U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Kleine-Levin syndrome

MedGen UID:
61511
•Concept ID:
C0206085
•
Disease or Syndrome
Synonyms: Familial hibernation syndrome; Familial Kleine-Levin syndrome; Kleine-Levin hibernation syndrome
SNOMED CT: Kleine-Levin syndrome (111488004); Kleine Levin syndrome (111488004)
 
Monarch Initiative: MONDO:0007863
OMIM®: 148840
Orphanet: ORPHA33543

Definition

The Kleine-Levin hibernation syndrome, a rare disorder that occurs predominantly in males, is characterized by episodic attacks of aberrant behavior, hypersomnia, and increased feeding (megaphagia) and sex drives (Kleine, 1925; Levin, 1929). [from OMIM]

Clinical features

From HPO
Confusion
MedGen UID:
3587
•Concept ID:
C0009676
•
Mental or Behavioral Dysfunction
Lack of clarity and coherence of thought, perception, understanding, or action.
See: Feature record | Search on this feature
Polyphagia
MedGen UID:
9369
•Concept ID:
C0020505
•
Finding
A neurological anomaly with gross overeating associated with an abnormally strong desire or need to eat.
See: Feature record | Search on this feature
Recurrent hypersomnia
MedGen UID:
155520
•Concept ID:
C0751226
•
Disease or Syndrome
Recurrent episodes of severe hypersomnia are accompanied by cognitive and behavioral disturbances, including confusion, derealization, apathy, compulsive eating, and hypersexuality.
See: Feature record | Search on this feature
Vivid hallucination
MedGen UID:
870530
•Concept ID:
C4024978
•
Mental or Behavioral Dysfunction
Perceptual experiences that are vivid, intense, and sensory in nature occur in the absence of corresponding stimuli from the external environment.
See: Feature record | Search on this feature

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVKleine-Levin syndrome
Follow this link to review classifications for Kleine-Levin syndrome in Orphanet.

Professional guidelines

PubMed

Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine systematic review, meta-analysis, and GRADE assessment.
Maski K, Trotti LM, Kotagal S, Robert Auger R, Swick TJ, Rowley JA, Hashmi SD, Watson NF
J Clin Sleep Med 2021 Sep 1;17(9):1895-1945. doi: 10.5664/jcsm.9326. PMID: 34743790Free PMC Article
Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine clinical practice guideline.
Maski K, Trotti LM, Kotagal S, Robert Auger R, Rowley JA, Hashmi SD, Watson NF
J Clin Sleep Med 2021 Sep 1;17(9):1881-1893. doi: 10.5664/jcsm.9328. PMID: 34743789Free PMC Article
Pharmacological treatment for Kleine-Levin syndrome.
de Oliveira MM, Conti C, Prado GF
Cochrane Database Syst Rev 2016 May 6;2016(5):CD006685. doi: 10.1002/14651858.CD006685.pub4. PMID: 27153153Free PMC Article

Recent clinical studies

Etiology

European guideline and expert statements on the management of narcolepsy in adults and children.
Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
J Sleep Res 2021 Dec;30(6):e13387. Epub 2021 Jun 25 doi: 10.1111/jsr.13387. PMID: 34173288
Excessive Daytime Sleepiness: A Clinical Review.
Gandhi KD, Mansukhani MP, Silber MH, Kolla BP
Mayo Clin Proc 2021 May;96(5):1288-1301. Epub 2021 Apr 9 doi: 10.1016/j.mayocp.2020.08.033. PMID: 33840518
The neurophysiological basis of excessive daytime sleepiness: suggestions of an altered state of consciousness.
Hitchcott PK, Menicucci D, Frumento S, Zaccaro A, Gemignani A
Sleep Breath 2020 Mar;24(1):15-23. Epub 2019 May 28 doi: 10.1007/s11325-019-01865-9. PMID: 31140116
Mazindol: a risk factor for pulmonary arterial hypertension?
Konofal E, Benzouid C, Delclaux C, Lecendreux M, Hussey E
Sleep Med 2017 Jun;34:168-169. Epub 2017 Mar 18 doi: 10.1016/j.sleep.2017.02.020. PMID: 28522087
High bicarbonate levels in narcoleptic children.
Franco P, Junqua A, Guignard-Perret A, Raoux A, Perier M, Raverot V, Claustrat B, Gustin MP, Inocente CO, Lin JS
J Sleep Res 2016 Apr;25(2):194-202. Epub 2015 Nov 17 doi: 10.1111/jsr.12357. PMID: 26574184

Diagnosis

Narcolepsy.
Barateau L, Pizza F, Plazzi G, Dauvilliers Y
J Sleep Res 2022 Aug;31(4):e13631. Epub 2022 May 27 doi: 10.1111/jsr.13631. PMID: 35624073
Sleep disorders and the hypothalamus.
Overeem S, van Litsenburg RRL, Reading PJ
Handb Clin Neurol 2021;182:369-385. doi: 10.1016/B978-0-12-819973-2.00025-3. PMID: 34266606
Idiopathic Hypersomnia and Other Hypersomnia Syndromes.
Trotti LM, Arnulf I
Neurotherapeutics 2021 Jan;18(1):20-31. doi: 10.1007/s13311-020-00919-1. PMID: 32901432Free PMC Article
Sleep-related psychosis.
Frase L, Sixt B, Nissen C
BMJ Case Rep 2013 Jul 8;2013 doi: 10.1136/bcr-2013-009373. PMID: 23839606Free PMC Article
Thalamo-frontal psychosis.
McGilchrist I, Goldstein LH, Jadresic D, Fenwick P
Br J Psychiatry 1993 Jul;163:113-5. doi: 10.1192/bjp.163.1.113. PMID: 8353678

Therapy

European guideline and expert statements on the management of narcolepsy in adults and children.
Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
J Sleep Res 2021 Dec;30(6):e13387. Epub 2021 Jun 25 doi: 10.1111/jsr.13387. PMID: 34173288
Idiopathic Hypersomnia and Other Hypersomnia Syndromes.
Trotti LM, Arnulf I
Neurotherapeutics 2021 Jan;18(1):20-31. doi: 10.1007/s13311-020-00919-1. PMID: 32901432Free PMC Article
Daytime sleepiness in children: When a quiet child is not necessarily a good thing.
Marcus CL
Paediatr Respir Rev 2018 Jan;25:1-2. Epub 2017 Jan 19 doi: 10.1016/j.prrv.2017.01.002. PMID: 28209474
Mazindol: a risk factor for pulmonary arterial hypertension?
Konofal E, Benzouid C, Delclaux C, Lecendreux M, Hussey E
Sleep Med 2017 Jun;34:168-169. Epub 2017 Mar 18 doi: 10.1016/j.sleep.2017.02.020. PMID: 28522087
Update on hypersomnias of central origin.
Drakatos P, Leschziner GD
Curr Opin Pulm Med 2014 Nov;20(6):572-80. doi: 10.1097/MCP.0000000000000098. PMID: 25165990

Prognosis

Narcolepsies, update in 2023.
Barateau L, Pizza F, Chenini S, Peter-Derex L, Dauvilliers Y
Rev Neurol (Paris) 2023 Oct;179(7):727-740. Epub 2023 Aug 25 doi: 10.1016/j.neurol.2023.08.001. PMID: 37634997
Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine clinical practice guideline.
Maski K, Trotti LM, Kotagal S, Robert Auger R, Rowley JA, Hashmi SD, Watson NF
J Clin Sleep Med 2021 Sep 1;17(9):1881-1893. doi: 10.5664/jcsm.9328. PMID: 34743789Free PMC Article
New 2013 incidence peak in childhood narcolepsy: more than vaccination?
Zhang Z, Gool JK, Fronczek R, Dauvilliers Y, Bassetti CLA, Mayer G, Plazzi G, Pizza F, Santamaria J, Partinen M, Overeem S, Peraita-Adrados R, da Silva AM, Sonka K, Del Rio-Villegas R, Heinzer R, Wierzbicka A, Young P, Högl B, Manconi M, Feketeova E, Mathis J, Paiva T, Canellas F, Lecendreux M, Baumann CR, Lammers GJ, Khatami R
Sleep 2021 Feb 12;44(2) doi: 10.1093/sleep/zsaa172. PMID: 32909046
Kleine-Levin Syndrome.
Afolabi-Brown O, Mason TBA 2nd
Paediatr Respir Rev 2018 Jan;25:9-13. Epub 2016 Dec 23 doi: 10.1016/j.prrv.2016.12.004. PMID: 28216256
Kleine-Levin Syndrome.
Miglis MG, Guilleminault C
Curr Neurol Neurosci Rep 2016 Jun;16(6):60. doi: 10.1007/s11910-016-0653-6. PMID: 27137943

Clinical prediction guides

Improvement in non-24-h sleep-wake rhythm disorder in a sighted individual treated with a melatonin receptor agonist.
Connolly PJ, Quigg M, Davis EM
Sleep Med 2024 Apr;116:41-42. Epub 2024 Feb 20 doi: 10.1016/j.sleep.2023.10.041. PMID: 38422783
The immunopathogenesis of narcolepsy type 1.
Liblau RS, Latorre D, Kornum BR, Dauvilliers Y, Mignot EJ
Nat Rev Immunol 2024 Jan;24(1):33-48. Epub 2023 Jul 3 doi: 10.1038/s41577-023-00902-9. PMID: 37400646
Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine systematic review, meta-analysis, and GRADE assessment.
Maski K, Trotti LM, Kotagal S, Robert Auger R, Swick TJ, Rowley JA, Hashmi SD, Watson NF
J Clin Sleep Med 2021 Sep 1;17(9):1895-1945. doi: 10.5664/jcsm.9326. PMID: 34743790Free PMC Article
Kleine-Levin syndrome; An update and mini-review.
Gadoth N, Oksenberg A
Brain Dev 2017 Sep;39(8):665-671. Epub 2017 Apr 20 doi: 10.1016/j.braindev.2017.04.003. PMID: 28434769
Hypocretin/Orexin: a molecular link between sleep, energy regulation, and pleasure.
Ganjavi H, Shapiro CM
J Neuropsychiatry Clin Neurosci 2007 Fall;19(4):413-9. doi: 10.1176/jnp.2007.19.4.413. PMID: 18070844

Recent systematic reviews

Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine systematic review, meta-analysis, and GRADE assessment.
Maski K, Trotti LM, Kotagal S, Robert Auger R, Swick TJ, Rowley JA, Hashmi SD, Watson NF
J Clin Sleep Med 2021 Sep 1;17(9):1895-1945. doi: 10.5664/jcsm.9326. PMID: 34743790Free PMC Article
Treatment of central disorders of hypersomnolence: an American Academy of Sleep Medicine clinical practice guideline.
Maski K, Trotti LM, Kotagal S, Robert Auger R, Rowley JA, Hashmi SD, Watson NF
J Clin Sleep Med 2021 Sep 1;17(9):1881-1893. doi: 10.5664/jcsm.9328. PMID: 34743789Free PMC Article
European guideline and expert statements on the management of narcolepsy in adults and children.
Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
Eur J Neurol 2021 Sep;28(9):2815-2830. Epub 2021 Jun 25 doi: 10.1111/ene.14888. PMID: 34173695
European guideline and expert statements on the management of narcolepsy in adults and children.
Bassetti CLA, Kallweit U, Vignatelli L, Plazzi G, Lecendreux M, Baldin E, Dolenc-Groselj L, Jennum P, Khatami R, Manconi M, Mayer G, Partinen M, Pollmächer T, Reading P, Santamaria J, Sonka K, Dauvilliers Y, Lammers GJ
J Sleep Res 2021 Dec;30(6):e13387. Epub 2021 Jun 25 doi: 10.1111/jsr.13387. PMID: 34173288
Pharmacological treatment for Kleine-Levin syndrome.
de Oliveira MM, Conti C, Prado GF
Cochrane Database Syst Rev 2016 May 6;2016(5):CD006685. doi: 10.1002/14651858.CD006685.pub4. PMID: 27153153Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • ClinVar
      Related medical variations
    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed
    • PubMed (OMIM)
      Related literature resources in PubMed

    Recent activity