Definition

Guillain-Barré syndrome is a rare inflammatory autoimmune disorder of the peripheral nerves. These are the nerves outside of the brain and spinal cord. Guillain-Barré is characterized by numbness, tingling, weakness, or paralysis in the legs, arms, breathing muscles, and face. It can affect all ages.

Causes

The exact cause of Guillain-Barré syndrome is unknown. However, in about 70% of patients, a recent infection or surgery serves as a trigger, which causes an autoimmune response. This autoimmune response attacks the peripheral nerves, leading to weakness and a loss of sensation.

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition. Risk factors for Guillain-Barré syndrome may include:

  • Recent bacterial or viral infection (most common are respiratory or gastrointestinal (GI) infections)
  • Recent vaccine
    • The swine flu vaccine administered from 1976-1977 was associated with excess cases of Guillain-Barré syndrome. (Since then, no other influenza virus vaccines have been associated with Guillain-Barré syndrome.)
  • Recent surgery
  • History of lymphoma, lupus, or AIDS

Symptoms

The first symptoms of Guillain-Barré syndrome include:

  • Progressive muscle weakness on both sides of the legs, arms, and face
  • Prickly, tingling sensations (paresthesias), usually in the feet or hands
  • Pain is a significant complaint; most common type is lower back pain
  • Loss of normal reflexes

Symptoms may develop over a period of hours, days, or weeks. They will vary in severity from minimal to total paralysis including respiratory weakness. The symptoms grow progressively worse. Most people experience the greatest weakness during the second or third week. Related complications also include facial weakness, blood pressure instability, heart rate changes, sweating abnormalities, cardiac arrhythmias, and urinary/GI dysfunction. Most recover fully, but as many as 25% will have some residual symptoms. For 5% of patients, Guillain-Barré syndrome is fatal.

Diagnosis

Your doctor will ask about your symptoms and medical history, and perform a physical exam. Diagnosis is dependant on the physical exam and history, cerebrospinal fluid findings, and nerve conduction studies. You may have a lumbar puncture, also called a spinal tap. For this test, a needle is inserted into your lower back to remove a sample of cerebrospinal fluid for testing. If high levels of a certain protein are detected, and there is no infection, this is an indication that you may have Guillain-Barré syndrome. Electrodiagnostic studies may also be done, which test the electrical conduction in the peripheral nerves and help differentiate Guillain-Barré from other disorders with similar symptoms.

Treatment

Treatment aims to reduce the body’s autoimmune response and decrease complications that result from immobility. Hospitalization is important because symptoms may rapidly become more severe, including respiratory failure, cardiac arrhythmias, and blood pressure instability. Most patients need to be in the hospital for at least one month. Common treatments include:

Plasmapheresis

Blood is removed from your body and passed through a machine that separates blood cells. The separated cells are then returned to your body with new plasma. This procedure may help shorten the course and severity of Guillain-Barré syndrome.

High-dose Immunoglobulin Therapy

Intravenous infusion with immunoglobulin (IVIg) may help reduce the severity of a Guillain-Barré attack. Immunoglobulins are proteins that are naturally produced by the body’s immune system.

Mechanical Ventilation

In 30% of cases, muscles necessary for breathing become paralyzed. This is treated with immediate emergency support from a mechanical ventilator.

Prevention

There are no guidelines for the prevention of Guillain-Barré syndrome.