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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:
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
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
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

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
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: clues to aetiology.
AlShareef SM, Smith RM, BaHammam AS
Sleep Breath 2018 Sep;22(3):613-623. Epub 2018 Mar 12 doi: 10.1007/s11325-017-1617-z. PMID: 29532411Free PMC Article
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
Kleine-Levin Syndrome.
Arnulf I
Sleep Med Clin 2015 Jun;10(2):151-61. doi: 10.1016/j.jsmc.2015.02.001. PMID: 26055863

Therapy

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
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
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

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
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
Self-representation in Kleine-Levin syndrome: a single case fMRI study.
Desaunay P, Dégeilh F, Guénolé F, Eustache F, Viard A, Bon L, Dayan J, Baleyte JM, Guillery-Girard B
Neurocase 2021 Oct;27(5):396-406. Epub 2021 Sep 19 doi: 10.1080/13554794.2021.1974488. PMID: 34541988
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
The Kleine-Levin syndrome. Some further observations.
Garland H, Sumner D, Fourman P
Neurology 1965 Dec;15(12):1161-7. doi: 10.1212/wnl.15.12.1161. PMID: 5295527

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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