Narcolepsy is characterized by excessive daytime sleepiness, with or without 
cataplexy. Associated features include sleep paralysis, hypnagogic or 
hypnopompic hallucinations, and disturbed nocturnal sleep. Narcolepsy is 
strongly associated with the HLA DQB1*0602 allele, and its symptoms stem from 
destruction of hypocretin-secreting neurons in the hypothalamus. Recently 
identified autoantibodies to Tribbles homologue 2 in some patients, as well as 
cases associated with H1N1 vaccination, support an autoimmune mechanism. There 
are many challenges in diagnosing and treating pediatric narcolepsy. Caution 
must also be used in interpreting polysomnography and multiple sleep latency 
test results in children. HLA testing is nonspecific, and no commercial test 
exists to measure cerebrospinal fluid hypocretin levels. Neuroimaging has not 
yet proven useful in primary narcolepsy. Treatment of sleepiness and cataplexy 
in children requires extrapolating from adult studies. Hopefully, further 
insights into the pathophysiology of narcolepsy will allow for new therapeutics 
to manage the symptoms and modify the course of the disease.