Guillain-Barré facts

*Guillain-Barré facts Medically Edited by: Melissa Conrad Stöppler, MD

  • Guillain-Barré syndrome (GBS) occurs when the immune system attacks the peripheral nervous system.
  • Weakness or tingling in the legs or arms and upper body are characteristic symptoms of GBS.
  • Severe cases of Guillain-Barré cause paralysis and are life-threatening.
  • GBS is a very rare condition that afflicts about 1 out of every 100,000 people.
  • Guillain Barre syndrome can occur after a respiratory or gastrointestinal viral infection. Guillain-Barré syndrome also can appear after surgery or vaccines.
  • The autoimmune reaction in Guillain-Barré syndrome is directed against the myelin sheaths (surrounding tissue) of the peripheral nerves or the nerves themselves.
  • The signs and symptoms of Guillain-Barré can vary, so it can be hard to diagnose in the early stages.
  • A physical exam as well as an examination of the cerebrospinal fluid (CSF) obtained from a spinal tap may help with diagnosis.
  • Treatment of Guillain-Barré syndrome may include plasma exchange (plasmapheresis) and high-dose immunoglobulin therapy.
  • There is no cure for Guillain-Barre, but most people recover fully.
  • The recovery period after a bout of Guillain-Barré syndrome may be as little as a few weeks or as long as a few years.

Causes of Numb Toes

Numbness of the toes is often associated with tingling. This is referred to as paresthesia of the toes.

Numbness to the leg and foot also can be caused by irritation of nerves in the low back with:

  • Radiculopathy and herniated disk
  • Guillain-Barré syndrome
  • Frostbit
  • Stroke
  • Diabetic neuropathy...

What is Guillain-Barré syndrome?

Guillain-Barré syndrome (GBS) is a rare neurological disorder in which the body's immune system mistakenly attacks part of its peripheral nervous system—the network of nerves located outside of the brain and spinal cord. GBS can range from a very mild case with a brief weakness to nearly devastating paralysis, leaving the person unable to breathe independently. Fortunately, most people eventually recover from even the most severe cases of GBS.  After recovery, some people will continue to have some degree of weakness.

Guillain-Barré syndrome can affect anyone. It can strike at any age (although it is more frequent in adults and older people) and both sexes are equally prone to the disorder. GBS is estimated to affect about one person in 100,000 each year.

What are the symptoms of Guillain-Barré syndrome?

Unexplained sensations often occur first, such as tingling in the feet or hands, or even pain (especially in children), often starting in the legs or back. Children will also show symptoms with difficulty walking and may refuse to walk. These sensations tend to disappear before the major, longer-term symptoms appear.  Weakness on both sides of the body is the major symptom that prompts most people to seek medical attention. The weakness may first appear as difficulty climbing stairs or walking. Symptoms often affect the arms, breathing muscles, and even the face, reflecting more widespread nerve damage. Occasionally symptoms start in the upper body and move down to the legs and feet.

Most people reach the greatest stage of weakness within the first two weeks after symptoms appear; by the third week, 90 percent of affected individuals are at their weakest.

In addition to muscle weakness, symptoms may include:

  • Difficulty with eye muscles and vision
  • Difficulty swallowing, speaking or chewing
  • Pricking or pins and needles sensations in the hands and feet
  • Pain that can be severe, particularly at night
  • Coordination problems and unsteadiness
  • Abnormal heartbeat/rate or blood pressure
  • Problems with digestion and/or bladder control.

These symptoms can increase in intensity over a period of hours, days, or weeks until certain muscles cannot be used at all and, when severe, the person is almost totally paralyzed. In these cases, the disorder is life-threatening—potentially interfering with breathing and, at times, with blood pressure or heart rate.

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What causes Guillain-Barré syndrome?

The exact cause of GBS is not known. Researchers don’t know why it strikes some people and not others. It is not contagious or inherited.  

What they do know is that the affected person's immune system begins to attack the body itself. It is thought that, at least in some cases, this immune attack is initiated to fight infection and that some chemicals on infecting bacteria and viruses resemble those on nerve cells, which, in turn, also become targets of attack.  Since the body’s own immune system does the damage, GBS is called an autoimmune disease (“auto” meaning “self”). Normally the immune system uses antibodies (molecules produced in an immune response) and special white blood cells to protect us by attacking infecting microorganisms (bacteria and viruses). In Guillain-Barré syndrome, however, the immune system mistakenly attacks the healthy nerves.

Most cases usually start a few days or weeks following a respiratory or gastrointestinal viral infection. Occasionally surgery will trigger the syndrome. In rare cases, vaccinations may increase the risk of GBS. Recently, some countries worldwide reported an increased incidence of GBS following infection with the Zika virus.

How is Guillain-Barré syndrome diagnosed?

The initial signs and symptoms of GBS are varied and there are several disorders with similar symptoms.  Therefore, doctors may find it difficult to diagnose GBS in its earliest stages.

Physicians will note whether the symptoms appear on both sides of the body (the typical finding in Guillain-Barré syndrome) and the speed with which the symptoms appear (in other disorders, muscle weakness may progress over months rather than days or weeks). In GBS, deep tendon reflexes in the legs, such as knee jerks, are usually lost. Reflexes may also be absent in the arms. Because the signals traveling along the nerve are slow, a nerve conduction velocity test (NCV, which measures the nerve’s ability to send a signal) can provide clues to aid the diagnosis. There is a change in the cerebrospinal fluid that bathes the spinal cord and brain in people with GBS. Researchers have found the fluid contains more protein than usual but very few immune cells (measured by white blood cells). Therefore, a physician may decide to perform a spinal tap or lumbar puncture to obtain a sample of spinal fluid to analyze. In this procedure, a needle is inserted into the person’s lower back and a small amount of cerebrospinal fluid is withdrawn from the spinal cord. This procedure is usually safe, with rare complications.

Key diagnostic findings include:

  • Recent onset, within days to at most four weeks of symmetric weakness, usually starting in the legs
  • Abnormal sensations such as pain, numbness, and tingling in the feet that accompany or even occur before weakness
  • Absent or diminished deep tendon reflexes in weak limbs
  • Elevated cerebrospinal fluid protein without elevated cell count. This may take up to 10 days from the onset of symptoms to develop.
  • Abnormal nerve conduction velocity findings, such as slow signal conduction
  • Sometimes, a recent viral infection or diarrhea.

How is Guillain-Barré syndrome treated?

There is no known cure for Guillain-Barré syndrome. However, some therapies can lessen the severity of the illness and shorten recovery time. There are also several ways to treat the complications of the disease.

Because of possible complications of muscle weakness, problems that can affect any paralyzed person (such as pneumonia or bed sores), and the need for sophisticated medical equipment, individuals with Guillain-Barré syndrome are usually admitted and treated in a hospital’s intensive care unit.

Acute care

There are currently two treatments commonly used to interrupt immune-related nerve damage. One is plasma exchange (PE, also called plasmapheresis); the other is high-dose immunoglobulin therapy (IVIg). Both treatments are equally effective if started within two weeks of the onset of GBS symptoms, but immunoglobulin is easier to administer. Using both treatments in the same person has no proven benefit.

In the process of plasma exchange, a plastic tube called a catheter is inserted into the person’s veins, through which some blood is removed. The blood cells from the liquid part of the blood (plasma) are extracted and returned to the person. This technique seems to reduce the severity and duration of the Guillain-Barré episode. Plasma contains antibodies and PE removes some plasma; PE may work by removing the bad antibodies that have been damaging the nerves.

Immunoglobulins are proteins that the immune system naturally makes to attack infecting organisms. IVIg therapy involves intravenous injections of these immunoglobulins. The immunoglobulins are developed from a pool of thousands of normal donors. When IVIg is given to people with GBS, the result can be a lessening of the immune attack on the nervous system. The IVIg can also shorten recovery time. Investigators believe this treatment also lowers the levels of effectiveness of antibodies that attack the nerves by both “diluting” them with non-specific antibodies and providing antibodies that bind to the harmful antibodies and take them out of commission.

Miller-Fisher syndrome is also treated with plasmapheresis and IVIg.

Anti-inflammatory steroid hormones called corticosteroids have also been tried to reduce the severity of Guillain-Barré syndrome. However, controlled clinical trials have demonstrated that this treatment is not effective.

Supportive care is very important to address the many complications of paralysis as the body recovers and damaged nerves begin to heal. Respiratory failure can occur in GBS, so close monitoring of a person’s breathing should be instituted initially. Sometimes a mechanical ventilator is used to help support or control breathing. The autonomic nervous system (that regulates the functions of internal organs and some of the muscles in the body) can also be disturbed, causing changes in heart rate, blood pressure, toileting, or sweating. Therefore, the person should be put on a heart monitor or equipment that measures and tracks body function. Occasionally GBS-related nerve damage can lead to difficulty handling secretions in the mouth and throat. In addition to the person choking and/or drooling, secretions can fall into the airway and cause pneumonia.

Rehabilitative care

As individuals begin to improve, they are usually transferred from the acute care hospital to a rehabilitation setting. Here, they can regain strength, receive physical rehabilitation and other therapy to resume activities of daily living, and prepare to return to their pre-illness life. 

Complications in GBS can affect several parts of the body. Often, even before recovery begins, caregivers may use several methods to prevent or treat complications. For example, a therapist may be instructed to manually move and position the person’s limbs to help keep the muscles flexible and prevent muscle shortening. Injections of blood thinners can help prevent dangerous blood clots from forming in leg veins. Inflatable cuffs may also be placed around the legs to provide intermittent compression. All or any of these methods help prevent blood stagnation and sludging (the buildup of red blood cells in veins, which could lead to reduced blood flow) in the leg veins. Muscle strength may not return uniformly; some muscles that get stronger faster may tend to take over a function that weaker muscles normally perform—called substitution. The therapist should select specific exercises to improve the strength of the weaker muscles so their original function can be regained.

Occupational and vocational therapy help individuals learn new ways to handle everyday functions that may be affected by the disease, as well as work demands and the need for assistive devices and other adaptive equipment and technology.

IMAGES

Guillain-Barre Syndrome See brain scan pictures of Alzheimer's and types of dementia See Images

What is the long-term outlook for those with Guillain-Barré syndrome?

Guillain-Barré syndrome can be a devastating disorder because of its sudden and rapid, unexpected onset of weakness—and usually actual paralysis. Fortunately, 70% of people with GBS eventually experience a full recovery. With careful intensive care and successful treatment of infection, autonomic dysfunction, and other medical complications, even those individuals with respiratory failure usually survive.

Typically, the point of greatest weakness occurs days to at most 4 weeks after the first symptoms occur. Symptoms then stabilize at this level for a period of days, weeks, or, sometimes months. Recovery, however, can be slow or incomplete. The recovery period may be as little as a few weeks up to a few years. Some individuals still report ongoing improvement after 2 years. About 30 percent of those with Guillain-Barré have a residual weakness after 3 years. About 3 percent may suffer a relapse of muscle weakness and tingling sensations many years after the initial attack. About 15 percent of individuals experience long-term weakness; some may require ongoing use of a walker, wheelchair, or ankle support. Muscle strength may not return uniformly.

Ongoing fatigue, pain, and other annoying sensations can sometimes be troublesome. Fatigue is best handled by pacing activities and providing time for rest when fatigue sets in. Those with Guillain-Barré syndrome face not only physical difficulties, but emotionally painful periods as well. It is often extremely difficult for individuals to adjust to sudden paralysis and dependence on others for help with routine daily activities. Individuals sometimes need psychological counseling to help them adapt. Support groups can often ease the emotional strain and provide valuable information.

What research is being done on Guillain-Barré syndrome?

The mission of the National Institute of Neurological Disorders and Stroke (NINDS) is to seek fundamental knowledge of the brain and nervous system and to use that knowledge to reduce the burden of neurological diseases. The NINDS conducts research on disorders including Guillain-Barré syndrome and funds research at major institutions and universities. The NINDS is a component of the National Institutes of Health (NIH), the leading supporter of biomedical research in the world.

Neuroscientists, immunologists, virologists, and pharmacologists are working collaboratively to learn how to prevent GBS and to make better therapies available when it strikes.

Scientists are concentrating on finding new treatments and refining existing ones. Scientists are also looking at the workings of the immune system to find which cells are responsible for beginning and carrying out the attack on the nervous system. The fact that many cases of Guillain-Barré syndrome begin after a viral or bacterial infection suggests that certain characteristics of some viruses and bacteria may activate the immune system inappropriately. Investigators are searching for those characteristics. Certain proteins or peptides in viruses and bacteria may be the same as those found in myelin, and the production of antibodies to neutralize the invading viruses or bacteria could trigger the attack on the myelin sheath.

Some studies show that normal variations in certain genes could increase the risk of developing Guillain-Barré syndrome; however, more research is necessary to identify and confirm associated genes. Since many of the genes that may increase the risk of Guillain-Barré syndrome are involved in the immune system, their roles in fighting infection might contribute to the development of the condition.           

NINDS-funded researchers have developed a mouse model with an altered autoimmune regulator gene that generates autoimmunity against the peripheral nervous system (PNS). Using this model, scientists hope to identify which PNS proteins are at the greatest risk of autoimmune attack and which components of the immune system contribute to the autoimmune response against the PNS. A greater understanding of how the immune system damages the PNS could lead to better treatments for autoimmune disorders such as GBS.    

Other NINDS-funded researchers are investigating the mechanisms by which IVIg treatment lessens the symptoms of GBS. By understanding these mechanisms, it may be possible to develop more effective treatments.

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Where can I get more information about Guillain-Barré syndrome?

For more information on neurological disorders or research programs funded by the National Institute of Neurological Disorders and Stroke, contact the Institute's Brain Resources and Information Network (BRAIN) at:

BRAIN
P.O. Box 5801
Bethesda, MD 20824
(800) 352-9424
http://www.ninds.nih.gov

Information also is available from the following organizations:

GBS/CIDP Foundation International
375 East Elm Street
Conshohocken, PA 19428
610-667-0131
866-224-3301

National Library of Medicine
8600 Rockville Pike
Bethesda, MD 20894
301-594-5983

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Medically Reviewed on 6/30/2019
References
SOURCE:

National Institute of Neurological Disorders and Stroke. Last update: June 2018.
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