Full-field electroretinograms in individuals with the Laurence-Mood-Bardet-Biedl syndrome

Acta Ophthalmol Scand. 1996 Dec;74(6):618-20. doi: 10.1111/j.1600-0420.1996.tb00747.x.


Purpose: To evaluate rod and cone function in individuals with the Laurence-Moon-Bardet-Biedl syndrome.

Methods: We obtained a full-field electroretinograms in 36 patients. If responses less than 10 microV were recorded with single white flashes a special techniques with narrow band filter and computer averaging was used.

Results: No rod responses to dim blue light could be obtained in any of the patients. Residual cone flicker responses were measurable in 28 of the individuals. Those with amplitudes < 0.05 microV were significantly older than those with amplitudes > 1.00 microV. The ERG pattern was consistent within affected pairs of siblings in 8 families.

Conclusion: The retinal dystrophy in Laurence-Moon-Bardet-Biedl syndrome is primarily a rod-cone dystrophy, but even cone flicker amplitudes are severely reduced with further progression with age. There is no intrafamilial variability of the electroretinograms in affected siblings.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Dark Adaptation / physiology
  • Electroretinography*
  • Female
  • Humans
  • Laurence-Moon Syndrome / genetics
  • Laurence-Moon Syndrome / physiopathology*
  • Male
  • Middle Aged
  • Photoreceptor Cells / physiology*
  • Retinal Degeneration / genetics
  • Retinal Degeneration / physiopathology*
  • Visual Acuity
  • Visual Fields