Niece’s death from GBS leaves many 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: 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. 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. The rate is far lower than the number of people who die from the flu. 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.
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. 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.