What Is Guillain-Barré Syndrome? What Causes Guillain-Barré Syndrome?

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Main Category: Neurology / Neuroscience
Article Date: 20 Oct 2009 - 0:00 PDT

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Guillain-Barré syndrome is a rare but serious disease of the peripheral nervous system, which is the network of nerves that control the body's senses and movements. In this disorder the body's immune system attacks the nerves. Usually, the first symptoms are tingling, weakness and numbness in the extremities. These sensations can quickly spread, eventually paralyzing the whole body.

In 1859, the disorder was first described by the French physician Jean Landry. In 1916, Georges Guillain, Jean Alexandre Barré, and Andre Strohl diagnosed two soldiers with the illness. They discovered the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count.

Recently, a peer-reviewed study found that U.S. president Franklin D. Roosevelt's paralysis (long attributed to poliomyelitis) was actually caused by Guillain-Barré syndrome.

According to Medilexicon's medical dictionary, Guillain-Barré syndrome is: "an acute, immune-mediated disorder of peripheral nerves, spinal roots, and cranial nerves, commonly presenting as a rapidly progressive, areflexive, relatively symmetric ascending weakness of the limb, truncal, respiratory, pharyngeal, and facial musculature, with variable sensory and autonomic dysfunction…"

The exact cause of Guillain-Barré syndrome is unclear. It is difficult determine who is most at risk. However, it is often preceded by an infectious illness such as a respiratory infection or stomach flu. It is likely that the infection causes the immune system to attack the body's own nerves. Fortunately, Guillain-Barré syndrome is relatively rare, affecting only 1 or 2 people per 100,000. It is slightly more common in men than women and can affect people of any age, including children.

In most severe cases, hospitalization is required. There is no known cure but several treatments can ease symptoms and reduce the duration of the illness. Most people affected with the syndrome make a full recovery within a few weeks or months and do not have any further problems.

What are the signs and symptoms of Guillain-Barré syndrome?

A symptom is something the patient feels or reports, while a sign is something other people, including the doctor detects. For example, a headache may be a symptom while a rash may be a sign.

Guillain-Barré syndrome usually develops quickly, over a few days. In some cases it can develop gradually, over a period of up to four weeks. These symptoms often do not cause much bother in the beginning. In some people, as the disorder progresses, muscle weakness can evolve into paralysis.

Symptoms first appear one to three weeks after a minor infection, such as a cold or sore throat.

Early symptoms

Symptoms usually start in the feet and hands and then they may spread up the limbs, affecting the legs and arms a few days later. They include:

• Instability
• Numbness
• Progressive muscle weakness
• Stinging
• Unsteady walking or inability to walk
• Weakness or tingling sensations in legs that spread to upper body

Possible later symptoms

In some people, the muscle weakness progresses to:

• Difficulty with bladder control or digestion
• Difficulty with chewing or swallowing
• Difficulty with eye movement and speech
• Slow heart rate or low blood pressure
• Temporary paralysis of the legs, arms and face
• Temporary paralysis of the respiratory muscles that control breathing
• Severe pain in lower back

The extent of paralysis varies with each case of Guillain-Barré syndrome.

This syndrome can be life-threatening because it can paralyze respiratory muscles and halt breathing. In this case, the patient will be put on a ventilator to help breathing. About one in four people need to be artificially ventilated.

If any of the following severe signs or symptoms appears, it is advised to seek emergency medical help:

• Tingling starting in the feet or toes ascending upward through the body
• Tingling or weakness that is spreading rapidly
• Tingling that involves both hands and feet
• Difficulty catching a breath
• Choking on saliva

Because Guillain-Barré syndrome worsens rapidly, it is a serious disease that requires immediate hospitalization. The faster appropriate treatment is started, the better the chance of a good outcome.

What are the causes of Guillain-Barré syndrome?

The precise cause of Guillain-Barré syndrome is unclear. In about 60 percent of cases, an infection of the lungs or the digestive tract (such as a throat infection, flu or sometimes a bacterial infection such as salmonella) precedes the disorder. However, experts do not know why for some people and not for others such an infection can lead to Guillain-Barré syndrome.

Usually the immune system only attacks foreign material and invading organisms. In this disorder, it begins attacking the nerves that carry signals between the body and the brain.

It is thought that the infection triggers the immune system to attack the body, in the following way:

• The virus or bacterium causes the immune system to damage the protective covering (myelin sheath) of the peripheral nerves.


• This damage stops the nerves from sending signals to the muscles.


• This then causes the tingling, weakness and eventual short-term paralysis that might be experienced.


• The peripheral nervous system is the network of nerves that control the body's senses and movements.


• They spread from the brain and spinal cord (the central nervous system) and carry impulses to and from the rest of the body, such as the limbs and organs.

Possible triggers

Guillain-Barré syndrome can affect all age groups but there is greater risk for adults and older adults. The following are believed to be possible causes of Guillain-Barré syndrome, although there is no scientific proof:

• A bacterial infection (such as salmonella poisoning which is a type of bacteria often found in undercooked food, especially poultry).
• A virus, such as cytomegalovirus (a member of the herpes group).
• Hodgkin's disease (tumor of the lymph glands).
• Epstein-barr virus.
• Mononucleosis.
• HIV virus.
• Recent surgery.

Vaccination (rabies or influenza immunizations) has also been linked to the onset of Guillain-Barré syndrome. However, studies suggest a very low risk of developing the syndrome after receiving a vaccine. Guillain-Barré syndrome is not contagious or hereditary.

How is Guillain-Barré syndrome diagnosed?

Guillain-Barré syndrome can sometimes be difficult to diagnose because the symptoms vary from person to person. During a medical examination doctors carefully review the patient's medical history and usually test their knee-jerk reflex. Also, the symptoms can be very similar to those of other brain and nervous system disorders. Therefore, doctors use two tests to confirm the diagnosis of Guillain-Barré syndrome:

• Electromyography (EMG). This is a test that measures the health of the muscles and the nerves that control them. A needle electrode is inserted through the skin and into the muscle to give an electrical recording of muscle activity to see if the muscles respond when certain nerves are stimulated. In Guillain-Barré syndrome, the muscles may not respond because of nerve damage. When testing the nerves, the electrode is also used to stimulate the nerves with a small electric shock and to measure the response. It determines how quickly nerves are able to conduct electric signals.


• Lumbar puncture (spinal tap). A needle is inserted into the spinal canal (usually in the lower back), under local anesthetic. This test helps determine the pressure of the spinal fluid inside. A sample of the spinal fluid is taken and tested for evidence of bleeding in the brain and spinal cord. It is also possible to check the protein levels in the spinal fluid and look for any bacteria or fungi.


• A blood test might be needed to rule out any other possible causes.

What are the treatment and drugs of Guillain-Barré syndrome?

Although in some cases recovery can take months and even years, most cases of Guillain-Barré syndrome follow this general evolution:

• Following the first symptoms, the condition tends to worsen progressively for about two weeks.
• Symptoms remain steady for two to four weeks.
• Recovery begins, usually lasting six to 12 months.

There's no cure for Guillain-Barre syndrome. But two types of treatments speed recovery and reduce the severity of the disorder:

• Plasmapheresis or plasma exchange. Damaging antibodies are removed from the blood. The liquid portion of the blood (plasma) is removed and separated from the actual blood cells. The blood cells are then put back into the body, which manufactures more plasma to make up for what was removed. It is unclear why this treatment works, but scientists believe that plasmapheresis rids plasma of certain antibodies that contribute to the immune system attack on the peripheral nerves.


• Intravenous immunoglobulin (IVIg). It involves administering high doses of healthy immunoglobulin, from blood donors. This can block the damaging antibodies that may contribute to Guillain-Barré syndrome.

After recovery has begun, physical therapy (UK: physiotherapy) is needed to help regain strength and proper movement as well as the use of adaptive devices, such as a wheelchair or braces. Physiotherapy involves a range of treatments, including manipulation, massage, exercise, electrotherapy and hydrotherapy.

Counseling can be reassuring and may help recovery from Guillain-Barré syndrome. The focus is on discussing problems, addressing issues and achieving a more positive outlook.

What are the complications of Guillain-Barré syndrome?

Less than 1 in 10 people with Guillain-Barré syndrome do not recover completely and become wheelchair-bound, or have permanent weakness or numbness. They can experience long-term complications, such as:

• Breathing difficulties. A potentially deadly complication of Guillain-Barré syndrome is that the weakness or paralysis can spread to the muscles that control breathing. This may lead to respiratory distress syndrome and heart attack. This usually only happens in the elderly.


• Residual numbness or other sensations. Most people with Guillain-Barré syndrome recover completely. Some may have only minor, residual weakness or abnormal sensations, such as numbness or tingling. Full recovery may be slow, often taking a year or longer.


• Disability. Serious, permanent problems with sensation and coordination.


• A relapse of Guillain-Barré syndrome.

Recovery

Usually, a full recovery takes a few weeks or months after symptoms started. Some people, however, spend a few months in hospital and take up to a year to recover.

The emotional impact of Guillain-Barré syndrome can be devastating. In severe cases, Guillain-Barré syndrome can suddenly transform healthy and independent individuals into critically ill and physically helpless people.

Most people eventually recover fully. However, a diagnosis of Guillain-Barré syndrome means confronting the possibility of long-term disability or paralysis. Individuals who do develop these complications must adjust to lasting, limited mobility. They become dependent on others to help manage daily activities.

Mental health therapy can play a critically important role in helping to cope with the mental and emotional strain of this illness. In some cases, family counseling may be recommended to help the patient and loved ones adjust to the changes caused by Guillain-Barré syndrome.

Talking with others who have experienced this illness may be beneficial. This can be done through a support group for people and families coping with Guillain-Barré syndrome.

Written by Stephanie Brunner B.A.
Copyright: Medical News Today
Not to be reproduced without permission of Medical News Today