Definition
Some patients
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.
These sensations can quickly spread, eventually paralyzing your
whole body.
The exact cause of Guillain-Barre syndrome is unknown, but it is
often preceded by an infectious illness such as a respiratory
infection or the stomach flu. Luckily, Guillain-Barre syndrome is
relatively rare, affecting only 1 or 2 people per 100,000.
In its most severe form, Guillain-Barre syndrome is a medical
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
do recover completely.
Cause
The exact cause of Guillain-Barre syndrome is unknown. In about
60 percent of cases, an infection affecting either the lungs or the
digestive tract precedes the disorder. But scientists don't know
why such an infection can lead to Guillain-Barre syndrome for
some people and not for others. Many cases appear to occur
without any triggers.
In Guillain-Barre syndrome, your immune system — which usually
only attacks foreign material and invading organisms — begins
attacking the nerves that carry signals between your body and
your brain. Specifically, the nerves' protective covering (myelin
sheath) is damaged and this interferes with the signaling process,
causing weakness, numbness or paralysis.
Overview
Guillain-Barre syndrome (GBS) is an inflammatory disorder of the
peripheral nerves. The peripheral nerves convey sensory
information (e.g., pain, temperature) from the body to the brain
and motor (i.e., movement) signals from the brain to the body.
GBS is characterized by weakness and numbness or tingling in the
legs and arms, and possible loss of movement and feeling in the
legs, arms, upper body, and face.
Chronic inflammatory demyelinating polyradicalneuropathy
(CIDP), is considered to be a related form of Guillain-Barre
syndrome. It is much less common than GBS, and evolves much
more slowly and usually is longer lasting. Some CIDP patients
experience periods of worsening and improvement, and individual
relapses can be confused with GBS.
GBS Causes
Guillain-Barre syndrome is not hereditary or contagious. What
causes GBS is not known; however, in about half of all cases the
onset of the syndrome follows a viral or bacterial infection, such as
the following:
• Campylobacteriosis (usually from eating undercooked
poultry)
• Flu (influenza), common cold
• Gastrointestinal viral infection
• HIV
• Infectious mononucleosis
• Porphyria (rare disease of red blood cells)
• Viral hepatitis
A small number of cases have been known to occur after a
medical procedure, such as minor surgery.
Guillain-Barre syndrome may be an autoimmune disorder in
which the body produces antibodies that damage the myelin
sheath that surrounds peripheral nerves. The myelin sheath is a
fatty substance that surrounds axons. It increases the speed at
which signals travel along the nerves.
GBS Signs and Symptoms
The first symptoms of GBS are usually numbness or tingling
(paresthesia) in the toes and fingers, with progressive weakness in
the arms and legs over the next few days.
experience paresthesia only in their toes and legs; others only
experience symptoms on one side of the body.
The symptoms may stay in this phase, causing only mild difficulty
in walking, requiring crutches or a walking stick. However,
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 patient dependent on a ventilator. If the swallowing muscles
are also affected, a feeding tube may be needed.
In chronic inflammatory demyelinating polyradicalneuropathy
(CIDP), the course of illness is longer and respiratory failure is
much more unlikely.
GBS Diagnosis
Because its symptoms vary and its cause is unknown, GBS can be
difficult to diagnose. If the symptoms occur uniformly across the
body and progress rapidly, the diagnosis is easier.
Observation of the patient's symptoms and an evaluation of the
medical history provide the basis for diagnosis of Guillain-Barre
syndrome, although no single observation is suitable to make the
diagnosis.
Tests
Three tests can confirm a diagnosis of Guillain-Barre syndrome.
Lumbar puncture (spinal tap)—The patient is given local
anesthetic. Once the anesthetic has taken effect, a needle is
inserted between two lower (lumbar) vertebrae and a sample of
cerebrospinal fluid is drawn. An elevated level of protein without
an increase in the number of white blood cells (WBCs) in the fluid
is characteristic of GBS.
Electromyogram (EMG)—This is an effective diagnostic tool
because it records muscle activity and can show the loss of
individual nerve impulses due to the disease's characteristic
slowing of nerve responses.
Nerve conduction velocity (NCV)—This test is performed with
EMG, and together, they are often referred to as EMG/NCV
studies. NCV records the speed at which signals travel along the
nerves. These signals are characteristically slowed in GBS,
although the findings may evolve over several weeks.
Treatment
GBS is considered a medical emergency and most patients are
admitted to the hospital soon after diagnosis. If the patient's
breathing seems to be at risk, he or she is usually managed in an
intensive care unit (ICU). Although GBS can improve
spontaneously, there are a number of treatments that facilitate
recovery.
Like GBS, CIDP can improve spontaneously. However, recovery
may be very slow and the illness can either get progressively
better or worse, or can follow a relapsing/remitting course.
Most patients with GBS and CIDP are treated with plasmapheresis
or immunoglobulin. Corticosteroids may be used to treat CIDP but
are not used to treat GBS, as it worsens rather than improves the
condition.
Plasmapheresis
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
passed through a series of filters that separate the different types
of blood cells. The blood cells are then suspended in donor or
synthetic plasma and returned to the patient's body. The patient's
plasma is discarded.
Plasmapheresis is thought to remove the substances that damage
myelin. It can shorten the course of GBS, alleviate symptoms, and
prevent paralysis.
Immunoglobulin
Large doses of immunoglobin given intravenously can help shorten
the duration of symptoms. This treatment is just as effective as
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plasmapheresis. It often is preferred to plasmapheresis because it
does not require insertion of a large venous catheter.
Overall, about 70% of patients respond to plasmapheresis or
immunoglobin. There is no evidence of additional benefit from
treatment with both procedures.
Medications
Muscle and joint pain can be treated with over-the-counter
analgesics such as aspirin. If necessary, stronger pain medication
(e.g., acetaminophen with hydrocodone) may be prescribed.
Muscle spasms can be controlled with relaxants such as diazepam
(Valium®).
Unpleasant sensation problems, such as painful tingling, can be
treated with tricyclic antidepressants or anticonvulsants such as
gabapentin (Neurontin®).
Corticosteroids, which often effectively treat the symptoms of
autoimmune disorders, actually worsen Guillain-Barre syndrome
and should not be used. However, they often are used to treat
CIDP.
Physical therapy
Before recovery begins, caregivers move the patient's arms and
legs to prevent stiffness. After symptoms subside, the
rehabilitation team will prescribe an active exercise routine to help
regain muscle strength and independence. Training with adaptive
devices, such as a wheelchair or braces, give the patient mobility.
Hydrotherapy
Whirlpool therapy (hydrotherapy) may help relieve pain and be
useful in retraining the movement of affected limbs.
Counseling
Counseling often is suggested to reassure patients diagnosed with
GBS or CIDP and to help them feel positive about their treatment
and recovery.
GBS Prognosis
Patients who have Guillain-Barre syndrome may remain in the
hospital for several months and recovery may take as long as a
year or more. Most patients with GBS recover completely, but
some have residual weakness, numbness, and occasional pain.A
small number of patients are unable to resume their normal daily
activities or occupation.
Fewer than 5% of GBS patients die. Those fatalities usually result
from cardiovascular or respiratory complications. Death resulting
from chronic inflammatory demyelinating polyradicalneuropathy
(CIDP) is rare.
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.
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.
What are symptoms of Guillain-Barré syndrome?
Symptoms of Guillain-Barré Syndrome include weakness, typically
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
induce a kick. In severe cases breathing can be affected enough to
require a ventilator and rarely the heart can be affected. The
maximal degree of weakness usually occurs within the first 2-3
weeks.
After the first clinical manifestations of the disease, the symptoms
can progress over the course of hours, days, or weeks.
How is Guillain-Barré syndrome diagnosed?
Guillain-Barré 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-Barré in its earliest stages.
What is the long-term outlook for those with Guillain-Barré
syndrome?
Guillain-Barré 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-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.
Guillain-Barré 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.
What research is being done on Guillain-Barré syndrome?
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
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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. 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.
Guillain–Barré syndrome (GBS) (French pronunciation: [ɡiˈjɛ̃ ba
ˈʁe];[1][2] in English, pronounced /ˈɡiːlæn ˈbɑreɪ/,[3] /ɡiːˈlæn bəˈreɪ/,[4]
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
acute infectious process. The syndrome was named after the
French physicians Guillain, Barré and Strohl, who were the first to
describe it in 1916. It is sometimes called Landry's paralysis, after
the French physician who first described a variant of it in 1859. It
is included in the wider group of peripheral neuropathies. There
are several types of GBS, but unless otherwise stated, GBS refers
to the most common form, AIDP. GBS is rare and has an incidence
of 1 or 2 people per 100,000.[6] It is frequently severe and usually
exhibits as an ascending paralysis noted by weakness in the legs
that spreads to the upper limbs and the face along with complete
loss of deep tendon reflexes. With prompt treatment by
plasmapheresis or intravenous immunoglobulins and supportive
care, the majority of patients will regain full functional capacity.
However, death may occur if severe pulmonary complications and
autonomic nervous system problems are present.[7] Guillain-Barré
is one of the leading causes of non-trauma-induced paralysis in
the world.[citation needed]
• Acute inflammatory demyelinating polyneuropathy
(AIDP) is the most common form of GBS, and the term is
often used synonymously with GBS. It is caused by an
auto-immune response directed against Schwann cell
membranes.
• Miller Fisher syndrome (MFS) is a rare variant of GBS
and manifests as a descending paralysis, proceeding in
the reverse order of the more common form of GBS. It
usually affects the eye muscles first and presents with
the triad of ophthalmoplegia, ataxia, and areflexia. Anti-
GQ1b antibodies are present in 90% of cases.
• Acute motor axonal neuropathy (AMAN),[8] aka
Chinese Paralytic Syndrome, attacks motor nodes of
Ranvier and is prevalent in China and Mexico. It is
probably due to an auto-immune response directed
against the axoplasm of peripheral nerves. The disease
may be seasonal and recovery can be rapid. Anti-GD1a
antibodies[9] are present. Anti-GD3 antibodies are found
more frequently in AMAN.
• Acute motor sensory axonal neuropathy (AMSAN) is
similar to AMAN but also affects sensory nerves with
severe axonal damage. Like AMAN, it is probably due to
an auto-immune response directed against the axoplasm
of peripheral nerves. Recovery is slow and often
incomplete.[10]
• Bickerstaff’s brainstem encephalitis (BBE), is a
further variant of Guillain–Barré syndrome. It is
characterized by acute onset of ophthalmoplegia, ataxia,
disturbance of consciousness, hyperreflexia or Babinski’s
sign (Bickerstaff, 1957; Al-Din et al.,1982). The course of
the disease can be monophasic or remitting-relapsing.
Large, irregular hyperintense lesions located mainly in
the brainstem, especially in the pons, midbrain and
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
the diagnosis of BBE.
Signs and symptoms
The disorder is characterized by symmetrical weakness which
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,
with or without dysesthesias (numbness or tingling). As the
weakness progresses upward, usually over periods of hours to
days, the arms and facial muscles also become affected.
Frequently, the lower cranial nerves may be affected, leading to
bulbar weakness, oropharyngeal dysphagia (drooling, or difficulty
swallowing and/or maintaining an open airway) and respiratory
difficulties. Most patients require hospitalization and about 30%
require ventilatory assistance.[11] Facial weakness is also
commonly a feature, but eye movement abnormalities are not
commonly seen in ascending GBS, but are a prominent feature in
the Miller-Fisher variant (see below.) Sensory loss, if present,
usually takes the form of loss of proprioception (position sense)
and areflexia (complete loss of deep tendon reflexes), an
important feature of GBS. Loss of pain and temperature sensation
is usually mild. In fact, pain is a common symptom in GBS,
presenting as deep aching pain, usually in the weakened muscles,
which patients compare to the pain from overexercising. These
pains are self-limited and should be treated with standard
analgesics. Bladder dysfunction may occur in severe cases but
should be transient. If severe, spinal cord disorder should be
suspected.
[edit] Influenza vaccine
GBS may be a rare side-effect of influenza vaccines; a study of the
Vaccine Adverse Event Reporting System (VAERS) indicates that it
is reported as an adverse event potentially associated with the
vaccine at a rate of 1 per million vaccines (over the normal risk).[16]
There were reports of GBS affecting 10 per million who had
received swine flu immunizations in the 1976 U.S. outbreak of
swine flu—25 of which resulted in death from severe pulmonary
complications, leading the government to end that immunization
campaign (By comparison, the average flu season kills around
30,000 people in the United States).[17] However, the role of the
vaccine in these cases has remained unclear, partly because GBS
had an unknown but very low incidence rate in the general
population making it difficult to assess whether the vaccine was
really increasing the risk for GBS. Later research has pointed to
the absence of or only a very small increase in the GBS risk due to
the 1976 swine flu vaccine.[18] Furthermore, the GBS may not have
been directly due to the vaccine but to a bacterial contamination
of the vaccine.[19]
Since 1976, no other influenza vaccines have been linked to GBS,
though as a precautionary principle, caution is advised for certain
individuals, particularly those with a history of GBS.[20][21] On the
other hand, getting infected by the flu increases both the risk of
death (up to 1 in 10,000) and the risk of developing GBS to a much
higher level (approx. 10 times higher by recent estimates[22]).R,
Sharshar T, Durand MC, Enouf V, et al. Guillain-Barré syndrome
and influenza virus infection. Clin Infect Dis 2009;48:48-56.</ref>
[edit] Prognosis
Most of the time recovery starts after the fourth week from the
onset of the disorder. Approximately 80% of patients have a
complete recovery within a few months to a year, although minor
findings may persist, such as areflexia. About 5–10% recover with
severe disability, with most of such cases involving severe
proximal motor and sensory axonal damage with inability of
axonal regeneration. However, this is a grave disorder and despite
all improvements in treatment and supportive care, the death rate
among patients with this disorder is still about 2–3% even in the
best intensive care units. Worldwide, the death rate runs slightly
higher (4%), mostly from a lack of availability of life support
equipment during the lengthy plateau lasting four to six weeks,
and in some cases up to one year, when a ventilator is needed in
the worst cases. About 5–10% of patients have one or more late
relapses, in which case they are then classified as having chronic
inflammatory demyelinating polyneuropathy (CIDP).
Poor prognostic factors include: 1) age >40 years, 2) history of
preceding diarrheal illness, 3) requiring ventilator support, 4) high
anti-GM1 titre and 5) poor upper limb muscle strength.
Case reports do exist of rapid patient recovery.
History
The disorder was first described by the French physician Jean
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
protein production, but normal cell count.[29]
GBS is also known as acute inflammatory demyelinating
polyneuropathy, acute idiopathic polyradiculoneuritis, acute
idiopathic polyneuritis, French Polio, Landry's ascending paralysis
and Landry Guillain Barré syndrome.
The acute immune-mediated polyneuropathies are classified under
the eponym Guillain-Barré syndrome (GBS), after the authors of
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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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