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Central nervous system
Central nervous system


Syringomyelia

Definition:

Syringomyelia is damage to the spinal cord due to a fluid-filled hole that forms in the cord.



Alternative Names:

Syrinx



Causes, incidence, and risk factors:

The fluid buildup in syringomyelia may be caused by:

  • Birth defects (especially "chiari malformation," in which part of the brain pushes down onto the spinal cord at the base of the skull).
  • Spinal cord trauma
  • Tumors of the spinal cord

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:

  • Headache
  • Loss of muscle mass (wasting , atrophy)
  • Muscle function loss , loss of ability to use arms or legs
  • Numbness or decreased feeling
    • Decreased feeling of pain or temperature
    • Less ability to feel when the skin is being touched
    • Neck, shoulders, upper arms, trunk -- in a cape-like pattern
    • Slowly gets worse over time
  • Pain down the arms, neck, or into the middle back or legs
  • Weakness (decreased muscle strength) in the arms or legs

Other symptoms that may occur with this disease:



Signs and tests:

A nervous system (neurologic) examination may show a loss of feeling or movement caused by pressure on the spinal cord.

Other tests that may be done include:



Treatment:

The goals of treatment are to stop the spinal cord damage from getting worse and to improve function.

Surgery to relieve pressure in the spinal cord may be needed. Physical therapy may be needed to improve muscle function.

The health care provider may need to drain the fluid buildup. See: Ventriculoperitoneal shunting



Support Groups:



Expectations (prognosis):

Without treatment, the disorder gets worse very slowly. Over time it will cause severe disability.

Surgery usually stops the condition from getting worse. About 50% of people will have improvements in nervous system  function after surgery.



Complications:

Without treatment, the condition will lead to:

  • Loss of nervous system function
  • Permanent disability

Possible complications of surgery include:

  • Infection
  • Other complications of surgery


Calling your health care provider:

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



Prevention:

There is no known way to prevent this condition, other than avoiding injuries to the spinal cord. Getting treated right away slows the disorder from getting worse.



References:

Rekate HL. Spinal cord disorders. In: Kliegman RM, Stanton BF, St. Geme J, Schor N, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 598.




Review Date: 5/21/2012
Reviewed By: Luc Jasmin, MD, PhD, Department of Neurosurgery at Cedars-Sinai Medical Center, Los Angeles, and Department of Anatomy at UCSF, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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