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多系统萎缩(Multiple system atrophy,MSA)是一组散发、原因不明侵犯锥体外系、自主神经系统、小脑、锥体系的慢性进行性神经系统变性疾病,其早期症状多不典型,临床上易与帕金森病(Parkinson dis-ease,PD)、体位性低血压、遗传性小脑性共济失调等疾病混淆,目前确诊 MSA 尚需依赖病理。因此寻找有效的支持临床诊断 MSA 的客观生物标记是目前神经科学领域的重要任务。1978年,Sakuda 等人首先发现了 MSA 患者存在括约肌肌电图(sphincter EMG,sp-EMG)异常,引起高度关注。此后20多年,sp-EMG 被广泛应用于 MSA的临床诊断和鉴别诊断,但同时亦引发了对其诊断价值的激烈讨论。本文复习了国内外相关文献,对 sp-EMG 在 MSA 早期诊断中的应用价值进行综述。MSA 患者 sp-EMG 异常的病理生理基础
Multiple system atrophy (MSA) is a group of chronic progressive neurodegenerative diseases that are exuded due to unexplained violations of the extrapyramidal system, the autonomic nervous system, the cerebellum, and the pyramidal system. The early symptoms are often atypical and clinically complex Parkinson’s disease (Parkinson dis-ease, PD), orthostatic hypotension, hereditary cerebellar ataxia and other diseases confusion, the current diagnosis of MSA is still dependent on the pathology. So looking for an effective objective biomarker supporting the clinical diagnosis of MSA is an important task in the field of neuroscience. In 1978, Sakuda and others first discovered the presence of sphincter EMG (sp-EMG) abnormalities in MSA patients, causing a high degree of concern. After more than 20 years, sp-EMG has been widely used in the clinical diagnosis and differential diagnosis of MSA, but also triggered a heated discussion on its diagnostic value. This review of the relevant literature at home and abroad, the application of sp-EMG in the early diagnosis of MSA are reviewed. Pathophysiological basis of sp-EMG abnormalities in MSA patients