Multisystem atrophy

Multisystem atrophy


Multisystem atrophy is one of the Parkinson plus syndromes, and is characterised by the three cardinal features of:-

  • Parkinsonism
  • Autonomic failure
  • Cerebellar ataxia

It presents in one of three ways:-

  • Shy-Drager Syndrome (mainly autonomic features)
  • Striatonigral degeneration (mainly Parkinson features)
  • Olivopontocerebellar atrophy (mainly cerebellar features)

Macroscopic features include:-

  • Pallor of substantia nigra
  • Greenish discolouration and atrophy of the putamen
  • Cerebellar atrophy

Microscopic features include:-

  • Papp-Lantos bodies (alpha-synuclein inclusions in oligodendrocytes found in the substantia nigra, cerebellum, and basal ganglia)