How Does Guillain-Barré Syndrome Happen?
November 09, 2021
Guillain-Barré syndrome (GBS) is a rare neurological disorder in which people’s immune system attacks their own peripheral nervous system. According to the National Institute of Health’s National Institute of Neurological Disorders and Stroke, GBS is estimated to affect about one in 100,000 people each year.
What causes GBS is not known, says Yelena Roshchina, D.O., a pediatric neurologist at Joseph M. Sanzari Children’s Hospitalat Hackensack University Medical Center. “It’s just really random,” she says.
How Does It Happen?
It’s not genetic or contagious. Typically, GBS will start within a few days or weeks of a respiratory or gastrointestinal infection, she says. What appears to happen is the immune response to a bacterial or viral infection or (very rarely) vaccination triggers a more aggressive response that causes the immune system to attack the peripheral nerves.
One theory is that part of a person’s immune system mistakenly perceives the sheathing around nerves—a fatty covering called myelin—as foreign to the body. To counter this “threat,” the immune system produces antibodies that attack the myelin, which leads to the nerve damage that causes muscle weakness, paralysis and, in some cases, nerve pain, says Ariel Sherbany, M.D., Ph.D., a pediatric neurologist at the Children’s Hospital.
What Are the Signs?
GBS affects all age groups, says Dr. Sherbany, and typically presents as weakness and loss of reflexes in the legs. Mild cases may resolve on their own, but severe cases can lead to paralysis of the respiratory muscles, which can be a life-threatening situation.
Common symptoms of GBS include:
- Tingling in the feet or hands
- Pain (particularly in children), which sometimes precedes weakness
- Weakness on both sides of the body (frequently noticed as having trouble climbing stairs or, especially in children, difficulty walking)
- Unsteadiness or coordination problems
- Difficulty swallowing, speaking, chewing or moving the facial muscles
- Digestive and/or bladder control problems
How Is It Diagnosed?
In addition to a thorough neurological assessment, which includes testing muscle strength and reflexes in the limbs that are experiencing weakness, key diagnostics are an MRI and a spinal tap (also called a lumbar puncture) to get a sample of spinal fluid. Those with GBS tend to have more protein than usual in their spinal fluid.
How Is It Treated?
Generally, if diagnosed early, GBS is treatable with therapies such as immunoglobulin (IVIg) infusions and physical therapy, Dr. Sherbany says, and frequently may completely resolve.
“The goal of treatment is to prevent things from getting worse,” he says. “IVIg has been shown to shorten the length of a period of disability, and also may mean avoiding the need for a ventilator.”
Once treatment is started, says Dr. Roshchina, patients typically get better in a matter of weeks to a couple of months and make a full recovery, especially if GBS is diagnosed early. More severe cases can take longer, with some patients still experiencing varying degrees of weakness years later.
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