http://www.ninds.nih.gov/disorders/syringomyelia/syringomyelia.

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NINDS Syringomyelia Information Page

What is 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.

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. 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. http://adam.about.net/encyclopedia/infectiousdiseases/Syringomyelia.htm

Syringomyelia

Definition

Syringomyelia is damage to the spinal cord due to the formation of a fluid-filled area within the cord.
Alternative Names

Syrinx
Causes

The fluid buildup seen in syringomyelia may be a result of spinal cord trauma, tumors of the spinal cord, or birth defects (specifically, "chiari malformation," in which part of the brain pushes down onto the spinal cord at the base of the skull). The fluid-filled cavity usually begins in the neck area. It expands slowly, putting pressure on the spinal cord and slowly causing damage.
Symptoms

There may be no symptoms, or symptoms may include:

y y y y

Gradual loss of muscle mass (wasting, atrophy) Headache Muscle function loss, loss of ability to use arms or legs Numbness or decreased sensation o Decreased sense of pain or temperature o Lessened ability to sense that the skin is being touched o Neck, shoulders, upper arms, trunk -- in a cape-like pattern o Slowly, but progressively, gets worse Pain down the arms, neck, or into the upper back

Weakness (decreased muscle strength, independent of exercise) in the arms or legs

Additional symptoms that may be associated with this disease:

y y y y

Muscle contractions Rashes Spasms in or tightness of the leg muscles Uncoordinated movement

Exams and Tests

A neurologic examination may show loss of sensation or movement caused by compression of the spinal cord. An MRI of the spine confirms syringomyelia and determines the exact location and extent. Often, an MRI of the head will be done to look for associated conditions including hydrocephalus (water on the brain). Rarely, an spinal CT with myelogram may be done.
Treatment

The goals of treatment are to stop the spinal cord damage from getting worse and to maximize functioning. Surgery to relieve pressure in the spinal cord may be appropriate. Physical therapy may be needed to maximize muscular function. It may be necessary to drain the fluid build up. See: Ventriculoperitoneal shunting
Outlook (Prognosis)

Untreated, the disorder gets worse very slowly, but it eventually causes severe disability. Surgical decompression usually stops the progression of the disorder, with about 50% of people showing significant improvement in neurologic function after surgical decompression.
Possible Complications

Without treatment, the condition will lead to:

y y

Continued or progressive loss of neurologic function Permanent disability

Possible complications of surgery include:

y

Postoperative infection and other complications common to all surgeries

When to Contact a Medical Professional

Call your health care provider if you have symptoms of syringomyelia.

Prevention

There is no known prevention, other than avoiding trauma to the spinal cord. Prompt treatment reduces progression of the disorder.