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ALTERNATIVE NAMES
Landry-Guillain-Barre syndrome; acute idiopathic polyneuritis; infectious polyneuritis; acute inflammatory polyneuropathy
DESCRIPTION
Guillain-Barré (ghee-yan-bah-ray) syndrome is a disorder in which the body's immune system attacks part of the nervous system, causing a neuromuscular autoimmune disease. It is a disorder involving progressive muscle weakness or paralysis, usually following an infectious illness. It is related to inflammation of multiple nerves.
In GBS the immune system starts to destroy the myelin sheath that surrounds the axons of many nerve cells, or even the axons themselves (axons are long, thin extensions of the nerve cells that carry nerve signals). This acute autoimmune attack on myelin in the peripheral nervous system (PNS) is associated with IgM antibodies to myelin glycolipid. The myelin sheath surrounding the axon speeds up the transmission of nerve signals and allows the transmission of signals over long distances. GBS can affect anybody. It can strike at any age, and both sexes are equally prone to the disorder. The syndrome is fairly rare, afflicting only about one person in every 100,000. It is not known why GBS strikes some people and not others. Nor do we know exactly what sets the disease in motion.
In autoimmune diseases in which the nerve cells' myelin sheaths are injured or degraded, the nerves cannot transmit signals efficiently. That is why the muscles begin to lose their ability to respond to the brain's commands, commands that must be carried through the nerve network.
CAUSES, INCIDENCE, & RISK FACTORS
Guillain-Barré Syndrome is an acute type of nerve inflammation. The inflammation damages portions of the nerve cell, resulting in muscle weakness or paralysis. The damage usually includes loss of the myelin sheath of the nerve (demyelination) ,which slows conduction of impulses through the nerve. The damage may also include destruction of the axon part of the nerve cell (denervation), which blocks conduction through the nerve.
The exact cause of this disorder is not known. It usually follows a minor infection, usually a respiratory (lung) infection or gastrointestinal (gut) infection. The signs of the infection usually have disappeared before the signs of Guillain-Barre begin.
Guillain-Barré Syndrome affects approximately 1 TO 8 out of 100,000 people (depending upon studies). It may occur at any age but is most common in people of both sexes between the ages 30 and 50.
Other factors with Guillain-Barré Syndrome include AIDS or AIDS-related complex, Mycoplasma infection, measles, herpes simplex, or other viral infection, major surgery within the past 6 weeks, systemic lupus erythematosus, Hodgkin's disease, other malignant diseases, and vaccines such as the anti-rabies vaccine and the swine flu vaccine.
PREVENTION
There is no known prevention for this disease.
SYMPTOMS
The disorder progresses rapidly (from days to about 1 month). Weakness and paralysis usually develop in a symmetrical manner from the feet and legs upward through the body, and is referred to as ascending paralysis. As paralysis reaches the level of the chest, the patient may require assisted ventilation in order to breathe. Movement and sensation changes occur simultaneously.
There is an initial phase of rapid symptom development that usually lasts for up to 3 weeks. This is followed by a plateau phase of no changes, then a recovery phase where symptoms improve. The recovery phase may last 4 to 6 months or longer.
Typical symptoms include:
- Muscle weakness or paralysis (the most common symptom) or uncoordinated movements.
- Weakness begins in the feet and legs and may progress upward to the arms and cranial (head) nerves.
- May progress rapidly over 24 to 72 hours.
- May begin in the arms and progress downward.
- May occur in the arms and legs at the same time.
- May occur in the cranial nerves only.
- May not occur (mild cases).
- Sensation changes.
- Numbness, decreased sensation.
- Tenderness or muscle pain (may be a cramp-like pain).
- Usually accompanies or precedes muscle weakness.
- May not occur at all.
Additional symptoms that may be associated with this disease:
- Blurred vision.
- Difficulty moving face muscles.
- Dizziness.
- Palpitations (sensation of feeling heartbeat).
- Difficulty beginning to urinate.
- Feeling of incomplete bladder emptying.
- Incontinence (leaking) of urine.
- Constipation.
- Muscle contractions.
Symptoms indicating an emergency:
- Difficulty swallowing, drooling.
- Difficulty breathing.
- Breathing absent temporarily.
- Feeling "unable to take a deep breath".
- fainting.
SIGNS & TESTS
A history of illness involving fever, followed by increasing muscle weakness and paralysis, may indicate Guillain-Barre syndrome.
Neuromuscular examination shows muscular (motor) dysfunction and may indicate dysfunction of the control of involuntary (autonomic) body functions. There is usually minimal sensory loss, even when there are sensory changes present. There may be evidence of decreased breathing ability caused by paralysis of the breathing muscles and a decrease in deep tendon reflexes in the arms and legs. Autonomic dysfunction may include changes in blood pressure (high, low, or fluctuating blood pressure), changes in heart rate (fast or slow), urinary retention (bladder unable to constrict to empty itself), and other abnormalities.
An NCV (nerve conduction velocity) shows demyelination.
An EMG (a test of electrical activity in muscles) shows lack of nervous stimulation.
A CSF (cerebrospinal fluid) examination may be abnormal, showing increase in protein without increase in white blood cell count.
An ECG may show abnormalities in some cases.
TREATMENT
The disease usually subsides spontaneously over time but symptoms may be severe during the course of the disorder. Hospitalization is usually required, and intensive care with life support (support of breathing, especially) may be necessary.
Corticosteroids such as prednisone may reduce inflammation of the nerves. They are not given for acute cases, but may be recommended if the disorder continues to progress. Medications that suppress the immune system may be used for some severe cases.
Plasmapheresis may benefit some cases if used in the initial phase. About 3 weeks, there is usually no benefit. Blood plasma, which contains antibodies, is removed from the body and replaced with intravenous fluids or antibody-free donated plasma.
Other symptoms are treated as necessary. This may include measures to prevent choking during feeding (through positioning or use of a feeding tube), intermittent bladder catheterization, or other treatments.
PROGNOSIS
Almost all cases (95 percent) survive. About 75 percent recover completely. Mild weakness may persist for some people. However, the disorder may be also be fatal. The outcome is most likely to be very good when symptoms are relieved within 3 weeks after they begin.
COMPLICATIONS
Breathing difficulty (respiratory failure).
Aspiration of food/fluids into the lungs.
Pneumonia.
Increased risk of infections.
Deep vein thrombosis.
Permanent loss of movement of an area.
Contractures of joints or other deformity.
CONTACT YOUR HEALTH CARE PROVIDER IF...
Call your health care provider or go to the emergency room or call the local emergency number (such as 911) if loss of movement, decreased sensation, or other emergency symptoms occur, including difficulty swallowing or breathing, feeling "unable to take a deep breath", or fainting.
HELPFUL RELATED LINKS
Guillain-Barré Syndrome Foundation International
Guillain-Barré Syndrome
Guillain-Barré Syndrome Support Group
Guillain-Barré Syndrome: Immunization Issues
Guillain-Barré Syndrome by Gabe Mirkin, M.D.
Estimated Annual Costs of Camphylobacer-Associated Guillain-Barré Syndrome
Intravenous Immunoglobulin is Preferred Therapy for Guillain-Barré Syndrome
Guillain-Barré Syndrome Fact Sheet
Guillain-Barré Syndrome (Acute Idiopathic Polyneuritis)
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