Central
hypersomnias principally involves type 1
narcolepsy (NT1), type 2
narcolepsy (NT2) and
idiopathic hypersomnia (IH). Despite great progress made in understanding the physiopathology of NT1 with low cerebrospinal fluid
hypocretin-1 levels, current treatment remains symptomatic. The same applies to NT2 and IH, for which the physiopathology is still largely unknown. Controlling
excessive daytime sleepiness (EDS),
cataplexy,
hypnagogic hallucinations,
sleep paralysis and disturbed night-time sleep are key therapeutic targets in NT1. For IH and NT2, reducing EDS is the main objective. Based on European and American directives for the treatment of
narcolepsy, we propose French recommendations for managing central
hypersomnias as well as strategies in the case of drug-resistance. Stimulating treatments target EDS, and
Modafinil is the first-line treatment. Other stimulants such as
methylphenidate,
pitolisant, and exceptionally
dextro-amphetamine can be prescribed. Selective
serotonin and
noradrenaline reuptake inhibitor
antidepressants are effective for the management of
cataplexy in NT1.
Sodium oxybate is an effective treatment for several symptoms, including EDS,
cataplexy and disturbed night-time sleep. Treatment of central
hypersomnia must also take into consideration frequent cardiovascular, metabolic and psychiatric comorbidities, particularly in NT1. New
therapies are currently under study with the development of new stimulants and anti-cataplectics. The next few years will see innovative emerging
therapies, based on a physiopathological approach, aiming to restore hypocretinergic transmission or to interrupt the autoimmune processes causing the loss of
hypocretin neurons.