Barr syndrome symptoms. What is Guillain Barre syndrome symptoms of the disease

Piradov M.A. 2000

Rehabilitation is possible
This disease has at least eight different names - Landry's syndrome (after the French neurologist who first described it in 1859), Guillain-Barré-Stroll syndrome (scientists who have made a significant contribution to the study of the disease), acute polyradiculoneuritis, etc. Today on International classification 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 (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 damage 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 Neuroreanimation, Professor Mikhail PIRADOV tells more. Guillain-Barré syndrome is the most common cause of acute peripheral tetraparesis and paralysis. Neurological symptoms develop very quickly, while not only motor, but also sensory functions (primarily joint-muscular sensitivity) are violated, and sometimes very roughly, and tendon reflexes decrease until complete extinction. Pelvic disorders are not characteristic of GBS, but in a third of cases, the respiratory and swallowing muscles are seriously affected. In 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, it will be the same as that of a healthy person, and as a person he is not intellectually changed in the slightest. In 70 per cent. cases of GBS occur a few days after the onset of flu-like symptoms: mild fever, muscle pain, runny nose - all that is usually called acute respiratory infections. Approximately 15 per cent. cases, the syndrome appears after profuse diarrhea, in 5 percent. - after surgical manipulations, whether it be abortions, herniotomy, appendectomy or more complex operations. Sometimes the disease develops after various kinds of vaccinations. GBS occurs in any part of the world, at any time of the year, equally common in both sexes. The average age in most observations is about 40 years. At the same time, two small age peaks are distinguished: at 20-25 years old and over 60 years old. In classical cases, the diagnosis of GBS is simple and includes two obligatory 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 nerve impulse conduction through the muscles with the formation of a conduction block and protein-cell dissociation in the cerebrospinal fluid. Guillain-Barré syndrome is based on autoimmune 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 a whole range of polyneuropathies is combined under the name GBS: acute inflammatory demyelinating polyneuropathy (occurs in 75-80 percent 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: the disease is self-limiting. If a seriously ill patient is given only artificial lung ventilation for several months, the affected nerves are restored. And almost as complete as when applying the main 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 a ventilator for 3-6 months and be bedridden? The 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 in the country many patients with severe forms of GBS die. This is largely due to the fact that many hospitals are not equipped with high-quality respiratory equipment or do not have qualified personnel for long-term artificial lung ventilation. Patients die due to banal infections and bedsores. In addition, far from everywhere there is the possibility of performing plasmapheresis operations with the replacement of large volumes of plasma (up to 200 ml of plasma/kg for a course of treatment consisting of 4-5 operations). It is absolutely unacceptable to treat such patients in a rural or small district hospital - they must be hospitalized in larger hospitals equipped with the necessary facilities and equipment. A typical mistake in many cases remains the treatment of patients with GBS. hormonal drugs: special studies of more than one thousand patients have shown that hormones do not affect the rate of recovery of impaired functions, but, on the contrary, they 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 be deprived of a medical 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, a course of program plasmapheresis in our country is much cheaper. And the therapeutic effect of these two methods of treatment is the same: approximately 85-90 percent. of cases, a person with Guillain-Barré syndrome, despite the most 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 best case recovering 20 percent. people, and timely treatment of the Guillain-Barré syndrome with no less severity of the lesion gives a much greater effect. And if every year about 200 people suffer from the SSS in Moscow alone, it is a lot to fully restore the health of 180 people. In my practice, there was a case when the disease struck an 18-year-old guy, a candidate for master of sports in athletics: he could not breathe, swallow, move on his own. A year later, this man fulfilled the standard of a master of sports. And there are many such examples - after proper treatment of GBS, young women give birth to children, the vast majority of patients return to a full life.

Definition. Guillain-Barré syndrome (GBS) is a severe autoimmune disease of the peripheral nervous system that is the most common cause of acute flaccid tetraparesis.

Epidemiology. According to global epidemiological studies, GBS occurs in 1-2 cases per 100,000 population per year, regardless of gender and age. Incidence of GBS in selected cities and regions Russian Federation corresponds to global data and varies from 0.34 to 1.9 per 100,000, with an average of 1.8 per 100,000 population per year.

Etiology. The leading role in the pathogenesis of GBS development is assigned to autoimmune mechanisms, while a feature of this disease is a self-limiting, monophasic course with extremely rare relapses (up to 3-5%).

GBS usually develops 1 to 3 weeks after infectious disease(SARS, influenza, sinusitis, bronchitis, pneumonia, tonsillitis, measles, mumps, diarrhea, etc.). Epstein-Barr virus, Mycoplasma pneumoniae, Campylobacter jejuni and cytomegalovirus are considered as the main triggers of the autoimmune process in GBS. It is assumed that the antigenic similarity of the shell of an infectious agent with individual structural elements peripheral nerves(sheath, axon) causes the production of specific autoantibodies and the formation of circulating immune complexes that attack the peripheral nerves by the type of "molecular mimicry".

Less commonly, GBS occurs after vaccination (against influenza, hepatitis, rabies, etc.), surgical interventions (hernia repair, appendectomy, artificial termination of pregnancy, etc.), stressful situations, hypothermia, or against the background of complete health.

Classification. There are several forms of GBS, which differ in the course of the pathological process, the primary point of application of autoimmune aggression (nerve sheath or axonal rod), recovery prognosis, and clinical manifestations.

Acute inflammatory demyelinating polyneuropathy (AIDP), in which autoantibodies attack the myelin sheath of the nerve, is diagnosed most frequently (70–80%) worldwide, including in Russia, as part of GBS. The second most common (5-10%) place is occupied by axonal forms - acute motor and motor-sensory axonal neuropathies (OMAN and OMSAN), characterized by primary damage to the axons of peripheral nerves and differing from each other by involvement (OMSAN) or intactness (OMAN) sensitive fibers. Other forms of GBS (Miller Fisher syndrome, pharyngocervicobrachial, acute pandysautonomia, paraparetic, sensory, Bickerstaff stem encephalitis [BSE]) are extremely rare (1-3%).


reference Information. Bickerstaff stem encephalitis (BSE) is clinically characterized by a combination of depression of consciousness, ophthalmoplegia, ataxia, and hyperreflexia. Today, the autoimmune mechanism for the development of SES is beyond doubt: the condition in 23% of cases is associated with diarrhea caused by Campylobacter jejuni, or is often associated with infection with cytomegalovirus or Mycoplasma pneumoniae. Anti-GQ1b IgG antibodies are detected in 66-68% of patients with SES.

Diagnostic difficulties arise in the case of the presence of so-called cross syndromes (overlap-syndrome), when clinical, biochemical, serological and instrumental signs characteristic of 2 diseases or syndromes are simultaneously detected in the same patient. In foreign literature are presented clinical cases cross-syndromes of GBS and SES. The addition of flaccid tetraparesis to the symptoms of SES indicates a possible parallel lesion of peripheral nerves due to the development of an overlap syndrome with GBS, which aggravates the course of SES.

It turned out that up to 60% of SES cases are associated with the development of GBS and, as a rule, with its axonal forms. Despite the rarity of overlapping autoimmune neurological syndromes and the subtle differences in their constituent pathological conditions, their existence should always be kept in mind.

read also the post: Bickerstaff encephalitis(to the website)

GBS is also classified according to the severity of the condition, depending on clinical manifestations: [1 ] the mild form is characterized by the absence or minimal paresis, which does not cause significant difficulties in walking and self-care; [ 2 ] at moderate there is a violation of walking, limiting the patient's movement or requiring outside help or support; [ 3 ] with a severe form of the disease, the patient is bedridden and requires constant care, dysphagia is often observed; [ 4 ] in an extremely severe form, patients require mechanical ventilation (ALV) due to weakness of the respiratory muscles.

Clinic. The disease is characterized by a rapid (up to 4 weeks) increase in muscle weakness with initial involvement lower extremities and the distribution of "ascending type" from the distal to the proximal muscle groups. Patients complain of increasing weakness in the legs, difficulty walking. As the disease progresses, the hands are involved in the pathological process, often the mimic muscles. In some cases, symptoms debut with damage to the cranial nerves, or proximal muscle groups, and may predominantly affect the upper limbs. In every fourth or fifth case, the musculature of the trunk is involved in the pathological process, accompanied by weakness of the respiratory muscles (intercostal, diaphragm), as a result of which every third patient with gross tetraparesis requires artificial lung ventilation (ALV). With GBS, bulbar syndrome is often observed, primarily manifested by difficulty in swallowing, aspiration of fluid.

Muscle weakness is accompanied by sensory disorders - pain hypoesthesia of the polyneuritic type and loss of deep sensitivity, as well as tendon areflexia. Pain is a fairly common symptom of GBS. There are forms of the disease in which there is an isolated motor deficit. Pelvic dysfunctions are not typical for GBS and can be observed in bedridden patients, mainly in the form of urinary retention.

Often there are signs of autonomic dysfunction in the form of changes blood pressure(hypertension, hypotension), tachycardia, cardiac arrhythmias, hypersalivation, hyperhidrosis, paralytic ileus, which is an extreme manifestation of dynamic intestinal obstruction.

Diagnostics. The diagnosis of GBS is based on international criteria adopted by the World Health Organization in 1993. Signs needed for diagnosis: [ 1 ] progressive muscle weakness in the legs and/or arms; [ 2 ] absence or extinction of tendon reflexes in the first days of the disease.

Signs supporting the diagnosis: [ 1 ] relative symmetry of the lesion; [ 2 ] symptoms progress within no more than 4 weeks; [ 3 ] disturbance of sensitivity on polyneuritic type; involvement of the cranial nerves (most often - damage to the facial nerve); [ 5 ] recovery usually begins 2 to 4 weeks after the cessation of the increase in the disease, but can sometimes be delayed for several months; [ 6 ] autonomic disorders: tachycardia, arrhythmias, postural hypotension, hypertension, vasomotor symptoms; [ 7 ] lack of fever at the onset of the disease (some patients have fever at the onset of the disease due to intercurrent infections); fever does not exclude GBS, but raises the question of the possibility of another disease; [ 8 ] increase in protein in the cerebrospinal fluid with normal cytosis - protein-cell dissociation (observed from the second week of the disease); [ 9 ] electroneuromyographic (ENMG) signs of demyelination and/or axonal damage to peripheral nerves.

signs, questionable in diagnosis: [ 1 ] pronounced remaining asymmetry of motor disorders; [ 2 ] conductor level of sensory disturbances, pyramidal and cerebral symptoms; [ 3 ] persistent violations of pelvic functions; [ 4 ] more than 50 mononuclear leukocytes in the cerebrospinal fluid;[ 5 ] the presence of polymorphonuclear leukocytes in the cerebrospinal fluid.

These criteria apply to AIDP, axonal, paraparetic, and pharyngo-cervico-brachial forms. Miller Fisher syndrome and acute pandysautonomia are clinically significantly different from other forms of GBS, so it is difficult to apply the generally accepted criteria for diagnosing this disease for them. The diagnosis in these cases is established, first of all, on the basis of anamnestic data and the clinical picture of the disease.

Characteristics of Miller Fisher Syndrome: [ 1 2 ] rapidly developing ataxia, tendon areflexia, ophthalmoplegia; [ 3 ] moderate weakness in the limbs may occur; [ 4 ] pain sensitivity is usually preserved; disturbances of deep sensitivity can be observed; [ 5 ] full recovery within 1 - 3 months; [ 6 ] with ENMG, the amplitude is reduced, or there are no sensitive potentials; H-reflex is not called.

Characteristics of acute pandysautonomy: [ 1 ] the occurrence of neurological symptoms 1-2 weeks after a viral or bacterial infection; [2 ] the presence of an isolated lesion of the autonomic nervous system; [ 3 ] is often affected the cardiovascular system(postural hypotension, arterial hypertension, tachycardia, cardiac arrhythmias); [ 4 ] blurred vision, dry eyes, anhidrosis; [ 5 ] dysfunction gastrointestinal tract(paralytic ileus); [ 6 ] difficulty urinating, acute urinary retention; [ 7 ] increased sweating, bluish coloration of the skin of the hands and feet, cold extremities; [ 8 ] stunning, confusion due to hyponatremia associated with overproduction of antidiuretic hormone; convulsions may occur when the sodium content in the plasma is less than 120 mmol / l; [ 9 ] recovery is gradual and often incomplete.

read also the article: Acute pandysautonomy(to the website)

Neurophysiological criteria for diagnosis. Electroneuromyography (ENMG) is the only instrumental diagnostic method that allows confirming lesions of the peripheral nervous system and the diagnosis of GBS, respectively, as well as clarifying the nature of pathological changes (demyelinating or axonal) and their prevalence. The protocol and scope of ENMG studies in patients with GBS depend on the clinical manifestations of the disease:

[1 ] with predominantly distal paresis, long nerves on the arms and legs are examined: at least 4 motor and 4 sensory (motor and sensory portions of the median and ulnar nerves; peroneal, tibial, superficial peroneal and sural nerves on one side);

[2 ] an assessment of the main ENMG parameters is carried out: motor responses (distal latency, amplitude, shape and duration), the presence of blocks of excitation and dispersion of responses is assessed; the speed of propagation of excitation along the motor fibers in the distal and proximal areas is analyzed; sensory responses (amplitude) and speed of conduction of excitation along sensory fibers in the distal sections; late ENMG phenomena (F-waves): latency, form and amplitude of responses, chronodispersion value, percentage of dropouts are analyzed;

[3 ] in the presence of proximal paresis, it is mandatory to additionally study two short nerves (axillary, musculocutaneous, femoral, etc.) with an assessment of the parameters of the motor response (latency, amplitude, shape).

Neurophysiological criteria for the classification of GBS (R.Hadden, D.Cornblath, R.Hughes et al., 1998):

[1 ] group with a primary demyelinating lesion: at least one of the following signs must be present in at least 2 nerves or two signs in one nerve if all other nerves are non-excitable and the amplitude of the M-response at the distal point is 10% and more than the lower limit of the norm: the speed of propagation of excitation (ERV) is less than 90% of the lower limit of the norm, or less than 85% with the amplitude of the M-response at the distal point of less than 50% of the lower limit of the norm; distal latency of the M-response exceeds upper bound the norm is more than 10%, or more than 20% if the amplitude of the M-response at the distal point is below the lower limit of the norm; the presence of a dispersion or excitation block; the latency of the F-wave exceeds the upper limit of the norm by more than 20%;

[2 ] group with a primary axonal lesion: there are no signs of demyelination listed above in any nerve (excluding any one sign in 1 nerve, if the amplitude of the M-response at the distal point is more than 10% below the lower limit of the norm), and at least in two nerves the amplitude of the M-response at the distal point is more than 80% below the lower limit of the norm;

[3 ] group with non-excitable nerves: the M-response cannot be registered in any of the studied nerves or is only in one nerve with an amplitude at the distal point of more than 10% below the lower limit of normal;

[4 ] indefinite group: changes detected during stimulation ENMG do not meet the criteria for any of the above groups.

Thus, in order to make a diagnosis of GBS, it is necessary to clearly determine the history of the development of the disease, in combination with an assessment of the neurological status, to compare it with the criteria for diagnosing GBS (WHO; 1993). It is advisable to perform a lumbar puncture with a study of the liquor, as well as to confirm the neural level of the lesion and clarify the form of the disease according to the ENMG examination.

Additionally, the following diagnostic tests may be recommended to confirm the diagnosis and clarify the features of GBS in a particular case: [ 1 ] a blood test for autoantibodies to gangliosides, with a mandatory study of GM1, GD1a, and GQ1b if the patient has oculomotor disorders; [ 2 ] blood test for IgA antibodies to Campylobacter jejuni; [ 3 ] study of the content of biomarkers of heavy chains of neurofilament, tau protein and gliofibrillary acid protein in blood serum.

Differential Diagnosis. Based on the characteristics of the clinical picture of the disease, GBS should first of all be differentiated from conditions that can lead to the development of acute peripheral tetraparesis.
The data in the presented table reflects how laborious in certain cases is differential diagnosis with the SGB. However, differential diagnostic search is greatly simplified when using a unique algorithm developed by researchers of the Federal State Budgetary Institution "NTsN" RAMS, with the help of which the percentage of erroneous diagnoses in patients with acute flaccid tetraparesis syndrome is sharply reduced, and the economic costs associated with using the entire arsenal of diagnostic methods, minimized.


note: OBT - acute flaccid tetraparesis; EMG - electromyography; PNP - polyneuropathy; GBS - Guillain-Barré syndrome; LP - lumbar puncture; BHAK - biochemical analysis blood; RF - rheumatic factor; CRP - C-reactive protein; CPK - creatinine phosphokinase; MRI - magnetic resonance imaging (not less than 1 T); CT - computed tomography.
Pathogenetic (specific) therapy for GBS. Specific methods of GBS treatment include program plasmapheresis and a course of intravenous immunotherapy with immunoglobulin G preparations. The effectiveness of both methods is equivalent, and the choice of one or another type of therapy depends on its availability, and is also determined by the presence of indications and contraindications. The goal of pathogenetic therapy is, first of all, to stop the impact of autoimmune mechanisms leading to the development of polyneuropathy, which will stop the further increase in neurological symptoms, accelerate the onset of the recovery period, and also reduce the severity of residual deficiency.

Indications for specific therapy in GBS: [ 1 ] increase in neurological symptoms (up to 4 weeks of illness); [ 2 ] re-growth of neurological disorders after a temporary improvement (with or without treatment); [ 3 ] spontaneous stabilization of the state or regression of neurological deficit in patients with severe and extremely severe forms of GBS (a course of specific therapy can accelerate the rate of recovery and reduce the severity of the consequences).

High Volume Programmed Plasmapheresis:

[1 ] Mechanism of action: mechanical removal of autoantibodies and circulating immune complexes involved in damage to peripheral nerves.

[2 ] Contraindications: anemia, thrombocytopenia, hypofibrinogenemia, erosive and ulcerative lesions of the gastrointestinal tract, exacerbation of hemorrhoids, menses, coagulopathy, as well as any other reasons that may contribute to the development of hemorrhagic complications.

[3 ] Mode: from 3 to 5 sessions of plasmapheresis are performed with the obligatory removal of at least 35 - 50 ml / kg of the patient's plasma in one procedure. For a two-week course, plasma should be removed in an amount of at least 140-160 (up to 250) ml / kg of the patient's weight. The intervals between sessions should be short (usually every other day), but it is always necessary to evaluate the state of the hemostasis system after each procedure.

[4 ] Methodology: Plasmapheresis operations for GBS should be performed on continuous separators. A prerequisite that determines the effectiveness of this type of treatment is the simultaneous removal of a significant amount of plasma. The recommended blood sampling rate is 30-60 ml/min, the rotation speed of the centrifuge separators is up to 7500 rpm. As an anticoagulant, heparin is used at a dose of 50–350 U/kg. An alternative is the membrane (filtration) method of plasmapheresis using plasma filters or cascade plasma filtration.

[5 ] Replacement media: crystalloid solutions (isotonic sodium chloride and other saline solutions, glucose-potassium mixture), colloidal plasma substitutes (hydroxyethyl starch (HES) solutions), and donor albumin (5%, 10% or 20% solution), sometimes in combination with fresh frozen donor plasma (in case of antithrombin III deficiency). Albumin is recommended to be administered at the end of plasmapheresis operations, in volumes that make up at least 30-35% of the total amount of replacement media. Vascular access is carried out by puncture and catheterization of two peripheral veins or a central vein (subclavian or jugular) with the installation of a two-channel catheter. In the case of using peripheral access, a cuff is applied to the patient's shoulder area from the side of blood sampling, in which pressure from 40 to 70 mm Hg is maintained during blood sampling. Premedication is used very rarely in patients with GBS and includes an analgesic, antihistamine and a tranquilizer (midazolam). In unstable hemodynamics, drug correction (dopamine, dobutamine) can be used, which is carried out in parallel with rehydration and hemodilution. Hemodilution is performed in cases of hypovolemia with hemoconstriction (hematocrit over 45%, hemoglobin over 140 g/l). Intravenous infusion with low molecular weight colloids and crystalloids in a ratio of 1:3 is carried out at a rate of up to 20 ml/kg of the patient's weight. In patients with hypovolemia without hemoconstriction and dehydration, infusion preparation before plasmapheresis is carried out by introducing colloidal solutions (albumin, HES, gelatinol).

[6 ] Complications: may be associated with the operation of filters or separators (erythrocyte hemolysis, platelet destruction, blood overheating, inadequate intake of anticoagulant and / or replacement media into the system of highways); and/or due to the procedure itself (possible transfer of hepatitis, HIV, cytomegalovirus, etc. viruses through donor plasma, allergic reactions to injected solutions and drugs, hemorrhagic syndrome, fluid imbalance, activation of coagulation, complement system, fibrinolytic cascade and platelet aggregation). Prevention of complications of plasmapheresis is carried out during preparation, performing plasmapheresis sessions and subsequent management of the patient, and is aimed at preventing serious complications. A carefully collected history and preoperative examination, including endoscopy, will minimize the risk of hemorrhagic complications. Before starting therapy, adequate hydration of the patient is necessary. The following indicators are monitored and corrected during the entire plasmapheresis operation and after it: plasma electrolytes, hematocrit, blood clotting time according to the Sukharev method (during the operation, the clotting time should be at least 25 minutes, after the operation, three measurements are taken at intervals of 4 hours, 5 thousand IU of heparin are additionally injected subcutaneously with a clotting time of less than 5 minutes). It is recommended to adhere to the tactics of refusing to replace donor plasma, except in cases associated with severe hypovolemia and the need to correct the hemostasis system. Before starting blood sampling, a preliminary administration of 250 to 500 ml of isotonic sodium solution or 6% HES solution to the patient is carried out.

Intravenous immunotherapy:

[1 ] For the treatment of GBS, intravenous human immunoglobulin preparations containing at least 95% immunoglobulins of class G are used exclusively. Ready-to-use solutions of 5% or 10% are preferred.

[2 ] Mechanism of action: class G immunoglobulins block the production of autoantibodies, reduce the production of pro-inflammatory cytokines, reduce the formation of damaging circulating immune complexes, etc. Immunoglobulin class G is also the first-line drug in the treatment of GBS in children.

[3 ] Contraindications: low level of IgA in immunological examination, the presence of an anaphylactic reaction to the previous administration of human immunoglobulin preparations.

[4 ] Mode: the course of treatment consists of administering the drug at a dose of 0.4 g/kg of the patient's weight per day daily for 5 days (2 g/kg of body weight per course).

[5 ] Method: if the drug was stored in the refrigerator, it must be warmed to room temperature before administration to avoid pyrogenic reactions. The rate of administration is determined depending on the selected drug. Usually in the first 15 minutes it should not exceed 1.4 ml/kg/hour, later - 1.9 - 2.5 ml/kg/hour, for some drugs the maximum possible injection rate can reach 5 ml/kg/hour . An infusion pump is used to ensure the required rate of administration.

[6 ] Vascular access: if the peripheral access is intact, there is no need to install a central venous catheter.

[7 ] Complications: adverse reactions occur in less than 10% of cases. Among them headache, muscle pain, discomfort in the area chest, fever, nausea, vomiting. Reducing the rate of drug infusion will usually reduce these reactions. For the purpose of prophylaxis, paracetamol and Reopoliglyukin (or Infucol HES) can be administered before starting an IV infusion. Serious complications include: an increased risk of thromboembolism (prevented by a low rate of drug administration and the appointment of prophylactic doses of direct anticoagulants); urticaria, petechiae, migraine. Hemolysis and renal tubular necrosis are extremely rare.

Non-specific therapies for GBS. Non-specific treatments for GBS include the following: [ 1 ] qualified care for immobilized patients and patients on mechanical ventilation (prevention of bedsores, hypostatic pneumonia, contractures, etc.); [ 2 ] prevention and timely adequate correction of secondary infectious complications; [ 3 ] drug and non-drug prevention of deep vein thrombosis and thromboembolism pulmonary artery; [4 ] control and correction of swallowing and breathing disorders (tube feeding, mechanical ventilation), as well as hemodynamic disorders; [ 5 ] function status control Bladder and gastrointestinal tract; [ 6 ] correction of pain syndrome (pregabalin, gabapentin, carbamazepine, non-steroidal anti-inflammatory drugs, tramadol); [ 7 ] psychological support.

note! The complex of rehabilitation therapy (for GBS) deserves special attention, which is determined individually, taking into account the stage and severity of the disease, the presence of indications and contraindications. Patients with severe forms of GBS are indicated for: [ 1 ] with immobility - passive gymnastics, and [ 2 ] in the future - exercise therapy (a prerequisite is the duration and continuity of classes), massage of the limbs, verticalization for training hemodynamics, electrical stimulation, with developing contractures - paraffin therapy, etc. When the patient reaches the ability to stand, keeping the body in a vertical position, it is possible to connect classes on simulators for training walking (Lokomat and others). To speed up the recovery of limb function, exercises on simulators with biofeedback are shown (Armeo, Pablo, Amadeo, RT-300 and others)

Unacceptable: [1 ] prescribing glucocorticosteroid drugs: [ !!! ] it has been proven that this type of immunosuppressive therapy in GBS is absolutely ineffective; the use of corticosteroids in the acute period of the disease causes an erosive and ulcerative lesion of the mucosa of the gastrointestinal tract, which makes plasmapheresis impossible; but long oral intake corticosteroids in patients with GBS contributes to the maintenance of persistent residual effects and development side effects; [2 ]carrying out operations of software plasmapheresis by a discrete method; [ 3 ] the use in the treatment of GBS of preparations of VIG containing less than 95% of class G immunoglobulins or with an unspecified composition of immunoglobulins; [ 4 ] in the case of severe forms of GBS, non-compliance with international and domestic recommendations on the amount of pathogenetic therapy carried out: removal of plasma less than 140 ml/kg of body weight or administration of VIG less than 2 g/kg per course.

Forecast. With the correct therapeutic tactics for managing patients with GBS and timely pathogenetic therapy, the recovery prognosis is favorable - most patients return to their previous lifestyle and professional activity. It should be noted that axonal forms of GBS are characterized by slow and worse recovery, so this category of patients requires special attention - the early start of pathogenetic therapy to the fullest extent, with the implementation of all recommendations on the methodology and mode of its implementation.

Unfavorable prognostic factors are also a high rate of increase in neurological disorders (immobility of the patient in the first week of the disease), age over 60 years, the presence of previous diarrhea, registration during ENMG examination of low amplitudes of motor responses (less than 10% of the lower limit of normal) and some others. . However, in the case of adequate pathogenetic therapy, in the vast majority of patients after AIDP already by a month, and after axonal forms - by six months, from the onset of the disease they are able to move independently. Nevertheless, in 5–10% of patients who, as a rule, have undergone axonal forms of GBS, persistent gross neurological deficit persists, which completely changes the way of life and requires constant outside help.

additional literature:

article "Guillain-Barré Syndrome" by D.E. Kutepov, N.I. Litvinov, Clinical Hospital No. 1 of the Administration of the President of the Russian Federation, Moscow, Russia (Kazan Medical Journal, 2015, volume 96, No. 6) [read];

article "Guillain-Bare Syndrome: clinical features, diagnosis, prognosis" I.V. Damulin, Department of Nervous Diseases, First Moscow State Medical University. THEM. Sechenov (Neurological Journal, No. 6, 2013) [read];

article “Peculiarities of the course of GBS in Russia: an analysis of 186 cases” by N.A. Suponeva, E.G. Mochalova, D.A. Grishina, M.A. Piradov; Federal State Budgetary Institution "Scientific Center of Neurology" RAMS, Moscow; Moscow State University M.V. Lomonosov (journal "Neuromuscular Diseases" No. 1, 2014) [read];

presentation "Differential diagnostic aspects of diseases accompanied by AFP syndrome" L.I. Yasinskaya, PhD, Associate Professor, Belarusian State Medical University, Department of Nervous and Neurosurgical Diseases (2014) [read];

abstract for the degree of d.m.s. "Guillain-Barré syndrome: epidemiology, differential diagnosis, pathomorphosis, risk factors" Suponeva N.A., Moscow, 2013 [read]

Clinical guidelines on the diagnosis and treatment of Guillain-Barré syndrome(All-Russian Society of Neurologists, 2014) [download]


© Laesus De Liro

Synonyms: acute demyelinating polyradiculo(neuro)pathy, acute post-infectious polyneuropathy, Landry-Guillain-Barré syndrome, obsolete. Landry's ascending palsy.

Term Guillain-Barré syndrome is an eponym (i.e. giving a name) for a set of autoimmune acute inflammatory polyradiculoneuropathy syndromes characterized by progressive symmetrical flaccid paralysis in the muscles of the extremities and muscles innervated by cranial nerves (with the possible development of dangerous respiratory and swallowing disorders) with or without sensitive and autonomic disorders (unstable blood pressure, arrhythmias, etc.).

Quite often the disease develops directly after the transferred infections. In the classic version of the syndrome, ascending (from the legs) tetraparesis (paresis (paralysis) of all four limbs) is observed.

The diagnosis is made on the basis of an analysis of the characteristic clinical picture and is confirmed by an examination of the cerebrospinal fluid and an electromyographic study (EMG).

Treatment of Guillain-Barré syndrome is carried out in the intensive care unit under the control of breathing and swallowing functions. The main methods of specific therapy are approximately equally effective plasmapheresis and intravenous pulse therapy with immunoglobulin G. Good recovery in paralyzed muscles is observed in approximately 75-85% of cases.

Along with the fact that Guillain-Barré syndrome is classically presented as a 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, which represent a heterogeneous group of immunodependent peripheral neuropathies: Miller-Fisher syndrome (3 - 5%), acute motor axonal polyneuropathy and acute sensorimotor axonal polyneuropathy (make up 15-20%), and more rarely acute sensory polyneuropathy, acute pandysautonomy, acute cranial polyneuropathy, pharyngo-cervico-brachial option. As a rule, these variants are clinically usually more difficult than the main one.

  • Epidemiology

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

  • ICD-10 code G.61.0

Treatment

  • Basic provisions
    • The treatment of Guillain-Barré syndrome includes two components: non-specific supportive therapy and specific plasmapheresis therapy or pulse therapy with immunoglobulin class G.
    • Due to the possibility of developing decompensation with severe respiratory failure within a few hours, as well as cardiac arrhythmias, it is necessary to treat Guillain-Barré syndrome in the acute phase as emergency. In cases of development of acute respiratory failure in a medical institution, it must be possible to conduct 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. Hourly monitoring of VC, blood gases, blood electrolytes, heart rate, blood pressure, the state of the bulbar muscles (appearance and increase of swallowing disorders that do not bring relief of cough, hoarseness, speech disorders) are carried out. With bulbar paralysis with swallowing disorders, choking, pouring out the drink through the nose, the introduction of a nasogastric tube is indicated, and often intubation (for the prevention of aspiration and aspiration pneumonia). Tracheal intubation with mechanical ventilation is indicated with the development of respiratory failure, if the VC drops below 12–15 ml/kg, and with bulbar paralysis and swallowing and speech disorders below 15–18 ml/kg. In the absence of a tendency to restore spontaneous breathing within 2 weeks, a tracheostomy is performed.
    • Corticosteroids are not currently used because they have been shown to be ineffective. They do not improve the outcome of the disease.
  • Specific Therapy

Specific therapy with plasmapheresis or intravenous administration high-dose immunoglobulin begins shortly after diagnosis. Approximately equal effectiveness of both methods of treatment is shown, as well as the absence of an additional effect from a combination of these methods. Currently, there is no consensus on the choice of specific therapy.

Given that there is a high likelihood of spontaneous recovery, treatment of patients with mild Guillain-Barré syndrome can be limited to non-specific and supportive therapy. With the average severity of the process, and especially with severe course specific therapy begins as early as possible.

Treatment with immunoglobulin has some advantage over plasmapheresis, since it is easier and more convenient to use, has a significantly lower number of side effects, is easier on 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, preparations - octagam, sandoglobulin, intraglobulin, human normal immunoglobulin) is indicated for patients who are unable to walk more than 5 m without assistance, or more severe (with paralysis, respiratory and swallowing disorders) patients with 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 (total course dose of 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 administered 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), in total for the course a total of 200 - 250 ml / kg (at least 160 ml of plasma per 1 kg of body weight per course). In mild cases and in the recovery phase, plasmapheresis is not indicated. Plasmapheresis showed a rather high efficiency when administered to seriously ill patients, when therapy was started more than 30 days after 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 resume treatment with the same method, or use an alternative method.

  • Non-specific therapy and rehabilitation
    • It is necessary to prevent deep vein thrombosis of the lower leg in bedridden patients (especially with paralysis in the legs). Anticoagulants of indirect action phenylin or warfarin are administered orally in doses that stabilize the INR at the level of 2.0, or fraxiparine (nadroparin) 0.3 ml. s / c 1 - 2 times / day, or sulodexide (Wessel Due F) 2 times a day, 1 ampoule (600 LSU) / m for 5 days, then orally 1 caps (250 LSU) 2 times a day . Prevention is carried out before the time when the patient begins to get out of bed. If thrombosis has developed before the start of therapy, prophylaxis is carried out according to the same scheme. Bandaging with an elastic bandage of the legs to the middle of the thigh is also used (or stockings with graduated compression are used) and raising the legs by 10-15º. Shown passive and, if possible, active "walking in bed" with bending the legs, emitting walking for 5 minutes 3-5 times a day.
    • With paresis of the facial muscles, measures are taken to protect the cornea: instillation of eye drops, eye patch at night
    • Prevention of contractures and paralysis. To do this, passive exercises are carried out 1-2 times a day, they ensure the 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 lung infection in the form of 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 (effort and vibration while turning the body in the supine position) every 2 hours during the day.
    • Symptomatic therapy: antiarrhythmic, hypotensive, analgesic. With arterial hypotension, a drop in blood pressure (approximately blood pressure 100 - 110/60 - 70 mm Hg and below), colloid or crystalloid solutions are administered intravenously (isotonic solution of sodium chloride, albumin, polyglucin), and in case of insufficiency effect in combination with corticosteroids: prednisolone 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. isotonic sodium chloride solution and administered at a rate of 6 12 drops / min), or norepinephrine, or mezaton. For moderate pain, simple analgesics and non-steroidal anti-inflammatory drugs are used. 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 includes massage, therapeutic exercises, physiotherapy. Transcutaneous muscle stimulation is performed for muscle pain and paresis of the limbs.

Guillain-Barré syndrome is an acute form of progressive inflammatory polyneuropathy characterized by muscle weakness and polyneuritic sensitivity disorder. The disease is also called acute idiopathic polyneuritis, Landry's palsy, or inflammatory demyelinating polyradiculoneuropathy. The disease is a representative of autoimmune anomalies. Usually, pathology has specific signs that allow it to be recognized at the most early dates development and timely start adequate treatment. It has been proven that more than 80% of patients have a favorable prognosis and are completely cured.

autoimmune demyelination of nerves in Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) occurs in all age groups, but is especially prevalent in people aged 35-50 years, with equal frequency in both women and men. The incidence per 100,000 people is from 0.4 to 4 cases.

Causes of the disease

Scientists different countries have been studying the syndrome for over 100 years, but still cannot figure out the exact causes that provoke the onset of the disease.

It is believed that the appearance and development of an anomaly occurs due to a malfunction of the patient's immune system. When a person is completely healthy, when foreign cells enter the body, the immune system begins to fight the infection, rejecting all its dangerous elements. The patient is recovering. With GBS, the body begins to confuse "friends and foes": the patient's neurons are accepted as foreign and are "attacked". There is a destruction of the nervous system - a syndrome occurs.

Because of what there are violations in the work of the immune system itself is not fully known. The most common causes include:

  • Traumatic brain injury. Swipe head, any damage to it, as well as swelling, tumors or hemorrhages in the brain can become the main factor in the development of the syndrome. That is why when a patient contacts a specialist, first of all, the doctor should find out about the presence of any craniocerebral injuries.
  • Infections. Recent viral infections greatly weaken a person's immune system, thereby increasing the likelihood of GBS. The body's defense mechanism perceives neurons as an infection and continues to kill them with the help of white blood cells. In this case, the syndrome manifests itself one to three weeks after the infectious disease.
  • Allergies. The disease often develops in allergic people, for example, after chemotherapy, vaccination against polio and diphtheria, or major surgery.
  • genetic predisposition. Most diseases are inherited, and Guillain-Barré syndrome is no exception. If someone in the family has already suffered a pathology, then, most likely, it will also occur in descendants. In this case, you need to especially monitor your health: take care of your head and try not to start infectious diseases.

In childhood, the syndrome develops extremely rarely. The disease may be congenital or acquired. Any anomalies of intrauterine development can lead to the development of the syndrome:

  1. preeclampsia;
  2. Frequent use of drugs during pregnancy;
  3. The presence of autoimmune diseases in the mother;
  4. Prolonged infections during the period of bearing a child;
  5. Use of drugs, alcohol or smoking.

Acquired reasons include:

  1. Passive smoking of a child;
  2. Hormonal disruptions in the body;
  3. Vaccinations;
  4. self-medication;
  5. Metabolic disease;
  6. Disorders of a neurological nature;
  7. Development of tumor processes.

Symptoms

The disease can manifest itself in 3 forms:

  • Acute. All signs of the disease appear simultaneously within 1-2 days.
  • Subacute. Incubation period is 15 to 20 days.
  • Sluggish, chronic. This is one of the most dangerous forms, as it is practically untreatable.

The first symptoms of GBS in both children and adults resemble infection with a common viral respiratory infection:

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

In addition to common features, there are also more distinct ones:

  • Weakness of the limbs. Due to the destruction of nerve cells, there is a decrease or complete loss of sensitivity in the muscles. At first, pain appears only in the shin of the legs, after - discomfort affects the hands and feet. The patient is disturbed by tingling and numbness of the fingers. In severe cases, coordination of movements is disturbed: it becomes difficult for a person to hold a pen on his own, write with the affected limb. It is worth noting that the symptoms of the disease appear symmetrically: 2 arms or legs are simultaneously affected.
  • An increase in the abdomen, which is noticeable even visually. A protruding abdomen is one of the main indicators of the presence of the disease. This is due to the fact that the patient's breathing is rebuilt to the abdominal type due to the weakening of the diaphragm.
  • Incontinence. In a person with GBS syndrome, the healthy functioning of the bladder is disrupted, and urine begins to flow involuntarily.
  • Difficulty swallowing. The swallowing reflex is disturbed due to the fact that the muscles of the pharynx are weakened. In this case, the patient may even choke on saliva. The muscles of the mouth gradually weaken, which leads to discomfort while chewing food.

Guillain-Barré syndrome affects almost all organ systems, so unreasonable hypertension, tachycardia or banal visual impairment can be the first, albeit hidden, sign of the development of pathology.

What is the danger of the syndrome?

Usually the anomaly develops slowly over 2-3 weeks. First, there is a slight weakness in the joints, which intensifies over time and really begins to cause discomfort to the patient.

Immediately after the tingling, in the acute course of the disease, there is a general malaise, weakness in the shoulder and hip sections. After a few hours, there are difficulties in breathing. In this case, it is imperative to seek help from the hospital. Usually, the patient is immediately connected to the artificial respiration system, and then the necessary medication and physiotherapy are provided.

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

Also, in the absence of timely treatment, the patient is threatened with:

  1. Decreased immunity;
  2. respiratory failure;
  3. stiffness of the joints;
  4. peripheral paralysis;
  5. Problems of adaptation in society;
  6. Difficulty in life;
  7. Disability;
  8. Fatal outcome.

Diagnosis of the disease

In order to diagnose GBS in a patient, several aspects need to be clarified:

  • When was the last time a person was ill with any viral disease. It has been proven that in 80% of cases the syndrome occurs due to recent infections.
  • Is the patient currently taking any medications, and if so, which ones? They will also help cause the development of GBS.
  • How long ago the patient was vaccinated against any diseases.
  • Whether the patient suffers from autoimmune or neoplastic diseases.
  • Has the person had recent surgery?
  • Whether there were serious injuries to any part of the body.

The following studies should also be carried out:

  1. General analysis of blood and urine;
  2. Blood chemistry;
  3. Serological and virological examinations;
  4. Examination of cerebrospinal fluid;
  5. Magnetic resonance imaging;
  6. electrocardiography;
  7. Registration electrical activity muscles;
  8. X-ray or ultrasound of the affected area;
  9. Examination of external respiration;
  10. Study of the main vital indicators.

Muscle weakness in several limbs at the same time and tendon areflexia can be another clear sign of Guillain-Barré syndrome. This also includes various disorders in the pelvic area, polymorphonuclear leukocytes, asymmetries of paresis and sensitivity disorders.

Differential Diagnosis

Although the symptoms of GBS are similar to those of many other diseases (diphtheria, porphyria, transverse myelitis, botulism, and myasthenia gravis), they still need to be distinguished for proper treatment. At differential diagnosis the following factors should be taken into account:

  • If poliomyelitis is suspected, it is necessary to collect data from an epidemiological study, take into account the symptoms of the gastrointestinal tract, identify high cytosis in the cerebrospinal fluid, asymmetry of the lesion and the absence of sensory disturbances. The diagnosis can be confirmed by serological or virological analysis.
  • Polyneuropathy is characterized by the appearance of psychopathological signs, as well as pain in the pelvis and abdomen. The deviation of the main indicators from the norm in the urine also indicates the development of the disease.
  • Transverse myelitis accompanies a violation of the functioning of the pelvic organs, the absence of damage to the nerves of the skull.
  • Symptoms of an anomaly can be confused with a cerebral infarction. But in this case, the pathology affects the body in a few minutes and often leads to coma. An MRI will help determine the exact cause of the dysfunction of the body's systems.
  • Botulism is characterized by the absence of sensitivity disorders and any changes in the cerebrospinal fluid.

Treatment

Patients with a diagnosis of GBS must be hospitalized in a hospital. In about 30% of cases, it is necessary to carry out mechanical ventilation. Pathology therapy is performed at the following levels:

  1. resuscitation;
  2. symptomatic;
  3. Blood-purifying;
  4. Preparative;
  5. Muscle recovery;
  6. Preventive.

Resuscitation therapy

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

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

Symptomatic therapy

This type of treatment is carried out using various medicines:

  1. Antihypertensive drugs: Anaprilin, Metaprolol;
  2. Antibiotic therapy: "Norfloxacin";
  3. Drugs that help stabilize heart rate and pressure: Propranolol, Anaprilin (with tachycardia), Piracetam (with bradycardia);
  4. Low molecular weight heparin: "Gemapaxan", "Certoparin";
  5. Pain-relieving drugs - NSAIDs or Gabapentin, Pregabalin;
  6. Antipyretic, when the temperature rises above 38 degrees: "Ibuklin", "Next";
  7. Laxatives: Bisacodyl, Laxatin.

Plasmapheresis

One of the most effective procedures aimed at the treatment of GBS is hardware blood purification - plasmapheresis. It helps to stop the autoimmune process in the body. It is indicated for severe and moderate course of the disease. Usually, about 4-6 operations are performed with a break of one day. Instead of plasma, a special isotonic solution of sodium or albumin is injected into the blood, through which the blood is cleansed and the functioning of all body systems is normalized.

Surgical treatment of the syndrome

If mechanical ventilation was carried out for more than 7-10 days, a tracheostomy should be applied - an artificial respiratory throat. In severe cases, a gastrostroma may also be required - an opening in the stomach created by surgery to feed the patient.

Non-drug therapy with folk remedies

It is impossible to cure GBS with folk remedies. But to cope with some of its symptoms is quite real:

  • Elevated temperature. Plentiful drinking and airing of the room is recommended. Tea with lemon, decoctions with various berries and dried fruits will help bring down the temperature: cranberries, strawberries, currants, blueberries, raspberries and dried apricots. You can brew lime blossom, chamomile, St. John's wort, aspen buds, mint and thyme - leave for half an hour, then drink in small sips.
  • Bone ache. Lingonberry tea, a compress of fresh cabbage leaves, horseradish and burdock, baths with coniferous extract or decoctions of medicinal herbs will help to cope with it.
  • Weakness. Doctors recommend as often as possible to breathe fresh air and ventilate the room. You should try to eat more protein. You also need food rich in vitamins and minerals. And sweet strong tea or chocolate will help to cheer you up.

Rehabilitation

Due to the fact that the syndrome affects not only neurons, but also the circumosseous muscles, the patient will have to learn to walk again and perform simple movements with the limbs.

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

  1. electrophoresis;
  2. Trituration;
  3. Baths with radon;
  4. Massage;
  5. Baths for relaxation of the body and muscle tone;
  6. Masks and compresses with paraffin or beeswax;
  7. Recreational gymnastics.

During the recovery of the body, you should definitely go on a special therapeutic diet and take a course of vitamin preparations in parallel. Complexes that contain calcium, potassium, magnesium and vitamin B will be especially useful.

Patients with GBS should be registered with a neurologist and regularly undergo preventive examinations. It is worth remembering that timely high-quality therapy can return the patient to a full life.

Syndrome prevention

There is no special prevention of pathology. Doctors can only advise against any vaccinations for a year so that the disease does not return again. After this time, vaccination is allowed, but only if it is really necessary.

It is also worth refusing to take alcoholic beverages, avoid overheating, hypothermia and reduce physical activity. In this case, the recurrence of the disease is excluded.

Forecast

Most often, with Guillain-Barré syndrome, the prognosis is favorable. Usually, the normal functioning of the limbs is restored after 7-12 months in 85% of people. The disease becomes chronic in 7-15% of cases. Lethal outcome is approximately 5%. The cause of death may be respiratory failure, pneumonia or viral infections. But most often all this can be prevented by contacting a specialist in time.

Video: lecture on Guillain-Barré syndrome

Video: Guillain-Barré syndrome in the program “Live Healthy”

All people get colds. Recovery, as a rule, is not long in coming, and most of these patients do not even seek medical help. This happens most often, but sometimes things do not develop so favorably.

Introduction to Guillain-Barré Syndrome

V recovery period it is important to conduct physiotherapy (massage), electrical stimulation of the muscles of the pharynx (if there are swallowing disorders) and exercise therapy. The patient's condition is assessed both clinically and objectively using electroneuromyography.

After a short period of malaise with symptoms of SARS, numbness in the arms and legs, a feeling of crawling (paresthesia) may appear. After 1-2 days, weakness in the arms and legs joins; a person gradually becomes completely immobilized, loses the ability to self-service. Often there is perspiration, hoarseness, impaired eye movements. At the same time, patients are fully conscious, they hear and see everything, appearance such patients received the name "talking head". The contractility of the intercostal muscles and 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 sufficiently well enriched with oxygen, oxygen starvation occurs, due to respiratory failure may develop fatal outcome. Patients are shown treatment in the intensive care unit, since due to respiratory failure, it may always be necessary to carry out artificial ventilation of the lungs.

The disease was first described by Georges Guillain (1876-1961); Alexandre Barre (1880-1967) and Andre Strohl (1887-1977). The article describes a case of illness of two soldiers, a hussar and an infantryman, who developed paralysis within two weeks due to the absence of tendon reflexes. The attention of the authors was also attracted by the increase in protein in the cerebrospinal fluid in these patients. As already mentioned, such patients often need artificial lung ventilation, 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 the paramedics of the senior class, 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 are affected with equal 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.

Symptoms of Guillain-Barré Syndrome

The clinical picture in initial stage characterized by the presence of paresthesias (feelings of crawling) together or separately, perspiration when swallowing, sensitivity disorders (first of all, deep sensitivity is disturbed - vibrational and so-called proprioceptive sensitivity - that is, a joint-muscular feeling, thanks to which we feel the position of parts of our body. We usually do not pay much attention to this feeling, but it is thanks to it that we can walk and, without thinking, perform other actions with our hands and feet). V rare case 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). Muscle tone decreases, in severe cases, pelvic disorders occur (violation of urination and defecation).

In the advanced stage, motor, sensory disorders occur, the absence of tendon reflexes (areflexia) and autonomic disorders, which include heart rhythm disturbances, arterial hypertension, arterial hypotension, constipation, intestinal obstruction, diarrhea, urinary retention, impaired sweating. It is in the advanced stage that the weakness of the respiratory muscles can reach the point where the patient must be transferred to artificial lung 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 people talk about GBS, they mean exactly this subtype (Landry's ascending type). There are other, atypical forms, in which there is a pronounced lesion of the axon (the process of a neuron, along which nerve impulses are carried from the cell body to other neurons, the bodies of which lie either in the brain stem or in the spinal cord). And the processes of those neurons, in turn, go to the muscles and internal organs. These forms include acute sensory polyneuropathy, acute motor polyneuropathy, acute pandysautonomia (vegetative 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 (drooping of the 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 violation of the conduction of a nerve impulse along damaged nerves.

Causes and risks of Guillain-Barré syndrome

Until the end of science is not known. It is assumed that the disease is based on autoimmune mechanisms. It means that the immune system a person "revolts" against his own body, producing antibodies to certain nerve sheath molecules. 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 starting factor for the development of the disease are 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".

Diagnosis of Guillain-Barré Syndrome

It is very important to recognize the disease early stages and start proper treatment on time. Upon questioning, it becomes clear that the symptoms of the disease progressed in the patient within a few days after a short period of fever, accompanied by symptoms of SARS or loosening of the stool.

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 except I, II and VIII pairs can be affected); autonomic disorders (tachycardia, arrhythmia, postural hypotension, etc., see above), the absence of fever at the onset of the disease (some patients have a fever due to concomitant diseases). Symptoms of the disease develop quickly, but stop increasing by the end of 4 weeks. Recovery usually begins 2-4 weeks after the cessation of the increase in the disease, but can sometimes be delayed for several months.

Guillain-Barré syndrome has a number of similar symptoms to other diseases; it must be distinguished from: myasthenia gravis, botulism, paralysis caused by antibiotics, diseases of the spinal cord, transverse myelitis, acute necrotizing myelitis, lesions of the brain stem, "locked-in" syndrome, stem encephalitis , hypermagnesemia; porphyria 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.).

Treatment of Guillain-Barré syndrome

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

The patient should be taken to the intensive care unit of a specialized hospital as soon as possible, where he will be given a final diagnosis and specific treatment. For Guillain-Barré syndrome, this is staged plasmapheresis. Plasmapheresis is a procedure for removing blood from a patient and separating formed elements from plasma by centrifugation. Formed elements are returned back to the bloodstream, the plasma is removed. Instead of plasma, the patient is transfused with an albumin solution and electrolyte solutions. Together with the 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 "cuts off" the development of autoimmune inflammation, and the patient's condition stabilizes. After stabilization of the patient's condition, he begins to recover.

The method of treatment with type G immunoglobulins, which are obtained from the blood serum of about 9,000 donors, is also used. In this regard, the treatment is very expensive and rarely used.

Careful care is needed, monitoring of indicators of the general blood test, coagulogram and biochemistry.

Rehabilitation and prognosis of Guillain-Barré syndrome

Most patients have prospects for a good recovery.

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