Neurobehavioral Phenotype of Children With Congenital Myotonic Dystrophy.

BACKGROUND AND OBJECTIVES: To describe the neurobehavioral phenotype of congenital myotonic dystrophy. Congenital myotonic dystrophy (CDM) is the most severe form of myotonic dystrophy, characterized by symptom presentation at birth and later, cognitive impairment, autistic features, and disordered sleep. METHODS: The neurobehavioral phenotype was assessed in this cross-sectional study by a neuropsychological battery consisting of the Wechsler Preschool and Primary Scale of Intelligence, Third Edition, Weschler Intelligence Scale for Children, Fourth Edition, Vineland Adaptive Behavior Scale, Second Edition (Vineland-II), Behavior Rating Inventory of Executive Function including preschool and teacher reports, Autism Spectrum Screening Questionnaire, Social Communication Scale, and Repetitive Behavior Scale-Revised. Sleep quality was evaluated with the Pediatric Sleep Questionnaire and Pediatric Daytime Sleepiness Scale. RESULTS: Fifty-five children with CDM, ages 5 weeks to 14 years, were enrolled. The mean age and (CTG)n repeats (±SD) were 6.4 ± 3.8 years and 1,263 ± 432, respectively. The mean IQ was 64.1 ± 14.9 on the Weschler scales with 65.6% of participants falling in the extremely low range for IQ. Adaptive functioning was significantly low for 57.1% of participants (n = 20). Caregiver report of executive functioning indicated 23.1% (9/39) of participants had clinically elevated levels of dysfunction, though teacher report was discrepant and indicated 53.3% of participants with CDM fell in this range (8/15). Spearman correlations were strongly positive (p ≤ 0.05) for estimated full scale IQ, overall adaptive functioning and with daily living and socialization domain standard scores on the Vineland-II ranging from r = 0.719 to r = 0.849 for all ages. Aspects of executive function were directly related to features of autism and sleep quality. Social communication was inversely related to all aspects of daily functioning, except communication, and directly related to aspects of autism behavior. DISCUSSION: Depressed IQ, adaptive skills, and executive functioning, poor sleep quality, and features of autism and altered social functioning individually describe different aspects of the neurobehavioral phenotype in CDM. These neurobehavioral and sleep measures could help quantitatively measure and assess the burden of cognitive impairment in CDM.

Brief video enhances teacher trainees' knowledge of epilepsy.

OBJECTIVE: Relative to the general population, children with epilepsy (CWE) demonstrate difficulties in aspects of cognition and mental health. Previous studies have found that teachers have poor knowledge about epilepsy, which may lead to inadequate management of CWE's comorbidities and seizures, potentially impacting CWE's school performance, social development, and safety. The current study aimed to evaluate whether a brief training program improves teachers' epilepsy knowledge. METHOD: Participants included pre-service teachers enrolled in the Faculty of Education at Western University (London, Ontario, Canada). There were no exclusion criteria. Pre-service teachers completed a questionnaire evaluating their experiences and knowledge of epilepsy prior to, and two weeks following, the training program. The training program consisted of a 10.5-min video and booklet that described the characteristics of seizures, seizure first aid, and the cognitive, psychological, and social consequences of epilepsy. RESULTS: Pre-service teachers' knowledge about epilepsy, seizure safety, and common comorbidities associated with epilepsy significantly improved after participating in the training program. Furthermore, pre-service teachers indicated greater self-reported knowledge about epilepsy and greater preparedness to handle a seizure in the classroom. CONCLUSION: The results of the current study demonstrated that a brief, cost-effective training program significantly enhances teachers' knowledge about epilepsy, seizure safety, and the comorbidities associated with epilepsy. Enhancing teachers' knowledge about epilepsy has the potential to improve children's academic and social success in school.

A live-online mindfulness-based intervention for children living with epilepsy and their families: protocol for a randomized controlled trial of Making Mindfulness Matter©.

BACKGROUND: Epilepsy extends far beyond seizures; up to 80% of children with epilepsy (CWE) may have comorbid cognitive or mental health problems, and up to 50% of parents of CWE are at risk for major depression. Past research has also shown that family environment has a greater influence on children's and parents' health-related quality of life (HRQOL) and mental health than epilepsy-related factors. There is a pressing need for low-cost, innovative interventions to improve HRQOL and mental health for CWE and their parents. The aim of this randomized controlled trial (RCT) is to evaluate whether an interactive online mindfulness-based intervention program, Making Mindfulness Matter (M3), can be feasibly implemented and whether it positively affects CWE's and parents' HRQOL and mental health (specifically, stress, behavioral, depressive, and anxiety symptoms). METHODS: This parallel RCT was planned to recruit 100 child-parent dyads to be randomized 1:1 to the 8-week intervention or waitlist control and followed over 20 weeks. The intervention, M3, will be delivered online and separately to parents and children (ages 4-10 years) in groups of 4-8 by non-clinician staff of a local community epilepsy agency. The intervention incorporates mindful awareness, social-emotional learning skills, and positive psychology. It is modeled after the validated school-based MindUP program and adapted for provision online and to include a parent component. DISCUSSION: This RCT will determine whether this online mindfulness-based intervention is feasible and effective for CWE and their parents. The proposed intervention may be an ideal vector to significantly improve HRQOL and mental health for CWE and their parents given its low cost and implementation by community epilepsy agencies. TRIAL REGISTRATION: ClinicalTrials.gov NCT04020484 . Registered on July 16, 2019.

Child-related disagreements, verbal aggression, and children's internalizing and externalizing behavior problems.

Dual-income parents (N = 122 couples) with an oldest child 18-60 months of age completed the Child-Rearing Disagreements Scale (E. N. Jouriles et al., 1991), the Child Behavior Checklist (T. M. Achenbach & L. A. Rescorla, 2000), and the Verbal Aggression subscale of the Conflicts and Problem-Solving Scale (P. K. Kerig, 1996). Replicating the results of E. N. Jouriles et al. (1991) and extending these findings to daughters and fathers, the authors found links between child-related disagreements and parental ratings of child behavior problems in this low-risk sample. There were no links between fathers' reports of verbal aggression and child behavior problems. Among mothers, however, use of verbal aggression mediated the link between child-related disagreements and ratings of sons' internalizing problems. Verbal aggression did not moderate the link between child-related disagreements and child behavior problems for either mothers or for fathers.