Guillain-Barrè Syndrome Fact Sheet
Table of Contents
Guillain-Barrè (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 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-Barrè syndrome, although some continue to have a certain degree of
weakness.
Guillain-Barrè 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-Barrè 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. 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.
No one yet knows why Guillain-Barrè 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-Barrè 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-Barrè 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-Barrè syndrome and other autoimmune diseases. The cause and
course of Guillain-Barrè syndrome is an active area of neurological
investigation, incorporating the cooperative efforts of neurological scientists,
immunologists, and virologists.
Guillain-Barrè is called a syndrome rather than a disease because it is not
clear that a specific disease-causing agent is involved. A syndrome is a medical
condition characterized by a collection of symptoms (what the patient feels) and
signs (what a doctor can observe or measure). The signs and symptoms of the
syndrome can be quite varied, so doctors may, on rare occasions, find it
difficult to diagnose Guillain-Barrè in its earliest stages.
Several disorders have symptoms similar to those found in Guillain-Barrè , so
doctors examine and question patients carefully before making a diagnosis.
Collectively, the signs and symptoms form a certain pattern that helps doctors
differentiate Guillain-Barrè from other disorders. For example, physicians will
note whether the symptoms appear on both sides of the body (most common in
Guillain-Barrè ) and the quickness with which the symptoms appear (in other
disorders, muscle weakness may progress over months rather than days or weeks).
In Guillain-Barrè , 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. In Guillain-Barrè patients,
the cerebrospinal fluid that bathes the spinal cord and brain contains more
protein than usual. Therefore a physician may decide to perform a spinal tap, a
procedure in which the doctor inserts a needle into the patient's lower back to
draw cerebrospinal fluid from the spinal column.
There is no known cure for Guillain-Barre syndrome. However, there are
therapies that 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. Both
of them are equally effective, but immunoglobulin is easier to administer.
Plasmapheresis is a method by which whole blood is removed from the body and
processed so that the red and white blood cells are separated from the plasma,
or liquid portion of the blood. The blood cells are then returned to the patient
without the plasma, which the body quickly replaces. Scientists still don't know
exactly why plasmapheresis works, but the technique seems to reduce the severity
and duration of the Guillain-Barrè episode. This may be because the plasma
portion of the blood contains elements of the immune system that may be toxic to
the myelin.
In high-dose immunoglobulin therapy, doctors give intravenous injections of
the proteins that, in small quantities, the immune system uses naturally to
attack invading organisms. Investigators have found that giving high doses of
these immunoglobulins, derived from a pool of thousands of normal donors, to
Guillain-Barrè patients can lessen the immune attack on the nervous system.
Investigators don't know why or how this works, although several hypotheses have
been proposed.
The use of steroid hormones has also been tried as a way to reduce the
severity of Guillain-Barrè , but controlled clinical trials have demonstrated
that this treatment not only is not effective but may even have a deleterious
effect on the disease.
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. The need for this sophisticated machinery is
one reason why Guillain-Barrè syndrome patients are usually treated in
hospitals, often in an intensive care ward. In the hospital, doctors can also
look for and treat the many problems that can afflict any paralyzed patient –
complications such as pneumonia or bed sores.
Often, even before recovery begins, caregivers may be instructed to manually
move the patient's limbs to help keep the muscles flexible and strong. Later, as
the patient begins to recover limb control, physical therapy begins. Carefully
planned clinical trials of new and experimental therapies are the key to
improving the treatment of patients with Guillain-Barrè syndrome. Such clinical
trials begin with the research of basic and clinical scientists who, working
with clinicians, identify new approaches to treating patients with the disease.
Guillain-Barrè syndrome can be a devastating disorder because of its sudden
and unexpected onset. In addition, recovery is not necessarily quick. As noted
above, patients usually reach the point of greatest weakness or paralysis days
or weeks after the first symptoms occur. Symptoms then stabilize at this level
for a period of days, weeks, or, sometimes, months. 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-Barrè 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.
Guillain-Barrè syndrome patients face not only physical difficulties, but
emotionally painful periods as well. It is often extremely difficult for
patients to adjust to sudden paralysis and dependence on others for help with
routine daily activities. Patients sometimes need psychological counseling to
help them adapt.
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-Barrè 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. Certain proteins or
peptides in viruses and bacteria may be the same as those found in myelin, and
the generation of antibodies to neutralize the invading viruses or bacteria
could trigger the attack on the myelin sheath. As noted previously, 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.
The National Institute of Neurological Disorders and Stroke conducts and
supports a wide range of research on neurological disorders, including
Guillain-Barrè syndrome.
Guillain-Barre Syndrome Foundation International P.O. Box 262
Wynnewood, PA 19096 info@gbsfi.com
http://www.gbsfi.com Tel: 610-667-0131
Fax: 610-667-7036
For information on other neurological disorders or research programs funded
by the National Institute of Neurological Disorders and Stroke, contact the
Institute's Brain Resources and Information Network (BRAIN) at:
BRAIN P.O. Box 5801 Bethesda, MD 20824 (800) 352-9424 www.ninds.nih.gov
Reviewed July 1,
2001
Page forms part of www.apls.tk, the information site on ANTIPHOSPHOLIPID SYNDROME (APS or ANTIPHOSPHOLIPID SYNDROME (APLS))
Medical Keywords: systemic antiphospholipid antibody syndrome, Antiphospholipid, Antiphospholipid Antibody Syndrome, Antiphospholipid Syndrome, APS, APLS, Hughes
Syndrome, Sticky Blood, Clotting Disorder, Stroke, TIA, PE, death, Antiphospholipid Antibody Syndrome, Antiphospholipid Syndrome, APS, APLS,
Hughes Syndrome, Sticky Blood, Clotting Disorder, Stroke, TIA, PE, death
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