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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]
Stage | Description |
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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 |