成人烟雾病临床特征分析

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-arteriosynangiosis， 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。

Clinical Features of Moyamoya Disease in Adults

Objective To explore the clinical features in adult patients with moyamoya disease. Methods The age of onset, gender and clinical features of 244 Chinese adult patients with moyamoya disease were analyzed and compared with the data of the patients with moyamoya disease in Korea, Japan and North America. Results In the 244 patients, the age was from 18 to 72 years old. The mean age was 36 years. The male and female ratio was 1:1.03, which 120 were females and 124 were males. The initial symptom was cerebral ischemia in 187 patients and cerebral hemorrhage in 57 patients. All the patients came from 28 different provinces. Patients from Henan and Hebei province were 34 and 30 cases, which the proportion was the most. All the patients had digital subtraction angiography (DSA)/ magnetic resonance angiography(MRA) examination, and according to Suzuki stage, the I to VI stage was in 24, 96, 112, 152, 80 and 24 hemispheres respectively. In our cases, 244 patients underwent encephaloduro- arterio-synangiosis (EDAS) operation. After the operation, 57 cases that the initial symptom was hemorrhage had no rebleeding, and 161 cases in 187 patients that the initial symptom was ischemia had relief obviously, 26 cases no marked change. Conclusion Adult patients in China with moyamoya disease present with different clinical features compared with other countries. These observations may indicate the various mechanisms involved in moyamoya disease in China. EDAS is an effective method for the adult patients with moyamoya disease.