About two years ago, Tammy Delacruz, now 52, of North Las Vegas, NV, began feeling a heaviness in her legs, as well as weakness and lightheadedness. She wasn't initially alarmed, but when she couldn't move her legs, her mother rushed her to the hospital. After blood tests and an MRI didn't reveal the cause, Delacruz was admitted for observation. Three days later, she was paralyzed from the waist down.
The doctors couldn't confirm a diagnosis and transferred her to a nursing home, where her symptoms worsened. Several days later she was transferred to another hospital, where the doctors diagnosed her with Guillain-Barre syndrome (GBS), a rare autoimmune neurologic disease in which the body's immune system produces antibodies against the peripheral nerves—the nerves outside the spinal column and brain, which connect the central nervous system to critical organs, including the eyes, ears, muscles, blood vessels, and glands.
Her doctors diagnosed her based on her symptoms and her responses to specific tests. In addition to weakness on both sides of her body and her rapid deterioration, Delacruz also experienced diminished deep tendon reflexes. Her doctors completed a nerve conduction velocity test to determine whether the signals traveling along some nerves were slower than they should be and a lumbar puncture to see if her cerebrospinal fluid (CSF) contained more protein than usual.
Delacruz's experience of a delayed diagnosis is not uncommon, says Alejandro Tobon, MD, assistant professor of neurology at the University of Texas Health Science Center in San Antonio. "We need to do a better job of educating health providers to consider GBS if patients report weakness and increasing paralysis," he says.
Supportive Treatment
There's no cure for GBS. Instead, doctors treat the condition with supportive therapy, such as a breathing tube if respiratory muscles are weakened, and immunotherapy—intravenous immunoglobulin (IVIG) or plasma exchange—to help speed recovery. Although researchers aren't sure how, high doses of IVIG can diminish the immune system's attack on the nervous system and reduce inflammation. Plasma exchange—a procedure that removes the patient's blood and separates the red and white blood cells from the plasma, the liquid part of the blood, then replaces the plasma with a protein fluid and pumps that mixture of red and white blood cells and protein fluid back into the patient—may work by eliminating some of the antibodies that were part of the immune system's attack on the peripheral nerves, says Avindra Nath, MD, MBBS, FAAN, a senior investigator in the section of infections of the nervous system at the National Institute of Neurological Disorders and Stroke (NINDS) in Bethesda, MD.
Cause Unclear
Experts aren't certain what causes GBS, but it has been linked to infection, having had surgery, and, very rarely, vaccinations; perhaps because in some people, any one or all of these occurrences stresses their immune systems, triggering GBS, says Dr. Tobon. (Delacruz had bariatric surgery and a flu shot in the three months before developing GBS.)
Another possible theory is that a virus may change the nature of the cells in the nervous system so that the immune system considers them foreign and starts attacking them, according to the NINDS. It is also possible that the virus makes the immune system less discriminating about what cells it recognizes as its own, allowing some of the immune cells, such as certain kinds of white blood cells, to attack the myelin, the fatty sheath that insulates nerve cells.
Regardless of its causes, GBS is a rare disorder: About 3,000 to 6,000 cases are reported each year, according to the US Centers for Disease Control and Prevention (CDC). It can affect anybody, at any age, and is just as likely to affect women as men, according to the NINDS.
Zika Virus Connection
Four years ago, GBS made headlines when the Zika virus epidemic was first reported. The virus, which is transmitted by the bite of infected Aedes mosquitoes and through sex, appears to have triggered cases of GBS in some people infected with Zika. CDC research suggests that GBS is strongly associated with Zika even though only a fraction of people with Zika get GBS. The CDC is studying the link to learn more.
Adults outside of the continental United States recovering from Zika infections seemed to have higher-than-average rates of GBS, according to a 2016 letter in the New England Journal of Medicine written by members of the Pan American Health Organization, an international health agency for the Americas. They also reported that as cases of Zika increased, so did cases of GBS. In Bahia, Brazil, for example, rates of GBS increased by 172 percent after the Zika outbreak. The researchers said more study was needed to determine if and how Zika causes GBS.
"GBS in Zika-affected patients behaves a bit differently," says Dr. Tobon. When GBS appears to be triggered by an infection, vaccination, or surgery, it can take a few weeks for symptoms to develop. In those affected by Zika in South America and the Caribbean, the symptoms can occur within a few days of the infection. "We think that may be because people in South America and the Caribbean are exposed to other, similar viruses, and their immune systems may already be primed to react to Zika," Dr. Tobon says.
"It also looks like the Zika virus causes an autoimmune phenomenon leading to GBS," says Dr. Nath, referring to a study published in Scientific Reports in January 2018.
Recovery Varies
Most people recover fully from GBS, but severe cases can leave people permanently disabled, says Daniel Pastula, MD, assistant professor of neurology, infectious diseases, and epidemiology at the University of Colorado. Early treatment is critical.
Delacruz recovered at a rehabilitation facility for a few months and is now living on her own again. She continues to experience muscle weakness, fatigue, and nerve pain, and she uses a cane or a walker to get around. But she's excited about her future, having recently graduated with a master's degree in social work.
How to Protect Yourself from Guillain-Barre Syndrome
The exact cause of Guillain-Barre syndrome (GBS) hasn't been determined, but about two-thirds of people who develop the syndrome experience symptoms several days or weeks after they've been sick with diarrhea or a respiratory illness, according to the US Centers for Disease Control and Prevention (CDC).
Infection with a diarrhea-causing bacterium called Campylobacter jejuni, which is often found in undercooked poultry, is one of the most common risk factors for GBS, according to the CDC.
People also have been known to develop GBS after having the flu or other infections such as cytomegalovirus and Epstein-Barr virus. In very rare cases, a vaccination, such as a flu shot, can trigger GBS: About one to two cases of GBS a year per 1 million flu vaccinations are reported, according to the CDC. Experts recommend avoiding flu shots in the future if you developed GBS after getting one.
There are many other infectious triggers, including the Zika virus, says Daniel Pastula, MD, assistant professor of neurology, infectious diseases, and epidemiology at the University of Colorado. "To avoid Zika virus disease and the rare complication of Zika-associated GBS, protect yourself against mosquito bites," says Dr. Pastula. "Apply insect repellent, wear long-sleeved shirts and pants when feasible, and use air conditioning or window screens when indoors to keep mosquitoes outside."
Guillain-Barre Resources
