Male (synonym ERB's palsy - Goldflame) - a disease characterized by weakness and fatigue striated muscles.
The reason for infants are metabolic disorders (mainly of violation of the ratio between the acetylcholine and cholinesterase). This leads to increase of cholinesterase in the blood and disturbance of the nervous conductivity. Have the value of the defeat of the thymus gland (hypertrophy her, tumors and other).
Typical and often the initial symptom is ptosis of the upper eyelid and the weakness of the eye muscles, causing double vision. Often violated chewing and swallowing food enters the nose. Celebrated speech disorder: a clear beginning, it gradually becomes quiet and illegible. Depleted facial muscles, and the face becomes expressive. Affects the muscles of the limbs and trunk, develops weakness of the neck muscles and inability to keep his head. Difficulty walking, especially climbing stairs.
The disease occurs in young and middle age and has a chronic course.
In the early stages of the disease, patients complain only to the excessive weakness. In the subsequent case of small muscles cause a rapid depletion of muscle strength, which leads to the violation of active movements up to development paralysis.
The condition is usually worse by the end of the day. After relaxing muscle weakness passes.
The treatment of myasthenia is to assign anticholinesterase drugs.
Applies neostigmine inside 0,015 g 2-3 times a day or intramuscularly in a dose of 1 ml of 0.05% solution.
The effect after oral administration comes after 20-30 minutes, when parenteral-faster and lasts 2-3 hours. Neostigmine inactivates holinesterzu and increases the effect of accumulating in the body of acetylcholine. Similar, but more prolonged action has oksazil (up to 5-10 hours. He was appointed to 0,005 grams inside. Starting with a single tablet, increase the dose depending on the effect to 2-3 times a day. This treatment can be alternated with the appointment of galantamine (0.25 ml to 1 ml of 1% solution subcutaneously).
It should be borne in mind that prolonged use of large doses of anticholinesterase drugs can suddenly occur, dangerous for the life of the patient disorder in the form of a crisis (anxiety, cramps, sweating, increased salivation, weakness, respiratory arrest, coma). In this case it is necessary to stop the use of anticholinesterase drugs and enter atropine. In the development of acute myasthenic crisis (see Crises) in severe disease with impaired breathing and swallowing resort to tracheostomy, intubation and controlled breathing. Recently extended surgical treatment of myasthenia (removal of the thymus gland or denervation carotid sinus)that gives a good effect.
Cm. also atrophy of the muscle, the Tabloid paralysis.

Male (myasthenia; from the Greek. mys, myos - muscle and asthenia weakness; synonym: myasthenia gravis pseudoparalytica, asthenic bulbar paralysis, disease - Erba goldflama) - the weakness and pathological fatigue striated muscles.
The disease is described by Albom (W. Erb) and Goldflame (S. S. Goldflam).
Feature typical infants changes elektrovozoremontnij [Galli (J. Jolly)], reflected in the rapid depletion, muscles at repeated irritation faradic current and recovery excitability after rest.
Pathology and pathogenesis. The question about changes in the nervous system is not solved, as described cases in which destruction could not be found, and cases with changes in the different departments of the Central and autonomic nervous system. A variety of conditions thymus - thymoma, hyperplasia tissue cancer, persistent thymus is found in infants often. Very frequent changes in muscles [perivascular round-cell infiltration ("lymphorrhea")]; these changes are found in the liver, kidneys, lungs, adrenal glands.
Theory of pathogenesis of infants based on modern data about mechanisms of neuromuscular stimulation and on exceptional effect when using drugs, which are the antidotes curare. For neuromuscular function is critical ratio of acetylcholine and cholinesterase. It is established experimentally that acetylcholine is a neurotransmitter in the process of the proceedings, while under the influence of the enzyme cholinesterase breaks down acetylcholine. The antidotes curare (ezerin, prostigmin) have anticholinesterase activity, contribute to the accumulation of acetylcholine, as delaying cholinesterase activity. Proposed two main theories of impaired movement in infants: 1) hindering factors is the presence in the blood or tissues of a blocking substances; 2) the lack of blood stimulating factors (acetylcholine). Metabolic disorders if M is not limited to the exchange of mediators, have also been violated tissue respiration, salt and creatine-creatininase currency.
In the pathogenesis of M. known is the role of the thymus gland. Later, Weigert (Laquer,C. Weigert) described Meters in malignant tumors of the prostate. Women have myasthenia 2 times more often than men; during pregnancy and menstrual period myasthenic disorders increase, which indicates that participation in the development of M and other endocrine glands. Myasthenia as a violation of intimate processes of neuro-muscular stimulation level mionevralnuu synapse caused by disorder of the Central regulation, most likely in the area of diencephalic, mediator, and other types of currency, as well as violation of the endocrine functions.
The clinical picture. When M is described muscular atrophy and sensitivity disorders. There is a certain selectivity and consistency in the development of pathological fatigue muscles at the monastery the First and hardest hit swallowing, chewing, speech and oculomotor muscles. Ptosis is a typical symptom because the muscle lifting the upper eyelid is affected most often. Myasthenic changes can be observed in many of the body's muscles. Proximal muscle groups of the hands and feet suffer stronger distal. The defeat of the muscles involved in breathing, may induce in severe cases breathing disorder, life-threatening. Especially dangerous is the defeat of the diaphragm. Radiographically detected were typical for infants violations in the heart muscle and smooth muscles of internal organs. M has a progressive course, but often observed remission for several months, rarely years. Typical is the variation in the severity of symptoms during the day with the best state in the morning after sleep; resulting in fatigue muscle weakness takes place after the holiday. M. more often sick at a young age, children are sick often. If diagnosed M should be differentiated from bulbar palsy, congenital ophthalmoplegia, stem arachnoiditis, bulbar form of progressive muscular dystrophy and some other diseases. Characteristic of M. reaction to neostigmine with a temporary reduction (disappearance) of pathological phenomena is of great diagnostic value. To distinguish the male from the myasthenic syndrome when encephalitis is very difficult.
Treatment. The basic method of treatment of myasthenia - anticholinesterase drugs. Neostigmine used per os and intramuscularly. Single dose inside 0,015-0,03 g; parenteral injected 1-2 ml of 0.05% solution in water. The ingestion effect occurs in 20-30 minutes, when parenteral introduction - faster. Depending on the severity of injury prescribe to take 1-2 tablets 2-6 times a day. You can combine reception prozerina inside with intramuscular injections. Similarly proserina act galantamine, revenge-non and other Galantamine is prescribed in the form of subcutaneous injections in the dose of 0,0025 to 0.01 g potassium Chloride (10% water solution by teaspoonful 4-5 times a day) increases the effect prozerina. Positive results give surgical removal of the thymus gland and exposures her x-rays.

Radiotherapy is performed with high doses -200 R a session every day, just 4000-5000 p (a control blood). Known positive results during radiotherapy diencephalic the field of low-dose - 30-50 R 2 times a week, at a rate of 8-10 exposures.
Surgical treatment of myasthenia. It is now recognised that the only way of surgical treatment of M is thymectomy. The indication for surgery are: 1) thymoma, hyperplasia of the thymus gland, established by pneumomediastinum, or suspected presence of the latter; 2) male, not amenable to therapeutic treatment, in particular to maximum doses of neostigmine methylsulfate; 3) relapses after radiotherapy; 4) life-threatening acute attack infants (C. A. Gmur and L. A. Anokhin).
After a thorough preoperative preparation, consisting in increased medication (neostigmine) and oxygen, to make a removal of the thymus gland under endotracheal anesthesia. For the anesthesiologist complicated issue is the choice muscle relaxant, as in patients with myasthenia sensitivity to relaxants antidepoliarizuth type (d-tubokurarin, diplatsin, premion) increased by 10-20 times, and depolarizing relaxants type (suktinilholin, decamethonium) often cause double block. Most of anesthesiologists recommends that you only use depolarizing relaxants actions in minimal doses. For anaesthesia preferable to apply nitrous oxide or cyclopropane, and not broadcast to not strengthen curariform actions relaxants. When applying halothane you can do without muscle relaxants.
Preferred access - longitudinal sternotomy (see the Thymus gland, clinic and treatment of tumors).