Elsevier

Survey of Ophthalmology

Volume 33, Issue 3, November–December 1988, Pages 137-177
Survey of Ophthalmology

Major review
Retinitis pigmentosa

https://doi.org/10.1016/0039-6257(88)90085-9Get rights and content

Abstract

Retinitis pigmentosa is a clinically and genetically heterogeneous group of hereditary disorders in which there is progressive loss of photoreceptor and pigment epithelial function. The prevalence of retinitis pigmentosa is between 13000 and 15000 making it one of the most common causes of visual impairment in all age groups. The natural history, differential diagnosis, diagnostic clinical and electrophysiologic findings are reviewed. Generalizations about the different genetic subtypes of retinitis pigmentosa are reviewed along with the uses of DNA probes for linkage studies. Syndromes in which retinitis pigmentosa is a manifestation are summarized.

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      Citation Excerpt :

      The criteria for clinical diagnosis of AIR were as follows: (1) presence of symptoms such as acute or subacute visual loss, photopsia, scotoma, dyschromatopsia, or nyctalopia; (2) decreased amplitudes on ERG compared with normative data with or without visual field defects; (3) absence of overt intraocular inflammation; and (4) no evidence of other retinal diseases such as IRD that may explain visual loss.1,3,5,6 RP was diagnosed clinically with reduced ERG responses, constriction of the visual field, retinal degeneration and pigmentation, retinal vascular narrowing, and visual disturbance that progressed slowly with or without genetic abnormalities.9 The normal controls had no eye disease and previous history of ocular surgery and had best-corrected visual acuity (BCVA) of 0.15 logarithm of the minimal angle of resolution (logMAR) or better.

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