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Background: Sleep problems are common in Smith-Magenis (SMS) and Angelman syndromes (AS). Effectiveness of interventions depends on appropriate assessment, complicated by compromised self-report and health and behaviour difficulties. Studying settling and waking in these syndromes could inform assessment., Aims: To describe settling and waking behaviours in children at high-risk of sleep and health problems, using direct observation., Methods and Procedures: Video and actigraphy data were collected for 12 participants with AS (Mean age = 8.02, SD = 2.81) and 11 with SMS (Mean age = 8.80, SD = 2.18). Settling (30 min prior to sleep onset) and night waking were coded for nineteen behaviours relating to pain, challenging behaviour and caregiver interaction. Lag sequential analyses were conducted for pain-related behaviours., Outcomes and Results: Percentage of time spent in behaviours was calculated. Parent-child interactions (0.00-9.93 %) and challenging behaviours (0 %) were rare at settling and waking in both groups. In the AS group, pain-related behaviours were more likely to occur before waking than by chance (p < 0.001)., Conclusions and Implications: Findings highlight the importance of considering pain as a cause of sleep problems in AS. The principle and methodology could be extended to individuals with ID experiencing sleep problems., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Background: Sleep disturbance is common in children with neurodevelopmental disorders, with high rates identified in children with Smith-Magenis syndrome (SMS), Angelman syndrome (AS), autism spectrum disorder (ASD) and tuberous sclerosis complex (TSC). Phenotypic sleep profiles for these groups may implicate different pathways to sleep disturbance. At present, cross-group comparisons that might elucidate putative phenotypic sleep characteristics are limited by measurement differences between studies. In this study, a standardised questionnaire was administered across groups affording comparison of the prevalence and profile of sleep disturbance between groups and contrast to chronologically age-matched typically developing (TD) peers., Methods: The modified version of Simonds and Parraga's sleep questionnaire, adapted for use in children with intellectual disabilities, was employed to assess sleep disturbance profiles in children aged 2-15 years with SMS (n = 26), AS (n = 70), ASD (n = 30), TSC (n = 20) and a TD contrast group (n = 47). Associations between sleep disturbance and age, obesity, health conditions and overactivity/impulsivity were explored for each neurodevelopmental disorder group., Results: Children with SMS displayed severe night waking (81%) and early morning waking (73%). In contrast, children with ASD experienced difficulties with sleep onset (30%) and sleep maintenance (43%). Fewer children with ASD (43%) and AS (46%) experienced severe night waking compared to children with SMS (both p < .01). Higher sleep-disordered breathing scores were identified for children with SMS (p < .001) and AS (p < .001) compared to the TD group. Sleep disturbance in children with AS and TSC was associated with poorer health. Children experiencing symptoms indicative of gastro-oesophageal reflux had significantly higher sleep-disordered breathing scores in the AS, SMS and ASD groups (all p < .01). A number of associations between overactivity, impulsivity, gastro-oesophageal reflux, age and sleep disturbance were found for certain groups., Conclusions: These data reveal syndrome-specific profiles of sleep disturbance. The divergent associations between sleep parameters and person characteristics, specifically symptoms of gastro-oesophageal reflux, overactivity and impulsivity and age, implicate aetiology-specific mechanisms underpinning sleep disturbance. The differences in prevalence, severity and mechanisms implicated in sleep disturbance between groups support a syndrome-sensitive approach to assessment and treatment of sleep disturbance in children with neurodevelopmental disorders.

Angelman syndrome is a rare genetic syndrome, in which sleep disturbances are reported for 20-80% of individuals (Williams et al., 2006). This interview study delineated parental perceptions of sleep problems experienced by children with Angelman syndrome and the impact on parental sleep quality, health and wellbeing. The nature of desired interventions was also explored. Semi-structured interviews were completed with parents of 50 children, aged 16 months-15 years with Angelman syndrome who experienced current or historic sleep problems; predominantly night waking and settling problems. Parents were concerned by the impact of their child's sleep quality upon their own ability to function during the day. The importance of considering parental experiences was evidenced by variability in coping e.g. despite the persistence of sleep problems 20% of parents did not feel the need for any additional support. Amongst a range of types of further support desired, 27% cited further support with a behavioural intervention, and information about the trajectory of sleep problems in Angelman syndrome (18%). The results suggest that behavioural interventions supporting both children and parents in improving their sleep quality and well-being, and longitudinal research into sleep problems should be prioritised., (Copyright © 2017 Elsevier Ltd. All rights reserved.)