Myasthenia - what is it? Symptoms, causes and treatment of myasthenia
Myasthenia: what is it?
Myasthenia is a special disease, the main symptom of which is weakness in striated muscles. It increases very rapidly during physical exertion and decreases after rest. It is believed that this disease has an autoimmune nature, so it is perfectly treatable with hormonal drugs.
This pathology is diagnosed quite often, with 2/3 of the patients being the fair sex. The disease "debuts" in people aged 15-45 years, but the vast majority of cases are registered in 30-year-olds.
It is noteworthy that this pathology is detected not only in humans. Quite often myasthenia is found in dogs and cats.
Causes of the disease and the mechanism of its development
Unfortunately, experts can not say exactly why myasthenia gravis develops. It is assumed that a certain role belongs to heredity.When interviewing a patient at a consultation, it often turns out that such a pathology was diagnosed earlier in close relatives.
Sometimes the myasthenia does not occur independently, but in combination with a tumor of the thymus or against the background of diseases of the connective tissue, diseases of oncological nature (lung cancer, breast cancer), is associated with ALS. If the pathology develops in combination with the above described diseases, it is considered not as an independent ailment, but as a syndrome.
Under the influence of certain factors (stress, the influence of infectious agents), the body begins to continuously produce antibodies to its own tissues, and more specifically to the receptors of the postsynaptic membrane. The main function of the synapse is the transmission of nerve impulses from one neuron to another. Under the influence of antibodies, the membrane begins to consistently break down, the transfer of momentum loses its strength, and then finally stops.
In the study of the central nervous system changes characteristic of myasthenia, most often not observed. In 30% of cases, doctors diagnose an enlarged thymus gland in size or with the presence of a tumor neoplasm.As for muscle fibers, microscopic examination of them reveals signs of atrophy.
What clinical signs indicate a disease of myasthenia gravis? What is this pathology, we have already told, it's time to talk about its characteristic symptoms.
According to experts, this ailment is diagnosed most often already in a running form. For this reason, any fatigue, muscle lethargy and a change in voice should be regarded as a possible symptom of the disease until the doctor refutes this diagnosis.
The earliest signs of pathology include the following:
- Voice attenuation during conversation.
- Difficulty chewing on solid food.
- Problems with swallowing.
- Fast fatiguability.
- The omission of the eyelids.
Most often affects swallowing, chewing and facial muscles. The following tests reveal hidden current myasthenia:
- If a patient is asked to open and close his mouth quickly for 40 seconds, then a healthy person during this time will do at least 100 repetitions, and the person suffering from the disease will be much less.
- Take a supine position, slightly raise the head and hold it in that position for a full minute.
- Perform 20 squats.
- Quickly compress and alternately unclench brushes.In patients with myasthenia, this task often provokes involuntary drooping of the eyelids.
The local variant of the disease is characterized by the appearance of weakness in a certain group of muscles, and with a generalized variant, the muscles of the entire body are involved in the pathological process. All of the above symptoms, as a rule, indicate a disease of myasthenia.
Treatment of pathology should begin only after the doctor confirms the diagnosis and determines the specific version of the disease. This form of the disease affects only the oculomotor, circular and eyelid muscles. The main manifestations of the pathology are considered: squint, double vision, the inability to look at objects for a long time, difficulty in focusing the eye. In addition, almost all patients present a special symptom - the omission of the upper eyelid. The specificity of this clinical feature is that it appears only in the evening.
Pathological fatigue of the facial and chewing muscles leads to changes in the voice, problems when eating.A normal conversation can be so tiring that it will take the patient several hours to fully recover. Chewing solid foods for people with this diagnosis can be an overwhelming task. They try to distribute meals within 24 hours so that the antibiotics used during myasthenia at this time act as strongly as possible. Even in moments of insignificant improvement in well-being, patients with such a diagnosis prefer to eat in the first half of the day, since by evening the clinical picture usually increases.
Generalized myasthenia gravis
This is the most dangerous form of the disease, which develops gradually. Initially, the eye muscles are affected. Then the muscles of the neck and extremities are involved in this pathological process. It becomes difficult for the patient to hold his head, drooling appears. Every day it becomes more difficult to move. The generalized form is characterized by weakness of the respiratory muscles. The resulting respiratory distress often leads to hypoxia and even death.
Symptoms of myasthenia in children
The disease in young patients is divided into several forms:
- Congenital myasthenia. What it is? Fatigue of the muscular system can be determined even in utero during prophylactic ultrasound. In this disease, fetal movement is not distinguished by activity. Death occurs due to impaired breathing.
- Myasthenia neonatal. It is diagnosed in those children whose mothers also suffer from this pathology. The disease manifests itself from the first days of life and is distinguished by the following symptoms: shallow breathing, the child sucks the breast poorly or refuses it, difficulty in swallowing, the gaze is immobile.
- Early childhood myasthenia. It occurs at the age of two years, when an absolutely healthy child has vision problems. The eyelids fall down without cause, a squint arises. Sometimes the baby is difficult to move, he constantly asks for his hands to his parents.
- Youthful myasthenia. What is this pathology? Develops mainly in children from two to ten years old or in adolescence. Ailments are more susceptible to girls.Among the early signs, experts recommend paying attention to fatigue, blurred vision, difficulties in performing basic household work.
Myasthenic crisis is a sudden deterioration of the patient's condition, associated with acute muscle weakness. Breathing first frequent, then rare and rather intermittent. At the same time the person gets a crimson shade. Pulse increases, blood pressure rises to 200 mm Hg. st. After the final cessation of breathing, patients faint, and without the provision of competent medical care, the likelihood of clinical death increases.
There are several ways to confirm the diagnosis of "myasthenia". Neurology is actively studying this disease, offering different options for the study of patients with this kind of pathology.
- Clinical examination.
- Blood test for antibodies.
- Prozerin test.
- CT of the thymus gland.
The most reliable recognized prozerinovaya sample. Prozerin is administered subcutaneously to the patient, after which a short-term improvement of the condition occurs.
If found myasthenia
Patients with such a diagnosis should be registered with a neurologist, constantly take the prescribed drugs and strictly follow all the recommendations of the doctor. In this case, possible prolonged retention.
With myasthenia, sunbathing, serious physical exertion, taking certain groups of drugs (quinine derivatives, muscle relaxants, neuroleptics, etc.) are contraindicated. All patients must be given a list of such medicines, which must always be with you.
Patients are shown sanatorium treatment in institutions with a medical profile.
It is not possible to recover from a myasthenia permanently, but it is in the strength of each patient to achieve stable remission. Drug therapy includes the following drugs:
- Potassium preparations. Promote impulse conduction from neurons and improve muscle contraction.
- Anticholinesterase drugs. Assign all patients with a diagnosis of "myasthenia" (eye form). Treatment is aimed at inhibiting the work of the enzyme that destroys acetylcholine.
- Cytostatics ("Azathioprine", "Cyclosporine").Preparations inhibit the autoimmune process, while reducing the number of antibodies and immune cells.
- Human immunoglobulin. Represents a mixture of protective antibodies taken from completely healthy people. The drug has a positive effect on the immune system.
- Antioxidants. Aimed at improving metabolic processes in the body.
- Hormonal agents. Most often, prednisolone is prescribed for myasthenia gravis. This tool has virtually no side effects and at the same time reduces the symptoms characteristic of this disease.
Sometimes plasmapheresis is recommended for patients. This method is based on the purification of blood from antibodies. During the procedure, a person makes a collection of several portions of blood, from which plasma is separated by means of a centrifuge, and the cells themselves are preserved.
Recently, gamma irradiation of the thymus has been actively used. This procedure contributes to the suppression of the autoimmune process due to the action of radial energy.
Surgical intervention is prescribed only for rapidly progressing disease of myasthenia gravis. The operation is also indicated in the presence of oncologic neoplasm, involvement of the pharyngeal muscles in the pathological process.
Drug treatment is long, patients are forced to take medication for life. The doctor prescribes a specific drug and its dosage. During the course of therapy, it is very important to monitor the patient's condition (monitor blood sugar levels, check blood pressure daily, prevent osteoporosis). In the period of exacerbation, when drug treatment is ineffective, plasmapheresis is recommended 5-7 times a day.
Above, we have listed the main contraindications for myasthenia gravis. It is not possible to carry out prevention of the disease due to the lack of sufficient information about the reasons for the formation of the autoimmune process against its own acetylcholine receptors.
Myasthenia usually develops in young people. The average age of patients barely exceeds the mark of 20 years. In the absence of timely diagnosis and treatment, the disease progresses rather quickly. Mortality from it is quite low, but the quality of life with this diagnosis is significantly deteriorating.
Neuromuscular diseases (myasthenia gravis, myopathy and others) are currently attracting the attention of specialists from all over the world.Pathologies of this kind require an exceptionally qualified approach to treatment. Scientists continue to actively explore these diseases, to look for effective drugs.