【摘要】 目的 研究不同亚型多系统萎缩（multiple system atrophy，MSA）患者的临床特点。 方法 回顾分析2009年1月—2011年1月收治的105例“很可能的”MSA患者的临床资料，包括发病年龄、首发症状、临床表现、治疗反应性等。 结果 105例MSA患者中，男57例，女48例，发病年龄58岁。以小脑性共济失调为主要特点的MSA（MSA with predominant cerebellar ataxia，MSA-C）患者76例，以帕金森综合征为主要特点的多系统萎缩（MSA with predominant parkinsonism，MSA-P）患者29例。39例患者仅以小脑功能障碍为首发症状；29例患者仅以帕金森综合征为首发症状，23例患者仅以自主神经功能障碍为首发症状，其余14例患者的首发表现至少包括2种症状组合。至最后一次随访时，54例患者同时存在小脑功能障碍、帕金森综合征、自主神经功能障碍和锥体束征，51例患者表现为自主神经功能障碍与小脑功能障碍和（或）帕金森综合征的不同形式的组合。 结论 MSA患者以MSA-C为主。由于在病程早期，MSA与其他帕金森综合征或小脑性共济失调疾病的鉴别较为困难，因此，仔细动态观察患者临床特点的演变情况，对MSA的诊断至关重要。【Abstract】 Objective To investigate subtypes and clinical features of multiple system atrophy (MSA). Methods The clinical data of 105 probable MSA patients treated in our hospital from January 2009 to January 2011 were analyzed, including the age at onset, initial symptoms, clinical manifestations and responsivity to levodopa. Results The 105 probable MSA patients consisted of 57 males and 48 females, including 76 patients (72.4%) of MSA with predominant cerebellar ataxia (MSA-C) and 29 patients (27.6%) of MSA with predominant parkinsonism (MSA-P). The mean age at onset was 58 years. The initial symptom of 39 patients was pure cerebellar dysfunction. Twenty-nine patients presented pure parkinsonism as the initial symptom. The initial symptom of 23 patients was pure dysautonomia. By the last clinical visit, 54 patients had cerebellar dysfunction, parkinsonism, autonomic failure and pyramidal signs. Conclusion The study revealed a predominance of MSA-C patients. The differentiation of MSA and other forms of parkinsonism or cerebellar ataxia may be difficult at the early stage. For more accurate diagnosis, it is important to carefully observe the clinical progression.