Meals for Miasthenia. Miasthenia: Modern approaches to the treatment of vitamins for miastic

Miastic - chronic neuromuscular disease, manifested by recurrent or gradually progressive type of flow. People who made miashenia suffer from increased muscle fatigue, which at the end of the day sometimes leads to a pronounced part of the muscles affected by the pathological process.

The reasons

There is no single point of view of the causes of this disease. At various times, hereditary, infectious, autoimmune theories were expressed. There is also a clear connection between the development of this state with a tumor of the fork gland (thymus), although the latter is not always available, and its removal does not always lead to an improvement in the state.

It should be said that the risk of sick miastenia is significantly increased with a family history. However, in the same family in one family, very often the manifestations of Miasthenia vary from the minimum (eye form of Miasthenia) to the highest possible (miastic gravis).

The disease is sufficiently common, the frequency of development of myasthenia varies from 5 to 10 people per 100,000 population. There is more often a woman (in a ratio of 2 to 1), the age of debut of the disease is mainly accounted for by 15-45 years. Cases are described when the disease occurs in children. More often there is an early muscular weakness that passes during the first month of life.

It should be noted the possibility of the development of miasthenic syndrome on the background of existing chronic diseases: dermatomyosis, tumors of endocrine organs, systemic diseases of the connective tissue, etc.

Symptoms

Symptoms have a variety of severity, localization, but they can be characterized by one phrase - muscle weakness. Depending on the localization of the process and the severity of manifestations, several forms of myasthenia are distinguished:

• Eye shape.
• Bulbarm.
• Generalized form (Miasthenia Gravis, Erba Goldflam's disease).
• Malignant lightning miastic.
• Miasthenic crisis.

Eye of the Miastic manifests itself, above all, the development of the weakness of the century, often on the one hand or uneven on both sides. At the same time, at the beginning of the disease, the eyelid goes closer towards the evening, after the auditorium. Freshness in the eyes and other transient violations of vision are often noted. If in two years from the moment of the debut of the disease, the process has not moved to other anatomical zones, then further generalization becomes unlikely. Localized myasthenia (eye shape) is not dangerous for life.

The bulbar form of miasthenia is characterized by the development of mild sky muscle weakness, language, pharynx. The patient suffers from violations of speech, violation of the act of swallowing. In this case, changes are dynamically changed during the day, with different loads, which distinguishes this form of the disease from true bulbar syndrome.

The generalized form is a common weakness of muscles. It all begins, as a rule, with the muscles of the neck, the sick person becomes difficult to keep his head, gradually the disease passes to other muscle groups, paresis of the muscles of the limbs begins to manifest. The patient is disturbed by the gait, Mimic is distorted. There is a noticeable improvement in the morning in the morning, after day sleep, after a long rest. The slightest physical exertion sometimes lead to severe weakness.

The malignant type of flow of the disease is available only in the presence of Timus tumor, often malignant. The disease debuts from age about 50 years and for a short term (months, and sometimes even the week) leads to complete disability. At extreme stages, skeletal respiratory muscles affects the likelihood of life-degrading states.

Miasthenic crisis is a special manifestation of a generalized form of this pathology. The exact causes of the chip is not detected, it develops more often on the background of menstruation, psycho-emotional overvoltage, fever or other reasons. Insufficient dosage of medicines in disease therapy may also lead to the development of complications. When crisis, there is a sharply pronounced weakness of all muscles, including respiratory. The duration of the crisis varies very widely and can proceed as several dozen minutes-hours and weeks, and in some cases months.

The difference between the miasthenic crisis from the cholinergic.

Diagnostics

As a rule, the diagnosis of the disease is not difficult, because all manifestations of myasthenia are defined quite characteristic. Also, even with a standard neurological inspection, it is possible to identify quick muscle fatigue, the miasthenic reaction of tendon reflexes and gradual exhaustion. Special tests and additional research methods are also used:

The testess of the eyelid - there is a characteristic view from under the lowered age, a man rarely can look with open eyes, the eyelids are quickly lowered.
Cooling test. It is done more often on centuries. A piece of ice is put on one eyelid, after cooling there is an improvement in the work of the century.
Transportation test. (There will be a section in symptoms-syndromes, and the request to check) the patient is administered subcutaneously 2-3 ml of prozermina 0.05% solution. During myasthenia, the function of affected muscles is almost restored after half an hour. This state sometimes comes to the original no earlier than 2-3 hours.
Allows you to identify the characteristic miasthenic reaction of neuromuscular transmission, reveal the quick fatigue of the muscles when they are re-stimulated.

Treatment

Miasthenia treatment is not the most simple task. It is necessary to immediately adjust the patient to a permanent or very long intake of anticholinesterase preparations acting on the pathogenesis of the disease. Calimine preparations, and less often used prozer, should be carefully dosed, given the body weight, the severity of symptoms, age and other factors.

Often they resort to hormone therapy, especially with acute development of symptoms and their rapid progression. Most often spend high doses pulserapy, with a further transition to minimum dosages. It is also advisable to conduct plasmapheresis sessions (blood purification from circulating autoimmune complexes) against the background of the use of pulserapy hormones (methipred, dexamethasone, etc.).

The treatment of a miasthenic crisis should be started with the maintenance of vital functions, gradually moving towards the rate of plasmapheresis, hormone therapy, refinement of the presence of tumor tumor. The presence of a timom (especially growing) in combination with frequent miasthenic crises is an indication of the operational removal of the timomy. Unfortunately, only about 50% of patients note the improvement of the state.

Also, the therapy of Miasthenia should include drugs of pure potassium (in no case Panangin, because magnesium can lead to the weighting of symptoms!), Purpose of potassium-saving diuretic (Verosampiron), metabolic preparations (riboxin, group vitamins B). Announced the appointment of physiotherapy, special exercise of the exercise of the exercise, massage.

List of drugs that can not be used during myasthenia

It should also be strictly followed by special approaches of therapy of other diseases. There is a list of drugs that cannot be used during myasthenia:

What if pregnancy? ..

It should be noted that pregnancy is not a contraindication to the appointment of the main drugs for the treatment of myasthenia. During pregnancy, the hormone therapy, plasmapheresis sessions, the use of large doses of potassium-containing drugs should be abandoned.

Nervous muscular disease with autoimmune development is Miasthenia - from Latin Myasthenia Gravis. Ten people out of 100,000 suffer from this ailment. More than 50% of patients achieve remission.

The reasons

Some are predisposed to the emergence of Miasthenia - a risk group. It includes:

• Young people aged 20 - 40 years;
• Female Paul - According to ladies statistics, they are sick 3 times more often than men, but in adulthood these indicators are equal.

The main reason for the development of myasthenia is an autoimmune response of the body on its own fabrics. Such a process can run:

Depending on the provoking factor of Miasthenia, it is divided into 2 large classes:

• Congenital;
• Acquired.

The first view is the outcome of the genetic mutation. A failure leads to a violation in the contractile function of the muscles. The second form is often a consequence of Timus tumor. Miastiy can affect muscle fibers of any body department. More often occurs the eye form of the disease. Children rarely suffer from this ailment. From the total number of patients, they constitute less than 3%.

Clinical picture

Whatever muscle group seized the disease, patients will celebrate general symptoms in the affected area:

• Excessive fatigue;
• Increased weakness;
• Reducing performance and functionality.

Pathological focus causes a person discomfort. This defective part does not cope with the tasks assigned to it. Muscles of any body department can suffer. But especially the eyes are involved in the process. It brings a patient a lot of inconvenience. However, rest for muscles, removing voltage from them, delivers relief. But it is short.

Over time, even a long-term relaxation will not reduce the severity of the patient's symptoms. Progress in the field of pharmacology allows patients to deal with the ailment. The following forms of miastic are allocated in clinical practice:

Diagnostics

In order to fix the fact of the disease, the doctor is not sufficiently symptoms. Any study of the pathological process is not limited to instrumental methods. The doctor examines the patient, finds out the clinical signs of the ailment. He studies the patient's disease. And then assigns a laboratory and tool diagnostics. A standard set of analyzes for identifying Miasthenia consists of the following:

• Functional samples aimed at detecting pathological fatigue muscles;
• Electromyographic study, which indicates activity in the affected areas;
• Decrement test, which allows you to identify the blockade of the neuromuscular signal, and at the same time the severity of the process;
• Transversive sample;
• Biochemical analysis of blood;
• Immunograms;

With difficulty making a diagnosis, a differential study may be assigned by:

• Needle electromyography;
• Study of the conducting functions of nerve fibers;
• Electromiography of certain muscles - jitter.

Treatment

The choice of the therapy line during myasthenia depends on the set of factors:

• Forms of the disease;
• Patient states;
• Concomitant pathologies;
• Prevalence of the process.

Medical treatment is prescribed standard. However, in some cases, surgical intervention is recommended. Autoimmune processes in thymus can cause a timectomy operation. Among the effective medicines are known for "Prezer", "Kalinin" and drugs with a high content of potassium. And also prescribe medicines that stimulate immunity.

To symptomatic treatment, which facilitates the patient's condition, includes the following medicines:

• Anticholinesterase - "Ipigrix";
• Cytostatics;
• Glucocorticoids - "prednisolone", "Metipred";
• Immunoglobulins.

In the case of fast-crossing changes, an extracorporeal hemocorrection is prescribed - a method that helps purify the blood of the patient from antibodies against its own tissues. Even the first procedure gives people a chance to feel improvement. Further therapy helps to achieve a resistant effect.

The effective method is cryosophoresis. This procedure allows you to clean blood from malicious substances, influencing with a reduced temperature. Such treatment is carried out by the course for 5-7 days in a row. Wide distribution received a cascade plasma filtering method. This procedure is carried out using nano cleaners. They purify blood, and then returned to the patient.

Another modern method of treatment of myasthenia can be considered an extracorporeal immunopharmacotherapy. It involves the extraction of lymphocytes in the patient, their medication and subsequent departure to the blood system. With this technique, it was possible to cause persistent remission in patients during the year. It is especially important to adhere to the recommendations of the doctor, since during myasthenia there are contraindicated drugs, the use of which is fraught with hazardous consequences.

Eye form

One of the most common types of disease is orbital. Often it is from her that the Miasthenia process begins, and then applies to other organs. The main symptoms that celebrate the sick:

• Diplopia, that is, bias in the eyes. Patients see not one holistic image;
• Reduction of acuteness and clarity of view;
• Violation in rotational and motor functions of orbits;
• Ptosis, that is, the omission of the eyelids. As a result, the eye slot is not able to open and close normally.

All signs described may be distributed both on both orbits. Usually, closing the eyes for a short time, patients experience relief. However, the slight voltage associated with reading or watching the TV causes discomfort.

Bulbarm

This type of myasthenia can be dangerous to the patient's life. He entails:

• Dysphony - vocal function disorder;
• Dysphagia - a violation of swallowing;
• Dysaryry - disorganization in the work of the muscular apparatus of the pharynx, larynx, as well as soft nose.

The symptomatic manifestations described are dangerous consequences. Dysphagia can move into full inability to swallowing. Food list for such patients is extremely zudd. Food appoints a doctor. Patients have to feed through the probe, they lose weight and weakening. So, their general condition worsens that it does not contribute to recovery.

Disorder of voice formation reduces the social scope of patients. And the dystellium can cause fatal outcome due to respiratory disorders caused by pararesis of voice ligaments that closed the larynx. This is fraught with asphyxis - choking.

Generalized form

The most unfavorable view of the disease is systemic, that is, common. This dangerous type of miasthenia invariably causes up to 1% of deaths among patients with this pathological process. The generalized form captures a large number of muscles, including respiratory facilities - it can cause insufficiency and death when noting assistance.

This disease is often accompanied by the prevalence of the process. Over time, the limited form progresses to systemic. And although patients are frequent remissions, they, as a rule, arise and end suddenly. Therefore, miasthenic episodes and conditions are isolated.

The first quickly begin and end. The second is a long-lasting process, up to several years. However, such a miasthenic state is not prone to progression.

Contraindications

Patients suffering from this ailment are forced to have some restrictions. These include:

• Excess physical exertion;
• Insolation, that is, staying under the right sunny rays;
• Medicinal products with magnesium - "Magnesia" and "Panangin", "Asparkam";
• Miorlaxants stripped;
• Neuroleptic, tranquilizers and preparations that enhance their action - "guides", "Corvalkaps";
• Diuretic, with the exception of "Veroshpiron" and "Spironolactones";
• The use of certain antibiotics according to the type of aminoglycoside - "gentamicin" and "streptomycin", fluoroquinolones - "ENUCSACIN" and "CIPROFLOXACIN";
• Vaccinations.

Contraindicated drugs during myasthenia should not be ignored. There are tables and listings of medicines that answer the question "What is the antibiotics during myasthenia?". Funds should be avoided, which in the list of complications have this disease. These drugs include "glutalit". And it means the use of these tablets is contraindicated. Compliance with the prescriptions of the doctor - a deposit to a favorable course of the disease.

umozg.ru.

Principles of treatment of bronchopulmonary infection in patients with myasthenia

Miasthenia is a classic autoimmune disease, which is based on an autoimmune process, directed against the acetylcholine receptor of the postsynaptic muscle membrane. The leading link in the development of miastation is a violation of neuromuscular conductivity, which is clinically expressed in the development of increasing muscle weakness of various localization. Currently, great successes have been achieved in the study of the pathogenesis of the disease, in diagnosis, in the approaches of the conduct and treatment of various groups of patients with myasthenia. However, despite the pathogenetic therapy, there are no specific approaches to the full cure of this disease. The outcome of the development of the disease depends on the effectiveness of selected therapy and the rate of progression of increasing muscle weakness, which can lead to the complete non-confidence of certain muscle groups.

The most clinically significant in patients with Miastenia is the involvement in the pathological process of the main and auxiliary respiratory muscles, which, ultimately, is expressed by varying degrees of respiratory failure. These changes lead to a violation of the ventilation function of the lungs, causing stagnant phenomena, which are "favorable" for the development of an infectious-inflammatory process. In a study conducted at the University Hospital of John Hopkins since 1990-1998, it was shown that in 46% of patients experiencing certain respiratory violations against the background of Miasthenia, pneumonia develops.

The importance of the development of an infectious process is associated with the treatment conducted in patients of various forms of miastic. In addition to the main therapy with anticholinesesese drugs, the treatment of myasthenia is aimed at suppressing the activity of the autoimmune process, which is carried out by suppressing the general immune response of the body. The methods of treatment of myasthenia include the use of pulse-therapy with glucocorticosteroids, the use of cytotoxic immunospressants (azathioprine, cyclophosphamide), thymectomy. The listed treatment methods in connection with the suppression of immunity create an additional background for the development of bacterial complications. It is important to note that for some drugs, such as cyclophosphane, a direct toxic effect on the pulmonary tissue is marked. Thus, the role of infection during myasthenia is obvious.

The relevance of the development of bronchopulmonary diseases is related to the complexity of the treatment of such complications in patients with Miastenia. Some drugs that are applied in standard practice in the treatment of concomitant diseases (stripping drugs, D-penicillamine, interferon-alpha, magnesium salts, beta blockers, calcium channels, etc.), contraindicated during myasthenia due to their inhibitory effects on neuromuscular conductivity. To preparations that are absolutely contraindicated during miastic, also include some antibiotics: aminoglycosides, macrolides, fluoroquinolones. This imposes certain limitations in these patients to the treatment of infectious complications (IO), as already noted earlier, so often manifested by the respiratory system.

Thus, due to the complexity of treating IO in patients with Miastenia, there is a need to search for new approaches in the therapy of this group of patients. Unfortunately, in the preparation of materials, there was no English-speaking literature, visiting this problem.

The purpose of this study was to study the microbiological landscape of sputum, tracheobronchial secret and assess the clinical efficacy of antibiotic and immunotherapy in patients with infectious inflammatory diseases against the background of Miasthenia.

Materials and research methods

A group of research amounted to 19 patients with a generalized form of miasthenia (6 men, 13 women; the age of patients was from 22 to 81); Of these, 3 men, 7 women - with exacerbation of chronic tracheobronchita, 3 men, 1 woman - with residual signs of postponed hospital pneumonia; 2 women - with chronic obstructive bronchitis; 1 woman - with Lambert-Iton syndrome; 2 women - with bacterial focal pneumonia (one of them is a timectomy in an anamnesis). The clinical signs of the exacerbation of the infectious process were the following: cough with mucous-purulent sputum, an increase in its products, shortness of breath, fast fatigue, in some patients there was a subfebilitation. Before the start of treatment, a generally accepted clinical and laboratory study was carried out, the study of the bacteriological composition of sputum, the secret from the trachea (or tracheostomas), studies of the indicators of the function of external respiration (FVD), radiography or computed tomography (CT) of the chest.

Samples obtained from patients were delivered within 2 hours to the bacteriological laboratory, where the microscopy of the smear was conducted to assess the quality of biomaterial and sowing on standard nutrient media. Samples of sputum were considered acceptable if the number of leukocytes was greater than 25, and the number of epithelial cells is less than 10 in one field of view. When sowing selected pathogens, a species identification was carried out (BBL Crystal test system). Antibiotic sensitivity of microorganisms was determined by diffusion in agar.

The severity of the state and the difficulty of treating patients are caused by immunodeficiency and yatrogen. So, in one patient there was stenosis of the trachea, which was developed due to long-term artificial ventilation of the lungs, carried out in connection with the miasthenical crisis. Another patient had a language cancer, in connection with which the patient was performed by open tracheostomy. In these cases, the situation is aggravated: immunological protection is reduced, the evacuation of the tracheobronchial secret is disturbed, which contributes to the colonization of nosocomial resistant strains of microorganisms and the development of purulent-infectious complications. And in one patient, the severity of the state was aggravated directly by the reception of the anticholinesterase preparation of pyridostigmine (Calimin) appointed during myasthenia. Against the background of taking a pyridostigmine in a patient, a sputum is increased to 300 ml / day. In this regard, the patient was forced to abandon the reception of the drug and independently carry out positional morning drainage.

Auscultative picture was scarce: Breathing vesicular, weakened in the lower dealers of lungs, local wet and scattered dry wheezes, breathing frequency alone 18-20 per minute.

In the study of the FVD, ventilation disorders were revealed. The decrease in the FEV1 was an average of 60% to 49%.

As antibiotic therapy, all patients were obtained cefoperazone / sulbactam intravenously or intramuscularly at a dose of 1.0 g 2 times a day. The duration of therapy was 7-10 days (depending on the degree of activity of the infectious process). In order to improve the rheological properties of sputum, all patients prescribed mercolytic (acetylcysteine \u200b\u200b(floimumucil) of 300 mg 2 p / day) through the nebulizer or PER OS.

The human intravenous immuno-optic treatment program was included in the treatment program with an immunocyal target. G (VVIG: Oktagam, Biaven V. I., Octaglobin). The treatment was carried out against the background of basic basic therapy, including methylprednisolone, pyridostigmine and potassium chloride.

It seems to us important to describe the clinical case in this study. The patient of 74 years was diagnosed with "generalized Miasthenia" from December 2010. In terms of gravity, 80 and 40 mg of methylprednisolone acted on an alternating daily scheme. He turned in August 2012 with complaints about sharp weakness, quick fatigue, increasing shortness of breath with a minor physical effort. In an objective examination of the patient, the state of moderate severity, skin covers without visible pathology, peripheral lymph nodes are not increased, the feet of pastous. At percussion of the chest, a box sound was determined, an excursion from both sides - 3 cm (1.5 + 1.5). With auscultation - Breathing in the projection of S4-5 segments, S9 on the right is sharply weakened, wheezing were absent, the number of respiratory movements was sitting 18 per minute. Heart rate - 85 per minute, hell - 130/85 mm Hg. Art., Temperature 36.8 ° C. Palpation of the abdomen is painless, abdominal organs are not increased. The results of the laboratory study are presented in Table. 1. On the presented images of the CT of the chest organs on the left in the projection of the S1-2 segments, the general formation of irregular shape with uneven, somewhat infiltrated circuits on the background of a limited pneumophibrosis (Fig. 1) is determined. On the left in the projection of the segment S9 is a large infiltration of the inhomogeneous structure (Fig. 2). Diagnostic bronchoscopy was produced: bronchi is passable, mucous pale pink color, atrophichna. Conclusion: Chronic atrophic tracheobronchite II Art.

So, in the patient against the background of myasthenia, pneumonia was developed with the destruction of pulmonary fabric. The etiological factor to identify was not possible due to the lack of sputum. It is important to note that against the background of long-term reception of high doses of methylprednisolone, the patient has symptoms of thrombocytopenia, manifested by skin bruises on the body, a course of complex antibiotic and immunotherapy has begun. Intravenously drip was introduced cefoperazone / sulbactam for 10 days. At the same time, VVIG was introduced, the term dose was 15.0 G. Therapy with the inclusion of VVIGA made it possible to achieve a more rapid remission of the infectious and inflammatory process, confirmed by clinical and laboratory data and the results of repeated CT studies of the lungs, where the positive dynamics of the pathological process was noted (Table 1) : Increased platelet levels - up to 131 × 109 / l, decreased leukocytosis to 15.0 × 109 / l, reducing the level of C - reactive protein - up to 5.0 mg / l.

The patient consulted by an endocrinologist: complaints against back pain when walking and exercise, a decrease in growth of 4 cm for 3 years, a sense of fatigue in the back sitting; In case of inspection, breast kyphosis is determined, the results of laboratory surveys are presented in Table. 2.

According to radiography of the spine in the lateral projection, compression fractures of the 1st, 2nd lumbar vertebrae were revealed. Twonergetic X-ray absorption: bone mineral density according to T-criterion in the lumbar spine - 3.0 SD, bone mineral density according to T-criterion in the hip neck - 2.0 SD.

Diagnosis: drug (glucocorticoid) osteoporosis with compression fractures of vertebral bodies. Recommended: rational nutrition with an increase in the use of products rich in calcium and vitamin D, maintaining adequate physical activity and exercises with a load corresponding to health status; Alfakalcidol (Alpha D3-TEV) 0.75 μg daily, ibandonic acid (bonviva) 3.0 ml Bolunno 1 time every 3 months.

Results and discussion

24 samples of sputum and bronchial secretions isolated in patients with bronchopile diseases were studied. The main clinically significant microorganisms were: S. pneumoniae (33.4%), S. aureus (20.8%), S. pyogenes (12.5%) (Fig. 3). Among the non-enzyme gram-negative bacteria were P. Aeruginosa strains (12.5%). In four samples of biomaterial, a microbial association was observed: P. Aeruginosa and Candida Albicans mushrooms and in one KL sample. Pneumoniae + S. Pneumoniae. Of great interest is the study of the sensitivity of the dedicated pathogens to cefreson / sulbactam. It was noted that all strains of microorganisms were entered into the antimicrobial spectrum of the action of cefoperazone / sulbactam; And only in one sample (P. Aeruginosa + Candida albicans) the antibiotic showed weak activity to the dedicated pathogen.

So, a clinical study of sputum in patients with exacerbation of chronic bronchitis and pneumonia against the background of Miasthenia made it possible to identify the heterogeneity of the microbiological landscape. The leading pathogens were gram-positive pathogens, such as S. Pneumoniae, S. Aureus, S. pyogenes (which amounted to 66.7%). Cefoperazone / Sulbaktam showed high activity to these strains of microorganisms. At the same time, the growth of gram-negative pathogens P. Aeruginosa and Kl was observed. Pneumoniae (12.5%, respectively), which were sensitive to cefoperazon / sulbactam. Some gram-negative microorganisms were represented by P. Aeruginosa association with mushrooms of Candida genus (4.2%), KL. Pneumoniae with S. pneumoniae (16.7%); In such cases, fungicidal therapy with fluconazole (diphlucan) was prescribed, which significantly improved the course of the disease.

The infectious process of the respiratory tract in this group of patients proceeded torpidly, despite adequate antimicrobial therapy. It is known that during myasthenia there is a general depression of the immune system due to the oppression of both specific and non-specific protection factors, which requires the correction of immune "breakdowns".

It is important to note the basic properties of the used antibiotic. Cefoperazone / Sulbactam showed activity both to gram-positive and gram-negative microorganisms, including P. Aeruginosa. In addition, the antibiotic is stable to the action of beta lactamas, due to an irreversible inhibitor - sulbactam, synergisticly increasing the efficiency of the antibiotic used (the minimum overwhelming concentration of cefoperazone is reduced to the strains of microorganisms sensitive to it). It is of great importance to the tolerability of patients with drug, i.e. the antibiotic does not affect neuromuscular conduct.

So, in addition to disinfecting, antibacterial and other types of therapy, VVIG is currently widely used with different localization of the infectious inflammatory process. VVIG (Oktagam, Biaven V. I., Pentaglobin, etc.) contain the repertoire of antibodies capable of inactivating a wide range of antigens. Immunoglobulins are a factor of the acquired immunity, which performs the overting of infectious foci, contributes to the rapid elimination of the bacterial agent and its metabolites, activating the entire cascade of immune reactions.

Conclusion

Many years of experience allows the use of VVIG in complex therapy of a bacterial infection of the respiratory tract. The course of the disease is significantly improved, the period of cure of patients is accelerated, and most importantly, the risk of colonization of microorganisms resistant strains is reduced and, accordingly, the risk of repeated episode of infection.

Literature

1. Sannovze A. G. Miasthenia and Miasthenic syndromes. 2012, p. 252.
2. Shcherbakova N. I. Pathogenetic substantiations of the strategy and tactics of the treatment of Miasthenia (the abstract of the doctoral dissertation). 2007, p. 3-50.
3. Varelas PN, Chua HC, Natterman J., Barmadia L., Zimmerman P., Yahia A., Ulatowski J., Bhardwaj A., Williams Ma, Hanley DF Ventilatory Care In Myasthenia Gravis CRISIS: ASSESSING THE Baseline Adverse Event Rate // Crit Care Med. 2002, DEC; 30 (12): 2663-2668.
4. Sulkowski S., Sulkowska M. Alveolar Cells in Cyclophosphamide-Induced Lung Injury. II. Pathogenesis of Experimental Endogenous Lipid Pneumonia // Histol Histopathol. 1999, Oct; 14 (4): 1145-1152.
5. Sannadze A. G., Sokolova V. I., Shcherbakova N. I., Nikiforuk N. M. The effectiveness of the use of minimum doses of immunoglobulin in the treatment of severe miastic, complicated by abscessive pneumonia // Actual issues of clinical transport medicine. 2001, vol. 6: p. 280-286.
6. SKEIE G. O. ET AL. Guidelines for Treatment of Autoimmune Neuromuscular Transmission Disorders // Journal of Neurology. 2010, 17: 893-902.
7. Serrano M. C., Rabinstein A. A. Causes and Outcomes of Acute Neuromuscular Respiratory Failure // Arch Neurol. SEP. 2010, Vol. 67 (No. 9): 1089-1092.
8. Latysheva E. A., Latysheva T. V. The use of intravenous immunoglobulins in intensive therapy // General resuscitation. 2012, VIII; 3: 45-49.

V. I. Sokolova, Candidate of Medical Sciences A. G. Sannadze, Doctor of Medical Sciences, Professor D. A. Sychev1, Doctor of Medical Sciences, Professor M. B. Babarina, Candidate of Medical Sciences D. A. Bane

GBOU DPO RMAPO MH RF, Moscow

www.lvrach.ru.

Generalized Miastration Gravis: Causes of increased muscle fatigue

Miastic gravis is a severe autoimmune pathology, accompanied by a progressive muscle weakness. At the same time, only cross-striped muscle fibers are affected. Cardiac and smooth muscles remain normally. When identifying the first symptoms of such a disease, contact a specialist immediately.

Pronounced weakness - the main symptom of miastic

Causes of development

For the first time, this disease was described by the British physician Thomas Willis in the second half of the XVII century. Currently, the exact causes of its development are already known. Miasthenia is formed in cases where the body's immune system begins to produce antibodies to its own acetylcholine receptors of the post-sheinctic membrane of neuromuscular compounds. It is due to such synapses that the nerve pulses of the transverse muscles take place.

During myasthenia, the activities of synapses transmitting nerve impulses on transverse-striped muscles are violated

A starting factor in the development of an autoimmune response is able to become any infectious disease of the upper respiratory tract, strong stress, as well as a violation of the work of the nervous system.

Another cause of this disease can be biochemical shifts in the work of neuromuscular synapses. This can occur against the background of violation of the activities of the hypothalamus and the fork gland.

All causes described above contribute to insufficient developing or excessively rapid destruction of acetylcholine, thanks to which the nerve impulse is transmitted to the cross-striped muscular cell.

Violations of Timus's activities can lead to the development of myasthenia

Note. Cross-striped muscle fibers differ from smooth and heart muscles by the fact that a person controls their activity on their own.

To date, it has been established that the Miastration is not inherited. It is more often formed in women of young age (20-40 years). The prevalence of such pathology is approximately 5 cases per 100,000 people.

Clinical picture

Currently, several types of this disease are distinguished. Each of them is manifested by its symptom. The main forms of pathology are:

• bulbar Miastic;
• eye-eye miastic;
• generalized myasthenia.

The easiest is the eye form of the disease. It is characterized by the following clinical manifestations:

1. The omission of the century on the one hand (this is the initial sign, which is further characteristic and for the second century).
2. With active blinking, the eyelid begins to fall even lower.
3. As a result of "hanging" one of the century there is a bone in the eyes.

This course of the disease occurs quite rarely. More frequent is a bulbar form of miastic. It will be characterized by the following clinical picture:

1. The patient quickly gets tired with prolonged meals.
2. At the same time, his voice becomes husky, bent. In the future, it becomes difficult to pronounce the letters "C", "P" and "W".
3. If we receive food do not stop in a timely manner, then the person loses the ability to swallow, and his speech may become almost silent.

In case of bulbar and generalized Miasthenia form, a patient has a number of characteristic external signs.

Note. Patients with bulbar violations during myasthenia are trying to eat at the peak of the action of medicines.

More often, the generalized form of Miasthenia is found. Such pathology implies the presence of the following symptoms:

1. The disease initially affects the eye muscles and only then into the pathological process involved cross-striped muscle fibers of other localities.
2. The patient becomes amymic.
3. He is hard to keep his head smoothly.
4. There is a salivation of mouth.
5. In the future, the patient becomes hard to walk long. Over time, this symptom is aggravated. A person becomes difficult to even get up and served himself.
6. Over time, muscle hypotrophy arises, especially pronounced in the limbs.
7. The severity of tendon reflexes is reduced.

Important! A feature of Miasthenia is the fact that after a sufficiently long rest or sleep all these symptoms become less pronounced and a person feels significantly better.

During sleep and rest, the stocks of acetylcholine in synapses are restored and the patient feels better

Diagnostic features

In the event that the characteristic manifestations of myasthenia arise, it is necessary to consult a doctor-neurologist. This doctor knows exactly what is the disease and how to confirm or refute its presence. Miasthenia diagnostics includes the following steps:

1. The patient's survey (not only the main complaints, but also all the circumstances preceded by the development of pathology).
2. Clinical inspection.
3. Conducting functional samples to clarify the presence of a quick muscle fatigue (for example, the patient is asked to quickly frog).
4. Electromyographic study.
5. Conducting the transversive sample.
6. Re-electromyographic study (is carried out in order to clarify how the transversive test influenced muscular activity).
7. Repeated clinical examination (it is carried out in order to determine the degree of influence of the transversive sample on muscle activity).
8. Blood test for the presence of antibodies to acetylcholine receptors and titin.
9. Holding computed tomography of Timus.

Diagnosis of Miasthenia implies the use of modern medicine achievements

After all these diagnostic events, the doctor either establishes the diagnosis or refutes it.

Note. In some cases, fewer diagnostic measures are carried out, especially when there is a pronounced clinical picture of myasthenia.

Treatment

If the diagnosis of myasthenia confirmed the presence of this heavy disease, its therapy should be started as early as possible. From the rate of commencement of the treatment of myasthenia will depend on its further course and degree of restriction of physical possibilities.

Eye form

In easy cases when the eye form of the disease takes place, the use of the following drugs is required:

• kalimin or prozerne;
• potassium chloride.

Important! Selection of dosage of these drugs is most often carried out in stationary conditions. This is due to the fact that heavy complications may develop during overdose. With their initial manifestations, the introduction of antidote is needed (in this case - atropine).

After selecting the necessary doses of calin or prozerne, as well as potassium chloride, the patient is discharged from the hospital. Further treatment and forecast will be a clear relationship. More precisely, the patient complies with the recommendations of a specialist, the higher the quality of his life and below the risk of major complications.

With myasthenia, the patient has to take a large amount of medicines.

Bulbarm

With bulbar form, the treatment of myasthenia has to supplement glucocorticosteroids. These drugs have their own side effects in the form of an increase in blood pressure and violations of glucose exchange, but often only they save a person from severe muscle weakness.

Most often use the tableted drug "prednisolone" or "methylprednisolone". Take them in the first half of the day. The dose of such drugs is selected in the calculation of 1 mg / 1 kg of human weight.

Note. The minimum dose of prednisolone capable of providing the therapeutic effect is considered 50 mg per day. As a result, patients have to use at least 10 tablets at the same time, which, naturally, often causes some difficulties.

Take glucocorticosteroids in such dosages follows for at least 1-2 months. In the future, there is a gradual decline in the dose of prednisolone. Fully cancel it will not succeed. For a normal state, patients have to take 10-20 mg of such a drug after a day. At the same time, it is necessary to control those negative effects that arise in the systematic reception of such drugs. The therapist sector will prescribe additional drugs to the patient for these purposes.

Tip! When developing side effects on the background of the reception of glucocorticosteroids, it should not be independently corrected their dose. Correctly make it can only doctor.

If the reception of glucocorticosteroids is contraindicated (for example, in high age), the patient recommends the use of cytostatic drugs. Typically, the initial means here is the Azatioprin medication. If its effectiveness is insufficient, stronger cytostatics are appointed.

In generalized the form of the disease, it is important to remove the animal gland in a timely manner.

Generalized form

The causes of the emergence and symptoms of the Miastion of generalized form require more serious medical events. For 1-2 years after the diagnosis is established, the patient conduct a surgical operation to remove the fork gland. Most often, the clinical effect from such manipulation is manifested after 1-12 months. After 1 year, doctors conduct a repeated full-fledged patient survey and clarify how much the benefit from surgical intervention was.

In the future, the same therapy is carried out by drugs that with eye and bulbar form.

With a sharp increase in muscle weakness, the patient is prescribed the drug "Immunoglobulin" and plasmferres.

What can not be done during myasthenia?

Currently known some contraindications for miastic. The main among them are the following:

1. Serious physical exertion.
2. Reception of drugs containing magnesium.
3. Long-term location under direct sunlight.
4. Reception of strip-like muscle relaxants.
5. The use of diuretic drugs (with the exception of the "Spironolactone" means).
6. Application of neuroleptics.
7. The use of tranquilizers (with the exception of the Grandaxin drugs).
8. Reception of most antibiotics from the group of fluoroquinolones and aminoglycosides.
9. The use of quinine drugs.
10. The use of corticosteroids that contain fluorine.
11. Reception of the drug "D-penicillamine".

In addition, the patient will have to comply with a certain diet. Proper nutrition during myasthenia practically eliminates products rich in magnesium (flabble, sea bass, shrimp, perch, cod, mackerel, dairy products, white beans, spinach, raw cereals, broccoli, blackberry, sesame, raspberries, dried, and others).

Miasthenia is not a contraindication to pregnancy

Miastations and pregnancy are not mutually exclusive concepts. Modern achievements of medicine allow a woman with this disease to carry a full-fledged baby, without hurting their own health. If there are no obstetric testimony, then such patients are conducted by a natural way. If they are presented, the cesarean section is carried out. In this case, anesthesia during myasthenia in a pregnant woman should be peridural. Such anesthesia will be safer. Common anesthesia in patients with miastenia try to apply only when removing the fork gland. Breastfeeding patients with miastenia is contraindicated.

Important! Neglecting these contraindications can lead to the aggravation of the course of the disease.

Miasthenia requires constant observation of specialists and a clear fulfillment of all their recommendations. Only such an approach will allow the patient to make his life as full as possible.

pozhelezam.ru.

Miasthenia - What is this disease?

Miasthenia is one of the autoimmune chronic diseases. It is characterized by a decrease in muscle tone and fast fatigue. Code of ICD 10 in this disease - G70, it is in one group with various impaired operations of neuromuscular fibers.

For the first time, such a state was described back in the 17th century, and recently the number of people who collided with it is growing. Miastic gravis, as completely called this disease, is more common in women, and develops at the age of 20-30 years, children are very rare. If the disease is not treated, it gradually progresses that with time it can lead to the death of the patient.

Causes of the disease

Miasthenia - the disease is poorly studied. It is believed that it has a genetic nature. The work of the gene responsible for the operation of neuromuscular fibers is disturbed. Normally, these compounds, which are called synapses, transmit pulses from nerves to the muscles, forcing them to shrink them. This process is regulated by various biochemical reactions involving the acetylcholine mediator and enzyme chinhestrase.

The pathogenesis of the disease is complicated: in some cases, the work of the fork gland and hypothalamus, which is responsible for the synthesis of these substances, is disturbed. If too little acetylcholine is produced or a lot of holiecerase, nerve impulses are blocked, and the muscle cannot perform their functions.

The reasons for the occurrence of such a violation are the failures in the work of the immune system, when the body produces antibodies to its own cells, destroying acetylcholine. This can happen after a cold or infectious disease, strong stress, hormonal failure or weakening of the body due to overwork.

Signs of the disease

The main symptom of the disease is an increased muscle fatigue. In the process of physical work, especially with recurring movements, muscle weakness gradually increases, which over time can lead to their paresa or paralysis. But after rest, these symptoms of myasthenia disappear, and in the mornings, patients feel quite good for several hours. At various stages and forms of the disease, such signs appear:

• shots in the eyes;
• ptosis - the omission of the upper century;
• salivation;
• voting change;
• violation of chewing functions, fast fatigue with solid food;
• food breakage;
• difficulty breathing;
• poor Mimica;
• change of gait;
• the weakness of the muscles of the limbs and neck;
• dry skin.

One of the first signs of Miasthenia is Ptoz - the omission of the Upper Century

Miasthenia forms

This disease develops in different ways. Most often the miasticity begins with the weakness of the eye and facial muscles, then this violation passes to the muscles of the neck and body. But some people have only some signs of the disease. In accordance with this, there are several types of myasthenia.

1. The eye shape is characterized by the damage to the cranial and brain nerves. The first sign of this is the omission of the upper eyelid, most often first on the one hand. The patient complains of bias in the eyes, difficulty in moving with eyeballs.
2. The bulbar form of myasthenia is the defeat of chewing and swallowing muscles. In addition to the violation of these functions, the patient changes speech, the voice becomes quiet, bent, there are difficulties in the pronunciation of some sounds, for example, "p" or "b".
3. But most often the generalized form of the disease occurs, in which the muscles of the eyes first are amazed, then the process applies to the neck, upper and lower limbs. Especially often amazed hips and muscles of hands, the patient is difficult to climb the stairs, hold items. The danger of such a form of the disease is that weakness applies to breathing muscles.

It is very important to put the correct diagnosis in time to start treatment

How the disease is determined

In addition to the complaints of the patient and collecting anamnesis, the diagnosis of Miasthenia includes various tests, a hardware examination and tests.

Electromyography helps to determine the muscle reaction to the load, and CT or MRI detect the absence of diseases that could cause similar symptoms. After all, miasthenic syndrome is observed with encephalitis, meningitis, cancerous diseases, botulism, thyrotoxicosis. But in this case, the Miasthenia is that the facial muscles are rarely affected, and during electromyography, it is not a slowdown, but an increase in muscle potential during re-stimulation.

It should also be distinguished by Miasthenia from muscle dosetrophy Duzhen, although they have different symptoms. Muscular dystrophy is found mainly in boys and begins in childhood.

Most often, a diagnosis is made by an immunological test to determine antibodies to acetylcholine and conduct a transversal sample. Its meaning is that after the introduction of intramuscularly 1 ml of the preparation "Prezero" The patient's condition in 30 minutes is significantly improved, and after 2-3 hours, the symptoms are returned.

A very important method of diagnosing myasthenia is various samples that allow to identify the degree of weakness of muscles, as well as which of them are more amazed. Since fatigue increases after repetitive movements, such tests may be effective:

• if you ask for a patient for at least 30 seconds to look aside or up, ptosis and shots in the eyes are manifested;
• to provoke dysarthria and a decrease in the power of voting, you need to ask the patient to read something out loud;
• detect the weakness of the neck muscles, if the patient is lying on his back and raises his head, he will not be able to keep it on weight more than a minute;
• sometimes during myasthenia, the phenomenon of M. Volker is manifested - repeated compressions and squeezing of the brushes causes strengthening PLOSE.

Electromiography helps determine the muscle reaction to the load

Crimi with disease

Miasthenia is chronic disease, it is constantly progressing. If the patient does not receive the correct treatment, its condition is worsening. A severe form of illness can be accompanied by the onset of my asthenic crisis. It is characterized by the fact that the patient is experiencing a sharp weakness of muscles responsible for swallowing and movement of the diaphragm. Because of this, his breathing is difficult, the heartbeat is readily, there is often salivament. Due to the paralysis of the respiratory muscles, the death of the patient may occur.

In case of overdose of anticholinesterase preparations, cholinergic crisis may occur. It is expressed in the slowdown in heartbeat, saliva, convulsions, strengthening intestinal peristals. This condition is also threatened with the patient's life, so he needs medical care. The anticholine-student drug must be canceled, and intramuscularly introduce its antite zone - the "Atropine" solution.

How to treat the disease

Miasthenia is a serious illness that requires constant medical observation and treatment. Often it leads to the disability of the patient. But the correct treatment of myasthenia helps to achieve long-term remission. The main direction of therapy is to apply drugs that block cholinesterase. Most effective in Miasthenia "Calmina", "Oksazil", "Prezeron", "Galanamin", "Ambenonius". They need to apply for a long time, alternating different means.

Potassium preparations enhance the effect of these drugs and improve muscle cuts, so they are also prescribed, for example, "potassium-norm" or "potassium chloride". Helps strengthen the effect of anticholinesterase agents is also a Diuretik "Veroshpiron".

Generalized myasthenia requires more serious treatment. When severely, hormonal means are used. Most often it is "prednisone" or a drug based on "Metipred". Usually prescribed 12-16 tablets per day in the morning every other day. If after a couple of months there is a persistent remission, the dose gradually reduce.

Recently, miasthenia also began to be treated with pulse therapy. This method is that the high doses of "Metipred" are introduced within 3-5 days. If remission managed to achieve, the drug gradually cancel. But sometimes you have to take it for a long time, often for several years, 60 mg in a day.

When myasthenia is very important to constantly accept the prescribed drugs

Cytostatic immunosuppressants are also used to treat such patients. "Azatioprin" is needed for blocking immunoglobulins and reduce the concentration of antibodies to acetylcholine. "Cyclophosphamide" and "Methotrexat" inhibit their activities. Such therapy is shown in patients who are contraindicated with glucocorticoids. But sometimes these medicines alternate.

As auxiliary therapy, it is possible to use products to improve metabolism in muscle tissue. These are calcium, "ephedrine", "methionine", glutamic acid, tocopherol acetate, vitamins of group V. Treatment with folk remedies does not apply, since many vegetable preparations are contraindicated to patients. Toning plants are admissible: ginseng, lemongrass and others on the recommendation of the doctor.

Miasthenic crisis necessarily requires the premises of the patient to the hospital into the branch of neurology. There it has plasmopheresis for cleansing blood from antibodies and artificial ventilation of the lungs. For a faster improvement in the condition, "prozerne", "ephedrine" and immunoglobulins are introduced.

In the absence of an effect from conservative therapy and the progressive course of the disease, surgical treatment is applied. Timectomy is the removal of the fork gland. In 70% of patients after the operation, an improvement in the state is observed. But the complexity is that anesthesia in this disease has features.

Many drugs are contraindicated with such patients, especially muscle relaxants and tranquilizers, as well as morphine and benzodeazepines. Therefore, the operation is carried out with a serious condition of the patient and only in patients under 70 years old.

Sick miastenia is very important attentive to the preparations taken, since many of them are contraindicated

Miasthenia Conduct Rules

If the diagnosis is delivered on time, and the patient performs all prescriptions of the doctor, its performance and lifestyle almost do not change. The treatment of myasthenia is the constant reception of special drugs and complying with some rules.

It is forbidden to sunbathe with such patients, perform heavy physical work and drink drugs without the recommendation of the doctor. Patients must know which contraindications for miasthenia are for drug intake. Many drugs may cause a complication of the course of the disease or the development of a miasthenic crisis. These are medicines:

• all drugs of magnesium and lithium;
• miorlaxants, especially stripped;
• tranquilizers, neuroleptics, barbiturates and benzodiazepines;
• many antibiotics, for example, "neomycin", "gentamicin", "Norfloxacin", "Penicillin", "Tetracycline" and others;
• all diuretic drugs, except for Veroshpiron;
• Lidokain, "Cinen", oral contraceptives, antacids, some hormones.

Is it possible to cure myasthenia

Previously, this disease was considered incurable, 30% of cases ended with a fatal outcome. Now the Miasthenia forecast is more favorable. With the right approach to therapy, more than 80% of patients experience persistent remission. They can continue normal life and even retain performance. Persistent disability or even the death of the patient may occur with a late appeal to the doctor or with non-compliance with the prescribed treatment.

About such a disease, like miastic, many did not even hear. Therefore, it is not always on time to seek medical care. It is dangerous, as it can cause complications. But the correct treatment and compliance with all the recommendations of the doctor will help the patient to lead a normal lifestyle.

moyaspina.ru.

Lactofilm when taking antibiotics

Miasthenia is a severe autoimmune disease, which is manifested by the pathological weakness of muscles and slowly progresses. Children suffer from him more often, but this pathology is also found in adults.

A little about the reasons

Miasthenia is a congenital hereditary disease. His signs appear in early childhood. Syndrome can develop at different speeds and severity. Due to genetic deviations, neurons and muscle fibers are disturbed. Due to the fact that the muscles are actually disconnected, they do not function, their atrophy is gradually developing.

Scientists have not yet been able to completely identify the mechanism for the occurrence of the disease, however, it is known that the reason lies in a shortage of a gene, which is responsible for the work of myioneral ties. First of all, the visual functions suffer, since the muscles of the eyes are atrophy. Then the process goes to the facial muscles, neck, muscles of the hands, legs, a swallowing muscles.

Often, this congenital syndrome leads to severe consequences and even the death of the patient, but with proper treatment it is possible to recovery or temporary remission. Inherited this pathology can be from one of the parents or through generation.

The causes of the disease among children are distinguished:

1. Failures of biochemical processes due to Timus pathologies, hypothalamus.
2. The thymus attack its own immune cells, which is why the acetylcholine is less generated and split.

Please note that the patient's condition may worsen stressful situations, ARVI, imminent impairment.

Symptoms

Miasthenia symptoms are directly dependent on its shape. The main symptom is unusual weakness in muscles. The patient is quickly tired, it is not able to cope with work, learning. This is especially noticeable if you need to make a number of one-type movements.

After rest, muscles are restored. Waking up in the morning, patients feel cheerful, rested, feel the tide of the strength. After some time, characteristic symptoms begin to increase, the patient feels literally broken.

Miasthenia Gravis

Miastic gravis can manifest itself in different ways, it all depends on the form. They allocate three:

1. bulbar
2. eye;
3. generalized.

With a bulbar form, only one local muscle group suffers. They provide chewing, swallowing, because the patient begins to change the voice. He becomes hoarse, quiet and even almost silent.

With the eye form of myasthenia, the muscles suffer, which ensure the movement of eyeballs. This is the muscles that raise the eyelid, outdoor circular. A patient suffering from myasthenia is easy to learn from lowered centuries - he cannot raise them due to the defeat of the muscles.

If the myasthenia is generalized, gradually affects the glazation, mimic, cervical muscles. Patients on the face appear deep wrinkles, and the smile becomes unnatural, stretched. Over time, the person becomes difficult to even keep his head. This is a consequence of the weakening of the muscles of the neck.

When the disease progresses, the muscles of the hands are involved in the pathological process. Such patients practically lose the ability to walk, move, as musculature does not experience a normal load, over time it is atrophy. It is the generalized form that meets most often.

Miastations may be accompanied by characteristic crises. This is the hardest form of the disease. During the crime, pharynses and respiratory muscles are completely disconnected. This is a direct threat to life, because the movements of the chest are completely stopped, in view of which an oxygen starvation of the body occurs.

Diagnostics

It is very important to spend a thorough examination to understand the extent to which the disease is progressing, which is why it develops. For the correct selection of the treatment scheme, you must pass all the diagnostic steps. It includes:

1. Electromiography. It will help identify a miasthenic reaction.
2. Prezero sample. The patient is introduced into the muscle antagonists of cholinesterase.
3. Serology study. Its goal is to identify the antibody antibodies for acetylcholine.
4. CT. Helps to identify possible tumors (for example, Timoma).

It is the transversive test that is the main diagnostic method that is able to confirm the Miasthenia finally.

Treatment

Miastic gravis is a serious and threatening life of pathology. When setting such a diagnosis, it is necessary to immediately begin treatment of myasthenia. Ophthalmic treatment is often required, since the disease can provoke disorders of the features of the eyes. It will also be necessary to eat correctly.

The mechanism of therapy is based on the fact that new manifestations of myasthenia are constantly taken into account and the dose of drugs is corrected. It should not exceed the one that provides a steady therapeutic effect. Therapies are easier to sick children and young people, the elderly remission comes less frequently.

It is important for parents to remember that even the usual cold can cause miastic, because any infectious disease should be treated. Such well-known infectiousists are insist on this, such as Academician Yuri Vladimirovich Lobsin. It is important to choose a good clinic, which will provide all modern methods for the treatment of this difficult disease.

Proper treatment can suspend the development of the disease, and in some cases it is possible to achieve complete recovery. Therapy should fully comply with modern standards, as recent years in medicine, methods for treating Miasthenia have been significantly improved.

Must be taken into account symptoms of a particular patient. The disease can have quite different forms and severity. It all depends on the cause of its development. This may be not only disturbed genetic code, but also infectious damage, head injury, snake bite, etc.

The treatment will be based on the maintenance of the desired level of anticholinesterase substances in the blood. These funds are constantly introduced into the body. It is sometimes difficult to immediately determine a safe dose for a particular patient, because the introduction of drugs begin with extremely small doses. Such patients need constant care and regular courses of treatment.

Overdose of these drugs is fraught with serious side effects and unpleasant phenomena from the side of the liver, kidneys. It can even provoke a cholinergic crisis, which is manifested in the form of convulsion, Miosis, bradycardia. They are accompanied by pain in the abdomen. If such crisis occurs, the patient is introduced immediately the desired dose of atropine.

The essence of treatment is that the acetylcholineserase antagonist is selected. This selection is carried out strictly individually. It is important to take into account the age of the patient, the weight, shape and severity of the disease. Also prescribe oxazil, prozerne, galantamine or calmina.

If pseudo-paralytic myasthenia is installed, the patient additionally introduces spirironolactone, potassium salt. They maintain the condition of the body. If the patient suffers from the severe form of the disease, glucocorticosteroids, cytostatics are definitely prescribed. In the case of the identification of the timomy, the only path of treatment is a surgical removal of the tumor.

Prezer, IVL, plasmapheresis, drugs based on human immunoglobulin are used to relieve a miasthenic crisis. If this is a gravis disease (severe hereditary form), therapy will differ from treatment with other forms of the disease.

Most often a pyridostigmine bromide is prescribed. The drug causes a number of side manifestations: diarrhea, abdominal pains, muscle beatsiculation. Increased dose of the drug is able to cause cholinergic crisis.

Immunomodulatory treatment

One of the directions of therapy is to modulate immunity. For this purpose, glucocorticoids are prescribed. They are effective, relatively safe and inexpensive. This is the secret of their global popularity. Scientists have not yet fully found out how these drugs work, but we cannot indisputate the fact that they can significantly alleviate the patient's condition and lead to long-term remission.

This group of medicines have a number of side effects, but they are directly dependent on the dose. Therefore, the doctor must prescribe a minimum effective dosage for a particular patient. The most popular drug of this group is prednisone.

It is prescribed with a minimum daily dose (10-25 mg) and then slowly increase the dosage. Ideally, the daily dose should be 60-80 mg (one-time reception). It can be replaced with methylprednisolone.

If the patient suffers from a severe form of the disease, the treatment is prescribed immediately with a high dose of corticosteroids. The drug is introduced every day. In parallel, plasmapheresis or immunoglobulin is introduced intravenously. The purpose of such enhanced therapy is to stabilize the patient's condition. It will take it from 4 to 16 weeks. After improving the state, the doses of corticosteroids gradually reduce. They are brought to the level of supporting therapy.

Azatioprine is an analogue of purine, which slows down the synthesis of nucleic acids. It affects lymphocytes. When using the drug, it is necessary to control the function of the liver, blood condition. At first, blood test takes every day. If the drug is transferred to the patient well, then after 1-2 weeks the dosage rises. The maximum dose is 2-3 mg per kg of mass (the average daily dose is 150-200 mg).

This remedy is pretty well tolerated, although it can sometimes be nauseous, lymphoprod, rash on the skin, pancreatitis, pancytopenia.

Please note that the therapeutic effect may not occur immediately. It is often manifested in 4-12 months after the start of treatment. The maximum effect is usually observed in six months a year.

Azatioprine is used as an addition to prednisone. It is prescribed by the patients who take long-term immunosuppressive therapy. Due to such a combination, the dose of corticosteroids can not be raised, without losing their effectiveness. This is the so-called sparring effect, when one drug enhances the therapeutic effect of the other.

Cyclosporin is another drug that is prescribed during myasthenia. He has a complex effect, which ultimately leads to a slowdown in the activation of T cells. The drug can cause tremor, insomnia, renal failure, increased pressure, headache. These side effects depend on the dose applied. If it is reduced, unpleasant manifestations can leave or minimize.

Cyclosporine is prescribed quite rarely. He has much more pronounced side effects than other drugs, therefore, this tool is used if the rest have shown low efficiency. If the medication is appointed, it is important to control the level of electrolytes in the blood, magnesium, the work of the kidneys. Start with small doses, gradually bringing the daily dosage to therapeutically effective.

If cyclosporine is appointed, it is impossible to take diuretics (potassium-saving) and NSAIDs, and when you need to take corticosteroids, it should be reduced to maximize their dose. Fully cancel prednisone will not work.

Mikophenolate Mifetila is a modern drug. Scientists have not yet fully studied, as it works, but the results cause optimism. The substance slows down the replication of V-, T-cells. When using the drug, it takes a blood test every month. Scientists agree that Micophenolate Mofetila is also effective as cyclosporp, but it has fewer side effects.

Cyclophosphamide is an effective immunosuppressant, which is prescribed during the severe form of the disease, inhibiting T- and B cells. It is rarely prescribed, only when other drugs have not shown their effectiveness. Already after a couple of months, a steady remission is observed in 50% of heavy patients. If there were noticeable side effects, this agent will have to be canceled.

Methotrexate slows down cell division, but can provoke nausea, cystitis, mucosites, alopecia, myelosuppression. Doctors consider it as a reserve drug if funds from the first row are ineffective.

Rituximab is an antibody that is characterized by elevated relationship with CD20 cell antigen. It can cause fever, rash on the skin, nausea, sometimes - bronchospasm. Between its reception you can make a sufficiently break - until six months.

Short-term therapy

Along with the drugs, short-term treatment is prescribed: plasmapheresis, immunoglobulin is intravenously introduced.

The mechanism of exposure to the immunoglobulin is that it neutralizes the activated compliment, autoanthele, modulates cytokines, etc. It can cause fever, headache, rash on the skin.

The purpose of plasmapheresis is removed from the blood of autoantibodies and other components produced by the immune system. 4-5 plasmapheresis sessions are performed. It is most often prescribed in the process of preparing cooperation, serious condition, when symptoms are rapidly growing. These two methods have about the same efficacy.

Surgical treatment

Surgical method - Timectomy. It is most often used to eliminate myasthenia. It is carried out if the exact diagnosis of Timoma is supplied, as well as, if patients with a generalized type of disease did not reach 60 years.

Pay attention, not always with generalized form, timetomy is shown.

During pregnancy, drugs are selected with special care.

Pathogenesis

Recent years there are many new information about the pathogenesis and clinical manifestation of myasthenia. Scientists have progressed significantly in the study of its development mechanism, although there are still quite a few gaps.

Now it is possible not only to achieve a resistant remission, but also to predict the solid development of the disease in a particular patient. For therapy, it is important to take into account the nature of the disease of the disease in a separate patient, the presence of therapeutic and side effects, their ratio.

It is important that the doctor understand the essence of this problem, the main stages of the development of the disease, thoroughly knew the entire arsenal of modern means of therapy, owned algorithms of treatment. All this will allow you to have a patient as effective help.

If myasthenia is revealed, the forecast depends on the form of the disease, as well as timeliness and systematic treatment. It is worse than the treatment of a generalized Miasthenia form.

The disease is periodic rhythmic. Remissance is replaced by a period of exacerbation. It is important to carry out regular stimulation of muscle work, so as not to bring to their atrophy. This is promoted by therapeutic physical culture and gymnastics.

So, for the successful treatment of myasthenia, it is important to carry out complete diagnosis and identify the cause of pathology. Then the complex of drugs that have a minimum of contraindications and side effects is selected. This disease is extremely dangerous, because it should be engaged only by physicians. Folk remedies are categorically contraindicated.

Description:

Miasthenia is an autoimmune disease that causes a violation of neuromuscular transmission and manifested by weakness and pathological fatigue of skeletal (transverse) muscles. In the process can be involved any muscle of the body, but there is a tendency to preferred the muscles of the face, lips, eyes, language, pharynx and neck. & NBSP & NBSP Neonatal Miasthenia is a transient state observed in infants born from mothers suffering from miastenia, and caused by the transition through the placenta of maternal antibodies to acetylcholine receptors.

Symptoms:

Myasthenia manifested by fatigue of muscles with concomitant weakness, & nbsp & nbsp especially & nbsp & nbsp eye & nbsp & nbsp and & nbsp & nbsp muscle, & nbsp & nbsp innervated & nbsp & nbsp bulbar & nbsp & nbsp nerves. & Nbsp & nbsp weakness of ocular muscles leads to and strabismus, mono- or bilateral ptosis, the most pronounced by the end of the day. Often there is a weakness of facial and chewing muscles. Difficulties of speech and swallowing can be detected after more or less long conversation and meals. Weakness and fatigue of the muscles of the language and the nasal shade of the voice are possible. Another transverse muscle of limbs and neck can be amazed, which leads to generalized weakness. The depletion of tendon reflexes is determined. With repeated electrical stimulation, the pathological fatigue of the muscles is revealed, a pronounced ability to restore after a short rest. The lability is characterized, the dynamism of symptoms with their amplification when reading, fixing the view, sometimes general physical exertion. Miastic can be generalized and local (eye muscles defeat, throat, larynx, mimic musculature or musculature of the body). The generalized form may be accompanied by respiratory disorders.
diseases has a progressive nature. Miasthenic episodes (short-time Miasthenic disorders and long-term spontaneous remissions are possible) and miasthenic states (stable manifestations throughout a significant period). In patients with myasthenia, there may be a sharp deterioration in the condition in the form of a crisis with muscular weakness generated by muscle weakness, eye-minded and bulbar and symptoms (aphoonia,), respiratory impairment, psychomotor excitation imposing lethargy, as well as vegetative disorders. This develops acute hypoxia brain with disorder of consciousness. Fully outcome is possible.

Causes of occurrence:

The disease is associated with the formation of antibodies against acetylcholine receptors located on a postsynaptic neuromuscular synapse membrane. Perennial studies have proven a connection of miastic with a forkneck. Inheritance factors playing the last role.

Treatment:

For treatment prescribe:

It is aimed at the correction of the relative deficiency of acetylcholine and suppressing an autoimmune process. In order to compensate for neuromuscular transmission disorders, anticholinesterase means are used: prozerne, oxazil, pyridostigmine bromide (Maltinon, Calimine, Amiridine). The choice of an optimal individually compensating dose is important depending on the clinical form, the severity of symptoms, concomitant diseases, reaction to the drug. With pharmaceutical and eye formats, the pyridostigmine bromide, prozerne and oxazil, are more effective. Doses of preparations and intake intervals are individual. Prescribed chloride or orotat potassium, Veroshpirius, Ephedrine. In severe cases, prozerne parenterally is introduced. The reception of large doses of anticholinesterase drugs can lead to a cholinergic critic. The main methods of treatment of this crisis are the abolition of anticholinergic agents and the re-administration of atropine.
In a miasthenic crisis resulting from an insufficient dose of anticholinesterase drugs, prozerne intravenously administered urgently. Oxazil can be entered in candles. The ephedrine solution is also used, potassium preparations intravenously. Progressive and threatening life The weakness of breathing muscles may be observed, despite the introduction of large quantities of prozer. Patients produce intubation or tracheostomy, translated on IVL. Patients are powered through a nasogastric probe. It is necessary to maintain the balance of fluids and electrolytes, vitamins; carry out the correction of disorders of the acid-alkaline balance.
The main methods of pathogenetic treatment of patients with myasthenia are timectomy, radiotherapy and hormonal therapy. The surgical method (thymectomy) is shown to all patients under the age of 60, suffering from miastenia, but in satisfactory condition. It is absolutely shown at a tumor of the fork gland. X-ray therapy on the region of this gland is prescribed after incomplete thymectomy, with the eye form of myasthenia, as well as in the presence of contraindications to surgery in senior patients with a generalized form of myasthenia. In severe cases, with generalized myasthenia, immunosuppressive treatment is shown. Corticosteroids are prescribed, the duration of receiving the maximum dose of corticosteroids is limited by the onset of significant improvement, which is subsequently reduced the dose to supporting.

Mixing changes depend on antibodies to acetylcholine receptors. They reduce the amount of acetylcholinesterase on synapse.

What is Miasthenia

How neuromuscular pathology occurs:

• The articulations between the muscles and nerves are called synapses. The nerve endings at the end of the synaps are equipped with bubbles that contain acetylcholine mediator.
• On the other hand, the muscles are receptors to the mediator.
• When the muscle team is given to shrink, the momentum is passed through the nerve. Acetylcholine is released, the pulse is connected to the desired receptor.
• Miastic gravis occurs when the transmission of the nerve impulse is blocked. Inheritance is not transmitted, enhanced after exercise.
• In case of disease, the number of nicotine receptors of acetylcholine in the end plates of muscles is only 30%.

Miasthenia forms

In neurology, the disease gave their own code on the ICD 10. Miastic forms are divided into two groups: local and generalized. The last type is expressed by the selectivity of the detection site during pathogenesis: Some muscles suffer more often than others. For instance:

1. Among the eye muscles is common pathology that raises the upper eyelid.
2. In the hand, the three-headed muscles of shoulder suffer significantly more than others.
3. A separate form is allocated for children's miastic, the signs of which are manifested at birth.

Miasthenia - Eye Form

Asthenic ophthalmoplegia is associated with such ailment as a Lagofalm, Ptosis, disturbed eye movement, diplopia. These symptoms cause an eye-eye miastic, at which there is a damage to muscle fibers, raising the upper eyelid, the circular muscles of the eyelids and the glasses. Signs of the weakness of the eye muscles are the pathological fatigue, capable of strengthening at:

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• physical exertion;
• splash of emotions, disorder;
• stress;
• after the course of anticholinesterase preparations.

Miasthenia - generalized form

A frequent form of myasthenia is generalized, in which all muscles suffer. First, the work of the eye muscles is disturbed, then muscle fibers of the neck and facial expressions. Generalized myasthenia prevents the patient to keep his head, he does it with great difficulty. A transverse smile is displayed on the face, deep wrinkles arise on the forehead. Increases the salivation and fatigue of the muscles of the hands and legs. The patient cannot trace himself, goes badly.

Bulbar Miasti

Violation of chewing processes, swallowing, changing speech, depletion of votes - signs that are characterized by a bulbar form of miasthenia (asthenic bulbarium paralysis, pseudobulberry syndrome). For the disease, the dysarthria (violation of speech) is characterized, dysphony (bent, hoarseness) and dysphagia (violation of the act of swallowing), i.e. the symptoms associated with the muscles of a soft sky and larynx, the Mimic muscles, and glazing. External manifestations: face youth, smile "growers", lethargy of mimici.

Miacency Lambert Syndrome-ITON

This phenomenon refers to the category of rare molds. Lambert-ITON syndrome is a disease in which the process of neuromuscular transmission is disturbed. The condition is characterized by rapid fatigue muscles, attacks of weakness, ophthalmoplegia (paralysis of the eye muscles), Malgia, vegetative disorders. Miasthenic syndrome may occur with smallllular lung cancer, malignant formations, autoimmune diseases. Patients with such syndrome are difficult to get out of the position lying or sitting.

Causes of Miasthenia

Miastations may be congenital or acquired disease. Congenital occurs as a consequence of the change of the COLQ gene (mutation in the gens of proteins responsible for the contraction of the muscles). In an eraset, women are sick of this disease more often than men. With an autoimmune process directed against acetylcholine receptors, human immunity produces antibodies in muscle tissue. The causes of myasthenia acquired are described below:

• tumor of the fork gland (timoma, thymus);
• various autoimmune processes;
• severe stress situations;
• viral diseases.

Symptoms Miasthenia

Details learn about the disease, to understand, Miasthenia - what it is, to recognize the cause will help consult a neurologist who will reliably determine the signs of myasthenia. Miasten-like symptoms are two types: people complain about the weakness of different muscle groups and the impaired function of the nervous system. The following problems are signs of the disease:

• with repeated movements - fatigue;
• diplopia associated with the weakness of the eye muscles;
• ptoz centosis;
• reduced clarity of vision;
• hoping process;
• dysarthria, fuzziness in pronunciation;
• breathing is disturbed;
• increasing heartbeat;
• problems with chairs, diarrhea, constipation.

Miasthenia Diagnostics

Before diagnosis, other diseases are excluded that may accompany pathology. The most reliable method of diagnosis is a transversal test. How does this happen? Patient subcutaneously introduced the drug Prezero. It will briefly improve the function of neuromuscular transmission. Miasthenia Diagnosis includes other methods for detecting the disease:

• blood test for antibodies;
• poll survey, clinical examination;
• decrement test (needle electromyography);
• computed tomography of the fork gland.

Decrement test

One of the options for studying the neuromuscular transmission process is a dekerent-test for miastic, at which there are surveys of the five muscle groups. Each of them is subject to the process of rhythmic stimulation, i.e., muscle fibers make abbreviations caused artificially. With this process, the indicators of muscle responses are recorded during stimulation. The term "decrement" is called a decrease in the frequency of muscle responses in response to stimulation, which is characteristic of myasthenia. The presence of a decrement indicates neuromuscular pathology.

Prezero test

A drug dramatically improves the work of neuromuscular synapses is prozer. When the question of the presence of pathology is raised, the test of the test is used for the test during myasthenia. The test is the following steps:

1. A decrement test is held.
2. It is introduced subcutaneously or intramuscularly prozer with a syringe.
3. Against the background of the action of the prozero, a decrement test is re-conducted.
4. If there is a significant improvement in the results of the decrement test, it means that the transmission is substantiated.

Miasthenia - Treatment and Forecast

Progressive weakness of transverse muscles must be treated with drugs. In some cases, nothing will help in the surgical operation. Miasthenia treatment with adequate therapy is able to translate the aggravation of the disease in remission. In Nervo-muscular pathology, as a whole, a favorable prediction that may worsen with improper treatment of drugs. However, this does not concern my asthenic crises for which acute respiratory failure is characteristic. The forecast in order is not consolation.

Medicines for Miasthenia

Medicinal products, doses, the duration of therapy must select the attending physician, since, with different forms of disease and severity, the state is prescribed its purpose. During the exacerbation of the disease, the procedure of plasmapheresis is prescribed (500 ml a day with a substitution of albumin and plasma) and introduce intravenously immunoglobulin. Common drugs during myasthenia include the relief of the impulse transmission defect from the nerves to the muscles:

• anticholinesterase preparations (Calminim);
• salts of potassium;
• glucocorticoid therapy (prednisone);
• cytostatic therapy (azathioprine, cyclosporine);
• mikophenolate Mofetil (Cellsept).

Timetomy at Miasthenia

A long time was discovered in the connection of muscle pathology with a violation of the fork gland. Now timectomy (operation to remove the fork gland) is considered to be a standard operation. In 75% of cases, after surgery, patients appear improvement. For complete removal of the fork gland use transstinal surgical access (with sternum dissection). Less often use transcervical access (catheter).

Miasthenia - Treatment by folk remedies

Official medicine categorically prohibits treating diseases by folk drugs. To cure pathology, they are not under power, but they can alleviate the course of the disease. It is more intelligent to use folk medicines in a complex with drugs that the doctor appointed. It is necessary to know whether it is possible to use the tool with your illness history. The treatment of myasthenia by folk remedies is made using such products like oats, onions with garlic, dried fruits.

Diet at Miasthenia

Patients with the disease must be established in accordance with the phase of the disease. The protective functions of the body are weakened, so the power in Miasthenia plays an important role in recovery. It is important to use baked potatoes, raisins, bananas and kuragu. It will not hurt to find sources of trace elements such as phosphorus, calcium. Calcium reception must be combined with phosphorus, this is better assimilation of substances. It is important to use potassium and vitamins.

Miastations in children

It is difficult to treat Miasthenia in children, since they cannot reliably describe their symptoms. It all begins with disorders of glazing, chewing, mimic muscles. There is a pathological fatigue in muscle fibers pelvis, hands, neck. What does the face of a child with a disease look like? There is no emotion, lifeless looks, the gaze remains still, the upper eyelid eyes are omitted. Muscle fatigue and weakness are stronger than in the evening.

The actions forbidden in pathology include excessive hobby of sports, severe physical exertion, long stay under direct sunlight (insolation). In addition to limiting motor functions, there are drugs contraindicated during myasthenia:

• magnesium (magnesia, asparks);
• D-penicillamine;
• neuroleptics;
• stripped muscle relaxants;
• diuretic drugs, except Veroshpiron;
• fluorine-containing corticosteroids;
• derivatives of quinine;
• antibiotics.

Video: Miasthenia disease

The information presented in the article is familiarized. Article materials do not call for independent treatment. Only a qualified doctor may diagnose and give recommendations on treatment based on the individual characteristics of a particular patient.

Miasthenia

1. Premipatic terminal

emerg / 325 Emerg / 325 (Emergency), MED / 3260 MED / 3260 (Pregnancy), OPH / 263 OPH / 263 (Eye)

Miasti (Lat. myasthenia gravis.; Dr. Greek. ῦςῦς - "Muscle" and ἀσθένεια - "powerlessness, weakness") - an autoimmune neuromuscular disease, characterized by pathologically rapid fatigue of transverse muscles.

Miasthenia (asthenic bulbarium paralysis, asthenic ophthalmoplegia, false bulbar paralysis, erba disease - Goldflam) is a classic autoimmune human disease. The main clinical manifestation of Miasthenia is the syndrome of pathological muscular fatigue (strengthening manifestations of miastic after physical activity and reduce them after rest).

History

For the first time the disease was described by Thomas Willis in 1672. Antibodies during myasthenia were first highlighted by Strauss in 1960. The finally autoimmune origin of the disease was proven by Patrick and Lindstrom in experience on rabbits immunized by Torpedo Californica ACH, who have signs of Miasthenia.

Epidemiology

The disease usually begins at the age of 20-40; Women suffer more often. Recently, the incidence of miastenia is growing, at the moment the prevalence is approximately 5-10 people of the nation.

Etiology

Miasthenia happens both congenital and acquired. The cause of congenital myasthenia is mutations in the genes of various proteins responsible for the construction and operation of neuromuscular synapses. In synapses (in particular, in the terminal plates of neuromuscular synapses) acetylcholinesterase is present in the form of the isoform tetramer attached to the collagen-like protein, which is encoded by a separate COLQ genome. The mutation of this gene is one of the most common causes of hereditary Miasthenia (Myasthenia Gravis). Another common cause of myasthenia is various mutations of acetylcholine nicotine receptor subunits.

Sometimes, more often in young, there is a tumor of the fork gland, which is removed by surgical way.

Pathogenesis

In the mechanism of development of myasthenia, autoimmune processes play the role, antibodies in muscle tissue and the fork gland are detected. The muscles of the eyelids are often amazed, ptosis appears, which varies in terms of severity during the day; Chewing muscles are affected, swallowing is disturbed, the gait changes. Patients are harmful to nervous, as it causes chest pain and shortness of breath.

The provoking factor may be stress transferred by ARVI, as well as a violation of the function of the body's immune system, which leads to the formation of antibodies against its own cell cells - acetylcholine receptors of the postsynaptic membrane of neuromuscular compounds (synapses). By inheritance, autoimmune myasthenia is not transmitted.

Most often, the disease is manifested during transitional age in girls (11-13 years old), less often occurs in boys at the same age. The disease in children of preschool age (5-7 years) is increasingly revealed.

Forecast

Previously, myasthenia was a heavy disease with high mortality - 30-40%. However, with modern methods of diagnosis and treatment, mortality has become minimal - less than 1%, about 80% against the background of proper treatment reaches full recovery or remission. The disease is chronic, requires careful observation and treatment.

Clinical picture

There are several forms of myasthenia (eye, generalized, bulbar and miasthenic Lambert - ITON syndrome (with lung cancer, etc.). The disease begins with eye symptoms (annoyance of the eyelid). Feature is the dynamism of symptoms: in the morning Ptosis may be less than In the evening, the cooler changes in severity. Then the weakness of the proximal departments of the muscles of the limbs are more often joined (it is difficult to climb the stairs, climb from the chair, raising your hands up). At the same time, on the background of physical load, weakly increases in all muscle groups (after samples with 10 squats weakness Increases not only in the muscles of the legs, but also hands, ptosis increases). Bulbarny violations can be joined (on the background of a long conversation or during reception, the voice acquires a bellged shade, dysarthria appears, it is difficult to pronounce "P", "sh", "C" . After rest, these phenomena pass). Next, bulbar disorders can become more pronounced (a violation of swallowing, pop Rooting, entering liquid food into the nose).

Acceptance of anticholinesterase preparations (Calimin, Prezero) significantly improves the condition of the patients - such patients are trying to eat at the peak of the action of anticholinesese drugs.

Diagnostics

In standard cases, the diagnosis of myasthenia includes:

1. Clinical inspection and clarification of the history of the disease.
2. Functional trial to identify pathological muscle fatigue syndrome. Electromiographic study: Demkent test
3. Prezero test
4. Repeated decrement test to identify reaction to prozer
5. Clinical inspection to identify reversibility of miasthenic changes against the background of prozer
6. Blood analysis on antibodies to acetylcholine receptors and antibodies to titin
7. Computed tomography of the anterior mediastinum (forks of the gland, synonym: thymus).

In cases of complex differential diagnostics, needle electromyography is carried out, a study of the conductive nerve function, the electromyography of individual muscle fibers (jitter), biochemical studies (creatinephosphocinate, lactate, pyruvate, 3-hydroxybutyrate).

Treatment

In cases, only Caline and Potassium preparations are applied to the first time identified miastic and eye shape in the treatment.

Kalimin 60n 1 tab 3 times a day with an interval of at least 6 hours. Potassium chloride 1 g 3 times a day or potassium-normon 1 tab 3 times a day.

In cases of pronounced muscle weakness or presence of bulbar disorders, glucocorticoid therapy is used: prednisone in a dose of 1 mg / kg weight strictly every other day in the morning hours (conventional doses are 60-80 mg per day, the minimum effective doses are 50 mg per day in a day).

One tablet prednisone contains 5 mg, respectively, the daily dose of prednisolone is 12-16 tablets. Metipred tablet contains 4 mg, but in efficiency it is equal to 1 tab of prednisolone 5 mg, therefore, in terms of methipred, the number of tablets is the same 12-16 tab, and the total dose will be less.

Prednisolone 60 mg in the morning every other day.

The reception of prednisolone is long, remission may occur after 1-2 months, then the prednisone dose of 0.5 tab decreases to a supporting dose of 10-40 mg every other day. And then slowly with caution of 0.25 tablets until the preparation is completely eliminated.

The reception of prednisolone requires control of blood sugar and control by a precinct therapist (blood pressure, prevention of steroid ulcers, osteoporosis).

In the first 1-2 years, an operational intervention is carried out on the removal of the fork gland (thymectomy) in the first 1-2 years of the Miasthenia. The effect of thymectomy is developing in the interval of 1-12 months from the moment of thymectomy, the timectomy efficiency assessment is made after 1 year.

In old age, with insufficient effectiveness of therapy with prednisolone, with the impossibility of prescription prednisolone and cytostatic therapy is prescribed during the abolition of prednisone. In light cases - Azatioprin at 50 mg (1 tab) 3 times a day. In more serious cases - Cyclosporine (Sandimmun) of 200-300 mg per day or Cellsept 1000-2000 mg per day.

With the exacerbation of Miastius, plasmapheresis and the introduction of intravenous immunoglobulin is permissible and justified. Plasmapheresis is advisable to carry out 500 ml every other day N5-7 with a replacement by plasma or albumin.

Immunoglobulin is introduced intravenously at a dose of 5-10 g per day to a total dose of 10-30 g, an average of 20 g. Immunoglobulin is introduced slowly, 15 drops per minute.

Contraindications for Miasthenia

Karen Van Rensburg, BPharm

Miasthenia is a neuromuscular disease characterized by pathological rapid fatigue of cross-striated muscles. A few years ago, this disease had a very unfavorable forecast. Currently, there are various ways of treating myasthenia, which significantly improves the quality of life of patients suffering from this disease.

Clinic

Miasthenia - neuromuscular transmission disorder. It is classified as an autoimmune disease, since the body begins to produce antibodies that prevent the nerve endings in the muscle to which the acetylcholine mediator acts. These antibodies can block, modify and destroy Acetylcholine receptors without giving muscles to shrink.

Major clinical manifestations of Miasthenia are a consequence of weakness (frequently non-permanent) of various groups of cross-striped muscles: oculomotive (elevation of the eyelid, squint, "double vision", restriction of the mobility of the eyeballs), chewing and mimic muscles (incomplete eye closure, the impossibility of cheeking cheeks, cushed Teeth, violation of chewing, etc.), bulbar muscles (difficulty swallowing, bezavidity, food intake in the nose, voices, voices, articulation disorders), skeletal muscles (muscles of legs, hands, neck, torso). With the weakness of intercostal muscles, the diaphragms, the muscles of the larynx, accompanied by the accumulation of sputum and saliva in respiratory tract, respiratory disorders arise. The most common lesions of the o'clock muscles (in 40-50% of patients with a first clinical inspection and up to 95% as the disease caused), bulbar (about 40%), muscles of limbs and torso (20-30%) patients. The degree of severity of symptoms in patients with myasthenia is very variable.

The extreme degree of myasthenic disorders is crisis (in 10-15% of patients) - the rapid exacerbation of motor disorders, combined with a respiratory impairment and swallowing. If at this time you do not have a patient emergency assistance, there is a real threat to death.

By the nature of the flow, there are a fixed form of miasthenia, as a rule, the symptoms of the disease, and the progressive (in the majority of patients) with the release of the following types: the rapidly progressive (developing for 6 months), the rapidly progressive (for 6 weeks) and Malignant, characterized by acute start and rapid development with the addition of bulbar and respiratory disorders within 2-3 weeks. The form of myasthenia can be local (usually eye) and generalized, with different degrees of severity of the defeat of certain muscle groups. In addition, given the degree of violation of the muscle function, it is possible to distinguish (very conventionally) lightweight, medium and heavy forms of miastic.

Diagnosis

Muscle weakness and a number of other symptoms observed during myasthenia may occur at various other neurological and endocrine diseases. In this regard, a qualified neurological examination with the definition of weakness and pathological fatigue of various muscle groups plays a major role in the diagnosis of miastic.

In order to eliminate the tumor of the fork gland (timomes), as well as to determine changes in the fork gland (hyperplasia, cyst, fat involution), a number of special research methods are applied. In 10-12% of the examined patients, Timoma observes, and in 60-70% - hyperplasia, in the rest - myasthenia. There is a suggestion that the thymus can be the source of the initial acetylcholine receptors to which antibodies are produced.

Treatment

There are various ways to treat Miasthenia, depending on factors such as age, the type of Miasthenia, progression pace.

The main methods of treatment of myasthenia

1. Anticholinesterase preparations (prozerne, oxazil, Kalimin et al.) Improve neuromuscular transmission, are the basis of symptomatic therapy of myasthenia. The effect of anticholinesterase preparations is enhanced with the simultaneous purpose of potassium salts.
2. Corticoid therapy (adrenocorticotropic hormone, prednisone and its derivatives) is carried out against the background of taking anticholinesterase drugs, but their dosages require a significant decrease in the avoidance of a cholinergic crisis. Due to the kaliuramic effect of glucocorticoid patients should receive potassium preparations.
3. Timectomy. Removal of the fork gland is a widespread and fairly effective method of treatment of miastic. The operation is effective only under a relatively non-aid cause of the disease (8-10 years) in patients not older than 50-60 years.
4. Radiation therapy is used to suppress the function of the fork gland. This is a method of choice for the treatment of patients with non-cultural thymomas in opposition to surgical treatment.
5. Immunosuppressive therapy. Azatioprin, methotrexate are used in the ineffectiveness of anticholinesterase preparations and corticosteroids, in older people with an acute form of miasthenia, which are not shown timectomy.
6. Plasmapheresis. In this case, the improvement in the state is explained by removal, together with the plasma of antibodies to acetylcholine receptors, as well as other plasma components that have a pathological effect. With proper treatment, patients may have the restoration of muscle strength, the ability to conduct a normal lifestyle and even a long-lasting remission.

The role of pharmacist

Since patients with Miashenia or their relatives may apply to the pharmacist, he must have the latest information on the safe use of prescribed drugs, about drugs that need to be avoided, and be able to give advice that will help the patient to cope with Miastenia.

1. Emotional support. It is important to prevent family members of patients suffering from myasthenia, how they need their support. They can be recommended to collect as much information on this disease and its treatment. The family should be ready to satisfy the needs of the patient who may arise and offer their help. For example, many patients reluctantly appeal for help in making purchases and child care. The main tips, such as using an electrical toothbrush, can facilitate life to patients with miastenia. A visit to a doctor together with the patient can also be useful, as family members can always get information about problems that may arise.
2. Food. To ensure proper nutrition of the patient, when the muscles needed for chewing and swallowing food are amazed by miastenia, such factors such as diet and choice of food are needed. If the patient experiences fatigue when eating, it can lead to insufficient nutrition or to choose such food that does not provide proper and healthy eating. We should plan meals for the time when patients feel satisfactorily and muscle weakness at a minimum. When the muscles that ensure swallowing, food can be stuck in the throat, and the presence of a family member who knows how to take the reception of Gamelich, can save life.
3. Visiting a dentist. Some dental procedures need to be slightly changed to adapt to the changed muscle strength in the mouth. Patients with Miastenia should avoid some drugs, and also need to be monitored so that there are no side articulation effects caused by drugs that take patients with miastenia. If a serious dental procedure has a serious dental procedure, a preoperative plasmapheresis is sometimes recommended for a patient with severe miastic. When applying local anesthesia, amide derivatives are more preferable than ethereal. Avoid intravascular anesthesia.
4. Side effects of drugs for the treatment of myasthenia. Some drugs appointed during myasthenia can cause gastrointestinal discomfort and diarrhea. At this time, you can give in small portions of rice, bananas, apple puree and dried croutons. If the symptoms do not disappear, you must refer to the doctor.
5. Preparations that should be used with care patients with miastenia. For Miasthenia, more than any other disease, it should be paid to the fact that many drugs cause deterioration of patients, in particular benzodiazepines, chinin, quinidine, morphine, muscle relaxants, inhalation anesthetics, neomycin, streptomycin, tetracycline, sulfonamides, D-penicillamine, hydantoin, magnesium salts, radiopatrants and citrates (table). Therefore, it is recommended to closely monitor whether patients have an increase in muscle weakness after the appointment of any new drug.
6. Other factors provoking myasthenia. The exacerbation of myasthenia may occur under the influence of infection, overheating, taking alcohol, menstruation, pregnancy, somatic diseases.
7. Safety of patients. Patients with generalized myasthenia often experience difficulties when walking, and patients with eye-eye myasthenia often suffer from diplopia (double vision). This may lead to the fact that the patient will not be safe, the house can be the place where accidents and injuries often occur with it.

Patients with a non-solid gait may feel more confident with the frame for walking. In the apartment you need to remove all the free carpets on the floor, and the cables can be accurately placed outside those places where it goes. The sole of the patient's shoes should be non-slip, without high heels. Handrails on the stairs, in the bathroom and in the soul can also provide additional support. It is also necessary to wear a medical alarm bracelet warning that the patient suffers from myasthenia.

Causes an induced Miasthenia in 1-7% of patients

Causes a media induced by the drug.

After the treatment of interferon-alpha may arise miasthenic crisis

Preventing the release of acetylcholine, magnesium can interfere with nervous transmission.

Usually, the amplification of miastic symptoms occurs during the parenteral administration of magnesium, but sometimes observed during oral administration.

Patients with Miastenia should avoid parenteral administration of magnesium

and with care to take drugs with magnesium orally

Possible electrolyte imbalances due to potassium loss

Preparations for general anesthesia

In patients with miastenia, general anesthesia can enhance the effects of drugs blocking nervous means. Inhalation anesthesia can affect directly on the transmission of excitement from the nerve to the muscle (Enflouran, halotan, isofluran)

Preparations for local anesthesia

Intravenous administration of lidocaine and procaine can enhance the effects of drugs that block nerveless transmission

Non-polarizing muscle relaxants (Mivakurium, Atrakurium, Tubokuran)

Cause a continuous neurogenic blockade, which can lead to neurogenic muscle atrophy, peripheral neuropathy and myopathy

Depolarizing Miorosanta (Sukcinilcholine)

Can be applied, but extremely careful

Aminoglycosidic antibiotics (amikatsin, gentamicin, kanamycin, neomycin, neutylmicin, streptomycin, tobramycin)

Imperative nervous transmission

Can cause acute muscular weakness

Can directly block the contractile ability of the muscles

Wakes up the symptoms of Miasthenia

Hinidin and Kinin

Comprehensive education or acetylcholine

Beta blockers (including thymolol, eye drops)

May cause muscle fatigue, short-term diplopy

Reduces the release of acetylcholine from the end of the motor nerve

Patients with myasthenia, which is shown treatment with corticosteroids, need to introduce the drug gradually, gradually increasing the dose to avoid the initial exacerbation of the disease. And also gradually upon completion of treatment it is necessary to reduce the dose

Based on materials sapj.co.za.

Translation Svetlana Bondareva

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Contraindicated drugs during myasthenia

Nervous muscular disease with autoimmune development is Miasthenia - from Latin Myasthenia Gravis. Ten people are extradited by this ailment. More than 50% of patients achieve remission.

The reasons

Some are predisposed to the emergence of Miasthenia - a risk group. It includes:

• Young people aged 20 - 40 years;
• Female Paul - According to ladies statistics, they are sick 3 times more often than men, but in adulthood these indicators are equal.

The main reason for the development of myasthenia is an autoimmune response of the body on its own fabrics. Such a process can run:

Depending on the provoking factor of Miasthenia, it is divided into 2 large classes:

The first view is the outcome of the genetic mutation. A failure leads to a violation in the contractile function of the muscles. The second form is often a consequence of Timus tumor. Miastiy can affect muscle fibers of any body department. More often occurs the eye form of the disease. Children rarely suffer from this ailment. From the total number of patients, they constitute less than 3%.

Clinical picture

Whatever muscle group seized the disease, patients will celebrate general symptoms in the affected area:

• Excessive fatigue;
• Increased weakness;
• Reducing performance and functionality.

Pathological focus causes a person discomfort. This defective part does not cope with the tasks assigned to it. Muscles of any body department can suffer. But especially the eyes are involved in the process. It brings a patient a lot of inconvenience. However, rest for muscles, removing voltage from them, delivers relief. But it is short.

Over time, even a long-term relaxation will not reduce the severity of the patient's symptoms. Progress in the field of pharmacology allows patients to deal with the ailment. The following forms of miastic are allocated in clinical practice:

Diagnostics

In order to fix the fact of the disease, the doctor is not sufficiently symptoms. Any study of the pathological process is not limited to instrumental methods. The doctor examines the patient, finds out the clinical signs of the ailment. He studies the patient's disease. And then assigns a laboratory and tool diagnostics. A standard set of analyzes for identifying Miasthenia consists of the following:

• Functional samples aimed at detecting pathological fatigue muscles;
• Electromyographic study, which indicates activity in the affected areas;
• Decrement test, which allows you to identify the blockade of the neuromuscular signal, and at the same time the severity of the process;
• Transversive sample;
• Biochemical analysis of blood;
• Immunograms;

With difficulty making a diagnosis, a differential study may be assigned by:

• Needle electromyography;
• Study of the conducting functions of nerve fibers;
• Electromiography of certain muscles - jitter.

Treatment

The choice of the therapy line during myasthenia depends on the set of factors:

• Forms of the disease;
• Patient states;
• Concomitant pathologies;
• Prevalence of the process.

Medical treatment is prescribed standard. However, in some cases, surgical intervention is recommended. Autoimmune processes in thymus can cause a timectomy operation. Among the effective medicines are known for "Prezer", "Kalinin" and drugs with a high content of potassium. And also prescribe medicines that stimulate immunity.

To symptomatic treatment, which facilitates the patient's condition, includes the following medicines:

• Anticholinesterase - "Ipigrix";
• Cytostatics;
• Glucocorticoids - "prednisolone", "Metipred";
• Immunoglobulins.

In the case of fast-crossing changes, an extracorporeal hemocorrection is prescribed - a method that helps purify the blood of the patient from antibodies against its own tissues. Even the first procedure gives people a chance to feel improvement. Further therapy helps to achieve a resistant effect.

The effective method is cryosophoresis. This procedure allows you to clean blood from malicious substances, influencing with a reduced temperature. Such treatment is carried out by the course for 5-7 days in a row. Wide distribution received a cascade plasma filtering method. This procedure is carried out using nano cleaners. They purify blood, and then returned to the patient.

Another modern method of treatment of myasthenia can be considered an extracorporeal immunopharmacotherapy. It involves the extraction of lymphocytes in the patient, their medication and subsequent departure to the blood system. With this technique, it was possible to cause persistent remission in patients during the year. It is especially important to adhere to the recommendations of the doctor, since during myasthenia there are contraindicated drugs, the use of which is fraught with hazardous consequences.

Eye form

One of the most common types of disease is orbital. Often it is from her that the Miasthenia process begins, and then applies to other organs. The main symptoms that celebrate the sick:

• Diplopia, that is, bias in the eyes. Patients see not one holistic image;
• Reduction of acuteness and clarity of view;
• Violation in rotational and motor functions of orbits;
• Ptosis, that is, the omission of the eyelids. As a result, the eye slot is not able to open and close normally.

All signs described may be distributed both on both orbits. Usually, closing the eyes for a short time, patients experience relief. However, the slight voltage associated with reading or watching the TV causes discomfort.

Bulbarm

This type of myasthenia can be dangerous to the patient's life. He entails:

• Dysphony - vocal function disorder;
• Dysphagia - a violation of swallowing;
• Dysaryry - disorganization in the work of the muscular apparatus of the pharynx, larynx, as well as soft nose.

The symptomatic manifestations described are dangerous consequences. Dysphagia can move into full inability to swallowing. Food list for such patients is extremely zudd. Food appoints a doctor. Patients have to feed through the probe, they lose weight and weakening. So, their general condition worsens that it does not contribute to recovery.

Disorder of voice formation reduces the social scope of patients. And the dystellium can cause fatal outcome due to respiratory disorders caused by pararesis of voice ligaments that closed the larynx. This is fraught with asphyxis - choking.

Generalized form

The most unfavorable view of the disease is systemic, that is, common. This dangerous type of miasthenia invariably causes up to 1% of deaths among patients with this pathological process. The generalized form captures a large number of muscles, including respiratory facilities - it can cause insufficiency and death when noting assistance.

This disease is often accompanied by the prevalence of the process. Over time, the limited form progresses to systemic. And although patients are frequent remissions, they, as a rule, arise and end suddenly. Therefore, miasthenic episodes and conditions are isolated.

The first quickly begin and end. The second is a long-lasting process, up to several years. However, such a miasthenic state is not prone to progression.

Contraindications

Patients suffering from this ailment are forced to have some restrictions. These include:

• Excess physical exertion;
• Insolation, that is, staying under the right sunny rays;
• Medicinal products with magnesium - "Magnesia" and "Panangin", "Asparkam";
• Miorlaxants stripped;
• Neuroleptic, tranquilizers and preparations that enhance their action - "guides", "Corvalkaps";
• Diuretic, with the exception of "Veroshpiron" and "Spironolactones";
• The use of certain antibiotics according to the type of aminoglycoside - "gentamicin" and "streptomycin", fluoroquinolones - "ENUCSACIN" and "CIPROFLOXACIN";
• Vaccinations.

Contraindicated drugs during myasthenia should not be ignored. There are tables and listings of medicines that answer the question "What is the antibiotics during myasthenia?". Funds should be avoided, which in the list of complications have this disease. These drugs include "glutalit". And it means the use of these tablets is contraindicated. Compliance with the prescriptions of the doctor - a deposit to a favorable course of the disease.