Syringomyelia

Aka: Syringomyelia, Syringobulbia, Syringohydromyelia, Syrinx

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II. 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

III. Epidemiology

  1. Symptom onset in second and third decade

IV. Pathophysiology

  1. Results from alterations in CSF flow
  2. Cervical spinal cord most often affected

V. Causes

  1. Chiari I Malformation (accounts for 90% of cervical Syringomyelia 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

VI. Symptoms

  1. Back Pain
  2. Back and Shoulder stiffness
  3. Provocative
    1. Postural Change
    2. Valsalva Maneuver

VII. 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

VIII. Associated conditions: Spinal cord disorders

  1. Scoliosis
  2. Vertebral fusions
  3. Platybasia

IX. Diagnosis

  1. Contrast-Enhanced MRI Spine
    1. Syrinx will appear as fluid-filled, gliosis lined cavity
    2. Defines extent of Syrinx (between C2 and T1)
    3. Identifies associated congenital abnormalities
  2. Electromyography (EMG)
    1. Denervation
  3. Lumbar Puncture
    1. CSF usually normal
  4. Nerve Conduction
    1. Normal

X. Management

  1. Neurosurgery Consultation (urgent if functional decline)
  2. Treat underlying cause (e.g. Chiari Malformation)

XI. 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

XII. References

  1. Girolami in Cotran (1999) Robbins Pathology, P. 1361
  2. Barkovich in Goldman (2000) Cecil Medicine, p. 2077
  3. Achar (2020) Am Fam Physician 102(1):19-28 [PubMed]

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