Syringomyelia

Definition:

Syringomyelia is damage to the spinal cord, caused by a formation of a fluid-filled cavity within the cord.

Alternative Names:

Syrinx

Causes, incidence, and risk factors:

Syringomyelia is caused by a formation of a fluid-filled cavity within the spinal cord. This fluid build-up may be a result of spinal cord trauma, tumors of the spinal cord, or congenital defects.

The cavity most often begins in the neck area. It expands slowly, causing progressive damage to the spinal cord due to the pressure exerted by the fluid. Symptoms result from the spinal cord damage.

Symptoms:

Numbness or decreased sensation
- Neck, shoulders, upper arms, trunk in a cape-like distribution
- Slowly, but progressively worsens
- Lessened ability to sense that the skin is being touched
- Decreased sense of pain or temperature
Weakness (decreased muscle strength, independent of exercise) in the arms or legs
Gradual loss of muscle mass (wasting, atrophy)
Muscle function loss, loss of ability to use arms or legs
Headache

Additional symptoms that may be associated with this disease:

Signs and tests:

Neurologic examination may show loss of sensation or movement caused by compression of the spinal cord.

A spinal CT with myelogram or 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).

Treatment:

The goals of treatment are to stop progression of spinal cord damage and to maximize functioning. Surgical decompression may be appropriate, if there is an identifiable mass compressing the spinal cord. Physical therapy may be needed to maximize muscular function.

Expectations (prognosis):

Untreated, the disorder is very slowly progressive, but it eventually results in 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.

Complications:

Postoperative infection
Other surgical complications
Continued or progressive loss of neurologic function
Permanent disability

Calling your health care provider:

Call your health care provider if symptoms indicating syringomyelia may be present.

Prevention:

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

Review Date: 7/28/2002
Reviewed By: Elaine T. Kiriakopoulos, M.D., MSc, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard University, Boston, MA. Review provided by VeriMed Healthcare Network.

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