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Kyoto University Graduate School of Medicine, Kyoto, Japan.
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Editor’s note: Moyamoya disease (MMD), an idiopathic steno-occlusive cerebrovascular disease, is an intriguing condition defined by an angiographic appearance rather than by a pathological process. It is seen occasionally in adults in the West as a cause of otherwise cryptogenic stroke, but is more common in the South-East Asian population, where it is an important cause of stroke, especially in childhood (where it may account for a quarter of such cases).