Guillain-Barré Syndrome
(Acute Inflammatory Demyelinating Polyradiculoneuropathy)
Pronounced: gee-yan bah-ray
Definition
Guillain-Barré syndrome is a rare inflammatory autoimmune disorder of the peripheral nerves. These are the nerves outside of the brain and spinal cord. Guillain-Barré is characterized by numbness, tingling, weakness, or paralysis in the legs, arms, breathing muscles, and face. It can affect all ages.
Nervous System
Causes
The exact cause of Guillain-Barré syndrome is unknown. However, in about 70% of patients, a recent infection or surgery serves as a trigger to an autoimmune response. This autoimmune response attacks the peripheral nerves, leading to weakness and a loss of sensation.
Risk Factors
A risk factor is something that increases your chance of getting a disease or condition. Risk factors for Guillain-Barré syndrome may include:
- Recent bacterial or viral infection (Most common are respiratory or gastrointestinal infections.)
-
Recent vaccine
- The swine flu vaccine administered from 1976-1977 was associated with excess cases of Guillain-Barré syndrome. (Since then, no other influenza virus vaccines have been associated with Guillain-Barré syndrome.)
- Recent surgery
- History of lymphoma , lupus , or AIDS
Symptoms
The first symptoms of Guillain-Barré syndrome include:
- Progressive muscle weakness on both sides of the legs, arms, and face
- Prickly, tingling sensations, usually in the feet or hands
- Pain is a significant complaint (Lower back pain is the most common complaint.)
- Loss of normal reflexes
Symptoms may develop over a period of hours, days, or weeks. They will vary in severity from minimal to total paralysis including respiratory weakness. The symptoms grow progressively worse. Most people experience the greatest weakness during the second or third week.
Related complications include:
- Facial weakness
- Blood pressure instability
- Heart rate changes
- Sweating abnormalities
- Cardiac arrhythmias
- Urinary/gastrointestinal dysfunction
Most patients recover fully, but as many as 25% will have some residual symptoms. Five to ten percent have permanent, disabling deficits, and for 5% the condition is fatal.
Diagnosis
Your doctor will ask about your symptoms and medical history, and perform a physical exam. Diagnosis is dependant on the physical exam and history, cerebrospinal fluid findings, and nerve conduction studies .
You may have a lumbar puncture, also called a spinal tap . For this test, a needle is inserted into your lower back to remove a sample of cerebrospinal fluid for testing. If high levels of a certain protein are detected, and there is no infection, this is an indication that you may have Guillain-Barré syndrome. Electrodiagnostic studies are usually done, which test the electrical conduction in the peripheral nerves and help differentiate Guillain-Barré from other disorders with similar symptoms.
Treatment
Treatment aims to reduce the body’s autoimmune response and decrease complications that result from immobility. Hospitalization is important because symptoms may rapidly become more severe, including respiratory failure, cardiac arrhythmias , and blood pressure instability. Most patients need to be in the hospital for at least one month. Common treatments include:
Plasmapheresis
During plasmapheresis , blood is removed from your body and passed through a machine that separates blood cells. The separated cells are then returned to your body with new plasma. This procedure may help shorten the course and severity of Guillain-Barré syndrome.
High-dose Immunoglobulin Therapy
Intravenous infusion with immunoglobulin (IVIg) may help reduce the severity of a Guillain-Barré attack. Immunoglobulins are proteins that are naturally produced by the body’s immune system.
Mechanical Ventilation
In 30% of cases, muscles necessary for breathing become paralyzed. This is treated with immediate emergency support from a mechanical ventilator.
Pain Control
Your doctor may prescribe medication to reduce the pain. These may include nonsteroidal anti-inflammatory drugs, gabapentin, carbamezepine, or narcotic analgesics.
If you are diagnosed with Guillain-Barré syndrome, follow your doctor's instructions .
RESOURCES:
Guillain-Barré Syndrome Foundation International
http://www.Guillain-Barré.com
National Institute of Neurological Disorders and Stroke
http://www.ninds.nih.gov
CANADIAN RESOURCES:
Canadian Institute for Health Information
http://www.cihi.ca/cihiweb/dispPage.jsp?cw_page=home_e
Health Canada
http://www.hc-sc.gc.ca/index-eng.php
References:
Amyotrophic lateral sclerosis. EBSCO Publishing DynaMed website. Available at: Available at: http://www.ebscohost.com/dynamed . Updated January 31, 2008. Accessed February 23, 2008.
Bradley WG, Daroff RB. Neurology in Clinical Practice . Philadelphia, PA: Butterworth Heinemann Publishing; 2004.
Brown B Amyotrophic lateral sclerosis. In: Gilman S, ed. MedLink Neurology. San Diego, CA: MedLink Corporation. Available at: http://www.medlink.com . Accessed February 23, 2008.
Goetz S. Acute inflammatory demyelinating polyradiculoneuropathy. In: Clinical Neurology. 3rd ed. Philadelphia, PA: Saunders; 2007.
National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov .
Textbook of Clinical Neurology . WB Saunders Company; 1999.
Last reviewed November 2008 by Rimas Lukas, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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