Death from Guillain-Barre syndrome leaves questions

DEAR DOCTOR » Could you explain Guillain-Barre syndrome? My 57-year-old niece died of it, but it’s still a mystery to me.

DEAR READER » First, please allow me to offer my condolences for the loss of your niece. There’s no way to make the loss any easier, but I will try to explain a bit more about this terrible disease.

Guillain-Barre syndrome (GBS) is an acute inflammatory attack on the nerves by the immune system. The theory is that after certain viral or bacterial infections, the immune system, in its attempt to attack the virus or bacteria, also attacks the nerve cells because their proteins are similar to the viral or bacterial proteins. As the nerves fail to work, the patient develops progressive muscle weakness and nerve dysfunction throughout the body. Each year in the United States, the syndrome is diagnosed in one or two of every 100,000 people; the risk increases as we get older.

Infections with the intestinal bacteria Campylobacter jejuni have been known to increase the chances of GBS, with one study finding that three out of 1,000 patients infected with Campylobacter jejuni develop GBS. The bacterium is found in contaminated, undercooked poultry and meat, but also unpasteurized milk. Infection with HIV, influenza virus, Epstein Barr virus and cytomegalovirus also have been linked to a higher risk of GBS.

The influenza vaccine may slightly increase the chance of developing GBS. Specifically, about one out of a million people vaccinated against the flu develop the condition, although the rates are a bit higher with the H1N1 influenza vaccine — about two in a million. Note that this is far lower than the number of people who die from the flu — 1.4 per 100,000 — and the number of people who develop GBS after the vaccine is actually lower than the number of unvaccinated people who develop it. The meningitis vaccine Menactra also has been linked to GBS in those ages 11 to 19, but the level of risk is still under investigation.

GBS manifests in many different ways, but symptoms generally begin with back pain and a feeling of tightness and tingling in the lower legs. One or two days later, weakness develops in the legs — so much so that getting up from a chair or walking up the stairs can be difficult. Weakness then can develop in the mouth, throat and face, causing difficulty talking and eating. Weakness can also develop in the eyes, causing altered vision.

When the muscles used to breathe are involved, progressive shortness of breath develops, with 9.1 percent of patients hospitalized for GBS needing a respirator. In 70 percent of patients, GBS can also affect the nerves that go to the heart, blood vessels, bladder and the intestines, leading to abnormal heart, bowel and bladder function.

Without treatment, 67 percent of people with GBS will start to recover after four weeks. The recovery may be slow and incomplete depending on the severity of the illness. The use of plasmapheresis, which filters antibodies from the blood, can speed improvement, as can intravenous immunoglobulin. Even with these treatments, the death rate of those hospitalized with GBS is 2.58 percent.