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Gaze evoked amaurosis in neurofibromatosis type II
  1. L SMITH,
  2. A KRISS,
  1. Department of Ophthalmology, Great Ormond Street Hospital NHS Trust, London WC1N 3JH
  1. Miss L F F Smith.

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Editor,—The electroretinogram (ERG) and visual evoked potential (VEP) to pattern reversal stimulation became degraded in association with visual loss in a young patient with gaze evoked amaurosis who had a mass at the apex of the left orbit. His visual symptoms improved following left orbital decompression and Snellen acuity was maintained at 6/6; however, the pattern VEP was attenuated and degraded compared with before surgery.


A 9 year old boy with neurofibromatosis type II initially presented with a history of intermittent visual disturbance affecting the left eye when in left gaze. The right eye had a retinal hamartoma and a visual acuity of 6/12. He had undergone previous surgery to correct a right divergent squint. A partial third nerve palsy on the left side was present.

On examination, visual acuity of the left eye was 6/6 when viewing in the primary position, and pupillary reactions were normal. However, acuity became perception of light on sustained left lateral gaze, and a mild relative afferent pupillary defect became detectable at …

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