Narcolepsy Treatment & Management

  • Author: Ali M Bozorg, MD; Chief Editor: Selim R Benbadis, MD   more...
 
Updated: Feb 19, 2010
 

Medical Care

  • Nonpharmacologic treatment
    • Sleep hygiene is important. Most patients improve if they maintain a regular sleep schedule, usually 7.5-8 hours of sleep per night.
    • Scheduled naps during the day also may help.[11]
    • Provide emotional support and career/vocational counseling to the patient and parent.
    • Assist with documentation for special academic needs, insurance, disability forms, and attaining a driver's license.
    • Question patients about high-risk behaviors such as alcohol and drug use, which may exacerbate symptoms.
    • Inquiries into depression, family conflict, and other psychosocial problems are also important.
    • Encourage children to participate in after-school activities and sports. A well-designed exercise program can be beneficial and stimulating. School personnel should have the child with narcolepsy refrain from activities if he or she appear drowsy.
    • Avoidance of foods high in refined sugars may improve daytime sleepiness.
  • Pharmacologic treatment
    • CNS stimulants such as methylphenidate, dextroamphetamine sulfate, methamphetamine, and amphetamine are used for treatment of narcolepsy.
      • Older stimulants are thought to act primarily through brainstem dopamine, nigrostriatal, and mesocorticolimbic pathways. These medications help reduce daytime sleepiness, improving the symptom in 65-85% of patients.
      • Methylphenidate, the most frequently used stimulant, improves sleep tendency in a dose-related fashion.
      • Undesirable side effects include headache, irritability, nervousness, and gastrointestinal complaints.
      • Nocturnal sleep may be impaired, thus decreasing total sleep time.
    • Modafinil is a novel wake-promoting agent.[12]
      • The mechanism of action is not understood, but it does not appear to alter levels of dopamine or norepinephrine.
      • Unlike traditional medications, modafinil does not appear to affect total sleep time or suppress REM sleep.
      • The most common adverse effect is headache. Its safety in children has not been established.
    • Armodafinil (Nuvigil)[13] is an enantiomer of modafinil with fewer side effects.
      • The mechanism of action of armodafinil is poorly understood.
      • Armodafinil is indicated for the treatment of excessive daytime somnolence associated with narcolepsy.
      • The most common adverse effects are headache, nausea, dizziness, and difficulty sleeping. Its safety has not been established in children younger than 17 years.
    • Sodium oxybate (Xyrem)[14, 15] is the only FDA-approved treatment for cataplexy. It is also used to treat EDS. Sodium oxybate is a CNS depressant and should not be used with alcohol or other CNS depressants.
    • Tricyclic antidepressants and selective serotonin reuptake inhibitors (SSRI) have also been used to treat cataplexy.
    • Currently, no FDA-approved pharmacotherapy is available for children with narcolepsy. However, the medications used to treat narcolepsy in adults have been used off-label in the pediatric population with positive results.
Next

Consultations

A child in whom narcolepsy is suspected must be evaluated by a pediatric neurologist. Further evaluation at a sleep disorders clinic is also imperative.

Previous
Next

Diet

Patients with narcolepsy should avoid heavy meals and alcohol.

Previous
Next

Activity

  • Recommendations
    • Scheduled short naps
    • Exercise program
    • Restrict driving when sleepy (Patients with narcolepsy should avoid driving or operating heavy machinery when sleepy.)
Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Ali M Bozorg, MD  Assistant Professor, Comprehensive Epilepsy Program, Department of Neurology, University of South Florida

Ali M Bozorg, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, and American Epilepsy Society

Disclosure: Nothing to disclose.

Coauthor(s)

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Ortho McNeil Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Speaking, consulting

Specialty Editor Board

Carmel Armon, MD, MSc, MHS  Professor of Neurology, Tufts University School of Medicine; Chief, Division of Neurology, Baystate Medical Center

Carmel Armon, MD, MSc, MHS is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American College of Physicians, American Epilepsy Society, American Medical Association, American Neurological Association, American Stroke Association, Massachusetts Medical Society, Movement Disorders Society, and Sigma Xi

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Jose E Cavazos, MD, PhD, FAAN  Associate Professor with Tenure, Departments of Neurology, Pharmacology, and Physiology, University of Texas Health Science Center at San Antonio; Co-Director, South Texas Comprehensive Epilepsy Center; Director of the Epilepsy Center, Audie L Murphy Veterans Affairs Medical Center

Jose E Cavazos, MD, PhD, FAAN is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, American Epilepsy Society, American Neurological Association, and Society for Neuroscience

Disclosure: Nothing to disclose.

Paul E Barkhaus, MD  Professor, Department of Neurology, Medical College of Wisconsin; Director of Neuromuscular Diseases, Milwaukee Veterans Administration Medical Center

Paul E Barkhaus, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Neurological Association

Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Ortho McNeil Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Speaking, consulting

References

  1. Lin L, Faraco J, Li R, et al. The sleep disorder canine narcolepsy is caused by a mutation in the hypocretin (orexin) receptor 2 gene. Cell. Aug 6 1999;98(3):365-76. [Medline].

  2. Chemelli RM, Willie JT, Sinton CM, Elmquist JK, Scammell T, Lee C, et al. Narcolepsy in orexin knockout mice: molecular genetics of sleep regulation. Cell. Aug 20 1999;98(4):437-51. [Medline].

  3. Thannickal TC, Moore RY, Nienhuis R, Ramanathan L, Gulyani S, Aldrich M, et al. Reduced number of hypocretin neurons in human narcolepsy. Neuron. Sep 2000;27(3):469-74. [Medline].

  4. Thannickal TC, Nienhuis R, Siegel JM. Localized loss of hypocretin (orexin) cells in narcolepsy without cataplexy. Sleep. Aug 1 2009;32(8):993-8. [Medline].

  5. Nishino S, Sakurai E, Nevsimalova S, Yoshida Y, Watanabe T, Yanai K. Decreased CSF histamine in narcolepsy with and without low CSF hypocretin-1 in comparison to healthy controls. Sleep. Feb 1 2009;32(2):175-80. [Medline].

  6. Kanbayashi T, Kodama T, Kondo H, Satoh S, Inoue Y, Chiba S. CSF histamine contents in narcolepsy, idiopathic hypersomnia and obstructive sleep apnea syndrome. Sleep. Feb 1 2009;32(2):181-7. [Medline].

  7. Dauvilliers Y, Abril B, Mas E, Michel F, Tafti M. Normalization of hypocretin-1 in narcolepsy after intravenous immunoglobulin treatment. Neurology. Oct 20 2009;73(16):1333-4. [Medline].

  8. Guilleminault C, Pelayo R. Narcolepsy in prepubertal children. Ann Neurol. Jan 1998;43(1):135-42. [Medline].

  9. Melberg A, Hetta J, Dahl N, et al. Autosomal dominant cerebellar ataxia deafness and narcolepsy. J Neurol Sci. Dec 1995;134(1-2):119-29. [Medline].

  10. Vossler DG, Wyler AR, Wilkus RJ, et al. Cataplexy and monoamine oxidase deficiency in Norrie disease. Neurology. May 1996;46(5):1258-61. [Medline].

  11. Rogers AE, Aldrich MS, Lin X. A comparison of three different sleep schedules for reducing daytime sleepiness in narcolepsy. Sleep. Jun 15 2001;24(4):385-91. [Medline].

  12. Fry JM. Treatment modalities for narcolepsy. Neurology. Feb 1998;50(2 Suppl 1):S43-8. [Medline].

  13. The Nuvigil website. Available at www.nuvigil.com. Accessed 12/8/2009.

  14. Xyrem Web site. Available at www.xyrem.com.

  15. Lockrane B, Bhatia P, Gore R. Successful treatment of narcolepsy and cataplexy: A review. Can Respir J. May-Jun 2005;12(4):225-7. [Medline].

  16. Abad VC, Guilleminault C. Review of rapid eye movement behavior sleep disorders. Curr Neurol Neurosci Rep. Mar 2004;4(2):157-63. [Medline].

  17. Aldrich MS. Diagnostic aspects of narcolepsy. Neurology. Feb 1998;50(2 Suppl 1):S2-7. [Medline].

  18. Aldrich MS, Chervin RD, Malow BA. Value of the multiple sleep latency test (MSLT) for the diagnosis of narcolepsy. Sleep. Aug 1997;20(8):620-9. [Medline].

  19. Bassetti C, Aldrich MS, Quint DJ. MRI findings in narcolepsy. Sleep. Aug 1997;20(8):630-1. [Medline].

  20. Benbadis SR. Daytime sleepiness: when is it normal? When to refer?. Cleve Clin J Med. Nov-Dec 1998;65(10):543-9. [Medline].

  21. Benbadis SR. Effective treatment of narcolepsy with codeine in a patient receiving hemodialysis. Pharmacotherapy. May-Jun 1996;16(3):463-5. [Medline].

  22. Benbadis SR, Mascha E, Perry MC, et al. Association between the Epworth sleepiness scale and the multiple sleep latency test in a clinical population. Ann Intern Med. Feb 16 1999;130(4 Pt 1):289-92. [Medline]. [Full Text].

  23. Blouin AM, Thannickal TC, Worley PF, Baraban JM, Reti IM, Siegel JM. Narp immunostaining of human hypocretin (orexin) neurons: loss in narcolepsy. Neurology. Oct 25 2005;65(8):1189-92. [Medline].

  24. Choo KL, Guilleminault C. Narcolepsy and idiopathic hypersomnolence. Clin Chest Med. Mar 1998;19(1):169-81. [Medline].

  25. Dauvilliers Y, Arnulf I, Mignot E. Narcolepsy with cataplexy. Lancet. Feb 10 2007;369(9560):499-511. [Medline].

  26. Dauvilliers Y, Baumann CR, Carlander B, Bischof M, Blatter T, Lecendreux M, et al. CSF hypocretin-1 levels in narcolepsy, Kleine-Levin syndrome, and other hypersomnias and neurological conditions. J Neurol Neurosurg Psychiatry. Dec 2003;74(12):1667-73. [Medline].

  27. Douglas NJ. The psychosocial aspects of narcolepsy. Neurology. Feb 1998;50(2 Suppl 1):S27-30. [Medline].

  28. Eguchi T, Nakase H, Tsujimoto S, et al. Serial change of cerebral blood flow and intracranial pressure in status epilepticus. Jpn J Psychiatry Neurol. Jun 1992;46(2):546-8. [Medline].

  29. Gerashchenko D, Kohls MD, Greco M, et al. Hypocretin-2-saporin lesions of the lateral hypothalamus produce narcoleptic-like sleep behavior in the rat. J Neurosci. Sep 15 2001;21(18):7273-83. [Medline]. [Full Text].

  30. Gregori J, Ortuno J, Ruiz Rivas JL, Arenas M. [Brucellosis and portal thrombosis]. Rev Esp Enferm Dig. Sep 1990;78(3):187-8. [Medline].

  31. Guilleminault C, Heinzer R, Mignot E, Black J. Investigations into the neurologic basis of narcolepsy. Neurology. Feb 1998;50(2 Suppl 1):S8-15. [Medline].

  32. Han F, Chen E, Wei H, et al. Childhood narcolepsy in North China. Sleep. May 1 2001;24(3):321-4. [Medline].

  33. Larrosa O, de la Llave Y, Bario S, et al. Stimulant and anticataplectic effects of reboxetine in patients with narcolepsy: a pilot study. Sleep. May 1 2001;24(3):282-5. [Medline].

  34. Longstreth WT Jr, Koepsell TD, Ton TG, Hendrickson AF, van Belle G. The epidemiology of narcolepsy. Sleep. Jan 1 2007;30(1):13-26. [Medline].

  35. Macleod S, Ferrie C, Zuberi SM. Symptoms of narcolepsy in children misinterpreted as epilepsy. Epileptic Disord. Mar 2005;7(1):13-7. [Medline].

  36. Maeda M, Tamaoka A, Hayashi A, et al. [A case of HLA-DR2, DQw1 negative post-traumatic narcolepsy]. Rinsho Shinkeigaku. Jul 1995;35(7):811-3. [Medline].

  37. Maret S, Tafti M. Genetics of narcolepsy and other major sleep disorders. Swiss Med Wkly. Nov 19 2005;135(45-46):662-5. [Medline].

  38. Mignot E. Genetic and familial aspects of narcolepsy. Neurology. Feb 1998;50(2 Suppl 1):S16-22. [Medline].

  39. Mignot E. Genetics of narcolepsy and other sleep disorders. Am J Hum Genet. Jun 1997;60(6):1289-302. [Medline].

  40. Mignot E, Lammers GJ, Ripley B, Okun M, Nevsimalova S, Overeem S, et al. The role of cerebrospinal fluid hypocretin measurement in the diagnosis of narcolepsy and other hypersomnias. Arch Neurol. Oct 2002;59(10):1553-62. [Medline].

  41. Naumann A, Daum I. Narcolepsy: Pathophysiology and neuropsychological changes. Behav Neurol. 2003;14(3,4):89-98. [Medline].

  42. Nishino S, Ripley B, Overeem S, et al. Hypocretin (orexin) deficiency in human narcolepsy [letter] [see comments]. Lancet. Jan 1 2000;355(9197):39-40. [Medline].

  43. Nishino S, Ripley B, Overeem S, Nevsimalova S, Lammers GJ, Vankova J, et al. Low cerebrospinal fluid hypocretin (Orexin) and altered energy homeostasis in human narcolepsy. Ann Neurol. Sep 2001;50(3):381-8. [Medline].

  44. Overeem S, Black JL 3rd, Lammers GJ. Narcolepsy: immunological aspects. Sleep Med Rev. Apr 2008;12(2):95-107. [Medline].

  45. Pakola SJ, Dinges DF, Pack AI. Review of regulations and guidelines for commercial and noncommercial drivers with sleep apnea and narcolepsy. Sleep. Nov 1995;18(9):787-96. [Medline].

  46. Pelin Z, Guilleminault C, Risch N, Grumet FC, Mignot E. HLA-DQB1*0602 homozygosity increases relative risk for narcolepsy but not disease severity in two ethnic groups. US Modafinil in Narcolepsy Multicenter Study Group. Tissue Antigens. Jan 1998;51(1):96-100. [Medline].

  47. Peterson PC, Husain AM. Pediatric narcolepsy. Brain Dev. Mar 27 2008;[Medline].

  48. Plazzi G, Serra L, Ferri R. Nocturnal aspects of narcolepsy with cataplexy. Sleep Med Rev. Apr 2008;12(2):109-28. [Medline].

  49. Servan J, Marchand F, Garma L, et al. [Narcolepsy disclosing neurosarcoidosis]. Rev Neurol (Paris). Apr 1995;151(4):281-3. [Medline].

  50. Silber MH, Krahn LE, Olson EJ, Pankratz VS. The epidemiology of narcolepsy in Olmsted County, Minnesota: a population-based study. Sleep. Mar 15 2002;25(2):197-202. [Medline].

  51. US Modafinil in Narcolepsy Multicenter Study Group. Randomized trial of modafinil as a treatment for the excessive daytime somnolence of narcolepsy. Neurology. Mar 14 2000;54(5):1166-75. [Medline].

  52. Vendrame M, Havaligi N, Matadeen-Ali C, Adams R, Kothare SV. Narcolepsy in children: a single-center clinical experience. Pediatr Neurol. May 2008;38(5):314-20. [Medline].

  53. [Best Evidence] Vignatelli L, D'Alessandro R, Candelise L. Antidepressant drugs for narcolepsy. Cochrane Database Syst Rev. Jul 20 2005;CD003724. [Medline].

  54. Wise MS. Childhood narcolepsy. Neurology. Feb 1998;50(2 Suppl 1):S37-42. [Medline].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.