
2. Are patients with preexisting leukoaraiosis at higher risk
for RIL?

Case
257 CLINICAL HISTORY 15-year-old male with persistent and chronic headaches.
FINDINGS Figure 257-1. Axial FLAIR through the thal- preserving the integrity of the graywhite matter interface
ami. There is bilateral diffuse enlargement and homogeneous between the thalami and the adjacent posterior limbs of the
hyperintensity of the thalami. Figure 257-2. Axial T1WI internal capsule for long time. All the differentials have
through the thalami. There is non-contrast-enhancing isoin- involvement of other parts of the brain outside the thalami.
tensity of the thalami. There is no hydrocephalus. Bithalamic gliomas are extremely rare tumors of the
CNS, affecting children and young adults typically present-
DIFFERENTIAL DIAGNOSISEncephalitis, mitochon- ing with behavioral compromise ranging from personality
drial encephalopathy, acute disseminated encephalopathy changes to dementia (more common in adults). They are
(rare without white matter involvement) or acute necrotiz- classified as low-grade tumors (WHO II). However, the
ing encephalopathy of childhood, astrocytoma, germinoma outcome is very poor. Treatment is often radiation and che-
(very rare). motherapy given their deep location is not easily amenable
to surgery. For a definitive diagnosis, biopsy is required.
DIAGNOSIS Primary bilateral thalamic astrocytoma. There is no logical explanation for bilateral involvement
as no evidence exists for direct extension across the massa
DISCUSSION Bilateral thalamic astrocytoma results in intermedia or other anatomic pathways. Infrequently, pineal
symmetrical and diffuse enlargement of the thalami, show- region tumors, particularly germ cell neoplasms, may infil-
ing homogeneous T2-weighted and FLAIR hyperintensity, trate bilateral thalami (a distinctive MRI finding might be
T1WI isointensity, and generally no enhancement. These restricted diffusion in these).
tumors tend to be of low grade when initially detected. MRS
shows an elevated creatine-phosphocreatine peak, normal-to- Questions for Further Thought
low choline, high myoinositol on short echo time studies, and 1. What is the relevance of biopsy?
low N-acetyl aspartate. Characteristically, in sequential MRIs 2. Diagnosis can be delayed in the absence of symptoms.
these tumors have stable features, without tumor p rogression, Which condition would cause an acute presentation?
556

Case 257 557

Direct reporting is essential as diagnosis could only be made 1. Current imaging modalities are insufficient to accurately
by biopsy. Describe the extent of the abnormalities. Perform predict histologic diagnosis and prognosis. Tumors can
and interpret MRS results to improve diagnostic accuracy have anaplastic areas that make them grade III or IV
and offer a differential diagnosis. neoplasms.
2. Hydrocephalus can develop and needs prompt surgical
What the Treating Physician Needs to Know treatment.
Particular features of macroscopic appearance on MRI and
possible differential diagnosis
Extent of the lesion and complementary study for stereo-
taxic biopsy

Case
Figure 258-1
Figure 258-2
Figure 258-3
FINDINGS Figure 258-1. Coronal T2WI through the fron-

tal horns. There are prominent right frontal lobe medullary
venous signal voids extending from the subcortical region to
the right frontal horn (arrows). Figure 258-2. Coronal post- Figure 258-4
contrast T1WI just anterior to Figure 258-1. There are mul-
tiple contrast-enhancing medullary veins in the right frontal
corona radiata and centrum semiovale (transverse arrows), large right parafalcine vein (vertical arrow). Figure 258-4.
the so-called caput medusa draining into a large collector CTA axial MIP through the inferior frontal lobes. There at
vein (inferior vertical arrow) over the anterior body of the least two large venous structures, caput medusa or horse
corpus callosum which empties into a large right parafal- whips (arrows) draining the subcortical frontal lobes into a
cine vein (superior vertical arrow). There are also multiple midline internal cerebral vein (chevron).
contrast-enhancing venous structures in the bilateral infe-
rior frontal lobes (line arrows) similar to the right frontal DIFFERENTIAL DIAGNOSISArteriovenous malforma-
corona radiata medullary veins. Figure 258-3. CTA coronal tion (AVM), developmental venous anomaly (DVA).
MIP through the frontal lobes. There are multiple bilateral
large medullary veins (transverse arrows) draining into the DIAGNOSIS Developmental venous anomaly (DVA).
558

Case 258 559
DISCUSSION These are multiple bilateral frontal lobe and hyalinized veins with interspersed normal brain tissue.
DVAs. The classical findings on MRI are a collection of small They are generally asymptomatic and require no treatment.
medullary venous signal voids on T1WI and T2WI which Headache, dizziness, and ataxia present in some of these
originate from the subcortical white matter (WM) draining individuals have been ascribed to other causes. The occa-
into a main venous trunk that empties into a larger deep or sional hemorrhage in the vicinity has been ascribed to a
superficial vein. The collection of medullary veins has been coexisting CCM.
likened to a medusa head or caput medusa. Some might
say horse whip. These venous structures are rarely visual- Question for Further Thought
ized on NCCT but are hyperdense on post-contrast CT in 1. What is venous angioma?
the same manner as the MRI. CTA and MRA demonstrates
the caput medusa or the horse whip as a collection of small Reporting Responsibilities
medullary veins draining into a collector or a holding vein Routine reporting is sufficient. There is usually no danger of
before finally draining into a recognizable anatomic venous imminent acute event in DVA. Presence of other associated
structure. Other than a recent report of brain signal abnor- anomalies such as CCM should be reported.
mality in the drainage territory of the DVA in 7.8% of DVA
and of infarct as a result of spontaneous thrombosis of the What the Treating Physician Needs to Know
DVA, there are usually no obvious significant parenchymal Location of DVA
changes associated with DVA. However, cerebral cavernous This is a benign leave me alone congenital anomaly
malformation (CCM) has been reported as coexisting with requiring no treatment or follow-up imaging
DVA in up to 33% of the patients. Other associated changes such as CCM and edema in the
DVA is considered a variation of normal venous drain- vicinity may require management however
age of otherwise normal brain tissue. The region of the mal-
formation does not have any other normal draining veins. Answer
Resection or occlusion of DVA in the past has resulted in 1. Venous angioma used to be the old name of DVA and
venous infarct of the area of drainage. It is found in all ages was classified as one of the four vascular malformations
and tend not to grow when observed over time. Its origin is of the brain along with AVM, capillary telangiectasia,
obscure but it is felt to represent an arrest during the devel- and CCMs. It underwent a name change to developmen-
opment of the venous system, which results in the retention tal venous anomaly when it was realized that it was actu-
of primitive embryologic medullary veins draining into a ally not a malformation but a variant of normal medullary
single longer vein or that they may result from intrauterine veins draining normal brain parenchyma. It is now called
occlusion or maldevelopment of normal medullary veins. developmental venous anomaly. It is present in about 2%
Histologically DVA is composed of sometimes thickened of the population.

Case
259 CLINICAL HISTORY 70-year-old female with cough and shortness of
breath. She lost 30 pounds over the last 3 months.
FINDINGS Figure 259-1. Axial NCCT through the lateral in the right frontoparietal junction with surrounding marked
ventricles. There is a mildly hyperdense mass in the right hyperintensity consistent with vasogenic edema. The medial
frontoparietal lobe with extensive surrounding hypodensity crescent is of intermediate hyperintensity (arrow). There is
consistent with vasogenic edema. There is local mass effect mild mass effect on the right lateral ventricle with efface-
but no midline shift. There is a crescentic medial compo- ment of overlying sulci. Figure 259-3. ADC map through the
nent that is less hyperdense (arrow) suggesting infiltration of mass. There is low ADC within the mass (arrow) consistent
surrounding edema. Figure 259-2. Axial T2WI through the with restricted diffusion. Figure 259-4. The mass demon-
mass. There is a heterogeneous minimally hyperintense mass strates marked homogeneous enhancement. Abutment of the
560

Case 259 561
PCNSLs tend to present as large masses. Edema is

present in 90% of patients. PCNSL has a preference for
the cerebral hemispheres (38.2%), basal ganglia (27%),
and masses involving the ventricular borders account for
11.8% of cases. Imaging is poor at detecting leptomenin-
geal disease in lymphoma and CSF analysis is required.
Glioblastoma (GB) may show heterogeneous high diffusion
and low ADC values in areas of hypercellularity. Perfusion
imaging demonstrates a leakage pattern more commonly in
lymphoma than in high-grade glioma and the perfusion is
usually low due to lack of angiogenesis. Massively elevated
lipid and greater elevation of the Cho/Cr level in PCNSL
may help differentiate lymphoma from glioma.
PCNSL occurs predominantly in older immunocompetent
adults with a mean age of 59.8 years with a range of 27 to
82 years in one series. It occurs equally in both male and
Figure 259-5
female. More than 90% of PCNSL are B-cell non-Hodgkin
lymphomas. They more often demonstrate an angiocentric
cortex is clearly identified. Anteriorly and medially there is proliferation of large malignant lymphocytes from where
mild infiltration seen into the adjacent brain (arrow). Figure they diffusely infiltrate the brain parenchyma. Mitoses are
259-5. Photomicrograph shows discohesive population of frequent, and necrosis is common. Intervening parenchyma
large lymphoid cells with atypical hyperchromatic nuclei; shows an astrocytic and microglial response, macrophages,
some with one or more nucleoli (H&E stain). and reactive lymphocytic infiltrates. T-cell phenotype is very
rare and anaplastic large cell lymphoma is exceptional. Low-
grade B-cell tumors occur, but a precise definition is lacking,
DIFFERENTIAL DIAGNOSISLymphoma, hypercellular
and less than 5% of CNS lymphoma cases are considered
metastatic disease, hypercellular glioma.
low grade.
DIAGNOSIS
Lymphoma (primary central nervous system lymphoma Question for Further Thought
[PCNSL]). 1. What type of CNS lymphoma presents with ischemia?
DISCUSSION Hyperdensity on CT is a key feature of lym- Reporting Responsibilities

phoma (PCNSL) and is due to its hypercellularity. This imag- Direct reporting is necessary as in any tumor. Mass effect
ing feature is very useful since a head CT without contrast is and herniations increase the urgency of the report. Location
often obtained initially when a patient presents with neuro- is important and unusual presentations should prompt con-
logic complaints. PCNSL could also be hypo- to isodense. sideration of alternative diagnosis. Advanced imaging help
The tumor has a great propensity to present adjacent to the in differentiating lymphoma from its mimics, and they
cerebrospinal fluid (CSF) either cortical or periventricular should be suggested.
including in the corpus callosum. When lymphoma presents
as a mass abutting the dura, it can mimic a meningioma in What the Treating Physician Needs to Know
appearance. However, MRI remains indispensable since even
Location is important for treatment planning
large lymphoma masses might present as isodense to brain on
Is it safe to perform LP to exclude CSF seeding? Large
a CT without contrast and be difficult to detect. The hyper-
masses and herniations may contraindicate LP
cellularity of a lymphoma mass results in lower ADC values
Steroids should be withheld if PCNSL is a likely diagnosis
and relatively hypointense masses on T2WIs. Theenhance-
by imaging criteria since steroid treatment can obfuscate
ment of PCNSL is more homogeneous than what is seen in
histologic diagnosis
the majority of gliomas. However, the enhancement pattern
in any particular case can be quite heterogeneous and in
unusual cases there may be infiltration into the brain with- Answer
out enhancement. These tumors pathologically could present 1. Intravascular lymphoma (IVL) is a rare presentation of
a very infiltrating pattern along perivascular spaces with a PCNSL, and it is usually of B-cell lineage proliferating
minimal degree of nodularity or mass. Ring enhancement is within lumens of capillaries, venules, and arterioles. The
more typically seen in immunosuppressed patients associated vascular occlusion results in ischemic features. The pres-
with transplantation or HIV. PCNSL may have many appear- ence of associated nodular areas of enhancement should
ances but necrosis, peripheral enhancement, hemorrhage, or suggest an etiology besides typical ischemia. Dural and
calcifications are unusual in an immunocompetent patient. arachnoid enhancement is potentially present.

Case
260 CLINICAL HISTORY 67-year-old female with 1-day history of rapidly
improving right side weakness, mental status changes, and fluent aphasia.
562

Case 260 563
mucoid production resulting in the so-called gelatinous

pseudocysts along the lenticulostriate vessels and the
thalamoperforators. The mucoid material tends to restrict
diffusion as shown within the left sylvian fissure in this
patient. The cysts are hypointense on T1WI and hyper-
intense on T2WI. The lesions as well as the leptomenin-
ges may not contrast enhance in the immunocompromised
patient but could show intense ring enhancement in the
immunocompetent patient because of the ability to mount
an immune response. Invasion of the parenchyma results
in contrast-enhancing granulomatous nodules with sur-
rounding edema called cryptococcomas. Large irregular
ring or heterogeneous contrast-enhancing masses with
surrounding edema have been reported in immunocom-
petent patients. Hydrocephalus may be a feature of CNS
cryptococcosis. Left middle cerebral artery (MCA) terri-
torial infarct is a possibility particularly in view of the
history but lack of parenchymal restricted diffusion sug-
gests otherwise. Any other form of meningitis or menin-
goencephalitis could produce similar findings. Contrast
enhancement is unusual in diffuse astrocytoma but pres-
ent in anaplastic astrocytoma. Both may show areas of
restricted diffusion.
Figure 260-5 Cryptococcus neoformans is one of the most common
agents of meningitis in the immunosuppressed patient. This
patient had a liver transplant 5 years earlier and was on an
FINDINGS Figure 260-1. Axial NCCT of the brain through immunosuppressant regimen. Cryptococcus gattii causes
the level of the sylvian fissures. There is effacement of the infection in apparently immunocompetent patients in tropi-
left sylvian fissure and a perisylvian hypodensity (arrows) cal regions of the world. The route of infection is by inhala-
which was initially interpreted as ischemic infarct because tion into the lungs and subsequent spread to the CNS. The
of the history. Figures 260-2 and 260-3. DWI and ADC ensuing meningoencephalitis presents usually with head-
map through same level. There is restricted diffusion within ache, fever, malaise, decreased level of consciousness, pap-
the left sylvian fissure (vertical arrow in Figure260-2) sur- illedema, and meningismus. This patient presented with a
rounded by a large area of elevated diffusion (transverse rapidly improving cerebrovascular pattern suggestive of a
arrows in Figure 260-3) consistent with edema. Figure 260-4. TIA with no obvious fever or other constitutional symptoms.
Axial FLAIR through the area. There is a left perisylvian The CSF evaluation showed exceedingly high protein, low
sharply marginated hyperintensity (arrows) surrounding the glucose, and cryptococcal antigen. Positive CSF cryptococ-
effaced heterogeneous left sylvian fissure. Figure 260-5. cal antigen has sensitivity over 90%.
Axial post-contrast T1WI through the left sylvian fissure.
There is thick leptomeningeal/cortical contrast enhance- Question for Further Thought
ment (arrows) surrounding the left sylvian fissure. There is 1. How is cryptococcosis treated?
left perisylvian hypointensity.
DIFFERENTIAL DIAGNOSISIschemic infarct, granulo- Direct reporting is important in this case because of the
matous meningitis, meningoencephalitis, astrocytoma. implications of delayed treatment. Presence of significant
swelling or hydrocephalus should be reported. A recommen-
DIAGNOSIS Cryptococcal meningitis. dation for lumbar puncture (LP) was made, and this subse-
quently proved to clinch the diagnosis.
DISCUSSION Central nervous system (CNS) crypto-
coccosis is an opportunistic infection of the immunocom- What the Treating Physician Needs to Know
promised patient rarely affecting the immunocompetent Is it safe to perform LP in this patient? LP is safe in this
patient. CNS cryptococcosis could be either meningeal or non-space-occupying lesion
parenchymal. The meningitis is predominant at the base of Are there other imaging tests that could narrow the differ-
the brain where it spreads along and dilates the perivascu- entials? Other imaging tests would still not be specific. LP
lar spaces in the basal ganglia and thalamus with copious is the most definitive way to make the diagnosis

564 Case 260
Answer could be a problem both at presentation and during treat-

1. In immunosuppressed patients, it is recommended that the ment of cryptococcosis. Suggested treatment for raised
immunosuppressant dose to be reduced if possible. The ICP include daily LP with serial large volume CSF with-
suggested mainstay of drug treatment is amphotericin B drawal to bring pressure down below 20 cm H2O or 30%
with flucytosine for the first 2 weeks followed by oral flu- of initial opening pressure. Medical treatment of ICP has
conazole for additional 8 weeks. Alternative regimens are not been shown to be effective. If hydrocephalus devel-
available. Significantly raised intracranial pressure (ICP) ops, surgical shunting could be offered.

Case
261 CLINICAL HISTORY 34-year-old female with sudden onset of headache
and altered mental status.
FINDINGS Figures 261-1 and 261-2. Mid-sagittal and DIAGNOSIS Pituitary hemorrhage.
axial non-contrast T1WI, respectively, through the sella
turcica. There is a mass in the sella that contains a blood DISCUSSION Typical MRI findings of pituitary hemor-
level (arrows). The sella is expanded, implying the presence rhage include hyperintensity on T1WI (reflecting acute and
of a previous mass. Both levels are bright, suggesting met- subacute blood products such as methemoglobin) with vari-
hemoglobin. Figure 261-3. Axial T2WI through the sella. able contrast enhancement. Restricted diffusion can be seen
The dependent portion of the blood is dark suggesting intra- in solid infarcted components. Hematocrit levels may be pres-
cellular methemoglobin, whereas the supernatant remains ent. On CT, the hemorrhagic gland appears hyperdense. Acute
hyperintense suggesting extracellular methemoglobin. hydrocephalus may be present. Large hemorrhage may spill
Figure261-4. Post-contrast sagittal T1WI shows no changes over into the subarachnoid space, resulting in subarachnoid
in the contents of the lesion and only posterior enhancement hemorrhage. Fluid level is common in craniopharyngioma and
(arrow), where the residual tumor tissue is located. may be present in a dermoid but not in Rathke cleft cyst. The
mural nodule is characteristic of Rathke cleft cyst.
DIFFERENTIAL DIAGNOSIS Pituitary apoplexy, Rathke Pituitary hemorrhage usually occurs as a result of bleed-
cleft cyst, dermoid cyst, craniopharyngioma. ing into a preexisting adenoma or cyst. If the patient presents
565

566 Case 261
with acute decompensation, the diagnosis is referred to as Reporting Responsibilities

apoplexy, which can result from hemorrhage or infarction Direct reporting is essential as this is an emergency requir-
of the pituitary gland with mass effect upon surrounding ing prompt treatment except in the subclinical apoplexy.
structures. Hemorrhage into pituitary adenomas has been Describe the abnormalities and their extent.
described in several clinical circumstances, including prior
radiation treatment, pregnancy (Sheehan syndrome), trauma, What the Treating Physician Needs to Know
anticoagulation therapy, open heart surgery, cerebral angi- Size and extent of the mass effect
ography, diabetic ketoacidosis, and bromocriptine treatment Complications such as compression of the chiasm, sub-
of a prolactinoma. Symptoms of pituitary apoplexy include arachnoid hemorrhage, and cavernous sinus compression
acute onset of headache, visual field deficits, ophthalmople- This is an emergency with a high mortality rate in patients
gia, and altered mental status. not treated promptly
Subacute hemorrhage into pituitary adenomas has also
been described with a more benign clinical course that can Answers
result in hypopituitarism. This is known as subclinical apo- 1. Underlying adenomas are present in up to 90% of cases of
plexy. The imaging findings are similar with the differentiat- pituitary hemorrhage.
ing factor being the clinical presentation. The treatment for 2. There are five described stages of degradation of hem-
pituitary apoplexy is urgent surgical decompression with a orrhage. In the hyperacute stage, intracellular oxyhe-
high mortality rate in patients not receiving surgical treat- moglobin is isointense on both T1WI and T2WI. In the
ment if the hematoma is large. Hormonal supplements are acute stage (1 to 2 days), intracellular deoxyhemoglobin
also instituted. is hypointense on T2WI and isointense on T1WI. In the
early subacute stage (2 to 7 days), intracellular methe-
Questions for Further Thought moglobin is hyperintense on T1WI and hypointense on
1. What is the incidence of an underlying adenoma in pitu- T2WI. In the late subacute stage (1 to 4 weeks), extra-
itary hemorrhage? cellular methemoglobin is hyperintense on T1WI and
2. What are the different MRI features when evaluating T2WI. In the chronic stage (>2 to 4 weeks), hemosiderin
stages of hematoma? is hypointense on T1WI and T2WI.

Case
agenesis of the corpus callosum, lissencephaly, and holo-

prosencephaly.
There is a raging debate as we write about HIMAL or IHI
and causality of seizures. HIMAL is found in about 6% to
30% of patients with seizures while it is present in about 1%
to 20.7% of healthy volunteers and patients without epilepsy
or obvious developmental brain anomalies. It is therefore an
unsettled question as to whether HIMAL causes seizures.
Some see HIMAL as a morphologic variant of the hippo-
campus without causal link to seizures but a link to abnor-
mal development elsewhere in the brain which may lead to
Figure 262-1 seizures.

FINDINGS Figure 262-1. Coronal T2WI of the hippocam- 1. What is the developmental anatomy of the hippocampus?
pus obtained perpendicular to the long axis of the hippocam-
pus. There is a rounded appearance of the left hippocampus Reporting Responsibilities
(vertical arrow) compared with the transverse lie of the right Routine reporting is sufficient. These patients, because of the
hippocampus (chevron). The left collateral sulcus is verti- nature of their clinical situationseizures, are well monitored
calized (transverse arrow) compared with the transverse and followed by their epileptologist who does not rely solely
position of the right collateral sulcus (line arrow). Both hip- on the imaging findings but also other diagnostic procedures
pocampi are of similar signal intensity. particularly closed-circuit TV electroencephalographic mon-
itoring as well to localize the seizure focus. Other findings
DIFFERENTIAL DIAGNOSIS N/A. such as local volume loss or associated congenital abnormali-
ties should be reported.
DIAGNOSIS Left hippocampal malrotation (HIMAL).
DISCUSSION The major prerequisites for the diagnosis of Whether HIMAL is unilateral or bilateral
HIMAL or as some would like to call it incomplete hippo- Other associated congenital lesions elsewhere in the brain
campal inversion (IHI) on coronal T2WI obtained perpen- Presence of mesiotemporal sclerosis
dicular to the long axis of the hippocampus are unilaterality The column of fornix and its anomalies
of the lesion, abnormal round shape of the hippocampus,
normal size and intensity of the hippocampus, verticaliza- Answer
tion of the ipsilateral collateral sulcus, and atypical position 1. The hippocampus at 13 to 14 weeks fetal age is a flat
and size of the fornix with or without apparent enlargement structure medially and inferiorly to the temporal horn
of the ipsilateral temporal horn. All these findings are usu- on the medial aspect of the temporal lobe with serial
ally not fulfilled in every case. The two most constant find- arrangement of the dentate gyrus, cornu ammonis,
ings are the round configuration of the hippocampus and the subiculum, and the parahippocampal gyrus. At about 16
verticalization of the collateral sulcus. In our patient, the weeks it starts to fold and push laterally into the tem-
unilaterality and similar signal intensity criteria are also ful- poral lobe medially to the temporal horn. This infolding
filled. HIMAL is most often unilateral and left-sided. It is marks the beginning of the formation of the hippocampal
found in all ages without gender preference. sulcus between the dentate gyrus and cornu ammonis.
The hippocampus plays a significant role in epilepsy, Progression of the infolding laterally into the temporal
Alzheimer disease (AD), amnesia, and schizophrenia. lobe deepens the hippocampal sulcus and emergence
Change in hippocampal signal intensity pattern and size is medially on the temporal surface of the parahippocampal
associated with mesiotemporal sclerosis where the hippo- gyrus and the subiculum. At about 18 weeks, as the hip-
campus is small and hyperintense compared with the normal pocampus infolding goes deeper into the temporal lobe,
side. Substantial hippocampal atrophy is found in patients the dentate gyrus, molecular stratum, cornu ammonis,
with AD, while damage to the hippocampus has been linked alveus, fimbria, and the subiculum rearrange themselves
to memory problems. Abnormality of the hippocampus has such that the dentate gyrus and the cornu ammonis change
also been described in congenital brain anomalies such as into an interlocking C-shape of the adult hippocampus.
567

568 Case 262
The hippocampal sulcus shifts into a position between fusion of the hippocampal sulcus may create a cerebro-
the dentate gyrus and the subiculum. By 24 weeks, the spinal fluid (CSF)-filled residual cavity that forms a cyst
hippocampus is deep in the temporal lobe with fusion within the hippocampus. Other congenital anomalies
of the hippocampal sulcus and formation of the collat- such as agenesis of the corpus callosum may result in
eral sulcus inferiorly to the hippocampus. Incomplete lack of folding of the hippocampus or a shallow hippo-
infolding may result in HIMAL or IHI. Lack of complete campal sulcus.

Case
263 CLINICAL HISTORY 67-year-old female with scalp mass.
FINDINGS Figure 263-1. NCCT coronal reformation as typical. For the case of a subgaleal lipoma, which is tightly
through the anterior parietal bones. There is a fat density confined between the tense galea and the noncompressible
lesion over which drapes the galea aponeurotica (arrow). skull, a firm or rubbery mass may be found on examination.
Figure 263-2. Sagittal MRI T1WI through the lesion. The It is benign and usually requires no treatment but could cre-
mass is hyperintense like the subcutaneous fat. ate a cosmetic problem!
DIFFERENTIAL DIAGNOSIS Lipoma, dermoid, sarcoma. Question for Further Thought

1. What is the approach to a fatty lesion which is not com-
DIAGNOSIS Subgaleal lipoma. posed exclusively of fat?
DISCUSSION Lipoma is a benign tumor composed

entirely or predominantly of mature fat cells. Imaging char-
Routine reporting is sufficient in this benign lesion unless the
acteristics of lipoma are usually specific enough to make a
imaging was specifically done to evaluate the bump. Confirm
confident diagnosis. On CT, the lesion will show character-
that the lesion is indeed composed of fatty material and rec-
istic fat attenuation, on the order of 100 HU, identical to
ognize this as benign.
fat elsewhere. On MRI, signal intensity of the lesion should
follow fat on all sequences, which is typically hyperintense
on T1WI and T2WI. Fat suppression MRI techniques may be What the Treating Physician Needs to Know
applied to confirm the loss of signal indicative of fat. If fat Lipoma is a common benign tumor which can be found
suppression is desired to further evaluate suspected lipoma, anywhere in the body
chemical shift or frequency selective techniques are pre- Imaging is usually specific enough to offer the diagnosis
ferred over STIR as STIR can sometimes suppress tissues with confidence
other than fat which could lead to a misdiagnosis. Benign The size and surrounding of the lesion to exclude invasion
lipoma should never enhance. Dermoid is usually hypoin- of structures
tense on T2WI. Sarcoma is usually heterogeneous in density
and intensity and invasive in character and contrast enhances. Answer
Lipoma is the most common tumor of mesenchymal ori- 1. Benign lipoma is most commonly composed entirely of
gin. Lipoma may occur in any location of the body and in fat. A minority may show thin septations or small nodules
virtually any space. In the head and neck region, it can be of soft tissue. When soft tissue elements are present, the
found in any of the deep spaces and sometimes straddling tumor is still usually benign. These elements are, along
two or more spaces. In the scalp and skull, lipoma can be with the fat, of mesenchymal origin and can include fibrous
superficial and contained within the subcutaneous space, or tissue (fibrolipoma), vascular tissue (angiolipoma), brown
deep, occupying the potential space between the galea apo- fat (hibernoma), as well as several other types. However,
neurotica and the periosteum of the skull. When superficial, when prominent soft tissue elements are present, and espe-
lipoma presents as a soft, nontender mass. When deep, how- cially when these enhance, the possibility of liposarcoma
ever, the characteristics on physical examination may not be must be excluded by biopsy or resection.
569

Case
264 CLINICAL HISTORY Patient 1: 25-year-old female with headache.
Patient 2: 15-year-old male with a familial syndrome.
FINDINGS Figure 264-1. Axial T2WI through the pons in Axial T2WI in patient 2 through the brainstem. There are
patient 1. There is a small focus of susceptibility (signal loss) multiple well-circumscribed lesions with mixed, hyper- and
along the floor of the fourth ventricle on the right (arrow). hypointensities without surrounding edema. The dominant
Figure 264-2. Axial post-contrast T1WI. There is an asso- lesion (arrow) is T2 hyperintense with a rim of hypointen-
ciated linear enhancing structure (arrow). Figure 264-3. sity and is somewhat bubbly or popcorn-like in morphology.
570

Case 264 571
Figure264-4. Axial GRE through same level. There are many a DVA can help confirm the diagnosis. Significant enhance-
more lesions, 15 to 20 are seen; many of which arepunctate ment may be present in hemorrhagic metastasis. Vascular
both in the brainstem and in the cerebellar hemispheres. signal voids are usual in AVM, while acute hematoma is
usually homogeneous in intensity.
DIFFERENTIAL DIAGNOSISArteriovenous malforma- Multifocal lesions are more common in the familial type
tion (AVM), hypertensive hemorrhage, hemorrhagic metas- more commonly seen in people of Mexican descent.
tases, cavernous malformations.
DIAGNOSIS Cerebral cavernous malformations (CCM). 1. How are CCMs treated?
DISCUSSION CCMs are hamartomas of immature, poorly Reporting Responsibilities

organized vascular channels. They occur in all age groups Direct reporting is necessary particularly if there is active
and range in size from punctate to several centimeters. hemorrhage. Describe the location of the CCM, any inter-
Lesions have a tendency to bleed into themselves, forming val growth if applicable, and any parenchymal edema. Look
central locules of blood of different ages, accounting for the carefully for the associated DVA. When one CCM is found,
heterogeneous, bright/dark internal signal characteristics. search carefully for others. Multiple CCMs may indicate a
Blood product also tends to ooze into the margins giving familial syndrome.
the appearance of a dark hemosiderin ring. It is the blood
product that makes these lesions visible on imaging. Prior
to bleeding, they are undetectable by any modality, even
conventional angiography. T2WI often shows them to best CCM is an uncommon entity which may be asymptomatic
advantage with a characteristic lobulated, popcorn-like mor- and incidental
phology, mixed bright/dark signal internally, and a complete They may produce focal symptoms (seizure, motor, and
dark hemosiderin rim peripherally. Gradient echo or sus- sensory deficits) depending on location
ceptibility imaging is much more sensitive to blood product
and may therefore reveal many more lesions than initially Answer
suspected. Although CCMs do not enhance themselves and 1. Surgical resection is the preferred treatment for a symp-
are occult angiographically, they are often associated with a tomatic or enlarging lesion. Stereotactic radiosurgery can
developmental venous anomaly (DVA), which does enhance also be effective. If a DVA is present, it must be preserved
(as in patient 1). When a CCM is suspected, the presence of to prevent venous infarction.

Case
265 CLINICAL HISTORY 35-year-old male presenting with liver insufficiency
and choreoathetoid movements which are progressively worsening.
FINDINGS Figure 265-1. Axial FLAIR through the basal creatine ratio in the basal ganglia. Neuroimaging studies can
ganglia. There is bilateral symmetrical hyperintensity in the be helpful in diagnosis and in assessing the clinical response
basal ganglia (especially the lateral aspect of the putamen), to treatment. Mitochondrial disorders such as Leigh syn-
thalamus, and claustrum (arrows). Figure 265-2. Axial T2WI drome shows symmetric areas of T2 hyperintensity in basal
through the midbrain. There is periaqueductal and cerebral ganglia, periaqueductal region, and cerebral peduncles. MRS
peduncle hyperintensity (arrow). reveals lactate peak in deep gray matter (GM) and normal
appearing brain. Brain atrophy and symmetrical diffuse
DIFFERENTIAL DIAGNOSISMitochondrial disorders abnormalities in the WM in addition to areas of T2 hyperin-
(such as Leigh syndrome), methylmalonic academia, toxins tensity in basal ganglia are common in methylmalonic aca-
(CO, methanol, cyanide), hypoxic ischemic encephalopathy demia, while bilateral globus pallidus hyperintensity may be
(HIE), Wilson disease (WD). a manifestation of toxins such as CO, methanol, cyanide poi-
soning sometimes with delayed onset leukoencephalopathy.
DIAGNOSIS Wilson disease (WD). Hemorrhagic necrosis of the putamen may be observed in
cyanide and methanol poisoning, and WM edema may be an
DISCUSSION NCCT may be normal or show only mild additional feature of methanol poisoning.
generalized atrophy. Areas of hypodensity within the basal WD is a rare autosomal recessive inherited disorder with
ganglia and thalami are occasionally demonstrated. MRI an incidence of 1 in 30,000 to 100,000 live births. It is char-
usually shows areas of focal signal abnormalities, hypoin- acterized by a deficiency of ceruloplasmin, a copper transport
tense on T1WI, and hyperintense on FLAIR and T2WI in the serum protein, with consequent loss of the ability to export
putamen and the ventrolateral aspect of the thalamus. The copper from the liver into bile resulting in excessive accumu-
globus pallidus, caudate nuclei, white matter (WM), dentate lation of copper in the body, mainly in the liver, brain, cornea,
nuclei, mesencephalon (Panda face sign), and the pons may and kidney. Liver disease and neuropsychiatric disturbances
also be involved. Diffusion restriction may be seen early in are the most frequent presentations, and progressive liver
the course of the disease especially in the claustrum and/or failure at early age is most commonly the initial presentation.
lateral putamen. Usually there is no contrast enhancement. Neurologic symptoms are due to accumulation of copper in
MRS usually demonstrates decrease in N-acetyl aspartate the basal ganglia and hepatic encephalopathy. Parkinsonian
(NAA) to creatine ratio in the parietal occipital cortex, fron- symptoms, including rigidity, bradykinesia, resting tremor,
tal WM, and basal ganglia and an increased myoinositol to and personality changes are common.
572

Case 265 573
The diagnosis of WD is based on the typical clinical find- other causes of signal hyperintensity of the basal ganglia.
ings (Kayser-Fleischer corneal ring in the eye, sunflower High-signal-intensity lesions in the basal ganglia on non-
cataracts) and laboratory abnormalities (serum copper levels contrast T1WIs reflect hepatic failure.
are generally low to normal while urine copper levels are
increased, serum ceruloplasmin is generally low and liver What the Treating Physician Needs to Know
biopsy demonstrates elevated copper levels). Treatment for Interval changes seen on follow-up MRI closely corre-
WD centers on chelation therapy. Penicillamine, trientine, late with clinical findings and can be helpful in assessing
and zinc are the most common agents used. Symptomatic response to treatment
patients require lifelong treatment because an interruption to T2 hyperintense lesions reflect cerebral involvement of
therapy or inadequate treatment may result in death. Patients WD and correlate with neurologic symptoms
typically display improvement of symptoms within 6 months
after the start of chelation therapy especially with respect to
psychiatric and neurologic symptoms. Liver disease is com- Answers
monly advanced at presentation and is progressive to the 1. Fine pigmented granular deposits of copper in Descemets
point of liver transplantation. membrane of the cornea, most pronounced at its inferior
and superior poles are known as Kayser-Fleisher rings.
Questions for Further Thought Even though this sign is characteristic for WD it is not
1. Are Kayser-Fleischer rings a pathognomonic sign for WD? pathognomonic and is variably present in 95% of patients
2. What additional neuroimaging techniques might be help- with neurologic symptoms but only in 50% to 60% of
ful in detecting early brain damage in WD? patients without neurologic symptoms, and in 10% of
asymptomatic siblings.
Reporting Responsibilities 2. SPECT demonstrates a reduction in the activity of dopa-
This requires direct reporting. The distribution and pattern of decarboxylase indicating impaired function of the nigros-
signal changes should be described and differentiated from triatal pathway.

Case
266 CLINICAL HISTORY Child with cerebral palsy.
polymicrogyria, and presence of subcortical GM or hetero-

topia. There may be decreased myelination or hypertrophy
of the WM either diffusely or focally. The ipsilateral lateral
ventricle is usually enlarged without evidence of periven-
tricular cerebrospinal fluid (CSF) flow to suggest hydro-
cephalus. The contralateral hemisphere is small and may be
hypodense suggesting edema or stroke except that the his-
tory is not that of acute event. There are reports of abnormal-
ities elsewhere in the brain outside the involved hemisphere.
These include ipsilateral olfactory nerve enlargement, cere-
bral vascular dilations, cerebellar architectural distortion,
and enlargement which could be bilateral or ipsilateral.
The patient may demonstrate intractable seizures, hemi-
plegia, and developmental delay. The evaluation of a patient
with suspected hemimegalencephaly will usually consist of
questions about medical history and family history, a physi-
cal examination that includes head measurements, and a
developmental and/or neurologic examination.
Figure 266-1
1. Is megalencephaly the same as macrocephaly?
FINDINGS Figure 266-1. Axial NCCT through the level of
the lateral ventricles. There is significant enlargement of the Reporting Responsibilities
right lateral ventricle. There is thickened, malformed right Routine reporting is sufficient. It is important not to mistaken
sylvian fissure with poor sulcation (arrow). There is also the smaller side for abnormality such as old stroke, hemiatro-
enlargement of the frontal white matter (WM) (star). The left phy, or Rasmussen encephalitis. Associated findings should
hemisphere is barely visualized at this level. be enumerated.
DIFFERENTIAL DIAGNOSISHemimegalencephaly, left What the Treating Physician Needs to Know

hemiatrophy. Which is the abnormal sidethe large one or the small one?
Any other associated findings
DIAGNOSIS Hemimegalencephaly right.
Answer
DISCUSSION Hemimegalencephaly is congenital enlarge- 1. No. Megalencephaly is different from macrocephaly (also
ment of part or all of a cerebral hemisphere. CT and MR called macrocranium or megalocephaly), which describes
can demonstrate focal or diffusely enlarged malformed cor- a big head and does not necessarily indicate parenchymal
tical gray matter (GM), cortical dysplasia, lissencephaly, brain abnormality.
574

Case
267 CLINICAL HISTORY 52-year-old female was admitted with generalized
tonicclonic seizures and confusion 3 months following a diagnosis of
syndrome ofinappropriate antidiuretic hormone SIADH.
Figure 267-4
Figure 267-3
DIFFERENTIAL DIAGNOSIS Limbic encephalitis (para-
neoplastic), herpes simplex encephalitis (HSE) and human
herpes virus 6 (HHV6) encephalitis, seizure-related changes,
FINDINGS Figure 267-1. Axial MR FLAIR through the gliomatosis.
temporal lobes. There is bilateral almost symmetrical mesio-
temporal smudgy hyperintensity (arrows). Figure 267-2. Axial DIAGNOSIS Paraneoplastic limbic encephalitis (PLE).
FLAIR through the hippocampi. There is asymmetric bilateral
hippocampal hyperintensity (vertical arrows) and hyperintense DISCUSSION MRI is the examination of choice in the
insula cortex bilaterally (transverse arrows). Figure267-3. evaluation of a patient suspected of PLE. CT is fairly insen-
Coronal FLAIR through hippocampi. There is bilateral asym- sitive to the changes as it may be difficult to appreciate the
metric hippocampal hyperintensity larger on the right than the hypodensity of the temporal lobes. The classical MRI finding
left (vertical arrows). The transverse arrow points to the hyperin PLE is bilateral mesiotemporal lobes smudgy T2 hyperin-
intense fornix. Figure 267-4. Coronal post-contrast T1WI tensity with or without significant mass effect which is best
through the temporal lobes showing a small right mesiotempo- demonstrated on the FLAIR images. These changes could be
ral lobe contrast enhancement (transverse arrow). significantly subtle on T2WI. Corresponding hypointensity
575

576 Case 267
could be present on T1WI. Other sites that could show T2 and multifocal seizures, arising out of the left hemisphere
hyperintensities are the hippocampus, the column of fornix, and the left temporal region.
insula, and the subfrontal orbital gyrus regions. Diffusion
restriction is not usually a feature of PLE but may be present Questions for Further Thought
in seizure-related changes. Contrast enhancement present in 1. How does SIADH fit in the clinical picture of PLE?
this case is a rare finding and has been reported in only a 2. What are the other supportive laboratory findings in PLE?
few cases. MRI may not be positive in some cases of PLE
and a high index of suspicion should be kept if the clinical Reporting Responsibilities
picture is suggestive and no other etiology is found. There is Direct reporting is important in view of the differential of
suggestion that positron emission tomography (PET) imag- herpes encephalitis since delay in treatment of HSE could be
ing may show temporal lobe lesions in this subset. It may be catastrophic. Mass effect if present should be reported.
difficult to differentiate PLE from other causes of mesiotem-
poral T2 hyperintensity such as HSE, HHV6, gliomatosis,
and seizure-related changes. The gold standard for exclud-
ing HSE and HHV6 infection is cerebrospinal fluid (CSF) Presence of significant mass effect since lumbar puncture
polymerase chain reaction (PCR) which has a sensitivity of (LP) is always indicated in this situation
over 95% for HSE. HSE and HHV6 are treatable diseases There are no other imaging methods to confirm the
with high mortality if treatment is delayed. Volume loss is diagnosis
the final pathway of a healed PLE. Gliomatosis will not show Techniques for cancer screening include CT chest, abdo-
volume loss on follow-up. men, and pelvis or body FDG-PET as well as assay for
PLE is an autoimmune disorder that is associated with the tumor-associated antibodies. Such tumors may include
onconeuronal antibodies to intracellular and cell surface ovarian, breast, testicular, small cell lung, and lymphoma
antigens in the neuropil. The tumor-associated antibodies in
PLE include anti-VGKC, NMDA-R, AMPA-R, HU, Ma2, Answers
amphiphysin, and CV2/CRMP5 antibodies. It may take up 1. PLE can be associated with hypothalamicpituitary dys-
to 5 years for the discovery of associated tumor. The clas- function that could result in SIADH.
sic clinical manifestations of PLE include rapid onset of 2. CSF typically shows lymphocytic pleocytosis, high pro-
mood dysfunction, hallucinations, seizures, and short-term tein, and frequent elevation of IgG index and synthesis. A
memory loss. CSF study in this patient showed minimal normal CSF study except for the presence of OGB is also
pleocytosis, normal protein, and matching oligoclonal band possible. Assay for the various paraneoplastic antibodies
(OGB) in the serum and CSF. Herpes virus panel and other is important. EEG is abnormal showing generalized or
extensive infectious and inflammatory workup were nega- focal slowing or epileptiform discharges in the temporal
tive. A 24-hour video electroencephalogram (EEG) dem- lobes. The constellation of findings on clinical examina-
onstrated mild-to-moderate diffuse cerebral dysfunction, tion, CSF study, EEG, and MRI should guide the clinician
intermittent poorly formed bifrontal epileptiform activity, to the right diagnosis.

268
CLINICAL HISTORY 67-year-old male noted to have gradually increased
Case
frequency of falling, right hand tremor particularly when he is walking,
smaller handwriting, trouble opening boxes and caps, softer voice, decreased
facial expression, depression, neck stiffness, stooped posture and shortened
strides, and executive dysfunction.
FINDINGS Figure 268-1. This is an abnormal 123I-FP-CIT dopamine therapy is not a contraindication for SPECT. Of
(DAT scan) SPECT. There is symmetrical decrease of tracer note, when SPECT does not imply dopaminergic dysfunc-
uptake in the bilateral putamen indicating loss of dopa- tion, the clinical diagnosis of PD is likely false positive, and
mine transporters (DATs) compatible with the diagnosis of the true diagnosis may be a variant of essential, dystonic,
Parkinson disease (PD). Figure 268-2. Axial T2 MRI through or drug-induced tremor and dopa-responsive dystonia with
the basal ganglia. There are no structural abnormalities in the parkinsonism. On the other hand, SPECT has no role in
bilateral basal ganglia. differentiating atypical parkinsonism from PD, as all show
dopaminergic dysfunction.
DIFFERENTIAL DIAGNOSISNormal pressure hydro- PD is the second most common neurodegenerative dis-
cephalus (NPH), Parkinson-plus syndromes (progressive order after Alzheimer disease. The majority of cases are
supranuclear palsy, multisystem atrophy, corticobasal degen- idiopathic. The clinical presentation is a combination of
eration), dementia with Lewy bodies, Alzheimer disease. rest tremor, rigidity, postural instability, and bradykinesia.
Pathologically there is accumulation of Lewy bodies, cyto-
DIAGNOSIS PD with early cognitive impairment. plasmic (alpha)-synuclein inclusion in surviving neurons
in the substantia nigra pars compacta. Neuronal degenera-
DISCUSSION Imaging is supportive or exclusionary in tion precedes the clinical presentation. Unfortunately, motor
cases of atypical presentations of PD. Positron emission symptoms are elicited after loss of about 60% of the dopami-
tomography (PET) with the fluorodopa ligand and single pho- nergic substantia nigra neurons. This translates into 80% less
ton emission computed tomography (SPECT) are the princi- than normal dopamine content in the striatum. There is addi-
pal options. MRI is nonspecific showing loss of structural tional involvement of nondopaminergic cholinergic neurons
integrity of substantia nigra pars compacta on T2-weighted in the nucleus basalis of Meynert, noradrenergic neurons in
scans. In SPECT studies, DAT radioligands are used to the locus coeruleus, and serotonergic neurons in the midline
determine the presynaptic integrity of dopamine producing raphe responsible for nonmotor symptoms such as cognition,
nigrostriatal neurons. The classic picture is usually asym- mood, autonomic function, and the sleep cycle.
metric, reduced radiotracer uptake in the striatum bilaterally, There is no cure or well-established disease-modifying
particularly affecting the posterior putamen. Continuation of therapies; symptomatic relief with levodopa is the most
577

578 Case 268
commonly used therapy. However, besides the inherent useful and FDA-approved, CMS-funded study, currently.
motor symptoms of PD, long-term exposure to levodopa However, its role in therapy assessment/ follow-up is not
itself has motor complications. Indeed, deep brain stimula- well established yet. Brain MRI is useful to exclude other
tion has replaced ablative procedures used in motor compli- structural abnormalities and provides a road map for deep
cations and the management of treatment refractory tremor. brain stimulation electrode placement; the exact location of
Targets for deep brain stimulation procedures are subtha- the electrode tip should be accurately described.
lamic nucleus (STN) stimulation, thalamic stimulation, and
pallidal stimulation. MRI is the best choice of modality for What the Treating Physician Needs to Know
surgical assistance. Despite the diverse clinical presentation, differentiation of
PD from NPH can be challenging. Moreover, NPH is a
Question for Further Thought treatable condition, whereas PD treatment options are only
1. What is the best follow-up imaging modality? symptomatic and imaging features may overlap

Routine reporting is sufficient. PD is primarily a clinical- 1. SPECT with DAT radioligands is promising to show
based diagnosis. 123I-FP-CIT (DAT scan) SPECT is the most dopamine producing nigrostriatal neuron reserves.

269
CLINICAL HISTORY 6-year-old female has symptoms (phantosmia and
Case paraosmia; stomachache subsequently followed by an angry outburst, followed

by a headache) that correlate with possible seizures. Subsequently she looks out
of it, is drowsy, and sleeps for approximately 12 to 14 hours. This has become
progressively worse over the past year.
Figure 269-1
Figure 269-2
FINDINGS Figure 269-1. Coronal T2WI through the of calcification or hemorrhage on the MRI. Figure269-5.
temporal lobes. There is a collection of hyperintense foci Photomicrograph shows characteristic mucinous pools con-
(cysts) (arrows) scattered inferolaterally in the left tempo- taining ganglion cells (floating neurons). The other compo-
ral lobe. Figure 269-2. Coronal T2-FLAIR through same nent is composed of monomorphic cells with round nuclei
region. The hyperintense foci (cysts) show cerebrospinal and pericellular clearing artifact (H&E stain).
fluid (CSF) hypointensity with surrounding hyperintense
rims within a poorly defined area of low-level hyperin- DIFFERENTIAL DIAGNOSIS Dysembryoplastic neu-
tensity (arrows) that is not adequately appreciated on the roepithelial tumor (DNET), astrocytoma or other glioma,
coronal T2WI. Figure 269-3. Coronal T1WI. The cysts neurocysticercosis, hydatid cysts, enlarged perivascular
are markedly hypointense (arrow). No enhancement was spaces, dysplastic brain, multiple bacterial abscesses, her-
present with gadolinium. Figure 269-4. ADC map dem- pes encephalitis, ganglioglioma (GG), pilocytic astrocytoma
onstrates high values throughout the region. No evidence (PA), pleomorphic xanthoastrocytoma (PXA).
579

580 Case 269
The bubbly pattern is almost pathognomonic excluding oli-

godendroglioma, PXA, PA, cortical dysplasia, abscess, and
neurocysticercosis. Abscess, PXA, and PA have much greater
rates of enhancement. An abscess should have restricted dif-
fusion centrally. The rarity of calcification in DNET helps
to exclude consideration of neurocysticercosis. GG can be
quite similar but tends to enhance more, commonly have
calcifications, and have a single or a few cysts rather than
a multicystic bubbly appearance. The spectral pattern of
DNET helps to exclude other tumors.
DNETs are WHO I tumors found usually in young adults
in the second decade but can be seen in younger and older
patients with a 1:1 malefemale ratio. A protracted history
of seizure is usually the presenting symptom. DNETs are
largely intracortical and lack diffuse infiltration as seen in
Figure 269-5
diffuse gliomas. They are composed of multiple intracortical
nodules and an internodular glioneuronal element, which
shows microcystic change and axons ensheathed by oligo-
DIAGNOSIS Dysembryoplastic neuroepithelial tumor dendroglia-like cells (OLCs), extending to the pial surface.
(DNET). Large cortical neurons floating in small pools of mucin is
a characteristic finding. Extranodular diffuse hypercellular
DISCUSSION DNET is most commonly seen in the tem- areas composed of OLCs are usually seen.
poral lobe followed by the frontal lobe. MRI and CT show
a sharply demarcated mass, ranging greatly is size (1 mm Questions for Further Thought
to 5cm), cortical and subcortical in location with or with- 1. What is the surgical outcome for treating DNET?
out surrounding vasogenic edema. The mass is hypodense 2. Can DNET have malignant transformation?
on CT, hypointense on T1WI, and hyperintense on T2WI.
Common radiologic features include cystic or microcystic Reporting Responsibilities
pattern, remodeling of the overlying calvarium with focal Direct reporting is important for all tumors to enable early
enhancement in a minority (19% to 21.6%) and rarely calcifi- treatment. Tumors located in eloquent areas may benefit
cation (11% to 15.2%). The cysts tend to have a hyperintense from functional imaging. Significant changes on follow-up
rim on FLAIR as seen in Figure 269-2. The enhancement has should be reported.
been described as focal, nodular, ring like, or heterogeneous.
DNETs are often described as having a multicystic or bubbly What the Treating Physician Needs to Know
appearance, but great variation exists on the reported rate of Location is important for treatment planning
cyst/pseudocysts ranging from being present in a minority to Will advanced imaging be useful in further characteriza-
100% of the lesions. Hemorrhage has been noted very rarely tion to exclude higher-grade lesion, malignant transforma-
in less than 5%. Another pattern described is the wedge- tion, or infiltration of eloquent sites?
shaped pattern with the tumor wider at the cortex with over- Any significant changes on follow-up to suggest malignant
lying deformation of the skull and extension into the white transformation?
matter (WM). MRS of DNET shows preservation or sup-
pression of N-acetyl aspartate (NAA) with nonelevated cho- Answers
line. DNETs generally have lower relative Cerebral Blood 1. Surgery alone for DNET is very successful for controlling
Volume (rCBV) compared to normal brain, but some tumors seizures.
may have mildly elevated rCBV. The ADC is elevated in 2. Malignant transformation of DNET has rarely been
DNET. reported. Given this regular follow-up imaging is sug-
DNET has to be differentiated from other cortical tumors/ gested to identify progression at an early stage for tumors
lesions such as oligodendroglioma, PXA, cortical dysplasia, located in eloquent areas. It is possible that the discovery
and enlarged perivascular spaces. The surrounding gliosis of of a higher-grade glioma within a histologically proven
T2 hyperintensity on FLAIR excludes perivascular spaces. DNET is a coincidence.

Case
270 CLINICAL HISTORY 60-year-old female with headache and tinnitus.
FINDINGS Figures 270-1 and 270-2. Axial FLAIR and DIFFERENTIAL DIAGNOSISGlomus jugulare (GJ),
T2WI through the jugular foramina. There is a 2.5-cm petrous apicitis or osteomyelitis, schwannoma, hypervascu-
mass with the epicenter in the right jugular foramen pre- lar metastasis.
dominantly hyperintense with punctate/linear signal voids
(transverse arrows) creating the so-called salt-and-pepper DIAGNOSIS Paraganglioma (GJ).
appearance. The margin is irregular. The mass extends to
the right CPA (vertical arrows). Figures 270-3 and 270-4. DISCUSSION Paraganglioma is the term used for tumor
Axial and coronal post-contrast T1WI through the mass. The derived from the paraganglia of the glomus cell found in
mass is avidly contrast enhancing within the right jugular various parts of the sympathetic system. GJ tumors are para-
foramen. The tumor abuts the internal carotid artery (ICA) gangliomas located in the jugular foramen. MRI and CT are
(transverse arrow in Figure 270-3) and extends posteriorly to complementary in the evaluation of this tumor. The MRI
the cerebellopontine angle (CPA) (vertical arrow in Figure shows the soft tissue extent of the tumor nicely. The epicenter
270-3). There is inferior extension into the upper cervical is usually in the jugular foramen with or without extensions
spinal canal (arrow in Figure 270-4). into surrounding structures of the CPA, middle ear, petrous
581

582 Case 270
apex, carotid space, and sometimes down the foramen mag- uncommon due to the critical structures of cranial nerves and
num. On T1WI and T2WI, it presents the salt-and-pepper blood vessels in the vicinity. Radiation therapy has also been
appearance; the salt represents blood products from hem- employed in the management of GJ.
orrhage or slow flow and the pepper represents flow voids
due to high vascularity. This is within a background of dif- Question for Further Thought
fuse hypointensity on T1WI and hyperintensity on T2WI. 1. What is the origin of paraganglioma?
GJ shares the salt-and-pepper appearance with other vascu-
lar tumors such as hypervascular metastasis from the kid- Reporting Responsibilities
ney or thyroid. This feature is not present in schwannoma Direct reporting is necessary in this nonmalignant but locally
and petrous apicitis or osteomyelitis and may also not be invasive tumor. Identifying the salt-and-pepper pattern is
visible in small GJ. GJ usually contrast enhances avidly. important in differentiating the other differentials.
Like in this case, the flow voids are still visible on the
enhanced tumor. The CT on the other hand is great at look- What the Treating Physician Needs to Know
ing for the pattern of bone destruction. This is described The location and size of the tumor and pattern of destruction.
as a moth-eaten pattern of destruction that can stretch Is there involvement of the middle ear?
from the jugular foramen into the temporal bone, destroy- Did the tumor start in the middle earglomus tympani-
ing structures in the middle ear cavity. The bone algorithm cum? Sometimes it is difficult to tell. Hence, the term
high-resolution CT is best for demonstrating the pattern glomus jugulotympanicum is used to describe the tumor
of bone destruction. DSA usually shows a highly vascu- that has crossed from one territory to the other
lar tumor that blushes with early venous opacification. The
tumor could be seen to encase the carotid artery. DSA also Answer
plays a significant role in the treatment of this lesion as 1. Paraganglioma originates from paraganglia in non-
preoperative embolization has been used to reduce the vas- chromaffin glomus bodies derived from the embryonic
cularity of the tumor and thereby prevent massive hemor- neural crest. The glomus bodies function as part of the
rhage during surgery. sympathetic nervous system that accompanies the cranial
GJ is a disease of the fifth and sixth decades but can occur nerves. These cells normally act as special chemorecep-
at all ages. It has a female preponderance. Because of the tors located along blood vessels, particularly in the carotid
close relationship to the cranial nerves within the jugular and aortic bodies. Although these cells belong to the neu-
foramen and adjacent hypoglossal canal and internal audi- roendocrine system, they are rarely functioning. GJ is a
tory canal (IAC), GJ could present clinically with a num- relatively benign tumor that is locally destructive arising
ber of symptoms, signs, and syndromes related to these from the paraganglia of the glomus bodies surrounding
nerves which may include hearing loss, Horner syndrome, the jugular bulb within the jugular foramen. Glomus tym-
facial nerve palsy, hoarseness, and swallowing difficulty. panicum tumors arise from glomus tissue in the middle
Invasion of the middle ear may result in bleeding into the ear. Both tumors are closely associated with Jacobson
middle ear. Tinnitus is common. Treatment is surgical with nerve, the tympanic branch of glossopharyngeal nerve,
or without preoperative embolization. Complications are not and Arnold nerve, the auricular branch of the vagus nerve.

Case
271 CLINICAL HISTORY 6-year-old female with headache, vomiting, and
ataxia.
FINDINGS Figure 271-1. Axial NCCT through the pos- transependymal flow of cerebrospinal fluid (CSF) associ-
terior fossa. There is a heterogeneous hyperdense mass ated with obstructive hydrocephalus. Figure 271-2. Axial
(white arrow) with central hypodensity indicating intratu- T2WI through the mass. The mass is isointense to gray mat-
moral cysts or necrosis. Expansion of the fourth ventricle ter (GM) (arrow) with hyperintense intratumoral cysts or
around the tumor indicates this is an intraventricular mass. necrosis. Figure 271-3. Axial post-contrast T1WI through
Periventricular hypodensity (black line arrows) reflects the mass. There is mild enhancement of the solid component
583

584 Case 271
of the mass (arrow). Figure 271-4. Axial DWI through the nodularity, anaplastic medulloblastoma, and large cell
mass. The mass demonstrates restricted diffusion (arrow). medulloblastoma. Most common symptoms include head-
ache, vomiting, and ataxia that develop over a relatively
DIFFERENTIAL DIAGNOSISEpendymoma, atypical short time frame, typically less than 3 months.
teratoid/rhabdoid tumor (AT/RhT), choroid plexus papil-

loma, pilocytic astrocytoma, and exophytic brainstem glioma. Questions for Further Thought
1. What additional imaging study is indicated in a patient
DIAGNOSIS Medulloblastoma. with new diagnosis of medulloblastoma?
2. How is medulloblastoma differentiated from ependy-
DISCUSSION Due to its hypercellularity, on CT, medul- moma and pilocytic astrocytoma based on their location
loblastoma usually manifests as solid contrast-enhancing of origin?
hyperdense mass. On MRI, it demonstrates T1 hypointensity
and T2 isointensity similar to GM. Greater than 90% of the
tumors demonstrate contrast enhancement which can be mild
Direct reporting is necessary in this highly malignant tumor.
to strong. Restricted diffusion is commonly seen probably
Differentiate medulloblastoma from other posterior fossa
related to the hypercellularity of the tumor. Calcification can
tumors. Assess complications such as hydrocephalus, CSF
be seen in 20% of cases but hemorrhage is rare. Most pos-
seeding with leptomeningeal enhancement.
terior fossa tumors in the pediatric age group are located at
midline. In contrast, posterior fossa tumors in older children
and adults, which are rare, often arise laterally in the cerebel-
lar hemispheres. Differentiation of medulloblastoma from other posterior
Medulloblastoma is indistinguishable from AT/RhT on fossa tumors
imaging. However, AT/RhT is seen in younger children usu- Accurate assessment of extent of tumor
ally less than 3 years of age. PA tends to be more complex Exclude subarachnoid tumor seeding in the entire neu-
in character, while brainstem gliomas tend not to enhance roaxis
unless transformation to a higher grade has occurred. Spinal imaging is required for exclusion of tumor seeding
Medulloblastoma is the most common posterior fossa
tumor in children, particularly those between 4 and 11 years Answers
old. Most medulloblastoma is thought to arise from cell rests 1. As many as 50% of patients may have subarachnoid
of posterior medullary velum at the roof of the fourth ven- tumor spread at initial diagnosis. It is therefore prudent to
tricle. The origin has a genetic component, and the tumor can image the entire neuroaxis including the entire spine for
be associated with a number of familial cancer syndromes complete workup.
including Gorlin (nevoid basal cell carcinoma) syndrome, 2. Both medulloblastoma and ependymoma often pres-
Li-Fraumeni syndrome, Turcot syndrome, Gardner ent as intraventricular mass, causing expansion of the
syndrome, and Cowden syndrome. It is a highly malignant fourth ventricle. Medulloblastoma classically arises from
WHO IV tumor. It includes five subtypes which cannot be the cerebellar vermis at the roof of the fourth ventricle.
differentiated reliably based on imaging. Classic medullo- Ependymoma arises from the floor of the fourth ventricle.
blastoma is the most common. Other subtypes include des- PA typically arises from the cerebellar vermis and there-
moplastic meduloblastoma, medulloblastoma with e xtensive fore effaces the fourth ventricle rather than expanding it.

Case
272 CLINICAL HISTORY18-year-old male on multimodality treatment
including methotrexate for acute lymphoblastic leukemia with new onset of
confusion, disorientation, and memory loss.
585

586 Case 272
FINDINGS Figures 272-1 and 272-2. Axial DWI with cor- and intramyelinic edema is the postulated pathophysiologic
responding ADC map through the genu of corpus callosum mechanism.
(CC). There is restricted diffusion in the genu of the CC Clinical presentation of MTX LE may include a pattern
(vertical arrow) with almost symmetrical restricted diffusion of deficits in attention, memory, visuospatial skills, executive
in the bilateral frontal corona radiata (transverse arrows). function, and emotional status; a form of encephalopathy;
Figures 272-3 and 272-4. Axial T2 FLAIR and T2WI and the absence of aphasia. Treatment is usually by with-
through the same level show subtle hyperintensity in the CC holding the medication or reducing the dosage. These lesions
and white matter (WM)! Other lesions not shown include tend to disappear as fast as they appear following cessation
bilateral multifocal cerebellar T2 hyperintensities. A repeat of the medication.
study 2 days after these images showed that lesions were fad-
ing on DWI with isointense areas on FLAIR and T2WI. Questions for Further Thought
1. Is there any correlation between clinical outcomes and
DIFFERENTIAL DIAGNOSIS Posterior reversible encep MRI findings in toxic LE?
halopathy syndrome (PRES), methotrexate leukoencepha- 2. What are the histologic findings in toxic LE?
lopathy (MTX LE), infarct.
DIAGNOSIS Corpus callosum Methotrexate leukoenceph- This requires direct reporting. Stopping the medication or
alopathy (MTX LE). reducing the dosage leads to improvement of not only the
clinical symptoms but also the imaging findings.
DISCUSSION MRI is the procedure of choice for imaging
MTX LE or other toxic LE because of its superior ability to
display WM lesions. The MRI findings in MTX LE have been
described elsewhere (refer to Case 16-7). Unilateral lesions 1. Location of lesions
and lesions isolated to the CC or specific areas (anterior or pos- 2. Toxic LE should be part of the differential diagnosis of any
terior) of the Centrum semiovale (CSO) have been reported. patient who presents with acute or chronic encephalopathy
There is usually no mass effect, and contrast enhancement has with a known exposure to agents that are toxic to the cere-
not been a feature. For unclear reasons, toxic-metabolic causes bral WM. It is not unusual for these cases of acute or chronic
of isolated CC lesions have been reported to affect more often LE to be confused with psychiatric diseases clinically
the splenium followed by the genu of the CC. Toxic LE shows 3. There is a vast array of known causes and potential mim-
restricted diffusion. The basic pathophysiologic mechanisms ics of toxic LE, and therefore, the differential diagnosis is
leading to restricted diffusion in MTX toxicity are unknown. broad and should include a number of yet undetermined
The offending agent might cause a direct injury on the micro- causes
vasculature or indirectly through excitotoxic effects. The
lesion usually disappears once the offending agent has been Answers
removed, suggesting that this may not be an infarctthe com- 1. The extent of abnormalities on initial MRI of the brain does
monest reason for restricted diffusion in the brain. Transient not correlate with the clinical outcome. This underscores
lesions in the CC particularly in the splenium have also been the unpredictability of the syndrome and the importance of
reported in association with some infections such as influ- early recognition in case of subtle clinical symptoms.
enza, rotaviruses, Puumala hantavirus, O-157 Escherichia 2. Histological findings in toxic demyelination are variable
coli, Salmonella, Staphylococcus aureus, Klebsiella, and and include demyelination, myelin pallor, vacuolation,
Legionnaires infection. Increased cytokine level in the blood axonal spheroids, and macrophage infiltration with necro-
and cerebrospinal fluid (CSF) with secondary vasodilation sis at the end of the severity spectrum.

Case
273 CLINICAL HISTORY Term baby with history of asphyxia.
FINDINGS Figure 273-1. Axial non-contrast T1WI through perirolandic cortical gray matter (GM) hyperintensity (arrows).
the basal ganglia. There is bilateral symmetrical basal gan- Figures 273-3 and 273-4. Axial DWI through the basal gan-
glia hyperintensity (arrows) with involvement of the internal glia and the high convexities, respectively. There is diffusion
capsules and lateral thalami. Figure 273-2. Axial non-con- restriction (arrows) in the bilateral basal ganglia and the periro-
trast T1WI through the high convexities. There is bilateral landic cortical GM as in Figures 273-1 and 273-2.
587

588 Case 273
DIFFERENTIAL DIAGNOSIS Trauma with diffuse brain ischemic injuries in the superficial venous system territory
swelling, hypoglycemia, maple syrup urine disease, and due to a mechanical injury during birth. The deep venous
nonketotic hyperglycinemia (NKHG), hypoxic-ischemic system territory may be involved due to systemic causes
encephalopathy (HIE) in term baby. such as hypercoagulability, sepsis, or dehydration. A clinical
history of peripartum asphyxia, traumatic delivery, neonatal
DIAGNOSIS HIE in term infants. resuscitation, seizures, abnormal neurologic status, and/or a
low APGAR score can be associated with HIE. Clinical find-
DISCUSSION MRI remains the imaging technique of ings are related to the structures involved and vary from mild
choice for the evaluation of infants with suspected HIE. On to severe neurologic impairment, seizures, and later, devel-
T1WI the abnormal GM may be hyperintense and the white opmental delay.
matter (WM) hypointense, while on T2WI GM signal can The neonatal brain has a remarkable potential for recov-
be variable and WM may be hyperintense due to cytotoxic ery when therapeutic measures are promptly applied, includ-
edema. DWI is sensitive for detecting acute HIE showing ing supportive care measures, hypothermia, and the use of
restricted diffusion. GRE and/or SWI detect hemorrhage. neuroprotective agents such as calcium channel blockers,
Proton MRS depicts the anaerobic metabolism revealing magnesium, and nitric oxide inhibitors.
elevated lactate and diminished N-acetyl aspartate (NAA)
(implying neuronal damage) concentrations. Transcranial Question for Further Thought
ultrasound plays a limited role in the initial detection of HIE 1. What is the distribution of ischemic lesions in the preterm
and may show increased echogenicity in the regions previ- brain?
ously mentioned as will decreased blood flow on Doppler
US. The changes in hypoglycemia and NKHG are very Reporting Responsibilities
similar to HIE while trauma with diffuse brain swelling may This is an acute situation and requires direct reporting.
show patchy nonsymmetrical changes. Identify the extent and pattern of the acute ischemic lesions
Since the neonatal brain is extremely vulnerable to isch- and describe any associated complication such as hemorrhage.
emia, the degree of brain maturity, and severity and dura-
tion of insult cause distinct patterns of brain lesions. The
degree of brain maturation dictates the regional metabolism
and configuration of the vascular system. In term infants Extent and pattern of changes
the central nervous systems vascular supply is similar to Presence of complications such as parenchymal and intra-
that of adults and the hypermetabolic structures include the ventricular hemorrhage and brain swelling
deep GM (lateral thalami, globus pallidus, posterior putam-
ina, and hippocampi), the brainstem, the sensorimotor cor- Answer
tex, and the myelinated WM fibers. Ischemic lesions result 1. In preterm infants with severe hypoperfusion the deep
from decreased arterial blood flow or difficulty in venous GM (especially the thalami) and the brainstem are usually
drainage. Severe arterial hypotension tends to involve these affected as they are the most metabolically active struc-
regions, while milder situations allow for redistribution of tures. Mild or moderate ischemic episodes cause periven-
cerebral blood flow to these vulnerable structures and away tricular leukomalacia, because unlike the term infant, the
from the watershed zones causing ischemic injuries at the vascular supply is ventriculopetal since penetrating arter-
watershed regions. Venous sinus thrombosis can cause ies extend inward from the surface of the brain.

Case
274 CLINICAL HISTORY Adult with treated hydrocephalus due to
intraventricular obstruction.
Figure 274-1
Figure 274-2
DIFFERENTIAL DIAGNOSIS Colloid cyst, intraventricu-

lar neurocysticercosis (INC), xanthogranuloma.
DIAGNOSIS Intraventricular neurocysticercosis (INC).
DISCUSSION INC more commonly occurs in the fourth

ventricle in between 54% and 64% of INC followed by
the third and lateral ventricles. INC forms part of multi-
compartmental lesions in about 33%. MRI features vary
and may include cerebrospinal fluid (CSF) intensity on all
sequences making it difficult to identify and hyperinten-
Figure 274-3
sity on T1WI and FLAIR due to the high protein content
in some. While the majority may not show enhancement,
FINDINGS Figure 274-1. Sagittal T1WI. There is a round thick irregular ring-like enhancement mimicking neoplasm
hyperintense mass within the third ventricle (vertical arrow). is not unusual in degenerating cysts. INC could be freely
Two anterior knobbly components show isointensity and mobile and change location due to change in head posi-
hyperintensity (transverse arrow). Figure 274-2. Axial GRE tion. INCs have been known to migrate from the lateral
through the temporal lobes. There is a target hypointense ring to the third and fourth ventricles. Calcification is uncom-
in the right temporal horn (arrow). Figure 274-3. Axial FLAIR mon in INC. When a scolex is present as in Cysticercus
through the third ventricle. There is a round hyperintensity cellulosae infection, such scolex is hyperintense on T1WI
within the anterior third ventricle (transverse arrow). There is and hypointense on GRE. Evolution through the calcified
a similar but smaller round hyperintensity in the left trigone stage in such a situation may present ring hypointensity on
(vertical arrow) with asymmetric dilatation of the left trigone. GRE. Hydrocephalus and ventriculitis may be present in up
589

590 Case 274
to 70% and 79%, respectively. The hydrocephalus is usu- Question for Further Thought
ally due to aqueductal stenosis or obstruction of any of the 1. Are there differences between the various forms of the
foramina. Ependymal enhancement in the aqueduct may be Cysticercus larva?
due to coexistent ependymitis or adhesion surrounding the
cyst. CT does not show INC because of its CSF density. Reporting Responsibilities
However, presence of a scolex usually manifest as a focal Direct reporting is important as these lesions are known to
hyperdensity (calcification). be very mobile and can move and block CSF pathways pro-
INC may not be distinguishable from other intraven- ducing acute hydrocephalus. Location and number if more
tricular lesions that do not contrast enhance such as colloid than one and presence of hydrocephalus should be reported.
cyst, epidermoid tumor, or arachnoid cyst. However, pres- Presence of cysts in different stages of evolution elsewhere
ence of coexisting lesions elsewhere in the brain suggests in the brain helps confirm the diagnosis.
the diagnosis.
Neurocysticercosis is usually caused by ingestion of the
egg of Taenia solium. It involves the central nervous system
(CNS) in 60% to 90% of patients with cysticercosis. The egg Location, size, and number if more than one
hatches in the stomach into Cysticercus larvae called onco- Presence of parenchymal and subarachnoid cysts
spheres which find their way into the brain via the blood- Is there hydrocephalus?
stream to form cysts (cysticerci) in the brain parenchyma, Is it safe to perform lumbar puncture (LP)?
subarachnoid space, or within the ventricles. INC may be
silent and only become symptomatic in the presence of Answer
hydrocephalus. Headache, drowsiness, papilledema, vom- 1. The two common Cysticerci are C. cellulosae and
iting, and seizures are some of the usual presentations of Cysticercus racemosus. The C. cellulose has a scolex and
symptomatic INC. Just as in neurocysticercosis in general, one vesicle usually not larger than 2 cm. The scolex is
INC is more common in underdeveloped world particularly hyperdense on CT and hypointense on GRE and T2WI
in Latin America. Diagnosis could be confirmed by ELISA but hyperintense on T1WI. It is the more common larva
of the serum or electroimmunotransfer blot (EITB) of the within the parenchyma. The C. racemosus on the other
serum and the CSF. INC responds to treatment with alben- hand has no scolex and propagates by wall proliferation
dazole but not with praziquantel. Patients may also require resulting in a multicystic mass reaching enormous size up
ventriculoperitoneal shunt for hydrocephalus as happened to 6 cm (the racemose type) usually within the subarach-
in this patient. The indications for surgical removal of INC noid space and particularly the basal cisterns and the syl-
include large size, cyst growth, obstruction of CSF path- vian fissures where it can cause arachnoiditis, extensive
ways, or if they complicate shunt function. contrast enhancement, and obstruction to CSF absorption.

Case
275 CLINICAL HISTORY Normal anatomic variants of the circle
of Willis (COW).
A2
MCA
ICA
PCA
Figure 275-2
Figure 275-1

591

592 Case 275
FINDINGS All images are MIP or volume-rendered 3D the tip of the funnel. This should be differentiated from
TOF MRA of the head without contrast rotated to give maxi- an aneurysm that arises usually from the side of the origin of
mum visualization of the structures intended. the P-Com. The A1 could be hypoplastic in about 10% and
Figure 275-1. Adult-type complete COW. Bilateral A1 absent in up to 2%. The A-Com is rarely absent in up to 5%.
(first portion of the anterior cerebral artery [ACA] before Other variations in the COW could include hypoplasia of
the anterior communicating artery [A-Com] vertical any of the arteries and presence of duplications and fenestra-
arrows), bilateral asymmetric posterior communicat- tions. Accessory arteries such as accessory A2, duplication
ing arteries (P-Com) (transverse arrows), and bilateral P1 of MCA, and A-Com have been reported.
(proximal posterior cerebral artery [PCA] from the basilar Both CTA and MRA offer excellent details of the COW.
arteryline arrows) are visualized. The A-Com joins the The source axial images should be scrutinized first as this is
bilateral A1 in the midline. Figure 275-2. This is the more the plane that neuroradiologists are most familial with fol-
common pattern where the P-Com is not visualized on the lowed by the MIP and volume-rendered 3D images. Better
MIP images but visible on the source images (not shown). evaluation could also be done by segmentation of the ante-
Figure 275-3. The right P-Com is visualized but not the left rior and posterior circulations separately.
on this volume-rendered image. The left P-Com is visible
on the source images (not shown). Figure 275-4. The left A1 Questions for Further Thought
(arrow points to location) and the left P1 (arrow head points 1. At what point is the COW development completed emby-
to location) are hypoplastic or absent. The left PCA has a ologically?
direct origin from the left internal carotid artery (ICA). The 2. Are there specific COW anatomic variants associated
A-Com (line arrow) serves as the conduit of blood to the with disease?
left A2. Figure 275-5. Funnel-shaped or infundibular ori-
gin (transverse arrow) of direct PCA from ICA. The P-Com Reporting Responsibilities
and PCA are of same size (vertical arrows). Figure 275-6. A detailed description of the COW on CTA or MRA is
Infundibular origin (transverse arrow) of P-Com from ICA. always necessary; describe what is present, absent, hypo-
P-Com (vertical arrow) extends from the tip of the funnel. plastic, narrowed, occluded, or aneurysmal and whether
there is duplication or fenestration as these variants could
DIFFERENTIAL DIAGNOSIS N/A. help explain clinical situations or useful in planning patient
management.
DIAGNOSIS Normal variant COW.
DISCUSSION The COW is a closed circuit connection The status of the major arteriesICA and the vertebro-
of all intracranial major arteries including bilateral ICA basilar system
and the vertebrobasilar system at the base of the brain first Which artery is present, absent, or inadequately visualized
described in 1662 by Thomas Willis. A complete COW Other anatomic variations such as duplication and fenes-
consists of anterior and posterior segments joined together tration
by communicating arteries that complete the circle. The
anterior segment is formed by the bilateral A1 ACA seg- Answers
ments connected together in the midline by the A-Com. 1. The complete COW is formed by 7 weeks post concep-
The posterior segment is formed by the bilateral PCA aris- tion (CR length 40 mm). Prior to that, the fetal configura-
ing from the basilar artery terminus and joined to the ICA tion dominates where the PCA has a direct origin from the
by the bilateral P-Com, thus forming a closed loop in which ICA. At about 7 weeks the P1 is formed, thus completing
blood could be freely and spontaneously shunted from one the circle. A fully formed PCA is now present and the
vessel to the other within the COW. This arrangement P-Com begins to form by the regression of the fetal PCA
without any significant variation is present in up to 52% of between the ICA and P1P2 junction.
the population. 2. Fenestrations are associated with increasing frequency
The most common variant relates to the P-Com. The of aneurysms but it has also been shown that there is no
P-Com could be rarely absent. A fetal configuration in which statistically significant difference between the general
there is a direct origin of the PCA from the ICA is present population and those with intracranial fenestration with
in between 5.8% and 40%. The P1 is usually hypoplastic regard to aneurysmal formation. Occlusion of the ICA
or rarely absent in this configuration. The adult configura- or basilar artery may predispose the brain to infarctions
tion in which the P-Com is smaller than the P1 is present in in the absence of a complete viable COW. Absence of
between 50% and 76%. The P-Com is equal in size to the P1 the communicating arteries or hypoplasia of the main
in between 7% and 18%. Funnel-shaped enlargement of the component parts of the COW may affect choices in
origin of the P-Com from the ICA up to 3 mm in diameter management options of diseases such as aneurysms and
is known as an infundibulum. The P-Com emanates from cerebrovascular diseases.

Case
276 CLINICAL HISTORY 20-year-old male with history of seizure disorder for
the last 5 years.
FINDINGS Figure 276-1. Axial non-contrast T1WI enhancement. The hypointensity is more profound later-
through the temporal lobes. There is an expanded left tem- ally (arrow) than medially. Figure 276-3. Axial T2 FLAIR.
poral lobe with mild to moderate hypointensity. The abnor- There is diffuse hyperintensity in the expanded left tempo-
mality is greater laterally but extends to the medial aspect ral lobe. The abnormality extends more posteriorly along
of the temporal lobe. There is mass effect on the left cere- the temporal horn and trigone (arrows). Figure 276-4. Axial
bral peduncle (arrow). Figure 276-2. Axial post-contrast ADC map through the mass. There is elevated diffusion
T1WI through same level as Figure 276-1. There is no in the anterior aspect of the mass with restricted diffusion
593

594 Case 276
occurs laterally into the inferior insula and into the inferior
posterior frontal lobe with vasogenic edema-like pattern,
more frequently bilateral than unilateral. HSE can develop
enhancement and hemorrhage. Infarct usually shows homo-
geneous restricted diffusion in a vascular territory unlike
this case. Confounding in this case is the restricted corti-
cal diffusion that is likely due to seizure activity but does
have an appearance somewhat mimicking ischemia. The
lesion does not have the bubbly appearance of DNET or
the well-circumscribed solid and/or cystic appearance of
cortical-based lesions such as ganglioglioma and PXA.
Oligodendroglioma is a distinct possibility with a greater
propensity to invade the cortex than astrocytomas.
Astrocytoma WHO II represents about 10% to 15% of all
astrocytic tumors and tends to occur in a younger popula-
Figure 276-5 tion than astrocytoma WHO III with the majority presenting
in the fourth to fifth decades. It has a slight male prepon-
through the lateral cortex (arrow) with extension into the derance. Seizures, neurologic deficits, visual problems, and
insula (not shown). Figure 276-5. Photomicrograph shows speech impediments are some of the presenting features gen-
slightly cellular tumor with round to mildly irregular hyper- erally on the basis of location. It is preferentially supratento-
chromatic nuclei. rial and frontotemporal in location. Diffuse astrocytoma is
an infiltrating cellular tumor composed of irregularly dis-
DIFFERENTIAL DIAGNOSISInfarct, herpes encephali- tributed astrocytes with slightly irregular, hyperchromatic
tis, astrocytoma WHO II, dysembryoplastic neuroepithelial nuclei. Cells with eccentric nuclei and abundant cytoplasm
tumor (DNET), ganglioglioma, pleomorphic xanthoastrocy- (gemistocytes) are present in variable number. Other fea-
toma (PXA), seizure-induced abnormality or contusion. tures include microcyst formation and subpial, perivascular,
and perineuronal accumulation of neoplastic cells; however,
DIAGNOSIS Diffuse astrocytoma WHO II.
microcalcifications are usually not present. Mitoses are none
to rare. Neither vascular endothelial proliferation nor necro-
DISCUSSION Astrocytoma WHO II has classic imaging
sis is a feature of astrocytoma WHO II. Astrocytomas are
characteristics for a brain lesion. It is poorly defined and
positive for glial fibrillary acidic protein (GFAP). Most (75%
hypodense on CT usually without contrast enhancement.
to 80%) of the diffuse (grade II and III) astrocytomas dem-
There may be mild mass effect but unusually herniation. It
onstrate mutations of the isocitrate dehydrogenase (1 or 2)
is hypointense on T1WI and diffusely hyperintense on T2WI
gene. TP53 mutation is also common.
with local mass effect. The appearance tends to be poorly
defined and homogeneous. There is usually no calcification,
hemorrhage, or associated edema. Areas of hypercellular-
ity, which are rare, can present as areas of hyperdensity on 1. Does extent of resection affect survival?
CT and hypointensity on T2WI. About 70% to 80% do not
enhance. Enhancement is present when it transforms to a Reporting Responsibilities
higher grade or anaplastic tumor. However, a small percent- Direct reporting is important for all tumors. Location and
age of the astrocytoma WHO II can have MRI enhancement. size of mass and its proximity to eloquent areas should be
Enhancement is a sign of poor prognosis. It is predominantly described. Presence of significant mass effect, herniation
a white matter (WM)-based lesion, but this case demonstrates risks, and growth rate if follow-up are important.
that it can have extensive gray matter involvement, and gray
matter involvement might be compounded by associated What the Treating Physician Needs to Know
seizure-induced changes. The ADC values tend to be higher Are there findings indicating a need for lumbar puncture
while higher-grade gliomas can have areas of restricted dif- such as findings indicating encephalitis or leptomeningeal
fusion that can be related to areas of hypercellulartiy. WHO disease?
II tends to have lower perfusion than higher grade astrocy- If a lumbar puncture is needed is it safe?
toma WHO III and glioblastoma. Elevated perfusion is also Location and size will affect management
associated with decreased survival in astrocytoma. There How can advanced imaging help?
is decreased N-acetyl aspartate (NAA), decreased creatine
(Cr), and increased choline (Cho) peaks at MRS with con- Answer
spicuous absence of lipid and lactate peaks. When diffusely 1. Greater extent of resection of low-grade gliomas has been
infiltrating the MRS changes can be minimal. associated with improving overall survival. Greater extent
Herpes simplex encephalitis (HSE) is predominantly of resection is also associated with delay of progression
mesial temporal in origin and location. Extension of HSE and malignant transformation.

Case
277 CLINICAL HISTORY 5-year-old male with neurofibromatosis has
nystagmus.
Figure 277-2
Figure 277-1
FINDINGS Figure 277-1. Axial FLAIR MRI through the DISCUSSION MRI is the imaging modality of choice
suprasellar region. There is a 1.3 cm 2 cm mildly lobulated in the primary evaluation of OPGs and also in monitor-
isointense mass in the region of the chiasm (arrows). Figure ing the evolution of the lesion and response to treatment.
277-2. Coronal T2WI slightly anteriorly to the chiasm. There T1WI usually shows isointense to hypointense mass which
is thickening of bilateral isointense (to gray matter [GM]) is hyperintense on T2WI. Contrast enhancement is usually
optic nerves (arrows). Figure 277-3. Axial T1WI through the homogeneous but could be variable with cystic degenera-
suprasellar cistern. There is an isointense mass in the chi- tion. Sometimes there could be a complete lack of enhance-
asm with bilateral posterior optic nerves thickening (arrows). ment. Leptomeningeal spread resulting in leptomeningeal
Figure 277-4. Coronal post-contrast T1WI through the mass. enhancement is present in a very small percentage of patients.
There is homogeneous contrast enhancement of the mass NCCT usually demonstrates fusiform isodense enlarge-
(transverse arrows). The mass is clearly separated from the ment of the optic nerves. Chiasmatic involvement is usually
pituitary gland (vertical arrow). a large often lobulated suprasellar mass that may calcify.
Contrast enhancement is variable. Bone window setting on
DIFFERENTIAL DIAGNOSISOptic pathway glioma CT may reveal widening of the optic canal. Hydrocephalus
(OPG), lymphoma, metastasis, sarcoidosis, granuloma, cra- may be present in large tumors if there is ventricular obstruc-
niopharyngioma. tion. OPG can be sporadic or associated with neurofibroma-
tosis type 1 (NF1) as in this child. OPGs can involve the
DIAGNOSIS Optic pathway glioma (OPG). optic nerve anywhere along its course from the optic nerve
595

596 Case 277
head to the occipital visual cortex. About 10% of OPGs are and tumors confined to the orbit or unilateral optic nerve.
located within an optic nerve, about 33% involve both optic Regression of OPG has been reported after partial resec-
nerves and chiasm, and another 33% involve predominantly tion, chemotherapy, radiotherapy, or biopsy, and some-
the chiasm itself while about 25% is predominantly in the times without any treatment.
hypothalamus. Optic nerve involvement is more common in
NF1-associated OPG, whereas chiasmal and postchiasmal Questions for Further Thought
lesions are more frequently seen in sporadic OPG. Extension 1. Should NF1 patient be screened for development of OPG?
beyond the optic pathway at diagnosis is uncommon in the 2. Should optic nerve glioma be treated?
NF1-associated OPG but frequent in the non-NF1 group.
The presence of cystic components is significantly more
common in the non-NF patients. OPG in the NF1 group is Reporting Responsibilities
more likely to be smaller with preservation of the original Direct reporting is necessary for this neoplasm. Location,
shape of the optic pathways. extent, and complications such as proptosis, hydrocephalus,
OPG is present in 11% to 30% of patients with NF1. The and significant mass effects should be reported. Cerebrospinal
period of greatest risk for the development of symptomatic fluid (CSF) seeding from occasional malignant degeneration
OPG in NF1 is during the first 6 years of life. OPGs arising in should be reported if present.
children with NF1 are low-grade WHO I astrocytomas (pilo-
cytic astrocytomas [PAs]) and are histologically identical to What the Treating Physician Needs to Know
PAs arising elsewhere in the brain in children without NF1. Location, size, and number if multicentric or multiple
Some NF1-associated OPGs, however, lack classic features Complications
of PA and may be classified as WHO II fibrillary astrocy-
toma. OPGs represent approximately 3% to 5% of childhood Answers
intracranial tumors occurring more frequently within the first 1. This is a controversial topic without any consensus as
6 years of life without any gender preference. Although the to how to proceed. Some centers do screen for OPG in
tumors of many patients with NF1-associated OPGs exhibit NF1. There is no conclusive evidence that early detec-
indolent behavior, up to 50% of these tumors will be symp- tion of OPG in NF1 would reduce the rate of vision loss.
tomatic presenting with vision loss, proptosis, and preco- Asymptomatic OPG would be identified that would
cious puberty. never progress, escalating costs and parental anxiety, and
The clinical course and natural history are highly vari- exposing the young child to the risks of repeated sedation
able, making treatment paradigms difficult. OPGs can be for imaging or ophthalmologic examinations.
observed over time for progression without initial treat- 2. OPG may be asymptomatic or may present with symp-
ment, especially in patients with NF1. Chemotherapy is toms that vary depending on the location. No treatment
the first-line treatment for progressive tumors, and radia- may be required in patients with no growth, good vision,
tion therapy is reserved for patients with progressive dis- and no cosmetic deformity. It is recommended that a
ease who are older than 5 to 7 years. Surgery is reserved treatment plan must be carefully individualized for each
for large tumors causing mass effect or hydrocephalus patient.

Case 278 CLINICAL HISTORY 6-year-old female with a history of medulloblastoma.
Figure 278-3
Figure 278-4
597

598 Case 278
FINDINGS Figure 278-1. Axial FLAIR through the lateral tumor and the lack of contrast enhancement excludes cystic
ventricles. There is distension of the left lateral ventricle by neoplastic tumors.
a large CSF intensity cyst (star) which displaces the septum Intraventricular cyst is generally asymptomatic and dis-
pellucidum medially (transverse arrow) and the left choroid covered incidentally as in this case which has been followed
plexus posterolaterally (vertical arrow). It is difficult to make up for a number of years while the underlying medullo-
out the cyst wall except where it compresses the ventricu- blastoma in this child is followed up. When a cyst becomes
lar walls laterally and medially. Figure 278-2. Axial T2WI symptomatic, the patient may present with headache, signs
through the trigones. The cyst is of CSF intensity with a and symptoms of obstructive hydrocephalus, focal neuro-
very thin dark membranous wall (arrows). There is dilatation logic deficit, or seizures. Intraventricular cyst represents a
ofthe left occipital horn. Figure 278-3. Coronal post-contrast number of different simple cystic structures differing only
T1WI. The outline of the cyst (star) blends with the com- in the histology of the wall. If treatment is ever desired on
pressed lateral ventricular wall. The displaced enhancing left account of being symptomatic, simple endoscopic fenestra-
choroid plexus is compressed against the lateral ventricu- tion or removal has been recommended.
lar wall (arrow). Figure 278-4. DTI color directional map
through the lesion. There is compression and attenuation of Question for Further Thought
the left inferior fronto-occipital fasciculus and the inferior 1. What is the origin of intraventricular simple cyst?
longitudinal fasciculus (arrow).
DIFFERENTIAL DIAGNOSIS Intraventricular simple
These are usually benign nonneoplastic lesions. Routine
cyst, neuroepithelial cyst, intraventricular arachnoid cyst,
reporting is sufficient. Location, size, and presence of
choroid plexus cyst, intraventricular neurocysticercosis.
obstruction to CSF pathway or complication should be
reported. Change in size and geography on follow-up should
DIAGNOSIS Intraventricular simple cyst.
also be noted.
DISCUSSION Intraventricular simple cyst often called
intraventricular arachnoid cyst could resemble all the
entities listed in the differential diagnosis. There is no Location and size
way of clearly distinguishing between them at imaging. Presence of obstruction or complication
Intraventricular neurocysticercosis, however, has been Usually requires no treatment if asymptomatic
known to shift position on different images. This cyst fol-
lows CSF intensity on all MRI sequences and on CT. There Answer
is usually no restricted diffusion. Like other arachnoid cysts 1. The origin of the cyst is controversial. It may derive from
in the subarachnoid space (SAS), it could be complicated by arachnoid tissue within the ventricular system or as an
hemorrhage, but this is unusual. They are mostly located in extension of an arachnoid cyst in the contiguous choroidal
the trigone where they displace the choroid plexus and may fissure lined by flattened arachnoid epithelial tissue. It has
rarely obstruct the temporal and occipital horns. Mass effect also been suggested that it could originate from vascular
on surrounding structures as demonstrated on the DTI is fre- mesenchyme or from the ependyma as ependymal cyst
quent with large cysts. The CSF intensity on MRI with lack which are lined by tall columnar epithelium or by cuboi-
of restricted diffusion sets this cyst apart from epidermoid dal choroidal cells.

Case
279 CLINICAL HISTORY 41-year-old male with left scalp pain and
tenderness.
FINDINGS Figure 279-1. Axial NCCT through the cra- Question for Further Thought
nial vault bone window. There is a wide region of mottled 1. What is chronic osteomyelitis?
hypodensity in the left parietal bone with smaller similar
areas elsewhere (arrows). Figure 279-2. Axial post-contrast
T1WI through the same region. There is enhancement within Reporting Responsibilities
the diploic space which correlates with hypodensities on CT. Direct reporting is essential in suspected osteomyelitis. Raise
There is perhaps underlying epidural thickening (arrows). the question of osteomyelitis when bony lucency and mar-
row replacement are seen, but do not neglect to mention
DIFFERENTIAL DIAGNOSIS Multiple myeloma, metas- other possibilities as these findings are by no means spe-
tases, osteomyelitis, eosinophilic granuloma. cific. Identify and characterize any associated complications,
such as extracranial or epidural/subgaleal abscess, subdural
DIAGNOSIS Osteomyelitis. empyema, and sinus thrombosis.
DISCUSSION Osteomyelitis of the calvarium and skull What the Treating Physician Needs to Know
base presents radiologically much the same as would osteo- The imaging appearance of calvarial and skull base osteo-
myelitis anywhere else in the skeletal system. CT will show myelitis is not specific. Without the benefit of clues, such
lysis with areas of irregular hypodensity, sometimes with as ongoing otitis/mastoiditis, or frank abscess formation, a
patchy areas of sclerosis (hyperdensity) indicating an attempt broad differential including benign and malignant etiolo-
at repair. Loss of cortical bone is always present. MRI will gies may be all that imaging can provide. Biopsy is fre-
show marrow replacement on T1WI and a variable degree quently necessary to make the diagnosis
of edemahyperintensity on T2WI. Osteomyelitis usually Location and extent of lesions are usually necessary for
enhances. Associated complications such as extracranial or surgical planning
epidural abscess, subdural empyema, and sinus thrombosis Complications if any
may be present. When encountered in the head, osteomyelitis
is often secondary to infection of the middle ear or mastoids.
The presence of adjacent otitis or mastoiditis would narrow Answer
the differential for a lucent or marrow replacing bony lesion 1. Chronic osteomyelitis is a low-level, smoldering infection
which would otherwise elicit a broad differential including of the bone which classically presents as a focal mixed den-
neoplastic etiologies. Osteomyelitis can also be seen in the sity lesion on CT containing a sequestrum of dead, sclerotic
postoperative state, where it may affect a craniotomy flap or bone. Occasionally, a sinus tract may be present draining
burr hole, or after trauma. the contained bony collection of pus to the skin surface.
599

Case 280 CLINICAL HISTORY Adult male with developmental delay.
MRI shows a homogeneous, hyperintense mass on T1WI

and T2WI, hypointense with fat suppression and usually
without contrast enhancement. The surrounding calcification
is hypointense on GRE. TOF MRI might show some vascu-
lar abnormalities.
IC lipoma is a rare and benign congenital malformation
of the brain rather than a neoplasm. They develop due to
abnormal persistence and maldifferentiation of the embry-
onic meninx primitiva during development of thesub-
arachnoid cisterns and are composed of adipose tissue
that is not normally present in the central nervous system
(CNS). They are generally asymptomatic and generally do
not require any treatment. The symptomatic ones might
cause compressive symptoms or seizures depending on the
location. Most lipomas occur at or near the midline typi-
cally in the pericallosal cistern as in this patient accounting
for 45% of all cases. Large CC lipomas may sometimes
extend into the lateral ventricles and choroid plexus. There
are two types of IC lipomas, tubulonodular and curvilinear.
Tubulonodular is anteriorly situated, round or cylinder-
shaped generally greater than 2 cm in diameter and have a
high incidence of CC dysgenesis, frontal lobe anomalies,
and frontal cephaloceles. Curvilinear lipoma is thin, pos-
teriorly situated, and curving around the splenium and
Figure 280-1 is generally associated with a normal CC with a low inci-
dence of associated anomalies. The remainder of the IC
lipomas tends to be found in the quadrigeminal/superior
FINDINGS Figure 280-1. Axial NCCT through the lateral cerebellar cistern (25%) suprasellar/interpeduncular cis-
ventricles. There is a large hypodensity (HU in the region tern (14%), cerebellopontine angle cistern (9%), and syl-
of fat) (star) surrounded by thick irregular linear peripheral vian cistern (5%). Rarely, they can be found on the surface
hyperdensitycalcification(arrows) replacing the genu
of the cerebral hemispheres.
and body of the corpus callosum (CC). There is wide separa-
tion of the frontal horns. Question for Further Thought
1. What is the differential diagnosis of the hyperdensity sur-
DIFFERENTIAL DIAGNOSIS Midline cyst in agenesis of rounding the lipoma?
CC, lipoma in agenesis of CC, abscess.
DIAGNOSIS Lipoma in agenesis of CC. Routine reporting is sufficient. It is however important to
distinguish this from pneumocephalus and to identify the
DISCUSSION NCCT of intracranial (IC) lipomas pres- associated CC dysgenesis.
ent a well-defined hypodensity that does not enhance with
contrast. Calcifications might be present peripherally as
hyperdensity surrounding the hypodense lipoma mostly in Location and associated anomalies
the interhemispheric fissure or replacing the CC. Depending Associated congenital CNS abnormalities may include
on the history, it may be pertinent to distinguish this hypoden- agenesis or dysgenesis of the CC (most frequent
sity from pneumocephalus or midline cyst. Hounsfield mea- abnormality), absence of the septum pellucidum, cranium
surement usually shows a lipoma in the range of 100 to bifidum, spinal bifida, encephalocele, myelomeningocele,
200 HU, air in pneumocephalus is usually in the range of hypoplasia of the vermis, and malformation of the cortex
900 to 1000 HU, and midline cyst always correlate as the lipoma may interfere with the growth of cortical
with cerebrospinal fluid (CSF) measurement at or near 0. tissue. Abnormal or prominent drainage has been described
600

Case 280 601
together with cortical dysplasia. Associated arterial abnor- Answer

malities such as azygos ACA, aneurysms, arteriovenous 1. The differential diagnosis of surrounding hyperdensity
malformations, and veins with abnormal drainage have includes calcification and hemorrhage. It is important in
been reported the context of trauma to exclude pneumocephalus with
Treatment is generally not necessary. Symptoms are gen- surrounding hemorrhage simply by taking the Hounsfield
erally not due to the lipomas but the underlying associated measurement.
anomaly

Case
281 CLINICAL HISTORY 36-year-old male with no significant previous medical
history, developed fast progressing weakness in his right arm and right leg
associated with headaches over the prior 2 days.
Figure 281-2
Figure 281-1
602

Case 281 603
Figure 281-5
Figure 281-6
FINDINGS Figure 281-1. Axial non-contrast T1WI through On MRI, the toxo is isointense to hypointense on T1WI.
the vertex. There is a left parietal parafalcine cortical some- Lesions tend to show concentric walls of alternating isoin-
what isointense (to surrounding gray matter) round lesion tense and hyperintense rings that represent various zones
(arrow). There is a central linear cleft and a thin peripheral of hemorrhage, inflammation, and necrosis, the so-called
hyperintensity. Figure 281-2. Axial MR FLAIR through the concentric ring target sign, on FLAIR and T2WI with sur-
temporal lobes. There are multiple lesions in bilateral tem- rounding hyperintense edema. There is a thin ring-enhancing
poral lobes and right occipital lobe (arrows). Within what lesion with an eccentric enhancing nodule on post-contrast
appears to represent smudgy hyperintensities (edema) are T1WI. The eccentric nodule or so-called eccentric target
well-defined isointense round lesions. Figure281-3. Axial sign represents abnormal vascular leash projecting into
FLAIR through the left parietal lesion. The mass shows alter- the mass. Contrast enhancement may be absent if the CD4
nating rings of hyperintensity and isointensity (concentric count is under 50. More often lesions are multiple and vary
target sign) also demonstrated on T2WI (not shown) (vertical in size from punctate to 3 cm. MRS is nonspecific show-
arrow). There are additional smaller cortical lesions in bilat- ing increased lactate and lipid peaks with reduced N-acetyl
eral frontal cortex (transverse arrows) showing similar con- aspartate (NAA), choline, and creatine. Lesions generally do
centric target sign. Figure 281-4. Axial post-contrast T1WI not restrict diffusion. The concentric target sign on FLAIR
through the basal ganglia. There is a thin ring-enhancing and T2WI, the eccentric target signs on post-contrast T1WI,
lesion in the right lentiform nucleus with a central contrast- and lack of diffusion restriction differentiate toxo from its
enhancing nodule (arrow). Figures 281-5 and 281-6. Coronal mimics particularly PCNSL. Thallium SPECT has a sensi-
post-contrast T1WI through different levels. There are mul- tivity of 100% and 90% specificity in differentiating toxo
tiple infratentorial and supratentorial irregular thin ring- from PCNSL.
enhancing lesions (arrows) with eccentric nodules (eccentric Toxo is an opportunistic infection that may be the
target sign) (line arrow in Figure 281-5). AIDS-defining condition in up to 44% of HIV-infected
patients as in this patient. It is usually a reactivation of
DIFFERENTIAL DIAGNOSISToxoplasmosis (toxo), pyoge indolent Toxoplasma gondii infection. Toxoplasma serop-
nic abscesses, lymphoma (primary CNS lymphoma [PCNSL]), revalence in the United States (15%) is significantly lower
mycobacterial infection (particularly tuberculosis), fungal infec- compared with certain European countries (50% to 75%).
tion, metastases. Patients usually present with headache, fever, mental status
changes, focal neurologic signs, seizures, and manifesta-
DIAGNOSIS Toxo in an HIV patient. tions consistent with encephalitis. Toxoplasma immuno-
globulin G(IgG) was positive (titer 1/2560) in this patient.
DISCUSSION Toxo lesions are generally found in the basal HIV ELISA and Western blot were positive, and the CD4
ganglia, thalami, graywhite matter junction of the cerebral count was 12. The patient was empirically started on
hemispheres, and the cerebellum. They are hypodense with treatment for toxo. Follow-up MRI after 2 weeks showed
smooth ring contrast enhancement on CT. Calcifications of decreased size of lesions and almost resolved surrounding
varying shapes and sizes may be present in treated lesions. vasogenic edema.

604 Case 281
Question for Further Thought Is there a chance that the lesions could be lymphoma? In
1. How is T. gondii transmitted? which case steroid may need to be withheld until after the
biopsy. Lack of response to 2 weeks of treatment may sug-
Reporting Responsibilities gest PCNSL
Direct reporting is essential so that treatment could begin. Thallium SPECT is 100% sensitive in diagnosing PCNSL
This is usually a rapidly evolving disease that could be fatal and may be necessary for differentiating toxo from
if not promptly treated. Significant mass effect should be PCNSL?
reported.
Answer
What the Treating Physician Needs to Know 1. T. gondii is a parasite that is transmitted by accidental
Location of lesion particularly if single and not typical ingestion of oocysts eliminated in feces of cat, the defini-
since a biopsy may be contemplated tive host or after consumption of undercooked meat con-
Is it safe to perform lumbar puncture (LP)? Yes, if there is taining tissue cysts. The oocysts rapidly multiply, and
no significant mass effect the resulting tachyzoites invade the CNS to form cysts
Cerebrospinal fluid (CSF) polymerase chain reaction which are subsequently surrounded by inflammatory
(PCR) is one of the ways to confirm the diagnosis of toxo changes.

Case
282 CLINICAL HISTORY 2-year-old child with progressive psychomotor
retardation and loss of developmental milestones, megalencephaly and
frontal bossing, seizures, hyperreflexia and pyramidal signs, and ataxia.
FINDINGS Figure 282-1. Axial T2WI through the centrum

semiovale. There is extensive confluent frontal and pari-
etal white matter (WM) hyperintensity involving deep and
subcortical regions (stars). Figure 282-2. Axial T2WI at the
level of the lateral ventricles. There is predominant bilateral
frontal lobe WM confluent hyperintensity (stars). Figure
282-3. Axial T2WI through the medulla. Areas of hyper-
intensity (arrow) are seen in the upper medulla too. (Case
courtesy of Dr. Karuna Shekdar, University of Pennsylvania,
Philadelphia.)
DIFFERENTIAL DIAGNOSIS Canavan disease, megalen-

cephalic leukoencephalopathy with subcortical cysts (MLC),
mucopolysaccharidoses (MPS), Alexander disease.
DIAGNOSIS Alexander disease.
DISCUSSION The hallmark of the imaging findings in

Alexander disease is extensive cerebral WM T2 hyperin-
tensity with frontal predominance. There is a periventricular
rim of T1 hyperintensity which is hypointense on T2WI and
often contrast enhances. The enhancement may also involve
the frontal WM, basal ganglia, thalami, parts of the midbrain,
fornix, brainstem, optic chiasm, and dentate nucleus of the
cerebellum. These areas may also demonstrate T2 hyper-
Figure 282-3 intensities. Volume loss is a prominent feature late in the
605

606 Case 282
disease, not only affecting the WM but also the deep gray infant or juvenile diseases with rapid deterioration, direct
matter with cystic degeneration. Hydrocephalus is often a reporting may be appreciated so that prompt genetic testing
feature of Alexander disease. may begin.
First described in 1949 by William Stuart Alexander, a
pathologist in New Zealand, Alexander disease is a rare, What the Treating Physician Needs to Know
sporadic, progressive, and fatal leukodystrophy or WM There is no known treatment, care is supportive and pal-
disease. The classic presentation is of an infant with mac- liative
rocephaly, seizures, and rapid neurologic deterioration. There may be a familial association in many cases of
There are less common juvenile/adult variants. Alexander Alexander disease; therefore, genetic counseling may be
disease is caused by a sporadic mutation of the glial fibril- indicated
lary acid protein (GFAP) gene that maps to chromosome 17.
Histologically, it is characterized by marked cytoplasmic Answers
accumulations containing GFAP and heat shock proteins, 1. There are four described variants: neonatal, infantile,
known as Rosenthal fibers. More specifically, Rosenthal juvenile, and adult forms of the disease. The neona-
fibers are abnormal intracytoplasmic proteinaceous inclu- tal form is very rare and rapidly fulminant with death
sions in fibrous astrocytes; therefore, the disease can be occurring within 2 years. The infantile form is the most
thought of a disease not of WM but of astrocytes proper. common affecting 63% of patients presenting with those
These are present in great quantities throughout the brain classical presentations described above. Affected chil-
in patients with Alexander disease but may also be found dren survive weeks to several years. The juvenile form,
in more limited distribution in other conditions, including 24% of cases, usually presents between ages4and 10
multiple sclerosis (MS), pilocytic astrocytoma, and the lin- years, occasionally in the mid-teens. Findings include
ing of syrinx cavities. Interestingly, the noncommunicating bulbar/pseudobulbar signs, ataxia, gradual loss of
hydrocephalus observed in Alexander disease is caused by intellectual function, seizures, megalencephaly, and

swelling of the astrocytes that line the cerebral aqueduct. breathing problems. Survival ranges from the early teens
The accumulation of Rosenthal fibers eventually produces to the twenties to thirties. The adult form is the most
narrowing or occlusion of the aqueduct. There is no cure for variable both in severity and in progression. Bulbar and
Alexander disease. Treatment is supportive. pseudobulbar symptoms as well as spasticity are seen,
and the disease may resemble MS.
Questions for Further Thought 2. Although adult-onset Alexander disease seems to be
1. What other variants of Alexander disease are known? sporadic in most cases, familial segregation, suggesting
2. Is there an inherited pattern of Alexander disease? an autosomal dominant mode of transmission, has been
reported. In these families, a new mutation in the GFAP
Reporting Responsibilities gene (17q21) has been identified. Affected adults may
Routine reporting is sufficient unless there are some acute transmit the mutation to their offspring in an autosomal
changes such as hydrocephalus. However, in most of these dominant fashion.

Case
283 CLINICAL HISTORY 71-year-old right-handed male presenting with
recurrent episodes of executive dysfunction lasting 5 to 10 seconds.
FINDINGS Figure 283-1. Axial FLAIR through the sple- (black arrow) suggestive of hemorrhagic product or calcifi-
nium of corpus callosum. There is hyperintense enlargement cations. Figure 283-3. Axial relative Cerebral Blood Volume
of the splenium of corpus callosum (CC) (arrow). There is a (rCBV) map. There is relative increased blood volume
thick hyperintense rim surrounding a less hyperintense core. within the splenium mass (arrows). Figure 283-4. Axial DWI
Figure 283-2. Axial GRE through same level as Figure283-1. through the splenium. There is peripheral restricted diffusion
There is irregular hypointensity within the splenium mass in the mass suggesting a highly cellular mass. Figure 283-5.
607

608 Case 283
localize higher-grade components of tumors for stereotactic

biopsy and can also provide a noninvasive estimate of tumor
grade and the true extent of tumor. Measurements of rCBV
have been shown to be useful in assessing response to radio-
therapy in patients treated with stereotactic radiosurgery.
MRS of GB reveals elevated choline and creatine peaks with
decreased N-acetyl aspartate (NAA). The necrotic areas may
exhibit lipid and lactate peaks. DTI helps identify multifocal
brain involvement by GB.
GB was suspected based on the imaging appearance and
confirmed by stereotactic brain biopsy. Unfortunately the
tumor was not resectable due to its location. He received
concomitant radiation and chemotherapy with temozolomide
and adjuvant chemotherapy. He, however, continued to dete-
riorate and repetitive MRI of the brain demonstrated further
progression in the tumor size. He expired 9 months after the
Figure 283-5 diagnosis was made.

Coronal post-contrast T1WI. There is heterogeneous irregu-
lar contrast enhancement (arrow). 1. What is the MGMT gene and what is the significance of
having MGMT promoter methylation?
DIFFERENTIAL DIAGNOSISGlioblastoma (GB), lym-
phoma, demyelinating lesion. Reporting Responsibilities
Direct reporting is required whenever a tumor is sus-
DIAGNOSIS CC GB WHO IV. pected. The location and other associated changes should be
documented.
DISCUSSION GB of the CC tends to present as a butterfly
lesion extending from one hemisphere to the other across the What the Treating Physician Needs to Know
midline. Otherwise the findings are similar to GB elsewhere Location of tumor and its extensions will determine oper-
in the brain. CT scan of the brain is frequently the first imag- ability
ing modality used to assess the brain quickly. On CT, GB is Maximal surgical resection is recommended whenever
an irregular heterogeneous mass with a peritumoral vaso- possible
genic edema and mass effect with internal area of necrosis Stereotactic biopsy rather than surgical resection is done
and possibly calcifications and hemorrhage. Usually there alone if the tumor is in an eloquent area or if the overall
is an irregular heterogeneous contrast enhancement depend- health of the patient is poor and cannot tolerate anesthesia
ing on the degree of necrosis, hemorrhage, and calcification.
MRI of GB shows a heterogeneously hypointense mass on Answer
T1WI which is T2 hyperintense with possible foci of suscep- 1.
O6-methylguanineDNA methyltransferase (MGMT)
tibility, vascular flow, and irregular mostly peripheral ring protein is important for genome stability. It repairs the
contrast enhancement. Perilesional hyperintense vasogenic naturally occurring promutagenic DNA lesion and pre-
edema is apparent on FLAIR/T2WI. GRE may show areas vents mismatch and errors during DNA replication and
of blooming due to hemorrhage or calcifications. Advanced transcription. MGMT promoter methylation presents in
MRI techniques in addition to conventional MRI provide up to 75% of GB results in loss of this protective mecha-
valuable information. MR perfusion imaging demonstrates nism and has been associated with better response to treat-
an increase in rCBV within the solid areas consistent with ment and longer overall survival in patients with gliomas
high-grade tumor. MR perfusion can help identify and treated with alkylating agents such as temozolomide.

Case
284 CLINICAL HISTORY 77-year-old male with a history of chronic
lymphocytic leukemia (CLL). He has new vision changes, increased
weakness, and confusion.
609

610 Case 284
Figure 284-5
FINDINGS Figures 284-1 and 284-2. Axial NCCT and post-

contrast CT, respectively, through the frontal horns. There
is an ovoid contrast-enhancing mildly hyperdense mass in Figure 284-6
the right frontal horn (transverse arrow) with adjacent right
frontal white matter (WM) hypodensity (vertical arrow) con-
sistent with edema. There is associated ependymal enhance-
ment. Figure 284-3. Axial FLAIR through the frontal horns. parenchymal tissue. Petechial hemorrhage is not uncommon
There is an ovoid irregular hypointense mass occupying the and may impact some heterogeneity to its signal pattern and
right frontal horn (transverse arrow) with surrounding paren- hypointensity on GRE. In the immunocompromised patient,
chymal hyperintensity reminiscent of vasogenic edema. there is gross hemorrhage and necrosis with or without ring
Figure 284-4. Axial T2WI through the frontal horns. The contrast enhancement, making it difficult to distinguish from
mass is relatively hypointense to gray matter (GM) (arrow). other entities such as glioblastoma (GB), metastasis, or infec-
There is bilateral peritrigonal hyperintensity. Figure 284-5. tion. Use of steroids could alter the imaging pattern reducing
Coronal post-contrast T1WI through the frontal horns. The the amount of edema and preventing contrast enhancement.
right frontal horn mass is avidly contrast enhancing (arrow) Lymphomas are also noted to shrink or resolve with steroid
and has a smooth margin. Non-contrast T1WI (not shown) therapy. NCCT shows mild hyperintensity of the lesion with
demonstrated an isointense mass. Figure 284-6. Axial GRE avid contrast enhancement in the immunocompetent.
through the lateral ventricles. There are multiple hypointense This patient had associated multiple leptomeningeal lym-
lesions within the right lateral ventricle (arrows). phomas. Orbital involvement is common in PCNSL. PCNSLs
are mostly parenchymal in location constituting between 1%
DIFFERENTIAL DIAGNOSIS Multifocal hemorrhage, meta and 6% of all CNS malignancies. Intraventricular PCNSL
stases, leukemic infiltrates, toxoplasmosis, primary CNS lym- forms about 11% of all PCNSL and is found in all ventricles
phoma (PCNSL). without associated increased incidence of hydrocephalus.
90% to 95% of PCNSL are DLBCLs as in this patient. CSF
DIAGNOSIS Intraventricular PCNSL, diffuse large B-cell showed atypical lymphocytes, and the diagnosis was made
lymphoma (DLBCL). by biopsy of the right frontal horn mass. PCNSL can occur
at any age but is generally common in the younger adult in
DISCUSSION MRI is the investigation of choice in the the immunocompromised and older adult in the immunocom-
evaluation of suspected PCNSL. PCNSL could be single or petent. Presentation typically includes mental status changes,
multiple. Imaging findings tend to vary with immune sta- focal neurologic deficit, and vision changes. Treatment
tus of the patient. In the immunocompetent patient, mass options include corticosteroids, radiation, and chemotherapy
particularly DLBCL is isointense/hypointense with GM on all with mixed result and significant recurrence of disease and
T1WI and T2WI with avid homogeneous contrast enhance- dismal prognosis. The 5-year survival rate is less than 10%.
ment. Lesions usually originate from the ventricular walls
projecting into the ventricles or from the choroid plexus. Question for Further Thought
They may restrict diffusion due to dense cellularity. They are 1. What other imaging techniques could be useful in the
commonly associated with edema of the adjacent cerebral evaluation of PCNSL?

Case 284 611

Presence of a new and unexpected lesion deserves direct 1. The relatively low rCBV in perfusion studies of PCNSL
reporting. Location and number of lesions if more than one distinguishes it from GB. MRS tends to show decreased
should be reported. Presence of hydrocephalus or ventricular N-acetyl aspartate (NAA) and creatine concentration with
obstruction deserves mention. elevated choline peak and associate lactate and lipid peaks
which may not be specific for PCNSL. FDG-PET tends to
What the Treating Physician Needs to Know show higher glucose avidity in PCNSL than in other intra-
Location of lesion or lesions. This will determine the cranial malignancies. 11-C Methionine PET also shows
trajectory of stereotactic biopsy very high uptake in PCNSL with area of uptake substan-
Presence of other associated lesions tially larger than tumor enhancement on CT and MR sug-
Is it safe to perform lumbar puncture (LP)? Yes. There is gesting a far larger tumor infiltration.
no reason to believe that this mass may result in herniation
or shift of anatomical landmark following LP

Case
285 CLINICAL HISTORY Incidental finding in a 63-year-old female..
FINDINGS Figure 285-1. Axial T2WI through the parietal well-delineated subcutaneous masses. It usually does not
bones. There is an ovoid hypointense posterior right parietal require any treatment unless it is infected, proliferating, or
scalp nodule (arrow). Figure 285-2. Right parasagittal T1WI complicated by carcinoma.
through the mass. The nodule is of intermediate intensity
almost isointense with brain. Question for Further Thought
1. Is there any risk for malignant degeneration of a trichil-
DIFFERENTIAL DIAGNOSISDermoid, epidermoid, emmal cyst?
sebaceous cyst, lipoma.
DIAGNOSIS Sebaceous (trichilemmal or pilar) cyst.
Routine reporting is sufficient for this benign usually inci-
DISCUSSION Trichilemmal or pilar cyst is the proper dental finding. Recognize the benign nature of the lesion, and
name for a lesion which more commonly goes by the label offer an appropriate differential. As there is imaging overlap
of sebaceous cyst. Technically, this is a misnomer, in that between trichilemmal cyst and other benign lesions of the
the lesion does not derive from the sebaceous glandular scalp, including lipoma, dermoid, and epidermoid, precise
apparatus, though cyst contents may be sebaceous in quality. diagnosis is usually not possible.
On CT, the density ranges from fatty to calcific, reflecting a
variety of internal contents from sebaceous fluid to calcifica- What the Treating Physician Needs to Know
tion. On T1WI, they are often intermediate in signal, while Trichilemmal cyst is a common incidental lesion of the
on T2WI, hypointensity is most common. If calcified, they scalp which, provided there are no unusual or aggressive
may bloom on gradient echo images. These lesions are not imaging findings, can be ignored or, if the patient prefers,
expected to enhance. They are usually small but could be as can be easily excised
large as 5 cm.
Trichilemmal cyst is a lesion of the dermis or subcuta- Answer
neous layer lined by squamous cells and containing kera- 1. The vast majority of trichilemmal cysts will never
tin. It occurs in about 5% to 10% of the population. Some undergo malignant transformation. At worst, they may
are hereditary in an autosomal dominant inheritance pat- grow slowly. Rarely, a component of proliferating cells
tern. Clinically, these are firm, nontender nodules in the can develop within a cyst giving rise to the so-called pro-
superficial subcutaneous space, most commonly occurring liferating trichilemmal tumor. These tumors, although
in the head, and specifically in areas with high density of still benign, may grow to a large size. On imaging, these
hair follicles. The typical patient is a middle-aged woman. lesions demonstrate a complex cystic morphology with
Histopathologically, it resembles the sheath of a hair fol- areas of nodular tissue and enhancement. Very rarely,
licle, from which it does in fact originate. These are frequent frank degeneration to a carcinoma can occur with local
incidental finding on imaging, where they present as small invasion and distant metastases.
612

Case
286 CLINICAL HISTORY 62-year-old male with history of stroke.
FINDINGS These images were obtained on the second ated. Its occlusion is probably responsible for the new
day of admission. Day 1 images (not shown) showed a left infarct on the right side. Bilateral anterior cerebral arteries
perisylvian area of restricted diffusion. Figures 286-1 and (ACAs) (transverse arrows) show robust intensity.
286-2. Axial DWI and corresponding ADC map through the
lateral ventricles. There is bilateral perisylvian restricted DIFFERENTIAL DIAGNOSIS N/A.
diffusion. The right basal ganglia is involved, while the left
posterior lentiform nucleus and the adjacent left corona DIAGNOSIS Bilateral MCA territory acute infarction.
radiata are involved as well. Inferior extension into the
anterior temporal lobes (not shown) was present bilaterally. DISCUSSION The classical MRI finding of acute/
Figures 286-3 and 286-4. 3D time of flight MRA of the subacute ischemic infarct is the restricted diffusion
head obtained on day 1 showing nonvisualization of bilat- hyperintense on DWI with low ADC. This becomes evident
eral internal carotid arteries and the left middle cerebral within the first 30 minutes before the FLAIR and T2WI
artery (MCA). The right MCA (vertical arrow) is attenu- changes are clearly visible. It may take up to 2 hours for
613

614 Case 286
FLAIR and T2WI changes to be seen. The initial mass Question for Further Thought
effect is local. Simultaneous acute bilateral MCA territorial 1. How does blood get to the ACAs despite complete occlu-
infarcts are unusual. CT may show hypodensity in the same sion of bilateral ICA?
locations. The MCA is the largest vascular territory in the
brain and the most common location for infarcts. Embolic Reporting Responsibilities
phenomenon tends to be common in the MCA territory Direct reporting is always necessary in acute infarcts.
since it receives a disproportionate share of the blood. Locations of infarcts, presence of mass effect, and hemor-
Atherothrombotic changes and in this situation Internal rhage should be mentioned. The vascular changes if MRA
carotid artery (ICA) occlusion are not uncommon. is obtained should be enumerated. MRA or CTA of the head
There is a necessity to evaluate the neck vasculature and neck should be recommended if not already done.
either by CTA or by contrast MRA in this patient in order
to prescribe appropriate management. Large MCA infarction What the Treating Physician Needs to Know
has been found to be associated with cardiogenic embolism, Size and location of infarcts
ICA occlusion, and ICA dissection. Subsequent CTA of the Complications such as mass effect, herniations, hydro-
neck revealed an occluded left ICA at origin with a (presum- cephalus, hemorrhage
ably recanalized) 95% stenosis of the right ICA origin. There Complete MRA or CTA of the head and neck is necessary
appear to be significant collaterals to the MCA territory since for evaluation of vascular structures
only the perisylvian territories are affected despite occlusion
of M1 on the left and significant disease on the right. Answer
MCA infarcts present with aphasia, hemiparesis, mental 1. A patent circle of Willis (COW) ensures adequate com-
status changes, and coma. The prognosis of a complete pensation for occlusions. The posterior communicating
MCA territory stroke is very poor. Life threatening MCA arteries are present, thus routing blood from the pos-
infarction, so called malignant MCA stroke, occurs in up to terior circulation to the ACA via the supraclinoid ICA.
10% of all stroke patients with the main cause of death being Anastomosis of the ECA and ICA via the ophthalmic
severe edema leading to raised intracranial pressure, clini- artery is another route of blood from the extracranial cir-
cal deterioration, coma, and death. Brain swelling with mass culation to the intracranial circulation. Other collateral
effect develops rapidly and appears to peak within 5days circulations include the posterior/anterior pericallosal
of the ictus. Uncal, subfalcine, and transtentorial hernia- arteries around the splenium of CC and leptomeningeal
tions and hemorrhagic transformations are not uncommon collaterals not necessarily visualized in this case. Contrast-
complications. Large MCA infarction is a major predictor of enhanced MRA or CTA may be useful in demonstrating
death and severe disability. these collaterals.

Case
287 CLINICAL HISTORY Adult patient being studied for acute headache
without any significant past medical history.
615

616 Case 287
DISCUSSION Idiopathic calcifications are considered

physiologic when no known underlying cause or disease is
present. These are usually punctate or granular hyperdensi-
ties located in the medial aspect of both globus pallidus, head
of the caudate nucleus, putamen, and dentate nuclei on CT.
Calcifications in the basal ganglia are very common in mid-
dle-aged and elderly individuals and tend to be bilateral but
may be asymmetric in appearance. Remember that calcium
may be hyperintense on MR T1WI due to presumed hydra-
tion of the mineral and could be hypointense on GRE and
T2WI. This condition is asymptomatic and therefore usually
represents an incidental finding on imaging studies, and its
incidence increases with age. Idiopathic calcifications can be
found in typical sites such as basal ganglia and cerebellum.
Basal ganglia calcification in children and young patients
cannot be considered idiopathic, and an underlying cause
must be excluded. These may include calcium phosphate
metabolism disturbances in parathyroid diseases such as
hypoparathyroidism, hyperparathyroidism, and pseudohypo-
parathyroidism. Additionally, after 10 months of age, children
who have HIV infection may develop basal ganglia calcifica-
tions. Congenital basal ganglia calcifications include familial
Figure 287-5 idiopathic syndrome such as Fahr syndrome and Cockayne
syndrome. Radiation to the brain and chemotherapy can
result in the long-term bilateral basal ganglia calcifications.
Some of these are demonstrated in these images.
FINDINGS Figure 287-1. Axial NCCT through the basal
ganglia. There are bilateral symmetrical calcifications in Questions for Further Thought
the basal ganglia, the lentiform nuclei and caudate heads 1. What is Fahr syndrome?
(transverse arrows). There is a small punctate calcification
in the white matter (WM) of the right frontal lobe (verti- Reporting Responsibilities
cal arrow). Figure 287-2. Corresponding non-contrast MR Routine reporting is sufficient as this is not a progressive or
T1WI through the basal ganglia. The calcifications are life-threatening disease. It is important to describe the dis-
slightly hyperintense (transverse arrows). Figure 287-3. tribution and extent of findings and identify associated signs
Axial NCCT in a patient with renal failure and second- to suspect underlying pathologic conditions. Look for calci-
ary parathyroid disease. There is calcifications in the fications elsewhere such as the parotid gland and skin which
basal ganglia and frontal WM (vertical arrows) and also point toward a systemic calcium metabolism abnormality.
superficially in scalp. Figure 287-4. In a patient with Fahr
disease this axial NCCT shows extensive calcifications What the Treating Physician Needs to Know
in the deep gray matter (GM), basal ganglia and thalami Other associated findings or calcifications elsewhere
(transverse arrows), WM, and occipital cortex (vertical Idiopathic calcifications have no clinical significance
arrows). Figure 287-5. Axial NCCT in an adolescent who Biochemical testing is necessary if calcification occurs in
was irradiated as a child for a medulloblastoma. There are young patients; the presence of basal ganglia calcifications
basal ganglia (arrows) and subcortical WM (frontal lobes) in patients <30 years of age should prompt clinical evalu-
calcifications. ation to rule out another etiology
Extensive calcifications and uncommon appearances or
distributions of calcifications
DIFFERENTIAL DIAGNOSIS Hypothyroidism, hypopara-
thyroidism, hyperparathyroidism, pseudohypoparathyroid- Answer
ism, postthyroidectomy, Fahr syndrome, carbon monoxide 1. Fahr syndrome, also known as bilateral striopallidoden-
or lead poisoning, radiation therapy, chemotherapy, ischemic tate calcinosis, is an inherited neurodegenerative disorder
injury. characterized by bilaterally symmetric deposition of cal-
cium in basal ganglia as well as in the cerebellum, cen-
DIAGNOSIS Idiopathic calcifications of the basal ganglia. trum semiovale, and subcortical WM.

Case
288 CLINICAL HISTORY 26-year-old female was involved in a motor vehicle
collision. She lost consciousness at the site of collision. She is not waking up.
FINDINGS Figure 288-1. Axial MRI GRE through the fourth products. Figure 288-2. Axial GRE through the trigones. There
ventricle. There are multifocal bilateral cerebellar, pons, and is hypointense sedimentation with fluid-fluid level in the right
left superior cerebellar peduncle punctate hypointensities trigone consistent with intraventricular hemorrhage (vertical
(arrows show some of them) consistent with hemorrhagic arrow). There is visible subcortical punctate h ypointensity in
617

618 Case 288
the left occipital lobe (transverse arrow). Figure 288-3. Axial Most patients with DAI are usually unconscious at the site
GRE through the corpus callosum (CC). There is heteroge- of injury. They are more likely to suffer posttraumatic coma,
neous enlargement of the posterior body and splenium of the fail to recover neurologically, and remain in vegetative
CC (vertical arrow). There are numerous bilateral frontal and state. Mild forms do exist. Young men are overrepresented.
parietal subcortical hypointensities (transverse arrows identify Unfortunately, there is no known treatment.
some of them). Figure288-4. Color directional MRI DTI map
through the CC. There is disorganization and asymmetry of Question for Further Thought
fiber tracts (transverse arrows). There is disruption, enlarge- 1. What is the pathogenesis of DAI?
ment, and heterogeneity of the usual red transverse fiber tracts
of the CC (vertical arrows). Reporting Responsibilities
This is an emergency requiring direct reporting. General
DIFFERENTIAL DIAGNOSIS N/A. location, size, and number of lesions should be noted. Other
associated primary injuries such as fractures, soft tissue inju-
DIAGNOSIS Traumatic brain injury with diffuse axonal ries, contusions, major hematomas, extraaxial hemorrhages,
injury (TBI/DAI). and hygromas should be reported. Presence of secondary
injuries or complications such as brain swelling, herniations,
DISCUSSION This is a grade III DAI involving the subcor-
intraventricular hemorrhage, infarcts, and hydrocephalus are
tical whitegray matter regions of the brain, the cerebellum,
categorized. Significant changes on follow-up should also be
the CC, and the brainstem. Grade I DAI usually involves the
directly communicated.
subcortical white matter of the brain mainly frontotemporal
lobes but elsewhere and the cerebellum. Grade II involves
the CC mostly splenium and posterior body in addition to
areas involved in grade I. Grade III encompasses grades I NCCT may be normal. Clinical suspicion should encour-
and II along with brainstem injury involving mostly fiber age the use of MRI
tracts, rostral brainstem, and superior cerebellar peduncles. Locations and number of visible lesions
Diagnosis of DAI could be difficult to make on CT since Other primary and secondary injuries
up to 80% of nonhemorrhagic DAI may have normal CT. Other imaging modalities that could help resolve incon-
Hemorrhagic lesions could be visible on CT depending on clusive findings
their size. A few hemorrhagic lesions in the appropriate loca- Significant changes on follow-up particularly new and
tions are usually enough to suspect that there are widespread secondary injuries
lesions. Presence of intraventricular hemorrhage may lead to
elevated intracranial pressure that is associated with signifi- Answer
cant mortality. 1. DAI is a common primary injury in patients with severe
DAI lesions are better categorized by MRI. MRI is the TBI. It is generally regarded as due to widespread shear-
modality of choice in suspected DAI. Unfortunately, it may ing injury during acceleration and rotation of the brain at
not be feasible to obtain MRI in the acute phase. Specifically, impact. DAI lesions depend on the magnitude and direc-
DWI, FLAIR, GRE, and SWI show both nonhemorrhagic tion of the shearing forces during impact and the rela-
and hemorrhagic DAI lesions. They restrict diffusion on tive rigidity and density of contiguous tissues. Lesions
DWI and are hyperintense on FLAIR and T2WI, while the are more commonly found in subcortical white matter
hemorrhagic lesions are hypointense on GRE and SWI. particularly of the centrum semiovale, the basal gan-
The age of the hemorrhagic lesions determines their inten- glia, CC, cerebellum, and brainstem. Lesions are usually
sity on FLAIR, T1WI, and T2WI. Associated brain swell- multifocal, hemorrhagic or nonhemorrhagic, and vary
ing could be diffuse or regional. DAI lesions are usually in size from punctate to over 1.5 cm. Initial CT imaging
widespreadvarying from small to large. DTI usually shows may not detect these lesions but as surrounding edema
decreased fractional anisotropy, with the color directional develops over the next few days they become visible and
maps showing disruption and disorganization of fiber tracts. their impact results in deterioration of clinical symptoms.
Brain perfusion studies may show patchy areas of hypoper- Hemorrhagic lesions may expand substantially. The natu-
fusion. Perfusion abnormalities tend to correlate with poor ral history is that of brain atrophy. The clinical outcome is
outcome in TBI. generally very poor.

Case
289 CLINICAL HISTORY 5-year-old female with headache for 1 year.
ranging from none to strong. CT findings mirror the MRI

findings of cystic/solid mass with mass effect and similar
pattern of contrast enhancement.
PA is the most common pediatric central nervous system
tumor. Among posterior fossa tumors, it is one of the most
common, with 60% arising from the cerebellum. Brainstem
lesions are least frequent. It is the most common tumor seen
in patients with neurofibromatosis type 1, who often have
lesions outside of the cerebellum. Ependymoma, medul-
loblastoma, and choroid plexus papilloma are major dif-
ferential diagnoses for posterior fossa mass, particularly
in pediatric patients. In patients with von Hippel-Lindau
disease, hemangioblastoma should be considered. PA is an
uncommon diagnosis for older patients. For these patients,
more likely differential diagnoses include hemangioblas-
toma, other gliomas, cystic metastasis, and infection such as
toxoplasmosis.
Figure 289-1 PA is classified as WHO I tumor. Because of their slower
growth rate, patients often present with prolonged symp-
toms that last for months to years. Most common symptoms
include headache, nausea and vomiting, ataxia, and cerebel-
FINDINGS Figure 289-1. Axial post-contrast T1WI through
lar signs. Although mass effect can be impressive, it is often
the posterior fossa. There is a large mass arising from the
well tolerated due to accommodation.
left cerebellum, compressing the brainstem and effacing the
fourth ventricle. The dominant features of the mass include
a large cyst (black arrow) and a mural nodule (white arrow),
which are classic imaging features of pilocytic astrocytoma 1. Does MR spectroscopy provide contributory diagnostic
(PA). Smaller cysts and septations with mural enhancement information for PA?
are also seen in the posterior aspect of the mass.
DIFFERENTIAL DIAGNOSISEpendymoma, medullo- Direct reporting is necessary. The fourth ventricular com-
blastoma, choroid plexus papilloma, hemangioblastoma, PA pression invariably results in hydrocephalus requiring imme-
cystic metastasis, and infection such as toxoplasmosis. diate attention. Differentiate PA from other posterior fossa
tumors. Accurately evaluate origin and degree of extension
DIAGNOSIS Pilocytic astrocytoma (PA). of tumor. Assess for complications such as hydrocephalus
and brainstem compression.
DISCUSSION This case demonstrates the most common
appearance of PA, a combination of solid and cystic com- What the Treating Physician Needs to Know
ponents with a characteristic pattern of nodular and mural Accurate differentiation of PA from more aggressive
contrast enhancement, particularly for those arising in the tumor such as medulloblastoma
posterior fossa. However, one-third of cases manifest as Presence of complications such as hydrocephalus, brain-
predominantly solid mass. Solid lesions are commonly seen stem compression, and cerebrospinal fluid (CSF) seeding
in supratentorial locations and may contain necrotic cen-
ter. Usually, there is very little or no surrounding edema. Answer
Calcifications may be present, particularly in predominantly 1. MR spectroscopy for PA is often misleading, showing
solid masses presenting a blooming appearance on GRE. features more typical of an aggressive tumor, including
Enhancement of the solid component of the cystic type is high choline peak, low N-acetyl aspartate (NAA), and
usually strong, while the solid type has variable enhancement high lactate peak.
619

Case
290 CLINICAL HISTORY 48-year-old female with chronic lymphocytic
leukemia and disseminated varicella-zoster virus infection presenting with
altered mental status.
Figure 290-4
FINDINGS Figures 290-1 and 290-2. Axial DWI through

the levels of the lateral ventricles and the vertex, respec-
tively. Multifocal bilateral areas of restricted diffusion in
multiple vascular territories, bilateral posterior cerebral artery
(PCA) (figure 1 arrows), right anterior cerebral artery (ACA)
(figure290-2 transverse arrows), and left watershed region
Figure 290-3
(figure 290-2 vertical arrow). Figure 290-3. Axial DWI follow-
up through the vertex. There is progression of bilateral cerebral
areas of restricted diffusion (arrows). Similar progression of
620

Case 290 621
Apart from meningitis and encephalitis, VZV also causes

Ramsay Hunt syndrome, Bells palsy, acute disseminated
encephalomyelitis (ADEM), and myelitis. In the adult
immunocompetent patients, VZVE presents as large vessel
vasculopathy (granulomatous arteritis) with large bland or
hemorrhagic infarctions, while in the immunocompromised
patients, small vessel vasculopathy results in small ovoid
GMWM junction mixed ischemic or demyelinative lesions.
Ventriculitis/periventriculitis is rare but makes up the third
pattern of encephalitis. Myelitis presentations include loss
of pain/temperature or position/vibration sense, Brown-
Squard syndrome, sphincter disturbances, and sensory
abnormalities.
VZV infection occurs in all ages and shows no gender
preference. Encephalitis tends to present more commonly in
children as acute cerebellar ataxia (ataxia, tremor, vomiting,
and headache 13 weeks after chickenpox). The typical zos-
ter dermatomal rash may not be present, but could precede
or occur after the encephalitis by a long period. Treatment is
usually with anti-viral.

1. What is the route of entry of the virus to the central ner-
Figure 290-5 vous system (CNS)?
T2 hyperintensities was present on FLAIR/T2WI (not shown) Presence of restricted diffusion indicates an acute event
in bilateral ACA and PCA territories. Figure 290-4. Right para- which could be ongoing and requires direct reporting. The
sagittal non-contrast T1WI on follow-up. There is gyriform multifocality of lesions with the appropriate history should
hyperintensity in the right frontoparietal lobes (arrows) consis- alert the radiologist to the diagnosis with the other differ-
tent with hemorrhagic infarcts. Figure 290-5. Axial post-con- entials mentioned. A mixed pattern of encephalitis could
trast T1WI through the level of the centrum semiovale. There is suggest the diagnosis. Cerebellitis in a child should raise
bilateral leptomeningeal enhancement (arrows) superimposed the suspicion of VZV infection. MRA or CTA may show
on the underlying gyriform hyperintensity of subacute hemor- occlusion of large vessels and should be recommended.
rhage in bilateral frontoparietal lobes. ICA occlusion occurs in herpes zoster ophthalmicus and
loss of signal void in the ICA may be a pointer to that
DIFFERENTIAL DIAGNOSIS Multifocal infarcts, embolic diagnosis.
infarcts, varicella-zoster virus encephalitis (VZVE).
DIAGNOSIS Varicella-zoster virus encephalitis (VZVE). Location of lesions for purposes of biopsy if it becomes
necessary
DISCUSSION VZVE presents as multifocal and mul- Presence of multifocal infarcts is not specific. Embolic
titerritorial areas of diffusion restriction on DWI with cor- phenomenon should be excluded
responding T2 hyperintensity consistent with infarctions. The history is key to the diagnosis. Unfortunately the typi-
Lesions are hypointense on T1WI. Hemorrhagic conversion cal rash could be absent, and that should not prevent suspi-
is common. When contrast enhancement occurs, it is patchy. cion of the diagnosis
The initial lesions in this patient did not contrast enhance Is there a mixed pattern of encephalitis? Small and large
and were not hemorrhagic. Follow-up study showed hemor- vessel patterns of infarcts and/or ventriculitis/periventricu-
rhagic conversion and leptomeningeal enhancement. White litis should also raise suspicion of VZVE
matter (WM) and gray matter (GM) may be affected show- Are there other imaging methods to confirm diagnosis?
ing typical patterns of T2 hyperintensity associated with CTA or MRA could be useful in defining the vasculopathy
infarcts. Cerebellitis with diffuse cerebellar T2 hyperinten-
sity is the commonest presentation in children. It may not be Answer
possible to distinguish embolic or multifocal infarcts from 1. The virus gains entry into the CNS via neurons. Virus
lesions of VZVE. The presence of recent vaccination or his- could remain dormant in the dorsal root ganglion for a
tory of chickenpox or the the presence of zoster rash and long time before subsequently invading the CNS through
prodromal symptoms help in this regard. the axons or the vasculature.

Case
291 CLINICAL HISTORY 60-year-old female with a history of rapidly
(about 3 months) progressive dementia.
FINDINGS Figure 291-1. Axial T2WI through the thal- forms are similar to sCJD MRI. The basal ganglia may also
ami. There is subtle hyperintensity in the basal ganglia and be affected and show similar findings to those seen in the
medial and posterior thalami bilaterally and symmetrically. thalami. All abnormalities in this disease may be more obvi-
Figure 291-2. The abnormalities are more obvious and bet- ous on DWI but remember that adequate narrowed windows
ter seen on the corresponding FLAIR image (arrows). may be needed which make the findings more obvious. The
pulvinar or hockey stick sign is very specific for the vCJD
DIFFERENTIAL DIAGNOSIS: IMAGING Postinfectious and differentiates it from the differentials. Hypoxic isch-
encephalitis, Creutzfeldt-Jacob disease (vCJD), cat-scratch emic encephalopathy (HIE) may be confused with the sCJD
disease, intracranial hypertension, and Alpers syndrome. imaging pattern.
CJD is caused by an infectious protein called prion and
DIAGNOSIS Variant form of Creutzfeldt-Jacob dise may be variant, sporadic, genetic, or iatrogenic. Clinically
ase (vCJD). it is characterized by rapidly progressive dementia, ataxia,
and myoclonus. The sporadic form is the most common and
DISCUSSION The characteristic finding on MRI of vCJD, usually occurs in patients above 65 years of age. It is char-
is the pulvinar sign, and is considered to be very specific, acterized by dementia, ataxia, and behavioral disturbance,
showing hyperintensity symmetrically in the pulvinar of and the EEG is very suggestive of the disease showing
the thalami on DWI, FLAIR, and T2WI. When the hyper- generalized periodic sharp wave complexes. The analysis
intensity extends to mediodorsal nucleus of the thalamus, it of cerebrospinal fluid (CSF) reveals the prion (14-3-3 pro-
gives the hockey stick appearance as in this case. In the tein), although only biopsy or autopsy provides definitive
sporadic form (sCJD), MRI most often shows involvement diagnosis.
of the striatum and the cortex, with hyperintensity in T2WI vCJD occurs at younger ages and is probably transmit-
and FLAIR, and restricted diffusion, which can be asym- ted by cattle infected with bovine spongiform encepha-
metric. Neuroimaging findings in the genetic and iatrogenic lopathy. This form manifests at early stages of disease with
622

Case 291 623
p sychiatric abnormalities and sensory symptoms, and only Once the diagnosis is suspected, necessary precaution
later progresses with dementia, ataxia, and other motor should be undertaken to prevent transmission of the
symptoms. disease
CSF evaluation is always necessary to confirm the
Questions for Further Thought diagnosis
1. What is the role of DWI in dementia studies?
2. Which neuropathologic findings are responsible for the Answers
signal changes? 1. DWI is more sensitive than T2WI to depict the lesions
mainly at the early stages of the disease, for detection of
cortical lesions, and it minimizes motion artifacts which is
very common in demented patients possibly due to myoc-
Direct reporting is necessary whenever CJD is suspected so
lonus. DWI also allows determining disease progression
that necessary precautions could be taken to prevent trans-
with serial imaging.
mission of this deadly disease. Always include DWI in stud-
2. Neuropathologic studies reveal vacuolation of gray mat-
ies of demented patients and do a careful analysis and report
ter (GM) (spongiform changes), astrocytic gliosis, and
of the findings.
nerve cell loss. When the pulvinar is affected changes are
attributed to gliosis and less likely to spongiform changes.
What the Treating Physician Needs to Know There is a concordance between neuroimaging and neuro-
DWI abnormalities at an early phase of disease pathology revealing deposition of prion in the cerebellum,
The history usually excludes HIE in view of basal ganglia striatum, thalamus, and cortex corresponding to the areas
involvement of signal abnormality.

Case
292 CLINICAL HISTORY 57-year-old male with headache and declining
executive functions.
Figure 292-4
Figure 292-3
624

Case 292 625
tumor growth. The bone may also become demineralized

and undergo permeative lysis, or as in our case, when
the lesion is based along a paranasal sinus, the bone may
blister toward the tumor (pneumosinus dilatans). On
T1WI and T2WI, similar to CT, the tumor is nearly isoin-
tense to GM and may have very low signal on all pulse
sequences if it is significantly calcified. With intravenous
contrast, the tumor most often shows early, avid, homoge-
neous enhancement, and the majority will exhibit a dural
tailmost readily identified on MR. Meningioma has a
propensity for growing along the anterior and central skull
base, occasionally encasing regional arteries and extending
into the adjacent foraminae, fossae, and nerve canals (e.g.,
orbital fissures, optic canal, sella turcica, foramen ovale). It
may exhibit extracranial extension into the olfactory recess
of the nasal fossa, orbital apex, or infratemporal fossa.
Malignant meningiomas cannot be reliably differentiated
from the more common benign tumors on a single exami-
nation. It has been reported that intratumoral cystic change
and extension through skull base foraminae may correlate
with tumor aggressivity. Prominent adjacent brain edema
correlates with higher operative morbidity and recurrence
rates. When considering an imaging diagnosis of menin-
Figure 292-5 gioma, it is imperative to classify the tumor as extraaxial.
Assigning an anatomic compartment is the most important
FINDINGS Figures 292-1 and 292-2. Contiguous NCCT initial discriminator in evaluating any intracranial mass.
through inferior frontal lobes. There is bilateral frontal lobe This process is often most easily accomplished by identify-
edema (arrows in Figure 292-1) and mild mass effect (par- ing a cleft of CSF between the tumor and brain parenchyma
tial regional sulcal effacement) secondary to an isodense as demonstrated in Figure 292-5. Prescribing thin slice T2
midline mass along the anterior skull base (arrowheads in or cisternographic imaging perpendicular to the tumor
Figure 292-2). Figures 292-3 and 292-4. Axial and coro- brain interface can facilitate this.
nal post-contrast T1WI demonstrates homogeneous avid Meningiomas are common extraaxial neoplasms that
enhancement of the dural-based, extraaxial mass (asterisk). are usually slow growing and benign. They are thought to
This tumor covers the skull base from the posterior margin of arise from arachnoid cap cells, which are specialized cells
the olfactory groove through the planum sphenoidale, nearly of the arachnoid granulations. Cushing first used the term
reaching the tuberculum sella. Note the upward doming, or meningioma in 1922 to clarify and unify the confusing,
blistering of the sphenoid sinus roof toward the tumor on descriptive terminology applied to these tumors by various
the sagittal image, a feature known as pneumosinus dilatans preceding authors. They account for approximately 30% of
(arrowheads in Figure 292-4). The cribriform plate was not intracranial neoplasms, are more common in middle-aged
transgressed by the tumor in this case. Figure 292-5. Axial and elderly patients, and show female predominance. As in
T2WI in a comparison case of a right frontal parafalcine our case, olfactory groove meningiomas may cause anosmia
meningioma. The mass is isointense with gray matter (GM). (unilateral or bilateral) in addition to personality changes
There is a cerebrospinal fluid (CSF) cleft between the mass through mass effect on the olfactory bulb/tract and frontal
and the brain parenchyma (arrow heads) defining the extra- lobes, respectively.
axial location of the mass.
DIFFERENTIAL DIAGNOSISDural metastasis, heman-
1. How often are meningiomas multiple? What are the pro-
giopericytoma, meningioma.
posed explanations?
DIAGNOSIS Meningioma.
DISCUSSION General imaging features on CT most In addition to tumor location and size, the presence of related
commonly include tumor density equal to or greater than brain edema and mass effect should be clearly stated and if
that of GM and may show any amount and quality of pronounced, urgently directly communicated. If surgery is
intratumoral calcification. The adjacent bone can respond deemed likely, the referring clinician may then expeditiously
by becoming hyperostotic and may indicate intraosseous initiate preoperative steroid.

626 Case 292
What the Treating Physician Needs to Know Presence and extent of tumoral calcification
Features noted above and others relevant to surgery Involvement of skull base foraminae/canals and any extra-
Associated bony changes and likelihood of intraosseous cranial extension
growth
Relationship to regional vasculature (arterial encase- Answer
ment, dural venous sinus invasion) with recommendations 1. Meningiomas may be multiple in up to 10% of spo-
for dedicated vascular imaging (CTA, MRA, MRV) as radic cases and are thought to arise clonally from the
needed. When possible, the radiologist should comment first tumor through CSF spread. Multiple meningiomas
on arterial supply to the tumor and the location of a vas- are the rule in neurofibromatosis type 2 (NF2). Rarely,
cular pedicle. Often, significant blood supply to the tumor genetic susceptibility may account for meningioma
arises from the external carotid circulation. In these cases, multiplicity via sporadic mutations in the NF2 gene or
the patient may benefit from preoperative angiographic other loci.
mapping and embolization

Case
293 CLINICAL HISTORY 57-year-old male with known aneurysm for f ollow-up
MRA.
FINDINGS Figure 293-1. Axial source image from 3D TOF DIFFERENTIAL DIAGNOSIS N/A.
MRA of the head through the third ventricle. There is a single
anterior cerebral artery (ACA) within the anterior interhemi- DIAGNOSIS Azygos anterior cerebral artery (AACA).
spheric fissure (arrow). Figure 293-2. Lateral MIP 3D MRA
of the brain. There is a single ACA A2 (arrow). Figure 293-3. DISCUSSION The single A2 otherwise known as azygos
Submentovertical 3D TOF MRA of the brain. There are two anterior cerebral artery (AACA) is a very rare congenital
A1s joining together to form a single A2 (transverse arrows). variant. There is fusion of the two A1. There is no anterior
There is no anterior communicating artery. The left A1 is mildly communicating artery (A-com). The single vessel then sup-
hypoplastic (vertical arrow). Figure 293-4. Axial non-contrast plies the bilateral parasagittal frontal lobes. AACA is found
T1WI. Asymmetric frontal medial sulcation abnormality; right in most midline congenital malformations such as agenesis
parasagittal microgyria (arrow). There is mildly malformed of the corpus callosum, lobar holoprosencephaly, syntelen-
thickening of the genu of corpus callosum (vertical arrow). cephaly, and septo-optic dysplasia. There are subtle midline
627

628 Case 293
malformations in this case. AACA has also been reported in What the Treating Physician Needs to Know
association with arteriovenous malformation (AVM) and sac- This could be an incidental finding with very minimal
cular aneurysm. Aneurysm at the bifurcation of the AACA associated lesions elsewhere
is supposedly common occurring in up to 71% most prob- CTA or MRA is equally effective in diagnosing AACA
ably due to hemodynamic stress or due to congenital anom- Presence of unexplained bifrontal infarcts may suggest
alous histology of the artery. The aneurysm in this patient presence of occluded AACA
(not well shown) is on the right supraclinoid ICA. The best
imaging technique for demonstrating AACA is either MRA
or CTA. DSA demonstration could be very difficult requir- Answer
ing cross-compression of one ICA during contralateral injec- 1. AACA is fairly common in fetuses occurring in 68% of
tion. The possibility of bilateral frontal lobe infarction should one series of fetal brains examined and 75% of another
the AACA be occluded is real. Clipping or embolization of series of infant brains examined. The incidence in the
AACA aneurysm could also be challenging. adult population, however, varies from about 0% to 5%.
It does appear that in the developmental process, the sin-
Question for Further Thought gle ACA duplicate and the A-com forms. It is not certain
1. What is the developmental origin of AACA? at which time this single ACA forms. It has been sug-
gested that this takes place at the 16-mm stage from the
Reporting Responsibilities medial branch of the olfactory artery or a continuation of
Routine reporting is sufficient. Presence of aneurysm or the median artery at the 20- to 24-mm stage. AACA is
vascular malformation, however, demands direct reporting. common adult mammals (pigs, squirrels, and rabbits) and
Presence of other congenital lesions should be documented. lower primates (chimpanzees and monkeys).

Case
294 CLINICAL HISTORY 34-year-old male with growing head mass over
2years.
629

630 Case 294
FINDINGS Figure 294-1. Axial NCCT bone window and extracranial projections. It may have the characteristic
through the cranial vault superiorly. There is a rather irregu- sunburst appearance and show patchy contrast enhance-
lar knobbly 16.4 cm 8.1 cm right hemicranial bony mass ment. MRI usually shows a destructive mass of heteroge-
involving the right frontotemporoparietal bones with patchy neous signal intensity on all sequences with patchy contrast
lytic and sclerotic areas projecting both intracranially and enhancement. Depending on its location, it may invade
extracranially (arrows). Figure 294-2. Axial T2WI through vascular structures. The MRA, CTA, or DSA may demon-
the body of the lateral ventricles. The mass is heteroge- strate recruitment of vascular supply from the extracranial
neous and of mixed intensity (star). There is a large com- circulation as in this case. Invasion of the venous sinuses is
ponent projecting intracranial compressing the right frontal demonstrated by MRV. Secondary osteosarcoma may dis-
lobe, right lateral ventricle and displacing midline structures tort the pattern of its primary host. It is a rather malignant
(falx) to the left. There is a right frontal white matter (WM) lesion that may metastasize. Indeed that was the case in this
hyperintensity adjacent to the intracranially projecting mass patient. The differential diagnostic considerations are also
consistent with vasogenic edema (arrow). Figure 294-3.
some of the primary hosts and may be difficult to differ-
Coronal post-contrast T1WI through the sella level. There entiate them from osteosarcoma. Aggressive behavior in an
is heterogeneous contrast enhancement of the huge mass. otherwise benign lesion should be suspicious for malignant
There is compression of the right hemisphere and right lat- transformation.
eral ventricle with midline shift. The dura has been destroyed Cranial osteosarcoma is rare but more common in the
for most part with dural enhancement in the region of the vault than the skull base. It presents in the second and third
superior sagittal sinus (SSS) which is not visualized suggest- decades of life with almost equal malefemale ratio. Clinical
ing invasion or occlusion (arrow). There is hypointensity presentation is that of a painless growing mass. Intracranial
of the right hemisphere laterally (star) corresponding to the invasion may result in neurologic deficit and is generally a
edema seen on the T2WI. Figure 294-4. 3D volume render- sign of poor prognosis. Because of the few cases that have
ing of the DWI with the vertex removed. The mass actually been reported, it has been difficult to establish a treatment
compresses and displaces the brain without brain surface paradigm for cranial osteosarcoma. Surgical removal is the
infiltration in the areas exposed (star). Figure294-5. MIP 3D primary treatment of choice. It is not known to be radiosen-
TOF MRA of the brain. There is hypertrophy of the bilat- sitive. Chemotherapy has been shown to improve survival.
eral external carotid artery branches (arrows) particularly
the superficial temporal, middle meningeal, and the occipital Question for Further Thought
arteriesall supplying the mass. Figure 294-6. 2D MRV of 1. In what way can DSA be useful in the treatment of cranial
the head. There is nonvisualization of the SSS and the trans- vault osteosarcoma?
verse sinuses. There is a maze of cortical and scalp venous
collaterals (arrows point to some of them). Reporting Responsibilities
Direct reporting is essential. There is edema of the brain and
DIFFERENTIAL DIAGNOSISPaget, fibrous dysplasia, the SSS is occluded. MRA and MRV are needed to evaluate
osteosarcoma, intraosseous meningioma, hemangioma. the SSS and should be recommended if not requested.
DIAGNOSIS Cranial vault osteosarcoma. What the Treating Physician Needs to Know
Location and size of tumor
DISCUSSION Osteosarcoma of the cranial vault is very Effect on surrounding structures particularly the brain and
rare. Osteosarcoma is predominantly a disease of the appen- the vascular structures
dicular skeleton and mostly occur as a primary tumor in chil-
dren. It is uncommon in adults. The adult form of the disease Answer
is mostly secondary within benign lesions such as Paget, 1. DSA could be useful in confirming the MRA findings and
fibrous dysplasia, chronic osteomyelitis and as a complica- precisely mapping out the vascular components of the tumor
tion of radiation treatment. The lesion in our patient is a pri- both arterial and venous. Recruitment of vessels from intra-
mary lesion without underlying benign process. CT shows cranial circulation may lead to modification of treatment
mostly mixed sclerotic and lytic lesion that is expansile and plan. DSA may also offer embolization for devascularizing
destructive at the same time. There is usually intracranial the tumor before surgery to prevent blood loss.

Case
295 CLINICAL HISTORY Adult with headache and left-sided CN XI palsy.
FINDINGS Figure 295-1. Axial NCCT at the level of fora- of thespinal canal (arrows) eroding the dens (arrowhead).
men magnum. There is a large, midline, rounded region Figure 295-5. The corresponding axial T1WI. The mass (arrow)
of geographic bony remodeling in the basion/clivus with a is isointense to surrounding muscle. Figure 295-6. Sagittal
narrow, nonsclerotic zone of transition (arrows). Note the post-contrast T1WI. The mass enhances heterogeneously pri-
absence of calcification in the lesion. Figures 295-2 and marily along the ventral margin of the tumor (arrow).
295-3. Axial, small FOV T2WI and sagittal T2WI MRI at
the same level, respectively. There is a corresponding mass DIFFERENTIAL DIAGNOSIS Metastasis, chordoma, chon
of mixed/heterogeneous intensity (arrows) anterior to brain- drosarcoma, plasmacytoma, extralymphatic/high-grade non-
stem and spinal cord. Figure 295-4. A slightly more inferior Hodgkin lymphoma, meningioma.
axial T2WI (at the level of the anterior C1C2 joint). There
is tumor extension into the ventral and left lateral aspects DIAGNOSIS Chordoma of the clivus.
631

632 Case 295
DISCUSSION Chordomas demonstrate variable and Meningiomas typically show much more intense contrast
often heterogeneous intensity characteristics on MRI. enhancement but may also appear heterogeneous if calcified.
Calcium and hemorrhage complicate the appearance, but Meningioma may invade the skull base, and the bony inva-
they frequently have cystic components and are generally sion is typically sclerotic. The imaging appearance of high-
hyperintense on T2WI. Enhancement is characteristically grade/extralymphatic non-Hodgkin lymphoma also overlaps
heterogeneous, as in our case. Preoperative MRI delineates with chordoma as it may appear cystic/necrotic but often
the tumor and its relationship to critical structures including invades bone with an aggressive lytic and destructive pattern.
the cavernous sinus, carotid and basilar arteries, and skull Chordomas are relatively slow-growing malignant neo-
base foraminae. As in our case, CT typically illustrates a plasms that arise from the remnants of the notochord.
midline, destructive clival mass with a narrow, nonscle- Approximately one-third of all chordomas arise in the cen-
rotic zone of transition. They often remodel the clivus as tral skull base, half arise in the sacrococcygeal vertebrae,
opposed to the fragmented bony destruction typically seen and the remainder originate in the intervening spine. The
with chondrosarcoma or metastatic disease. Intratumoral notochord takes an undulating course through the central
calcium is described as commonly representing residual skull base, and its cranial terminus is just below dorsum
native bone fragments. While they lack typical chondroid sella. These tumors may therefore arise along almost any
matrix features, these calcifications may confuse the diag- point in the basisphenoid or basiocciput as well as within the
nosis, suggesting a chondrosarcomaespecially if the adjacent nasopharynx, intracranial compartment, or upper
tumor is growing off midline (along petrooccipital synchon- cervical spinal canal. Classic chordomas have a very char-
drosis). Large intracranial soft tissue components are not acteristic histologic appearance, containing physaliphorous
unusual in chordoma and may exert significant mass effect cells that appear bubbly due to large intracellular mucin-
on the brainstem. In our case, the tumor fills the left lateral containing vacuoles. A chondroid variant of the chordoma
aspect of the spinal canal, likely accounting for the left CN has received much attention in the past, but it only mimics
XI palsy. Recall that the spinal accessory (motor) contribu- the appearance of chondroid tissue on routine histologic
tion to the CN XI ascends in the lateral cervical subarach- stains. It does not actually derive from or contain cartilage
noid space and passes upward through foramen magnum to elements. It is interesting to note that tumoral notochordal
descend laterally through the skull base via the jugular fora- tissue remnants assume a spectrum of activityfrom the
men (pars vascularis). benign ecchordosis physaliphora (usually a small, nonen-
The differential diagnosis includes plasmacytoma, which hancing intradural nodule that approximates cerebrospinal
shares many imaging features with chordoma, especially fluid [CSF] intensity/density along the posterior clivus/pre-
when arising in the midline basicranium. Sharply margin- pontine cistern) to the very aggressive dedifferentiated and
ated, scalloped margins indicating remodeling along regions undifferentiated forms of chordoma.
of bone lysis/loss may also be seen in plasmacytoma.
Plasmacytoma is less hyperintense than chordoma on T2WI Question for Further Thought
but more likely to have homogeneous contrast enhancement. 1. What are the treatment options and prognosis?

Case 295 633
Reporting Responsibilities Mass effect on/relationship to critical structures (cavern-

Direct reporting is desirable as in all tumors particularly if ous sinus, vessels, brainstem and spinal cord, skull base
critical structures are involved. Recommend CT and MRI foraminae)
as complementary modalities. MRI is needed for detailed Presence of metastasis/CSF spread (rare)
tumor mapping and biopsy planning. CT improves detection
of thin/cortical bony structural involvement and may help Answer
to detect and characterize intralesional calcium (chondroid 1. Combination therapy is the mainstay with surgical deb-
matrix). Mass effect and relation to the brainstem, sella, and ulking and standard or proton beam radiosurgery, as these
cord must be described with any related sequelae, that is, tumors are insensitive to conventional external beam radi-
central nervous system (CNS) edema, hydrocephalus. ation therapy. Local recurrence is common and may be
seen along the surgical tract. Prognosis is generally worse
What the Treating Physician Needs to Know in women, adults, and those with larger necrotic tumors.
Anatomic extent of disease for tumor mapping and biopsy A 5-year control rate of up to 70% can be achieved in a
planning majority of patients.

Case
296 CLINICAL HISTORY 16-year-old female with fainting spells and loss of
consciousness.
FINDINGS Figure 296-1. Axial T1WI through the temporal nodules isointense with cortical GM projecting into the
horns. There is a subependymal nodule isointense with corti- lateral ventricles (arrows). Figures 296-3 and 296-4. Axial
cal gray matter (GM) projecting into the right temporal horn FLAIR through the temporal horns and body of the lateral
(vertical arrow). Similar but less defined nodules surround ventricles, respectively. There are multiple symmetrical
the left temporal horn (transverse arrow). Figure296-2. isointense (to GM) contiguous subependymal nodules pro-
Axial T1WI through the body of the lateral ventricles. There jecting into the temporal horns and body of the lateral ven-
are multiple bilateral symmetrical contiguous subependymal tricles (arrows).
634

Case 296 635
DIFFERENTIAL DIAGNOSIS Heterotopic GM, tuberous cases are characterized by a female predominance, associated
sclerosis complex, toxoplasmosis, subependymoma. with the FLIN1 gene, positive family history for epilepsy,
and familial occurrence, while the unilateral ones are mostly
DIAGNOSIS Periventricular nodular GM heterotopia (PNH). sporadic. Women with subependymal heterotopia typically
present with partial epilepsy during the second decade of life
DISCUSSION Both CT and MRI are capable of dem- with physical and neurologic development up to that point
onstrating the changes of PNH but MRI best depicts the being typically normal. Men with subependymal heterotopia
classical findings. Classically, these are round or ovoid vary in their clinical presentation depending on whether they
subependymal nodules that follow GM density on CT and have the X-linked or autosomal form. Men with the X-linked
GM intensity on all MRI sequences. These nodules could form more commonly have associated central nervous system
be single or multiple, matted or confluent, of varying sizes (CNS) and visceral anomalies with typical abnormal develop-
and usually project into the ventricles. They are more comment and suffer significant perinatal mortality. Symptomatic
mon around the trigones but are found all around the lateral men with the autosomal variety have clinical courses similar
ventricles. PNH does not contrast enhance. This case rep- to symptomatic women.
resents the bilateral and symmetrical PNH, in which mul-
tiple and contiguous nodules symmetrically line the entire Question for Further Thought
ventricular walls. PNH is usually associated with other mal- 1. Is there a role for MR spectroscopy in the diagnosis of PNH?
formations such as Chiari II malformation, Dandy-Walker
malformation, agenesis of the corpus callosum, pachygyria,
schizencephaly, polymicrogyria, and cerebellar dysplasia. It
Routine reporting is sufficient. Location and type of the
is always important to look out for these other associated
heterotopia and other associated congenital abnormalities
anomalies. Differential diagnosis may include tuberous
should be itemized and reported.
sclerosis complex (TSC) and toxoplasmosis except that PNH
does not calcify. Subependymoma does not usually contrast
enhance like PNH. A small subependymoma could therefore
mimic PNH. The histology could mimic gangliocytoma. Location of the heterotopia and other associated congenital
PNH is the most common migrational disorder due to abnormalities
failed migration of proliferated neuronal and glial tissues Location may not always correspond to seizure focus at
from the germinal matrix around the ventricles resulting in electroencephalography (EEG) or magnetoencephalogra-
abnormal locations of disorganized neuronal tissue in the phy (MEG)
subependymal region. These tissues are devoid of the final
organization exhibited by the cortical neurons. Five types of Answer
PNH have been identified. These are (1) bilateral and sym- 1. Usually the MR diagnosis of PNH is not in doubt. MR
metrical, (2) bilateral single-noduled, (3) bilateral and asym- spectroscopy demonstrates no difference in the spectro-
metrical, (4) unilateral, and (5) unilateral with extension to scopic pattern of heterotopic GM compared with normal
the cortex. It is likely that genetic factors play a major role in GM. It does not seem to contribute to the diagnosis except
the bilaterally affected patients. The bilateral and symmetrical that it demonstrates normal GM pattern.

Case
297 CLINICAL HISTORY 20-year-old male, currently asymptomatic. History
of incidental white matter (WM) hypodensities on head CT performed after
traumatic loss of consciousness.
FINDINGS Figures 297-1 and 297-2. Axial FLAIR through T2-weighted hyperintensities throughout the WM. Based on
the temporal lobes and the corona radiata, respectively. the acuity of the arterial infarct diffusion restriction may coex-
There are multiple hyperintense foci throughout the subcor- ist. Recent studies indicate high frequency of microhemor-
tical, deep, and periventricular WM of the temporal lobes rhages in CADASIL. As with other WM diseases, total lesion
in Figure 297-1 and bilateral corona radiata in Figure 297-2 volume load calculated on T1WI images correlates signifi-
(arrows). The lesions did not enhance nor restrict diffusion cantly with degree of disability and neuropsychologic impair-
(images are not provided). ment. CT findings are nonspecific with hypodense subcortical
and basal ganglia lesions. These locations should address the
DIFFERENTIAL DIAGNOSISMultiple sclerosis (MS), possibility of CADASIL in a young patient. FDG-PET and
sporadic subcortical arteriosclerotic encephalopathy (sSAE), SPECT are sensitive for early diagnosis. PET shows regional
primary angiitis of central nervous system (CNS), posterior glucose hypometabolism. Reduced cerebral blood flow (CBF)
reversible encephalopathy syndrome (PRES), mitochondrial and later cerebral blood volume (CBV) without mean transit
encephalopathy with lactic acidosis and strokes (MELAS), time (MTT) alteration corresponding to WM signal abnormal-
cerebral autosomal dominant arteriopathy with subcortical ity sites are the characteristic findings on SPECT. DSA is nor-
infarcts and leukoencephalopathy (CADASIL). mal due to preserved endothelium. Uncommon but treatable
etiologies of infarcts such as sSAE and CNS angiitis should
DIAGNOSIS CADASIL. be carefully evaluated and excluded.
CADASIL is a hereditary, autosomal dominant arteri-
DISCUSSION MRI often show parenchymal changes opathy characterized by multiple WM infarcts. It is a non-
in CADASIL before symptom onset as it was in the above arteriosclerotic, amyloid-negative, small vessel arteriopathy
case. Anterior temporal lobe and superior frontal lobe sub- affecting the penetrating cerebral and leptomeningeal arter-
cortical WM involvement is almost pathognomonic. Cerebral ies. Multiple organs can be affected such as the skin, muscle,
cortex is spared; basal ganglia and brainstem involvement is liver, spleen, and heart. Diagnosis is genetic based on Notch3
not uncommon. The typical MRI changes are large confluent gene mutations on chromosome 19q12. Pathologic ultimate
636

Case 297 637
outcome is smooth muscle cell degeneration of the vessel abnormalities or strokes with negative CT or MR angio-
wall and extracellular granular osmiophilic deposits thicken- grams. Clinical investigation should exclude hypercoagula-
ing the basal membrane. blity with antiphospholipid syndrome, natural anticoagulant
The clinical presentation includes ischemic stroke, protein defects (protein C, protein S, antithrombin), factor V
migraine with aura, subcortical dementia/cognitive impair- Leiden mutation, and hyperhomocysteinemia.
ment, apathy, and mood disorders. The typical presentation
is first episode of transient ischemic stroke at third to fourth What the Treating Physician Needs to Know
decade. The architectural changes of the brain parenchyma Imaging could point in the direction of CADASIL, but
occur 10 to 15 years before the onset of clinical symptoms. ultimate diagnosis is genetic
Early in the disease course there is almost perfect recov- Angiograms are usually negative in CADASIL
ery after each infarct; but with cumulative effect, complete
recovery is less in long term. There is no known treatment. Answer
1. MS clinical presentation is quite different from CADASIL.
Question for Further Thought Subcortical U fiber involvement is one of the diagnostic
1. What are the imaging pearls for differential diagnosis? criterion of CADASIL. sSAE is associated with hyper-
tension. WM lesions usually do not involve subcortical
Reporting Responsibilities U fibers. MELAS, normalized ADC within 48 hours of
Routine reporting is sufficient. WM abnormalities in young onset favors MELAS; waning and waxing stroke-like cor-
adults are extremely uncommon. When a young adult with- tical lesions (parieto-occipital most common) with basal
out any vascular risk factors presents with acute encepha- ganglia calcification. In primary angiitis of CNS the MRA
lopathy, CADASIL should be addressed whenever there is or CTA will be abnormal. PRES always has a prominent
superior frontal and anterior temporal lobe subcortical WM inciting event.

Case
298 CLINICAL HISTORY 48-year-old female with a history of migraine
headaches now develops intense new onset headache with photophobia
andphonophobia.
FINDINGS Figures 298-1 and 298-2. Axial FLAIR through the sylvian fissures in the same patient. There is extensive
the midbrain and the centrum semiovale. There is wide- mostly linear subarachnoid space enhancement (arrows)
spread sulcal hyperintensity (arrows). Figures 298-3 and around the midbrain in Figure 298-3 and in the sylvian fis-
298-4. Axial post-contrast T1WI through the midbrain and sures and convexity sulci in Figure 298-4.
638

Case 298 639
DIFFERENTIAL DIAGNOSISLeptomeningeal enhance- o xygen and propofol administration. There was no fever or
ment (LME) due to meningitis, vasculitis, carcinomatosis, other constitutional symptoms in this patient. Lumbar punc-
lymphoma. ture opening pressure of 24 cm of water was the only abnor-
mal finding with otherwise normal CSF. The assumption was
DIAGNOSIS LME due to migraine headache. that this was another manifestation of the severe migraine
in this patient. The patient responded to aggressive manage-
DISCUSSION LME involves the pia mater covering the ment of her headache.
brain and extends into the subarachnoid space presenting as
hyperintensity covering the brain surface and filling in the Question for Further Thought
sulci on post-contrast T1WI. LME could be linear as in this 1. What is the pathogenesis of LME?
case which could be associated with infection or inflamma-
tion of the pia-arachnoid. A thicker, more nodular enhance- Reporting Responsibilities
ment may be associated with fungal, granulomatous, or LME requires direct reporting because of the possibility of
carcinomatous (metastases from breast, lymphoma, or from meningitis or subarachnoid space inflammation or neoplasm.
primary intracranial tumors such as medulloblastoma and The pattern of enhancement and other underlying brain
ependymomas) meningitis. It may also be associated with pathology such as abscess, hydrocephalus, ventriculitis,
vasculitis. Involvement of cranial nerves could be seen in tumor, or granuloma should be reported.
lymphoma, sarcoidosis, and Lyme disease. Specific focal
lumpy or mass-like enhancement in the subarachnoid space What the Treating Physician Needs to Know
may be present in neoplastic lesions of the cranial nerves Pattern and extent of enhancement
such as schwannomas and astrocytomas. The changes are Presence of other pathology that may help explain the
hyperdense on post-contrast CT. Hydrocephalus may be a findings
complication due to inflammatory changes blocking the Presence of complications such as hydrocephalus or
arachnoid granulations or obstruction to cerebrospinal fluid infarcts
(CSF) pathways.
LME is thought to be due to breakdown of the bloodbrain Answer
barrier that allows leakage of contrast into the subarachnoid 1. In bacterial meningitis, it is thought that the glycoproteins
spaces from inflamed pial vessels. The hyperintensity on the released by the bacteria increase the permeability of the
FLAIR is thought to reflect increased CSF protein or cellular vessels allowing contrast leakage into the subarachnoid
debris. The differential of the FLAIR hyperintensity should space. In cases of carcinomatosis, the implanted tumor
always include subarachnoid hemorrhage, leptomeningeal cells lead to bloodbrain barrier breakdown and leakage
collaterals, and in the appropriate setting supplemental of contrast into the subarachnoid space.

Case
299 CLINICAL HISTORY 20-year-old female with severe headache and loss of
consciousness.
Figure 299-4
Figure 299-3
640

Case 299 641
FINDINGS Figure 299-1. Axial NCCT through the thal- in the hematoma. The classical spot sign is a 1- to 2-mm
ami. There is a large left thalamic well-defined homogeneous hyperdense focus on CTA source images usually located
hyperdensity consistent with acute hematoma with surround- peripherally within the hematoma. The spot sign has been
ing hypodense halo (black arrow)edema. There is similar associated with hematoma expansion which is very predic-
hyperdensity within the dilated bilateral frontal horns of the tive of neurologic deterioration in primary ICH. Hematoma
lateral ventricles (line arrows) consistent with intraventricular expansion is an independent predictor of mortality and mor-
hemorrhage. Bilateral trigones are dilated (stars) consistent bidity. About 30% of PICH undergo expansion. Enlargement
with hydrocephalus. There is effacement of all convexity of the spot sign on post-contrast CT following a CTA has
subarachnoid spaces due to increased intracranial pressure. been suggested as representing active extravasation which
Figure 299-2. Axial head CTA source image through the is also predictive of hematoma expansion. It is interesting
lateral ventricles. There is irregular contrast enhancement that the spot sign in this hematoma is located peripherally
(arrow) in the left basal ganglia just lateral to the superior within the hematoma at the end of a single thalamoperforator
aspect of the left thalamic hematoma. The lateral ventricles which does not appear to have any link to the basal ganglia
are dilated with intraventricular hemorrhage. Figure 299-3. AVM. Spot sign mimics have been described in SICH due
Axial CTA source image through the hematoma showing to neoplastic calcification, micro AVM, pseudoaneurysms,
a focal peripheral contrast enhancement (arrow)the so- and aneurysms. About 20% of SICH undergo expansion.
called spot sign. Figure 299-4. Coronal CTA MIP through
the basal ganglia. There is a left basal ganglia collection of Question for Further Thought
vessels-a nidus (vertical white arrow) just laterally to the 1. What is the basis of the spot sign?
left thalamic hematoma fed by multiple left lenticulostriate
arteries (transverse white arrow) and draining via a large Reporting Responsibilities
thalamostriate vein (black vertical arrow). There is an iso- Acute hematoma requires direct reporting. The complica-
lated large arterial branch, a thalamoperforator, traced to the tions should be categorized and reported accordingly.
left posterior communicating artery within the left thalamic
hematoma with a small rounded end corresponding to the
spot sign (line arrow) possibly a microaneurysm. A right
transfrontal external ventricular drain is present (star). Size and location of the parenchymal hematoma
Complications such as intraventricular hemorrhage (IVH),
DIFFERENTIAL DIAGNOSIS N/A. subarachnoid hemorrhage (SAH), mass effect, and hydro-
cephalus
DIAGNOSIS Left thalamic hematoma with spot sign (asso- Enumeration of arterial feeders and venous outflow on
ciated basal ganglia arteriovenous malformation [AVM]). CTA to the AVM
Presence of the spot sign or extravasation if visible
DISCUSSION The thalamus is a common location for pri- If follow up, is there expansion of the hematoma?
mary intracerebral hematoma (PICH) of hypertensive ori- All hematoma in the young should be aggressively inves-
gin. The CT demonstrates the classical hyperdense mass of tigated
acute hematoma in the left thalamus with surrounding edema
and dissection into the ventricles. Hypertensive hematomas Answer
are more common in the elderly. Elderly patients are often 1. It is not clear whether the spot sign represents a primary
considered unlikely to harbor secondary vascular causes or a secondary phenomenon in primary ICH. Presence of
for hematoma and they may not be investigated. However, a primary phenomenon such as pseudoaneurysm, amy-
in the young, parenchymal hematoma anywhere should be loid-related microaneurysms, and Charcot-Bouchard
considered secondary (SICH) and investigated for treatable aneurysms in hypertension has been debated for years.
causes such as AVM, aneurysm, cerebral cavernous mal- The peripheral location of the spot sign in most PICH
formation (CCM), neoplasm, coagulopathy, or infection. may suggest a secondary phenomenon such as vascular
CTA in this situation demonstrates an AVM in the adja- injury; torn or stretched vessel. Spot sign mimics have
cent left basal ganglia. A spot sign is also demonstrated been demonstrated in secondary ICH.

Case
300 CLINICAL HISTORY Patient with known hepatic insufficiency with
confusion.
Figure 300-4
FINDINGS Figure 300-1. Axial T1WI through the basal

ganglia. There is symmetrical hyperintensity in the globi
pallidus (arrows). Figure 300-2. Axial T2WI in a different
Figure 300-3 patient. There is hyperintensity in the medial globi pallidi
642

Case 300 643
(arrows). Figure 300-3. Axial T2WI through the centrum and increased intracellular osmolality which are responsible
semiovale. There are patchy and nonspecific white matter for profound astrocyte changes including Alzheimer type II
abnormalities in both hemispheres (arrows). Figure 300-4. changes seen commonly in chronic HE and astrocyte swelling
Short TE (31 ms) proton MR spectra show elevation of glu- in acute HE. These abnormalities induce compensatory meta-
tamine and glutamate (arrow). bolic changes as brain edema, from mild to severe edema and
intracranial hypertension (as in fulminant hepatic failure).
DIFFERENTIAL DIAGNOSIS Hypoxic-ischemic enceph- HE is a clinical neuropsychiatric syndrome that occurs in
alopathy, neurofibromatosis type 1, Japanese encephalitis, patients with liver dysfunction, in the setting of either acute
hyperalimentation or total parenteral nutrition (manganese liver failure or acute superimposed on chronic or chronic
deposition), hemorrhage (methemoglobin), Wilson disease liver disease. The manifestations of HE vary from mild alter-
(copper toxicity and subsequent gliosis), basal ganglia cal- ations of mental status to severe coma and are sometimes
cification, nonketotic hyperglycemia associated with chorea- associated with motor function changes and cerebellar signs
balism, hypothyroidism, and carbon monoxide intoxication. (these are mainly in chronic stages), depending on the liver
disease clinical stage as designated by the West Haven clas-
DIAGNOSIS Chronic hepatic encephalopathy (HE). sification.
DISCUSSION MRI is the examination of choice in the Questions for Further Thought
evaluation of the patient with HE. CT may show nonspe- 1. Are there any other conditions than liver dysfunction that
cific findings. Hyperintense signal on T1WI in the basal gan- can cause manganese deposition in the brain?
glia (predominantly globus pallidus [GP]) and midbrain and 2. What is the underlying mechanism of T2WI signal abnor-
hyperintense signal in T2WI in thalami, posterior limb of the malities?
internal capsule, and periventricular white matter that may
also extend to the subcortical white matter and perirolandic Reporting Responsibilities
regions are the hallmarks of HE. Proton MR spectroscopy Routine reporting is sufficient in the absence of acute
may show elevated glutamine-glutamate and a decrease of changes. The diagnosis of hepatic insufficiency is a clinical
choline and myoinositol. These changes are interpreted as one. Imaging may be done to exclude other abnormality such
compensatory response to the increase in intracellular osmo- as cerebrovascular event. Correlate imaging findings with
lality caused by the accumulation of glutamine in astrocytes. the clinical presentation and beware of the variety of signal
The T1 signal abnormalities commonly resolve within a year abnormalities that can be found in brain MRI with underly-
following liver transplantation, and there is also a reduction ing acute and/or chronic hepatic failure.
in T2 signal abnormalities in the hemispheric white matter
or around the corticospinal tracts after liver transplantation, What the Treating Physician Needs to Know
suggestive of brain edema not only in the acute setting but Exclude intracranial hypertension, acute hemorrhages, or
also in chronic HE. Apart from hyperalimentation, methe- space-occupying lesions
moglobin, and some forms of calcification, bilateral sym-
metrical T1-weighted hyperintensity in the GP is not present Answers
in any of the differentials. 1. Manganese-related MR signal abnormalities have also
The pathophysiologic mechanisms of HE are not defini- been described in patients receiving total parenteral nutri-
tively established, but liver dysfunction disables detoxi- tion, patients with occupational exposure to manganese
fication of portal venous blood so that toxic substances from welding, and patients with noncirrhotic portal vein
accumulate and enter into the bloodstream (via a portosys- thrombosis or congenital portal-systemic bypass without
temic shunt) causing potential damage to the central nervous intrinsic hepatocellular disease.
system (CNS). Manganese is one of these toxic substances 2. The signal changes in T2WIs are increasingly reported
that shows preference to accumulate in the basal ganglia, as attributable to brain edema and reside in astrocytes but
predominantly in the GP, and is revealed by MRI signal may also be located in the interstitial compartment or be
changes described earlier. These changes reflect hyperam- secondary to disturbances in the bloodbrain barrier caus-
monemia which causes a rise in brain glutamine in astrocytes ing mild brain edema in cirrhotic patients.

Case
301 CLINICAL HISTORY 82-year-old female brought to the emergency room
because she was not acting herself, has new onset of mental status changes
andgarbled speech.
644

Case 301 645
FINDINGS Figures 301-1 and 301-2. Axial DWI with cor- right transfrontal external ventricular drain (EVD) place-
responding ADC map through the trigones. The trigones and ment. Yellow foul-smelling CSF was obtained during EVD
lateral ventricles are dilated. There are irregular fluidfluid placement. The Gram stain showed Gram-negative rods with
levels with restricted diffusion posteriorly in the trigones culture yielding Streptococcus viridans. There is continu-
(vertical arrows) consistent with cellular debris. There is a ing hydrocephalus and fluid levels (vertical arrows) in the
very thin ependymal diffusion restriction particularly around trigones. There is a rind of hypodensity (edema) around the
the right trigone (line arrows). There is a 2-cm round area of lateral ventricles outside the mildly hyperdense ependyma,
restricted diffusion in the right frontal lobe with surround- more prominent on the right than left (arrows).
ing vasogenic edema consistent with an abscess (transverse
arrow) with surrounding edema. Figure 301-3. Axial FLAIR DIFFERENTIAL DIAGNOSIS Intraventricular hematoma
through the frontal horns and trigones. There is hyperin- (IVH), ventriculitis due to ruptured abscess, intraventricular
tense (to cerebrospinal fluid [CSF]) cellular debris within the masses.
dilated trigones with fluidfluid levels (vertical arrow). There
is a confluent right frontal/caudate nucleus hyperintensity DIAGNOSIS Pyogenic ventriculitis (PV) (S. viridans).
(transverse arrow). There is a rind of hyperintensity (edema)
surrounding the lateral ventricles. Figure 301-4. Axial T2WI DISCUSSION PV is a bacterial inflammation of the ventri-
through the trigones. The debris posteriorly in the trigones cles. The typical MRI findings include dilatation of the ventri-
below the irregular fluid levels is relatively hypointense com- cles resulting in varying degrees of hydrocephalus, presence
pared with CSF (vertical arrows). The ependyma is hypoin- of dependent intraventricular cellular debris, ependymal
tense (transverse arrow) separating the cellular debris from thickening, and periventricular edema. Hydrocephalus was
the periventricular hyperintense edema. Figure 301-5. Axial present in 76% of one series. The dependent cellular debris is
post-contrast T1WI through the frontal horns. There is a the commonest finding in PV being present in 94% of same
rind of ependymal contrast enhancement (transverse arrows) series. It forms fluidfluid levels with the CSF and restricts
around the lateral ventricles with a ring enhancement around diffusion. The debris is usually slightly hyperintense to CSF
the right frontal abscess. The ring could be seen to open into on T1WI, hyperintense on FLAIR, and hypointense (to CSF)
the right frontal horn (vertical arrow), the point of rupture on T2WI. The thickened ependymal is diffusion restrict-
of the abscess. The enhancing ependymal is surrounded ing, hypointense on T2WI with a smooth contrast enhance-
by a hypointense edema. Figure 301-6. NCCT following a ment on post-contrast T1WI. The periventricular edema and

646 Case 301
s ignal abnormality were present in 78% of cases and is usu- tests. CSF as obtained by lumbar puncture (LP) may not
ally hypointense on T1, hyperintense on FLAIR and T2WI, be significantly abnormal as the ventriculitis could have
and clearly visualized on all sequences but more prominent been walled off from the rest of the CSF spaces. CSF by
on the FLAIR. It could be due to periventricular inflamma- other means particularly ventricular tap may show pleocy-
tion or transependymal CSF flow. The edema could be thin tosis and high protein. Hence imaging becomes very cru-
initially and depending on the severity of the hydrocephalus cial in the diagnosis. Treatment is by antimicrobial agents
and infection could extend to the subcortical WM. In this as determined by organism susceptibility test or empirical
case, one is able to visualize the cause of the ventriculitis, the choice due to inability to identify the offending microbe.
ruptured right frontal lobe abscess which restricts diffusion Direct intraventricular drug delivery has been recommended
centrally and has the usual thin smooth ring enhancement in cases of PV refractory to systemic antimicrobial therapy.
of a pyogenic abscess. There may be associated leptomen- CSF clearing of debris and disappearance of debris diffusion
ingeal enhancement in the presence of meningitis. The CT restriction on MRI has been advocated as a means of moni-
changes mirror the MRI findings. There is hydrocephalus, toring efficacy of therapy.
the dependent cellular debris which is slightly hyperdense
compared with CSF, the fluidfluid level, slightly hyper- Question for Further Thought
dense thickened ependymal and the surrounding hypodense 1. Are the imaging findings different in non-PV?
halo of periventricular edema. Ependymal smooth contrast
enhancement following contrast administration is common
on CT. Intraventricular hemorrhage is hyperdense on CT and
This is an emergency and requires direct reporting. Presence
shows varying intensity pattern on MRI depending on the
of an abscess and evidence of meningitis should be reported.
sequence and age of the hemorrhage. Ependymal enhance-
It is important that the abscess and the ventricular infection
ment is not usual in IVH, but there may be periventricular
be treated promptly and the hydrocephalus drained to relieve
edema if there is hydrocephalus. Intraventricular masses or
raised intracranial pressure.
ependymal masses may have nodular enhancing pattern.
PV otherwise known as ependymitis, pyocephalus, ven-
tricular empyema, or ventricular abscess is a common life- What the Treating Physician Needs to Know
threatening complication of meningitis in children but rarely Degree of hydrocephalus and location of the abscess or
present in the adult population. When it is not a complica- abscesses if there is more than one
tion of meningitis, it could be due to hematogenous infection Other parenchymal or leptomeningial changes if present
of the choroid plexus, complication of traumatic CSF leak, Is LP safe? There was no need for an LP in this instance
direct inoculation of organism during neurosurgical manipu- since the patient went for EVD placement immediately
lation or intraventricular drug delivery, and ruptured brain
abscess as in this situation. There is predominance of Gram- Answer
negative bacteria as the cause of PV closely followed by 1. Cellular debris is uncommon in non-PV. The irregular
staphylococcus sp. Clinical presentation could be insidious fluid levels of PV are usually absent. Ependymal contrast
without those constitutional symptoms and signs associated enhancement, periventricular edema, and hydrocephalus
with infection. However, fever, evidence of m eningismus are the predominant findings in this group of infections.
particularly if there is meningitis, headache, and mental sta- These microbes include toxoplasmosis, cytomegalovirus
tus changes are some of the presenting features. Diagnosis is (CMV), granulomatous infections such as TB and fungi.
usually by a combination of clinical, laboratory and i maging CMV is the most common responsible microbe in AIDS.

Case
302 CLINICAL HISTORY A 7-year-old female was initially seen to establish care
for known neurofibromatosis. Over the years her lesions grew substantially.
Figure 302-1
Figure 302-2
FINDINGS Figure 302-1. Coronal T2WI through the 302-4 are presumed schwannomas of the trigeminal and ves-
trigones. There is a 3.9-cm right trigonal isointense (to tibular nerves, respectively. Not shown here are right facial
gray matter [GM]) mass (arrow) with surrounding peritri- nerve and extensive schwannomas of the spinal nerves.
gonal vasogenic edema superiorly. Figure 302-2. Coronal
post-contrast T1WI. The intraventricular mass enhances DIFFERENTIAL DIAGNOSIS Neurofibromatosis type 2
homogeneously and avidly. This is a surgically proven intra- (NF2), Neurofibromatosis type 1 (NF1), schwannomatosis.
ventricular meningioma. Figure 302-3. Axial post-contrast
T1WI through the cavernous sinuses. There are bilateral DIAGNOSIS Neurofibromatosis type 2 (NF2).
lobulated contrast-enhancing masses along the path of the
bilateral trigeminal nerves (transverse arrows). Figure 302-4. DISCUSSION The hallmark of NF2 is bilateral vestibu-
Axial post-contrast T1WI through the internal auditory canal lar schwannomas. These could be demonstrated on MRI
(IAC). There are lobulated contrast-enhancing masses in as avidly contrast enhancing iso- to slightly hypointense
bilateral cerebellopontine angle (CPA) extending into the masses in the IAC and CPA on T1WI. If they grow sub-
IACs (transverse arrows). There is a similar contrast-enhanc- stantially, they become lobulated and compress the bra-
ing smooth marginated mass in the region of the right fora- chium pontis and the adjoining cerebellum and pons.
men ovale (vertical arrow). Tumors in Figures 302-3 and Intracanalicular extension with widening of the IAC may
647

648 Case 302
occur. The mass effect increases as the tumors grow. Cutaneous schwannomas are common. Neurofibromas are
Schwannomas of other cranial nerves such as facial, tri- rarely present. Caf-au-lait spots are not as common as in
geminal, and oculomotor nerves are present in about 50% NF1. Diagnosis is made on the basis of established criteria
of NF2 population. Meningiomas and ependymomas are that categorize the diagnosis into definite and probable NF2.
common in these patients and they are generally multiple. Genetic testing is necessary to confirm the diagnosis. In
Intraventricular meningioma is a very rare tumor and pres- view of the unpredictable nature of the many tumors in these
ence of intraventricular meningioma in a child should raise patients, it is not possible to treat all tumors. It is recom-
the suspicion for neurofibromatosis. MRI with contrast is mended that only symptomatic tumors be treated.
the most appropriate test for these patients in view of life-
long surveillance for new tumor and tumor growth. The Question for Further Thought
growth of these tumors is usually unpredictable and new 1. Is imaging surveillance necessary in NF2?
tumors spring up all the time. Spinal schwannomas and
ependymomas are usually multiple and can grow to enor- Reporting Responsibilities
mous sizes. Schwannomatosis usually presents with non- The presence of tumors calls for direct reporting and more
vestibular multifocal schwannomas mostly of the spine and so if there is hydrocephalus or compression of eloquent brain
peripheral nerves with a small percentage of intracranial regions. Suspicion of NF2 should prompt recommendation
and subcutaneous tissue schwannomas. It does not have for spinal imaging to exclude spinal tumors.
any cutaneous stigmata. Most schwannomatosis are spo-
radic with only about 15% with a family history. NF1 is
largely extracranial in its manifestations with cutaneous and
peripheral neurofibromas and bone changes predominating. Location and types of intracranial masses
About 15% of NF1 show intracranial manifestations. Presence of complications such as hydrocephalus, or
NF2 is a rare autosomal dominant disease due to muta- edema
tion in NF2 tumor suppressor gene (Merlin) on chromo- Recommendation for spinal imaging
some 22q12. The prevalence is estimated at 1:25,000 births. Growth or quiescence of masses on follow-up imaging
Family history may be absent in about 50% of NF2. Most
NF2 patients present in their second to fourth decades, with Answer
a minority of patients presenting in the first decade as in 1. MRI surveillance is recommended in NF2 because of the
this patient. There is no gender preference. Most will pres- propensity for multiple and sometimes debilitating intra-
ent with vestibular dysfunctions of tinnitus and sensorineu- cranial tumors. Both cranial and spine contrast-enhanced
ral hearing loss. Trigeminal neuralgia, seizures, cataracts, MRI are usually used in this population to screen for
and facial nerve palsy are other common presentations. tumors.

Case 303 CLINICAL HISTORY Teenage male with precocious sexual development.
FINDINGS Figure 303-1. Sagittal non-contrast T1WI. Axial T2WI in a companion case. There is a mass within
There is a broad-based isointense mass (arrow) in the region the suprasellar cistern of heterogeneous intensity contain-
of the tuber cinereum. Figure 303-2. Coronal non-contrast ing some central hyperintensities (arrows). Figure 303-4.
T1WI in the same patient shows that the mass has exactly the Sagittal post-contrast T1WI shows the extent of the nonen-
same signal as the gray matter (GM) (arrow). Figure 303-3. hancing mass (arrow).
649

650 Case 303
DIFFERENTIAL DIAGNOSISHypothalamic-chiasmatic u sually administered for the gelastic epilepsy and the preco-
glioma, craniopharyngioma, germinoma, and Langerhans cious puberty associated with hamartomas, although some
cell histiocytosis, hamartoma of tuber cinereum. authors propose a surgical approach in exceptional cases.
DIAGNOSIS Hamartoma of tuber cinereum (HTC). Questions for Further Thought

1. With what syndrome is HTC associated?
DISCUSSION Hamartomas (from the Greek word hamar- 2. What is the gender preference and age at presentation of
tia that means error) arising from the tuber cinereum (Latin HTC?
for ash-colored mass), a part of the hypothalamus located
between the mamillary bodies and the pituitary stalk, are rare
nonneoplastic congenital heterotopias of GM. Frequently,
Routine reporting is sufficient in this benign nonneoplastic
HTCs are seen as subcentimeter homogeneous sessile (intra-
mass. Describe the location, morphology, and extent of the
hypothalamic) or pedunculated (parahypothalamic) nonen-
lesion. Identify and describe any other epileptogenic abnor-
hancing masses with a similar density or intensity to GM.
malities such as focal cortical dysplasias or findings that may
They are sometimes cystic and generally do not grow when
suggest mesiotemporal sclerosis. Exclude the possibility of a
compared on repeated examinations. It has been described
hypothalamic or optic nerve glioma by the location, lack of
in some published series that the variability of iso- to hyper-
enhancement, and absence of growth when compared with
intense signal on T2WI relates directly to the glial content
prior examinations.
found in hamartomas. Some authors have pointed out that the
proton MR spectroscopy findings, consisting of a decrease in
NAA/Cr ratio, an increase in the mI, and a probable increase What the Treating Physician Needs to Know
in the Cho/Cr ratio, probably reflect an increased number of Localize the seizure-remediable focus or region so that a
dysplastic neurons. Moreover, ictal SPECT and FDG-PET possible surgical treatment can be determined
studies demonstrate hypermetabolism localized at the region The location and morphology of the lesion, as well as the
of the HTC. extent and the relationship with other important structures
Occasionally, HTCs may be considered as giant when they such as suprasellar structures (optic nerves)
measure over 1 cm in size. Sometimes, truly giant ones are
encountered and their signal intensities tend to vary more than Answers
that of the more common smaller ones. HTC presents clinically 1. Pallister-Hall syndrome is characterized by HTC, anal
with the classic triad of gelastic seizures, central precocious atresia, renal anomalies, and limb malformations.
puberty (oversecretion of gonadotropin-releasing hormone 2. Most patients present in the first or second decade of life,
[GnRH]), and developmental delay. Medical treatment is with males being more affected than females.

SuggeSuggested
s t e dReadings
R e a ding s
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A P P E NDIX
Case 1 Vasogenic Edema

Stephen Bagg Mauricio Castillo Matthew F. Omojola
Case 2 Pituitary Metastasis

John M. Collins Colin S. Poon
Case 3 Pantothenate Kinase-Associated Neurodegeneration (PKAN)

Sofia Pina Mauricio Castillo
Case 4 Chiari III Malformation (CIIIM)

Matthew F. Omojola
Case 5 Malignant Pituitary Macroadenoma

Miguel Lemus Mauricio Castillo
Case 6 Corpus Callosum Infarct

Rana K. Zabad Matthew F. Omojola
Case 7 Intralabyrinthine (cochlea) Schwannoma (ILS)

William B. Zucconi Michele H. Johnson
Case 8 Longitudinal Extensive Transverse Myelitis (LETM)
Case 9 Acute right MCA Infarct (Contrast Enhancing)

Matthew F. Omojola Pierre Fayad
Case 10 Unilateral Brachium Pontis Lesion

Case 11 Epidermoid Cyst

Matthew F. Omojola Karen Ragland Cole
Case 12 Focal Cortical Dysplasia

Karen Ragland Cole Matthew F. Omojola
Case 13 Optic Neuritis

Matthew F. Omojola Rana K. Zabad
Case 14 Corpus Callosum Tumefactive Demyelination (CC TDL)

Case 15 Epidural Abscess

Frank J. Minja Michele H. Johnson Matthew F. Omojola
Case 16 Neurofibromatosis Type 1 (NF1)

Matthew F. Omojola
Case 17 Rathkes Cleft Cyst

Gibson J Mauricio Castillo
Case 18 CTA and Perfusion of Brain Ischemia

Case 19 Rhombencephalosynapsis (RES)

Matthew F. Omojola
Case 20 Vestibular Schwannoma (VS)

William B. Zucconi Michele H. Johnson
Case 21 Choroid Plexus Carcinoma WHO III

Matthew F. Omojola
Case 22 Watershed infarcts

675
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676 Appendix
Case 23 Artery of Percheron Infarcts

Case 24 Parasellar Aneurysm

Case 25 Basilar Summit Aneurysm with SAH

Case 26 Multiple Sclerosis MS

Case 27 Cavernous Aneurysm

Case 28 Cryptococcosis of Basal Ganglia

Case 29 Treatment Related Changes

Matthew F. Omojola
Case 30 Bilateral Brachium Pontis Lesions

Case 31 Corpus Callosum Tuberculoma (CC TB)

Case 32 Choroid Plexus Metastasis
Matthew F. Omojola
Case 33 Wernickes Encephalopathy

Case 34 Lateral Medullary Infarct (LMI)

Case 35 Arteriovenous Malformation (AVM)

Case 36 Gliosarcoma
Matthew L. White Syed J. Kazmi Matthew F. Omojola
Case 37 Ommaya Reservoirs

Matthew F. Omojola
Case 38 Chronic Calcified Subdural Hematoma (CCSDH)

Matthew F. Omojola
Case 39 Superficial Siderosis

Case 40 Subgaleal Cyst

Case 41 Tumefactive MS
Case 42 Dementia with Lewy Bodies (DLB)

Elcin Zan Alin Chirindel Matthew Kruse David Yousem Rathan Case M. Subramaniam
Case 43 Tectal Glioma

Matthew F. Omojola
Case 44 Posterior Fossa Ependymoma

John M. Collins Colin S. Poon Matthew F. Omojola
Case 45 Anaplastic Ependymoma WHO III

Matthew F. Omojola
Case 46 Acute Disseminated Encephalomyelitis (ADEM)

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Appendix 677
Case 47 Creutzfeld Jakob Disease (CJD)

Elcin Zan Alin Chirindel Matthew Kruse David Yousem Case Rathan M. Subramaniam
Case 48 Pituicytoma
Case 49 Posterior Reversible Encephalopathy Syndrome (PRES)

Case 50 Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis and Stroke-Like Episodes (MELAS)
Case 51 Cholesterol Granuloma

Case 52 Subarachnoid Hemorrhage (non-Aneurysmal)

Case 53 Cytotoxic Edema

Stephen Bagg Mauricio Castillo Matthew F. Omojola
Case 54 Parasellar Meningioma

Miguel Lemus Mauricio Castillo Matthew F. Omojola
Case 55 Dural Arteriovenous Fistula (DAVF)

Vahe M. Zohrabian Michele H. Johnson
Case 56 Craniopharyngioma
Miguel Lemus Mauricio Castillo Matthew F. Omojola
Case 57 Traumatic Intracerebral Hematoma (TICH)

Matthew F. Omojola
Case 58 Chiari II malformation (CIM) MRI

Matthew F. Omojola
Case 59 Lobar Hematoma with Microhemorrhages

Case 60 Osmotic Demyelination Syndrome (ODS): Central Pontine Myelinolysis

Case 61 Fahrs Disease (Bilateral Striopallidodentate Calcinosis)

Mauricio Castillo
Case 62 Cerebellar Atrophy

Case 63 Pilomyxoid Astrocytoma

Case 64 Pilocytic Astrocytoma

Case 65 CT Perfusion in Ischemic Infarct

Case 66 Idiopathic Hypertrophic Pachymeningitis

William B. Zucconi Michele H. Johnson Matthew F. Omojola
Case 67 Transependymal Cerebrospinal Fluid Flow

Ray Peeples Mauricio Castillo Matthew F. Omojola
Case 68 Von Hippel-Lindau Disease (VHL Disease)

Matthew F. Omojola
Case 69 Cortical Ependymoma

Case 70 Carbon Monoxide Poisoning

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678 Appendix
Case 71 Chiari I Malformation (CIM)

Matthew F. Omojola
Case 72 Cerebellopontine Angle (CPA) Meningioma

Ajay Malhotra Michele H. Johnson
Case 74 Optic Glioma

Case 75 Mitochondrial encephalopathy with lactic acidosis and strokes (MELAS)

Mauricio Castillo
Case 76 Cortical Laminar Necrosis

Case 77 Uncal Herniation (UH)

Matthew F. Omojola
Case 78 Delayed Post-Hypoxic Leukoencephalopathy (DPHL)

Case 79 Leptomeningeal Metastasis

Case 80 Acute Hypertensive Basal Ganglia Hematoma
Case 81 Krabbe Disease (Globoid Cell Leukodystrophy)

Stephen Bagg Mauricio Castillo
Case 82 Neurocysticercosis Giant Vesicular Cyst

Diana F. Florescu Matthew F. Omojola
Case 83 Alzheimer Disease (AD)

Elcin Zan Alin Chirindel Matthew Kruse Case David Yousem Rathan M. Subramaniam
Case 84 Pituitary Macroadenoma

Case 85 Bacterial Meningitis

Case 86 Posterior Cerebral Artery Territory Infarct. (PCA Infarct)

Case 87 Persistent Trigeminal Artery (PTA)

Case 88 Intraventricular Meningioma

Matthew F. Omojola
Case 89 Posterior Fossa Arteriovenous Malformation (AVM)

Matthew F. Omojola
Case 90 Acute Subacute Hypoxic Ischemic Encephalopathy CT (Adult)

Case 91 Dural Metastasis Breast

Frank J. Minja Michele H. Johnson
Case 92 Cavernous Sinus Schwannoma

Case 93 Multi System Atrophy (MSA)

Case 94 Neurocysticercosis Vesicular/Colloidal

Matthew F. Omojola Diana F. Florescu
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Appendix 679
Case 95 Lacunar Infarct Basal Ganglia

Case 96 Hypertrophic Olivary Degeneration

Case 97 Oligodendroglioma II
Case 98 Methotrexate Leukoencephalopathy (MTX LE)

Case 99 Mycotic Aneurysm Left ICA

Case 100 Spontaneous Intracranial Hypotension

Case 101 Corpus Callosum MS

Case 102 Brain Stem Encephalitis

Matthew F. Omojola
Case 103 Acute Subdural Hematoma. ASDH

Matthew F. Omojola
Case 104 Canavan Disease
Felipe Espinoza Mauricio Castillo Matthew F. Omojola
Case 105 Normal Brain Venous Anatomy

Case 106 Carotid Cavernous Fistula (I)

Case 107 Nocardiosis

Matthew F Omojola Diana F. Florescu
Case 108 Post Transplant Lymphoproliferative Disease PTLD

Case 109 Corpus Callosum Susacs Syndrome

Case 110 Holoprosencephaly with Lissencephaly

Matthew F. Omojola Karen Cole
Case 111 Hemangioblastoma

Colin S. Poon John M. Collins
Case 112 Dural Arteriovenous Fistula (DAVF)

Case 113 Pelizaeus-Merzbacher Disease (PMD)

Gibson J Mauricio Castillo Matthew F. Omojola
Case 114 Developmental Venous Anomaly

Case 115 Pleomorphic Xanthoastrocytoma PXA

Case 116 Moyamoya Disease (MMD)

Case 117 Acute on Chronic Subdural Hematoma

Matthew F. Omojola
Case 118 Septic Emboli (SE)

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680 Appendix
Case 119 Zellweger Syndrome

Case 120 Huntingtons Disease

Noelia Silva Priegue Mauricio Castillo
Case 121 ICA Dissection with MCA Infarct

Case 122 Aneurysm Coil

Matthew F. Omojola
Case 123 Septo-optic dysplasia (SOD)

Case 124 Brain Miliary Tuberculosis

Case 125 Sinus Pericranii

Case 126 Lipoma

Case 127 Hemangiopericytoma (HPC)

Case 128 Vascular Fenestrations (VF)
Case 129 Agenesis of Corpus Callosum (ACC)

Case 130 Methanol Intoxication

Case 131 Meningoencephalitis Streptococcus pneumoniae

Case 132 Intraventricular hematoma (IVH)

Matthew F. Omojola
Case 133 Cerebral Arterial Gas Embolism (CAGE)

Case 134 Meningioma

Case 135 Brain Tuberculomata and Tuberculous Abscess

Case 136 Cavernous Sinus Thrombosis

Case 137 Metachromatic Leukodystrophy (MLD)

Felipe Espinoza Mauricio Castillo Matthew F. Omojola
Case 138 Traumatic Subarachnoid Hemorrhage

Matthew F. Omojola
Case 139 Posterior Fossa Choroid Plexus Papilloma

Case 140 Middle Cerebral Artery Aneurysm With Cerebral Vasospasm

Case 141 Dandy Walker Malformation (DWM)

Matthew F. Omojola
Case 142 Schizencephaly

Case 143 Multiple myeloma

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Appendix 681
Case 144 Duplicated Vertebral Artery with Pseudoaneurysm

Case 145 Tuberous Sclerosis Complex (TSC)

Matthew F. Omojola
Case 146 DTI Color Directional Maps Supratentorial WM Tracts

Matthew F. Omojola
Case 147 Hypoxic-Ischemic Injury

Case 148 Disseminated Toxoplasmosis Encephalitis (DTE)

Case 149 Chronic Subdural Hematoma (CSDH)

Matthew F. Omojola
Case 150 Susacs Syndrome

Case 151 Aneurysm Clip

Matthew F. Omojola
Case 152 Superior Sagittal Sinus Thrombosis (SS thrombosis)

Case 153 Cerebellopontine Angle Arachnoid Cyst
Case 154 Xanthogranulomatous Degeneration of the Choroid Plexus (XGD)

Matthew F. Omojola
Case 155 Subfalcine Herniation (SFH)

Matthew F. Omojola
Case 156 Cerebellar Hypoplasia or Chiari IV Malformation

Matthew F. Omojola
Case 157 Corpus Callosum PRES

Case 158 Progressive Multifocal Leukoencephalopathy (PML)

Case 159 Infantile Hemangioma

Case 160 Hemorrhage Into a High Grade Tumor

Case 161 Parietal Vertex Cephalocele

Matthew F. Omojola
Case 162 Granular Cell Astrocytoma

Case 163 Calcified Neurocysticercosis

Matthew F Omojola Diana F. Florescu
Case 164 Adult Medulloblastoma

Matthew F. Omojola

Case 166 Germinoma

Matthew F. Omojola
Case 167 Sturge-Weber Syndrome (SWS)

Matthew F. Omojola
Case 168 Extraaxial (Dural) Lymphoma

William B. Zucconi DO and Michele H. Johnson MD
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682 Appendix
Case 169 Traumatic Subdural Hygroma (SDG)

Matthew F. Omojola
Case 170 Neuromyelitis Optica (NMO)

Case 171 Choroid Plexus Papilloma WHO I (CPP)

Matthew F. Omojola
Case 172 Foramen Magnum Meningioma

Robert D. Messina Michele H. Johnson
Case 173 Subependymoma

Matthew F. Omojola
Case 174 Vein of Galen Aneurysmal Malformation (VGAM)

Case 175 Ganglioglioma

Case 176 Lissencephaly

Case 177 Post-Traumatic CSF Collection

Samer Salhab Michele H. Johnson Matthew F. Omojola
Case 178 Occipital Cephalocele
Matthew F. Omojola
Case 179 Corpus Callosum Oligodendroglioma WHO II

Case 180 Hypertensive Brain Stem Hematoma

Case 181 Multifocal hemorrhagic metastases (HM)

Case 182 Bilateral thalamic hemorrhagic infarcts (Deep cerebral venous sinus thrombosis)
Case 183 Aberrant Neurohypophysis

Case 184 Post transplant lymphoproliferative disorder (PTLD)

Case 185 Normal Brain MR Imaging Anatomy

Matthew F. Omojola
Case 186 Aneurysmal Bone Cyst (ABC)

Matthew F. Omojola
Case 187 HIV Encephalopathy

Case 188 Leptomeningial Venous Collaterals

Case 189 Intraventricular Lipoma (IVL)

Matthew F. Omojola
Case 190 Joubert Syndrome

Matthew F. Omojola
Case 191 Multifocal White Matter Changes


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Appendix 683
Case 193 West Nile Virus

Case 194 Cylindrical and Fusiform Aneurysms

Case 195 Top of the Basilar Syndrome

Case 196 Brainstem Glioma (BSG)

Matthew F. Omojola
Case 197 Fronto-temporal Dementia

Case 198 Mesial Temporal Sclerosis (MTS)

Case 199 Cowden Syndrome

Matthew F. Omojola
Case 200 Central Neurocytoma

Matthew F. Omojola
Case 201 Facial Nerve Schwannoma

William Zucconi Michele H. Johnson
Case 202 Pilocytic Astrocytoma Lobar
Case 203 Anterior Communicating Artery Aneurysm (A-Com A)

Case 204 Cerebral White Matter Disease (Leukoaraiosis)

Case 205 Arachnoid Cyst

Case 206 Petrous Apicitis

Case 207 Descending Transtentorial Herniation (DTTH)

Matthew F. Omojola
Case 208 Posterior Fossa Atypical Teratoid Rhabdoid Tumor

Case 209 Normal Pressure Hydrocephalus (NPH)

Case 210 Peripheral Mycotic Aneurysm

Case 211 Osmotic Demyelination Syndrome (ODS): Central Pontine Myelinolysis (CPM) and Extrapontine Myelinolysis (EPM)
Matthew F Omojola Rana K Zabad
Case 212 Reversible Cerebral Vasoconstriction Syndrome (RCVS)

Case 213 Cerebellar Dysplasia

Matthew F. Omojola
Case 214 Adrenoleukodystrophy (ALD)

Felipe Espinoza Mauricio Castillo
Case 215 Pituitary Microadenoma

Case 216 Brain Aspergillosis

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684 Appendix
Case 217 Dermoid Tumor

Case 218 Hyperacute Subdural Hematoma (HSDH)

Matthew F. Omojola
Case 219 Lymphocytic Hypophysitis

Case 220 Paget Disease

Case 221 Anterior cerebral artery territory infarct (ACA infarct)

Case 222 Progressive Supranuclear Palsy (PSP)

Case 223 Posterior Communicating Artery Aneurysm (P-Com-A)

Case 224 Glioblastoma

Case 225 Alobar Holoprosencephaly

Case 226 Spontaneous Thrombosis of AVM
Case 227 Hepatic Encephalopathy (HE)

Case 228 Pituitary Axis Neurosarcoidosis

Case 229 Fibrous Dysplasia (FD)

Matthew F. Omojola
Case 230 Pneumocephalus

Matthew F. Omojola
Case 231 Subarachnoid Space Lymphoma

Case 232 Cerebral Cavernous Malformation (CCM)

Case 233 Hypertensive Basal Ganglia Hematoma

Case 234 Carotid-cavernous Fistula (II)

Case 235 Langerhans Cell Histiocytosis

Matthew F. Omojola Matthew L. White
Case 236 Cerebral Contusion

Matthew F. Omojola
Case 237 Enlarged Endolymphatic Sac Syndrome

Case 238 Foramen Magnum Herniation

Matthew F. Omojola
Case 239 Hemangioma of Cranial Vault

Case 240 Acute Epidural Hematoma (AEDH)

Matthew F. Omojola
73284_app_p675-687.indd 684 10/10/14 6:13 PM

Appendix 685
Case 241 Brain Pyogenic Abscess with Ventriculitis

Case 242 Corpus Callosum Lymphoma

Case 243 Anaplastic Astrocytoma III

Case 244 Vascular Supply to the Head: The Aortic Arch Variants
Case 245 Crossed Cerebellar Diaschisis and Atrophy

Case 246 Hypoglossal Schwannoma

Bryan S. Jeun Michele H. Johnson
Case 247 Subarachnoid Hemorrhage CT

Case 248 Anaplastic Oligodendroglioma III

Case 249 Hydranencephaly

Matthew F. Omojola
Case 250 Langerhans Cell Histiocytosis (LCH)
Case 251 Corpus Callosum Traumatic Hematoma

Case 252 Chiari II Malformation CT

Matthew F. Omojola
Case 253 Hydrocephalus Aqueductal Stenosis

Matthew F. Omojola
Case 254 Orbital and Nasofrontal Cephalocele

Case 255 Colloid Cyst of the Third Ventricle

Matthew F. Omojola
Case 256 Radiation Induced Leukoencephalopathy

Case 257 Bilateral Thalamic Glioma

Case 258 Developmental Venous Anomaly (DVA)

Case 259 Lymphoma

Case 260 Cryptococcal Meningoencephalitis

Case 261 Pituitary Hemorrhage

Case 262 Hippocampal Malrotation (HIMAL)

Matthew F. Omojola
Case 263 Subgaleal Lipoma

Case 264 Familial Cerebral Cavernous Malformation

73284_app_p675-687.indd 685 10/10/14 6:13 PM

686 Appendix
Case 265 Wilsons Disease

Case 266 Hemimegalencephaly

Case 267 Paraneoplastic Limbic Encephalitis (PLE)

Case 268 Parkinson Disease

Case 269 Dysembryoplastic Neuroepithelial Tumor (DNET)

Case 270 Paraganglioma (Glomus Jugulare)

Matthew F. Omojola
Case 271 Medulloblastoma

Case 272 CC Methotrexate Leukoencephalopathy MTX LE (Reversible CC lesion)

Case 273 Hypoxic-ischemic Encephalopathy in Term Infants

Case 274 Intraventricular Neurocysticercosis
Matthew F. Omojola
Case 275 Normal Brain Circle of Willis (COW)

Case 276 Diffuse Astrocytoma II

Case 277 Optic Pathway Glioma

Case 278 Intraventricular Simple Cyst

Matthew F. Omojola
Case 279 Osteomyelitis

Case 280 Corpus Callosum Lipoma

Case 281 Toxoplasmosis

Case 282 Alexander Disease

Felipe Espinoza Mauricio Castillo
Case 283 Corpus Callosum Glioblastoma WHO IV

Case 284 Intraventricular primary CNS lymphoma (PCNSL)

Matthew F. Omojola
Case 285 Sebaceous (Trichilemmal or Pilar) Cyst

Case 286 Bilateral Middle Cerebral Artery Territory Infarction

Case 287 Idiopathic Calcifications

Case 288 Traumatic Brain Injury/Diffuse Axonal Injury (TBI/DAI)

Matthew F. Omojola
73284_app_p675-687.indd 686 10/10/14 6:13 PM

Appendix 687
Case 289 Pilocytic Astrocytoma (Posterior Fossa)

Case 290 Varicella-Zoster-Virus Encephalitis (VZVE)

Case 291 Creutzfeldt-Jakob disease variant (vCJD)

Case 292 Skull Base Meningioma

William Zucconi
Case 293 Azygos Anterior Cerebral Artery

Case 294 Cranial Vault Osteosarcoma

Matthew F. Omojola
Case 295 Chordoma

William Zucconi Michele H. Johnson
Case 296 Periventricular Nodular Gray Matter Heterotopia (PNH)

Case 297 CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy)
Case 298 Leptomeningeal Enhancement
Matthew F. Omojola
Case 299 Thalamic Hematoma with Spot Sign

Case 300 Hepatic Encephalopathy

Case 301 Pyogenic Ventriculitis

Matthew F. Omojola
Case 302 Neurofibromatosis Type 2 (NF2)

Matthew F. Omojola
Case 303 Hamartoma of Tuber Cinereum

73284_app_p675-687.indd 687 10/10/14 6:13 PM

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Neuroimaging

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Matthew F. Omojola, MD, FRCPC

Mauricio Castillo, MD, FACR

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Copyright 2015 Wolters Kluwer Health

Library of Congress Cataloging-in-Publication Data

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73284_fm_pi-x.indd 5 08/08/14 6:59 AM

Stephen Bagg, MD Diana F. Florescu, MD

Mauricio Castillo, MD, FACR J. Gibson, MD

Alin Chirindel, MD Bryan S. Jeun, MD

Karen Ragland Cole, MD, MPH, MBA Michele H. Johnson, MD

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Robert D. Messina, MD Rathan Subramaniam, MD

Colin S. Poon, MD, PhD, FRCPC Elcin Zan, MD

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Figure 1-1 Figure 1-2

Figure 1-3 Figure 1-4

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73284_Online-case001_p001-002.indd 2 10/7/14 7:44 PM

Figure 2-1 Figure 2-2

Figure 2-3 Figure 2-4

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Figure 3-1 Figure 3-2

DIAGNOSIS Pantothenate kinase-associated neurodegen- Questions for Further Thought

73284_Online-case003_p005-006.indd 5 22/09/14 9:20 PM

73284_Online-case003_p005-006.indd 6 22/09/14 9:20 PM

Figure 4-1 Figure 4-2

extends down to the ventricular CSF, indicating corpus cal-

DIFFERENTIAL DIAGNOSISChiari III malformation

DISCUSSION Cardinal imaging findings in CIIIM are the

73284_Online-case004_p007-008.indd 7 22/09/14 9:22 PM

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Figure 5-1 Figure 5-2

Figure 5-3 Figure 5-4

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best reflects the tumors aggressiveness, being almost cer-

DISCUSSION MRI is the best imaging technique to Reporting Responsibilities

73284_Online-case005_p009-011.indd 10 22/09/14 9:23 PM

73284_Online-case005_p009-011.indd 11 22/09/14 9:23 PM

Figure 6-1 Figure 6-2

Figure 6-3 Figure 6-4

73284_Online-case006_p012-013.indd 12 19/09/14 3:06 PM

hyperintensity on FLAIR and T2WI. MRA or CTA may be

Question for Further Thought

DIAGNOSIS CC infarct (right anterior cerebral artery Answer

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Figure 7-1 Figure 7-2

Figure 7-3 Figure 7-4

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Figure 8-1 Figure 8-2

Figure 8-3 Figure 8-4

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DIAGNOSIS Longitudinally extensive transverse myelitis

73284_Online-case008_p016-017.indd 17 19/09/14 3:07 PM

Figure 9-1 Figure 9-2

Figure 9-3 Figure 9-4