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

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.