The Guillain-Barré Syndrome

Source: By Deutsche Welle

The Peruvian government has recently declared a state of national emergency for up to three months, due to a spike in the number of cases of a rare neurological disorder called Guillain-Barré Syndrome.

The disorder, which affects the body’s nervous system, is characterized by muscle weakness and breathing difficulties, and can even lead to total paralysis in extreme situations. Back in 2019, Peru faced a similar problem following an outbreak of a bacterial infection called campylobacter.

What is Guillain-Barré Syndrome?

Guillain-Barré Syndrome is a rare neurological disorder where the body’s immune system — which normally protects it from infections and other foreign bodies — mistakenly attacks its own peripheral nerve cells. More specifically, the myelin sheath — an insulating layer of fat and protein that surrounds the nerve cells — becomes inflamed.

The myelin sheath enables signals to pass through the nerve tracts at breakneck speed under normal conditions. If the sheath is inflamed, the nerves can hardly transport stimuli.

Simply put, a person with this syndrome will have difficulty speaking, walking, swallowing, excreting or performing other normal functions of the body. The condition can get progressively worse. Thus, the peripheral nerves— the nerves that branch out from the brain and the spinal cord — get damaged as a result and the muscles can become weak or paralyzed.

The first symptoms include a tingling sensation in the body’s extremeties, weakness in the legs that spreads to the upper body, difficulty in facial movements, unsteady walking or inability to walk, pain and, in severe cases, paralysis.

What causes Guillain-Barré Syndrome?

The exact reasons for Guillain-Barré Syndrome are not yet understood. However, it often develops shortly after a person gets an infectious disease. Rarely, vaccinations can cause it. Guillain-Barré Syndrome, or GBS, also was linked to the cytomegalovirus, Epstein Barr virus, Zika virus and even the COVID-19 pandemic.

Why does this happen? Scientists say that our immune system is highly specialized to recognize foreign substances such as viruses, bacteria and fungi. It produces proteins called antibodies that bind to the surface structures of pathogens while building up an immune response against them.

In an autoimmune disease like Guillain-Barré Syndrome, the invaders camouflage themselves with a surface that mimics the body’s own structures. “For example, the surface structures of the bacterium Campylobacter look very similar to the myelin sheath,” explained immunologist Julian Zimmermann.

So the antibodies also target the body’s own cells and structures as foreign bodies and attach themselves to the surface. This results in a cascade of reactions. The exact nature of these interactions in autoimmune diseases are not yet known.

Occasionally vaccinations can also cause GBS. This is because vaccines tend to have similar weakend or inactive structures akin to the pathogens they protect against. The body’s immune system then triggers an immune response.

Is Guillain-Barré Syndrome curable?

The condition of the patient tends to worsen for up to two weeks after the onset of the disease. At week four, the symptoms plateau, after which recovery begins. The recovery can extend from anywhere between six to 12 months and occasionally up to three years.

Currently, there is no certain cure for Guillain-Barré Syndrome. The paralysis not only affects the legs and arms, but also important parts of the nervous system that regulate breathing, blood pressure and heartbeat.

To prevent this from happening, doctors continuously monitor the patient’s vital signs and, in case of an emergency, put them on a ventilator. There are also two treatments that can help recovery and reduce the severity of the disease.

The first is plasma exchange or plasmapheresis. The plasma or the liquid part of the blood is removed and separated from the blood cells, inducing new plasma production to make up for the loss. This treatment is aimed at removing the antibodies which are attacking the peripheral nerves.

The second available therapy is called immunoglobin therapy, where healthy antibodies from blood donors are injected intravenously. The damaged antibodies contributing to GBS are then blocked by the high doses of the immunoglobulins. Apart from this, physical therapy might also be useful in alleviating pain.

Why is this happening in Peru?

There are no reports on the current scenario suggesting that this outbreak of GBS cases are being triggered by another infection. The last known outbreak was in 2019. The country was also struggling with the worst dengue outbreak in its recorded history, this year.

A surge of GBS cases following a wave of Zika virus infections was also observed in the French Polynesia between 2012-2014.

Celebrities who have suffered from GBS

Markus Babbel, a former international football player from Germany, lost almost an entire year of his career after contracting GBS. Babbel’s case of GBS followed an infection with the Epstein-Barr Virus.

Some neurologists and historians believe that Franklin D. Roosevelt, the 32nd President of the United States, contracted GBS in 1921. And Vicente Fernández Gómez, a Mexican cultural icon who recorded more than 100 albums, also had GBS.