Moyamoya病是一种以双侧颈内动脉远端进行性狭窄或闭塞为特征的慢性脑血管闭塞病。脑底异常血管网(即Moyamoya血管)是继发于脑进行性缺血后形成的侧支循环。组织病理学发现,其主要血管改变为血管内膜的纤维细胞增厚所致的狭窄或闭塞。对于Moyamoya病的病因目前尚不明确,但有家族史的群体发病率高,提示可能与遗传因素有关。 Moyamoya disease is a chronic cerebrovascular occlusive disease characterized by progressive stenosis or occlusion of the distal bilateral internal carotid artery. The abnormal brain network (ie, Moyamoya blood vessels) is a collateral circulation secondary to brain ischemia. Histopathology revealed that the major vascular change was stenosis or occlusion due to thickened fibrocytes in the intima. The cause of Moyamoya disease is not yet clear, but the high incidence of family history of the group, suggesting that genetic factors may be related.