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Kleine-Levin Syndrome, or KLS, is a rare neurological disorder characterized by recurring periods of excessive amounts of sleep and altered behavior. At the onset of an episode the patient becomes drowsy and sleeps for most of the day and night (hypersomnolence), waking only to eat or go to the bathroom. When awake, the patient’s whole demeanor is changed, often appearing “spacey” or childlike. When awake, they experiences confusion, disorientation, complete lack of energy (lethargy), and lack of emotions (apathy). Individuals are not able to attend school, work or care for themselves. Most are bedridden, tired and uncommunicative even when awake.
Most patients report that everything seems out of focus, and that they are hypersensitive to noise and light. In some cases, food cravings (compulsive hyperphagia) are exhibited. Instances of uninhibited hypersexuality during episodes (mostly males) and instances of depression (mostly females) have also been reported.
Affected individuals may go for a period of weeks, months or even years without experiencing any symptoms, and then they reappear with little warning. In between episodes those diagnosed with KLS appear to be in perfect health with no evidence of behavioral or physical dysfunction.
The cause of Kleine-Levin Syndrome is not known. Thus, family support and education are the best management currently available.[1] [2]
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.
While some researchers speculate that there may be a hereditary predisposition, others believe the condition may be the result of an autoimmune disorder.[3] 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).[4] Recent studies also suggest that there may be a link to a deficiency of dopamine transporter density in the lower striatum.[5]
A 2008 study of 108 KLS patients found that the majority of cases were present in adolescent males, with a mean sex ratio of 3:1. The average age of onset was 15.7 years, with 81.7% experiencing their first episode between 10 and 20 years of age. Age ranged from 6 to 59 years when patients experienced their first episode. Females tended to be slightly older than males at first onset, even though they tended to experience puberty earlier. In the US population, KLS presents in Caucasians with three times the expected frequency, and with six times the expected frequency in those of Jewish heritage.[6]
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