Syringomyelia

Answer 1

Definition

Syringomyelia is damage to the spinal cord due to the formation of a fluid-filled area within the cord.

Alternative Names

Syrinx

Causes, incidence, and risk factors

The fluid buildup 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.

Symptoms

There may be no symptoms, or symptoms may include:

• Gradual loss of muscle mass (wasting, atrophy)
• Headache
• 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:

• Muscle contractions
• Rashes
• Spasms in or tightness of the leg muscles
• Uncoordinated movement

Signs and tests

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

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).

Rarely, an spinal CT with myelogram may be done.

Treatment

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.

It may be necessary to drain the fluid build up. See: Ventriculoperitoneal shunting

Expectations (prognosis)

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.

Complications

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.

Prevention

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

References

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.

Answer 2

Definition

Syringomyelia is damage to the spinal cord due to the formation of a fluid-filled area within the cord.

Alternative Names

Syrinx

Causes, incidence, and risk factors

The fluid buildup 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.

Symptoms

There may be no symptoms, or symptoms may include:

• Gradual loss of muscle mass (wasting, atrophy)
• Headache
• 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:

• Muscle contractions
• Rashes
• Spasms in or tightness of the leg muscles
• Uncoordinated movement

Signs and tests

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

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).

Rarely, an spinal CT with myelogram may be done.

Treatment

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.

It may be necessary to drain the fluid build up. See: Ventriculoperitoneal shunting

Expectations (prognosis)

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.

Complications

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.

Prevention

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

References

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.

Reviewed By

Review Date: 06/16/2010

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.