Little attention has been paid to the long-term development of idiopathic hypersomnia symptoms and idiopathic hypersomnia comorbidities. The aim of this study was to describe the general health of patients with idiopathic hypersomnia years after the initial diagnosis, focusing on current subjective hypersomnolence and the presence of its other possible causes. Adult patients diagnosed with idiopathic hypersomnia ≥ 3 years ago at sleep centres in Prague and Kosice were invited to participate in this study. A total of 60 patients were examined (age 47.3 ± SD = 13.2 years, 66.7% women). In all participants, their hypersomnolence could not be explained by any other cause but idiopathic hypersomnia at the time of diagnosis. The mean duration of follow-up was 9.8 + 8.0 years. Fifty patients (83%) reported persisting hypersomnolence, but only 33 (55%) had no other disease that could also explain the patient's excessive daytime sleepiness and/or prolonged sleep. In two patients (3%), the diagnosis in the meantime had changed to narcolepsy type 2, and 15 patients (25%) had developed a disease or diseases potentially causing hypersomnolence since the initial diagnosis. Complete hypersomnolence resolution without stimulant treatment lasting longer than 6 months was reported by 10 patients (17%). To conclude, in a longer interval from the diagnosis of idiopathic hypersomnia, hypersomnolence may disappear or may theoretically be explained by another newly developed disease, or the diagnosis may be changed to narcolepsy type 2. Thus, after 9.8 years, only 55% of the examined patients with idiopathic hypersomnia had a typical clinical picture of idiopathic hypersomnia without doubts about the cause of the current hypersomnolence.

• Klíčová slova
• comorbidity, depression, diagnosing, follow-up, hypersomnolence, idiopathic hypersomnia,
• MeSH
• dospělí MeSH
• idiopatická hypersomnie * diagnóza   epidemiologie   farmakoterapie   MeSH
• komorbidita MeSH
• lidé středního věku MeSH
• lidé MeSH
• narkolepsie * diagnóza   epidemiologie   MeSH
• poruchy nadměrné spavosti * diagnóza   epidemiologie   komplikace   MeSH
• pozornost MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

STUDY OBJECTIVES: Microbial antigens can elicit an immune response leading to the production of autoantibodies cross-reacting with autoantigens. Still, their clinical significance in human sera in the context of brain diseases is unclear. Therefore, assessment of natural autoantibodies reacting with their neuropeptides may elucidate the autoimmune etiology of central hypersomnias. The study aims to determine whether serum autoantibody levels differ in patients with different types of central hypersomnias (narcolepsy type 1 and 2, NT1 and NT2; idiopathic hypersomnia, IH) and healthy controls and if the differences could suggest the participation of autoantibodies in disease pathogenesis. METHODS: Sera from 91 patients with NT1, 27 with NT2, 46 with IH, and 50 healthy controls were examined for autoantibodies against assorted neuropeptides. Participants were screened using questionnaires related to sleep disorders, quality of life, and mental health conditions. In addition, serum biochemical parameters and biomarkers of microbial penetration through the intestinal wall were determined. RESULTS: A higher prevalence of autoantibodies against neuropeptides was observed only for alpha-melanocytes-stimulating hormone (α-MSH) and neuropeptide glutamic acid-isoleucine (NEI), which differed slightly among diagnoses. Patients with both types of narcolepsy exhibited signs of microbial translocation through the gut barrier. According to the questionnaires, patients diagnosed with NT2 or IH had subjectively worse life quality than patients with NT1. Patients displayed significantly lower levels of bilirubin and creatinine and slightly higher alkaline phosphatase values than healthy controls. CONCLUSIONS: Overall, serum anti-neuronal antibodies prevalence is rare, suggesting that their participation in the pathophysiology of concerned sleep disorders is insignificant. Moreover, their levels vary slightly between diagnoses indicating no major diagnostic significance.

• Klíčová slova
• Autoantibodies, Hypersomnia, Immunity, Narcolepsy, Neuropeptides, Sleepiness,
• MeSH
• autoprotilátky MeSH
• kvalita života MeSH
• lidé MeSH
• narkolepsie * epidemiologie   MeSH
• neuropeptidy * MeSH
• poruchy nadměrné spavosti * epidemiologie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• autoprotilátky MeSH
• neuropeptidy * MeSH

Increased incidence rates of narcolepsy type-1 (NT1) have been reported worldwide after the 2009-2010 H1N1 influenza pandemic (pH1N1). While some European countries found an association between the NT1 incidence increase and the H1N1 vaccination Pandemrix, reports from Asian countries suggested the H1N1 virus itself to be linked to the increased NT1 incidence. Using robust data-driven modeling approaches, that is, locally estimated scatterplot smoothing methods, we analyzed the number of de novo NT1 cases (n = 508) in the last two decades using the European Narcolepsy Network database. We confirmed the peak of NT1 incidence in 2010, that is, 2.54-fold (95% confidence interval [CI]: [2.11, 3.19]) increase in NT1 onset following 2009-2010 pH1N1. This peak in 2010 was found in both childhood NT1 (2.75-fold increase, 95% CI: [1.95, 4.69]) and adulthood NT1 (2.43-fold increase, 95% CI: [2.05, 2.97]). In addition, we identified a new peak in 2013 that is age-specific for children/adolescents (i.e. 2.09-fold increase, 95% CI: [1.52, 3.32]). Most of these children/adolescents were HLA DQB1*06:02 positive and showed a subacute disease onset consistent with an immune-mediated type of narcolepsy. The new 2013 incidence peak is likely not related to Pandemrix as it was not used after 2010. Our results suggest that the increased NT1 incidence after 2009-2010 pH1N1 is not unique and our study provides an opportunity to develop new hypotheses, for example, considering other (influenza) viruses or epidemiological events to further investigate the pathophysiology of immune-mediated narcolepsy.

• Klíčová slova
• H1N1 influenza, childhood narcolepsy, narcolepsy,
• MeSH
• chřipka lidská * epidemiologie   prevence a kontrola   MeSH
• dítě MeSH
• dospělí MeSH
• incidence MeSH
• lidé MeSH
• mladiství MeSH
• narkolepsie * epidemiologie   etiologie   MeSH
• vakcinace MeSH
• vakcíny proti chřipce * MeSH
• virus chřipky A, podtyp H1N1 * MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé MeSH
• mladiství MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Geografické názvy
• Asie MeSH
• Evropa MeSH
• Názvy látek
• vakcíny proti chřipce * MeSH

INTRODUCTION: We investigate how characteristics of sleep-wake dynamics in humans are modified by narcolepsy, a clinical condition that is supposed to destabilize sleep-wake regulation. Subjects with and without cataplexy are considered separately. Differences in sleep scoring habits as a possible confounder have been examined. AIMS AND METHODS: Four groups of subjects are considered: narcolepsy patients from China with (n = 88) and without (n = 15) cataplexy, healthy controls from China (n = 110) and from Europe (n = 187, 2 nights each). After sleep-stage scoring and calculation of sleep characteristic parameters, the distributions of wake-episode durations and sleep-episode durations are determined for each group and fitted by power laws (exponent α) and by exponentials (decay time τ). RESULTS: We find that wake duration distributions are consistent with power laws for healthy subjects (China: α = 0.88, Europe: α = 1.02). Wake durations in all groups of narcolepsy patients, however, follow the exponential law (τ = 6.2-8.1 min). All sleep duration distributions are best fitted by exponentials on long time scales (τ = 34-82 min). CONCLUSIONS: We conclude that narcolepsy mainly alters the control of wake-episode durations but not sleep-episode durations, irrespective of cataplexy. Observed distributions of shortest wake and sleep durations suggest that differences in scoring habits regarding the scoring of short-term sleep stages may notably influence the fitting parameters but do not affect the main conclusion.

• Klíčová slova
• cataplexy *, exponential distribution *, narcolepsy *, power-law distribution *, sleep scoring *, sleep–wake dynamics *, wake-episode durations *,
• MeSH
• dítě MeSH
• dospělí MeSH
• kataplexie diagnóza   epidemiologie   patofyziologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• narkolepsie diagnóza   epidemiologie   patofyziologie   MeSH
• předškolní dítě MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• spánek fyziologie   MeSH
• stadia spánku fyziologie   MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Čína epidemiologie   MeSH
• Evropa epidemiologie   MeSH

Narcolepsy-cataplexy (NC) is a chronic neurological disease with suggested autoimmune etiopathogenesis. Nicotine stimulates central nervous system and smoking increases the risk of autoimmune diseases. Assessment of smoking habits and its correlation to clinical parameters among 87 adult NC patients (38 male, 49 female) included night polysomnography and multiple sleep latency test. In our sample, 43.7% NC patients were regular smokers, and 19.5% former smokers compared to 22.2%, and 12.6%, respectively, in the general population. Patients started to smoke in the mean age of 20.0 (SD ±6.0) years. 72.2% of NC smokers started to smoke before the onset of NC and the mean of the delay between smoking onset and NC onset was 9.1 (±5.8) years. We found a direct correlation between smoking duration and the number of awakenings, duration of N1 sleep, REM sleep latency, and apnoea/hypopnoea index (AHI), and, on the contrary, indirect correlation between smoking duration and N3 sleep duration, showing that smoking duration consistently correlates with sleep macrostructure. Smoking is highly prevalent in NC and has relationship with clinical features of NC.

• Klíčová slova
• Etiopathogenesis, Narcolepsy-cataplexy, Nicotine, Smoking, Stimulating effect,
• MeSH
• dospělí MeSH
• kataplexie diagnóza   farmakoterapie   epidemiologie   MeSH
• komorbidita MeSH
• kouření epidemiologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• narkolepsie diagnóza   farmakoterapie   epidemiologie   MeSH
• polysomnografie MeSH
• prevalence MeSH
• rizikové faktory MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• stimulanty centrálního nervového systému terapeutické užití   MeSH
• syndromy spánkové apnoe epidemiologie   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika MeSH
• Názvy látek
• stimulanty centrálního nervového systému MeSH

Narcolepsy with cataplexy is a rare disease with an estimated prevalence of 0.02% in European populations. Narcolepsy shares many features of rare disorders, in particular the lack of awareness of the disease with serious consequences for healthcare supply. Similar to other rare diseases, only a few European countries have registered narcolepsy cases in databases of the International Classification of Diseases or in registries of the European health authorities. A promising approach to identify disease-specific adverse health effects and needs in healthcare delivery in the field of rare diseases is to establish a distributed expert network. A first and important step is to create a database that allows collection, storage and dissemination of data on narcolepsy in a comprehensive and systematic way. Here, the first prospective web-based European narcolepsy database hosted by the European Narcolepsy Network is introduced. The database structure, standardization of data acquisition and quality control procedures are described, and an overview provided of the first 1079 patients from 18 European specialized centres. Due to its standardization this continuously increasing data pool is most promising to provide a better insight into many unsolved aspects of narcolepsy and related disorders, including clear phenotype characterization of subtypes of narcolepsy, more precise epidemiological data and knowledge on the natural history of narcolepsy, expectations about treatment effects, identification of post-marketing medication side-effects, and will contribute to improve clinical trial designs and provide facilities to further develop phase III trials.

• Klíčová slova
• European Narcolepsy Centres, epidemiology, multicentre studies, narcolepsy, prospective data collection, standardized prospective database,
• MeSH
• databáze faktografické * normy   MeSH
• dospělí MeSH
• fenotyp MeSH
• internet MeSH
• kataplexie farmakoterapie   epidemiologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• narkolepsie * farmakoterapie   epidemiologie   MeSH
• postmarketingový dozor MeSH
• prospektivní studie MeSH
• registrace * normy   MeSH
• řízení kvality MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• šíření informací MeSH
• vzácné nemoci farmakoterapie   epidemiologie   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Evropa epidemiologie   MeSH

We present the case of an 86 year old female, in whom narcolepsy with cataplexy (NC) manifested at 52 years of age. She was treated by an amphetamine-like drug phenmetrazine and tricyclic antidepressants for more than 10 years. Hypokinetic-rigid syndrome manifested at 83 years of age and Parkinson´s disease (PD) was diagnosed. Detailed examination at the age of 86 confirmed the previous diagnosis of NC and the diagnosis of PD. Severe periodic limb movements in sleep, severe sleep apnea, REM sleep behavior disorder and restless legs syndrome, which are frequently comorbid in NC and PD, were revealed. The patient's somnolence worsened, apparently accentuated by pramipexole treatment, as changing therapy to levodopa led to a reduction of sleepiness.

• MeSH
• kataplexie diagnóza   epidemiologie   MeSH
• komorbidita MeSH
• lidé MeSH
• narkolepsie diagnóza   epidemiologie   MeSH
• Parkinsonova nemoc diagnóza   epidemiologie   MeSH
• senioři nad 80 let MeSH
• Check Tag
• lidé MeSH
• senioři nad 80 let MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• práce podpořená grantem MeSH

OBJECTIVES: Antidepressants substantially affect REM sleep characteristics and trigger manifestations of REM sleep behavior disorder (RBD) in the general, non-narcoleptic, population. Antidepressants are also frequently administrated in an attempt to suppress cataplexy. We investigated the role of antidepressants in the development of RBD in narcolepsy with cataplexy (NC) patients. PATIENTS/METHODS: Seventy-five patients diagnosed with NC were assessed by a structured interview (focused on RBD manifestations and the use of antidepressants) and night video-polysomnography followed by the multiple sleep latency test. RESULTS: Of all 75 NC patients (36 male, 39 female; mean age 46.1±18.5 years), 34 cases had a history of antidepressant use (45.3%; 18 male, 16 female). In this antidepressant-positive group, 13 patients suffered from RBD (38.2%). Among antidepressant-naïve patients, only 5 subjects (12.2%) were diagnosed with RBD. Polysomnographic data showed significantly increased REM latency (p<0.01) and reduced percentage of REM sleep (p<0.01) in the antidepressant-positive group, as well as more periodic limb movements during sleep (p=0.01). CONCLUSIONS: NC patients with a history of antidepressant use showed a three-fold higher occurrence of RBD in comparison to antidepressant-naïve patients.

• MeSH
• antidepresiva škodlivé účinky   terapeutické užití   MeSH
• depresivní poruchy farmakoterapie   MeSH
• dospělí MeSH
• kohortové studie MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• narkolepsie chemicky indukované   epidemiologie   patofyziologie   MeSH
• polysomnografie MeSH
• porucha chování v REM spánku chemicky indukované   patofyziologie   MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• senioři MeSH
• spánek REM účinky léků   MeSH
• studie případů a kontrol MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Názvy látek
• antidepresiva MeSH

In a retrospective cohort study undertaken in 12 European countries, 249 female narcoleptic patients with cataplexy (n = 216) and without cataplexy (n = 33) completed a self-administrated questionnaire regarding pregnancy and childbirth. The cohort was divided further into patients whose symptoms of narcolepsy started before or during pregnancy (308 pregnancies) and those in whom the first symptoms of narcolepsy appeared after delivery (106 pregnancies). Patients with narcolepsy during pregnancy were older during their first pregnancy (P < 0.001) and had a higher body mass index (BMI) prior to pregnancy (P < 0.01). Weight gain during pregnancy was higher in narcoleptic patients with cataplexy (P < 0.01). More patients with narcolepsy-cataplexy during pregnancy had impaired glucose metabolism and anaemia. Three patients experienced cataplexy during delivery. The rate of caesarean sections was higher in the narcolepsy-cataplexy group compared to the narcolepsy group (P < 0.05). The mean birth weight and gestational age of neonates were within the normal range and did not differ across groups. Neonatal care was affected adversely by symptoms of narcolepsy in 60.1% of those with narcolepsy during pregnancy. This study reports more obstetric complications in patients with narcolepsy-cataplexy during pregnancy; however, these were not severe. This group also had a higher BMI and higher incidence of impaired glucose metabolism during pregnancy. Caesarian section was conducted more frequently in narcolepsy-cataplexy patients, despite cataplexy being a rare event during delivery. Furthermore, symptoms of narcolepsy may render care of the infant more difficult.

• Klíčová slova
• cataplexy, delivery, metabolism, newborn, pregnancy, puerperium,
• MeSH
• anemie epidemiologie   MeSH
• časové faktory MeSH
• císařský řez statistika a číselné údaje   MeSH
• gestační stáří MeSH
• hmotnostní přírůstek MeSH
• index tělesné hmotnosti MeSH
• kataplexie epidemiologie   MeSH
• kohortové studie MeSH
• kojení MeSH
• komplikace těhotenství epidemiologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• narkolepsie epidemiologie   MeSH
• novorozenec MeSH
• poporodní období psychologie   MeSH
• porodní hmotnost MeSH
• průzkumy a dotazníky MeSH
• retrospektivní studie MeSH
• těhotenství MeSH
• zpráva o sobě MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• novorozenec MeSH
• těhotenství MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Evropa MeSH

Narcolepsy-cataplexy (N-C) is a focal neurodegenerative disease with a genetic predisposition and autoimmune etiology; the pathogenesis of narcolepsy without cataplexy (Nw/oC) is less clear. One hundred and forty eight patients underwent clinical face-to face interviews, polysomnography, multiple sleep latency testing and HLA-DQB1*0602 typing. The cohort was divided into four age groups: children and adolescents under 19 years (N = 31), adults aged 20-39 years (N = 51), 40-59 years (N = 28) and over 60 years (N = 38). N-C was found in 93 adults (79.5 %) compared with 16 pediatric patients (51.6 %) (p < 0.01), suggesting that at least some of the children were candidates for developing cataplexy in the future. Statistical evaluation showed an increasing age-related proportion of associated sleep disorders-obstructive sleep apnea, periodic leg movements and restless leg syndrome (p < 0.001). Nw/oC patients showed sleep comorbidities less frequently than N-C group. A close connection with N-C was found particularly in REM behavior disorder (RBD) (p < 0.05). RBD affected a third of the patients in the youngest as well as in the oldest groups. However, association with other sleep disorders had no significant effect on nocturnal sleep (with the exception of obstructive sleep apnea), and the sleep comorbidities under study had no noticeable effect on daytime sleepiness.

• MeSH
• dítě MeSH
• dospělí MeSH
• kohortové studie MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• narkolepsie diagnóza   epidemiologie   patofyziologie   MeSH
• polysomnografie metody   MeSH
• porucha chování v REM spánku diagnóza   epidemiologie   patofyziologie   MeSH
• poruchy spánku a bdění diagnóza   epidemiologie   patofyziologie   MeSH
• průřezové studie MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• stadia spánku fyziologie   MeSH
• syndrom neklidných nohou diagnóza   epidemiologie   patofyziologie   MeSH
• věkové faktory MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• srovnávací studie MeSH