Guillain-Barré syndrome symptoms and treatment. What is Guillain-Barré syndrome disease symptoms

Guillain-Barré syndrome is an acute form of progressive inflammatory polyneuropathy characterized by muscle weakness and polyneuritic sensation disorder. The disease is also called idiopathic polyneuritis in acute form, Landry's palsy or inflammatory demyelinating polyradiculoneuropathy. The disease is a representative of autoimmune abnormalities. Usually, the pathology has specific signs that make it possible to recognize it at the earliest stages of development and start adequate treatment on time. It has been proven that more than 80% of patients have a favorable prognosis and are completely cured.

autoimmune demyelination of nerves in Guillain-Barré syndrome

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

The causes of the disease

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

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

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

• Traumatic brain injury. A severe blow to the head, any damage to it, as well as swelling, swelling or hemorrhage in the brain can become the main factor in the development of the syndrome. That is why, when a patient turns to a specialist, first of all, the doctor must find out about the presence of any craniocerebral injuries.
• Infections. Recent viral infections greatly weaken a person's immunity, thereby increasing the likelihood of GBS. The body's defense mechanism perceives neurons as an infection and continues to kill them with the help of leukocytes. In this case, the syndrome appears one to three weeks after infectious disease.
• Allergies. The disease often develops in allergy sufferers, for example, after chemotherapy, vaccination against polio and diphtheria, or major surgical interventions.
• Genetic predisposition. Most diseases are inherited, and Guillain-Barré syndrome is no exception. If someone in the family has already suffered a pathology, then, most likely, it will arise in the descendants. In this case, you need to especially monitor your health: take care of your head and try not to start infectious diseases.

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

1. Gestosis;
2. Frequent medication during pregnancy
3. The presence of autoimmune diseases in the mother;
4. Long-term infections during the period of bearing a child;
5. Use of drugs, alcohol, or smoking.

The acquired reasons include:

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

Symptoms

The disease can manifest itself in 3 forms:

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

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

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

Besides common features also distinguish more distinct:

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

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

Why is the course of the syndrome dangerous?

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

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

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

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

1. Decreased immunity;
2. Respiratory failure;
3. Sedentary joints;
4. Peripheral paralysis;
5. Adaptation problems in society;
6. Difficulty in life;
7. Disability;
8. Fatal outcome.

Diagnosis of the disease

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

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

You should also conduct the following studies:

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

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

Differential diagnosis

Despite the fact that GBS symptoms are similar to those of many other diseases (diphtheria, porphyria, transverse myelitis, botulism and myasthenia gravis), they still need to be distinguished for further correct treatment. In differential diagnosis, the following factors should be considered:

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

Treatment

Patients diagnosed with GBS must be admitted to a hospital. In about 30% of cases, mechanical ventilation is necessary. Pathology therapy is performed at the following levels:

1. Resuscitation;
2. Symptomatic;
3. Blood-purifying;
4. Drug;
5. Muscular recovery;
6. Preventive.

Resuscitation therapy

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

• The patient is connected to the artificial respiration system;
• Use a catheter to drain urine;
• A tracheal tube and probe are inserted if there are problems with swallowing.

Symptomatic therapy

This type of treatment is carried out using various medicines:

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

Plasmapheresis

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

Surgical treatment of the syndrome

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

Non-drug therapy with folk remedies

Cure GBS folk remedies impossible. But it is quite possible to cope with some of its symptoms:

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

Rehabilitation

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

Traditional treatments can be used to help normalize healthy muscle function:

1. Electrophoresis;
2. Trituration;
3. Radon baths;
4. Massage;
5. Baths for body relaxation and muscle tone;
6. Masks and compresses with paraffin wax or beeswax;
7. Wellness gymnastics.

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

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

Prevention of the syndrome

There is no specific prevention of pathology. Doctors can only advise you to refuse any vaccinations within a year so that the disease does not come back again. After this time, vaccination is permitted, but only if it is actually necessary.

It is also worth refusing to take alcoholic beverages, avoiding overheating, hypothermia and reducing physical activity. In this case, a relapse of the disease is excluded.

Forecast

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

Video: Lecture on Guillain-Barré Syndrome

Video: Guillain-Barré Syndrome in the Living Healthy Program

Almost everyone has a cold from time to time or even gets a more serious viral infection, gets vaccinated. But after some time after recovery, it begins to seem that the symptoms are returning - the patient feels a breakdown, aching joints, an increase in temperature. The danger is that these can be signs of a serious illness - Guillain-Barré syndrome, sometimes leading to complete paralysis and death. What is this disease and how to protect yourself from it?

General information

Guillain-Barré syndrome is an autoimmune lesion of the peripheral nervous system, in which muscle weakness can rapidly develop, which turns into paralysis. It often becomes the cause of acute flaccid tetraparesis, in which the motor activity of the lower and upper extremities decreases. In ICD-10, Guillain-Barré syndrome is designated by the code G61.0 and is included in the group of inflammatory polyneuropathies.

Guillain-Barré syndrome is an autoimmune lesion of the peripheral nervous system.

There are two types of GBS classification - according to the form of the disease and according to its severity. According to the first indicator, the following types of syndrome are distinguished:

• AIDP, also called acute inflammatory demyelinating polyneuropathy. This is the most common form - it affects from 65 to 90% of cases;
• acute axogonal neuropathies of a motor or motor-sensory nature affect from 5 to 20% of patients. Designated in medical practice OMAN and OMSAN, respectively;
• 2-3% get sick with Miller-Fisher syndrome, about the same number of people have a paragenetic form of GBS;
• less than 1% falls on such types as sensory, pharyngo-cervico-brachial and paraparetic.

According to the severity, the following categories can be distinguished:

• Easy, in which the patient does not experience difficulties in self-care. Muscle weakness is almost not expressed, the person walks by himself.
• Medium - the patient cannot walk 5 meters without additional help, his motor functions are impaired, fatigue quickly sets in.
• Severe - the patient is no longer able to move, often cannot take food on his own and requires constant care.
• Extremely difficult when a person needs supporting life support.

The course of Guillain-Barré syndrome is divided into several stages of development:

• in the first, lasting 1-4 weeks, the symptoms increase until the acute period occurs;
• on the second, the disease proceeds smoothly, the patient in this state spends up to 4 weeks;
• the recovery period is the longest, it can last for several years. A person at this time comes back to normal and may even be completely cured.

Guillain-Barré Syndrome Can Be Cured

Causes of Guillain-Barré Syndrome

There is still no reliable information about why this ailment appears. In modern medicine, it is believed that the consequences of past infections, including respiratory, cytomegalovirus, herpetic, lead to the disease, and a person can also get sick due to previous mononucleosis and enteritis. Doctors explain this by the fact that immune cells confuse tissues infected with viruses with nerve endings and seeks to destroy them.

Less often, the appearance of the syndrome is attributed to trauma (especially craniocerebral), complications after surgery, the influence of systemic lupus, malignant tumors or HIV.

Another risk group is made up of people with a hereditary predisposition. If your family has had the syndrome, then it is better to take care of yourself - avoid infections and injuries.

Other reasons are possible, but it is much more important not to determine where the disease came from, but to notice its first manifestations and start treatment on time.

Guillain-Barré syndrome symptoms

It is not easy to identify the first manifestations of GBS, at first they are quite similar to the signs of acute infectious diseases. Specific indicators appear only at later stages. Usually to initial symptoms Guillain-Barré syndrome includes the following:

• the temperature rises sharply to very high, sometimes subfebrile;
• goosebumps and tingling sensations are felt on the tips of the clubs;

One of the symptoms of the disease is a sharp rise in temperature.

• the patient feels pain in the muscles;
• over time, weakness appears, the patient loses strength.

As soon as you see these symptoms either in yourself or in someone close to you, see your doctor immediately. Every minute wasted increases the risk of developing paralysis and even death.

Complications associated with Guillain-Barré syndrome

The disease has a huge impact on the functioning of the human body. Its course may be associated with such disabilities:

• difficulty breathing and the lack of oxygen caused by them;
• pain and numbness in different parts of the body;
• disorders in the intestines and genitourinary system caused by weakening of smooth muscles;
• the formation of a large number of blood clots;
• heart problems and fluctuations in blood pressure;
• bed sores appear in bedridden patients.

For each of the complications should be applied symptomatic treatment, aimed at improving the patient's condition and bringing the body back to normal.

Blood pressure fluctuations may appear

Manifestations of Guillain-Barré syndrome in children

Adults are more likely to develop GBS, especially after forty years. In children, it is much less common, but is characterized by the same symptoms, supplemented by eye paralysis, the absence of some reflexes and incoherent muscle work. Guillain-Barré syndrome in children often does not cause fever, which complicates the diagnosis and leads to complications.

Diagnostics of the Guillain-Barré syndrome

First of all, the doctor collects a complete anamnesis of the disease, while paying attention to both the presence or absence of causes of the disease, and the identified symptoms - the speed of their manifestation, the presence of pain and weakness, impaired sensitivity.

The next stage - a physical examination - should give answers to questions about the clarity of the patient's consciousness, the absence or decrease of reflexes, the presence of pain, autonomic problems. The lesions should be symmetrical and get worse over time.

At the third stage, laboratory research... The patient donates blood for biochemical analysis, as well as for the presence of autoantibodies and antibodies to past diseases. A lumbar puncture is often done for general analysis of cerebrospinal fluid.

The patient needs to donate blood for biochemical analysis

Clinical guidelines when determining Guillain-Barré syndrome, it is assumed that instrumental diagnostics... The patient may be prescribed electromyography, which shows the speed of signal movement along the nerves, and neurophysiological examination. It tests the work of the long nerves (sensory and motor) in the arms and legs. The survey is subject to at least four of both. The results of the two methods are compared, and a decision is made on the diagnosis.

Treatment of Guillain-Barré syndrome

There are two different systems therapy that perfectly complement each other - symptomatic and specific. The first is to remove the consequences of the disease for the body - to help digestion, care for the body and eyes, support breathing, and control the work of the heart. Such care should protect the patient from further deterioration and complications.

Specific therapy should help the patient return to normal. There are several methods:

1. Treatment of Guillain-Barré syndrome with the introduction of immunoglobulin into a vein. This drug is especially important for those patients who cannot walk.
2. Plasmapheresis can speed up recovery in moderate and severe disease. For an easy form, it is not relevant. Removal of large amounts of plasma helps to normalize immune functions.

An important caveat - in no case should you combine both types of therapy for simultaneous use, as this can give unpredictable and dangerous results.

Plasmapheresis in the treatment of Guillain-Barré syndrome

Recovery from GBS

Guillain-Barré Syndrome damages both nerve endings and other tissues in the body. The patient often needs comprehensive rehabilitation, which must resume physical activity and the necessary skills in everyday life. For this, massage, electrophoresis, relaxing baths, a contrast shower to raise muscle tone, physiotherapy, medical gymnastics and much more are usually used. All this will give the patient the opportunity to return to a full life and no longer remember about the syndrome.

Prevention of recurrence of the syndrome

There are no special techniques that can protect against GBS recurrence. But by following simple recommendations, you can at least make the risk of the disease less:

• refuse vaccination for at least six months;
• do not visit countries that have reported outbreaks of Zika virus or other dangerous infections;
• regularly visit neurologists and rehabilitation specialists at the clinic;
• temporary disability can be issued to reduce possible workloads.

Forecast for the future

Mortality in GBS is quite low - only up to 5%. It is caused by the most severe manifestations of the syndrome - weakening of breathing, immobilization and related complications - pneumonia, pulmonary embolism and sepsis. The older the patient, the higher the chance of death.

Most people - about 85% - will fully recover and return to a fulfilling life. Moreover, only some of them will have the disease again, the rest will leave it in the past forever.

WHO about the syndrome

The World Health Organization is taking a number of measures to reduce the incidence and increase the number of people cured. It improves surveillance of epidemics viral infectionsZika, in particular, makes recommendations for therapy, supports GBS research programs around the world.

Guillain-Barré syndrome is a serious illness, but even if you have been diagnosed with it, do not despair. Timely diagnosis and comprehensive treatment will quickly put you on your feet, literally and figuratively. Enjoy life, be healthy and take care of yourself.

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

Epidemiology... According to the world's epidemiological studies, GBS occurs in 1 - 2 cases per 100,000 population per year, regardless of gender and age. The incidence of GBS in certain cities and regions of the Russian Federation corresponds to global data and varies from 0.34 to 1.9 per 100,000, on average 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 - 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 the infectious agent with the individual structural elements of the peripheral nerves (sheath, axon) causes the production of specific autoantibodies and the formation of circulating immune complexes that attack the peripheral nerves by the type of "molecular mimicry".

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

Classification... 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 common (5-10%) place is occupied by axonal forms - acute motor and motor-sensory axonal neuropathies (OMAN and OMSAN), characterized by primary damage to the axons of peripheral nerves and differing from each other by involvement (OMSAN) or intactness (OMAN) sensitive fibers. Other forms of GBS (Miller Fisher syndrome, pharyngo-cervic-brachial, acute pandizautonomy, paraparetic, sensory, Bickerstaff brainstem encephalitis [SEB]) are diagnosed extremely rarely (1 - 3%).

reference Information... Bickerstaff stem-like 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 the 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. 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, you should always remember about their existence.

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

GBS is also classified according to the severity of the condition depending on clinical manifestations: [1 ] light form characterized by the absence or minimal paresis, which do not cause significant difficulties in walking and self-care; [ 2 ] at moderate 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 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 growing weakness in the legs, difficulty walking. As the disease progresses, hands, often 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 artificial 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. Enough frequent symptom GBS is pain. There are forms of the disease in which there is an isolated motor deficit. Pelvic dysfunctions are not typical for GBS and can be observed in bedridden patients, mainly in the form of urinary retention.

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 based on 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 4 weeks or less; [ 3 ] violation of sensitivity of the polyneuritic type; involvement of cranial nerves (most often - lesion 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 ] increased 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; therefore, the generally accepted diagnostic criteria for this disease are difficult to apply to them. The diagnosis in these cases is established primarily on the basis of anamnestic data and clinical picture diseases.

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 is usually 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 ] occurrence 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 ] is often amazed the cardiovascular system (postural hypotension, arterial hypertension, tachycardia, heart rhythm disturbances); [ 4 ] blurred vision, dry eyes, anhidrosis; [ 5 ] dysfunction of the gastrointestinal tract (paralytic ileus); [ 6 ] Difficulty urinating, acute urinary retention; [ 7 ] increased sweating, bluish color 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) is the only instrumental diagnostic method that 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 main ENMG parameters are assessed: motor responses (distal latency, amplitude, shape and duration), the presence of blocks of excitation conduction and response dispersion is assessed; the speed of propagation of excitation along motor fibers in the distal and proximal areas is analyzed; sensory responses (amplitude) and the rate of conduction of excitation along sensory fibers in the distal regions; 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 unexcited 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 normal), and at least in two nerves the amplitude of the M-response at the distal point is more than 80% below the lower limit of the norm;

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

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

Thus, 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 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 in certain cases it is differential diagnosis with GBS. However, 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 blood test; 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 terminate the effect of autoimmune mechanisms leading to the development of polyneuropathy, which will allow to suspend 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 ] a repeated increase in 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 software 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: operations of plasmapheresis for GBS should be carried out on continuous separators. A prerequisite for 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 separator centrifuge 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 region 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, antihistamine and a tranquilizer (midazolam). With unstable hemodynamics, medical correction (dopamine, dobutamine) can be used, which is carried out in parallel with rehydration and hemodilution. Hemodilution is carried out in cases of hypovolemia with blood contraction (hematocrit is more than 45%, hemoglobin is more than 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 contraction 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 of hepatitis, HIV, cytomegalovirus, etc. viruses through donor plasma, allergic reactions to injected solutions and drugs, hemorrhagic syndrome, fluid imbalance, activation of coagulation, complement system, fibrinolytic cascade and platelet aggregation). Prevention of plasmapheresis complications 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 starting 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. Preferably 5% or 10% ready-to-use solutions.

[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 of IgA during immunological examination, the presence of anaphylactic reaction to the previous administration of human immunoglobulin preparations.

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

[5 ] Method: if the drug was stored in the refrigerator, it must be warmed up to room temperature before administration in order 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 infusion pump is used to ensure the required infusion rate.

[6 ] Vascular access: as long as the peripheral access is intact, there is no need for a central venous catheter.

[7 ] Complications: adverse reactions do not occur more often than in 10% of cases. Among them headache, muscle pain, chest discomfort, 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 IV infusion. Serious complications include: increased risk of thromboembolism (prevented by the low rate of drug administration and the appointment of prophylactic doses of direct anticoagulants); urticaria, petechiae, migraine. Hemolysis and renal tubular necrosis are extremely rare.

Non-specific therapies for GBS... Non-specific treatments for GBS include the following: [ 1 ] qualified care for immobilized patients and patients on mechanical ventilation (prevention of pressure sores, 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 ] monitoring 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 biological feedback (Armeo, Pablo, Amadeo, RT-300 and others)

Unacceptable: [1 ] 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 plasmapheresis impossible; and long-term oral administration of corticosteroids in patients with GBS contributes to the maintenance 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 the therapy of GBS GIV 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: plasma removal 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.

Unfavorable prognostic factors are also a high rate of increase in neurological disorders (immobility of the patient in the first week of the disease), age over 60 years, the presence of previous diarrhea, registration of low amplitudes of motor responses (less than 10% of the lower limit of normal) during ENMG examination, 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 have undergone, 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, diagnosis, prognosis" I.V. Damulin, Department of Nervous Diseases, First Moscow State Medical University. 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; Federal State Budgetary Institution Scientific Center of Neurology, Russian Academy of Medical Sciences, 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, candidate of medical sciences, 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 practice guidelines for the diagnosis and treatment of Guillain-Barré syndrome (All-Russian Society of Neurologists, 2014) [download]

© Laesus De Liro

The disease most often has an ascending nature: the muscles of the lower extremities are the first to suffer, then paralysis of the facial muscles and respiratory tract may occur. Guillain-Barré syndrome is diagnosed in 4-40 people per 1,000,000 population per year. Most of all, people from 30 to 50 years old are affected by it.

Causes

The exact causes of Guillain-Barré syndrome have not been established by medical science. The pathogenesis is associated with autoimmune disorders. Normally, the immune system should fight only against foreign agents, but in some situations it begins to destroy healthy body tissues.

In Guillain-Barré syndrome, Schwann cells and myelin, the elements that form the sheath of nerve fibers, are affected. The result is edema, lymphocytic infiltration and partial demyelination (exposure) of the peripheral nerves. The spinal cord and brain are not affected, but the transmission of nerve impulses is significantly disrupted, which leads to muscle weakening and paralysis.

Factors that can trigger an autoimmune reaction include:

• traumatic brain injury, brain tumors;
• viral and bacterial infections caused by Campylobacter jejuni (the most common prerequisite), cytomegalovirus, Epstein-Barr virus, Haemophilus influenzae, mycoplasma, HIV, and so on;
• heredity;
• vaccination;
• allergy;
• surgery;
• chemotherapy.

Classification

There are several forms of Guillain-Barré syndrome, depending on the symptoms and the area of \u200b\u200bthe lesion:

• classical (80% of cases) - inflammatory demyelinating polyradiculoneuropathy;
• axonal (15%) - motor or motor-sensory neuropathy, which is accompanied by damage to the axons of nerve fibers responsible for the movement and sensitivity of muscles;
• miller-Fisher syndrome (3%) - a combination of ophthalmoplegia, areflexia with mild paresis and;
• atypical (rare) - sensory neuropathy, pandizautonomy and cranial polyneuropathy.

Depending on the duration of the increase in the symptoms of the disease:

• acute - 7-14 days;
• subacute - 15-28 days;
• chronic - observed for a long period (up to several months), characterized by a slow development of symptoms and a change in periods of remission and deterioration.

Chronic Guillain-Barré disease is considered one of the most dangerous, because it is difficult to diagnose, which significantly reduces the chances of starting timely treatment.

Symptoms

The main symptoms of Guillain-Barré syndrome:

1. Signs of a respiratory or intestinal infection - fever, joint aches, inflammation in respiratory tract and / or nausea, vomiting, diarrhea.
2. Muscle weakness (paresis) and impairment sensory perception (paresthesia) in the proximal and distal extremities.
3. Pain that is localized in the limbs and lumbosacral region.
4. Autonomic disorders - cardiac arrhythmia, hypo- or hypertension, increased sweating.
5. Incontinence / retention of urine due to loss of bladder control as a result of muscle weakness.

As a rule, muscle flaccidity is felt first of all in the legs and feet, then in the hands. It becomes difficult for a person to write, put on socks, hold a spoon. He feels tingling and numbness. Signs of Guillain-Barré syndrome develop symmetrically. In addition, there is a decrease or complete absence of tendon reflexes. Less commonly, there is weakness in the muscles of the face, while the patient has difficulties with sound pronunciation and swallowing.

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.

The severity of violations varies greatly. With a mild course, only minor paresis are observed. In severe cases, Guillain-Barré syndrome is accompanied by paralysis of the muscles of the respiratory and bulbar groups, as well as the eye muscles, diaphragm, abdomen, and back. Without medical care, in such a situation, there is a high risk of death due to respiratory failure. Other life-threatening complications of the disease include:

• pneumonia;
• thromboembolism;
• heart failure;
• sepsis.

Diagnostics

Diagnosis of Guillain-Barré syndrome involves the following activities:

1. Taking anamnesis - not only the current symptoms are clarified in the patient, but also the facts that preceded their occurrence, for example, SARS, gastrointestinal upset, vaccination, and so on.
2. Neurological examination, the purpose of which is to identify sensory and movement disorders. It includes palpation, assessment of coordination and tendon reflexes, electromyography (recording of muscle electrical activity).
3. Laboratory research - clinical urinalysis, general and biochemical blood tests, serology.

It is important to study the cerebrospinal fluid (cerebrospinal fluid) using lumbar or occipital puncture. With Guillain-Barré syndrome, a high protein concentration is found in it - 3-5 g / l.

In severe cases, the main indicators of the body's vital activity are monitored - heart function (ECG, pulse oximetry), blood pressure, respiration.

During the examination, Guillain-Barré syndrome is differentiated from porphyria, ischemic stroke, myasthenia gravis, poliomyelitis and other diseases. Signs that exclude polyradiculoneuropathy are:

• asymmetry of paresis;
• exclusively sensory disorders;
• persistent pelvic disorders;
• severe pelvic disorders;
• recently transferred diphtheria;
• the presence of psychopathological symptoms - hallucinations,;
• proven poisoning with salts of heavy metals and others.

It is imperative to identify symptoms and begin treatment for Guillain-Barré syndrome early. In this case, the probability of complete recovery is high.

Treatment

Treatment of Guillain-Barré syndrome is carried out only in a medical institution. It includes several aspects: proper patient care, instrumental observation and assistance, specific and symptomatic drug therapy, as well as surgical intervention if necessary.

Caring for a person with Guinea-Barre syndrome is aimed at ensuring normal living conditions and preventing complications associated with immobility - bedsores, thrombosis, and so on. To do this, it is necessary to change the position of the patient's body at least once every 2 hours, periodically cleanse his skin, control the work of the intestines and bladder, and carry out passive gymnastics.

Since Guillain-Barré syndrome is sometimes accompanied by weakness and paralysis of the respiratory and bulbar muscles, it is extremely important to constantly monitor the patient's respiratory activity, heart rate and blood pressure using special devices. In some cases, the installation of a pacemaker, connection to a ventilator (with a decrease in lung capacity by 25-30%), the introduction of a nasogastric tube (with bulbar paralysis) and bladder catheterization are required.

Therapy for Guillain-Barré syndrome, the purpose of which is to arrest the autoimmune reaction, has 2 directions:

1. Appointment of class G immunoglobulin. It is administered intravenously for 5 days at 0.4 g / kg. Side effects include headaches, hyperthermia, and nausea. Respiratory functions are improved as a result of treatment.
2. Membrane plasmapheresis. Part of the patient's blood plasma (about 40 ml / kg) is replaced with a chloride solution or rheopolyglucin. Usually 4-6 sessions are performed every other day. Thanks to plasmapheresis, the severity of paresis and the duration of mechanical ventilation are reduced. Side effects - allergies, hemolysis, electrolyte imbalance.

In addition, B vitamins, antihistamines, antipyretics, analgesics, medicines to regulate the heart rate and pressure, antithrombotic and anticholinesterase agents, and drops to moisturize the cornea are prescribed to relieve the symptoms of Guinea-Barré syndrome. With prolonged bulbar disorders and respiratory failure, a tracheo- or gastrostomy may be necessary.

Rehabilitation

Rehabilitation for Guien-Barré syndrome can take months or years. Due to damage to nerve fibers and muscles, a person has to re-learn how to perform elementary actions - walking, writing, using cutlery, and so on. To assess the dynamics of the patient's condition, electroneuromyography is performed.

Major recovery activities include:

• remedial gymnastics;
• massage;
• baths with rhodon and hydrogen sulfide;
• cold and hot shower;
• ozokeritotherapy;
• paraffin applications;
• electrophoresis;
• magnetotherapy and others.

In addition, adherence to a diet high in vitamins B, calcium, magnesium and potassium is shown.

A patient who has had Guillain-Barré syndrome should have regular check-ups. This will allow you to identify the possibility of a relapse in time. Vaccination is strictly prohibited for 12 months after the onset of the disease.

Forecast and prevention

Guillain-Barré syndrome in most cases has a positive prognosis - 70% of patients fully recover. In 15% of cases, residual paresis and paralysis are observed, leading to disability. Mortality is 5%, and its probability increases with age: in children - 0.7%, in the elderly - 8%. In 2-5% of patients, the disease becomes a chronic recurrent form.

Guillain-Barré syndrome does not have specific preventive measures. To reduce the risk of developing pathology, it is necessary to lead a healthy lifestyle and strengthen the immune system.

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

Term Guillain-Barré syndrome is an eponym (i.e., giving a name) to designate 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 vegetative 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 analysis of the characteristic clinical picture and is confirmed by the study of cerebrospinal fluid and electromyographic study (EMG).

Guillain-Barré syndrome is treated 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 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 accounting for 75 - 80% of cases, several atypical variants or subtypes of this syndrome are described and distinguished in the literature, which are 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 pandisautonomy, acute cranial polyneuropathy, pharyngocystic 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 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. 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: non-specific 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 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 medical institution it must be possible to carry out long-term artificial ventilation.
  • 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, the content of blood electrolytes, heart rate, blood pressure, the state of the bulbar muscles (the appearance and growth of swallowing disorders that does not bring relief to cough, hoarseness, speech impairment) are carried out. 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 in the development of respiratory failure, if VC falls below 12-15 ml / kg, and in case of bulbar paralysis and disorders of swallowing and speech below 15-18 ml / kg. In the absence of a 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 using 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 a 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 a 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 significantly fewer side effects, is easier for the patient to tolerate, 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 2-4 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 is 2 g / kg or about 140 g). An alternative regimen of 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 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 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 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 in the same way. 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 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, an eye patch 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, 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-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 (tapping and vibration with simultaneous rotation of the body in the supine position) is indicated every 2 hours during the day.
  • Symptomatic therapy: antiarrhythmic, hypotensive, analgesic. With arterial hypotension, a fall in 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) is performed, and in case of insufficiency effect in combination with corticosteroids: prednisolone 120 - 150 mg, dexazone 8 - 12 mg .. In case of insufficiency of these funds, vasopressors are used: dopamine (50 - 200 mg. diluted in 250 ml. isotonic sodium chloride solution and injected at a rate of 6 12 drops / min), or norepinephrine, or mezaton. For moderate pain, simple analgesics and non-steroidal anti-inflammatory drugs are used. With severe pain syndrome, tramal or cabamazepine (tigretol) or gabapentin (neurontin) is 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.