Guillain-Barré syndrome (pronounced gee-YAH-buh-RAY) is a rare autoimmune disorder that has been getting attention recently because of its potential connection with the coronavirus. In a letter to the New England Journal of Medicine, three hospitals in Northern Italy reported that five patients with COVID-19 presented with symptoms consistent with GBS. Now many people are curious about this condition, which is known to cause muscle weakness and paralysis of the extremities.
TODAY spoke to Dr. Peter Donofrio, a professor of neurology at Vanderbilt University Medical Center in Nashville, for more insight into the causes, symptoms and treatments of this disorder, which typically only develops in about 3,000 to 6,000 people annually in the U.S., according to the Centers for Disease Control and Prevention.
Donofrio, who is also a chairman of the GBS/CIDP Foundation International, noted that there are only about 15 reported cases in the world of people developing GBS after testing positive for COVID-19. One of these cases is multiple Grammy-winning singer and composer Christopher Cross. On Instagram, he shared his experience with the disease that has left him temporarily in a wheelchair.
“GBS resulted in a paralysis of my legs, part of my face and a numbness in my fingertips,” Cross wrote. “It has been a terribly difficult situation, but I’ve been fortunate to have excellent medical care and I’m slowly making progress. I’m working with a physical therapist to build strength in my muscles and eventually walk.”
What is Guillain-Barré syndrome?
According to Donofrio and the National Institute of Neurological Disorders and Stroke (NINDS), GBS is a serious neurological condition in which antibodies develop in the bloodstream and wrongly attack part of the peripheral nervous system — a dense network that connects the brain and spinal cord with the rest of the body.
People with GBS will often first feel a tingling sensation in their feet that will move up the legs and into the hands. Some feel it in their back — or in rare instances, their face. Numbness or pain usually follows, then weakness on both sides of the body. The intensity of the symptoms may progress over the course of hours, days or weeks, culminating in a symmetric paralysis of the arms and legs, which can range from mild to more severe — and in the most severe cases GBS can be life threatening.
GBS usually requires individuals to be hospitalized to undergo treatment. Donofrio said most people will recover in a few weeks or months. He added that the majority of people “reach a nadir of the illness at four weeks” and then improve soon after. Though NINDS noted that recovery for some can take time, it could "as little as a few weeks up to a few years."
What is the cause of Guillain-Barré syndrome?
While the cause of GBS remains largely unknown, Donofrio noted that about 70% of individuals have a bacterial or viral infection that later leads to GBS. According to the CDC, the disease can affect any age group, though in the U.S. it is more common in men and adults over age 50. Experts don't believe that the disease is hereditary or contagious.
There are a few things that are known to trigger GBS: According to the CDC, roughly two-thirds of people with GBS had diarrhea or a respiratory illness in the weeks leading up to developing symptoms. A bacterial foodborne infection of Campylobacter jejuni, which causes diarrhea, is believed to be one of the more common risk factors for GBS. The disorder can also be triggered by numerous viral infections like the flu, Epstein Barr, Zika and others. In some very rare instances, people have developed GBS in the days or weeks after receiving a vaccine.
“Guillain-Barré syndrome is overwhelmingly more likely to occur from the natural flu than any vaccination,” Donofrio said.
What are early signs and symptoms of Guillain-Barré syndrome?
Along with tingling and muscle weakness, Donofrio and NINDS outlined these early signs and symptoms:
- Eye muscles and vision difficulties
- Trouble swallowing, chewing or speaking
- Weakness of the the feet or ankles
- Poor balance
- Pain in the body that can be severe, especially at night
- Problems with coordination and unsteadiness
- Abnormal heart beat or blood pressure
- Digestive problems or bladder control issues
In more rare instances, Donofrio said 30% of patients develop a weakness of the diaphragm and have to be intubated and placed on a ventilator.
Even though GBS is categorized as a rare disease, Donofrio said that GBS is widely lectured on in medical school and tends to have more identifiable symptoms than other rare ailments.
“So if you develop tingling in your feet or hands, and you mention to the doctor that a week earlier you had had a cold, or pneumonia or bronchitis, or something like that, the two of them should make you think of development of Guillain-Barré syndrome,” he said.
What is the link between Guillain-Barré syndrome and COVID-19?
Experts are still unsure if there is a connection between GBS and COVID-19; however, in a case report published this month in the Journal of Clinical Neuroscience, researchers concluded that GBS should be considered as a possible "neurological complication of infection with COVID-19.”
Donofrio emphasized that people are overwhelmingly more likely to develop GBS from the common flu or another viral or bacterial infection.
“The COVID-19 virus is pervasive,” he said. “As you know, it has sickened many, many people with this viral infection. We don't know if the incidence of Guillain-Barré after COVID-19 is any higher than it is after any other viral illness."
How is Guillain-Barré syndrome treated?
As of now, there is no known cure. However, there are two immediate treatments to address the antibodies in the bloodstream attacking the peripheral nervous system. The first is intravenous immunoglobulin therapy, which involves periodic injections of immunoglobulins — naturally-occuring proteins the immune system employs to fend off dangerous organisms.
The second treatment is plasma exchange. Donofrio likened this method to dialysis.
“It's similar to dialysis, where they take out your blood, they separate it out, and then give you back your red blood cells, white blood cells and your platelets,” he said. “So it's removing the antibodies in the bloodstream that are attacking the peripheral nervous system.”
Once patients begin to improve, a NINDS report says individuals undergo rehabilitative care that aids in restoring pre-illness strength levels.