Guillain-Barré syndrome is a rare but serious autoimmune disease of the peripheral nervous system. It can lead to weakness and paralysis that may last for months or years.
The condition frequently follows a mild viral infection that resembles flu or gastroenteritis, and some cases of Guillan-Barré syndrome (GBS) occur after a bacterial infection. Symptoms start within a few days or weeks after the infection.
The condition affects around 1 in 100,000 people in the United States (U.S.).
GBS can affect people of any age or either sex, although it is slightly more common in older people and males. The condition usually begins following an infectious disease.
This article will cover the symptoms, causes, diagnosis, and treatment of GBS. Medical News Today will also investigate connections between this condition, the Zika virus, and vaccinations.
Fast facts on Guillain-Barré syndrome:
Here are some key points about Guillain-Barré syndrome. More detail and supporting information is in the main article.
- Guillain-Barré syndrome (GBS) is an autoimmune disease.
- The first symptoms of GBS are usually tingling and muscle weakness that begins in the lower extremities. The entire body can eventually become paralyzed.
- The exact causes are still unknown.
- Once they start to occur, the symptoms of GBS tend to develop very rapidly, over a small number of days, usually causing the highest levels of weakness within the first 2 to 3 weeks of symptom onset.
- The onset often follows an infection.
- Most people fully recover within 12 months, but full recovery may take up to 3 years in some cases.
GBS is a rare but serious autoimmune disorder that can affect any part of the nervous system outside of the brain and spinal cord. This is known as the peripheral nervous system.
An autoimmune disease involves the immune system attacking and destroying certain groups of healthy cells. In the case of GBS, the immune system attacks the myelin sheaths of peripheral nerves.
The myelin sheaths are the coatings on the axons of nerves, and myelin is essential for the speedy carrying of axonal nerve impulses. Axons are the long, thin extensions of nerve cells. In some cases, these are also attacked.
As the myelin is damaged, nerves can no longer send certain information to the spinal cord and brain, such as touch sensations. This causes the sensation of numbness. In addition, the brain and spinal cord are no longer able to transmit signals back to the body, leading to muscle weakness.
The disease often begins with tingling sensations and weakness in the feet and legs. It then slowly spreads upward until a large portion of the body is affected. The nerves connected to the lower extremities are the longest in the body. This travel distance makes these nerves more prone to a break in nerve signals due to GBS and its symptoms.
This condition is considered a medical emergency, and an individual should receive medical attention as soon as possible.
Initially, GBS was considered to be a single condition. Now, it is thought to take a number of forms. The three most common types of GBS are as follows:
- Acute inflammatory demyelinating polyradiculoneuropathy (AIDP): This is the most common type in the U.S. Typically, the weakness begins in the lower part of the body and gradually ascends to the other body parts.
- Miller Fisher syndrome (MFS): MFS occurs in around 5 to 10 percent of GBS cases in the U.S. However, this form of GBS is more prevalent in Asia. Paralysis begins with the eyes, and problems with walking are common.
- Acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN): These versions of the condition are rare in the U.S. but more common in Japan, China, and Mexico.
The symptoms of GBS typically begin with tingling and weakness in the feet and legs that gradually spreads to the rest of the body.
Around 1 in 10 people with GBS report tingling sensations starting in their face or neck. The weakness gradually gets worse and becomes paralysis.
Symptoms and other complications include:
- weakness in the lower body, moving upward
- general instability when walking
- less control over facial muscles during activities such as chewing or talking
- cramp-like pain that gets worse at night
- lack of control over the bowel or bladder
- pain, with around 50 percent of people with GBS experiencing severe nerve pain that may need drug management
- a faster heart rate than normal
- high or low blood pressure
- blood clots
- pressure sores if a person is immobile for a considerable length of time
- difficulty breathing
- a future relapse of the condition in 3 percent of people with GBS
- psychological and cognitive difficulties
Contact a healthcare professional immediately if these symptoms occur.
GBS can also cause emotional symptoms, as adjusting to rapid paralysis and requiring assistance from others for daily tasks can be distressing.
The exact causes of GBS are still not known.
The condition often develops a few days or weeks after an infection of the digestive or respiratory tract. This suggests that they could be related to GBS. In rarer cases, the syndrome can occur following surgery, other infections, or immunization.
Some physicians believe that viral and bacterial infections may change the how the immune system reacts to the peripheral nerves, possibly causing the myelin and underlying axon not to be recognized as body tissue. This would make them a target for immune response.
There are some known risk factors, including:
- Sex: Males are slightly more likely to contract GBS.
- Age: Risk increases with age.
- Campylobacter jejuni bacterial infection: A common cause of food poisoning, this infection sometimes occurs before GBS.
- Influenza virus, HIV, or Epstein-Barr virus (EBV): These have occurred in association with cases of GBS.
- Mycoplasma pneumonia: This is a bacterial infection of the lungs.
- Surgery: Some surgeries can trigger GBS.
- Hodgkin’s lymphoma: Cancer of the lymphatic system can lead to GBS.
- Influenza vaccination or childhood vaccinations: These have also been linked to GBS in rare cases.
Is the Zika virus linked?
Recent findings reported in The Lancet suggest that the Zika virus might also cause GBS.
Zika virus has previously been associated with GBS. The link was first described in 2013 and 2014 when the incidence of GBS showed a significant increase over 4 to 5 years during a Zika outbreak in the French Polynesian islands.
This was the first evidence of such a link, and more research is required. However, there have been a few studies looking at the in the incidence of GBS in French Polynesia and Latin America after recent Zika outbreaks.
Zika has been associated with mild flu-like symptoms in most people that contract the disease, similar to many of the infections that appear before GBS.
In 1976, there was a small increase in the risk of contracting GBS following a swine flu virus vaccination.
However, the increase was by just one additional case per 100,000. Research has also shown that individuals are more at risk of developing the condition after a case of the flu than they are from the vaccination.
According to the Centers for Disease Control and Prevention (CDC):
“It is important to keep in mind that severe illness and death are associated with influenza, and vaccination is the best way to prevent influenza infection and its complications.”
It is generally regarded as safe to continue receiving flu vaccinations.
GBS can be difficult to diagnose, especially in its earlier phases. The symptoms vary between people, and they are similar to the symptoms of other neurological conditions.
Initially, the doctor will check if the weakness occurs on both sides of the body, as this is a typical a feature of GBS. The rapid onset is another indicator. Many neurological conditions are much slower than GBS.
After a physical exam, a physician may recommend the following tests:
- Nerve conduction exam: Electrodes are taped to the skin, and the speed of nerve signal conduction is tested by passing small shocks along the nerves through the skin. In GBS, the signals travel more slowly along the nerves.
- Electromyography (EMG): Thin, needle-like electrodes are used to test the nerve function within muscle fibers.
- Spinal tap, also known as a lumbar puncture: A sample of cerebrospinal fluid (CSF) is removed from the spinal canal and tested in a laboratory for specific signs of the disease. More protein is present in the CSF of people with GBS.
There is currently no cure for GBS, but medications are available to ease symptoms.
There are two types of treatment that can reduce the severity of symptoms and improve the rate of recovery:
- Immunoglobulin therapy: Antibodies from donors are given intravenously (IV). This treatment seems to reduce the autoimmune response that occurs.
- Plasma exchange, also known as plasmapheresis: Blood is taken from the body. The blood plasma is then separated from the blood cells. The blood cells are returned, and the body regenerates plasma. This process removes some of the antibodies attacking healthy cells.
Both methods are similarly effective. However, using them together does not improve outcomes. Researchers do not yet know exactly why either method works.
In general, for most patients who have GBS, the nerve damage worsens quickly for around a couple of weeks and stops deteriorating by around 4 weeks. The average recovery time is 6 to 12 months.
Recovery can be very slow. It can also demand a lot of support, physiotherapy, counseling, and occupational therapy.
A few patients may be left with long-term disability, and about 30 percent of people with GBS still experience some weakness after three years. Patients recover at different rates, and some experience incomplete or delayed recovery.
One in 20 cases of GBS is fatal. However, the majority of patients make a full recovery.