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目的探讨244例成人烟雾病(Moyamoya病)的临床特征。方法分析244例成人Moyamoya病患者的发病年龄、性别及临床症状,并与日韩及北美Moyamoya病患者进行比较分析。结果244例病例年龄为18岁～72岁,平均36岁,其中男124例,女120例,男女比例为1∶1.03;以脑出血为首发症状入院的57例,187例以脑缺血为首发症状入院。本组患者来自我国28个不同省市,河南省34人、河北省30人,所占病例数最多。全部患者均行数字减影血管造影(DSA)和磁共振血管成像(MRA)检查,血管成像按照铃木分期,其中1期24侧半球,2期96侧半球,3期112侧半球,4期152侧半球,5期80侧半球,6期24侧半球。本组244例Moyamoya病患者行硬脑膜动脉血管融通术(encephalo-duro-arterio-synangiosis,EDAS),57例出血发病的病例均未出现再出血。187例缺血发病的病例中症状明显缓解的161例,26例无明显变化。结论本研究样本显示的Moyamoya病临床特征与日本、韩国及北美有所不同,即出血型病例发病率较低而脑梗死发病率明显较高。其发病机制是否有所不同需进一步深入研究;EDAS手术是治疗成人Moyamoya病的有效方法。
Objective To investigate the clinical features of 244 adult moyamoya disease (Moyamoya disease). Methods The onset age, gender and clinical symptoms of 244 adults with Moyamoya disease were analyzed and compared with Moyamoya disease patients in Japan, Korea and North America. Results A total of 244 cases were aged from 18 to 72 years, with an average of 36 years old, including 124 males and 120 females, with a ratio of 1: 1.03. Among them, 57 were admitted with cerebral hemorrhage as their first symptom and 187 were cerebral ischemia Symptoms were admitted. This group of patients from 28 different provinces and cities in our country, Henan Province, 34 people, 30 people in Hebei Province, the largest number of cases. All patients underwent digital subtraction angiography (DSA) and magnetic resonance angiography (MRA). Angiography was performed in accordance with Suzuki staging. One of 24 hemispheres, two 96-hemispheres, three 112-hemispheres and four 152 Side of the hemisphere, 5 side of the 80 hemispheres, 6 24 side hemispheres. 244 patients with Moyamoya disease underwent encephalo-duro-arterio-synangiosis (EDAS), and no rebleeding occurred in 57 patients with hemorrhage. Among the 187 cases of ischemic disease, 161 cases were relieved of symptoms and 26 cases showed no significant change. Conclusions The clinical characteristics of the Moyamoya disease shown in this study are different from those in Japan, South Korea and North America, in that the incidence of hemorrhagic cases is low and the incidence of cerebral infarction is significantly higher. Whether its pathogenesis is different needs further study; EDAS is an effective method to treat Moyamoya disease in adults.