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Diseases and Conditions
Guillain-Barre syndrome
From MayoClinic.com
Special to CNN.com

Introduction

Guillain-Barre (ge-YAH buh-RA) syndrome (GBS) is an inflammatory disorder in which your body's immune system attacks the nerves outside the brain and spinal cord (peripheral nerves) and, rarely, parts of the brain itself. Severe weakness and numbness in your legs and arms characterize GBS. Loss of feeling and movement (paralysis) may occur in your legs, arms, upper body and face.

GBS affects an estimated one to three in every 100,000 persons annually in the United States. It can strike any race at any age, but its incidence increases with age. GBS may occur within days or weeks after a viral infection such as influenza (flu) or diarrhea. It may be triggered by pregnancy or a medical procedure, such as a vaccination or minor surgery, or have no evident reason for developing. Because the cause of GBS is unknown, there's no way to prevent the disease from occurring.

In its most severe form, GBS is a medical emergency and may require hospitalization. Severe GBS may result in total paralysis, potentially dangerous fluctuations in heart rate and blood pressure, and inability to breathe without respiratory assistance. The muscles you use for eye movement, speaking, chewing and swallowing also may become weak or paralyzed. People with severe GBS often need long-term rehabilitation to regain normal independence, and as many as 15 percent experience lasting physical impairment. In some cases, GBS can be fatal.

Most people recover from even the most severe cases of GBS. Available treatments, if started soon after signs and symptoms appear, may lessen the severity of GBS and reduce recovery time.

Signs and symptoms

The signs and symptoms of GBS usually appear rapidly over the course of a single day. These may include:

  • Weakness, tingling or loss of sensation that often begins in your feet and legs and spreads to your upper body and arms
  • Moderate pain throughout your body
  • Difficulty breathing
  • Paralysis of your legs, arms, respiratory muscles and face
  • Difficulty with eye movement, facial movement, speaking, chewing or swallowing
  • Very slow heart rate or low blood pressure
  • Difficulty with bladder control or intestinal functions

GBS progresses quickly, with most people experiencing the most significant weakness in the legs, arms, chest and other areas within three weeks of the start of this disorder. In some cases, the signs and symptoms of GBS may progress very rapidly with complete paralysis of legs, arms and breathing muscles over the course of a few hours.

If GBS is mild, the signs and symptoms may not extend beyond a feeling of general weakness. GBS may improve on its own within a few weeks, and some people initially may think the signs and symptoms are due simply to a common virus.

The signs and symptoms of GBS may last days, weeks or months before muscle sensation begins to return. Regaining your pre-illness strength and functioning is slow, sometimes requiring months or years. However, most people with GBS return to normal within months.

Causes

Doctors and scientists don't know what causes Guillain-Barre syndrome. However, they believe that GBS is a disorder of the immune system. Research indicates that the immune system may destroy the protective covering of the peripheral nerves (myelin sheath), which disables the nerves from transmitting signals to the muscles. When this happens, muscles become unable to respond to nerve commands. Weakness, numbness or paralysis may occur. The brain also receives fewer sensory signals, resulting in the inability to feel heat, cold, pain and other sensations.

The most recognized conditions that occur before GBS develops are bacterial or viral infections. Approximately two-thirds of people affected by GBS have had a recent infectious illness, such as sore throat, diarrhea, cold or flu, within one to four weeks before the onset of GBS. The Epstein-Barr virus or Hodgkin's disease also may precede GBS.

A bacterial infection such as campylobacteriosis — caused by campylobacter, bacteria commonly found in undercooked food, especially poultry — may be a possible cause of GBS. This form of the disorder is usually more severe because it directly attacks the nerves, instead of the nerve coverings.

GBS may also be triggered by medical procedures including surgery and, in rare cases, influenza immunizations. However, the connection between the flu vaccine and GBS is weak, and the risk the vaccine poses to your health is much less significant than your risk of serious flu-related illness.

When to seek medical advice

GBS is a serious disease that requires immediate hospitalization because of the rapid rate at which it worsens. Early treatment is important. The sooner appropriate treatment is started, the better the chance of a good outcome. If treatment can result in reducing the inflammation of your peripheral nerves, the chances of serious permanent damage to the myelin sheath and nerve fibers may be substantially decreased.

If you experience a tingling sensation in your toes, feet or legs followed by muscle weakness, seek medical attention immediately. Failure to see your doctor promptly may lead to progression of the disorder and paralysis, which can leave you incapacitated and unable to get help.

Screening and diagnosis

GBS can be difficult to diagnose in its earliest stages. Its signs and symptoms are similar to those of other neurological disorders and may vary from person to person.

The first step in diagnosing GBS is for your doctor to take a careful medical history to fully understand the cluster of signs and symptoms that you're experiencing. People with GBS have a relatively sudden onset of signs and symptoms, and muscle weakness usually occurs on both sides of the body as opposed to on one side only, as in a stroke.

Doctors commonly use two tests to determine if GBS is present:

  • Electromyography (EMG). An EMG helps establish a diagnosis and the extent of a neurological problem by measuring the electrical activity of a muscle in response to nerve stimulation. The test also measures the nature and speed of impulse conduction along a nerve. In an EMG, a technician inserts electrodes in fine needles into the muscles being tested and places electrodes on your skin over peripheral nerves.
  • Spinal tap (lumbar puncture). This procedure involves inserting a needle into your spinal canal, usually at the low back (lumbar) level. Your doctor can determine the pressure of the cerebrospinal fluid (CSF), and a sample of fluid can be removed for laboratory analysis. This analysis may include checking for evidence of bleeding, the number and types of white blood cells, the levels of glucose and protein, the types of proteins, and tests for bacteria and fungi.

Complications

The long-term outlook for most people with GBS is good. About 75 percent to 85 percent of those affected recover completely or have only minor, residual weakness or abnormal sensations such as numbness or tingling.

Up to 15 percent of people with GBS experience more serious, permanent problems with sensation and coordination, including some cases of severe disability. About one in ten of those affected is at risk of experiencing a relapse.

GBS may cause severe damage to your muscles and nervous system, weakening your heart and lungs. About one-third of people with GBS require assisted ventilation while they are ill. Up to one in 12 people with GBS die of related complications such as respiratory failure, pulmonary embolism and cardiac arrest. Older adults with other medical problems are at greatest risk of death from GBS.

Treatment

There's no cure for GBS. However, certain therapies can lessen the severity of the illness and accelerate recovery for most people. The general treatment for GBS is supportive care to help with activities of daily living, such as eating and using the bathroom. For some, recovery can take a long time — from several months to a year or more.

According to a guideline developed by the American Academy of Neurology (AAN), treating GBS early, within two to four weeks after signs and symptoms first appear, may speed recovery time. Two main treatments, both equally effective, have been shown to speed the recovery from and reduce the severity of GBS in adults:

  • Plasmapheresis (plaz-muh-fuh-RE-sis). This treatment — also known as plasma exchange — is a type of "blood cleansing" in which damaging antibodies are removed from your blood. Plasmapheresis consists of removing the liquid portion of your blood (plasma) and separating it from the actual blood cells. The blood cells are then put back into your body, which manufactures more plasma to make up for what was removed. It's not clear why this treatment works, but scientists believe that plasmapheresis removes certain antibodies from plasma that contribute to the immune system attack on the peripheral nerves.
  • Intravenous immunoglobulin (IVIg). Immunoglobulin contains healthy antibodies from blood donors. High doses of immunoglobulin can block the damaging antibodies in your blood that may contribute to GBS.

The AAN guideline also shows no benefit in combining plasma exchange and IVIg. Mixing the treatments or administering one after the other is no more effective than if either method is used alone.

Your treatment also may include pain medications including acetaminophen and nonsteroidal anti-inflammatory drugs, possibly in combination with narcotic painkillers.

Often before recovery begins, caregivers may need to manually move your arms and legs to help keep your muscles flexible and strong. After recovery has begun, you'll likely need physical therapy to help regain strength and proper movement to be able to function on your own. Whirlpool therapy (hydrotherapy) may help relieve pain and retrain the movement of your affected limbs. After your symptoms subside, your rehabilitation team will prescribe an active exercise routine to help you regain muscle strength and independence. You may need training with adaptive devices, such as a wheelchair or braces, to give you mobility and self-care skills.

Self-care

Depending on the severity of GBS, self-care may be limited until the recovery process has begun. Once the crisis phase is over and rehabilitation starts, work closely with your doctor and rehabilitation team to ensure the best possible outcome. Although it's often difficult and sometimes painful, try to be as cooperative as possible during physical therapy and other demanding rehabilitation activities. Know that your hard work, dedication and perseverance will pay off with the regaining of your mobility and independence.

Coping skills

Living with a physical disability presents daily challenges. Some of these suggestions may make it easier for you to cope:

  • Set priorities. Decide which tasks you need to do on a given day and which can wait until another time. Stay active, but don't overdo it.
  • Get out of the house. When your mobility is restricted, it's natural to want to be alone. But this only makes it easier to focus on your disability. Instead, ask a friend to visit or take you on short errands.
  • Seek and accept support. It isn't a sign of weakness to ask for or accept help when you need it. In addition to support from family and friends, consider joining a support group. Although support groups aren't for everyone, they can be good places to hear about coping techniques or treatments that have worked for others. You'll also meet people who understand what you're going through. To find a support group in your community, check with your doctor, a nurse or the county health department.
  • Talk to a counselor or therapist. You may find it helpful to talk to a counselor or therapist in addition to your primary care doctor. Counseling can help relieve the emotional difficulties you're feeling because of your sudden paralysis and dependence on others.

  • Video: Therapeutic plasmapheresis
  • May 31, 2005

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