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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。
Multi-system atrophy is a late-onset degenerative disease of the nervous system characterized by autonomic dysfunction, Parkinson’s disease and cerebellar ataxia. It has been confirmed that glial intracellular inclusion bodies are characteristic pathological markers of the disease, the main component is a highly phosphorylated α-synuclein. Abnormal accumulation of this protein in the inclusion body, so that patients with oligodendrocyte dysfunction, myelin rupture, eventually leading to neuronal degeneration. In recent years, many systems atrophy in clinical manifestations, pathogenesis and revised diagnostic criteria and so on are reviewed in order to provide help for the early diagnosis and treatment of the disease.