目的 探讨烟雾病合并后循环病变的流行病学及临床特征,以提高对烟雾病的认识.方法 回顾性分析我科2002年1月～2010年1月收治的烟雾病合并后循环病变252例的临床资料.结果 252例中＜10岁46例(18.3％),10 ～ 18岁47例(18.6％),≥18岁159例(63.1％),年龄分布上有两个发病高峰,最高峰位于10～14岁,另一高峰位于40～44岁.男女发病年龄无明显差异.地域分布中,河南省57例,山东省42例,河北省35例,其余分布于其他27个省、直辖市.19例(7.5％)有家族遗传史.本组以短暂性脑缺血发作、脑梗死、头痛、头晕、脑出血、癫痫等为首发症状,均经全脑血管造影或磁共振动脉造影(MRA)确诊.结论 烟雾病合并后循环病变流行病学及临床特征独特,遇到疑似病例可行全脑血管造影或MRA等检查,以助诊断.%Objective To explore the epidemiological and clinical characteristics of moyamoya disease complicated with posterior circulation lesions and to improve awareness of moyamoya disease. Methods The data of 252 patients with moyamoya disease complicated with posterior circulation lesions during Jan. 2002 and Jan. 2010 were retrospectively analyzed. Results Among the 252 patients, 46 patients were less than 10 years old (18.3% ), 47 patients were 10 - 18 years old (18.6% ), 159 patients were older than or e-qual to 18 years old (63.1% ). There were two disease peaks in the age distribution, with the highest peak at 10 to 14 years of age, another peak at 40 to 44 years of age. There were no significant differences l>etween the male and female patients in terms of the age distribution curves. In distribution of the regions, 57 patients were from Henan Province, 42 patients from Shangdnng Province and 35 patients from Hebei Province. The remaining patients were distributed among the other 27 provinces and municipalities. 19 patients (7.5% ) had familial inheritance history. This group of patients were first admitted for with TIA, cerebral infarction, headaches, dizziness, cerebral hemorrhage, and epilepsy. They were identified by the cerebral angiogram or MR A tests. Conclusion The epidemiology and clinical characteristics of lesions moyamoya disease complicated with posterior circulation lesions are unique. Suspected cases should take cerebral angiogram or MR A for early dijignosis and treatment.
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