Guillain-Barré Syndrome

What is Guillain-Barré Syndrome?

Guillain-Barré (ghee-yan bah-ray) syndrome is a disorder in which the body's immune system attacks part of the peripheral nervous system. The first symptoms of this disorder include varying degrees of weakness or tingling sensations in the legs.

In many instances, the weakness and abnormal sensations spread to the arms and upper body. These symptoms can increase in intensity until the muscles cannot be used at all and the patient is almost totally paralyzed.

In these cases, the disorder is life-threatening and is considered a medical emergency. The patient is often put on a respirator to assist with breathing. Most patients, however, recover from even the most severe cases of Guillain-Barré syndrome, although some continue to have some degree of weakness. Guillain-Barré syndrome is rare. Usually Guillain-Barré occurs a few days or weeks after the patient has had symptoms of a respiratory or gastrointestinal viral infection.

Occasionally, surgery or vaccinations will trigger the syndrome. The disorder can develop over the course of hours or days, or it may take up to 3 to 4 weeks. No one yet knows why Guillain-Barré strikes some people and not others or what sets the disease in motion. What scientists do know is that the body's immune system begins to attack the body itself, causing what is known as an autoimmune disease.

Guillain-Barré is called a syndrome rather than a disease because it is not clear that a specific disease-causing agent is involved. Reflexes such as knee jerks are usually lost.

Because the signals traveling along the nerve are slower, a nerve conduction velocity (NCV) test can give a doctor clues to aid the diagnosis. The cerebrospinal fluid that bathes the spinal cord and brain contains more protein than usual, so a physician may decide to perform a spinal tap.


In 1859, Jean B.O. Landry, a French physician, described a disorder in which the nerves of the legs, arms, neck, and breathing muscles were paralyzed. Georges Guillain, Jean Alexander Barré, and Andre Strohl discovered the characteristic abnormality of increased protein but normal cell count in the cerebrospinal fluid in 1916.

Several names have been given for the syndrome including acute idiopathic polyneuritis, acute idiopathic polyradiculoneuritis, Landry's ascending paralysis, and Guillain Barré syndrome.


Guillain-Barré syndrome (GBS) can affect any individual regardless of age or sex, although incidence increases with age (mean 50-74 years) and men are more commonly affected than women. The frequency of GBS is about 1 to 2 cases in 100,000 (0.001 ­ 0.0002%) for adults and approximately 0.38/100,000 for children under 15 years of age, with only 1.4/10 million of those cases causing permanent neurological effects. About 50% of the cases follow a viral illness.


The causes of Guillain Barré syndrome are unknown. Many researchers theorize that the autoimmune reaction in which macrophages and T-cells attack myelin in the peripheral and cranial nerves is associated with a bacteria or virus, as many cases occur a few days to a few weeks after an infection including the common cold, sore throat, and stomach and intestinal viruses with vomiting and diarrhea.

The virus might induce the demyelination via a possible mimicry between the effector virus and a human ganglioside. Salloway and colleagues (1996) discovered that the lipopolysaccharide structure of some strains of Campylobacter jejuni, specifically, the terminal structures of the core oligosaccharide, resemble the human gangliosides GM1 and GD1a.

A possible mimicry also exists between the influenza A NS2 protein and a sequence region of the human P2 (myelin) protein thought to be neuritogenic in animals and mitogenic for lymphocytes from patients with GBS.

This finding could provide a possible link between the large number of cases of GBS associated with the 1976 USA swine flu vaccination program (Weise and Carnegie, 1988).

Other viruses, such as the cytomeglavirus have also been implicated, although the exact pathogenesis has not been discovered.


Studies have shown that GBS patients have raised serum and cerebrospinal fluid concentrations of interleukin (IL)-2, an agent that aids in regulation of sodium currents and gating mechanisms (Bendahhou et al., 1995), IL-2 receptor, IL-6, and TNF-alpha, a major proinflammatory cytokine implicated in early breakdown of blood-nerve barrier, upregulation of endothelial adhesion molecules, prerequisite for leukocyte transport to nerve tissue, macrophage activation, and myelin damage.

Accompanying down-regulation of transforming growth factor beta 1 (TGF-beta1), an immunosuppressive molecule that antagonizes IL-1, IL-2, TNF-alpha, and IFN-gamma, occurs with concentrations of TGF-beta1 remaining low during the illness and progressively increasing to control concentrations during early recovery. (Creange et al, 1998).

Thus, TNF-alpha begins to breakdown the blood-nerve barrier, allowing antibodies produced against the invading virus to attack the nerves. As the virus mimics an anti-ganglioside, the blood contains neuritogenic material.

Areas in which the virus has made weak via a breakdown of the blood-nerve barrier are most susceptible to axonal damage.

Clinical deficit is caused by failure of conduction in nerve fibers, usually due to demyelination of the nerves' axons. Sensory deficit is sometimes, but not always present.

Demyelination follows a centripetal pattern, occurring first in the most distal part of the nerve and progressing to the spinal root, preferentially targeting small myelinated fibers.

The central/peripheral nervous system transition regions and most proximal zones of the spinal nerve roots generally are not damaged. Clinically motor weakness dominates, though sensory and motor nerves are generally affected equally.

Central conduction is the first to improve, and the first nerve segments to demyelinate are the first to remyelinate.

Clinical recovery follows the remyelination of the spinal root (Wexler, 1983). Occasionally, degeneration of the axon will occur alone or in conjunction with demyelination.

Axon regeneration is slow, if present. Thus, conduction block is the main cause of acute paralysis and sensory loss, while axonal degeneration is the cause of lasting disability.

B) Transection of the spinal cord with dorsal and ventral horns and roots and dorsal root ganglion. Demyelination in GBS begins at the distal part of the spinal nerve and progresses towards the dorsal root ganglion. Occasionally degeneration of the axon will occur as well.

Clinical Features

Symptomology is variable among individuals. Common initial symptoms include numbness and tingling in the lower limbs or back pain, followed by weakness in the legs and arms. Tendon reflexes are generally absent or greatly reduced and paralysis of extraocular muscles, causing ptosis (eyelid drop) and diplopia (double vision), occurs in about 10% of cases.

Cranial nerves are affected in over 50% of all cases, with the facial nerves being most affected. Typically protein levels in the cerebral spinal fluid are also elevated; while white cell counts are not increased (Cortson et al., 1991).

Patients with GB also have inexcitable motor nerves with low amplitude compound muscle action potentials (CMAPs), as well as deficits in sensory nerves.

Low amplitude CMAPs or inexcitable motor nerves in the initial stages of the syndrome are thought to be due to either distal conduction block or axonal degeneration.

Improvement of CAMP amplitude on sequential studies suggests a good outcome at 1 year (Triggs, 1992).


Progression of the disorder can continue for up to four weeks, with about one third of patients beginning to improve within two weeks.

Onset may take a range of hours to weeks, but about 30% of patients experience maximum deficit within seven days.

Approximately 60% of patients are unable to walk during their deficit plateau, and respiratory function is impaired in over half of the cases, requiring ventilation in about 25% of these patients (Rees, Thompson, Smeeton, & Hughes, 1998).

Recovery can last from 6 months to 2 years with a full recovery rate of up to 90% of patients. About 35 to 45% of patients will have mild, long lasting deficits, and 5 to 15% will have significant long-term disability.

Although generally nonfatal, death due to GBS occurs in approximately 1 to 5% of cases, usually due to respiratory or cardiovascular complications (Rantala, Uhari, & Niemela, 1991).

Poor prognosis has been associated with old age, rapid progression of illness, ventilator dependence, mean CMAP amplitude less than or equal to 20% or 10% of lower limit of normal, a compound abductor pollicis brevis muscle action potentials being less then 1 mV or absent, antecedent gastroenteritis, disability, electrophysiological signs of axonopathy, latency to nadir, and duration of active disease (Lyu et al., 1997).

Treatment Options

Corticosteriods : Steroids have been used to try to increase muscle usage, but no evidence supports this therapy (Visser et al., 1998).

Plasmapheresis (Plasma Exchange): Approximately 200 to 250mL/kg of plasma is exchanged over 7-14 days to filter out possible antibodies and harmful substances in the blood. This treatment has been shown to provide improvement for only a small population of patients (Kennard, Newland, & Ridley, 1982).

Intravenous immunoglobulin : Injections of high dosages of human immunoglobulin (0.4 g/kg per day for 5 days) have been found to be somewhat effective for a small population of patients.

Antibiotics : Possible early treatment with erythromycin may help produce a shorter duration of illness if the patient has campylobacter associated GBS (Goddard, Lastovica, Argent, 1997).

Rehabilitation : Physical therapy to improve muscle tone and strength as well as counseling is the major form of treatment.

Subtypes of Guillain-Barré Syndrome

Recurrent Guillain-Barré Syndrome : Patients who have more than 32 episodes of Guillain Barré attacks are classified as having recurrent Guillain Barré syndrome (RGBS), or chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Patients with RGBS have a more rapid onset of symptoms with subsequent or near complete recovery, high incidence of an antecedent illness, lack of response to immunosuppressive therapy, and normal cerebral spinal fluid protein levels at the onset of recurrence.

Peak deficit and duration of attacks vary but the distribution of weakness remains constant.

Miller-Fisher Syndrome : The most common subtype is Miller-Fisher syndrome (MFS), or acute disseminated encephalomyeloradiculopathy, which is characterized by triad of ataxia, areflexia, and ophthalmoplegia.

This subtype of GBS occurs with low frequency (about 2% to 7%) in the western world but about 19% in the eastern world.

The cause of MFS may be due to a brain stem abnormality alone or in combination with peripheral nerve damage.

Central nervous system involvement is evident through symptoms such as drowsiness, ophthalmological signs such as ptosis, bulbar palsy, Bell's palsy, and abnormal imaging study in the brain stem (Lyu et al., 1997).

Acute motor-sensory axonal neuropathy (AMSAN): which is characterized by early axonal degeneration of motor and sensory fibers (Lyu et al., 1997).

Acute motor axonal neuropathy (AMAN) : includes patients with Guillain Barré syndrome who do not have sensory loss during a follow-up period of six months and demonstrate early axonal degeneration of only motor fibers.

Compared to other Guillain Barré patients, these patients all experienced a more rapid onset of weakness (3.9 versus 6.1 days), an earlier nadir (6.3 versus 9.1 days), an initially predominant distal weakness (67% versus 27%), sparing of cranial nerves (26% versus 68%), and preceding gastro-intestinal illness (41% versus 13%) generally caused by Campylobacter jejuni (67% versus 28%) (Visser et al., 1995).

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