Abstract
We analyzed the clinical features and natural history of 52 consecutive patients with the Steele-Richardson-Olszewski syndrome. The most common symptoms at onset were unsteady gait, backward falls because of poor balance, visual disturbances, slurred speech, and forgetfulness. The median duration from onset to death was 5.9 years, with a median survival after diagnosis of only 1.8 years. Bronchopneumonia was the usual cause of death recorded on death certificates.