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Moyamoya and Down Syndrome:Clinical and Radiological Features Stroke 27:2131-2135, Cramer,S.C.,et al, 1996 See this aricle in Pubmed Article Abstract The clinical and radiological features of primary moyamoya disease overlap with those of MM-DS.Hemiplegia and aphasia were the most common presentations.Motor recovery was excellent in five of seven cases.Cerebral infarcts were superficial or deep and can occur in a watershed distribution.Angiography demonstrated involvement of the internal carotid artery and its branches bilaterally in all seven cases and the posterior cerebral arteries in four cases.The clinical and radiological features of MM-DS overlap with primary moyamoya disease.We postulate that a protein encoded on chromosome 21 may be related to the pathogenesis of moyamoya disease.Although the neuronal substrate is abnormal in Down syndrome patients,recovery from hemiplegic stroke in patients with MM-DS is comparable to recovery in patients with primary moyamoya.

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