Visual acuity impairment in patients with retinitis pigmentosa at age 45 years or older

Grover, Sandeep; Fishman, Gerald A.; Anderson, Robert J.; Tozatti, Marcia S.V; Heckenlively, John R.; Weleber, Richard G.; Edwards, Albert O.; Brown, Jeremiah

doi:10.1016/s0161-6420(99)90342-1

Cited by 187 publications

(123 citation statements)

References 12 publications

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“…Two major studies found that 52 to 55 per cent of people with RP had a visual acuity of 6/12 or better in at least one eye, 69 to 73 per cent had a visual acuity of 6/20 or better in at least one eye and 20 to 25 per cent of patients had a visual acuity of 6/60 or worse. 38,39 In summary, the most common final visual acuity level in people with RP was between 6/12 and 6/20.…”

Section: Central Visionmentioning

confidence: 95%

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Retinitis pigmentosa: visual function and multidisciplinary management

Herse

2005

Clinical and Experimental Optometry

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Retinitis pigmentosa (RP) is a leading cause of blindness and visual disability in younger people. Optometrists have a major role in detecting RP and in reducing the visual disability associated with RP. This review summarises the literature relating to visual function in people with RP, with particular attention given to night‐blindness, visual acuity decrease and visual field contraction. The range of low vision aids available for people with RP is reviewed and suggestions given on aids that have been found to be most successful. Most importantly, this review overviews the range of services available to people with RP and emphasises how optometrists need to work with a network of professionals to ensure the best possible visual outcomes for people with RP. Particular mention is made of current findings relating to orientation and mobility training, driving, sensory substitution and adaptive technology. The modern optometrist needs to be aware of the multiple needs of people with RP and have the ability to link them with the professionals best able to help them.

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Section: Central Visionmentioning

confidence: 95%

“…The most common distance visual acuity in people with RP has been reported at about 6/12 to 6/20 38,39 indicating a need for magnification. Spectacle mounted telescopes are not indicated for most people with RP due to the problems of reduced peripheral field and the limitation of scanning.…”

Section: Distance Magnificationmentioning

confidence: 99%

Retinitis pigmentosa: visual function and multidisciplinary management

Herse

2005

Clinical and Experimental Optometry

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“…Counting finger and hand motion were quantified as 2.0 and 2.3 logMAR, 5 and light perception as 2.8 logMAR. 6 KaplanMeier survival analysis was performed using best corrected VA below 20/400 (6/60 or 1.3 logMAR) as threshold. The better eye was used for the analysis of VA as it was thought to best reflect of the effect of the central visual loss on the patient's daily life.…”

Section: Clinical Assessmentmentioning

confidence: 99%

Phenotype and Progression of Retinal Degeneration Associated With Nullizigosity of ABCA4

Fakin

Robson

Fujinami

et al. 2016

Invest. Ophthalmol. Vis. Sci.

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“…Acuity in the better eye was compared between the three most common mutations, all other PRPF8 mutations and with cumulated average data on generic RP obtained from the literature (Marmor 1980, Grover et al 1999 (see Table 4). This suggests that the R2310K mutation is associated with relatively good acuity in all age ranges, by comparison with approximate figures for RP in general, but patients with the H2309P or H2309R mutations appear to have similar or worse visual loss than would be expected for combined RP.…”

Section: Identifiermentioning

confidence: 99%

Prognosis for splicing factor PRPF8 retinitis pigmentosa, novel mutations and correlation between human and yeast phenotypes

et al. 2010

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PRPF8-retinitis pigmentosa is said to be severe but there has been no overview of phenotype across different mutations. We screened RP patients for PRPF8 mutations and identified three new missense mutations, including the first documented mutation outside exon 42 and the first de novo mutation. This brings the known RP-causing mutations in PRPF8 to nineteen. We then collated clinical data from new and published cases to determine an accurate prognosis for PRPF8-RP. Clinical data for 75 PRPF8-RP patients were compared, revealing that while the effect on peripheral retinal function is severe, patients generally retain good visual acuity in at least one eye until the fifth or sixth decade. We also noted that prognosis for PRPF8-RP differs with different mutations, with p.H2309P or p.H2309R having a worse prognosis than p.R2310K. This correlates with the observed difference in growth defect severity in yeast lines carrying the equivalent mutations, though such correlation remains tentative given the limited number of mutations for which information is available. The yeast phenotype is caused by lack of mature spliceosomes in the nucleus, leading to reduced RNA splicing function. Correlation between yeast and human phenotypes suggests that splicing factor RP may also result from an underlying splicing deficit.

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Visual acuity impairment in patients with retinitis pigmentosa at age 45 years or older

Cited by 187 publications

References 12 publications

Retinitis pigmentosa: visual function and multidisciplinary management

Retinitis pigmentosa: visual function and multidisciplinary management

Phenotype and Progression of Retinal Degeneration Associated With Nullizigosity of ABCA4

Prognosis for splicing factor PRPF8 retinitis pigmentosa, novel mutations and correlation between human and yeast phenotypes

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