Kleine-Levin Syndrome (KLS), also known as “Sleeping Beauty” syndrome, is a rare neurological disorder characterized by recurrent bouts of hypersomnolence (excessive sleeping) along with cognitive and behavioural problems during wakefulness.
The condition is named after Will Kleine and Max Levin, who described cases of the disease in the early 20th century. It was added to the International Classification of Sleep Disorders in 1990.
Many patients experience hyperphagia, hypersexuality and other symptoms. Patients generally experience recurrent episodes of the condition for more than a decade. Individual episodes generally last more than a week but less than a month. The condition greatly affects the personal, professional, and social lives of sufferers, but symptoms spontaneously resolve and seldom cause permanent issues.
The severity of symptoms and the course of the disease vary between sufferers. Patients commonly have about 20 episodes over about a decade. Several months generally elapse between episodes. The onset of the condition usually follows a viral infection; several different viruses have been observed to trigger KLS.
Patients with Kleine-Levin syndrome (KLS) experience reoccurring feelings of excessive tiredness and prolonged sleep (hypersomnia). In most cases, patients sleep 15 to 21 hours a day during episodes. Excessive appetite (hyperphagia) and unusual cravings are present in half to two thirds of cases. About half of patients, mainly male patients, experience dramatically increased sexual urges (hypersexuality).
Patients experience confusion, diminished comprehension, poor concentration, memory problems and difficulty in communicating. Speech impairments are also common such as; monosyllabic, slurred and childish speech. Altered perceptions: most patients report feeling ‘disconnected’ with the real world in some way during an episode, as if their surroundings seemed wrong, distorted, as in a dream. In addition some patients experience auditory and visual hallucinations.
Several other symptoms usually accompany the syndrome, including marked changes in mood. Derealization and severe apathy are present in at least 80 percent of cases. Psychotic symptoms also occur in at least half of patients. Depression and anxiety occur less commonly; one study found them in about 25 percent of patients. Individuals usually cannot remember what happened during episodes.
Other repetitive behaviors and headaches are commonly reported. Some patients act very childlike during episodes, and communication skills and coordination sometimes suffer. In some cases, the autonomic nervous system malfunctions as well.
KLS is very rare, occurring at a rate of one in a million, which limits research into genetic factors. The condition primarily affects adolescent males, although some patients are female and the age of onset varies. As of yet there is no known cure for this syndrome, and there is little evidence supporting drug treatment
Several drug therapies have been used on patients with KLS, but none of them have been subject to randomized controlled trials. A 2013 Cochrane Review concluded that "No evidence indicates that pharmacological treatment for Kleine-Levin syndrome is effective and safe".
In several cases, stimulants, including modafinil, have been reported to have a limited effect on patients, often alleviating sleepiness. They can cause behavioral problems, but they may pose less issues if used in older patients with mild symptoms. In some case reports, lithium has been reported to decrease the length of episodes and the severity of their symptoms and to increase the time between episodes.
It has been reported to be effective in about 25 to 60 percent of cases. Its use carries the risk of side effects in the thyroid or kidneys. Anti-psychotics and benzodiazepines can help alleviate psychotic and anxiety related symptoms, respectively. Carbamazepine has been reported to be less effective than lithium but more effective than some drugs in its class. Electroconvulsive Therapy is not effective and worsens symptoms
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