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Chiari I malformation presenting as downbeat nystagmus: Clinical presentation, diagnosis, and management

Optometry - Journal of the American Optometric Association
DOI: 10.1016/j.optm.2011.09.012
  • Downbeat Nystagmus
  • Chiari Malformation
  • Oscillopsia
  • Medicine


Abstract Chiari I malformation is a congenital, neurologic condition characterized by defects of the skull base resulting in herniation of the cerebellum through the foramen magnum into the cervical spinal canal. Because the condition can result in visual symptoms, patients often will search for answers from their eye care providers. Case Report A 28-year-old Hispanic diabetic man with a 10-year history of nystagmus was referred to the neuro-ophthalmic disease clinic after initiation of oscillopsia 1 year previous. Downbeat nystagmus, which worsened in right and down gaze, was evident. Cranial nerve testing was unremarkable, but the patient did report trouble with choking on food and drink. Neuroimaging found Chiari I malformation. The patient underwent a suboccipital craniectomy, which resulted in lessened nystagmus and improved symptoms. Discussion The majority of patients with Chiari I malformation have an onset of symptoms in the second or third decade of life. Most commonly, a suboccipital headache that worsens with Valsalva maneuver is present. Visual symptoms include retro-orbital pain, flashing lights or floaters, blurred vision, photophobia, diplopia, transient vision loss, and peripheral vision loss. Objective evidence is often lacking in these patients; however, horizontal or vertical nystagmus is present in up to 45% of those with Chiari I malformation. Surgery has proven to be an effective and safe method to treat symptomatic Chiari I malformation.

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