Clinical and neurophysiological characteristics of 89 patients with narcolepsy and cataplexy from the Russian Narcolepsy Network

Study Objectives: The first reports of narcolepsy with cataplexy in Russia were made by Mankovsky (The pathogenesis of narcolepsy (the case of epidemic encephalitis with cataplexy) published in the Sovremennaya psihonevrologia) in 1925. The largest series of patients (n = 110) was reported by A. Vein (doctoral thesis: Hypersomnia Syndrome) in 1964. However, until today, narcolepsy remained relatively unknown in Russia. The aim of this study is to report clinical and polysomnography (PSG)/multiple sleep latency test (MSLT) results in the Russian population and compare them with the European Narcolepsy Network (EU-NN) data (n = 1099) reported. Methods: Eleven sleep centers from Russia agreed to participate and completed a questionnaire including 58 questions concerning demographic, clinical, PSG, and MSLT data. Results: There were 89 patients with a mean age of 35.6 & PLUSMN; 16.9 years (& PLUSMN; here and further indicates standard deviation), 58% males, and 42% females. Narcolepsy started at a mean age of 25.6 & PLUSMN; 14.6 years (range 5-74 years). The mean Epworth Sleepiness Scale score was 18.4 & PLUSMN; 3.5 points (range: 11-24). Sleep paralysis was reported by 59.1%, and hallucinations by 82% of patients. In MSLT, & GE; 2 sleep-onset REM (rapid eye movement) periods (SOREMPs) were found in 81.6%. No center provided human leukocyte antigen (HLA) or cerebral spinal fluid hypocretin data. Conclusions: Clinical and neurophysiological data from this first study of the Russian Narcolepsy Network suggest a similar profile to the recently reported EU-NN data. The more severe and higher percentage of patients with cataplexy and presenting with both excessive daytime sleepiness and cataplexy may reflect low awareness of narcolepsy in Russia. Clinical Trial Registration: Registry: ClinicalTrials.gov; URL: https://clinicaltrials.gov/ct2/show/NCT05375890; Name: Clinical and Neurophysiological Characteristics of Narcolepsy; Identifier: NCT05375890.

Automated summary

This study aims to report the clinical and polysomnography/multiple sleep latency test (MSLT) results of narcolepsy in the Russian population and compare them with the data from the European Narcolepsy Network (EU-NN). The results of this first study of the Russian Narcolepsy Network suggest a similar profile to the recently reported EU-NN data, indicating a low awareness of narcolepsy in Russia.