Objectives The purpose of our study was to describe the behavioral aspects and quality of life of childhood narcolepsy with cataplexy (NC).

Methods We performed a case-control study based on self-administered questionnaires in 30 NC hypocretin-deficient patients, 39 epilepsy patients, and 39 healthy controls matched for sex and age.

Results Our population of children and adolescents with NC showed an increase in internalizing problems in line with previous reports, typically represented by withdrawal and depression symptoms, and somatic complaints. The two patients groups share higher scores than controls for anxiety disorders, attention, social and oppositional-defiant problems. Psychopathological profile in NC were found to be positively correlated with early NC onset, diagnostic delay, nocturnal disturbed sleep, shorter sleep latency and greater number of Sleep Onset REM Periods at Multiple Sleep Latency Test. On the other hand, treatment and disease duration, positively influenced the behavioral evolution. The psychosocial health of pediatric NC also turned out to be worse than in healthy controls, while the physical health showed no significant differences.

Conclusions We found a specific psychopatological profile in a large pediatric NC sample, compared with another neurological chronic disease (epilepsy) and healthy controls. Symptoms of withdrawal, depression and somatic complaints, were specific of NC, and not observed in the two other groups. Effective treatment, and self-awareness of the disease should be promoted in NC children for the positive impact on behaviour and psychosocial health.