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Kleine-Levin syndrome

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A strange condition of unknown aetiology which almost exclusively affects young males aged 15-25 years. Characterized by periods lasting from days to weeks of attacks of somnolence accompanied by excessive food intake, and various mental symptoms. Common mental symptoms are confusion, irritability, restlessness, euphoria, hallucinations, delusions, and schizophreniform states. Attacks may occur every 3 to 6 months and last from 2 to 3 days. Between attacks the patients recover completely and the sleep periods usually disappear in adult age. This syndrome may easily be confused for other neurological, metabolical or psychiatric disease

Willi Kleine described a series of nine cases of recurrent hypersomnia in 1925. In 1936, Max Levin added a series of five other cases, focusing on the relationship between hypersomnia and disturbed eating. In 1962, Critchley, who gave the disorder its name, added to the literature 11 cases he had personally observed as a physician for the British Royal Navy. In a recent review of the literature, Arnulf et al compiled 186 cases dating from 1962 to 2004. Huang and Arnulf report on a description of KLS written by Alexandre Jacques François Brierre de Boismont (1798-1881).

The syndrome was first described in 1786 by the French physician Edmé Pierre Chauvot de Beauchêne (1748-1824) who specialised in women's diseases. He took a special interest in the case of a 26 year old woman. Between the ages of 7 and 11, she had experienced episodes of extensive erysipelas, accompanied by fever, digestive problems, headaches and convulsions. "In her fourteenth year, she was overcome with a lethargic sleep which lasted several days, and it was so profound that she was believed dead. From that point forward, the affection of sleep recurred at regular intervals; it usually lasted eight to ten days, continuing at times for fifteen; and upon one sole occasion. It persisted into the seventeenth day."

Beauchêne first examined the patient when she came to Paris at the age of 24 years. He personally observed four episodes of hypersomnia, lasting between 24 hours and 8 days. He reports: "During the first years of her disease, this girl had appetites as bizarre as they were dangerous, causing her to eat lime, plaster, soil and vinegar. Thereafter these appetites subsided, and she nourished herself indiscriminately with all sorts of aliment, excepting bread, for which she maintained an insuperable loathing till she was perfectly cured. This food always occasioned vomiting."

We thank Olivier Walusinski for information submitted.

Bibliography

  • E. P. Chauvot de Beauchêne:
    Observation sur une maladie nerveuse. Avec complication d'un sommeil tantôt léthargique, tantôt convulsif.
    A Amsterdam et à Paris: chez Méquignon l'aine, 1786.
    [Observation of a nervous disease attended by disturped sleep, at times lethargic and at times convulsive.]
  • A. Brierre de Boismont:
    Des hallucinations ou histoire raisonnée des apparitions des visions, des songes, de l'extase, des rêves, du magnetisme et du somnambulisme.
    Paris, 1845, 1852, 1861.
    On Hallucinations: A History and Explanation. London: Renshaw, 1859.
  • W. Kleine:
    Periodische Schlafsucht.
    Monatsschrift für Psychiatrie und Neurologie, 1925, 57: 285-320.
  • M. Levin:
    Periodic somnolence and morbid hunger: A new syndrome.
    Brain, Oxford, 1936, 59: 494-504.
  • M. Critchley, H. Hoffman:
    The syndrome of periodic somnolence and morbid hunger (Kleine-Levin syndrome).
    British Medical Journal, London, 1942, 1: 137-139.
  • M. Critchley:
    Periodic hypersomnia and megaphagia in adolescent males.
    Brain, Oxford, 1962, 85: 627-656.
  • I. Arnulf, J. M. Zeitzer, J. File et al:
    Kleine-Levin syndrome: a systematic review of 186 cases in the literature.
    Brain, 2005, 128: 2763-2776.
  • Y. S. Huang, I. Arnulf:
    The Kleine-Levin syndrome. Sleep Medicine Clinics, 2006, 1: 89-103.
  • Olivier Walusinski:
    Historical note. Observation of a nervous disease attended by disturbed sleep, at times lethargic and at times convulsive. Edmé Pierre Chauvot de Beauchêne.
    Journal of Neurology, Neurosurgery, and Psychiatry, September 2007.

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