Narcolepsy in childhood and adolescence

The present review article discusses the most important aspects of narcolepsy in children. The main objective of this review is to describe the clinical and laboratory characteristics of narcolepsy in patients within a targeted age range and to discuss hypotheses regarding the physiopathology of this disease. Excessive daytime sleepiness is reported by up to 20% of schoolchildren and adolescents. In 16% of these cases, narcolepsy begins before the age of 10 years, whereas 30% of narcoleptic patients exhibit its initial symptoms during childhood. A delayed diagnosis might lead to severe, negative future consequences for the affected patients. Human narcolepsy is a complex disease. The association of HLA-DQB1*0602 with low hypocretin levels indicates a genetic susceptibility with an associated immune component. Narcolepsy is characterized by excessive daytime sleepiness and cataplexy and might be associated with hypnagogic hallucinations, sleep paralysis, and sleep fragmentation. The diagnosis of narcolepsy depends on the clinical assessment and the performance of multiple sleep latency tests preceded by polysomnography. In children, the search for secondary causes of narcolepsy is important because approximately 25% of these patients are symptomatic. The treatment of narcolepsy in children is basically symptomatic, and most cases require behavioral and pharmacological approaches. New therapeutic modalities which impede progression of the disease at the onset of symptoms have also been investigated.