Congenital or acquired myasthenia gravis

Myasthenia gravis is a disease in which a person develops a disorder in the transmission of nerve impulses, leading to weakness of the striated muscles.

Depending on the type of disease, the location of the affected areas is different. Weakening muscles affect the functioning of organs. For example, a patient may have constantly half-closed eyelids, difficulty urinating, and have difficulty chewing and walking. All this is associated with dysfunction of the neuromuscular system. As a result, the disease can lead to disability and even death. The disease is not widespread. The quality of a person's life deteriorates significantly.

Women suffer from myasthenia in 2/3 of cases. Most patients are young people. But symptoms can also be observed in children, adolescents, and the elderly. Myasthenia gravis can be congenital or acquired. It can manifest itself as a symptom of another disease, including cancer.

The disease affects not only people, but also animals, including human pets - cats and dogs.

General information

What kind of disease is this and what is this strange prefix gravis? In ancient Greek, the name of the disease reads as myasthenia gravis, which literally means muscle weakness. In order to save vocabulary or time, or perhaps for some other reason, the second word was omitted.

Autoimmune - This type of disease means that immune cells confuse their cells with an infection and begin to attack them.

Myasthenia gravis is a fairly common autoimmune disease that is characterized by decreased muscle tone and excessive fatigue, which occurs quite quickly. Despite its prevalence, the disease has not been fully studied.

There is an opinion that the disease is associated with a hereditary predisposition, however, it has not been proven. Well, if there is an opinion about heredity, then there are other options for the development of the disease, which are described in more detail in the causes section.

Myasthenia gravis affects adults aged 30–35 years, but there is evidence of the disease at younger ages (20 to 30 years). In addition, in medical practice there are cases of the disease in children aged 15 years and older.

As for pananatomy (pathological anatomy), it is also not fully understood. It is known that follicular hyperplasia is often found in the thymus gland, and lymphoid infiltrates are found in the liver and from endocrinology, in particular in the thyroid gland and adrenal gland.

Follicular hyperplasia is an increase in the rate of reproduction of tissue cells of any type.

Lymphoid infiltrate - blood cells that contain an increased content of lymphocytes.

At risk are women, who are 4 times more likely to have the disease than men.

Nerve impulse transmission

How does the disease manifest itself?

The main symptom of myasthenia gravis is weakness and increased fatigue of skeletal muscles, which occurs with prolonged work and stress. Weakness increases when the same movements are repeated over and over again. This condition can be described as follows: the muscles become very tired and the person stops obeying, and then complete paralysis occurs. After a rest, which can be quite long, they begin to function normally again.

What kind of disease is myasthenia gravis? There are three forms of this serious illness. These are ocular, bulbar and generalized. Each of them has its own symptoms and its own treatment. This is why it is so important to make a correct diagnosis before starting treatment. With the development of a severe form of the disease, a condition such as myasthenic crisis develops, which threatens a person’s life.

Classification

Myasthenia gravis mainly affects the muscles of the upper body (eyes, neck, throat, tongue and face). There are two types of classification. So, according to development they divide:

1. Congenital.
2. Acquired.

According to localization, they are distinguished:

• bulbar form (Erb-Goldflam disease);
• ocular (ophthalmological) form;
• generalized.

Sometimes the disease is called pseudoparalytic myasthenia, since it does not paralyze the muscles, but weakens them, which in some cases gives the impression of paralysis.

What is myasthenia gravis?

Myasthenia gravis is an autoimmune disease that affects the receptors that connect the muscles and the nervous system. The immune system begins to attack the body's own cells, which are absolutely healthy. This can lead to impaired swallowing movements, uncharacteristic weakness of the respiratory system, as well as other equally serious disorders. As the pathology progresses, the patient’s muscles of the lips, face, pharynx, tongue, neck, eyes, and whole body atrophy.

The medical term that characterizes myasthenia gravis is “myasthenia gravis pseudoparalytica.” Translated into Russian, this means asthenic bulbar palsy. Russian doctors often use the term “myasthenia gravis.”

The disease boils down to the fact that a nerve impulse is transmitted from the brain to the muscles with disorders. This occurs due to a failure in the metabolism of acetylcholine in the body.

Skeletal muscles are responsible for the movement of human limbs. The work of internal organs and blood vessels is ensured by smooth muscles. To move the skeletal muscles, motor nerves will be used. At the point where the nerve connects to the muscle, an electrical impulse occurs. Acetylcholine is required for its transmission. This is a special substance that enters the gap between the nerve and the muscle, providing an electrical discharge. Due to it, the muscle contracts.

When a person develops myasthenia gravis, the synthesis and release of acetylcholine is impaired. Therefore, the impulse will pass to the muscle with failure. Each time the conductivity becomes worse. Therefore, human movements suffer. In the terminal stages of the disease, the patient is completely immobilized.

Often myasthenia gravis develops against the background of a tumor of the thymus gland, as well as with hyperplasia. Sometimes patients are diagnosed with organic damage to the nervous system, breast tumors, lung tumors, ovarian or prostate cancer.

Causes of the disease

As stated earlier, the cause of the development of this disease is not fully known. Neurologists have assumptions and some results of treating other patients, but these are just guesses based on medical practice. Not research results.

However, the suspected causes of the disease are the following:

• presence of ARVI in the body;
• nervous system disorders;
• sleeping sickness;
• thyrotoxicosis;
• botulism;
• failure of the immune system (dermatomyositis, scleroderma);
• hormonal imbalance (impaired testosterone production, etc.);
• the presence of tumor processes occurring in the thymus gland;
• liver, lung, breast cancer;
• nervous experiences and stress.

It is worth noting that stress is a trigger, while the presence of nervous problems creates a problem from the inside.

Often myasthenia gravis is a harbinger of amyotrophic lateral sclerosis (ALS), and in such a situation the disease acts as a concomitant myasthenic symptom. And not as an independent disease. Moreover, for a long time the patient may not even be aware of the onset of ALS development.

Prevention of myasthenia gravis and its complications

It is impossible to prevent the disease, but you can do everything possible to live a full life with such a diagnosis. Firstly , doctor's control. Such patients are managed by neurologists. In addition to the prescribed treatment regimen and systematic visits to a neurologist, it is necessary to monitor the general condition (blood sugar, blood pressure, etc.) in order to prevent the development of other diseases during the treatment of myasthenia gravis. Secondly , you should avoid excessive stress - physical and emotional. Stress, hard physical work, and overly active sports worsen the condition of patients. Moderate exercise and walking are even beneficial. Thirdly , you should avoid exposure to the sun. Fourthly , it is necessary to know the contraindications for patients with myasthenia gravis and strictly observe them.

The doctor is obliged to issue a list of medications contraindicated for such a patient. It includes magnesium preparations, muscle relaxants, tranquilizers, some antibiotics, diuretics with the exception of veroshpiron, which, on the contrary, is indicated.

You should not get carried away with immunomodulatory drugs or any sedatives, even those that seem safe (for example, valerian or peony tincture). Fifthly , strictly follow the treatment regimen prescribed by the doctor, do not skip medications and do not take more medications than prescribed by the attending physician.

Signs of the disease

This disease is characterized by two general symptoms:

• increased fatigue;
• muscle weakness.

It is these symptoms that indicate the presence of the disease. When fatigue accumulates for a long time, a person begins to feel paralysis of the affected muscle, but this feeling is temporary. To eliminate it, rest is required.

How impulses are transmitted at the cellular level

After the painful paralysis subsides, you can continue to do your job or do what you were doing.

In addition to general signs, symptoms can be divided depending on the type of disease.

The ocular type has typical features, including:

• ptosis (involuntary drooping of the upper eyelid);
• double vision;
• problems with focusing.

You can check for the presence of ocular myasthenia in a simple way: the patient must clench and unclench his hand at least 20 times; a characteristic sign of myasthenia will be ptosis after just a few repetitions.

The bulbar variant of the disease, in turn, differs:

• problems with swallowing (mainly occur when eating dry or rough foods);
• problems raising your voice;
• the appearance of "nasality".

Fatigue occurs even after a short conversation

Symptoms worsen in the evening.

In the bulbar form, the swallowing and chewing muscles are affected, but sometimes the disease also affects the facial muscles.

For example, a sick person will not be able to open and close his mouth more than 100 times in 40 seconds, and in most cases even less.

The generalized form is the most common and most dangerous. It includes all of the above symptoms, which increase gradually from top to bottom. First, signs of ocular myasthenia appear, then bulbar, and after that the muscles of the neck, trunk and limbs are involved in the process. Paralysis of some limbs occurs and damage to internal organs may occur.

Symptoms

Crises during illness

First you need to understand what a crisis is? This is a kind of acute form of the disease that appears suddenly (sometimes even with lightning speed).

The causes of myasthenia gravis crisis are:

• sleeping sickness;
• thyrotoxicosis;
• oncological diseases (breast cancer);
• skipping pills;
• high psychological or physical stress;
• taking hormonal medications;
• surgical intervention.

According to the severity of myasthenic crisis, there are:

• easy;
• average;
• heavy;
• fulminant.

Depending on the type of crisis, the severity of symptoms varies. But the main signs of this condition are paralysis of the respiratory or swallowing muscles.

In addition, possible manifestations of a crisis are:

• entry of mucus or saliva into the respiratory tract as a result of local paralysis of the swallowing muscles;
• cold sweating;
• suffocation as a result of respiratory muscle dysfunction;
• vegetative manifestations (dry skin, redness, dilated pupils);
• uncontrolled bladder emptying;
• “special” days for women.

It should be remembered that the occurrence of a myasthenic crisis is not just an exacerbation of myasthenia, but a special condition in which it is necessary to immediately consult a doctor, since death can occur if medical care is not provided.

Causes

Myasthenia gravis is an autoimmune disease, i.e. develops as a result of disruption of the normal production of autoimmune antibodies or a rapid increase in the number of auto-aggressive cells that begin to destroy healthy cells, destroy tissue, and provoke inflammation. With myasthenia gravis, the neuromuscular structure of the body is affected, which leads to weakness of the striated muscles. It is believed that the main cause of the disease is a disruption of the normal functioning of the thymus gland, which can be caused by congenital or acquired factors.

Doctors identify 2 mechanisms for the development of the disease:

1. Biochemical disorders of neuromuscular junctions (synapses) with dysfunction of the hypothalamus with further damage to the thymus gland (damage to the thymus gland is secondary).
2. An autoimmune lesion of the thymus gland, in which neuromuscular transmission is disrupted.

The immediate cause of myasthenia gravis is a decrease in the synthesis of acetylcholine (a neurotransmitter responsible for neuromuscular transmission through biochemical reactions) and an increase in the activity of cholinesterase, which blocks neuromuscular junctions. As a result, the conduction of a nerve impulse becomes impossible.

The trigger for the onset of a pathological autoimmune process can be severe stress, viral infectious diseases (for example, influenza, tonsillitis, viral pneumonia, etc.), severe hypothermia and other factors leading to a sharp decrease in immunity.

Diagnostics

To make a correct diagnosis, a comprehensive examination of the body is required. The main test for diagnosing myasthenia gravis is the proserine test.

A special drug, prozerin, has a powerful effect that promotes increased production of the neurotransmitter in the brain. However, this effect is quite short-term and is not suitable for full treatment of the patient.

This test is administered to a person intramuscularly and after 30–40 minutes, neurological indicators are taken, which will make it clear whether the person has problems or not.

However, the test is not always unambiguous and often additional instrumental diagnostics may be required, which includes:

• electromyography - Decrement test (this study is also carried out using proserine);
• electroneurography (if there is any doubt after electromyography);
• laboratory blood tests (a mixed analysis of both clinical and biochemical analysis may be required);
• computed tomography of the brain.

The presence of such a set of studies is necessary for differential diagnosis, which will allow not to confuse myasthenia gravis with other diseases with similar symptoms.

Where is the thymus gland located?

Diseases that can manifest as myasthenia gravis include:

• inflammatory brain diseases (encephalitis, meningitis);
• brain tumors;
• amyotrophic lateral sclerosis;
• Guillain Barre syndrome;
• ischemic stroke.

Diagnosis of myasthenia gravis

In advanced stages of the disease it is not difficult. The assumption of myasthenia gravis should arise in every case of oculomotor and bulbar disorders. In this case, especially typical for myasthenia gravis, in contrast to stem-radicular processes, are the absence of pupillary disorders, randomness of damage to the external eye muscles and the frequent lability of the severity of ophthalmoplegia, especially fluctuations in the degree of ptosis. Finally, ophthalmoplegic syndrome in myasthenia gravis is usually combined with weakness of the orbicularis oculi muscle, which is intact when the 3rd, 4th and 6th pairs of cranial nerves are affected.

Various fatigue tests play a significant role in the diagnosis of myasthenia gravis. Thus, fixation of upward gaze for several minutes, while tracking, for example, a hammer or a doctor’s hand, usually leads to the appearance or worsening of ptosis. Equally, counting out loud to 50-100 gradually reveals a weakening of the voice and the appearance of a nasal tint. It is curious that increased ptosis can sometimes be observed after the patient performs long-term repeated movements in other muscle groups, for example, when squeezing and unclenching the hand.

The proserine test is of decisive importance in the final diagnosis. The introduction of 1-1.5-2 ml of 0.05% proserin under the skin after 20-30 minutes usually significantly reduces muscle weakness. It should only be taken into account that long-term myasthenic oculomotor disorders may not respond to proserin.

Relatively often, the reason for the assumption of the presence of myasthenia gravis is exophthalmic ophthalmoplegia - a special variant of endocrine orbitopathy. However, in these cases, diagnosis is simplified by the presence, along with paresis of the external muscles of the eye, of exophthalmos, periorbital edema, and a sharp thickening of the orbital tissues. Diagnosis is much more difficult in cases of ocular myopathy, which is sometimes indistinguishable from stationary myasthenic ophthalmoplegia. Rarely occurring monocular forms of myasthenia gravis may resemble multiple sclerosis or aneurysm of the circle of Willis vessels in a relapsing course.

The most annoying and sometimes tragic mistake is mistaking myasthenia gravis for hysteria, which is facilitated by the absence of conduction symptoms in patients and the lability of symptoms.

A special problem is differentiating myasthenia gravis from myasthenic syndrome. The latter has now been described in thyrotoxicosis, polymyositis, anterior horn cell disease and polyneuropathies, as well as malignant tumors. Thus, in particular, with bronchogenic carcinoma, combined myastheno-myopathic Lambert-Eaton syndrome may occur, in which, along with myasthenic weakness of the skeletal and cranial muscles, atrophy of the proximal limbs with loss of tendon reflexes is observed. Unlike ordinary myasthenia, muscle weakness in Lambert-Eaton syndrome is clearly reduced by taking guanidine.

Characteristic of all symptomatic forms of myasthenia gravis is a significantly less pronounced effect of anticholinesterase drugs.

Treatment

Treatment of myasthenia gravis is a long and consistent process that should not be interrupted, since sudden withdrawal of drug therapy can provoke a myasthenic crisis.

The disease can be treated in the following ways:

1. Conservative.
2. Conservative in combination with surgery.
3. Folk remedies.

The drug method is the most conservative, but even drug treatment has a depressing effect on the general condition of the patient, primarily due to long-term therapy (up to several years).

The basis of treatment is to increase the amount of acetylcholine at neuromuscular synapses.

This is done with the help of drugs that preserve acetylcholine, and do not provoke its synthesis, since this is quite difficult.

Preparations that have these properties:

1. Pyridostigmil.
2. Oxazyl.

The standard course of taking these medications is about 60 days, but possibly more.

Often the body begins to reject these medications and then it is necessary to adjust the treatment, introducing additional agents that promote the absorption of the active substance (glucocorticosteroids - prednisolone, metipred, immunosuppressants).

At the very initial stage, conservative treatment is used, which includes:

1. Kalimin 60N.
2. Potassium chloride.
3. Potassium-normine.

At the very first stage, potassium-based drugs are widely used, together with drugs that promote its retention in the body (veroshpiron).

It is possible to use painkillers in cases where pain is present (analgin, ketanov, etc.).

Surgery is indicated as a last resort when the prescribed treatment does not help. In addition, the operation is mandatory in patients over 70 years of age.

The essence of the operation is to remove the thymus gland. This surgical intervention is performed under anesthesia and guarantees relief from the disease, but not immediately. A person returns to his normal form after a year or a year and a half, depending on the severity of the disease.

How is the operation performed?

It is worth noting the difficulty of using anesthesia for this disease, since the presence of problems with the respiratory and swallowing muscles complicates the anesthesiologist’s task, but this does not mean that local anesthesia will be used, no. The doctor will simply use a special technique for preparing the patient.

In addition to tablets, concomitant treatment of this disease with folk remedies at home is possible. In no case should you replace drug therapy with home recipes, as this can provoke a crisis.

Be sure to check the use of home recipes with your doctor, as he or she may add or adjust your treatment.

The most famous recipes are:

Oat tincture:

200 grams of washed oats are boiled over medium heat for an hour. After this, the broth is infused for 1.5–2.5 hours. Take the infusion four times a day, an hour before meals, 2 tbsp. spoons.

Onion honey

200 grams of peeled onions are boiled for one and a half hours over medium heat with 200 grams of sugar. After this, add 2 tablespoons of honey to the resulting mixture. The resulting decoction is used for 2 teaspoons 3 times a day.

Garlic mixture

Three heads of garlic, 200 grams of flaxseed oil, a liter of honey and four peeled lemons are crushed and mixed with each other. Take this mixture 1 teaspoon three times a day (this mixture is rich in vitamins necessary for the body).

Among other things, to keep the body in shape you need at least good nutrition. Ideally, increase food rich in potassium, calcium and phosphorus in your diet. Prolonged fasting for myasthenia gravis is not recommended, as it can only worsen the situation.

Products containing potassium:

• oranges;
• bananas;
• avocado;
• beans;
• lentils;
• potato;
• parsnip;
• dried apricots;
• raisin.

Products containing calcium:

• dairy products;
• cabbage;
• turnip leaves;
• asparagus;
• nuts;
• figs;
• salmon.

Products containing phosphorus:

• cottage cheese;
• cheese;
• meat;
• fish;
• eggs;
• buckwheat;
• walnuts.

Oatmeal Phosphorus is needed by the body for better absorption of calcium.

Causes of myasthenia gravis

Modern ideas about the mechanisms of development of pathological muscle fatigue as the basis of the myasthenic process are based on three cardinal discoveries. In 1899, Oppenheim first drew attention to the connection between myasthenia gravis and thymoma. In 1934, Mary Walker pointed out the similarity of the clinical phenomena in myasthenia gravis with the symptoms of curare poisoning and proposed the use of a curare antagonist - the anticholinesterase drug physostigmine (synthetic analogs - prostigmine and proserine) for treatment. Thus, the localization and essence of the main myasthenic defect was established - a conduction block in the myoneural synapse. Finally, Simpson clearly summarized numerous facts that allow us to consider myasthenia gravis as an autoimmune disease: the detection in the blood of patients of antibodies against thymus tissue and skeletal muscles, the possibility of combining myasthenia with other autoimmune diseases (disseminated lupus erythematosus, polymyositis, ulcerative colitis, lymphosarcoma, rheumatoid arthritis, sarcoidosis, Hashimoto's goiter), the presence of remissions during the disease, a significant predominance of sick women, the dependence of exacerbations on infection and pregnancy, the characteristic occurrence of the disease in the 2-3rd decade of life and, finally, the almost constant presence of persistent, hyperplastic or tumor tissue of the thymus gland, the leading organ of immunogenesis.

Reflection of autoimmune mechanisms are the proliferation of epithelial cells of the thymus gland (generative centers) and lymphorrhagia in skeletal muscles. Since removal of the thymus gland does not always alleviate the course of myasthenia gravis, it is assumed that the thymus plays a leading role in the first stages of the disease, and then the autoimmune process is supported by other elements of the reticuloendothelial system. It is assumed that, as part of the autoimmune process at the level of the neuromuscular synapse, a competitive relationship develops between antibodies directed against the motor plate protein and acetylcholine.

The study of the pathogenesis of myasthenia gravis has revealed, like no other form of neuromuscular pathology, a huge number of new facts. However, their final generalization is not yet possible.

Many points remain unexplained in the unusually complex chain of neuro-endocrine, humoral and immunological disorders present in this disease. Particular difficulties are caused by the interpretation of the block of presynaptic transmission from the standpoint of the most common autoimmune concept of myasthenia gravis. And yet, it was precisely the advances in studying the pathogenesis of myasthenia gravis that caused a dramatic change in the fate of many patients. So, if by the time of the discovery of the therapeutic effect of prozerin, 90% of patients died during the first 2 years of the disease, now this figure has dropped to 20-25%.

Myasthenia in children

Despite the fact that the main age of patients with myasthenia gravis is 30–40 years, 10–15% of the total number of patients are children. Of which more than 60% are girls.

The onset of myasthenia gravis in a child is observed in most cases from 7.5 years of age, but there is a possibility of the disease manifesting itself in infants in the neonatal period of development.

Neonatal - the period of development of an infant from the moment of birth until he reaches 28 days of life

If we compare adult and childhood myasthenia, the difference between them is minimal, since the disease progresses in the same way.

There is the following classification:

• local (bulbar, which can progress with or without impairment of respiratory functions; ocular form);
• musculoskeletal (with or without breathing problems);
• generalized (with respiratory and cardiac dysfunction and without these symptoms).

The symptoms of the disease are practically no different from myasthenia gravis in adults. The only thing is that problems may arise with diagnosing the disease, since a small child is not able to clearly explain what and where it hurts and what sensations he experiences.

Diagnosis and treatment differs from the classical one only in the dosage of drugs, which is selected depending on the age of the small patient.

Surgical intervention, by the way, is also carried out, and it gives a 70–80% guarantee that the problem will be eliminated.

The main recommendations for a sick child are:

• restriction of physical activity;
• monitoring the child’s exposure to the sun (long sunbathing is contraindicated);
• taking immunostimulating therapy as a preventive measure;
• eating low-calorie foods.

Treatment of myasthenia gravis

Therapy for myasthenia gravis can be conservative or surgical. The basis of conservative therapy is the systematic use of anticholinesterase drugs - prozerin, mestinon, oxazil, ubretide. Prozerin is used in tablets in a standard dose of 0.015. For injections, a 0.05% solution of proserin is used, which is administered 1-2-3 ml intramuscularly. The effect of 15 mg of proserine taken orally (standard tablet) corresponds to 0.5 mg of proserine administered parenterally (1 ml of 0.05% solution). The effect of proserin when taken orally occurs after 1 hour and lasts 2-4 hours, intramuscularly administered proserin acts after 30 minutes for no more than 2 hours. The average dose of proserin in mild cases is 1-2 tablets every 6-8 hours, in the most in severe cases - 3-4 tablets hourly; observations are described where the daily dose was increased to 100 or more tablets. The absence of toxicosis is explained by the significantly higher tolerance of myasthenia gravis patients to anticholinesterase drugs compared to healthy people. This allows, in necessary cases, to significantly exceed the doses prescribed by the pharmacopoeia. If the state of compensation cannot be achieved with 25-30 standard doses of proserin, then a further increase in the daily dose in most cases does not change the situation. Prozerin, like other anticholinesterase drugs, is best administered orally, as this achieves a longer and more even effect. Parenteral administration is indicated when a rapid effect is needed (crisis) or when the effect of a drug taken orally is insufficient.

Mesti non is available in tablets of 10 and 60 mg. One 60 mg tablet is equivalent to a standard proserine tablet (15 mg). The duration of action of mestinon is much longer - 7-8 hours. Mestinon selectively acts on the cranial muscles and is therefore especially indicated for the ocular and pharyngeal-facial forms of myasthenia. The drug is slightly toxic even at high doses. It is advisable to prescribe mestinon along with proserin.

Oxazil is also used only orally in doses of 0.005-0.01 g. A standard tablet (0.005 g) corresponds in strength to half a proserin tablet. The effect of oxazil occurs after 2 hours and lasts up to 4-8 hours. Increasing the daily dose of the drug is fraught with the development of cholinergic complications, however, in some cases the daily dose was increased to 20 or even 30 tablets. Oxazil has a predominant effect on skeletal muscles. The drug is more toxic than prozerin and mestinon.

Ubretide, administered orally or intramuscularly at a dose of up to 20 mg per day, has a very strong anticholinesterase effect.

To mitigate the side muscarinic effects of anticholinesterase drugs (nausea, hypersalivation, abdominal pain), atropine is prescribed.

The most difficult and important therapeutic problem is the relief of myasthenic crisis, especially the respiratory disorders caused by it. Here it is often necessary to resort to tracheotomy and mechanical breathing. An intravenous infusion of proserine may be required. Antibiotics are needed to prevent pneumonia.

The administration of large quantities of anticholinesterase drugs during myasthenia gravis can be complicated by the development of a cholinergic crisis, the differentiation of which from a myasthenic crisis can be very difficult. Cholinergic crisis consists of three groups of symptoms - muscarinic (constriction of the pupils, nausea, diarrhea, drooling, sweating, abdominal pain), nicotinic (fascicular twitching, cramps) and symptoms from the central nervous system (drowsiness, headaches, tremors, confusion) . In severe cases, these disorders are accompanied by breathing disorders. Unlike myasthenic crisis, cholinergic crisis does not occur acutely, but gradually; Over the course of the day, the patient's condition progressively worsens, despite increasing doses of anticholinesterase drugs.

Treatment of cholinergic crisis requires discontinuation of anticholinesterase drugs and the use of resuscitation measures. A temporary transfer to mechanical breathing followed (after toxicosis has passed) resuming the administration of an adequate amount of anticholinesterases is all the more justified in such confusing situations, since a combination of myasthenic and cholinergic crisis may occur here.

In general, choosing the optimal dose of anticholinesterase drugs and the daily rhythm of their administration requires a purely individual approach and careful monitoring of the patient. Straightforward dose increases do not always lead to the desired goal.

Auxiliary agents in the treatment of myasthenia gravis are potassium chloride, aldactone (veroshpiron), which increase the potassium content, and thereby indirectly help overcome the block of neuromuscular transmission.

In 1929, physician Harriet Edgeworth, who had suffered from myasthenia gravis for many years, discovered that ephedrine, which she took to relieve menstrual pain, significantly reduced muscle weakness. Until 1934, when Mary Walker introduced physostigmine, ephedrine was the only treatment for myasthenia gravis. Hydrochloric ephedrine (0.025 g tablets) is still readily prescribed to patients with myasthenia gravis, but its effectiveness is insignificant and the mechanism of action is unclear.

X-ray therapy of the thymus gland has occupied a significant place in the treatment of myasthenia gravis. Surgical treatment is widely used - removal of the thymus gland. Excellent and good results, according to long-term observations, occur on average in 60% of patients. The results of the intervention are significantly worse in the presence of thymoma.

Patients suffering from myasthenia gravis should not be prescribed quinine, quinidine, morphine, streptomycin, neomycin, aminazine, elenium, since these substances impair neuromuscular conduction.
Patients also do not tolerate diuretics and barbiturates well. The article was prepared and edited by: surgeon I.B. Pigovich.

Video:

Healthy:

Related articles:

1. Forms of myasthenia Gravis Forms of myasthenia in children Neonatal myasthenia (myasthenia in newborns) Do you have any questions or is something unclear? Ask...
2. Diagnosis of myasthenia gravis Several tests can help diagnose myasthenia gravis...
3. Myasthenia gravis in children Myasthenia gravis is a disease of the nervous and muscular systems, characterized by progressive weakness and pathological fatigue...
4. Dystrophic myotonia Dystrophic myotonia (Kurschmann-Batten-Steinert disease) is characterized by myotonia, progressive muscle atrophy, a complex of somato-endocrine...
5. Werdnig-Hoffmann disease Werdnig-Hoffmann disease (childhood spinal amyotrophy) is one of the forms of neuromuscular pathology that occurs in early childhood...
6. Motor manifestations of polyneuropathy The main motor manifestation of polyneuropathy is paresis, accompanied by hypotension, muscle wasting and predominantly involves the limbs (more often...

Pregnancy

Myasthenia gravis can affect not only an ordinary person, but also a girl who is in an interesting position. As such, pregnancy is not a factor that leads to the development of the disease. The impetus for the development of the disease will be:

• hormonal imbalances;
• stress;
• nervous instability;
• factors characteristic of the development of myasthenia gravis in an ordinary person.

The main complications in such a situation is the risk of transmitting the disease to the child, so there are several options:

1. Transient myasthenia gravis (does not threaten serious pathologies and is curable).
2. Arthrogryposis (a more serious form of the disease that can result in joint deformity).

Among other things, this disease during pregnancy threatens the expectant mother with problems with the respiratory and cardiovascular systems, up to their complete failure (in the worst case scenario).

Diagnosis and treatment are no different from usual, with a slight adjustment for pregnancy.

A contraindication during pregnancy is natural childbirth, as serious problems may arise during the process and there is a risk of losing either the mother or the baby. Particular attention is also paid to the use of epidural anesthesia.

In most cases, births take place via caesarean section.

The condition that should be taken into account is that pain relief should also be carried out taking into account the presence of myasthenia gravis.

Preventive measures

Prevention of myasthenia gravis is not carried out, in its classical sense, since the disease can occur suddenly, and its true nature is still in question. Unfortunately, there is also no vaccine against this disease.

Naturally, the doctor will indicate to the patient the recommendations that he will need to follow, including:

• maintaining a healthy lifestyle;
• it is necessary to prohibit the patient from drinking alcohol during the therapeutic course;
• restriction (in some cases a complete ban) on active physical activity (therapeutic gymnastics is allowed), work associated with brute physical force is also prohibited;
• you need to monitor your diet; you cannot neglect the classic recommendations about eating junk food;
• avoid stressful situations.

The most basic and important rule is not to stop taking medications, as this can provoke a crisis.

Forecast

The prognosis of the disease is favorable. Currently, any form of the disease has a favorable outcome, but only if the specialist’s recommendations are strictly followed.

Visual myasthenia is the easiest to tolerate, while the generalized form of the disease is difficult to cure. Improvements (remissions) are possible, alternating with periods of exacerbation.

As an unfavorable complication, we can note the possibility of the patient developing blepharospasm.

So, myasthenia gravis is a dangerous disease that, if not properly treated, can lead to the death of the patient. You should not delay contacting a specialist, as this can seriously harm yourself. Take care of yourself and your health, get treatment on time!