Cerebellum

Chiari Malformation

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Chiari Malformation, Chiari I Malformation

  • Definition
  • Chiari I Malformation
  1. Cerebellar Tonsils fall >5 mm below foramen magnum
  • Pathophysiology
  1. Congenital disorder
  • Epidemiology
  1. Chiari I symptom onset 30-40 years of age
  • 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
  • Differential Diagnosis
  • Chiari I
  1. Multiple Sclerosis
  2. Bony compression at craniocervical junction
  3. Syringohydromyelia
  4. Syringobulbia
  • Radiology
  • Chiari I
  1. Head MRI shows ectopic cerebellar Tonsils, compression
  • Management
  • Chiari I
  1. Decompression of bone at craniocervical junction
  • 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