Multiple system atrophy–parkinsonism with slow progression and prolonged survival: A diagnostic catch

Abstract

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