Self-reported symptoms and objective measures in idiopathic hypersomnia and hypersomnia associated with psychiatric disorders: a prospective cross-sectional study.

STUDY OBJECTIVES: In some patients, it is difficult to correctly nosologically classify daytime sleepiness. Clinical manifestations may be nonspecific; on the basis of objective measures it is possible to determine the current severity of sleepiness, but they do not always allow accurate diagnosis. It is especially difficult to distinguish between idiopathic hypersomnia (IH) and hypersomnia associated with a psychiatric disorder (PSY). METHODS: To find significant differences between the IH and PSY groups, we included 67 patients (IH, n = 15; PSY, n = 52) in the study, focusing on differences in self-reported symptoms, evaluating current depressive symptoms using the Beck Depression Inventory-II score and personality traits measured by the Temperament and Character Inventory. All of the patients underwent polysomnography, the Multiple Sleep Latency Test, and ad libitum sleep monitoring. RESULTS: The patients with IH showed greater difficulty than those in the PSY group with waking up in the morning (P < .001) and complained of memory (P = .04) and attention deficit (P = .006). They also showed higher total sleep time (P < .001) and sleep efficiency (P = .007) and a shorter mean sleep latency on the Multiple Sleep Latency Test (P < .001). Nevertheless, the IH and PSY groups did not differ in Beck Depression Inventory scores or personality characteristics. CONCLUSIONS: IH is a syndrome in which depression/external life stressors and personality characteristics also play a role. Patients with IH may benefit from the cooperation of sleep specialists with psychotherapists/psychiatrists. CITATION: Busková J, Novák T, Miletínová E, et al. Self-reported symptoms and objective measures in idiopathic hypersomnia and hypersomnia associated with psychiatric disorders: a prospective cross-sectional study. J Clin Sleep Med. 2022;18(3):713-720.

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.

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.

Associations Between Neuropsychological, Neurobehavioral and Emotional Functioning and Either Narcolepsy or Idiopathic Hypersomnia in Children and Adolescents.

STUDY OBJECTIVES: Narcolepsy and idiopathic hypersomnia are chronic neurological sleep disorders characterized by hypersomnolence or excessive daytime sleepiness. This review aims to systematically examine the scientific literature on the associations between narcolepsy and idiopathic hypersomnia and their effect on intellectual functioning, academic achievement, behavior, and emotion. METHODS: Published studies that examined those associations in children and adolescents were included. Studies in which children or adolescents received a clinical diagnosis, and in which the associated function was measured with at least one objective instrument were included. Twenty studies published between 1968 and 2017 were eligible for inclusion in this review. RESULTS: There does not appear to be a clear association between intellectual functioning and narcolepsy or idiopathic hypersomnia; however, limited research is an obstacle to obtaining generalizability. The variability in results from studies investigating associations between academic achievement and these two hypersomnolence disorders suggests that further research using standardized and validated assessment instruments is required to determine if there is an association. Behavior and emotion appear to be significantly affected by narcolepsy. Only two studies included populations of children and adolescents with idiopathic hypersomnia. CONCLUSIONS: Further research using larger populations of children and adolescents with narcolepsy or idiopathic hypersomnia while utilizing standardized and validated instruments is required, because the effect of these conditions of hypersomnolence varies and is significant for each individual.

Hypersomnolence, Hypersomnia, and Mood Disorders.

Relationships between symptoms of hypersomnolence, psychiatric disorders, and hypersomnia disorders (i.e., narcolepsy and idiopathic hypersomnia) are complex and multidirectional. Hypersomnolence is a common complaint across mood disorders; however, patients suffering from mood disorders and hypersomnolence rarely have objective daytime sleepiness, as assessed by the current gold standard test, the Multiple Sleep Latency Test. An iatrogenic origin of symptoms of hypersomnolence, and sleep apnea syndrome must be considered in a population of psychiatric patients, often overweight and treated with sedative drugs. On the other hand, psychiatric comorbidities, especially depression symptoms, are often reported in patients with hypersomnia disorders, and an endogenous origin cannot be ruled out. A great challenge for sleep specialists and psychiatrists is to differentiate psychiatric hypersomnolence and a central hypersomnia disorder with comorbid psychiatric symptoms. The current diagnostic tools seem to be limited in that condition, and further research in that field is warranted.

Evaluation of the Effect of Modafinil on Cognitive Functions in Patients with Idiopathic Hypersomnia with P300.

BACKGROUND: Modafinil is a well-tolerated psychostimulant drug with low addictive potential that is used to treat patients with narcolepsy and other excessive sleepiness. Whereas favorable effects of modafinil on cognitive functions have been shown in a large number of studies, there are very few reports presenting the effects of modafinil electrophysiologically. The aim of this study was to investigate the effects of modafinil on auditory P300 latency and amplitude electrophysiologically. MATERIAL AND METHODS: Eighteen patients (age range: 16-48 years) with a diagnosis of idiopathic hypersomnia (IH) were included in the present study. As a standard treatment, 200 mg/day modafinil was administered to each patient. The P300 auditory test was performed for each patient before and at the end of 1 week of modafinil treatment. RESULTS: After 1 week of modafinil treatment, mean P300 latencies (at all electrode sites) were significantly lower than the latencies before the treatment (P values for Fz, Cz and Pz recording sites were 0.039, 0.002, and 0.004, respectively). An increase in the P300 amplitudes was detected only at the Fz recording site, but not at Cz or Pz recording sites (P values for Fz, Cz, and Pz recording sites were 0.014, 0.100, and 0.05, respectively). CONCLUSIONS: One week of modafinil treatment improved the cognitive performance, alertness, and executive functions in IH patients. Our electrophysiologically obtained findings provide further confirmation for previous reports in which modafinil has been shown to exert favorable effects on cognitive performance, alertness, and executive functions.

Are dysfunctional attitudes and beliefs about sleep unique to primary insomnia?

OBJECTIVE: Dysfunctional thinking about sleep is a central aspect in the perpetuation of primary insomnia and a target symptom of cognitive behavioral therapy for insomnia (CBT-I). Insomnia symptoms also occur in other sleep disorders, but it is not known to what extent it is related to dysfunctional thinking about sleep. METHODS: The Dysfunctional Beliefs and Attitudes about Sleep Scale (DBAS) was administered to inpatients at a sleep center. The following groups were included: 34 patients with primary insomnia (PI), 30 patients with sleep apnea syndrome (SAS), 31 patients with restless legs syndrome (RLS), 26 patients with SAS comorbid with RLS (SAS + RLS), and 24 patients with idiopathic hypersomnia or narcolepsy. Eighty-four healthy subjects served as a control group. The DBAS scores were compared across the different sleep disorders and correlated with polysomnographic (PSG) variables, subjective sleep parameters, scores of the Beck Depression Inventory (BDI), and the Regensburg Insomnia Scale (RIS; measuring psychological symptoms of insomnia). RESULTS: Compared to healthy controls, DBAS scores were increased in PI, RLS and RLS + SAS. There was a low correlation between DBAS scores and PSG variables, moderate correlations between DBAS and subjective sleep parameters and BDI scores (r = 0.528), and a high correlation between DBAS and the RIS score (r = 0.603). CONCLUSION: The observation of increased DBAS scores in other sleep disorders besides primary insomnia underscores the usefulness of a broadened diagnostic procedure and suggests that CBT-I modules may be a complementary treatment tool for these disorders.

Quality of life in patients with narcolepsy with cataplexy, narcolepsy without cataplexy, and idiopathic hypersomnia without long sleep time: comparison between patients on psychostimulants, drug-naïve patients and the general Japanese population.

OBJECTIVE: To assess the quality of life of patients with narcolepsy with cataplexy (NA-CA), narcolepsy without cataplexy (NA w/o CA), and idiopathic hypersomnia without long sleep time (IHS w/o LST) who were taking psychostimulant medication, and to ascertain which factors (including psychosocial and environmental variables) influence quality of life in this population. METHODS: In total, 185 patients who had received regular treatment were enrolled in the study (NA-CA, n=83; NA w/o CA, n=48; IHS w/o LST, n=54). Patients were asked to complete questionnaires including the Short Form-36 Health Survey (SF-36), the Epworth Sleepiness Scale (ESS), and items concerning psychosocial and environmental variables. RESULTS: All three diagnostic groups had significantly lower scores for most SF-36 domains compared with the Japanese normative data, and the ESS score was significantly reduced with treatment. Multiple logistic regression analyses revealed that several SF-36 domains were associated with the ESS score; autonomy in controlling own job schedule, experience of divorce or break up with a partner due to symptoms, experience of being forced to relocate or being dismissed due to symptoms, and perception of support from others. CONCLUSIONS: The severity of subjective sleepiness and psychological and environmental variables influenced quality of life in patients with these hypersomnias of central origin.

Subjective symptoms in idiopathic hypersomnia: beyond excessive sleepiness.

Patients with idiopathic hypersomnia never feel fully alert despite a normal or long sleep night. The spectrum of the symptoms is insufficiently studied. We interviewed 62 consecutive patients with idiopathic hypersomnia (with a mean sleep latency lower than 8 min or a sleep time longer than 11 h) and 50 healthy controls using a questionnaire on sleep, awakening, sleepiness, alertness and cognitive, psychological and functional problems during daily life conditions. Patients slept 3 h more on weekends, holidays and in the sleep unit than on working days. In the morning, the patients needed somebody to wake them, or to be stressed, while routine, light, alarm clocks and motivation were inefficient. Three-quarters of the patients did not feel refreshed after short naps. During the daytime, their alertness was modulated by the same external conditions as controls, but they felt more sedated in darkness, in a quiet environment, when listening to music or conversation. Being hyperactive helped them more than controls to resist sleepiness. They were more frequently evening-type and more alert in the evening than in the morning. The patients were able to focus only for 1 h (versus 4 h in the controls). They complained of attention and memory deficit. Half of them had problems regulating their body temperature and were near-sighted. Mental fatigability, dependence on other people for awakening them, and a reduced benefit from usually alerting conditions (except being hyperactive or stressed) seem to be more specific of the daily problems of patients with idiopathic hypersomnia than daytime sleepiness.

Hypersomnia in children: interface with psychiatric disorders.

Patients being evaluated in child psychiatry clinics for behavior and mood disturbances frequently exhibit daytime sleepiness. Conversely, patients being evaluated for hypersomnia by sleep specialists may have depressed mood or hyperactive and aggressive behavior. The etiology of daytime sleepiness in children and adolescents is diverse and includes inadequate sleep hygiene, obstructive sleep apnea, delayed sleep phase syndrome, idiopathic hypersomnia, periodic hypersomnia, narcolepsy, and mood disorders per se. Treatment of a sleep disorder can have a favorable impact on alertness and quality of life. A high index of suspicion for sleep problems should be maintained in children and adolescents with psychiatric disorders.

Psychological health in central hypersomnias: the French Harmony study.

BACKGROUND: A large observational French study of central hypersomnia, including narcolepsy with cataplexy (C+), without cataplexy (C-) and idiopathic hypersomnia (IH), was conducted to clarify the relationships between the severity of the condition, psychological health and treatment response. METHODS: 601 consecutive patients over 15 years of age suffering from central hypersomnia were recruited on excessive daytime sleepiness, polysomnography and Multiple Sleep Latency Test (MSLT) results. 517 (47.6% men, 52.4% women) were finally included: 82.0% C+, 13.2% C- and 4.8% IH. Face to face standardised clinical interviews plus questionnaires (Epworth Sleepiness Scale (ESS), short version Beck Depression Inventory (S-BDI), Pittsburgh Sleep Quality Index (PSQI) and 36-item Short Form Health Survey (SF-36)) were performed. Patients affected with a different diagnosis and with and without depressive symptoms were compared. RESULTS: Mean ESS and body mass index were higher in C+ compared with C-/IH patients. Half of the patients (44.9%) had no depressive symptoms while 26.3% had mild, 23.2% moderate and 5.6% severe depressive symptoms. C+ patients had higher S-BDI and PSQI and lower SF-36 scores than C-/IH patients. Depressed patients had higher ESS scores than non-depressed patients, with no difference in age, gender, duration of disease or MSLT parameters. Finally, C+ patients treated with anticataplectic drugs (38.7%) had higher S-BDI and lower SF-36 scores than C+ patients treated with stimulants alone. CONCLUSION: Our data confirmed the high frequency of depressive symptoms and the major impact of central hypersomnias on health related quality of life, especially in patients with cataplexy. We recommend a more thorough assessment of mood impairment in central hypersomnias, especially in narcolepsy-cataplexy.

Development of a Japanese version of the Epworth Sleepiness Scale (JESS) based on item response theory.

BACKGROUND: Various Japanese versions of the Epworth Sleepiness Scale (ESS) have been used, but none was developed via standard procedures. Here we report on the construction and testing of the developer-authorized Japanese version of the ESS (JESS). METHODS: Developing the JESS involved translations, back translations, a pilot study, and psychometric testing. We identified questions in the ESS that were difficult to answer or were inappropriate in Japan, proposed possible replacements for those questions, and tested them with analyses based on item response theory (IRT) and classical test theory. The subjects were healthy people and patients with narcolepsy, idiopathic hypersomnia, or obstructive sleep apnea syndrome. RESULTS: We identified two of our proposed questions as appropriate replacements for two problematic questions in the ESS. The JESS had very few missing data. Internal consistency reliability and test-retest reliability were high. The patients had significantly higher JESS scores than did the healthy people, and higher JESS scores were associated with worse daytime function, as measured with the Pittsburgh Sleep Quality Index. CONCLUSIONS: In Japan, the JESS provides reliable and valid information on daytime sleepiness. Researchers who use the ESS with other populations should combine their knowledge of local conditions with the results of psychometric tests.

Possible confusion between primary hypersomnia and adult attention-deficit/hyperactivity disorder.

We explored the possibility of diagnostic confusion between hypersomnias of central origin (narcolepsy and idiopathic hypersomnia, IH) and the adult form of attention-deficit/hyperactivity disorder (ADHD). We included 67 patients with narcolepsy, 7 with IH and 61 with ADHD. All patients completed the Epworth Sleepiness Scale and the ADHD Rating Scale. We found that 18.9% of the hypersomnia patients fulfilled the self-reported criteria for ADHD in adulthood, compared with 77% of the ADHD patients. A score > or =12 on the Epworth Sleepiness Scale (usually regarded to indicate excessive daytime sleepiness) was found in 37.7% of the ADHD patients compared 95.9% of the hypersomnia patients. In ADHD patients, inattention scores correlated with the excessive daytime sleepiness score. We conclude that one should be aware of possible diagnostic confusion between narcolepsy or IH and adult ADHD when using self-report questionnaires. The high percentage of symptom overlap found in our study raises questions about possible misdiagnosing of both conditions, comorbidity with sleep problems in adult ADHD, and the validation of the used scales. It remains unclear whether our findings indicate pathophysiological overlap.