Guillain-Barre Syndrome

What is Guillain-Barré Syndrome?

Guillain-Barré (ghee-yan bah-ray) Syndrome (GBS), also called acute inflammatory demyelinating polyneurapathy and Landry's ascending paralysis, is an inflammatory/immune disorder of the peripheral nerves - those outside the brain and spinal cord.

Under ordinary circumstances, the cells of the immune system attack only foreign material and invading organisms. In GBS, however, the immune system starts to attack part of the peripheral nervous system. In particular, it is the myelin sheath, the insulating layer that covers the axons of many peripheral nerves, that is destroyed. The myelin sheath speeds up the transmission of nerve signals and allows the transmission of signals over long distances. In some cases of GBS, the immune system attacks the axons themselves.

GBS is characterised by the rapid onset of weakness and, often, paralysis of the legs, arms, breathing muscles and face. It is the most common cause of rapidlly acquired paralysis in the United States today, affecting one to two people in every 100,000.

GBS can affect anybody, at any age, regardless of gender. It can develop over the course of hours or days, or it may take up to three to four weeks. 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.

What are the symptoms of Guillain-Barré Syndrome?

The first symptoms of Guillain-Barré Syndrome (GBS) include varying degrees of weakness or tingling sensations in the legs. Patients often lose their reflexes in the lower extremeties, which may be an early clue toward diagnosis. In many instances, the weakness and abnormal sensations spread to the arms and upper body.

Although many cases are mild, the symptoms can increase in intensity until certain muscles cannot be used at all, and, when severe, the patient is almost totally paralyzed. In these cases, GBS can be life threatening - potentially interfering with breathing and, at times, with blood pressure or heart rate - and is considered a medical emergency. Such a patient is often put on a respirator to assist with breathing and is watched closely for problems such as an abnormal heartbeat, infections, blood clots, and high or low blood pressure.

Although most people recover, the length of illness is unpredictable and often months of hosptital care are required. The majority of patients eventually return to a normal or near-normal lifestyle, but many endure a protracted recovery and some remain in a wheelchair indefinately.

What causes Guillain-Barré Syndrome?

At this time it is not clear if there is a specific disease-causing agent involved in Guillain-Barré Syndrome (GBS), which is why it is called a syndrome rather than a disease. It is also not yet known why GBS strikes some people and not others.

What we do know is that GBS usually occurs a few days or weeks after an individual has had symptoms of a viral infection of the respiratory (lungs) or gastrointestinal (stomach and intestines) systems. Surgery or vaccinations have also, on occasion, triggered the syndrome.

How is Guillain-Barré Syndrome diagnosed?

Since several disorders have symptoms similar to those found with Guillain-Barré Syndrome (GBS), doctors must examine and question patients carefully to be sure of their diagnosis. It is the pattern of signs and symptoms that help doctors make the correct diagnosis. Some of the things your doctor may check for include:

  • Are symptoms on both sides of your body? This is most common with GBS.

  • How quickly did muscle weakness progress? With GBS, muscle weakness progresses over days or weeks, rather than months as with other conditions.

  • Do you still have the jerk reflex in your knees? Your doctor will check the reflexes in your knees because these are usually lost in GBS.

  • Has nerve conduction slowed down? You may be given a nerve conduction velocity (NCV) test to see if signals traveling along the nerve are slower. Because the myelin sheath, which speeds signal conduction, is damaged in GBS, the velocity of nerve signals would be slower than normal. In children, these studies may point to damage of the axons, rather than the nerve sheaths themselves.

  • Is there excess protein in the cerebrospinal fluid? Your doctor may request a spinal tap to check for increased protein level in your cerebrospinal fluid, the fluid that bathes your brain and spinal cord. With GBS, there will be more protein in this fluid than normal. This finding may help in making the diagnosis.

How is Guillain-Barré Syndrome treated?

There is no known cure for Guillain-Barré Syndrome (GBS), but there are some therapies that can alleviate symptoms and speed recovery. These therapies include:

  • Plasmapheresis: Whole blood is removed from the body and processed so the red and white blood cells are separated from the liquid portion of the blood, called plasma. The blood cells are then returned to the body without the plasma, which the body replaces. Researchers don't know why this therapy works, however they suspect it is because the plasma contains the parts of the immune system that are harmful to the myelin.

  • High-dose Immunoglobin Therapy: High doses of immunoglobins - immune system proteins - are given by intravenous injection to attack invading organisms and other foreign bodies. Research has shown that giving high doses of these proteins, collected from thousands of people without GBS, can reduce the severity of the GBS episode. At present, the reason why this therapy works is unknown.

Because many patients with GBS become paralyzed and may lose the ability to breath on their own, they are most often treated in hospitals where they have access to respirators, heart monitors and other machines that help the body function. Also, hospital staff can better treat GBS patients for the many potential side effects of paralysis, such as bedsores or pneumonia.

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