[Assessments of sleepiness in adolescents: A key tool for better intervene in mental health]. / Évaluer la somnolence diurne auprès des adolescents : un incontournable pour mieux intervenir en santé mentale.

Objectives Daytime sleepiness in adolescents has negative impacts on physical, cognitive, and emotional health, with direct or indirect consequences on their mental health. This review aims to describe specialized tools assessing daytime sleepiness in adolescents so that mental health professionals can screen for a variety of sleep disorders, from the rarest ones, such as narcolepsy, to the most common ones, such as sleep-wake cycle delay in adolescents. Method Articles were selected in Medline (https://pubmed.ncbi.nlm.nih.gov/) and targeted adolescents aged between 13 and 18 or the keyword "adolescent*". The keywords used were: "sleepiness test" AND "questionnaire*". Only articles in French or English and published until January 9, 2023 were included. A total of 277 scientific articles were screened. Final sample included a total of 35 articles describing sleepiness measurement tools in adolescents. Results Among the 35 articles, a total of seven daytime sleepiness measurement tools in adolescents were identified. Four of them were subjective: 1) the Epworth Sleepiness Scale for Children and Adolescents (ESS-CHAD), 2) the Pediatric Daytime Sleepiness Scale (PDSS), 3) the Cleveland Adolescent Sleepiness Questionnaire (CASQ) and 4) the French Sleepiness Scale for Adolescents (FSSA). These self-reported questionnaires are less expensive and they can be used easily by mental health professionals as opposed to objective tools. Three objective tools have been identified: 1) the multiple sleep latency test (MSLT), 2) the maintenance of wakefulness test or called the "Maintenance Wakefulness Test" (MWT) and 3) the pupillographic sleepiness test (PST). Conclusion Given that adolescents end-of the-day sleep pressure, often resulting in a greater opportunity to light exposure, they are more at risk for daytime sleepiness and consequently to mental health challenges. Mental health professionals should therefore systematically screen for daytime sleepiness in adolescents using subjective tools. There are reliable and validated tools that are translated into French, such as the FSSA and the ESS-CHAD to measure daytime sleepiness in adolescents and lifestyles problems associated with sleep loss When daytime sleepiness suggests the presence of medical-based sleep disorders, such as narcolepsy, restless sleep disorders or sleep apnea, it is important to pursue an investigation with objective tools (nocturnal polysomnography, MLST and MWT) in collaboration with the adolescent's physician.

Dream-Reality confusion: differential psychiatric diagnosis in narcoleptic subjects / Confusão sonho-realidade: diagnóstico psiquiátrico diferencial em narcolépticos

Dream-reality confusion (DRC) is the consequence of hypnagogic content confusion with real events and memories. Narcoleptic subjects eventually have DRC and can be misdiagnosed as schizophrenic or with another disorder with delusional or hallucinatory symptoms. Although dream-related experiences and hallucinatory perception share neurophysiological pathways, they are phenomenologically distinct. The lack of phenomenological intentionality in Dreamrelated perceptions, the different cognitive pathways for delusion generation, and other differences between mental disorders psychopathology, and DRC-related phenomena are here discussed. The lived world and awake experience interpretation, and dream neurobiology in narcoleptic subjects related to DRC, might indicate some hints for the mind-brain gap issue that still exists in neurology and psychiatry.

A confusão entre realidade e sonho (CRS) é a consequência da confusão do conteúdo hipnagógico com eventos e memórias reais. Sujeitos narcolépticos eventualmente têm CRS e podem ser diagnosticados erroneamente como esquizofrênicos ou com outro transtorno com sintomas delirantes ou alucinatórios. Embora as experiências relacionadas ao sonho e à percepção alucinatória compartilhem vias neurofisiológicas, elas são fenomenologicamente distintas. A falta de intencionalidade fenomenológica nas percepções relacionadas ao sonho, as diferentes vias cognitivas para a geração do delírio e outras diferenças entre a psicopatologia dos transtornos mentais e os fenômenos relacionados à CRS são discutidos aqui. A interpretação do mundo vivido e da experiência de vigília, e a neurobiologia dos sonhos em sujeitos narcolépticos relacionados à CRS, podem indicar algumas dicas para a questão do gap mente-cérebro que ainda existe na neurologia e na psiquiatria.

[Dream content in different sleep disorders: sleep apnoea and hypopnoea syndrome, primary insomnia, idiopathic REM sleep behaviour disorder and narcolepsy type 1]. / Contenido onírico en diferentes trastornos del sueño: síndrome de apnea e hipopnea del sueño, insomnio primario, trastorno de la conducta del sueño REM idiopático y narcolepsia de tipo 1.

AIM: The aim of this study is to determine whether there are any differences in the dream content in different sleep disorders and to describe their characteristics. PATIENTS AND METHODS: We studied four sleep disorders: sleep apnoea and hypopnoea syndrome (SAHS), primary insomnia (PI), idiopathic REM sleep behaviour disorder (IRBD) and narcolepsy type I. Each patient was asked to keep a dream diary for two weeks. The content of the diaries was transcribed and analysed for length, mental content, complexity and threat. The results were compared to establish differences. RESULTS: Eighty-nine patients were studied: 23 with SAHS without continuous positive airway pressure (CPAP) who had the highest number of dreams involving threats (32.5%); 19 with SAHS treated with CPAP who had the highest number of dreams involving objects (64.8%), descriptive elements (38%) and higher complexity (9.5%); 22 with primary insomnia who had the highest number of dreams with threatening events in the social sphere (57.7%); 12 with IRBD who had the highest number of dreams with failures (14%) and lower complexity (71.7%); and 13 with narcolepsy type I who had the highest number of dreams related to activities (84.3%) and threats to life (41.4%) These differences were statistically significant (p <0.05). CONCLUSIONS: Different sleep disorders are associated with different dream contents, which would be translating different underlying neurological processes. These findings should be replicated in studies that analyse more patients and add a control group without sleep disorders.

TITLE: Contenido onírico en diferentes trastornos del sueño: síndrome de apnea e hipopnea del sueño, insomnio primario, trastorno de la conducta del sueño REM idiopático y narcolepsia de tipo 1.Objetivo. Determinar si existen diferencias en el contenido onírico en diferentes trastornos del sueño y describir sus características. Pacientes y métodos. Estudiamos cuatro trastornos del sueño: síndrome de apnea e hipopnea del sueño (SAHS), insomnio primario (IP), trastorno de conducta del sueño REM idiopático (TCSRI) y narcolepsia de tipo 1. Se solicitó a cada paciente que llenara un diario de sus sueños durante dos semanas. El contenido de los diarios fue transcrito y analizado en longitud, contenido mental, complejidad y amenaza. Los resultados se compararon para establecer diferencias. Resultados. Se estudió a 89 pacientes: 23 con SAHS sin presión positiva continua de la vía aérea (CPAP) que tuvieron la mayor cantidad de sueños con participación en amenazas (32,5%); 19 con SAHS tratados con CPAP que tuvieron la mayor cantidad de sueños con objetos (64,8%), elementos descriptivos (38%) y de más alta complejidad (9,5%); 22 con insomnio primario con la mayor cantidad de sueños con eventos amenazantes al ámbito social (57,7%); 12 con TCSRI que tuvieron en sus sueños la más alta cantidad de fracasos (14%) y menor complejidad (71,7%), y 13 con narcolepsia de tipo 1 que tuvieron la mayor cantidad de sueños relacionados con actividades (84,3%) y amenazas hacia la vida (41,4 %). Estas diferencias fueron estadísticamente significativas (p menor de 0,05). Conclusiones. Los distintos trastornos del sueño sí se asocian a contenidos oníricos diferentes que traducirían distintos procesos neurológicos subyacentes. Estos hallazgos deberían replicarse en estudios que analicen más pacientes y añadan un grupo control sin trastornos del sueño.

A practical guide to the pharmacological and behavioral therapy of Narcolepsy.

Narcolepsy is a rare, chronic, and disabling central nervous system hypersomnia; two forms can be recognized: narcolepsy type 1 (NT1) and narcolepsy type 2 (NT2). Its etiology is still largely unknown, but studies have reported a strong association between NT1 and HLA, as well as a pathogenic association with the deficiency of cerebrospinal hypocretin-1. Thus, the most reliable pathogenic hypothesis is an autoimmune process destroying hypothalamic hypocretin-producing cells. A definitive cure for narcolepsy is not available to date, and although the research in the field is highly promising, up to now, current treatments have aimed to reduce the symptoms by means of different pharmacological approaches. Moreover, overall narcolepsy symptoms management can also benefit from non-pharmacological approaches such as cognitive behavioral therapies (CBTs) and psychosocial interventions to improve the patients' quality of life in both adult and pediatric-affected individuals as well as the well-being of their families. In this review, we summarize the available therapeutic options for narcolepsy, including the pharmacological, behavioral, and psychosocial interventions.

Sexual excitation induces courtship ultrasonic vocalizations and cataplexy-like behavior in orexin neuron-ablated male mice.

Cataplexy is triggered by laughter in humans and palatable food in mice. To further evaluate mice's cataplexy, we examined courtship behavior in orexin neuron-ablated mice (ORX-AB), one of the animal models of narcolepsy/cataplexy. Wild-type female mice were placed into the home cage of male ORX-AB and cataplexy-like behavior was observed along with ultrasonic vocalizations (USVs), also known as the "love song". ORX-AB with a female encounter showed cataplexy-like behavior both during the dark and light periods, whereas ORX-AB with chocolate predominantly showed it during the dark period. During the light period observation, more than 85% of cataplexy-like bouts were preceded by USVs. A strong positive correlation was observed between the number of USVs and cataplexy-like bouts. Cataplexy-like behavior in narcoleptic mice is a good behavioral measure to study the brain mechanisms behind positive emotion because they can be induced by different kinds of positive stimuli, including chocolate and female courtship.

How Does Narcolepsy Impact Health-Related Quality of Life? A Mixed-Methods Study.

Study Objectives: The purpose of this study was to identify patient-centered issues affecting Health-Related Quality of Life (HRQoL) in people with narcolepsy (PWN) and to evaluate patient-reported outcome measures using a mixed-methods approach. Methods: Twenty-nine adults (93% female, mean age = 31 years) with an established diagnosis of narcolepsy (Type I = 58.6%) completed focus group interviews using live videoconferencing. Additionally, participants completed the Patient-Reported Outcomes Measurement Information System (PROMIS) measures along with legacy measures commonly used in narcolepsy research (Epworth Sleepiness Scale, Patient Health Questionnaire, Short-Form 36). Results: Thematic analysis of qualitative data revealed that HRQoL was impacted by the constancy of sleepiness, unpredictability of narcolepsy symptoms, and negative public perception of narcolepsy. Challenges to accessibility and/or quality of care included dissatisfaction with non-sleep specialists' understanding of narcolepsy, the unpredictability of symptoms, and the cost of health care. There was enthusiasm for developing a psychosocial intervention to improve HRQoL using online access, but there were mixed opinions regarding the format, provider background, and content of the intervention. Elevations (T-score > 60) were found on PROMIS measures of depression, anxiety, fatigue, and sleep impairment. These patterns were consistent with the levels reported on legacy measures. PWN Type I reported lower levels of general health relative to Type II (p < .05). Conclusions: These findings lay the groundwork for more targeted efforts to address areas of diminished HRQoL in PWN. Additionally, PROMIS measures appear to be suitable and efficient instruments for assessing HRQoL in PWN.

Relationship between sleep efficacy endpoints and measures of functional status and health-related quality of life in participants with narcolepsy or obstructive sleep apnea treated for excessive daytime sleepiness.

This study examined the correlation between improvements in excessive daytime sleepiness in participants with obstructive sleep apnea or narcolepsy and changes in functional status, work productivity and health-related quality of life. Data from two 12-week randomized controlled trials of solriamfetol were analyzed. Participants completed the Epworth Sleepiness Scale, 10-item Functional Outcomes of Sleep Questionnaire, Work Productivity and Activity Impairment questionnaire and 36-Item Short Form Health Survey and performed the Maintenance of Wakefulness Test at baseline and weeks 4, 8 and 12. Patient Global Impression of Change was assessed at weeks 4, 8 and 12. Pearson correlations were calculated for change in scores from baseline to week 12. For both studies, changes in the 10-item Functional Outcomes of Sleep Questionnaire were highly correlated (absolute value >0.5) with changes in Epworth Sleepiness Scale scores; changes in multiple domain scores of the 36-Item Short Form Health Survey and Work Productivity and Activity Impairment questionnaire were moderately correlated (0.3-0.5) with changes in Epworth Sleepiness Scale scores in both studies and highly correlated for participants with narcolepsy. Changes in Maintenance of Wakefulness Test scores correlated moderately with changes in Epworth Sleepiness Scale scores in both studies. At week 12, Patient Global Impression of Change ratings correlated highly with Epworth Sleepiness Scale and 10-item Functional Outcomes of Sleep Questionnaire scores for both disorders. Other correlations were low. Self-reported assessments of sleepiness and global improvement appear to be more strongly correlated with measures of functioning and health-related quality of life than objectively assessed sleepiness.

Topical Review: A Biopsychosocial Framework for Pediatric Narcolepsy and Idiopathic Hypersomnia.

OBJECTIVE: Provide an overview of current research findings in pediatric central disorders of hypersomnolence (CDH) and propose a biopsychosocial model for clinical management, with a focus on interdisciplinary care and future directions for research and clinical practice. METHODS: Literature review drawing from pediatric and adult narcolepsy, as well as pediatric sleep and chronic pain research to develop an integrative biopsychosocial model for pediatric CDH. RESULTS: Youth with CDH are vulnerable to impairments in academics, emotional, and behavioral functioning, activity engagement and quality of life (QOL). There is a complex interrelationship between neurobiological features of disease, treatment-related factors, and psychological, sleep-related, and contextual factors across development. Research is limited largely to adults and pediatric narcolepsy type 1 and the mechanisms and evolution of morbidity remain poorly understood. CONCLUSIONS: In addition to first-line treatment (pharmacotherapy), routine screening of bio-behavioral and psychosocial functioning and QOL is needed to identify risk for compromised functioning warranting adjunctive interventions with behavioral health specialists.

Neuropsychological Alterations in Narcolepsy with Cataplexy and the Expression of Cognitive Deficits.

OBJECTIVES: The objective of our study was to assess attention processes and executive function in patients with narcolepsy with cataplexy (NT1). To do so, we compared the results with those of a control group from the general population using an extensive neuropsychological test battery. METHODS: We studied 28 patients with NT1 and 28 healthy control participants matched for age, gender, and educational level. They all completed questionnaires on sleepiness, anxiety, and depression symptoms. In addition, they underwent neuropsychological tests. The ability to maintain attention was assessed using three computer tasks with different levels of complexity. RESULTS: Patients had significantly more daytime sleepiness than controls. A significant negative correlation between depression and disease duration was found in NT1 patients. The results of the anxiety questionnaire correlated with the presence of sleep paralysis. There were significant differences in information processing speed subtasks. Patients made significantly more omissions and generally reacted slower and more variably than controls in computerized tasks. As for executive function, patients performed worse in phonologic fluency tasks than controls. However, when the influence of processing speed on fluency tasks was statistically controlled, part of this significant difference disappeared. CONCLUSIONS: Our results indicate that the negative correlation between depression and disease duration probably reflects progressive adaptation to the functional burden of the disease. Information processing speed plays a fundamental role in the expression of cognitive deficits. We emphasized the need to control the influence of processing speed and sustained attention in the neuropsychological assessment of NT1 patients.

Academic and professional paths of narcoleptic patients: the Narcowork study.

OBJECTIVE/BACKGROUND: To study educational and professional pathways of narcoleptic patients and examine demographic, disease-related and environmental factors associated with a better academic and professional prognosis. PATIENTS/METHODS: In sum, 69 narcoleptic patients (51 narcolepsy type 1 and 18 narcolepsy type 2, age 42.5 ± 18.2 years) were enrolled in this pilot monocentric cross-sectional study with a comparison group (80 age- and sex-matched controls) between October 2017 and July 2018 in Lyon Center for Sleep Medicine. They completed questionnaires about their academic and professional trajectories and specific scales of quality of life (EuroQol quality of life scale EQ-5D-3L), depression (beck depression inventory, BDI), sleepiness (Epworth Sleepiness Scale, ESS) and narcoleptic symptoms severity (narcolepsy severity scale, NSS). RESULTS: No difference in grade repetition or final obtained diploma was observed between patients and controls, but patients evaluated their academic curricula as more difficult (45.5% vs 16.9%, p = 0.0007), complained for more attentional deficits (75% vs 22.1%, p < 0.0001), and had needed more educational reorientation (28.6% vs 9.9%, p = 0.01). Even if no difference was observed in occupational category and professional status, patients expressed significantly less satisfaction about their work. Patients had more signs of depression [OR severe depression = 4.4 (1.6-12.6), p = 0.02] and their quality of life was significantly decreased (67.3 ± 18.4 vs 80.6 ± 13.2, p = 0.0007) as compared to controls. Multivariate analysis showed that a more favorable professional career was associated with a better quality of life. CONCLUSIONS: Educational and professional pathways do not seem to be significantly impaired in narcoleptic patients, but their experience and quality of life are affected. These findings may allow to reassure patients and should lead to a more comprehensive management of the disease. CLINICAL TRIAL REGISTRATION: Narcowork, https://clinicaltrials.gov/ct2/show/NCT03173378, N° NCT03173378.

[Psychogenically induced narcolepsy]. / Psikhogenno-indutsirovannaia narkolepsiia.

Based on own clinical experience in diagnostics and treatment of 4 patients, the authors describe main clinical presentations of narcolepsy. A case report of a 20-year female patient with psychogenic narcolepsy induced by a conflict in the family is described in details. According to polysomnography and Multiple Sleep Latency Test, a reduction in latency to sleep is 22 sec - 3 min 30 sec and the time of occurrence of REM sleep is 7 min 30 sec. The diagnosis is made on the basis of diagnostic criteria of narcolepsy. On average, it takes about 7-10 years to diagnose narcolepsy that reduces the quality of life of patients and leads to social and household maladaptation. Physicians should be wary of early identification of this disease for dynamic monitoring, the appointment of symptomatic therapy, prevention of anxiety and depression and the generation of behavior algorithm in the patient and his relatives in order to maximize social and household adaptation.

Pregnancy and Contraception Experiences in Women With Narcolepsy: A Narcolepsy Network Survey.

STUDY OBJECTIVES: To explore knowledge and experiences of women with narcolepsy on pregnancy and contraception issues and their relationships with narcolepsy pharmacotherapy. METHODS: An 18-item survey was administered through the Narcolepsy Network website for 8 weeks during the fall of 2012. The survey ascertained demographic information; prescription narcolepsy medication use and discontinuation during pregnancy; physician counseling regarding pregnancy, contraception, and medication usage; and pregnancy history and outcomes. Frequencies of responses were analyzed and compared between pharmacotherapy groups. RESULTS: Surveys from 182 women (age 41.5 ± 15.2 years) with narcolepsy were analyzed. Most of the respondents (78.7%) who reported a history of pregnancy did not use pharmacotherapy during pregnancy. Most of them discontinued narcolepsy pharmacotherapy during pregnancy because of their own fear of harming the fetus (82.9%), and 58.5% noted advice of discontinuation from their narcolepsy physician as a factor in their decision. As an alternative to pharmacotherapy, 72.1% of women extended their sleep time, 32.6% discontinued working, and 27.9% discontinued driving. Similar pregnancy and fetal outcomes were reported between women using monotherapy, polytherapy, or no therapy during pregnancy, but some outcomes were worse than national averages. In general, women with narcolepsy were dissatisfied with the amount and type of counseling that they received regarding pregnancy and contraception. CONCLUSIONS: Improved health education counseling and symptom management options are needed for women with narcolepsy to improve pregnancy management and outcomes in this population.

Clinical autonomic dysfunction in narcolepsy type 1.

STUDY OBJECTIVES: (1) To compare the presence of autonomic symptoms using the validated SCOPA-AUT questionnaire in untreated patients with narcolepsy type 1 (NT1) to healthy controls, (2) to study the determinants of a high total SCOPA-AUT score in NT1, and (3) to evaluate the effect of drug intake on SCOPA-AUT results in NT1. METHODS: The SCOPA-AUT questionnaire that evaluates gastrointestinal, urinary, cardiovascular, thermoregulatory, pupillomotor, and sexual dysfunction was completed by 92 consecutive drug-free adult NT1 patients (59 men, 39.1 ± 15.6 years old) and 109 healthy controls (63 men, 42.6 ± 18.2 years old). A subgroup of 59 NT1 patients completed the questionnaire a second time, under medication (delay between two evaluations: 1.28 ± 1.14 years). RESULTS: Compared to controls, NT1 patients were more frequently obese, had more dyslipidemia, with no difference for age and gender. The SCOPA-AUT score of NT1 was higher than in controls in crude and adjusted models. Patients experienced more problems than controls in all subdomains. A higher score in NT1 was associated with older age, longer disease duration, altered quality of life and more depressive symptoms, but not with orexin levels and disease severity. Among patients evaluated twice, the SCOPA-AUT score total did not differ according to treatment status, neither did each subdomain. CONCLUSION: We captured a frequent and large spectrum of clinical autonomic dysfunction in NT1, with impairment in all SCOPA-AUT domains, without key impact of medication intake. This assessment may allow physicians to screen and treat various symptoms, often not spontaneously reported but associated with poor quality of life.

Increased creative thinking in narcolepsy.

Some studies suggest a link between creativity and rapid eye movement sleep. Narcolepsy is characterized by falling asleep directly into rapid eye movement sleep, states of dissociated wakefulness and rapid eye movement sleep (cataplexy, hypnagogic hallucinations, sleep paralysis, rapid eye movement sleep behaviour disorder and lucid dreaming) and a high dream recall frequency. Lucid dreaming (the awareness of dreaming while dreaming) has been correlated with creativity. Given their life-long privileged access to rapid eye movement sleep and dreams, we hypothesized that subjects with narcolepsy may have developed high creative abilities. To test this assumption, 185 subjects with narcolepsy and 126 healthy controls were evaluated for their level of creativity with two questionnaires, the Test of Creative Profile and the Creativity Achievement Questionnaire. Creativity was also objectively tested in 30 controls and 30 subjects with narcolepsy using the Evaluation of Potential Creativity test battery, which measures divergent and convergent modes of creative thinking in the graphic and verbal domains, using concrete and abstract problems. Subjects with narcolepsy obtained higher scores than controls on the Test of Creative Profile (mean ± standard deviation: 58.9 ± 9.6 versus 55.1 ± 10, P = 0.001), in the three creative profiles (Innovative, Imaginative and Researcher) and on the Creative Achievement Questionnaire (10.4 ± 25.7 versus 6.4 ± 7.6, P = 0.047). They also performed better than controls on the objective test of creative performance (4.3 ± 1.5 versus 3.7 ± 1.4; P = 0.009). Most symptoms of narcolepsy (including sleepiness, hypnagogic hallucinations, sleep paralysis, lucid dreaming, and rapid eye movement sleep behaviour disorder, but not cataplexy) were associated with higher scores on the Test of Creative Profile. These results highlight a higher creative potential in subjects with narcolepsy and further support a role of rapid eye movement sleep in creativity.

Measurement of symptoms in idiopathic hypersomnia: The Idiopathic Hypersomnia Severity Scale.

OBJECTIVE: To validate the Idiopathic Hypersomnia Severity Scale (IIHSS), a self-report measure of hypersomnolence symptoms, consequences, and responsiveness to treatment. METHODS: The 14-item IHSS (developed and validated by sleep experts with patients' feedback) was filled in by 218 participants (2.3% missing data). Among the 210 participants who fully completed the IHSS, there were 57 untreated and 43 treated patients with idiopathic hypersomnia (IH) aged 16 years or older, 37 untreated patients with narcolepsy type 1 (NT1), and 73 controls without sleepiness. IHSS psychometric properties, discriminant diagnostic validity, and score changes with treatment were assessed. RESULTS: The IHSS showed good internal consistency and content validity. Factor analysis indicated a 2-component solution with good reliability expressed by satisfactory Cronbach α values. IHSS scores were reproducible without changes in the test-retest evaluation (13 treated and 14 untreated patients). Convergent validity analysis showed that IHSS score was correlated with daytime sleepiness, depressive symptoms, and quality of life in patients with IH. The IHSS score was lower in treated than untreated patients (5-8 unit difference, without ceiling effect). The cutoff value for discriminating between untreated and treated patients was 26/50 (sensitivity 55.8%, specificity 78.9%). IHSS scores were higher in drug-free IH patients than NT1 and controls. The best cutoff value to differentiate between untreated IH patients and controls was 22 (sensitivity 91.1%, specificity 94.5%), and 29 with NT1. CONCLUSIONS: The IHSS is a reliable and valid clinical tool for the quantification of IH symptoms and consequences that might be useful for patient identification, follow-up, and management.

Maintenance of Wakefulness Test, real and simulated driving in patients with narcolepsy/hypersomnia.

STUDY OBJECTIVES: To assess the relationship between real and simulated driving performance and the objective level of alertness as measured by the Maintenance of Wakefulness Test (MWT) in patients suffering from narcolepsy or idiopathic hypersomnia. METHODS: Twenty-seven patients (10 patients with narcolepsy, type 1 (n = 7) and type 2 (n = 3), and 17 patients with idiopathic hypersomnia, mean age = 33.8 ± 11.1 years, range = 18-65 y; four males) were recruited in a randomized, crossover, double-blind placebo-controlled trial, and compared to 27 matched healthy controls. Patients were randomly assigned to receive modafinil (400 mg) or placebo before the driving test (2 h of real and 2 h of simulated highway driving for each patient). Standard deviation of lateral position (SDLP) of the vehicle in real and simulated driving and mean sleep latency in a 4 × 40 min MWT were assessed. RESULTS: Untreated patients presented shorter sleep latencies on the MWT (20.8 (IQ range 16.1-32.9) vs. 34.9 min (IQ range 28.1-40.0)) and worse simulated driving performance (P < 0.001) than treated patients. Nevertheless, treated patients still exhibited shorter mean sleep latencies on the MWT than controls (34.9 (IQ range 28.1-40.0) vs. 40 min (IQ range 37.1-40.0), P < 0.05), but driving performance was identical in both groups. The SDLP of the vehicle in real driving conditions and the MWT score correlated with the SDLP in simulated driving (respectively, r = 0.34, P < 0.05 and r = -0.56, P < 0.001). CONCLUSIONS: In patients with narcolepsy/idiopathic hypersomnia, simulated driving and MWT explore different dimensions of fitness-to-drive and could be used complementarily to better evaluate sleep-related driving impairment.

A population-based and case-controlled study of children and adolescents with narcolepsy: Health-related quality of life, adaptive behavior and parental stress.

OBJECTIVE: To investigate health-related quality of life (HrQoL) and adaptive behavior in young people with narcolepsy and stress among their parents. METHODS: In a cross-sectional exploratory quantitative study design, 37 young people with narcolepsy (8-20 years of age) and their parents were recruited. Thirty-one had post-H1N1 vaccination-related narcolepsy (PHV) and six had narcolepsy not related to PHV (nPHV). In addition, 40 age- and gender-matched controls (aged 5-20 years) were recruited. RESULTS: Thirty-one patients completed the generic HrQoL questionnaire KIDSCREEN and the disease-specific NARQoL-21. HrQoL was found to be significantly diminished in all domains in the PHV group (p = 0.001) and in the School/Concentration domain (p = 0.004) in the nPHV group compared to age- and gender-matched controls. The Adaptive Behavior Assessment System was completed by parents of 32 patients. They rated their children significantly lower in the General adaptive composite (p = 0.026) and the Conceptual (p = 0.050) and Social composite scores (p = 0.001) compared with reference data on healthy Swedish children's and young people's adaptive behavior. Parents of 36 patients filled in the 36-item short form of the Parenting Stress Index questionnaire. They rated significantly higher Total stress, Parent-child dysfunctional interaction, and Difficult child scores compared with parents of controls (p = 0.001, p = 0.005, p < 0.001). CONCLUSIONS: Children with narcolepsy have diminished HrQoL compared with controls. Parents of children with narcolepsy experience impaired adaptive behavior in their children and high levels of parenting stress. Identifying the contributory factors is necessary, and early intervention is crucial in order to improve the HrQoL of these children and their families.

Psychiatric symptoms in patients with post-H1N1 narcolepsy type 1 in Norway.

STUDY OBJECTIVES: Several studies have reported psychiatric comorbidity in patients with narcolepsy type 1 (NC1). The primary aim of this study was to explore the extent of psychiatric symptoms in a cohort of Norwegian NC1 patients, most of whom were H1N1-vaccinated. We also wanted to explore possible causes of the psychiatric symptoms seen in NC1. METHODS: Cross-sectional study. Psychiatric symptoms were assessed by the Achenbach System of Empirically Based Assessment (ASEBA) Child Behavior Check List (CBCL) in children and by Adult Self Report (ASR) in adults. RESULTS: The mean (SD) total T-scores were 58.6 (9.2) for children and 57.0 (9.8) for adults, these being mainly driven by internalizing problems. Internalizing symptom T-scores showed that 37.5% of the children and 33.3% of the adults were in the clinical range of concern. T-scores were lower when the questionnaire's sleep-related items were excluded. However, 27.5% of children and 22.2% of adults still remained within the total psychiatric symptoms clinical range. Psychiatric symptoms and excessive daytime sleepiness were not associated. However, in children fragmented sleep, measured by sleep-stage shift index was significantly negatively associated with all the psychiatric summary scores (all p ≤ 0.020), and awakening index was negatively associated with externalizing (p = 0.042) and total summary scores (p = 0.042). In adults, awakening index, but not sleep-stage shift index, was positively associated with internalizing score (p = 0.015). Hypocretin-1 levels showed no association with psychiatric symptoms. CONCLUSIONS: We found a high prevalence of psychiatric symptoms in NC1 patients. Fragmented sleep was significantly associated with psychiatric symptoms.

Health-Related Quality of Life in Patients With Narcolepsy: A Review of the Literature.

Narcolepsy is a chronic disorder characterized by symptoms of excessive daytime sleepiness, irresistible sleep attacks that may be accompanied by cataplexy brought on by emotions, sleep paralysis, and hypnagogic hallucinations. This is a review of 32 empirical articles on health-related quality of life (HRQoL) published in peer-reviewed journals over the past 37 years. Deleterious implications on education, recreation, driving, sexual life, and personality are associated with the disease with a consequent negative psychosocial impact. Sleepiness has an important influence on HRQoL, more than the other symptoms of this disorder that have disrupting roles, too. Therefore, patients with narcolepsy need assistance not only for medication prescription but also in terms of psychological and social support. It is also of importance to assess patients with narcolepsy carefully in terms of depressive symptoms because they may have a major impact on HRQoL with important clinical implications.

Impaired social functioning in children with narcolepsy.

Study Objectives: To explore impairments in social functioning in children with narcolepsy compared to healthy children. Methods: Parents of 53 pediatric patients with narcolepsy type 1 and 64 matched healthy children completed the Social Responsiveness Scale (SRS) and the Child Behavior Checklist 6-18 (CBCL 6-18). Results: Patients scored significantly higher on the total score of the SRS (median 56, interquartile range [IQR] 23.5) compared to controls (median 44.5, IQR 8.5, U = 797.0, p < 0.001). Patients also scored higher on the sum of the CBCL 6-18 subscales indicative of social functioning (Withdrawn/Depressed, Social Problems, and Thought Problems; median 183, IQR 30.5) compared to controls (median 155, IQR 13, U = 500.0, p < 0.001). A total of 24 patients (45.3%) reported at least mild-to-moderate difficulties in social functioning compared to seven controls (10.9%, χ2 = 17.165, p < 0.001). Eleven patients (20.8%) and only one control (1.6%) had T scores above 75, which points to severely impaired social functioning (χ2 = 11.602, p = 0.001). Within the patient group, girls reported mild-to-moderate difficulties in social functioning significantly more often compared to boys on the SRS (77.8% versus 28.6%, χ2 = 17.560, p < 0.001). Conclusions: Impaired social functioning is common in children with narcolepsy type 1, especially in girls. Questionnaires such as the SRS and the CBCL 6-18 may help in early detection of social problems in pediatric narcolepsy. Recognition of these problems could be valuable in the management of young people with narcolepsy.