.png){kind=icon}
Return
Multiple system atrophy
.png){kind=icon}
PRE
AI
DOI：10.1038/s41572-022-00382-6.png)
.png){kind=icon}
.png){kind=icon}
Share.png){kind=icon}
Save
Abstract
En 中文
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.
Keywords:
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
Journal
N
IF:
60.6
Papers:
661
Citations:
4.5W
Organization
Cited Papers
ShareSaveAlpha-Synuclein Expression in the Oligodendrocyte Lineage: an In Vitro and In Vivo Study Using Rodent and Human Models.png)
STEM CELL REPORTS
OAAI
STEM CELL REPORTSIF5.1
OAAIShareSaveIdentification and Quantification of Oligodendrocyte Precursor Cells in Multiple System Atrophy, Progressive Supranuclear Palsy and Parkinson's DiseaseBRAIN PATHOLOGYOAAI
BRAIN PATHOLOGYIF6.2
OAAIShareSaveShareSaveDisease progression and prognostic factors in multiple system atrophy: A prospective cohort studyOAAI
OAAIShareSaveShareSave
View more