Hyena disease Bar infection treatment. Features of the treatment of hyena-barre syndrome

Hyien - Barre syndrome is a disease in which the shell of nerve fibers (myeline) is destroyed, which leads to disruption of movements, sensitivity disorders. It is usually developing after a while after the suffered infection.

Melin is a special shell of nerve fibers, which is necessary for carrying nerve impulses. In the Hyien - Barre syndrome, it is destroyed by its own immune system of the body. Normally, the immune system detects and destroys alien objects (for example, causative agents of infectious diseases), but in some cases it begins to struggle with native cells. As a result of the damage to the myelin shell, there is a manifestation of the disease: a decrease in force in the muscles, tingling in the limbs and others. Most patients require hospitalization.

The timely started treatment makes it possible to achieve complete recovery, although some people have weakness in the muscles, a feeling of numbness.

Russian synonyms

Acute inflammatory demyelinating polyradiculoneuropathy, acute polyradiculitis.

Synonymsenglish

GUILLAIN-BARRE SYNDROME, ACUTE IDIOPATHIC POLYNEURITIS, ACUTE INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY.

Symptoms

• Reducing power in the muscles, tingling - first in the legs, then in the overlying bodies
• Intensive pain in the field of shoulder belt, back, hips
• Violation of chewing, swallowing, pronunciation of sounds, facial facials as a result of a decrease in muscle strength performing data
• Cheating or slowing the rhythm of heart abbreviations
• Raising or decline arterial pressure
• Respiratory disorders, at the increasing of which artificial ventilation of the lungs may be required (conducted by a special apparatus when independent breathing is inefficient)
• Urinary delay
• Constipation

General information about the disease

Hyien - Barre syndrome - a disease in which the Mielinic shell of nerves is destroyed, as a result of which the nerve impulses are disturbed and the power in the muscles is reduced.

The exact causes of the disease are unknown. In most cases, symptoms appear in 1-3 weeks after transferred acute infection Respiratory system, gastrointestinal infections.

These infections may be caused by these and other pathogens:

• campylobacteriums are contained in the meat of infected birds and cause gastrointestinal infection when a person is inhabited into the body;
• flu virus;
• epstein - Barr virus (causative agent of infectious mononucleosis);
• mycoplasma can cause inflammation of the lungs in people infected with the immunodeficiency virus (HIV).

Also, a starting factor in the development of the disease can be vaccinations, operational interventions.

An important role is played by autoimmune mechanisms. The immune system is struggling with alien objects that fall into the body. In response to infection, special protein particles are produced - antibodies. They detect and neutralize various infections, viruses. According to the researchers, under the hyeien - Barre syndrome, the antibodies not only destroy the causative agents of infection, but also damage the shell of nerve cells, this is possible due to the similarity in the molecular structure of these objects.

Myelin shell covers nerve fibers and provides a certain rate of nerve pulses between the brain and various structures organism. Violation of the passage of nerve pulses to muscle fibers leads to a decrease in force in the muscles. The nerve fibers of the autonomous nervous system are also affected (which regulates the activity internal organs). At the same time, the work of the cardiovascular system can be disturbed, changing the rhythm of the heart, blood pressure, etc.

With severe forms of the disease, the following complications are possible.

• Breathing disorder. Arises as a result of weakness or paralysis (the complete ability to move the respiratory muscles and threatens the patient's life. In cases where independent breathing is inefficient, artificial ventilation of the lungs is carried out (with the help of a special apparatus).
• Violations in the work of the cardiovascular system.
• Long immobility. Increases the risk of thromboembolism (blockage of blood vessels with blood circulation, resulting in circulatory disorders).
• Prolesidery - dead skin, underlying soft fabrics, which arise with prolonged immobility of patients due to blood supply disorders.

The disease is developing for several weeks, and for the restoration of lost functions may require several months. In most cases, complete recovery occurs.

Who is in the risk group?

• Faces of young and elderly.
• Patients with some kinds of infectious diseases.
• Moved surgical interventions.

Diagnostics

The diagnosis of Guien - Barre syndrome is quite complex, since there are no specific studies that allow it to reveal it. At the same time, the diagnosis is based on the analysis of clinical manifestations, studying the history of the disease, analyzes to eliminate other diseases of the nervous system.

Laboratory diagnostics is of great importance.

• Protein common in Likvore. The spinal fluid (liquor) is washes the head and spinal cord. Various diseases of the nervous system cause certain changes in its composition. With Guillana Syndrome - Barre, the level of protein in the liquor rises.

To eliminate other diseases, it may be necessary to carry out the following laboratory studies:

• . Allows you to determine the number of uniform elements in the blood: ,. Reducing the number of erythrocytes and possibly at, raising the level of leukocytes - with different inflammatory processes.
• . This indicator deviates from the norm when various diseasesIn particular, rises with inflammatory processes in the body.
• . With insufficient number in the body of vitamin B 12, anemia can develop, violations in the work of the nervous system. Some symptoms of the damage to the nervous system at B 12-state anemia are similar to the manifestations of Guien syndrome - Barre.
• Detection of heavy metals in the urine. The accumulation of heavy metals (for example, lead) in the body contributes to the defeat of the nervous system and the development of polyneuropathy (the defeat of different nerves).

Other studies:

• Electromyography. Allows you to fix the electrical impulses that come on the nerves to the muscles. According to their intensity, the conductivity of nerve fibers is estimated, for this, special electrodes are superimposed on the muscle under study. The study is carried out in a calm state and when the muscle is reduced.

Additional research

• Magnetic resonance tomography (MRI). The diagnostic method based on the action of the magnetic field to the human body. After processing the received signals, layers of the internal structures of the body are obtained. Allows you to eliminate the presence of other diseases of the nervous system (for example, volume formations).

Treatment

Treatment of disease conservative. Different preparations are used to eliminate individual manifestations of the disease, the fight against the complications of Guien syndrome - Barre.

The most effective are the following techniques:

• Plasmapheresis. The patient takes blood, which is divided into a liquid part (plasma) and a portion containing blood cells (red blood cells, leukocytes, platelets). The blood cells are then returned to the human body, and the liquid part is removed. This achieves peculiar blood purification from antibodies that can destroy myelin sheath of nerves.
• Introduction Immunoglobulin intravenously. Immunoglobulin contains antibodies from healthy blood donors. They block the destructive effect of the patient's antibodies on the shell of nerves.

It is very important to maintain the disturbed functions of the body (artificial ventilation of the lungs), carefully care for the patient, to carry out the prevention of complications associated with the prolonged immobility of patients.

The recovery period uses therapeutic physical culture, physiotherapy to restore the strength of various muscle groups.

Prevention

Specific prevention of Guien syndrome - Barre does not exist.

• Common protein in liquor

Literature

• Dan L. Longo, Dennis L. Kasper, j. Larry Jameson, Anthony S. Fauci, Harrison "S Principles of Internal Medicine (18th Ed.). New York: McGraw-Hill Medical Publishing Division, 2011. CHAPTER 385. GUILLAIN-BARRÉ SYNDROME.
• Carey Foster, Neville F. Mistry, Parvin F. Peddi, Shivak Sharma, The Washington Manual Of Medical Therapeuticts (33th Ed.). Lippincott Williams & Wilkinsphiladelphia, 2010.23 Neurology Disorders. GUILLAIN-BARRÉ SYNDROME.

Guillana Barre syndrome refers to severe autoimmune diseases affecting the peripheral nervous system. The most frequent manifestation is a sharp tetrapapes, when moving all four limbs become almost impossible. Other movements are stopped, including swallowing, the ability to raise eyelids and self-breathing. Despite this, the course of the disease is benign, most cases ends with recovery. There are less often a transition to a chronic course or relapse. Hyien-Barre syndrome is found in all countries regardless of the level of their development with the same frequency - about 2 cases per 100 thousand population, there is no dependence on sexuality. The disease can affect patients of various ages.

Why does the syndrome occur?

The leading mechanism of development is autoimmune. In most cases, the beginning of the disease falls on the first three weeks after an acute respiratory or intestinal infection. Since since the disease there is a sufficient amount of time, and the symptoms characteristic of the infectious process have time to pass, the patients themselves themselves, as a rule, do not communicate with each other. The reason may be such pathogens as:

• epstein-Barra virus or Herpes of Human 4 type;
• mycoplasma;
• campylobacter causing infectious diarrhea;
• cytomegalovirus.

The researchers found that the "shell" of these pathogens has a similarity with an axon's myelin shell of peripheral nerves. This similarity is caused by the attack of nerves by antibodies, which are initially produced and circulated in the blood in response to the appearance of an infectious agent. This phenomenon is called "molecular mimicry" and explains why immune complexes attack their own tissues of the body.

There are cases when the syndrome occurs after vaccination, after surgical operations and abortions, supercooling, stress. In some cases, the reason can not be found.

How does the syndrome manifest?

For several days, maximally to 1 month, muscle weakness increases in the legs, difficulties occur when walking. Next we are weakened, the latter suffers from the Mimic Musculature. Such symptoms have a separate name - ascending paralysis of Landry.

But sometimes paralysis begins on top, with hands, spreading down, but always suffer from all limbs.

Each fifth case is accompanied by paralysis of the musculature of the body, namely the diaphragms and intercostal muscles. With such paralysis, the breathing becomes impossible, artificial ventilation of the lungs is required.

Frequent manifestation - Bulbarium syndrome or double-sided paralysis of the muscles of the soft sky, when it is impossible to swallowing and clear speech.

Together with engine fibers, sensory are sometimes affected. Sensitivity violations are developing, tendon reflexes are reduced, bolted pain in the limbs. Pains are pronounced "neuropathic" character - burning, feeling of current, tingling. Pelvic disorders are rare, but most often the retention of urination, which in some cases is combined with excess urine production.

Vegetative dysfunction is joined, which is manifested by blood pressure oscillations, heartbeat, other impaired heart rhythm, sweating, lack of intestinal motility.

Classification

Several forms allocate depending on whether a myelin shell is damaged or axon, in terms of severity of flow and forecast:

• acute inflammatory demyelinizing polyneuropathy or OWD, when a myelin shell is destroyed;
• acute motor or sensory-motor axonal neuropathy when axons are destroyed;
• rare forms - Miller Fisher Syndrome, Acute Pandisavtonia and others whose frequency does not exceed 3%.

Diagnostic events

• muscular weakness in the limbs that progresses;
• reduced or lack of tendon reflexes from the first days of the disease.

WHO also highlights additional features confirming the diagnosis to which:

• symmetry lesion;
• symptoms increase no more than 4 weeks;
• disturbance of sensitivity by the type of "gloves and socks";
• involvement of cranial brain nerves, especially facial;
• possible spontaneous restoration of functions after stopping the progression of the disease (the so-called "plateau");
• the presence of vegetative violations;
• the absence of hyperthermia (if there is a fever, it is caused by other infections);
• increase the amount of protein in spinal fluidAt the same time, the cell composition does not change (protein-cell dissociation).

The final diagnosis is impossible without electroneuromiography or ENG. This study reveals, which part of the nerve is damaged - myelin shell or axon. The ENGM also accurately establishes the length of the defeat, its severity and the possibility of recovery.

Since, in addition to Hyien-Barre's syndrome, there are a number of sharp, subacute and chronic polyneuropathies, eleganoomyography allows you to carry out differential diagnosis between them and contribute to the development of the correct tactics of treatment.

Often, the diagnosis requires a lumbar puncture, followed by the study of the liquor, and may also be informative such analyzes as:

• blood on the autoantibodies to the structures of neurons;
• blood to Gammaglobulins of Class A (especially if immunoglobulin therapy is planned);
• neurofilament biomarkers (part of the neuron cytoplasm);
• tau-protein markers (special protein destroying neurons).

Specialists of the Calt Clinic additionally use their own differential diagnostic algorithms, allowing reliably to distinguish Hyien Bar syndrome from other diseases that cause progressive muscle weakness in all limbs or tetrapireps.

Our doctors

Rules of treatment

Today, two main pathogenetic methods of treatment of Guillana Barre syndrome are known, and both are successfully used by Calt specialists. This plasmapheresis and intravenous immunotherapy. These methods can be used isolated or used in the complex, it all depends on the specific clinical situation. Treatment is aimed at removing immune complexes circulating in the patient's blood, or neutralized them. Both treatment methods are equivalent, almost always lead to recovery. Treatment stops the process of destruction of peripheral nerves, reduces the duration of the recovery period, helps to reduce the neurological deficit.

Plasmapheresis is a blood purification operation. The hardware plasmapheresis on continuous separators is used, during which the blood selected from the body is divided into uniform elements (or blood cells) and plasma (or serum). All toxic substances are plasma, so it is removed. Man returns his own blood cells, divorced by plasma-substituting solutions or donor plasma, if necessary. The duration of the procedure is about one and a half hours, the entire course consists of 3 or 5 sessions. Related at no more than 50 ml / kg of plasma body weight.

In the process of treatment, blood indicators are controlled: electrolytes, hematocrit, coagulation time and others.

Intravenous immunotherapy is the introduction of the drug of human immunoglobulin class G. These immunoglobulins stop the production of antibodies to their own nerves, in terms of reducing the products of substances supporting inflammation. These drugs are shown for the pathogenetic treatment of Guienen-Barre syndrome in both adults and children.

Simultaneously with specific treatment, careful patient care is ensured, comprising preventing the prevention, pneumonia, contractures. Often the treatment of concomitant infections is often required. The prevention of venous thrombosis is performed, feeding through the probe is carried out, the excretory function is controlled. Legging patients are subject to passive gymnastics, as well as early verticalization, allowing to avoid blood flow disorders. In the threat of contacting the contact (immobility of the joints), paraffin procedures are possible. If necessary, motor simulators based on biological feedback.

Patients with damage to myelin shells are restored faster, the axonal damage require a longer rehabilitation period. Axion lesions often leave neurological deficit, difficult to correction.

Prevention

The main method is a complete cure of infections that we consider banal, habitual. Hyien Barre syndrome is often developing with a minor weakening of immunity, which is possible for each person.

The easiest way to protect yourself is to check the current immune status. It takes only a few days, and detected deviations can be accelerated on time.

At the disposal of the Calt Clinic doctors not only the latest diagnostic equipment, but also the newest medical techniques that have received world recognition. The main role in prevention belongs to the patient, who is addressed for the examination and treatment in a timely manner.

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Hyien-Barre syndrome (acute idiopathic polyneurite; Landry paralysis; acute inflammatory demyelinizing polyradiculonereropathy) is an acute, usually rapidly progressive inflammatory polyneuropathy, characterized by muscle weakness and a moderate loss of distal sensitivity. An autoimmune disease. Diagnosis for clinical data. Treatment of Guillana Barre Syndrome: Plasmapheresis, U-Globulin, according to the testimony of artificial ventilation of the lungs. The outcome of the syndrome is significantly improved with adequate supporting treatment under the conditions of separation of intensive therapy and the use of modern methods of immunomodulatory therapy.

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Code of the ICD-10

G61.0 Hyien Barre Syndrome

Epidemiology

The incidence is from 0.4 to 4 cases per 100,000 population per year. Hyien Barre syndrome is observed in any age groups, but more often in people of 30-50 years old, with equal frequency of men and women. Racial, geographical and seasonal differences in the incidence for Hyien-Barre syndrome are not characteristic, the possible exception is cases of acute motor axonal neuropathy, which are most common in China and are usually associated with intestinal infection due to Campylobacter jejuni.and therefore somewhat often arise in the summer.

The incidence is significantly increased after 40 years. A year from Guillana Barre syndrome, 600 people die in the United States. Thus, Guillana Barre syndrome is a very important health problem, especially relevant for older people.

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Causes of Guillana Barre Syndrome

The most common of acquired inflammatory neuropathy. An autoimmune mechanism is not fully studied. Several options are known: demelination prevails in some, the axon suffers in others.

About 2/3 cases syndrome appears in 5 days - 3 weeks after infectious disease, operations or vaccination. In 50% of cases, the disease is associated with infection Campylobacter jejuni,enterovirus and herpes viruses (including cytomegalovirus and mononucleosis viruses), as well as Mycoplasma SPP.In 1975 there was an outbreak, associated with the program of vaccinations against flu flu.

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Pathogenesis

Demyelinization and inflammatory infiltration in the roots of spinal nerves and proximal nerves can explain the clinical symptoms of Guienen-Barre syndrome. It is believed that in the pathogenesis of the disease, both humoral and cellular immunity are involved. The presence of lymphocytes and macrophages in the perivozny zones and their interaction with myelinized axons indicate, above all, the possible role of autoimmune reactions in the demyelinizing process. This provision is confirmed by earlier observations, according to which the immunization of laboratory animals peripheral myelin with adjuvant causes experimental allergic neuritis. Although in the future it was shown that purified myelin proteins - for example, the main protein of myelin P2 or peptide fragments P2 and protein ro - can cause experimental neuropathy, the antibodies to these compounds are rarely detected during Guien-Barre syndrome. T-cells isolated from spleen and lymph nodes of rats immunized with P2 synthetic peptide 53-78 can be reproduced by severe experimental allergic neuritis in singenic mice. Thus, cellular and possibly humoral immune mechanisms may mediate the creation of an experimental model of inflammatory damage to the peripheral nerves.

In recent studies, attention was drawn to the role of glucoconjugates and lipopolysaccharides of myelin shell, the membranes of Schwann cells or axonal membranes as basic antigens, initiating an inflammatory / immune response with Guienen-Barre syndrome. In a detailed study conducted in Japan, antigens were identified in patients Campylobacter jejuni.. In this study, the Penner method was used to detect thermostable lipopolysaccharides, and to determine the thermolabile protein antigens - the Lior method. Antigens Pen 19 and Lio 7 S. Jejuni.we were more common in patients with hyena-barre syndrome (respectively in 52 and 45% of cases) than in patients with sporadic enteritis caused by S. Jejuni.(respectively 5 and 3%), and were associated with an increase in antibody titer to GM1 (possibly due to the presence of GML-like lipopolysaccharide antigen). According to other countries, infection S. Jejuni.much less often precedes the development of the SGB. In addition, the percentage of patients with antiganglide antibodies was much more variable, hesitated from 5% to 60%. In addition, no correlation was found between the presence of antibodies to GM1 and clinical and electrophysiological manifestations of the disease.

In the Fisher Miller syndrome, antibodies to GQLB are often detected. Using immunohistochemical GQLB methods, it was detected in the paranodal region of human cranial nerves, innervating eyes. It has been established that the GQLB antibodies can block the transmission in the neuromuscular mouse system.

During the axonial motor version of Hyien-Barre syndrome, S. jejuni infection was often preceded by an antibody of GM1 ganglioside and the C3D complement activation product were associated with acceum motion engine fibers.

Antibodies to GMI can also contact Ranvier interceptions, thus disrupting pulses. In addition, these antibodies are able to cause degeneration of endings of motor fibers and intramuscular axons, which was recently shown in patients with acute motor axonal polyneuropathy. Enteritis caused by S. Jejuni can provoke Guienen Barre syndrome, enhancing gamma delta-T-lymphocyte products that can actively participate in inflammatory / immune processes. The high level in the serum of the necrosis of tumor-alpha (FNO-A), but not the interleukin-1b or soluble interleukin-2 receptor, correlated with electrophysiological changes in the Hyien Barre syndrome. The study of the samples obtained in autopsy indicates that at least in some cases, the classic acute inflammatory demyeelinizing form of Guien-Barre syndrome is involved in the complement - it indicates the detection on the outer surface of Schvanna cells C3D- and C5D-9-components forming a membrane pumping complex.

Thus, during the syndrome, Guillana Barre is represented by most of the components that are usually involved in the pathogenesis of immuno-consistent diseases. Although glucoponegate antibodies are likely to participate in the pathogenesis of several different clinical forms of Guienen-Barre syndrome, their exact role is unknown. Even if the antibodies to GM1 are present, they can be born not only with GM1, but also with other glycolipids or glycoproteins that have similar carbohydrate areas. In this connection, specific antigens of Svannov cells or axonal membranes against which the inflammatory / immune response is directed, as well as the possible role of immunoglobulins require clarification. Moreover, in many cases, Guienen-Barre syndrome, there are no signs of preceding or concomitant infection. S. Jejuni,antibodies to GM1 or signs of another microorganism, whose antigens may initiate an immune response (for example, due to molecular mimicry).

The study of materials obtained in biopsy of nerves and autopsy shows that cellular immune mechanisms also contribute to the development of Guienen-Barre syndrome. In severe cases of Guillana Barre syndrome, there are lymphocytes and macrophages throughout the motorcycle fibers from the roots, and the activated macrophages are closely in close contact with myelin or phagocyse myelin. Although the experimental model of inflammatory neuropathy obtained data confirming the participation of T-lymphocytes in nerve damage, there are no convincing evidence that it takes place in patients with Guien-Barre syndrome. Currently accumulated data confirm the involvement of activated T-lymphocytes crossing the hematorecephalic barrier and initiating demyelinization together with antibodies to specific antigens of nerve fibers, cytokines (such as TNF-A and interferon-y), complement components, possibly, including membrane complex and activated macrophages. . Additional research is needed to clarify the role of each of these elements, as well as the sequence in which they are involved in the pathogenesis of Guillana Barre syndrome.

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Symptoms of Guienen Barre Syndrome

The symptoms of Guienen-Barre syndrome are in the dominance of a sluggish pan (which is proximal, the more deep), the sensitivity disturbances are less pronounced. It is typical that almost symmetrical weakness with paresthesia begins with legs, less often with hands or head. In 90% of cases, weakness reaches a maximum at the 3rd week of the disease. Deep tendon reflexes fall out. The sphincter feature is saved. With severe flow in half cases, the weakness of the facial and orofaring muscles is obvious. In 5-10% of cases, intubation and IVL are required due to the paralolies of the respiratory muscles.

Sometimes (apparently, under optional form), pronounced vegetative dysfunction with hesitation of blood pressure, pathological secretion of antidiuretic hormone, arrhythmias, intestinal stained, urine delay and violation of the reaction of pupils into light. Fisher's syndrome is a rare version of Guillana Barre syndrome, suggests ophthalmoplegia, ataxia and areflexia.

The first symptoms, the order of their appearance and dynamics

In typical cases, Guillana Barre syndrome begins with muscle weakness and / or sensory disorders (Feeling numbness, paresthesia) in the lower limbs that in a few hours or day apply to the upper limbs.

The first symptoms of Guillana Barre are impairment of sensitivity, for example, paresthesia in the footsteps. Although the objective signs of sensitivity impairment are detected quite often, they are usually lungs. Early and extremely unpleasant for patients with manifestations of the disease can be deep many pain In the back and painful diesesthesia in the limbs. Paraliances may initially involve the lower limbs, and then quickly, for several hours or days, spread in the ascending direction to the upper limbs, mimic, boulevard and respiratory muscles. However, the development of events is possible when the disease begins with weakness in the mimic muscles and upper limbs, then involves the lower limbs. From the very beginning, the symptoms are usually symmetrical, and paralysis is accompanied by the fallout or weakening of tendon and periosteal reflexes. In the Hyien Barre syndrome, vegetative fibers are often involved. Vegetative symptoms are detected in about 50% of cases, but the sphincter features usually do not suffer. The disease has a monophasis flow: following the random period of symptoms that continues for several days or weeks, a period of a plateau is followed by a duration of several days to several months, after which the restoration occurs over several months. In 1976-1977, there was some increase in the incidence of hyena-barre syndrome, associated with the immunization of pork flu vaccine, but during immunization with another version of the flu vaccine in 1980-1988, not registered with a similar phenomenon.

In classical cases that manifest themselves with a combination of motor, sensitive and vegetative symptoms, which are based on demyelinizing polyradiculoneuropathy, the diagnosis of Guienen-Barre syndrome rarely causes difficulties. However, the axonal version of Guillana Barre syndrome is also found, mainly manifested by motor disorders, and acute motor-sensoryaxonal neuropathy. Acute axonal form usually manifests itself a roughest functional defect and has a more unfavorable forecast. The combination of ophthalmoplegia, the ataxia of the hi-ereflexia is characteristic of another version of Guillana Barre syndrome, known as Miller Fisher's syndrome. From a diagnostic point of view, in the absence of symptoms of the defeat of the cranial nerves, even with the preserved function of the sphincters, it is necessary to eliminate the spinal cord with the help of neurovalization. In differential diagnosis, it is also important to keep in mind the acute intermittent porphyria, intoxication by metals capable of causeing acute polyneuropathy, as well as systemic diseases such as infectious mononucleosis, paranoplastic syndromes or various metabolic disorders. HIV-infected patients are predisposed to the development of polyneuropathy or polyradiculoneuropathy, which can be associated with hyena-barre syndrome, cytomegalovirus polyradiculoneuropathy or lymphoma. These states are difficult to differentiate, based only on clinical manifestationsBut the study of the cerebrospinal fluid during polyradiculoneuropathies associated with HIV infection usually reveals neutrophilic Pleasitosis and signs of virus replication.

Vegetative dysfunction (including accommodation disorders, abdominal pain and chest, arterial hypotension, tachycardia) can significantly exacerbate the patient's condition and serves as an unfavorable prognostic sign. In one of the studies, subclinical signs of involvement of both the sympathetic and parasympathetic nervous system, detected by the tests for vegetative functions, are noted in the overwhelming majority of patients.

North American motor deficit severity

• Third patients develop respiratory failure.
• In most cases, there are surface sensitivity disorders in the form of a light or moderate hypo-or hyperesthesia by polynelectric type (according to the type "socks and gloves"). Often marked the pains in the hips, lumbar and berry areas. They can be both nociceptive (muscle) and neuropathic (due to the lesion of sensory nerves). Disorders of deep sensitivity (especially vibration and muscular-articular feelings), extinguishing very rough (up to complete loss), reveal at about half of the patients.
• The defeat of the cranial nerves is observed in the majority of patients. It is possible to engage in the process of any cranial nerves (with the exception of the I and II Course), but with the greatest constancy, the defeat VII, IX and X pairs are observed, which is manifested by parares mimic muscles and bulbar disorders.
• Vegetative disorders are observed more than half of the patients and can be represented by the following violations.
  • Transient or persistent arterial hypertension or less often arterial hypotension.
  • Cardiac arrhythmias, most often sinus tachycardia.
  • Potting Disorder [Local (palms, stop, face) or general hyperhydrosis].
  • Disorders of the Functions of the GTS (constipation, diarrhea, in rare cases intestinal obstruction).
  • Violations of the functions of pelvic organs (usually urine delay) rarely occur, they are usually weak and transit.
• When Miller Fisher's syndrome in a clinical picture, an ataxia is dominated in a clinical picture, which usually has a cerebelchkova feature, in rare cases - mixed (cerebelchikovo-sensitive), and partial or total ophthalmoplegia, it is also possible to defeat other cranial nerves (VII, IX, X). Parares are usually weakly visible, in a quarter of cases there are sensitivity disorders.

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Diagnostic criteria Guienen-Barre syndrome

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Signs of Guillana Barre Syndrome necessary for diagnosis

• A. Progressive muscular weakness in more than one limb
• B.Areflexia (lack of tendon reflexes)

Signs of Guillana Barre Syndrome, supporting the diagnosis

• A. Clinical signs (listed in the order of importance)
  • Progression: Muscular weakness develops quickly, but stops progressing within 4 weeks after the start of the disease.
  • Relative symmetry: symmetry is rarely absolute, but with a defeat of one limb, the opposite is also amazed (comment: patients often report symmetricity at the beginning of the disease, but by the time of an objective examination of the lesion, as a rule, symmetrical).
  • Subjective and objective symptoms of sensitivity impairment.
  • The defeat of the cranial nerves: parishes of mimic muscles.
  • Restoration: usually begins in 2-4 weeks after it stops progressing the disease, but sometimes can be lingering for several months. Most patients have full restoration of functions.
  • Vegetative violations: Tachycardia and other arrhythmias, postural arterial hypotension, arterial hypertension, vasomotor disorders.
  • Lack of fever in the debut of the disease (in some cases, fever in the debut of the disease is possible due to intercurrent diseases or other reasons; the presence of fever does not exclude Hyien Barre syndrome, but increases the likelihood of the presence of another disease, in particular polio).
• B. Options
  • Pronounced sensitivity disorders with pain.
  • Progression of more than 4 weeks. Sometimes it is possible to progress the disease for many weeks or the presence of small recurrences.
  • Termination of progression without subsequent restoration or preservation of pronounced residual symptoms.
  • Sphincter features: Usually sphincters are not affected, but in some cases urination disorders are possible.
  • Defeat CNS: In the syndrome of Guien-Barre, the peripheral nervous system is affected, reliable evidence of the possibility of damage to the CNS. Some patients have a coarse attack of cerebellary character, pathological stop signs of an extension type, dysarthria or fuzzy level of sensory disorders (implying conduction type of disorders), but they do not exclude the diagnosis of Guienen-Barre syndrome if there are other typical symptoms
• C. Changes in liquor confirming the diagnosis
  • Protein: 1 week after the start of the disease, the protein concentration in the liquor becomes elevated (during the first week it may be normal).
  • Cytosis: Content of mononuclear leukocytes in liquor up to 10 in 1 μl (when the leukocyte content of 20 in 1 μl and it is more necessary to examine. If their content is more than 50 in 1 μl, the diagnosis of the Guillana Barre syndrome is rejecting; the exception is patients with HIV infection and Lime Burreliosis).

Signs of Guillana Barre Syndrome, causing doubts about the diagnosis

1. Pronounced persistent asymmetry of paresis.
2. Resistant pelvic violations.
3. The presence of pelvic disorders in the debut of the disease.
4. The content of mononuclear leukocytes in the liquor is more than 50 in 1 μl.
5. The presence of polymorphoid leukocytes in the liquor.
6. Clear level of sensitivity disorders

Signs of Guillana Barre syndrome, excluding the diagnosis

1. Abuse Currently volatile organic solvents (toxicomia).
2. Disorders of the exchange of porphyrins that imply a diagnosis of acute intermittent porphyry (increased excretion with porpobilinegen or aminolevulinic acid).
3. Recently transferred diphtheria.
4. The presence of neuropathy symptoms due to intoxication by lead (partares of the muscles of the upper limb, sometimes asymmetrical, with a pronounced weakness of the extensor of the brush) or proof of intoxication lead.
5. The presence of exclusively sensory disorders.
6. A reliable diagnosis of another disease that is manifested by the syndrome of Guienen-Barre symptomatomy (poliomyelitis, botulism, toxic polyneuropathy).

Forms

Currently, within the framework of Hyien-Barre syndrome, four main clinical options are distinguished.

• Acute inflammatory demyelinizing polyradiconeuropathy - the most frequent (85-90%), the classic form of Guienen-Barre syndrome.
• The axonal forms of Guienen-Barre syndrome are significantly less common (10-15%). Acute motor axonal neuropathy is characterized by an isolated lesion of motor fibers, most common in Asian countries (China) and South America. With acute motor-sensory axonal neuropathy, both motor and sensitive fibers are affected, this form is associated with a protracted flow and adverse outlook.
• Miller-Fisher syndrome (no more than 3% of cases) is characterized by ophthalmoplegia, cerebellar attacksia and areflexia with usually low-voltage passes.

In addition to the main, recently, several other atypical forms of the disease are also distinguished - sharp pandisavtonia, acute sensory neuropathy and acute cranial polyneuropathy, which observed very rarely.

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Diagnosis of Guillana Barre Syndrome

When collecting anamnesis, it is necessary to clarify the following aspects.

• The presence of provoking factors. Approximately 80% of cases of Guillana Barre syndrome in 1-3 weeks are preceded by certain diseases or states.
• - Georgian infections, upper respiratory tract or other localization. With the greatest constancy, the association with intestinal infection caused by Campylobacter jejuni.In persons who moved campylobacteriosis, the risk of developing Hyien Barre syndrome for 2 months after the disease is approximately 100 times higher than in the overall population. Guillana Barre syndrome can also develop after infections caused by herpes viruses (cytomegalovirus, Epstein-Barr virus, virus vARICELLA-ZOSTER), Haemophilus Influenzae,mycoplasmas, Corey, Parotitis, Lime Burreliosis, etc. In addition, Guienyen-Barre syndrome can develop with HIV infection.
• Vaccination (anti-executive, anticipatible, against influenza, etc.).
• Operational interventions or injuries of any localization.
• Reception some medicines (Thrombolytic preparations, isotretinoin, etc.) or contact with toxic substances.
• Sometimes hyena-barre syndrome develops against the background of autoimmune (systemic red lupus) and tumor (lymphogranulomatosis and other lymphoma) diseases.

Laboratory and instrumental research

• General Studies ( general analysis blood, general urine analysis).
• Biochemical blood test: serum electrolyte concentration, gas composition of arterial blood. When planning specific therapy with immunoglobulins of class G, it is necessary to determine the IG fractions in the blood. Low concentration Iga is usually associated with its hereditary deficit, in such cases there is a high risk of developing anaphylactic shock (immunoglobulin therapy is contraindicated).
• Research of liquor (cytosis, protein concentration).
• Serological studies when suspected by the etiological role of certain infections (markers HIV, Cytomegalovirus, Epstein-Barr virus, Borrelia Burgdorferi, Campylobacter Jejunietc.). In case of suspected polio, virological and serological (titer antibodies in paired serums) studies are necessary.
• EMG, the results of which are of fundamental importance to confirm the diagnosis and definition of the shape of Guienen-Barre syndrome. It should be borne in mind that the results of EMG may be normal during the first week of the disease.
• Neurovulization methods (MRI) do not allow to confirm the diagnosis of Guienen-Barre syndrome, but may be necessary for the differential diagnosis with the CNS pathology (acute violation of cerebral circulation, encephalitis, myelites).
• Monitoring the function of the external respiration [determination of the vital capacity of light (jerking) for timely detection of indications for the translation of the patient to the IVL.
• In severe cases (especially with the rapid progression of the disease, bulbar disorders, expressed vegetative disorders), as well as during the IVL, monitor the main vital indicators (under conditions of intensive therapy): blood pressure, ECG, pulse oximetry, external respiratory functions and other ( Depending on the specific clinical situation and therapy).

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Neurophysiological criteria for the classification of Hyien-Barre syndrome

The norm (all the signs below must be present in all the nerves studied)

1. Distal motor latency
2. Preservation of F-Wave and its latency
3. SRV\u003e 100% of the lower limit of the norm.
4. M-response amplitude when stimulated in the distal point\u003e 100% of the lower limit.
5. The amplitude of the M-response when stimulated in the proximal point\u003e 100% of the lower boundary of the norm.
6. The ratio of the "M-response amplitude during stimulation at the proximal point / amplitude of the M-reply when stimulated in a distal point"\u003e 0.5

Primary-demyelinizing lesion (the presence of at least one of the signs of at least two studied nerves is needed or the presence of two signs in one nerve, if all other nerves are not excited and the amplitude of the M-response during stimulation in the distal point\u003e 10% of the lower boundary of the norm ).

1. Standard motor latency\u003e 110% of the upper limit boundary (\u003e 120%, if the amplitude of the M-response when stimulated in the distal point
2. The ratio of the "amplitude of the M-reply when stimulated in the proximal point / amplitude of the M-response during stimulation in the distal point" 20% of the lower limit of the norm.
3. F-wave latency\u003e 120% of the upper limit of the norm

Primary-axonal defeat

• The absence of all the above signs of demyelination in all the nerves studied (permissible the presence of one of them in one of the nerves if the amplitude of the M-response when stimulation in the distal point

The non-accidentability of nerves

• M-response When stimulated in a distal point, it is not possible to call in any of the nerves (or it is possible to call only in one nerve at its amplitude

Uncertain defeat

Does not correspond to the criteria for any of the forms listed above

This form may include cases of primary heavy axonopathy, severe distal demyelination with a block of conducting and secondary Valerian degeneration after demyelinization; Neurophysiologically, they are impossible to distinguish them.

Indications for consultation of other specialists

• Treatment of patients with heavy forms of Guien-Barre syndrome is carried out in conjunction with a physician of resuscitation and intensive therapy.
• With pronounced cardiovascular disorders (persistent pronounced arterial hypertension, arrhythmias), consultation of the cardiologist may be required.

Data of additional research methods

Important diagnostic value In the syndrome, Guillana Barre have electromyography (EMG) and the study of the velocity of pulses on nerves, as well as the study of the cerebrospinal fluid. Starting from 3-7 days after the appearance of the first symptoms in electrophysiological examination, a slowdown in the motor and (to a lesser extent) on sensory fibers, the elongation of distal latenpia and the latent period of the F-wave, reducing the amplitude of the total muscular potential of the action (M-reply ) and sometimes sensory potentials of action, as well as focal and asymmetric blocks of conduct, which indicate a segmental demyelinizing polyneuropathy. On the other hand, during acute motor axonal polyneuropathy of the amplitude of sensory potentials and the speed of the sensory fibers can be normal, but there is a decrease in the amplitude of the total muscle potential of the action and only a slight slowdown in the conduct of motion fibers. With damage to both motor and sensory fibers, it can be roughly changed both the total muscle potentials of the actions and sensory potentials of the action, and the distal lancer and the speed is difficult to measure, which indicates severe motor-sensory axonopathy. With Miller's Fisher's syndrome, manifesting ataxia, ophthalmoplegia of the Jareflexia, the strength of the muscles remains preserved, and EMG indicators and the velocity of the nerves of the limbs may be normal.

In the study cerebrospinal fluidin patients with hyena-barre syndrome, an increase in protein content to a level exceeding 60 mg / dl is detected, with normal cytosis (no more than 5 cells in 1 μl). However, in the first days of the disease, the protein content in cerebro-spinal fluid can be normal, while the increase in cytosis up to 30 cells in 1 μl does not exclude the diagnosis of Guienen-Barre syndrome.

Since in the study of the bioptate of calf nerves, as a rule, signs of inflammation or demyelinization are not detected, this method is not included in a standard range of research in most patients with Guienen-Barre syndrome, but may be important in scientific research. Patomorphological studies suggest that the proximal departments of nerves and spinal nerves roots are presented primarily affecting the hyiren-barra syndrome: it is precisely in them swelling, segmental demyelinization, infiltration of endonervia mononuclear, including macrophages. Mononuclears interact both with Schwann cells and with myelin shell. Although hyena-barre syndrome - polyradiconeuropathy, pathological changes can be detected in the central nervous system (CNS). In most of the 13 autopsy cases, mononuclear infiltration of lymphocytes and activated macrophages was found in the spinal cord, the oblong brain, the bridge. However, the CNS did not reveal primary demyelinization. With a long flow, the prevailing type of inflammatory cells in the central and peripheral nervous system was activated macrophages, in addition, CD4 + and CD8 + T-lymphocytes were detected there.

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Differential diagnosis

Guillana Barre syndrome must be differentiated from other diseases that are manifested by sharp peripherals, primarily from polio (especially in children early age) and other polyneuropathies (diphtheria, in porphyria). In addition, similar clinical picture They may have damage to the spinal cord and brain barrel (transverse myelitis, stroke in the vertebrobasilar system) and diseases with impaired neuromuscular transmission (miastic, botulism).

• With differential diagnosis with polio, the data of epidemiological history should be taken into account, the presence in the debut of diseases of fever, symptoms from the gastrointestinal tract, asymmetry of the lesion, the absence of objective sensitivity disorders, high cytosis in the liquor. The diagnosis of poliomyelitis is confirmed by virological or serological studies.
• Polynevropathy with acute intermittent porphyry can be reminded by Guillana Barre syndrome, but, as a rule, is accompanied by a variety of psychopathological symptoms (nonsense, hallucinations, etc.) and pronounced abdominal pains. The diagnosis is confirmed by revealing the increased concentration of porpobilinegen in the urine.
• For transverse mixtures, early and persistent violations of the functions of pelvic organs are characterized, the presence of a level of sensitive disorders, the lack of damage to the cranial nerves.
• Symptomtics, which resembles such a hyeien-barre syndrome, is possible with the extensive heart attacks of the brain barrel with the development of tetraprack, which in the sharp period has a peripheral feature. However, such cases are characteristic. acute development (Usually within a few minutes) and in most cases, the inhibition of consciousness (coma), which is not observed during the hyeien barre syndrome. Finally, the diagnosis is confirmed by MRI.
• Miasthenia differs from Hyien-Barre syndrome variability of symptomatics, lack of sensitive disorders, characteristic changes in tendon reflexes. The diagnosis is confirmed by EMG (detection of the decrement phenomenon) and pharmacological samples.
• For botulism, in addition to the corresponding epidemiological data, the descending type of the spread of paresis, the safety in terms of cases of tendon reflexes, the lack of sensitive disorders and changes in the liquor is characterized.

Treatment of Guillana Barre Syndrome

The objectives of the treatment of hyena-barre syndrome is to maintain vital functions, the relief of the autoimmune process with the help of specific therapy, the prevention of complications.

Indications for hospitalization

All patients with Hyien-Barre syndrome are subject to hospitalization in a hospital with an intensity and intensive care unit.

Non-media methods for the treatment of Guien-Barre syndrome

Approximately in 30% of the cases of Guillana Barre syndrome develops heavy respiratory failure (due to the paresis of the diaphragm and respiratory muscles), which determines the need for IVL. Indications for intubation with further ventilator - a decrease of up to 15-20 ml / kg, r a 2 50 mm Hg. The duration of the IVL (from several days to months) is determined individually, focusing on the fault, the restoration of swallowing and cough reflex and the overall dynamics of the disease. Turn off the patient from the IVL apparatus gradually, through the stage of intermittent forced ventilation.

In severe cases with severe paresis, fundamental importance for preventing complications associated with prolonged immobility of the patient (prolesideling, infection, thromboembolic complications, etc.) has correct care: Periodic (every 2 h or more) Change of the patient's position, skin care, prevention of aspiration [Sanitation of the oral and nose cavity, feeding through a nasogastric probe, Sanitation of trachea and bronchi (when conducting an IVL)], control over functions bladder and intestines, passive gymnastics and limb massages, etc.

With a stale bradyarithmy with the threat of the development of asistolia, it may be necessary to install a temporary driver of the rhythm.

Drug treatment and plasmapheresis

As a specific therapy of Hyien-Barre syndrome, aimed at relieving the autoimmune process, the pulse-therapy of immunoglobulins of class G and plasmapheresis is currently used. Methods of specific therapy are shown in severe (assessment on the North American scale of the severity of the motor deficiency of 4 and 5 points) and medium-sized (2-3 points) of the disease. The effectiveness of both methods is approximately the same, their simultaneous conduct is inappropriate. The treatment method is chosen individually, taking into account the availability, possible contraindications, etc.

• Plasmapheresis is an effective method for the treatment of Guienen-Barre syndrome, significantly reducing the severity of paresis, the duration of the IVL and the improvement of the functional outcome. Typically spend 4-6 operations with an interval in one day; The volume of the replaced plasma for one operation should be at least 40 ml / kg. A 0.9% sodium solution of chloride, refooliglukin, albumin solution is used as substitutors. Plasmapheresis relatively contraindicated liver failure, severe pathology of the cardiovascular system, blood gaining disorders, presence of infections. Possible complications - Hemodynamic disorders (drop in blood pressure), allergic reactions, disorders of electrolyte composition, hemorrhagic violations, the development of hemolysis. All of them are observed quite rarely.
• Immunoglobulin class G is administered intravenously at a dose of 0.4 g / kg once a day for 5 days. Medium treatment with immunoglobulin, as well as plasmapheresis, reduces the duration of staying on the IVL and improves the functional outcome. The most frequent side effects are head and muscle pain, fever, nausea; Their severity can be reduced by reducing the infusion rate. Weight side effects, such as thromboembolism, aseptic meningitis, hemolysis, acute renal failure, etc., are extremely rare. Immunoglobulin human normal is contraindicated with an innate IGA deficiency and the abstract anaphylactic reactions to the drugs of immunoglobulin.

Symptomatic treatment of Guienen-Barre syndrome

• Infusion therapy for the correction of disorders of acid-base, water-electrolyte balances, pronounced arterial hypotension.
• With persistent pronounced arterial hypertension, antihypertensive preparations are prescribed (beta-adrenoblays or block plates of slow calcium channels).
• With pronounced tachycardia, beta-blockers are prescribed (propranolol), in bradycardia - atropine.
• In the development of intercurrent infections, antibiotic therapy is needed (drugs are used wide spectrum Actions, such as fluoroquinolones).
• For the prevention of deep veins and thromboembolism of the light artery, a low molecular weight heparin is prescribed in prophylactic doses twice a day).
• Under the pains of nociceptive origin (muscle, mechanical), paracetamol or NSAIDs, in the case of a neuropathic nature of pain, gabapentin, carbamazepine, pregabalin are recommended.

Operational treatment of Guienen-Barre syndrome

If necessary, long-term (more than 7-10 days) ZVL is advisable to impose tracheostas. With heavy and long-term bulbar violations, the exhibition of gastrostomy may be required.

General Principles of Treatment of Guillana Barre Syndrome

Treatment of acutely developing and rapidly increasing manifestations of Guienen-Barre syndrome requires supporting therapy in the conditions of separation of intensive therapy, as well as the impact on immune mechanisms for the development of the disease. Patients with Hyien Barre syndrome should be hospitalized for careful observation of the condition of respiration and vegetative functions. The faster paralympus increases, the higher the likelihood that artificial ventilation of the lungs will be required. In the rate of symptoms, a regular neurological inspection is needed, an assessment of the life capacity of the lungs, maintenance of respiratory tract with regular suction of mucus. At an early stage of the disease, constant alertness is needed, because even in the absence of obvious disorders of respiratory and bulbarous functions, small aspiration can significantly increase vegetative dysfunction and provoke respiratory failure.

Improving the forecast and reduced mortality in the syndrome of Guienen-Barre, achieved in last yearsare mainly due to early hospitalization of patients in the separation of intensive therapy. Indications for the translation of the patient in the intensive care unit and the consideration of the issue of intubation can be a decrease in the life capacity of the lungs below 20 ml / kg and the difficulty when removing the secret from the respiratory tract. The goal of early translation is to avoid urgent intubation under conditions of severe respiratory failure with sharp fluctuations in blood pressure and heart rate, which can provoke dysfunction or myocardial infarction. One of the most important tasks of supporting therapy is a prevention and timely treatment of pulmonary and urinary infections, as well as the prevention of a deep vein thrombosis and the subsequent thromboembolism of the pulmonary artery by subcutaneous administration Heparin (5000 ed 2 times a day). It is also necessary to monitor power and intestinal function. Since vegetative dysfunction has a significant impact on mortality, constant control over cardiac activity and arterial pressure is necessary.

One of the important aspects of helping the patient with Guillana Barre syndrome in the conditions of the intensive care unit, which, however, is not always taken into account, the correction of a pronounced anxiety, which is caused by the complete immobilization of the patient against the background of the preserved intelligence. In this regard, psychological support is important. The patient needs to explain the essence of the disease, the features of its flow, including the possibility of progression, familiarize with the methods of treatment at various stages. It is important for them to clarify that the probability of complete recovery is very high, even if for some time they will be on the artificial ventilation of the lungs. The establishment of contact with the help of eye movements reduces the feeling of insulation from the world in patients. In our experience, the introduction of 0.5 mg of Lorazepam every 4-6 hours is effective in night hallucinations. It is also possible to assign 0.5 mg of risperidone or 0.25 mg of olanzapine.

The practice of treating Hyien Barre syndrome has undergone significant changes over the past decade. For example, the effectiveness of plasmapheresis was proved. Although the mechanism of its action remains unknown, it is believed that it may be associated with the removal of antibodies, cytokines, complement and other mediators of the immunimipal reaction. In an open multicenter North American study, compared the outcomes of the disease when using Plasmapheresis and in the absence of special treatment, it was shown that plasmapheresis over five days in a row reduces the duration of hospitalization and leads to a more significant improvement than in the control group. The treatment was more efficient if the disease began in the first week. Similar results were received by the FRENCH Cooperative Group, which conducted a randomized multicenter study and showed that four sessions of plasmapheresis lead to a faster recovery in 220 patients included in the study (FRENCH COOPEROTIVE GROUP, 1987). The study of the same patients in a year has shown that the complete restoration of muscle force is marked in 71% of patients who were conducted by plasmapheresis, and only 52% of patients in the control group (FRENCH COOPERATIVE GROUP, 1992). The following study combed the effectiveness of a different number of plasmapheresis sessions in 556 patients with Guienen-Barre syndrome with various symptom sensions (FRENCH COOPERATIVE GROUP, 1997). In patients with light symptoms, which were held two sessions of plasmapheresis, the restoration turned out to be more significant than in patients, the treatment diagram of which did not include plasmapheresis. In patients with moderate expression of symptoms, four plasmapheresis sessions were more efficient than two sessions of Plasmapheresis. At the same time, six plasmapheresis sessions on efficiency did not exceed four sessions in patients with both moderate and severe symptoms. Currently, in most centers specializing in the treatment of Guienen-Barre syndrome, it still uses five to six sessions that are conducted for 8-10 days to avoid stress associated with the daily conduct of this procedure. Exchange transfusion is carried out using catheter sewed. Plasmapheresis is also effective in children with Hyien Barre syndrome, speeding up the process of restoring the ability to independently movement. Although plasmapheresis is a relatively safe procedure, its conducting during the Hyien Barre syndrome requires special caution due to the danger of vegetative dysfunction in patients and their tendency to develop infections.

Intravenous administration of high doses of immunoglobulin is also recognized effective method The treatment of hyena-barre syndrome capable of significantly reduce the duration and severity of the disease. As in the case of plasmapherester, the mechanism of the therapeutic effect of immunoglobulin remains unclear. It is assumed that it can eliminate pathogenic antibodies due to anti-sidiotic antibodies, block the FC component of antibodies on target cells, and also slow down the complement deposition, dissolve immune complexes, weaken the functions of lymphocytes, disrupt the products or prevent the implementation of cytokine functions. Immunoglobulin is prescribed in a summary dose of 2 g / kg, which is administered for 2-5 days. In a randomized study, which compared the effect of immunoglobulin and plasmapheresis, it was shown that when using Plasmapheresis, the improved average occurs after 41 days, and when applying immunoglobulin - after 27 days. In addition, in patients who were injected with immunoglobulin, significantly less complications were noted and an artificial ventilation of the lungs was needed. The main unfavorable prognostic factor was elderly age. The subsequent randomized multicenter study of plasmapheresis and immunoglobulin in 383 patients who were prescribed these techniques for the first 2 weeks after the appearance of symptoms showed that both methods have comparable efficacy, but their combination does not have significant advantages before applying each of the methods separately.

The introduction of immunoglobulin at a dose of 2 g / kg for 2 days turned out to be an effective and safe method of treatment and in children with severe Hyien Barre syndrome. Side effects were lightweight and rarely observed. Part of the patients, especially those who suffered migraine, was noted headachewhich was sometimes accompanied by aseptic meningitis with pleyocytosis in the cerebrospinal fluid. Sometimes, chills, fever and myalgia were also observed, as well as a sharp impairment of kidney function with development renal failure. With the introduction of immunoglobulin, an anaphylactic reaction is possible, especially in persons with immunoglobulin deficiency A. The main drawback and immunoglobulin, and plasmapheresis - high cost. However, it clearly outweighs the effectiveness of these treatment methods, which is obvious even in the current era, forcing money.

As a double-blind placebo-controlled multicenter study, conducted on 242 patients with Guillana Barre syndrome, intravenous administration Corticosteroids in a high dose (methylprednisolone, 500 mg per day for 5 days) does not affect any of the indicators that estimated the outcome of the Guillana Barre syndrome, as well as on the likelihood of its relapse. In the subsequent open study, during which 25 patients with Hyien-Barre syndrome were treated in / in the administration of immunoglobulin (0.4 g / kg / day for 5 days) and methylprednisolone (500 mg / day for 5 days), the effect was compared With control data obtained earlier when using one immunoglobulin. With a combination of immunoglobulin and methylprednisolone, recovery occurred better, while 76% of patients by the end of the 4th week an improvement was noted at least one functional level - in the control group, a similar degree of recovery was noted only in 53% of patients. This may indicate that corticosteroids can still play a role in the treatment of Guienen Barre syndrome. In order to clarify this issue and determine whether an essential improvement in the disease is noted if intravenously added intravenous corticosteroids are added to the plasmapheresis or immunoglobulin, randomized clinical trials are needed.

Further maintenance

After the end of the acute period, comprehensive rehabilitation measures are needed, the plan of which is individually depending on the severity of residual symptoms (LFC, massage, etc., while thermal procedures are contraindicated!).

Patients who moved Guillana Barre syndrome should be informed about the need to observe the security regime of at least 6-12 months after the end of the disease. Invalid physical overload, overheating, supercooling, excess insolation, alcohol reception. Also during this period should be refracted from vaccination.

Forecast

Mortality in the Hyien-Barre syndrome is 5%. The cause of death may be respiratory failure, the fatal outcome is also possible due to aspiration pneumonia, sepsis and other infections, thromboembolism of the light artery. Mortality increases significantly with age: in children under 15 years old, it does not exceed 0.7%, while persons over 65 reaches 8.6%. Other adverse predictive factors for full-fledged recovery include prolonged (more than 1 month) period of the IVL, the presence of preceding diseases of the lungs.

Most patients (85%) observe a complete functional recovery for 6-12 months. Persistent residual symptoms remains approximately 7-15% of cases. The predictors of an unfavorable functional outcome are the age of 60 years, the rapidly progressive course of the disease, the low amplitude of the M-response during stimulation in the distal point (implied by the heavy defeat of axons). The recurrence frequency of Guienen-Barre syndrome is approximately 3-5%.

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It's important to know!

Possible causes of insulated damage VI (dischargeable) nerve: diabetes, arterial hypertension (with these forms, paralysis of the nerve has a benign current and is usually subjected to reverse development for 3 months), aneurysms, strokes, metastases, pituitary adenoma, sarcoidosis, gianta Arteritis, scarm sclerosis, syphilis

Hyien-Barre syndrome (acute inflammatory demyelinating polyradiculoneuropathy) (G61.0) is an acutely developing autoimmune inflammatory disease The peripheral nervous system, characterized by the acute demyelinization of the roots of spinal and cranial nerves, clinically manifested by the paresthesies of the limbs, the weakness of muscles and / or sluggish paralysis.

The prevalence of the disease: 1-1.9 per 100 thousand people. Debit the disease is observed aged 30-50 years.

The causes of the disease are unknown, therefore, the syndrome is otherwise called idiopathic polyneuropathy. In the development of the disease, immuno-mediated factors are important. 2 weeks before the appearance of the symptoms of the disease, most of the patients celebrate the symptoms of respiratory or gastrointestinal infection.

Symptoms of Guienen Barre Syndrome

Symptoms of the disease appear sharply. Most patients are detected by pain syndrome (up to 80%) and paresthesia (up to 20%). Weakness in the legs, then in the hands, the muscles of the torso increases for several days (90%). Muscular weakness develops quickly, but stops progressing within 4 weeks after the start of the disease. Numbness, pain in the feet, brushes, sometimes around the mouth worried from the beginning of the disease (70%). Weakness in the muscles of the face, disrupting swallowing and respiration appear after 1-2 weeks. In 30% of patients, violation of the sphincter functions may be noted.

With an objective inspection, symmetrical sluggish predominantly distal tetrapapes (lower parapsum) is detected, up to tetraplegia; paresthesia, hyperesthesia in the type of "socks", "gloves"; Palpation diseases in the course of nervous trunks (up to 100%). In 30% of cases, symptoms of tension (Lasega, Nerry) can be determined. It is characteristic of a sharp oppression or discharge of deep reflexes. In 60-80% of cases, bulbar violations, parires of mimic muscles are noted. Typically, the defeat of the sympathetic nervous system, which is manifested by the disassembly disorders (profuse of sweating, hypertension, postural hypotension, etc.). Development of respiratory failure (diaphragm and respiratory dyees) and violation of the heart rhythm can threaten life (30%).

Diagnostics

• The study of the liquor (protein-cell dissociation, from the 2nd week - a moderate increase in protein content).
• Serological blood tests on infection.
• ENG (primary demyelinizing defeat).
• Arterial pressure monitoring, ECG, study of the function of external respiration.

Differential diagnosis:

• Other polyneuropathy (with diphtheria, porphyria).
• Cross Melit.
• Acute violations of the cerebral circulation in the vertebobasilar pool.

Treatment of Guillana Barre Syndrome

Treatment is assigned only after confirming the diagnosis by a specialist doctor. It requires maintenance of vital functions (IVL), holding plasmapheresis, pulse therapy with immunoglobulin class G.

Basic drugs

There are contraindications. Consultation of a specialist is necessary.

• (Immunoglobulin human class G). Dosing mode: administered intravenously at a dose of 0.4 g / kg once a day for 5 days.
• (Nonteroid anti-inflammatory means). Dosing mode: in / m - 100 mg 1-2 times a day; After the relief of pain syndrome is prescribed inside in a daily dose of 300 mg in 2-3 reception, supporting dose of 150-200 mg / day.
• (Anticonvulsant). Dosing mode: inside, starting from 0.1 g 2 times a day, then the dose is raised by 0.1 g per day to 0.6-0.8 g (in 3-4 receptions). After the disappearance of pain, the dose is gradually reduced to 0.1-0.2 g per day.
• (sedative, sleeping pills, antihistamine). Dosing mode: V / m 1-5 ml of 1% solution. Inside 0.025-0.05 g 1-3 times a day. Course of treatment 10-15 days.
• Prezero (inhibitor of acetylcholinesterase and pseudocholinesterase). Dosing mode: inside adults 10-15 mg 2-3 times a day; subcutaneously - 1-2 mg 1-2 times a day.

Hyien-Barre syndrome is an acute form of progressive inflammatory polyneuropathy, which is characterized by muscle weakness and polynelectric sensitivity disorder. The disease is also called idiopathic polyneurite in acute form, Landry paralysis or inflammatory demyelinizing polyradiculoneropathy. NEUCH is a representative of autoimmune anomalies. Usually pathology has specific signs that allow you to recognize it on the most early timing Development and start adequate treatment in time. It has been proven that more than 80% of the diseases have a favorable forecast and are fully cured.

autoimina demyeelination of nerves in the syndrome of Guien-Barre

Guillana Barre (SGB) syndrome is observed in all age groups, but people aged 35-50 years old are particularly susceptible to him, and with equal frequency of both women and men. The incidence of 100,000 people ranges from 0.4 to 4 cases.

Causes of disease

Scientists of different countries are studying the syndrome for over 100 years, but to find out the exact causes provoking the occurrence of the disease, still can not fully.

It is believed that the emergence and development of an anomaly occurs due to a violation of the patient's immune system. When a person is completely healthy, when you hit alien cells to the body, immunity begins to fight with infection, rejecting all its dangerous elements. Patient recovers. When SGB, the body begins to confuse "his and strangers": the patient's neurons are accepted as foreign and exposed to "attack." The destruction of the nervous system occurs - syndrome occurs.

Because of what disorders occur in the work of the immune system itself to the end unknown. To the most common reasons, experts include:

• Heart injuries. A strong hit head, any damage, as well as swelling, tumors or hemorrhage to the brain can be the main factor in the development of the syndrome. That is why when treating a patient to a specialist, first of all, the doctor should learn about the presence of any brain damage.
• Infections. Recently transferred viral infections The immunity of a person is strongly weakened, thereby increasing the likelihood of the appearance of the SGB. The protective mechanism of the body perceives neurons as an infection and with leukocytes continues to kill them. In this case, the syndrome is manifested one or three weeks after an infectious disease.
• Allergies. The disease is often developing in allergies, for example, after chemotherapy, vaccination against poliomyelitis and diphtheria or severe surgical interventions.
• Genetic predisposition. Most diseases are inherited, no exception to the Hyien Barre syndrome. If someone has already suffered pathology in the family, then, most likely, it will arise in the descendants. In this case, you need to especially follow your health: take care of your head and try not to launch infectious diseases.

IN childhood The syndrome develops extremely rarely. The disease may have a congenital or acquired character. Any anomalies of intrauterine development are able to lead to the development of the syndrome:

1. Gestosis;
2. Frequent reception of drugs during pregnancy;
3. Availability autoimmune diseases Mother;
4. Long-term infections during the baby tooling;
5. Drug use, alcohol or smoking.

For acquired reasons can be attributed:

1. Passive smoking of the child;
2. Hormonal malfunctions in the body;
3. Vaccinations;
4. Self-treatment;
5. Metabolic disease;
6. Neurological disorders;
7. Development of tumor processes.

Symptoms

The disease can be manifested by 3 forms:

• Acute. All signs of the disease appear simultaneously within 1-2 days.
• Subacute. The incubation period is from 15 to 20 days.
• Sluggish, chronic. This is one of the most hazardous formsSince it is practically not amenable to treatment.

The first symptoms of the SGB of both children and adults are reminded by an ordinary viral respiratory infection:

1. Slabs in the bones, joints;
2. Hyperthermia;
3. Severe weakness;
4. Inflammation of the upper respiratory tract;
5. Numbness of the limbs;
6. Sometimes patients are worried about various gastrointestinal disorders.

In addition to general features, more pronigible:

• Weak limbs. Due to the destruction of nervous cells, there is a decrease or complete loss of sensitivity in the muscles. At first, the pain appears only in a hunted part of the legs, after - discomfort affects the hand and footbrush. The patient concerned the tingling and numbness of the fingers. In severe cases - the coordination of movements is disturbed: it becomes difficult for a person to keep the pen on behalf of themselves, to write an affected limb. It is worth noting that the symptoms of the disease appear symmetrically: at the same time 2 hands or legs are affected.
• An increase in the abdomen, which is noticeable even visually. The abdicted belly is one of the main indicators of the presence of the disease. This is due to the fact that the breath of the patient is rebuilt on the abdominal type due to the weakening of the diaphragm.
• Incontinence. A person with the SGB syndrome is disturbed by the healthy functioning of the bladder, urine begins to flow involuntarily.
• Difficult swallowing. The swallowing reflex is broken due to the fact that the throat muscles are weakened. In this case, the patient can chollow even saliva. Muscles of the mouth gradually weaken, which leads to discomfort while chewing food.

Hayen-Barre syndrome affects almost all organ systems, so unreasonable hypertension, tachycardia or banal impaired impacts can be the first, albeit hidden, sign of the development of pathology.

What is dangerous the flow of syndrome?

Usually anomaly develops slowly for 2-3 weeks. First, there is a light weakness in the joints, which in time is enhanced and really begins to deliver the patient's discomfort.

Immediately after tingling, in the acute course of the disease, there is general ailment, weakness in the shoulder and hip departments arises. After a few hours, difficulty in breathing appear. In this case, it is necessary to seek help in the hospital. Typically, the patient is immediately connected to the system of artificial respiration, and after the necessary drug and physiotherapeutic treatment have necessary.

In the acute form of the disease, pathology already on the second or third day can fully paralyze any limb.

Also, in the absence of timely treatment, the patient threatens:

1. Decrease in immunity;
2. Respiratory failure;
3. Suspended joints;
4. Peripheral paralysis;
5. Problems of adaptation in society;
6. Difficulty of life;
7. Disabled;
8. Fatal outcome.

Diagnosis of the disease

In order to make a diagnosis of the SGB to the patient, it is necessary to clarify several aspects:

• When the last time a person was sick with any viral disease. It has been proven that in 80% of cases, the syndrome arises due to recently suffered infections.
• Does patient drug preparations at present and if so, then what. They will also help to cause SGB development.
• How long ago the patient was taken from any disease.
• Whether the patient suffers with autoimmune or tumor diseases.
• Whether a person has transferred surgery recently.
• Whether there were serious injuries to any part of the body.

The following studies should also be conducted:

1. General analysis of blood and urine;
2. Blood chemistry;
3. Serological and virological surveys;
4. Study of the spinal fluid;
5. Magnetic resonance tomography;
6. Electrocardiography;
7. Registration of electrical activity of muscles;
8. X-ray or ultrasound affected area;
9. Study of external breathing;
10. The study of the main vital indicators.

Muscular weakness at the same time in several limbs and terfluxia of tendons can be another obvious sign of Guillana Barre syndrome. This also includes various disorders in the field of pelvis, polymorphic leukocytes, asymmetry of paresis and sensitivity disorders.

Differential diagnosis

Despite the fact that the symptoms of the SGB are similar to the signs of many other diseases (diphtheria, porphyria, transverse myelitis, botulism and myasthenia), they are still needed to be distinguished for further faithful treatment. In differential diagnostics, it is worth considering the following factors:

• In suspected polio, it is necessary to collect data from epidemiological research, take into account the symptoms from the gastrointestinal tract, to identify high cytosis in the spinal fluid, the asymmetry of the lesion and the absence of sensitivity disorders. You can confirm the diagnosis using serological or virological analysis.
• Polynevropathy is characterized by the appearance of psychopathological signs, as well as pain in the field of pelvis and abdomen. The deviation of the main indicators from the norm in the urine also testifies to the development of the ailment.
• Transverse Melitis accompanies the violation of the functioning of the pelvic organs, the lack of damage to the nerves of the skull.
• The symptoms of the anomalies can be confused with the heart attack of the brain. But in this case, pathology is striking the body in a few minutes and often leads to a coma. MRI will help to establish the exact cause of violation of the functioning of the body's systems.
• Botulism is distinguished by the lack of sensitivity disorders and any changes in the liquor.

Treatment

Patients with a diagnosis of SGB must be hospitalized in the hospital. About 30% of cases it is necessary to hold an IVL. Pathology therapy is performed at the following levels:

1. Resuscitative;
2. Symptomatic;
3. Blood purity;
4. Preparation;
5. Muscular restoration;
6. Preventive.

Resuscitation therapy

If an anomaly is in acute form, a resuscitation treatment is carried out, which is aimed at relieving symptoms:

• The patient is connected to an artificial respiratory system;
• Apply a catheter to remove urine;
• Install the trachene tube and probe if there are problems with swallowing.

Symptomatic therapy

This type of treatment is carried out using various medicines:

1. Antihypertensive means: "Anaprilin", "Metaprolol";
2. Antibiotics therapy: Norfloxacin;
3. Preparations that help stabilize heart rhythm and pressure: "Propranolol", "Anaprilin" (with tachycardia), "piracetam" (in bradycardia);
4. Low molecular weight heparin: "Gemapaxan", "Tortopar";
5. Preparations that buy pain - NSAIDs or Gabapentin, "Prestabalin";
6. Antipyretic when the temperature rises above 38 degrees: "Ibuklin", "Nex";
7. Laxative means: "Bisakodil", "Laksatin".

Plasmapheres

One of the most effective procedures aimed at the treatment of SGB is the hardware cleaning of blood - plasmapheresis. It helps to stop the autoimmune process in the body. It is shown in the heavy and medium-spent the course of the disease. Typically spend about 4-6 operations with a break in one day. Instead of plasma, a special isotonic solution of sodium or albumin is introduced into the blood, by which the blood is cleared and normalizes the functioning of all organism systems.

Operational treatment of syndrome

If the ventilation was carried out more than 7-10 days, the trachestoma should be imposed - artificial respiratory throat. In severe cases, a gastrostrome may be required - a hole in the stomach created by the operational way for feeding the patient.

Non-media therapy by folk remedies

It is impossible to cure a SGB of folk remedies. But it is quite realistic to cope with some of his symptoms:

• Increased temperature. It is recommended to drink and ventilating the room. To bring down the temperature will help tea with lemon, decoctions with different berries and dried fruits: cranberries, strawberries, currant, blueberries, raspberry berries and Kuragoy. You can boil the lime color, chamomile, St. John's wort, aspen kidneys, mint and a chamber - to leave for half an hour, after - drinking small sips.
• Sounds in the bones. Credit with her will help cubber tea, compress from fresh cabbage leaves, horseradish and burdock, bath with coniferous extract or with decoctions of medicinal herbs.
• Weakness. Doctors recommend to breathe fresh air as often as possible and ventilated the room. You need to try to use a larger amount of proteins. Also necessary food rich in vitamins and microelements. And sweet strong tea or chocolate will help raise the mood.

Rehabilitation

Due to the fact that the syndrome is striking not only neurons, but also the near muscles, the patient will have to learn again and perform simple movements of the limbs.

To normalize the healthy functioning of the muscles, you can use traditional treatment, which includes:

1. Electrophoresis;
2. Trituration;
3. Baths with radon;
4. Massage;
5. Baths for relaxation of the body and muscle tone;
6. Masks and compresses with paraffin or bees wax;
7. Health gymnastics.

During the restoration of the body, it is necessary to sit on a special medicinal diet and in parallel to take a course of vitamin preparations. Especially useful will be complexes, which contains calcium, potassium, magnesium and vitamin B.

Patients with SGB should be registered with a neurologist and regularly undergo preventive examinations. It is worth remembering that timely qualitative therapy is able to return the patient to a full-fledged life.

Prevention of syndrome

There is no special prevention of pathology. Doctors can only advise to abandon any vaccinations during the year so that the disease does not return to be returned. After this time, vaccination is allowed, but only if it is valid.

It is also worth refusing to receive alcoholic beverages, avoid overheating, supercooling and reduce physical exercise. In this case, the recurrence of the disease is excluded.

Forecast

Most often, when Hyien-Barre syndrome, the forecast is favorable. Usually, the normal functioning of the limbs is restored already after 7-12 months in 85% of people. In chronic form, the disease passes in 7-15% of cases. Flower outcome is approximately 5%. The cause of death can be respiratory failure, pneumonia or viral infections. But most often all this can be prevented, bypassing a specialist in time.

Video: Lecture on Hyien Barre syndrome

Video: Guienen-Barre syndrome in the program "Live Great"