Medicines for myasthenia gravis for blood vessels. What antibiotics are available for myasthenia gravis? Artificial ventilation

A neuromuscular disease with an autoimmune nature of development is myasthenia gravis - from the Latin Myasthenia Gravis. Ten people out of 100,000 suffer from this disease. More than 50% of patients achieve remission.

Some are predisposed to the occurrence of myasthenia gravis - a risk group. It includes:

Young people aged 20 - 40 years;
Female gender - according to statistics, women get sick 3 times more often than men, but in adulthood these figures become equal.

The main reason for the development of myasthenia gravis is the body’s autoimmune reaction to its own tissues. This process can be started:

Genes;
Stress;
ARVI.

Depending on the provoking factor, myasthenia gravis is divided into 2 large classes:

Congenital;
Acquired.

The first type is the outcome of a genetic mutation. A failure leads to a violation in contractile function muscles. The second form is often a consequence of a thymus tumor. Myasthenia gravis can affect muscle fibers any part of the body. The ocular form of the disease is more common. Children rarely suffer from this disease. They make up less than 3% of the total number of patients.

Clinical picture

Regardless of which muscle group the disease affects, patients will notice general symptoms in the affected area:

Excessive fatigue;
Increased weakness;
Decreased performance and functionality.

The pathological focus causes discomfort to a person. This defective part cannot cope with the tasks assigned to it. Muscles in any part of the body can suffer. But the eyes are especially often involved in the process. This brings a lot of inconvenience to the patient. However, resting the muscles, relieving tension from them, brings relief. But it doesn't last long.

Over time, even prolonged relaxation will not reduce the severity of the patient’s symptoms. Progress in the field of pharmacology allows patients to fight the disease. IN clinical practice The following forms of myasthenia are distinguished:

Lightweight;
Heavy.

Diagnostics

In order to record the fact of the disease, symptoms alone are not enough for a doctor. Any study of a pathological process is not limited to instrumental methods. The doctor examines the patient, finds out the clinical signs of the disease. Reviews the patient's medical history. And then assigns him laboratory and instrumental diagnostics. The standard set of tests to detect myasthenia gravis consists of the following:

Functional tests aimed at detecting pathological muscle fatigue;
Electromyographic study, which indicates activity in the affected areas;
Decrement test, which allows you to identify the blockade of the neuromuscular signal, and at the same time the severity of the process;
Proserine test;
Biochemical blood test;
Immunograms;

If it is difficult to make a diagnosis, it may be prescribed differential study through:

Needle electromyography;
Studying the conductive functions of nerve fibers;
Electromyography of certain muscles – jitter.

Treatment

The choice of therapy for myasthenia gravis depends on many factors:

Forms of the disease;
Patient's condition;
Concomitant pathologies;
Prevalence of the process.

Drug treatment is usually prescribed. However, in some cases, surgery is recommended. Autoimmune processes in the thymus can cause thymectomy surgery. Among the effective medicines, “Prozerin”, “Kalinin” and drugs with a high potassium content are known. They also prescribe medications that stimulate the immune system.

TO symptomatic treatment, which alleviates the patient’s condition, include the following medications:

Anticholinesterase - "Ipigrix";
Cytostatics;
Glucocorticoids – “Prednisolone”, “Metypred”;
Immunoglobulins.

In case of rapidly progressing changes, extracorporeal hemocorrection is prescribed - a method that helps cleanse the patient’s blood of antibodies against his own tissues. Even the first procedure gives people a chance to feel improvement. Further therapy helps to achieve a lasting effect.

An effective method is cryophoresis. This procedure allows you to cleanse the blood of harmful substances, influencing low temperature. This treatment is carried out over a course of 5–7 days in a row. The method of cascade plasma filtration has become widespread. This procedure is carried out using nano cleaners. They purify the blood and then return it to the patient.

One more in a modern way Treatment of myasthenia gravis can be considered extracorporeal immunopharmacotherapy. It involves the extraction of lymphocytes from the patient, their drug treatment and subsequent release into the blood system. Using a similar technique, it was possible to induce stable remission in patients for a year. It is especially important to adhere to the doctor’s recommendations, since for myasthenia gravis there are contraindicated drugs, the use of which is fraught with dangerous consequences.

Eye shape

One of the most common types of disease is orbital. Often, it is from this that the process of myasthenia gravis begins, and then spreads to other organs. The main symptoms noted by patients:

Diplopia, that is, double vision. Patients see more than one holistic image;
Decreased visual acuity and clarity;
Violation in rotational and motor functions orbits;
Ptosis, that is, drooping eyelids. As a result, the palpebral fissure is unable to open and close normally.

All the described signs can apply to either one or both orbits. Usually, by closing their eyes for a short time, patients experience relief. However, mild tension associated with reading or watching TV causes discomfort.

Bulbar form

This type of myasthenia gravis can become life-threatening for the patient. It entails:

Dysphonia is a disorder of voice function;
Dysphagia – difficulty swallowing;
Dysarthria is disorganization in the functioning of the muscular apparatus of the pharynx, larynx, and soft palate.

Described symptomatic manifestations entail dangerous consequences. Dysphagia can progress to a complete inability to swallow. The list of food products for such patients is extremely scarce. The doctor prescribes the food. Patients have to be fed through a tube, they lose weight and become weaker. This means that their general condition worsens, which does not contribute to recovery.

Voice disorder reduces the social sphere of patients’ lives. And dysatria can cause death due to respiratory problems caused by paresis of the vocal cords that cover the larynx. This is fraught with asphyxia - suffocation.

Generalized form

The most unfavorable type of disease is systemic, that is, widespread. This dangerous type of myasthenia gravis invariably causes up to 1% of deaths among patients with this pathological process. The generalized form is captivating a large number of muscles, including respiratory ones - this can cause failure and death if assistance is not provided.

This disease is often accompanied by the prevalence of the process. Over time, the limited form progresses to the systemic form. And although remissions are not uncommon in patients, they usually arise and end suddenly. Therefore, myasthenic episodes and conditions are distinguished.

The first ones start and end quickly. The latter represent a long-term ongoing process, up to several years. However, this myasthenic condition is not prone to progression.

Contraindications

Patients suffering from this disease are forced to have some restrictions. These include:

Excessive physical activity;
Insolation, that is, exposure to direct sunlight;
Medicines with magnesium - “Magnesia” and “Panangin”, “Asparkam”;
Muscle relaxants are curare-like;
Neuroleptics, tranquilizers and drugs that enhance their effect - “Gidazepam”, “Corvalcaps”;
Diuretics, with the exception of Veroshpiron and Spironolactones;
The use of certain antibiotics such as aminoglycosides - Gentamicin and Streptomycin, fluoroquinolones - Enoxacin and Ciprofloxacin;
Vaccinations.

Contraindicated drugs for myasthenia gravis should not be ignored. There are tables and lists of medications that answer the question “What antibiotics can be used for myasthenia gravis?” You should avoid drugs that have this disease in the list of complications. Such medications include “Glutalit”. This means that the use of these tablets is a contraindication. Compliance with the doctor’s instructions is the key to a favorable course of the disease.

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

Forms and symptoms of myasthenia gravis

1 Ophthalmic (lowered upper eyelid- in the morning a person opens his eyes normally and completely, but in the evening the upper eyelid begins to droop).
2 Bulbar (impaired swallowing and chewing functions).
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 violation of the neuromuscular synapse at the biochemical level;
2 autoimmune damage to the thymus ( thymus gland) - the patient’s immune system mistakes its 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 are for better treatment myasthenia gravis must be alternated.

Dangerous and harmful foods for myasthenia gravis

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

products plant origin(spinach, broccoli, white beans, unprocessed grains, dried apricots, blackberries, raspberries, sesame);
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.

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.

Causes

There is no consensus on the causes of this disease. IN different time 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 form of 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 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 exercise sometimes lead to severe weakness.

The malignant type of the disease occurs only in the presence of a thymus tumor, often malignant. The disease debuts at about 50 years of age and within short term(months, and sometimes even weeks), leads to complete disability. On 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 develops more often against the background of menstruation, psycho-emotional stress, fever or other reasons. Insufficient dosage of drugs 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 muscles, 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 are also used and additional methods research:

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

Treating myasthenia gravis is not an easy 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.

Often resort to hormone therapy, especially when acute development 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 and special physical therapy exercises, massage.

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.

The choice of drugs for myasthenia gravis depends on the degree of damage muscle tissue and the magnitude of the disruption of synaptic conduction. Changes in ion exchange associated with potassium deficiency also require a special approach to the selection of drugs for therapy. Apply radical methods treatment - surgery or irradiation of the thymus gland.

Modern aspects of treatment

Autoimmune pathological process accompanied by paresis and paralysis. Modern drugs for myasthenia gravis allow patients to maintain their ability to work, avoid disability, and improve their quality of life. Assign effective treatment Electromyography data, a pharmacological test using anticholinesterase drugs, and a blood serum test for the presence of autoantibodies help.

What painkillers are available for myasthenia gravis is determined by the doctor individually depending on the stage of the disease. The following drugs are prescribed for treatment:

Fortalgin;
Voltaren;
Ibuprofen;
Ketoprofen;
Spasmalgon;
Coldrex;
Tempalgin.

Psychotropic substances - Aminazine, Amitriptyline - can aggravate the course of myasthenia gravis. By safe means are benzodiazepine derivatives and the drug Sonapax.

In patients with a generalized form of myasthenia, Chlorophyll is used to treat concomitant diseases of the nasopharynx. Antiseptic therapy ensures the saturation of infected tissues with oxygen. The general condition of the patient is improved by Actovegin, which dilates the coronary vessels and improves function nervous system. If the patient suffers from heart failure, medications are used to maintain normal operation important organ:

Preductal;
Mildronate.

Immunosuppressive therapy

Immunosuppressants are prescribed for:

Cyclosporine;
Prednisolone.

However, during therapy the risk of developing infectious complications and the development of malignant tumors.

Azathioprine is the most safe drug. It affects the absorption of glucocorticoids and can significantly reduce their dose. Side effects of the drug lead to its withdrawal. The patient complains of headache, chills, and fever. A person develops symptoms of liver dysfunction.

Methotrexate is a strong immunosuppressant, it is used in a small dose, because the drug has significant toxicity. The patient experiences discomfort in the epigastric region, nausea, and vomiting. Many people experience pain in the liver area, enzyme activity changes, and signs of cirrhosis appear.

Leucovorin, prescribed after Methotrexate therapy, reduces its toxicity. For a patient suffering from myasthenia gravis, treatment with antipsychotics and tranquilizers is contraindicated.

Medicines to reduce muscle tone

In some cases, non-depolarizing muscle relaxants are used for myasthenia gravis:

Tracrium;
Esmeron.

Considering the contraindications, the doctor tries not to prescribe muscle-relaxing drugs to treat patients, since many patients have increased sensitivity to their effects.

Non-depolarizing muscle relaxants are not used, since in many cases the patient develops an unpredictable reaction to their administration. The drug Succinylcholine causes a marked increase in the level of potassium in the blood serum and high temperature in the patient.

Patients suffering from periodic paralysis experience attacks accompanied by muscle weakness. During surgery to remove the thymus gland, the doctor does not use decompensated muscle relaxants. Sodium thiopental provides complete anesthesia.

The use of tablets for myasthenia gravis, which have a muscle relaxant effect, is prohibited for all categories of patients. Mydocalm, Sirdalud, Meprotan are especially dangerous to the patient’s health. The use of muscle relaxants in patients with initial form illness causes respiratory arrest.

Glucocorticoid therapy

Prednisolone increases the number of cholinergic receptors. After taking it, muscle strength increases. To avoid certain risks in the early stages of the disease, therapy is carried out in a hospital setting. The patient is additionally prescribed anticholinesterase drugs. Treatment with glucocorticoids is long-term. The intermittent method, where the patient takes an increased dose of medication over several hours, is very popular. Side effects may occur during treatment:

promotion blood pressure;
stomach ulcer.

Azathioprine is used in patients with myasthenia gravis that is difficult to treat with Prednisolone. Dexamethasone is recommended by the doctor, taking into account the patient’s condition, since the drug is 10 times more active than other glucocorticoids. However, it is unsuitable for circadian therapy because it worsens the patient's condition.

Treatment with glucocorticoids involves taking alkalizing drugs: the patient is prescribed Phosphalugel or Ranitidine. To prevent the development diabetes mellitus the patient must comply special diet. Limit consumption of foods containing large amounts of carbohydrates. Blood is drawn regularly to determine glucose levels.

Cholinesterase inhibitors

In mild cases of the disease, the patient is prescribed medications that prevent the decrease in acetylcholine in the area of the neuromuscular nodes. The use of Proserin in the treatment of patients with myasthenia gravis provides active muscle stimulation, but large doses of the drug cause disruption of muscle conduction.

Diclofenac sodium is used to carry out therapeutic blockade with nerve damage and intense pain. It is the drug of choice, since procedures using Novocaine and Lidocaine are prohibited in patients suffering from myasthenia gravis.

Axamon (Ipidacrine) is used for diseases of the peripheral nervous system. The medicine is well tolerated by patients. The drug has a double effect, while Proserin, Oksazil and Kalimin act only on the peripheral nervous system.

Patients are prescribed medications containing potassium. For therapy, KCL is used in powder form. Considering its side effect on the gastric mucosa, it is taken after meals with milk. Medicines Potassium-Normin and Kalipoz are intended for oral administration several times a day.

The following medications containing magnesium and potassium should not be prescribed to the patient:

Panangin;
Asparkam.

Pathogenetic impact

When treating myasthenia gravis, the doctor performs pulse therapy using Methylprednisolone and certain treatment regimens. Corticosteroids are prescribed in a therapeutic dose daily or every other day. The course of treatment lasts a week, and then the doctor reduces the dose of the drug.

If the patient's condition worsens, a stepwise therapy regimen is used, based on increasing the single dose until the maximum allowable amount of medication is reached at one time. Metypred is a drug with high mineralocorticoid activity, therefore it is often used for treatment and stabilizes the patient’s condition.

Use of immunoglobulins

For treatment (MG), infusions of immunoglobulins (IVIG) obtained from donor blood are prescribed. The purpose of the method is to increase the patient’s body’s defenses. Functional changes in patients with MG reach significant values. Immunoglobulin administered to the patient does not cause serious side effects. The following drugs are used in the treatment of patients:

Gamimun-N;
Pentaglobin;
Octagam 10%;
Intraglobin.

During a crisis, immunoglobulins are prescribed only after emergency resuscitation measures. Human specific protein prevents the development of severe complications. It is administered every other day at the dose prescribed by the doctor.

Often, patients with myasthenia gravis complain of nausea and headache after infusion. The doctor evaluates the functioning of the patient’s immune system and notes the number of T cells. During the study, defects in immune particles are detected, and in the serum - increased activity thymic humoral factors.

The concentration of immunoglobulins reflects the state of internal organs that affect the body's defenses. Normal human protein containing a special fraction, administered in a standard dose for the first time, causes the appearance of flu-like symptoms:

heartbeat;
drowsiness;
convulsions;
high temperature.

There is another serious problem - it is necessary to constantly monitor the patient’s condition; in case of collapse and increased blood pressure, discontinue treatment, administer intravenous plasma solution and antihistamines.

In the treatment of myasthenia gravis, cytostatics are used:

Cyclophosphamide;
Cyclosporine;
Cyclophosphamide;

Often, after achieving the effect, the dose of the medicine is reduced. Taking Cyclophosphamide causes side effects:

leukopenia;
hepatitis A;
inflammation of the pancreas;
septicemia;
intestinal disorders;
dizziness,
visual impairment.

Harmful drugs

The following drugs are contraindicated for patients with myasthenia gravis:

anticonvulsants;
antibiotics (aminoglycosides);
B-blockers;
Lithium carbonate;
Procainamide hydrochloride;
Trihexyphenidyl hydrochloride;
antimalarial and antirheumatic drugs;
eye drops;
hypoglycemic drugs.

Illicit drugs contribute to the development of myasthenic symptoms and increase skeletal muscle weakness. Antibacterial medications worsen the symptoms of the disease. The following drugs are not recommended for use:

Ampicillin;
Imipenem;
Erythromycin.

Sleeping pills are contraindicated for myasthenia gravis. Treatment with benzodiazepine derivatives and barbiturates is unacceptable. Medicines containing magnesium significantly worsen the patient's condition. You should not take diuretics that affect the conduction of neuromuscular impulses.

The patient must take the medications prescribed by the doctor in courses, monitor his well-being and lead a healthy lifestyle.

Causes

Some are predisposed to the occurrence of myasthenia gravis - a risk group. It includes:

Young people aged 20 - 40 years;
Female gender - according to statistics, women get sick 3 times more often than men, but in adulthood these figures become equal.

The main reason for the development of myasthenia gravis is the body’s autoimmune reaction to its own tissues. This process can be started:

Depending on the provoking factor, myasthenia gravis is divided into 2 large classes:

Congenital;
Acquired.

The first type is the outcome of a genetic mutation. Failure leads to disruption of muscle contractile function. The second form is often a consequence of a thymus tumor. Myasthenia gravis can affect muscle fibers in any part of the body. The ocular form of the disease is more common. Children rarely suffer from this disease. They make up less than 3% of the total number of patients.

Clinical picture

Regardless of which muscle group the disease affects, patients will notice general symptoms in the affected area:

Excessive fatigue;
Increased weakness;
Decreased performance and functionality.

Over time, even prolonged relaxation will not reduce the severity of the patient’s symptoms. Progress in the field of pharmacology allows patients to fight the disease. In clinical practice, the following forms of myasthenia are distinguished:

Diagnostics

In order to record the fact of the disease, symptoms alone are not enough for a doctor. Any study of a pathological process is not limited to instrumental methods. The doctor examines the patient, finds out the clinical signs of the disease. Reviews the patient's medical history. And then he prescribes laboratory and instrumental diagnostics. The standard set of tests to detect myasthenia gravis consists of the following:

Functional tests aimed at detecting pathological muscle fatigue;
Electromyographic study, which indicates activity in the affected areas;
Decrement test, which allows you to identify the blockade of the neuromuscular signal, and at the same time the severity of the process;
Proserine test;
Biochemical blood test;
Immunograms;

If it is difficult to make a diagnosis, a differential study may be prescribed by:

Needle electromyography;
Studying the conductive functions of nerve fibers;
Electromyography of certain muscles – jitter.

Treatment

The choice of therapy for myasthenia gravis depends on many factors:

Forms of the disease;
Patient's condition;
Concomitant pathologies;
Prevalence of the process.

Drug treatment is usually prescribed. However, in some cases, surgery is recommended. Autoimmune processes in the thymus can cause thymectomy surgery. Among the effective medicines, Prozerin, Kalinin and drugs with a high potassium content are known. They also prescribe medications that stimulate the immune system.

Symptomatic treatment that alleviates the patient’s condition includes the following medications:

Anticholinesterase - "Ipigrix";
Cytostatics;
Glucocorticoids – “Prednisolone”, “Metypred”;
Immunoglobulins.

An effective method is cryophoresis. This procedure allows you to cleanse the blood of harmful substances, exerting an effect through lowered temperature. This treatment is carried out over a course of 5–7 days in a row. The method of cascade plasma filtration has become widespread. This procedure is carried out using nano cleaners. They purify the blood and then return it to the patient.

Another modern method of treating myasthenia gravis can be considered extracorporeal immunopharmacotherapy. It involves the extraction of lymphocytes from the patient, their drug treatment and subsequent release into the blood system. Using a similar technique, it was possible to induce stable remission in patients for a year. It is especially important to adhere to the doctor’s recommendations, since for myasthenia gravis there are contraindicated drugs, the use of which is fraught with dangerous consequences.

Eye shape

One of the most common types of disease is orbital. Often, it is from this that the process of myasthenia gravis begins, and then spreads to other organs. The main symptoms noted by patients:

Diplopia, that is, double vision. Patients see more than one holistic image;
Decreased visual acuity and clarity;
Impairment in the rotational and motor functions of the orbits;
Ptosis, that is, drooping eyelids. As a result, the palpebral fissure is unable to open and close normally.

Bulbar form

This type of myasthenia gravis can become life-threatening for the patient. It entails:

Dysphonia is a disorder of voice function;
Dysphagia – difficulty swallowing;
Dysarthria is disorganization in the functioning of the muscular apparatus of the pharynx, larynx, and soft palate.

The described symptomatic manifestations entail dangerous consequences. Dysphagia can progress to a complete inability to swallow. The list of food products for such patients is extremely scarce. The doctor prescribes the food. Patients have to be fed through a tube, they lose weight and become weaker. This means that their general condition worsens, which does not contribute to recovery.

Generalized form

The most unfavorable type of disease is systemic, that is, widespread. This dangerous type of myasthenia gravis invariably causes up to 1% of deaths among patients with this pathological process. The generalized form involves a large number of muscles, including the respiratory ones - this can cause failure and death if help is not provided.

The first ones start and end quickly. The latter represent a long-term ongoing process, up to several years. However, this myasthenic condition is not prone to progression.

Contraindications

Patients suffering from this disease are forced to have some restrictions. These include:

Excessive physical activity;
Insolation, that is, exposure to direct sunlight;
Medicines with magnesium - “Magnesia” and “Panangin”, “Asparkam”;
Muscle relaxants are curare-like;
Neuroleptics, tranquilizers and drugs that enhance their effect - “Gidazepam”, “Corvalcaps”;
Diuretics, with the exception of Veroshpiron and Spironolactones;
The use of certain antibiotics such as aminoglycosides - Gentamicin and Streptomycin, fluoroquinolones - Enoxacin and Ciprofloxacin;
Vaccinations.

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Principles of treatment of bronchopulmonary infection in patients with myasthenia gravis

Myasthenia gravis is a classic autoimmune disease, which is based on an autoimmune process directed against the acetylcholine receptor of the postsynaptic muscle membrane. The leading link in the development of myasthenia gravis is a violation of neuromuscular conduction, which is clinically expressed in the development of increasing muscle weakness various localizations. Currently, great strides have been made in studying the pathogenesis of the disease, in diagnosis, in approaches to the management and treatment of various groups of patients with myasthenia gravis. However, despite pathogenetic therapy There are no specific approaches to completely cure this disease. The outcome of the disease depends on the effectiveness of the selected therapy and the rate of progression of increasing muscle weakness, which can lead to complete inexcitability of certain muscle groups.

The most clinically significant in patients with myasthenia gravis is the involvement of the main and auxiliary respiratory muscles in the pathological process, which ultimately manifests itself in varying degrees respiratory failure. These changes lead to disruption of the ventilation function of the lungs, causing congestion, which are “favorable” for the development of the infectious-inflammatory process. A study conducted at Johns Hopkins University Hospital from 1990 to 1998 found that 46% of patients experiencing some form of respiratory distress associated with myasthenia gravis developed pneumonia.

The importance of the development of the infectious process is associated with the treatment carried out in patients with various forms of myasthenia gravis. In addition to the main therapy with anticholinesterase drugs, treatment of myasthenia gravis is aimed at suppressing the activity of the autoimmune process, which is carried out by suppressing the general immune response of the body. Treatment methods for myasthenia gravis include the use of pulse therapy with glucocorticosteroids, the use of cytotoxic immunosuppressants (azathioprine, cyclophosphamide), and thymectomy. The listed treatment methods in connection with immune suppression create an additional background for the development of bacterial complications. It is important to note that some drugs, such as Cyclophosphamide, have a direct toxic effect on lung tissue. Thus, the role of infection in myasthenia gravis is obvious.

The relevance of the problem of the development of bronchopulmonary diseases is also associated with the complexity of treating such complications in patients with myasthenia gravis. Some drugs that are used in standard practice in the treatment of concomitant diseases (curare-like drugs, D-penicillamine, interferon-alpha, magnesium salts, beta blockers, blockers calcium channels etc.), are contraindicated in myasthenia gravis due to their inhibitory effect on neuromuscular conduction. Drugs that are absolutely contraindicated for myasthenia gravis also include some antibiotics: aminoglycosides, macrolides, fluoroquinolones. This imposes certain restrictions on the treatment of infectious complications (IO) in these patients, as noted earlier, which so often manifest themselves in the respiratory system.

Thus, due to the complexity of treating IO in patients with myasthenia gravis, there is a need to search for new approaches in the treatment of this group of patients. Unfortunately, when preparing the materials, no English-language literature on this issue was found.

The purpose of this study was to study the microbiological landscape of sputum, tracheobronchial secretions and evaluate the clinical effectiveness of antibiotic and immunotherapy in patients with infectious and inflammatory diseases associated with myasthenia gravis.

Materials and research methods

The study group consisted of 19 patients with a generalized form of myasthenia (6 men, 13 women; patient age ranged from 22 to 81 years); of which 3 men, 7 women - with exacerbation of chronic tracheobronchitis, 3 men, 1 woman - with residual signs of hospital-acquired pneumonia; 2 women - with chronic obstructive bronchitis; 1 woman - with Lambert–Eaton syndrome; 2 women with bacterial focal pneumonia (one of them had a history of thymectomy). Clinical signs exacerbations of the infectious process were as follows: cough with mucopurulent sputum, an increase in its production, shortness of breath, fatigue, and some patients had low-grade fever. Before starting treatment, a standard clinical and laboratory study was carried out, a study of the bacteriological composition of sputum, secretions from the trachea (or tracheostomy), studies of external respiratory function (RPF), radiography or computed tomography (CT) of the chest.

Samples obtained from patients were delivered within 2 hours to the bacteriological laboratory, where smear microscopy was performed to assess the quality of the biomaterial and cultured on standard nutrient media. Sputum samples were considered acceptable if the white blood cell count was greater than 25 and the epithelial cell count was less than 10 per field of view. When inoculating the isolated pathogens, species identification was carried out (BBL Crystal test systems). The antibiotic sensitivity of microorganisms was determined by the agar diffusion method.

The severity of the condition and the difficulty of treating patients are due to immunodeficiency and iatrogenicity. Thus, one patient had tracheal stenosis, which developed as a result of prolonged artificial ventilation performed in connection with myasthenic crisis. Another patient had tongue cancer, and therefore the patient underwent an open tracheostomy. In these cases, the situation worsens: immunological protection is reduced, the evacuation of tracheobronchial secretions is disrupted, which contributes to the colonization of nosocomial resistant strains of microorganisms and the development of purulent-infectious complications. And in another patient, the severity of the condition was aggravated directly by taking the anticholinesterase drug pyridostigmine (Kalimin), prescribed for myasthenia gravis. While taking pyridostigmine, the patient's sputum production increased to 300 ml/day. In this regard, the patient was forced to stop taking the drug and independently carry out positional morning drainage.

The auscultatory picture was scanty: vesicular breathing, weakened in the lower lateral parts of the lungs, local moist and scattered dry rales, resting respiratory rate 18–20 per minute.

At FVD study ventilation problems were identified. The decrease in FEV1 averaged from 60% to 49%.

As antibiotic therapy, all patients received cefoperazone/sulbactam intravenously or intramuscularly at a dose of 1.0 g 2 times a day. The duration of therapy was 7–10 days (depending on the degree of activity of the infectious process). In order to improve the rheological properties of sputum, all patients were prescribed mucolytics (acetylcysteine (Fluimucil) 300 mg 2 times a day) through a nebulizer or per os.

Human intravenous immunoglobulin G (IVIG: Octagam, Biaven V.I., Octaglobin) was included in the immunoreplacement treatment program. Treatment was carried out against the background of basic basic therapy, including methylprednisolone, pyridostigmine and potassium chloride.

We think it is important to describe clinical case in this study. A 74-year-old patient was diagnosed with “generalized myasthenia gravis” since December 2010. Depending on the severity of the condition, he took 80 and 40 mg of methylprednisolone on an alternating daily regimen. He came in in August 2012 with complaints of severe weakness, fatigue, and increasing shortness of breath with little physical effort. An objective examination of the patient revealed a moderate condition, the skin was without visible pathology, the peripheral lymph nodes were not enlarged, and the feet were pasty. When percussing the chest, a box sound was detected, the excursion on both sides was 3 cm (1.5 + 1.5). On auscultation, breathing in the projection of segments S4–5, S9 on the right was sharply weakened, there were no wheezes, the number of respiratory movements while sitting was 18 per minute. Heart rate - 85 per minute, blood pressure - 130/85 mm Hg. Art., temperature 36.8 °C. Palpation of the abdomen is painless, the abdominal organs are not enlarged. results laboratory research are presented in table. 1. On the presented CT images of the chest organs on the left in the projection of segments S1–2, a cavity formation of irregular shape with uneven, somewhat infiltrated contours is determined against the background of limited pneumofibrosis (Fig. 1). On the left in the projection of segment S9 there is a large infiltrate of a heterogeneous structure (Fig. 2). A diagnostic bronchoscopy was performed: the bronchi were patent, the mucous membrane was pale pink and atrophic. Conclusion: chronic atrophic tracheobronchitis stage II.

So, the patient developed pneumonia with destruction against the background of myasthenia gravis. lung tissue. Etiological factor It was not possible to detect due to the lack of sputum. It is important to note that, against the background of long-term use of high doses of methylprednisolone, the patient developed signs of thrombocytopenia, manifested by skin bruises on the body, and a course of complex antibiotic and immunotherapy was started. Cefoperazone/sulbactam was administered intravenously for 10 days. At the same time, IVIG was administered, the course dose was 15.0 g. The therapy with the inclusion of IVIG allowed us to achieve a faster remission of the infectious-inflammatory process, confirmed by clinical and laboratory data and the results of repeated CT studies of the lungs, where positive dynamics of the pathological process were noted (Table 1) : increased platelet level - up to 131 × 109/l, decreased leukocytosis to 15.0 × 109/l, decreased level of C-reactive protein - up to 5.0 mg/l.

The patient was consulted by an endocrinologist: there were complaints of back pain when walking and physical activity, a decrease in height by 4 cm over 3 years, a feeling of fatigue in the back while sitting; determined upon examination thoracic kyphosis, results laboratory examination are presented in table. 2.

X-ray of the spine in the lateral projection revealed compression fractures of the 1st and 2nd lumbar vertebrae. Dual-energy X-ray absorptiometry: bone mineral density according to T-score in lumbar region spine - 3.0 SD, bone mineral density according to the T-criterion in the femoral neck - 2.0 SD.

Diagnosis: drug-induced (glucocorticoid) osteoporosis with compression fractures of the vertebral bodies. Recommended: balanced diet with increasing consumption of foods rich in calcium and vitamin D, maintaining adequate physical activity and performing exercises with a load corresponding to the state of health; alfacalcidol (Alfa D3-Teva) 0.75 mcg daily, ibandronic acid (Bonviva) 3.0 ml bolus once every 3 months.

Results and discussion

24 samples of sputum and bronchial secretions isolated from patients with bronchopulmonary diseases were studied. The main clinically significant microorganisms were: S. pneumoniae (33.4%), S. aureus (20.8%), S. pyogenes (12.5%) (Fig. 3). Non-fermentative gram-negative bacteria included P. aeruginosa strains (12.5%). In four samples of biomaterial, an increase in microbial association was observed: P. aeruginosa and Candida albicans fungi, and in another sample Kl. pneumoniae + S. pneumoniae. Of great interest is the study of the sensitivity of isolated pathogens to cefperazone/sulbactam. It was noted that all strains of microorganisms were included in the antimicrobial spectrum of action of cefoperazone/sulbactam; and only in one sample (P. aeruginosa + Candida albicans) the antibiotic showed weak activity against the isolated pathogen.

So, a clinical study of sputum in patients with exacerbation of chronic bronchitis and pneumonia against the background of myasthenia gravis revealed the heterogeneity of the microbiological landscape. The leading pathogens were gram-positive pathogens, such as S. pneumoniae, S. aureus, S. pyogenes (accounting for 66.7%). Cefoperazone/sulbactam showed high activity against these strains of microorganisms. At the same time, there was an increase in the gram-negative pathogens P. aeruginosa and Kl. pneumoniae (12.5% each, respectively), which were sensitive to cefoperazone/sulbactam. Some gram-negative microorganisms represented associations of P. aeruginosa with fungi of the genus Candida (4.2%), Kl. pneumoniae with S. pneumoniae (16.7%); in such cases, fungicidal therapy with fluconazole (Diflucan) was prescribed, which significantly improved the course of the disease.

Infectious process respiratory tract in this group of patients was torpid, despite adequate antimicrobial therapy. It is known that with myasthenia gravis there is a general depression of the immune system due to the inhibition of both specific and nonspecific protective factors, which requires correction of immune “breakdowns”.

It is important to note the basic properties of the antibiotic used. Cefoperazone/sulbactam showed activity against both gram-positive and gram-negative microorganisms, including P. aeruginosa. In addition, the antibiotic is stable to the action of beta-lactamases, thanks to the irreversible inhibitor - sulbactam, which synergistically increases the effectiveness of the antibiotic used (the minimum inhibitory concentration of cefoperazone against strains of microorganisms sensitive to it is reduced by up to 4 times). Good tolerance of the drug by patients is of great importance, i.e. the antibiotic does not affect neuromuscular conduction.

So, in addition to detoxification, antibacterial and other types of therapy, IVIG is currently widely used for various localizations of the infectious and inflammatory process. IVIG (Octagam, Biaven V.I., Pentaglobin, etc.) contain a repertoire of antibodies that can inactivate wide range antigens. Immunoglobulins are a factor of acquired immunity, which opsonizes infectious foci, contributes to the rapid elimination of the bacterial agent and its metabolites, activating a whole cascade of immune reactions.

Conclusion

Many years of experience allows the use of IVIG in complex therapy of bacterial infections of the respiratory tract. The course of the disease is significantly improved, the recovery time for patients is accelerated, and most importantly, the risk of colonization by resistant strains of microorganisms and, accordingly, the risk of a repeated episode of infection is reduced.

Literature

Sanadze A. G. Myasthenia and myasthenic syndromes. 2012, p. 252.
Shcherbakova N. I. Pathogenetic justifications for the strategy and tactics of treatment of myasthenia gravis (Abstract of doctoral dissertation). 2007, p. 3–50.
Varelas P. N., Chua H. C., Natterman J., Barmadia L., Zimmerman P., Yahia A., Ulatowski J., Bhardwaj A., Williams M. A., Hanley D. F. Ventilatory care in myasthenia gravis crisis: assessing the baseline adverse event rate. Crit Care Med. 2002, Dec; 30(12):2663–2668.
Sulkowski S., Sulkowska M. Alveolar cells in cyclophosphamide-induced lung injury. II. Pathogenesis of experimental endogenous lipid pneumonia // Histol Histopathol. 1999, Oct; 14(4):1145–1152.
Sanadze A. G., Sokolova V. I., Shcherbakova N. I., Nikiforuk N. M. Efficacy of minimal doses of immunoglobulin in the treatment of myasthenia gravis complicated by abscess pneumonia // Current issues of clinical transport medicine. 2001, vol. 6: p. 280–286.
Skeie G. O. et al. Guidelines for treatment of autoimmune neuromuscular transmission disorders // Journal of Neurology. 2010, 17: 893–902.
Serrano M. C., Rabinstein A. A. Causes and outcomes of acute neuromuscular respiratory failure // Arch Neurol. Sep. 2010, Vol. 67(No. 9): 1089–1092.
Latysheva E. A., Latysheva T. V. Use of intravenous immunoglobulins in intensive care // General resuscitation. 2012, VIII; 3:45–49.

V. I. Sokolova, Candidate of Medical Sciences A. G. Sanadze, Doctor of Medical Sciences, Professor D. A. Sychev1, Doctor of Medical Sciences, Professor M. B. Babarina, Candidate of Medical Sciences D. A. Zaikov

GBOU DPO RMAPO Ministry of Health of the Russian Federation, Moscow

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Generalized myasthenia gravis: causes of increased muscle fatigue

Myasthenia gravis is severe autoimmune pathology accompanied by progressive muscle weakness. In this case, only striated muscle fibers are affected. Cardiac and smooth muscles remain normal. If you detect the first symptoms of such a disease, you should immediately contact a specialist.

Severe weakness is the main symptom of myasthenia gravis

Reasons for development

This disease was first described by the British physician Thomas Willis in the second half of the 17th century. Currently, the exact reasons for its development are already known. Myasthenia gravis occurs when the body’s immune system begins to produce antibodies to its own acetylcholine receptors on the postsynoptic membrane of the neuromuscular junction. It is thanks to such synapses that nerve impulses are transmitted to striated muscles.

With myasthenia gravis, the activity of synapses transmitting nerve impulses to striated muscles

The triggering factor in the development of an autoimmune reaction can be any infectious disease of the upper respiratory tract, severe stress, as well as disruption of the nervous system.

Another cause of this disease may be biochemical changes in the functioning of neuromuscular synapses. This can occur against the background of disruption of the hypothalamus and thymus gland.

All the reasons described above contribute to the insufficient production or excessively rapid destruction of acetylcholine, due to which the transmission of a nerve impulse to a striated muscle cell occurs.

Disturbances in the activity of the thymus can lead to the development of myasthenia gravis.

Note. Striated muscle fibers differ from smooth and cardiac muscles in that a person controls their activity independently.

To date, it has been established that myasthenia gravis is not inherited. It is more often formed in young women (20-40 years old). The prevalence of this pathology is approximately 5 cases per 100,000 people.

Clinical picture

Currently, there are several types of this disease. Each of them manifests itself with its own symptoms. The main forms of pathology are:

bulbar myasthenia;
ocular myasthenia;
generalized myasthenia.

The mildest form of the disease is the eye. It is characterized by the following clinical manifestations:

Drooping of the eyelid on one side (this is the initial symptom, which is later characteristic of the second eyelid).
With active blinking, the eyelid begins to drop even lower.
As a result of the drooping of one of the eyelids, double vision occurs.

This course of the disease is quite rare. The more common form of myasthenia is the bulbar form. It will be characterized by the following clinical picture:

The patient gets tired quickly long-term use food.
At the same time, his voice becomes hoarse and nasal. Later it becomes difficult for him to pronounce the letters “s”, “r” and “sh”.
If food intake is not stopped in a timely manner, the person loses the ability to swallow, and his speech may become almost silent.

In bulbar and generalized forms of myasthenia, the patient has a number of characteristic external signs

Note. Patients with bulbar disorders in myasthenia gravis try to eat food at the peak of the drug effect.

The most common form of myasthenia gravis is the generalized form. This pathology implies the presence of the following symptoms:

The disease initially affects the oculomotor muscles and only then striated muscle fibers of other localizations are involved in the pathological process.
The patient becomes amicable.
It is difficult for him to keep his head straight all the time.
There is drooling from the mouth.
Later it becomes difficult for the patient to walk for a long time. Over time, this symptom gets worse. It becomes difficult for a person to even get up and take care of himself.
Over time, muscle wasting occurs, especially pronounced in the limbs.
The severity of tendon reflexes decreases.

Important! A feature of myasthenia gravis is the fact that after a sufficiently long rest or sleep, all these symptoms become less pronounced and the person feels much better.

During sleep and rest, acetylcholine reserves in synapses are restored and the patient feels better

Diagnostic features

If characteristic manifestations of myasthenia gravis occur, it is necessary to consult a neurologist. This doctor knows exactly what kind of disease this is and how to confirm or deny its presence. Diagnosis of myasthenia gravis includes the following steps:

Questioning the patient (not only the main complaints are clarified, but also all the circumstances that preceded the development of the pathology).
Clinical examination.
Conducting functional tests to determine the presence of rapid muscle fatigue (for example, the patient is asked to blink quickly).
Electromyographic study.
Carrying out a proserine test.
Repeated electromyographic study (carried out in order to clarify how the proserine test affected muscle activity).
Repeated clinical examination (it is carried out in order to determine the degree of influence of the proserine test on muscle activity).
Blood test for the presence of antibodies to acetylcholine receptors and titin.
Carrying out computed tomography thymus.

Diagnosis of myasthenia gravis involves the use of modern medical advances

After carrying out all these diagnostic measures, the doctor either establishes a diagnosis or refutes it.

Note. In some cases, fewer diagnostic measures are carried out, especially when there is severe clinical picture myasthenia gravis.

Treatment

If the diagnosis of myasthenia gravis has confirmed the presence of this serious disease, then its therapy should be started as early as possible. The speed of initiation of treatment for myasthenia gravis will determine its further course and degree of limitation. physical capabilities.

Eye shape

In mild cases, when the ocular form of the disease occurs, the use of the following drugs:

kalimin or proserin;
potassium chloride.

Important! The dosage selection of these drugs is most often carried out in a hospital setting. This is due to the fact that in case of overdose they can develop severe complications. At their initial manifestations, it is necessary to administer an antidote (in this case, atropine).

After selecting the required doses of kalimine or proserin, as well as potassium chloride, the patient is discharged from the hospital. Further treatment and the forecast will have a clear relationship. The more accurately a patient follows the specialist’s recommendations, the higher his quality of life and the lower the risk of developing major complications.

With myasthenia gravis, the patient has to take a large number of medications

Bulbar form

In the bulbar form, treatment of myasthenia gravis must be supplemented with glucocorticosteroids. These drugs have their side effects in the form of increased blood pressure and glucose metabolism disorders, but often only they save a person from severe muscle weakness.

The most commonly used tablets are Prednisolone or Methylprednisolone. They are taken every other day in the morning. The dose of such drugs is selected at the rate of 1 mg/1 kg of a person’s weight.

Note. The minimum dose of prednisolone that can have a therapeutic effect is considered to be 50 mg every other day. As a result, patients have to use at least 10 tablets at a time, which, naturally, often causes some difficulties.

Glucocorticosteroids should be taken in such dosages for at least 1-2 months. Subsequently, there is a gradual reduction in the dose of prednisolone. It will not be possible to completely cancel it. For a normal condition, patients have to take 10-20 mg of this drug every other day. At the same time, it is necessary to control those negative effects that occur when taking such medications systematically. The local physician will prescribe additional medications to the patient for these purposes.

Advice! If side effects develop while taking glucocorticosteroids, you should not adjust their dose yourself. Only a doctor can do this correctly.

If the use of glucocorticosteroids is contraindicated (for example, in old age), then the patient is recommended to use cytostatic drugs. Usually the initial remedy here is the drug "Azathioprine". If its effectiveness is insufficient, then stronger cytostatics are prescribed.

In case of a generalized form of the disease, it is important to remove the thymus gland in a timely manner.

Generalized form

The causes and symptoms of generalized myasthenia require more serious therapeutic measures. Within 1-2 years after diagnosis, the patient undergoes surgery to remove the thymus gland. Most often, the clinical effect of such manipulation appears after 1-12 months. After 1 year, doctors conduct a second full examination of the patient and clarify how great the benefit of the surgical intervention turned out to be.

In the future, the same drug therapy is carried out as for the ocular and bulbar form.

With a sharp increase in muscle weakness, the patient is prescribed the drug “Immunoglobulin” and plasmapheresis.

What not to do with myasthenia gravis?

Currently, some contraindications for myasthenia gravis are known. The main ones among them are the following:

Serious physical activity.
Taking medications containing magnesium.
Prolonged exposure to direct sunlight.
Taking curare-like muscle relaxants.
Use of diuretics (with the exception of Spironolactone).
Use of neuroleptics.
Use of tranquilizers (with the exception of Grandaxin drugs).
Taking most antibiotics from the group of fluoroquinolones and aminoglycosides.
The use of drugs that are derivatives of quinine.
Use of corticosteroids that contain fluoride.
Taking the drug "D-penicillamine".

In addition, the patient will have to follow a certain diet. Proper nutrition with myasthenia gravis, practically excludes foods rich in magnesium (flounder, sea bass, shrimp, perch, cod, mackerel, dairy products, white beans, spinach, unprocessed cereals, broccoli, blackberries, sesame, raspberries, dried apricots and others).

Myasthenia gravis is not a contraindication to pregnancy

Myasthenia gravis and pregnancy are not mutually exclusive concepts. Modern achievements medicine allows a woman with this disease to bear a full-fledged baby without harming her own health. If there are no obstetric indications, then in such patients delivery is carried out naturally. If available, it is carried out C-section. In this case, anesthesia for myasthenia gravis in a pregnant woman should be epidural. Such anesthesia will be safer. General anesthesia in patients with myasthenia gravis is attempted only when the thymus gland is removed. Breastfeeding is contraindicated in patients with myasthenia gravis.

Important! Neglecting these contraindications can lead to aggravation of the disease.

Myasthenia gravis requires constant monitoring by specialists and strict implementation of all their recommendations. Only this approach will allow the patient to make his life as fulfilling as possible.

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Myasthenia gravis - what is this disease?

Myasthenia gravis is one of the autoimmune chronic diseases. It is characterized by decreased muscle tone and rapid fatigue. The ICD 10 code for this disease is G70, it is in the same group with various disorders of the neuromuscular fibers.

This condition was first described in the 17th century, and in Lately the number of people facing it is growing. Myasthenia gravis, as this disease is fully called, is more common in women, and develops at the age of 20-30 years; it occurs very rarely in children. If the disease is not treated, it gradually progresses, which over time can lead to the death of the patient.

Causes of the disease

Myasthenia gravis is a poorly understood disease. It is believed that it is genetic in nature. The work of the gene responsible for the functioning of neuromuscular fibers is disrupted. Normally, these connections, called synapses, transmit impulses from nerves to muscles, causing them to contract. This process is regulated by various biochemical reactions involving the mediator acetylcholine and the enzyme chylinesterase.

The pathogenesis of the disease is complex: in some cases, the functioning of the thymus and hypothalamus, which are responsible for the synthesis of these substances, is disrupted. If too little acetylcholine or too much cholinesterase is produced, nerve impulses are blocked and the muscle cannot perform its functions.

The causes of this disorder are malfunctions of the immune system, when the body produces antibodies to its own cells, destroying acetylcholine. This can happen after a cold or infectious disease, severe stress, hormonal imbalance or weakening of the body due to overwork.

Signs of the disease

The main symptom of the disease is increased muscle fatigue. During physical work, especially with repetitive movements, muscle weakness gradually increases, which over time can lead to paresis or paralysis. But after rest, these symptoms of myasthenia gravis disappear, and in the morning patients feel completely fine for several hours. At various stages and forms of the disease, the following signs appear:

double vision;
ptosis - drooping upper eyelid;
salivation;
voice change;
impaired chewing function, fatigue when eating solid foods;
choking when eating;
difficulty breathing;
poor facial expressions;
change in gait;
weakness of the muscles of the limbs and neck;
dry skin.

One of the first signs of myasthenia gravis is ptosis - drooping of the upper eyelid.

Forms of myasthenia

This disease develops differently in everyone. Most often, myasthenia gravis begins with weakness of the eye and facial muscles, then this disorder spreads to the muscles of the neck and torso. But some people have only some signs of the disease. Accordingly, there are several types of myasthenia gravis.

The ocular form is characterized by damage to the cranial nerves. The first sign of this is drooping of the upper eyelid, most often on one side first. The patient complains of double vision and difficulty moving the eyeballs.
The bulbar form of myasthenia gravis is a lesion of the masticatory and swallowing muscles. In addition to the disruption of these functions, the patient’s speech changes, the voice becomes quiet, nasal, and there are difficulties in pronouncing certain sounds, for example, “r” or “b”.
But most often there is a generalized form of the disease, in which the eye muscles are first affected, then the process spreads to the neck, upper and lower limbs. The hips and arm muscles are especially often affected; it is difficult for the patient to climb stairs or hold objects. The danger of this form of the disease is that weakness spreads to the respiratory muscles.

It is very important to make a correct diagnosis in time to begin treatment

How is the disease determined?

In addition to the patient’s complaints and medical history, the diagnosis of myasthenia gravis includes various tests, hardware examinations and analyses.

Electromyography helps determine muscle response to stress, and CT or MRI reveals the absence of diseases that could cause similar symptoms. After all, myasthenic syndrome is observed in encephalitis, meningitis, cancer, botulism, and thyrotoxicosis. But the differences in this case from myasthenia gravis are that the facial muscles are rarely affected, and when conducting electromyography, there is not a slowdown, but an increase in muscle potential with repeated stimulation.

It is also necessary to distinguish myasthenia gravis from Duchenne muscular dystrophy in time, although the symptoms are different. Muscular dystrophy occurs mainly in boys and begins in childhood.

Most often, to make a diagnosis they do immunological test to determine antibodies to acetylcholine and perform a proserine test. Its meaning is that after intramuscular injection of 1 ml of the drug “Proserina”, the patient’s condition improves significantly after 30 minutes, and after 2-3 hours the symptoms return.

A very important method for diagnosing myasthenia gravis is various tests that reveal the degree of muscle weakness, as well as which of them are more affected. Since fatigue increases after repetitive movements, the following tests may be effective:

if you ask the patient to look to the side or up for at least 30 seconds, ptosis and double vision appear;
to provoke dysarthria and decreased voice strength, you need to ask the patient to read something out loud;
weakness of the neck muscles can be detected if the patient lies on his back and raises his head, he will not be able to hold it up for more than a minute;
sometimes with myasthenia gravis the M. Volker phenomenon appears - repeated clenching and unclenching of the hands causes increased ptosis.

Electromyography helps determine muscle response to stress

Crises during illness

Myasthenia gravis is a chronic disease, it constantly progresses. If the patient does not receive proper treatment, his condition worsens. A severe form of the disease may be accompanied by the onset of myasthenic crisis. It is characterized by the fact that the patient experiences severe weakness of the muscles responsible for swallowing and movement of the diaphragm. Because of this, his breathing is difficult, his heart rate increases, and drooling is often observed. Due to paralysis of the respiratory muscles, the patient's death may occur.

An overdose of anticholinesterase drugs may cause a cholinergic crisis. It is expressed in a slow heartbeat, salivation, convulsions, and increased intestinal motility. This condition also threatens the patient’s life, so he needs medical attention. The anticholinesterase drug must be discontinued, and its antidote, Atropine solution, must be administered intramuscularly.

How to treat the disease

Myasthenia is serious illness, which requires constant medical supervision and treatment. It often leads to disability of the patient. But proper treatment of myasthenia gravis helps to achieve long-term remission. The main direction of therapy is the use of drugs that block cholinesterase. The most effective for myasthenia gravis are “Kalimin”, “Oxazil”, “Proserin”, “Galantamine”, “Ambenonium”. They need to be applied long time, alternating different means.

Potassium preparations enhance the effect of these drugs and improve muscle contractility, so they are also prescribed, for example, “Potassium-normine” or “Potassium chloride”. The diuretic “Veroshpiron” also helps to enhance the effect of anticholinesterase drugs.

Generalized myasthenia gravis requires more serious treatment. At severe course hormonal agents are used. Most often this is Prednisolone or a drug based on it, Metipred. Usually prescribed 12-16 tablets per day in the morning every other day. If a stable remission is observed after a couple of months, the dose is gradually reduced.

Recently, myasthenia gravis has also been treated with pulse therapy. This method involves administering high doses of Metipred for 3-5 days. If remission is achieved, the drug is gradually discontinued. But sometimes you have to take it for a long time, often for several years, 60 mg every other day.

With myasthenia gravis, it is very important to constantly take the medications prescribed by your doctor.

Cytostatic immunosuppressants are also used to treat such patients. "Azathioprine" is needed to block immunoglobulins and reduce the concentration of antibodies to acetylcholine. Cyclophosphamide and Methotrexate inhibit their activity. This therapy is indicated for patients for whom glucocorticoids are contraindicated. But sometimes these medications are alternated.

As an auxiliary therapy, agents can be used to improve metabolism in muscle tissue. These are calcium preparations, Ephedrine, Methionine, glutamic acid, tocopherol acetate, B vitamins. Treatment folk remedies not applicable, since many herbal preparations contraindicated for patients. Acceptable tonic plants: ginseng, lemongrass and others on the recommendation of a doctor.

Myasthenic crisis necessarily requires hospitalization of the patient in the neurology department. There he undergoes plasmapheresis to cleanse the blood of antibodies and artificial ventilation. To improve the condition more quickly, Proserin, Ephedrine and immunoglobulins are administered.

In the absence of effect from conservative therapy and the progressive course of the disease, it is used surgery. Thymectomy is the removal of the thymus gland. 70% of patients experience improvement after surgery. But the difficulty is that anesthesia for this disease has its own peculiarities.

Many drugs are contraindicated in such patients, especially muscle relaxants and tranquilizers, as well as morphine and benzodiazepines. Therefore, the operation is performed when the patient’s condition is serious and only in patients under 70 years of age.

It is very important for patients with myasthenia gravis to be careful about the medications they take, since many of them are contraindicated for them

Rules of conduct for myasthenia gravis

If the diagnosis is made on time and the patient follows all the doctor’s instructions, his performance and lifestyle remain almost unchanged. Treatment of myasthenia gravis consists of constantly taking special medications and following certain rules.

It is forbidden for such patients to sunbathe or perform heavy physical work and drink medications without a doctor's recommendation. Patients should definitely know what contraindications for taking medications for myasthenia gravis. Many drugs can cause complications of the disease or the development of myasthenic crisis. These are the following medications:

all magnesium and lithium preparations;
muscle relaxants, especially curare-like ones;
tranquilizers, antipsychotics, barbiturates and benzodiazepines;
many antibiotics, for example, Neomycin, Gentamicin, Norfloxacin, Penicillin, Tetracycline and others;
all diuretics, except Veroshpiron;
Lidocaine, Quinine, oral contraceptives, antacids, some hormones.

Is it possible to cure myasthenia gravis?

Previously, this disease was considered incurable, 30% of cases were fatal. Now the prognosis for myasthenia gravis is more favorable. With the right approach to therapy, more than 80% of patients experience stable remission. They can continue normal life and even remain functional. Permanent disability or even death of the patient can occur if the patient does not consult a doctor in a timely manner or if the prescribed treatment is not followed.

Many people have never even heard of a disease such as myasthenia gravis. Therefore, they do not always seek medical help on time. This is dangerous as it can cause complications. But proper treatment and compliance with all doctor’s recommendations will help the patient lead a normal life.

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