ABSTRACT INTRODUCTION. Amyotrophic lateral sclerosis is a disease of the central nervous system, characterized by a steadily progressive course with selective damage to the upper and lower motor neurons. AIM. Conduct an analysis of literary sources of domestic and foreign authors describing the modern understanding of methods of diagnosis and treatment of amyotrophic lateral sclerosis. Present a clinical case of a patient with amyotrophic lateral sclerosis. MATERIAL AND METHODS. An analysis of the literature was carried out for the period from 2013–2023. Sources from the Russian scientific electronic library eLIBRARY, RSCI databases, and PubMed were used. A clinical case is presented. RESULTS AND DISCUSSION. The article presents clinical and epidemiological features, diagnostic methods, modern achievements and promising directions in the treatment of the disease. CASE PRESENTATION. Patient N., 60 years old, was admitted to the neurological department of a multidisciplinary hospital with complaints of weakness in the upper extremities, more in the distal parts, impaired fine motor skills, impaired articulation, general weakness, and fatigue. Considers himself sick for a year, when complaints of blurred speech and weakness in the hands first appeared. Needle electromyography — signs of morphofunctional reorganization of motor units associated with damage to motor neurons. Based on clinical data and the results of additional examination methods, a clinical diagnosis was established — motor neuron disease, amyotrophic lateral sclerosis, bulbospinal form, bulbar disorder syndrome, upper paraparesis with impaired selfcare. The features of the presented clinical example are the rapid rate of progression of the disease, as well as the importance of risk factors in the development of the disease. As is known from the family history, the patient served in the missile forces for a long time. The problem of environmental safety still remains relevant. Falling separated parts of launch vehicles have a detrimental effect not only on the environment, but also on the human body. CONCLUSION. Identification of risk factors is an important task necessary for the early detection of electromyography, as well as the possibility of taking preventive measures that can lead to a significant reduction in morbidity and mortality. KEYWORDS: amyotrophic lateral sclerosis, motor neuron disease, diagnosis, therapy, SOD1 gene