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December 26, 2017

Guillain–Barré Syndrome - Causes, Symptoms, Treatment and Key Facts

In Guillain-Barré syndrome, the body's immune system attacks part of the peripheral nervous system. The syndrome can affect the nerves that control muscle movement as well as those that transmit feelings of pain, temperature and touch. This can result in muscle weakness and loss of sensation in the legs and/or arms.

It is a rare condition with an overall incidence of 0.4 to 4.0 people per 100 000 per year. People of all ages can be affected, but it is more common in adults and in males. Even in the best of settings, 3%-5% of GBS patients die from complications, which can include paralysis of the muscles that control breathing, blood infection, lung clots or cardiac arrest.

Symptoms

Symptoms typically last a few weeks, with most individuals recovering without long-term, severe neurological complications.
  • The first symptoms of Guillain-Barré syndrome include weakness or tingling sensations. They usually start in the legs, and can spread to the arms and face.
  • For some people, these symptoms can lead to paralysis of the legs, arms, or muscles in the face. In 20%-25% of people, the chest muscles are affected, making it hard to breathe.
  • Severe cases of Guillain-Barré syndrome are rare, but can result in near-total paralysis. These cases are considered life-threatening, and affected individuals are typically treated in intensive-care units.
  • Most people recover fully from even the most severe cases of Guillain-Barré syndrome, although some continue to experience weakness.

Causes

The cause of Guillain-Barré cannot always be determined, but it is often triggered by a bacterial or viral infection (such as HIV, dengue, or influenza) and less commonly by immunization, surgery, or trauma.

Back in 2016, the Brazil Ministry of Health had reported an increased number of people who were infected with Zika virus also had GBS. This prompted the US Centers for Disease Control (CDC) to work with Brazil to study the possibility of a link between Zika and GBS. Since then, several countries that have experienced Zika outbreaks recently have reported increases in people who have GBS.

Current CDC research suggests that GBS is strongly associated with Zika; however, only a small proportion of people with recent Zika virus infection get GBS. The most likely explanation of available evidence from outbreaks of Zika virus infection and Guillain-Barré syndrome is that Zika virus infection is a trigger of Guillain-Barré syndrome. As at today (December 2017), CDC notes on its website that it is continuing to investigate the link between GBS and Zika to learn more.

Diagnosis

Diagnosis is based on symptoms and findings on neurological examination including diminished or loss of deep-tendon reflexes. A lumbar puncture may be done for supportive information, though should not delay treatment. Other tests, such as blood tests, to identify the underlying trigger are not required to make the diagnosis of GBS and should not delay treatment.

Treatment and care

The following are recommendations for treatment and care of people with Guillain-Barré syndrome:
  • Guillain-Barré syndrome is potentially life-threatening. GBS patients should be hospitalized so that they can be monitored closely.
  • Supportive care includes monitoring of breathing, heartbeat and blood pressure. In cases where a patient's ability to breathe is impaired, he or she is usually put on a ventilator. All GBS patients should be monitored for complications, which can include abnormal heart beat, infections, blood clots, and high or low blood pressure.
  • There is no known cure for GBS. But treatments can help improve symptoms of GBS and shorten its duration.
  • Given the autoimmune nature of the disease, its acute phase is typically treated with immunotherapy, such as plasma exchange to remove antibodies from the blood or intravenous immunoglobulin. It is most often beneficial when initiated 7 to 14 days after symptoms appear.
  • In cases where muscle weakness persists after the acute phase of the illness, patients may require rehabilitation services to strengthen their muscles and restore movement.

Key Facts

  • Guillain-Barré syndrome is a rare condition in which a person’s immune system attacks their peripheral nerves.
  • People of all ages can be affected, but it is more common in adults and in males.
  • Most people recover fully from even the most severe cases of Guillain-Barré syndrome.
  • Severe cases of Guillain-Barré syndrome are rare, but can result in near-total paralysis.
  • People with Guillain-Barré syndrome should be treated and monitored; some may need intensive care. Treatment includes supportive care and some immunological therapies.

WHO Response

WHO is supporting countries to manage GBS in context of Zika virus infection by:
  • Enhancing surveillance of GBS in Zika affected countries.
  • Providing guidelines for the assessment and management of GBS.
  • Supporting countries to implement guidelines and strengthen health systems to improve the management of GBS cases.
  • Defining the research agenda for GBS.
Also see: All You Need To Know On Zika Virus Diesease

Reference(s)
1). World Health Organization: Guillain–Barré syndrome - WHO Fact Sheets. Accessed 11 February 2016. Available here: http://who.int/mediacentre/factsheets/guillain-barre-syndrome/en/
2). Centers for Disease Control: Guillain-Barré syndrome Q & A - CDC Zika Virus Home. Accessed 26 December 2017. Available here: http://www.cdc.gov/zika/qa/gbs-qa.html
3). Nobuhiro Yuki and Hans-Peter Hartung: Guillain–Barré Syndrome - N Engl J Med 2012. Accessed 5 February 2016. Available here: http://www.nejm.org/doi/full/10.1056/NEJMra1114525

Written February 2016; Updated December 2017

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