Primary Hypersomnia Treatment & Management

  • Author: Adrian Preda, MD; Chief Editor: Iqbal Ahmed, MBBS, FRCPsych (UK)   more...
 
Updated: May 14, 2012
 

Approach Considerations

Severe idiopathic hypersomnia is a disabling problem that often leads to permanent unemployment and responds poorly to medical treatment.[9, 8] Moreover, because the underlying cause of idiopathic hypersomnia is unknown, treatment remains symptomatic in nature.

The American Academy of Sleep Medicine practice parameters state that successful treatment of hypersomnia of central origin requires an accurate diagnosis, individual tailoring of therapy to produce maximum possible return of function, and regular follow-up to monitor response to therapy.

Modafinil, sodium oxybate, amphetamine, methamphetamine, dextroamphetamine, methylphenidate, and selegiline are effective treatments for excessive sleepiness associated with narcolepsy and primary hypersomnias. Scheduled naps can be beneficial to combat sleepiness in these patients.[28]

Behavioral approaches and sleep hygiene techniques are recommended, although they have little overall positive impact on this disease.

Consultations

The diagnosis of primary hypersomnia is made after excluding neurologic, pulmonary, and psychiatric disorders known to cause excessive sleepiness. Therefore, if an underlying cause is suggested, appropriate consultations with a neurologist, pulmonologist, and psychiatrist should be obtained.

Activity

Caution is recommended in activities in which hypersomnolence may be hazardous.

Next

Pharmacologic Therapy

Medications that have been used in the treatment of this disorder include tricyclic antidepressants (TCAs), monoamine oxidase inhibitors (MAOIs), clonidine, levodopa, bromocriptine, amantadine, methysergide, pemoline (as of October 2005, this is no longer available in the United States; risk of liver toxicity outweighs benefits), and modafinil. (Patients develop tolerance to their medications; exercise caution in prescribing drugs.)

Therapy for idiopathic hypersomnolence involves maintaining the patient on a daily use of stimulants. The drug dose is titrated so that the patient stays alert during the day, but adverse effects should be avoided.

Methylphenidate (Ritalin), mazindol (withdrawn from the US market in 2001), and dextroamphetamine are the most commonly prescribed medications.

Modafinil

Modafinil has proved clinically useful in the treatment of narcolepsy and other causes of excessive daytime sleepiness, such as idiopathic hypersomnolence.[29] It is a psychostimulant that enhances wakefulness and vigilance, but its pharmacologic profile is notably different from the amphetamines, methylphenidate, or cocaine. Modafinil is less likely to produce side effects such as jitteriness, anxiety, or excess locomotor activity or to lead to a hypersomnolent rebound effect. It is long-acting; the normal elimination half-life of modafinil in humans is between 12-15 hours.[3, 30]

The mechanism of action of modafinil is not fully understood. Modafinil induces wakefulness in part by its action in the anterior hypothalamus. Its dopamine-releasing action in the nucleus accumbens is weak and dose dependent; the likelihood of a euphoric response, and, therefore, the abuse potential and tolerance, is small.

Modafinil has central alpha 1-adrenergic agonist effects (ie, it directly stimulates the receptors). Modafinil inhibits the reuptake of noradrenaline by the noradrenergic terminals on sleep-promoting neurons of the ventrolateral preoptic nucleus (VLPO). More significant, perhaps, is its ability to increase excitatory glutaminergic transmission and reduce local gamma-aminobutyric acid (GABA)–ergic transmission, thereby diminishing GABA(A) receptor signaling on the mesolimbic dopamine terminals.[30, 31]

Previous
Next

Physician Legal Responsibilities

Physicians have a legal responsibility to know which medical conditions may impede driving ability, to diagnose these conditions in their patients, and to discuss the implications of these conditions.

The requirement to report unfit drivers varies among different jurisdictions, and interpretations of the law vary among the courts. Therefore, a physician’s risk of liability is unclear. Physicians may face legal action by their patients if they fail to counsel the patients on the dangers of driving associated with certain medications or medical conditions.

Physicians’ legal responsibilities to report patients with certain medical conditions, when required by law, override their ethical responsibilities to keep patients’ medical information confidential.

Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Adrian Preda, MD  Health Sciences Associate Professor of Psychiatry and Human Behavior, University of California Irvine School of Medicine

Adrian Preda, MD is a member of the following medical societies: International Congress of Schizophrenia Research, Schizophrenia International Research Society, and Society of Biological Psychiatry

Disclosure: Nothing to disclose.

Chief Editor

Iqbal Ahmed, MBBS, FRCPsych (UK)  Faculty, Department of Psychiatry, Tripler Army Medical Center; Clinical Professor of Psychiatry, Uniformed Services University of Health Sciences: Clinical Professor of Psychiatry, Clinical Professor of Geriatric Medicine, University of Hawaii, John A Burns School of Medicine

Iqbal Ahmed, MBBS, FRCPsych (UK) is a member of the following medical societies: Academy of Psychosomatic Medicine, American Association for Geriatric Psychiatry, American Neuropsychiatric Association, American Psychiatric Association, American Society of Clinical Psychopharmacology, and Royal College of Psychiatrists

Disclosure: Nothing to disclose.

Additional Contributors

Jennifer S Morse, MD Associate Medical Director, Optum Health

Jennifer S Morse, MD is a member of the following medical societies: Academy of Psychosomatic Medicine, Aerospace Medical Association, and American Psychiatric Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References

  1. American Psychiatric Association. Primary hypersomnia. In: Diagnostic and Statistical Manual of Mental Disorders. Text Revision (DSM-IV-TR). 4th Edition. Washington, DC: American Psychiatric Association; 2000:604-9.

  2. American Academy of Sleep Medicine. The international classification of sleep disorders: diagnostic - coding manual. 2nd ed. Westchester, IL: American Academy of Sleep Medicine; 2005.

  3. Dement W, Rechtschaffen A, Gulevich G. The nature of the narcoleptic sleep attack. Neurology. Jan 1966;16(1):18-33. [Medline].

  4. Roth B, Nevsimalova S, Rechtschaffen A. Hypersomnia with "sleep drunkenness". Arch Gen Psychiatry. May 1972;26(5):456-62. [Medline].

  5. Roehrs T, Zorick F, Sicklesteel J. Excessive daytime sleepiness associated with insufficient sleep. Sleep. 1983;6(4):319-25. [Medline].

  6. Billiard M. Diagnosis of narcolepsy and idiopathic hypersomnia. An update based on the International classification of sleep disorders, 2nd edition. Sleep Med Rev. Oct 2007;11(5):377-88. [Medline].

  7. Bassetti C, Pelayo R, Guilleminault C. Idiopathic Hypersomnia. In: Kryger MH, Roth T, Dement WC. Principles and Practices of Sleep Medicine. 4th Edition. Philadelphia, PA: Elsevier; 2005:791-800.

  8. Roth T. Introduction: narcolepsy and excessive daytime sleepiness: from the bench to the bedside. J Clin Psychiatry. 2007;68 Suppl 13:4. [Medline].

  9. Guilleminault C. Disorders of excessive sleepiness. Ann Clin Res. 1985;17(5):209-19. [Medline].

  10. Roth B. Narcolepsy and hypersomnia: review and classification of 642 personally observed cases. Schweiz Arch Neurol Neurochir Psychiatr. 1976;119(1):31-41. [Medline].

  11. Arnulf I, Zeitzer JM, File J, et al. Kleine-Levin syndrome: a systematic review of 186 cases in the literature. Brain. Dec 2005;128(Pt 12):2763-76. [Medline].

  12. Billiard M, Guilleminault C, Dement WC. A menstruation-linked periodic hypersomnia: Kleine-Levin syndrome or new clinical entity?. Neurology. 1975;25:436-443. [Medline].

  13. Montplaisir J, Poirier G. HLA in disorders of excessive sleepiness without cataplexy in Canada. In: Honda Y, Juti T. HLA in Narcolepsy. Berlin, Germany: Springer-Verlag; 1988:186-190.

  14. Guilleminault C, Faull KF, Miles L. Posttraumatic excessive daytime sleepiness: a review of 20 patients. Neurology. Dec 1983;33(12):1584-9. [Medline].

  15. Montplaisir J, de Champlain J, Young SN. Narcolepsy and idiopathic hypersomnia: biogenic amines and related compounds in CSF. Neurology. Nov 1982;32(11):1299-302. [Medline].

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

  17. Nishino S, Okuro M, Kotorii N, Anegawa E, Ishimaru Y, Matsumura M, et al. Hypocretin/orexin and narcolepsy: new basic and clinical insights. Acta Physiol (Oxf). Jun 25 2009;[Medline].

  18. Nishino S, Kanbayashi T. Symptomatic narcolepsy, cataplexy and hypersomnia, and their implications in the hypothalamic hypocretin/orexin system. Sleep Med Rev. Aug 2005;9(4):269-310. [Medline].

  19. Yamanaka A, Tsujino N, Funahashi H, Honda K, Guan JL, Wang QP, et al. Orexins activate histaminergic neurons via the orexin 2 receptor. Biochem Biophys Res Commun. 2002;290:1237-45. [Medline]. [Full Text].

  20. Ohayon MM, Dauvilliers Y, Reynolds CF 3rd. Operational Definitions and Algorithms for Excessive Sleepiness in the General Population: Implications for DSM-5 Nosology. Arch Gen Psychiatry. Jan 2012;69(1):71-9. [Medline].

  21. Guilleminault C, Faull KF. Sleepiness in nonnarcoleptic, non-sleep apneic EDS patients: the idiopathic CNS hypersomnolence. Sleep. 1982;5 Suppl 2:S175-81. [Medline].

  22. Bassetti C, Gugger M, Bischof M. The narcoleptic borderland: a multimodal diagnostic approach including cerebrospinal fluid levels of hypocretin-1 (orexin A). Sleep Med. Jan 2003;4(1):7-12. [Medline].

  23. Ohayon MM. From wakefulness to excessive sleepiness: what we know and still need to know. Sleep Med Rev. Apr 2008;12(2):129-41. [Medline].

  24. Sangal RB; Mitler MM; Sangal JM. Subjective sleepiness ratings (Epworth sleepiness scale) do not reflect the same parameter of sleepiness as objective sleepiness (maintenance of wakefulness test) in patients with narcolepsy. Clin Neurophysiol. Dec 1999;(110)12:2131-5. [Medline].

  25. Sangal RB, Sangal JM, Belisle C. Subjective and objective indices of sleepiness (ESS and MWT) are not equally useful in patients with sleep apnea. Clin Electroencephalogr. Apr 1999;30(2):73-5. [Medline].

  26. Rechtschaffen A, Roth B. Nocturnal sleep of hypersomniacs. Act Nerv Super (Praha). 1969;11(3):229-33. [Medline].

  27. Anderson KN, Pilsworth S, Sharples LD, Smith IE, Shneerson JM. Idiopathic hypersomnia: a study of 77 cases. Sleep. Oct 1 2007;30(10):1274-81. [Medline].

  28. [Best Evidence] [Guideline] 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. Dec 1 2007;30(12):1705-11. [Medline].

  29. Ballon JS, Feifel D. A systematic review of modafinil: Potential clinical uses and mechanisms of action. J Clin Psychiatry. Apr 2006;67(4):554-66. [Medline].

  30. Valentino RM, Foldvary-Schaefer N. Modafinil in the treatment of excessive daytime sleepiness. Cleve Clin J Med. Aug 2007;74(8):561-6, 568-71. [Medline].

  31. Schwartz JR. Modafinil: new indications for wake promotion. Expert Opin Pharmacother. Jan 2005;6(1):115-29. [Medline].

  32. Poppe M, Friebel D, Reuner U, Todt H, Koch R, Heubner G. The Kleine-Levin syndrome - effects of treatment with lithium. Neuropediatrics. 2003;34:113-9. [Medline].

 
Previous
Next
 
Primary hypersomnia. Polysomnographic study demonstrates apnea (absence of carbon dioxide fluctuation indicating no flow), chest wall paradox, abrupt increase in tidal volume at the end of apnea, and oxygen desaturation. All of these features are consistent with obstructive sleep apnea.
Primary hypersomnia. In contrast to obstructive sleep apnea, mixed apnea shows absence of respiratory efforts in the first segment of the apnea.
Primary hypersomnia. Periodic limb movements show intermittent leg electromyogram activity accompanied by electroencephalogram arousals.
 
 
 
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.