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Keywords:

  • multiple system atrophy;
  • parkinsonism;
  • MSA-P;
  • autonomic dysfunction;
  • visual hallucination;
  • slow progression

Abstract

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. © 2012 Movement Disorder Society