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Narcolepsy - Highlights

Description

An in-depth report on the causes, diagnosis, and treatment of narcolepsy.

Highlights:

Genetic Basis

Nearly 98% of patients with narcolepsy and cataplexy test positive for specific human leukocyte antigen (HLA) subtypes, particularly HLA-DQB1*0602. This antigen is found in just 20% of the general population.

Having a family member with narcolepsy presents a 20 to 40 times higher risk of developing the condition compared to the general population.

Drug Treatments for Narcolepsy

Sleep regulation is a complex process and there is no single chemical responsible. The two main drug treatments for narcolepsy are:

Modafinil (Provigil) for excessive, uncontrollable, daytime sleepiness
Sodium oxybate (Xyrem) for cataplexy (sudden muscle weakness)

These medications should be used with caution and are not safe during pregnancy. Modafinil comes with potentially rare side effects and Sodium oxybate poses high abuse potential.

Lifestyle Strategies for Managing Narcolepsy

Educational counseling for patients and family members affected by the symptoms can be very helpful.
Setting alert time goals and scheduled naps can help combat narcolepsy-associated sleepiness and cataplexy attacks, and improve alertness. Patients with narcolepsy should try to take three or more short naps on a regular basis during the day, and avoid caffeine, heavy meals before bedtime, and alcohol.
Patients often benefit from a combined lifestyle and pharmacotherapy treatment regimen.

Resources

www.aasmnet.org -- American Academy of Sleep Medicine
www.sleepfoundation.org -- National Sleep Foundation
www.narcolepsynetwork.org -- Narcolepsy Network
www.med.stanford.edu/school/psychiatry/narcolepsy -- Stanford Center For Narcolepsy
www.nhlbi.nih.gov/sleep -- National Center on Sleep Disorders Research
www.ninds.nih.gov -- National Institute on Neurological Disorders and Stroke

References

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

Dodel R, Peter H, Spottke A, et al. Health-related quality of life in patients with narcolepsy. Sleep Med. 2007 Nov;8(7-8):733-41. Epub 2007 May 18.

Durmer, J. Narcolepsy. Instant diagnosis and treatment. In: Ferri FF, ed. Ferri’s Clinical Advisor 2009. 1st ed. Philadelphia, Pa: Mosby Elsevier; 2009.

Harsh JR, Hayduk R, Rosenberg R, Wesnes KA, Walsh JK, Arora S, et al. The efficacy and safety of armodafinil as treatment for adults with excessive sleepiness associated with narcolepsy. Curr Med Res Opin. 2006;22(4):761-774.

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Mahowald, M. Disorders of sleep: Specific Sleep Disorders. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 429.

Morgenthaler TI, Kapur VK, Brown T, Swick TJ, Alessi C, Aurora RN, et al. Practice parameters for the treatment of narcolepsy and other hypersomnias of central origin. Sleep. 2007 Dec 1;30(12):1705-11.

Owens, J. Sleep medicine. In: Kliegman: Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 18.

Pagel JF. Excessive daytime seepiness. American Family Physician. 2009;79(5).

Thorpy MJ. Cataplexy associated with narcolepsy: epidemiology, pathophysiology and management. CNS Drugs. 2006;20(1):43-50.

Vignatelli L, D’Alessandro R, Candelise L. Antidepressant drugs for narcolepsy. Cochrane Database Syst Rev. 2008;(1):CD003724.

Xyrem International Study Group. Further evidence supporting the use of sodium oxybate for the treatment of cataplexy: a double-blind, placebo-controlled study in 228 patients. Sleep Med. 2005 Sep;6(5):415-421.

Reviewed last on: 8/5/2009
Harvey Simon, MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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