Syringomyelia is damage to the spinal cord due to the formation of a fluid-filled area within the cord.
Causes, incidence, and risk factors:
The fluid build-up 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.
- Gradual loss of muscle mass (wasting , atrophy)
- Muscle function loss , loss of ability to use arms or legs
- Numbness or decreased sensation
- Decreased sense of pain or temperature
- Lessened ability to sense that the skin is being touched
- Neck, shoulders, upper arms, trunk -- in a cape-like pattern
- 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:
Signs and tests:
A 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).
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.
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.
Without treatment, the condition will lead to:
- Continued or progressive loss of neurologic function
- Permanent disability
Possible complications of surgery include:
- Postoperative infection and other complications common to all surgeries
Calling your health care provider:
Call your health care provider if you have symptoms of syringomyelia.
There is no known prevention, other than avoiding trauma to the spinal cord. Prompt treatment reduces progression of the disorder.
Feske SK, Cochrane, TI. Degenerative and compressive structural disorders. In: Goetz, CG, ed. Textbook of Clinical Neurology. 3rd ed. Philadelphia, PA: Saunders Elsevier;2007:chap 29.
Golden JA, Bonnemann CG. Etiological Categories of Neurological Diseases. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, PA: Saunders Elsevier;2007:chap 28, part 3.
|Review Date: 6/19/2008|
Reviewed By: Daniel B. Hoch, PhD, MD, Assistant Professor of Neurology, Harvard Medical School, Department of Neurology, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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