Recurrent hypersomnia

Nature and cause - Clinical signs / Clinical forms - Assessment / Management

Nature and cause

Recurrent hypersomnia is rare, of variable duration and periodicity, affecting mainly boys in their second decade.

The cause remains mysterious but the most likely hypothesis is that of a hypothalamic dysfunction of autoimmune origin occurring during the period of brain maturation.

Clinical signs / Clinical forms

Kleine-Levin syndrome

This is the most classic clinical picture, characterised by recurrent sleep attacks, disturbances in eating and sexual behaviour (inconsistent), and psychological disturbances during the sleep attacks, contrasting with normal alertness and behaviour in between.

A viral trigger is often found in the weeks preceding the sleep attacks.

Other clinical forms

There are clinical variants of Kleine-Levin syndrome, including incomplete forms without eating or sexual behaviour disorders.

In girls, periodic menstrual hypersomnia is sometimes found.

Finally, rare recurrent hypersomnias of psychiatric origin occurring in the context of bipolar disease or major depression have been described.

The evolution is usually spontaneously favourable after several years of recurrent sleep attacks.

Assessment / Management

Balance sheet

The diagnosis is purely clinical. Sometimes there is an association with HLA DQB1*0201 typing.

Support

Depending on the recurrence and severity of the episodes, preventive treatment may be offered, based on carbamazepine, sodium valproate or lithium.
In the case of periodic menstrual hypersomnia, a normodose oestroprogestative preventive treatment will most often be proposed.
Treatment of sleep attacks with modafinil is mostly ineffective.