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作者progress (爆片的迷失...), 信區: birdbig_301
標題Guillain-Barre Syndrome(英文版) (轉載)
時間成大物治 黃金之島 (Mon May 10 01:37:44 2004) , 轉信
【 以下文字轉載自 Bremen 討論區 】
【 原文由 progress 所發表 】
Guillain-Barre (ghee-yan bah-ray) syndrome is a disorder in which the body's im
mune system attacks part of the peripheral nervous system. The first symptoms o
f this disorder include varying degrees of weakness or tingling sensations in t
he legs. In many instances the weakness and abnormal sensations spread to the a
rms 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 cas
es the disorder is life threatening -- potentially interfering with breathing a
nd, at times, with blood pressure or heart rate -- and is considered a medical 
emergency. The patient is often put on a respirator to assist with breathing an
d is watched closely for problems such as an abnormal heart beat, infections, b
lood clots, and high or low blood pressure. Most patients, however, recover fro
m even the most severe cases of Guillain-Barre syndrome, although some continue
 to have some degree of weakness.
Guillain-Barre syndrome can affect anybody. It can strike at any age and both s
exes are equally prone to the disorder. The syndrome is rare, however, afflicti
ng only about one person in 100,000. Usually Guillain-Barre occurs a few days o
r weeks after the patient has had symptoms of 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 f
irst 2 weeks after symptoms appear, and by the third week of the illness 90 per
cent of all patients are at their weakest.
What causes Guillain-Barre syndrome?
No one yet knows why Guillain-Barre strikes some people and not others. Nor doe
s anyone know exactly what sets the disease in motion.
What scientists do know is that the body's immune system begins to attack the b
ody itself, causing what is known as an autoimmune disease. Usually the cells o
f the immune system attack only foreign material and invading organisms. In Gui
llain-Barre syndrome, however, the immune system starts to destroy the myelin s
heath that surrounds the axons of many peripheral nerves, or even the axons the
mselves (axons are long, thin extensions of the nerve cells; they carry nerve s
ignals). The myelin sheath surrounding the axon speeds up the transmission of n
erve signals and allows the transmission of signals over long distances.
In diseases in which the peripheral nerves' myelin sheaths are injured or degra
ded, the nerves cannot transmit signals efficiently. That is why the muscles be
gin to lose their ability to respond to the brain's commands, commands that mus
t be carried through the nerve network. The brain also receives fewer sensory s
ignals from the rest of the body, resulting in an inability to feel textures, h
eat, pain, and other sensations. Alternately, the brain may receive inappropria
te signals that result in tingling, "crawling-skin," or painful sensations. Bec
ause the signals to and from the arms and legs must travel the longest distance
s they are most vulnerable to interruption. Therefore, muscle weakness and ting
ling sensations usually first appear in the hands and feet and progress upwards
.
When Guillain-Barre is preceded by a viral infection, it is possible that the v
irus has changed the nature of cells in the nervous system so that the immune s
ystem treats them as foreign cells. It is also possible that the virus makes th
e immune system itself less discriminating about what cells it recognizes as it
s own, allowing some of the immune cells, such as certain kinds of lymphocytes,
 to attack the myelin. Scientists are investigating these and other possibiliti
es to find why the immune system goes awry in Guillain-Barre syndrome and other
 autoimmune diseases. The cause and course of Guillain-Barre syndrome is an act
ive area of neurological investigation, incorporating the cooperative efforts o
f neurological scientists, immunologists, and virologists.
How is Guillain-Barre syndrome diagnosed?
Guillain-Barre is called a syndrome rather than a disease because it is not cle
ar that a specific disease-causing agent is involved. A syndrome is a medical c
ondition 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 sy
ndrome can be quite varied, so doctors may, on rare occasions, find it difficul
t to diagnose Guillain-Barre in its earliest stages.
Several disorders have symptoms similar to those found in Guillain-Barre, so do
ctors examine and question patients carefully before making a diagnosis. Collec
tively, the signs and symptoms form a certain pattern that helps doctors differ
entiate Guillain-Barre from other disorders. For example, physicians will note 
whether the symptoms appear on both sides of the body (most common in Guillain-
Barre ) and the quickness with which the symptoms appear (in other disorders, m
uscle weakness may progress over months rather than days or weeks). In Guillain
-Barre, reflexes such as knee jerks are usually lost. Because the signals trave
ling along the nerve are slower, a nerve conduction velocity (NCV) test can giv
e a doctor clues to aid the diagnosis. In Guillain-Barre patients, the cerebros
pinal fluid that bathes the spinal cord and brain contains more protein than us
ual. Therefore a physician may decide to perform a spinal tap, a procedure in w
hich the doctor inserts a needle into the patient's lower back to draw cerebros
pinal fluid from the spinal column.
How is Guillain-Barre treated?
There is no known cure for Guillain-Barre syndrome. However, there are therapie
s that lessen the severity of the illness and accelerate the recovery in most p
atients. There are also a number of ways to treat the complications of the dise
ase.
Currently, plasmapheresis and high-dose immunoglobulin therapy are used. Both o
f them are equally effective, but immunoglobulin is easier to administer. Plasm
apheresis is a method by which whole blood is removed from the body and process
ed so that the red and white blood cells are separated from the plasma, or liqu
id portion of the blood. The blood cells are then returned to the patient witho
ut the plasma, which the body quickly replaces. Scientists still don't know exa
ctly why plasmapheresis works, but the technique seems to reduce the severity a
nd duration of the Guillain-Barre episode. This may be because the plasma porti
on 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 i
mmunoglobulins, derived from a pool of thousands of normarks, although several hypotheses have been propo
sed.
The use of steroid hormones has also been tried as a way to reduce the severity
 of Guillain-Barre, but controlled clinical trials have demonstrated that this 
treatment not only is not effective but may even have a deleterious effect on t
he disease.
The most critical part of the treatment for this syndrome consists of keeping t
he patient's body functioning during recovery of the nervous system. This can s
ometimes 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-Barre syndrome patients are usually treated in hospi
tals, often in an intensive care ward. In the hospital, doctors can also look f
or and treat the many problems that can afflict any paralyzed patient -- compli
cations such as pneumonia or bed sores.
Often, even before recovery begins, caregivers may be instructed to manually mo
ve 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 improv
ing the treatment of patients with Guillain-Barre syndaches to treating patients with the disease.
What is the long-term outlook for those with Guillain-Barre syndrome?
Guillain-Barre syndrome can be a devastating disorder because of its sudden and
 unexpected onset. In addition, recovery is not necessarily quick. As noted abo
ve, 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-Barre still have a residual weakness after 3 years. About 3 percent m
ay suffer a relapse of muscle weakness and tingling sensations many years after
 the initial attack.
Guillain-Barre syndrome patients face not only physical difficulties, but emoti
onally painful periods as well. It is often extremely difficult for patients to
 adjust to sudden paralysis and dependence on others for help with routine dail
y activities. Patients sometimes need psychological counseling to help them ada
pt.
What research is being done?
Scientists are concentrating on finding new treatments and refining existing on
es. Scientists are also looking at the workings of the immune system toillain-Barre begin after a viral i
nfection suggests that certain characteristics of these viruses may activate th
e immune system inappropriately. Investigators are searching for those characte
ristics. As noted previously, neurological scientists, immunologists, virologis
ts, and pharmacologists are all working collaboratively to learn how to prevent
 this disorder and to make better therapies available when it strikes.
Where can I go for more information?
The National Institute of Neurological Disorders and Stroke conducts and suppor
ts a wide range of research on neurological disorders, including Guillain-Barre
 syndrome. The organization listed below provides printed information and assis
tance to Guillain-Barre patients and other interested parties.
Guillain-Barre Syndrome Foundation International
P.O. Box 262
Wynnewood, PA 19096
610-667-0131


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