This is a non-inflammatory vasocclusive disease of ICA, MCA and ACA and is a cause of both ischaemic and to a lesser extent haemorrhagic stroke. The abnormal net-like vessels at the base of the brain seen in cerebral angiograms of this disease were described by most native speakers of Japanese as "Moyamoya," a Japanese expression for something hazy, such as a puff of cigarette smoke drifting in the air. The name was popularised by Jiro Suzuki. [Suzuki J et al. 1983]
About
Genetics
Aetiology
Associations
Clinical
Investigations
Stage Description 1 Stenosis of the suprasellar ICA usually bilateral 2 Development of Moyamoya vessels with dilatation of all main cerebral arteries at base of brain 3 Increasing ICA stenosis and prominence of Moyamoya vessels with reduction of flow in the middle and anterior cerebral arteries 4 Entire Circle of Willis and PCAs occluded. Extracranial collateralisation with minimisation of Moyamoya vessels. Proximal portions of the posterior cerebral arteries become involved 5 Reduction of Moyamoya and absence of all main cerebral arteries 6 Disappearance of Moyamoya vessels and major cerebral vessels; the cerebral circulation is supplied only by the external carotid system Management
Further Reading
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Moyamoya disease
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