Skip to main content
SpringerLink
Log in

Current Diagnostic Criteria for Adult Narcolepsy

  • Chapter
  • First Online:
Narcolepsy

Abstract

Narcolepsy is clinically characterized by excessive daytime sleepiness (EDS) and other symptoms including cataplexy, sleep paralysis, hypnagogic and hypnopompic hallucinations, automatic behaviors, disrupted nocturnal sleep, REM sleep behavior disorder, and moderate weight gain [1–5]. Vehicle accidents and social, familial, professional, and psychological problems are common and can dramatically impact quality of life. More than 95% of the cases are sporadic but the disease has been described in some families and in monozygotic twins. The risk of a first-degree relative developing narcolepsy with cataplexy (NC) is 10–40 times higher (1–2%) than that in the general population. The cause of NC is still unknown, although an autoimmune process occurring in the brain is suspected since the condition is linked to a loss of hypocretin- (orexin-) producing cells in the hypothalamus, low levels of cerebrospinal fluid (CSF) hypocretin [6–13], and the presence of the human leukocyte antigen (HLA) DQB1*0602 subtype [14].

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 149.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 199.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. American Academy of Sleep Medicine. ICSD-2. International Classification of Sleep disorders. 2nd ed: Diagnostic and coding manual. American Academy of Sleep Medicine, 2005.

    Google Scholar 

  2. Overeem S, Mignot E, van Dijk G, Lammers GJ. Narcolepsy: clinical features, new pathophysiological insights, and future perspectives. J Clin Neurophysiol. 2001;18:78–105.

    Article  PubMed  CAS  Google Scholar 

  3. Chakravorty SS, Rye DB. Narcolepsy in the older adult. Epidemiology, diagnosis and management. Drugs Aging. 2003;20:361–76.

    Article  PubMed  Google Scholar 

  4. Dauvilliers Y, Arnulf I, Mignot E. Narcolepsy with cataplexy. Lancet. 2007;369:499–511.

    Article  PubMed  Google Scholar 

  5. Plazzi G, Serra L, Ferri R. Nocturnal aspects of narcolepsy with cataplexy. Sleep Med Rev. 2008;12:109–28.

    Article  PubMed  Google Scholar 

  6. De Lecea L, Kilduff TS, Peyron C, et al. The hypocretins: hypothalamus-specific peptides with neuroexcitatory activity. Proc Natl Acad Sci USA. 1998;95:322–7.

    Article  PubMed  Google Scholar 

  7. Peyron C, Faraco J, Rogers W, et al. A mutation in a case of early onset narcolepsy and a generalized absence of hypocretin peptides in human narcoleptic brains. Nat Med. 2000;6:991–7.

    Article  PubMed  CAS  Google Scholar 

  8. Thannickal TC, Moore RY, Nienhuis R, et al. Reduced number of hypocretin cells in human narcolepsy. Neuron. 2000;27:469–74.

    Article  PubMed  CAS  Google Scholar 

  9. Gerashchencko D, Murillo-Rodriguez E, Lin L, et al. Relationship between CSF hypocretin levels and hypocretin neuronal loss. Exp Neurol. 2003;184:1010–6.

    Article  Google Scholar 

  10. Ripley B, Overeem S, Fujiki N, et al. CSF hypocretin/orexin levels in narcolepsy and other neurological conditions. Neurology. 2001;57:2253–8.

    Article  PubMed  CAS  Google Scholar 

  11. Mignot E, Lammers GJ, Ripley B, et al. The role of cerebrospinal fluid hypocretin measurement in the diagnosis of narcolepsy and other hypersomnias. Arch Neurol. 2002;59:1553–62.

    Article  PubMed  Google Scholar 

  12. Bourgin P, Zeitzer JM, Mignot E. CSF hypocretin-1 assessment in neurological disorders. Lancet Neurol. 2008;7:649–62.

    Article  PubMed  CAS  Google Scholar 

  13. Fronczek R, Baumann CR, Lammers GJ, Bassetti CL, Overeem S. Hypocretin/orexin disturbances in neurological disorders. Sleep Med Rev. 2009;13:9–22.

    Article  PubMed  Google Scholar 

  14. Mignot E, Hayduk R, Black J, Grumet FC, Guilleminault C. HLA DQB1*0602 is associated with cataplexy in 509 narcoleptics patients. Sleep. 1997;20:1012–20.

    PubMed  CAS  Google Scholar 

  15. Silber MH, Krahan LE, Olson EJ. Diagnosing narcolepsy: validity and reliability of new diagnostic criteria. Sleep Med. 2002;3:109–13.

    Article  PubMed  Google Scholar 

  16. ICSD-International classification of sleep disorders. Diagnostic and coding manual. Diagnostic Classification Steering Committee, Thorpy MJ, Chairman. Rochester, MA. American Sleep Disorders Association, 1990.

    Google Scholar 

  17. Billiard M. Diagnosis of narcolepsy and idiopathic hypersomnia. An update based on the International Classification of Sleep disorders, 2nd edition. Sleep Med Rev. 2007;11:377–88.

    Article  PubMed  Google Scholar 

  18. Anic-Labat S, Guilleminault C, Kraemer HC, et al. Validation of a cataplexy questionnaire in 983 sleep-disorders patients. Sleep. 1999;22:77–87.

    PubMed  CAS  Google Scholar 

  19. Aarand D, Bonnet M, Hurwitz M, Rosa R, Sangal B. The clinical use of the MSLT and MWT. Sleep. 2005;28:123–44.

    Google Scholar 

  20. Mignot E, Lin L, Finn L, et al. Correlates of sleep-onset REM periods during the multiple sleep latency test in community adults. Brain. 2006;129:1609–23.

    Article  PubMed  Google Scholar 

  21. Santamaria J, Falup C, Iranzo A, Salamero M. Themporal distribution of sleep onset REM periods in narcolepsy. J Sleep Research. 2006;15 Suppl 1:76.

    Google Scholar 

  22. Nishino S, Kanbayashi T. Symptomatic narcolepsy, cataplexy and EDS, and their implications in the hypothalamic hypocretin/orexin system. Sleep Med Rev. 2005;9:269–310.

    Article  PubMed  Google Scholar 

  23. Dauvilliers Y, Montplaisir J, Molinari N, et al. Age at onset of narcolepsy in two large populations of patients in France and Quebec. Neurology. 2001;57:2029–33.

    Article  PubMed  CAS  Google Scholar 

  24. Rye DB, Dihenia B, Weissman JD, Epstein CM, Bliwise DL. Presentation of narcolepsy after 40. Neurology. 1998;50:459–65.

    Article  PubMed  CAS  Google Scholar 

  25. Bahammam A. Periodic leg movements in narcolepsy patients: impact on sleep architecture. Acta Neurol Scand. 2007;115:351–5.

    Article  PubMed  CAS  Google Scholar 

  26. Schenck CH, Mahowald M. Motor dyscontrol in narcolepsy: rapid-eye-movement (REM) sleep without atonia and REM sleep behaviour disorder. Ann Neurol. 1992;32:3–10.

    Article  PubMed  CAS  Google Scholar 

  27. Sansa G, Iranzo A, Santamaria J. Obstructive sleep apnea in narcolepsy. Sleep Med. 2009;11:93–5.

    Google Scholar 

  28. Allen RP. When, if ever, can we use REM-onset naps on the MSLT for the diagnosis of narcolepsy? Sleep Med. 2006;7:657–9.

    Article  PubMed  Google Scholar 

  29. Aldrich MS. The clinical spectrum of narcolepsy and idiopathic hypersomnia. Neurology. 1996;46:393–401.

    Article  PubMed  CAS  Google Scholar 

  30. Marti I, Valko PO, Khatami R, Bassetti CL, Baumann CR. Multiple sleep latency measures in narcolepsy and behaviourally induced insufficient sleep syndrome. Sleep Med. 2009;10:1146–50.

    Article  PubMed  Google Scholar 

  31. Oka Y, Inoue Y, Kanbayashi T, et al. Narcolepsy without cataplexy: 2 subtypes based on CSF Hypocretin/orexin-A findings. Sleep. 2006;29:1439–43.

    PubMed  Google Scholar 

  32. Thannickal TC, Lai YY, Siegel JM. Hypocretin (orexin) cell loss in Parkinson’s disease. Brain. 2007;130:1586–95.

    Article  PubMed  Google Scholar 

  33. Fronczek R, Overeem S, Lee SY, et al. Hypocretin (orexin) loss in Parkinson’s disease. Brain. 2007;130:1577–85.

    Article  PubMed  Google Scholar 

  34. Compta Y, Santamaria J, Ratti L, et al. Excessive daytime sleepiness, cerebrospinal fluid hypocretin-1 and sleep architecture in Parkinson’s disease with dementia. Brain. 2009;132:3308–17.

    Article  PubMed  Google Scholar 

  35. Thannickal TC, Nienhuis R, Siegel JM. Localized loss of hypocretin (orexin) cells in narcolepsy without cataplexy. Sleep. 2009;32:993–8.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Neurology Service and Multidisciplinary Sleep Unit, Hospital Clínic and Institut D’Investigació Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain

    Alex Iranzo

Authors
  1. Alex Iranzo
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Alex Iranzo .

Editor information

Editors and Affiliations

  1. , Department of Neurology, University Hospital of Zurich, Frauenklinikstrasse 26, Zurich, 8091, Switzerland

    Christian R. Baumann

  2. Neurocenter EOC of Southern Switzerland, Department of Neurology, University Hospital Zurich, Via Tesserete 46, Lugano, 6903, Switzerland

    Claudio L. Bassetti

  3. and Harvard Medical School, Department of Neurology, Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, 02215, Massachusetts, USA

    Thomas E. Scammell

Rights and permissions

Reprints and permissions

Copyright information

© 2011 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Iranzo, A. (2011). Current Diagnostic Criteria for Adult Narcolepsy. In: Baumann, C., Bassetti, C., Scammell, T. (eds) Narcolepsy. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-8390-9_34

Download citation

  • DOI: https://doi.org/10.1007/978-1-4419-8390-9_34

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4419-8389-3

  • Online ISBN: 978-1-4419-8390-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation