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Guillain-Barré Syndrome

by Michael Rubin, MDCM

Guillain-Barré syndrome (acute inflammatory demyelinating polyneuropathy) is a form of polyneuropathy causing muscle weakness, which usually worsens over a few days to weeks, then slowly returns to normal on its own. With treatment, people may improve more quickly.

  • An autoimmune reaction may damage the myelin sheath around nerves.

  • Usually, weakness begins in both legs and moves up the body.

  • Electromyography and nerve conduction studies can help confirm the diagnosis.

  • People with this syndrome are hospitalized immediately because symptoms can worsen rapidly.

  • Immune globulin given intravenously or plasma exchange speeds recovery.

The presumed cause is an autoimmune reaction. The body’s immune system attacks the myelin sheath, which surrounds the nerve and enables nerve impulses to travel quickly. In about two thirds of people with this syndrome, symptoms begin about 5 days to 3 weeks after a mild infection (such as a Campylobacter infection, mononucleosis, or another viral infection), surgery, or an immunization.

Weakness caused by Guillain-Barré syndrome usually worsens over 3 or 4 weeks, then remains the same or starts to return to normal. If it worsens for more than 8 weeks, it is considered chronic inflammatory demyelinating polyneuropathy (CIDP), not Guillain-Barré syndrome.

Symptoms

Symptoms usually begin in both legs, then progress upward to the arms. Occasionally, symptoms begin in the arms or head and progress downward.

Symptoms include weakness and a pins-and-needles sensation or loss of sensation. Weakness is more prominent than abnormal sensation. Reflexes are decreased or absent. In 90% of people who have Guillain-Barré syndrome, weakness is most severe within 3 weeks. In 5 to 10%, the muscles that control breathing become so weak that a ventilator is needed. The facial and swallowing muscles become weak in more than half of affected people, When these muscles are weak, people may choke when they are eating or become dehydrated and malnourished.

If the disorder is very severe, internal functions controlled by the autonomic nervous system may be impaired. For example, blood pressure may fluctuate widely, heart rhythm may become abnormal, people may retain urine, and severe constipation may develop.

In a variant called Miller-Fisher syndrome, only a few symptoms develop: Eye movements become paralyzed, walking becomes difficult, and normal reflexes disappear.

Diagnosis

  • Electromyography and nerve conduction studies, magnetic resonance imaging, blood tests, and a spinal tap

Doctors suspect the diagnosis based on the pattern of symptoms. Tests are done to confirm the diagnosis. People are admitted to the hospital to have the tests because the syndrome can worsen rapidly and impair the muscles involved in breathing.

Analysis of cerebrospinal fluid (which surrounds the brain and spinal cord) obtained by a spinal tap (lumbar puncture—see Figure: How a Spinal Tap Is Done), electromyography (see Tests for Brain, Spinal Cord, and Nerve Disorders : Electromyography and Nerve Conduction Studies), magnetic resonance imaging (MRI), nerve conduction studies, and blood tests can help doctors exclude other possible causes of severe weakness. MRI can help exclude spinal cord damage due to compression (for example, by a tumor or an abscess—see Compression of the Spinal Cord) or transverse myelitis (inflammation of the spinal cord—see Acute Transverse Myelitis). A combination of high protein levels and few or no white blood cells in the cerebrospinal fluid and characteristic results from electromyography strongly suggest Guillain-Barré syndrome.

Prognosis

Damage stops progressing within 8 weeks. Without treatment, most people improve slowly over several months. However, with early treatment, people can improve very quickly—in days or weeks. About 30% of adults and even more children with the disorder have residual weakness 3 years after the syndrome began. On average, fewer than 2% of people die.

Treatment

  • Hospitalization

  • If needed, use of a ventilator to help with breathing

  • Immune globulin or plasma exchange

Guillain-Barré syndrome can worsen rapidly and is a medical emergency. People who develop this syndrome should be hospitalized immediately. Establishing the diagnosis is crucial because the sooner appropriate treatment is started, the better the chance of a good outcome.

In the hospital, people are closely monitored so that breathing can be assisted with a ventilator if necessary. People with weak facial and neck muscles may need to be fed through a catheter inserted in a vein (intravenously) or through a tube placed directly into the stomach or small intestine through a small incision in the abdomen.

Nurses take precautions to prevent pressure sores and injuries by providing soft mattresses and by turning the people with severe weakness every 2 hours.

If weakness is less severe, physical therapy is started to help preserve joint and muscle function and the ability to walk. Heat therapy may be used first to relieve pain and thus make physical therapy more comfortable.

Immune globulin, given early and intravenously for 5 days, is the treatment of choice. If immune globulin is ineffective, plasma exchange (filtering of toxic substances, including antibodies to the myelin sheath, from the blood—see ) can help. These treatments are relatively safe, shorten the hospital stay, speed recovery, and reduce the risk of death and permanent disability. Because plasma exchange removes immune globulin from the blood, plasma exchange is not used at the same time as immune globulin. It is delayed for at least 2 days after this drug is given.

Corticosteroids do not help and may worsen the syndrome.