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RADIOLOGY

CASE REVIEW SERIES | Brain Imaging


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RADIOLOGY
CASE REVIEW SERIES | Brain Imaging

Cha ng Yu eh Ho, MD
Assistant Professor of Clinical Radiology
Director of Pediatric Neuroimaging
Riley Hospital for Children at IU Health
Indiana University School of Medicine
Indianapolis, Indiana

Rocky Sa enz, DO, FAOCR


Vice-Chairman, Botsford Department of Radiology
Director of MRI, Botsford Hospital
Clinical Faculty, Michigan State University
Farmington Hills, Michigan

S ERIES ED ITO R

Rola n d Ta la n ow, MD, Ph D


President
Department of Radiology Education
Radiopolis, a subdivision of InnoMed, LLC
Stateline, Nevada

New York Chicago San Francisco Athens London


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Sydney Toronto
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To my family, especially my wife Angel and my daughters,
as well as good friends in the work and church communities:
You keep me grounded, supported,
and focused on the most important relationships in life.
— Chang Yueh Ho, MD

First, I would like to thank Roland Talanow, as the mastermind


behind this case review series and its larger online component.
I would also like to give special thanks to my coauthor Chang Ho
for his dedication and brilliance in making this book
the quality project that it is. Next, I would like to thank my
residents Sharon Kreuer and Deeshali Patel-Shah for contributing cases.
Lastly and most importantly, I thank my mother Angelita, wife Blanca,
and sons Rocky, Russell, Ronin, and Rex. Without my strong family support,
I would not be able to complete this academic project or any
of my other creative works.
— Rocky Saenz, DO
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Contents

Series Preface ix
Preface xi

Easy Cases 1

Moderately Di cult Cases 205

Most Di cult Cases 387

Regional Subject Index 459


Subchapter Index 461
Alphabetical Subject Index 463
Di culty Level Index 465
Author Index 467
Acknowledgment Index 469

vii
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Series Preface

M
aybe I have an obsession or cases, but when I was with images and questions and page 2 with the answers and
a radiology resident I loved to learn especially rom explanations. This approach avoids unintentional peeking at
cases, not only because they are short, exciting, and the answers be ore deciding on the correct answers yoursel .
un—similar to a detective story in which the aim is to get to We keep it strict: one case per page! This way it remains up
“the bottom” o the case—but also because, in the end, that’s to your own knowledge to f gure out the right answer.
what radiologists are aced with during their daily work. Another example that residents (including me) did miss
Since medical school, I have been ascinated with learning, in traditional case review books is that these books did not
not only or my own benef t but also or the sake o teaching highlight the pertinent f ndings on the images: sometimes,
others, and I have enjoyed combining my IT skills with my even looking at the images as a group o residents, we could
growing knowledge to develop programs that help others in not f nd the abnormality. This is not only rustrating but also
their learning process. Later, during my radiology residency, time-consuming. When you prepare or the boards, you want
my passion or case-based learning grew to a level where the to use your time as e iciently as possible. Why not show
idea was born to create a case-based journal: integrating new annotated images? We tackled that challenge by provid-
concepts and technologies that aid in the traditional learn- ing, on the second page o each case, the same images with
ing process. Only a ew years later, the Journal of Radiology annotations or additional images that highlight the f ndings.
Case Reports became an internationally popular and PubMed When you are preparing or the boards and managing
indexed radiology journal—popular not only because o your clinical duties, time is a luxury that becomes even more
the interactive eatures but also because o the case-based precious. Does the resident preparing or the boards truly
approach. This led me to the next step: why not tackle some- need lengthy discussions as in a typical textbook? Or does the
thing that I especially admired during my residency but that resident rather want a “rapid ire” mode in which he or she
could be improved—creating a new interactive case-based can “ ly” through as many cases as possible in the shortest
review series. I imagined a book series that would take into possible time? This is the reality when you start your work
account new developments in teaching and technology and a ter the boards! Part o our concept with the new series is
changes in the examination process. providing short “pearls” instead o lengthy discussions. The
As did most other radiology residents, I loved the tradi- reader can easily read and memorize these “pearls.”
tional case review books, especially or preparation or the Another challenge in traditional books is that questions
boards. These books are quick and un to read and ocus in a are asked on the f rst page and no direct answer is provided,
condensed way on material that will be examined in the f nal only a lengthy block o discussion. Again, this might become
boards. However, nothing is per ect and these traditional case time-consuming to ind the right spot where the answer
review books had their own intrinsic aws. The authors and is located i you have doubts about one o several answer
I have tried to learn rom our experience by putting the good choices. Remember: time is money—and li e! There ore,
things into this new book series but omitting the bad parts we decided to provide explanations to each individual ques-
and exchanging them with innovative eatures. tion, so that the reader knows exactly where to f nd the right
What are the eatures that distinguish this series rom answer to the right question. Questions are phrased in an
traditional series o review books? intuitive way so that they it not only the print version but
To save space, traditional review books provide two also the multiple-choice questions or that particular case in
cases on one page. This requires the reader to turn the page our online version. This system enables you to move back
to read the answer or the irst case but could lead to unin- and orth between the print version and the online version.
tentional “cheating” by seeing also the answer o the second In addition, we have provided up to 3 re erences or
case. Doesn’t this de eat the purpose o a review book? From each case. This case review is not intended to replace tra-
my own authoring experience on the USMLE Help book ditional textbooks. Instead, it is intended to reiterate and
series, it was well appreciated that we avoided such acciden- strengthen your already existing knowledge ( rom your train-
tal cheating by separating one case rom the other. Taking the ing) and to f ll potential gaps in your knowledge.
positive experience rom that book series, we decided that However, in a collaborative e ort with the Journal of
each case in this series should consist o two pages: page 1 Radiology Case Reports and the international radiology

ix
x Series Preface

community Radiolopolis, we have developed an online this series is structured on di f culty levels so that the series
repository with more comprehensive in ormation or each also becomes use ul to an audience with limited experience
case, such as demographics, discussions, more image exam- in radiology (nonradiologist physicians or medical students)
ples, interactive image stacks with scroll, a window/level up to subspecialty-trained radiologists who are preparing or
eature, and other interactive eatures that almost resemble their CAQs or who just want to re resh their knowledge and
a workstation. In addition, we are planning ahead toward use this series as a re erence.
the new Radiology Boards ormat and are providing rapid I am delighted to have such an excellent team o US and
ire online sessions and mock examinations that use the international educators as authors on this innovative book
cases in the print version. Each case in the print version is series. These authors have been thoroughly evaluated and
crosslinked to the online version using a case ID. The case selected based on their excellent contributions to the Journal
ID number appears to the right o the diagnosis heading at of Radiology Case Reports, the Radiolopolis community, and
the top o the second page o each case. Each case can be other academic and scientif c accomplishments.
accessed using the case ID number at the ollowing web It brings especially personal satis action to me that this
site: www.radiologycasereviews.com/case/ID, in which project has enabled each author to be involved in the over-
“ID” represents the case ID number. I you have any ques- all decision-making process and improvements regarding
tions regarding this web site, please e-mail the series editor the print and online content. This makes each participant not
directly at roland@talanow.in o. only an author but also part o a great radiology product that
I am particularly proud o such a symbiotic endeavor o will appeal to many readers.
print and interactive online education and I am grate ul to Finally, I hope you will experience this case review book
McGraw-Hill or giving me and the authors the opportunity as it is intended to be: a quick, pertinent, “get to the point”
to provide such a unique and innovative method o radiology radiology case review that provides essential in ormation or
education, which, in my opinion, may be a trendsetter. the radiology boards in the shortest time available, which, in
The primary audience o this book series is the radiol- the end, is crucial or preparation or the boards.
ogy resident, particularly the resident in the inal year who
is preparing or the radiology boards. However, each book in Roland Talanow, MD, PhD
Preface

“Whatever good things we build end up building us.”


—Jim Rohn

C
ountless hours upon hours were poured into the writ- abnormal indings with the book cases that demonstrate a
ing and image editing by my coauthor and me, which classic appearance o pathology, when possible. While the
we hope ultimately bene its you, the reader. And yet pure randomness and potpourri style o the prior board or-
the experience in the building o this book has already great- mat is lost, we believe the systematic organization by anat-
ly benef ted me as an educator in a f eld that I am passionate omy will help solidi y learning not by illing in holes rom
about: neuroradiology. Those o you amiliar with teaching random chance, but by supporting anatomy-based learn-
know that the teacher either gains or solidi ies knowledge ing which will in turn improve per ormance on the newer
while preparing to teach. This has certainly been the case in boards’ ormat. It is our hope that this book is use ul or both
writing this book or me. trainees just learning neuroradiology and more experienced
We were ambitious or this book in that we did not want practitioners.
to only o er the usual ormat or a case review book. In Finally, I would like to thank Roland Talanow or the
addition to neuroradiology cases with probing questions and opportunity to author this book, and his creation o an e f -
detailed but concise answers organized in easy, medium, and cient and easy-to-use authoring system; Rocky Saenz, my
hard categories, we also wanted a practical re erence book coauthor, or his shared dedication and input, who worked
that showed a wide variety o cases organized by anatomy in the trenches with me despite being a whole state away;
with practical pearls that help summarize the salient points. and Nucharin “Tanya” Supakul or her hard work and help
Most re erence books are organized such that a radiologist in creating case material, particularly her desire to educate
must already have a relatively accurate di erential diagnosis in pediatric neuroradiology. Most o all, I give much thanks
when considering abnormal imaging f ndings. The re erence and love to my wi e, Angel, or her support and care o our
is then checked against the radiologist’s best educated guess young children while I was sequestered in ront o a com-
or the top pathologies in the di erential, requiring signif - puter typing away like a madman. Without her this would not
cant preexisting knowledge and experience. In contrast, by have been possible.
using the index, and also because the cases are organized I hope you f nd this book use ul and a treasure.
anatomically, it is our hope that this book can be a help ul,
e f cient tool at the workstation by comparing the real-time Chang Yueh Ho, MD

xi
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RADIOLOGY
CASE REVIEW SERIES | Brain Imaging
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43-year-old male with progressive ataxia, and swallowing and balance
di culty

1. What are the described MRI f ndings or this


disease?

2. What are the di erential diagnoses or this


f nding?

3. What is the cause o hot cross bun sign?

4. What is a pathologic hallmark o multisystem


atrophy?

5. What is the clinical presentation or this


disease?
1
Olivopontocerebellar atrophy Case 1 (3159)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Sagittal T1 shows attening Coronal T2 shows prominence Axial T2 shows middle cerebellar Axial T2 image through the
of the pons (white arrow) and of the cerebellar folia from peduncle atrophy (black arrows) medulla shows decreased
enlargement of the vermian folia di use atrophy (white arrows). and cruciate hyperintensity of the prominence of the olivary
(red arrow) from pontine and transverse pontocerebellar bers nuclei (white arrows) from
cerebellar atrophy. (red arrows) with sparing of the atrophy.
corticospinal tract in the “hot cross
bun” sign. There is decreased signal
of the dentate nuclei (blue arrow)
Answers
described with multisystem
1. Cerebellar, pontine, and olivary atrophy, as well as atrophy. Pontine atrophy with
putaminal atrophy and the “hot cross bun” sign, can associated enlarged fourth
be seen in some orms o olivopontocerebellar atrophy ventricle is again noted.
(OPCA). “Hot cross bun” sign is nonspecif c and can
be seen in other neurodegenerative diseases. Putaminal
atrophy is seen in multisystem atrophy (MSA), which Pearls
encompasses some sporadic orms o OPCA. • OPCA is a heterogeneous group o disorders that
2. Spinocerebellar ataxias, alcohol abuse, congenital include sporadic orms primarily classif ed as
pontocerebellar hypoplasia, hyperthermia, and multisystem atrophy and inherited amilial orms.
paraneoplastic syndromes can cause cerebellar • All OPCAs have similar f ndings o cerebellar and
atrophy with the addition o brainstem atrophy in brainstem atrophy, including the pons and medullary
neurodegenerative disorders such as spinocerebellar olives.
ataxias and congenital pontocerebellar hypoplasias. • Sporadic OPCA classif ed in MSA can also have
cerebral and putaminal atrophy.
3. The “hot cross bun” sign is T2 hyperintensity in a • The “hot cross bun” sign o the pons is not specif c but
cruciate pattern within the atrophied pons and is thought can be seen in many neurodegenerative disorders.
to be rom degeneration o transverse pontocerebellar • No treatment is available: Most patients die o
f bers and pontine neurons as well as sparing o the bronchopneumonia rom central respiratory ailure
corticospinal tract. This f nding is nonspecif c and can within a decade.
be seen in other neurodegenerative disorders including
multisystem atrophy and spinocerebellar atrophy.
Case reports have also described this f nding in variant
Creutz eldt-Jakob disease and a patient with vasculitis Suggested Readings
presenting with parkinsonian symptoms. Naka H, Ohshita T, Murata Y, Imon Y, Mimori Y, Nakamura S.
Characteristic MRI f ndings in multiple system atrophy:
4. Multisystem atrophy demonstrates cytoplasmic
comparison o the three subtypes. Neuroradiology. 2002
inclusions o alpha-synuclein within the cytoplasm o
Mar;44(3):204-209.
oligodendroglia, neurons, and axons.
Ozawa T, Revesz T, Paviour D, et al. Di erence in MSA
5. Olivopontocerebellar atrophy, including the multisystem phenotype distribution between populations: genetics or
atrophy-C subtype, primarily presents with cerebellar environment? J Parkinsons Dis. 2012 Jul;2(1):7-18.
symptoms. Furthermore, MSA-P subtype has more Savoiardo M, Strada L, Girotti F, et al. Olivopontocerebellar
parkinsonian symptoms. Almost all MSA patients will atrophy: MR diagnosis and relationship to multisystem
eventually develop autonomic ailure. atrophy. Radiology.1990 Mar;174(3, pt 1):693-696.
2
1-year-old male with venous malformation of face

1. What is the diagnosis?

2. What are the classic imaging f ndings or this


entity?

3. What are the symptoms seen with this entity?

4. What are the various causes o cerebellar


ectopia/herniation?

5. What is the treatment or this entity when


symptomatic?

3
Chiari I malformation Case 2 (812)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Sagittal T1 demonstrates herniation of cerebellar Sagittal T2 shows herniation Axial T2 image shows syringomyelia (blue arrow)
tonsil (asterisk) at foramen magnum. Cerebellar of cerebellar tonsil (green of the spinal cord.
tonsils lie more than 5 mm below the line arrow) at foramen magnum.
between basion (blue arrow) and opisthion Syringomyelia (red arrow) of
(green arrow). the spinal cord.

Answers
1. Chiari 1 mal ormation o hindbrain is more than 5 mm Pearls
herniation o cerebellar tonsils below the oramen • Chiari 1 mal ormation is associated with an
magnum. underdeveloped posterior cranial ossa.
• Adults may have cerebellar tonsil ectopia o 5 mm.
2. CSF ow in Chiari 1 mal ormation shows decreased
• Children less than 4 years can have tonsillar
CSF ow at oramen magnum and through the vallecula
ectopia o up to 6 mm without any symptoms, i the
cerebelli. Quantitative f ndings include prolonged CSF
subarachnoid space at oramen magnum remains
systole in the prepontine cistern, increased CSF velocity
patent.
at the oramen magnum, reduced systolic velocities and
• Pointed/compressed cerebellar tonsils with e acement
duration o CSF systole just below oramen magnum, and
o retrocerebellar cistern at oramen magnum are
increased duration o CSF systole at C-2 to C-3 level.
usually symptomatic.
3. Occipital headache, swallowing dys unction, nystagmus, • Posterior inclination o dens is associated with
lower cranial nerve palsies, vertigo, and tinnitus are syringomyelia.
some o the symptoms o Chiari 1 mal ormation. • MRI o the spine is needed given the approximate
20% incidence o syrinx.
4. Various causes o cerebellar ectopia/herniation include
a small posterior ossa, chronic ventriculoperitoneal
shunts, mass e ect produced rom tumors, chronic
pseudotumor cerebri, larger hemorrhages, and Suggested Readings
intracranial hypotension.
Bunck AC, Kroeger JR, Juettner A, et al. Magnetic resonance
5. The symptomatic Chiari I patient is usually treated with 4D ow analysis o cerebrospinal uid dynamics in
posterior ossa decompression surgery. Asymptomatic Chiari I mal ormation with and without syringomyelia.
individuals are usually not treated. Eur Radiol. 2012 Sep;22(9):1860-1870.
Heiss JD, Su redini G, Smith R, et al. Pathophysiology
o persistent syringomyelia a ter decompressive
craniocervical surgery. Clinical article. J Neurosurg Spine.
2010 Dec;13(6):729-742.

4
31-month-old male with history of myelomeningocele

1. What are the typical brain MRI f ndings or


this disease?

2. What di erentiates the numbered subtypes o


this entity?

3. What morphological changes involve the


skeletal system in this disease?

4. What is the theoretic etiology o this disease?

5. What is the treatment or this disease?


5
Chiari II malformation Case 3 (3251)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Axial T2 image at the midbrain level shows gyral interdigitation Sagittal T1 image of the brain at midline shows tonsillar herniation
(white arrows) and beaked tectum (red arrow). (white arrow), e acement of the fourth ventricle (red arrow), tectal
beaking (white arrowhead), and enlarged massa intermedia (red
arrowhead). These are typical ndings of Chiari II malformation.

Axial T1 image though the ganglionic level shows falcine Sagittal T2 image of the lumbosacral spine shows dysraphic
fenestration and gyral interdigitation (white arrow). Also note posterior elements in the sacral spinal canal with herniation of
the bilateral frontal periventricular gray matter heterotopia (red the cord (white arrow). The distal neural placode is attached to a
arrows). lipoma distinguished from the subcutaneous fat by a fascial plane
(red arrow). This is termed a lipomyelomenigocele. There is also a
distal hydrosyringomyelia (white arrowhead).

6
Chiari II malformation (Cont.) Case 3 (3251)

Answers
1. Myelomeningocele, tonsillar herniation, beaked tectum, Pearls
and ourth ventricular e acement are all hallmarks o • Chiari II mal ormation is nearly 100% associated with
Chiari II mal ormation. myelomeningocele.
Additional f ndings include enlarged massa intermedia, • CSF loss theory through the neural tube de ect is
corpus callosal dysgenesis, alcine enestration and gyral thought to give rise to small posterior ossa and
interdigitation, stenogyria, and periventricular heterotopia. resulting mal ormations.
• Abnormalities include ( rom most common to least
2. Chiari I will usually only have cerebellar tonsillar common): myelomeningocele, hydrocephalus,
herniation and ourth ventricular e acement. There may small posterior ossa, tonsillar herniation, lacunar
be a spinal cord syrinx without myelomeningocele. skull, brainstem descent, cervicomedullary kinking,
Chiari III has similar brain f ndings o Chiari II with a towering cerebellum, beaked tectum, enlarged massa
cranial cervical posterior encephalocele that involves intermedia, dysgenesis o the corpus callosum, alcine
dysraphism o the C1-C2 posterior elements. enestration/interdigitating gyri, syringohydromyelia,
and nodular heterotopia.
3. Bony changes o Chiari II mal ormation include spinal
dysraphism, clival notching, enlarged oramen magnum,
and Lückenschädel.
Suggested Readings
Lückenschädel (lacunar skull) describes craniolacunia
involving both inner and outer tables due to a Geerdink N, van der Vliet T, Rotteveel JJ, Feuth T,
mesenchymal de ect, not rom increased intracranial Roeleveld N, Mullaart RA. Essential eatures o Chiari II
pressure. This typically resolves by 6 months o age. mal ormation in MR imaging: an interobserver reliability
study—part 1. Childs Nerv Syst. 2012 Jul;28(7):977-985.
4. The avored theory includes CSF leakage through a McLone DG, Naidich TP. Developmental morphology o the
myelomeningocele, leading to ourth ventricle collapse, subarachnoid space, brain vasculature, and contiguous
and posterior ossa hypoplasia. This causes the cascade structures, and the cause o the Chiari II mal ormation.
o tonsillar herniation, and towering cerebellum, AJNR Am J Neuroradiol. 1999 Oct;13(2):463-482.
inducing other brain abnormalities seen in Chiari II.
5. Folate supplementation prior to conception and or
6 weeks ollowing helps prevent neural tube de ects.
Repair o the myelomeningocele and shunting o
hydrocephalus are typically per ormed shortly a ter
birth; however, in utero repair may help lessen brain
mal ormations.

7
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9-year-old male with decreased level of consciousness and vomiting

1. What is the di erential diagnosis?

2. What imaging f ndings suggest high-grade


neoplasm?

3. What morphological f nding o the tumor


can help di erentiate this rom other ourth
ventricular tumors?

4. What is the typical mode o disease


dissemination?

5. What are some molecular subtypes and


prognosis associations?
9
Medulloblastoma Case 4 (3344)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Axial T2 image of the posterior fossa shows a heterogeneous Sagittal post contrast T1 image shows the mildly enhancing
mass lling the fourth ventricle with areas of T2 hypointensity and heterogeneous tumor, which causes hydrocephalus and
(arrowheads). Note the CSF cleft with the dorsal pons (arrow). ventricular dilatation (asterisk). Notice the CSF cleft separating the
tumor from the dorsal brainstem (arrow) but with poor separation
from the vermis, or roof of the fourth ventricle (arrowhead).

Axial DWI b = 1000 ADC map shows areas of decreased di usion


(arrows) consistent with a high-grade neoplasm.
Axial relative cerebral blood volume map from dynamic
susceptibility contrast technique shows signi cant hyperperfusion
of the tumor (arrow).

10
Medulloblastoma (Cont.) Case 4 (3344)

Answers
1. Medulloblastoma and ependymoma are classic ourth Pearls
ventricular tumors in the pediatric population. Pilocytic • Medulloblastomas are in ratentorial primitive
astrocytomas tend to be o midline and pedunculate neuroectodermal tumors (PNET) and are the most
into the ourth ventricle. Atypical teratoid rhabdoid common PNET o the brain, as well as the most
tumors are di f cult to separate rom medulloblastoma common malignant brain tumor in children.
by imaging as both are high-grade neoplasms but tend to • As with all high-grade neoplasms, there are areas o
be o midline compared to medulloblastoma. Brainstem T2 hypointensity, decreased di usion, and increased
gliomas with exophytic component into the ourth per usion rom hypercellularity and increased
ventricle could be considered but is unlikely in this case neoangiogenesis.
due to a clean margin and a small CSF cle t between the • Tumor origin is rom the medullary velum, or granular
mass and the dorsal sur ace o the brainstem. layer o the cerebellum, which explains the typical
appearance o arising rom the roo o the ourth
2. Decreased di usion and T2 signal can be seen in
ventricle, and causing obstructive hydrocephalus.
hypercellular components o tumors suggesting
• Medulloblastomas uncommonly herniate out o
high grade. High-grade tumors also tend to cause
the oramina Luschka and Magendie, which help
neoangiogenesis, which will demonstrate increased
distinguish it rom ependymoma.
cerebral blood volume on per usion imaging.
• Atypical teratoid rhabdoid tumor can appear very
3. Medulloblastomas classically arise rom the roo o the similar to medulloblastoma as both are high-grade
ourth ventricle, and are di f cult to separate rom the tumors, but may present o midline in comparison.
cerebellar vermis on imaging. In contrast, ependymomas • Spinal imaging or leptomeningeal metastasis is
generally arise rom the oor or dorsal brainstem always indicated in medulloblastoma prior to surgery,
ependymal sur ace o the ourth ventricle, and usually as up to 25% o cases present with leptomeningeal
show some herniation o the tumor through the ourth disease.
ventricular oramina.
4. Up to 25% o medulloblastomas will demonstrate CSF/
leptomeningeal dissemination at presentation. Care ul Suggested Readings
evaluation o the leptomeninges along the entire neuraxis Fruehwald-Pallamar J, Puchner SB, Rossi A, et al. Magnetic
is indicated prior to surgery and treatment. Extraneural resonance imaging spectrum o medulloblastoma.
metastasis is rare, usually involving the bone, and can be Neuroradiology. 2011 Jun;53(6):387-396.
seen in late stages o disease. Yeom KW, Mobley BC, Lober RM, et al. Distinctive MRI
5. Molecular subgroups o medulloblastoma correlate eatures o pediatric medulloblastoma subtypes. AJR Am J
with incidence o leptomeningeal dissemination and Roentgenol. 2013 Apr;200(4):895-903.
prognosis. A greater incidence o medulloblastomas
is associated with amilial cancer syndromes such as
Li-Fraumeni, basal cell nevus, Turcot, Gardner, and
Cowden. Amplif cation o the sonic the hedgehog (SHH)
protein is seen with 30% o medulloblastomas and can
involve di erent mutations. Mutations involving the SHH
pathway is associated with a desmoplastic variant as in
Gorlin syndrome as well as TP53 mutation in Li-Fraumeni
syndrome, which carries a poorer prognosis. Subgroups
with high amplif cation o the MYC proto-oncogene
has the poorest prognosis with 5-year survival at 50%
and is associated with the large cell anaplastic variant,
with requent leptomeningeal metastasis at diagnosis.
In contrast, the wingless (WNT) protein pathway is the
least common subgroup at 15% with 5-year survival
o 95%, good prognosis, and less likely to metastasize.
This subgroup is associated with Turcot syndrome.

11
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6-year-old male with headache and vomiting

1. What is the di erential diagnosis?

2. What imaging f ndings re ect a low-grade


neoplasm?

3. What is the MR spectroscopic prof le or this


lesion?

4. Where are the common locations o occurrence


or this tumor?

5. What is the prognosis o these lesions?


13
Pilocytic astrocytoma Case 5 (3346)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Axial T2 image of the posterior fossa shows a well-circumscribed Axial T1 postcontrast image demonstrates heterogeneous
mass arising from the vermis and left cerebellar hemisphere enhancement of the solid component (arrow).
with little to no adjacent edema, and mass e ect on the fourth
ventricle (white arrowhead). There is a prominent cyst (black
arrow) and relative T2 hyperintensity of the solid component
(black arrowhead).

Axial relative cerebral blood volume map from dynamic


susceptibility contrast perfusion shows no increased perfusion in
the tumor (arrow).

Axial DWI b = 1000 image shows no restricted di usion of the


tumor (arrow).

14
Pilocytic astrocytoma (Cont.) Case 5 (3346)

4. In decreasing order o requency, pilocytic astrocytomas


most commonly arise rom the cerebellum,
supratentorial structures around the third ventricle, and
optic apparatus/hypothalamus. Rarely do they arise rom
peripheral lobar distributions.
5. The prognosis or pilocytic astrocytomas is excellent
with greater than 95% survival in 10 years, regardless o
complete surgical excision or chemoradiation.

Pearls
• Pilocytic astrocytomas are benign WHO grade I
tumors with excellent prognosis with or without
therapy.
• The classic cyst with an enhancing mural nodule
appearance can be seen in hal o cases.
• Solid portions o the tumor are usually T2 hyperintense
and CT hypodense without decreased di usion.
• Per usion imaging is usually hypoper used, although
Axial fractional anisotropy map shows the mass displacing
contrast leakage and areas o increased vascularity can
cerebellar white matter tracts (arrowheads) rather than invading
the tracts suggestive of low-grade neoplasm.
cause some increase in CBV.
• MR spectroscopy is paradoxical in that there is
elevated choline, decreased NAA, and the presence o
Answers lactate, although ratios o choline to NAA are lower
1. Pilocytic astrocytoma and hemangioblastoma are classic than or malignant tumors.
di erentials or the cyst with mural enhancing nodule • Most common locations include the cerebellum
in the posterior ossa, although hemangioblastomas > around the third ventricle > optic pathway and
are seen in adults associated with Von-Hippel Lindau hypothalamus > brainstem.
disease. Medulloblastoma, ependymoma, and choroid
plexus tumors should primarily arise rom the ourth
ventricle and do not typically have the cyst and nodule Suggested Readings
appearance. Furthermore, the tumor demonstrates
Arai K, Sato N, Aoki J, et al. MR signal o the solid portion
low-grade characteristics, virtually excluding
o pilocytic astrocytoma on T2-weighted images: is
medulloblastoma rom the di erential.
it use ul or di erentiation rom medulloblastoma?
2. Low-grade neoplasms generally have lower cellularity Neuroradiology. 2006 Apr;48(4):233-237.
than high-grade neoplasms and demonstrate increased T2 Ho CY, Cardinal JS, Kamer AP, Kralik SF. Relative cerebral
signal and increased di usion. Decreased per usion can blood volume rom dynamic susceptibility contrast
also suggest low-grade neoplasm. The presence o cysts per usion in the grading o pediatric primary brain tumors.
can be seen in both high- and low-grade neoplasms. Neuroradiology. 2015 Mar;57(3):299-306.
Koeller KK, Rushing EJ. From the archives o the AFIP:
3. MR spectroscopy or pilocytic astrocytomas can be
pilocytic astrocytoma: radiologic-pathologic correlation.
paradoxical as a malignant tumor prof le o decreased
Radiographics. 2006 Apr;24(6):1693-1708.
NAA, elevated choline, and the presence o lactate can
be seen. However, when compared to ependymomas
and medulloblastomas, the ratio o choline to NAA is
increased or higher-grade tumors.

15
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3-year-old male with vomiting

1. What is the di erential diagnosis?

2. What is a morphologic f nding o this tumor


that can help distinguish it rom other ourth
ventricular tumors?

3. What syndrome has an increased incidence o


this tumor?

4. What is the prognosis or this disease?

5. What is the most common location or this


tumor in children?

17
Infratentorial ependymoma (anaplastic) Case 6 (3363)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Axial CT image of the Axial T2 image of the posterior Sagittal T1 postcontrast shows Axial DWI ADC map shows
posterior fossa shows a fossa shows a heterogenous a heterogeneous enhancing areas of decreased di usion
hyperdense mass in the mass centered in the fourth mass in the fourth ventricle suggesting a higher-grade
fourth ventricle (arrow). ventricle with herniation (arrow) causing hydrocephalus tumor (arrow).
through the right foramen of with distention of the cerebral
Luschka (arrow). This is a typical aqueduct (arrowhead).
nding for an ependymoma.
5. Ependymomas most commonly occur in the posterior ossa
in younger children and less commonly supratentorial in
a periventricular location in older children. In adults, the
spinal cord is the most common location.

Pearls
• Ependymomas are tumors arising rom ependymal cells
or rests commonly occurring in the ourth ventricle.
• Supratentorial ependymomas are usually
periventricular, arising rom ependymal rests.
• Ependymomas commonly have calcif cation, cysts,
Cerebral blood volume map from dynamic susceptibility contrast and hemorrhage.
perfusion imaging shows a hyperperfused portion of the tumor • In ratentorial ependymomas are usually located within
(arrow) consistent with high-grade anaplastic astrocytoma. the ourth ventricle and have a tendency to squeeze out
o the oraminas Luschka and Magendie.
Answers • DWI and per usion imaging can help distinguish
higher-grade anaplastic varieties rom low-grade
1. The di erential o ourth ventricular masses classic ependymomas
include ependymoma, medulloblastoma (primitive • Spinal imaging prior to surgery is important to
neuroectodermal tumor), and atypical teratoid rhabdoid evaluate or drop metastasis.
tumor.
2. Although not specif c, ependymomas more
commonly have calcif cation and hemorrhage than Suggested Readings
medulloblastomas and pilocytic astrocytomas. Cystic
Rumboldt Z, Camacho DL, Lake D, Welsh CT, Castillo
change can be commonly seen with all three tumors,
M. Apparent di usion coe f cients or di erentiation o
especially with pilocytic astrocytomas. Squeezing out o
cerebellar tumors in children. AJNR Am J Neuroradiol.
the oramina is more typical o ependymomas.
2008 Jan;27(6):1362-1369.
3. There is an increased incidence o ependymomas in Spoto GP, Press GA, Hesselink JR, Solomon M. Intracranial
neurof bromatosis type 2. ependymoma and subependymoma: MR mani estations.
4. Despite being a predominantly low-grade tumor, AJNR Am J Neuroradiol. 1990 May;11(1):83-91.
ependymomas have a generally poor prognosis with only
50%-60% 5-year survival rate.
18
3-year-old female with unsteady gait

1. What is the di erential diagnosis?

2. What are the typical MRI f ndings or this


entity?

3. What is the typical histologic cell type o this


tumor?

4. Which type o brainstem tumors have good


prognosis?

5. What is the treatment or this disease?

19
Di use pontine glioma Case 7 (3370)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Axial T2 image shows di use Sagittal T1 postcontrast image Axial b = 1000 DWI image Axial CBV map from
expansile enlargement of shows no enhancing foci within the shows no restricted di usion dynamic susceptibility
the pons with increased pontine mass. There is obstructive of the pontine mass (arrow). contrast perfusion imaging
T2 signal (arrow). There is hydrocephalus with e acement of shows no areas of increased
encasement of the basilar the cerebral aqueduct (arrow) causing perfusion. The linear
artery (arrowhead). enlargement of the third and lateral structure of increased
ventricles. perfusion corresponds to
a vessel (arrowhead).

Answers
1. The di erential or di use T2 hyperintense swelling Pearls
o the pons includes di use pontine glioma, acute • Di use pontine gliomas (DPG) are biologically
disseminated encephalomyelitis, and osmotic aggressive astrocytomas with poor prognosis in
demyelination. children; median survival is less than 1 year despite
therapy.
2. Typical MRI f ndings o di use pontine glioma include
• Other ocal brainstem gliomas have an indolent course
di use T2 hyperintensity o the pons with mass-like
and generally good prognosis such as the exophytic
expansion, and no enhancement. There is usually no
pilocytic astrocytoma, tectal plate glioma, and
uni orm restricted di usion or increased per usion.
brainstem tumors associated with NF1.
When signif cantly large, the mass may encase the
• Neuroimaging shows di use pontine swelling and
basilar artery and cause obstructive hydrocephalus on the
uni orm T2 hyperintensity.
ourth ventricle.
• Enhancement or hemorrhage is atypical.
3. Typically di use pontine gliomas have been classif ed as • Encasement o the basilar artery may be seen.
f brillary astrocytomas, with WHO grading varying rom
II to IV; however, all DPGs have poor prognosis.
4. All ocal, exophytic, tectal, or cervicomedullary Suggested Readings
brainstem tumors have good prognosis approaching
Löbel U, Sedlacik J, Reddick WE, et al. Quantitative
100% long-term survival, although some have signif cant
di usion-weighted and dynamic susceptibility-weighted
morbidity. This includes tumors associated with NF1.
contrast-enhanced per usion MR imaging analysis o
Di use pontine gliomas are the exception with poor
T2 hypointense lesion components in pediatric di use
prognosis and median survival o less than 1 year.
intrinsic pontine glioma. AJNR Am J Neuroradiol. 2011
5. Corticosteroids are indicated to help with tumoral edema Feb;32(2):315-322.
and symptoms in di use pontine glioma to improve Sedlacik J, Winchell A, Kocak M, Loe er RB, Broniscer A,
quality o li e. Radiation therapy can prolong survival Hillenbrand CM. MR imaging assessment o tumor
by a ew months. Chemotherapy has not been shown to per usion and 3D segmented volume at baseline, during
be help ul. Surgery does not play a signif cant treatment treatment, and at tumor progression in children with
role due to the location o the tumor. newly diagnosed di use intrinsic pontine glioma.
AJNR Am J Neuroradiol. 2013 Jul;34(7):1450-1455.

20
15-year-old male with history of headache and dizziness. Recent history
of trauma to his neck

1. What is the most common cause o this


f nding?

2. I no clinical history o trauma, what is the


possibility etiology?

3. What are possible clinical presentations?

4. What f nding determines treatment options?

5. What is a medical treatment option in


extracranial vertebral artery dissection?
21
Vertebral artery dissection Case 8 (3324)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Axial FLAIR image demonstrates hyperintensity in the left V4 Axial FLAIR image demonstrates a focal area of FLAIR hyperintensity
vertebral artery (white arrow). involving the left cerebellar hemisphere in the posterior inferior
cerebellar artery distribution (white arrowheads).

DWI image demonstrates restricted di usion in the left cerebellar


hemisphere in the PICA distribution (white arrowheads), Axial postcontrast CT angiography shows occlusion of the distal
consistent with acute infarct. V3, proximal V4 junction with tapering (arrowheads), indicating
dissection in this teenager with a history of trauma.

22
Vertebral artery dissection (Cont.) Case 8 (3324)

Pearls
• Blunt trauma is the most common cause o vertebral
artery dissection.
• Spontaneous vertebral artery dissection may be
associated with underlying connective tissue disease
and f bromuscular dysplasia.
• It is important to determine whether the dissection
extends into the intracranial V4 segment, due to
di erent treatment options and prognosis.
• Intracranial dissections are prone to rupture with
subarachnoid hemorrhage, with signif cant increase in
mortality.
• CT/CTA is o ten the f rst imaging modality,
demonstrating posterior ossa ischemia, subarachnoid
hemorrhage, occluded vertebral artery, or mural
thrombus.
• MRI/MRA is more sensitive in small posterior ossa
ischemia, and also able to demonstrate intraluminal
thrombus and intraluminal hemorrhage.
Sagittal MIP CTA image demonstrates smooth tapering of the left • Treatment or extracranial dissection is antiplatelet
V3 vertebral artery, consistent with dissection (arrowheads). therapy to prevent artery-to-artery embolization and
posterior circulation in arcts.
• Intracranial dissection, especially with subarachnoid
Answers
hemorrhage or large in arcts, requires endovascular
1. Blunt trauma is the most common cause o vertebral treatment or surgery.
artery dissection.
2. Spontaneous vertebral artery dissection may be associated
with f bromuscular dysplasia (15%) or underlying Suggested Readings
connective tissue disease (Ehlers-Danlos syndrome,
Rodallec MH, Marteau V, Gerber S, Desmottes L, Zins M.
Mar an syndrome, autosomal dominant polycystic kidney
Craniocervical arterial dissection: spectrum o imaging
disease, and osteogenesis imper ecta type 1).
f ndings and di erential diagnosis. Radiographics. 2008
3. Most requent clinical mani estation o vertebral artery Oct;28(6):1711-1728.
dissection is headache or neck pain accompanied or Vertinsky AT, Schwartz NE, Fischbein NJ, Rosenberg J,
ollowed by posterior circulation ischemia (57%-84% Albers GW, Zaharchuk G. Comparison o multidetector
o patients). Rare clinical mani estations include CT angiography and MR imaging o cervical
isolated head or neck pain, cervical spinal cord artery dissection. AJNR Am J Neuroradiol. 2008
ischemia, cervical nerve root impingement, and Horner Oct;29(9):1753-1760.
syndrome.
4. Patients with intracranial involvement o vertebral artery
dissection should be treated by surgery or endovascular
treatment, rather than antiplatelet therapy, especially
with subarachnoid hemorrhage or large in arcts.
5. Extracranial vertebral artery dissection can be treated
with antiplatelet agents to prevent artery-to-artery
embolization and posterior circulation in arcts.

23
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53-year-old male with a severe headache and visual changes followed by
a loss of consciousness

1. Where is the abnormality located?

2. What is the arterial distribution involved with


this pattern?

3. What is the di erential diagnosis?

4. What are risk actors or this disease?

5. What is the treatment or this entity?

25
Basilar artery occlusion Case 9 (3473)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Axial CT shows hypodensity of the pons, left temporal lobe, Axial CT image shows hypodensity of the bilateral medial thalami
and cerebellum. There is increased density of the basilar artery and left occipital temporal lobe from posterior circulation infarct
(arrowhead), which supplies these vascular territories. (asterisks).

Answers
1. The abnormality involves the cerebellum, thalami, le t Pearls
occipital lobe, le t temporal lobe, and portions o the • Basilar artery occlusion results in in arction o the
brainstem. cerebellum, brainstem, posterior temporal lobes,
portions o the internal capsules, thalami, and occipital
2. The abnormality involves the basilar artery territory.
lobes.
Small portions o the le t cerebellar hemisphere remain
• This is a neurointerventional emergency because o
una ected likely due to the le t posterior in erior
in arction o the brainstem.
cerebellar artery, which originates rom the vertebral
• High density within the basilar artery is a nonspecif c
artery.
sign or thrombosis.
3. The di erential diagnosis includes basilar occlusion, • Top o the basilar syndrome occurs when only the
artery o Percheron in arct, and dural venous sinus distal basilar artery is occluded, which results in
thrombosis. The artery o Percheron is a variant o the in arction o the thalami, posterior limbs o internal
posterior circulation where a solitary trunk (Percheron) capsules, and midbrain.
supplies the paramedian thalami and rostral midbrain. • CTA is help ul to visualize the thrombus.
• Mortality o basilar artery thrombosis is two to three
4. Basilar artery occlusion risk actors include
times higher than internal carotid artery occlusion.
atherosclerosis, hyperlipidemia, propagation o clot
secondary to dissection, and meningitis.
5. In the acute setting, mechanical thrombectomy has Suggested Readings
shown success with regard to improving mortality and Cormier PJ, Long ER, Russell EJ. MR imaging o posterior
overall outcome. ossa in arctions: vascular territories and clinical
correlates. Radiographics. 1992 Nov;12(6):1079-1096.
Kostanian V, Cramer SC. Artery o Percheron thrombolysis.
AJNR Am J Neuroradiol. 2007 May;28(5):870-871.
Puetz V, Sylaja PN, Hill MD, et al. CT angiography source
images predict f nal in arct extent in patients with
basilar artery occlusion. AJNR Am J Neuroradiol. 2009
Nov;30(10):1877-1883.
26
70-year-old male with headache, vertigo, and gait disturbance

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the etiology or this f nding?

5. What is the treatment or this entity?

27
PICA infarct Case 10 (3526)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Axial T2 image shows swelling Coronal FLAIR image shows the high signal is seen Axial T1 postcontrast shows linear
involving the inferior left cerebellar involving the lower left cerebellar hemisphere sulcal enhancement of the left
hemisphere and vermis (arrow) with (arrow) with associated regional mass e ect. cerebellar hemisphere (arrows).
mass e ect on the medulla. This is likely from “luxury perfusion.”

4. PICA in arction is typically rom atherosclerotic occlusion.


5. The treatment or this entity is typically supportive
care, but consideration can be given to thrombolytic
therapy with progressive basilar involvement. Surgical
intervention may be necessary in cases o hydrocephalus
or upward transtentorial herniation with resection o
cerebellar tissue.

Pearls
• PICA supplies the in erior cerebellar hemispheres,
Axial DWI b-1000 and ADC map demonstrate restricted di usion
in erior vermis, tonsils, and lateral medulla.
(green arrow) with corresponding low signal on ADC map (blue
arrow) within the lower left cerebellar hemisphere.
• The ourth ventricle may be e aced secondary to mass
e ect; upward transtentorial herniation may occur.
• PICA in arcts are the most common cerebellar in arcts
Answers associated with hemorrhagic trans ormation.
1. The abnormality is located within the le t cerebellar • The most common etiology in PICA in arcts is
hemisphere. thrombosis related to atherosclerotic disease.
• Lateral medullary (Wallenberg) syndrome involves
2. The classic imaging f ndings or this entity on CT include ipsilateral Horner syndrome, ipsilateral ace loss o
obscuration o the cerebellar olia and low density within temperature and sensation with contralateral body loss
the a ected cerebellar parenchyma. On MR, there is high o temperature and sensation.
signal on the T2 images with corresponding T1 low signal
ollowing the signal o edema. Specif cally, di usion-
weighted imaging (DWI) is the most sensitive and Suggested Readings
specif c MRI sequence or detecting acute in arcts, which
Cano LM, Cardona P, Quesada H, Mora P, Rubio F.
will demonstrate increased signal with corresponding
[Cerebellar in arction: prognosis and complications o
decreased signal on ADC (“restricted di usion”).
vascular territories]. Neurologia. 2012 Nov;27(6):330-335.
3. The di erential diagnosis includes cerebellar in arction, Kim MJ, Chung J, Kim SL, et al. Stenting rom the vertebral
metastasis, cerebellitis, astrocytoma, and dysplastic artery to the posterior in erior cerebellar artery. AJNR Am
gangliocytoma. J Neuroradiol. 2012 Feb;33(2):348-352.

28
34-year-old male with headaches, status post fusiform aneurysm coiling,
and vessel sacri ce

1. What is the di erential diagnosis?

2. What are the typical MRI f ndings or this


entity?

3. What structures are supplied by this artery?

4. What are the clinical symptoms or this


f nding?

5. What symptom helps di erentiate this arterial


territory in arct rom the more common PICA
in arct?
29
AICA infarct Case 11 (3585)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Axial FLAIR image shows increased Axial DWI b = 1000 image shows AP view of left vertebral AP view of left vertebral
T2 signal of the left anterior inferior di usion restriction of the left artery injection DSA shows a artery injection DSA
cerebellum extending to the anterior inferior cerebellum fusiform aneurysm of the left shows coiling of the left
left middle cerebellar peduncle and middle cerebellar peduncle AICA (arrow). AICA aneurysm with
(asterisk) in the expected left AICA (asterisk). sacri ce of the left AICA
territory. (arrow).

Answers
1. The di erential or mass-like swelling o the anterior Pearls
in erior cerebellar hemisphere includes cerebellitis, • AICA supplies the anterior in erior cerebellar
neoplasm, and acute in arct o the anterior in erior hemisphere along the petrosal sur ace.
cerebellar artery (AICA) territory. • Middle cerebellar peduncle, in erior lateral pons and
upper medulla, and ourth ventricular choroid plexus
2. Typical MRI f ndings or acute in arct include T2
are also supplied by AICA.
hyperintensity, T1 hypointensity, and DWI increased
• Labyrinthine branch supplies the inner ear, with
signal, and decreased ADC corresponding with cytotoxic
hearing loss helping clinically di erentiate rom PICA
edema.
in arct.
3. The AICA supplies the anterior in erior cerebellum • Variations in AICA are common, including common
along the petrosal margin, the middle cerebellar PICA/AICA trunk.
peduncle, in erolateral pons and upper medulla, and
ourth ventricular choroid plexus at the oramen o
Luschka. Labyrinthine branch o the AICA supplies the
Suggested Reading
vestibulocochlear structures and acial nerve.
Chang HM, Linn FH, Caplan LR. Bilateral anterior in erior
4. Patients with AICA in arct present with dysarthria, cerebellar artery territory in arcts. J Neuroimaging. 1998
Horner syndrome, and ipsilateral limb ataxia. Inner ear Jan;8(1):42-44.
symptoms are also noted such as hearing loss, vertigo,
and tinnitus.
5. Hearing loss is a clinical symptom that can di erentiate
an AICA rom a PICA in arct due to labyrinthine branch
supply to the inner ear rom the AICA.

30
55-year-old male with acute dysarthria and right limb ataxia

1. What is the di erential diagnosis?

2. What are the typical MRI f ndings or this


entity?

3. What structures are supplied by this artery?

4. What are the clinical symptoms or this


f nding?

5. What is Horner syndrome?


31
Superior cerebellar artery infarction Case 12 (3586)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Axial FLAIR image shows T2 Axial DWI b = 1000 image shows Axial CT angiography shows 3D reformation of the
hyperintensity and swelling of di usion restriction of the right severe stenosis of the proximal posterior arterial circulation
the right superior cerebellum superior cerebellum (asterisk) right superior cerebellar artery from CT angiography
(asterisk). and dorsal pons (arrowhead). (arrowhead) with associated shows severe stenosis of
hypodensity of the cerebellum the right superior cerebellar
and dorsal pons from acute artery (white arrow). There
infarct (asterisk). is incidental fetal origin of
the right posterior cerebral
artery (red arrow) and normal
variant hypoplasia of the
distal right vertebral artery
after the origin of the right
PICA (arrowhead).

Answers
1. The di erential or mass-like swelling o the superior Pearls
cerebellum includes cerebellitis, traumatic edema, • Superior cerebellar artery (SCA) supplies the superior
neoplasm, and superior cerebellar artery (SCA) in arct. cerebellum and dorsal lateral pons.
• Clinical symptoms include ataxia, dysarthria, and
2. Typical MRI f ndings or acute in arct include
Horner syndrome.
T2 hyperintensity, T1 hypointensity, and DWI
• Isolated SCA in arct is rare and typically associated
hyperintensity correlating with decreased ADC rom
with basilar tip in arction.
cytotoxic edema.
3. The SCA supplies the superior cerebellum and dorsal
lateral pons.
Suggested Readings
4. Clinical presentation o SCA in arction includes Cano LM, Cardona P, Quesada H, Mora P, Rubio
dysarthria, ipsilateral ataxia, and Horner syndrome. F.[Cerebellar in arction: prognosis and complications o
5. Horner syndrome includes ptosis (drooping eyelid), vascular territories]. Neurologia. 2012 Nov;27(6):330-335.
miosis (constricted pupils), and anhidrosis (ipsilateral Cho TH, Berthezene Y, Mechtou L, Derex L, Nighoghossian
decreased sweating) rom sympathetic dys unction. N. Evolving basilar artery stenosis with watershed
ischemia. J Neuroimaging. 2013 Sep.

32
51-year-old female with slurred speech, upper extremity weakness,
dysphagia, and shortness of breath

1. What is the structure demonstrating pathology


in these images?

2. Which sequence is the most specif c or T2


hyperintensity along this tract?

3. What is the method o inheritance in this disease?

4. What are the clinical presentations o this disease?

5. What is the prognosis or this disease?


33
Amyotrophic lateral sclerosis Case 13 (3153)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial FLAIR image at the level of the corona radiata shows Axial FLAIR image at the ganglionic level shows hyperintensity of
hyperintensity of the corticospinal tract in the subcortical white the posterior limb of the internal capsule (white arrows).
matter of the primary motor cortex (white arrows) and extending
to the corona radiata (red arrows).

Axial T2 image of the superior midbrain shows symmetric Axial T2 image of the pons shows corticospinal hyperintensity
hyperintensity of the corticospinal tract in the cerebral peduncles (white arrows).
(white arrows).

34
Amyotrophic lateral sclerosis (Cont.) Case 13 (3153)

Axial T2 image at the medulla shows hyperintensity of the Axial FLAIR of the upper cervical cord shows hyperintensity of the
pyramids (white arrows). lateral corticospinal tract (white arrows) and anterior corticospinal
tract (red arrow).

Answers 5. There is no current e ective treatment or ALS, with all


1. The corticospinal tract begins superiorly at the motor and patient eventually progressing and deteriorating. Some
primary proprioception cortex, through the central corona patients have rapid progression to death within a ew
radiata, posterior limb o the internal capsule, cerebral years while others can live or several decades.
peduncles, pons, pyramid o the medulla, and into the
lateral and anterior portions o the spinal cord. The
corticobulbar tract runs with the corticospinal tract but is
Pearls
situated primarily in the genu o the internal capsule and
more medial portions o the cerebral peduncle, separating • T2 hyperintensity o the corticalspinal tract is typical
rom the corticospinal tract to innervate cranial nerve o ALS.
nuclei. The corticospinal tract is involved in ALS. • FLAIR imaging is sensitive but not specif c as there
is normal FLAIR hyperintensity o the corticalspinal
2. Although T1 signal changes have been reported in ALS, tract especially on 3T.
proton density is the most specif c with regard to T2 • DWI is nonspecif c with normal anisotropy o the
hyperintense signal. Spin echo T2 is also more specif c corticalspinal tract.
than FLAIR hyperintensity or DWI decreased di usion • T2 and proton density are more specif c or pathology.
as hyperintensity on FLAIR and DWI can be seen in
normal individuals especially on 3T imaging.
3. Although the majority o ALS is sporadic, most o the Suggested Readings
genetic mutations have been described in amilial orms
on the ALS1 gene (Cu/Zn superoxide dismutase) located Agosta F, Chiò A, Cosottini M, et al. The present and the
on 21q. A rare juvenile orm is associated with the ALS2 uture o neuroimaging in amyotrophic lateral sclerosis.
gene on 2q. AJNR Am J Neuroradiol. 2010 Nov;31(10):1769-1777.
da Rocha AJ, Maia AC, Valério BC. Corticospinal tract MR
4. ALS classically presents with both upper motor neuron signal-intensity pseudonormalization on magnetization
(hyperre exia, spasticity, and Babinski) and lower motor trans er contrast imaging: a potential pit all in the
neuron (hypore exia, asiculations, weakness, and interpretation o the advanced compromise o upper motor
atrophy). Variations include bulbar signs (dysphagia and neurons in amyotrophic lateral sclerosis. AJNR Am J
dysarthria) as well as hypoxia and arrhythmia, which have Neuroradiol. 2012 May;33(5):E79-E80.
a more rapid deterioration rom brain stem involvement.
35
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19-year-old male with medically refractory complex partial seizures

1. What is the di erential diagnosis?

2. What are associated secondary f ndings o this


entity?

3. What are typical MRI f ndings o this entity?

4. What is the likely etiology o this entity?

5. What is the treatment or this disease?


37
Mesial temporal sclerosis Case 14 (3506)
Case ranking/dif culty: Category: Intra-axial supratentorial

Coronal STIR image through the body Coronal FLAIR image shows increased Axial T1 SPGR reformatted obliquely through
of the hippocampi shows atrophy of signal of the smaller left hippocampus the hippocampi shows left hippocampal
the left hippocampus (arrow) with (arrow). atrophy and enlargement of the left temporal
associated enlargement of the temporal horn (arrow).
horn (arrowhead).

Answers
1. The di erential diagnosis or increased T2 signal o Pearls
the hippocampus includes status epilepticus, herpes • Most common cause o complex partial seizures in
encephalitis, cortical dysplasia, and mesial temporal young adults.
sclerosis (MTS). Volume loss is primarily seen with • Coronal FLAIR and T2 imaging increase sensitivity.
MTS. • Volumetrics increase sensitivity.
• 20% bilateral.
2. Associated secondary f ndings o MTS include ex-vacuo
• Hippocampal atrophy, increased T2, and loss o
enlargement o the ipsilateral temporal horn and choroid
internal architecture.
f ssure with atrophy o the ipsilateral mammillary body
• Secondary signs: ipsilateral temporal horn and choroid
and ornix.
f ssure dilation, ipsilateral ornix, and mammillary
3. MTS typically shows decreased size o the hippocampus body atrophy.
with increased T2 signal rom sclerosis and loss o the
internal architecture. 20% o MTS is bilateral.
4. There is lack o consensus whether MTS is caused by Suggested Readings
developmental actors or is acquired. The “two-hit” theory Coan AC, Kubota B, Bergo FP, Campos BM, Cendes F. 3T
suggests that any primary insult to the hippocampus MRI quantif cation o hippocampal volume and signal
(in ection, seizures, ischemia) with the predisposition o in mesial temporal lobe epilepsy improves detection o
genetics or other developmental anomalies (associated hippocampal sclerosis. AJNR Am J Neuroradiol. 2014
tumors) is required to develop MTS. Jan;35(1):77-83.
Howe KL, Dimitri D, Heyn C, Kiehl TR, Mikulis D, Valiante
5. MTS is the most common f nding or temporal
T. Histologically conf rmed hippocampal structural
lobectomy or complex partial seizures. Surgery is
eatures revealed by 3T MR imaging: potential to increase
indicated or medically re ractory seizures or intolerable
diagnostic specif city o mesial temporal sclerosis. AJNR
side e ects o the medication.
Am J Neuroradiol. 2010 Oct;31(9):1682-1689.

38
47-year-old male with altered mental status

1. What is the most common cause o accidental


toxic exposure?

2. What CNS structures are most commonly


involved?

3. What is the pathophysiology o this disease?

4. What is the di erential diagnosis o this f nding?

5. What is the treatment or this disease?


39
Carbon monoxide poisoning Case 15 (3510)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image shows increased Axial FLAIR image at the level of the Axial DWI b = 1000 shows bright signal
symmetric T2 hyperintensity of the basal ganglia shows increased T2 of the bilateral globi pallidi (arrows).
bilateral globi pallidi (arrows). signal of the globi pallidi (arrows).

4. The di erential or bilateral, symmetric globi pallidi


cytotoxic edema includes toxic metabolic processes
such as carbon monoxide poisoning and mitochondrial
disease such as Leigh disease. Other causes o anoxic
injury include drowning. Creutz eldt-Jakob can also
show bilateral deep gray nuclei restricted di usion.
5. Hyperbaric oxygen or 100% oxygen i hyperbaric
chamber is not available within the f rst 6 hours may
reduce neuropsychiatric sequelae.

Pearls
• Carbon monoxide poisoning typically occurs in
winter months and is the most common nonaccidental
toxicity in Europe and North America.
• The most common CNS site o abnormality is the
globi pallidi ollowed by deep cerebral white matter in
Axial ADC map shows dark signal of the bilateral globi pallidi a bilateral symmetric distribution.
(arrows) consistent with di usion restriction and cytotoxic edema. • Involvement o the deep gray nuclei, ornix, and
hippocampi can also be seen.
• Acute lesions demonstrate T2 hyperintensity and
Answers restricted di usion, with variable enhancement.
1. Carbon monoxide poisoning is the most common cause • A ected parenchyma progress to cystic
o accidental toxicity, especially in winter months in encephalomalacia in the chronic stage.
North America and Europe.
2. The globi pallidi are most commonly involved in a Suggested Readings
symmetric distribution with carbon monoxide poisoning Beppu T. The role o MR imaging in assessment o brain
ollowed by con uent deep cerebral white matter. damage rom carbon monoxide poisoning: a review o the
3. Carbon monoxide has over 200× binding a f nity with literature. AJNR Am J Neuroradiol. 2014 Apr;35(4):625-631.
hemoglobin than oxygen, not allowing appropriate Sharma P, Eesa M, Scott JN. Toxic and acquired metabolic
oxygen delivery to cells. encephalopathies: MRI appearance. AJR Am J Roentgenol.
2009 Sep;193(3):879-886.
40
80-year-old male with a severe headache and left paralysis

1. Where is the abnormality located?

2. In the acute/subacute setting, what f ndings are


present?

3. Several months a ter the event, what f ndings


are present?

4. What is the etiology or this f nding?

5. What is the treatment or this entity?

41
Wallerian degeneration Case 16 (3514)
Case ranking/dif culty: Category: Intra-axial supratentorial

3. Many months a ter injury, the diagnosis can be made by


noting atrophy o the ipsilateral cerebral peduncle and
pons.
4. Wallerian degeneration etiologies include in arction,
hemorrhage, neoplasm, demyelination, trauma, and
vascular mal ormations. Basically any process that
cause neuronal damage, leading to anterograde axonal
degeneration.
5. There is no specif c treatment or Wallerian degeneration.

Pearls
• Anterograde axonal degeneration rom proximal
neuronal death.
• Best seen in the ipsilateral descending corticalspinal
tract rom proximal injury.
• DWI can have restricted di usion acutely.
• T1 hyperintensity is noted in 4-14 weeks with myelin
breakdown.
• T2 hyperintensity is noted in >14 weeks with gliosis.
• Atrophy best noted in the ipsilateral brainstem is seen
in the chronic phase.
• Presence o restricted di usion and T2 hyperintensity
correlates with morbidity.
Axial CT image shows volume loss of the right cerebral peduncle
(red arrow). There is encephalomalacia from previous large right
MCA injury (green arrow).
Suggested Readings

Answers Ho ML, Moonis G, Ginat DT, Eisenberg RL. Lesions


o the corpus callosum. AJR Am J Roentgenol. 2013
1. The abnormality is located within the cerebral peduncle Jan;200(1):W1-W16.
o the midbrain. Puig J, Pedraza S, Blasco G, et al. Wallerian degeneration
2. In Wallerian degeneration, the MR signal changes in the corticospinal tract evaluated by di usion tensor
depend on the time rom the event. Weeks 1 to 4, T1 imaging correlates with motor def cit 30 days a ter middle
and T2 are normal but DWI can show high signal. No cerebral artery ischemic stroke. AJNR Am J Neuroradiol.
contrast enhancement is seen. 2010 Aug;31(7):1324-1330.

42
54-year-old female with memory loss and behavioral disturbances

1. What is the di erential diagnosis?

2. What comprises an abnormal amyloid ligand


PET scan?

3. Which lobes are most a ected in this disease?

4. What is seen on FDG PET imaging in this


disease?

5. What percentage o patients are a ected with this


disease in the older than 85 years age group?
43
Alzheimer disease Case 17 (3546)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial FLAIR image Axial CT image shows Axial PET FDG of the brain Axial PET FDG shows symmetric
demonstrates cortical increased sulcal prominence shows hypometabolism of the hypometabolism of the parietal
atrophy most prominent from atrophy of the parietal temporal lobes (arrows). lobes (arrows).
in the temporal lobes and lobes (arrows).
hippocampi (arrows).

Answers
• Early-onset Alzheimer (<60 years) is uncommon and
1. Di erential diagnosis or nonreversible dementias
may be associated with amilial orms (5-10%).
includes Alzheimer, rontotemporal, dementia with
• Def nitive diagnosis requires brain biopsy with
Lewy bodies, vascular dementia, and corticobasal
visualization o beta amyloid plaques in the cortex and
degeneration.
intracellular neurof brillary tangles.
2. An abnormal orbetapir scan demonstrates increased • The role o imaging is to support the clinical diagnosis
uptake in the cortex with blurring o the gray-white by excluding other causes, evaluate or degree
di erentiation. A normal amyloid scan demonstrates o atrophy with volumetric imaging, and provide
greater white matter uptake, with visualization o the specif city with PET.
gray-white inter ace. • Cortical atrophy is classically in the temporal and
parietal lobes with hypometabolism o these regions
3. Alzheimer predominantly a ects the temporal and
on PET FDG or SPECT.
parietal lobes with greater atrophy.
• PET orbetapir, which has high sensitivity o binding
4. Decreased uptake in the parietal and temporal lobes can to beta amyloid, can show increased abnormal uptake
be seen on FDG PET in Alzheimer disease. in the cortex over normal uptake in the white matter
• Amyloid angiopathy with multiple hemorrhages can
5. Up to 45% o people older than 85 year o age have
be seen in 80%-90% o Alzheimer patients.
late-onset Alzheimer disease, which is the most common
cause o dementia.
Suggested Readings
Guo H, Song X, Vandorpe R, et al. Evaluation o common
Pearls structural brain changes in aging and Alzheimer disease
• Late-onset Alzheimer disease is very common and the with the use o an MRI-based brain atrophy and lesion
greatest risk actor is advanced age with up to 45% o index: a comparison between T1WI and T2WI at 1.T and
people greater than 85 years o age demonstrating this 3T. AJNR Am J Neuroradiol. 2014 Mar;35(3):504-512.
disease. Kantarci K. Molecular imaging o Alzheimer disease
pathology. AJNR Am J Neuroradiol. 2014 Jun;35(6 suppl):
S12-S17.

44
70-year-old female with headaches

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What mal ormations are associated with this


entity?

4. What is the etiology o this entity?

5. What is the treatment or this entity?


45
Corpus callosum agenesis Case 18 (3111)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT image shows colpocephaly with nonvisualization of the Axial CT image shows colpocephaly with a “tear drop”appearance
genu and splenium of the corpus callosum (asterisks). to the lateral ventricular bodies (arrows).

3. Callosal dysgenesis may be associated with Aicardi


syndrome, Chiari II mal ormation, pericallosal lipoma,
migration anomalies, and Dandy-Walker mal ormation.
Callosal dysgenesis is the most common f nding in CNS
mal ormations.
4. CC agenesis is a congenital process and thought to
be a ailure o axons to reach midline and cross to
the contralateral hemisphere. Large, abnormal Probst
bundles medial to the lateral ventricle are the result.
5. No treatment is needed.

Pearls
• An embryonic insult during ormation o the CC may
result in dysgenesis to agenesis.
• Sagittal midline image demonstrates a central gyri
with a radiating pattern.
Sagittal T1 image shows absence of the corpus callosum (white • Axial images show abnormal parallel alignment o
arrow) and radiating appearance of the gyri. the lateral ventricles with a “tear drop” appearance
(colpocephaly).
• CC dysgenesis has associations with midline lipomas,
Answers interhemispheric cysts, acial anomalies, azygous
1. The morphology o the lateral ventricles is abnormal ACA, and cortical maldevelopment.
rom callosal agenesis.
2. Corpus callosum (CC) agenesis classic imaging f ndings Suggested Reading
are colpocephaly—lateral ventricular “tear drop”
appearance (with a parallel orientation), and radiating Ho ML, Moonis G, Ginat DT, Eisenberg RL. Lesions
pattern o the central gyri. o the corpus callosum. AJR Am J Roentgenol. 2013
Jan;200(1):W1-W16.
46
25-year-old with seizure disorder

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the etiology o this entity?

5. What is the treatment or this entity?

47
Gray matter heterotopia Case 19 (3151)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT image shows focal nodules along the subependymal Axial T2 images show the nodules isointense to cortical gray
lining of the lateral ventricles (arrows). matter (arrows).

Answers
1. The abnormality is located along the ependymal lining. Pearls
• Heterotopic gray matter (HGM) is a migration
2. The classic imaging f ndings or this entity are lesions
anomaly where gray matter is located within the white
that have the same attenuation on CT or intensity on MR
matter.
as gray matter.
• This arrest in migration may occur rom the start along
3. The di erential diagnosis includes tuberous sclerosis, the ventricular lining to the cortex.
heterotopic gray matter, and metastasis. • Subependymal heterotopia is associated with callosal
agenesis, Chiari II, polymicrogyria, and basilar
4. Heterotopic gray matter (HGM) is a congenital
cephaloceles.
migration anomaly where gray matter is located within
• HGM may be band like in morphology.
the white matter. Periventricular heterotopias are o ten
• I enhancement is present consider tumor or tubers.
genetic.
• I calcif cations are present consider subependymal or
5. The treatment or this entity is medical aimed at seizure cortical tubers.
control. Surgery may be needed with nodule removal
when intractable seizures are present.
Suggested Readings
Barkovich AJ, Chuang SH, Norman D. MR o neuronal
migration anomalies. AJR Am J Roentgenol. 1988
Jan;150(1):179-187.
Mitchell LA, Simon EM, Filly RA, Barkovich AJ. Antenatal
diagnosis o subependymal heterotopia. AJNR Am J
Neuroradiol. 2000 Feb;21(2):296-300.

48
10-year-old boy with cafe-au-lait spots

1. What is the genetic inheritance o this disease?

2. What are characteristic brain f ndings in this


disease?

3. What are characteristic tumors and bony


involvement or this disease?

4. What is the best imaging modality in the


diagnosis and ollow-up NF1?

5. Where are the oci o abnormal signal


intensities in the brain located in this disease?

49
Neuro bromatosis type 1 Case 20 (3219)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image demonstrates extensive, ropy T2 hyperintense Axial T2 image at the level of the basal ganglia shows bilateral foci
lesions consistent with plexiform neuro bromas along the right of abnormal signal intensity of the globi pallidi (arrows). Again
side of the face extending to the intra- and extraconal space of the noted is the right facial plexiform neuro broma extending to the
right orbit and along the right Meckel cave (white arrows). Noted is temporal scalp (arrowhead).
right sphenoid wing dysplasia (white arrowhead). Target appearance
is a characteristic nding for plexiform neuro bromas in NF1 (red
arrowhead).

Oblique T1 postcontrast in a 4-year-old male with NF1 shows


an enhancing optic nerve glioma extending through the orbital
canal (arrow).
Axial T2 image of a 2-year-old female with NF1 shows multiple
bilateral foci of abnormal signal intensity in the deep cerebellar
white matter and dentate nuclei (arrows).

50
Neuro bromatosis type 1 (Cont.) Case 20 (3219)

Answers
1. NF 1 is caused by mutation or deletion o the NF1 gene Pearls
located on chromosome 17q11.2, causing interruption • Neurof bromatosis type 1 (NF1) is the most common
o neurof bromin protein production. This can be rom neurocutaneous disease.
autosomal dominant amilial inheritance (50%) or new • Mutation o NF1 gene in chromosome 17q11.2,
sporadic mutation (50%). causing disruption o neurof brin, a tumor suppressor.
• Autosomal dominant inheritance (50%) or new
2. NF1 changes involving the brain include ocal T2
mutation (50%).
hyperintense lesions termed oci o abnormal signal
• Present with cutaneous, neurological, and skeletal
intensity or unidentif ed bright objects in the deep gray
mani estations.
matter and periventricular white matter, brainstem
• Highly associated with learning disability and autism.
gliomas, and optic nerve gliomas.
• Classic radiographic f ndings include plexi orm
3. Classic radiographic f ndings include plexi orm neurof bromas, sphenoid wing dysplasia, optic nerve/
neurof bromas, sphenoid wing dysplasia, optic nerve optic pathway/brainstem glioma, and white matter
gliomas, and white matter changes (myelin vacuolization). changes (myelin vacuolization).
• Plexi orm neurof bromas are ropy tumors with
4. Neurof bromatosis type 1 (NF1) or von Recklinghausen
increased T2 signal and central T2 hypointensity
disease presents with multisystem genetic disorders
orming a “target sign” with variable enhancement.
including cutaneous, neurological, and orthopedic
• Optic nerve gliomas demonstrate di use or ocal
mani estations. STIR imaging is help ul to evaluate or
thickening o the optic nerve with variable enhancement.
neurof bromas throughout the body, oci o abnormal
• Focal areas o signal intensity (FASI) or unidentif ed
signal intensity within the brain, brain stem, and optic
bright objects (UBO) are seen involving the deep
nerve gliomas.
cerebellar white matter, dentate nuclei, brainstem,
5. Typical locations or NF1 oci o abnormal signal hippocampi, globi pallidi, and periventricular white
intensity include the globi pallidi, deep cerebellar white matter, thought to represent hamartomas.
matter and dentate nuclei, hippocampi, brainstem, and • Surgical resection, radiation, or chemotherapy reserved
periventricular white matter. There are amorphous oci or symptomatic patients and progressing masses.
o typically nonenhancing T2 hyperintensity without
signif cant mass e ect.
Suggested Readings
Jacquemin C, Bosley TM, Svedberg H. Orbit de ormities
in cranio acial neurof bromatosis type 1. AJNR Am J
Neuroradiol. 2003 Sep;24(8):1678-1682.
Lim R, Jaramillo D, Poussaint TY, Chang Y, Kor B.
Superf cial neurof broma: a lesion with unique MRI
characteristics in patients with neurof bromatosis type 1.
AJR Am J Roentgenol. 2005 Mar;184(3):962-968.
Patel NB, Stacy GS. Musculoskeletal mani estations o
neurof bromatosis type 1. AJR Am J Roentgenol. 2012
Jul;199(1):W99-W106.

51
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3-year-old female with history of facial hemangioma and seizure

1. What is the di erential diagnosis?

2. What are the characteristic radiographic


eatures?

3. What is the typical clinical prof le?

4. Where are typical locations or leptomeningeal


enhancement?

5. What is the treatment option?

53
Sturge-Weber syndrome Case 21 (3403)
Case ranking/dif culty: Category: Intra-axial supratentorial

CT image of the brain demonstrates severe atrophy of the Axial T2 image demonstrate severe atrophy of the right cerebral
right cerebral hemisphere with extensive cortical/subcortical hemisphere with T2 hypointensity involving cortical/subcortical
calci cations in the right cerebral hemisphere and left parieto- white matter of the right fronto-parieto-occipital and left parieto-
occipital lobe (red arrowheads). Incidentally noted is cavum occipital lobes (white arrowheads). Bilateral choroidal cysts are
septum pellucidum (white asterisk). noted (black asterisks).

GRE image demonstrates hypointensity without blooming Postcontrast T1 image demonstrates extensive leptomeningeal
involving the right cerebral hemisphere and left occipital cortical/ enhancement involving right cerebral cortical sulci and left
subcortical white matter (white arrowheads), consistent with parieto-occipital sulci (white arrowheads) as well as avid
calci cations seen in the prior CT. enhancement of the choroid plexi bilaterally (white arrows).
Note the relative lack of cortical veins of the atrophied right
cerebral hemisphere compared to the contralateral normal brain
parenchyma.

54
Sturge -Weber syndrome (Cont.) Case 21 (3403)

Answers
1. The di erential diagnosis or a combination o Pearls
cerebral calcif cation, cerebral hemiatrophy, and • Rare sporadic congenital vascular mal ormation,
leptomeningeal enhancement includes Sturge- with increased levels o endothelial proli eration, and
Weber, Klippel-Trenaunay-Weber, cerebral AVM, ailure o development o etal cortical veins.
and meningioangiomatosis. All can have thickened • Increasing venous stasis leads to decreased cortical
leptomeningeal enhancement with calcif cation. per usion.
• Clinical diagnosis based on presence o hemi acial
2. In Sturge Weber, serpentine pial enhancement corresponds
port-wine stain, usually along the V1 and V2
to leptomeningeal angiomatosis, in combination with
distributions, and ocular choroidal angiomas.
lack o cortical veins and progressive venous stasis, leads
• Usually present with seizures and hemiparesis,
to cortical atrophy and subsequent calcif cation. The
• Radiographic eatures include hemispheric gyral/
ipsilateral choroid plexus is typically enlarged.
subcortical white matter calcif cations (tram-track
3. The typical clinical prof le or Sturge-Weber syndrome gyral calcif cation) associated with brain atrophy,
is a port-wine stain o the ace, ipsilateral glaucoma, and serpentine leptomeningeal enhancement, and
seizures in an in ant. ipsilateral engorged choroid plexi.
• Absence o pial angiomas at age 1 year can reliably
4. In Sturge-Weber, leptomeningeal enhancement rom pial
exclude the uture development o abnormal
angiomatosis can involve any pial sur ace but is most
leptomeningeal vessels.
commonly posterior (parieto-occipital) and unilateral.
• Approximately 90% o in ants with acial port-wine
Bilateral involvement is seen in 20% o cases.
stains do not have intracranial lesions and would be
5. There is no curative treatment or Sturge-Weber expected to develop normally.
syndrome. Treatment o seizures can include
hemispherectomy. Low-dose aspirin may help decrease
requency o stroke-like episodes. Suggested Reading
Nozaki T, Nosaka S, Miyazaki O, et al. Syndromes
associated with vascular tumors and mal ormations:
a pictorial review. Radiographics. 2013;33(1):175-195.

55
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3-month-old boy with history of developmental delay

1. What is the di erential diagnosis or this


f nding?

2. What is the pathogenesis o this f nding?

3. What is the classic radiographic description?

4. What are clinical characteristics o patients


demonstrating only band heterotopia?

5. In prenatal US or etal MRI what must be


known about the patient prior to diagnosing
lissencephaly?

57
Lissencephaly—type I Case 22 (3430)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT image demonstrates hourglass con guration of cerebral Axial T2 image demonstrates hourglass con guration of cerebral
hemispheres with smooth cortical surface and shallow sylvian hemispheres with smooth cortical surface and shallow sylvian
ssures (white arrowheads) with lateralized middle cerebral ssures (white arrowheads) with lateralized middle cerebral vessels
vessels (green arrowheads). Also noted is symmetric subcortical (green arrowheads). Also noted is continuous subcortical hypointense
gray matter thick band heterotopia (white arrow). band consistent with heterotopic gray matter (white arrow).

Answers
Pearls
1. Di erential diagnosis includes cobblestone lissencephaly
(usually associated with congenital muscular • Severe mal ormation o the cerebral cortex rom
dystrophies), other migrational abnormalities (band impaired neuronal migration during third to ourth
heterotropia), simplif ed gyral pattern, and intrauterine months o gestation.
in ections. This severe orm is consistent with Miller- • Divided into two types: Classic (type 1) and
Dieker syndrome, a type I lissencephaly. cobblestone (type 2) lissencephaly.
• Classic lissencephaly results rom undermigration;
2. Classic lissencephaly (type 1) results rom may be syndromic, x-linked, or isolated.
undermigration o neuron secondary to syndromic • Typical radiographic eatures include hourglass
(Miller-Dieker, or Norman-Roberts syndrome), x-linked, (f gure eight) conf guration with smooth sur ace and
or isolated orms secondary to in utero toxin exposure or shallow sulci involving bilateral cerebral hemispheres
congenital in ection. as well as lateral displacement o the middle cerebral
3. Classic radiographic eatures include hourglass or f gure vessels; may be associated with band heterotopia.
eight conf guration with smooth cortical sur ace, shallow • Miller-Dieker syndrome can be di erentiated
sylvian f ssures with lateral displacement o the middle rom isolated lissencephaly by presence o acial
cerebral vessels. Subcortical gray matter and thick band dymorphism and severe lissencephaly.
heterotopia may be seen. • Cobblestone lissencephaly results rom overmigration
leading to disorganized underlayered and irregular cortex,
4. Patients with only band heterotopia are overwhelmingly usually associated with congenital muscular dystrophies.
emale and with mild symptoms. This is associated with • Typical radiographic eatures include cobblestone
the x-linked DCX gene with mild phenotype in emales appearance with severe ventriculomegaly associated
with isolated band heterotopia. Male children o these with hypomyelination, vermian hypogenesis,
emales have more severe lissencephaly with rontal kinked brainstem, cerebellar polymicrogyria or
predominance. cysts, cephalocele, corpus callosal hypogenesis, and
5. Findings suggesting lack o sulcation must be correlated hydrocephalus.
with gestational age to diagnose lissencephaly versus
normal immature brain.
Suggested Reading
Ghai S, Fong KW, Toi A, Chitayat D, Pantazi S, Blaser S.
Prenatal US and MR imaging f ndings o lissencephaly:
review o etal cerebral sulcal development.
Radiographics. 2006;26(2):389-405.
58
1-month-old female with abnormal prenatal US and fetal MRI

1. What is the di erential diagnosis?

2. What is the possible cause o this disease?

3. What are the typical radiographic eatures?

4. What are treatment options?

5. What cerebral cortex is typically intact, i any?

59
Hydranencephaly Case 23 (3448)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT demonstrates uid lled in cranial Axial CT demonstrates uid- lled cranial Axial T2-weighted image fetal MRI
vault (white asterisks) with intact falx vault (white asterisks). There is residual demonstrates uid- lled cranial vault
cerebri (white arrowhead), thalami (green brain parenchyma in the frontal and (white asterisks) with intact falx cerebri
asterisk), and cerebellar hemisphere (blue occipital lobes (green asterisks). VP shunt (black arrowhead), thalami (green
asterisk). is noted (white arrow) asterisk), and occipital lobe (blue asterisk).

intact alx cerebri, intact thalami, brainstem, and


cerebellum.
4. Hydranencephaly patients have a poor prognosis,
usually dying in in ancy. Supportive treatment with
ventriculostomy placement is suggested or macrocephaly.
5. Residual supratentorial cortex seen in hydranencephaly
can include structures that can be supplied by the
posterior cerebral artery, including the occipital lobes,
and paramedian parietal and posterior rontal lobes.

Pearls
• Congenital brain de ect secondary to massive brain
in arction.
• Postnatal presentations include macrocephaly,
Fetal MRI demonstrates uid- lled cranial vault (white asterisks).
There is residual brain parenchyma in the frontal lobes (green developmental ailure, calvarial translumination,
asterisks) with intact thalami (blue asterisk). seizure, or neurological unction limited to brainstem.
• Radiographic eatures are the same in both pre- and
postnatal imaging.
Answers
• Classic radiographic eatures include uid-f lled
1. Di erential diagnosis or hydranencephaly includes cranial vault, absent cerebral mantle, partially
severe hydrocephalus, severe bilateral open lip or completely intact alx cerebri, intact thalami,
schizencephaly, and alobar holoprosencephaly. brainstem, and cerebellum.
2. Occlusion o bilateral supraclinoid internal carotid
arteries is the avored etiology. Other possible etiologies Suggested Readings
include intrautero in ection, maternal carbon monoxide
Gentry M, Connell M. Hydranencephaly. Ultrasound Q. 2013
or butane exposure, and thromboembolism rom a
Sep;29(3):267-268.
deceased monochorionic twin.
Poe LB, Coleman LL, Mahmud F. Congenital central
3. Classic radiographic eatures include uid-f lled cranial nervous system anomalies. Radiographics. 1989
vault, absent cerebral mantle, partially or completely Sep;9(5):801-826.
60
12-year-old male with headache

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the etiology o this entity?

5. What is the treatment or this entity?


61
Neuroglial cyst Case 24 (3165)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image shows a well-circumscribed lesion of high T2 signal Axial T1 image shows a well-circumscribed lesion of low signal
within the white matter adjacent to the right frontal horn (arrow). within the white matter adjacent to the right frontal horn (arrow).

3. The di erential diagnosis includes perivascular space,


porencephalic cyst, and neuroglial cyst.
4. Neuroglial cysts are also known as glioependymal cysts
and are congenital, benign neuroepithelial-lined lesions.
They are thought to be the sequestered lining o the
embryonic neural tube.
5. No treatment is needed or this entity.

Pearls
• Neuroglial cyst (NGC) and glioependymal cyst are
benign epithelial-lined lesions.
• NGC can be seen anywhere along the neural axis but
are more common within the brain parenchyma (most
commonly rontal lobe).
• These lesions are usually well circumscribed, round,
Axial T1 postcontrast image demonstrates no abnormal and unilocular.
enhancement (arrow). • I multiloculated, consider perivascular space.
• I cyst communicates with a ventricle, consider
porencephaly.
Answers • I contrast enhancement is seen, consider tumor.
1. The abnormality is located within the white matter
adjacent to the right rontal horn.
2. Neuroglial cysts ollow CSF on CT and all MRI Suggested Reading
sequences. There ore, they are low T1, high T2 Osborn AG, Preece MT. Intracranial cysts: radiologic-
signal with low density on CT. Also, no associated pathologic correlation and imaging approach. Radiology.
enhancement is seen. 2006 Jun;239(3):650-664.

62
79-year-old female with a headache

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the etiology o this entity?

5. What is the treatment or this entity?

63
Hippocampal sulcus remnant Case 25 (3465)
Case ranking/dif culty: Category: Intra-axial supratentorial

Coronal FLAIR shows the hippocampal lesions suppress, following


CSF signal (arrows).

Axial T2 image shows well-de ned, subcentimeter high T2 signal


Pearls
lesions within the hippocampal gyri appearing as a “string of cysts.”
• Hippocampal sulcus remnants (HSR), also called
hippocampal sulcal cavities, are a common f nding
Answers and are likely the result o incomplete olding o the
1. A string o cystic lesions is noted along the bilateral hippocampus along the vestigial sulcus.
hippocampi. • Seen as string o CSF cysts along the lateral margin o
the hippocampus.
2. Hippocampal sulcus remnants (HSR) on MRI have • I ocal increased FLAIR signal consider in arct and
corresponding high T2 signal and low T1 and FLAIR encephalitis.
signal, ollowing CSF. HSR do not show contrast • Consider mesial temporal sclerosis with increased
enhancement or restriction on DWI. T2/FLAIR signal and hippocampal volume loss.
3. The di erential diagnosis includes hippocampal sulcus
remnant, mesial temporal sclerosis, and choroidal f ssure
cyst. Suggested Reading
4. HSR is thought to be a partial lack o usion o the van Veluw SJ, Wisse LE, Kuij HJ, et al. Hippocampal T2
hippocampal sulcus as the cornu ammonis and dentate hyperintensities on 7 Tesla MRI. Neuroimage Clin. 2013
gyrus old to orm the characteristic hippocampal gyrus. Jan;3(3):196-201.
These may become more prominent with volume loss
and are more easily seen on high-resolution imaging.
5. No treatment is needed or this entity.

64
33-year-old male with headache

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the etiology o this entity?

5. What is the treatment or this entity?

65
Enlarged perivascular spaces Case 26 (3559)
Case ranking/dif culty: Category: Intra-axial supratentorial

Sagittal T1 image shows multiple, well- Axial T2 image shows a cluster of high- Coronal FLAIR image demonstrates
circumscribed low-signal structures within the signal cystic structures within the suppression of the cystic lesions
white matter of the right parietal lobe (arrows). white matter of the right parietal lobe following CSF (arrow).
(arrow). Note the radial orientation to
the lateral ventricle.

4. PVS is thought to be developmental, rom accumulation


o interstitial uid between the penetrating vessel and pia.
5. No treatment is needed or this entity.

Pearls
• Perivascular spaces (PVS) are also known as Virchow-
Robin spaces.
• Follows CSF on all pulse sequences and do not
enhance.
• 25% have some minimal increased surrounding
FLAIR signal.
• White matter and deep gray nuclei location.
Axial T1 postcontrast image shows no enhancement within the • The most common site involves the lower basal
right parietal lobe white matter cystic lesions (arrow). ganglia.
• Radial orientation to the ventricle in the deep white
matter.
Answers • As age increases, the size o PVS enlarges.
1. The abnormality is located within the subcortical white • These spaces are usually 2 to 5 mm in size.
matter o the right parietal lobe.
2. On imaging, perivascular spaces (PVS) always ollow
CSF signal on all pulse sequences. There ore, these are Suggested Readings
low T1 and high T2 signals with suppression on FLAIR. Osborn AG, Preece MT. Intracranial cysts: radiologic-
These spaces do not restrict di usion or enhance. pathologic correlation and imaging approach. Radiology.
3. The di erential diagnosis includes lacunar in arction, 2006 Jun;239(3):650-664.
perivascular spaces, cystic neoplasm, cryptococcoma, Kwee RM, Kwee TC. Virchow-Robin spaces at MR imaging.
and neurocysticercosis. Radiographics. 2007;27(4):1071-1086.

66
3-month-old boy with history of microcephaly and hearing loss

1. What is the incidence or this disease?

2. What is the typical clinical presentation?

3. What are characteristic radiographic f ndings?

4. What is the di erential diagnosis?

5. What are common late sequelae o this


disease?

67
Congenital CMV Case 27 (3182)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T1 image demonstrates multiple punctate foci of T1 Coronal T1 image demonstrates foci of T1 hyperintensity in
hyperintensity in bilateral periventricular white matter (white right periventricular white matter (white arrows), consistent with
arrows), consistent with periventricular calci cations. periventricular calci cations. There is open-lip schizencephaly
in the right parietal lobe (white arrowhead). Also noted is right
cerebellar hypoplasia (asterisk).

Axial T2 image demonstrates di use migrational anomalies with


pachygyria (white arrow), lissencephaly (white arrowhead), and
polymicrogyria (red arrowheads). Axial T2 image demonstrates pachygyria in the left frontoparietal
cortices (arrows). Again noted is the right parietal schizencephalic
cleft (red arrowheads).

68
Congenital CMV (Cont.) Case 27 (3182)

Noncontrast CT image from another infant with congenital CMV Noncontrast CT image demonstrates periventricular calci cations
demonstrates periventricular calci cations (arrows). (white arrows) and hypoplasia of the left cerebellum (asterisk).

Answers
1. Congenital CMV in ection is the most common serious Pearls
viral in ection a ecting approximately 1% o all • Most common cause o intrauterine in ection with
newborns in the United States. congenital brain damage in the United States.
• Usually asymptomatic (90%); only 10% o patients
2. Only 10% o in ected in ants are symptomatic at birth.
have signs and symptoms at birth.
Most common systemic signs o disease involvement
• Classic clinical presentations: microcephaly,
include prematurity, hepatosplenomegaly, jaundice,
seizure, chorioretinitis, petechiae, dea ness, and
petechiae, chorioretinitis, and intrauterine growth
hepatosplenomegaly.
retardation. CNS involvement includes microcephaly,
• Classic brain f ndings: periventricular calcif cations,
seizure, hypo- or hypertonia, and late f ndings o
cortical mal ormation ranging rom agyria,
sensorineural hearing loss.
pachygyria, di use polymicrogyria, ocal cortical
3. Classic CNS radiographic f ndings include periventricular dysplasia, and schizencephaly, in addition to delayed
calcif cations, cortical mal ormations ranging rom myelination and cerebellar hypoplasia.
agyria, pachygyria, di use polymicrogyria, ocal cortical • Degree o intracranial abnormalities depends on
dysplasia, and schizencephaly. Delayed myelination and gestational age at time o in ection.
cerebellar hypoplasia are commonly seen. • Sensorineural hearing loss is the most common late
sequela.
4. Di erential diagnosis o congenital CMV in ection
includes other TORCH in ections, pseudo-TORCH
syndrome, and congenital lymphocytic choriomeningitis.
Suggested Readings
5. Sensorineural hearing loss is the most common
late sequela; however, other CNS sequelae include Malinger G, Lev D, Zahalka N, et al. Fetal cytomegalovirus
seizures, lack o coordination, mental disability, and in ection o the brain: the spectrum o sonographic
microcephaly. f ndings. AJNR Am J Neuroradiol. 2003 Jan;24(1):28-32.
Teissier N, Fallet-Bianco C, Delezoide AL, et al.
Cytomegalovirus-induced brain mal ormations in etuses.
J Neuropathol Exp Neurol. 2014 Feb;73(2):143-158.

69
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17-year-old male with severe aplastic anemia status post stem cell
transplant. Development of fever and neurologic changes

1. What is the di erential diagnosis or this


f nding?

2. What is an imaging sign help ul to distinguish


between ring-enhancing etiologies such as
abscess and metastatic disease?

3. What is the cause o T2 hypointensity in the


periphery o these lesions?

4. What vascular distribution is more common or


these lesions?

5. What organism(s) has more likelihood o


angioinvasion?
71
Aspergillosis Case 28 (3233)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial FLAIR image shows two Axial T1 postcontrast image Axial DWI b = 1000 shows Coronal T1 postcontrast
rounded ring-like lesions in the shows rim enhancement central di usion restriction demonstrates another posterior
right frontal parietal lobe, at (white arrows) which is (white arrows) suggesting parietal faint ring-enhancing
the gray-white junction. Note relatively faint, likely due to abscesses. lesion at the gray-white
the T2 hypointense rim and immunosuppressed state. junction (white arrow).
minimal surrounding edema of
the lesion (white arrows).

Answers
1. The di erential or multiple ring-enhancing lesions Pearls
includes both ungal and bacterial abscesses, • Think about ungal disease in immunocompromised
toxoplasmosis, and metastatic disease. patients!
• MRI f ndings o hematogenous ungal disease are not
2. Abscesses can show central restricted di usion rom
distinguishable rom bacterial causes.
pyogenic material. Likewise, metastases can show
• Postcontrast images can show meningeal enhancement
peripheral restricted di usion rom hypercellularity and
or nodular and ring appearance in the parenchyma.
central acilitated di usion in areas o necrosis.
• Ring-enhancing lesions may show less than expected
3. T2 hypointense rims coinciding with peripheral enhancement and edema in immunocompromised
enhancement correlate histologically with the abscess patients.
capsule. It is thought that paramagnetic ree radicals • Central di usion restriction and a rim o T2
ormed by macrophages give the susceptibility e ect o hypointensity may be seen with ungal abscesses,
decreased T2 signal. It has been suggested that iron is helping distinguish rom metastatic disease.
a signif cant component o this paramagnetic e ect in • Aspergillosis and mucormycoses can be angioinvasive,
ungal abscesses. causing mycotic aneurysms and hemorrhagic in arcts.
4. The anterior circulation is involved more o ten rom
hematogenous seeding o abscesses, with the gray-white
junction most commonly involved. Suggested Readings

5. Angioinvasion has typically been described with Boes B, Bashir R, Boes C, Hahn F, McConnell JR, McComb
invasive aspergillosis and mucormycosis in the R. Central nervous system aspergillosis. Analysis o 26
immunocompromised. This has also been described to a patients. J Neuroimaging. 1994 Jul;4(3):123-129.
lesser extent with candidiasis. Mathur M, Johnson CE, Sze G. Fungal in ections o the
central nervous system. Neuroimaging Clin N Am. 2012
Nov;22(4):609-632.

72
5-year-old female with headache and vision changes, recent viral illness

1. What is the di erential diagnosis or this


f nding?

2. What other f ndings can be seen with this


disease?

3. What is the treatment or this disease?

4. What is the prognosis o this disease?

5. How well does imaging correlate with disease


progress?

73
Acute disseminated encephalomyelitis Case 29 (3236)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image shows bilateral patchy asymmetric white matter Axial FLAIR image shows patchy white matter hyperintensities
hyperintensities (white arrows). (white arrows) that do not extend to the callosal septal interface.

Axial FLAIR image demonstrates left temporal subcortical white Coronal postcontrast image shows vague amorphous enhancement
matter T2 hyperintensity (white arrow). of the white matter lesions (white arrows) without signi cant T1
hypointensity of the involved areas. Note an isolated lesion of the
left cerebellum.

74
Acute disseminated encephalomyelitis (Cont.) Case 29 (3236)

Axial T1 postcontrast image through the orbits shows bilateral


Coronal T1 postcontrast through the orbital apex shows the
thickening and enhancement of the optic nerves (white arrows).
bilateral enlargement and enhancement of the optic nerves
This is consistent with optic neuritis.
(white arrows).

Answers
1. Di erential diagnosis or bilateral patchy white matter Pearls
lesions with vague enhancement includes demyelinating • ADEM is a monophasic, immune-mediated
processes such as ADEM and MS. With the bilateral in ammatory demyelination 1-2 weeks a ter viral or
posterior distribution in this case, PRES could be bacterial in ection, as well as immunization.
considered. • Bilateral patchy white matter involvement is typical,
with variable enhancement.
2. ADEM can also have basal ganglia involvement in hal
• Central white matter is not usually involved like MS.
o cases and spinal cord involvement in 30% o cases.
• Hal o cases have basal ganglia involvement.
Cranial nerve involvement with edema and enhancement
• Cranial nerve involvement with enhancement can be seen.
is also described.
• Spinal cord involvement can be seen in 30%.
3. Immunosuppression rom corticosteroids is the • The majority make a ull recovery with treatment.
typical f rst-line treatment or ADEM. Patients who Less than 1/3 have neurologic sequelae or progress to
do not respond to steroid therapy may benef t rom atality.
plasmapheresis or IVIG.
4. 50%-60% o patients with ADEM will recover with
adequate treatment without permanent def cit. Up to Suggested Readings
30% can have persistent sequelae, primarily seizures. Honkaniemi J, Dastidar P, Kähärä V, Haapasalo H. Delayed MR
10%-20% o cases o ADEM lead to atality. imaging changes in acute disseminated encephalomyelitis.
5. With ADEM, imaging f ndings typically lag the clinical AJNR Am J Neuroradiol. 2002 Feb;22(6):1117-1124.
symptoms. The initial imaging at presentation may be Marin SE, Callen DJ. The magnetic resonance imaging
normal. appearance o monophasic acute disseminated
encephalomyelitis: an update post application o the
2007 consensus criteria. Neuroimaging Clin N Am. 2013
May;23(2):245-266.

75
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78-year-old male with progressive headache, dizziness, slurred speech,
altered mental status, and progressive aphasia

1. What is the di erential or these MRI f ndings?

2. What is the usual anatomy a ected in this


disease?

3. What is the prognosis o this disease?

4. What are typical MRI f ndings on di erent


pulse sequences or this disease?

5. Which population groups have a higher risk o


this disease?

77
Herpes encephalitis Case 30 (3239)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image through the midbrain shows sulcal e acement Axial FLAIR image shows hyperintensity with sulcal e acement of
from swelling and T2 hyperintensity of the medial temporal lobe the medial temporal lobe (white arrow).
(white arrow).

Axial DWI b = 1000 image shows increased cortical and subcortical


Axial FLAIR image shows further involvement with T2 signal of the left medial temporal lobe (white arrow).
hyperintensity of the insular cortex and subthalamic nuclei (white
arrow). Note involvement of the tail of the hippocampus (red
arrow).

78
Herpes encephalitis (Cont.) Case 30 (3239)

Axial ADC map at the level of the midbrain shows decreased Axial DWI b = 1000 image also shows di usion restriction of the
di usion involving primarily the cortex of the medial temporal left insular cortex (white arrow) and hippocampal tail (red arrow).
lobe (black arrow).

Answers
1. The di erential or medial temporal lobe swelling Pearls
includes herpes encephalitis, acute stroke, and limbic • Herpes encephalitis typically involves the medial
encephalitis (which does not consistently cause temporal lobes, insular cortex, and in erior rontal lobes.
restricted di usion). Neurosyphilis has also been • Cortical di usion restriction is typical with variable
reported to involve the medial temporal lobes as a herpes enhancement early in the disease course.
encephalitis mimicker. • Sparing o the deep white matter and basal ganglia is
2. Herpes encephalitis usually involves the medial temporal commonly seen.
lobes, insular cortex, and in erior rontal lobes. The • Any pathology involving the medial temporal lobe
disease can be bilateral but asymmetric. with the typical clinical history o ever and headaches
must include herpes in the di erential.
3. Untreated herpes encephalitis can have up to 70% • I herpes encephalitis is suspected, urgent
mortality. Even with acyclovir treatment, a majority communication with the clinical team is necessary to
(2/3) o patients will have some neurologic sequelae. quickly start antiviral therapy (acyclovir), as untreated
Treatment with acyclovir should be initiated rapidly in herpes encephalitis is o ten atal.
patients with suspected herpes encephalitis.
4. In herpes encephalitis, swelling o the medial temporal
lobe causes increased signal on T2 and FLAIR, which
Suggested Readings
can be more sensitive than T2. Enhancement is variable
early in the disease; however, gyral enhancement a week Küker W, Nägele T, Schmidt F, Heckl S, Herrlinger U.
a ter presentation is more typical. DWI images typically Di usion-weighted MRI in herpes simplex encephalitis:
show bright cortical signal with corresponding decreased a report o three cases. Neuroradiology. 2004
signal on ADC, indicating restricted di usion. Feb;46(2):122-125.
Noguchi T, Yoshiura T, Hiwatashi A, et al. CT and
5. Herpes encephalitis has a bimodal distribution occurring
MRI f ndings o human herpesvirus 6-associated
more o ten in people less than 20 and older than 50 years
encephalopathy: comparison with f ndings o herpes
o age. 1/3 o cases occur in the less than 20 demographic,
simplex virus encephalitis. AJR Am J Roentgenol. 2010
who also tend to have better prognosis than patients older
Mar;194(3):754-760.
than 50. There is no gender or ethnic predilection.
79
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89-year-old female with confusion

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What brain structures can this entity involve?

5. What is the most common source o the high


attenuation?

81
Fahr disease Case 31 (3039)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT shows symmetric high density in the dentate nuclei Axial CT shows symmetric high density in the basal ganglia
(arrows). bilaterally (arrows).

Answers
1. Symmetric high density is seen in the basal ganglia and Pearls
the dentate nuclei. • Fahr disease is also called bilateral striopallidodentate
calcinosis.
2. Symmetric, bilateral high density within the basal
• The key to this diagnosis on CT is the symmetric
ganglia, especially in a young adult.
high density (calcif cation) o the basal ganglia in the
3. The di erential diagnosis includes hypoparathyroidism, absence o hypercalcemia.
pseudohypoparathyroidism, Fahr disease, mineralizing • Bilateral, symmetric basal ganglia calcif cations in a
microangiopathy rom prior injury, and normal young adult (<40) or children are usually not normal.
calcif cation in the middle aged to elderly. • Calcif cations are readily seen on CT.
• Dentate nuclei commonly are calcif ed.
4. Fahr disease may involve the basal ganglia (caudate
nucleus and lenti orm nucleus), dentate nuclei, thalami,
subcortical white matter, and centrum semiovale.
Suggested Readings
5. The most common source o the high attenuation is
calcium deposition. Avrahami E, Cohn DF, Feibel M, Tadmor R. MRI
demonstration and CT correlation o the brain in patients
with idiopathic intracerebral calcif cation. J Neurol. 1994
May;241(6):381-384.
Govindarajan A. Imaging in Fahr’s disease: how CT and
MRI di er? BMJ Case Rep. 2013 Nov 27;2013.
Hegde AN, Mohan S, Lath N, Lim CC. Di erential diagnosis
or bilateral abnormalities o the basal ganglia and
thalamus. Radiographics. 2011;31(1):5-30.
82
56-year-old male with left hemiparesis, syncope, and vision changes

1. What are the key imaging f ndings?

2. What are causes or intracranial herniation?

3. What clinical symptoms are associated with


le t uncal herniation?

4. What is Kernohan notch phenomenon?

5. Where do Duret hemorrhages occur?

83
Uncal herniation Case 32 (3316)
Case ranking/dif culty: Category: Intra-axial supratentorial

Coronal FLAIR image shows left frontal lobe Axial T2 image shows left uncal Axial T1 postcontrast image shows left
vasogenic edema with resultant subfalcine herniation with uncus located in the left frontal lobe enhancing extraaxial mass
herniation from left to the right (arrow) with anterior prepontine cistern (circle). (asterisk) causing subfalcine herniation
left uncal herniation (circle). to the right (arrow).

Answers
1. The key imaging f ndings is signif cant vasogenic edema, Pearls
resulting in rightward sub alcine herniation and le t • Uncal herniation is an emergent f nding.
uncal herniation. • Look or e acement o the suprasellar and prepontine
cistern.
2. Chiari mal ormation, di use cerebral edema, CNS mass
• Sub alcine herniation typically precedes transtentorial
lesions, hydrocephalus, and intracranial hemorrhage are
herniation.
causes o intracranial herniation.
• With a “blown pupil” look or ipsilateral uncal
3. Mass e ect on the ipsilateral third cranial nerve with herniation.
resultant dilation o the pupil and ailed constriction with • With uncal herniation, the tentorium cerebelli may
light stimulus due to mal unction o the parasympathetic create mass e ect on the cerebral peduncle, which may
f bers. The third cranial nerve compression will also result lead to ipsilateral hemiparesis.
in the ipsilateral eye to deviate “down and out,” rom
retained unction o the lateral rectus and superior oblique
muscles rom cranial nerves VI and IV, respectively.
Suggested Reading
4. Kernohan notch phenomenon is a result o transtentorial Mejía Kattah J, Vilá Barriuso E, García Bernedo C, Gallart
herniation causing contralateral compression o the Gallego L. [Kernohan-Woltman notch phenomenon
midbrain over the tentorium (Kernohan notch), which in secondary to a cranial epidural hematoma]. Rev Esp
turn causes hemiparesis ipsilateral to the transtentorial Anestesiol Reanim. 2014 Jun-Jul;61(6):332-335.
herniation but contralateral to Kernohan notch.
5. Duret hemorrhages are microbleeds in the medulla or
pons in patients with rapid herniation. They are typically
in the midline and possibly due to laceration o pontine
per orators or draining veins.

84
65-year-old female with left sided weakness

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What are the most common etiologies o this


f nding?

5. What is the treatment or this entity?


85
Metastasis, multifocal Case 33 (3249)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T1 image shows bilateral heterogeneous lesions within Axial T2 image shows heterogeneous appearance of the lesions
both basal ganglia. These lesions show T1 shortening, which may with mild edema adjacent to the largest lesion in the left basal
correspond to blood products (arrows). ganglia (arrow).

4. The most common etiologies include lung, melanoma,


and breast carcinoma.
5. The treatment or CNS metastases is dependent on the
primary carcinoma, but usually disseminated disease is
treated with whole brain radiation and/or chemotherapy.

Pearls
• Suspect metastasis with multiple lesions.
• Most commonly involves the supratentorial brain.
• The most common etiologies include lung, melanoma,
and breast carcinoma.
• 50% o CNS metastases are solitary.
• 50% o all brain tumors.
• History o primary carcinoma.
• Consider in ectious etiology in immunocompromised
Axial T1 postcontrast image shows multiple, bilateral patients.
heterogeneously enhancing lesions within both basal ganglia
(arrows). Also note that there is regional mass e ect resulting
from the left basal ganglia lesion with e acement of the anterior
Suggested Readings
horn of the left lateral ventricle and mild midline shift.
Tang YM, Ngai S, Stuckey S. The solitary enhancing
Answers cerebral lesion: can FLAIR aid the di erentiation between
glioma and metastasis? AJNR Am J Neuroradiol. 2006
1. The abnormality is located within the basal ganglia. Mar;27(3):609-611.
2. Metastasis can a ect any part o the brain. The imaging Toh CH, Wei KC, Ng SH, Wan YL, Lin CP, Castillo
appearance is variable and depends on the primary M. Di erentiation o brain abscesses rom necrotic
tumor. Classically, the lesions enhance and are multiple. glioblastomas and cystic metastatic brain tumors with
di usion tensor imaging. AJNR Am J Neuroradiol. 2011
3. The di erential diagnosis includes metastasis, abscess, Oct;32(9):1646-1651.
and demyelinating disease.
86
50-year-old male with increased headaches, memory loss, and speech
di culty

1. What is the di erential diagnosis?

2. What does the adjacent T2 hyperintensity o


the white matter represent?

3. What does the central nonenhancing


component represent?

4. What is the prognosis or this disease?

5. What can be seen with per usion and di usion


imaging?

87
Glioblastoma multiforme Case 34 (3334)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T1 postcontrast image Axial T2 image shows the Axial DWI ADC map shows areas Axial relative cerebral blood
shows an irregular ring- heterogeneous nature of of peripheral decreased ADC volume map from dynamic
enhancing lesion in the left the lesion with a rim of T2 from hypercellular portions of susceptibility contrast
temporal lobe (arrow). hypointensity (black arrowhead) the tumor (arrows). perfusion imaging shows a
and adjacent white matter rim of hyperperfusion largely
T2 hyperintensity (white correlating with the enhancing
arrow), typically representing components (arrow).
a combination of vasogenic
4. Prognosis remains dismal or glioblastoma multi orme,
edema and tumor in ltration in
this case of GBM. Note the mass with most cases resulting in death within 12 months.
e ect on the midbrain. 5. A hallmark o GBM is central necrosis with
hypercellular, viable tumor at the periphery, which
shows increased per usion and decreased di usion,
similar to other high-grade neoplasms.

Pearls
• Glioblastoma multi orme (GBM) is the most common
primary brain neoplasm in adults.
• Heterogeneous enhancement, sometimes in a ring with
central necrosis, can be seen.
• The enhancing component may demonstrate decreased
T2 signal and decreased ADC, as well as increased
per usion rom hypercellular portions o the tumor.
• There is typically adjacent white matter T2
Axial fractional anisotropy map shows a loss of anisotropy of hyperintensity, which represents vasogenic edema and
Meyer loop likely from tumor in ltration (arrows). tumor inf ltration.
• Centrally necrotic areas will not have central restricted
Answers di usion, which can be seen with abscesses.
1. The di erential or ring-enhancing mass-like lesions
includes glioblastoma, abscess, CNS lymphoma, solitary Suggested Readings
metastasis, and tume active demyelination. Hirai T, Murakami R, Nakamura H, et al. Prognostic value
2. The adjacent white matter T2 hyperintensity o o per usion MR imaging o high-grade astrocytomas:
glioblastomas typically represents a combination o long-term ollow-up study. AJNR Am J Neuroradiol. 2008
vasogenic edema and tumor inf ltration. Sep;29(8):1505-1510.
Young GS, Setayesh K. Spin-echo echo-planar per usion MR
3. The central nonenhancing components o glioblastomas imaging in the di erential diagnosis o solitary enhancing
are typically necrotic portions which have outgrown its brain lesions: distinguishing solitary metastases rom primary
blood supply. glioma. AJNR Am J Neuroradiol. 2009 Mar;30(3):575-577.
88
49-year-old male with new onset of headaches

1. Where is the abnormality located?

2. What are the classic imaging f ndings?

3. What is the di erential diagnosis?

4. Which part o the brain does this entity


commonly involve?

5. What are the most common primary etiologies


in adults with this entity?

89
Metastasis, solitary Case 35 (3467)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T1 image shows a heterogeneous Axial T2 image shows the heterogeneous Coronal FLAIR image shows the left frontal
lesion within the left frontal lobe, lesion with signi cant adjacent T2 mass and associated edema causing left to
cortical/subcortical location in the hyperintensity in the white matter, likely right midline shift (asterisk).
superior frontal gyrus (asterisk). from vasogenic edema (asterisks).
4. Metastasis can a ect any part o the brain. Most
commonly it involves the supratentorial brain.
5. CNS metastases are most commonly rom lung,
melanoma, and breast carcinoma.

Pearls
• At least 50% o brain tumors are rom metastasis.
• The most common etiologies include lung, melanoma,
and breast carcinoma.
• 50% o intracranial metastasis presents as a solitary
lesion.
• The imaging appearance is variable and lesions
enhance with di erent patterns rom solid
enhancement to a “ring” pattern.
• With history o primary carcinoma or signif cant risk
Axial T1 postcontrast image shows heterogeneous contrast actors or cancer, metastasis should be avored.
enhancement within the left frontal lobe lesion (asterisk). • I there is no diagnosis o a primary carcinoma, then
consider body CT in search o the primary tumor.
Answers • Consider in ectious etiology in immunocompromised
patients.
1. The abnormality is located within the le t rontal lobe.
2. The imaging appearance o parenchymal metastasis is Suggested Readings
variable. There ore, there is no classic appearance, as it
Tang YM, Ngai S, Stuckey S. The solitary enhancing
depends on the primary tumor. The majority o lesions
cerebral lesion: can FLAIR aid the di erentiation between
enhance with di erent patterns rom solid enhancement
glioma and metastasis? AJNR Am J Neuroradiol. 2006
to a “ring” pattern with variable adjacent edema. There is
Mar;27(3):609-611.
a predilection or the gray-white junction with relatively
Toh CH, Wei KC, Ng SH, Wan YL, Lin CP, Castillo
circumscribed masses.
M. Di erentiation o brain abscesses rom necrotic
3. The di erential diagnosis includes glioblastoma, glioblastomas and cystic metastatic brain tumors with
metastasis, and abscess. di usion tensor imaging. AJNR Am J Neuroradiol. 2011
Oct;32(9):1646-1651.
90
32-year-old female with head pain after fall

1. Where is the abnormality located?

2. Which MRI sequence is specif cally use ul or


this type o injury?

3. What is the di erential diagnosis?

4. What other CNS f ndings can be associated


with this type o injury?

5. What is the source o the high attenuation?

91
Cortical contusion Case 36 (3109)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT shows focus of hyperattenuation Axial CT image shows focus of Axial T1 image shows focus of high signal
within the anterior right temporal lobe hypoattenuation within the inferior within the anterior right temporal lobe
with surrounding vasogenic edema right frontal lobe (arrow). corresponding with blood products (green
(arrow). arrow). Also seen is a small right subdural
hematoma (red arrow).

3. The di erential diagnosis includes high-density


metastasis, cortical contusion, and glioma.
4. Brain contusions may be associated with subarachnoid
hemorrhage, epidural hematoma, subdural hematoma,
di use axonal injury, and skull ractures.
5. The high attenuation is secondary to hemorrhage.

Pearls
• Brain contusions occur with deceleration injury o the
brain against the skull.
• Contusions are classif ed with calvarial racture, coup,
Axial FLAIR image shows foci of high signal within the inferior and contrecoup injury.
right frontal lobe (white arrow) and involving the right gyrus • Common sites are the anterior temporal and rontal
rectus, not visible on the CT (blue arrow). Right subdural lobes.
hematoma again noted (red arrows). • With contusions less than 5 mm, edema may not be
seen on CT.
• Look or small oci o subarachnoid blood and small
Answers subdural hematomas.
1. The abnormality is located in the right temporal and • Use GRE and SWI MRI sequences to detect trauma-
in erior rontal lobes. associated hemorrhage.
2. Although the entity is likely visible on all pulse
sequences, T2* imaging, such as gradient echo (GRE), is
use ul or visualizing small oci o traumatic blood due Suggested Reading
to the induced arti act o “blooming” (makes the oci o Aiken AH, Gean AD. Imaging o head trauma. Semin
blood appear larger, hence easier to detect). Roentgenol. 2010 Apr;45(2):63-79.

92
57-year-old female with acute onset of left facial droop

1. Where is the abnormality located?

2. Which MRI sequence is the most accurate or


the diagnosis o this entity?

3. What is the di erential diagnosis?

4. What are presenting symptoms or this lesion?

5. What is the treatment or this entity?

93
Basal ganglia infarct Case 37 (3108)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT image shows an area of low Axial DWI and ADC map shows high signal seen within the right basal ganglia on the
attenuation within the right basal ganglia di usion-weighted image (green arrow) with corresponding low signal on the ADC image
a ecting the head of caudate nucleus, (red arrow). This is consistent with cytotoxic edema.
anterior limb of the internal capsule, and
anterior lateral lentiform nucleus (arrow).

Answers
• Bilateral basal ganglia (BG) in arcts consider toxic/
1. The abnormality is located within the right basal ganglia.
metabolic causes.
2. MRI with di usion-weighted imaging has the highest • Cortical in arcts may have gyri orm enhancement.
sensitivity and specif city or acute in arct. • Ring-like enhancement consider tumor or abscess.
• Look or gray matter involvement in the MCA
3. The di erential diagnosis includes CVA, abscess, and
distribution to conf rm BG in arct related to MCA
metastasis.
vessel occlusion.
4. Patients with basal ganglia in arcts usually present with
contralateral muscular weakness.
5. TPA (tissue plasminogen activator) thrombolysis Suggested Readings
can be delivered systemically through IV or directly de Lucas EM, Sánchez E, Gutiérrez A, et al. CT protocol
intra-arterial with microcatheters. Mechanical catheter or acute stroke: tips and tricks or general radiologists.
thrombolysis is also another treatment option. Radiographics. 2008 Oct;28(6):1673-1687.
Saenz RC. The disappearing basal ganglia sign. Radiology.
2005 Jan;234(1):242-243.
Pearls Tomura N, Uemura K, Inugami A, Fujita H, Higano S,
Shishido F. Early CT f nding in cerebral in arction:
• The majority o in arcts are caused by occlusion o a obscuration o the lenti orm nucleus. Radiology. 1988
blood vessel resulting in ischemia (80%). Aug;168(2):463-467.
• The middle cerebral artery (MCA) is the most
commonly a ected vessel.
• Basal ganglia (BG) derive their blood supply rom the
lenticulostriate arteries.
• Restricted di usion must be conf rmed with low signal
on ADC.

94
71-year-old female with a headache

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What MR sequences are help ul to visualize


this entity?

4. What are presenting symptoms or this lesion?

5. What is the treatment or this entity?

95
Developmental venous anomaly Case 38 (3121)
Case ranking/dif culty: Category: Intra-axial supratentorial

Sagittal T1 image shows a frontal lobe Axial T1 image demonstrates the Axial T2 image shows the faint
vascular structure (arrow) with branching vascular malformation that is vascular malformation within the
extending to the level of the left lateral low signal adjacent to the lateral left frontal lobe white matter with
ventricle (upside down umbrella shape). ventricle (arrow). branching pattern (arrow).

Pearls
• Developmental venous anomaly (DVA) is a congenital
vascular mal ormation.
• It is the most common type o vascular mal ormation
seen at autopsy.
• The most common location is adjacent to the rontal
horns.
• Morphologically, it has an upside down “umbrella” or
“Medusa head” appearance.
• Multiple DVAs can be seen with blue rubber-bleb
nevus syndrome.
• Postcontrast imaging and SWI make detection easier.
• I vasogenic edema is seen consider metastasis versus
Sagittal CT with contrast better demonstrates the vascular
DVA associated with cavernous mal ormation and/or
malformation (arrow) within the frontal lobe white matter.
Morphologically, it has an upside down “umbrella” or “medusa recent hemorrhage.
head” appearance.

Suggested Readings
Answers
Fushimi Y, Miki Y, Togashi K, Kikuta K, Hashimoto
1. The abnormality is located within the rontal lobe.
N, Fukuyama H. A developmental venous anomaly
2. A large vein within the parenchyma resembling an presenting atypical f ndings on susceptibility-weighted
upside down “umbrella.” imaging. AJNR Am J Neuroradiol. 2008 Aug;29(7):E56.
Robert T, Villard J, Oumarou G, Daniel RT, Pollo C, Uské
3. On MRI, T1 PD, FLAIR, and T2 the signal intensity is
A. Intracerebellar hemorrhage caused by developmental
variable. The lesions are more readily seen with the use o
venous anomaly, rom diagnosis to treatment. J Neurol
contrast or susceptibility-weighted imaging (SWI) on MRI.
Surg A Cent Eur Neurosurg. 2013 Dec;74(suppl 1):
4. Developmental venous anomalies are usually e275-e278.
asymptomatic but can, on occasion, be associated with
headaches.
5. No treatment is usually necessary or this entity.
96
30-year-old female with neurological de cits, vertigo, and vomiting

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What other f ndings are associated with this


entity?

4. What is the imaging method o choice?

5. What is the treatment or this entity?

97
Cavernous malformation Case 39 (3244)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT image shows high Sagittal T1 image shows mixed signal intensity Axial T2 image shows central
attenuation representing within the posterior right frontal lobe lesion high signal is present within the
calci cation vs subacute blood extending inferiorly into the body of the right lateral posterior right frontal lobe lesion
products in the subcortical ventricle (circle). There is central isointense signal to with peripheral low signal intensity
white matter of the posterior white matter and peripheral low signal intensity. (circle). Central high signal is likely
right frontal lobe (circle). There is late subacute blood products
no signi cant vasogenic edema. within a cystic locule. There is no
signi cant vasogenic edema.

4. MRI with susceptibility weighted or gradient echo


sequences to evaluate or multiple lesions.
5. Larger, symptomatic lesions with higher risk o
rehemorrhage can be removed surgically.

Pearls
• Cavernous mal ormations may occur in combination
with other vascular mal ormations, particularly
developmental venous anomalies.
• “Popcorn” calcif cations typical on noncontrast CT.
• Hemorrhagic lesions can have varying ages o blood
products on MRI.
Axial gradient echo shows blooming artifact primarily lining • Have no vasogenic edema unless recent hemorrhage is
the lesion (circle) consistent with hemosiderin rim. Cavernous
present.
malformations can grow in size with repeated hemorrhages.
• Larger lesions may have central cystic locules with
uid- uid levels.
Answers
• May have minimal to no enhancement.
1. There is a ocal lesion within the subcortical white • Typically not seen on DSA (“angiographically occult”).
matter o the right rontal lobe. • Patients o ten asymptomatic, however can present with
2. Classic f ndings or cavernous mal ormation include seizures when hemorrhage is present.
calcif ed “popcorn” lesion seen on noncontrast CT
containing di erent stages o blood degradation on MRI. Suggested Readings
Larger lesions demonstrate central cystic locules with
Ginat DT, Meyers SP. Intracranial lesions with high signal
possible uid- uid levels and a hemosiderin rim. No
intensity on T1-weighted MR images: di erential
vasogenic edema is seen unless hemorrhage is present.
diagnosis. Radiographics. 2012;32(2):499-516.
3. Other vascular mal ormations may be present, most notably Vilanova JC, Barceló J, Smirniotopoulos JG, et al. Hemangioma
developmental venous anomaly. Cavernous mal ormations rom head to toe: MR imaging with pathologic correlation.
may be singular (sporadic) or multiple ( amilial). Radiographics. 2010 Jul;24(2):367-385.
98
92-year-old female with weakness and dementia

1. Where is the abnormality?

2. What is the di erential diagnosis?

3. What are the most common locations or this


entity?

4. Which vessels supply the basal ganglia?

5. What structures are usually supplied by the


recurrent artery o Heubner?

99
Basal ganglia hemorrhage—hypertensive bleed Case 40 (3317)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT image shows high density within the right caudate Axial CT image shows a uid- uid level that is high density
head (green arrow). Similar high density is identi ed within the within the dependent posterior horn of the left lateral ventricle
anterior horn of the right lateral ventricle (red arrow). There is (red arrow). The right caudate hemorrhage is again seen (circle).
encephalomalacia of the left frontal lobe. There are signi cant
con uent areas of low attenuation involving the periventricular
white matter likely from hypertensive arteriolosclerosis.
Pearls
• Hypertension is the most common cause or
Answers spontaneous intracranial hemorrhage in the elderly
1. The abnormality is located within the right basal ganglia population.
and lateral ventricles. • Hypertensive hemorrhage most commonly involves the
basal ganglia, thalamus, pons, and cerebellum.
2. The di erential diagnosis includes vascular
• Second most common cause o stroke (10%-20% o
mal ormation, tumor-related hemorrhage, and
stroke patients).
hypertensive hemorrhage.
• Check the dependent portions o the lateral ventricles
3. Most common locations or hypertensive hemorrhages or blood.
are the basal ganglia ollowed by the thalamus, pons, and • Check the position o the septum pellucidum to
cerebellum. evaluate or midline shi t.
• Midline shi t (sub alcine herniation) is seen prior to
4. Branches o the A1-segment o the anterior cerebral
transtentorial herniation.
artery (medial lenticulostriate arteries) supply the
anterior in erior parts o the basal nuclei and the
anterior limb o the internal capsule. Branches o the
M1-segment o the middle cerebral artery (lateral Suggested Readings
lenticulostriate arteries) supply the superior part o the Lin WM, Yang TY, Weng HH, et al. Brain microbleeds:
head and the body o the caudate nucleus, most o the distribution and in uence on hematoma and perihematomal
globus pallidus, putamen, and the posterior limb o the edema in patients with primary intracerebral hemorrhage.
internal capsule. Neuroradiol J. 2013 Apr;26(2):184-190.
5. It supplies the head o caudate, anterior portion o the Zheng T, Wang S, Barras C, Davis S, Yan B. Vascular
lenti orm nucleus, and anterior limb o the internal imaging adds value in investigation o basal ganglia
capsule. It originates rom the proximal ACA and is the hemorrhage. J Clin Neurosci. 2012;19(2):277-280.
largest branch vessel o the proximal ACA typically seen Zheng W, Zhang C, Hou D, Cao C. Comparison on di erent
on angiography. strategies or treatments o hypertensive hemorrhage in
the basal ganglia region with a volume o 25 to 35 ml.
Acta Cir Bras. 2012 Oct;27(10):727-731.

100
17-year-old female with acute headache and left neurologic de cit

1. What is the di erential diagnosis?

2. How do patients with this abnormality


typically present?

3. What are the three basic vascular components


o this lesion?

4. What is the key f nding on digital subtraction


angiography or arteriovenous shunting?

5. What is an associated syndrome at risk or


development o AVMs?
101
Arteriovenous malformation Case 41 (3437)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT image shows Axial MIP image from Frontal radiograph of a right Lateral radiograph of a right internal
a large hemorrhage CT angiography shows a internal carotid artery injection carotid artery injection shows a
(arrowhead) centered in prominent draining vein shows a tangle of vessels consistent tangle of vessels consistent with
the right basal ganglia with (arrowhead) medial to the with nidus (arrowhead) with an early nidus (arrowhead) with an early
mild midline shift (arrow). hematoma. draining vein (arrow) emptying into draining vein (arrow) emptying into
the basal vein of Rosenthal. the basal vein of Rosenthal.

Answers
1. Di erential diagnosis or an intraparenchymal Pearls
hemorrhage with associated abnormal vessels includes • Arteriovenous mal ormations (AVM) are abnormal
arteriovenous mal ormation (AVM), dural AV f stula, pial vascular mal ormations with direct shunting rom
and possibly a high-grade neoplasm with signif cant arterial to venous without intervening capillaries.
neoangiogenesis causing shunting. • 2% o cases are associated with syndromes such as
hereditary hemorrhagic telangiectasia (Osler-Weber-
2. The typical prof le is a young adult with headache and
Rendu).
hemorrhage. 50% o AVMs present with headache and
• 50% present with hemorrhage, the remainder with
spontaneous hemorrhage.
seizure or ocal def cit rom vascular steal ischemia.
3. The three basic components o AVMs are enlarged • Three basic components on imaging: enlarged arterial
arterial eeders, compact nidus, and dilated draining eeders, compact nidus o abnormal vessels, and
veins. Intranidal aneurysms (>50%) and eeding artery dilated draining veins.
aneurysms (10-15%) are associated but not necessary. • >50% have intranidal aneurysm.
• Digital subtraction angiography best details the
4. The early draining vein on DSA is the key f nding or
vascular architecture with visualization o the early
arteriovenous shunting without intervening capillary bed
draining vein rom real-time imaging.
seen in AVMs and AV f stulas.
• Treatment includes embolization, surgery, and
5. Although syndromic CNS AVMs are rare (2%), stereotactic radiosurgery.
knowledge o associated syndromes including hereditary • Spetzler-Martin scale assesses risk o surgery.
hemorrhagic telangiectasias, Klippel Trenaunay
Weber, Wyburn-Mason, and capillary mal ormation-
arteriovenous mal ormation syndrome is necessary in a
Suggested Readings
patient with multiple vascular lesions.
Dmytriw AA, Ter Brugge KG, Krings T, Agid R.
Endovascular treatment o head and neck arteriovenous
mal ormations. Neuroradiology. 2014 Mar;56(3):227-236.
Fiehler J, Illies T, Piening M, et al. Territorial and microvascular
per usion impairment in brain arteriovenous mal ormations.
AJNR Am J Neuroradiol. 2009 Feb;30(2):356-361.
Mamourian A, Wallace R. When is an atypical DVA an
AVM? AJNR Am J Neuroradiol. 2009 Feb;30(2):E24;
author reply E25.

102
16-year-old female with headache

1. What is the di erential diagnosis?

2. What is the most common clinical


presentation?

3. What is the common age group with this


diagnosis?

4. What are typical radiographic f ndings?

5. What is the most sensitive modality or


diagnosis?

103
Cerebral venous infarction Case 42 (3438)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT image demonstrates dense tubular structures (dense Axial CT venogram demonstrates intraluminal thrombus in the
cord sign) in the left parietal lobe, suggestive of thrombus in the left transverse/sigmoid sinus junction (black arrowhead).
cortical veins (white arrowheads). A hyperdense focus in the left
parietal lobe (black asterisk) with surrounding vasogenic edema
(white arrow) is consistent with intraparenchymal hemorrhage.
Pearls
• Cerebral venous in arction is relatively uncommon
Answers compared with arterial in arct.
• 20%-25% o cases are unable to identi y an etiology.
1. Hemorrhagic tumor, rom primary neoplasm or metastasis, • More common in emales and young adults.
hemorrhagic arterial in arct, hypertensive hemorrhage, and • Headache is the most common clinical presentation.
amyloid angiopathy can be considered. Venous in arcts • CT is normal in up to 30% o cases.
should be considered in young emales, especially in a • Direct CT signs o CVT: dense triangular clot sign,
pattern not consistent with an arterial territory. cord sign, and postcontrast empty delta sign.
2. Most common presentation or cerebral venous in arct • Indirect CT signs: intense contrast enhancement o the
is headache. Clinical symptoms can be grouped into alx and tentorium, dilated transcerebral veins, small
three clinical syndromes: isolated signs o intracranial ventricles, and parenchymal abnormality.
hypertension, seizures or ocal neurologic def cits, and • MRI with contrast and MR venography (MRV) are
multi ocal signs including encephalopathy, stupor, or coma. the most sensitive imaging modality to demonstrate
thrombus and occluded dural sinus/vein (caution: TOF
3. CVT is more common in young adult emales. MRV is sensitive to ow heterogeneity with loss o
4. Direct CT signs or cerebral venous thrombosis include signal, which may lead to alse positive).
“cord sign” (hyperdensity o thrombosed veins) and • Venous in arcts are T2 hyperintense, prone to hemorrhage
empty delta sign (lack o luminal enhancement in the with variable signal due to evolution o blood.
sagittal sinus). On MRI, susceptibility arti act on T2* • The in arct may be outside o a typical arterial
imaging can be seen o the thrombus and or hemorrhagic distribution, with variable restricted di usion.
in arcts, which are predominantly T2 hyperintense and
may be in a pattern outside typical arterial distributions. Suggested Readings
5. MRI with contrast and MR venography is the most Buyck PJ, De Keyzer F, Vanneste D, Wilms G, Thijs V,
sensitive to evaluate or venous thrombosis and in arct. Demaerel P. CT density measurement and H:H ratio are
Contrast-enhanced vessels in conjunction with time use ul in diagnosing acute cerebral venous sinus thrombosis.
o ight MRV help decrease alse positives rom AJNR Am J Neuroradiol. 2013 Aug;34(8):1568-1572.
noncontrast time o ight MRV, alone, which is highly Leach JL, Strub WM, Gaskill-Shipley MF. Cerebral venous
sensitive to intraluminal ow dynamics. thrombus signal intensity and susceptibility e ects
on gradient recalled-echo MR imaging. AJNR Am J
Neuroradiol. 2007 May;28(5):940-945.
104
68-year-old female with altered mental status

1. What is the di erential diagnosis?

2. What are common clinical presentations o this


disease?

3. What are typical radiographic eatures?

4. What is the best imaging modality?

5. What are other mani estations or associations


o cerebral amyloid disease?

105
Cerebral amyloid angiopathy Case 43 (3441)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT brain image demonstrates a left parietal hematoma Axial GRE image demonstrates multiple punctate foci of
(black asterisk) with minimal surrounding vasogenic edema. Also susceptibility artifacts scattered in the bilateral cerebral hemispheres
noted is a dystrophic calci cation in the left frontal lobe (white (white arrowheads). Also noted are a rim of susceptibility artifacts in
arrowhead) associated with encephalomalacia, related to prior the left frontal as well as layering uid- uid level in the left parietal
hemorrhage. lobe (white arrows). Multiple cortical hemorrhages in an elderly
patient are compatible with amyloid angiopathy.

Answers
1. In addition to amyloid angiopathy, di erential diagnosis • Cerebral amyloid angiopathy (CAA) is the most
includes hypertensive hemorrhage, hemorrhagic common presentation o cerebral amyloid disease,
in arct, bleeding metastasis or primary brain tumor, which also includes uncommon ocal amyloidoma and
coagulopathy, or underlying vascular mal ormation. a rare di use white matter inf ltrating orm.
• Up to 88% Alzheimer patients have CAA.
2. Stroke-like symptoms can be seen in the acute setting, • Stroke-like symptoms in the acute setting and dementia
while dementia is more common in chronic settings. in the chronic setting.
3. Typical radiographic eatures include lobar hemorrhage • Radiographic eatures include lobar hemorrhage,
in the cortex/subcortical white matter, which may usually in the cortical and subcortical white matter.
extend to the ventricle or subarachnoid spaces, multiple • In younger patients with in ammatory CAA may
hematomas o di ering ages, and microhemorrhages. mimic PRES.
• Consider CAA in cases o normotensive demented
4. MRI is modality o choice due to sensitivity to multiple elderly patients older than 60 years old with multiple
ages o hematoma and microhemorrhages. GRE/SWI episodes o intracranial hemorrhage.
sequences are the most sensitive modality to detect • SWI is the most sensitive technique to detect chronic
microbleeds. microbleeds.
5. Cerebral amyloid angiopathy is the most common
mani estation o cerebral amyloid disease, and is seen in Suggested Readings
up to 88% o Alzheimer patients. Other mani estations
include uncommon ocal amyloidoma and a rare di use Chao CP, Kotsenas AL, Broderick DF. Cerebral amyloid
white matter leukoencephalopathic orm. angiopathy: CT and MR imaging f ndings. Radiographics.
2009 Dec;26(5):1517-1531.
Haacke EM, DelProposto ZS, Chaturvedi S, et al. Imaging
cerebral amyloid angiopathy with susceptibility-weighted
Pearls imaging. AJNR Am J Neuroradiol. 2007 Feb;28(2):316-317.
• A cerebrovascular disorder secondary to deposition o Wagle WA, Smith TW, Weiner M. Intracerebral hemorrhage
amyloid beta peptide deposits within small- to medium- caused by cerebral amyloid angiopathy: radiographic-
sized blood vessels o the brain and leptomeninges. pathologic correlation. AJNR Am J Neuroradiol. 1990
May;5(2):171-176.
106
70-year-old male with headache and confusion

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the most common arterial distribution


involved in this entity?

5. What is the treatment or this entity?

107
Anterior cerebral artery infarct Case 44 (3527)
Case ranking/dif culty: Category: Intra-axial supratentorial

Pearls
• Anterior cerebral artery (ACA) is the least common
“circle o Willis” vessel responsible or in arcts.
• Maximum cytotoxic edema occurs at 48 to 72 hours.
• DWI is highly sensitive and specif c or acute in arct in
the f rst 7-10 days.
• “Mohawk” sign is cytotoxic edema involving the
medial rontal lobe corresponding to the ACA territory.
• May have gyri orm enhancement secondary to “luxury
per usion.”
• Susceptibility-weighted imaging may show
“blooming” with acute clot and parenchymal
hemorrhage.
• High FLAIR signal within the artery may be seen
with slow ow and thrombus.
• CTA or MRA to evaluate or arterial thrombus/
occlusion.

Suggested Readings
King S, Khatri P, Carrozella J, et al. Anterior cerebral artery
emboli in combined intravenous and intra-arterial rtPA
treatment o acute ischemic stroke in the IMS I and II trials.
Axial CT shows low attenuation involving the gray matter and
AJNR Am J Neuroradiol. 2008 Mar;28(10):1890-1894.
subcortical white matter with loss of the gray-white discrimination
of the medial aspect of the superior frontal gyrus (arrows). Moussouttas M, Boland T, Chang L, Patel A, McCourt
J, Malten ort M. Prevalence, timing, risk actors, and
mechanisms o anterior cerebral artery in arctions
Answers ollowing subarachnoid hemorrhage. J Neurol. 2013
1. The abnormality is located within the medial right Jan;260(1):21-29.
rontal lobe, along the anterior cerebral artery Park YW, Kim CH, Kim MO, Jeong HJ, Jung HY. Alien hand
distribution. syndrome in stroke—case report & neurophysiologic
study. Ann Rehabil Med. 2012 Aug;36(4):556-560.
2. Anterior cerebral artery (ACA) in arcts have the same
f ndings as other arterial strokes. CT signs o in arction
include obscuration o the gray-white junctions and
high density within the vessels. On MR, there is high
signal on the T2 images (including PD and T2 FLAIR)
with corresponding T1 low signal in cytotoxic edema.
Di usion-weighted imaging (DWI) demonstrates
increased signal, corresponding with decreased ADC
rom restriction.
3. The di erential diagnosis includes anterior cerebral
artery in arction, metastasis, and cerebritis.
4. Anterior cerebral artery (ACA) in arcts are the least
common “circle o Willis” vessel in in arcts. The most
common etiology o ischemic stroke is MCA in arct.
5. The typical treatment or this entity is supportive care
with consideration or thrombolytic therapy.

108
68-year-old female with headache and visual eld defects

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the likely etiology o this entity?

5. What is the treatment or this entity?

109
Posterior cerebral artery infarct Case 45 (3532)
Case ranking/dif culty: Category: Intra-axial supratentorial

Sagittal T1 image shows the gyri Axial T2 image shows the gyri of the Coronal FLAIR shows the gyri
of the inferior right temporal lobe right mesial temporal lobe and occipital of the right occipital lobe
with enlargement, gray-white lobe with high T2 signal consistent with high T2 signal from
blurring, and low signal (arrow). from cytotoxic edema (arrows). cytotoxic edema (arrow).

Pearls
• Posterior cerebral artery (PCA) in arcts account or
5%-10% o ischemic strokes.
• PCA high FLAIR signal can be seen with slow ow
and thrombus.
• PCA supplies the medial occipital, temporal lobes,
thalamus, hypothalamus, and posterior limb o the
internal capsule.
• Herpes cerebritis should be considered with
Axial DWI b-1000 and ADC show the gyri of the right temporal involvement o the temporal lobe, insular cortex, and
lobe and right occipital lobe with DWI high signal (green arrow) in erior rontal lobe with deep white matter sparing.
and corresponding low signal on the ADC map (red arrow) • The most common etiology o PCA in arcts is
con rming cytotoxic edema and acute PCA infarct. thrombosis related to atherosclerotic disease.
• Transtentorial herniation may also cause bilateral
Answers PCA in arction secondary to compression between the
1. The abnormality is located within the right temporal lobe temporal lobe and the tentorium.
and occipital lobe. • In arction o the medial occipital lobe causes
2. CT signs o in arction include obscuration o the gray- homonymous hemianopsia (a visual f eld de ect involving
white junctions, sulcal e acement, and high density either the le t or right halves o the visual f eld).
within the vessels. With regard to acute stroke imaging, • Consider CTA or MRA to evaluate or arterial
noncontrast MRI is more accurate than CT. On MR, there thrombus/occlusion.
is high signal on the T2 images (including PD and T2
FLAIR) with corresponding T1 low signal. Specif cally, Suggested Readings
di usion-weighted imaging (DWI) is the most accurate
Cereda C, Carrera E. Posterior cerebral artery territory
imaging sequence or detecting acute in arcts.
in arctions. Front Neurol Neurosci. 2012 Jul;30(30):128-131.
3. The di erential diagnosis includes in arction, metastasis, Förster A, Gass A, Kern R, Wol ME, Hennerici MG, Szabo
and cerebritis. Metastasis is typically multi ocal and K. MR imaging-guided intravenous thrombolysis in
associated with vasogenic edema. Cerebritis o the posterior cerebral artery stroke. AJNR Am J Neuroradiol.
temporal lobe is common with herpes simplex encephalitis. 2011 Feb;32(2):419-421.
4. The most common etiology o PCA in arcts is Seo KD, Lee KO, Choi YC, Kim WJ, Lee KY. Fluid-
thrombosis related to atherosclerotic disease. attenuated inversion recovery hyperintense vessels in
posterior cerebral artery in arction. Cerebrovasc Dis
5. The treatment or this entity is typically supportive with
Extra. 2013 Dec;3(1):46-54.
consideration or thrombolytic therapy.
110
45-year-old female with syncope and hypotension

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the etiology o this entity?

5. What is the treatment or this entity?

111
Watershed infarct Case 46 (3533)
Case ranking/dif culty: Category: Intra-axial supratentorial

3. The di erential diagnosis includes border zone (watershed)


in arct, embolic in arct, PRES, and cerebritis.
4. Watershed in arcts are typically secondary to vascular
disease, arterial stenosis, hypotension, and hemodynamic
compromise.
5. The treatment or this entity is typically supportive
with correction o hypotension and consideration o
antithrombolytics or occlusive disease.

Pearls
• Watershed in arcts are also called border zone in arcts.
• Between major arterial territories (ACA, MCA, PCA)
in the cortex and subcortical white matter.
• Between per orating arteries (parasagittal deep white
matter), “string o beads” appearance.
• When watershed injuries are bilateral the etiology is
usually secondary to hypotension.
• Unilateral may be rom vessel stenosis.

Suggested Reading
Axial CT image shows a wedge-shaped area of low attenuation Mangla R, Kolar B, Almast J, Ekholm SE. Border zone
located between the right occipital and parietal lobes, between in arcts: pathophysiologic and imaging characteristics.
the MCA and PCA arterial distribution (asterisks). Note there is an Radiographics. 2011 Oct;31(5):1201-1214.
area of encephalomalacia within the left occipital lobe within the
contralateral watershed territory (arrowhead).

Answers
1. The abnormality is located within the arterial watershed
region between major vascular territories o the MCA
and PCA involving the right parietal and occipital lobes.
2. Watershed in arcts are diagnosed on imaging based
on their location either in wedge-shaped cortical
in arcts between major vascular territories or in the
deep white matter. The CT signs o in arction include
hypodensity, obscuration o the gray-white junctions,
and sulcal e acement. On MR, there is high signal
on the T2 images (including PD and T2 FLAIR) with
corresponding T1 low signal. Di usion-weighted
imaging (DWI) shows high signal corresponding with
low signal on apparent di usion coe f cient (ADC) map.

112
68-year-old female with headache after fall

1. Where is the abnormality located?

2. What is the age o the injury?

3. What is the di erential diagnosis?

4. What is the signif cance o mixed attenuation


within this entity?

5. What injured anatomic structure is the cause o


the high attenuation?

113
Subdural hematoma Case 47 (2983)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Axial CT image at the level of the basal ganglia demonstrates Axial CT image at the level of the basal ganglia demonstrates
a right extraaxial uid collection (arrows). The uid is of mixed the extraaxial uid collection extending along the posterior falx
attenuation with higher density seen posteriorly. (green arrow). Mixed density right hemispheric crescentic SDH
is again seen (blue arrows). A focal right chronic lacunar basal
ganglia infarct is also noted (red arrow).
Answers
1. Given the appearance o the extraaxial collection
(crescent shape) and because it crosses suture lines it is
in the subdural space. Pearls
• Subdural hematomas (SDH) occur most commonly
2. The layering hyperdensity may suggest an acute on
secondary to trauma.
subacute hemorrhage. The increased attenuation when
• Crescent in shape.
compared to the brain parenchyma indicates there is an
• SDH may be subdivided into three categories based on
acute component.
age: acute 1-7 days, subacute 1-3 weeks, and chronic
3. The di erential diagnosis or extraaxial hemorrhages older than 3 weeks.
includes subdural hematoma, epidural hematoma, and • Mixed attenuation indicates rebleeding o a chronic
subarachnoid hemorrhage. injury or acute clotted and nonclotted blood products.
• Majority are caused by bridging vein injury.
4. The signif cance o mixed attenuation involving SDH
• Contrecoup location most common.
usually represents the presence o hyperacute blood in an
acute hematoma or acute on chronic blood products.
5. The most common source or blood products seen in
Suggested Reading
SDH is the bridging veins.
Aiken AH, Gean AD. Imaging o head trauma. Semin
Roentgenol. 2010 Apr;45(2):63-79.

114
43-year-old female with head pain after blow to head

1. Where is the abnormality located?

2. What pathology is usually associated with this


entity?

3. What is the di erential diagnosis?

4. What is the signif cance o mixed attenuation


within this entity?

5. What is the most common source o the high


attenuation?

115
Epidural hematoma Case 48 (2984)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Axial CT image shows a lenticular (convex) extraaxial uid This 3D reconstructed image of the skull demonstrates a fracture
collection adjacent to the right hemisphere (arrow). (green arrow) traversing the right parietal bone, joining the
squamosal suture (red arrow).

3. The di erential diagnosis or extraaxial hemorrhage


includes epidural hematoma, subdural hematoma, and
subarachnoid hemorrhage.
4. Mixed attenuation within an epidural hematoma is
usually indicative o mixed clotted and unclotted blood
products. This may indicate an expanding hematoma.
5. The most common source o the high attenuation (blood)
is arterial injury.

Pearls
• Epidural hematomas (EDH) occur most commonly
secondary to trauma.
• Lenticular shape.
• Bound by sutures.
• Can cross midline.
Axial CT bone window image demonstrates a ne fracture line
• High association with skull racture.
traversing the right parietal bone (arrow) overlying the epidural • Primarily middle meningeal arterial injury, although
hematoma. can be rom torn dural venous sinus in the minority o
cases.

Answers
1. The abnormality is in the epidural space. This is evident Suggested Reading
given the characteristic lenticular shape.
Aiken AH, Gean AD. Imaging o head trauma. Semin
2. EDH is usually associated with a skull racture. Roentgenol. 2010 Apr;45(2):63-79.

116
30-year-old female with history of headache, nausea, vomiting,
and dizziness

1. What is the di erential diagnosis?

2. What are the characteristic radiographic


f ndings or this disease?

3. Which chromosome has an abnormality in this


disease?

4. What are criteria or the diagnosis o this


disease?

5. What is the primary treatment option or this


disease?

117
Neuro bromatosis type 2 Case 49 (3220)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Axial T1 postcontrast image shows a small homogeneously Axial FLAIR image shows a large extraaxial mass in the left
enhancing lesion in the frontal falx consistent with meningioma occipital region (arrow) with homogeneous hyperintensity
(arrow). and well-circumscribed borders with little adjacent edema
(arrowhead).

Axial T1 postcontrast image shows the extraaxial mass with


homogeneous enhancement consistent with a meningioma Axial T1 postcontrast image shows bilateral enhancing masses in
(arrow). the internal auditory canals (arrows), consistent with vestibular
schwannomas, which is diagnostic of neuro bromatosis type 2.

118
Neuro bromatosis type 2 (Cont.) Case 49 (3220)

Sagittal postcontrast T1 image shows moderately enhancing


Axial T1 postcontrast image shows a mixed enhancing mass in mass in the medulla with a dorsal cyst (arrow) just superior to a
the medulla (arrow) with a dorsal cyst (arrowhead) consistent with homogeneous intensely enhancing mass at the cranial cervical
an ependymoma. junction consistent with a cervical meningioma (arrowhead).

Answers Pearls
1. The di erential diagnosis or multiple enhancing • Neurocutaneous syndrome; 50% autosomal dominant
primarily extraaxial masses includes neurof bromatosis inheritance and 50% new mutation.
2, schwannomatosis, and multiple meningiomatosis. • Chromosome 22q12 abnormalities.
2. The radiographic eatures represent the mnemonic • De ect in Merlin gene, causing truncated Merlin
“MISME” multiple inherited schwannomas, protein production, a tumor suppression protein.
meningiomas, and ependymomas. • “MISME”—multiple inherited schwannomas
meningiomas and ependymomas.
Patients with suspected eatures o NF2 need a care ul
• Less common than NF1, less cutaneous mani estation.
evaluation o other cranial nerves and entire neuraxis.
• Diagnostic criteria includes:
3. NF2 gene is located on chromosome 22q12 and encodes • Bilateral vestibular schwannomas
or the Merlin protein, with tumor suppression unction. • First degree relative with NF2 and one vestibular
schwannoma
4. Criteria or diagnosis or NF2 include bilateral vestibular
• First degree relative with NF2 and two o the
schwannomas, or f rst degree relative with NF2 and one
ollowing f ndings: neurof broma, meningioma,
vestibular schwannoma, or f rst degree relative with
glioma, schwannoma, or posterior subcapsular
NF2 and two o the ollowing tumors: neurof broma,
lenticular opacity.
meningioma, schwannoma, glioma, or posterior
subcapsular lenticular opacity.
5. Surgical resection is the primary option or symptomatic Suggested Readings
tumors. Adjuvant chemotherapy has shown some Aboukais R, Zairi F, Baroncini M, et al. Intracranial
decrease in tumor size in trials or unresectable tumor. meningiomas and neurof bromatosis type 2. Acta Neurochir
Radiation therapy is controversial due to potential (Wien). 2013 Jun;155(6):997-1001; discussion 1001.
increased incidence o treatment-induced tumor. Koontz NA, Wiens AL, Agarwal A, Hingtgen CM, Emerson
RE, Mosier KM. Schwannomatosis: the overlooked
neurof bromatosis? AJR Am J Roentgenol. 2013
Jun;200(6):W646-W653.
119
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12-year-old female with painless lump on head

1. What is the di erential diagnosis?

2. What are the classic imaging f ndings or this


entity?

3. What are common locations or this disease?

4. What is the most common syndrome


associated with this entity?

5. What are possible clinical f ndings in patients


with this entity?

121
Fibrous dysplasia Case 50 (3581)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Axial CT image bone algorithm shows an expansile lesion Axial CT brain window shows the sparing of the inner table and
primarily in the frontal bone with ground glass matrix (asterisk) no signi cant mass e ect to the underlying brain parenchyma
and narrow zone of transition. There is crossing of the left coronal (arrowheads).
suture (arrow) with relative sparing of the inner table (arrowheads).

Answers Pearls
1. The di erential or an expansile bony lesion includes • CT with bone algorithm help ul to assess bony matrix,
f brous dysplasia (FD), blastic metastasis, nonossi ying and local extent o disease.
f broma, Paget disease, and sclerosing osteomyelitis. • MRI may have variable appearance and enhancement,
2. Classic f ndings or FD include an expansile bony lesion which may con use the diagnosis, but can be help ul
with narrow zone o transition and ground glass matrix. or extent o disease and marrow involvement.
When the skull is a ected, there is relative inner table • Variable PET and bone scan uptake, although bone
sparing. Mixed lytic and sclerotic f ndings can be seen in scan can be help ul in nonspecif c evaluation o extent
“pagetoid” FD, and homogeneous sclerosis is seen in up o polyostotic disease.
to 1/3 o cases. • Ground glass matrix classic.
• Mixed lytic and sclerosis in “pagetoid” FD.
3. The three most common sites o monostotic FD involve • Homogenous sclerosis can also be seen.
the ribs, proximal emur, and cranio acial bones with
the orbit, skull base, mandible, and maxilla commonly
involved in cranio acial disease. Polyostotic orms may
be monomelic, involving one side o an extremity. Suggested Readings
Lui YW, Dasari SB, Young RJ. Sphenoid masses in children:
4. 3% o cases o FD are associated with McCune-Albright
radiologic di erential diagnosis with pathologic correlation.
syndrome, which has the classic triad o polyostotic FD,
AJNR Am J Neuroradiol. 2011 Apr;32(4):617-626.
ca e-au-lait spots, and precocious puberty. Monostotic
Sirvanci M, Karaman K, Onat L, Duran C, Ulusoy OL.
FD can occur in McCune-Albright syndrome. Mazabraud
Monostotic f brous dysplasia o the clivus: MRI and CT
syndrome is a rare disease with polyostotic FD and
f ndings. Neuroradiology. 2002 Oct;44(10):847-850.
muscular myxomas.
5. Presenting symptoms include swelling and de ormity
with pathological ractures contributing to pain. Cranial
neuropathy can be seen along with proptosis rom
skull base and orbital involvement. Multiple endocrine
disorders associated with polyostotic f brous dysplasia
can be seen with or without the setting o McCune-
Albright syndrome. Increased risk o sarcomatous
trans ormation has been described with radiation.
122
18-year-old female with headache and neck sti ness

1. What is the biochemical makeup o the T1


bright substance?

2. What are typical MRI f ndings or this lesion?

3. How does this lesion develop?

4. What is a li e-threatening consequence o this


lesion?

5. What can cause chemical meningitis?

123
Chemical meningitis from ruptured dermoid Case 51 (3245)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Axial T2 image at the suprasellar cistern shows a hyperintense, Axial T1 image at the suprasellar cistern shows T1 hyperintensity
heterogeneous, extraaxial mass displacing the right MCA (arrow). following fat of the extraaxial lesion (white arrow). There are also
extraaxial foci of T1 hyperintensity suggesting rupture (arrowheads).

Axial T1 shows multiple extraaxial hyperintense fat droplets from


rupture (arrowheads) in this patient with signs and symptoms of Axial gradient echo shows suppression of these subarachnoid fat
chemical meningitis. Note the fat uid level of the droplet in the droplets (arrowheads) from dermoid rupture.
right lateral ventricle, which is nondependent (arrows).

124
Chemical meningitis from ruptured dermoid (Cont.) Case 51 (3245)

Coronal T1 postcontrast with fat suppression con rms the fatty Axial CT image of the head at the suprasellar cistern con rms the
nature of the mass (arrow); there is no central enhancement. low-density fatty nature of this extraaxial dermoid (arrow) and
associated rupture into the subarachnoid space (arrowheads).

Answers 4. Chemical meningitis rom dermoid cyst rupture is the


1. In this case o ruptured dermoid cyst, the T1 most well-known potentially li e-threatening sequela
hyperintensity is rom at. o this lesion. Chemical meningitis may vary rom mild
and sel -limited to severe and li e threatening. Seizures
2. Dermoid cysts are extraaxial lesions that tend to be have also been described with dermoids with or
midline. There is typically heterogeneity within the cyst without rupture. Larger masses may cause obstructive
with atty elements and sometimes at- uid levels both in hydrocephalus. Malignant degeneration is a rare but
the cyst and in the ventricles i there is rupture. Capsular described risk o dermoid and epidermoid cysts.
enhancement is variable, but no internal enhancement
is expected. There is decreased signal on sequences that 5. Causes o chemical meningitis can be blood products,
naturally have at suppression such as STIR or GRE. post-neurologic surgery, and rupture o intracranial
cystic neoplasms, such as epidermoid and dermoid
3. Dermoid and epidermoid cysts are thought to arise rom cysts, craniopharyngiomas, and Rathke cle t cyst. Other
sequestered portions o sur ace ectoderm included within reported causes include epidural injection o steroids or
neural tube closure during third to f th weeks o embryo bupivacaine, and intrathecal injection o contrast material.
development.

125
Chemical meningitis from ruptured dermoid (Cont.) Case 51 (3245)

Suggested Readings
Pearls Burke JW, Podrasky AE, Bradley WG. Meninges: benign
• Chemical meningitis can be caused by various postoperative enhancement on MR images. Radiology.
etiologies o intracranial biochemical irritation o 1990 Jan;174(1):99-102.
the meninges and include blood products, post- Smirniotopoulos JG, Chiechi MV. Teratomas, dermoids, and
neurological surgery, rupture o intracranial cystic epidermoids o the head and neck. Radiographics. 1995
tumors, and epidural medication. Nov;15(6):1437-1455.
• MR imaging o chemical meningitis may not be
sensitive to detect meningeal enhancement in all cases,
with clinical history and CSF analysis required.
• Dermoid cysts are congenital predominantly midline
lesions that can rupture into the subarachnoid space.
• Fat suppression techniques can be help ul to conf rm
the atty nature o the cyst to distinguish dermoids
rom epidermoids.
• Fat- uid level within the ventricles and in the cyst is
also help ul to conf rm the presence o at.
• Dermoids tend to be more heterogeneous than
lipomas.
31-month-old female with diabetes insipidus

1. What is the di erential diagnosis or the


suprasellar lesion?

2. What is the di erential o the calvarial lesions?

3. What is the prognosis o this disease?

4. What is the treatment or this disease?

5. What organ system is the most commonly


involved?

127
Langerhans cell histiocytosis Case 52 (3247)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Sagittal T1 postcontrast of the midline shows a suprasellar


enhancing mass involving the infundibulum (arrow).

Axial T1 image postcontrast shows an enhancing soft tissue mass


focally destroying the left parietal calvarium (arrow).

Axial T1 image postcontrast shows mass-like enhancement of the


right temporal bone (arrow) in this patient with Langerhans cell
Axial CT image with bone windows shows a lytic lesion of the
histiocytosis.
high right parietal calvarium with a “beveled edge” (arrow).

128
Langerhans cell histiocytosis (Cont.) Case 52 (3247)

Multi ocal multisystem—previously Letterer-Siwe


disease with multiorgan involvement and 50% mortality
even with aggressive chemotherapy.
4. Systemic steroids and/or chemotherapy are used in
multi ocal disease. Surgical excision and ocal radiation
can be used on isolated lesions.
5. Skeletal lesions are the most common mani estation o
Langerhans cell histiocytosis.

Pearls
• Langerhans cell histiocytosis is an in ammatory or
neoplastic process more commonly seen in children.
• LCH is in the di erential o single or multiple bony
lytic lesions in a child, especially with diabetes
insipidus.
• CNS involvement o LCH (multi ocal unisystem
or Hand-Schüller-Christian) is typically seen in the
pituitary stalk and hypothalamus with enhancement
and mild T2 hyperintensity.
• Rare enhancing masses involving the choroid
plexus, leptomeninges, and basal ganglia have been
Axial CT image of the head with bone windows shows a lytic described.
mass of the right temporal bone (arrow), a common location for • Rare cerebellar white matter demyelination has also
Langerhans cell histiocytosis.
been reported.

Answers
1. Di erential o solid-enhancing masses o the pituitary Suggested Readings
stalk include Langerhans cell histiocytosis, germinoma, Chung EM, Murphey MD, Specht CS, Cube R,
and lymphocytic hypophysitis. Smirniotopoulos JG. From the archives o the AFIP.
2. With multiple lytic calvarial lesions in children, Pediatric orbit tumors and tumorlike lesions: osseous lesions
Langerhans cell histiocytosis ollowed by metastatic o the orbit. Radiographics. 2008 Aug;28(4):1193-1214.
disease o childhood malignancies should be considered. D’Ambrosio N, Soohoo S, Warshall C, Johnson A, Karimi S.
TB can present with multiple bone lesions especially Cranio acial and intracranial mani estations o langerhans
in endemic areas. Medulloblastoma does not usually cell histiocytosis: report o f ndings in 100 patients. AJR
metastasize to the bone, even late in the disease. Am J Roentgenol. 2008 Aug;191(2):589-597.

3. LCH was previously divided into three named subtypes,


which are now classif ed by systemic involvement:
Uni ocal—previously eosinophilic granuloma, typically
involves one or multiple lesions in the bone without
extraskeletal involvement, with excellent prognosis.
Multi ocal unisystem—previously Hand-Schüller-
Christian disease with 50% involvement o the pituitary
stalk, as well as scalp and calvarial involvement.
Diabetes insipidus, exophthalmos, and lytic bone
lesions is considered the Hand-Schüller-Christian triad.
Prognosis is variable with 30% o complete remission,
60% with chronic course, and 10% mortality.
129
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12-year-old male with fever, facial pain, headache, and neck sti ness

1. What is the di erential diagnosis?

2. What imaging sequence has the greatest


sensitivity or this disease?

3. What is the most common etiology o this


disease in adults?

4. What is the most common etiology o this


disease in in ants?

5. What procedure is potentially lethal in this


disease?
131
Empyema Case 53 (3512)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Axial FLAIR image shows Axial T1 postcontrast image shows Axial DWI ADC map shows
hyperintense collections in the the epidural collection is super decreased di usion of
right frontal epidural space cial to the enhancing dura (arrow) the epidural and subdural
(arrow), which extends up to the while the subdural collection has collections (arrows).
anterior falx and a hemispheric enhancement in the dura and
subdural collection (arrowhead) leptomeninges (arrowheads).
with similar characteristics.
5. Lumbar puncture has been associated with rapid decline
and death in patients with empyema.

Pearls
• Empyema is a collection o pyogenic material in the
subdural or epidural space, with subdural being more
common.
• Majority o cases are rom paranasal sinus disease
(supratentorial) or mastoiditis (in ratentorial)
• Empyema in in ants is more common rom bacterial
meningitis.
• Complications such as cerebritis, abscess,
Sagittal T1 postcontrast shows the epidural empyema (arrow) hydrocephalus, and sinus thrombosis are more likely
associated with sinusitis (arrowhead). with subdural than with epidural empyema.
• Imaging typically shows peripherally enhancing
Answers extraaxial uid collection without FLAIR suppression
(hyperintense).
1. The di erential or extraaxial uid collections includes • Restricted di usion is typical.
subdural hematomas, hygromas, and e usions. Thick • Empyema is an emergency necessitating neurosurgical
enhancement should suggest empyema or dural drainage.
metastasis. • Lumbar puncture can be atal.
2. Restricted di usion is typical o subdural empyema and
is the most sensitive modality.
Suggested Readings
3. Paranasal sinusitis or mastoiditis accounts or 75%
o the cases o empyema in adults and older children. Han KT, Choi DS, Ryoo JW, et al. Di usion-weighted
Paranasal sinusitis is the cause in 2/3 o cases leading to MR imaging o pyogenic intraventricular empyema.
supratentorial empyema while mastoiditis occurs in 20% Neuroradiology. 2007 Oct;49(10):813-818.
with in ratentorial empyema. Nickerson JP, Richner B, Santy K, et al. Neuroimaging
o pediatric intracranial in ection—part 1: techniques
4. Bacterial meningitis is the most common etiology o and bacterial in ections. J Neuroimaging. 2012
empyema in in ants. Apr;22(2):e42-e51.
132
73-year-old male with headaches and enlarging hat size

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What are presenting symptoms or this lesion?

5. What is the treatment or this entity?

133
Paget disease Case 54 (3560)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Lateral skull radiograph shows areas of patchy increased density Technetium 99m MDP bone scan shows marked radiotracer
involving the skull both anteriorly and posteriorly (circles). Note uptake involving the skull (arrow).
that the diploic space is widened with increased density (arrow).
Increased density is also noted involving the skull base and oor
of the anterior cranial fossa. Pearls
• Paget disease has three phases: lytic phase (bone
Answers resorption), mixed phase (gradual return o yellow
marrow), and blastic phase (sclerosis).
1. Areas o patchy increased density are seen involving
• Paget disease is most common in the axial skeleton and
the skull both anteriorly within the rontal bone and
polyostotic.
posteriorly within occipital bone.
• The skull is involved in 25%-65% o cases.
2. The skull in the early phase o Paget disease • Osteoporosis circumscripta is seen early in the lytic
demonstrates ocal areas o lucency involving the rontal phase.
and occipital bones termed osteoporosis circumscripta. • Later in disease “cotton wool” skull is seen.
Later, osteoblastic activity dominates and ocal, patchy • Sarcomatous trans ormation rare especially in skull
opacities are seen termed “cotton wool” skull. Marked (<1%).
thickening o the diploic space with thickening o the • Sarcomatous degeneration hallmarks include cortical
inner table and resultant enlargement may also be seen destruction and so t tissue mass.
called “tam-o-shanter” skull. Platybasia with basilar • Giant cell tumor trans ormation shows a lytic lesion
invagination may be seen. with marrow replacement without periosteal reaction
or so t tissue mass.
3. The di erential diagnosis includes Paget disease,
metastasis, and renal osteodystrophy.
Suggested Readings
4. Patient symptoms include pain, increased bone size, bowing
de ormities, cranial nerve def cits ( rom compression), Love C, Din AS, Tomas MB, Kalapparambath TP, Palestro
and decreased range o motion. Patients will have elevated CJ. Radionuclide bone imaging: an illustrative review.
serum alkaline phosphatase during the mixed phase. Radiographics. 2007 Oct;23(2):341-358.
Secondary osteoarthritis, gout, CPPD, and rheumatoid Smith SE, Murphey MD, Motamedi K, Mulligan ME,
are all associated with Paget disease. Sarcomatous Resnik CS, Gannon FH. From the archives o the AFIP.
trans ormation is rare (most commonly osteosarcoma). Radiologic spectrum o Paget disease o bone and its
complications with pathologic correlation. Radiographics.
5. The goal o treatment is pain control and is achieved via 2007 Oct;22(5):1191-1216.
the inhibition o bone resorption. The medications used Tjon-A-Tham RT, Bloem JL, Falke TH, et al. Magnetic
include calcitonin, bisphosphonates, mithramycin, and resonance imaging in Paget disease o the skull. AJNR Am
gallium nitrate. J Neuroradiol. 2008 Jan;6(6):879-881.
134
74-year-old female with severe headache and visual changes

1. Where is the abnormality?

2. What is the di erential diagnosis?

3. In which diseases can ocal hyperostosis also


be seen?

4. What percentage o these lesions are


symptomatic?

5. With which syndromes or history is an increased


incidence o meningiomas associated?

135
Meningioma Case 55 (3211)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Sagittal T1 postcontrast image shows a homogeneously Axial FLAIR image shows the interhemispheric lesion with iso- to
enhancing extraaxial lesion above the planum sphenoidale and slightly hyperintense signal to gray matter (asterisk).
extends posteriorly into the sella turcica (asterisk). This lesion
causes mass e ect on the diaphragma sellae and there is an
associated empty sella.
Pearls
• Meningiomas are neoplasms that arise rom the
Answers
meningeal arachnoid cells.
1. This is an extraaxial lesion with mass e ect on the optic • Meningioma may grow very large and can demonstrate
chiasm sitting on the planum sphenoidale. There is dural venous sinus invasion as well as intraosseous
extension into the sella turcica. extension.
2. The di erential includes dural metastasis, • Most common extraaxial CNS tumor.
hemangiopericytoma, and meningioma. • Key imaging f ndings include extraaxial lesion with
associated hyperostosis o adjacent inner table,
3. Focal hyperostosis is present with meningioma, bright/homogenous enhancement, and occasional
f brous dysplasia, Paget disease, Dyke-Davido - calcif cations.
Mason syndrome, and hyperostosis rontalis interna. • Consider NF2 when a young patient presents with a
Generalized skull thickness occurs with chronic severe meningioma.
anemia, acromegaly, osteopetrosis, Camurati-Engelmann • Meningiomas may grow during pregnancy.
disease, and hyperparathyroidism.
4. Less than 10% o all meningiomas are symptomatic.
Suggested Readings
5. Neurof bromatosis 2, basal cell nevus syndrome, and
prior radiation therapy are associated with increased Buetow MP, Buetow PC, Smirniotopoulos JG. Typical,
incidence o meningiomas. atypical, and misleading eatures in meningioma.
Radiographics. 1991 Nov;11(6):1087-1106.
Wang CW, Li YY, Zhu SG, et al. Surgical management and
evaluation o prognostic actors in uencing postoperative
visual outcome o suprasellar meningiomas. World
Neurosurg. 2011 Feb;75(2):294-302.
Wong RH, Wong AK, Vick N, Farhat HI. Natural history o
multiple meningiomas. Surg Neurol Int. 2013 Nov;4(4):71.

136
57-year-old woman with a headache

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What are common etiologies or this f nding?

5. What is the treatment or this entity?

137
Skull metastasis Case 56 (3494)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Sagittal T1 noncontrast image shows Sagittal T1 postcontrast image shows Axial T1 postcontrast shows the clivus
multiple, irregular, low T1 signal lesions the multiple low T1 signal lesions within lesion with more obvious contrast
within the clivus and occipital bone (arrows). the calvarium on precontrast imaging enhancement with fat saturation (arrow).
demonstrate contrast enhancement (arrows).

5. The treatment depends on the primary carcinoma


and i the skull metastases are symptomatic. A
combination o radiotherapy (usually or single lesions)
and chemotherapy (typically aimed at systemic or
widespread disease) is typically utilized.

Pearls
• The most common skull metastases in adults include
breast, lung, prostate, renal cell, ollicular thyroid,
melanoma, and multiple myeloma.
• Direct spread rom head and neck carcinomas into the
skull base may be seen (squamous cell, lymphoma, and
adenoid cystic carcinoma).
Axial T1 postcontrast image shows the posterior skull lesions with
• Postcontrast at saturation images increase sensitivity.
more obvious contrast enhancement with fat saturation (arrows). • Lytic metastasis is more common.
• I a low T1 signal bone lesion is detected without a
primary carcinoma history, then urther evaluation
Answers must be per ormed in order to f nd the primary tumor.
• Consider whole-body bone scan to assess the extent o
1. The abnormality is located within the clivus and
osseous involvement.
posterior skull (occipital and parietal bones).
• High T1 signal bone lesions are likely benign.
2. On MR, the classic f ndings o osseous metastasis are
ocal low T1 signal that is lower than skeletal muscle
and demonstrates enhancement. The enhancement is Suggested Readings
variable. There is also variable T2 signal depending on Abdel Khalek Abdel Razek A, King A. MRI and CT o
the primary neoplasm. nasopharyngeal carcinoma. AJR Am J Roentgenol. 2012
Jan;198(1):11-18.
3. The di erential diagnosis includes metastasis, red
Barakos JA, Dillon WP, Chew WM. Orbit, skull base, and
marrow expansion in anemia, sarcoidosis, and renal
pharynx: contrast-enhanced at suppression MR imaging.
osteodystrophy.
Radiology. 1991 Apr;179(1):191-198.
4. Skull metastases are commonly seen in adults with Lauenstein TC, Goehde SC, Herborn CU, et al. Whole-
primary cancers including breast, lung, prostate, renal body MR imaging: evaluation o patients or metastases.
cell, ollicular thyroid, melanoma, and multiple myeloma. Radiology. 2004 Oct;233(1):139-148.
138
71-year-old female with a lucent lesion seen on x-ray

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What are presenting symptoms or this lesion?

5. What is the typical treatment or this entity?


139
Skull hemangioma Case 57 (3515)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Axial T1 noncontrast image shows a well-marginated right Axial T2 image shows the lesion to be mildly expansile and with
frontal bone lesion with high T1 signal (arrow). Note the internal high T2 signal (arrow).
punctate areas of thickened trabeculation (arrowhead).
3. The di erential diagnosis includes metastasis,
hemangioma, dermoid/epidermoid,
hemangioendothelioma, and Paget disease.
4. Hemangiomas are benign vascular lesions. Typically
the lesions are asymptomatic. Rarely, larger lesions may
cause extraaxial intracranial hemorrhage.
5. Usually no treatment is needed. Larger lesions
may require surgical resection due to mass e ect,
hemorrhage, or cosmesis.

Pearls
• Hemangiomas are benign vascular lesions.
• The most common locations are rontal and parietal
bones.
Coronal FLAIR image shows the lesion is mildly expansile and
thins the inner table (arrow). Note the punctate thickened
• Hemangiomas are usually located in the medullary
trabeculae within the lesion (arrowhead) suggesting space o the skull.
hemangioma. • Bright T1 bone lesions are almost always benign.
• Well circumscribed.
• May demonstrate enhancement on postcontrast
Answers imaging.
1. The abnormality is located within the rontal bone. • Inner and outer table typically intact.
• Look or thickened trabecula on CT.
2. On CT, the hemangiomas classically have thickened
trabeculae. On MR, the tumors may have iso- to
hyperintense T1 and T2 bright signal. Interosseous Suggested Reading
hemangiomas may demonstrate enhancement on
postcontrast imaging. Stages o blood products can occur Bastug D, Ortiz O, Schochet SS. Hemangiomas in the
within the lesion. calvaria: imaging f ndings. AJR Am J Roentgenol. 1995
Mar;164(3):683-687.
140
35-year-old male with loss of consciousness after fall

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What imaging modality is use ul or evaluation


o possible arterial injury?

4. What is the etiology o this f nding?

5. What are common sequelae o this entity?

141
Basilar skull fracture Case 58 (3297)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Axial CT bone window image shows a fracture extending from


Axial CT bone window image shows fracture lines involving the
the left occiput into the left temporal bone (arrow).
occiput (arrows).

Answers
• Depressed ragments may result in intracranial
1. The abnormality is located within the occipital bone.
hemorrhage or brain injury (parenchymal contusion).
2. The classic imaging f ndings or this entity are • Evaluate brain or parenchymal contusions and
comminuted or depressed ractures o the skull base extraaxial hemorrhage.
involving the basiocciput, basisphenoid, and/or temporal • Follow racture lines or involvement o oramina.
bones. • CTA needed i racture traverses vascular oramen
to exclude arterial dissection, thrombosis, or
3. Basilar skull ractures that have suspected vascular injury
pseudoaneurysm.
can be studied with CTA. The gold standard remains
• Pneumocephalus is a clue or racture.
catheter angiogram, but this is minimally invasive.
• Venous epidural hematoma may occur with disruption
4. Most commonly a direct blow to the back o the head o venous sinus.
or a all on the back o the head can cause basilar skull
ractures.
5. Basilar skull ractures may have depressed ragments Suggested Readings
resulting in brain parenchymal contusion. These ractures
may also extend into the temporal bones or vascular Aiken AH, Gean AD. Imaging o head trauma. Semin
canals/cranial nerve oramen resulting in hearing loss, Roentgenol. 2010 Apr;45(2):63-79.
arterial dissection, thrombosis, or pseudoaneurysm. York G, Barboriak D, Petrella J, DeLong D, Provenzale JM.
Association o internal carotid artery injury with carotid
canal ractures in patients with head trauma. AJR Am J
Roentgenol. 2005 May;184(5):1672-1678.
Pearls Zayas JO, Feliciano YZ, Hadley CR, Gomez AA, Vidal
• Basilar skull ractures may have comminution or JA. Temporal bone trauma and the role o multidetector
depressed ragments. CT in the emergency department. Radiographics. 2011
Oct;31(6):1741-1755.
142
61-year-old man with acute head pain after a recent fall

1. Where is the abnormality located?

2. What are the imaging f ndings or this entity?

3. What is the di erential diagnosis?

4. What is the etiology o this f nding?

5. What is the treatment or this entity?

143
Depressed skull fracture Case 59 (3360)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Axial CT brain window image shows multiple punctate foci of


Axial CT bone window image shows a depressed, comminuted
hemorrhage from brain contusion (arrow).
fracture posteriorly involving the right parietal bone (arrow).

Answers
1. The abnormality involves the right parietal bone. Pearls
• There are three main types o skull racture: basilar,
2. The imaging f ndings or depressed skull racture include
linear, and depressed.
displacement o the inner table into the skull with
• Evaluate brain or parenchymal contusion and
ragments.
extraaxial hemorrhage.
3. The di erential diagnosis includes suture diastasis, • Skull racture may disrupt venous sinuses.
arachnoid granulation, and depressed racture. • Depressed ractures also are at risk or in ection.
• Pneumocephalus is a clue or racture.
4. Depressed skull ractures are caused by high-energy
blunt trauma to a small area o the skull.
5. Depressed ractures also are at risk or in ection and Suggested Reading
injury to venous sinuses. There ore, these ractures are
Aiken AH, Gean AD. Imaging o head trauma. Semin
typically explored surgically and racture ragments are
Roentgenol. 2010 Apr;45(2):63-79.
elevated and/or removed.

144
6 week old baby boy with lump on head

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the etiology o this entity?

5. What is the treatment or this entity?

145
Cephalohematoma Case 60 (3466)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Pearls
• Cephalohematoma is a subperiosteal hematoma that is
bound by sutures.
• I hematoma crosses midline, consider caput
succedaneum or subgaleal hematoma.
• Cephalohematoma may peripherally calci y, then
ossi y and be incorporated in the skull over time.
• Cephalohematoma most commonly involves the
parietal bone ollowed by the occipital bone.
• Do not mistake or f brous dysplasia, which does not
typically present in the neonatal period.

Suggested Readings
Nabavizadeh SA, Bilaniuk LT, Feygin T, Shekdar KV,
Zimmerman RA, Vossough A. CT and MRI o pediatric
skull lesions with uid- uid levels. AJNR Am J
Neuroradiol. 2014;35(3):604-608.
Winter TC, Mack LA, Cyr DR. Prenatal sonographic
diagnosis o scalp edema/cephalohematoma mimicking
an encephalocele. AJR Am J Roentgenol. 1993
AP view of the skull shows a well-circumscribed oval-shaped
Dec;161(6):1247-1248.
structure over the left parietal bone. Notice that it does not cross
midline and has peripheral calci cation (arrows).

Answers
1. The abnormality is located along the le t parietal bone.
2. A cephalohematoma typically appears as a well-
circumscribed scalp mass that is bound by cranial
sutures.
3. The di erential diagnosis includes cephalohematoma,
subgaleal hematoma, caput succedaneum, and
cephalocele.
4. Cephalohematoma is typically rom trauma and
commonly seen in newborns with instrument delivery.
There can be associated skull racture.
5. No treatment is needed or this entity. Cephalohematomas
can eventually ossi y and be remodeled with the calvarium.

146
27-month-old female with uctuant scalp mass

1. What is the di erential diagnosis?

2. What intracranial vascular structures


communicate with the mass?

3. What imaging should be per ormed prior to


possible surgical resection?

4. What imaging f ndings can be seen in the


extracranial component?

5. What are the causes o this lesion?


147
Sinus pericranii Case 61 (3508)
Case ranking/dif culty: Category: Meninges, skull, and scalp

Axial CT image with contrast Axial CT postcontrast image Sagittal CT postcontrast delayed phase Oblique reformat of CT with
in the venous phase delayed phase shows bony image shows the bony defect with contrast delayed phase shows
shows serpentine vascular defect connecting the scalp communication with the right transverse the right bony defect (arrow) with
structures in the occipital vascular mass with the right sinus (arrow). communication of scalp vascular
scalp (arrows), right greater transverse sinus (arrow). mass and right transverse sinus.
than left.

Answers
1. Di erentials or vascular masses o the scalp Pearls
include sinus pericranii, venous and arteriovenous • Sinus pericranii represents a variant communication
mal ormations. In antile hemangiomas may also be between intracranial and extracranial venous
considered, although this appearance is not typical. circulation.
• Typically seen as a scalp venous varix communicating
2. Sinus pericranii typically communicate with
with a dural venous sinus through a transcalvarial
dural venous sinuses or cortical veins and rarely
emissary vein.
developmental venous anomalies.
• Etiology can be congenital or traumatic.
3. Venography, CT, MR, or catheter is essential or • The extracranial venous structure may also present
evaluating the associated venous structures in sinus as a venous mal ormation with septations, cysts, and
pericranii and evaluate or adequate venous drainage o phleboliths.
the brain to prevent venous in arcts a ter ligation. • Intracranial communications are typically a dural
venous sinus, cortical vein, or less commonly, a
4. Extracranial component o sinus pericranii typically
developmental venous anomaly.
involves a scalp venous varix that may be dilated and
• CT or MR venography is help ul to identi y all
serpentine or a vascular mal ormation with cysts,
associated venous vascular components.
septations, and phleboliths.
• Assessment o appropriate pathways o venous
5. The majority o sinus pericranii are thought to be drainage is necessary to prevent venous in arcts, prior
congenital, possibly associated with other venous to surgical ligation.
mal ormations and variations, or rom sinus thrombosis.
Posttraumatic laceration o emissary veins is also an
etiology.
Suggested Readings
Gandol o C, Krings T, Alvarez H, et al. Sinus pericranii:
diagnostic and therapeutic considerations in 15 patients.
Neuroradiology. 2007 Jun;49(6):505-514.
Kim YJ, Kim IO, Cheon JE, Lim YJ, Kim WS, Yeon KM.
Sonographic eatures o sinus pericranii in 4 pediatric
patients. J Ultrasound Med. 2011 Mar;30(3):411-417.

148
40-year-old female with headaches

1. Where is the abnormality located?

2. What are the imaging f ndings or this entity?

3. What is the di erential diagnosis?

4. What is a complication o this entity?

5. What is the treatment or this entity?

149
Pineal cyst Case 62 (3064)
Case ranking/dif culty: Category: Pineal region

Sagittal T1 image shows a lesion within the Axial T2 image shows a cystic lesion in Coronal FLAIR shows that the lesion
pineal gland that is hypointense but has more T1 the pineal gland that is hyperintense in the pineal gland is hyperintense to
shortening than CSF (arrow). and follows CSF signal (arrow). CSF (arrow).

Pearls
• Pineal cysts (PC) have an outer f brous connective
tissue layer with pineal parenchyma with or without
hemorrhagic products internally.
• Key to diagnosis is noting a uid signal lesion within
the pineal gland.
• PC larger than 1.5 cm may compress the aqueduct
resulting in hydrocephalus.
• Check lateral ventricle size.
• Check aqueduct or compression.
• Consider pineocytoma when solid components are seen.

Suggested Reading

Coronal postcontrast image shows that the lesion in the pineal Osborn AG, Preece MT. Intracranial cysts: radiologic-
gland does not enhance (arrow). pathologic correlation and imaging approach. Radiology.
2006 Jun;239(3):650-664.

Answers
1. The abnormality is located in the pineal gland.
2. The imaging f ndings or this entity include intermediate
and slightly brighter than CSF on T1, ollow CSF on T2,
and have peripheral or rim contrast enhancement.
3. The di erential diagnosis includes arachnoid cyst, pineal
cyst, and pineocytoma.
4. This entity may result in obstructive hydrocephalus
when lesions are larger than 1.5 cm in size.
5. Usually no treatment is needed unless it gets large
enough to cause obstructive hydrocephalus.
150
17-year-old male with vomiting and seizure

1. What is the di erential diagnosis?

2. What are the most common locations or this


tumor?

3. What is the most common location o these


tumors in males and emales?

4. What are the typical imaging characteristics o


this tumor?

5. What is the prognosis or this disease?


151
CNS germinoma Case 63 (3372)
Case ranking/dif culty: Category: Pineal region

Axial FLAIR image shows Axial DWI b = 1000 image Sagittal T1 postcontrast shows intense Axial color CBVmap from
a lobular mass in the shows areas of decreased enhancement of the pineal mass with dynamic susceptibility
pineal region, which is di usion (arrows) due to possible invasion of the tectal plate contrast perfusion imaging
slightly hyperintense to hypercellularity. (arrow). A second smaller enhancing shows areas of hyperperfusion
gray matter (arrow), which mass is seen in the suprasellar cistern in the tumor (arrow).
causes hydrocephalus (arrowhead).
and transependymal CSF
(arrowhead).

Answers
1. A pineal region mass should include the di erentials o Pearls
germ cell tumor, pineoblastoma, and tectal plate glioma. • Germinomas are a subclassif cation o germinomatous
germ cell tumors while nongerminomatous germ cell
2. The most common locations or germ cell tumors are
tumors include yolk sac tumors and teratomas.
the pineal region and suprasellar locations. Pineal region
• CNS germ cell tumors commonly occur in the midline
germ cell tumors are more common than suprasellar with
at the pineal and suprasellar locations.
a ratio o 2:1.
• CNS germinoma in the pineal region have a 10:1
3. Pineal germinomas are 10× more likely to occur in males predilection or boys while those in the suprasellar
than emales while 75% o germ cell tumors in emales location have a 75% predilection or girls.
are in the suprasellar location. • Imaging shows lobular-enhancing tumors engulf ng
the pineal gland or in undibulum. Pituitary
4. Germinomas typically show homogeneous enhancement
calcif cations are engul ed rather than exploded as in
with restricted di usion due to hypercellularity. Tumor
pineoblastomas.
cysts and necrosis, along with adjacent structure
• Germinomas can show restricted di usion due to high
invasion, can be seen as the tumor increases in size. CSF
cellularity. On CT, this shows hyperdensity.
dissemination is not uncommon at presentation.
• Larger tumors can invade adjacent brain parenchyma.
5. Pure germinomas have good prognosis despite the • Pure germinomas are highly responsive to radiation
hypercellular nature due to sensitivity to radiation and chemotherapy, with avorable prognosis.
and platinum-based chemotherapy. Greater than
90% 5-year survival is seen with radiation alone. By
contrast, nongerminomatous germ cell tumors typically Suggested Readings
have poorer prognosis due to relative insensitivity to Dumrongpisutikul N, Intrapiromkul J, Yousem DM.
treatment. Distinguishing between germinomas and pineal cell
tumors on MR imaging. AJNR Am J Neuroradiol. 2012
Mar;33(3):550-555.
Mathews VP, Broome DR, Smith RR, Bognanno JR, Einhorn
LH, Edwards MK. Neuroimaging o disseminated
germ cell neoplasms. AJNR Am J Neuroradiol. 2003
Jan;11(2):319-324.

152
13-month-old male with vomiting and lethargy

1. What is the di erential diagnosis o this


tumor?

2. How does di usion imaging help in


di erentiating these tumors?

3. What are typical imaging f ndings in this


entity?

4. What hereditary conditions are associated with


this tumor?

5. What is the prognosis o these pineal tumors?


153
Pineoblastoma Case 64 (3375)
Case ranking/dif culty: Category: Pineal region

Axial CT image shows a Axial T2 image shows Axial DWI b = 1000 image shows Axial postcontrast image shows
pineal region tumor with hypointensity of the solid restricted di usion of the pineal heterogeneous enhancement
“exploded” peripheral portions of the tumor (arrow). tumor (arrow). of the pineal tumor (arrow).
calci cations (arrows). There
is obstructive hydrocephalus
with transependymal CSF
(arrowheads). A shunt catheter Pearls
is noted in the right lateral
• Pineoblastomas are poorly di erentiated, embryonal
ventricle.
WHO grade IV tumors originating rom the pineal
gland.
Answers • There is signif cant histological overlap with PNET,
with pineal region PNETs and pineoblastomas
1. Pineal parenchymal tumors, germ cell tumors, and tectal
considered as related entities.
plate glial tumors should be considered in tumors o the
• Known association with sporadic and hereditary
pineal region.
retinoblastoma, orming the “trilateral retinoblastoma”
2. Pineal tumors with decreased di usion suggest high- in the mutations o RB1 gene.
grade lesions such as pineoblastoma and poorly • Imaging shows a large lobular and heterogeneous
di erentiated germ cell tumors. tumor in the pineal region with requent invasion o
adjacent structures.
3. Typical imaging f ndings or pineoblastoma include
• CT can show “exploded” peripheral calcif cation rather
an “exploded” peripheral calcif cation, decreased T2
than central engul ed calcif cation as seen with germ
and di usion o the solid components, heterogeneous
cell tumors.
enhancement, and invasion o adjacent structures.
• Solid components show T2 hypointensity and restricted
4. Pineoblastomas and the related pineal region primitive di usion due to hypercellularity.
neuroectodermal tumors have well-known associations • Heterogeneous enhancement, necrosis, and
with amilial retinoblastoma (adding bilateral hemorrhage can be common.
retinoblastomas orms the “trilateral retinoblastoma”). • The typical clinical prof le is a toddler with increased
Also, pineoblastomas have also been reported with intracranial pressure and possible Parinaud syndrome.
Turcot syndrome, a subset o amilial adenomatous
polyposis.
5. Two major tumor types occur in the pineal region, Suggested Readings
pineal parenchymal tumors and germ cell tumors. As a Kakigi T, Okada T, Kanagaki M, et al. Quantitative imaging
whole, pineal parenchymal tumors have worse survival values o CT, MR, and FDG-PET to di erentiate pineal
than germ cell tumors. This is likely re ected in that parenchymal tumors and germinomas: are they use ul?
45% o pineal parenchymal tumors are pineoblastomas. Neuroradiology. 2014;56(4):297-303.
Pineoblastomas have 1- to 2-year median survival a ter Rodjan F, de Graa P, Moll AC, et al. Brain abnormalities on
presentation despite treatment. MR imaging in patients with retinoblastoma. AJNR Am J
Neuroradiol. 2010 Sep;31(8):1385-1389.

154
21-year-old female with headache

1. What is the di erential diagnosis?

2. What imaging sign is specif c or this disease?

3. What is the most common clinical symptom in


this diagnosis?

4. What are the characteristic radiographic


eatures?

5. What is the primary treatment option or this


disease?

155
Rathke cleft cyst Case 65 (3237)
Case ranking/dif culty: Category: Sellar/suprasellar

Noncontrast CT brain Sagittal T1 image demonstrates Axial T2 image demonstrates Postcontrast coronal T1 image
demonstrates a round a large T1 slightly hyperintense mixed signal intensity of demonstrates mild peripheral
hyperdense mass in the sellar/ mass in the sella extending to a sellar/suprasellar mass. enhancement of a sellar/suprasellar
suprasellar region (asterisk). the suprasellar region (asterisk), There is a T2 hypointense mass (white arrowhead).
causing mild anterior deviation of intracystic nodule (arrow).
pituitary stalk (white arrow). Note
a pituitary gland in the oor of
pituitary fossa (white arrowhead).

Answers
• Usually incidentally ound and asymptomatic,
1. Craniopharyngioma, pituitary macroadenoma, Rathke
1 in 10 autopsies.
cle t cyst, thrombosed aneurysm, and dermoid/
• Radiographic eatures seen as well-def ned,
epidermoid cyst can all occur in the same location,
nonenhancing midline cyst within the sella arising
usually suprasellar in origin, and should be considered in
between the anterior lobes and pars intermedia.
the di erential diagnosis.
• 60% suprasellar extension.
2. Specif c imaging f ndings o Rathke cle t cyst include • Varying signal characteristics due to cystic protein
the “claw” sign o a peripheral enhancing pituitary gland concentration.
and an intracystic T2 hypointense nodule. • T2 hypointense intracystic nodule in the majority o
3. Most Rathke cle t cysts are incidentally discovered and cases.
asymptomatic. When present, headaches are usually the • Top three di erential diagnoses include
most common symptom, and can be accompanied by craniopharyngioma, cystic microadenoma, and
symptoms re erable to compression on the optic chiasm, aneurysm.
pituitary, and hypothalamus. • Transsphenoidal resection is a treatment option in the
symptomatic patient.
4. A nonenhancing, midline cystic lesion in the sellar/
suprasellar region arising rom the anterior lobe and
pars intermedia o the pituitary gland are characteristic
radiographic eatures. However, wall calcif cation and Suggested Readings
enhancement may be present in some cases (10%-15%) Bonneville F, Cattin F, Marsot-Dupuch K, Dormont D,
Internal calcif cation and enhancement are not seen. Bonneville JF, Chiras J. T1 signal hyperintensity in the
5. When symptomatic, Rathke cle t cysts are typically sellar region: spectrum o f ndings. Radiographics. 2006
treated by surgical resection. May;26(1):93-113.
Sumida M, Uozumi T, Mukada K, Arita K, Kurisu K,
Eguchi K. Rathke cle t cysts: correlation o enhanced
MR and surgical f ndings. AJNR Am J Neuroradiol. 1994
Pearls Mar;15(3):525-532.
• Benign, sellar/suprasellar epithelium-lined cystic Takanashi J, Tada H, Barkovich AJ, Saeki N, Kohno Y.
lesion arising rom the remnants o Rathke pouch. Pituitary cysts in childhood evaluated by MR imaging.
• Female predominance. AJNR Am J Neuroradiol. 2005 Sep;26(8):2144-2147.

156
45-year-old male with headaches, hemianopsia, and panhypopituitarism

1. What is the di erential diagnosis?

2. What imaging eature can help separate the top


2 di erential considerations?

3. What is the pathophysiology o the disease?

4. What percentage o patients will have other


autoimmune diseases?

5. What is the treatment or this disease?


157
Lymphocytic hypophysitis Case 66 (3248)
Case ranking/dif culty: Category: Sellar/suprasellar

Sagittal T1 postcontrast at midline shows Sagittal postcontrast image Axial T2 of the suprasellar Coronal T1 postcontrast
an isointense mass centered at the at midline shows solid cistern shows T2 again shows the
pituitary stalk (arrow). There is loss of the enhancement of the pituitary hypointensity of the infundibular mass with
posterior pituitary bright spot. stalk, which also involves infundibular mass extension into the sella
predominantly the anterior (arrow). This is speci c for (arrow).
pituitary gland (arrow). lymphocytic hypophysitis
over pituitary adenoma.

Answers
1. Likely di erentials o a homogeneous enhancing Pearls
mass involving the in undibulum and extending into • Lymphocytic hypophysitis (LH) is an autoimmune
the anterior pituitary gland include lymphocytic in ammation o the anterior pituitary gland and the
hypophysitis and pituitary adenoma. While germinoma pituitary stalk.
and hypothalamic glioma are considered, invasion into • While there is an association with peripartum women,
the pituitary is rare. LH can a ect both men and women rom adolescence
through old age.
2. The main di erential or a sellar and suprasellar
• Males are eight times less likely to have LH than
homogeneous enhancing mass is lymphocytic
emales and tend to present a decade later with mean
hypophysitis and pituitary macroadenoma. Both can
age o 45 years.
have homogeneous enhancement, but lymphocytic
• MRI imaging shows homogeneous enhancement
hypophysitis has not been described with cystic change.
with thickening o the pituitary stalk with or without
Loss o posterior pituitary bright spot is seen in 3/4 o
enlargement o the pituitary gland.
lymphocytic hypophysitis, but i the pituitary adenoma is
• 75% o LH have loss o the posterior pituitary bright
large enough, the neurohypophysis can be obliterated. T2
spot on T1.
hypointensity o lymphocytic hypophysitis is a specif c
• T2 hypointensity is a characteristic sign o LH and
sign, not seen with adenomas.
may help di erentiate rom pituitary adenoma.
3. Lymphocytic hypophysitis is an autoimmune-mediated
in ammation o the adenohypophysis and stalk.
4. Nearly a quarter o patients with lymphocytic Suggested Readings
hypophysitis will have other autoimmune diseases. Bellastella A, Bizzarro A, Coronella C, Bellastella G,
5. Lymphocytic hypophysitis is treated with Sinisi AA, De Bellis A. Lymphocytic hypophysitis: a
immunosuppression such as corticosteroids with rare or underestimated disease? Eur J Endocrinol. 2003
hormone replacement as needed. Nov;149(5):363-376.
Nakata Y, Sato N, Masumoto T, et al. Parasellar T2 dark
sign on MR imaging in patients with lymphocytic
hypophysitis. AJNR Am J Neuroradiol. 2010
Nov;31(10):1944-1950.

158
29-year-old female with galactorrhea

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What are possible presenting symptoms or


this lesion?

5. What is the treatment or this entity?

159
Pituitary microadenoma Case 67 (3070)
Case ranking/dif culty: Category: Sellar/suprasellar

Coronal T1 of the sella without contrast shows fullness along the


Coronal T1 of the sella postcontrast shows an area of diminished
right parasagittal pituitary gland (arrow).
contrast enhancement relative to the remainder of the gland (arrow).

Answers
1. The abnormality is located in the right aspect o the Pearls
pituitary gland. • Pituitary adenomas are benign tumors o the pituitary
gland.
2. Classically pituitary microadenomas are isointense
• Pituitary microadenomas < 1 cm.
to gray matter on T1- and T2-weighted images and
• Microadenomas typically are hormonally active in
demonstrate diminished contrast enhancement, which
contrast to macroadenomas, which usually are not.
appears as a “f lling de ect” in the pituitary gland.
• The most common microadenoma is the prolactinoma.
3. The di erential diagnosis includes microadenoma, • Adenomas usually enhance more slowly than rest o
craniopharyngioma, and Rathke cle t cyst. the gland; 10%-30% can be seen only with dynamic
sella imaging.
4. Prolactinomas are the most common type o hormonally
• Consider Rathke cle t cyst with a nonenhancing midline
active microadenomas. This usually results in
pituitary lesion.
galactorrhea in emales and decreased libido in male
• I heterogeneous sellar lesion with calcif cations,
patients.
consider craniopharyngioma.
5. The treatment or this entity includes medical treatment
or hormonally active lesions versus surgical resection.
Most microadenomas can be treated conservatively i
Suggested Reading
non unctioning.
Johnsen DE, Woodru WW, Allen IS, Cera PJ, Funkhouser
GR, Coleman LL. MR imaging o the sellar and juxtasellar
regions. Radiographics. 1991 Sep;11(5):727-758.

160
51-year-old male with visual changes

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What are presenting symptoms or this lesion?

5. What is the treatment or this entity?


161
Pituitary macroadenoma Case 68 (3068)
Case ranking/dif culty: Category: Sellar/suprasellar

Coronal T1 noncontrast image shows a large sellar mass that is Axial T2 image shows a large sellar mass that is isointense to brain
isointense to brain parenchyma (green arrow). Note that the mass parenchyma (arrow).
is extending into the suprasellar cistern and impressing upon the
optic chiasm (red arrow).
5. The treatment or this entity is medical (when tumors are
hormonally active) or surgical removal i symptomatic
compression occurs.

Pearls
• Pituitary adenomas are benign tumors o the pituitary
gland.
• Macroadenoma has a classic “snowman or f gure 8”
shape and is greater than 1 cm.
• Macroadenomas may invade the cavernous sinus or
sphenoid sinus.
• I ow voids are seen consider aneurysm.
• I a lesion is seen that is isointense to brain and does
not involve the pituitary consider a diaphragmatic
meningioma.
• Total encasement o the cavernous ICA is a highly
Coronal T1 postcontrast image shows enhancement of the sellar specif c but not sensitive sign or cavernous sinus
mass (green arrow). Note that the mass is impressing upon the invasion.
optic chiasm (red arrow). • CTA or MRA or exclusion o aneurysm.

Answers
1. The abnormality is located within the pituitary gland. Suggested Readings
2. These lesions classically are isointense to gray matter Douglas-Akinwande AC, Hattab EM. AJR teaching f le:
on T1- and T2-weighted images. There is associated central skull base mass. AJR Am J Roentgenol. 2010
enhancement o the tumor. Sep;195(3 suppl):S22-S24.
Johnsen DE, Woodru WW, Allen IS, Cera PJ, Funkhouser
3. The di erential diagnosis includes pituitary
GR, Coleman LL. MR imaging o the sellar and juxtasellar
macroadenoma, meningioma, and aneurysm.
regions. Radiographics. 1991 Sep;11(5):727-758.
4. Patients usually present with bitemporal hemianopsia
rom compression o the optic chiasm.
162
86-day-old male with enlarging head circumference

1. What is the di erential diagnosis?

2. What are typical imaging f ndings or this


entity?

3. What is the gender predilection or this entity?

4. What is the natural history o this entity?

5. What must be excluded with subdural


hematomas?
163
Enlarged subarachnoid spaces Case 69 (3579)
Case ranking/dif culty: Category: Subarachnoid spaces

Axial T2 image shows enlarged Coronal T2 shows increased Axial FLAIR shows complete Sagittal T1 image o midline
CSF spaces overlying the frontal subarachnoid spaces suppression of the symmetric shows the frontal and temporal
lobes (asterisks). Note the lack associated with the frontal prominent frontal and temporal enlarged subarachnoid spaces
of mass e ect with prominence and temporal lobes (asterisks). subarachnoid spaces (asterisks). following CSF signal (asterisks).
of the sulci and bridging veins, Again note the bridging veins
which traverse this space from traversing this CSF space
the cortex to the inner table of (arrows).
the skull (arrows).

Answers
1. The di erential diagnosis or prominent extra-axial Pearls
spaces in an in ant includes enlarged subarachnoid • Accumulation o increased CSF in the subarachnoid
spaces, subdural hematoma, communicating spaces due to immaturity o the arachnoid villi.
hydrocephalus (extraventricular obstructive • No mass e ect on brain parenchyma with bridging
hydrocephalus), and brain atrophy. veins traversing through subarachnoid space.
• MRI is the most sensitive and specif c or the
2. In enlarged subarachnoid spaces, the rontal and anterior
evaluation o bridging veins through the enlarged
temporal symmetric uid collections ollow CSF on all
subarachnoid spaces.
imaging sequences. A key f nding is the visualization
• CT with contrast may be help ul to distinguish the
o bridging veins that travel through this space rom the
bridging veins.
cortex to inner table. No mass e ect on the parenchyma
• Ultrasound with Doppler may be use ul with
is seen.
persistence o the anterior ontanelle.
3. Enlarged subarachnoid spaces typically involve males at • Follows CSF on all sequences.
a 4 to 1 ratio. • Di f cult to exclude chronic subdural hematomas on
CT, raising the possibility o nonaccidental trauma.
4. Likely due to maturation o arachnoid villi, almost all
• Possible increased risk o small subdural hematomas
cases resolve without any intervention by 2 years o age,
with minimal head trauma with enlarged subarachnoid
with resolution o enlarged subarachnoid spaces and any
spaces—clinical history very important!
associated mild motor delays. Persistent macrocephaly is
common.
5. Enlarged subarachnoid spaces have been suggested to be
Suggested Readings
a risk actor or small subdural hematomas with minimal
head trauma. However, a good clinical history must be Greiner MV, Richards TJ, Care MM, Leach JL. Prevalence o
obtained to exclude nonaccidental trauma. subdural collections in children with macrocrania. AJNR
Am J Neuroradiol. 2013 Dec;34(12):2373-2378.
Kendall B, Holland I. Benign communicating hydrocephalus
in children. Neuroradiology. 1981 Mar;21(2):93-96.

164
30-year-old female with headaches

1. Where is the abnormality located?

2. What are the classic imaging f ndings?

3. What is the di erential diagnosis?

4. What is the etiology o this entity?

5. What is the treatment or this entity?


165
Arachnoid cyst Case 70 (3063)
Case ranking/dif culty: Category: Subarachnoid spaces

Axial T1 image shows a hypointense CSF Axial T2 image shows the left middle Axial DWI b-1000 shows no restricted
signal lesion (green arrow) in the extraaxial cranial fossa lesion has high T2 di usion (arrow).
space within the left middle cranial fossa. signal following CSF (arrow). There is
Note the bony remodeling (white arrow) displacement of the brain parenchyma.
suggesting a long-standing lesion.

5. Arachnoid cysts are benign, and the majority o cases do


not require treatment. However, a small number o cases
can cause symptoms that can be relieved by surgical
resection, enestration, or shunting.

Pearls
• Arachnoid cysts are the most common congenital
cystic lesion
• The most common location is the middle cranial ossa
(60%).
• Follows CSF on all MRI pulse sequences.
• No contrast enhancement.
• I DWI shows restriction, then consider epidermoid/
Coronal FIESTA image shows extension of cystic lesion to involve dermoid cyst and neurenteric cyst.
the sylvian ssure (arrow).

Answers Suggested Reading


1. The abnormality is within the le t middle cranial ossa. Osborn AG, Preece MT. Intracranial cysts: radiologic-
pathologic correlation and imaging approach. Radiology.
2. These lesions will ollow CSF on CT and all MRI
2006 Jun;239(3):650-664.
sequences. There ore, T1 prolongation (low signal) and
T2 prolongation (high signal).
3. The di erential diagnosis includes dermoid cyst,
epidermoid cyst, and arachnoid cyst.
4. Arachnoid cysts are primarily congenital, although rarely
they are associated with surgery or in ection.

166
48-year old female with a headache

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the etiology o this entity?

5. What is the treatment or this entity?


167
Choroidal ssure cyst Case 71 (3548)
Case ranking/dif culty: Category: Subarachnoid spaces

Coronal T1 image shows a well-circumscribed low signal lesion Coronal FIESTA image shows the right choroidal ssure cystic
within the right choroidal ssure (arrow). lesion with high signal following CSF (arrow).

4. Choroidal f ssure cysts are extraaxial neuroepithelial or


arachnoid cysts, which are likely congenital in nature.
5. No treatment is typically necessary or this entity. Some
authors advocate surgical resection in the setting o
medically re ractory seizures.

Pearls
• A benign extraaxial cyst located within the choroidal
f ssure.
• The choroidal f ssure is a cle t located along the wall o
the lateral ventricle where the choroid plexus attaches.
• May be associated with seizures and may be secondary
to compression o the hippocampus.
• Follows CSF on all sequences without contrast
enhancement.
Coronal T1 postcontrast image demonstrates no contrast
enhancement of the cystic lesion (arrow). The enhancement • I contrast enhancement is seen, consider neoplasm.
inferior to the lesion is from choroid plexus. • I gyral enhancement is seen, consider in arct.

Answers Suggested Readings


1. The abnormality is within the right choroidal f ssure. de Jong L, Thewissen L, van Loon J, Van Calenbergh F.
2. On CT and MRI, the key to diagnosis is the location Choroidal f ssure cerebrospinal uid-containing cysts:
within the choroidal f ssure and that the lesion ollows case series, anatomical consideration, and review o the
CSF signal on all sequences. A ter the administration o literature. World Neurosurg. 2011 Dec;75(5-6):704-708.
contrast, no enhancement is seen. Osborn AG, Preece MT. Intracranial cysts: radiologic-
pathologic correlation and imaging approach. Radiology.
3. The di erential diagnosis includes hippocampal sulcus 2006 Jun;239(3):650-664.
remnant, choroidal f ssure cyst, and mesial temporal Sherman JL, Camponovo E, Citrin CM. MR imaging o CSF-
sclerosis. like choroidal f ssure and parenchymal cysts o the brain.
AJR Am J Roentgenol. 1990 Nov;155(5):1069-1075.
168
55-year-old male with chronic headache

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the etiology o this entity?

5. What is the treatment or this entity?

169
Intracranial lipoma Case 72 (3112)
Case ranking/dif culty: Category: Subarachnoid spaces

Axial CT demonstrates the midline lipoma that is easier to visualize


Axial CT image shows a low attenuating lesion consistent with
utilizing a wide window, demonstrating decreased attenuation
fat within the midline with associated calci cations (green arrow).
compared to uid (arrow).
Also note that there is colpocephaly (white arrows) with associated
agenesis of the corpus callosum.

Pearls
Answers
• Intracranial lipomas are a product o maldi erentiation
1. The abnormality is located within the interhemispheric o the meninx primitiva (meninges precursor).
f ssure. • The most common location or intracranial lipomas is
2. The classic imaging f ndings are low attenuation, at within the pericallosal region.
density within the lesion. • Will ollow at on all MRI pulse sequences.
• Loss o signal on MRI at saturation sequences is
3. The di erential diagnosis includes lipoma, dermoid, and conf rmatory.
teratoma. • No enhancement.
4. Intracranial lipomas are a product o maldi erentiation • Hounsf eld units less than −30.
o the meninx primitiva (meninges precursor). The most
common location or intracranial lipomas is within the
pericallosal region. Lipomas will ollow at on CT and Suggested Readings
MRI. Calcif cations are commonly seen. There is an Ginat DT, Meyers SP. Intracranial lesions with high signal
association with corpus callosum dysgenesis. intensity on T1-weighted MR images: di erential
5. No treatment needed. diagnosis. Radiographics. 2012;32(2):499-516.
Ho ML, Moonis G, Ginat DT, Eisenberg RL. Lesions
o the corpus callosum. AJR Am J Roentgenol. 2013
Jan;200(1):W1-W16.
170
72-year-old man with head pain after fall

1. Where is the abnormality located?

2. What is the di erential diagnosis or high


attenuation within a sulcus?

3. What is the most common cause o the high


attenuation?

4. What other common etiologies can cause this


appearance?

5. Which routine MRI sequences are highly


sensitive to this sequence?

171
Subarachnoid hemorrhage Case 73 (2964)
Case ranking/dif culty: Category: Subarachnoid spaces

Axial CT shows linear high attenuation within one of the left Axial FLAIR shows linear high signal within one of the left frontal
frontal lobe sulcus (arrow). lobe sulci (arrow). The remaining sulci all have dark or nulled signal,
which is normal on this uid attenuated inversion recovery image.

Answers
1. Subarachnoid hemorrhage (SAH) is blood within the
subarachnoid space and may f ll sulci and/or cisterns.
Pearls
• Subarachnoid hemorrhage (SAH) f lls sulci and cisterns.
2. The di erential diagnosis or high attenuation within a • CT noncontrast, check or high attenuation within sulci
sulcus includes subarachnoid hemorrhage, metastatic and/or cisterns.
disease (leptomeningeal spread), and an in ectious • Check dependent sylvian f ssures and interpeduncular
process. cistern.
3. SAH is usually the result o injury to pial or arachnoidal • Nontraumatic SAH needs to be ollowed with vascular
cortical vessels rom trauma. imaging in order to exclude an aneurysm or AVM.
• With in ection history, consider pus rom meningitis.
4. SAH is also associated with aneurysms o the circle o • With cancer history, consider carcinomatosis.
Willis. Trauma is the most common cause, with vascular
mal ormations and amyloid angiopathy as other potential
causes. Perimesencephalic nonaneurysmal subarachnoid
hemorrhage is an uncommon clinically benign entity that Suggested Reading
is likely venous in origin. Aiken AH, Gean AD. Imaging o head trauma. Semin
5. O the “routine” MRI sequences, SAH is easiest Roentgenol. 2010 Apr;45(2):63-79.
to diagnose on FLAIR images rom lack o CSF
suppression.

172
69-year-old male with chronic headaches

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What are acute presenting symptoms or this


lesion?

5. What is the treatment or this entity?

173
Giant intracranial aneurysm Case 74 (3073)
Case ranking/dif culty: Category: Subarachnoid spaces

Axial T2 image shows spherical low signal structure (arrow) that


Axial CT image shows high-density structure with associated appears to arise from the right ICA bifurcation.
peripheral calci cation adjacent to the right ICA bifurcation (arrow).

Answers
1. The abnormality is located in the extraaxial space Pearls
adjacent to the circle o Willis. • Giant aneurysms are def ned as aneurysms larger than
25 mm.
2. Key to the diagnosis is to conf rm aneurysm connection
• Most o these are saccular type.
to its arterial origin. Commonly, giant aneurysms will
• On CT, peripheral calcif cations may be help ul or
exhibit peripheral calcif cation. Heterogeneous contrast
diagnosis.
enhancement and ow characteristics may be seen due to
• Key to the diagnosis is to conf rm aneurysm connection
partial aneurysm thrombosis.
to its arterial origin (utilize MRA and CTA).
3. The di erential diagnosis includes aneurysm, • Angiography reserved or treatment.
meningioma, and metastasis. • I no vascular connection, consider meningioma.
4. In the acute setting, patients may present with the
“worst headache o my li e” secondary to subarachnoid
hemorrhage, loss o consciousness, nausea, and vomiting. Suggested Reading
5. The treatment or this entity depends on multiple actors, Mehta RI, Salamon N, Zipser BD, Mehta RI. Best cases rom
some o which include neck dimensions, shape (saccular the AFIP: giant intracranial aneurysm. Radiographics.
vs usi orm), and the presence o collateral circulation. 2010;30(4):1133-1138.
Endovascular coiling vs surgical clipping are typical
options or saccular aneurysms. Fusi orm aneurysms
can be treated endovascularly with covered or pipeline
stents.

174
61-year-old male with headache and confusion

1. What is the abnormality?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What are associated risk actors or this entity?

5. What is the treatment or this entity?

175
Fusiform aneurysm Case 75 (3161)
Case ranking/dif culty: Category: Subarachnoid spaces

Axial 3D time of ight image demonstrates Axial CT image shows the bilateral Axial CT image shows circumferential
circumferential enlargement of both circumferential enlargement to originate enlargement of both supraclinoid portions
internal carotid arteries involving their from the internal carotid arteries (green of the internal carotid arteries. Note the
supraclinoid portions (arrows). arrows). Also, note the dolichoectasia of peripheral calci cations in these vessels
the basilar artery (red arrow). indicating atherosclerotic disease (arrows).

Answers
1. The internal carotid arteries are abnormally dilated. Pearls
• Fusi orm aneurysms are typically related to
2. Fusi orm aneurysms typically demonstrate abnormal
atherosclerotic disease.
enlargement that does not protrude away rom the lumen
• More common in vertebrobasilar arteries.
(saccular aneurysm) but involves the whole lumen.
• Calcif cation common.
Peripheral calcif cation is common, with no contrast
• Intraluminal clot and ow turbulence cause
f lling o the intraluminal thrombus. MRI demonstrates
heterogeneous signal on MR.
mixed signal due to thrombus and ow characteristics.
• CTA provides better spatial resolution than MRA.
3. The di erential diagnosis includes aneurysm, • Aneurysms less than 7 mm have a rupture rate o 0.1%.
meningioma, and vascular mal ormation. The key to the • In general the larger the aneurysms, the more likely it
diagnosis is noting ocal dilation involving the whole is to rupture.
vessel circum erentially. This excludes meningioma and • Re ormatted images including maximum intensity
a vascular mal ormation. projection images and 3D volume-rendered images
may also be help ul.
4. Fusi orm aneurysms involve the whole vessel
• Utilize noncontrast CT in order to exclude
circum erentially. These are also called dolichoectatic
subarachnoid hemorrhage.
aneurysms and are o ten associated with atherosclerotic
disease. Risk actors include amily history o
aneurysms, hypertension, hyperlipidemia, tobacco use,
vasculopathies, connective tissue disorders, trauma, and Suggested Readings
vascular mal ormations. Hacein-Bey L, Provenzale JM. Current imaging assessment
5. The treatment or this entity includes endovascular and treatment o intracranial aneurysms. AJR Am J
therapy (stenting). Roentgenol. 2011 Jan;196(1):32-44.
Kemmling A, Noelte I, Gerigk L, Singer S, Groden C, Schar
J. A diagnostic pit all or intracranial aneurysms in time-
o - ight MR angiography: small intracranial lipomas. AJR
Am J Roentgenol. 2008 Jan;190(1):W62-W67.

176
65-year-old male with acute right sided weakness

1. Where is the abnormality?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the most common etiology o this


f nding?

5. What is the treatment or this entity?

177
Internal carotid artery occlusion Case 76 (3201)
Case ranking/dif culty: Category: Subarachnoid spaces

Axial T1 noncontrast image shows loss of the normal “ ow void” Axial T2 image shows increased signal and loss of the normal
of the left petrous internal carotid artery (arrow). “ ow void” of the left petrous internal carotid artery (arrow).

Pearls
• Arterial occlusion is usually the mani estation o
atherosclerotic disease.
• Cerebral in arction occurs in greater than two-thirds o
patients with carotid occlusion.
• Consider CTA or US (neck) to conf rm occlusion on
time o ight MRA, as this technique overestimates
stenosis.
• Catheter angiogram remains gold standard.
• T1 and T2 demonstrate “ ow void” loss with increased
Maximum intensity projection image from time of ight imaging
signal.
again shows nonvisualization of the left internal carotid artery
(arrow). • 2D time o ight use ul or detecting ow direction and
is instrumental in diagnosing “subclavian steal” (no
ow in vertebral artery on time o ight MRA, normal
Answers contrast opacif cation with contrast-enhanced MRA,
1. The abnormality involves the le t internal carotid artery. CTA, or DSA).
2. On imaging, nonvisualization o the vessel is the classic
appearance.
Suggested Readings
3. The di erential diagnosis includes vasculitis, dissection,
and atherosclerotic occlusive disease. Huang BY, Castillo M. Radiological reasoning: extracranial
causes o unilateral decreased brain per usion. AJR Am J
4. Arterial occlusion is usually the mani estation o Roentgenol. 2007 Dec;189(6 suppl):S49-S54.
atherosclerotic disease, which is an acquired process. Kerwin WS, Hatsukami T, Yuan C, Zhao XQ. MRI o
5. The treatment or occlusive disease is supportive care. carotid atherosclerosis. AJR Am J Roentgenol. 2013
Endarterectomy and endovascular therapy are classically Mar;200(3):W304-W313.
reserved or symptomatic nonocclusive stenosis over
70% at the internal carotid bulb.

178
59-year-old male with acute headache, nausea, and vomiting

1. What can be seen on MRI imaging within the


sulci di usely in this entity?

2. What percentage o patients with this lesion


have more than one?

3. What is the most common location o these


lesions?

4. What are relative contraindications to


endovascular coiling?

5. What is the li etime risk o aneurysm rupture?

179
Basilar tip aneurysm Case 77 (3504)
Case ranking/dif culty: Category: Subarachnoid spaces

Frontal angiogram with injection in the left vertebral artery shows


a basilar tip saccular aneurysm (arrow).

Axial CT image shows hyperdensity within the suprasellar cistern Pearls


consistent with subarachnoid blood (arrowheads). There is • Only 10%-15% o intracranial aneurysms arise rom
prominence in the prepontine cistern at the location of the basilar the posterior circulation with a majority o them being
tip (arrow). basilar tip aneurysms.
• 20% o patients with aneurysms are multiple.
Answers • 80%-90% o nontraumatic subarachnoid hemorrhage
is due to aneurysm rupture with a “thunderclap” severe
1. Subarachnoid hemorrhage rom aneurysm rupture can headache.
demonstrate increased susceptibility on gradient echo • Vasospasm occurs in 5-10 days a ter subarachnoid
or susceptibility-weighted imaging and incomplete hemorrhage in up to 70% o patients and is a
suppression (bright signal) on FLAIR. signif cant contributor to morbidity and mortality rom
2. Approximately 20% o patients with an aneurysm will brain ischemia.
have multiple. • CT angiography is >95% sensitive or aneurysms >2
mm and 3D time o ight MRA is >90% sensitive or
3. 85%-90% o intracranial aneurysms present in the aneurysms >3 mm.
anterior circulation. Intracranial saccular aneurysms are • Digital subtraction angiography remains the gold
thought to be more common due to lack o the internal standard as a workup or treatment.
elastic lamina.
4. Relative contraindications to endovascular coiling o
Suggested Readings
intracranial aneurysms include a wide neck, existence
o branch vessels arising rom the aneurysm, and MCA S orza DM, Putman CM, Scrivano E, Lylyk P, Cebral
aneurysms. JR. Blood- ow characteristics in a terminal basilar
tip aneurysm prior to its atal rupture. AJNR Am J
5. The risk o aneurysm rupture is correlated with size; Neuroradiol. 2010 Jun;31(6):1127-1131.
however, there is a li etime risk o 1%-2% per year, Tsurumi A, Tsurumi Y, Negoro M, et al. Delayed rupture
cumulative regardless o size. o a basilar artery aneurysm treated with coils: case
report and review o the literature. J Neuroradiol. 2013
Mar;40(1):54-61.
180
63-year-old male with syncope and hypotension

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the etiology o this entity?

5. What is the treatment or this entity?


181
Arachnoid granulation Case 78 (3550)
Case ranking/dif culty: Category: Subarachnoid spaces

Axial CT image shows a low attenuation lesion within the left Axial CT bone window image demonstrates smooth erosion of
transverse sinus (arrow). the inner table of calvarium (arrow)

3. The di erential diagnosis includes arachnoid


granulation, thrombus, and skull metastasis.
4. Arachnoid granulations may be a normal variant, or
developmental rom CSF pulsations.
5. No treatment is typically needed or this entity. Aberrant
AGs do not communicate with the dural venous sinus
and can rarely cause CSF leaks, requiring surgical repair.

Pearls
• Arachnoid granulation (AG) is an enlarged arachnoid
villus that is macroscopic.
• Smooth inner table erosion.
• The most common location or AG is the transverse
sinus (le t greater than right).
• Follows CSF on MR.
Coronal CT reformat shows the low-density lesion with smooth • AG can mimic thrombus in dural venous sinus but is
inner table erosion (arrow). usually round or oval.
• Sinus thrombus is typically long, cylinder shape.

Answers
1. The abnormality is located within the transverse sinus.
Suggested Reading
2. An arachnoid granulation (AG) on imaging classically Kan P, Stevens EA, Couldwell WT. Incidental giant
ollows CSF on CT and MRI. There ore on MR imaging, arachnoid granulation. AJNR Am J Neuroradiol. 2006
they have high T2 signal and low T1 signal. On CT, they Aug;27(7):1491-1492.
will be hypodense and show inner table smooth calvarial
erosion. AGs do not show enhancement.

182
58-year-old female with stroke like symptoms

1. Where is the abnormality located?

2. What are enhancement characteristics o this


entity?

3. What is the di erential diagnosis?

4. What material is within this entity?

5. What is the treatment or this entity?


183
Choroid plexus cyst Case 79 (3361)
Case ranking/dif culty: Category: Ventricles and cisterns

Axial T2 image shows within the choroid Axial T2 inversion recovery image Axial T1 shows nonenhancement of the
plexus are bilateral high signal lesions demonstrates the choroid plexus mass-like well-circumscribed cystic lesions
(arrows). bilateral lesions have low signal that is involving the atria of the bilateral lateral
higher than CSF. ventricles (arrows).

4. Choroid plexus cysts (CPC) are lipid f lled (lipid-


laden histiocytes) and/or with desquamating choroid
epithelium.
5. No treatment is needed or this entity.

Pearls
• Choroid plexus cysts (CPC) are the most common
o all neuroepithelial cysts.
• CPC are usually bilateral.
• On MRI, CPC can be slightly hyperintense on T1 and
T2 compared to CSF.
• Enhancement is variable rom none to ring to solid.
• 60%-80% will show di usion restriction.

Axial DWI shows restriction within the choroid plexus on the left
(arrow). Suggested Reading
Osborn AG, Preece MT. Intracranial cysts: radiologic-
Answers pathologic correlation and imaging approach. Radiology.
2006 Jun;239(3):650-664.
1. The abnormality is located within the lateral ventricles,
involving the choroid plexus.
2. Enhancement is variable rom none to ring or solid.
3. The di erential diagnosis includes arachnoid cyst,
choroid plexus cyst, and ependymal cyst.

184
35-year-old male with a headache

1. Where is the abnormality located?

2. What are the classic imaging f ndings?

3. What is the di erential diagnosis?

4. What is the etiology or this f nding?

5. What is the treatment or this entity?

185
Cavum velum interpositum Case 80 (3414)
Case ranking/dif culty: Category: Ventricles and cisterns

Pearls
• Cavum velum interpositum (CVI) is a normal variant
o the ventricular system that occurs when the tela
choroidea ails to use in etal li e.
• CVI is also called cavum velum triangulare and cyst o
the velum interpositum.
• Triangle-shaped CSF space between lateral ventricles.
• Splays and elevates the ornix.
• Consider epidermoid i peripheral contrast
enhancement and di usion restriction.
• On imaging, CVI will ollow CSF signal and
attenuation.
• CVI does not enhance.

Suggested Readings
Ciołkowski MK. Cavum velum interpositum, cavum septum
pellucidum and cavum Vergae: a review. Childs Nerv Syst.
2011 Dec;27(12):2027-2028; author reply 2029.
Tubbs RS, Krishnamurthy S, Verma K, et al. Cavum
velum interpositum, cavum septum pellucidum,
and cavum vergae: a review. Childs Nerv Syst. 2011
Axial CT image shows triangular CSF collection between both Nov;27(11):1927-1930.
lateral ventricles (asterisk). The collection splays the columns of
the fornix (arrows).

Answers
1. The abnormality is located between the lateral ventricles.
2. On imaging, the cavum velum interpositum will ollow
CSF on CT and all pulse sequences on MRI. There ore,
high signal on T2 and low signal on T1.
3. The di erential diagnosis includes arachnoid cyst,
cavum velum interpositum, and cavum vergae.
4. Cavum velum interpositum is a normal variant o the
ventricular system, with congenital ailure o usion o
the double layers o the tela choroidea.
5. No treatment is usually needed or this normal variant.
Cysts o the velum interpositum may be large enough
to cause hydrocephalus, or which shunting may be
necessary.

186
Asymptomatic 55-year-old female with a recent fall

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the etiology o this entity?

5. What is the treatment or this entity?

187
Cavum septum pellucidum with cavum vergae Case 81 (3417)
Case ranking/dif culty: Category: Ventricles and cisterns

Pearls
• Cavum vergae (CV) is a normal variant o the
ventricular system (also called Verga ventricle, sixth
ventricle, and cavum psalterii).
• Rectangular-shaped CSF space between lateral
ventricles.
• CV typically never occurs without cavum septum
pellucidum (CSP).
• 100% o etal age o 6 months have CV, 30% at term,
and <1 % o adults.
• 100% o premature in ants have CSP, with 85% at
term, typically with normal progressive usion and
residual 1%-20% in adults.
• On imaging, the CV will ollow CSF on CT and all
pulse sequences on MRI.

Suggested Readings
Ciołkowski MK. Cavum velum interpositum, cavum septum
pellucidum and cavum Vergae: a review. Childs Nerv Syst.
2011 Dec;27(12):2027-2028; author reply 2029.
Tubbs RS, Krishnamurthy S, Verma K, et al. Cavum
Axial CT image shows rectangular CSF collection located between
lateral ventricles extending from the genu of the corpus callosum velum interpositum, cavum septum pellucidum,
to the splenium (asterisk), bound by the septi pellucidi lea ets and cavum vergae: a review. Childs Nerv Syst. 2011
anteriorly (arrows), and fornices posteriorly (arrowheads). Nov;27(11):1927-1930.

Answers
1. The abnormality is located between the lateral ventricles.
2. On imaging, the cavum septi pellucidi (CSP) with
cavum vergae (CV) will ollow CSF on CT and all
pulse sequences on MRI. It will not enhance a ter
administration o contrast. CSP is only between the
rontal horns o the lateral ventricle while the addition
o CV will extend back to the splenium between the
ornices.
3. The di erential diagnosis includes cavum septum
pellucidum et vergae, arachnoid cyst, and cavum velum
interpositum.
4. Cavum septum pellucidum with cavum vergae is a
congenital normal variant o the ventricular system, due
to ailure o usion.
5. There is no treatment needed or this entity. Rarely cystic
enlargement may cause mass e ect.

188
57-year-old male with positional headaches

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What are presenting symptoms or this lesion?

5. What is the treatment or this entity?

189
Colloid cyst Case 82 (3026)
Case ranking/dif culty: Category: Ventricles and cisterns

Pearls
• Colloid cysts (CC) are benign cysts derived rom
endoderm and lined by a pseudostratif ed epithelium
with scattered cilia which are f lled with a viscous
gel (a combination o mucin, blood products, and
cholesterol crystals).
• 99% o CC are within the oramen o Monro/anterior
third ventricle.
• This can result in sudden death o the patient rom
rapid hydrocephalus.
• Always check or hydrocephalus or lateral ventricle
asymmetry.
• CC can be di erentiated rom other lesions with
contrast, showing no or mild rim enhancement.

Suggested Readings
Armao D, Castillo M, Chen H, Kwock L. Colloid cyst o the
third ventricle: imaging-pathologic correlation. AJNR Am
J Neuroradiol. 2000 Sep;21(8):1470-1477.
Osborn AG, Preece MT. Intracranial cysts: radiologic-
pathologic correlation and imaging approach. Radiology.
2006 Jun;239(3):650-664.
Wilms G. MR imaging o colloid cysts o the third ventricle.
AJNR Am J Neuroradiol. 2001 Sep;22(8):1632.

Axial CT noncontrast image shows a spherical focal high


attenuated structure in the anterior third ventricle at the foramen
of Monro (arrow).

Answers
1. The abnormality is located at the anterior third ventricle
roo at the oramen o Monro.
2. The classic imaging f ndings or this entity are a high-
density lesion on CT within the anterior third ventricle at
the oramen o Monro.
3. A nonenhancing cystic lesion at this site is characteristic
or colloid cyst. Other cystic di erentials can include a
choroid plexus cyst or subependymoma (cyst-like mass).
CSF ow arti acts on MRI also occur at this location.
4. More than hal o patients may present with headaches,
which may be positional.
5. These patients are ollowed and only surgically resected
when symptomatic.

190
37-year-old male with fever, altered mental status, nausea, and headache

1. What is the di erential diagnosis?

2. What imaging sequences are sensitive or


layering pyogenic debris?

3. What is the most common class o pathogens


in immunocompetent patients?

4. What is a common iatrogenic cause o this


disease?

5. What age group has a high association o this


disease with meningitis?
191
Ventriculitis Case 83 (3522)
Case ranking/dif culty: Category: Ventricles and cisterns

Axial T2 image shows heterogeneous Sagittal FLAIR image demonstrates incomplete Axial T1 postcontrast image
material within the left lateral suppression of the left lateral ventricle uid with demonstrates ependymal enhancement
ventricle with adjacent T2 adjacent T2 hyperintensity (arrow). of the left lateral ventricle (arrows).
hyperintense edema (arrow).
4. Intraventricular catheters are a common cause o
ventriculitis and can be seen in up to 20% o ventricular
catheters. These patients may have little initial clinical
symptoms.
5. 80%-90% o bacterial meningitis in in ants less than 6
months are associated with ventriculitis.

Pearls
• Ventriculitis is an ependymal ventricular in ection that
may be an extension o cerebral abscess or meningitis,
or a complication o neurosurgical procedure, typically
involving an intraventricular catheter
• Etiology is most commonly bacterial, with
ungal or viral ventriculitis occurring mostly in
immunosuppressed patients.
• The dependent debris within the ventricles may be
Axial DWI b = 1000 image shows restricted di usion within the
left lateral ventricle from pyogenic material (arrows). There is also hyperdense on CT, hyperintense on T1, hypointense on
a left occipital parafalcine focal subdural empyema (arrowhead). T2, hyperintense on FLAIR, with restricted di usion.
• Postcontrast imaging may show enhancing ventricular
ependymal margins with associated T2 hyperintense
Answers
edema.
1. The di erential or ependymal enhancement o the • Ventriculitis may carry a high mortality rate especially
ventricular walls includes ventriculitis, ependymal when associated with extension o CNS in ection.
carcinomatosis, and CNS lymphoma.
2. DWI and FLAIR are sensitive or dependent layering Suggested Readings
pyogenic debris and can show restricted di usion and
Mohan S, Jain KK, Arabi M, Shah GV. Imaging o
hyperintensity on FLAIR.
meningitis and ventriculitis. Neuroimaging Clin N Am.
3. Fungal and viral ventriculitis are commonly seen in 2012 Nov;22(4):557-583.
immunocompromised patients with bacterial ventriculitis Vandesteen L, Drier A, Galanaud D, et al. Imaging f ndings
more common in the immunocompetent. o intraventricular and ependymal lesions. J Neuroradiol.
2013 Oct;40(4):229-244.
192
10-year-old boy with VP shunt placement, presents with headache

1. What is the di erential diagnosis?

2. What is the clinical presentation?

3. What are complications o shunt placement?

4. What are initial imaging modalities to evaluate


shunt mal unction?

5. What percentage o patients will need multiple


shunt revisions?

193
CSF shunt complication Case 84 (3325)
Case ranking/dif culty: Category: Ventricles and cisterns

CT image 1 month ago demonstrates right frontal ventriculostomy CT image demonstrates a hypodense cystic collection (white
catheter (white arrow) with slit-like appearance of the lateral asterisk) along the right frontal ventriculostomy catheter (white
ventricles (white arrowheads). arrow) with mass e ect on the adjacent brain parenchyma (black
arrowhead), suggestive of CSF pseudocyst. There is slight increase
Answers in the size of the lateral ventricles bilaterally (white arrowheads).
1. A CSF density collection along the ventriculostomy
catheter in conjunction with increased size o the
ventricle is highly suggestive o VP shunt mal unction • Clinical presentations o shunt mal unction include
with a CSF pseudocyst. Abscess may be considered but headache, vomiting, neuropsychologic, cognitive, and
is unlikely given lack o parenchymal edema. Arachnoid behavioral impairment.
cyst does not typically grow rapidly. • CT is usually an initial imaging modality to evaluate
2. Clinical presentations o shunt mal unction include shunt mal unction by evaluation o ventricular size
headache, vomiting, lethargy, irritability, increasing head compared to the prior study.
size, and seizure. • Tc-99m sul ur colloid or DTPA is an available option
to evaluate shunt unction. However, it takes more time
3. Complications o VP shunt placement include in ection, and needs a gamma camera to obtain images.
mechanical complications (occlusion, disconnection • Shunt series radiographs are use ul to evaluate shunt
and racture, migration), improper placement, and disconnections or ractures.
overdrainage. • Disconnection usually occurs at the connector while
4. CT brain and shunt series radiographs can evaluate or shunt racture usually occurs in the area o greater
change in ventricular size and continuity o the shunt. mobility.
Nuclear medicine shunt study can evaluate the unction • Chronic overdrainage can cause noncompliance o the
and patency o the shunt. ventricle, which do not signif cantly enlarge even with
shunt obstruction.
5. 50% o patients will need multiple shunt revisions, with • Acute overdrainage can present with subdural
progressively shorter intervals to next shunt ailure. hematomas.

Pearls
• Ventriculoperitoneal shunt is the most commonly used Suggested Reading
shunt to bypass obstruction o the CSF. Goeser CD, McLeary MS, Young LW. Diagnostic imaging
• Complications include shunt obstruction, mal unction, o ventriculoperitoneal shunt mal unctions and
ractures, in ections, or overdrainage. complications. Radiographics. 2000 Oct;18(3):635-651.

194
8-year-old female with headache and vertigo

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the signif cance o periventricular low


attenuation in this entity?

5. What is the treatment or this entity?

195
Intraventricular obstructive hydrocephalus Case 85 (3460)
Case ranking/dif culty: Category: Ventricles and cisterns

CT axial image shows the fourth ventricle e aced by a mass CT axial image shows enlargement of the lateral ventricles with
(arrow). This results in dilation of the frontal horns and temporal periventricular hypodensity (arrows). This indicates interstitial CSF,
horns of the lateral ventricles (asterisks) and third ventricle. seen in an acute, uncompensated obstructive process.

Answers
1. The lateral and third ventricles are abnormal in size with Pearls
associated periventricular edema due to obstruction by a • Intraventricular obstructive hydrocephalus = “non-
mass in the ourth ventricle. communicating,” obstruction proximal to the oramina
o Luschka and Magendie.
2. On imaging, at least the lateral ventricular size will be
• Extraventricular hydrocephalus = “communicating,”
enlarged with both intraventricular and extraventricular
typically due to decreased CSF absorption at the
hydrocephalus. With intraventricular obstruction,
arachnoid granulations.
depending upon where the obstruction is located, the
• CSF signal around the ventricular horns signif es an
ventricles downstream will be e aced or normal in size. In
uncompensated, acute process.
extraventricular obstruction, all the ventricles are enlarged.
• Chronic hydrocephalus will typically not have
3. The di erential diagnosis includes obstructive periventricular interstitial CSF due to compensation
hydrocephalus, normal-pressure hydrocephalus, and over time.
atrophy. • Size o ventricles does not correlate with intracranial
pressure.
4. The signif cance o periventricular low attenuation,
which appears as a “halo” o water signal or low
attenuation, is the ow o CSF retrograde through the
ependymal lining o the ventricle. This indicates an Suggested Readings
acute, uncompensated process.
Glastonbury CM, Osborn AG, Salzman KL. Masses and
5. The treatment or hydrocephalus is CSF diversion mal ormations o the third ventricle: normal anatomic
typically with an intraventricular shunt or relationships and di erential diagnoses. Radiographics.
ventriculostomy to relieve the acute obstruction. I 2011;31(7):1889-1905.
possible, the cause o obstruction should be addressed. Siddiqui A, Chew NS, Miszkiel K. Vertebrobasilar
dolichoectasia: a rare cause o obstructive hydrocephalus:
case report. Br J Radiol. 2008 Apr;81(964):e123-e126.
Uluğ AM, Truong TN, Filippi CG, et al. Di usion imaging in
obstructive hydrocephalus. AJNR Am J Neuroradiol. 2003
Aug;24(6):1171-1176.

196
3-year-old male with trauma, incidental lesion on head CT

1. What is the di erential diagnosis or this


f nding in a young child?

2. What location is the most common or these


tumors?

3. What are typical MRI f ndings or this tumor?

4. In which patient age group is this tumor the


most common?

5. What syndromes are associated with this


tumor?

197
Choroid plexus papilloma Case 86 (3366)
Case ranking/dif culty: Category: Ventricles and cisterns

Axial T2 image shows the well-circumscribed, lobular right


intraventricular mass, which is predominantly T2 hypointense likely
Axial CT image shows a hyperdense right intraventricular mass at due to calci cation (white arrow). There is adjacent periventricular
the atria (arrow). T2 hyperintensity (white arrowhead). There is an unrelated epidural
hematoma from a history of trauma (red arrow).

Axial T1 image shows increased T1 signal of the lobular Axial susceptibility-weighted image shows signi cant hypointensity
intraventricular mass (arrow). without blooming, consistent with calci cation (arrow).
198
Choroid plexus papilloma (Cont.) Case 86 (3366)

Axial T1 postcontrast image shows intense enhancement of the Axial pre- and postcontrast subtraction image shows the
tumor (arrow). enhancement beyond precontrast T1 shortening (arrow).

Answers
1. Choroid plexus papillomas and carcinomas are not Pearls
reliably distinguishable on imaging, but are the • Choroid plexus tumors can appear in all ages but are
most likely diagnoses or this well-circumscribed most common in young children.
intraventricular tumor in a young child. Supratentorial • Choroid plexus papillomas are the most common
ependymoma occur in children but tend to be tumor in a child less than 1 year o age.
periventricular. Intraventricular meningiomas can appear • They are typically lobulated, well-circumscribed
similar but is primarily a tumor occurring in adults. intraventricular tumors with intense enhancement.
2. The commonest occurrence o choroid plexus tumors • Calcif cation is seen in 25% with increased T1 and
also coincides with the amount o choroid tissue by decreased T2 signal on MR imaging.
location. In decreasing order o requency, choroid • The atrium is the most common location ollowed by
plexus tumors occur in the trigone o the lateral ourth and third ventricles.
ventricle, ourth ventricle, and third ventricle. • No neuroimaging criteria currently exist to di erentiate
papillomas rom carcinomas.
3. Intense enhancement is typical or choroid plexus
papillomas. Calcif cation is seen in 25% o tumors that
demonstrate decreased T2 and increased T1 signal.
Periventricular edema and invasion are not reliable Suggested Readings
di erentiators between papillomas, atypical papillomas, Naeini RM, Yoo JH, Hunter JV. Spectrum o choroid
and carcinomas. plexus lesions in children. AJR Am J Roentgenol. 2009
4. Choroid plexus tumors occur most commonly in younger Jan;192(1):32-40.
children and are the most common brain tumor under 1 Pereira DB, Gasparetto EL, Marcondes de Souza J, Chimelli
year o age. Choroid plexus tumors have been described L. Choroid plexus papilloma with osseous metaplasia as
in etal imaging, also making it a common congenital a di erential diagnosis o calci ying pseudoneoplasms
CNS tumor. o the neuraxis. AJNR Am J Neuroradiol. 2010
5. Increased incidence o choroid plexus tumors are seen in Jun;31(6):E51-E52; author reply E53.
Li-Fraumeni and Aicardi syndromes.
199
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13-year-old female with history of long-standing seizures, now with
new-onset vomiting

1. What is the di erential diagnosis?

2. What other lesional f ndings narrow the


di erential diagnosis or the intraventricular
tumor?

3. What are typical neuroimaging characteristics


o this tumor?

4. What is the prognosis or this tumor?

5. What is the WHO grade or this neoplasm?


201
Subependymal giant cell astrocytoma Case 87 ( 3379)
Case ranking/dif culty: Category: Ventricles and cisterns

Axial FLAIR image shows a heterogeneous mass in the right Axial T2 image shows iso- to hypointense signal of the solid
lateral ventricle arising near the foramen of Monro with T2 nodule (arrow). There are multiple hypointense subependymal
hyperintensity of the solid component (white arrow). There are nodules, which are calci ed (arrowheads).
associated cysts (white arrowhead). Multiple T2 hyperintense
lesions are also noted in the cortical and subcortical areas in the
frontal and parietal lobes (red arrowheads).

Axial DWI b = 1000 image shows the right ventricular mass has
increased signal (arrow) representing decreased di usion relative
to the other subependymal nodules (arrowhead).
Coronal T1 postcontrast image shows the enhancing mass
near the right foramen of Monro (arrow). There is a second
contralateral smaller enhancing mass (arrowhead).
202
Subependymal giant cell astrocytoma (Cont.) Case 87 ( 3379)

Pearls
• Subependymal giant cell astrocytomas (SEGA) are
low-grade neoplasms and are almost always associated
with tuberous sclerosis.
• Up to 15% o tuberous sclerosis patients will have
SEGAs, which is also the most common CNS tumor in
tuberous sclerosis.
• SEGAs always occur at the oramen o Monro,
demonstrate slow growth, and are larger than 1 cm.
• These tumors are usually lobular and well marginated.
• They can have heterogeneous signal intensity with
calcif cation and cysts.
• Intense enhancement o solid components is typical.
• SEGAs may show decreased di usion relative to other
subependymal nodules.
• Obstructive hydrocephalus is a common presenting
symptom.
• Spontaneous or intraoperative hemorrhage is a
signif cant risk actor.
Axial FLAIR image near the vertex shows multiple cortical and
subcortical areas of T2 hyperintensity associated with gyral
thickening (arrows) consistent with cortical tubers. Suggested Readings
Jelinek J, Smirniotopoulos JG, Parisi JE, Kanzer M. Lateral
Answers ventricular neoplasms o the brain: di erential diagnosis
based on clinical, CT, and MR f ndings. AJR Am J
1. Intraventricular tumors should include subependymal
Roentgenol. 1990 Aug;155(2):365-372.
giant cell astrocytoma, choroid plexus tumor, and central
Roth J, Roach ES, Bartels U, et al. Subependymal giant
neurocytoma in the di erential.
cell astrocytoma: diagnosis, screening, and treatment.
2. Location o the tumor at the oramen o Monro, f ndings Recommendations rom the International Tuberous
o other subependymal nodules, and cortical tubers Sclerosis Complex Consensus Con erence 2012. Pediatr
narrow the di erential to subependymal giant cell Neurol. 2013 Dec;49(6):439-444.
astrocytoma in a tuberous sclerosis patient.
3. Subependymal giant cell astrocytomas are greater than
1 cm, located exclusively at the oramen o Monro,
typically lobular and well circumscribed with intense
enhancement o the solid components.
4. Surgical resection is usually curative or subependymal
giant cell astrocytoma. Recurrence is uncommon.
Chemotherapy and radiation are usually not indicated.
5. Subependymal giant cell astrocytomas are low-grade,
WHO grade I tumors. While case reports o atypia have
been described, these may not have signif cant impact on
prognosis, which is usually excellent in regard to the tumor.

203
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38-year-old female with right hearing loss

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What are possible presenting symptoms?

5. What is the treatment or this entity?

205
Cerebellar pontine angle lipoma Case 88 (3218)
Case ranking/dif culty: Category: IAC and CP angle

Axial T1 noncontrast image shows a bright subcentimeter lesion Axial T1 postcontrast with fat saturation shows that the
centered at the right internal auditory canal (arrow). subcentimeter lesion demonstrates loss of signal on fat saturation
images (arrow). Note there is no enhancement of the lesion.

Answers
1. The abnormality is located within the internal auditory
canal.
Pearls
• Cerebellar pontine angle (CPA) lipomas are
2. The classic imaging f ndings or this entity include a uncommon lesions but they can mimic schwannomas.
classic homogeneous atty appearance. Lipomas will • These lesions are no di erent than other lipomas
ollow at on all MR pulse sequences and have high within the skull at other locations and are rom
signal on T1 and ast spin echo T2. Fat saturation is maldevelopment o the meninx primitiva.
conf rmatory and shows loss o signal. Typically, no • Fat sat images are conf rmatory and show loss o
contrast enhancement is seen. signal.
3. The di erential diagnosis includes schwannoma, • I the CPA lesion enhances, consider schwannoma.
dermoid cyst, and lipoma. • I the CPA lesion restricts di usion, consider
epidermoid cyst.
4. Patients with a mass in the internal auditory canal
may present with sensorineural hearing loss, vertigo,
headache, or acial muscle weakness.
Suggested Reading
5. Surgical removal is needed i the lesion is symptomatic.
Bonneville F, Sarrazin JL, Marsot-Dupuch K, et al. Unusual
lesions o the cerebellopontine angle: a segmental
approach. Radiographics. 2001;21(2):419-438.

206
45-year-old female with a headache

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the etiology o this f nding?

5. What is the treatment or this entity?

207
Petrous apex cephalocele Case 89 (3362)
Case ranking/dif culty: Category: IAC and CP angle

Axial T2 image shows multiloculated uid signal lesion Axial T1 postcontrast shows no enhancement of the uid- lled
connecting the right Meckel cave and the petrous apex. A similar lesion connecting the right Meckel cave and the petrous apex.
lesion is seen on the left (arrows). A similar lesion is seen on the left (arrow).

Answers
• PAC is contiguous with the trigeminal cave.
1. The abnormality is located within the trigeminal
• Identi ying the epicenter o the cyst outside o the
(Meckel) cave.
petrous apex makes mucocele and cholesteatoma
2. On MRI, petrous apex cephalocele will ollow CSF with unlikely.
corresponding low T1 and high T2 signal. These lesions • On imaging, PAC will ollow CSF signal and
do not enhance. The key to diagnosis is noting the attenuation.
connection between the trigeminal cave and the petrous • PAC lesions do not enhance.
apex cyst. • The key to diagnosis is noting the connection between
the trigeminal cave and the PA.
3. The di erential diagnosis includes petrous apex
• PAC may be associated with idiopathic intracranial
cephalocele, mucocele, cholesterol granuloma, and
hypertension.
cholesteatoma.
4. Petrous apex cephalocele (PAC) are either acquired or
congenital herniation o the trigeminal cave contents into
the petrous apex. Suggested Readings
5. No treatment is usually needed or this entity. On Bialer OY, Rueda MP, Bruce BB, Newman NJ, Biousse
occasion, i complicated by in ection or CSF leak, then V, Saindane AM. Meningoceles in idiopathic
surgery may be necessary. intracranial hypertension. AJR Am J Roentgenol. 2014
Mar;202(3):608-613.
Lin BM, Aygun N, Agrawal Y. Imaging case o the month:
cystic lesions o the petrous apex: identif cation based
Pearls on magnetic resonance imaging characteristics. Otol
• Petrous apex cephalocele (PAC) are cystic Neurotol. 2012 Dec;33(9):e75-e76.
structures that occur in the trigeminal (Meckel) cave Moore KR, Fischbein NJ, Harnsberger HR, et al. Petrous
representing arachnoid cysts or meningoceles that apex cephaloceles. AJNR Am J Neuroradiol. 2009
erode into the petrous apex (PA) Feb;22(10):1867-1871.
• PAC is a “don’t touch lesion.”

208
51-year-old female with right hearing loss

1. Where is the abnormality located?

2. This entity is best diagnosed on MRI with


which sequence?

3. What is the di erential diagnosis or a cystic


extraaxial lesion in this location?

4. This entity can have what e ect on adjacent


nerves?

5. Where are the most common intracranial


locations o this entity?

209
Epidermoid cyst Case 90 (3012)
Case ranking/dif culty: Category: IAC and CP angle

Axial T1 image demonstrates Axial T2 image demonstrates CSF Axial DWI b-1000 and ADC images demonstrate restricted
anterior displacement of the isointensity of the mass (arrow) in the di usion (increased DWI signal and dark ADC) of the mass
seventh or eighth cranial nerve right cerebellopontine angle. in the right cerebellopontine angle (arrows).
(red arrow) by a CSF isointense
mass (green arrow) in the right
cerebellopontine angle. 5. EC are most commonly located in the cerebellopontine
angle. The second most common location is the ourth
ventricle ollowed by the sella.

Pearls
• Epidermoid cysts (EC) are due to an accumulation
o keratin and cholesterol with a stratif ed squamous
epithelial lining.
• EC are the third most common CPA tumors.
• Use FLAIR and DWI to di erentiate EC rom
arachnoid cyst.
• Use T1 to di erentiate EC rom dermoid.
• EC encase nerves and vessels.

Coronal FIESTA image demonstrates an obvious mass that is of Suggested Readings


intermediate signal in the right cerebellopontine angle (arrow).
Bonneville F, Sarrazin JL, Marsot-Dupuch K, et al. Unusual
lesions o the cerebellopontine angle: a segmental
Answers approach. Radiographics. 2009 Nov;21(2):419-438.
1. The abnormality is located in the cerebellopontine angle. Kallmes DF, Provenzale JM, Clo t HJ, McClendon RE.
Typical and atypical MR imaging eatures o intracranial
2. Epidermoid cysts (EC) are readily seen on di usion- epidermoid tumors. AJR Am J Roentgenol. 1997
weighted imaging, and demonstrate incomplete Sep;169(3):883-887.
suppression on FLAIR. They usually ollow CSF signal Osborn AG, Preece MT. Intracranial cysts: radiologic-
on standard T2 and T1 imaging and are di f cult to pathologic correlation and imaging approach. Radiology.
visualize. 2006 Jun;239(3):650-664.
3. The di erential diagnosis includes arachnoid cyst,
epidermoid cyst, and dermoid.
4. EC can encase adjacent nerves, compared to arachnoid
cyst that displaces the nerves.

210
9-year-old male with ascending paralysis followed by facial droop

1. What is the di erential diagnosis?

2. What is the typical clinical presentation?

3. How o ten are cranial nerves involved in this


disease process?

4. What is the pathophysiology o this disease?

5. What is the prognosis or the disease?

211
Guillain-Barre Case 91 (3242)
Case ranking/dif culty: Category: IAC and CP angle

Axial T1 postcontrast through the pons shows Axial T1 postcontrast through the internal Sagittal T1
thickened and enhancing bilateral trigeminal auditory canals shows bilateral enhancement postcontrast of the
nerves (black arrows). This is a case of Guillain- of the labyrinthine segments of the facial lumbar spine shows
Barre syndrome. nerves (white arrows). di use enhancement
of the ventral nerve
roots of the cauda
equina (white arrows).
4. Guillain-Barre is an immune-mediated in ammation
o the myelin sheath o peripheral nerves induced rom
prior exposure to a oreign antigen.
5. In Guillain-Barre, 75%-80% o patient ully recover
within a year. Mortality is at 2%-6% with severe
disability in 5% o patients.

Pearls
• Symmetric bilateral cranial nerve enhancement is
likely to be in ammatory.
• With a history o ascending paralysis, acute
in ammatory demyelinating polyneuropath (AIDP)
should be considered.
Axial T1 postcontrast through the L1 vertebral body level shows
enhancement of the ventral nerve roots of the cauda equina • Cranial nerves are involved in 45%-75% o AIDP
(white arrows). (Guillain-Barre).
• Classic f ndings o AIDP include di use enhancement
o the ventral nerve roots.
Answers
• Clinical history o a recent prior in ection is typical.
1. The di erential or di use cranial nerve and peripheral
nerve enhancement includes both acute and chronic
Suggested Readings
in ammatory demyelinating polyneuropathy,
carcinomatous neural invasion, and radiation neuritis. Li HF, Ji XJ. The diagnostic, prognostic, and di erential value
o enhanced MR imaging in Guillain-Barre syndrome. AJNR
2. The classic presentation o Guillain-Barre is a rapidly Am J Neuroradiol. 2011 Aug;32(7):E140; author reply E141.
progressive paralysis a ter a bacterial or viral in ection. Zuccoli G, Panigrahy A, Bailey A, Fitz C. Redef ning the
3. Cranial nerve involvement is common in Guillain-Barre, Guillain-Barré spectrum in children: neuroimaging
in 45%-75% o cases. f ndings o cranial nerve involvement. AJNR Am J
Neuroradiol. 2011 Apr;32(4):639-642.
212
35-year-old male with unilateral facial weakness

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the etiology o this entity?

5. What is the treatment or this entity?

213
Bell palsy Case 92 (3517)
Case ranking/dif culty: Category: IAC and CP angle

Pearls
• Bell palsy is an in ammatory process involving the
seventh cranial nerve ( acial nerve) characterized by
the acute onset o unilateral acial paralysis.
• Labyrinthine and intracanalicular segment
enhancement o the seventh cranial nerve should be
considered abnormal.
• Mild enhancement o the geniculate ganglion,
tympanic, and mastoid segments is normal
(use contralateral nerve or comparison).
• Assess the acial nerve or a ocal mass.
• Labyrinth, seventh, and eighth cranial nerve
enhancement consider Ramsay Hunt syndrome.
• Both acial nerves may enhance but typically the
symptomatic side enhances more intensely.
• Facial nerve enhancement may also be seen with a
temporal bone racture.

Axial T1 postcontrast with fat saturation image through the Suggested Readings
internal auditory canal shows asymmetric enhancement
involving the seventh cranial nerve. Speci cally, enhancement is Al-Noury K, Lot y A. Normal and pathological f ndings or
seen involving the distal intracanalicular segment, labyrinthine the acial nerve on magnetic resonance imaging. Clin
segment, geniculate ganglion (red arrow), and proximal tympanic Radiol. 2011 Aug;66(8):701-707.
segment. Tien R, Dillon WP, Jackler RK. Contrast-enhanced MR
imaging o the acial nerve in 11 patients with Bell’s
Answers palsy. AJR Am J Roentgenol. 1990 Sep;155(3):573-579.

1. The abnormality involves the seventh cranial nerve.


2. On MRI, the acial nerve will demonstrate asymmetric
linear contrast enhancement rom the intracanalicular
segment through the temporal bone. CT is typically not
use ul as the f ndings o neuritis are not seen.
3. The di erential diagnosis includes Bell palsy,
hemangioma, and schwannoma.
4. Bell palsy is a latent herpes simplex in ection
particularly o the geniculate ganglion with in ammatory
spread to other segments o the acial nerve.
5. The treatment or this entity includes a corticosteroid
taper with possible surgical decompression reserved or
pro ound denervation.

214
45-year-old female with left sensorineural hearing loss

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis or a mass at


the cerebellopontine angle?

4. What symptoms are associated with this


entity?

5. What is the treatment or this entity?

215
Vestibular schwannoma Case 93 (3006)
Case ranking/dif culty: Category: IAC and CP angle

Axial T1 image shows a left Axial T1 postcontrast with fat Coronal FIESTA image again shows the left
cerebellopontine angle “ice-cream saturation shows the lesion within cerebellopontine angle mass (arrow) which lls the
cone” shaped mass (arrow) which is the left cerebellopontine angle is internal auditory canal.
isointense to the cerebellum. a contrast-enhancing mass with
extension into the internal auditory
canal (arrow).

Answers
1. The abnormality is located within the cerebellopontine Pearls
angle and internal auditory canal (IAC). • Vestibular schwannomas (VS) are the most common
cerebellopontine angle (CPA) mass.
2. Vestibular schwannomas (VS) have a classic “ice-cream
• Classic appearance is “ice-cream cone” shaped.
cone” shaped mass at the cerebellopontine angle and
• On CT beware o a widened internal auditory canal as
IAC. Smaller lesions may only be in the IAC while
VS may not be visible.
larger lesions extrude into the CP angle.
• VS are the most common CPA mass.
3. The di erential diagnosis or a mass at the • VS may show cystic degeneration.
cerebellopontine angle includes meningioma, epidermoid • Bilateral vestibular schwannomas are diagnostic or
cyst, and schwannoma. Vestibular schwannomas (VS) neurof bromatosis II.
are the most common cerebellopontine angle (CPA) • CT may be help ul to distinguish meningioma
mass. (hyperostosis and hyperdense) rom VS.
4. Patients with VS usually present with sensorineural
hearing loss, vertigo, or tinnitus.
5. Surgical removal vs gamma kni e is very success ul with Suggested Readings
minimal recurrence. Babu R, Sharma R, Bagley JH, et al. Vestibular
schwannomas in the modern era: epidemiology, treatment
trends, and disparities in management. J Neurosurg. 2013
Jul;119(1):121-130.
Bonneville F, Sarrazin JL, Marsot-Dupuch K, et al. Unusual
lesions o the cerebellopontine angle: a segmental
approach. Radiographics. 2009 Nov;21(2):419-438.
Silk PS, Lane JI, Driscoll CL. Surgical approaches to
vestibular schwannomas: what the radiologist needs to
know. Radiographics. 2009 Nov;29(7):1955-1970.

216
1-day-old female with a posterior head mass

1. According to some authors what bony structure


should be de ective to make this diagnosis?

2. What can be included in the sac contents?

3. What are the intracranial f ndings in this


mal ormation?

4. What is the treatment or this disease?

5. What are relative risk actors or this disease?

217
Chiari III malformation Case 94 (3250)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Sagittal T2 image at midline shows a large encephalocele


herniating through the low occipital bone involving defects
of the opisthion and C1. There is primarily dysplastic occipital
lobe (asterisks) with bilateral CSF collections (red arrows). Note
brainstem and tonsillar descent into the spinal canal (white
arrowhead). Axial T1 postcontrast of the encephalocele sac shows a midline
structure, likely falx (white arrow), with bilateral uid from
herniated occipital horns of the lateral ventricles (asterisks).
Note the enhancing choroid plexus (red arrow).

Axial T2 image at the opisthion defect shows bilateral hypoplastic


cerebellar hemispheres (white arrows), a severely elongated and Axial T2 image of the brain shows enlarged massa intermedia
distorted midbrain and tectum (asterisk), and dysplastic occipital (white arrow), and gyral interdigitation (red arrow).
lobe (red arrow).

218
Chiari III malformation (Cont.) Case 94 (3250)

Sagittal T1 postcontrast image of midline after encephalocele Axial CT of the skull base shows the opisthion defect (asterisk).
repair shows persistent herniation of occipital lobes (asterisk),
enhancing choroid (arrowhead), severe beaking of the midbrain
5. Increased risks o neural tube de ects such as olate
(white arrow), tonsillar descent (red arrow), and herniation of the
temporal lobe into the prepontine cistern (blue arrow). Note the
def ciency, through diet or genetic mutation o methylene
lack of corpus callosum. tetrahydro olate reductase, are associated with increased
incidence o Chiari mal ormations. Toxins such as
arsenic and tripterygium wil ordii (a Chinese herb) have
Answers also been shown to cause neural tube de ects.
1. Some authors suggest that the opisthion must be
involved in the low occipital/high cervical encephalocele
to diagnose a Chiari III. Chiari III was f rst described Pearls
as essentially a high cervical myelocystocele through • Chiari III includes a low occipital and high cervical
dysraphic C1-C2 levels. encephalocele in addition to other Chiari II
2. The encephalocele sac typically includes dysplastic morphologic f ndings in the brain.
cerebellum and cerebral elements, and may also • Some authors suggest that Chiari III encephaloceles
include brainstem/upper cervical cord, lateral ventricles must always involve the opisthion o the oramen
including choroid plexus, and dural venous sinuses. magnum.
• Herniated contents may include cerebellum,
3. The intracranial f ndings o Chiari III are similar to cerebrum, brainstem, and cord, or even lateral
Chiari II, including brainstem descent, tectal beaking, ventricles and dural venous sinuses.
callosal dysgenesis, and alcine enestration with gyral • Chiari II changes o the brain include tectal beaking,
interdigitation. enlarged massa intermedia, callosal dysgenesis,
4. Folate supplementation be ore and during embryo alcine enestration and gyral interdigitation,
organogenesis may help reduce the incidence o neural stenogyria and periventricular nodular heterotopia.
tube de ects associated with Chiari mal ormations.
Surgical repair or resection o the encephalocele is
indicated as long as there is more CNS tissue in the Suggested Readings
cranium than in the sac. CSF diversion o hydrocephalus Aribal ME, Gürcan F, Aslan B. Chiari III mal ormation:
is also indicated. MRI. Neuroradiology. 1996 May;38 (suppl 1):S184-S186.
Castillo M, Quencer RM, Dominguez R. Chiari III
mal ormation: imaging eatures. AJNR Am J Neuroradiol.
1996 May;13(1):107-113.
219
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20-year-old male with history of severe headache and back pain

1. What chromosome is associated with von


Hippel-Lindau disease?

2. What is the most common location o CNS


hemangioblastomas?

3. What is the classic radiographic f nding in


intracranial hemangioblastoma?

4. What is the radiographic f nding o spinal


hemangioblastoma?

5. What is the most common cause o hearing


loss in these patients?
221
von Hippel-Lindau Case 95 (3318)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Axial T1 postcontrast image Coronal T1 postcontrast image Sagittal STIR image of the Sagittal T1 postcontrast
demonstrates cystic lesions with demonstrates multiple nodular- thoracic spine demonstrates image of the thoracic
mural nodular enhancement enhancing lesions in bilateral cerebellar hyperintensity involving spine demonstrates
(white arrowheads) in the left hemispheres (white arrowheads), with the entire spinal cord several enhancing
cerebellum and right-sided cystic lesion in the left cerebellum and central spinal canal, lesions in the spinal cord
pons. Also noted are few (asterisk) and obstructive hydrocephalus. suggestive of combination (white arrowheads). The
nodular-enhancing foci in the Note that the enhancing nodules are of cord edema and associated edema is
right cerebellar hemisphere close to a pial surface, which is typical of syringohydromyelia usually out of proportion
(white arrows). hemangioblastomas due to the vascular (arrows). to the enhancing nodules.
nature of these tumors.

Answers
endolymphatic sac tumors, renal cysts and tumors,
1. von Hippel-Lindau disease is a rare, autosomal dominant
pancreatic cysts and tumors, pheochromocytomas,
inherited multisystemic disorder associated with
epididymal cystadenomas.
inactivation o a tumor suppressor gene (VHL gene)
• CNS hemangioblastoma is one o the most common
located on Chromosome 3. Sporadic mutation is ound
mani estations ollowing pancreatic cysts.
in 20% o cases.
• Typical location or CNS hemangioblastoma is
2. The most common location o CNS hemangioblastoma cerebellum (40%-70%), spinal cord (10%-60%), and
is the cerebellum (40%-70%), ollowed by the spinal medulla (5%).
cord (10%-60%) and medulla (5%). Supratentorial • Classic radiographic f ndings o CNS
lesions are less common. hemangioblastoma include cystic lesion with solid-
enhancing mural nodules, usually near a pial sur ace.
3. Classic radiographic f nding is a cystic lesion with mural
• Spinal cord lesions may be associated with
nodular enhancement, typically in the cerebellum.
syringohydromyelia, with greater than expected edema.
4. Intramedullary enhancing nodule typically on the pial • Renal cell carcinoma and neurological complications
sur ace with edema out o proportion to the size o are common causes o death.
the nodule, associated with syringohydromyelia, is a • Combination o clinical and radiographic screening
characteristic f nding o spinal hemangioblastoma. substantially decreases complications, morbidity, and
mortality.
5. The most common cause o hearing loss in VHL disease
is due to an increased risk o developing endolymphatic
sac tumors, with a tendency or bilateral lesions.
Suggested Readings
Leung RS, Biswas SV, Duncan M, Rankin S. Imaging
Pearls eatures o von Hippel-Lindau disease. Radiographics.
• Rare, autosomal dominant, inherited multisystemic 2009 Apr;28(1):65-79; quiz 323.
disorder. Slater A, Moore NR, Huson SM. The natural history
• Broad clinical mani estations involving retinal and o cerebellar hemangioblastomas in von Hippel-
central nervous system (CNS) hemangioblastomas, Lindau disease. AJNR Am J Neuroradiol. 2003
Sep;24(8):1570-1574.
222
18-year-old male with history of autism, developmental delay, and seizure

1. What is the clinical presentation o this


disease?

2. What is the di erential diagnosis o this


f nding?

3. What are typical neuroimaging f ndings in this


disease?

4. What radiographic exams are relatively


contraindicated in this disease?

5. What is the clinical course and prognosis o


this disease?

223
Ataxia telangiectasia Case 96 (3322)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Sagittal T1 image demonstrates severe atrophy of the cerebellar Coronal T2 image demonstrates cerebellar brain volume loss with
hemisphere with widening of the cerebellar folia and CSF space enlargement of extra axial CSF space (white arrows).
(white arrows). Note the hypoplastic adenoid tissue for this age
(arrowhead).

Pearls
Answers • Ataxia telangiectasia is an autosomal recessive,
1. Main clinical characteristics o ataxia complex multisystemic disorder with cerebellar
telangiectasia include progressive cerebellar ataxia, degeneration, telangiectasia ormation,
oculomucocutaneous telangiectasias, and recurrent immunodef ciency, tumor ormation, and progeria.
sinopulmonary in ection. • Clinical eatures include cerebellar ataxia,
oculocutaneous telangiectasia, and recurrent
2. Cerebellar atrophy can be caused by multiple etiologies, bronchopulmonary in ections.
rom inherited degeneration, the most common being • Ataxia is usually an initial presenting symptom and
Friedreich ataxia, to damage to the Purkinje cells rom becoming apparent when the child begins to walk.
hyperthermia, autoimmune paraneoplastic process, • Most common cause o death is recurrent
and seizure medications. This case is rom ataxia sinopulmonary in ection.
telangiectasia, which is considered a neurocutaneous • MRI is a modality o choice due to nonionizing radiation.
disorder. • Radiographic f ndings include di use cerebellar
3. Neuroimaging f ndings in ataxia telangiectasia include volume loss with compensatory enlargement
progressive cerebellar atrophy, beginning in the vermis, o the ourth ventricle, cerebral white matter
and hypoplastic or absent pharyngeal lymphoid tissue dysmyelination/demyelination, microhemorrhages, or
rom immunodef ciency. CNS angiomas and associated telangiectasias, as well as decreased or absent adenoid
hemorrhage can be seen in adults with the disease. gland, thymus, and mediastinal lymphoid tissue.

4. Patients with ataxia telangiectasia have increased


sensitivity to the DNA breakage e ects o ionizing Suggested Readings
radiation. Benef ts rom the modality o diagnostic
Farina L, Uggetti C, Ottolini A, et al. Ataxia-telangiectasia:
imaging should be care ully considered.
MR and CT f ndings. J Comput Assist Tomogr.
5. Ataxia is usually a presenting symptom in patients with 1994;18(5):724-727.
ataxia telangiectasia becoming apparent when child Lin DD, Barker PB, Lederman HM, Craw ord TO. Cerebral
begins to walk. Ataxia telangiectasia patients usually abnormalities in adults with ataxia-telangiectasia. AJNR
have recurrent sinopulmonary in ections, which is the Am J Neuroradiol. 2014 Jan;35(1):119-123.
most common cause o death. Median age at death is Wallis LI, Gri f ths PD, Ritchie SJ, Romanowski CA,
approximately 20 years. Darwent G, Wilkinson ID. Proton spectroscopy and
imaging at 3T in ataxia-telangiectasia. AJNR Am J
Neuroradiol. 2007 Jan;28(1):79-83.
224
12-month-old male with ataxia, nystagmus, alternating apnea,
and hyperpnea

1. What is the di erential diagnosis?

2. What are the classic imaging f ndings or this


entity?

3. What is the classic clinical presentation?

4. What are the subtypes o this entity?

5. What is the prognosis or this disease?


225
Joubert syndrome Case 97 (3588)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Axial T1 postcontrast image Axial T2 image shows the Sagittal T1 image shows Axial T1 noncontrast image
shows the “molar tooth sign” thickened cerebellar peduncles hypoplastic superiorly shows the “bat-wing fourth
with deep interpeduncular (arrows) and midline vermian displaced vermis (asterisk) ventricle (asterisk) and vermian
cistern (red arrow) and cleft (arrowheads). with dysmorphic fourth cleft (arrowheads).
thickened superior cerebellar ventricular roof (arrowhead).
peduncles (white arrows). Note The cerebellar hemispheres are
the leptomeningeal vessels apposed (circle).
within a midline vermian cleft
(arrowheads).

Answers
1. The di erential or vermian hypoplasia/cle ting includes Pearls
Joubert syndrome–related disorders (JSRD), Dandy- • Joubert syndrome (JS)–related disorders (JSRD) all
Walker, pontocerebellar hypoplasia, and vermian atrophy have the midbrain-hindbrain mal ormation o “molar
rom heterogeneous insult. tooth” sign and vermian cle ting.
• Multiple genes have been implicated that encode or
2. Joubert syndrome–related disorders all have the obligatory
proteins o primary cilia.
“molar tooth” midbrain mal ormation with deep
• Eye abnormalities, renal, liver, and oro aciodigital
interpeduncular cle t and elevated and thickened superior
de ects are associated with the cardinal neurologic
cerebellar peduncles. Additional f ndings include vermian
f ndings o JS and help classi y JSRD into subsets.
cle ting, and “bat wing” ourth ventricle.
• Other neuroimaging f ndings includes “bat-wing”
3. Neurologic cardinal eatures o hypotonia, ataxia, ourth ventricle, prominent CSF spaces, migrational
developmental delay, alternating apnea and tachypnea, anomalies, and abnormal myelination.
nystagmus, and intellectual disability are seen in all
JSRD.
4. JSRD includes syndromes previously considered Suggested Readings
separate entities such as Dekaban-Arima, COACH,
Senior-Loken, Varadi-Papp, and Joubert-polymicrogyria Poretti A, Huisman TA, Scheer I, Boltshauser E.
syndromes. However, recent classif cation starts with Joubert syndrome and related disorders: spectrum
pure Joubert syndrome (JS) with the cardinal neurologic o neuroimaging f ndings in 75 patients. AJNR Am J
eatures and molar tooth mal ormation. Related disorders Neuroradiol. 2011 Sep;32(8):1459-1463.
include JS with ocular de ect (retinal dystrophy), JS with Saleem SN, Zaki MS. Role o MR imaging in prenatal
renal de ect, JS with oculorenal de ects, JS with hepatic diagnosis o pregnancies at risk or Joubert syndrome
de ect, and JS with oro aciodigital de ects. and related cerebellar disorders. AJNR Am J Neuroradiol.
2010 Mar;31(3):424-429.
5. Treatment is supportive, with early death in patients
not well supported or episodes o prolonged apneas. In
most cases these respiratory abnormalities will resolve
spontaneously with renal and hepatic complications
representing major causes o death in older children.

226
15-month-old female vomiting for 3 weeks

1. What is the di erential diagnosis?

2. What are typical MRI f ndings or this entity?

3. What is the prognosis o this disease?

4. Which other tumors have similar histological


eatures rom which the name is derived?

5. What is the most common location or this


tumor?

227
Atypical teratoid rhabdoid tumor Case 98 (3333)
Case ranking/dif culty: Category: Intra-axial in ratentorial

Axial CT image of the posterior fossa shows a hyperdense mass Axial T2 image of the posterior fossa shows separate heterogeneous
(typically seen with high grade tumors) lling the fourth ventricle tumors in the left cerebellar hemisphere (arrow) with a mild amount
and extending to the left cerebellar hemisphere (arrow) causing of adjacent peritumoral T2 hyperintensity, as well as a mass anterior
obstructive hydrocephalus as demonstrated by dilated temporal to the medulla (arrowhead). Note areas of tumor which show
horns (arrowheads). decreased signal on T2, corresponding with hypercellular regions of
tumor seen with high grade malignancies.

Axial T1 post contrast of the same patient shows multiple Sagittal postcontrast image at midline shows three of the four
heterogenously enhancing tumors in the superior fourth ventricle separate heterogeneously enhancing masses (arrows).
(arrow) and adjacent to the left tentorial incisura involving the left
temporal lobe (arrowhead).

228
Atypical teratoid rhabdoid tumor (Cont.) Case 98 (3333)

Axial ADC map shows that these masses have areas of Axial relative cerebral blood volume map from dynamic
decreased di usion corresponding likely corresponding with susceptibility contrast perfusion technique shows increased CBV
hypercellularity (arrowheads). of these masses (arrowheads) favoring a high grade malignancy.

Answers
1. This tumor demonstrates decreased ADC, and increased
Pearls
per usion with areas o decreased T2 signal. These f ndings • Consider atypical teratoid rhabdoid tumor in large,
avor high-grade malignancy such as atypical teratoid heterogeneous CNS masses in children under 3 years
rhabdoid tumor, medulloblastoma, and glioblastoma. o age.
• Tends to be o midline in the posterior ossa
2. Atypical teratoid rhabdoid tumor will usually be located compared to medulloblastoma.
o -midline in the posterior ossa, which may help • Heterogeneous on both T1, T2, and postcontrast.
distinguish it rom medulloblastoma. The mass is usually • Areas o decreased attenuated di usion coe f cient
heterogeneous on T1, T2, and postcontrast with areas o likely due to hypercellularity.
low T2 signal re ecting highly cellular areas, which also • May have increased relative cerebral blood volume
show decreased ADC. On MR spectroscopy, signif cant with dynamic susceptibility contrast per usion imaging.
elevation o choline is typical or a high-grade neoplasm.
3. Atypical teratoid rhabdoid tumor carries a dismal
prognosis (>80% mortality) or patients younger than Suggested Readings
3, partly due to the lack o radiation as an option, which
would devastate immature, developing brains. Children Han L, Qiu Y, Xie C, et al. Atypical teratoid/rhabdoid tumors
older than 3 can have up to 70% survival. in adult patients: CT and MR imaging eatures. AJNR Am
J Neuroradiol. 2011 Jan;32(1):103-108.
4. A particular histological eature o ATRT is the rhabdoid Koral K, Zhang S, Gargan L, et al. Di usion MRI improves
cells, which are similar to malignant rhabdoid tumor o the accuracy o preoperative diagnosis o common
the kidney. pediatric cerebellar tumors among reviewers with
5. 50% o atypical teratoid rhabdoid tumors present di erent experience levels. AJNR Am J Neuroradiol. 2013
in the posterior ossa, which is the most common Dec;34(12):2360-2365.
presentation or this tumor, with 40% supratentorial and Warmuth-Metz M, Bison B, Dannemann-Stern E, Kortmann
the remainder presenting both supra- and in ratentorial. R, Rutkowski S, Pietsch T. CT and MR imaging in
15%-20% can present with dissemination. atypical teratoid/rhabdoid tumors o the central nervous
system. Neuroradiology. 2008 May;50(5):447-452.
229
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60-year-old female presents with a headache after a hypertensive
episode

1. Which part o the brain does this entity most


commonly involve?

2. What are the classic imaging f ndings?

3. What diseases are associated with this entity?

4. What is the presumed pathophysiology o this


disorder?

5. What typically happens to the imaging f nding


a ter treatment?

231
Posterior reversible encephalopathy syndrome Case 99 (2978)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT image at the level of superior aspect of the lateral Axial CT image above the level of the lateral ventricles shows
ventricular bodies demonstrates low attenuation within the continued low attenuation within the cortex and subcortical
white matter of the parietal lobes (arrows). Notice that the low white matter of the parietal lobes (arrows).
attenuation relatively spares the gray matter and has a vasogenic
edema pattern.

Pearls
Answers • PRES (posterior reversible encephalopathy syndrome)
1. PRES (posterior reversible encephalopathy syndrome) is is a transient neurotoxic state most likely secondary to
usually symmetric and may involve any part o the brain. dys unctional autoregulation.
It most commonly involves the parietal and occipital • Bilateral and symmetric distribution.
lobes. • Vasogenic edema pattern.
• Parietal and occipital lobes most commonly a ected.
2. PRES (posterior reversible encephalopathy syndrome)
• Restricted di usion is less common but has been
classically demonstrates bilateral symmetric vasogenic
reported.
edema.
• 25% o patients do not have hypertension.
3. PRES is associated with these conditions: hypertension, • The vasogenic edema usually completely reverses
toxemia o pregnancy (preeclampsia, eclampsia), a ter removal o etiology.
posttransplant (allo-bone marrow transplant, solid
organ transplant), immune suppression (cyclosporine,
tacrolimus), thrombotic microangiopathy (HUS,
DIC, TTP), sepsis/shock (multiorgan dys unction Suggested Readings
syndrome, systemic in ammatory response syndrome), Bartynski WS. Posterior reversible encephalopathy
autoimmune disease (lupus, scleroderma, Wegener), syndrome, part 1: undamental imaging and
high-dose chemotherapy, and other miscellaneous clinical eatures. AJNR Am J Neuroradiol. 2008
conditions. Jun;29(6):1036-1042.
Bartynski WS. Posterior reversible encephalopathy
4. PRES most likely is secondary to a ailure in cerebral
syndrome, part 2: controversies surrounding
autoregulation.
pathophysiology o vasogenic edema. AJNR Am J
5. PRES mani ests as vasogenic edema, which usually Neuroradiol. 2008 Jun;29(6):1043-1049.
resolves, especially in the setting o hypertension.

232
22-year-old male found unresponsive at a party

1. Where is the abnormality located?

2. What other electrolyte or hormonal imbalance


can cause these f ndings?

3. What is the di erential diagnosis or bilateral


basal ganglia cytotoxic edema?

4. What are some toxic causes o this injury?

5. What diseases can cause unilateral basal


ganglia f ndings?

233
Basal ganglia toxicity from drug use Case 100 (3044)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT image shows bilateral basal ganglia low attenuation Axial FLAIR image shows bilateral basal ganglia high signal
(arrows). involving the caudate nuclei, internal capsules, lentiform nuclei,
and thalami (arrows).

Answers
1. The abnormality is located within both basal ganglia. Pearls
• Toxic or metabolic etiologies should be considered
2. The basal ganglia are susceptible to metabolic changes with bilateral basal ganglia cytotoxic edema.
resulting in electrolyte imbalance. These include • The basal ganglia are susceptible to subtle changes in
hyperammonemia, hypoglycemia, hyperglycemia, blood ow (autoregulation) and hypoxemia because
hyponatremia, and others. o their high metabolic rate and blood supply.
3. The di erential or bilateral basal ganglia cytotoxic • In young patients consider illicit drug abuse (heroin,
edema includes toxic etiology ( rom CO poisoning or opiates, ecstasy MDMA).
illicit drug use), osmotic myelinolysis (extrapontine • Consider hypoxic ischemic injury with cortical
myelinolysis), and hypoxic ischemic encephalopathy involvement.
(HIE). Creutz eldt-Jakob disease is an uncommon • Consider osmotic myelinolysis with pontine
in ectious cause o bilateral basal ganglia decreased involvement.
di usion.
4. Toxicity o the basal ganglia can occur with exposure to
Suggested Readings
carbon monoxide, methanol, ethylene glycol, cyanide,
heroin, opiates, and methylenedioxymethamphetamine Beltz EE, Mullins ME. Radiological reasoning:
(MDMA or ecstasy). hyperintensity o the basal ganglia and cortex on FLAIR
and di usion-weighted imaging. AJR Am J Roentgenol.
5. Unilateral basal ganglia f ndings may be seen with 2010 Sep;195(3 suppl):S1-S8 (Quiz S9-S11).
neoplasia, in ection, and MCA in arction. Hegde AN, Mohan S, Lath N, Lim CC. Di erential diagnosis
or bilateral abnormalities o the basal ganglia and
thalamus. Radiographics. 2011;31(1):5-30.
Saenz RC. The disappearing basal ganglia sign. Radiology.
2005 Jan;234(1):242-243.
234
50-year-old female with altered consciousness and ataxia

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What are typical presenting symptoms in this


entity?

5. What is the treatment or this entity?

235
Wernicke encephalopathy Case 101 (3045)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial FLAIR image demonstrates high Axial FLAIR image demonstrates high Axial FLAIR image demonstrates high
signal surrounding the periaqueductal signal involving the hypothalami (arrows). signal involving the border of the third
gray (arrow). ventricle and medial thalami (circle).

Answers
1. The abnormality is located within the periaqueductal Pearls
gray, hypothalamus, and medial thalami. • Wernicke encephalopathy (WE) is a neurologic
disorder related to thiamine def ciency.
2. The classic MRI f ndings o Wernicke encephalopathy
• Patients present with altered consciousness, ocular
(WE) include symmetric high T2 signal along the
dys unction, and ataxia.
borders o the third ventricle and the periaqueductal
• Key to diagnosis is high T2 signal involving the
gray. Also commonly involved are the medial thalamus,
borders o the third ventricle and the periaqueductal
tectal plate, and mamillary bodies.
gray.
3. The di erential diagnosis includes Wernicke • Contrast enhancement o the mamillary bodies is
encephalopathy, artery o Percheron in arct, and osmotic highly suggestive o WE.
myelinolysis. • With pons involvement, consider osmotic
myelinolysis.
4. WE patients present with altered consciousness, ocular
• Artery o Percheron in arct involves the medial
dys unction, and ataxia.
thalami and/or midbrain.
5. This is considered a medical emergency as thiamine
replacement must be given.
Suggested Readings
Hegde AN, Mohan S, Lath N, Lim CC. Di erential diagnosis
or bilateral abnormalities o the basal ganglia and
thalamus. Radiographics. 2011;31(1):5-30.
Zuccoli G, Siddiqui N, Cravo I, Bailey A, Gallucci
M, Harper CG. Neuroimaging f ndings in alcohol-
related encephalopathies. AJR Am J Roentgenol. 2010
Dec;195(6):1378-1384.

236
58-year-old male with progression of dementia over several months,
gait disturbance, and decreased attention span

1. What are the typical MRI f ndings o this


disease?

2. Which sequence is the most sensitive or the


disease?

3. Which o these f ndings can be seen late in the


disease?

4. What is the transmitted orm o the disease?

5. What are typical clinical symptoms o the


disease?
237
Creutzfeldt-Jakob syndrome Case 102 (3181)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 at the ganglionic level Axial FLAIR through the Axial DWI b = 1000 shows Axial ADC shows corresponding
shows increased T2 signal of the ganglionic level shows T2 bright signal of the bilateral decreased signal of the bilateral
bilateral caudate (white arrows) hyperintensity through the caudate, putamina, thalami, caudate, putamina, thalami
and putamina (red arrows). caudate and putamina as well and occipital parietal cortices (asterisks), and occipital cortex.
as more subtle increased signal (red arrows).
of the medial thalami (white
arrows) and occipital parietal
cortices (red arrows).

Answers
1. T2 hyperintensity associated with di usion restriction Pearls
o the basal ganglia, thalami, and cortex are typical MRI • Creutz eldt-Jakob disease is a spongi orm
f ndings in Creutz eldt-Jakob disease. As the disease encephalopathy characterized by rapidly progressive
progresses, cerebral atrophy is invariable. dementia and movement abnormalities
• MRI imaging shows bilateral or unilateral T2
2. Di usion-weighted imaging is the most sensitive or
hyperintensity o the basal ganglia, thalami, and
early and intermediate changes o Creutz eldt-Jakob
cerebral cortex not ollowing a specif c vascular
disease. Restricted di usion may persist beyond the
distribution
usual 10-14 days seen in cytoxic edema rom acute
• DWI show restricted di usion o involved structures
in arction.
early in the disease and may persist greater than the
3. As Creutz eldt-Jakob progresses, there can be expected 10-14 days or acute in arct.
disappearance o DWI hyperintensity and development
o white matter T2 hyperintensity. Cerebral atrophy
always occurs as the disease progresses. Suggested Readings
4. Creutz eldt-Jakob disease (CJD) can be transmitted Degnan AJ, Levy LM. Inherited orms o Creutz eldt-Jakob
through in ected bee (variant CJD) and iatrogenically disease. AJNR Am J Neuroradiol. 2013 Sep;34(9):
rom soiled instruments. 1690-1691.
5. Rapid progression o dementia, myoclonic jerks, and Letourneau-Guillon L, Wada R, Kucharczyk W. Imaging
akinetic mutism are clinical hallmarks o Creutz eldt- o prion diseases. J Magn Reson Imaging. 2012
Jakob disease. May;35(5):998-1012.
Ukisu R, Kushihashi T, Kitanosono T, et al. Serial di usion-
weighted MRI o Creutz eldt-Jakob disease. AJR Am J
Roentgenol. 2005 Feb;184(2):560-566.

238
Abnormal prenatal ultrasound

1. What is the diagnosis?

2. What are the classic imaging f ndings or this


entity?

3. What are the di erent orms o this entity?

4. What are associated midline anomalies?

5. What is the vascular supply to the brain in this


entity?
239
Holoprosencephaly, alobar Case 103 (814)
Case ranking/dif culty: Category: Intra-axial supratentorial

Coronal ultrasound image shows Axial T2 image shows a monoventricle Sagittal T1 image shows monoventricle
monoventricle (green arrow) and fusion (green arrow) and dorsal cyst (blue arrow) (green arrow) and dorsal cyst (blue arrow)
of thalami (blue arrow) across the midline. in alobar holoprosencephaly. Septum in alobar holoprosencephaly. Corpus
Septum pellucidum is absent. pellucidum is absent. callosum is absent.

is milder compared to the most severe alobar orm.


Middle interhemispheric variant or syntelencephaly
shows absence o body o corpus callosum with usion
o posterior rontal or anterior parietal lobes across
the midline. Genu and splenium o corpus callosum
are present. Septum pellucidum is absent. Falx and
interhemispheric f ssure are hypoplastic and ocally
absent at the site o the usion.
4. Midline acial anomalies are seen in holoprosencephaly:
hypoplasia o premaxillary segment, single central
Axial T2 image shows an increase in size of the monoventricle maxillary incisor, hypotelorism, and cyclopia.
(green arrow) and dorsal cyst (blue arrow) seen 10 months later. 5. Anterior and middle cerebral arteries are usually absent
Septum pellucidum is absent (red asterisk).
or there is azygos anterior cerebral artery. The cerebrum
is supplied by multiple small vessels arising directly
rom internal carotid and basilar arteries.
Answers
1. This alobar holoprosencephaly shows crossing o gyrus
across the midline. Failure o midline cleavage o the
prosencephalon leads to holoprosencephaly. Pearls
2. Crossing o gyrus across the midline is the most • Holoprosencephaly is a congenital mal ormation that
important eature in alobar holoprosencephaly. involves a spectrum o anomalies o midline usion.
Monoventricle, dorsal cyst, usion o hypothalami and • The best clue or alobar holoprosencephaly is a
basal ganglia, absent interhemispheric f ssure, absent monoventricle with used rontal lobes.
alx cerebri, absent septum pellucidum, absent corpus • Severe hydrocephalus will show thinned cortical
callosum, and midline acial anomalies are typical mantle.
f ndings. Sylvian f ssure is absent or located more • Midline acial anomalies are associated.
anteriorly and near the midline.
3. Lobar orm o holoprosencephaly shows relatively well-
developed rontal horns, rontal lobes, temporal horns, Suggested Reading
third ventricle, and hippocampi compared to semilobar Vaz SS, Chodirker B, Prasad C, Seabrook JA, Chudley
orm. Lobar orm also shows nearly normal sylvian AE, Prasad AN. Risk actors or nonsyndromic
f ssure; basal ganglia and interhemispheric alx may be holoprosencephaly: a Manitoba case-control study. Am J
present but hypoplastic. However, septum pellucidum Med Genet A. 2012 Apr;158A(4):751-758.
is absent even in lobar orm. Clinically, lobar orm
240
2-month-old male with intermittent episodes of right eye deviation and
jerking movements of right upper and lower extremities for last 3 days

1. Where is the abnormality located?

2. What is the classic clinical triad in this entity?

3. What are other f ndings seen outside the CNS


in this entity?

4. What are classic CNS imaging f ndings?

5. What are the major eatures in this disease?

241
Tuberous sclerosis complex Case 104 (818)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT image shows a hyperdense cortical/ Axial CT image shows a calci ed Axial CT image shows calci ed
subcortical tuber (blue arrow) without subependymal nodule (green arrow). subependymal nodule (arrow) seen in
vasogenic edema. tuberous sclerosis.

Answers
1. The abnormality is located within the le t rontal lobe Pearls
and the le t periventricular white matter. • Tuberous sclerosis (TS) is a neurocutaneous syndrome
2. Typical clinical triad in tuberous sclerosis is acial that has autosomal dominant inheritance (via either
angiof broma, mental retardation, and in antile spasm. gene 9q34 or 16p13).
Most patients are diagnosed by the age o 2 years. • Lymphangioleiomyomatosis and renal
angiomyolipoma are major eatures o TS but either
3. Renal angiomyolipomas require monitoring by one alone do not make a def nitive diagnosis.
ultrasound as they can rapidly grow in size. When • Subependymal nodules calci y a ter f rst year o li e
they reach more than 4 cm in size, there is a higher and are easily seen on CT scan.
incidence o rupture and li e-threatening hemorrhage. • Subependymal nodules in neonatal ultrasound appear
Angiomyolipoma appear echogenic on ultrasound and as an echogenic area and mimic subependymal
hypodense on CT due to at content in the lesion. MRI hemorrhage or subependymal heterotopia.
also demonstrates presence o at within the lesion using • Subependymal nodules in unmyelinated brain appear
at-saturated sequences. hyperintense on T1 and hypointense on T2 whereas in
Lymphangioleiomyomatosis appear as multiple cystic myelinated brain they appear isointense.
lesions in the lungs with very thin walls. They are • FLAIR is sensitive or visualization o cortical tubers.
uni ormly distributed within the lung parenchyma. • Retinal hamartomas, i present, appear a ter the
f rst ew months o li e and are usually multiple and
Ash lea spots are depigmented nevi in the skin. bilateral.
4. The classic imaging f ndings include cortical tubers and
calcif ed subependymal nodules. The cortical tubers have
variable signal on T2-weighted images, depending on Suggested Reading
calcif cation. The subependymal nodules commonly calci y,
and can be easily detected on CT secondary to calcif cation. Roach ES, Gomez MR, Northrup H. Tuberous sclerosis
complex consensus con erence: revised clinical diagnostic
5. Def nitive diagnosis o tuberous sclerosis requires at least criteria. J Child Neurol. 1998 Dec;13(12):624-628.
two major eatures or one major + two minor eatures.
Major eatures include acial angiof bromas or orehead
plaque, ungual or periungual f bromas, more than
three hypomelanotic macules, shagreen patch, retinal
nodular hamartomas, cortical tuber, subependymal
nodule, subependymal giant cell astrocytoma, cardiac
rhabdomyoma, lymphangioleiomyomatosis, and renal
angiomyolipoma.
242
44-year-old female with microcephaly and mental retardation

1. Where is the abnormality located?

2. What are the classic imaging f ndings?

3. What are possible genetic causes?

4. What is the etiology o this entity?

5. What are possible clinical f ndings in patients


with this mal ormation?

243
Lobar-holoprosencephaly Case 105 (3167)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT image shows a monoventricle without septum Axial image shows monoventricle (asterisk) with absence of the
pellucidum (asterisk). Note the genu of the corpus callosum is falx cerebri anteriorly and interdigitation of the interhemispheric
present (arrow). ssure (arrows). A posterior falx is present (arrowhead).

Answers
1. Holoprosencephalies involve lack o separation or Pearls
development o midline structures. Absence o the • In semilobar holoprosencephaly, there is partial
septum pellucidum and monoventricle are typically seen separation o the prosencephalon and there ore partial
within the whole spectrum. ormation o midline structures.
• Mildest spectrum o holoprosencephalies.
2. Absence o the septum pellucidum, monoventricle,
• Absence o the septum pellucidum with
partial agenesis o the corpus callosum, and
monoventricle.
underdevelopment o the rontal lobes are at least
• Partial agenesis o the corpus callosum, splenium
typically seen with lobar holoprosencephaly, the mildest
typically present.
orm.
• At least posterior presence o the alx cerebri and
3. 25%-50% o holoprosencephalies involve cytogenetic interhemispheric f ssure.
abnormalities, including trisomies 13 or 9, or deletions • +/− partial usion o midline structures and presence
involving chromosomes 1, 2, 3, 7, 11, or 18. o dorsal cyst.
• Facial anomalies typically are not as severe as alobar
4. This congenital mal ormation results rom lack
holoprosencephaly.
o separation o the embryonic orebrain or
prosencephalon.
5. Patients with holoprosencephaly spectrum may be
normal, or have seizures, hypothalamic/pituitary Suggested Reading
dys unction, developmental delay, mental retardation, Cayea PD, Balcar I, Alberti O, Jones TB. Prenatal diagnosis
and dystonia and hypotonia rom central nuclei usion. o semilobar holoprosencephaly. AJR Am J Roentgenol.
Phenotypical presentation is variable depending on the 1984 Feb;142(2):401-402.
amount o brain mal ormation. The treatment or this
entity is typically supportive.
244
10-year-old female with headache and dizziness

1. Which part o the brain does this entity most


commonly involve?

2. What are the classic imaging f ndings or this


entity?

3. What mal ormations are associated with this


entity?

4. What is the etiology o this entity?

5. How is this entity treated?


245
Closed-lip schizencephaly (bilateral) Case 106 (3241)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image shows linear clefts lined by Axial T1 image with contrast Sagittal T1 noncontrast shows the gray matter
gray matter isointense tissue extending shows nonenhancing, gray matter lined cleft extending through the right frontal
from the cortex to the lateral ventricles isointense lined clefts extending lobe (arrow).
with nipple-like outpouching (arrows). from the cortical surface to the
There is no CSF within the cleft, consistent lateral ventricles bilaterally (arrows).
with a “closed-lip” type schizencephaly.

Pearls
• Schizencephaly is an abnormal cle t lined by gray
matter extending rom the pia matter o the cerebral
cortex to the ventricular ependyma.
• In open-lip schizencephaly there is a readily apparent
cerebral spinal uid (CSF) tract that is lined by gray
matter.
• Closed-lip schizencephaly demonstrates no visible
Coronal FLAIR image CSF cle t between the approximated gray matter, and
shows the right frontal there is o ten a dimple or de ect where the tract enters
cleft enface (arrow) and the lateral ventricle.
isointense to gray matter. • Secondary to in utero insult, commonly in ection, or
vascular vs genetic abnormality.
• Up to 50% may be bilateral and most commonly
Answers parasylvian in location.
1. Schizencephaly can occur anywhere, but is more common • Patients can present with seizures, developmental
involving the parasylvian rontal or parietal lobes. delay, and motor de ects.
• Best imaged with multiplanar MRI.
2. Classic imaging f ndings are an abnormal cle t lined by
gray matter extending rom the pial sur ace o the cortex
to the ependymal sur ace o the ventricle.
Suggested Readings
3. Associated mal ormations include septo-optic dysplasia
with absence o the septum pellucidum and hypoplasia Oh KY, Kennedy AM, Frias AE, Byrne JL. Fetal
o the optic nerves, gray matter heterotopia, pachygyria, schizencephaly: pre- and postnatal imaging with a review
polymicrogyria, and lissencephaly. o the clinical mani estations. Radiographics. 2010
Oct;25(3):647-657.
4. This entity is thought to be secondary to an in utero insult, Patel AC, Cohen HL, Hotson GC. US case o the day.
commonly in ection, or vascular vs genetic abnormality. Open-lip schizencephaly with an area o heterotopic gray
5. Supportive care is given with control o seizures and matter and associated absence o the septa pellucida.
hydrocephalus i present. Radiographics. 2002 Oct;17(1):236-239.

246
13-day-old term infant with multiple congenital nevi

1. What are the diagnostic criteria or this


disease?

2. What are typical clinical presentation(s)?

3. What is the di erential diagnosis?

4. What are the characteristic radiographic


eatures?

5. Is ollow-up imaging recommended or this


disease?
247
Neurocutaneous melanosis Case 107 (3300)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T1 noncontrast image Axial T1 noncontrast image Axial T1 noncontrast image Axial T2 FLAIR image
demonstrates a focus of T1 demonstrates a wedge- demonstrates two foci of demonstrates two foci of T2
hyperintensity in the left shaped T1 hyperintensity T1 hyperintensity in the FLAIR hyperintensity in the
periventricular white matter in the right caudal thalamic right medial temporal lobe/ right medial temporal lobe/
(white arrow). groove (white arrow). amygdala (white arrows). amygdala (white arrows)

Answers
• Symptoms are related to increased intracranial
1. Neurocutaneous melanosis is a rare noninherited pressure secondary to impaired CSF ow rom
condition, characterized by cutaneous congenital leptomeningeal melanosis.
melanocytic nevi (giant or multiple small lesions) and • Extremely poor prognosis in symptomatic patients.
melanocyte deposition in the central nervous system. • 5%-15% li etime risk o malignant trans ormation o
2. Most patients are asymptomatic. Symptomatic giant cutaneous nevi.
patients present at an early age and are related to • More than hal o patients with neurocutaneous melanosis
melanocyte deposition in the CNS causing increased will have leptomeningeal malignant melanoma.
intracranial pressure secondary to poor CSF circulation. • No def nite treatment.
Symptomatic patients typically have extremely poor • Characteristic radiographic f ndings include T1
prognosis. hyperintense lesions with no contrast enhancement o
parenchymal lesions.
3. Di erential diagnosis o T1 hyperintense lesions • Parenchymal lesions are usually located in the
includes lipoma, dermoid, and hemorrhage. amygdala, cerebellum, brain stem, in erior rontal
4. Characteristic imaging is T1 hyperintensity with lobes, and thalami.
susceptibility arti acts in SWI/GRE sequences secondary • Leptomeningeal melanosis is typically di use and can
to melanocyte deposition. Parenchymal lesions are have enhancement.
stable over time; a ew case reports show regression. • GRE/SWI shows blooming arti acts.
Leptomeningeal melanosis can show enhancement with • 10% associated with Dandy-Walker mal ormation.
progression to melanoma. • Recommend screening MRI o the central nervous
system (brain and spine) and skin examination or
5. Patients with neurocutaneous melanosis need to be malignant degeneration.
ollowed to assess or malignant trans ormation to
melanoma. In one study this occurred in over hal o the
patients. Suggested Readings
Demirci A, Kawamura Y, Sze G, Duncan C. MR o
parenchymal neurocutaneous melanosis. AJNR Am J
Pearls Neuroradiol. 1995 Mar;16(3):603-606.
• Rare genetic disorder, 100+ case reports. Ginat DT, Meyers SP. Intracranial lesions with high signal
• Giant or multiple melanocytic nevi in the skin with intensity on T1-weighted MR images: di erential
parenchymal and/or leptomeningeal melanosis o the diagnosis. Radiographics. 2012;32(2):499-516.
central nervous system. Smith AB, Rushing EJ, Smirniotopoulos JG. Pigmented
• Most patients are asymptomatic. lesions o the central nervous system: radiologic-pathologic
correlation. Radiographics. 2009;29(5):1503-1524.
248
3-month-old boy with history of seizures

1. What is the di erential diagnosis?

2. What are the typical radiographic f ndings o


the a ected hemisphere?

3. What is the typical clinical presentation?

4. What are common syndromic associations with


this f nding?

5. What is the treatment or this disease?

249
Hemimegalencephaly Case 108 (3323)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial noncontrast CT image demonstrates Axial FLAIR image demonstrates Axial FLAIR image demonstrates
di use enlargement of the right cerebral hemihypertrophy of the right hemihypertrophy of the right cerebral
hemisphere with enlarged right lateral cerebral hemisphere associated with hemisphere associated with pachygyria (white
ventricle (white asterisks) and right-sided pachygyria (white arrowheads). arrowheads). Also noted is enlargement of the
calvarium thickening (white arrowheads). right lateral ventricle (white asterisk).

Answers
1. Di erential diagnosis o hemimegalencephaly include Pearls
disorders that enlarge the hemisphere (gliomatosis • Rare congenital cortical mal ormation with
cerebri), or hemiatrophy diseases making the normal hamartomatous overgrowth o cerebral hemisphere o
hemisphere appear large (Rasmussen encephalitis, Dyke- unknown etiology.
Davido -Masson syndrome, Sturge-Weber syndrome). • The a ected hemisphere may have ocal or di use
cortical mal ormation and migrational anomalies.
2. The a ected hemisphere usually demonstrates increased
• Typical clinical presentations are in antile spasm,
size o the lateral ventricle, shallow sulci with enlarged
developmental delay, hemiparesis, and hemianopia.
gyri, thickened calvarium, white matter gliosis,
• May be isolated or have syndromic association (47%).
association with developmental venous anomalies,
• Diagnostic clues are increased parenchymal brain
cortical mal ormation, and gray matter heterotopia, as
volume and ipsilateral ventricle volume, with
well as contralateral displacement o the posterior alx.
associated migrational anomalies.
3. The majority (90%) o patients present with ocal or • Treatment is targeted to control epilepsy.
generalized seizures. Developmental delay, hemiparesis,
and hemianopia are usually also evident.
4. Syndromic associations (47%) with hemimegalencephaly Suggested Readings
include Klippel-Trenaunay syndrome, proteus syndrome, Abdel Razek AA, Kandell AY, Elsorogy LG, Elmongy A,
epidermal nevus syndrome, and hypomelanosis o Ito. Basett AA. Disorders o cortical ormation: MR imaging
eatures. AJNR Am J Neuroradiol. 2009 Jan;30(1):4-11.
5. Hemispherectomy may help control intractable
Broumandi DD, Hayward UM, Benzian JM,
seizures; anticonvulsive therapy is usually ine ective in
Gonzalez I, Nelson MD. Best cases rom the
hemimegalencephaly.
AFIP: hemimegalencephaly. Radiographics. 2004
Aug;24(3):843-848.
Sato N, Yagishita A, Oba H, et al. Hemimegalencephaly:
a study o abnormalities occurring outside the
involved hemisphere. AJNR Am J Neuroradiol. 2007
Apr;28(4):678-682.

250
70-year-old female with headaches and fever

1. Where is the abnormality located?

2. What is the most common presenting


symptom?

3. What is the di erential diagnosis?

4. What etiologies can cause this entity?

5. What is the treatment or this entity?

251
Pyogenic brain abscess Case 109 (3062)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T1 image shows a Axial T2 image shows a Axial DWI and ADC shows the lesion with central high signal on DWI
round lesion with low to round, T2 hyperintense (green arrow) and corresponding low ADC signal (red arrow). This is
intermediate signal lesion lesion with T2 dark rim indicative of “restricted di usion.”
centrally within the right and surrounding T2
frontal lobe (arrow). hyperintensity representing
edema (arrow).
Pearls
• The most common pathogens are Staphylococcus and
Streptococcus.
• Abscess rim has low T2 signal.
• Most abscesses are solitary.
• Rim enhancement may be thicker along the gray
matter inter ace and thinner along the side that abuts
the white matter.
• Multiple rim enhancing lesions, think metastasis.
• GBM typically do not have smooth rim enhancement.
• Toxoplasma abscess does not typically restrict
di usion.

Suggested Readings
Axial postcontrast T1 shows ring enhancement (arrow).
Chong-Han CH, Cortez SC, Tung GA. Di usion-weighted
MRI o cerebral toxoplasma abscess. AJR Am J
Answers Roentgenol. 2003 Dec;181(6):1711-1714.
Smirniotopoulos JG, Murphy FM, Rushing EJ, Rees JH,
1. The abnormality is located within the posterior rontal
Schroeder JW. Patterns o contrast enhancement in the
lobe and involves the precentral gyrus.
brain and meninges. Radiographics. 2007;27(2):525-551.
2. The most common symptoms include headache (>90%)
and ever (50%).
3. The di erential diagnosis includes metastasis, abscess,
and GBM.
4. This entity may be secondary to trauma,
immunocompromised status, sinus/ear in ection, postop,
and septicemia.
5. The treatment is antibiotic therapy. When lesions are
large (>3 cm), surgical drainage may be needed.

252
28-year-old female with lower extremity weakness and numbness

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the most sensitive MR sequence or


detecting lesions o all ages in this entity?

4. What anatomical structures are a ected by


lesions in this entity?

5. What is the treatment or this entity?


253
Multiple sclerosis Case 110 (3138)
Case ranking/dif culty: Category: Intra-axial supratentorial

Sagittal FLAIR image shows multiple high Axial FLAIR image shows multiple high Sagittal T1 postcontrast shows
signal periventricular lesions (arrows), signal periventricular lesions are present and enhancement of the periventricular
some of which approach the callosal- a couple within the anterior and posterior lesions (arrows), indicating active
septal margin. corpus callosum (arrows). The periventricular demyelination.
lesions are oriented perpendicular to the
ventricles, termed “Dawson ngers.”

Pearls
• High-f eld (3T) MRI is more sensitive or detecting
demyelinating plaques.
• Large MS lesions may mimic tumor; key is “incomplete”
ring enhancement (horseshoe appearance).
• Utilize at saturation sequences or optic neuritis.
• Approximately hal o optic neuritis patients develop MS.
• 80%-90% o MS patients have CSF oligoclonal bands.
• In young patients with ebrile prodrome consider
Axial DWI and ADC show one of the periventricular lesions on the Marburg variant, which presents with an acute,
right demonstrates restricted di usion (green arrow). Note the ulminant demyelinating mass.
low signal on the ADC image (white arrow). This indicates early • With only optic nerve and spinal cord involvement
acute demyelination. consider neuromyelitis optica (Devic disease).
• Corpus callosum and deep gray nuclei involvement
consider Susac syndrome.
Answers
1. The abnormality is located within the white matter.
2. On MRI, there is variable signal, usually hypointense Suggested Readings
on T1 and hyperintense on T2 within the white matter.
Eisele P, Szabo K, Griebe M, et al. Reduced di usion in a
Characteristic lesions at the level o the lateral ventricles
subset o acute MS lesions: a serial multiparametric MRI
orientated perpendicular to the long axis are termed “Dawson
study. AJNR Am J Neuroradiol. 2012 Aug;33(7):1369-1373.
f ngers” and are a classic f nding or multiple sclerosis.
Filippi M, Rocca MA. MR imaging o multiple sclerosis.
3. FLAIR is the most sensitive standard MR sequence or Radiology. 2011 Jun;259(3):659-681.
detecting demyelinating lesions o all ages. Given CA, Stevens BS, Lee C. The MRI appearance o
tume active demyelinating lesions. AJR Am J Roentgenol.
4. Multiple sclerosis is a demyelinating disease that may
2004 Jan;182(1):195-199.
involve the brain (predominantly white matter), spinal
cord, and cranial nerves.
5. The treatment or MS includes corticosteroids and other
immunosuppressants.
254
7-year-old male with headache, seizure, fever

1. What is the prognosis or this disease?

2. What can be seen in specif c MRI sequences o


this disease?

3. What is the di erential diagnosis o this


f nding?

4. What MRI f ndings can be seen with viral


encephalitides?

5. What is the most common pathogen o


sporadic viral encephalitis?
255
Viral encephalitis (La Crosse) Case 111 (3188)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT image at the level of Axial CT image at the level Axial T2 image at the Axial T2 image at the skull
the midbrain shows di use of the basal ganglia shows supraganglionic level shows base shows cerebellar
e acement of the basilar di use cerebral swelling di use cerebral gray matter tonsillar herniation from
cisterns and cerebral swelling. with e acement of sulci and swelling from lack of sulcal di use cerebral edema
There is “pseudosubarachnoid” ventricles in this patient with visualization and focal areas (white arrows).
sign (red arrows). La Crosse viral encephalitis. of right frontal cortical edema
Focal hypodensity from more (white arrow) in this patient with
severe edema is seen in the La Crosse viral encephalitis.
right frontal lobe (red arrow).

Answers
1. Although dependent on the specif c pathogen, as a Pearls
whole, viral encephalitides can have high morbidity and • Viral encephalitides have high morbidity and
mortality, especially without treatment. mortality especially in certain age groups, depending
on the specif c pathogen.
2. Viral encephalitides will typically show T2
• Herpes is the most common cause o sporadic viral
hyperintensity rom in ammatory swelling with some
encephalitis.
di usion restriction. Contrast enhancement can be seen
• MRI usually shows a pre erence or gray matter
but is not obligatory or the diagnosis. Hemorrhage is
involvement, although white matter can be commonly
uncommon in the majority o viral encephalitides.
involved with specif c pathogens.
3. With di use brain swelling, ischemia, traumatic brain • Di use in ammation, poorly delineated T2
injury, and viral encephalitides should be considered. hyperintensity, and areas o di usion restriction
Status epilepticus can also show di use swelling. The +/− enhancement are typical.
clinical history is help ul to distinguish these entities. • The cerebellum can be a ected in isolation or
conjunction.
4. Although with a predilection or gray matter (including
• Rapid diagnosis with treatment using available
basal ganglia) di use in ammation, white matter lesions
antivirals can signif cantly improve outcome.
and brainstem involvement can be characteristic o some
specif c pathogens. Ring-enhancing lesions are not usual
or viral encephalitis.
5. The herpes virus, specif cally the herpes simplex 1 virus, Suggested Readings
is the most common sporadic cause o viral encephalitis Gupta RK, Soni N, Kumar S, Khandelwal N. Imaging o
with 70% mortality i not treated. central nervous system viral diseases. J Magn Reson
Imaging. 2012 Mar;35(3):477-491.
Kirolğu Y, Calli C, Yunten N, et al. Di usion-weighted MR
imaging o viral encephalitis. Neuroradiology. 2006
Dec;48(12):875-880.
Parmar H, Ibrahim M. Pediatric intracranial in ections.
Neuroimaging Clin N Am. 2012 Nov;22(4):707-725.

256
65-year-old female with progressive memory decline, confusion,
and agitation over several weeks

1. What is the di erential or these MRI f ndings?

2. What is the usual anatomy a ected in this


disease?

3. What is the pathophysiology o the disease?

4. What is the treatment or this disease?

5. What are other related syndromes with similar


pathophysiology as this disease?

257
Paraneoplastic limbic encephalitis Case 112 (3240)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial FLAIR image shows bilateral swelling and T2 hyperintensity Axial FLAIR image shows involvement of the bilateral insular
of the medial temporal lobes (white arrow). cortex (white arrows) and tails of the hippocampi (arrowheads).
This patient was eventually diagnosed with small cell lung cancer.

Answers
1. The di erential or bilateral swelling o the medial
Pearls
temporal lobes includes limbic encephalitis, herpes
encephalitis, and status epilepticus. Clinical history • Limbic encephalitis is the most common
should be help ul to distinguish between these entities. I paraneoplastic syndrome involving the CNS.
herpes encephalitis cannot be excluded, rapid treatment • Small cell lung cancer is classically implicated in
with acyclovir must be considered. limbic encephalitis, although other neoplasms can
cause it as well.
2. Limbic encephalitis a ects the medial temporal lobes, • Clinical presentation usually involves subacute
insular cortex, and in erior rontal lobes. presentation with progressive cognitive decline over
3. Paraneoplastic syndromes are thought to be immune weeks to months.
mediated with autoantibodies or cytotoxic T-cell activity. • MRI f ndings o bilateral medial temporal lobe,
Anti-Hu autoantibodies are ound in the majority o insular cortex, and in erior rontal lobe involvement
cases o limbic encephalitis, which is associated with are typical with T2 hyperintensity and swelling.
small cell lung cancer. • Patchy enhancement and restricted di usion have
been described but are variable.
4. Limbic encephalitis is a paraneoplastic syndrome
caused by autoimmune actors. Treatment with
immunomodulators and suppressive agents such as
corticosteroids, as well as plasmapheresis has shown Suggested Readings
some benef ts; however, no clinical trial has been Demaerel P, Van Dessel W, Van Paesschen W, Vandenberghe
per ormed. Symptoms o paraneoplastic syndromes R, Van Laere K, Linn J. Autoimmune-mediated
generally improve as the primary neoplasm is treated. encephalitis. Neuroradiology. 2011 Nov;53(11):837-851.
5. Both Lambert-Eaton and opsoclonus-myoclonus are Urbach H, Soeder BM, Jeub M, Klockgether T, Meyer
paraneoplastic syndromes. B, Bien CG. Serial MRI o limbic encephalitis.
Neuroradiology. 2006 Jun;48(6):380-386.

258
27-year-old immunocompromised male with headaches, left-sided
weakness and fever

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the typical clinical presentation?

5. What is the treatment or this entity?

259
Toxoplasmosis Case 113 (3311)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T1 postcontrast demonstrates an irregular peripherally


enhancing lesion within the left periventricular basal ganglia
Axial T2 image shows extensive vasogenic edema centered in the and white matter extending to the subependymal surface and
left basal ganglia, and extending to the hemispheric white matter into the subcortical white matter of the left frontal lobe (arrows).
(arrows). The vasogenic edema results in subfalcine herniation A focus of leptomeningeal enhancement is seen posteriorly
from left to right. Notice the compression of the left lateral involving the right occipital lobe (arrowhead).
ventricle.

Answers Pearls
1. The abnormality is located in the le t periventricular • Toxoplasmosis is secondary to Toxoplasmosis gondii,
region involving the superior aspect o the le t basal which is an intracellular protozoan.
ganglia. Toxoplasmosis most commonly involves the • It may be transmitted to humans via undercooked
basal ganglia. pork, contaminated vegetables, or cat eces.
• Toxoplasmosis is the most likely in ection with ring-
2. On CT imaging, the lesions are usually low density. On enhancing lesions involving the basal ganglia in AIDS
MRI, lesions are low T1 signal with corresponding high patients.
or mixed T2 signal. A ter administration o contrast, the • “Target” sign with central nodular enhancement
lesions will show rim or ring enhancement. within the ring enhancement is suggestive o toxo.
3. The di erential diagnosis includes toxoplasma • A solitary mass is more likely to be lymphoma in
encephalitis (cerebral toxoplasmosis), lymphoma, and HIV/AIDS.
glioma. • Consider PML or HIV encephalopathy in
nonenhancing lesions o the subcortical white matter
4. Toxoplasmosis is secondary to Toxoplasmosis gondii, in AIDS patients.
which is an intracellular protozoan. The most common • Immunocompromised patients with basilar meningeal
symptom is headache. Other common symptoms enhancement consider tuberculosis.
include ever, altered mental status, seizures, sensory • With thallium, lymphoma is “hot,” but toxo is not.
disturbances, motor weakness, and speech disturbance.
5. The treatment or this entity is medical treatment with
pyrimethamine and sul adiazine. The duration o therapy Suggested Readings
is 6 weeks and pyrimethamine requires olinic acid to Hegde AN, Mohan S, Lath N, Lim CC. Di erential diagnosis
prevent toxicity. or bilateral abnormalities o the basal ganglia and
thalamus. Radiographics. 2011;31(1):5-30.
Lee GT, Antelo F, Mlikotic AA. Best cases rom the
AFIP: cerebral toxoplasmosis. Radiographics.
2009;29(4):1200-1205.
260
65-year-old female treated for follicular lymphoma with progressive
memory di culty and behavioral changes over the past 2 weeks

1. What is the di erential diagnosis?

2. What are typical imaging f ndings or this


entity?

3. What is the etiology o this disease?

4. What are associated risk actors or this entity?

5. What is the treatment or this disease?


261
Progressive multifocal leukoencephalopathy Case 114 (3507)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial FLAIR image shows patchy Axial FLAIR image at the Axial T1 postcontrast image Axial DWI b = 1000 image
white matter lesions primarily in corona radiata shows the shows no enhancement of the shows di usion restriction
the left parietal lobe extending patchy white matter lesions white matter lesions (arrow). of the white matter lesions
toward the splenium of the to be bilateral (arrows) but (arrows).
corpus callosum, which is asymmetric, predominantly
con uent and appears mass like on the left. A lesion is
(arrows). seen involving the left
parietal subcortical U- ber Pearls
(arrowhead).
• Opportunistic in ection by the JC papovavirus o
oligodendrocytes with widespread demyelination.
Answers • Greatest risk actor is immunosuppression.
1. In an immunosuppressed patient, the di erential • Variable-size T2 hyperintense and T1 hypointense
includes progressive multi ocal leukoencephalopathy white matter lesions, which may become con uent
(PML), HIV encephalitis, and immune reconstitution and mass like.
in ammatory syndrome (IRIS). In immunocompetent • Subcortical U-f bers are typically involved.
patients, ADEM or MS should be considered. • Lesions may be bilateral but asymmetric.
• Acute lesions can demonstrate di usion restriction.
2. Typical MRI imaging f ndings or PML include patchy, • Enhancement is not typical.
bilateral asymmetric white matter lesions, which involve
the subcortical U-f bers. Lesions may become con uent
and mass like. Enhancement is atypical. DWI shows
di usion restriction in acute lesions. Suggested Readings
Buckle C, Castillo M. Use o di usion-weighted imaging
3. PML is an opportunistic in ection o the JC papovavirus
to evaluate the initial response o progressive multi ocal
involving the oligodendrocytes with resulting demyelination.
leukoencephalopathy to highly active antiretroviral
4. Immunosuppression is the greatest risk actor or therapy: early experience. AJNR Am J Neuroradiol. 2010
PML, primarily including AIDS, transplantation, and Jun;31(6):1031-1035.
chemotherapy. PML was initially described associated Post MJ, Thurnher MM, Cli ord DB, Nath A, Gonzalez
with hematologic and lymphoid malignancies. RG, Gupta RK, Post KK. CNS-immune reconstitution
Rheumatologic diseases are also associated. in ammatory syndrome in the setting o HIV in ection,
part 1: overview and discussion o progressive
5. No specif c treatment or PML currently exists. HAART
multi ocal leukoencephalopathy-immune reconstitution
in AIDS patients prolongs survival but increases the risk
in ammatory syndrome and cryptococcal-immune
or IRIS.
reconstitution in ammatory syndrome. AJNR Am J
Neuroradiol. 2013 Jul;34(7):1297-1307.

262
37-year-old male with history of HIV and new-onset vertigo and ataxia

1. What is the di erential diagnosis?

2. What CNS structures are most commonly


involved?

3. What anatomic structure is the primary method


o spread?

4. Prior to HAART therapy, what percentage o


AIDS patients had this disease?

5. What is the treatment or this disease?


263
Cryptococcosis Case 115 (3511)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial FLAIR image shows increased Axial T2 image shows abnormal signal of the Axial T1 postcontrast shows no
asymmetric signal of the basal ganglia caudate and putamen (arrows). There are also enhancement of the basal ganglia lesions
(arrows). enlarged perivascular spaces (arrowheads) (arrows).
not completely suppressed on FLAIR
(not shown).

5. Anti ungals such as amphotericin B are used as the


mainline o treatment. Maintenance therapy with
uconazole is indicated in AIDS patients.

Pearls
• Cryptococcus in ection is the third most common
CNS in ection in AIDS patients.
• Cryptococci typically involves the lungs, which spread
hematogenously to the CNS with deposition in the
leptomeningeal and perivascular spaces.
• Perivascular spaces are f lled with ungi and mucinous
Axial DWI ADC image shows facilitated di usion of the lesions
(arrows).
material orming gelatinous pseudocysts and
cryptococcomas.
• Deep gray nuclei, brainstem, cerebellum, and white
Answers matter can be involved.
1. Di erential or bilateral asymmetric basal ganglia • MRI imaging shows dilated perivascular spaces
lesions without di usion restriction includes with T2 hyperintense lesions with variable FLAIR
cryptococcosis, viral encephalitis, and CNS lymphoma. suppression, and typically nonenhancement with
weakened immune status.
2. CNS cryptococcosis typically involves deep structures
such as the deep gray nuclei, white matter, and
brainstem.
Suggested Readings
3. CNS cryptococcosis typically spreads through the
Khandelwal N, Gupta V, Singh P. Central nervous system
perivascular spaces, reaching deep brain structures in
ungal in ections in tropics. Neuroimaging Clin N Am.
addition to causing meningitis.
2011 Nov;21(4):859-866, viii.
4. 10% o patients with AIDS had cryptococcosis prior to Sanossian N, Shatzmiller RA, Djabiras C, Liebeskind
the onset o HAART therapy. Cryptococcus is the third DS. FLAIR vascular hyperintensity preceding stroke
most common CNS in ection in AIDS patients a ter HIV in cryptococcal meningitis. J Neuroimaging. 2013
and toxoplasmosis. Jan;23(1):126-128.
264
9-year-old male with disorientation, developmental regression,
and altered mental status

1. What are the typical imaging characteristics o


this entity?

2. What is the symptomatic pathophysiological


process o this disease?

3. What is typically seen on MRI spectroscopy


within the a ected white matter?

4. What is the genetic inheritance and age- and


gender-based phenotypes or this disease?

5. What is the MR scoring system o this disease?

265
X-linked adrenoleukodystrophy Case 116 (3066)
Case ranking/dif culty: Category: Intra-axial supratentorial

Symmetric T2 prolongation Peripheral areas of Postcontrast enhancement MRI spectroscopy of the involved
involving the splenium (blue arrow), di usion restriction (white arrows) of the white matter demonstrates decrease
periatrial (white arrows), and (black arrows) are intermediate zone of in NAA (white arrow), elevation of
capsular white matter (red arrows) indicative of acute demyelination is typical of choline (red arrow) and myo-inositol
is seen in this classic pattern of demyelination. progressive X-ALD. (green arrow) with signi cant
x-linked adrenoleukodystrophy. presence of lactate (blue arrow) and
lipid breakdown products and very
long chain fatty acid molecules in
the 0.9 to 2.4 ppm range (asterisk).

Answers 5. The Loes MRI scoring system correlates with disease


1. Posterior symmetric, con uent involvement o the progression:
parietal, occipital, and temporal white matter is classic • Pattern 1 is the typical posterior white matter
with subcortical U f bers usually spared early. The involvement associated with late childhood onset.
splenium is typically involved. The “leading-edge” • Pattern 2 has a rontal white matter predominance with
enhancement, which correlates with the intermediate similar progression as pattern 1.
zone o in ammation, is an imaging sign correlating • Pattern 3 involves the corticospinal tracts usually seen
with disease progression. in adults with slower progression.
• Pattern 4 includes the cerebellar white matter in
2. X-ALD is an inherited peroxisomal metabolism disorder addition to the corticospinal tract and is seen in the
where there is ailure o oxidation o very long chain adolescent-onset orm.
atty acids. While metabolites are deposited in all tissues, • Pattern 5 has ulminant rontal and posterior
including the liver, only the CNS myelin, adrenal cortex, involvement with rapid progression.
and Leydig cell o the testes lead to symptomatology.
3. While the MRI spectroscopic prof le is nonspecif c,
elevation o lactate, choline, and myo-inositol is a Pearls
typical f nding. NAA is decreased within the abnormal
• Posterior, symmetric, and central involvement o the
appearing white matter. Decrease in NAA in normal-
splenium, periatrial, and capsular white matter is typical.
appearing white matter may re ect progression. Lipids
• Intermediate zone o enhancement and peripheral zone
are typically elevated, which re ects the deposition o
o di usion restriction is typical as the demyelination
very long chain atty acids.
progresses.
4. Classic adrenoleukodystrophy is x-linked, with
the expected male predominance. Up to 50% o
carrier emales will have adult-onset symptoms,
usually adrenomyeloneuropathy-like symptoms with Suggested Readings
predominantly spinal and peripheral nerve involvement, Kim JH, Kim HJ. Childhood X-linked adrenoleukodystrophy:
as well as adrenal ailure. Variant adult-onset orms are clinical-pathologic overview and MR imaging
less common, although with increasing recognition, mani estations at initial evaluation and ollow-up.
the percentage will likely increase. Adult-onset orms Radiographics. 2005 Jul;25(3):619-631.
typically present with adrenomyeloneuropathy. Vijay K, Ouyang T. Anterior pattern disease in
adrenoleukodystrophy. Pediatr Radiol. 2010
Dec;40(suppl 1):S157.
266
6-year-old female with progressive hypotonia

1. What are the typical MRI f ndings o this


disease?

2. What is the pattern o white matter


involvement as the disease progresses?

3. What is the pathophysiology o this disease?

4. What are the risk actors or this disease?

5. What is the usual clinical presentation o the


common orm o this disease?
267
Metachromatic leukodystrophy Case 117 (3105)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 FLAIR shows di use T2 axial image above the Postcontrast imaging shows There is decreased
white matter demyelination ventricles demonstrates no enhancement, due to the di usion seen in the areas
with T2 hyperintensity in a con uent symmetric involvement lack of in ammation seen of active demyelination
symmetric, con uent distribution of the centrum semiovale. There with this disease. Note the (white arrows).
in a “butter y” pattern with both is sparing of the subcortical U sparing of the subcortical
anterior and posterior involvement bers (red arrows) as well as the U- bers, seen early in the
(white arrows). Demyelination of perivenular myelin (blue arrows), disease (white arrows).
the corpus callosum is seen later in giving the appearance of a
the disease (red arrows). “tigroid”pattern.

Answers 5. The late in antile orm is the most common presentation


1. Metachromatic leukodystrophy (MLD) classically o MLD. Insidious onset o symptoms begins around
is con uent, symmetric, and involves both anterior 2 years o age with gait disturbance, weakness,
and posterior portions o the cerebral hemispheres hypotonia, and ataxia as common presenting signs.
in a “butter y” pattern. Decreased di usion can be Abdominal pain can be seen with sul atide deposition in
seen in active demyelination but a lack o histological the gallbladder.
in ammation corresponds with a lack o contrast
enhancement. Sparing o the perivenular myelin can Pearls
lead to “leopard” spots and “tigroid” stripes within the
a ected white matter. • Bilateral symmetric and con uent “butter y” pattern
o demyelination o the hemispheric cerebral white
2. Metachromatic leukodystrophy typically spares the matter.
subcortical U-f bers, internal capsule, and deep callosal • Lack o white matter enhancement due to lack o
f bers early in the disease. As the disease progresses, in ammation. Cranial nerves have been described to
these white matter structures are usually involved. demonstrate enhancement.
Corticospinal tract involvement in the brainstem can • Subcortical and capsular white matter typically spared
be seen in the late phase o the disease. Histological early in the disease.
demyelination o the Schwann cells o the peripheral • “Tigroid” appearance o the spared perivenular myelin.
nervous system is a typical f nding o all subtypes o
metachromatic leukodystrophy.
Suggested Readings
3. MLD is a lysosomal storage disorder with de ect
o arylsul atase-A, located at 22q13, leading to Martin A, Sevin C, Lazarus C, Bellesme C, Aubourg P,
the accumulation o sul atides. These deposit Adamsbaum C. Toward a better understanding o brain
symptomatically in the CNS, PNS, and gallbladder, but lesions during metachromatic leukodystrophy evolution.
can also be seen asymptomatically in other abdominal AJNR Am J Neuroradiol. 2012 Oct;33(9):1731-1739.
organs. There are three main clinical orms o MLD: late Sener RN. Metachromatic leukodystrophy: di usion
in antile, juvenile, and adult onset, with the late in antile MR imaging f ndings. AJNR Am J Neuroradiol. 2002
being the most common o the three. Sep;23(8):1424-1426.
van der Voorn JP, Pouwels PJ, Kamphorst W, et al.
4. Risk actors or MLD include Navajo Indian ancestry, Histopathologic correlates o radial stripes on MR images
Habbanite Jewish ancestry, or a relative with the disease. in lysosomal storage disorders. AJNR Am J Neuroradiol.
2005 Mar;26(3):442-446.
268
4-month-old male with new-onset seizure and enlarging head size

1. What are the typical MRI f ndings o this


disease?

2. What is the method o inheritance o this


disease?

3. What is the pathophysiology o this disease?

4. What are clinical symptoms and prognosis o


this disease?

5. What diseases can show elevation o NAA on


MR spectroscopy?

269
Canavan disease Case 118 (3147)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial DWI b = 1000 images Axial ADC map con rms Axial T2 through the ganglionic Corresponding axial DWI
show bright di usion signal restricted di usion of the level on follow-up imaging in b = 1000 through the
in the subcortical U- bers of subcortical white matter 3 months of the same patient ganglionic level shows
the frontal and occipital lobes (white arrows). shows increased T2 signal of the restricted di usion of the
(white arrows). bilateral caudate (white arrows) corpus striatum (white arrows).
and putamen (red arrows).
Answers
1. Canavan disease MRI f ndings have typically been
described to involve the subcortical U-f bers early Pearls
with progression centrally, but sparing the central • Typical descriptions o Canavan disease include
white matter tracts o the corpus callosum and internal early involvement o the subcortical U-f bers with
capsules early. Central gray structures such as the progression, sparing the central white matter tracts
thalami and globi pallidi can be involved. Elevation o such as the internal capsule and corpus callosum.
NAA is a hallmark o this disease. • Central gray matter involvement usually involves
2. Canavan disease is autosomal recessive with de ect o the thalami and globi pallidi, although caudate and
the enzyme aspartoacylase, and predominantly seen in putamen have been reported.
Ashkenazi Jewish populations. • MR spectroscopy shows the typical relative elevation o
N-acetylaspartate (NAA), which is relatively specif c.
3. Canavan disease is associated with mutations o the
ASPA gene on chromosome 17. The ASPA gene encodes
aspartylaminase enzyme, which breaks down N-acetyl
aspartic acid. In Canavan disease, NAA deposition leads Suggested Readings
to toxicity o the CNS and can be detected in the urine. Brismar J, Brismar G, Gascon G, Ozand P. Canavan
disease: CT and MR imaging o the brain. AJNR Am J
4. Patients with Canavan disease typically present within
Neuroradiol. 1997 Jan;11(4):805-810.
the f rst year o li e with macrocephaly, hypotonia,
Engelbrecht V, Scherer A, Rassek M, Witsack HJ, Mödder U.
seizures, spasticity, and other signs o neurologic
Di usion-weighted MR imaging in the brain in children:
deterioration, leading to death in the f rst decade. A
f ndings in the normal brain and in the brain with white
clinical subtype with a di erent mutation o the ASPA
matter diseases. Radiology. 2002 Feb;222(2):410-418.
gene may have delayed onset, slower progression, and
Hane eld FA, Brockmann K, Pouwels PJ, Wilken B,
longer survival. No e ective treatment currently exists,
Frahm J, Dechent P. Quantitative proton MRS o
although ongoing trials with gene therapy and acetate
Pelizaeus-Merzbacher disease: evidence o dys- and
supplementation may hold promise.
hypomyelination. Neurology. 2005 Sep;65(5):701-706.
5. In addition to Canavan disease, Pelizaeus Merzbacher,
a primary hypomyelination syndrome, can show mild
increase in NAA with absolute measurements. Salla
disease leads to a buildup o N-acetylneuraminic acid
( ree sialic acid), which has similar resonance with NAA.
In addition, Salla disease may show increased creatine,
which has not been reported in Canavan disease.
270
6-month old with history of prematurity, necrotizing enterocolitis,
developmental delay, and spasticity. Had minor head trauma with
abnormal imaging ndings

1. What are MRI f ndings that have been proposed


as obligatory criteria or this diagnosis?

2. What is the genetic basis o the disease?

3. What other organ systems have been described


to present with pathology with this disease?

4. What clinical actors cause episodes o


deterioration?

5. Which group has a higher risk o this disease?

271
Vanishing white matter disease Case 119 (3154)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT for head trauma Axial T2 image shows di use Axial T2 image of the pons Coronal ultrasound at birth shows a
shows severe hypodensity of abnormal white matter shows relative sparing of normal volume of white matter.
the frontal white matter (white involving both central and the temporal lobes (white
arrows), which has the same peripheral subcortical bers arrows). There is prominence
density as CSF in the dilated with high T2 signal intensity of the fourth ventricles
lateral ventricles. following CSF. There are from pontine and cerebellar
hypointense radial bands of atrophy. The bilateral fth
tissue traveling through the cranial nerves appear
a ected white matter (black prominent (red arrows).
arrows). The frontal gyri are
lissencephalic (red arrows) but
with normal thickness and
intensity.

Answers
1. Di use involvement o the cerebral white matter with Pearls
progression to CSF signal is a hallmark o this disease. • Rare action o white matter ollowing CSF signal
FLAIR suppression is key to imaging diagnosis. White with suppression on FLAIR is characteristic.
matter uid-f lled space without complete collapse • Disease progresses rom central to peripheral without
and without contrast enhancement is typical, with the sparing o subcortical U-f bers.
temporal lobes relatively spared. Cerebellar white • Relative sparing o the temporal lobes.
matter can be involved, demonstrating atrophy and T2 • No rare action o cerebellum or brainstem;
hyperintensity without rare action to CSF signal. however, these structures may show atrophy and T2
prolongation.
2. Vanishing white matter disease is autosomal recessive
with mutations involving the EIF2B protein complex.
This complex is involved in regulating RNA translation
to prevent protein mis olding in times o cellular stress. Suggested Readings
3. In adult-onset orms o vanishing white matter, Senol U, Haspolat S, Karaali K, Lüleci E. MR imaging
premature ovarian ailure has been described in a ew o vanishing white matter. AJR Am J Roentgenol. 2000
cases. Sep;175(3):826-828.
van der Knaap MS, Pronk JC, Scheper GC. Vanishing white
4. Episodes o deterioration in vanishing white matter matter disease. Lancet Neurol. 2006;5:413.
disease are precipitated by a recent history o stressors. Vermeulen G, Seidl R, Mercimek-Mahmutoglu S, et al.
5. Cree leukoencephalopathy is a subgroup o vanishing Fright is a provoking actor in vanishing white matter
white matter disease, which presents in the in ant with disease. Ann Neurol. 2005;57:560.
rapid decline. This group o people lives predominantly
in the northern Quebec and Manitoba regions. Carrier
rates in this population are higher than general at 1 in 10
in a study o 50 una ected Cree adults.

272
58-year-old male with chorea and personality changes

1. What are the described MRI f ndings or this


disease?

2. What is the cause o decreased T2 signal o the


striatum in this disease?

3. What are the classic clinical symptoms o this


disease?

4. What is the genetic inheritance o this disease?

5. What are the organ systems a ected in this


disease?

273
Huntington disease Case 120 (3175)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image through the ganglionic Coronal T1 with contrast demonstrates Axial DWI b = 0 images demonstrate
level shows caudate atrophy with dilatation of the frontal horns from caudate T2 hypointensity (T2*) from iron
increased T2 signal (white arrows), as well atrophy (white arrows). deposition in the bilateral putamen
as di use cerebral atrophy. and globi pallidi (white arrows).

Answers
1. Caudate atrophy, with increased T2 signal rom gliosis,
Pearls
is the hallmark o Huntington disease. Di use cerebral • Huntington disease is an autosomal dominant
atrophy and decreased T2 signal o the striatum are inherited disease characterized by chorei orm
typical associated f ndings. movements.
• MRI demonstrates caudate atrophy with increased T2
2. Decreased T2 signal in Huntington disease within the signal rom gliosis.
corpus striatum is due to iron deposition. Iron deposition • Di use cerebral atrophy is also seen with decreased
in the globus pallidus is a normal unction o aging, so T2 signal o the striatum rom iron deposition.
care should be made not to overcall iron deposition in • No treatment exists with most patients dying within
this structure. two decades rom disease onset.
3. Huntington disease typically presents with variable
severity o the triad o chorea, dementia, and psychosis
(personality changes).
Suggested Readings
4. Huntington disease is inherited in an autosomal Hobbs NZ, Barnes J, Frost C, et al. Onset and progression o
dominant ashion with trinucleotide repeat mutations o pathologic atrophy in Huntington disease: a longitudinal
the huntingtin protein gene on chromosome 4p16.3. The MR imaging study. AJNR Am J Neuroradiol. 2010
greater the number o trinucleotide repeats, the worse the Jun;31(6):1036-1041.
disease. There is complete penetrance o the mutation. Mahalingam S, Levy LM. Genetics o Huntington disease.
5. In addition to the brain, abnormal Huntingtin protein AJNR Am J Neuroradiol. 2014;35(6):1070-1072.
causes pathology in the gonads, heart, lungs, and liver.

274
54-year-old male with progressive balance problems, falls,
and speech di culty

1. What are the MRI f ndings in this disease?

2. What causes the “ ace o the giant panda” sign?

3. What is the genetic basis o this disease?

4. What is the pathophysiology o this disease?

5. What are treatment options or this disease?


275
Wilson disease Case 121 (3179)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial FLAIR shows Axial b = 0 DWI image Axial FLAIR through the Axial b = 0 DWI zoomed in image
suppression of the central shows the decreased signal midbrain demonstrates focal of the midbrain demonstrates the
putamen lesions with from copper deposition in T2 hyperintensity of the face of the giant panda sign with
surrounding hyperintensity. the bilateral caudate and midbrain (white arrows). tegmental T2 hyperintensity, T2
This is consistent with late putamen (white arrows). hypointensity of the red nuclei (red
cystic degeneration. There arrows), and cerebral peduncles
is bilateral hyperintensity (black arrows). This is characteristic in
of the globi pallidi and Wilson disease.
left internal capsule
(white arrows).

Answers
1. Bilateral symmetric T2 hyper- and hypointensities o Pearls
the basal ganglia and thalami are hallmarks o Wilson • Wilson disease is an autosomal recessive disease with
disease rom copper deposition. T2 hyperintensity o the impairment o copper transport, leading to copper
midbrain causing a “ ace o the giant panda” sign is also deposition in the liver and other organs.
characteristic. T2 hyperintense lesions in the cerebral • MRI f ndings o the brain primarily involve the basal
and cerebellar white have also been described. No ganglia and thalami with mixed T2 hyperintensity
enhancement is typically seen. rom swelling and atrophy, as well as hypointensity
rom copper deposition.
2. The “ ace o the giant panda” sign is characteristic o
• The “ ace o the giant panda” sign rom T2
Wilson disease when the midbrain tegmentum becomes
hyperintensity o the midbrain with normal
edematous with increased T2 signal. The normal T2
hypointensity o the red nuclei is characteristic.
hypointense signal o the red nuclei rom iron deposition
• Patchy white matter T2 hyperintensity o the
makes the eyes o the panda while the cerebral peduncles
cerebrum and cerebellum can also be seen.
make the ears.
• Treatment with chelating agents can reverse MRI
3. Wilson disease is an autosomal recessive disease f ndings.
with mutations o the ATP7B gene on chromosome
13q14-q21, which encodes an ATPase copper
transporting peptide.
Suggested Readings
4. Wilson disease is a disorder o copper transportation, Aggarwal A, Bhatt M. Update on Wilson disease. Int Rev
with the inability to excrete excess copper leading to Neurobiol. 2013 Nov;110(110):313-348.
deposition in the liver and other organ systems causing Trocello JM, Woimant F, El Balkhi S, et al. Extensive striatal,
ree radicals and cellular damage. cortical, and white matter brain MRI abnormalities in
5. Wilson disease can be treated by restricting dietary Wilson disease. Neurology. 2013 Oct;81(17):1557.
intake o copper, impairing the absorption o copper with
zinc, and chelating excess copper with penicillamine.
Orthotopic liver transplant can cure Wilson disease.

276
11-year-old female with delayed onset of walking and progressive
loss of function

1. What disease is the eye o the tiger sign


associated with?

2. What causes decreased signal o the globi


pallidi in this disease?

3. What areas other than the globi pallidi can


show decreased T2 signal?

4. What is the treatment o this disease?

5. What are typical clinical symptoms o the


disease?

277
Neurodegeneration with brain iron accumulation Case 122 (3180)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image of Axial T1 image of the Axial T2 of the midbrain Axial T2 image of a patient with “eye
the ganglionic level ganglionic level shows shows greater than expected of the tiger” sign seen with PANK2
shows signi cant T2 T1 shortening of the decreased signal of the mutations shows central globi pallidi T2
hypointensity of the globi pallidi (white cerebral peduncles from iron hyperintensity (white arrows), thought
bilateral globi pallidi from arrows) blending in with deposition in the substantia to represent cystic degeneration within
pathologic iron deposition myelinated white matter. nigra (white arrows). the iron deposition.
(white arrows).

Answers
1. The “eye o the tiger” sign is specif c or pantothenate Pearls
kinase-associated neurodegeneration with PANK2 • Neurodegeneration with brain iron accumulation
mutation. (NBIA) is an umbrella term that includes pantothenate
kinase–associated degeneration (PKAN), previously
2. Neurodegeneration with brain iron accumulation all
known as Hallervorden-Spatz.
demonstrate abnormal deposition o iron in the globi
• Decreased T2 signal and susceptibility on T2*
pallidi.
imaging rom iron deposition are seen symmetrically
3. Typical locations or normal or pathologic iron within the globi pallidi, substantia nigra, and variably
deposition other than the globi pallidi are the dentate in the dentate nuclei, corpus striatum, and cortex.
nuclei, substantia nigra, red nucleus, and putamen. • The “eye o the tiger” sign with central T2
hyperintensity within the T2 dark globi pallidi is
4. Aceruloplasminemia is the only subtype o
specif c or PANK2 mutation o PKAN.
neurodegeneration with brain iron accumulation that
• Care must be made to distinguish pathology rom
can be treated with iron chelation. Palliative care o
normal iron deposition o the globi pallidi with aging,
extrapyramidal symptoms, which develop in these
especially on 3T.
diseases, are indicated.
5. Extrapyramidal signs such as dysarthria and dystonia are
common symptoms. Ataxia and vision loss rom retinal
degeneration are also common symptoms. Suggested Readings
Guillerman RP. The eye-o -the-tiger sign. Radiology. 2000
Dec;217(3):895-896.
Hay ick SJ, Hartman M, Coryell J, Gitschier J, Rowley
H. Brain MRI in neurodegeneration with brain iron
accumulation with and without PANK2 mutations. AJNR
Am J Neuroradiol. 2010 Mar;27(6):1230-1233.
Kruer MC, Boddaert N, Schneider SA, et al.
Neuroimaging eatures o neurodegeneration with
brain iron accumulation. AJNR Am J Neuroradiol. 2012
Mar;33(3):407-414.

278
23-month-old male with loss of milestones, developmental regression,
and macrocephaly

1. What are the brain f ndings o this disease?

2. What can be seen within the spine o this


disease?

3. What is the metabolite deposited in the organs?

4. What is the treatment or this disease?

5. What are common initial presenting symptoms?

279
Hurler syndrome Case 123 (3183)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image of the supraganglionic brain shows numerous Axial FLAIR of the supraganglionic brain demonstrates
dilated perivascular spaces, including extension into the corpus suppression of the numerous dilated perivascular spaces with
callosum (white arrows). adjacent periventricular white matter T2 hyperintensity (white
arrows).

CT of the frontal skull shows ridging from metopic suture


Sagittal T1 just left of midline shows involvement of the corpus synostosis (white arrow).
callosum (white arrows), which is not usually involved in normal
variant dilated perivascular spaces.

280
Hurler syndrome (Cont.) Case 123 (3183)

Sagittal T2 image of the upper spine shows cervical stenosis with Sagittal T2 image of the lower spine shows a gibbus deformity
increased T2 signal in the cord (white arrows). Note large cystic and focal kyphosis at L1 (white arrow).
space of the posterior fossa (red arrow). Mega cisterna magna is
associated with mucopolysaccharidoses.

Answers
1. Mucopolysaccharidoses demonstrate dilated perivascular Pearls
spaces rom glycosaminoglycan (GAG) deposition with • Mucopolysaccharidoses are a group o inherited
T2 hyperintensities o the surrounding white matter. All metabolic disease that ail to break down
mucopolysaccharidoses can demonstrate this f nding glycosaminoglycans (GAG).
with the exception o Morquio syndrome (MPS4). • Type 1H (Hurler) is the prototype and most severe
orm.
2. Within the spine, cranial cervical junction stenosis
• All organ systems can be involved.
causing cervical myelopathy can be caused by deposition
• In the CNS, GAG is deposited in the perivascular
o GAG around the dens, atlantoaxial instability, and
spaces leading to dilatation and adjacent white matter
a short arch o C1. Lumbar gibbus de ormity with
T2 hyperintensity.
kyphosis can be seen in the most severe orms. Anterior
• Skeletal changes include metopic suture beaking,
wedging o the vertebral bodies is a skeletal dysplasia in
lumbar gibbus de ormity, and cranial cervical
mucopolysaccharidoses.
stenosis.
3. Heparan, dermatan, and chondroitin sul ate are all
glycosaminoglycans and are deposited in di erent types
o mucopolysaccharidoses.
Suggested Readings
4. Bone marrow transplant and recombinant enzyme Kwee RM, Kwee TC. Virchow-Robin spaces at MR imaging.
therapy can be used to treat mucopolysaccharidoses. Radiographics. 2010 Aug;27(4):1071-1086.
With the most severe orm MPS1H (hurler) the disease is Za eiriou DI, Batzios SP. Brain and spinal MR imaging
atal within a decade. f ndings in mucopolysaccharidoses: a review. AJNR Am J
5. A wide range o symptoms can be seen with Neuroradiol. 2013 Jan;34(1):5-13.
mucopolysaccharidoses including macrocephaly,
developmental delay, corneal clouding, gargoylism, and
sensorineural hearing loss.

281
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20-year-old female with new-onset left facial droop and sudden vision
loss, history of headaches and seizures, prior strokes

1. What are the typical MRI f ndings o this


disease?

2. What is the di erential diagnosis or this


appearance?

3. What is the clinical presentation o this


disease?

4. What is the pathogenesis o the disease?

5. What is the inheritance o this disease?


283
MELAS Case 124 (3187)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial FLAIR image through Axial DWI b = 1000 Coronal T2 image shows right Axial FLAIR image shows
the ganglionic level shows T2 image above the temporal and occipital hyperintensity the right temporal and
hyperintensity and swelling of ganglionic level shows and swelling from acute stroke-like occipital acute stroke-like
the right temporal lobe, insula, right posterior parietal lesion (white arrow). Note the focal lesion (white arrow) as well
and occipital lobe. In addition, the bright signal from an hyperintensity and swelling in the as a chronic lesion of the
pulvinar region of the thalamus is acute stroke-like lesion inferior left cerebellar hemisphere left temporal lobe tip with
also involved (white arrow) in these (asterisk). (red arrow), which showed no gliosis (red arrow).
stroke-like lesions. This involves di usion restriction consistent with a
both the PCA and MCA arterial subacute lesion.
distributions. Note the atrophy of
the left occipital lobe from prior
stroke-like involvement (red arrow).
Pearls
• MELAS (mitochondrial myopathy, encephalopathy,
Answers lactic acidosis, and stroke-like episodes) has variable
1. MELAS typically will have multiple stroke-like lesions phenotype and di erent clinical presentations.
primarily involving the posterior cerebrum and basal • MRI o MELAS shows stroke-like lesions
ganglia with di erent stages o evolution. Angiography indistinguishable rom vascular in arction but
is typically normal. typically does not ollow a vascular distribution.
• Stroke-like lesions usually involve the posterior
2. Leigh disease, MERRF, and vasculitis can give multiple
cerebrum and basal ganglia.
areas o parenchymal cytotoxic edema at di erent times.
• There may be multiple stroke-like lesions
Embolic stroke, especially with multiple emboli, can
demonstrating di ering ages o evolution.
give multiple vascular distribution in arcts and can give
• Consider MELAS or unexplained strokes in a young
strokes o di erent ages i the embolic source is not
patient.
treated.
3. The clinical presentation is varied with myopathy
inducing exercise intolerance, migraines, diabetes,
Suggested Readings
hearing loss, ailure to thrive or in antile presentation,
neuropsychiatric illness (such as depression), and Abe K, Yoshimura H, Tanaka H, Fujita N, Hikita T,
blindness (either rom cortical blindness or rom optic Sakoda S. Comparison o conventional and di usion-
atrophy). weighted MRI and proton MR spectroscopy in
patients with mitochondrial encephalomyopathy, lactic
4. Pathogenesis o MELAS is not exactly known but is acidosis, and stroke-like events. Neuroradiology. 2004
thought to involve oxidative phosphorylation def ciency Feb;46(2):113-117.
o the brain and vessel wall cells. Kim IO, Kim JH, Kim WS, Hwang YS, Yeon KM, Han MC.
5. MELAS, as a mitochondrial DNA disorder, is inherited Mitochondrial myopathy-encephalopathy-lactic acidosis-
through maternal mitochondria. Familial inheritance is and strokelike episodes (MELAS) syndrome: CT and MR
more common than sporadic mutations. 80% o MELAS f ndings in seven children. AJR Am J Roentgenol. 1996
patients will have mutation o MT-TL1. Mar;166(3):641-645.

284
7-year-old female with spasticity, developmental delays, and loss
of ambulation

1. What is the di erential diagnosis?

2. What is the signif cance o the di usion


restriction in this case?

3. What is the most common genetic de ect or


this group o diseases?

4. What is the prognosis o this disease?

5. What is the mode o genetic inheritance?

285
Leigh syndrome (non-SURF1) Case 125 (3229)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image through Axial FLAIR image through Axial b = 1000 DWI image MR spectroscopy of the basal ganglia
the ganglionic level of a the ganglionic level shows shows decreased di usion shows a prominent lactate doublet
patient with non-SURF1 bilateral symmetric caudate corresponding to bright peak at 1.4 ppm (white arrow).
Leigh syndrome shows T2 and putamen hyperintensity signal of the bilateral
hyperintensity of the bilateral as well as callosal and caudate heads and some
caudate and putamen (white periventricular white matter spots in the putamina (red
arrows) as well as the corpus involvement. Note the arrows). This correlates with
callosum and periventricular cavitation of the splenium more acute injury.
white matter (red arrows). and periatrial white matter
The bilateral caudate heads (white arrows).
appear swollen, while the 5. Leigh syndrome genetic mutations can be both
putamina appear atrophic. mitochondrial and nuclear with inheritance including
mitochondrial, autosomal recessive, and x-linked.

Answers Pearls
1. The di erential or bilateral, relatively symmetric basal • Leigh syndrome is a genetically heterogeneous
ganglia swelling, and di usion restriction includes group o metabolic neurodegenerative disorders with
anoxic injury and metabolic disease, which includes non- abnormality o mitochondrial respiration.
SURF1 Leigh syndrome, Krabbe, and Glutaric aciduria. • SURF1 mutations are the most common o the
The white matter involvement is commonly seen with Leigh syndromes that show characteristic symmetric
Krabbe, and is uncommon or Leigh syndrome. This involvement o the subthalamic nuclei, cerebellum,
patient has a non-SURF1 Leigh syndrome. SURF1 and lower brainstem. There is worse prognosis and
mutation Leigh syndrome characteristically does not rapid deterioration to death.
involve the basal ganglia. • Non-SURF1 Leigh disease usually involves the
2. In this case o Leigh syndrome, metabolic stressors bilateral basal ganglia, thalami, cerebral peduncles,
cause increased mitochondrial demand, which cannot be and midbrain tegmentum.
met by dys unctional mitochondrial respiration, leading • Acute areas o involvement typically show T2
to deterioration. hyperintensity rom swelling and di usion restriction
without signif cant enhancement.
3. Leigh syndrome is a genetically heterogeneous group • No treatment is available with most cases progressing
o diseases that causes de ects in mitochondrial to death in childhood.
respiration. The most common genetic de ect are
SURF1 mutations, which contribute to cytochrome C
oxidase (COX). Suggested Readings
4. Leigh syndrome typically presents within the f rst Arii J, Tanabe Y. Leigh syndrome: serial MR imaging
year o li e with progressive deterioration to death in and clinical ollow-up. AJNR Am J Neuroradiol. 2000
childhood or most patients. The SURF1 mutation Leigh Sep;21(8):1502-1509.
syndrome has an earlier onset with rapid progression Rossi A, Biancheri R, Bruno C, et al. Leigh syndrome with COX
and dismal prognosis. Episodes o deterioration seem to def ciency and SURF1 gene mutations: MR imaging f ndings.
coincide with metabolic stressors, such as in ection. AJNR Am J Neuroradiol. 2003 Sep;24(6):1188-1191.
286
69-year-old male with bilateral weakness

1. Where is the abnormality located?

2. What are the classic imaging f ndings o this


entity on MRI?

3. What is the di erential diagnosis?

4. What is the signif cance o high attenuation on


noncontrast CT with this entity?

5. What is the treatment or this entity?


287
Lymphoma metastasis Case 126 (3176)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT images without contrast shows high-density masses Axial CT postcontrast shows the high-density areas now
along both ependymal surfaces of the basal ganglia. Similar demonstrate enhancement along both ependymal surfaces of
attenuation is seen in the interpeduncular cistern (arrows). the basal ganglia and in the interpeduncular cistern (arrows).
Surrounding mass e ect is seen within the basal ganglia

3. The di erential diagnosis includes lymphoma, glioma,


and metastasis.
4. Non-Hodgkin lymphoma CNS metastasis classically
demonstrates high density on CT. This is likely
secondary to the high cellularity.
5. The treatment typically is intrathecal chemotherapy.

Pearls
• Non-Hodgkin lymphoma with CNS metastasis occurs
in 5% to 9% o patients.
• Metastatic CNS lymphoma commonly involves dura
and leptomeninges; parenchymal lesions can also
occur.
• When parenchymal lesions are seen, it is usually
secondary to inf ltration o perivascular spaces.
• Lymphoma typically has low T2 signal and high
density on CT.
• Lymphoma may restrict di usion on DWI.
Axial T2 image shows the high-density areas on CT with • A ter administration o contrast, homogeneous
corresponding low T2 signal (arrows). enhancement is seen.

Answers
1. The abnormality is located along the ependymal sur aces Suggested Readings
o the anterior horns o the lateral ventricle, basal Comert M, Bassullu N, Kaya E, Kocak A. Intracranial
ganglia, and third ventricle. involvement in a patient with Hodgkin’s lymphoma.
2. CNS metastasis o non-Hodgkin lymphoma typically Singapore Med J. 2011 Sep;52(9):e180-e183.
shows isointense to hypointense T1 signal and Slone HW, Blake JJ, Shah R, Guttikonda S, Bourekas EC.
hypointense T2 signal on MRI. A ter administration o CT and MRI f ndings o intracranial lymphoma. AJR Am
contrast, homogeneous enhancement is seen. Di usion- J Roentgenol. 2005 May;184(5):1679-1685.
weighted imaging may demonstrate restriction.

288
58-year-old male with history of headache

1. What is the di erential diagnosis?

2. What is the most common age group in this


tumor?

3. What is an imaging diagnostic clue in this


disease?

4. Where is the common location o this tumor?

5. What is the prognosis o this disease?

289
Gliosarcoma Case 127 (3319)
Case ranking/dif culty: Category: Intra-axial supratentorial

Noncontrast CT Axial T1 image Axial T2 FLAIR image Axial DWI ADC image Axial T1 postcontrast
image demonstrates a demonstrates a T1 demonstrates mass shows the mass has image demonstrates
hyperdense mass with hypointense lesion (white asterisk) with restricted di usion, an irregular thick rim
a hypodense center (white asterisk) with faint surrounding vasogenic likely representing enhancing lesion (white
(white asterisk) in the peripheral hyperintensity edema or tumor hypercellularity in a arrows) in the right
right temporo-occipital (white arrowheads) in the in ltration (white high-grade neoplasm temporo-occipital lobe.
lobe with surrounding right temporo-occipital arrowheads) in the right (asterisk).
vasogenic edema (white lobe. This may represent temporo-occipital lobe.
arrowheads). blood products. The rim of the mass is
dark while the center is
T2 hyperintense likely
representing necrosis
(red arrow).

Answers
1. Gliosarcoma and glioblastoma multi orme are Pearls
indistinguishable on imaging. Peripheral location with • Rare, primary intra-axial brain tumor (WHO grade IV).
dural and skull invasion is a clue to suggest gliosarcoma. • Considered as a histological variant o glioblastoma
Metastasis, abscess, and lymphoma can also be included multi orme (GBM).
in the di erential. • Two histological components: glial and mesenchymal
(sarcomatous) components.
2. Gliosarcoma occurs most commonly in males
• Predilection toward the temporal lobes.
(1.6:1, male: emale), typically f th to sixth decade.
• Unlike other primary CNS neoplasms, 15%-30%
3. A diagnostic clue or gliosarcoma is intra-axial metastasis to lungs, liver, and lymph nodes via the
peripheral location with dural and skull invasion. blood stream.
Glioblastoma will rarely have dural and skull invasion. • Radiographic eatures are indistinguishable rom
Hemangiopericytoma can appear similarly as an GBM, a heterogenous rim enhancing mass with
extraaxial mass with dural and skull invasion. central necrosis or hemorrhage associated with more
propensity or dural and/or skull invasion.
4. In decreasing order o requency, gliosarcoma most
• Poor prognosis with median survival o 6-12 months.
commonly occurs in temporal > parietal > rontal >
• Treatment options include surgical resection,
occipital lobes. Cerebellar hemisphere occurrence has
chemotherapy, and radiation.
been reported in the literature.
5. The prognosis o gliosarcoma is poor, with median
survival 6-12 months. Extracranial metastasis to lungs, Suggested Reading
liver, and lymph nodes is seen in 15%-30%, atypical or
primary CNS neoplasms. Lee YY, Castillo M, Nauert C, Moser RP. Computed
tomography o gliosarcoma. AJNR Am J Neuroradiol.
2000 Feb;6(4):527-531.

290
59-year-old male with increasing confusion

1. What are the di erential diagnoses?

2. What is the greatest risk actor or this disease?

3. What are di erences in imaging between


the immunodef cient and immunocompetent
individuals with this disease?

4. What imaging studies can be help ul in


distinguishing this disease rom glioblastoma?

5. What is the prognosis or this disease?


291
CNS lymphoma (multifocal) Case 128 (3365)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial FLAIR image shows Axial T1 postcontrast image Coronal T1 postcontrast image Axial DWI ADC map shows
a left frontal mass with shows intense enhancement shows ame-shaped irregular central decreased di usion
central hypointensity and of the central portions of the enhancement of the bilateral (arrow) corresponding to
surrounding T2 hyperintensity multifocal noncontiguous parietal lobe lesions (arrows). hypercellularity or high nuclear
(arrow). This extends to the masses is noted (arrows). to cytoplasmic ratio in this case
ventricular margin with focal of multifocal lymphoma.
mass e ect. A second T2
hyperintense lesion is seen
in the right temporal parietal Pearls
lobe subcortical white matter • Primary CNS Lymphoma (PCNSL) is predominantly
(arrowhead).
a B-cell non-Hodgkin lymphoma that presents in the
CNS without systemic involvement at diagnosis.
Answers
• Immunodef ciency is the greatest risk or developing
1. In this case o primary multi ocal CNS lymphoma, PCNSL.
the di erential includes progressive multi ocal • Incidence increases with age in immunocompetent
leukoencephalopathy, metastatic disease, tume active individuals.
demyelination, and multi ocal GBM. • PCNSL has a predilection or central periventricular
2. The greatest risk actor or primary CNS lymphoma is structures such as basal ganglia, thalami, white matter
immunodef ciency o which 95% is associated with the including the corpus callosum.
presence o the Epstein-Barr virus genome. • Multi ocal disease can be seen in 25%.
• Intense enhancement is seen with immunocompetent
3. Primary CNS lymphoma typically demonstrates intense
individuals with ring enhancement in
enhancement in immunocompetent individuals and ring
immunocompromised patients.
enhancement in immunocompromised individuals.
• Decreased ADC is typically seen either rom high
4. The vast majority o glioblastoma will show central nuclear to cytoplasmic ratio or rom hypercellularity.
necrosis, while central necrosis is a less common eature
or CNS lymphoma in immunocompetent individuals.
Primary CNS lymphoma shows lower CBV, ADC, and Suggested Readings
FA values compared to GBM; however, this is based on Berger JR. Mass lesions o the brain in AIDS: the dilemmas o
relatively ew studies. Overshoot o the baseline rom distinguishing toxoplasmosis rom primary CNS lymphoma.
f rst pass bolus on time to signal intensity curves o MRI AJNR Am J Neuroradiol. 2003 Apr;24(4):554-555.
DSC per usion seem to be characteristic o lymphomas Johnson BA, Fram EK, Johnson PC, Jacobowitz R. The variable
compared to GBM or metastatic disease. MR appearance o primary lymphoma o the central nervous
5. Treatment with chemotherapy and radiation helps prolong system: comparison with histopathologic eatures. AJNR Am
survival, although most patients succumb to disease J Neuroradiol. 1997 Mar;18(3):563-572.
recurrence. Age greater than 60 years and involvement Yamashita K, Yoshiura T, Hiwatashi A, et al. Di erentiating
o the deep brain regions are some o the poor prognostic primary CNS lymphoma rom glioblastoma multi orme:
actors. Corticosteroid administration can show dramatic assessment using arterial spin labeling, di usion-weighted
imaging improvement, although all patients demonstrate imaging, and 18F- uorodeoxyglucose positron emission
recurrence o disease once corticosteroids are stopped. tomography. Neuroradiology. 2013 Feb;55(2):135-143.
292
13-year-old male with seizures

1. What is the di erential diagnosis?

2. What is the embryological structure o origin


or this tumor?

3. What are typical MRI f ndings o this tumor?

4. What is the typical clinical presentation?

5. What is the prognosis or this disease?

293
Dysembryoplastic neuroepithelial tumor Case 129 (3369)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image shows a Axial FLAIR image shows Axial DWI ADC map shows Sagittal T1 postcontrast image
T2 hyperintense, cyst-like little adjacent edema with increased di usion of the shows the lesion involving the
cortically based lesion increased to isointense tumor (arrow). cortex spanning the parietal
(arrow) in the right parietal signal of the tumor (arrow). occipital ssure without signi cant
occipital lobe, which is well The tumor is mildly wedge enhancement. The central linear
circumscribed. shaped and points toward enhancing structures represent
the lateral ventricle. cortical vessels (arrowhead).

Answers
1. The di erential or a cortically based T2 hyperintense, Pearls
cystic appearing lesion includes dysembryoplastic • Dysembryoplastic neuroepithelial tumors (DNET)
neuroepithelial tumor, cortical dysplasia o Taylor, and are benign WHO grade I cortically based tumors that
neuroepithelial cyst. typically present with complex partial seizures in the
second to third decade o li e.
2. The origin o dysembryoplastic neuroepithelial tumors
• The typical appearance is a bubbly, cyst-like cortically
is thought to be dysplastic cells that originate in the
based lesion that is wedge shaped and points toward
germinal matrix and migrate out to the cortex.
the lateral ventricles.
3. Dysembryoplastic neuroepithelial tumors typically • The cyst-like lesions are typically T2 bright with
appear as bubbly, cyst-like, well-circumscribed, isointense to hypointense FLAIR signal, and FLAIR
cortically based lesions without adjacent edema. There bright rim to the cyst-like lesions.
is a characteristic bright FLAIR rim sign o the cyst- • There is usually no adjacent edema.
like lesions, which usually are wedge shaped and point • Enhancement occurs in only 30% o lesions with
toward the lateral ventricles. nodular and ring enhancement.
• Recurrence is rare and may show atypical
4. Patients with dysembryoplastic neuroepithelial tumors
enhancement, but remains benign.
typically present with complex partial seizures.
• Complete surgical resection is curative.
5. Dysembryoplastic neuroepithelial tumors are indolent, • Malignant degeneration is rare.
with cure rom complete surgical resection. Recurrence
and malignant trans ormation are rare.
Suggested Readings
Campos AR, Clusmann H, von Lehe M, et al. Simple and
complex dysembryoplastic neuroepithelial tumors (DNT)
variants: clinical prof le, MRI, and histopathology.
Neuroradiology. 2009 Jul;51(7):433-443.
Fernandez C, Girard N, Paz Paredes A, Bouvier-Labit
C, Lena G, Figarella-Branger D. The use ulness o
MR imaging in the diagnosis o dysembryoplastic
neuroepithelial tumor in children: a study o 14 cases.
AJNR Am J Neuroradiol. 2003 May;24(5):829-834.
294
12-year-old male with history of complex partial seizures

1. What is the di erential diagnosis?

2. What are typical f ndings or this tumor?

3. What part o the brain is most commonly


involved or this tumor?

4. What is the typical clinical presentation?

5. What is the prognosis or this disease?

295
Ganglioglioma Case 130 (3371)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image shows an Coronal FLAIR image shows Axial T1 postcontrast shows Axial DWI b = 1000 image
isointense mass projecting mild hyperintensity of the homogeneous enhancement shows no di usion restriction
from the left uncus into the nodule (arrow) projecting of the nodular mass (arrow). of the nodular mass (arrow).
suprasellar cistern (arrow). from the uncus with
associated thickening of the
parahippocampal cortex 4. Temporal lobe seizures are the most common (90%)
(arrowhead). presentation o gangliogliomas. Gangliogliomas are
the most common neoplastic cause o temporal lobe
seizures.
5. Gangliogliomas are typically WHO grade I-II tumors
with complete surgical resection being curative. 94%
recurrence- ree survival at 7.5 years has been reported.

Pearls
• Gangliogliomas are the most common neoplastic
cause o temporal lobe epilepsy.
• Gangliogliomas are the most common mixed glial-
neuronal tumors.
• Gangliogliomas are primarily a tumor o children and
young adults with 80% o patients less than 30 years
o age.
• Most common location is the temporal lobe (>75%).
• Morphology o the tumor can be solid with gyral
Color CBV map from dynamic susceptibility contrast perfusion thickening or cyst with mural nodule.
imaging shows no increased perfusion of the tumor (arrow). • Enhancement is variable, with calcif cation common.
• Prognosis is excellent with complete surgical
resection.
Answers • Malignant degeneration is rare o the glial component.
1. The di erential or a well-circumscribed tumor in
the temporal lobe includes ganglioglioma, pilocytic
astrocytoma, pleomorphic xanthoastrocytoma, and
oligodendroglioma. Suggested Readings
Adachi Y, Yagishita A. Gangliogliomas: characteristic
2. Gangliogliomas can have a solid nodular appearance
imaging f ndings and role in the temporal lobe epilepsy.
that can cause gyral thickening or a circumscribed cyst
Neuroradiology. 2008 Oct;50(10):829-834.
with mural nodule.
Shin JH, Lee HK, Khang SK, et al. Neuronal tumors o the
3. Greater than 75% o gangliogliomas occur in the central nervous system: radiologic f ndings and pathologic
temporal lobe ollowed by the rontal and parietal lobes. correlation. Radiographics. 2003 Jan;22(5):1177-1189.
296
15-year-old male with a witnessed seizure

1. What is the di erential diagnosis?

2. What is the most common location or this


tumor?

3. Where does the tumor usually originate?

4. What are the typical imaging characteristics o


this tumor?

5. What is the prognosis or the disease?

297
Oligodendroglioma Case 131 (3373)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T1 image shows a well-circumscribed densely calci ed Axial FLAIR image shows a well-circumscribed T2 hyperintense
lesion in the right frontal lobe (arrow). lesion in the cortical subcortical region of the right frontal lobe
without surrounding T2 hyperintensity. The central aspect of the
lesion shows decreased T2 signal likely due to calci cation (arrow).

Axial GRE image shows signi cant hypointensity without


blooming artifact of the lesion from calci cation (arrow).
Axial T1 postcontrast image shows heterogeneous internal
enhancement (arrow).

298
Oligodendroglioma (Cont.) Case 131 (3373)

Axial DWI b = 1000 image shows no restricted di usion of the Axial color CBV map from dynamic susceptibility contrast
tumor (arrow). perfusion technique shows relative low perfusion in the tumor
(arrow). Oligodendrogliomas can show high CBV and is a
confounding tumor on perfusion imaging, which can be mistaken
Answers for a high-grade neoplasm.
1. The di erential o a well-circumscribed cortically based
mass should include oligodendroglioma, ganglioglioma,
and pleomorphic xanthoastrocytoma. Given the
Pearls
calcif cation, late-stage neurocysticercosis could be
considered. • Oligodendrogliomas are WHO grade II tumors that have
a tendency to progress to the grade III anaplastic variant.
2. 50%-65% o oligodendrogliomas are located in the • These are tumors o young to middle-aged adults.
rontal lobe. • They occur most commonly in the rontal lobes
3. Oligodendrogliomas are usually cortical with subcortical involving the cortex and subcortical white matter.
extension. • They are usually circumscribed with little adjacent
edema but may also uncommonly present in an
4. Oligodendrogliomas are usually well demarcated inf ltrative pattern.
with minimal adjacent edema. They are commonly • Oligodendrogliomas are typically T2 hyperintense
T2 hyperintense with variable heterogeneous with variable heterogeneous enhancement.
enhancement. 70%-90% will show some calcif cation. • Progression to enhancement o a previously
Oligodendrogliomas can be con ounding tumors on nonenhancing tumor may suggest malignant
per usion imaging as low-grade tumors can show degeneration.
increased cerebral blood volume. • 70%-90% o oligodendrogliomas will have calcif cation.
5. Although the median survival is longer in grade II • Increased CBV on per usion imaging can be seen,
oligodendrogliomas compared to grade II astrocytomas, which may mistake this tumor or a more higher-grade
most patients succumb to the tumor due to recurrence neoplasm.
and malignant trans ormation to anaplastic • MR spectroscopy may be help ul to distinguish grade
oligodendroglioma. Complete surgical resection and II and grade III tumors with the presence o lactate in
1p, 19q deletions (80% o oligodendrogliomas) are the higher-grade neoplasm.
associated with more avorable prognosis as these
deletions are associated with increased sensitivity o
tumor cells to treatment.
299
Oligodendroglioma (Cont.) Case 131 (3373)

Suggested Readings
Chawla S, Krejza J, Vossough A, et al. Di erentiation
between oligodendroglioma genotypes using dynamic
susceptibility contrast per usion-weighted imaging and
proton MR spectroscopy. AJNR Am J Neuroradiol. 2013
Aug;34(8):1542-1549.
Khalid L, Carone M, Dumrongpisutikul N, et al. Imaging
characteristics o oligodendrogliomas that predict grade.
AJNR Am J Neuroradiol. 2012 May;33(5):852-857.

300
16-year-old male with history of slurred speech, blurred vision,
and altered mental status

1. What is the di erential or these MRI f ndings


in a child?

2. What MRI f ndings correlate with malignancy?

3. Compared to medulloblastoma (cerebellar


PNET) what is the prognosis or this disease?

4. What are the typical imaging characteristics o


this tumor?

5. What imaging is necessary or staging prior to


surgery?

301
Supratentorial primitive neuroectodermal tumor Case 132 (3376)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image shows a heterogeneous mass centered in the Axial FLAIR image shows the in ltrative adjacent T2 prolongation
left basal ganglia with surrounding in ltrative T2 hyperintensity. likely representing tumor in ltration with associated mass e ect
Note the central T2 hypointensity (white arrow) that can be (arrows).
seen with highly cellular tumors. There is also a small focal
area of hemorrhage (red arrowhead) and cystic necrosis (white
arrowhead).

Axial DWI b = 1000 image shows focal areas of di usion restriction


in the central enhancing component (white arrow), the medial rim
(white arrowhead), and an anterior region that is nonenhancing
Axial T1 postcontrast shows the central heterogeneous (red arrowhead). These correlate with areas of high cellularity and
enhancement (arrow). tumor in ltration.

302
Supratentorial primitive neuroectodermal tumor (Cont.) Case 132 (3376)

MRI spectroscopy at 30 TE of the nonenhancing region shows Axial color CBV image from dynamic susceptibility contrast
signi cant elevation of choline (white arrowhead), decreased perfusion imaging shows areas of increased perfusion, which
NAA (white arrow), and the presence of a lactate doublet (red correspond to the areas of di usion restriction (arrows).
arrowhead), consistent with a malignant tumor pro le.

Answers
Pearls
• Supratentorial primitive neuroectodermal tumors
1. The di erential or a heterogeneous hemispheric mass
(S-PNET) are uncommon malignant, poorly
with malignant eatures in a child includes glioblastoma
di erentiated WHO grade IV embryonal tumors
multi orme, primitive neuroectodermal tumor, atypical
that share signif cant pathology with medulloblastoma.
teratoid rhabdoid tumor, and choroid plexus carcinoma
• Compared to medulloblastoma (Cerebellar PNET),
with signif cant parenchymal invasion.
S-PNET patients generally have worse outcome,
2. T2 hypointensity and decreased di usion correlate with suggesting di erent oncogene expression.
hypercellular areas o neoplasms, which correspond to • Hemispheric S-PNET is typically large and
higher-grade malignancy. This can be seen in this case heterogeneous at presentation and may show
o primitive neuroectodermal tumor. signif cant inf ltration, or surprisingly little adjacent
T2 prolongation or size.
3. Medulloblastomas with similar histology to supratentorial
• Cystic necrosis, hemorrhage, and calcif cation are
PNET are also considered in ratentorial or cerebellar
common.
PNET. However, prognosis is signif cantly worse or
• Heterogeneous enhancement is typical.
supratentorial PNET than medulloblastoma. Furthermore,
• Solid components will show decreased T2 and
younger children with supratentorial PNET tend to do
decreased di usion corresponding with high cellularity.
worse than older children, which may be a re ection o
• CSF seeding is common, which necessitates spine
underutilization o radiotherapy in very young children.
imaging prior to surgery.
4. Supratentorial PNETs are typically large at presentation,
with heterogeneous enhancement and areas o
hemorrhage and cystic necrosis. Suggested Readings

5. Spine imaging with contrast is necessary prior to surgery Erdem E, Zimmerman RA, Haselgrove JC, Bilaniuk
to evaluate or drop metastases. Supratentorial PNET, LT, Hunter JV. Di usion-weighted imaging and uid
similar to medulloblastoma, has a propensity or CSF attenuated inversion recovery imaging in the evaluation o
dissemination. primitive neuroectodermal tumors. Neuroradiology. 2001
Nov;43(11):927-933.
Klisch J, Husstedt H, Hennings S, von Velthoven V,
Pagenstecher A, Schumacher M. Supratentorial primitive
neuroectodermal tumours: di usion-weighted MRI.
Neuroradiology. 2000 Jun;42(6):393-398.
303
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49-year-old male with severe headache and visual changes

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the signif cance o low T1 signal in


this entity?

5. What is the signif cance o the high T1 signal


with this lesion?

305
Hemorrhagic metastasis Case 133 (3487)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T1 image shows a parenchymal Axial T2 image shows the lesion Axial T1 postcontrast shows the intra-axial
lesion within the left occipital lobe that is of mixed T2 signal but mostly lesion within the left occipital lobe has central
is of mixed T1 signal with peripheral T1 hyperintense to brain with a rim of low enhancement (red arrow). A second focus of
predominantly isointense signal (arrow). signal (arrows): The peripheral blood enhancement is seen within the left frontal
product is primarily acute in nature. lobe, which is much smaller (blue arrow).

Pearls
• Hematomas on MRI have variable signal depending
on the age o the blood.
• T2* imaging blooming conf rms blood products.
• Intermediate T1, Dark T2 = acute, deoxyhemoglobin.
• Bright T1, Dark T2 = early subacute, intracellular
methemoglobin.
• Bright T1, Bright T2 = late subacute, extracellular
Axial CT image shows the methemoglobin.
peripheral hemorrhage of
• Dark T1, Dark T2 = chronic, hemosiderin.
the left occipital mass (red
arrow) with adjacent edema
• Follow-up MRI should be considered in patients
(asterisk). without a diagnosis o cancer in order to exclude an
underlying tumor.
• Melanoma, renal cell, choriocarcinoma, and thyroid
Answers metastases are prone to hemorrhage (“Mr. CT”).
1. The abnormality is located within the le t occipital lobe. • A hemorrhagic metastasis is likely to be lung or breast
carcinoma due to higher incidence o these cancers.
2. The classic imaging f ndings or hemorrhagic metastases
are multiple intra-axial lesions with associated hemorrhage.
On CT, high density is seen, and on MRI, the f ndings are Suggested Readings
variable and depend on the age o the blood products. Chan JH, Peh WC. Methemoglobin suppression in T2-
3. The di erential diagnosis includes metastasis, abscess, weighted pulse sequences: an adjunctive technique in MR
and hematoma. imaging o hemorrhagic tumors. AJR Am J Roentgenol.
1999 Jul;173(1):13-14.
4. Chronic blood is identif ed by low signal on both T1 Janick PA, Hackney DB, Grossman RI, Asakura T. MR
and T2 secondary to magnetic susceptibility rom imaging o various oxidation states o intracellular and
hemosiderin and erritin, which coincides with a bleed extracellular hemoglobin. AJNR Am J Neuroradiol. 1993
that is weeks to years in age. May;12(5):891-897.
5. On MRI, the appearance o hyperintense T1 signal Toh CH, Wei KC, Ng SH, Wan YL, Lin CP, Castillo M.
indicates methemoglobin, which signif es subacute Di erentiation o brain abscesses rom necrotic glioblastomas
hemorrhage. and cystic metastatic brain tumors with di usion tensor
imaging. AJNR Am J Neuroradiol. 2011 Oct;32(9):1646-1651.
306
23-year-old male with altered consciousness after trauma

1. Where is the CT abnormality located?

2. Where are lesions most commonly located in


this entity?

3. What is the most sensitive MR sequence or


this entity?

4. What is the etiology or these lesions?

5. What causes the high CT attenuation?

307
Di use axonal injury Case 134 (3118)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial CT shows small foci of hyperattenuation. Two within the Axial T2 GRE shows multiple, small foci of low signal are seen within
midbrain (red arrows) and one within the right frontal lobe (green the midbrain and temporal lobes gray-white junctions (arrows).
arrow). A small amount of subarachnoid blood is present in the
interpeduncular cistern (blue arrow). Note the paucity of sulci and
poor de nition of the ambient cistern indicating elevated ICP
(white arrow). Pearls
• Di use axonal injury (DAI) is caused by a high-
velocity traumatic event resulting in a tensile strain
Answers
injury.
1. Hyperattenuation is seen within the midbrain and right • DAI occurs secondary to the di erent densities o
rontal lobe. white and gray matter, which accelerate and decelerate
2. The classic lesion locations in di use axonal injury at di erent rates.
include the gray-white junctions, dorsolateral midbrain, • The resultant hemorrhages mani est most commonly
and corpus callosum (splenium the most common). at the gray-white junctions, midbrain, corpus callosum
(splenium most common), ependymal lining, and
3. The most sensitive MR sequences or this entity are superior cerebellar peduncles.
GRE (gradient recalled echo) and SWI (susceptibility- • These hemorrhages are usually less than 1 cm in size.
weighted imaging), which are T2* weighted sequences. • Use GRE and SWI images or easier detection.
4. This entity is secondary to traumatic brain injury. • A negative CT with a low GCS, consider ollow-up
MRI to exclude DAI.
5. The cause o high CT attenuation is blood products. • DAI location triad: gray-white junction, corpus
callosum, and dorsolateral midbrain.

Suggested Readings
Aiken AH, Gean AD. Imaging o head trauma. Semin
Roentgenol. 2010 Apr;45(2):63-79.
Blitstein MK, Tung GA. MRI o cerebral microhemorrhages.
AJR Am J Roentgenol. 2007 Sep;189(3):720-725.

308
Prematurity; born at 24 weeks of gestation

1. Where is the abnormality located?

2. What are the f ndings in this entity?

3. What is the best modality to diagnose this


entity?

4. What are the causes o this f nding?

5. What is in the di erential or periatrial


hyperintense FLAIR signal in in ants and
young children?

309
Periventricular leukomalacia Case 135 (811)
Case ranking/dif culty: Category: Intra-axial supratentorial

Sagittal T1 image shows thinning Coronal ultrasound at 10 weeks


Axial T1 image shows undulating, irregular walls of the splenium (blue arrow) and of age shows cystic change (green
(green arrow) of lateral ventricles (red arrow) due to posterior part of the body (green arrow) in the periventricular white
thinning of periventricular white matter (blue arrows). arrow) of the corpus callosum due matter. Ventricles are dilated (red
White matter is so thinned that the sulci and gyri to decreased number of crossing arrow).
(black arrow) almost reach the ventricles. Ventricles bers from loss of white matter.
have collapsed after shunt placement.

Answers
1. The abnormality is located within the white matter. Pearls
2. White matter injury o prematurity (W-MIP) is a better • Periventricular leukomalacia is a white matter
terminology or periventricular leukomalacia as any injury o prematurity and the sequela o a watershed
white matter may be a ected: deep, subcortical, or in arction.
periventricular. In addition to white matter, neuronal • Three types o white matter injury o prematurity in
and axonal injury may also involve the thalami, basal order o increasing severity o clinical mani estations:
ganglia, brain stem, cerebellum, and cerebral cortex. di use noncavitary, ocal/multi ocal noncavitary, and
Volume loss o the periventricular white matter and ocal/multi ocal cavitary.
corpus callosum with ex-vacuo dilatation o ventricles • MRI shows hyperintense punctate T1 oci in early
and irregular margins o the ventricles is typically seen stages ollowed by some hypointensities within these
in the chronic stage. hyperintense oci due to lique active necrosis.
• Necrosis may lead to small cavitations that may
3. MRI is the best modality to detect white matter injury. coalesce and orm larger cavities.
Ultrasound diagnoses cavitary white matter injury but • Imaging in the chronic stage shows di use white
underdiagnoses noncavitary white matter injury. matter loss, angular and wavy ventricular margins,
4. Most o the time, the periventricular white matter injury and periventricular gliosis.
is due to hypoxic ischemic injury in premature neonates.
Premature neonates are also at risk or intraventricular
hemorrhage. Hence, many o the in ants with W-MIP Suggested Readings
may have past history o intraventricular hemorrhage that
may be seen as old hemorrhagic products on gradient/ Izbudak I, Grant PE. MR imaging o the term and preterm
susceptibility MR sequence. However, periventricular neonate with di use brain injury. Magn Reson Imaging
white matter injury can also occur in other situations Clin N Am. 2011 Nov;19(4):709-31; vii.
like in ection, metabolic disease, hydrocephalus, and Sie LT, van der Knaap MS, van Wezel-Meijler G, Taets
congenital heart disease. van Amerongen AH, La eber HN, Valk J. Early MR
eatures o hypoxic-ischemic brain injury in neonates
5. Other than white matter injury rom hypoxic ischemic with periventricular densities on sonograms. AJNR Am J
injury, terminal myelination zones in the periatrial white Neuroradiol. 2000 May;21(5):852-861.
matter can also be a normal cause o T2 hyperintensity Volpe JJ. Hypoxic-ischemic encephalopathy: neuropathology
in the periventricular white matter. Transependymal and pathogenesis. In: Volpe JJ, ed. Neurology of the
seepage o CSF in hydrocephalus can also cause Newborn. Philadelphia, PA: Saunders-Elsevier; 2008.
hyperintense T2 signal in periventricular regions.
310
70-year-old male with headaches

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What is the di erential diagnosis?

4. What is the signif cance o low signal on MRI


GRE images with this entity?

5. What is the treatment or this entity?

311
Capillary telangiectasia Case 136 (3072)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T1 image does not show any ndings. The (arrow) denotes Axial T2 image shows increased signal in the right midbrain near
where the lesion will be seen on the other sequences. the red nucleus (arrow).

3. The di erential diagnosis includes glioma, capillary


telangiectasia, and metastasis.
4. Hemorrhage is only seen when associated with
cavernous mal ormations. Decreased T2* is thought to
be deoxyhemoglobin within dilated capillaries.
5. No treatment is needed. These are considered “no touch”
vascular mal ormations.

Pearls
• Capillary telangiectasia is a benign vascular
mal ormation that is usually asymptomatic and does
not require treatment.
• The most common location is in the brain stem
(most o ten the pons).
• Faint brain stem “brush-like or stipple” enhancement.
• Usually subcentimeter in size.
Axial T1 postcontrast image shows correlating faint enhancement • When mass e ect is present consider tumor (metastasis
(arrow). or glioma).

Answers
1. The abnormality is located within the midbrain. Suggested Reading
Castillo M, Morrison T, Shaw JA, Bouldin TW. MR imaging
2. The classic appearance on MRI is almost nonperceptable
and histologic eatures o capillary telangiectasia
on T1 and T2. T2 may demonstrate high signal, but the
o the basal ganglia. AJNR Am J Neuroradiol. 2001
hallmark f nding is “brush-like” enhancement.
Sep;22(8):1553-1555.

312
65-year-old female with aphasia for 2 weeks

1. Where is the abnormality located?

2. What are the classic imaging f ndings or this


entity?

3. What are possible causes or this entity?

4. What time course a ter an ischemic event does


this entity appear?

5. What is the treatment or this entity?

313
Cortical laminar necrosis Case 137 (3113)
Case ranking/dif culty: Category: Intra-axial supratentorial

Sagittal T1 image shows gyriform high signal within the left Axial T2 images shows gyriform high T2 signal within the left
parietal cortex with volume loss (circle). parietal cortex (green arrow). There is associated volume loss and
gliosis (red arrow).

Answers
1. The abnormality is located in the parietal lobe. Pearls
• Cortical laminar necrosis (CLN) can be seen in the
2. On CT, there is usually linear gyri orm high density
subacute phase o cytotoxicity o the cortex, most
within the cortical gray matter. On MRI, this is
commonly the sequela o an ischemic event.
recognized by gyri orm high T1 signal involving the
• CLN can also be seen with chemotherapy or
gray matter with corresponding volume loss.
immunosuppressive treatment.
3. Cortical laminar necrosis can be a subacute sequela • This insult occurs in the third layer o the cortex.
o an ischemic event, but has also been described with • I ring enhancement is seen, consider metastasis.
chemotherapy or immunosuppressive treatment. • I mass e ect is present, consider neoplasm.
4. Cortical laminar necrosis can typically appear 2 weeks
a ter an ischemic event.
Suggested Reading
5. No treatment is necessary or this entity. Workup
or stroke or other causes o cortical injury may be Ginat DT, Meyers SP. Intracranial lesions with high signal
necessary. intensity on T1-weighted MR images: di erential
diagnosis. Radiographics. 2012;32(2):499-516.

314
19-year-old male, postcardiac arrest

1. What are common causes o this f nding?

2. What is the most sensitive imaging modality?

3. What is the di erential diagnosis?

4. In mild-to-moderate ischemic injury, where are


the expected location o abnormalities?

5. What structures are more susceptible to


ischemic injury due to high metabolic activity?

315
Di use cerebral edema from hypoxic ischemic injury Case 138 (3320)
Case ranking/dif culty: Category: Diagnostic

Axial noncontrast CT Axial noncontrast CT Axial T1 image demonstrates Axial FLAIR image demonstrates
demonstrates di use e acement demonstrates di use e acement di use e acement of di use e acement of cortical
of sulci, suprasellar, and of cortical sulci and lateral cortical sulci (white arrows) sulci and lateral ventricles
quadrigeminal cisterns (white ventricles (white arrowheads). and lateral ventricles (white (white arrowheads). The basal
arrowheads). arrowheads). ganglia appear increased in
T2 hyperintensity (asterisks).

Pearls
• Cardiac arrest and cerebrovascular disease are the
most common causes o hypoxia in the adult.
• Highly variable radiographic f ndings depend on
severity and duration o insult, and type and timing o
imaging studies.
• Mild-to-moderate global ischemia usually results in
watershed zone in arcts.
DWI ADC image demonstrates DWI ADC image demonstrates • Severe ischemia primarily a ects gray matter
di use restricted di usion of di usion restricted cortices structures due to high metabolic activity.
the cortices (white arrowheads) and hippocampi bilaterally • CT f ndings include di use cerebral edema,
and basal ganglia (white arrow) (white arrowheads), sparing the e acement o the cortical sulci, loss o gray-white
bilaterally, relatively sparing cerebellum (white asterisk). di erentiation, reverse gray-white attenuation, and
bilateral thalami (white asterisks). white cerebellum sign.
• DWI and MRS are the most sensitive modalities with
Answers DWI becoming positive in the f rst hour.
1. Hypoxic ischemic injury in adults is more o ten a result
o cardiac arrest or cerebrovascular disease. Drowning
and asphyxia remain common causes in older children. Suggested Reading
2. DWI is the most sensitive imaging modality and becomes Huang BY, Castillo M. Hypoxic-ischemic brain injury:
positive in the f rst hour a ter an ischemic event. imaging f ndings rom birth to adulthood. Radiographics.
2008 Aug;28(2):417-439; quiz 617.
3. Hypertensive encephalopathy, toxic/metabolic
encephalopathy, and CJD are the di erential diagnoses.
4. In mild-to-moderate hypoxic events, the watershed zone
is the typical location due to lowest per usion in the
distal f eld o vascular supply.
5. Gray matter and deep gray nuclei are areas at risk rom
a severe hypoxic event due to high number o dendritic
synapses and high metabolic activity.
316
5-day-old term infant status postcomplicated vaginal delivery with
vacuum extraction

1. What structures are a ected in mild-to-


moderate injury?

2. What is the most common cause o this


disease?

3. When is DWI most help ul in this disease and


what is typically seen?

4. What can be seen in the subacute phase on T1


and T2 imaging?

5. What imaging f ndings correlate with worse


prognostic outcome?

317
Hypoxic ischemic injury (term) Case 139 (3440)
Case ranking/dif culty: Category: Intra-axial supratentorial

Sagittal T1 image demonstrates a large cephalhematoma (white Axial T1 image at the level of the basal ganglia demonstrates greater
asterisk) in the vertex as well as a thin layer of T1 shortening along than expected T1 hyperintensity of the putamen and ventrolateral
the tentorium cerebelli and posterior fossa (white arrowheads), thalami (arrowheads) indicating subacute ischemic injury.
representing subdural blood from birth trauma.

Axial ADC image demonstrates resolving restricted di usion


involving subcortical white matter di usely of the bilateral
Axial T2 image shows T2 hypointensity of the putamen and cerebral hemispheres (white arrowheads) with more intense
ventrolateral thalami from subacute injury (arrowheads). Note the restriction in the splenium of the corpus callosum and parietal
layering intraventricular blood (arrow), and areas of gyral swelling subcortical white matter (white arrows). This is indicative of
from edema (red arrows). delayed cellular death of severe di use ischemic injury.
318
Hypoxic ischemic injury (term) (Cont.) Case 139 (3440)

Pearls
• Perinatal asphyxia is the most important cause o
hypoxic ischemic injury.
• Mild-to-moderate hypoper usion usually a ects
intervascular watershed zones along parasagittal
locations, including cortex and white matter.
• Severe hypoper usion usually a ects gray matter
including ventrolateral thalami, posterior putamina,
hippocampi, corticospinal tracts, and sensorimotor
cortex.
• DWI per ormed between 24 hours and 5 days o li e
is more sensitive or the detection o cytotoxic edema,
with aster pseudonormalization compared to adults.
• Subacute (3-6 days) changes typically show increased
T1 and decreased T2 signal.
• Chronic changes will show progression to volume
loss, cystic encephalomalacia, and gliosis.
• MR spectroscopy is the most sensitive in detection
o metabolic derangement o brain injury with
decreasing NAA and increasing lactate peaks
MR spectroscopy with voxel placement in the right periventricular correlating with worse prognosis.
white matter (TE 270 ms) demonstrates decreased NAA (white
arrowhead) with increased lactate peaks (white arrow).

Suggested Readings
Answers Chao CP, Zaleski CG, Patton AC. Neonatal hypoxic-ischemic
1. Mild-to-moderate hypoper usion injury in hypoxic encephalopathy: multimodality imaging f ndings.
ischemic injury o the term neonate a ects intravascular Radiographics. 2006 Oct;26(suppl 1):S159-S172.
watershed zones, including the cortex and parasagittal Liauw L, van der Grond J, van den Berg-Huysmans AA,
white matter. Palm-Meinders IH, van Buchem MA, van Wezel-Meijler
G. Hypoxic-ischemic encephalopathy: diagnostic value o
2. Perinatal asphyxia secondary to di f cult birth is the most
conventional MR imaging pulse sequences in term-born
common cause o hypoxic ischemic injury o the term
neonates. Radiology. 2008 Apr;247(1):204-212.
neonate.
3. ADC pseudonormalization occurs aster in neonates
than in adults, within 5-7 days versus 7-10 days. DWI is
more sensitive than T1/T2 images in the acute phase o
hypoxic ischemic injury, especially in the background
o normal unmyelinated white matter. DWI abnormality
can increase a ter the f rst ew days o injury rom
delayed cell death.
4. In the subacute phase o hypoxic ischemic injury (starting
3-6 days), a ected brain structures show T1 hyperintensity
and T2 hypointensity. As ADC begins to pseudonormalize
in this phase, care ul evaluation o T1 and T2 changes is
important to understand the extent o injury.
5. Severe hypoper usion injury involves the deep gray
matter, perirolandic cortex, brainstem, and cerebellum.
These f ndings, along with decreasing NAA and
increasing lactate, correlate with poor outcome.
319
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19-year-old female, on hemodialysis, with altered mental status

1. What is the di erential diagnosis?

2. What is the typical clinical prof le?

3. How o ten is there CNS involvement in this


disease?

4. What are the patterns o radiographic eatures?

5. How o ten does angiography o any modality


demonstrate vasculitic changes o this disease?

321
Lupus vasculitis Case 140 (3442)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial FLAIR image demonstrates multifocal FLAIR hyperintensities Axial FLAIR image demonstrates multifocal FLAIR hyperintensities
predominantly involving the cortex and juxtacortical/subcortical predominantly involving the cortex and juxtacortical/subcortical
white matter of the bilateral cerebral hemispheres (white arrowheads). white matter of the temporal occipital lobes (white arrowheads) as
well as in the right thalamus (black asterisk).

Coronal FLAIR image again demonstrates bilateral cortical and


subcortical hyperintensities (white arrowheads) also involving the DWI image demonstrates restricted di usion in the left thalamus
right thalamus (black asterisk) and pons (white arrows). (white arrowhead) from focal infarct.
322
Lupus vasculitis (Cont.) Case 140 (3442)

MRA MIP image of the right internal carotid artery demonstrates


di use irregular narrowing of the precavernous, cavernous
internal carotid artery as well as A1 and M1 segments (white
arrowheads), consistent with vasculitis.
MRA MIP image of the left internal carotid artery demonstrates
di use irregular narrowing of the precavernous, cavernous 5. Vasculitis demonstrated on any angiographic modality
internal carotid artery as well as A1 and M1 segments (white
rom lupus is rare. Although catheter angiography
arrowheads), consistent with vasculitis.
remains the gold standard or anatomic vessel evaluation,
vasculitic changes rom SLE are rarely demonstrated.
Answers
1. This is a case o systemic lupus erythematosus.
Di erential diagnosis o the appearance o multiple Pearls
T2 hyperintense lesions a ecting the gray matter and
• Multisystem autoimmune connective tissue disease.
subcortical white matter is broad and includes ADEM,
• 75% o cases have CNS involvement.
Susac syndrome, inherited stroke disorder such as
• Most common presentation is organic encephalopathy
CADASIL, and vasculitides such as Behcet disease.
(35%-75% o case series) including acute con usion,
2. The classic presentation o SLE is a woman o child- lethargy, coma, chronic dementia, depression, or
bearing age with ever, joint pain, and malar rash. psychosis.
3. CNS involvement occurs in approximately 75% o SLE • Other clinical presentations include acute or subacute
patients. mental status changes secondary to di use cerebritis,
PRES, seizure, cranial nerve involvement, stroke,
4. MRI o neuropsychiatric SLE is o ten normal. However, peripheral neuropathy, myopathy, spinal cord
when imaging f ndings are positive, there are our involvement, and atigue.
recognized patterns: (1) ocal in arcts are seen in patients • Neuropsychiatric involvement o lupus o ten has
with increased anticardiolipin and lupus anticoagulant normal imaging.
antibodies; (2) multiple T2 hyperintensities o the white • DWI images demonstrate restricted di usion in
matter can be seen in microin arctions; (3) ocal areas ischemia or in arct and increased di usion in
o T2 hyperintensity in the gray matter— “migratory” vasogenic edema.
edematous areas; and (4) di use steroid-responsive • Vasculitis associated with lupus is rarely demonstrated
subcortical lesions associated with antineurof lament on angiography.
antibodies.

323
Lupus vasculitis (Cont.) Case 140 (3442)

Suggested Readings
Luyendijk J, Steens SC, Ouwendijk WJ, et al.
Neuropsychiatric systemic lupus erythematosus: lessons
learned rom magnetic resonance imaging. Arthritis
Rheum. 2011 Mar;63(3):722-732.
Steup-Beekman GM, Zirkzee EJ, Cohen D, et al.
Neuropsychiatric mani estations in patients with systemic
lupus erythematosus: epidemiology and radiology
pointing to an immune-mediated cause. Ann Rheum Dis.
2013 Apr;72(suppl 2):ii76-ii79.

324
87-year-old female with altered mental status

1. What is the di erential diagnosis?

2. What is the typical clinical syndrome o entity?

3. What structures are usually involved?

4. Where does the artery o Percheron arise?

5. Which modality is most sensitive or acute


in arction?

325
Top of basilar infarction Case 141 (3443)
Case ranking/dif culty: Category: Intra-axial supratentorial

Coronal CTA MIP image demonstrates intraluminal thrombus in


the distal basilar artery extending to the bilateral P1 segments
(white arrowheads).

Axial noncontrast CT image demonstrates hypodensity foci in Pearls


bilateral paramedian thalami (white arrowheads).
• Top o basilar in arct is a clinically recognizable
syndrome.
Answers • Visual, oculomotor def cits, somnolence, and
behavioral changes.
1. Di erential diagnosis o bilateral thalamic hypodensity
• Relative absence o motor def cits.
includes artery o Percheron in arct, deep venous
• Involves rostral midbrain, thalamus, and posterior
thrombosis, Wernicke encephalopathy, and top o basilar
cerebral lobes.
in arction.
• Hypodensity on CT and restricted di usion on MRI.
2. Top o basilar in arction is a recognized syndrome • CTA or MRA is help ul to evaluate extension o
with visual and oculomotor def cits, somnolence, or intraluminal thrombus.
behavioral changes. • Bilateral paramedian thalamic lesions can also
suggest artery o Percheron in arct or Wernicke
3. Structures supplied by the distal basilar artery include
encephalopathy.
the midbrain and thalami while the PCA distribution
(occipital and paramedian parietal lobes) is typically also
involved.
Suggested Reading
4. Artery o Percheron is a rare anatomical variation in
the brain in which a single arterial trunk arises rom the Matheus MG, Castillo M. Imaging o acute bilateral
posterior cerebral artery (PCA) to supply paramedian paramedian thalamic and mesencephalic in arcts. AJNR
thalami and the rostral midbrain bilaterally. Am J Neuroradiol. 2007 May;24(10):2005-2008.
5. Di usion-weighted imaging is the most sensitive or
in arct in the acute phase, lasting up to 7-10 days.

326
63-year-old female with history of left temporal brain mass

1. What is the di erential diagnosis?

2. What is the typical clinical presentation?

3. What are typical radiographic eatures?

4. What are the most common locations or this


lesion?

5. What is the treatment or this entity?


327
Giant capillary telangiectasia Case 142 (3444)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T1 image demonstrates a Axial T2 image demonstrates Axial GRE image demonstrates Axial T1 postcontrast image
focus of T1 isointensity in the a focus of T2 hyperintensity a focus of susceptibility demonstrates amorphous
right frontal subcortical white in the right frontal subcortical artifact in the right frontal contrast enhancement without
matter (white arrowhead). white matter (white arrowhead). subcortical white matter (white adjacent edema in the right
Also noted are few foci of T2 arrowhead). frontal subcortical white
hyperintensity in bilateral matter (white arrowhead).
centrum semiovale (white
arrows), consistent with gliosis
related to small vessel disease.

Answers
• Most common locations are in the pons, middle
1. In this case o capillary telangiectasia, the di erential
cerebellar peduncles, dentate nuclei, and spinal cord.
diagnosis includes AVM, cavernoma, and metastasis.
• Usually asymptomatic.
2. Most patients with capillary telangiectasia are • MRI with GRE/SWI and contrast is the most
asymptomatic and incidentally ound on neuroimaging. sensitive.
• Typical radiographic eatures include T1 normal to
3. Capillary telangiectasias are best seen on postcontrast
subtle hypointensity, T2 slight hyperintensity with
images with intense brush-like enhancement and T2*
blooming/susceptibility arti act rom blood pooling,
images with susceptibility arti act rom blood pooling.
and brush-like contrast enhancement.
T1 may be normal or slightly hypointense to white
• No mass e ect to adjacent brain parenchyma.
matter with slight increased T2 signal. There is no
• No treatment required.
surrounding edema.
4. Most common locations are pons, midbrain, cerebellum,
dentate nuclei, and spinal cord. Suggested Readings
5. No treatment or ollow-up is required. Hemorrhage El-Koussy M, Schroth G, Gralla J, et al. Susceptibility-
occurs only when associated with other vascular weighted MR imaging or diagnosis o capillary
mal ormations. telangiectasia o the brain. AJNR Am J Neuroradiol. 2012
Apr;33(4):715-720.
Huddle DC, Chaloupka JC, Sehgal V. Clinically aggressive
Pearls di use capillary telangiectasia o the brain stem: a clinical
radiologic-pathologic case study. AJNR Am J Neuroradiol.
• Area o abnormal capillaries interspersed within brain 1999 Oct;20(9):1674-1677.
parenchyma. Lee RR, Becher MW, Benson ML, Rigamonti D. Brain
• Second most common vascular mal ormation a ter capillary telangiectasia: MR imaging appearance
DVA (developmental venous anomaly). and clinicohistopathologic f ndings. Radiology. 1997
• Can be single or multiple lesions and may Dec;205(3):797-805.
demonstrate areas o microhemorrhage i associated
with DVA and/or cavernous mal ormation.

328
36-year-old female with intractable headaches

1. What is the di erential diagnosis?

2. What is the typical clinical prof le in this


disease?

3. What are commonly involved brain structures?

4. What is a sensitive and specif c sign or this


disease?

5. What is the prognosis o this disease?

329
CADASIL Case 143 (3505)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial T2 image shows O -midline sagittal FLAIR image Axial FLAIR image shows Axial T2 image through the
multiple hyperintense lesions shows multiple periventricular bilateral subcortical white basal ganglia also shows focal
in the centrum semiovale and subcortical hyperintensities matter hyperintensity of and patchy T2 hyperintensity
(arrowheads). (arrowheads). the anterior temporal lobes in the deep gray nuclei
(arrows), a sensitive and speci c (arrowheads).
sign of CADASIL.

Answers
1. Patchy periventricular white matter areas o gliosis are Pearls
typical o small vessel vasculopathy, with subcortical • Cerebral autosomal dominant arteriopathy with
arteriosclerosis rom hypertension as the most common subcortical in arcts and leukoencephalopathy
cause. CNS vasculitis and CADASIL are rare causes o (CADASIL) is a hereditary disease o small vessels
this imaging f nding. with mutation o NOTCH3.
• Transient ischemic attacks, stroke-like symptoms,
2. The typical clinical prof le is a young adult presenting
and migraines are the typical initial presentation
with TIA, stroke-like symptoms, or migraines o ten
with progression to neurologic disability, including
without the traditional risk actors or stroke.
cognitive def cits and behavioral disturbances.
3. Involved areas o stroke in CADASIL are similar • Patients may have absence o traditional stroke risk
to those a ected by hypertension with small vessel actors and present at a younger age.
distribution, including periventricular and subcortical • Distribution o lacunar in arcts is similar to
white matter, basal ganglia, and capsular white matter. subcortical arteriosclerotic encephalopathy seen with
The cerebral cortex is usually spared. hypertension.
• Involvement o the anterior temporal subcortical
4. A sensitive and specif c sign or CADASIL is T2
white matter is a highly sensitive and specif c sign
hyperintense involvement o the bilateral anterior
o CADASIL not typically seen in hypertensive
temporal subcortical white matter, not commonly seen in
arteriosclerosis.
small vessel disease rom hypertension.
5. No specif c treatment exists or CADASIL. Most patient
progress to cognitive decline and behavioral changes, Suggested Readings
with men demonstrating a shorter li espan.
De Guio F, Reyes S, Duering M, Pirpamer L, Chabriat H,
Jouvent E. Decreased T1 contrast between gray matter
and normal-appearing white matter in CADASIL. AJNR
Am J Neuroradiol. 2014 Jan;35(1):72-76.
Liem MK, Lesnik Oberstein SA, Haan J, et al. Cerebrovascular
reactivity is a main determinant o white matter
hyperintensity progression in CADASIL. AJNR Am J
Neuroradiol. 2009 Jun;30(6):1244-1247.

330
9-year-old male with acute weakness

1. What is the di erential diagnosis?

2. What are associated vasculopathies with this


disease?

3. What abnormality can be seen on intracranial


arteriography with this disease?

4. What therapy induces etal hemoglobin as a


treatment or this disease?

5. Where is the typical location o cerebral


in arcts on imaging in this disease?
331
Sickle cell disease Case 144 (3509)
Case ranking/dif culty: Category: Intra-axial supratentorial

Axial FLAIR image shows Axial DWI b = 1000 image shows Axial DWI b = 1000 image shows Coronal postcontrast with
punctate subcortical white bright punctate signal of the left bilateral frontal subcortical fat-sat image shows calvarial
matter hyperintensities of the frontal subcortical white matter white matter areas of di usion thickening and expansion of
left frontal lobe (arrowheads). (arrowheads) from restricted restriction (arrowheads) from the diploic space from marrow
di usion from acute infarct. acute infarct. expansion (arrows).

Answers
1. The di erential o small punctate or patchy white Pearls
matter in arcts includes CADASIL, CNS vasculitis, • Sickle cell disease is an autosomal recessive disease
hypertensive arteriosclerosis, and sickle cell disease. with mutation o the hemoglobin beta gene on
O this list, only sickle cell disease causes red marrow chromosome 11p15.5.
expansion o the calvarium. • The sickling o deoxygenated RBCs causes clumping
and vascular occlusion o capillaries as well as
2. Vasculopathies associated with sickle cell disease
larger vessel vasculopathy rom adherence to the
includes vaso-occlusive crises rom red cell sickling,
endothelium.
large vessel vasculopathy and aneurysm, and moyamoya.
• Patients typically present in childhood with stroke and
3. Distal internal carotid stenosis with lenticulostriate is the primary cause o stroke in A rican American
collaterals in a moyamoya pattern can be seen with children.
sickle cell disease. Arterial aneurysms, venous • MR brain imaging may show predominantly small
thrombosis, and arterial dissection have also been vessel distribution in arcts: ACA, MCA watershed
described. distribution including the parasagittal white matter.
• Angiography can show associated moyamoya
4. Hydroxyurea induces etal hemoglobin and lessens the
vasculopathy with severe stenosis o the distal
number o pain ul vaso-occlusive crises in sickle cell
internal carotid artery with prominent lenticulostriate
disease.
collaterals.
5. Watershed locations and small vessel locations are • Expanded diploic space o the calvarium is typical
most common such as the parasagittal white matter and rom red marrow hyperplasia rom anemia.
between the ACA and MCA distributions.

Suggested Readings
Sun B, Brown RC, Hayes L, et al. White matter damage in
asymptomatic patients with sickle cell anemia: screening