The Facts

Guillain-Barré syndrome (GBS) is a rare neurological disorder which causes progressive paralysis, starting from the feet and progressing up throughout the rest of the affected person's body (ascending paraplegia). It occurs when the body's immune system attacks the peripheral nerves in the body. This is known as an autoimmune disease and can be triggered following a surgery, or by a flu-like illness or a stomach infection. As the immune system fights off the infection, it mistakenly attacks the peripheral nerves.

GBS affects men and women of all ethnicities and ages. Treatment is available for this condition and 80% of those affected recover with minor or no residual neurologic deficits (lasting damage to the nervous system). More severe cases of the condition require emergency medical treatment, admission to hospital, and longer rehabilitation periods. About 10% to 15% of affected people will have major residual deficits. The residual neurologic deficits range from having difficulty running or walking to difficulty breathing; some patients may need to be permanently on a respirator. Less than 2% of affected individuals will die.

Causes

Healthy nerves are wrapped in an outer coating known as myelin, which is similar to the plastic insulation wrapped around a wire. In cases of GBS, the myelin is damaged. Without this coating, the nerves may not be able to send messages or they send weaker messages that may result in muscle weakness, tingling, numbness, or paralysis. People with this condition may experience significant weakness and have difficulty performing everyday tasks, including walking.

Infections by certain bacteria or viruses seem to trigger GBS in more than 50% of cases. In particular, infections of the gastrointestinal (GI) tract or respiratory system are most commonly associated with the condition.

There is no clear association between seasonal influenza vaccination and GBS, although some reviewss have suggested that vaccination against the flu may trigger the disease in a very small number of people. A person's risk of developing GBS after a flu shot is about one in one million. Keep in mind that receiving the flu shot helps protect your own health and that of those around you and is recommended despite the extremely small increased risk of GBS.

People with lymphoma, HIV, or lupus seem to be more at risk for GBS.

Symptoms and Complications

Symptoms of GBS often start with tingling or numbness in the extremities of the body. The hands, feet, and face are often affected first. Symptoms usually show up between 5 days and 3 weeks after exposure to the trigger.

People with GBS suffer from a wide range of symptoms that may include:

• weakness in leg, arm, and facial muscles
• problems with speech and swallowing
• pain in the muscles of the back
• shortness of breath
• decreased ability to move the eyes

Symptoms typically progress over a period of a few days and are usually at their worst 2 weeks after onset for 50% of those affected, and at 4 weeks for 90%. Weakness will peak and then it will typically remain steady for around one week before resolving. A person experiencing a mild case of GBS may only have slight weakness or numbness and loss of tendon reflexes. They may not require hospitalization or treatment. In more severe cases, profound weakness, respiratory difficulties, heart arrhythmias, or unstable blood pressure may develop, requiring admission to a hospital or an intensive care unit and possibly assistance from a breathing machine called a ventilator.

Guillan-Barré syndrome can have lasting emotional and physical affects because of the sudden and unexpected onset. A person will need to depend on family or caregivers for months after the initial attack, and will require extensive therapy to return to their previous state of health. Approximately 30% of people still experience residual weakness after 3 years, and about 3% may experience a relapse of muscle weakness and tingling many years after the first diagnosis.

In addition to the classical form of GBS, there are variations such as a form that attacks the nerve endings and Miller-Fisher syndrome which starts with paralysis of the facial muscles and progresses down (descending paraplegia).

Making the Diagnosis

To diagnose GBS, your doctor will take your medical history and ask you about your symptoms to help rule out any other conditions that may affect the nervous system, such as a stroke. A stroke usually causes muscle weakness or paralysis on one side of the body, whereas GBS usually occurs on both sides.

After discussing your symptoms, your doctor may ask you about any recent infections or illnesses. Recent infections are closely related to the appearance of GBS and are reported by about two-thirds of people with this condition.

The doctor will perform a neurological examination and may also order special tests to examine the nerves.

Two main tests help doctors diagnose a patient with GBS:

• To test how well your nerves transmit signals to muscles, doctors use a test called electromyography (EMG) and nerve conduction studies. Using small electrodes placed in and on the skin, the technician can measure the speed and strength of electrical messages being sent along the nerve. Nerves damaged by GBS conduct signals at a slower speed.
• To examine the cerebrospinal fluid (CSF), doctors may perform a spinal tap (also called a lumbar puncture). This fluid surrounds the brain and spinal cord. Examining your CSF is critical in making the diagnosis.

Treatment and Prevention

Available treatments for GBS help speed up recovery. As with many conditions, the earlier the condition is recognized and treated, the greater the chance of a fast recovery. Recovery may take longer for more severe cases of the condition. Treatment with either plasma exchange or intravenous immunoglobulin (IVIg) therapy is often very successful in helping people recover faster.

Plasma exchange helps remove antibodies from the blood. Antibodies are a part of the immune system and normally help your body fight inflammation and infection. With conditions like GBS, certain antibodies damage nerves and cause a loss of transmission of electrical signals to the muscles. Plasmapheresis works by separating a person's blood into its two main components: cells and plasma. The cells are returned to the body. The plasma is discarded and replaced with artificial plasma. Plasmapheresis works best for people with GBS when started within the first 2 weeks of symptom onset.

Intravenous immunoglobulin (IVIg) contains antibodies collected from donated blood. This therapy has also been shown to speed recovery from GBS when given in the first 2 weeks. The exact way IVIg works is unknown.

On the road to recovery, a person with GBS may need to consider pain relief and how to maintain muscle strength. Heat therapy has been helpful for patients in relieving pain and beginning physical therapy earlier. Physiotherapy is helpful in assisting recovery. Pain medications may also be required to help alleviate nerve pain.

There is currently no known prevention for GBS. However, the best way to stay healthy is to make regular physical activity a part of your daily routine and to eat a well-balanced, healthy diet.