Section of Clinical Neurobiology, Department of Neurology, Innsbruck Medical University, Anichstrasse 35, 6020 Innsbruck, Austria.
J Neurol. 2009 Nov;256(11):1791-808. doi: 10.1007/s00415-009-5173-8. Epub 2009 May 27.
Multiple system atrophy (MSA) is a rare late onset neurodegenerative disorder which presents with autonomic failure and a complicated motor syndrome including atypical parkinsonism, ataxia and pyramidal signs. MSA is a glial alpha-synucleinopathy with rapid progression and currently poor therapeutic management. This paper reviews the clinical features, natural history and novel diagnostic criteria for MSA as well as contemporary knowledge on pathogenesis based on evidence from neuropathological studies and experimental models. An outline of the rationale for managing symptomatic deterioration in MSA is provided together with a summary of novel experimental therapeutic approaches to decrease disease progression.
多系统萎缩（MSA）是一种罕见的迟发性神经退行性疾病，以自主神经衰竭和复杂的运动综合征为特征，包括非典型帕金森病、共济失调和锥体束征。MSA 是一种胶质细胞α-突触核蛋白病，进展迅速，目前治疗效果不佳。本文综述了 MSA 的临床特征、自然病程和新的诊断标准，以及基于神经病理学研究和实验模型的发病机制的最新知识。本文还概述了管理 MSA 症状恶化的基本原理，并总结了减少疾病进展的新的实验治疗方法。
