Moyamoya syndrome

Atherothromboembolism ntracranial small vessel disease Leukoaraiosis Dissection (Table 6.4) Fibromuscular dysplasia Congenital arterial anomalies Moyamoya syndrome Embolism from arterial aneurysms Inflammatory vascular diseases Irradiation... 

Infections infective endocarditis, herpes simplex, leptospirosis, anthrax Sickle cell disease Moyamoya syndrome (Ch. 6)... 

Moyamoya syndrome (Ch. 6) causes gradual stenosis or occlusion of the terminal portions of the internal carotid arteries or middle cerebral arteries. This leads to formation of an abnormal collateral network of fragile vessels, which occasionally rupture. It has been proposed that constructing a bypass to relieve the pressure on the collaterals would be beneficial, for example between the superficial temporal artery and the middle cerebral... 

Hereditary hemorrhagic telangiectasia" Moyamoya syndrome Klinefelter s syndrome Progeria... 

Moyamoya seems to be mainly confined to the Japanese and other Asians, and in most cases the cause is unknown (Bruno et al. 1988 Chiu et al. 1998). Some cases are familial (Kitahara et al. 1979) others appear to be caused by a generalized fibrous disorder of arteries (Aoyagi et al. 1996), and a few may result from a congenital hypoplastic anomaly affecting arteries at the base of the brain, or associated with Down s syndrome (Cramer et al. 1996). The syndrome may present in infancy with recurrent episodes of cerebral ischemia and infarction, mental retardation, headache, epileptic seizures and, occasionally, involuntary movements. In adults, subarachnoid or primary intracerebral hemorrhage are also common owing to rupture of collateral vessels. There have also been a few reports of associated intracranial aneurysms (Iwama et al. 1997) and also of cerebral arteriovenous malformations. 

Cramer SC, Robertson RL, Dooling EC et al. (1996). Moyamoya and Down syndrome. Clinical and radiological features. Stroke 27 2131-2135... 

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