Supernormal scotopic ERG in cone dystrophy

Br J Ophthalmol. 1984 Feb;68(2):69-78. doi: 10.1136/bjo.68.2.69.

Abstract

Three patients with a bull's-eye macular lesion and other signs characteristic of cone dystrophy gave an unusual ERG finding. In response to a white flash of moderate intensity the scotopic b-wave amplitude was considerably larger than normal. One patient had elevated rod thresholds and nyctalopia, while the other 2 had normal rod sensitivity associated with the supernormal scotopic b-wave amplitude. In the latter 2 patients the abnormal ERG pattern was unchanged for 4 years and 7 years respectively. This atypical finding, of a supernormal scotopic b-wave amplitude in response to light of moderate intensity, appears to characterise a subgroup of patients with cone dystrophy, probably of autosomal recessive inheritance. The pathogenesis of the abnormal ERG remains uncertain.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adaptation, Ocular
  • Adult
  • Electroretinography*
  • Female
  • Fluorescein Angiography
  • Humans
  • Light
  • Macular Degeneration / complications
  • Macular Degeneration / diagnosis*
  • Macular Degeneration / physiopathology
  • Night Blindness / complications
  • Night Blindness / physiopathology
  • Photoreceptor Cells / physiopathology*
  • Sensory Thresholds / physiology