Myasthenia Gravis Myasthenia GRAVIS
Endocrinology / / May 19, 2016
The disease was first described by the English physician Willis in 1672 Myasthenia gravis is more common in adults, at least - in children;uniformly distributed over the floor.Myasthenia gravis is a rare disease.Thus, according to Hurxthal and Musulin, 60 000 patients there is only one case of myasthenia gravis.
Often, but not always, accompanied by hyperplasia of the disease or a tumor of the thymus gland.According to different authors, thymus tumor encountered while suffering from 10 to 25% of cases, and all gland hyperplasia or fraction - in 50-57% of cases.
should be noted that all the tumors of the thymus gland called the calm surroundings regardless of their origin and histological structure.Malignant tumors are relatively rare.According to the data, they make up 2.1% of all tumors and cysts of the mediastinum.According to the frequency in the first place are sarcoma (54.5%), second - cancer (22%) and 23.5% are other breast cancers.Of the benign tumors are described fibroma, lipoma, dermoid cyst
Pathogenesis and causes myasthenia
pathogenesis of myasthenia gravis has not been elucidated completely.Regarding pathogenesis, there are several hypotheses.Supporters exchange hypothesis underlying cause of suffering see in violation of the process of liberation from muscle fatigue products, primarily lactic acid.Authors who hold endocrine hypothesis, the disease is associated with hyperactivity of the thymus gland, which is specifically active substances, possessing the properties of cholinesterase by humoral have influence on the processes of depolarization of acetylcholine.The rapid destruction of acetylcholine hampers pulse to motor neurons, and its transition to the muscle, causing the phenomenon of muscular weakness (myasthenia gravis).
The panel, along with the changes of the thymus gland (thymoma or hypertrophy) often exhibit adrenal hypoplasia and degenerative phenomena (stushevannost cross-striations, and divers paintability muscle fibers, their various sizes) in striated muscle.In addition, the affected muscle cell infiltration found pockets of lymphocytes and lymphoid elements (these lymphoid nodules in the muscles are regarded as a reaction to the violation of the local exchange).No changes in the central and peripheral nervous system can not detect.
characteristic clinical manifestations of the disease are fatigue, severe muscle weakness, extending mainly in chewing, swallowing, oculomotor muscles and facial muscles.These disorders are transient in nature in the beginning, they appear in the normal course of business and disappear after a short rest.If involvement of the facial muscles of the face becomes lifeless, masklike or takes a sinister expression.As a result of the defeat of m.levator palpebrae superioris develop ptosis.This symptom is fairly constant (it is observed in approximately 80% of cases).Much less developed paresis of other eye muscles.As a result of the defeat of the larynx voice becomes weak and sometimes ill very deprived of the ability to produce sounds.Due to fatigue the muscles involved in chewing and swallowing, impaired food act droops lower jaw, salivation occurs, the patient begins to choke on food.Violation of the act of swallowing often leads to aspiration of food pieces in the respiratory tract and the subsequent development of pneumonia.Preferential involvement in the process with myasthenia gravis muscles innervated by cranial nerves (bulbar muscles), was the basis for neurologists called myasthenia gravis "asthenic tabloid paralysis" or "bulbar psevdoparalichom".
As the disease progresses to the above phenomena of fatigue joins the proximal and distal limb muscles less.Smooth muscles are not affected.The disease is sometimes accompanied by emaciation.
noticeable changes in the nervous system are not observed.Tendon and skin reflexes are not changed.The activities of the senses does not suffer.
in blood, besides tendency to lymphocytosis, no changes are detected.Note that in a few cases the literature myasthenia gravis combination with aplastic anemia.Thymectomy in these cases, eliminated the phenomenon of myasthenia and anemia.
At diagnosis attaches great importance to the discovery of the so-called myasthenic reaction.For myasthenia gravis is characterized by irritability and rapid depletion rate by repeated stimulation of muscles faradic current.Myasthenic reaction is detected as in the stimulation of the muscle itself, and in its indirect stimulation through the nerve.X-ray examination in a number of cases it is possible to detect an enlarged shadow (or thymoma hypertrophy) in the anterior mediastinum directly behind the breastbone.In doubtful cases, resorted to the diagnosis ex juvantibus.Fast therapeutic effect of subcutaneous injection of neostigmine confirms the diagnosis of myasthenia gravis.
course and prognosis of myasthenia
Myasthenia gravis is a serious disease with a tendency to a gradual progression.Sometimes there are spontaneous remission.Improving the condition of patients women may occur during pregnancy.The disease usually begins in the young age of its length varies over a wide range (from 1 to 20 years).Patients with myasthenia gravis usually die of aspiration pneumonia or asphyxia, due to the growing weakness of the respiratory muscles.Death may occur from total exhaustion, caused by sharp disorder act of chewing and swallowing.
- Menopause (Menopause) and diseases of menopause in women
- Pathology of menopause (menopause).Climacteric neurosis
- diagnosis and treatment of climacteric neurosis
- menstrual cycle Hormonal
- menstrual irregularities
- The use of estrogen for the treatment of climacteric neurosis
- The use of androgens in the treatment of climacteric neurosis
- morphology of adrenal
- MACROGENITOSOMIA PRAECOX.Diagnosis and differential diagnosis
treatment of myasthenia
myasthenia gravis patients require long-term physical rest.In connection with the violation of chewing and swallowing food should be prepared in the form of a pasty.When complete inability to resort to swallowing nutrient enemas.Because drugs widely used anticholinesterase agents (Neostigmine, galantamine), which have the ability to inhibit cholinesterase activity and thereby prevent acetylcholine from rapid hydrolytic cleavage.
In the literature there are indications of successful application in myasthenia adrenocorticotropic hormone.In the treatment of hormone not just disappear clinical manifestations of the disease, but also dramatically reduced the thymus gland.However, after the treatment of the disease often recurs ACTH.
In cases where therapeutic treatments are ineffective or disease caused by a tumor of the thymus gland, surgery is indicated.
Enough effective treatment for the disease, according to some authors, not inferior to surgery, radiotherapy is on the area of the thymus gland.This method of therapy is particularly indicated in hypertrophy of the thymus gland.