Myopathy ( Medical Encyclopedia )

Myopathy (progressive muscular dystrophy) - a group of hereditary diseases, which are based on the degeneration of muscle tissue and its replacement by fatty and connective.The group of myopathies include Duchenne muscular dystrophy, Becker muscular dystrophy, muscular dystrophy Erb - Roth, of shoulder-shape front Landuzi - Dejerine.

For all myopathies characterized by a gradual progressive deterioration of the patient's condition.

myopathy in most cases men suffer.The most common Duchenne myopathy - 14 cases per 100 000 births.The etiology of the disease is unknown.

disease appears in the first 3 years of life, first drew the lag in motor development.Localization of damage depends on the form of the disease.In Duchenne myopathy due to atrophy of the muscles of the pelvic and shoulder girdle and then changes the child's gait.If myopathy Erb - Roth first affects the muscles of the lower extremities, to the front of shoulder-shaped Landuzi - Dejerine typically defeat muscles of the face and shoulders.T

here are difficulties in climbing the ladder, it produced a kind of duck gait: the patient goes, sticking out his chest and stomach, swaying from side to side.Getting up from a low chair or on the floor, the patient resorted to using their hands, resting them on her hips.For lesions of the lower extremities characteristic psevdogipertrofiya calf muscles due to the accumulation of adipose tissue.With the defeat of the shoulder girdle the patient can not lift the arm above the horizontal level.If it affects the muscles of the face appears gipomimiya.The weakness of the circular muscles of the eye leads to difficulty closing the eyelids.

Atrophy affects symmetrical groups of muscles, reduced strength and muscle tone, fade tendon and periosteal reflexes.The sensitivity, reflexes of the skin and mucous membranes are not affected.May include the moderate pain in atrophied muscles.

conduct electromyography (EMG) to confirm the diagnosis.In the blood revealed an increase in the content of creatine, creatine phosphokinase and aldolase.Recovery is not possible, muscle weakness progresses, leading to total immobility, with the defeat of the respiratory muscle death occurs.

no specific treatment, therapy aims to improve nerve conduction and muscle trophism.To improve neuromuscular conduction used methionine, glutamic acid, ATP, vitamin B, E, Neostigmine, pahikarpin.To improve trophism muscle appoint kokarboksilazu, Riboxinum, Cerebrolysin, nicotinic acid, pentoxifylline.
Apply galvanic collar on Shcherbak on the neck, moderate toning massage, physiotherapy exercises.

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