Contents
Myasthenia gravis is a chronic disease in which the muscles suffer. The main manifestation of the pathology is their rapid fatigue. The peculiarity of the disease is its constant progression. The cause of myasthenia is hidden in the disorder of neuromuscular connections.
Кроме повышенной утомляемости мышц, у человека нарастает мышечная слабость, что негативным образом сказывается на объёмах выполняемых действий.
Myasthenia is accompanied by myasthenic crises, which can end in the death of a person. Modern medicine can reduce the number of deaths from this pathology. Thanks to complex therapy, it is possible to achieve a stable remission of the disease.
Что такое миастения?
Myasthenia gravis is an autoimmune disease that affects the receptors responsible for the connection between the muscles and the nervous system. The immune system begins to attack the body’s own cells, which are perfectly healthy. This can lead to a violation of swallowing movements, to uncharacteristic weakness of the respiratory organs, as well as to other equally severe disorders. As the pathology progresses, the muscles of the lips, face, pharynx, tongue, neck, eyes, and the whole body atrophy.
Заболевание сводится к тому, что нервный импульс передаётся от головного мозга к мышцам с нарушениями. Это происходит из-за сбоя в метаболизме ацетилхолина в организме.
Skeletal muscles are responsible for the movement of human limbs. The work of internal organs and blood vessels is provided by smooth muscles. To set the skeletal muscles in motion, the motor nerves will be involved. Where a nerve connects to a muscle, an electrical impulse is generated. Acetylcholine is required for its transmission. This is a special substance that enters the gap between the nerve and the muscle, providing an electrical discharge. It causes the muscle to contract.
When a person develops myasthenia gravis, the synthesis and release of acetylcholine is disrupted. Therefore, the impulse will fail to pass to the muscle. Each time the conductivity gets worse. Therefore, human movements suffer. In the terminal stages of the disease, the patient is completely immobilized.
Нередко миастения развивается на фоне опухоли вилочковой железы, а также при гиперплазии. Иногда у пациентов выявляют органическое поражение нервной системы, опухоли молочных желёз, опухоль лёгких, рак яичников или простаты.
Causes of myasthenia gravis
To date, the reasons why the disease develops are unknown to science. Doctors can only name risk factors that increase the likelihood of myasthenia gravis.
They include:
Hormonal imbalance.
Chronic stress.
Taking certain drugs that affect the work of the musculo-nerve synapse.
Infectious diseases.
Рак лёгких, молочной железы и других органов. Растущая в организме опухоль заставляет тимус работать на пике его возможностей, а также провоцирует гиперплазию железы.
Myasthenia classification
Миастения, в зависимости от характера её возникновения может быть двух форм:
congenital disease. Its cause is a gene mutation that occurs even during the intrauterine development of the child. The first signs of the disease occur immediately after the baby is born. The respiratory function suffers, which often causes the death of the baby.
Acquired disease. In most cases, people are diagnosed with acquired myasthenia gravis, which develops throughout life.
Depending on the age of the person, myasthenia gravis is divided into adult and pediatric.
У детей различают три формы болезни:
Neonatal myasthenia gravis, which is transmitted from mother to child.
Early childhood myasthenia gravis, which manifests itself at the age of 3-5 years.
Миастения юношеская, которая развивается в возрасте 12-17 лет.
В зависимости от того, какие именно мышцы подверглись поражению, различают следующие формы болезни:
Local myasthenia. Certain muscle groups are affected. The muscles of the face are often affected.
Generalized myasthenia gravis. In this case, various muscles of the body will be affected. This form of pathology progresses rapidly and has a severe course.
Depending on which muscle groups are affected in local myasthenia gravis, the following forms are distinguished:
Myasthenia gravis. The muscles of the limbs are affected.
Myasthenia gravis of the eyes and eyelids. The organs of vision suffer, it begins to fall. The patient’s upper eyelid droops. Over time, the patient develops myopia.
Myasthenia pharynx and face. It is difficult for a person to swallow food, his speech suffers, his voice changes to nasal.
Depending on the nature of the course of myasthenia gravis, the following types are distinguished:
Myasthenic crises, which are accompanied by impaired motor activity. After their completion, the symptoms of the disease regress.
myasthenic condition. This is a classic myasthenia gravis that progresses over several years.
Progressive myasthenia gravis. The disease progresses all the time, but doctors regard it as a relatively benign type of pathology.
malignant form. Pathology develops quickly, muscle performance is severely impaired.
Миастенические кризы являются наиболее опасными состояниями для жизни человека.
Symptoms of myasthenia gravis
Symptoms of myasthenia gravis include the following:
Uncharacteristic for a healthy person muscle weakness.
Нарушение функционирования глазных, лицевых, жевательных и бульбарных мышц, мышц ног и рук.
Double vision.
Офтальмопарез.
Strabismus.
Птоз.
Difficulty swallowing food.
Loss of voice, articulation disorder, voice change.
Gait disturbance.
Increased drowsiness, rapid fatigue.
Muscle pain.
As the pathology progresses, they will become more pronounced. Over time, a person develops myasthenic crises. They are accompanied by fainting and acute oxygen starvation of the brain.
Myasthenic crisis
Over time, the person will feel worse and worse. A severe form of the disease will lead to the fact that he will begin to have myasthenic crises. This condition is understood as a sharp deterioration in well-being. The crisis develops due to acute muscle weakness.
Сначала дыхание человека учащается, а затем урежается и становится прерывистым. Лицо приобретёт багряный цвет. Пульс ускоряется, артериальное давление повышается до 200 мм рт. ст.
When breathing stops, the person loses consciousness. If at this time he is not provided with medical assistance, he will die.
Diagnosis of myasthenia gravis
Диагностические мероприятия, направленные на обнаружение миастении:
Collection of anamnesis:
The doctor specifies how long ago the person had disturbing symptoms: muscle weakness, double vision, drooping of the upper eyelid.
The doctor finds out if there were cases of myasthenia gravis in the family, or other autoimmune diseases. In this regard, the danger is rheumatoid arthritis, etc. It is also necessary to clarify whether someone in the family had cancer.
In addition, the diagnosis of myasthenia gravis includes:
Neurological examination. Доктор проводит тесты с повторяющимися движениями. Слабость у человека с миастенией будет усиливаться.
Proserine test. Врач оценивает мышечную силу пациента. Затем ему ставят инъекцию прозерина (это препарат усиливает нервный импульс, подаваемый к мышцам). Затем доктор снова измеряет мышечную силу больного. Если у него развивается миастения, то она повышается.
Determination of antibodies in the blood. The analysis will show the presence of proteins that are produced against acetylcholine receptors. Most often they circulate in the blood, but their absence is not proof of the refutation of the diagnosis.
Conducting electroneuromyography. This diagnostic procedure allows you to assess the excitability of the muscles and the conductivity of the nerve impulse. Myasthenia gravis is accompanied by normal impulse transmission, but the muscles do not give the desired reaction to it. With prolonged stimulation of muscles by electric current, the doctor reveals the phenomenon of post-tetanic exhaustion.
CT and MRI of the anterior mediastinum. These studies provide information about the condition of the chest wall. They allow you to identify a tumor of the thymus, which is often combined with myasthenia gravis.
Лечение миастении
Treatment of myasthenia gravis is implemented along 2 lines: efforts must be directed to the removal of pathological symptoms and to influence the cause of the disease. To stop the symptoms of the disease, the patient is prescribed anticholinesterase drugs. Treatment begins with minimal dosages. In the future, the doctor raises them, bringing them to the optimal values for a particular patient. To reduce the amount of antibodies in the blood, the patient is prescribed corticosteroid drugs and immunosuppressants.
Medicines that are used to combat myasthenia gravis:
К прочим методам лечения миастении относят:
Plasmapheresis, which allows you to mechanically purify the blood of antibodies. Several portions of blood are taken from the patient and passed through a centrifuge. Blood cells are preserved, and antibodies are removed from it. Then the person’s own blood is diluted with rheopolyglucin and returned to the patient’s bloodstream through a vein.
Операция по удалению вилочковой железы. Эта процедура позволяет справиться с активно прогрессирующей миастенией на фоне опухоли тимуса с нарушением процесса проглатывания пищи.
Облучение тимуса гамма-лучами. Эта процедура позволяет уменьшить интенсивность воспалительного процесса.
Extracorporeal immunopharmacotherapy. Patients secrete lymphocytes from the blood, which are treated with drugs and returned to the human body. This procedure allows you to achieve a stable remission of the disease for a year.
Прохождение лечения в санаториях.
Человек с миастенией должен настроиться на пожизненный приём препаратов. Назначают их курсами, в ходе которых пациент должен находиться под контролем врача. Каждый день необходимо измерять уровень артериального давления. Важно знать показатели глюкозы крови. Параллельно проводят профилактику остеопороза. Когда заболевание обостряется и справиться с ним приёмом препаратов не удаётся, человеку показан плазмаферез. Проводят процедуру до 7 раз в день.
If the patient has a crisis, then he is resuscitated and connected to a ventilator. Simultaneously, plasmapheresis is performed. To purify the blood from antibodies, the patient is prescribed cryopheresis, cascade plasma filtration, immunopharmacotherapy. Such therapeutic measures allow you to cope with myasthenia gravis for up to a year.