AJR:191, September 2008 S3
Nonaumai Emegen Neuoadiology
Solution to Question 1
Arteriovenous malformation (AVM) is not an unusual causeof 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. CTangiography often reveals a tangle of markedly dilated arter-ies and veins with a nidus of AVM. If the AVM is small, it maynot be visualized or diagnosed correctly with CT angiography.
In this setting, cerebral angiography is the most denitive test.
However, cerebral angiography in this patient did not show
early venous lling 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 smallvessels, are often centered at the basal ganglia, particularlythe putamen and the external capsule; the thalamus; thepons; 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 inolder patients. Option B is not the correct response.Certain types of brain tumors are often associated withintracranial hemorrhage. These are often aggressive tumors,such as glioblastoma multiforme, or vascular tumors, such asmetastases 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 signicant mass effect
or vasogenic edema. Option C is not the best response.Vasculitis is one of the causes of intracranial hemorrhageand is more frequently seen in young patients. Vasculitis canpresent with acute hemorrhage or ischemic symptoms. Manycauses of vasculitis affect small- to medium-sized vessels;therefore, CT angiography may not show an irregularity of
the blood vessels as denitively as cerebral angiography. Cere
-bral angiography in Figures 1C and 1D shows a classic ap-pearance of segmental narrowing and poststenotic dilatationinvolving multiple small- to medium-sized blood vessels, con-sistent with vasculitis.
Option D is the best response.
Amyloid angiopathy often causes a lobar intracranialhemorrhage centered at the deep white matter in the frontalor parietal lobes. Amyloid angiopathy is much more commonin 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 othercauses in elderly patients. Option E is not the best response.
Solution to Question 2
The causes of vasculitis are often divided into infectiousand noninfectious. Infectious causes include tuberculosis, fun-gal infection, bacterial vasculitis, and HIV vasculitis. The fre-quent causes of noninfectious vasculitis are immune-mediatedvasculitis such as rheumatoid arthritis and systemic lupus er-ythematosus, and granulomatous disease, such as Wegener’sgranulomatosis and sarcoid, as well as giant cell arteritis (alsocalled temporal arteritis). Drugs, particularly amphetaminesand cocaine, are frequent causes of noninfectious vasculitis.
An amphetamine causes inammatory vasculitis with vascu
-lar wall necrosis and subsequent hemorrhage. The pathologicfeatures of amphetamine-related vasculitis are similar tothose of polyarteritis nodosa. This patient had taken amphet-amines for several years. Cocaine, on the other hand, inducescerebral infarction or ischemia as well as hemorrhage by vaso-constrictive effect and increased platelet aggregation, rather
than a vasculitis-type inammation 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 disproportionallylong limbs and tall stature. It affects the heart and aortaand causes aortic root dilatation, aortic regurgitation, anddissection. 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 insensitivefor the diagnosis of CNS vasculitis. CT angiography is lessaccurate than cerebral angiography. Cerebral angiography isused as the gold standard for diagnosis at many institutions.
When characteristic angiographic ndings such as alternat
-ing areas of stenosis and dilatation or a beading appearanceare 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 specicity 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 supercial temporal artery as a
screening tool for temporal arteritis. However, transcranialDoppler sonography is not suitable to evaluate arteries fullyto diagnose CNS vasculitis. Options A, B, D, and E are notthe best responses.Biopsy of CNS tissue would logically be considered theultimate gold standard of diagnosis, but clearly the proce-dure is limited by several factors. It is highly invasive andcarries certain risks. Successful biopsy requires a willingand experienced neurosurgeon, who may not be readilyavailable. Sampling error could result in limited sensitivity.The site of biopsy should be tailored to the individual pa-
tient [2]. The biopsy of the supercial temporal artery is
often performed in patients suspected of having giant cellvasculitis. The false-negative rate of biopsy for a diagnosis
of vasculitis has been reported to be 16%, yielding a sensi
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