Syringomyelia

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.

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.

Prognosis

Symptoms usually begin in young adulthood, with symptoms of one form usually beginning between the ages of 25 and 40.  Symptoms 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.

Research

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.

Organizations

American Chronic Pain Association (ACPA)
Provides self-help coping skills and peer support to people with chronic pain. Sponsors local support groups throughout the U.S. and provides assistance in starting and maintaining support groups.

P.O. Box 850
Rocklin, CA 95677-0850
ACPA@theacpa.org
http://www.theacpa.org
Tel: Rocklin
Fax: 916-652-8190

American Syringomyelia & Chiari Alliance Project (ASAP)
Non-profit organization that works to improve the lives of people with syringomyelia, Chiari malformations, and related disorders. Publishes a newsletter and offers other written information, videotapes, an annual conference, and other services.

P.O. Box 1586
Longview, TX 75606-1586
info@asap.org
http://www.asap.org
Tel: Longview
Fax: 903-757-7456

Christopher and Dana Reeve Foundation
The Reeve Foundation is dedicated to curing spinal cord injury by funding innovative research, and improving the quality of life for people living with paralysis through grants, information and advocacy.

636 Morris Turnpike
Suite 3A
Short Hills, NJ 07078
informations@christopherreeve.org
http://www.christopherreeve.org
Tel: Short Hills
Fax: 973-912-9433

March of Dimes
Works to improve the health of babies by preventing birth defects and infant mortality through programs of research, community services, education, and advocacy.

1275 Mamaroneck Avenue
White Plains, NY 10605
askus@marchofdimes.com
http://www.marchofdimes.com
Tel: White Plains
Fax: 914-428-8203

Content Provided By

NINDS Disorders is an index of neurological conditions provided by the National Institute of Neurological Disorders and Stroke. This valuable tool offers detailed descriptions, facts on treatment and prognosis, and patient organization contact information for over 500 identified neurological disorders.

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