title: Narcolepsy with cataplexy and comorbid immunopathological diseases.
abstract: Evidence suggests that autoimmune diseases tend to co-occur so that patients with an autoimmune disorder are at higher risk of a second autoimmune disease. The association between allergic and autoimmune diseases is also of considerable interest. There are no reports on the association between sporadic or familial narcolepsy with cataplexy and other non-neurological immune-mediated diseases. This study reported on the comorbid immunopathological diseases associated with narcolepsy. One-hundred and fifty six narcoleptic patients with a mean age at diagnosis of 39.1 ± 17.8 years (range, 6-70 years) were assessed using the clinical history, physical and neurological examinations, sleep questionnaires, neuroimaging and human leucocyte antigen typing. Diagnosis was confirmed by polysomnography followed by a multiple sleep latency test or by measuring hypocretin-1 levels. Patients with immunopathological diseases were matched for gender and age at the onset of narcoleptic symptoms with narcoleptic patients without immunopathological diseases. Twenty-six patients (16.6%; 50% women; one familial, 25 sporadic) had one or more immunopathological diseases associated: autoimmune diseases, such as idiopathic thrombocytopenic purpura, multiple sclerosis, systemic lupus erythematosus, psoriasis, Crohn's disease, ulcerative colitis, autoimmune thyroid disease, Peyronie's disease and idiopathic recurrent facial palsy; other immunopathological diseases, like atopic dermatitis, allergic asthma and allergic rhinitis. Although not significant, the age at diagnosis of narcolepsy was 9.3 years earlier in patients with narcolepsy + immunopathological diseases. The results demonstrate that the prevalence of comorbid immunopathological diseases is high in narcolepsy, and cataplexy is significantly more severe in patients with narcolepsy + immunopathological diseases.
authors.affiliation: Sleep Unit, Clinical Neurophysiology Service, San Carlos University Hospital, Madrid, Spain.
authors.forename: Francisco J
authors.initials: FJ
authors.lastname: Martínez-Orozco
authors.forename: José L
authors.initials: JL
authors.lastname: Vicario
authors.forename: Isabel
authors.initials: I
authors.lastname: Villalibre-Valderrey
authors.forename: Clara
authors.initials: C
authors.lastname: De Andrés
authors.forename: Miguel
authors.initials: M
authors.lastname: Fernández-Arquero
authors.forename: Rosa
authors.initials: R
authors.lastname: Peraita-Adrados
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D001327:Autoimmune Diseases / Q000453:epidemiology*; D002385:Cataplexy / Q000175:diagnosis / Q000453:epidemiology*; D002648:Child; D015897:Comorbidity; D005260:Female; D006801:Humans; D006967:Hypersensitivity / Q000453:epidemiology*; D047908:Intracellular Signaling Peptides and Proteins / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D009479:Neuropeptides / Q000378:metabolism; D000068797:Orexins; D017286:Polysomnography; D012894:Sleep Stages; D055815:Young Adult
chemical_list: C000596229:HCRT protein, human; D047908:Intracellular Signaling Peptides and Proteins; D009479:Neuropeptides; D000068797:Orexins
keywords: comorbidity; diagnostic delay; immunopathological diseases; narcolepsy; severity of cataplexy