Nonclassic retinitis pigmentosa: A challenging clinical diagnosis solved by pedigree analysis and electrodiagnostic testing

Optometry. 2010 Apr;81(4):181-7. doi: 10.1016/j.optm.2009.09.022.

Abstract

Purpose: The aim of this study was to describe a case of nonclassic retinitis pigmentosa, to highlight ancillary testing tools for proper diagnosis, and to differentiate between common hereditary fundus dystrophies.

Methods: Methods used in this study included complete ophthalmologic evaluation, optical coherence tomography, visual field testing, pedigree analysis, and electrodiagnostic testing.

Results: Reduced vision and photopsia were the initial complaints of a patient who had an overall normal ocular appearance. However, a strong family history of retinitis pigmentosa and depressed scotopic and photopic electroretinograms confirmed the diagnosis of autosomal dominant retinitis pigmentosa.

Conclusion: In cases of atypical-appearing retinitis pigmentosa, both pedigree analysis and electrodiagnostic testing are fundamental in correct diagnosis of this multifaceted hereditary fundus disorder.

Publication types

  • Case Reports

MeSH terms

  • Electroretinography
  • Humans
  • Male
  • Middle Aged
  • Pedigree
  • Retinitis Pigmentosa / diagnosis*
  • Retinitis Pigmentosa / genetics