Myopathy

Myopathy (progressive muscular dystrophy) is a group of hereditary diseases based on the degeneration of muscle tissue and its replacement by fatty and connective tissue. The group of myopathies includes Duchenne muscular dystrophy, Becker muscular dystrophy, Erb-Roth muscular dystrophy, Landouzi-Dejerine muscular dystrophy. All myopathies are characterized by a gradual progressive deterioration of the patient's condition.

Myopathy affects men in the vast majority of cases. Duchenne myopathy is the most common - 14 cases per 100,000 births. The etiology of the disease is unknown.

Symptoms of myopathy

Myopathy manifests itself in the first 3 years of life, first affecting the lag in motor development. The location of the lesion depends on the form of the disease. With Duchenne myopathy, due to atrophy of the muscles of the pelvic and then the shoulder girdle, the child’s gait changes. With Erb-Roth myopathies, the muscles of the lower extremities are the first to be affected; for the Landouzi-Dejerine form, the muscles of the face and shoulders are typically affected.

Difficulties arise when climbing stairs, and a peculiar duck gait is developed: the patient walks, sticking out his chest and stomach, waddling from side to side. When getting up from a low chair or from the floor, the patient resorts to using his hands, resting them on his hips.

Damage to the lower extremities is characterized by pseudohypertrophy of the calf muscles due to the accumulation of adipose tissue. If the shoulder girdle is affected, the patient cannot raise his arm above the horizontal level. When the facial muscles are damaged, hypomimia occurs. Weakness of the orbicularis oculi muscle leads to difficulty closing the eyelids.

Atrophy affects symmetrical muscle groups, muscle strength and tone decrease, tendon and periosteal reflexes fade. Sensitivity and reflexes of the skin and mucous membranes do not change. There may be moderate pain in atrophied muscles.

Diagnosis of myopathy

Electromyography (EMG) is performed to confirm the diagnosis. An increase in the content of creatine, creatine phosphokinase and aldolase is detected in the blood. Recovery is impossible, muscle weakness progresses, leading to complete immobility, and death occurs when the respiratory muscles are damaged.

Treatment of myopathy

1. There is no specific treatment for myopathy; therapy is aimed at improving nerve conduction and muscle trophism.

2. To improve neuromuscular conduction, methionine, glutamic acid, ATP, vitamins B, E, proserine, pachycarpine are used.

3. To improve muscle trophism, cocarboxylase, riboxin, cerebrolysin, nicotinic acid, and pentoxifylline are prescribed.

4. A galvanic collar according to Shcherbak is applied to the neck area, moderate tonic massage, and physical therapy.

Myopathy is a group of inherited diseases and conditions associated with impaired neuromuscular transmission and progressive muscle weakness. This leads to decreased muscle strength and endurance, as well as poor coordination and stability of the body. Myopathy can manifest itself in different forms: dystrophic myopathy, spinal muscular atrophy, muscular dystrophy and others. There are currently about 350 different types of myopathies, most of which are inherited diseases with high morbidity and mortality rates.

There are three main types of myopathic diseases: muscle dysfunction, diseases of the neuromuscular synapses, and visceral forms, when the disease affects internal organs. Some types of myopathies may be associated with impaired brain function. Myopathy develops gradually and has an increasing form of symptoms. Most often, it impairs the motor function of muscles, which manifests itself in the form of fatigue, weakness

Myopathy is a complex and very rare disease that affects the muscle tissue of the body. With this disease, the muscles lose their strength, elasticity and become hard, causing problems in the functioning of certain parts of the body. Let's look at the main symptoms, causes and treatment of myopathies.

Symptoms of myopathy Symptoms of myopathy can vary and depend on the type of disease, but the main ones include weakness, loss of muscle strength and endurance, poor coordination of movements, decreased height, facial deformation, curvature of the spine, decreased appetite, difficulty swallowing, weakness, breathing problems and vision. Sometimes the only symptom is asymmetric muscle weakness or cramps in the limbs.

Causes of myopathies The causes of myopathies are unknown. This occurs after certain genes are passed from parents to child. The difficulty is that the disease can be detected even if the parents have no manifestations of myopathies and are absent