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

Description of the disease

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

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

Historical reference

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

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

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

The reasons for the development of pathology

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

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

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

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

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

What symptoms indicate illness?

Guillain-Barré neuropathology is characterized by symptoms of three forms of the development of the disease:

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

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

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

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

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

Clinical course of the disease

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

GBS classification

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

The first three are manifested by muscle weakness:

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

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

Diagnostic measures

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

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

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

Why is Guillain-Barré syndrome dangerous?

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

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

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

GBS treatment methods

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

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

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

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

Rehabilitation after treatment

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

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

Forecast and consequences

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

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

Preventive actions

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

Let's sum up

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

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

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

Overview of Guillain-Barré Syndrome

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

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

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

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

Guillain-Barré syndrome symptoms

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

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

Clinical subtypes of Guillain-Barré syndrome.

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

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

Causes and risks of Guillain-Barré syndrome

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

Diagnostics of the Guillain-Barré syndrome

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

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

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

Treatment of Guillain-Barré syndrome

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

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

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

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

Rehabilitation and prognosis of Guillain-Barré syndrome

Most patients have the prospect of a good recovery.

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

Guillain-Barré syndrome is a severe autoimmune disease that affects the peripheral nervous system. The most common manifestation is acute tetraparesis, when movement of all four limbs becomes almost impossible. Other movements also cease, including swallowing, the ability to lift the eyelids, and spontaneous breathing. Despite this, the course of the disease is benign, most cases end in recovery. Transition to a chronic course or relapses are less common. Guillain-Barré syndrome occurs in all countries, regardless of their level of development, with the same frequency - about 2 cases per 100 thousand population, there is no sex dependence. The disease can affect patients of all ages.

Why does the syndrome occur?

The leading developmental mechanism is autoimmune. In most cases, the onset of the disease occurs in the first three weeks after an acute respiratory or intestinal infection. Since a sufficient amount of time passes from the moment of the disease, and the symptoms characteristic of the infectious process have time to pass, the patients themselves, as a rule, do not associate these conditions with each other. The cause may be pathogens such as:

• epstein-Barr virus or human herpes type 4;
• mycoplasma;
• campylobacter, which causes infectious diarrhea;
• cytomegalovirus.

The researchers found that the "sheath" of these pathogens resembles the myelin sheath of the axon of peripheral nerves. This similarity causes antibodies to attack the nerves, which are initially produced and circulated in the blood in response to the appearance of an infectious agent. This phenomenon is called "molecular mimicry" and explains why immune complexes attack the body's own tissues.

Cases are described when the syndrome occurs after vaccination, after surgery and abortion, hypothermia, stress. In some cases, the cause cannot be found.

How does the syndrome manifest?

Within a few days, up to a maximum of 1 month, muscle weakness in the legs increases, and difficulty in walking occurs. Further, the hands weaken, the mimic muscles suffer the last. Such symptoms have a separate name - Landry ascending paralysis.

But sometimes the paralysis starts from the top, from the arms, spreading downward, but all the limbs always suffer.

Every fifth case is accompanied by paralysis of the musculature of the trunk, namely the diaphragm and intercostal muscles. With such paralysis, breathing becomes impossible, and artificial ventilation is required.

A common manifestation is bulbar syndrome or bilateral paralysis of the muscles of the soft palate, when swallowing and clear speech is impossible.

Together with motor fibers, sensory fibers are sometimes affected. Sensory disturbances develop, tendon reflexes decrease, and pain in the limbs worries. The pains are pronounced "neuropathic" in nature - burning, a feeling of current flow, tingling. Pelvic disorders are rare, but urinary retention is the most common cause of urinary retention, in some cases associated with excess urine production.

Autonomic dysfunction joins, which is manifested by fluctuations in blood pressure, palpitations, other heart rhythm disturbances, sweating, and a lack of intestinal motility.

Classification

Several forms are distinguished, depending on whether the myelin sheath or axon is damaged, according to the severity of the course and the prognosis:

• acute inflammatory demyelinating polyneuropathy or ARDP, when the myelin sheath is destroyed;
• acute motor or sensory-motor axonal neuropathy, where axons are destroyed
• rare forms - Miller-Fisher syndrome, acute pandisautonomy and others, the frequency of which does not exceed 3%.

Diagnostic measures

• muscle weakness in the limbs that progresses;
• decrease or absence of tendon reflexes from the first days of the disease.

WHO also identifies additional signs that confirm the diagnosis, which include:

• symmetry of the lesion;
• symptoms increase no more than 4 weeks;
• sensitivity disorders like "gloves and socks";
• involvement of the cranial nerves, especially the facial;
• possible spontaneous restoration of functions after stopping the progression of the disease (the so-called "plateau");
• the presence of vegetative disorders;
• absence of hyperthermia (if there is a fever, then it is caused by other infections);
• an increase in the amount of protein in the cerebrospinal fluid, while its cellular composition does not change (protein-cell dissociation).

Final diagnosis is impossible without electroneuromyography or ENMG. This test identifies which part of the nerve is damaged - the myelin sheath or the axon. ENMG also accurately determines the extent of the lesion, its severity and the possibility of recovery.

Since, in addition to Guillain-Barré syndrome, there are a number of acute, subacute and chronic polyneuropathies, elecroneuromyography allows differential diagnosis between them and contributes to the development of the correct treatment tactics.

Often, for diagnosis, a lumbar puncture is necessary, followed by a study of the cerebrospinal fluid, and such analyzes as can be informative:

• blood for autoantibodies to neuronal structures;
• blood tests for class A gammaglobulins (especially if immunoglobulin therapy is planned);
• biomarkers of neurofilament (part of the cytoplasm of a neuron);
• tau protein markers (a special protein that destroys a neuron).

The specialists of the CELT clinic additionally use their own algorithms for differential diagnosis, which make it possible to reliably distinguish Guillain-Barré syndrome from other diseases that cause progressive muscle weakness in all limbs or tetraparesis.

Our doctors

Treatment rules

Today, there are two main pathogenetic methods of treatment of Guillain-Barré syndrome, and both are successfully used by CELT specialists. These are plasmapheresis and intravenous immunotherapy. These methods can be used in isolation or used in combination, it all depends on the specific clinical situation. Treatment is aimed at removing or neutralizing immune complexes circulating in the patient's blood. Both methods of treatment are equivalent, almost always lead to recovery. Treatment stops the process of destruction of peripheral nerves, shortens the recovery period, and helps to reduce neurological deficits.

Plasmapheresis is a blood purification operation. Most often, hardware plasmapheresis is used on continuous separators, during which the blood taken from the body is separated into formed elements (or blood cells) and plasma (or serum). All toxic substances are in the plasma, so it is removed. A person is returned to his own blood cells, diluted, if necessary, with plasma-substituting solutions or donor plasma. The duration of the procedure is about one and a half hours, the whole course consists of 3 or 5 sessions. No more than 50 ml / kg of plasma body weight is removed at a time.

In the course of treatment, blood parameters are monitored: electrolytes, hematocrit, clotting time and others.

Intravenous immunotherapy is the introduction of a preparation of human immunoglobulin of class G. These immunoglobulins stop the production of antibodies to their own nerves, simultaneously reducing the production of substances that support inflammation. These drugs are indicated for the pathogenetic treatment of Guillain-Barré syndrome in both adults and children.

Along with specific treatment, careful patient care is provided, including the prevention of pressure sores, pneumonia, contractures. Treatment of associated infections is often required. Venous thrombosis is prevented, tube feeding is performed, and excretory function is monitored. In bed patients, passive gymnastics is performed, as well as early verticalization to avoid blood flow disorders. With the threat of the development of contact (immobility of the joints), paraffin procedures are possible. Biofeedback motor trainers are used as needed.

Patients with damage to the myelin sheaths recover faster, while axonal injuries require a longer rehabilitation period. Axonal lesions often leave behind a neurological deficit that is difficult to correct.

Prevention

The main method is the complete cure of infections that we consider commonplace and commonplace. Guillain-Barré syndrome often develops with a slight weakening of the immune system, which is possible in every person.

The easiest way to keep yourself safe is to check your current immune status. It only takes a few days, and the detected deviations can be treated in time.

The doctors of the CELT clinic have at their disposal not only the latest diagnostic equipment, but also the newest medical techniques that have received worldwide recognition. The main role in prevention belongs to the patient, who applies for examination and treatment in a timely manner.

Guillain-Barré Syndrome - symptoms and treatment

What is Guillain-Barré Syndrome? We will analyze the causes of occurrence, diagnosis and treatment methods in the article by Dr. Zhuikov A.V., a neurologist with 19 years of experience.

Definition of disease. Causes of the disease

Guillain-Barré Syndrome (GBS) - an acute autoimmune disease of the peripheral nervous system, characterized by muscle weakness. This disorder encompasses a group of acute disorders of the peripheral nervous system. Each variant is characterized by peculiarities of the pathophysiology and clinical distribution of weakness in the limbs and cranial nerves.

70% of patients with GBS had a previous infectious disease before the onset of neurological symptoms.

If you find similar symptoms, consult your doctor. Do not self-medicate - it is dangerous for your health!

Guillain-Barré syndrome symptoms

Symptoms of ARVI or gastrointestinal tract disorders are observed in 2/3 of patients. The first symptoms of GBS are paresthesias of the fingers of the extremities, followed by progressive muscle weakness of the lower extremities and gait disturbances. The disease progresses over several hours or days, with weakness in the upper limbs and cranial nerve palsy. Paralysis is usually symmetrical and, of course, peripheral. In half of patients, pain may be the initial complaint, making diagnosis difficult. Ataxia and pain are more common in children than in adults. Retention of urine occurs in 10% -15% of patients. Damage to the autonomic nerves is manifested by dizziness, hypertension, excessive sweating, and tachycardia.

An objective examination reveals ascending muscle weakness, as well as areflexia. Tendon reflexes of the lower extremities are absent, but reflexes of the upper extremity can be elicited. Muscle weakness can also involve the respiratory muscles. The defeat of the cranial nerves is noted in 35-50%, autonomic instability in 26% -50%, ataxia in 23%, dysesthesia in 20% of cases.

The most common signs of autonomic dysfunction are sinus tachycardia or bradycardia and hypertension. In patients with severe autonomic dysfunction, changes in peripheral vasomotor tone with hypotension and lability of blood pressure are observed.

Infrequent variants of the clinical course of the disease include fever at the beginning of neurological symptoms, severe sensory impairment with pain (myalgias and arthralgias, meningismus, radicular pain), and sphincter dysfunction.

GBS should be considered in any patient with rapid onset of acute neuromuscular weakness. In the early stages of GBS, it should be distinguished from other diseases with progressive symmetrical muscle weakness, including transverse myelitis and myelopathy, acute toxic or diphtheria polyneuropathy, porphyria, myasthenia gravis, and electrolyte disturbances (eg, hypokalemia).

Pathogenesis of Guillain-Barré syndrome

The neurophysiological processes underlying GBS are subdivided into several subtypes. The most common subtypes include:

• acute inflammatory demyelinating polyradiculopathy;
• acute motor axonal neuropathy;
• acute motor and sensory axonal neuropathy;
• miller-Fisher syndrome, as a variant of GBS, is characterized by a triad of signs: ophthalmoplegia, ataxia and areflexia.

It is believed that GBS develops due to the production of antibodies against the protein of the infectious agent, which cross-react with the gangliosides of human nerve fibers. Autoantibodies bind to myelin antigens and activate complement, with the formation of a membrane-attacking complex on the outer surface of Schwann cells. Damage to the sheaths of the nerve trunks leads to conduction disturbances and muscle weakness (axonal degeneration can also occur at a later stage). Demyelinating lesion occurs along the entire length of the peripheral nerve, including the nerve roots.

All types of nerves are affected, including autonomic, motor, and sensory fibers. Motor nerve involvement occurs significantly more frequently than sensory nerve involvement.

Complications of Guillain-Barré syndrome

Patients with HBS are at risk of life-threatening respiratory complications and autonomic disorders.

Indications for transfer to the intensive care unit include:

• rapid progression of motor weakness with damage to the respiratory muscles;
• ventilation respiratory failure;
• pneumonia;
• bulbar disorders;
• severe autonomic insufficiency.

Complications of ongoing treatment that require intensive care include fluid overload, anaphylaxis to intravenous immunoglobulin administration, or hemodynamic disturbances during plasmapheresis.

15% -25% of children with HBS develop decompensated respiratory failure, which requires mechanical ventilation. Respiratory disorders are more common in children with rapid disease progression, upper limb weakness, autonomic dysfunction, and cranial nerve damage. Intubation of the trachea may be required in patients to protect the respiratory tract, mechanical ventilation. In GBS, rapid progression, bilateral facial nerve palsy, and autonomic dysfunction determine an increased likelihood of intubation. Planning for early intubation is essential to minimize the risk of complications and the need for emergency intubation.

Autonomic dysfunction increases the risk of endotracheal intubation. On the other hand, dysautonomy may increase the risk of hemodynamic reactions to drugs used to induce anesthesia during intubation.

Signs indicating the need for mechanical ventilation:

1. ventilation respiratory failure;
2. an increase in oxygen demand to maintain SpO2 above 92%;
3. signs of alveolar hypoventilation (PCO2 above 50 mm Hg);
4. rapid decrease in vital capacity by 50% compared to the baseline;
5. inability to cough

Autonomic dysfunction is the main factor in mortality in GBS. Fatal cardiovascular collapse due to autonomic dysfunction occurs in 2% -10% of critically ill patients. Heart rate, blood pressure, and electrocardiogram monitoring should be continued for as long as patients require respiratory support. Percutaneous pacing may be required for severe bradycardia. Hypotension is corrected by circulating blood volume (BCC) replenishment, and if the patient is unresponsive to BCC replenishment, α-agonists are used, such as norepinephrine, mezaton, adrenaline.

With unstable hemodynamics, continuous recording of arterial and central venous pressure should be performed to control the volume of infusion therapy.

Arterial hypertension can occur, but this complication does not require special treatment unless it is complicated by pulmonary edema, encephalopathy, or subarachnoid hemorrhage.

Diagnostics of the Guillain-Barré syndrome

Instrumental diagnostics

Lumbar puncture

With lumbar puncture, CSF results usually show elevated protein levels (\u003e 45 mg / dL), without pleocytosis (<10 клеток/мм3) (белково-клеточная диссоциация). Иногда уровень белка может оставаться нормальным, при умеренном повышении количества клеток (10-50 клеток/мм3). Цитоз выше, чем 50 клеток/мм3, свидетельствует против диагноза ГБС. В ряде случаев могут быть необходимы повторные люмбальные пункции для уточнения диагноза.

Neurofunctional diagnostics

ENMG (Electroneuromyography) - the only instrumental diagnostic method that allows to confirm the diagnosis of GBS and clarify the nature of pathological changes (demyelinating or axonal) and their prevalence.

Needle electromyography is characterized by the presence of signs of the current denervation-reinnervation process in polyneuropathy. Examine the distal muscles of the upper and lower extremities (for example, the tibialis anterior muscle, the common extensor of the fingers), and, if necessary, the proximal muscles (for example, the quadriceps muscle of the thigh).

The ENMG study in patients with GBS depends on the clinical manifestations:

• with distal paresis, long nerves on the arms and legs are examined: at least four motor and four sensory (motor and sensory portions of the median and ulnar nerves; peroneal, tibial, superficial peroneal and sural nerves on one side).

Assessment of the main ENMG parameters:

• motor responses (distal latency, amplitude, shape and duration), the presence of blocks of conduction and dispersion of responses; the speed of propagation of excitation along motor fibers in the distal and proximal areas is analyzed.
• sensory responses: the amplitude and speed of excitation conduction 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.
• in case of proximal paresis, a study of two short nerves (axillary, musculocutaneous, femoral, etc.) with an assessment of the parameters of the motor response (latency, amplitude, shape) is mandatory.

The first signs of a denervation process appear two to three weeks after the onset of the disease, signs of a reinnervation process - after a month.

Treatment of Guillain-Barré syndrome

General supportive care and treatment

Patients requiring intensive care require careful general care. Constipation is observed in more than 50% of cases of patients with GBS as a result of dynamic intestinal obstruction.

Paracetamol is used for pain. Katadolon and tramadol are used for severe pain syndrome. For neuropathic pain, carbamazepine and gabapentin are effective.

In the treatment of GBS, various types of immunomodulatory therapy are being undertaken.

Intravenous immunoglobulin is given as a daily infusion (0.4 g / kg / day) for 5 days in the first 2 weeks of illness. A second course of immunoglobulin may be required in 5% -10% of patients, with negative dynamics after initial improvement. The mechanism of action of intravenous immunoglobulin is likely multifactorial and is believed to include modulation of complement activation, neutralization of idiotypic antibodies, suppression of inflammatory mediators (cytokines, chemokines).

Side effects of immunoglobulin include headache, myalgia and arthralgia, flu-like symptoms, and fever. Patients with IgA deficiency may develop anaphylaxis after the first course of intravenous immunoglobulin.

Plasmapheresis helps to remove antibodies involved in the pathogenesis of GBS. During each session, 40-50 ml / kg of plasma is replaced with a mixture of 0.9% sodium chloride and albumin solution. Plasmapheresis leads to a reduction in the recovery time and a decrease in the need for artificial ventilation. These advantages are evident if plasmapheresis is performed within the first two weeks after the onset of the disease. Complications associated with plasmapheresis include venipuncture hematoma, pneumothorax after subclavian vein catheterization, and sepsis. Plasmapheresis is contraindicated in patients with severe hemodynamic instability, bleeding, and sepsis.

The combination of plasmapheresis and immunoglobulin has not shown clinical benefit.

Corticosteroids should not be used in the treatment of GBS, since they do not accelerate recovery, do not reduce the likelihood of mechanical ventilation, and do not affect long-term outcome.

Forecast. Prevention

GBS remains a serious illness despite improved treatment outcomes. Compared to adults, children are more likely to have a more favorable course of the disease, with complete rather than partial recovery. The reasons for an unfavorable outcome in GBS are respiratory failure, complications of mechanical ventilation (pneumonia, sepsis, acute respiratory distress syndrome and thromboembolic complications), cardiac arrest secondary to dysautonomy.

Recovery usually begins two to four weeks after symptom progression stops. The average time from the onset of the disease to complete recovery is 60 days. Data on the long-term outcome of GBS are limited. 75% - 80% of patients recover completely. About 20% of patients are unable to walk after six months.

The younger age group (less than 9 years old), rapid progression and maximum muscle weakness, and the need for mechanical ventilation are important predictors of long-term motor deficits.

Guillain-Barré syndrome is a disease in which the sheath of nerve fibers (myelin) is destroyed, which leads to impaired movement, sensitivity disorders. It usually develops some time after the infection.

Myelin is a special sheath of nerve fibers that is necessary for the conduction of nerve impulses. In Guillain-Barré syndrome, it is destroyed by the body's own immune system. Normally, the immune system detects and destroys foreign objects (for example, pathogens of infectious diseases), but in some cases, it begins to fight native cells. As a result of damage to the myelin sheath, manifestations of the disease occur: decreased strength in the muscles, tingling in the extremities, etc. Most patients require hospitalization.

Timely started treatment allows you to achieve complete recovery, although some people may still have weakness in the muscles, a feeling of numbness.

Synonyms Russian

Acute inflammatory demyelinating polyradiculoneuropathy, acute polyradiculitis.

Synonymsenglish

Guillain-Barre syndrome, Acute Idiopathic Polyneuritis, Acute Inflammatory Demyelinating Polyradiculoneuropathy.

Symptoms

• Decreased strength in the muscles, tingling - first in the legs, then in the overlying parts of the body
• Intense pain in the shoulder girdle, back, hips
• Violation of chewing, swallowing, pronunciation of sounds, facial expressions as a result of a decrease in the strength of the muscles that perform these functions
• Increased or slowed heart rate
• Increase or decrease in blood pressure
• Respiratory disorders, with an increase in which artificial ventilation of the lungs may be required (carried out by a special apparatus when spontaneous breathing is ineffective)
• Retention of urine
• Constipation

General information about the disease

Guillain-Barré syndrome is a disease in which the myelin sheath of nerves is destroyed, as a result of which the conduction of nerve impulses is disrupted and the strength in the muscles decreases.

The exact causes of the disease are unknown. In most cases, symptoms appear 1-3 weeks after an acute infection of the respiratory system, gastrointestinal infections.

These infections can be caused by these and other pathogens:

• campylobacter - found in the meat of infected birds and causes gastrointestinal infection when ingested with food;
• influenza virus;
• epstein-Barr virus (causative agent of infectious mononucleosis);
• mycoplasma - can cause pneumonia in people infected with the immunodeficiency virus (HIV).

Also, the triggering factor for the development of the disease can be vaccinations, surgical interventions.

Autoimmune mechanisms also play an important role. The immune system fights against foreign objects that enter the body. In response to infection, special protein particles are produced - antibodies. They detect and neutralize various infections and viruses. According to the researchers, in Guillain-Barré syndrome, antibodies not only destroy infectious agents, but also damage the membrane of nerve cells, this is possible due to the similarity in the molecular structure of these objects.

The myelin sheath covers nerve fibers and provides a certain speed of nerve impulses between the brain and various structures of the body. Violation of the passage of nerve impulses to muscle fibers leads to a decrease in strength in the muscles. The nerve fibers of the autonomic nervous system (which regulates the activity of internal organs) are also affected. In this case, the work of the cardiovascular system can be disrupted, the heart rhythm, blood pressure, etc.

In severe forms of the disease, the following complications are possible.

• Breathing disorder. It occurs as a result of weakness or paralysis (complete lack of ability to move) of the respiratory muscles and threatens the patient's life. In cases where spontaneous breathing is ineffective, artificial ventilation of the lungs is carried out (using a special apparatus).
• Disturbances in the work of the cardiovascular system.
• Prolonged immobility. Increases the risk of thromboembolism (blockage of blood vessels by blood clots, leading to poor circulation).
• Pressure ulcers are dead skin, underlying soft tissues that occur with prolonged immobility of patients due to a violation of blood supply.

The disease develops within a few weeks, and it may take several months to restore the lost functions. In most cases, complete recovery occurs.

Who is at risk?

• Persons of young and old age.
• Patients with certain types of infectious diseases.
• Underwent surgical interventions.

Diagnostics

Diagnosis of Guillain-Barré syndrome is quite difficult, since there are no specific studies to identify it. In this case, the diagnosis is based on the analysis of clinical manifestations, the study of the history of the disease, analyzes to exclude other diseases of the nervous system.

Laboratory diagnostics is of great importance.

• The protein is common in the cerebrospinal fluid. Cerebrospinal fluid (CSF) washes the brain and spinal cord. Various diseases of the nervous system cause certain changes in its composition. In Guillain-Barré syndrome, the level of protein in the cerebrospinal fluid increases.

To rule out other diseases, the following laboratory tests may be required:

• ... Allows you to determine the number of formed elements in the blood:,. A decrease in the number of erythrocytes and, possibly, with an increase in the level of leukocytes, with various inflammatory processes.
• ... This indicator deviates from the norm for various diseases, in particular, it increases with inflammatory processes in the body.
• ... With an insufficient amount of vitamin B 12 in the body, anemia and disturbances in the functioning of the nervous system may develop. Some of the symptoms of damage to the nervous system in B 12 deficiency anemia are similar to the manifestations of Guillain-Barré syndrome.
• Detection of heavy metals in urine. The accumulation of heavy metals (for example, lead) in the body contributes to damage to the nervous system and the development of polyneuropathy (damage to various nerves).

Other studies:

• Electromyography. Allows you to fix electrical impulses that travel along the nerves to the muscles. According to their intensity, the conductivity of nerve fibers is assessed; for this, special electrodes are applied to the muscle under study. The study is carried out in a calm state and with muscle contraction.

Additional research

• Magnetic resonance imaging (MRI). A diagnostic method based on the effect of a magnetic field on the human body. After processing the received signals, layer-by-layer images of the internal structures of the body are obtained. Allows to exclude the presence of other diseases of the nervous system (for example, masses).

Treatment

Treatment of the disease is conservative. Various drugs are used to eliminate individual manifestations of the disease, to combat the complications of Guillain-Barré syndrome.

The most effective are the following techniques:

• Plasmapheresis. Blood is taken from the patient, which is divided into a liquid part (plasma) and a part containing blood cells (erythrocytes, leukocytes, platelets). The blood cells are then returned to the human body, and the liquid portion is removed. This achieves a kind of purification of blood from antibodies that can destroy the myelin sheath of the nerves.
• Intravenous administration of immunoglobulin. Immunoglobulin contains antibodies from healthy blood donors. They block the destructive effect of the patient's antibodies on the nerve sheath.

It is very important to maintain the impaired functions of the body (artificial ventilation of the lungs), to carefully look after the patient, to prevent complications associated with prolonged immobility of patients.

In the recovery period, physiotherapy exercises, physiotherapy are used to restore the strength of various muscle groups.

Prevention

There is no specific prophylaxis for Guillain-Barré syndrome.

• Total protein in cerebrospinal fluid

Literature

• Dan L. Longo, Dennis L. Kasper, J. Larry Jameson, Anthony S. Fauci, Harrison's principles of internal medicine (18th ed.). New York: McGraw-Hill Medical Publishing Division, 2011. Chapter 385. Guillain-Barré Syndrome.
• Corey Foster, Neville F. Mistry, Parvin F. Peddi, Shivak Sharma, The Washington Manual of Medical Therapeuticts (33th ed.). Lippincott Williams & Wilkins Philadelphia, 2010.23 Neurologic Disorders. Guillain-Barré Syndrome.