Sleep disturbances and behavioural problems in adults with Prader-Willi syndrome

摘要

Background Individuals with Prader-Willi syndrome (PWS) are at risk of sleep disturbances, such as excessive daytime sleepiness (EDS) and sleep apnoea, and behavioural problems. Sleep disturbances and their relationship with other variables had not been researched extensively in adults with PWS.Method Sleep disturbances and behavioural problems were investigated in adults with genetically confirmed PWS using standardised questionnaires. Results of adults with paternal deletion (n - 45) were compared with those of adults with maternal uniparental disomy (n = 33). Results Eleven adults with PWS (i.e. 15) had a current sleep problem, mostly night waking problems. Twenty-six adults with PWS (i.e. 33) suffered from severe EDS. No differences in prevalence of sleep disturbances between genetic subtypes were found. Seventeen adults with deletion (i.e. 38) and 17 adults with maternal uniparental disomy (i.e. 52) had behavioural problems. No significant relationships were found between sleep disturbances and behavioural problems. Conclusions In adults with PWS, EDS is the most common type of sleep disturbance. Men and individuals with relative high body mass index are at increased risk for EDS. More research, aimed at developing a suitable screening instrument for sleep apnoea in adults with PWS, is necessary. Clinical implications of the findings are discussed.