Evaluation of a behavioral treatment package to reduce sleep problems in children with Angelman Syndrome

Allen, Keith D.; Kuhn, Brett R.; DeHaai, Kristi A.; Wallace, Dustin P.

doi:10.1016/j.ridd.2012.10.001

Cited by 72 publications

(40 citation statements)

References 24 publications

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“…Despite sleep disruption, most individuals with AS do not exhibit daytime somnolence. With behavior modification52 and/or pharmacologic treatment, sleep difficulties can be overcome in most patients, and sleep patterns improve with age 48. Epilepsy severity correlates with sleep problems, but whether more severe seizures create sleep disturbance or whether poor sleep patterns exacerbate epilepsy remains unclear 53…”

Section: Clinical Reviewmentioning

confidence: 99%

Angelman syndrome: review of clinical and molecular aspects

Bird

2014

TACG

179

155

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“Angelman syndrome” (AS) is a neurodevelopmental disorder whose main features are intellectual disability, lack of speech, seizures, and a characteristic behavioral profile. The behavioral features of AS include a happy demeanor, easily provoked laughter, short attention span, hypermotoric behavior, mouthing of objects, sleep disturbance, and an affinity for water. Microcephaly and subtle dysmorphic features, as well as ataxia and other movement disturbances, are additional features seen in most affected individuals. AS is due to deficient expression of the ubiquitin protein ligase E3A (UBE3A) gene, which displays paternal imprinting. There are four molecular classes of AS, and some genotype–phenotype correlations have emerged. Much remains to be understood regarding how insufficiency of E6-AP, the protein product of UBE3A, results in the observed neurodevelopmental deficits. Studies of mouse models of AS have implicated UBE3A in experience-dependent synaptic remodeling.

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Section: Clinical Reviewmentioning

confidence: 99%

Angelman syndrome: review of clinical and molecular aspects

Bird

2014

TACG

179

155

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“…These phenotypes suggest that imprinted genes of paternal and maternal origin have contrasting effects on sleep in infants without deletions, with genes of paternal origin promoting suckling and waking. Small-scale behavioral interventions in which parents were instructed not to respond to night waking by children with AS have resulted in dramatic improvements in sleep quality [53, 54]. …”

Section: Suckling and Sleepmentioning

confidence: 99%

Troubled sleep: Night waking, breastfeeding and parent-offspring conflict

Haig

2014

Evolution, Medicine, and Public Health

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Disrupted sleep is probably the most common complaint of parents with a new baby. Night waking increases in the second half of the first year of infant life and is more pronounced for breastfed infants. Sleep-related phenotypes of infants with Prader-Willi and Angelman syndromes suggest that imprinted genes of paternal origin promote greater wakefulness whereas imprinted genes of maternal origin favor more consolidated sleep. All these observations are consistent with a hypothesis that waking at night to suckle is an adaptation of infants to extend their mothers’ lactational amenorrhea, thus delaying the birth of a younger sib and enhancing infant survival.

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“…Thus, the combination of good sleep hygiene and restricting the time that children spent awake in bed was successful in reducing sleep problems. As with Allen et al's study (Allen et al 2013), this study does not allow for determination of which component of the treatment was most effective. However, visual data presented in the report suggest that there was an additive effect.…”

Section: Behavioural Approaches That Have Been Studied In Childrenmentioning

confidence: 98%

“…Another study, including five children with Angelman syndrome, combined sleep hygiene changes, extinction and sleep scheduling in combination (Allen et al 2013). All children showed disruptive bedtime behaviour and/or sleep onset problems.…”

Section: Behavioural Approaches That Have Been Studied In Childrenmentioning

confidence: 99%