Documenta Ophthalmologica最新文献

{"title":"The long-term impact of retinopathy of prematurity on retinal function assessed with new patterns of mfERG analysis.","authors":"Youjin Elizabeth Youn, Allison L Dorfman, Anna Polosa, Patrick Hamel, Thuy Mai Luu, Anne Monique Nuyt, Sylvain Chemtob, Shigufa Kahn Ali, Cynthia X Qian","doi":"10.1007/s10633-026-10096-9","DOIUrl":"10.1007/s10633-026-10096-9","url":null,"abstract":"<p><strong>Purpose: </strong>To assess the long-term impact of retinopathy of prematurity (ROP) on macular function in pediatric and adult patient cohorts through the analysis of multifocal ERG (mfERG) results. Sectoral analyses were employed to uncover subtle localized findings that ring assessments could mask.</p><p><strong>Methods: </strong>25 children and 28 adults were recruited into three groups: \"ex-ROP\" (individuals with a history of ROP), \"preterm\" (preterm-born without ROP), and \"term\" (term-born controls). mfERG P1 amplitudes and latencies were evaluated in five concentric rings centered on the fovea, as well as superior, inferior, nasal and temporal quadrants following Patterns 1, 2 and 3 for sectoral analysis. Macular optical coherence tomography (OCT) was performed to measure central retinal thickness and evaluate correlation with mfERG results.</p><p><strong>Results: </strong>Using a sectoral mfERG analysis approach, applied for the first time in this population, we identified functional alterations that were not apparent with traditional global ring averages. In the pediatric cohort, sectoral evaluation revealed a significant reduction in temporal inner retinal responses in both ex-ROP and preterm children, changes that were obscured when relying solely on traditional mfERG ring analysis. Within the ex-ROP pediatric group, laser-treated eyes showed more pronounced dysfunction, with significantly prolonged P1 latencies and increased central retinal thickness compared to untreated eyes. In adults, sectoral analysis again highlighted functional abnormalities: ex-ROP and preterm participants demonstrated significantly decreased P1 amplitudes in multiple localized regions, including Rings 2 and 3. Finally, both pediatric and adult ex-ROP cohorts exhibited greater central retinal thickness than term-born controls, reinforcing the structural-functional relationship captured more sensitively through this refined sectoral method.</p><p><strong>Conclusion: </strong>Our study demonstrated that a history of ROP is associated with diminished macular function, as evidenced by mfERG assessments, a finding more pronounced in adults compared to the younger pediatric cohort, possibly suggestive of an age-related degeneration in retinal function.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":"327-339"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147765394","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"L- and M-cone-directed Global flash multifocal electroretinogram: conceptualization and development.","authors":"Nandini Ravi, Henry H L Chan","doi":"10.1007/s10633-026-10092-z","DOIUrl":"10.1007/s10633-026-10092-z","url":null,"abstract":"<p><strong>Background and purpose: </strong>Multifocal Electroretinogram (mfERG) applies a fast flicker-based stimulation on the human retina and is considered a valuable tool in studying the cone photoreceptor functional pathways. At the same time, the global flash paradigm of the mfERG (MOFO mfERG) is found to be advantageous in the simultaneous evaluation of retinal responses arising from outer (direct component, DC) and inner (induced component, IC) retinal levels. Incorporating a silent substitution stimulus to the MOFO mfERG remains unexplored yet has broad potential utility. The present study aimed to develop an L- and M-cone directed global flash mfERG.</p><p><strong>Methods: </strong>A silent substitution stimulus was created appropriate for the commercially available LED monitor. Using the conventional 96% contrast MOFO mfERG as a reference, L- and M-cone directed MOFO mfERG with 19-hexagon stimulation was created and tested in 36 Chinese adults with normal colour vision. Experimental validation was conducted using high-intensity red and green light adaptation to simulate colour vision deficiency.</p><p><strong>Results: </strong>Mathematical validation was performed by calculating and ensuring that proper cone quantal catches are achieved at both targeted and non-targeted cone photoreceptors. The cone response amplitude from participants with simulated protanopia and deuteranopia showed a reduction of up to 50% (p < 0.05) following pigment bleach due to light adaptation. The mean L/M cone amplitude ratio for the Chinese adults concerning the DC and IC was 0.84 ± 0.29 and 0.77 ± 0.32, respectively, for all rings combined. The M-cone amplitudes were higher than that of the L-cone. The M-cone responses were phase-advanced or faster compared to the L-cone responses (p < 0.001).</p><p><strong>Conclusion: </strong>The L- and M-cone-directed global flash mfERG protocols may provide valuable details on the specific cone-related outer and inner retinal responses and hold extensive utility in cone-related diseases and the evaluation of colour vision.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":"309-326"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13194218/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147456278","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Incomplete congenital stationary night blindness associated with a novel variant in the CACNA1F gene.","authors":"Shi Pei Loo, Chloe Shipton, Mark Hamilton, Andrew Brown, Eoghan Millar, Iqbal Malik, Madeleine Craig, Ruth Hamilton","doi":"10.1007/s10633-025-10062-x","DOIUrl":"10.1007/s10633-025-10062-x","url":null,"abstract":"<p><strong>Purpose: </strong>Incomplete congenital stationary night blindness (icCSNB) is a subtype of inherited, non-progressive retinal diseases. Most cases of icCSNB result from mutations in the X-linked gene CACNA1F. We describe the clinical findings of two male siblings diagnosed with icCSNB, both carrying a novel variant c.4008 + 5G > T in CACNA1F inherited from their mother.</p><p><strong>Methods: </strong>We carried out a comprehensive ophthalmic assessment, including fundus imaging, optical coherence tomography (OCT) scanning and electroretinography. We performed genetic testing with next generation sequencing, in-silico and functional analyses to further characterise the novel variant.</p><p><strong>Results: </strong>Two male siblings presented with high myopia and reduced visual acuities  at age three. Examination and OCT demonstrated no significant abnormalities in both siblings. Full-field electroretinogram (ffERG) testing demonstrated markedly reduced amplitude to weak flashes and an electronegative waveform to strong flashes in dark-adapted ERGs, resembling that of icCSNB, leading to its diagnosis in both children. Next generation sequencing in the older sibling identified a novel hemizygous c.4008 + 5G > T variant in CACNA1F. In-silico analysis of this variant predicted that it would disrupt normal splicing of CACNA1F, though it was not possible to confirm this by RNA sequencing. This same variant was found in the younger sibling, as well as in their mother who had normal examination and ffERG findings.</p><p><strong>Conclusions: </strong>We report a novel CACNA1F variant not previously identified in the literature in three patients. Although functional analyses were unable to confirm pathogenicity of this variant, in-silico tools predicted that its effect is consistent with the pathogenesis of icCSNB. Reporting of this family further widens the genotypic spectrum of icCSNB.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":"401-407"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145457763","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Functional and structural retinal alterations in Alzheimer's disease: insights from photopic negative response (PhNR) and OCT-based analysis.","authors":"Hidayet Sener, Furkan Ozer, Osman Ahmet Polat, Beyza Ogurlu, Hatice Kubra Sonmez, Murat Gultekin, Fatih Horozoglu","doi":"10.1007/s10633-026-10089-8","DOIUrl":"10.1007/s10633-026-10089-8","url":null,"abstract":"<p><strong>Background: </strong>We evaluated retinal function and structure in Alzheimer's disease (AD) using photopic negative response (PhNR) from full-field electroretinography (ERG) and using optical coherence tomography (OCT)-based retinal layer measurements.</p><p><strong>Methods: </strong>Ninety eyes from 61 subjects (31 controls, 30 AD patients) were included. Full-field ERGs were recorded using chromatic red-on-blue stimulation (1.7 cd·s/m² flashes over an 8 cd/m² blue background) in accordance with the ISCEV extended PhNR protocol. The AD group included 16 females and 14 males with moderate cognitive impairment (mean MMSE 15.5 ± 3.9). ERG a-wave, b-wave, and PhNR amplitudes were recorded. Macular and peripapillary retinal nerve fiber layer (RNFL) thicknesses were measured by OCT.</p><p><strong>Results: </strong>A-wave (AD: - 13.4 ± 11.9 µV, control: - 32.5 ± 13.7 µV, p < 0.001), b-wave (AD: 59.1 ± 28 µV, control: 156 ± 48.8 µV, p < 0.001), PhNR (AD: - 21.4 ± 14.9 µV, control: - 51 ± 15.4 µV, p < 0.001) amplitudes were lower in AD. Thicknesses of the central INL (AD: 25 ± 6.6 µm, control: 25.2 ± 8.2 µm, p = 0.04), inferior OPL (AD: 32.7 ± 6.2 µm, control: 36.9 ± 8.9 µm, p = 0.01), and temporal OPL (AD: 29.3 ± 5.2 µm, control: 34.8 ± 7.0 µm, p = 0.001) were reduced in AD, while other retinal layers showed no significant differences between groups (p > 0.05). The b-wave amplitude showed the highest discriminative power with an AUC of 0.956, followed by the PhNR amplitude (AUC = 0.930) and the a-wave amplitude (AUC = 0.904).</p><p><strong>Conclusion: </strong>AD was associated with a generalized reduction in full-field ERG amplitudes, accompanied by selective retinal layer changes on OCT. Among electrophysiological measures, b-wave amplitude demonstrated the highest ability to distinguish AD patients from controls.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":"369-380"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13194298/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147354228","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Retained cone-responses in homozygous start codon variant in KIZ-associated retinitis pigmentosa.","authors":"Maximilian D Kong, Mia O'Connell, Abdhel Exinor, Megan Soucy, Scott E Brodie, Stephen H Tsang","doi":"10.1007/s10633-025-10066-7","DOIUrl":"10.1007/s10633-025-10066-7","url":null,"abstract":"<p><strong>Purpose: </strong>To report the clinical phenotype, imaging characteristics, and electrophysiologic findings of a 62-year-old patient with retinitis pigmentosa (RP) harboring a likely pathogenic homozygous KIZ (NM_018474.6) start codon variant (c.3G > A, p.Met1?), only previously reported in the compound heterozygous state.</p><p><strong>Methods: </strong>The patient underwent clinical evaluation including full medical history, best-corrected visual acuity (BCVA), slit lamp exam, and dilated fundus examination (DFE), spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), and full-field electroretinography (ffERG), following the ISCEV standard protocols. Genetic testing was performed using the Invitae inherited retinal disorders panel of 330 genes.</p><p><strong>Results: </strong>BCVA was 20/40 in both eyes. Fundus examination revealed mild optic disc pallor, arteriolar attenuation, peripheral pigment migration, and macular hyper-autofluorescence along the arcades. with macular sparing. Outside the arcades, there are hypo-autofluorescence spots corresponding to retinal pigement epithelium atrophy. There is marked peri-papillary atrophy. SD-OCT showed diffuse outer retinal thinning, ellipsoid zone constriction, mild cystoid macular edema, and epiretinal membrane. ffERG was consistent with a rod-cone dystrophy, with extinguished dark-adapted responses and severely attenuated 30 Hz flicker amplitudes. Genetic testing identified a heterozygous variant of uncertain significance in CTNNA1 (c.1486C > T, p.Arg496Cys) and a homozygous KIZ variant (c.3G > A, p.Met1?), previously observed only in compound heterozygosity. The patient was diagnosed with KIZ-associated RP and initiated on topical dorzolamide.</p><p><strong>Conclusions: </strong>This case expands the clinical spectrum of KIZ-associated RP by describing the phenotype associated with a homozygous start codon variant. Despite the disruptive nature of the mutation, the patient exhibited a relatively mild rod-cone dystrophy with retained cone responses into the seventh decade. These findings support the inclusion of KIZ in diagnostic panels for autosomal recessive RP and contribute valuable genotype-phenotype correlation data for this rare ciliopathy.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":"409-414"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146009074","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Review of the clinical electrooculogram - Part 1: Mechanism of the Light-Rise.","authors":"Paul A Constable, Lynne Loh, Maja Šuštar Habjan, Srikanta Kumar Padhy","doi":"10.1007/s10633-026-10103-z","DOIUrl":"10.1007/s10633-026-10103-z","url":null,"abstract":"<p><p>The clinical electrooculogram (EOG) is the sole clinical electrophysiological test for assessing the function of the retinal pigment epithelium (RPE). However, despite several lines of investigation, the complete mechanism of the response has evaded a comprehensive description. The standard model implicates the rod photoreceptors and a signaling molecule termed the 'light-rise substance' that binds to an apical membrane 'light-rise receptor' or is transported across the membrane to elevate intracellular calcium concentration. The identity of the calcium activated chloride channel in the basolateral membrane was thought to be bestrophin, given the association of mutations in the hbest1 gene with Best Vitelliform Macular Dystrophy. However, recent findings have implicated a member of the anoctamin family as the calcium activated chloride channel with bestrophin regulating intracellular calcium in conjunction with the L-type calcium channel. How the changes in intracellular calcium are manifested as well as how the interaction with light in the dark-adapted state gives rise to the slow-dark and -light damped oscillations are yet to be described fully. This review summarizes the cellular mechanisms of the RPE that have been implicated in the generation of the light-rise and describes the likely candidates for the light-rise substance. A companion paper provides a summary of the bestrophinopathies and possible clinical modifications to enhance the EOG's clinical utility.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":"267-285"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13194270/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147688424","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The area under the waveform as an alternative measure of the photopic negative response.","authors":"Radouil Tzekov, Jan Kremers, Sara Safari","doi":"10.1007/s10633-026-10088-9","DOIUrl":"10.1007/s10633-026-10088-9","url":null,"abstract":"<p><strong>Purpose: </strong>To explore how the photopic negative response (PhNR), measured as an area defined by the ERG waveform can be used as complementary or an alternative measure to the traditionally measured PhNR amplitude.</p><p><strong>Methods: </strong>A retrospective chart review and data analysis of light-adapted 3.0 ERG records in patients and normal subjects patients aged 18 and older undergoing routine ERG testing was conducted. A new measure was analyzed: area defined by the PhNR curve. It was obtained in two ways: as an area under the ERG waveform (AUW) and an area above the ERG waveform (AAW) with starting points defined by the b-wave peak and time windows ranging from 5 to 45 ms in duration. A linear regression was conducted between PhNR1 amplitude (trough before i-wave), PhNR2 amplitude (trough after i-wave), PhNR AUW, and PhNR AAW. Furthermore, a prediction analysis based on AUW/AAW was conducted where the strongest correlated measures were used to predict the PhNR2 amplitude.</p><p><strong>Results: </strong>The ERG recordings of 70 patients/135 eyes (52F/18 M, average age: 49.2 ± 15.3 years) and six healthy subjects (1F/5 M, age between 22 and 58) were included in this study. 26 patients had well-defined PhNR2 troughs and were used for prediction analysis. There was good correlation between PhNR1 and PhNR2 amplitudes (r² 0.9755-0.9759 when amplitude was measured from the b-wave peak and 0.7265-0.6413, when amplitude was measured from baseline). The best predictors for PhNR2 amplitude were areas of 35 ms duration: AAW starting 30 ms after b-wave peak and AUW starting at 35 ms after the peak, when amplitude was measured from b-wave peak or baseline, respectively. Similar results were obtained by conducting similar prediction analysis on a small number of healthy volunteers.</p><p><strong>Conclusions: </strong>Determining and using AAW/AUW for PhNR2 prediction could be a valuable method in cases where the PhNR2 peak is not well defined.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":"357-368"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147324845","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Does EyeKinetix pupillometry clarify RAPD detection?","authors":"Mark A Kahrhoff, Carl J Bassi","doi":"10.1007/s10633-026-10097-8","DOIUrl":"https://doi.org/10.1007/s10633-026-10097-8","url":null,"abstract":"<p><strong>Purpose: </strong>To evaluate variability observed in pupil light response with the EyeKinetix pupillometer in healthy non-dark-adapted individuals.</p><p><strong>Methods: </strong>We performed objective pupillometry in 440 non-dark-adapted patients with 20/20 corrected visual acuity, normal visual fields, and no ophthalmic disease who presented for routine exam. EyeKinetix was performed as part of the routine exam screening protocol. Metrics reviewed were RAPDx amplitude scores, latency scores, and quantitative metrics of pupil dynamics measured by the EyeKinetix. Sequential retesting was analyzed for repeatability. Statistical analyses included normality testing, confidence intervals, t-tests, and Intraclass Correlation Coefficient (ICC) calculations for repeated measures.</p><p><strong>Results: </strong>Amplitude and latency scores exhibited significant variability. The mean amplitude score was 0.0249 ± 0.247, with 5% of patients >  = 2 standard deviations [> 0.51 log unit (LU)]. Latency scores showed low reliability (ICC = 0.165), whereas amplitude, constriction velocity, and release velocity demonstrated moderate to high reliability (ICC = 0.472-0.966).</p><p><strong>Conclusion: </strong>Objective pupillometry without dark adaptation using the EyeKinetix device displays substantial variability and identifies a number of relative afferent pupil defects (RAPDs) in this cohort of healthy patients. These findings indicate that, in healthy populations, reliance on RAPDx scores alone could generate false positives. Establishing robust normative cutoffs and validating them in disease cohorts will be necessary before reliable use as a screener. Further optimization may be necessary for more clinical confidence in a primary care setting.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147590607","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Full-field electroretinogram (ffERG) over 48 months correlates with baseline retinal dysfunction in Vogt-Koyanagi-Harada disease: a longitudinal study.","authors":"Fernanda Maria Silveira Souto, Ruy Felippe Brito Gonçalves Missaka, Marcelo Mendes Lavezzo, Viviane Mayumi Sakata, Maria Kiyoko Oyamada, Joyce Hisae Yamamoto","doi":"10.1007/s10633-025-10080-9","DOIUrl":"10.1007/s10633-025-10080-9","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate full-field electroretinogram (ffERG) parameters and evaluate their clinical associations in patients with acute Vogt-Koyanagi-Harada (VKH) disease over a 4-year follow-up.</p><p><strong>Methods and analysis: </strong>This retrospective cohort study included 21 patients with acute VKH disease followed for 48 months after initiation of systemic corticosteroid therapy, with or without adjunctive immunosuppression. ffERG was performed at 1, 6, 12, and 48 months (M) post-treatment. At M48, eyes were classified into two groups: Group 1 (normal ffERG) and Group 2 (subnormal ffERG), based on whether any ffERG parameters fell below the 5th percentile of age-matched healthy controls. Main outcomes included longitudinal ffERG changes, clinical associations, and the 6-month recovery ratios relative to baseline (M1).</p><p><strong>Results: </strong>All ffERG parameters improved significantly from baseline to M6 (p < 0.001) and to M12 (p < 0.001) and stabilized thereafter. Group 2 exhibited consistently reduced ffERG amplitudes compared to Group 1 throughout follow-up (p < 0.01 to p < 0.001), despite similar recovery trends. Recovery ratios at M6 ranged from 27%-78% in Group 1 and 26%-175% in Group 2; however, Group 2 remained below normal levels at M48. Sunset glow fundus (SGF) at M12 was significantly more frequent in Group 2 (60.7%) than in Group 1 (21.4%, p = 0.025).</p><p><strong>Conclusions: </strong>Retinal function improved during the first year and stabilized thereafter, irrespective of treatment type. Persistent subnormal ffERG at 48 months reflected a poorer baseline function and was associated with the development of SGF.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":"185-196"},"PeriodicalIF":2.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13083520/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146084863","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The short wavelength electro-oculogram (SW-EOG) in best disease: preliminary results.","authors":"Srikanta Kumar Padhy, Paul A Constable","doi":"10.1007/s10633-026-10081-2","DOIUrl":"10.1007/s10633-026-10081-2","url":null,"abstract":"<p><strong>Purpose: </strong>The clinical electrooculogram (EOG) is used for the diagnosis of bestrophinopathies to evaluate the function of the retinal pigment epithelium. The current ISCEV test protocol uses a broad band white light with luminance of 100 cd/m². An alternative monochromatic 448 nm short wavelength (SW-EOG) has been proposed but to date has not been evaluated in clinical cases where the standard EOG is abnormal.</p><p><strong>Methods: </strong>To evaluate the clinical potential of the SW-EOG four genetically confirmed cases of Best Vitelliform Macular Dystrophy were tested using the standard white (100 cd/m²) and SW-EOG (448 nm) at 30 cd/m² to ascertain if the SW-EOG was also affected. In addition, a qualitative 5-point Likert survey was conducted to gauge overall patient comfort.</p><p><strong>Results: </strong>In all four cases the SW-EOG was reduced and provided an equivalent clinical measure for RPE dysfunction. All four participants rated the SW-EOG as being more comfortable than the white standard EOG test.</p><p><strong>Conclusions: </strong>This is the first demonstration of an alternative stimulus for the EOG that provided clinically valid results with greater comfort than the current ISCEV protocol. Further studies are required to validate the SW-EOG as an alternative to the white broad band stimulus.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":"117-127"},"PeriodicalIF":2.0,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13083315/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146104280","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}