多系统萎缩患者的临床、影像及电生理分析

The clinical, neuroimaging and electrophysiological presentations in 62 cases with multiple system atrophy

目的探讨多系统萎缩（multiple system atrophy，MSA）的临床、影像以及电生理改变特点，为临床诊断提供依据。方法对62例MSA患者的临床资料、神经影像学以及肌电图检查结果进行回顾性分析。结果我们共收集拟诊MSA患者62例，其中MSA—A型29例（46．8％），主要临床特点表现为直立性低血压为主的自主神经系统症状；MSA—C型24例（38．7％），主要表现为小脑性共济失调；MSA—P型9例（14．5％），则以锥体外系症状为主。头颅MRI显示MSA—A型患者部分出现小脑病灶；MSA—C型患者主要病变在小脑、脑桥和延髓；MSA—P型患者病变主要在壳核。51例患者行肛门括约肌肌电图（external anal sphincter electromyography，EAS—EMG）检查，其中46例示典型神经源性损害；19例（30．6％）曾被误诊为其他疾病。结论MSA早期易漏诊或误诊，结合临床表现、神经影像学以及EAS—EMG检查，可提高MSA的诊断率。

Objective To evaluate the clinical, neuroimaging and electrophysiology features of 62 patients with multiple system atrophy （MSA）. Methods Sixty-two cases with diagnosis of probable MSA were recruited in a retrospective studied. Clinical, neuroimage and external anal sphincter electromyography （EAS-EMG） data was retrospectively analyzed. Results In 62 cases （44 male and 18 female）, the onset age was between 37 and 76. Among them, 29 cases （46. 8% ） were MSA-A, with orthostatic hypotension as the main clinical manifestation; 24 cases （38.7%） were MSA-C, with cerebellar ataxia as the main clinical manifestation; 9 cases （14.5%） were MSA-P, with extrapyramidal symptoms as the main clinical manifestation. MRI showed that main lesion of MSA-A was in the cerebellum; that of MSA-C was in the cerebellum, pons and medulla; and that of MSA-P was in the putamen. Fifty-one eases did EAS-EMG and 46 cases showed neurogenic impairments. Nineteen cases were initially misdiagnosed with other diseases. Conclusions MSA is easy to be omitted or misdiagnosed at early stage. The diagnostic rate of MSA can be increased by the combination of clinical expressions, neuroimage, EAS-EMG and other necessary examinations.