Syringomyelia
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.
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.
What are the symptoms?
Damage from Syringomyelia 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.
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 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.
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. 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.
References
Adapted with permission from the National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health, Bethesda, MD 20892, USA.
Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.
Last updated August 5, 2014