Any crisis is a condition of a person in which the course of the disease suddenly worsens, and life-threatening symptoms increase very quickly. Myasthenic and cholinergic crises, which are satellites of myasthenia gravis, are dangerous because the patient’s breathing may disappear and the heart may stop. Sometimes a person’s life is calculated literally in minutes, for which doctors or people nearby should have time to provide the right help. Why is there an exacerbation of a seemingly not deadly disease of myasthenia gravis? We offer a simple language that can be understood by anyone to talk about what everyone should know: the causes of myasthenic and cholinergic crises, the clinic, emergency care for those who have experienced such a disaster. Perhaps someone near us, if suddenly in a vehicle or just on the street he will feel bad, the information in this article will help save a life.
Myasthenia gravis
We begin the story of the crisis by explaining the concept of myasthenia gravis. It happens that people around take this disease as a simulation, since those suffering from myasthenia gravely complain of fatigue, lethargy, are not able 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 to produce the right antibodies or the production of killer cells that attack healthy tissues and cells, which becomes a big disaster.
Myasthenic crisis develops against the background of a general disease and has symptoms similar to it, only manifesting to a much greater extent, which previously led to the death of about 40% of patients. Now, if treatment is started immediately, death can be avoided. I would like to note that 10 people for every 100 thousand citizens of the Earth suffer from myasthenia, and women suffer from it 3 times more often than men. Myasthenia gravis can manifest itself in childhood, but such cases are rare. Basically, it is observed in people from 20 years old and to old age.
Symptoms of myasthenia gravis
Without myasthenia gravis, if a person has it, a myasthenic crisis cannot occur. However, it is sometimes taken for some other diseases with similar symptoms, for example, such as the aforementioned lethargy, weakness, and fatigue. Additional symptoms with myasthenia gravis:
- drooping eyelids, most noticeable in the evening and decreasing in the morning after a night's rest;
- double vision;
- fatigue, high fatigue after the usual loads for other people, for example, climbing stairs;
- initial bulbar signs (the appearance of a nasal voice after eating and a long conversation, difficulty in pronouncing individual letters);
- dynamics of bulbar symptoms (difficulty swallowing, frequent choking);
- vegetative disorders (intestinal paresis, tachycardia);
- facial expressions (very deep wrinkles on the forehead, characteristic facial expression);
- salivation;
- difficulties in holding the head;
- difficulty walking.
A distinctive feature of myasthenia gravis is that all of the above manifestations intensify after physical exertion and towards evening, and after a good rest they decrease 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), salivation, are intensified. Also for the crisis, the following manifestations are characteristic:
- paralysis of the swallowing muscles and tongue, as a result of which mucus, saliva, food can get into the respiratory tract;
- choking;
- strong excitement and panic due to lack of air;
- cold sweat;
- sometimes spontaneous urination and / or defecation;
- loss of consciousness;
- dry skin;
- jumps in blood pressure;
- dilated pupils;
- acute heart failure, that is, disturbances in the work of the heart.
Myasthenic crisis is of several degrees:
- easy;
- medium;
- heavy;
- lightning fast.
The differences are in the strength of the manifestation of the above symptoms. A severe and lightning crisis is especially dangerous, in which a person very quickly, literally in a couple of minutes, develops weakness of the respiratory and swallowing muscles. Breathing first becomes quickened, the face turns red, the pressure jumps up, the pulse reaches about 160 beats per minute. Then breathing begins to interrupt, it may disappear altogether, the face turns blue (in medicine this is called cyanosis), the pressure drops, the pulse is almost not felt.
Causes of myasthenic crisis
Myasthenia gravis can be congenital or acquired. The first occurs due to 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, myasthenic crisis can develop in such cases:
- acute infectious diseases, including acute respiratory viral infections, influenza, bronchitis;
- operations;
- strong psychological stress;
- high physical activity;
- taking certain medications (in particular, tranquilizers);
- hormonal disorders;
- admission of pills to patients with myasthenia gravis, violation of the course of treatment.
Cholinergic crisis
Myasthenic crisis and cholinergic often manifest in parallel, due to which there are errors in differentiation and, as a consequence, in treatment. However, these two states, somewhat similar in appearance, are caused by different reasons and have different etiologies.
So, during myasthenic crisis, the density of the cholinergic receptors of the membrane decreases due to their destruction, and the remaining ones change their functions. And with cholinergic crisis, excessive activation of cholinergic receptors (nicotinic and / or muscarinic) occurs. This process starts taking medications for the treatment of myasthenia gravis in high doses, as well as drugs prohibited for this disease.
It is not easy to diagnose this crisis, since its main symptoms coincide with myasthenic. To help correctly determine what is happening to a person, such a feature in his condition that is characteristic of a cholinergic crisis can: the patient has signs of intoxication: the stomach hurts, vomiting opens, diarrhea begins. Myasthenic crisis is characterized by everything except these symptoms.
The second feature of the cholinergic crisis is that the symptoms of myasthenia gravis worsen without physical exertion, but after taking anticholinesterase drugs.
Mixed crisis
This is the most dangerous type of pathology for health and life. It combines a myasthenic and cholinergic crisis, presenting immediately all the symptoms noted in both states. This makes it difficult to correctly diagnose, but even more difficult to treat, because those drugs that save from a myasthenic crisis, aggravate cholinergic even more. In mixed crises, two phases of the flow are distinguished:
1. Myasthenic . Patients have pronounced bulbar disorders, breathing problems, motor activity causes fatigue, but taking drugs (Klamin, Proserin) does not cause negative reactions.
2. Cholinergic , characterized by symptoms of intoxication.
Practice has shown that mixed crises most often occur in people who have already suffered a crisis in myasthenia gravis.
You can suspect a mixed crisis by the following features of the manifestation:
- in patients, difficulty in breathing and bulbar disturbance are clearly observed, and the motor function of the limbs is little changed;
- taking drugs unequally reduces pathological symptoms, for example, improves motor activity and almost does not help stabilize breathing.
Diagnostics
In order not to make a mistake and quickly provide effective assistance with a myasthenic crisis, it is important to correctly diagnose the patient. As noted above, some symptoms of myasthenic crisis may be present in diseases that have nothing to do with myasthenia gravis (for example, difficulty breathing, heart rhythm failure). Symptoms of a cholinergic crisis are similar to those that occur with intoxication and some problems with the digestive tract. If there is an accompanying person with the patient who is able to give information about the presence of myasthenia gravis and about the medications he is taking, the diagnosis is greatly simplified. To differentiate the type of crisis, doctors perform a proserin test.
Particular difficulties of diagnosis are observed with mixed crisis. In order to accurately determine its first phase, a clinical analysis of the patient’s condition is carried out, as well as an electrophysiological assessment of the effect obtained from taking anticholinesterase drugs.
The very presence of a person with myasthenia gravis (before the onset of a crisis) is detected by electromyography, computed tomography, pharmacological and immunological tests.
Emergency care for myasthenic and cholinergic crisis
If a patient with myasthenia gravis suddenly worsens (there is a crisis), life counts for minutes. The main thing that others should do is immediately call an ambulance. Unfortunately, in our reality there are situations when specialized assistance is late. How can one help a dying man in this case? Firstly, try to ensure his breathing, remove mucus from his throat. According to the rules, those suffering from myasthenia should have a note with them about the presence of this disease, as well as medicines (for example, Proserinum) and a syringe. If there is no possibility of a quick ambulance, a person with a myasthenic crisis needs an injection according to the information in the note.
The ripened doctors are urgently required to hospitalize the patient, and in intensive care, where intensive emergency therapy is carried out:
- ensuring airway patency;
- oxygen supply;
- hardware artificial ventilation.
If the patient does not have symptoms of a cholinergic crisis (vomiting, diarrhea), the following medications are administered: Proserinum, Atropine. In the presence of symptoms of intoxication, emergency therapy consists only in artificial ventilation of the lungs and in injections of such drugs: Atropine, Immunoglobulin, as well as some other medical devices as indicated.
Treatment
If a person has a myasthenic crisis, treatment after emergency care is carried out on the basis of clinical and laboratory tests, analyzes and the dynamics of the disease. Ventilation (that is, artificial ventilation of the lungs), depending on the clinical picture of the patient’s condition, as well as on the testimony of the presence of oxygen in the blood, can be performed up to six days, but if the patient has a positive reaction to Prozerin after 16 or more hours, the ventilation is canceled . In general, the mechanical ventilation procedure is very serious and responsible, requiring ongoing monitoring of respirators,% of the gases in the blood, blood circulation, temperature, balance of fluids in the body and others.
An excellent method to cope with all types of crises in myasthenia gravis is exchange plasmapheresis. At the same time, blood is drawn from the central (or ulnar) vein, centrifuged, and 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 in a course of 7 to 14 days.
One of the stages of treatment is drug therapy. According to the indications, patients are attributed immunoglobulins, antioxidants, anticholinesterase drugs, and in the presence of inflammatory processes, antibiotics.
Forecast and Prevention
Thirty or forty years ago, fatal outcomes in patients with myasthenia with exacerbation of the disease occurred quite often. Now mortality has been reduced by 12 times. You need to understand that sometimes the life of a person who has had a myasthenic crisis depends on our actions. Emergency care should be provided very quickly. Therefore, if suddenly on the street, in transport, anywhere we see a person starting to suffocate, an ambulance must be called immediately.
Patients with myasthenia gravis must also follow a number of measures to prevent a crisis:
- be under the supervision of a physician and strictly carry out the prescribed treatment;
- avoid overwork, nervous breakdowns;
- if possible, guard against infectious diseases;
- Do not expose your body to intoxication;
- include potassium-rich foods (e.g. potato dishes, raisins) in the diet.