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multiple system atrophy

Three neurodegenerative disorders are included under this heading:

  • olivopontocerebellar atrophy (OPCA)
  • strionigral degeneration
  • Shy-Drager syndrome

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
  • Parkinsonism in strionigral degeneration
  • autonomic failure in Shy-Drager syndrome

Refs: 1) The Oxford Textbook of Medicine (1987). Oxford Medical Publications. p21.226-227. 2) Tolosa, E. & Berciano, J. (1993). Choreas, hereditary and other ataxias, tics, myoclonus and other movement disorders. Curr Op Neurol Neurosurg. 6, 358-368.

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