Cataplexy with narcolepsy4/19/2023 ![]() ![]() 11 Indeed, many people with an NC onset during childhood or adolescence are diagnosed only when they are adults, after they completed and possibly failed their academic studies, gained an almost irreversible obesity and experienced low self-esteem. Despite more than half of patients with NC have a disease onset prior to the age of 18 years, 10 there is a delay of more than 10 years between the disease onset and its diagnosis. The question of age at onset and age at diagnosis is important in the context of NC. Additionally, the human leukocyte antigen (HLA) DQB1*0602 genotype is closely associated with NC. In opposite, in narcolepsy type 2, there is no decrease in hypocretin levels. 7, 8 Indeed, low cerebrospinal fluid (CSF) hypocretin-1 levels (less than or equal to 110 pg/mL) are found in more than 90% of patients with NC (also called narcolepsy type 1 or hypocretin deficient). 4, 5 Narcolepsy with cataplexy is caused by a loss of hypocretin-1 neurons located in the lateral hypothalamus, 6 probably via an autoimmune mechanism. 2, 3 The NC prevalence increased after the H1N1 infection and vaccine. The prevalence of narcolepsy ranges from 0.02% to 0.05% in European and North American populations. 1 The frequent comorbidities of NC include obesity, restless legs syndrome (RLS), periodic limb movement syndrome (PLMS), depressive mood, and symptoms of attention-deficit/hyperactivity disorder (ADHD). Narcolepsy with cataplexy (NC) is a rare neurological disorder characterized by excessive daytime sleepiness (EDS) with irresistible sleep attacks and cataplexy (sudden loss of muscle tone triggered by emotions), associated sometimes with other abnormal rapid eye movement (REM) manifestations such as hypnagogic hallucinations, sleep paralysis, REM sleep behavior disorder (RBD), and disturbed nocturnal sleep. These differences should be considered to ensure a prompt diagnosis. ConclusionĪt time of NC diagnosis, children have more frequent obesity, night eating, parasomnia, sleep talking, drunkenness, and ADHD symptoms than adults, even if sleepiness and cataplexy do not differ. The body mass index ( z-score) was correlated with OAHI ( r = .32). ![]() No between-group differences were found at the multiple sleep latency test. Obstructive apnea-hypopnea index (OAHI) was lower in children with higher mean and minimal oxygen saturation than in adults. Quality of life was essentially impacted by depressive feelings in both children and adults. There was no difference between groups for insomnia and fatigue scores. However, adults had lower quality of life than children. Depressive feelings affected not differently children (24%) and adults (32%). Children scored higher than adults at the attention-deficit/hyperactivity disorder (ADHD) scale. ![]() The frequency of obesity (54% vs 17%), night eating (29% vs 7%), parasomnia (89% vs 43%), sleep talking (80% vs 34%), and sleep drunkenness (69% vs 24%) were higher in children than in adults, the frequency of sleep paralysis was lower (20% vs 55%) but the frequency of cataplexy and the severity of sleepiness were not different. The clinical and sleep measures were compared between patients diagnosed before (46 children, median age: 12 year old) and after (46 adults, median age: 28.5 year old) 18 years of age. MethodsÄe novo patients with NC diagnosis completed a standardized questionnaire and interview, followed by a sleep study. Sporadic outbreak in horses without other affected horses on a premises.To compare symptoms and sleep characteristics in patients diagnosed with narcolepsy-cataplexy (NC) before and after the age of 18 years.Episodes may last from several minutes to 15-20 min.Sudden onset of muscular weakness and collapse without prior warning.Episodes may occur daily or one every few months. ![]() Feeding (after first few mouthfuls of feed or water).Attacks of acute weakness and recumbency may follow feeding, grooming, leading out of a stall, putting on tack or exercise.Normal behavior and physical examination findings between attacks.Low CSF concentrations of orexin have been found in human narcolepsy patients with cataplexy, whereas normal CSF concentrations of orexin were found in narcolepsy patients without cataplexy.Orexin-containing neurons are found in the posterior and lateral hypothalamus and project widely throughout the CNS, innervating aminergic and cholinergic regions that promote wakefulness.Recent research in people suggests that a deficiency or impaired signaling of the neuropeptide orexin (hypocretin) from a loss of orexin-producing neurons leads to episodes of daytime sleepiness.Unknown, but thought to be pathologic manifestation of REM sleep, cerebrocortical and voluntary muscle disassociation. ![]()
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