What Is Myopathy?

It is a group of neuromuscular diseases that lead to gradual muscle atrophy and degeneration. Mostly these are progressive hereditary pathologies. Genetically unrelated muscle atrophy is also possible if a person, for one reason or another (usually due to injury or disease that restricts mobility), cannot have normal, daily physical activity.

Muscular dystrophy or myopathy occurs due to a violation of biochemical processes in the muscle fiber itself. The synthesis of nucleic acids changes, the breakdown of proteins is accelerated, the content of creatine phosphokinase increases, and the level of ATP decreases. Some scientists adhere to the theory of defective membranes, which is based on the loss of enzymes and amino acids by muscle fiber due to the so-called defects in cellular structures.

A part of the muscle fibers is replaced with adipose tissue with myopathy, while the muscles themselves are thinned.

There are primary myopathies, which gradually develop already in childhood and adolescence and are acquired. The clinical manifestations of myopathy can be aggravated by unfavorable provoking factors (infections, intoxication, nervous stress).

Myopathy is considered to be a genetic disease since doctors do not have more accurate information about the causes of its occurrence. It is known that with myopathy, metabolic processes in muscles are disturbed, and there is a violation of the innervation of the connection between muscle tissue and the central nervous system. Since myopathy is often hereditary, it mainly manifests itself in childhood and adolescence, mainly in males.

Myopathy isn’t just muscle weakness. It is a complex of disorders in the body, which lead to a deterioration in the activity of various systems and organs. However, the most obvious are changes in the muscles that lead to specific mobility impairments.

With myopathy, gait is disturbed; a person walks with lowered shoulders, which, as a rule, causes a curvature of the spine. Since the disease is considered progressive, the patient’s condition can deteriorate significantly without appropriate therapy. Several decades ago, myopathy was considered incurable, but modern medications can slow or even stop the progression of the disease and improve physical activity.

The role of nutrition and vitamins in myopathy

There are forms of myopathy in which the first symptoms of the disease are observed in childhood, and there are forms that appear only in adolescence or even adulthood.

Methods for treating myopathy depend specifically on its type; this should be especially taken into account when prescribing physical activity. With myopathy, physical activity is advised; however, some patients need light physiotherapy exercises, massage, and exertion without overwork, while others, on the contrary, are recommended active gymnastics and vigorous movements.

With myopathy, proper nutrition plays a huge role. It should be varied and contain a sufficient amount of vitamins, micro and macroelements. The patient with myopathy must receive the required amount of B vitamins both with food and in preparations.

• B vitamins are essential for the functioning of the muscular system; without them, the regenerative processes in the muscles and nerves slow down. Vitamins of this group are called neurotropic for a reason; they take an active part in restoring the functions of nerve fibers and maintaining their normal functioning. In particular, vitamins B1 (benfotiamine) and B6 (pyridoxine) are essential.
• In addition, with myopathy, you need to take vitamin E, calcium, and other elements necessary for musculoskeletal tissue.

Neurologists and orthopedic traumatologists treat myopathy and, if necessary, a cardiologist. To be successful in treatment, you need to make a timely diagnosis. Since the disease develops mainly in childhood and adolescence, the responsibility for timely access to a doctor and adequate treatment lies, of course, with the parents.

What to do with muscle atrophy after an injury?

Unlike myopathy, muscle atrophy caused by temporary immobility does not progress. Muscle work is restored, if not in full, then in a significant amount (depending on the age and physical condition of the person), but this requires some effort.

Moderate physical activity is prescribed, often using simulators to restore muscles after a forced restriction of movement. Massages, physiotherapy exercises, and swimming have a positive effect. The provision of the body plays an essential role with the nutrients necessary for the restoration of muscle activity.

Myopathy symptoms

Myopathy begins with the appearance of slight muscle weakness in the limbs. Fatigue occurs faster when walking or during other exercise than it was before the onset of the disease. It becomes more difficult for such people to overcome long distances, so they often have to take short rest breaks to continue their journey with renewed vigor. Further, muscle atrophy joins the growing weakness due to which deformities of the limbs appear. As a rule, atrophic changes are observed in the proximal (which are closer to the body) parts of the arms and legs, making the distal extremities appear hypertrophied. This phenomenon is called pseudohypertrophy. With the progression of muscle weakness, it becomes difficult to jump, run, and climb stairs.

The characteristic appearance of the patient with myopathy is gradually formed:

• pterygoid scapula
• drooping shoulders
• bulging forward abdomen and enhanced lumbar lordosis, due to which the so-called wasp waist is formed.

Moreover, a duck gait is observed (movement is accompanied by swinging to the sides).

Myopathies can be accompanied by damage to facial muscles. It is possible to suspect the involvement of facial muscles in the pathological process when a person cannot stretch their lips with a tube, puff out their cheeks, frown or smile. The development of dysarthria accompanies the defeat of the circular muscle of the mouth due to the difficulty in pronouncing vowel sounds. Dysarthria itself denotes a speech disorder expressed in the difficult pronunciation of some words, individual sounds, syllables, or distorted pronunciation.

The defeat of the respiratory muscles is accompanied by impaired ventilation of the lungs, leading to congestive pneumonia. This form of pneumonia is one of the most severe along the course, is difficult to treat, and quickly leads to respiratory failure.

There is also evidence of the possibility of damage to the heart muscle. In this case, cardiomyopathy develops, which leads to the development of heart failure.