Myasthenia crisis treatment. Myasthenia. myasthenic and cholinergic crises. Causes of myasthenic crisis

Modern ideas about the heterogeneity of the pathophysiological mechanisms of myasthenia gravis indicate the ambiguity of the effect of the main types of treatment for different patients: from complete recovery in some patients, slight improvement or no effect in others, to the development of exacerbations of the disease with myasthenic crises in others. This indicates the need to search for additional pathogenetically substantiated therapy for the disease and requires clarification of the criteria for the feasibility of such important measures as thymectomy, intravenous administration of high doses of glucocorticoid steroids, and the justification for prescribing expensive cytostatic immunosuppressants and human immunoglobulins.

MYASTHENIC CRISIS - this is a suddenly developed critical condition in patients with myasthenia gravis, which indicates not only a quantitative, but also a qualitative change in the nature of the process.

Approximately 10% In patients with myasthenia, myasthenic crises require artificial ventilation and ensuring airway patency.

Etiology. Myasthenic crisis can be triggered by respiratory infections and surgical operations, for example, thymectomy, as well as intercurrent diseases, physical and mental stress, and in women - the menstrual period.

Pathogenesis. The pathogenesis of the crisis is associated not only with a decrease in the density of cholinergic receptors of the postsynaptic membrane due to their complement-mediated destruction (autoimmune damage), but also with a change in the functional state of the remaining receptors and ion channels.

Clinical picture. At the beginning of the development of myasthenic crisis, breathing becomes shallow and frequent with the inclusion of auxiliary muscles; subsequently it becomes rare and intermittent, which further leads to hypoxia with facial hyperemia, followed by cyanosis. This condition is obligately (in the absence of pronounced depression of consciousness) accompanied by anxiety and psychomotor agitation. It should be emphasized that the progression of respiratory failure sometimes occurs within a few minutes and in the absence of timely medical assistance, complete cessation of breathing and loss of consciousness occurs. Changes associated with breathing also occur in the cardiovascular system. At the moment of crisis, an increase in heart rate to 160-180 per minute and an increase in blood pressure to 200 mm is recorded. rt. Art. As the hypoxic condition worsens, blood pressure decreases, the pulse first becomes tense, then arrhythmic, rare, and thread-like. Also, a severe generalized myasthenic crisis is indicated by the appearance in the patient of symptoms of hypoxic encephalopathy (with the appearance of intermittent pyramidal symptoms), pronounced bulbar disorders (dysphagia, dysphonia), severe weakness of skeletal muscles, increased autonomic symptoms in the form of severe salivation and sweating (these signs are also characteristic and for cholinergic crisis). In extreme cases of myasthenic crisis, loss of consciousness is accompanied by involuntary urination and defecation.

THERAPY FOR MYASTHENIC CRISIS

PRE-HOSPITAL THERAPY

At the prehospital stage, it is necessary to carry out measures aimed at restoring and maintaining the patency of the upper respiratory tract, and if there is a threat or in case of already developed suffocation, tracheal intubation, sanitation of the tracheobronchial tree and, if necessary, artificial ventilation of the lungs with a manual or any available automatic respirator are performed.

!!! CHOLINESTERASE INHIBITORS SHOULD BE TEMPORARILY DISCONTINUED: THIS WILL AVOID INCREASED SECRETION IN THE RESPIRATORY TRACT AND OVERDOSE OF ANTICHOLINESTERASE DRUGS (ACHEDS) WITH THE DEVELOPMENT OF “CHOLINERGIC CRISIS”

INTENSIVE CARE IN A HOSPITAL

1. Artificial pulmonary ventilation (ALV).

In each specific case, the issue of transferring the patient to mechanical ventilation is decided on the basis of the clinical picture:
disturbance of the rhythm and depth of breathing,
cyanosis,
excitation,
loss of consciousness,
participation in breathing of auxiliary muscles,
change in pupil size,
lack of response to the administration of anticholinesterase drugs, etc.,
as well as objective indicators reflecting the gas composition of the blood, hemoglobin oxygen saturation, acid-base state (ABS), etc.:
BH - over 40 per minute,
VC less than 15 ml/kg,
PaO2 below 60 mm. rt. Art.,
PaCO2 above 60 mm. rt. Art.,
pH about 7.2,
НbО2 below 70-80%.

!!! SIMULTANEOUS ADMINISTRATION OF ACEP AND THE USE OF VENTILATION IS NOT ACCEPTABLE

The patient must be on mechanical ventilation for 24 hours. The next day, it is recommended that the patient be tested with ACEP, taking into account the onset of action of the drug, and be disconnected from the ventilator (without extubating!), then evaluate the effectiveness of spontaneous breathing. If the patient breathes independently within 1.5-2 hours, repeat the procedure 3-4 times during the day with ACEP. If the result is positive, the patient is extubated. If the respiratory function remains insufficient and the effect of the administration of ACEP is short-lived, the patient is connected to a ventilator. Do not administer prozerin until the next day! Repeat the above steps the next day. If a positive effect (spontaneous breathing) is not observed within 3-4 days, the patient must have a trachiostomy to exclude tracheal pressure ulcers. As the vital functions of breathing and swallowing are restored, the patient is disconnected from the ventilator and prescribed ACEP.

2. Carrying out pulse therapy (methylprednisolone). The most effective pulse therapy is methylprednisolone intravenously at a dose of 1000 mg (the average dose is 10–15 mg per 1 kg of patient weight per day). Methylprednisolone in 100–250 ml of 0.9% isotonic sodium chloride solution or 5% glucose solution and administered intravenously over 35–45 minutes daily, consecutively for three days. Slower (60–90 minutes) or, conversely, rapid (10–15 minutes) administration is not recommended, as it either significantly reduces the clinical effectiveness of pulse therapy or can lead to severe complications, including the development of acute heart failure. As a rule, no other medications are added to the IV. At the same time, in older people, with a sufficiently long course of the disease, the presence of arterial hypertension, and myocardial damage, it is possible to prescribe lower doses of methylprednisolone (5 mg/kg/day) during those days. This method is known as mini-pulse therapy.

3. Introduction of potassium chloride. Potassium chloride (70 ml of 4% solution, or 30 ml of 10% solution in 400 ml of 5% glucose solution or physiological solution) during myasthenic crises is administered intravenously, slowly at a rate of 20-30 drops per minute with the introduction of 4 - 7 units. short-acting insulin at the end of the drip.

4. Administration of immunoglobulin G (normal human immunoglobulin, humaglobin, octagam, Biaven, Vigam, intraglobin) or plasmapheresis with removal of 35-40 ml of plasma per 1 kg of weight with replacement either with donor or artificial, taking into account the disturbance of protein metabolism and the shortage of infusion devices containing donor proteins.

Currently, immunoglobulin G therapy is an alternative to plasmapheresis due to the similarities in the mechanisms underlying these treatments. Human immunoglobulin is an immunoactive protein. The use of high doses of immunoglobulins has the ability to suppress immune processes. The generally accepted treatment regimen is short 5-day courses of intravenous administration of the drug at a dose of 400 mg/kg daily. On average, the clinical effect is observed on the 4th day of therapy and persists for 50-100 days. After 3-4 months, a second course of immunoglobulin therapy is possible. Octagam and Bioven can also be used intravenously drip at a minimum dose of 4-5 mg/kg No. 10, the total dose is 25g.

The most effective treatment measure for the development of myasthenic crisis is exchange plasmapheresis. Examination of a patient who is scheduled for plasmapheresis should include: assessment of vital functions, complete clinical blood count (including platelets, hematocrit), determination of blood group and Rh factor, RV, HIV carriage, Australian antigen, total protein, protein fractions , basic indicators of peripheral and venous blood coagulation, clinical urine analysis. The plasmapheresis method is based on collecting blood from the ulnar or one of the central veins, followed by centrifugation, separation of the formed elements and replacement of the plasma with either donor or artificial plasma. This procedure leads to rapid - sometimes within a few hours - improvement in the condition of patients. It is possible to extract plasma again over several days or every other day. Depending on the indications, centrifugal plasmapheresis (manual or hardware), filtration (hardware), plasmapheresis in combination with plasma sorption are used. The operation is carried out in an operating room or intensive care ward, equipped and equipped in accordance with the requirements for the management of patients in critical condition, the presence of monitoring and treatment equipment, appropriate medications and infusion media, and the ability to perform cardiopulmonary resuscitation. Premedication is prescribed according to indications and includes analgesics and antihistamines. As a rule, plasmapheresis is carried out over a course of 1-2 weeks with a frequency of 2-5 operations. Intermittent plasmapheresis leads to improvement after 3–4 sessions. The effectiveness of continuous plasmapheresis, despite the large capacity for the volume of replaced plasma, does not differ significantly from intermittent plasmapheresis. The duration of improvement in the condition of patients after exchange plasmapheresis ranges from 2 weeks to 2 - 3 months. A contraindication to the use of plasmapheresis is the presence of pneumonia or other inflammatory processes.

5. Antioxidants (for example, lipoic acid). Antioxidants promote the activation of mitochondrial synthesis, reduce the severity of oxidative stress in patients in a state of myasthenic crisis, by reducing the content of free radicals in the blood, which contribute to damage to cellular and mitochondrial membranes during ischemia. Lipoic acid is administered intravenously in the amount of 600–900 mg/day, followed by oral administration in the same dosage.

6. Prevention of pneumonia (do not use aminoglycosides).

7. Carrying out symptomatic therapy.

8. Special care.

(p. cholinergica) K. caused by an overdose of anticholinesterase drugs (eg, in the treatment of myasthenia gravis), characterized by weakness, arterial hypotension, bradycardia, miosis, vomiting, salivation, convulsions and fasciculations.

  • - a sudden short-term condition in a patient with the appearance of new and intensification of existing symptoms of the disease...

    Medical terms

  • - a sudden, relatively short-term condition in a patient, characterized by the appearance of new or intensification of existing symptoms of the disease. Addisonian - see adrenal...

    Medical encyclopedia

  • - a sudden, relatively short-term condition in a patient, characterized by the appearance of new or intensification of existing symptoms of the disease...

    Large medical dictionary

  • - S., in which the mediator is acetylcholine...

    Large medical dictionary

  • - 1. Used to characterize neurons, nerve fibers and their endings that use acetylcholine as a neurotransmitter. 2...

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  • - ...

    Word forms

  • - ; pl. cry/zy, R....
  • - ...

    Spelling dictionary of the Russian language

  • - -and husband. . A sudden onset of acute illness...

    Ozhegov's Explanatory Dictionary

  • - crisis m. A sudden sharp deterioration in the patient’s condition against the background of an existing disease; attack...

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  • - ...
  • - cholinergic "...

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  • - KRISA a, m. KRISA s, w. CRISIS a, m. crise f., lat. crisis. 1. diploma A difficult situation, a condition that requires a decisive turn, a turning point. Sl. 18. A good way to free yourself from some obligations, which...

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  • - sudden exacerbation of the disease...
  • - cholinergic biol. sensitive to acetylcholine, excited by it cf. adrenergic)...

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"cholinergic crisis" in books

CRISIS DURING FASTING

From the book A Way of Living in the Age of Aquarius author Vasiliev E V

CRISIS DURING FASTING When the body is dirty, a crisis occurs during the period from the 8th to the 12th day of fasting. A crisis is a state of discomfort, bad mood and well-being. The crisis usually occurs at night. If before the crisis there is malaise, then after the crisis there is no longer any malaise

2.9. Hypertensive crisis

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Hypertensive crisis Hypertensive crisis is a condition in which a sharp increase in blood pressure occurs, accompanied by a deterioration in well-being. Hypertension is very common, therefore, with manifestations of a crisis among relatives and friends

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From the book Emergency Care Directory author Khramova Elena Yurievna

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Thyrotoxic crisis

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Hypertensive crisis

author Vyatkina P.

Hypertensive crisis Psychoemotional overload and severe nervous tension in a patient with hypertension can lead to a hypertensive crisis, a complication of hypertension that occurs both during a stressful situation and after it.

Hypertensive crisis

From the book Complete Medical Diagnostics Guide author Vyatkina P.

Hypertensive crisis Medical assistance in such a situation should be immediate, since a prolonged crisis can lead to serious complications in the brain and heart. Before the doctor arrives, the patient should be put to bed, and a heating pad can be placed at his feet. Crises

Hyperglycemic crisis

From the book Complete Medical Diagnostics Guide author Vyatkina P.

Hyperglycemic crisis Vomiting and abdominal pain during a hyperglycemic crisis can simulate an acute abdomen. In diabetics, a crisis and subsequent coma develop from excess sugar in the blood (hyperglycemia). The development of a typical coma picture is usually preceded by the phenomena

Adrenal crisis

From the book Complete Medical Diagnostics Guide author Vyatkina P.

Adrenal crisis During decompensation of chronic adrenal insufficiency, nausea, vomiting, and pain in the upper abdomen are often observed. Usually the subsequent addition of cardiovascular failure, muscle weakness and fever

Hypercalcemic crisis

From the book Complete Medical Diagnostics Guide author Vyatkina P.

Hypercalcemic crisis Repeated vomiting with dehydration may be the earliest and most obvious sign of a hypercalcemic crisis in hyperparathyroidism. This severe complication (a state of rapid and sharp increase in calcium in the blood) is associated with increased production

Hypercalcemic crisis

From the book Complete Medical Diagnostics Guide author Vyatkina P.

Hypercalcemic crisis For emergency treatment, the patient must be taken to the hospital. An intravenous infusion of an isotonic sodium chloride solution is administered in an amount of 3–4 liters per day to stimulate renal secretion, in the absence of renal failure and

Hypoglycemic crisis

From the book Complete Medical Diagnostics Guide author Vyatkina P.

Hypoglycemic crisis The feeling of palpitations is one of the symptoms that occurs in patients during attacks of hypoglycemia. Attacks of hypoglycemia caused by increased secretion of insulin by beta cells of the pancreas due to hormonally active

Hypertensive crisis

From the book Hypertension Handbook author Savko Liliya Mefodievna

Hypertensive crisis A hypertensive crisis is a sudden increase in blood pressure, during which health deteriorates significantly. Indicators of the severity of a crisis are not the level of increase in blood pressure, but signs of brain damage or

Hypertensive crisis

From the book Nurse's Handbook [Practical Guide] author Khramova Elena Yurievna

Hypertensive crisis Clinical manifestations - see Nursing in

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









The most dangerous, life-threatening condition with myasthenia gravis are crises - myasthenic And cholinergic.

Myasthenic crisis in myasthenia gravis usually associated with impairment of neuromuscular transmission. This may be due to a change in the functional state of the synaptic apparatus under the influence of various factors: malaise, fever, menstruation, fatigue, etc. or a decrease in the dose of ACEP for one reason or another. The main danger is deterioration of bulbar functions, up to aphagia, as well as weakness of the respiratory muscles with increasing respiratory failure. At the prehospital stage, immediate subcutaneous or even intravenous administration of a 0.05% proserine solution is necessary; after 30 minutes, proserin is re-injected subcutaneously; intravenous administration of methylprednisolone is mandatory - up to 1.5-2 g or prednisolone 1.5-2 mg/kg.

Patients with complications of myasthenia gravis are hospitalized in centers of neuromuscular pathology or resuscitation, neurointensive care units, or intensive care units, where they usually undergo plasmapheresis, and, if necessary and possible (the drug is very expensive!), immunoglobulin G is administered intravenously - 0.4 mg/kg, according to indications mechanical ventilation is used.

Cholinergic crisis in myasthenia gravis is associated with an overdose of anticholinesterase drugs, resulting in symptoms of muscarinic and nicotinic intoxication: muscle fasciculations, ptosis, hypersecretion of saliva, sweating, pallor, bradycardia, abdominal pain, diarrhea. AHEP are immediately temporarily cancelled. In a threatening situation, 0.5-1.0 ml of a 0.1% atropine solution is slowly injected intravenously. Patients are taken to the above departments, where, according to indications, they are prescribed mechanical ventilation and plasmapheresis is performed.

If anticholinesterase drugs are ineffective, the patient’s condition worsens - myasthenic crisis, in case of an overdose of these drugs - cholinergic crisis. Muscle weakness increases sharply, severe bulbar phenomena develop, sharply complicating breathing, both due to weakness of the muscles of the chest, diaphragm, and due to obstruction of the upper respiratory tract (accumulation of tracheobronchial secretions).

Myasthenic crisis: wide pupils, tachycardia, normal or low blood pressure .

Cholinergic crisis: narrow pupils, bradycardia, rapidly developing hypotension, pronounced muscle fasciculations, hypersecretion of saliva and bronchial mucus, abdominal pain, diarrhea, vomiting due to rapid intestinal motility.

Emergency care for patients with myasthenia gravis

should be aimed at correcting breathing - intubation and transfer to mechanical ventilation.

With myasthenic crisis immediately inject 1 ml of 0.05% proserin solution intravenously and after 15 minutes 1 ml of proserin subcutaneously. The patient is hospitalized in the intensive care unit of the hospital. In addition to prozerin, you can use oxazil in suppositories and orally (0.01 g) or mestinon (0.06) orally. The administration of prednisolone 90 - 120 mg per day is indicated. The best results in myasthenic crisis are achieved by plasmapheresis or intravenous administration of immunoglobulins.

At cholinergic crisis anticholinesterase drugs are discontinued and 0.5 - 1 ml of 0.1% atropine solution is administered intravenously at intervals of 1 - 1.5 hours until the pupils dilate and dry mouth appears. Administration of the cholinesterase reactivator dipyroxime subcutaneously or intramuscularly (1 ml of 15% solution) is indicated. Patients are fed through a nasal tube.


This disease is of a neuromuscular nature. It occurs chronically with a relapsing or progressive course. The main feature of the pathology is increased weakness of the muscles of the striated group. The progression of symptoms usually occurs slowly. And they get sick in childhood.

With myasthenia gravis, any muscle can be involved in the process. However, the most often affected group is the neck, face, eyes, and pharynx. The progression of the disease can reach a state similar to paralysis. The pathology was first described in the 16th century. Data accumulated over such a long period of time indicate that women are three times more likely to suffer from myasthenia gravis.

The incidence is increasing from year to year. On average, it is detected in 6-7 cases per 100,000. The largest proportion of patients with myasthenia gravis is in the age group from 20 to 40 years. However, the pathological condition has no preference at the beginning of development and can start at any time, and even be congenital.

Causes

Depending on the type of myasthenia gravis, there are several factors that can trigger it. Pathology is classified into two main types:

  • Congenital;
  • Acquired.

The first appears due to a genetic defect. It is predominantly hereditary. Such a failure creates conditions for the formation of muscle weakness at the cellular level. Pathogenesis consists of disruptions in the functioning of synapses - intermediaries between nerves and muscles. Neuroscience studies disorders of synaptic transmission.

Acquired myasthenia is registered more often than the first. However, it is much better treated. It may be due to several factors:

  • Oncological. Thus, a number of myasthenia gravis are formed against the background of tumors. These may be neoplasms of the thymus, genital organs (ovaries, prostate), less commonly the lungs and liver;
  • The autoimmune nature of the pathology has recently become one of the most common causes of myasthenia gravis. This means that the disease often occurs against the background of diseases associated with immune failure. In which your own cells attack your body. This can occur with dermatomyositis or scleroderma. With the autoimmune nature of the disease, antibodies are produced to the receptors of synapses - adapters between muscle and nerve. As a result, the delivery of substances necessary for muscle contraction - mediators - is blocked. A failure occurs that leads to myasthenia gravis.

Symptoms

The main symptom that will manifest the disease is pathological muscle weakness. The muscles become tired quickly and cannot work for a long time. This is especially obvious when performing repetitive movements. Rest has a beneficial effect on muscle recovery. Waking up in the morning, the patient may not feel any signs of illness. But, after a few hours, it becomes noticeable again.

Depending on the clinical course of the pathology, there are 3 forms:

  • Bulbar;
  • Ophthalmic;
  • Generalized.

The first is responsible for damage to the muscles - chewing, swallowing and voice-forming muscles. In patients with this form, speech changes - it becomes hoarse, quiet, even silent. The act of passing food is disrupted. Patients have difficulty swallowing and chewing. Due to insufficient food intake, patients lose a lot of weight.

The second type affects the eye muscles - the external, round, levator pallid. As a result, it becomes difficult for patients to blink and open their eyes. This reduces vision function. And it affects people's social lives. They prefer to stay at home and not go out. Another clinical form of the disease is generalized.

It sequentially captures the oculomotor, facial, and cervical muscles. This affects not only the functions, but also the appearance of the patient. The face becomes unplastic and looks prematurely aged. The smile becomes strained, and weak neck muscles make it difficult to hold the head upright.

Progression leads to pathology of the limbs, which impairs walking and free movements. Lack of stress leads to atrophy. This makes the muscles sluggish, flabby and weak. It decreases in volume. Patients look exhausted and emaciated. Therefore, the generalized form is considered the most dangerous - it immediately affects a large number of muscles.

However, this type is more common than others. Another severe and acute condition in myasthenia gravis is a crisis. This is an instant attack of weakness, which will be accompanied by cessation of muscle function, including vital ones - swallowing and breathing. This condition is deadly due to a decrease in oxygen supply to the body.

Cholinergic crisis

This attack rarely develops in 3% of cases. He progresses more slowly. If we compare an attack with a myasthenic crisis. The cause of such conditions is often an overdose of anticholinesterase drugs. This looks like an increasing deterioration in well-being followed by signs of intoxication. After which a seizure develops.

It is quite difficult to distinguish between cholinergic and myasthenic crises. Both conditions have severe muscle weakness that affects the bulbar and respiratory groups. However, it is still possible to distinguish them. The cholinergic crisis develops against the background of an increased dose of anticholinesterase drugs, which is accompanied by a paradoxical decrease in muscle strength.

Diagnosis of the condition

The proserine test has proven itself well as a research method. This substance is capable of creating a block to the breakdown of mediators that cause muscle contractions. The proserine test is carried out as follows - a preliminary examination of the patient. This is necessary to assess the condition of the muscular system before diagnosis.

Then the drug is injected subcutaneously. After 30 minutes, the doctor examines the patient again. Revealing changes after taking proserin. Another study necessary if myasthenia gravis is suspected is electromyography. It is based on recording the electrical activity of muscles. It is carried out twice - before the proserine test and after. This method can help in determining the origin of the pathology.

Is it associated with a disorder of neuromuscular transmission or occurs in isolation. Another test used in myasthenia gravis is electroneurography. CT (computed tomography) is considered a valuable method. It is aimed at identifying neoplasms and possible causes of myasthenia gravis. Among laboratory methods, blood tests deserve attention. They are necessary for the detection of specific antibodies.

Crisis treatment

This pathological condition requires emergency care. Due to an attack of sudden weakness of the breathing and swallowing muscles, a person can die. Therefore, patients in this condition require urgent hospitalization in intensive care. These patients have their own algorithm and treatment protocol. The first activity that is carried out is the formation of airway patency with oxygen supply.

Doctors perform tracheal intubation or perform mechanical ventilation. After this, respiratory function is constantly monitored and the patency of the respiratory tract is assessed. If there are no signs of an overdose of proserin, then this particular drug is administered. Side effects are eliminated by administering Atropine.

According to indications, glucocorticoids are prescribed at a dosage of 100 mg per day or higher. Such remedies are not indicated for everyone because of their possible negative effect in the form of new weakness of breathing. If a secondary infection occurs, antibiotics are prescribed. Laboratory values ​​are constantly monitored to exclude electrolyte disturbances. In some cases, plasmapheresis and immunoglobulin administration are indicated.

Forecast

The course of myasthenia gravis depends on many factors:

  • Forms of the disease;
  • Start of the process;
  • Rate of symptom progression;
  • The patient's living conditions;
  • Gender and age;
  • Timeliness and quality of treatment provided.

The ocular type of myasthenia is considered the most favorable. The worst course is generalized. Currently, the arsenal of medications and diagnostic procedures allows us to give a large number of patients a favorable prognosis. This does not mean that they will recover; the goal of therapy is to stop the progression of myasthenia gravis.

Classifying the pathology as a chronic process gives the likelihood of lifelong use of medications. This is carried out in courses or continuously. Timely diagnosis allows patients to be given a good prognosis. These research methods manage to detect pathology before it affects all muscles and causes irreversible changes.

Prevention

Avoiding the disease is not easy. Due to the fact that the main causes of myasthenia gravis are genetic mutations, tumor formations and autoimmune pathology, it is not easy to prevent the disease. Preliminary medical and genetic consultation before pregnancy can serve as prevention. As well as maintaining a healthy lifestyle, which increases the chances of avoiding myasthenia gravis.

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