Myasthenia gravis is a chronic neuromuscular disease manifested by a recurrent or gradually progressive type of course. People with myasthenia gravis suffer from increased muscle fatigue, which at the end of the day sometimes leads to severe paresis of the muscles affected by the pathological process.

Reasons

There is no consensus on the causes of this disease. At different times, hereditary, infectious, and autoimmune theories were expressed. There is also a clear connection between the development of this condition and a tumor of the thymus gland (thymus), although the latter is not always present, and its removal does not always lead to an improvement in the condition.

It should be said that the risk of developing myasthenia gravis increases significantly with an existing family history. However, among sick people in the same family, very often the manifestations of myasthenia gravis vary from minimal (ocular myasthenia) to the maximum possible (myasthenia gravis).

The disease is quite common, the incidence of myasthenia gravis varies from 5 to 10 people per 100,000 population. Women are more likely to get sick (in a ratio of 2 to 1); the age of onset of the disease is mainly between 15 and 45 years. Cases have been described where the disease occurs in children. More often there is early muscle weakness, which resolves during the first month of life.

It should be noted the possibility of developing myasthenic syndrome against the background of existing chronic diseases: dermatomyositis, tumors of endocrine organs, systemic connective tissue diseases, etc.

Symptoms

The symptoms vary in severity and localization, but they can be characterized by one phrase – muscle weakness. Depending on the localization of the process and the severity of manifestations, several forms of myasthenia are distinguished:

• Eye shape.
• Bulbar form.
• Generalized form (myasthenia gravis, Erb-Goldflam disease).
• Malignant myasthenia fulminans.
• Myasthenic crisis.

Ocular myasthenia is manifested primarily by the development of eyelid weakness, often on one side or unevenly on both sides. At the same time, at the beginning of the disease, the eyelid droops in the evening, after visual stress. Double vision and other transient visual disturbances are often observed. If within two years from the onset of the disease the process has not moved to other anatomical zones, then further generalization becomes unlikely. Localized myasthenia gravis (ocular form) is not life-threatening.

The bulbar form of myasthenia is characterized by the development of weakness of the muscles of the soft palate, tongue, and pharynx. The patient suffers from speech disorders and swallowing disorders. Moreover, the changes change dynamically throughout the day, with different loads, which distinguishes this form of the disease from true bulbar syndrome.

The generalized form is widespread muscle weakness. It all starts, as a rule, with the muscles of the neck; it becomes difficult for a sick person to hold his head, gradually the disease spreads to other muscle groups, and paresis of the muscles of the limbs begins to appear. The patient's gait is disturbed and facial expressions are distorted. There is a noticeable improvement in the condition in the morning, after a nap, and after a long rest. The slightest physical activity sometimes leads to severe weakness.

The malignant type of the disease occurs only in the presence of a thymus tumor, often malignant. The disease debuts at the age of about 50 years and within a short period of time (months, and sometimes even weeks), leads to complete disability. At extreme stages, the skeletal respiratory muscles are affected with the likelihood of developing life-threatening conditions.

Myasthenic crisis is a special manifestation of the generalized form of this pathology. The exact causes of the crisis have not been identified; it often develops against the background of menstruation, psycho-emotional stress, fever or other reasons. Insufficient dosage of medications in the treatment of the disease can also lead to the development of complications. During a crisis, pronounced weakness of all muscles, including the respiratory ones, develops. The duration of the crisis varies very widely and can last from several tens of minutes to hours, to weeks, and in some cases, months.

Difference between myasthenic crisis and cholinergic crisis.

Diagnostics

As a rule, diagnosing the disease is not difficult, because all manifestations of myasthenia gravis are expressed quite characteristically. Also, even with a standard neurological examination, rapid muscle fatigue, myasthenic reaction of tendon reflexes and gradual exhaustion can be detected. Special tests and additional research methods are also used:

Test of drooping eyelids - there is a characteristic look from under drooping eyelids, a person can rarely look with his eyes open, the eyelids quickly droop.
Cooling test. It is carried out more often on the eyelids. A piece of ice is placed on one eyelid; after cooling, an improvement in the functioning of the eyelid is noted.
Proserine test. (there will be a section in symptoms-syndromes later, and check the request) The patient is injected subcutaneously with 2-3 ml of proserine 0.05% solution. With myasthenia gravis, the function of the affected muscles is practically restored after half an hour. This state sometimes returns to its original state no earlier than 2-3 hours.
makes it possible to identify a characteristic myasthenic reaction of neuromuscular transmission and to identify rapid muscle fatigue upon repeated stimulation.

Treatment

Treatment of myasthenia gravis is not the easiest task. The patient should be immediately set up for constant or very long-term use of anticholinesterase drugs that act on the pathogenesis of the disease. Kalimin drugs, and the less commonly used proserin, should be carefully dosed, taking into account body weight, severity of symptoms, age and other factors.

Hormone therapy is often resorted to, especially with acute development of symptoms and their rapid progression. Most often, pulse therapy is carried out in high doses, with a further transition to minimal dosages. It is also advisable to conduct sessions of plasmapheresis (purification of blood from circulating autoimmune complexes) while using pulse therapy with hormones (metipred, dexamethasone, etc.).

Treatment of myasthenic crisis should begin with maintaining vital functions, gradually moving to a course of plasmapheresis, hormonal therapy, and clarifying the presence of a thymic tumor. The presence of thymoma (especially growing) in combination with frequent myasthenic crises is an indication for surgical removal of thymoma. Unfortunately, only about 50% of patients report improvement.

Also, therapy for myasthenia gravis should include pure potassium preparations (in no case Panangin, because magnesium can lead to worsening of symptoms!), the prescription of potassium-sparing diuretics (veroshpiron), metabolic drugs (riboxin, B vitamins). The prescription of physiotherapy, special physical therapy exercises, and massage is justified.

List of drugs that cannot be used for myasthenia gravis

You should also strictly adhere to specific approaches to the treatment of other diseases. There is a list of drugs that cannot be used for myasthenia gravis:

What if pregnancy?..

It should be noted that pregnancy is not a contraindication to the prescription of the main drugs for the treatment of myasthenia gravis. During pregnancy, you should avoid hormone therapy, plasmapheresis sessions, and the use of large doses of potassium-containing drugs.

Myasthenia gravis is a severe autoimmune disease that manifests itself as pathological muscle weakness and slowly progresses. Children most often suffer from it, but this pathology also occurs in adults.

A little about the reasons

Myasthenia gravis is a congenital hereditary disease. Its signs appear in early childhood. The syndrome can develop at different speeds and degrees of severity. Due to genetic abnormalities, the connection between neurons and muscle fibers is disrupted. Due to the fact that the muscles actually turn off and do not function, their atrophy gradually develops.

Scientists have still not been able to fully identify the mechanism of the disease, but it is known for certain that the cause lies in a deficiency of the gene that is responsible for the functioning of myoneural connections. First of all, visual functions suffer, as the eye muscles atrophy. Then the process moves to the facial muscles, neck, muscles of the arms, legs, and swallowing muscles.

Often this congenital syndrome leads to serious consequences and even death of the patient, but with proper treatment, recovery or temporary remission is possible. This pathology can be inherited from one of the parents or through a generation.

The following causes of disease among children are identified:

1. Failures of biochemical processes due to pathologies of the thymus and hypothalamus.
2. The thymus is attacked by its own immune cells, which causes less acetylcholine to be produced and broken down.

Please note that the condition of a sick child can be worsened by stressful situations, acute respiratory viral infections, and impaired immunity.

Symptoms

The symptoms of myasthenia gravis directly depend on its form. The main symptom is unusual weakness in the muscles. The patient gets tired quickly and is unable to cope with work or training. This is especially noticeable if you need to make a series of similar movements.

After rest, muscle function is restored. Waking up in the morning, patients feel cheerful, rested, and feel a surge of strength. After some time, characteristic symptoms begin to increase, the patient feels literally overwhelmed.

Myasthenia gravis

Myasthenia gravis can manifest itself in different ways, depending on the form. There are three of them:

1. bulbar;
2. ophthalmic;
3. generalized.

In the bulbar form, only one local muscle group is affected. They provide chewing and swallowing, so the patient’s voice begins to change. It becomes hoarse, quiet and even almost silent.

In the ocular form of myasthenia, the muscles that allow movement of the eyeballs are affected. These are the muscles that raise the eyelid, the external circular muscle. A patient suffering from myasthenia gravis is easily recognized by drooping eyelids - he cannot lift them due to muscle damage.

If myasthenia gravis is generalized, the oculomotor, facial, and cervical muscles are gradually affected. Patients develop deep wrinkles on their faces, and their smile becomes unnatural and forced. Over time, it becomes difficult for a person to even hold his head up. This is a consequence of weakened neck muscles.

As the disease progresses, the muscles of the arms and legs are involved in the pathological process. Such patients practically lose the ability to walk and move, since the muscles do not experience normal stress, and over time they atrophy. It is the generalized form that occurs most often.

Myasthenia gravis may also be accompanied by characteristic crises. This is the most severe form of the disease. During a crisis, the pharyngeal and respiratory muscles are completely disabled. This is a direct threat to life, because the movements of the chest completely stop, resulting in oxygen starvation of the body.

Diagnostics

It is very important to conduct a thorough examination to understand the extent to which the disease progresses and what causes it to develop. To select the correct treatment regimen, it is necessary to go through all stages of diagnosis. It includes:

1. Electromyography. It will help identify myasthenic reaction.
2. Proserine test. The patient is injected into the muscle with cholinesterase antagonist drugs.
3. Serology study. Its goal is to identify acetylcholine receptor antibodies in the patient.
4. CT. Helps identify possible tumors (for example, thymoma).

It is the proserine test that is the main diagnostic method that can definitively confirm myasthenia gravis.

Treatment

Myasthenia gravis is a serious and life-threatening pathology. When such a diagnosis is made, it is imperative to immediately begin treatment for myasthenia gravis. Often, ophthalmological treatment is also required, since the disease can provoke dysfunction of the eyes. You will also need to eat right.

The mechanism of therapy is based on the fact that new manifestations of myasthenia gravis are constantly taken into account and the dose of drugs is adjusted. It should not exceed that which provides a sustainable therapeutic effect. Sick children and young people respond more easily to therapy; in older people, remission occurs less frequently.

It is important for parents to remember that even a common cold can cause myasthenia gravis, so any infectious disease should be treated. Such well-known infectious disease specialists as, for example, Academician Yuri Vladimirovich Lobzin insist on this. It is important to choose a good clinic that will provide all modern methods of treating this difficult disease.

Proper treatment can stop the progression of the disease, and in some cases, complete recovery can be achieved. Therapy must fully comply with modern standards, since in recent years the methods of treating myasthenia gravis have been significantly improved in medicine.

The symptoms of a particular patient must be taken into account. The disease can have quite different forms and severity. It all depends on the reason for its development. This can be not only a broken genetic code, but also an infectious lesion, head injury, snake bite, etc.

Treatment will be based on maintaining the required level of anticholinesterase substances in the blood. These drugs are constantly introduced into the body. Sometimes it is difficult to immediately determine a safe dose for a particular patient, so the administration of drugs begins with extremely small doses. Such patients require constant care and regular courses of treatment.

An overdose of these drugs is fraught with serious side effects and unpleasant effects on the liver and kidneys. It can even provoke a cholinergic crisis, which manifests itself in the form of convulsions, miosis, and bradycardia. They are accompanied by pain in the abdominal area. If such a crisis occurs, the patient is immediately given the required dose of atropine.

The essence of the treatment is that an acetylcholinesterase antagonist is selected for the patient. This selection is carried out strictly individually. It is important to take into account the patient’s age, weight, shape and severity of the disease. Oxazil, prozerin, galantamine or kalimine are also prescribed.

If pseudoparalytic myasthenia gravis is diagnosed, the patient is additionally administered spironolactone and potassium salts. They maintain the condition of the body. If the patient suffers from a severe form of the disease, glucocorticosteroids and cytostatics are prescribed. If thymoma is detected, the only treatment option is surgical removal of the tumor.

To relieve myasthenic crisis, prozerin, mechanical ventilation, plasmapheresis, and drugs based on human immunoglobulin are used. If it is gravis disease (a severe hereditary form), treatment will differ from treatment for other forms of the disease.

The most commonly prescribed drug is pyridostigmine bromide. The drug causes a number of side effects: diarrhea, abdominal pain, muscle fasciculations. An increased dose of the drug can cause a cholinergic crisis.

Immunomodulatory treatment

One of the areas of therapy is modulation of immunity. For this purpose, glucocorticoids are prescribed. They are effective, relatively safe and inexpensive. This is the secret of their worldwide popularity. Scientists have not yet fully figured out how these drugs work, but the fact is undeniable that they can significantly alleviate the patient’s condition and lead to long-term remission.

This group of medications has a number of side effects, but they directly depend on the dose. Therefore, the physician must prescribe the minimum effective dosage for a particular patient. The most popular drug in this group is Prednisolone.

It is prescribed with a minimum daily dose (10-25 mg) and then the dosage is slowly increased. Ideally, the daily dose should be 60-80 mg (one-time dose every other day). It can be replaced with Methylprednisolone.

If the patient suffers from a severe form of the disease, treatment is prescribed immediately with a high dose of corticosteroids. The drug is administered every day. At the same time, plasmapheresis is performed or immunoglobulin is administered intravenously. The goal of such enhanced therapy is to stabilize the patient’s condition. It will take from 4 to 16 weeks to achieve it. After improvement, the dose of corticosteroids is gradually reduced. They are brought to the level of maintenance therapy.

Azathioprine is a purine analogue that slows down the synthesis of nucleic acids. It affects lymphocytes. When using the drug, it is necessary to monitor liver function and blood condition. At first, a blood test is taken every day. If the drug is well tolerated by the patient, then after 1-2 weeks the dosage is increased. The maximum dose is 2-3 mg per kg of body weight (the average daily dose is 150-200 mg).

This drug is quite well tolerated, although it can sometimes cause nausea, lymphopenia, skin rashes, pancreatitis, and pancytopenia.

Please note that the therapeutic effect may not occur immediately. It often appears 4-12 months after the start of treatment. The maximum effect is usually observed after six months to a year.

Azathioprine is used as an addition to Prednisolone. It is prescribed to those patients who are taking long-term immunosuppressive therapy. Thanks to this combination, the dose of corticosteroids can not be increased without losing their effectiveness. This is the so-called sparring effect, when one drug enhances the healing effect of another.

Cyclosporine is another drug prescribed for myasthenia gravis. It has a complex action that ultimately results in slower T cell activation. The drug can cause tremor, insomnia, kidney failure, high blood pressure, and headache. These side effects depend on the dose used. If it is reduced, unpleasant manifestations may go away or be minimized.

Cyclosporine is prescribed quite rarely. It has much more pronounced side effects than other drugs, so this drug is used if the others have shown low effectiveness. If a medication is prescribed, it is important to monitor the level of electrolytes in the blood, magnesium, and kidney function. Start with small doses, gradually increasing the daily dosage to a therapeutically effective one.

If Cyclosporine is prescribed, diuretics (potassium-sparing) and NSAIDs should not be taken, and when corticosteroids must be taken, their dose should be reduced as much as possible. It is not possible to completely stop Prednisolone.

Mycophenolate mifetil is a modern drug. Scientists have not yet fully studied how it works, but the results are encouraging. The substance slows down the replication of B and T cells. When using the drug, you need to take a blood test every month. Scientists agree that mycophenolate myfetil is as effective as Cyclosporn, but has fewer side effects.

Cyclophosphamide is an effective immunosuppressant, which is prescribed for severe forms of the disease, inhibiting T and B cells. It is rarely prescribed, only when other drugs have not shown their effectiveness. After just a couple of months, stable remission is observed in 50% of severe patients. If noticeable side effects occur, this remedy will have to be discontinued.

Methotrexate slows down cell division, but can provoke nausea, cystitis, mucositis, alopecia, and myelosuppression. Doctors consider it as a backup drug if first-line treatments are ineffective.

Rituximab is an antibody that has increased affinity for the CD20 cell antigen. It can cause fever, skin rashes, nausea, and sometimes bronchospasm. You can take a fairly long break between taking it - up to six months.

Short-term therapy

Along with medications, short-term treatment is prescribed: plasmapheresis, immunoglobulin is administered intravenously.

The mechanism of action of immunoglobulin is that it neutralizes activated complement, autoantibodies, modulates cytokines, etc. it can cause fever, headache, and skin rash.

The purpose of plasmapheresis is to remove autoantibodies and other components produced by the immune system from the blood. 4-5 sessions of plasmapheresis are performed. More often it is prescribed in the process of preparing for cooperation, a serious condition, when symptoms quickly increase. These two methods have approximately the same effectiveness.

Surgical method of treatment

The surgical method is thymectomy. It is most often used to treat myasthenia gravis. It is carried out if an accurate diagnosis of thymoma is made, and also if patients with a generalized type of the disease have not reached 60 years of age.

Please note that thymectomy is not always indicated for the generalized form.

During pregnancy, medications are selected with special care.

Pathogenesis

In recent years, there has been a lot of new information about the pathogenesis and clinical manifestations of myasthenia gravis. Scientists have made significant progress in studying the mechanism of its development, although there are still many gaps.

Now it is possible not only to achieve stable remission, but also to predict the further development of the disease in a particular patient. During therapy, it is important to take into account the nature of the disease in an individual patient, the presence of therapeutic and side effects, and their relationship.

It is important that the doctor understands the essence of this problem, the main stages of the development of the disease, thoroughly knows the entire arsenal of modern therapeutic agents, and knows the treatment algorithms. All this will allow us to provide the patient with the most effective care.

If myasthenia gravis is detected, the prognosis depends on the form of the disease, as well as the timeliness and systematicity of treatment. The generalized form of myasthenia gravis is the most difficult to treat.

The disease is periodic and rhythmic in nature. Remission is replaced by a period of exacerbation. It is important to regularly stimulate the muscles so as not to lead to their atrophy. This is facilitated by physical therapy and gymnastics.

So, for successful treatment of myasthenia gravis, it is important to conduct a full diagnosis and identify the cause of the pathology. Then a set of drugs is selected that have a minimum of contraindications and side effects. This disease is extremely dangerous, therefore only doctors should treat it. Folk remedies are strictly contraindicated.

18596 0

Myasthenia gravis is a neuromuscular disease characterized by muscle weakness and pathological fatigue. Its development is based on a violation of neuromuscular transmission due to blockade and lysis of acetylcholine receptors of the postsynaptic membrane by polyclonal autoantibodies. The production of antibodies is due to a genetic predisposition to immune system disorders. Damage to skeletal muscles such as polymyositis is also associated with this. In 70-90% of patients, pathology of the thymus gland (hyperplasia, thymitis, thymoma) is detected.

There are localized (ocular, bulbar, skeletal or trunk) and generalized forms of the disease. When making a diagnosis, take into account the increase in muscle weakness in the evening or after physical activity, as well as a significant decrease or complete disappearance of symptoms after administration of 2 ml of a 0.05% solution of proserin. EMG (using the rhythmic stimulation method) reveals the myasthenic exhaustion reaction.

Treatment. Anticholinesterase drugs (ACEDs) are prescribed, which increase the content of acetylcholine in the neuromuscular junction, and a number of measures are taken that affect the immune state - thymectomy, radiation exposure to the thymus gland, corticosteroid drugs, immunosuppressants, plasmapheresis and hemosorption are used.

ACEPs differ in duration of action (Table 26), effectiveness in different forms of the disease (kalimin is more effective in the ocular form, and oxazil in the bulbar and trunk forms), and in the degree of toxicity (increases in the series; kalimin, galantamine, oxazil, proserin). The choice of ACEP depends on the individual sensitivity of patients.

Table 26. Dynamics of the clinical effect of anticholinesterase drugs

The intervals between doses are determined by the duration of action of the drug in each patient. The medication should be re-taken 30-60 minutes before the expected cessation of the previous dose. When replacing drugs, it should be taken into account that according to the effectiveness of 1 table. proserin, kalimine or oxazil corresponds to 1 ml of a 0.05% solution of proserin. The individual adequate daily dose averages 3-9 tablets. In some cases this dose has to be increased to 20 tablets. In any case, the use without prior testing of large doses or combinations of ACEP should be avoided due to the risk of cholinergic crisis.

Sensitivity to ACEP can vary significantly. This happens during pregnancy, during the menstrual period, with various concomitant infections, after thymectomy, the start of hormonal therapy, and during remission. Therefore, single and daily doses require constant adjustment. In case of an overdose of ACEP, miosis, hypersapivation, nausea, diarrhea, and frequent urination are observed. Muscle weakness increases, fasciculations appear, first in the muscles of the face, throat of the neck, then in the muscles of the shoulder girdle, external eye muscles and muscles of the pelvic girdle. Relative contraindications for the use of ACEP: bronchial asthma, angina pectoris, severe atherosclerosis, epilepsy. In case of an overdose of ACEP, anticholinergics are used, most often a 0.1% solution of atropine sulfate, 1 ml subcutaneously.

Potassium preparations improve acetylcholine synthesis and synaptic transmission, prolong the action of ACEP. They are indicated at all stages of treatment. In patients with local forms of the disease and in stable long-term remission, they are used as monotherapy, in other cases - as part of combination treatment. Prescribe potassium orotate (Dioron, Oronur) in tablets of 0.5 g 3 times a day; potassium chloride in powder or tablets 0.5-1 g or 1 g or 50 ml of 4% solution (10 ml of 10% solution) orally 2-3 times a day: spironolactone (veroshpiron, aldactone) in tablets 25 mg 3-4 times a day. Veroshpiron is contraindicated in mastopathy, gynecomastia, pregnancy, atrioventricular block and renal failure.

Thymectomy improves the course of myasthenia gravis, since the operation removes the source of formation of antibodies to acetylcholine receptors and pathologically activated lymphocytes. Thymectomy is currently considered crucial in the treatment of myasthenia gravis. The indication for thymectomy is the steady progression of the disease, especially in the case of a generalized form with impaired swallowing, speech and breathing. Preparation for surgery includes general restorative therapy, treatment of concomitant diseases, sometimes irradiation of the thymus gland, corticosteroid drugs, and plasmapheresis.

It is preferable to use short-acting barbiturates (hexenal, sodium thiopental or sodium hydroxybutyrate) as induction anesthesia, and nitrous oxide as the main anesthetic. The rate of improvement and remission after thymectomy reaches 70-90%, and improvement can occur within 5 years after surgery. It is not advisable to use thymectomy in case of stationary mild form, as well as in local ocular form of myasthenia. Contraindications to thymectomy are severe decompensated somatic diseases. The mortality rate for thymectomy was reduced to 0.8%.

Gamma or X-ray irradiation of the thymus produces a less lasting positive effect than thymectomy in 30-50% of cases. Radiation therapy is carried out in cases where thymectomy is contraindicated (senile age, incurable somatic diseases), with tolerance to drug therapy, before and after thymectomy to stabilize the condition of patients (usually 1-2 courses). This method is contraindicated for children and patients during puberty.

Corticosteroid drugs reduce the level of antibodies to acetylcholine receptors, reduce the manifestations of polymyositis and, apparently, improve neuromuscular conduction. The indication for their use is the lack of effectiveness of combined treatment of myasthenia gravis with other drugs, as well as the period of preparation of patients for thymectomy. In severe forms of myasthenia gravis, prednisolone is prescribed daily, and when significant improvement occurs, every other day, taking the entire daily dose on an empty stomach in the morning. If you cannot quickly switch to taking the drug every other day, you can prescribe unequal doses: for example, 100 mg on even days, 50 mg on odd days. The initial dose (60-150 mg per day) is gradually reduced as the condition improves (by 5 mg every week).

A maintenance dose (50 mg per day) can be prescribed for several years. Taking prednisolone every other day allows you to avoid side effects even with long-term treatment. Since when taking prednisolone, the content of antibodies to acetylcholine receptors decreases and the release of acetylcholine increases, it is advisable to slightly reduce the dose of ACEP before prescribing prednisolone in order to avoid a cholinergic crisis. In some cases, when starting treatment with prednisone, the condition may worsen, so hormonal therapy should be started in a hospital setting.

Side effects observed with prolonged therapy with prednisolone: ​​virile obesity, hirsutism, menstrual irregularities, Itsenko-Cushing syndrome, mental disorders. Ulceration of the mucous membrane of the digestive tract, perforation of an unrecognized ulcer, and disturbances in water and electrolyte balance are possible. To prevent the side effects of corticosteroids, antacids (Almagel), a diet low in sodium, salt, carbohydrates, and potassium supplements are prescribed.

Immunosuppressants reduce the level of antibodies to acetylcholine receptors and correct the reactions of cellular and humoral immunity. The indication for such treatment is the insufficient effectiveness of therapy for progressive myasthenia gravis by other means. Azathioprine (Gshuran) is prescribed in small doses (50 mg per day) at the beginning of treatment. Every week the dose is increased by 50 mg. The maximum daily dose is 2-3 mg/kg or an average of 100-200 mg per day. The effect is usually observed within 2-3 months in 79-80% of patients.

When the effect is achieved, the dose of the cytostatic is gradually reduced. Azathioprine is usually prescribed along with a maintenance dose of prednisolone. Side effects: thrombo-, leukopenia, hepatitis, pancreatitis, secondary infection (especially when azathioprine is combined with prednisolone), septicemia, etc. In the first weeks of azathioprine therapy, it is necessary to examine peripheral blood at least once every 3 days. When the number of leukocytes in the peripheral blood decreases to 3-3.5 * 109/L, azathioprine is discontinued.

Cyclophosphate for myasthenia gravis is prescribed at a dose of 1 mg/kg per day, then the dose is gradually increased to 2-3 mg/kg per day. until clinical improvement, after which the dose of the cytostatic is reduced. Side effects: dyspeptic and dysuric disorders, leukopenia, alopecia, dizziness, blurred vision. Cyclosporine inhibits the activity of T-helper and T-killer cells. It is prescribed in an average dose of 3-5 mg per day. The tactics for changing the dose are the same as for treatment with other cytostatics.

Clinical improvement occurs earlier than with azathioprine, but the incidence of side effects is 2 times higher than with azathioprine. Methotrexate is a highly toxic cytostatic. It is used only for severe forms of myasthenia gravis, if the combination of azathioprine with prednisolone is ineffective. The initial dose is 20 mg intramuscularly or intravenously 2 times a week, then the dose is increased to 40 mg 2 times a week. Course duration is 1-1.5 months.

Improvement in condition is observed in 50% of patients. Once the effect is achieved, it is advisable to switch to the less toxic azathioprine. Side effects: nausea, diarrhea, stomatitis, alopecia, ulcerative intestinal lesions, thrombocytopenia with hemorrhagic complications, toxic hepatitis, kidney damage.

Immunoglobulin with repeated intravenous drip infusions causes improvement in 70-90% of patients. It occurs on the 2-6th day after the start of treatment and lasts from 3 weeks to 3 months. This allows, during an exacerbation of myasthenia gravis, to gain the time necessary for the onset of the effect of other drugs. The degree of improvement is sometimes such that it is possible to reduce the dose of corticosteroid drugs by half. Immunoglobulin preparations are administered intravenously daily for 5 days or 3 times a week for 2-3 weeks. Side effects are rare: transient headache, swelling of the distal extremities. In 20-25% of cases, as with the beginning of treatment with prednisolone, a transient increase in muscle weakness is observed.

Plasmapheresis ensures the washout of toxic circulating immune complexes, eliminates excess AChE during a cholinergic crisis, and reduces the level of cholinesterase. Indications for plasmapheresis: exacerbation of myasthenia gravis, ineffectiveness of corticosteroid and immunosuppressive therapy, myasthenic or cholinergic crisis, preparation for thymectomy in myasthenia gravis, deterioration of condition after thymectomy. 3-5 sessions of plasmapheresis are performed, first every other day, and then once a week, with replacement of 1-2 liters of plasma per session depending on tolerance (up to 5-10 liters per course). The clinical effect appears after a few days, it is usually unstable and lasts several months. A complication of plasmapheresis is venous thrombosis.

Hemosorption is the extraction of blood from a vein, passing it through an adsorbent and infusion into the cubital vein. Usually, 1 session is carried out, in which 6-10 liters of blood are passed through the adsorbent. Subsequent sessions are ineffective.

Enterosorption is carried out by prescribing the fibrous salt adsorbent vaulen for oral administration at a dose of 50-60 mg/kg body weight 3 times a day (2 hours after meals and no earlier than 2 hours before the next meal) for 20 days.

In recent years, other methods of influencing the immune status of a patient with myasthenia gravis have been studied: the use of antilymphocyte and antithymic globulin, interferon, splenectomy, X-ray irradiation of the spleen, drainage of the thoracic duct.

Shtok V.N.

Myasthenia gravis (literally translated as “ muscle weakness") is a neuromuscular disease of an autoimmune nature.

Forms and symptoms of myasthenia gravis

1. 1 Ocular (drooping upper eyelid - in the morning a person opens his eyes normally and completely, but in the evening the upper eyelid begins to droop).
2. 2 Bulbar (impaired swallowing and chewing functions).
3. 3 Generalized (difficulty holding your head up, the presence of a transverse smile, deep wrinkles on the forehead, excessive salivation; then the muscles of the limbs weaken, and over time they completely atrophy).

Primary symptoms:

• excessive fatigue;
• skeletal muscle weakness;
• muscles weaken with intense movements and prolonged work;
• the muscle completely stops functioning, and after some time resumes its functions;
• the patient feels good in the morning, just waking up, a few hours later all the above symptoms appear.

Causes of myasthenia gravis:

1. 1 violation of the neuromuscular synapse at the biochemical level;
2. 2 autoimmune damage to the thymus (thymus gland) - the patient’s immune system mistakes its own tissues for foreign ones.

Useful foods for myasthenia gravis

Potassium is necessary for skin diseases and helps restore muscle function. Therefore, it is recommended to eat foods rich in potassium ions.

• fresh fruits (oranges, bananas, avocados, melon);
• fresh vegetables (legumes: beans, lima and lentils; potatoes, rutabaga, pumpkin);
• not crushed grain;
• dried apricots, raisins.

Also, with myasthenia gravis, it is extremely necessary to consume foods rich in calcium. He takes part in such processes as:

• muscle contractility and increases the excitability of nerve tissues;
• keeps bones and teeth strong, which prevents fractures due to injuries. After all, people with myasthenia gravis are more susceptible to injury than ordinary people.

Sources of calcium:

• main: dairy products;
• fresh vegetables: cabbage (cauliflower, white cabbage), turnip (its leaves), asparagus, lentils, legumes;
• nuts;
• egg yolks;
• soft sardine and salmon bones.

In order for calcium to be well absorbed in the body, it must be consumed with phosphorus (in a ratio of 1 to 2). Another very important property of phosphorus for myasthenia gravis: it helps preserve energy in the cells.

• cottage cheese;
• meat;
• brain;
• beef liver;
• fish;
• cereals: buckwheat, pearl barley, oatmeal;
• nuts (walnuts).

Traditional medicine for myasthenia gravis:

Recipe No. 1

Take 200 grams of oats, rinse well and add 0.5 liters of water. Boil for 40 minutes. Then leave for an hour and filter.

You need to take it for 3 months - 4 times a day, 0.5 cups and add a teaspoon of honey 30 minutes before meals. Then take a break for 3 weeks. After they expire, repeat taking the oat decoction (another 3 months).

Recipe No. 2

Take 3 heads of garlic, chop them, add 4 lemons to them (2 lemons with peel, 2 peeled). Add a glass of flax oil and 1 kilogram of honey to the resulting mixture. Mix all ingredients with a wooden spoon. Take this paste 3 times a day, 10 grams 30 minutes before meals.

Recipe No. 3

Take a glass of sugar and 200 grams of onion, add a liter of water, mix. Add 30-40 grams of honey and cook for 90 minutes on low heat. The prepared mixture should be taken three times a day, one tablespoon at a time.

All these recipes must be alternated for better treatment of myasthenia gravis.

Dangerous and harmful foods for myasthenia gravis

You should exclude foods that contain magnesium, as it relaxes muscles:

• plant products (spinach, broccoli, white beans, unprocessed grains, dried apricots, blackberries, raspberries, sesame seeds);
• products of animal origin (flounder fish, shrimp, carp, sea bass, mackerel, cod, dairy products).

Diuretic products are contraindicated; they wash away potassium and calcium, which is essential in the treatment of myasthenia gravis.

Table of contents of the topic "Myasthenia. Myasthenic syndromes.":

Basic directions of treatment of myasthenia and - improvement of neuromuscular transmission by the use of cholinesterase inhibitors (anticholinesterase drugs, AChEP), effects on the thymus gland and the immune system.

Cholinesterase inhibitors or anticholinesterase drugs - these are proserine, pyridostigmine bromide and oxazil. Prozerin is used subcutaneously in injections (ampoules of 1-2 ml of 0.05% solution) and orally in tablets of 0.015 g; pyridostigmine bromide (kalimin, mestinone) also subcutaneously (ampoules of 1 ml of 0.5% solution) and orally (tablets of 0.06 g); oxazil (ambenonium, methylase) - orally (0.005 g tablets).

Doses of drugs for myasthenia gravis are selected individually. It should be taken into account that the duration of action of proserin when taken orally is 2-4 hours, pyridostigmine bromide - 4-6 hours, oxazil - 6-8 hours. Taking one tablet of proserin is equivalent to taking one tablet of kalimine and two tablets of oxazil (respectively, 15, 60 and 10 mg). Proserin is also used as a mandatory diagnostic test. At the same time, cholinesterase inhibitors, especially prozerin, cause side effects (salivation, gastrointestinal discomfort, diarrhea, bronchorrhea, muscarinic effect).

For long-term treatment of myasthenia gravis As a rule, pyridostigmine bromide is used in an individual dosage - usually 60-120 mg 3-4 times a day. The drug is best used after eating a small amount of food (to reduce side effects on the gastrointestinal tract).

Effect of cholinesterase inhibitors in myasthenia gravis increases with the addition of potassium chloride or potassium-sparing agents. Potassium chloride is administered orally at 0.5-1.0 g/day (in milk, juice or water during meals). Blockade of the cardiac conduction system and impaired renal function are contraindications. The potassium-sparing drug of choice is spironolactone (veroshpiron, aldactone). The drug retains potassium in the cells of the body. Prescribed 0.025-0.05 g 3-4 times a day; it should not be used in the first trimester of pregnancy, with mastopathy and gynecomastia.

To others direction in the treatment of myasthenia gravis is the correction of immunological disorders. The most effective was the use of glucocorticoids. Currently, it is generally accepted to prescribe them in a higher dose every other day. It does not suppress the adrenal glands' own glucocorticoid function on days off these drugs. Doses: 1-1.5 mg/kg per day. The effect occurs on the 4-6th day of treatment. After achieving and consolidating a stable therapeutic effect, the dose is gradually reduced to 50% of the maximum. If the effect persists, you can gradually (by 5 mg per month) carefully reduce the dose. As a rule, it is possible to achieve a dose of 10-20 mg every other day, which lasts for many years. If deterioration occurs when the dose is reduced, the dose is slightly increased to compensate for the patient’s condition or other treatment methods are used.

In recent years it has been adopted pulse therapy for myasthenia gravis prednisolone at a dose of 30 mg/kg per intravenous administration. This method is indicated for severely ill patients, who are usually in intensive care or intensive care units, which in most cases leads to a pronounced improvement.

Side effects with glucocorticoid therapy for myasthenia gravis well known: inflammatory and ulcerative changes in the stomach and duodenum, autonomic disorders, hyperglycemia (possible steroid diabetes), spinal osteoporosis (possible hormonal spondylopathy), Cushingoid.