How does the myasthenic crisis manifest? Treatment

Any crisis is a state of a person in which the course of a disease suddenly and sharply deteriorates, and the life-threatening symptomatology grows very quickly. Myasthenic and cholinergic crises, which are the companions of myasthenia gravis, are dangerous because the patient can lose his breath and stop the heart. Sometimes a person's life is calculated literally by minutes, for which doctors or people in the vicinity should be in time to provide the proper help. Why is there an exacerbation of a seemingly non-deadly myasthenia gravis? We offer a simple, understandable language for anyone to talk about what everyone should know: the causes that cause myasthenic and cholinergic crises, a clinic, emergency help for those with whom such a disaster happened. Perhaps someone near us, if suddenly in transport or just on the street it becomes bad, the information in this article will help save lives.

Myasthenia gravis

The story of the crisis begins with an explanation of the concept of myasthenia. Sometimes, others take this disease for simulation, since those suffering from myasthenia constantly complain of rapid fatigue, lethargy, unable to perform any physical work, only the easiest. In fact, myasthenia gravis is a neuromuscular disease that belongs to the category of autoimmune, that is, caused by a malfunction in the body producing the right antibodies or the production of killer cells attacking healthy tissues and cells, which becomes a big disaster.

The myasthenic crisis develops against the background of a common disease and has similar symptoms, only manifesting to a much greater extent, which earlier led to the death of approximately 40% of patients. Now, if treatment begins without delay, a lethal outcome can be avoided. I would like to note that myasthenia graze 10 people for every 100 thousand citizens of the Earth, and women suffer it 3 times more often than men. To manifest myasthenia gravis can already in childhood, but such cases are rare. In general, it is observed in persons from 20 years old and to old age.

Symptoms of myasthenia gravis

Without myasthenia gravis, if it is present in a person, a myasthenic crisis can not occur. However, sometimes other diseases with similar symptoms, for example, such as the aforementioned lethargy, weakness, increased fatigue, are sometimes taken for him. Additional symptoms with myasthenia gravis:

- the lowering of the eyelids, most noticeable by evening and decreasing in the mornings after an overnight rest;

- double vision;

- exhaustion, high fatigue after normal for other people loads, for example, climbing the stairs;

- initial bulbar signs (appearance of a nasal voice after eating and a long conversation, difficulty in pronouncing individual letters);

- dynamics of bulbar signs (difficulty in swallowing, frequent pinches);

- vegetative disorders (intestinal paresis, tachycardia);

- mimic signs (on the forehead very deep wrinkles, characteristic facial expression);

- drooling;

- difficulty in keeping the head;

- difficulties in walking.

Distinctive feature of myasthenia gravis - all the above manifestations increase after physical exertion and in the evening, and after a full rest are reduced or completely disappear.

Symptoms of myasthenic crisis

If a person suffers from myasthenia gravis, under certain circumstances he may have a myasthenic crisis. Symptoms of the underlying disease, especially such as tachycardia, high fatigue of vital muscles (respiratory, cardiac), drooling, while increasing. Also for the crisis characterized by the following manifestations:

- paralysis of swallowing muscles and tongue, as a result of which mucus, saliva, food can enter the respiratory tract;

- suffocation;

- a strong excitement and panic due to lack of air;

- cold sweat;

- sometimes spontaneous urination and / or defecation;

- loss of consciousness;

- dry skin;

- blood pressure jumps;

- dilated pupils;

- acute heart failure, that is, a violation in the work of the heart.

Myasthenic crisis occurs in several degrees:

- light;

- medium;

- heavy;

- lightning fast.

Differences are in the strength of the manifestation of the above symptoms. Especially dangerous are the severe and lightning crisis, in which a person quickly, literally in a couple of minutes, develops a weakness of the respiratory and swallowing muscles. Breathing first becomes rapid, the face turns red, the pressure jumps up, the pulse reaches about 160 beats per minute. Then the breath begins to break, it may disappear altogether, the face turns blue (in medicine this is called cyanosis), the pressure drops, the pulse is hardly probed.

Causes of myasthenic crisis

Myasthenia gravis can be both congenital and acquired. The first arises because of mutations in the genes. The second develops if a person has:

- problems with the thymus gland;

- Some forms of cancer (in particular, breast, lungs, ovaries);

- thyrotoxicosis;

- lethargic encephalitis.

Against the background of these diseases, the myasthenic crisis can develop in such cases:

- acute infectious diseases, including SARS, influenza, bronchitis;

- operations;

- strong psychological stress;

- high physical load;

- taking certain medications (in particular, tranquilizers);

- hormonal disorders;

- admission of patients with myasthenia gravis receiving pills, violation of the course of treatment.

Cholinergic crisis

Myasthenic crisis and cholinergic often manifest themselves in parallel, because of which there are errors in differentiation and, consequently, in treatment. However, these two somewhat similar external manifestations of the state are caused by different causes and have different etiologies.

Thus, with the myasthenic crisis, the density of the cholinergic receptors of the membrane decreases because of their destruction, and the remaining ones change their functions. And with the cholinergic crisis, excessive activation of the cholinergic receptors (nicotinic and / or muscarinic) occurs. This process is started by the administration of drugs for the treatment of myasthenia gravis in elevated doses, as well as the means banned in this disease.

Diagnosing this crisis is not easy, since its main symptoms coincide with the myasthenic. To help correctly determine what happens to a person, such a feature in his condition, characteristic of a cholinergic crisis, can appear: the patient has signs of intoxication: the stomach hurts, vomiting opens, diarrhea begins. For the myasthenic crisis, everything is common, except for these symptoms.

The second feature of the cholinergic crisis is that myasthenia gravis symptoms worsen without physical exertion, but after taking anticholinesterase drugs.

Mixed crisis

This is the most dangerous for health and life pathology. It combines the crisis of myasthenic and cholinergic, presenting immediately all the symptoms noted in both states. This makes it difficult to correctly diagnose, but even more - treatment, because those drugs that save from the myasthenic crisis, cholinergic aggravate even more. With mixed crises, two phases of flow are distinguished:

1. Myasthenic . Patients have pronounced bulbar disorders, breathing problems, motor activity causes fatigue, but the administration of drugs ("Clamin", "Proserin") does not cause negative reactions.

2. Cholinergic , characterized by symptoms of intoxication.

Practice has shown that mixed crises often occur in people who have already suffered a crisis in myasthenia gravis.

To suspect a mixed crisis is possible on such features of manifestation:

- in patients, breathing difficulties and bulbar disorders are clearly observed, and the motor function of the extremities is changed little;

- taking drugs unequally reduces pathological symptoms, for example improves motor activity and almost does not help to stabilize breathing.

Diagnostics

In order not to make a mistake and quickly provide effective help with a myasthenic crisis, it is important to correctly diagnose the patient. As noted above, some symptoms of the myasthenic crisis may be present in diseases that have nothing to do with the myasthenia gravis (eg, difficulty breathing, cardiac rhythm failure). The symptomatology of the cholinergic crisis is similar to that seen with intoxication and some problems with the digestive system. If there is an accompanying person with the patient who is able to give information about the presence of his myasthenia gravis and about the medications he takes, the diagnosis is much simplified. To differentiate the type of crisis, doctors perform a proserin test.

Special difficulties in diagnosing are observed with a mixed crisis. For the purpose of an unmistakable determination of its first phase, a clinical analysis of the patient's condition is carried out, as well as an electrophysiological evaluation of the effect obtained from taking anticholinesterase medications.

The very presence of a male myasthenia gravis (before the onset of a crisis) is detected with the help of electromyography, computed tomography, pharmacological and immunological tests.

Emergency care for myasthenic and cholinergic crisis

If the patient myasthenia suddenly suddenly worsened state (there was a crisis), the account of life goes for a minute. The main thing that others should do is immediately call an ambulance. Unfortunately, in our reality there are situations when specialized assistance is late. In what way can you help a dying person? First, try to ensure his breathing, remove the mucus from his throat. According to the rules, those suffering from myasthenia gravis should have a note about the presence of this disease, as well as medicines (for example, "Proserin") and a syringe. If there is no possibility of a quick arrival, the person with a myasthenic crisis needs to be injected according to the information in the note.

Prepared physicians must urgently hospitalize the patient, and in intensive care, where intensive emergency therapy is carried out:

- ensuring airway patency;

- supply of oxygen;

- hardware artificial ventilation.

If the patient has no symptoms of a cholinergic crisis (vomiting, diarrhea), the following drugs are administered: "Prozerin", "Atropine". In the presence of symptoms of intoxication emergency therapy is only in the artificial ventilation of the lungs and in injections of such drugs: "Atropine", "Immunoglobulin", as well as some other medical products according to indications.

Treatment

If a person has a myasthenic crisis, treatment after emergency treatment is performed based on clinical and laboratory tests, analyzes and dynamics of the disease. Ventilation (i.e., artificial ventilation of the lungs) depending on the clinical picture of the patient's condition, as well as on the presence of oxygen in the blood can be conducted for up to six days, but if the patient has a positive reaction to Prozerin after 16 or more hours, the ventilator is canceled . In general, the procedure of ventilation is very serious and responsible, requiring continuous monitoring of respirators,% composition of gases in the blood, circulation, temperature, balance of fluids in the body and other things.

An excellent method, coping with all types of crises in myasthenia, is exchange plasmapheresis. In this case, blood is taken from the central (or ulnar) vein, it is centrifuged, the plasma is changed to donor or artificial. This method gives excellent results - in a couple of hours the patient's condition improves significantly. Plasmapheresis is performed for 7 to 14 days.

One of the stages of treatment is drug therapy. According to the indications, immunoglobulins, antioxidants, anticholinesterase drugs are attributed to the patients, and antibiotics if there are inflammatory processes.

Prognosis and prevention

Thirty-forty years ago, lethal outcomes in myasthenia patients with exacerbation of the disease occurred quite often. Now the mortality rate has been reduced by 12 times. It must be understood that sometimes the life of a person depends on our actions with you, who has a myasthenic crisis. Emergency care must be provided very quickly. Therefore, if suddenly on the street, in the transport, anywhere we see a person beginning to suffocate, the ambulance should be called immediately.

The patients with myasthenia gravis should also follow a number of measures to prevent a crisis:

- to be under the supervision of a doctor and strictly follow the prescribed treatment;

- avoid overwork, nervous breakdowns;

- if possible, be protected from infectious diseases;

- do not expose your body to intoxication;

- in the diet include foods rich in potassium (for example, potato dishes, raisins).