Establishing the content validity of the Epworth Sleepiness Scale for Children and Adolescents in Prader-Willi syndrome

Patel, Vanessa; Patroneva, Albena; Glaze, Daniel G.; Davis, K.; Merikle, Elizabeth; Revana, Amee

doi:10.5664/jcsm.9632

Cited by 4 publications

(8 citation statements)

References 23 publications

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“…The Epworth sleepiness scale (ESS) is a simple 8-item measure widely used in sleep research including in people with intellectual disabilities and PWS ( 42 , 56 , 57 ). It has been shown to have good content validity when used with people with PWS ( 58 ). The primary caregiver of the person with PWS completed the ESS.…”

Section: Methodsmentioning

confidence: 99%

The relationship between endogenous oxytocin and vasopressin levels and the Prader-Willi syndrome behaviour phenotype

Rice,

Agu,

Carter

et al. 2023

Front. Endocrinol.

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BackgroundOxytocin and vasopressin systems are altered in Prader Willi syndrome (PWS). However, investigations into endogenous oxytocin and vasopressin levels as well as clinical trials evaluating the effect of exogenous oxytocin on PWS symptoms have had mixed results. It is also unknown whether endogenous oxytocin and vasopressin levels are associated with certain PWS behaviours.MethodWe compared plasma oxytocin and vasopressin and saliva oxytocin levels in 30 adolescents and adults with PWS to 30 typically developing age-matched controls. We also compared neuropeptide levels between gender and genetic subtypes within the PWS cohort and examined the relationship between neuropeptide levels and PWS behaviours.ResultsWhile we did not measure a group difference in plasma or saliva oxytocin levels, plasma vasopressin was significantly lower in individuals with PWS compared to controls. Within the PWS cohort, saliva oxytocin levels were higher in females compared to males and individuals with the mUPD compared to the deletion genetic subtype. We also found the neuropeptides correlated with different PWS behaviours for males and females and for genetic subtypes. For the deletion group, higher plasma and saliva oxytocin levels were related to fewer behaviour problems. For the mUPD group, higher plasma vasopressin levels were related to more behaviour problems.ConclusionThese findings support existing evidence of a vasopressin system defect in PWS and for the first time identify potential differences in the oxytocin and vasopressin systems across PWS genetic subtypes.

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Section: Methodsmentioning

confidence: 99%

The relationship between endogenous oxytocin and vasopressin levels and the Prader-Willi syndrome behaviour phenotype

Rice,

Agu,

Carter

et al. 2023

Front. Endocrinol.

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“…The Epworth Sleepiness Scale adapted for children and adolescents (ESS-CHAD) is a measure of daytime sleepiness [ 31 ]. To date, this questionnaire is validated as a reliable measure of self-reported daytime sleepiness in TD adolescents between the ages of 12 and 18 years old [ 31 ] and has been subsequently used in children with Prader–Willi syndrome who are also at an increased risk of SDB [ 32 ]. Parents completed the questionnaire in this study on behalf of their child for eight common daily situations, which were scored by how likely their child was to doze or fall asleep during each situation on a scale of 0 (would never doze or sleep) to 3 (high chance of dozing or sleeping).…”

Section: Methodsmentioning

confidence: 99%

Effects of Treatment of Sleep Disordered Breathing on Sleep Macro- and Micro-Architecture in Children with Down Syndrome

et al. 2022

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Background: Children with Down syndrome (DS) are at increased risk of obstructive sleep disordered breathing (SDB), which is associated with intermittent hypoxia and sleep disruption affecting daytime functioning. We aimed to examine the effects of treatment of SDB on sleep quality and daytime functioning in children with DS. Methods: Children with DS and SDB (n = 24) completed a baseline and follow-up overnight polysomnographic (PSG) study 22 ± 7 months (mean ± SD) later. Sleep micro-architecture was assessed using EEG spectral analysis, and parents completed a number of questionnaires assessing sleep, behavior, daytime functioning, and quality of life (QOL). Results: A total of nine children (38%) were treated. At baseline, the treated group had more severe SDB compared to the untreated group. SDB severity was significantly improved from 40.3 ± 46.9 events/h to 17.9 ± 26.9 events/h (p < 0.01) at follow up in children who were treated. There were no significant differences in sleep macro-architecture parameters from baseline to follow up in either the treated or untreated group. Sleep micro-architecture was not different between studies in the treated group, however this tended to improve in the untreated group, particularly in REM sleep. Daytime functioning and behavior were not different between the studies in either group, however, QOL improved after treatment. Conclusions: Our study identified that treatment of SDB improves severity of the disease as defined by PSG, and this was associated with parental reports of improved QOL, despite treatment having no demonstrable impacts on sleep quality, behavior, or daytime functioning.

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“…54,55 The Epworth Sleepiness Scale for Children and Adolescents (ESS-CHAD) has been validated in children with PWS down to age 6 years of age, and therefore provides a useful tool for screening for excessive daytime sleepiness in the clinical setting. 57 There are multiple challenges in the diagnostic evaluation of the child with PWS and hypersomnia. While the diagnostic work-up for hypersomnia and narcolepsy in the child without PWS may consist of multiple sleep latency testing (MSLT), narcolepsy antigen testing, and possible cerebrospinal fluid (CSF) hypocretin testing, these diagnostic tools may have different test characteristics within the PWS population.…”

Section: Hypersomnia and Narcolepsymentioning

confidence: 99%

Section: Hypersomnia and Narcolepsymentioning

confidence: 99%

Sleep Disorders in Children with Prader Willi Syndrome: Current Perspectives

Ingram¹,

Arganbright²,

Paprocki³

et al. 2022

NSS

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Children with Prader-Willi syndrome (PWS) face a multitude of potential health challenges including life-threatening obesity, endocrinopathies, behavioral and emotional dysregulation, developmental delays, and sleep disorders. In the current perspective piece, we provide a focused review of the condition's etiology and clinical findings, as well as a more in-depth discussion of sleep disorders frequently associated with PWS. In particular, we highlight and discuss difficult clinical scenarios frequently encountered by the pediatric sleep physician caring for this patient population, including diagnosis and treatment of complex sleep-related breathing disorders, considerations for sleep apnea surgery, the interplay between growth hormone and sleep apnea, diagnostic challenges in hypersomnia/narcolepsy, and current and emerging therapies for hypersomnia/narcolepsy. Overall, although there are many areas that need further research, sleep disorders remain a fruitful target for improving quality of life of children with PWS and their families.

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Establishing the content validity of the Epworth Sleepiness Scale for Children and Adolescents in Prader-Willi syndrome

Cited by 4 publications

References 23 publications

The relationship between endogenous oxytocin and vasopressin levels and the Prader-Willi syndrome behaviour phenotype

The relationship between endogenous oxytocin and vasopressin levels and the Prader-Willi syndrome behaviour phenotype

Effects of Treatment of Sleep Disordered Breathing on Sleep Macro- and Micro-Architecture in Children with Down Syndrome

Sleep Disorders in Children with Prader Willi Syndrome: Current Perspectives

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