Guillain-Barré Syndrome

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Definition

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

Description

The classic scenario in GBS involves a patient who has just recovered from a typical, seemingly uncomplicated viral infection. Symptoms of muscle weakness appear one to four weeks later. The most common preceding infections are cytomegalovirus, herpes, Epstein-Barr virus, and viral hepatitis. A gastrointestinal infection with the bacteria Campylobacter jejuni is also common and may cause a severe type of GBS from which it is particularly difficult to recover. About 5% of GBS patients have a surgical procedure as a preceding event. Patients with lymphoma, systemic lupus erythematosus, or AIDS have a higher than normal risk of GBS. Other GBS patients have recently received an immunization, while still others have no known preceding event. In 1976–77, there was a vastly increased number of GBS cases among people who had been recently vaccinated against the Swine flu. The reason for this phenomenon has never been identified, and no other flu vaccine has caused such an increase in GBS cases.

— Rosalyn Carson-DeWitt, MD

A temporary inflammation of the nerves, causing pain, weakness, and paralysis in the extremities and often progressing to the chest and face. It typically occurs after recovery from a viral infection or, in rare cases, following immunization for influenza.

[After Georges Guillain (1876–1961) and and Jean Alexandre Barré (1880–1967), French neurologists.]

Definition

Guillain-Barré syndrome (GBS) is an inflammation of the covering that surrounds nerve cells of the brain and spinal cord. The basis of the inflammation is not conclusively known, but is generally considered to arise from a malfunctioning immune system that recognizes host tissues as being foreign. The inflammation reaction damages the nerves of the brain and spinal cord, producing weakness in the muscles, loss of sensation (such as the sense of touch in the fingers), or outright paralysis.

GBS is termed a syndrome rather than a disease because there is no conclusive evidence to support the possibility that a specific disease-causing agent such as a bacteria or a virus is the direct cause of the malady. Infections may be a trigger to the development of GBS, however.

Description

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

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

The syndrome is an inflammatory disorder, in which a person's own immune system attacks the nerves outside the brain and the spinal cord. These nerves are known as peripheral nerves. The nerve inflammation that occurs can damage the nerve cells. The covering (sheath) of a fatty material called myelin that surrounds the cells can be lost. This loss is called demyelination.

Additionally, the elongated portion of the nerve cell called the axon can be killed. This phenomenon is called denervation. The axon conveys electrical impulses to more distant areas of muscles, and from one nerve cell to another. Demyelination and denervation bring about muscle weakness, loss of sensation, or paralysis because the affected nerves cannot transmit signals to muscles. This loss of signal transmission inhibits the muscles from being able to respond to nerve signals. As well, the brain receives fewer signals and the person can become unable to feel heat, cold, or pain.

GBS is also known as Landry-Guillain-Barré syndrome, acute idiopathic polyneuritis, infectious polyneuritis, and acute inflammatory demyelinating polyneuropathy (AIDP). Another malady called chronic inflammatory demyelinating polyradicalneuropathy is possibly related to GBS. It is far less common than GBS (which itself is rare) and persists longer.

Demographics

GBS can occur at any age. However, the syndrome tends to be more prevalent in men and women aged 15–35 years and 50–75 years (a bimodal pattern of age distribution), respectively. Males are slightly more susceptible than females (the ratio of those affected is approximately 1.5 male per female). There is no known racial group that is any more susceptible to GBS, nor any known geographical localization of the syndrome.

In the United States, the syndrome is rare. For example, the annual incidence of GBS in the United States ranges from 0.6 to 2.4 cases per 100,000 people. Nonetheless, GBS is the most common cause of neuromuscular paralysis among Americans.

Causes and symptoms

Causes

The exact cause of GBS is not known. However, bacterial or viral infections may be a trigger for its development. Almost 70% of those who develop GBS have had an infectious illness in the preceding two to four weeks. Examples of infections include sore throat, cold, flu, and diarrhea. Bacteria that have been associated with the subsequent development of GBS include chlamydia, Mycoplasma pneumoniae, and Campylobacter jejuni.

The suspected involvement of Campylobacter is noteworthy, as this bacterium is a common contaminant of poultry. Inadequate cooking can allow the microbe to survive and cause an infection in those who consume the food. Thus, there may be a connection between GBS and food quality. The form of GBS that may be associated with the presence of Campylobacter may be particularly severe. For reasons that are unclear, the peripheral nerves can themselves be directly attacked, rather than just the myelin sheath around the nerves.

Usually, infections such as those caused by Campylobacter have abated before the onset of GBS. As well, chronic infection with the viruses responsible for mononucleosis, herpes, and acquired immunodeficiency syndrome can prelude the appearance of GBS. The latter is also known as HIV-1 associated acute inflammatory demyelinating polyneuropathy.

Other possible associated factors include vaccination (rabies, swine flu, influenza, Group A streptococci), surgery, pregnancy, and maladies such as Hodgkin's disease and systematic lupus erythematosus.

Whether there is direct (causal) connection between infections and maladies and the subsequent development of GBS, or whether the events are only coincidental, is not known. For example, vaccination of Americans against the swine flu in 1976 increased the rate of GBS by less than one case per 100,000 people. Whether this increase was directly due to the vaccine is impossible to determine. Furthermore, more than 99% of people suffering from GBS who have been surveyed by the United States Centers for Disease Control and Prevention (CDC) have not recently been vaccinated. According to the CDC, the chance of developing GBS as a result of vaccination is remote.

It is conceivable that the infections or illnesses disrupt the body's immune system such that autoimmune destruction of nerve cell components occurs. Although this intriguing possibility is favored among many scientists, it remains unsubstantiated.

There is no evidence to indicate that GBS is an infection or that it is a genetically linked (heritable) disorder.

Symptoms

The initial sensation of weakness or paralysis in the toes spreads upward within days to a few weeks to the arms and the central part of the body. In medical terminology, this represents an ascending pattern of spread. The weakness and paralysis can also be accompanied by a tingling sensation, and a cramping or more constant pain in the feet, hands, thighs, shoulders, lower back, and buttocks. Use of the hands and feet can become impaired. More serious development of paralysis can make breathing difficult, even to the point that mechanical ventilation becomes necessary.

Other, less typical symptoms include blurred vision, clumsiness, difficulty in moving facial muscles, involuntary muscle contractions, and a pronounced heartbeat. Symptoms that are indicative of an emergency include difficulty in swallowing, drooling, breathing difficulty, and fainting.

Progression from the early symptoms to the more severe symptoms can occur very quickly (i.e., 24–72 hours). Typically, the exacerbated condition persists for several weeks. Recovery then typically occurs gradually, and can take anywhere from days to six months or more.

In very mild cases, an individual may just have a general feeling of weakness. As the symptoms abate after a few weeks, the person may dismiss the incident as a viral infection, without ever knowing the true nature of the illness.

Diagnosis

GBS is suspected if a patient displays muscle weakness or paralysis that has been increasing in severity, especially if an illness has occurred recently. Loss of reflexes such as the knee jerk reaction can be an early clue to a clinician.

Clinical data can be useful in diagnosis. For example, a hormone that is involved in maintaining the proper chemical balance of urine can be affected in GBS. The result is called the syndrome of inappropriate antidiuretic hormone. Antibodies to nerve cells may be present as a result of the body's immune reaction against its own constituents.

Another clue to the diagnosis of GBS can be the finding of muscle weakness by neurological examination. One such test is known as nerve conduction velocity. In this test, the selected nerve is stimulated, usually with surface electrodes contained in a patch that is applied to the surface of the skin. The nerve can be stimulated using a very mild electrical current put out from one electrode, and the resulting electrical activity is recorded by the other electrodes in the patch. The nerve conduction velocity is calculated knowing the distance between electrodes and measuring the time it takes for the impulses to travel from the generating to the measuring electrodes. A person with GBS whose nerves have usually lost some or most of the myelin sheath will display a slower conduction velocity than that displayed by an unaffected person. Electrical impulses travel along the damaged nerve slower than along an undamaged nerve.

Muscle response to electrical stimulation can also be measured by electromyography (EMG). In this test, a needle electrode is inserted through the skin into the muscle. When the muscle is stimulated, for example, by contracting it, the resulting visual or audio pattern carries the information about the muscle's response. The characteristic pattern of wavelengths produced by a healthy muscle (the action potential) can be compared to a muscle in someone suspected of having GBS.

When paralysis of the heart muscle is suspected, an electrocardiogram can be used to record the electrical activity of the heart. GBS muscle paralysis can alter the normal pattern of the heartbeat.

Finally, an examination of the cerebrospinal fluid by means of a spinal tap (also known as a lumbar puncture) may detect a higher-than-normal level of protein in the absence of an increase in the number of white blood cells (WBCs). An increase in WBCs is a hallmark of an infection.

Treatment team

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

Treatment

As recently as the 1980s, treatment for GBS consisted of letting the syndrome run its course. While most people recovered completely with time, some people were not as lucky. Those who develop severe symptoms such as breathing difficulty are routinely hospitalized.

One medical procedure that can be useful in the treatment of GBS is called plasmaphoresis. It is also known as plasma exchange. In plasmapheresis, antibody-laden blood plasma (the liquid portion of the blood) is removed from the body. Red blood cells are separated and put back into the body with antibody-free plasma or intravenous fluid. The treatment can lessen the symptoms of GBS and hasten recovery time. As of December 2003, it is not known why plasmapheresis works. It is suspected that the removal of antibodies may lessen the effects of the body's immune attack on the nerve cells.

Another procedure that produces similar results involves the administration of intravenous immune globulin (IVIG). Both treatments have been shown to speed up recovery time by up to 50%. IVIG has been shown to be an effective treatment for immune-system-related neuropathies in general. IVIG may act by reducing the amount of anti-myelin antibodies through the binding of the defective antibodies by healthy antibodies contained in the IVIG solution, and in suppressing the immune response.

Other treatments are designed to prevent or lessen complications of GBS. For example, choking during eating, because of throat muscle weakness or paralysis, can be prevented using a feeding tube, and formation of blood clots can be lessened by the use of chemicals that thin the blood. The pain associated with GBS can be treated with anti-inflammatory drugs or, if necessary, stronger-acting narcotic medication. For patients who have breathing difficulties, clinicians may first need to supply oxygen, install a breathing tube (intubation), and/or use a mechanical device that helps in breathing.

Physical therapy is helpful. Caregivers can move a patient's arms and legs to help maintain the flexibility and strength of the muscles. Later in recovery, sessions in a whirlpool (hydrotherapy) can help restore function to arms and legs. Often, therapists will design a series of exercises to be performed when the patient returns home.

Recovery and rehabilitation

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

Clinical trials

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

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

Prognosis

Most of those afflicted with GBS recover completely, although the recovery can in some cases be slow (months to years). Complete recovery usually occurs when the symptoms fade within three weeks of appearing. The typical scenario is for a patient to experience the most weakness from 10–14 days after the appearance of symptoms, with complete recovery occurring within weeks or a few months. In contrast, a poor prognosis can be associated with a rapid appearance of symptoms, use of assisted ventilation for a month or more, severe nerve damage, and with advancing age.

While recovery is complete for most of those afflicted with GBS, in 10–20% of cases the symptoms reappear, in 15–20% the neurologic complications can persist and can cause a long-term disability, and 5–10% of those who are afflicted die. The main cause of death historically was from respiratory failure due to muscle paralysis. With mechanical ventilation, respiratory failure in GBS is less often fatal. Currently the main cause of death is malfunctioning of the autonomic nervous system, which controls involuntary processes such as heart rate, blood pressure, and body temperature.

Resources

PERIODICALS

Quarles, R. H., and M. D. Weiss. "Autoantibodies Associated with Peripheral Neuropathy." Muscle Nerve (July 1999): 800–822.

OTHER

Guillain-Barré Syndrome (GBS) and Influenzae Vaccine. Centers for Disease Control and Prevention. CDC. December 15, 2003 (April 4, 2004). http://www.cdc.gov/nip/vacsafe/concerns/GBS/default.htm.

Fanion, David, and Daniel M. Joyce. "Guillain-Barré Syndrome." eMedicine. December 12, 2003 (April 4, 2004). http://www.emedicine.com/EMERG/topic222.htm.

Mayo Foundation for Medical Education and Research. "Guillain-Barré Syndrome." MayoClinic.com. December 13, 2003 (April 4, 2004). http://www.mayoclinic.com/invoke.cfm?id=DS00413.

National Institutes of Health. "Guillain-Barré Syndrome." MEDLINEplus Medical Encyclopedia. December 13, 2003 (April 4, 2004). http://www.nlm.nih.gov/medlineplus/ency/article/000684.htm.

NINDS Guillain-Barré Syndrome Information Page. National Institute of Neurological Disorders and Stroke. December 10, 2003 (April 4, 2004). http://www.ninds.nih.gov/health_and_medical/disorders/gbs.htm.

ORGANIZATIONS

Centers for Disease Control and Prevention. 1600 Clifton Road, Atlanta, GA 30333. (404) 639-3311 or (800) 311-3435. http://www.cdc.gov/.

Guillain-Barré Syndrome Foundation International. P.O. Box 262, Wynnewood, PA 19096. (610) 667-0131; Fax: (610) 667-7036. info@gbsfi.com. http://www.gbsfi.com.

National Institutes of Health. 9000 Rockville Pike, Bethesda, MD 20892. (301) 496-4000. NIHInfo@od.nih.gov. http://www.nih.gov.

National Institute for Neurological Disorders and Stroke. P.O. Box 5801, Bethesda, MD 20824. (301) 496-5761 or (800) 352-9424. http://www.ninds.nih.gov.

Brian Douglas Hoyle, PhD

A relatively rare disease of humans affecting the peripheral nervous system, especially the spinal nerves, but also the cranial nerves. Often observed after parenteral administration of drugs and vaccines. Pathological changes include demyelination, inflammation, edema and nerve root decompression. Called also acute idiopathic polyneuritis, postinfectious polyneuritis and Landry's paralysis. The cause is unknown but is believed to be related to an autoimmune mechanism. Idiopathic polyradiculoneuritis (coonhound paralysis) in dogs and cauda equina neuritis in horses are similar.

Guillain-Barré syndrome
Classification and external resources
ICD-10	G61.0
ICD-9	357.0
OMIM	139393
DiseasesDB	5465
MedlinePlus	000684
eMedicine	emerg/222  neuro/7 pmr/48 neuro/598
MeSH	D020275

Guillain-Barré syndrome (GBS) (French pronunciation: [ɡiˈlɛ̃ baˈʁe];^[1][2] in English, pronounced /ˈɡiːlæn ˈbɑreɪ/,^[3] /ɡiːˈlæn bəˈreɪ/,^[4] etc.^[5]) is an acute inflammatory demyelinating polyneuropathy (AIDP), an autoimmune disorder affecting the peripheral nervous system, usually triggered by an acute infectious process. It is included in the wider group of peripheral neuropathies. There are several types of GBS, but unless otherwise stated, GBS refers to the most common form, AIDP. It is frequently severe and usually exhibits as an ascending paralysis noted by weakness in the legs that spreads to the upper limbs and the face along with complete loss of deep tendon reflexes. With prompt treatment by plasmapheresis or intravenous immunoglobulins and supportive care, the majority of patients will regain full functional capacity. However, death may occur if severe pulmonary complications and dysautonomia are present.^[6]

Contents 1 Pathophysiology 2 Signs and symptoms 2.1 Clinical variants 3 Diagnosis 3.1 Diagnostic criteria 3.1.1 Required 3.1.2 Supportive 3.2 Differential diagnosis 4 Treatment 5 Prognosis 6 History 7 Notable sufferers 8 References 9 External links

Pathophysiology

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All forms of Guillain-Barré syndrome are due to an immune response to foreign antigens (such as infectious agents) that are mistargeted at host nerve tissues instead (a form of antigenic mimicry). The targets of such immune attack are thought to be gangliosides, which are complex glycosphingolipids present in large quantities on human nerve tissues, especially in the nodes of Ranvier. An example is the GM1 ganglioside, which can be affected in as many as 20-50% of cases, especially in those preceded by Campylobacter jejuni infections. Another example is the GQ1b ganglioside, which is the target in the Miller Fisher syndrome variant (see below).

The most common antecedent infection is Campylobacter jejuni.^[7] However, 60% of cases do not have a known cause.^[8]

The end result of such autoimmune attack on the peripheral nerves is inflammation of myelin and conduction block, leading to a muscle paralysis that may be accompanied by sensory or autonomic disturbances.

However, in mild cases, axonal function remains intact and recovery can be rapid if remyelination occurs. In severe cases, such as in the AMAN or AMSAN variants (see 2.1 below), axonal degeneration occurs, and recovery depends on axonal regeneration. Recovery becomes much slower, and there is a greater degree of residual damage. 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.

GBS is a rare side-effect of influenza vaccines, with an incidence of about one case per million vaccinations.^[9] Other estimates suggest the incidence of GBS among those receiving the vaccine was one case per 105,000 and that the GBS was not directly due to the vaccine but to its being contaminated with a bacterium that triggers GBS.^[10]

There were reports of GBS affecting some people who had received swine flu immunizations in the 1976 U.S. outbreak of swine flu. Overall, there were about 500 cases of GBS—25 of which resulted in death from severe pulmonary complications—which, according to Dr. P. Haber, were probably caused by an immunopathological reaction to the 1976 swine flu vaccine. Other influenza vaccines have not been linked to GBS, though caution is advised for certain individuals, particularly those with a history of GBS.^[11][12]

Serum sickness can rarely manifest as the Guillain-Barre syndrome (GBS), one of its most serious complications. The most serious complications of serum sickness are nerve conditions and peripheral neuritis. Serum sickness is of itself a type of delayed allergic response.

GBS is a form of autoimmune disorder with a delayed hypersensitivity reaction, or a rare manifestation of serum sickness, or transient syndrome resembling serum sickness with loss of appetite, nausea, vomiting, and stomach pain accompanied by weakness (tired feeling), chills, low grade fever and possible evidence of brain involvement, indicated by lethargy and migraine headaches, although one theory of the cause of migraine is a central nervous system (CNS) disorder, or Bickerstaff's brain stem encephalitis, a regional variant of GBS. Typical pain is occipital or in the back of the head. Alterations of consciousness go with this headache type affecting the brainstem implicated in the maintenance of arousal, but is a worrisome feature of this type of headache called a Bickerstaff migraine. Other features of Bickerstaff migraine go with involvement primarily of the brainstem including clumsiness and gait unsteadiness, e.g. "pulling to the right"..

Signs and symptoms

The disorder is characterized by weakness which affects the lower limbs first, and rapidly progresses in an ascending fashion. Patients generally notice weakness in their legs, manifesting as "rubbery legs" or legs that tend to buckle, with or without dysesthesias (numbness or tingling). As the weakness progresses upward, usually over periods of hours to days, the arms and facial muscles also become affected. Frequently, the lower cranial nerves may be affected, leading to bulbar weakness, (oropharyngeal dysphagia, that is difficulty with swallowing, drooling, and/or maintaining an open airway) and respiratory difficulties. Most patients require hospitalization and about 30% require ventilatory assistance. 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-Barre syndrome is usually related to sodium channel blocking factor in the cerebrospinal fluid. Morbid and iatrogenic events involving IV salt and water may occur unpredictably in this patient group, resulting in SIADH. SIADH is often the first symptom of Guillain–Barré syndrome. Sodium overload is almost always iatrogenic. Rapid correction of hyponatraemia can cause central pontine demyelination.^{[citation needed]}

The symptoms are similar to those for progressive inflammatory neuropathy.^[13]

Clinical variants

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

• Acute inflammatory demyelinating polyneuropathy (AIDP) is the most common form of GBS, and the term is often used synonymously with GBS. It is caused by an auto-immune response directed against Schwann cell 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 ocular 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)^[14], aka. Chinese Paralytic Syndrome, attacks motor nodes of Ranvier and is prevalent in China and Mexico. It is likely due to an auto-immune response directed against the axoplasm of peripheral nerves. The disease may be seasonal and recovery can be rapid. Anti-GD1a antibodies^[15] are present. Anti-GD3 antibodies are found more frequently in AMAN.

• Acute motor sensory axonal neuropathy (AMSAN) is similar to AMAN but also affects sensory nerves with severe axonal damage. Like AMAN, it is likely due to an auto-immune response directed against the axoplasm of peripheral nerves. Recovery is slow and often incomplete.^[16]

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

• Bickerstaff’s brainstem encephalitis (BBE), is further variant of Guillain-Barré syndrome. It is characterized by acute onset of ophthalmoplegia, ataxia, disturbance of consciousness, hyperreflexia or Babinski’s sign (Bickerstaff, 1957; Al-Din et al.,1982). The course of the disease can be monophasic or remitting-relapsing. Large, irregular hyperintense lesions located mainly in the brainstem, especially in the pons, midbrain and medulla are described in the literature. BBE despite severe initial presentation usually has a good prognosis. 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.

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. CSF and ECD is used almost every time to verify symptoms, but because of the acute nature of the disorder, they may not become abnormal until after the first week of onset of signs and symptoms.

There currently is no cure for Guillain-Barre syndrome. However, treatments have been proven effective against this syndrome.

• CSF

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 pleocytosis (increased cell count). A sustained pleocytosis may indicate an alternative diagnosis such as infection.

Researchers speculate as to why protein is elevated without pleocytosis in the CSF. Infiltration of white blood cells in the myelin are responsible for demyelination in the peripheral nerves. Specific 2D gel electrophoresis showed proteins unspecifically affected in different inflammatory and non-inflammatory neurological diseases, and may be of limited value as disorder-related biochemical markers in GBS. [Proteome analysis of cerebrospinal fluid in Guillain–Barré syndrome.]

• Electrodiagnostics

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

Diagnostic criteria

Required

• Progressive, relatively symmetrical weakness of 2 or more limbs due to neuropathy
• Areflexia
• Disorder course < 4 weeks
• Exclusion of other causes (see below)

Supportive

• relatively symmetric weakness accompanied by numbness and/or tingling
• mild sensory involvement
• facial nerve or other cranial nerve involvement
• absence of fever
• typical CSF findings obtained from lumbar puncture
• electrophysiologic evidence of demyelination from electromyogram

Differential diagnosis

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

Treatment

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

Once the patient is stabilized, treatment of the underlying condition should be initiated as soon as possible. Either high-dose intravenous immunoglobulins (IVIg) at 400 mg/kg for 5 days or plasmapheresis can be administered, as they are equally effective and a combination of the two is not significantly better than either alone. Therapy is no longer effective 2 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) is 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, there will be established a team of different therapists and nurses according to patient needs. An occupational therapist can offer equipment (such as wheel chair and cutlery) to help the patient achieve ADL independence. A physiotherapist would plan a progressive training programme, 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 with their knowledge to guide the patient towards his or her goals, and it is important that all goals set by the separate team members are relevant for the patient's own priorities. After rehabilitation the patient should be able to function in his or her own home and attend necessary training as needed.

Prognosis

Most of the time recovery starts after 4th 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 4 to 6 weeks, and in some cases up to 1 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).

Case reports do exist of rapid patient recovery.

History

The disorder was first described by the French physician Jean Landry in 1859. In 1916, Georges Guillain, Jean Alexandre Barré, and Andre Strohl diagnosed two soldiers with the illness and discovered the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count.^[17]

GBS is also known as acute inflammatory demyelinating polyneuropathy, acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French Polio, Landry's ascending paralysis and Landry Guillain Barre syndrome.

Notable sufferers

• Morten Wieghorst, International Danish footballer who once won the Scottish Football Championship with Celtic FC. He was diagnosed in 2000, and missed a year of his footballing career before returning in November 2001. He played 2 games for Celtic before returning to Denmark with Brøndby IF.
• Andy Griffith, actor on Andy Griffith Show, and Matlock. He developed Guillain-Barré in 1983. ^[18]
• Rachel Chagall, actress, contracted GBS in 1982. In 1987 she portrayed Gabriela Brimmer, a notable disabilities activist. ^[19]
• Joseph Heller, author, contracted GBS in 1981. This episode in his life is recounted in the autobiographical No Laughing Matter, which contains alternating chapters by Heller and his good friend Speed Vogel.^[20]
• Raymond J. Wieczorek, former Mayor, Manchester, New Hampshire and current Member of New Hampshire Executive Council. He developed Guillain-Barré in 1993, but recovered and served three additional terms as mayor.
• Franklin D. Roosevelt, U.S. president. In 2003, a peer-reviewed study^[21] found that it was more likely that Roosevelt's paralysis--long attributed to poliomyelitis--was actually Guillain-Barré syndrome.
• Markus Babbel, former international footballer, contracted GBS in 2001, following a period suffering from the Epstein-Barr virus. He lost almost an entire year of his footballing career between the two illnesses and never again demonstrated the same level of ability that won him over 50 caps for Germany.^[22]
• Serge Payer, Canadian-born professional hockey player. After battling and overcoming the syndrome, He set up the Serge Payer foundation, which is dedicated to raising money for research into new treatments and cures for Guillain-Barré syndrome^[23].
• Lucky Oceans, Grammy Award winning musician with Asleep at the Wheel was diagnosed with GBS in 2008.^[24]
• William “The Refrigerator” Perry, former professional American football player with the Chicago Bears was diagnosed with GBS in 2008.^[25]
• Tony Benn, British politician.^[26]
• Len Pasquarelli, sports writer and analyst for ESPN and resident of the Pro Football Writers of America, diagnosed in 2008.^[27]

References

1. ^ "John Wells's phonetic blog, 23rd February, 2007". http://www.phon.ucl.ac.uk/home/wells/blog0702b.htm. 
2. ^ "See also, in the same blog, the entry of October 20th, 2008". http://www.phon.ucl.ac.uk/home/wells/blog.htm. 
3. ^ Recommended by the "GBS Support Group". http://www.gbs.org.uk/quickguide.html. 
4. ^ "Guillain-Barre Syndrome". Dictionary.com Unabridged (v 1.1). Random House. http://dictionary.reference.com/browse/Guillain-Barre%20Syndrome. 
5. ^ In English, Guillain may be pronounced with an L sound as in French, but it is common to pronounce it without one, originally based on the mistaken assumption that the French pronunciation of the ll is [j] and not [l]. In English, both Guillain and Barré may be pronounced with the stress on either the first or the last syllable. The nasal vowel [ɛ̃] at the end of Guillain is either kept in English or replaced by a sequence of an oral vowel and a nasal consonant such as [æn].
6. ^ www.nlm.nih.gov/medlineplus/ency/article/000777.htm
7. ^ PMID 15304587
8. ^ PMID 19025491
9. ^ Vellozzi C, Burwen DR, Dobardzic A, Ball R, Walton K, Haber P (March 2009). "Safety of trivalent inactivated influenza vaccines in adults: Background for pandemic influenza vaccine safety monitoring". Vaccine 27 (15): 2114–2120. doi:10.1016/j.vaccine.2009.01.125. PMID 19356614. 
10. ^ "FLU SHOTS: Vaccine decisions complex". http://www.lvrj.com/news/45975417.html. 
11. ^ Haber P, Sejvar J, Mikaeloff Y, Destefano F (2009). "Vaccines and guillain-barré syndrome". Drug Saf 32 (4): 309–23.. doi:10.2165/00002018-200932040-00005 (inactive 2009-04-26). PMID 19388722. 
12. ^ "Influenza / Flu Vaccine". University of Illinois at Springfield. http://www.uis.edu/healthservices/immunizations/influenzavaccine.html. Retrieved on 26 April 2009. 
13. ^ David Brown (2008-02-04). "Inhaling Pig Brains May Be Cause of New Illness". The Washington Post. http://www.washingtonpost.com/wp-dyn/content/article/2008/02/03/AR2008020302580.html?hpid=topnews. Retrieved on 2008-02-04. 
14. ^ McKhann GM, Cornblath DR, Ho T, et al (1991). "Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China". Lancet 338 (8767): 593–7. doi:10.1016/0140-6736(91)90606-P. PMID 1679153. 
15. ^ Ho TW, Mishu B, Li CY, et al (1995). "Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies". Brain 118 ( Pt 3): 597–605. doi:10.1093/brain/118.3.597. PMID 7600081. http://brain.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=7600081. 
16. ^ Griffin JW, Li CY, Ho TW, et al (1995). "Guillain-Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases". Brain 118 ( Pt 3): 577–95. doi:10.1093/brain/118.3.577. PMID 7600080. http://brain.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=7600080. 
17. ^ Guillain-Barré-Strohl syndrome and Miller Fisher's syndrome at Who Named It?
18. ^ "Andy in Guidepos ts Magazine". http://www.mayberry.com/tagsrwc/wbmutbb/anewsome/private/guidpost.htm. 
19. ^ "Gaby, A True Story (1987)". Films involving Disabilities. http://www.disabilityfilms.co.uk/general1/gabyatruestory.htm. 
20. ^ Vogel, Speed; Heller, Joseph (2004). No Laughing Matter. New York: Simon & Schuster. ISBN 0-7432-4717-5. 
21. ^ Goldman AS, Schmalstieg EJ, Freeman DH, Goldman DA, Schmalstieg FC (2003). "What was the cause of Franklin Delano Roosevelt's paralytic illness?" (PDF). J Med Biogr 11 (4): 232–40. PMID 14562158. http://web.archive.org/web/20080307005449/http://www.rsmpress.co.uk/jmb_2003_v11_p232-240.pdf. 
22. ^ http://www.telegraph.co.uk/sport/main.jhtml?xml=/sport/2002/08/10/sfnliv10.xml
23. ^ Serge Payer Foundation, Serge Payer Foundation Mission.
24. ^ "Lucky Oceans in hospital". The Australian. 2008-10-13. http://www.theaustralian.news.com.au/story/0,,24485771-22822,00.html. Retrieved on 2008-10-28. 
25. ^ . YumaSun.com. 2008-09-08. http://www.yumasun.com/sports/tatum_44249___article.html/perry_night.html. Retrieved on 2008-10-28. 
26. ^ "Relative Values: Tony and Josh Benn". The Times. 2002-10-17. http://www.timesonline.co.uk/tol/life_and_style/article824739.ece. Retrieved on 2009-01-15. 
27. ^ "Chris Mortensen on Len Pasquarelli's comeback". ESPN.com. 2009-01-26. http://sports.espn.go.com/nfl/playoffs2008/columns/story?id=3861360. Retrieved on 2009-01-26. 

External links

• GBS/CIDP Foundation International
• Guillain-Barré Syndrome Support Group (UK and Ireland)
• Information from GBS Association of New South Wales (AU)
• miller_fisher at NINDS
• Lucky Oceans interview - Oceans is interviewed by Dr Norman Swan on ABC Radio National - (8 March 2009)

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