ArticleDocumenta ophthalmologica. Advances in ophthalmology2015
Electroretinography and optical coherence tomography reveal abnormal post-photoreceptoral activity and altered retinal lamination in patients with enhanced S-cone syndrome.
Article in Documenta ophthalmologica. Advances in ophthalmology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed, 16 citations in OpenAlex.
- Application of Electrophysiology in Non-Macular Inherited Retinal Dystrophies.Journal of clinical medicine · 2023Review
- Suspected Enhanced S-Cone Syndrome: A Case Report.Cureus · 2023Article
- Enhanced S-cone Syndrome, a Mini-review.Advances in experimental medicine and biology · 2023Review
- The electroretinogram in the genomics era: outer retinal disorders.Eye (London, England) · 2021Review
- ISCEV extended protocol for the S-cone ERG.Documenta ophthalmologica. Advances in ophthalmology · 2020Article
- Novel clinical findings in autosomal recessive NR2E3-related retinal dystrophy.Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie · 2019Article
- ISCEV extended protocol for the photopic On-Off ERG.Documenta ophthalmologica. Advances in ophthalmology · 2018Article
- Two-color pupillometry in enhanced S-cone syndrome caused by NR2E3 mutations.Documenta ophthalmologica. Advances in ophthalmology · 2016Article
- A 5-Year-Old Case of Choroidal Neovascularization in Enhanced S-Cone Syndrome Treated with Ranibizumab.Case reports in ophthalmologyArticle
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6 authors at 1 institution in 1 country.
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Abstract
purposeTo compare functional abnormalities of enhanced S-cone syndrome (ESCS), as examined using standard and extended electroretinography (ERG), with structural findings and retinal architecture obtained by spectral domain optical coherence tomography (SD-OCT).
methodsFour patients with ESCS underwent standard full-field and multifocal ERGs, with extended S-cone and ON/OFF ERG protocols also performed. SD-OCT was also carried out, and longitudinal reflectivity profiles (LRPs) were calculated for the perifoveolar retina.
resultsAll four patients exhibited pathognomonic full-field ERG findings for ESCS, with delayed responses of similar waveforms to the same intensity flash under both scotopic and photopic conditions. The amplitudes of the full-field ERGs showed considerable variation between patients, which were not related to the extent of the visual field defects. Multifocal ERGs reflected preserved central function in eyes with good visual acuity (Snellen visual acuity >0.7). The ERGs to S-cone-specific stimulation confirmed the expected predominant activity of the S-cone system in all four patients. The ON/OFF ERG recordings revealed abnormal presence of both ON-response and OFF-response activities in three patients; the remaining patient showed only OFF-response activity. SD-OCT showed a significantly thickened outer nuclear layer in all four patients, as obtained by LRP analysis. Furthermore, in the patient with selective preservation of the OFF-response activity, LRP showed reduced numbers of hyper-reflectivity sub-peaks in the inner plexiform layer.
conclusionPatients with ESCS show characteristic full-field ERG waveform abnormality, predominance of S-cone ERG activity, and thickening of the outer nuclear layer on SD-OCT. Moreover, they can also show abnormal post-photoreceptor connectivity through S-cone-related OFF-bipolar cell activity.
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