Multiple system atrophy-parkinsonism with slow progression and prolonged survival: a diagnostic catch

Mov Disord. 2012 Aug;27(9):1186-90. doi: 10.1002/mds.25115. Epub 2012 Jul 17.


Background: Multiple system atrophy (MSA) is a neurodegenerative disease leading to severe physical impairment, with a disease duration from onset to death of 6-9 years.

Methods: The clinical and neuropathological features of 4 MSA cases with disease duration of 15 years or more were analyzed.

Results: All patients presented with parkinsonism and had a mean latency of 11 years before the development of dysautonomia. Mean duration from onset of first symptoms to anterocollis, inspiratory stridor, and dysphagia was 9 years. Despite the limited levodopa response, all patients developed levodopa-induced dyskinesia.

Conclusions: Late appearance of dysautonomia is a favorable prognostic factor in MSA-P. Greater awareness of this uncommon "benign" subgroup of MSA will improve diagnostic accuracy and help to more accurately inform treatment options.

MeSH terms

  • Activities of Daily Living
  • Adult
  • Age of Onset
  • Aged
  • Autopsy
  • Brain / pathology
  • Cognition Disorders / complications
  • Cognition Disorders / psychology
  • Diagnostic Errors
  • Disease Progression
  • Female
  • Hallucinations / etiology
  • Hallucinations / psychology
  • Humans
  • Immunohistochemistry
  • Male
  • Middle Aged
  • Multiple System Atrophy / diagnosis
  • Multiple System Atrophy / pathology*
  • Neuropsychological Tests