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Background. Dystrophic myotonia type 1 (DM1) is the most common muscular dystrophy in patients of any age. Myotonia “delayed relaxation of muscle” is the leading symptom in DM1 and can occur at any time after onset disease. Myotonia symptoms and electrical myotonia registration are delayed after onset in patients with congenital and infantile forms of DM1. This makes it difficult to diagnose and prevent fatal complications in these patients in a timely manner. Objective: presentation of the clinical data and results of needle electromyography in patients with DM1 onset in the first decade of the life; determination of the first symptoms of the disease, to estimate the age of myotonia and electrical myotonia manifestation for the optimization of the timely diagnostics of the disease.Materials and methods. 13 patients with DM1 aged from 2 months to 34 years were described. 10 patients underwent needle electromyography with analysis of spontaneous activity and needle EMG pattern. The diagnosis was made on the basis of clinical and paraclinical manifestations of the disease and identification of an increase in CTG repeats (>50) in the DMPK gene.Results. The onset with extramuscular signs of respiratory and/or feeding disturbances, dysarthria, school learning disorders, autism spectrum disturbance and “floppy infant syndrome” was noted as the first symptoms of the disease. Clinical myotonia symptoms and electrical manifestations of myotonia were absent in all patients for a long time after the disease onset. DM1 was confirmed in all mothers, however in 5 cases the onset of the disease was later than the first symptoms in patients with congenital and childhood onset forms of DM1.Conclusion. The first symptoms of the congenital and infantile forms of DM1 are not specific and occur in a wide range of diseases. Such discriminating signs of DM1 as clinical myotonia, distal muscle atrophy and electrical myotonia appear much later than the onset disease. In the group of patients before and after the formation of phrasal speech, the presented combinations of symptoms allow diagnostics of the congenital and infantile forms of DM1 at the onset of the disease. In its turn, it allows genetic counseling in burdened families and timely prevention of fatal complications.

The article deals with modern features of the Arctic economy. It is shown that in those spheres and directions of economic activity, which are associated with the development of natural resources (primarily mineral resources) and focused on obtaining returns on investment, there is a strengthening of the role not only of new knowledge and new technologies, but also significantly increase the role and importance of forms of cooperation of the parties involved in the implementation of projects. This approach allows, on the one hand, to solve the problem of attracting investment in high-risk and, at the same time, high-yield projects, but it also will not allow to fully realize the opportunities associated with the development and use of domestic research and production potential in the implementation of projects in the framework of such “hybrid forms”. One of the direct and immediate consequences of this approach in the practice of field development in the Arctic zone of the Russian Federation (AZRF) is a significant increase in the knowledge-intensive service sector, which meets the needs for equipment and labor by borrowing foreign advanced technologies and the widespread use of interregional watch. These processes lead, in particular, to the fragmentation of the economic space of the country (reducing the degree of connectivity of the economies of different regions), as well as to the stagnation and extinction of urbanized settlements in the Russian Federation.