Clinical and Neurophysiological Characteristics of Narcolepsy

Narcolepsy Type 1 Clinical Trial

Official title:

Clinical and Neurophysiological Characteristics of Narcolepsy in Russia Basing on Clinical, Polysomnographic and Genome-wide Association Analyses of Narcolepsy With Cataplexy: a European Narcolepsy Network Study of 2013

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT05375890
• Other study ID #: 246188
• Status: Completed
• Start date: November 1, 2019
• Est. completion date: September 28, 2023

Study information

• Verified date: May 2022
• Source: I.M. Sechenov First Moscow State Medical University
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational [Patient Registry]

Clinical Trial Summary

The study is the first attempt in post-Soviet Russian history to collect and analyze the existing available data of narcolepsy cohort in order to get prove characteristics of narcolepsy in Russia according to known data. Investigators created the system of national narcolepsy centers in Russia - Russian narcolepsy network, with a purpose of collecting clinical and neurophysiological data with subsequent analyze and formation of Russian narcolepsy profile.

Description:

First reports of narcolepsy with cataplexy in Russia were made by Mankovsky in 1925 (narcolepsy with cataplexy, NC). The largest series of patients (n=110) was reported by A. Vein in 1964. Narcolepsy remains however until today relatively unknown in Russia. The aim of this study is to report clinical and polysomnographic (PSG)/multiple sleep latency test (MSLT) results in a Russian population and compare them with the European narcolepsy network (n=1099) reported (Luca G, Haba-Rubio J, Dauvilliers Y, Lammers GJ, Overeem S, Donjacour CE, Mayer G, Javidi S, Iranzo A, Santamaria J, Peraita-Adrados R, Hor H, Kutalik Z, Plazzi G, Poli F, Pizza F, Arnulf I, Lecendreux M, Bassetti C, Mathis J, Heinzer R, Jennum P, Knudsen S, Geisler P, Wierzbicka A, Feketeova E, Pfister C, Khatami R, Baumann C, Tafti M; European Narcolepsy Network. Clinical, polysomnographic and genome-wide association analyses of narcolepsy with cataplexy: a European Narcolepsy Network study. J Sleep Res. 2013 Oct;22(5):482-95. doi: 10.1111/jsr.12044. Epub 2013 Mar 18. PMID: 23496005., 2013).

For the purpose of this study only patients with narcolepsy with cataplexy were considered because of the uncertainty about the diagnosis of narcolepsy without cataplexy.

In order to create a network of sleep centers with expertise/interest in narcolepsy 79 centers from 27 Russian cities were connected in 2019. A total of 11 centers officially joined the Russian Narcolepsy Network (rnane.ru) and agreed to participate in the current study. A standardized questionnaire, similar to the one reported by the EU-NN in a series of 1099 patients (Luca G, Haba-Rubio J, Dauvilliers Y, Lammers GJ, Overeem S, Donjacour CE, Mayer G, Javidi S, Iranzo A, Santamaria J, Peraita-Adrados R, Hor H, Kutalik Z, Plazzi G, Poli F, Pizza F, Arnulf I, Lecendreux M, Bassetti C, Mathis J, Heinzer R, Jennum P, Knudsen S, Geisler P, Wierzbicka A, Feketeova E, Pfister C, Khatami R, Baumann C, Tafti M; European Narcolepsy Network. Clinical, polysomnographic and genome-wide association analyses of narcolepsy with cataplexy: a European Narcolepsy Network study. J Sleep Res. 2013 Oct;22(5):482-95. doi: 10.1111/jsr.12044. Epub 2013 Mar 18. PMID: 23496005., 2013) was sent by mail to the 11 centers. The questionnaire included the following parameters:

1. Demographic characteristics: date of birth, gender, height, weight, BMI(body mass index) at diagnosis.

2. Age at EDS (excessive daytime sleepines) onset and age at cataplexy onset. Investigators defined the age at onset of NC(narcolepsy with cataplexy) as the age at occurrence of EDS and/or cataplexy, determined during the clinical interview.

3. Frequency of cataplexy attacks at diagnosis. The frequency of cataplexy was assessed by a scale from 1 to 5, reporting rare to very frequent cataplexy attacks: 1 = one or less cataplexy attacks per year; 2 = more than one cataplexy attack per year but less than one per month; 3 = more than one attack per month but less than one per week; 4 = more than one per week but less than one per day; 5 = at least one cataplexy attack per day.

4. ESS (Epworth Sleepiness Scale) score at diagnosis.

5. Polysomnographic variables [sleep onset latency at diagnosis, apnea-hypopnea index (AHI) and periodic leg movements during sleep index (PLMSI) when available] and MSLT results (mean sleep latency, number of SOREMPs) at diagnosis. Even if the recording procedures were different among centers, most of the patients underwent nocturnal in-lab PSG followed by an MSLT as part of the diagnostic evaluation. For PSG and MSLT, sleep latency was defined as the time from lights off to the first epoch scored as sleep. A SOREMP was defined as the occurrence of an epoch of REM(rapid eye movement) sleep within 15 min after the first epoch scored as sleep. Although MSLT was performed according to standard methods (Carskadon MA, Dement WC, Mitler MM, Roth T, Westbrook PR, Keenan S. Guidelines for the multiple sleep latency test (MSLT): a standard measure of sleepiness. Sleep. 1986 Dec;9(4):519-24. doi: 10.1093/sleep/9.4.519. PMID: 3809866.), the number of scheduled naps could be variable. To standardize the results, investigators calculated the percentage of SOREMPs of the total number of naps: percentage of naps with SOREMPs.

7. Associated features, with particular attention to symptoms frequently reported with narcolepsy: sleep paralysis (SP); hypnagogic/hypnopompic hallucinations (HH); and poor nocturnal sleep.

8. Co-morbidities (sleep-related, somatic or psychiatric) and treatment when available.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 89
• Est. completion date: September 28, 2023
• Est. primary completion date: February 1, 2022
• Accepts healthy volunteers: No
• Gender: All
• Age group: 7 Years to 78 Years

Eligibility

Inclusion Criteria:

1. Signed written informed consent of the patient and/or parent/adoptive parent (in the case of a minor) to participate in the study;

2. Age: 6-80 years;

3. Gender: Men and women;

4. Established diagnosis: Narcolepsy of type I and II (according to the criteria of the International classification of sleep disorders of 2014).

Exclusion Criteria:

1. Refusal of the patient or his parent / adoptive parent (in the case of a minor) from further participation in the study;

2. The occurrence of a serious illness or a significant deterioration of a disease unrelated to narcolepsy;

3. Pregnancy started after inclusion.

Related Conditions & MeSH terms

• Narcolepsy
• Narcolepsy Type 1

Intervention

Other:
• no intervention
Narcolepsy patients in Russia gathered according to criteria.

Locations

Country	Name	City	State
Russian Federation	Regional Clinical Hospital No.3	Chelyabinsk
Russian Federation	Kazan State Medical University	Kazan
Russian Federation	Private healthcare institution 'Clinical hospital 'RZD-Medicine'	Khabarovsk
Russian Federation	Burnasyan Federal Medical Biophysical Center of Federal Medical Biological Agency	Moscow
Russian Federation	FSBI NMRCO FMBA Russia	Moscow
Russian Federation	I.M. Sechenov First Moscow State Medical University (Sechenov University)	Moscow
Russian Federation	Novosibirsk State Medical University	Novosibirsk
Russian Federation	Almazov National Medical Research Centre	Saint Petersburg
Russian Federation	Nikiforov Russian Center of Emergency and Radiation Medicine, EMERCOM of Russia	Saint Petersburg
Russian Federation	V. M. Bekhterev National Research Medical Center for Psychiatry and Neurology	Saint Petersburg
Russian Federation	Samara State Medical University	Samara

Sponsors (1)

Lead Sponsor	Collaborator
I.M. Sechenov First Moscow State Medical University

Country where clinical trial is conducted

Russian Federation, 

References & Publications (1)

Kuts A, Poluektov M, Bassetti CLA. The evolution of the narcolepsy concept in Russia: A historical view. J Hist Neurosci. 2021 Apr-Jun;30(2):113-127. doi: 10.1080/0964704X.2020.1777502. Epub 2020 Jun 30. — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	date of birth	year of birth	during 1-3 years after beginning and when patient and data are available
Primary	gender	male or female	during 1-3 years after beginning and when patient and data are available
Primary	height	meters	during 1-3 years after beginning and when patient and data are available
Primary	weight	kilograms	during 1-3 years after beginning and when patient and data are available
Primary	BMI at diagnosis	Weight and height will be combined to report BMI in kg/m^2 at the time of diagnosis	during 1-3 years after beginning and when patient and data are available
Primary	age at EDS (excessive daytime sleepioness) onset	Age when EDS first time was reported by patient	during 1-3 years after beginning and when patient and data are available
Primary	age at cataplexy onset	Age when cataplexy first time was reported by patient	during 1-3 years after beginning and when patient and data are available
Primary	frequency of cataplexy attacks at diagnosis	Reported at diagnosis. The frequency of cataplexy was assessed by a scale from 1 to 5, reporting rare to very frequent cataplexy attacks: 1 = one or less cataplexy attacks per year; 2 = more than one cataplexy attack per year but less than one per month; 3 = more than one attack per month but less than one per week; 4 = more than one per week but less than one per day; 5 = at least one cataplexy attack per day.	during 1-3 years after beginning and when patient and data are available
Primary	ESS score at diagnosis	Unit of Measure at diagnosis	during 1-3 years after beginning and when patient and data are available
Primary	Apnea-hypopnea index (AHI)	PSG at diagnosis, Unit of Measure at diagnosis	during 1-3 years after beginning and when patient and data are available
Primary	Periodic leg movements during sleep index (PLMSI)	Number of episodes and hours of sleep combined to report PLMSI in episodes per hour.	during 1-3 years after beginning and when patient and data are available
Primary	Sleep onset latency at diagnosis	Time from lights off to the first epoch scored as sleep in PSG. Measured in minutes.	during 1-3 years after beginning and when patient and data are available
Primary	Sleep latency	Time from lights off to the first epoch scored as sleep in MSLT. Measured in minutes.	during 1-3 years after beginning and when patient and data are available
Primary	Number of SOREM	It was defined as the occurrence of an epoch of REM sleep within 15 min after the first epoch scored as sleep. Then investigators calculated the percentage of SOREMPs of the total number of naps: percentage of naps with SOREMPs.	during 1-3 years after beginning and when patient and data are available
Primary	Frequently of sleep paralysis, hypnagogic/hypnopompic hallucinations and poor nocturnal sleep, co-morbidities (sleep-related, somatic or psychiatric) and treatment when available.	Percentage of occurence in group.	during 1-3 years after beginning and when patient and data are available
Secondary	summarization of collected database with European one	analyzing and comparison collected data with European one leading to final summarization of studying	1 year after receiving data from sleep centers from Russia and publishing results

External Links

•   Official website of Russian narcolepsy network