多系统萎缩的研究进展

多系统萎缩是一种迟发性神经系统变性疾病,以自主神经功能障碍、帕金森病及小脑性共济失调为特征性表现。现已证实,神经胶质细胞内包涵体是此病的特征性病理学标志物,主要成分是一种高度磷酸化的α-突触核蛋白。此蛋白在包涵体内的异常聚集,使患者少突胶质细胞的功能出现异常、髓鞘破裂,最终导致神经元变性。现就近几年多系统萎缩在临床表现、发病机制和修订后的诊断标准等方面进行综述,以期为该病的早期诊断和治疗提供帮助。

Recent Developments in Multiple System Atrophy

Multiple system atrophy(MSA)is a sporadic and late onset neurodegenerative disorder which presents with autonomic failure and a complicated parkinsonism or cerebellar ataxia.Several new important improvements have confirmed that GCIs composed of hyperphosphorylated α-synuclein are now accepted as the hallmarks for the definite neuropathological diagnosis of MSA and α-synuclein accumulation is their main constituent,causing oligodendroglial pathology,myelin disruption and,finally,neuronal degeneration in MSA.This review summarize the clinical features,pathogenesis and novel diagnostic criteria for MSA and help to improve the diagnostic precision and facilitate early diagnosis and treatment.