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Both genetic and environmental factors play an important role in the pathogenesis of narcolepsy. There is increasing evidence that autoimmune processes may play a critical role in the loss of hypocretin neurons. Infections especially streptococcus and influenza have been proposed as a potential trigger for the autoimmune\u2010mediated mechanism. Several recent studies have shown increased cases of pediatric narcolepsy following the 2009 H1N1 pandemic. The increased cases in Europe seem to be related to a specific type of H1N1 influenza vaccination (Pandemrix), while the increased cases in China are related to influenza infection. Children with narcolepsy can have an unusual presentation at disease onset including complex motor movements which may lead to delayed diagnosis. All classic narcolepsy tetrads are present in only a small proportion of children. The diagnosis of narcolepsy is confirmed by either obtaining cerebrospinal fluid hypocretin or overnight sleep study with the multiple sleep latency test (MSLT). There are limitations of using MSLT in young children such that a negative MSLT test cannot exclude narcolepsy. HLA markers have limited utility in narcolepsy, but it may be useful in young children with clinical suspicion of narcolepsy. For management, both pharmacologic and non\u2010pharmacologic treatments are important in the management of narcolepsy. Pharmacotherapy is primarily aimed to address excessive daytime sleepiness and REM\u2010related symptoms such as cataplexy. In addition to pharmacotherapy, routine screening of behavioral and psychosocial issues is warranted to identify patients who would benefit from bio\u2010behavior intervention.<\/jats:p>","DOI":"10.1002\/ppul.25512","type":"journal-article","created":{"date-parts":[[2021,5,22]],"date-time":"2021-05-22T12:30:01Z","timestamp":1621686601000},"page":"1952-1962","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Narcolepsy in Children: Sleep disorders in children, A rapidly evolving field seeking consensus"],"prefix":"10.1002","volume":"57","author":[{"given":"Thomas J.","family":"Dye","sequence":"first","affiliation":[{"name":"Division of Pulmonary and Sleep Medicine Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"},{"name":"Division of Neurology Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"},{"name":"Department of Pediatrics University of Cincinnati College of Medicine  Cincinnati 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