Syringomyelia

Overview

Normally, the spinal cord and brain are bathed in a fluid called cerebrospinal fluid. Syringomyelia is an accumulation of spinal fluid that actually forms within the spinal cord. This accumulation is called a syrinx. Most of the time, syringomyelia is caused by Chiari malformations, which is a birth defect of the back and lower part of the brain. Sometimes syringomyelia is caused by hydrocephalus, tethered spinal cord, tumor or other rare causes.

Signs & symptoms

The symptoms of syringomyelia are very variable, and often progress slowly over years. Classic findings include:

• Scoliosis
• Sensory loss in a “cape” distribution (over the tops of the shoulders); loss of pain and temperature sensation without loss of touch and position sense
• Neck pain and pain in the back of the head
• Pain in legs and arms, and hands and feet
• Hand and arm weakness and loss of muscle function
• High muscle tone in the arms and legs (spasticity)
• Sleep apnea

Diagnosis

Syringomyelia is diagnosed with MRI. Small syrinxes or hydromyelia in the spinal cord can be a normal finding.

Treatment

Large syringomyelia requires surgical treatment. The treatment is directed at the cause of the syrinx, such as Chiari I malformation, or Tethered cord, rather than the syrinx itself.