Progressive muscular dystrophy

Progressive muscular dystrophy is an essential progressive degeneration of the muscular tissue that occurs outside of any lesion of the nervous system and leads to severe atrophies and weakness of certain muscle groups.

Etiology, pathogenesis. The reason is unknown. The disease is often family or hereditary. There is a progressive death of muscle fibers with replacement of their connective tissue.

Symptoms, course. The disease usually manifests itself in childhood. There is a weakness of the proximal parts of the limbs, muscles of the shoulder and (or) pelvic girdle. The gait becomes a duck, there are hyperlordosis, scoliosis, "pterygoid" scapula. Progression of muscle atrophy, and in some muscle groups, weakness is accompanied by pseudohypertrophy; There are contractures. In the affected muscle groups, deep reflexes gradually fade away. Electromyography reveals changes that are characteristic of primary-muscular (non-neurogenic) lesions. As a rule, the level of certain enzymes in the blood increases (creatine phosphokinase, etc.).

Differential diagnosis with neurogenic atrophies (syringomyelia, amyotrophic lateral sclerosis, polyneuropathy) is based on the purely elective nature of atrophy in progressive muscular dystrophy, their characteristic distribution, typical changes in posture, the presence of hyperfermentemia, EMG data and, finally, the results of muscle biopsy.

Treatment is symptomatic.

The outlook is unfavorable. There are no spontaneous and significant therapeutic remissions. Steadily increasing immobility creates conditions for secondary infections, most often respiratory (pneumonia). The disease lasts for years. There are malignant and relatively benign variants of muscular dystrophy; In the latter case, the disease does not affect life expectancy.