Abstract
Narcolepsy is a lifelong central hypersomnia characterized by excessive daytime sleepiness,
cataplexy, sleep paralysis, hypnagogic hallucinations, and disrupted nocturnal sleep.
Behavioral and psychiatric comorbidities are often associated clinical features. It
is divided into two subtypes, narcolepsy type-1, and narcolepsy type-2, depending
on the presence of cataplexy and the cerebrospinal fluid hypocretin-1 levels. An autoimmune
process, along with environmental factors, has been hypothesized to cause the disease.
Among children and adolescents, incidence in Europe falls between 0.14 and 0.3 in
100,000, with a reported increase in the incidence after the 2009 H1N1 pandemic influence
and vaccination. Currently, specific pediatric diagnostic criteria and cut-off instrumental
values are lacking. Clinical presentation of the disease may differ between children
and adults, and misdiagnoses or diagnostic delays are still an issue. The treatment
is based on behavioral and pharmacological therapy, but drugs in children are prescribed
off-label. Overall, pathogenic mechanism of narcolepsy and data on drug efficacy in
children are still limited: more research is needed to develop new drugs and to reach
approval of current treatments in the pediatric population.
Keywords
children - sleep - sleepiness - cataplexy - cataplectic facies
