Multi-Method Assessment of Sleep in Children With Angelman Syndrome: A Case–Controlled Study

Abstract

Objectives: To assess sleep quality and timing in children with Angelman syndrome (AS) with sleep problems using questionnaires and actigraphy and contrast sleep parameters to those of typically developing (TD) children matched for age and sex.Methods: Week-long actigraphy assessments were undertaken with children with AS (n = 20) with parent-reported sleep difficulties and compared with age and sex matched TD controls. The presence of severe sleep problems was assessed using the modified Simonds and Parraga sleep questionnaire. Sleep hygiene was measured using the Family Inventory of Sleep Habits.Results: Actigraphy and parent-completed sleep diary data indicated that children with AS had significantly earlier bedtimes (p = .003, Cohen d = .47) and poorer sleep efficiency (78%, p = .04, d = .33) than TD children (84%). No significant differences in total sleep time, sleep onset latency or wake after sleep onset were found between the two groups. The expected relationship between later bedtimes and increasing age found for the TD group (p < .001, β.78) was not evidenced for the AS group (p = .09, β.39). Considerable inter-individual and night to night variation in actigraphy assessed total sleep time and wake after sleep onset was found for children with AS compared to TD children. Parent report indicated that a greater proportion of children with AS had severe night waking problems compared to TD children (81 versus 5%). No significant differences in sleep hygiene and excessive daytime sleepiness were found between the two groups (p > .05).Conclusions: This study reports the largest objective dataset of sleep quality parameters in children with AS. Sleep quality in this group was characterised by poor efficiency and significant intra- and inter-individual variability that warrants further investigation. This variability should inform assessment and intervention for sleep in children with AS, as averages of total sleep, even across a 7 day period may not capture the difficulties with night waking highlighted by parental questionnaire report.

Sleep in people with and without intellectual disabilities: a systematic review and meta‐analysis

“…Conversely, new data on the sleep quality of this subgroup seem to strengthen the original finding that individuals with idiopathic ID experience poorer sleep quality than TD controls (Surtees et al 2018;Chan et al 2019). Further studies have also been published including people with ID and an associated genetic syndrome or neurodevelopmental condition, a number of which have suggested that original subgroup conclusions may not be congruous (Gunes et al 2019;Smith et al 2019;Trickett et al 2019).…”

Behavioural sleep problems in children and adults with intellectual disabilities: An integrative literature review

“…In addition to rare conditions which are linked to sleep disturbances such as Christianson Syndrome (Gruber et al, 2022), Dup15q Syndrome (Barstein et al, 2021), Pallister‐Killian Syndrome (Fetta et al, 2022), Phelan‐McDermid Syndrome and SYNGAP1‐ID (Smith‐Hicks et al, 2021), MBD5‐associated neurodevelopmental disorder (Gandhi et al, 2021), Arboleda‐Tham syndrome (Smith & Harris, 2021), Bosch‐Boonstra‐Schaaf optic atrophy syndrome (Rech et al, 2020) and emerging genetic variants including microdeletion syndrome at 2q22.1q22.3 and de novo DDX3X variants (Chen et al, 2020; Verhoeven et al, 2020). This shows that a wide array of specific intellectual disability syndromes are associated with sleep problems, although variation from night to night and within syndrome groupings is found (Trickett et al, 2019).…”

Sleep disorder in Angelman syndrome: causes, mechanisms and methods of correction. Literature review

“…Для оценки сна у детей с СА использовался также метод актиграфии в течение 7 сут [36]. Не отмечено превышения по частоте пробуждений и значительного снижения длительности сна по сравнению с контрольной группой, имеются лишь статистически значимые расхождения во времени засыпания после выключения света.…”

“…С возрастом не отмечено значимой динамики изменения сна у детей с СА, в то время как в контрольной группе отмечается тенденция к засыпанию в более позднее время и укорочению длительности сна, что является отражением развития способности волевой депривации сна у здоровых детей для реализации собственных социальных нужд (подготовка к учебе, подъем по будильнику и т.д.) [36].…”