Guillain-Barré syndrome
Encyclopedia
Guillain–Barré syndrome (ɡiˈlɛ̃ baˈʁe, lang), sometimes called 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 immunoglobulin
s 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 the most common cause 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.
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
for treatment of Type II respiratory failure
. 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 analgesic
s. 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-Barré 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
.
. 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.
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.
(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). 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 United States Center of Disease Control to end that immunization campaign within the United States. (By comparison, the average flu season kills around 30,000 people in the United States). 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. Furthermore, the GBS may not have been directly due to the vaccine but to a bacterial contamination of the 1976 vaccine.
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.
From October 6 to November 24, 2009, the U.S. CDC, through the VAERS reporting system, received ten reports of Guillain-Barré 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
case criteria for Guillain–Barré syndrome, while four do not meet the criteria and four remain under review. 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.
Although one review gives an incidence of about one case per million vaccinations, 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-Barré syndrome (0.1 per million) 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."
Supportive:
Differential diagnosis
:
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, 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. There is also some evidence supporting physiotherapy
in helping patients with Guillain–Barré syndrome regain strength, endurance, and gait quality, as well as helping them prevent contractures, bedsores, and cardiopulmonary difficulties. 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.
(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.
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.
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.
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.
.jpg)
Peripheral nervous system
The peripheral nervous system consists of the nerves and ganglia outside of the brain and spinal cord. The main function of the PNS is to connect the central nervous system to the limbs and organs. Unlike the CNS, the PNS is not protected by the bone of spine and skull, or by the blood–brain...
. Ascending paralysis
Ascending paralysis
Ascending paralysis refers to a form of paralysis where the presentation appears in the lower limbs before the upper limbs.It can be associated with:* Guillain–Barré syndrome *Tick 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
Muscles of respiration
The various muscles of respiration aid in both inspiration and expiration, which require changes in the pressure within the thoracic cavity. The respiratory muscles work to achieve this by changing the dimensions of the thoracic cavity....
are affected or if there is dysfunction of the autonomic nervous system
Dysautonomia
Dysautonomia is a broad term that describes any disease or malfunction of the autonomic nervous system. This includes postural orthostatic tachycardia syndrome , inappropriate sinus tachycardia , vasovagal syncope, mitral valve prolapse dysautonomia, pure autonomic failure, neurocardiogenic...
. The disease is usually triggered by an acute infection. Guillain–Barré syndrome is a form of peripheral neuropathy
Peripheral neuropathy
Peripheral neuropathy is the term for damage to nerves of the peripheral nervous system, which may be caused either by diseases of or trauma to the nerve or the side-effects of systemic illness....
.
The diagnosis is usually made by nerve conduction studies
Nerve conduction study
A nerve conduction study is a test commonly used to evaluate the function, especially the ability of electrical conduction, of the motor and sensory nerves of the human body.Nerve conduction velocity is a common measurement made during this test...
. With prompt treatment by intravenous immunoglobulin
Intravenous immunoglobulin
Intravenous immunoglobulin is a blood product administered intravenously. It contains the pooled IgG extracted from the plasma of over one thousand blood donors. IVIG's effects last between 2 weeks and 3 months...
s or plasmapheresis
Plasmapheresis
Plasmapheresis is the removal, treatment, and return of blood plasma from blood circulation. It is thus an extracorporeal therapy...
, 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 the most common cause of acute non-trauma-related paralysis in the world. The syndrome is named after the French physicians Georges Guillain
Georges Guillain
Georges Charles Guillain was a French neurologist born in Rouen.He studied medicine in Rouen and Paris, where he learned clinical education at several hospitals. He developed an interest in neurology, and his first important scientific work involved lesions of the plexus brachialis...
and Jean Alexandre Barré
Jean Alexandre Barré
Jean Alexandre Barré , French neurologist, worked in 1916 on the identification of the Guillain-Barré syndrome....
, who described it in 1916.
Classification
Six different subtypes of Guillain–Barré syndrome exist:- Acute inflammatory demyelinating polyneuropathyPolyneuropathyPolyneuropathy is a neurological disorder that occurs when many peripheral nerves throughout the body malfunction simultaneously. It may be acute and appear without warning, or chronic and develop gradually over a longer period of time. Many polyneuropathies have both motor and sensory...
(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 cellSchwann cellSchwann cells or neurolemmocytes are the principal glia of the peripheral nervous system . Glial cells function to support neurons and in the PNS, also include satellite cells, olfactory ensheathing cells, enteric glia and glia that reside at sensory nerve endings, such as the Pacinian corpuscle...
membranes. - Miller Fisher syndrome (MFS) is a rare variant of GBS and manifests as a descending paralysisParalysisParalysis is loss of muscle function for one or more muscles. Paralysis can be accompanied by a loss of feeling in the affected area if there is sensory damage as well as motor. A study conducted by the Christopher & Dana Reeve Foundation, suggests that about 1 in 50 people have been diagnosed...
, proceeding in the reverse order of the more common form of GBS. It usually affects the eyeHuman eyeThe human eye is an organ which reacts to light for several purposes. As a conscious sense organ, the eye allows vision. Rod and cone cells in the retina allow conscious light perception and vision including color differentiation and the perception of depth...
muscles first and presents with the triad of ophthalmoplegia, ataxiaAtaxiaAtaxia is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum...
, and areflexia. Anti-GQ1b antibodies are present in 90% of cases. - Acute motor axonal neuropathyAcute motor axonal neuropathyAcute motor axonal neuropathy is a variant of Guillain-Barré syndrome. It is characterized by acute paralysis and loss of reflexes without sensory loss...
(AMAN), also known as Chinese paralytic syndrome, attacks motor nodes of RanvierNodes of RanvierMyelin sheath gaps or nodes of Ranvier are the gaps formed between the myelin sheaths generated by different cells. A myelin sheath is a many-layered coating, largely composed of a fatty substance called myelin, that wraps around the axon of a neuron and very efficiently insulates it...
and is prevalent in ChinaChinaChinese civilization may refer to:* China for more general discussion of the country.* Chinese culture* Greater China, the transnational community of ethnic Chinese.* History of China* Sinosphere, the area historically affected by Chinese culture...
and MexicoMexicoThe United Mexican States , commonly known as Mexico , is a federal constitutional republic in North America. It is bordered on the north by the United States; on the south and west by the Pacific Ocean; on the southeast by Guatemala, Belize, and the Caribbean Sea; and on the east by the Gulf of...
. It is probably due to an auto-immune response directed against the axoplasmAxoplasmAxoplasm is the cytoplasm within the axon of a neuron. Neural processes contain about 99.6% of the cell’s cytoplasm, and 99.7% of that is in the axons....
of peripheral nervesPeripheral nervous systemThe peripheral nervous system consists of the nerves and ganglia outside of the brain and spinal cord. The main function of the PNS is to connect the central nervous system to the limbs and organs. Unlike the CNS, the PNS is not protected by the bone of spine and skull, or by the blood–brain...
. The disease may be seasonal and recovery can be rapid. Anti-GD1a antibodies 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 axoplasmAxoplasmAxoplasm is the cytoplasm within the axon of a neuron. Neural processes contain about 99.6% of the cell’s cytoplasm, and 99.7% of that is in the axons....
of peripheral nerves. Recovery is slow and often incomplete. - Acute panautonomic neuropathy is the most rare variant of GBS, sometimes accompanied by encephalopathyEncephalopathyEncephalopathy means disorder or disease of the brain. In modern usage, encephalopathy does not refer to a single disease, but rather to a syndrome of global brain dysfunction; this syndrome can be caused by many different illnesses.-Terminology:...
. It is associated with a high mortality rate, owing to cardiovascular involvement, and associated dysrhythmias. Impaired sweating, lack of tear formation, photophobiaPhotophobiaPhotophobia is a symptom of abnormal intolerance to visual perception of light. As a medical symptom photophobia is not a morbid fear or phobia, but an experience of discomfort or pain to the eyes due to light exposure or by presence of actual physical photosensitivity of the eyes, though the term...
, dryness of nasal and oral mucosa, itching and peeling of skin, nauseaNauseaNausea , is a sensation of unease and discomfort in the upper stomach with an involuntary urge to vomit. It often, but not always, precedes vomiting...
, dysphagiaDysphagiaDysphagia is the medical term for the symptom of difficulty in swallowing. Although classified under "symptoms and signs" in ICD-10, the term is sometimes used as a condition in its own right. Sufferers are sometimes unaware of their 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, constipation, 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 signPlantar reflexThe plantar reflex is a reflex elicited when the sole of the foot is stimulated with a blunt instrument. The reflex can take one of two forms. In normal adults the plantar reflex causes a downward response of the hallux...
. The course of the disease can be monophasic or remitting-relapsing. Large, irregular hyperintense lesions located mainly in the brainstem, especially in the ponsPonsThe pons is a structure located on the brain stem, named after the Latin word for "bridge" or the 16th-century Italian anatomist and surgeon Costanzo Varolio . It is superior to the medulla oblongata, inferior to the midbrain, and ventral to the cerebellum. In humans and other bipeds this means it...
, midbrain and medullaMedullaMedulla refers to the middle of something and derives from the Latin word for marrow. Its anatomical uses include:* Medulla oblongata, a part of the brain stem* Renal medulla, a part of the kidney* Adrenal medulla, a part of the adrenal gland...
are described in the literature. BBE despite severe initial presentation usually has a good prognosis. Magnetic resonance imagingMagnetic resonance imagingMagnetic resonance imaging , nuclear magnetic resonance imaging , or magnetic resonance tomography is a medical imaging technique used in radiology to visualize detailed internal structures...
(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.
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 nervesCranial nerves
Cranial nerves are nerves that emerge directly from the brain, in contrast to spinal nerves, which emerge from segments of the spinal cord. In humans, there are traditionally twelve pairs of cranial nerves...
may be affected, leading to bulbar weakness, oropharyngeal dysphagia
Dysphagia
Dysphagia is the medical term for the symptom of difficulty in swallowing. Although classified under "symptoms and signs" in ICD-10, the term is sometimes used as a condition in its own right. Sufferers are sometimes unaware of their dysphagia....
(drooling, or difficulty swallowing and/or maintaining an open airway) and respiratory difficulties. Most patients require hospitalization and about 30% require ventilatory assistance
Mechanical ventilation
In medicine, mechanical ventilation is a method to mechanically assist or replace spontaneous breathing. This may involve a machine called a ventilator or the breathing may be assisted by a physician, respiratory therapist or other suitable person compressing a bag or set of bellows...
for treatment of Type II respiratory failure
Respiratory failure
The term respiratory failure, in medicine, is used to describe inadequate gas exchange by the respiratory system, with the result that arterial oxygen and/or carbon dioxide levels cannot be maintained within their normal ranges. A drop in blood oxygenation is known as hypoxemia; a rise in arterial...
. 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
Proprioception
Proprioception , from Latin proprius, meaning "one's own" and perception, is the sense of the relative position of neighbouring parts of the body and strength of effort being employed in movement...
(position sense) and areflexia
Hyporeflexia
Hyporeflexia is the condition of below normal or absent reflexes. It can be tested for by using a reflex hammer. It is the opposite of hyperreflexia....
(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 analgesic
Analgesic
An analgesic is any member of the group of drugs used to relieve pain . The word analgesic derives from Greek an- and algos ....
s. Bladder
Urinary bladder
The urinary bladder is the organ that collects urine excreted by the kidneys before disposal by urination. A hollow muscular, and distensible organ, the bladder sits on the pelvic floor...
dysfunction may occur in severe cases but should be transient. If severe, spinal cord
Spinal cord
The spinal cord is a long, thin, tubular bundle of nervous tissue and support cells that extends from the brain . The brain and spinal cord together make up the central nervous system...
disorder should be suspected.
Fever
Fever
Fever is a common medical sign characterized by an elevation of temperature above the normal range of due to an increase in the body temperature regulatory set-point. This increase in set-point triggers increased muscle tone and shivering.As a person's temperature increases, there is, in...
should not be present, and if it is, another cause should be suspected.
In severe cases of GBS, loss of autonomic
Autonomic nervous system
The autonomic nervous system is the part of the peripheral nervous system that acts as a control system functioning largely below the level of consciousness, and controls visceral functions. The ANS affects heart rate, digestion, respiration rate, salivation, perspiration, diameter of the pupils,...
function is common, manifesting as wide fluctuations in blood pressure
Blood pressure
Blood pressure is the pressure exerted by circulating blood upon the walls of blood vessels, and is one of the principal vital signs. When used without further specification, "blood pressure" usually refers to the arterial pressure of the systemic circulation. During each heartbeat, BP varies...
, orthostatic hypotension
Orthostatic hypotension
Orthostatic hypotension, also known as postural hypotension, orthostasis, and colloquially as head rush or dizzy spell, is a form of hypotension in which a person's blood pressure suddenly falls when the person stands up or stretches. The decrease is typically greater than 20/10 mm Hg, and may be...
, and cardiac arrhythmias.
Acute paralysis in Guillain–Barré syndrome may be related to sodium channel blocking factor in the cerebrospinal fluid
Cerebrospinal fluid
Cerebrospinal fluid , Liquor cerebrospinalis, is a clear, colorless, bodily fluid, that occupies the subarachnoid space and the ventricular system around and inside the brain and spinal cord...
(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-Barré 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
Progressive inflammatory neuropathy
Progressive inflammatory neuropathy is a disease that was identified in a report, released on January 31, 2008, by the Centers for Disease Control and Prevention. The first known outbreak of this neuropathy was in southeastern Minnesota in the United States. The disease was reported among pig...
.
Cause
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 jejuniCampylobacter jejuni
Campylobacter jejuni is a species of curved, helical-shaped, non-spore forming, Gram-negative, microaerophilic bacteria commonly found in animal feces. It is one of the most common causes of human gastroenteritis in the world. Food poisoning caused by Campylobacter species can be severely...
. 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.
The end result of such autoimmune attack on the peripheral nerves is damage to the myelin
Myelin
Myelin is a dielectric material that forms a layer, the myelin sheath, usually around only the axon of a neuron. It is essential for the proper functioning of the nervous system. Myelin is an outgrowth of a type of glial cell. The production of the myelin sheath is called myelination...
, 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
Autonomic nervous system
The autonomic nervous system is the part of the peripheral nervous system that acts as a control system functioning largely below the level of consciousness, and controls visceral functions. The ANS affects heart rate, digestion, respiration rate, salivation, perspiration, diameter of the pupils,...
disturbances.
However, in mild cases, nerve axon
Axon
An axon is a long, slender projection of a nerve cell, or neuron, that conducts electrical impulses away from the neuron's cell body or soma....
(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
Myelin
Myelin is a dielectric material that forms a layer, the myelin sheath, usually around only the axon of a neuron. It is essential for the proper functioning of the nervous system. Myelin is an outgrowth of a type of glial cell. The production of the myelin sheath is called myelination...
loss, whereas, in the remaining 20%, the pathologic hallmark of the disorder is indeed axon
Axon
An axon is a long, slender projection of a nerve cell, or neuron, that conducts electrical impulses away from the neuron's cell body or soma....
loss.
Guillain-Barré, unlike disorders such as multiple sclerosis
Multiple sclerosis
Multiple sclerosis is an inflammatory disease in which the fatty myelin sheaths around the axons of the brain and spinal cord are damaged, leading to demyelination and scarring as well as a broad spectrum of signs and symptoms...
(MS) and Lou Gehrig's disease
Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis , also referred to as Lou Gehrig's disease, is a form of motor neuron disease caused by the degeneration of upper and lower neurons, located in the ventral horn of the spinal cord and the cortical neurons that provide their efferent input...
(ALS), is a peripheral nerve disorder and does not generally cause nerve damage to the brain or spinal cord.
Influenza
Becoming infected with influenza increases both the risk of developing GBS and the risk of death (up to 1 in 10,000 who get influenza will die from it). While influenza vaccines have sometimes been suspected to raise the incidence of GBS, the evidence is equivocal, as shown below. Moreover, the highest level of suspected risks from the influenza vaccine is less than one-tenth the risk from the influenza disease itself, as discussed in more detail in the next section. The risk of GBS following influenza vaccination is about one case per 1 million vaccines given.Influenza vaccine
GBS may be a rare side-effect of influenza vaccines; a study of the Vaccine Adverse Event Reporting SystemVaccine Adverse Event Reporting System
The Vaccine Adverse Event Reporting System is a United States program for vaccine safety, co-managed by the Centers for Disease Control and Prevention and the Food and Drug Administration . VAERS is a post-marketing safety surveillance program, collecting information about adverse events that...
(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). There were reports of GBS affecting 10 per million who had received swine flu immunizations in the 1976 U.S. outbreak of swine flu
1976 swine flu outbreak
The 1976 swine flu outbreak, also known as the swine flu fiasco, or the swine flu debacle, was a strain of H1N1 influenza virus that appeared in 1976. Infections were only detected from January 19 to February 9, and were not found outside Fort Dix. The outbreak is most remembered for the mass...
—25 of which resulted in death from severe pulmonary complications, leading the United States Center of Disease Control to end that immunization campaign within the United States. (By comparison, the average flu season kills around 30,000 people in the United States). 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
Incidence (epidemiology)
Incidence is a measure of the risk of developing some new condition within a specified period of time. Although sometimes loosely expressed simply as the number of new cases during some time period, it is better expressed as a proportion or a rate with a denominator.Incidence proportion is the...
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. Furthermore, the GBS may not have been directly due to the vaccine but to a bacterial contamination of the 1976 vaccine.
Since 1976, no other influenza vaccines have been linked to GBS, though as a precautionary principle
Precautionary principle
The precautionary principle or precautionary approach states that if an action or policy has a suspected risk of causing harm to the public or to the environment, in the absence of scientific consensus that the action or policy is harmful, the burden of proof that it is not harmful falls on those...
, caution is advised for certain individuals, particularly those with a history of GBS.
From October 6 to November 24, 2009, the U.S. CDC, through the VAERS reporting system, received ten reports of Guillain-Barré 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
Brighton Collaboration
The ' is an independent global vaccine safety research network for health care professionals. It is a non-profit partnership providing high-quality vaccine safety information.- Background :...
case criteria for Guillain–Barré syndrome, while four do not meet the criteria and four remain under review. A preliminary report by the CDC
Centers for Disease Control and Prevention
The Centers for Disease Control and Prevention are a United States federal agency under the Department of Health and Human Services headquartered in Druid Hills, unincorporated DeKalb County, Georgia, in Greater Atlanta...
'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.
Although one review gives an incidence of about one case per million vaccinations, 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-Barré syndrome (0.1 per million) 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."
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 fluidCerebrospinal fluidCerebrospinal fluid , Liquor cerebrospinalis, is a clear, colorless, bodily fluid, that occupies the subarachnoid space and the ventricular system around and inside the brain and spinal cord...
- 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
- ElectromyographyElectromyographyElectromyography is a technique for evaluating and recording the electrical activity produced by skeletal muscles. EMG is performed using an instrument called an electromyograph, to produce a record called an electromyogram. An electromyograph detects the electrical potential generated by muscle...
(EMG) and nerve conduction studyNerve conduction studyA nerve conduction study is a test commonly used to evaluate the function, especially the ability of electrical conduction, of the motor and sensory nerves of the human body.Nerve conduction velocity is a common measurement made during this test...
(NCS) may show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. In primary axonal damage, the findings include reduced amplitude of the action potentials without conduction slowing.
Diagnostic criteria
Required:- Progressive, relatively symmetrical weakness of two or more limbs due to neuropathy
- Areflexia
- Disorder course < 4 weeks
- Exclusion of other causes (see below)
Supportive:
- relatively symmetric weakness accompanied by numbness and/or tingling
- mild sensory involvement
- facial nerve or other cranial nerve involvement
- absence of fever
- typical CSF findings obtained from lumbar puncture
- electrophysiologic evidence of demyelination from electromyogram
Differential diagnosis
Differential diagnosis
A differential diagnosis is a systematic diagnostic method used to identify the presence of an entity where multiple alternatives are possible , and may also refer to any of the included candidate alternatives A differential diagnosis (sometimes abbreviated DDx, ddx, DD, D/Dx, or ΔΔ) is a...
:
- acute myelopathies with chronic back pain and sphincter dysfunction
- botulismBotulismBotulism also known as botulinus intoxication is a rare but serious paralytic illness caused by botulinum toxin which is metabolic waste produced under anaerobic conditions by the bacterium Clostridium botulinum, and affecting a wide range of mammals, birds and fish...
with early loss of pupillary reactivity and descending paralysis - diphtheriaDiphtheriaDiphtheria is an upper respiratory tract illness caused by Corynebacterium diphtheriae, a facultative anaerobic, Gram-positive bacterium. It is characterized by sore throat, low fever, and an adherent membrane on the tonsils, pharynx, and/or nasal cavity...
with early oropharyngeal dysfunction - Lyme diseaseLyme diseaseLyme disease, or Lyme borreliosis, is an emerging infectious disease caused by at least three species of bacteria belonging to the genus Borrelia. Borrelia burgdorferi sensu stricto is the main cause of Lyme disease in the United States, whereas Borrelia afzelii and Borrelia garinii cause most...
polyradiculitis and other tick-borne paralyses - porphyriaPorphyriaPorphyrias are a group of inherited or acquired disorders of certain enzymes in the heme bio-synthetic pathway . They are broadly classified as acute porphyrias and cutaneous porphyrias, based on the site of the overproduction and accumulation of the porphyrins...
with abdominal pain, seizures, psychosis - vasculitis neuropathy
- poliomyelitisPoliomyelitisPoliomyelitis, often called polio or infantile paralysis, is an acute viral infectious disease spread from person to person, primarily via the fecal-oral route...
with fever and meningeal signs - CMVCytomegalovirusCytomegalovirus is a viral genus of the viral group known as Herpesviridae or herpesviruses. It is typically abbreviated as CMV: The species that infects humans is commonly known as human CMV or human herpesvirus-5 , and is the most studied of all cytomegaloviruses...
polyradiculitis in immunocompromised patients - critical illness neuropathy
- myasthenia gravisMyasthenia gravisMyasthenia gravis is an autoimmune neuromuscular disease leading to fluctuating muscle weakness and fatiguability...
- poisonings with organophosphateOrganophosphateAn organophosphate is the general name for esters of phosphoric acid. Phosphates are probably the most pervasive organophosphorus compounds. Many of the most important biochemicals are organophosphates, including DNA and RNA as well as many cofactors that are essential for life...
, poison hemlock, thalliumThalliumThallium is a chemical element with the symbol Tl and atomic number 81. This soft gray poor metal resembles tin but discolors when exposed to air. The two chemists William Crookes and Claude-Auguste Lamy discovered thallium independently in 1861 by the newly developed method of flame spectroscopy...
, or arsenicArsenicArsenic is a chemical element with the symbol As, atomic number 33 and relative atomic mass 74.92. Arsenic occurs in many minerals, usually in conjunction with sulfur and metals, and also as a pure elemental crystal. It was first documented by Albertus Magnus in 1250.Arsenic is a metalloid... - intoxication with Karwinskia humboldtianaKarwinskia humboldtianaKarwinskia humboldtiana, commonly known as Coyotillio or Humboldt Coyotillo, is a species of flowering shrub or small tree in the buckthorn family, Rhamnaceae. It is native southern and western Texas in the United States as well as much of Mexico...
leaves or seeds - paresis caused by West Nile virusWest Nile virusWest Nile virus is a virus of the family Flaviviridae. Part of the Japanese encephalitis antigenic complex of viruses, it is found in both tropical and temperate regions. It mainly infects birds, but is known to infect humans, horses, dogs, cats, bats, chipmunks, skunks, squirrels, domestic...
- spinal astrocytoma
- motor neurone diseaseMotor neurone diseaseThe motor neurone diseases are a group of neurological disorders that selectively affect motor neurones, the cells that control voluntary muscle activity including speaking, walking, breathing, swallowing and general movement of the body. They are generally progressive in nature, and can cause...
- West Nile virusWest Nile virusWest Nile virus is a virus of the family Flaviviridae. Part of the Japanese encephalitis antigenic complex of viruses, it is found in both tropical and temperate regions. It mainly infects birds, but is known to infect humans, horses, dogs, cats, bats, chipmunks, skunks, squirrels, domestic...
can cause severe, potentially fatal neurological illnesses, which include encephalitis, meningitis, Guillain-Barré syndrome, and anterior myelitis.
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 intubationIntubation
Tracheal intubation, usually simply referred to as intubation, is the placement of a flexible plastic or rubber tube into the trachea to maintain an open airway or to serve as a conduit through which to administer certain drugs...
should be considered in any patient with a vital capacity
Vital capacity
Vital capacity is the maximum amount of air a person can expel from the lungs after a maximum inspiration. It is equal to the inspiratory reserve volume plus the tidal volume plus the expiratory reserve volume....
(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
Plasmapheresis
Plasmapheresis is the removal, treatment, and return of blood plasma from blood circulation. It is thus an extracorporeal therapy...
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 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
Activities of daily living
Activities of Daily Living is a term used in healthcare to refer to daily self-care activities within an individual's place of residence, in outdoor environments, or both...
) 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. There is also some evidence supporting physiotherapy
Physical therapy
Physical therapy , often abbreviated PT, is a health care profession. Physical therapy is concerned with identifying and maximizing quality of life and movement potential within the spheres of promotion, prevention, diagnosis, treatment/intervention,and rehabilitation...
in helping patients with Guillain–Barré syndrome regain strength, endurance, and gait quality, as well as helping them prevent contractures, bedsores, and cardiopulmonary difficulties. A speech and language therapist would be essential in the patient regaining speaking and swallowing ability if they were intubated and received a tracheostomy. The speech and language therapist would also offer advice to the medical team regarding the swallowing abilities of the patient and would help the patient regain their communication ability pre-dysarthria. There would also be a doctor, nurse and other team members involved, depending on the needs of the patient. This team contribute their knowledge to guide the patient towards his or her goals, and it is important that all goals set by the separate team members are relevant for the patient's own priorities. After rehabilitation the patient should be able to function in his or her own home and attend necessary training as needed.
Prognosis
Most of the time recovery starts after the fourth week from the onset of the disorder. Approximately 80% of patients have a complete recovery within a few months to a year, although minor findings may persist, such as areflexia. About 5–10% recover with severe disability, with most of such cases involving severe proximal motor and sensory axonal damage with inability of axonal regeneration. However, this is a grave disorder and despite all improvements in treatment and supportive care, the death rate among patients with this disorder is still about 2–3% even in the best intensive care units. Worldwide, the death rate runs slightly higher (4%), mostly from a lack of availability of life support equipment during the lengthy plateau lasting four to six weeks, and in some cases up to one year, when a ventilator is needed in the worst cases. About 5–10% of patients have one or more late relapses, in which case they are then classified as having chronic inflammatory demyelinating polyneuropathyChronic inflammatory demyelinating polyneuropathy
Chronic inflammatory demyelinating polyneuropathy is an acquired immune-mediated inflammatory disorder of the peripheral nervous system. The disorder is sometimes called chronic relapsing polyneuropathy. CIDP is closely related to Guillain-Barré syndrome and it is considered the chronic...
(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.
Epidemiology
The incidence of GBS during pregnancy is 1.7 cases per 100,000 of the population. 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 jejuniCampylobacter jejuni
Campylobacter jejuni is a species of curved, helical-shaped, non-spore forming, Gram-negative, microaerophilic bacteria commonly found in animal feces. It is one of the most common causes of human gastroenteritis in the world. Food poisoning caused by Campylobacter species can be severely...
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.
History
The disorder was first described by the FrenchFrance
The French Republic , The French Republic , The French Republic , (commonly known as France , is a unitary semi-presidential republic in Western Europe with several overseas territories and islands located on other continents and in the Indian, Pacific, and Atlantic oceans. Metropolitan France...
physician Jean Landry
Jean Landry (physician)
Jean Baptiste Octave Landry de Thézillat was a French physician and medical researcher. He is credited with discovering the paralytic disease Guillain-Barré syndrome He was born in Limoges, Haute-Vienne...
in 1859. In 1916, Georges Guillain
Georges Guillain
Georges Charles Guillain was a French neurologist born in Rouen.He studied medicine in Rouen and Paris, where he learned clinical education at several hospitals. He developed an interest in neurology, and his first important scientific work involved lesions of the plexus brachialis...
, Jean Alexandre Barré
Jean Alexandre Barré
Jean Alexandre Barré , French neurologist, worked in 1916 on the identification of the Guillain-Barré syndrome....
, and André Strohl
André Strohl
André Strohl , was a French physiologist who was a native of Poitiers. He is remembered for his role in the diagnosis of Guillain-Barré syndrome , a form of areflexic paralysiswhich exhibits normal cell count, but with an abnormal increase in spinal fluid protein...
diagnosed two soldiers with the illness and discovered the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count.
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
Miller Fisher
Charles Miller Fisher, usually known as Miller Fisher is a neurologist. Trained in Canada, he spent 1949 at Massachusetts General Hospital, followed by several years in Montreal. In 1954 he returned to Massachusetts General on the stroke service, beginning a long career in stroke neurology...
described the variant that bears his name in 1956.
Notable cases
- Markus BabbelMarkus BabbelMarkus Babbel is a former international German football player, who is currently manager of German Bundesliga club Hertha BSC. He played as a defender for clubs in Germany and England.-Career:...
, 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 capsCap (sport)In sports, a cap is a metaphorical term for a player's appearance on a select team, such as a national team. The term dates from the practice in the United Kingdom of awarding a cap to every player in an international match of association football...
for GermanyGermanyGermany , officially the Federal Republic of Germany , is a federal parliamentary republic in Europe. The country consists of 16 states while the capital and largest city is Berlin. Germany covers an area of 357,021 km2 and has a largely temperate seasonal climate...
. - Tony BennTony BennAnthony Neil Wedgwood "Tony" Benn, PC is a British Labour Party politician and a former MP and Cabinet Minister.His successful campaign to renounce his hereditary peerage was instrumental in the creation of the Peerage Act 1963...
, British politician. - Rachel ChagallRachel ChagallRachel Chagall is an American actress, who specialized in comedic roles. She best known for roles as Gaby on the film Gaby: A True Story , , for which she was nominated for Golden Globe Award for Best Actress – Motion Picture Drama , and as Val Toriello on The Nanny .- Life and career :Born as...
, actress, contracted GBS in 1982. In 1987 she portrayed Gabriela BrimmerGabriela BrimmerGabriela "Gaby" Brimmer , a writer and activist for persons with disabilities, was born in Mexico as a daughter of Austrian Jewish immigrants. Gaby was born with cerebral palsy and since childhood learned to act in a world that has difficulty accepting diversity...
, a notable disabilities activist. - Samuel GoldsteinSamuel GoldsteinSamuel Hyman Goldstein was an American athlete and Paralympian.He took two silver medals and one bronze in swimming at the 1964 Summer Paralympics, and one silver medal in table tennis at the 1964 Summer Paralympics....
, American athlete and Paralympian - Andy GriffithAndy GriffithAndy Samuel Griffith is an American actor, director, producer, Grammy Award-winning Southern-gospel singer, and writer. He gained prominence in the starring role in director Elia Kazan's epic film A Face in the Crowd before he became better known for his television roles, playing the lead...
, American actor on Andy Griffith Show, and MatlockMatlock (TV series)Matlock is an American television legal drama, starring Andy Griffith in the title role of attorney Ben Matlock. The show originally aired from September 23, 1986 to May 8, 1992 on NBC, where it replaced The A-Team, then from November 5, 1992 until May 7, 1995 on ABC.The show's format was similar...
. He developed Guillain–Barré in 1983. - Joseph HellerJoseph HellerJoseph Heller was a US satirical novelist, short story writer, and playwright. His best known work is Catch-22, a novel about US servicemen during World War II...
, 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 VogelSpeed VogelIrving "Speed" Vogel was an American sculptor, painter, and co-author, along with Joseph Heller, of the best-selling memoir, No Laughing Matter....
. - Luci Baines JohnsonLuci Baines JohnsonLuci Baines Johnson Turpin, formerly Nugent, is the younger daughter of U.S. President Lyndon Johnson and his wife, the former Claudia Alta Taylor . Her name was originally spelled "Lucy"; she informally changed the spelling in her teens...
, daughter of President Lyndon Johnson and Lady Bird Johnson. Diagnosed and under treatment for Guillain–Barré in April 2010. - Hugh McElhennyHugh McElhennyHugh Edward McElhenny is a former American football running back in the National Football League who played from 1952–1964, for the San Francisco 49ers, Minnesota Vikings, New York Giants, and Detroit Lions. He was noted for his explosive, elusive running style and was frequently called "The...
, former Hall-of-Fame, professional American footballAmerican footballAmerican football is a sport played between two teams of eleven with the objective of scoring points by advancing the ball into the opposing team's end zone. Known in the United States simply as football, it may also be referred to informally as gridiron football. The ball can be advanced by...
player with the San Francisco 49ersSan Francisco 49ersThe San Francisco 49ers are a professional American football team based in San Francisco, California, playing in the West Division of the National Football Conference in the National Football League . The team was founded in 1946 as a charter member of the All-America Football Conference and...
. - Lucky OceansLucky OceansLucky Oceans is a pedal steel guitarist and a former member of Country/Western Swing band Asleep at the Wheel. He is now a broadcaster in Perth, Western Australia with the Australian Broadcasting Corporation...
, Grammy AwardGrammy AwardA Grammy Award — or Grammy — is an accolade by the National Academy of Recording Arts and Sciences of the United States to recognize outstanding achievement in the music industry...
winning musician with Asleep at the WheelAsleep at the WheelAsleep at the Wheel is a American country music group that was formed in Paw Paw, West Virginia, but based in Austin, Texas. Altogether, they have won nine Grammy Awards since their 1970 inception. In their career, they have released more than twenty studio albums, and have charted more than twenty...
was diagnosed with GBS in 2008. - Len PasquarelliLen PasquarelliLen Pasquarelli is an American sports writer and analyst with The Sports Xchange and a 25-year vet of covering the NFL.The Sports Xchange is a network of professional, accredited reporters and analysts who cover each team or sport full time....
, sports writer and analyst for ESPNESPNEntertainment and Sports Programming Network, commonly known as ESPN, is an American global cable television network focusing on sports-related programming including live and pre-taped event telecasts, sports talk shows, and other original programming....
and resident of the Pro Football Writers of America, diagnosed in 2008. - Serge PayerSerge PayerSerge Payer is a Canadian professional ice hockey player. Payer currently plays for Vålerenga Ishockey of the GET-ligaen. Payer has played 119 career NHL games with the Florida Panthers and Ottawa Senators, scoring 7 goals and 6 assists for 13 points.-Playing career:Payer was signed as an...
, 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. - William “The Refrigerator” PerryWilliam Perry (American football)William Perry is a former professional American football player. He is best known for his years as a defensive lineman for the Chicago Bears. In reference to his large size, he was popularly known as "The Refrigerator" or, abbreviated, "The Fridge".-Life and athletic career:Perry was born in...
, former professional American footballAmerican footballAmerican football is a sport played between two teams of eleven with the objective of scoring points by advancing the ball into the opposing team's end zone. Known in the United States simply as football, it may also be referred to informally as gridiron football. The ball can be advanced by...
player with the Chicago BearsChicago BearsThe Chicago Bears are a professional American football team based in Chicago, Illinois. They are members of the North Division of the National Football Conference in the National Football League...
was diagnosed with GBS in 2008. - Franklin D. RooseveltFranklin D. RooseveltFranklin Delano Roosevelt , also known by his initials, FDR, was the 32nd President of the United States and a central figure in world events during the mid-20th century, leading the United States during a time of worldwide economic crisis and world war...
, U.S. president. In 2003, a peer-reviewed study found that Roosevelt's paralysisFranklin D. Roosevelt's paralytic illnessFranklin D. Roosevelt's paralytic illness began in 1921 at age 39, when Roosevelt got a fever after exercising heavily at a vacation in Canada. While his bout with illness was well known during his terms as President of the United States, the extent of his paralysis was kept from public view. After...
—long attributed to poliomyelitisPoliomyelitisPoliomyelitis, often called polio or infantile paralysis, is an acute viral infectious disease spread from person to person, primarily via the fecal-oral route...
—was more likely caused by Guillain–Barré syndrome. - Norton SimonNorton SimonNorton Winfred Simon , in the United States was a millionaire industrialist and philanthropist based in California. A significant art collector, he is the namesake of the Norton Simon Museum in Pasadena, California.-Early life:...
, American industrialist and philanthropist. - Hans Vonk, Dutch conductor.
- Danny WuerffelDanny WuerffelDaniel Carl "Danny" Wuerffel is a former American college and professional football player who won the 1996 Heisman Trophy and the 1996 national football championship while playing college football for the University of Florida. After graduating from Florida, he played for four National Football...
, 1996 Heisman TrophyHeisman TrophyThe Heisman Memorial Trophy Award , is awarded annually to the player deemed the most outstanding player in collegiate football. It was created in 1935 as the Downtown Athletic Club trophy and renamed in 1936 following the death of the Club's athletic director, John Heisman The Heisman Memorial...
winner from the University of FloridaFlorida Gators footballThe Florida Gators football team represents the University of Florida in the sport of American football. The Florida Gators compete in the Football Bowl Subdivision of the National Collegiate Athletics Association and the Eastern Division of the Southeastern Conference...
.