Chiari Malformation

Aka: Chiari Malformation, Chiari I Malformation

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II. Definition: Chiari I Malformation

  1. Cerebellar Tonsils fall >5 mm below foramen magnum

III. Pathophysiology

  1. Congenital disorder

IV. Epidemiology

  1. Chiari I symptom onset 30-40 years of age

V. Symptoms (Chiari I is often asymptomatic)

  1. Headaches provoked by straining
  2. Neck Pain
  3. Lower Cranial Nerve disorders
  4. Vertigo
  5. Vertical Nystagmus (down-beating Nystagmus)
  6. Ataxia
  7. Urinary Frequency
  8. Lower extremity spasticity
  9. Dissociated Anesthesia

VI. Differential Diagnosis: Chiari I

  1. Multiple Sclerosis
  2. Bony compression at craniocervical junction
  3. Syringohydromyelia
  4. Syringobulbia

VII. Radiology: Chiari I

  1. Head MRI shows ectopic cerebellar Tonsils, compression

VIII. Management: Chiari I

  1. Decompression of bone at craniocervical junction

IX. References

  1. Behrman (2000) Nelson Pediatrics, Saunders, p. 1810
  2. Goetz (1999) Clinical Neurology, Saunders, p. 530
  3. Goldman (2000) Cecil Medicine, Saunders, p. 2076

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