At the beginning of the twentieth century, researchers Barre, Guillain and Strohl described an unknown disease in soldiers of the French army. The fighters were paralyzed, they did not have tendon reflexes, and there was a loss of sensitivity. Scientists examined the cerebrospinal fluid of patients and determined that it contained an increased protein content, while the number of other cells was absolutely normal. Based on the protein-cell association, as evidenced by the result of the analysis of cerebrospinal fluid, Guillain-Barré syndrome was diagnosed, which differs from other demyelinating diseases nervous system fast course and favorable prognosis. The studied soldiers recovered earlier than 2 months later.

Subsequently, it turned out that Guillain-Barré syndrome is not as harmless as the discoverers described it. For 20 years before the appearance of the description of the disease, the neuropathologist Landry observed patients with similar diseases. They also had flaccid paralysis, rapidly developing along the ascending nerve tracts. The rapid development of the disease was fatal. The damage to the nervous system was called Landry's palsy. It was subsequently revealed that Guillain-Barré syndrome can also be fatal by disabling muscle transmission in the diaphragm. But even in such patients, there was a laboratory picture of protein-cell association in the cerebrospinal fluid.

Then they decided to combine both diseases and assign the pathology one name Landry-Guillain-Barré syndrome, and to this day neuropathologists use the proposed terminology. but international classification diseases registered only one name: Guillain-Barré syndrome or acute postinfectious polyneuropathy.

Guillain-Barré syndrome, causes

Since the disease develops after infection, there is an assumption that it is she who causes the process of demylination of nerve fibers. However, no direct infectious agent has yet been found. Antigen-antibody complexes are deposited on the myelin fibers of the nervous tissue, which cause the destruction of myelin.

The myelin sheaths are located along the nerve trunk at regular intervals. They play the role of capacitors, so nerve impulses are transmitted several tens of times faster and reach the "addressee" unchanged. When Guillain-Barré syndrome develops, its causes lie in a decrease in the capacity of the "capacitors". As a result, the nerve transmission is delayed and becomes ineffective. The person intends to clench his fingers, but can only move them.

This is the essence of all demilienizing diseases of the nervous system. When a person develops Guillain-Barré syndrome, impulse transmission to major vital organs suffers, such as:

• Cardiac muscle;
• Diaphragm;
• Swallowing muscles.

With the paralysis of these organs, the vital activity of the organism stops.

Guillain-Barré syndrome, symptoms

The paradox of the disease lies in the fact that in acute development, a successful outcome occurs in two-thirds of patients, and in chronic course the prognosis is poor.

Guillain-Barré syndrome begins after acute viral infections, most often respiratory. In the form of complications after the flu, a person develops general weakness, which is transmitted to the arms and legs. Subsequently, the subjective feeling of weakness progresses to flaccid paralysis. In an acute course, the following symptoms develop:

• The disappearance of the swallowing reflex;
• Paradoxical type of breathing - during inhalation, the abdominal wall does not expand, but, on the contrary, collapses;
• Violation of the sensitivity of the distal extremities by the type of "gloves" and "stockings".

In severe cases, breathing is shut off due to paralysis of the diaphragm.

When it develops primarily chronic syndrome Guillain-Barré, symptoms increase slowly over several months, but at their peak they are difficult to treat. As a result, the effects of paralysis remain for the rest of your life.

Clinical course of Guillain-Barré syndrome

During the course of the disease, 3 stages are determined:

• Prodromal;
• Razgara;
• Exodus.

The prodromal period is characterized by general malaise, muscle pain in the arms and legs, and a slight increase in temperature.

During the peak period, all the symptoms characteristic of Guillain-Barré syndrome appear, which reach their peak by the end of the phase.

The outcome stage is characterized by the complete absence of signs of any infection, but manifests itself only with neurological symptoms. The disease ends either with a complete restoration of all functions, or with disability.

Guillain-Barré syndrome, treatment

With an acute onset, especially when Guillain-Barré syndrome develops in children, resuscitation measures are the first to be found. Timely connection of the device artificial respiration saves the patient's life.

A long stay in the intensive care ward requires additional treatment, bedsores are prevented and the fight against infections, including hospital ones.

The uniqueness of Guillain-Barré's disease lies in the fact that with adequate artificial ventilation of the lungs, the regeneration of the myelin sheaths occurs without any medication.

Modern methods of treatment of Guillain-Barré syndrome, in children in particular, involve plasmapheresis. Cleansing blood plasma from autoimmune complexes prevents the progression of demyelination of nerve fibers and significantly shortens the period of artificial ventilation.

Currently, Guillain-Barré syndrome can be treated with immunoglobulin infusions. The method is expensive but effective. In the recovery period, methods of physiotherapy, physiotherapy exercises and massage are used.

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

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

Often, the disease develops immediately 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 research cerebrospinal fluid and electromyographic examination (EMG).

Treatment of Guillain-Barré syndrome is carried out in the intensive care unit under the control of respiratory 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 about 75-85% of cases.

Along with the fact that the Guillain-Barré syndrome is classically presented as demyelinating polyneuropathy with ascending weakness, called acute inflammatory demyelinating polyneuropathy and constituting 75 - 80% of cases, several atypical variants or subtypes of this syndrome are described and distinguished in the literature, representing a heterogeneous group of immunodependent 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 option. As a rule, these options are clinically usually more severe than the main one.

• Epidemiology

  Guillain-Barré syndrome is 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 years it reaches 8.6 per 100,000. The mortality rate ranges from 2 to 12%.

• ICD-10 code G.61.0

Treatment

• Basic Provisions
  • Treatment of Guillain-Barré syndrome includes two components: nonspecific supportive therapy and specific therapy with plasmapheresis or pulse therapy with class G immunoglobulin.
  • Due to the possibility of developing decompensation with severe respiratory failure for several hours, as well as disorders heart rate, it is necessary to treat Guillain-Barré syndrome in the acute phase as 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. Hourly monitoring of VC, blood gases, blood electrolytes, heart rate, blood pressure, the state of bulbar muscles (the appearance and growth of swallowing disorders, which does not bring relief to cough, hoarseness, speech impairment). With bulbar paralysis 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). Intubation of the trachea with mechanical ventilation is shown 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. If there is no tendency to restore spontaneous breathing, tracheostomy is performed within 2 weeks.
  • 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.

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 an average severity of the process, and especially with severe course specific therapy begins 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 enough high efficiency when prescribing to seriously ill patients, when starting therapy in a period of 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 and rehabilitation
  • It is necessary to prevent deep vein thrombosis of the lower leg in bedridden patients (especially with paralysis in the legs). Orally administered anticoagulants 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, prophylaxis is carried out according to the same scheme. They also apply bandaging with an elastic bandage of the legs to the middle of the thigh (or use stockings with graduated compression) and raising the legs by 10-15º. Shown is passive and, if possible, active "walking in bed" with bending 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: eye drops, blindfold at night
  • Prevention of contractures and paralysis. To do this, passive exercises are carried out 1 - 2 times a day, ensure the correct position in bed (comfortable bed, support for the feet), massage the limbs. Subsequently, an active physiotherapy exercises.
  • Prevention of bedsores - change the position in bed every 2 hours, wipe the skin with special compounds, use anti-bedsore mattresses.
  • Prevention of pulmonary infection in the form of breathing exercises, as early as possible mobilization of the patient. With a decrease in the vital capacity of the lungs, difficulty in separating bronchial secretions, massage is indicated (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 solution of chloride sodium, albumin, polyglucin), and if the effect is insufficient in combination with corticosteroids: prednisolone 120 - 150 mg., dexazone 8 - 12 mg .. If these funds are insufficient, vasopressors are used: dopamine (50 - 200 mg. solution of sodium chloride and injected at a speed of 6-12 drops / min), or norepinephrine, or mezaton. For moderate pain, simple analgesics and non-steroidal anti-inflammatory drugs are used. With severe pain syndrome, tramal or cabamazepine (tigretol) or gabapentin (neurontin) are used, 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, remedial gymnastics, physiotherapy procedures. Percutaneous muscle stimulation is performed for muscle pain and paresis of the limbs.

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

Epidemiology... According to world epidemiological studies, GBS occurs in 1 - 2 cases per 100,000 population per year, regardless of gender and age. The incidence of GBS in individual cities and regions of the Russian Federation corresponds to global data and varies from 0.34 to 1.9 per 100,000, 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 develops, as a rule, 1 to 3 weeks after an infectious disease (ARVI, 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 sheath of an infectious agent with individual structural elements of peripheral nerves (sheath, axon) causes the production of specific autoantibodies and the formation of circulating immune complexes that attack peripheral nerves in a molecular mimicry manner.

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... Several forms of GBS are distinguished, differing in the characteristics of the course of the pathological process, the primary point of application of autoimmune aggression (nerve sheath or axonal rod), recovery prognosis, and clinical manifestations.

Most often (70 - 80%) throughout the world, including in Russia, acute inflammatory demyelinating polyneuropathy (AIDP) is diagnosed within GBS, in which autoantibodies attack the myelin sheath of the nerve. The second most frequent (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, pharyngo-cervico-brachial, acute pandizautonomy, paraparetic, sensory, Bickerstaff brainstem encephalitis [SEB]) are diagnosed extremely rarely (1-3%).

reference Information... Bickerstaff stem encephalitis (SEB) is clinically characterized by a combination of depression of consciousness, ophthalmoplegia, ataxia and hyperreflexia. Today, the autoimmune mechanism of SEB development 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. In 66 - 68% of patients with SEB, anti-GQ1b IgG antibodies are detected.

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

It turned out that up to 60% of cases of SEP 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, one should always be aware of their existence.

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

GBS is also classified according to the severity of the condition, depending on the clinical manifestations: [ 1 ] light form characterized by the absence or minimal paresis, which does not cause significant difficulties in walking and self-care; [ 2 ] with moderate severity, there is a violation of walking, restricting the patient in 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 extremely severe form, patients require mechanical ventilation (ALV) due to the 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 limbs and ascending from distal to proximal muscle groups. Patients complain of increasing weakness in the legs, difficulty walking. As the disease progresses, the hands, often the mimic muscles, are involved in the pathological process. In some cases, the symptomatology debuts with lesions of the cranial nerves, or proximal muscle groups, can mainly affect the upper limbs. In every fourth to 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 mechanical 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 - painful hyposthesia of the polyneuritic type and loss of deep sensitivity, as well as tendon areflexia. Pain is a common symptom of GBS. There are forms of the disease in which there is an isolated motor deficit. Pelvic dysfunctions are not common in GBS and may occur in bedridden patients, mainly in the form of urinary retention.

Signs of autonomic dysfunction are often present in the form of changes in 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 established on the basis of international criteria adopted by the World Health Organization in 1993. Signs required for the 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 ] the relative symmetry of the lesion; [ 2 ] symptoms progress within no more than 4 weeks; [ 3 ] violation of sensitivity of the polyneuritic type; involvement of cranial nerves (most often - damage to the facial nerve); [ 5 ] recovery usually begins 2 - 4 weeks after the cessation of the growth of the disease, but sometimes it can be delayed for several months; [ 6 ] autonomic disorders: tachycardia, arrhythmias, postural hypotension, hypertension, vasomotor symptoms; [7 ] the absence 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 ] an 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 the diagnosis: [ 1 ] Expressed persistent asymmetry of motor disorders; [ 2 ] conduction level of sensitive disorders, pyramidal and cerebral symptoms; [ 3 ] persistent disorders 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 are applicable for ATP, axonal, paraparetic and pharyngo-cervico-brachial forms. Miller Fisher's syndrome and acute pandizautonomy are clinically significantly different from other forms of GBS, so it is difficult to apply the generally accepted diagnostic criteria for this disease for them. The diagnosis in these cases is established primarily 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 ] there may be moderate weakness in the limbs; [ 4 ] pain sensitivity, as a rule, is preserved; there may be violations of deep sensitivity; [ 5 ] full recovery within 1 - 3 months; [ 6 ] at ENMG, the amplitude is reduced, or there are no sensitive potentials; The H-reflex is not triggered.

Characteristics of acute pandizautonomy: [ 1 ] the onset of neurological symptoms 1 - 2 weeks after the transferred viral or bacterial infection; [2 ] the presence of an isolated lesion of the autonomic nervous system; [ 3 ] often affects the cardiovascular system (postural hypotension, arterial hypertension, tachycardia, heart rhythm disturbances); [ 4 ] blurred vision, dry eyes, anhidrosis; [ 5 ] functional impairment gastrointestinal tract(paralytic ileus); [ 6 ] Difficulty urinating, acute urinary retention; [ 7 ] increased sweating, bluish discoloration 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 plasma is less than 120 mmol / l; [ 9 ] recovery is gradual and often incomplete.

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

Neurophysiological Diagnostic Criteria... Electroneuromyography (ENMG) - the only one instrumental method diagnostics, which allows to confirm lesions of the peripheral nervous system and the diagnosis of GBS, respectively, as well as to clarify the nature of pathological changes (demyelinating or axonal) and their prevalence. The protocol and scope of the ENMG study in patients with GBS depends 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 ] the assessment of the main ENMG parameters is carried out: motor responses (distal latency, amplitude, shape and duration), the presence of blocks of excitation conduction 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 the rate of conduction of excitation along sensory fibers in distal parts; late ENMG-phenomena (F-waves): latency, form and amplitude of responses, amount of chronodispersion, percentage of loss are analyzed;

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

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

[1 ] group with primary demyelinating lesion: the presence of at least one of the following signs in at least 2 nerves or two signs in one nerve is necessary, 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 velocity of propagation of excitation (SRV) 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 less than 50% of the lower limit of the norm; the distal latency of the M-response exceeds the upper limit of the normal by more than 10%, or more than 20% if the amplitude of the M-response at the distal point is lower than the lower limit of the normal; the presence of dispersion or block of excitation conduction; F-wave latency exceeds the upper limit of the norm by more than 20%;

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

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

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

Additionally, the following diagnostic tests can 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 ] a blood test for IgA antibodies to Campylobacter jejuni; [ 3 ] study of the content of biomarkers of heavy chains of neurofilament, tau protein and gliofibrillar acidic 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 reflect how laborious the differential diagnosis of GBS is in certain cases. However, the differential diagnostic search is greatly simplified when using a unique algorithm developed by researchers of the FGBU "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 the use of the entire arsenal of diagnostic methods, reduced to a minimum.

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 (at least 1 T); CT - computed tomography.
Pathogenetic (specific) therapy for GBS... Specific methods of treating GBS include programmed plasmapheresis and a course of intravenous immunotherapy with immunoglobulins G. The effectiveness of both methods is equal, 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 effect of autoimmune mechanisms leading to the development of polyneuropathy, which will allow to stop the further increase in neurological symptoms, accelerate the onset of the recovery period, and also reduce the severity of residual deficit.

Indications for specific therapy for GBS: [ 1 ] an increase in neurological symptoms (up to 4 weeks of illness); [ 2 ] re-growth of neurological disorders after 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 carried out 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 assess the state of the hemostatic system after each procedure.

[4 ] Methodology: plasmapheresis operations for GBS should be carried out on continuous separators. A prerequisite that determines the effectiveness of this type of treatment is the simultaneous removal of a significant volume of plasma. The recommended blood sampling rate is 30-60 ml / min, the speed of rotation of the centrifuge of the 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 ] Substitution media: crystalloid solutions (isotonic sodium chloride solution and other saline solutions, glucose-potassium mixture), colloidal plasma substitutes (solutions of hydroxyethyl starch (HES)), as well as 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 constituting 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 the peripheral access, a cuff is applied to the patient's shoulder area from the side of blood sampling, in which a pressure of 40 to 70 mm Hg is maintained during blood sampling. Premedication is extremely rare in patients with GBS and includes an analgesic, an antihistamine, and a tranquilizer (midazolam). With 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 blood contraction (hematocrit is more than 45%, hemoglobin is 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 blood control 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 (hemolysis of erythrocytes, destruction of platelets, overheating of blood, inadequate intake of anticoagulant and / or replacement media into the system of highways); and / or due to the procedure itself (possible transfer through donor plasma of hepatitis, HIV, cytomegalovirus, etc., allergic reactions on 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 patient management, and is aimed at preventing serious complications. A thoroughly collected history and preoperative examination, including EGDS, will minimize the risk of hemorrhagic complications. Before starting therapy, it is necessary to carry out adequate hydration of the patient. Monitoring and correction are carried out during the entire plasmapheresis operation and after it of the following indicators: plasma electrolytes, hematocrit, blood coagulation time according to the Sukharev method (during the operation, the coagulation time should be at least 25 minutes, after the operation, three measurements are taken at intervals of 4 hours, additionally, 5 thousand units of heparin are injected subcutaneously with a clotting time of less than 5 minutes). It is recommended to adhere to the tactics of refusing to replace with donor plasma, except in cases associated with severe hypovolemia and the need to correct the hemostatic system. Before the start of blood sampling, the patient is preliminarily administered from 250 to 500 ml of isotonic sodium solution or 6% HES solution.

Intravenous immunotherapy:

[1 ] For the treatment of GBS, only intravenous human immunoglobulin preparations containing at least 95% of class G immunoglobulins are used. 5% or 10% ready-to-use solutions 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. Class G immunoglobulin is also the first-line drug for GBS therapy in children.

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

[4 ] Regimen: 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 up to room temperature 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 administration rate can reach 5 ml / kg / hour ... An infusomat is used to ensure the required infusion rate.

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

[7 ] Complications: adverse reactions occur no more often than in 10% of cases. Among them headache, muscle pain, discomfort in the area chest, fever, nausea, vomiting. Reducing the rate of infusion of the drug will usually reduce these reactions. For the purpose of prophylaxis, paracetamol and "Reopolyglucin" (or "Infukol HES") can be administered before starting the intravenous infusion. Serious complications include: an increased risk of thromboembolism (prevented by the low rate of administration of the drug 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 pressure ulcers, 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 ] control over the state of the functions of the bladder and gastrointestinal tract; [ 6 ] correction of pain syndrome (pregabalin, gabapentin, carbamazepine, non-steroidal anti-inflammatory drugs, tramadol); [ 7 ] psychological support.

note! Special attention should be paid to the complex of rehabilitation therapy (for GBS), 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 shown: [ 1 ] in case of immobility - passive gymnastics, and [ 2 ] hereinafter - exercise therapy (a prerequisite is the duration and continuity of classes), massage of the extremities, verticalization for training hemodynamics, electrical stimulation, with developing contractures - paraffin therapy, etc. When the patient reaches the ability to stand, holding the torso in upright position, it is possible to connect classes on walking simulators (Lokomat and others). To accelerate the recovery of limb function, exercises are shown on simulators with biofeedback (Armeo, Pablo, Amadeo, RT-300 and others)

Unacceptable: [1 ] the appointment of glucocorticosteroid drugs: [ !!! ] it has been proven that this type of immunosuppressive therapy for GBS is absolutely ineffective; the use of corticosteroids in the acute period of the disease causes erosive and ulcerative damage to the mucous membrane of the gastrointestinal tract, which makes it impossible to carry out plasmapheresis; and long-term oral administration of corticosteroids in patients with GBS contributes to the preservation of persistent residual effects and the development of side effects; [ 2 ] carrying out operations of software plasmapheresis by a discrete method; [ 3 ] the use in GBS therapy of GIG preparations 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 volume of pathogenetic therapy: removal of plasma less than 140 ml / kg body weight or the introduction 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 prognosis for recovery is favorable - most patients return to their previous lifestyle and professional activity. It should be noted that axonal forms of GBS are characterized by slower and worse recovery, therefore, this category of patients requires special attention - early initiation of pathogenetic therapy as fully as possible with the implementation of all recommendations on the method and regimen of its implementation.

Adverse 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 of low amplitudes of motor responses during ENMG examination (less than 10% of the lower limit of the norm) and some others. ... However, in the case of adequate pathogenetic therapy in the overwhelming majority of patients after OVDP 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 underwent, as a rule, axonal forms of GBS, persistent gross neurological deficit remains, which completely changes the way of life and requires constant outside help.

additional literature:

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

article "Guillain-Bare Syndrome: clinical features, diagnostics, prognosis "I.V. Damulin, Department of Nervous Diseases, I.M. THEM. Sechenov (Neurological journal, No. 6, 2013) [read];

article "Features of the course of GBS in Russia: analysis of 186 cases" N.А. Suponeva, E.G. Mochalova, D.A. Grishina, M.A. Pirads; FSBI " Science Center 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, Ph.D., Associate Professor, EE "Belarusian State Medical University", Department of Nervous and Neurosurgical Diseases (2014) [read];

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

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

© Laesus De Liro

RCHD (Republican Center for Healthcare Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical protocols MH RK - 2016

Guillain-barré syndrome (G61.0)

Neurology

general information

Short description

Approved
Joint Commission on the Quality of Medical Services
Ministry of Health and Social Development of the Republic of Kazakhstan
dated November 29, 2016
Protocol No. 16

Guillain-Barré Syndrome(Guillain-Barrésyndrome) (GBS) is an acute, rapidly progressive autoimmune lesion of the peripheral nervous system, manifested in the form of paresthesia of the extremities, muscle weakness and / or flaccid paralysis (monophasic immune-mediated neuropathy).

Synonyms for Guillain-Barré syndrome: acute inflammatory demyelinating polyneuropathy, acute idiopathic polyneuropathy, infectious polyneuritis (polyneuropathy), acute polyradiculitis, Guillain-Barré-Strohl syndrome (Guillain-Barré-Barré-Strohlsyndrome) (Guillain-Barré-Strohlsyndrome-Guillain-Barré-Barré-Barré-Barré-Barré Syndrome), Landéna syndrome Landry-Guillain-Barré-Strohlsyndrome syndrome, Landry'ssyndrome syndrome, Landry's ascendingparalysis, French polio, etc.
A feature of this disease is a self-limiting, monophasic course with extremely rare relapses.

The ratio of the codes ICD-10 and ICD-9

Classification

Classification

GBS refers to both the number of neuroinfections and post-infectious conditions. Several forms of GBS are distinguished, differing in the characteristics of the course of the pathological process, the primary point of application of autoimmune aggression (nerve sheath or axonal rod), recovery prognosis, and clinical manifestations.

According to modern concepts, there are at least 8 varieties (clinical variants / subtypes) of Guillain-Barré syndrome:
1) acute inflammatory demyelinating polyneuropathy (the classic form of Guillain-Barré syndrome);
2) acute motor-sensory axonal neuropathy (OMSAN);
3) acute motor-axonal neuropathy (OMAN);
4) Miller-Fisher syndrome (SMF);
5) acute panautonomous neuropathy (acute panautonomous Guillain-Barré syndrome, acute pandizautonomy);
6) stem Bickerstaff encephalitis (Bickerstaff);
7) pharyngo-cervico-brachial variant;
8) acute cranial polyneuropathy.
There are also options for combining Miller-Fisher syndrome with other forms of Guillain-Barré syndrome (MFS / GBS overlapsyndrome).

GBS is also classified according to the severity of the condition, depending on the clinical manifestations:
· Mild forms are characterized by the absence or minimal paresis, which do not cause significant difficulties in walking and self-care;
With moderate severity, there is a violation of walking, limiting the patient in movement or requiring outside help or support;
With a severe form of the disease, the patient is bedridden and requires constant care, dysphagia is often observed;
In an extremely severe form, patients require artificial lung ventilation (ALV) due to weakness of the respiratory muscles.

Neurophysiological criteria for the classification of GBS (R. Hadden, D. Cornblath, R. Hughesetal., 1998).
Group with primary demyelinating lesion:
the presence of at least one of the following signs in at least 2 nerves or two signs in one nerve is necessary if all other nerves are unexcited and the amplitude of the M-response at the distal point is 10% or more of the lower limit of the norm:
· The speed of propagation of excitation (SRV) is less than 90% of the lower limit of the norm, or less than 85% when the amplitude of the M-response at the distal point is less than 50% of the lower limit of the norm;
· Distal latency of the M-response exceeds the upper limit of the norm by more than 10%, or more than 20% if the amplitude of the M-response at the distal point is lower than the lower limit of the norm;
· The presence of dispersion or block of excitation conduction;
· F-wave latency exceeds the upper limit of the norm by more than 20%.

Primary axonal lesion group:
There are no the above signs of demyelination 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% lower than 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.

Group with non-excitable nerves:
· The M-response cannot be registered in any of the examined nerves or is present only in one nerve with an amplitude at the distal point more than 10% below the lower limit of the norm.

Undefined group:
· The changes revealed during stimulation ENMG do not meet the criteria of any of the above groups.

Diagnostics (outpatient clinic)

DIAGNOSTICS AT THE AMBULATORY LEVEL

Diagnostic criteria:
Complaints:
· Increasing muscle weakness in the arms and / or legs;
Numbness and decreased sensitivity;
· Increased sensitivity (tactile, temperature, etc.) in the hands and feet;
· Pain in the back, shoulder and pelvic girdle;
· Violation of swallowing, both solid food and liquid;
· Violation of respiratory functions, up to the lack of spontaneous breathing, due to the weakening of the respiratory muscles, weakening of the voice and cough;
• heart rate disorder, in some it can be very frequent, in others it can be slowed down;
· Facial muscle paralysis;
· Increased sweating;
· Fluctuations in blood pressure;
· The occurrence of uncontrolled emission of urine is possible;
• loss of tendon reflexes;
• shaky and unsure gait, impaired coordination of movements;
Changes in the volume of the abdomen, this happens because it is difficult for a person to breathe with the help of the diaphragm, and he is forced to use the abdominal cavity;
· Decreased visual acuity - most often there is a split and strabismus.
Symptoms are common in adults, children and newborns.

Anamnesis: GBS develops, as a rule, 1-3 weeks after an infectious disease (ARVI, influenza, sinusitis, bronchitis, pneumonia, tonsillitis, measles, mumps, diarrhea, etc.).
Neurological symptoms appear suddenly; most patients have pain and paresthesias.
When taking an anamnesis, it is important 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, can develop after an intestinal infection caused by Campylobacterjejuni, after infections caused by herpes viruses (cytomegalovirus, Epstein-Barr virus, varicella-zoster virus), Haemophilusinfluenzae, mycoplasma, measles, mumps, Lyme borreliosis, etc. development of Guillain-Barré syndrome is possible.
· Vaccination (rabies, tetanus, influenza, etc.);
· Surgical interventions or injuries of any localization;
· 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.

There is a certain pattern in the increase in symptoms, based on which 3 stages of the disease are distinguished:
· Progression (1-4 weeks) - the appearance and intensification of neurological disorders;
· Plateau (10-14 days) - stabilization of the clinical picture;
· Reverse development (from several weeks to 2 years) - restoration of the normal functioning of the body.

Physical examination includes:
· general somatic status: general condition and its severity, body temperature, measurement of the patient's weight, examination of the skin, respiration, pulse, blood pressure, condition internal organs(lungs, heart, liver, kidneys, etc.).
· neurological status:
Neurological examination is aimed at identifying and assessing the severity of the main symptoms of Guillain-Barré syndrome - sensory, motor and autonomic disorders.
· Assessment of the strength of the muscles of the extremities;
· Study of reflexes - areflexia is characteristic of Guillain-Barré syndrome (that is, the absence of most reflexes);
· Sensitivity assessment - the presence of areas of the skin with a feeling of numbness or tingling;
· Assessment of the function of the pelvic organs - possible short-term urinary incontinence;
· Assessment of cerebellar function - the presence of unsteadiness in the Romberg position (standing with arms outstretched in front of him and closed eyes), lack of coordination of movements;
· Assessment of eyeball movements - with Guillain-Barré syndrome, a complete lack of ability to move the eyes is possible;
· Conducting vegetative tests - to assess the damage to the nerves that innervate the heart;
· The reaction of the heart to a sudden getting up from a lying position, physical activity is assessed;
· Assessment of the function of swallowing.

Assessment of the severity of motor deficit in children over 3 years old is carried out using the North American scale:

Stage 0 Guillain Barré syndrome is the norm;

Stage 1 - minimal movement disorders;

Stage II - the ability to walk 5m without support or support;

Stage III - the ability to walk 5m with support or support;

Stage IV - inability to walk 5m with support or support (confined to a bed or wheelchair);

Stage V Guillain-Barré syndrome - the need for mechanical ventilation;

Stage VI - death.

V clinical practice to assess the severity of movement disorders use the scale of muscle strength of the extremities (A. Szobor, 1976).

0 points - there are no movements in the muscle.

1 point - minimal movements in the muscle, but the patient does not hold the weight of the limb.

2 points - the patient maintains the weight of the limb, but the resistance to the investigator is minimal.

3 points - the patient resists efforts to change the position of the limb, but it is insignificant.

4 points - the patient resists well the efforts to change the position of the limb, but there is some decrease in strength.

5 points - muscle strength corresponds to the age and constitutional norm of the subject.

System for diagnosing GBS, the criteria of which are formulated by the National Institute for the Study of Neurological and Communication Disorders and Stroke (USA):

Mandatory criteria:

· Progressive motor weakness in more than one limb;

· The severity of paresis varies from minimal weakness in the legs to tetraplegia;

· Suppression of reflexes of varying degrees.

Subsidiary criteria for the diagnosis of the syndrome:

1.weakness increases within 4 weeks from the onset of the disease;

2. the relative symmetry of the lesion;

3. mild sensory impairment;

4. involvement of the cranial nerves in the pathological process;

5. recovery;

6. symptoms of autonomic dysfunction;

7. the usual absence of a febrile period at the onset of the disease;

8. an increase in the level of protein in the cerebrospinal fluid (CSF) 1 week after the onset of symptoms of the disease, provided that the number of mononuclear leukocytes usually does not exceed 10 cells per 1 mm3;

9. impairment of the conductive function of the nerves during the course of the disease in approximately 80% of cases;

10.the absence of established causes of damage to peripheral nerves, such as the effect of hexacarbon, porphyria, diphtheria, other toxic and infectious diseases imitating GBS.

Diagnostics (hospital)

DIAGNOSTICS AT STATIONARY LEVEL

Diagnostic criteria at the inpatient level: see ambulatory level.

Complaints and anamnesis: see ambulatory level.

Physical examination: see ambulatory level.

* NB! The criteria given in clause 9, subparagraph 1 are typical for GBS, axonal, paraparetic and pharyngo-cervico-brachial forms, and such forms as Miller Fisher syndrome and acute pandisautonomy clinically differ significantly from other forms of GBS, therefore, the generally accepted criteria for the diagnosis of this disease it is difficult to apply for them. The diagnosis in these cases is established primarily on the basis of anamnestic data and the clinical picture of the disease.

Characteristics of Miller Fisher's syndrome.







Stunning, confusion due to hyponatremia associated with overproduction of antidiuretic hormone. Convulsions may occur when plasma sodium levels are less than 120 mmol / L.

Characteristics of acute pancreatic autonomy.
The emergence of neurological symptoms 1-2 weeks after the transferred viral or bacterial infection;
· The presence of an isolated lesion of the autonomic nervous system;
· Often the cardiovascular system is affected (postural hypotension, arterial hypertension, tachycardia, cardiac arrhythmias);
Blurred vision, dry eyes, anhidrosis;
Dysfunction of the gastrointestinal tract (paralyticileus);
· Difficulty urinating, acute urinary retention;
· Increased sweating, bluish color of the skin of the hands and feet, cold extremities;
Stunning, confusion due to hyponatremia associated with overproduction of antidiuretic hormone. Convulsions may occur when plasma sodium levels are less than 120 mmol / L;
· Recovery is gradual and often incomplete.

To make a diagnosis of Guillain-Barré syndrome, it is necessary to clearly find out the history of the development of the disease, in conjunction with an assessment of the neurological status, to compare it with the criteria for diagnosing GBS (WHO; 1993). It is advisable to carry out a lumbar puncture with a study of the cerebrospinal fluid, as well as to confirm the neural level of the lesion and to clarify the form of the disease according to the ENMG examination.

Diagnostic algorithm:
GBS should primarily be differentiated from conditions that can lead to the development of acute peripheral tetraparesis. Differential-diagnostic search is greatly simplified when using a unique algorithm developed by researchers of the Federal State Budgetary Institution "NTSN" RAMS.

Differential-diagnostic algorithm for acute flaccid tetraparesis (OBT)

Note: OBT-acute flaccid tetraparesis; EMG electromyography; PNP polyneuropathy; GBS - Guillain-Barré syndrome; LP - lumbar puncture; BHAK - biochemical blood test; RF - rheumatic factor; CRP - C-reactive protein; CPK - creatinine phosphokinase; MRI - magnetic resonance imaging (at least 1 T); CT - computed tomography.

Laboratory research: see ambulatory level (for those surveys that were listed additionally).

List of basic laboratory tests:
Blood for immunoglobulins - when planning specific therapy with class G immunoglobulins, it is necessary to determine the Ig fractions in the blood, a low concentration of IgA is usually associated with its hereditary deficiency, in such cases there is a high risk of developing anaphylactic shock (therapy with immunoglobulin is contraindicated);
· Studies of cerebrospinal fluid (cytosis, protein concentration). When analyzing cerebrospinal fluid, it is customary to include the following three indicators among the diagnostic criteria confirming GBS:
The presence of a high protein content,
An increase in the fraction of albumin,
· The absence of a concomitant increase in cytosis.
Additionally, the following diagnostic tests can be recommended to confirm the diagnosis and clarify the features of GBS in a particular case:
· A blood test for autoantibodies to gangliosides, with a mandatory study of GM1, GD1a, and GQ1b if the patient has oculomotor disorders;
· Blood test for IgA antibodies to Campylobacter jejuni;
· Study of the content of biomarkers of heavy chains of neurofilament, tau protein and gliofibrillar acidic protein in blood serum.

Instrumental research: see ambulatory level.

In severe cases of the disease (rapid progression, bulbar disorders), daily monitoring of blood pressure, ECG, pulse oximetry and a study of the function of external respiration (spirometry, peak flowmetry), 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.

Differential diagnosis

GBS must be differentiated from other diseases manifested by acute peripheral paresis, primarily from poliomyelitis (especially in children early age) 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.

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Treatment

Preparations (active ingredients) used in the treatment

Treatment (outpatient clinic)

TREATMENT AT THE AMBULATORY LEVEL

Treatment tactics:
Suspicion of Guillain-Barré syndrome, even with minimal severity of symptoms, is the basis for emergency hospitalization, and symptomatic treatment is carried out at the outpatient stage, and when a diagnosis is made, they are sent to a hospital, and the patient and his relatives must be warned of a possible rapid deterioration of the condition.

Non-drugtreatment: no.

Drug treatment:
Symptomatic therapy:
· With an increase in blood pressure, nifedipine, 10-20 mg under the tongue, can be prescribed;
· To reduce tachycardia, propranalol is used, at an initial dose of 20 mg 3 times a day; then the dose is gradually increased to 80-120 mg in 2-3 doses, under the control of blood pressure, heart rate, ECG;
With bradycardia - atropine, for adults: IV bolus under the control of ECG and blood pressure - 0.5-1 mg, if necessary, the administration is repeated after 3-5 minutes; maximum dose 0.04 mg / kg (3 mg). Children - 10 mcg / kg;
to reduce pain, analgesics, non-steroidal anti-inflammatory drugs are administered:
· Ketorolac, orally once at a dose of 10 mg or repeatedly, depending on the severity of the pain syndrome, 10 mg up to 4 times a day. The maximum daily dose should not exceed 40 mg, or no more than 60 mg is administered intramuscularly for 1 administration; usually 30 mg every 6 hours.
· Diclofenac, intramuscularly. A single dose is 75 mg, the maximum daily dose is 150 mg (with a break between injections for at least 30 minutes).
Ibuprofen, 1-2 tablets 3-4 times a day; if necessary, 1 tablet every 4 hours. Do not take more than 4 hours later. The maximum daily dose for adults should not exceed 1200 mg (no more than 6 tablets in 24 hours).

Algorithm for emergency situations: symptomatic therapy measures.

Other treatments: no.

Consultation with an infectious disease specialist - the establishment or exclusion of an infectious ( Infectious mononucleosis, Lyme disease, HIV, etc.);
· Consultation of a therapist - establishment or exclusion of a therapeutic disease (inflammatory disease of internal organs: lungs, kidneys, liver, etc.);
· Consultation of an endocrinologist, nephrologist, rheumatologist - if necessary, to exclude somatic pathology.

Preventive actions:
· There is no specific prevention of the disease, doctors can recommend treating all infectious diseases at the very beginning of their development, this will reduce the negative effect of pathogens on the nervous system.

Patient monitoring:
· Assessment of the general condition of the patient with a description of the condition of the skin; the patient's weight;
Hemodynamic indices: the number of respiratory movements, A / D, heart rate, pulse;
· Assessment of neurological status.

· Etiopathogenetic treatment at this stage is not carried out, and therefore, there are no indicators.

Treatment (ambulance)

DIAGNOSTICS AND TREATMENT AT THE STAGE OF EMERGENCY EMERGENCY

Diagnostic measures:
Often, GBS has an acute course and is potentially life-threatening, because, starting from the legs, the lesion progresses, spreads to the bulbar and other cranial nerves, and therefore the following measures are necessary:

Swallowing assessment- with bulbar paralysis, swallowing disorder, to prevent aspiration
· Nasogastric tube.

Breath assessment- Perhaps the development of progressive respiratory failure, and not only of the obstructive type in connection with bulbar paralysis, but also with damage to the phrenic nerve (a paradoxical type of breathing is characteristic - when inhaling, the anterior abdominal wall sinks) and intercostal.
· Intubation of the trachea (for further transfer of the patient to mechanical ventilation).

Evaluation of the work of the heart:
· ECG - decrease and even inversion segment S-T, an increase in the Q-T interval, cardiac arrest is possible.
During transportation, it is important to take care of maintaining airway patency, carefully monitor blood pressure and heart rate, tachycardia, orthostatic hypotension, arrhythmia, etc.

Drug treatment:
· Syndromic therapy according to the protocol of emergency medical care.

Treatment (hospital)

STATIONARY TREATMENT

Treatment tactics: The main goal of the treatment is: restoration of vital functions, elimination of symptoms of an autoimmune disease using specific techniques, the patient's rehabilitation period, prevention of complications. The first thing to do is to place the patient in a hospital, and if necessary, connect him to a ventilator, install a catheter if urine is not emitted, install a nasogastric tube if swallowing is difficult.

Non-drug treatment:
In severe cases with severe paresis, proper care is of particular importance for the prevention of complications associated with prolonged immobility of the patient (infections, bedsores, pulmonary embolism). It is necessary to periodically (at least once every 2 hours) change the position of the patient, skin care, control over the functions of the bladder and intestines, passive gymnastics, and prevention of aspiration. With persistent bradycardia, the threat of asystole may require the installation of a temporary pacemaker.

Drug treatment:
Specific therapy for Guillain-Barré syndrome, aimed at arresting the autoimmune process, is currently used pulse therapy with class G immunoglobulins and plasmapheresis (see paragraph - other types of treatment). The effectiveness of each of the methods is relatively the same, therefore, their simultaneous use is considered impractical.
Immunoglobulin G class, like plasmapheresis, reduces the duration of stay on mechanical ventilation; it is administered intravenously daily for 5 days at a dose of 0.4 g / kg. Possible side effects: nausea, head and muscle aches, fever.
Symptomatic therapy for Guillain-Barré syndrome is carried out to correct violations of acid-base and water- electrolyte balance, correction of blood pressure, prevention of deep vein thrombosis, thromboembolism.
Infusion therapy for the correction of violations of acid-base, water-electrolyte balances, severe arterial hypotension.
With persistent severe arterial hypertension, antihypertensive drugs (β-blockers or blockers of slow calcium channels) are prescribed (see KP Arterial hypertension).
With severe tachycardia, appoint (β-blockers (propranolol), with bradycardia - atropine (see below).
With the development of intercurrent infections, antibiotic therapy is required (drugs of a wide spectrum of action are used).
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) NSAIDs are recommended; in case of neuropathic pain, the drugs of choice are gabapentin, carbamazepine, pregabalin (only for adults!) (See below).

List of essential medicines:.

Medical rehabilitation

is carried out according to the Standard of the organization of rendering medical rehabilitation population of the Republic of Kazakhstan, approved by order of the Minister of Health of the Republic of Kazakhstan dated December 27, 2013 No. 759.

Palliative care

Depending on the type and severity of complications after the illness, additional treatment may be required, such as:
· Immobilized patients are prescribed heparin subcutaneously at a dose of 5000 IU every 12 hours and temporary compression of the calf muscles to prevent deep vein thrombosis;
· Massage has a beneficial effect on the metabolism, which also accelerates the growth of nerves and reinnervation;
· Kinesiotherapy has been proven to stimulate reinnervation and restore muscle volume;
· Physiotherapy to improve strength, to prevent the formation of contractures (electrical stimulation, heat therapy, drug electrophoresis);
Rehabilitation to develop daily skills and use adaptive products to assist in Everyday life;
The patient may require orthopedic aids or other supportive methods to improve movement;
· Psychotherapy;

Hospitalization

Indications for planned hospitalization: no.

Indications for emergency hospitalization:
· Patients with GBS must be admitted to a hospital in the intensive care unit.

Information

Sources and Literature

1. Minutes of the meetings of the Joint Commission on the Quality of Medical Services of the Ministry of Healthcare of the Republic of Kazakhstan, 2016
   1. 1. Bykova O. V., Boyko A. N., Maslova O. I. Intravenous administration immunoglobulins in neurology (literature review and own observations) // Nevrol. zhurn. - 2000, 5.P. 32-39. 2. Gekht BM, Merkulova DM Practical aspects of the clinic and treatment of polyneuropathies // Nevrol. zhurn.-1997.-№ 2.-С.4-9. 3. Piradov M.A., Suponeva N.A. Guillain-Barré Syndrome: Diagnosis and Treatment. A guide for doctors "-2011. 4. Suponeva N.A., Piradov M.A. "Intravenous immunotherapy in neurology" -2013. 5. Sladky J. T. Guillain-Barre syndrome in children // J. Child Neurol. 2004. V. 19. P. 191-200. 6. Schmidt B., Toyka K. V., Kiefer R. et al. Inflammatory infiltrates in sural nerve biopsies in Guillain-Barre syndrome and chronic inflammatory demyelinating neuropathy // 1996. V. 19. P. 474–487. 7. Khalili-Shirazi A., Hughes R. A., Brostoff S. W. et al. T cell responses to myelin proteins in Guillain-Barre syndrome // J. Neurol. Sci. 1992. V. 111. P. 200-203. 8. Van Rhijn I., Bleumink-Pluym N. M., Van Putten J. P. et al. Campylobacter DNA is present in circulating myelomonocytic cells of healthy persons and in persons with Guillain-Barre syndrome // J. Infect. Dis. 2002. V. 185. P. 262-265. 9. Cooper J. C., Ben-Smith A., Savage C. O. et al. Unusual T cell receptor phenotype V gene usage of gamma delta T cells in a line derived from peripheral nerve of a patient with Guillain-Barre syndrome // J. Neurol. Neurosurg. Psychiatry. 2000. V. 69. P. 522-524. 10. Ilyas A. A., Chen Z. W., Cook S. D. et al. Immunoglobulin G subclass distribution of autoantibodies to gangliosides in patients with Guillain-Barre syndrome // Res. Commun. Pathol. Pharmacol. 2002. V. 109. P. 115-123. 11. Tsang R. S., Valdivieso-Garcia A. Pathogenesis of Guillain-Barre syndrome // Expert Rev. Anti Infect. Ther. 2003. V. 1. P. 597-608. 12. Kieseier B. C., Kiefer R., Gold R. et al. Advances in underswtanding and treatment of immune-mediated disorders of the peripheral nervous system // Muscle Nerve. 2004. V. 30. P. 131-156. 13. Adams D., Gibson J. D., Thomas P. K. et al. HLA antigens in Guillain-Barre syndrome // Lancet. 1977. No. 2. P. 504-505. 14. Koga M., Yuki N., Kashiwase K. et al. Guillain-Barre and Fisher's syndromes subsequent to Campylobacter jejuni enteritis are associated with HLA-54 and Cwl independent of anti-ganglioside antibodies // J. Neuroimmunol. 1998. V. 88. P. 62-66. 15. Magira E. E., Papaijakim M., Nachamkin I. et al. Differential distribution of HLA-DQ beta / DR beta epitopes in the two forms of Guillain-Barre syndrome, acute motor axonal neuropathy and acute inflammatory demyelinating polyneuropatrhy (AIDP); identification of DQ beta epitopes associated with susceptibility to and protection from AIDP // J. Immunol. 2003. V. 170. P. 3074-3080. 16. Geleijns K., Schreuder G. M., Jacobs B. C. et al. HLA class II alleles are not a general susceptibility factor in Guillain-Barre syndrome // Neurology. 2005. V. 64. P. 44-49. 17. Asbury A. K., Cornblath D. R. Assessment of current diagnostic criteria for Guillain-Barre syndrome // Ann. Neurol. 1990. V. 27. S. 21-24.

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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 progressive symmetric flaccid paralysis in the muscles of the limbs and muscles innervated by cranial nerves (with the possible development of dangerous breathing and swallowing disorders) with or without sensory and autonomic disorders (unstable other blood pressure, arrhythmias and ) .

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 clinical course diseases.

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 the variants with purely movement disorders), and other parts of the peripheral nervous system may 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 disease- 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, 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 mild deep sensitivity disorders, 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 lesions of the 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 - paresis of the abduction of the 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 are impaired 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 of 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:
rapid onset with severe sensory impairment and areflexia, rapidly involving the 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
It is characterized by isolated weakness in the facial, oropharyngeal, cervical and upper limb muscles without involvement of the lower limbs.

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
no 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, should be excluded:
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 swallowing disorders that do not bring relief from 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 begins 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 Douet 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 apply bandaging with an elastic bandage of the legs to the middle of the 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 severe pain syndrome, use 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. In general, a 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.