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

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.