X-linked retinitis pigmentosa. Profile of clinical findings

Arch Ophthalmol. 1988 Mar;106(3):369-75. doi: 10.1001/archopht.1988.01060130395029.


An evaluation of 56 patients with X-linked retinitis pigmentosa revealed a profile of findings that include the following: night blindness within the first two decades of life; spherical refractive errors of -2.00 diopters or greater in addition to an increased prevalence of a cylindrical correction of +1.50 diopters or greater; appreciable impairment of central visual acuity to 20/200 or less by the fifth decade of life; characteristic patterns of field loss; presence of a foveal lesion in up to 75% of the study group; posterior subcapsular lens opacities; and nondetectable electroretinographic amplitudes in more than two thirds of the patients (using conventional full-field recording procedures). These observations are of general value in diagnosis of this disease and for counseling of patients afflicted with this severe form of hereditary night blindness.

Publication types

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

MeSH terms

  • Actuarial Analysis
  • Adult
  • Cataract / complications
  • Child
  • Child, Preschool
  • Electroretinography
  • Fluorescein Angiography
  • Fovea Centralis / pathology
  • Fundus Oculi
  • Genetic Linkage*
  • Humans
  • Male
  • Middle Aged
  • Night Blindness / complications
  • Refractive Errors / complications
  • Retinitis Pigmentosa / complications
  • Retinitis Pigmentosa / genetics*
  • Retinitis Pigmentosa / pathology
  • Retinitis Pigmentosa / physiopathology
  • Visual Acuity
  • Visual Fields
  • X Chromosome*