Muscle atrophy neural (amyotrophy Charcot-Marie)

Muscle atrophy neural, or amyotrophy Charcot-Marie (synonym: personalna amyotrophy Tuta, neurotic Muscle amyotrophy Hoffmann). Was first shown as a separate form C. K. room in 1884, while Charcot Marie and Here (J. M. Charcot, P. Marie, N. N. Tooth) described it only in 1886 the Disease belongs to the group of neurogenic muscle atrophy with chronic spinal-neuroticeski process. Occur as sporadic and family cases with the dominant type of inheritance. There is a high sensitivity to exogenous factors. Any weakening of the body increases for atrophic process.
The etiology and pathogenesis is unclear. Any chromosomal anomalies and imperfections of the currency is not identified. Pathomorphological data indicate primary degenerative changes in the cells of the front horns spinal cord, in front roots, peripheral nerves, and also in the rear roots, ways Gaulle and pillars Clark. The muscles involved in the process a second time, with a typical group, line distribution atrophied fibers. The disease begins in childhood and adolescence, although there are cases and a later onset of the disease. See the weakness in the legs and disorder gait. Frequent paresthesias in the distal limb and pain. Atrophic process mainly affects the muscles of the feet and lower legs. First, develop weakness and atrophy in the extensor and lateral muscles of the foot, then in other distal groups. Frequent strain into a concave feet (pes excavatus), known as the "foot disease" (Fig. 6.). On the thigh muscles atrophy do not apply (Fig. 7.). Upper limb affected only a few years later. Atrophy is also limited distal departments, capturing small muscles of the hand and leading to the development of the so-called monkey paws. Atrophy of the nature of neurogenic and are accompanied by the reaction of rebirth. Tendon areflexia is very common, capturing and unaffected segments. In the distal upper and lower extremities has been decreasing surface sensitivity. Gait acquires the character of the so-called stoppage (steppage): patients highly raise your knees so that overhanging feet not clung to the floor. The disease progresses slowly and the patients keep their ability to work.
Diagnosis of family cases not difficult. Sporadic disease can be mixed with distal myopathy (see) and polinevrita (see). In these cases make clear data electric diagnostics of electromyography and muscle biopsy. The disease responds well to treatment. Apply a blood transfusion, vitamin therapy (B1, B12, E). Vitamin b, administered intramuscularly in a 5% solution of 0.5-1 ml (depending on age) once a day; vitamin B12 100-200 ' injection a day; vitamin E 10% solution of 1 ml intramuscularly daily. At the rate of 20-30 injection of these vitamins. Assign also nivalin (0.25% solution subcutaneously, from 0.1 ml, then 0,4; 0,7; 0,8; 1 ml), neostigmine, adenosine triphosphate. Recommended electro-gymnastics wasting of muscles, massage, iontophoresis with calcium. The treatment is carried out systematically, for individual courses.

Fig. 6. Neural amyotrophy. Concave stop.
Fig. 7. Neural amyotrophy. Sharply who had lost a leg in a well-preserved the trophic thighs.