Guillain-Barré syndrome (GBS) is a rare disorder in which a person’s own immune system damages their nerve cells, causing muscle weakness. It is an important cause of acute neuromuscular paralysis.
The term GBS is often considered to be synonymous with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), but with the increasing recognition over the past few decades of variants, the number of diseases that fall under the rubric GBS has grown to include axonal variants and more restricted variants such as Miller Fisher syndrome (MFS).
In GBS the nerve insulation (myelin) and sometimes even the inner covered part of the nerve (axon) is damaged and signals are delayed or otherwise changed. The resultant antibodies attack the myelin sheath, and sometimes the axon, causing paralysis and muscular weakness as well as strange sensations, as the sensory nerves of the skin may be affected.
The syndrome appears to be triggered by acute viral or bacterial illnesses, such as respiratory or gastrointestinal infections, occurring one to three weeks earlier. However, other events such as pregnancy, dengue fever, surgical procedures, insect bites and Bell’s palsy have also been shown to cause GBS. Usually, the symptoms start in the feet or legs and progress up the body over a few days or weeks. The disorder can be mild, moderate or severe, with life support needed in the worst cases. The exact cause is unknown and there is no known cure. Most people spontaneously recover, although some will be left with permanent disabilities.
In the United States, for example, an estimated 3,000 to 6,000 people develop GBS each year on average, whether or not they received a vaccination. Anyone can develop GBS; however, it is more common among older adults. The incidence of GBS increases with age, and people older than 50 years are at greatest risk for developing GBS.
Causes
The precise cause of Guillain-Barre is unknown. Some of the cases have followed a lung infection or a gastrointestinal infection. The following infections have been associated with Guillain-Barre –
- Campylobacterjejuni infection. Campylobacteris the most common bacterial cause of diarrhea in the United States. Campylobacterinfection is also the most common risk factor for Guillain-Barre. It is often found in undercooked food, especially poultry.
- Influenza (the flu)
- Cytomegalovirus (a strain of the herpes virus)
- Epstein-Barr virus infection (mononucleosis)
- Mycoplasma pneumonia (‘atypical pnueumonia’ caused by bacteria-like organisms)
- HIV or AIDS
- Hodgkin disease, a malignant disease of the lymphatic system
- Systemic lupus erythromatosus, an autoimmune disease of the skin and other organs
There is no evidence to suggest that Guillain-Barrè syndrome is contagious.
Symptoms
Symptoms of Guillain-Barré syndrome can develop quickly over a few hours. The muscle weakness often gets progressively worse within a few days or weeks.
Symptoms often start in your feet and hands before spreading to your arms and legs. Initially, you may have:
- Pain, tingling and numbness
- Progressive muscle weakness
- Co-ordination problems and unsteadiness (you may be unable to walk unaided)
The weakness usually affects both sides of your body and may get worse over a period of several days.
In mild cases of Guillain-Barré syndrome, your muscles may only be slightly weakened. However, in more severe cases, the muscle weakness can progress to:
- Temporary paralysis of the legs, arms and face
- Temporary paralysis of the respiratory muscles
- Blurred or double vision
- Difficulty speaking
- Difficulty chewing or swallowing (dysphagia), resulting in the need to be fed through a tube
- Difficulty with digestion or bladder control
- Fluctuations in heart rate or blood pressure
Some people with Guillain-Barré syndrome don’t experience any pain, while others have severe pain in their spine, arms and legs.
Emergency symptoms (seek medical help right away) –
- Breathing temporarily stops
- Cannot take a deep breath
- Difficulty breathing
- Difficulty swallowing
- Drooling
- Fainting
- Feeling light-headed when standing
Treatment
Conventional Treatment
- Plasma Exchange or Plasmapheresis – Plasma exchange is a method by which whole blood is removed from the body and processed so that the red and white blood cells are separated from the plasma, or liquid portion of the blood. The blood cells are then returned to the patient without the plasma, which the body quickly replaces. Scientists still don’t know exactly why plasma exchange works, but the technique seems to reduce the severity and duration of the Guillain-Barré episode. This may be because plasmapheresis can remove antibodies and other immune cell-derived factors that could contribute to nerve damage.
- Immunoglobin therapy – In this therapy the patient is given intravenous injections of the proteins that, in small quantities, the immune system uses naturally to attack invading organisms. Researchers have found that giving high doses of these immunoglobulins, derived from a pool of thousands of normal donors, to GBS patients can lessen the immune attack on the nervous system. Investigators don’t know why or how this works, although several hypotheses have been proposed.
- Steroid Hormones (corticosteroids) – This is helpful in reducing the severity of Guillain-Barré, but controlled clinical trials have demonstrated that this treatment not only is not effective but may even have a deleterious effect on the disease.
- Medications – Muscle and joint pain can be treated with over-the-counter analgesics such as aspirin. If necessary, stronger pain medication (e.g., acetaminophen with hydrocodone) may be prescribed. Muscle spasms can be controlled with relaxants such as diazepam (Valium). Unpleasant sensation problems, such as painful tingling, can be treated with tricyclic antidepressants (TCAs) or anticonvulsants such as gabapentin (Neurontin). Corticosteroids, which often effectively treat the symptoms of autoimmune disorders, actually worsen GBS and should not be used. However, they often are used to treat CIDP.