An exploratory study of sleep quality and quantity in children with causal variants in SYNGAP1, an autism risk gene.

STUDY OBJECTIVES: Sleep disturbances are reported in 62% of children with SYNGAP1-Intellectual Disability (SYNGAP1-ID), a rare neurodevelopmental disorder characterized by intellectual disability, epilepsy, autism spectrum disorder (ASD), sensory and behavioral challenges. Although Children's Sleep Habits Questionnaire (CSHQ) scores are elevated in children with SYNGAP1-ID factors that predict sleep disturbance are not well understood. The goal of this study is to identify predictors of sleep problems. METHODS: Parents of 21 children with SYNGAP1-ID completed questionnaires, and 6 children wore the Actiwatch2 for 14 continuous days. Non-parametric analysis of psychometric scales and actigraphy data were performed. Actigraphy derived sleep parameters were compared to controls and rest activity rhythms were assessed using arctools an open-source R package. RESULTS: CSHQ total sleep scores in children with SYNGAP1-ID and ASD were not different from children with SYNGAP1 without ASD (p = 0.61). Sleep anxiety (ß 1.646, 95% CI 0.9566 to 2.336) and parasomnias (ß 0.6294, 95% CI 0.06423 to 1.195) were strong predictors of bedtime resistance (R2 = 0.767, p < 0.001). The sedentary to active transition probability during the 12-18 h epoch (ß = 0.004, p = 0.008, R2 = 0.85) and the duration of the active bout during the 18-24 h epoch (ß = 0.166, p = 0.029, R2 = 0.74) were strong predictors of total sleep disturbance. CONCLUSION: The CSHQ may be a reliable measure of sleep difficulties in children with SYNGAP1-ID. Sleep anxiety, parasomnias and difficulty winding-down are significant contributors to sleep disturbances.

Conducting preference assessments for youth with disorders of consciousness during rehabilitation.

PURPOSE: Care and rehabilitation for individuals with disorders of consciousness (DOC) can be challenging; the use of observational data collection, individualized treatment programs, and incorporation of preferred, personally meaningful and salient items may be helpful in addressing such challenges during assessment and intervention. In this article, we extend the predominantly adult literature on use of salient items to promote differential responding by describing our methodology to identify preferred items across sensory domains for application during inpatient rehabilitation with children with DOC. METHOD: Details on the indirect and direct preference assessment procedures rooted in applied behavior analysis that we have tailored for this population are provided. We describe steps of the procedures, including structured caregiver interview, staff survey, item inclusion, in vivo single-item stimulus preference assessment, and treatment. Clinical case examples further illustrate implementation of our methodology, observed response topographies, individually identified preferred items, and their application for 3 children in a minimally conscious state. In addition, we introduce a new structured caregiver interview, the Preference Assessment for Youth with Disorders of Consciousness (PAYDOC), modeled on the Reinforcer Assessment for Individuals with Severe Disabilities (RAISD; Fisher, Piazza, Bowman, & Amari, 1996) and modified to be appropriate for future use as a clinical tool to enhance assessment of preferences with this pediatric brain injury population. IMPLICATIONS: This methodology can be used to identify highly idiosyncratic stimuli that can be incorporated in multiple ways throughout rehabilitation to optimize care for youth with DOC. (PsycINFO Database Record

Technical tips: performing EEGs and polysomnograms on children with neurodevelopmental disabilities.

Electroencephalograms (EEGs) and polysomnograms (PSGs) are critical and frequently ordered tests in the care of children with neurodevelopmental disabilities (NDD). Performing studies with this population can be very intimidating, given that the referral reasons and seizure types can be unique, and children with NDD may have any combination of behavioral or sensory challenges that can make it difficult to successfully complete a study. This article presents a variety of strategies that can be used to overcome these challenges through good preparation, patience, caregiver involvement, effective behavioral management techniques, and education about the medical aspects of EEG/ PSG in NDD. This Technical Tips article features ideas and experiences from an EEG/PSG technologist, two board-certified child neurologists (one who is further certified in Clinical Neurophysiology, while the other is further certified in Sleep Medicine), and two behaviorally trained pediatric psychologists.

Development and validation of the Family Health Behavior Scale.

OBJECTIVE: The purpose of the current study was to develop a psychometrically sound, parent-report measure of family and child behaviors related to obesity in children between 5- and 12-years-old. METHODS: Item generation, item selection, and initial exploratory factor analysis yielded a 27-item measure called the Family Health Behavior Scale (FHBS). RESULTS: The FHBS contains four subscales measuring health-promoting family behaviors, obesogenic behaviors, meal-time routines, and family physical activity habits. Parent ratings on the FHBS were shown to predict their child's weight classification. The FHBS also demonstrated adequate internal consistency and temporal stability. CONCLUSIONS: The results of the study suggest that the FHBS is a promising measure of family eating and physical activity habits related to obesity in children.

The moderating effects of maternal psychopathology on children's adjustment post-Hurricane Katrina.

This study investigated the role of maternal psychopathology in predicting children's psychological distress in a disaster-exposed sample. Participants consisted of 260 children (ages 8-16) recruited from public schools and their mothers. These families were displaced from New Orleans because of Hurricane Katrina in 2005. Assessment took place 3 to 7 months postdisaster. Hierarchical regression analyses revealed that global maternal psychological distress and maternal posttraumatic stress disorder moderated the relation between child hurricane exposure and mother-reported child internalizing and externalizing symptoms.