Guillain-Barré Syndrome (GBS)

What is Guillain-Barré Syndrome (GBS)?

Guillain-Barré Syndrome (GBS), or acute inflammatory polyradiculitis, is a rare autoimmune disease of the peripheral nervous system, i.e. a disease that affects the outgoing roots from the spinal cord and peripheral nerves, without affecting the spinal cord and brain. It was named in honour of the neurologists who first described it in the early 20th century.

In GBS, the immune system creates antibodies which are directed against the sheath of both motor and sensory nerves, i.e. myelin and/or neurons. As a result, this attack causes nerve inflammation, nerve dysfunction, and sensory and motor symptoms.

The cause of the onset of the syndrome is not always clear. The production of autoantibodies is often triggered by an infection, with the syndrome following 2 to 3 weeks after the onset of symptoms of the infection. Potentially, all viral and microbial infections can trigger the syndrome. There is a greater correlation with infections of the gastrointestinal tract and the same as those caused by Campylobacter jejuni, where the clinical picture is usually more severe. Other viral infections that have been implicated are mainly influenza, Epstein-Barr, and cytomegalovirus.

Clinical picture and symptoms

The syndrome usually starts with a symmetrical feeling of numbness or pain in the upper and lower limbs, starting peripherally and ascending.

Muscle weakness with the same ascending course is present, resulting in impaired gait and use of the upper limbs. Other symptoms may include paresis of the facial nerve, often bilaterally, diplopia, and ataxia. In severe syndrome, the muscles of breathing and swallowing may also be affected, resulting in dysphagia and dyspnoea, and the patient may even require mechanical ventilatory support and transfer to an intensive care unit.

In addition, disorders of the autonomic nervous system often coexist with disturbances of the arterial and heart rhythm and orthocystic disorders. Depending on the type of syndrome, different symptoms may be present.

Diagnosis

GBS is a disease that requires early diagnosis and treatment as it can be potentially fatal if left untreated. After the initial clinical suspicion, the diagnosis is established by clinical examination, where symmetrical usually muscle weakness with accompanying abolition of tendon reflexes is noted. This is followed by laboratory testing with electromyography and electroneurography with abnormal motor and sensory conduction velocity and lumbar puncture with demonstration of high leukocyte count without the presence of abnormal cells (leukocytic bifurcation). Specific antibody testing is an additional tool for the diagnosis and determination of the type of GBS.

Treatment & Therapy

Treatment should begin immediately after diagnosis. Treatment is aimed at counteracting the immune stimulation. Available treatments are intravenous immunoglobulin for 3 to 5 days depending on the severity of the disease and plasmapheresis where autoantibodies are removed to reduce the severity and speed up the recovery of the syndrome: In addition to the specific treatments there is systematic physiotherapy, supportive care of the patient and management of complications, with the main focus on the treatment of autonomic disorders and respiratory support.

Prognosis

The majority of patients recover completely or with minimal residuals. The acute stage usually lasts 1-4 weeks with stabilisation and partial recovery following in the next period. Recovery is often completed in 3-6 months, although it may extend up to 2 years, especially in severe cases in which residual neurological involvement may remain. A small proportion (<5%) may experience serious complications or death without early treatment.

In conclusion

GBS is a rare but serious autoimmune syndrome often triggered by infections. Early diagnosis and treatment are crucial to avoid serious complications, including death.