Introduction

Guillain-Barre syndrome is an uncommon inflammatory disorder in which your body's immune system attacks your nerves, typically causing severe weakness and numbness that usually starts in your extremities and quickly worsens. Eventually your whole body can become paralyzed, even the muscles used for breathing.

The exact cause of Guillain-Barre syndrome is unknown, but it sometimes is triggered by a respiratory infection or the stomach flu. Luckily, this potentially deadly disorder is relatively rare, occurring worldwide in only one or two people per 100,000. All age groups can be affected, but it occurs most often in young adults and the elderly.

In its most severe form, Guillain-Barre syndrome is a medical emergency requiring hospitalization. About 30 percent of the people who have the disorder will temporarily need the help of a machine to breathe.

There is no cure for the disorder, but several treatments can ease symptoms and reduce the duration of the illness. Most people recover completely from even the most severe cases of Guillain-Barre syndrome.

Signs and symptoms

Guillain-Barre syndrome usually begins with weakness, tingling or loss of sensation starting in your feet and legs and spreading to your upper body and arms. In some people, these symptoms begin in the arms or even the face. As the disorder progresses, muscle weakness can evolve into paralysis. Other signs and symptoms may include:

• Difficulty with eye movement, facial movement, speaking, chewing or swallowing

• Severe pain in the lower back

• Difficulty with bladder control or intestinal functions

• Very slow heart rate or low blood pressure

• Difficulty breathing

Most people with Guillain-Barre syndrome experience their most significant weakness within three weeks after symptoms begin. In some cases, signs and symptoms may progress very rapidly with complete paralysis of legs, arms and breathing muscles over the course of a few hours.

Causes

The exact cause of Guillain-Barre syndrome is still unknown. In about 60 percent of the cases, an infection affecting either the lungs or the digestive tract precedes the disorder. But scientists don't know why such an infection can lead to Guillain-Barre syndrome for some people and not for others. Many cases appear to occur without any triggers.

In Guillain-Barre syndrome, your immune system — which usually only attacks foreign material and invading organisms — begins attacking the nerves that carry signals between your body and your brain. Specifically, the nerves' protective covering (myelin sheath) is damaged and this interferes with the signaling process, causing weakness, numbness or paralysis.

Risk factors

The most common triggering factor for Guillain-Barre syndrome appears to be infection with campylobacter, a type of bacteria commonly found in undercooked food, especially poultry. Guillain-Barre may also be triggered by surgery and, in very rare cases, influenza immunizations. Other triggers include:

• Hodgkin's disease

• Mononucleosis

• HIV, the virus that causes AIDS

When to seek medical advice

Guillain-Barre syndrome is a serious disease that requires immediate hospitalization because of the rapid rate at which it worsens. Early treatment is important. The sooner appropriate treatment is started, the better the chance of a good outcome. If treatment can result in reducing the inflammation of your peripheral nerves, the chances of serious permanent damage to the myelin sheath and nerve fibers may be substantially decreased.

If you experience a tingling sensation in your toes, feet or legs followed by muscle weakness, seek medical attention immediately. Failure to see your doctor promptly may lead to progression of the disorder and paralysis, which can leave you incapacitated and unable to get help.

Screening and diagnosis

Guillain-Barre syndrome can be difficult to diagnose in its earliest stages. Its signs and symptoms are similar to those of other neurological disorders and may vary from person to person.

The first step in diagnosing Guillain-Barre syndrome is for your doctor to take a careful medical history to fully understand the cluster of signs and symptoms you're experiencing. People with Guillain-Barre syndrome have a relatively sudden onset of signs and symptoms, and muscle weakness usually occurs on both sides of the body as opposed to on one side only, as in a stroke.

A spinal tap (lumbar puncture) and nerve function tests are commonly used to help confirm a diagnosis of Guillain-Barre syndrome.

Spinal tap (lumbar puncture)
This procedure involves inserting a needle into your spinal canal, at the low back (lumbar) level. You generally lie on your side with your knees pulled up and your chin tucked in. A local anesthetic will be injected to numb your skin, but you'll probably feel pain for a few seconds when the needle punctures the tissue surrounding your spinal cord.

A small amount of cerebrospinal fluid is withdrawn. It will be tested for a specific type of change that commonly occurs in people who have Guillain-Barre syndrome. After the test, you may need to stay in the hospital for at least a few hours for observation.

Nerve function tests
Your doctor may want information from two types of nerve function tests — electromyography and nerve conduction velocity.

Electromyography involves the insertion of a thin needle electrode through the skin and into a muscle. You'll then be asked to contract that muscle, and the electrical activity detected by the electrode can help determine if your muscle weakness is caused by muscle damage or nerve damage.

In a nerve conduction velocity test, patch-like electrodes are affixed to the skin at various intervals, following the path of a particular nerve. That nerve is then stimulated with a mild electric shock, and the test records how long it takes for the electrical impulse to travel from one electrode to another.

Complications

A potentially deadly complication of Guillain-Barre syndrome is that the weakness or paralysis can spread to the muscles that control your breathing. About 30 percent of the people who have the disorder will require temporary help from a machine to breathe. About 5 percent will die, despite intensive care.

But 80 percent of the people with Guillain-Barre syndrome recover completely or have only minor, residual weakness or abnormal sensations, such as numbness or tingling. Five percent to 10 percent experience more serious, permanent problems with sensation and coordination, including some cases of severe disability. And about one in 10 of those affected is at risk of experiencing a relapse.

Treatment

There's no cure for Guillain-Barre syndrome. But two treatments have been shown to speed the recovery from and reduce the severity of the disorder:

• Plasmapheresis. This treatment — also known as plasma exchange — is a type of "blood cleansing" in which damaging antibodies are removed from your blood. Plasmapheresis consists of removing the liquid portion of your blood (plasma) and separating it from the actual blood cells. The blood cells are then put back into your body, which manufactures more plasma to make up for what was removed. It's not clear why this treatment works, but scientists believe that plasmapheresis rids plasma of certain antibodies that contribute to the immune system attack on the peripheral nerves.

• Intravenous immunoglobulin. Immunoglobulin contains healthy antibodies from blood donors. High doses of immunoglobulin can block the damaging antibodies that may contribute to Guillain-Barre syndrome.

Each of these treatments is equally effective. Mixing the treatments or administering one after the other is no more effective than using either method alone.

Although some people can take months and even years to recover, most cases of Guillain-Barre syndrome follow this general timeline:

• Following the first symptoms, the condition tends to progressively worsen for about two weeks.

• Symptoms reach a plateau and remain steady for two to four weeks.

• Recovery begins.

Treatment with plasmapheresis or intravenous immunoglobulin shortens the time period before recovery begins by as much as 50 percent.

Often before recovery begins, caregivers may need to manually move your arms and legs to help keep your muscles flexible and strong. After recovery has begun, you'll likely need physical therapy to help regain strength and proper movement so you'll be able to function on your own. You may need training with adaptive devices, such as a wheelchair or braces, to give you mobility and self-care skills.