What are the most common muscle diseases? Muscular disorders. Muscles: weakness (myopathy), muscle wasting, myasthenia gravis

Inflammation of the muscles - diseases, the main symptoms of which are muscle weakness associated with inflammation of the striated muscles. Muscle inflammation includes idiopathic inflammatory myopathies, myopathies associated with infection, and myopathies associated with exposure to drugs and toxins. Among them, the most important are signs of polymyositis and dermatomyositis. In this article, we will look at the symptoms of muscle inflammation and the main signs of muscle inflammation in humans. In addition, we will talk about the diagnosis of inflamed muscles.

Symptoms of muscle inflammation

In the debut of symptoms of muscle inflammation, most patients note signs of malaise, general weakness, skin lesions (with dermatomyositis). Subsequently, gradually (over several weeks), symptoms of a progressive increase in weakness in the proximal muscle groups join the inflammation of the muscles. In some patients with signs of muscle inflammation (children and persons young age) observe an acute onset, often combined with pronounced constitutional signs (fever, weight loss, etc.) and myalgia.

A very slow (over several years) increase in muscle weakness with symptoms of muscle inflammation is observed more often in elderly patients suffering from inclusion myositis. It is extremely rare that the so-called amyotrophic dermatomyositis develops with inflamed muscles, in which the main symptom for a very long time is a typical skin lesion. In patients with antisynthetase syndrome early signs muscle inflammation may be Raynaud's phenomenon, polyarthralgia or polyarthritis, and dyspnea due to interstitial pulmonary fibrosis.

Symptoms of muscle damage during inflammation

The leading clinical sign of muscle inflammation is symmetrical weakness of the proximal muscle groups of the upper and lower extremities, as well as the muscles involved in neck flexion. This leads to difficulty getting up from a low chair, getting into transport, washing and combing. The gait with symptoms of muscle inflammation becomes clumsy, waddling, patients cannot rise without assistance and tear their heads off the pillow. Inflammation of the muscles of the pharynx, larynx and esophagus leads to dysphonia, difficulty swallowing, coughing fits. Signs of damage to the distal muscles are rare (10%), less pronounced than the defeat of the proximal muscles, and is detected mainly in myositis with "inclusions". In half of the patients with symptoms of muscle inflammation, myalgia or muscle tenderness on palpation, muscle edema are possible, but muscle atrophy develops only in patients suffering from polymyositis/dermatomyositis for a long time, especially in the absence of adequate therapy. Muscular hypertrophy is a characteristic feature of muscular dystrophies and is not observed in polymyositis/dermatomyositis.

Symptoms of skin lesions with inflammation of the muscles

Pathognomonic sign of dermatomyositis with inflammation of the muscles. Skin signs include an erythematous (heliotrope) rash localized on the upper eyelids, cheekbones, wings of the nose, in the area of ​​the nasolabial fold, in the "décolleté" area and on the upper back, over the elbows and knees, metacarpophalangeal and proximal interphalangeal joints, on the scalp parts of the head. Slightly raised or flat erythematous scaly rashes, localized over the knuckles of the fingers, are called "Gottron's sign" with inflammation of the muscles. Characteristic skin signs observed not only in dermatomyositis, but also in polymyositis: redness, peeling and cracking of the skin of the palms ("mechanic or craftsman's hand"), cuticle hypertrophy, periungual erythema, telangiectasias. With capillaroscopy of vessels with inflammation of the muscles of the periungual bed, expansion and dilatation of capillary loops are noted, more often with cross syndrome, less often with dermatomyositis. Photodermatitis and pruritus are less common.

Symptoms of joint damage with muscle inflammation

Symptoms of joint damage often precede the development of muscle pathology with muscle inflammation. Most often, small joints of the hands, wrist joints are involved, less often - elbow and knee joints. The lesion is bilaterally symmetrical, reminiscent of that in rheumatoid arthritis, as a rule, is transient, the symptoms of muscle inflammation are quickly stopped when glucocorticoids are prescribed. However, the development of chronic deforming arthritis with subluxations of the joints of the hands, but without erosive changes according to X-ray examination, has been described.

Symptoms of calcification in muscle inflammation

Signs of calcification appear on late stages, more often with juvenile dermatomyositis. Calcifications are localized subcutaneously or in connective tissue around muscle fibers, often in areas of microtrauma over the elbow and knee joints, on the flexor surfaces of the fingers and buttocks.

Symptoms of lung damage with muscle inflammation

The leading clinical sign of muscle inflammation is expiratory dyspnea, which may be associated with damage to the diaphragmatic muscles, the development of heart failure, intercurrent pulmonary infection, and toxic lung damage associated with taking certain drugs, such as methotrexate. The development of symptoms of acute diffuse alveolitis, which comes to the fore in clinical picture inflammation of the muscles and manifested by an unproductive cough and rapidly progressive respiratory failure. More often, slow progression of interstitial pulmonary fibrosis is observed, in some patients it is detected only during a special examination. In the most severe cases, aspiration pneumonia develops.

Symptoms of heart damage with inflammation of the muscles

Signs of heart damage in polymyositis / dermatomyositis in most cases are asymptomatic. Sometimes, during a special examination, symptoms of rhythm and conduction disturbances (tachycardia, arrhythmia) are revealed. Congestive heart failure associated with dilated cardiomyopathy is rare. Raynaud's phenomenon is more often observed in dermatomyositis, antisynthetase syndrome, and in patients with polymyositis/dermatomyositis cross syndrome with systemic connective tissue diseases.

Signs of others vascular disorders with muscle inflammation

Infarctions of the periungual bed, petechiae, livedo reticularis (branched pattern on the skin of the limbs and trunk) are described. Kidney damage is rarely observed, although proteinuria and even nephrotic syndrome may develop. Severe myoglobinuria can lead to cirrhosis.

Signs of muscle inflammation

Cellular immune responses are of primary importance in the pathogenesis of polymyositis/dermatomyositis. Immunopathological examination of the affected muscle reveals infiltration with T- and B-lymphocytes and macrophages that are in an activated state. At the same time, T cells have cytotoxic activity against myofibrils. There are signs of certain immunopathological differences between polymyositis and dermatomyositis. In dermatomyositis, CD4+-T-lymphocytes, macrophages, and B-lymphocytes predominate in the muscle infiltrate, while in polymyositis, cytotoxic CD8+-T-lymphocytes predominate. It is assumed that with signs of dermatomyositis, a humoral immune response develops, leading to complement activation, affecting intramuscular microvessels, and in polymyositis, cellular cytotoxic reactions mediated by CD8+-T-lymphocytes that synthesize cytotoxic substances (perforin, granzyme) predominate. The pathogenetic significance of myositis-specific autoantibodies in muscle inflammation has not been proven.

Causes of symptoms of muscle inflammation

The causes of muscle inflammation are not exactly understood. The role of infectious factors is indirectly indicated by a more frequent onset of the disease in winter and early spring (especially in patients with juvenile dermatomyositis), which coincides in time with epidemics of infections. The participation of a genetic predisposition is evidenced by the possibility of developing polymyositis / dermatomyositis in monozygotic twins and blood relatives of patients. The carriage of some antigens of the major histocompatibility complex (HLA) is more closely associated not with muscle inflammation itself, but with certain immune disorders, primarily with hyperproduction of myositis-specific autoantibodies.

Prevalence of signs of muscle inflammation

The incidence of muscle inflammation in the population ranges from 2 to 10 cases per 1 million population per year. Depending on age, two peaks of incidence are observed: at 5-15 years (juvenile dermatomyositis) and 40-60 years. The predominant sex is female (the ratio of the number of sick women and men is 2-3: 1)

Diagnosis of muscle inflammation

Complete blood count for muscle inflammation: characteristic features no, an increase in ESR is rarely observed, mainly with the development of systemic manifestations.

Biochemical analyzes blood in the diagnosis of inflamed muscles

The generally accepted measure of skeletal muscle damage is CPK, which increases in polymyositis/dermatomyositis with higher sensitivity and specificity than other laboratory tests. An increase in CPK during muscle inflammation at various periods of the disease occurs in 95% of patients with polymyositis / dermatomyositis. The concentration of CPK may increase until the appearance clinical signs inflammation of the muscles exacerbation of polymyositis / dermatomyositis, and its level may decrease until the development of clinical improvement. Sometimes in patients, the level of CPK may be within the normal range, despite severe muscle damage according to the morphological study, in this case, the indicator does not correlate with the dynamics of clinical and morphological features activity. It must be borne in mind that normal level CK can be observed in patients with severe muscle atrophy in the late stages of the disease, in the debut of dermatomyositis and with symptoms of tumor myositis.

An increase in the CPK MB fraction is observed with signs of polymyositis / dermatomyositis in the absence of myocardial necrosis. An increase in transaminase activity is not specific for skeletal muscle damage. In some patients with generalized weakness, an isolated increase in transaminases raises the suspicion of hepatitis.

Immunological diagnosis of inflamed muscles

Myositis-specific ATs include ATs to aminoacyl synthetases of transfer RNA (antisynthetase ATs), primarily ATs to histidyl tRNA synthetase (Jo-1). AT Jo-1 is detected in half of patients with polymyositis/dermatomyositis, while other antisynthetase ATs are extremely rare (5%). The production of antisynthetase ATs is associated with the development of the so-called antisynthetase syndrome, characterized by an acute onset, interstitial lung disease, fever, symmetrical arthritis, Raynaud's phenomenon, and "mechanic's hand" skin lesions during muscle inflammation.

Instrumental methods for determining muscle inflammation

Electromyography for the diagnosis of muscle inflammation is a sensitive but non-specific method for diagnosing inflammatory myopathies. Typical symptoms observed in more than 90% of patients in the study of proximal and paraspinal muscles include signs of pathological spontaneous activity of myofibrils (fibrillation potentials, complex repetitive discharges, etc.) during stimulation and at rest, short low-amplitude polyphasic potentials during contraction. Normal electrical activity on electromyography will rule out the diagnosis of polymyositis/dermatomyositis in most cases. Electromyography - useful method monitoring the effectiveness of the treatment of muscle inflammation, especially with questionable results of laboratory and clinical studies. However, electromyography data do not correlate well with clinical manifestations of muscle weakness. It is important that the same (though less pronounced) changes are observed in steroid myopathy as in active myositis.

Muscle biopsy with symptoms of inflammation is used to confirm the diagnosis, even in the presence of characteristic clinical, laboratory and instrumental signs of muscle inflammation. The most informative biopsy of the muscle involved in the pathological process, but without severe atrophy.

X-ray studies for the diagnosis of inflammation of the muscles X-ray symptoms of inflammation of the joints are not typical. At x-ray examination lungs often show signs of basal pneumosclerosis and interstitial pulmonary fibrosis. X-ray CT is considered a more sensitive method with high resolution(RKT).

ECG in the diagnosis of signs of muscle inflammation. For early detection of prognostically unfavorable rhythm and conduction disturbances, it is advisable to conduct daily ECG monitoring (according to Holter).

neuromuscular disorders) N. - m. R. are pathological processes, in which motor neurons are affected, incl. axons and innervation of muscle fibers by motor neurons. Mn. from N.-m. R. are hereditarily caused though at nek-ry from them the genetic communication is not found. In genetic transmission, the carrier is usually the mother. Initial symptoms N.-m. R. - the appearance of asymmetric muscle weakness with intact sensory. As the disease progresses, symmetry loss muscle mass becomes apparent, there is a similar pattern of muscular atrophy on each side of the body. N.-m. R. and diseases are most easily conceptualized according to the level and degree of involvement of motor neurons. N.-m. r., due to the involvement of upper motor neurons, can manifest itself in progressive spastic bulbar palsy with bilateral intracerebral damage to the corticobulbar and corticospinal tracts. N.-m. R. may be accompanied by such demyelinating pathological processes as multiple sclerosis (PC), amyotrophic lateral sclerosis (ABS), as well as cerebrovascular disorders. Involvement of the diencephalon can cause impairments to speech, swallowing, and, in some cases, emotional control. Death usually occurs within two to three years as a result of intercurrent diseases. Often, ABS causes damage to both the upper and lower motor neuron pathways. The prevalence in women is usually lower than in men; the peak incidence is in average age(35-55 years). The first symptom is often a loss of muscle mass in the hand. In the future, this process extends to all limbs with the addition of spastic manifestations. Death occurs within 1 to 5 years of the course of the disease. The etiology of ABS is unknown. The pathology of the lower motor neurons includes Werdnig-Hoffmann's disease and Oppenheim's disease; progressive neuropathic muscular atrophy can also be classified in this category. These diseases are characteristic childhood, with the exception of Oppenheim's disease, which occurs preim. in teenagers. Death occurs within one to two years; normal life expectancy can be observed with Dejerine-Sottas disease, which can be attributed to this group. Dr. myasthenia gravis and Duchenne muscular dystrophy are quite common diseases. Myasthenia is due to a deficiency of acetylcholine due to damage to synaptic transmission. The onset of the disease usually occurs in the third decade of life. To early symptoms include ptosis and disorders of swallowing, breathing and speech with the involvement of peripheral muscles. Muscular dystrophy in Duchenne disease is inherited by an X-linked recessive gene. The carrier of the gene is a woman, the disease manifests itself in males. Muscle weakness develops no earlier than the third, fourth or even fifth year of life. Appeared dystrophic disorders progress until the onset lethal outcome at the end of the second decade of life. N.'s treatment - m. R. aimed at preventing infections and controlling spasticity. Psych. interventions include counseling and strengthening patient support mechanisms. See also Mental and Behavioral Disorders in CNS Lesions, Multiple Sclerosis, Psychophysiology J. Hind

Neuromuscular diseases (NMD) are the most numerous group of hereditary diseases, which are based on genetically determined damage to the anterior horns of the spinal cord, peripheral nerves and skeletal muscles.

Neuromuscular diseases include:

1) progressive muscular dystrophies(primary myopathies);

2) spinal and neural amyotrophies (secondary myopathies);

3) congenital non-progressive myopathies;

4) neuromuscular diseases with myotonic syndrome;

5) paroxysmal myoplegia;

6) myasthenia gravis.

15.2. Progressive muscular dystrophies (primary myopathies)

Progressive muscular dystrophies (PMD), or primary myopathies, are characterized by degenerative changes in muscle tissue.

Pathological changes PMD is characterized by thinning of the muscles, replacing them with adipose and connective tissue. The foci of focal necrosis are revealed in the sarcoplasm, the nuclei of the muscle fibers are arranged in chains, the muscle fibers lose their transverse striation.

Pathogenesis issues remain unresolved to date. Myopathy is based on a defect in the membrane of muscle cells. Great hopes are placed on molecular genetics.

Various forms of myopathy differ in the type of inheritance, the timing of the onset of the process, the nature and speed of its course, and the topography of muscle atrophy.

Myopathies are clinically characterized by muscle weakness and atrophy. There are various forms of PMD.

15.2.1. Duchenne myodystrophy (pseudohypertrophic form of PMD)

It occurs most frequently of all PMD (30:100,000). This form is characterized by an early onset (2-5 years) and a malignant course, predominantly boys are ill. Duchenne myopathy is inherited in an X-linked recessive manner. The pathological gene is localized in the short arm of the chromosome (X, or chromosome 21).

The mutation of the gene is quite high, which explains the significant frequency of sporadic cases. Mutation (most often deletion) of the gene leads to the absence of dystrophin in the membrane of muscle cells, which leads to structural changes in the sarcolemma. This promotes the release of calcium and leads to the death of myofibrils.

One of the first signs of the disease is the compaction of the calf muscles and a gradual increase in their volume due to pseudohypertrophy. The process is ascending. The advanced stage of the disease is characterized by a "duck" gait, the patient walks, waddling from side to side, which is mainly due to weakness of the gluteal muscles.

As a result, there is a tilt of the pelvis towards the non-supporting leg (Trendelenburg phenomenon) and a compensatory tilt of the torso in the opposite direction (Duchenne phenomenon). When walking, the side of the slope changes all the time. This can be checked in the Trendelenburg position by asking the patient to raise one leg, bending it at a right angle at the knee and hip joint: the pelvis on the side of the raised leg drops (and does not rise as normal) due to weakness of the gluteus medius muscle of the supporting leg.

With Duchenne myopathy, pronounced lordosis, pterygoid scapulae, typical muscle contractures, knee jerks fall out early. It is often possible to detect changes in the skeletal system (deformity of the feet, chest, spine, diffuse osteoporosis). There may be a decrease in intelligence and various endocrine disorders (adiposogenital syndrome, Itsenko-Cushing's syndrome). By the age of 14-15, patients are usually already completely immobilized; in the terminal stage, weakness can spread to the muscles of the face, pharynx, and diaphragm. They die most often in the 3rd decade of life from cardiomyopathy or the addition of intercurrent infections.

A distinctive feature of Duchenne myopathy is a sharp increase in a specific muscle enzyme - creatine phosphokinase (CPK) by tens and hundreds of times, as well as an increase in myoglobin by 6-8 times.

For medical genetic counseling, it is important to establish heterozygous carriage. In 70% of heterozygotes, subclinical and clinical signs of muscle pathology are determined: compaction and increase calf muscles, rapid muscle fatigue during exercise, changes in muscle biopsy specimens and biopotentials according to EMG.

muscles(musculi; synonymous with muscles). Functionally, involuntary and voluntary muscles are distinguished. Involuntary muscles are formed by smooth (non-striated) muscle tissue. It forms the muscular membranes of hollow organs, the walls of blood and lymphatic vessels. The structural unit of smooth muscle tissue is the myocyte, in the cytoplasm of which there are thin fibers - myofibrils. Smooth muscle tissue provides peristalsis of hollow organs, tone of blood and lymphatic vessels.

Voluntary muscles are formed by striated (striated) muscle tissue, which is the active part of the motor apparatus and ensures the movement of the body in space. A special place is occupied by the myocardium, consisting of striated muscle tissue, but contracting involuntarily (see Fig. Heart ). The structural and functional unit of skeletal muscle tissue is the striated muscle fiber, which is a multinuclear symplastic formation ( rice. one ). The length of muscle fibers ranges from a few millimeters to 10-12 cm, diameter - from 12 to 100 micron. The muscle fiber has a cytoplasm called sarcoplasm; externally surrounded by a thin shell - the sarcolemma. The specific contractile apparatus of the muscle fiber is made up of myofibrils. The transverse striation of the muscle fiber is determined special structure myofibrils, in which sections with different physicochemical and optical properties alternate - the so-called anisotropic and isotropic disks. The different optical properties of these discs are due to different combination they contain thin and thick myofilaments - the thinnest protein filaments that make up myofibrils. Thin myofilaments are built from the protein actin, and thick ones are built from myosin. When these proteins interact, the myofibril shortens, and as a result of this process, which occurs synchronously in almost all myofibrils, muscle fiber contraction occurs.

Muscle fibers contain a specific protein myoglobin, which accumulates oxygen that enters the muscles during breathing and releases it as needed during muscle contraction.

Muscle fibers are combined into bundles of various orders. The loose connective tissue within the muscle bundles is called the endomysium. Between themselves, the bundles of muscle fibers are connected by a loose fibrous connective tissue - the inner perimysium. Outside, M. are covered with a denser connective tissue - the outer perimysium.

In the places of attachment of skeletal M. to the bones, they often pass into tendons, especially well expressed in long M. All tendons are built from dense connective tissue and are highly resistant to stretching. Muscle fibers are connected to tendons through collagen fibers, and endomysium and perimysium fibers are directly woven into the tendon tissue.

dislocations) using a standard goniometer. Muscle strength is determined using the Coplen dynamometer, as well as during active movements in the joint with resistance provided by the examiner's hand. Electromyography allows you to more objectively judge the functional state of the muscles.

Pathology

Pathology includes malformations, injuries, inflammatory and degenerative changes, tumors.

Malformations muscles are found in almost all areas of the body, but are more commonly seen on the upper extremities. M.'s anomalies can be divided into three groups: 1) total absence of separate muscles; 2) the appearance of additional muscles; 3) various changes in shape, including the absence or underdevelopment of any part of the M. or the presence of additional tendons and heads, splitting of the M., etc. Congenital underdevelopment of the sternocleidomastoid muscle is more common, leading to torticollis, as well as a defect in the development of the diaphragm, which leads to the formation of diaphragmatic hernias (see. Diaphragm ). Treatment of these defects is usually surgical.

Damage divided into closed and open. Carry to the closed damages of M. bruises, complete and incomplete ruptures of M. and their tendons, the formation of muscle hernias. With a bruise and a partial rupture in the thickness of M., a painful compaction without clear boundaries is determined. As a result of damage to small blood vessels, subcutaneous and intermuscular develops, the contractility of M is disturbed. As edema increases, contraction and shortening of M occurs. Active movements become sharply painful, which leads to pain contracture, or antalgic posture; passive movements are preserved. The outcome of an extensive intermuscular hematoma may be cicatricial replacement, or ossification of the damaged area of ​​M. Closed (subcutaneous) M. ruptures occur with a sharp muscle tension and in some cases with direct trauma. The rupture occurs more often at the site of M.'s transition to the tendon, which is due to dystrophic processes. At the moment of injury, patients feel a sudden click, accompanied by sharp pain and loss of active movements. In cases of complete rupture of M., it is possible to determine the retraction at the site of damage and the bulging of the contracted edges of the muscle.

Dystrophic diseases muscle tissue are progressive in nature and often hereditary (see. Myopathies, myasthenia gravis, Myatonia ) or autoimmune ( dermatomyositis etc.) nature.

Tumors can occur in any organ where there are muscle elements. They develop from both striated and smooth muscles. They can be benign and malignant.

benign tumors. Leiomyoma occurs more often in people aged 30-50 years. It occurs in all organs where there are smooth muscle fibers (in the uterus, gastrointestinal tract, bladder, prostate, skin, etc.). Leiomyomas have a rounded shape, densely elastic consistency, clearly delimited from the surrounding tissues. Often, leiomyomas are multiple.

Leiomyomas of the skin - see. Leather, tumors. Rhabdomyoma is more common in children. Usually located in the thickness of the muscles and in the area of ​​​​large joints. The tumor is a node, sometimes reaching 10-15 cm in diameter, densely elastic consistency, mobile and well delimited from the surrounding tissues, has a pronounced capsule. Grows slowly.

The diagnosis of benign tumors of muscle tissue is established on the basis of clinical data, the results of morphological and additional methods research - ultrasound and computed tomography, angiography. Due to the poor clinical picture, it is difficult to differentiate between benign and malignant tumors. Rhabdomyomas of the extremities are differentiated from myogenic mi and synovioma. Myomas coming from the walls internal organs, - with other neoplasms of these organs.

Surgical treatment. Radical excision of the tumor provides a cure. With skin leiomyomas, electroexcision or cryodestruction can be applied. The prognosis is favorable.

Malignant tumors. Leiomyosarcoma is localized, as well as leiomyoma, most often in the uterus, less often in the organs of the gastrointestinal tract, bladder. It is rare in the soft tissues of the limbs and trunk. It accounts for 4% of all soft tissue sarcomas. The tumor has the form of a node of soft elastic consistency, it can reach 15-20 cm in diameter. It proceeds extremely malignantly. Multiple early metastases to the lungs are characteristic. Metastases in regional lymph nodes are found in approximately 2% of patients.

Rhabdomyosarcomas are relatively rare - they account for 4.1%, ranking fifth among malignant soft tissue tumors. They are observed mainly in mature and old age, in men - 2 times more often than in women. They are localized mainly on the limbs, in the head and neck, in the small pelvis. Polymorphic rhabdomyosarcoma occurs mainly in the elderly and is localized on the extremities; alveolar rhabdomyosarcoma - in adolescents and young people,

found in any part of the body; embryonic rhabdomyosarcoma - in newborns and children younger age, its typical localization in the pelvic area. Rhabdomyosarcomas can also develop outside of muscle tissue (in the retroperitoneal tissue, mediastinum, nasopharynx, etc.). The main symptom of rhabdomyosarcoma is the presence of a single (sometimes multiple) fast-growing node in the thickness of the muscles. Pain and dysfunction of the limb, as a rule, does not happen. The neoplasm is prone to skin germination and ulceration. Early hematogenous metastasis to the lungs is typical. Metastases in regional lymph nodes occur in 6-8% of cases. In the early stages, diagnosis is difficult. For the correct interpretation of the nature of the lesion, one should take into account the typical localization in the thickness of the muscles, fast growth tumors, skin lesions and ulceration. The final diagnosis is established using ultrasound and computed tomography, angiography.

Surgical treatment - wide excision of the tumor. With recurrence of the tumor on the extremities, amputation (exarticulation) is indicated. Radiation therapy is used for palliative purposes in non-removable tumors or in the postoperative period. Rhabdomyosarcomas are usually resistant to radiation exposure. Chemotherapy is used to treat disseminated forms and is included in the combination therapy plan for the primary tumor. The most active drug is the antitumor antibiotic adriamycin. The prognosis is unfavorable.

Operations

Operations on M. are carried out in order to remove the pathological focus, tumor, opening a, emptying the hematoma or for suturing the gap, plasty with contracture, etc. M.'s intersection - myotomy or its complete removal is used to eliminate contractures caused by irreversible shortening of the M., in where tenotomy cannot be performed. In some cases, the muscle is used as a plastic material for closing tissue defects, for example, for filling bone cavities in osteomyelitis, etc.

For suturing M. use, as a rule, absorbable suture material. Seams are applied to M. without great tension, so as not to cause a violation of their nutrition (see. Surgical sutures ).

Bibliography: Human Anatomy, ed. M.R. Sapina, vol. 1, p. 162, M., 1986; Daniel-Beck K.V. and Kolobyakov A.A. Malignant tumors of skin and soft tissues, M., 1979, bibliogr.; Clinical Oncology, ed. N.N. Blokhin and B.E. Peterson, vol. 1. p. 385, M., 1979; Kovanov V.V. and Travin A.A. Surgical anatomy of human limbs, M., 1983: Movshovich I.A. Operative orthopedics, M., 1983, Guidelines for the pathoanatomical diagnosis of human tumors, ed. ON THE. Kraevsky and others, p. 43, M., 1982; Trubnikova F. Traumatology and orthopedics, Kyiv, 1986; Ham A. and Cormac L. Histology, trans. from English, vol. 3, p. 241, M., 1983.

A symptom of a neuromuscular disease can be muscle spasms or, conversely, their sharp relaxation.

Hereditary neuromuscular diseases combine a whole group of diseases, common characteristic which is "recorded" in the genome violations in the work of the neuromuscular apparatus. Muscle atrophy, their excessive contraction or, on the contrary, relaxation - all this can be a sign of inherited diseases.

Types of hereditary neuromuscular diseases

Hereditary neuromuscular diseases include many different disorders, which are divided into several groups:

• primary progressive muscular dystrophies or myopathies.
• secondary progressive muscular dystrophies.
• congenital non-progressive myopathies
• myotonia
• hereditary paroxysmal myoplegia.

Primary progressive muscular dystrophies or myopathies

Myopathies include a group of diseases that are manifested by muscle weakness and muscle dystrophy, which increase over time. In diseases of this group, it occurs in muscle cells, which leads to atrophy of muscle fibers.

With myopathies, the muscles of the limbs, pelvis, hips, shoulders, torso can be affected, depending on the specific type of disease. The most common are: the youthful form of Erba-Roth, the shoulder-scapular-facial form of Landuzy-Dejerine, the pseudo-hypertrophic form of Duchenne.

In myopathies, muscle strength and muscle tone decrease symmetrically. Pseudohypertrophy often develops - an increase in muscles due to the growth of adipose and connective tissue. Infections, intoxication, stress can accelerate the course of the disease.

Primary progressive muscular dystrophies with an active course, they can lead to disability and complete immobilization.

Secondary progressive muscular dystrophies

With the development of these diseases, the work of peripheral nerves is primarily disrupted. The innervation of the muscles is disturbed, which leads to the occurrence of muscular dystrophies.

Secondary progressive muscular dystrophies include three varieties: congenital, early childhood and late. This classification is based on the time of appearance of the first signs of the disease. Depending on the form of the disease, it proceeds more or less aggressively. Depending on this, people suffering from this kind of genetic abnormalities live up to 9-30 years.

Non-progressive myopathies

Myotonia congenita

Myotonia congenita(Thomsen's disease) - rare hereditary disease, characterized by prolonged tonic muscle spasms that occur after the initial voluntary movements.

etiologists

This group includes diseases also associated with muscle dystrophy. Problems appear immediately at birth. At the same time, a “sluggish child syndrome” is detected - a condition when muscle lethargy, motor inhibition and a lag in the motor development of the child are observed. But non-progressive myopathies differ from other types of hereditary neuromuscular diseases in that the condition does not worsen over time and the disease does not progress.

Myotonia

This group of diseases is characterized by muscle spasms at the beginning of movement. At the beginning of the action, the muscle contracts and cannot relax for 5-30 seconds. After that, gradual relaxation still occurs and the second movement is a little easier to do. But after the rest, everything repeats again.

With this disease, spasm can involve the muscles of the face, trunk, limbs.

Hereditary myotonias include dystrophic myotonia, Thomsen's congenital myotonia, atrophic myotonia, paramyotonia and other diseases.

Enough in a simple way define myotonia is a symptom of "fist". If you suspect myotonia, the doctor asks you to quickly open your fist. A person suffering from this genetic disease cannot do this quickly and effortlessly. As a test, you can also offer to quickly open your jaws, get up from a chair, or open your squinted eye.

People suffering from myotonia often have an athletic build. This is due to the fact that in these diseases certain muscle groups are hypertrophied. Under the influence of cold and muscle spasm usually increases.

As a rule, a person who "has" myotonia in his genome can coexist with it. Such people just need to choose the right profession, in which there is no need for sudden movements. But there are varieties of myotonia, in which there is a risk of disability or sudden death.

Myoplegia

Another type of hereditary neuromuscular disease is myoplegia. In this case salient feature diseases are bouts of muscle weakness. There are several forms of paroxysmal myoplegia: hypokalemic, hyperkalemic and normokalemic.

With this disease in muscle cells, the polarization of membranes is disrupted and the electrolytic properties of muscles change.

At the time of the attack, there is usually a sharp weakness in the muscles of the arms of the legs or torso. Sometimes there may be weakness of the pharynx, larynx, respiratory muscles, which can cause death.

All forms of hereditary neuromuscular diseases are difficult to treat. But modern medicine continues to look for ways to influence genetic diseases. And in the near future, perhaps, will be developed effective ways impact on similar genetic diseases.