Medical Edge Newspaper Column

Guillain-Barre Syndrome: Most Patients Completely Recover

Jan. 22, 2007
Dear Mayo Clinic:
My 61-year-old uncle has been diagnosed with Guillain-Barre syndrome. What can you tell me about this condition and the available treatment options? How can one know if the syndrome will lead to paralysis? -- Winona, Minn.

Answer:
Guillain-Barre syndrome (GBS) is a relatively rare neurological disorder -- affecting about one in 100,000 people each year -- in which the body's immune system attacks its own peripheral nerves, which transmit electrical impulses to and from the brain and spinal cord. The primary target of this attack is believed to be myelin, the material that insulates nerves in the body. Resulting damage leads to the slowing or failure of signal transmission down the nerve, which limits or prevents muscles from responding appropriately.

Initial symptoms typically include numbness and tingling followed by weakness in the extremities. Symptoms can appear rapidly, even over the course of a single day. The weakness can progress to paralysis of the legs, arms and face; impaired breathing; difficulty in speaking, chewing, swallowing and eye movement; or problems with bladder control or intestinal function.

Severe GBS may result in total paralysis, potentially dangerous fluctuations in heart rate and blood pressure, or inability to breathe on one's own. In a minority of cases the syndrome can cause lasting physical impairment or even be fatal.

While its underlying cause is unknown, patients frequently report that symptoms begin within one to four weeks after a bacterial or viral infection such as sore throat, diarrhea, cold or flu. GBS may be also triggered by pregnancy or a medical procedure -- a vaccination or minor surgery, for example -- or simply have no evident reason for developing. Given the unknown cause, there is no available way to prevent GBS or to predict who will experience which symptoms and to what degree.

The good news is that most patients recover quite well from Guillain-Barre syndrome, usually within a few months. Approximately 80 percent of patients will completely recover within 12 months of onset.

Nevertheless, GBS is a serious disease that requires immediate hospitalization because of the rapid rate at which it can worsen. Early treatment is important. The sooner it is started, the better the chances of a good outcome. By reducing inflammation of the peripheral nerves, the risk of serious, permanent damage to the myelin sheath and nerve fibers -- and of permanent disability -- can be substantially decreased. This also can increase the speed of recovery. That's why individuals who experience tingling in their toes, feet, or legs followed by muscle weakness should seek medical attention immediately.

The two main treatment options for Guillain-Barre syndrome -- plasmapheresis (also known as "plasma exchange") and intravenous immunoglobulin (IVIg) -- are directed at suppressing the abnormal immune attack on the peripheral nerves and allowing for nerve regeneration and recovery. These two treatments are equally effective.

Plasmapheresis consists of separating the liquid portion of the blood (plasma) from the actual blood cells. The plasma is discarded and the blood cells are put back into the body, which manufactures more plasma to make up for what was removed. Though it is not yet clear how this treatment works, scientists believe that it removes certain antibodies from plasma that contribute to the immune system attack on the peripheral nerves.

In IVIg, high doses of immunoglobulin, which consists of antibodies from healthy blood donors, can block the damaging effect of antibodies in the patient's blood that may be contributing to GBS.

Before recovery begins, caregivers may need to manually move patients' arms and legs to help keep their muscles flexible and prevent contractures. After recovery has begun, patients usually require physical therapy, including an active exercise routine prescribed by a rehabilitation team, to help regain strength and mobility. They may need instruction in adaptive devices (such as a wheelchair or braces) to be able to function on their own.

With early treatment, prevention of medical complications, and physical therapy, most people recover from even severe cases of Guillain-Barre syndrome.

-- Brent P. Goodman, M.D., Neurology, Mayo Clinic, Scottsdale, Ariz.