Recent developments in multiple system atrophy

被引:71
作者
Wenning, Gregor K. [1 ]
Stefanova, Nadia [1 ]
机构
[1] Innsbruck Med Univ, Dept Neurol, Sect Clin Neurobiol, A-6020 Innsbruck, Austria
关键词
Multiple system atrophy; Atypical parkinsonism; Neurodegeneration; Alpha-synuclein; Autonomic failure; PROGRESSIVE SUPRANUCLEAR PALSY; GLIAL CYTOPLASMIC INCLUSIONS; NEUROGENIC ORTHOSTATIC HYPOTENSION; ATYPICAL PARKINSONIAN SYNDROMES; AMYOTROPHIC-LATERAL-SCLEROSIS; SPINOCEREBELLAR ATAXIA TYPE-1; SILDENAFIL CITRATE VIAGRA; CHRONIC AUTONOMIC FAILURE; OXIDASE-B INHIBITOR; LESION RAT MODEL;
D O I
10.1007/s00415-009-5173-8
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
引用
收藏
页码:1791 / 1808
页数:18
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