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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:
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:
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:
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:
May 31, 2005 |