Moyamoya Disease in China Its Clinical Features and Outcomes
Moyamoya Disease in China Its Clinical Features and Outcomes
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DOI：
10.1161/strokeaha.111.621300
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发表时间：
2012-01-01
期刊：
影响因子：
8.3
通讯作者：
Feng, Jie
中科院分区：
文献类型：
--
作者：
Duan, Lian;Bao, Xiang-Yang;Feng, Jie
背景和目的：本文描述了在中国一家医院接受手术治疗的烟雾病患者的临床特征和预后。方法：我们的队列包括802例烟雾病患者。通过回顾性图表复习获得人口学和临床特征；随访信息和结果通过临床访问、电话或信件访问获得。我们使用Kaplan-Meier方法根据治疗状态估计卒中风险。结果：出现症状的中位年龄为28岁（范围0.5 ~ 77岁）。在年龄分布上发现了两个明确的高峰。男女比例为1:1（398/404）。家族性烟雾病发生率为5.2%。分别有564例、113例和125例患者的初始症状为缺血、出血或其他症状。802例患者中29例（3.6%）接受保守治疗。其余773例（96.4%）患者接受了神经外科血管重建术，其中502例为双侧。手术（n=773）或保守治疗（n=26）后的中位随访时间为26.3个月（范围为6.0-101.9个月）。大多数随后的缺血事件发生在手术后的头2年。Kaplan-Meier估计前2年卒中风险为10.1%，所有接受手术血运重建术的患者术后5年卒中Kaplan-Meier风险为12.7%。结论：本研究对中国大陆地区烟雾病的临床特征进行了研究，其发病率呈双峰分布，男女比例为1:1，成人出血率低于儿童。患者术后缺血性或出血性卒中发生率低，大多数患者在血运重建术后仍保持功能状态。(中风。2012;43:56-60)。
Background and Purpose-Here we describe the clinical features and outcomes of patients with moyamoya disease who were surgically treated at a single institution in China.Methods-Our cohort included 802 patients with moyamoya disease. Demographic and clinical characteristics were obtained by retrospective chart review; follow-up information and outcome were obtained through clinical visits, telephone, or letter interview. We used the Kaplan-Meier methods to estimate stroke risk by treatment status.Results-The median age for the onset of symptoms was 28 (range, 0.5-77) years. Two definite peaks in age distribution were found. The ratio of women to men was 1: 1 (398/404). Familial occurrence of moyamoya disease was 5.2%. The initial symptom was ischemia, hemorrhage, or others in 564, 113, and 125 patients, respectively. Twenty-nine of the 802 patients (3.6%) received conservative management. The remaining 773 patients (96.4%) underwent neurosurgical revascularization procedures, and 502 of these were bilateral. The median follow-up after surgery (n=773) or conservative management (n=26) was 26.3 months (range, 6.0-101.9 months). Most subsequent ischemic events appeared in the first 2 years after surgery. The Kaplan-Meier estimated stroke risk was 10.1% in the first 2 years, and the 5-year-Kaplan-Meier risk of stroke was 12.7% after surgery for all patients treated with surgical revascularization.Conclusions-This study on the clinical features of moyamoya disease in mainland China indicated bimodal incidence distribution with women-to-men ratios of 1: 1 and lower rate of hemorrhages in adults compared with in children. Patients had low rates of postoperative ischemic or hemorrhagic strokes, and the majority of patients had preserved functional status after revascularization. (Stroke. 2012;43:56-60.)