多系统萎缩的临床特征与疾病进展的分析.docVIP

多系统萎缩的临床特征与疾病进展的分析 多系统萎缩的临床特征与疾病进展的分析 作者：王博，张朝东，李昭 作者单位：110001沈阳，中国医科大学附属第一医院神经内科 【摘要】 目的 探讨多系统萎缩（MSA）的临床特征与疾病进展的特点。方法 回顾性分析28例拟诊MSA患者的临床资料。用代表日常生活活动度（ADL）的3个重要事件（辅助行走、依靠轮椅、卧床状态）对疾病进展进行评估。通过KaplanMeier曲线分析各亚组间的不同。结果 3个以上系统受累26例（92.9%），普遍存在自主神经功能障碍。从起病进展为临床MSA的平均时间为2年。从起病进展为辅助行走、依靠轮椅和卧床状态的平均时间分别为3年、5年、7年。表现为运动和自主神经系统同时受累的患者在起病3年内疾病进展的危险性明显增加（P0.01）。结论 MSA是一种多系统受累的慢性进展性疾病，从起始症状到同时出现运动和自主神经功能障碍的间隔时间能够预测MSA的功能衰退。 【关键词】 多系统萎缩；临床表现，进展；日常生活活动度；诊断标准 　　An analysis of clinical features and progression of multiple system atrophy WANG Bo, ZHANG Chaodong, LI Zhao.Department of Neurology, First Hospital Affiliated China Medical University, Shenyang 110001, China Abstract：Objective To analyze the clinical features and progression of multiple system atrophy (MSA).Methods The clinic data of twentyeight subjects diagnosed as probable MSA according to Gilman diagnostic criteria were studied retrospectively. Three aspects of activities of daily living (ADL) (aidrequiring walking, wheelchairbound state and bedridden state) were used to assess the progression of disease. KaplanMeier analysis was also used to estimate the difference between subgroups.Results Three systems were involved in twentysix cases (92.9%) and autonomic functional disturbance was common. The calculated median time from onset to evolution to MSA was 2 years. The median times from onset to aidrequiring walking, wheelchair requirement and bedridden state were 3, 5 and 7 years，respectively. The patients impaired both of motor and autonomic systems progressed fastly within 3 years from onset of the disease (P0.01).Conclusion MSA is a chronic and progressive disease with multiple systems affected. The interval from initial symptom to both dysfunctions of motor and autonomic systems may predict functional deterioration in MSA. Key words：multiple system atrophy;clinical features;progression;activities of daily living;diagnostic criteria 　　多系统萎缩(MSA)是一种少见的、散发的、慢性神