Moyamoya disease

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Cerebrovascular disease of uncertain aetiology characterised by progressive stenosis of both internal carotid arteries with accompanying collateral formation. The diagnosis is primarily based on angiographic appearances of these collaterals which resembles wisps or a mist of smoke, moyamoya (もやもや) in Japanese, from which the disease derives its name.[1][2] The collateral vessels may bleed, resulting in intra-cranial haemorrhage, or in less severe cases, pseudo-aneurysms.


  1. Kudo T, Takayama R, Mikawauchi K. Occlusion of the internal carotid artery (Japanese). Proceedings of 15th Annual Meeting of Japan Neurosurgical Society 1956.
  2. Takeuchi K, Shimizu K. Hypoplasia of the bilateral internal carotid arteries. Brain Nerve 1957;9:37-43.
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