Abstract
Respiratory and sleep disturbances may be important causes of morbidity in Steele-Richardson-Olszewski syndrome but the frequency and character of nocturnal abnormalities remains uncertain. A prospective study of 11 patients with Steele-Richardson-Olszewski syndrome and age-matched control subjects was undertaken using clinical assessments, a structured sleep questionnaire, spirometry, static maximum inspiratory and expiratory pressures and nocturnal oximetry. The mean age of the Steele-Richardson-Olszewski syndrome patients was 63.2 (52-70) years and mean disease duration was 4.0 (2-6) years. There was moderate to severe motor disability in nine and mild to moderate dementia in eight. In the patients with Steele-Richardson-Olszewski syndrome the following abnormalities contributed to sleep disturbances significantly more frequently than in normal controls: depression, dysphagia, frequent nocturnal awakenings (usually associated with urinary frequency), immobility in bed, difficulty with transfers, impaired dressing and feeding. There was profound impairment of voluntary respiratory control whilst automatic and limbic control were well maintained. Nocturnal respiratory abnormalities were not present even in the most severely disabled. In Steele-Richardson-Olszewski syndrome sleep abnormalities are common; they relate to the cognitive, pseudobulbar and extrapyramidal disturbances and may therefore be amenable to symptomatic control.