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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
Episodic hypersomnia
MedGen UID:
870479
•Concept ID:
C4024925
•
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
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
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
Central Disorders of Hypersomnolence.
Trotti LM
Continuum (Minneap Minn) 2020 Aug;26(4):890-907. doi: 10.1212/CON.0000000000000883. PMID: 32756227Free 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

Diagnosis

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
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
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
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
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

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
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
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
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
Kleine-Levin syndrome: current status.
Huang YS, Lakkis C, Guilleminault C
Med Clin North Am 2010 May;94(3):557-62. doi: 10.1016/j.mcna.2010.02.011. PMID: 20451032

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
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; 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
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

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
Safety and efficacy of pitolisant in children aged 6 years or older with narcolepsy with or without cataplexy: a double-blind, randomised, placebo-controlled trial.
Dauvilliers Y, Lecendreux M, Lammers GJ, Franco P, Poluektov M, Caussé C, Lecomte I, Lecomte JM, Lehert P, Schwartz JC, Plazzi G
Lancet Neurol 2023 Apr;22(4):303-311. doi: 10.1016/S1474-4422(23)00036-4. PMID: 36931805
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
Sleep-Wake Disorders in Childhood.
Licis A
Continuum (Minneap Minn) 2020 Aug;26(4):1034-1069. doi: 10.1212/CON.0000000000000897. PMID: 32756235
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

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

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      Related records in OMIM
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