The clinical and polygraphic characteristics of narcolepsy in children were established on the analysis of 97 reported cases in children (including 12 personal cases). In idiopathic narcolepsies (77 cases) narcoleptic attacks occurred in 97% of the cases, cataplexy in 80.5%, hypnagogic hallucination in 39% and sleep paralysis in 29%; 13% of the children had the tetrad; dyssomnia was a prominent feature. Polygraphic data showed no significant differences between adults and children. In symptomatic narcolepsies (20 cases): cataplexy was the prominent feature occurring in 95% of the cases, 26% of the children had status cataplecticus; in these narcoleptic-cataplectic syndromes there was often an absence of polygraphic evidence of narcolepsy. Symptomatic narcolepsy should be suspected in cases where narcolepsy is detected in preteenage children, where cataplectic attacks are abnormally frequent, where there is an absence of polygraphic evidence of classical narcolepsy (although this criterion may not apply in the case of younger children) or where human leukocyte antigen typing for DR2 is negative. An association with a Niemann-Pick disease type C was found in 12 out of the 20 symptomatic cases, this association merits further study.

Charles University Prague

FLNI Buenos Aires

Unité 52 Professeur M Jouvert

Unite de Sommeil Hopital Lyon Sud Pierre Benite France