Effectiveness of an intervention program on physical activity in children with narcolepsy type 1.

OBJECTIVES: Physical activity (PA) is recommended as part of the management of narcolepsy type 1 (NT1). This study aimed at 1) characterizing PA in children and adolescents treated for NT1 using objective and subjective measurements, 2) evaluating how PA is associated with NT1 symptoms and comorbidities, and 3) evaluating the effects of an Adapted Physical Activity (APA) program on PA and clinical characteristics. PATIENTS/METHODS: Patients with NT1 from the National Reference Center of Narcolepsy (Lyon, France) were consecutively included in an APA intervention protocol. Narcolepsy symptoms and comorbidities were collected using standardized questionnaires and sustained attention was evaluated using the Bron-Lyon Attention Stability Test before and after the four-week APA intervention. PA was measured objectively using actigraphy throughout the study. RESULTS: Twenty-seven NT1 patients were included (median age 14.7 years [8.3-18.4], cataplexy 88.9%, obesity 37.0%). At baseline, 52.4% of the patients had satisfactory PA levels according to international recommendations. Patients with leisure-time PA (LTPA) showed higher quality of life than patients without. 45% of the patients increased PA during the intervention compared to baseline. These responsive patients had more depressive feelings and tended to have lower objective PA than non-responsive patients at baseline. No significant correlation was found between PA levels before and during the intervention and other clinical data. CONCLUSIONS: Most children with NT1 showed satisfying PA levels despite their daytime sleepiness. LTPA engagement was associated with higher quality of life. An APA intervention could be effective in children with narcolepsy, especially for those with depressive feelings.

Gas exchange parameters for the prediction of obstructive sleep apnea in infants.

STUDY OBJECTIVES: Sleep laboratory polysomnography (PSG) is the gold standard for obstructive sleep apnea (OSA) diagnosis in infants, but its access remains limited. Oximetry-capnography is another simple and widely used tool that can provide information on the presence of desaturations and alveolar hypoventilation. However, its reliability is debated. This study aimed at examining its use in determining OSA severity in infants. METHODS: This retrospective study was conducted in a sleep unit in a tertiary hospital, in infants < 4 months old with clinical signs of OSA or Pierre Robin Sequence (PRS) who underwent a one-night PSG coupled with oximetry-capnography. RESULTS: Among the 78 infants included (median [IQR] age: 61 [45-89] days at PSG), 44 presented with PRS, and 34 presented with isolated airway obstruction. The clinical, sleep and respiratory characteristics were not significantly different between the two subgroups. In the entire cohort, 63.5% had severe OSA. Median OAHI was 14.5 events/h [7.4-5.9], SpO2 was 97.4% [96.5-98.1], and PtcCO2 was 41.1 mmHg [38.3-44.9]. The optimal threshold to predict OAHI > 10 events/h was 6 events/h for ODI3% (sensitivity 95.7%, specificity 51.9%) and 2 events/h for ODI4% (sensitivity 95.7%, specificity 48.1%). CONCLUSIONS: Whereas transcutaneous capnography does not appear to be sufficient in predicting severe OSA in infants < 4 months old with PRS or clinical signs of OSA, oximetry may be a useful alternative for the screening of severe OSA in infants in the absence of PSG.

Hypoventilation in patients with Prader-Willi syndrome across the pediatric age.

OBJECTIVES: Few data on alveolar hypoventilation in Prader-Willi syndrome (PWS) are available and the respiratory follow-up of these patients is not standardized. The objectives of this study were to evaluate the prevalence of alveolar hypoventilation in children with PWS and identify potential risk factors. STUDY DESIGN: This retrospective study included children with PWS recorded by polysomnography (PSG) with transcutaneous carbon dioxide pressure (PtcCO2) or end-tidal CO2 (ETCO2) measurements, between 2007 and 2021, in a tertiary hospital center. The primary outcome was the presence of alveolar hypoventilation defined as partial pressure of carbon dioxide (pCO2) ≥ 50 mmHg during ≥2% of total sleep time (TST) or more than five consecutive minutes. RESULTS: Among the 57 included children (38 boys, median age 4.8 years, range 0.1-15.6, 60% treated with growth hormone [GH], 37% obese), 19 (33%) had moderate-to-severe obstructive sleep apnea syndrome (defined as obstructive apnea-hypopnea index ≥5/h) and 20 (35%) had hypoventilation. The median (range) pCO2 max was 49 mmHg (38-69). Among the children with hypoventilation, 25% were asymptomatic. Median age and GH treatment were significantly higher in children with hypoventilation compared to those without. There was no significant difference in terms of sex, BMI, obstructive or central apnea-hypopnea index between both groups. CONCLUSION: The frequency of alveolar hypoventilation in children and adolescents with PWS is of concern and may increase with age and GH treatment. A regular screening by oximetry-capnography appears to be indicated whatever the sex, BMI, and rate of obstructive or central apneas.

Does the brain sleep differently depending on intellectual abilities?

AIMS: To compare the children's sleep electroencephalogram according to their intellectual profile. METHODS: Children were grouped according to their Wechsler Intelligence Scale for Children (WISC) scores (17 with normal intelligence quotient [IQ, NIQ] and 24 with high IQ [HIQ]). Comparisons of spectral power between groups and its relationship with WISC scores were assessed using analyses of variance and linear regression models, adjusted for age and sex. RESULTS: Children with HIQ had more rapid eye movement (REM) sleep, especially late at night, and more power in slow-frequency bands during REM sleep than those with NIQ. There were also positive associations between the processing speed index and the spectral power in ß bands in NREM sleep, and with the spectral power in α, σ, ß, and γ bands in REM sleep, with different associations between groups. CONCLUSION: The enhanced power in slow bands during REM sleep in children with HIQ overlaps with that of typical REM sleep oscillations thought to be involved in emotional memory consolidation. The dissimilar relationships between spectral power and WISC scores in NIQ and HIQ groups may underlie functional differences in brain activity related to cognitive efficiency, questioning the direction of the relationship between sleep and cognitive functioning.

Salivary α-amylase as a marker of sleep disorders: A theoretical review.

Sleep disorders are commonplace in our modern societies. Specialized hospital departments are generally overloaded, and sleep assessment is an expensive process in terms of equipment, human resources, and time. Biomarkers would usefully complement current measures in the screening and follow-up of sleep disorders and their daytime repercussions. Among salivary markers, a growing body of literature suggests that salivary α-amylase (sAA) may be a cross-species marker of sleep debt. However, there is no consensus as to the direction of variation in sAA with sleep disorders. Herein, after describing the mechanisms of sAA secretion and its relationship with stress, studies assessing the relationship between sAA and sleep parameters are reviewed. Finally, the influence of confounding factors is discussed, along with methodological considerations, to better understand the fluctuations in sAA and facilitate future studies in the field.

Metabolic disturbances in children with narcolepsy: a retrospective study.

STUDY OBJECTIVES: To determine the prevalence of metabolic syndrome (MS) in children with narcolepsy and to evaluate their clinical and sleep characteristics according to the different components of MS. METHODS: This retrospective study consisted of 58 de novo children with narcolepsy (median age: 12.7 years, 48.3% of boys). The recently published MS criteria in a French population of children were used. Clinical and sleep characteristics were compared between groups with different components of MS. RESULTS: MS was present in 17.2% of children with narcolepsy, among whom 79.3% presented with high homeostasis model assessment for insulin resistance (HOMA-IR), 25.9% with high body mass index, 24.1% with low high-density lipoprotein cholesterol (HDL-C), and 12.1% with high triglycerides. Patients with at least two MS components had more night eating behaviors and tended to have lower percentage of slow-wave sleep and more fragmented sleep. On multiple sleep latency test, they had shorter mean sleep latencies to rapid eye movement (REM), non-REM sleep and tended to have more sleep onset REM periods (SOREMPs) than those with less than two MS components. CONCLUSIONS: Insulin resistance was found to be the core metabolic disturbance in obese as well as in nonobese children with narcolepsy. Children with narcolepsy with at least two MS components presented a more severe daytime sleepiness and a higher prevalence of night-eating behaviors than those with less than two MS components. Such children might benefit from early evaluation and management in order to prevent future complications.

Obesity in children with narcolepsy: metabolic and endocrine comorbidities.

STUDY OBJECTIVES: Narcolepsy with cataplexy is associated with obesity in children. We proposed to assess whether metabolic complications were linked to narcolepsy regardless of obesity. The second aim of the study was to compare endocrine comorbidities in obese children with narcolepsy and control patients. METHODS: We performed a case-control study in Pediatric Sleep Unit and Pediatric Endocrinology Unit of Woman Mother Child Hospital (Lyon, France) comparing 22 children with narcolepsy with 22 sex-, pubertal stage-, and BMI-matched non-syndromic obese patients. Clinical examination, biological measurements including an oral glucose tolerance test, and abdominal ultrasound were performed. RESULTS: No difference regarding glucidic, lipid profile, hepatic, respiratory, and cardiovascular parameters were found between narcoleptic and control participants. Insulin sensitivity did not differ between the two groups. Control patients had more first-degree family history of overweight or obesity than children with narcolepsy (83% vs. 50%, p = .05). Prevalence of precocious puberty in children with narcolepsy was not higher than in control participants, but all the cases of advanced puberty involved children with narcolepsy who were diagnosed before 11 years old. All cases of central hypothyroidism belong to the narcoleptic group, who presented lower thyroid-stimulating hormone and fTA values compared to control children (respectively p = .03 and p = .001). CONCLUSIONS: No difference regarding metabolic complications was found between children with narcolepsy and control participants. Thus, metabolic disorders may be related to weight gain rather than a narcolepsy-specific risk. The presence of hypothyroidism and advanced puberty suggests a global involvement of hypothalamic structures in children with narcolepsy.

Neurocognitive evaluation of children with down syndrome and obstructive sleep apnea syndrome.

Obstructive sleep apnea syndrome (OSAS) treatment has been shown to improve cardiac behavioral and cognitive functions in typically developing children. Early OSAS diagnosis in children with Down syndrome (DS) would be important to prevent its complications, especially cognitive ones, but remains overlooked. The main objective of our study was to assess the cognitive function of children with DS, with and without OSAS. The second objective was to determine the impact of the therapeutic intervention on the cognitive function of children with OSAS. This study included 41 children with DS who underwent polysomnography for OSAS diagnosis and a cognitive evaluation. They were aged between 3.4 and 17.3 years and 24 (59%) were boys. Their median OAHI was 2.6 (0-31)/h of sleep, 30 (73%) were diagnosed with OSAS (15 had mild OSAS, and 15 had moderate/severe OSAS). Some scores of the Raven's colored progressive matrices were negatively correlated with the respiratory arousal index, OAHI tended to be positively correlated with Reiss behavioral problems. 24 (59%) patients received a treatment. Even if we were unable to demonstrate this formally due that only 16 children (39%) accepted a follow-up visit, some displayed improvement in their neuropsychological scores, especially those with moderate/severe OSAS after treatment. Children with DS have low intellectual abilities and more risk of developing OSAS compared to the general population, which may lead to further neurocognitive impairment. Early screening and management are important in this population to prevent any further neurocognitive delay in their development.

Interest of the BLAST paradigm and salivary markers for the evaluation of sleepiness in drivers.

Objectives: Sleepiness is associated with decreased cognitive abilities and remains one of the main causes of fatal road accidents. The tools currently available to assess sleepiness, such as questionnaires, are subject to intra- and inter-individual variability, while multiple sleep latency tests are only feasible in few sleep laboratories. The main objective of this study was to explore new potential markers (neurocognitive, biological) to objectively assess sleepiness in drivers. Methods: A total of 186 drivers (median age 44 years, range 20-74 years, 73% men, 14% obese) were included during a break at a highway service area, in the morning, while on the road for vacation. Questionnaires on sleepiness and sleep characteristics (habitual and on the night before travel), the Bron-Lyon Attention Stability Test (BLAST), and two salivary samples (α-amylase and oxalate) were collected. Associations between measures of sleepiness [Epworth Sleepiness Scale (ESS), and Stanford Sleepiness Scale (SSS)], sleep characteristics, neurocognitive, and biological markers were tested using regression models adjusted for confounding factors. Results: The night before travel, 83% of the drivers reduced their sleep time and 30% slept 5 h or less. The higher the number of miles to be traveled, the higher the decrease, and the shorter the sleep time. The night before travel, 18 and 24% of the drivers complained of poor sleep quality and difficulty falling asleep. The sleep characteristics on the night before travel were associated with the habitual sleep characteristics. At the time of the test, 47% of the drivers scored pathologically on the SSS. Poor sleep quality and difficulty falling asleep the night before travel were associated with increased sleepiness as assessed by the SSS and decreased attentional ability as assessed by the BLAST. No association between salivary markers and acute sleepiness was observed. Conclusions: The sleep characteristics of the night before travel were associated with sleepiness and attentional performance. The SSS and the BLAST could be used by individual drivers in a self-evaluation context. Biological markers showed a high variability and limited association with sleep parameters across subjects, emphasizing the need for within-subject designs to assess their usefulness.

Sleep and Psychosocial Characteristics of Children with Narcolepsy According to Their Intellectual Profile: A Case-Control Study.

Adequate intellectual abilities are a protective factor for psychosocial adjustments in chronic disorders. The main objective of this study was to assess the cognitive abilities, sleep, and psychosocial characteristics of children with narcolepsy compared to controls, according to their intellectual profile. Children underwent a polysomnography, completed an intellectual ability assessment, and filled out standardized questionnaires. The group with an intelligence quotient (IQ) in the area of high intellectual potential (high IQ, HIQ) consisted of 25 children with narcolepsy (HIQ-N, 40% boys, median age 11.5 years, 48% with obesity, 60% under treatment) and 25 controls (HIQ-C, 68% boys, median age 11.7 years). Compared to HIQ-C, HIQ-N had a lower perceptual reasoning index and fewer conduct disorders. The group with an IQ in the normal range (NIQ) consisted of 22 children with narcolepsy (NIQ-N, 55% boys, median age 12.1 years, 59% with obesity, 64% under treatment) and 21 controls (NIQ-C, 68% boys, median age 10 years). NIQ-N presented the same intellectual profile as NIQ-C but reported more school difficulties. In children with HIQ, those with narcolepsy appear to have a different cognitive profile than controls. NIQ seems to predict a greater impact of narcolepsy on daily-life functioning.

Characterization of rapid weight gain phenotype in children with narcolepsy.

OBJECTIVES: To characterize the rapid weight gain (RWG) phenotype associated with the onset of childhood narcolepsy and to determine whether it could constitute a marker of severity of the disease. METHODS: RWG was defined using the BMI z-score slope reported to one year (>0.67 SD) from symptom onset to disease diagnosis. We compared the clinical, metabolic, and sleep characteristics between patients with or without RWG at diagnosis. Pharmacological management, anthropometric, and clinical progression were also evaluated during the follow-up. RESULTS: A total of 84 de novo narcoleptic pediatric patients were included; their median age at diagnosis was 12.0 years; 59.5% boys, 90.5% cataplexy, and 98.7% HLA-DQB1*06:02, 57% had RWG profile. RWG patients were younger at diagnosis than non-RWG patients, despite a shorter diagnostic delay. They had a higher BMI z-score and a higher prevalence of obesity at diagnosis, but not at symptom onset, and higher adapted Epworth Sleepiness Scale and Insomnia Severity Index scores than non-RWG patients. No differences on nocturnal polysomnography and multiple sleep latency tests were found between groups at disease diagnosis. After a median follow-up of 5 years, RWG patients still had a higher BMI z-score and a higher prevalence of obesity despite benefiting from the same therapeutic management and displaying improvement in sleepiness and school difficulties. CONCLUSIONS: Narcoleptic RWG patients were younger, sleepier, and the prevalence of obesity was higher at diagnosis despite a shorter diagnostic delay than that of non-RWG patients. These patients had also a higher risk of developing a long-term obesity, despite a positive progression of their narcoleptic symptoms. RGW could then represent a maker of a more severe phenotype of childhood narcolepsy, which should inspire a prompt and more offensive management to prevent obesity and its complications.

Maturation of Arousals during Day and Night in Preterm Infants.

The objective of this study was to compare the maturation of spontaneous arousals during day and night sleep in preterm and term infants. From the Autonomic Baby Evaluation study, the sleep and arousal characteristics of 12 preterm (35.1 ± 2.1 weeks' gestational age, GA) and 21 term (39.8 ± 0.8 weeks GA) newborns were compared between diurnal and nocturnal sleep periods at birth (M0) and 6 months (M6) of age. Models were adjusted for time (night/day), maturation (M0/M6), prematurity (yes/no). We found that preterm infants had less active sleep (AS)% than term infants with maturation during both day and night sleep, which may reflect accelerated brain maturation secondary to stress or environmental exposure after birth. Moreover, there was a difference in arousal maturation during day and night sleep in the preterm infants, as shown previously for term infants, which suggests the emergence of a circadian rhythm during the earliest postnatal period. We also showed that compared to term infants, these moderate preterm infants had fewer total arousals and, more specifically, fewer arousals in AS during day and night sleep, exposing them to a higher risk of sudden infant death syndrome.

Sleep disorders and ADHD symptoms in children and adolescents with typical absence seizures: An observational study.

OBJECTIVE: To assess the occurrence of sleep disorders (SD) and attention deficit hyperactivity disorder (ADHD) symptoms in children with typical absence seizures (TAS) compared to control children and to evaluate the impact of epilepsy-related factors on sleep and attention in children with TAS. METHODS: The Sleep Disturbance Scale for Children (SDSC) and the ADHD rating scale were filled in by parents of a cohort composed by 82 children aged from 5 to 15.6 years, 49% of boys (41 with TAS with a syndromic diagnosis of childhood absence epilepsy and 41 controls). For children with TAS, the Pediatric Epilepsy Side Effects Questionnaire was completed. Statistical analyses were conducted in order to compare sleep and attention scores between groups. In children with TAS, a correlation was computed between these scores. Logistics regression models were conducted to identify predictors of excessive diurnal sleepiness and inattention in children with TAS. RESULTS: Compared to controls, children with TAS had higher total scores for subjective sleep (mean 42.9 vs 38.3, p = 0.05) and attention disorders (mean 16.8 vs 11.6, p = 0.01), especially for excessive diurnal sleepiness (mean 3.9 vs 3.2, p = 0.02) and inattention (mean 9.3 vs 5.6, p = 0.003) components. In children with TAS, sleep problems were significantly under-reported by parents. Sleep disorders symptoms as breathing-related sleep disturbance, excessive diurnal sleepiness or naps at or after 7 years of age were reported. Subjective sleep and attention disorders were significantly correlated (r = 0.43, p = 0.01). Subjective excessive diurnal sleepiness may be the result of a polytherapy (p = 0.05) or a side effect of anti-seizure medication (ASM) (p = 0.03) but children without medication side effects also reported subjective SD. In children with TAS, the risk of inattention symptoms was increased in boys (p = 0.02), with a high BMI (p = 0.05), or with ASM side effects (p = 0.03). CONCLUSIONS: This study demonstrates that children with TAS are at risk of sleep and attention disorder symptoms. If attention disorders in a context of epilepsy are now widely assessed and identified, sleep disorders are still under-estimated. An accurate identification and management of sleep disorders could improve academic performances, quality of life, and seizure management in children with TAS.

Pulse transit time as a diagnostic test for OSA in children with Down syndrome.

STUDY OBJECTIVES: Children with Down syndrome (DS) are at risk of obstructive sleep apnea (OSA), but the access to sleep lab polysomnography (PSG) is limited. Simplified techniques are needed, such as polygraphy coupled with pulse transit time (PTT-PG) that detects respiratory events and the total autonomic arousals index (PTTAI). Our objective was to assess the ability of PTT-PG compared with PSG to diagnose OSA in children with DS. METHODS: In this prospective multicenter study, patients with DS underwent a full-night PSG coupled with PTT. Sleep questionnaires (Sleep Disturbance Scale for Children and Pediatric Sleep Questionnaire) were filled by parents. PSG and PTT-PG results were compared to test their sensibility and specificity to diagnose OSA. RESULTS: A total of 53 patients with DS were included; their median age was 9.3 years. An obstructive apnea-hypopnea index (OAHI) by PSG > 1 event/h was found in 36 (68%) patients, OAHI was > 1 and < 5 events/h in 18 patients (34%), ≥ 5 and < 10 events/h in 11 patients (21%), and ≥ 10 events/h in 7 patients (13%). OAHI was larger on PSG than on PTT-PG (P = .0005). For OSA diagnosis, the sensitivity was excellent for OAHI by PTT-PG if the added total PTTAI was > 1 event/h (1.0) and the specificity was high for the Pediatric Sleep Questionnaire (0.88) and OAHI > 1 event/h on PTT-PG (1.0). CONCLUSIONS: More than two-thirds of children with DS referred for screening by a genetics specialist had OSA diagnosed by PSG. With its excellent sensitivity and specificity, PTT-PG could be a good and simplified alternative to PSG to diagnose OSA in children with DS. CITATION: Ioan I, Weick D, Sevin F, et al. Pulse transit time as a diagnostic test for OSA in children with Down syndrome. J Clin Sleep Med. 2022;18(1):119-128.

Intellectual Abilities of Children with Narcolepsy.

High cognitive functioning could be a protective factor for school difficulties, behavioral and mood impairments in children with narcolepsy. To investigate this factor, we studied the intellectual abilities of 74 children with narcolepsy (43 boys, 11.7 years old at diagnosis, 91% of cataplexies, 64% obese, 100% HLA positive for DR-DQB1*06:02). All children underwent a one-night polysomnography followed by Multiple Sleep Latency Tests, an evaluation of intelligence quotient (IQ), and filled standardized questionnaires. Thirty-eight percent had high potentialities (HP defined by IQ > 130) and 48% had school difficulties. Using non-parametric tests, we found that HP children reported less difficulties at school and tended to have less impulsivity, conduct, and learning disorders than those without HP. They also tended to be less obese and had less desaturation. Using a multivariate regression analysis, we found an association between the REM sleep percentage and the IQ. REM sleep could be involved in the dynamic changes contributing to the equilibrium of intellectual functioning. This study highlights that despite their frequent school difficulties, narcolepsy per se is unlikely to be a cause of intellectual disability in children. Prompt diagnosis and management of comorbidities such as obesity and obstructive sleep apnea (OSA) could improve cognitive and school performances in these children.

Sleep of Children with High Potentialities: A Polysomnographic Study.

The involvement of sleep in cognitive functioning is well known, but only a few studies have examined objective sleep parameters in children with high intellectual potential (HP). The main objective of this study was to compare sleep characteristics of 33 children with high intellectual potentialities (HP) (median 10 years old, 64% of boys) compared to 25 controls (median 11 years old, 64% of boys) and assess the difference between children with a homogeneous vs. a heterogeneous intelligence quotient (IQ) (i.e., a difference ≥15 points between verbal and non-verbal IQ). All children underwent a one-night polysomnography, an evaluation of intellectual quotient (IQ) and filled standardized questionnaires. Using non-parametric tests to compare groups' characteristics, we found that children with HP had more heterogeneous IQ, more rapid eyes movement (REM) sleep and tended to have less stage 1 sleep than controls. They also had more insomnia and sleep complaints. The high amount of REM sleep in children with HP could be advantageous for learning and could partially explain their gift. This study highlights the necessity of investigating sleep disorders in children with HP during clinical routine and reinforces the hypothesis of the involvement of nocturnal sleep, and especially REM sleep, in daytime cognition and behavior.

Narcolepsy with cataplexy: Does age at diagnosis change the clinical picture?

OBJECTIVE: To compare symptoms and sleep characteristics in patients diagnosed with narcolepsy-cataplexy (NC) before and after the age of 18 years. METHODS: De novo patients with NC diagnosis completed a standardized questionnaire and interview, followed by a sleep study. The clinical and sleep measures were compared between patients diagnosed before (46 children, median age: 12 year old) and after (46 adults, median age: 28.5 year old) 18 years of age. RESULTS: The frequency of obesity (54% vs 17%), night eating (29% vs 7%), parasomnia (89% vs 43%), sleep talking (80% vs 34%), and sleep drunkenness (69% vs 24%) were higher in children than in adults, the frequency of sleep paralysis was lower (20% vs 55%) but the frequency of cataplexy and the severity of sleepiness were not different. Children scored higher than adults at the attention-deficit/hyperactivity disorder (ADHD) scale. Depressive feelings affected not differently children (24%) and adults (32%). However, adults had lower quality of life than children. There was no difference between groups for insomnia and fatigue scores. Quality of life was essentially impacted by depressive feelings in both children and adults. Obstructive apnea-hypopnea index (OAHI) was lower in children with higher mean and minimal oxygen saturation than in adults. No between-group differences were found at the multiple sleep latency test. The body mass index (z-score) was correlated with OAHI (r = .32). CONCLUSION: At time of NC diagnosis, children have more frequent obesity, night eating, parasomnia, sleep talking, drunkenness, and ADHD symptoms than adults, even if sleepiness and cataplexy do not differ. These differences should be considered to ensure a prompt diagnosis.

BLAST paradigm: A new test to assess brief attentional fluctuations in children with epilepsy, ADHD, and normally developing children.

BACKGROUND: Pure attentional deficits are still underdiagnosed in children with epilepsy. While attention-deficit hyperactivity disorder (ADHD) is historically the most studied cause of attentional disorders, an important number of children with epilepsy and attentional complaints do not fully meet the DSM-V (Diagnostic and Statistical Manual of Mental Disorders - Fifth Edition) criteria for ADHD and may be excluded from specific care. Clinical tools currently available are insufficient to detect more subtle but clinically relevant attentional fluctuations. OBJECTIVE/METHODS: The recently developed Bron-Lyon Attention Stability Test (BLAST) was used to evaluate brief attentional fluctuations with a high temporal precision. Drawing on two new attentional indices, we evaluated spontaneous fluctuations of response accuracy and timing, underlying attentional stability. The main objective was to assess attentional stability in children with i) epilepsy with comorbid ADHD, ii) epilepsy without comorbid ADHD, iii) ADHD not medicated and without epilepsy, and iv) normal development. Further objectives were to assess the main determinants of attentional stability in those groups, including the effect of factors related to the epileptic condition. RESULTS: In 122 children with epilepsy (67 with comorbid ADHD), 52 children with ADHD, and 53 healthy controls, we demonstrated lower attentional stability in both the groups with epilepsy and ADHD compared with healthy children. In children with epilepsy, BLAST scores were negatively associated with earlier seizure onset and AED (antiepileptic drug) polytherapy, while the seizure frequency, epilepsy duration, or type did not influence BLAST scores. CONCLUSIONS: This study demonstrates that attentional stability is impaired in children with epilepsy and/or ADHD. Bron-Lyon Attention Stability Test seems to be a sensitive test to detect attentional stability deficit in children with epilepsy and with attentional complaints who did not meet all criteria of ADHD. We propose that BLAST could be a useful clinical neuropsychological tool to assess attentional disorders in children.