Comparison of clinical characteristics among narcolepsy with and without cataplexy and idiopathic hypersomnia without long sleep time, focusing on HLA-DRB1 ∗1501/DQB1 ∗0602 finding

Abstract

Background Clinical characteristics of narcolepsy without cataplexy (NA w/o CA) and its relation to positivity of HLA-DRB1 ∗1501/DQB1 ∗0602 remain unclarified. We investigated clinical features of NA w/o CA, particularly addressing HLA-DRB1 ∗1501/DQB1 ∗0602. Methods Comparisons of the Epworth Sleepiness Scale (ESS), multiple sleep latency test (MSLT) variables, rapid eye movement (REM)-related symptoms, and treatment response to psychostimulant medication were made for four patient groups (narcolepsy with cataplexy; NA–CA, NA w/o CA HLA-positive, NA w/o CA HLA-negative, and idiopathic hypersomnia without long sleep time; IHS w/o LST). Results Mean sleep latency was significantly shorter and the rate of reduction of ESS after medication was lower in both NA–CA and NA w/o CA HLA-positive groups than those in the IHS w/o LST group. Among the three narcoleptic groups, the NA w/o CA HLA-negative group showed the lowest REM latency and the highest reduction rate of ESS after treatment. Neither these subjective and objective sleepiness measures nor the treatment response measure was significantly different between this group and the IHS w/o LST group. Conclusions In NA w/o CA, HLA-positivity might affect hypersomnia severity and REM propensity. The NA w/o CA HLA-negative group and the IHS w/o LST group exhibit equivalent hypersomnia severity.