Neuromuscular diseases. Myositis. Causes, symptoms and signs, diagnosis and treatment of pathology

Neuromuscular diseases are a conditionally distinguished group of diseases that are characterized by dysfunction of the muscles, primarily by their weakness. Neuromuscular diseases include muscle disease, peripheral nerve disease, neuromuscular junction disease, and motor neuron disease. The same symptom of muscle weakness can be a manifestation of diseases that are very different in mechanism. This determines a completely different prognosis and methods of treatment.

Muscle diseases

Acquired myopathies:

inflammatory myopathies: (polymyositis, dermatomyositis, myositis with inclusions, sarcoid myopathy;

drug and toxic myopathies (corticosteroid myopathy, myopathy when using drugs to lower cholesterol, alcoholic myopathy, myopathy in critical conditions).

Secondary metabolic and endocrine myopathies:

hypokalemia myopathy;

hypophosphatemic myopathy;

myopathy in chronic renal failure;

myopathy in diabetes;

myopathy in hypothyroidism;

myopathy in hyperthyroidism;

myopathy in hyperparathyroidism;

Cushing's disease.

Primary metabolic myopathies:

myoglobinuria;

channelopathy;

hereditary myopathies;

muscular dystrophies.

Diseases of the peripheral nerves

Diseases of the neuromuscular junction

myasthenia gravis

Lambert-Eaton syndrome

Botulism

tick paralysis

Motor neuron diseases

amyotrophic lateral sclerosis

Diseases of the lower motor neuron

spinal muscular atrophy

monomelic amyotrophic lateral sclerosis

Kennedy disease

Diseases of the upper motor neuron

hereditary spastic paraparesis

primary lateral sclerosis

Diseases of the neuromuscular junction

Neuromuscular junction or neuromuscular junction- this is the connection of the nerve ending and the muscle fiber with the formation of the so-called synaptic cleft, in which the impulse is transmitted from the nerve to the muscle membrane. The impulse is transmitted using the neurotransmitter acetylcholine, which is secreted by the end of the nerve and then attached to the muscle membrane. In some diseases, there is a violation of neuromuscular transmission due to insufficient release of acetylcholine from the nerve ending or due to a violation of its attachment to the membrane of the muscle fiber.

Myasthenia Gravis

The Greek term myasthenia is translated as "muscle weakness" and gravis as "serious". Myasthenia gravis is a disease characterized by severe muscle weakness and fatigue. With myasthenia gravis, there is a violation of the transmission of impulse from the nerve fiber to the muscle. The disease is based on the production of autoantibodies that block the attachment of the neurotransmitter acetylcholine to the muscle membrane at the neuromuscular junction.

Symptoms

Muscle weakness changes during the day, is usually less pronounced in the morning and increases in the afternoon and evening. early signs diseases are (ptosis), double vision, weakness of the facial muscles, impaired swallowing, chewing, decreased strength in the arms and legs. The disease affects both men and women, and in women the diagnosis is more often made before the age of 40 years, and in men after 60 years.

How is the diagnosis made?

The diagnosis of myasthenia gravis is made by a doctor based on a blood test and electroneuromyography. If necessary, computed tomography of the chest is prescribed to assess the size and condition of the thymus gland as a search for a possible cause of the disease (production of autoantibodies).

Treatment

In the treatment of myasthenia gravis, anticholinesterase drugs (Pyridostigmine or Kalimin) and drugs that suppress the immune system (prednisolone and others) are used. Removal of the thymus gland (thymectomy) is performed when drug therapy is ineffective. Plasmapheresis and immunoglobulins can also be used in the treatment.

Lambert-Eaton syndrome

Lambert-Eaton syndrome is a syndrome of muscle weakness and fatigue that develops due to an autoimmune process. Usually the cause of the syndrome is a malignant oncological process, most often lung cancer. Therefore, when making a diagnosis of Lambert-Eaton syndrome, the patient is always shown an additional examination for the purpose of oncosearch.

Symptoms

Symptoms of the disease are most often associated with weakness of the muscles of the shoulders, hips, neck, swallowing, respiratory muscles, as well as the muscles of the larynx and muscles associated with speech articulation. Early signs of Lambert-Eaton syndrome are usually difficulty walking up stairs, getting up from a sitting position, raising your arms above your head. Sometimes vegetative functions are disturbed, manifested by dry mouth, impotence.

Why does Lambert-Eaton syndrome occur?

The cause is antibodies produced by the body itself (a similar autoimmune conflict is observed in myasthenia gravis). In particular, antibodies destroy nerve endings, thereby disrupting the regulation of the amount of neurotransmitter released. When the amount of the neurotransmitter is insufficient, the muscles cannot contract. The disease is not hereditary, predominantly young people under 40 suffer. The prevalence of the disease is 1 per 1,000,000 people. In 40% of patients with Lambert-Eaton syndrome, cancer is found.

How is Lambert-Eaton syndrome diagnosed?

Diagnosis includes a blood test for antibodies, test administration of an anticholinesterase drug, electroneuromyography.

Treatment

The most effective measure is the removal of a malignant tumor found in the body. Symptomatic therapy includes drugs that increase the release or amount of the neurotransmitter acetylcholine, which acts in the synaptic cleft (kalimin, 3,4-diaminopyridine). Drugs that suppress the immune system (prednisolone, etc.), plasmapheresis and immunoglobulins are also used.

You are an athlete. The pride of bodybuilding, fitness medal, aerobics dream, and other high elements of other areas of beauty and strength. You just need to leave the house, walk down the street, move yourself, smile (at least at the window), and the surrounding population forgets where and why they live. Because visually enjoys you.

And in this case, and in all others, you may be disturbed by a trifle - muscle disease. Don't want it? Nobody wants.

In addition to painful phenomena resulting from injuries (ruptures, sprains, etc.), muscle troubles can occur without external factors. So to speak, on your own!.

muscle cramp

• The manifestation of dehydration of the body (exicosis). Visits at night or in the morning. Mostly elderly, but there are exceptions. When goes to the muscles huge pressure, solidifications appear. Alas. Massage. Visit to the doctor.
• Rheumatic diseases. Pain in hips and shoulders. Muscles may be directly affected (dermatomyositis). Occurs more often in women. Treatment with hormones - glucocorticoids. Anti-inflammatory drugs, physiotherapy.
• Hormonal disorders. Muscle weakness (endocrine myopathy) appears as a result of increased thyroid or adrenal function.
• Muscle inflammation. Inflammation of the muscles (neck, back, chest...) (myositis). Similar to rheumatism, but in addition, the muscles become inflamed. Pain, muscle weakness. The treatment is similar to the treatment of rheumatism.
• Lack of minerals. With potassium deficiency, paralysis smiles. This “smile” is especially felt by young people and children. Treatment - preparations containing potassium. Do not eat before bed, and generally play sports. Or at least the choreography.
• Lack of enzymes. In children, violations of the functions of enzymes that break down glucose and glycogen are more common. The source of energy for the muscles goes on vacation. Physical activity must be avoided.

Painful muscle fatigue

Appears after acidosis. That is, with fair loads, glucose is broken down into lactic acid. And the acid is not easily excreted from the body. Moreover, sometimes causes pain. You will have to drink mangosteen juice (this is what athletes on our planet do), or clean water.

Causes of stretching muscles

• injuries, overloads, sprains;
• taking certain medications (statins, angiotensin-converting enzyme inhibitors);
• inflammatory diseases of an autoimmune nature;
• electrolyte disturbances (lack of potassium and calcium);
• fibromyalgia;
• infectious diseases (influenza, malaria, poliomyelitis, trichinosis, muscle abscess ...);
• systemic lupus erythematosus;
• polymyalgia rheumatica (rheumatic manifestations are generally sociable).

Let your muscles rest. Pamper them with a massage, or even with anti-inflammatory drugs (paracetamol, ibuprofen). Periodically - but carefully, carefully, without overexerting yourself - tempt yourself with physical exercises. And don't meditate in the cold or draft. And then the population will definitely find in you one more medal.

is an inflammatory process in skeletal muscles. Can affect any muscle. most characteristic common symptom is local pain in the muscle (or muscles), aggravated by movement and palpation. Over time, due to the protective tension of the muscles, there may be a limitation in the range of motion in the joints. With a long course of some myositis, there is an increase in muscle weakness, and sometimes even atrophy of the affected muscle. The diagnosis is established on the basis of complaints and examination results. According to indications additional researches are appointed. The treatment regimen is selected individually and depends on the form of the disease and the cause that caused it.

Favorably flowing myositis of mild, less often moderate severity may occur after hypothermia, trauma, muscle cramps or intense physical exertion (especially in patients with untrained muscles). Pain, swelling and weakness for several hours or several days in the latter case is due to small tears in the muscle tissue. In extremely rare cases, usually with extreme physical exertion, the development of rhabdomyosis, necrosis of muscle tissue, is possible. Rhabdomyosis can also occur with polymyositis and dermatomyositis.

In people of certain professions (violinists, pianists, PC operators, drivers, etc.), myositis can develop due to an uncomfortable body position and prolonged stress on certain muscle groups. The cause of purulent myositis can be an open injury with infection, a focus of chronic infection in the body, or local infection due to poor hygiene during intramuscular injections.

Classification

Taking into account the nature of the process in surgery, neurology, traumatologists and orthopedics distinguish acute, subacute and chronic myositis, taking into account the prevalence - local (limited) and diffuse (generalized). In addition, there are several special forms myositis:

Most often, a local process (damage to one or more, but not many muscles) develops in the muscles of the neck, lower back, chest and legs. characteristic symptom myositis is aching pain, aggravated by movement and palpation of the muscles and accompanied by muscle weakness. In some cases, with myositis, there is a slight reddening (hyperemia) of the skin and a slight swelling in the affected area. Sometimes myositis is accompanied by general manifestations: subfebrile temperature or fever, headache and an increase in the number of leukocytes in the blood. On palpation of the affected muscle, painful seals can be determined.

Myositis can develop acutely or have a primary chronic course. The acute form can also become chronic. This usually occurs in the absence of treatment or with inadequate treatment. Acute myositis occurs after muscle strain, injury or hypothermia. Infectious and toxic myositis is characterized by a gradual onset with less pronounced clinical symptoms and primarily chronic.

Chronic myositis proceeds in waves. Pain appears or intensifies with prolonged static loads, weather changes, hypothermia or overexertion. Muscle weakness is noted. It is possible to limit movements (usually insignificant) in adjacent joints.

Types of myositis

Cervical and lumbar myositis

Dermatomyositis and polymyositis

Dermatomyositis belongs to the group of systemic connective tissue diseases. It is quite rare - according to foreign researchers, five people per 1 million people are sick. It usually affects children under the age of 15 or people of mature age (50 years and older). It is twice as common in women as in men.

Classical manifestations of such myositis are considered typical symptoms of the skin and muscles. There is weakness of the muscles of the pelvic and shoulder girdle, abdominal muscles and neck flexors. Patients have difficulty getting up from a low chair, climbing stairs, etc. With the progression of dermatomyositis, it becomes difficult for the patient to hold his head. In severe cases, it is possible to damage the swallowing and respiratory muscles with the development of respiratory failure, difficulty swallowing and a change in the timbre of the voice. Pain syndrome in dermatomyositis is not always expressed. There is a decrease in muscle mass. Over time, muscle areas are replaced by connective tissue, tendon-muscle contractures develop.

From the side of the skin, a heliotrope rash (red or purple rashes on the eyelids, sometimes on the face, neck and trunk) and a symptom of Gottron (pink or red scaly plaques and nodules along the extensor surface of small and medium joints of the extremities) are observed. It may also affect the lungs, heart, joints, gastrointestinal tract and disorders of the endocrine system. Approximately a quarter of patients have manifestations only from the muscles. In this case, the disease is called polymyositis.

The diagnosis is made on the basis of the clinical picture and data from biochemical and immunological blood tests. A muscle biopsy may be performed to confirm the diagnosis. The basis of therapy is glucocorticoids. According to the indications, cytostatic drugs (azathioprine, cyclophosphamide, methotrexate), as well as drugs aimed at maintaining the functions of internal organs, eliminating metabolic disorders, improving microcirculation and preventing the development of complications are used.

Myositis ossificans

It is not one disease, but a group of connective tissue diseases. It is characterized by the formation of ossification areas in the muscles. It can occur due to trauma or be congenital, genetically determined. Traumatic ossifying myositis has a relatively favorable course. Only the muscles and articular ligaments in the area of ​​injury are affected. Treated surgically. The final result of the operation depends on the location and extent of the damage.

Myositis ossificans progressive is hereditary disease. It starts spontaneously, gradually covers all muscle groups. The course of myositis is unpredictable. Specific prevention and treatment does not yet exist. Death in progressive myositis occurs due to ossification of the swallowing and pectoral muscles. It is extremely rare - 1 case per 2 million people.

Myositis treatment

In acute myositis and exacerbation of chronic myositis, the patient is recommended bed rest and limitation of physical activity. When the temperature rises, antipyretics are prescribed. Analgesics are used to combat pain, and anti-inflammatory drugs, usually from the NSAID group (ketoprofen, ibuprofen, diclofenac, etc.), are used to eliminate inflammation. With local myositis, warming ointments are effective. The local irritant effect of these drugs helps to relax the muscles and reduces the intensity of the pain syndrome. Massage is also used (contraindicated in purulent myositis), physiotherapy and physiotherapy exercises. With purulent myositis, an opening and drainage of a purulent focus is performed, antibiotics are prescribed.

Speaking of muscle diseases, meaning diseases of the striated muscles - which a person can control by willpower (the muscles of the internal organs, called smooth muscles, are set in motion regardless of the will of the person, because their function is controlled by the autonomic (autonomous) nervous system).

The main muscle diseases are ruptures (most often the result of trauma), as well as acquired and congenital diseases. The first symptoms of congenital myopathy (muscle weakness and atrophy) appear in children and even newborns. Congenital myopathy is incurable. Acquired myopathy is most often associated with autoimmune diseases (eg, scleroderma, dermatomyositis).

Diagnostics

The diagnosis is established by a blood test. There are certain proteins that in a healthy body are found in muscle cells. When these cells (myocytes) become ill, some of them die, and these protein compounds enter the bloodstream. The doctor determines by a blood test whether the amount of protein in the blood has increased. With the help of a special device, the doctor records an electromyogram, according to its data, one can judge the nature of the muscle disease. In addition, it is necessary to find out if the nerves are affected. For this purpose, a device has been created that provides information on the propagation of impulses along the nerves. The final diagnostic method is a biopsy. The doctor inserts a hollow needle into the muscle tissue and takes a sample of it, which is examined under a microscope. Genetic testing is also carried out to confirm the diagnosis.

muscle tear

Symptoms:

• Muscle pain due to overuse.
• A deepening or swelling is felt in the muscle due to hematoma.

Due to excessive stress, individual muscle fibers or the entire muscle can tear. This can happen during an accident or sports. If most of the muscle fibers are torn, a depression appears, which is determined by touch.

There is severe pain. If most or all of the muscle is torn, surgery is needed. When providing first aid, it is recommended to apply ice to the affected area. Cold reduces pain, acts anti-inflammatory, protects tissues from severe swelling. Later, therapeutic exercises help to strengthen the muscles.

Most often, “cold” muscles are still torn, i.e. when excessive load falls on insufficiently prepared muscles. Therefore, before intensive classes sports need exercises to warm them up (stretching exercises, massage).

congenital myopathy

Symptoms:

• Weakness of the affected muscle.
• Soft, atonic muscles that may be abnormally large or small.
• Pain.
• Seizures.
• Twitching of individual muscle fibers.

All diseases that cause a decrease in muscle tissue and are accompanied by a violation or cessation of muscle function are congenital. At first, it is not possible to diagnose muscle atrophy in newborns. However, it is immediately clear if the newborns have weakened muscle tone. Such people remain disabled for life. In addition, in the presence of some forms of muscle atrophy, the average life expectancy is reduced, children may die in the first or second year of life.

It is necessary to follow a special diet and avoid foods high in carbohydrates and fats. In addition, therapeutic exercises are prescribed, but the exercises are selected very carefully, because. in some diseases it can be harmful.

Spasm

As a result of exicosis (dehydration of the body) and an imbalance of electrolytes (salts), a muscle cramp may appear: the muscles contract and harden, then they slowly relax. This cramp usually occurs at night or in the morning. The person suddenly feels very severe pain. Seizures are especially common in the elderly. If the muscles are constantly under too much load and their nutrition is disturbed, hardening may appear. Muscle fibers are reborn into connective tissue, which is palpable in the form of dense knots. The patient is advised to drink plenty of fluids. Thus, the water-salt balance of the body is restored.

If the painful condition of the muscles does not go away, then you need to see a doctor. Muscle hardening is treated with massage, vitamin E and thermal treatments.

Rheumatic diseases

There are many different rheumatic diseases that affect either the muscles themselves or (more often) the blood vessels that feed them. First of all, there are pains in the shoulders and hips. Some rheumatic diseases, such as dermatomyositis, affect the skeletal muscles. Hormonal treatment with glucocorticoids is effective. They suppress the inflammatory process, but cause many side effects. Therefore, usually the symptoms of rheumatic diseases are tried to suppress with other effective anti-inflammatory drugs or with the help of physiotherapy.

Muscle inflammation (myositis)

The symptoms of myositis are similar to those of rheumatic diseases, however, with myositis, the muscles themselves are also affected. Myositis is characterized not only by pain, but also by pronounced muscle weakness. Myositis is treated in the same way as rheumatic diseases.

Mineral deficiency

Muscles need a sufficient amount of certain substances to function properly. For example, paralysis can occur as a result of potassium deficiency. This is especially acute for children and young people in the morning after a hard day. Treatment is carried out with potassium preparations. In addition, before going to bed, you should not overeat or exercise intensively.

Enzyme Deficiency

Children occasionally have a congenital deficiency of a particular enzyme. Quite often there is a violation of the function of enzymes that break down glycogen and glucose, which are a source of energy for muscles. Due to a congenital deficiency of enzymes, the muscles receive insufficient energy, resulting in their weakening. A person with enzyme deficiency syndrome should avoid intense physical activity.

Painful fatigue

Painful muscle fatigue occurs due to acidosis. To obtain the necessary energy during heavy physical exertion, the existing glucose is broken down to lactic acid, which the blood is not able to quickly remove from the body. Lactic acid begins to build up in the muscles, causing pain.