Guillain barre treatment. Guillain-Barré syndrome - symptoms and treatment. Data from additional research methods

Piradov M.A. 2000 year

Rehabilitation is possible
This disease has at least eight different names - Landry syndrome (named after the French neurologist who first described it in 1859), Guillain-Barré-Strohl syndrome (scientists who made a significant contribution to the study of the disease), acute polyradiculoneuritis, etc. Today according to the International Classification of Diseases, it is officially called Guillain-Barré syndrome (GBS) or acute post-infectious polyneuropathy. In neurology, GBS is considered a unique disease. And not so much because of its relative rarity (it occurs in 2 people per 100 thousand of the population), but because of the possibility of complete rehabilitation of the patient, although sometimes the severity of GBS lesions is comparable to the most serious diseases. The Deputy Director for Science of the Research Institute of Neurology of the Russian Academy of Medical Sciences, Head of the Department of Neuroresuscitation, Professor Mikhail PIRADOV tells in more detail. Guillain-Barré syndrome is the most common reason acute peripheral tetraparesis and paralysis. Neurological symptomatology develops very quickly, while disturbed, and sometimes very roughly, not only motor, but also sensory functions (primarily articular-muscular sensitivity), tendon reflexes are reduced to the point of complete extinction. Pelvic disorders are not characteristic of GBS, but in a third of those affected, the respiratory and swallowing muscles are seriously affected. V severe cases a person appears before the doctor, lying motionless in bed, who cannot breathe at all, swallow and even open his eyes. But if an electroencephalogram is taken from a patient at the same time, it will be the same as that of a healthy person, and as a personality he is not intellectually changed in the least. In 70 percent. cases of GBS occur a few days after the onset of flu-like phenomena: moderate fever, muscle pain, runny nose - all that is usually called acute respiratory infections. At about 15 percent. cases, the syndrome appears after profuse diarrhea, in 5 percent. - after surgical procedures, be it abortion, hernia repair, appendectomy or more complex operations. Sometimes the disease develops after various types of vaccinations. GBS occurs in any region of the world, at any time of the year, and is equally common in both sexes. The average age in most observations is about 40 years. At the same time, two small age peaks stand out: at 20-25 years old and over 60 years old. In classical cases, the diagnosis of GBS is simple and includes two mandatory signs: increasing muscle weakness in at least two limbs and a significant decrease, up to the complete loss of tendon reflexes. Additional diagnostic criteria are a decrease in the speed of conduction of nerve impulses through the muscles with the formation of a conduction block and protein-cell dissociation in the cerebrospinal fluid. At the heart of Guillain-Barre syndrome are auto immune mechanisms where the role of the triggering factor is assigned to certain viruses and bacteria. However, there is still no final opinion on the nature of the antigen or antigens that cause the development of cascade immune reactions. In the last five years, it has been established that under the name GBS a whole spectrum of polyneuropathies is combined: acute inflammatory demyelinating polyneuropathy (occurs in 75-80% of cases); acute motor neuropathy and, as its variant, acute motor-sensory axonal neuropathy (15-20 percent); Fisher's syndrome (3 percent). Most autoimmune diseases are irreversible. But with GBS, the picture is completely different, unique: this disease is self-limiting. If a seriously ill patient is carried out for several months only artificial ventilation lungs, the affected nerves are restored. And almost as completely as when applying the basic modern methods treatment of GBS - plasmapheresis or intravenous therapy with class G immunoglobulins. The question may arise: why treat a patient with expensive methods? But imagine what it means to be on ventilator for 3-6 months and be bedridden? Timely use of plasmapheresis and class G immunoglobulins can reduce the time spent on mechanical ventilation to several weeks and even days, fundamentally change the course and outcome of the disease. It is no secret that today many patients with severe forms of GBS die in the country. This is largely due to the fact that many hospitals are not equipped with high-quality breathing apparatus or do not have qualified personnel for long-term mechanical ventilation. Patients die due to common infections and bedsores. In addition, it is not always possible to perform plasmapheresis operations with replacement of large plasma volumes (up to 200 ml of plasma / kg for a course of treatment consisting of 4-5 operations). It is completely unacceptable to treat such patients in a rural or small district hospital- they need to be hospitalized in larger hospitals equipped with the necessary means and equipment. A typical mistake in many cases is the treatment of patients with GBS with hormonal drugs: special studies more than one thousand patients have shown that hormones do not affect the rate of restoration of impaired functions, but, on the contrary, carry many complications. However, hormones continue to be unreasonably used even in a number of clinics in the largest Russian cities. Abroad, for this they can simply deprive a doctor's license. If we talk about the financial side of the matter, of course, today for most patients, treatment with imported class G immunoglobulins, which are widely used in the West, is simply not affordable, but, fortunately, the course of programmed plasmapheresis in our country is much cheaper. And the therapeutic effect of these two methods of treatment is the same: about 85-90 percent. cases, a person with Guillain-Barré syndrome, despite severe damage to the peripheral nervous system, recovers completely, and only 10-15 percent. patients experience residual effects. Of course, the prevalence of Guillain-Barré syndrome is incomparable with stroke, traumatic brain injury or epilepsy. But with a stroke, at best, 20 percent is restored. people, and timely treatment of Guillain-Barré syndrome with no less severity of the lesion gives a much greater effect. And if about 200 people suffer annually in Moscow alone, the SGB suffers, fully recovering 180 health is a lot. In my practice, there was a case when a disease struck an 18-year-old guy, a candidate for master of sports in athletics: he could not breathe, swallow, move by himself. A year later, this person fulfilled the standard of the master of sports. And there are many such examples - young women after proper GBS treatment give birth to children, the overwhelming majority of patients return to full-fledged life.

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Guillain-Barré syndrome (acute idiopathic polyneuritis; Landry palsy; acute inflammatory demyelinating polyradiculoneuropathy) is an acute, usually rapidly progressive inflammatory polyneuropathy characterized by muscle weakness and moderate loss of distal sensation. Autoimmune disease. Diagnosis by clinical findings. Treatment of Guillain-Barré syndrome: plasmapheresis, γ-globulin, if indicated, artificial ventilation of the lungs. The outcome of the syndrome improves significantly with adequate supportive treatment in the intensive care unit and the use of modern methods of immunomodulatory therapy.

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ICD-10 code

G61.0 Guillain-Barré syndrome

Epidemiology

The incidence ranges from 0.4 to 4 cases per 100,000 population per year. Guillain-Barré syndrome is observed in all age groups, but more often in persons 30-50 years old, with equal frequency in men and women. Racial, geographic and seasonal differences in incidence are generally uncommon for Guillain-Barré syndrome, with the possible exception of acute axonal motor neuropathy, which is most common in China and is usually associated with intestinal infection due to Campylobacter jejuni and therefore occur somewhat more frequently in the summer.

The incidence increases significantly after 40 years. An average of 600 people die from Guillain-Barré syndrome in the United States each year. Thus, Guillain-Barré syndrome is a very important health problem, especially for the elderly.

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Causes of Guillain-Barré Syndrome

The most common acquired inflammatory neuropathy. The autoimmune mechanism is not fully understood. Several options are known: in some, demyelination predominates, in others, the axon suffers.

In about 2/3 of cases, the syndrome appears 5 days - 3 weeks after an infectious disease, surgery or vaccination. In 50% of cases, the disease is associated with an infection Campylobacter jejuni, enteroviruses and herpes viruses (including cytomegalovirus and viruses that cause mononucleosis), and Mycoplasma spp. In 1975 there was an outbreak associated with the swine flu vaccination program.

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Pathogenesis

Demyelination and inflammatory infiltration in the spinal nerve roots and proximal nerves may explain clinical symptoms Guillain-Barré syndrome. It is believed that both humoral and cellular immunity are involved in the pathogenesis of the disease. The presence of lymphocytes and macrophages in the perivenous zones and their interaction with myelinated axons indicate, first of all, the possible role of autoimmune reactions in the demyelinating process. This position is confirmed by earlier observations, according to which immunization of laboratory animals with peripheral myelin with an adjuvant causes experimental allergic neuritis. Although purified myelin proteins - for example, myelin basic protein P2 or peptide fragments P2 and PO protein - have been shown to induce experimental neuropathy, antibodies to these compounds are rarely found in Guillain-Barré syndrome. T cells isolated from the spleen and lymph nodes rats immunized with P2-synthetic peptide 53-78 can experimentally reproduce severe experimental allergic neuritis in syngeneic mice. Thus, cellular and possibly humoral immune mechanisms can mediate the creation of experimental model inflammatory damage to peripheral nerves.

Recent studies have drawn attention to the role of glucoconjugates and lipopolysaccharides of the myelin sheath, Schwann cell membrane, or axonal membrane as major antigens initiating the inflammatory / immune response in Guillain-Barré syndrome. In a detailed study conducted in Japan, antigens were identified in patients Campylobacter jejuni... In this study, the Penner method was used to identify thermostable lipopolysaccharides and the Lior method was used to determine the thermolabile protein antigens. Antigens PEN 19 and LIO 7 S. jejuni were more often isolated in patients with Guillain-Barré syndrome (in 52 and 45% of cases, respectively) than in patients with sporadic enteritis caused by S. jejuni(5 and 3%, respectively), and were associated with an increase in the titer of antibodies to GM1 (possibly due to the presence of a GMl-like lipopolysaccharide antigen). Infection reported from other countries S. jejuni much less often precedes the development of GBS. In addition, the percentage of patients with antiganglioside antibodies was much more variable, ranging from 5% to 60%. In addition, no correlation was found between the presence of antibodies to GM1 and the clinical and electrophysiological manifestations of the disease.

Antibodies to GQlb are often found in Miller Fisher syndrome. Using immunohistochemical methods, GQlb was detected in the paranodal region of human cranial nerves that innervate the eyes. It has been established that antibodies to GQlb can block transmission in the neuromuscular system of mice.

In the axonal motor variant of Guillain-Barré syndrome, the disease was more often preceded by infection with C. jejuni, and antibodies to GM1 ganglioside and the complement activation product C3d were associated with the motor fiber axolemma.

Anti-GMI antibodies can also bind to Ranvier interceptions, thus disrupting impulse conduction. In addition, these antibodies are capable of causing degeneration of motor fiber endings and intramuscular axons, which has recently been shown in patients with acute motor axonal polyneuropathy. C. jejuni enteritis can provoke Guillain-Barré syndrome by increasing the production of gamma-delta-T lymphocytes, which can actively participate in inflammatory / immune processes. High serum levels of tumor necrosis factor-alpha (TNF-a), but not interleukin-1b or soluble interleukin-2 receptor, correlated with electrophysiological changes in Guillain-Barré syndrome. Examination of autopsy specimens indicates that at least In some cases of the classic acute inflammatory demyelinating form of Guillain-Barré syndrome, complement is involved - this is indicated by the detection of C3d and C5d-9 components on the outer surface of Schwann cells, which form a membrane-attacking complex.

Thus, in Guillain-Barré syndrome, most of the components usually involved in the pathogenesis of immune-mediated diseases are represented. Although antibodies against glucoconjugates are likely to be involved in the pathogenesis of several different clinical forms of Guillain-Barré syndrome, their precise role is unknown. Even if antibodies to GM1 are present, they can bind not only to GM1, but also to other glycolipids or glycoproteins that have similar carbohydrate regions. In this regard, the specific antigens of Schwann 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 of Guillain-Barré syndrome, there are no signs of prior or concomitant infection. S. jejuni, antibodies to GM1 or traits of another microorganism, whose antigens can initiate an immune response (for example, through molecular mimicry).

Investigation of material obtained from nerve biopsies and autopsies shows that cellular immune mechanisms also contribute to the development of Guillain-Barré syndrome. In severe cases of Guillain-Barré syndrome, lymphocytes and macrophages are present along the entire length of the motor fibers from the roots to the endings, and the activated macrophages are in close contact with myelin or phagocytose myelin. Although the experimental model of inflammatory neuropathy provides evidence that T-lymphocytes are involved in nerve damage, there is no convincing evidence that this occurs in patients with Guillain-Barré syndrome. The data accumulated to date confirm the involvement of activated T-lymphocytes that cross the blood-brain barrier and initiate demyelination together with antibodies to specific antigens of nerve fibers, cytokines (such as TNF-a and interferon-y), complement components, possibly including a membrane attack complex and activated macrophages ... More 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 Guillain-Barré syndrome.

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Guillain-Barré Syndrome Symptoms

Symptoms of Guillain-Barré syndrome are dominated by flaccid paresis (the more proximal, the deeper), sensitivity disorders are less pronounced. Typically, almost symmetrical weakness with paresthesias begins in the legs, less often in the arms or head. In 90% of cases, weakness reaches its maximum at the 3rd week of the disease. Deep tendon reflexes are lost. The sphincter function is preserved. In severe cases, weakness of the facial and oropharyngeal muscles is evident in half of the cases. In 5-10% of cases, intubation and mechanical ventilation are required due to paralysis of the respiratory muscles.

Sometimes (apparently, with a variant form), pronounced autonomic dysfunction develops with fluctuations in blood pressure, pathological secretion of antidiuretic hormone, arrhythmias, intestinal stasis, urinary retention and impaired pupil response to light. Fisher's syndrome is a rare variant of Guillain-Barré syndrome and suggests ophthalmoplegia, ataxia, and areflexia.

The first symptoms, the order of their appearance and dynamics

In typical cases, Guillain-Barré syndrome begins with muscle weakness and / or sensory disorders (numbness, paresthesia) in the lower extremities, which spread to the upper extremities after a few hours or days.

The first symptoms of Guillain-Barré are sensory disturbances, for example, paresthesia in the feet. Though objective signs sensitivity disorders are detected quite often, they are usually mild. Early and extremely unpleasant manifestations of the disease for patients can be deep aching pain in the back and painful dysesthesia in the extremities. Paralysis can initially involve the lower extremities, and then quickly, over several hours or days, spread in an ascending direction to the upper extremities, facial, tabloid and respiratory muscles. However, a different development of events is possible, when the disease begins with weakness in the facial muscles and upper limbs, then involves the lower limbs. From the very beginning, symptoms are usually symmetrical, and paralysis is accompanied by prolapse or weakening of tendon and periosteal reflexes. In Guillain-Barré syndrome, autonomic fibers are often involved. Autonomic symptoms occur in about 50% of cases, but sphincter function is usually not affected. The disease has a monophasic course: after a period of increase in symptoms, lasting for several days or weeks, there is a plateau period lasting from several days to several months, after which recovery occurs over several months. In 1976-1977, there was a slight increase in the incidence of Guillain-Barré syndrome associated with immunization with the swine influenza vaccine, however, when immunization with another variant of the influenza vaccine in 1980-1988, a similar phenomenon was not recorded.

In classic cases, manifested by a combination of motor, sensory and vegetative symptoms, which are based on demyelinating polyradiculoneuropathy, the diagnosis of Guillain-Barré syndrome is rarely difficult. However, there are also axonal variant of Guillain-Barré syndrome, mainly manifested by motor disorders, and acute motor-sensory axonal neuropathy. The acute axonal form usually presents with a more severe functional defect and has a poorer prognosis. The combination of ophthalmoplegia, ataxia and reflexia is characteristic of another variant of Guillain-Barré syndrome, known as Miller Fisher syndrome. From a diagnostic point of view, in the absence of symptoms of cranial nerve damage, even with intact sphincter function, it is necessary to exclude spinal cord compression using neuroimaging. In differential diagnosis, it is also important to keep in mind acute intermittent porphyria, metal intoxication that can cause acute polyneuropathy, as well as systemic diseases such as infectious mononucleosis, paraneplastic syndromes or various metabolic disorders... HIV-infected patients are predisposed to the development of polyneuropathy or polyradiculoneuropathy, which may be associated with Guillain-Barré syndrome, cytomegalovirus polyradiculoneuropathy, or lymphoma. These conditions are difficult to differentiate based on clinical presentation alone, but CSF examination in HIV-associated polyradiculoneuropathy usually reveals neutrophilic pleocytosis and signs of viral replication.

Autonomic dysfunction (including disorders of accommodation, pain in the abdomen and chest, arterial hypotension, tachycardia) can significantly aggravate the patient's condition and serves as an unfavorable prognostic sign. In one study, subclinical signs of involvement of both the sympathetic and parasympathetic nervous systems, detected using tests for autonomic functions, were noted in the vast majority of patients.

North American Motor Deficit Severity Scale

• A third of patients develop respiratory failure.
• In most cases, there are disorders of superficial sensitivity in the form of mild or moderate hypo- or hyperesthesia of the polyneuritic type (like "socks and gloves"). Pain in the hips, lumbar and gluteal regions is often noted. They can be either nociceptive (muscle) or neuropathic (due to damage to sensory nerves). Disorders of deep sensitivity (especially vibrational and muscular-articular feelings), which are very gross (up to complete loss), are detected in about half of patients.
• Cranial nerve lesions are observed in most patients. It is possible to involve any cranial nerves in the process (with the exception of pairs I and II), but the most consistent damage is observed in pairs VII, IX and X, which is manifested by paresis of facial muscles and bulbar disorders.
• Vegetative disorders are observed in more than half of patients and can be represented by the following disorders.
  • Transient or persistent arterial hypertension or, more rarely, arterial hypotension.
  • Cardiac arrhythmias, most commonly sinus tachycardia.
  • Sweating disorder [local (palms, feet, face) or general hyperhidrosis].
  • Dysfunction of the gastrointestinal tract (constipation, diarrhea, in rare cases, intestinal obstruction).
  • Dysfunctions of the pelvic organs (usually urinary retention) are rare and are usually mild and transient.
• In Miller-Fisher syndrome, the clinical picture is dominated by ataxia, which usually has features of the cerebellar, in rare cases - mixed (cerebellar-sensitive), and partial or total ophthalmoplegia, possibly also affecting other cranial nerves (VII, IX, X). Paresis are usually mild, in a quarter of cases there are sensory disorders.

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Diagnostic criteria for Guillain-Barré syndrome

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Signs of Guillain-Barré Syndrome Required for Diagnosis

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

Signs of Guillain-Barré Syndrome Supporting the Diagnosis

• A. Clinical signs (listed in order of importance)
  • Progression: Muscle weakness develops rapidly, but stops progressing within 4 weeks after the onset of the disease.
  • Relative symmetry: symmetry is rarely absolute, but when one limb is affected, the opposite is also affected (comment: patients often report asymmetric symptoms at the onset of the disease, however, by the time of physical examination, the lesions are usually symmetrical).
  • Subjective and objective symptoms of sensitivity disorders.
  • Damage to the cranial nerves: paresis of the facial muscles.
  • Recovery: Usually begins 2-4 weeks after the disease stops progressing, but sometimes it can be delayed for several months. In most patients, complete recovery of functions is observed.
  • Autonomic disorders: tachycardia and other arrhythmias, postural arterial hypotension, arterial hypertension, vasomotor disorders.
  • The absence of fever at the onset of the disease (in some cases, fever at the onset of the disease is possible due to intercurrent diseases or other reasons; the presence of fever does not exclude Guillain-Barré syndrome, but increases the likelihood of having another disease, in particular poliomyelitis).
• B. Options
  • Severe sensory disorders with pain.
  • Progression over 4 weeks. Sometimes the disease may progress over many weeks or have minor relapses.
  • Cessation of progression without subsequent recovery or preservation of severe persistent residual symptoms.
  • Function of the sphincters: Usually the sphincters are not affected, but in some cases, urinary disturbances are possible.
  • Damage to the central nervous system: in Guillain-Barré syndrome, the peripheral nervous system is affected, there is no reliable evidence of the possibility of damage to the central nervous system. Some patients have gross cerebellar ataxia, abnormal extensor foot signs, dysarthria, or an indistinct level of sensory impairment (implying a conductive type of impairment), but these do not rule out the diagnosis of Guillain-Barré syndrome if other typical symptoms are present.
• C. CSF changes confirming the diagnosis
  • Protein: 1 week after the onset of the disease, the concentration of protein in the cerebrospinal fluid becomes elevated (during the first week it may be normal).
  • Cytosis: the content of mononuclear leukocytes in the cerebrospinal fluid is up to 10 in 1 μl (if the content of leukocytes is 20 in 1 μl or more, a thorough examination is necessary. If their content is more than 50 in 1 μl, the diagnosis of Guillain-Barré syndrome is rejected; the exception is patients with HIV infection and Lyme borreliosis).

Signs of Guillain-Barré syndrome that raise doubts about the diagnosis

1. Pronounced persistent asymmetry of paresis.
2. Persistent pelvic disorders.
3. The presence of pelvic disorders at the onset of the disease.
4. The content of mononuclear leukocytes in the cerebrospinal fluid is more than 50 in 1 μl.
5. The presence of polymorphonuclear leukocytes in the cerebrospinal fluid.
6. A clear level of sensitivity disorders

Signs of Guillain-Barré Syndrome Ruling Out the Diagnosis

1. The current abuse of volatile organic solvents (substance abuse).
2. Porphyrin metabolism disorders, implying a diagnosis of acute intermittent porphyria (increased urinary excretion of porphobilinogen or aminolevulinic acid).
3. Recently transferred diphtheria.
4. The presence of symptoms of neuropathy due to lead intoxication (paresis of the muscles of the upper limb, sometimes asymmetric, with severe weakness of the extensors of the hand) or evidence of lead intoxication.
5. The presence of purely sensory impairments.
6. Reliable diagnosis of another disease, manifested by symptoms similar to Guillain-Barré syndrome (poliomyelitis, botulism, toxic polyneuropathy).

Forms

Currently, within the framework of Guillain-Barré syndrome, four main clinical variants are distinguished.

• Acute inflammatory demyelinating polyradiculoneuropathy is the most common (85-90%), classic form of Guillain-Barré syndrome.
• Axonal forms of Guillain-Barré syndrome are observed much less frequently (10-15%). Acute axonal motor neuropathy is characterized by isolated damage to motor fibers, most common in Asia (China) and South America. In acute motor-sensory axonal neuropathy, both motor and sensory fibers are affected; this form is associated with a prolonged course and a poor prognosis.
• Miller-Fisher syndrome (no more than 3% of cases) is characterized by ophthalmoplegia, cerebellar ataxia and areflexia with usually mild paresis.

In addition to the main ones, several more atypical forms of the disease have recently also been distinguished - acute pandizautonomy, acute sensory neuropathy and acute cranial polyneuropathy, which are very rare.

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Diagnostics of the Guillain-Barré syndrome

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

• The presence of provoking factors. In approximately 80% of cases, the development of Guillain-Barré syndrome is preceded by one or another disease or condition in 1-3 weeks.
• - Infections of the gastrointestinal tract, upper respiratory tract or other localization. The association with intestinal infection caused by Campylobacter jejuni. Individuals who have had campylobacteriosis have an approximately 100 times higher risk of developing Guillain-Barré syndrome within 2 months of the disease than in the general population. Guillain-Barré syndrome can also develop after infections caused by herpes viruses (cytomegalovirus, Epstein-Barr virus, varicella-zoster), Haemophilus influenzae, mycoplasmas, measles, mumps, Lyme borreliosis, etc. In addition, Guillain-Barré syndrome can develop with HIV infection.
• Vaccination (rabies, tetanus, influenza, etc.).
• Surgical interventions or injuries of any location.
• Taking certain medications (thrombolytic drugs, isotretinoin, etc.) or contact with toxic substances.
• Sometimes Guillain-Barré syndrome develops against the background of autoimmune (systemic lupus erythematosus) and neoplastic (lymphogranulomatosis and other lymphomas) diseases.

Laboratory and instrumental research

• General clinical studies (complete blood count, general urine analysis).
• Biochemical analysis blood: serum electrolyte concentration, arterial blood gas composition. When planning specific therapy with class G immunoglobulins, it is necessary to determine the Ig fractions in the blood. Low concentration IgA is usually associated with hereditary deficiency, in such cases, the risk of developing anaphylactic shock(therapy with immunoglobulin is contraindicated).
• Studies of cerebrospinal fluid (cytosis, protein concentration).
• Serological tests with suspicion of the etiological role of certain infections (markers of HIV, cytomegalovirus, Epstein-Barr virus, Borrelia burgdorferi, Campylobacter jejuni etc.). If poliomyelitis is suspected, virological and serological (antibody titer in paired sera) studies are necessary.
• EMG, the results of which are of fundamental importance for confirming the diagnosis and determining the form of Guillain-Barré syndrome. It should be borne in mind that EMG results may be normal during the first week of illness.
• Neuroimaging (MRI) techniques do not confirm the diagnosis of Guillain-Barré syndrome, but may be necessary for differential diagnosis with pathology of the central nervous system (acute cerebral circulation, encephalitis, myelitis).
• Monitoring of the function of external respiration [determination of the vital capacity of the lungs (VC) for the timely identification of indications for transferring the patient to mechanical ventilation.
• In severe cases (especially with the rapid progression of the disease, bulbar disorders, expressed autonomic disorders), as well as during mechanical ventilation, it is necessary to monitor the main vital parameters (in the conditions of an intensive care unit): blood pressure, ECG, pulse oximetry, respiratory function and others (depending on the specific clinical situation and therapy).

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Neurophysiological criteria for the classification of Guillain-Barré syndrome

Normal (all of the following signs should be present in all examined nerves)

1. Distal motor latency
2. F-wave preservation and its latency
3. SRV> 100% of the lower limit of the norm.
4. The amplitude of the M-response when stimulated at the distal point is> 100% of the lower limit of the norm.
5. Amplitude of the M-response with stimulation at the proximal point> 100% of the lower limit of the norm.
6. The ratio "Amplitude of the M-response during stimulation at the proximal point / Amplitude of the M-response during stimulation at the distal point"> 0.5

Primary demyelinating lesion (the presence of at least one of the signs in at least two examined nerves or the presence of two signs in one nerve is necessary if all other nerves are not excitable and the amplitude of the M-response when stimulated at the distal point is> 10% of the lower limit of the norm ).

1. Distapous motor latency> 110% of the upper limit of the norm (> 120% if the amplitude of the M-response during stimulation at the distal point
2. The ratio “Amplitude of the M-response during stimulation at the proximal point / Amplitude of the M-response during stimulation at the distal point” is 20% of the lower limit of the norm.
3. F-wave latency> 120% upper limit of normal

Primary axonal lesion

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

Non-excitability of nerves

• When stimulated at the distal point, the M response cannot be elicited in any of the nerves (or can be elicited only in one nerve with its amplitude

Uncertain defeat

Does not meet the criteria for any of the above forms

This form may include cases of primary severe axonopathy, severe distal demyelination with conduction block, and secondary Wallerian degeneration after demyelination; it is impossible to distinguish them neurophysiologically.

Indications for consulting other specialists

• Treatment of patients with severe forms of Guillain-Barré syndrome is carried out in conjunction with the doctor of the intensive care unit.
• In case of severe cardiovascular disorders (persistent severe arterial hypertension, arrhythmias), a consultation with a cardiologist may be required.

Data from additional research methods

Electromyography (EMG) is of great diagnostic value in Guillain-Barré syndrome. and the study of the speed of conduction of impulses along the nerves, as well as the study of cerebrospinal fluid. Starting from the 3-7th day after the onset of the first symptoms, electrophysiological examination reveals a slowdown in conduction along motor and (to a lesser extent) along sensory fibers, lengthening of the distal latency and latent period of the F-wave, a decrease in the amplitude of the total muscle action potential (M-response ) and sometimes sensory action potentials, as well as focal and asymmetric conduction blocks, which indicate segmental demyelinating polyneuropathy. On the other hand, in acute axonal motor polyneuropathy, the amplitude of sensory action potentials and the velocity of conduction along sensory fibers may be normal, but there is a decrease in the amplitude of the total muscular action potential and only a slight deceleration of conduction along the motor fibers. When both motor and sensory fibers are damaged, both total muscle action potentials and sensory action potentials can be grossly altered, and distal latency and conduction velocity can be difficult to measure, which indicates severe motor-sensory axonopathy. In Miller Fisher syndrome, which is manifested by ataxia, ophthalmoplegia and reflexion, muscle strength remains intact, and EMG and conduction velocities along the nerves of the extremities may be normal.

When researching cerebrospinal fluid in patients with Guillain-Barré syndrome, an increase in the 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 early days of the disease, the protein content in the cerebrospinal fluid may be normal, while an increase in cytosis to 30 cells in 1 μl does not exclude the diagnosis of Guillain-Barré syndrome.

Since a biopsy of the sural nerves, as a rule, does not reveal signs of inflammation or demyelination, this method is not included in the standard set of studies in most patients with Guillain-Barré syndrome, but it can be important in scientific research. Pathomorphological studies indicate that in Guillain-Barré syndrome, the proximal parts of the nerves and the roots of the spinal nerves are mainly affected: it is in them that edema, segmental demyelination, infiltration of endonervium by mononuclear cells, including macrophages, are detected. Mononuclear cells interact with both Schwann cells and myelin sheath. Although Guillain-Barré syndrome is a polyradiculoneuropathy, pathological changes can also be detected in the central nervous system (CNS). In most of the 13 autopsy cases, mononuclear infiltration with lymphocytes and activated macrophages was found in the spinal cord, medulla oblongata, and pons. However, no primary demyelination was detected in the central nervous system. With a long course, the predominant type of inflammatory cells in the central and peripheral nervous system were activated macrophages, in addition, CD4 + and CD8 + T-lymphocytes were detected there.

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Differential diagnosis

Guillain-Barré syndrome must be differentiated from other acute peripheral paresis, primarily from poliomyelitis (especially in young children) and other polyneuropathies (diphtheria, with porphyria). In addition, lesions of the spinal cord and the brain stem (transverse myelitis, stroke in the vertebrobasilar system) and diseases with impaired neuromuscular transmission (myasthenia gravis, botulism) may have a similar clinical picture.

• In the differential diagnosis with poliomyelitis, one should take into account the data of the epidemiological history, the presence of fever at the onset of the disease, symptoms from the gastrointestinal tract, asymmetry of the lesion, the absence of objective sensitivity disorders, and high cytosis in the cerebrospinal fluid. The diagnosis of poliomyelitis is confirmed by virologic or serologic testing.
• Polyneuropathy in acute intermittent porphyria may resemble Guillain-Barré syndrome, but, as a rule, is accompanied by a variety of psychopathological symptoms (delusions, hallucinations, etc.) and severe abdominal pain. The diagnosis is confirmed by identifying increased concentration porphobilinogen in urine.
• Transverse myelitis is characterized by early and persistent dysfunctions of the pelvic organs, the presence of a level of sensory disorders, and the absence of damage to the cranial nerves.
• Symptoms similar to those in Guillain-Barré syndrome are possible with extensive cerebral stem infarctions with the development of tetraparesis, which has peripheral features in the acute period. However, such cases are characterized by acute development (usually within a few minutes) and, in most cases, depression of consciousness (coma), which is not observed in Guillain-Barré syndrome. The final diagnosis is confirmed by MRI.
• Myasthenia gravis differs from Guillain-Barré syndrome in the variability of symptoms, the absence of sensory disorders, and characteristic changes in tendon reflexes. The diagnosis is confirmed by EMG (detection of the decrement phenomenon) and pharmacological tests.
• Botulism, in addition to the corresponding epidemiological data, is characterized by a descending type of paresis distribution, preservation in some cases of tendon reflexes, the absence of sensory disorders and changes in the cerebrospinal fluid.

Guillain-Barré Syndrome Treatment

The goals of treatment for Guillain-Barré syndrome are to maintain vital functions, arrest the autoimmune process with the help of specific therapy, and prevent complications.

Indications for hospitalization

All patients with Guillain-Barré syndrome must be admitted to a hospital with an intensive care unit.

Non-drug treatments for Guillain-Barré syndrome

In about 30% of cases of Guillain-Barré syndrome, severe respiratory failure develops (due to paresis of the diaphragm and respiratory muscles), which necessitates mechanical ventilation. Indications for intubation with further mechanical ventilation are a decrease in VC to 15-20 ml / kg, P a O 2 50 mm Hg. The duration of mechanical ventilation (from several days to months) is determined on an individual basis, focusing on the VC, restoration of swallowing and cough reflex and the general dynamics of the disease. Disconnect the patient from the ventilator gradually, through the stage of intermittent forced ventilation.

In severe cases with severe paresis, it is of fundamental importance for the prevention of complications associated with prolonged immobility of the patient (bedsores, infections, thromboembolic complications, etc.) proper care: periodic (every 2 hours or more) change of position of the patient, skin care, prevention of aspiration [sanitation of the mouth and nose, feeding through a nasogastric tube, sanitation of the trachea and bronchi (during mechanical ventilation)], monitoring the functions of the bladder and intestines, passive gymnastics and massage of the limbs, etc.

In case of persistent bradyarrhythmias with a threat of asystole, it may be necessary to install a temporary pacemaker.

Drug treatment and plasmapheresis

As a specific therapy for Guillain-Barré syndrome, aimed at arresting the autoimmune process, pulse therapy with class G immunoglobulins and plasmapheresis are currently used. Specific therapy methods are indicated for severe (4 and 5 points according to the North American scale of motor deficit severity) and moderate (2-3 points) course of the disease. The effectiveness of both methods is approximately the same; their simultaneous implementation is impractical. The method of treatment is chosen on an individual basis, taking into account availability, possible contraindications, etc.

• Plasmapheresis is an effective method of treating Guillain-Barré syndrome, significantly reducing the severity of paresis, the duration of mechanical ventilation and improving the functional outcome. Usually 4-6 operations are performed with an interval of one day; the volume of plasma to be replaced in one operation must be at least 40 ml / kg. As replacement media, 0.9% sodium chloride solution, rheopolyglucin, albumin solution are used. Plasmapheresis is relatively contraindicated in liver failure, severe pathology cardiovascular system, blood clotting disorders, the presence of infections. Possible complications are hemodynamic disorders (drop in blood pressure), allergic reactions, electrolyte disturbances, hemorrhagic disorders, and the development of hemolysis. All of them are observed quite rarely.
• Class G immunoglobulin is administered intravenously at a dose of 0.4 g / kg once a day for 5 days. Immunoglobulin treatment, like plasmapheresis, reduces the length of stay on mechanical ventilation and improves functional outcome. The most common side effects are headaches and muscle aches, fever, and nausea; their severity can be reduced by decreasing the infusion rate. Severe side effects such as thromboembolism, aseptic meningitis, hemolysis, acute renal failure, etc. are extremely rare. Normal human immunoglobulin is contraindicated in congenital IgA deficiency and a history of anaphylactic reactions to immunoglobulin preparations.

Symptomatic treatment of Guillain-Barré syndrome

• Infusion therapy for the correction of violations of acid-base, water-electrolyte balances, severe arterial hypotension.
• With persistent severe arterial hypertension, antihypertensive drugs are prescribed (beta-blockers or slow calcium channel blockers).
• With severe tachycardia, beta-blockers (propranolol) are prescribed, with bradycardia - atropine.
• With the development of intercurrent infections, antibiotic therapy is required (drugs of a wide spectrum of action are used, for example, fluoroquinolones).
• For the prevention of deep vein thrombosis and pulmonary embolism, low molecular weight heparin is prescribed in prophylactic doses twice a day).
• For pain of nociceptive origin (muscle, mechanical), paracetamol or NSAIDs are recommended, in the case of a neuropathic nature of pain, the drugs of choice are gabapentin, carbamazepine, pregabalin.

Surgical treatment of Guillain-Barré syndrome

If necessary, long-term (more than 7-10 days) mechanical ventilation is advisable to impose a tracheostomy. For severe and prolonged bulbar disorders, a gastrostomy tube may be required.

General principles of treatment of Guillain-Barré syndrome

Treatment of acutely developing and rapidly growing manifestations of Guillain-Barré syndrome requires supportive therapy in an intensive care unit, as well as an impact on the immune mechanisms of the disease. Patients with Guillain-Barré syndrome should be hospitalized for close monitoring of the state of breathing and autonomic functions. The faster the paralysis builds up, the higher the likelihood that mechanical ventilation is required. During the period of increasing symptoms, regular neurological examination is necessary, assessment of the vital capacity of the lungs, maintenance of airway patency with regular suction of mucus. At an early stage of the disease, constant vigilance is necessary, since even in the absence of obvious disturbances in respiratory and bulbar functions, small aspiration can significantly increase autonomic dysfunction and provoke respiratory failure.

The improvement in prognosis and reduction in mortality in Guillain-Barré syndrome achieved in recent years is mainly due to the early hospitalization of patients in intensive care units. Indications for transferring the patient to the intensive care unit and considering intubation may be a decrease in the vital capacity of the lungs below 20 ml / kg and difficulties in removing secretions from the airways. The purpose of early transfer is to avoid urgent intubation in 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 maintenance therapy is the prevention and timely treatment of pulmonary and urinary infections, as well as prevention of deep vein thrombosis of the lower leg and subsequent thromboembolism of the pulmonary artery by subcutaneous injection heparin (5000 IU 2 times a day). You should also monitor your diet and bowel function. Since autonomic dysfunction has a significant impact on mortality, constant monitoring of cardiac activity and blood pressure is necessary.

One of the important aspects of helping patients with Guillain-Barré syndrome in an intensive care unit, which, however, is not always taken into account, is the correction of severe anxiety, which is caused by the complete immobilization of the patient against the background of preserved intelligence. In this regard, psychological support is essential. Patients need to explain the essence of the disease, the features of its course, including the possibility of progression, to familiarize with the methods of treatment for different stages... It is important to explain to them that the likelihood of full recovery is very high, even if they will be on mechanical ventilation for some time. Establishing contact through eye movements reduces the patient's feeling of isolation from the world. In our experience, administering 0.5 mg lorazepam every 4-6 hours is effective for nocturnal hallucinations. It is also possible to prescribe 0.5 mg risperidone or 0.25 mg olanzapine.

The practice of treating Guillain-Barré syndrome has undergone significant changes over the past decade. For example, plasmapheresis has been proven to be effective. Although its mechanism of action remains unknown, it is believed that it may be associated with the elimination of antibodies, cytokines, complement and other mediators of the immune-inflammatory response. In an open-label multicenter North American study comparing disease outcomes with and without plasmapheresis special treatment, it was shown that carrying out plasmapheresis for five consecutive days reduces the length of hospital stay and leads to more significant improvement than in the control group. Treatment was more effective when started in the first week of illness. Similar results were obtained by the French Cooperative Group, which conducted a randomized multicenter study and showed that four plasmapheresis sessions resulted in faster recovery in 220 patients included in the study (French Cooperative Group, 1987). A study of the same patients a year later showed that complete recovery of muscle strength was observed in 71% of patients undergoing plasmapheresis, and only in 52% of patients in the control group (French Cooperative Group, 1992). The next study compared the effectiveness of different numbers of plasmapheresis sessions in 556 patients with Guillain-Barré syndrome with varying severity of symptoms (French Cooperative Group, 1997). In patients with mild symptoms who underwent two sessions of plasmapheresis, recovery was more significant than in patients whose treatment regimen did not include plasmapheresis. In patients with moderate severity of symptoms, four plasmapheresis sessions were more effective than two plasmapheresis sessions. At the same time, six sessions of plasmapheresis were not more effective than four sessions in patients with both moderate and severe symptoms. Currently, most centers specializing in the treatment of Guillain-Barré syndrome still use five to six sessions over 8-10 days to avoid the stress of doing the procedure daily. Exchange transfusion is performed using a Shealy catheter. Plasmapheresis is also effective in children with Guillain-Barré syndrome, accelerating the recovery of the ability to move independently. Although plasmapheresis is a relatively safe procedure, its implementation in Guillain-Barré syndrome requires special care due to the danger of autonomic dysfunction in patients and their tendency to develop infections.

Intravenous administration high doses of immunoglobulin are also recognized effective method treatment of Guillain-Barré syndrome, which can significantly reduce the duration and severity of the disease. As in the case of plasmapheresis, the mechanism of therapeutic action of immunoglobulin remains unclear. It is assumed that it can eliminate pathogenic antibodies through anti-idiotypic antibodies, block the Fc-component of antibodies on target cells, and also inhibit the deposition of complement, dissolve immune complexes, weaken lymphocyte functions, disrupt the production or interfere with the functioning of cytokines. Immunoglobulin is prescribed in a total dose of 2 g / kg, which is administered for 2-5 days. In a randomized study comparing the effect of immunoglobulin and plasmapheresis, it was shown that with the use of plasmapheresis, an improvement occurs on average after 41 days, and with the use of immunoglobulin - after 27 days. In addition, in patients who received immunoglobulin, significantly less complications and to a lesser extent, artificial ventilation was required. The main adverse prognostic factor was elderly age... A subsequent randomized multicenter study of plasmapheresis and immunoglobulin in 383 patients who were prescribed these techniques within the first 2 weeks after the onset of symptoms showed that both methods have comparable efficacy, but their combination does not have significant advantages over using either method separately.

The introduction of immunoglobulin at a dose of 2 g / kg for 2 days was effective and safe method treatment and in children with severe Guillain-Barré syndrome. Side effects were mild and rare. In some patients, especially those suffering from migraine, it was noted headache, which was sometimes accompanied by aseptic meningitis with pleocytosis in cerebrospinal fluid. Chills, fever, and myalgia were also occasionally observed, as well as acute renal impairment with the development of renal failure. With the introduction of immunoglobulin, an anaphylactic reaction is possible, especially in individuals with a deficiency of immunoglobulin A. The main disadvantage of both immunoglobulin and plasmapheresis is the high cost. However, it is clearly outweighed by the effectiveness of these therapies, which is evident even in this money-making era.

As shown in a double-blind, placebo-controlled multicenter study conducted on 242 patients with Guillain-Barré syndrome, high-dose intravenous corticosteroids (methylprednisolone, 500 mg per day for 5 days) did not affect any of the indicators that assessed the outcome of Guillain's syndrome. Barre, as well as the likelihood of his recurrence. Subsequently open study, during which 25 patients with Guillain-Barré syndrome were treated with intravenous 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 the combination of immunoglobulin and methylprednisolone, recovery was better, while in 76% of patients by the end of the 4th week there was an improvement by at least one functional level - in the control group, a similar degree of recovery was noted in only 53% of patients. This may indicate that corticosteroids may still play a role in the treatment of Guillain-Barré syndrome. Randomized clinical trials are needed to clarify this issue and determine whether there is a significant improvement in disease outcome when intravenous corticosteroids are added to plasmapheresis or immunoglobulin.

Further management

After the end of the acute period, complex rehabilitation measures are required, the plan of which is made up on an individual basis, depending on the severity of residual symptoms (exercise therapy, massage, etc., while thermal procedures contraindicated!).

Patients who have had Guillain-Barré syndrome should be informed about the need to observe a protective regime for at least 6-12 months after the end of the disease. Physical overload, overheating, hypothermia, excessive sun exposure, alcohol intake are unacceptable. Also during this period, you should refrain from vaccination.

Forecast

Mortality in Guillain-Barré syndrome averages 5%. The cause of death may be respiratory failure, death is also possible due to aspiration pneumonia, sepsis and other infections, pulmonary embolism. Mortality increases significantly with age: in children under 15 years of age, it does not exceed 0.7%, while in people over 65 it reaches 8.6%. Other unfavorable prognostic factors for full recovery include a prolonged (more than 1 month) period of mechanical ventilation, the presence of previous lung diseases.

In the majority of patients (85%), complete functional recovery is observed within 6-12 months. Persistent residual symptoms persist in about 7-15% of cases. Predictors of unfavorable functional outcome are age over 60 years, rapidly progressive course of the disease, low amplitude of the M-response when stimulated at the distal point (implying severe damage to axons). The recurrence rate of Guillain-Barré syndrome is approximately 3-5%.

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It's important to know!

Symptoms of neuroblastoma are extremely diverse and depend on the location and degree of damage to a particular organ. Most often, the tumor is localized in the adrenal glands (40% of cases), followed by the frequency of the retroperitoneal space (25-30%), posterior mediastinum(15%), small pelvis (3%) and neck (1%). Rare and unknown localizations are observed in 5-15% of cases of neuroblastoma.

ICD-10 codeG.61.0

Synonyms:acute demyelinating polyradiculo (neuro) pathy, acute postinfectious polyneuropathy, Landry-Guillain-Barré syndrome, outdated. Landry ascending palsy.

The term Guillain-Barré syndrome is an eponym (i.e., giving a name) to refer to a set of syndromes of acute inflammatory polyradiculoneuropathy of an autoimmune nature, a characteristic manifestation of which is a progressive symmetric flaccid paralysis in the muscles of the extremities and muscles innervated by the cranial nerves (with the possible development of dangerous breathing and swallowing disorders) with or without sensory and autonomic disorders (unstable blood pressure, arrhythmias, etc.) .

Along with the fact that the Guillain-Barré syndrome is classically presented as demyelinating polyneuropathy with ascending weakness, called acute inflammatory demyelinating polyneuropathy and accounting for 75 - 80% of cases, Several atypical variants or subtypes of this syndrome have been described and identified in the literature, representing a heterogeneous group of immune-dependent peripheral neuropathies : Miller-Fisher syndrome (3 - 5%), acute motor axonal polyneuropathy and acute sensorimotor axonal polyneuropathy (15-20%), and more rare acute sensory polyneuropathy, acute pandizautonomy, acute cranial polyneuropathy, pharyngo-cervic-brachial variant. As a rule, these options are clinically usually more severe than the main one.

EPIDEMIOLOGY

Guillain-Barré Syndrome the most common acute polyneuropathy... The incidence rate is 1.7 - 3.0 per 100,000 population per year, is approximately equal in men and women, has no seasonal fluctuations, and is more common in old age. The incidence rate at the age of 15 years is 0.8 - 1.5, and at the age of 70 - 79 it reaches 8.6 per 100,000. Mortality rates range from 2 to 12%.

ETIOLOGY and PATHOGENESIS

Etiology of the disease not definitively known.

Guillain-Barré syndrome is post-infectious autoimmune disease.

1 - 3 weeks before the development of the syndrome, 60 - 70% of patients have respiratory or gastrointestinal infections, which can be:
viral nature (cytomegalovirus, Epstein-Barr virus)
bacterial nature(caused by Campylobacter jejuni)
mycoplasma nature

Much less often the syndrome develops:
after injury to peripheral nerves
surgical interventions
vaccinations
with tick-borne borreliol (Lyme disease)
sarcoidosis
systemic lupus erythematosus
AIDS
malignant tumors

Both cellular and humoral immune mechanisms are likely to play a role in the development of the disease.

Infectious agents appear to trigger an autoimmune reaction directed against antigens of the peripheral nervous tissue (lemmocytes and myelin), in particular with the formation of antibodies to peripheral myelin - gangliosides and glycolipids, such as GM1 and GD1b, located on the myelin of the peripheral nervous system.

!!! The titer of antibodies to GM1 and GD1b correlates with the clinical course of the disease.

Also apparently Immunological cross reaction possible between Campylobacter jejuni lipopolysaccharides and GM1 ganglioside... There is still no final opinion on the nature of the antigen or antigens that cause the development of cascade immune reactions.

Myelinated nerve fiber consists of an axial cylinder (the actual process containing the cytoplasm) covered with a myelin sheath.

Depending on the purpose of the defeat,:
demyelinating variant diseases (more common)
axonal variant diseases

diseases, the myelin sheaths of the axons suffer, demyelination is observed without the involvement of the axonal cylinders of the axons, and therefore the speed of conduction along the nerve fiber decreases with the development of paresis, but this condition is reversible. These changes are detected primarily at the junction of the anterior and posterior roots of the spinal cord, while only the anterior roots can be involved (which explains variants with purely motor disorders), and other parts of the peripheral nervous system can also be involved. The demyelinating variant, in particular, is characteristic of the classic Guillain-Barré syndrome.

The axonal variant of the lesion is much less common. , more severe, in which degeneration of the Waller type develops (distal to the site of the lesion) of the axial cylinders of axons with the development of, as a rule, gross paresis or paralysis. In the axonal variant, the antigens of the axons of the peripheral nerves are primarily exposed to the autoimmune attack, and a high titer of GM1 antibodies is often found in the blood. This variant, in particular observed in acute sensorimotor axonal polyneuropathies, is characterized by a more severe and less reversible course of the syndrome than in the first case.

Most cases of Guillain-Barré syndrome are characterized by self-limiting autoimmune lesions., in particular, due to the elimination of autoantibodies after a certain time, i.e. reversible nature of the lesion. For the clinic, this means that if a seriously ill patient with paralysis, swallowing disorders and respiratory failure is given adequate nonspecific supportive therapy (prolonged mechanical ventilation, prevention of infectious complications, etc.), then recovery can often be as complete as with the use of specific therapy, but more late dates.

CLINIC

The main manifestation of the disease is:
growing over several days or weeks (on average 7 - 15 days) relatively symmetrical flaccid tetraparesis - weakness in the arms and legs with low muscle tone and low tendon reflexes
tetraparesis initially often involves the proximal legs, which is manifested by difficulty climbing stairs or getting up from a chair
only after a few hours or days the hands are involved - "ascending paralysis"

The disease can quickly (within a few hours) lead to paralysis of the respiratory muscles.

Often the initial manifestation of Guillain-Barré syndrome is paresthesia.(unpleasant sensation of numbness, tingling, burning, crawling creeps) in the tips of the fingers and toes.

Less common are the following options for the onset of the disease:
Paresis primarily develops in the arms (“descending paralysis”).
Paresis develops in the arms and legs at the same time.
The hands remain intact during the course of the disease (paraparetic variant of the syndrome).
At first, the paralysis is one-sided, but after a while the defeat of the other side is sure to join.

Depending on the severity of symptoms, there are:
mild degree diseases- can walk more than 5 m without assistance
moderate disease- moderate paresis is noted (the patient cannot walk confidently without support or cannot walk more than 5 m on his own), pain syndrome and sensitivity disorders
severe disease- cases are considered, accompanied by paralysis or gross paresis of the limbs, often with respiratory disorders

The course of the disease
Symptom escalation phase within 7 - 15 days it is replaced by a plateau phase (stabilization of the process), lasting 2 - 4 weeks, and then recovery begins, lasting from several weeks to months (sometimes up to 1 - 2 years).

Full recovery occurs in 70% of cases.
Severe residual paresis of the extremities and impaired sensitivity persist in 5-15% of patients.
In 5-10% of cases, the syndrome recurs, often after completion of the course of treatment, or is provoked by a respiratory or intestinal infection.

Clinical variants of the disease

In a typical case of Guillain-Barré syndrome:
sensitive disorders, as a rule, they are moderately expressed and are represented by paresthesias, hypalgesia (reduced sensitivity), hyperesthesia (increased sensitivity) in the distal parts of the limbs like "socks and gloves", sometimes minor violations deep sensitivity, pain in the muscles of the shoulder and pelvic girdle, back, radicular pain, symptoms of tension (may persist against the background of regression of paralysis).
myalgias usually subside spontaneously after a week
with an ascending direction of development, paresis captures the muscles
legs, arms, torso
respiratory muscles
cranial muscles, mainly: mimic (characterized by bilateral lesion facial nerves)
bulbar with the development of aphonia - loss of sonority of the voice, dysarthria - speech impairment, dysphagia - swallowing disorders up to aphagia - inability to swallow
less often the external muscles of the eyes - abduction paresis eyeball
may involve flexors of the neck and muscles lifting the shoulders, weakness of the intercostal muscles and diaphragm with the development of respiratory failure.
characteristic are shortness of breath on exertion, shortness of breath, difficulty swallowing, speech disorders.
all patients have loss or sharp suppression of deep tendon reflexes, the degree of which may not correspond to the severity of the paralysis
also developing muscle hypotension and hypotrophy (in the late period)
vegetative disorders in the acute period, they occur in more than half of the cases, and are often the cause of death; there is a violation of sweating, intestinal paresis, an increase or decrease in blood pressure, orthostatic hypotension, tachycardia or bradycardia, supraventricular, ventricular arrhythmias, cardiac arrest.

17-30% of patients may develop (acutely, within hours and days) respiratory distress requiring mechanical ventilation, as a result of damage to the phrenic nerve, paresis of the diaphragm and weakness of the respiratory muscles. With paresis of the diaphragm, paradoxical breathing develops with the retraction of the abdomen while inhaling.

Clinical signs respiratory failure are:
rapid breathing (tachypnea)
perspiration on the forehead
weakening of the voice
the need to pause for inhalation while talking
weakening of the voice
tachycardia with forced breathing
also with paresis of the bulbar muscles, it is possible to develop impaired airway patency, impaired swallowing (with the development of aspiration) and speech

V initial stage disease, fever is usually absent.

ATYPICAL OPTIONS for Guillain-Barré Syndrome

Miller-Fisher syndrome- occurs in 5% of cases of Guillain-Barré syndrome.
It manifests itself:
motor ataxia - gait disorder and ataxia (coordination disorder) of the trunk muscles
ophthalmoplegia involving the external, less often internal muscles of the eye
areflexia
typical preservation of muscle strength
usually ends with full or partial recovery over weeks or months
rarely, in severe cases, tetraparesis, paralysis of the respiratory muscles may join

Acute sensory polyneuropathy
It manifests itself:
quick start with severe violations sensitivity and areflexia, quickly involving limbs and having a symmetrical character
sensitive ataxia (impaired coordination of movements)
the prognosis is often favorable

Acute motor axonal polyneuropathy
Closely associated with intestinal infection with C. jejuni, with about 70% being seropositive for C. jejuni.
Clinically manifested by: purely motor disorders: increasing paresis of the ascending type.
Diagnosed by electromyography for purely motor axonopathy.
This type is characterized by a higher proportion of pediatric patients.
In most cases, the prognosis is good.

Acute sensorimotor axonal polyneuropathy
It is usually represented by rapidly developing and gross tetraparesis with long and poor recovery.
As well as acute motor axonal polyneuropathy, it is associated with C. Jejuni-induced diarrhea.

Acute pandizautonomy
Rare.
It proceeds without significant motor or sensory disturbances.
Dysfunctions of the autonomic nervous system are manifested:
severe postural hypotension
postural tachycardia
fixed heart rate
constipation
delayed urination
sweating disorders
decreased salivation and lacrimation
pupillary disorders

Faringo-cervico-brachial variant
Characterized by isolated weakness in the facial, oropharyngeal, neck and neck muscles upper limbs without involvement of the lower extremities.

Acute cranial polyneuropathy
It is manifested by the involvement of only the cranial nerves in the pathological process.

COMPLICATIONS
Paresis and paralysis in the limbs, neck.
Persistent loss of sensitivity.
Deep vein thrombosis of the lower leg.
5% of patients subsequently develop chronic inflammatory demyelinating polyradiculoneuropathy with a recurrent or progressive course, sensitive to corticosteroids.
Death due to respiratory failure, pneumonia, pulmonary embolism, cardiac arrest, sepsis, respiratory distress syndrome, autonomic nervous system dysfunction.

DIAGNOSTICS

Polyradiculoneuritis should be suspected when the patient develops a relatively symmetrical growing muscle weakness in the limbs. Acute or subacutely increasing ascending flaccid tetraparesis with areflexia is characteristic of the disease.

The main diagnostic criteria for Guillain-Barré syndrome:
increasing muscle weakness in at least two limbs
significant decrease in muscle strength, up to complete loss, tendon reflexes

Additional diagnostic criteria are:
a decrease in the speed of conduction of nerve impulses through the muscles with the formation of a conduction block during EMG
protein-cell dissociation in cerebrospinal fluid

The diagnosis is supported by:
disease progression within 4 weeks
start of recovery after 2 - 4 weeks
relative symmetry of symptoms
lack of severe sensitivity disorders
involvement of the cranial nerves (primarily bilateral lesions of the facial nerves)
autonomic dysfunction
absence of fever at the onset of the disease
uncharacteristic pelvic disorders
(neurogenic urinary disorders)

Cerebrospinal fluid examination
during the 1st week of the disease, the protein content in the cerebrospinal fluid remains normal
starting from 2 weeks, protein-cell dissociation is detected - an increased protein content with normal or slightly increased cytosis (no more than 30 cells in 1 μl.)
with a higher cytosis, another disease should be looked for
on the background high level squirrel, stagnant nipples may appear optic nerves

Electromyographic examination
Allows to identify the peripheral nature of the lesion, as well as to differentiate the demyelinating and axonal variants of the disease.
With demyelinating variant the disease is characterized by: a decrease in the amplitude of the M-response against the background of signs of demyelination of nerve fibers - a decrease in the speed of conduction along the motor fibers by more than 10% from normal, lengthening of the distal latency, partial conduction blocks.
With the axonal variant a decrease in the amplitude of the M-response is detected against the background of a normal conduction velocity along motor fibers (or a decrease in velocity, but not more than 10%), a normal value of the distal latency and F-response.

Determination of blood plasma autoantibodies
Has limited diagnostic value.
Not usually done as a routine test.
It is being investigated for scientific purposes and can be useful in complex, diagnostically unclear cases, in particular for the diagnosis of acute axonal lesions.
Antibodies to glycolipids (ganglioside GM-1 and GQ1b) are detected in blood plasma in 60 - 70% of patients in the acute phase of the disease.
GM1 antibodies are often found in axonal motor neuropathy and in acute inflammatory demyelinating polyneuropathy (classical). C. jejuni antecedent intestinal infection is closely associated with high titers of antibodies to GM-1.
Antibodies to GQ1b are found in patients with Guillain-Barré syndrome with ophthalmoplegia, including those with Miller-Fisher syndrome.

DIFFERENTIAL DIAGNOSTICS

The possibility of the following diseases, which may be accompanied by a similar clinical picture :
tumors and vascular myelopathy of the spinal cord
stem or spinal stroke
diphtheria polyneuropathy
periodic paralysis
polymyositis
polio
botulism
myasthenia gravis
hysteria
critical illness polyneuropathy
Wernicke's encephalopathy
stem encephalitis

TREATMENT

Treatment for Guillain-Barré syndrome includes two components:
non-specific- supportive therapy
specific - plasmapheresis therapy or pulse therapy with class G immunoglobulin.

!!! Due to the possibility of developing decompensation with severe respiratory failure for several hours, as well as heart rhythm disturbances, it is necessary to treat Guillain-Barré syndrome in the acute phase as an emergency.

In cases of the development of acute respiratory failure in a medical institution, it must be possible to carry out long-term artificial ventilation of the lungs.

In severe cases with early development of acute respiratory failure, treatment is carried out in an intensive care unit or intensive care unit:
carry out hourly monitoring VC, blood gases, the content of blood electrolytes, heart rate, blood pressure, the state of the bulbar muscles (the appearance and growth of impaired swallowing, which does not relieve coughing, hoarseness, speech impairment)
with bulbar palsy with impaired swallowing, choking, pouring a drink through the nose, the introduction of a nasogastric tube is indicated, and often intubation (for the prevention of aspiration and aspiration pneumonia)
shows tracheal intubation with mechanical ventilation with the development of respiratory failure, if VC falls below 12 - 15 ml / kg, and with bulbar paralysis and disorders of swallowing and speech below 15 - 18 ml / kg.
with no tendency to recover spontaneous breathing within 2 weeks, tracheostomy is performed

!!! Corticosteroids are not currently used because of their proven ineffectiveness. They do not improve the outcome of the disease.

SPECIFIC THERAPY

Specific therapy with plasmapheresis or intravenous administration of high doses of immunoglobulin begins soon after diagnosis. Shown approximately equal effectiveness of both methods of treatment, as well as the absence of additional effect from the combination of these methods. There is currently no consensus on the choice of specific therapy.

With a light flow Guillain-Barré syndrome, given that there is a high likelihood of spontaneous recovery, treatment of patients can be limited to non-specific and supportive therapy.

With an average severity of the process, and especially in severe cases specific therapy starts as early as possible.

Immunoglobulin treatment has some advantage over plasmapheresis, since it is easier and more convenient to use, has a significantly smaller number of side effects, is more easily tolerated by the patient, and therefore immunoglobulin is the drug of choice in the treatment of Guillain-Barré syndrome.

Intravenous pulse therapy with immunoglobulin
Intravenous pulse therapy with immunoglobulin (IgG, drugs - octagam, sandoglobulin, intraglobulin, normal human immunoglobulin) is indicated for patients who are unable to walk more than 5 m without assistance, or for more severe (with paralysis, breathing and swallowing disorders) patients, with a maximum the effectiveness of the drug at the beginning of therapy within 2 to 4 weeks from the onset of the disease. It is administered intravenously at a dose of 0.4 g / kg / day for 5 days (the total course dose is 2 g / kg or about 140 g). An alternative scheme for the administration of the same course dose: 1 g / kg / day in two administrations for two days. Its use is limited by its high cost.

Plasmapheresis
Plasmapheresis, prescribed in the phase of disease progression (approximately in the first two weeks), almost doubles the recovery process and reduces the residual defect. It is prescribed in moderate and severe cases according to the scheme of 4 - 6 sessions every other day, with an exchange of 50 ml / kg per session (at least 35-40 ml of plasma per kg of body weight), for a total course of 200 - 250 ml / kg (at least 160 ml of plasma per 1 kg of body weight per course). In mild cases and the recovery phase, plasmapheresis is not indicated. Plasmapheresis has shown a fairly high efficiency when prescribed to seriously ill patients, when therapy is started within more than 30 days from the onset of the disease.

In 5-10% of patients, a relapse of the disease occurs after the end of treatment with plasmapheresis or immunoglobulin... In this case, either the treatment is resumed with the same method, or an alternative method is used.

NON-SPECIFIC THERAPY

It is necessary to prevent deep vein thrombosis of the leg in bedridden patients (especially with paralysis in the legs):
use oral anticoagulants of indirect action phenylin or warfarin in doses stabilizing the INR at 2.0, or fraxiparin (nadroparin) 0.3 ml. n / a 1 - 2 times / day, or sulodexide (Wessel Duet F) 2 times a day, 1 ampoule (600 LSU) IM for 5 days, then orally 1 caps (250 LSU) 2 times a day
prevention is carried out until the time the patient starts to get out of bed
if thrombosis develops before starting therapy, prevention is carried out according to the same scheme
also use bandaging elastic bandage legs to mid-thigh (or use stockings with graduated compression) and raising the legs by 10-15
shows passive and, if possible, active "walking in bed" with flexion of the legs, emitting walking for 5 minutes 3-5 times a day

In case of paresis of the facial muscles, measures are taken to protect the cornea:
instillation of eye drops
blindfold for the night

Prevention of contractures and paralysis:
for this, passive exercises are carried out 1 - 2 times a day
ensure correct position in bed - comfortable bed, foot supports
massage the limbs
subsequently, active physiotherapy exercises are connected

Prevention of bedsores:
change position in bed every 2 hours
wipe the skin with special compounds
use anti-decubitus mattresses

Prevention of pulmonary infection:
breathing exercises
the earliest possible mobilization of the patient

With a decrease in the vital capacity of the lungs, difficulty in separating bronchial secretions:
massage is shown (tapping and vibration with simultaneous rotation of the body in the supine position) every 2 hours during the day.

Symptomatic therapy:
antiarrhythmic
hypotensive
analgesic

With arterial hypotension, falling blood pressure (approximately blood pressure 100 - 110/60 - 70 mm Hg and below):
intravenous administration of colloidal or crystalloid solutions - isotonic sodium chloride solution, albumin, polyglucin
in case of insufficient effect in combination with corticosteroids: prednisone 120 - 150 mg., dexazone 8 - 12 mg
in case of insufficiency of these funds, vasopressors are used: dopamine (50-200 mg is diluted in 250 ml of isotonic sodium chloride solution and injected at a rate of 6-12 drops / min), or norepinephrine, or mezaton

For moderate pain use simple analgesics and non-steroidal anti-inflammatory drugs.

With pronounced pain syndrome apply tramal or cabamazepine (tigretol) or gabapentin (neurontin), possibly in combination with tricyclic antidepressants (imipramine, amitriptyline, azafen, etc.).

Classes with a speech therapist for the treatment and prevention of speech and swallowing disorders.

Rehabilitation

Rehabilitation includes massage, remedial gymnastics, physiotherapy procedures. Percutaneous muscle stimulation is performed for muscle pain and paresis of the limbs.

FORECAST

Poor prognostic factors include:
elderly age
rapid progression of the disease in the initial phase
development of acute respiratory failure with the need for mechanical ventilation
anamnestic indications of intestinal infection caused by C. jejuni

Although the majority of patients with Guillain-Barré syndrome have a good recovery with adequate therapy, 2-12% die from complications and a significant proportion of patients retain a persistent motor deficit.

Approximately 75-85% have good recovery, 15-20% have moderate motor deficits, and 1-10% have profound disability.

Recovery rate of motor functions can vary and takes from several weeks to months. With axonal degeneration, recovery may take 6 to 18 months. V general case, slower and less complete recovery will be observed in older patients.

Mortality in Guillain-Barré syndrome is largely determined by the capacity of the hospital to conduct modern nonspecific supportive therapy (long-term mechanical ventilation, prevention of infectious complications, etc.), and in modern hospitals is about 5%. Previously, mortality was up to 30% due to the development of respiratory failure and secondary complications.

PREVENTION

Specific prevention methods absent.

Patients are advised avoid vaccinations within 1 year from the onset of the disease, as they can provoke a relapse of the syndrome.
In the future, immunizations are carried out if there is an appropriate justification for their need for this.

If Guillain-Barré syndrome develops within 6 months after any vaccination, it makes sense to advise the patient to refrain from this vaccination in the future.

All people suffer from colds. Recovery, as a rule, does not take long, and most of these patients do not even seek help from a doctor. This happens most often, but sometimes events do not develop so favorably.

Overview of Guillain-Barré Syndrome

In the recovery period, it is important to carry out physiotherapy (massage), electrostimulation of the pharyngeal muscles (if there are swallowing disorders) and exercise physiotherapy exercises... The patient's condition is assessed both clinically and objectively using electroneuromyography.

After a short period of malaise with ARVI symptoms, numbness in the arms and legs may appear, a feeling of creeping creeps (paresthesia). After 1-2 days, weakness in the arms and legs joins; the person gradually becomes completely immobilized, loses the ability to self-service. Often there is soreness, hoarseness, and disturbed eye movements. At the same time, the patients are fully conscious, everyone hears and sees, the appearance of such patients is called the "talking head". The contractility of the intercostal muscles and the diaphragm gradually decreases, the volume of respiratory movements decreases and the vital capacity of the lungs (VC) decreases. In this regard, the blood in the lungs is not well enriched with oxygen, oxygen starvation occurs, due to respiratory failure, a lethal outcome can develop. Patients are indicated for treatment in the intensive care unit, since due to respiratory failure, it may always be necessary to carry out artificial ventilation.

The disease was first described by Georges Guillain (1876-1961); Alexandre Barre (1880-1967) and Andre Strohl (1887-1977). The article describes the case of two soldiers, a hussar and an infantryman, who developed paralysis within two weeks due to the absence of tendon reflexes. The authors' attention was also drawn to the increased protein in the cerebrospinal fluid in these patients. As already mentioned, such patients often need artificial ventilation of the lungs, and so, for the first time this was done in Russia. In 1912, the Russian doctor Golovinsky first applied manual artificial respiration to a peasant at the age of 21, suffering from polyradiculoneuritis with paralysis of the respiratory muscles. For 18 days, the doctor, together with senior medical assistants, continuously supported the patient's breathing in this way.

The disease occurs with approximately the same frequency on all continents of the globe. It is 1-2 cases per 100,000 people. Men and women get sick with the same frequency. The youngest patient was 3 weeks old, and the oldest was 95 years old. The most massive incidence was noted in the United States in the period 1976-1977. as a result of national influenza vaccination.

Guillain-Barré Syndrome Symptoms

The clinical picture at the initial stage is characterized by the presence of paresthesias (a feeling of creeping creeps) together or separately, tickling when swallowing, impaired sensitivity (first of all, deep sensitivity is disturbed - vibrational and so-called proprioceptive sensitivity - that is, the joint-muscular feeling, thanks to which we feel the position of parts of our body.We usually do not pay attention to this feeling special attention, but it is thanks to him that we can walk and, without hesitation, perform other actions with our hands and feet). In rare cases, there is only weakness in the arms and / or legs. Weakness often develops in those parts of the limbs that are closer to the median axis of the body (proximal). Reduced muscle tone, in severe cases, pelvic disorders occur (violation of the acts of urination and defecation).

In the expanded stage, there are motor, sensory disorders, the absence of tendon reflexes (areflexia) and autonomic disorders, which include heart rhythm disturbances, arterial hypertension, arterial hypotension, constipation, intestinal obstruction, diarrhea, urinary retention, and perspiration disorders. It is in the expanded stage that the weakness of the respiratory muscles can reach the degree when the patient must be transferred to artificial ventilation. Respiratory resuscitation helps patients survive the critical phase of the disease, which continues until the connection between the central and peripheral parts of the nervous system is restored.

Clinical subtypes of Guillain-Barré syndrome.

The main clinical subtype of Guillain-Barré syndrome is acute ascending demyelinating polyneuropathy. The lesion rises from the bottom up, from the limbs to the cranial nerves. Usually, when talking about GBS, they mean this particular subtype (Landry ascending type). There are others atypical forms, in which there is a pronounced damage to the axon (the process of a neuron, along which nerve impulses are carried from the cell body to other neurons, whose bodies lie either in the brain stem or in the spinal cord). And the processes of those neurons, in turn, are sent to the muscles and internal organs. These forms include acute sensory polyneuropathy, acute motor polyneuropathy, acute pandizautonomy (autonomic failure) and some other subtypes. These clinical subtypes are found mainly in the provinces of China, Japan, and Spain.

There is also the so-called Miller-Fisher syndrome, which occurs in non-Asian countries and is characterized by weakness of the oculomotor muscles, ptosis (prolapse upper eyelid), cerebellar ataxia. These symptoms lead the doctor to think about the possibility of damage to the central nervous system, but, according to magnetic resonance imaging and sectional studies, there are none. To determine the subtypes of the disease and the dynamics of its course, the method of electroneuromyography is widely used. This is a method that allows you to assess the degree and nature of the disturbance in the conduction of a nerve impulse through damaged nerves.

Causes and risks of Guillain-Barré syndrome

Until the end, science is not known. It is assumed that the disease is based on autoimmune mechanisms. This means that the human immune system "rebelles" against its own body, producing antibodies to certain molecules of the nerve sheath. The nerves themselves and their roots are affected (they are located at the junction of the central and peripheral nervous systems). The brain and spinal cord are not affected. The trigger factor for the development of the disease is viruses (among them, cytomegalovirus, Epstein-Barr virus are important); bacteria (Campylobacter jejuni). The immune system always reacts to any foreign agent that enters the body, but sometimes at the molecular level there is a failure in the "friend or foe" system, and then the immune system begins to fight the cells of its body. In science, this phenomenon is called "molecular mimicry."

Diagnostics of the Guillain-Barré syndrome

It is very important to recognize the disease on early stages and start the correct treatment on time. Upon questioning, it becomes clear that the patient's symptoms progressed within a few days after a short period of fever, accompanied by symptoms of acute respiratory viral infections or loose stools.

The necessary criteria for the diagnosis of Guillain-Barré syndrome are progressive muscle weakness in the arms and / or legs and tendon areflexia. It is important to pay attention to the symmetry of the lesion, sensory disturbances, damage to the cranial nerves (all cranial nerves can be affected except for pairs I, II and VIII); vegetative disorders (tachycardia, arrhythmia, postural hypotension, etc., see above), the absence of fever at the onset of the disease (some patients are feverish due to concomitant diseases). Symptoms develop rapidly but stop increasing by the end of 4 weeks. Recovery usually begins 2-4 weeks after the disease has stopped worsening, but sometimes it can be delayed for several months.

Guillain-Barré syndrome has a number of similar symptoms with other diseases, it must be distinguished from: myasthenia gravis, botulism, paralysis caused by taking antibiotics, diseases of the spinal cord, transverse myelitis, acute necrotizing myelitis, brain stem damage, “locked-in person” syndrome, stem encephalitis , hypermagnesemia; porphyric polyneuropathy (for its diagnosis, a urine test for porphobilinogen should be taken), polyneuropathy of critical conditions, neuroborreliosis (Lyme disease), acute tetraparesis (this is when all 4 limbs are paralyzed) tick bite, poisoning with salts of heavy metals (lead, gold, arsenic, thallium) , drug poisoning (vincristine, etc.).

Guillain-Barré Syndrome Treatment

Unfortunately, steroid hormone therapy is often undertaken, which worsens the prognosis in these patients.

The patient should be taken to the intensive care unit as early as possible specialized hospital where the final diagnosis will be made and specific treatment will be started. For Guillain-Barré syndrome, this is staged plasmapheresis. Plasmapheresis is a procedure for removing blood from a patient and separating corpuscles from plasma by centrifugation. The corpuscles return back to the bloodstream, the plasma is removed. Instead of plasma, the patient is transfused with albumin solution and electrolyte solutions. Together with plasma, antibodies and other molecular factors that lead to autoimmune damage to the myelin sheath of the nerves are removed from the patient's body. Plasmapheresis "interrupts" the development of autoimmune inflammation, and the patient's condition stabilizes. After stabilization of the patient's condition, the patient begins to recover.

The method of treatment with type G immunoglobulins, which are obtained from the blood serum of about 9000 donors, is also used. Therefore, treatment is very expensive and rarely used.

Careful care is required, monitoring of indicators of a general blood test, coagulogram and biochemistry.

Rehabilitation and prognosis of Guillain-Barré syndrome

Most patients have the prospect of a good recovery.

With timely and correct treatment the prognosis is favorable. Patients recover, fully serve themselves - live fully, although moderate weakness in the arms and legs may persist for life.

One of the serious diseases of a neurological nature is Guillain-Barré syndrome, when the immune system reverses polarity and begins to kill its own cells. This pathological process leads to autonomic dysfunctions. The disease is distinguished by a pronounced clinical picture, which allows it to be detected in a timely manner and to start therapy.

Description of the disease

Some pathologies develop in the form of a secondary immune response to the source of infection. They are accompanied by deformation of neurons and impairment nervous regulation... Among such ailments, the most heavy course different autoimmune polyneuropathy (Guillain-Barré syndrome, or GBS).

The disease is characterized by numerous inflammatory processes, destruction of the protective layer of the nerves of the peripheral system. The result is rapidly progressive neuropathy, accompanied by paralysis in the muscles of the limbs. The disease usually proceeds in an acute form and develops against the background of previous colds or infectious pathologies... With proper treatment, the chances of a full recovery increase.

Historical reference

At the beginning of the 20th century, researchers Guillain, Barre and Strol described a previously unknown disease in French soldiers. The fighters were paralyzed, there was a loss of sensation in the limbs. A group of scientists investigated cerebrospinal fluid in patients. In it, they revealed an increased protein content, while the number of other cells was normal. On the basis of the protein-cell association, Guillain-Barré syndrome was diagnosed, which differed from other pathologies of the nervous system of a demyelinating nature by a rapid course and a positive prognosis. Within 2 months, the soldiers recovered completely.

Subsequently, it turned out that this pathology is not as harmless as the discoverers described it. Approximately 20 years before the disclosure of information about her, the neuropathologist Landry monitored the condition of patients with a similar clinical picture. Patients also had paralysis. The rapid development of the pathological process was fatal. Later it became known that a disease diagnosed in French soldiers can also lead to death in the absence adequate treatment... However, in such patients, a picture of protein-cell association in the cerebrospinal fluid was observed.

After some time, it was decided to combine the two ailments. They were given one name that is used to this day - Guillain-Barré syndrome.

The reasons for the development of pathology

This disease has been known to science for over 100 years. Despite this, all the factors provoking its occurrence have not yet been clarified.

It is assumed that the pathology develops against the background of disturbances in the functioning of the immune system. When an infection enters the body of a healthy person, a protective reaction is triggered and a fierce fight against viruses and bacteria begins. In the case of this syndrome, the immune system perceives neurons for foreign tissue... The immune system begins to destroy the nervous system, as a result of which pathology develops.

Why there are such malfunctions in the work of the body's defenses is a poorly understood question. Common trigger factors include the following:

1. Traumatic brain injury. Mechanical damage that lead to cerebral edema or the formation of tumors are especially dangerous.
2. Viral infections. The human body is able to cope with many bacteria on its own. At frequent illnesses viral or long-term therapy immunity begins to weaken. Prolonged treatment and the use of potent antibiotics increase the risk of Guillain-Barré syndrome.
3. Hereditary predisposition. If close relatives of the patient have already encountered this pathology, he automatically falls into the risk group. Minor injuries and infectious diseases can act as a source of illness.

Other reasons are also possible. The syndrome is diagnosed in people with allergies who have undergone chemotherapy or complex surgeries.

What symptoms indicate illness?

Guillain-Barré neuropathology is distinguished by the symptomatology of three forms of the development of the disease:

• Acute when symptoms persist over several days.
• Subacute, when the pathology "swings" from 15 to 20 days.
• Chronic. Due to the inability to timely diagnose and prevent the development of serious complications, this form is considered the most dangerous.

The primary symptoms of the syndrome resemble a viral-respiratory infection. The patient's temperature rises, weakness appears throughout the body, and the upper respiratory tract becomes inflamed. In some cases, the onset of pathology is accompanied by gastrointestinal disorders.

Also, doctors distinguish other symptoms that distinguish Guillain-Barré syndrome from SARS.

1. Weakness of the limbs. Deformed nerve cells provoke a decrease or total loss sensitivity of muscle tissue. Originally discomfort appear in the lower leg area, then the discomfort spreads to the feet and hands. Aching pain is replaced by numbness. A person gradually loses control and coordination while performing simple actions (cannot hold a fork, write with a pen).
2. An enlarged abdomen is another sign of Guillain-Barré syndrome. Photos of patients with such a diagnosis are presented in the materials of this article. The patient is forced to rebuild his breathing from the upper to the abdominal type. As a result, the abdomen increases in size and protrudes strongly forward.
3. Difficulty swallowing. Muscles weakening every day interfere with the swallowing reflex. It becomes more and more difficult for a person to eat, he can choke on his own saliva.
4. Incontinence.

This pathology, as it develops, affects all systems of internal organs. Therefore, attacks of tachycardia, visual impairment and other symptoms of body dysfunction are not excluded.

Clinical course of the disease

During this pathology, doctors distinguish three stages: prodromal, peak and outcome. The first is characterized by general malaise, a slight increase in temperature and muscle pain. During the peak period, all the symptoms characteristic of the syndrome are observed, which, as a result, reach their peak. The outcome stage is characterized by the complete absence of any signs of infection, but manifests itself in neurological disorders. Pathology ends with either the restoration of all functions, or complete disability.

GBS classification

Depending on which clinical symptom predominates, Guillain-Barré syndrome is classified into several forms.

The first three are manifested by muscle weakness:

1. Acute inflammatory demyelinating polyneuropathy. This is the most common form of the disease.
2. Acute axonal motor neuropathy. During the study of the conduction of nerve impulses, signs of damage to the axons are revealed, due to which they are nourished.
3. Acute motor-sensory axonal neuropathy. In addition to the destruction of axons, the examination reveals symptoms of muscle weakness.

Another form of this disease is distinguished, which differs in its clinical manifestations (Miller-Fisher syndrome). Pathology is characterized by double vision, cerebellar disorders.

Diagnostic measures

Diagnosis of Guillain-Barré syndrome begins with questioning the patient, clarifying the symptoms and taking anamnesis. This disease is characterized by bilateral damage to the limbs and the preservation of the functions of the pelvic organs. Of course, there are atypical symptoms, therefore, a number of additional studies are required for differential diagnosis:

• Electromyography (assessment of the speed of movement of impulses along nerve fibers).
• Lumbar puncture (an analysis that can detect protein in the cerebrospinal fluid).
• Blood test.

It is important to differentiate the disease with oncological processes, encephalitis and botulism.

Why is Guillain-Barré syndrome dangerous?

Symptoms and treatment of pathology may vary, but the lack of therapy always leads to serious complications... The disease is characterized by gradual development. Only the appearance of weakness in the extremities forces the patient to seek help from a doctor. Usually 1-2 weeks pass until this point.

Such a period of time allows you to consult with doctors and pass necessary examination... On the other hand, it threatens with misdiagnosis and complicated treatment in the future. Symptoms appear very slowly and are often perceived as the onset of a different pathology.

At acute current the syndrome develops so rapidly that within a day a person may be paralyzed most of the body. Then tingling and weakness spreads to the shoulders, back. The longer the patient hesitates and postpones the visit to the doctor, the higher the likelihood that the paralysis will remain with him forever.

GBS treatment methods

It is important to hospitalize the patient in a timely manner, since in some cases Guillain-Barré syndrome is characterized by a rapid course and can lead to serious consequences. The patient's condition is kept under constant control, in case of deterioration, they are connected to a ventilator.

If the patient is lying, it is necessary to take care of the prevention of pressure sores. Various physiotherapy treatments can help protect against muscle atrophy.

In case of stagnant processes in the body, bladder catheterization is used to drain urine. For the prevention of vein thrombosis, "Heparin" is prescribed.

Intravenous administration of "Immunoglobulin" and plasmapheresis is a specific option of therapy. Plasma replacement is a procedure during which a liquid portion is removed from the blood and replaced with salt water (saline). Intravenous administration of "Immunoglobulin" allows you to strengthen the body's defenses, which helps him to more actively fight the disease. Both therapy options are especially effective at the initial stage of the development of the syndrome.

Rehabilitation after treatment

This disease causes irreparable damage not only to the nerve cells, but also to the periosteal muscles. During rehabilitation period the patient has to re-learn how to hold a spoon in his hand, walk and perform other actions necessary for a full-fledged existence. To restore muscle activity, traditional treatment is used (physiotherapy, electrophoresis, massage, exercise therapy, paraffin application).

During rehabilitation, a wellness diet and vitamin therapy are recommended to replenish the deficiency of micro- and macroelements. Patients diagnosed with Guillain-Barré syndrome, the symptoms of which are described in this article, are registered with a neurologist. They should periodically undergo a preventive examination, the main task of which is to identify early prerequisites for relapse.

Forecast and consequences

It usually takes 3 to 6 months for the body to fully recover. Do not expect a quick return to the usual rhythm of life. In many patients, the long-term effects of Guillain-Barré syndrome persist. The disease affects the sensitivity of the fingers and toes.

In about 80% of cases, previously lost functions are returned. Only 3% of patients remain disabled. Death is usually due to a lack of adequate therapy as a result of the development of heart failure or arrhythmia.

Preventive actions

No specific methods of preventing this disease have been developed. General recommendations include giving up addictions, a balanced diet, an active lifestyle, and timely treatment of all pathologies.

Let's summarize

Guillain-Barré syndrome is a disease characterized by muscle weakness and areflexia. It develops against the background of nerve damage as a result of an autoimmune attack. This means that the body's defenses perceive their own tissues as foreign and form antibodies against the membranes of their own cells.

The disease has its own characteristic symptoms, which allows you to recognize the disease in time and start therapy. Otherwise, the likelihood of developing autonomic dysfunctions and paralysis increases.