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.
- 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
The pathophysiology of recurrent isolated sleep paralysis (RISP) has yet to be fully clarified. Very little research has been performed on electroencephalographic (EEG) signatures outside RISP episodes. This study aimed to investigate whether sleep is disturbed even without the occurrence of a RISP episode and in a stage different than conventional REM sleep. 17 RISP patients and 17 control subjects underwent two consecutive full-night video-polysomnography recordings. Spectral analysis was performed on all sleep stages in the delta, theta, and alpha band. EEG microstate (MS) analysis was performed on the NREM 3 phase due to the overall high correlation of subject template maps with canonical templates. Spectral analysis showed a significantly higher power of theta band activity in REM and NREM 2 sleep stages in RISP patients. The observed rise was also apparent in other sleep stages. Conversely, alpha power showed a downward trend in RISP patients' deep sleep. MS maps similar to canonical topographies were obtained indicating the preservation of prototypical EEG generators in RISP patients. RISP patients showed significant differences in the temporal dynamics of MS, expressed by different transitions between MS C and D and between MS A and B. Both spectral analysis and MS characteristics showed abnormalities in the sleep of non-episodic RISP subjects. Our findings suggest that in order to understand the neurobiological background of RISP, there is a need to extend the analyzes beyond REM-related processes and highlight the value of EEG microstate dynamics as promising functional biomarkers of RISP.
- 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.
- 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
In recent years, there has been an increased interest in elucidating the influence of the gut microbiota on sleep physiology. The gut microbiota affects the central nervous system by modulating neuronal pathways through the neuroendocrine and immune system, the hypothalamus-pituitary-adrenal axis, and various metabolic pathways. The gut microbiota can also influence circadian rhythms. In this study, we observed the gut microbiota composition of patients suffering from narcolepsy type 1, narcolepsy type 2, and idiopathic hypersomnia. We did not observe any changes in the alpha diversity of the gut microbiota among patient groups and healthy controls. We observed changes in beta diversity in accordance with Jaccard dissimilarities between the control group and groups of patients suffering from narcolepsy type 1 and idiopathic hypersomnia. Our results indicate that both these patient groups differ from controls relative to the presence of rare bacterial taxa. However, after adjustment for various confounding factors such as BMI, age, and gender, there were no statistical differences among the groups. This indicates that the divergence in beta diversity in the narcolepsy type 1 and idiopathic hypersomnia groups did not arise due to sleep disturbances. This study implies that using metabolomics and proteomics approaches to study the role of microbiota in sleep disorders might prove beneficial.
- MeSH
- idiopatická hypersomnie * MeSH
- lidé MeSH
- narkolepsie * MeSH
- poruchy nadměrné spavosti * MeSH
- poruchy spánku a bdění * MeSH
- spánek MeSH
- střevní mikroflóra * MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
The neurodegenerative synucleinopathies, including Parkinson's disease and dementia with Lewy bodies, are characterized by a typically lengthy prodromal period of progressive subclinical motor and non-motor manifestations. Among these, idiopathic REM sleep behaviour disorder is a powerful early predictor of eventual phenoconversion, and therefore represents a critical opportunity to intervene with neuroprotective therapy. To inform the design of randomized trials, it is essential to study the natural progression of clinical markers during the prodromal stages of disease in order to establish optimal clinical end points. In this study, we combined prospective follow-up data from 28 centres of the International REM Sleep Behavior Disorder Study Group representing 12 countries. Polysomnogram-confirmed REM sleep behaviour disorder subjects were assessed for prodromal Parkinson's disease using the Movement Disorder Society criteria and underwent periodic structured sleep, motor, cognitive, autonomic and olfactory testing. We used linear mixed-effect modelling to estimate annual rates of clinical marker progression stratified by disease subtype, including prodromal Parkinson's disease and prodromal dementia with Lewy bodies. In addition, we calculated sample size requirements to demonstrate slowing of progression under different anticipated treatment effects. Overall, 1160 subjects were followed over an average of 3.3 ± 2.2 years. Among clinical variables assessed continuously, motor variables tended to progress faster and required the lowest sample sizes, ranging from 151 to 560 per group (at 50% drug efficacy and 2-year follow-up). By contrast, cognitive, olfactory and autonomic variables showed modest progression with higher variability, resulting in high sample sizes. The most efficient design was a time-to-event analysis using combined milestones of motor and cognitive decline, estimating 117 per group at 50% drug efficacy and 2-year trial duration. Finally, while phenoconverters showed overall greater progression than non-converters in motor, olfactory, cognitive and certain autonomic markers, the only robust difference in progression between Parkinson's disease and dementia with Lewy bodies phenoconverters was in cognitive testing. This large multicentre study demonstrates the evolution of motor and non-motor manifestations in prodromal synucleinopathy. These findings provide optimized clinical end points and sample size estimates to inform future neuroprotective trials.
- MeSH
- biologické markery MeSH
- demence s Lewyho tělísky * diagnóza MeSH
- lidé MeSH
- Parkinsonova nemoc * komplikace diagnóza MeSH
- porucha chování v REM spánku * diagnóza MeSH
- prodromální symptomy MeSH
- progrese nemoci MeSH
- prospektivní studie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- práce podpořená grantem MeSH
OBJECTIVE: This study aimed to determine the frequency, type, and correlates of a broad spectrum of sleep disorders in adults with COVID-19 up to 32 months after infection. METHODS: We conducted a national online survey (Jun 2021-Dec 2022), gathering information on COVID-19 diagnosis, acute disease course, and the subsequent development of sleep disorders from 1507 respondents (mean age 44.5 ± 13.1 years, 64.1% women). RESULTS: 81.3% (1223) reported at least one sleep difficulty that either worsened or first appeared with COVID-19. Females reported a higher number of symptoms (2.03 ± 1.44 versus 1.72 ± 1.43 in men, p < 0.0001). Most common were insomnia symptoms (59.4%), followed by night sweats (38.4%), hypersomnolence (33.3%), vivid dreams or nightmares (26.4%), restless leg syndrome (RLS) (22.8%), and sleep-related breathing disorders (11.1%). All symptoms were associated with a more severe acute disease. A mild decreasing trend in the persistence of sleep symptoms with a longer latency since infection was observed, with 66.7% reporting at least half of their symptoms present at 3-5 months after acute infection, compared to 64.9% at 6-8 months, and 62.4% at 9-11 months (p = 0.0427). However, among those after 12 or more months, over half of the symptoms persisted in 69.5%. The frequency of vivid dreams and nightmares increased in association with COVID-19 in 32.9% (p < 0.001). 9.4% (141) reported new-onset or increased parasomnic manifestations after the infection. CONCLUSIONS: Our research shows that sleep disturbances are a common and persistent manifestation of COVID-19 that affects a large proportion of the population and deserves careful monitoring.
- Publikační typ
- časopisecké články MeSH
Cíl: Studie si kladla za cíl ověřit vhodnost použití Škály tíže narkolepsie (Narcolepsy Severity Scale; NSS) jako základního klinického nástroje pro stanovení subjektivní tíže onemocnění u pacientů s narkolepsií typu 1 (NT1) v ČR. Soubor a metodika: Celkem 78 pacientů ze 2 spánkových center s diagnózou NT1 (29 mužů, 49 žen, průměrný věk 36,1 ± 11,7 let, rozmezí 18–71 let, z toho léčených n = 51) vyplnilo škálu NSS sestávající z 15 otázek zaměřených na výskyt, frekvenci a dopad na denní aktivity všech hlavních narkoleptických příznaků. Současně byli instruováni vyplnit Epworthskou škálu spavosti (Epworth Sleepiness Scale; ESS), Škálu tíže únavy (Fatigue Severity Scale; FSS), Škálu hodnocení úzkosti a deprese při hospitalizaci (Hospital Anxiety and Depression Rating Scale; HADS) a zkrácenou verzi Dotazníku kvality života (Quality of Life Questionnaire; SF-36). Výsledky: Škála NSS vykazuje dobrou vnitřní konzistenci dotazníku pomocí koeficientu Cronbachova a, která je pro celou kohortu pacientů s NT1 0,80, pro skupinu léčených pacientů 0,79 a pro skupinu neléčených pacientů 0,82. Keiser-Meyer-Olkinův index pro celou kohortu je 0,73, což potvrzuje dostatečnou strukturální validitu dotazníku. Nebyl zjištěn signifikantní rozdíl ve skóre NSS léčených a neléčených pacientů, nicméně byla potvrzena korelace celkového skóre NSS s ESS (ρ = 0,61; p < 0,0001) a FSS (ρ = 0,4438; p < 0,0001). Závěr: NSS představuje vhodný a snadno aplikovatelný klinický nástroj ke stanovení subjektivní tíže onemocnění, dobře vystihuje hlavní narkoleptické příznaky a hodnotí jejich vliv na denní aktivity.
Aim: The aim of the study was to verify the applicability of the Narcolepsy Severity Scale (NSS) as a basic clinical tool for determining the subjective severity of the disease in patients with narcolepsy type 1 (NT1) in the Czech Republic. Patients and methods: A total of 78 patients from 2 sleep centers with a diagnosis of NT1 (29 men, 49 women, mean age 36.1 ± 11.7 years, range 18–71 years, N = 51 were treated) completed the NSS scale consisting of 15 questions focusing on the occurrence, frequency, and impact on daily activities of all major narcoleptic symptoms. At the same time, they were instructed to complete the Epworth Sleepiness Scale (ESS), the Fatigue Severity Scale (FSS), the Hospital Anxiety and Depression Rating Scale (HADS) and a short version of the Quality of Life Questionnaire (SF-36). Results: The NSS scale shows good internal consistency of the questionnaire using Cronbach‘s a, which is 0.80 for the whole cohort of NT1 patients, 0.79 for the treated group and 0.82 for the untreated group. The Keiser-Meyer-Olkin index for the entire cohort is 0.73, confirming sufficient structural validity of the questionnaire. There was no significant difference in the NSS scores of treated and untreated patients; however, the correlation of the total NSS score with ESS (ρ = 0.61; P < 0.0001) and FSS (ρ = 0.4438; P < 0.0001) was confirmed. Conclusions: The NSS is a convenient and practical clinical tool for determining the subjective severity of the disease, well capturing the main narcoleptic symptoms and assessing their impact on daily activities.
OBJECTIVES: Pregnancy is often associated with reduced sleep quality and an increase in sleep disorders, such as restless leg syndrome, obstructive sleep apnea, and insomnia. There are few studies investigating the prevalence of parasomnias in pregnancy, although they may be expected to be a significant problem, as disturbed sleep in this time period in addition to these sleep disorders may trigger parasomnia episodes. METHODS: We conducted a survey using an online questionnaire focusing on a comparison of the prevalence of parasomnias in three time periods: 3 months before pregnancy, during pregnancy, and 3 months after delivery. We also inquired about psychiatric and neurological comorbidities, current anxiety and depression symptoms, and pregnancy complications. RESULTS: A total of 325 women (mean age 30.3 ± 5.3 years) participated in the online survey. The overall number of reported parasomnias increased during pregnancy compared to the 3 months before pregnancy (p < 0.001) and decreased after childbirth (p < 0.001). Specifically, we found a significant increase in sleepwalking (p = 0.02) and night terrors (p < 0.001), as well as in vivid dreams (p < 0.001) and nightmares (p < 0.001) during pregnancy. A similar significant increase during pregnancy was reported for head explosion (p < 0.011). In contrast, the number of episodes of sleep paralysis increased after delivery (p = 0.008). At the individual level, an increase in the severity/frequency of individual parasomnia episodes was also observed during pregnancy. Participants whose vivid dreams/nightmares persisted after delivery had higher BDI-II and STAI-T scores. Our data also suggest a significant impact of migraines and other chronic pain, as well as complications during pregnancy, on the presence of parasomnia episodes in our cohort. CONCLUSIONS: We have shown that the prevalence of parasomnias increases during pregnancy and needs to be targeted, especially by non-pharmacological approaches. At the same time, it is necessary to inquire about psychiatric and neurological comorbidities and keep in mind that more sleep disorders may be experienced by mothers who have medical complications during pregnancy.
- Publikační typ
- časopisecké články MeSH
INTRODUCTION: Huntington's disease (HD) is often on the margin of standard medical practice due to its low prevalence, the lack of causal treatment, and the typically long premanifest window prior to the onset of the symptoms, which contrasts with the long-lasting burden that the disease causes in affected families. METHODS: To capture these socio-psychological aspects of HD and map the experiences of affected individuals, persons at risk of HD, and caregivers, we created a questionnaire using a qualitative research approach. The questionnaire containing 16 questions was conducted online for a period of three months through patient associations in Slovakia and their infrastructures. RESULTS: In total, we received 30 responses. The survey results, in particular, indicate insufficient counselling by physicians with explicitly missing information about the possibility of preimplantation genetic diagnostic. There was also a necessity to improve comprehensive social and health care in the later stages of the disease, raise awareness of the disease in the general health community, and provide more information on ongoing clinical trials. CONCLUSION: The psychosocial effects, as well as the burden, can be mitigated by comprehensive genetic counselling as well as reproductive and financial guidelines and subsequent therapeutic programs to actively support patients, caregivers, children, and adolescents growing up in affected families, preferably with the help of local HD community association. LIMITATIONS: We have used online data collection to reach a wider HD community, but at the same time, we are aware that the quality of the data we would obtain through face-to-face interviews would be considerably better. Therefore, future studies need to be conducted to obtain more detailed information.
- Publikační typ
- časopisecké články MeSH
Cíl: Komplexní přehled neurologických příznaků u osob nakažených virem SARS-CoV-2 v rámci ČR dosud není znám. Naším cílem proto bylo zjistit zastoupení, četnost a trvání nových neurologických příznaků, které se objevily v souvislosti s onemocněním COVID-19 u dospělých osob na území celé ČR. Metodika: Pro tyto účely byl vyvinut online dotazník Neurocovid. Předkládáme vyhodnocená data z období 1. 6. až 30. 8. 2021. Výsledky: Elektronický dotazník vyplnilo 1 012 osob ve věkovém rozmezí 18–79 let (průměrný věk 45,6 ±12,88 let, medián 45 let, 65 % žen). Pokrytí krajů ČR většinově odpovídalo demografickému rozložení obyvatelstva. Nejčastěji udávané nové neurologické příznaky asociované s onemocněním COVID-19 zahrnovaly kognitivní změny (pomalé myšlení, poruchy paměti, nesoustředěnost, problémy s řečí a dezorientace), přičemž alespoň jeden z těchto příznaků uvedlo 70 % účastníků. Obdobně četné byly poruchy spánku, které se objevily u více než 70 % osob. U obou skupin příznaků bylo zároveň časté dlouhodobé trvání. Kognitivní změny přetrvávaly déle než 3 měsíce u 46 %, poruchy spánku u 72 %. Častým neurologickým příznakem byla rovněž porucha čichu a/nebo chuti, kterou pociťovalo 67 % účastníků. K dalším obtížím patřily závratě (32 %), brnění na těle/končetinách (27 %), nemotornost nebo ztráta stability při chůzi (20 %), tinnitus (15 %), porucha zraku (14 %) a třes (13 %). Nebyla zjištěna žádná souvislost mezi tíží akutního onemocnění COVID-19 a počtem nebo typem nových neurologických příznaků. Závěr: Tento celostátní online průzkum na reprezentativní skupině obyvatel ČR potvrdil široké spektrum neurologických příznaků v souvislosti s onemocněním COVID-19. Dotazník Neurocovid je vhodné šířit a vyplňovat k dalšímu zvyšování kvality dat a sledování problematiky v čase.
Aim: There are no broad-scale data available on the presence of neurological symptoms in persons afflicted by COVID-19 in the Czech Republic. Therefore, our goal was to establish the incidence, frequency and duration of new neurological symptoms present in the adult population in the Czech Republic in connection with the COVID-19 disease. Methods: For this purpose, an online questionnaire Neurocovid was developed. The data presented were collected during the period of June 6–August 30, 2021. Results: The questionnaire was fi lled in by 1,012 individuals aged between 18–79 years (average age 45.6 ±12.88 years, median age 45 years, 65% female). Regional coverage of the Czech Republic mostly corresponded with the demographic distribution of the population. The most common new neurological symptoms that occurred in connection with COVID-19 were cognitive changes such as “slow thinking”, memory difficulties, lack of concentration, speech disorders and disorientation. About 70% of the participants reported at least one of these symptoms. Similar frequency was noted for sleep disorders which were present in more than 70% of the participants. Both groups of symptoms also had a higher tendency to persist long-term. Cognitive changes persisted for over 3 months in 46%, whereas sleep disorders in 72%. Another frequent neurological symptom was olfactory/gustatory dysfunction, which was observed by 67% of the participants. Other symptoms included vertigo (32%), tingling in the body/ limbs (27%), clumsiness/loss of stability while walking (20%), tinnitus (15%), issues with eyesight (14%), and tremor (13%). We did not find a correlation between the severity of the acute phase of the COVID-19 disease and the number or type of new neurological symptoms. Conclusion: This nation-wide online survey of a representative group of the Czech population confirmed a variety of neurological symptoms that occur in connection with COVID-19. The Neurocovid questionnaire should be distributed further to increase data quality and provide an insight into the long-term development of these symptoms.
- Klíčová slova
- postcovidový syndrom,
- MeSH
- COVID-19 * komplikace MeSH
- kognitivní poruchy etiologie MeSH
- lidé MeSH
- neurologické manifestace * MeSH
- poruchy spánku a bdění etiologie MeSH
- průzkumy a dotazníky MeSH
- SARS-CoV-2 MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- práce podpořená grantem MeSH
