![]() Spontaneous evolution of the disease has not been much studied, but the phenotype and evolution seems variable : stability, disappearance of the symptoms, development of cataplexy (conversion to narcolepsy type 1), or change to idiopathic hypersomnia. Previously, narcolepsy was divided into two types: narcolepsy with cataplexy (a sudden loss of muscle tone brought on by strong emotions) and narcolepsy without cataplexy. In narcolepsy without cataplexy, excessive daytime sleepiness must be present almost daily for at least three months, but there is either no history of clear-cut cataplexy or a history of atypical (questionable) cataplexy-like episodes. ![]() The disease has a negative impact on scholarly and professional performances. Second-line treatments include methylphenidate, sodium oxybate, solriamfetol or amphetamines. Modafinil or pitolisant are the first line treatments with the best benefit/risk ratio. Treatment is only symptomatic, based on stimulant drugs and a good sleep hygiene (sufficient sleep duration at night is required, with scheduled short naps during the day). Rare familial cases have been reported however, the mode of inheritance is unclear. Cataplexy is when the leg, arm or face muscles suddenly become weak. Other causes of sleepiness, including narcolepsy type 1, chronic insufficient sleep and idiopathic hypersomnia (especially the form without long sleep time), must be systematically taken into account. Narcolepsy with cataplexy Narcolepsy without cataplexy. A pure clinical diagnosis is not possible due to the absence of pathognomonic symptom such as cataplexy, and daytime sleepiness being a non-specific symptom. According to the ICSD 2nd Edition (ICSD-2), narcolepsy with cataplexy (NwithC), narcolepsy without cataplexy (Nw/oC), idiopathic hypersomnia with long sleep. Nocturnal and daytime polysomnography demonstrates an average sleep latency of under eight minutes with at least two sleep onset rapid eye movement periods (SOREMP) on multiple sleep latency tests. Diagnostic methodsÄefinitive diagnosis requires the presence of clinical symptoms and characteristics polysomnography findings. The presence of the HLA DQB1*0602 allele is reported in 40% of cases (more than in the general population but less than in cases of narcolepsy type 1, 98%). Reduction of hypocretin-1 levels is found in the cerebrospinal fluid in 10-20% of cases, and in which case the disorder is reclassified narcolepsy type 1. The etiology is still unknown, probably because this disorder is heterogeneous. It can also be a lifelong disease, with stable symptomatology. Narcolepsy type 2 has a variable phenotype and evolution, with sometimes improvement or even disappearance of the symptoms, rarely the development of cataplexy (conversion to narcolepsy type 1), or a change in the phenotype to idiopathic hypersomnia. Narcolepsy type 2 manifests generally between the age of 10 and 30 years old, although onset in childhood is rare. Narcolepsy type 2 prevalence numbers are controversial, sometimes higher and sometimes lower than those of narcolepsy type 1, depending on reports. No clear epidemiological data are available.
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