As a result, developing abnormal fatigue and weakness of the skeletal muscles, mutations in proteins of neuromuscular junctions may lead to the development of congenital myasthenic syndromes.
What it is?
Myasthenia gravis - a rather rare disease of autoimmune type, characterized by weakness and lethargy muscle. Myasthenia communication failure occurs between nerve and muscle tissues.
Official scientific name of the disease sounds - myasthenia gravis pseudoparalitica, which translates as myasthenia gravis in the Russian language. commonly use the term "myasthenia gravis" in Russian medical terminology.
Causes of myasthenia gravis
To date, experts do not have clear information about what it provokes in human myasthenia gravis symptoms. Myasthenia gravis is an autoimmune disease, because in the serum of patients with multiple autoantibodies are detected. The doctors fixed a certain number of familial cases of myasthenia gravis, but evidence of the influence of genetic factors on the manifestation of the disease is not present.
Quite often, myasthenia gravis manifested in parallel with hyperplasia or a tumor of the thymus gland. Also myasthenic syndrome may occur in patients who complain of organic disease of the nervous system, polidermatomiozit, cancers.
Most often suffer from myasthenia female. As a rule, the disease manifests itself in people aged 20 to 30 years. In general, the disease is diagnosed in patients between the ages of 3 and 80 years. In recent years, experts are showing significant interest in this disease due to the high frequency of symptoms of myasthenia gravis in children and young people, which leads to subsequent disability. For the first time this disease was described more than a century ago.
The mechanism of development of myasthenia play the role of autoimmunity were detected antibodies in muscle tissue, and thymus. Commonly affected muscles age, there is ptosis, which varies in severity throughout the day; chewing muscles are affected, disturbed ingestion, gait changes. Patients harmful nervous, because it causes chest pain and shortness of breath.
Precipitating factor can be stress ported SARS, impaired immune function of the organism leads to the formation antibodies against the body's own cells - against acetylcholine receptors of the postsynaptic membrane neuromuscular junctions (Synapses). Inherited autoimmune myasthenia gravis is not passed.
Most often, the disease manifests itself during puberty in girls (11-13 years old), it is less common in boys at the same age. Increasingly, the disease is detected in children of preschool age (5-7 years).
This disease develops at all differently. myasthenia begins most often with weak eye and facial muscles, then this is a violation of passes on the muscles of the neck and trunk. But for some people there are only a few signs of the disease. Accordingly, there are several types of myasthenia gravis.
- Eye shape is characterized by lesions of the cranial nerves. The first sign of this is the drooping of the upper eyelid, often first on one side. The patient complains of double vision, difficulty in movement of the eyeballs.
- Myasthenia bulbar form - the defeat of chewing and swallowing muscles. In addition to violations of these functions, the patient changes it, the voice becomes low, snuffling, there are difficulties in the pronunciation of certain sounds, such as "p" or "b".
- But the most common generalized form of the disease, in which first affects the muscles of the eye, then the process extends to the neck, upper and lower limbs. Very often affects the hip and arm muscles, the patient is difficult to climb the stairs, hold the items. The danger of this form of the disease is that the weakness spreads to the respiratory muscles.
Unfortunately, most of myasthenia gravis are diagnosed as early as when the disease occurs several years in a row and goes into running shape. For this reason, any unexplained fatigue, weakness of muscles, increases sharply when repetitive movements weakness It must be considered as a possible symptom of myasthenia as long as the diagnosis is completely refuted.
Early symptoms include:
- swallowing disorder,
- difficulty chewing solid foods until the refusal of food,
- Conversation - "attenuation votes"
- fatigue when combing, climbing stairs, normal walking,
- the emergence of a shuffling gait,
- drooping eyelids.
The most commonly affected eye movement, facial expression, chewing muscles and the muscles of the larynx and pharynx. The following tests help to identify latent myasthenia gravis:
- If a patient is asked to quickly open and close your mouth for 30 seconds, then a healthy person will make about 100 movements, while suffering from myasthenia gravis - less.
- Lie on your back, lift your head and hold it in this position for 1 minute while looking at her belly.
- Pull your hands and stand as 3 minutes.
- 15-20 do deep knee bends.
- Quickly open and close the hand - in a patient with myasthenia this often causes drooping eyelids.
Local shape myasthenia characterized manifestations of muscle weakness certain muscle groups, and in generalized form in the process involved muscles of the trunk or limbs.
As shown by clinical practice, myasthenia gravis is a progressive disease, which means under the influence of certain faktorparalichov (or the external environment causes endogenous character) and degree of severity of symptomatic manifestations of the disease may increase. And even in patients with a mild form of myasthenia gravis may occur myasthenic crisis.
The reason for this condition may include:
- stress conditions:
- Any acute infection;
- receiving medical preparations with neuroleptic or tranquilizing effect;
- surgical intervention into the body, and so on.
Symptomatology manifested by the fact that first appears diplopia. Then, the patient feels a sudden attack increasing muscle weakness, motor activity laryngeal muscles is reduced, which leads to disruption processes:
- breathing and swallowing;
- enhanced salivation and quickens the pulse;
- may be dilated pupils, tachycardia occur and come complete paralysis without loss of sensitivity.
The development of acute deficiency of oxygen in the brain can lead to a direct threat to life, so urgently necessary to carry out resuscitation.
In order to make the correct diagnosis of the patient prescribe a comprehensive study, as the clinical picture of myasthenia gravis may be similar to other diseases. The main diagnostic methods are:
- Deployed biochemical blood tests for antibody detection;
- Electromyography - Research, during which you can evaluate the potential of muscle fibers when they are excited;
- Genetic screening, which is performed for detection of congenital myasthenia form;
- Electroneurogram - research, allowing to evaluate the transmission speed of nerve impulses to the muscle fibers;
- MRI - with the help of this study can be seen even the smallest signs of hyperplasia of the thymus;
- Muscle fatigue test - the patient is asked to quickly open and close your mouth several times, waving his arms and legs, open and close the hand, crouch. The main myasthenia syndrome is the appearance of increasing muscle weakness by repeating these movements.
- Prozerinovaya sample - Neostigmine administered to the patient subcutaneously, followed by waiting until a half hour, and then the result is evaluated. Myasthenia patient feels significant improvement in his condition, and after a few hours of clinical symptoms resumed unabated.
How to treat myasthenia gravis?
In severe myasthenia shows removal of the thymus gland during surgery. The most effective drugs successfully employed for removal of disease symptoms include neostigmine methylsulfate and Kalinin. Along with them using drugs that enhance the immune system, and a number of other drugs that improve patient health. It is important to remember that the sooner treatment is started, the more effective it will be.
In the first stage of the disease as symptomatic therapy is used anticholinesterase drugs, cytostatic agents, glucocorticoids and immunoglobulins. If the cause of myasthenia gravis is a tumor, the surgery is performed at its disposal. In cases where myasthenia symptoms quickly progress is shown extracorporeal hemocorrection allowing to clean the blood of antibodies. Already after the first treatment the patient noted improvement in the state for more stable effect treatment is carried out for several days.
To new, effective methods of treatment refers krioforez - blood purification from harmful substances under the influence of low temperatures. The procedure is done the course (5-7 days). Benefits krioforeza before plasmapheresis are obvious: in the plasma, which is returned to the patient after treatment, retains all the nutrients intact, which helps to avoid allergic reactions and viral infection.
Also hemocorrection to new methods used in the treatment of myasthenia gravis, refers plasma cascade filtration at which purified blood passing through nanofilters is returned to the patient. Already after the first minutes of the procedure, the patient noted improvement of health, the full course of treatment of myasthenia requires five to seven days.
By modern methods of treatment of myasthenia gravis also applies extracorporeal immunopharmacotherapy. As part of the procedure, the patient is isolated from blood lymphocytes, which are treated with medicines and sent back to the patient's blood stream. This procedure in the treatment of myasthenia gravis is considered to be as efficient as possible. It allows you to reduce the activity of immune system, reducing the production of lymphocytes and antibodies. This technique gives stable remission for a year.
Prevention of myasthenia gravis and its complications
can not prevent the disease, but we can do everything possible to make this diagnosis and to live fully.
- First, the control of the doctor. Such patients are neurologists. In addition to prescribed treatment regimen and systematically visiting neurologist is necessary to control the total state (blood sugar, blood pressure and so on. p.), to prevent the development of other diseases during treatment myasthenia.
- Secondly, you should avoid excessive stress - physical and emotional. Stress, heavy physical work, too active sports worsen the condition of patients. Moderate fizzaryadka walking even useful.
- Thirdly, it is necessary to eliminate exposure to the sun.
- Fourthly, it is necessary to know the contraindications for patients with myasthenia and strictly comply with them.
- Fifth, adhere strictly to prescription regimen, not to miss taking medication and not taking the medicine more than the designated physician.
The doctor is obliged to give a list of medications are contraindicated such a patient. It includes magnesium preparations, muscle relaxants, tranquilizers, some antibiotics, diuretics except veroshpirona which, conversely, is shown.
Do not get involved immunomodulatory drugs and any soothing, even those that appear to be safe (eg, valerian tincture or peony).
Earlier, myasthenia gravis has severe disease with a high mortality rate - 30-40%. However, with modern methods of diagnosis and treatment mortality rate was the lowest - less than 1%, about 80% on the correct treatment achieved a complete recovery or remission. The disease is chronic, but requires careful observation and treatment.