Myasthenia gravis: symptoms and treatment, causes
What it is? Myasthenia gravis is a classic autoimmune disease that is characterized by impaired excitation transfer at the level of the neuromuscular synapse, manifested by pathological impotence, fatigue of predominantly skeletal muscles intended for performing various actions.
The danger of the disease lies in the development of critical conditions - myasthenic crises, which can result in a fatal outcome.
Although it should be said that modern methods of diagnosis and treatment have made it possible to reduce the death rate as much as possible, and the majority of patients achieve stable remission.
How does myasthenia develop and what is it? Most often the disease begins to manifest itself in adolescence, but the peak incidence falls on 20-40 years. According to statistics, women are more susceptible to it, but in recent years, men are increasingly ill.
This form is extremely rare and manifests itself from the first minutes of a child's life with shortness of breath due to muscle atrophy. Mortality is high.
The acquired form of myasthenia gravis is more prevalent. Often it causes an infectious disease. A provoking factor can be stress. Under the influence of various causes, the work of the immune system is disrupted, and the body begins to produce antibodies that act against their own cells.
The role of autoimmune processes is obvious, as antibodies were detected in muscles and thymus gland during the studies. Often, myasthenia gravis is associated with thymoma( a tumor from epithelial cells of the thymus) because it is the most common syndrome that accompanies this disease.
Thymus changes in the form of hyperplasia occur in most patients with myasthenia gravis. Its cells can be generators of autoantigens and trigger a pathological process, the mechanisms of which have not yet been fully explored.
By inheritance the disease is not transmitted, but there is a high probability of its new appearance in predisposed families. Very often tandems - mother-daughter or sister-sister. There are reports that some medications can cause a debut or relapse of the disease. They block neuromuscular transmission and provoke only short-term myasthenic symptoms in healthy people in this regard.
But if the synapse defect is latent, drugs can trigger the occurrence of pathological processes. These drugs include antibiotics, antiarrhythmics, calcium channel blockers.
For the first time, myasthenia gravis begins in many cases with a sudden attack, when swallowing and breathing are disturbed. This condition is called a crisis, the indirect factors of which can be:
Myasthenia gravis, the main symptoms of which are muscle weakness and fatigue. Oculomotor, facial, chewing, and also muscles of the upper and lower extremities are more often affected. Weakness progresses with the continuation of movements and passes after rest. There are obvious and hidden symptoms of myasthenia gravis. The most common include:
Myasthenia gravis is a chronic disease that progresses and eventually causes muscle atrophy. Pathology requires constant monitoring and treatment, because it often leads to disability.
There are many effective methods for diagnosis of myasthenia gravis. They are recommended to be used in an integrated manner in order to avoid mistakes in the diagnosis. Often at first the clinic may not be clear, since the symptoms are similar to the manifestations of many other diseases. The professional level of the doctor is very important.
Diagnosis is performed using:
Myasthenia gravis is treated for years with the use of anticholinesterase drugs( AKHEP), which include kalimine, proserin, galantamine. These drugs differ in the main duration of action.
Usually, preference is given to calymin( valid for 4-5 hours).In addition, prescribe potassium preparations, since they have the property of prolonging the action of AHEP.
Highly effective appointment of glucocorticoids( prednisolone) and immunoglobulins. The dose is adjusted depending on the severity of the course of the disease. On the basis of therapy there is a stable remission. In the presence of progressive malignant thymoma, surgical intervention or X-ray irradiation of the thymus gland is indicated.
In order to treat myasthenia gravis it was necessary to follow the rules:
The danger of the disease lies in the development of critical conditions - myasthenic crises, which can result in a fatal outcome.
Although it should be said that modern methods of diagnosis and treatment have made it possible to reduce the death rate as much as possible, and the majority of patients achieve stable remission.
Causes of myasthenia gravis
How does myasthenia develop and what is it? Most often the disease begins to manifest itself in adolescence, but the peak incidence falls on 20-40 years. According to statistics, women are more susceptible to it, but in recent years, men are increasingly ill.
Muscle weakness can be congenital and acquired. In the case of congenital myasthenia, the cause of the disease is the gene mutation of proteins involved in the functioning of neuromuscular synapses.
This form is extremely rare and manifests itself from the first minutes of a child's life with shortness of breath due to muscle atrophy. Mortality is high.
The acquired form of myasthenia gravis is more prevalent. Often it causes an infectious disease. A provoking factor can be stress. Under the influence of various causes, the work of the immune system is disrupted, and the body begins to produce antibodies that act against their own cells.
The role of autoimmune processes is obvious, as antibodies were detected in muscles and thymus gland during the studies. Often, myasthenia gravis is associated with thymoma( a tumor from epithelial cells of the thymus) because it is the most common syndrome that accompanies this disease.
Thymus changes in the form of hyperplasia occur in most patients with myasthenia gravis. Its cells can be generators of autoantigens and trigger a pathological process, the mechanisms of which have not yet been fully explored.
By inheritance the disease is not transmitted, but there is a high probability of its new appearance in predisposed families. Very often tandems - mother-daughter or sister-sister. There are reports that some medications can cause a debut or relapse of the disease. They block neuromuscular transmission and provoke only short-term myasthenic symptoms in healthy people in this regard.
But if the synapse defect is latent, drugs can trigger the occurrence of pathological processes. These drugs include antibiotics, antiarrhythmics, calcium channel blockers.
Symptoms of myasthenia gravis
For the first time, myasthenia gravis begins in many cases with a sudden attack, when swallowing and breathing are disturbed. This condition is called a crisis, the indirect factors of which can be:
- long stay under the sun;
- stress;
- colds;
- intensive physical activity.
Myasthenia gravis, the main symptoms of which are muscle weakness and fatigue. Oculomotor, facial, chewing, and also muscles of the upper and lower extremities are more often affected. Weakness progresses with the continuation of movements and passes after rest. There are obvious and hidden symptoms of myasthenia gravis. The most common include:
- ptosis( descent of the upper eyelid);
- diplopia( double vision);
- violation of chewing, swallowing;
- change the voice, right up to its loss;
- weakness of the extremities in the proximal parts( shoulders, hips);
- drooling;
- changing facial expressions;
- difficulty with head retention due to neck muscle damage;
- change of gait;
- self-service problems;
- development of paralysis.
Myasthenia gravis is a chronic disease that progresses and eventually causes muscle atrophy. Pathology requires constant monitoring and treatment, because it often leads to disability.
Diagnosis of myasthenia gravis
There are many effective methods for diagnosis of myasthenia gravis. They are recommended to be used in an integrated manner in order to avoid mistakes in the diagnosis. Often at first the clinic may not be clear, since the symptoms are similar to the manifestations of many other diseases. The professional level of the doctor is very important.
Diagnosis is performed using:
- 1) Electromyography - a method for studying bioelectric potentials that arise in skeletal muscles when fibers are excited;
- 2) Electroneurography, which allows to investigate the speed of pulses passing along the nerves;
- 3) Genetic analysis that is conducted to identify congenital pathology;
- 4) Proserin test, when a solution of prozerol is injected subcutaneously( up to 3 ml).The sample is evaluated after about 20-30 minutes. When myasthenia gravis after the introduction of this drug symptoms completely pass, and the person feels healthy, but after a few hours they resume;
- 5) Laboratory methods for the detection of antibodies;
- 6) Magnetic resonance imaging, capable of detecting a tumor of the thymus gland.
Treatment of myasthenia gravis
Myasthenia gravis is treated for years with the use of anticholinesterase drugs( AKHEP), which include kalimine, proserin, galantamine. These drugs differ in the main duration of action.
Usually, preference is given to calymin( valid for 4-5 hours).In addition, prescribe potassium preparations, since they have the property of prolonging the action of AHEP.
Highly effective appointment of glucocorticoids( prednisolone) and immunoglobulins. The dose is adjusted depending on the severity of the course of the disease. On the basis of therapy there is a stable remission. In the presence of progressive malignant thymoma, surgical intervention or X-ray irradiation of the thymus gland is indicated.
In order to treat myasthenia gravis it was necessary to follow the rules:
- to exclude heavy physical labor;
- does not rest in countries with hot climates;
- does not sunbathe under the open sun;
- is observed regularly with a neurologist;
- always carry a list of unacceptable drugs;
- does not engage in self-medication.