X-linked retinitis pigmentosa. Profile of clinical findings.
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.
Fishman
G A
GA
Department of Ophthalmology, Eye and Ear Infirmary, University of Illinois College of Medicine 60612.
Farber
M D
MD
Derlacki
D J
DJ
D000206:Actuarial Analysis; D000328:Adult; D002386:Cataract / Q000150:complications; D002648:Child; D002675:Child, Preschool; D004596:Electroretinography; D005451:Fluorescein Angiography; D005584:Fovea Centralis / Q000473:pathology; D005654:Fundus Oculi; D008040:Genetic Linkage; D006801:Humans; D008297:Male; D008875:Middle Aged; D009755:Night Blindness / Q000150:complications; D012030:Refractive Errors / Q000150:complications; D012174:Retinitis Pigmentosa / Q000150:complications / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D014792:Visual Acuity; D014794:Visual Fields; D014960:X Chromosome