ArticleInvestigative ophthalmology & visual science2015
Full-Field Pupillary Light Responses, Luminance Thresholds, and Light Discomfort Thresholds in CEP290 Leber Congenital Amaurosis Patients.
Article in Investigative ophthalmology & visual science, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers, 1 of them a synthesis that pooled it.
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Who cites it
21 citing papers in PubMed, 1 synthesis or guideline pooled it, 32 citations in OpenAlex.
- ISCEV and IPS guideline for the full-field stimulus test (FST).Documenta ophthalmologica. Advances in ophthalmology · 2024Guideline
- Gene Editing forThe New England journal of medicine · 2024Trial
- A rapid pupillometry protocol for clinical use: effect of age and test-retest repeatability.Documenta ophthalmologica. Advances in ophthalmology · 2025Article
- A rapid pupillometry protocol for clinical use: Effect of age and test-retest repeatability.Research square · 2025Article
- Full-field stimulus threshold testing: a scoping review of current practice.Eye (London, England) · 2024Article
- Evaluation of the Glaucomatous Macular Damage by Chromatic Pupillometry.Ophthalmology and therapy · 2023Article
- Chromatic Pupillometry as a Putative Screening Tool for Heritable Retinal Disease in Rhesus Macaques.Translational vision science & technology · 2023Article
- Cone pathway dysfunction in Jalili syndrome due to a novel familial variant ofOphthalmic genetics · 2022Article
- Durable vision improvement after a single treatment with antisense oligonucleotide sepofarsen: a case report.Nature medicine · 2021Article
- Raloxifene Modulates Microglia and Rescues Visual Deficits and Pathology After Impact Traumatic Brain Injury.Frontiers in neuroscience · 2021Article
- Electrophysiological and Pupillometric Abnormalities in PROM1 Cone-Rod Dystrophy.Translational vision science & technology · 2020Article
- Full-Field Electroretinography, Pupillometry, and Luminance Thresholds in X-Linked Retinoschisis.Investigative ophthalmology & visual science · 2020Article
- Transient pupillary light reflex in CEP290- or NPHP5-associated Leber congenital amaurosis: Latency as a potential outcome measure of cone function.Vision research · 2020Article
- Two-color pupillometry in KCNV2 retinopathy.Documenta ophthalmologica. Advances in ophthalmology · 2019Article
- Leber Congenital Amaurosis (LCA): Potential for Improvement of Vision.Investigative ophthalmology & visual science · 2019Article
- Chromatic Pupillometry Methods for Assessing Photoreceptor Health in Retinal and Optic Nerve Diseases.Frontiers in neurology · 2019Review
- iPhone-based Pupillometry: A Novel Approach for Assessing the Pupillary Light Reflex.Optometry and vision science : official publication of the American Academy of Optometry · 2018Article
- The ipRGC-driven pupil response with light exposure and refractive error in children.Ophthalmic & physiological optics : the journal of the British College of Ophthalmic Opticians (Optometrists) · 2018Article
- Pupillary Light Reflexes in Severe Photoreceptor Blindness Isolate the Melanopic Component of Intrinsically Photosensitive Retinal Ganglion Cells.Investigative ophthalmology & visual science · 2017Article
- Pupillary responses in non-proliferative diabetic retinopathy.Scientific reports · 2017Article
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
Abstract
purposeTo investigate visual function in patients with CEP290 Leber congenital amaurosis (LCA-CEP290), using three full-field tests that can be performed by patients with poor fixation.
methodsSix patients (age range, 9-39 years) with LCA-CEP290 participated in the study. Stimuli for all three tests (full-field stimulus test [FST], pupillometry, and light discomfort threshold [LDT] testing) were generated by the Diagnosys ColorDome ganzfeld, by using achromatic stimuli as well as long- and short-wavelength stimuli to target rod and cone photoreceptors with all three tests and, in the latter two tests, melanopsin photoreceptors.
resultsDark-adapted FST thresholds in LCA-CEP290 patients were cone mediated and elevated between 4.8 and 6.2 log units above the normal achromatic threshold. The FST threshold was not measurable in one patient. The rod-mediated transient pupillary light reflex (PLR) was absent in all but the youngest patient, where unreliable responses precluded PLR quantification. Cone-mediated transient PLRs were subnormal in five patients, and absent in another. Sustained melanopsin-mediated PLRs were measurable in all patients. Full-field LDT thresholds were elevated compared to normal controls, and were lower for short-wavelengh than for long-wavelength stimuli.
conclusionsThe FST thresholds and transient PLRs were cone mediated in our cohort LCA-CEP290 patients. Rod-mediated PLRs were undetectable, whereas melanopsin-mediated sustained responses were detected in all patients, suggesting a relative preservation of inner-retina function. The LDT elevations for the patients are somewhat paradoxical, given their subjective perception of photoaversion. Relative aversion to short-wavelength light suggests influence from melanopsin on LDTs in these patients.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.