Treatment of myasthenia abroad
Myasthenia is a pathological illness relating to the increased tiredness of skeletal muscles. It mostly affects children from 6-7 years onwards. Myasthenia can affect muscles that are responsible for the respiratory function.
Hence a patient can die because of acute respiratory failure without medical intervention as a consequence of the myasthenic crisis.
Causes of the myasthenia
This illness develops mostly as a result of malfunctions with autoimmune system . A person’s immune system destroys the acetylcholine receptors located on the surface of striated muscles. Striated muscles are muscles that can be contracted and relaxed intentionally.
Some of the other causes are:
- Tumour of the thymus gland (thymoma)
- Congenital defect of nicotinic acetylcholine receptors
The following factors can trigger the autoimmune form of the disease:
- Viral infection
- Severe stress
- Puberty period
- Accompanying autoimmune pathologies
Symptoms of myasthenia
The clinical signs of myasthenia can vary. They mostly depend on the variety of the disease.
Myasthenia with an early onset is peculiar with the weakness of body and muscle extremities. Muscles are then involved into the pathological process:
- Extraocular muscles (80% of patients) – patients have eye symptoms related to movement due to the weak muscles around the eye
- Bulbar (65%) - patients experience speech and swallowing disorders as a result of damage to the cranial nerves: sublingual, glossopharyngeal and vagal
- Mimic muscles (92%)
Muscle function is seldom affected:
- Chewing (40%)
- Respiratory (20%)
- Neck muscles (30%)
Myasthenia in combination with thymoma (tumour of the thymus gland) and myasthenia with a late onset. Significantly more often in the debut of the disease are affected:
- Bulbar muscles (80%)
- Chewing muscles (80%)
- Neck muscles (65%)
- Deltoid muscle (75%)
Seronegative myasthenia is characterised by the weakened respiratory muscles (65%) that leads to the risk of the respiratory failure. The majority of patients experience bulbar muscle also have speech and swallowing disabilities. Oculomotor disorders (double vision, drop of the eyelid) are extremely rare (10% of patients). Damage of the muscles in the torso is also uncommon (35%).
Sometimes the disease worsens and develops into a myasthenic crisis. In severe cases, this condition manifests itself with:
- Depression of consciousness
- Severe weakness of the affected muscles
- Respiratory failure
- Redness, and then cyanosis( going blue in the face)
- Psychomotor agitation
- Increase in pulse and pressure
- Increased salivation and sweating
Certain patients may experience involuntary defecation or urination. Hypoxic encephalopathy (disordered brain function due to oxygen starvation) can follow.
Diagnostics of myasthenia
Diagnostics is based on the clinical examination of the patient. Stage of the movement malfunctions in the limbs muscles is rated from zero to five:
- 5 – movements are fully preserved in the limbs;
- 4 – muscle weakness is observed;
- 3 – sharp decrease of the muscle strength;
- 2 – it is hard for a patient to hold the weight of the limb;
- 1 – a patient is unable to hold the weight of the limb;
- 0 – movements in the extremity are absent.
Other muscle group’s condition are also evaluated depending on the clinical stage of myasthenia. Various tests are undergone to evaluate the strength of the neck muscles for instance, ptosis, speech, breathing, swallowing disorders are also determined. All malfunctions are counted in points and the severity of myasthenia gravis is evaluated.
Laboratory diagnostics. One of the main criteria is the increased level of autoantibodies in the blood serum to the acetylcholine muscle receptor. This indicator is important not only to establish the diagnosis but also to assess the effectiveness of the therapeutic procedures used.
Other laboratory methods of diagnostics are:
- Patients with thymoma or late onset of myasthenia without thymoma are tested for antibodies to titanium protein.
- The level of antibodies to ryanodine receptors of sarcoplasmic reticulum may increase in the blood. This is an unfavourable prognostic sign observed in 35% of patients when myasthenia is combined with thymoma.
- Patients with seronegative myasthenia, antibodies to muscle-specific tyrosine kinase occur in about half the cases.
Instrumental diagnostics. Electromyography is one of the basic methods that help to detect a violation of neuromuscular transmission. With the help of EMG, one can compare the degree of involvement of different muscles in the pathological process: masticatory, respiratory, facial, musculature of the extremities.
CT and MRI are visualization methods thatcan be used to identify thymoma in the mediastinum. However, the tumour of the thymus gland does not clearly indicate the presence of myasthenia, and therefore cannot be considered a diagnostic criterion.
Proserin test. The patient is injected with anticholinesterase drugs. They temporarily improve the neuromuscular transmission, increasing the strength of the muscles. The sensitivity of the sample is about 90%. The increase in the strength of damaged muscles after the introduction of proserin confirms the diagnosis.
Treatment of myasthenia abroad
Conservative treatment includes the use of anticholinesterase drugs, potassium and potassium-sparing diuretics. To enhance the effectiveness of therapy glucocorticoid hormones are used to suppress autoimmune processes. They bring results among 80% of patients. If the results are unsatisfactory then cytotoxic agents are prescribed.
Different forms and stages of myasthenia (acute, chronic, crisis) demand separate treatment approaches. Hence choose an experienced doctor for the best prescribed therapy scheme. It helps to minimize the risk of the side effects, including the risk of cholinergic crisis, which is a consequence of drug therapy used for myasthenia. In German hospitals specialists choose the best treatment tactics for their patients with the use of the latest medicines.
Plasmapheresis or hemadsorption is and extracorporeal (outside the body) blood purification methods that can remove autoantibodies that destroy cholinesterase receptors in the muscles. This allows for a temporary clinical improvement.
Treatment of the myasthenic crisis includes:
- Intubation of the trachea and performing artificial lungs ventilation if there is a sharp weakness of the respiratory muscles
- Control of blood gas composition
- The introduction of immunoglobulins
Surgery is performed in a number of cases. It is based on the thymus function. Indications for the operation are:
- Presence of thymus tumour
- Progression of myasthenia
- Bulbar form of the disease (accompanied by a violation of swallowing and speech)
- Generalised form of myasthenia among children
Average effectiveness of thymus removal is about 70%. A part of these patients are completely healed from myasthenia, some other ones experience significant clinical improvement which means that the doses of the medicines is reduced.
Myasthenia - Prognosis
Natural course (without diagnosis and treatment) of myasthenia is dangerous with a lethality of 30-40% of patients. Though, the majority of patients seek medical help and receives at least conservative treatment. Therefore, general mortality rate of patients suffering from myasthenia doesn’t go over 1%. Possible death cause is the respiratory failure on the basis of a myasthenic, cholinergic or mixed crisis.
Modern diagnostics and treatment ways allow a complete compensation of the movement disorders among 80% of patients having myasthenia with the help of surgical and conservative methods. Thus, life and work capacity prognosis is favourable for the majority of patients.
Illness can be treated without any further consequences based on the condition that one receives adequate treatment. It’s better to receive medical care in a trusted European or German clinic. High-quality healthcare service will help to quickly restore the muscle function and to further monitor and and keep the disease at bay.