{"title":"Intra- and inter-examiner reliability of ERGs recorded with two types of skin electrodes.","authors":"Ryunosuke Nagashima, Kumiko Kato, Keitaro Mizumoto, Sumine Mori, Daisuke Kurose, Iroha Fujimoto, Hisashi Matsubara, Hidetaka Kudo, Daphne L McCulloch, Mineo Kondo","doi":"10.1007/s10633-026-10107-9","DOIUrl":"https://doi.org/10.1007/s10633-026-10107-9","url":null,"abstract":"<p><strong>Purpose: </strong>To determine whether the intra-examiner and the inter-examiner reliabilities of the implicit times and amplitudes of the ERGs differed when recorded with two different types of skin electrodes.</p><p><strong>Subjects and methods: </strong>Eleven subjects (5 men and 6 women) were studied. The RETeval system was used to record ERGs, and the Sensor Strip of the RETeval system or the Red Dot electrocardiography electrodes (Red Dot) were used to record the ERGs. Flicker ERGs were recorded with a natural pupil. To test the intra-examiner reliability, one examiner positioned electrodes and recorded the ERGs three times on three different days from each subject. For the inter-examiner reliability, three different examiners recorded the ERGs from the 11 subjects. The implicit times and amplitudes of the flicker ERGs were analyzed using one-way intraclass correlation coefficients (ICC (1,1)) to assess intra-examiner variability and using a two-way intraclass correlation coefficient (ICC (2,1)) for inter-examiner variability.</p><p><strong>Results: </strong>For implicit times both the ICC (1,1) and ICC (2,1) were > 0.8 for both electrode types. For the amplitude, ICC (1,1) was > 0.8 for both electrodes, but the ICC (2,1) was lower at 0.474 for the Sensor Strip and 0.672 for the Red Dot electrodes. After Benjamini-Hochberg adjustments, the amplitudes did not differ between electrodes in either setting; implicit time was slightly shorter with the Red Dot than with Sensor Strip in the three-examiner setting (BH-adjusted P = 0.032; mean difference, 0.13 ms).</p><p><strong>Conclusion: </strong>The intra-examiner reliability was high for both types of electrodes, but the inter-examiner reliability indicated that the amplitudes of the ERGs tended to be less reliable for both types of electrodes. We conclude that standardizing the electrode placement and ensuring precise applications may help improve inter-examiner reproducibility, especially for the amplitude measurements.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147834999","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}
Anna Baldaquí-Baeza, Jesús Díaz-Cascajosa, Delia Yubero-Siles, Gema Escribano-Serrano, Diana Salinas-Chaparro, Cinthia Aguilera, Anna Esteve-Garcia, Itziar Alonso-Colmenero, Jaume Català-Mora
{"title":"New genetic feature associated with fundus albipunctatus: case series of two Spanish children with LRAT gene mutation.","authors":"Anna Baldaquí-Baeza, Jesús Díaz-Cascajosa, Delia Yubero-Siles, Gema Escribano-Serrano, Diana Salinas-Chaparro, Cinthia Aguilera, Anna Esteve-Garcia, Itziar Alonso-Colmenero, Jaume Català-Mora","doi":"10.1007/s10633-026-10106-w","DOIUrl":"https://doi.org/10.1007/s10633-026-10106-w","url":null,"abstract":"<p><strong>Objective: </strong>To describe the genetic mutation and its action mechanism in patients with compatible diagnosis of fundus albipunctatus (FAP).</p><p><strong>Subjects and methods: </strong>We describe the clinical evolution and genetic findings of two female Spanish children of 14 and 16 years old who have been followed at the children's hospital Sant Joan de Déu (Barcelona, Spain). Nyctalopia without detectable field constriction was the initial symptom. Retinal fundus was compatible with FAP or retinitis punctata albescens (RPA). We have registered ophthalmological examination from each visit, including visual acuity, visual field assessment, wide-field fundoscopy imaging, fundus autofluorescence and optical coherence tomography. Full-field electroretinogram (ERG) was also performed. DNA sampling was analysed by next-generation sequency (NGS).</p><p><strong>Results: </strong>Compound heterozygous pathogenic variants in the LRAT gene were identified in both children. Interestingly, these were the same two pathogenic variants, despite the families being unrelated. No additional genetic alterations were found that could explain the disease in our patients.</p><p><strong>Conclusions: </strong>To the best of our knowledge, up to the present time, only a single clinical case involving a genetic variant in the LRAT gene leading to an FAP phenotype has been reported in the literature. LRAT genetic variants, despite their low frequency in clinical practice, seem to lead to a FAP-like phenotype, in addition to their established association with Leber congenital amaurosis (LCA) and Early Onset Severe Retinal Dystrophy (EOSRD).</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147812057","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}
Youjin Elizabeth Youn, Allison L Dorfman, Anna Polosa, Patrick Hamel, Thuy Mai Luu, Anne Monique Nuyt, Sylvain Chemtob, Shigufa Kahn Ali, Cynthia X Qian
{"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":"https://doi.org/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":""},"PeriodicalIF":2.9,"publicationDate":"2026-04-29","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}
Tsai-Chu Yeh, Gabriel Velez, Soo Hyeon Lee, Gia-Han Ngo, Aarushi Kumar, Ahmad Al-Moujahed, Charles Yu, Loh Shan Leung, Prithvi Mruthyunjaya, Vinit B Mahajan
{"title":"A novel IDS variant associated with an isolated ocular phenotype in Hunter syndrome.","authors":"Tsai-Chu Yeh, Gabriel Velez, Soo Hyeon Lee, Gia-Han Ngo, Aarushi Kumar, Ahmad Al-Moujahed, Charles Yu, Loh Shan Leung, Prithvi Mruthyunjaya, Vinit B Mahajan","doi":"10.1007/s10633-026-10104-y","DOIUrl":"https://doi.org/10.1007/s10633-026-10104-y","url":null,"abstract":"<p><strong>Introduction: </strong>Hunter syndrome (mucopolysaccharidosis type II, MPS II) is an X-linked lysosomal storage disorder caused by iduronate-2-sulfatase (IDS) mutations and is classically associated with multiple-organ-systems involvement. Ocular findings are usually reported in conjunction with systemic manifestations, and isolated ocular presentations have not been well characterized. Here, we report a novel hemizygous IDS variant in a patient who initially presented with isolated corneal and retinal pathology and was subsequently diagnosed with attenuated Hunter syndrome.</p><p><strong>Methods: </strong>Comprehensive ophthalmic and retinal evaluation was performed, including multimodal retinal imaging and full-field electroretinography, alongside genetic testing.</p><p><strong>Results: </strong>A 44-year-old male with progressive nyctalopia demonstrated bilateral parafoveal and peripheral retinal depigmentation with central macular sparing on optical coherence tomography and a symmetric bull's-eye pattern on fundus autofluorescence. Visual field testing revealed bilateral ring scotomas, and full-field electroretinography showed subnormal rod-predominant responses. Pedigree analysis suggested X-linked inheritance. Genetic testing identified a novel hemizygous IDS variant (c.707A > G, p.Lys236Arg), and the diagnosis of Hunter syndrome was supported by markedly reduced IDS activity and elevated urinary glycosaminoglycans with increased heparan sulfate.</p><p><strong>Conclusion: </strong>A novel missense IDS mutation was identified in association with Hunter syndrome, highlighting the importance for ophthalmologists to consider MPS II in patients presenting with isolated retinopathy and the value of genetic diagnosis in revealing atypical systemic disease.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147765372","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":"The effect of physiological changes in estradiol hormone during the follicular phase of the menstrual cycle in healthy women on retinal ganglion cell function, retinal and optic nerve vascularization and structure.","authors":"Mualla Hamurcu, Burcu Polat Gültekin, Melisa Göçmen, Zarife Nurbanu Mendi, Zehra Kurdoğlu","doi":"10.1007/s10633-026-10105-x","DOIUrl":"https://doi.org/10.1007/s10633-026-10105-x","url":null,"abstract":"<p><strong>Purpose: </strong>This study aimed to investigate the effects of estradiol changes occurring during the early follicular phase of the menstrual cycle on retinal ganglion cell function, retinal and optic nerve vascularity and structure in healthy adults using pattern electroretinography (PERG), optical coherence tomography (OCT), and optical coherence tomography angiography (OCTA).</p><p><strong>Methods: </strong>The study included 26 healthy subjects with a visual acuity of 1.0. Serum hormone levels, intraocular pressure (IOP), cycloplegic spherical equivalent (SE), PERG, retinal nerve fiber layer (RNFL) thickness and OCTA measurements were evaluated. PERG tests were performed using the Metrovision Monpack system. Spectral-domain OCT (Topcon Corporation, Tokyo, Japan); OCTA (Optovue, Inc, Fremont, CA) devices were used. All tests were conducted on the 1st and 14th days of the menstrual cycle, and the results were compared.</p><p><strong>Results: </strong>The mean serum estradiol values on days 1 and 14 were 51 ± 19 ng/L and 304 ± 50 ng/L, respectively. PERG revealed no statistically significant differences in the amplitude or latency of the P50 and N95 waves between days 1 and 14. RNFL thickness did not differ significantly in the mean, superior, or inferior quadrants (p = 0.90, p = 0.85, and p = 0.39, respectively). OCTA analysis showed that peripapillary and macular vascular density values were similar between days 1 and 14 (p > 0.05). Correlation analysis revealed no significant relationship between electrophysiological parameters, RNFL thickness measurements, and retinal vascular densities (p > 0.05).</p><p><strong>Conclusion: </strong>In healthy women, no significant functional, vascular, or structural differences were observed in PERG, OCT, or OCTA parameters between low and high estradiol levels on days 1 and 14 of the menstrual cycle. Unlike the persistently low estradiol levels seen in menopause, physiological estradiol fluctuations during a healthy menstrual cycle do not cause functional, structural, or vascular changes in the optic nerve or retina.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147765355","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}
Paul A Constable, Lynne Loh, Maja Šuštar Habjan, Srikanta Kumar Padhy
{"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":"https://doi.org/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":""},"PeriodicalIF":2.9,"publicationDate":"2026-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jeremy J Chou, Rachael C Heath Jeffery, Jennifer A Thompson, Samuel McLenachan, Enid S Chelva, Tina M Lamey, Terri L McLaren, Fred K Chen
{"title":"Autosomal dominant Riggs-type congenital stationary night blindness with fundus sheen and retinal atrophy due to a novel GNAT1 p.Gln200Arg variant.","authors":"Jeremy J Chou, Rachael C Heath Jeffery, Jennifer A Thompson, Samuel McLenachan, Enid S Chelva, Tina M Lamey, Terri L McLaren, Fred K Chen","doi":"10.1007/s10633-026-10100-2","DOIUrl":"https://doi.org/10.1007/s10633-026-10100-2","url":null,"abstract":"<p><strong>Purpose: </strong>To report an Australian family with congenital stationary night blindness (CSNB, OMIM#139,330) harbouring a novel GNAT1 c.599A > G (p.Gln200Arg) variant. In contrast to previous case reports we observed a fundus sheen, outer retinal changes and electrophysiological features of cone dysfunction in addition to a Riggs-type CSNB.</p><p><strong>Methods: </strong>Ophthalmic history, clinical examination and multimodal imaging including fundus autofluorescence (FAF) and optical coherence tomography (OCT) were obtained. Full-field electroretinography (ERG) was conducted according to the International Society for Clinical Electrophysiology of Vision (ISCEV) standards. Genetic testing was performed using a targeted next-generation sequencing panel with familial segregation confirmed by Sanger sequencing. In silico prediction tools and protein structural modelling were used to evaluate the pathogenicity and functional impact of the GNAT1 p.Gln200Arg variant respectively.</p><p><strong>Results: </strong>Our proband, a 21-year-old transgender male, and his 62-year-old mother had a lifelong history of nyctalopia and visual acuity of 6/6 OU. The mother exhibited a golden sheen, sectoral chorioretinal atrophy and bone spicules. In the proband FAF imaging revealed hypoAF inferiorly with an arc of hyperAF whilst his mother had regions of hypoAF associated with outer retinal atrophy. Full-field electroretinography in the proband showed a cone-isolated retina with normal light-adapted responses. The mother had reduced and delayed light-adapted 30 Hz flicker. Both carried the GNAT1 variant NM_000172.4:c.599A > G (p.Gln200Arg). In silico analysis predicted impaired GTPase activity in Arg200 and constitutively active signally after photoactivation. The c.599A > G variant was classified as likely pathogenic.</p><p><strong>Conclusions: </strong>Our study suggests the GNAT1 p.Gln200Arg variant can manifest as both a Riggs-type CSNB and a rod-cone dystrophy within the same pedigree. This work expands the phenotypic spectrum of GNAT1-associated retinopathy and identifies GNAT1 as another potential cause of a fundus sheen.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147638302","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}
Oliver R Marmoy, Emma Hodson-Tole, Dorothy A Thompson
{"title":"The luminance-response function of the photopic negative response (PhNR): analysing different stimulation, recording and measurement approaches.","authors":"Oliver R Marmoy, Emma Hodson-Tole, Dorothy A Thompson","doi":"10.1007/s10633-026-10102-0","DOIUrl":"https://doi.org/10.1007/s10633-026-10102-0","url":null,"abstract":"<p><strong>Purpose: </strong>The photopic negative response (PhNR) is a measure of generalised retinal ganglion cell function. There has been heterogenous methodology to record this, with varied electrode type, stimulus luminance, temporal frequency and measurement approach. This study aimed to empirically explore these features to identify an optimal PhNR luminance-response protocol which produces the lowest variance and maximal efficiency to guide clinical protocols.</p><p><strong>Methods: </strong>Twelve healthy participants were recruited (age range 27-51y). Flash ERGs were simultaneously recorded from infraorbital skin and corneal fibre electrodes to a range of red flash stimuli (- 0.3-2.4 log cd.s/m<sup>2</sup>, incremented in 0.3 log units), whilst varying temporal frequency (1-5 Hz), background blue luminance (1, 1.5, 2 log cd/m<sup>2</sup>), and PhNR measurement approach (from baseline or b-wave, as an amplitude or ratio). The luminance-response series data were analysed for changes according to these variables, alongside a calculation of variability.</p><p><strong>Results: </strong>The PhNR luminance-response curves showed few significant differences with increasing temporal frequency, though inter-subject variability was highest for the slowest (1 Hz) and highest flash (5 Hz) stimulation rates. Background luminance reduced the relative sensitivity (K) but not maximal amplitude of the luminance-response curves (V<sub>max</sub>). With skin electrodes the b-PhNR amplitude and b-PhNR ratio showed the lowest levels of variability compared with other measurement approaches or electrodes.</p><p><strong>Conclusion: </strong>This study demonstrates that temporal frequency can be increased significantly, optimally at 4 Hz, without compromising the PhNR. PhNR variance is lower with skin electrode recordings and PhNR amplitude measurements from the b-wave compared to corneal fibre electrodes and baseline-PhNR amplitudes.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147626964","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":2.9,"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}