烟雾病医学知识宣讲讲义.ppt

A normal B 1-2 narrow of ICA C 3-4 significant narrowing of the ICA and characteristic puff-of –smoke collaterals D 5-6 obliteration of the ICA Patients with the characteristic moyamoya vasculopathy who also have wellrecognized associated conditions (described below) are categorized as having the moyamoya syndrome, whereas patients with no known associated risk factors are said to have moyamoya disease. By definition, the pathognomonic arteriographic findings are bilateral in moyamoya disease, although the severity can differ between sides. Patients with unilateral findings have the moyamoya syndrome, even if they have no other associated risk factors. However, contralateral disease eventually develops in up to 40% of patients initially presenting with unilateral findings. When used alone, without the distinguishing modifier of “disease” or “syndrome,” “moyamoya” refers solely to the distinctive findings on cerebral arteriography, independently of the cause. 烟雾病医学知识宣讲 定义 烟雾病(moyamoya disease ,MMD) 又称脑底异常血管网，是一组以颈内动脉虹吸部及大脑前、中动脉起始部（前循环）狭窄或闭塞，脑底出现异常的小血管网为特点的脑血管病。因在脑血管造影时呈现许多密集成堆的小血管影似吸烟吐出的烟雾故名。 1955 年,日本学者首先报道了MMD 的脑血管造影情况 1969 年,Suzuki 和Takaku 根据脑血管造影所表现出来的形态将其命名为Moyamoya 病 日语中“moyamoya”一词的含义有烟雾弥漫、模糊的意思,患者脑底异常血管网在造影中显示正如“烟雾”一样 全世界国家均有发现,但主要发生在日本及亚洲国家。 病因 遗传和环境因素 炎症后的自身免疫性反应可能 导致脑血管的闭塞性病变 病理 病变血管 血管内膜明显增厚, 内弹力层弯曲, 间质萎缩变薄, 血管壁上出现附壁血栓及脂质沉积 等现象。 发病年龄 Moyamoya出现在两个年龄段：小于10岁的儿童和30岁左右的成人。 儿童经常出现头痛，癫痫，行为障碍、反复轻偏瘫发作和相对少见的蛛网膜下腔出血。 成人多引起出血或中风。 血管闭塞的过程一旦开始，就倾向于持续恶化，除非使用外科手术治疗。反复的中风可以引起严重的功能障碍甚至死亡。 病因 (一)发病原因虽然以前有学者发现个别家族中母子或兄妹，或孪生姐妹有类似疾病，考虑与先天因素有关，但目前根据临床，病理，免疫及动物实验研究可以认为本病是一组后天获得性闭塞性脑血管病，据文献报道多种疾病均可引起本病，但最多见的是原发于脑底动脉的闭塞性脑血管病。目前有关此病的病因尚不完全清楚，多数学者认为烟雾病由先天和后天因素在内的各种因素引起，或在原有特殊遗传素质基础上由后天因素作用促发而成的脑血管病，概括起来有以下两种观点： Effects of Surgical Revascularization in Moyamoya. Angiographic images obtained 1 year after surgical treatment of moyamoya with pial synangiosis show internal (Panel A) and external (Panel B) carotid injections. There is abundant filling of the midd