Guillain–Barré syndrome (GBS) (French pronunciation: [ɡiˈlɛ̃ baˈʁe], English pronunciation: /

ˈɡiːlæn ˈbɑreɪ/), sometimes Landry's paralysis, is an acute inflammatory

demyelinating polyneuropathy (AIDP), a disorder affecting the peripheral nervous
system. Ascending paralysis, weakness beginning in the feet and hands and migrating
towards the trunk, is the most typical symptom. It can cause life-threatening
complications, particularly if the breathing muscles are affected or if there is dysfunction
of the autonomic nervous system. The disease is usually triggered by an acute infection.
Guillain–Barré syndrome is a form of peripheral neuropathy.

The diagnosis is usually made by nerve conduction studies. With prompt treatment by
intravenous immunoglobulins or plasmapheresis, together with supportive care, the
majority will recover completely. Guillain–Barré syndrome is rare, at 1–2 cases per
100,000 people annually, but is one of the leading causes of acute non-trauma-related
paralysis in the world. The syndrome is named after the French physicians Georges
Guillain and Jean Alexandre Barré, who described it in 1916.

Six different subtypes of Guillain–Barré syndrome exist:[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),[1] also known as 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[2] 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.[3]
• Acute panautonomic neuropathy is the most rare variant of GBS, sometimes
accompanied by encephalopathy. It is associated with a high mortality rate, owing
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. 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 (drooling, or difficulty swallowing and/or maintaining an open
airway) and respiratory difficulties. Most patients require hospitalization and about 30%
require ventilatory assistance.[4] 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.
SIADH is one of the causes of hyponatremia and can be accompanied with various
conditions such as malignancies, infections and nervous system diseases. Symptoms of
Guillain- Barre syndrome such as general weakness, decreased consciousness, and
seizure are similar to those of hyponatremia

The symptoms of Guillain–Barré syndrome are also similar to those for progressive
inflammatory neuropathy.[5]

[edit] Cause
Structure of a typical neuron
Neuron

Dendrite
Soma
Axon
Nucleus
Node of
Ranvier
Axon terminal
Schwann cell
Myelin sheath

All forms of Guillain–Barré syndrome are due to an immune response to foreign antigens
(such as infectious agents) that is 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
bacterium Campylobacter jejuni.[6][7] However, 60% of cases do not have a known cause.
One study suggests that a minority of cases may be triggered by the influenza virus, or by
an immune reaction to the influenza virus.[8]

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.

[edit] Influenza

While influenza vaccines have sometimes been suspected to raise the incidence of GBS,
the evidence is equivocal. On the other hand, getting infected by influenza itself increases
both the risk of death (up to 1 in 10,000) and increases the risk of developing GBS to a
much higher level than the highest level of suspected vaccine involvement (approx. 10
times higher by recent estimates).[9][10]

[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).[11] 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).[12] However, the role of the vaccine even in those 25 cases in 1976 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.[13] Furthermore, the GBS
may not have been directly due to the vaccine but to a bacterial contamination of the
1976 vaccine.[14]

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.[15][16]

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.[17] A preliminary report by the CDC's Emerging
Infections Programs (EIP) calculates the rate of GBS observed in patients who previously
received the 2009 H1N1 influenza vaccination is an excess of 0.8 per million cases,
which is on par with the rate seen with the seasonal trivalent influenza vaccine.[18]

Although one review gives an incidence of about one case per million vaccinations,[19] a
large study in China, reported in the NEJM covering close to 100 million doses of
vaccine against the 2009 H1N1 "swine" flu found only eleven cases of Guillain-Barre
syndrome, (0.1%) total incidence in persons vaccinated, actually lower than the normal
rate of the disease in China, and no other notable side effects; "The risk-benefit ratio,
which is what vaccines and everything in medicine is about, is overwhelmingly in favor
of vaccination."[20]

[edit] 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.

[edit] Diagnostic criteria

Required:[citation needed]

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

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

Supportive:[citation needed]
 • 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:[citation needed]

• acute myelopathies with chronic back pain and sphincter dysfunction

• botulism with early loss of pupillary reactivity and descending paralysis
• 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
• intoxication with Karwinskia humboldtiana leaves or seeds
• 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.

[edit] 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) that is less negative
(i.e., closer to zero) than -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,[21][22] 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.

[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, over 40 years, 2) history of preceding diarrheal
illness, 3) requiring ventilator support, 4) high anti-GM1 titre and 5) poor upper limb
muscle strength.

[edit] Epidemiology
The incidence of GBS during pregnancy is 1.7 cases per 100,000 of the population.[23]
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.[24]

[edit] 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.[25]

GBS is also known as acute idiopathic polyradiculoneuritis, acute idiopathic

polyneuritis, French polio, Landry's ascending paralysis and Landry Guillain Barré
syndrome.

Canadian neurologist C. Miller Fisher described the variant that bears his name in 1956.
[26]

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 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.

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.

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.

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In this Article

• What is Guillain-Barré syndrome?

• What causes Guillain-Barré syndrome?
• What are symptoms of Guillain-Barré syndrome?
• How is Guillain-Barré syndrome diagnosed?
• How is Guillain-Barré syndrome treated?
• What is the long-term outlook for those with Guillain-Barré syndrome?
• What research is being done on Guillain-Barré syndrome?
• Where can I get more information about Guillain-Barré syndrome?
• Guillain-Barre Syndrome Glossary
• Guillain-Barre Syndrome Index
• Find a local Neurologist in your town

How is Guillain-Barré syndrome 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 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 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.

Medical Author: Benjamin C. Wedro, MD, FAAEM

Medical Editor: Melissa Conrad Stöppler, MD

It takes a celebrity to draw attention to illnesses that often fly under the radar. Such is the
case of William "The Fridge" Perry, the Ex-Chicago Bear football player who came to
national attention when the team won the Super Bowl with Perry as a defensive star. In
2008, Mr. Perry spent five months in the hospital because of Guillain-Barré syndrome
and survived an illness that is a potential killer.

Nobody knows what why Guillain-Barré syndrome occurs. It's an autoimmune disease in
which the body attacks its own tissue; in this disease, the tissue is the myelin that covers
nerve fibers and the attack causes failure of those nerves to work. This leads to symptoms
that begin with numbness and tingling of the arms or legs and can progress to muscle
weakness. The major worry with this condition is that important muscles like those that
help us breathe become weak, and respiratory failure can occur.

While doctors don't know why, Guillain-Barré syndrome often occurs after colds and
stomach flu. The disease can progress slowly over a few weeks or can rapidly cause
paralysis of the arms, legs, and chest muscles within hours. The symptoms are pretty non-
specific when they begin, perhaps a little tingling in the feet and hands. The tingling
doesn't spread, but soon symmetric weakness or paralysis can begin. It most often starts
in the legs and spreads upwards to the trunk and the head. There may be problems with
vision and swallowing as a variety of muscles fail. The big deal, though, is weakness of
the diaphragm and rib muscles that allow breathing to happen.

The disease is rare and presents a diagnostic challenge to doctors. A variety of illnesses
cause neuropathy, the failure of nerves to function. From common diseases such as
diabetes and alcoholism to those most rare, such as botulism and heavy metal poisoning
and many diseases in between, there are plenty of reasons for nerves to stop conducting
electricity and fail to work properly. The history and physical examination are what
makes the diagnosis along with the doctor's index of suspicion. Tests to confirm the
diagnosis include a spinal tap to check protein levels in the spinal fluid and nerve
conduction studies to see whether the nerves are functioning normally.

Treatment is supportive, making certain the patient can breathe. Some of those affected
may end up on a ventilator until the nerves begin to function again. Supportive treatment
may include plasmapheresis, in which plasma is cleansed of antibodies with the hope of
removing those that have attacked the nerve cells and intravenous immunoglobulin which
reintroduces normal antibodies to the body.

The Fridge is one of the unlucky persons to have survived the initial attack of Guillain-
Barré syndrome and then have a recurrence. More than 80 percent of people recover from
the illness with little complication. Only one percent or less can have a relapse and when
that happens, the treatment and worry begin all over again.

Medical Author: Benjamin C. Wedro, MD, FAAEM

Medical Editor: Melissa Conrad Stöppler, MD

It takes a celebrity to draw attention to illnesses that often fly under the radar. Such is the
case of William "The Fridge" Perry, the Ex-Chicago Bear football player who came to
national attention when the team won the Super Bowl with Perry as a defensive star. In
2008, Mr. Perry spent five months in the hospital because of Guillain-Barré syndrome
and survived an illness that is a potential killer.

Nobody knows what why Guillain-Barré syndrome occurs. It's an autoimmune disease in
which the body attacks its own tissue; in this disease, the tissue is the myelin that covers
nerve fibers and the attack causes failure of those nerves to work. This leads to symptoms
that begin with numbness and tingling of the arms or legs and can progress to muscle
weakness. The major worry with this condition is that important muscles like those that
help us breathe become weak, and respiratory failure can occur.

While doctors don't know why, Guillain-Barré syndrome often occurs after colds and
stomach flu. The disease can progress slowly over a few weeks or can rapidly cause
paralysis of the arms, legs, and chest muscles within hours. The symptoms are pretty non-
specific when they begin, perhaps a little tingling in the feet and hands. The tingling
doesn't spread, but soon symmetric weakness or paralysis can begin. It most often starts
in the legs and spreads upwards to the trunk and the head. There may be problems with
vision and swallowing as a variety of muscles fail. The big deal, though, is weakness of
the diaphragm and rib muscles that allow breathing to happen.

The disease is rare and presents a diagnostic challenge to doctors. A variety of illnesses
cause neuropathy, the failure of nerves to function. From common diseases such as
diabetes and alcoholism to those most rare, such as botulism and heavy metal poisoning
and many diseases in between, there are plenty of reasons for nerves to stop conducting
electricity and fail to work properly. The history and physical examination are what
makes the diagnosis along with the doctor's index of suspicion. Tests to confirm the
diagnosis include a spinal tap to check protein levels in the spinal fluid and nerve
conduction studies to see whether the nerves are functioning normally.

Treatment is supportive, making certain the patient can breathe. Some of those affected
may end up on a ventilator until the nerves begin to function again. Supportive treatment
may include plasmapheresis, in which plasma is cleansed of antibodies with the hope of
removing those that have attacked the nerve cells and intravenous immunoglobulin which
reintroduces normal antibodies to the body.

The Fridge is one of the unlucky persons to have survived the initial attack of Guillain-
Barré syndrome and then have a recurrence. More than 80 percent of people recover from
the illness with little complication. Only one percent or less can have a relapse and when
that happens, the treatment and worry begin all over again.

• Also known as polyradiculoneuritis.

• It is an acute inflammatory polyneuropathy of the peripheral sensory and motor
and nerve roots.
• Affected nerves are demyelinated with possible axonal degeneration.
• It’s exact cause is unknown, Guillain-Barré Syndrome is believed to be an
autoimmune disorder that may be triggered by viral infection, Campylobacter
diarrheal illness, immunization, or other precipitating event.
• The syndrome is marked by acute onset of symmetric progressive muscle
weakness, most often beginning in the legs and ascending to involve the trunk,
upper extremities, and facial muscles. Paralysis may develop.
• Complications may include respiratory failure, cardiac arrhythmias, and
complications of immobility.

Assessment:

1. Acute onset (hours to weeks) of progressive, usually ascending muscle weakness

and fasciculation, possibly leading to paralysis (maximal weakness is reached
within 2 weeks).
2. Paresthesia and painful sensations.
3. Possible hypoventilation due to chest muscle weakness.
4. Difficulty with swallowing, chewing, speech, and gag, indicating fifth
(trigeminal) and ninth (glossopharyngeal) cranial nerve movement.
5. Reduce or absent deep tendon reflexes, position and vibratory perception.
 6. Autonomic dysfunction with orthostatic hypotension and tachycardia.

Diagnostic Evaluation:

1. Lumbar puncture obtains cerebrospinal fluid samples, which reveal low cell count
and high protein levels.
2. Nerve conduction studies, which allow decreased conduction velocity of
peripheral nerves due to demyelination.
3. Abnormal laboratory studies may point to prior infection or illness.

Pharmacologic Interventions:

1. Analgesics and muscle relaxants to control painful sensations and fasciculations.

Nursing Interventions:

1. Monitor respiratory status through vital capacity measurements, rate and depth of
respirations, and breath sounds.
2. Monitor level of muscle weakness as it ascends toward respiratory muscles.
Watch for breathlessness while talking which is a sign of respiratory fatigue.
3. Monitor the patient for signs of impending respiratory failure.
4. Monitor gag reflex and swallowing ability.
5. Position patient with the head of bed elevated to provide for maximum chest
excursion.
6. Avoid giving opioids and sedatives that may depress respirations.
7. Position patient correctly and provide range-of-motion exercises.
8. Provide good body alignment, range-of-motion exercises, and change of position
to prevent complications such as contractures, pressure sores, and dependent
edema.
9. Ensure adequate nutrition without the risk of aspiration.
10. Encourage physical and occupational therapy exercises to help the patient regain
strength during rehabilitation phase.
11. Provide assistive devices as needed (cane or wheelchair) to maximize
independence and activity.
12. If verbal communication is possible, discuss the patient’s fears and concerns.
13. Provide choices in care to give the patient a sense of control.
14. Teach patient about breathing exercises or use of an incentive spirometer to
reestablish normal breathing patterns.
15. Instruct patient to wear good supportive and protective shoes while out of bed to
prevent injuries due to weakness and paresthesia.
16. Instruct patient to check feet routinely for injuries because trauma may go
unnoticed due to sensory changes.
17. Urge the patient to maintain normal weight because additional weight will further
stress monitor function.
18. Encourage scheduled rest periods to avoid fatigue.
Guillain-Barre Syndrome in Emergency Medicine
Author: Andrew C Miller, MD, Fellow, Department of Pulmonary, Allergy, and
Critical Care Medicine, University of Pittsburgh Medical Center; Attending Physician,
Department of Emergency Medicine, University of Pittsburgh Medical Center
Coauthor(s): Razi M Rashid, MD, MPH, Resident Physician, Department of
Neurology, Northwestern University Hospital, Chicago; Richard H Sinert, DO,
Associate Professor of Emergency Medicine, Clinical Assistant Professor of Medicine,
Research Director, State University of New York College of Medicine; Consulting Staff,
Department of Emergency Medicine, Kings County Hospital Center
Contributor Information and Disclosures

Updated: Jan 14, 2011

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Introduction
Background

In 1859, Landry published a report on 10 patients with an ascending paralysis.1

Subsequently, in 1916, 3 French physicians (Guillain, Barré, and Strohl) described 2
French soldiers with motor weakness, areflexia, CSF albuminocytological dissociation,
and diminished deep tendon reflexes.2 The identified syndrome was later named Guillain-
Barré syndrome (GBS). Historically, GBS was a single disorder; however, current
practice acknowledges several variant forms.

GBS is a heterogeneous grouping of immune-mediated processes generally characterized

by motor, sensory, and autonomic dysfunction. In its classic form, GBS is an acute
inflammatory demyelinating polyneuropathy characterized by progressive symmetric
ascending muscle weakness, paralysis, and hyporeflexia with or without sensory or
autonomic symptoms; however, variants involving the cranial nerves or pure motor
involvement are not uncommon. In severe cases, muscle weakness may lead to
respiratory failure. Labile autonomic dysfunction may complicate the use of vasoactive
and sedative drugs.

Pathophysiology

Although the clinical syndrome of GBS classically presents as a rapidly progressive acute
polyneuropathy, several pathologic and etiologic subtypes exist. Most patients with
Guillain-Barré syndrome exhibit absent or profoundly delayed conduction in action nerve
fibers resulting from axon demyelination occurring primarily in peripheral nerves and
spinal roots (cranial nerves may be involved as well).

GBS is believed to result from autoimmune humoral- and cell-mediated responses to a

recent infection or any of a long list of medical problems. Its relation to antecedent
infections and the identification of various antiganglioside antibodies suggest that
molecular mimicry may contribute.3,4 Antibodies formed against ganglioside-like epitopes
in the lipopolysaccharide (LPS) layer of some infectious agents have been shown in both
necropsy and animal models to cross-react with ganglioside surface molecules on
Schwann cells.3

Symptoms generally coincide pathologically with various patterns of lymphocytic

infiltration and macrophage-mediated demyelination, depending on the subtype in
question. Recovery is typically associated with remyelination. In a subset of patients,
GBS is associated primarily with myelin-sparing axonal damage resulting from a direct
cellular immune attack on the axon itself.

The acute inflammatory demyelinating polyneuropathy (AIDP) subtype is the most

commonly identified form in the United States. It is generally preceded by a bacterial or
viral infection. Nearly 40% of patients are seropositive for Campylobacter jejuni.
Lymphocytic infiltration and macrophage-mediated peripheral nerve demyelination is
present. Symptoms generally resolve with remyelination.

The acute motor axonal neuropathy (AMAN) subtype is a purely motor subtype that is
more prevalent amongst pediatric age groups. Nearly 70-75% of patients are seropositive
for Campylobacter. One-third of these cases may actually be hyperreflexic. The
mechanism for this hyperreflexia is unclear; however, dysfunction of the inhibitory
system via spinal interneurons may increase motor neuron excitability. Hyperreflexia is
significantly associated with the presence of anti-GM1 antibodies.1 Inflammation of the
spinal anterior roots may lead to disruption of the blood-CNS barrier.3 AMAN is
generally characterized by a rapidly progressive weakness, ensuing respiratory failure,
and good recovery.

Acute motor-sensory axonal neuropathy (AMSAN) is an acute severe illness differing

from AMAN in that AMSAN also affects sensory nerves and roots.5 Patients are typically
adults with both motor and sensory dysfunction, marked muscle wasting, and poor
recovery.

Miller-Fisher syndrome (MFS) is a rare variant that classically presents with the triad of
ataxia, areflexia, and ophthalmoplegia. Acute onset of external ophthalmoplegia is a
cardinal feature.3 Ataxia tends to be out of proportion to the degree of sensory loss.
Patients may also have mild limb weakness, ptosis, facial palsy, or bulbar palsy. Patients
have reduced or absent sensory nerve action potentials and absent tibial H reflex.6 Anti-
GQ1b antibodies are prominent in this variant, and patients with acute oropharyngeal
palsy carry anti-GQ1b/GT1a IgG antibodies.3 Recovery generally occurs within 1-3
months.

Acute panautonomic neuropathy, the rarest variant, involves both the sympathetic and
parasympathetic nervous systems. Cardiovascular involvement is common, and
dysrhythmias are a significant source of mortality in this form of the disease. Recovery is
gradual and often incomplete.

Frequency

United States

The incidence of Guillain-Barré syndrome (GBS) is 1.2-3 per 100,000 inhabitants,

making GBS the most common cause of acute flaccid paralysis in the United States.1,2,7 8

International

AMAN and AMSAN occur mainly in northern China, Japan, and Mexico, and they
comprise 5-10% percent of GBS cases in the United States.9

AIDP accounts for up to 90% of cases in Europe, North America, and the developed
world.

Amongst members of the US Armed Forces, an acute episode of infectious gastroenteritis

was a significant risk factor for the development of GBS (odds ratio, 5.33; p = 0.03).10

Epidemiologic studies from Japan indicate that, in this region, a greater percentage of
GBS cases are associated with antecedent C jejuni infections and a lesser number are
related to antecedent cytomegalovirus infections compared with that in North America
and Europe.

Similarly, it has been reported that 69% of GBS cases in Dhaka, Bangladesh, have
clinical evidence of antecedent C jejuni infection.11

A study in Iran showed that 47% of pediatric GBS cases had evidence of recent C jejuni
infection.12

Mortality/Morbidity
Most patients (up to 85%) with GBS achieve a full and functional recovery within 6-12
months. Recovery is maximal by 18 months.13

Patients may experience persistent weakness, areflexia, imbalance, or sensory loss.

Approximately 7-15% of patients have permanent neurologic sequelae including bilateral
footdrop, intrinsic hand muscle wasting, sensory ataxia, and dysesthesia.

The mortality rate varies but may be less than 5% in tertiary care centers with a team of
medical professionals who are familiar with GBS management. Causes of death include
acute respiratory distress syndrome, sepsis, pneumonia, venous thromboembolic disease,
and cardiac arrest.

A recent epidemiological study reported 2-12% mortality despite ICU management.8

GBS may rarely be a recurrent disorder.14

Race

No racial preponderance exists.

Sex

GBS has a male-to-female ratio of 1.5:1. A Swedish epidemiologic study reported that
GBS rates decrease during pregnancy and increase in the months immediately following
delivery.15

Age

GBS may occur at any age; however, a bimodal distribution has been observed with
peaks in young adulthood (age 15-35 y) and in elderly persons (age 50-75 y). Rare cases
have been noted in infants.16

Clinical
History

The typical patient with Guillain-Barré syndrome (GBS), likely AIDP, presents 2-4
weeks following a relatively benign respiratory or gastrointestinal illness with complaints
of finger dysesthesias and proximal muscle weakness of the lower extremities. The
weakness may progress over hours to days to involve the arms, truncal muscles, cranial
nerves, and muscles of respiration. The illness progresses from days to weeks. The mean
time to the clinical function nadir is 12 days, with 98% of patients reaching a nadir by 4
weeks. A plateau phase of persistent, unchanging symptoms then ensues, followed days
later by gradual symptom improvement. The mean time to improvement and clinical
recovery are 28 and 200 days, respectively.
As many as one third of patients require mechanical ventilation during the course of their
illness. Causes for this include cranial nerve involvement affecting airway maintenance,
and respiratory muscle paralysis.

Symmetric limb weakness typically begins as proximal lower extremity weakness and
ascends to involve the upper extremities, truncal muscles, and head. Inability to stand or
walk despite reasonable strength, especially when ophthalmoparesis or impaired
proprioception is present. Respiratory muscle weakness with shortness of breath may be
present.

Cranial nerve palsies (III-VII, IX-XII) may be present. Patients may present with facial
weakness mimicking Bell palsy, dysphagia, dysarthria, ophthalmoplegia, and pupillary
disturbances. The Miller-Fisher variant is unique in that this subtype begins with cranial
nerve deficits. Lack of deep tendon reflexes is a hallmark sign.

Paresthesias generally begin in the toes and fingertips, progressing upward but generally
not extending beyond the wrists or ankles. Pain is most severe in the shoulder girdle,
back, buttocks, and thighs and may occur with even the slightest movements. Loss of
vibration, proprioception, touch, and pain distally may be present.

Cardiovascular signs may include tachycardia, bradycardia, dysrhythmias, wide

fluctuations in blood pressure, and postural hypotension. Urinary retention due to urinary
sphincter disturbances may be noted. Constipation due to bowel paresis and gastric
dysmotility may be present. Facial flushing and venous pooling secondary to abnormal
vasomotor tone may be present.

Hypersalivation and anhydrosis may be noted.

Tonic pupils have been reported in case reports but only in severely affected cases.

Renal salt wasting has also been noted in case reports as a manifestation of the autonomic
dysfunction of GBS.

Papilledema secondary to elevated intracranial pressure is present in rare cases.

Miller-Fisher variant presents with a predominance of cranial nerve findings, ataxia, and
areflexia. Facial weakness, ophthalmoplegias, dysarthria, and dysphagia may precede
ataxia, areflexia, and weakness.17

Physical

Vital signs

Patients may have tachy- or bradycardia, hyper- or hypotension, or hyper- or

hypothermia.
Low oxygen saturation may be present with advanced respiratory muscle involvement.

Cranial nerves

Patients may present with facial weakness mimicking Bell palsy, dysphagia, dysarthria,
ophthalmoplegia, and pupillary disturbances.

Dysreflexia

Patients with manifest weakness are invariably hypo- or areflexic in the involved areas.

• Respiratory symptoms
• Poor inspiratory effort or diminished breath sounds

Motor

Symmetric limb weakness typically progresses from proximal lower extremity to involve
the upper extremities, truncal muscles, and head.

Inability to stand or walk despite reasonable strength, especially when ophthalmoparesis

or impaired proprioception is present.

• Hypotonia
• Limb muscle wasting is not an acute finding.

Abdominal

Paucity or absence of bowel sounds suggests paralytic ileus.

Suprapubic tenderness or fullness may be suggestive of urinary retention.

Sensory

Patients may experience numbness, paresthesias, impaired proprioception, and pain.

Papilledema secondary to elevated intracranial pressure is rarely present.

Causes

GBS has been associated with antecedent bacterial and viral infections, administration of
certain vaccinations, and other systemic illnesses. Case reports exist regarding numerous
medications and procedures; however, whether any causal link exists is unclear.

Bacterial infections include C jejuni, Haemophilus influenzae, Mycoplasma pneumoniae,

and Borrelia burgdorferi.1,7,11,18,19
Viral infections including cytomegalovirus, Ebstein-Barr virus, HSV, during human
immunodeficiency virus (HIV) seroconversion.1,7,20

Vaccines

A review of all post-vaccination cases of GBS from 1990-2005 did not reveal an increase
in mortality with postvaccination cases of GBS compared with cases by other causes.21

A study reviewing GBS cases during the 1992-1993 and 1993-1994 influenza seasons
found an adjusted relative risk of 1.7 cases per 1 million influenza vaccinations.22

Despite an increased risk of GBS with the 1976 swine-origin flu vaccine a similar risk
has not manifested with more recent swine flu vaccinations.23,24

Recent studies found no evidence of an increased risk of GBS after seasonal influenza
vaccine, nor after the 2009 H1N1 mass vaccination program.25,23,24

Epidemiologic studies from Finland and southern California failed to validate an earlier
retrospective study from Finland that suggested a cause-effect relationship between oral
polio vaccination and GBS,26,27 whereas a Brazilian study suggested that, based on a
temporal association between the vaccine and the onset of GBS, the vaccine may rarely
correlate with GBS.28

Data from a large-scale epidemiologic study reported a decreased GBS incidence

following administration of tetanus toxoid containing vaccinations when compared to the
baseline population.29

An epidemiologic study failed to show any conclusive epidemiologic association between

GBS and the hepatitis B vaccine.30

A large Latin American study of more than 2000 children with GBS following a mass
measles vaccination program in 1992-1993 failed to establish a statistically significant
causal relationship between administration of the measles vaccine and GBS.31

A report from the Centers for Disease Control and Prevention (CDC) suggests that an
increased incidence of GBS may exist amongst recipients of the Menactra meningococcal
conjugate vaccine.32

Case reports exist regarding group A streptococci vaccines and the rabies vaccine,
however, conclusive statistically significant evidence is lacking.

Medications33

In a case-controlled study, GBS patients reported more frequent penicillin and

antimotility drug use, and less frequent oral contraceptive use. No definite cause-effect
relationship has been established.
Case reports exist in the setting of tumor necrosis factor antagonist agents used in
rheumatoid arthritis.34,35,36,37

Case reports exist regarding streptokinase, isotretinoin, danazol, captopril, gold, heroin,
and epidural anesthesia among others.

Case reports cite associations between bariatric and other gastric surgeries.38

Anecdotal associations include systemic lupus erythematosus, sarcoidosis, lymphoma,

surgery, renal transplantation, and snakebite.

TNF polymorphisms may increase susceptibility to axonal GBS subtypes; however, the
role of TNF in GBS remains unclear and warrants further investigation.39

Workup
Laboratory Studies

Guillain-Barré syndrome (GBS) is generally diagnosed on clinical grounds. Laboratory

studies are useful to rule-out other diagnoses, and to better assess functional status and
prognosis.

Lumbar puncture and spinal fluid analysis

Elevated or rising protein levels on serial lumbar punctures and 10 or fewer mononuclear
cells/mm3 strongly support the diagnosis.

Most, but not all, patients have an elevated cerebrospinal fluid (CSF) protein level (>400
mg/L), with normal CSF cell counts.

Normal CSF protein level does not rule out GBS as the level may remain normal in 10%
of patients. CSF protein may not rise until 1-2 weeks post- onset of weakness.

CSF pleocytosis is well recognized in HIV-associated GBS.

Biochemical screening

Biochemical screening includes electrolyte levels; liver function tests (LFTs); creatine
phosphokinase (CPK) level; erythrocyte sedimentation rate (ESR); antiganglioside
antibodies; and antibodies to C jejuni, cytomegalovirus, Ebstein-Barr virus, herpes
simplex virus (HSV), HIV, and M pneumoniae.

Syndrome of inappropriate antidiuretic hormone (SIADH) may occur.

LFT results are elevated in as many as one third patients.

CPK and ESR may be elevated with myopathies or systemic inflammatory conditions.

Patients with the Miller-Fisher variant may have anti-GQ1b antibodies.

Patients who have antibody subtype GM1 may have worse prognoses.

A stool culture for C jejuni and pregnancy test are also indicated.

Imaging Studies

MRI40,41

MRI is sensitive but nonspecific for diagnosis; however, it may be an effective diagnostic
adjunct.

Spinal nerve root enhancement with gadolinium is a nonspecific feature seen in

inflammatory conditions and is caused by disruption of the blood-nerve barrier.

Selective anterior nerve root enhancement appears to be strongly suggestive of GBS.

The cauda equine nerve roots are enhanced in 83% of patients.

Other Tests

Forced vital capacity42

Forced vital capacity (FVC) is very helpful in guiding disposition and therapy.

Patients with an FVC of less than 15-20 mL/kg, maximum inspiratory pressure of less
than 30 cm H2 O, or a maximum expiratory pressure of less than 40 cm H2 O generally
progress to require prophylactic intubation and mechanical ventilation.

Nerve conduction studies2,43

A delay in F-waves is present, implying nerve root demyelination.

Nerve motor action potentials may be decreased. This is technically difficult to determine
until the abnormality is severe.

Compound muscle action potential (CMAP) amplitude may be decreased.

Frequently, patients show evidence of conduction block or dispersion of responses at

sites of natural nerve compression. The extent of decreased action potentials correlates
with prognosis.

Prolonged distal latencies may be present.

Abnormal H-reflex may be noted.

Rarely neurophysiologic testing is normal in patients with GBS. This is believed to be

due to the location of demyelinating lesions in proximal sites not amenable to study.

Muscle biopsy

Muscle biopsy may help to distinguish GBS from a primary myopathy in unclear cases.

Other

Many different abnormalities may be seen on ECG, including 2 º and 3 º atrioventricular

(AV) block, T-wave abnormalities, ST depression, QRS widening, and various rhythm
disturbances.

Procedures

• Lumbar puncture and spinal fluid analysis

• Intubation and mechanical ventilation
• Nerve conduction studies

Treatment
Prehospital Care

• ABCs, IV, oxygen, and assisted ventilation may be indicated.

• Monitor for cardiac arrhythmias.
• Transport expeditiously.

Emergency Department Care

ABCs, IV, oxygen, and assisted ventilation may be indicated.

Intubation should be performed on patients who develop any degree of respiratory

failure. Clinical indicators for intubation include hypoxia, rapidly declining respiratory
function, poor or weak cough, and suspected aspiration. Typically, intubation is indicated
when the FVC is < 15 mL/kg.44

Patients should be monitored closely for changes in blood pressure, heart rate, and other
arrhythmias.

• Treatment is rarely needed for tachycardia.

• Atropine is recommended for symptomatic bradycardia.
 • Because of the lability of dysautonomia, hypertension is best treated with short-
acting agents, such as a short-acting beta-blocker or nitroprusside.
• Hypotension of dysautonomia usually responds to intravenous fluids and supine
positioning.
• Temporary pacing may be required for patients with 2 º and 3 º degree heart
block.

Consultations

• Consult a neurologist if any uncertainty exists as to the diagnosis.

• Consult the ICU team for evaluation of need for admission to the unit.

Medication
Only plasma exchange (PE) therapy and intravenous immune serum globulin (IVIG)
have proven effective for Guillain-Barré syndrome (GBS). Both have been shown to
shorten recovery time by as much as 50%. IVIG is easier to administer and has fewer
complications than PE.45 The cost and efficacy of each are comparable.

Randomized trials in severe disease show that IVIG started within 4 weeks from onset
hastens recovery as much as plasma exchange.46,47,48,49 Combining PE and IVIG neither
improved outcomes nor shortened illness duration.50 IVIG has also been shown safe and
effective in the treatment of pediatric GBS.50,51 Additionally, IVIG is the preferential
treatment in hemodynamically unstable patients and in those unable to ambulate
independently.52,50 Some evidence suggests that in select patients who do not respond
initially to IVIG, a second dose may be beneficial.53 However, this is not currently
standard therapy and warrants further investigation.

Corticosteroids are ineffective as monotherapy.54,2,5,55 According to moderate-quality

evidence, corticosteroids given alone do not significantly hasten recovery from GBS or
affect the long-term outcome.54,55 According to low-quality evidence oral corticosteroids
delay recovery.54,47,55 Diabetes requiring insulin was significantly more common and
hypertension less common with corticosteroids. Substantial evidence shows that IV
methylprednisolone alone neither produces significant benefit nor harm.55 In combination
with IVIG, IV methylprednisolone may hasten recovery but does not significantly affect
long-term outcome.47,56

Immune adsorption is an alternative that is still in the early stages of investigation. A

small prospective study reported no difference in outcome between patients treated with
immunoadsorption and those treated with PE.57 In critically ill patients, a small German
study reported that treatment with selective immune adsorption (SIA) seems to be safe
and effective.58 In comparison to treatment with SIA only, sequential therapy with IVIG
was not more effective.58

Interferon beta was not associated with significant clinical improvement compared with
controls in a small randomized control trial.59
A recent pilot study showed no benefit to mofetil as an adjunct therapy with IVIG.60

In an animal model, intravenous eculizumab was reported to prevent respiratory paralysis

and the functional and morphological hallmarks of terminal motor neuropathy. Human
studies have not been reported.61

Simple analgesics or nonsteroidal anti-inflammatory drugs may be tried but often provide
inadequate analgesia. Single, small randomized controlled trials support the use of
gabapentin or carbamazepine in the intensive care unit for management during the acute
phase of GBS. Adjuvant therapy with tricyclic antidepressant medication, tramadol,
gabapentin, carbamazepine, or mexiletine may aid in the long-term management of
neuropathic pain.62

Time to development of deep vein thrombosis (DVT) or pulmonary embolism varies

from 4-67 days following symptom onset.62 DVT prophylaxis with gradient compression
hose and subcutaneous low molecular weight heparin (LMWH) may cause a dramatic
reduction in the incidence of venous thromboembolism, one of the major sequela of
extremity paralysis.62 True gradient compression stockings (30-40 mm Hg or higher) are
highly elastic, providing a gradient of compression that is highest at the toes and
gradually decreases to the level of the thigh. This reduces capacity venous volume by
approximately 70% and increases the measured velocity of blood flow in the deep veins
by a factor of 5 or more.

The ubiquitous white stockings known as antiembolic stockings or thromboembolic

disease (TED) hose produce a maximum compression of 18 mm Hg and rarely are fitted
in such a way as to provide adequate gradient compression. They have not been shown to
be effective as prophylaxis against thromboembolism.

Blood product derivatives

These agents are used to improve the clinical and immunologic aspects of the disease.
They may decrease autoantibody production and increase solubilization and removal of
immune complexes.

Intravenous immune globulin (IVIG, Gammagard S/D)

May neutralize circulating myelin antibodies through anti-idiotypic antibodies and down-
regulate proinflammatory cytokines, including interferon-gamma (INF-gamma). In
addition, may block the complement cascade and promote remyelination.

• Dosing
• Interactions
• Contraindications
 • Precautions

Adult

0.4 g/kg/d IV for 5 d

Pediatric

Administer as in adults

• Dosing
• Interactions
• Contraindications
• Precautions

None reported

• Dosing
• Interactions
• Contraindications
• Precautions

Documented hypersensitivity; IgA deficiency and anti-IgE/IgG antibodies

• Dosing
• Interactions
• Contraindications
• Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans;
may use if benefits outweigh risk to fetus

Precautions

Check serum IgA level before IVIG (use an IgA-depleted product, eg, Gammagard S/D);
infusions may increase serum viscosity and thromboembolic events; infusions may
increase risk of migraine attacks, aseptic meningitis (10%), urticaria, pruritus, or
petechiae (2-30 d postinfusion)
Increases risk of renal tubular necrosis in elderly patients and in patients with diabetes,
volume depletion, and preexisting kidney disease; laboratory result changes associated
with infusions include elevated antiviral or antibacterial antibody titers for 1 mo, 6-fold
increase in ESR for 2-3 wk, and apparent hyponatremia
Albumin (Albuminar, Albumisol, Albunex, Albutein)

Used in plasma exchange when the patient's plasma is exchanged with a plasma
substitute. May remove autoantibodies and immune complexes from serum. Plasma
exchange is carried out with albumin (50 mL/kg) over a 10-d period. Has been shown to
decrease recovery time by 50%. May aid in removing cytotoxic constituents from serum.

• Dosing
• Interactions
• Contraindications
• Precautions

Adult

Remove 3-4 L of the patient's plasma and substitute with albumin; administered IV

Pediatric

<16 years: Not established

>16 years: Administer as in adults

• Dosing
• Interactions
• Contraindications
• Precautions

None reported

• Dosing
• Interactions
• Contraindications
• Precautions

Pulmonary edema; renal insufficiency

• Dosing
• Interactions
• Contraindications
• Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans;
may use if benefits outweigh risk to fetus

Precautions
While theoretically attractive, no proven benefit of colloid resuscitation over isotonic
crystalloids exists

Fractionated low molecular weight heparins

These agents are used in the prophylaxis of DVT. Fractionated LMWH first became
available in the United States as enoxaparin (Lovenox). LMWH has been used widely in
pregnancy, although clinical trials are not yet available to demonstrate that it is as safe as
unfractionated heparin.

Reversible elevation of hepatic transaminase levels occurs occasionally. Heparin-

associated thrombocytopenia has been observed with fractionated low molecular weight
heparin.

Enoxaparin (Lovenox)

Enhances the inhibition of factor Xa and thrombin by increasing antithrombin III activity.
Also slightly affects thrombin and clotting time and preferentially increases the inhibition
of factor Xa. Has a wide therapeutic window; prophylactic dose is not adjusted based on
the patient's weight. Enoxaparin is safer and more effective than unfractionated heparin
for prophylaxis of venous thromboembolism. Average duration of treatment is 7-14 d.

• Dosing
• Interactions
• Contraindications
• Precautions

Adult

30 mg SC bid

Pediatric

Not established
The following doses have been suggested:
<2 months: 0.75 mg/kg/dose bid SC
>2 months to 18 years: 0.5 mg/kg/dose bid SC

Follow-up
Further Inpatient Care
 • Admission to the ICU should be considered for all patients with labile
dysautonomia, an FVC of less than 20 mL/kg, or severe bulbar palsy.62
• Any patients exhibiting clinical signs of respiratory compromise, in any degree,
also should be admitted to an ICU.62
• In addition to those criteria listed earlier in the ED management section, Medical
Research Council sum score as well as bulbar weakness have been reported to be
predictive of requiring inpatient mechanical ventilation.63
• The risk of sepsis and infection can be decreased by use of minimal sedation,
frequent physiotherapy, and mechanical ventilation with positive end expiratory
pressure where appropriate.62
• The risk of DVT and pulmonary embolus may be minimized by administration of
heparin or a low molecular weight heparin and intermittent pneumatic
compression devices.62
• The use of cardiac telemetry and pacing in the case of severe bradycardia may
help to reduce the risk of cardiac morbidity and mortality.62
• Pain may be symptomatically improved by frequent passive limb movements,
gentle massage, frequent position changes, and use of carbamazepine and
gabapentin.64,62
• Narcotics should be used judiciously because patients may already be at risk for
ileus.62

Further Outpatient Care

• Physical therapy and occupational therapy may be beneficial in helping patients

with Guillain-Barré syndrome to regain their baseline functional status.13,62

Transfer

• Transfer may be appropriate if a facility does not have the proper resources to
care for patients who may require prolonged intubation or prolonged intensive
care.

Complications

With modern methods of respiratory management, most complications result from long-
term paralysis. Possible complications include the following:

• Persistent paralysis
• Respiratory failure, mechanical ventilation
• Hypotension or hypertension
• Thromboembolism, pneumonia, skin breakdown
• Cardiac arrhythmia
• Ileus
• Aspiration
• Urinary retention
• Psychiatric problems such as depression and anxiety
 • Nephropathy reported in pediatric patients65

Prognosis

Poor prognosis for patients with GBS is associated with rapid progression of symptoms,
advanced age, prolonged ventilation (>1 mo), and severe reduction of action potentials on
neuromuscular testing.66

Published reports indicate full recovery may be expected in 50-95% of patients with
GBS.

Factors associated with poorer levels of functioning and well-being may include females,
age older than 57 years, acute hospital stay of longer than 11 days, ICU requirement, and
those discharged to rehabilitation.67

Long-term follow-up studies show nearly 50% of patients exhibit histological and clinical
residual neuropathies years after the initial syndrome. Patients may have residual motor
and sensory dysfunction.68

Increased CSF levels of neurone-specific enolase and S-100b protein are associated with
longer duration of illness.1

A longer-lasting increase in IgM anti-GM1 predicts slow recovery.1

Neurologic sequelae

Reported incidence of permanent neurologic sequelae ranges from 10-40%.

The worst-case scenario is tetraplegia within 24 hours with incomplete recovery after 18
months or longer.

The best-case scenario is mild difficulty walking, with recovery within weeks.

The usual scenario is peak weakness in 10-14 days with recovery in weeks to months.
Average time on a ventilator (without treatment) is 50 days.

Recent studies report that patients with GBS may exhibit long-term differences in pain
intensity, fatigability, and functional impairment compared with healthy controls.69

Mortality

Most cases of mortality are due to severe autonomic instability or from the complications
of prolonged intubation and paralysis.70,71,72,42

Mortality rates range from 5-10%.

Patient Education

For excellent patient education resources, visit eMedicine's Brain and Nervous System
Center. Also, see eMedicine's patient education article Guillain-Barré Syndrome.

Miscellaneous
Medicolegal Pitfalls

• Failure to anticipate dysrhythmias and autonomic instability

• Failure to anticipate progressive respiratory failure
• Failure to correctly diagnose Guillain-Barré syndrome in patients with a variant
form of the disease or in those with a normal CSF protein
• Failure to provide adequate DVT prophylaxis in a patient that develops a DVT
and/or pulmonary embolism

Special Concerns

• The leading cause of death in elderly patients with Guillain-Barré syndrome is

arrhythmia.
• Recurrence of Guillain-Barré syndrome is rare but has been reported in 2-5% of
patients.73
• Variants of Guillain-Barré syndrome may present with pure motor dysfunction or
acute dysautonomia.
• The Miller-Fisher syndrome is a variant of Guillain-Barré syndrome in which the
initial symptoms include ataxia, ophthalmoplegia, and areflexia.

Guillain-Barré Syndrome
Overview | Signs & Symtpoms | Tests | Treatment | Related Pages

Also known as: Infectious polyneuritis; Acute inflammatory demyelinating

polyneuropathy; Landry's ascending paralysis

What is it?

Guillain-Barré Syndrome (GBS) is an acute condition associated with progressive muscle

weakness and paralysis. It is an autoimmune disorder in which the body's immune system
attacks its own nervous system. This causes inflammation that damages or destroys the
myelin sheaths covering and insulating nerve fibers (axons) and sometimes damages the
fibers themselves. This demyelination process slows or stops the conduction of impulses
through the nerve, interfering with motor control and causing symptoms such as tingling
or numbness that typically start in the hands and feet and move progressively upward,
affecting both sides of the body. GBS is a medical emergency and must be closely
monitored. Those affected may become so weak that they have trouble breathing and
their heart rate may become abnormal.
Guillain-Barré syndrome is a relatively rare condition, but it is the most common
acquired inflammatory neuropathy and the most common cause of acute paralysis in the
United States. According to the National Institute of Neurological Disorders and Stroke
(NINDS), about 1 person in 100,000 will have GBS. It can affect anyone at any age. It is
an unusual neuropathy in that it spontaneously reverses, and most patients recover most
or all of the lost nerve and muscle function.

The exact cause of GBS and why it affects one person and not another is not well
understood. The autoimmune process may be spontaneous or may be triggered by some
specific disease or exposure. Many cases have been linked with a viral or bacterial
infection that occurs a week or two before GBS develops. Cases have also been seen in
people with HIV infection, in those with chronic diseases such as lupus (SLE), Hodgkin
lymphoma (and some other malignancies), and rarely in those who have recently had a
vaccination (such as for rabies or swine flu). Something in these circumstances leads to a
change in the immune system's ability to discriminate between "self" and "non-self."
Damage to the myelin sheath and nerve is thought to involve antibodies that mistakenly
target these tissues.

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This article was last reviewed on March 2, 2011. | This article was last modified on
March 2, 2011.

Also known as: Infectious polyneuritis; Acute inflammatory demyelinating

polyneuropathy; Landry's ascending paralysis

Signs and Symptoms

Signs and symptoms can progress relatively quickly once they appear. They typically
start in the feet and/or hands and spread upward to the legs, arms and trunk. At first they
may include:

• Tingling or pins-and-needle sensations

• Numbness
• Tenderness
• Weakness, especially in the legs
• Muscle spasms
• Loss of coordination
• Loss of reflexes

More serious symptoms, some of which may require emergency medical assistance,
include:

• Paralysis
• Difficulty breathing and/or swallowing
• Abnormal heart rate

In most cases, symptoms develop over hours to days and may continue to worsen for up
to a month, after which they slowly resolve. Up to 30% of those affected may still have
some lingering weakness after 3 years, and a small percentage may have a relapse years
later.

Also known as: Infectious polyneuritis; Acute inflammatory demyelinating

polyneuropathy; Landry's ascending paralysis

Tests

Patient history is important in diagnosis. The progression of ascending paralysis –

starting with feet or hands and advancing upward – is a typical presentation. About 50%
of cases also include a history of a recent mild infection or illness like a sore throat, a
cold, the flu, or diarrhea. Several tests are commonly used to diagnose or confirm the
disease and, sometimes, to monitor recovery.

• Cerebrospinal fluid (CSF) analysis – to identify the presence of increased protein;

for this test, a needle is inserted into the spine between vertebrae and a small
amount of fluid is withdrawn. While some protein is normally present, an
 increased amount without an increase in the white blood cells in the CSF may be
indicative of Guillain Barré syndrome.
• Nerve conduction velocity – tests the speed at which impulses travel through a
nerve; the nerve conduction velocity test uses electrodes placed on the skin over
peripheral nerves and measures the amount of time it takes for an impulse to
travel between electrodes.
• Electromyography (EMG) – measures the electrical activity of muscles fibers; the
EMG test measures the electrical activity within muscle fibers by placing a needle
electrode through the skin directly into the muscle and measuring the electrical
activity of that muscle. It is usually done in conjunction with a nerve conduction
velocity test.

There are several variants of GBS that are associated with specific signs and symptoms
and with the production of different types of antibodies directed against gangliosides.
Rarely, ganglioside autoantibody tests may be ordered.

Other testing may be performed to help distinguish GBS from other causes of weakness,
neuropathy, and immune dysfunction and to monitor the person's health status during
illness and recovery.

Also known as: Infectious polyneuritis; Acute inflammatory demyelinating

polyneuropathy; Landry's ascending paralysis

Tests

Patient history is important in diagnosis. The progression of ascending paralysis –

starting with feet or hands and advancing upward – is a typical presentation. About 50%
of cases also include a history of a recent mild infection or illness like a sore throat, a
cold, the flu, or diarrhea. Several tests are commonly used to diagnose or confirm the
disease and, sometimes, to monitor recovery.

• Cerebrospinal fluid (CSF) analysis – to identify the presence of increased protein;

for this test, a needle is inserted into the spine between vertebrae and a small
amount of fluid is withdrawn. While some protein is normally present, an
increased amount without an increase in the white blood cells in the CSF may be
indicative of Guillain Barré syndrome.
• Nerve conduction velocity – tests the speed at which impulses travel through a
nerve; the nerve conduction velocity test uses electrodes placed on the skin over
peripheral nerves and measures the amount of time it takes for an impulse to
travel between electrodes.
• Electromyography (EMG) – measures the electrical activity of muscles fibers; the
EMG test measures the electrical activity within muscle fibers by placing a needle
electrode through the skin directly into the muscle and measuring the electrical
activity of that muscle. It is usually done in conjunction with a nerve conduction
velocity test.
There are several variants of GBS that are associated with specific signs and symptoms
and with the production of different types of antibodies directed against gangliosides.
Rarely, ganglioside autoantibody tests may be ordered.

Other testing may be performed to help distinguish GBS from other causes of weakness,
neuropathy, and immune dysfunction and to monitor the person's health status during
illness and recovery.

Also known as: Infectious polyneuritis; Acute inflammatory demyelinating

polyneuropathy; Landry's ascending paralysis

Treatment

Guillain-Barré syndrome usually resolves on its own. In most cases, symptoms will
stabilize and then begin to resolve within weeks or months. The goals of treatment are to
try to help decrease the severity of symptoms, speed healing, and to prevent and/or
minimize complications. Many people with GBS require hospitalization for careful
monitoring and supportive care. If the symptoms are severe, the person may require
breathing assistance.

Two approaches are sometimes used early in the disease to lessen the severity and hasten
the recovery. Both are intended to decrease the effectiveness of the antibodies that attack
the myelin sheath. Plasmapheresis, a process of removing blood, filtering out the liquid
plasma that contains antibodies that may be involved in the autoimmune disorder, and
then returning the red and white blood cells to the circulation, has proven effective in
some people. Immunoglobulin injections to block the activity of the damaging antibodies
have also been shown to be beneficial to some people.

In the recovery phase, most of those with GBS will need to undergo physical therapy to
help regain muscle strength.
Tingling in arms and hands
(Keywords: Tingling in arms and hands, chiropractic, tingling in legs and feet, spinal
cord )

Tingling in arms and hands, also in the lower limbs and the head and neck are distinct
signs of nerves under stress. One should not jump to conclusions of rare neurological
diseases, but they do occur and we will discuss some later, but rather think of basics.

For more information about Lou Gehrig's disease ... MessageViewer?

em_id=75667.0&dlv_id= 78022

An aside. Neurological diseases strike fear into our hearts, and not without good reason,
and images like this one below flash through our minds. Bad taste? Perhaps, but for the
first time there have been some real break throughs in the prevention and possible
treatment of serious illnesses like Motor Neuron Disease. Read further.

The first thought that should go through your mind should you begin to experience
tingling, is one of adequate rest. Are you getting enough sleep? When did you last take a
holiday? Have you been working too hard, or watching too many thrillers on TV? Or
long hours trolling the net?

"An unhurried sense of time is in itself a form of wealth."

 Bonnie Friedman

"If a cluttered desk is a sign of a cluttered mind, then whatare we to think of an empty
desk?"

Albert Einstein.

The second thought concerns the B vitamin group. They are vitally involved in healthy
nerves, preventing tingling in arms and hands, and legs and feet. Any deficiency will
cause nerve degeneration. One such condition is called Pernicious Anemia, a deficiency
of vitamin B12. Perhaps a B complex supplement? Jabs in the butt have been shown to be
beneficial. Chat to your doctor.

Obviously a B vitamin deficiency means aspects of the diet are lacking. (There is a rare
condition is which B12 is not well absorbed, no matter how much you eat, but we'll
ignore that for the time being.) For more info about foods that you should be consuming
for B vitamins, to prevent tingling in arms and hands, and elsewhere, click here.
VITAMIN B COMPLEX ...

A third thought concerns an irritated nerve in your spine, or even after it emerges from
your spine. There are hundreds of possible causes, but these three webpages will give you
some idea of what I am talking about. For information about possible causes of tingling
in arms and hands, and elsewhere, click here.

• slipped disc in the neck. ARM PAIN ...

• carpal tunnel syndrome ...

• thoracic outlet syndrome ...

And now for a bit of science. Perhaps not your strong point, but it's interesting. Stick with
me!

SPINAL CORD
This is a cross-section of your spinal cord. Ignore the little bits of details, but see two
parts of the cord:

1. The grey matter in the centre, in those funny shaped structures with horns. This is
where the cell bodies of the nerves are. More about that later.
2. The white matter outside of the horns. This is where the long tracts of nerves
travelling up and down your spinal cord to the brain are found.

 Also the motor nerve emerging at the bottom of the picture from the anterior motor
horn in the grey matter on the way to muscles, and the sensory nerve returning higher up
to the posterior sensory horn. Damage to the motor nerve causes muscle weakness, to the
posterior sensory nerve you might experience tingling in arms and hands. Or legs.

Below you can see how it looks in situ in the spine. Now you can see the nerves coming
in and out from of the spine, supplying the tissues of the body. They glide through a
small gap in the spine called a foramen. Again, the one at the top, the sensory nerve
brings information in to the cord, and that at the bottom, the motor nerve, sends
information to the muscles. This is where the so-called Peripheral Nervous System
begins. (as compared to the Central Nervous System, the brain and spinal cord)

It is with this foramen where chiropractors work, nerves being irritated by either a facet
joint injury, or a disc joint, both in close proximity to the mixed nerve in the foramen.
More regularly we see tingling in arms and hands, and legs, but weakness occurs too if
the motor nerve is injured.
Guillain-Barre Syndrome
If you live in a city of 100 000 people, then one person will this year get GBS. Fairly rare
I think you will agree, so don't start assuming that the tingling you are experiencing is
caused by GBS!

It's not known what causes GBS but it usually starts after a viral infection. Heavy sport
while infected has been implicated. It can also be caused by vaccinations, still a very
controversial subject in chiropractic circles, and has been reported after surgery.

It is a devastating, life threatening illness, from which victims usually spontaneously

recover. It strikes both the sensory and motor parts of these peripheral nerve roots, so the
cardinal signs are sudden onset of tingling in arms and hands and legs, usually starting in
the legs, and then quite profound weakness.

For the science bofs, this is a Lower Motor Neuron condition, so the reflexes are
diminished, bilaterally.

Since it may affect breathing, it can be life threatening.

For more detailed information, click here. ( details gbs )

More about PERNICIOUS ANAEMIA

Pernicious Anaemia was a fatal disease until about a century ago.

Bizarre. The very smart doctor who worked this out, cured his patients by regurgitating
his own digestive juices, and feeding it to his patients!

Pernicious Anaemia too causes tingling in arms and hands, but it's a whole different
kettle of fish.

Firstly, the cause is different, a vitamin B-12 deficiency. Whilst this can be a dietary
deficiency, especially for Vegans who eat absolutely no foods from animal sources, but
more usually it is caused by repeated stomach disturbances leading to a chronic gastritis.
For more about indigestion and heartburn, click here. INDIGESTION HEARTBURN ...

Secondly, it is a Central Nervous System disease (brain and spinal cord, remember?!!
We'll do page on Alzheimers later!), so the reflexes are increased and there is spasticity
and muscle spasms.

Take the Alzheimer's Test. How many fff's do you see?

FINISHED FILES ARE THE RE

SULT OF YEARS OF SCIENTI

FIC STUDY COMBINED WITH

THE EXPERIENCE OF YEARS...

 • Alzheimer's Heavy: You counted 3 fff's
• Alzheimer's Light: Not so bad, you saw 4 ffff's
• Senior Moment: You missed only one. 5 fffff's
• Genius: I don't believe it. You got all 6 ffffff's. Well done. For some odd reason
the brain has difficulty processing the word OF.

A bit more SCIENCE, I'm afraid!

We mentioned above the white matter in the spinal cord where the long bundles of nerves
to and from the brain are found. Pernicious Anaemia affects two of them because
Vitamin B-12 is essential for the building blocks of these nerves.

• One of the bundles affected is travelling UP the cord, carrying vital information
TO the brain.
• The other travelling DOWN the cord FROM the brain.

In the next picture, can you see those columns of white matter with the green dots? They
are the so-called Posterior Columns that carry four very important nerve impulses to the
brain, viz the ability to feel

1. light touch
2. knowing where your limbs are in space
3. what is in your hand with your eyes closed
4. vibration

All of this is lost in Pernicious Anaemia which is one of the reasons why the patient
experiences tingling in arms and hands.

Pernicious Anaemia is particularly nasty because it also affects another bundle of nerves
in the white matter, running in the opposite direction, down the cord. Can you see the
Cortico-spinal tract in the next picture? This is the long bundle of motor nerves coming
down the cord from your brain (mine too! I'm using the CST right now to type) in order
to give instructions to the muscles. Take note of it. If it's damaged it produces quite
different signs - weakness. No tingling in arms and hands either because it is a bundle of
motor nerves.

Actually, it's three different tracts. Makes no difference. Because they emerge from the
Medullary Pyramids in the brain, a lesion of the cortico-spinal tract is called 'pyramidal'.
Probably a bit of useless information for you so just forget it. Perhaps if you are a
chiropractic student, rememember that a pyramidal lesion causes spastic weakness.

Can you see why a stroke (just one of many conditions that can affect the Cortico-spinal
tract) produces weakness on the opposite side of the body?

Now, in the picture below, can you see the cell body of the motor neuron? (in the grey
matter), and its Anterior Root (aka Ventral Root) going down to the muscle? This is what
that bastard virus polio attacks. So the victim of polio would experience weakness rather
than tingling in arms and hands and legs. But weakness of a quite different kind. When a
cell body in Anterior Horn (in the grey matter) is attacked one gets a different sort of
weakness, called flaccid paralysis.
Think of the difference between someone with a stroke (spastic paralysis) and another
with polio or Guillain-Barre (flaccid paralysis). Quite different, eh?

AMYOTROPHIC LATERAL SCLEROSIS (ALS)

ALS is a particularly nasty disease because, unlike Guillain-Barre it doesn't
spontaneously resolve, nor like Pernicious Anaemia can it be cured (not yet, anyway) and
unlike Polio which strikes, leaves its mark and move on, ASL just progresses. When the
diagnosis is made, the patient finds their daily life torpedoed and their most fundamental
expectations about life blown to smithereens.

It's not usually hereditary (it is in 10% of cases).

If you live that city of 100 000 people, two will get it this year. They will both die,
usually within about three years, probably because they can't breathe. The muscles just
stop working.
ALS is horrid because it not only attacks the cell body of the motor neuron (same as polio
does), but it also attacks the Corticospinal tract that brings info from the brain to the
ventral motor cell body. Double motor wammy.

The result is that ALS produces a bizarre mixture of signs. Weakness and wasting of
muscles, muscle twitches and cramps, and increased reflexes. For the bofs, Upper Motor
Neuron signs (because the Corticospinal Tract is damaged) and Lower Motor Neuron
signs (because the cell body of the motor neuron is damaged). The end result - severe
paralysis.

Please note. Many of these signs occur in otherwise normal people. The twitching,
cramping, etc. associated with ALS are the result of a dying muscle, therefore these
symptoms without clinical weakness or atrophy of an affected muscle is likely not ALS.
Because you have cramps in a calf muscle does not mean you are about to get a severe
neurological disease! That on its own is usually caused by a mineral deficiency, perhaps
zinc or magnesium, or another mineral.

ALS may start with weakness but not tingling in arms and hands, and clumsiness (such as
in doing up a button) or in the legs, in which case victims begin to stumble; or in the face
- usually first affecting the voice and later swallowing - and finally the trunk muscles so
breathing becomes difficult. This is the usual cause of death.

Lou Gehrig

ALS first hit the headlines when a very famous American baseball player contracted the
disease. It usually starts around 40-50 years old. If you've seen the film, you will not have
forgotten how Lou wacked the bottom of a bottle of tomato sauce and splattered the
whole table.

There is still no known cure or cause for Lou Gehrig's disease(ALS). Is it like polio and
Guillain-Barre, a virus? Probably not. However, just this year 2008, there have been
some extremely interesting developments into the causes of ALS, and with what is
known as a Ketogenic diet for a cure.

RESEARCH
• Netherlands study.

The diet of pre-illness ALS patients was compared with healthy controls, and the
following results were reported:

"A high intake of Poly-unsaturated fatty acids and vitamin E is associated with
a 50–60% decreased risk of developing ALS, and these nutrients appear to
act synergistically." For more information, click here. ( abstract/78/4/367 )

• Japanese study
 Again the diet of pre-illness ALS sufferers was compared with a normal group,
and researchers came to the conclusion:

"Our findings suggest that high intakes of carbohydrate and low intakes of
fat and some kinds of fatty acids may, when combined, increased the risk of
ALS." For more info, click here. ( content~content=a780527171~ db=all )

A bit of History
In the 1920's an American chiropractor /osteopath, Dr Conklin postulated that epilepsy
was caused by toxic chemicals in the brain, and put patients on long fasts of up to three
weeks. Pretty extreme, I think you will agree, but the results were impressive, and despite
his critics, considerable interest ensued.

This produces a state known as ketosis, in which the body is forced to derive energy from
body protein and fat in the absence of any dietary calories. Exactly how ketosis helps
epilepsy is still not known, and much research is currently being done.

Then the Mayo clinic started a treatment program, less severe than fasting, which
produced similar effects with ketone bodies. The diet was very low in carbohydrate, with
adequate protein for growth, and high in fat. It too produced convincing results with
epileptic children.

Interest then waned as anti-convulsive drugs started coming on to the market, and interest
in Ketogenic diets lessened to the extent that it was almost non-existent.

However, some clinics continued the use of the Ketogenic diet, one of which was the
Johns Hopkins clinic. What really got the ball rolling was enormous interest generated by
the successful treatment of Charlie Abrahams, son of a famous Hollywood producer, who
despite medication suffered from uncontrollable seizures. Charlie's father then produced a
film, First, Do No Harm, featuring Meryl Streep. Roughly 30% of children on the
ketogenic diet had either no seizures, or they were 90% lessened. Most remain well
controlled after returning to a 'normal' diet.

The rest is history. The surge in interest in the Ketogenic diet, not just for seizures but for
all neurological diseases soared.

Conclusions
Much of this confirms what we already know. A diet high in starches, particularly simple
carbohydrates like sugar, may be extemely toxic to the body.

Secondly, that nerves are made primarily of fat, and that many of these diseases have to
do with fat metabolism. Interestingly, on this high fat Ketogenic diet, the children did not
get fat. In fact, they lost weight. The children of Europe and America are not fat simply
because they eat too much fat, but too much starch and sugar.

A diet rich in 'good' fats is extremely beneficial to the body. Where the research is now
heading is whether long chain fatty acids are better, or mono-unsaturated fats, or ... and
what are the best ratios, and sources, of these different oils. More fish oil? Or Avocados?
More Olive oil or Evening Primrose oil? Cold pressed Sunflower ...Salvia Hispanica?
Time will tell. Meantime, if a loved one starts with signs of a serious neurological
disease, you know if which direction to guide them. Even Parkinsons is showing
promising results.

Hint This salad (without the tomato) would score high on the ketogenic diet. Perhaps add
more Feta (or tuna) and plenty of olive oil. Click here for an Olive garden salad recipe ...

 Worms for diseases of the Immune System ? Are you serious? Deadly serious!

Strike Four Walk

Research costs money. Big money. The kind of research that cracks frontiers takes the
best minds and highly sophisticated equipment.

We started this page with a depressing picture of a skeleton. I've pondered whether it was
in poor taste. However, the reality of these nasty neurological disease is that people for
no obvious reason, get sick and die.

On the hand, for the first time there is now hope for sufferers of diseases like ALS. And
it's only because of top thinkers who are stretching their minds, hoping against hope that
they will discover a way forwards to stretch out the lives of the sick.

Strike Four is a team of walkers dedicated to support these researchers. Will you help
them strike ALS out? For more information, click here. Web alsa

This page is still under construction. More will be added about tingling in arms and hands
and feet and face. And about paralysis.