Guillain Barré Syndrome
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.
Synonyms of Guillain Barré Syndrome
- acute autoimmune peripheral neuropathy
- acute immune-mediated polyneuropathy
- acute inflammatory demyelinating polyneuropathy
- acute inflammatory demyelinating polyradiculoneuropathy
- acute inflammatory neuropathy
- acute inflammatory polyneuropathy
- Landry-Guillain-Barre-Strohl syndrome
- Landry's ascending paralysis
- post-infective polyneuritis
- acute motor axonal neuropathy
- acute motor neuropathy with conduction block
- acute motor-sensory axonal neuropathy
- chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
- Miller-Fisher syndrome
- multifocal motor neuropathy
- multifocal motor sensory demyelinating neuropathy
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 viral 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 (acute inflammatory neuropathy or acute inflammatory demyelinating polyradiculoneuropathy) are recognized: Miller Fisher syndrome, acute motor-sensory axonal neuropathy, acute motor axonal neuropathy.
GBS is a rare, rapidly progressive disorder of the peripheral nerves, those outside the brain and spinal cord. It is usually characterized by ascending (moving upward) paralysis. 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 sensations (paresthesias), such as numbness, tingling, pins and needles, and pain. This reflects damage to sensory nerves that extend from the limbs and skin to the brain and register our surroundings. 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 days up to weeks. Tendon reflexes such as knee and ankle jerks are typically lost. Other findings in GBS may include weakness or paralysis of the head muscles (face, lips and eye movements). Increased spinal fluid protein is also typical of GBS.
The symptoms of GBS usually are 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 heart beat (sinus tachycardia) that may be felt as palpitations, abnormally low heart rate (bradycardia), high blood pressure (hypertension), or a sudden drop in blood pressure upon arising from a bed or chair (postural hypotension). Other symptoms may include changes in body temperature, vision, bladder function and blood chemistry.
Symptoms experienced from GBS vary widely from patient to patient. An occasional patient may only develop mild weakness not affecting walking. At the other extreme, a patient may become completely paralyzed, so even the eyes cannot be opened. Maximum weakness or paralysis is often reached about ten days from the onset of symptoms, but can take up to a month to develop. If paralysis progresses over a longer time, another diagnosis is likely. Recovery typically starts days to several weeks after maximal paralysis has been reached takes about six months or sometimes more. Improvement can continue for two years or more.
GBS consists of 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 or sometimes on its own. 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. 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 implicated as triggers of GBS include the bacteria, Campylobacter jejuni (C. jejuni), Mycoplasma pneumoniae, viruses such as cytomegalovirus, and Epstein-Barr virus, and the swine flu injection of 1976.
Infection with C. jejuni has been implicated as triggering two variants of GBS, acute axonal motor 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 rather than paralysis. C. jejuni is a common cause of gastrointestinal illness and may be ingested with contaminated water, raw milk, and some poultry, especially inadequately cleaned or cooked chicken. Some cases of C. jejuni-triggered GBS have had more prolonged courses and more severe symptoms.
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 and in men than women.
Symptoms of the following disorders can be similar to those of Guillain-Barré Syndrome. Comparisons may be useful for a differential diagnosis:
Many other disorders of the peripheral nerves called peripheral neuropathies, especially those that affect the limbs, These include peripheral neuropathies associated with metabolic disorders, such as diabetes, vitamin B12 or thyroid gland; , alcohol overuse, cancer, and infectious agents, such as Lyme disease (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 a rare subtype or variant of GBS that may follow an upper respiratory tract infection. It is characterized by three major features, 1) weakness of the eye muscles, leading to double vision, 2) loss of deep tendon reflexes such as knee and ankle jerks, and 3) an awkward, unsteady gait (ataxia). Variants may occur in which double vision and abnormal gait predominate or weakness elsewhere, such as muscles of the face and neck, leading to impaired speech and sagging of the face. (For more information on this disorder, choose "Fisher" as your search term in the Rare Disease Database.)
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP is related to GBS. The paralysis of GBS occurs rapidly, not more than 28 days. In contrast, the weakness of CIDP develops slowly, over at least 8 weeks. Weakness of the legs may become severe enough to limit walking, but muscles of respiration are rarely affected enough to require mechanical ventilation. CIDP patients may have a slow recovery, over months, and the disorder may sometimes recur. GBS rarely recurs.
The debilitating effects of GBS can vary greatly. Maximal weakness is usually reached within one to four weeks from the onset of symptoms. After a plateau of days, weeks, or longer, recovery begins.
Muscle strength is first regained in the upper body and then descends. Improvement from GBS can extend over a period of six months to two years, and sometimes longer. The patient tends to regain strength more rapidly early in the recovery phase of the disorder and more slowly later. Patients whose recovery is rapid are less likely to experience long-term disabilities.
General supportive measures for GBS are those used for any paralyzed patient. Some interventions, immune globulin and plasma exchange, are used specifically to try to shorten the severity and duration of the disorder, especially ventilator dependency and paralysis.
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 phlebitis, 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 in many cases. 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 a pooled collection of gamma globulin from healthy people.
PE or IVIG may be helpful if started within the first two weeks of the onset of GBS symptoms. They are usually prescribed only if the patient cannot walk unassisted. Their use appears to shorten 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 have not been found to be 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 the 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 sensations, 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 is the related condition, CIDP, is also treated with PE and IVIg but, unlike GBS, it also responds to corticosteroids.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.
For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
Organizations related to Guillain Barré Syndrome
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