An eHealth Program for Insomnia in Children With Neurodevelopmental Disorders (Better Nights, Better Days): Protocol for an Economic Evaluation of a Randomized Controlled Trial.

BACKGROUND: Children with neurodevelopmental disorders have a high risk of sleep disturbances, with insomnia being the most common sleep disorder (ie, chronic and frequent difficulties with going and staying asleep). Insomnia adversely affects the well-being of these children and their caregivers. Pediatric sleep experts recommend behavioral interventions as the first-line treatment option for children. Better Nights, Better Days for Children with Neurodevelopmental Disorders (BNBD-NDD) is a 5-session eHealth behavioral intervention delivered to parents to improve outcomes (eg, Pediatric Quality of Life Inventory [PedsQL]) for their children (ages 4-12 years) with insomnia and who have a diagnosis of mild to moderate attention-deficit/hyperactivity disorder, autism spectrum disorder, cerebral palsy, or fetal alcohol spectrum disorder. If cost-effective, BNBD-NDD can be a scalable intervention that provides value to an underserved population. OBJECTIVE: This protocol outlines an economic evaluation conducted alongside the BNBD-NDD randomized controlled trial (RCT) that aims to assess its costs, efficacy, and cost-effectiveness compared to usual care. METHODS: The BNBD-NDD RCT evaluates the impacts of the intervention on children's sleep and quality of life, as well as parents' daytime functioning and psychosocial health. Parent participants were randomized to the BNBD-NDD treatment or to usual care. The economic evaluation assesses outcomes at baseline and 8 months later, which include the PedsQL as the primary measure. Quality of life outcomes facilitate the comparison of competing interventions across different populations and medical conditions. Cost items include the BNBD-NDD intervention and parent-reported usage of private and publicly funded resources for their children's insomnia. The economic evaluation involves a reference case cost-effectiveness analysis to examine the incremental cost of BNBD-NDD per units gained in the PedsQL from the family payer perspective and a cost-consequence analysis from a societal perspective. These analyses will be conducted over an 8-month time horizon. RESULTS: Research funding was obtained from the Kids Brain Health Network in 2015. Ethics were approved by the IWK Health Research Ethics Board and the University of Calgary Conjoint Health Research Ethics Board in January 2019 and June 2022, respectively. The BNBD-NDD RCT data collection commenced in June 2019 and ended in April 2022. The RCT data are currently being analyzed, and data relevant to the economic analysis will be analyzed concurrently. CONCLUSIONS: To our knowledge, this will be the first economic evaluation of an eHealth intervention for insomnia in children with neurodevelopmental disorders. This evaluation's findings can inform users and stakeholders regarding the costs and benefits of BNBD-NDD. TRIAL REGISTRATION: ClinicalTrial.gov NCT02694003; https://clinicaltrials.gov/study/NCT02694003. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/46735.

Physical Activity and Sedentary Behavior Patterns Across Weekdays and Weekend Days in Youth With Multiple Sclerosis and Controls.

Background: This study quantified and compared weekday and weekend patterns of device-measured physical activity (PA) and sedentary behavior between youth with pediatric multiple sclerosis (MS) and controls for the purpose of informing future PA behavior change interventions. Methods: Participant data were obtained from 3 ongoing observational studies, and the sample included 40 participants with pediatric MS and 41 controls. Light PA (LPA), moderate to vigorous PA (MVPA), and sedentary behavior data were collected using activity monitors (ActiGraph LLC) over 1 week. The main analysis involved a 2-way mixed factor analysis of variance with group as a between-subjects factor (pediatric MS vs control) and day as a within-subjects factor (weekday vs weekend day). Results: There was no group by day interaction from the analysis of variance for percentage of activity monitor wear time spent in LPA, MVPA, or sedentary behavior. There was no effect of group for LPA, MVPA, or sedentary behavior. There was an effect of day of week on percentage of day spent in LPA, MVPA, and sedentary behavior. Conclusions: These results suggest that youth with pediatric MS and controls were less physically active and more sedentary on weekends than on weekdays, but there were no differences between groups in PA and sedentary behavior overall or by day of the week. Physical activity interventions may be more successful by initially targeting weekend day activity.

Children's Sleep During COVID-19: How Sleep Influences Surviving and Thriving in Families.

OBJECTIVE: The COVID-19 pandemic has the potential to disrupt the lives of families and may have implications for children with existing sleep problems. As such, we aimed to: (1) characterize sleep changes during the COVID-19 pandemic in children who had previously been identified as having sleep problems, (2) identify factors contributing to sleep changes due to COVID-19 safety measures, and (3) understand parents' and children's needs to support sleep during the pandemic. METHODS: Eighty-five Canadian parents with children aged 4-14 years participated in this explanatory sequential, mixed-methods study using an online survey of children's and parents' sleep, with a subset of 16 parents, selected based on changes in their children's sleep, participating in semi-structured interviews. Families had previously participated in the Better Nights, Better Days (BNBD) randomized controlled trial. RESULTS: While some parents perceived their child's sleep quality improved during the COVID-19 pandemic (14.1%, n = 12), many parents perceived their child's sleep had worsened (40.0%, n = 34). Parents attributed children's worsened sleep to increased screen time, anxiety, and decreased exercise. Findings from semi-structured interviews highlighted the effect of disrupted routines on sleep and stress, and that stress reciprocally influenced children's and parents' sleep. CONCLUSIONS: The sleep of many Canadian children was affected by the first wave of the COVID-19 pandemic, with the disruption of routines influencing children's sleep. eHealth interventions, such as BNBD with modifications that address the COVID-19 context, could help families address these challenges.

Cognitive outcome in children with infantile spasms using a standardized treatment protocol. A five-year longitudinal study.

AIM: To evaluate the long-term developmental trajectory of children with infantile spasms (IS) and identify the clinical protective and risk factors associated with their cognitive outcome. METHODS: We analyzed the five-year follow-up results of 41 children (13 female) from the previously published cohort (n = 68) recruited in a multicenter randomized controlled trial for 2-years, examining the effect of an adjunctive therapy (Flunarizine) on standardized IS treatment. The children were subsequently monitored in an open-label study for additional 3 years.  The Vineland Adaptive Behavior Scale, second edition, and either the Stanford-Binet Intelligence Scale, Fifth Edition (SB5) or the Bayley Scales of Infant Development, second edition (BSID-II) were used as cognitive outcome measures. RESULTS: Etiology was the strongest predictor of outcome. Children with no identified etiology (NIE) showed a progressive improvement of cognitive functions, mostly occurring between 2 and 5 years post-diagnosis.  Conversely, symptomatic etiology was predictive of poorer cognitive outcome. Developmental delay, other seizure types (before and after IS diagnosis), and persistent electroencephalographic abnormalities following treatment were predictive of poor cognitive outcome. INTERPRETATION: Given the 5-year cognitive improvement, children with IS should undergo a developmental assessment before school entry. Factors influencing their cognitive outcome emphasize the importance of thorough investigation and evidence-based treatment.

Objective Sleep Characteristics and Factors Associated With Sleep Duration and Waking During Pediatric Hospitalization.

Importance: Children's sleep may be affected by hospitalization, yet few objective determinations of sleep patterns are reported for children in intensive care or general medicine units. There is limited research on relationships between sleep in hospital and child (eg, age, pain), treatment (eg, medications, nurse presence), or environmental (eg, noise, light, type of unit) factors. Objective: To determine sleep quantity and patterns in hospitalized children and determine factors associated with sleep quantity and nighttime waking for children in hospital. Design, Setting, and Participants: This was a prospective cross-sectional study of children admitted to a general pediatric unit or a pediatric intensive care unit at a pediatric quaternary teaching hospital in Toronto, Ontario, Canada, from October 2007 to July 2008. Participants included children aged 1 to 18 years who were expected to stay in hospital for at least 2 nights. Demographic data, information about the hospital stay and illness, and usual sleep habits were collected. Children wore an actigraph for 1 to 3 consecutive days and nights and completed a sleep diary. Sound and light meters were placed at the bedside. Data analyses took place in April 2009. Main Outcomes and Measures: The primary outcome was the mean number of minutes of child nighttime sleep from 7:30 pm to 7:29 am. Sleep variables were averaged over days and nights recorded (mean [SD] days and nights of wear, 2.54 [0.71]) and examined for associations with sleep quantity and patterns, as well as hazard of waking in the night. Results: Of 124 eligible children approached for inclusion, 69 children consented (35 [51%] female; 20 [29%] aged 1-3 years, 10 [14%] aged 4-7 years, 17 [24%] aged 8-12 years, and 22 [32%] aged 13-18 years; 58 [84%] in the general pediatric unit). Children aged 1 to 3, 4 to 7, 8 to 12, and 13 to 18 years obtained a mean (SD) of 444 (132), 475 (86), 436 (114), and 384 (83) minutes of nighttime sleep, respectively; mean (SD) number of night awakenings was 14 (3), 18 (3), 14 (8), and 12 (6), respectively. Children on general pediatric units slept 258 minutes more per night than children sleeping in the pediatric intensive care unit (95% CI, 165.16-350.56 minutes; P < .001), children admitted for planned surgery slept 123 minutes more than children admitted for exacerbations of chronic illness (95% CI, 49.23-196.01 minutes; P < .01), and children sleeping in rooms with other patients slept 141 minutes fewer than children in private rooms (95% CI, -253.51 to -28.35 minutes; P = .01). Sound events greater than 80 dB were significantly associated with increased risk of instantaneous waking (hazard ratio [HR], 1.35; 95% CI, 1.02-1.80; P = .04), as were light events greater than 150 lux (HR, 1.17; 95% CI, 1.01-1.36; P = .03), receiving a medication that promoted sleep (HR, 1.04; 95% CI, 1.00-1.08; P = .03), and having a nurse in the room for most or all of the night (HR, 1.08; 95% CI, 1.03-1.13; P = .003). Sleeping on the general pediatrics unit was significantly associated with decreased risk of instantaneous waking (HR, 0.81; 95% CI, 0.77-0.85; P < .001), as was being admitted for planned surgery (HR, 0.95; 95% CI, 0.91-0.99; P = .04), receiving a medication that promoted wakefulness (HR, 0.96; 95% CI, 0.93-0.995; P = .02), and sharing a room with another patient (HR, 0.78; 95% CI, 0.72-0.84; P < .001). Conclusions and Relevance: In this cross-sectional study of hospitalized children, children experienced considerable nighttime waking and sleep restriction to levels below national clinical recommendations at a time when they most needed the benefits of sleep. Given light and noise were the greatest contributors to nighttime waking in hospital, clinicians, administrators and hospital design experts should work together for solutions.

INSPIRED but Tired: How Medical Faculty's Job Demands and Resources Lead to Engagement, Work-Life Conflict, and Burnout.

BACKGROUND: Past research shows that physicians experience high ill-being (i.e., work-life conflict, stress, burnout) but also high well-being (i.e., job satisfaction, engagement). OBJECTIVE: To shed light on how medical faculty's experiences of their job demands and job resources might differentially affect their ill-being and their well-being with special attention to the role that the work-life interface plays in these processes. METHODS: Qualitative thematic analysis was used to analyze interviews from 30 medical faculty (19 women, 11 men, average tenure 13.36 years) at a top research hospital in Canada. FINDINGS: Medical faculty's experiences of work-life conflict were severe. Faculty's job demands had coalescing (i.e., interactive) effects on their stress, work-life conflict, and exhaustion. Although supportive job resources (e.g., coworker support) helped to mitigate the negative effects of job demands, stimulating job resources (e.g., challenging work) contributed to greater work-life conflict, stress, and exhaustion. Thus, for these medical faculty job resources play a dual-role for work-life conflict. Moreover, although faculty experienced high emotional exhaustion, they did not experience the other components of burnout (i.e., reduced self-efficacy, and depersonalization). Some faculty engaged in cognitive reappraisal strategies to mitigate their experiences of work-life conflict and its harmful consequences. CONCLUSION: This study suggests that the precise nature and effects of job demands and job resources may be more complex than current research suggests. Hospital leadership should work to lessen unnecessary job demands, increase supportive job resources, recognize all aspects of job performance, and, given faculty's high levels of work engagement, encourage a climate that fosters work-life balance.

Sleep phenotype of individuals with autism spectrum disorder bearing mutations in the PER2 circadian rhythm gene.

The Per family of genes functions as a primary circadian rhythm maintenance in the brain. Mutations in PER2 are associated with familial advanced sleep-phase syndrome 1 (FASPS1), and recently suggested in delayed sleep phase syndrome and idiopathic hypersomnia. The detection of PER2 variants in individuals with autism spectrum disorder (ASD) and without reported sleep disorders, has suggested a role of circadian-relevant genes in the pathophysiology of ASD. It remains unclear whether these individuals may have, in addition to ASD, an undiagnosed circadian rhythm sleep disorder. The MSSNG database was used to screen whole genome sequencing data of 5,102 individuals with ASD for putative mutations in PER2. Families identified were invited to complete sleep phenotyping consisting of a structured interview and two standardized sleep questionnaires: the Pittsburgh Sleep Quality Index and the Morningness-Eveningness Questionnaire. From 5,102 individuals with ASD, two nonsense, one frameshift, and one de novo missense PER2 variants were identified (0.08%). Of these four, none had a diagnosed sleep disorder. Three reported either a history of, or ongoing sleep disturbances, and one had symptoms highly suggestive of FASPS1 (as did a mutation carrier father without ASD). The individual with the missense variant did not report sleep concerns. The ASD and cognitive profiles of these individuals varied in severity and symptoms. The results support a possible role of PER2-related circadian rhythm disturbances in the dysregulation of sleep overall and sometimes FASPS1. The relationship between dysregulated sleep and the pathophysiology of ASD require further exploration.

Barriers and facilitators to treating insomnia in children with autism spectrum disorder and other neurodevelopmental disorders: Parent and health care professional perspectives.

BACKGROUND/AIMS: Insomnia is highly prevalent in children with neurodevelopmental disorders (NDDs), yet little research exists on sleep treatment access, utilization, and provision in this population. This study explores barriers and facilitators to access, use, and provision of treatment for sleep problems as experienced by parents of children with NDDs, including Autism Spectrum Disorder (ASD), Attention-Deficit/Hyperactivity Disorder (ADHD), Cerebral Palsy (CP) and Fetal Alcohol Spectrum Disorder (FASD), and health care professionals who work with children with these conditions. METHOD: Transcripts from online focus groups and interviews, conducted separately with parents of children with NDDs (n = 43) and health care professionals (n = 44), were qualitatively analyzed using content analysis for key themes. RESULTS: Barriers included limited access to/availability of treatment, lack of knowledge/training, NDD-specific factors (e.g., symptoms, medications, and comorbidities), parent factors (e.g., capacity to implement treatment, exhaustion), and the challenging, intensive nature of sleep treatment. Facilitators included positive beliefs and attitudes, education, support, and ability to modify treatments for NDD symptoms. Barriers and facilitators were similar across all four NDDs. CONCLUSIONS: Results highlight a need for more education about sleep in NDDs and to develop accessible interventions, as well as the potential of a transdiagnostic approach to sleep treatment in this population.

An eHealth insomnia intervention for children with neurodevelopmental disorders: Results of a usability study.

BACKGROUND: Sleep problems, particularly insomnia, are highly prevalent in children with neurodevelopmental disorders (NDD) and can negatively affect health and development. eHealth interventions may increase access to evidence-based care for insomnia for children with NDD, as programs are rare in most communities. Better Nights, Better Days (BNBD) is an online, parent-implemented intervention for pediatric insomnia in typically developing 1- to 10-year-olds. AIMS: The present study examined whether parents of children with NDD perceived the original BNBD to be usable, acceptable, and feasible, and what modifications might be necessary to adapt it for children with NDD. METHODS AND PROCEDURES: Twenty Canadian parents/caregivers of children aged 4-10 years with NDD and insomnia implemented the BNBD intervention with their children, and completed usability questionnaires. Questionnaire data were analyzed quantitatively (descriptive statistics) and qualitatively (thematic analysis). OUTCOMES AND RESULTS: Participants reported the intervention to be usable, useful, acceptable, and feasible. Several modifications were suggested to make the intervention more appropriate and acceptable for use with children with NDD. CONCLUSIONS AND IMPLICATIONS: Results support a largely transdiagnostic approach to treating sleep in children with NDD, and will inform the development of BNBD for Children with Neurodevelopmental Disorders (BNBD-NDD).

Randomized, Placebo-Controlled Trial of Ferrous Sulfate to Treat Insomnia in Children With Autism Spectrum Disorders.

BACKGROUND: Insomnia and low iron stores are common in children with autism spectrum disorders, and low iron stores have been associated with sleep disturbance. METHODS: We performed a randomized placebo-controlled trial of oral ferrous sulfate to treat insomnia in children with autism spectrum disorders and low normal ferritin levels. Twenty participants who met inclusion criteria and whose insomnia did not respond to sleep education were randomized to 3 mg/kg/day of ferrous sulfate (n = 9) or placebo (n = 11) for three months. RESULTS: Iron supplementation was well tolerated, and no serious adverse events were reported. Iron supplementation improved iron status (+18.4 ng/mL active versus -1.6 ng/mL placebo, P = 0.044) but did not significantly improve the primary outcome measures of sleep onset latency (-11.0 minutes versus placebo, 95% confidence interval -28.4 to 6.4 minutes, P = 0.22) and wake time after sleep onset (-7.7 minutes versus placebo, 95% confidence interval -22.1 to 6.6 min, P = 0.29) as measured by actigraphy. Iron supplementation was associated with improvement in the overall severity score from the Sleep Clinical Global Impression Scale (-1.5 points versus placebo, P = 0.047). Changes in measures of daytime behavior did not differ between groups. CONCLUSION: This trial demonstrated no improvement in primary outcome measures of insomnia in subjects treated with ferrous sulfate compared with placebo. Interpretation was limited by low enrollment.

Family functioning among adolescents with narcolepsy.

BACKGROUND: Narcolepsy is a sleep disorder with no cure with onset typically during adolescence. Caring for an adolescent with a lifelong medical condition can negatively impact family structure, cohesion, relationships, and overall functioning. The primary objective of this study was to evaluate family functioning in a cohort of adolescents with narcolepsy using the PedsQL Family Impact Module. The secondary objective was to compare family functioning in adolescents with narcolepsy to adolescents with chronic pain based on published data. METHODS: This was a cross-sectional study of adolescents (aged 10 to 18 years) with narcolepsy. The narcolepsy group was recruited from The Hospital for Sick Children in Toronto, Canada. Family functioning was assessed by the PedsQL family impact module total scores, which was completed by the patient's caregiver. The PedsQL family impact module yields a total scale which encompasses parent health-related quality of life, daily activities, family relationships, communication, and worry subscales. Lower scores suggest poorer family functioning. Secondary data analyses were used to compare participants' family functioning to a cohort of adolescents with chronic pain. RESULTS: Thirty adolescents with narcolepsy participated (mean age=13.8 ± 2.2 years, 76.7% male). Family functioning was impaired in this cohort of adolescents with narcolepsy and similar to adolescents with chronic pain (64.0 ± 19.8 versus 64.7 ± 19.5; P=0.849). CONCLUSION: Family functioning is impaired in adolescents with narcolepsy. Clinical teams should assess family functioning at routine clinic visits by asking about concerns and challenges related to caring for an adolescent with narcolepsy and providing resources and support as needed.

Utility of depth electrode placement in the neurosurgical management of bottom-of-sulcus lesions: technical note.

OBJECTIVE: Small lesions at the depth of the sulcus, such as with bottom-of-sulcus focal cortical dysplasia, are not visible from the surface of the brain and can therefore be technically challenging to resect. In this technical note, the authors describe their method of using depth electrodes as landmarks for the subsequent resection of these exacting lesions. METHODS: A retrospective review was performed on pediatric patients who had undergone invasive electroencephalography with depth electrodes that were subsequently used as guides for resection in the period between July 2015 and June 2017. RESULTS: Ten patients (3-15 years old) met the criteria for this study. At the same time as invasive subdural grid and/or strip insertion, between 2 and 4 depth electrodes were placed using a hand-held frameless neuronavigation technique. Of the total 28 depth electrodes inserted, all were found within the targeted locations on postoperative imaging. There was 1 patient in whom an asymptomatic subarachnoid hemorrhage was demonstrated on postprocedural imaging. Depth electrodes aided in target identification in all 10 cases. CONCLUSIONS: Depth electrodes placed at the time of invasive intracranial electrode implantation can be used to help localize, target, and resect primary zones of epileptogenesis caused by bottom-of-sulcus lesions.

Depressive symptoms, sleep patterns, and physical activity in adolescents with narcolepsy.

STUDY OBJECTIVES: To evaluate the association between depressive symptoms, sleep patterns (duration and quality), excessive daytime sleepiness (EDS), and physical activity (PA) in adolescents with narcolepsy. METHODS: This cross-sectional study included adolescents (ages 10-18 years) with narcolepsy attending a tertiary care facility (The Hospital for Sick Children, Toronto, Canada). Adolescents with narcolepsy completed questionnaires evaluating depressive symptoms (Children's Depression Inventory-2nd edition [CDI-2]), sleep quality (Pittsburgh Sleep Quality Index), EDS (Epworth Sleepiness Scale), and PA (Godin Leisure-Time Exercise Questionnaire). Wrist-based actigraphy was worn by adolescents for 1 week to measure total sleep time (over 24 hr) and sleep efficiency percentage. RESULTS: Thirty adolescents with narcolepsy (mean age = 13.8 ± 2.2 years, 76.7% male) participated. In this cohort of adolescents with narcolepsy, 23.3% had CDI-2 total scores in the elevated range. Greater CDI-2 total scores were associated with poor sleep quality (ρ = 0.571; p = 0.02), EDS (ρ = 0.360; p = 0.05), and lower self-reported PA levels (ρ = -0.512; p < 0.01). CONCLUSIONS: Adolescents with narcolepsy report experiencing depressive symptoms, which are associated with poor sleep quality, EDS, and low PA levels. Strategies to improve nocturnal sleep quality and symptoms of EDS as well as promoting increased PA levels in adolescents with narcolepsy may provide an opportunity to improve depressive symptoms in this population. Multidisciplinary care with mental health and sleep specialists for adolescents with narcolepsy is needed.

A child with a stroke, drug-refractory epilepsy and congenital heart disease: can a hemispherectomy be safely performed between staged cardiac procedures?

BACKGROUND: The safety of hemispherectomy between staged cardiac procedures is unknown and not previously reported. METHOD: Retrospective review of a case with drug-resistant epilepsy due to stroke following bidirectional cavopulmonary connection (BDCPC). RESULTS: This report describes the first case of a successful pediatric peri-insular functional hemispherectomy in the setting of a BDCPC. A discussion of the complex preoperative planning from both a cardiac and neurological perspective is presented. Considerations regarding hemispherectomy and its effects on the cardiac physiology, and perioperative considerations are emphasized in clinical decision making. CONCLUSIONS: A multidisciplinary approach was critical in this child which led to a successful outcome.

MEF2C-related epilepsy: Delineating the phenotypic spectrum from a novel mutation and literature review.

PURPOSE: MEF2C-related epilepsy has been poorly described in the literature, despite a consistent MEF2C haploinsufficiency phenotype characterized by severe language impairment and motor delay (MIM# 613443). We aimed to delineate the spectrum of electroclinical manifestations of MEF2C-related epilepsy from an illustrative case and literature review. METHODS: A retrospective chart review of our case was performed followed by a literature review on PubMed and OMIM. Publications including patients with MEF2C pathogenic, likely pathogenic variants, or microdeletions without involvement of other genes were selected. RESULTS: The index case is a 2-year-old male with global developmental delay who presented at 7 months with atypical febrile seizures, generalized myoclonias, and focal impaired awareness seizures. Neuroimaging studies were unremarkable and electroencephalograms showed high voltage 200-400uV, 2-2.5 Hz generalized spike-and-waves and polyspikes with alternating frontal predominance, and multifocal spike-and-slow waves. Whole exome sequencing showed an unreported de novo likely pathogenic variant in the MEF2C gene c.236 G > C (p.Arg79Pro). Data from ten additional publications including 22 patients were gathered. From the 23 patients in total, 19 (82%) had seizures. Febrile seizures were most common, but myoclonic, focal-onset and generalized seizures were also reported. Electroencephalogram findings were described in eleven, and nine (82%) showed epileptiform abnormalities. CONCLUSION: MEF2C-related epilepsy may be described as a spectrum of manifestations including febrile seizures, myoclonia, and focal-onset or generalized seizures. Electroencephalogram is consistently abnormal, showing findings such as background slowing, multifocal and generalized epileptiform discharges and polyspikes. It remains unclear whether most patients are responsive or refractory to treatment with anti-epileptic medications.

The impact of the Canadian Sleep Society on sleep science, clinical practice, and advocacy: past, present, and future.

The Canadian Sleep Society/Societe Canadienne du Sommeil (CSS-SCS), is the only Canadian national organization which is dedicated to the clinical practice of sleep medicine as well as the advancement of sleep science. This article outlines the extent to which the CSS-SCS has shaped the field of sleep science and practice in Canada. A description of the CSS-SCS, the history of the CSS, the impact associated with the Society's activities, with an emphasis on the society's contribution to pediatric sleep, and future directions are outlined.

Pediatric sleep disorder medicine training in Canada: past, present and future.

Pediatric sleep medicine is rapidly evolving in Canada. As pediatric sleep disorders are increasingly recognized, there is a growing need for clinicians educated in the evaluation and treatment of pediatric sleep disorders. Current pediatric sleep resources in Canada are inadequate to meet the needs of the population. Until this year, there was no formal pathway or specific requirements for pediatric sleep disorder medicine training in Canada and exposure to this field of medicine in post-graduate training was limited. In 2018, the Royal College of Physicians of Canada approved an Area of Focused Competence program for certification and maintenance of competence in Sleep Disordered Medicine. It was designed to ensure adequate breadth and depth of training experiences in this diverse field. The goals of the program are to ensure competence in the assessment and management of adults and children with a range of sleep-wake disorders and sleep-disordered breathing, to interpret sleep investigations, to administratively manage a sleep laboratory and to advance the discipline of Sleep Disorder Medicine through research. The program follows a competency-based model in which, within the year of training, trainees accumulate a dossier of experiences which are evaluated by a Royal College of Physicians and Surgeons committee. This new program will ensure that a critical mass of trained sleep medicine physicians is developed to meet the needs of the Canadian pediatric population and to contribute to advancement of the field of pediatric sleep medicine.

A retrospective analysis of clinical characteristics of childhood narcolepsy.

AIM: Narcolepsy, encompassing excessive daytime sleepiness (EDS), cataplexy, sleep paralysis and hypnogogic hallucinations, was previously considered rare in childhood. Recently, cases of childhood narcolepsy have increased significantly and the reasons for this may include the increasing awareness of narcolepsy as well as the H1N1 vaccination. The aim of this study was to describe the clinical characteristics of childhood narcolepsy, specifically focusing on cataplexy subtypes that may facilitate early recognition of narcolepsy. METHODS: We retrospectively reviewed and analyzed the medical records of 33 children diagnosed with narcolepsy at the Hospital for Sick Children, in Toronto, Ontario. All patients were seen prior to 18 years of age and symptoms were self-reported by parents and/or children themselves. RESULTS: At presentation, 32 of 33 children reported EDS and 28 of 33 reported cataplexy. Among the 28 patients with cataplexy, 18 of 28 reported cataplexy referred to as 'cataplectic facies' (e.g., facial hypotonia and/or tongue protrusion) while 10 of 28 patients reported characteristic cataplexy, defined as bilateral loss of muscle tone. Children with cataplectic facies reported higher BMI z-scores compared to those with characteristic cataplexy, 1.8 and 0.8, respectively. Children with cataplectic facies also tended to be younger than those with characteristic cataplexy, 9.2 and 11.8 years of age, respectively. Cataplectic facies appear to be related to narcolepsy close to disease onset. CONCLUSIONS: Children, especially young, obese children, presenting with a history of EDS with associated facial hypotonia or tongue protrusion raises the index of suspicion of narcolepsy and should prompt a referral to a specialized sleep facility to establish the diagnosis.

Influence of sleep on developing brain functions and structures in children and adolescents: A systematic review.

This systematic review examined the associations between sleep and brain functions and structures in children and adolescents aged 1-17 ys. Included studies (n = 24) were peer-reviewed and met the a priori determined population (apparently healthy children and adolescents aged 1 y to 17 ys), intervention/exposure/comparator (various sleep characteristics including duration, architecture, quality, timing), and outcome criteria (brain functions and/or brain structures, excluding cognitive function outcomes). Collectively, the reviewed studies report some relationships between inadequate sleep and resultant differences in brain functions or structures. Although the research presented supports and offers more insight into the importance of sleep for the developing brain of children and adolescents, no firm conclusions that apply broadly may be drawn from these results, particularly because of the diversity of the sleep variables and outcomes. However, it is clear that sleeping habits in the pediatric population should be prioritized. Health care providers should continue to recommend healthy sleep practices and adequate time for sleep, as they are essential for overall health, including brain health.

Children with autism spectrum disorders and drug-resistant epilepsy can benefit from epilepsy surgery.

OBJECTIVE: The objective of this research was to evaluate a cohort of children with both autism spectrum disorder (ASD) and drug-resistant epilepsy (DRE) after epilepsy surgery to determine predictors of best outcome. METHODS: Retrospective chart review was done for 29 children ages 2 to 18 years with ASD and DRE who had neurosurgical intervention for seizure management over 15 years at one institution. All subjects had at least 1 year of follow-up. Data abstraction included demographic information, seizure diagnosis, treatment, investigations, surgical intervention, neuropsychological assessment, and outcome. Statistical analysis software (SAS) was used for statistical analysis. Engel classification was used to assess seizure outcome. RESULTS: Fifteen subjects had resective surgery. Fourteen had palliative surgery with vagal nerve stimulator (VNS) insertion (13) and corpus callosotomy (1). Of the 29 subjects, 35% had class I outcome (all in the resective group). When combining all subjects (resective and palliative), 66% of subjects benefited with class I-III outcomes. In the total cohort, age at time of surgery was significant, with class I outcome more frequently seen in the younger age group when compared with classes II-IV (p = 0.01). CONCLUSION: A subset of children with ASD can benefit from resective surgery, and for those who are not candidates, a VNS can offer significant improvements in seizure control.