Narcolepsy and idiopathic hypersomnolence.

ABSTRACT

Narcolepsy is among the leading causes of excessive daytime sleepiness. Its classic form associates daytime sleepiness with cataplexy, sleep paralysis, hypnopompic hallucinations, and nocturnal disrupted sleep. This form is associated with HLA DQ betal-0602 in about 85% to 90% of affected subjects, independently of their ethnicity. But the definition of the variants of narcolepsy remains controversial, despite the fact that, in some cases, narcolepsy may be limited to daytime sleepiness. In its classic form, it is associated with two or more sleep onset rapid eye movement periods at the Multiple Sleep Latency Test. This test, performed after nocturnal polysomnography, can be helpful in diagnosing narcolepsy, in the absence of a convincing history of partial or complete attacks of cataplexy--a pathognomonic symptom. Investigation of narcoleptic Dobermans has indicated that a muscarinic cholinergic hypersensitivity exists in the brain of affected animals and abnormalities involve also the dopaminergic system. Despite its prevalence of 0.03% to 0.05%, it is still a neurologic entity often missed. Investigations of families of narcoleptics, including monozygotic twins, indicate that this syndrome is polygenic in nature with association of environmental factors. As the peak of onset of disabling symptoms occurs between 15 and 25 years of age, it is important to improve the treatment of this lifelong, disabling illness. Stimulants medications, independently of their mode of action, are prescribed to help daytime sleepiness, and tricyclic antidepressant drugs or serotonergic reuptake blockers are used on the other symptoms. But these medications have a limited efficacy. Short naps at regular intervals during the day are a strong therapeutic adjuvent.