We have verified the eligibility of the Idiopathic Hypersomnia Severity Scale (IHSS) as a basic clinical tool for determining the subjective severity of illness in patients with idiopathic hypersomnia (IH) in the Czech Republic. Total of 37 patients with a diagnosis of IH (9 men, 28 women, mean age 40.2 ± 12.8) completed the IHSS scale. At the same time, they were instructed to complete the Epworth Sleepiness Scale (ESS), the Fatigue Severity Scale (FSS), the Hospital Anxiety and Depression Scales (HADS-A and HADS-D), and a short version of the Quality of Life Questionnaire (SF-36). The control group consisted of 88 age- and sex-matched healthy volunteers. The IHSS scale showed good internal consistency of the questionnaire using Cronbach's α, which was 0.88. The KMO (Keiser-Meyer-Olkin index) was 0.72, confirming sufficient structural validity of the questionnaire. The correlation of the total IHSS score with the ESS (ρ = 0.59, p=0.0001) and FSS (ρ = 0.84, p<0.0001) as well as with the HADS-A scales (ρ = 0.64, p<0.0001), HADS-D (ρ = 0.79, p<0.0001) and SF-36 in both the mental (ρ = -0.85, p<0.0001) and physical health (ρ = -0.66, p<0.0001) components. The IHSS is a convenient and easy-to-apply clinical tool to assess subjective severity of illness, which describes well the symptoms of idiopathic hypersomnia and assesses their impact on health and daily activities.
- MeSH
- dospělí MeSH
- idiopatická hypersomnie * diagnóza MeSH
- kvalita života MeSH
- lidé středního věku MeSH
- lidé MeSH
- průzkumy a dotazníky MeSH
- reprodukovatelnost výsledků MeSH
- stupeň závažnosti nemoci * MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- validační studie MeSH
- Geografické názvy
- Česká republika MeSH
Sleep symptoms, including excessive sleepiness, are frequently reported by patients with functional motor disorders (FMD). We aimed to classify the comorbid sleep disorders in FMD, and to investigate the relationship between subjective sleepiness and objective measures of hypersomnia, comparing them with data from people with central hypersomnia. A total of 37 patients (mean [SD] age 46.4 [11.2] years) with clinically definite FMD, and 17 patients (mean [SD] age 41.1 [11.6] years) with central hypersomnia underwent structured medical and sleep history, neurological examination, polysomnography, multiple sleep latency test (MSLT), and questionnaires assessing sleepiness, fatigue, and depression. In all, 23 patients with FMD (62%) reported excessive daytime sleepiness. Evidence of specific sleep disorders was identified in our cohort, with 35% having restless legs syndrome; 49% obstructive sleep apnea; and 8% periodic limb movements in sleep; however, the presence of these disorders was not correlated with subjective sleepiness. Patients with FMD with self-reported sleepiness reported higher fatigue (p = 0.002), depression (p = 0.002), and had longer sleep latencies in the MSLT (p < 0.001) compared to the patients with central hypersomnia. No correlation was found between subjective and objective sleepiness in either group. Fatigue positively correlated with self-reported sleepiness in patients with FMD (p < 0.001). This study did not find objective correlates of increased sleepiness in patients with FMD. While sleep abnormalities were found to be common in FMD, they were not correlated with self-reports of excessive sleepiness. Positive correlations between self-reported sleepiness and fatigue support the current unified model of non-motor symptoms in FMD.
- MeSH
- deprese epidemiologie patofyziologie MeSH
- dospělí MeSH
- komorbidita * MeSH
- lidé středního věku MeSH
- lidé MeSH
- periodické pohyby končetinami ve spánku epidemiologie patofyziologie MeSH
- polysomnografie * MeSH
- poruchy nadměrné spavosti * epidemiologie patofyziologie MeSH
- poruchy spánku a bdění epidemiologie patofyziologie MeSH
- průzkumy a dotazníky MeSH
- somnolence MeSH
- spánková latence fyziologie MeSH
- syndrom neklidných nohou patofyziologie epidemiologie MeSH
- únava patofyziologie epidemiologie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- srovnávací studie MeSH
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
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.
INTRODUCTION: Idiopathic hypersomnia (IH) is a rare orphan disease characterized by excessive daytime sleepiness, frequently accompanied by prolonged nocturnal sleep and difficulties awakening, termed sleep inertia or sleep drunkenness. Severe sleepiness usually causes a greater handicap than manifestations of narcolepsy. METHODS: Forty-three IH patients (17 male, mean age 42.8 ± SD 12.2 years, range 20-67), diagnosed in the past 20 years according to ICSD-2 or ICSD-3 criteria were invited for clinical examination to evaluate the course, manifestations and severity of the disease, as well as clinical comorbidities. The patients completed a set of questionnaires scoring sleepiness, sleep inertia, fatigue, depression, anxiety, circadian preference, and quality of life. RESULTS: IH patients were divided according to the duration of nocturnal sleep at the time of their diagnosis into two cohorts: (1) with normal sleep duration (n = 25, 58.1%) and (2) with long sleep duration (n = 18, 41.9%). The mean duration of ad libitum sleep per 22 h in the second cohort was 732.0 ± 115.4 min (range 603-1100), and women markedly prevailed (n = 14, 77.8%). Age at disease onset was younger in the group with long sleep duration (21.2 ± 11.4 years versus 28.1 ± 13.6 years, p = 0.028), their MSLT latency was longer (7.2 ± 3.7 min versus 5.1 ± 1.7 min, p = 0.005), a history of sleep inertia prevailed (p = 0.005), and daily naps were mostly non-refreshing (p = 0.014). Additionally, questionnaires in the group with long sleep duration showed more severe sleep inertia (p = 0.007), fatigue (p = 0.004), and a tendency towards evening chronotype (p = 0.001). CONCLUSIONS: IH patients with long sleep duration differ clinically as well as by objective measures at the time of diagnosis and in long-term follow up from IH patients without long 24-h sleep time. In our opinion they represent an independent clinical entity to be considered in the revised ICSD-3 criteria.
- MeSH
- dospělí MeSH
- idiopatická hypersomnie * diagnóza MeSH
- kvalita života MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- narkolepsie * MeSH
- polysomnografie MeSH
- poruchy nadměrné spavosti * diagnóza epidemiologie etiologie MeSH
- senioři 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
