Definition of illness. Causes of the disease. Surgical treatment of the syndrome

Guillain-Barré Syndrome (GBS)- acute inflammatory demyelinating polyradiculoneuropathy of autoimmune etiology. characteristic feature diseases - peripheral paralysis and protein-cell dissociation in the CSF (in most cases). The diagnosis of Guillain-Barré syndrome is established in the presence of increasing weakness and areflexia in more than 1 limb. However, other neurological diseases accompanied by peripheral paresis: poliomyelitis, acute period stem stroke, toxic lesions CNS, etc. Treatment of patients with Guillain-Barré syndrome is carried out in a hospital, since the patient may need mechanical ventilation.

ICD-10

G61.0

General information

Guillain-Barré Syndrome (GBS)- acute demyelinating inflammatory polyneuropathy of autoimmune etiology. A characteristic symptom of the disease is peripheral paralysis and protein-cell dissociation in the cerebrospinal fluid (in most cases). There are currently four main clinical variants within GBS:

• classic form of SGB- acute inflammatory demyelinating polyradiculoneuropathy (up to 90% of cases)
• axonal form of GBS- acute motor axonal neuropathy. A characteristic feature of this form of GBS is an isolated lesion of motor fibers. In acute motor-sensory axonal neuropathy, both motor and sensory fibers are affected (up to 15%)
• Miller-Fischer syndrome- a form of GBS characterized by ophthalmoplegia, cerebellar ataxia and areflexia with mild paresis (up to 3%)

In addition to the above forms of Guillain-Barré syndrome, in recent times there are several more atypical forms of the disease - acute sensory neuropathy, acute pandysautonomy, acute cranial polyneuropathy, which are quite rare.

Clinical picture of Guillain-Barré syndrome

The first manifestations of Guillain-Barré syndrome are, as a rule, muscle weakness and/or sensory disturbances (numbness, paresthesias) in lower limbs, which, after a few hours (days) spread to upper limbs. In some cases, the disease manifests itself with pain in the muscles of the limbs and the lumbosacral region. Very rarely, CN lesions (oculomotor disorders, impaired phonation and swallowing) become the first manifestation. The degree of movement disorders in Guillain-Barré syndrome varies considerably, from minimal muscle weakness to tetraplegia. Paresis, as a rule, is symmetrical and is more pronounced in the lower extremities.

Hypotension and a significant decrease (or complete absence) tendon reflexes. In 30% of cases, respiratory failure develops. Disorders of superficial sensitivity are manifested in the form of mild or moderate hypo- or hyperkinesia of the polyneuritic type. Approximately half of the patients have disorders of deep sensitivity (sometimes up to its complete loss). CN lesions, detected in most patients, are manifested by paresis of facial muscles and bulbar disorders. Of the vegetative disorders, the most common are cardiac arrhythmias, arterial hypertension, sweating disorders, dysfunction of the gastrointestinal tract and pelvic organs (urinary retention).

Guillain-Barré Syndrome Diagnosis

When taking an anamnesis, it is necessary to pay attention to the presence of provoking factors, since in more than 80% of cases the development of GBS is preceded by certain diseases and conditions (past infections of the gastrointestinal tract, upper respiratory tract, vaccination, surgical interventions, intoxication, tumor). Neurological examination is aimed at identifying and assessing the severity of the main symptoms of Guillain-Barré syndrome - sensory, motor and autonomic disorders.

It is necessary to conduct general clinical studies (common urinalysis, complete blood count), biochemical analysis blood ( gas composition blood, serum electrolyte concentration), cerebrospinal fluid examination, serological studies (if an infectious etiology of the disease is suspected), as well as electromyography, the results of which are of fundamental importance for confirming the diagnosis and determining the form of GBS. In severe cases of the disease (rapid progression, bulbar disorders), daily monitoring of blood pressure, ECG, pulse oximetry and a study of function should be carried out. external respiration(spirometry, peak flowmetry).

Confirmation of the diagnosis requires the presence of progressive muscle weakness in more than one limb and the absence of tendon reflexes (areflexia). The presence of persistent pelvic disorders, pronounced persistent asymmetry of paresis, polymorphonuclear leukocytes, as well as a clear level of sensitivity disorders should raise doubts about the diagnosis of Guillain-Barré syndrome. In addition, there are a number of signs that absolutely exclude the diagnosis of GBS, among them: recent diphtheria, symptoms of lead intoxication or evidence of lead intoxication, the presence of exclusively sensory disorders, impaired porphyrin metabolism.

Differential Diagnosis

First of all, the neurologist differentiates the Guillain-Barré syndrome from other diseases, which are also manifested by peripheral paresis (poliomyelitis), as well as other polyneuropathies. Polyneuropathy in acute intermittent porphyria may resemble Guillain-Barré syndrome, but, as a rule, is accompanied by a variety of psychopathological symptoms (hallucinations, delusions) and pronounced abdominal pain. Symptoms similar to the signs of GBS are possible with extensive strokes of the brain stem with the development of tetraparesis, which in the acute period takes on the features of a peripheral one. The main differences between myasthenia gravis and GBS are the variability of symptoms, the absence of sensory disorders, and characteristic changes in tendon reflexes.

Treatment of Guillain-Barré syndrome

All patients diagnosed with Guillain-Barré syndrome are subject to hospitalization in a hospital with an intensive care unit and resuscitation. In approximately 30% of cases of GBS, due to the development of severe respiratory failure, there is a need for mechanical ventilation, the duration of which is determined individually, focusing on the VC, the restoration of swallowing and cough reflex. Disconnection from the ventilator is carried out gradually with the obligatory stage of intermittent forced ventilation.

In severe cases with severe paresis special meaning to prevent complications associated with prolonged immobility of the patient (infections, bedsores, pulmonary embolism), has the right care. It is necessary to periodically (at least once every 2 hours) change the position of the patient, skin care, control over functions Bladder and intestines, passive gymnastics, aspiration prevention. With persistent bradycardia with the threat of asystole, it may be necessary to install a temporary pacemaker.

As a specific therapy for Guillain-Barré syndrome, aimed at stopping the autoimmune process, pulse therapy with immunoglobulins of class G and is currently used. The effectiveness of each of the methods is relatively the same, so their simultaneous use is considered inappropriate. significantly reduces the severity of paresis and the duration of mechanical ventilation. As a rule, 4-6 sessions are carried out with an interval of one day. As replacement media, 0.9% sodium chloride solution or dextran is used.

It should be remembered about contraindications to plasmapheresis (infections, blood clotting disorders, liver failure), as well as possible complications (violation of the electrolyte composition, hemolysis, allergic reactions). Immunoglobulin class G, like plasmapheresis, reduces the length 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, headache and muscle pain, fever.

Symptomatic therapy for Guillain-Barré syndrome is carried out to correct violations of the acid-base and water-electrolyte balance, to correct the level blood pressure, prevention of deep vein thrombosis and thromboembolism. Surgical intervention may be needed for tracheostomy in case of prolonged mechanical ventilation (more than 10 days), as well as gastrostomy in case of severe and prolonged bulbar disorders.

Prognosis for Guillain-Barré syndrome

Most patients diagnosed with Guillain-Barré syndrome experience a complete functional recovery within 6-12 months. Persistent residual symptoms persist in approximately 7-15% of cases. The recurrence rate of GBS is about 4%, and the mortality rate is 5%. Possible reasons death - respiratory failure, pneumonia or other infections, thromboembolism pulmonary artery. The likelihood of death in to a large extent depends on the age of the patient: in children under the age of 15, it does not exceed 0.7%, while in the WTO, as in patients over 65, it reaches 8%.

Prevention of Guillain-Barré syndrome

There are no specific methods for the prevention of Guillain-Barré syndrome. However, the patient should be informed about the ban on vaccinations during the first year from the onset of the disease, since any vaccination can cause a relapse of the disease. Further immunizations are permitted, but must be justified. In addition, the development of Guillain-Barré syndrome within 6 months after any vaccination is in itself a contraindication to the use of this vaccine in the future.

Guillain-Barré syndrome(other name acute polyradiculitis ) - this is acute inflammatory polyradiculoneuropathy, which is manifested by disorders of a vegetative nature, sluggish, impaired sensitivity.

As a result of this disease, a person is in a state of complete or partial immobility. Gradually, in the course of the development of the disease, a person loses the ability to take things by hand, to swallow, lift the eyelids etc. This disease is relatively rare. SGB diagnosed in all countries of the world, manifested in both adults and children. It develops more often in men, but there is no significant difference in the number of cases of the disease in different sexes.

The reasons

This disease was first described in 1916. neuropathologists from France G. Guillain and J. Barre, by whose names the disease was subsequently named. But there is still no exact information about the causes of its occurrence. Basically, the disease develops as a consequence of an acute infection that has previously manifested in the patient. There is an assumption that the disease provokes filterable virus . However, most experts tend to believe that Guillain-Barré syndrome It has allergic nature.

The disease is considered autoimmune process, in which the destruction of the nervous tissue develops. Consequently, the human immune system begins to " fight» with its own body and produces to some nerve sheath molecules. There is damage to the nerves and nerve roots, which are located at the junction of the peripheral and central nervous systems. There is no damage to the head and spinal cord. The disease begins to develop under the influence of viruses. AT this case most often the disease begins with damage to the body, Epstein-Barr virus , as well as bacteria. The immune response to the penetration of a foreign agent develops in the normal state of the body. But in some cases, there are failures in the reaction to one's own and other people's cells. As a result, the immune system fights against the body.

Symptoms

Symptoms Guillain-Barré syndrome initially appear general weakness, as well as an increase in body temperature to subfebrile and pain in arms and legs. The pain can also be excruciating in nature. But still hallmark GBS is clear feeling of muscle weakness in the limbs. In the distal sections, both of the arms and legs, there are paresthesia , in some cases they also occur around the mouth and in the tongue. Weakness of the muscles of the face and other nerves of the face is sometimes noted, as well as autonomic disorders. Movement disorders initially appear in the legs, and later - move to the hands. Sometimes the general symptom complex with this syndrome resembles signs myopathies .

If during development Guillain-Barré syndrome are celebrated lesions of the nerves of the bulbar group , then without timely respiratory resuscitation, a person may die.

Also pronounced vegetative disorders, which are manifested by chilliness and coldness distal departments limbs, phenomenon hyperhidrosis. At severe development diseases sometimes there is a violation of pelvic functions, which is manifested by urinary retention. This symptom usually continues 3 to 5 days.

Acute polyradiculitis, as a rule, develops over two to four weeks, after which the person's condition stabilizes and the condition improves. Diagnosis is not only sharp forms diseases , but also subacute , as well as chronic . In most cases, we are talking about a favorable prognosis, but sometimes the disease develops as an ascending Landry's palsy when extended to arm muscles, torso and bulbar muscles.

Diagnostics

In order for the outcome to be favorable, it is important to recognize the disease on early stages and start treatment in a timely manner. Initially, the specialist necessarily conducts a detailed questioning, during which he determines how the symptoms of the disease increased, what signs were manifested. An important criterion to establish a diagnosis Guillain-Barré syndrome counts in the arms and/or legs , which is progressive, and tendon areflexia .

The doctor necessarily notes the presence of symmetry of the lesion, sensory disturbances, damage to the cranial nerves, autonomic disorders. An important point is also the absence in the first days of the disease. In the diagnostic process, it is important to determine the presence of a typical protein-cell dissociation in the cerebrospinal fluid. During the puncture, it is noted high concentration squirrel.

Guillain polyradiculoneuritis (Guillain-Barre) has some features similar to those of other diseases. It is important to distinguish its manifestations from botulism , myasthenia gravis , paralysis , which occurs as a consequence of taking . It is also important to differentiate the disease from lesions of the spinal cord and brain stem, transverse myelitis , porphyria etc. For this purpose, laboratory research, appointed additional methods examination of the patient. In particular, if you suspect SGB research is underway composition cerebrospinal fluid , electromyography. If the cerebrospinal fluid has high level protein after the first week of illness, or its content rises later, then we can talk about suspicion of Guillain-Barré syndrome. The features of the cellular composition are also studied.

Treatment

Treatment Guillain-Barré disease carried out immediately after diagnosis. effective method therapy is considered plasmapheresis with intravenous administration. Sick SGB partially removed blood plasma, the shaped elements are returned. Also in the process of treatment practiced reception glucocorticoids(in particular, patients are prescribed 1-2 mc / kg per day), antihistamines( , ), reception vitamins(mainly group B). The complex treatment also includes anticholinesterase drugs ( , ).

During therapy, it is important to ensure quality care for the patient , as well as constantly monitor the functions of its cardiovascular and respiratory systems . After all, at severe course disease, respiratory failure can occur very quickly, and in the absence of quick treatment available fatal outcome. With a decrease in lung capacity or with the manifestation bulbar syndromes necessary urgent intubation or tracheotomy, to provide artificial lung ventilation . In the process of caring for a patient, at least, once every two hours, very carefully turn the patient over in bed.

If the patient has symptoms arterial hypertension , as well as , then seizures must be removed with the help of calcium ion antagonists and beta blockers. To avoid a rise in blood pressure, intravenous fluid is practiced to increase intravascular volume.

If he is observed acute delay urinary excretion , then due to reflex disorders, the pulse and blood pressure may fluctuate. With this development of the disease, it is necessary to use an indwelling catheter.

It is equally important to provide high-quality rehabilitation therapy during a period when a person's condition improves. At this time, the patient is shown physiotherapy, ozokerite, massage, paraffin and etc.

If we talk about forecasts, then the recovery normal state health without serious consequences is observed in approximately 80% of people who were diagnosed Guillain-Barré polyneuropathy. However, it should be borne in mind that with a severe course of the disease, recovery will also be longer and more difficult. Patients fully recover and live full life. But still ill throughout life SGB people may feel slight weakness in their arms and legs.

The presence or absence of residual effects also depends on the severity of the disease. Sometimes recovery takes place around 1.5-2 years. But it also happens that recovery does not occur, and then the person is completely dependent on the help of other people. This pattern is seen in approximately 5-8% of GBS cases. According to various sources, mortality due to the disease can range from 1 to 18%. Most people die as a result of bronchopneumonia and respiratory distress syndrome .

The doctors

Medications

List of sources

• Piradov M.A., Suponeva N.A. Guillain-Barré Syndrome: Diagnosis and Treatment. A Guide for Physicians" - 2011;
• Zhulev N.M., Osetrov B.A., Zhulev S.N., Lalayan T.V. Neuropathies: A Guide for Physicians. - St. Petersburg: SPbMAPO Publishing House, 2005;
• Diseases of the nervous system in children: in 2 volumes. T. 2 / ed. J. Aicardi and others; per. from English; total ed. A. A. Skoromets. - M.: Panfilov Publishing House; BINOMIAL. Knowledge Lab 2013;
• Levin, O.S. Polyneuropathies: a clinical guide / O.S. Levin. - M.: MIA, 2011;
• Ponomarev V.V. Rare neurological syndromes and illness. - St. Petersburg, 2005.

Piradov M.A. 2000

Rehabilitation is possible
This disease has at least eight different names - Landry's syndrome (after the French neurologist who first described it in 1859), Guillain-Barré-Stroll syndrome (scientists who have made a significant contribution to the study of the disease), acute polyradiculoneuritis, etc. Today on International classification diseases, it is officially called Guillain-Barré syndrome (GBS) or acute post-infectious polyneuropathy. In neurology, GBS is considered a unique disease. And not so much because of its relative rarity (occurs in 2 people per 100,000 of the population), but because of the possibility of complete rehabilitation of the patient, although sometimes GBS can be compared with the most serious diseases in terms of the severity of the lesion. The Deputy Director for Science of the Research Institute of Neurology of the Russian Academy of Medical Sciences, Head of the Department of Neuroreanimation, Professor Mikhail PIRADOV tells more. Guillain-Barré syndrome is the most common cause acute peripheral tetraparesis and paralysis. Neurological symptoms develop very quickly, while not only motor, but also sensory functions (primarily joint-muscular sensitivity) are violated, and sometimes very roughly, and tendon reflexes decrease until complete extinction. Pelvic disorders are not characteristic of GBS, but in a third of cases, the respiratory and swallowing muscles are seriously affected. In severe cases, a person appears before the doctor, lying motionless in bed, who cannot breathe at all, swallow, and even open his eyes. But if an electroencephalogram is taken from a patient, it will be the same as that of a healthy person, and as a person he is not intellectually changed in the slightest. In 70 per cent. cases of GBS occur a few days after the onset of flu-like symptoms: moderate fever, muscle pain, runny nose - all that is usually called acute respiratory infections. Approximately 15 per cent. cases, the syndrome appears after profuse diarrhea, in 5 percent. - after surgical manipulations, whether it be abortions, herniotomy, appendectomy or more complex operations. Sometimes the disease develops after various kinds vaccinations. GBS occurs in any part of the world, at any time of the year, equally common in both sexes. Average age in most observations is about 40 years. At the same time, two small age peaks are distinguished: at 20-25 years old and over 60 years old. In classic cases, the diagnosis of GBS is simple and includes two mandatory features: progressive muscle weakness in at least two limbs and significant reduction up to the complete loss of tendon reflexes. Additional criteria diagnostics are a decrease in the speed of nerve impulse conduction through the muscles with the formation of a conduction block and protein-cell dissociation in the cerebrospinal fluid. Guillain-Barré syndrome is based on autoimmune mechanisms, where the role of the triggering factor is assigned to certain viruses and bacteria. However, a final opinion on the nature of the antigen or antigens causing development cascade immune responses are still missing. In the last five years, it has been established that a whole range of polyneuropathies is combined under the name GBS: acute inflammatory demyelinating polyneuropathy (occurs in 75-80 percent of cases); acute motor neuropathy and, as its variant, acute motor-sensory axonal neuropathy (15-20 percent); Fisher's syndrome (3 percent). Majority autoimmune diseases irreversible. But with GBS, the picture is completely different, unique: the disease is self-limiting. If a seriously ill patient is given only artificial lung ventilation for several months, the affected nerves are restored. And almost as complete as when applying the main modern methods treatment of GBS - plasmapheresis or intravenous therapy with class G immunoglobulins. The question may arise: why treat a patient with expensive methods? But imagine what it means to be on a ventilator for 3-6 months and be bedridden? The timely use of plasmapheresis and class G immunoglobulins can reduce the time spent on mechanical ventilation to several weeks and even days, fundamentally change the course and outcome of the disease. It is no secret that today in the country many patients with severe forms of GBS die. This is largely due to the fact that many hospitals are not equipped with high-quality respiratory equipment or do not have qualified personnel for long-term artificial lung ventilation. Patients die due to banal infections and bedsores. In addition, far from everywhere there is the possibility of performing plasmapheresis operations with the replacement of large volumes of plasma (up to 200 ml of plasma/kg for a course of treatment consisting of 4-5 operations). It is absolutely unacceptable to treat such patients in a rural or small district hospital- they need to be hospitalized in larger hospitals equipped with necessary funds and hardware. A typical mistake in many cases remains the treatment of patients with GBS. hormonal drugs: special studies of more than one thousand patients have shown that hormones do not affect the rate of recovery of impaired functions, but, on the contrary, they carry many complications. However, hormones continue to be unreasonably used even in a number of clinics in the largest Russian cities. Abroad, for this, they can simply be deprived of a medical license. If we talk about the financial side of the matter, of course, today, for most patients, treatment with imported class G immunoglobulins, which are widely used in the West, is simply not affordable, but, fortunately, a course of program plasmapheresis in our country is much cheaper. BUT therapeutic effect of these two methods of treatment is the same: approximately 85-90 percent. of cases, a person with Guillain-Barré syndrome, despite the most severe damage to the peripheral nervous system, recovers completely, and only 10-15 percent. patients experience residual effects. Of course, the prevalence of Guillain-Barré syndrome is incomparable with stroke, traumatic brain injury or epilepsy. But with a stroke best case recovering 20 percent. people, and timely treatment of the Guillain-Barré syndrome with no less severity of the lesion gives a much greater effect. And if every year about 200 people suffer from the SSS in Moscow alone, it is a lot to fully restore the health of 180 people. In my practice, there was a case when the disease struck an 18-year-old guy, a candidate for master of sports in athletics: he could not breathe, swallow, move on his own. A year later, this man fulfilled the standard of a master of sports. And there are many such examples - after proper treatment of GBS, young women give birth to children, the vast majority of patients return to a full life.

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

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

Often the disease develops immediately after transferred infections. AT classic version syndrome, there is an ascending (from the legs) tetraparesis (paresis (paralysis) of all four limbs).

Diagnosis is made on the basis of characteristic clinical picture and is confirmed by the study of cerebrospinal fluid and electromyographic study (EMG).

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

Along with the fact that Guillain-Barré syndrome is classically presented as a demyelinating polyneuropathy with ascending weakness, called acute inflammatory demyelinating polyneuropathy and accounting for 75–80% of cases, several atypical variants or subtypes of this syndrome have been described and identified in the literature, representing a heterogeneous group of immunodependent peripheral neuropathies: Miller-Fisher syndrome (3 - 5%), acute motor axonal polyneuropathy and acute sensorimotor axonal polyneuropathy (make up 15-20%), and more rarely acute sensory polyneuropathy, acute pandysautonomy, acute cranial polyneuropathy, pharyngo-cervico-brachial option. As a rule, these variants are clinically usually more difficult than the main one.

• Epidemiology

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

• ICD-10 code G.61.0

Treatment

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

• Specific Therapy

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

Given the high likelihood of spontaneous recovery, treatment of patients with easy flow Guillain-Barré syndrome can be limited to non-specific and supportive therapy. At moderate process, and especially in severe cases, specific therapy begins as early as possible.

Treatment with immunoglobulin has some advantage over plasmapheresis, since it is easier and more convenient to use, has a significantly smaller number of side effects, is easier 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 more severe (with paralysis, respiratory and swallowing disorders) patients with maximum the effectiveness of the drug at the beginning of therapy within 2 to 4 weeks from the onset of the disease. It is administered intravenously at a dose of 0.4 g / kg / day for 5 days (total course dose of 2 g / kg or about 140 g). An alternative scheme for the administration of the same course dose: 1 g / kg / day in two administrations for two days. Its use is limited by its high cost.

• Plasmapheresis Plasmapheresis administered in the phase of disease progression (approximately in the first two weeks) almost doubles the recovery process and reduces the residual defect. It is prescribed in moderate and severe cases according to the scheme of 4-6 sessions every other day, with an exchange of 50 ml / kg per session (at least 35-40 ml of plasma per kg of body weight), 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 in the recovery phase, plasmapheresis is not indicated. Plasmapheresis showed a rather high efficiency when administered to seriously ill patients, when therapy was started more than 30 days after the onset of the disease.

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

• Nonspecific therapy and rehabilitation
  • It is necessary to prevent deep vein thrombosis of the lower leg in bedridden patients (especially with paralysis in the legs). Oral anticoagulants are used indirect action phenylin or warfarin in doses that stabilize the INR at the level of 2.0, or fraxiparine (nadroparin) 0.3 ml. s / c 1 - 2 times / day, or sulodexide (Wessel Due F) 2 times a day, 1 ampoule (600 LSU) / m for 5 days, then orally 1 caps (250 LSU) 2 times a day . Prevention is carried out before the time when the patient begins to get out of bed. If thrombosis has developed before the start of therapy, prophylaxis is carried out according to the same scheme. Bandaging is also used elastic bandage legs to the middle of the thigh (or use stockings with graduated compression) and raising the legs by 10-15º. Shown passive and, if possible, active "walking in bed" with bending the legs, emitting walking for 5 minutes 3-5 times a day.
  • With paresis of the facial muscles, measures are taken to protect the cornea: instillation eye drops, blindfold for the night
  • Prevention of contractures and paralysis. To do this, passive exercises are carried out 1-2 times a day, they ensure the correct position in bed (comfortable bed, foot supports), massage the limbs. Subsequently, active physiotherapy exercises are connected.
  • Prevention of bedsores - change position in bed every 2 hours, wipe the skin special formulations, use anti-decubitus mattresses.
  • Prevention of lung infection in the form breathing exercises, the earliest possible mobilization of the patient. With a decrease in the vital capacity of the lungs, difficulty in separating bronchial secretions, massage is shown (effort and vibration while turning the body in the supine position) every 2 hours during the day.
  • Symptomatic therapy: antiarrhythmic, hypotensive, analgesic. With arterial hypotension, a drop in blood pressure (approximately blood pressure 100 - 110/60 - 70 mm Hg and below), colloid or crystalloid solutions are administered intravenously (isotonic chloride solution sodium, albumin, polyglucin), and if the effect is insufficient in combination with corticosteroids: prednisolone 120 - 150 mg., Dexazone 8 - 12 mg. In case of insufficiency of these funds, vasopressors are used: dopamine (50 - 200 mg. Diluted in 250 ml. isotonic solution sodium chloride and administered at a rate of 6 to 12 drops / min), or norepinephrine, or mezaton. For moderate pain, simple analgesics and non-steroidal anti-inflammatory drugs are used. With pronounced pain syndrome apply tramal or cabamazepine (tigretol) or gabapentin (Neurontin), possibly in combination with tricyclic antidepressants (imipramine, amitriptyline, azafen, etc.).
  • Classes with a speech therapist for the treatment and prevention of speech and swallowing disorders.
  • Rehabilitation includes massage, therapeutic exercises, physiotherapy. Transcutaneous muscle stimulation is performed with muscle pain and paresis of the limbs.

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

Introduction to Guillain-Barré Syndrome

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

After a short period of malaise with symptoms of SARS, numbness in the arms and legs, a feeling of crawling (paresthesia) may appear. After 1-2 days, weakness in the arms and legs joins; a person gradually becomes completely immobilized, loses the ability to self-service. Often there is perspiration, hoarseness, impaired eye movements. At the same time, patients are fully conscious, they hear and see everything, appearance such patients received the name "talking head". Gradually decreasing contractility intercostal muscles and diaphragm, the volume decreases respiratory movements and reduced lung capacity (VC). In this regard, the blood in the lungs is not sufficiently well enriched with oxygen, there is oxygen starvation due to respiratory failure, death can occur. Patients are treated with intensive care unit, as due to respiratory failure, it may always be necessary to carry out artificial ventilation of the lungs.

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

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

Symptoms of Guillain-Barré Syndrome

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

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

Clinical subtypes of Guillain-Barré syndrome.

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

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

Causes and risks of Guillain-Barré syndrome

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

Diagnosis of Guillain-Barré Syndrome

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

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

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

Treatment of Guillain-Barré syndrome

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

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

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

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

Rehabilitation and prognosis of Guillain-Barré syndrome

Most patients have prospects for a good recovery.

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