Guillain–Barré syndrome

31 October 2016

Key facts

  • Guillain-Barré syndrome (GBS) is a rare condition in which a person’s immune system attacks the 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.
  • Guillain-Barré syndrome is potentially life-threatening. People with Guillain-Barré syndrome should be treated and monitored; some may need intensive care. Treatment includes supportive care and some immunological therapies.

Introduction

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 pain, temperature and touch sensations. This can result in muscle weakness and loss of sensation in the legs and/or arms.

It is a rare condition, and while it is more common in adults and in males, people of all ages can be affected.

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%–30 % of people, the chest muscles are affected, making it hard to breathe.
  • The ability to speak and swallow may become affected in severe cases of Guillain-Barré syndrome. These cases are considered life-threatening, and affected individuals should be 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.
  • Even in the best of settings, 3%–5% of Guillain-Barré syndrome patients die from complications, which can include paralysis of the muscles that control breathing, blood infection, lung clots, or cardiac arrest.

Causes

Guillain-Barré syndrome is often preceded by an infection. This could be a bacterial or viral infection. Guillain-Barré syndrome may also be triggered by vaccine administration or surgery.

In the context of Zika virus infection, unexpected increase in cases of Guillain-Barré syndrome has been described in affected countries. 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.

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.

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.