Some specific clinical features differentiate multiple system atrophy (striatonigral variety) from Parkinson's disease.

Abstract

The clinical recognition of multiple system atrophy (MSA) in patients presenting with parkinsonian signs is difficult. We attempted to verify the predictive value of some pointers that are used in clinical practice. Sixteen consecutive patients with pathologically confirmed MSA who presented with a parkinsonian syndrome over an 8-year period were studied retrospectively, and their clinical features were analyzed in detail. Parkinson's Disease Society, Brain Tissue Bank, Institute of Neurology, London, England. Sixteen patients with pathologically proven MSA who presented with parkinsonian syndrome in the first 3 years since disease onset. Clinical features that were analyzed included the rapidity of disease progression, the relative symmetry of symptom onset, the presence or absence of tremor at initial presentation, the therapeutic response to levodopa and the associated presence of autonomic dysfunction. Fourteen of the 16 patients also had a computed tomographic scan of the brain performed. The frequency of selected items in MSA was compared with that found in 20 pathologically confirmed cases of Parkinson's disease and 16 pathologically confirmed cases of progressive supranuclear palsy (Steele-Richardson-Olszewski disease). It was found that a probability scale based on five selected items discriminated MSA with a pure parkinsonian presentation from Parkinson's disease, but not from progressive supranuclear palsy. Patients affected by the latter disorder, however, commonly presented with additional clinical features (supranuclear vertical down-gaze palsy, axial dystonia, and cognitive impairment), which helped to differentiate it from MSA.