Myopathy

Myopathy (synonym progressive muscular dystrophy) - a disease characterized by primary degeneration of the skeletal muscles. For different gradual, steady progression of the process. In the basis of the disease is hereditary factor, the manifestation of which may contribute to various hazards (traumas, infections, intoxications). In more rare cases of myopathy may be secondary, symptomatic arising in connection with the primary violation of the functions of the endocrine system, the upper autonomic centers, etc. When myo have been violations of the functions of a number of enzymesthat are involved in muscle metabolism. The increase in the level of serum enzymes goes along with the decrease of their content in the muscle tissue.
Myopathy begins in childhood or adolescence, for several years, develop atrophy of muscles, limb deformations, significant muscle weakness. The first affects the muscles of the proximal limbs (shoulders, forearms, thighs). Fatigue with walking difficulties climbing stairs, getting up from the floor. The process develops symmetrically, selectively affecting the muscles of the limbs and trunk. This leads to peculiar position of the patient. Shoulders down and anteriorly, kryloviana the distance between the shoulder blades, increasing lumbar lordosis, belly comes forward. Patients go swaying from side to side ("duck walk"). Getting up from the floor, from the chair, patients are special "myopathy" techniques. On the background of the atrophied muscles of the thighs clearly are often expressed condensed and increased calf muscles ("pseudohypertrophy"). A similar phenomenon can be observed in other muscle groups. Tendon reflexes are first decreased and then disappear.
Myopathy includes several independent forms, differing in the clinical picture and the nature of inheritance. The most severe and early is pseudohypertrophic form.
Begins at the age of 3-5 years and progresses rapidly coming immobility and secondary deformation of the skeletal system. The lethal outcome. The disease is inherited coalesced heritable genes. with the X-chromosome (see Hereditary diseases). Sick just boys, transmits the disease mother.
Youth form of myopathy begins at the age of 10-20 years. Unlike pseudohypertrophic form, it runs more slowly. Patients can go a long time. This form is inherited in an autosomal recessive type.


The shoulder-blade-facial shape myo: 1 - the patient at rest; 2 and 3 - when you try to take arms to the horizontal.

The shoulder-blade-facial shape have been relatively benign compared to the first two. Starts at different ages and different primary defeat of the shoulder muscles (Fig) and face. Inheritance of this form is dominant. Besides these, there are other, more rare forms.
Treatment of myopathy is complex. Applies stimulating, symptomatic and rehabilitation therapy aimed at the prolongation of life. Assign the disodium salt of adenosine triphosphate 1% solution of 1 ml intramuscularly daily, in the course of 30 injections vitamin E 1 ml intramuscularly at the rate of 20-30 injection; thiamine chloride (vitamin B1) 5% solution of 1 ml intramuscularly, 20-30 injection; cyanocobalamin (vitamin B12) 100-200 mcg injection a day, only 20-30 injection; methionine 0.5-1 g orally 3 times a day for 30 days; glutamic acid 0.5 g orally 3 times a day for 30 days; retabolil 50 mg intramuscularly 1 time a week, only 5 injections. Transfusion single-group of blood by 100-150 ml of 1 times a week for 4-6 weeks. Iontophoresis with chloride calcium. Easy therapeutic exercises. Repeat treatments 2-3 times a year. Cm. also, muscular Atrophy.

Myopathy (myopathia; from the Greek. mys, myos - muscle and pathos - sickness; synonym; progressive muscular dystrophy, protomoteca muscle atrophy, muscle the " dryness"; the last two terms are now only of historical interest) is one of the forms of a large group of muscle atrophy, is characterized by a primary lesion of the muscular system while maintaining the structure and function of the peripheral motor neuron.
The etiology and pathogenesis. Myopathy is a hereditary disease caused by a certain type of inheritance with different clinical forms (characteristic of these types of inheritance provided in the section below Genetics separate forms). Essential in the etiology of the disease is also acute and chronic infections, injuries, malnutrition and other factors of the external environment, as well as physical stress. These factors lead to the manifestation of available genetic disposition and are a direct impetus to the development of the progressing process (S. N. Davidenkov).
Genetic and biochemical studies recently given the opportunity to more clearly identify the nature of the available predilection. When myo found hereditary metabolic disorders that cause changes in functions specific to myo enzymes and lead to defects of the content in the serum of patients aldolase, transaminases, CPK and other
Along with genetically determined abnormalities of metabolism in the pathogenesis of myopathy identified and violations of the sympathetic innervation of muscles. So, it is known that at the monastery mainly affects the proximal parts of the limbs, where relatively richer vegetative innervation. Affected vegetative and Central apparatus of diencephalic level, which is clearly seen in the violation of a number of Central caused autonomic reflexes.
Pathological anatomy. When myo revealed no changes on the part of the animal's nervous system. Dystrophic process, directly affecting the muscles leads to atrophy of individual muscle fibers. For histological pattern is characterized by disordered, dispersed distribution thinned, atrophic fibers among fibers relatively intact or exaggerated. Muscle tissue is replaced with fatty and connective tissue. By electron microscopy detected violations of the myofibrils and proteins.