烟雾病6例临床分析及文献复习

Clinical analyses of 6 patients with Moyamoya disease and relative literature review

目的探讨成人出血型烟雾病的病因、发病机制及诊断治疗方法。方法回顾性分析6例成人出血型烟雾病患者的临床资料。结果成人出血型烟雾病以突发头痛、呕吐、意识障碍及偏瘫为主要临床表现。脑血管造影显示双侧病变4例,单侧2例,6例病变区均可见异常增生小血管。经治疗,4例临床治愈。结论成人出血型烟雾病临床表现多种多样。脑血管造影是诊断成人出血型烟雾病的主要方法。

Objective To investigate the cause, pathogenesis, diagnosis and treatment of the hemorrhagic moyamoya disease in adult. Methods The clinical materials of 6 patients who were suffered from hemorrhagic moyamoya disease were analyzed retrospectively. Result The main clinical manifestations of hemorrhagic moyamoya disease were abrupt headache, vomiting, disturbance of consciousness and hemi-paralysis. On conventional cerebral angiography, bilateral arterial pathological process was observed in 4 cases and the unilateral was observed in 2 cases. In all cases, the abnormal hyperplasias of small vessels were showed at the focus area. 4 cases were clinically cured of. Conclusion The hemorrhagic moyamoya disease in adults has multiplicate clinical manifestations. Cerebral angiography is the main diagnostic method for moyamoya disease.