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Multiple System Atrophy (MSA) 

Multiple System Atrophy (MSA)
Chapter:
Multiple System Atrophy (MSA)
Author(s):

Gregor K. Wenning

and Florian Krismer

DOI:
10.1093/med/9780199609536.003.0013
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date: 25 February 2020

Multiple system atrophy (MSA) is an adult-onset atypical parkinsonian disorder (APD) characterized by either rapidly progressive levodopa-unresponsive parkinsonism or a cerebellar syndrome associated with early autonomic failure. According to current consensus criteria two motor subtypes can be distinguished clinically, namely a parkinsonian variant labelled MSA-P and a cerebellar variant labelled MSA-C . Abundant (oligodendro-)glial cytoplasmic inclusions (GCI) represent the histopathological signature lesion that is accompanied by a selective neuronal multisystem degeneration. The principle component of GCIs is α-synuclein, a finding which firmly places MSA among other α-synucleinopathies such as Parkinson’s disease (PD) and dementia with Lewy bodies (DLB). Currently, the management of MSA patients is based on symptomatic treatment targeting parkinsonian and autonomic features. Although research into disease-modifying strategies has accelerated, effective neuroprotective treatments are still lacking.

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