Syringomyelia (sear-IN-go-my-EEL-ya) is a disorder in which a cyst forms within the spinal cord. This cyst, called a syrinx, expands and elongates over time, destroying the center of the spinal cord. Since the spinal cord connects the brain to nerves in the extremities, this damage results in pain, weakness, and stiffness in the back, shoulders, arms, or legs. Other symptoms may include headaches and a loss of the ability to feel extremes of hot or cold, especially in the hands. Each patient experiences a different combination of symptoms. Magnetic resonance imaging (MRI) has significantly increased the number of syringomyelia cases diagnosed in the beginning stages of the disorder. Signs of the disorder tend to develop slowly, although sudden onset may occur with coughing or straining. If not treated surgically, syringomyelia often leads to progressive weakness in the arms and legs, loss of hand sensation, and chronic, severe pain. In most cases, the disorder is related to a congenital abnormality of the brain called a Chiari I malformation. This malformation occurs during the development of the fetus and causes the lower part of the cerebellum to protrude from its normal location in the back of the head into the cervical or neck portion of the spinal canal. Syringomyelia may occur as a complication of trauma, meningitis, hemorrhage, a tumor, or arachnoiditis. Symptoms may appear months or even years after the initial injury, starting with pain, weakness, and sensory impairment originating at the site of trauma. Some cases of syringomyelia are familial, although this is rare.
In some patients it may be necessary to drain the syrinx, which can be accomplished using a catheter, drainage tubes, and
valves.
In the absence of symptoms, syringomyelia is usually not treated. In addition, a physician may recommend not treating the
condition in patients of advanced age or in cases where there is no progression of symptoms. Whether treated or not, many
patients will be told to avoid activities that involve straining.
Investigators have found that as the heart beats, syrinx fluid is forced downward. This finding suggests a role for the cardiovascular
system in syringomyelia.
Surgical techniques are also being refined by the neurosurgical research community. It is also important to understand the
role of birth defects in the development of hindbrain malformations that can lead to syringomyelia. Dietary supplements of
folic acid during pregnancy have already been found to reduce the number of cases of certain birth defects.
Diagnostic technology is another area for continued research. Diagnostic tests have improved greatly with the availability
of new, non-toxic, contrast dyes. Patients can expect even better techniques to become available in the future.
American Chronic Pain Association (ACPA) P.O. Box 850 Rocklin, CA 95677-0850 ACPA@theacpa.org http://www.theacpa.org Tel: 916-632-0922 800-533-3231 Fax: 916-652-8190 |
American Syringomyelia & Chiari Alliance Project (ASAP) P.O. Box 1586 Longview, TX 75606-1586 info@asap.org http://www.asap.org Tel: 903-236-7079 800-ASAP-282 (272-7282) Fax: 903-757-7456 |
Christopher and Dana Reeve Foundation 636 Morris Turnpike Suite 3A Short Hills, NJ 07078 informations@christopherreeve.org http://www.christopherreeve.org Tel: 973-379-2690 800-225-0292 Fax: 973-912-9433 |
March of Dimes 1275 Mamaroneck Avenue White Plains, NY 10605 askus@marchofdimes.com http://www.marchofdimes.com Tel: 914-997-4488 888-MODIMES (663-4637) Fax: 914-428-8203 |
National Organization for Rare Disorders (NORD) 55 Kenosia Avenue Danbury, CT 06810 orphan@rarediseases.org http://www.rarediseases.org Tel: 203-744-0100 Voice Mail 800-999-NORD (6673) Fax: 203-798-2291 |
National Spinal Cord Injury Association 75-20 Astoria Blvd Suite 120 East Elmhurst, NY 11370-1177 info@spinalcord.org http://www.spinalcord.org Tel: 800-962-9629 Fax: 866-387-2196 |
Paralyzed Veterans of America (PVA) 801 18th Street, NW Washington, DC 20006-3517 info@pva.org http://www.pva.org Tel: 202-USA-1300 (872-1300) 800-555-9140 Fax: 202-785-4452 |
Spina Bifida Association 4590 MacArthur Blvd. NW Suite 250 Washington, DC 20007-4266 sbaa@sbaa.org http://www.spinabifidaassociation.org Tel: 202-944-3285 800-621-3141 Fax: 202-944-3295 |
Spinal Cord Society 19051 County Highway 1 Fergus Falls, MN 56537 scs-nc@nc.rr.com http://scsus.org/ Tel: 218-739-5252 or 218-739-5261 Fax: 218-739-5262 |
Chiari & Syringomyelia Foundation 29 Crest Loop Staten Island, NY 10312 info@CSFinfo.org http://www.csfinfo.org Tel: 718-966-2593 Fax: 718-966-2593 (Call First) |
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National Institute of Neurological Disorders and Stroke
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Last updated February 1, 2012