Children with Angelman Syndrome Experience Sleep Disturbance Associated with Poor Health

Children with Angelman syndrome experience severe night waking and higher sleep-disordered breathing scores, which were associated with poor health compared to age-matched peers, a new study looking at sleep disturbance in children with rare genetic syndromes shows.

The study titled, “A cross-syndrome cohort comparison of sleep disturbance in children with Smith-Magenis syndrome, Angelman syndrome, autism spectrum disorder and tuberous sclerosis complex,” was published in the Journal of Neurodevelopment Disorders.

Sleep disturbance is common in children with neurodevelopmental disorders and is often associated with detrimental outcomes for individuals and families, including difficult behavior and increased parental irritability and fatigue.

A recent systematic review that determined the prevalence of sleep disturbance revealed that children with Angelman syndrome have some of the the highest rates of sleep disturbance, between 48–70%, compared with children who have other rare genetic syndromes.

Determining the sleep profiles of patients may help establish the different pathways associated with sleep disturbance, while comparisons between groups of patients with different neurodevelopmental disorders using a standardized approach might help researchers understand the sleep characteristics associated with each group.

The research team used a standardized questionnaire across groups of patients with neurodevelopmental disorders considered at risk of sleep disturbance to help determine its prevalence and profile relative to age-matched, typically developing peers.

The cohort included children with Smith-Magenis syndrome, Angelman syndrome, autism spectrum disorders, and tuberous sclerosis complex.

Researchers determined the association between sleep disturbance and age, obesity, health conditions, and overactivity/impulsivity for each neurodevelopmental disorder group.

A total of 81% of children with Smith-Magenis syndrome displayed severe night waking and 73% exhibited early morning waking. In contrast, 30% of children with autism spectrum disorders experienced difficulties with sleep onset and 43% with sleep maintenance.

Among children with autism spectrum disorders and Angelman syndrome, 43% and 46%, respectively, experienced severe night waking.

Children with Smith-Magenis syndrome and Angelman syndrome also had significantly higher sleep-disordered breathing scores compared with their typically developing peers. Sleep disturbance in children with Angelman and tuberous sclerosis complex was associated with poorer health.

Sleep-disordered breathing scores — a measurement that requires overnight recordings — were significantly higher in children with Angelman, Smith-Magenis syndrome, and autism spectrum disorders. Children with autism spectrum disorders also presented with gastroesophageal reflux.

The data shows that the associations between sleep parameters and personal characteristics, specifically symptoms of gastroesophageal reflux, over-activity and impulsivity, and age could be some of the reasons that lead to 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,” the authors concluded.