Wenning Gregor K, Krismer Florian
Division of Clinical Neurobiology, Department of Neurology, Medical University, Innsbruck, Austria.
Handb Clin Neurol. 2013;117:229-41. doi: 10.1016/B978-0-444-53491-0.00019-5.
Multiple system atrophy (MSA) is a sporadic and fatal α-synuclein-linked oligodendrogliopathy manifesting with progressive autonomic failure, poorly levodopa-responsive parkinsonism, and cerebellar ataxia, in any combination. Here we review key aspects of MSA integrating important insights from rapidly emerging fields such as genetics, diagnostic work-up including imaging, and translational therapies aimed at disease modification.
多系统萎缩(MSA)是一种散发性致命性α-突触核蛋白相关的少突胶质细胞病,表现为进行性自主神经功能衰竭、左旋多巴反应不佳的帕金森综合征和小脑共济失调,可任意组合出现。在此,我们综述多系统萎缩的关键方面,整合来自遗传学等快速发展领域的重要见解、包括影像学在内的诊断检查以及旨在改变疾病进程的转化治疗。