What is Syringomyelia?
Syringomyelia 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.
Is there any treatment?
Surgery is usually recommended for syringomyelia patients. Recurrence of
syringomyelia after surgery may make additional operations necessary; these may
not be completely successful over the long term.
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.
What is the prognosis?
Symptoms usually begin between the ages of 25 and 40 and may worsen with
straining or any activity that causes cerebrospinal fluid pressure to fluctuate.
Some patients, however, may have long periods of stability. Surgery results in
stabilization or modest improvement in symptoms for most patients. Delay in
treatment may result in irreversible spinal cord injury.
What research is being done?
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. contrast dyes.
Patients can expect even better techniques to become available in the future
from the research efforts of scientists today.
Organizations
American Chronic Pain Association (ACPA)
P.O. Box 850
Rocklin, CA 95677-0850
ACPA@pacbell.net
www.theacpa.org
Tel: 916.632.0922 800.533.3231
Fax: 916.652.8190
American Syringomyelia Alliance Project (ASAP)
P.O. Box 1586
Longview, TX 75606-1586
info@asap.org
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
info@christopherreeve.org
www.christopherreeve.org
Tel: 973.379.2690 800.225.0292
Fax: 973.912.9433
March of Dimes Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
askus@marchofdimes.com
www.marchofdimes.com
Tel: 914.428.7100 888.MODIMES (663.4637)
Fax: 914.428.8203
National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT 06813-1968
orphan@rarediseases.org
www.rarediseases.org
Tel: 203.744.0100 Voice Mail 800.999.NORD (6673)
Fax: 203.798.2291
National Spinal Cord Injury Association
1 Church Street, #600
Rockville, MD 20850
info@spinalcord.org
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
www.pva.org
Tel: 202.USA.1300 (872.1300) 800.424.8200
Fax: 202.785.4452
Spina Bifida Association of America
4590 MacArthur Blvd. NW, Suite 250
Washington, DC 20007-4266
sbaa@sbaa.org
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
www.scsus.org
Tel: 218.739.5252 or 218.739.5261
Fax: 218.739.5262
Source: National Institutes of Health; The National Institute of Neurological Disorders and Stroke
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