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Myasthenia gravis or Erb-Goldflam disease is a chronic autoimmune disease that is characterized by increased fatigue of all muscles of the human body. This pathology usually develops between 20 and 40 years of age. With timely treatment, remission can be achieved in 80% of cases. Treatment in Germany for myasthenia gravis involves the use of the latest proven techniques, which increases the percentage of remissions without neurological deficit.
Etiology and symptoms of myasthenia gravis
There are two types of myasthenia gravis worldwide:
- congenital
- acquired
In the acquired version of myasthenia, the cause of development is a genetic mutation. Usually other family members also have a history of myasthenia gravis. Various types of proteins, which are structural elements of synapses, are involved in muscle function. Acetylcholinesterase is present at neuromuscular synapses, which is responsible for transmitting impulses, without which muscle fibers do not contract. With a mutation in the COLQ gene, which is responsible for the linking protein between acetylcholinesterase and collagen-like protein, myasthenia gravis develops. In addition, the mutation may be a structural component of the synapse itself. In the acquired version of myasthenia gravis, there are some catalysts that trigger the disease. These include:
- thymus tumors (which is most common) and other cancers
scleroderma
dermatomyositis
The last two processes also apply to diseases of an autoimmune nature.
Myasthenia gravis usually affects quite young people. Most cases of onset of Erb-Goldflam disease are described in 20-30 year old women. In children, most often hereditary forms of the disease. Since myasthenia gravis is a lesion of all striated muscle fibers that are involved in movement, the disease can begin with damage to any muscle.
Most often, the eye muscles are affected first. Patients note double vision, drooping eyelids — ptosis. However, in the morning the symptoms are less pronounced. After damage to the eye muscles, the disease spreads to the muscles of the upper and lower extremities. In such cases, it becomes difficult for a person to climb the stairs or raise his arms up. To check symptoms, the patient is asked to sit down several times. After physical activity, a person experiences increased weakness in the limbs and may even develop ptosis.
Later, damage to the muscles responsible for swallowing occurs. This is accompanied by bulbar disorders. These include:
- nasal tone of voice
- it’s hard to pronounce the letters r, sh, s
- difficulty swallowing, food getting into the nose.
With further progression of myasthenia gravis without appropriate treatment, the process spreads to the respiratory muscles. It becomes difficult for the patient to breathe, in some cases requiring connection to a ventilator.
Diagnostics
German specialists approach the patient in a personalized manner, diagnosing and treating the disease collectively, with the involvement of world experts. At the first stage, the doctor will find out in detail the circumstances of the disease, genetic predisposition, symptoms that have appeared and the treatment carried out before the examination.
Next, the specialist will examine the patient to determine both the symptoms of the disease and possible concomitant diseases. If suspected and to confirm the diagnosis, a functional test is performed.
This test is called a decrement test. The test methodology is described in sufficient detail. This electromyography test is performed using an electromyograph. Electrodes are installed on the muscles being studied, an impulse is sent through the stimulating electrode and then a response is expected — the M-response of the muscles. With the help of a decrement test, a specialist can determine the type of disorders, their severity, and, very importantly, the reversibility of the pathology. The test is also performed after the administration of drugs to determine the reversibility of changes during treatment.
In addition, a blood test is performed to detect the presence of antibodies to acetylcholine receptors, and, if necessary, to detect antibodies to titin. A CT scan is performed to identify the cause. In this case, the main area of interest is the thymus gland; a tumor is often found in such patients.
Therapy
At the initial stages of the disease, drug treatment is carried out. The drugs kalimine (an anticholinesterase agent) and potassium are prescribed. For more pronounced changes, glucocorticoids (prednisolone) are added to therapy. If treatment does not help, surgical tactics are used in the form of removal of the thymus gland. Typically, the effect of therapy is assessed during the first year. If there are contraindications to hormonal drugs, as well as surgical treatment, cytostatic therapy is prescribed. Clinical trials of new immunosuppressive drugs are constantly being conducted in German clinics.
In case of severe exacerbations of the disease, plasmapheresis and immunoglobulin administration are performed. Plasmapheresis is carried out both in day and round-the-clock hospital settings. There are clear indications for plasmapheresis. With progressive deterioration of the condition, with removal of the thymus gland (before and after removal), with drug resistance and myasthenic crisis.
It is expected after the procedure:
- decrease in antibody titer
- increase in physical activity
- remission from 3 months
- increased sensitivity to drugs
There are two options for plasmapheresis:
- discrete. In this procedure, blood is taken in portions, after which it is separated into blood cells and plasma. The blood cells come back. Plasma is replaced with donor plasma.
- hardware option. The extraction and return of blood occurs simultaneously and continuously.
Both options have been used for several decades. Typically, 1.5-2 liters of plasma are replaced during one session, and 4.5-6 liters over the entire course of treatment.
The administration of immunoglobulin is also indicated for severe Erb-Goldflam pathology and exacerbation. It is administered intravenously, also to prepare for surgery. No preparation is required for the administration of immunoglobulin, and, unlike plasmapheresis, the installation of a large-volume intravenous catheter is not required. Usually the course is carried out over 5 days. After another 5 days, improvement occurs, lasting from several weeks to several months.
Cost of treatment and choice of clinic
The price of therapy depends on the severity of the patient, the type of pathology, the volume of affected muscles, and the required method of therapy and diagnosis.
The minimum cost for diagnosis and treatment of the disease is from 5600 Euro.
Myasthenia gravis is a rather specific pathology; a small number of clinics treat it. However, to obtain high-quality information about the clinic and its specialists, it is necessary to work through a large amount of information. A team of experts led by Maxim Rykov has been working on this issue for more than 10 years. He and his team provide consultations on choosing a clinic and specialist.
Previously, myasthenia gravis was accompanied by a high mortality rate of up to 40%. Today, due to new methods of therapy and diagnostics, the mortality rate is less than 1%. In most cases, complete remission is achieved.
Myasthenia gravis requires high-quality medical care, since late onset and poor-quality therapy can lead to serious complications.