represents a group of chronic muscle diseases. It is characterized by progressive muscle weakness and degeneration of muscles. This reduces the diameter (thickness) of muscle fibers. The process involved mainly the skeletal muscles, rarely the internal organs. Sore muscles gradually lose their ability to shrink, disintegrate, giving way to the fatty and connective tissues.
The development of this disease a person becomes disabled. The spine is deformed, lost motor ability. The disease affects other systems of the body developing heart and respiratory failure, occur violations of intellect, memory and lost the ability to learn.
There are several forms of the disease.
Muscular dystrophy Duchenne most often appears in childhood. It hurt mostly boys. The first symptoms at the age of 2-5 years. Initially, the weakness encompasses a group of leg muscles and pelvic girdle. Then the disease affects the whole body.
Degeneration of muscle tissue leads to an increase in the volume of calf muscles, fat and connective tissue grow rapidly.
The disease progresses fairly rapidly in the 12 years patients not able to move, and by 20 years most of them die.
Myodystrophy Becker rare, and suffer from it mostly undersized men. Unlike the previous forms, is not such a rapid development. Long enough a person is in satisfactory condition. If diagnosed progressive muscular dystrophy, also known as this form of the disease, the disability happens only on the background related injuries and illnesses.
ERB’s muscular dystrophy develops during adolescence 10-20 years. In this case the muscles affected in a downward direction first, the destructive process affecting muscles of the shoulder girdle, pelvis, and lower extremities. The patient can be identified by characteristic gait and posture; he rolls over, belly pushed forward, the sternum is pushed back. Progresses this form of the disease is slow.
Myotonic form of muscular dystrophy often affects adult men and women aged 20-40 years, but in some cases it occurs in infants. This condition is characterized by delayed muscle relaxation after contraction (myotonia), weakening of facial muscles. The peculiarity of this form of pathology lies in the fact that in addition to skeletal and affects the the internal muscles, including the heart. The destructive process can affect other muscle groups, including arms and legs. The disease slowly.
Shoulder-blade-facial muscular dystrophy Landouzy-Dejerine affects people of different ages from 6 to 52 years. Most at risk adolescents 10-15 years. First, there is a degeneration of the facial muscles, then of the trunk and extremities. The first symptoms of incomplete closure of the eyelids, lips, which worsens the diction. The patient for a long time retains the ability to work. However, after 15-25 years of atrophy in the muscles of the pelvic girdle. It impairs motor function.
While not thoroughly studied all the mechanisms of the development of dystrophy of muscle tissue in humans. The main reason is called mutation of the gene responsible for the ability of muscle cells to synthesize special proteins.
Today discovered the gene that causes Duchenne pathology. It is located on the X chromosome. Mother with a defective gene passes it on to his son, who is ill, reaching 2-5 years of age.
Women cause of disease is and hormonal changes caused by pregnancy, menopause or associated with the onset of menses.
First symptoms of muscular dystrophy, some forms of the disease can appear at a very early age of two years. The child often falls quickly tired even with slight physical exertion, he is clumsy in his movements, which is particularly noticeable on the background of peers.
Muscular dystrophy in children to years is evident in the sharp degradation of development in comparison with initially normal growth and activity. Kid loses previously obtained skills loses the ability to hold the head, stops to roll over. As a rule, such children do not survive to 3 years.
About the development of this disease in adults, says loss of muscle tone due to weakness, impaired gait, atrophy of skeletal muscle. The patient does not feel muscle pain with stable sensitivity. He complains of constant fatigue. Is the growth and increase calf muscles.
The doctor will gather family history, trying to find out whether any of the relatives of such pathology, explains the nature of the dystrophy. These data will help to make further prognosis for the patient.
Is electromyography, during which examines the electrical activity of the muscle tissue, which is detected by the primary muscular dystrophy, a sample taken of the tissue for the study of degenerative tissue changes and fat deposits. With the availability of modern equipment is also carried out molecular biological and immunological analysis to determine the probability of the disease development of children.
Myodystrophy an incurable disease. All of the event therapy is aimed at slowing the destructive processes in muscle. To do this, the patient is prescribed vitamins, corticosteroids, adenosine triphosphate.
To slow down or even stop the process of degeneration help preparations based on fetal stem cells.
In the treatment of muscular dystrophy effective physiotherapy. It is aimed at minimizing the development of contractures and deformities, which develop complex physical movements.
Its effectiveness has proven therapeutic massage. If there is a risk of defeat of respiratory muscles, is breathing exercises.
Before planning pregnancy, women need to be screened for the presence of pathological genes, especially if someone from the family had such a disease. To identify it even in the fetus. To this end, we study the amniotic fluid, fetal cells or blood.