Moyamoya disease is a chronic, occlusive cerebrovascular disease with unknown etiology which has two peaks in age distribution, a first peak in children and the second peak in young adults . The most recent epidemiological national survey in Japan estimates that the annual number of the patients with moyamoya disease is 7,700 with the incidence rate of 5.4 per million populations, with the female/male ratio of 1.8:1 . The peaks in age distribution of moyamoya disease consist of a first peak in children and the second peak in young adults. The incidence of patients with family history was 12.1% among definitive moyamoya patients . In this chapter, the authors seek to overview the characteristics of moyamoya disease in children and young adults.
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