Guillain Syndrome - Barre

1-3 weeks before the onset of the disease in most patients, signs of infection of the gastrointestinal tract or respiratory tract are noted. Most often - after enteritis caused by Campylobacter jejuni (the risk of developing GBS increases by about 100 times) ^{[1] [2] [3]} . It can also be an infection caused by herpes viruses ( cytomegalovirus , Epstein-Barr virus , etc.), mycoplasmas , Haemophilus influenzae , etc. In particular, Guillain-Barré syndrome can be a manifestation of HIV infection ^[4] . Probably, the transmitted infection triggers an autoimmune reaction ( the body's immune system affects its own cells). Vaccinations , surgical interventions, and injuries of peripheral nerves can play the same role. An autoimmune reaction against Schwann cell and myelin antigens leads to edema , lymphocytic infiltration and segmental demyelination of the roots of the spinal and cranial nerves . Less commonly, peripheral nerve axon antigens are exposed (with the axonal version of the syndrome).

This syndrome may be one of the side effects of pembrolizumab (Kitruda®), a drug from the cancer immunotherapy group ^[5] .

The following clinical options for GBS are distinguished:

• Acute inflammatory demyelinating polyneuropathy is the most common, classic form.
• Acute motor axonal neuropathy.
• Acute motor-sensory axonal neuropathy.
• Charles Miller Fisher Syndrome (Charles Miller Fisher, Canadian neurologist (1913-2012), described the syndrome in 1956) is a combination of areflexia , cerebellar ataxia , ophthalmoplegia with mild paresis.

The disease is characterized by relatively symmetrical muscle weakness (flaccid paresis ), which typically begins in the proximal sections of the leg muscles and spreads to the hands after a few hours or days. Often weakness is accompanied by paresthesias of the toes and hands ^[6] . Sometimes weakness primarily occurs in the arms or at the same time in the arms and legs ^[7] . The protein content in the cerebrospinal fluid increases (starting from the 2nd week of the disease). In severe cases, paralysis of the respiratory and cranial muscles, mainly facial and bulbar, occurs. There are frequent pains in the back, shoulder and pelvic girdle, sometimes radiating along the roots, symptoms of tension. Patients, especially those with concomitant diabetes mellitus, are prone to developing pressure sores. Pronounced autonomic disorders are often noted: increase or decrease in blood pressure, orthostatic hypotension , sinus tachycardia , bradyarrhythmia , transient urinary retention. Intubation or suction of mucus can provoke a sharp bradycardia, collapse and even cardiac arrest. Having reached a peak, the symptoms stabilize (the plateau phase lasts 2-4 weeks), and then recovery begins, which can last from several weeks to 1-2 years. Death is possible from respiratory failure associated with paralysis of the respiratory and / or bulbar centers, pneumonia , pulmonary embolism , cardiac arrest, sepsis , but thanks to modern intensive care methods, especially mechanical ventilation , mortality in the last decade has decreased to 5%.

Even in mild cases, Guillain-Barré syndrome in the acute phase should be treated as an emergency due to the danger of rapid development of severe respiratory failure or heart rhythm disturbance. Urgent hospitalization of patients in the intensive care unit is required . In the progression phase, hourly monitoring of the patient's condition with an assessment of respiratory function, heart rate, blood pressure , condition of bulbar muscles, pelvic functions. Early signs of respiratory failure: weak voice, the need to pause for inspiration during a conversation, sweat on the forehead and tachycardia during forced breathing, weakening cough. With bulbar paralysis, intubation and administration of a nasogastric tube are necessary. Plasmapheresis and immunoglobulin intravenously, capable of accelerating recovery and reducing residual defect, are especially useful in the progression phase (usually in the first 2-3 weeks from the onset of the disease). Corticosteroids do not improve the outcome of the disease. In order to prevent deep vein thrombosis of the lower leg (with plegia in the legs), small doses of heparin or low molecular weight heparin (fraksiparin) are prescribed. With paresis of facial muscles, measures are necessary to protect the cornea (instillation of eye drops, dressing for the night). Early rehabilitation measures, including massage , therapeutic exercises , other physiotherapeutic procedures ( paraffin baths, magnetotherapy, radon and hydrogen sulfide baths, electrical stimulation , etc.) are important.

Full recovery - in 80% of cases , 15% patients expressed pronounced residual paralysis causing disability . In 2-5%, relapse develops and a chronic recurrent polyneuropathy forms.

• Guillain-Barré Syndrome. Abstract of the Doctor. Medical newspaper. - 2001.-No.6