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NORD is very grateful to Richard Hughes, MD, Cochrane Neuromuscular Disease Group, MRC Centre for Neuromuscular Disease, National Hospital for Neurology and Neurosurgery, London, UK, for assistance in the preparation of this report.
Guillain-Barré syndrome (GBS) is a rare, rapidly progressive disorder that consists of inflammation of the nerves (polyneuritis) causing muscle weakness, sometimes progressing to complete paralysis. Although the precise cause of GBS is unknown, a gastrointestinal or respiratory infection precedes the onset of the syndrome in about half of the cases. This has led to the theory that GBS may be an autoimmune disease (caused by the body's own immune system). Damage to the covering (myelin) of nerve axons (the extension of the nerve cell that conducts impulses away from the nerve cell body) results in delayed nerve signal transmission. This causes weakness of the muscles that are supplied by the damaged nerves.
The following variants of GBS are recognized acute inflammatory demyelinating polyradiculoneuropathy, acute motor sensory axonal neuropathy, and acute motor axonal neuropathy.
GBS is a rare, rapidly progressive disorder of the peripheral nerves, which are those outside the brain and spinal cord. This reflects inflammatory damage of motor nerves, the nerves that supply muscles and make them move. The paralysis usually begins in the feet and progresses upward to involve the trunk and arms; the breathing muscles and face can also be affected. In up to 30 percent of GBS patients, the breathing muscles become so weak that mechanical support of respiration on a breathing machine is temporarily required. Paralysis is usually accompanied by abnormal tingling or pins and needles sensations (paresthesias), numbness (loss of feeling) and pain. This reflects damage to the sensory nerves that convey feeling from the skin and joints to the spinal cord and brain. As with muscle weakness, abnormal sensations also often ascend from the feet upward, although they can start in the hands. Pain is not always present but when it is it may be severe. GBS symptoms usually progress over about two weeks but the progression may be as short as one day or as long as four weeks. Tendon reflexes such as the knee and ankle jerks are reduced and typically lost. Other findings in GBS may include weakness or paralysis of muscles in the head affecting face, lip and eye movements.
The symptoms of GBS are usually symmetrical (that is, they affect both sides of the body equally). The limbs (legs and arms) are typically affected early in GBS. In addition, the patient's internal, automatic body functions, controlled by autonomic nerves, can be affected, leading to internal organ malfunction. For example, weakness of internal muscles may lead the patient to experience difficulty breathing or swallowing. Vital functions may be affected, leading to an abnormally rapid or abnormally low heart rate, high blood pressure (hypertension), or a sudden drop in blood pressure upon arising from a bed or chair (postural hypotension). In severe cases the ability to pass urine may be lost so that a catheter has to be inserted to drain the bladder.
Symptoms experienced from GBS vary widely from patient to patient. Some patients only develop mild weakness not affecting walking. Others become completely paralyzed so that they cannot even move their eyes. Improvement typically starts days to several weeks after maximal paralysis has been reached and continues for several months. About 20% of patients still have disability, for instance needing aids to walk, for longer than a year. In such severely affected patients improvement continues for two years or more.
GBS is caused by damage to the peripheral nerves, those outside the brain and spinal cord. Usually the outer coating or insulation of the nerve, called myelin, is damaged. In many cases, the central conducting core of the nerve, the axon, is damaged as well. In some cases the axon is the primary target of the damage. The majority of research supports an autoimmune basis for the disorder. In an autoimmune disorder, the body's natural defenses, such as antibodies, that typically fight infection, attack healthy tissue, in this case the peripheral nerves. The reasons for this attack are being studied.
Several infections as well as other factors have been identified as preceding, and thus likely triggering, GBS. Infectious agents particularly implicated as triggers of GBS include the bacterium, Campylobacter jejuni (C. jejuni) and viruses such as cytomegalovirus and the glandular fever or Epstein-Barr virus. Infection with C. jejuni has been implicated as triggering two variants of GBS, acute motor axonal neuropathy, common in children during the summer in northern rural China but rare elsewhere, and Miller Fisher syndrome, characterized by double vision and a very wobbly gait but not paralysis. C. jejuni is a common cause of gastrointestinal illness and may be ingested with contaminated water, raw milk, and undercooked infected chicken.
GBS affects approximately one or two persons each year in every 100,000 population. All ages can be affected and it is slightly more common in the elderly than the young and in men than women.
Guillain-Barré syndrome is only one cause of acute peripheral neuropathy. Its diagnosis requires exclusion of other causes including neuropathy caused by alcohol, poisons, drugs, vasculitis (inflammation of blood vessels) and cancer. (For more information on these disorders, choose "Neuropathy, Peripheral" as your search term in the Rare Disease Database.)
Fisher Syndrome, described by Dr. CJ Miller Fisher, is related to GBS. It is characterized by 1) weakness of the eye muscles, leading to double vision, 2) loss of deep tendon reflexes such as knee and ankle jerks, and 3) unsteadiness of the limbs. It may overlap with GBS in some cases and partial variants with only weakness of eye movements, weakness of the face or unsteadiness also occur.
A longer drawn-out illness, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is also related to GBS. In this disease progression is much slower with the illness developing over at least 8 weeks. Weakness of the legs may become severe enough to limit walking, but breathing muscles are not usually affected. The disease may be gradually progressive over many months or years or may pursue a spontaneously remitting and relapsing course. By contrast GBS is usually a disease which occurs once and does not recur. Exceptions to this rule are rare, less than 5% of cases.
Several general supportive measures are used for the newly diagnosed GBS patient. Because of the risk of breathing failure and heartbeat instability, most new GBS patients with severe disease are placed where the heartbeat can be monitored such as in an intensive care unit. Supportive measures include mechanical ventilation, if breathing becomes severely impaired; methods to reduce the risk of deep vein thrombosis, bedsores and constipation; and comprehensive physical therapy to optimize muscle strength and function. When patients become medically stable, they are often transferred to a rehabilitation center for comprehensive physical and occupational therapy.
Two methods have been found to shorten the course and severity of GBS. Plasmapheresis, also called plasma exchange (PE), is a method of 'cleansing' the blood. Some of the patient's blood is removed and the blood cells are separated from the liquid portion of the blood. The cells are then mixed in a safe liquid (e.g. pooled plasma from healthy people) and transfused back into the patient. The other method is intravenous administration of high-dose immune globulin (IVIG) which utilizes immune globulin prepared from the purified pooled plasma of thousands of healthy people.
PE or IVIG are equally helpful. They need to be started within the first two weeks after the onset of GBS symptoms. They are usually prescribed only if the patient cannot walk unassisted. Their use shortens the time on the mechanical respirator as well as the time needed to recover sufficiently to walk unassisted. IVIG may be preferable to PE since it is less invasive and does not require special equipment.
The mechanisms underlying the benefits of these treatments are not well understood. PE may work, in part, by removing harmful antibodies from the patient's blood. IVIG may be helpful by loading up good antibodies on the patient's nerves and displacing harmful antibodies. Combinations of PE and IVIG are not more beneficial than either treatment alone.
Since GBS is generally accepted as an autoimmune disorder in which the nerves are inflamed, clinical researchers have explored the use of anti-inflammatory agents such as corticosteroids to treat GBS. Clinical trials have not shown significant benefit from corticosteroids.
As a generalization, younger patients tend to have a better outcome from GBS. Some patients experience a complete, or nearly complete, recovery but many have persistent fatigue for months or years afterwards. Up to 45 percent may have some mild, residual effects such as foot drop or abnormal sensation, that last more than two years from the onset of the disorder. Fewer than 15 percent have substantial long term disability requiring the use of a walker or wheelchair. Death from GBS is rare, occurring in fewer than 5 percent of patients.
Most variants of GBS are treated in a similar manner with IVIG or PE. The related condition, CIDP, is also treated with PE and IVIg but, unlike GBS, it also responds to corticosteroids.
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Report last updated: 2014/01/29 00:00:00 GMT+0