Related Experiment Video
Updated: Aug 7, 2026
Behavioral Characterization of an Angelman Syndrome Mouse Model
Published on: October 20, 2023
Profiles of sleep disturbances in Angelman, Prader-Willi, and Rett syndromes: analysis of standardized questionnaires
Lindsay M Oberman1, Olivia J Veatch2, Sarika U Peters3
1National Institute of Mental Health Intramural Research Program, National Institutes of Health, Bethesda, MD, United States.
Introduction:
Individuals with neurodevelopmental disorders (NDDs) are at increased risk of having sleep difficulties. A variety of sleep problems have been reported in Angelman syndrome (AS), Prader-Willi syndrome (PWS), and Rett syndrome (RTT). The present study intended to expand an earlier characterization of sleep difficulties in a large AS, PWS, and RTT sample by analyzing subscales of standardized sleep questionnaires.
Methods:
Scores from children (2-18 years) with AS (n = 74), PWS (n = 90) or RTT (n = 241) on components of the Children's Sleep Habits Questionnaire (CSHQ), the Sleep-Related Breathing Disorder (SRBD) scale, and the Pediatric Daytime Sleepiness Scale (PDSS) were compared between NDDs and with those from a group of neurotypical siblings (n = 282). Additional comparisons of scores after a 12-month follow-up evaluation, in a subset of individuals, were also performed. Data were analyzed using nonparametric tests and, for changes over time, both cross-sectionally and longitudinally.
Results:
Comparisons with neurotypical children showed that night waking and snoring were increased in the three NDDs while parasomnias and daytime sleepiness only in AS and RTT. Children with RTT also had the highest scores on measures of disordered breathing. At the 12-month follow-up, scores decreased in neurotypical children but had variable courses in the NDDs, with increased disordered breathing scores characterizing AS and RTT. There was high agreement among disordered breathing measures, but not among daytime sleepiness scales. Overall, CSHQ scores were relatively stable within NDDs.
Conclusion:
Sleep questionnaires revealed disorder-specific profiles of sleep problems that could assist in their identification and management. The CSHQ and the SRBD, including their subscales, appear to be consistent measures particularly for sleep-disordered breathing and, therefore, suitable for clinical and research use in severe NDDs. Follow-up studies should expand the range of instruments to include objective measures in the characterization of sleep abnormalities in AS, PWS, and RTT.
