Myotonia is a hereditary disease that is characterized by injury and as a result of abnormal development of the striated musculature, which lead...
Myasthenic crisis is a condition requiring immediate help
Myasthenic crisis: am I trembling creature or have the right? !
But if in the mouth of the King-father it is permeated with pride for the victorious son, then for the hearing of the neuropathologist it is just a patient complaint, meaning literally: muscles, my muscles! In the sense: my poor muscles!
After all, "myo-" is the first part of any compound word that relates to muscles in general and muscular pathology, in particular. To such as myasthenia gravis.
- What is this state and why does it generate crises?
- Non-random chance, or occasions for the crisis
- Tremble, ventricle - play a crisis!
- The diagnosis is obvious, the symptoms are on the face
- Differentiation from other deviations
- When emergency help is needed
- Further therapy after an arrest of an attack
- Forecast for life, or Hasta la vista, Myasthenia!
What is this state and why does it cause crises?
Myasthenia gravis pseudoparalitica, or asthenic bulbar paralysis, or simply my
Other name: myasthenic pseudoparachy. That is, the rapidly progressive pathological fatigue of the musculature innervated by the cranial nerves, with the possibility of involving respiratory muscles and other muscle groups.
A disease characterized by a hereditarily conditioned state of immunity with the development of aggression towards the cellular structures of one's own body.
More precisely, autoaggression, because the body is protected from them, forming antibodies - it perceives them as alien entities, with the emergence of a "full serious" reaction "antigen-antibody".
And this reaction, aimed at fighting against one's own organism, arises after living the most banal life situations.
For example, the elevator refused, and the suffering myasthenia had to go through a couple of flights of stairs with their own feet. Another would catch his breath - and forget everything, but myasthenic death may come. .. from overwork.
For in the autoimmune reaction, not only the muscles of the eyes, lips, face, pharynx, tongue and neck may be involved, but also any muscles in general. Therefore, elementary walking on the stairs can lead to the most unpredictable consequences, up to direct damage to the heart.
The mechanism of the development of the neuromuscular blockade is simple. Due to the formation of antibodies, the destruction of postsynaptic membranes of myocardiocytes begins together with the cholinoreceptors "soldered" in them - this is quite enough for the development of acute heart failure.
However, such a situation arises rarely, because the patient knows his capabilities and carefully calculates forces in a situation where others "take the storm."
But even in conditions of quiet, measured life, a patient after night's sleep is difficult to raise "lead" eyelids, after an inadequate physical exercise, his legs fall, fall off due to fatigue of the hand, fatigue of the tongue with difficulty in pronouncing words, yesand swallowing is hard.
And his day, without going half way, ends with "fallen" in the eyes of fatigue for centuries and "trembling" from the weakness of his legs. Or shortness of breath, coughing, shuffling. Either all a little.
And this is not a simulation, nor is it natural laziness. These are signs of bulbar disorders of asthenic properties - signs of rapid depletion of the chemistry of neuromuscular synaptic transmission, the mechanism of violation of which can be confirmed by the conduct of drug tests. The mechanism of the development of myasthenia gravis
Non-random accident, or occasions for the
crisis The reason for the myasthenia itself is the genetic "whim" of nature, which the body "plays along" allowing the appearance of hyperplasia or thymus tumors, or allowing the existence of amyotrophic lateral sclerosis, polydermatomyositis, lung cancer, ovary,dairy or prostate.
Factors that trigger the onset of the myasthenic crisis are:
- introduction into the body of foreign protein ( development of acute infectious - especially viral - process or exacerbation of chronic);
- effects of stress( mental or physical), as well as physical injuries( including surgical interventions);
- reception of neuroleptics, tranquilizers with the effect of muscle relaxation ( including medications not prescribed by the treating doctor, either - prescribed but incorrectly taken when inadequate doses are allowed or if there are gaps in the drug intake);
- "special periods" in the life of a woman ( PMS with its hormone-immune "squalls").
Tremble, ventricle - play a crisis!
The myasthenic crisis is a "peak condition" during the course of the disease, which, if the timely( resuscitative) care of fails to cause a fatal outcome, causes profound disorders of vital processes in the body.
It always develops unexpectedly, having the appearance of an acutely increasing respiratory insufficiency against the background of the progressive weakness of the generalized character that developed in large muscle groups( due to the development of paresis in them).
These groups can be:
- muscles of the pharynx , swallowing and preventing ingress of food or mucus into the respiratory tract( with asphyxiation asphyxia);
- muscles serving the larynx ;
- acting in accordance with the musculature of the diaphragm and providing the intercostal muscles for inspiration .
Depending on the time of the course and the degree of severity of the patient's condition, the crises have a gradation from mild and moderate to severe and lightning.
With the usual "scenario" there is a progressive increase in the sudden development of respiratory failure, and the process takes just a few minutes.
Along with the appearance of a feeling of "lack" of air and the sensation of anxiety and general excitement caused by this, there is an increase in respiration with face hyperemia, a pulse rate of 160( or more) beats per minute and an increase in systolic blood pressure to 200( or more) mm Hg.
Then the frequency and activity of respiratory movements decreases - breathing is reduced and becomes superficial, reaching the appearance of complete cessation. In connection with the progression of hypoxia, the color of the skin becomes cyanotic( cyanotic), AD hardly reaches the figure of 80 mm Hg, the pulse is determined with great difficulty or reaches a "filiform".
Consciousness due to the onset of acute hypoxia of the brain acquires the character of "confused", then it is lost.
In addition to the violation of basic life functions, the appearance of aphonia, dysphagia, dysarthria, vegetative disorders such as mydriasis, intestinal and bladder paresis with involuntary defecation and urination, flowing saliva from the corner of the mouth, protrusion of cold sticky sweat on the skin also join.
Isolated or combined myocardial damage also can lead to death due to onset of acute heart failure - either because of myocardial infarction or due to severe heart rhythm disorder( flutter and fibrillation of the ventricles, which are the main cause of sudden cardiac death).
The diagnosis is obvious, the symptoms are on the face of
. The doctor-neurologist, who is "sharpened" to recognize this pathology, is unable to diagnose the difficulties due to the classic appearance, movements, gait and speech of the myasthenia sufferer.
A physician therapeutist, seeing a carrier rare enough( from 5 to 10/100 thousand people) for the first time, will think about an acute disorder of cerebral circulation( in the vertebrobasilar basin), a brainstem tumor, or one of the forms of meningitis with a disorder of oculomotor functionsnerves.
Fallen( or almost "fallen") on the eyes of the eyelids, disorders of eyeball movements with the appearance of diplopia and strobism( strabismus);Difficulty of speech( "porridge in the mouth" when pronouncing words), "nasal" voice from rapid fatigue of the muscles of the tongue and palate;a drooping "dangling" head, which the neck does not hold;immense fatigue, even from a brief conversation and a more or less prolonged fixation of the look( not to mention the fatigue of chewing food and reading) are quite typical symptoms of the myasthenic crisis.
Confirm the assumption of myasthenia gravis and anamnestic information.
This is a chronic character of the disease with a steady( without treatment) progression and a tendency to spread pathology to all new muscle groups( on the trunk, limbs right up to the development of tetraplegia).
And ultimately - with the transition from local( with myasthenic episodes) to a generalized form leading to generalized weakness and development of myasthenic states( periods of stable manifestations that have significant periods) and crises.
In favor of myasthenia, there is an increase in the manifestations of the disease in the second half of the day, a clear link between the increase in weakness with physical exertion or the impact on the nervous system.
In view of the fact that such a patient can not get to the polyclinic on his own, all the questions are addressed to the person accompanying the myasthenic, who is well acquainted with all the nuances of his life and details of the treatment.
Objective research reveals the dynamic lability of symptoms - their amplification when trying to fix sight and reading, a clear outline of the manifestations of muscle groups located in the sphere of cerebral nerves( at the beginning of the disease or at its local form).
With stimulated electromyography, the normal value of the total evoked potential of the action of the muscle being examined is noted with a decrease in the amplitude at a rhythm of stimulation with a frequency of 3-5 and 50 pulses / sec.
An even more significant factor in the diagnosis of myasthenia gravis is the conduct of a proserin test.
The essence of the method is a significant improvement in physical condition with a sharp weakening of the manifestations of the disease in half an hour-hour after subcutaneous administration of 1-2 ml of a 0.05% solution of Prozerin.
Differentiation from other deviations
To differentiate myasthenia from ophthalmoplegia of a different genotype, the impedance of the acoustic stapedal reflex is used, the data of which are also confirmed by the performance of pharmacological samples.
When differentiating the myasthenic crisis from cholinergic( caused by an overdose of AChEP) in favor of the second, the occurrence of diarrhea, vomiting, abdominal pain, general shivering in the whole body;during the proserin test, there is a decrease in the severity of symptoms of the myasthenic crisis, while in a cholinergic crisis this is not observed.
When emergency aid is needed
When developing a myasthenic crisis due to insufficient dose of AChEP( anticholinesterase drugs),
- requires immediate intravenous injection of Prozerin( in a dose of 0.5 to 1 ml of 0.05% solution) supplemented with intramuscular( 2-3 mlevery 2-3 hours);
- subcutaneous administration of a 5% solution of ephedrine;
- intravenous administration of potassium preparations;
- Oksazila in the form of candles, or Kalinin( Mestinon), or a combination of Mestinone and Oksazil.
Subcutaneous administration of 0.5-1 ml of Atropine solution allows to reduce hyper-salivation and hyperproduction of tracheobronchial secretions.
If necessary, use:
- of cardiac glycosides;
- of caffeine;
- in the occurrence of vascular collapse apply adrenomimetics( Adrenaline, Mesaton, Dopamine).
Myasthenic crisis is treated in the intensive care unit.
If, despite the use of large doses of Prozerin, airway patency does not return and the development of a life-threatening respiratory muscle weakness increases, resort to tracheostomy for vital signs, or, by making intubation, transfer the patient to ventilator breathing( ALV).
Balance of fluid and electrolytes is maintained, infusions of Polyglukin and Reopoliglyukin are carried out;with the aim of controlling metabolic acidosis, an intravenous drip injection of 1% soda solution( sodium bicarbonate) is performed.
The most effective method of emergency treatment is the purification of blood from pathological autoimmuno-complexes( hemosorption or plasmapheresis).
An exceptionally important point is the correct differentiation of the nature of the onset of a crisis, because a mixed-type crisis excludes the use of AChEP during the entire period of mechanical ventilation.
Overdose of AChEP can lead to a cholinergic crisis( reminiscent of a crisis of myasthenic) and require the withdrawal of anticholinergics and intravenous or subcutaneous injection of 0.5 ml of atropine solution( if necessary, repeated).
After the disappearance of the cholinergic crisis, the use of proserin test is possible not earlier than in a day.
Nasogastric tube is used to supply the patient.
Further therapy after arresting an attack
Treatment of myasthenia gravis has two main objectives:
- replenishment of arisen( relative) deficiency of acetylcholine for the restoration of neuromuscular transmission;
- suppression of an autoimmune response of the body.
The first line of therapy( in the treatment of the local ophthalmic and pharyngeal myasthenia gravis) suggests the use of anticholinesterase agents: Prozerin, Pyridostigmine bromide( Mestinone, Kalimin), Oksazil, where the choice of the optimal individual dose that allows compensation of the process depends not only on the severitysymptoms of this clinical type, but also from complicating the underlying disease pathology and individual reaction to the drug used. Supplement to the treatment used are potassium preparations( orotate or chloride), Ephedrine, Veroshpiron, in a more severe version - intramuscular injection of 1.5-2 ml of 0.05% solution of Prozerin 30 minutes before meals.
Generalized myasthenia gravis requires more serious means, including the use of immunosuppressive drugs and corticosteroids( primarily Methylprednisolone at a dose of 100 mg every other day).With the onset of a persistent improvement achieved by prolonged intake of the maximum dose of CS, it decreases to a supporting dose.
Serious success in the therapy of suffering allows us to achieve the use of enterosorbents( Polyphepan obtained by hydrolysis of lignin), immunomodulators, extracorporeal photochemotherapy( photopheresis).
The surgical method for the treatment of myasthenia gravis is suggested with:
- generalized moderate and severe disease;
- progression of generalized myasthenia gravis;
- occurrence of myasthenic crises( including a history of mention);
- signs of tumor degeneration of the thymus gland.
The standard method of surgical treatment of the thymus gland is thymectomy( with tumor degeneration being replaced by thymomectomy);Glomectomy with the removal of the carotid glomus is currently not used in treatment.
With incomplete or contraindicated( in elderly people who have a generalized form of myasthenia gravis), thymectomy shows x-ray therapy on the thymus gland region.
Forecast for life, or Hasta la vista, Myasthenia!
Despite the seriousness of the prognosis of a disease that has an autoimmune basis, the time when myasthenia gravis was the death sentence in the vast majority of cases has passed - now it is capable of "only" bringing the patient to a wheelchair.
Is there hope for complete healing now, and is self-healing possible?
The hope for spontaneous remission is a "thing" that is very unstable, and the improvement that comes with pregnancy will not necessarily be persistent.
Myasthenic crises with the possibility of a lethal outcome - this is the most probable future, waiting for the patient with persistent ignoring of the first anxiety symptoms.
Therefore, the issue of the future, which "threatens" the myasthenic - is largely a matter of timely treatment for specialized neurological care and early treatment.
And then years of ascetic, scrupulous fulfillment of medical appointments should come, because only an attentive attitude in the use of prescribed medicines will make it possible to achieve a noticeable improvement.
No less important is the care for the maximum possible in this situation to strengthen their health, because the presence in the musty atmosphere for years does not clean apartment health clearly does not add.
As well as the endless "mourning" of one's destiny, leading to depression and disease progression.