Chiu D, Shedden P, Bratina P, Grotta J C
Department of Neurology, Baylor College of Medicine, Houston, Tex 77030, USA.
Stroke. 1998 Jul;29(7):1347-51. doi: 10.1161/01.str.29.7.1347.
We report the clinical features and longitudinal outcome of the largest cohort of patients with moyamoya disease described from a single institution in the western hemisphere. Moyamoya disease in Asia usually presents with ischemic stroke in children and intracranial hemorrhage in adults.
Our study population included all patients with moyamoya disease evaluated at a university hospital in Houston, Texas from 1985 through 1995 (n = 35). We used Kaplan-Meier methods to estimate individual and hemispheric stroke risk by treatment status (medical versus surgical). Predictors of neurological outcome were assessed.
The ethnic background of our patients was representative of the general population in Texas. The mean age at diagnosis was 32 years (range, 6 to 59 years). Ischemic stroke or transient ischemic attack was the predominant initial symptom in both adults and children. Of the 6 patients with intracranial hemorrhage, 5 had an intraventricular site of hemorrhage. The crude stroke recurrence rate was 10.3% per year in 116 patient-years of follow-up. Twenty patients underwent surgical revascularization, the most common procedure being encephaloduroarteriosynangiosis. The 5-year risk of ipsilateral stroke after synangiosis was 15%, compared with 20% for medical treatment and 22% overall for surgery.
Our observations indicate that moyamoya disease may have a different clinical expression in the United States than in Asia, and may demonstrate a trend toward a lower stroke recurrence rate and better functional outcome after synangiosis.
我们报告了西半球单一机构所描述的最大队列烟雾病患者的临床特征及长期预后情况。亚洲的烟雾病通常在儿童中表现为缺血性卒中，在成人中表现为颅内出血。
我们的研究人群包括1985年至1995年在得克萨斯州休斯顿一家大学医院接受评估的所有烟雾病患者（n = 35）。我们使用Kaplan-Meier方法，根据治疗状态（药物治疗与手术治疗）估计个体及半球性卒中风险。评估神经功能预后的预测因素。
我们患者的种族背景代表了得克萨斯州的一般人群。诊断时的平均年龄为32岁（范围6至59岁）。缺血性卒中或短暂性脑缺血发作是成人和儿童的主要初始症状。在6例颅内出血患者中，5例出血部位在脑室内。在116患者年的随访中，粗略的卒中复发率为每年10.3%。20例患者接受了手术血管重建，最常见的手术是脑硬脑膜动脉血管融合术。血管融合术后同侧卒中的5年风险为15%，药物治疗为20%，手术总体为22%。
我们的观察结果表明，烟雾病在美国的临床表现可能与亚洲不同，血管融合术后可能呈现出卒中复发率较低和功能预后较好的趋势。
