Guillain-Barre Syndrome

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What is Guillain-Barre syndrome?

Guillain-Barre (gee-YAH-buh-RAY) syndrome (GBS) is a rare autoimmune disorder involving nerves that control movements and bodily functions (peripheral nerves). Weakness and tingling in feet, legs and arms are usually the first symptoms. The disease can progress quickly and may eventually paralyze the whole body. In severe cases it is a medical emergency requiring intensive care hospitalization. A severe case of GBS can be fatal without immediate medical attention. Mild cases may only cause brief weakness. 

While the cause is unknown, GBS is often linked to an infection before symptoms begin. Several treatments can ease symptoms and shorten the illness, but there is no known cure for GBS. It can affect people of all ages but is more common after age 50, and slightly more common in men than in women. Most people with GBS (60%-80%) are able to walk again after about six months. However, there can be after effects including weakness, numbness and fatigue. The GBS death rate is 4%-7%. 

There are several types of GBS including:

  • Acute inflammatory demyelinating polyradiculoneuropathy (AIDP), the most common form in North America and Europe, starts as muscle weakness in the lower part of the body that spreads upward.
  • Miller Fisher syndrome (MFS) starts with paralysis in the eyes and an unsteady gait. This type is more common in Asia.
  • Acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN) are less common in the United States; most cases are in China, Japan and Mexico.

Causes

What causes Guillain-Barre syndrome?

The cause of GBS is unknown. However, about 65% of GBS patients have an infection in the days to six weeks before symptoms begin, including respiratory or digestive tract infections, or Zika or COVID-19 viruses. Although rare, a recent surgery or vaccination can trigger it. GBS is not contagious nor is it inherited. However, if a group of people ingest the same campylobacter bacteria, it can cause a localized outbreak of GBS.

The most common type in the United States, AIDP, causes the immune system to attack the nerves, resulting in damage to the protective covering around nerves (myelin sheath). This damage prevents nerves from sending messages to the brain, which can cause weakness, numbness or paralysis. 

GBS may be triggered by:

  • Campylobacter infection, a bacterium often found in undercooked poultry, and causes about 40% of cases in the United States
  • Influenza virus
  • Cytomegalovirus
  • Epstein-Barr virus
  • Zika virus
  • Hepatitis A, B, C and E
  • HIV infection
  • Mycoplasma pneumonia
  • Surgery
  • Trauma
  • Hodgkin's lymphoma

Although very rare, GBS can be triggered by the COVID-19 virus, or vaccinations for influenza or childhood diseases. It’s also been reported as a rare reaction to the COVID-19 Johnson & Johnson vaccine.

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Symptoms

What are the symptoms of Guillain-Barre syndrome?

Symptoms of GBS may include:

  • Prickling, or pins and needles sensations in fingers, toes, ankles or wrists
  • Weakness on both sides of the body, and tingling in feet and legs that spreads to your upper body and arms; this symptom is worse during the two weeks after symptoms begin
  • Unsteady walking or inability to walk or climb stairs
  • Difficulty with facial movements, including speaking, chewing or swallowing
  • Double vision or inability to move eyes
  • Severe pain that may feel achy, shooting or cramping; may be worse at night
  • Digestive problems
  • Sluggish bowel function; problems with urine control
  • Rapid heart rate and or irregular heart beat
  • Abnormal (low or high) blood pressure
  • Blood clots if immobilized by paralysis
  • Difficulty breathing if the weakness/paralysis spreads to the muscles that control breathing (potentially fatal); shortness of breath when lying down
  • Choking on saliva

Diagnosis

How is Guillain-Barre syndrome diagnosed?

GBS is difficult to diagnose in its earliest stages because its symptoms are like other nerve disorders. Symptoms can also vary among patients. Unlike several other look-alike neurological disorders, GBS symptoms progress quickly and affect both sides of the body. Your doctor will take your medical history, do a physical exam and ask about your symptoms. Other tests may be needed including:

  • Spinal tap (lumbar puncture) withdraws a small amount of fluid from your spinal canal for testing.
  • Electromyography uses thin-needle electrodes inserted into specific muscles to measure their nerve activity.
  • Nerve conduction study uses electrodes taped to the skin above the nerve. A small shock is passed through the nerve to measure the speed of nerve signals.

Treatment

How is Guillain-Barre syndrome treated?

While there’s no cure for GBS, the sooner treatment is started, the better the chance of reducing its severity and speeding recovery. Treatment should be started within two weeks from when symptoms begin. Two, equally effective treatments are available:

  • Plasma exchange (plasmapheresis) is a process where blood plasma (liquid part of blood) is removed and separated from your blood cells. The blood cells are returned to your body to make more plasma. This treatment removes certain antibodies from your plasma. These are the antibodies that allow the immune system to attack your nerves.
  • Immunoglobulin therapy uses high doses of immunoglobulin containing healthy antibodies from blood donors. Given through a vein, the donor immunoglobulin can block the damaging antibodies, which are believed to trigger GBS. This therapy is easier to deliver, compared to plasmapheresis.

In addition to one of these treatments, you will also receive medications to relieve severe pain and prevent blood clots (if you’re immobile). Physical therapy will be prescribed to keep muscles flexible and strong, and help you regain strength. If needed, training to help you use adaptive devices (wheelchair or braces) will keep you mobile and able to care for yourself. 

How long will it take to recover?

Recovery times vary with some people taking months or years to fully recover. Children can recover more completely than adults. Most adults follow this general timeframe to recovery:

  • Symptoms worsen for about two weeks, reaching their maximum severity within four weeks. 
  • Recovery begins about four weeks after the beginning of symptoms.
  • Recovery phase lasts six to 12 months, although rarely, it can last three years.
  • At six months, about 80% of patients can walk independently.
  • One year after diagnosis, about 70% fully recover motor strength.
  • About 30% of patients have some weakness after three years.
  • About 15% have a very delayed and/or incomplete recovery and long-term weakness.
  • About 3% have a relapse of muscle weakness and tingling sensations many years after the initial attack.

References

Mayo Clinic. (July 2021). Guillain-Barre syndrome. Retrieved 11-24-21, {https://www.mayoclinic.org/diseases-conditions/guillain-barre-syndrome/symptoms-causes/syc-20362793}
Mayo Clinic. (July 2021). Guillain-Barre syndrome, Diagnosis and Treatment. Retrieved 11-24-21, {https://www.mayoclinic.org/diseases-conditions/guillain-barre-syndrome/diagnosis-treatment/drc-20363006}
Centers for Disease Control and Prevention. (Dec. 2019). Guillain-Barre Syndrome. Retrieved 11-29-21, {https://www.cdc.gov/campylobacter/guillain-barre.html}
National Institute of Neurological Disorders and Stroke. (Nov. 2021). Guillain-Barre Syndrome Fact Sheet. Retrieved 11-29-21, {https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Guillain-Barr%C3%A9-Syndrome-Fact-Sheet}

Information

Medically reviewed by:

Dr. Desiree Levyim

Dr. Desiree Levyim is a board eligible neurologist in practice since 2020. She joins TeleMed2U in our mission to provide increased access to healthcare.

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