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Moyamoya Disease:MR Findings J Comput Assist Tomogr 13:720-721, Brown,W.D.,et al, 1989 See this aricle in Pubmed Article Abstract Moyamoya disease is an uncommon entity first described in Japanese children and young adults,primarily in females;it has subsequently been demonstrated in patients of many ethnic groups and up into the fifth decade of life.The most characteristic finding is a tangle of hypertrophied diencephalic vessels,with a"puff of smoke"(Japanese moyamoya)(1)angiographic appearance,which gives the condition one of several names found in the literature.These vessels supply collateral flow to the brain after progressive occlusion of one or both supraclinoid internal carotid arteries,generally early in life(2).Several specific etiologies for such occlusions have been documented(3),though most cases are idiopathic.Children with moyamoya disease usually present with an ischemic event;young adults most often present with subarachnoid hemorrhage(2).The severity of the prognosis is usually inversely proportional to the patient's age at presentation(4).Diagnosis of moyamoya has required angiography,but early experience with magnetic resonance(MR) imaging indicates that this modality may be helpful in suggesting the diagnosis.We present a patient in whom the diagnosis was initially determined from MR images.

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