Definition of Kleine-Levin Syndrome

This edited definition of Kleine-Levin Syndrome was obtained from the Rare Disease Database of the National Organization of Rare Diseases (NORD). Be aware that not everyone fits neatly into this definition. It is just a general definition.

Kleine-Levin Syndrome is a rare disorder characterized by the need for excessive amounts of sleep. The patient becomes progressively more drowsy and sleeps for most of the day and night, (hypersomnolence), and in some cases requires excessive food intake (compulsive hyperphagia). The disorder primarily strikes adolescents. When awake, affected individuals may exhibit irritability, lack of energy (lethargy),  lack of emotions (apathy) and they may appear confused  (disoriented).  Many patients report a hypersensitivity to noise. Some patients show an abnormally uninhibited sexual drive. Symptoms of Kleine-Levin Syndrome are cyclical. An affected individual may go for weeks or months without experiencing any symptoms, with perfect health and no evidence of behavioral or physical dysfunction. When present, KLS symptoms may persist for days to weeks or even months. The exact cause of Kleine-Levin Syndrome is not yet known. It is thought that symptoms of Kleine-Levin Syndrome may be related to malfunction of the portions of the brain (hypothalamus) that help to regulate functions such as sleep, appetite, and body temperature. It appears to be self limiting with cessation of episodes by early adult life."

Medication Management

It is important to remember that only a handful of doctors in the world have ever seen a patient with KLS. Therefore, it is likely that your doctor is getting his information from the currently available information, which may not always be accurate. This is due to the limited number of patients studied and the limited length of these studies. KLS episodes can be separated by many months. Therefore a doctor can prescribe a medication to a patient, who then does not have an episode for a number of months. This doctor assumes that he has cured the patient and publishes a paper to that effect. Unfortunately, if the patient continues to have episodes after the paper is published, the article is still taken as fact causing more patients to be put on a drug that is ineffective.

Additionnal Information

KLS Diary - Jeff

KLS Links