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Definition the arms and legs over the next few days.

Some patients
experience paresthesia only in their toes and legs; others only
experience symptoms on one side of the body.
Guillain-Barre syndrome is an uncommon disorder in which your
body's immune system attacks your nerves. Weakness and
numbness in your extremities are usually the first symptoms. The symptoms may stay in this phase, causing only mild difficulty
These sensations can quickly spread, eventually paralyzing your in walking, requiring crutches or a walking stick. However,
whole body. sometimes the illness progresses, leading to complete paralysis of
the arms and legs. About one quarter of the time, the paralysis
continues up the chest and freezes the breathing muscles, leaving
The exact cause of Guillain-Barre syndrome is unknown, but it is
the patient dependent on a ventilator. If the swallowing muscles
often preceded by an infectious illness such as a respiratory
are also affected, a feeding tube may be needed.
infection or the stomach flu. Luckily, Guillain-Barre syndrome is
relatively rare, affecting only 1 or 2 people per 100,000.
In chronic inflammatory demyelinating polyradicalneuropathy
(CIDP), the course of illness is longer and respiratory failure is
In its most severe form, Guillain-Barre syndrome is a medical
much more unlikely.
emergency requiring hospitalization. There's no known cure for
Guillain-Barre syndrome, but several treatments can ease
symptoms and reduce the duration of the illness. And most people GBS Diagnosis
do recover completely.
Because its symptoms vary and its cause is unknown, GBS can be
Cause difficult to diagnose. If the symptoms occur uniformly across the
body and progress rapidly, the diagnosis is easier.
The exact cause of Guillain-Barre syndrome is unknown. In about
60 percent of cases, an infection affecting either the lungs or the Observation of the patient's symptoms and an evaluation of the
digestive tract precedes the disorder. But scientists don't know medical history provide the basis for diagnosis of Guillain-Barre
why such an infection can lead to Guillain-Barre syndrome for syndrome, although no single observation is suitable to make the
some people and not for others. Many cases appear to occur diagnosis.
without any triggers.
Tests
In Guillain-Barre syndrome, your immune system — which usually Three tests can confirm a diagnosis of Guillain-Barre syndrome.
only attacks foreign material and invading organisms — begins
attacking the nerves that carry signals between your body and
Lumbar puncture (spinal tap)—The patient is given local
your brain. Specifically, the nerves' protective covering (myelin
anesthetic. Once the anesthetic has taken effect, a needle is
sheath) is damaged and this interferes with the signaling process,
inserted between two lower (lumbar) vertebrae and a sample of
causing weakness, numbness or paralysis.
cerebrospinal fluid is drawn. An elevated level of protein without
an increase in the number of white blood cells (WBCs) in the fluid
Overview is characteristic of GBS.

Guillain-Barre syndrome (GBS) is an inflammatory disorder of the Electromyogram (EMG)—This is an effective diagnostic tool
peripheral nerves. The peripheral nerves convey sensory because it records muscle activity and can show the loss of
information (e.g., pain, temperature) from the body to the brain individual nerve impulses due to the disease's characteristic
and motor (i.e., movement) signals from the brain to the body. slowing of nerve responses.
GBS is characterized by weakness and numbness or tingling in the
legs and arms, and possible loss of movement and feeling in the
Nerve conduction velocity (NCV)—This test is performed with
legs, arms, upper body, and face.
EMG, and together, they are often referred to as EMG/NCV
studies. NCV records the speed at which signals travel along the
Chronic inflammatory demyelinating polyradicalneuropathy nerves. These signals are characteristically slowed in GBS,
(CIDP), is considered to be a related form of Guillain-Barre although the findings may evolve over several weeks.
syndrome. It is much less common than GBS, and evolves much
more slowly and usually is longer lasting. Some CIDP patients
Treatment
experience periods of worsening and improvement, and individual
relapses can be confused with GBS.
GBS is considered a medical emergency and most patients are
admitted to the hospital soon after diagnosis. If the patient's
GBS Causes
breathing seems to be at risk, he or she is usually managed in an
intensive care unit (ICU). Although GBS can improve
Guillain-Barre syndrome is not hereditary or contagious. What spontaneously, there are a number of treatments that facilitate
causes GBS is not known; however, in about half of all cases the recovery.
onset of the syndrome follows a viral or bacterial infection, such as
the following:
Like GBS, CIDP can improve spontaneously. However, recovery
may be very slow and the illness can either get progressively
• Campylobacteriosis (usually from eating undercooked better or worse, or can follow a relapsing/remitting course.
poultry)
• Flu (influenza), common cold Most patients with GBS and CIDP are treated with plasmapheresis
or immunoglobulin. Corticosteroids may be used to treat CIDP but
• Gastrointestinal viral infection
are not used to treat GBS, as it worsens rather than improves the
• HIV condition.
• Infectious mononucleosis
• Porphyria (rare disease of red blood cells) Plasmapheresis
• Viral hepatitis Patients diagnosed early in the course of the disease and those
who are acutely ill often respond well to blood plasma exchange
(plasmapheresis). In this procedure, blood is withdrawn and
A small number of cases have been known to occur after a passed through a series of filters that separate the different types
medical procedure, such as minor surgery. of blood cells. The blood cells are then suspended in donor or
synthetic plasma and returned to the patient's body. The patient's
Guillain-Barre syndrome may be an autoimmune disorder in plasma is discarded.
which the body produces antibodies that damage the myelin
sheath that surrounds peripheral nerves. The myelin sheath is a Plasmapheresis is thought to remove the substances that damage
fatty substance that surrounds axons. It increases the speed at myelin. It can shorten the course of GBS, alleviate symptoms, and
which signals travel along the nerves. prevent paralysis.

GBS Signs and Symptoms Immunoglobulin


Large doses of immunoglobin given intravenously can help shorten
The first symptoms of GBS are usually numbness or tingling the duration of symptoms. This treatment is just as effective as
(paresthesia) in the toes and fingers, with progressive weakness in

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plasmapheresis. It often is preferred to plasmapheresis because it nerves, or even the axons themselves (axons are long, thin
does not require insertion of a large venous catheter. 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.
Overall, about 70% of patients respond to plasmapheresis or
immunoglobin. There is no evidence of additional benefit from
treatment with both procedures. 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
Medications
the brain's commands, commands that must be carried through
Muscle and joint pain can be treated with over-the-counter
the nerve network. The brain also receives fewer sensory signals
analgesics such as aspirin. If necessary, stronger pain medication
from the rest of the body, resulting in an inability to feel textures,
(e.g., acetaminophen with hydrocodone) may be prescribed.
heat, pain, and other sensations. Alternately, the brain may
Muscle spasms can be controlled with relaxants such as diazepam
receive inappropriate signals that result in tingling, "crawling-
(Valium®).
skin," or painful sensations. Because the signals to and from the
arms and legs must travel the longest distances they are most
Unpleasant sensation problems, such as painful tingling, can be vulnerable to interruption. Therefore, muscle weakness and
treated with tricyclic antidepressants or anticonvulsants such as tingling sensations usually first appear in the hands and feet and
gabapentin (Neurontin®). progress upwards.

Corticosteroids, which often effectively treat the symptoms of When Guillain-Barré is preceded by a viral or bacterial infection, it
autoimmune disorders, actually worsen Guillain-Barre syndrome is possible that the virus has changed the nature of cells in the
and should not be used. However, they often are used to treat nervous system so that the immune system treats them as foreign
CIDP. 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
Physical therapy
lymphocytes and macrophages, to attack the myelin. Sensitized T
Before recovery begins, caregivers move the patient's arms and
lymphocytes cooperate with B lymphocytes to produce antibodies
legs to prevent stiffness. After symptoms subside, the
against components of the myelin sheath and may contribute to
rehabilitation team will prescribe an active exercise routine to help
destruction of the myelin. Scientists are investigating these and
regain muscle strength and independence. Training with adaptive
other possibilities to find why the immune system goes awry in
devices, such as a wheelchair or braces, give the patient mobility.
Guillain-Barré syndrome and other autoimmune diseases. The
cause and course of Guillain-Barré syndrome is an active area of
Hydrotherapy neurological investigation, incorporating the cooperative efforts of
Whirlpool therapy (hydrotherapy) may help relieve pain and be neurological scientists, immunologists, and virologists.
useful in retraining the movement of affected limbs.
What are symptoms of Guillain-Barré syndrome?
Counseling
Counseling often is suggested to reassure patients diagnosed with
GBS or CIDP and to help them feel positive about their treatment Symptoms of Guillain-Barré Syndrome include weakness, typically
and recovery. beginning in the legs and progressing upward. The weakness is
accompanied by decreased feeling (paresthesia). Reflexes are
lost, for example, the hammer to the front of the knee will not
GBS Prognosis induce a kick. In severe cases breathing can be affected enough to
require a ventilator and rarely the heart can be affected. The
Patients who have Guillain-Barre syndrome may remain in the maximal degree of weakness usually occurs within the first 2-3
hospital for several months and recovery may take as long as a weeks.
year or more. Most patients with GBS recover completely, but
some have residual weakness, numbness, and occasional pain.A After the first clinical manifestations of the disease, the symptoms
small number of patients are unable to resume their normal daily can progress over the course of hours, days, or weeks.
activities or occupation.
How is Guillain-Barré syndrome diagnosed?
Fewer than 5% of GBS patients die. Those fatalities usually result
from cardiovascular or respiratory complications. Death resulting
from chronic inflammatory demyelinating polyradicalneuropathy Guillain-Barré is called a syndrome rather than a disease because
(CIDP) is rare. 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
What is Guillain-Barré syndrome? observe or measure). The signs and symptoms of the syndrome
can be quite varied, so doctors may, on rare occasions, find it
Guillain-Barré syndrome (often misspelled Guillain-Barre) is a difficult to diagnose Guillain-Barré in its earliest stages.
disorder in which the body's immune system attacks part of the
peripheral nervous system. The first symptoms of this disorder What is the long-term outlook for those with Guillain-Barré
include varying degrees of weakness or tingling sensations in the syndrome?
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 Guillain-Barré syndrome can be a devastating disorder because of
severe, the patient is almost totally paralyzed. In these cases the its sudden and unexpected onset. In addition, recovery is not
disorder is life threatening - potentially interfering with breathing necessarily quick. As noted above, patients usually reach the point
and, at times, with blood pressure or heart rate - and is considered of greatest weakness or paralysis days or weeks after the first
a medical emergency. Such a patient is often put on a respirator symptoms occur. Symptoms then stabilize at this level for a period
to assist with breathing and is watched closely for problems such of days, weeks, or, sometimes, months. The recovery period may
as an abnormal heart beat, infections, blood clots, and high or low be as little as a few weeks or as long as a few years. About 30
blood pressure. Most patients, however, recover from even the percent of those with Guillain-Barré still have a residual weakness
most severe cases of Guillain-Barré syndrome, although some after 3 years. About 3 percent may suffer a relapse of muscle
continue to have a certain degree of weakness. weakness and tingling sensations many years after the initial
attack.
What causes Guillain-Barré syndrome?
Guillain-Barré syndrome patients face not only physical difficulties,
but emotionally painful periods as well. It is often extremely
No one yet knows why Guillain-Barré - which is not contagious - difficult for patients to adjust to sudden paralysis and dependence
strikes some people and not others. Nor does anyone know exactly on others for help with routine daily activities. Patients sometimes
what sets the disease in motion. need psychological counseling to help them adapt.

What scientists do know is that the body's immune system begins What research is being done on Guillain-Barré syndrome?
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 Scientists are concentrating on finding new treatments and
syndrome, however, the immune system starts to destroy the refining existing ones. Scientists are also looking at the workings
myelin sheath that surrounds the axons of many peripheral of the immune system to find which cells are responsible for
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beginning and carrying out the attack on the nervous system. The require ventilatory assistance.[11] Facial weakness is also
fact that so many cases of Guillain-Barré begin after a viral or commonly a feature, but eye movement abnormalities are not
bacterial infection suggests that certain characteristics of some commonly seen in ascending GBS, but are a prominent feature in
viruses and bacteria may activate the immune system the Miller-Fisher variant (see below.) Sensory loss, if present,
inappropriately. Investigators are searching for those usually takes the form of loss of proprioception (position sense)
characteristics. Certain proteins or peptides in viruses and bacteria and areflexia (complete loss of deep tendon reflexes), an
may be the same as those found in myelin, and the generation of important feature of GBS. Loss of pain and temperature sensation
antibodies to neutralize the invading viruses or bacteria could is usually mild. In fact, pain is a common symptom in GBS,
trigger the attack on the myelin sheath. As noted previously, presenting as deep aching pain, usually in the weakened muscles,
neurological scientists, immunologists, virologists, and which patients compare to the pain from overexercising. These
pharmacologists are all working collaboratively to learn how to pains are self-limited and should be treated with standard
prevent this disorder and to make better therapies available when analgesics. Bladder dysfunction may occur in severe cases but
it strikes. should be transient. If severe, spinal cord disorder should be
suspected.
Guillain–Barré syndrome (GBS) (French pronunciation: [ɡiˈjɛ̃ ba
ˈʁe];[1][2] in English, pronounced /ˈɡiːlæn ˈbɑreɪ/,[3] /ɡiːˈlæn bəˈreɪ/,[4] [edit] Influenza vaccine
etc.[5] [pronounced ghee-YAN bah-RAY]) is an acute inflammatory
demyelinating polyneuropathy (AIDP), an autoimmune disorder
affecting the peripheral nervous system, usually triggered by an GBS may be a rare side-effect of influenza vaccines; a study of the
acute infectious process. The syndrome was named after the Vaccine Adverse Event Reporting System (VAERS) indicates that it
French physicians Guillain, Barré and Strohl, who were the first to is reported as an adverse event potentially associated with the
describe it in 1916. It is sometimes called Landry's paralysis, after vaccine at a rate of 1 per million vaccines (over the normal risk).[16]
the French physician who first described a variant of it in 1859. It There were reports of GBS affecting 10 per million who had
is included in the wider group of peripheral neuropathies. There received swine flu immunizations in the 1976 U.S. outbreak of
are several types of GBS, but unless otherwise stated, GBS refers swine flu—25 of which resulted in death from severe pulmonary
to the most common form, AIDP. GBS is rare and has an incidence complications, leading the government to end that immunization
of 1 or 2 people per 100,000.[6] It is frequently severe and usually campaign (By comparison, the average flu season kills around
exhibits as an ascending paralysis noted by weakness in the legs 30,000 people in the United States).[17] However, the role of the
that spreads to the upper limbs and the face along with complete vaccine in these cases has remained unclear, partly because GBS
loss of deep tendon reflexes. With prompt treatment by had an unknown but very low incidence rate in the general
plasmapheresis or intravenous immunoglobulins and supportive population making it difficult to assess whether the vaccine was
care, the majority of patients will regain full functional capacity. really increasing the risk for GBS. Later research has pointed to
However, death may occur if severe pulmonary complications and the absence of or only a very small increase in the GBS risk due to
autonomic nervous system problems are present.[7] Guillain-Barré the 1976 swine flu vaccine.[18] Furthermore, the GBS may not have
is one of the leading causes of non-trauma-induced paralysis in been directly due to the vaccine but to a bacterial contamination
the world.[citation needed] of the vaccine.[19]

Since 1976, no other influenza vaccines have been linked to GBS,


• Acute inflammatory demyelinating polyneuropathy though as a precautionary principle, caution is advised for certain
(AIDP) is the most common form of GBS, and the term is individuals, particularly those with a history of GBS.[20][21] On the
often used synonymously with GBS. It is caused by an other hand, getting infected by the flu increases both the risk of
auto-immune response directed against Schwann cell death (up to 1 in 10,000) and the risk of developing GBS to a much
membranes. higher level (approx. 10 times higher by recent estimates[22]).R,
• Miller Fisher syndrome (MFS) is a rare variant of GBS Sharshar T, Durand MC, Enouf V, et al. Guillain-Barré syndrome
and manifests as a descending paralysis, proceeding in and influenza virus infection. Clin Infect Dis 2009;48:48-56.</ref>
the reverse order of the more common form of GBS. It
usually affects the eye muscles first and presents with
[edit] Prognosis
the triad of ophthalmoplegia, ataxia, and areflexia. Anti-
GQ1b antibodies are present in 90% of cases.
• Acute motor axonal neuropathy (AMAN),[8] aka Most of the time recovery starts after the fourth week from the
Chinese Paralytic Syndrome, attacks motor nodes of onset of the disorder. Approximately 80% of patients have a
Ranvier and is prevalent in China and Mexico. It is complete recovery within a few months to a year, although minor
probably due to an auto-immune response directed findings may persist, such as areflexia. About 5–10% recover with
against the axoplasm of peripheral nerves. The disease severe disability, with most of such cases involving severe
may be seasonal and recovery can be rapid. Anti-GD1a proximal motor and sensory axonal damage with inability of
antibodies[9] are present. Anti-GD3 antibodies are found axonal regeneration. However, this is a grave disorder and despite
more frequently in AMAN. all improvements in treatment and supportive care, the death rate
among patients with this disorder is still about 2–3% even in the
• Acute motor sensory axonal neuropathy (AMSAN) is
best intensive care units. Worldwide, the death rate runs slightly
similar to AMAN but also affects sensory nerves with
higher (4%), mostly from a lack of availability of life support
severe axonal damage. Like AMAN, it is probably due to
equipment during the lengthy plateau lasting four to six weeks,
an auto-immune response directed against the axoplasm
and in some cases up to one year, when a ventilator is needed in
of peripheral nerves. Recovery is slow and often
the worst cases. About 5–10% of patients have one or more late
incomplete.[10]
relapses, in which case they are then classified as having chronic
• Bickerstaff’s brainstem encephalitis (BBE), is a inflammatory demyelinating polyneuropathy (CIDP).
further variant of Guillain–Barré syndrome. It is
characterized by acute onset of ophthalmoplegia, ataxia,
disturbance of consciousness, hyperreflexia or Babinski’s Poor prognostic factors include: 1) age >40 years, 2) history of
sign (Bickerstaff, 1957; Al-Din et al.,1982). The course of preceding diarrheal illness, 3) requiring ventilator support, 4) high
the disease can be monophasic or remitting-relapsing. anti-GM1 titre and 5) poor upper limb muscle strength.
Large, irregular hyperintense lesions located mainly in
the brainstem, especially in the pons, midbrain and Case reports do exist of rapid patient recovery.
medulla are described in the literature. BBE despite
severe initial presentation usually has a good prognosis.
Magnetic resonance imaging (MRI) plays a critical role in History
the diagnosis of BBE.
The disorder was first described by the French physician Jean
Signs and symptoms Landry in 1859. In 1916, Georges Guillain, Jean Alexandre Barré,
and André Strohl diagnosed two soldiers with the illness and
discovered the key diagnostic abnormality of increased spinal fluid
The disorder is characterized by symmetrical weakness which protein production, but normal cell count.[29]
usually affects the lower limbs first, and rapidly progresses in an
ascending fashion. Patients generally notice weakness in their
legs, manifesting as "rubbery legs" or legs that tend to buckle, GBS is also known as acute inflammatory demyelinating
with or without dysesthesias (numbness or tingling). As the polyneuropathy, acute idiopathic polyradiculoneuritis, acute
weakness progresses upward, usually over periods of hours to idiopathic polyneuritis, French Polio, Landry's ascending paralysis
days, the arms and facial muscles also become affected. and Landry Guillain Barré syndrome.
Frequently, the lower cranial nerves may be affected, leading to
bulbar weakness, oropharyngeal dysphagia (drooling, or difficulty The acute immune-mediated polyneuropathies are classified under
swallowing and/or maintaining an open airway) and respiratory the eponym Guillain-Barré syndrome (GBS), after the authors of
difficulties. Most patients require hospitalization and about 30%
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early descriptions of the disease. GBS is an acute monophasic
paralyzing illness usually provoked by a preceding infection.

• Supportive care is extremely important in Guillain-Barré


syndrome (GBS) since about 30 percent of patients
develop neuromuscular respiratory failure requiring
mechanical ventilation. In addition, severe autonomic
dysfunction occurs in about 20 percent and warrants
intensive care unit (ICU) monitoring.
• Close respiratory monitoring with frequent measurement
of vital capacity and negative inspiratory force should be
instituted in all patients with GBS on admission and
continued while weakness is progressing. Patients with a
forced vital capacity <20 mL/kg, maximum inspiratory
pressure <30 cmH2O, or maximum expiratory pressure
<40 cmH2O are at high risk of impending respiratory
failure and should undergo urgent intubation and
mechanical ventilation.
• For patients with GBS and progressive weakness, we
recommend cardiac and blood pressure monitoring.
• For patients with GBS and progressive weakness, we
recommend daily abdominal auscultation to monitor for
bowel silence and the development of adynamic ileus.
• Neuropathic pain occurs in about 40 to 50 percent of
patients during the course of GBS and often requires
treatment.
• In the absence of disease-modifying treatment, most
patients with GBS show continued progression for up to
two weeks, followed by a plateau phase of about two
weeks, and then recovery of function over several weeks
to months.
• The main modalities of disease modifying therapy for GBS
are plasma exchange and intravenous immune globulin
(IVIG). The treatments are equivalent and improve
outcome. Treatment shortens the time to walking
independently by 40 to 50 percent.
• For nonambulatory adult patients with GBS who are
within four weeks of neuropathic symptom onset, we
recommend treatment with plasma exchange or IVIG
(Grade 1A). For ambulatory adult patients with GBS who
are not yet recovering within four weeks of neuropathic
symptom onset, we suggest treatment with plasma
exchange or IVIG (Grade 2B). The choice between
plasma exchange and IVIG is dependent on local
availability and on patient preference, risk factors, and
contraindications. When both therapies are equally
available and there are no contraindications for either, we
suggest treatment with IVIG (Grade 2B).
• For adult patients with GBS, we recommend NOT treating
with glucocorticoids (Grade 1A).
• Even with treatment, approximately 5 to 10 percent of
patients have a prolonged course with very delayed and
incomplete recovery, and 5 percent die despite intensive
care. In addition, relapses occur in up to 10 percent of
patients. (See "Clinical course" above).

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