Myasthenia gravis. Symptoms, causes, treatment. That is, eye, generalized. Diet, drugs, diagnostics

Autoimmune diseases, one of which is male, lead to the fact that the organism produces substances which destroy own tissues. Such diseases are also called systemic. They are particularly difficult to treat because the doctor can act only on external manifestations of the disease, and partly on the mechanism of disease development.

definition of myasthenia gravis

Myasthenia gravis is shown during pathological fatigue and rapidly increasing muscle weakness. It is a result of the gradual destruction of acetylcholine receptors immunity factors. This violates the impulse transmission from the nerve ending to the muscle fiber, the muscle does not contract.

Increasing muscle weakness arises from the violation of the right balance between acetylcholine (Compound transmitting nerve impulses to muscle fiber - the mediator) and cholinesterase (degrading enzyme acetylcholine).

There are other names of this disease, in the place of injury or on behalf of the scientists who studied it: Erba Goldflama and Jolly.

forms of the disease

Classification of disease takes into account several criteria:

The disease is classified and on such grounds:

• speed development of symptoms;
• presence of visceral, cardiovascular and respiratory disorders;
• the presence of thymic tumors and other hyperplastic and neoplastic processes;
• quantity in blood serum for myasthenia specific antibodies to acetylcholine receptor (AChR).

Causes of myasthenia gravis

Myasthenia gravis (symptoms, the causes need to know to start early treatment) - the actual problem in modern medicine, because of the increase in incidence is observed pathology. Different forms of the disease occur at a frequency of 3 to 12 cases per 100 000 people. Most often ill people 20-30 years old, and it is the most active group of population.

Cause of the disease is unknown.

Scientists have found a link between him and certain predisposing factors:

• infection, stimulating immune responses;
• tumors (thymoma, thymus hyperplasia, thymus persistent);
• menstruation or pregnancy (women are affected 3 times more often), other hormonal disorders;
• stress;
• Genetic predisposition: in the family, there are cases of autoimmune, neurological, or hyperplastic diseases.

mutational nature proved (genetic variation) many cases of severe forms of inherited myasthenia gravis (myasthenia gravis).

symptoms

The patient feels the following symptoms of the disease:

• drooping of the upper eyelid;
• sees double minded image;
• hard to chew and swallow;
• slurred speech;
• inactive muscles of the face, which has a negative effect on the facial expressions;
• difficult to keep your head straight, the lower jaw droops;
• difficulty walking, especially on stairs.

Typically, symptoms of the disease are more visible in the evening, but after a rest they decrease or disappear.

At an early stage of the disease is manifested only weakness of one or more muscle groups. Later symptoms worsen, muscle wasting occurs even small loads. Further development of myasthenia gravis can lead to paralysis.

myasthenic crisis

Myasthenia gravis in some cases can not be cured. Regardless of the causes, the disease develops, and often comes myasthenic crisis. This symptom indicates the appearance of new elements of the disease process. In this step varies operability preserved receptors and ion channels in the postsynaptic membrane.

Manifestations of myasthenic crisis in a generalized form:

• Restlessness, the patient is difficult to sleep.
• Disturbances of consciousness: stunning, which preserves a limited verbal contact; stupor or deep depression when the patient may for a short time to withdraw from the sleepy state; coma or absence of consciousness.
• Bulbar syndrome: swallowing difficulties (dysphagia), speech (dysarthria), snuffles and unscrupulous speech until the complete disappearance of voice (aphonia).
• Autonomic symptoms: excessive salivation, severe sweating.

In more severe cases, there are respiratory distress, growing in a matter of minutes or hours:

• quickens breathing becomes shallow;
• later breathing becomes rare and discontinuous;
• asphyxiation occurs (hypoxia), redness of the face and neck, then blueness (cyanosis);
• possible respiratory arrest.

Perhaps equally rapid development of cardiac activity:

• pulse becomes increasingly (160-170 beats / min..);
• blood pressure increases (up to 190-210 mm Hg);
• later the pressure sharply decreases, slows the pulse, it becomes tense, then filiform.

Neurological symptoms are observed:

• increased tendon reflexes;
• pathological and paradoxical reflexes.

Myasthenic crisis - threatening complication.

Deep breathing disorders require:

• cancellation AHEP;
• connecting the patient to the ventilator;
• prescribe morphine to synchronize breathing and the patient work of the ventilator;
• Sodium hydroxybutyrate application to relax the muscles.

Another method of relief of crisis - or centrifugal filtration plasmapheresis and plasmasorption that quickly improves the condition of the patient. If necessary, the procedure may be repeated at intervals of several days.

Displaying drip (25 drops per minute.) Administration of 2.5-3 g of potassium chloride in 400 ml of physiological saline or 5% glucose solution. At the end of the procedure to the solution is added 4.7 units of insulin. Neostigmine and its analogues are used in the tests to decide on the termination of intubation.

Diagnostics

On examination, the doctor can identify:

• ptosis century (ptosis), double vision, and sometimes the asymmetry of the pupils, decreased visual acuity;
• reduction ptosis in performing the century, local cooling (e.g., ice);
• decrease in sensitivity, especially temperature and pain;
• variability characteristic symptoms: evening or they are amplified by repeating movements (stereotyped load), after a rest decreases;
• poor mimicry (face mask).

Used pharmacological tests with subcutaneous administration AHEP (or Neostigmine Neostigmine).

After 30-90 min. Sample according to the degree of symptoms of compensation is estimated as:

• positive - the disappearance of signs;
• dubious - reduction of feature;
• negative - there is no sign of change.

Laboratory studies determined:

• degree of conservation of neuromuscular transmission (electroneuromyography, ENMG);
  
• the presence or absence of tumors (radiological methods).

Myasthenia gravis (symptoms, the causes of the disease are similar to other serious pathologies) can occur when:

• malignancies of the ovaries, lung, thoracic glands;
• neurological diseases (Charcot-Marie syndrome);
• systemic diseases: scleroderma, dermatomyositis, myopathies;
• amyotrophic lateral sclerosis.
• gravis;
• congenital and chronic progressive ophthalmoplegia;
• brain-stem arachnoiditis;
• progressive muscular dystrophy.

Lambert-Eaton syndrome also manifests itself in a painful weakness, but there are important differences between them.

sign	Lambert-Eaton syndrome	myasthenia
Violation signaling	Violation occurs in the presynaptic membrane	Violation on the postsynaptic membrane
In the study of blood serum to detect various autoantibodies	Autoantibodies to calcium channels	Autoantibodies to receptors atsetiholinovym
The defeat of the muscles of different parts of the body	Affects the muscles of the thigh and buttocks	Affects the eye muscles, throat, facial
Different m-response during rhythmic motor nerve electromyostimulation	m-increased response	progressive reduction of m-Answer
The difference of tendon reflexes	reflexes reduced	Reflexes are not changed
It noted the frequent development of the disease in people suffering from different pathologies	In patients with small cell lung cancer	In patients with thymoma

treatment with drugs

Myasthenia gravis (symptoms, the causes of the disease to help determine the therapeutic actions) can lead to severe forms of disability, therefore, absolutely necessary symptomatic treatment. It compensates for the pathological changes in the body and prevents the violation of vital functions (respiration and cardiac activity).

1. Anticholinesterase drugs (AHEP):

• neostigmine methylsulfate - can be used in tablets of 15 mg 2-3 times a day, or intramuscular injection of 0.05% solution.
  
• oksazil - administered in tablets of 5 mg, with a gradual increase in the daily dose of one or two or three tablets.
• Galantamine in kontsenratsii 1% - is administered subcutaneously in 0.5 ml 1-2 times a day.
• Kalimin - at the beginning of the disease designate 0.03-0.06 g up to two times per day. If necessary, your doctor may increase the dose. You can take no more than 0.72 g / day.
• analogs kalimina - pyridostigmine containing, for example, by Mestinon 0,06-0,18 g three times a day.

AHEP Overdose can cause cholinergic crisis.

This life-threatening condition is manifested by the following symptoms:

• restless behavior of the patient;
• constriction of the pupil (miosis);
• sweating, salivation;
• redness (flushing) of the skin;
• nausea, and other symptoms of dyspepsia (vomiting, intestinal colic, involuntary defecation);
• rapidly increasing weakness;
• bronchospasm, pulmonary edema, respiratory arrest;
• coma;
• decrease in blood pressure may collapse;
• paradoxical tachycardia or slowing of the heart, up to the stop.

Specific antidote (safener) AHEP - Atropine. A single dose of 0.001-0.002 g slowly administered intravenously. If necessary (if the pulse frequency is high) after 2-4 h infusion solution can be repeated.

2. Potassium chloride - designate the interior of 1 hour. l. 10% solution 4 / day. It enhances the action AHEP. Potassium orotate (providing the same effect) assume in tablets of 500 mg 3 / day.

3. glucocorticosteroids - used during exacerbation. Dose doctor selects individually an average dose of prednisolone - 1 mg / kg body weight per day for 7 days, then reduce the frequency - the same dose administered every second day.

Rapidly improve the condition of patients with human immunoglobulin G:

• Humaglobin;
• Octagam;
• Biaven;
• Whigs;
• Intraglobin.

Suppressing immune reaction in a dosage of 0.4 g / kg body weight per day. Improving briefly. The usual duration of 4-7 days. Repeated treatment with these drugs is prescribed in 3-5 months.

In some patients (2-4%), they cause side effects:

• temperature rise;
• nausea;
• headache.

4. Thioctacid and other drugs lipoic acid amplify synthesis processes in the mitochondria, and decrease the amount of substances that deplete the cell membrane (antioxidant effect). At the beginning of the treatment medication is administered by intravenous drip 0.7-0.9 g / d., After switching to reception of tablets inside at the same dose.

If the treatment is severe form of myasthenia these drugs ineffective, can be used cytostatics. The dose and the drug is determined by the physician individually in a hospital. Outpatient management of these patients is possible after stabilization of their condition.

You need to limit physical activity, sun exposure, stress. Patients magnesium contraindicated drugs, substances that increase the excretion of urine, neuroleptics, Curariform, tranquilizers, most antibiotics. The decision on the compatibility of drugs should be taken by the attending physician.

Crises require a special, emergency treatment and even resuscitation (tracheostomy, intubation, mechanical ventilation transition).

Surgery and radiation therapy

Diseases of the thymus gland (not only the tumor) found during the examination of the vast majority of patients with myasthenia gravis. Therefore, removal of the thymus (thymectomy) made in the early stages, improves overall, reduces the risk of crises, increases the average life expectancy.

Risk of surgery compared with the severity of the preoperative condition significantly decreased over the last 50 years.

The best results are obtained by complete removal of the thymus in patients whose condition is stable after the transition to maintenance doses AHEP. First, the body needs to accumulate a certain margin of safety. This reduces the likelihood of complications associated with surgery.

Besides neostigmine and analogues for 2-5 years before surgery appointed hormonal drugs, plasmapheresis, blood pH correction, antibiotic therapy and vitamin therapy. Thymectomy for the treatment of these patients used more than 100 years. Now the severe forms of the disease, especially the rapidly progressing, considered an absolute indication for surgery in the complex treatment.

The operation is performed under general anesthesia. To access the gland dissect the sternum (sternotomy). During the operation, the thymus gland is removed together with its surrounding fatty tissue. Complications during and after operations occur rarely, up to 10% of cases.

Are more likely to be described:

• suppurative complications;
• bleeding;
• myasthenic crises.

Complications may partly be due to the late diagnosis of the disease and bad sound treatment. Early postoperative period requires a stay of the patient in the intensive care unit of one to several weeks. No improvement was observed after thymectomy in patients with myasthenia condition is extremely rare (less than 1%).

Another method of surgical treatment of myasthenia, denervation of the carotid sinus, recently almost does not apply. There are reports of successful use of large doses of mediastinal and diencephalic area (midbrain), but this method has not been studied.

Folk remedies

Myasthenia gravis (symptoms, the causes must be a reason for examination of the patient) - a serious disease that can lead to disability and death. Even with a good medical compensation for signs of the disease the patient with myasthenia gravis should be considered seriously ill and throughout life to be under medical supervision.

Traditional medicine advised by muscular weakness give the patient oats, onions, garlic, lemons, flaxseed, honey, dried fruits.

You can use the following tools:

• onion with sugar (broth);
  
• chopped garlic with lemon, flaxseed and honey;
• dried fruits;
• decoction of oats.

Diet in infants

Patients with myasthenia nutritionists recommend foods rich in micronutrients involved in the conduction of an impulse from a nerve to the muscle fibers and muscle contraction.

Such a diet somewhat compensates painful changes in synapses:

• Potassium in large numbers contained dried fruits, melon, pumpkin, some legumes.
• Calcium is a person receives from dairy products, cabbage, asparagus, nuts, egg yolks.
• Phosphorus is needed for a more complete absorption of calcium contained in fish, meat, liver, buckwheat and pearl barley, nuts.

Patient with bulbar disorders will be easier to chew and swallow chopped food, drinking water. Prior to the opening AHEP myasthenia leads to rapid death of the majority of patients, regardless of the causes of the disease. Now available earlier diagnosis of symptoms that increases the effectiveness of treatment.

There are new drugs to compensate for the disease state, at least partially. They make it possible to extend the patient's life and improve its quality significantly. Thymectomy was significantly improves the patient's condition and prognosis for the future.

Registration of the article: Lozinski Oleg

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