The clinical and polygraphic characteristics of narcolepsy in children
were established on the analysis of 97 reported cases in children (in
cluding 12 personal cases). In idiopathic narcolepsies (77 cases) narc
oleptic attacks occurred in 97% of the cases, cataplexy in 80.5%, hypn
agogic hallucination in 39% and sleep paralysis in 29%; 13% of the chi
ldren had the tetrad; dyssomnia was a prominent feature. Polygraphic d
ata showed no significant differences between adults and children. In
symptomatic narcolepsies (20 cases): cataplexy was the prominent featu
re occurring in 95% of the cases, 26% of the children had status catap
lecticus; in these narcoleptic-cataplectic syndromes there was often a
n absence of polygraphic evidence of narcolepsy. Symptomatic narcoleps
y should be suspected in cases where narcolepsy is detected in preteen
age 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 assoc
iation with a Niemann-Pick disease type C was found in 12 out of the 2
0 symptomatic cases, this association merits further study.