eMedicine Specialties > Psychiatry > Psychosomatic

Primary Hypersomnia: Follow-up

Author: Adrian Preda, MD, Health Sciences Associate Professor of Psychiatry and Human Behavior, University of California Irvine School of Medicine
Contributor Information and Disclosures

Updated: Nov 3, 2009

Follow-up

Prognosis

  • After a typical onset between the ages 15-30 years, untreated primary hypersomnia presents a chronic but stable course.
  • Idiopathic hypersomnia is a lifelong disorder with no tendency to remit spontaneously.
  • Consequences of this disease are mostly social and professional in nature.

Patient Education

  • While treating patients with primary hypersomnia, the patient's close family should be involved in the overall education and decision-making process.
  • Because these disorders may lead to marriage breakdown, extensive counseling for the patient's partners, educating them about the symptomatology and treatment options, must be part of a comprehensive management plan.
  • Patients with primary hypersomnia often need significant support because they are at risk of being misunderstood as being incompetent or slothful. Therefore, education of relatives, friends, and colleagues helps the patient function much better with this incurable disease.
  • For excellent patient education resources, visit eMedicine's Sleep Disorders Center. Also, see eMedicine's patient education articles Disorders That Disrupt Sleep (Parasomnias) and Narcolepsy.
  • Medline Plus/NIH provides concise and to the point summaries of the diagnosis and recommendations for patients and families dealing with primary hypersomnia and Kleine-Levine syndrome.
  • The Mayo clinic offers an additional, more comprehensive patient resource on idiopathic/primary hypersomnia.

Miscellaneous

Medicolegal Pitfalls

  • Distinguish primary hypersomnia from sleep-related breathing disorders, particularly upper airway resistance syndrome.
  • During automatic behavior episodes, these patients may endanger themselves through risk of injury.
  • Patients develop tolerance to their medications; exercise caution in prescribing medications.
  • Physicians have a legal responsibility to know which medical conditions may impede driving ability, to diagnose these conditions in their patients, and to discuss implications of these conditions.
    • The requirement to report unfit drivers varies among different jurisdictions and interpretations of the law vary among the courts.
    • Therefore, physician’s risks of liability are 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.
    • Physician’s legal responsibilities to report patients with certain medical conditions, when required by law, override their ethical responsibilities to keep patient’s medical information confidential.

Special Concerns

  • The ICSD describes primary hypersomnia as idiopathic hypersomnia. Recurrent hypersomnia is classified separately to describe the recurrent form of primary hypersomnia according to the DSM-IV-TR.
  • Kleine-Levine syndrome (See Background.)
 


More on Primary Hypersomnia

Overview: Primary Hypersomnia
Differential Diagnoses & Workup: Primary Hypersomnia
Treatment & Medication: Primary Hypersomnia
Follow-up: Primary Hypersomnia
Multimedia: Primary Hypersomnia
References
[ CLOSE WINDOW ]

References

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

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

  3. 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.

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

  5. 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.

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

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

  8. Critchley M. The syndrome of hypersomnia and periodical megaphagia in the adult male (Kleine-Levin): what is its natural course? ). Rev Neurol (Paris. 1967;116:647–650. [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. 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].

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

  20. 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].

  21. 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].

  22. 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].

  23. 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].

  24. 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].

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

  26. [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].

  27. 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].

  28. 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].

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

  30. 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].

  31. [Medline].

  32. 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].

  33. Baker TL, Guilleminault C, Nino-Murcia G. Comparative polysomnographic study of narcolepsy and idiopathic central nervous system hypersomnia. Sleep. 1986;9(1 Pt 2):232-42. [Medline].

  34. Ebrahim IO, Sharief MK, de Lacy S. Hypocretin (orexin) deficiency in narcolepsy and primary hypersomnia. J Neurol Neurosurg Psychiatry. Jan 2003;74(1):127-30. [Medline].

  35. 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].

  36. Partinen M, Hublin C. Epidemiology of Sleep Disorders. In: Krygwr MH, Roth T, Dement WC. Principles and Practice of Sleep Medicine. Philadelphia: WB Saunders Company; 2000:558–79.

  37. Shepard JW Jr, Gefter WB, Guilleminault C. Evaluation of the upper airway in patients with obstructive sleep apnea. Sleep. Aug 1991;14(4):361-71. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

idiopathic hypersomnia, primary hypersomnia, sleep disorder treatment, narcolepsy, sleep drunkenness, excessive daytime sleepiness, obstructive sleep apnea, sleep apnea, Kleine-Levin syndrome

[ CLOSE WINDOW ]

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.

Medical Editor

Jennifer S Morse, MD, Assistant Clinical Professor, Department of Psychiatry, University of California at San Diego
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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Iqbal Ahmed, MBBS, Professor, Department of Psychiatry, John A Burns School of Medicine, University of Hawaii
Iqbal Ahmed, MBBS is a member of the following medical societies: Academy of Psychosomatic Medicine, American Association for Geriatric Psychiatry, American Neuropsychiatric Association, and American Psychiatric Association
Disclosure: Nothing to disclose.

CME Editor

Harold H Harsch, MD, Program Director of Geropsychiatry, Department of Geriatrics/Gerontology, Associate Professor, Department of Psychiatry and Department of Medicine, Froedtert Hospital, Medical College of Wisconsin
Harold H Harsch, MD is a member of the following medical societies: American Psychiatric Association
Disclosure: lilly Honoraria Speaking and teaching; Forest Labs Honoraria Speaking and teaching; AstraZeneca Honoraria Speaking and teaching; Pfizer Grant/research funds Speaking and teaching; Northstar Grant/research funds Research; Novartis Grant/research funds research; Pfizer  Speaking and teaching; Sanofi-avetis Grant/research funds research; Otsuke Grant/research funds reseach; GlaxoSmithKline Grant/research funds research

Chief Editor

Stephen Soreff, MD, President of Education Initiatives, Nottingham, NH; Faculty, Metropolitan College of Boston University, Boston, MA
Stephen Soreff, MD is a member of the following medical societies: American College of Mental Health Administration and American Psychosomatic Society
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
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.