Moyamoya Disease (definition)

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Moyamoya Disease

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Definition: A noninflammatory, progressive occlusion of the intracranial CAROTID ARTERIES and the formation of netlike collateral arteries arising from the CIRCLE OF WILLIS. Cerebral angiogram shows the puff-of-smoke (moyamoya) collaterals at the base of the brain. It is characterized by endothelial HYPERPLASIA and FIBROSIS with thickening of arterial walls. This disease primarily affects children but can also occur in adults.

Other names Progressive Intracranial Occlusive Arteropathy (Mo; Cerebrovascular Moyamoya Disease; Secondary Moyamoya Disease; Primary Moyamoya Diseases; Primary Moyamoya Disease; Moyamoya Diseases, Primary; Moya Moya Disease; Disease, Primary Moyamoya; Disease, Moya-Moya; Disease, Classic Moyamoya; Classic Moyamoya Disease; Moyamoya Syndrome; Moyamoya Disease, Secondary; Moyamoya Disease, Primary; Moyamoya Disease, Classic; Moya-Moya Disease

Substance	CAS Registry & name	Categories	Source
RNF213 protein, human	EC 6.3.2.19	*Ubiquitin-Protein Ligases Moyamoya Disease RING Finger Domains.	J Hum Genet 2011 Jan;56(1):34-40
Moyamoya disease 3	0	*Moyamoya Disease.
Moyamoya disease 2	0	*Moyamoya Disease.
Moyamoya disease 1	0	*Moyamoya Disease.

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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