Muscular dystrophy, progressive

Progressive muscular dystrophy - (Dystrophia muscularis progressiva, DMP) is a hereditary rapidly progressive disease of skeletal muscles, manifested in the form of stereotypical degenerative changes, developing predominantly in the muscles of the shoulder and pelvic girdle and the proximal parts of the legs with predominant damage to the anterior muscles. Cause of the disease: With IVD, a characteristic phenomenon of heterozygous dominance is observed; it was suggested that there are two alleles of the gene in question, causing different variants of the disease. Boys aged 4 to 23 years are affected, the sex ratio is 7: 1. As for progressive muscular dystrophy (Duchenne-Becker congenital myopathy), this is a congenital hereditary degenerative progressive dysplasia of muscle tissue in the form of progressive atrophy and replacement of normal muscle fibers by progressive bundles of connective tissue fabrics. There are myotonic and hypotonic forms of muscular dystrophy.

Symptoms of diffuse Duchenne myopathy include weakness

Progressive muscular dystrophy (ASMD) is a rare inherited disease characterized by slowly progressive destruction of muscle tissue and progressive muscle atrophy.

ASD manifests itself in childhood. Over several years, degeneration of skeletal muscle fibers and neural tissue develops. The affected muscles lose efficiency, flexibility and volumetric capacity, in addition, multiple dysfunctions of the heart, lungs, and nervous system develop. Some of the complications that may occur with ASD include respiratory failure, liver dysfunction, and cardiac dysfunction. Before an ASD is diagnosed, patients often exhibit signs of constitutional muscle hypoplasia, imbalances in body parameters, and mental regressions.

Overexpression of proteins called aldose reductase proteins, or PDRs, is a key factor leading to the development of dystrophy. PDR1 is the major allele, present in 90-95% of disease cases and 85-90% of disease severity. Although about 20 "sensitive genes" are involved in the development of ASD. A recent mutation of the SMN2 gene appeared in the classical form of the disease and led to a decrease in the manifestation and prevalence of the SMA syndrome, so the change in the course of the disease led to a significant change in the severity of the disease.

The presence of weak and multiple neurons in the brains of patients with ASD may lead to their further development under the influence of a lack of key signaling proteins such as NGF. Therefore, the development of such approaches to the treatment of skeletal muscle dystrophies is an urgent task for researchers around the world.

Muscle dystrophy is a genetic and hereditary disease characterized by a disorder in the structure of muscles. There are several types of muscle dystrophy, but the most dangerous is progressive muscular dystrophy. This is a serious disease that requires timely diagnosis and treatment.

Dystrophy progresses slowly and does not manifest itself in any way in the initial stages. Often in the first stages it can be identified only by research results. The rate of progression can depend on many factors, including age, genetics, lifestyle, level of physical activity and the presence of other diseases. With each stage, muscles lose their function, and progressive muscular dystrosias can become