Guillain-Barre Syndrome a Rare But Serious Disorder

P. James Dyck, M.D., Neurology, Mayo Clinic

DEAR MAYO CLINIC:

I was recently diagnosed with mononucleosis and was told that because of it, I am more susceptible to other illnesses, including Guillain-Barre.

What is Guillain-Barre syndrome? What do I need to watch for? Is it preventable? How is it treated?

ANSWER:

Guillain-Barre syndrome is an inflammatory disorder that can cause dramatic weakness and even complete paralysis. Most people recover but may require months, or even years, to regain their strength.

What causes the syndrome is unknown. It seems to be triggered by infection, such as a respiratory infection or stomach flu. Other triggers are mononucleosis; HIV, the virus that causes AIDS; and Hodgkin's lymphoma. Sometimes it occurs without any known trigger.

Your care provider was wise to mention the risk of Guillain-Barre syndrome. If symptoms start, prompt care is necessary. But there's no need to panic. Guillain-Barre is rare, affecting about 1 in 100,000 people per year. Even if you have mononucleosis, or the stomach flu or HIV, you are unlikely to develop Guillain-Barre syndrome.

The syndrome causes the body's immune system to go awry. In addition to attacking viruses, the immune system attacks nerves, destroying the protective covering on the nerves called the myelin sheath. That process, called demyelination, slows or stops signals from passing through the nerves.

Usually, the syndrome's first signs are weakness or numbness starting in the lower limbs, then progressing to the upper body and arms. Additional symptoms can include light-headedness; a heartbeat that races or, conversely, is too slow; difficulty speaking, chewing or swallowing; and difficulty breathing.

Symptoms progress quickly over the course of days up to four weeks. In some cases, symptoms can become severe in just a few hours. Needless to say, the rapid changes are frightening. Patients with severe symptoms need hospitalization. About one-third of patients will require breathing assistance for a time.

To diagnose Guillain-Barre, a doctor will carefully review a person's symptoms and medical history. Because of the nerve damage, tendon reflexes are affected and in fact usually disappear. Another differentiating factor is the rapid onset of symptoms. Illnesses with similar nerve signs and symptoms typically develop more gradually. A nerve conduction velocity test, which tests the speed at which signals pass through a nerve, may be helpful -- nerve conduction velocity is typically reduced in Guillain-Barre syndrome. The doctor might order a spinal tap to obtain a sample of cerebrospinal fluid. Patients with Guillain-Barre often have an increased protein level in their spinal fluid.

Two effective treatments help prevent symptoms from worsening and speed recovery:

Plasma exchange:

The patient's blood is removed and cleansed of the immune cell antibodies causing the nerve damage. The cleansed blood is replaced, and the body manufactures plasma to compensate for what was removed.

Intravenous immunoglobulin:

Collected via blood donors, immunoglobulin is a concentrated dose of healthy antibodies. For reasons we don't completely understand, immunoglobulin reduces the nerve damage caused by the patient's own antibodies.

After about four weeks, the symptoms usually stabilize and begin to improve. The myelin begins to repair. Patients may need physician therapy and mobility aids such as a wheelchair or braces during recovery.

About 80 percent of patients recover completely or have minor weaknesses.

Five to 10 percent have permanent difficulties with their strength or coordination. In about 5 percent of cases, the disorder is fatal.

Awareness of Guillain-Barre and its association with mononucleosis is your best defense. There's no known way to prevent Guillain-Barre. Early diagnosis and treatment improve prognosis in the rare event the syndrome occurs.

-- P. James Dyck, M.D., Neurology, Mayo Clinic, Rochester, Minn.

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