BackgroundSleep 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.MethodsThe 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.ResultsChildren 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.ConclusionsThese 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.Electronic supplementary materialThe online version of this article (10.1186/s11689-018-9226-0) contains supplementary material, which is available to authorized users.

ObjectiveTo determine whether improvements in school age outcomes had occurred between two cohorts of births at 22–25 weeks of gestation to women residents in England in 1995 and 2006.DesignLongitudinal national cohort studies.SettingSchool-based or home-based assessments at 11 years of age.ParticipantsEPICure2 cohort of births at 22–26 weeks of gestation in England during 2006: a sample of 200 of 1031 survivors were evaluated; outcomes for 112 children born at 22–25 weeks of gestation were compared with those of 176 born in England during 1995 from the EPICure cohort. Classroom controls for each group acted as a reference population.Main outcome measuresStandardised measures of cognition and academic attainment were combined with parent report of other impairments to estimate overall neurodevelopmental status.ResultsAt 11 years in EPICure2, 18% had severe and 20% moderate impairments. Comparing births at 22–25 weeks in EPICure2 (n=112), 26% had severe and 21% moderate impairment compared with 18% and 32%, respectively, in EPICure. After adjustment, the OR of moderate or severe neurodevelopmental impairment in 2006 compared with 1995 was 0.76 (95% CI 0.45 to 1.31, p=0.32). IQ scores were similar in 1995 (mean 82.7, SD 18.4) and 2006 (81.4, SD 19.2), adjusted difference in mean z-scores 0.2 SD (95% CI −0.2 to 0.6), as were attainment test scores. The use of multiple imputation did not alter these findings.ConclusionImprovements in care and survival between 1995 and 2006 are not paralleled by improved cognitive or educational outcomes or a reduced rate of neurodevelopmental impairment.

Study Objectives The objectives of the study were (1) to compare both actigraphy and questionnaire-assessed sleep quality and timing in children with Smith–Magenis syndrome (SMS) to a chronologically age-matched typically developing (TD) group and (2) to explore associations between age, nocturnal and diurnal sleep quality, and daytime behavior. Methods Seven nights of actigraphy data were collected from 20 children with SMS (mean age 8.70; SD 2.70) and 20 TD children. Daily parent/teacher ratings of behavior and sleepiness were obtained. Mixed linear modeling was used to explore associations between total sleep time and daytime naps and behavior. Results Sleep in children with SMS was characterized by shorter total sleep time (TST), extended night waking, shorter sleep onset, more daytime naps, and earlier morning waking compared to the TD group. Considerable inter-daily and inter-individual variability in sleep quality was found in the SMS group, so caution in generalizing results is required. An expected inverse association between age and TST was found in the TD group, but no significant association was found for the SMS group. No between-group differences in sleep hygiene practices were identified. A bidirectional negative association between TST and nap duration was found for the SMS group. In the SMS group, increased afternoon sleepiness was associated with increased irritability (p = .007) and overactivity (p = .005). Conclusion These findings evidence poor sleep quality in SMS and the need to implement evidence-based interventions in this population.