A 10-Year Longitudinal Observational Study Of Cataplexy In A Cohort Of Narcolepsy Type 1 Patients

Nat Sci Sleep. 2019 Oct 21;11:231-239. doi: 10.2147/NSS.S229105. eCollection 2019.


Purpose: Narcolepsy type 1 (NT1) is thought to have a chronic persistent course. This study aimed to assess the natural course of cataplexy in patients with NT1 at 2, 6, and 10 years after stabilizing symptoms. Other secondary objectives included assessing sleep quality, body mass index (BMI), and comorbidities at recruitment and 10 years later.

Patients and methods: Cataplexy symptoms, the Epworth sleepiness scale (ESS), sleep quality (assessed using the Pittsburgh sleep quality index [PSQI]), BMI, and comorbid conditions were prospectively monitored in 38 patients with NT1. The study sample comprised 38 patients with narcolepsy (males=27). The mean ages at disease onset and recruitment were 17.7 ± 5.6 years and 24.3 ± 8.6 years, respectively.

Results: In 42% of the cohort, the anti-cataplectic medications were stopped at the end of the study without disturbing symptoms of cataplexy. Additionally, there was an apparent significant reduction in the frequency of cataplexy over time. The mean ESS score decreased by more than 4 points from 19.4 ± 2.9 to 15 ± 4.3 (p<0.001) while on the same pharmacotherapy. The number of patients with a PSQI score of <5 (indicating good sleep quality) increased from 6 (15.8%) to 15 (39.5%) (p=0.004). The BMI increased from 30 ± 5.1 to 33.3 ± 6 kg/m2 (p=0.001). No changes were documented in comorbidities.

Conclusion: The findings suggest that the course of NT1 is not stable. Over a 10-year period, cataplexy symptoms improved or disappeared in a large proportion of patients, and there was an improvement in daytime sleepiness and nighttime sleep quality. More prospective studies that repeatedly monitor CSF-HCRT are needed to confirm the current findings.

Keywords: Pittsburgh Sleep Quality Index; body mass index; daytime sleepiness; sleep quality.