Kleine-Levin syndrome

Kleine-Levin syndrome

Infobox_Disease
Name = PAGENAME


Caption =
DiseasesDB = 29520
ICD10 = ICD10|G|47|8|g|40
ICD9 = ICD9|327.13
ICDO =
OMIM = 148840
MedlinePlus =
eMedicineSubj =
eMedicineTopic =
MeshID = D017593

Kleine-Levin Syndrome, or KLS, is a rare disorder characterized by the need for excessive amounts of sleep (hypersomnia), (e.g. up to 20 hours a day); excessive food intake (compulsive hyperphagia); and an abnormally uninhibited sexual drive. Adolescent males are the predominant victims of the disorder. There are only about 500 known sufferers of this condition in the world. However, because Kleine Levin is sporadic in its appearances, some doctors believe that this number is, in reality, much higher and the reason that it is not publicly noted is because this disease is extremely hard to diagnose.

Presentation

Individual sufferers may often become irritable, lethargic, and/or apathetic. KLS patients may appear disoriented and report hallucinations. Symptoms are cyclical; with days to weeks (even up to months) of suffering interspersed by weeks or months (even up to years) symptom-free. Although resolution of the disorder may occur for some in later life, this is not universal.

Causes

While some researchers speculate of a hereditary predisposition; others believe the condition may be the result of an autoimmune disorder. [ [http://med.stanford.edu/school/Psychiatry/narcolepsy/KLS.html Kleine-Levin Syndrome - Center for Narcolepsy - Stanford University School of Medicine ] ] Both proposals need not be mutually exclusive with the result being a malfunction of the portion of the brain that helps to regulate functions such as sleep, appetite, and body temperature (hypothalamus).

Similarities between KLS and Klüver-Bucy syndrome (another rare condition characterized by hyperphagia, hypersexuality, and emotional blunting) may warrant further attention.

Treatment

There is no definitive treatment for Kleine-Levin syndrome. Stimulants, including amphetamines, methylphenidate, imipramine and modafinil, administered orally, are used to treat sleepiness. Because of similarities between Kleine-Levin syndrome and certain mood disorders, lithium and carbamazepine may be prescribed. Responses to treatment have often been limited. This disorder needs to be differentiated from cyclic re-occurrence of sleepiness during the premenstrual period in teenage girls that may be controlled with hormonal contraception. There is no treatment for Kleine-Levin syndrome as of yet however, stimulants are often used to keep the patient awake. This does not alter their mental state. [ [http://www.ninds.nih.gov/disorders/kleine_levin/kleine_levin.htm Kleine-Levin Syndrome Information Page: National Institute of Neurological Disorders and Stroke (NINDS) ] ]

Eponym

It is named for Willi Kleine and Max Levin. [ 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.]

References

External links

* [http://www.klsfoundation.org The KLS Foundation]
*NINDS|kleine_levin


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