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目的:研究12例多系统萎缩(multiple system atrophy MSA)患者的临床特点,分析诊断要点。方法:回顾性分析12例多系统萎缩病例,探讨其临床特点和早期诊断的要点。结果:本组病例均为慢性、隐袭性起病,男性居多,男:女=1.4:1。平均发病年龄49.2岁(35.0岁～72.0岁)。平均病程33个月(6个月～60个月)。MSA-P亚型4例,MSA-C亚型8例,以MSA-C亚型占优势。MSA-P亚型中以行动迟缓为主要首发表现(75%),主要体征分别为肌张力增高(100%)、直立性低血压(75%)、锥体束征(75%);MSA-C亚型以行走不稳为主要首发表现(62.5%),主要体征为共济失调(100%),直立性低血压、小脑性语言、锥体束征(分别为62.5%)。结论:多系统萎缩是一种散发性的神经系统变性疾病,成年起病,临床表现多样。对于早期单纯以帕金森综合征、小脑功能障碍或自主神经功能紊乱为主要症状的患者,特别是无家族史者,要注意甄别有无其他系统受累的症状或体征,同时结合影像学检查、左旋多巴类药物治疗反应性等临床特点,对于早期正确诊断MSA有一定帮助。
Objective: To study the clinical features of 12 patients with multiple system atrophy MSA and to analyze the main points of diagnosis. Methods: A retrospective analysis of 12 cases of multiple system atrophy, to explore the clinical features and early diagnosis points. Results: The patients in this group were all chronic, cryptogenic onset, mostly male, male: female = 1.4: 1. The average age of onset was 49.2 years (35.0 to 72.0 years). The average duration of 33 months (6 months to 60 months). MSA-P subtype in 4 cases, MSA-C subtype in 8 cases, with MSA-C subtype dominant. In MSA-P subtypes, delayed onset of brachial plexus occlusion was the main initial manifestation (75%). The main signs were hypertensive tone (100%), orthostatic hypotension (75%) and pyramidal tract sign (75% The main manifestation of C subtype was unstable walking (62.5%). The main signs were ataxia (100%), orthostatic hypotension, cerebellar language and pyramidal signs (62.5%, respectively). CONCLUSIONS: Multiple system atrophy is a sporadic neurodegenerative disease that begins with onset and has multiple clinical manifestations. For the early simple to Parkinson’s disease, cerebellar dysfunction or autonomic dysfunction as the main symptoms of patients, especially those without family history, should pay attention to screening for the presence of other symptoms or signs of systemic involvement, combined with imaging studies, levorotatory Toba class drug treatment response and other clinical features, for the early diagnosis of MSA have some help.