Spinal Cord And Root Compression

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Pathogenesis

The exact cause of this condition remains unknown but theories abound. In 1965, Gardner proposed the 'hydrodynamic theory', suggesting that the craniovertebral anomaly may impair CSF outflow from the 4th ventricle to the cisterna magna. This in turn was believed to result in transmission of a CSF arterial pulse wave through a patent central canal, dilating the canal below the level of compression. This theory, however, does not explain the occurrence of syringomyelia in patients with nonpatent central canals. Most now attribute the formation of the syrinx to pressure changes transmitted through the epidural veins to the spinal canal during coughing or straining. In the presence of an obstructive element these pressure changes appear to force CSF into the cord substance. This develops into a cystic cavity which progressively extends.

Arnold-Chiari malformation with cerebellar tonsils impacted in the foramen magnum.

Arnold-Chiari malformation with cerebellar tonsils impacted in the foramen magnum.

Syrinx containing fluid identical to CSF

Clinical features

- Dissociated sensory loss (i.e. loss of pain and temperature sensation with retention of other senses) occurring in a cape-like distribution. Painless burns are a classic sign.

- Wasting and weakness of the small muscles of the hand and winging of the scapula from anterior horn cell involvement. Scoliosis often results.

- Long tract signs follow.

- Brain stem signs may appear, either from syringobulbia or an associated Chiari malformation.

- Hydrocephalus occurs in 25% but is usually asymptomatic.

Syrinx containing fluid identical to CSF

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