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Guillain-Barre syndrome

Alternative Names
infectious polyneuritis, acute demyelinating polyneuropathy, acute idiopathic polyneuritis, Landry-Guillain-Barre syndrome, acute inflammatory polyneuropathy, Ascending palsy

Definition
Guillain-Barre is a progressive weakness of the muscles. It is related to inflamed nerves. The inflammation often follows an infection.

What is going on in the body?
Guillain-Barre is associated with a severe inflammatory reaction around the nerves. This results in muscle weakness or paralysis.

Nerves are covered by a fatty layer called myelin. This covering becomes swollen in Guillain-Barre syndrome and the nerves do not work well. Impulses and messages cannot travel along the course of the nerve. In time, nerve impulses are blocked. Weakness usually begins in the feet and legs and moves up the body.

What are the signs and symptoms of the condition?
After upper respiratory or stomach infections, the disease follows within 24 hours. Muscle weakness or paralysis starts in the legs and feet. It progresses up toward the arms and head. The spread from feet to head can occur within 24 and 72 hours but can take longer. Sometimes it starts in the arms and moves downward. Sometimes it starts in the arms and legs at the same time. Symptoms can range from a feeling of general weakness to total paralysis. Symptoms are:
  • numbness and decreased sensation
  • unco-ordinated movements
  • muscle pain
  • blurred vision
  • difficulty breathing
  • dizziness
  • heart palpitations
  • difficulty urinating or even starting to urinate
  • loss of bowel and/or bladder control
  • constipation
  • muscle contractions
  • drooling
  • fainting
  • difficulty swallowing
What are the causes and risks of the condition?
The exact cause is not known. It could be secondary to an infectious disease, including some viral infections. Usually Guillain-Barre follows an infection of the upper respiratory system or stomach. These infections have gone away before the signs of Guillain-Barre emerge.

Approximately eight out of 100,000 people develop the condition. It occurs in both sexes. People with the condition are usually between the ages of 30 and 50. AIDS, some cancers, lupus, other viral infections such as herpes or surgery in the past six weeks may increase the risk of developing Guillain-Barre.

What can be done to prevent the condition?
There is no known way to prevent the condition.

How is the condition diagnosed?
A review of recent illnesses followed by muscle weakness and paralysis may signal Guillain-Barre. A doctor will do an examination to see how well the nerves and muscles in the body are working. The doctor will also check vital signs and watch for problems breathing or swallowing.

A test called a nerve conduction velocity (NCV) will show demyelination. Demyelination occurs when the outer fatty layer covering the nerves, the myelin, is destroyed and no longer protects the nerves or allows them to function as they should. Without this layer protecting them, the nerves do not work as well and signals do not travel well from nerve to muscle.

An electromyograph(EMG) is a test that may be done to 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 are measured by inserting a needle into the muscle. This test helps physicians decide if a patient really does have Guillain-Barre or if the symptoms are caused by some other disease. Looking at spinal fluid may show abnormalities. An electrocardiogram, or ECG, is a test where wires are connected to areas of the chest in order examine exactly how the heart is working. There may be ECG changes in some cases of Guillain-Barre.

What are the long-term effects of the condition?
About 95% of those with Guillain-Barre survive. About 75% completely recover. For some, mild weakness will continue throughout life. This disorder can be fatal because it causes problems with breathing. Full recovery can be expected if symptoms go away not long after they appear. This usually happens within three weeks.

What are the risks to others?
This is not a contagious disease.

What are the treatments for the condition?
Supportive treatment is important especially if there are problems breathing or swallowing. 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 and adds fluids or plasma back that do not have the antibodies. Giving these treatments early can shorten the duration of the inflammation and can help recovery occur more quickly. Intravenous immunoglobulin is also effective if used early in the course of the disease.

Frequently if there are problems with breathing or swallowing, hospitalisation may be required. Breathing problems may be assisted by a ventilator. Food may be given by a tube through the nose into the stomach, or directly into the stomach. This is to prevent food from going to the lungs and causing further problems.

What are the side effects of the treatments?
Receiving immunoglobulin is associated with a small risk of getting bloodborne infectious diseases and allergic reactions. Plasmapheresis can be associated with changes in blood volume and can cause conditions such as low blood pressure.

What happens after treatment for the condition?
Complications can be serious and can even result in death.

Weakness from decreased nerve impulses and decreased sensation can cause contractures. Contractures occur because muscles becomes thick and tight since they are not being used. As the muscles thicken they shrink and shorten losing a lot of their strength. Other joint deformities can be caused by lack of movement. There can also be loss of movement in the affected areas. Contractures and other joint deformities can be prevented by therapy to maintain motion in all of the joints. A physiotherapist usually provides this type of therapy. Families can also be taught how to take joints through range of motion to prevent complications.

Blood clots may develop because of loss of motion and muscle contractions. Blood thinning medications, such as warfarin or heparin, can be given to help prevent blood clots. There are also machines available that simulate contraction and relaxation of muscles to help prevent blood from pooling.

There is an increased risk of infection and pneumonia. There can also be difficulty breathing and aspiration of body fluids into the lungs.

How is the condition monitored?
Early in the disease, people may need to be in the hospital to be watched for breathing problems. However, once the disease starts subsiding, a doctor will help people monitor the disease.

Recovery may include a program for rehabilitation. People may need therapy to retrain movement in the affected limbs and assist with regaining muscle strength, co-ordination and independence.

Pain is frequently a complaint of Guillain Barre patients. This can be treated by both medication and physiotherapy. Recovery time can range from several months to over a year.

Author: James Broomfield, MD
Reviewer: HealthAnswers Australia Medical Review Panel
Editor: Dr David Taylor, Chief Medical Officer HealthAnswers Australia
Last Updated: 1/10/2001
Contributors
Potential conflict of interest information for reviewers available on request


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