Search

BACKGROUND Children with neurodevelopmental disorders have a high risk of sleep disturbances, with insomnia being the most common sleep disorder (i.e., 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 five-session eHealth behavioural intervention delivered to parents to improve outcomes (e.g., Pediatric Quality of Life Inventory) for their children (4-12 years) with insomnia and who have a diagnosis of mild-to-moderate attention-deficit/hyperactivity disorder, autism spectrum disorder, cerebral palsy, and/or fetal alcohol spectrum disorder. If 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 and 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 parent 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 Pediatric Quality of Life Inventory (PedsQL) as the primary measure. Quality of life outcomes facilitates the comparison of competing interventions across different populations and medical conditions. Cost items include the BNBD-NDD intervention and parent-reported utilization 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 unit(s) gain 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 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. CLINICALTRIAL ClinicalTrial.gov ID NCT02694003

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.

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.

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.

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.

OBJECTIVESmall 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.METHODSA 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.RESULTSTen 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.CONCLUSIONSDepth 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.

The safety of hemispherectomy between staged cardiac procedures is unknown and not previously reported. Retrospective review of a case with drug-resistant epilepsy due to stroke following bidirectional cavopulmonary connection (BDCPC). 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. A multidisciplinary approach was critical in this child which led to a successful outcome.

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.

Objectives/background This paper outlines the current state of Canadian training, clinical services, research, and advocacy initiatives related to non-respiratory sleep disorders, with a specific focus on insomnia, the most common sleep problem in children. Methods Information for this narrative review was collected from peer-reviewed publications, web-resources, and personal communications and experiences. Results It is estimated that approximately one-third of Canadian children and youth present with insomnia, and that this is impacting their physical and mental health, as well as learning in school. Training in pediatric sleep is limited and highly inconsistent within and across disciplines. While there are some publicly and privately funded pediatric sleep services available, these are mostly focused on respiratory sleep problems and are not equally accessible across the country. Conclusions Pediatric assessment and treatment services for non-respiratory sleep disorders needs to be more integrated into the Canadian health care system.

Heterozygous mutations in syntaxin-binding protein 1 (STXBP1) gene are associated with early infantile epileptic encephalopathy 4 (EIEE4). This condition is characterized by epilepsy, developmental delay (DD), and various movement disorders. Herein, we will report 5 unrelated patients with different de novo mutations in STXBP1. In addition, we conducted an online survey through Facebook to identify the incidence of bruxism (BRX) in these patients. Four out of 5 patients (80%) presented with awake BRX (A-BRX). Bruxism was also reported in 81.4% (57/70) of the patients with STXBP1 encephalopathy through the online questionnaire. No consistent correlation was identified between the type of mutation and development of movement disorders or BRX. This is the first study to demonstrate A-BRX in patients with STXBP1 mutation. Given the role of STXBP1 in exocytosis of neurotransmitters and other manifestations of dopamine dysregulation in patients with STXBP1-EIEE4, we suggest that in patients with STXBP1 encephalopathy, A-BRX might be the result of the involvement of dopaminergic circuits.

This cross-sectional study explores the quantity and patterns of sleep in hospitalized children to determine factors associated with sleep quantity and nighttime waking., Key Points Question What is the quantity and patterns of sleep in hospitalized children, and what factors are associated with sleep duration and awakenings? Findings In this cross-sectional study of 69 hospitalized children, those aged 1 to 3, 4 to 7, 8 to 12, and 13 to 18 years obtained only 444, 475, 436, and 384 minutes of nighttime sleep in hospital, respectively, which was less than at home and recommended sleep durations. Sound events exceeding 80 dB and light events greater than 150 lux were associated with increased hazards of nighttime waking. Meaning During hospitalization, children may experience considerable sleep restriction and frequent waking from noise and light., 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

Introduction: Pediatric arterial ischemic stroke (AIS) is a serious cause of lifelong neurological and cognitive disabilities. Although they are at increased risk of obstructive sleep apnea (OSA), children with AIS may have abnormal sleep indices even in the absence of OSA diagnosis. This can adversely affect their blood vessels health (impaired cerebrovascular reactivity, CVR) and can lead to neurocognitive impairments. Therefore, we aimed to investigate the association of cerebral blood vessels health with polysomnographic measures and cognitive function in children with AIS. Hypothesis: Quantitative measure of negative CVR, i.e. fractional negativity (fneg) and negative CVR correlate significantly with abnormal overnight oxygen and carbon dioxide levels in children with AIS, even in the absence of OSA. Methods: We conducted a single-centre cross-sectional study in children with AIS aged between 8 and 18 years old and who were not at risk of OSA (i.e., pediatric sleep questionnaire score (1) Non-invasive Blood Oxygen Level Dependent magnetic resonance imaging to study CVR. (2) An overnight polysomnography (3) Comprehensive evaluation of intellectual abilities, attention and executive function. Results: Ten children with AIS (40% males; median age: 14.3 years) were included after a median of 8.8 years following stroke onset. None of the children was diagnosed with OSA. We found significant correlations between fneg and minimum oxygen saturation during REM sleep (r=0.81, r=0.68 and r=0.76 in grey matter, white matter and whole brain, respectively; p Conclusion: Impaired CVR in children with AIS correlates with abnormal sleep indices even in the absence of OSA diagnosis. This can potentially result in cognitive difficulties, the majority of which remain not fully explained by stroke-related factors.

BackgroundPast 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).ObjectiveTo 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.MethodsQualitative 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.FindingsMedical 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.ConclusionThis 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.

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.

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.

Children and adults with genetic generalized epilepsy may have focal clinical seizure symptoms as well as electroencephalographic (EEG) findings. This may pose a diagnostic challenge to clinicians, especially when concomitant focal neuroimaging findings exist and the epilepsy is medically refractory. We sought to highlight the challenges that clinicians may face through the description of 2 children with suspected genetic generalized epilepsy who had both focal seizure symptoms and EEG/neuroimaging findings and underwent invasive EEG monitoring. Ultimately, invasive monitoring failed to demonstrate a focal origin for the seizures in both cases, and instead confirmed the presence of genetic generalized epilepsy. We demonstrate that ≥3-Hz generalized monomorphic spike and waves are less likely to represent secondary bilateral synchrony, that focal neuroimaging findings may not always be causal and that repeated hyperventilation is an essential activation procedure for genetic generalized epilepsy.

Objectives The objective of this study was to review our experience with intra-operative “train of five” stimulation using subdural grid for motor mapping in children undergoing epilepsy surgery evaluation. Methods Twenty consecutive children below 18-years of age with drug-refractory epilepsy who underwent invasive-EEG monitoring using subdural-grid placement and intra-operative motor mapping using direct cortical stimulation by sub-dural grid electrodes (IODCS-SDG) at our institution between January-2016 and June-2017 were reviewed. Stimulation was delivered through the subdural-grid electrodes using a train-of-five pulses and muscle responses were recorded by motor-evoked-potentials (MEPs). Intra-operative direct cortical stimulation delivered through a ball-tipped probe (IODCS-probe) and extra-operative motor-mapping (EODCS-SDG) were also performed. Results IODCS-SDG was completed in 20 patients and subsequent EODCS-SDG was done in 17/20 patients. MEP responses were more commonly obtained in the deltoid (19/20), extensor-digitorum-communis (20/20) and first-dorsal-interosseus (19/20). The median thresholds varied between 40 V and 60 V for the six muscle groups. The respective IODCS-probe thresholds tended to be similar. No stimulation-provoked seizures or anaesthesia-related complications were noted during IODCS-SDG. EODCS-SDG could not be completed in 4/17 children and mapping data obtained was frequently inadequate. Nine patients demonstrated 100% concordance between IODCS-SDG and EODCS-SDG for the common mapped body regions. Stimulation-provoked seizures during EODCS-SDG were seen in 6/17 (35.3%) and after-discharges in 7/17 (41.2%) children. Conclusions IODCS-SDG could be performed safely in children with drug refractory epilepsy undergoing invasive EEG monitoring. Significance IODCS-SDG may be a useful adjunct to EODCS-SDG in motor mapping for children.

Children with neurodevelopmental disorders (NDD) are at high risk for sleep problems, especially insomnia. It is currently not known whether behavioural sleep interventions developed for typically developing (TD) children are effective for children with NDD, and if interventions need to be modified for each diagnostic group. The aim of this systematic review was to identify and evaluate commonalities, trends in outcomes, and the methodological quality of parent-delivered behavioural sleep interventions for children with NDD, specifically Attention-Deficit/Hyperactivity Disorder (ADHD), Autism Spectrum Disorder (ASD), Cerebral Palsy, and Fetal Alcohol Spectrum Disorder. Nine databases were searched. A total of 40 studies met eligibility criteria. The majority of studies were conducted with ASD and ADHD populations. Common sleep problems were evident across the NDD populations. The most frequently reported included bedtime resistance, night-waking, early morning awakening, and co-sleeping. The most common interventions used were implementation of healthy sleep practices, reinforcement, graduated extinction, and faded bedtime. All studies reported at least one behavioural treatment component as effective. Commonalities across NDD populations, as well as the TD population, for both sleep problems reported and behavioural interventions implemented, suggest the feasibility of developing a transdiagnostic behavioural sleep intervention suitable for children with a range of NDD.

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.

OBJECTIVEIntracranial electroencephalography (iEEG) monitoring is an important method of identifying the seizure focus in patients with medically refractory epilepsy. While previous studies have demonstrated low rates of surgical complications, reported rates of surgical site infection (SSI) are highly variable. To date, no studies have specifically evaluated the patient or operative risk factors contributing to SSI. The goals of this study were to examine the rate of SSI after iEEG monitoring for epilepsy workup in pediatric patients and to determine the variables that might contribute to the development of SSI.METHODSA retrospective analysis of hospital charts at the Hospital for Sick Children was performed for all patients who had undergone iEEG monitoring between 2000 and 2016. Univariate and multivariate analyses were performed to look for statistically significant variables in relation to SSI.RESULTSAmong 199 patients eligible for analysis, 8 (4.0%) developed SSIs within a period ranging from 21 to 51 days postoperatively. Univariate analysis yielded 4 factors related to SSI: number of people present in the operating room on electrode insertion (p = 0.02), length of insertion surgery (p = 0.04), previous operation at the same surgical site (p = 0.04), and number of depth electrodes inserted (p = 0.01). Multivariate analysis revealed that both the number of people present during the implant operation (OR 0.08, 95% CI 0.01–0.70) and the number of depth electrodes inserted (OR 3.52, 95% CI 1.44–8.59) independently contributed to SSI.CONCLUSIONSThis is the largest case series and the first comprehensive review of both patient and operative risk factors in the development of SSI from iEEG monitoring in a pediatric population. The authors’ institution had a lower rate of infection than those in most other studies, which could be explained by their protocol of administering intravenous antibiotics perioperatively and post–implant removal antibiotics for 14 days. The authors found a correlation between SSI and the number of people present during the implant operation, as well as the number of depth electrodes; both may contribute to breaks in sterility.

It has been extensively communicated that Health Canada’s Community Noise and Health Study (CNHS) did not find positive associations between wind turbine noise (WTN) levels and any of the evaluated health outcomes, beyond an increase in the prevalence of high annoyance toward several wind turbine features. The authors emphasize that this general conclusion remains bound by the study strengths and limitations. Following the publication of the CNHS findings, there has been interest among some individuals to present alternative interpretations of the results originally reported by Michaud et al. (J Acoust Soc Am 139(3):1443–1454, 2016. https://doi.org/10.1121/1.4942391 ). While recognizing the importance of independent scientific re-evaluation and/or reinterpretation, this commentary serves to clarify and, where necessary, correct some of the information put forward by others. One factor that has been re-evaluated by external stakeholders is the subsample of participants that comprise the lowest WTN category. In their reanalysis, they have eliminated this category, or introduced alternative comparative data. This paper identifies substantial issues associated with the re-evaluation put forth. To thoroughly address these issues and to avoid further confusion or misinterpretation, the authors of the CNHS provide a comparison between the CNHS health condition prevalence data and nationally representative health-based surveys conducted in Canada during the same calendar year. In addition, this paper responds to comments received to date on the CNHS, including the study’s age range, the generalization of findings, the provision of raw data, and conclusions on the association between WTN level and health.

Hypnic jerks represent a benign physiological phenomenon of sleep, which must be differentiated from other sleep-related movement disorders and epilepsy. A comprehensive review of hypnic jerks is presented with emphasis on recognition, physiology, evaluation, and treatment. The recent edition of the International Classification of Sleep Disorders (ICSD-3) has classified hypnic jerks as a sleep-related movement disorder under the subheading isolated symptoms and normal variants. Although generally benign, there have been increasing reports of excessive hypnic jerks in neurological disorders such as Parkinsonism, migraine, and brainstem lesions and children with neurologic disorders. Recent research identifying changes in the polysomnogram has led to a better understanding of the physiology of hypnic jerks. A variety of different motor patterns have been described; however, the origin and physiology of hypnic jerks remain enigmatic. Hypnic jerks represent a fascinating phenomenon of sleep; however, additional studies are needed to clarify their physiology and origin.

Occipital lobe epilepsies can present with complex or elementary visual hallucinations and oculomotor symptoms like contraversive tonic or clonic deviation of eyes and head. Brief occipital seizures can be difficult to diagnose. We report a child with occipital plus epilepsy who was investigated with stereo-EEG.

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.

Introduction: Sleep-related breathing disorders (SRBDs) in childhood are associated with adverse outcomes including developmental and behavioral disorders. The association of SRBDs with pediatric stroke is poorly understood. We aimed to assess the risk of SRBDs in children with stroke or non-stroke high risk arteriopathies (NSA) and to evaluate their association with cognitive and behavioral function. Hypothesis: Children with stroke or NSA have a higher rate of SRBD and associated poor cognitive and behavioral function. Methods: We conducted a cross-sectional study of children with arterial ischemic stroke (AIS), cerebral sinovenous thrombosis (CSVT) and NSA such as moyamoya. All children completed the Pediatric Sleep Questionnaire (PSQ). High risk for SRBDs was defined as a PSQ score ≥0.33. Children completed the Adaptive Behavior Assessment System (ABAS-2) parent and teacher forms and/or The Wechsler Intelligence Scale for Children (WISC-V) within 1 year of completing the PSQ. Results: Overall, 213 were included (54% males, median age: 9 years [IQR 5-14]). Forty-nine children (23%) were at high risk of SRBDs. The risk of SRBDs did not vary with age, gender or stroke subtype: AIS (25%), CSVT (20%), other stroke subtypes (20%) and NSA (24%) (p= 0.8). Children with SRBD had higher mean BMI z-scores (0.9±1.5 vs 0.4±1.3, p=0.06). Twenty-four and 15 children underwent ABAS-2 parent and teacher assessment, respectively. The global adaptive composite scores of both forms significantly correlated with the total PSQ scores (r s =-0.6, ps =-0.63, p=0.01 for the teacher form). Correlation coefficients remained statistically significant upon controlling for BMI. In 19 children who completed the WISC-V, those at risk of SRBDs (n=7) had significantly lower Full Scale IQ (70±24.7 vs 91±17, p=0.03), processing speed (55±44 vs 96±19, p=0.02) and working memory (77±23 vs 98±15, p=0.02) compared to children at no risk. Cognitive and behavioral function did not differ across stroke subtypes. Conclusions: Pediatric stroke or NSA is commonly associated with SRBDs which in turn is linked to significant decline in cognitive and behavioral function. Further studies are required to understand the underlying pathological mechanisms.

To assess the prevalence of obstructive sleep apnea syndrome (OSAS) in children with arterial ischemic stroke (AIS) and to evaluate its association with neuropsychological outcomes.We conducted a cross-sectional study of sleep health and neuropsychological outcome in children with AIS. A consecutive cohort of children attending a stroke clinic were assessed using a standardized pediatric sleep questionnaire (PSQ) and standardized measures of pediatric stroke outcome and intellectual, executive and adaptive function. High risk for OSAS was defined as PSQ score ≥0.33.Overall, 102 children were included (55% males, median age: 9 years [interquartile-range [IQR]: 6-14]). The prevalence of OSAS in children with AIS was significantly higher compared to published normative prevalence rate (25.5% vs 5%, p 0.001). Children with OSAS were more likely to have infarcts affecting both the anterior and posterior circulation (37.5% vs 9.5%, p = 0.021). In addition, children with OSAS had significantly higher median Pediatric Stroke Outcome Measure (PSOM) scores (2 [IQR: 0-2] vs 1 [IQR: 1-3.5], p = 0.01) and were more likely to be prescribed concomitant medications affecting sleep architecture (50% vs 22.4%, p = 0.007). OSAS was associated with significantly lower scores on intellectual, memory, cognitive, behavioral, attention, executive and adaptive function scales. The association between PSQ and intellectual ability and working memory remained statistically significant upon controlling for potential confounding factors including stroke related characteristics (neurologic impairment and arterial territory).The prevalence of OSAS in children with AIS compared to healthy controls is significantly elevated and is associated with poor neuropsychological outcomes. We highlight the importance of regular screening for OSAS - a modifiable risk factor - in children with AIS. The specific risk factors for OSAS and the potential benefits of therapeutic interventions in this patient population warrant further investigation.

Sleep hygiene behaviors (or more appropriately termed “healthy sleep practices”) are commonly cited in health professionals’ recommendations for sleep difficulties among children, yet research evidence for such practices are lacking. This chapter defines and outlines healthy sleep practices, as well as differentiates between healthy sleep practices and specific behavioral sleep interventions in the treatment of sleep difficulties, specifically behavioral insomnia among children. The ABCs of SLEEPING mnemonic, a framework for many commonly made recommendations around healthy sleep practices, will be introduced and used to organize and highlight current evidence in each area among populations of typically developing children and children with attention deficit hyperactivity disorder (ADHD), focusing on children ages 1–12 years of age. This chapter also includes a review of the literature examining the evidence of healthy sleep practices specifically among children with ADHD as compared to typically developing peers. The barriers to assessing, treating, and accessing evidence-based interventions targeting healthy sleep practices for children with behavioral insomnia will be discussed. Finally, a new tool, the ABCs of SLEEPING, will be described. This tool holds promise for bridging the gap in provision of evidence-based recommendations for healthy sleep practices interventions. After completing this chapter, the reader will be able to describe many healthy sleep practices recommendations commonly made by health professionals, as well as understand the evidence behind such recommendations for children with ADHD, and the treatment options currently available.

Leaders from the Canadian Society for Exercise Physiology convened representatives of national organizations, content experts, methodologists, stakeholders, and end-users who followed rigorous and transparent guideline development procedures to create the Canadian 24-Hour Movement Guidelines for Children and Youth: An Integration of Physical Activity, Sedentary Behaviour, and Sleep. These novel guidelines for children and youth aged 5-17 years respect the natural and intuitive integration of movement behaviours across the whole day (24-h period). The development process was guided by the Appraisal of Guidelines for Research Evaluation (AGREE) II instrument and systematic reviews of evidence informing the guidelines were assessed using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach. Four systematic reviews (physical activity, sedentary behaviour, sleep, integrated behaviours) examining the relationships between and among movement behaviours and several health indicators were completed and interpreted by expert consensus. Complementary compositional analyses were performed using Canadian Health Measures Survey data to examine the relationships between movement behaviours and health indicators. A stakeholder survey was employed (n = 590) and 28 focus groups/stakeholder interviews (n = 104) were completed to gather feedback on draft guidelines. Following an introductory preamble, the guidelines provide evidence-informed recommendations for a healthy day (24 h), comprising a combination of sleep, sedentary behaviours, light-, moderate-, and vigorous-intensity physical activity. Proactive dissemination, promotion, implementation, and evaluation plans have been prepared in an effort to optimize uptake and activation of the new guidelines. Future research should consider the integrated relationships among movement behaviours, and similar integrated guidelines for other age groups should be developed. Refereed/Peer-reviewed

Objective Better Nights/Better Days, a distance intervention for insomnia in school-aged children (with and without attention-deficit/hyperactivity disorder [ADHD]), was evaluated to determine its effectiveness on children's sleep and psychosocial functioning. Methods A single center, parallel group design randomized controlled trial (stratified on ADHD diagnosis) was conducted. Parents were randomized to intervention (n = 31) or waitlist control (n = 30), and completed questionnaires administered over the phone at baseline, postintervention (2 months), and follow-up (6 months). Actigraphy was also collected. The intervention consisted of a five-session manual and weekly telephone coach support. Results The intervention group demonstrated a significant reduction in sleep problems and improved psychosocial functioning at postintervention and follow-up. Actigraphy results indicated improved sleep onset, but not sleep duration. Children with and without ADHD responded in a similar manner to this intervention. Parents provided high satisfaction ratings. Conclusions This intervention holds promise as an accessible, sustainable, and effective program to address insomnia in school-aged children.

OBJECT Hemispherectomy for unilateral, medically refractory epilepsy is associated with excellent long-term seizure control. However, for patients with recurrent seizures following disconnection, workup and investigation can be challenging, and surgical options may be limited. Few studies have examined the role of repeat hemispherotomy in these patients. The authors hypothesized that residual fiber connections between the hemispheres could be the underlying cause of recurrent epilepsy in these patients. Diffusion tensor imaging (DTI) was used to test this hypothesis, and to target residual connections at reoperation using neuronavigation. METHODS The authors identified 8 patients with recurrent seizures following hemispherectomy who underwent surgery between 1995 and 2012. Prolonged video electroencephalography recordings documented persistent seizures arising from the affected hemisphere. In all patients, DTI demonstrated residual white matter association fibers connecting the hemispheres. A repeat craniotomy and neuronavigation-guided targeted disconnection of these residual fibers was performed. Engel class was used to determine outcome after surgery at a minimum of 2 years of follow-up. RESULTS Two patients underwent initial hemidecortication and 6 had periinsular hemispherotomy as their first procedures at a median age of 9.7 months. Initial pathologies included hemimegalencephaly (n = 4), multilobar cortical dysplasia (n = 3), and Rasmussen's encephalitis (n = 1). The mean duration of seizure freedom for the group after the initial procedure was 32.5 months (range 6–77 months). In all patients, DTI showed limited but definite residual connections between the 2 hemispheres, primarily across the rostrum/genu of the corpus callosum. The median age at reoperation was 6.8 years (range 1.3–14 years). The average time taken for reoperation was 3 hours (range 1.8–4.3 hours), with a mean blood loss of 150 ml (range 50–250 ml). One patient required a blood transfusion. Five patients are seizure free, and the remaining 3 patients are Engel Class II, with a minimum follow-up of 24 months for the group. CONCLUSIONS Repeat hemispherotomy is an option for consideration in patients with recurrent intractable epilepsy following failed surgery for catastrophic epilepsy. In conjunction with other modalities to establish seizure onset zones, advanced MRI and DTI sequences may be of value in identifying patients with residual connectivity between the affected and unaffected hemispheres. Targeted disconnection of these residual areas of connectivity using neuronavigation may result in improved seizure outcomes, with minimal and acceptable morbidity.

Background:Little knowledge exists on the availability of academic and community paediatric neurology positions. This knowledge is crucial for making workforce decisions. Our study aimed to: 1) obtain information regarding the availability of positions for paediatric neurologists in academic centres; 2) survey paediatric neurology trainees regarding their perceptions of employment issues and career plans; 3) survey practicing community paediatric neurologists 4) convene a group of paediatric neurologists to develop consensus regarding how to address these workforce issues.Methods:Surveys addressing workforce issues regarding paediatric neurology in Canada were sent to: 1) all paediatric neurology program directors in Canada (n=9) who then solicited information from division heads and from paediatric neurologists in surrounding areas; 2) paediatric neurology trainees in Canada (n=57) and; 3) community paediatric neurologists (n=27). A meeting was held with relevant stakeholders to develop a consensus on how to approach employment issues.Results:The response rate was 100% from program directors, 57.9% from residents and 44% from community paediatric neurologists. We found that the number of projected positions in academic paediatric neurology is fewer than the number of paediatric neurologists that are being trained over the next five to ten years, despite a clinical need for paediatric neurologists. Paediatric neurology residents are concerned about job availability and desire more career counselling.Conclusions:There is a current and projected clinical demand for paediatric neurologists despite a lack of academic positions. Training programs should focus on community neurology as a viable career option.

Study objectives To investigate the association between self-reported and objective measures of sleep and wind turbine noise (WTN) exposure. Methods The Community Noise and Health Study, a cross-sectional epidemiological study, included an in-house computer-assisted interview and sleep pattern monitoring over a 7 d period. Outdoor WTN levels were calculated following international standards for conditions that typically approximate the highest long-term average levels at each dwelling. Study data were collected between May and September 2013 from adults, aged 18-79 y (606 males, 632 females) randomly selected from each household and living between 0.25 and 11.22 kilometers from operational wind turbines in two Canadian provinces. Self-reported sleep quality over the past 30 d was assessed using the Pittsburgh Sleep Quality Index. Additional questions assessed the prevalence of diagnosed sleep disorders and the magnitude of sleep disturbance over the previous year. Objective measures for sleep latency, sleep efficiency, total sleep time, rate of awakening bouts, and wake duration after sleep onset were recorded using the wrist worn Actiwatch2® from a subsample of 654 participants (289 males, 365 females) for a total of 3,772 sleep nights. Results Participant response rate for the interview was 78.9%. Outdoor WTN levels reached 46 dB(A) with an arithmetic mean of 35.6 and a standard deviation of 7.4. Self-reported and objectively measured sleep outcomes consistently revealed no apparent pattern or statistically significant relationship to WTN levels. However, sleep was significantly influenced by other factors, including, but not limited to, the use of sleep medication, other health conditions (including sleep disorders), caffeine consumption, and annoyance with blinking lights on wind turbines. Conclusions Study results do not support an association between exposure to outdoor WTN up to 46 dB(A) and an increase in the prevalence of disturbed sleep. Conclusions are based on WTN levels averaged over 1 y and, in some cases, may be strengthened with an analysis that examines sleep quality in relation to WTN levels calculated during the precise sleep period time.

Sleep and epilepsy are closely related as they share the same neurobiological cellular mechanisms and the same anatomical circuits. There is a bidirectional relationship between sleep and epilepsy. Seizures and interictal epileptiform discharges can adversely affect sleep organization, and, conversely, sleep deprivation and sleep disorders directly influence the clinical course for children with epilepsy. Epilepsy is common in children, with an estimated lifetime prevalence of 1%. It is essential for clinicians caring for children with NDD to be aware of the interrelationship of sleep and epilepsy. By appropriate recognition, evaluation, and treatment of both sleep disorders and epilepsy in children with NDD, it is possible to limit the impact of these comorbidities on daytime functioning for both the child and the family.

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).

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.

Study ObjectivesTo evaluate the association between depressive symptoms, sleep patterns (duration and quality), excessive daytime sleepiness (EDS), and physical activity (PA) in adolescents with narcolepsy.MethodsThis 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.ResultsThirty 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).ConclusionsAdolescents 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.

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.

To investigate the association between self-reported and objective measures of sleep and wind turbine noise (WTN) exposure.The Community Noise and Health Study, a cross-sectional epidemiological study, included an in-house computer-assisted interview and sleep pattern monitoring over a 7 d period. Outdoor WTN levels were calculated following international standards for conditions that typically approximate the highest long-term average levels at each dwelling. Study data were collected between May and September 2013 from adults, aged 18-79 y (606 males, 632 females) randomly selected from each household and living between 0.25 and 11.22 kilometers from operational wind turbines in two Canadian provinces. Self-reported sleep quality over the past 30 d was assessed using the Pittsburgh Sleep Quality Index. Additional questions assessed the prevalence of diagnosed sleep disorders and the magnitude of sleep disturbance over the previous year. Objective measures for sleep latency, sleep efficiency, total sleep time, rate of awakening bouts, and wake duration after sleep onset were recorded using the wrist worn Actiwatch2® from a subsample of 654 participants (289 males, 365 females) for a total of 3,772 sleep nights.Participant response rate for the interview was 78.9%. Outdoor WTN levels reached 46 dB(A) with an arithmetic mean of 35.6 and a standard deviation of 7.4. Self-reported and objectively measured sleep outcomes consistently revealed no apparent pattern or statistically significant relationship to WTN levels. However, sleep was significantly influenced by other factors, including, but not limited to, the use of sleep medication, other health conditions (including sleep disorders), caffeine consumption, and annoyance with blinking lights on wind turbines.Study results do not support an association between exposure to outdoor WTN up to 46 dB(A) and an increase in the prevalence of disturbed sleep. Conclusions are based on WTN levels averaged over 1 y and, in some cases, may be strengthened with an analysis that examines sleep quality in relation to WTN levels calculated during the precise sleep period time.

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.

Background: Up to 25% of 1- to 10-year-old children experience insomnia (ie, resisting bedtime, trouble falling asleep, night awakenings, and waking too early in the morning). Insomnia can be associated with excessive daytime sleepiness and negative effects on daytime functioning across multiple domains (eg, behavior, mood, attention, and learning). Despite robust evidence supporting the effectiveness of behavioral treatments for insomnia in children, very few children with insomnia receive these treatments, primarily due to a shortage of available treatment resources. Objective: The Better Nights, Better Days (BNBD) internet-based program provides a readily accessible electronic health (eHealth) intervention to support parents in providing evidence-based care for insomnia in typically developing children. The purpose of the randomized controlled trial (RCT) is to evaluate the effectiveness of BNBD in treating insomnia in children aged between 1 and 10 years. Methods: BNBD is a fully automated program, developed based on evidence-based interventions previously tested by the investigators, as well as on the extant literature on this topic. We describe the 2-arm RCT in which participants (500 primary caregivers of children with insomnia residing in Canada) are assigned to intervention or usual care. Results: The effects of this behavioral sleep eHealth intervention will be assessed at 4 and 8 months postrandomization. Assessment includes both sleep (actigraphy, sleep diary) and daytime functioning of the children and daytime functioning of their parents. Results will be reported using the standards set out in the Consolidated Standards of Reporting Trials statement. Conclusions: If the intervention is supported by the results of the RCT, we plan to commercialize this program so that it is sustainable and available at a low cost to all families with internet access. Trial Registration: ClinicalTrials.gov NCT02243501; https://clinicaltrials.gov/show/NCT02243501 (Archived by WebCite at http://www.webcitation.org/6x8Z5pBui) [JMIR Res Protoc 2018;7(3):e76]

Sleep disturbance is common in children with autism, resulting in a great need for effective treatments. To evaluate treatments for sleep disturbance in this population, it is critical to understand the relationship between measures of sleep captured by parent report and objective measures. The Children's Sleep Habits Questionnaire (CSHQ) and actigraphy-measured data from 80 children with autism and sleep-onset delay were evaluated. Reported problems with sleep-onset delay were concurrent with sleep duration problems in 66% of children, night wakings in 72% of children, and bedtime resistance in 66% of children; 38% of children were reported to have problems with all CSHQ insomnia domains. Actigraphy-measured sleep duration was correlated with estimates using CSHQ-reported bed and wake times.

SummaryObjective Epilepsy surgery can be successful in children with extensive congenital or early acquired focal or hemispheric brain lesion on magnetic resonance imaging (MRI) despite generalized interictal epileptiform discharges (IEDs). The aim of this study was to assess if rapid eye movement (REM) sleep reduced generalized IEDs and revealed lateralized IEDs to identify the epileptogenic hemisphere in children with generalized IEDs and normal/subtle changes on MRI. Methods We studied 20 children with generalized IEDs on scalp electroencephalography (EEG) and normal/subtle changes on MRI who underwent intracranial video-EEG for epilepsy surgery. We assessed a minimum of 100 IEDs during REM, non-REM, and wakefulness, and assigned the distribution (generalized, left, or right hemisphere) to each IED. The number of lobes in the resected areas and seizure outcome were compared between 20 children with generalized IEDs and a comparison group of 28 children without generalized IEDs. Results The mean occurrence rate of generalized IEDs during REM (37%) was significantly lower than that during non-REM (67%, p

SummaryObjective Epilepsy is a common neurologic disorder of childhood. To determine the genetic diagnostic yield in epileptic encephalopathy, we performed a retrospective cohort study in a single epilepsy genetics clinic. Methods We included all patients with intractable epilepsy, global developmental delay, and cognitive dysfunction seen between January 2012 and June 2014 in the Epilepsy Genetics Clinic. Electronic patient charts were reviewed for clinical features, neuroimaging, biochemical investigations, and molecular genetic investigations including targeted next-generation sequencing of epileptic encephalopathy genes. Results Genetic causes were identified in 28% of the 110 patients: 7% had inherited metabolic disorders including pyridoxine dependent epilepsy caused by ALDH7A1 mutation, Menkes disease, pyridox(am)ine-5-phosphate oxidase deficiency, cobalamin G deficiency, methylenetetrahydrofolate reductase deficiency, glucose transporter 1 deficiency, glycine encephalopathy, and pyruvate dehydrogenase complex deficiency; 21% had other genetic causes including genetic syndromes, pathogenic copy number variants on array comparative genomic hybridization, and epileptic encephalopathy related to mutations in the SCN1A, SCN2A, SCN8A, KCNQ2, STXBP1, PCDH19, and SLC9A6 genes. Forty-five percent of patients obtained a genetic diagnosis by targeted next-generation sequencing epileptic encephalopathy panels. It is notable that 4.5% of patients had a treatable inherited metabolic disease. Significance To the best of our knowledge, this is the first study to combine inherited metabolic disorders and other genetic causes of epileptic encephalopathy. Targeted next-generation sequencing panels increased the genetic diagnostic yield from 25% in patients with epileptic encephalopathy.

Key Clinical Message Patients presenting with infantile spasms, dysmorphic features, and periventricular nodular heterotopia may benefit from genetic copy number variation microarray, or whole‐exome sequencing to identify candidate genes. This will allow personalized diagnosis and prognostication and the eventual understanding of single and combined gene functions in brain health and disease.

Insomnia, which is related to daytime deficits and is a common problem for children with neurodevelopmental disorders (NDDs), is often successfully treated with behavioral strategies. However, there are barriers to accessing these treatments, and there has been little research examining what these interventions need to be usable and effective. The goal of this study was to gain consensus from experts in the field on the key components of an eHealth, parent-implemented, intervention program aimed at improving sleep in children with attention-deficit/hyperactivity disorder, autism spectrum disorder, cerebral palsy, and fetal alcohol spectrum disorder. This was achieved using the Delphi method, which involves asking participants to respond to open-ended questions about a topic of interest and then, in iterative rounds, to rate the recommendations that were made by the group. In the current study, participants (27 responders in the first round, 21 in the second, and 18 in the third) rated a total of 131 recommendations. Of those 131 recommendations, 52 items had high importance and high consensus and were deemed to be priority items to consider for creating an eHealth, parent-delivered, behaviorally-based intervention for insomnia in children with NDD. Furthermore, 75% (n = 84) of the 112 recommendations from the first round were believed to be applicable across all 4 NDD groups, thus providing evidence of the potential for a transdiagnostic intervention.

Sleep disorders occur in up to 25% of children and are more prevalent in children who have attention problems and attachment issues. Research shows that foster children display sleep problems, but limited knowledge exists on sleep problems in adopted children. This pilot study aimed to identify the types of sleep disorders in adopted children and associated psychosocial factors. Parents of adopted children in Ontario, Canada, ages 2-10 years were asked to complete questionnaires evaluating demographic measures, sleep history, and the presence of behavioral problems. Insomnias and parasomnias were reported in adopted children and were associated with attention problems. This pilot study emphasizes the need for further research on the underlying factors governing the relationship between poor sleep and behavioral problems in adopted children.

The indications for operating on lesions in or near areas of cortical eloquence balance the benefit of resection with the risk of permanent neurological deficit. In adults, awake craniotomy has become a versatile tool in tumor, epilepsy and functional neurosurgery, permitting intra-operative stimulation mapping particularly for language, sensory and motor cortical pathways. This allows for maximal tumor resection with considerable reduction in the risk of post-operative speech and motor deficits. We report our experience of awake craniotomy and cortical stimulation for epilepsy and supratentorial tumors located in and around eloquent areas in a pediatric population (n=10, five females). The presenting symptom was mainly seizures and all children had normal neurological examinations. Neuroimaging showed lesions in the left opercular (n=4) and precentral or peri-sylvian regions (n=6). Three right-sided and seven left-sided awake craniotomies were performed. Two patients had a history of prior craniotomy. All patients had intra-operative mapping for either speech or motor or both using cortical stimulation. The surgical goal for tumor patients was gross total resection, while for all epilepsy procedures, focal cortical resections were completed without any difficulty. None of the patients had permanent post-operative neurologic deficits. The patient with an epileptic focus over the speech area in the left frontal lobe had a mild word finding difficulty post-operatively but this improved progressively. Follow-up ranged from 6 to 27 months. Pediatric awake craniotomy with intra-operative mapping is a precise, safe and reliable method allowing for resection of lesions in eloquent areas. Further validations on larger number of patients will be needed to verify the utility of this technique in the pediatric population.