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Genetic and Rare Diseases Information Center (GARD)

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Syringomyelia


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Your Question

I have syringomyelia. My physician believes that it is congenital, not post-traumatic. What are the different types of syringomyelia and how are they diagnosed? 

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is syringomyelia?

Syringomyelia is a disorder in which a cyst, called a syrinx, forms within the spinal cord. This cyst expands and elongates over time, destroying the center of the spinal cord which can result in pain, weakness, stiffness in the back, shoulders, arms, or legs, headaches, and insensitivity to temperature (especially in the hands). Symptoms vary from person to person. Syringomyelia is often related to a congenital abnormality of the brain called a Chiari I malformation, but may also occur as a complication of trauma, meningitis, hemorrhage, a tumor, or arachnoiditis. Symptoms may appear months or even years after the initial injury. Some cases of syringomyelia are familial, although this is rare. Treatment often involves surgery and avoiding activities that involve straining. Drainage of the cysts using a catheter, drainage tubes, and valves may be required in some patients. If not treated, the disorder can lead to progressive weakness in the arms and legs, loss of hand sensation, and chronic, severe pain.[1]
Last updated: 5/1/2009

Are there different forms of syringomyelia?

Generally, there are two forms of syringomyelia. In most cases, the disorder is related to an abnormality of the brain called a Chiari I malformation. This anatomic abnormality 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. A syrinx may then develop in the cervical region of the spinal cord. Because of the relationship that was once thought to exist between the brain and spinal cord in this type of syringomyelia, physicians sometimes refer to it as communicating syringomyelia. Symptoms usually begin between the ages of 25 and 40 and may worsen with straining or any activity that causes cerebrospinal fluid (CSF) pressure to fluctuate suddenly. Some patients, however, may have long periods of stability. Some patients with this form of the disorder also have hydrocephalus, in which CSF accumulates in the skull, or a condition called arachnoiditis, in which a covering of the spinal cord--the arachnoid membrane--is inflamed.[2]

The second major form of syringomyelia occurs as a complication of trauma, meningitis, hemorrhage, a tumor, or arachnoiditis. Here, the syrinx or cyst develops in a segment of the spinal cord damaged by one of these conditions. The syrinx then starts to expand. This is sometimes referred to as noncommunicating syringomyelia. Symptoms may appear months or even years after the initial injury, starting with pain, weakness, and sensory impairment originating at the site of trauma.[2]

The primary symptom of post-traumatic syringomyelia is pain, which may spread upward from the site of injury. Symptoms, such as pain, numbness, weakness, and disruption in temperature sensation, may occur on one or both sides of the body. Syringomyelia can also adversely affect sweating, sexual function, and, later, bladder and bowel control.[2]

Last updated: 5/1/2009

How is syringomyelia diagnosed?

The initial evaluation of patients suspected of having a spinal cord syrinx includes a comprehensive history and physical examination. Information obtained from examinations guides the imaging studies. Essential tests include plain radiographic series with dynamic views and high-resolution CT scan to assess the bony spinal canal.[3]

Physicians now commonly use magnetic resonance imaging (MRI) to diagnose syringomyelia. The MR imager takes pictures of body structures, such as the brain and spinal cord, in vivid detail. This test will show the syrinx in the spine as well as other conditions, such as the presence of a tumor. MRI is safe, painless, and informative and has greatly improved the diagnosis of syringomyelia.[2] MRI is the most sensitive imaging test for soft tissue. Gadolinium-enhanced images are also helpful in differentiating between tumor, scar, and disk material, especially in postoperative or posttraumatic cases.[3]
Last updated: 5/1/2009

References
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.