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Guillain-Barré syndrome

Guillain-Barré 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-Barré syndrome, although some
continue to have a certain degree of weakness. Guillain-Barré 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-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. After the first clinical manifestations of the disease, the symptoms can progress over the course
of hours, days, or 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.

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.

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.

Several disorders have symptoms similar to those found in Guillain-Barré, 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-Barré from other disorders. For example, physicians will note whether the symptoms appear on both
sides of the body (most common in Guillain-Barré) and the quickness with which the symptoms appear (in other disorders,
muscle weakness may progress over months rather than days or weeks). In Guillain-Barré, 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-Barré 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.

How is Guillain-Barré treated?


There is no known cure for Guillain-Barré 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, plasma exchange (sometimes called plasmapheresis) and high-dose immunoglobulin therapy are used. Both of
them are equally effective, but immunoglobulin is easier to administer. Plasma exchange 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 plasma exchange works, but the technique seems to reduce the severity and duration
of the Guillain-Barré 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-Barré 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-Barré, 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-Barré 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-Barré
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.

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

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. 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 baˈʁe];[1][2] in English, pronounced /ˈɡiːlæn ˈbɑreɪ/,[3] /ɡiːˈlæn bəˈreɪ/,[4] etc[5]) 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.

Six different subtypes of Guillain-Barré syndrome (GBS) exist:

• 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
membrane
• 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 likely 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 likely due to an auto-immune response directed against the axoplasm of
peripheral nerves. Recovery is slow and often incomplete.[10]
• Acute panautonomic neuropathy is the most rare variant of GBS, sometimes accompanied by encephalopathy.
It is associated with a high mortality rate, due to cardiovascular involvement, and associated dysrhythmias.
Impaired sweating, lack of tear formation, photophobia, dryness of nasal and oral mucosa, itching and peeling of
skin, nausea, dysphagia, constipation unrelieved by laxatives or alternating with diarrhea occur frequently in this
patient group. Initial nonspecific symptoms of lethargy, fatigue, headache, and decreased initiative are followed by
autonomic symptoms including orthostatic lightheadedness, blurring of vision, abdominal pain, diarrhea, dryness of
eyes, and disturbed micturition. The most common symptoms at onset are related to orthostatic intolerance, as
well as gastrointestinal and sudomotor dysfunction (Suarez et al. 1994). Parasympathetic impairment (abdominal
pain, vomiting, obstipation, ileus, urinary retention, dilated unreactive pupils, loss of accommodation) may also be
observed.
• 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.

A considerable number of BBE patients have associated axonal Guillain-Barré syndrome, indicative that the two disorders
are closely related and form a continuous spectrum.

[edit] 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, that is difficulty with swallowing, drooling, 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.

Fever should not be present, and if it is, another cause should be suspected.

In severe cases of GBS, loss of autonomic function is common, manifesting as wide fluctuations in blood pressure,
orthostatic hypotension, and cardiac arrhythmias.

Acute paralysis in Guillain-Barré syndrome may be related to sodium channel blocking factor in the cerebrospinal fluid (CSF).
Significant issues involving intravenous salt and water administration may occur unpredictably in this patient group,
resulting in SIADH.
The symptoms are similar to those for progressive inflammatory neuropathy.[12]

Cause

All forms of Guillain-Barré syndrome are due to an immune response to foreign antigens (such as infectious agents) that are
mistargeted at host nerve tissues instead. The targets of such immune attack are thought to be gangliosides, compounds
naturally present in large quantities in human nerve tissues. The most common antecedent infection is the bacteria
Campylobacter jejuni.[13] However, 60% of cases do not have a known cause; one study suggests that some cases are
triggered by the influenza virus, or by an immune reaction to the influenza virus.[14]The end result of such autoimmune
attack on the peripheral nerves is damage to the myelin, the fatty insulating layer of the nerve, and a nerve conduction
block, leading to a muscle paralysis that may be accompanied by sensory or autonomic disturbances.However, in mild
cases, nerve axon (the long slender conducting portion of a nerve) function remains intact and recovery can be rapid if
remyelination occurs. In severe cases, axonal damage occurs, and recovery depends on the regeneration of this important
tissue. Recent studies on the disorder have demonstrated that approximately 80% of the patients have myelin loss,
whereas, in the remaining 20%, the pathologic hallmark of the disorder is indeed axon loss.

Guillain-Barré, unlike disorders such as multiple sclerosis (MS) and Lou Gehrig's disease (ALS), is a peripheral nerve disorder
and does not generally cause nerve damage to the brain or spinal cord.

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 about 1 per million
vaccines.[15] There were reports of GBS affecting about 500 people 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.[16] 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.[17] Furthermore, the GBS may not have been directly due to the vaccine but to a
bacterial contamination of the vaccine.[18]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.[19][20] On the other
hand, getting infected by the flu increases the risk of developing GBS to a much higher level (approx. 10 times higher by
recent estimates[21]) and, all in all, the flu vaccination contributes protection against the risk of GBS.[22]From October 6 to
November 24, 2009, the U.S. CDC, through the VAERS reporting system, received ten reports of Guillain-Barre syndrome
cases associated with the H1N1 vaccine and identified two additional probable cases from VAERS reports (46.2 million doses
were distributed within the U.S. during this time). Only four cases, however, meet the Brighton Collaboration Criteria for
Guillain-Barré syndrome, while four do not meet the criteria and four remain under review.[23]

Diagnosis

The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis, areflexia, absence of
fever, and a likely inciting event. Cerebrospinal fluid analysis (through a lumbar spinal puncture) and electrodiagnostic tests
of nerves and muscles (such as nerve conduction studies) are common tests ordered in the diagnosis of GBS.

• cerebrospinal fluid

Typical CSF findings include albumino-cytological dissociation. As opposed to infectious causes, this is an elevated
protein level (100–1000 mg/dL), without an accompanying increased cell count pleocytosis. A sustained increased
white blood cell count may indicate an alternative diagnosis such as infection.

• Electrodiagnostics

Electromyography (EMG) and nerve conduction study (NCS) may show prolonged distal latencies, conduction
slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. In primary
axonal damage, the findings include reduced amplitude of the action potentials without conduction slowing.

Diagnostic criteria

Required

• Progressive, relatively symmetrical weakness of two or more limbs due to neuropathy


• Areflexia
• Disorder course < 4 weeks
• Exclusion of other causes (see below)

Supportive

• relatively symmetric weakness accompanied by numbness and/or tingling


• mild sensory involvement
• facial nerve or other cranial nerve involvement
• absence of fever
• typical CSF findings obtained from lumbar puncture
• electrophysiologic evidence of demyelination from electromyogram

Differential diagnosis

• acute myelopathies with chronic back pain and sphincter dysfunction


• botulism with early loss of pupillary reactivity
• diphtheria with early oropharyngeal dysfunction
• Lyme disease polyradiculitis and other tick-borne paralyses
• porphyria with abdominal pain, seizures, psychosis
• vasculitis neuropathy
• poliomyelitis with fever and meningeal signs
• CMV polyradiculitis in immunocompromised patients
• critical illness neuropathy
• myasthenia gravis
• poisonings with organophosphate, poison hemlock, thallium, or arsenic
• paresis caused by West Nile virus
• spinal astrocytoma
• Motor Neurone Disease
• West Nile virus can cause severe, potentially fatal neurological illnesses, which include encephalitis, meningitis,
Guillain-Barre syndrome, and anterior myelitis.
• Severe Myalgic Encephalomyelitis/Chronic Fatigue Syndrome.

Management

Supportive care with monitoring of all vital functions is the cornerstone of successful management in the
acute patient. Of greatest concern is respiratory failure due to paralysis of the diaphragm. Early
intubation should be considered in any patient with a vital capacity (VC) <20 ml/kg, a negative
inspiratory force (NIF) <-25 cmH2O, more than 30% decrease in either VC or NIF within 24 hours, rapid
progression of disorder, or autonomic instability.

Once the patient is stabilized, treatment of the underlying condition should be initiated as soon as possible. Either high-dose
intravenous immunoglobulins (IVIg) at 400 mg/kg for 5 days or plasmapheresis can be administered,[24][25] as they are equally
effective and a combination of the two is not significantly better than either alone. Therapy is no longer effective two weeks
after the first motor symptoms appear, so treatment should be instituted as soon as possible. IVIg is usually used first
because of its ease of administration and safety profile, with a total of five daily infusions for a total dose of 2 g/kg body
weight (400 mg/kg each day). The use of intravenous immunoglobulins is not without risk, occasionally causing hepatitis, or
in rare cases, renal failure if used for longer than five days. Glucocorticoids have not been found to be effective in GBS. If
plasmapheresis is chosen, a dose of 40-50 mL/kg plasma exchange (PE) can be administered four times over a
week.Following the acute phase, the patient may also need rehabilitation to regain lost functions. This treatment will focus
on improving ADL (activities of daily living) functions such as brushing teeth, washing, and getting dressed. Depending on
the local structuring on health care, a team of different therapists and nurses will be established according to patient needs.
An occupational therapist can offer equipment (such as wheelchair and special cutlery) to help the patient achieve ADL
independence. A physiotherapist would plan a progressive training program and guide the patient to correct, functional
movement, avoiding harmful compensations which might have a negative effect in the long run. A speech and language
therapist would be essential in the patient regaining speaking and swallowing ability if they were intubated and received a
tracheostomy. The speech and language therapist would also offer advice to the medical team regarding the swallowing
abilities of the patient and would help the patient regain their communication ability pre-dysarthria. There would also be a
doctor, nurse and other team members involved, depending on the needs of the patient. This team contribute their
knowledge to guide the patient towards his or her goals, and it is important that all goals set by the separate team members
are relevant for the patient's own priorities. After rehabilitation the patient should be able to function in his or her own home
and attend necessary training as needed.

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.

Epidemiology

The incidence of GBS during pregnancy is 1.7 cases per 100,000 of the population.[26] The mother will generally
improve with treatment but death of the fetus is a risk. The risk of Guillain-Barré syndrome increases
after delivery, particularly during the first two weeks postpartum. There is evidence of Campylobacter
jejuni as an antecedent infection in approximately 26% of disease cases, requiring special care in the
preparation and handling of food. Congenital and neonatal Guillain–Barré syndrome have also been
reported.[27]

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.[28]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.Guillain-Barre syndrome is a serious disorder that occurs when the body's
defense (immune) system mistakenly attacks part of the nervous system. This leads to nerve inflammation that causes
muscle weakness

Causes

Guillain-Barre syndrome is an autoimmune disorder (the body's immune system attacks itself). Exactly what triggers
Guillain-Barre syndrome is unknown. The syndrome may occur at any age, but is most common in people of both sexes
between ages 30 and 50.It often follows a minor infection, usually a lung infection or gastrointestinal infection. Usually, signs
of the original infection have disappeared before the symptoms of Guillain-Barre begin.Guillain-Barre syndrome causes
inflammation that damages parts of nerves. This nerve damage causes tingling, muscle weakness, and paralysis. The
inflammation usually affects the nerve's covering (myelin sheath). Such damage is called demyelination. Demyelination
slows nerve signaling. Damage to other parts of the nerve can cause the nerve to stop working.

Guillain-Barre syndrome may occur along with viral infections such as:

• AIDS
• Herpes simplex
• Mononucleosis

It may also occur with other medical conditions such as systemic lupus erythematosus or Hodgkin's disease.
Some people may get Guillain-Barre syndrome after a bacterial infection or certain vaccinations (such as rabies and swine
flu). A similar syndrome may occur after surgery, or when critically ill.

Symptoms

Symptoms of Guillain-Barre can get worse very quickly. It may take only a few hours to reach the most severe symptoms,
but weakness increasing over several days is also common.Muscle weakness or the loss of muscle function (paralysis)
affects both sides of the body. In most cases, the muscle weakness starts in the legs and then spreads to the arms. This is
called ascending paralysis.Patients may notice tingling, foot or hand pain, and clumsiness. If the inflammation affects the
nerves to the diaphragm, and there is weakness in those muscles, the person may need breathing assistance.

Typical symptoms include:

• Loss of reflexes in the arms and legs


• Muscle weakness or loss of muscle function (paralysis)
o In mild cases, there may be no weakness or paralysis
o May begin in the arms and legs at the same time
o May get worse over 24 to 72 hours
o May occur in the nerves of the head only
o May start in the arms and move downward
o May start in the feet and legs and move up to the arms and head
• Numbness, decreased sensation
• Sensation changes
• Tenderness or muscle pain (may be a cramp-like pain)
• Uncoordinated movement

Additional symptoms may include:

• Blurred vision
• Clumsiness and falling
• Difficulty moving face muscles
• Muscle contractions
• Palpitations (sensation of feeling heartbeat)

Emergency symptoms (seek immediate medical help):

• Breathing temporarily stops


• Can't take a deep breath
• Difficulty breathing
• Difficulty swallowing
• Drooling
• Fainting
• Feeling light-headed when standing

Exams and Tests

A history of increasing muscle weakness and paralysis may be a sign of Guillain-Barre syndrome, especially if
there was a recent illness.

A medical exam may show muscle weakness and problems with involuntary (autonomic) body functions such as blood
pressure and heart rate. The examination may also show that reflexes, such as the "knee jerk," are decreased or missing.

There may be signs of decreased breathing (caused by paralysis of the breathing muscles).

The following tests may be ordered:


• Cerebrospinal fluid sample ("spinal tap") may have increased levels of protein without an increase in white blood
cells.
• ECG may show heart problems in some cases.
• EMG tests the electrical activity in muscles. It may show that nerves do not react properly to stimulation.
• Nerve conduction velocity test shows that electrical activity along the nerves is slowed or blocked.

Treatment

There is no cure for Guillain-Barre syndrome. However, many treatments are available to help reduce symptoms, treat
complications, and speed up recovery.

When symptoms are severe, the patient will need to go to the hospital for breathing help, treatment, and physical therapy.

A method called plasmapheresis is used to remove proteins, called antibodies, from the blood. The process involves taking
blood from the body, usually from the arm, pumping it into a machine that removes the antibodies, then sending it back into
the body.

High-dose immunoglobulin therapy (IVIg) is another treatment used to reduce the severity and length of Guillain-Barre
symptoms. In this case, the immunoglobulins are added to the blood in large quantity, blocking the antibodies that cause
inflammation.

Other treatments are directed at preventing complications.

• Blood thinners may be used to prevent blood clots.


• If the diaphragm is week, breathing support or even a breathing tube and ventilator may be needed.
• Pain is treated aggressively with anti-inflammatory medicines and narcotics, if needed.
• Proper body positioning or a feeding tube may be used to prevent choking during feeding if the muscles for
swallowing are weak.

Support Groups

Guillain-Barre Syndrome Foundation International - www.gbsfi.com

Outlook (Prognosis)

Recovery can take weeks or years. Most people survive and recover completely. According to the National Institute of
Neurological Disorders and Stroke, about 30% of patients still have some weakness after 3 years. Mild weakness may persist
for some people.

A patient's outcome is most likely to be very good when the symptoms go away within 3 weeks after they first started.

Possible Complications

• Breathing difficulty (respiratory failure)


• Contractures of joints or other deformity
• Deep vein thrombosis (blood clots that form when someone is inactive or confined to bed)
• Increased risk of infections
• Low or unstable blood pressure
• Permanent loss of movement of an area
• Pneumonia
• Sucking food or fluids into the lungs (aspiration)

When to Contact a Medical Professional

Seek immediate medical help if you have any of the following symptoms:
• Can't take a deep breath
• Decreased feeling (sensation)
o Difficulty breathing
o Difficulty swallowing
o Fainting
o Loss of movement

Alternative Names

Landry-Guillain-Barre syndrome; GBS; Acute idiopathic polyneuritis; Infectious polyneuritis; Acute inflammatory
polyneuropathy

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.Guillain-Barré syndrome (GBS) causes progressive muscle weakness
and paralysis (the complete inability to use a particular muscle or muscle group), which develops over days or up to four
weeks, and lasts several weeks or even months.

Description

The classic scenario in GBS involves a patient who has just recovered from a typical, seemingly uncomplicated viral
infection. Symptoms of muscle weakness appear one to four weeks later. The most common preceding infections are
cytomegalovirus, herpes, Epstein-Barr virus, and viral hepatitis. A gastrointestinal infection with the bacteria Campylobacter
jejuni is also common and may cause a severe type of GBS from which it is particularly difficult to recover. About 5% of GBS
patients have a surgical procedure as a preceding event. Patients with lymphoma, systemic lupus erythematosus, or AIDS
have a higher than normal risk of GBS. Other GBS patients have recently received an immunization, while still others have
no known preceding event. In 1976–77, there was a vastly increased number of GBS cases among people who had been
recently vaccinated against the Swine flu. The reason for this phenomenon has never been identified, and no other flu
vaccine has caused such an increase in GBS cases.Guillain-Barré syndrome (GBS) is an inflammation of the covering that
surrounds nerve cells of the brain and spinal cord. The basis of the inflammation is not conclusively known, but is generally
considered to arise from a malfunctioning immune system that recognizes host tissues as being foreign. The inflammation
reaction damages the nerves of the brain and spinal cord, producing weakness in the muscles, loss of sensation (such as the
sense of touch in the fingers), or outright paralysis.GBS is termed a syndrome rather than a disease because there is no
conclusive evidence to support the possibility that a specific disease-causing agent such as a bacteria or a virus is the direct
cause of the malady. Infections may be a trigger to the development of GBS, however.

Description

The syndrome is named after George Charles Guillen and Jean-Alexandre Barré, French co-authors of a classic paper on the
syndrome that was published in 1916. A third author, André Strohl, was not subsequently associated with the syndrome that
was the subject of the paper.

GBS is a rare and acute disorder. An acute disorder displays a rapid appearance of symptoms, and a rapid worsening of the
symptoms. In the case of GBS, symptoms typically appear over just a single day. Most often, symptoms are first noticed in
the feet and legs. The symptoms often progress to involve different parts of the body over the next several days to several
weeks. In addition, during that time other more severe symptoms can appear. In more than 90% of cases, the symptoms
reach their peak by four weeks.

The syndrome is an inflammatory disorder, in which a person's own immune system attacks the nerves outside the brain and
the spinal cord. These nerves are known as peripheral nerves. The nerve inflammation that occurs can damage the nerve
cells. The covering (sheath) of a fatty material called myelin that surrounds the cells can be lost. This loss is called
demyelination.Additionally, the elongated portion of the nerve cell called the axon can be killed. This phenomenon is called
denervation. The axon conveys electrical impulses to more distant areas of muscles, and from one nerve cell to another.
Demyelination and denervation bring about muscle weakness, loss of sensation, or paralysis because the affected nerves
cannot transmit signals to muscles. This loss of signal transmission inhibits the muscles from being able to respond to nerve
signals. As well, the brain receives fewer signals and the person can become unable to feel heat, cold, or pain.GBS is also
known as Landry-Guillain-Barré syndrome, acute idiopathic polyneuritis, infectious polyneuritis, and acute inflammatory
demyelinating polyneuropathy (AIDP). Another malady called chronic inflammatory demyelinating polyradicalneuropathy is
possibly related to GBS. It is far less common than GBS (which itself is rare) and persists longer.

Demographics

GBS can occur at any age. However, the syndrome tends to be more prevalent in men and women aged 15–35 years and
50–75 years (a bimodal pattern of age distribution), respectively. Males are slightly more susceptible than females (the ratio
of those affected is approximately 1.5 male per female). There is no known racial group that is any more susceptible to GBS,
nor any known geographical localization of the syndrome.In the United States, the syndrome is rare. For example, the annual
incidence of GBS in the United States ranges from 0.6 to 2.4 cases per 100,000 people. Nonetheless, GBS is the most
common cause of neuromuscular paralysis among Americans.

Causes and symptoms

Causes

The exact cause of GBS is not known. However, bacterial or viral infections may be a trigger for its development. Almost
70% of those who develop GBS have had an infectious illness in the preceding two to four weeks. Examples of infections
include sore throat, cold, flu, and diarrhea. Bacteria that have been associated with the subsequent development of GBS
include chlamydia, Mycoplasma pneumoniae, and Campylobacter jejuni.The suspected involvement of Campylobacter is
noteworthy, as this bacterium is a common contaminant of poultry. Inadequate cooking can allow the microbe to survive and
cause an infection in those who consume the food. Thus, there may be a connection between GBS and food quality. The
form of GBS that may be associated with the presence of Campylobacter may be particularly severe. For reasons that are
unclear, the peripheral nerves can themselves be directly attacked, rather than just the myelin sheath around the
nerves.Usually, infections such as those caused by Campylobacter have abated before the onset of GBS. As well, chronic
infection with the viruses responsible for mononucleosis, herpes, and acquired immunodeficiency syndrome can prelude the
appearance of GBS. The latter is also known as HIV-1 associated acute inflammatory demyelinating polyneuropathy.Other
possible associated factors include vaccination (rabies, swine flu, influenza, Group A streptococci), surgery, pregnancy, and
maladies such as Hodgkin's disease and systematic lupus erythematosus.Whether there is direct (causal) connection
between infections and maladies and the subsequent development of GBS, or whether the events are only coincidental, is
not known. For example, vaccination of Americans against the swine flu in 1976 increased the rate of GBS by less than one
case per 100,000 people. Whether this increase was directly due to the vaccine is impossible to determine. Furthermore,
more than 99% of people suffering from GBS who have been surveyed by the United States Centers for Disease Control and
Prevention (CDC) have not recently been vaccinated. According to the CDC, the chance of developing GBS as a result of
vaccination is remote.

It is conceivable that the infections or illnesses disrupt the body's immune system such that autoimmune destruction of
nerve cell components occurs. Although this intriguing possibility is favored among many scientists, it remains
unsubstantiated.There is no evidence to indicate that GBS is an infection or that it is a genetically linked (heritable) disorder.

Symptoms

The initial sensation of weakness or paralysis in the toes spreads upward within days to a few weeks to the arms and the
central part of the body. In medical terminology, this represents an ascending pattern of spread. The weakness and paralysis
can also be accompanied by a tingling sensation, and a cramping or more constant pain in the feet, hands, thighs,
shoulders, lower back, and buttocks. Use of the hands and feet can become impaired. More serious development of paralysis
can make breathing difficult, even to the point that mechanical ventilation becomes necessary.Other, less typical symptoms
include blurred vision, clumsiness, difficulty in moving facial muscles, involuntary muscle contractions, and a pronounced
heartbeat. Symptoms that are indicative of an emergency include difficulty in swallowing, drooling, breathing difficulty, and
fainting.Progression from the early symptoms to the more severe symptoms can occur very quickly (i.e., 24–72 hours).
Typically, the exacerbated condition persists for several weeks. Recovery then typically occurs gradually, and can take
anywhere from days to six months or more.In very mild cases, an individual may just have a general feeling of weakness. As
the symptoms abate after a few weeks, the person may dismiss the incident as a viral infection, without ever knowing the
true nature of the illness.

Diagnosis

GBS is suspected if a patient displays muscle weakness or paralysis that has been increasing in severity, especially if an
illness has occurred recently. Loss of reflexes such as the knee jerk reaction can be an early clue to a clinician.Clinical data
can be useful in diagnosis. For example, a hormone that is involved in maintaining the proper chemical balance of urine can
be affected in GBS. The result is called the syndrome of inappropriate antidiuretic hormone. Antibodies to nerve cells may be
present as a result of the body's immune reaction against its own constituents.Another clue to the diagnosis of GBS can be
the finding of muscle weakness by neurological examination. One such test is known as nerve conduction velocity. In this
test, the selected nerve is stimulated, usually with surface electrodes contained in a patch that is applied to the surface of
the skin. The nerve can be stimulated using a very mild electrical current put out from one electrode, and the resulting
electrical activity is recorded by the other electrodes in the patch. The nerve conduction velocity is calculated knowing the
distance between electrodes and measuring the time it takes for the impulses to travel from the generating to the
measuring electrodes. A person with GBS whose nerves have usually lost some or most of the myelin sheath will display a
slower conduction velocity than that displayed by an unaffected person. Electrical impulses travel along the damaged nerve
slower than along an undamaged nerve.Muscle response to electrical stimulation can also be measured by
electromyography (EMG). In this test, a needle electrode is inserted through the skin into the muscle. When the muscle is
stimulated, for example, by contracting it, the resulting visual or audio pattern carries the information about the muscle's
response. The characteristic pattern of wavelengths produced by a healthy muscle (the action potential) can be compared to
a muscle in someone suspected of having GBS.When paralysis of the heart muscle is suspected, an electrocardiogram can
be used to record the electrical activity of the heart. GBS muscle paralysis can alter the normal pattern of the
heartbeat.Finally, an examination of the cerebrospinal fluid by means of a spinal tap (also known as a lumbar puncture) may
detect a higher-than-normal level of protein in the absence of an increase in the number of white blood cells (WBCs). An
increase in WBCs is a hallmark of an infection.

Treatment team

Neurologists, immunologists, physical therapists, occupational therapists, and nurses figure prominently in GBS treatment.
The assistance of support groups such as the Guillen-Barré Syndrome Foundation International can also be a useful adjunct
to treatment.

Treatment

As recently as the 1980s, treatment for GBS consisted of letting the syndrome run its course. While most people recovered
completely with time, some people were not as lucky. Those who develop severe symptoms such as breathing difficulty are
routinely hospitalized.One medical procedure that can be useful in the treatment of GBS is called plasmaphoresis. It is also
known as plasma exchange. In plasmapheresis, antibody-laden blood plasma (the liquid portion of the blood) is removed
from the body. Red blood cells are separated and put back into the body with antibody-free plasma or intravenous fluid. The
treatment can lessen the symptoms of GBS and hasten recovery time. As of December 2003, it is not known why
plasmapheresis works. It is suspected that the removal of antibodies may lessen the effects of the body's immune attack on
the nerve cells.Another procedure that produces similar results involves the administration of intravenous immune globulin
(IVIG). Both treatments have been shown to speed up recovery time by up to 50%. IVIG has been shown to be an effective
treatment for immune-system-related neuropathies in general. IVIG may act by reducing the amount of anti-myelin
antibodies through the binding of the defective antibodies by healthy antibodies contained in the IVIG solution, and in
suppressing the immune response.Other treatments are designed to prevent or lessen complications of GBS. For example,
choking during eating, because of throat muscle weakness or paralysis, can be prevented using a feeding tube, and
formation of blood clots can be lessened by the use of chemicals that thin the blood. The pain associated with GBS can be
treated with anti-inflammatory drugs or, if necessary, stronger-acting narcotic medication. For patients who have breathing
difficulties, clinicians may first need to supply oxygen, install a breathing tube (intubation), and/or use a mechanical device
that helps in breathing.Physical therapy is helpful. Caregivers can move a patient's arms and legs to help maintain the
flexibility and strength of the muscles. Later in recovery, sessions in a whirlpool (hydrotherapy) can help restore function to
arms and legs. Often, therapists will design a series of exercises to be performed when the patient returns home.

Recovery and rehabilitation

More than 95% of people afflicted with GBS survive. In about 20% of people, however, muscle weakness and fatigue may
remain. Some people find that wearing highly elastic gradient compression stockings beneficial. The stockings produce the
greatest compression at the toes, with a tapering-off upwards to the thigh. The effect is to reduce the volume of veins, which
increases the rate of blood flow through the veins. The increased blood flow can reduce the feeling of numbness in the toes.

Clinical trials

As of early 2004, three clinical trials were recruiting patients, including:

• Assessment of chronic Guillain-Barré syndrome improvement with use of 4-aminopyridine. The study, funded by the
United States Food and Drug Administration Office of Orphan Products Development, seeks to assess the potential
of 4-aminopyridine in increasing the transmission of impulses in damaged nerves. It is hoped that increased nerve
activity could restore some lost muscle activity, as has occurred using the drug with those afflicted with multiple
sclerosis. The contact is the Spain Rehabilitation Center, University of Alabama at Birmingham, 35249-7330; Jay
Meythaler, M.D. (205) 934-2088, (email: Jmeythal@uab.edu).
• Safety, tolerability, and efficacy of rituximab in patients with anti-glycoconjugate antibody-mediated demyelinating
neuropathy: a double-blind placebo-controlled randomized trial. While not directly related to GBS, the study
concerns the loss of the myelin sheath of nerves and so is relevant. The study, sponsored by the National Institute
of Neurological Disorders and Stroke (NINDS), is designed to evaluate the usefulness of rituximab in preventing the
antibody damage to nerves. The contact is the National Institutes of Health Patient Recruitment and Public Liaison
Office, Building 61, 10 Cloister Court, Bethesda, MD, 20892-4754; (800) 411-1222; prpl@mail.cc.nih.gov.
• Diagnostic evaluation of patients with neuromuscular diseases. This NINDS-sponsored study is designed to screen
patients for other studies and to help train clinicians in the diagnosis of maladies including GBS. The contact
information is the same as the above item.

Prognosis

Most of those afflicted with GBS recover completely, although the recovery can in some cases be slow (months to years).
Complete recovery usually occurs when the symptoms fade within three weeks of appearing. The typical scenario is for a
patient to experience the most weakness from 10–14 days after the appearance of symptoms, with complete recovery
occurring within weeks or a few months. In contrast, a poor prognosis can be associated with a rapid appearance of
symptoms, use of assisted ventilation for a month or more, severe nerve damage, and with advancing age.While recovery is
complete for most of those afflicted with GBS, in 10–20% of cases the symptoms reappear, in 15–20% the neurologic
complications can persist and can cause a long-term disability, and 5–10% of those who are afflicted die. The main cause of
death historically was from respiratory failure due to muscle paralysis. With mechanical ventilation, respiratory failure in GBS
is less often fatal. Currently the main cause of death is malfunctioning of the autonomic nervous system, which controls
involuntary processes such as heart rate, blood pressure, and body temperature.

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