Abstract: Moyamoya disease (MMD) is a rare cerebral vascular disease. And it is characterizedby the stenosis or even occlusive patent. The cardinal and basic pathological change is the fibercell-alike intimal thickening at the stenosis part of the cerebral artery, accompanied by the thinnedsmooth muscular layer and proliferated intimal elastic layer. Usually, the terminal ICA and its mainbranches, such as the ACA and MCA are the most common lesions. However, further researchesrevealed that the pulmonary artery and renal artery may have similar changes. Besides, the MCAwere proved with significant apoptosis.

Key words: Moyamoya disease; Pathology; Blood vessels; Apoptosis

摘要： 烟雾病（Moyamoya Disease，MMD）是一种少见的狭窄、闭塞性脑血管疾病，基本病理改变主要为颅内狭窄段动脉的内膜呈纤维细胞性增厚，内弹力膜增生分层，中膜平滑肌层变薄。经典的病变部位为颈内动脉（Interal Carotid Artery，ICA）末端以及其主要分枝的起始段，例如大脑前动脉（anterior cerebral artery，ACA），大脑中动脉（Middle Cerebral Artery，MCA）。随着相关病理学的进展，更多MMD血管病理改变被发现，例如脑血管系统之外的肺动脉、肾动脉也存在特征性病理改变。MCA被证实存在明显的细胞凋亡。这些病理改变的研究，有助于最终探明烟雾病的病因。

关键词: 脑底异常血管网病; 病理学; 血管; 细胞凋亡