12 例多系统萎缩患者的临床特点和诊断分析

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。

The Clinical Features and Diagnosis Points of 12 Cases with MSA

Objective: Discuss the clinical features and diagnosis points of 12 cases of MSA(multiple system atrophy MSA).Methods: Through a restrospective study of 12 patients with MSA,to discuss the clinical features and early diagnosis points of the disease.Results: In this group,all the patients were chronic,insidious onset,male: female = 1.4: 1.Average age of onset were 49.2 years(35.0 years to 72.0 years).Average duration of the disease was 33 months(6 months to 60 months).4 cases were MSA-P subtype,and the other 8 cases were MSA-C subtype.Bradypraxia as the initial symptom was found in 75 % of MSA-P subtype,the other main signs were respectively increased muscle tone(100%),orthostatic hypotension(75 %) and pyramidal signs(75 %);unstable walking as the main initial presentation was seen in 62.5 % of MSA-subtype C,the other main signs were ataxia(100 %),orthostatic hypotension,cerebellar language and pyramidal signs(62.5 %).Conclusions: MSA is a sporadic neurodegenerative disease which is adult-onset mostly with multiple clinical manifestation.For the patients with early simply symptoms such as Parkinson’s syndrome,cerebellar dysfunction or autonomic dysfunction,especially those without family history,we should pay close attention to screen whether there exits symptoms or signs of other systems.And it will be helpful for the early diagnosis of MSA if combined with some particular clinical features such as imaging studies and the treatment response of levodopa.