Guillain-Barré Syndrome

Guillain-Barré syndrome (GBS) is a rare neurological disorder in which the immune system mistakenly attacks part of the peripheral nervous system.

Overview

Guillain-Barré syndrome (GBS) is a rare neurological disorder in which a person’s immune system mistakenly attacks part of their peripheral nervous system. The peripheral nervous system is the network of nerves that carries signals from the brain and spinal cord to the rest of the body.

Guillain-Barré syndrome begins suddenly and can increase in intensity over a period of hours, days, or weeks until certain muscles cannot be used at all. Some cases are very mild and only cause brief weakness. Others cause severe paralysis, leaving the person unable to breathe on their own. In these cases, the disorder is life-threatening, potentially interfering with breathing, blood pressure, or heart rate.

Fortunately, most people eventually recover from even the most severe cases of Guillain-Barré syndrome. After recovery, people may continue to have some weakness.

Related Terms: Guillain-Barré syndrome goes by several alternative names and related terms:

  • Landry-Guillain-Barré syndrome
  • Acute idiopathic polyneuritis
  • Infectious polyneuritis
  • Acute inflammatory polyneuropathy
  • Ascending paralysis
  • Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
  • Acute motor axonal neuropathy (AMAN)
  • Acute motor-sensory axonal neuropathy (AMSAN)
  • Miller Fisher syndrome

Signs and Symptoms

The body’s nerves have a central part called the axon that carries an electric signal. The axon is surrounded by an insulating layer called the myelin sheath. The myelin sheath speeds up nerve signals and allows them to move over long distances. In some types of Guillain-Barré syndrome, the immune system damages the myelin sheath or the axons. As a result, the nerves can’t send signals efficiently and the muscles begin to lose their ability to respond to the brain’s commands, causing weakness.

Miller Fisher syndrome is another type of Guillain-Barré syndrome involving the cranial nerves, which extend from the brain to areas of the head and neck. Its main symptoms are weakness or paralysis of the muscles that move the eyes, problems with balance and coordination, and unusual or absent reflexes.

Weakness

The weakness seen in Guillain-Barré syndrome usually comes on quickly and worsens over hours or days. Often, people feel weakness in the feet first, and weakness may move up the body to eventually affect the legs, arms, face, and breathing muscles. People may first notice unexpected difficulty climbing stairs or walking. Less commonly, symptoms start in the face and move down to the legs and feet. Most people reach the greatest extent of weakness within the first two weeks after symptoms appear.

Sensation Changes

In Guillain-Barré syndrome, the brain may receive unusual sensory signals from the rest of the body due to nerve damage. This results in unexplained, spontaneous sensations called paresthesias, felt as tingling, a sense of insects crawling under the skin (called formications), and pain. Some people with Guillain-Barré syndrome feel a deep muscular pain in the back or legs.

Unexplained sensations often happen first, such as tingling in the feet or hands, or even pain, especially in children, often starting in the legs or back. Children may also begin to have difficulty walking and may refuse to walk. These sensations tend to disappear before the major, longer-term symptoms appear.

Other Symptoms

Other symptoms of Guillain-Barré syndrome may include:

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

Related Health Effects

Guillain-Barré syndrome can be a very difficult disorder because of its sudden and rapid start of weakness and often paralysis. In severe cases, the disorder is life-threatening, potentially limiting breathing, blood pressure, or heart rate.

Respiratory failure (little or no oxygen) can happen in Guillain-Barré syndrome. It can affect the autonomic nervous system which regulates the functions of internal organs and some muscles. This causes changes in heart rate, blood pressure, digestion, or sweating.

Recovery can be slow, anywhere from a few weeks to a few years. Some people don’t recover completely and experience long-term weakness, numbness, fatigue, or pain. People recovering from Guillain-Barré syndrome may face physical challenges and emotionally painful periods.

Medline plus has the following information that may be relevant: "Possible complications of GBS include:

  • Breathing difficulty (respiratory failure)
  • Shortening of tissues in the joints (contractures) or other deformities
  • Blood clots (deep vein thrombosis) that form when the person with GBS is inactive or has to stay in bed
  • Increased risk of infections
  • Low or unstable blood pressure
  • Paralysis that is permanent
  • Pneumonia
  • Skin damage (ulcers)
  • Breathing food or fluids into the lungs (aspiration)

Causes and Risk Factors

Guillain-Barré syndrome can affect anyone, but most often affects people older than 50. Guillain-Barré syndrome isn’t contagious or inherited (passed down in families), and the exact cause is unknown.

Because the body’s own immune system does the damage, Guillain-Barré syndrome is called an autoimmune disease (“auto” meaning “self”). Normally, the immune system uses antibodies and special white blood cells to protect the body by attacking infecting bacteria and viruses but in Guillain-Barré syndrome, the immune system mistakenly attacks healthy nerves. One possible reason is that some chemicals found on bacteria and viruses resemble those on nerve cells, so the immune system may not be able to tell them apart.

Guillain-Barré syndrome usually starts a few days or weeks following a bacterial or viral infection in the nose, throat, lungs, intestines, or stomach. Specific triggers may include:

  • Infection with the bacteria Campylobacter jejuni, a cause of gastroenteritis, that presents with nausea, vomiting, and diarrhea, is one of the most common risk triggers for Guillain-Barré syndrome
  • Surgery, or other trauma, can sometimes trigger the syndrome
  • Some countries report an increased incidence of GBS following infection with the COVID-19, Zika, cytomegalovirus, or Epstein-Barr viruses
  • In very rare cases, certain vaccinations may be associated with Guillain-Barré syndrome

Diagnosis

Guillain-Barré syndrome can be difficult to diagnose in its earliest stages because cases begin differently for different people, and several disorders have similar symptoms. Doctors may use the following tests:

  • History and physical exam: A doctor performs a physical exam and reviews the person’s medical history, seeing how muscles and nerves are working. The doctor notes whether symptoms appear on both sides of the body, which is common in Guillain-Barré syndrome, and how fast symptoms appeared. They also check reflexes, as some reflexes are lost in people with Guillain-Barré syndrome.
  • Nerve conduction velocity (NCV) test: This test measures the nerve’s ability to send a signal. In Guillain-Barré syndrome, signals traveling along damaged nerves are slowed because of damage to the myelin sheath.
  • Cerebrospinal fluid analysis: A doctor may analyze the cerebrospinal fluid that bathes and protects the spinal cord. This fluid contains more protein and fewer immune cells in people with GBS.
  • Imaging: In some cases, a magnetic resonance image (MRI) of the spinal cord or brain may help find other potential causes of muscle weakness.

Treatment and Management

Currently, there is no cure for Guillain-Barré syndrome. But some therapies can reduce its severity and shorten recovery time.

Hospital Care

People with Guillain-Barré syndrome are usually admitted to the hospital. Hospital staff closely monitor muscle weakness, breathing, heart rate, and blood pressure.

Acute (Short-Term) Treatments

Two treatments are commonly used to interrupt immune-related nerve damage. Both are equally effective if started within two weeks of Guillain-Barré syndrome symptoms:

  • Plasma exchange (PE), also called plasmapheresis, involves removing some blood through a catheter. Plasma, the liquid part of the blood, is separated from the blood cells and discarded, along with antibodies it contains. The blood cells are combined with replacement fluid and returned to the body. Plasma exchange is thought to work by removing the antibodies that are damaging the nerves.
  • Intravenous immunoglobulin therapy (IVIg) involves injections of immunoglobulins, proteins that the immune system naturally makes to attack infecting organisms. The immunoglobulins come from a pool of thousands of healthy donors. IVIg can lessen the immune attack on the nervous system and shorten recovery time. Researchers believe this treatment reduces the effectiveness of antibodies that attack the nerves by “diluting” them with non-specific antibodies and reducing the number of harmful antibodies.

In the past, doctors have tried using anti-inflammatory steroid hormones called corticosteroids to reduce the severity of Guillain-Barré syndrome, but recent research has shown this treatment isn’t effective.

Supportive Care

Supportive care is very important to address the many complications of nerve damage as the body recovers. Doctors will closely monitor a person’s breathing, and sometimes they will use a mechanical ventilator to help support or control breathing. Heart rate and blood pressure should also be carefully monitored. If the person has problems swallowing, they may need special care to prevent choking, which can cause pneumonia.

Rehabilitative Care

As people with severe Guillain-Barré syndrome begin to improve, they may be transferred from the hospital to a rehabilitation center to regain strength and start parts of their daily life again. Examples of rehabilitative approaches may include:

  • Physical therapy to manually move and position limbs, keep muscles flexible, and prevent muscle shortening
  • Targeted exercises to improve the strength of weaker muscles to regain their original function
  • Occupational and vocational therapy to help people learn new ways to handle everyday functions, manage work demands, and identify the need for assistive devices and adaptive equipment and technology

Living With Guillain-Barré syndrome

Guillain-Barré syndrome can severely affect people because of how quickly and unexpectedly weakness and paralysis start. The good news is that most people with Guillain-Barré syndrome have a full recovery. With careful intensive care and successful treatment, even people who have respiratory failure usually survive.

Recovery can be anywhere from a few weeks to a few years. Some people don’t recover completely and experience long-term weakness, numbness, fatigue, or pain. People recovering from Guillain-Barré syndrome may face physical challenges and emotionally painful periods.

It can be extremely difficult to adjust to sudden paralysis and dependence on others for help with routine, daily activities. Some people with Guillain-Barré syndrome need mental health counseling to help them adapt. Support groups can often help with emotional strain and provide valuable information.

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Research Information

The National Institutes of Health (NIH) is the largest public funder of biomedical research in the world. NIH invests most of its budget in medical research seeking to enhance life and to reduce illness and disability. NIH-funded research has led to breakthroughs and new treatments helping people live longer, healthier lives, and building the research foundation that drives discovery.

NIH is the nation’s leading federal funder of research on neurological disorders. NIH conducts research on Guillain-Barré syndrome and funds research on Guillain-Barré syndrome at major institutions and universities. Neuroscientists, immunologists, virologists, and pharmacologists are working collaboratively to learn how to prevent Guillain-Barré syndrome and to develop new and better therapies.

Current research areas include:

  • Identifying which immune cells are responsible for beginning and carrying out the attack on the nervous system in Guillain-Barré syndrome
  • Investigating what features of certain viruses and bacteria may activate the immune system inappropriately
  • Studying a treatment to stop the disruption of myelin junctions to prevent demyelination when the immune system damages the myelin sheath
  • Examining normal variations in certain genes that could increase the risk of developing Guillain-Barré syndrome
  • Using a mouse model with an altered autoimmune regulator gene to identify which peripheral nervous system (PNS) proteins are at greatest risk of autoimmune attack
  • Investigating the mechanisms by which IVIg treatment lessens the symptoms of Guillain-Barré syndrome to develop more effective treatments
  • Testing a new treatment that can reduce macrophage inflammation in the nerves of animal models

Find NIH-funded research projects using NIH RePORTER, a searchable database of current and past research projects supported by NIH and other federal agencies. 

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