F.A. KHABIROV^1,2, T.I. KHAIBULLIN^1,2, E.V. GRANATOV², E.F. RAHMATULLINA¹, V.A. SHIKHOVA², M.A. YAKUPOV², A.R. KHAIBULLINA¹

 ¹Kazan State Medical Academy, Kazan

²Republican Clinical Neurological Centre, Kazan

 Contact details:

Khabirov F.A. — MD, Professor, Head of the Department of Neurology, Head of the Republican Clinical Diagnostic Centre on Demyelinating Diseases

Address: 13 Vatutin St., Kazan, Russian Federation, 420012, tel.: +7 (843) 278-23-17, e-mail: rkbvl@tatar.ru

The purpose — to study the clinical and epidemiological characteristics of neuromyelitis optica spectrum disorders (NMOSD) in the Republic of Tatarstan (RT).

Material and methods. A retrospective analysis of the patients with NMOSD (according to 2015 diagnostic criteria), observed in our Centre in 2017–2021 (n = 35), was carried out. The parameters analyzed were demographic features, age of the disease onset, first clinical manifestations and course of disease, anti-AQP4 serum status, disease modifying therapy, and EDSS on baseline and follow up visits.

Results and conclusions. We found that the prevalence of NMOSD in RT in 2021 was 0.9/100,000, the averaged (for 2017–2020) incidence was 0.83/1,000,000 per year. Among 35 patients (average age 43,4 (12,7) years old), 24 (69%) were female and 11 (31%) were male. The most common clinical manifestations at onset included optical neuritis and/or myelitis (94%). Most cases (83%) had relapsing course. Positive anti-AQP4 serum status was revealed in 49% of patients. The probably monophasic disease (n = 6) was observed predominantly in anti-AQP4 negative patients (5 vs. 1). DMT was prescribed to all patients with relapsing NMOSD, the most commonly used agents included corticosteroids, azathioprine and mitoxantron (49, 20, and 11% accordingly). The rate of remission rate for all DMT varied from 35 to 57%. Ultimately all patients with relapsing NMOSD showed EDSS increase over the follow-up period, which was more prominent among anti-AQP4 positive patients (from 3,8 to 5,4 over 4,4 (3,3) years, compared to 2,9 to 3,8 over 4,0 (3,0) years accordingly).

Key words: neuromyelitis optica, Devic’s disease, neuromyelitis optica spectrum disorders, epidemiology, incidence, prevalence, the Republic of Tatarstan.

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