Myopathy - what is this disease, the cause of the? Description, symptoms of myopathy and prevention of disease

Atia myopia (ancient Greek. μῦς - «muscle», passion - «болезнь, suffering ») - progressive, hereditary neuromuscular disease is a chronic nature that affects muscle tissue.

The cause of the

The main reasons for the development of myopathy:

• hereditary factor, characteristic of hereditary myopathies;
• genetic defect;
• hormonal disorders in the patient's body (eg, disorders of the thyroid gland);
• systemic connective tissue disease.

Симптомы миопатии

1. Permanent muscle weakness, which does not disappear even after a rest or decrease slightly.
2. Muscle atrophy. muscles become thinner, become less active.
3. Muscle tone is greatly reduced, muscles become flaccid and flabby.
4. Rachiocampsis, It occurs due to the inability to maintain muscle carcass body in the anatomical position.
5. Psevdogipertrofii, as a result of the thinning of some extremities others look significantly increased.

Diagnostics

Major diagnostic steps:

1. Анализ анамнеза и жалоб пациента:

• determination time of the first complaint;
• How was the development in infancy and childhood;
• whether a family history of myopathy have close relatives.

2. Neurological examination:

• assessment of the severity of muscle weakness;
• the presence of the patient is characterized by atrophy of muscles (thin arms and legs, psevdogipertrofii);
• assessment of the patient's muscle tone;
• Score presence skeletal deformities;
• assessment of limb reflexes;

3. Laboratory analysis of blood.

4. Determining the level of thyroid hormones.

5. Biopsy mыshts.

6. genetic testing.

Types of disease

There are two main forms of myopathies: primary and secondary.

1. primary myopathies, In most cases, are hereditary. The primary belong:

• congenital myopathies – develop in infancy;
• early childhood myopathy – развиваются в возрасте 5-10 years;
• youth myopathy – develop in adolescence;

2. secondary myopathy, develop on the background of other pathologies and are subordinated (eg, in the case of endocrine disorders, acute poisoning, etc.).

В зависимости от степени выраженности миопатии и ее локализации в различных отделах конечностей, secrete:

• predominantly proximal, pronounced muscle weakness in the extremities, которые расположены ближе к туловищу;
• predominantly distal, muscle weakness is expressed in the limbs, which are located farther away from the body;
• hybrid – It combines both forms.

Actions of the patient

The patient requires medical care and selection of rational treatment.

Лечение миопатии

If a hereditary form of myopathy full recovery does not occur. Symptomatic therapy is, It is aimed at addressing the symptoms of the disease and improve the patient's quality of life.

The main methods of treatment of myopathies belong:

• methods of orthopedic correction. For the purposes of correction requires the use of special devices, helps patients to move independently (AFOs, wheelchairs);
• respiratory gymnastics. Использование данного метода направлено на улучшение процесса вентиляции легких и снижение риска развития воспалительных процессов в легких;
• physiotherapy – It includes a set of exercises, направленный на воспаление потребности мышц тела в двигательной активности. Application of this method slows down the development of muscle atrophy;
• rational selection of profession;
• myopathy, evolved as a result of excess thyroid hormone, подлежит лечению при помощи препаратов антагонистов тиреоидных гормонов – thyreostatics;
• myopathies, cause of which served as a systemic connective tissue disease (e.g.. scleroderma), are treated with the help of steroid hormones and cytostatics.

Complications

In a patient with myopathy may develop the following complications:

• в случае вовлечения в патологический процесс дыхательной мускулатуры может развиться дыхательная недостаточность;
• inability to walk without assistance;
• stagnant pneumonia;

the risk of death increases significantly during the development of the above-mentioned complications.

Профилактика миопатии

Perhaps the prophylaxis of hereditary myopathies in case, если ранее в семье уже имели место подобные заболевания. To this end,, performed genetic counseling couples, to assess the risk of having a child with myopathy.