Narcolepsy is a life-long sleep disorders with two distinct subtypes, narcolepsy type I and narcolepsy type II. It is now well recognized that the loss of hypocretin neurons underlies pathogenesis of narcolepsy type I, however, the pathogenesis of narcolepsy type II is currently unknown. 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 especailly streptococcus and influenza have been proposed as a potential trigger for the autoimmune-mediated mechanism. Several recent studies have shown increased cases of pediatric narcolepsy following 2009 H1N1 pandemic. The increased cases in Europe seems 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 unusual presentation at disease onset including complex motor movements which may lead to delayed diagnosis. All classic narcolepsy tetrad are present in only a small proportion of children. The diagnosis of narcolepsy is confirmed by either obtaining CSF 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 suspicious of narcolepsy. For management, both pharmacologic and non-pharmacologic treatment are important in the management of narcolepsy. Pharmacotherapy is primarily aimed to address excessive daytime sleepiness and REM related 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-behavior intervention. This article is protected by copyright. All rights reserved.
This article is protected by copyright. All rights reserved.