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December 14, 2011
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The myasthenia is the illness affecting muscles and nerves. The main characteristic of this state is muscular weakness. The myasthenia is called still asthenic bulbar paralysis. First of all at this disease are surprised face muscles, eyes, lips, language, a neck, a throat. However process of defeat can overtake any muscle of a body.
This illness because of difficulty of transfer from nerves of a signal which has to set muscles in motion develops. There is pathology owing to disturbance in acetylcholine exchange organism. If to consider a human body generally, then it represents rather difficult electrochemical mechanism which moves by means of a muscle work. So, functioning of extremities of the person is ensured by skeletal muscles, activity of internals and vessels — smooth muscles. For muscles of skeletal muscles signals are transmitted through motor nerves. The electric impulse in the place where the nerve is attached to a muscle, is transmitted through acetylcholine – special chemical. Acetylcholine gets from a nerve to a crack between a nerve and a muscle owing to what there is an electric discharge which provides reduction of a muscle. In case of manifestation of a myasthenia in an organism there are changes in system of synthesis and emission of this chemical. Thereof the following impulse every time approaches more difficult a muscle therefore it becomes more difficult for person to make the movements. After the movement of a muscle stop completely. It is accepted to call a similar state a syndrome of pathological muscular fatigue.
Today specialists have no accurate data on what provokes emergence at the person of symptoms of a myasthenia. The myasthenia is an autoimmune illness, in serum of patients multiple autoantibodies are found. Doctors fix a certain quantity of family cases of a myasthenia, however there are no proofs of influence of a hereditary factor on display of an illness. Rather often the myasthenia is shown in parallel with a hyperplasia or a tumor of a thymus. Also myasthenic syndrome can arise at patients who complain of organic diseases of a nervous system, a polydermatomyositis, oncological diseases. More often female representatives are ill a myasthenia. As a rule, the illness at aged people of 20 - 30 years is shown. In general the disease is diagnosed for patients aged from 3 till 80 flyings. In recent years specialists show essential interest in this disease in view of the high frequency of manifestation of a myasthenia at children and young people that leads to the subsequent invalidism. For the first time this illness was described more than a century ago.
In most cases the disease begins to be shown with gradual lowering a century. In the late afternoon at the person begins to appear doubled in eyes, he changes a voice. Also the voice can change after rather strong speech loading. Very often the myasthenia is shown by the increased fatigue: so, patients often notice that they after rise from a bed feel rather well, and here after performance of elementary actions of a morning toilet they feel fatigue, as after a hard work. In view of weakness of eye muscles at the person the diplopia and squint is shown over time, and, symptoms of the given state to the termination of day are most expressed. Rather often at a myasthenia the patient has a weakness of muscles — front and chewing. At the same time the person also feels low language muscles. In the course of progressing of a myasthenia also cross-striped muscles of extremities, and also necks sometimes are surprised. Owing to such changes generalized weakness is observed.
For a myasthenia characteristics is dynamism, lability of symptoms. They amplify in the course of reading, attempts to fix a look on a certain subject, and also at a strong exercise stress. It is accepted to distinguish a generalized and local myasthenia. At a generalized form of a disease respiratory frustration can be shown.
The person, a sick myasthenia, feels in a cold season better, and in heat its state becomes worse. After the certain time allowed for rest, recovery of forces happens also quickly, as well as their loss. Very often the myasthenia proceeds for several years, at the same time the person does not suspect about the state. But nevertheless in view of progressing of a myasthenia the illness will declare itself sooner or later.
At patients with a myasthenia at children's age complaints to the high level of fatigue are observed. Children can cover unusually often eyelids, fall in the course of too fast walking or at run, drop a portfolio because of impossibility to hold it in a hand. Muscular weakness at children quickly progresses.
In the course of the course of a disease myasthenic episodes – frustration which take place during a short interval of time, and the subsequent spontaneous remissions can be shown. Also myasthenic states at which symptoms of an illness are shown steadily during long term are characteristic. The condition of patients can sharply worsen: sometimes there is crisis to generalized muscular weakness. At the same time also oculomotor and bulbar symptoms, trouble breathing, psychomotor excitement which replaces a condition of slackness, vegetative frustration are shown. This state threatens with the subsequent development of an acute hypoxia of a brain and frustration of consciousness.
Complications of a myasthenia
By means of acetylcholine as it is described above, also respiratory muscles of the person function. Therefore owing to problems with work of these muscles there can be a sharp disturbance of breath, up to its stop. Thereof there can come the lethal outcome. Adults have to show special attention always to complaints of the child. At development of a children's myasthenia often the child complains that he cannot twist bicycle pedals, rise by an eminence, etc. It is important to understand the reasons of a similar state, at a myasthenia at children respiratory insufficiency develops suddenly.
Diagnosis of a myasthenia
As a rule, process of diagnosis of this illness begins with complaints of the patient to the high level of fatigue, progressing of symptoms by the end of day and in the course of active exercise stresses. For installation of the exact diagnosis in similar cases the specialist carries out prozerinovy test. To the patient 1–2 ml of 0,05% of solution of a prozerin then in case of a myasthenia in 30–60 minutes symptoms sharply decrease are entered subcutaneously. For a myasthenia the condition of change of electroexcitability of muscles is also typical. For definition of such state the irritation of muscles by means of current is carried out, at the same time fast exhaustion of reduction of muscles is observed during repeated irritations. After the certain time allowed for rest, excitability of muscles is recovered again. Also specialists consider as one of the most informative methods of research elektromiografichesky inspection.
In the course of installation of the diagnosis it is important to carry out the differential diagnosis with a brainstem tumor, trunk encephalitis, basal meningitis, a myopathy, disturbance of blood circulation in a brain, a polymiositis.
Treatment of a myasthenia
First of all all therapeutic measures go for correction of deficit of acetylcholine, and also for suspension of autoimmune process. To compensate frustration of a neuromuscular transmission antikholinesterazny medicines are applied. The specialist studies disease individually and appoints the optimum compensating drug dose, being guided by weight of symptoms, a clinical form of an illness, reaction to a certain drug.
If for the patient it is diagnosed pharyngeal and front or eye forms of a myasthenia, then drugs prozerin, pyridostigmine bromide, an ambenonium chloride will be effective remedies of treatment. Are also in certain cases appointed veroshpiron, chloride or orotat potassium, ephedrine. The doctor selects doses of reception of the specified drugs individually. It is important to consider that high doses of antikholinesterazny drugs can provoke cholinergic crisis. In case of developing of myasthenic crisis to the patient it is urgently entered intravenously prozerin.
Except traditional drug treatment methods of a thymectomy, a roentgenotherapy and hormonal therapy are appointed sick with a myasthenia. The thymectomy is the surgical method of treatment applied to therapy to patients who were not 60 years old yet. The main condition for use of a similar method is the satisfactory condition of the person. In the presence of a thymus tumor this method has absolute indications. After carrying out an incomplete thymectomy appoint a roentgenotherapy to area of a thymus. Also this method is applied the patient with an eye form of a disease, and that who has available contraindications to operation (generally patients of advanced age). At a heavy current of a generalized myasthenia therapy with use of immunosuppressive drugs is applied. Generally sick appoint reception of corticosteroids, a dose and duration of treatment the specialist defines individually.
Patients with a myasthenia can periodically have spontaneous remissions, however in most cases the illness becomes aggravated repeatedly. The course of a disease can change during pregnancy. Sometimes the state improves, but also aggravation of symptoms is in certain cases observed.
Prevention of a myasthenia
There are no final data on the reasons of manifestation of a myasthenia today. Therefore, adequate measures of prevention do not exist. However there is a number of data that the postponed infections, craniocereberal injuries, stresses, mental loadings become the reasons of development of a similar illness often. Therefore, as measures of prevention of a myasthenia it is necessary to consider protection from the states described above.