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

Alexander Kuts; Michael Poluektov; Alexander Zakharov; Vlada Govzman; Irina Ponomareva; Eduard Yakupov; Irina Zavalko; Olga Tikhomirova; Yurii Sviryaev; Alexey Yakovlev; Alexander Polyakov; Alexander Melnikov; Claudio L A Bassetti

doi:10.5664/jcsm.10340

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

J Clin Sleep Med. 2023 Feb 1;19(2):355-359. doi: 10.5664/jcsm.10340.

Authors

Affiliations

¹ Sechenov University, Moscow, Russia.
² Department of Neurology and Neurosurgery, Samara State Medical University, Samara, Russia.
³ Samara State Medical University, Research Institute of Neurosciences, Samara, Russia.
⁴ Clinical Hospital "RZD-Medicine", Khabarovsk, Russia.
⁵ Regional Clinical Hospital No. 3, Chelyabinsk, Russia.
⁶ Kazan State Medical University, Kazan, Russia.
⁷ Research Center of Neurology, Moscow, Russia.
⁸ Nikiforov Russian Center of Emergency and Radiation Medicine, EMERCOM of Russia, Saint-Petersburg, Russia.
⁹ Almazov National Medical Research Centre, Saint-Petersburg, Russia.
¹⁰ Novosibirsk State Medical University, Novosibirsk, Russia.
¹¹ V.M. Bekhterev National Research Medical Center for Psychiatry and Neurology, Saint-Petersburg, Russia.
¹² FSBI NMRCO FMBA Russia, Moscow, Russia.
¹³ University Hospital (Inselspital), Bern, Switzerland.

Abstract

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 ± 16.9 years (± here and further indicates standard deviation), 58% males, and 42% females. Narcolepsy started at a mean age of 25.6 ± 14.6 years (range 5-74 years). The mean Epworth Sleepiness Scale score was 18.4 ± 3.5 points (range: 11-24). Sleep paralysis was reported by 59.1%, and hallucinations by 82% of patients. In MSLT, ≥ 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.

Citation: Kuts A, Poluektov M, Zakharov A, et al. Clinical and neurophysiological characteristics of 89 patients with narcolepsy and cataplexy from the Russian Narcolepsy Network. J Clin Sleep Med. 2023;19(2):355-359.

Keywords: MSLT; PSG; Russia; cataplexy; excessive daytime sleepiness; narcolepsy.

Publication types

Clinical Trial

MeSH terms

Adolescent
Adult
Aged
Cataplexy* / complications
Child
Child, Preschool
Disorders of Excessive Somnolence*
Female
Humans
Male
Middle Aged
Narcolepsy* / complications
Narcolepsy* / diagnosis
Sleep
Sleep, REM / physiology
Young Adult

Associated data

ClinicalTrials.gov/NCT05375890