The temporal and bi-directional relationship between physical activity and sleep in ambulatory children with cerebral palsy.

PURPOSE: Exploring the temporal and bi-directional relationship between device-based measures of physical activity and sleep in ambulatory children with cerebral palsy (CP). MATERIALS AND METHODS: 24-hour activity data were collected from children with CP (n = 51, 43% girls, mean age (range); 6.8 (3-12) years; Gross Motor Function Classification System levels I to III). Nocturnal sleep parameters and daily physical activity were measured for seven consecutive days and nights using ActiGraph GT3X accelerometers. Linear mixed models were constructed to explore the relationships between sleep and activity. RESULTS: Light and moderate-to-vigorous activity were negatively associated with sleep efficiency (SE) (resp. p = 0.04, p = 0.010) and total sleep time (TST) (resp. p = 0.007, p = 0.016) the following night. Sedentary time was positively associated with SE and TST the following night (resp. p = 0.014, p = 0.004). SE and TST were positively associated with sedentary time (resp. p = 0.011, p = 0.001) and negatively with moderate-to-vigorous physical activity (resp. p < 0.001, p = 0.002) the following day. Total bedtime and TST were negatively associated with light physical activity (resp. p = 0.046, p = 0.004) the following day. CONCLUSIONS: The findings from this study suggest that ambulatory children with CP may not sleep better after physical activity, and vice versa, indicating that the relationship is complex and needs further investigation.

The use of device-based accelerometry is a feasible method to measure 24-hour activity patterns with sleep and physical activity in ambulatory children with cerebral palsy.The relationships between sleep and physical activity in children with cerebral palsy are not as expected based on patterns shown in peers with typical development.Interventions for sleep in children with cerebral palsy require a holistic approach, focusing on daily physical activity patterns and relevant child- and contextual factors.

The short and long-term effects of a lifestyle intervention in children with mental illnesses: a randomized controlled trial (Movementss study).

BACKGROUND: A lifestyle including poor diet, physical inactivity, excessive gaming and inadequate sleep hygiene is frequently seen among Dutch children. These lifestyle behaviors can cause long-term health problems later in life. Unhealthy lifestyle and poor physical health are even more prevalent among children with mental illness (MI) such as autism, attention-deficit/hyperactivity disorder, depression, and anxiety. However, research on lifestyle interventions among children with MI is lacking. As a result, there are currently no guidelines, or treatment programs where children with MI and poor lifestyle can receive effective support. To address these issues and to provide insight into the effectiveness of lifestyle interventions in children with MI and their families, the Movementss study was designed. This paper describes the rationale, study design, and methods of an ongoing randomized controlled trial (RCT) comparing the short-term (12 weeks) and long-term (1 year) effects of a lifestyle intervention with care as usual (CAU) in children with MI and an unhealthy lifestyle. METHODS: A total of 80 children (6-12 years) with MI according to DSM-V and an unhealthy lifestyle are randomized to the lifestyle intervention group or CAU at a specialized child and adolescent mental hospital. The primary outcome measure is quality of life measured with the KIDSCREEN. Secondary outcomes include emotional and behavior symptoms, lifestyle parameters regarding diet, physical activity, sleep, and screen time, cognitive assessment (intelligence and executive functions), physical measurements (e.g., BMI), parenting styles, and family functioning, prior beliefs, adherence, satisfaction, and cost-effectiveness. Assessments will take place at the start of the study (T0), after 12 weeks (T1), six months (T2), and 12 months of baseline (T3) to measure long-term effects. DISCUSSION: This RCT will likely contribute to the currently lacking knowledge on lifestyle interventions in children with MI. TRIAL REGISTRATION: trialsearch.who.int/ NL9822. Registered at November 2nd, 2021.

School Problems and School Support for Children with Narcolepsy: Parent, Teacher, and Child Reports.

OBJECTIVE: To assess problems faced by children with type 1 narcolepsy (NT1) at school and obtain insight into potential interventions for these problems. METHODS: We recruited children and adolescents with NT1 from three Dutch sleep-wake centers. Children, parents, and teachers completed questionnaires about school functioning, interventions in the classroom, global functioning (DISABKIDS), and depressive symptoms (CDI). RESULTS: Eighteen children (7-12 years) and thirty-seven adolescents (13-19 years) with NT1 were recruited. Teachers' most frequently reported school problems were concentration problems and fatigue (reported by about 60% in both children and adolescents). The most common arrangements at school were, for children, discussing school excursions (68%) and taking a nap at school (50%) and, for adolescents, a place to nap at school (75%) and discussing school excursions (71%). Regular naps at home on the weekend (children 71% and adolescents 73%) were more common than regular naps at school (children 24% and adolescents 59%). Only a minority of individuals used other interventions. School support by specialized school workers was associated with significantly more classroom interventions (3.5 versus 1.0 in children and 5.2 versus 4.1 in adolescents) and napping at school, but not with better global functioning, lower depressive symptom levels, or napping during the weekends. CONCLUSIONS: Children with NT1 have various problems at school, even after medical treatment. Interventions to help children with NT1 within the classroom do not seem to be fully implemented. School support was associated with the higher implementation of these interventions. Longitudinal studies are warranted to examine how interventions can be better implemented within the school.

Accelerometer-measured physical activity, sedentary behavior, and sleep in children with cerebral palsy and their adherence to the 24-hour activity guidelines.

AIM: To measure and describe the 24-hour activities (i.e. physical activity, sedentary behavior, and sleep) and to examine adherence to the 24-hour activity guidelines among children with cerebral palsy (CP) using actigraphy. METHOD: Children's 24-hour activities were recorded over 7 days using hip- and wrist-worn ActiGraph wGT3X-BT accelerometers. RESULTS: In total, 362 days and 340 nights from 54 children with CP (Gross Motor Function Classification System [GMFCS] levels I-III; 44% females; median age [range] 6 years 6 months [3-12 years]) were included. Mean (SD) daily wear time was 746.2 (48.9) minutes, of which children spent on average 33.8% in light physical activity (251.6 [58.7] minutes per day), 5.2% in moderate-to-vigorous physical activity (38.5 [20.1] minutes per day), and the remaining 61.1% being sedentary (456.1 [80.4] minutes per day). Physical activity decreased while sedentary behavior increased with increasing GMFCS level. In total, 13% of all children met the physical activity recommendations, and 35% met the age-appropriate sleep duration recommendation. The proportion of children meeting the combined 24-hour guidelines for physical activity and sleep was low (5.9%), especially in those classified in GMFCS level III (0%). INTERPRETATION: The observed low 24-hour guideline adherence rates emphasize the importance of considering the entire continuum of movement behaviors in the care of children with CP, in efforts to promote healthy lifestyle behaviors and prevent negative health outcomes.

Device-based and subjective measurements of sleep in children with cerebral palsy: a comparison of sleep diary, actigraphy, and bed sensor data.

STUDY OBJECTIVES: To investigate how subjective assessments and device-based measurements of sleep relate to each other in children with cerebral palsy (CP). METHODS: Sleep of children with CP, classified at Gross Motor Function Classification System levels I-III, was measured during 7 consecutive nights using 1 subjective (ie, sleep diary) and 2 device-based (ie, actigraphy and bed sensor) instruments. The agreement between the instruments was assessed for all nights and separately for school- and weekend nights, using intraclass correlation coefficients (ICC) and Bland-Altman plots. RESULTS: A total of 227 nights from 38 children with CP (53% male; median age [range] 6 [2-12] years), were included in the analyses. Sleep parameters showed poor agreement between the 3 instruments, except for total time in bed, which showed satisfactory agreement between (1) actigraphy and sleep diary (ICC > 0.86), (2) actigraphy and bed sensor (ICC > 0.84), and (3) sleep diary and bed sensor (ICC > 0.83). Furthermore, agreement between sleep diary and bed sensor was also satisfactory for total sleep time (ICC > 0.70) and wakefulness after sleep onset (ICC = 0.55; only during weekend nights). CONCLUSIONS: Researchers and clinicians need to be aware of the discrepancies between instruments for sleep monitoring in children with CP. We recommend combining both subjective and device-based measures to provide information on the perception as well as an unbiased estimate of sleep. Further research needs to be conducted on the use of a bed sensor for sleep monitoring in children with CP. CITATION: van Rijssen IM, Hulst RY, Gorter JW, et al. Device-based and subjective measurements of sleep in children with cerebral palsy: a comparison of sleep diary, actigraphy, and bed sensor data. J Clin Sleep Med. 2023;19(1):35-43.

Parental perspectives on care for sleep in children with cerebral palsy: a wake-up call.

PURPOSE: Sleep problems are common in children with cerebral palsy (CP) and have a large impact on child health and family functioning. This qualitative study aimed to explore parental perspectives regarding the care for sleep of their young child (age 1-8 years) with CP. MATERIALS AND METHODS: Individual, semi-structured interviews were conducted with eighteen parents of a child with CP (GMFCS levels I-V). Inductive thematic analysis of the data was performed within each of the three preidentified domains: 1) Current situation; 2) Concerns; 3) Needs. RESULTS: In total, sixteen themes were identified across the three domains. Within the families' Current situation, parents expressed various issues concerning the care for sleep of their child both at night and during daytime, which are hampered by perceived deficiencies in healthcare, such as limited attention for sleep and lack of knowledge among health professionals. Themes within the Concerns and Needs domains encompassed experiences in the home environment relating to child, family and social aspects, while experiences in the healthcare setting included clinical practices and attitudes of healthcare professionals, as well as the broader organisation of care for sleep. CONCLUSIONS: Parents face numerous challenges caring for their child's sleep and the burden placed on families by sleep problems is underappreciated. In order to break the vicious circle of sleep problems and their disastrous consequences on the wellbeing of families, we need to wake up to parent-identified issues and shortcomings in healthcare. Care for sleep should be integrated into paediatric rehabilitation through routine inquiries, using a family-centered and multidisciplinary approach.IMPLICATIONS FOR REHABILITATIONThe heavy burden placed on families by sleep problems in children with cerebral palsy warrants acknowledgement in paediatric healthcare.Sleep should be routinely addressed by clinicians during health assessments using a family-centered, and multidisciplinary approach.Healthcare professionals ought to adopt a proactive, understanding, and non-judgmental attitude when addressing sleep problems.Future research should focus on developing sleep intervention strategies that take into account the diverse parental concerns and needs unique to each family situation.

What is in a name? Definitions of insomnia in people with intellectual disabilities.

BACKGROUND: The reported prevalence of insomnia symptoms in people with intellectual disabilities varies greatly, possibly due to the lack of a common definition. This article provides an overview of the different definitions used and formulates key points for a general definition. METHODS: A literature search was performed. An overview of the definitions used was given and compared to the third edition of the international classification of sleep disorders. RESULTS: The search yielded 16 studies. No uniform definition was used. Terminology and cut-off points of insomnia symptoms differed. Insomnia symptoms were mostly described as night-time problems. A minority of studies incorporated daytime consequences. CONCLUSION: An insomnia disorder entails more than merely night-time complaints and should include daytime consequences. A general definition is warranted. This definition should focus on night-time and daytime insomnia symptoms, incorporate subjective features, and discuss the use of objective measurements and influence of environmental circumstances.

Epilepsy and Sleep in the ATR-X Syndrome.

BACKGROUND: This study explores the prevalence, clinical characteristics, and treatment of epilepsy and sleep disorders in α thalassemia mental retardation (ATR-X) syndrome. DESIGN: In this cross-sectional study, 37 participants with ATR-X syndrome aged 1.8 to 44 years were studied using a customized epilepsy questionnaire, review of electroencephalography (EEG) findings, the modified Sleep Questionnaire of Simonds and Parraga and 2-week sleep diary. RESULTS: Eleven participants had a clinical diagnosis of generalized epilepsy (29.7%). Seizure types were generalized tonic-clonic seizures, absences, and myoclonia. Interictal EEG recordings in participants with GTCS showed no epileptic discharges in 78%. Similarly, EEG recordings during myoclonia and absences often demonstrated no epileptic discharges. Sleep problems (difficulty falling or maintaining sleep, and early awakening) were reported in 70%. Participants with reported sleep problems went to bed earlier (p = 0.027) and had a lower sleep efficiency (p < 0.01) than participants without sleep problems, but as a group they both had a sufficient total sleep time (9 hours and 52 minutes vs. 10 hours and 55 minutes). Sixteen participants (43.2) used medication to improve sleep (predominantly melatonin n = 10), being effective in only two. CONCLUSION: One-third of participants with ATR-X syndrome had a clinical diagnosis of epilepsy, but the absence of EEG abnormalities in suspected epileptic seizures questions this diagnosis in these patients. EEG recording during seizure like symptoms is warranted before making an epilepsy diagnosis. Seventy percent experienced sleep problems, although total sleep time was normal in most participants. Long bedtimes might have a negative influence on sleep efficiency.

Recognizing the Symptom Spectrum of Narcolepsy to Improve Timely Diagnosis: A Narrative Review.

Narcolepsy is a chronic sleep disorder with a strong negative impact on quality of life, especially when untreated. Diagnostic delay is a persistent problem, with obvious detrimental effects on patients. A diagnosis of narcolepsy may be delayed because of its broad symptom presentation which is much more encompassing than the classical "tetrad" of sleepiness, cataplexy, hallucinations, and sleep paralysis. Furthermore, symptoms can vary over time. Presentation of symptoms can also be markedly different between children and adults. Finally, common sleep-related comorbidities increase the risk of narcolepsy being masked by overlapping symptoms or treatment. In this review, we provide a detailed description of the broad and dynamic symptom spectrum of narcolepsy, with specific attention to the different manifestations in both adults and children. The overarching goal is to help not only sleep specialists, but general practitioners, pediatricians, and other caregivers with early recognition and prompt diagnosis of this severe but treatable disorder.

It is All in the Wrist: Wearable Sleep Staging in a Clinical Population versus Reference Polysomnography.

PURPOSE: There is great interest in unobtrusive long-term sleep measurements using wearable devices based on reflective photoplethysmography (PPG). Unfortunately, consumer devices are not validated in patient populations and therefore not suitable for clinical use. Several sleep staging algorithms have been developed and validated based on ECG-signals. However, translation from these techniques to data derived by wearable PPG is not trivial, and requires the differences between sensing modalities to be integrated in the algorithm, or having the model trained directly with data obtained with the target sensor. Either way, validation of PPG-based sleep staging algorithms requires a large dataset containing both gold standard measurements and PPG-sensor in the applicable clinical population. Here, we take these important steps towards unobtrusive, long-term sleep monitoring. METHODS: We developed and trained an algorithm based on wrist-worn PPG and accelerometry. The method was validated against reference polysomnography in an independent clinical population comprising 244 adults and 48 children (age: 3 to 82 years) with a wide variety of sleep disorders. RESULTS: The classifier achieved substantial agreement on four-class sleep staging with an average Cohen's kappa of 0.62 and accuracy of 76.4%. For children/adolescents, it achieved even higher agreement with an average kappa of 0.66 and accuracy of 77.9%. Performance was significantly higher in non-REM parasomnias (kappa = 0.69, accuracy = 80.1%) and significantly lower in REM parasomnias (kappa = 0.55, accuracy = 72.3%). A weak correlation was found between age and kappa (ρ = -0.30, p<0.001) and age and accuracy (ρ = -0.22, p<0.001). CONCLUSION: This study shows the feasibility of automatic wearable sleep staging in patients with a broad variety of sleep disorders and a wide age range. Results demonstrate the potential for ambulatory long-term monitoring of clinical populations, which may improve diagnosis, estimation of severity and follow up in both sleep medicine and research.

The CHD8/CHD7/Kismet family links blood-brain barrier glia and serotonin to ASD-associated sleep defects.

Sleep disturbances in autism and neurodevelopmental disorders are common and adversely affect patient's quality of life, yet the underlying mechanisms are understudied. We found that individuals with mutations in CHD8, among the highest-confidence autism risk genes, or CHD7 suffer from disturbed sleep maintenance. These defects are recapitulated in Drosophila mutants affecting kismet, the sole CHD8/CHD7 ortholog. We show that Kismet is required in glia for early developmental and adult sleep architecture. This role localizes to subperineurial glia constituting the blood-brain barrier. We demonstrate that Kismet-related sleep disturbances are caused by high serotonin during development, paralleling a well-established but genetically unsolved autism endophenotype. Despite their developmental origin, Kismet's sleep architecture defects can be reversed in adulthood by a behavioral regime resembling human sleep restriction therapy. Our findings provide fundamental insights into glial regulation of sleep and propose a causal mechanistic link between the CHD8/CHD7/Kismet family, developmental hyperserotonemia, and autism-associated sleep disturbances.

Sleep problems in children with cerebral palsy and their parents.

AIM: To describe: (1) the frequency and types of sleep problems, (2) parent-rated satisfaction with their child's and their own sleep, and (3) child factors related to the occurrence of sleep problems in children with cerebral palsy (CP) and their parents. The secondary objective was to compare the sleep outcomes of children with CP with those from typically developing children and their parents. METHOD: The Sleep section of the 24-hour activity checklist was used to assess the sleep of children with CP and their parents and the sleep of typically developing children and their parents. RESULTS: The sleep outcomes of 90 children with CP (median age 5y, range 0-11y, 53 males, 37 females, 84.4% ambulatory) and 157 typically developing peers (median age 5y, range 0-12y; 79 males, 78 females) and their parents were collected. Children with CP were more likely to have a sleep problem than typically developing children. Non-ambulatory children with CP were more severely affected by sleep problems than ambulatory children. The parents of non-ambulatory children were less satisfied about their child's and their own sleep. Waking up during the night, pain/discomfort in bed, and daytime fatigue were more common in children with CP and more prevalent in children who were non-ambulatory. INTERPRETATION: These findings highlight the need to integrate sleep assessment into routine paediatric health care practice. What this paper adds Children with cerebral palsy (CP) are more likely to have a sleep problem than typically developing peers. Non-ambulatory children with CP are more severely affected by sleep problems. One-third of parents of children with CP report feeling sleep-deprived often or always compared to a quarter of parents of typically developing children.

The value of cardiorespiratory parameters for sleep state classification in preterm infants: A systematic review.

Cardiorespiratory activity is highly associated with infants' sleep duration and quality. We performed a systematic literature search of PubMed and EMBASE databases to investigate if and how cardiorespiratory parameters can be used for sleep state classification in preterm infants and in what way maturation influences this relation. All retrieved citations were screened against predetermined inclusion and exclusion criteria. Only studies of preterm infants (<37 wk postmenstrual age during sleep state classification) admitted to a neonatal ward and of whom at least one sleep state and one cardiorespiratory parameter was measured, were included. Two researchers independently reviewed the included studies on methodological quality. Of the 1097 initially retrieved studies, 23 were included for analysis. Heart rate and respiration frequency are strongly correlated with active sleep and quiet sleep. In quiet sleep, as compared to active sleep, respiratory frequency is more stable, and the heart rate is lower and less variable. This association, however, differed across preterm birth subtypes (i.e., extremely, very or late preterm), indicating that maturation - in the form of both gestational and postnatal age - influences the cardiorespiratory characteristics of preterm sleep states. The knowledge gained from this review can help improve behavioral sleep classification and automated sleep classification algorithms for preterm infants.

The relationship between preterm birth and sleep in children at school age: A systematic review.

Premature birth (before 37 weeks of gestation) has been linked to a variety of adverse neurological outcomes. Sleep problems are associated with decreased neurocognitive functioning, which is especially common in children born preterm. The exact relationship between prematurity and sleep at school age is unknown. A systematic review is performed with the aim to assess the relationship between prematurity and sleep at school age (5th to 18th year of life), in comparison to sleep of their peers born full-term. Of 347 possibly eligible studies, nine were included. The overall conclusion is that prematurity is associated with earlier bedtimes and a lower sleep quality, in particular more nocturnal awakenings and more non-rapid eye movement stage 2 sleep. Interpretations and limitations of the review are discussed. Moreover, suggestions for future research are brought forward, including the need for a systematic approach with consistent outcome measures in this field of research. A better understanding of the mechanisms that influence sleep in the vulnerable group of children born preterm could help optimize these children's behavioral and intellectual development.

Psychometric properties of the Patient-Reported Outcomes Measurement Information System (PROMIS) Sleep Disturbance and Sleep-Related Impairment item banks in adolescents.

Sleep problems have a high prevalence and negative daytime consequences in adolescents. Current sleep measures for this age group have limitations. The Patient-Reported Outcomes Measurement Information System (PROMIS® ) developed sleep item banks for adults. In a previous validation study, these item banks were adapted to a shortened version for adolescents. The current study aimed to further explore the psychometric properties of the 11-item Sleep-Related Impairment and 23-item Sleep Disturbance item banks in Dutch adolescents. We investigated structural validity by testing item response theory assumptions and model fit; measurement invariance by performing differential item functioning analyses; performance as a computerized adaptive test; reliability by marginal reliability estimates and test-retest reliability (intraclass correlation coefficients and limits of agreement); and construct validity by hypothesis testing. Additionally, we provide mean values for the item banks. The study sample consisted of 1,046 adolescents (mean age 14.3 ± 1.6), including 1,013 high-school students and 33 sleep-clinic patients. The Sleep Disturbance-23 showed lack of unidimensionality, but had sufficient test-retest reliability, and could distinguish between adolescents with and without sleep or health issues. The Sleep-Related Impairment-11 showed sufficient unidimensionality and model fit and was thus tested as a computerized adaptive test, demonstrating an equal amount of reliable measures to the full item bank. Furthermore, the Sleep-Related Impairment-11 could distinguish between adolescents with and without sleep or health issues and test-retest reliability was moderate. The use of both item banks in the full form and the use of the Sleep-related Impairment-11 as a computer adaptive test is recommended.

24-hour activity for children with cerebral palsy: a clinical practice guide.

The association between physical activity and health has been clearly established, and the promotion of physical activity should be viewed as a cost-effective approach that is universally prescribed as a first-line treatment for nearly every chronic disease. Health care providers involved in the care for individuals with cerebral palsy (CP) are encouraged to take an active role in promoting their health and well-being. Balancing activity behaviours across the whole day, with improved physical activity, reduced sedentary time, and healthy sleep behaviours, can set up infants, preschool-, and school-aged children with CP for a healthy trajectory across their lifetime. However, most clinicians do not apply a systematic surveillance, assessment, and management approach to detect problems with physical activity or sleep in children with CP. Consequently, many children with CP miss out on an important first line of treatment. This article presents an evidence-informed clinical practice guide with practical pointers to help practitioners in detecting 24-hour activity problems as a critical step towards adoption of healthy lifestyle behaviours for children with CP that provide long-term health benefits.

La asociación entre la actividad física y la salud se ha establecido claramente, y la promoción de la actividad física debe verse como un enfoque rentable que se prescribe universalmente como tratamiento de primera línea para casi todas las enfermedades crónicas. Se alienta a los proveedores de atención médica involucrados en la atención de personas con parálisis cerebral (PC) a que tomen un papel activo en la promoción de su salud y bienestar. Equilibrar las conductas de actividad durante todo el día, con una mejor actividad física, una reducción del tiempo sedentario y conductas de sueño saludables, puede preparar a los bebés, niños en edad preescolar y escolar con PC para una trayectoria saludable a lo largo de su vida. Sin embargo, la mayoría de los médicos no aplican un enfoque sistemático de vigilancia, evaluación y manejo para detectar problemas con la actividad física o el sueño en niños con PC. En consecuencia, muchos niños con PC pierden una importante primera línea de tratamiento. Este artículo presenta una guía de práctica clínica basada en evidencia con consejos prácticos para ayudar a los profesionales a detectar problemas de actividad durante las 24 horas como un paso crítico hacia la adopción de comportamientos de estilo de vida saludables para niños con PC que brindan beneficios para la salud a largo plazo.

A associação entre atividade física e saúde tem sido claramente estabelecida,e a promoção de atividade física deve ser vista como abordagem custo-efetiva que é universalmente prescrita como tratamento de primeira linha para toda doença crônica. Profissionais da saúde envolvidos no cuidado para indivíduos com paralisia cerebral (PC) são encorajados a assumir um papel ativo na promoção da sua saúde e bem estar. Balancear comportamentos ativos ao longo de todo o dia, com melhora da atividade física, redução do tempo sedentário, e hábitos de sono saudáveis, pode preparar lactentes, pré-escolares e escolares com PC para uma trajetória saudável ao longo de sua vida. No entanto, a maior parte dos clínicos não aplica uma vigilância, avaliação e manejo sistemáticos para detectar problemas de atividade física ou sono em crianças com PC. Consequentemente, muitas crianças com PC deixam de receber uma importante primeira linha de tratamento. Este artigo apresenta um guia para prática clínica informado por evidências com pontos práticos para ajudar profissionais a detectar problemas de atividade física ao longo de 24 horas como um passo crítico em direção à adoção de comportamentos ativos de estilo de vida para crianças com PC que oferecem benefícios de longo prazo para a saúde.

Two sides of a coin: differential response to COVID-19 distancing measures in children with narcolepsy.

NONE: Narcolepsy is a chronic neurological sleep disorder, debuting before age 15 years in one-third of patients. Narcolepsy has a negative influence on quality of life, with daily functioning being affected by concomitant cognitive, behavioral, and social problems. In December 2019, a new coronavirus emerged worldwide, causing the severe respiratory disease COVID-19. In the Netherlands, a partial lockdown was implemented that included the closure of schools. Here we present 3 illustrative case reports that teach important lessons for the treatment of pediatric narcolepsy. We observed significant consequences of the partial lockdown measures on daytime functioning, well-being, and school performance, both negative and positive. The consequences of the lockdown led to valuable insights for further treatment, substantiating the importance of personalizing education. Involvement of specialized student counselors, flexibility in the way that education is delivered, and enhancement of environmental factors could help in guiding young patients with narcolepsy through challenges at school.

Sleep health practices and sleep knowledge among healthcare professionals in Dutch paediatric rehabilitation.

BACKGROUND: Sleep disorders are highly prevalent in children with neurodisabilities, yet they seem under-recognized in paediatric rehabilitation settings. The aim of this study was to assess among two groups of healthcare professionals (HCPs) working in paediatric rehabilitation: (1) sleep health practices and (2) knowledge about sleep physiology, sleep disorders and sleep hygiene. METHODS: We performed a cross-sectional sleep survey among medical and non-medical HCPs and the general population. Participants (30 rehabilitation physicians [RPs], 54 allied health professionals [AHPs] and 63 controls) received an anonymous 30-item survey consisting of three domains: (1) general information, (2) application of sleep health practices and (3) sleep knowledge. RESULTS: RPs address sleep issues more frequently in clinical practice than AHPs. Sleep interventions mostly consist of giving advice about healthy sleep practices and are given by the majority of HCPs. While RPs demonstrated the highest scores on all knowledge domains, total sleep knowledge scores did not exceed 50% correct across groups, with AHPs and controls showing equal scores. Sleep hygiene rules closest to bedtime and related to the sleep environment were best known, whereas those related to daytime practices were rarely mentioned across all groups. A small minority of HCPs (RPs 20%; AHPs 15%) believed to possess sufficient sleep knowledge to address sleep in clinical practice. No association was found between self-perceived knowledge and sleep knowledge scores among HCPs. CONCLUSIONS: Sleep should become a standard item for review during routine health assessments in paediatric rehabilitation settings. HCPs' limited exposure to sleep education may result in feelings of incompetence and inadequate sleep knowledge levels, affecting their sleep health practices. Appropriate sleep training programs should be implemented to empower HCPs with knowledge, skills and confidence, needed to recognize and treat sleep disorders in children with neurodisabilities, as well as to be able to guide parents.

Effect of treatment on cognitive and attention problems in children with narcolepsy type 1.

STUDY OBJECTIVES: To ascertain the presence of cognitive and attention problems in treatment naïve children with narcolepsy type 1 (NT1) and to explore whether children recently diagnosed with NT1 improve with respect to cognition and attention problems 1 year after regular treatment for NT1. METHODS: A total of 15 treatment naïve children (7-15 years) with recently diagnosed NT1 were recruited from three sleep medicine centers in the Netherlands. The control group consisted of 15 healthy children, being frequency matched on age and gender. Both groups were investigated at baseline to examine intelligence profile (Wechsler Intelligence Scale for Children [WISC] III), attention problems, and processing speed (Bourdon Vos and sustained attention to respond task [SART]). These tests were repeated in children with NT1 1 year after regular (behavioral and medication) treatment for NT1. RESULTS: Children with NT1 scored significantly lower on the verbal scale and processing speed subscale of the WISC III, showed more fluctuations in reaction time of the Bourdon Vos and made more mistakes during the SART than the healthy control group at baseline. Children with NT1 significantly improved on total IQ score, and on the WISC indices processing speed, and perceptual organization 1 year after treatment. At follow-up, test scores of treated children were largely comparable to those of the control group at baseline. CONCLUSIONS: Children with NT1 show improvement in several cognitive domains 1 year after start of treatment. Our findings stress the need for early detection and treatment of narcolepsy in childhood.

A Mobile App for Longterm Monitoring of Narcolepsy Symptoms: Design, Development, and Evaluation.

BACKGROUND: Narcolepsy is a chronic sleep disorder with a broad variety of symptoms. Although narcolepsy is primarily characterized by excessive daytime sleepiness and cataplexy (loss of muscle control triggered by emotions), patients may suffer from hypnagogic hallucinations, sleep paralysis, and fragmented night sleep. However, the spectrum of narcolepsy also includes symptoms not related to sleep, such as cognitive or psychiatric problems. Symptoms vary greatly among patients and day-to-day variance can be considerable. Available narcolepsy questionnaires do not cover the whole symptom spectrum and may not capture symptom variability. Therefore, there is a clinical need for tools to monitor narcolepsy symptoms over time to evaluate their burden and the effect of treatment. OBJECTIVE: This study aimed to describe the design, development, implementation, and evaluation of the Narcolepsy Monitor, a companion app for long-term symptom monitoring in narcolepsy patients. METHODS: After several iterations during which content, interaction design, data management, and security were critically evaluated, a complete version of the app was built. The Narcolepsy Monitor allows patients to report a broad spectrum of experienced symptoms and rate their severity based on the level of burden that each symptom imposes. The app emphasizes the reporting of changes in relative severity of the symptoms. A total of 7 patients with narcolepsy were recruited and asked to use the app for 30 days. Evaluation was done by using in-depth interviews and user experience questionnaire. RESULTS: We designed and developed a final version of the Narcolepsy Monitor after which user evaluation took place. Patients used the app on an average of 45.3 (SD 19.2) days. The app was opened on 35% of those days. Daytime sleepiness was the most dynamic symptom, with a mean number of changes of 5.5 (SD 3.7) per month, in contrast to feelings of anxiety or panic, which was only moved 0.3 (SD 0.7) times per month. Mean symptom scores were highest for daytime sleepiness (1.8 [SD 1.0]), followed by lack of energy (1.6 [SD 1.4]) and often awake at night (1.5 [SD 1.0]). The personal in-depth interviews revealed 3 major themes: (1) reasons to use, (2) usability, and (3) features. Overall, patients appreciated the concept of ranking symptoms on subjective burden and found the app easy to use. CONCLUSIONS: The Narcolepsy Monitor appears to be a helpful tool to gain more insight into the individual burden of narcolepsy symptoms over time and may serve as a patient-reported outcome measure for this debilitating disorder.