Low Luminance Visual Acuity and Low Luminance Deficit in Choroideremia and<i>RPGR</i>-Associated Retinitis Pigmentosa

Wood, Laura J.; Jolly, Jasleen K; Josan, Amandeep S.; Buckley, Thomas M W; MacLaren, Robert E.

doi:10.1167/tvst.10.2.28

Cited by 11 publications

(16 citation statements)

References 35 publications

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“…19 Loss of VA under low-luminance conditions is reported to precede loss of BCVA in RP. 20 This may be due to LLVA being dependent on the preserved function of a larger central macular area than standard BCVA. 20 Because of the relatively slow rate of decline in BCVA as XLRP progresses, a treatment benefit in LLVA might be observed earlier than a benefit in BCVA.…”

Section: Discussionmentioning

confidence: 99%

“…20 This may be due to LLVA being dependent on the preserved function of a larger central macular area than standard BCVA. 20 Because of the relatively slow rate of decline in BCVA as XLRP progresses, a treatment benefit in LLVA might be observed earlier than a benefit in BCVA. Improvements in LLVA were observed in dosed eyes of participants in the XIRIUS trial as early as month 1 and were sustained through 12 months of follow-up.…”

Section: Discussionmentioning

confidence: 99%

“…LLVA is a measure of mesopic visual function at low light levels . Loss of VA under low-luminance conditions is reported to precede loss of BCVA in RP . This may be due to LLVA being dependent on the preserved function of a larger central macular area than standard BCVA .…”

Section: Discussionmentioning

confidence: 99%

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Changes in Retinal Sensitivity Associated With Cotoretigene Toliparvovec in X-Linked Retinitis Pigmentosa With RPGR Gene Variations

et al. 2023

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ImportanceX-linked retinitis pigmentosa (XLRP) is a severe cause of early-onset RP in male individuals, characterized by degeneration of photoreceptors, an extinguished electroretinogram, and vision loss.ObjectiveTo assess the duration of improvements in retinal sensitivity associated with a single, subretinal injection of cotoretigene toliparvovec (BIIB112/AAV8-RPGR) gene therapy after vitrectomy surgery in the dosed eye over 12 months in part 1 of the Clinical Trial of Retinal Gene Therapy for X-linked Retinitis Pigmentosa Using BIIB112 (XIRIUS) study, compared with untreated fellow eyes and eyes from the untreated subgroup from the Natural History of the Progression of X-Linked Retinitis Pigmentosa (XOLARIS) study.Design, Setting, and ParticipantsThis was a post hoc analysis of the XIRIUS and XOLARIS studies. Part 1 of the XIRIUS study was a phase 1, dose-escalation study of 18 male participants 18 years or older enrolled between March 8, 2017, and October 16, 2018, with genetically confirmed RPGR-variant XLRP with active disease and best-corrected visual acuity better than or equal to light perception (cohort 1), 34 to 73 letters (20/40 to 20/200 Snellen equivalent; cohorts 2-3), or greater than or equal to 34 letters (better than or equal to 20/200 Snellen equivalent; cohorts 4-6). Participants from the noninterventional, multicenter, global, prospective XOLARIS clinical study who met the inclusion and exclusion criteria of part 1 of XIRIUS were included as a comparator group (n = 103). Safety assessments included all XIRIUS participants; post hoc associations of retinal sensitivity assessments in XIRIUS only included the 12 participants receiving the 4 highest doses of cotoretigene toliparvovec. Data were analyzed on June 30, 2021.Main Outcomes and MeasuresIncidence of dose-limiting toxicities (DLTs), treatment-emergent adverse events, changes from baseline in retinal sensitivity (as assessed by macular integrity assessment microperimetry), retinal sensitivity response (achievement of ≥7-dB improvement from baseline at ≥5 of 16 central loci), and low-luminance visual acuity were assessed over 24 months.ResultsA total of 18 participants (mean [SD] age, 31.9 [9.4] years; male, 100%) were enrolled and completed the XIRIUS study. A subgroup of 103 participants (mean [SD] age, 30.8 [11.4] years; male, 100%) from the XOLARIS study was included. Administration of the 4 highest doses of cotoretigene toliparvovec (n = 12) among the 18 XIRIUS participants was associated with early improvements in retinal sensitivity. One of 103 untreated participants (1%) in the XOLARIS subgroup achieved improved retinal sensitivity at month 12. No DLTs were noted at any dose, and serious adverse events of reduced visual acuity (n = 2) and noninfective retinitis (n = 1) occurred.Conclusions and RelevanceResults suggest that early and sustained improvements in retinal sensitivity and low-luminance visual acuity in some participants through 12 months support consideration of additional clinical trials.Trial RegistrationClinicalTrials.gov Identifier: XIRIUS: NCT03116113; XOLARIS: NCT04926129

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Changes in Retinal Sensitivity Associated With Cotoretigene Toliparvovec in X-Linked Retinitis Pigmentosa With RPGR Gene Variations

et al. 2023

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“…practice. Recent studies indicated that macular function, especially under mesopic or scotopic conditions, drew increasing attention in recent years (19,20).…”

Section: Discussionmentioning

confidence: 99%

“…It suggested that microperimetry is connected closely to ophthalmological clinical practice. Recent studies indicated that macular function, especially under mesopic or scotopic conditions, drew increasing attention in recent years ( 19 , 20 ).…”

Section: Discussionmentioning

confidence: 99%

Trends in research related to ophthalmic microperimetry from 1992 to 2022: A bibliometric analysis and knowledge graph study

Ming

Qin

2023

Front. Med.

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BackgroundMicroperimetry is a novel technology to assess macular function. The aim of the study was to explore the global research status and trends in microperimetry.MethodsDocuments related to microperimetry in ophthalmology from 1992 to 2022 were extracted from the Science Citation Index Expanded (SCI-E) database of the Web of Science Core Collection (WOSCC). Raw data were analyzed using the VOSviewer and CiteSpace software. Bibliometric parameters included annual publication quantity, countries, authors, journals, international cooperation, and keywords.ResultsA total of 1,217 peer-reviewed documents were retrieved. Annual research output has increased significantly since 2005, especially since 2013. Holz F, Rubin G, and Guymer R contributed most to the number of articles published about microperimetry. Rubin G, Fitzke F, and Holz F, respectively, received the most citations for their study. The countries publishing most were the USA, Italy, and the UK, while the USA, the UK, and Germany received the most citation frequency. Univ Bonn, UCL, and Moorfields Eye Hosp were the top three productive institutions for microperimetry research in the world. The top three journals that publish articles about microperimetry were Retina-The Journal of Retinal and Vitreous Diseases, Investigative Ophthalmology and Visual Science, and the American Journal of Ophthalmology. The top 10 common keywords included microperimetry, optical coherence tomography, eye, retinal sensitivity, macular degeneration, fundus autofluorescence, scanning laser ophthalmoscope, visual acuity, sensitivity, and degeneration. Keywords “optical coherence tomography angiography,” “retinitis pigmentosa,” and “internal limiting membrane” burst in the last 3 years.ConclusionThe bibliometric and knowledge graph analysis of research status and trends in microperimetry provided global researchers with valuable information to propose future cooperation and track cutting-edge progress.

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Additional measures of macular function beyond visual acuity

Ríos,

Lövestam-Adrian,

Plainis

et al. 2023

Graefes Arch Clin Exp Ophthalmol

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Purpose Visual function is a complex process in which external visual stimuli are interpreted. Patients with retinal diseases and prolonged follow-up times may experience changes in their visual function that are not detected by the standard visual acuity measure, as they are a result of other alterations in visual function. With the advancement of different methods to evaluate visual function, additional measurements have become available, and further standardization suggests that some methods may be promising for use in clinical trials or routine clinical practice. The objectives of this article are to review these additional measurements and to provide guidance on their application. Methods The Vision Academy’s membership of international retinal disease experts reviewed the literature and developed consensus recommendations for the application of additional measures of visual function in routine clinical practice or clinical trials. Results Measures such as low-luminance visual acuity, contrast sensitivity, retinal fixation and microperimetry, and reading performance are measures which can complement visual acuity measurements to provide an assessment of overall visual function, including impact on patients’ quality of life. Measures such as dark adaptation, color vision testing, binocular vision testing, visual recognition testing, and shape discrimination require further optimization and validation before they can be implemented in everyday clinical practice. Conclusion Additional measurements of visual function may help identify patients who could benefit from earlier diagnosis, detection of disease progression, and therapeutic intervention. New and additional functional clinical trial endpoints are required to fully understand the early stages of macular disease, its progression, and the response to treatment.

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Low Luminance Visual Acuity and Low Luminance Deficit in Choroideremia andRPGR-Associated Retinitis Pigmentosa

Cited by 11 publications

References 35 publications

Changes in Retinal Sensitivity Associated With Cotoretigene Toliparvovec in X-Linked Retinitis Pigmentosa With RPGR Gene Variations

Changes in Retinal Sensitivity Associated With Cotoretigene Toliparvovec in X-Linked Retinitis Pigmentosa With RPGR Gene Variations

Trends in research related to ophthalmic microperimetry from 1992 to 2022: A bibliometric analysis and knowledge graph study

Additional measures of macular function beyond visual acuity

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