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Guillain-barré Syndrome

Alternate Names

  • Landry-Guillain-Barrésyndrome

Definition

Guillain-Barré syndrome, abbreviated GBS, is believed to be an autoimmune disorder in which the body creates antibodies against its own tissue. In people with GBS, the antibodies generally attack the myelin sheath, or lining of the nerves. They may also attack part of the nerves themselves.

What is going on in the body?

Guillain-Barré syndrome is thought to be an autoimmune disorder that is triggered by an infection or other factors. It causes a severe inflammatory reaction around the nerves. The myelin sheath becomes swollen. Impulses and messages cannot travel along the course of the nerve. In time, nerve impulses are blocked.

Risks

What are the causes and risks of the condition?

Most cases of Guillain-Barré syndrome are triggered by an infection. Two-thirds of individuals with GBS have had an upper respiratory infection or gastrointestinal infection one to three weeks before weakness develops. Other infections that may trigger GBS include:
  • chlamydia infections
  • cytomegalovirus infections
  • hepatitis B
  • HIV infections, which cause AIDS
  • mononucleosis
  • mycoplasma pneumonia
During the 1976 swine flu vaccination campaign, a handful of cases of GBS occurred in vaccinees, significantly more than would have been expected had the vaccine not been given. The campaign was terminated for that reason. Some cases of GBS have since been reported following the receipt of other flu vaccines, and after rabies vaccine, but causative relationships have not been shown.
Certain medicines have been identified as triggers for GBS. These include:
  • captopril, used to treat high blood pressure
  • danazol, a hormone derivative
  • gold, which is injected for diseases such as rheumatoid arthritis
  • heroin, a highly addictive illegal drug
  • penicillamine, which is used for chelation and to treat rheumatoid arthritis
  • streptokinase, used to dissolve blood clots
GBS may be triggered by a variety of other factors, such as:
  • blood cancers, especially Hodgkin lymphoma
  • pregnancy
  • surgery
No one knows for sure why these factors trigger Guillain-Barrésyndrome in some people but not in others. More research is needed in this area.

Prevention

What can be done to prevent the condition?

Most cases of Guillain-Barrésyndrome cannot be prevented. Following safer sex guidelines may prevent some cases that are triggered by sexually transmitted diseases, such as HIV. Illegal drugs, such as heroin, should not be used.

Diagnosed

How is the condition diagnosed?

Diagnosis of Guillain-Barré syndrome begins with a medical history and physical exam. A test called a nerve conduction velocity (NCV) test can detect damage to the myelin sheath. An electromyograph (EMG) can show if there is a lack of nerve signals to the muscles. During an EMG, wires are connected to the skin, and the activity of nerves and muscles is measured. An electrocardiogram (ECG) may also be ordered.

Long Term Effects

What are the long-term effects of the condition?

About 95% of those with Guillain-Barré syndrome survive, and about 75% completely recover. For some, mild weakness or chronic pain will continue throughout life. GBS can be fatal if it causes difficulty breathing. Full recovery can be expected if symptoms go away not long after they appear, usually within 3 weeks.
Weakness and decreased sensation can cause contractures. Contractures occur because muscles become thick and tight when they are not used. They shrink and shorten, causing joint deformities. There can also be loss of movement in the affected areas. Blood clots may develop because of loss of motion and muscle contractions. Blood-thinning medicines, such as warfarin or heparin, can be given to help prevent blood clots. Individuals with GBS are also at increased risk of infections, including pneumonia.

Other Risks

What are the risks to others?

Guillain-Barrésyndrome is not contagious and poses no risk to others. If GBS is triggered by an infection, the infection itself may be contagious.

Treatments

What are the treatments for the condition?

Initial treatment of GBS focuses on life support. A ventilator (artificial breathing machine) may be needed. Fluids can be given through an intravenous line and food through a tube into the stomach.
A procedure called plasmapheresis can be effective if used early in the course of the illness. Plasmapheresis removes antibodies that may be present in the bloodstream. It also provides the person with fluids or plasma that is free of the antibodies that trigger GBS.
Immunoglobulin therapy is also used to treat GBS. High doses of immunoglobulins, or proteins from the immune system of normal donors, are given to the person with GBS. For unknown reasons, these globulins reduce the body's attack on its own nerves. Corticosteroids, such as methylprednisolone, are sometimes given together with plasmapheresis or immune serum globulin. However, their effectiveness is questionable, and they may worsen the disease.

Side Effects

What are the side effects of the treatments?

Plasmapheresis is associated with a small risk of getting a bloodborne infectious disease. Corticosteroids and immune globulins may increase the person's risk for many types of infection.

After Treatment

What happens after treatment for the condition?

Physical therapy may be ordered to prevent contractures and other complications. Along with occupational therapy, it can be helpful in restoring the person's normal level of function. Pain medicines and physical therapy may be needed if the person has chronic pain.

Monitor

How is the condition monitored?

After the acute phase of GBS, the individual needs to make have regular visits to a healthcare professional, to whom any new or worsening symptoms should be reported.

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