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8.85: Kleine Levin Syndrome (Sleeping Beauty Syndrome

  • Page ID
    23281
  • Proposed addition to DSM-V.

    Criteria

    A. The patient experiences recurrent episodes of excessive sleep (>11 hours/day).

    B. Episodes occur at least once a year, and are generally 2 days to 4 weeks in duration.

    C. During episodes, when awake, cognition is abnormal with feeling of unreality or confusion. Behavioral abnormalities such as megaphagia or hypersexuality may occur in some episodes.

    D. The patient has normal alertness, cognitive functioning, and behavior between the episodes.

    E. The condition is not better accounted for by another mental disorder (e.g, mood disturbance), and is not due to the direct physiological effects of a substance (e.g., a drug of abuse, a medication) or another general medical condition (e.g. a metabolic disorder).

    Treatment:

    Treatment for KLS has not been established, although lithium has been used in limited cases with insignificant efficacy. Gabapentin has shown some promise in being effective for the prevention of hypersomnia attacks. There is considerable speculation that the recurrent hypersomnia and behavior disturbance are related to epilepsy-like neuronal discharge from the thalamus due to dysfunction in GABAnergic receptors (Itokawa et al., 2009).
    For more information, check out the KLS Foundation: http://www.kleinelevin.com/

    Dsm5 proposed changes (dsm5.org)

    A. The patient experiences recurrent episodes of excessive sleep (>11 hours/day).

    B. Episodes occur at least once a year, and are generally 2 days to 4 weeks in duration.

    C. During episodes, when awake, cognition is abnormal with feeling of unreality or confusion. Behavioral abnormalities such as megaphagia or hypersexuality may occur in some episodes.

    D. The patient has normal alertness, cognitive functioning, and behavior between the episodes.

    E. The condition is not better accounted for by another mental disorder (e.g, mood disturbance), and is not due to the direct physiological effects of a substance (e.g., a drug of abuse, a medication) or another general medical condition (e.g. a metabolic disorder).

    Rationale for change

    • There is increasing evidence that the disorder is a genuine disease entity based on its consistent description in term of clinical presentation, demographics (70% male, adolescent), evolution (eventually disappears), and therapeutic response (almost nothing is effective). It may be misdiagnosed as depression or other psychiatric consequences, with devastating consequences.
    • Relationship to International Classification of Diseases- 10
    • Other Sleep Disorders: Kleine-Levin Syndrome G47.8
    • Relationship to International Classification of Sleep Disorders 2nd Edition
    • Recurrent Hypersomnia 327.13

    Severity

    Recommendations for severity criteria for this disorder are forthcoming.

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