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Syringomyelia

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Syringomyelia, Syringobulbia, Syringohydromyelia, Syrinx

  • Definitions
  1. Syringe
    1. Fluid-filled cavity
  2. Syringomyelia or Syrinx
    1. Syringe forms at inner portion of spinal cord, forming an expanding central cavity
    2. Chronic progressive spinal cord degeneration
  3. Syringobulbia
    1. Syringomyelia extending into Brainstem
  • Epidemiology
  1. Symptom onset in second and third decade
  • Pathophysiology
  1. Results from alterations in CSF flow
  2. Cervical spinal cord most often affected
  • Causes
  1. Chiari I Malformation (accounts for 90% of cases)
  2. Foramen magnum narrowing
  3. Achondroplasia
  4. Intraspinal tumors
  5. Subarachnoid scarring
    1. Traumatic spine injury (e.g. Whiplash) or Hemorrhage
    2. Spinal Infection
  • Symptoms and signs
  1. Sensory dissociation in neck, arms and upper trunk
    1. Touch and position sense preserved
    2. Pain and Temperature sense lost (Spinothalamic Tract)
  2. Anterior horn cell involvement
    1. Upper extremity changes
      1. Asymmetric muscle atrophy, weakness of hands, arms (esp. intraosseous muscles)
      2. Fasciculations
      3. Deep Tendon Reflexes absent in upper extremities
    2. Lower extremity
      1. Increased muscle tone
      2. Hyperactive Deep Tendon Reflexes
  3. Corticospinal tract involvement
    1. Muscle spasticity
  4. Spinal sympathetic fiber involvement
    1. Trophic changes
  • Associated conditions
  • Spinal cord disorders
  1. Scoliosis
  2. Vertebral fusions
  3. Platybasia
  • Diagnosis
  1. MRI Spine
    1. Defines extent of Syrinx
  2. Electromyography (EMG)
    1. Denervation
  3. Lumbar Puncture
    1. CSF usually normal
  4. Nerve conduction
    1. Normal
  • Management
  1. Treat underlying cause (e.g. Chiari Malformation)
  • Patient Resources
  1. American Syringomyelia Alliance Project
    1. http://www.asap.org
    2. Phone: 1-800-272-7282
    3. Address: P.O. Box 1586, Longview, Texas, 75606
  • References
  1. Girolami in Cotran (1999) Robbins Pathology, P. 1361
  2. Barkovich in Goldman (2000) Cecil Medicine, p. 2077