Guillain-Barré syndrome

Guillain-Barré syndrome (GBS), is an acquired immune-mediated inflammatory disorder of the peripheral nervous system (i.e. not the brain or spinal cord). It is also called acute inflammatory demyelinating polyneuropathy, acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French Polio and Landry's ascending paralysis. more...

Overview

The pathologic hallmark of the disease is loss of myelin in peripheral nerves due to an acute and progressive inflammation of unknown cause. It is suggested that it is an autoimmune disease, in which the sufferer's immune system is triggered into damaging the nerve covering. There is some support for this in that half of all cases occur soon after a microbial infection or respiratory or gastrointestinal viral infection. Many cases developed in people who received the 1976 swine flu vaccine.

Peripheral nerves originate in the spinal cord and proceed to their target tissues (mainly muscle, skin and all internal organs). Their most proximal parts emerging from the spinal cord are called nerve roots and the inflammation in most (but not all) typical Guillain-Barré syndrome cases starts in these roots. Therefore, this condition is also referred to as acute polyradiculoneuritis.

Recent studies on the disease have demonstrated that approximately 80% of the patients have myelin loss, whereas, in the remaining 20%, the pathologic hallmark of the disease is indeed axon loss. The cases indicating the demyelinating form (AIDP) are called "acute motor and sensory axonal neuropathy" (AMSAN); the cases showing only motor symptoms (diffuse weakness) are called "acute motor axonal neuropathy" (AMAN). In a different and infrequent variant called Miller Fisher syndrome, patients develop ataxia, loss of tendon reflexes, and difficulty moving eye muscles but not weakness or sensory loss. All variants of Guillain-Barré syndrome are now supposed to be an autoimmune disease caused by antibodies against a variety of gangliosides found in abundant amounts in the peripheral nerve tissue.

Prevalence

GBS is a rare disease affecting about 1 to 2 people in every 100,000 annually. It does not discriminate with regard to the age or sex of sufferers. When diagnosed in young teenagers, it generally does not recur for many years, although when it does, it often does so in the fourth or fifth decade of life, long after the patients may have forgotten the details of the original episode.

Cause

About one half of patients have a history of preceding viral infection within two to four weeks prior to exhibiting the onset of Guillain-Barré syndrome. Guillain-Barré syndrome may also be associated with immunizations, recent surgery or trauma, pregnancy, Hodgkin's disease, chemo-therapy, and connective tissue diseases. The most frequently associated viral agents are cytomegalovirus (CMV), HIV, measles and herpes simplex virus. A bacterium called Campylobacter jejuni has recently been shown to be closely related with certain subtypes of the disease.

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