Skip to main content
SpringerLink
Log in
Book cover

Sleep Medicine pp 229–236Cite as

Kleine–Levin Syndrome

  • Chapter
  • First Online:

  • 3041 Accesses

Abstract

The first account of a man with recurrent sleep episodes was reported by William Oliver in 1705. During the eighteenth and nineteenth centuries other cases appeared in the literature. In 1925, Kleine, published a case of “recurrent episodes of somnolence and morbid hunger” and Levin, in 1936, described “a syndrome characterized by recurring periods of somnolence and morbid hunger.” The name Kleine–Levin syndrome (KLS) was coined by Critchley and Hoffman in 1942. Twenty years later Critchley published a milestone article in which he gave an outstanding description of 26 cases of “a syndrome composed of recurrent episodes of undue sleepiness lasting some days, associated with an inordinate intake of food and often with abnormal behavior.” In the same review he emphasized four hallmarks: Males preponderance, onset in adolescence, spontaneous eventual disappearance, and the possibility that megaphagia may indicate compulsive eating. From 2000 onward use of neuroimaging techniques, in particular single-photon emission computerized tomography (SPECT) and positron emission tomography (PET), has led to a better understanding of the neuronal basis of the syndrome. Finally, in 2005, the international classification of sleep disorders-2 (ICSD-2) has established criteria for recurrent hypersomnia, including KLS and menstrual-related hypersomnia. Recently, large series of patients have been published and reports of familial cases and monozygotic twins have set the path for genetic studies.

This is a preview of subscription content, 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   109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   139.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
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

Learn about institutional subscriptions

Notes

  1. 1.

    The Ramanaya is one of the two great epics of India (third century BC–AD third century), the other being the Mahabharata. Some cultural evidence suggests that the Ramayana predates the Mahabharata.

References

  1. Critchley M, Hoffman HL. The syndrome of periodic somnolence and morbid hunger (Kleine-Levin syndrome). Brit Med J. 1942;1:137–9.

    Google Scholar 

  2. Oliver W. An account of an extraordinary sleepy person. Philos Trans. 1705;304:2177–82.

    Google Scholar 

  3. Kleine W. Periodische Schlafsuch. Mschr Psychiat Neurol. 1925;57:285–20.

    Google Scholar 

  4. Lewis NDC. The psychoanalytic approach to the problems of children under twelve years of age. Psychoanal Rev. 1926;13:424–43.

    Google Scholar 

  5. Levin M. Narcolepsy (Gélineau syndrome) and other varieties of morbid somnolence. Arch Neurol Psychiat. 1929;22:1172–200.

    Google Scholar 

  6. Levin M. Periodic somnolence and morbid hunger. A new syndrome. Brain. 1936;59:494–4.

    Google Scholar 

  7. Critchley M. Periodic hypersomnia and megaphagia in adolescent males. Brain. 1962;85:627–57.

    Google Scholar 

  8. American Academy of Sleep Medicine. International classification of sleep disorders, 2. Diagnostic and coding manual. Westchester: American Academy of Sleep Medicine; 2005.

    Google Scholar 

  9. American Academy of Sleep Medicine. International classification of sleep disorders, 3rd ed. Darien IL: American Academy of Sleep Medicine; 2014.

    Google Scholar 

  10. Chauvot de Beauchêne EP. Observation sur une maladie nerveuse, avec complication d’un sommeil, tantôt léthargique, tantôt convulsif. A Amsterdam et à Paris ; chez Méquignon l’aîné. 1786. 22p.

    Google Scholar 

  11. Satterley RP. A case of fever, attended with inordinate appetite. Medical Trans. 1815: 350–7.

    Google Scholar 

  12. Brière de Boismont A. Des hallucination ou histoire raisonnée des apparitions, des visions, des songes, de l’extase, des rêves, du magnétisme et du somnambulisme. Paris ; Germer Baillière ; 1862.

    Google Scholar 

  13. Lyon Med, Mendel M., Oct. 27, 1872, quoted by Dana CL. On morbid drowsiness and somnolence: a contribution to the pathology of sleep. J Nerv Ment Dis. 1884;11:153–76.

    Google Scholar 

  14. Laségue. Gaz Hôp (Paris) 1882, quoted by Dana CL. On morbid drowsiness and somnolence: A contribution to the pathology of sleep. J Nerv Ment Dis. 1884;11:153–76.

    Google Scholar 

  15. Anfimoff JA (1898). Quoted by Kaplinsky MS and Schulmann ED. Über die periodische Schlafsucht. Acta Med Scand. 1935;85:107–28.

    Google Scholar 

  16. Stöcker W. Zur Narkolepsiefrage. Ztschr f d ges Neurol u Psychiat. 1913;18:216–46.

    Google Scholar 

  17. Schröder. Ungewöhnliche period. Psychosen. Mtsschr f Psych. 1918;44:S 261.

    Google Scholar 

  18. Krüger. Episodisch Schlafzustände (Lethargien). (Inaugural-Dissertation) Greifswald 1920.

    Google Scholar 

  19. Kanabich JW. (1923). Quoted by Kaplinsky and Schulmann ED. Über die periodische Schlafsucht. Acta Med Scand. 1935;85:107–28.

    Google Scholar 

  20. Daniels LE. Medicine, Baltimore. 1934;13:1.

    Google Scholar 

  21. Kaplinsky MS, Schulmann ED. Über die periodische Schlafsucht. Acta Med Scand. 1935;85:107–28.

    Google Scholar 

  22. Hylkema EA. Een bijdrage tot de kennis van de narcolepsie. Thesis, Groningen. 1940.

    Google Scholar 

  23. Badino R, Caja A, Del Conte I et al. Kleine-Levin syndrome in an 82 year old man. Ital J Neurol Sci. 1992;13:355–6.

    Google Scholar 

  24. Ba Hamman AS, GadElRab MO, Owais SM et al. Clinical characteristics and HLA typing of a family with Kleine-Levin syndrome. Sleep Med. 2008;9:575–8.

    Google Scholar 

  25. Dauvilliers Y, Mayer G, Lecendreux M, et al. Kleine-Levin syndrome. An autoimmune hypothesis based on clinical and genetic analyses. Neurology. 2002;59:1739–45.

    Google Scholar 

  26. Carpenter S, Yassa R, Ochs R. A pathologic basis for Kleine-Levin syndrome. Arch Neurol. 1982;39:25–8.

    Google Scholar 

  27. Koerber RK, Torkelson ER, Haven G et al. Increased cerebrospinal fluid 5-hydroxytryptamine and 5-hydoxyindoleacetic acid in Kleine-Levin syndrome. Neurology. 1984;34:1597–600.

    Google Scholar 

  28. Fenzi F, Simonati A, Crozato F, et al. Clinical features of Kleine-Levin syndrome with localized encephalitis. Neuropediatrics. 1993;24:292–5.

    Google Scholar 

  29. Takrani LB, Cronin D. Kleine-Levin syndrome in a female patient. Can Psychiatr Assoc J. 1976;21:315–8.

    Google Scholar 

  30. Lu ML, Liu HC, Chen CH, et al. Kleine-Levin syndrome and psychosis: Observation from an unusual case. Neuropsychiatry Neuropsychol Behav Neurol. 2000;13:140–2.

    Google Scholar 

  31. Landtblom AM, Dige N, Schwerdt K, et al. A case of Kleine-Levin syndrome examined with SPECT and neuropsychological testing. Acta Neurol Scand. 2002;105:318–21.

    Google Scholar 

  32. Landtblom AM, Dige N, Schwerdt K, et al. Short-term memory dysfunction in Kleine-Levin syndrome. Acta Neurol Scand. 2003;108:363–7.

    Google Scholar 

  33. Arias M, Crespo-Iglesias JM, Pérez J, et al. Syndrome de Kleine-Levin : Aportacion diagnostica de la SPECT cerebral. Rev Neurol. 2002;35:531–3.

    Google Scholar 

  34. Nose I, Ookawa T, Tanaka J, et al. Decreased blood flow of the left thalamus during somnolent episodes in a case of recurrent hypersomnia. Psychiatry Clin Neurosci. 2002;56:277–8.

    Google Scholar 

  35. Portilla P, Durand E, Chalvon A. et al. Hypoperfusion temporomésiale gauche en TEMP dans un syndrome de Kleine-Levin. Rev Neurol (Paris). 2002;158:593–5.

    Google Scholar 

  36. Dauvilliers Y, Baumann CR, Carlander B, et al. CSF hypocretin −1 levels in narcolepsy, Kleine-Levin syndrome and other hypersomnias and neurological conditions. J Neurol Neurosurg Psychiatry. 2003;74:1667–73.

    Google Scholar 

  37. Billiard M, Jaussent I, Dauvilliers Y, et al. Recurrent hypersomnias : A review of 339 cases. Sleep Med Rev. 2011;15:247–57.

    Google Scholar 

  38. Arnulf I, Zeitzer JM, File J, et al. Kleine-Levin syndrome: A systematic review of 186 cases in the literature. Brain. 2005;128:2763–76.

    Google Scholar 

  39. Arnulf I, Lin L, Gadoth N, et al. Kleine-Levin syndrome: A systematic study of 108 patients. Ann Neurol. 2008;63:482–92.

    Google Scholar 

  40. Huang CJ, Liao HT, Yeh GC, et al. Distribution of HLA-DQB1 alleles in patients with Kleine-Levin syndrome. J Clin Neurosci. 2012;19:628–30.

    Google Scholar 

  41. Billiard M, Peraita-Adrados R, Tafti M. Genetics of recurrent hypersomnia. In: Shaw P, Tafti M, Thorpy MJ, editors. The genetic basis of sleep and sleep disorders. Cambridge: Cambridge University Press, 2013. 272–77.

    Google Scholar 

  42. Suwa K, Toru M. A case of periodic somnolence whose sleep was induced by glucose. Folia Psychiatr Neurol Jpn. 1969;23:253–62.

    Google Scholar 

  43. Popper JSD, Hsia YE, Rogers T, et al. Familial hibernation (Kleine-Levin syndrome). Am J Hum Genet. 1980;32:123A.

    Google Scholar 

  44. Peraita-Adrados R, Vicario JL, Tafti M, et al. Monozygotic twins affected with Kleine-Levin syndrome. Sleep. 2012;35:595–6.

    Google Scholar 

  45. Ueno T, Fukuhara A, Ikegami A, et al. Monozygotic twins concordant for Kleine-Levin syndrome. BMC Neurol. 30 May 2012 ;12:31. (Epub ahead of print).

    Google Scholar 

  46. Huang YS, Guilleminault C, Kao PF, et al. SPECT findings in the Kleine-Levin syndrome. Sleep. 2005;28:955–60.

    Google Scholar 

  47. Hsieh CF, Lai CI, Lan SH et al. Modafinil-associated vivid visual hallucination in a patient with Kleine-Levin syndrome: Case report. J Clin Psychopharmacol. 2010;30:347–50.

    Google Scholar 

  48. Huang YS, Guilleminault C, Lin K, et al. Relatinship between Kleine-Levin syndrome and upper respiratory infection in Taïwan. Sleep. 2012;35:123–9.

    Google Scholar 

  49. Hong SB, Joo EY, Tae WS, et al. Episodic diencephalic hypoperfusion in Kleine-Levin syndrome. Sleep. 2006;29:1091–3.

    Google Scholar 

  50. Itokawa K, Fukui M, Ninomiya M, et al. Gabapentine for Kleine-Levin syndrome. Intern Med. 2009;48:1183–5.

    Google Scholar 

  51. Billings ME, Watson NF, Keogh BP. Dynamics fMRI changes in Kleine-Levin syndrome. Sleep Med. 2011;12:532.

    Google Scholar 

  52. Lo YC, Chou YH, Yu HY. PET finding in Kleine-Levin syndrome. Sleep Med. 2012;13:771–2.

    Google Scholar 

  53. Haba-Rubio J, Prior JO, Guedj E, et al. Kleine-Levin syndrome: Functional imaging correlates of hypersomnia and behavioral symptoms. Neurology. 2012;79:1927–9.

    Google Scholar 

  54. Hirst J, Mignot E, Stein MT. Episodic hypersomnia and unusual behaviors in a 14-year old adolescent. J Dev Behav Pediatr. 2007;28:475–7.

    Google Scholar 

  55. Li Q, Wang J, Dong X et al. CSF hypocretin level in patients with Kleine-Levin syndrome. Sleep Med 2013;14,Suppl 1,e47.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Gui de Chauliac Hospital, 80, avenue Augustin Fliche, 34295, Montpellier cedex 5, France

    Michel Billiard MD

  2. School of Medicine, University Montpellier I, Montpellier, France

    Michel Billiard MD

Authors
  1. Michel Billiard MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Michel Billiard MD .

Editor information

Editors and Affiliations

  1. Department of Neurology, New Jersey Neuroscience Institute JFK Medical Center, Edison, New Jersey, USA

    Sudhansu Chokroverty

  2. Department of Neurology, Gui de Chauliac Hospital, Montpellier, France

    Michel Billiard

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Billiard, M. (2015). Kleine–Levin Syndrome. In: Chokroverty, S., Billiard, M. (eds) Sleep Medicine. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-2089-1_28

Download citation

  • DOI: https://doi.org/10.1007/978-1-4939-2089-1_28

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-2088-4

  • Online ISBN: 978-1-4939-2089-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation