Syringomyelia

Alternative names

Syrinx

Definition

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

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.

Syringomyelia may occur in certain developmental abnormalities of the nervous system, including Chiari malformations.

Symptoms

• Numbness or decreased sensation
  o Neck, shoulders, upper arms, trunk in a cape-like distribution
  o Slowly, but progressively worsens
  o Lessened ability to sense that the skin is being touched
  o 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:

• Rashes
  
• Muscle contractions
  
• Uncoordinated movement
  
• Spasticity
  

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)