The Kleine-Levin syndrome is a rare neurological disease of unknown origin. It is characterised by, among other things, episodes of recurrent hypersomnia.
Hypersomnia and Kleine-Levin syndrome
Hypersomnia can have many facets. In particular, there is a rare neurological disease, affecting only one to two people in a million, called Kleine-Levin syndrome The origin of this neurological disease is still unknown. The origin of this neurological disease is still undetermined, but its main symptoms are as follows:
- Hypersomnia with intense fatigue over a few days to a few weeks;
- Apathy and a sense of derealization;
- Cognitive disorders;
- Confusion in some cases;
- In one out of two patients, gluttony, sexual, anxiety, compulsive or mood disorders, or even hallucinations.
Apart from symptomatic episodes, patients often show no particular signs. The disease most often occurs during adolescence, although cases have been described in children and in the elderly. Over the years, the symptomatic attacks tend to become less frequent and disappear completely after 8 to 13 years of evolution.
Some risk factors and an often difficult diagnosis
While the precise causes of this hypersomnia condition remain mysterious, scientists have identified several risk factors for Kleine-Levin syndrome :
- Male, as two thirds of the patients are boys;
- Perinatal or developmental problems, which increase the risk of developing this syndrome by more than 6 times;
- An Ashkenazi origin, with one sixth of the world's cases occurring in Israel;
- A genetic predisposition, as there are families with several cases of the disease.
Kleine-Levin syndrome is a rare disease, sometimes little known to health professionals. It belongs to the group of hypersomnias of central origin. It is not uncommon for the diagnosis to be made late, after multiple, sometimes invasive medical examinations. However, the diagnosis of Kleine-Levin syndrome is purely clinical, based on the combination of the following symptoms:
- Recurrent hypersomnia, interspersed with phases of remission;
- Cognitive disorders;
- A feeling of derealization.
In Kleine-Levin syndrome, the adolescent sleeps more than 18 hours out of 24 at the time of the seizures. It is possible to wake him up, but he is then so listless and exhausted that he just wants to go back to bed.
No suitable and effective treatment
Currently, there is no specific treatment for Kleine-Levin syndrome. A large number of drugs have been tried to relieve patients' symptoms, but generally with very modest results.
The most commonly used drugs include:
- L'amantadine prescribed during symptomatic episodes of the disease, with limited effectiveness;
- Thymoregulatory drugs, such as lithium or sodium valproate, prescribed as background treatment;
- Medicines to relieve particular symptoms, such as risperidone in case of psychotic disorders or benzodiazepines in case of major anxiety.
Given the poor efficacy of treatments, abstention from treatment is often advocated. However, episodes of hypersomnia can considerably affect the quality of life of adolescents and compromise their schooling and socio-professional integration. Both the patient and his or her family must therefore be given the best possible support, both during and between attacks.
Estelle B., Doctor of Pharmacy
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