AJR Integrative Imaging
LIFELONG LEARNING
Nontraumatic Emergent Neuroradiology:
Review and Self-Assessment Module
Yoshimi Anzai1 and Basavaraj Ghodke2
Abstract
Objective
The educational objectives for this case-based self-assess-
ment module on imaging of nontraumatic emergent neuro-
radiology are to use case examples to teach the imaging
features of CT and MRI of emergent neuroradiologic diag-
noses and to improve the reader’s understanding of the
pathophysiology and clinical management of each clinical
scenario. Imaging plays a critical role in assessing patients
with acute neurologic symptoms. CT is quick and easy to
perform in an emergency setting and is often the technique
of choice for any neurologic emergency situations. Clinical
correlation is crucial for accurate diagnosis and triaging pa-
tients for further evaluation. Persistent or progressive neu-
rologic symptoms despite negative CT should prompt other
imaging studies.
Conclusion
At the end of this self-assessment module, readers will be
able to generate a concise list of differential diagnoses for
imaging findings that often are encountered in patients with
nontraumatic neurologic emergency.
INTRODUCTION
This self-assessment module on nontraumatic emergent
neuroradiology diagnoses has an educational component
and a self-assessment component. The educational compo-
nent consists of six case scenarios that the participant
should work through and four recommended articles that
may provide additional information and perspective. The
self-assessment component consists of 19 multiple-choice
questions with solutions. All of these materials are avail-
able on the ARRS Website (www.arrs.org). To claim CME
and SAM credit, each participant must enter his or her re-
sponses to the questions online.
Keywords: brain imaging, case-based learning, emergency, neuroradiology, self-assessment
DOI:10.2214/AJR.07.7042
Received September 7, 2007; accepted after revision January 30, 2008.
1
Department of Radiology, Division of Neuroradiology, University of Washington, 1959 NE Pacific St., NW 011, Box 357115, Seattle, WA 98195-7115. Address
correspondence to Y. Anzai (anzai@u.washington.edu).
2
Department of Radiology, Harborview Medical Center, Seattle, WA.
AJR 2008;191:S1–S17 0361–803X/08/1913–S1 © American Roentgen Ray Society
AJR:191, September 2008 S1
FOR RADIOLOGY
EDUCATIONAL OBJECTIVES
By completing this educational activity, the participant will:
A. Exercise, self-assess, and improve his or her understand-
ing of the imaging features of nontraumatic emergent
neuroradiology diagnoses.
B. Exercise, self-assess, and improve his or her understand-
ing of the clinical features of nontraumatic emergent
neuroradiology diagnoses.
RECOMMENDED READING
1. Provenzale JM. Centennial dissertation. Honoring Ar-
thur W. Goodspeed, MD and James B. Bullitt, MD. CT
and MR imaging and nontraumatic neurologic emergen-
cies. AJR 2000; 174:289–299
2. Wintermark M, Albers GW, Alexandrov AV, et al. Acute
stroke imaging research roadmap. Stroke 2008; 39:
1621–1628
3. Beauchamp NJ Jr, Bryan RN. Acute cerebral ishemic
infarction: a pathophysiologic review and radiologic per-
spective. AJR 1998; 171:73–84
4. Zuccoli G, Gallucci M, Capellades J, et al. Wernicke en-
cephalopathy: MR findings at clinical presentation in
twenty-six alcoholic and nonalcoholic patients. AJNR
2007; 28:1328–1331
INSTRUCTIONS
1. Complete the educational and self-assessment components.
2. Visit www.arrs.org and select Publications/Journals/
SAM Articles from the left-hand menu bar.
3. Using your member login, order the online SAM as di-
rected.
4. Follow the online instructions for entering your respons-
es to the self-assessment questions and complete the test
by answering the questions online.
 Anzai and Ghodke

Scenario 1 angiography did not reveal any cerebral aneurysms, arte-

Clinical History riovenous malformation, or enhancing focus as possible
A 32-year-old man presented to the emergency depart- causes of a hematoma. Cerebral angiography showed ir-
ment complaining of headache and general fatigue. He was regularity of small- to medium-sized vessels and a beaded
diagnosed with a viral syndrome and discharged with symp- appearance, consistent with vasculitis. The patient admit-
tomatic treatment. Two weeks later he presented to the ted that he smoked marijuana almost daily and had a long
emergency department with severe headache and confusion. history of amphetamine use.
He was afebrile but his mental status had changed since his
previous emergency department visit. A working diagnosis
of bacterial meningitis was considered. The patient denied QUESTION 2
any focal weakness or sensory or visual changes.
Unenhanced CT (Fig. 1A) and CT angiography (Fig. 1B) Which one of the following is NOT associated
of the head were performed. The patient also underwent with vasculitis?
cerebral angiography (Figs. 1C and 1D). A. Polyarteritis nodosa.
B. Tuberculosis.
Description of Images C. Systemic lupus erythematosus.
Unenhanced CT of the head showed a large right frontal
D. Drugs (amphetamine, cocaine).
intraparenchymal hematoma with surrounding edema,
E. Marfan syndrome.
likely representing a subacute intracranial hematoma. CT

QUESTION 3
QUESTION 1 What is the most definitive test for diagnosing
Which of the following is the preferred diagnosis? CNS vasculitis?
A. Arteriovenous malformation. A. CT angiography with 3D volume rendering.
B. Hypertensive hemorrhage. B. Cerebral angiography.
C. Hemorrhagic tumor or metastasis. C. Biopsy.
D. Vasculitis. D. MR angiography.
E. Amyloid angiopathy. E. Transcranial Doppler sonography.

A B D
Fig. 1—32-year-old man with headache and general fatigue who was diagnosed with viral syndrome and discharged but returned with severe headache and confusion.
A and B, Unenhanced CT (A) and CT angiography (B) of head were performed. Unenhanced CT shows large right frontal intraparenchymal hematoma and surrounding
edema (arrow, A), likely representing subacute intracranial hematoma. CT angiography does not reveal any cerebral aneurysms, arteriovenous malformation, or en-
hancing focus as possible cause of hematoma.
C and D, Cerebral angiography shows irregularity of small- to medium-sized vessels (arrows, D) and beaded appearance, consistent with vasculitis. Patient admitted
that he smokes marijuana almost daily and has long history of amphetamine use.

S2 AJR:191, September 2008

 Nontraumatic Emergent Neuroradiology
Solution to Question 1
Arteriovenous malformation (AVM) is not an unusual cause
of intracranial hematoma for the young patient in this case.
Patients with AVM often present with acute intracranial hem-
orrhage or ischemic symptoms due to steal phenomenon. CT
angiography often reveals a tangle of markedly dilated arter-
ies and veins with a nidus of AVM. If the AVM is small, it may
not be visualized or diagnosed correctly with CT angiography.
In this setting, cerebral angio­graphy is the most definitive test.
However, cerebral angio­graphy in this patient did not show
early venous filling or an abnormal tangle of blood vessels to
indicate AVM. Therefore, option A is not the best response.
Acute intracranial hematoma can have a number of causes.
The most common cause is hypertensive hemorrhage. Hy-
pertensive hemorrhages, which originate from terminal small
vessels, are often centered at the basal ganglia, particularly
the putamen and the external capsule; the thalamus; the
pons; and occasionally, the cerebellum. Lobar hemorrhage
can be seen in a setting of hypertension (≈ 1–2%), but it is
rare. Hypertensive hemorrhage is much more common in
older patients. Option B is not the correct response.
Certain types of brain tumors are often associated with
intracranial hemorrhage. These are often aggressive tumors,
such as glioblastoma multiforme, or vascular tumors, such as
metastases from renal cell carcinoma or melanoma, but rare-
ly papillary thyroid cancer and choriocarcinoma. Lung can-
cer and breast cancer are not considered vascular; however,
the incidence of brain metastasis from lung and breast can-
cers is exceedingly higher than that of other vascular can-
cers. Thus, when all hemorrhagic brain metastases are re-
viewed, lung and breast remain the two top primary choices.
Intracranial hemorrhage associated with brain tumor often
has a focal area of enhancement and significant mass effect
or vasogenic edema. Option C is not the best response.
Vasculitis is one of the causes of intracranial hemorrhage
and is more frequently seen in young patients. Vasculitis can
present with acute hemorrhage or ischemic symptoms. Many
causes of vasculitis affect small- to medium-sized vessels;
therefore, CT angiography may not show an irregularity of
the blood vessels as definitively as cerebral angiography. Cere-
bral angiography in Figures 1C and 1D shows a classic ap-
pearance of segmental narrowing and poststenotic dilatation
involving multiple small- to medium-sized blood vessels, con-
sistent with vasculitis. Option D is the best response.
Amyloid angiopathy often causes a lobar intracranial
hemorrhage centered at the deep white matter in the frontal
or parietal lobes. Amyloid angiopathy is much more common
in elderly patients and is rare in young patients. Cerebral an-
giography may not show any irregularity or narrowing. Am-
yloid angiopathy is often diagnosed after excluding other
causes in elderly patients. Option E is not the best response.
Solution to Question 2
The causes of vasculitis are often divided into infectious
and noninfectious. Infectious causes include tuberculosis, fun-
AJR:191, September 2008 S3
gal infection, bacterial vasculitis, and HIV vasculitis. The fre-
quent causes of noninfectious vasculitis are immune-mediated
vasculitis such as rheumatoid arthritis and systemic lupus er-
ythematosus, and granulomatous disease, such as Wegener’s
granulomatosis and sarcoid, as well as giant cell arteritis (also
called temporal arteritis). Drugs, particularly amphetamines
and cocaine, are frequent causes of noninfectious vasculitis.
An amphetamine causes inflammatory vasculitis with vascu-
lar wall necrosis and subsequent hemorrhage. The pathologic
features of amphetamine-related vasculitis are similar to
those of polyarteritis nodosa. This patient had taken amphet-
amines for several years. Cocaine, on the other hand, induces
cerebral infarction or ischemia as well as hemorrhage by vaso-
constrictive effect and increased platelet aggregation, rather
than a vasculitis-type inflammation of the vessels [1]. Options
A, B, C, and D are not the best responses.
Marfan syndrome is an autosomal dominant disorder of
the connective tissue characterized by disproportionally
long limbs and tall stature. It affects the heart and aorta
and causes aortic root dilatation, aortic regurgitation, and
dissection. However, involvement of CNS vessels is unusual.
Option E is the best response.
Solution to Question 3
Because many cases of vasculitis affect small to medium-
sized blood vessels, MR angiography is relatively insensitive
for the diagnosis of CNS vasculitis. CT angiography is less
accurate than cerebral angiography. Cerebral angiography is
used as the gold standard for diagnosis at many institutions.
When characteristic angiographic findings such as alternat-
ing areas of stenosis and dilatation or a beading appearance
are observed in multiple vessels and multiple vascular beds,
cerebral angiography is diagnostic. However, angiography
can be normal in up to 40% of biopsy-proven cases. Thus,
negative cerebral angiography does not completely exclude
the diagnosis. Moreover, its specificity is not perfect. Intra-
cranial atherosclerotic disease may show irregularity of mul-
tiple vessels, mimicking CNS vasculitis.
Transcranial Doppler sonography is used to characterize
the morphology of the superficial temporal artery as a
screening tool for temporal arteritis. However, transcranial
Doppler sonography is not suitable to evaluate arteries fully
to diagnose CNS vasculitis. Options A, B, D, and E are not
the best responses.
Biopsy of CNS tissue would logically be considered the
ultimate gold standard of diagnosis, but clearly the proce-
dure is limited by several factors. It is highly invasive and
carries certain risks. Successful biopsy requires a willing
and experienced neurosurgeon, who may not be readily
available. Sampling error could result in limited sensitivity.
The site of biopsy should be tailored to the individual pa-
tient [2]. The biopsy of the superficial temporal artery is
often performed in patients suspected of having giant cell
vasculitis. The false-negative rate of biopsy for a diagnosis
of vasculitis has been reported to be 16%, yielding a sensi-
 Anzai and Ghodke
tivity of 84% [2]. Therefore, the most definitive test is bi-
opsy of the blood vessels. Option C is the best response.
Conclusion
Vasculitis involving the CNS presents diagnostic chal-
lenges to clinicians as well as radiologists. There are many
causes, including infection, autoimmune disease, drug ex-
Scenario 2
Clinical History
A 19-year-old woman presented to an outside hospital with
severe headache, nausea, vomiting, and photophobia. She had
no significant medical history. Laboratory examinations were
unremarkable. Her initial head CT scans (unenhanced and
contrast-enhanced) are shown in Figures 2A and 2B.
Description of Images
Unenhanced CT of the head showed a focal area of low
attenuation in the left temporal lobe and mild regional mass
effect. The ventricles were normal in size with no shift of
midline structures. No parenchymal hemorrhage was evi-
dent. The contrast-enhanced image showed the empty delta
QUESTION 4
What is your preferred diagnosis after the
initial CT scans (Figs. 2A and 2B)?
A. Acute infarction of the left middle cerebral artery
(MCA).
B. Herpes encephalitis.
C. Underlying tumor.
D. Sinus thrombosis.
A B
S4 AJR:191, September 2008
posure, radiation, and malignancies. Cerebral angiography
is more sensitive than noninvasive vascular imaging (CT an-
giography or MR angiography), but it is invasive and has
limited sensitivity and specificity. Treatment of noninfec-
tious vasculitis is long-term, high-dose immunosuppression,
which causes profound morbidity as a result of opportunis-
tic infection or organ failures.
sign (Fig. 2B). Brain MRI, including sagittal T1-weighted
imaging (Fig. 2C), diffusion-weighted imaging (Fig. 2D),
and apparent diffusion coefficient (ADC) mapping (Fig. 2E)
were also performed at the outside hospital. The sagittal
T1-weighted image showed an isointense clot along the su-
perior sagittal sinus that was of concern for superior sagit-
tal sinus thrombosis. The diffusion-weighted image showed
no areas of restricted diffusion or ADC map abnormality.
The patient’s mental status deteriorated at the outside hos-
pital and she was transferred to the emergency department
of our hospital, at which time she was unresponsive and
obtunded, and her pupils were dilated. CT of the head was
QUESTION 5
Which one of the following techniques is
LEAST appropriate to confirm a diagnosis of
venous sinus thrombosis?
A. Rapid contrast-enhanced gradient-echo MRI with
contrast-enhanced multiplanar imaging.
B. MDCT angiography or venography.
C. Brain MRI and phase-contrast MR venography.
D. Cerebral angiography.
E. Brain MRI with time-of-flight MR venography.
Fig. 2—19-year-old woman who presented to an-
other hospital with severe headache, nausea, vomit-
ing, and photophobia.
A, Unenhanced CT scan of head obtained at outside
hospital shows focal area of low attenuation in left
temporal lobe and mild regional mass effect. Ventri-
cles are normal in size with no shift of midline struc-
tures. No parenchymal hemorrhage is evident.
B, Contrast-enhanced image from other hospital shows
empty delta sign (arrows).
(Fig. 2 continues on next page)
 Nontraumatic Emergent Neuroradiology

obtained immediately after her admission (Figs. 2F and 2G)
and showed marked worsening of the cerebral edema and
diffuse effacement of the cortical sulci and basilar cistern.
In addition, a new focus of venous infarction and parenchy-
mal hemorrhage was present in the left frontal lobe.
Solution to Question 4
In the inferior aspect of the superior sagittal sinus on ini-
tial head CT was an area of high attenuation with a convex
border that was of concern for superior sagittal sinus throm-
bosis. Contrast-enhanced head CT showed lack of contrast
enhancement in the superior sagittal sinus, the empty delta
sign. Findings were consistent with venous sinus thrombo-
sis and venous infarction involving the left temporal lobe.
The patient had been taking oral contraceptives for the pre-
vious 3 months.
CT findings of middle cerebral artery (MCA) infarction
are loss of gray and white matter differentiation or presence
of an acute clot in the expected location of the proximal
MCA (dense MCA sign). In this case, an area of low attenu-
ation predominantly involved the white matter and spared
the gray matter. In addition, diffusion-weighted imaging
and the ADC map showed no area of restricted diffusion.
Option A is not the best response.
QUESTION 6
Which one of the following is NOT associated
with venous sinus thrombosis?
A. Sickle cell disease.
B. Oral contraceptives.
C. Cancer.
D. Disseminated intravascular coagulation.
E. Dehydration.

C D E

Fig. 2 (continued)—19-year-old woman who pre-

sented to another hospital with severe headache,
nausea, vomiting, and photophobia.
C–E, Brain MR images, including sagittal T1-weight-
ed (C), diffusion-weighted (D), and apparent diffu-
sion coefficient (ADC) map (E) images obtained at
outside hospital. T1-weighted image shows hyperin-
tense clot along superior sagittal sinus (arrows),
causing concern for superior sagittal sinus thrombo-
sis. Diffusion-weighted images show no areas of
restricted diffusion or ADC map abnormality.
F and G, CT scans of head obtained immediately af-
ter admission to our hospital show marked worsen-
ing of cerebral edema and diffuse effacement of
cortical sulci and basilar cistern. In addition, new
focus of venous infarction and parenchymal hemor-
rhage (arrow, F) is present in left frontal lobe.
F G

AJR:191, September 2008 S5

 Anzai and Ghodke
Herpes encephalitis typically involves the medial tempo-
ral lobe and is often associated with parenchymal hemor-
rhage. This was the diagnosis made at the outside hospital,
and the patient was treated with acyclovir. However, herpes
encephalitis does not explain the presence of a clot in the
superior sagittal sinus. Option B is not the best response.
The presence of tumor with vasogenic edema is also a
possibility based on the area of low attenuation in the left
temporal lobe. However, this does not explain the presence
of a clot in the superior sagittal sinus. Option C is not the
best response.
The correct diagnosis is venous sinus thrombosis and ve-
nous infarction involving the left temporal lobe. Option D is
the best response. The venous sinus can show slightly high
attenuation, particularly among athletes, smokers, or neo-
nates. The lateral border of the venous sinus, in these normal
settings, should be concave, not convex toward the brain pa-
renchyma. After the administration of contrast material, the
dura may enhance but the venous sinus does not, giving the
empty delta sign. Venous sinus thrombosis can be a challeng-
ing diagnosis to make unless one has a high clinical suspicion.
Patients present with nonspecific headache or confusion, and
a clinical history of oral contraceptive use is often not avail-
able to radiologists at the time of interpretation.
Sagittal T1-weighted MR images at the outside hospital
showed lack of flow void along the superior sagittal sinus,
highly suggestive of the diagnosis. Please note that diffu-
sion-weighted images and the ADC map did not necessar-
ily show an area of restrictive diffusion in the setting of
venous infarction because increased venous pressure ini-
tially causes vasogenic edema. This is distinctly different
from arterial infarction, when the arterial supply to an
area of the brain is abruptly terminated, resulting in cyto-
toxic edema. Lack of diffusion signal change should not
exclude venous infarction. However, venous infarction
does progress to cytotoxic edema with restricted diffusion
if the disease remains untreated.
Solution to Question 5
The next diagnostic test to confirm venous sinus throm-
bosis is brain MRI with MR venography. On conventional
MRI, venous sinus thrombosis may be suspected from lack
of a flow void or high-signal thrombus in the dural sinuses.
Lack of flow void is best appreciated on FLAIR or T2-
weighted spin-echo images. Parenchymal changes, such as
venous infarction or hemorrhage, along with lack of a flow
void on conventional MRI raises the suspicion of venous
sinus thrombosis. Superacute thrombus is relatively iso-
intense on T1- and hypointense on T2-weighted images be-
cause of deoxyhemoglobin potentially mimicking slow ve-
nous flow on conventional MR images and thus requiring
MR venography to confirm the diagnosis of venous sinus
S6 AJR:191, September 2008
thrombosis [3]. A subacute blood clot in the venous sinus is
often seen as hyperintense on T1-weighted images as a re-
sult of methemoglobin mimicking flowing blood on time-of-
flight (TOF) MR venography. Phase-contrast MR venogra-
phy is a fairly reliable test for the diagnosis of subacute
venous sinus thrombosis.
Recently, rapid contrast-enhanced gradient-echo imag-
ing has been reported to yield a higher diagnostic accuracy
than 2D TOF MR venography [4, 5]. With advances in
MDCT technology, CT angiography and venography have
been increasingly used to diagnose venous sinus thrombo-
sis and are considered a quick and reliable alternative to
MR venography. Options A, B, C, and E, the appropriate
next tests to confirm venous sinus thrombosis, are not the
best responses.
Cerebral angiography also shows lack of flow in the dural
venous sinuses. However, cerebral angiography is an inva-
sive test that is associated with complication rates of 1–2%.
Noninvasive imaging should be considered first to confirm
the diagnosis of venous sinus thrombosis. Option D, which
is the least appropriate technique, is the best response.
Solution to Question 6
Common medical conditions associated with venous si-
nus thrombosis are pregnancy, a postpartum state, and
hypercoagulable states such as sickle cell disease, oral con-
traceptives use, and cancer. Dehydration often seen in neo-
nates and elderly patients is associated with venous sinus
thrombosis as a result of low-flow circulatory states. Ex-
trinsic compression (tumor) or adjacent infection (mas-
toiditis) is also a risk factor. Options A, B, C, and E are not
the best responses.
Disseminated intravascular coagulation, however, is a
hypocoagulable state and is not associated with venous si-
nus thrombosis. Option D is the best response.
Venous sinus thrombosis is an underdiagnosed condition
and can be a life-threatening disease if left untreated. Delay
in diagnosis often leads to rapid deterioration and poor clin-
ical outcomes. Intracranial hemorrhage is associated with
20–50% of cases of venous sinus thrombosis. Treatment for
venous sinus thrombosis is immediate IV anticoagulation
with low-molecular-weight heparin, despite the risk of
hemorrhagic complications [6].
Conclusion
Venous sinus thrombosis is an underdiagnosed condition
that can potentially lead to adverse clinical sequelae. Find-
ings on contrast-enhanced CT or conventional T1-weighted
sagittal images should make one suspicious in the appropri-
ate clinical setting. Understanding the risk factors and med-
ical history of patients will help to guide further diagnostic
testing and immediate treatment.
 Nontraumatic Emergent Neuroradiology

Scenario 3 veloping hydrocephalus. Focal hematoma in the medial

Clinical History
A 48-year-old woman presented to the emergency de-
partment with “the worst headache of my life” and a de-
clining level of consciousness. No known medical history or
medication use was noted. Unenhanced CT of head was
first performed.
Description of Images
Unenhanced head CT (Figs. 3A and 3B) showed diffuse
subarachnoid hemorrhage in the basilar cistern and sylvian
fissures bilaterally and along the anterior falx, associated
with intraventricular hemorrhage. The temporal horns of
the lateral ventricle were mildly dilated, suggestive of de-
frontal region was also evident. The patient underwent CT
angiography of head for further evaluation. Thin-slice
maximum-intensity-projection (Fig. 3C) and 3D volume-
rendering (Fig. 3D) images showed a lobulated saccular an-
eurysm arising from the anterior communicating artery
and measuring approximately 5 mm.
The proximal A2 segments of the anterior cerebral arter-
ies were displaced laterally, likely due to the presence of he-
matoma surrounding the aneurysm. Three-dimensional ro-
tational angiography (Fig. 3E) showed detailed anatomy
and morphology of the anterior communicating aneurysm
and the adjacent ophthalmic artery and perforating arter-
ies, which are not easily visualized on CT angiography.

A B C

Fig. 3—48-year-old woman with “the worst head-

ache of my life” and declining level of consciousness.
A and B, Unenhanced CT scans show diffuse sub-
arachnoid hemorrhage in basilar cistern and sylvian
fissures bilaterally and along anterior falx, associ-
ated with intraventricular hemorrhage. Temporal
horns of lateral ventricle are mildly dilated, sugges-
tive of developing hydrocephalus. Focal hematoma
(arrow) in medial frontal region is also evident.
C and D, Thin-slice maximum-intensity-projection
(C) and 3D volume-rendering (D) images show lobu-
lated saccular aneurysm (arrow) arising from ante-
rior communicating artery and measuring approxi-
mately 5 mm. Proximal A2 segments (arrowhead, D)
of anterior cerebral arteries are displaced laterally,
likely due to surrounding aneurysm.
E, Three-dimensional rotational angiography shows
detailed anatomy and morphology of anterior com-
municating aneurysm, adjacent ophthalmic artery,
and perforating arteries (arrow), which are not eas-
ily visualized on CT angiography.
D E

AJR:191, September 2008 S7

 Anzai and Ghodke
QUESTION 7
What is the most likely cause of the findings
on unenhanced CT?
A. Arteriovenous malformation rupture.
B. Venous sinus thrombosis.
C. Cerebral aneurysm rupture.
D. Amyloid angiopathy.
E. Hypertensive hemorrhage.
QUESTION 8
What is the 30-day mortality rate of
subarachnoid hemorrhage secondary to
aneurysm?
A. 15%.
B. 30%.
C. 45%.
D. 65%.
E. 80%.
QUESTION 9
Which of the following factors does NOT
influence management decisions for a
ruptured aneurysm?
A. Age.
B. Sex.
C. Aneurysm size.
D. Aneurysm location.
E. History of hypertension.
QUESTION 10
Despite CT angiography showing an aneurysm
causing subarachnoid hemorrhage, cerebral
angiography is still performed at some
institutions. Which one of the following is
NOT a rationale for performing cerebral
angiography in this setting?
A. Searching for an additional incidental aneurysm that
could be treated at the same time.
B. Better assessing the degree of incorporation of the
aneurysm wall into the parent vessel.
C. Assessing flow dynamics—that is, the side of the
internal carotid artery feeding anterior
communicating aneurysm.
D. Measuring the aneurysm neck–dome ratio.
E. Suspecting mycotic aneurysm in patients with IV
drug use.
S8 AJR:191, September 2008
Solution to Question 7
Arteriovenous malformation (AVM) is a congenital anom-
aly of blood vessels with one or more large feeding arteries
and drainage veins. AVM often presents not only with sub-
arachnoid hemorrhage but also with parenchymal hemor-
rhage or symptoms of ischemia due to steal phenomenon.
Blood flow preferentially goes to AVMs with low resistance,
leaving the adjacent normal brain relatively hypoperfused.
CT may show a hyperdense mass or curvilinear or speckled
calcification adjacent to a parenchymal hematoma. MRI
may show numerous foci of flow voids and venous aneurys-
mal dilatation. Option A is not the best response.
Venous sinus thrombosis could present with subarachnoid
hemorrhage; however, it tends to be focal to the area of ve-
nous infarction or hypertension. Venous sinus thrombosis
does not normally present diffuse subarachnoid hemor-
rhage. Option B is not the best response.
Unenhanced CT of the head shows diffuse subarachnoid
hemorrhage as well as a focal hematoma along the anterior
falx, a typical location for a ruptured anterior communicating
artery aneurysm. Option C is the best response. Approximately
50–70% of subarachnoid hemorrhages are due to aneurysm
rupture. Of patients with subarachnoid hemorrhage, 10–15%
have no aneurysm found even on cerebral angiography.
Amyloid angiopathy commonly affects elderly patients
and presents with a lobar rather than a subarachnoid hemor-
rhage. Option D is not the best response. Hypertensive hem-
orrhage often affects the small perforating vessels along the
lenticulostriate or thalamoperforating vessels and thus is of-
ten present with parenchymal hemorrhage in the basal gan-
glia, thalamus, and pons. Option E is not the best response.
Solution to Question 8
Aneurysmal subarachnoid hemorrhage (SAH) has a
30-day mortality rate of 45%, with approximately half of
the survivors sustaining irreversible brain damage [7]. Option
C is the best response. The annual incidence of aneurysmal
SAH is six per 100,000 in the United States. Approximately
5–10% of stroke cases are secondary to ruptured saccular
aneurysms [8]. Recurrent hemorrhage remains a serious con-
sequence, with a 70% fatality rate in patients who rebleed.
Solution to Question 9
Treatment options for a ruptured aneurysm are surgical
resection or endovascular coil embolization. Endovascular oc-
clusion of aneurysms using electrolytically detachable Gug-
lielmi detachable coil system (GDC, Target Therapeutics [now
Boston Scientific]) has been used to treat ruptured or unrup-
tured aneurysms in a large number of patients worldwide.
Published reports suggest that the endovascular technique is
associated with fewer treatment-related complications than
open surgery [7]. However, the long-term efficacy in the pre-
vention of rupture or recurrence of aneurysm remains in-
determinate. The recent International Subarachnoid Aneu-
 Nontraumatic Emergent Neuroradiology
rysm Trial (ISAT) showed that retreatment was performed
in 17.4% of patients treated with endovascular coiling and
in 3.8% of patients after surgical clipping [9]. Younger age
and larger aneurysm size were risk factors for retreatment.
A higher rate of recurrence is seen in posterior communicat-
ing artery aneurysms after endovascular coiling and anterior
communicating artery aneurysms after surgical clipping,
which might reflect the technical difficulty. Hyper­tension is
also associated with an increasing rate of rupture in patients
with an unruptured aneurysm. Thus age, medical history
such as hypertension, aneurysm location, and size are all rel-
evant factors in treatment decisions and, therefore, options
A, C, D, and E, all relevant factors in treatment decisions,
are not the best responses. Although aneurysms are more
common in women than in men, sex is not a factor affecting
management decisions or predicting outcomes. Option B is
the best response.
Solution to Question 10
CT angiography is a noninvasive vascular imaging tech-
nique that has replaced catheter angiography in some insti-
tutions. CT angiography may show aneurysms larger than 3
mm with a sensitivity of 77–97% and specificity of 87–100%
[10]. CT angiography also has been used as a screening tool in
populations at high risk for cerebral aneurysms. Cerebral an-
giography, however, still remains the gold standard in the di-
agnostic evaluation of cerebral aneurysms. In particular, 3D
rotational angiography shows the most information about
Scenario 4
Clinical History
A 65-year-old woman presented to the emergency de-
partment 4 hours after the onset of right-sided weakness
and dysarthria. Unenhanced CT of the head was obtained.
Description of Images
Unenhanced CT of the head (Figs. 4A and 4B) showed
loss of gray–white matter differentiation in the left insular
cortex and the temporal lobe. The left middle cerebral ar-
tery was hyperdense compared with the basilar artery.
The patient underwent cerebral angiography for poten-
tial endovascular intervention. A left internal carotid artery
injection image (Fig. 4C) showed the presence of a clot in
the right internal carotid bifurcation and no flow visible in
the anterior and middle cerebral arteries. The microcathe-
ter was placed proximal to the clot before advancing the
Merci retrieval device (Merci Retrieval System, Concentric
Medical, Inc.) (Fig. 4D). Subsequent angiography showed
persistent occlusion of the MCA branches (Fig. 4E). In-
traarterial tissue plasminogen activator (tPA) was infused
through the left MCA; after tPA infusion, angiography (Fig.
4F) showed opening of the anterior division of the left MCA
and occlusion of the posterior division of the left MCA.
AJR:191, September 2008 S9
small perforating vessels, the relationship of the aneurysm to
the parent vessels (how much of the aneurysm wall is incor-
porated into the parent vessel), and the flow dynamics of an-
eurysms that affect surgical planning. Multiple aneurysms
can be seen in patients with subarachnoid hemorrhage. These
incidental aneurysms are often smaller than 3 mm, which CT
angiography has a limited ability to detect. Small unrup-
tured aneurysms are associated with a risk of future SAHs.
If they are in a surgically or endovascularly accessible loca-
tion, these incidental aneurysms can be treated at the same
time as the ruptured primary aneurysm. Mycotic aneurysms
often involve peripheral vessels and may present with sub-
arachnoid or parenchymal hemorrhage or septic emboli. Be-
cause mycotic aneurysms involve distal vessels, cerebral angi-
ography is a more definitive test than CT angiography.
Options A, B, C, and E are not the best responses. The aneu-
rysm neck–dome ratio can be calculated on the basis of CT
angiography. Option D is the best response.
Conclusion
SAH associated with aneurysm rupture has high rates of
mortality and morbidity. Accurate detection and assess-
ment of cerebral aneurysm lead to proper treatment deci-
sions by either surgical clipping or endovascular coiling.
Although CT angiography has rapidly replaced cerebral an-
giography in some institutions, 3D rotational angiography
provides the most information regarding characterization
of aneurysms in relation to parent or adjacent vessels.
QUESTION 11
Assuming the patient does not have any
medical conditions, what would be the most
appropriate urgent therapy at this point?
A. IV recombinant tissue plasminogen activator (tPA).
B. IV streptokinase.
C. IV heparin.
D. Antiplatelet therapy and aspirin.
E. Intraarterial tPA and mechanical clot removal.
QUESTION 12
What percentage of patients with acute
ischemic stroke are treated with IV tPA?
A. Less than 6%.
B. 8–15%.
C. 20–25%.
D. 25–50%.
E. 50–60%.
 Anzai and Ghodke

Solution to Question 11
QUESTION 13 Current therapy for acute stroke is limited to IV recombi-
nant tPA administered within 3 hours of symptom onset. The
Which of the following is NOT associated with results of the National Institute of Neurologic Disorders and
poor neurologic outcomes in patients with Stroke (NINDS) tPA trial showed that the use of tPA within 3
acute ischemic infarction? hours of ischemic stroke onset substantially improved func-
tional outcomes compared with a placebo group at 3 months
A. Hypoventilation.
[11]. Based on this trial, for every 100 patients given tPA, 12
B. Extensive area of low attenuation and mass effect
more experienced complete neurologic recovery than patients
on initial head CT.
given a placebo. The European Corporative Acute Stroke
C. Hyperglycemia. (ECASS) randomized trial of tPA using a 6-hour therapeutic
D. Hypothermia. window did not show an overall benefit, primarily because of a
E. Arrhythmia. high rate of cerebral hemorrhage [12]. The current strict

A B C

D E F
Fig. 4—65-year-old woman 4 hours after onset of right-sided weakness and dysarthria who underwent cerebral angiography for potential endovascular intervention.
A and B, Unenhanced CT scans show loss of gray matter–white matter differentiation (arrowheads) in left insular cortex and temporal lobe. Left middle cerebral ar-
tery (MCA) is hyperdense compared with basilar artery (arrow, A).
C, Left internal carotid artery injection image shows presence of clot (arrow) in internal carotid bifurcation and no flow visible in anterior and middle cerebral arteries.
D, Unsubtracted angiography shows microcatheter placed proximal to clot (arrow) before retrieval device was advanced.
E, Subsequent angiography shows persistent occlusion of MCA branches (arrow). Intraarterial tissue plasminogen activator (tPA) was infused through left MCA.
F, After infusion, tPA angiography shows opening of anterior division of left MCA and occlusion (arrow) of posterior division of left MCA.

S10 AJR:191, September 2008

 Nontraumatic Emergent Neuroradiology
guideline is that IV tPA must be given within 3 hours of stroke
onset. Option A is not the best response. Although administra-
tion of tPA after the 3-hour window may benefit some pa-
tients, the interval between onset of symptoms and initiation
of thrombolysis remains the most critical factor in treatment,
because therapeutic efficacy decreases even within the 3-hour
window [12]. Clearly, “time is brain.” Whether diffusion–per-
fusion mismatch can be used to better triage patients who ben-
efit from endovascular treatment beyond the 6-hour window is
yet to be determined in a large clinical trial.
Streptokinase, used to treat acute ischemic stroke, is no lon-
ger used because of unacceptably high rates of hemorrhage. It
should not be used. Option B is not the best response. Another
thrombolytic agent, urokinase, has been occasionally used
intraarterially to treat vertebral and basilar thrombosis in
some institutions up to 24 hours after symptom onset.
A randomized controlled trial from the International
Stroke Trial showed that fewer recurrent ischemic strokes
occur in patients given heparin, but this improvement was
offset by an increase in hemorrhagic stroke [13]. Recent
evidence does not support the routine use of heparin in pa-
tients with acute stroke. Option C is not the best response.
The study evaluating the value of aspirin enrolled a large
number of subjects. Aspirin was started between 12 and 24
hours after stroke onset. The results showed that aspirin sig-
nificantly improved outcomes at 6 months, but the magnitude
of the reduction was small. The early use of aspirin offers only
modest benefit [14]. Option D is not the best response.
Intraarterial tPA and mechanical clot removal can be per-
formed up to 6 hours after the onset of symptoms to an area
of blood clot via a microcatheter to an area of blood clot. The
presence of intracranial hemorrhage, severely elevated blood
pressure, low platelet count, anticoagulation therapy, and
end-stage liver or kidney disease excludes the use of tPA. Op-
tion E is the best response.
Solution to Question 12
Neurons die within a few minutes of oxygen deprivation.
Neuronal death occurs in areas of no blood flow within a few
minutes of stroke onset. Adjacent to such areas of neuronal
death is a region of hypoperfused, electronically silent tissues
that receive barely enough blood flow to keep neurons alive.
This tissue is called the “ischemic penumbra.” A major goal
of acute stroke management is resuscitation of the ischemic
penumbra. Because neuronal death is time-dependent, it is
critical to intervene as early as possible.
Treatment of acute ischemic stroke with IV tPA has prov-
en to be efficacious in clinical trials by reducing functional
disability. However, only a fraction of patients with ischemic
stroke receive IV tPA. In a community-based observational
study of 13,440 patients, approximately 3% of all ischemic
stroke patients, and 10.4% of patients admitted within 3
hours of stroke onset were treated with tPA [15]. Multicenter
studies also report the rate of tPA use outside clinical trials
ranges from 1.6% to 6%. Option A is the best response.
AJR:191, September 2008 S11
Solution to Question 13
Maintaining adequate tissue oxygenation is critical in the
setting of acute stroke to prevent hypoxia and potential
worsening of brain damage. Patients with decreased con-
sciousness or brain stem dysfunction have the greatest risk of
airway compromise. The prognosis of a patient who requires
endotracheal intubation is generally poor; approximately
50% of these patients die within 30 days of their stroke. Op-
tion A is not best response.
An extensive area of low attenuation on initial head CT
indicates widespread damage to the brain tissue. An “ear-
ly infarct sign” on unenhanced CT involving more than
one third of the territory of the middle cerebral infarction
indicates a poor outcome. The presence of mass effect or
edema is also associated with an eightfold increase in the
risk of symptomatic hemorrhage [11]. Option B is not the
best response.
Hyperglycemia is associated with poor clinical outcomes,
presumably due to increased tissue acidosis secondary to an-
aerobic glycolysis and lactic acidosis. Hyperglycemia may af-
fect the blood–brain barrier and lead to brain edema. Hypo-
glycemia may cause focal neurologic signs and symptoms
that mimic acute ischemic stroke. Hypoglycemia itself may
aggravate neuronal ischemia. The prompt assessment of the
serum glucose level and correction of the glucose level are
important. Option C is not the best response.
Fever in the setting of acute ischemic stroke is associated with
a poor neurologic outcome secondary to increased metabolic de-
mands and enhanced release of neurotransmitters. Hypo­
thermia is not associated with poor clinical outcomes. In fact,
hypothermia has been reported to be neuroprotective in experi-
mental models and small clinical trials. Hypothermia may delay
depletion of the energy reserve, slow tissue acidosis, and slow
calcium iron influx into cells. Option D is the best response.
Patients with acute ischemic stroke have an increased risk
of developing myocardial infarction and cardiac arrhythmia.
Patients with infarctions of the right hemisphere, particu-
larly those involving the insula, may have an increased risk
of cardiac complications, presumably secondary to distur-
bances in autonomic nervous system function. The most
common arrhythmia associated with acute stroke is atrial fi-
brillation, which may be either the cause of stroke or a com-
plication. Life-threatening arrhythmia is relatively uncom-
mon, but sudden death may occur. Cardiac monitoring is
often required for at least first 24 hours after the onset of
stroke symptoms. Option E is not the best response.
Conclusion
Stroke continues to have a devastating impact on public
health and is the third leading cause of death in the United
States. At least 700,000 new stroke cases occur every year.
Approximately 85% of all strokes are ischemic in nature. Be-
cause of the narrow therapeutic windows for treatment of
acute ischemic stroke, timely evaluation, diagnosis, and
treatment are of paramount importance.
 Anzai and Ghodke
Scenario 5
Clinical History
A 38-year-old woman presented to the emergency de-
partment with severe headache, nausea, and vomiting. Un-
enhanced CT of the head was ordered. The patient had a
declining level of consciousness and was admitted on that
day. MRI of the brain was performed 2 days later. The pa-
tient also had a history of a heart transplantation.
Description of Images
Unenhanced CT of the head showed a vague area of low
attenuation in the right cerebellar hemisphere and mild
mass effect on the right aspect of the fourth ventricle (Figs.
5A and 5B). Otherwise, no hemorrhage, hydrocephalus, or
midline shift was present. Contrast-enhanced MRI was rec-
ommended for further imaging workup.
QUESTION 14
What is your preferred diagnosis in this patient?
A. Brain metastases.
B. Multiple sclerosis.
C. Tuberculosis.
D. Septic emboli.
E. CNS lymphoma.
QUESTION 15
Which of the following in a patient’s medical
history is LEAST likely to be associated with
septic emboli in the brain?
A. Organ transplantation with pulmonary infection.
B. Cancer and presently receiving systemic chemo-
therapy.
C. IV drug abuse.
D. Recent travel to Southeast Asia.
E. Aortic valve replacement and endocarditis.
QUESTION 16
What are the characteristic MRI findings of
disseminated cerebral aspergillosis in
immunocompromised patients?
A. Numerous foci of restricted diffusion in the cor-
ticomedullary junction, basal ganglia, and thalami,
with minimum or no enhancement.
B. Markedly bright signal on diffusion-weighted images.
C. Involvement of the middle cerebellar peduncle.
D. Leptomeningeal invasion.
E. Infection in the paranasal sinuses.
S12 AJR:191, September 2008
FLAIR images (Figs. 5C and 5D) showed numerous foci of
hyperintensity throughout the cerebral and cerebellar hemi-
spheres, predominantly at the corticomedullary junction.
Some lesions were seen in the basal ganglia as well as the thal-
amus. The extent of disease on MRI was much more than
expected from head CT performed 2 days earlier, indicating
rapid progression of the disease process. These numerous foci
were markedly hyperintense on diffusion-weighted images
(Figs. 5E and 5F). Contrast-enhanced images showed no area
of abnormal enhancement on any of the lesions (Fig. 5G).
Solution to Question 14
In this patient, the combination of rapid progression of
disease, numerous small foci of restricted diffusion, and
lack of enhancement makes septic emboli the most likely
diagnosis. Option D is the best response.
Brain metastasis and CNS lymphoma usually do en-
hance, making options A and E incorrect responses. Tuber-
culosis can have numerous small foci of parenchymal le-
sions, or could be miliary tuberculosis less than 2 mm in
size. However, CNS tuberculous lesions are typically associ-
ated with a rim of enhancement or the target sign on gado-
linium-enhanced images. Restricted diffusion is not typical
for CNS tuberculosis. Option C is not the best response. The
lesions involve both gray matter and white matter, and the
rapid progression of disease is highly unusual for multiple
sclerosis. Option B is not the best response.
Solution to Question 15
Septic emboli are often seen in immunocompromised pa-
tients such as those who have undergone organ transplanta-
tion, those who have AIDS, and patients who have under-
gone chemotherapy. In these cases, organisms may include
tuberculosis or fungal infections. Among immunocompe-
tent patients, infection with Staphylococcus organisms is
most often seen in IV drug abusers or in patients with endo-
carditis. Options A, B, C, and E are not the best responses.
A history of recent travel to Southeast Asia can be seen in
other infections such as tuberculosis, brucellosis, West Nile
virus, hepatitis, and malaria. This is not the expected his-
tory in this patient. Option D is the best response.
This patient had a history of heart transplantation and
pulmonary aspergillosis. Pulmonary aspergillosis in severe-
ly immunocompromised patients is highly invasive and has
a dismal prognosis (near 100% mortality). It quickly gains
access to the systemic circulation and is disseminated
throughout the body, including the brain. This patient died
4 days after MRI was performed.
Solution to Question 16
Disseminated cerebral aspergillosis infection in immuno-
compromised patients is most often caused by hemato­
genous spread from pulmonary infection. Hematogenous,
or angioinvasive, Aspergillus organisms characteristically
 Nontraumatic Emergent Neuroradiology

A B C

D E F

Fig. 5—38-year-old woman with severe headache, nausea, and vomiting. Patient had history of heart trans-
plantation.
A and B, Unenhanced CT scans of head shows vague area of low attenuation in right cerebellar hemisphere
and mild mass effect on right aspect of fourth ventricle (arrow, A). No hemorrhage, hydrocephalus, or midline
shift is present.
C–G, MR images of brain obtained 2 days after A and B. FLAIR images (C and D) show numerous foci of hy-
perintensity throughout cerebral and cerebellar hemispheres, predominantly at corticomedullary junction.
Some lesions are seen in basal ganglia as well as thalamus. Extent of disease is more than expected from CT,
indicating rapid progression of disease process. These numerous foci are markedly hyperintense on diffu-
sion-weighted images (E and F). Contrast-enhanced T1-weighted image (G) shows no area of abnormal en-
hancement on any lesions.
G

AJR:191, September 2008 S13

 Anzai and Ghodke
lodge inside medium-sized blood vessels, resulting in multi-
focal infarction, and then invade through the vascular walls,
causing hemorrhagic transformation or direct extension
into the parenchyma. This vasculopathy-mediated septic
infarction has regional vulnerability to basal ganglia or
thalami, in addition to the corticomedullary junction. The
predilection to basal ganglia and thalami indicates involve-
ment of the lenticulostriate and thalamoperforating arter-
ies. Aspergillosis often destroys the internal elastic lamina
of the cerebral arteries. Perforating vessels are the first ones
to lose their patency because of their narrow diameter.
MRI characteristics of disseminated aspergillosis in-
volvement of the brain in 18 patients was reported by De-
Lone et al. [16] and others [17, 18]. Those authors reported
that the typical MRI appearance is a predilection to basal
ganglia or thalami. Enhancement was minimal or absent.
Lack of enhancement is most likely related to the host’s
immune capacity. Severely immunocompromised patients
have no or little immune capacity to react to an infectious
organism to form capsule or inflammatory response; thus,
lack of enhancement may indicate poor prognosis and rapid
dissemination of angioinvasive aspergillosis. Option A is the
best response.
Marked bright signal on diffusion-weighted images in this
patient likely reflects infarction and cytotoxic edema. Numer-
Scenario 6
Clinical History
A 27-year-old woman with a 2-week history of nausea
and vomiting after a recent cholecystectomy presented to
the emergency department with abdominal pain and nau-
sea. Abdominal CT and pelvic sonography were negative.
The patient later developed slurred speech and confusion.
Brain MRI was performed (Figs. 6A–6D).
Description of Images
FLAIR images showed bilateral symmetric hyperinten-
sity involving the mamillary bodies, the medial thalami
along the third ventricle, and the periaqueductal gray mat-
QUESTION 17
What is the diagnosis given the imaging
abnormality and clinical presentations?
A. Creutzfeldt-Jakob disease.
B. Leigh disease.
C. Wernicke’s encephalopathy.
D. Maple syrup urine disease.
E. Wilson’s disease.
S14 AJR:191, September 2008
ous foci of restricted diffusion can be seen in patients with
embolic infarction, brain abscesses, and metastases from high-
ly cellular tumors [19]. Option B is not the best response.
Neither involvement of the middle cerebellar peduncle
nor leptomeningeal involvement is a typical finding for dis-
seminated aspergillosis. Options C and D are not the best
responses.
Involvement of the paranasal sinuses is often seen in dia-
betic patients who have angioinvasive mucormycosis. Mu-
cormycosis is a rare opportunistic infection caused by ubi­
quitous fungi typically found in soil or dust. The route of
infection is usually rhinocerebral and is commonly seen in
patients with uncontrolled diabetes, which is often associ-
ated with metabolic acidosis or ketoacidosis [20]. Mucormy-
cosis can spread from the paranasal sinuses to the brain in a
few days. Treatment should include aggressive débridement
and IV amphotericin B. Option E is not the best response.
Conclusion
Rapid progression, early ischemic manifestation, and
predilection for the perforating arteries are characteristic
features of disseminated aspergillosis infection in severely
immunocompromised patients. Diagnosis should be made
when clinical suspicion is high so that aggressive IV anti-
fungal therapy can be initiated.
ter. Diffusion-weighted images also showed an area of hy-
perintensity in the medial thalami.
QUESTION 18
Which of the followings is LEAST likely to be a
risk factor for Wernicke’s encephalopathy?
A. Chronic alcoholism.
B. Prolonged parenteral nutrition without a vitamin
supplement.
C. Hyperemesis gravidarum.
D. Gastrectomy.
E. Anorexia nervosa.
QUESTION 19
Which one of the following is a characteristic
clinical feature of Wernicke’s encephalopathy?
A. Learning disability.
B. Rigidity and tremor.
C. Visual hallucination.
D. Nystagmus and bilateral lateral rectus paralysis.
E. Global ataxia.
 Nontraumatic Emergent Neuroradiology
Fig. 6—27-year-old woman with 2-week history of
nausea and vomiting after recent cholecystectomy.
Patient presented with abdominal pain and nausea.
A–D, FLAIR images show bilateral symmetric hyper-
intensity involving mamillary bodies, medial thalami
along third ventricle, and periaqueductal gray mat-
ter (arrows, A–C). Diffusion-weighted image (D) also
shows area of hyperintensity in medial thalami.
Solution to Question 17
Brain MRI shows T2 hyperintensity predominantly in-
volving the medial thalami, periaqueductal gray matter, and
mamillary bodies. The distribution of signal abnormalities
on MRI and a history of prolonged emesis make Wernicke’s
encephalopathy the most likely diagnosis. Wernicke’s en-
cephalopathy is a severe neurologic disorder caused by thia-
mine (vitamin B1) deficiency. It is a disabling and potentially
lethal condition that can be prevented or reversed if treated
early. It is often unrecognized and is likely more prevalent
than reported. Wernicke’s encephalopathy can progress to a
state of chronic amnesia called “Korsakoff ’s syndrome.” Pa-
tients suffering from Wernicke’s encephalopathy show de-
generation of the diencephalic regions, specifically the ma-
millary bodies and the medial thalamic nuclei along the
mammillothalamic tract. Option C is the best response.
AJR:191, September 2008 S15
A B
C D
Creutzfeldt-Jakob disease (CJD) is a rare neurodegenera-
tive disorder that is currently thought to be caused by an
abnormal protein called “prion.” Pathologically, CJD leads
to spongiform encephalopathy. Patients with CJD often
present with progressive dementia. CJD is divided into
three types: sporadic CJD, with no known risk factors, the
most common type in the United States; hereditary CJD,
associated with genetic mutation; and acquired CJD, which
is acquired by medical procedures such as corneal trans-
plantation, human growth hormone injection, and so forth.
Bovine spongiform encephalopathy (BSE) or mad cow dis-
ease, is called “variant CJD,” and is believed to be due to
the ingestion of infected beef. The signal abnormality on
FLAIR and diffusion images is seen in the cerebral cortex,
striatum, and posterior thalami. Mamillary bodies are not
usually involved in CJD. Option A is not the best response.
 Anzai and Ghodke
Leigh disease is a mitochondrial disorder caused by pyru-
vate carboxylase deficiencies. It leads to necrotizing en-
cephalomyelopathy. Leigh disease often affects the putami-
na bilaterally, other deep gray matter structures, and the
brain stem [21]. Option B is not the best response.
Maple syrup urine disease results from severe inherited
defects in branched-chain amino acids. Patients are unable
to catabolize branched-chain amino acids (leucine, isoleu-
cine, and valine), which are increased in the blood and urine.
The primary therapy is a protein-restricted diet. MRI find-
ings are white matter involvement in the cerebellum, the
periaqueductal gray matter along the dorsal midbrain, the
cerebral peduncle, and the basal ganglia and thalami. Ma-
millary bodies are not involved. Patients with maple syrup
urine disease present as newborns or in infancy. Option D is
not the best response.
Wilson’s disease is an autosomal recessive disorder. The Wil-
son’s disease gene is mapped to chromosome 13. The main fea-
ture is accumulation of copper in the tissues, predominantly
in the cornea, brain, and liver. A suppressed level of cerulo-
plasmin is observed in more than 80% of patients. Clinical
symptoms include dysarthria, dystonia, rigidity, and ataxia.
MRI findings in Wilson’s disease are signal abnormality in the
lentiform nucleus and the thalami as well as tegmentum of
the midbrain, red nuclei, and substantia nigra. Periaqueduc-
tal gray matter involvement has been reported in Wilson’s
disease. Mamillary bodies are not normally abnormal in Wil-
son’s disease. Option E is not the best response.
Solution to Question 18
Wernicke’s encephalopathy is a neurologic disorder with
acute onset. It is caused by a thiamine deficiency due to
poor oral intake in chronic alcoholics, food refusal in an-
orexia nervosa, or recurrent vomiting in pregnant patients.
Approximately 50% of patients with Wernicke’s encepha­
lopathy are not alcoholic. This patient presented with 2
weeks of nausea and vomiting. Other causes include chron-
ic infection and a febrile status, pancreatitis, cancer, chron-
ic dialysis, and prolonged parenteral hyperalimentation
without a vitamin supplement [22]. Options A, B, C, and E
are not the best responses.
Gastrectomy is often associated with vitamin B12 deficien-
cies. In order for vitamin B12 to be absorbed from the small
bowel, it needs to be chemically linked to an intrinsic factor
that is produced in the stomach. Gastrectomy leads to mal-
absorption of vitamin B12, which results in pernicious ane-
mia. Gastrectomy is least likely to be associated with Wer-
nicke’s encephalopathy. Option D is the best response.
Solution to Question 19
The triad of encephalopathy, ataxic gait, and oculomotor
dysfunction is seen in only one third of patients with Wer-
nicke’s encephalopathy. Ocular abnormalities are the hall-
marks of Wernicke’s encephalopathy. The oculomotor signs
S16 AJR:191, September 2008
are nystagmus, bilateral lateral rectus palsies, and conjugate
gaze palsies reflecting involvement of the oculomotor and ab-
ducens nerves. Option D is the best response. Gait ataxia is
believed to be due to focal midline degeneration of the supe-
rior vermis, as opposed to global ataxia, a sign of cerebellar
dysfunction. Option E is not the best response. Cerebellar
testing with the finger-to-nose or heel-to-shin test may not
elicit any notable deficit. Vestibular dysfunction without
hearing loss is also a common finding.
Rigidity and tremor as well as bradykinesia and postur-
al instability are common symptoms seen in patients with
Parkinson’s disease, not in patients with Wernicke’s enceph-
alopathy. Visual hallucination is associated with psychiat-
ric disorders and drugs, particularly alcohol. Patients with
schizo­phrenia often have visual and, more often, auditory
hallucinations. Learning disability refers to a group of dis-
orders affecting academic and functional skills, including the
abilities to listen, speak, write, read, and organize informa-
tion. It is not specific for Wernicke’s encephalopathy. Thus,
options A, B, and C are not the correct responses.
Conclusion
Wernicke’s encephalopathy is a severe medical emergency
that is often associated with malnutrition states. It is an un-
derdiagnosed disease that can be reversed or treated with IV
thiamin. Wernicke’s encephalopathy should be considered in
patients with chronic alcohol abuse and malnutrition or pro-
longed vomiting along with acute confusion, ataxia, oculo-
motor abnormalities, and memory disturbance. Brain MRI
should be ordered to assess changes in the patient’s mental
status. It is important for radiologists to recognize this dis-
ease so that appropriate treatment is initiated immediately.
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