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Updated 15 February 2016

Guillain-Barré syndrome

This is a disorder in which the body's immune system begins to attack the body itself.

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Summary

  • Guillain-Barré syndrome is a disorder in which the body's immune system attacks part of the peripheral nervous system.  
  • This syndrome is rare. 
  • Occasionally, surgery or vaccinations will trigger the syndrome.
  • Usually Guillain-Barré occurs a few days or weeks after the patient has had symptoms of a respiratory or gastrointestinal viral infection.  
  • The first symptoms of this disorder include varying degrees of weakness or tingling sensations in the legs.

Cause

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.

Symptoms

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. Reflexes such as knee jerks are usually lost.

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.

Diagnosis

A nerve conduction velocity (NCV) test can give a doctor clues to aid the diagnosis because the signals travelling along the nerve are slower than normal.

The cerebrospinal fluid that bathes the spinal cord and brain contains more protein than usual, so a doctor may decide to perform a spinal tap to confirm the diagnosis.

Prognosis

Guillain-Barré syndrome can be a devastating disorder because of its sudden and unexpected onset. Most people reach the stage of greatest weakness within the first two weeks after symptoms appear, and by the third week of the illness 90 percent of all patients are at their weakest.

The recovery period may be as little as a few weeks or as long as a few years. About 30 percent of those with Guillain-Barré still have a residual weakness after 3 years.

About 3 percent may suffer a relapse of muscle weakness and tingling sensations many years after the initial attack.

Treatment

There is no known cure for Guillain-Barré syndrome, but therapies can lessen the severity of the illness and accelerate the recovery in most patients. There are also a number of ways to treat the complications of the disease. Currently, plasmapheresis and high-dose immunoglobulin therapy are used.

Plasmapheresis seems to reduce the severity and duration of the Guillain-Barré episode. In high-dose immunoglobulin therapy, doctors give intravenous injections of the proteins that in small quantities, the immune system uses naturally to attack invading organisms. Investigators have found that giving high doses of these immunoglobulins to Guillain-Barré patients can lessen the immune attack on the nervous system.

The most critical part of the treatment for this syndrome consists of keeping the patient's body functioning during recovery of the nervous system. This can sometimes require placing the patient on a respirator, a heart monitor, or other machines that assist body function.

Reviewed by Dr Andrew Rose-Innes, MD, Department of Neurology, Yale University School of Medicine, New Haven

 
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