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Myasthenia - chronic illness, characterized by weakness and pathological muscle fatigue, refers to autoimmune diseases. Many patients find changes thymus(tumors and hyperplasia of the gland tissue).
The development of the disease is characterized by a combination of neuroendocrine and immune deficiency. Pathological weakness caused by a violation of the neuromuscular impulse transmission. Clinic. More often and earlier the muscles innervated by craniocerebral insufficiency are affected. It is manifested by ptosis, doubling (diplopia), chewing, swallowing is difficult, weakness of the mimic muscles, neck, shoulder girdle, arms, chest appears.
In the morning the patient feels healthy. After a few hours, the eyelids droop, the voice becomes quiet, weak, speech is slurred. The face is amimic, the smile of "La Gioconda". The head hangs or tilts to the side, salivation is common, shortness of breath, weakness in the legs and arms after a slight physical exertion. Muscle tone is not disturbed, tendon reflexes are preserved, sensory disorders no.
Forms of the disease:
1 - generalized
2 - eye
3 - bulbar
4 - trunk
Under the influence external factors(infections, intoxications, physical, nervous – mental stress), a change in the dose of the drugs used is possible sharp deterioration with development myasthenic crisis(this is a sharp, paroxysmal developing muscle weakness with impaired respiratory function, cardiac activity).
Myasthenic crisis (5-10 minutes) - dangerous state- muscle weakness occurs sharply, the patient does not breathe on his own, does not speak, does not swallow saliva. A common cause is infections of the upper respiratory tract(bronchitis, pneumonia) in some cases, the cause remains unclear. The course of the myasthenic crisis is aggravated by the uncontrolled intake of anticholinesterase drugs (prozerin) by the patient.
Nurse tactics:
1. call a doctor or SP.
2. Provide physical and emotional rest.
3. Give the head an elevated position.
4. When breathing and cardiac activity stop - closed heart massage.
5. Perform mechanical ventilation when breathing is weakened.
6. Prepare prozerin for the arrival of the doctor.
Treatment for myasthenic crisis: Prozerin 1-2 ml IV. The effect occurs after 30 minutes and lasts 2 hours. AT interictal period: prozerin (0.15), kalemin are taken orally; the effect after 1 hour lasts 2-4 hours.
Patients with myasthenic crisis are usually hospitalized in the department intensive care. It is necessary to ensure the patency of the respiratory tract (remove mucus, vomit from the mouth, make sure that there is no retraction of the tongue). In the hospital, they are transferred to a ventilator to avoid death from respiratory failure. Then they urgently take a common and biochemical analysis blood (to detect signs of inflammation, exclude hypokalemia, and other metabolic changes). If the patient shows signs of infection, antibiotics (cephalosporins) are added. Assign plasmapheresis.
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Some nervous diseases at the initial treatment of patients by doctors, it is quite difficult to diagnose. These diseases include myasthenia gravis. Initial complaints, voiced by the patient, of rapid fatigue. But after rest, muscle fatigue on short time recedes, and the patient again feels quite normal.

Meanwhile, myasthenia gravis is a violation of the neuromuscular transmission of signals from the central nervous system into striated muscles, resulting in abnormal cyclic fatigue after minor exertion.

Information about the disease

There are immune-dependent myasthenia gravis and myasthenic syndromes.

The cause of the first is autoimmune diseases, the development of syndromes is due to a combination of developmental defects: postsynaptic and presynaptic.

These defects are nothing more than violations of the synthesis necessary for normal life organism of substances and defect of organs of perception. Due to pathologies of organic processes, the function of the thymus gland is disrupted.

Push to launch autoimmune diseases or violations biochemical processes in the body can be all the factors that weaken immune status, namely infectious diseases, stress or injury.

Can be distinguished the following forms myasthenia:

  • eye;
  • bulbar;
  • generalized.


Some physicians believe that myasthenia gravis is combined with the eye, and how separate state it is not classified.

The first symptoms of myasthenia gravis eye shape- damage to the muscles of the eyelids. Patients complain of drooping eyelids, rapid eye fatigue, doubling of the image.

Then the signs of bulbar myasthenia join - the pharyngeal muscles, also innervated by the cranial nerves, atrophy.

Chewing and swallowing functions are violated, in the future the timbre of the voice will change, the ability to articulate speech will disappear.

With generalized myasthenia gravis, all muscles are gradually blocked - from top to bottom - from the cervical and scapular to dorsal, then the muscles of the limbs are affected. Salivation appears, it becomes difficult for the patient to serve himself, perform the simplest actions, there is a feeling of weakness in the limbs.


The increase in symptoms can stop at any stage.

In children, the disease does not manifest itself before the age of six months, in most cases it is diagnosed in boys older than 10 years. They have up to 2 years from the first symptoms - weakness of the muscles of the eyelids - to the next ones.

In adulthood, in the period from 20 to 40 years, women are more likely to get sick, and at the age of over 65, the manifestation of the disease no longer depends on gender.

Types of myasthenic syndrome

There are several myasthenic syndromes caused by genetic defects.

All of them are inherited in an autosomal recessive manner, except for the autosomal dominant syndrome, which is caused by the slow closing of immune channels:


  1. Lambert-Eaton syndrome is more often diagnosed in men over 40 years of age. Its main features are weakness of the proximal limb muscles while maintaining the bulbar and extraocular muscles. Symptoms may precede clinical manifestations, at physical activity- playing sports, muscle weakness can be stopped;
  2. Congenital myasthenic syndrome. Signs - violation of symmetrical movement eyeballs and ptosis of the eyelids;
  3. Symptoms - weakness of the facial and skeletal muscle, the sucking function is impaired;
  4. Muscle hypotension and underdevelopment of the synaptic apparatus causes a rare myasthenic syndrome, in which tendon reflexes are reduced. Typical signs conditions - asymmetry of the face, mammary glands and torso;
  5. Myasthenic syndromes can be caused by taking certain drugs: D-penicillamine and antibiotics: aminoglycosides and polypeptides. Improvement occurs 6-8 months after discontinuation of the drug.

Syndrome due to slow closure of ionic channels has the following symptoms:

  • weakness of the oculomotor muscles;
  • muscle atrophy;
  • weakness in the limbs.

Treatment of each case of myasthenia gravis is carried out according to a specific algorithm.

Can be applied:

  • corticosteroids;
  • antibiotics;
  • anticholinestazor drugs;
  • plasmapheresis and other types of specific therapy.

Drugs used in one form of the disease are ineffective for other forms.

myasthenic crisis


The main symptoms of a myasthenic crisis are extensive dysfunctions of the bulbar muscles, which include the respiratory one, up to the onset of apnea.

The intensity of symptoms increases at a critical rate - brain hypoxia can occur within half an hour.

If emergency care is not provided for a sudden myasthenic crisis, the patient will suffocate.

The reasons for the development of the crisis can be the following factors:

  • stressful conditions;
  • increased physical activity;
  • flu;
  • acute respiratory diseases;
  • violations of metabolic processes;
  • intoxication of various etiologies.

These factors lead to blockage neuromuscular conduction cause loss of excitability in muscles and tendons.

Patients with myasthenia always have a note with them, which says that they suffer from such and such a form of the disease and what drugs are needed for first aid. In most cases, patients carry drugs with them - these are prozerin and canevin.

If among those around, in whose eyes a myasthenic crisis developed, there is at least 1 person who knows how to inject, a person's life will be saved. But you still need to call an ambulance.

Crisis treatment


The method of treatment of a myasthenic crisis is completely determined by the patient's condition and how quickly emergency care was provided to the specialist who arrived at the place of the call. brigade.

As soon as possible, the victim must be placed in intensive care and connected to a ventilator - artificial respiration. artificial ventilation lung should be produced within 24 hours, no less.

Effectively restores the state of plasmapheresis, but may be required intravenous administration immunoglobulin. Together with immunoglobulin, methylprednisolone and potassium chloride are used.

Joint treatment with immunoglobulin, potassium chloride and methylprednisolone is used in the presence of a history of inflammatory processes.

Antioxidants are used lipoic acid in particular. They reduce the amount free radicals accumulated in the blood, reduce the severity of oxidative stress in the body of patients.

Myasthenic crisis (hereinafter - MK) is a life-threatening condition that is characterized by impaired breathing and swallowing to the extent that compensation is impossible without a set of intensive care and resuscitation measures (including intensive ventilation of the lungs). According to the literature, a crisis course is observed in 30-40% of patients with myasthenia gravis and is more common in women.

Molecular basis MK is probably a sharp decrease in the number of functioning acetylcholine receptors (hereinafter referred to as AChR) due to a massive attack by their autoantibodies.

Differentiate MK from others severe conditions accompanied by respiratory disorders, it is possible by the presence bulbar syndrome, hypomimia, ptosis, asymmetric external ophthalmoparesis, weakness and fatigue of the muscles of the limbs and neck, decreasing in response to the introduction of acetylcholinesterase inhibitors (hereinafter referred to as AChE).

There is an idea that the highest incidence of MC occurs in the first 2 years from the onset of the disease, while there is a group of patients in whom the manifestation of myasthenia MC debuts. In the literature, there are cases of the development of MC, accompanied by severe respiratory failure, as the first manifestation of myasthenia gravis (most often with a "late" onset of the disease). trend towards acute development myasthenia gravis in the elderly was described by K. Osserman, who identified these patients in a separate group in his classification as acute "fulminant" malignant form with late onset of the disease and early atrophy. Currently, many authors note the clinical evolution of myasthenia gravis and a tendency to a significant "aging" of the disease. Thus, the researchers note that approximately since the mid-80s of the XX century, the incidence in young age and 3 times increased in old age. This fact underlies one of the actual problems diagnosis of myasthenia gravis in the elderly: currently, according to statistics, 4 out of 5 patients with acute onset of myasthenia in late age a diagnosis of stroke, botulism (or polymyositis) is made. In most cases it is possible to determine trigger factor or a combination of several factors leading to the development of crises, however, there is also a "sudden" onset of crises for no apparent reason.

Many authors point to the polyetiology of MC development, on the one hand, and the absence of any visible reasons crises in some patients with myasthenia gravis, on the other. The literature describes whole line factors (exogenous and endogenous) that can provoke the development of exacerbation and MK. Among the most common causes of MC, according to domestic and foreign authors: upper respiratory tract infection (10-27% of cases), aspiration (bacterial) pneumonia (10-16%), surgical intervention– thymectomy (5 - 17%), start of treatment high doses steroids or their cancellation (2 - 5%), pregnancy and childbirth (4 - 7%); in 35 - 42% of cases they do not find etiological factor crisis.

In most cases, MC comes on suddenly and develops rapidly, leaving no time to change treatment tactics and prevent them, so it is important to assess the significance clinical symptoms, immunological, electrophysiological and pharmacological characteristics, which could serve as criteria for predicting the development of MC.

The crisis nature of myasthenia gravis can be predicted already in the onset of the disease. Reliable predictors of a malignant course are mimic weakness, bulbar and respiratory disorders, weakness of the muscles of the neck and hands (a symptom of "sagging" 3-5 fingers of the hands) and the absence of "classic" oculomotor disorders (double vision) and proximal weakness of the limbs in the clinical pattern of myasthenia gravis onset (moreover, a similar selective pattern, having formed early, persists throughout throughout the course of the disease and is observed in patients with the most severe course diseases resistant to the main species pathogenetic therapy- GCS, thymectomy (often during the development emergency patients retain the characteristic selectivity of the defeat of individual muscle groups, having the so-called "partial" type of crisis).

According to the result of the study (from 1997 to 2012) N.I. Shcherbakov et al. (FGBU " Science Center neurology" RAMS, Moscow):

Crises in patients with myasthenia often develop in the first year from the onset of the disease, which is primarily due to late diagnosis and the lack of adequate pathogenetic therapy. The development of the crisis in more late dates diseases are significantly more often associated with the abolition or reduction of the dose of basic corticosteroid therapy, which reflects the objective difficulties in selecting the minimum maintenance dose of steroids.

MC predominate in women at a young age, in men over 60 years of age. With a "late" onset of the disease, the likelihood of developing crises in women and men is the same.

A feature of myasthenia gravis in the elderly is a tendency to an acute fulminant debut of the disease, up to manifestation with MC, which, as shown clinical practice often leads to misdiagnosis.

Important predictors of a malignant "crisis" course of the disease should be considered the presence of a selective craniobulbar clinical pattern, the combination of myasthenia gravis with thymoma, and the lack of effect on thymectomy in young people. age groups(up to 40 years), the presence of antibodies (AT) to MTK (specific muscle tyrosine kinase), poor sensitivity of patients to acetylcholinesterase inhibitors.

Titer (concentration) of AT to AChR has no predictive value in determining the severity of the disease. Detection of antibodies to MTK in the serum of patients indicates a high risk of developing a malignant crisis course of the disease.

Electrophysiological criterion high risk MK is a decrease in the amplitude of the M-response to the first electrical stimulus in a clinically affected muscle in combination with small degree decrement (block of neuromuscular transmission) that does not correspond to the severity of paresis.

Myasthenic crisis is manifested by life-threatening weakness of the respiratory and bulbar muscles, leading to respiratory arrest.

Treatment. During a crisis, first of all, it is necessary to ensure the patency of the respiratory tract, remove mucus from the pharynx using an electric suction or using the body position with the head down. Then it is necessary to carry out IVL by all accessible ways. The use of masks during mechanical ventilation in patients is not recommended due to the existing dysphagia. For the speedy relief of the crisis, proserin is administered - 0.5-1.5 mg (1-3 ml of a 0.05% solution) intravenously with a preliminary injection of 0.6 mg of atropine (proserin administered intravenously can cause cardiac arrest), after 15 minutes 0.5 mg of prozerin is administered subcutaneously. Perhaps the introduction of 60 mg of neostigmine (prozerin) into the endotracheal tube or 2.5 mg intramuscularly. Throughout critical period mandatory drainage of the respiratory tract. As an additional help, prednisolone 90-120 mg per day, immunoglobulin 0.4 mg per day, cardiotonic and symptomatic agents are used.

Plasmapheresis and hemosorption are used to remove autoantibodies to acetylcholine receptors from the body.

The cholinergic crisis, as a result of an overdose of anticholinesterase drugs (prozerin, neostigmine, kalimin, mestinon), is manifested by miosis, excessive salivation, bronchospasm, sweating, agitation, muscle weakness, abdominal pain, diarrhea, fibrillations and fasciculations.

When diagnosing this condition, all anticholinesterase drugs are immediately canceled, 1-2 mg (1-2 ml of a 0.1% solution) of atropine is administered subcutaneously or intravenously every hour, controlling the diameter of the pupils. IVL is carried out with intermittent positive pressure through the endotracheal tube, sanitize the airways.

Traumatic brain injury, according to the severity of the injury, is divided into concussion, contusion, and contusion with compression, which determines the tactics and extent of rendering emergency care.

Treatment. All patients with traumatic brain injury inpatient treatment and observation. With the preservation of vital functions, the patient must be provided with peace, cold on the head, symptomatic therapy(painkillers, sedatives, hypnotics) and correction of microcirculatory disorders (venotonics, vasoactive drugs, antiplatelet agents). expedient early appointment antibiotic therapy.

The use of neuroleptics and narcotic analgesics for prehospital stage and before exclusion intracranial hematoma Not recommended.

Gradual depression of consciousness is usually a sign of intracranial hematoma, compression, edema, or herniation of the brain, which require surgical intervention, hemostatic therapy (intravenously, 1 ml of 12.5% ​​solution of dicynone or etamsylate with an interval of 6 hours) and dehydration therapy. Development psychomotor agitation, hyperthermia, coma requires non-specific emergency treatment.

Ed. prof. A. Skoromets

"Myasthenic crisis" and other articles from the section

(c. cholinergica) K., due to an overdose of anticholinesterase drugs (for example, in the treatment of myasthenia gravis), characterized by weakness, arterial hypotension, bradycardia, miosis, vomiting, salivation, convulsions and fasciculations.

  • - sudden momentary 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 increased 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 ...

    Big medical dictionary

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

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

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  • - ; pl. kri/zy, R....
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  • - -and husband. . sudden onset acute attack diseases...

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  • - crisis m. Sudden sharp deterioration of the patient's condition against the background of an existing disease; attack...

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  • - CRISIS a, m. CRISIS s, f. CRISIS a, m. crise f., lat. crisis. 1. dipl. A difficult situation, a condition requiring a decisive turn, a fracture. Sl. 18. A good way to get rid of some obligations that ....

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