Features of treatment of Guillain-Barre syndrome. Guillain-Barre syndrome: signs, diagnosis, treatment - Online diagnosis Barre pain syndrome j a barre

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

Guillain-Barre syndrome (G61.0)

Neurology

General information

Brief description

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

Guillain-Barre syndrome(Guillain-Barrésyndrome) (GBS) is an acute, rapidly progressive autoimmune lesion of the peripheral nervous system, manifested as paresthesia of the limbs, 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, Landry-Guillain-Barré syndrome, Landry-Guillain-Barré-Strohlsyndrome, Landry's syndrome, Landry's ascending paralysis, French polio, etc.
A feature of this disease is a self-limiting, monophasic course with extremely rare relapses.

Correlation of ICD-10 and ICD-9 codes

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

Date of protocol development/revision: 2016

Protocol users: GPs, therapists, resuscitators, neurologists (adults, children).

Level of evidence scale:

A	A high-quality meta-analysis, systematic review of RCTs, or large RCTs with a very low probability (++) of bias, the results of which 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 low (+) risk of bias, the results of which can be generalized to relevant population.
WITH	Cohort or case-control study or controlled trial without randomization with low risk of bias (+). Results that can be generalized to the relevant population or RCTs with very low or low risk of bias (++ or +) whose results cannot be directly generalized to the relevant population.
D	Case series or uncontrolled study or expert opinion.

Classification

Classification

GBS is classified as both a neuroinfection and a post-infectious condition. There are several forms of GBS, differing in the characteristics of the pathological process, the primary point of application of autoimmune aggression (nerve sheath or axonal core), prognosis of recovery, 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 (AMAN);
4) Miller-Fisher syndrome (MFS);
5) acute panautonomic neuropathy (acute panautonomic Guillain-Barre syndrome, acute pandysautonomia);
6) Bickerstaff brainstem encephalitis;
7) pharyngo-cervico-brachial variant;
8) acute cranial polyneuropathy.
There are also options for combining Miller-Fisher syndrome with other forms of Guillain-Barre syndrome (MFS/GBS overlap syndrome).

GBS is also classified according to the severity of the condition depending on the clinical manifestations:
· mild form is characterized by the absence or minimal paresis that does not cause significant difficulties when walking and self-care;
· in moderate cases, a walking disorder occurs that limits the patient’s movement or requires outside help or support;
· in severe cases of the disease, the patient is bedridden and requires constant care, dysphagia is often observed;
· in extremely severe cases, patients require artificial ventilation (ALV) due to weakness of the respiratory muscles.

Neurophysiological criteria for the classification of GBS(R. Hadden, D. Cornblath, R. Hughesetal., 1998).
Group with primary demyelinating lesions:
the presence of at least one of the following signs in at least 2 nerves or two signs in one nerve is necessary if all other nerves are non-excitable and the amplitude of the M-response at the distal point is 10% or more of the lower limit of normal:
· the velocity of propagation of excitation (SPT) is less than 90% of the lower limit of normal, or less than 85% when the amplitude of the M-response at the distal point is less than 50% of the lower limit of normal;
· the distal latency of the M-response exceeds the upper limit of the norm by more than 10%, or by more than 20% if the amplitude of the M-response at the distal point is below the lower limit of the norm;
· presence of 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 normal), and in at least 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 recorded in any of the examined nerves or is present in only one nerve with an amplitude at the distal point more than 10% below the lower limit of normal.

Unspecified group:
· changes identified during stimulation ENMG do not meet the criteria of any of the above groups.

Diagnostics (outpatient clinic)

OUTPATIENT DIAGNOSTICS

Diagnostic criteria:
Complaints:
· Increasing muscle weakness in the arms and/or legs;
Numbness and decreased sensitivity;
· increased sensitivity (tactile, temperature, etc.) in the hands and feet;
· pain in the back, shoulder and pelvic girdle;
· impaired swallowing, both solid food and liquids;
· violation of respiratory functions, up to the absence of spontaneous breathing, due to weakening of the respiratory muscles, weakening of the voice and cough;
· heart rate disorder, in some it can be very rapid, in others it can be slow;
· paralysis of facial muscles;
· increased sweating;
fluctuations in blood pressure;
· uncontrolled emission of urine may occur;
loss of tendon reflexes;
· shaky and uncertain gait, poor coordination of movements;
· changes in the volume of the abdomen, this happens because it is difficult for a person to breathe using the diaphragm, and he is forced to use the abdominal cavity;
· decreased visual acuity - double vision and strabismus most often occur.
Symptoms are common to both adults, children and newborns.

Anamnesis: GBS usually develops 1-3 weeks after an infectious disease (ARVI, influenza, sinusitis, bronchitis, pneumonia, tonsillitis, measles, mumps, diarrhea, etc.).
Neurological symptoms appear suddenly; Most patients experience pain and paresthesia.
When collecting anamnesis, it is important to clarify the following aspects.
Presence of provoking factors. In approximately 80% of cases, the development of Guillain-Barré syndrome is preceded 1-3 weeks by certain diseases or conditions.
· infections of the gastrointestinal tract, upper respiratory tract, can develop after an intestinal infection caused by Campylobacter jejuni, 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 (rabies, tetanus, influenza, etc.);
· surgical interventions or injuries of any localization;
· taking certain medications (thrombolytic drugs, isotretinoin, etc.) or contact with toxic substances;
· sometimes Guillain-Barré syndrome develops against the background of autoimmune (systemic lupus erythematosus) and 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 normal functioning of the body.

Physical examination includes:
· general somatic status: general condition and its severity, body temperature, measurement of the patient’s weight, examination of the skin, breathing, pulse, blood pressure, condition of 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 limb muscle strength;
· study of reflexes - Guillain-Barré syndrome is characterized by areflexia (that is, the absence of most reflexes);
· sensitivity assessment - the presence of skin areas with a feeling of numbness or tingling;
· assessment of pelvic organ function - short-term urinary incontinence is possible;
· assessment of cerebellar function - the presence of unsteadiness in the Romberg position (standing with arms outstretched in front of you and eyes closed), lack of coordination of movements;
· assessment of eye movements - with Guillain-Barré syndrome there may be a complete lack of ability to move the eyes;
· Carrying out autonomic tests - to assess damage to the nerves innervating the heart;
· the heart's reaction to sudden rising from a lying position and physical activity is assessed;
· assessment of swallowing function.

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

Stage 0 of Guillain-Barre syndrome is normal;

Stage 1 - minimal motor impairment;

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

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

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

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

Stage VI - death.

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

0 points - there is no movement in the muscle.

1 point - minimal movements in the muscle, but the patient cannot support the weight of the limb.

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

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

4 points - the patient resists efforts well 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 a typical clinical picture
Acute inflammatory demyelinating polyradiculoneuropathy (typical GBS) (>85%)	Weakness in the extremities with relatively mild sensory disturbances (possibly isolated motor disturbances).
Acute motor axonal polyneuropathy (>5%)	Weakness in the limbs with no changes in sensitivity. Deep reflexes may be preserved. Rapid restoration of functions. Mostly occurs in children.
Acute motor-sensory axonal polyneuropathy (>1%)	Weakness and sensory disturbances in the limbs. Rapid development of severe motor deficits with slow and incomplete recovery. Mostly occurs 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 mild weakness in the limbs. Sensitivity is usually preserved.

Laboratory research:

· OAC - to exclude inflammatory disease of internal organs, accompanied by polyneuropathic syndrome;
· blood sugar test (to exclude diabetic polyneuropathy);
· biochemical blood tests - creatine, urea, AST, ALT, bilirubin (to exclude metabolic polyneuropathy);
· blood tests for gas composition and electrolyte concentrations - biochemical blood tests help to exclude metabolic polyneuropathies;
· PCR of blood 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, Campylobacter jejuni, etc.) - if an infectious etiology of GBS is suspected.

Instrumental studies:
· X-ray of the chest organs - to exclude inflammatory lung disease or associated pulmonary complications when the respiratory muscles are weakened;
· ECG - to identify or exclude vegetative heart rhythm disturbances 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 of the brain *-necessary for differential diagnosis with CNS pathology (acute cerebrovascular accident, 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; if muscles are damaged, a denervation type of ENMG curve is detected, pulse conduction is slow, signs of damage to myelin or axons. Needle electromyography is characterized by the presence of signs of the ongoing denervation-reinnervation process in polyneuropathy. Most often, distal muscles of the upper and lower extremities are examined (for example, tibialis anterior, extensor digitorum communis), and, if necessary, proximal muscles (for example, quadriceps femoris).

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

** N.B.! ENMG is the only instrumental diagnostic method that allows you to confirm lesions of the peripheral nervous system and the diagnosis of GBS, respectively, as well as clarify the nature of pathological changes (demyelinating or axonal) and their prevalence.

The protocol and scope of ENMG studies in patients with GBS depends on the clinical manifestations of the disease:
- for 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 excitation conduction blocks and response dispersion is assessed; the speed of excitation propagation along 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): the latency, shape and amplitude of responses, the magnitude of chronodispersion, and the percentage of dropouts are analyzed.
- in the presence of proximal paresis, additional examination of two short nerves (axillary, musculocutaneous, femoral, etc.) with assessment of motor response parameters (latency, amplitude, shape) is mandatory.
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 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 involving more than one limb in the pathological process;
areflexia or severe hyporeflexia;
· cerebrospinal fluid 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 of which are formulated by the National Institute of Neurological and Communicative Disorders and Stroke (USA):

Mandatory criteria:

progressive motor weakness in more than one limb;

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

· inhibition of reflexes of varying degrees.

Auxiliary criteria for diagnosing the syndrome:

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

2. relative symmetry of the lesion;

3. mild sensory impairment;

4. involvement of cranial nerves in the pathological process;

5. recovery;

6. symptoms of autonomic dysfunction;

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

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

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

10. absence of established causes of damage to peripheral nerves, such as the influence 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;
· presence of psychopathological symptoms - hallucinations, delusions;
· proven poisoning with salts of heavy metals and others.

Diagnostic algorithm:

Diagnostics (hospital)

DIAGNOSTICS AT THE INPATIENT LEVEL

Diagnostic criteria at the hospital level: see outpatient level.

Complaints and anamnesis: see outpatient level.

Physical examination: see outpatient level.

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

Characteristics of Miller Fisher syndrome.







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

Characteristics of acute pandysautonomia.
· the appearance of neurological symptoms 1-2 weeks after a viral or bacterial infection;
· presence of isolated damage to the autonomic nervous system;
· the cardiovascular system is often affected (postural hypotension, arterial hypertension, tachycardia, cardiac arrhythmias);
blurred vision, dry eyes, anhidrosis;
· dysfunction of the gastrointestinal tract (paralytic ileus);
Difficulty urinating, acute urinary retention;
· increased sweating, bluish coloration of the skin of the hands and feet, coldness of the extremities;
· stunning, confusion due to hyponatremia associated with overproduction of antidiuretic hormone. Convulsions may occur when plasma sodium levels are less than 120 mmol/l;
· recovery occurs gradually and often not completely.

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, and compare it with the diagnostic criteria for GBS (WHO; 1993). It is advisable to perform a lumbar puncture with a study of the cerebrospinal fluid, as well as 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 from the Federal State Budgetary Institution “NTsN” of the Russian Academy of Medical Sciences.

Differential diagnostic algorithm for acute flaccid tetraparesis (AFT)

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

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

List of basic laboratory tests:
· blood for immunoglobulins - when planning specific therapy with class G immunoglobulins, it is necessary to determine Ig fractions in the blood, low concentrations of IgA are usually associated with its hereditary deficiency, in such cases there is a high risk of developing anaphylactic shock (immunoglobulin therapy is contraindicated);
· studies of cerebrospinal fluid (cytosis, protein concentration). When analyzing cerebrospinal fluid, the following three indicators are usually considered among the diagnostic criteria confirming GBS:
presence of high protein content,
increased albumin fraction,
· absence of concomitant increase in cytosis.
Additionally, the following diagnostic tests may be recommended to confirm the diagnosis and clarify the characteristics of GBS in a particular case:
· blood test for autoantibodies to gangliosides, with mandatory testing 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 neurofilament heavy chains, tau protein and gliofibrillary acidic protein in blood serum.

Instrumental studies: see outpatient level.

In severe cases of the disease (rapid progression, bulbar disorders), 24-hour monitoring of blood pressure, ECG, pulse oximetry and examination of pulmonary function (spirometry, peak flowmetry), monitoring of pulmonary function (determination of vital capacity of the lungs) should be carried out (in an 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, 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) may 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 with a therapist; · consultation infectious disease specialist	· epidemiological anamnesis; · presence of fever at the onset of the disease; · symptoms from the gastrointestinal tract; asymmetry of the lesion; · absence of objective sensitivity disorders; · high cytotosis in the cerebrospinal fluid; · the diagnosis of poliomyelitis is confirmed using virological or serological studies.
Other polyneuropathies (inflammatory: chronic inflammatory polyneuropathy with acute onset, Sjögren's disease, Churg-Strauss disease, cryoglobulinemic vasculitis; Infectious: HIV-associated, Lyme disease; Toxic: diphtheria, porphyria, medicinal, acute alcohol, heavy metal poisoning Dysmetabolic: polyneuropathy of critical conditions, with renal, liver failure, acute hyperglycemic polyneuropathy)	Acute peripheral paresis	· ENMG; · needle EMG; · Consultant therapist; · infectious disease specialist; · biochemical blood and urine tests	· signs of the current denervation-reinnervation process; · in favor of porphyria is the combination of predominantly motor polyneuropathy with severe abdominal pain, intestinal paresis, arterial hypertension, tachycardia, severe mental changes (from depression to delirium), sleep disturbance, and epileptic seizures. Porphyria has a change in the color of urine, which in the light takes on a reddish tint, and then a rich reddish-brown color
Transverse myelitis. Lesion at the level of the cervical thickening of the spinal cord (C4 - Th2) post-infectious (M.pneumoniae, Schistosoma), post-vaccination, viral (enteroviruses, herpes), myelitis associated with HIV, with demyelinating diseases of the central nervous system, with systemic diseases (systemic red lupus, Sjögren's disease, acute necrotizing vasculitis)	Acute peripheral paresis	MRI of the spinal cord and brain; · ENMG; · cons. therapist; · infectious disease consultant.	· segmental border of sensory impairment; · persistent pelvic disorders; · lack of involvement of facial and respiratory muscles in severe tetraparesis.
Acute violation of spinal circulation, in the vertebero-basilar region. (spinal cord vascular thrombosis, vascular malformation, aneurysm, compression, trauma, spinal cord neoplasm)	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); · The diagnosis is finally confirmed using MRI of the brain/spinal cord.
Myasthenia gravis	Acute peripheral paresis	· ENMG.	· variability of symptoms; · absence of sensory disorders; characteristic changes in tendon reflexes; · the diagnosis is confirmed using EMG (detection of the decrement phenomenon); · positive pharmacological test with proserin.
Botulism	Acute peripheral paresis	· ENMG; · infectious disease consultant.	· relevant epidemiological data, · descending type of spread of paresis, · safety in some cases of tendon reflexes, absence of sensory disorders, no changes in quora.

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Treatment

Drugs (active ingredients) used in treatment

Treatment (outpatient clinic)

OUTPATIENT TREATMENT

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

Non-drugtreatment: No.

Drug treatment:
Symptomatic therapy:
· if blood pressure increases, nifedipine can be prescribed, 10-20 mg sublingually;
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;
· for bradycardia - atropine, adults: intravenous bolus under ECG and blood pressure control - 0.5-1 mg, if necessary, repeat the administration after 3-5 minutes; maximum dose 0.04 mg/kg (3 mg). Children - 10 mcg/kg;
To reduce pain, analgesics and non-steroidal anti-inflammatory drugs are administered:
· ketorolac, orally in a single 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 in 1 administration; usually 30 mg every 6 hours.
Diclofenac, intramuscularly. Single dose - 75 mg, maximum daily dose - 150 mg (with a break between doses 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 every 4 hours. The maximum daily dose for adults should not exceed 1200 mg (no more than 6 tablets in 24 hours).

Algorithm of action in emergency situations: symptomatic therapy measures.

Other types of treatment: No.

· consultation with an infectious disease specialist - establishing or ruling out an infectious disease (infectious mononucleosis, Lyme disease, HIV, etc.);
· consultation with a therapist - establishing or excluding a therapeutic disease (inflammatory disease of internal organs: lungs, kidneys, liver, etc.);
· consultation with an endocrinologist, nephrologist, rheumatologist - if necessary, to exclude somatic pathology.

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

Patient monitoring:
· assessment of the patient’s general condition with a description of the condition of the skin; patient's weight;
· hemodynamic indicators: 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)

DIAGNOSIS AND TREATMENT AT THE EMERGENCY CARE STAGE

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

Swallowing assessment- in case of bulbar palsy, swallowing impairment, to prevent aspiration
· nasogastric tube.

Breathing assessment- possible development of progressive respiratory failure, and not only of the obstructive type due to bulbar palsy, but also with damage to the phrenic nerve (characterized by a paradoxical type of breathing - when inhaling, the anterior abdominal wall collapses) and intercostal nerves.
· tracheal intubation (for further transfer of the patient to mechanical ventilation).

Heart function assessment:
· ECG - decrease and even inversion of the S-T segment, increase in the Q-T interval, cardiac arrest is possible.
During transportation, it is important to take care to maintain airway patency, carefully monitor blood pressure and heart rhythm (tachycardia, orthostatic hypotension, arrhythmia, etc.).

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

Treatment (inpatient)

INPATIENT TREATMENT

Treatment tactics: The main goal of treatment is: restoration of vital functions, elimination of symptoms of an autoimmune disease using specific techniques, rehabilitation period of the patient, prevention of complications. The first thing that needs to be done is to place the patient in a hospital, and, if necessary, connect him to a ventilator, install a catheter in case of difficulty urinating, install a nasogastric tube in case of difficulty swallowing.

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

Drug treatment:
Specific therapy for Guillain-Barré syndrome, aimed at stopping the autoimmune process, currently uses pulse therapy with class G immunoglobulins and plasmapheresis (see paragraph - other types of treatment). The effectiveness of each method is relatively the same, so their simultaneous use is considered inappropriate.
Immunoglobulin class G, like plasmapheresis, reduces the duration of stay on mechanical ventilation; it is administered intravenously daily for 5 days at a dose of 0.4 g/kg. Possible side effects: nausea, headaches, muscle pain, fever.
Symptomatic therapy for Guillain-Barré syndrome is carried out to correct disturbances in the acid-base and water-electrolyte balance, correct blood pressure levels, and prevent deep vein thrombosis and thromboembolism.
Infusion therapy for the correction of acid-base and water-electrolyte imbalances, severe arterial hypotension.
For persistent severe arterial hypertension, antihypertensive drugs (β-blockers or slow calcium channel blockers) are prescribed (see KP Arterial hypertension).
For severe tachycardia, β-blockers (propranolol) are prescribed; for bradycardia, atropine is prescribed (see below).
With the development of intercurrent infections, antibiotic therapy is necessary (broad-spectrum drugs are used).
To prevent 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 the case of neuropathic pain, the drugs of choice are gabapentin, carbamazepine, pregabalin (for adults only!) (see below).

List of essential medicines:.

Drugs	Single dose	Frequency of administration
Immunoglobulin class G	0.4 g/kg i.v.	. 0.4 g/kg/day for 5 days, 1 time per day, 5 days.
gabapentin	300 mg	1 day 300 mg 1 time / day, 2 day 300 mg 2 times / day, 3 day 300 mg 3 times / day, then depending on individual tolerability and effectiveness, the dose can be increased by 300 mg/day every 2-3 days 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 patient's individual response and tolerability, after 3-7 days the dose can be increased to 300 mg per day, and, if necessary, after another 7 days - to a maximum dose of 600 mg per day.

List of additional medicines:.

Drugs	Single dose	Frequency of administration
nifedipine	10 mg	1-2 times under the tongue
Propranalol	10 mg	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
Atropine	0,5-1,0	adults: intravenous bolus under ECG and blood pressure monitoring - 0.5-1 mg, if necessary, repeat the administration after 3-5 minutes; maximum dose 0.04 mg/kg (3 mg). Children - 10 mcg/kg;
Ketorolac	10 mg	orally once in 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 in 1 administration; usually 30 mg every 6 hours. Not used in children.
Diclofenac	75 mg	intramuscularly, single dose 75 mg, maximum daily dose - 150 mg (with a break between doses 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 every 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 necessary for tracheostomy in case of prolonged mechanical ventilation (more than 10 days), as well as gastrostomy for severe and prolonged bulbar disorders.

Other types of treatment:
You should always remember the exceptional importance of a set of rehabilitation measures to prevent complications due to the patient’s immobility and to maintain the functional state of the muscles until a sufficient range of independent movements appears.
The patient needs:
- Physical therapy
- Massage has a beneficial effect on metabolism, which also accelerates nerve growth and reinnervation
- Physiotherapy to prevent the formation of contractures (electrical stimulation, heat therapy, medicinal electrophoresis, etc.).
- Hyperbaric oxygenation.
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 replaced plasma in one session must be at least 40 ml/kg. 0.9% sodium chloride solution or rheopolyglucin are used as replacement media.
You should remember about contraindications to plasmapheresis (infections, bleeding disorders, liver failure), as well as possible complications (electrolyte imbalance, hemolysis, allergic reactions).

Indications for specialist consultation:
· consultation with an infectious disease specialist if necessary (in the absence of a specialist at the pre-hospital level) - establishment or exclusion of a chronic infection (brucellosis, borreliosis, etc.), as well as in the case of confirmation of an infectious agent for correction of etiological therapy;
· consultation with a therapist if necessary (in the absence of a specialist at the pre-hospital level) - 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 with an ICU doctor - treatment of patients with severe forms of Guillain-Barré syndrome is carried out jointly with a doctor in the intensive care unit;
· consultation with a cardiologist - in case of severe cardiovascular disorders (persistent severe arterial hypertension, arrhythmias).

Indications for transfer to the intensive care unit:
· severe and extremely severe degree of neurological disorders;
hemodynamic instability;
· respiratory dysfunction.

Indicators of treatment effectiveness:
· stabilization of the immunological status (quantitative and qualitative composition of IgG in blood and cerebrospinal fluid);
· regression of focal neurological symptoms.

Further management.
After the patient’s health status has normalized, he must be registered with a neurologist. In addition, it will be necessary to undergo preventive examinations in order to identify the preconditions for 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, comprehensive rehabilitation measures are necessary, the plan of which is drawn up individually depending on the severity of residual symptoms (physical therapy, massage, while thermal procedures are contraindicated!).
Patients who have had GBS. should be informed of the need to observe a protective regime for at least 6-12 months after the end of the disease. Physical overload, overheating, hypothermia, excessive insolation, and 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 prescribed heparin subcutaneously at a dose of 5,000 units 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 nerve growth and reinnervation;
· kinesiotherapy has been proven to stimulate reinnervation and restore muscle volume;
· physical therapy to improve strength, to prevent the formation of contractures (electrical stimulation, heat therapy, medicinal electrophoresis);
· rehabilitation to develop daily living skills and the use of adaptive products to help with daily living;
· the patient may require orthopedic aids or other auxiliary methods 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

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

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	erythrocyte sedimentation rate
Ig	immunoglobulin
Heart rate	heart rate
AIDS	acquired immunodeficiency syndrome
EMG	electromyography
ENMG	electroneuromyography
IVIG	normal human immunoglobulin for intravenous administration
GK	glucocorticoids

List of protocol developers with qualification information:
1. Kayshibaeva 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. Tuleutaeva Raikhan Yesenzhanovna, clinical pharmacologist, candidate of medical sciences, professor of RAE, head of the department of pharmacology and evidence-based medicine of Semey State Medical University.

Disclosure of no conflict of interest: No.

List of reviewers:
Dushchanova 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 reviewing the protocol: Review of the protocol 3 years after its publication and from the date of its entry into force or if new methods with a level of evidence are available.

Attached files

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Synonyms: acute demyelinating polyradiculo(neur)opathy, acute post-infectious polyneuropathy, Landry-Guillain-Barré syndrome, obsolete. Landry's ascending paralysis.

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

Often the disease develops immediately after previous infections. In the classic version of the syndrome, ascending (from the legs) tetraparesis (paresis (paralysis) of all four limbs) is observed.

The diagnosis is made based on the analysis of the characteristic clinical picture and is confirmed by examination of the cerebrospinal fluid and electromyographic study (EMG).

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

Along with the fact that Guillain-Barré syndrome is classically presented as a demyelinating polyneuropathy with ascending weakness, called acute inflammatory demyelinating polyneuropathy and accounting for 75–80% of cases, several atypical variants or subtypes of this syndrome, representing a heterogeneous group of immune-dependent peripheral neuropathies: Miller-Fisher syndrome (3 - 5%), acute motor axonal polyneuropathy and acute sensorimotor axonal polyneuropathy (account for 15-20%), and more rare acute sensory polyneuropathy, acute pandysautonomia, acute cranial polyneuropathy, pharyngo-cervico-brachial option. As a rule, these variants are clinically more severe than the main one.

• Epidemiology

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

• ICD-10 code G.61.0

Treatment

• Basic provisions
  • Treatment of Guillain-Barré syndrome includes two components: nonspecific supportive therapy and specific therapy with plasmapheresis or pulse therapy with immunoglobulin G.
  • Due to the possibility of decompensation with severe respiratory failure within several hours, as well as cardiac arrhythmias, it is necessary to treat Guillain-Barré syndrome in the acute phase as an emergency. In cases of the development of acute respiratory failure in a medical institution, it must be possible to carry out long-term artificial ventilation of the lungs.
  • In severe cases with early development of acute respiratory failure, treatment is carried out in an intensive care unit or intensive care unit. Hourly monitoring of vital capacity, blood gases, the content of blood electrolytes, heart rate, blood pressure, and the condition of the bulbar muscles (the appearance and increase of swallowing disorders that do not bring relief from coughing, hoarseness, and speech impairment) is carried out. In case of bulbar paralysis with swallowing disorders, choking, pouring drinks through the nose, the introduction of a nasogastric tube, and often intubation (to prevent aspiration and aspiration pneumonia), is indicated. Tracheal intubation with mechanical ventilation is indicated for the development of respiratory failure, if vital capacity drops below 12 - 15 ml/kg, and for bulbar paralysis and swallowing and speech disorders below 15 - 18 ml/kg. If there is no tendency to restore spontaneous breathing, a tracheostomy is performed within 2 weeks.
  • Corticosteroids are not currently used because they have been proven to be ineffective. They do not improve the outcome of the disease.

• Specific therapy

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

Given that there is a high probability of spontaneous recovery, treatment of patients with mild Guillain-Barré syndrome can be limited to nonspecific and supportive therapy. With moderate severity of the process, and especially with severe disease, specific therapy begins as early as possible.

Treatment with immunoglobulin has some advantages over plasmapheresis, since it is easier and more convenient to use, has significantly fewer side effects, and is easier to tolerate by the patient, and therefore immunoglobulin is the drug of choice in the treatment of Guillain-Barré syndrome.

• Intravenous immunoglobulin pulse therapy Intravenous pulse therapy with immunoglobulin (IgG, drugs - octagam, sandoglobulin, intraglobulin, normal human immunoglobulin) is indicated for patients who are unable to walk more than 5 m without assistance, or for more severe patients (with paralysis, breathing and swallowing disorders) patients with maximum the effectiveness of the drug when starting therapy no later than 2–4 weeks from the onset of the disease. It is administered intravenously at a dose of 0.4 g/kg/day for 5 days (total course dose of 2 g/kg or about 140 g). An alternative administration regimen for the same course dose: 1 g/kg/day in two doses for two days. Its use is limited by its high cost.

• Plasmapheresis Plasmapheresis, prescribed in the disease progression phase (approximately in the first two weeks), almost doubles the recovery process and reduces the residual defect. Prescribed in moderate and severe cases according to a scheme of 4 - 6 sessions every other day, with an exchange of 50 ml/kg per session (at least 35-40 ml of plasma per kg of body weight), in total for the course 200 - 250 ml/kg (at least 160 ml of plasma per 1 kg of body weight per course). In mild cases and the recovery phase, plasmapheresis is not indicated. Plasmapheresis has shown quite high effectiveness when prescribed to seriously ill patients, when therapy is started more than 30 days from the onset of the disease.

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

• Nonspecific therapy and rehabilitation
  • It is necessary to prevent thrombosis of the deep veins of the leg in bedridden patients (especially with paralysis in the legs). Indirect anticoagulants phenylin or warfarin are used orally in doses that stabilize the INR at 2.0, or fraxiparin (nadroparin) 0.3 ml. subcutaneously 1 - 2 times a day, or sulodexide (Wessel Due F) 2 times a day, 1 ampoule (600 LSU) IM for 5 days, then orally 1 caps (250 LSU) 2 times a day . Prevention is carried out until the patient begins to get out of bed. If thrombosis developed before the start of therapy, prophylaxis is carried out according to the same scheme. They also use elastic bandaging of the legs to the middle of the thigh (or use stockings with graduated compression) and raising the legs by 10-15º. Passive and, if possible, active “walking in bed” is indicated with bending the legs, simulating walking for 5 minutes 3-5 times a day.
  • In case of paresis of the facial muscles, measures are taken to protect the cornea: instillation of eye drops, blindfolding at night
  • Prevention of contractures and paralysis. To do this, perform passive exercises 1–2 times a day, ensure the correct position in bed (comfortable bed, supports for the feet), massage the limbs. Subsequently, active physical therapy is included.
  • Prevention of bedsores - change position in bed every 2 hours, wipe the skin with special compounds, use anti-bedsore mattresses.
  • Prevention of pulmonary infection in the form of breathing exercises, mobilization of the patient as early as possible. If the vital capacity of the lungs decreases and the separation of bronchial secretions becomes difficult, massage (effleurage and vibration with simultaneous rotation of the body in a supine position) is recommended every 2 hours during the day.
  • Symptomatic therapy: antiarrhythmic, hypotensive, analgesic. In case of arterial hypotension, a drop in blood pressure (approximately blood pressure 100 - 110/60 - 70 mm Hg and below), colloid or crystalloid solutions are administered intravenously (isotonic solution of sodium chloride, albumin, polyglucin), and in case of insufficiency effect in combination with corticosteroids: prednisolone 120 - 150 mg, dexazone 8 - 12 mg. If these drugs are insufficient, vasopressors are used: dopamine (50 - 200 mg, diluted in 250 ml of isotonic sodium chloride solution and administered at a rate of 6 12 drops/min), or norepinephrine, or mesaton. For moderate pain, simple analgesics and non-steroidal anti-inflammatory drugs are used. For severe pain, Tramal or cabamazepine (Tigretol) or gabapentin (Neurontin) are used, possibly in combination with tricyclic antidepressants (imipramine, amitriptyline, azaphene, etc.).
  • Classes with a speech therapist for the treatment and prevention of speech and swallowing disorders.
  • Rehabilitation includes massage, therapeutic exercises, and physiotherapeutic procedures. Transcutaneous muscle stimulation is performed for muscle pain and paresis of the limbs.

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

General information

Guillain-Barré syndrome is an autoimmune lesion of the peripheral nervous system that can quickly develop muscle weakness, leading to paralysis. It often causes acute flaccid tetraparesis, which reduces the motor activity of the lower and upper extremities. In ICD-10, Guillain-Barré syndrome is designated by code G61.0 and is included in the group of inflammatory polyneuropathies.

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

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

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

Based on severity, the following categories can be distinguished:

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

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

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

Guillain-Barre syndrome can be cured

Causes of Guillain-Barré syndrome

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

Less commonly, the appearance of the syndrome is attributed to previous injuries (especially traumatic brain injuries), complications after operations, the influence of systemic lupus, malignant tumors or HIV.

Another risk group consists of people with a hereditary predisposition. If you have a family with the syndrome, then it is better to take care of yourself - avoid infections and injuries.

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

Symptoms of Guillain-Barré syndrome

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

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

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

• the patient feels muscle pain;
• Over time, weakness appears and the patient loses strength.

As soon as you see these symptoms either in yourself or someone you love, contact your doctor immediately. Every minute lost increases the risk of paralysis and even death.

Complications associated with Guillain-Barré syndrome

The disease has a huge impact on the functioning of the human body. Its occurrence may be associated with the following life disorders:

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

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

Fluctuations in blood pressure may occur

Manifestations of Guillain-Barré syndrome in children

Adults are more prone to developing GBS, especially after forty years. In children, it is much less common, but is characterized by the same symptoms, supplemented by paralysis of the eyes, the absence of some reflexes and uncoordinated muscle function. Guillain-Barré syndrome in children often does not cause an increase in temperature, which complicates the diagnosis and leads to complications.

Diagnosis of Guillain-Barré syndrome

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

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

At the third stage, laboratory tests are carried out. The patient donates blood for biochemical analysis, as well as for the presence of autoantibodies and antibodies to past diseases. A lumbar puncture is often ordered to perform a general analysis of the cerebrospinal fluid.

The patient needs to donate blood for a biochemical analysis

Clinical recommendations for determining Guillain-Barré syndrome involve instrumental diagnostics. The patient may be prescribed electromyography, which shows the speed of signal movement along the nerves, and a neurophysiological study. It tests the functioning of the long nerves (sensory and motor) in the arms and legs. At least four of both are examined. The results of the two methods are compared, and a decision is made to make a diagnosis.

Treatment of Guillain-Barré syndrome

There are two different systems of therapy that perfectly complement each other - symptomatic and specific. The first is to relieve the effects of the disease on the body - helping digestion, caring for the body and eyes, supporting breathing, and controlling heart function. Such care should protect the patient from further deterioration of the condition and complications.

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

1. Treatment of Guillain-Barré syndrome by intravenous administration of immunoglobulin. This drug is especially important for those patients who cannot walk.
2. Plasmapheresis can speed up recovery in moderate to severe cases of illness. For mild forms it is not relevant. Removing large amounts of plasma helps normalize immune function.

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

Plasmapheresis in the treatment of Guillain-Barré syndrome

Recovery after GBS

Guillain-Barré syndrome causes damage to both nerve endings and other tissues in the body. The patient often needs comprehensive rehabilitation, which should restore motor activity and the necessary skills in everyday life. For this, massage, electrophoresis, relaxing baths, contrast showers to increase muscle tone, physiotherapy, therapeutic exercises and much more are usually used. All this will give the patient the opportunity to return to a full life and no longer remember the syndrome.

Preventing the return of the syndrome

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

• refuse vaccination for at least six months;
• do not visit countries where there have been outbreaks of the Zika virus or other dangerous infections;
• visit neurologists and rehabilitation specialists at the clinic regularly;
• Temporary disability may be issued to reduce possible stress at work.

Forecast for the future

The mortality rate for GBS is quite low - only up to 5%. It is caused by the most severe manifestations of the syndrome - weakened breathing, immobilization and associated complications - pneumonia, pulmonary embolism and sepsis. The older the patient, the higher the chance of death.

Most people - about 85% - will make a full recovery and return to a full life. Moreover, only some of them will experience the disease again; the rest will leave it in the past forever.

WHO about the syndrome

The World Health Organization is taking a number of measures to reduce the incidence and increase the number of recoveries. It improves surveillance of epidemics of viral infections, in particular Zika, makes treatment recommendations, and supports GBS research programs around the world.

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

Diseases of an autoimmune nature have not been fully studied to date. They can be diagnosed in both a child and an adult, and manifest themselves in different ways. One such problem is Guillain-Barre 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 disorder is a consequence of a previous infection or prolonged suppression of the body's defenses. It is better to begin treatment of the syndrome as soon as possible after the first signs appear. It is aimed at combating movement disorders and cleansing the blood of abnormal immune complexes.

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:

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

Polyradiculoneuropathy (multiple damage to peripheral nerves, manifested by flaccid paralysis, sensory disturbances, trophic and vegetative-vascular disorders) diffusely affects the body, that is, many nerves, both central and peripheral, are involved in the process. This is what causes such a variety of symptoms.

It is also common to distinguish several forms of the disease. Polyradiculoneuropathy accounts for up to 90% of cases of problem identification and is accompanied by various manifestations of dysfunction of the nervous structures. The axonal type of disorder occurs with selective damage to motor fibers. Miller-Fisher syndrome is another type of disease that is manifested by cerebellar ataxia and lack of reflex reactions.

Causes of pathology

The exact pathogenesis of the formation of Guillain-Barré syndrome is currently unknown. Only the presence of a provoking factor, which is an immunological reaction, has been confirmed. Autopsies of patients in whom this diagnosis was made revealed a large number of macrophages that were involved in damage to the myelin sheath of nerve structures. This explains both the infections preceding the syndrome and the clinical manifestations of the problem.

One of the most studied triggers of the disease is Campylobacter juni, 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, one of the components of the immune response, as well as macrophages is recorded. They react with their own glycolipids that make up the nerve sheath, which causes the manifestation of clinical signs. This pathogenesis explains the success of methods based on replacing patient blood plasma.

Since the disease is of an autoimmune nature, the factors influencing its occurrence are in one way or another related to the work of the body’s defenses.

1. Various infections, both bacterial and viral, provoke the development of polyneuritis. Normally, antibodies are formed that are aimed at fighting the pathogen, but in Guillain-Barre syndrome, these compounds also attack healthy cells of the nervous system, causing the development of the clinical picture. Moreover, one of the reasons also indirectly related to the penetration of a foreign agent into the body is vaccination. The greatest danger is the manifestation of polyneuritis in HIV-positive patients.
2. Damage to nerve structures, such as traumatic brain injury or disruption of the integrity of peripheral connections, provokes 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 deficits.
3. The significance of genetic predisposition does not have precise medical evidence, but the presence in the patient’s family history of cases of autoimmune polyneuritis increases the risk of its diagnosis in the future.
4. Inhibition of the natural protective reaction associated with surgical interventions, a course of taking corticosteroid drugs, as well as chemotherapy treatment increases the risk of developing the disease.

Diagnostics

Identifying Guillain-Barré syndrome is usually not difficult. A careful history and duration of clinical manifestations, as well as previous infectious problems, 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 nonspecific urine and blood tests, as well as electromyography, which can confirm the presence of abnormalities 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 due to polio, and stroke. This may require a number of specific tests, as well as photographs 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 symptoms, since the further prognosis depends on this.

Therapeutic measures

The basis of treatment for Guillain-Barré syndrome is stopping autoimmune processes and maintaining 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 disruption 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 performed, which involves installing a special tube for artificial ventilation of the lungs. This procedure is only possible in a hospital setting and requires the patient to be admitted to the intensive care unit.

Adequate 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

The basis of drug treatment is the use of class G immunoglobulins. Their use improves the outcome of the disease and allows the restoration of spontaneous breathing in patients undergoing artificial ventilation. Side effects from the administration of the drug are rare.

An effective method is plasmapheresis - replacing 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 involves 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 find it quite difficult to feed. If a person does not receive adequate nutrition, a gastrostomy tube is indicated. It is a tube installed in the stomach. Surgical treatment is in many cases associated with a poor prognosis and a long period of rehabilitation.

Symptomatic therapy

This type of treatment is aimed at restoring salt balance through intravenous infusions of solutions. For severe arterial hypertension and tachycardia, specific drugs are used, for example, adrenergic blockers. Often, Guillain-Barré syndrome is also associated with pain, which is relieved 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. The mortality rate of the disease is low, but the percentage of complications is high. After the patient is discharged from the hospital, he requires long-term assistance to restore a normal lifestyle. The prognosis is also related to the accuracy of compliance with the doctor’s recommendations.

Guillain-Barré syndrome is one of the most severe neurological diseases, which in every third patient during the height of the disease requires treatment in the intensive care unit. The term refers to rapidly progressive neuropathy, characterized by flaccid paralysis in the symmetrical muscles of the limbs with sensory and autonomic disturbances. The condition develops acutely, usually after colds and other infections. However, with adequate treatment, complete recovery is possible.

Reasons

Guillain-Barre disease is usually classified as an autoimmune disease. Having coped with the infection, the human immune system does not recognize this and begins to attack its own body, in particular the nervous tissue. Cells of the immune system produce antibodies that lead to demyelination, that is, damage to the myelin sheath of the nerves. As a result of autoimmune processes, axons, the processes involved in the innervation of muscles and internal organs, can also be damaged.

The first signs of the disease are recorded one to three weeks after such infectious diseases as:

• Viral enteritis.
• Respiratory infections (ARVI).
• Cytomegalovirus infection.
• Infectious mononucleosis.

Signs of the development of the syndrome appear much less frequently after:

• Operations.
• Injury
• For systemic lupus erythematosus.
• In HIV carriers.
• For malignant neoplasms.

Medical studies have not confirmed the connection between the occurrence of Guillain-Barré syndrome and influenza vaccination.

Species

Guillain-Barré syndrome is usually divided into two types - demyelinating and axonal; the first variant of damage to peripheral nerves is more common.

• Demyelinating. Only myelin sheaths are involved in the pathological process; destruction of axon cylinders is not detected. This leads to a slowdown in the speed of impulse conduction, which provokes the development of reversible paralysis. Pathological changes affect the anterior, less often the posterior roots of the spinal cord; damage to other parts of the central nervous system is also possible. The demyelinating type is considered a classic variant of the syndrome.
• In the axonal variant, the axial cylinders of the axons are also affected, which leads to the development of severe paresis and paralysis. The axonal type of polyneuropathy is considered more severe, after which motor functions are not fully restored.

Guillain-Barré syndrome occurs with self-limiting development of autoimmune processes. This means that sooner or later the immune cells stop their attack and the body begins to recover even in the absence of specific treatment.

However, in any case, the patient requires medical supervision and, possibly, an intensive course of supportive therapy - ventilation, tube feeding, prevention of secondary infectious infection, etc.

Symptoms

If after an infection a person does not feel recovered, but notices the following symptoms:

• low-grade fever;
• tingling and goosebumps in the fingertips;
• muscle pain of different localization;
• growing weakness

This is a reason to immediately consult a doctor. The disease usually develops quickly and there is no time to waste.

The first symptoms of the disease include:

• Tetraparesis that grows over several days is weakness in the lower and upper extremities. Tetraparesis is usually symmetrical; examination reveals sluggish tendon reflexes and low muscle tone.
• In the first days, weakness is noted only in the legs - the sick person notices that it is more difficult for him to climb stairs.
• Chilliness of the extremities, as well as sweating, may bother you.

Sensory disorders occur with a decrease or increase in sensitivity in the distal parts of the arms and legs. The pathological process may involve the muscles of the neck, respiratory muscles, and, less commonly, the muscles of the eyes.

In case of severe damage, swallowing and breathing dysfunction is observed; a patient with similar symptoms requires intensive care, including tracheal intubation. The maximum development of all signs is observed in the third week of the disease. In some forms of the disease, atypical symptoms are also detected:

• Increased blood pressure.
• Arrhythmias.
• Urinary retention.
• Paresis of the facial muscles.

Autonomic disorders can lead to severe arrhythmias and cardiac arrest, which is often the main cause of death.

An increase in symptoms is observed over two weeks, then the disease enters a stabilization phase that lasts up to 4 weeks. The recovery phase lasts on average from one to two months; in some patients, it is possible to achieve normalization of functions only after one to two years.

The disease can be suspected already by questioning and examining the patient. Guillain-Barré syndrome is characterized by symmetrical damage to the extremities and preservation of the function of the pelvic organs. Of course, there are also atypical signs of the disease, so for differential diagnosis it is necessary to conduct a number of studies.

• Electromyography – determination of the speed of impulse transmission along nerve fibers.
• A spinal tap can detect protein in the cerebrospinal fluid. Its content increases a week after the onset of the disease and reaches its peak by the end of the first month of the disease.
• EGC can detect arrhythmias.
• Blood tests show an increase in ESR and white blood cell count without other signs of infection.

Confirmation of the diagnosis is supported by the rate of increase in symptoms (no more than 4 weeks) and the recovery period lasting up to two months. The disease must be differentiated from:

• Spinal cord tumor.
• Poliomyelitis.
• Botulism.
• Diphtheria polyneuropathy.
• Intoxication with salts of heavy metals.

In many cases, the outcome of the disease depends on correct and early diagnosis.

Treatment

Treatment of Guillain-Barré syndrome is divided into two complementary types: nonspecific and specific therapy. Treatment of patients with acute development of symptoms, respiratory dysfunction, severe cardiac arrhythmias begins with nonspecific therapy. The patient is admitted to the intensive care unit. In the phase of increasing symptoms, continuous monitoring of respiratory function and cardiac activity is carried out.

Specific therapy includes the administration of immunoglobulin and plasmapheresis.

• Immunoglobulin is prescribed intravenously. This is especially necessary for those patients who cannot move without assistance or have difficulty swallowing and breathing.
• Plasmapheresis is prescribed for moderate to severe disease. Its use significantly speeds up recovery time and prevents the development of residual effects. In mild cases of the disease, plasmapheresis is not used.
• For arrhythmias, increased blood pressure and other autonomic disorders, symptomatic therapy is used.

In case of paralysis, bedsores and pneumonia are prevented by turning the patient over, treating the body, and performing a massage.

During the rehabilitation period, it is necessary to use sets of physical exercises, physiotherapy, and massage courses. If you have a speech disorder, sessions with a speech therapist are necessary.

Prevention

There is no specific prevention of the disease. Doctors can only recommend treating all infectious diseases at the very beginning of their development, this will reduce the negative impact of pathogens on the nervous system.

Patients with previous Guillain-Barre syndrome should refrain from any vaccination for at least six months. Relapse of the disease can occur after any other infectious disease, so it is necessary to avoid possible sites of infection.

Forecast

In almost 80% of cases, lost functions are completely restored; some patients may experience minor motor impairments. In approximately 3% of cases, patients become disabled. Mortality is due to the lack of adequate therapy for the development of arrhythmias and heart failure, therefore all patients with the syndrome should be under the supervision of health workers at the height of the disease.