Clinical and epidemiological features of amyotrophic lateral sclerosis in the Republic of Bashkortostan (Russia)

Background. Amyotrophic lateral sclerosis (ALS) is a degenerative disease of the nervous system, the prevalence of which varies considerably in different regions of the world. Epidemiologic studies of ALS in the Russian Federation are scarce.Aim. To analyze the epidemiological features of ALS in the Republic of Bashkortostan (RB) according to the data of the unified state information system in the field of health care and clinical characteristics according to the hospital register for the period 2013–2024.Materials and methods. A retrospective analysis of electronic medical records of all patients living in the RB who were diagnosed with G12.2 according to the 10th revision of the International Classification of Diseases within analyzed period was performed. The study of clinical characteristics of patients with ALS was done using data from the hospital registry, which was maintained since 2013.Results. The primary incidence of ALS in RB increased from 0.7 to 1.1–1.3 cases per 100,000 population over the period 2013–2024. The overall incidence of ALS for this period increased more than 3-fold, from 0.8 to 2.7 cases per 100 thousand population. The first manifestations of the disease were more often noted at the age of 53–66 years, among patients prevailed rural residents (59.5 %), people of physical labor (48.3 %). Men were more often suffered from ALS in the group with onset before the age of 45 years (p = 0.028), women more often had bulbar form of the disease (p = 0.046). The diagnosis was established on average one year after the development of the first symptoms of the disease.Conclusion. There was an increase in the incidence of ALS over 2012–2024, which probably reflects improved detection of the disease in the RB. Future studies should clarify the role of environmental and biological factors in the development of ALS.