Department of Neurosurgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
Stroke. 2013 Jul;44(7):1997-9. doi: 10.1161/STROKEAHA.111.000307. Epub 2013 May 7.
Moyamoya disease is an uncommon, cerebrovascular occlusive disease of unknown pathogenesis. Previously described Moyamoya cohorts include predominantly Asian populations or ethnically diverse North American cohorts. To gain further insight into the pathogenesis of moyamoya, we examined clinical characteristics of a primarily white, Midwestern US population
Retrospective analysis of patients with angiographically confirmed moyamoya disease evaluated at our institution was performed. Prevalence of comorbidities, cerebrovascular risk factors, and autoimmune diseases were compared with the general population.
Ninety-four patients with moyamoya were evaluated; 72.3% were female. Ethnic composition was primarily white (85%). A significantly higher prevalence of autoimmune disease was seen, particularly type 1 diabetes mellitus (8.5% versus 0.4% in the general population) and thyroid disease (17.0% versus 8.0% in the institutional general patient population). Hyperlipidemia was also increased (27.7% versus 16.3% in the general population).
This study of a unique, primarily white, Midwestern population of moyamoya patients demonstrates a significantly higher prevalence of autoimmune disease than in the general population. This supports a possible autoimmune component to the pathogenesis of moyamoya disease.
烟雾病是一种罕见的、病因不明的脑血管闭塞性疾病。以前描述的烟雾病队列主要包括亚洲人群或种族多样化的北美队列。为了更深入地了解烟雾病的发病机制，我们研究了主要为白人的美国中西部人群的临床特征。
对我院确诊为烟雾病的患者进行回顾性分析。将合并症、脑血管危险因素和自身免疫性疾病的患病率与一般人群进行比较。
共评估了 94 例烟雾病患者，其中 72.3%为女性。种族构成主要为白人（85%）。自身免疫性疾病的患病率显著升高，尤其是 1 型糖尿病（8.5%比一般人群中的 0.4%）和甲状腺疾病（17.0%比一般人群中的 8.0%）。高脂血症也增加（27.7%比一般人群中的 16.3%）。
这项对独特的、主要为白人的美国中西部烟雾病患者群体的研究表明，自身免疫性疾病的患病率明显高于一般人群。这支持了烟雾病发病机制中可能存在自身免疫成分的假说。
