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Daytime sleepiness and emotional and behavioral disturbances in Prader-Willi syndrome

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posted on 2022-06-03, 01:19 authored by CS Choong, GM Nixon, AM Blackmore, W Chen, P Jacoby, H Leonard, AR Lafferty, G Ambler, N Kapur, PB Bergman, Cara SchofieldCara Schofield, C Seton, A Tai, E Tham, K Vora, P Crock, C Verge, Y Musthaffa, G Blecher, A Wilson, J Downs
Individuals with Prader-Willi syndrome (PWS) often have excessive daytime sleepiness and emotional/behavioral disturbances. The objective of this study was to examine whether daytime sleepiness was associated with these emotional/behavioral problems, independent of nighttime sleep-disordered breathing, or the duration of sleep. Caregivers of individuals with PWS (aged 3 to 25 years) completed the Pediatric Sleep Questionnaire (PSQ), Epworth Sleepiness Scale for Children and Adolescents (ESS-CHAD), and the parent version of the Developmental Behavior Checklist (DBC-P). Sleep adequacy was adjusted for age by computing sleep duration against age-specific recommendations. The associations between ESS-CHAD and the total DBC and its subscale scores were evaluated by linear regression, adjusted for sleep-related breathing difficulties, sleep adequacy, and body mass index (BMI). There were 54 responses for individuals with PWS (including 22 males) aged 4.4–24.0 (mean 12.5) years. Daytime sleepiness predicted a substantial proportion of the variance in total DBC-P scores in the unadjusted model (28%; β = 0.028; p < 0.001) and when adjusted for sleep adequacy, BMI, and sleep-related breathing difficulties (29%; β = 0.023; p = 0.007). This relationship was not moderated by BMI Z-scores, but the relationship was more prominent for children younger than 12 years than for children older than 12 years. Conclusions: These findings provide preliminary novel evidence that daytime sleepiness may drive the expression of emotional/behavioral disturbances, and should be explored as a potential modifiable risk factor for these disturbances in PWS, particularly pre-adolescent children.


Open Access funding enabled and organized by CAUL and its Member Institutions. CS Choong, J Downs, and G Nixon received funding to support this work from the Perth Children's Hospital Foundation to establish the Australasian Prader-Willi Syndrome Database for which participants in this study were recruited, and funds were paid to the institution. AM Blackmore was employed to work on the project. The other authors did not receive financial support.


Publication Date



European Journal of Pediatrics






10p. (p. 2491-2500)


Springer Nature



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