Structural and functional characteristics in carriers of X-linked retinitis pigmentosa with a tapetal-like reflex

Retina. 2010 Nov-Dec;30(10):1726-33. doi: 10.1097/IAE.0b013e3181dde629.

Abstract

Purpose: The purpose of this study was to identify the functional and structural characteristics in three female obligate carriers of X-linked retinitis pigmentosa from the same family by using spectral domain optical coherence tomography, fundus autofluorescence, and microperimetry.

Methods: Three female obligate carriers with a tapetal-like reflex, 21, 49, and 57 years of age, from a single family of X-linked retinitis pigmentosa that was seen in the ophthalmology department at the University of Illinois at Chicago, were enrolled in the study. All carriers underwent a complete ophthalmic examination. Spectral domain optical coherence tomography measurements, a macular microperimetry examination, and fundus autofluorescence testing were performed.

Results: The spectral domain optical coherence tomography examination in all three carriers showed a normal retinal microstructure and thickness. Microperimeter testing showed subnormal retinal sensitivity in the areas of the tapetal-like reflex. Fundus autofluorescence examination showed the presence of speckled areas of enhanced autofluorescence.

Conclusion: Our study demonstrates that the carriers of X-linked retinitis pigmentosa with a tapetal-like reflex can show an enhanced reflectance on infrared images, abnormal autofluorescence properties, elevated retinal thresholds, and a normal retinal morphology within the posterior pole on spectral domain optical coherence tomography testing.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Chromosomes, Human, X / genetics
  • Female
  • Fluorescein Angiography
  • Genetic Diseases, X-Linked / genetics
  • Genetic Diseases, X-Linked / physiopathology*
  • Heterozygote*
  • Humans
  • Middle Aged
  • Pedigree
  • Reflex / physiology*
  • Retina / physiopathology*
  • Retinitis Pigmentosa / genetics
  • Retinitis Pigmentosa / physiopathology*
  • Tomography, Optical Coherence
  • Visual Acuity / physiology
  • Visual Field Tests
  • Visual Fields / physiology
  • Young Adult