多系统萎缩的诊治进展

Advances in the Diagnosis and Treatment of Multiple System Atrophy

多系统萎缩(MSA)是一种快速进展的罕见神经退行性疾病,以自主神经功能障碍、帕金森症、小脑性共济失调和锥体束征的不同组合为主要临床表现。该病起病隐匿、进展快、临床异质性大、早期确诊困难,阻碍了神经保护剂的开发。本文就MSA目前诊断标准、早期诊断标志物和治疗进展进行重点讨论,以期对临床医师临床诊疗提供帮助。

Multiple system atrophy(MSA) is a rare and rapidly-progressive neurodegenerative disorder, characterized by the combination of dysautonomia, poor levodopa responsive parkinsonism, cerebellar ataxia, and pyramidal tract signs. Insidious onset, clinical heterogeneity and progression of the disease complicate the difficulty of early diagnosis and challenge, the development of neuroprotective drugs. In order to improve the knowledge of diagnosis and treatment of the disease, this paper reviews advances in its diagnostic criteria, biomarkers of early diagnosis and management of the disease.