Early- and late-onset narcolepsy: possibly two distinct clinical phenotypes

Abstract

Purpose: To investigate the clinical characteristics and the risk factors associated with excessive daytime sleepiness (EDS) in patients with early- and late-onset narcolepsy.

Methods: Patients with narcolepsy were consecutively recruited. All patients were separated into early- and late-onset groups according to the onset age of disease ≤ 15 and > 15 years, respectively. Demographic, clinical, and sleep parameters were compared between the two groups. Linear regressions were performed to examine the risk factors of subjective and objective EDS in patients with early- and late-onset narcolepsy.

Results: A total of 101 patients with narcolepsy (median age at recruitment = 18.0 years) were classified into an early-onset group (67 patients with median age at onset = 12.0 years) and a late-onset group (34 patients with median age at onset = 28.5 years). Compared with early-onset group, late-onset group scored significantly higher on Epworth Sleepiness Scale (ESS), Ullanlinna Narcolepsy Scale (UNS), sleep paralysis, rapid eye movement (REM) sleep behavior disorder (RBD) questionnaire-Hong Kong (all P < 0.050). UNS-cataplexy and sleep paralysis had significantly positive associations with subjective EDS, and N1%, arousal index, and periodic limb movements index were positively associated with objective EDS in the early-onset group (all P < 0.050). However, these associations were not observed in late-onset narcolepsy.

Conclusion: Late onset narcolepsy had more severe self-reported narcolepsy symptoms. REM sleep related symptoms and disrupted nighttime sleep were associated with EDS in early-onset narcolepsy. These findings suggest that early- and late-onset narcolepsy may represent two distinct phenotypes.

Keywords: Cataplexy; Excessive daytime sleepiness (EDS); Multiple sleep latency test (MSLT); Narcolepsy; Onset age.