Guillain-Barré Syndrome: when the immune system attacks peripheral nerves

Today we are going to talk about one of those diseases classified as rare, for which there is, as yet, no solution. It is one of the diseases we are working on and researching at Oligofastx: Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is a neurological disorder in which the body’s immune system attacks the peripheral nervous system, causing various symptoms such as numbness, tingling and muscle weakness that can progress to paralysis.

GBS is considered a rare disease with an incidence that varies from 0.9 to 1.9 cases per 100,000 inhabitants per year, mainly in adult men.

Although its origin is still not very clear, it seems to be caused by an autoimmune reaction. When it occurs, the body’s immune system attacks either the myelin sheath, which surrounds many nerves and allows nerve impulses to travel fast, or the part of the nerve that sends messages, called the axon.

In two-thirds of the patients who suffer from it, the syndrome is triggered in response to a mild infection, such as those caused by Campylobacter, mononucleosis or another viral infection. Cases have also appeared in patients who have been infected by Zika virus or after COVID-19.

Progressive but rapid symptoms

The first symptoms are usually detected by weakness in both legs progressing to the arms, or in the opposite direction: starting in the arms or head and progressing downwards. This weakness is accompanied by a tingling sensation or loss of sensation, as well as a decrease or absence of reflexes.

Its evolution is very rapid. So much so that three to four weeks after onset, the symptoms may have become quite severe, even requiring mechanical ventilation due to the involvement of the muscles that control breathing.

Some patients (possibly with a variant form) have significant, potentially fatal, autonomic dysfunction resulting in blood pressure fluctuations, inappropriate ADH secretion, cardiac arrhythmias, gastrointestinal stasis, urinary retention and pupillary changes.

An unusual variant (Fisher variant or Miller-Fisher syndrome) may cause only ophthalmoparesis, ataxia and areflexia.

Diagnosis and treatment

The syndrome is diagnosed by electrodiagnostic tests and cerebrospinal fluid analysis, although the diagnosis is primarily clinical.

As such, there is no treatment for the disease. Most patients improve considerably over a period of months, but about 30% of adults and even more children have some residual weakness at 3 years, and 2-5% develop chronic inflammatory demyelinating demyelinating polyneuropathy.

However, intensive symptomatic care is the key to recovery.



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