title: [Narcolepsy with cataplexy: an autoimmune disease?].
abstract: Narcolepsy type 1 (also named narcolepsy-cataplexy or hypocretin deficiency syndrome) is a rare sleep disorder characterized by excessive daytime sleepiness and cataplexy, plus frequently hypnagogic hallucinations, sleep paralysis and nocturnal sleep disturbances. Narcolepsy type 1 is an immune system-associated disease linked with the destruction of 70.000-90.000 hypocretin neurons notably involved in wakefulness. Among narcoleptic patients, 98% are positive for HLA-DQB1*06:02, a HLA class II allele, against 20-25% in general population. Individuals carrying HLA-DQB1*06:02 have an extraordinary risk to develop narcolepsy (odd ratio: 251). Other genes involved in CD4+ T cells and immune system activation as T-cell receptor α are also associated with narcolepsy. The development of the disease is linked with environmental factors such as influenza and streptococcal infections. Narcolepsy type 1 incidence also increased in Europe following the use of Pandemrix, a 2009 H1N1 AS03-adjuvanted vaccine manufactured by GlaxoSmithKline. Interestingly, such increase was not observed with Arepanrix, another vaccine developed by GSK very similar to Pandemrix.
authors.affiliation: Master biosciences, École normale supérieure de Lyon, 46, allée d'Italie, 69007 Lyon, France.
authors.forename: Louis
authors.initials: L
authors.lastname: Jacob
authors.affiliation: Centre de référence national maladie rare-narcolepsie et hypersomnie idiopathique, service de neurologie, hôpital Gui-de-Chauliac, Inserm U1061, Montpellier, France.
authors.forename: Yves
authors.initials: Y
authors.lastname: Dauvilliers
mesh_terms: D001327:Autoimmune Diseases / Q000235:genetics; D020022:Genetic Predisposition to Disease; D059866:HLA-DQ beta-Chains / Q000235:genetics; D006801:Humans; D057221:Influenza A Virus, H1N2 Subtype / Q000276:immunology; D007252:Influenza Vaccines / Q000009:adverse effects / Q000276:immunology; D007251:Influenza, Human / Q000276:immunology; D047908:Intracellular Signaling Peptides and Proteins / Q000502:physiology; D009290:Narcolepsy / Q000235:genetics / Q000276:immunology* / Q000503:physiopathology; D009474:Neurons / Q000737:chemistry / Q000276:immunology; D009479:Neuropeptides / Q000502:physiology; D000068797:Orexins; D013290:Streptococcal Infections / Q000150:complications
chemical_list: D059866:HLA-DQ beta-Chains; C069051:HLA-DQB1 antigen; D007252:Influenza Vaccines; D047908:Intracellular Signaling Peptides and Proteins; D009479:Neuropeptides; D000068797:Orexins; C568072:arepanrix; C556153:pandemrix