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Multiple system atrophy (MSA)

Authoring team

Multiple system atrophy (MSA) is a fatal, adult-onset, neurodegenerative disease with rapid progression and limited symptomatic treatment options

  • clinical features include parkinsonism and cerebellar and autonomic signs and symptoms in various combinations
    • two major motor variants are distinguished according to the predominant motor presentation:
      • MSA parkinsonian variant (MSA-P) and
      • MSA cerebellar variant (MSA-C)
  • in Europe and North America, the distribution is in favor of the MSA-P variant, whereas MSA-C is more prevalent in Japan.
    • neuropathological correlates of MSA-P and MSA-C are striatonigral degeneration and olivopontocerebellar atrophy, respectively

Shy-Drager syndrome is considered another form of MSA.

Pathologically multiple system atrophy is characterised by widespread neuronal loss and gliosis.

Clinically there are three distinct pictures according to which parts of the brain are most severely affected:

  • cerebellar ataxia in OPCA (MSA-C)
  • Parkinsonism in strionigral degeneration (MSA-P)
  • autonomic failure in Shy-Drager syndrome

Reference:

  1. Poewe W et al. Multiple system atrophy. Nat Rev Dis Primers. 2022 Aug 25;8(1):56.

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