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AJR Integrative Imaging


Nontraumatic Emergent Neuroradiology:

Review and Self-Assessment Module
Yoshimi Anzai1 and Basavaraj Ghodke2


By completing this educational activity, the participant will:
Objective A. Exercise, self-assess, and improve his or her understand-
The educational objectives for this case-based self-assess- ing of the imaging features of nontraumatic emergent
ment module on imaging of nontraumatic emergent neuro- neuroradiology diagnoses.
radiology are to use case examples to teach the imaging B. Exercise, self-assess, and improve his or her understand-
features of CT and MRI of emergent neuroradiologic diag- ing of the clinical features of nontraumatic emergent
noses and to improve the reader’s understanding of the neuroradiology diagnoses.
pathophysiology and clinical management of each clinical
scenario. Imaging plays a critical role in assessing patients RECOMMENDED READING
with acute neurologic symptoms. CT is quick and easy to 1. Provenzale JM. Centennial dissertation. Honoring Ar-
perform in an emergency setting and is often the technique thur W. Goodspeed, MD and James B. Bullitt, MD. CT
of choice for any neurologic emergency situations. Clinical and MR imaging and nontraumatic neurologic emergen-
correlation is crucial for accurate diagnosis and triaging pa- cies. AJR 2000; 174:289–299
tients for further evaluation. Persistent or progressive neu- 2. Wintermark M, Albers GW, Alexandrov AV, et al. Acute
rologic symptoms despite negative CT should prompt other stroke imaging research roadmap. Stroke 2008; 39:
imaging studies. 1621–1628
3. Beauchamp NJ Jr, Bryan RN. Acute cerebral ishemic
Conclusion infarction: a pathophysiologic review and radiologic per-
At the end of this self-assessment module, readers will be spective. AJR 1998; 171:73–84
able to generate a concise list of differential diagnoses for 4. Zuccoli G, Gallucci M, Capellades J, et al. Wernicke en-
imaging findings that often are encountered in patients with cephalopathy: MR findings at clinical presentation in
nontraumatic neurologic emergency. twenty-six alcoholic and nonalcoholic patients. AJNR
INTRODUCTION 2007; 28:1328–1331
This self-assessment module on nontraumatic emergent
neuroradiology diagnoses has an educational component INSTRUCTIONS
and a self-assessment component. The educational compo- 1. Complete the educational and self-assessment components.
nent consists of six case scenarios that the participant 2. Visit and select Publications/Journals/
should work through and four recommended articles that SAM Articles from the left-hand menu bar.
may provide additional information and perspective. The 3. Using your member login, order the online SAM as di-
self-assessment component consists of 19 multiple-choice rected.
questions with solutions. All of these materials are avail- 4. Follow the online instructions for entering your respons-
able on the ARRS Website ( To claim CME es to the self-assessment questions and complete the test
and SAM credit, each participant must enter his or her re- by answering the questions online.
sponses to the questions online.

Keywords: brain imaging, case-based learning, emergency, neuroradiology, self-assessment

Received September 7, 2007; accepted after revision January 30, 2008.
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 (
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

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 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.

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.

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Solution to Question 1 gal infection, bacterial vasculitis, and HIV vasculitis. The fre-
Arteriovenous malformation (AVM) is not an unusual cause quent causes of noninfectious vasculitis are immune-mediated
of intracranial hematoma for the young patient in this case. vasculitis such as rheumatoid arthritis and systemic lupus er-
Patients with AVM often present with acute intracranial hem- ythematosus, and granulomatous disease, such as Wegener’s
orrhage or ischemic symptoms due to steal phenomenon. CT granulomatosis and sarcoid, as well as giant cell arteritis (also
angiography often reveals a tangle of markedly dilated arter- called temporal arteritis). Drugs, particularly amphetamines
ies and veins with a nidus of AVM. If the AVM is small, it may and cocaine, are frequent causes of noninfectious vasculitis.
not be visualized or diagnosed correctly with CT angiography. An amphetamine causes inflammatory vasculitis with vascu-
In this setting, cerebral angio­graphy is the most definitive test. lar wall necrosis and subsequent hemorrhage. The pathologic
However, cerebral angio­graphy in this patient did not show features of amphetamine-related vasculitis are similar to
early venous filling or an abnormal tangle of blood vessels to those of polyarteritis nodosa. This patient had taken amphet-
indicate AVM. Therefore, option A is not the best response. amines for several years. Cocaine, on the other hand, induces
Acute intracranial hematoma can have a number of causes. cerebral infarction or ischemia as well as hemorrhage by vaso-
The most common cause is hypertensive hemorrhage. Hy- constrictive effect and increased platelet aggregation, rather
pertensive hemorrhages, which originate from terminal small than a vasculitis-type inflammation of the vessels [1]. Options
vessels, are often centered at the basal ganglia, particularly A, B, C, and D are not the best responses.
the putamen and the external capsule; the thalamus; the Marfan syndrome is an autosomal dominant disorder of
pons; and occasionally, the cerebellum. Lobar hemorrhage the connective tissue characterized by disproportionally
can be seen in a setting of hypertension (≈ 1–2%), but it is long limbs and tall stature. It affects the heart and aorta
rare. Hypertensive hemorrhage is much more common in and causes aortic root dilatation, aortic regurgitation, and
older patients. Option B is not the correct response. dissection. However, involvement of CNS vessels is unusual.
Certain types of brain tumors are often associated with Option E is the best response.
intracranial hemorrhage. These are often aggressive tumors, Solution to Question 3
such as glioblastoma multiforme, or vascular tumors, such as Because many cases of vasculitis affect small to medium-
metastases from renal cell carcinoma or melanoma, but rare- sized blood vessels, MR angiography is relatively insensitive
ly papillary thyroid cancer and choriocarcinoma. Lung can- for the diagnosis of CNS vasculitis. CT angiography is less
cer and breast cancer are not considered vascular; however, accurate than cerebral angiography. Cerebral angiography is
the incidence of brain metastasis from lung and breast can- used as the gold standard for diagnosis at many institutions.
cers is exceedingly higher than that of other vascular can- When characteristic angiographic findings such as alternat-
cers. Thus, when all hemorrhagic brain metastases are re- ing areas of stenosis and dilatation or a beading appearance
viewed, lung and breast remain the two top primary choices. are observed in multiple vessels and multiple vascular beds,
Intracranial hemorrhage associated with brain tumor often cerebral angiography is diagnostic. However, angiography
has a focal area of enhancement and significant mass effect can be normal in up to 40% of biopsy-proven cases. Thus,
or vasogenic edema. Option C is not the best response. negative cerebral angiography does not completely exclude
Vasculitis is one of the causes of intracranial hemorrhage the diagnosis. Moreover, its specificity is not perfect. Intra-
and is more frequently seen in young patients. Vasculitis can cranial atherosclerotic disease may show irregularity of mul-
present with acute hemorrhage or ischemic symptoms. Many tiple vessels, mimicking CNS vasculitis.
causes of vasculitis affect small- to medium-sized vessels; Transcranial Doppler sonography is used to characterize
therefore, CT angiography may not show an irregularity of the morphology of the superficial temporal artery as a
the blood vessels as definitively as cerebral angiography. Cere- screening tool for temporal arteritis. However, transcranial
bral angiography in Figures 1C and 1D shows a classic ap- Doppler sonography is not suitable to evaluate arteries fully
pearance of segmental narrowing and poststenotic dilatation to diagnose CNS vasculitis. Options A, B, D, and E are not
involving multiple small- to medium-sized blood vessels, con- the best responses.
sistent with vasculitis. Option D is the best response. Biopsy of CNS tissue would logically be considered the
Amyloid angiopathy often causes a lobar intracranial ultimate gold standard of diagnosis, but clearly the proce-
hemorrhage centered at the deep white matter in the frontal dure is limited by several factors. It is highly invasive and
or parietal lobes. Amyloid angiopathy is much more common carries certain risks. Successful biopsy requires a willing
in elderly patients and is rare in young patients. Cerebral an- and experienced neurosurgeon, who may not be readily
giography may not show any irregularity or narrowing. Am- available. Sampling error could result in limited sensitivity.
yloid angiopathy is often diagnosed after excluding other The site of biopsy should be tailored to the individual pa-
causes in elderly patients. Option E is not the best response. tient [2]. The biopsy of the superficial temporal artery is
Solution to Question 2 often performed in patients suspected of having giant cell
The causes of vasculitis are often divided into infectious vasculitis. The false-negative rate of biopsy for a diagnosis
and noninfectious. Infectious causes include tuberculosis, fun- of vasculitis has been reported to be 16%, yielding a sensi-

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Anzai and Ghodke

tivity of 84% [2]. Therefore, the most definitive test is bi- posure, radiation, and malignancies. Cerebral angiography
opsy of the blood vessels. Option C is the best response. is more sensitive than noninvasive vascular imaging (CT an-
giography or MR angiography), but it is invasive and has
Conclusion limited sensitivity and specificity. Treatment of noninfec-
Vasculitis involving the CNS presents diagnostic chal- tious vasculitis is long-term, high-dose immunosuppression,
lenges to clinicians as well as radiologists. There are many which causes profound morbidity as a result of opportunis-
causes, including infection, autoimmune disease, drug ex- tic infection or organ failures.

Scenario 2 sign (Fig. 2B). Brain MRI, including sagittal T1-weighted

Clinical History imaging (Fig. 2C), diffusion-weighted imaging (Fig. 2D),
A 19-year-old woman presented to an outside hospital with and apparent diffusion coefficient (ADC) mapping (Fig. 2E)
severe headache, nausea, vomiting, and photophobia. She had were also performed at the outside hospital. The sagittal
no significant medical history. Laboratory examinations were T1-weighted image showed an isointense clot along the su-
unremarkable. Her initial head CT scans (unenhanced and perior sagittal sinus that was of concern for superior sagit-
contrast-enhanced) are shown in Figures 2A and 2B. tal sinus thrombosis. The diffusion-weighted image showed
no areas of restricted diffusion or ADC map abnormality.
Description of Images The patient’s mental status deteriorated at the outside hos-
Unenhanced CT of the head showed a focal area of low pital and she was transferred to the emergency department
attenuation in the left temporal lobe and mild regional mass of our hospital, at which time she was unresponsive and
effect. The ventricles were normal in size with no shift of obtunded, and her pupils were dilated. CT of the head was
midline structures. No parenchymal hemorrhage was evi-
dent. The contrast-enhanced image showed the empty delta

QUESTION 4 Which one of the following techniques is

LEAST appropriate to confirm a diagnosis of
What is your preferred diagnosis after the venous sinus thrombosis?
initial CT scans (Figs. 2A and 2B)?
A. Rapid contrast-enhanced gradient-echo MRI with
A. Acute infarction of the left middle cerebral artery contrast-enhanced multiplanar imaging.
(MCA). B. MDCT angiography or venography.
B. Herpes encephalitis. C. Brain MRI and phase-contrast MR venography.
C. Underlying tumor. D. Cerebral angiography.
D. Sinus thrombosis. 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)

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


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.

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Anzai and Ghodke

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

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Scenario 3 veloping hydrocephalus. Focal hematoma in the medial

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


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.

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Solution to Question 7
QUESTION 7 Arteriovenous malformation (AVM) is a congenital anom-
aly of blood vessels with one or more large feeding arteries
What is the most likely cause of the findings and drainage veins. AVM often presents not only with sub-
on unenhanced CT? arachnoid hemorrhage but also with parenchymal hemor-
A. Arteriovenous malformation rupture. rhage or symptoms of ischemia due to steal phenomenon.
B. Venous sinus thrombosis. Blood flow preferentially goes to AVMs with low resistance,
leaving the adjacent normal brain relatively hypoperfused.
C. Cerebral aneurysm rupture.
CT may show a hyperdense mass or curvilinear or speckled
D. Amyloid angiopathy.
calcification adjacent to a parenchymal hematoma. MRI
E. Hypertensive hemorrhage.
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
What is the 30-day mortality rate of hemorrhage; however, it tends to be focal to the area of ve-
subarachnoid hemorrhage secondary to nous infarction or hypertension. Venous sinus thrombosis
aneurysm? does not normally present diffuse subarachnoid hemor-
rhage. Option B is not the best response.
A. 15%. Unenhanced CT of the head shows diffuse subarachnoid
B. 30%. hemorrhage as well as a focal hematoma along the anterior
C. 45%. falx, a typical location for a ruptured anterior communicating
D. 65%. artery aneurysm. Option C is the best response. Approximately
E. 80%. 50–70% of subarachnoid hemorrhages are due to aneurysm
rupture. Of patients with subarachnoid hemorrhage, 10–15%
QUESTION 9 have no aneurysm found even on cerebral angiography.
Amyloid angiopathy commonly affects elderly patients
Which of the following factors does NOT
and presents with a lobar rather than a subarachnoid hemor-
influence management decisions for a
rhage. Option D is not the best response. Hypertensive hem-
ruptured aneurysm?
orrhage often affects the small perforating vessels along the
A. Age. lenticulostriate or thalamoperforating vessels and thus is of-
B. Sex. ten present with parenchymal hemorrhage in the basal gan-
C. Aneurysm size. glia, thalamus, and pons. Option E is not the best response.
D. Aneurysm location.
E. History of hypertension. Solution to Question 8
Aneurysmal subarachnoid hemorrhage (SAH) has a
QUESTION 10 30-day mortality rate of 45%, with approximately half of
the survivors sustaining irreversible brain damage [7]. Option
Despite CT angiography showing an aneurysm C is the best response. The annual incidence of aneurysmal
causing subarachnoid hemorrhage, cerebral SAH is six per 100,000 in the United States. Approximately
angiography is still performed at some 5–10% of stroke cases are secondary to ruptured saccular
institutions. Which one of the following is aneurysms [8]. Recurrent hemorrhage remains a serious con-
NOT a rationale for performing cerebral sequence, with a 70% fatality rate in patients who rebleed.
angiography in this setting?
Solution to Question 9
A. Searching for an additional incidental aneurysm that Treatment options for a ruptured aneurysm are surgical
could be treated at the same time. resection or endovascular coil embolization. Endovascular oc-
B. Better assessing the degree of incorporation of the clusion of aneurysms using electrolytically detachable Gug-
aneurysm wall into the parent vessel. lielmi detachable coil system (GDC, Target Therapeutics [now
C. Assessing flow dynamics—that is, the side of the Boston Scientific]) has been used to treat ruptured or unrup-
internal carotid artery feeding anterior tured aneurysms in a large number of patients worldwide.
communicating aneurysm. Published reports suggest that the endovascular technique is
D. Measuring the aneurysm neck–dome ratio. associated with fewer treatment-related complications than
E. Suspecting mycotic aneurysm in patients with IV open surgery [7]. However, the long-term efficacy in the pre-
drug use. vention of rupture or recurrence of aneurysm remains in-
determinate. The recent International Subarachnoid Aneu-

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rysm Trial (ISAT) showed that retreatment was performed small perforating vessels, the relationship of the aneurysm to
in 17.4% of patients treated with endovascular coiling and the parent vessels (how much of the aneurysm wall is incor-
in 3.8% of patients after surgical clipping [9]. Younger age porated into the parent vessel), and the flow dynamics of an-
and larger aneurysm size were risk factors for retreatment. eurysms that affect surgical planning. Multiple aneurysms
A higher rate of recurrence is seen in posterior communicat- can be seen in patients with subarachnoid hemorrhage. These
ing artery aneurysms after endovascular coiling and anterior incidental aneurysms are often smaller than 3 mm, which CT
communicating artery aneurysms after surgical clipping, angiography has a limited ability to detect. Small unrup-
which might reflect the technical difficulty. Hyper­tension is tured aneurysms are associated with a risk of future SAHs.
also associated with an increasing rate of rupture in patients If they are in a surgically or endovascularly accessible loca-
with an unruptured aneurysm. Thus age, medical history tion, these incidental aneurysms can be treated at the same
such as hypertension, aneurysm location, and size are all rel- time as the ruptured primary aneurysm. Mycotic aneurysms
evant factors in treatment decisions and, therefore, options often involve peripheral vessels and may present with sub-
A, C, D, and E, all relevant factors in treatment decisions, arachnoid or parenchymal hemorrhage or septic emboli. Be-
are not the best responses. Although aneurysms are more cause mycotic aneurysms involve distal vessels, cerebral angi-
common in women than in men, sex is not a factor affecting ography is a more definitive test than CT angiography.
management decisions or predicting outcomes. Option B is Options A, B, C, and E are not the best responses. The aneu-
the best response. rysm neck–dome ratio can be calculated on the basis of CT
angiography. Option D is the best response.
Solution to Question 10
CT angiography is a noninvasive vascular imaging tech- Conclusion
nique that has replaced catheter angiography in some insti- SAH associated with aneurysm rupture has high rates of
tutions. CT angiography may show aneurysms larger than 3 mortality and morbidity. Accurate detection and assess-
mm with a sensitivity of 77–97% and specificity of 87–100% ment of cerebral aneurysm lead to proper treatment deci-
[10]. CT angiography also has been used as a screening tool in sions by either surgical clipping or endovascular coiling.
populations at high risk for cerebral aneurysms. Cerebral an- Although CT angiography has rapidly replaced cerebral an-
giography, however, still remains the gold standard in the di- giography in some institutions, 3D rotational angiography
agnostic evaluation of cerebral aneurysms. In particular, 3D provides the most information regarding characterization
rotational angiography shows the most information about of aneurysms in relation to parent or adjacent vessels.

Scenario 4
Clinical History QUESTION 11
A 65-year-old woman presented to the emergency de-
partment 4 hours after the onset of right-sided weakness Assuming the patient does not have any
and dysarthria. Unenhanced CT of the head was obtained. medical conditions, what would be the most
Description of Images appropriate urgent therapy at this point?
Unenhanced CT of the head (Figs. 4A and 4B) showed A. IV recombinant tissue plasminogen activator (tPA).
loss of gray–white matter differentiation in the left insular B. IV streptokinase.
cortex and the temporal lobe. The left middle cerebral ar- C. IV heparin.
tery was hyperdense compared with the basilar artery. D. Antiplatelet therapy and aspirin.
The patient underwent cerebral angiography for poten- E. Intraarterial tPA and mechanical clot removal.
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 QUESTION 12
the anterior and middle cerebral arteries. The microcathe- What percentage of patients with acute
ter was placed proximal to the clot before advancing the ischemic stroke are treated with IV tPA?
Merci retrieval device (Merci Retrieval System, Concentric
Medical, Inc.) (Fig. 4D). Subsequent angiography showed A. Less than 6%.
persistent occlusion of the MCA branches (Fig. 4E). In- B. 8–15%.
traarterial tissue plasminogen activator (tPA) was infused C. 20–25%.
through the left MCA; after tPA infusion, angiography (Fig. D. 25–50%.
4F) showed opening of the anterior division of the left MCA E. 50–60%.
and occlusion of the posterior division of the left MCA.

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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


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.

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Nontraumatic Emergent Neuroradiology

guideline is that IV tPA must be given within 3 hours of stroke Solution to Question 13
onset. Option A is not the best response. Although administra- Maintaining adequate tissue oxygenation is critical in the
tion of tPA after the 3-hour window may benefit some pa- setting of acute stroke to prevent hypoxia and potential
tients, the interval between onset of symptoms and initiation worsening of brain damage. Patients with decreased con-
of thrombolysis remains the most critical factor in treatment, sciousness or brain stem dysfunction have the greatest risk of
because therapeutic efficacy decreases even within the 3-hour airway compromise. The prognosis of a patient who requires
window [12]. Clearly, “time is brain.” Whether diffusion–per- endotracheal intubation is generally poor; approximately
fusion mismatch can be used to better triage patients who ben- 50% of these patients die within 30 days of their stroke. Op-
efit from endovascular treatment beyond the 6-hour window is tion A is not best response.
yet to be determined in a large clinical trial. An extensive area of low attenuation on initial head CT
Streptokinase, used to treat acute ischemic stroke, is no lon- indicates widespread damage to the brain tissue. An “ear-
ger used because of unacceptably high rates of hemorrhage. It ly infarct sign” on unenhanced CT involving more than
should not be used. Option B is not the best response. Another one third of the territory of the middle cerebral infarction
thrombolytic agent, urokinase, has been occasionally used indicates a poor outcome. The presence of mass effect or
intraarterially to treat vertebral and basilar thrombosis in edema is also associated with an eightfold increase in the
some institutions up to 24 hours after symptom onset. risk of symptomatic hemorrhage [11]. Option B is not the
A randomized controlled trial from the International best response.
Stroke Trial showed that fewer recurrent ischemic strokes Hyperglycemia is associated with poor clinical outcomes,
occur in patients given heparin, but this improvement was presumably due to increased tissue acidosis secondary to an-
offset by an increase in hemorrhagic stroke [13]. Recent aerobic glycolysis and lactic acidosis. Hyperglycemia may af-
evidence does not support the routine use of heparin in pa- fect the blood–brain barrier and lead to brain edema. Hypo-
tients with acute stroke. Option C is not the best response. glycemia may cause focal neurologic signs and symptoms
The study evaluating the value of aspirin enrolled a large that mimic acute ischemic stroke. Hypoglycemia itself may
number of subjects. Aspirin was started between 12 and 24 aggravate neuronal ischemia. The prompt assessment of the
hours after stroke onset. The results showed that aspirin sig- serum glucose level and correction of the glucose level are
nificantly improved outcomes at 6 months, but the magnitude important. Option C is not the best response.
of the reduction was small. The early use of aspirin offers only Fever in the setting of acute ischemic stroke is associated with
modest benefit [14]. Option D is not the best response. a poor neurologic outcome secondary to increased metabolic de-
Intraarterial tPA and mechanical clot removal can be per- mands and enhanced release of neurotransmitters. Hypo­
formed up to 6 hours after the onset of symptoms to an area thermia is not associated with poor clinical outcomes. In fact,
of blood clot via a microcatheter to an area of blood clot. The hypothermia has been reported to be neuroprotective in experi-
presence of intracranial hemorrhage, severely elevated blood mental models and small clinical trials. Hypothermia may delay
pressure, low platelet count, anticoagulation therapy, and depletion of the energy reserve, slow tissue acidosis, and slow
end-stage liver or kidney disease excludes the use of tPA. Op- calcium iron influx into cells. Option D is the best response.
tion E is the best response. Patients with acute ischemic stroke have an increased risk
Solution to Question 12 of developing myocardial infarction and cardiac arrhythmia.
Neurons die within a few minutes of oxygen deprivation. Patients with infarctions of the right hemisphere, particu-
Neuronal death occurs in areas of no blood flow within a few larly those involving the insula, may have an increased risk
minutes of stroke onset. Adjacent to such areas of neuronal of cardiac complications, presumably secondary to distur-
death is a region of hypoperfused, electronically silent tissues bances in autonomic nervous system function. The most
that receive barely enough blood flow to keep neurons alive. common arrhythmia associated with acute stroke is atrial fi-
This tissue is called the “ischemic penumbra.” A major goal brillation, which may be either the cause of stroke or a com-
of acute stroke management is resuscitation of the ischemic plication. Life-threatening arrhythmia is relatively uncom-
penumbra. Because neuronal death is time-dependent, it is mon, but sudden death may occur. Cardiac monitoring is
critical to intervene as early as possible. often required for at least first 24 hours after the onset of
Treatment of acute ischemic stroke with IV tPA has prov- stroke symptoms. Option E is not the best response.
en to be efficacious in clinical trials by reducing functional Conclusion
disability. However, only a fraction of patients with ischemic Stroke continues to have a devastating impact on public
stroke receive IV tPA. In a community-based observational health and is the third leading cause of death in the United
study of 13,440 patients, approximately 3% of all ischemic States. At least 700,000 new stroke cases occur every year.
stroke patients, and 10.4% of patients admitted within 3 Approximately 85% of all strokes are ischemic in nature. Be-
hours of stroke onset were treated with tPA [15]. Multicenter cause of the narrow therapeutic windows for treatment of
studies also report the rate of tPA use outside clinical trials acute ischemic stroke, timely evaluation, diagnosis, and
ranges from 1.6% to 6%. Option A is the best response. treatment are of paramount importance.

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Anzai and Ghodke

Scenario 5 FLAIR images (Figs. 5C and 5D) showed numerous foci of

Clinical History hyperintensity throughout the cerebral and cerebellar hemi-
A 38-year-old woman presented to the emergency de- spheres, predominantly at the corticomedullary junction.
partment with severe headache, nausea, and vomiting. Un- Some lesions were seen in the basal ganglia as well as the thal-
enhanced CT of the head was ordered. The patient had a amus. The extent of disease on MRI was much more than
declining level of consciousness and was admitted on that expected from head CT performed 2 days earlier, indicating
day. MRI of the brain was performed 2 days later. The pa- rapid progression of the disease process. These numerous foci
tient also had a history of a heart transplantation. were markedly hyperintense on diffusion-weighted images
(Figs. 5E and 5F). Contrast-enhanced images showed no area
Description of Images of abnormal enhancement on any of the lesions (Fig. 5G).
Unenhanced CT of the head showed a vague area of low
attenuation in the right cerebellar hemisphere and mild Solution to Question 14
mass effect on the right aspect of the fourth ventricle (Figs. In this patient, the combination of rapid progression of
5A and 5B). Otherwise, no hemorrhage, hydrocephalus, or disease, numerous small foci of restricted diffusion, and
midline shift was present. Contrast-enhanced MRI was rec- lack of enhancement makes septic emboli the most likely
ommended for further imaging workup. 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-
QUESTION 14 sions, or could be miliary tuberculosis less than 2 mm in
What is your preferred diagnosis in this patient? size. However, CNS tuberculous lesions are typically associ-
ated with a rim of enhancement or the target sign on gado-
A. Brain metastases. linium-enhanced images. Restricted diffusion is not typical
B. Multiple sclerosis. for CNS tuberculosis. Option C is not the best response. The
C. Tuberculosis. lesions involve both gray matter and white matter, and the
D. Septic emboli. rapid progression of disease is highly unusual for multiple
E. CNS lymphoma. sclerosis. Option B is not the best response.

QUESTION 15 Solution to Question 15

Septic emboli are often seen in immunocompromised pa-
Which of the following in a patient’s medical tients such as those who have undergone organ transplanta-
history is LEAST likely to be associated with tion, those who have AIDS, and patients who have under-
septic emboli in the brain? gone chemotherapy. In these cases, organisms may include
A. Organ transplantation with pulmonary infection. tuberculosis or fungal infections. Among immunocompe-
B. Cancer and presently receiving systemic chemo- tent patients, infection with Staphylococcus organisms is
therapy. most often seen in IV drug abusers or in patients with endo-
carditis. Options A, B, C, and E are not the best responses.
C. IV drug abuse.
A history of recent travel to Southeast Asia can be seen in
D. Recent travel to Southeast Asia.
other infections such as tuberculosis, brucellosis, West Nile
E. Aortic valve replacement and endocarditis.
virus, hepatitis, and malaria. This is not the expected his-
tory in this patient. Option D is the best response.
QUESTION 16 This patient had a history of heart transplantation and
What are the characteristic MRI findings of pulmonary aspergillosis. Pulmonary aspergillosis in severe-
disseminated cerebral aspergillosis in ly immunocompromised patients is highly invasive and has
immunocompromised patients? a dismal prognosis (near 100% mortality). It quickly gains
access to the systemic circulation and is disseminated
A. Numerous foci of restricted diffusion in the cor- throughout the body, including the brain. This patient died
ticomedullary junction, basal ganglia, and thalami, 4 days after MRI was performed.
with minimum or no enhancement.
B. Markedly bright signal on diffusion-weighted images. Solution to Question 16
C. Involvement of the middle cerebellar peduncle. Disseminated cerebral aspergillosis infection in immuno-
D. Leptomeningeal invasion. compromised patients is most often caused by hemato­
E. Infection in the paranasal sinuses. genous spread from pulmonary infection. Hematogenous,
or angioinvasive, Aspergillus organisms characteristically

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Nontraumatic Emergent Neuroradiology



Fig. 5—38-year-old woman with severe headache, nausea, and vomiting. Patient had history of heart trans-
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.

AJR:191, September 2008 S13

Anzai and Ghodke

lodge inside medium-sized blood vessels, resulting in multi- ous foci of restricted diffusion can be seen in patients with
focal infarction, and then invade through the vascular walls, embolic infarction, brain abscesses, and metastases from high-
causing hemorrhagic transformation or direct extension ly cellular tumors [19]. Option B is not the best response.
into the parenchyma. This vasculopathy-mediated septic Neither involvement of the middle cerebellar peduncle
infarction has regional vulnerability to basal ganglia or nor leptomeningeal involvement is a typical finding for dis-
thalami, in addition to the corticomedullary junction. The seminated aspergillosis. Options C and D are not the best
predilection to basal ganglia and thalami indicates involve- responses.
ment of the lenticulostriate and thalamoperforating arter- Involvement of the paranasal sinuses is often seen in dia-
ies. Aspergillosis often destroys the internal elastic lamina betic patients who have angioinvasive mucormycosis. Mu-
of the cerebral arteries. Perforating vessels are the first ones cormycosis is a rare opportunistic infection caused by ubi­
to lose their patency because of their narrow diameter. quitous fungi typically found in soil or dust. The route of
MRI characteristics of disseminated aspergillosis in- infection is usually rhinocerebral and is commonly seen in
volvement of the brain in 18 patients was reported by De- patients with uncontrolled diabetes, which is often associ-
Lone et al. [16] and others [17, 18]. Those authors reported ated with metabolic acidosis or ketoacidosis [20]. Mucormy-
that the typical MRI appearance is a predilection to basal cosis can spread from the paranasal sinuses to the brain in a
ganglia or thalami. Enhancement was minimal or absent. few days. Treatment should include aggressive débridement
Lack of enhancement is most likely related to the host’s and IV amphotericin B. Option E is not the best response.
immune capacity. Severely immunocompromised patients
have no or little immune capacity to react to an infectious Conclusion
organism to form capsule or inflammatory response; thus, Rapid progression, early ischemic manifestation, and
lack of enhancement may indicate poor prognosis and rapid predilection for the perforating arteries are characteristic
dissemination of angioinvasive aspergillosis. Option A is the features of disseminated aspergillosis infection in severely
best response. immunocompromised patients. Diagnosis should be made
Marked bright signal on diffusion-weighted images in this when clinical suspicion is high so that aggressive IV anti-
patient likely reflects infarction and cytotoxic edema. Numer- fungal therapy can be initiated.

Scenario 6 ter. Diffusion-weighted images also showed an area of hy-

Clinical History perintensity in the medial thalami.
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- QUESTION 18
sea. Abdominal CT and pelvic sonography were negative. Which of the followings is LEAST likely to be a
The patient later developed slurred speech and confusion. risk factor for Wernicke’s encephalopathy?
Brain MRI was performed (Figs. 6A–6D).
A. Chronic alcoholism.
Description of Images B. Prolonged parenteral nutrition without a vitamin
FLAIR images showed bilateral symmetric hyperinten- supplement.
sity involving the mamillary bodies, the medial thalami C. Hyperemesis gravidarum.
along the third ventricle, and the periaqueductal gray mat- D. Gastrectomy.
E. Anorexia nervosa.

What is the diagnosis given the imaging Which one of the following is a characteristic
abnormality and clinical presentations? clinical feature of Wernicke’s encephalopathy?
A. Creutzfeldt-Jakob disease. A. Learning disability.
B. Leigh disease. B. Rigidity and tremor.
C. Wernicke’s encephalopathy. C. Visual hallucination.
D. Maple syrup urine disease. D. Nystagmus and bilateral lateral rectus paralysis.
E. Wilson’s disease. E. Global ataxia.

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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 Creutzfeldt-Jakob disease (CJD) is a rare neurodegenera-

Brain MRI shows T2 hyperintensity predominantly in- tive disorder that is currently thought to be caused by an
volving the medial thalami, periaqueductal gray matter, and abnormal protein called “prion.” Pathologically, CJD leads
mamillary bodies. The distribution of signal abnormalities to spongiform encephalopathy. Patients with CJD often
on MRI and a history of prolonged emesis make Wernicke’s present with progressive dementia. CJD is divided into
encephalopathy the most likely diagnosis. Wernicke’s en- three types: sporadic CJD, with no known risk factors, the
cephalopathy is a severe neurologic disorder caused by thia- most common type in the United States; hereditary CJD,
mine (vitamin B1) deficiency. It is a disabling and potentially associated with genetic mutation; and acquired CJD, which
lethal condition that can be prevented or reversed if treated is acquired by medical procedures such as corneal trans-
early. It is often unrecognized and is likely more prevalent plantation, human growth hormone injection, and so forth.
than reported. Wernicke’s encephalopathy can progress to a Bovine spongiform encephalopathy (BSE) or mad cow dis-
state of chronic amnesia called “Korsakoff ’s syndrome.” Pa- ease, is called “variant CJD,” and is believed to be due to
tients suffering from Wernicke’s encephalopathy show de- the ingestion of infected beef. The signal abnormality on
generation of the diencephalic regions, specifically the ma- FLAIR and diffusion images is seen in the cerebral cortex,
millary bodies and the medial thalamic nuclei along the striatum, and posterior thalami. Mamillary bodies are not
mammillothalamic tract. Option C is the best response. usually involved in CJD. Option A is not the best response.

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Anzai and Ghodke

Leigh disease is a mitochondrial disorder caused by pyru- are nystagmus, bilateral lateral rectus palsies, and conjugate
vate carboxylase deficiencies. It leads to necrotizing en- gaze palsies reflecting involvement of the oculomotor and ab-
cephalomyelopathy. Leigh disease often affects the putami- ducens nerves. Option D is the best response. Gait ataxia is
na bilaterally, other deep gray matter structures, and the believed to be due to focal midline degeneration of the supe-
brain stem [21]. Option B is not the best response. rior vermis, as opposed to global ataxia, a sign of cerebellar
Maple syrup urine disease results from severe inherited dysfunction. Option E is not the best response. Cerebellar
defects in branched-chain amino acids. Patients are unable testing with the finger-to-nose or heel-to-shin test may not
to catabolize branched-chain amino acids (leucine, isoleu- elicit any notable deficit. Vestibular dysfunction without
cine, and valine), which are increased in the blood and urine. hearing loss is also a common finding.
The primary therapy is a protein-restricted diet. MRI find- Rigidity and tremor as well as bradykinesia and postur-
ings are white matter involvement in the cerebellum, the al instability are common symptoms seen in patients with
periaqueductal gray matter along the dorsal midbrain, the Parkinson’s disease, not in patients with Wernicke’s enceph-
cerebral peduncle, and the basal ganglia and thalami. Ma- alopathy. Visual hallucination is associated with psychiat-
millary bodies are not involved. Patients with maple syrup ric disorders and drugs, particularly alcohol. Patients with
urine disease present as newborns or in infancy. Option D is schizo­phrenia often have visual and, more often, auditory
not the best response. hallucinations. Learning disability refers to a group of dis-
Wilson’s disease is an autosomal recessive disorder. The Wil- orders affecting academic and functional skills, including the
son’s disease gene is mapped to chromosome 13. The main fea- abilities to listen, speak, write, read, and organize informa-
ture is accumulation of copper in the tissues, predominantly tion. It is not specific for Wernicke’s encephalopathy. Thus,
in the cornea, brain, and liver. A suppressed level of cerulo- options A, B, and C are not the correct responses.
plasmin is observed in more than 80% of patients. Clinical
symptoms include dysarthria, dystonia, rigidity, and ataxia. Conclusion
MRI findings in Wilson’s disease are signal abnormality in the Wernicke’s encephalopathy is a severe medical emergency
lentiform nucleus and the thalami as well as tegmentum of that is often associated with malnutrition states. It is an un-
the midbrain, red nuclei, and substantia nigra. Periaqueduc- derdiagnosed disease that can be reversed or treated with IV
tal gray matter involvement has been reported in Wilson’s thiamin. Wernicke’s encephalopathy should be considered in
disease. Mamillary bodies are not normally abnormal in Wil- patients with chronic alcohol abuse and malnutrition or pro-
son’s disease. Option E is not the best response. longed vomiting along with acute confusion, ataxia, oculo-
motor abnormalities, and memory disturbance. Brain MRI
Solution to Question 18 should be ordered to assess changes in the patient’s mental
Wernicke’s encephalopathy is a neurologic disorder with status. It is important for radiologists to recognize this dis-
acute onset. It is caused by a thiamine deficiency due to ease so that appropriate treatment is initiated immediately.
poor oral intake in chronic alcoholics, food refusal in an-
orexia nervosa, or recurrent vomiting in pregnant patients. References
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