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Kleine-Levin syndrome

Last edited: 4/15/2026

Overview

Kleine-Levin syndrome (KLS) is a rare neurological disorder characterized by recurrent episodes of excessive sleep (hypersomnia), cognitive disturbances, and behavioral changes. 12

Diagnosis

  • Key Diagnostic Criteria: Periodic hypersomnia, cognitive impairment, behavioral changes.
  • Recommended Tests: Polysomnography to rule out other sleep disorders; complete during and between hypersomnolentic episodes if possible 1.
  • Grading: No standardized grading system; clinical criteria are primarily used 1.
  • Management

  • First-Line Treatments: No specific first-line treatments universally recommended; symptomatic management with stimulants like modafinil may be considered 1.
  • Adjunctive Treatments: Addressing triggers; in one case, discontinuation of isotretinoin was associated with symptom resolution 2.
  • Special Populations

  • Isotretinoin Treatment: Potential triggering role noted in one case report; caution advised in patients on retinoids 2.
  • Key Recommendations

  • Further investigate breathing abnormalities during hypersomnolentic episodes via comprehensive polysomnography to clarify their association with KLS (Evidence: Moderate 1).
  • Consider the possibility of isotretinoin triggering KLS onset and monitor patients on retinoids closely for symptom development (Evidence: Weak 2).
  • Symptomatic treatment with stimulants like modafinil may be beneficial for managing hypersomnia in KLS, though evidence is limited (Evidence: Expert opinion).
  • References

    1 Gadoth N. Breathing abnormalities during sleep in Kleine-Levin Syndrome: fact or coincidence?. Current opinion in pulmonary medicine 2018. link 2 Smedje H, Schwan S, Hallberg E, Hallberg P. Onset of Kleine-Levin Syndrome in association with isotretinoin treatment. Acta paediatrica (Oslo, Norway : 1992) 2010. link

    Original source

    1. [1]
      Breathing abnormalities during sleep in Kleine-Levin Syndrome: fact or coincidence?Gadoth N Current opinion in pulmonary medicine (2018)
    2. [2]
      Onset of Kleine-Levin Syndrome in association with isotretinoin treatment.Smedje H, Schwan S, Hallberg E, Hallberg P Acta paediatrica (Oslo, Norway : 1992) (2010)

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