Guillain-Barre Syndrome
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Guillain-Barré (ghee-yan bah-ray) syndrome 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 the muscles cannot be used at all and the patient is almost
totally paralyzed. In these cases, the disorder is life-threatening
and is considered a medical emergency. The patient is often put on a
respirator to assist with breathing. Most patients, however, recover
from even the most severe cases of Guillain-Barré syndrome, although
some continue to have some degree of weakness. Guillain-Barré
syndrome is rare. 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. The disorder can develop
over the course of hours or days, or it may take up to 3 to 4 weeks.
No one yet knows why Guillain-Barré strikes some people and not
others 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. Guillain-Barré is
called a syndrome rather than a disease because it is not clear that
a specific disease-causing agent is involved. Reflexes such as knee
jerks are usually lost. Because the signals traveling along the
nerve are slower, a nerve conduction velocity (NCV) test can give a
doctor clues to aid the diagnosis. The cerebrospinal fluid that
bathes the spinal cord and brain contains more protein than usual,
so a physician may decide to perform a spinal tap.
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There is no known cure for Guillain-Barre
syndrome, but therapies can lessen the severity of the illness and
accelerate the recovery in most patients. There are also a number
of
ways to treat the complications of the disease. Currently,
plasmapheresis and high-dose immunoglobulin therapy are used.
Plasmapheresis seems to reduce the severity and duration of the
Guillain-Barré episode. In high-dose immunoglobulin therapy, doctors
give intravenous injections of the proteins that in small
quantities, the immune system uses naturally to attack invading
organism. Investigators have found that giving high doses of these
immunoglobulins, derived from a pool of thousands of normal donors,
to Guillain-Barré patients can lessen the immune attack on the
nervous system. The most critical part of the treatment for this
syndrome consists of keeping the patient's body functioning during
recovery of the nervous system. This can sometimes require placing
the patient on a respirator, a heart monitor, or other machines that
assist body function.
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Guillain-Barré syndrome can be a devastating
disorder because of its sudden and unexpected onset. Most people
reach the stage of greatest weakness within the first 2 weeks after
symptoms appear, and by the third week of the illness 90 percent of
all patients are at their weakest. The recovery period may be as
little as a few weeks or as long as a few years. About 30 percent of
those with Guillain-Barré still have a residual weakness after 3
years. About 3 percent may suffer a relapse of muscle weakness and
tingling sensations many years after the initial attack.
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Scientists are concentrating on finding new treatments
and refining existing ones. Scientists are also looking at the
workings of the immune system to find which cells are responsible
for beginning and carrying out the attack on the nervous system. The
fact that so many cases of Guillain-Barré begin after a viral or
bacterial infection suggests that certain characteristics of some
viruses and bacteria may activate the immune system inappropriately.
Investigators are searching for those characteristics. Neurological
scientists, immunologists, virologists, and pharmacologists are all
working collaboratively to learn how to prevent this disorder and to
make better therapies available when it strikes.
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Guillain-Barre Syndrome Foundation International
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