Multiple system atrophy

被引：136

作者：

Poewe, Werner ^{[1
]}

Stankovic, Iva ^{[2
]}

Halliday, Glenda ^{[3
]}

Meissner, Wassilios G. ^{[4
,5
,6
]}

Wenning, Gregor K. ^{[1
]}

Pellecchia, Maria Teresa ^{[7
]}

Seppi, Klaus ^{[1
]}

Palma, Jose-Alberto ^{[8
]}

Kaufmann, Horacio ^{[8
]}

机构：

[1] Med Univ Innsbruck, Dept Neurol, Innsbruck, Austria

[2] Univ Belgrade, Fac Med, Clin Ctr Serbia, Neurol Clin, Belgrade, Serbia

[3] Univ Sydney, Fac Med & Hlth, Brain & Mind Ctr, Sch Med Sci, Sydney, NSW, Australia

[4] CHU Bordeaux, MNc, CRMR AMS, Serv Neurol Malad Neurodegenerat, Bordeaux, France

[5] Univ Bordeaux, CNRS, IMN, UMR 5293, Bordeaux, France

[6] Univ Otago, New Zealand Brain Res Inst, Dept Med, Christchurch, New Zealand

[7] Univ Salerno, Ctr Neurodegenerat Dis, Dept Med Surg & Dent, Scuola Med Salernitana, Salerno, Italy

[8] NYU, Dept Neurol, Grossman Sch Med, New York, NY 10016 USA

来源：

NATURE REVIEWS DISEASE PRIMERS | 2022年 / 8卷 / 01期

基金：

欧盟地平线“2020”; 澳大利亚国家健康与医学研究理事会; 美国国家卫生研究院; 英国医学研究理事会; 奥地利科学基金会;

关键词：

PROGRESSIVE SUPRANUCLEAR PALSY; SLEEP BEHAVIOR DISORDER; OLIGODENDROCYTE PRECURSOR CELLS; IDIOPATHIC PARKINSONS-DISEASE; DEEP BRAIN-STIMULATION; PURE AUTONOMIC FAILURE; QUALITY-OF-LIFE; ALPHA-SYNUCLEIN; NATURAL-HISTORY; ORTHOSTATIC HYPOTENSION;

D O I：

10.1038/s41572-022-00382-6

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

This Primer by Poewe and colleagues summarizes the epidemiology, diagnosis, pathophysiology and treatment of multiple system atrophy. Moreover, this Primer provides an overview of the quality of life issues faced by patients with this disorder and future research avenues. Multiple system atrophy (MSA) is a rare neurodegenerative disease that is characterized by neuronal loss and gliosis in multiple areas of the central nervous system including striatonigral, olivopontocerebellar and central autonomic structures. Oligodendroglial cytoplasmic inclusions containing misfolded and aggregated alpha-synuclein are the histopathological hallmark of MSA. A firm clinical diagnosis requires the presence of autonomic dysfunction in combination with parkinsonism that responds poorly to levodopa and/or cerebellar ataxia. Clinical diagnostic accuracy is suboptimal in early disease because of phenotypic overlaps with Parkinson disease or other types of degenerative parkinsonism as well as with other cerebellar disorders. The symptomatic management of MSA requires a complex multimodal approach to compensate for autonomic failure, alleviate parkinsonism and cerebellar ataxia and associated disabilities. None of the available treatments significantly slows the aggressive course of MSA. Despite several failed trials in the past, a robust pipeline of putative disease-modifying agents, along with progress towards early diagnosis and the development of sensitive diagnostic and progression biomarkers for MSA, offer new hope for patients.

引用

页数：21