Any crisis is a condition of a person in which the course of the disease suddenly worsens sharply, and life-threatening symptoms increase very quickly. Myasthenic and cholinergic crises, which are companions of myasthenia gravis, are dangerous because the patient may stop breathing and stop the heart. Sometimes a person's life is measured in literally minutes, during which doctors or people nearby must have time to provide the right assistance. Why is there an exacerbation of the seemingly non-deadly disease of myasthenia gravis? We offer a simple language that anyone can understand about what everyone should know: the causes of myasthenic and cholinergic crises, the clinic, emergency care for those who have had such a misfortune. Perhaps someone close to us, if he suddenly becomes ill in transport or just on the street, the information in this article will help save a life.
Myasthenia gravis
The story of the crisis will begin with an explanation of the concept of myasthenia gravis. It happens that others take this disease for a simulation, sincethose suffering from myasthenia constantly complain of fatigue, lethargy, unable to perform any physical work, only the lightest.
Actually, myasthenia gravis is a neuromuscular disease that belongs to the category of autoimmune, that is, caused by a failure in the body to produce the right antibodies or the production of killer cells that attack he althy tissues and cells, which becomes a big problem.
Myasthenic crisis develops against the background of a general disease and has symptoms similar to it, only manifested to a much greater extent, which previously led to the death of about 40% of patients. Now, if treatment is started without delay, death can be avoided. I would like to note that 10 people for every 100 thousand citizens of the Earth suffer from myasthenia gravis, and women suffer from it 3 times more often than men. Myasthenia can manifest itself already in childhood, but such cases are rare. It is mainly observed in people from 20 years old to extreme old age.
Myasthenia gravis symptoms
Without myasthenia gravis, if a person has it, a myasthenic crisis cannot occur. However, some other diseases with similar symptoms are sometimes mistaken for it, for example, such as the aforementioned lethargy, weakness, and increased fatigue. Additional symptoms in myasthenia gravis:
- drooping of the eyelids, most noticeable in the evening and decreasing in the morning after a night's rest;
- double vision;
- weakness, high fatigue after the usual loads for other people, for example, climbingsteps;
- initial bulbar signs (the appearance of a nasal voice after eating and a long conversation, difficulty in pronouncing individual letters);
- dynamics of bulbar signs (difficulty swallowing, frequent choking);
- vegetative disorders (intestinal paresis, tachycardia);
- mimic signs (very deep wrinkles on the forehead, characteristic facial expression);
- drooling;
- difficulty holding head;
- difficulty walking.
A distinctive feature of myasthenia is that all of the above manifestations increase after physical exertion and in the 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 experience a myasthenic crisis. Symptoms of the underlying disease, especially such as tachycardia, high fatigue of vital muscles (respiratory, cardiac), salivation, are intensified. Also, the crisis is characterized by the following manifestations:
- paralysis of the swallowing muscles and tongue, as a result of which mucus, saliva, food can enter the respiratory tract;
- suffocation;
- strong arousal and panic due to lack of air;
- cold sweat;
- sometimes spontaneous urination and/or defecation;
- loss of consciousness;
- dry skin;
- jumps in blood pressure;
- pupil dilation;
- acute cardiacinsufficiency, that is, disturbances in the work of the heart.
Myasthenic crisis comes in several degrees:
- easy;
- medium;
- heavy;
- lightning fast.
The difference lies in the strength of the above symptoms. A severe and lightning-fast crisis is especially dangerous, in which a person develops weakness of the respiratory and swallowing muscles very quickly, literally in a couple of minutes. Breathing at first becomes rapid, the face turns red, the pressure jumps up, the pulse reaches about 160 beats per minute. Then breathing begins to be interrupted, it may even disappear, the face turns blue (in medicine this is called cyanosis), the pressure drops, the pulse is almost not felt.
Causes of myasthenic crisis
Myasthenia can be both congenital and acquired. The first occurs due to mutations in genes. The second develops if a person has:
- thymus problems;
- some forms of cancer (particularly breast, lung, ovarian);
- thyrotoxicosis;
- lethargic encephalitis.
Against the background of these diseases, myasthenic crisis can develop in such cases:
- acute infectious diseases, including SARS, influenza, bronchitis;
- operations;
- strong psychological stress;
- high physical activity;
- taking certain medications (particularly tranquilizers);
- hormonal disorders;
- skipping pills for patients with myasthenia gravis, violation of the course of treatment.
Cholinergic crisis
Myasthenic crisis and cholinergic crisis often occur in parallel, which is why there are errors in differentiation and, as a result, in treatment. However, these two somewhat similar external manifestations of the condition are caused by different causes and have different etiologies.
Thus, during myasthenic crisis, the density of cholinergic receptors of the membrane decreases due to their destruction, and the rest change their functions. And with a cholinergic crisis, excessive activation of cholinergic receptors (nicotinic and / or muscarinic) occurs. This process is started by taking drugs for the treatment of myasthenia gravis in high doses, as well as drugs prohibited for this disease.
Diagnosing this crisis is not easy, since its main symptoms coincide with myasthenic. To help correctly determine what is happening to a person, such a feature in his condition, characteristic of a cholinergic crisis, can help: the patient has signs of intoxication: the stomach hurts, vomiting opens, diarrhea begins. Myasthenic crisis is characterized by everything but these symptoms.
The second feature of the cholinergic crisis is that the symptoms of myasthenia worsen without exercise, but after taking anticholinesterase drugs.
Mixed crisis
This is the most dangerous type of pathology for he alth and life. It combines a myasthenic and cholinergic crisis, presenting at once all that is noted duringboth states are symptomatic. This makes it difficult to diagnose correctly, but even more so - treatment, because those drugs that save from a myasthenic crisis aggravate the cholinergic crisis even more. In mixed crises, two phases of the flow are distinguished:
1. Myasthenic. Patients have pronounced bulbar disorders, breathing problems, physical activity causes fatigue, but taking drugs (Klamin, Prozerin) 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 one or another crisis with myasthenia gravis.
You can suspect a mixed crisis by the following features of manifestation:
- in patients, difficulty in breathing and bulbar disturbance are clearly observed, and the motor function of the limbs is little changed;
- taking drugs unevenly reduces pathological symptoms, for example, improves motor activity and almost does not help to stabilize breathing.
Diagnosis
In order not to make a mistake and quickly provide effective assistance in myasthenic crisis, it is important to correctly diagnose the patient. As noted above, some symptoms of a myasthenic crisis may be present in diseases that have nothing to do with myasthenia gravis (for example, difficulty breathing, failure of heart rhythms). The symptoms of a cholinergic crisis are similar to those that occur with intoxication and someproblems with the digestive tract. If there is an accompanying person with the patient who can give information about the presence of myasthenia gravis and the medications he is taking, the diagnosis is greatly simplified. To differentiate the type of crisis, doctors perform proserine test.
Special difficulties in diagnosing are observed in a 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 myasthenia gravis in a person (before the onset of a crisis) is detected using electromyography, computed tomography, pharmacological and immunological tests.
Emergency care for myasthenic and cholinergic crisis
If a patient with myasthenia gravis suddenly worsens (a crisis occurs), life counts for minutes. The main thing that others should do is to immediately call an ambulance. Unfortunately, in our reality there are situations when specialized assistance is late. How can you help a dying person in such a case? First, try to get him breathing, remove the mucus from his throat. According to the rules, those suffering from myasthenia should have a note about the presence of this disease, as well as medicines (for example, Prozerin) and a syringe. If there is no possibility of a quick arrival of an ambulance, a person with a myasthenic crisis must be given an injection according to the information in the note.
Armed doctors are obliged to urgently hospitalizethe patient, moreover, to the intensive care unit, where intensive emergency therapy is carried out:
- ensuring airway patency;
- oxygen supply;
- hardware artificial lung ventilation.
If the patient does not have symptoms of a cholinergic crisis (vomiting, diarrhea), the following drugs are administered: "Prozerin", "Atropine". If there are symptoms of intoxication, emergency therapy consists only in artificial ventilation of the lungs and in injections of such drugs: Atropine, Immunoglobulin, and some other medical products as indicated.
Treatment
If a person has a myasthenic crisis, treatment after emergency care is based on clinical and laboratory tests, analyzes and the dynamics of the disease. IVL (that is, artificial ventilation of the lungs), depending on the clinical picture of the patient's condition, as well as on indications of the presence of oxygen in the blood, can be carried out up to six days, but if the patient has a positive reaction to Prozerin after 16 or a little more hours, the IVL is canceled. In general, the ventilator procedure is very serious and responsible, requiring ongoing monitoring of respirators, % of gases in the blood, blood circulation, temperature, balance of fluids in the body and other things.
Exchange plasmapheresis is an excellent method for coping with all types of crises in myasthenia gravis. At the same time, blood is taken from the central (or ulnar) vein, it is centrifuged, the plasma is changed to donor orartificial. 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 indications, patients are prescribed immunoglobulins, antioxidants, anticholinesterase drugs, and in the presence of inflammatory processes - antibiotics.
Forecast and prevention
Thirty or forty years ago, deaths in patients with myasthenia gravis during an 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 must be provided very quickly. Therefore, if suddenly on the street, in transport, anywhere we see a person starting to choke, an ambulance should be called immediately.
Themselves patients with myasthenia should also follow a number of measures to prevent a crisis:
- be under the supervision of a doctor and strictly follow the prescribed treatment;
- avoid overwork, nervous breakdowns;
- stay away from infectious diseases as much as possible;
- do not expose your body to intoxication;
- include foods rich in potassium in the diet (for example, potato dishes, raisins).
