Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Narcolepsy in children

Suresh Kotagal, MD
Section Editors
Thomas E Scammell, MD
Ronald D Chervin, MD, MS
Deputy Editor
April F Eichler, MD, MPH


Narcolepsy is a chronic neurologic disorder characterized by excessive and irresistible sleepiness, cataplexy, hypnagogic hallucinations (vivid dreams at sleep onset), and sleep paralysis (a momentary inability to move the body as one is drifting off to sleep). Although relatively rare in general, it is one of the more common causes of disabling daytime sleepiness beginning in adolescence or early adulthood.

Narcolepsy can present in children as young as five or six years of age, and delays in diagnosis are common. Early-onset narcolepsy has some unique clinical features compared with later onset cases, including cataplexy with prominent buccofacial involvement and daytime sleepiness manifesting primarily as habitual napping or irritability and hyperactivity.

This topic will review the clinical features, diagnosis, and management of narcolepsy in children. Narcolepsy in adults is reviewed separately. (See "Clinical features and diagnosis of narcolepsy in adults" and "Treatment of narcolepsy in adults".)


The International Classification of Sleep Disorders, third edition (ICSD-3), recognizes two forms of narcolepsy [1]:

Type 1 (previously called narcolepsy with cataplexy) – Patients with narcolepsy type 1 generally show cataplexy along with sleepiness at the very onset of the disorder or within the first several years of the onset of sleepiness. The underlying pathophysiology is a deficiency of central nervous system hypocretin, which is a peptide essential for maintaining alertness. (See 'Pathophysiology' below.)


Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Sep 2016. | This topic last updated: Jul 1, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
  1. American Academy of Sleep Medicine. International Classification of Sleep Disorders, 3rd ed, American Academy of Sleep Medicine, Darien, IL 2014.
  2. Challamel MJ, Mazzola ME, Nevsimalova S, et al. Narcolepsy in children. Sleep 1994; 17S:17.
  3. Wijnans L, Lecomte C, de Vries C, et al. The incidence of narcolepsy in Europe: before, during, and after the influenza A(H1N1)pdm09 pandemic and vaccination campaigns. Vaccine 2013; 31:1246.
  4. Longstreth WT Jr, Koepsell TD, Ton TG, et al. The epidemiology of narcolepsy. Sleep 2007; 30:13.
  5. Szakács A, Darin N, Hallböök T. Increased childhood incidence of narcolepsy in western Sweden after H1N1 influenza vaccination. Neurology 2013; 80:1315.
  6. Feltelius N, Persson I, Ahlqvist-Rastad J, et al. A coordinated cross-disciplinary research initiative to address an increased incidence of narcolepsy following the 2009-2010 Pandemrix vaccination programme in Sweden. J Intern Med 2015; 278:335.
  7. Sturkenboom MC. The narcolepsy-pandemic influenza story: can the truth ever be unraveled? Vaccine 2015; 33 Suppl 2:B6.
  8. Ahmed SS, Volkmuth W, Duca J, et al. Antibodies to influenza nucleoprotein cross-react with human hypocretin receptor 2. Sci Transl Med 2015; 7:294ra105.
  9. Nishino S, Ripley B, Overeem S, et al. Hypocretin (orexin) deficiency in human narcolepsy. Lancet 2000; 355:39.
  10. Thannickal TC, Moore RY, Nienhuis R, et al. Reduced number of hypocretin neurons in human narcolepsy. Neuron 2000; 27:469.
  11. Kotagal S, Krahn LE, Slocumb N. A putative link between childhood narcolepsy and obesity. Sleep Med 2004; 5:147.
  12. Hara J, Beuckmann CT, Nambu T, et al. Genetic ablation of orexin neurons in mice results in narcolepsy, hypophagia, and obesity. Neuron 2001; 30:345.
  13. Autret A, Lucas B, Henry-Lebras F, de Toffol B. Symptomatic narcolepsies. Sleep 1994; 17:S21.
  14. Vankova J, Stepanova I, Jech R, et al. Sleep disturbances and hypocretin deficiency in Niemann-Pick disease type C. Sleep 2003; 26:427.
  15. Kandt RS, Emerson RG, Singer HS, et al. Cataplexy in variant forms of Niemann-Pick disease. Ann Neurol 1982; 12:284.
  16. Manni R, Politini L, Nobili L, et al. Hypersomnia in the Prader Willi syndrome: clinical-electrophysiological features and underlying factors. Clin Neurophysiol 2001; 112:800.
  17. Vossler DG, Wyler AR, Wilkus RJ, et al. Cataplexy and monoamine oxidase deficiency in Norrie disease. Neurology 1996; 46:1258.
  18. Kotagal S, Hartse KM, Walsh JK. Characteristics of narcolepsy in preteenaged children. Pediatrics 1990; 85:205.
  19. Babiker MO, Prasad M. Narcolepsy in children: a diagnostic and management approach. Pediatr Neurol 2015; 52:557.
  20. Drake C, Nickel C, Burduvali E, et al. The pediatric daytime sleepiness scale (PDSS): sleep habits and school outcomes in middle-school children. Sleep 2003; 26:455.
  21. Yang CM, Huang YS, Song YC. Clinical utility of the Chinese version of the Pediatric Daytime Sleepiness Scale in children with obstructive sleep apnea syndrome and narcolepsy. Psychiatry Clin Neurosci 2010; 64:134.
  22. Kotagal S, Nichols CD, Grigg-Damberger MM, et al. Non-respiratory indications for polysomnography and related procedures in children: an evidence-based review. Sleep 2012; 35:1451.
  23. Serra L, Montagna P, Mignot E, et al. Cataplexy features in childhood narcolepsy. Mov Disord 2008; 23:858.
  24. Prasad M, Setty G, Ponnusamy A, et al. Cataplectic facies: clinical marker in the diagnosis of childhood narcolepsy-report of two cases. Pediatr Neurol 2014; 50:515.
  25. Pizza F, Franceschini C, Peltola H, et al. Clinical and polysomnographic course of childhood narcolepsy with cataplexy. Brain 2013; 136:3787.
  26. Stores G, Montgomery P, Wiggs L. The psychosocial problems of children with narcolepsy and those with excessive daytime sleepiness of uncertain origin. Pediatrics 2006; 118:e1116.
  27. Avis KT, Shen J, Weaver P, Schwebel DC. Psychosocial Characteristics of Children with Central Disorders of Hypersomnolence Versus Matched Healthy Children. J Clin Sleep Med 2015; 11:1281.
  28. Poli F, Pizza F, Mignot E, et al. High prevalence of precocious puberty and obesity in childhood narcolepsy with cataplexy. Sleep 2013; 36:175.
  29. Harris SF, Monderer RS, Thorpy M. Hypersomnias of central origin. Neurol Clin 2012; 30:1027.
  30. Reiter J, Katz E, Scammell TE, Maski K. Usefulness of a Nocturnal SOREMP for Diagnosing Narcolepsy with Cataplexy in a Pediatric Population. Sleep 2015; 38:859.
  31. Lloyd R, Tippmann-Peikert M, Slocumb N, Kotagal S. Characteristics of REM sleep behavior disorder in childhood. J Clin Sleep Med 2012; 8:127.
  32. Carskadon MA, Dement WC, Mitler MM, et al. Guidelines for the multiple sleep latency test (MSLT): a standard measure of sleepiness. Sleep 1986; 9:519.
  33. American Academy of Sleep Medicine. International Classification of Sleep Disorders, 3rd ed, American Academy of Sleep Medicine, Darien, IL 2014.
  34. Hoban TF, Chervin RD. Assessment of sleepiness in children. Semin Pediatr Neurol 2001; 8:216.
  35. Bourgin P, Zeitzer JM, Mignot E. CSF hypocretin-1 assessment in sleep and neurological disorders. Lancet Neurol 2008; 7:649.
  36. Morgenthaler TI, Kapur VK, Brown T, et al. Practice parameters for the treatment of narcolepsy and other hypersomnias of central origin. Sleep 2007; 30:1705.
  37. Aran A, Einen M, Lin L, et al. Clinical and therapeutic aspects of childhood narcolepsy-cataplexy: a retrospective study of 51 children. Sleep 2010; 33:1457.
  38. Lecendreux M. Pharmacological management of narcolepsy and cataplexy in pediatric patients. Paediatr Drugs 2014; 16:363.
  39. Lecendreux M, Bruni O, Franco P, et al. Clinical experience suggests that modafinil is an effective and safe treatment for paediatric narcolepsy. J Sleep Res 2012; 21:481.
  40. Billiard M, Bassetti C, Dauvilliers Y, et al. EFNS guidelines on management of narcolepsy. Eur J Neurol 2006; 13:1035.
  41. Alshaikh MK, Tricco AC, Tashkandi M, et al. Sodium oxybate for narcolepsy with cataplexy: systematic review and meta-analysis. J Clin Sleep Med 2012; 8:451.
  42. Murali H, Kotagal S. Off-label treatment of severe childhood narcolepsy-cataplexy with sodium oxybate. Sleep 2006; 29:1025.
  43. Vendrame M, Havaligi N, Matadeen-Ali C, et al. Narcolepsy in children: a single-center clinical experience. Pediatr Neurol 2008; 38:314.
  44. Lecendreux M, Poli F, Oudiette D, et al. Tolerance and efficacy of sodium oxybate in childhood narcolepsy with cataplexy: a retrospective study. Sleep 2012; 35:709.
  45. Møller LR, Østergaard JR. Treatment with venlafaxine in six cases of children with narcolepsy and with cataplexy and hypnagogic hallucinations. J Child Adolesc Psychopharmacol 2009; 19:197.
  46. Ratkiewicz M, Splaingard M. Treatment of cataplexy in a three-year-old using venlafaxine. J Clin Sleep Med 2013; 9:1341.
  47. Valko PO, Khatami R, Baumann CR, Bassetti CL. No persistent effect of intravenous immunoglobulins in patients with narcolepsy with cataplexy. J Neurol 2008; 255:1900.
  48. Knudsen S, Biering-Sørensen B, Kornum BR, et al. Early IVIg treatment has no effect on post-H1N1 narcolepsy phenotype or hypocretin deficiency. Neurology 2012; 79:102.
  49. Lin JS, Dauvilliers Y, Arnulf I, et al. An inverse agonist of the histamine H(3) receptor improves wakefulness in narcolepsy: studies in orexin-/- mice and patients. Neurobiol Dis 2008; 30:74.
  50. Leu-Semenescu S, Nittur N, Golmard JL, Arnulf I. Effects of pitolisant, a histamine H3 inverse agonist, in drug-resistant idiopathic and symptomatic hypersomnia: a chart review. Sleep Med 2014; 15:681.
  51. Weinhold SL, Seeck-Hirschner M, Nowak A, et al. The effect of intranasal orexin-A (hypocretin-1) on sleep, wakefulness and attention in narcolepsy with cataplexy. Behav Brain Res 2014; 262:8.