Syringomyelia

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Introduction:

  • Syringomyelia is a rare disorder in which a cyst forms within spinal cord
  • As this fluid-filled cyst, or syrinx, expands and lengthens over time, it compresses and damages part of the spinal cord from its center outward.

MC site: Lower cervical cord (Ref.: Harrison 20th)

Causes:

  • Congenital
  • Acquired: Trauma/ TB/ tumor
  • Associations: May be associated with both Chiari type1 & type 2 malformations; but more than half of all cases are associated with Chiari type 1 malformations in which the cerebellar tonsils protrude through the foramen magnum and into the cervical spinal canal.

Characteristic clinical features:

  • Asymmetric selective loss of pain and temperature sensation with sparing of touch and vibration → Dissociated anesthesia (Read more)
  • Painless burn injury to the hand
  • May be associated with Charcot joint (Painless progressive destruction of joint)
  • Cape like distribution of sensory loss

  • At the level of lesion: LMN type of paralysis
  • Below the level of lesion: UMN type of paralysis
  • As the cavity enlarges and compresses the long tracts, spasticity and weakness of the legs, bladder and bowel dysfunction, and a Horner’s syndrome appear.

IOC: MRI

Rx: Decompressive laminectomy.

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