EPIDEMIOLOGICAL FEATURES OF MYASTHENIA GRAVIS

Aim. To assess the main epidemiological indicators of myasthenia gravis in various countries of the world, as well as in the territory of the Russian Federation.Materials and methods. Materials of publications on the study of epidemiological and population characteristics of myasthenia gravis of native and foreign authors are studied.Results. The indicator of the prevalence of myasthenia in the world varies from 0.5 to 20.0 per 100,000. The average primary morbidity of myasthenia gravis in the world is 0.53 per 100,000 population. The indicator varies from 0.17 to 2.13 per 100 thousand of the population.Conclusion. A fairly large range of epidemiological characteristics of myasthenia gravis in the world is revealed. The high prevalence of this pathology in the territory of the Russian Federation is established, which causes further interest in this type of research

Эпидемиологические и популяционные аспекты миастении в Краснодарском крае

Objective: to study epidemiological and population indicators of myasthenia in the Krasnodar Territory. Materials and methods. 344 patients with a diagnosis of myasthenia living in the Krasnodar Territory underwent a retrospective analysis. Results. On January 1, 2017 the prevalence of myasthenia was 8.1 cases per 100,000 population, morbidity in 2011–2016 ranged from 0.27–0.71 cases per 100,000 population, and the average was 0.52 cases per 100,000. The average age of manifestation of the disease of registered patients was 46.2 ± 0.97 years. The minimum age of debut of the disease was 11 years, the maximum – 80 years. The ratio of men and women among patients with myasthenia was 1:2.5. The peak of the onset of the disease in women falls on the period of 20–40 years. A bimodal pattern of incidence in women was revealed in the period from 20 to 40 years and from 50 to 70 years. After 60 years 35 % of men fell ill. The generalized form of myasthenia was observed in 82.8 % of patients, eye cases in 16.3 %, oculopharyngeal form in 0.9 %. Of the observed patients, rural residents accounted for 58.7 %, urban residents for 41.3 %. Conclusion. The main epidemiological indicators of myasthenia obtained in the course of the study in the Krasnodar Territory are on average comparable with the Russian ones and meet the global trends. Epidemiological monitoring of myasthenia is more effective on the basis of specialized myasthenic centers.Цель исследования – изучение эпидемиологических и популяционных показателей миастении в Краснодарском крае.Материалы и методы. Ретроспективному анализу были подвергнуты данные 344 пациентов с диагнозом миастении, проживающих в Краснодарском крае.Результаты. По данным на 1 января 2017 г. распространенность миастении составила 8,1 случая на 100 тыс. населения, заболеваемость в 2011–2016 гг. колебалась в пределах 0,27–0,71 случая на 100 тыс. населения и в среднем составила 0,52 случая на 100 тыс. населения. Средний возраст манифестации заболевания зарегистрированных больных – 46,2 ± 0,97 года. Минимальный возраст дебюта болезни – 11 лет, максимальный – 80 лет. Соотношение мужчин и женщин среди пациентов с миастенией – 1:2,5. Пик начала заболевания у женщин приходится на период 20–40 лет, был выявлен бимодальный паттерн заболеваемости у женщин в периодах с 20 до 40 лет и с 50 до 70 лет. После 60 лет заболели 35 % мужчин. Генерализованная форма миастении выявлена у 82,8 % пациентов, глазная форма – у 16,3 %, краниофарингеальная форма – у 0,9 %. Из наблюдающихся пациентов жители сельской местности составили 58,7 %, городские жители – 41,3 %.Заключение. Полученные в ходе исследования основные эпидемиологические показатели миастении в Краснодарском крае в среднем сопоставимы с российскими и отвечают общемировым тенденциям. Эпидемиологический мониторинг миастении эффективнее проводить на базе специализированных миастенических центров

Epidemiologic and populational aspects of myasthenia gravis in the Krasnodar Region

Objective: to study epidemiological and population indicators of myasthenia in the Krasnodar Territory. Materials and methods. 344 patients with a diagnosis of myasthenia living in the Krasnodar Territory underwent a retrospective analysis. Results. On January 1, 2017 the prevalence of myasthenia was 8.1 cases per 100,000 population, morbidity in 2011–2016 ranged from 0.27–0.71 cases per 100,000 population, and the average was 0.52 cases per 100,000. The average age of manifestation of the disease of registered patients was 46.2 ± 0.97 years. The minimum age of debut of the disease was 11 years, the maximum – 80 years. The ratio of men and women among patients with myasthenia was 1:2.5. The peak of the onset of the disease in women falls on the period of 20–40 years. A bimodal pattern of incidence in women was revealed in the period from 20 to 40 years and from 50 to 70 years. After 60 years 35 % of men fell ill. The generalized form of myasthenia was observed in 82.8 % of patients, eye cases in 16.3 %, oculopharyngeal form in 0.9 %. Of the observed patients, rural residents accounted for 58.7 %, urban residents for 41.3 %. Conclusion. The main epidemiological indicators of myasthenia obtained in the course of the study in the Krasnodar Territory are on average comparable with the Russian ones and meet the global trends. Epidemiological monitoring of myasthenia is more effective on the basis of specialized myasthenic centers

DIAGNOSTIC POSSIBILITIES OF RESEARCHING THE STATE OF NEUROMUSCULAR TRANSMISSION IN PATIENTS WITH MYASTHENIA AND MYASTHENIC SINDROMES

Aim. To study and analyse diagnostic meaningfulness of the different electromyographic methods applied for the exposure of violations of neuromuscular transmission transmission.Materials and methods. There are many methods of the researching of the condition of neuro muscular transfer, each of them carries the certain diagnostic importance allowing to define character of a lesion of a neuro muscular synapse, to tap mechanisms of its development not only in clinically affected muscles, but also intact ones, and also at an early stage of a disease, at local forms of a myasthenia. The research of the condition of neuromuscular transmission was carried out with the use of the following electromyographic techniques: stimulation electroneuromyography (ENMG), the decrement test, the jitter-phenomenon, research of density of a single muscle fiber.Results. The result of the analysis of electromiographic diagnostic opportunities of researching the condition of neuro muscle fiber development is the developing of the clinic-instrumental algorithm of inspection of patients with a myasthenia.Conclusion. The different neurophysiological methods of study of the state of neuromuscular transmission carry certain diagnostic meaningfulness. Advantages of separate electromyographic methods are shown: research of initial amplitude of M-answer, decrement-test, джиттер-phenomenon. The indexes of ENMG-tests are analysed in detail-tests, and also their change at violation of neuromuscular transmission for patients with myasthenia gravis and myasthenic syndromes