Guillain barre treatment. Features of the treatment of Guillain-Barré syndrome

Piradov M.A. 2000

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

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

Guillain-Barré syndrome (G61.0)

Neurology

general information

Short description

Approved
Joint Commission on the quality of medical services
Ministry of Health and Social Development of the Republic of Kazakhstan
dated November 29, 2016
Protocol No. 16

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

Synonyms of Guillain-Barré syndrome: acute inflammatory demyelinating polyneuropathy, acute idiopathic polyneuropathy, infectious polyneuritis (polyneuropathy), acute polyradiculitis, Guillain-Barré-Strohlsyndrome syndrome, Landry-Guillain-Barrésyndrome, Landry-Guillain-Barré-Strohlsyndrome syndrome, Landry'ssyndrome syndrome, Landry's ascending paralysis (Landry'sascendingparalysis), French poliomyelitis (Frenchpolio), etc.
feature this disease is a self-limiting, monophasic course with extremely rare relapses.

Correlation between ICD-10 and ICD-9 codes

CodeMKB-10		ICD-9 code
G61.0	Guillain-Barré syndrome	357.0	Guillain-Barré syndrome

Date of development/revision of the protocol: 2016

Protocol Users: GPs, therapists, resuscitators, neuropathologists (adults, children).

Evidence level scale:

BUT	High-quality meta-analysis, systematic review of RCTs, or large RCTs with a very low probability (++) of bias whose results can be generalized to an appropriate population.
IN	High-quality (++) systematic review of cohort or case-control studies or High-quality (++) cohort or case-control studies with very low risk of bias or RCTs with not high (+) risk of bias, the results of which can be extended to the appropriate population.
FROM	Cohort or case-control or controlled trial without randomization with low risk of bias (+). Results that can be generalized to an appropriate population or RCTs with very low or low risk of bias (++ or +) that cannot be directly generalized to an appropriate population.
D	Description of a case series or uncontrolled study or expert opinion.

Classification

GBS is classified as both a neuroinfection and a post-infection condition. There are several forms of GBS that differ in the course of the pathological process, the primary point of application of autoimmune aggression (nerve sheath or axonal rod), recovery prognosis, and clinical manifestations.

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

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

Neurophysiological criteria for classifying GBS(R. Hadden, D. Cornblath, R. Hughesetal., 1998).
Group with primary demyelinating lesion:
the presence of at least one of the following signs in at least 2 nerves or two signs in one nerve is required if all other nerves are non-excitable and the amplitude of the M-response at the distal point is 10% or more of the lower limit of normal:
Excitation propagation velocity (ERV) 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 upper bound the norm is more than 10%, or more than 20% if the amplitude of the M-response at the distal point is below the lower limit of the norm;
presence of a dispersion or excitation block;
F-wave latency exceeds the upper limit of normal by more than 20%.

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% below the lower limit of the norm), and at least in two nerves the amplitude of the M-response at the distal point is more than than 80% below the lower limit of normal.

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

Indefinite group:
· Changes detected during stimulation ENMG do not meet the criteria of any of the above groups.

Diagnostics (outpatient clinic)

DIAGNOSTICS AT OUTPATIENT LEVEL

Diagnostic criteria:
Complaints:
On increasing muscle weakness in the arms and / or legs;
numbness and decreased sensitivity;
Increased sensitivity (tactile, temperature, etc.) in the hands and feet;
pain in the back, shoulder and pelvic girdle;
Difficulty swallowing, both solid food and liquids;
violation of respiratory functions, up to the absence of independent breathing, due to the weakening of the respiratory muscles, weakening of the voice and cough;
a disorder in the heart rate, in some it can be very fast, in others it can be slowed down;
Paralysis of facial muscles
Increased sweating
fluctuations blood pressure;
possible occurrence of uncontrolled emission of urine;
Loss of tendon reflexes
unsteady and unsteady gait, impaired coordination of movements;
changes in the volume of the abdomen, this happens because it is difficult for a person to breathe with the help of the diaphragm, and he is forced to use the abdominal cavity;
Decreased visual acuity - most often there are bifurcation and strabismus.
Symptoms are inherent in both adults and children and newborns.

Anamnesis: GBS develops, as a rule, 1-3 weeks after an infectious disease (SARS, influenza, sinusitis, bronchitis, pneumonia, tonsillitis, measles, parotitis, diarrhea, etc.).
Neurological symptoms appear suddenly; most patients present with pain and paresthesia.
When taking an anamnesis, it is important to clarify the following aspects.
Presence of precipitating factors. In approximately 80% of cases, the development of Guillain-Barré syndrome is preceded by certain diseases or conditions in 1-3 weeks.
Infections of the gastrointestinal tract, upper respiratory tract, can develop after an intestinal infection caused by Campylobacterjejuni, after infections caused by herpes viruses (cytomegalovirus, Epstein-Barr virus, varicella-zoster virus), Haemophilus influenzae, mycoplasmas, measles, mumps, Lyme borreliosis, etc. In addition, with HIV infection, the development of Guillain-Barré syndrome is possible.
Vaccination (anti-rabies, anti-tetanus, against influenza, etc.);
surgical interventions or injuries of any localization;
taking some medicines(thrombolytic drugs, isotretinoin, etc.) or contact with toxic substances;
Sometimes Guillain-Barré syndrome develops against the background of autoimmune (systemic lupus erythematosus) and tumor (lymphogranulomatosis and other lymphomas) diseases.

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

Physical examination includes:
· general somatic status: general condition and its severity, body temperature, measurement of the patient's weight, examination skin, respiration, pulse, blood pressure, state internal organs(lungs, heart, liver, kidneys, etc.).
· neurological status:
A neurological examination is aimed at identifying and assessing the severity of the main symptoms of Guillain-Barré syndrome - sensory, motor and autonomic disorders.
assessment of the strength of the muscles of the limbs;
Study of reflexes - for Guillain-Barré syndrome, areflexia is characteristic (that is, the absence of most reflexes);
assessment of sensitivity - the presence of skin areas with a feeling of numbness or tingling;
assessment of the function of the pelvic organs - possibly short-term urinary incontinence;
Evaluation of the function of the cerebellum - the presence of unsteadiness in the Romberg position (standing with arms outstretched in front of him and eyes closed), uncoordinated movements;
assessment of movements eyeballs- with Guillain-Barré syndrome, there may be a complete absence of the ability to move the eyes;
Carrying out vegetative tests - to assess the damage to the nerves that innervate the heart;
The reaction of the heart to a sharp rise from a prone position, physical activity is assessed;
Evaluation of swallowing function.

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

Stage 0 Guillain-Barré syndrome is the norm;

Stage 1 - minimal movement disorders;

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

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

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

Stage V of the Guillain-Barre syndrome - the need for artificial ventilation of the lungs;

Stage VI - death.

IN clinical practice to assess the severity of movement disorders, a scale of muscle strength of the limbs is used (A. Szobor, 1976).

0 points - there are no movements in the muscle.

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

2 points - the patient holds the weight of the limb, but the resistance to the researcher is minimal.

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

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

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

Clinical variants of AIDP

Option	Main clinical symptoms
with typical clinical presentation
Acute inflammatory demyelinating polyradiculoneuropathy (typical GBS variant) (>85%)	Weakness in the limbs with relatively mild sensory disturbances (possibly isolated movement disorders).
Acute motor axonal polyneuropathy (>5%)	Weakness in the limbs with no change in sensation. Deep reflexes can be preserved. Quick Recovery functions. Mostly found in children.
Acute motor-sensory axonal polyneuropathy (>1%)	Weakness and sensory disturbances in the limbs. Rapid development of severe motor deficit with slow and incomplete recovery. Mostly found in adults.
With an atypical clinical picture
Miller-Fisher syndrome (>3%)	A combination of ataxia, predominantly of the cerebellar type, with areflexia, ophthalmopolegia, and sometimes slight weakness in the limbs. Sensitivity is usually preserved.

Laboratory research:

KLA - to exclude an inflammatory disease of the internal organs, accompanied by a polyneuropathic syndrome;
blood sugar test (to exclude diabetic polyneuropathy);
biochemical blood tests - creatine, urea, AST, ALT, bilirubin (to exclude metabolic polyneuropathy);
blood test for gas composition, electrolyte concentration - biochemical blood tests help to exclude metabolic polyneuropathies;
Blood PCR for hepatitis viruses - to exclude polyneuropathic syndrome in hepatitis
blood test for HIV infection - to exclude polyneuropathy associated with HIV infection;
· PCR blood test for viral infections (cytomegalovirus, Epstein-Barr virus, Borreliaburgdorferi, Campylobacterjejuni, etc.) - if an infectious etiology of GBS is suspected.

Instrumental research:
X-ray of the chest organs - to exclude inflammatory lung disease or associated pulmonary complications with weakening of the respiratory muscles;
ECG - to detect or exclude vegetative cardiac arrhythmias in the GBS clinic;
Ultrasound of the abdominal organs - diseases of the internal organs (liver, kidneys, etc.) may be accompanied by polyneuropathy similar to GBS;
MRI-brain *-required for differential diagnosis with pathology of the central nervous system (acute cerebral circulation, encephalitis);
MRI of the spinal cord* - to exclude lesions (myelitis) at the level of the cervical thickening of the spinal cord (C4 - Th2);
Electroneuromyography ** (ENMG) - may be normal during the first week of the disease, with muscle damage, a denervation type of ENMG curve is detected, pulse conduction is slow, signs of damage to myelin or axons. Most often, the distal muscles of the upper and lower extremities (eg, tibialis anterior, extensor digitorum common) are examined, and if necessary, proximal muscles (eg, quadriceps femoris).

*NB! Absolute contraindications for MRI are: metallic foreign body in the orbit; intracranial aneurysms clipped with ferromagnetic material; electronic devices in the body (pacemaker); hematopoietic anemia (for contrasting).
Relative contraindications for MRI are:
severe claustrophobia;
metal prostheses, clips located in non-scanned organs;
Intracranial aneurysms clipped with non-ferromagnetic material.

** NB! ENMG is the only instrumental diagnostic method that allows confirming the lesions of the peripheral nervous system and the diagnosis of GBS, respectively, as well as clarifying the nature of pathological changes(demyelinating or axonal) and their prevalence.

The protocol and scope of ENMG studies in patients with GBS depend on the clinical manifestations of the disease:
- 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). The main ENMG parameters are assessed:
motor responses (distal latency, amplitude, shape and duration), the presence of blocks of excitation and dispersion of responses is assessed; the speed of propagation of excitation along the motor fibers in the distal and proximal areas is analyzed;
sensory responses (amplitude) and speed of excitation along sensory fibers in the distal sections;
· late ENMG phenomena (F-waves): latency, form and amplitude of responses, chronodispersion value, dropout percentage are analyzed.
- in the presence of proximal paresis, it is mandatory to additionally study two short nerves (axillary, musculocutaneous, femoral, etc.) with an assessment of the parameters of the motor response (latency, amplitude, shape).
It must be remembered that the first signs of the denervation process appear no earlier than 2-3 weeks after the onset of the disease, and the signs of the reinnervation process - no earlier than 4-6 weeks.

Diagnostic criteria for classic GBS by Asbury A. K. and Cornblath D. R.
based on clinical and laboratory data:
the presence of progressive motor weakness with involvement in the pathological process of more than one limb;
areflexia or severe hyporeflexia;
CSF analysis - the presence in 1 µl of cerebrospinal fluid of no more than 50 monocytes and / or 2 granulocytes 2+.

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

Required Criteria:

progressive motor weakness in more than one limb;

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

Inhibition of reflexes of varying degrees.

Auxiliary criteria for the diagnosis of the syndrome:

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

2. relative symmetry of the lesion;

3. mild degree of sensory disturbances;

4. involvement in the pathological process of cranial nerves;

5. recovery;

6. symptoms of autonomic dysfunction;

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

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

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

10. absence established causes defeat peripheral nerves, such as the effect of hexacarbon, porphyria, diphtheria, other toxic and infectious diseases that mimic GBS.

Signs that absolutely exclude the diagnosis of GBS:
asymmetry of paresis;
exclusively sensory disorders;
Persistent pelvic disorders
Pronounced pelvic disorders;
recent diphtheria;
The presence of psychopathological symptoms - hallucinations, delusions;
Proven poisoning with salts of heavy metals and others.

Diagnostic algorithm:

Diagnostics (hospital)

DIAGNOSTICS AT THE STATIONARY LEVEL

Diagnostic criteria at the hospital level: see ambulatory level.

Complaints and anamnesis: see ambulatory level.

Physical examination: see ambulatory level.

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

Characteristics of the Miller Fisher syndrome.

Stunning, confusion due to hyponatremia associated with overproduction of antidiuretic hormone. Convulsions may occur when the sodium content in the plasma is less than 120 mmol / l.

Characteristics of acute pandisautonomy.
the occurrence of neurological symptoms 1-2 weeks after a viral or bacterial infection;
The presence of an isolated lesion of the autonomic nervous system;
often affected the cardiovascular system(postural hypotension, arterial hypertension, tachycardia, cardiac arrhythmias);
blurred vision, dry eyes, anhidrosis;
dysfunction gastrointestinal tract(paralytic ileus);
Difficulties in urination, acute urinary retention;
Increased sweating, bluish coloration of the skin of the hands and feet, cold extremities;
Stunning, confusion due to hyponatremia associated with overproduction of antidiuretic hormone. Convulsions may occur when the sodium content in plasma is less than 120 mmol / l;
Recovery is gradual and often incomplete.

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

Diagnostic algorithm:
GBS should first of all be differentiated from conditions that can lead to the development of acute peripheral tetraparesis. Differential diagnostic search is greatly simplified when using a unique algorithm developed by researchers of the Federal State Budgetary Institution "NTsN" of the Russian Academy of Medical Sciences.

Differential diagnostic algorithm for acute flaccid tetraparesis (AFT)

Note: OBT-acute flaccid tetraparesis; EMG electromyography; PNP polyneuropathy; GBS - Guillain-Barré syndrome; LP - lumbar puncture; BHAK - biochemical analysis blood; RF - rheumatic factor; CRP - C-reactive protein; CPK - creatinine phosphokinase; MRI - magnetic resonance imaging (not less than 1 T); CT - computed tomography.

Laboratory research: see outpatient level (to those examinations that were listed additionally).

List of main laboratory research:
blood for immunoglobulins - when planning specific therapy with class G immunoglobulins, it is necessary to determine the Ig fractions in the blood, a low concentration of IgA is usually associated with its hereditary deficiency, in such cases there is a high risk of developing anaphylactic shock (immunoglobulin therapy is contraindicated);
CSF research (cytosis, protein concentration). When analyzing liquor, the number diagnostic criteria confirming GBS, it is customary to attribute the following three indicators:
The presence of high protein content,
an increase in the fraction of albumin,
No concomitant increase in cytosis.
Additionally, the following diagnostic tests may be recommended to confirm the diagnosis and clarify the features of GBS in a particular case:
· a blood test for autoantibodies to gangliosides, with a mandatory study of GM1, GD1a, and GQ1b if the patient has oculomotor disorders;
blood test for IgA antibodies to Campylobacter jejuni;
· study of the content of biomarkers of heavy chains of neurofilament, tau-protein and gliofibrillar acidic protein in blood serum.

Instrumental Research: see ambulatory level.

In severe cases of the disease (rapid progression, bulbar disorders), daily monitoring of blood pressure, ECG, pulse oximetry and examination of the function of external respiration (spirometry, peak flowmetry), monitoring of the function of external respiration (determination of the vital capacity of the lungs (VC) should be carried out (in the conditions of the intensive care unit). ) for timely identification of indications for transferring the patient to mechanical ventilation.

Differential Diagnosis

GBS must be differentiated from other diseases manifested by acute peripheral paresis, primarily from poliomyelitis (especially in young children) and other polyneuropathies (diphtheria, with porphyria). In addition, lesions of the spinal cord and brain stem (transverse myelitis, stroke in the vertebrobasilar system) and diseases with impaired neuromuscular transmission (myasthenia gravis, botulism) can have a similar clinical picture.

Diagnosis	Rationale for differential diagnosis	Surveys	Exclusion Criteria diagnosis
Poliomyelitis (especially in young children)	Acute peripheral paresis	· ENMG; needle EMG; consultation of a therapist; consultation infectiologist.	epidemiological history; The presence of fever at the onset of the disease; symptoms of the gastrointestinal tract; asymmetry of the lesion; absence of objective disorders of sensitivity; high cytosis in the cerebrospinal fluid; The diagnosis of poliomyelitis is confirmed by virological or serological testing.
Otherpolyneuropathies (inflammatory: chronic inflammatory polyneuropathy with acute onset, Sjögren's disease, Churg-Strauss disease, cryoglobulinemic vasculitis; Infectious: HIV-associated, Lyme disease; Toxic: diphtheria, porphyria, drug, acute alcohol, heavy metal poisoning Dysmetabolic: polyneuropathy of critical conditions, with renal, hepatic insufficiency, acute hyperglycemic polyneuropathy)	Acute peripheral paresis	· ENMG; needle EMG; · cons.therapist; · kons.nfektionista; biochemical blood and urine tests	signs of the current denervation-reinnervation process; Porphyria is supported by a combination of predominantly motor polyneuropathy with severe abdominal pain, intestinal paresis, arterial hypertension, tachycardia, pronounced mental changes (from depression to delirium), sleep disturbance, and epileptic seizures. priporphyria has a change in the color of urine, which becomes reddish in the light, and then a rich reddish-brown color
Transverse myelitis. Damage at the level of the cervical thickening of the spinal cord (C4 - Th2) post-infectious (M.pneumoniae, Schistosoma), post-vaccination, viral (enteroviruses, herpes), HIV-associated myelitis, with demyelinating diseases of the central nervous system, with systemic diseases (systemic red lupus, Sjögren's disease, acute necrotic vasculitis)	Acute peripheral paresis	· MRI of the spinal cord and brain; · ENMG; cons. therapist; cons.infectionist.	Segmental border of sensory impairment; Persistent pelvic disorders Lack of involvement of mimic and respiratory muscles in severe tetraparesis.
Acute violation of the spinal circulation, in the vertebero-basilar basin. (thrombosis of the spinal cord vessels, vascular malformation, aneurysm, compression, trauma, neoplasm of the spinal cord)	Acute peripheral paresis	· MRI of the brain and spinal cord; · ENMG; cons. therapist; cons. neurosurgeon.	acute development (usually within a few minutes); In most cases, depression of consciousness (coma); Definitive diagnosis is confirmed by MRI of the brain/spinal cord.
myasthenia gravis	Acute peripheral paresis	ENMG.	The variability of symptoms absence of sensory disturbances; characteristic changes in tendon reflexes; Diagnosis is confirmed by EMG (decrement phenomenon detection); positive pharmacological test with prozerin.
Botulism	Acute peripheral paresis	· ENMG; cons.infectionist.	Relevant epidemiological data Descending type of distribution of paresis, Preservation in terms of cases of tendon reflexes, lack of sensory disturbances, no change in whether quore.

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Treatment

Drugs (active substances) used in the treatment

Treatment (ambulatory)

TREATMENT AT OUTPATIENT LEVEL

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

Non-drugtreatment: no.

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

Algorithm of actions in emergency situations: symptomatic treatment measures.

Other types of treatment: no.

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

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

Patient Monitoring:
assessment of the general condition of the patient with a description of the condition of the skin; patient weight;
hemodynamic parameters: number of respiratory movements, A/D, heart rate, pulse;
assessment of neurological status.

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

Treatment (ambulance)

DIAGNOSTICS AND TREATMENT AT THE EMERGENCY STAGE

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

Swallowing score- with bulbar paralysis, swallowing disorder, to prevent aspiration
nasogastric tube.

Breath assessment- perhaps the development of progressive respiratory failure, and not only of the obstructive type due to bulbar palsy, but also with damage to the phrenic nerve (a paradoxical type of breathing is characteristic - when inhaling, the anterior abdominal wall sinks) and intercostal.
Tracheal intubation (for further transfer of the patient to mechanical ventilation).

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

Medical treatment:
Posyndromic therapy according to the protocol for the provision of emergency medical care.

Treatment (hospital)

TREATMENT AT THE STATIONARY LEVEL

Treatment tactics: The main goal of the treatment is: the restoration of vital functions, the elimination of symptoms of an autoimmune disease using specific techniques, the rehabilitation period of the patient, and the prevention of complications. The first thing to do is to place the patient in a hospital, and if necessary, connect him to a lung ventilator, install a catheter if there is a violation of urine emission, install a nasogastric tube if swallowing is difficult.

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

Medical treatment:
Specific therapy for Guillain-Barré syndrome, aimed at stopping the autoimmune process, is currently used pulse therapy with class G immunoglobulins and plasmapheresis (see paragraph - other types of treatment). The effectiveness of each of the methods is relatively the same, so their simultaneous use is considered inappropriate.
Immunoglobulin class G, like plasmapheresis, reduces the length of stay on mechanical ventilation; it is administered intravenously daily for 5 days at a dose of 0.4 g/kg. Possible side effects: nausea, headache and muscle pain, fever.
Symptomatic therapy for Guillain-Barré syndrome is carried out to correct violations of the acid-base and water-electrolyte balance, to correct the level blood pressure, prevention of deep vein thrombosis thromboembolism.
Infusion therapy for the correction of violations of acid-base, water-electrolyte balances, severe arterial hypotension.
With persistent expressed arterial hypertension prescribe antihypertensive drugs (beta-blockers or slow calcium channel blockers) (see CP Hypertension).
With severe tachycardia, β-blockers (propranolol) are prescribed, with bradycardia - atropine (see below).
With the development of intercurrent infections, antibiotic therapy is necessary (broad-spectrum drugs are used).
For the prevention of deep vein thrombosis and pulmonary embolism, low molecular weight heparin is prescribed in prophylactic doses twice a day).
For pain of nociceptive origin (muscular, mechanical), NSAIDs are recommended, in case of neuropathic pain, the drugs of choice are gabapentin, carbamazepine, pregabalin (only for adults!) (see below).

List of essential medicines:.

Preparations	single dose	Multiplicity of introduction
Immunoglobulin class G	0.4 g/kg IV.	. 0.4 g/kg/day for 5 days once a day, 5 days.
gabapentin	300 mg	Day 1 300 mg 1 time / day, Day 2 300 mg 2 times / day, Day 3 300 mg 3 times / day, then, depending on individual tolerability and effectiveness, the dose may be increased by 300 mg / day every 2-3 days up to a maximum of 3600 mg / day.
carbamazepine	200 mg	The recommended starting dose is 200-400 mg per day. The dose can be gradually increased until a satisfactory clinical effect is obtained, in some cases it can be 1600 mg per day. After the pain syndrome goes into remission, the dosage can be gradually reduced.
pregabalin	150 mg	Treatment begins with a dose of 150 mg per day, divided into two or three doses. Depending on the individual response of the patient and tolerability, after 3-7 days the dose can be increased to 300 mg per day, and if necessary after another 7 days - up to a maximum dose of 600 mg per day.

List of additional medicines:.

Preparations	single dose	Multiplicity of introduction
nifedipine	10 mg	1-2 times under the tongue
Propranolol	10 mg	20 mg 3 times / day, then the dose is gradually increased to 80-120 mg in 2-3 doses, under the control of blood pressure, heart rate, ECG
Atropine	0,5-1,0	adults: intravenous bolus under the control of ECG and blood pressure - 0.5-1 mg, if necessary, the introduction is repeated after 3-5 minutes; the maximum dose is 0.04 mg/kg (3 mg). Children - 10 mcg / kg .;
Ketorolac	10 mg	orally once at a dose of 10 mg or repeatedly, depending on the severity of the pain syndrome, 10 mg up to 4 times a day. The maximum daily dose should not exceed 40 mg, or no more than 60 mg is administered intramuscularly for 1 injection; usually 30 mg every 6 hours. Not used in children.
Diclofenac	75 mg	intramuscularly, a single dose of 75 mg, the maximum daily dose is 150 mg (with a break between injections of at least 30 minutes). Children do not apply.
Ibuprofen	0.2 g	1-2 tablets 3-4 times a day; if necessary - 1 tablet every 4 hours. Do not take more often than after 4 hours. The maximum daily dose for adults should not exceed 1200 mg (no more than 6 tablets in 24 hours). Children: 10-20 mg/kg 3 times a day for 2-3 days.

Surgical intervention, indicating indications for surgical intervention: Surgical intervention may be needed for tracheostomy in case of prolonged mechanical ventilation (more than 10 days), as well as gastrostomy in case of severe and prolonged bulbar disorders.

Other types of treatment:
You should always remember the exceptional importance of a complex of rehabilitation measures for the prevention of complications due to immobility of the patient and for maintaining the functional state of the muscles until a sufficient volume of independent movements appears.
The patient needs:
- Physiotherapy
- Massage has a beneficial effect on metabolism, which also accelerates nerve growth and reinnervation
- Physiotherapy to prevent the formation of contractures (electrical stimulation, heat treatment, drug electrophoresis, etc.).
- Hyperbaric oxygen therapy.
Membrane plasmapheresis significantly reduces the severity of paresis and the duration of mechanical ventilation. As a rule, 4-6 sessions are carried out with an interval of one day; the volume of plasma to be replaced in one session should be at least 40 ml/kg. As replacement media, 0.9% sodium chloride solution or reopoliglyukin is used.
It should be remembered about contraindications to plasmapheresis (infections, bleeding disorders, liver failure), as well as about possible complications(violation of the electrolyte composition, hemolysis, allergic reactions).

Indications for expert advice:
consultation of an infectious disease specialist if necessary (in the absence of a specialist at the prehospital level) - the establishment or exclusion of a chronic infection (brucellosis, borreliosis, etc.), as well as in case of confirmation of an infectious agent to correct etiological therapy;
consultation of a therapist if necessary (in the absence of a specialist at the prehospital level) - the establishment or exclusion of a therapeutic disease (inflammatory disease of internal organs: lungs, kidneys, liver, etc.), correction of hemodynamic parameters, electrolyte balance during therapy;
consultation of an ICU doctor - treatment of patients with severe forms of Guillain-Barré syndrome is carried out jointly with the doctor of the intensive care unit;
consultation of a cardiologist - in case of severe cardiovascular disorders (persistent severe arterial hypertension, arrhythmias).

Indications for transfer to the intensive care unit and resuscitation:
Severe and extremely severe degree of neurological disorders;
hemodynamic instability;
violation of respiratory function.

Treatment effectiveness indicators:
Stabilization of the immunological status (quantitative and qualitative composition of IgG blood and cerebrospinal fluid);
regression of focal neurological symptoms.

Further management.
After the normalization of the patient's state of health, he must be registered with a neurologist. In addition, it will be necessary to undergo preventive examinations in order to identify the prerequisites for a relapse of the disease at an early stage. Dispensary observation in the clinic at the place of residence.
After the end of the acute period, complex rehabilitation measures are necessary, the plan of which is drawn up on an individual basis, depending on the severity of the residual symptoms (exercise therapy, massage, while thermal procedures are contraindicated!).
Patients with GBS. should be informed about the need to observe the protective regime for at least 6-12 months after the end of the disease. Physical overload, overheating, hypothermia, excessive insolation, alcohol intake are unacceptable. Also during this period, you should refrain from vaccination.

medical rehabilitation

is carried out in accordance with the Standard for organizing the provision of medical rehabilitation to the population of the Republic of Kazakhstan, approved by order of the Minister of Health of the Republic of Kazakhstan dated December 27, 2013 No. 759.

Palliative care

Depending on the type and severity of complications following the illness, additional treatment may be required, such as:
Immobilized patients are given heparin subcutaneously at a dose of 5,000 IU every 12 hours and temporary compression of the calf muscles to prevent deep vein thrombosis;
massage has a beneficial effect on metabolism, which also accelerates the growth of nerves and reinnervation;
kinesiotherapy has been proven to stimulate reinnervation and restore muscle volume;
physiotherapy to improve strength, to prevent the formation of contractures (electrical stimulation, heat therapy, drug electrophoresis);
· rehabilitation for the development of everyday skills and the use of adaptive products that help in everyday life;
The patient may need orthotics or other assistive devices to improve movement.
psychotherapy;

Hospitalization

Indications for planned hospitalization: no.

Indications for emergency hospitalization:
Patients with GBS are subject to hospitalization in the intensive care unit.

Information

Sources and literature

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

Information

ABBREVIATIONS USED IN THE PROTOCOL

CIDP	chronic inflammatory demyelinating polyradiculoneuropathy
PNP	polyneuropathy
NMSP	hereditary motor-sensory polyneuropathy
SGB	Guillain-Barré syndrome
HELL	arterial hypertension
PNS	peripheral nervous system
CNS	central nervous system
MRI	Magnetic resonance imaging
PCR	polymerase chain reaction
CSF	cerebrospinal fluid
ESR	sedimentation rate of erythrocytes
Ig	immunoglobulin
heart rate	heart rate
AIDS	acquired immunodeficiency syndrome
EMG	electromyography
ENMG	electroneuromyography
IVIG	normal human immunoglobulin intravenous administration
GC	glucocorticoids

List of protocol developers with qualification data:
1. Kaishibayeva Gulnaz Smagulovna, Candidate of Medical Sciences, JSC "Kazakh Medical University of Continuing Education", Head of the Department of Neurology, certificate "adult neuropathologist".
2. Zhumagulova Kulparam Gabibulovna, candidate of medical sciences, certificate "adult neuropathologist of the highest category", JSC "Kazakh Medical University of Continuing Education", associate professor of the department of neurology.
3. Raykhan Yesenzhanovna Tuleutaeva, clinical pharmacologist, candidate of medical sciences, professor of the Russian Academy of Natural Sciences, head of the Department of Pharmacology and Evidence-Based Medicine, Semey State Medical University.

Indication of no conflict of interest: no.

List of reviewers:
Dushanova G.A. - Doctor of Medical Sciences, Professor, Head of the Department of Neurology, Psychiatry and Psychology of the South Kazakhstan State Pharmaceutical Academy.

Indication of the conditions for revising the protocol: Revision of the protocol 3 years after its publication and from the date of its entry into force or if there are new methods with a level of evidence.

Attached files

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Diseases of an autoimmune nature have not been fully understood to date. They can be diagnosed in both a child and an adult, and manifest themselves in different ways. One such problem is Guillain-Barré syndrome. It is accompanied by damage to the myelin sheath of the nerves, which leads to the formation of symptoms of motor and sensory disorders.

As a rule, this violation is the result of an infection or a long-term suppression of the body's defenses. Treatment of the syndrome is best started as soon as possible after the first signs appear. It is aimed at combating movement disorders and cleansing the blood of abnormal immune complexes.

The main signs of Guillain-Barré syndrome

Initially, the pathology has much in common with viral infections and is manifested by fatigue and weakness, a rise in body temperature and aching joints. As Guillain-Barré disease progresses, it acquires pronounced specific symptoms:

Weakness of the arms and legs, which begins with discomfort in the feet, then spreads to the shins and hands. The pain is replaced by a complete lack of sensitivity and numbness of the limbs, and both left and right are affected immediately. The patient loses control over his own movements: he is not able to get out of bed and hold any objects in his hands.
Since the main pathogenesis of the disease is muscle damage, difficulty in swallowing is manifested. A person chokes not only on food and drink, but also on his own saliva. At the same time, along with paralysis of the larynx, weakening of the masticatory muscles also develops.
Guillain-Barré disease also accompanies such a symptom as incontinence. The bladder and urethral sphincters relax, which leads to unpleasant consequences. In 90% of cases, such a manifestation is also associated with flatulence, as well as the inability to control the passage of gases from the intestines.
Patients have a significant increase in the volume of the abdomen. It is difficult for them to breathe from the chest due to the weakening of the diaphragm, so they have to use the abdominal muscles. The consequences of such a symptom can be threatening, as it leads to asphyxia.
The most dangerous are autonomic disorders, manifested by a decrease in blood pressure, a decrease in the frequency of respiratory movements and tachycardia.

Polyradiculoneuropathy (multiple lesions of peripheral nerves, manifested by flaccid paralysis, sensory disturbances, trophic and vegetovascular disorders) diffusely affects the body, that is, many nerves, both central and peripheral, are involved in the process. This is the reason for such a variety of symptoms.

It is also customary to distinguish several forms of the disease. Polyradiculoneuropathy accounts for up to 90% of cases of problem detection and is accompanied by a variety of manifestations of dysfunctions of nervous structures. The axonal type of disorder proceeds with a selective lesion of the motor fibers. Miller-Fisher syndrome is another type of disease that is manifested by cerebellar ataxia and the absence of reflex reactions.

Causes of pathology

The exact pathogenesis of the formation of Guillain-Barré syndrome is currently unknown. Only the fact of the presence of a provoking factor, which is an immunological reaction, has been confirmed. At autopsy of patients who had this diagnosis, revealed a large number of macrophages involved in damage to the myelin sheath of nerve structures. This explains both the pre-existing infection syndrome and the clinical manifestations of the problem.

One of the most studied triggers of the disease is Campylobacter jejuni, a bacterium that provokes the development of gastrointestinal disorders. Cytomegalovirus also takes an active part in the formation of autoimmune processes that provide clinical manifestations of the disease. Normally, only the formation of antibodies against these pathogens occurs. In patients with Guillain-Barré syndrome, pathological activity of complement is recorded - one of the components of the immune response, as well as macrophages. They react with their own glycolipids, which are part of the nerve sheath, and this causes the manifestation of clinical signs. This pathogenesis explains the success of methods based on the replacement of patients' blood plasma.

Since the disease has an autoimmune nature, the factors that influence its occurrence are in one way or another connected with the work of the body's defenses.

Various infections, both bacterial and viral, provoke the development of polyneuritis. Normally, antibodies are formed to fight the pathogen, but with Guillain-Barré syndrome, these compounds also attack healthy cells of the nervous system, causing the development of the clinical picture. At the same time, one of the reasons, also indirectly associated with the penetration of a foreign agent into the body, is vaccination. The greatest danger is the manifestation of polyneuritis in HIV-positive patients.
Damage to the nervous structures, such as traumatic brain injury or violation of the integrity of peripheral connections, provoke the development of the inflammatory process. Since the immune system takes an active part in this cascade of reactions, damage to normal neurons occurs, which entails further neurological deficit.
The significance of genetic predisposition does not have precise medical evidence, but the presence of cases of detection of autoimmune polyneuritis in the patient's family history increases the risks of its diagnosis in the future.
Inhibition of the natural defense reaction associated with surgical interventions, the course of taking corticosteroid drugs, as well as chemotherapy treatment increases the risk of the disease.

Diagnostics

Identifying Guillain-Barré syndrome is usually not difficult. A careful history and duration of clinical manifestations, as well as the infectious problems preceding them, are important. Diagnosis is carried out by a neurologist who examines the patient to determine the severity of motor and sensory disorders. The examination includes non-specific urine and blood tests, as well as electromyography, which allows you to confirm the presence of anomalies in neuromuscular impulse transmission.

Of great importance is differential diagnosis, which is aimed at excluding pathologies characterized by similar clinical signs. These include Epstein-Barr syndrome, peripheral paresis against the background of poliomyelitis, stroke. This may require a number of specific tests, as well as a photo of the brain using magnetic resonance imaging if a central origin of the anomaly is suspected. It is important to determine the root cause of the onset of symptoms, since the further prognosis depends on this.

Therapeutic activities

The basis of the treatment of Guillain-Barré syndrome is the relief of autoimmune processes and the maintenance of the functioning of vital organs. All patients with a confirmed diagnosis require hospitalization.

Non-drug methods

The most dangerous complication of polyneuropathy accompanying the disease is a violation of normal respiratory activity. This is due to paralysis of the diaphragm and intercostal muscles. In such cases, patients require oxygen therapy aimed at preventing hypoxia. Intubation is carried out, which consists in installing a special tube for artificial ventilation of the lungs. Such a procedure is possible only in stationary conditions and requires the patient to be placed in the intensive care unit and intensive care unit.

Proper care is also important. Bladder catheterization is performed, which avoids complications associated with incontinence. It is also important to prevent the formation of bedsores in patients who cannot independently change position.

Drugs and plasmapheresis

basis drug treatment is the use of class G immunoglobulins. Their use improves the outcome of the disease and allows the restoration of spontaneous respiration in patients who undergo mechanical ventilation. Side effects from the introduction of the drug are rare.

An effective method is plasmapheresis - the replacement of the liquid part of the patient's blood. This allows you to cleanse the body of pathological immune complexes that provoke disruption of the nervous system.

Surgical treatment

Surgical intervention is indicated in the absence of restoration of normal respiratory activity. It consists in the formation of a tracheostomy. Its installation is also indicated for spasm of the larynx, when asphyxia occurs as a result of paralysis of the upper respiratory tract. Many patients are difficult to feed. If the person is not receiving adequate nutrition, a gastrostomy is indicated. It is a tube placed in the stomach. Surgical treatment in many cases is associated with a poor prognosis and a long period of rehabilitation.

Symptomatic therapy

This type of treatment is aimed at restoring the salt balance due to intravenous infusions of solutions. With severe arterial hypertension and tachycardia, specific agents are used, for example, adrenoblockers. Often, Guillain-Barré syndrome is also associated with pain, which is stopped with the help of non-steroidal anti-inflammatory drugs. The use of antidepressants also has good reviews, since many patients are diagnosed with severe anxiety.

Forecast

The outcome depends on the severity of clinical manifestations and the reasons that provoked their manifestation. Mortality in the disease is low, but the percentage of complications is high. After the patient is discharged from the hospital, he needs long-term assistance to restore a normal lifestyle. The prognosis is also related to the accuracy of following the doctor's recommendations.

Guillain-Barré syndrome is a group of acute autoimmune diseases characterized by rapid progression. The period of rapid development is approximately one month. In medicine, this disorder has several names - Landry's palsy or acute idiopathic polyneuritis. The main symptoms are muscle weakness and lack of reflexes, which occur against the background of extensive nerve damage (as a result of an autoimmune process). This means that the human body accepts its own tissues as foreign, and the immune system forms antibodies against the affected nerve sheaths.

Diagnosis is based on a hardware study of the patient and the presence of specific signs on at least one limb. This disorder affects people of any age group, regardless of gender, but it is most often seen in people of middle age from 30 to 50 years, but, nevertheless, it is often observed in children.

Treatment of the disease is carried out only in a hospital, as very often patients require artificial ventilation of the lungs. The prognosis after therapy, rehabilitation and recovery is favorable in half of the cases.

Etiology

In most cases, the reasons for the expression of Guillain-Barré syndrome are unclear, since it refers to autoimmune processes. But experts identify several predisposing factors:

complex course of infectious diseases;
damage to the upper respiratory tract;
infectious nature;
complications from surgery or vaccination;
craniocerebral diseases or injuries resulting in swelling or neoplasms in the brain. That is why the likelihood of the syndrome affecting the human nervous system is high;
genetic predisposition. If one of the close relatives was diagnosed with this disease, then the person automatically falls into the risk zone. For this reason, the disease can manifest itself in a newborn child and school-age children;
infections belonging to the group of viruses.

Experts agree that Guillain-Barré syndrome is expressed during or after the course of the above diseases.

Varieties

Currently, there are several forms of the course of this disorder:

demyelinating - occurs in most cases. It got its name because during the course, an element such as myelin is significantly damaged;
axonal - characterized by a violation of the processes that feed the nerves - axons. The main symptom is weakness in the joints and muscles;
motor-sensory - the flow is similar to the previous form. Signs include weakness and significant numbness of the skin.

A separate type of syndrome is one that affects vision. At the same time, it is quite difficult for a person to move his eyes, his visual acuity is disturbed, and there is unsteadiness while walking.

Depending on the development, Guillain-Barré syndrome is characterized by:

gradual course - weakness increases within a few weeks, after a person ceases to perform elementary functions, for example, hold cutlery while eating or write with a pen. This course is good because a person has time to consult a doctor. The danger lies in untimely treatment and the threat of complications, especially in children or pregnant women;
acute development - the disease develops so quickly that in a day a person can be partially paralyzed. The spread of weakness begins with the lower extremities and gradually passes into the shoulders, back and pelvis. The more it spreads, the more likely it is to become paralyzed.

Symptoms

The main symptom of Guillain-Barré syndrome is rapidly progressive weakness, which stops its development after a month from the first manifestation of other symptoms. It most often strikes lower limbs, and after three weeks of flowing, it moves to the upper ones. First, discomfort is felt by a person in the shin area, after which the feet are affected and at the same time the hands are affected. It is noteworthy that weakness, numbness and tingling are simultaneously manifested in both the lower and upper limbs. In addition, there are the following signs, but they occur individually:

difficulty during swallowing, not only when eating food, but also when taking liquids;
violations of respiratory functions, up to the point that a person cannot breathe on his own;
the occurrence of pain of varying intensity in the back and affected limbs. Such a symptom is difficult to treat;
heart rate disorder, in some it can be very fast, in others it can be slowed down;
paralysis of the muscles of the face;
loss of tendon reflexes;
lack of sensitivity in the feet and hands;
increased sweating;
fluctuations in blood pressure;
possible occurrence of uncontrolled emission of urine;
shaky and unsteady gait;
changes in the volume of the abdomen. This happens because it is difficult for a person to breathe with the help of the diaphragm, and he is forced to use the abdominal cavity;
impaired coordination of movements;
decreased visual acuity - most often there are bifurcation and strabismus.

Such symptoms are inherent in both adults and children and newborns.

Complications

For any person, there is a possibility of death, due to the possible occurrence or complete cardiac arrest. In addition, there is a high probability that paralysis will remain until the end of life. When diagnosing this syndrome in a pregnant woman, there is a risk of miscarriage or death of the fetus in the womb.

Diagnostics

The main task of a specialist during diagnosis is to exclude other diseases of a neurological nature, which may be, and various lesions of the central nervous system. Determination of the diagnosis consists in the following activities:

collection by the doctor of complete information about previous diseases and clarification of the first time of occurrence of unpleasant symptoms;
implementation of a neurological examination, which consists in assessing motor reflexes, sensitivity of the affected limbs, eye movements, gait, as well as the reaction of the heart to a sharp change in body position;
blood test - carried out to detect the presence of antibodies and detect the inflammatory process;
cerebrospinal fluid sampling - for this, a puncture is performed, i.e., a puncture in the lumbar back, during which up to two milliliters of fluid is collected to count blood cells, proteins and the presence of antibodies in it;
daily monitoring of blood pressure;
studies of respiratory functions - using spirometry;
conducting ENMG. This will allow the specialist to assess the passage of the nerve impulse. With this syndrome, it will be slow, as myelin and axon pathology are exposed;
additional consultations with specialists such as an immunologist and an obstetrician-gynecologist.

In addition, another sign to confirm the diagnosis is the presence of weakness and lack of reflexes in more than one limb. After receiving all the diagnostic results, the specialist prescribes the most effective individual therapy tactics.

Treatment

The main goal of the treatment is:

restoration of vital functions;
elimination of symptoms of an autoimmune disease using specific techniques;
the rehabilitation period of the patient;
prevention of complications.

The first thing to do is to place the patient in a hospital, and if necessary, connect him to a lung ventilation device, install a catheter if urine output is disturbed, use a special tube or probe if swallowing is difficult. If the paralysis is clearly expressed, it is necessary to provide proper care - change the position of the human body every two hours, take hygiene measures, feed, monitor the functioning of the intestines and bladder.

Specific treatment is to use:

plasmapheresis, i.e. purification of blood from antibodies - can be carried out from four to six operations, the interval should be one day. Thanks to its use, it is possible to reduce the severity of paralysis. The course of therapy will be different for children and adults;
injections of immunoglobulin (protective antibodies), which were taken from healthy people - are used once a day for five days. Its use improves prognosis.

To eliminate symptoms, the patient is prescribed:

drugs to restore normal heart rhythm;
antibiotics if infection occurs;
heparin - to avoid the occurrence of blood clots;
hormonal medications;
antioxidants - improve metabolism.

Since Guillain-Barré syndrome has a negative effect on the muscles, sometimes a person has to re-learn how to perform elementary movements. For this purpose, rehabilitation methods are used:

a course of therapeutic massage of the affected limbs;
the use of physiotherapy;
taking contrast and relaxing baths that will restore muscle tone. Radon baths are often used;
compress based on wax and paraffin;
performing physical therapy exercises;
a special diet enriched with vitamins and nutrients (potassium, calcium and magnesium).

After the normalization of the patient's state of health, he must be registered with a neurologist. In addition, it will be necessary to undergo preventive examinations in order to identify the prerequisites for a relapse of the disease at an early stage. If the treatment was started in a timely manner, it makes it possible to return the patient to a full, active life.

At the beginning of the 20th century, researchers Barre, Guillain and Strohl described an unknown disease in French army soldiers. The fighters were paralyzed, they did not have tendon reflexes, there was a loss of sensitivity. Scientists examined the cerebrospinal fluid of patients and determined that it contained an increased content of protein, while the number of other cells was absolutely normal. Based on the protein-cell association, as evidenced by the result of the analysis of cerebrospinal fluid, Guillain-Barré syndrome was diagnosed, which differs from other demyelinating diseases of the nervous system by its rapid course and favorable prognosis. The studied soldiers recovered earlier than 2 months later.

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

Then they decided to combine both diseases and give the pathology the same name Landry-Guillain-Barré syndrome, and to this day neuropathologists use the proposed terminology. However, the international classification of diseases has registered only one name: Guillain-Barré syndrome or acute post-infectious polyneuropathy.

Guillain-Barré syndrome, causes

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

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

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

cardiac muscle;
Diaphragm;
Swallowing muscles.

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

Guillain-Barré syndrome, symptoms

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

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

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

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

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

Clinical course of Guillain-Barré syndrome

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

prodromal;
Razgara;
Exodus.

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

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

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

Guillain-Barré syndrome, treatment

With an acute onset, especially when Guillain-Barré syndrome develops in children, first of all, resuscitation measures are provided. Timely connection of the artificial respiration apparatus saves the life of the patient.

A long stay in the intensive care unit requires additional treatment, pressure sores are prevented and infections are combated, including hospital ones.

The uniqueness of Guillain-Barré disease lies in the fact that with adequate mechanical ventilation of the lungs, regeneration of the myelin sheaths occurs without any drug exposure.

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

Guillain-Barré syndrome is currently treated with immunoglobulin infusions. The method is expensive but effective. IN recovery period methods of physiotherapy, physiotherapy exercises and massage are used.

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