Perceived and actual performance of daily tasks: relationship to visual function tests in individuals with retinitis pigmentosa11The authors have no proprietary interest in this study.

Szlyk, Janet P.; Seiple, William; Fishman, Gerald A.; Alexander, Kenneth R.; Grover, Sandeep; Mahler, Carolyn L

doi:10.1016/s0161-6420(00)00413-9

Cited by 107 publications

(100 citation statements)

References 8 publications

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“…Expectations may need revision, but there is still hope that some useful cone photoreceptor-mediated central vision will be restored in this serious eye disease. Even a limited, partly functional central island of (cone) vision would be a boon for mobility and the tasks of everyday life (44).…”

Section: Discussionmentioning

confidence: 99%

Human cone photoreceptor dependence on RPE65 isomerase

Jacobson

Alemán

Cideciyan

et al. 2007

Proc. Natl. Acad. Sci. U.S.A.

138

144

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The visual (retinoid) cycle, the enzymatic pathway that regenerates chromophore after light absorption, is located primarily in the retinal pigment epithelium (RPE) and is essential for rod photoreceptor survival. Whether this pathway also is essential for cone photoreceptor survival is unknown, and there are no data from man or monkey to address this question. The visual cycle is naturally disrupted in humans with Leber congenital amaurosis (LCA), which is caused by mutations in RPE65, the gene that encodes the retinoid isomerase. We investigated such patients over a wide age range (3-52 years) for effects on the cone-rich human fovea. In vivo microscopy of the fovea showed that, even at the youngest ages, patients with RPE65-LCA exhibited cone photoreceptor loss. This loss was incomplete, however, and residual cone photoreceptor structure and function persisted for decades. Basic questions about localization of RPE65 and isomerase activity in the primate eye were addressed by examining normal macaque. RPE65 was definitively localized by immunocytochemistry to the central RPE and, by immunoblotting, appeared to concentrate in the central retina. The central retinal RPE layer also showed a 4-fold higher retinoid isomerase activity than more peripheral RPE. Early cone photoreceptor losses in RPE65-LCA suggest that robust RPE65-based visual chromophore production is important for cones; the residual retained cone structure and function support the speculation that alternative pathways are critical for cone photoreceptor survival.optical coherence tomography ͉ retinal degeneration ͉ retinoid cycle ͉ Leber congenital amaurosis ͉ gene therapy V ertebrate retinas have two types of photoreceptors: rods for the perception of dim light and cones for the perception of bright light. Light absorbed by visual pigments of photoreceptors triggers a series of enzymatic reactions known as phototransduction (1). The light-capturing part of the visual pigment, the chromophore, must then be regenerated. The pathway for regeneration of rhodopsin (the rod visual pigment) is known as the visual (retinoid) cycle (2, 3) and involves both rod cells and the adjacent retinal pigment epithelium (RPE). All-trans-retinal is reisomerized to 11-cis-retinal by a reaction in the RPE that requires the retinal isomerase, retinal pigment epithelium-specific 65-kDa protein (RPE65), and lecithinretinol acyltransferase (LRAT) activities. Functional visual pigment is then reformed by combining the 11-cis-chromophore with the photoreceptor opsin apoprotein (4). Molecular knowledge of the visual cycle has increased as the genes encoding the enzymes of this pathway have been elucidated (2, 5, 6).Decades ago, cone pigment regeneration was proposed to be RPE cell-independent (7, 8). More recently, cone-dominant ground squirrel and chicken retinas have been shown to exhibit retinoid isomerase activity that is distinct from RPE65 (5, 9, 10). Murine double knockout experiments, however, provide evidence that RPE65 and the RPE are essential for cone function (11...

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Section: Discussionmentioning

confidence: 99%

Human cone photoreceptor dependence on RPE65 isomerase

Jacobson

Alemán

Cideciyan

et al. 2007

Proc. Natl. Acad. Sci. U.S.A.

138

144

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“…More recently there is a coming up of trend in developing a method for evaluation of functional vision-performance-based measures (PBMs) of visual function. [13][14][15][16][17][18][19][20][21][22][23] It involves presenting patients with commonly encountered tasks of daily life in a standardized format and getting a grade in accordance with their visual ability to complete the tasks. Pioneering studies has demonstrated that PBMs can be reliable and valid methods in assessing real-life visual ability.…”

Section: Introductionmentioning

confidence: 99%

“…Pioneering studies has demonstrated that PBMs can be reliable and valid methods in assessing real-life visual ability. [13][14][15][16][17][18][19][20][21][22][23] Owing to its significance in visual hierarchy, PBMs of visual function is becoming an important part of researches on the real-life effects of visual diseases.…”

Section: Introductionmentioning

confidence: 99%

Impact of cataract surgery on vision-related life performances: the usefulness of Real-Life Vision Test for cataract surgery outcomes evaluation

Hou

et al. 2015

Eye

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Purpose Real-Life Vision Test (RLVT) is a newly developed performance-based measures of functional vision. This present study is designed to determine whether it could be a meaningful assessment for cataract surgery outcomes evaluation. Patients and methods Age-related cataract patients (56) who scheduled for bilateral cataract surgery and 44 age-matched controls were evaluated by four types of measurements: (1) demographic, medical, cognitive and depressive evaluation, and the reaction time testing; (2) clinical measures (visual acuity, contrast sensitivity, stereopsis, and color perception); (3) the 25-item National Eye Institute's Visual Functioning Questionnaire (NEI-VFQ); (4) the RLVT. Spearman's coefficients and multiple regression analysis were conducted to investigate the relationship among RLVT, clinical measures, and self-report assessment of visual function. Results The results of RLVT, clinical measures, and NEI-VFQ total scores were improved significantly after cataract surgery. There were no differences between control subjects and post-surgery patients with respect to NEI-VFQ-25 total scores, self-rating depression scale scores and three tasks of RLVT. Change of RLVT was significantly associated with the change of clinical measures in the cataract group. Multiple regression analysis demonstrated that change of distance, intermediate, and near visual acuity, and binocular contrast sensitivity were significant predictors of improvements of RLVT.Conclusions Cataract surgery could improve real-world visual ability effectively for cataract patients. Our study highlights the potential usefulness of RLVT as an adjunct to the current outcomes evaluation system for cataract surgery. The use of RLVT combined with clinical and self-survey methods may be the comprehensive strategy to manifest the impact of cataract surgery on patients' overall vision-related quality of life.

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“…2,10,17 Based on the inherent limitations of clinical tests and self-report questionnaires, more recently there has been an upcoming interest in developing a complementary method for evaluation and outcome measures of visual function-the performancebased measures (PBMs) of visual function. 4,10,13,[18][19][20][21][22][23][24][25][26][27] Vision-specific PBMs take on actual performance of vision-related tasks. It involves presenting patients with commonly encountered tasks of daily life in a standardized format and getting a grade in accordance to their visual ability to complete the tests.…”

Section: Introductionmentioning

confidence: 99%

“…13,18,20,[23][24][25][26] However, little research has been done on Chinese population so far. Because PBMs are designed to understand how patients react in real-life activities, the 'tasks' that we selected should cover the most usual activities in daily life.…”

Section: Introductionmentioning

confidence: 99%

Using the real-life vision test to assess the functional vision of age-related cataract patients

et al. 2012

Eye

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Purpose (1) To describe and validate a newly developed, timed performance-based measures of functional vision-the real-life vision test (RLVT). (2) To determine how RLVT relates to clinical measures and selfreport assessment of visual function and the complex interactions among visual impairment, psychosocial status, and demographic factors. Methods A total of 64 patients with age-related cataract and 45 age-matched controls were evaluated by four types of measurements: (1) demographic, medical, cognitive, and depressive evaluation and the reaction time (RT) testing; (2) clinical measures (visual acuity, contrast sensitivity, stereopsis, and the color perception); (3) the 25-item National Eye Institute's Visual Functioning Questionnaire; and (4) the RLVT. Spearman's coefficients, partial correlation, and multiple regression analysis were conducted to determine the relationship among RLVT, clinical measures, and selfreport assessment of visual function while controlling for confounders. Results Control subjects performed RLVT significantly better than the cataract patients. RLVT correlated well with both clinical and self-report assessments of visual function. All subscales of RLVT remained highly associated with most of the clinical measures, even after adjusting for age, years of education, depression, cognitive status, and the RT. Distance, intermediate and near visual acuity, and binocular contrast sensitivity were significant predictors of the RLVT performances. Conclusions Given the strong relationship among RLVT, clinical measures, and the self-report assessments, our results highlight the potential usefulness of RLVT for assessing the functional vision of cataract patients. RLVT may provide information not obtainable from clinical measures or surveys and therefore it is essential to be incorporated into future ophthalmological practice.

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Perceived and actual performance of daily tasks: relationship to visual function tests in individuals with retinitis pigmentosa11The authors have no proprietary interest in this study.

Cited by 107 publications

References 8 publications

Human cone photoreceptor dependence on RPE65 isomerase

Human cone photoreceptor dependence on RPE65 isomerase

Impact of cataract surgery on vision-related life performances: the usefulness of Real-Life Vision Test for cataract surgery outcomes evaluation

Using the real-life vision test to assess the functional vision of age-related cataract patients

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