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Behavioural Neurology
Volume 5, Issue 4, Pages 229-232
http://dx.doi.org/10.3233/BEN-1992-5405

The Clinical Features of 67 Patients with Clinically Definite Steele-Richardson-Olszewski Syndrome

V. M. S. de Bruin and A. J. Lees

The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK

Copyright © 1992 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

The clinical features of 67 patients with probable Steele-Richardson-Olszewski syndrome have been analysed. Bradykinesia was present in 92.5% of cases at the time of initial assessment and the two commonest presenting complaints were walking difficulties and stiffness. Only 11 patients had a supranuclear gaze palsy when first seen. Forty-nine patients developed cognitive impairment in the course of their illness based on neuropsychological testing. In addition to the classical clinical findings occasional other findings included focal dystonia, tonic inhibition of levator palpebrae and blepharospasm, respiratory disturbances and impaired hearing. Only nine cases responded at all to levodopa therapy.