Performance and symptom validity indicators among children undergoing cognitive surveillance following treatment for craniopharyngioma.

Background: Performance validity tests (PVTs) and symptom validity tests (SVTs) are essential to neuropsychological evaluations, helping ensure findings reflect true abilities or concerns. It is unclear how PVTs and SVTs perform in children who received radiotherapy for brain tumors. Accordingly, we investigated the rate of noncredible performance on validity indicators as well as associations with fatigue and lower intellectual functioning. Methods: Embedded PVTs and SVTs were investigated in 98 patients with pediatric craniopharyngioma undergoing proton radiotherapy (PRT). The contribution of fatigue, sleepiness, and lower intellectual functioning to embedded PVT performance was examined. Further, we investigated PVTs and SVTs in relation to cognitive performance at pre-PRT baseline and change over time. Results: SVTs on parent measures were not an area of concern. PVTs identified 0-31% of the cohort as demonstrating possible noncredible performance at baseline, with stable findings 1 year following PRT. Reliable digit span (RDS) noted the highest PVT failure rate; RDS has been criticized for false positives in pediatric populations, especially children with neurological impairment. Objective sleepiness was strongly associated with PVT failure, stressing need to consider arousal level when interpreting cognitive performance in children with craniopharyngioma. Lower intellectual functioning also needs to be considered when interpreting task engagement indices as it was strongly associated with PVT failure. Conclusions: Embedded PVTs should be used with caution in pediatric craniopharyngioma patients who have received PRT. Future research should investigate different cut-off scores and validity indicator combinations to best differentiate noncredible performance due to task engagement versus variable arousal and/or lower intellectual functioning.

Health-related quality of life, obesity, fragmented sleep, fatigue, and psychosocial problems among youth with craniopharyngioma.

OBJECTIVE: Youth with craniopharyngioma experience weight gain, fragmented sleep, excessive daytime sleepiness (EDS), fatigue, and psychosocial problems that negatively impact their overall health-related quality of life (HRQoL). Greater hypothalamic tumor involvement (HI) may be associated with higher rates or severity of these impairments; however, the direct and indirect impact of HI on the physical and psychosocial consequences associated with pediatric craniopharyngioma remain unclear. The purpose of the current study was to examine relations between HI, body mass index (BMI), fragmented sleep, EDS, fatigue, psychosocial problems, and HRQoL among youth with craniopharyngioma. METHODS: Eighty-four youth with craniopharyngioma (Mage  = 10.27 ± 4.3 years, 53.6% female, 64.3% White) were assessed with actigraphy, nocturnal polysomnography, and multiple sleep latency tests prior to proton therapy, when indicated. Caregivers completed measures of fatigue, psychosocial functioning, and HRQoL. RESULTS: Hypothalamic tumor involvement was associated with greater BMI (Est. = 2.97, p = 0.003) and daytime sleepiness (Est. = 2.53, p = 0.01). Greater fatigue predicted more psychosocial problems (Est. = 0.29, p < 0.001) and lower HRQoL (Est. = 0.23, p = 0.001). Psychosocial problems also predicted lower HRQoL (Est. = -0.34, p = 0.004). Fragmented sleep (Est. = 0.03, p = 0.04) and fatigue (Est. = 0.10, p = 0.02) indirectly predicted lower HRQoL through psychosocial problems. CONCLUSIONS: Youth with craniopharyngioma with greater HI may benefit from weight reduction interventions and management of excessive sleepiness. Patients should be prospectively monitored for sleep problems, fatigue, and psychosocial problems, as these patients may benefit from interventions targeting fatigue and psychosocial health to improve HRQoL.

Sleep and daytime behavior in individuals with Christianson Syndrome.

BACKGROUND: The objectives of this study were to: 1) characterize the sleep behaviors and symptoms of individuals with Christianson Syndrome (CS) by means of validated questionnaires; and 2) determine their associations with daytime emotional and behavioral symptoms in this population. METHODS: Participants included 16 boys genetically diagnosed with CS, between 2.5 and 40 years of age (M = 14.5 ± 8.08). Parents completed questionnaires regarding the sleep, daytime behavior, and health of their child. RESULTS: Of the participants, 31% did not obtain the recommended amount of sleep for their age, 43% experienced a prolonged sleep latency, and 88% had a clinical or sub-clinical score for at least one subscale of the Sleep Disturbance Scale for Children (SDSC). Specific problems detected included insomnia, sleep-wake transition disorders, periodic limb movements in sleep, and sleep related breathing disorders. About half of the participants manifested emotional and behavioral problems at clinical levels. Higher levels of sleep disturbances were associated with higher levels of behavioral and emotional daytime symptoms. CONCLUSIONS: Sleep problems are common in individuals with CS and are associated with daytime behavioral and emotional symptoms.

Impact of sleep, neuroendocrine, and executive function on health-related quality of life in young people with craniopharyngioma.

AIM: To examine the impact of clinical factors, cognitive deficits, and sleepiness on health-related quality of life (HRQoL) among young people with craniopharyngioma. METHOD: Seventy-eight patients (67% White; 41 males, 37 females; mean age 10y 8mo, SD 3y 11mo, range 6-20y) with craniopharyngioma were assessed for tumor extent and diabetes insipidus. All patients underwent overnight polysomnography and multiple sleep latency tests after surgical resection. Executive functioning was assessed using parent-reported measures. Patients and their parents completed measures of HRQoL. None had a history of previous radiation therapy. RESULTS: Path analysis was used to test hypothesized relations while controlling for demographic and disease characteristics. Analyses revealed poorer parent-reported HRQoL among young people with greater executive functioning symptoms (estimate -0.83; p<0.001). Direct and indirect effects were found among diabetes insipidus, executive functioning, and parent-reported HRQoL. Diabetes insipidus directly predicted greater global executive functioning impairment (estimate 5.15; p=0.04) and indirectly predicted lower HRQoL through executive functioning impairment (estimate -4.25; p=0.049). No significant effects were found between excessive daytime sleepiness, tumor hypothalamic involvement, diabetes insipidus, executive functioning, and patient-reported HRQoL. INTERPRETATION: These findings suggest that young people with craniopharyngioma presenting with diabetes insipidus may benefit from targeted neurocognitive and psychosocial screening to inform interventions. What this paper adds Children with craniopharyngioma and executive functioning impairment are more likely to have poorer health-related quality of life (HRQoL). Diabetes insipidus, a complication associated with surgery, predicted greater executive functioning impairment. Diabetes insipidus indirectly predicted lower parent-reported HRQoL through executive functioning impairment.

The impact of COVID-19 related school shutdown on sleep in adolescents: a natural experiment.

OBJECTIVE: During the outbreak of the COVID-19 pandemic in 2020, high schools closed or transitioned to remote teaching. The aim of this study was to describe how the COVID-19 related school shutdown impacted the sleep behaviors of typically developing adolescents. METHODS: A qualitative study was conducted between April 28 and June 3, 2020 with 45 adolescents using one-on-one semi-structured phone interviews. RESULTS: The "natural experiment" caused by the shutdown of schools due to the COVID-19 pandemic led to a 2-h shift in the sleep of typically developing adolescents, longer sleep duration, improved sleep quality, and less daytime sleepiness compared to those experienced under the regular school-time schedule. DISCUSSION: These findings are consistent with previous studies showing that delaying high school start times could be an effective way to extend sleep duration, improve sleep quality, reduce daytime sleepiness and lower adolescents' stress during the school year. As many countries look for ways to reduce the number of interactions between students in schools so that physical distancing is feasible, following teens' delayed sleep biology could offer an affordable solution. For example, staggering arrival times by delaying school start time for older adolescents compared with younger adolescents can reduce the total number of students attending school at the same time. This strategy offers a practical means to reduce school density and the number of interactions between students which are needed to reduce the potential transmission of COVID-19 in schools, while improving adolescents sleep health.

Predictors of narcolepsy and hypersomnia due to medical disorder in pediatric craniopharyngioma.

PURPOSE: Several studies describe sleep-wake disturbances in pediatric craniopharyngioma, but none have determined the prevalence or associated predictors of excessive sleepiness in this group after diagnosis and prior to post-operative observation or adjuvant radiotherapy. In this study, we report sleep-wake disturbances in children and adolescents with craniopharyngioma and associated clinical and treatment variables. METHODS: After surgery and prior to radiotherapy or observation, pediatric patients (n = 110) with craniopharyngioma ≥ 3 years old completed a baseline sleep clinic evaluation by a pediatric sleep specialist, polysomnography (PSG) and next-day multiple sleep latency test (MSLT). MSLT was limited to those ≥ 6 years old. Logistic regression models were used to determine the relationship between patient characteristics and the presence and type of hypersomnia. RESULTS: Amongst patients completing PSG and MSLT, 80% had polygraphic evidence of excessive daytime sleepiness. Hypersomnia due to medical condition was diagnosed in 45% and narcolepsy in 35%. Overweight or obese patients were more likely to be diagnosed with hypersomnia (P = 0.012) or narcolepsy (P = 0.009). Grade 2 hypothalamic involvement (HI) at diagnosis was associated with the diagnosis of narcolepsy (P = 0.0008). CONCLUSIONS: This study describes the prevalence and associated predictors of hypersomnia for patients with craniopharyngioma after surgical resection. HI was predictive of narcolepsy diagnosis, and a higher body mass index z-score was associated with hypersomnia due to medical condition and narcolepsy. We recommend that sleep assessment and intervention begin after surgical resection, especially in overweight or obese patients and those with extensive tumors.

Actigraphy versus Polysomnography to Measure Sleep in Youth Treated for Craniopharyngioma.

OBJECTIVE/BACKGROUND: Youth with craniopharyngioma are at increased risk for excessive daytime sleepiness and narcolepsy. Polysomnography (PSG) is the gold standard for diagnosing sleep disorders, but is time-intensive, costly, and does not offer an in vivo measure of typical sleep routine. We determined the sensitivity, specificity, and accuracy of actigraphy compared with PSG in measuring nocturnal sleep in pediatric craniopharyngioma. PARTICIPANTS: Fifty youth with craniopharyngioma (age 3-20 years) were assessed by overnight PSG and concurrent actigraphy after surgical resection and before proton therapy. METHODS: PSG and actigraphy data were synchronized utilizing an epoch-by-epoch comparison method. Sensitivity, specificity, and accuracy were calculated using measures of true wake, true sleep, false wake, and false sleep. Bland-Altman plots were conducted to further assess level of agreement. RESULTS: Actigraphy was 93% sensitive (true sleep [TS]) and 87% accurate (ability to detect TS and true wake) in measuring sleep versus wakefulness and was a reliable measure of sleep efficiency (SE) and sleep latency (SL). Specificity (true wake) was poor (55%) and total sleep time (TST) was underestimated by an average of 15.1 min. Wake after sleep onset (WASO) was overestimated by an average of 14.7 min. CONCLUSIONS: Actigraphy was highly sensitive and accurate and was a reliable measure of SE and SL. Although there were differences in TST and WASO measurements by actigraphy and PSG, our findings provide the basis for future studies on the use of actigraphy to monitor treatment response to wakefulness-promoting medications in youth with craniopharyngioma who demonstrate excessive daytime sleepiness.

Sensitivity and Specificity of the Modified Epworth Sleepiness Scale in Children With Craniopharyngioma.

STUDY OBJECTIVES: Children with craniopharyngioma are at risk for excessive daytime sleepiness (EDS). Multiple Sleep Latency Testing (MSLT) is the gold standard for objective evaluation of EDS; however, it is time and resource intensive. We compared the reliability, sensitivity, and specificity of the modified Epworth Sleepiness Scale (M-ESS) and MSLT in monitoring EDS in children with craniopharyngioma. METHODS: Seventy patients (ages 6 to 20 years) with craniopharyngioma completed the M-ESS and were evaluated by polysomnography and MSLT. Evaluations were made after surgery, if performed, and before proton therapy. RESULTS: MSLT revealed that 66 participants (81.8%) had EDS, as defined by a mean sleep latency (MSL) < 10 minutes, with only 28.8% reporting EDS on the M-ESS by using a cutoff score of 10. The M-ESS demonstrated adequate internal consistency and specificity (91.7%) but poor sensitivity (33.3%) with the established cutoff score of 10. A cutoff score of 6 improved the sensitivity to 64.8% but decreased the specificity to 66.7%. CONCLUSIONS: Patients with craniopharyngioma are at high risk for EDS, as documented objectively on the MSLT, but they frequently do not recognize or accurately report their sleepiness. Future sleep studies should investigate whether specific items or alternative self- and parent-reported measures of sleepiness may have greater clinical utility in monitoring sleepiness in this population.

Prevalence, risk factors, and response to treatment for hypersomnia of central origin in survivors of childhood brain tumors.

Daytime sleepiness is recognized in childhood brain tumor survivors. Our objective was to determine prevalence, risk factors for PSG/MLST proven hypersomnia/narcolepsy, and response to stimulants in childhood brain tumor survivors. Standard PSG/MSLT criteria were used to diagnose hypersomnia/narcolepsy. Medical records of brain tumor survivors having undergone a PSG/MSLT were reviewed for the diagnostic code of hypersomnia/narcolepsy. Survivors with hypersomnia/narcolepsy were matched with 2-3 survivors without reported hypersomnia/narcolepsy by age at tumor diagnosis, gender, and time from tumor diagnosis. Between January 2000 to April 2015, 39 of the 2336 brain tumor patients treated at our institution were diagnosed with hypersomnia/narcolepsy for a prevalence rate of 1670/100,000. Hypersomnia/narcolepsy was diagnosed at a median of 6.1 years (range 0.4-13.2) from tumor diagnosis and 4.7 years (range - 1.5 to 10.4) from cranial radiation. Midline tumor location (OR 4.6, CI 1.7-12.2, p = 0.002) and anti-epilepsy drug (AED) use (OR 11, CI 2.4-54) correlated with hypersomnia/narcolepsy while radiation dose > 30 Gray trended towards significance (OR 1.8, CI 0.9-3.6); posterior fossa tumor location reduced the risk (OR 0.1, CI 0.04-0.5, p = 0.002). AED use also correlated with midline tumor location. Thirty-seven survivors were treated with stimulants and reported improved wakefulness and school performance [response rate CI 0.97 (0.86-0.99) and 0.83 (0.65-0.94)]. Prevalence of hypersomnia/narcolepsy among childhood brain tumor survivors was higher than the general population. Tumor location and radiation dose were possible risk factors, and stimulants were reported to be beneficial.

Delaying Middle School and High School Start Times Promotes Student Health and Performance: An American Academy of Sleep Medicine Position Statement.

ABSTRACT: During adolescence, internal circadian rhythms and biological sleep drive change to result in later sleep and wake times. As a result of these changes, early middle school and high school start times curtail sleep, hamper a student's preparedness to learn, negatively impact physical and mental health, and impair driving safety. Furthermore, a growing body of evidence shows that delaying school start times positively impacts student achievement, health, and safety. Public awareness of the hazards of early school start times and the benefits of later start times are largely unappreciated. As a result, the American Academy of Sleep Medicine is calling on communities, school boards, and educational institutions to implement start times of 8:30 AM or later for middle schools and high schools to ensure that every student arrives at school healthy, awake, alert, and ready to learn.

Training, knowledge, attitudes and practices of Canadian health care providers regarding sleep and sleep disorders in children.

BACKGROUND: Sleep disorders are prevalent in children and are associated with significant comorbidity. OBJECTIVE: To assess the training, knowledge, attitudes and practices of Canadian health care providers (HCPs) regarding sleep disorders in children. METHOD: A 42-item survey, designed to collect information on frequency of paediatric sleep disorders-related screening and diagnosis, implementation of evidence-based interventions and related knowledge base, was completed by HCPs. RESULTS: Ninety-seven HCPs completed the survey. One per cent obtained training in paediatric sleep during undergraduate training and 3% obtained such training during their residencies, yet 34.9% estimated that 25 to 50% of their patients suffered from sleep disorders. Most HCPs thought that sleep disorders significantly impacted children's health and daytime function. Most HCPs screened for developmental sleep issues, but not consistently for sleep disorders. Most recommended evidence-based behavioural interventions for behavioural sleep disorders, but some also reported behavioural interventions that were not first-line or recommended. Inadequate knowledge regarding melatonin use was evident. Most participants reported rarely/never ordering a sleep study for a child with suspected obstructive sleep apnea (OSA). Most were familiar with surgical and weight loss management options for OSA; many were unfamiliar with benefits of continuous positive airway pressure. Participants' knowledge scores were highest on developmental and behavioural aspects of sleep, and lowest on sleep disorders. CONCLUSIONS: HCPs exhibit significant gaps in their knowledge, screening, evaluation and treatment practices for paediatric sleep disorders. Training at the undergraduate, graduate and postgraduate levels, as well as Continuing Medical Education are needed to optimize recognition, treatment and follow-up of paediatric sleep disorders.

Consensus Statement of the American Academy of Sleep Medicine on the Recommended Amount of Sleep for Healthy Children: Methodology and Discussion.

ABSTRACT: Members of the American Academy of Sleep Medicine developed consensus recommendations for the amount of sleep needed to promote optimal health in children and adolescents using a modified RAND Appropriateness Method. After review of 864 published articles, the following sleep durations are recommended: Infants 4 months to 12 months should sleep 12 to 16 hours per 24 hours (including naps) on a regular basis to promote optimal health. Children 1 to 2 years of age should sleep 11 to 14 hours per 24 hours (including naps) on a regular basis to promote optimal health. Children 3 to 5 years of age should sleep 10 to 13 hours per 24 hours (including naps) on a regular basis to promote optimal health. Children 6 to 12 years of age should sleep 9 to 12 hours per 24 hours on a regular basis to promote optimal health. Teenagers 13 to 18 years of age should sleep 8 to 10 hours per 24 hours on a regular basis to promote optimal health. Sleeping the number of recommended hours on a regular basis is associated with better health outcomes including: improved attention, behavior, learning, memory, emotional regulation, quality of life, and mental and physical health. Regularly sleeping fewer than the number of recommended hours is associated with attention, behavior, and learning problems. Insufficient sleep also increases the risk of accidents, injuries, hypertension, obesity, diabetes, and depression. Insufficient sleep in teenagers is associated with increased risk of self-harm, suicidal thoughts, and suicide attempts. COMMENTARY: A commentary on this article apears in this issue on page 1439.

Sleep Spindle Characteristics in Children with Neurodevelopmental Disorders and Their Relation to Cognition.

Empirical evidence indicates that sleep spindles facilitate neuroplasticity and "off-line" processing during sleep, which supports learning, memory consolidation, and intellectual performance. Children with neurodevelopmental disorders (NDDs) exhibit characteristics that may increase both the risk for and vulnerability to abnormal spindle generation. Despite the high prevalence of sleep problems and cognitive deficits in children with NDD, only a few studies have examined the putative association between spindle characteristics and cognitive function. This paper reviews the literature regarding sleep spindle characteristics in children with NDD and their relation to cognition in light of what is known in typically developing children and based on the available evidence regarding children with NDD. We integrate available data, identify gaps in understanding, and recommend future research directions. Collectively, studies are limited by small sample sizes, heterogeneous populations with multiple comorbidities, and nonstandardized methods for collecting and analyzing findings. These limitations notwithstanding, the evidence suggests that future studies should examine associations between sleep spindle characteristics and cognitive function in children with and without NDD, and preliminary findings raise the intriguing question of whether enhancement or manipulation of sleep spindles could improve sleep-dependent memory and other aspects of cognitive function in this population.

Recommended Amount of Sleep for Pediatric Populations: A Consensus Statement of the American Academy of Sleep Medicine.

ABSTRACT: Sleep is essential for optimal health in children and adolescents. Members of the American Academy of Sleep Medicine developed consensus recommendations for the amount of sleep needed to promote optimal health in children and adolescents using a modified RAND Appropriateness Method. The recommendations are summarized here. A manuscript detailing the conference proceedings and the evidence supporting these recommendations will be published in the Journal of Clinical Sleep Medicine.

Investigating the Role of Hypothalamic Tumor Involvement in Sleep and Cognitive Outcomes Among Children Treated for Craniopharyngioma.

OBJECTIVE: Despite excellent survival prognosis, children treated for craniopharyngioma experience significant morbidity. We examined the role of hypothalamic involvement (HI) in excessive daytime sleepiness (EDS) and attention regulation in children enrolled on a Phase II trial of limited surgery and proton therapy. METHODS: Participants completed a sleep evaluation (N = 62) and a continuous performance test (CPT) during functional magnetic resonance imaging (fMRI; n = 29) prior to proton therapy. RESULTS: EDS was identified in 76% of the patients and was significantly related to increased HI extent (p = .04). There was no relationship between CPT performance during fMRI and HI or EDS. Visual examination of group composite fMRI images revealed greater spatial extent of activation in frontal cortical regions in patients with EDS, consistent with a compensatory activation hypothesis. CONCLUSION: Routine screening for sleep problems during therapy is indicated for children with craniopharyngioma, to optimize the timing of interventions and reduce long-term morbidity.

The association between sleep spindles and IQ in healthy school-age children.

Recent studies have suggested that sleep is associated with IQ measures in children, but the underlying mechanism remains unknown. An association between sleep spindles and IQ has been found in adults, but only two previous studies have explored this topic in children. The goal of this study was to examine whether sleep spindle frequency, amplitude, duration and/or density were associated with performance on the perceptual reasoning, verbal comprehension, working memory, and processing speed subscales of the Wechsler Intelligence Scale for Children-IV (WISC-IV). We recruited 29 typically developing children 7-11 years of age. We used portable polysomnography to document sleep architecture in the natural home environment and evaluated IQ. We found that lower sleep spindle frequency was associated with better performance on the perceptual reasoning and working memory WISC-IV scales, but that sleep spindle amplitude, duration and density were not associated with performance on the IQ test.