Case Evaluation

Types of Brain Injuries: Guillain-Barre Syndrome (GBS)

Guillain-Barré Syndrome (GBS)

What is Guillain-Barré syndrome (GBS)?

Guillain-Barre syndrome is an uncommon inflammatory disorder in which your body's immune system attacks your nerves, typically causing severe weakness and numbness that usually starts in your extremities and quickly worsens. Eventually your whole body can become paralyzed, even the muscles used for breathing.

Luckily, this potentially deadly disorder is relatively rare, occurring worldwide in only one or two people per 100,000. All age groups can be affected, but it occurs most often in young adults and the elderly.

In its most severe form, Guillain-Barre syndrome is a medical emergency requiring hospitalization. About 30 percent of the people who have the disorder will temporarily need the help of a machine to breathe.

There is no cure for the disorder, but several treatments can ease symptoms and reduce the duration of the illness. Most people recover completely from even the most severe cases of Guillain-Barre syndrome.

What causes Guillain-Barré syndrome?

The exact cause of GBS is not known. However, it is believed that the disorder is an autoimmune disease, a condition in which the immune system attacks its own tissues as though they were foreign substances. Nerves are damaged by the immune system, usually in response to a viral or bacterial infection or other illness. As the immune system produces antibodies to fight the infection or illness, it may also produce antibodies that attack the covering (myelin sheath) of the peripheral nerves and sometimes the nerve fibers (axons). The resulting nerve damage leads to tingling and numbing sensations, muscle weakness, and paralysis.

Although it is not known exactly what triggers the body's response, GBS most often develops after a respiratory or gastrointestinal infection. A variety of infections may be associated with GBS. Those that have been most commonly linked to the disease are Campylobacter jejuni (a bacterial infection that affects the intestinal tract), mycoplasma (a type of bacteria that can cause pneumonia), cytomegalovirus (CMV), Epstein-Barr virus (EBV), and varicella-zoster virus, the virus that causes chickenpox and shingles.

It was thought that the onset of GBS may have been linked to flu vaccines prior to 1977. However, a cause-and-effect relationship has not been established between any vaccine currently in use and GBS.

What are the symptoms?

Symptoms of Guillain-Barré syndrome (GBS) include:

  • Numbness or tingling in the hands and feet and occasionally around the mouth and lips.
  • Muscle weakness on both sides of the body in the legs, arms,face.
  • Loss of reflexes.
  • Difficulty speaking, chewing, and swallowing.
  • Inability to move the eyes.
  • Back pain.

The first symptoms of Guillain-Barré syndrome include tingling in the fingers and toes and weakness in the arms and legs that may develop several days or weeks after a respiratory or gastrointestinal infection. Tingling is a very common symptom that may be caused by a wide variety of medical problems.

However, once muscle weakness develops in addition to tingling, GBS becomes a more likely cause of these symptoms. Tingling and weakness may spread to the arms and upper body. GBS can become life-threatening if weakness spreads to muscles that control breathing, heart rate, and blood pressure.

Muscle weakness caused by GBS often gets worse over 1 to 4 weeks before it stabilizes and then gradually improves. In some people, symptoms continue to get worse for up to 3 months. In children, persistent symptoms of GBS may include tiring easily and having muscle pain during exercise.

How is Guillain-Barré syndrome diagnosed?

Guillain-Barré syndrome (GBS) may be difficult to diagnose in the early stages of the disease. A detailed examination of your nervous system is needed to diagnose the condition. Your health professional will ask questions about your symptoms, including when they started and how they have changed over time. A history of recent infection (especially respiratory or gastrointestinal illness) may also be an important clue in the diagnosis.

The two signs that must be present to diagnose GBS are:

  1. Progressive weakness in both arms and both legs.
  2. Loss of reflexes.

When these two signs are present, a diagnosis of GBS may be more likely if:

  • The symptoms developed over a period of days to several weeks.
  • The symptoms affect both sides of your body equally.
  • Mild sensory symptoms are present, such as numbness and tingling.
  • The muscles on each side of your face are affected.
  • You begin to recover 2 to 4 weeks after symptoms have stabilized.
  • You did not have a fever when symptoms first developed.

Some testing may be necessary to rule out other conditions, such as electrolyte imbalances and certain types of poisoning, that could also be causing the symptoms and to help confirm the diagnosis of GBS. These tests include:

  • Nerve conduction studies, which use electrical impulses to indicate how well the nerves are working. If you have GBS, the results may show a slowing of nerve function, which usually suggests that damage to the covering (myelin sheath) of the peripheral nerves has occurred.
  • A lumbar puncture, which is an analysis of the fluid around the spine. If you have GBS, this test may show increased amounts of protein in your spinal fluid without other signs of infection.

In the very early stages of GBS, the results of these tests may be normal. Abnormal results may not show up for a week or more after symptoms begin.

If the diagnosis is unclear, you may be referred to a doctor who specializes in brain and nervous system disorders (neurologist).

How is it treated?

Treatment of Guillain-Barré syndrome (GBS) depends on the severity of your symptoms and whether complications develop. The main treatment for GBS is immunotherapy, which includes plasma exchange or intravenous immune globulin (IVIG). Treatment is started immediately after you have been diagnosed with GBS that is getting worse. Early intervention with either of these treatments appears to be effective and may reduce recovery time. Neither treatment is better than the other, and there is no benefit to combining these treatments.

Additional treatment for symptoms of GBS may include:

  • Easing your breathing problems, sometimes through the use of a breathing machine (ventilator).
  • Monitoring your blood pressure and heart rate.
  • Providing adequate nutrition if you have problems chewing and swallowing.
  • Managing bladder and bowel problems.
  • Using physical therapy to help maintain muscle strength and flexibility
  • Preventing and treating complications such as pneumonia, blood clots in the legs, or urinary tract infections.

Careful monitoring is very important during the early stages of GBS because breathing problems and other life-threatening complications can occur within 24 hours after symptoms first develop.

Admission to a hospital or intensive care unit is often needed when muscle weakness progresses quickly. Muscle weakness may rapidly affect the muscles that control breathing. In such cases, temporary use of a mechanical ventilator may be necessary to help you breathe until you can breathe on your own again. Over the past 50 years, treating breathing problems from GBS with a ventilator has reduced the death rate from 30% to less than 5%.

Careful outpatient monitoring may be enough in cases where significant muscle weakness has not developed. It is important to continue to watch closely for any signs that the condition is getting worse so that you can contact your doctor or hospital immediately.

You probably will need to be hospitalized if you:

  • Are unable to move around on your own.
  • Develop significant paralysis.
  • Have breathing problems.
  • Develop blood pressure problems or an abnormal, very fast, or very slow heart rate.

If possible, you will be referred to a medical center that has experience treating the illness.

Recovery

Recovery may take 3 to 6 months, sometimes longer-in some cases, up to 18 months. People who have severe muscle weakness may need to stay at a rehabilitation hospital to receive ongoing physical therapy and occupational therapy as their motor function returns. For those who stay at home, devices that help perform certain daily activities can be used until motor function and muscle strength return.

Physical therapy and regular exercise are needed throughout the recovery period to strengthen the weakened muscles. The therapy program can be tailored to your specific needs.

Although recovery can be slow, most people who have GBS eventually recover.

  • Many people have some minor long-term effects, such as numbness in the toes and fingers. In most cases, these problems will not significantly interfere with your lifestyle.
  • Up to 20% of people have permanent problems that tend to be more disabling, such as weakness or balance problems. These problems may interfere with daily activities.
  • About 3% to 8% of people who develop GBS die from complications of the illness, such as respiratory failure, infection (often pneumonia), or heart attack.
  • Up to 67% of people who get GBS have some problems with persistent fatigue.

Relapses

Relapses or repeated episodes of GBS occur in about 5% to 10% of cases, and they may be very serious. If you have a relapse, aggressive treatment with plasma exchange or IV immune globulin may reduce the severity of the attack and prevent further relapses. If you have more than one relapse, treatment with other drugs may be needed.

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