Guillain-Barre syndrome (GBS) is an acute inflammatory demyelinating polyradiculoneuropathy of autoimmune etiology. A characteristic sign of the disease is peripheral paralysis and protein—cell dissociation in the cerebrospinal fluid (in most cases). The diagnosis of Guillain-Barre syndrome is established in the presence of increasing weakness and areflexia in more than 1 limb. At the same time, other neurological diseases accompanied by peripheral paresis should be excluded: polio, acute period of stem stroke, toxic lesions of the central nervous system, etc. Treatment of patients with Guillain-Barre syndrome is carried out in a hospital, because the patient may need a ventilator.
ICD 10
G61.0 Guillain-Barre syndrome
General information
Currently, there are four main clinical variants within the framework of GBS:
- The classical form of GBS is acute inflammatory demyelinating polyradioloneuropathy (up to 90% of cases)
- The axonal form of GBS is acute motor axonal neuropathy. A characteristic feature of this form of GBS is an isolated lesion of motor fibers. In acute motor-sensory axonal neuropathy, both motor and sensory fibers are affected (up to 15%)
- Miller-Fisher syndrome is a form of GBS characterized by ophthalmoplegia, cerebellar ataxia and areflexia with mild paresis (up to 3%)
In addition to the above forms of Guillain-Barre syndrome, several other atypical forms of the disease have recently been distinguished — acute sensory neuropathy, acute pandisautonomia, acute cranial polyneuropathy, which are quite rare.
Guillain-Barre syndrome symptoms
The first manifestations of Guillain-Barre syndrome are, as a rule, muscle weakness and / or sensory disorders (feeling of numbness, paresthesia) in the lower extremities, which, after a few hours (days), spread to the upper extremities. In some cases, the disease manifests pain in the muscles of the extremities and the lumbosacral region. Very rarely, the first manifestation is CN lesions (oculomotor disorders, impaired phonation and swallowing). The degree of motor disorders in Guillain-Barre syndrome varies significantly — from minimal muscle weakness to tetraplegia. Paresis is usually symmetrical and more pronounced in the lower extremities.
Hypotension and a significant decrease (or complete absence) are typical tendon reflexes. Respiratory failure develops in 30% of cases. Disorders of surface sensitivity are manifested in the form of mild or moderate hypo- or hyperkinesia of the polyneuritic type. Approximately half of the patients have disorders of deep sensitivity (sometimes up to its complete loss). CN lesions detected in most patients are manifested by paresis of facial muscles and bulbar disorders. Of the vegetative disorders, cardiac arrhythmias, arterial hypertension, sweating disorder, impaired functions of the gastrointestinal tract and pelvic organs (urinary retention) are most often observed.
Diagnosis
When collecting anamnesis, it is necessary to pay attention to the presence of provoking factors, since in more than 80% of cases, the development of GBS is preceded by certain diseases and conditions (transmitted infections of the gastrointestinal tract, upper respiratory tract, vaccination, surgical interventions, intoxication, tumor). Neurological examination is aimed at identifying and assessing the severity of the main symptoms of Guillain-Barre syndrome — sensitive, motor and autonomic disorders.
It is necessary to conduct general clinical studies (urine analysis, blood test), biochemical blood analysis (blood gas composition, serum electrolyte concentration), cerebrospinal fluid studies, serological studies (if infectious etiology of the disease is suspected), as well as electromyography, the results of which are of fundamental importance for confirming the diagnosis and determining the form of GBS. In severe cases of the disease (rapid progression, bulbar disorders), daily monitoring of blood pressure, ECG, pulse oximetry and examination of the function of external respiration (spirometry, peak flowmetry) should be carried out.
To confirm the diagnosis, it is necessary to have progressive muscle weakness in more than one limb and the absence of tendon reflexes (areflexia). The presence of persistent pelvic disorders, pronounced persistent asymmetry of paresis, polymorphonuclear leukocytes, as well as a clear level of sensitivity disorders should raise doubts about the diagnosis of “Guillain-Barre syndrome”. In addition, there are a number of signs that absolutely exclude the diagnosis of GBS, among them: recently suffered diphtheria, symptoms of lead intoxication or evidence of lead intoxication, the presence of exclusively sensory disorders, porphyrin metabolism disorders.
Differential diagnosis
First of all, the Guillain-Barre syndrome is differentiated by a neurologist from other diseases that are also manifested by peripheral paresis (polio), as well as other polyneuropathies. Polyneuropathy in acute intermittent porphyria may resemble Guillain-Barre syndrome, but, as a rule, is accompanied by a variety of psychopathological symptoms (hallucinations, delusions) and pronounced abdominal pain. Symptoms similar to the signs of GBS are possible with extensive strokes of the brain stem with the development of tetraparesis, which in the acute period takes on peripheral features. The main differences between myasthenia gravis and GBS are the variability of symptoms, the absence of sensory disorders, and characteristic changes in tendon reflexes.
Treatment
All patients diagnosed with Guillain-Barre syndrome are subject to hospitalization in a hospital with an intensive care unit and intensive care unit. In approximately 30% of cases of GBS, due to the development of severe respiratory insufficiency, there is a need for a ventilator, the duration of which is determined individually, focusing on VEL, restoration of swallowing and cough reflex. Disconnection from the ventilator is carried out gradually with the obligatory stage of intermittent forced ventilation.
In severe cases with severe paresis, proper care is of particular importance for the prevention of 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 gymnastics, prevention of aspiration. With persistent bradycardia with the threat of asystole development, it may be necessary to install a temporary pacemaker.
Pulse therapy with class G immunoglobulins and plasmapheresis is currently used as a specific therapy for Guillain-Barre syndrome aimed at stopping the autoimmune process. The effectiveness of each of the methods is relatively the same, so their simultaneous use is considered impractical. Membrane plasmapheresis significantly reduces the severity of paresis and the duration of ventilation. As a rule, 4-6 sessions are carried out with an interval of one day. 0.9% sodium chloride solution or dextran is used as replacement media.
It should be remembered about contraindications to plasmapheresis (infections, blood clotting disorders, liver failure), as well as possible complications (electrolyte imbalance, hemolysis, allergic reactions). Class G immunoglobulin, like plasmapheresis, reduces the duration of stay on a ventilator; 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-Barre syndrome is carried out to correct violations of acid-base and water-electrolyte balance, correction of blood pressure, prevention of deep vein thrombosis and thromboembolism. Surgical intervention may be necessary for tracheostomy in case of prolonged ventilation (more than 10 days), as well as gastrostomy for severe and prolonged bulbar disorders.
Forecast
The majority of patients diagnosed with Guillain-Barre syndrome have complete functional recovery within 6-12 months. Persistent residual symptoms persist in approximately 7-15% of cases. The recurrence rate of GBS is about 4%, the mortality rate is 5%. Possible causes of death are respiratory failure, pneumonia or other infections, pulmonary embolism. The probability of a fatal outcome largely depends on the age of the patient: in children under the age of 15, it does not exceed 0.7%, while in patients over 65 it reaches 8%.
Prevention
There are no specific methods for the prevention of Guillain-Barre syndrome. However, the patient should be notified of the ban on vaccinations during the first year from the onset of the disease, since any vaccination can cause a relapse of the disease. Further immunization is allowed, and its necessity must be justified. In addition, Guillain-Barre syndrome, which developed within 6 months after any vaccination, is in itself a contraindication to the use of this vaccine in the future.