Guillain-Barre Syndrome

Biology 202
2003 First Web Paper
On Serendip

Guillain-Barre Syndrome

Amelia Turnbull

Most people do not expect to become paralyzed during the course of their lives. Barring injury to the nervous system or debilitating disease, one does not expect to lose motor function. In spite of these expectations, people of all races, sexes, ages, and classes can be afflicted with a debilitating syndrome that can lead to difficulty in walking or even to temporary paralysis in the most severe cases. This syndrome is known commonly as Guillain-Barre Syndrome, or GBS.

GBS is an inflammatory disorder of the peripheral nerves. When the syndrome occurs, the body's peripheral nerves become inflamed and cease to work due to an unknown cause. (1) (3) Around 50% of the cases of GBS appear after a bacterial or viral infection. (1) The syndrome can also appear after surgery or vaccination. GBS can appear hours or days after these incidences or can even take up to three or four weeks to appear. (4) Some theories propose that GBS is caused by a mechanism of the autoimmune system that prompts antibodies and white blood cells to attack the covering and insulation of the nerve cells, which leads to abnormal sensation. GBS is considered a syndrome rather than a disease, because its description is based on a set of symptoms reported by the patient to her doctor. (5)

GBS is also known as acute inflammatory demylinating polyneuropathy and Landry's ascending paralysis after Jean B. O. Landry, a French physician who described a disorder that "paralyzed the legs, arms, neck, and breathing muscles of the chest." (4) (1) GBS was named after French physicians Georges Guillain and Jean Alexander Barre who, along with fellow physician Andre Stohl, described the differences of the spinal fluid of those who suffered from the syndrome. (5) The syndrome affects one to two people per 100,000 in the United States, making it the most common cause of rapidly acquired paralysis in this country. (1) Some patients initially diagnosed with GBS are later diagnosed with chronic inflammatory demyelinating poly[radiculo]neuropathy, or CIDP. (Sometimes radiculo is left out of the name, hence the brackets.) CIDP was initially known as "chronic GBS," but is now widely considered a related condition. (3)

Although patients can be preliminarily diagnosed with the syndrome based on an analysis of the physical symptoms, two tests can be used to confirm the diagnosis of GBS. The first is a lumbar puncture, or spinal tap, in order to obtain a small amount of spinal fluid for analysis. The spinal fluid of those with GBS often contains more protein than usual. The second is an electromyogram (EMG), which is an electrical measure of nerve conduction and muscle activity. (3) (4) The symptoms of GBS begin with numbness and tingling in the fingers and toes leading to weakness in the arms, legs, face, and breathing muscles. The weakness begins in the lower portion of the body and rapidly moves upward. This weakness eventually leads to loss of sensation in the affected areas; although a number of cases are mild, temporary limb paralysis is not uncommon. In the milder cases, the numbness can only cause difficulty in walking, "requiring sticks, crutches, or a walking frame." (3) Pain is not uncommon, and abnormal sensations, such as the feeling of "pins-and-needles," can affect both sides of the body equally. Loss of reflexes, for example the knee jerk, is common. (1) (3) Most patients are at their weakest point about two weeks after the onset of the symptoms, and around 90% will have reached their weakest state three weeks after the onset of symptoms. (4)

The progression of the syndrome is unpredictable, especially in the early stages, and patients may preemptively put in the hospital to be cared for. (1) In the more severe cases, patients require hospitalization and a stay in the intensive care unit in the early stages of their disease, especially if they need the assistance of a respirator, or ventilator, to breathe. In about a quarter of the cases of GBS, the paralysis moves up to the patient's chest, requiring this assistance from a respirator. The face and throat may also be affected by the paralysis, necessitating a feeding tube through the nose or directly into the stomach. (3) The period that the patient is afflicted with the syndrome can be extremely long and long-term hospital stays are not uncommon. Care is generally confined to supportive measures to the patient as the condition improves spontaneously, and efforts are made to speed recovery of nerve function, including physical therapy and hydrotherapy. Most patients eventually recover and go on to lead normal, or very near normal lives. Some patients, however, may remain partially paralyzed and require the use of a wheelchair for a long period of time. (1) (3) Around 30% of GBS patients still feel residual weakness three years after the onset of symptoms. (4) Death due to GBS is highly unlikely with modern medical practices, but death does occur in about 5% of the cases. (3) For those with CIDP, the course of the disease is generally longer than those with GBS and the recovery period can follow a recovery/relapse cycle, but the patients are less likely to suffer respiratory failure. (3)

The syndrome came to brief public attention in 1976 when a number of people vaccinated against Swine Flu were stricken with the syndrome. (1) A Minnesota doctor had reported to his local health board that after inoculating a man for Swine Flu, the man had developed GBS. The health board reported this the Center for Disease Control, who were running the Swine Flu vaccination program, and the CDC began to ask doctors to report cases of newly diagnosed GBS. It did not take long for doctors to begin to associate GBS with the vaccine, even though the CDC tried not to relay this impression. As GBS is difficult to diagnose and has many symptoms similar to other neurological diseases, doctors at the time might have been more likely to diagnose patients who came in with muscle weakness who did receive the vaccine with GBS more often than those patients who presented muscle weakness and did not receive the vaccine. As the CDC could not say with certainty that the flu vaccine was not causing the high number of cases of GBS, the CDC announced cessation of the vaccination program on December 16, 1976. To this day, some people avoid flu vaccines because of this one incidence of extreme side affects. (2)

In the end, a person with GBS may eventually fully recover her motor function to the level it was before her affliction with the syndrome. While she may be left with no physical reminders of the disorder, she and her family and friends will always remember her sudden incapacitation. The emotional scars from such an incident can last a lifetime.

References

1) Guillain-Barre Syndrome International - GBS: An Overview, An overview of GBS on the website of the Guillain-Barre Syndrome Foundation International, based in Wynnewood, PA.

2) Kolata, Gina. Flu: The Story of the Great Influenza Pandemic of 1918 and the Search for the Virus That Caused It. Simon & Schuster: New York. Pgs. 167-185.

3) Guillain-Barré Support Group, The homepage for the Guillain-Barre Syndrome Support Group based in the United Kingdom. The organization disseminates information to sufferers of the syndrome and their family and friends.

4) NINDS Guillain-Barre Information Page, National Institute of Neurological Disorders and Stroke information page on GBS.

5) GBS - An Overview For The Layperson, An overview of GBS written by Dr. Joel S. Steinberg, a neurologist that once suffered from GBS.

Comments made prior to 2007

Hello I was reading the comments on GBS. In 1975, August I came down with GBS and with in two days of the onset I was in a coma. It only lasted for two days and the nightmare began, I spent 6 moths in intensive care and two years in rehab. I lost all functions, breathing, talking, closing my eyes, you name it I lost it. I was told to be grateful I had such a strong heart. I was told several times I was going to die. I went from 120 pounds to a mere 48, looked like bones drapped in a sheet. To this day I am doing so much better. I have had 5 surgeries on my feet, they hurt all the time. I can't straighten my legs out while sitting on the floor, I can't put my hands out flat and apply preasure, I can shut one eye and not move the other, it looks much like an owl. I have muscle cramps often. My doctors say my body goes in cycle, I'm good for a few years and poof I need surgery. I have no spare parts. I'm so greatful for what I have. God truly kept his hand on me. I was in St John's hospital in Springfield Illinois 1975-1976. They took such wonderful care of me, I was 21 when it hit. I had not been ill. It came in my feet and I kept falling by the next day it was all the way in my hands, and that evening I went into the coma. It took awhile for the diagnosis, the first doctor I had thought I was faking and never bothered with feeding tubes. Long story short I am good but left with residual effects, 40% feeling in my feet and 50% in my lower legs. I can't remember a day since that my feet have not hurt. I am a teacher,a high school science teacher, and I am so grateful to God for this opportunity ... Barbara Saathoff, 7 November 2007