Guillain-Barré Syndrome
Guillain-Barré Syndrome Symptoms
Medical Author: Benjamin C. Wedro, MD, FAAEM
Medical Editor: Melissa Conrad Stöppler, MD
It takes a celebrity to draw attention to illnesses that
often fly under the radar. Such is the case of William "The Fridge" Perry, the
Ex-Chicago Bear football player who came to national attention when the team won
the Super Bowl with Perry as a defensive star. In 2008, Mr. Perry
spent five months in the hospital because of Guillain-Barré syndrome and survived an illness that
is a potential killer.
Nobody knows what why Guillain-Barré
syndrome occurs. It's an autoimmune disease in
which the body attacks its own tissue; in this disease, the tissue is the myelin
that covers nerve fibers and the
attack causes failure of those nerves to work. This leads to symptoms that begin
with numbness and tingling of the arms or legs and can progress to muscle
weakness. The major worry with this condition is that
important muscles like those that help us breathe become weak, and respiratory
failure can occur.
What is Guillain-Barré syndrome?
Guillain-Barré syndrome (often misspelled Guillain-Barre) is a disorder in which the body's immune system attacks part of the peripheral nervous system. The first symptoms of this disorder include varying degrees of weakness or tingling
sensations in the legs. In many instances the weakness and abnormal sensations
spread to the arms and upper body. These symptoms can increase in intensity
until certain muscles cannot be used at all and, when severe, the patient is
almost totally paralyzed. In these cases the disorder is life threatening -
potentially interfering with breathing and, at times, with blood pressure or heart rate - and is
considered a medical emergency. Such a patient is often put on a respirator to
assist with breathing and is watched closely for problems such as an abnormal
heart beat, infections, blood clots, and high or low blood pressure. Most patients, however, recover from even the most severe cases of Guillain-Barré syndrome, although some continue to have a certain degree of weakness.
Guillain-Barré syndrome can affect anybody. It can strike at any age and both sexes are equally prone to the disorder. The syndrome is rare, however, afflicting only about one person in 100,000. Usually Guillain-Barré occurs a few days or weeks after the patient has had symptoms of a respiratory or gastrointestinal viral infection.
Occasionally surgery or vaccinations will trigger the syndrome.
What causes Guillain-Barré syndrome?
No one yet knows why Guillain-Barré - which is not contagious - strikes some people and not others. Nor does anyone know exactly what sets the disease in motion.
What scientists do know is that the body's immune system begins to attack the body itself, causing what is known as an autoimmune disease. Usually the cells of the immune system attack only foreign material and invading organisms. In Guillain-Barré syndrome, however, the immune system starts to destroy the myelin sheath that
surrounds the axons of many peripheral nerves, or even the axons themselves (axons are long, thin extensions of the nerve cells; they carry nerve signals). The myelin sheath surrounding the axon speeds up the transmission of nerve signals and allows the transmission of signals over long distances.
In diseases in which the peripheral nerves' myelin sheaths are injured or degraded, the nerves cannot transmit signals efficiently. That is why the muscles begin to lose their ability to respond to the brain's commands, commands that must be carried through the nerve network. The brain also receives
fewer sensory signals from the rest of the body, resulting in an inability to feel textures, heat, pain, and other
sensations. Alternately, the brain may receive inappropriate signals that result
in tingling, "crawling-skin," or painful sensations. Because the signals to and
from the arms and legs must travel the longest distances they are most
vulnerable to interruption. Therefore, muscle weakness and tingling sensations usually first appear in the hands and feet and progress upwards.
When Guillain-Barré is preceded by a viral or bacterial infection, it is possible that the virus has changed the
nature of cells in the nervous system so that the immune system treats them as
foreign cells. It is also possible that the virus makes the immune system itself
less discriminating about what cells it recognizes as its own, allowing some of
the immune cells, such as certain kinds of lymphocytes and macrophages, to
attack the myelin. Sensitized T lymphocytes cooperate with B lymphocytes to
produce antibodies against components of the myelin sheath and may contribute to
destruction of the myelin. Scientists are investigating these and other
possibilities to find why the immune system goes awry in Guillain-Barré syndrome
and other autoimmune diseases. The cause and course of Guillain-Barré syndrome is an active area of neurological investigation, incorporating the cooperative efforts of neurological scientists, immunologists, and virologists.
Next: What are symptoms of Guillain-Barré syndrome? »
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