Myasthenia gravis

Myasthenia gravis

Description myasthenia gravis

Myasthenia gravis is a neuromuscular autoimmune disease. Autoimmune means that the human body produces antibodies against its own structures which are harmful. In myasthenia gravis regard specifically to antibodies against receptors for acetylcholine. Acetylcholine is a neurotransmitter, a substance mediating neuromuscular transmission signal. Receptor on a cell surface structure, which binds to a specific substance (here acetylcholine) and trigger certain reactions in cells. This reaction is muscle contraction. Because there is a shortage of available receptors (autoantibodies are occupied) and on these receptors is progressively less and less, has acetylcholine where established, there is no transmission signal to be pulled muscle and leads to muscle fatigue. It mainly affects the muscles of facial expression, eye (extraocular muscles), muscles of the soft palate, tongue and neck (bulbar muscles) and upper limb girdle.

The first description of the disease gave Willis the year 1692, although references are from the early 17th century, when the nature of the disease mystanie gravis has been described in the Native American chief Openchankanougha. In the late 19th century brought a true description of the disease Erb, Goldflam and Jolly, while Jolly has used the term myasthenia gravis pseudoparalytica (pseudoobrna – on the grounds that with myasthenia gravis is not about damage to the nervous system itself). 1960 was recognized by the autoimmune nature of the disease, Mr. Simpson.Myasthenia gravis was the first evidence of an autoimmune disease.

Myasthenia gravis disease is not common, however, is not rare. It occurs as often as multiple sclerosis (multiple sclerosis), especially in temperate climates. Mainly affects women aged between 20-40 years, then later a man between 60-80 years. In recent years there has been an increase rather diseases in older age groups, which may be due to the ever increasing use of medication (for some drugs is suspected ability to cause disease). Increased risk of the patients suffering and other autoimmune diseases (autoimmune thyroid disease, pemphigus, lupus erythematosus, pernicious anemia …).

In 65% of patients had shown affection sweetbreads (thymus) hyperplasia (hypertrophy), it is often described as benign (noncancerous) tumor – thymoma. Thymus (thymus) is the body normally used for the creation and training of the active immune white blood cells are more active in young people. If it does not properly formed white blood cells that produce antibodies against erroneous own structures – are against receptors for acetylcholine, and myasthenia gravis occurs. For patients with established thymoma is then indicated by surgical removal of the entire thymus. However, not only in these patients, but also in patients with a severe form of the disease in which thymoma was not established and the treatment does not lead to management of the disease or symptoms of the disease are very hard, then the operation still results in a large majority to improve symptoms.

Risk factors for myasthenia gravis

Among the risk factors we include age between 20-40 and 60-80 years, inhabiting the temperate
podnebního belt, occurrence of myasthenia gravis in a family, the occurrence of otherautoimmune diseases (including in the family) and medications suspected of possibilities invocation myasthenia gravis, although it is believed that these drugs only reveal hidden assumption to the disease and not cause disease itself . This fact is not disputed. Among the suspected drugs include. Penicillamine. Another risk factor is the above-mentioned thymoma.

Prevention myasthenia gravis

Prevention of the disease is not known. In case of other autoimmune diseases in individuals should consider giving drugs suspected the possible association with induction of myasthenia gravis.

The symptoms of myasthenia gravis

Myasthenia gravis manifests easy muscle fatigability. Patients often complain of falling eyelids during the day (ptosis), can not keep my eyes open, seeing double (diplopia), which is caused by fatigue eye extraocular muscles, some bad talk and awkwardly pronounce words (dysarthria) may be disrupted and the receipt and processing food in the mouth and swallowing (dysphagia), hold upright head, unable to perform the work in vzpažení (clothesline), and in some cases are affected and leg muscles (pelvic girdle), so that they can suddenly fall, some are unable to climb the stairs like. difficulties heighten during the day and increasing physical exertion, so the mornings are usually ill for some time without any problems and evening symptoms worsen. Difficulties can gradually occur during breathing. About 10% of patients may experience a myasthenic crisis, when the patient is no longer able to breathe on his own and needed mechanical ventilation by a fan (see complications).

Treatment of myasthenia gravis

Myasthenia gravis is currently very well treatable disease. You can achieve remission (relief of symptoms) for more than 80% of patients. The basic step in a large proportion of patients (particularly up to 60 years of age) is a delete operation thymus (thymektomie), which leads to permanent remission in approximately 25% of patients, a significant improvement, then 80% of patients. Among the medicines taken as acetylcholinesterase inhibitors (e.g. neostygmin – block decomposition of the neurotransmitter acetylcholine in the transmission signal from the nerve to the muscle), and further drugs dampening the immune system – immunosuppressive therapy.

In myasthenic crisis or with significant disease progression is used a so-called. Plasmapheresis (i.e. adjustment of plasma autoantibodies) or are administered high doses of immunoglobulins (antibodies). If the patient is unable to breathe adequately alone, it is necessary to start ventilation.

How can I help myself

Sami can not help – it is advisable to seek expert (neurologist), which will help you manage your symptoms, your treatment will now already very effective drugs and provide you with valuable advice on how to deal with the disease in daily life. Without treatment expert prognosis is uncertain.

Complications myasthenia gravis

The most serious complication of the disease is called. Myasthenic crisis. When mystenické crisis affected the respiratory muscles are no longer able to fully perform its function and the patient gradually he is unable to breathe on their own. Dyspnea state begins and progresses to respiratory arrest on the basis of respiratory muscle dysfunction. Such a situation should be recognized and treated in time, otherwise it may result in death.

Other names: Myasthenia gravis

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