June 01, 2020
Years published: 1985, 1987, 1988, 1990, 1992, 1996, 1997, 1998, 2002, 2003, 2004, 2007, 2008, 2011, 2014, 2017, 2020
NORD gratefully acknowledges Richard Hughes MD, MRC Centre for Neuromuscular Disease, UCL Queen Square Institute of Neurology, London, UK for assistance in the preparation of this report.
Guillain-Barré syndrome (GBS) is a rare, rapidly progressive disease due to inflammation of the nerves (polyneuritis) causing muscle weakness, sometimes progressing to complete paralysis. GBS affects about one or two people each year in every 100,000 population. Its exact cause is unknown. About half the people with the disease have a gastrointestinal or respiratory infection a few days before the onset. According to strong evidence, the infection produces an immune response which damages the nerve fibers causing weakness and loss of sensation. In milder disease the damage only affects the sheaths of the nerve fibers (like the coating round an electric wire). This blocks the transmission of nerve impulses. It can recover completely in a few weeks. In more severe disease, the immune response damages the conducting cores of the nerve fibers (that is the electric wires themselves). This takes longer to get better and may cause permanent weakness.
Weakness usually begins in the legs and spreads to the arms and body. Sometimes it spreads to the breathing muscles, face and throat. In up to a quarter of people with GBS, the breathing muscles become so weak as to need temporary support of respiration on a breathing machine. Feelings of tingling, pins and needles, and numbness (loss of feeling) are common. These are due to damage to the sensory nerves that signal feeling from the skin and joints. The abnormal feelings are usually worst in the feet and hands. The tingling may be painful and the muscles may also hurt. The symptoms usually worsen for the first two weeks but the progression may last as little as one day or as long as four weeks. Symptoms usually affect both sides of the body equally.
Most people only have symptoms in their arms and legs but severely affected people have more serious symptoms. They may have difficulty swallowing and become breathless. They may become unable to empty their bladder and bowel. Their pulse may go too fast or too slow and the blood pressure may rise too high or fall too low. Modern medical and nursing care can cope with all these problems as explained below.
Symptoms vary widely. Some people only develop mild weakness not affecting walking and lasting only a few weeks. Others become completely paralyzed so that they cannot even move their eyes. Improvement typically starts days to several weeks after the worst has been reached and people go on improving for several months. About 20% of patients still have disability for more than a year, for instance needing aids to walk. In people left with severe weakness, slow improvement continues for two or more years.
The cause of GBS is inflammation of the peripheral nerves. These nerves normally take messages to and from the skin and muscles to the brain and spinal cord. There is strong evidence that the cause is autoimmune. The immune system produces an immune response to an infection which cross-reacts with the nerves. It usually reacts with and damages the outer coating sheath of the nerve fibers, called myelin. In more severely affected people, this damage also affects the central conducting core of the nerve, called the axon. In some people the axon is itself the main target of the autoimmune response.
There are three different forms of GBS:
The different forms have similar symptoms, signs and disease courses except for the absence of sensory symptoms and signs in acute motor axonal neuropathy. They are distinguished by nerve conduction tests (see below). The treatments used and outcomes are also the same.
The infections which trigger the autoimmune response include:
Less, usually much less, than 1 in a 1,000 of people who catch these infections get GBS.
Guillain-Barré syndrome is only one of many causes of acute weakness and numbness. The diagnosis requires recognition of the characteristic symptoms and signs. Because the disease is rare, diagnosis may be difficult for the non-specialist and referral to a neurologist is usually appropriate. Clinical examination shows loss of the tendon reflexes and supports the diagnosis of peripheral nerve disease. Two tests are commonly used to support the diagnosis:
Other tests exclude many other causes of neuropathy such as alcohol, poisons, drugs, vasculitis (inflammation of blood vessels), vitamin deficiency and cancer. Blood tests and X-rays or scans may also be done.
The most important parts of treatment are general medical and nursing care, physiotherapy, and rehabilitation. Because of the risks of breathing failure and heart-beat instability in the acute stage, people with severe disease need to be in a ward with facilities for monitoring the pulse and breathing. If breathing becomes difficult, mechanical ventilation with a breathing machine in an intensive care unit becomes necessary. For this, a special plastic tube, called an endotracheal tube, connects the person to the breathing machine via the mouth or nose. If mechanical ventilation lasts more than a few days, it is more comfortable to put the tube in the throat by making a small opening in the windpipe for the purpose (an operation called a tracheostomy). After recovery, the tube is removed and the opening gradually closes on its own. Difficulty swallowing requires insertion of a thin plastic tube through the nose into the stomach for feeding and drinking. Medicines and nursing measures treat pain and reduce the risk of veins clotting, bed sores and constipation. Physical therapy helps muscle strength and function and prevents muscle shortening and joint stiffness. When people become medically stable, they often move to a rehabilitation center for physical and occupational therapy. Psychological support is important throughout the illness.
Two treatments accelerate recovery from GBS, plasma exchange (PE, also called plasmapheresis) and intravenous immune globulin (IVIg). PE connects one vein via a thin plastic tube to a machine which separates the plasma (the liquid portion of the blood) from the red blood cells and returns the red blood cells with a plasma substitute via another vein. It removes harmful substances, especially the antibodies which cause GBS. IVIg consists of giving high doses of immune globulin (the antibodies in the blood) into a vein. The immune globulin comes from highly purified pooled plasma from thousands of healthy people. It probably works by blocking the effects of the harmful antibodies which cause GBS. IVIg is more convenient and more widely available than PE but both are equally helpful. Combining the two does not help more. Early treatment within the first two weeks after the onset of GBS symptoms is preferable. No other treatments hasten recovery. Against expectations, steroids have not been effective in clinical trials.
The majority of people recover completely or nearly completely. However, some have mild residual effects such as foot drop or abnormal feeling in the feet and hands for two years or more. Persistent fatigue and pain may be problematic. Fewer than 15 percent have substantial long-term disability severe enough to need a cane, walker or wheelchair. Death from GBS does occur but in fewer than 5 percent of patients and is rare in countries with intensive care facilities. Recurrence is rare.
Most variants of GBS are treated in a similar manner with IVIg or PE. The related condition, chronic inflammatory demyelinating polyradiculoneuropathy (https://rarediseases.org/rare-diseases/chronic-inflammatory-demyelinating-polyneuropathy/), is also treated with PE and IVIg but, unlike GBS, it also responds to corticosteroids.
The International GBS Outcome Study (IGOS) seeks to increase understanding of GBS, its causes, mechanisms and treat mechanisms and treatment is ongoing https://gbsstudies.erasmusmc.nl/. Ongoing research is looking for better drugs to reduce damage to the nerves in the early stages of the disease.
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 web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Some current clinical trials also are posted on the following page on the NORD website: https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact: www.centerwatch.com
For information about clinical trials conducted in Europe, contact: https://www.clinicaltrialsregister.eu/
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