Skip to main content

Narcolepsy in Childhood

  • Chapter
  • First Online:
Narcolepsy

Abstract

Establishing a diagnosis of narcolepsy can be challenging in children, given the non-specific initial symptoms, limited descriptive ability of the child, and variability in reliability of the parents as historians. In the pre-school-age child, it may be hard to distinguish physiologic napping from pathologic sleepiness. In school age children, the combination of inattentiveness, trouble sleeping at night, sleepiness, and hypnagogic hallucinations can be mistaken for depression, emphasizing the need for a thorough history. Sleep logs, nocturnal polysomnography, the multiple sleep latency test (that sometimes needs to be repeated serially over months when the diagnosis is uncertain), and cerebrospinal fluid hypocretin analysis are key diagnostic studies. Management strategies include regularizing sleep–wake schedules, taking planned daytime naps to dissipate sleepiness, and exercising regularly. Parents should monitor the child closely for symptoms of depression. Counseling for emotional support is also suggested. Pharmacotherapy should target the symptom(s) most bothersome to the child. Drug treatment of daytime sleepiness, cataplexy, sleep fragmentation, and comorbid depression is with agents that are used on an “off-label” basis and are, at best, only modestly effective.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 199.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Westphal C. (1877) Eigenthu¨mliche mit Einschlafen verbundene Anfa¨lle. Arch f. Psychiat 7: 631–635.

    Google Scholar 

  2. Gélineau J. (1880) De la narcolepsie. Gaz Hosp (Paris) 53: 626–628.

    Google Scholar 

  3. Schenk CH, Bassetti CL, Arnulf I, Mignot E. (2007) English translations of the first clinical reports on narcolepsy by Gélineau and on cataplexy by Westphal in the late 19th century, with commentary. Bassetti CL, Billiard M, Mignot E (eds): Narcolepsy and Hypersomnia. New York, Informa Healthcare USA, Inc., pp 7–24.

    Google Scholar 

  4. Daniels LE. (1934) Narcolepsy. Medicine 13(1): 1–122.

    Google Scholar 

  5. Adie, W. (1928) Idiopathic narcolepsy: a disease sui generic, with remarks on the mechanisms of sleep. Brain 49: p. 257–306

    Article  Google Scholar 

  6. American Academy of Sleep Medicine. (2005). The international classification of sleep disorders: diagnostic and coding manual (2nd ed). Westchester, IL: American Academy of Sleep Medicine, xviii, 297 pp

    Google Scholar 

  7. Hublin C, Partinen M, Kaprio J, Kosekenvuo M, et al. (1994) Epidemiology of narcolepsy. Sleep 17 (Suppl 1): 7–12.

    Google Scholar 

  8. Honda Y. (1979) Census of narcolepsy, cataplexy and sleep life among teenagers in Fujisawa city. Sleep Res 8: 191.

    Google Scholar 

  9. Han F, Chen E, Wei H, Dong X, He Q, Ding D, Strohl KP. (2001) Childhood narcolepsy in North China. Sleep 24 (3): 321–324.

    CAS  PubMed  Google Scholar 

  10. Silber M, Krahn L, Olson E, Pandranz V. (2002) The epidemiology of narcolepsy in Olmstead County, Minnesota: a population-based study. Sleep 25: 197–202.

    PubMed  Google Scholar 

  11. Guilleminault C, Mignot E, Partinen M. (1994) Narcolepsy: diagnosis and epidemiology. Controversies in the diagnosis of narcolepsy. Sleep 17: S1–S6.

    CAS  PubMed  Google Scholar 

  12. Longstreth WT, Koepsell TD, Ton TG, et al. (2007) The epidemiology of narcolepsy. Sleep 30(1): 13–26.

    PubMed  Google Scholar 

  13. Silber MH, Krahn LE, Slocumb NL. (2005) Distribution of age of onset of narcolepsy. Sleep 28: A22.

    Google Scholar 

  14. Dauvilliers Y, Montplasier J, Molinari N, et al. (2001) Age of onset of narcolepsy in two large populations of patients in France and Quebec. Neurology 57: 2029–2033.

    CAS  PubMed  Google Scholar 

  15. Yoss RE, Daly DD. (1960) Narcolepsy in children. Pediatrics 25: 1025–1033.

    CAS  PubMed  Google Scholar 

  16. Challamel MJ, Mazzola ME, Nevsimalova S, et al. (1994) Narcolepsy in children. Sleep 17: S17–S20.

    CAS  PubMed  Google Scholar 

  17. Nevsimalova S, Roth B, Zouhar A, Zemanova H. (1986) Narkolepsie-kataplexie a periodicka hypersomnie se zacatkem v kojeneckem veku. Cs Pediatr 41: 324–327.

    CAS  Google Scholar 

  18. Nevsimalova S. (2007) Narcolepsy in children and adolescents. Bassetti CL, Billiard M, Mignot E (eds): Narcolepsy and Hypersomnia. New York: Informa Healthcare USA, pp 67–75.

    Google Scholar 

  19. Sharp SJ, D’Cruz OF. (2001) Narcolepsy in a 12 month old boy. J Child Neurol 16: 145–146.

    CAS  PubMed  Google Scholar 

  20. Pearl PL, Efron L, Stein MA. (2002) Children, sleep and behavior: a complex association. Minerva Pediatr 54: 79–91.

    CAS  PubMed  Google Scholar 

  21. Kotagal S, Hartse KM, Walsh JK. (1990) Characteristics of narcolepsy in pre-teenaged children. Pediatrics 85: 205–209.

    CAS  PubMed  Google Scholar 

  22. Navalet Y, Anders TF, Guilleminault C. (1976) Narcolepsy in children. In Guilleminault C, Dement WC, Passouant P (eds): Narcolepsy. New York: Spectrum, pp 171–177.

    Google Scholar 

  23. Dahl RE, Pelham WB, Wierson MC. (1991) The role of sleep disturbance in attention deficit disorder symptomatology. J Pediatr Psychol 16: 229–239.

    Article  CAS  PubMed  Google Scholar 

  24. Teixeira VG, Faccenda JF, Douglas NJ. (2004) Functional status in patients with narcolepsy. Sleep Med 5: 477–483.

    Article  PubMed  Google Scholar 

  25. Stores G, Montgomery P, Wiggs L. (2007) The psychosocial problems of children with narcolepsy and those with excessive daytime sleepiness of uncertain origin. Pediatrics 118: e1116–e1123.

    Article  Google Scholar 

  26. Guilleminault C, Wilson RA, Dement WC. (1974) A study of cataplexy. Arch Neurology 31: 255–261.

    CAS  PubMed  Google Scholar 

  27. Guilleminault C, Pelayo R. (1998) Narcolepsy in prepubertal children. Ann Neurol 43: 135–142.

    Article  CAS  PubMed  Google Scholar 

  28. Kotagal S. ( 2005) Narcolepsy in childhood. In Sheldon SH, Ferber R, Kryger MH (eds): Principles and Practice of Pediatric Sleep Medicine. Philadelphia, PA: Elsevier, 171–182.

    Google Scholar 

  29. Young D, Zorick F, Wittig R, et al. (1988) Narcolepsy in a pediatric population. Am J Dis Child 142: 210–214.

    CAS  PubMed  Google Scholar 

  30. Henry GK, Satz P, Heilbronner RL. (1993) Evidence of a perceptual encoding deficit in narcolepsy. Sleep 16: 123–127.

    CAS  PubMed  Google Scholar 

  31. Juji T, Satake M, Honda Y, Doi Y. (1984) HLA antigens in Japanese patients with narcolepsy. Tissue Antigens 24: 316–319.

    Article  CAS  PubMed  Google Scholar 

  32. Mignot E, Lin L, Rogers W, et al. (2001) Complex HLA-DR and -DQ interactions confer risk of narcolepsy-cataplexy in three ethnic groups. Am J Hum Genet 68: 686–699.

    Article  CAS  PubMed  Google Scholar 

  33. Pelin Z, Guilleminault C, Risch N, et al. (1998) HLA DQB1*0602 homozygosity increase relative risk for narcolepsy but not disease severity in two ethnic groups. Tissue Antigens 51: 96–100.

    Article  CAS  PubMed  Google Scholar 

  34. Nishino S, Ripley B, Overem S, et al. (2000) Hypocretin (orexin) deficiency in human narcolepsy. Lancet 355: 39–40.

    Article  CAS  PubMed  Google Scholar 

  35. Nishino S, Ripley B, Overeem S, et al. (2001) Low cerebrospinal fluid hypocretin (orexin) and altered energy homeostasis in human narcolepsy. Ann Neurol 50: 381–388.

    Article  CAS  PubMed  Google Scholar 

  36. Krahn LE, Pankrantz VS, Oliver L, et al. (2002) Hypocretin (orexin) levels in cerebrospinal fluid of patients with narcolepsy: Relationship to cataplexy and DQB1*0602. Sleep 25: 733–738.

    PubMed  Google Scholar 

  37. Thannickal TC, Moore RY, Nienhuis R, et al. (2000) Reduced number of hypocretin neurons in human narcolepsy. Neuron 27: 469–474.

    Article  CAS  PubMed  Google Scholar 

  38. Mignot E. (1998) Genetic and familial aspects of narcolepsy. Neurology 50 (Suppl 1): S16–S22.

    CAS  PubMed  Google Scholar 

  39. Partinen M, Hbulin C, Kaprio J, Koskenvuo M, Guilleminault C. (1994) Twin studies in narcolepsy. Sleep 17: S13–S16.

    CAS  PubMed  Google Scholar 

  40. Kaprio J, Hublin C, Partinen M, Heikkila K, Koskenvuo M. (1996) Narcolepsy-like symptoms among adult twins. J Sleep Res 5(1): 55–60.

    Article  CAS  PubMed  Google Scholar 

  41. Honda M, Honda Y, Uchida S, Miyazaki S, Tokunaga K. (2001) Monozygotic twins incompletely concordant for narcolepsy. Biol Psychiatry 49: 943–947.

    Article  CAS  PubMed  Google Scholar 

  42. Khatami R, Maret S, Werth E, et al. (2004) A monozygotic twin pair concordant for narcolepsy-cataplexy without any detectable abnormality in the hypocretin (orexin) pathway. Lancet 363: 1199–1200.

    Article  CAS  PubMed  Google Scholar 

  43. Orellana C, Villemin E, Tafti M, et al. (1994) Life events in the year preceding the onset of narcolepsy. Sleep 17 (suppl 1): 50–53.

    Google Scholar 

  44. Billiard M, Orellana C. (2007) Environmental Factors in Narcolepsy. Bassetti CL, Billiard M, Mignot E (eds): Narcolepsy and Hypersomnia. New York, New York, Informa Healthcare USA, pp 443–450.

    Google Scholar 

  45. Guilleminault C, Mignot E, Grumet FC. (1989) Familial patterns of narcolepsy. Lancet 334: 8676: 1376–1379.

    Article  Google Scholar 

  46. Autret A, Lucas F, Henry-Lebras F, et al. (1994) Symptomatic narcolepsies. Sleep 17S (Suppl 1): 21–24.

    Google Scholar 

  47. Tridon P, Montaut J, Picard L, et al. (1969) Syndrome de Gélineau et hemangioblastome kystique u cervelet. Rev Neurol 121: 186–189.

    CAS  PubMed  Google Scholar 

  48. Onofrj M, Curatola L, Ferracci F, Fulgente T. (1992) Narcolepsy associated with primary temporal lobe B-cell lymphoma in a HLA DR2 negative subject. J Neurol Neurosurg Psychiatry 55: 852–853.

    Article  CAS  PubMed  Google Scholar 

  49. Schwartz WJ, Stakes JW, Hobson JA. (1984) Transient cataplexy after removal of craniopharyngioma. Neurology 34: 1372–1375.

    CAS  PubMed  Google Scholar 

  50. Lankford DA, Wellman JJ, Ohara C. (1994) Post-traumatic narcolepsy in mild to moderate closed head injury. Sleep 17S(Suppl 1): 25–28.

    Google Scholar 

  51. Bonduelle C, Degos C. (1976) Symptomatic narcolepsies: A critical study. In Guilleminault C, Dement WC, Passouant P (eds): Narcolepsy. New York: Spectrum, pp 312–332.

    Google Scholar 

  52. Rivera VM, Meyer JS, Hata T, et al. (1986) Narcolepsy following cerebral ischemia. Ann Neurol 19: 505–508.

    Article  CAS  PubMed  Google Scholar 

  53. Schrader H, Gotlibsen OB. (1980) Multiple sclerosis and narcolepsy-cataplexy in a monozygotic twin. Neurology 30: 105–108.

    CAS  PubMed  Google Scholar 

  54. Kotagal S, Krahn LE, Slocumb N. (2004) A putative link between childhood narcolepsy and obesity. Sleep Med 5(2): 147–150.

    Article  PubMed  Google Scholar 

  55. Dahl RE, Holttum J, Trubnick L. (1994) A clinical picture of child and adolescent narcolepsy. J Am Acad Child and Adol Psych 33(6): 834–841.

    Article  CAS  PubMed  Google Scholar 

  56. Carskadon MA. (1982) The second decade. In Guilleminault C (ed): Sleeping and Waking Disorders: Indications and Techniques. Menlo Park, Calif: Addison-Wesley, 99–125.

    Google Scholar 

  57. Hoban TF, Chervin RD. (2001) Assessment of sleepiness in children. Semin Pediatr Neurol 8(4): 216–228.

    Article  CAS  PubMed  Google Scholar 

  58. Maldonado CC, Bentley AJ, Mitchell D. (2004) A pictoral sleepiness scale based on cartoon faces. Sleep 27(3): 541–548.

    PubMed  Google Scholar 

  59. Spilsbury JC, Drotar D, Rosen CL, et al. (2007) The Cleveland adolescent sleepiness questionnaire: a new measure to assess excessive daytime sleepiness in adolescents. J Clin Sleep Med 3(6): 603–612.

    PubMed  Google Scholar 

  60. Wolfson AR, Carskadon MA. (1998) Sleep schedules and daytime functioning in adolescents. Child Dev 69(4): 875–887.

    CAS  PubMed  Google Scholar 

  61. Drake C, Nickel C, Burduvali E, et al. (2003) The pediatric daytime sleepiness scale (PDSS): Sleep habits and school outcomes in middle-school children. Sleep 26(4): 455–458.

    PubMed  Google Scholar 

  62. Kotagal S, Goulding PM. (1996) The laboratory assessment of daytime sleepiness in childhood. J Clin Neurophysiol 13: 208–218.

    Article  CAS  PubMed  Google Scholar 

  63. Kotagal S. (2000) A developmental perspective on narcolepsy. In Loughlin GM, Carrol JL, Marcus CL (eds): Sleep and Breathing in Children. New York: Marcel Dekker, 347–362.

    Google Scholar 

  64. Carskadon MA, Dement WC, Mitler MM, et al. (1986) Guidelines for the MSLT: a standard measure of sleepiness. Sleep 9: 519–524.

    CAS  PubMed  Google Scholar 

  65. Aldrich MS, Chervin RD, Malow BA. (1997) Value of the multiple sleep latency test (MSLT) for the diagnosis of narcolepsy. Sleep 20(8): 620–629.

    CAS  PubMed  Google Scholar 

  66. Kotagal S, Swink TD. (1996) Excessive daytime sleepiness in a 13-year-old. Semin Pediatr Neurol 3: 170–172.

    Article  CAS  PubMed  Google Scholar 

  67. Kanbayashi T, Yano T, Ishiguro H, et al. (2002) Hypocretin (orexin) levels in human lumbar CSF in different age groups: infants to elderly persons. Sleep 25: 337–339.

    PubMed  Google Scholar 

  68. Kubota H, Kanbayashi T, Tanabe Y, et al. (2003) Decreased cerebrospinal fluid hypocretin-1 levels near the onset of narcolepsy in 2 prepubertal children. Sleep 26: 555–557.

    PubMed  Google Scholar 

  69. Brown LW, Billiard M. (1995) Narcolepsy, Klein-Levin syndrome, and other causes of sleepiness in children. In Ferber R, Kryger M (eds): Principles and Practice of Sleep Medicine in the Child. Philadelphia: WB Saunders, pp 125–134.

    Google Scholar 

  70. Carskadon MA. (2002) Factors influencing sleep patterns of adolescents. In Carskadon MA (ed): Adolescent Sleep Patterns: Biological, Social, and Psychological Perspectives. Cambridge, MA: Cambridge University Press, pp 18–19.

    Chapter  Google Scholar 

  71. Pollack CP. (1995). The rhythms of narcolepsy. Narcolepsy Netw 8: 1–7

    Google Scholar 

  72. Anderson KN, Pilsworth S, Sharples LD, et al. Idiopathic hypersomnia: a study of 77 cases. Sleep 2007; 30(10): 1274–1281.

    PubMed  Google Scholar 

  73. Dauvilliers Y, Mayer G, Lecendreux M, et al. Kleine Levin syndrome: an autoimmune hypothesis based on clinical and genetic analyses. Neurology 2002; 59: 1739–1745

    CAS  PubMed  Google Scholar 

  74. Patterson MC. (2003) A riddle wrapped in a mystery: understanding Niemann-Pick disease, type C. Neurologist 9 (6): 301–10.

    Article  PubMed  Google Scholar 

  75. Vossler DG, Wyler AR, Wilkus RJ, Gardner-Walker G, Vlcek BW. (1996) Cataplexy and monoamine oxidase deficiency in Norrie disease. Neurology 46(5): 1258–1261

    CAS  PubMed  Google Scholar 

  76. Fryns JP. (1998) “Cataplexy” in Coffin Lowry syndrome. J Med Genet 35: 702.

    Article  CAS  PubMed  Google Scholar 

  77. Garma L, Marchand F. (1994) Non-pharmacological approaches to the treatment of narcolepsy. Sleep 17(Suppl 1): 97–102.

    Google Scholar 

  78. Broughton R, Ghanem Q, Hishikawa Y, et al. (1981) Life effects of narcolepsy in 180 patients from North-America, Asia, and Europe compared to matched controls. Can J of Neurol Sci 8(4): 299–304.

    CAS  PubMed  Google Scholar 

  79. Leon-Munoz L, de la Calzada MD, Guitart M. (2000) Accident prevalence in a group of patients with the narcolepsy-cataplexy syndrome. Rev Neurol 30(6): 596–598.

    CAS  PubMed  Google Scholar 

  80. Wise MS, Arand DL, Auger RR, et al. (2007) Treatment of narcolepsy and other hypersomnias of central origin. Sleep 30: 1712–1727.

    PubMed  Google Scholar 

  81. Littner M, Johnson SF, McCall VW, et al. (2001) Practice parameters for treatment of narcolepsy: An update for 2000. Sleep 24: 451–466.

    CAS  PubMed  Google Scholar 

  82. Stahl SM. (2002) Awakening to the psychopharmacology of sleep and arousal: novel neurotransmitters and wake-promoting drugs. J Clin Psychiatr 63: 467–468.

    Google Scholar 

  83. Mitler MM. (1994) Evaluation of treatment with stimulants in narcolepsy. Sleep 17 (Suppl 1): 103–106.

    Google Scholar 

  84. Billiard M, Besset A, Montplasier F, et al. (1994) Modafanil: a double blind multicentric study. Sleep 17 (Suppl 1): 107–112.

    Google Scholar 

  85. The US Xyrem Multicenter Study Group (2002) A randomized, double blind, placebo controlled multicenter trial comparing the effects of three doses of orally administered sodium oxybate with placebo for the treatment of narcolepsy. Sleep 25: 42–49.

    Google Scholar 

  86. Ivanenko A, Tauman R, Gozal D. (2003) Modafanil in the treatment of excessive daytime sleepiness in children. Sleep Med 4: 579–582.

    Article  PubMed  Google Scholar 

  87. Dauvilliers Y, Nevsimalova S, Lecendreux M, et al. (2007) Modafinil reduces symptoms of excessive sleepiness in children and adolescents with obstructive sleep apnea or narcolepsy following 6 months of open-label therapy. Sleep 30(Suppl): A218.

    Google Scholar 

  88. Owens J, Bogan R, Lankford A, et al. (2007) Modafinil is well tolerated in children and adolescents with excessive sleepiness and obstructive sleep apnea: A 6-week double-blind study. Sleep 30: (Suppl) A88–A89.

    Google Scholar 

  89. Black J, Houghton WC. (2006) Sodium oxybate improves excessive daytime sleepiness in narcolepsy. Sleep 29: 939–946.

    PubMed  Google Scholar 

  90. Murali H, Kotagal S. (2006) Off-label treatment of severe childhood narcolepsy-cataplexy with sodium oxybate. Sleep 29(8): 1025–1029.

    PubMed  Google Scholar 

  91. Lecendreaux M, Maret S, Bassetti C, et al. (2003) Clinical efficacy of high-dose intravenous immunoglobulins near the onset of narcolepsy in a 10-year-old boy. J Sleep Res 12(4): 347–348.

    Google Scholar 

  92. Hecht M, Lin L, Kushida CA, et al. (2003) Report of a case of immunosuppression with prednisone in an 8-year-old boy with an acute onset of hypocretin-deficiency narcolepsy. Sleep 26(7): 809–810.

    PubMed  Google Scholar 

  93. Dauvilliers Y, Carlander B, Rivier F, et al. (2004) Successful management of cataplexy with intravenous immunoglobulins at narcolepsy onset. Ann Neurol 56(6): 905–908.

    Article  CAS  PubMed  Google Scholar 

  94. Honda Y. (1997) A 10–40 year follow-up study of narcolepsy. Conference Information: Japanese/German International Symposium on Sleep-Wake Disorders, Date: OCT 09–10, 1996 Erfurt, Germany. Sleep-wake Disorders, pp 105–114.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2010 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Kotagal, S., Paruthi, S. (2010). Narcolepsy in Childhood. In: Goswami, M., Pandi-Perumal, S., Thorpy, M. (eds) Narcolepsy. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-0854-4_5

Download citation

  • DOI: https://doi.org/10.1007/978-1-4419-0854-4_5

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4419-0853-7

  • Online ISBN: 978-1-4419-0854-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics