成人出血型烟雾病自然病史研究  

Study on Natural History of Adult Patients with Hemorrhagic Moyamoya Disease

作　　者：刘兴炬[1,2,3] 康帅 张东[1,2,3] 王嵘 张岩[1,2,3] 张谦 赵继宗[1,2,3] 

机构地区：[1]首都医科大学附属北京天坛医院神经外科 [2]国家神经系统疾病临床研究中心 [3]北京脑重大疾病研究院脑卒中研究所,北京100050

出　　处：《中国卒中杂志》2017年第11期995-999,共5页Chinese Journal of Stroke

基　　金：国家"十三五"科技支撑计划:缺血性脑血管病和脑肿瘤关键技术研究(2015BAI12B04);北京市优秀人才青年项目(D161100003816005);国家自然科学基金青年项目(81701137)

摘　　要：目的探讨成人出血型烟雾病的长期自然病史及预后相关危险因素。方法回顾性分析115例成人(>17岁)出血型烟雾病患者临床资料,对其中40例行保守治疗患者进行长期随访,主要观察指标为新发脑出血和死亡事件。结果 40例保守治疗的患者中有6例失访,34例累计随访398.7年,平均随访(11.7±4.6)年,15例(44.1%)出现21次脑再出血,平均每年出血率5.3%。5例(33.3%)死于再出血,4例有严重后遗症[改良Rankin评分(modified Rankin Scale,m RS)≥3]。年再出血率前5年为2.6%,10年后为7.9%。年龄大于35岁的患者再出血风险高于年龄低于35岁者[风险比(hazard ratio,HR)2.8,95%可信区间(confidence interval,CI)1.1~7.7,P=0.04]。患者性别、出血部位、数字减影血管造影(digital subraction angiography,DSA)分期、高血压、烟雾病家族史以及是否合并脑动脉瘤与再出血不相关。结论成人出血型烟雾病再出血风险随着时间延长明显增加;年龄大于35岁患者再出血风险偏高。Objective To explore the risk factors related with the long term natural history and outcome of adult patients with hemorrhagic moyamoya disease（MMD）.Methods A retrospective analysis was made upon the clinical data of 115 cases of adult patients（age17） with hemorrhagic MMD. Long-term follow-up study was conducted on 40 patients who received conventional therapy. The main observation indexes were newly onset cerebral hemorrhage and death event.Results Among 40 patients, who received conventional therapy, 6 cases were losted and 34 cases were followed up cumulatively for 398.7 person-years. The median follow-up time was（11.7±4.6） years. During 398.7 person-years, 15 patients experienced（44.1%） twenty-one episodes of rebleeding, rendering an average annual incidence of 5.3%. Among them, 5 patients（33.3%） died from rebleeding and 4 patients had serious disability（modified Ranking Scale ≥3; 26.6%）. The annual risk of rebleeding increased gradually from 2.6% in the first five years to 7.9% after 10 years after the first bleeding. Patients older than 35 years sustained a higher risk of rebleeding than ones under the age of 35 [hazard ratio（HR） 2.8, 95% confidence interval（CI） 1.1-7.7, P=0.04]. Gender, bleeding location, DSA stage, hypertension, family history of MMD and whether there were complication with aneurysm were not associated with the risk of rebleeding.Conclusion The risk of rebleeding of adult patients with hemorrhagic MMD obviously increased as time went on; and patients older than 35 years had a higher risk of rebleeding.

关 键 词：烟雾病 自然病史 脑出血 再出血 

分 类 号：R743[医药卫生—神经病学与精神病学]