Disease of the nervous system, muscle dystrophy, myasthenia. Inception of skeletal mussels.

Nerve malaise (NMP) is the largest number of recessive malignancies, which are based on genetically determined damage to the anterior horns of the spinal cord, peripheral nerves and skeletal ovaries.

Before the nerve-and-myazovyh get sick:

1) progressive diseases of dystrophy (primary myopathies);

2) spinal and neural amyotrophy (secondary myopathy);

3) congenital non-progressive myopathies;

4) nerve-malignancy with myotonic syndrome;

5) paroxysmal myoplegia;

6) mіasthenіya.

15.2. Progressive myases of dystrophy (primary myopathies)

Progressive symptoms of dystrophy (PMD), abo the primary myopathy, characterized by degenerative changes in the tissue.

pathomorphological changes with PMD, it is characterized by the vitality of ointments, the replacement of fatty tissue and the resulting tissue. In sarcoplasma, focal necrosis appears in the focal necrosis, the nuclei of the oleaginous fibers grow out with lances, the ooze fibers consume darkness.

Nutrition pathogenesis zalizhayutsya to the present hour non-virulent. Myopathy is based on a defect in the membrane of myazovykh cells. It is great hopes to rely on molecular genetics.

The development of myopathies is derived from the type of sedation, the terms of the cob to the process, the nature and the speed of its current and the topography of myase atrophy.

Myopathies are clinically characterized by weakness and atrophy. Find out the form of PMD.

15.2.1. Duchenne myodystrophy (pseudohypertrophic form of PMD)

To meet the most frequent with the PMD (30: 100,000). A form is given to be characterized by an early ear (2-5 years) and an ill-fated interruption, ailments are overwhelmingly lads. Myopathy Duchenne settles down for a recessive type, an X-chromosome connection. The pathological gene is localized in the short arm of the chromosome (X, or the 21st chromosome).

To reach the site of a gene mutation, which explains the frequency of sporadic symptoms. Mutation (most often deletion) of the gene is produced to the extent of dystrophin in the membrane of the oyster cells, which is to produce the structural changes of sarcolemia. The process of flowing calcium and vede to the bend of myofibril.

One of the first signs of an affliction є an infringement of litkovny m'yazіv and deeds to improve їkh ossyagu for rakhunok pseudohypertrophy. The process is of a temporary nature. For the razgornoi stage of ailment is characterized by a "kachina" course, ailments to walk, passing from side to side, which is tied by the head rank due to the weakness of sympathetic muzzles.

As a result, there is a heel of the pelvis in the side of the unsupported leg (Trendelenburg phenomenon) and compensatory heel of the tulub in the opposite side (Duchenne phenomenon). When walking, the party is fucked up for the whole hour. The price can be reconfigured in the position of Trendelenburg by asking the ailing one to lift one leg, bending it down with a straight kut in the knee and tazostegon slope: the pelvis on the boot of the raised leg should fall (and not fall through the middle)

With Duchenne myopathy, it is often indicated that lordosis, cryopodic scapulae, types of contractures are often diagnosed, colony reflexes early on. It is not easy to see changes in the cyst system (deformity of the feet, breast tissue, ridge, diffuse osteoporosis). There may be a decrease in intelligence and development of endocrine disruptions (Adiposogenital syndrome, Isenko-Cushing's syndrome). Up to 14-15 rocky in ailments, you will grow even more wicked, in the thermal stage, weakness can expand on the muzzle of the face, pharynx, diaphragms. To go stench most often on the 3rd ten years of life from cardiomyopathy or the introduction of inter-competitive infections.

Due to the peculiarity of Duchenne myopathy the increase in the adjustment of specific meat enzyme - creatine phosphokinase (CPK) in dozens and hundreds of times, as well as increasing myoglobin in 6-8 times.

For medical genetic counseling, it is important to establish a heterozygous carrier. In 70% of heterozygotes, subclinical and clinical signs of malignant pathology begin: impaired and abnormal lithic mucosa, swelling of mucous membranes when physically imbued with biologic data.

Storinka 44 s 44

Skeletal lymphomas occur before a pathological process in case of malignant degenerative, communicable and fiery ills... In a large number of cases, degeneration of meat fibers is observed at a large amount, and in chronic forms - it is replaced by fatty tissue and fat. Proximal groups of languages ​​are more significant, lower distal, as well as lower values ​​in relation to the upper ones. The sickness of a child grows up to be so called kachinoy (to be overloaded) by foot, not a building big, going on gatherings and getting up, as if to be in the proper sitting position. Tendon reflexes in new attitudes, the steps of which are reduced in proportion to the degree of weakening of the muscular force. Sensitivity does not break.
Prior to diagnostics with valuable laboratory methods, trace the value of the activity of enzymes, especially creatine phosphokinase, in syrovats. It is an enzyme that catalyzes the reaction: phosphocreatine + ADP-creatine + ATP, the presence of a head in the cells of the brain and tissue. With a lot of diffuse malignant diseases, especially with muscular dystrophy, those oversized parts penetrate into the small space and shelter. In ailments, the activity of syvate lactate dehydrogenase and glutamic acid dehydrogenase and glutamic acid acetic transaminase is increased, however, a wide variety in other tissues, including liverworm, changes in the specificity of the test. For a more precise diagnosis, you need a biopsy of myazovaya fabric.
ignited ailments m'yaz_v. Filling of the meat tissue of the supernatant diseases, especially trichinellosis, toxoplasmosis and viclican with the Coxsaki virus. It is often a component of collagen ailments, including dermatomyositis, corvus arthritis, periarteritis and rheumatoid arthritis.
Polymyositis. Diffuse isolation is called polymyositis. For a new type of shvidke progressively stretching, weakness and bile in the proximal groups of ointments. Often in the process, the muses are drawn into the process, in the connection with whom the child is important to lift his head and put it in the right position. Prior to laboratory signs of malignancy, the increase in the number of leukocytes is considered. However, the visibility is not a polymiozit. Rivn syrovatkovyh enzymes evolve. Meat biopsy shows degeneration and partial regeneration of fibers and infiltration of lymphoid cells. Differentiation polymyositis from muscular dystrophy and dermatomyositis is important. I can be myself atypical form dermatomyositis, if there is a histological picture in case of cich two camps, there is a growth: for dermatomyositis, characteristic vasculitis, it is noticeable in case of polymyositis. The prognosis for the last one is friendly. Likuvannya corticosteroids supravodzhutsya efect, otherwise with їkh skasuvannya may relapse.
Progressive ostifikuyuchiy myositis. Ethiology of the tsyogo ridko grow up to become ill with good fabrics and the language of the home. To see how many brothers and sisters are suffering, including twins, and be passed on to blood relatives along a straight line. Prypuskayut, scho calms down ailments on an autosomal dominant type. Boys are sick 2-3 times more often than girls.
Pathological signs to lie down at the stage of becoming ill. At the early stages of muscular embryos and ignition cells, infiltrates are found in the muscles and tendons. As a result of the production process, it is replaced by granular tissue, and in the middle of the process, a collection of cartilage and cyst tissue is formed.
In 75% of ailing children, there is a development of wadi development, most often underactive fingers and ankylosis of the phalanges of the first toes, polydactylation, malformation of the fingers, syndromes, deformity in the shells, Deafness, teeth visibility. The same kind of wadi can be found among the relatives of the sick, among those who did not develop progressively ill-gotten gains in the fabric of the disease. Vіk, in any way you can see an osifikuyuchiy mіosit, varіyu from the moment of the nation to the eldest childish vіk. Consider the development of three stages of this: 1) at the sites of minor muscle injuries, there are intercourse, often warm and mild to a little more similar swelling of soft tissues; 2) after a few days the symptoms become hotter, and the symptoms are harder; 3) ossification of the unguarded dilyanka is observed. Periodically, there are new brands, especially in the areas of the neck and back. let me know first Maybe the crookedness, which is the process of developing into the sternocleidomastoid-nipple-like meat. The deep ossification expands to the rich tendon and ligament. Infusion of ankylosis of the ridge and slopes of the arms and legs (Fig. 21-5). The zapalennya can be widened to the skrone-lower slope slopes, in which there can not be zhivalny ruffs. Kistkovi virosty can be seen through the shkіra. In a young person's sickness, it is often caused to an increase in sickness and death due to dysfunctional deficiencies and emotional distress, if you want to know about vision problems. In case of osteogenic myositis, the development of osteogenic sarcoma is not safe.

Small. 21-5. Ditin with progressive ostifikuyuyu myositis (typical posture with a rigid mucosa and back).

One pathological process of bridging the mice overflow with injuries of soft tissues (miositis ossificans circumscripta). The calcification of the tissue tissue is widely expanded and it can occur in chronic polymyositis and dermatomyositis.
results laboratory methods Do not miss the diagnostic value.
Equivalent in calcium, phosphorus, puddle phosphatase, as well as the activity of creatine phosphokinase and other enzymes are too low in the norm. Kistkova fabric in the middle of the day, the school does not follow the Buda norms.
The most advanced methods of treatment are not satisfactory. In some cases, it is indicated that there is a hope for the development of sickness in the presence of ACTH and corticosteroids. Їх role in the final results
Endocrine and common myopathies. Myopathy at hyperthyroidism For new types of ptosis, bilateral paresis of the facial muscles and the muscles of the proximal symptoms. With a large number of symptoms, hyperthyroidism may mask the muscle weakness, but in manifestation, tachycardia may be lost, as well as the persistence and improvement. thyroid gland... Tendon reflexes on the basis of various forms of myopathy become normal. For the correction of hyperthyroidism, the weakness of the step by step is known.
Myopathy in hypothyroidism. Hypothyroidism in infants can be a dressing with weakness and hypothyroidism. In children of an older child's age, a quick and weakening of the muscles develop with a mixdema, and in some cases there is a type of hypertrophy (Debreu-Semelu syndrome). The presence of such signs, such as weakness and hypertrophy of the muscular tissues, and the possibility of allowing the muscular dystrophy.
Myopathy in the treatment of corticosteroids. You can slow down Isenko-Cushing's ailments, or develop more often when you use large doses of synthetic steroids. The weakness is especially noticeable in the joints of the pelvic girdle, which manifests itself in the cross-over (kachina) walk, which is difficult when going through the gatherings and trying to get up from the seated position. Colinny reflex in the daytime. The vitonishing of myaziv may come. Myopathic changes in myazovy tissue give rise to insignificant changes in case of severe weakness. The force of the corticosteroids is updated every now and then (with a hand of decalcaps).
Myopathy in hyperparathyroidism. Hyperparathyroidism can be associated with dressings due to weakness and hyporeflexia, zooming in by hyperkalemia. Smell the stench shyly know about the parathyroid dectomy.

Deficiency of carnitis (lipid dystrophy) is a supernatant accumulated in great numbers of lipids in myases and damage in connection with the cym of energy-induced disease of the remains. Carnitine is carried to the ligamentous components of the system, so that the transfer of fatty acids from the cytosol to the cytosol in the mitochondria is prevented from transferring fatty acids into the cytosol, so that it becomes oxidized. Myazova weakness develops with two forms of lack of carnitine.
Lack of carnitine in meat is classically represented by progressive weakness of proximal groups, often among schoolchildren and children. Some weakness is intermittent and goes back to myoglobinuria. In case of severe interruption, there may be a paralysis of dichal muscles. The enzyme rin (creatine kinase and aldolase) is growing in syrovaty. On the electromyogram, nonspecific changes, the power of myopathies, appear. In biopsy specimens, a large number of specks of fat can be found. Rivn carnitin in syrovattsi does not change, but it doesn’t decrease in the meanings. The recognition of pathology is not very significant, so it can be curable. It’s not easy to take me for dystrophy. The effect can occur if the consumption is in the middle of 100 mg / (kg / dobu) carnitine. In some cases, corticosteroids are more effective than corticosteroids.

1. The systemic lack of carnitis manifests itself as progressive myopathy, including cardiomyopathy, and liver dysfunction, which is supervised by a clinical hepatic encephalopathy of the type of Reye's syndrome. As a result of the last carnitic deficiency, it turns out to be a recurrent interruption and to be prevented by periods of aggravated encephalopathy with a pronounced muscle weakness. The level of creatine phosphokinase in syrovatz has been adjusted, some of the carnitine has been changed in syrovatz, as well as in meat. The changes in biopsy are analogous to those in case of deficiency of carnitine in the tissue. See more clientele morphological changes In addition, carnitic deficiency can occur with damaged exchange of organic acids, for example, with methylmalonic and glutaric aciduria (secondary carnitine deficiency).

Small. 21-6. Ditina with a vivid view of the great breast.



I respect the visibility of the anterior groin folds and the low-worn nipple.

Likuvannya polyagaє in the pretrimanny we are sick of children, rich in carbohydrates and zezhirenoi, and priyomi carnitinu in extra dose 100 mg / kg.
There are signs of defects. Vrodzhena vіdnіst m'yazi. An underactive mutilation can be increased by broadening and leading to an additional blockade of rukhiv in the slopes or congenital arthrogryposis. Yak incidence defect most often every day is one m'yaz. Before reaching a broader anomaly, the visibility of the sternal part of the great breast meat is seen (Fig. 21-6), in some cases, the defect is associated with syndactylation on the side (Poland's syndrome). The visibility of the breast meat is often superficial to myase dystrophy. The appearance of the abdominal mucosa is often tied with defects in the development of the sečovyh nozzles.

Small. 21-7. Deformation of the shia and asymmetry of an individual in a boy with natural crookedness, which has not been treated for a 12-year period.

Vrodzhena crankshaft is pummeled with unilateral shortening or contracture of the sternocleidomastoid nipple meat. The head of the sick person is nailed towards the contracture, and when it is straightened from the bottom to the opposite side (Fig. 21-7). When correcting the position of the head, a significant opyr is seen. In the urazhen_y m'yazi there will be a lot of gorseries. The reason for the defect is unclear; after a trivial hour, he was respected as a result of birth trauma. However, crookedness is seen in children, the people of the bridegroom for the additional operation of the cesarean rosette; it is possible to let it go, but in some cases the reason for the defect is introduced to the intrauterine period. Crooked necks of differentiation from pathological head nagging due to deformations of shinny ridges, for example, in case of Klippel-Weyl anomalies, and because of fractures or vivikh of shinny ridges. Їх viklyuchayut with the help of X-ray dosage. In older children, Viku has nahil heads can be found with oblique bones, dystonia, puffins of the posterior cranial fossa and shyny viddilu spinal cord, Ostifikuyuchiy mіositі, shy lymphadenitis for diaphragmatic grids. Most vipadkiv vrodzhen krivoshego korostiya korektsii for additional help educational gymnastics... However, at chronic form curl to produce an asymmetric development of an individual and a head (see Fig. 21-7), in conjunction with which it is possible to develop the muzzle, as well as for cosmetic purposes.
Generous myopathies. The group includes a sprinkle of rare forms of sedated ailments, with any type of weakness and hypotonia from the breast (div. Table 22-1). Їх accurate diagnostics is less significant from the point of view of the forecast. In general, for a normal life and triviality of life, I am friendly to the sight of the ailment of Verdnig - Hoffman or an inborn malignant dystrophy. Revealing of congenital myopathies will develop into a biopsy.

1. Syringe of the central swivel. The central part of the ovary fibers is barbed abnormally, ale є one-sided. With electronic microscopic dosage, there is a change in the number of mitochondria and the formation of sarcoplasmic reticulum in the central part of the fibers.

Non-malinovaya myopathy. The term "non-malinovaya" is explained by the fact that in the meat fibers there are no similar structures.

Small. 21-8. Myotonic fast movement (a) with a sharp blow with a percussion hammer on the right half and vik (b) in a child with a hypercalated form of family periodic parallel.

When we look down, we’ll get too fast.

The date of the electronic microscopic dosage is to indicate that the result is a change in the Z-mice of myofibril.
Mytochondriacal myopathies. Talk about some forms of myopathy, with some of the most important changes vіdbuvajutsya in the mitochondria of oily fibers. The stench can be boiled down to a yak in number, as well as in size. M'yazovi weakness and hypotonia can begin already in the breast, but sometimes it’s only possible to progress in school. Cardiomyopathy, encephalopathy and lactic acidosis often superimpose the myopathy of the group.
myotony... Tsei stan є I am familiar with the diseases of the young, for example, the dystrophic myotonia, the hypercaline form of the family paroxysmal parallel and the ailment accumulated to the glycogen. Myotony begins as a sign of confidence in the development of the weakening of the languages ​​in the minds of those who are quite or very quickly. It is usually manifested in the lack of a tight fist, or in the visible trivial fast mouth after its stimulation, which appears to rotate in a thin razdrauvanny (Fig. 21-8). The price can be played by hitting a percussion hammer on the surface group of muzzles, for example on the muses of the mov and the hollow surfaces in the area of ​​the first finger. Myotony is confirmed by the data of electromyography. In general, the spontaneous activity of the muses is characteristic of the spontaneous activity of the mucous in the form of a weakening, or rather high speed (myotonic discharges).
Vrodzhena myotonia (Thomsen's disease)... I will be the only acquaintance of myotonia, who was sedated for the dominant type. It can be manifested in the breast in viglyadi the trust of covtal rukhiv and blues along
due to lack of gaps to normal weakening of the pharyngeal mucosa. At the elder one childish myotonia is manifested as a weakness of a sick person squeezed into a fist with a finger. When you first try it, the roc of the child's m'yazi will become solid. When bagatorazovy is repeated, the stench of the stench grows loose. So, for example, the sickness of the child is great difficulties for the cob to the act of walking. The first little bit of crockwines will make you sound even worse and more often. After a few seconds of the stroke, it becomes normal or normal. Symptoms of myotonia are lost with an uncomfortable emotional state of a sick person and a cold organism. M'yazov's power to be in the norm, m'yazi to finish the rozvineni and not so badly to the healthier, but also to the feud of the athletics statues of the sick.
Diagnosis is based on clinical and electromyography data. The activity of syrovatkovyh enzymes is in the range of norms. Let us serve as a histological acquaintance with the hypertrophy of the myaz fibers.
The form of dystrophic myotonia, congestion leads to the appearance of weakness and atrophy and dystrophic changes in the biopsy of the tissue. Lіkuvannya novokainom or sulphate hіnіdinu supernatant is shown in case of functional breakdowns. Perebіg ill-fated to call in good-heartedness, and the camp of the ailing one can be polished.
Paroxysmal parallel. For the whole group, the disease is characterized by periodic weakness due to increased or even more changes in strength during the period between attacks. Prior to it, there is a deficiency of myazovy phosphorylase (McArdle's ailment).
Hypercalated paroxysmal parallich. Spadkova epizodic adynamia, or paramiotonia, is passed on to the dominant type and a particularly important change in particular cholovic status. To fix up for an early child (one in a breast). Attack will be found in the period due to an important contribution. Weakness quickly develops, as it can be trivial with a stretch of decilkoh years. Vona is especially visible in the legs; the function of the dichotomy is not ruined. Often, adynamia of superficial myotonia, which is likely to occur between attacks, is most likely to manifest itself in the eyes of the eyes when looking down (Div. Fig. 21-8, b).
Before an hour attack, there are often attacks in Syrovatz, in order to ensure that there are more attacks. Piece by piece it is possible to provoke an attack for additional support (2-3 g in the middle), however, it should only be carried out under the control of the EKG. Repeated attacks will stop the diakarb. Important forms of congestion are characterized by the development of chronic, weakly rotated weakness and degenerative changes in the meat.
Hypokaline paroxysmal paralych... Family paroxysmal parallich, also sedated for the dominant type, is especially important for the boys. On the vidminu from the hyperkalієmіchnіy, the first attack is to appear in a young childish or early adult. The reason to serve as an implantation of ryasnoi їzhі, rich in carbohydrates, for reasons of food physical navantazhennya... Zvvychay attack to obey on the offensive wound of an important physical nurturing and ryas evening. Wine is characterized by its weakness and reflexion. The dichanny function may have been ruined. Arrhythmias may occur, including suckers, extrasystoles and tachycardia. Attack can be trivial for more than 24 years. In the paralytic phase, the calories in syrovatz gradually decrease (2-3 mmol / l). The main defect is nevidomy. In sick people, with repeated important attacks, chronic muscle weakness and pathological changes in the meat develop. After an hour of attacks, store in the amount of calcium chloride; the dose of cob to become 2-3 g. Diacarb with a decrease in the frequency of attacks.
Paroxysmal myoglobinuria (idiopathic myoglobinuria)... Idiopathic myoglobinuria is a rare group of illnesses, when they attack parallel with myoglobinuria, they are found spontaneously or because of intensive physical intervention. Zakvoryuvannya settles for the dominant type, with the X-chromosome. M'yazi, most often litkovi and stegnovy, before an hour the attack will become painful and swelling. Cut nabuvaє dark red or brown color. Myoglobinuria can zoom the necrosis of the nyrcoval tubules, which can lead to a lethal result as a result nirkova lack.
Diagnosis is confirmed to occur in the section of myoglobulin. A positive benzidine test is positive when there is a presence in the section of the epithelial cells, and the presence of hemoglobin is not detected, especially if there is no hemoglobin in syrovat. Hemoglobin is used for additional spectrophotometry. Paroxysmal myoglobinuria following the development of McArdle's ailments, lack of carnitinpalmityltransferase and myoglobinuria due to uninvolved intensive physical therapy due to injuries of the ligament great people... Myoglobinuria for the important meaty nurturing to develop in case of pseudohypertrophic muscular dystrophy (Duchenne ailment).
Lіkuvannya polyagaє in a pre-trimmed postіlny mode; if necessary carry out piece ventilation lungs. To get rid of nirkova deficiencies, it is necessary to recognize the ailing bagato piti.
Lack of carnitinpalmityltransferase. With a deficiency of the enzyme, the transfer of low-lanceolate fatty acids in the mitochondrial segments, in which the oxidized and production of ketones, will break down. Lack of isoenzyme II type settles down behind the recessive type. As a result of this deficiency, ketogenesis in tissues, including in tissues and tissues, is impaired. The first signs of ailments are often found in children of school and adulthood. Stink in repeated episodes of pain in the muscles, weakness and increased temperature, because of physical pressure or hunger. Myoglobinuria, when attacked by a supervisor, can lead to a nirk disadvantage. Hunger to produce hypooglycemia. When children attack, they look healthy. It is necessary to differentiate from one country to another in order to be supervised by periodic weakness and microbiality. Differential and diagnostic value of volodya method of determining the activity of carnitinpalmityltransferase. Vona decrease in meat and liver tissue, leukocytes and culture of fibroblasts. Dotrimannya dієti, scho stock up on products, substituted with carbohydrates and fatty, with a decrease in the number of attacks.
M'yazovy dystrophies. These anomalies are reported to a group of family members who are ill, which are supervised by degeneration of the myaz fibers. The classification of muscular dystrophy is based on such signs as the ear, the speed of progress, the growth of changes in the groups of mussels and the type of decline.
Pseudohypertrophic muscular dystrophy... A child, or a form of Duchenne, is the most extended form of muscular dystrophy; її frequency to become 0.14 per 1000 children. In the classic form of the virus, it is opposed only to the boys, moreover, the sedation, the X-chromosome, is found in approximately 50% of the probands. In іnshih vipadki, the disease is surrounded by new mutations. Talk about the rare form of muscular dystrophy, on the basis of the identical form of Duchenne, but not for the recessive type with the same frequency of sickness in lads and children. It is quite possible to diagnose a child's illness for up to 3 years. In the anamnesis, pick up tales for those who didn’t have a child’s ability to develop their functions, they didn’t seem to be able to sit, walk and grow, and, naturally, tell us about more early ear getting sick. Overwhelming (kachina) of the course, difficult when going along the descents, the hypertrophy of the lithic joints is carried to the most important critical events. In children, before the process, there are also іnshі mucus, deltoid sprinkle, brachioradial, tongue mucus.

Small. 21-9. Typical positions taken when inserted from a pidlogue (Govers symptom) by a child in 7 years from a pseudohypertrophic myopathy.
In the position of standing (the rest of the photograph), the lordosis bends are signified.

On the cob of getting sick of hypertrophies, there is a significant power, but it does not change (pseudohypertrophy), so as to increase the mass of muses to be found for the booze. The power of the hypertrophied lithic meat significantly outweighs the force of the joints of the anterior surface of the thigh, which explains the part of the tendon contracture and the movement of the child on the fingers. The weakness of the muscles of the pelvic girdle turns into a characteristic quality (lordic) walk and difficult, as the child sees, if she stands up from the seated position on the floor. When reaching important forms of malignant dystrophy in a child, Govers' symptom is indicated: standing up from the podlogs, win persh for all the age, spiraling onto his hands, and then walking, lastly coming out with his hands, as many as 9 ). Visually, the weakness of the joints of the shoulder girdle is possible if the child is in a raised position for groin. At normі win, we wrap ourselves up, press down our hands to the tulub; with muscular dystrophy, win yak bi lick through the hands. The sick child is often unable to lift his hands over his head. At the beginning of the stage, the atrophy develops. Call up to 12 rocky, the child can't walk anymore. Ailments in 75% of the populations die at the age of up to 20 years. In most of them, cardiomyopathy is indicated, as in some cases raptor death... As a decline in the X-chromosome, and sickness in an older child, the triviality of life becomes great (Becker's dystrophy). The average rate of pink development for children with the Duchenne shape should be 80; 25% of children have a pink rose.
at differential diagnostics M'yazovy dystrophy Duchenne follow the mother on the view of Verdnig-Hoffmann's ailment in children of senior breast cancer and such m'yazov, Yak endocrine myopathy, lack of carnitine, ailments accumulating glycogen and polymyositis. In some cases, with contractures of the tendon and movement of the child on the fingers, cerebral palsy can be suppressed, however, with muscular dystrophy of the onset, spasticity and hyperreflexia are characteristic of cerebral palsy.
Diagnosis of the basis for the significance of the activity of sirovatkovy enzymes, data from electro-chromatography and biopsies of tissue tissue. The activity of enzymes, especially creatine phosphokinase, before development key symptoms I often change the norms in 10 times to get breastfeeding in children. On the electromyogram, they appear before a decrease in triviality and a decrease in the amplitude of their potential. Histological changes appear in the degeneration of the myaz fibers. The stench often grows behind size and partly substituted with fat and full tissue. The size of the kernels is also varied. Diagnosis can be made in the case of a popular way of determining the activity of creatine phosphokinase. Methods of revealing women-noses are not yet broken, do not marvel at those, but in 60-80% of them, they appear insignificantly or even more so. Signs of types are great for a child's life, not for the coming periods of life.
effective methods lіkuvannya not ісnu. The slid of the yakomog is more likely to receive the active camp of the sick and the healthier walks. It is necessary to follow the team, because the child has a unique intensive physical preparation, as it can be the reason for the ripping of the meat fibers. In some cases of surgical hypotension of the tendon with the ability to walk, a protested postal regimen for orthopedic correction can lead to muscle atrophy. The role of genetic counseling is important.
Vrodzhena myazova dystrophy. Affection for an autosomal recessive type and is characterized by oily hypotension and weakness in a baby's breast. You should enter before the group of stanіv, where you start yak "mlyaviy ditina" (div. Table 21-1). An ear of corn is introduced until the intrauterine period. In some cases, the new-born one has an atrophy of the mucous membranes, contractures, and the decay of the slopes. Differentiation from Verdnig-Hoffman's ailments is difficult. Fastsіkulyatsіі movi, characteristic for the rest, visible in the muscular dystrophy. Tendon reflexes are impaired, but more often not involved. The processes draw in the diaphragms, as they take part in the dichanna, including the diaphragm. In case of a serious interruption, death persists in a person up to 1 fate due to a dysfunctional deficiency; with more mild forms, normal life is taken for a trivial hour. The increase in the activity of fermentous enzymes does not mean that there are dystrophic changes in the meats.
Shoulder-blade-face form of muscular dystrophy. qia finish easy form myazovaya dystrophy calmed down on an autosomal dominant type. To fix it, you should be born in 10-20 years and be characterized by weakness and atrophy of the face and shoulder girdle. A special thing is amicable, ailments cannot close their eyes and whistle. Inspiration is progressing by and large and summarily with the normal triviality of life. Diagnosis of tests on key donations and types of sedation. The results of the biopsies of myazovaya fabrics indicate about dystrophic changes in them. The level of creatine phosphokinase in syrovattsi can be lost between the norms or slightly move.
Pelvic form of muscular dystrophy. A group of non-uniform depressions is characterized by the general progress of muscular dystrophy and settling down by the autosomal recessive type. An ear of zhvoryuvannya is introduced to an older child, or a grown-up one. Zazvychay urazhayutsya m'yazi pelvic girdle.
Full-time myopathy form. Dystrophic changes are seen in the main in the most recent ones. To recover from getting sick in a childish abo pidlitkovy vіtsі. When new, ptosis and intercourse ruchiv progress real apples... Some weakness expands on the face of conviction and shia. Inspiration of the slid of differentiation from the masthenia and parallels cranial nerves with puffs of stovbur to the brain.
Progressive ophthalmoplegia, which can be repaired in a childish abnormally old age, is associated with atypical pigment degeneration of the heart and heart block (Kerns-Sayers syndrome). Call with her the knitting and progressing ataxia, the extension of the growth and the statutory maturity. From the sarcolemma, there is a large collection of atypical mitochondria. The genetic nature of this process has not been established. It is possible to control the possibility of raptor death due to the deterioration of the heart conductivity due to the additional cardiac pacemaker.
Myotonic dystrophy. Unimportant for those who have myotonic dystrophies, it is necessary to repair them among the older people, and the cob is more and more often restructuring in the children of the breast and the big childish child. Vona calmed down on an autosomal dominant type. One ear in a childish vіtsі to tell about those who mіotonіyu mіotonіyu mіtі mati. Depending on the inner uterine factor, it can be influenced by the turn of the child's illness. Already at the moment of the nation's life, there may already be a hypotonia at a new age, at a new day of birth there may be a hysteria. Renovation of physical and rose development will start to appear more and more. In an early child's life, weakness and atrophy expands mainly on the basis of individuals, cracked and scarce muscles. Name it double-sided ptosis. Prior to diagnostically significant methods, there should be percussion of music, electromyography; typical for tsikh ailments nezdatnіst roztisnuti stisla in a fist brush (div. Vrodzhena mіotonia). The weakness and atrophy of the muscles of the kidneys and the pelvic girdle (the distal group) appear in the older childish abnormally old child. In older adults, there is a cataract, oblivion, atrophy of the testicles.

Diagnosis of symptoms for the revealed signs of myotonia, characteristic of the growth of the muscular weakness, the decline for the dominant type, dystrophic symptoms of the muscularis. A child's child may have become uncomfortable, and often he will be uncomfortable. Until the youthful viku myazov, the weakness of the vistupa is in the foreground. In case of functional damage, treatment with novokain and chinidin is shown.

In addition to the sickly depressions, which are removed in case of injuries (for example, breakdowns and breakdowns), breakdowns in the muscles can be present and at the time of the daytime, they are injected. Up to the ailment m'yaz_v can be brought:

M'yazova sudoma;

Rheumatic illnesses;

fired;

Genetic illnesses;

Destruction of the exchange of speech;

Zmіna of myazovykh clitins.

All illnesses are readily available in a report.

m'yazova sudoma

Sudoma can occur as a result of vomiting organism (exicosis). At the same moment, the muses quickened and became solid, then the stench became more and more relaxed. Sudoma can win in the night or vrance. Lyudina quickly started to see strong beat in music. Judge most often to see people with a kidney disease. If there is a lot of navantazhennya on the m'yazi, or if the hardening of them collapses, then solidification will appear. M'yazovy fibers are transformed into m'yazovy fabric, in which solid dylyankas are washed at viglyadi universities. In such vipadki it is necessary to drink a lot of ridini, in order to bring the water-salt balance into the body. So also to come to help massage. If there is no pain in the meat, then it is necessary to turn up to the lykar. Likuyuyu hardening for additional massage, vitamins E and warm baths.

rheumatic fever

Є There is even a great number of ailments, which can be brought to rheumatic ones .. In cases of illnesses, dzherelo battle is the muz itself, or the bloodthirsty judges, as it is to live the mujaz. There are pains in the legs and shoulders. Deyakі zvoryuvannya rheumatic character (for example, dermatomіosіti) oppose m'yazi. In this vypadku need to use hormones - glucocorticoids. The stench will bring the hot air, ale viklikayut side effects... To that rheumatic ailment try to strangle with the help of the anti-fatigues or physiotherapy.

Loss of hormonal character

Ailments are the weakness of myopathies in medicine, as endocrine myopathy is changing, as there is a strengthening of the function of the thyroid gland or supra-nirkovy glands. Pislya likuvannya pain to know.

firing myaziv

The firing of myazіv is called myositis. Symptoms of this illness are also like those with rheumatism, altered rice, and the burning of the mucus themselves. For myositis, myositis is a weakness. Eliminate the incineration of malignancies in the same way as rheumatic malaise.

Incomplete mineral speech

For the normal function of the language you need a speech. If there is a deficiency of calories, the vine is parallich. Especially young people and children are welcomed on an important day. Likuyuyu for additional drugs, to avenge calories. In addition, before going to bed, it’s not so big є і actively go in for sports.

not enough enzymes

Children can rarely suffer from enzyme deficiency. Often, there is a deterioration in the functions of enzymes, which take part in the broken down glucose and glucogen, as well as energy for the mucous. As a result of the congenital deficiency of the enzyme, the ointment will consume little energy as a result of the weakening of their robots. With such a diagnosis, Lyudin is guilty of a unique physical nurturing.

It is ailing in the m'yaz_v

In the case of myaziv, which is accompanied by pain, there is a history of acidosis. For the removal of energy in case of great navantazhennya, the breakdown of glucose to lactic acid is taken into account, as it is important to vivod in the body. Accumulated in meats, lactic acid is sickly.

Everybody has a lot of athletes for getting pain in the muscles, polishing food, renewing and living with a mangosteen.

It is necessary to live clean water.

Progressive degenerative illness nervous system to be diagnosed as a result of genetically determined pathology or a defect in the development of the membranes. Serve with sleeping symptoms: degenerative character and systemic character nerve tissue, Progressive reboot. To them, it is brought up, zokrema, syringomyєliya, when the spinal cord is formed in the spinal cord, it’s empty, rear horns... Tse produce up to a defect in temperature sensitivity, Atropatena.
The group of recessive ataxias is numerous, їkh the main manifestation is ataxia, it is tied to the pathology of cerebellar paths or to a great sensitivity.
Bichny amyotrophic sclerosis (ALS)- important and rapidly progressing ill, Sopron nobles in the spinal cord. With a wide variety of symptoms, a combination of atrophic paresis and pyramidal symptoms, so that the development of rukhovi razlady has a character at once, both peripheral and central parallels.
Parkinson's ailment is a progressive disease, which is based on the primary deterioration of the pigment-containing dopaminergic neurons of the main part of the black substance and the small pigment-containing nuclei of the stovbur. Rizik for the closest relatives of a sick person is about 10 times more common than in the population. For Parkinson's ailment, a triad of symptoms is characteristic: tremor, podvyshennya myazovogo tone and hypochondria; Criteria for diagnosis to complete folding. Klіnіka of ailments is only a little bit, if more than 80% of neurons are present. In a number of vypadkiv ailments debut in children up to 18 years (so the titles of young parkinsonism), in the older generation. Tse visuvaє pіdvishenі vimogu to the freedom and adequacy of treatment.
The therapy is very adequate and given a lot of opportunities for good compensation of damaged functions, as well as saving or renewing social adaptation.

Diseases of the nervous system

Degenerative nerve diseases- zagvoryuvannya z perevazhnymi urazhennye nerve-m'yazovogo apparatus of a recessive nature. The stench is also known as progressive neuro-malignant dystrophy (PMD) and becoming the best group in the midst of declining ailments.

Classification of progressive meat dystrophies

1. Progressive diseases of dystrophies - genetically determined changes with the primary progressive degenerative changes in the muscles (without primary pathology of the peripheral motor neuron). With them m'yazova fabricє The target of the primary gene defect, through which abnormal synthesis of the myadic protein myodistrophin occurs, and the rapid decline. The dissection of the nervous system in myopathy is of a secondary nature.
2. Spinal amyotrophies are primarily genetically determined striations of the anterior horns of the spinal cord with secondary progressive peripheral parallels and atrophies.
3. Neural amyotrophy is the primary genetically determined syndrome of polyneuropathy (as a result of myelinopathy) with the development of secondary amyotrophy and vegetative-sensory damage.

Primary progressive symptoms of dystrophy
The development of illnesses is manifested in the development of the age - from 1-2 years to 40-50 and older. The stench is characterized by rukhovoy non-handiness, unstykistiu, fall when walking, and stomachache. The child is victorious and fearful and walks without prejudice. For the sick, there is a molded vineyard rolling motion"- waddling.
Pseudohypertrophy of muscular forms is characteristic of deyaky forms, often litkov_ m'yazi: Їх atrophy due to masks of atrophy and to help increase the size through the growth of fatty tissue. Weakness and atrophy of the muscles are localized in the muscles of the pelvic girdle, with the maximum variability in the proximal parts of the nig.
Є bends of transverse lordosis, scoliasis, "krylopodіbni" scapula, vuzka "aspen" talіya. From the position of sedentary complications and children to go to the additional priyomy (priyomi Govers) - "scramble for a drabink", "scramble for oneself." As a result of the development of dementia. Guardian - heart m'yaz. How many ailments go to the building are walking on their own. Do processes get involved heart-vascular system(To develop dilatation or hypertrophic cardiomyopathy).

Secondary - spinal and neural muscular dystrophy

Spinal dystrophy (amyotrophy) settles down in an autosomal recessive type. The gene for spinal muscular atrophy is mapped to chromosome 5gl1.2-13.3.

You can see early signs of boulevard razladiv. Covering the motor connection. When conducting electromyography, lesions of the anterior horns of the spinal cord appear. Perebіg became ill progress.
In case of spinal forms of amyotrophy, the potential for fibrillation is reestablished on the electromist-ogram in a calm state; The speed of the increased impulse along the nerves of the nerves is also saved, and even less as a result of the loss of the spinal motor neurons.
A common variant of neural amyotrophy is Sharko-Mari-Tooth neural amyotrophy. Behind her clinical manifestations, she has a sensory-motor polyneuropathy, distally accentuated and repaired from feet and homilies. The flow is good, generally. Deformation of nig is characteristic every year - behind the type of "nig leleki" or "galife": thin as a result of atrophy of homilka with preserving stegon meat. A collection of "vipadayut" Achilles reflexes, then decrease the number.
When electroneuromyography is re-structured, a gross decrease in the speed of an expanded pulse along the nerves of the brain is being re-structured.

Mіasthenіya. Myasthenic and cholinergic crises

mіasthenіya(Miastenia gravis pseudoparalitica) is an important nerve disorder of the autoimmune nature, which is characterized by pathological stomatology and weak transverse swarthy muscles (Akimov G.A., Odinak M.M., 2000).
etiopathogenesis... The main lanka is the diagnosis of autoantitils to nicotinic cholinergic receptors of the endocytic lamina of the ovary fiber and the block of nerve-mucous transmission. Є links to the pathogenesis of myasthenia with lesions of the pitchfork. Often it appears as Tim (up to 40% of vipads), before it is atrophy of the thymus.
clinika... Mystenia can be recognized in any occasion, or more often - between 16 and 40 rocks, protest and more early, and more forms (in terms of the period of sickness in 30 and 70 rocks). Women are sick more often for the choloviks. The main symptom is the pathological stoma of the mucus with the development of weakness in case of repeated collapses, for example, the diagnosis of double or ptosis in the case of reading.
When developing at the muscle mass, the weakness appears in peripheral or central paresis, but with repeated collapses, especially at a fast pace, there is a rapid growth and you can reach the stage of a secondary parallel. When I start to recover, sleep, and persistent rucks can be normal, protest at the onset there is a moment, the steps of what progress are made at the continuation of renewal.
Myasthenic epizod can develop among new-born children who have come to see mothers who are suffering from myasthenia (so called the myasthenia of new-born women) The step of compensation for ruffian problems can be more abundant (for self-service in a beggar), a rotten (a necessary third-party look). The most serious threat of accelerated myasthenia is a myasthenic crisis.
myasthenic crisis- the rapid development of a non-critical camp as a result of the block of nerve-and-meat conductivity. The main symptoms are a generalized weakness developing rapidly, reaching the level of tetraplegia.

expedited:

1. destructive behavior with bulbar form,
2. risik obturation dikhalnyh nobles accumulate viscous mucus,
3. the possibility of aspirations of the epiglottis or "valvular asphyxia"
4. vimicance of diaphragms and weakness of intercostal dichal muses.

An overdose of anticholinergic drugs can lead to the development of a cholinergic crisis with severe self-esteem. Unavailable assistance»Appear medical advisors in the intensive care unit (ward).

Fumes are weak, the main symptoms are weakness, which is tied to the flesh of the muscles transversely. Before firing myopathies, there are idiopathic firing myopathies, myopathies, tying with infectious diseases, and myopathies, tying with injecting medicinal products and toxins. Among them are the most important є signs of polymyositis and dermatomyositis. In these statistics, the symptoms of the firing of the tongue are clear and the main signs of the firing of the tongue in the people. In addition, we provide information on diagnostics of fired compounds.

Symptoms of inflammation of myaziv

In the debut of symptoms, there are a lot of ailments in the presence of ailments, signs of unconsciousness, impaired weakness, and a decrease in shkiri (with dermatomyositis). In a frivolous act (stretching out decliners) to the firing of the mucous membranes, the symptoms of a progressive build-up of weakness in the proximal groups of mucous membranes will appear. The children of the children with signs of fiery language (children and young vik) Sposter gostriy cob, often bewildered by swelling constitutional signs (feverishness, skinny and іn.) And myalgias.

Much more (with a stretch of decile rock) the growth of muscular weakness in case of symptoms of inflammation of the musculature is more likely to occur in young people who suffer from myositis with "included". In the region, it is rare to develop so the titles of amiotrophic dermatomyositis in case of fired m'yaz, with which the main familiarity is even more of a type of urazhennya shkiri. In ailments with antisintetase syndrome, early signs of inflammation of the muscles can be the Raynaud's phenomenon, polyarthritis or polyarthritis and backwardness, surrounded by interstitial legenevim fibrosis.

Symptoms of mutilation when ignited

leading keynote firing of compounds - symmetric weakness of proximal groups of compounds of the upper and lower kintzivok, And soo m'yaz_v, how to take part in zginanní shia. It’s going to be difficult to make it difficult when you are low in style, when you get into transport, when you are wisely and appropriately. In case of symptoms of burning mucus, it is not grabbing, shkutilgau, ailments cannot be done without outside help and remove the head from the pillow. Filling the throat, larynx and stratohode to the point of dysphonia, making it difficult to cope, attacking a cough. Signs of lesion of the distal musculature are rare (10%), is manifested in the lesser world, less striking of the proximal musculature, and is a head rank in case of myositis with "included". In half of the ailments with symptoms of inflammation of the mucous membrane of the possible mialgia, for the ailment of the mucosa on palpation, the swelling of the mucosa, ale myase atrophy develop only in the ailment, as the therapy is trivially positive / polymorphic. myazova hypertrophy characteristic sign for muscular dystrophy and does not become susceptible to polymyositis / dermatomyositis.

Symptoms of the fight against shkiri with ignited compounds

Pathognomonic signs of dermatomyositis in case of inflammation of the mucus. Shkіrnі signs include erythematous (heliotropic) visip, which is localized on upper tables, Vilitsyah, krillas of the nose, in the area of ​​the nasolabial folds, in the zone of the "decollete" and on the upper part of the back, above the lytic and colonic, metacarpophalangeal and proximal midphalangeal slopes, on the hairy parts. Slightly flaky or flat erythematous peeling, localized over the nodules of the fingers of the hands, they called the name "Gottron's sign" in case of ignited muzzles. Characteristic signs that are not only susceptible to dermatomyositis, ale and polymyositis: dermatitis, peeling and rooting of shkiri dolon ("the hand of a mechanic of an overachiema"), hypertrophy, hypertrophy In case of capillaroscopy of sudin with fired mucous membranes of the periungual bed, it means enlargement and dilatation of capillary loops, often in case of perechial syndrome, and more in dermatomyositis. Photodermatitis і sverbіzh shkіri come first.

Symptoms of the defeat of slopes with ignited compounds

Symptoms of the defeat of the loam are not too fast for the development of myazovaya pathology in case of fired myazov. Most often, there are some small slopes of the brushes, interchangeable slopes, and more often - literal and colony slopes. The striking is bilaterally symmetric; rheumatoid arthritis As a rule, it has a long-term character, symptoms of inflammation of the mucus are quickly stopped when glucocorticoids are detected. However, the development of chronic deformable arthritis is described with the detachment of the slopes of the hands, or without erosive changes according to the data of the radiological dosage.

Symptoms of calcification with fired ointments

Signs of calcification are found on in different stages, Often with juvenile dermatomyositis. Calcification is localized in the middle of the tissue, near the fat fibers, often in the areas of microtraumatization over the licks and knee slopes, on the back surfaces of the fingers and the back.

Symptoms of a fight against a fire

With a provincial familiarity, I have ignited the muses in the form of an expiratory backbone, which can be tied to the lesions of diaphragmatic mutilations, the development of heart failure, intercurrent legacy infection, toxic lesions lungs tied with the reception of drugs, for example, methotrexate. The development of symptoms in a severe diffuse alveolitis is described. classy paintings inflamed mucus and manifested by unproductive coughing and progressive dysfunctional deficiency. Most often, there is more progress in the development of interstitial legendary fibrosis, and some ailments only appear with special circumstances. Aspirations of pneumonia develop in the most important cases.

Symptoms of a heart attack when the heart is ignited

Signs of heart disease with polymyositis / dermatomyositis in a large number of cases are asymptomatic. In some cases, with special conditions, symptoms of impaired rhythm and performance (tachycardia, arrhythmia) appear. Stagnant heart failure, tied to dilatation cardiomyopathy, grows up pretty much. Raynaud's phenomenon is often promoted in dermatomyositis, antisintetase syndrome, and in ailments with hypersensitivity polymyositis / dermatomyositis syndrome with systemic congestion of the resulting tissue.

Signs of the largest shipwrecks when ignited

Described is the infarction of the periungual bed, petechia, livedo reticularis (gillyaste babies on shkiri kintsivok and tuluba). Urazhennya nirok sposterіgayut rarely, I want a powerful development of proteinurії and navіt nephrotic syndrome. Myoglobinuria is common and can produce a spear.

Signs of fired m'yaziv

The main significance in the pathogenesis of polymyositis / dermatomyositis may be clinical reactions. In case of immunopathological pre-lesions of the mucus, they show infiltration of T-and B-lymphocytes and macrophages, which are found in the active state. With a large amount of T-cells, they may have a cytotoxic activity of myofibril. Between polymyositis and dermatomyositis, there are signs of singular immunopathological symptoms. In case of dermatomyositis, CE4 + -T-lymphocytes, macrophages and B-lymphocytes, and in case of polymyositis - cytotoxic CE8 + -T-lymphocytes are transfected. Pripuskayut scho at Find our dermatomyositis rozvivaєtsya humoral іmunnu vіdpovіd scho lead to complement aktivatsії scho vrazhaє vnutrіshnom'yazovі mіkrosudini, while polіmіozitі perevazhayut klіtinnі tsitotoksichnі reaktsії, oposeredkovuvanі SE8 + T lіmfotsitami, SSMSC sintezuyut tsitotoksichnі substantsії (perforin, granzіm). The pathogenetic significance of myosespecific autoantitils in case of inflammation of the mucous membranes has not been reported.

Reasons for the appearance of symptoms

The reasons for the blame are not precisely identified. For the role of infectious bureaucrats, one by one, I will order more often a cob to get sick in early spring (especially in those with juvenile dermatomyositis), which for an hour get sick with infectious diseases. About the fate of genetic schlichnosti to reduce the possibility of development of polymyositis / dermatomyositis in monozygous twins and blood relatives of ailments. The nosy of the AHs in the head complex of histosumity (HLA) is tied not to the fired words themselves, but to the singing immune disorders, in the first place to the hyperproducts of the autoantized autoantids.

The breadth of the meaning of the firing of the languages

The incidence of fumes in the population varies from 2 to 10 per 1 million population in Rik. There are two peaks of sickness in the past: in 5-15 years (juvenile dermatomyositis) and 40-60 years. Overvolving the child's diet (the number of sick women and children is 2-3: 1)

Diagnostics of the firing of compounds

Zagalny analiz blood in case of fired m'yaziv: characteristic signs of nemaє, improvement of SHOE sposterіgayut іdko, a leading rank in the development of systemic manifestations.

Biochemical analysis of blood in the diagnosis of ignited muses

Primary acceptance indicator of the reduction of skeletal muscles - CPK, improvement of which in polymyositis / dermatomyositis occurs more high sensitiveness and specificity in the context of other laboratory tests. Improvement of CPK in case of inflammation of myositis in the period of ailments in 95% of patients with polymyositis / dermatomyositis. The concentration of CPK can grow before the appearance of key signs of inflammation of the inflammation of the polymyositis / dermatomyositis, and the її level can decrease until the development of key polyphenia. In some ailments, KFK can be in between the norms, unimportant on the important part of the message for the data morphological dosage In general, the indicator is not correlated with the dynamism of clinical and morphological signs of activity. It is necessary for mothers to be on good health, for a normal CPK level can be promoted in ailments with important muscular atrophy at the lower stages of ailment, at the onset of dermatomyositis and with symptoms of puffy myositis.

The improvement of the CF-fraction of CPK is susceptible to signs of polymyositis / dermatomyositis for the appearance of myocardial necrosis. The improvement in transaminase activity is not specific for the improvement of skeletal muscles. In children with ailments with generalized weakness and isolation of transaminases, hepatitis is suspected.

Immunological diagnosis of ignited mucus

Prior to myosespecific AT, carry AT to aminoacylsintetases of transport RNA (antisintetase AT), in the first place AT to hystidyl tRNA synthetase (Jo-1). AT Jo-1 is found in half of the ailments with polymyositis / dermatomyositis, and only anti-syntetic AT in the region is rare (5%). Products of antisintetase AT are associated with the development of the so-called antisintetase syndrome, which is characterized by an ear, interstitial lesions, feverishness, symmetric arthritis, Raynaud's phenomenon, type of muscle lesions.

instrumental methods viznachennya firing m'yaziv

Electromyography for diagnostics of ignited myopathies is a sensitive, rather nonspecific method for diagnostics of ignited myopathies. Prior types simptomіv scho sposterіgaєtsya bіlsh nіzh 90% ailments at a proximal doslіdzhennі i paraspіnalnih m'yazіv, vіdnosyat Find our patologіchnoї spontannoї aktivnostі mіofіbril (potentsіali fіbrilyatsії, skladnі povtoryuvanі rozryadi іn i.) If i podraznennі in spokoї, korotkі nizkoamplіtudnі polіfazny potentsіali at skorochennі ... Is normal electrical activity with electromyography in a large number of cases, it allows the diagnosis of polymyositis / dermatomyositis to be diagnosed. Electromyography is a brown method of monitoring the efficiency of the process of firing mucus, especially when summarized results laboratory and clinical reports. However, the data of electromyography is disgusting. key manifestations myazovoi weakness. It is important that in case of steroid myopathy one can also (if it is less likely to turn) changes, as in case of active myositis.

Biopsies of symptoms in case of symptoms of fired up vikoristovuyutsya to diagnose diagnostics, to navigate with the presence of characteristic clinical, laboratory and instrumental signs of fired up mucous. Naybilsh informative biopsy of the tongue, involved in the pathological process, even without the atrophy.

Radiologic preliminaries for diagnostics of fouling of joints The radiologic symptoms of fouling of slopes are not typical. With roentgenologic dyslgenic lesions, there are often signs of basal pneumosclerosis and interstitial legenous fibrosis. With a more sensitive method, X-ray CT is inserted into a high-grade structure (RKT).

EKG in diagnostics is a sign of firing myaziv. For early detection of prognostically unpleasant disturbances to the rhythm and performance, additional monitoring of the EHC was carried out (according to Holter).

Symptoms of myositis myaziv

Miozit z "included" - signs of fired m'yaziv

Among ailments with symptoms of inflammation of the myositis, the frequency of myositis with "included" ranges from 15 to 28%. Prior to this, there are the following:

• abduction vik ailments (middle age is close to 60 years);
• more often the number of people (the number of people and women is 2: 1);
• even more general development of weakness and atrophy is not only in the proximal, but in the distal groups of musculoskeletons - a characteristic sign in case of fired mussels;
• asymmetric performance;
• normal or abnormally adjusted CPK activity;
• іdkіsna association with systemic incapacitation with the resultant fabrics and malignant new ideas;
• the visibility of myosespecific and іnshih autoantitils - a characteristic feature of the type of inflammation;
• resistance to glucocorticoids and other methods of pharmacotherapy (signs are especially characteristic).

A characteristic morphological feature is the amyloidogenic "oxidation" of the vascuoli, the great internal nucleus and intracytoplasmic "inclusion" (in case of microscopic microscopy) and microtubular elements, which appear during microscopy. singing diagnostic value low elektromyography, when there are symptoms of myopathic and neuropathic signs.

Symptoms of myositis associated with systemic diseases of the resulting tissue

Proximal muscle weakness is a part of the clinical sign of systemic inflammation of the muscle, and signs of other ailments of the whole group appear in about 20% of patients with polymyositis / dermatomyositis. Symptoms of myopathy can prevail in clinical pictures in case of systemic SJS, VKV, rheumatoid arthritis, Sjogren's syndrome, systemic vasculitis. In general, the frequency of Raynaud's phenomenon and polyarthritis, even higher titers of antinuclear factors (ale the appearance of myosespecific autoantibodies), a good response to glucocorticoids, is characteristic of the temporal syndrome. Patients with polymyositis in the case of SSD in syrovatki have AT PM / Scl. The increase in CPK activity in case of transient myositis syndromes in cases of inflammation of the mucous membranes is manifested in the same world, as in case of idiopathic polymyositis / dermatomyositis, and histological changes in cases of quiet diseases

Signs of myositis m'yaziv

Signs of myositis in case of malignant new solutions in case of firing of mucous membranes

Symptoms of myositis, associated with puffins (puffy, or paraneoplastic, myositis), store approximately 20% of all types of ignition myopathies. On the background evil new creatures dermatomyositis, less polymyositis develop more often. The number of sick people and the number of sick women becomes 1: 1. Puffs can develop before the signs of myopathy appear, at the same time, for them. The frequency of malignant neoplasms in polymyositis / dermatomyositis is 12 times higher than in the outlying population.

From the key point of development in patients with vasculitis (aka school necrosis), or amyotrophic dermatomyositis, malignant myositis, and the formation of lung fibrosis, manifesting myositis specific systemic Localization and type of fluff in case of inflammation of the mucous membranes, which associate with myositis, are associated with the rise in frequency in the type of disease groups. Protein swelling is often associated with ovarian and nasopharyngeal cancer. When a patient is suspected of puffy myositis, it is necessary to carry out additional diagnostics of the condition, which includes the appointment of prostate-specific Ar (in cholovikiv), CA-125 (Ar of puffiness).