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200 papers
Brief report: harmonic analysis of the 30 Hz flicker ERG in early-stage diabetic retinopathy.
McAnany JJ, Park JC
Doc Ophthalmol · 2025 Dec · PMID 40448804 · Full text
PURPOSE: To determine if harmonic components of the 30 Hz flicker ERG are useful for detecting neural dysfunction in diabetics who have mild or no non-proliferative diabetic retinopathy (NPDR). METHODS: Previously report...
PURPOSE: To determine if harmonic components of the 30 Hz flicker ERG are useful for detecting neural dysfunction in diabetics who have mild or no non-proliferative diabetic retinopathy (NPDR). METHODS: Previously reported light-adapted flicker ERG data recorded from 20 diabetics who had no clinically-apparent retinopathy (NDR), 20 who had mild NPDR (MDR), and 20 non-diabetic controls were reanalyzed. From this dataset, the amplitude and phase of the 31.25 Hz flicker ERG fundamental and second harmonic were extracted. The 62.5 Hz flicker ERG fundamental was also extracted. Similar responses were also acquired prospectively from 10 controls, 5 NDR, and 5 MDR subjects, comprising a second dataset. RESULTS: Analysis of variance indicated that both diabetic groups had normal amplitudes elicited by the 31.25 Hz stimulus (fundamental and second harmonic), whereas the 62.5 Hz amplitude was reduced significantly in both diabetic groups. This pattern was found in both the retrospective and prospective analyses. CONCLUSIONS: The second harmonic of the 31.25 Hz flicker response (equivalent to 62.5 Hz) was normal in early-stage DR, whereas the response to 62.5 Hz flicker stimuli was abnormal. The second harmonic of the ISCEV standard 30 Hz flicker ERG does not appear to be a useful indicator of neural dysfunction in early DR.
Visual pathway function in adults born preterm with very low birth weight: a two-country birth cohort study.
Jørgensen APM, Kulmala M, Austeng D … +6 more , Sand T, Grøtting A, Evensen KAI, Kajantie E, Majander A, Morken TS
Doc Ophthalmol · 2025 Aug · PMID 40437207 · Full text
PURPOSE: To investigate if preterm birth with very low birth weight (VLBW; birth weight < 1500 g) affects macular and visual pathway function in an adult population and explore if best corrected visual acuity (BCVA) was...
PURPOSE: To investigate if preterm birth with very low birth weight (VLBW; birth weight < 1500 g) affects macular and visual pathway function in an adult population and explore if best corrected visual acuity (BCVA) was associated with any of the electrophysiologic responses. METHODS: Fifty participants born preterm with VLBW and 77 term-born controls years were recruited when aged 31-41 years from the Helsinki Study of Very Low Birth Weight Adults (Finland) and the NTNU Low Birth Weight Life study (Norway) studies. Pattern reversal electroretinogram (PR-ERG), visual evoked potential (PR-VEP) and BCVA were examined. PR-ERG components (P50, N95 peak time, amplitude and N95:P50 amplitude ratio) and PR-VEP components (N75, P100 and N145 peak time and amplitude) in the better-seeing eye were compared between the groups, and association with BCVA was examined. RESULTS: The VLBW group showed longer N145 peak time compared to the control group (mean difference 6.8 ms, CI 2.0 to 11.5, p = 0.006) and lower N95:P50 amplitude ratio (CI - 0.3 to - 0.1, p = 0.003). Otherwise, both groups showed similar electrophysiological waveforms. No relationship was found between electrophysiologic responses and BCVA. BCVA was normal in birth groups and showed no group difference. CONCLUSION: The responses in the primary visual cortex (N75 and P100) to visual stimuli presented to the better-seeing eye were similar in adults born preterm with VLBW and term-born controls. However, in the VLBW group, there was an indication that subtle electrophysiological deviation may exist at a higher cortical level (N145) and in the ganglion cell response in the macula. These significant differences were not related to reduced visual acuity.
Response: Aflibercept for the treatment of pigmentary retinopathy in Kearns-Sayre Syndrome?
Leung V, Wong J, Grigg JR
Doc Ophthalmol · 2025 Jun · PMID 40392487 · Publisher ↗
Abstract loading — click title to view on PubMed.
Aflibercept for the treatment of pigmentary retinopathy in Kearns-Sayre syndrome?
Finsterer J
Doc Ophthalmol · 2025 Jun · PMID 40389721 · Publisher ↗
Abstract loading — click title to view on PubMed.
Macular retinal function after autologous retinal transplantation in patients with refractory macular holes.
Arasaki R, Inoue T, Terasaki H … +7 more , Ueno S, Tsunoda K, Takeuchi J, Kitahata S, Yanagi Y, Maruyama-Inoue M, Kadonosono K
Doc Ophthalmol · 2025 Dec · PMID 40347223 · Publisher ↗
PURPOSE: To evaluate subjective and objective macular retinal function and morphology in eyes after autologous retinal transplantation (ART). METHODS: We conducted the study in three patients with large macular holes (MH...
PURPOSE: To evaluate subjective and objective macular retinal function and morphology in eyes after autologous retinal transplantation (ART). METHODS: We conducted the study in three patients with large macular holes (MHs) who underwent ART. The examination modalities included optical coherence tomography (OCT), microperimetry (MP-3), and focal macular electroretinography (FMERG) with 10-degree and 5-degree stimulus spots under infrared camera monitoring centered on the treated MHs after ART. RESULTS: All three patients showed improved visual acuity after the ART; MP-3 showed relatively good sensitivity around the fixation point with a dense scotoma at the center of the graft. All MHs were closed with autologous grafts and the size of MHs was decreased. OCT revealed clearly visible ellipsoid zones of the host retina around the grafted retina, however one transplanted eye showed disorganized outer layer of the host retina near the border of graft-host retina. FMERGs with the 10-degree stimulus were recorded successfully in all three treated eyes with more than half of a- and b-wave amplitudes of the fellow eyes. FMERGs with the 5-degree stimulus were recorded successfully in two of the treated and their fellow eyes. CONCLUSIONS: The FMERGs showed well-maintained macular retinal function after ART. The electrophysiologic and anatomic outcomes suggested that the host retina around the transplanted retina may play an important role in the postoperative macular retinal function with the mechanical support by the graft.
Evaluation of visual function and morphological changes in patients with adrenoleukodystrophy using SS-OCT and PVEP.
Yu Y, Zhang L, Wen S … +5 more , Li C, Tan L, Wu X, Zou Y, Liu T
Doc Ophthalmol · 2025 Aug · PMID 40347222 · Full text
PURPOSE: This study aims to evaluate visual function and morphological changes in patients with adrenoleukodystrophy (ALD) through sweep-source optical coherence tomography (SS-OCT) and patterned visual evoked potentials...
PURPOSE: This study aims to evaluate visual function and morphological changes in patients with adrenoleukodystrophy (ALD) through sweep-source optical coherence tomography (SS-OCT) and patterned visual evoked potentials (PVEP), with the goal of providing more accurate and comprehensive data for the diagnosis, monitoring, and assessment of treatment efficacy in patients with ALD. METHODS: This study included 46 ALD patients and 44 healthy controls. The thickness of the retinal nerve fiber layer (RNFL) around the optic disc, as well as the macular RNFL and ganglion cell-inner plexiform layer (GCIPL) thickness, was assessed using SS-OCT. The peak time and amplitude of the P100 wave were also measured using PVEP. Factors that were statistically significant in the univariate analysis were subjected to stepwise binary logistic regression analysis for further investigation. Predictive performance was evaluated by constructing receiver operating characteristic (ROC) curves and compared using DeLong's test. RESULTS: Compared with the control patients, ALD patients presented a significant increase in the peak time of the P100 wave (P < 0.05); however, no notable difference in amplitude was observed (P > 0.05). Additionally, substantial decreases in the RNFL and GCIPL thicknesses were observed within the parafoveal ring, especially in the superior and nasal quadrants (P < 0.05). The area under the curve for the binary logistic stepwise regression model was 0.883, with a sensitivity of 0.95, which surpassed the performance of the individual parameters. CONCLUSION: ALD patients present with abnormal retinal structures and a PVEP peak time delay. Combining these two parameters could increase the accuracy of an early ALD diagnosis.
The diagnostic value of multichannel VEPs for children without nystagmus.
Handley SE, Cowe J, Tucker L … +2 more , Marmoy OR, Thompson DA
Doc Ophthalmol · 2025 Aug · PMID 40244527 · Full text
PURPOSE: This study explored the clinical value of routine multichannel pattern reversal visual evoked potential (prVEP) recordings in children without nystagmus. METHODS: A single centre, retrospective case note review...
PURPOSE: This study explored the clinical value of routine multichannel pattern reversal visual evoked potential (prVEP) recordings in children without nystagmus. METHODS: A single centre, retrospective case note review was carried out of children without nystagmus who had multichannel prVEP recordings from midline, O1 and O2 electrodes referred to Fz to an ISCEV large check (50' check width), reversing 3/s in a full 30° field and right and left 0-15° half fields, during 2020. Full-field (FF) prVEPs were classified as abnormal if midline P100 amplitude and peak time fell outside reference limits. Trans-occipital distribution asymmetry was defined as differences ≥ 20% amplitude between FF-prVEP the O and O at the peak time of the midline P100. Half field (HF) prVEPs acted as the gold standard discriminator of abnormality. The trans-occipital distribution and amplitude of the HF-prVEP ipsilateral positive peak (iP100) were compared for each eye. RESULTS: FF-prVEP and HF-prVEP data from 63 children were classified. Group 1, 7/63 (11%), had abnormal midline FF-prVEP evidence of visual pathway dysfunction, whilst Group 2, 56/63 (89%), had normal midline FF-prVEPs. Group 2 was subdivided further according to the trans-occipital distribution of FF-prVEPs followed by HF-prVEPs. Group2A, 14/56 (25%), had symmetrical FF-prVEP distribution and normal HF-prVEPs. Group2B, 31/56 (55.4%), had asymmetrical FF-prVEP distribution, but lateralised HF-prVEPs that explained the FF-prVEP asymmetric distribution. Group2C, 11/56 (19.6%), had HF-prVEP evidence of pathway dysfunction with symmetric (n = 2) or asymmetric (n = 9) FF-prVEP distributions. Common referral reasons in all groups were reduced vision, glioma, craniopharyngioma, epilepsy presurgical evaluation, craniosynostosis, papilloedema/disc drusen, with various other specific conditions. CONCLUSIONS: Multichannel prVEPs add value to investigations of reduced or unexplained vision in children without nystagmus. Visual pathway abnormalities would not have been identified without a multichannel FF- or HF-prVEP in 11/56 (19.6%) of children in this study who had normal midline FF-prVEPs.
Synthetic electroretinogram signal generation using a conditional generative adversarial network.
Kulyabin M, Zhdanov A, Lee IO … +4 more , Skuse DH, Thompson DA, Maier A, Constable PA
Doc Ophthalmol · 2025 Oct · PMID 40240677 · Full text
PURPOSE: The electroretinogram (ERG) records the functional response of the retina. In some neurological conditions, the ERG waveform may be altered and could support biomarker discovery. In heterogeneous or rare populat...
PURPOSE: The electroretinogram (ERG) records the functional response of the retina. In some neurological conditions, the ERG waveform may be altered and could support biomarker discovery. In heterogeneous or rare populations, where either large data sets or the availability of data may be a challenge, synthetic signals with Artificial Intelligence (AI) may help to mitigate against these factors to support classification models. METHODS: This approach was tested using a publicly available dataset of real ERGs, n = 560 (ASD) and n = 498 (Control) recorded at 9 different flash strengths from n = 18 ASD (mean age 12.2 ± 2.7 years) and n = 31 Controls (mean age 11.8 ± 3.3 years) that were augmented with synthetic waveforms, generated through a Conditional Generative Adversarial Network. Two deep learning models were used to classify the groups using either the real only or combined real and synthetic ERGs. One was a Time Series Transformer (with waveforms in their original form) and the second was a Visual Transformer model utilizing images of the wavelets derived from a Continuous Wavelet Transform of the ERGs. Model performance at classifying the groups was evaluated with Balanced Accuracy (BA) as the main outcome measure. RESULTS: The BA improved from 0.756 to 0.879 when synthetic ERGs were included across all recordings for the training of the Time Series Transformer. This model also achieved the best performance with a BA of 0.89 using real and synthetic waveforms from a single flash strength of 0.95 log cd s m. CONCLUSIONS: The improved performance of the deep learning models with synthetic waveforms supports the application of AI to improve group classification with ERG recordings.
A novel mutation in CNNM4 is associated with a case of Jalili syndrome in Egypt.
Tawfik CA, Aly HS, Kabeel M … +2 more , Yousri I, Mohamed SA
Doc Ophthalmol · 2025 Jun · PMID 40232358 · Full text
PURPOSE: To report a novel homozygous mutation in CNNM4 gene associated with Jalili syndrome (JS) which is a rare, recessively inherited oculo-dental syndrome which encompasses cone-rod dystrophy (CORD) and amelogenesis...
PURPOSE: To report a novel homozygous mutation in CNNM4 gene associated with Jalili syndrome (JS) which is a rare, recessively inherited oculo-dental syndrome which encompasses cone-rod dystrophy (CORD) and amelogenesis imperfecta (AI). METHODS: A 4-year-old male patient of consanguineous Egyptian parents, who presented with progressive visual impairment and tooth decay underwent complete ophthalmological examination, dental, and systemic examination. Additionally, color fundus photography, fundus autofluorescence (FAF), spectral domain optical coherence tomography (SD-OCT) of the macula, full field electroretinogram (ffERG) were obtained. Orthopantomogram (OPG) were also obtained. NGS-based gene panel testing was done in a commercial laboratory from a peripheral blood sample. RESULTS: Fundus examination demonstrated typical features of CORD in the form of loss of foveal reflexes with macular retinal pigment epithelial mottling and atrophy reminiscent of bull's eye maculopathy. Dental assessment revealed evidence of AI. NGS-based gene panel identified a novel mutation in CNMM4 gene c.1423 G>A consistent with a diagnosis JS, thereby confirming the rare diagnosis. CONCLUSION: To the best of our knowledge, this is the first report of Jalili syndrome in Egypt. We are reporting a novel mutation in CNMM4 gene. We are also expanding the clinical spectrum of dental manifestation by reporting early eruption of the first permanent molars and suggesting that hyperopia could be a rather constant feature of JS. This case emphasizes the importance of comprehensive multidisciplinary assessment beyond visual complaints in IRD patients in order to reach an accurate diagnosis.
The ERGtools2 package: a toolset for processing and analysing visual electrophysiology data.
Lindner M
Doc Ophthalmol · 2025 Jun · PMID 40220212 · Full text
PURPOSE: To introduce ERGtools2, an open-source R package for processing, analysing and long-term storing visual electrophysiology data. METHODS: A dataset comprising Electroretinogram (ERG) recordings of C57Bl/6J mice,...
PURPOSE: To introduce ERGtools2, an open-source R package for processing, analysing and long-term storing visual electrophysiology data. METHODS: A dataset comprising Electroretinogram (ERG) recordings of C57Bl/6J mice, subjected to standard ISCEV stimuli, was used to present the functionality of ERGtools2. ERGtools2 stores and organizes all recordings, metadata, and measurement information from an individual examination in a single object, maintaining raw data throughout the analysis process. RESULTS: A standard workflow is presented exemplifying how ERGtools2 can be used to efficiently import, pre-process and analyse ERG data. Following this workflow, basic ERG measurements and visualisation of a single exam as well as group statistics are obtained. Moreover, special use cases are described, including for the handling of noisy data and the storage of data in the HDF5 format to ensure long-term preservation and accessibility. CONCLUSIONS: ERGtools2 provides a comprehensive, flexible, and device-independent solution for visual electrophysiology data analysis. Its emphasis on maintaining raw data integrity, combined with advanced processing and analysis capabilities, makes it a useful tool for preclinical and clinical research applications. The open-source nature and the use of open data formats promote reproducibility and data sharing in visual neurosciences.
DTL versus skin electrodes in recording of multifocal pattern electroretinogram and multifocal photopic negative response.
Nowitzki HM, Hoffmann MB, Al-Nosairy KO
Doc Ophthalmol · 2025 Jun · PMID 40205293 · Full text
OBJECTIVE: To compare the photopic negative response of the multifocal ERG (mfERG) and the multifocal pattern electroretinogram (mfPERG) using DTL electrode (E) vs skin electrode (E) in healthy young and old adults. METH...
OBJECTIVE: To compare the photopic negative response of the multifocal ERG (mfERG) and the multifocal pattern electroretinogram (mfPERG) using DTL electrode (E) vs skin electrode (E) in healthy young and old adults. METHODS: Ten "Young" [20-27 years] and eight "Old" [60-72 years] participants took part in this study. The electrophysiological responses were recorded binocularly using E and E. 5-way ANOVAs were applied to investigate the following factors on mfERG: i) ELECTRODE, ii) DILATATION, iii) AGE, iv) EYE, and v) ECCENTRICITY. For mfPERG, the same factors, except dilatation, were investigated applying 4-way ANOVAs. These were conducted for amplitude and peak time of different components as well as signal-to-noise-ratio (SNR). RESULTS: Amplitudes of mfERG [mfPERG]-based E recording were reduced to 32-38% [37-38%] compared to E, . This corresponded to SNR reduction to 80% [60%], . E based responses had shorter peak times, by 0.2-0.5 ms for N1 and P1, , [P1: 1.5 ms, ]. Both age groups had comparable amplitudes and SNRs, but Young had shorter peak times, by 1.5-2.2 ms for N1 and P1, [3.7-4.2 ms for N1, P1, N2, ]. Compared to dilated recordings, undilated mfERG amplitudes were reduced to 47-87%, , and peak times were delayed by 2.0-11.8 ms, . CONCLUSIONS: mfPERG & mfERG traces were similar for E and E. However, for skin electrodes, amplitudes and SNRs were lower and peak times shorter. E thus seem to be a viable alternative in patients in whom the use of corneal electrodes is precluded, e.g., children and disabled patients, but at the expense of SNR and with reference to E normative data.
Diagnostic accuracy of B scan ultrasound for posterior segment ocular disorders: a meta-analysis.
Wu F, Wang Q, Zheng T … +2 more , Wang X, Lin C
Doc Ophthalmol · 2025 Apr · PMID 40189719 · Publisher ↗
PURPOSE: B-scan ultrasound is widely utilized for diagnosing posterior segment ocular disorders due to its non-invasive nature and ability to provide real-time imaging. This meta-analysis evaluates the diagnostic accurac...
PURPOSE: B-scan ultrasound is widely utilized for diagnosing posterior segment ocular disorders due to its non-invasive nature and ability to provide real-time imaging. This meta-analysis evaluates the diagnostic accuracy of B-scan ultrasound in detecting various posterior segment ocular disorders. METHODS: A comprehensive search was conducted across multiple databases including Medline, EMBASE, Cochrane Library, and SCOPUS, from their inception until May 2024. The Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2) tool was employed to assess the risk of bias in the included studies. A bivariate meta-analysis was performed to calculate pooled estimates of sensitivity, specificity, and other diagnostic performance measures. Statistical analyses were conducted using STATA 14.2, which included generating summary receiver operating characteristic curves and diagnostic odds ratios. RESULTS: Ten studies met the inclusion criteria, encompassing a total of 1,617 reference-tested units. The pooled sensitivity and specificity of B-scan ultrasound for diagnosing posterior segment ocular disorders were remarkably high at 96% (95% CI 91-98%) and 94% (95% CI 87-98%), respectively. The diagnostic odds ratio was 363 (95% CI 94-1406), indicating substantial diagnostic accuracy. The area under the curve (AUC) was 0.99, confirming the excellent capability of B-scan ultrasound. Notable heterogeneity was observed (I = 86%), and no significant publication bias was detected. CONCLUSION: B-scan ultrasound demonstrates high sensitivity and specificity in diagnosing posterior segment ocular disorders, confirming its utility as a reliable diagnostic tool in clinical practice.
Phenotypic spectrum and theoretical prime editing analysis of WDR19-mediated retinal degeneration.
Pincay J, da Costa BL, Quinn PMJ … +5 more , Rodriguez M, Zhou A, Kong MD, Sparrow JR, Tsang SH
Doc Ophthalmol · 2025 Jun · PMID 40183892 · Full text
PURPOSE: The ciliopathies are a broad category of pleiotropic disease with numerous genes involved in pathogenesis. One of the genes implicated in the ciliopathies is WDR19, which can lead to several syndromic diseases t...
PURPOSE: The ciliopathies are a broad category of pleiotropic disease with numerous genes involved in pathogenesis. One of the genes implicated in the ciliopathies is WDR19, which can lead to several syndromic diseases that may manifest with a form of retinal degeneration. There is a lack of reporting on the WDR19-mediated retinal phenotype, and therefore warrants more clinical investigation. With retinal degeneration being the most prevalent symptom among the ciliopathies, phenotypic reporting is needed to enhance understanding of pathogenesis. METHODS: Clinical, imaging, and diagnostic records of patients with two variants in the WDR19 gene and a form of retinal degeneration were retrospectively reviewed. Two different individuals analyzed the variants in the studied patients using SnapGene (Version 4.3.11), employing both the canonical NGG PAM and the NGA PAM prime editors. RESULTS: Four patients from three families each carrying biallelic variants the WDR19 gene were reviewed. Two of the six unique variants identified among the patients were novel. Two identical twin patients presented with a recessive Stargardt (STGD)-like phenotype while the other two patients presented with a clinical picture more characteristic of retinitis pigmentosa (RP). Three of four patients had thickened external limiting membrane (ELM) on spectral-domain optical coherence tomography (SD-OCT). Full-field electroretinograms (ffERG) performed on two patients with the STGD-like phenotype showed a cone-rod pattern of degeneration. Quantitative short-wave fundus autofluorescence (qAF) performed on the two STGD-like patients was within the 95th percentile of normal eyes. CONCLUSIONS: WDR19-mediated retinal degeneration is heterogenous in presentation, and in some cases can phenocopy STGD. The foveal sparing phenotype was apparent in three of four patients with relatively preserved visual acuity, which may serve as a retinal prognostic factor in patients with pathogenic variants in WDR19. All six variants evaluated are correctable by prime editing, establishing a foundation for future research in therapeutic development.
ISCEV standard full-field ERG reference limits from 407 healthy subjects, derived from transference and validation of reference data between electrode types and centres.
Baker RA, Leo SM, Clowes WIN … +8 more , Chow I, Jiang X, Georgiou AL, Calcagni A, Hammond CJ, Neveu MM, Mahroo OA, Robson AG
Doc Ophthalmol · 2025 Apr · PMID 40164784 · Full text
PURPOSE: To establish age-adjusted reference intervals for the ISCEV standard full-field electroretinogram (ERG) recorded with silver thread electrodes in the lower fornix, based on a combined reference sample involving...
PURPOSE: To establish age-adjusted reference intervals for the ISCEV standard full-field electroretinogram (ERG) recorded with silver thread electrodes in the lower fornix, based on a combined reference sample involving recordings from reference subjects and transference of data between two centres and two types of electrode. METHODS: Silver thread lower fornix ERG reference data from two centres underwent verification for inclusion in the reference sample (n = 251). Comparison analysis was performed to determine whether gold foil reference data could be included in the silver thread reference range, directly or with adjustment. Reference subjects and patients underwent ERG testing with both silver thread and gold foil electrodes (n = 53) and skin electrodes (n = 41). A linear model, fitted to the electrode comparison data, was used to transform gold foil ERG reference data for inclusion in the reference sample (n = 156). The combined sample of 407 reference individuals was used to derive age-adjusted reference limits for the main DA 0.01, DA 3, DA 10, LA 30 Hz and LA 3 ERG components. RESULTS: Silver thread ERG reference data was sufficiently similar across two centres to justify combination into a single reference sample. Peak times for gold foil and silver thread ERGs were closely comparable (r 0.75-0.98, Bland-Altman bias ≤ 1.6 ms for all ERG components), with LA 30 Hz peak time showing the highest agreement (bias: - 0.2 ms, 95% limits of agreement (LOA): - 1.1 to 0.7 ms, 'silver thread-gold foil'). There was a clinically significant amplitude difference between electrode types: silver thread ERGs were 55-65% of the amplitude of gold foil ERGs (LOA ranged from 29 to 90%) and skin ERGs were 35-38% of the amplitude of silver thread ERGs (LOA ranged from 18 to 54%). Pooled reference data formed a sufficient sample covering 8 decades, from which age-adjusted parametric and nonparametric reference limits were calculated with reference to current guidelines. CONCLUSIONS: ISCEV standard silver thread ERG data were consistent across the two centres, allowing transference of reference data. Reference data recorded with gold foil electrodes can be transformed for inclusion in a silver thread ERG reference range. The study highlights methods of pooling multiple sources of reference data into a larger, more robust sample, pertinent to standardization, clinical management, and multi-centre studies. These reference data could be adopted by other centres or combined with other datasets, following suitable verification.
Multi-channel pattern VEPs with full and half field stimulation: methods of interpretation and diagnostic evaluation.
Thompson DA, Marmoy OR, Cowe J … +1 more , Handley SE
Doc Ophthalmol · 2025 Apr · PMID 40128423 · Full text
AIM: To describe methods of evaluating multichannel full and half field pattern VEPs using the ISCEV VEP Standard montage. METHODS: The dependence of full field and half field pattern VEPs on retinal areas and cortical g...
AIM: To describe methods of evaluating multichannel full and half field pattern VEPs using the ISCEV VEP Standard montage. METHODS: The dependence of full field and half field pattern VEPs on retinal areas and cortical generators is reviewed and applied to the interpretation and evaluation of multichannel half field pattern VEPs. RESULTS: There are predictable differences in the trans-occipital distributions of components of monocular full, and half field, pattern-reversal and full field, onset-offset VEPs. In combination, the differing distribution and dependence of these components on foveal and macular fields can help to identify and localise chiasmal and retro-chiasmal dysfunction and distinguish this from trans-occipital distribution due to individual variations of cortical architecture. A decision tree synthesising published evidence and current practice is suggested to guide interpretation of trans-occipital VEP distributions. CONCLUSION: The routine application of two additional lateral channels to acquire multichannel VEPs is quick, easy and adds clinical diagnostic value. The combination of full and half field pattern-reversal and fullfield, onset-offset VEPs can help evaluate chiasmal and retro-chiasmal visual pathway function, and minimise false positive interpretation of asymmetric VEP distributions, which may be due to cortical architecture or cranial anatomy alone.
Reference ranges of light-adapted full-field electroretinogram and associated factors in a large cohort of healthy school-aged children and adolescents.
Chan SS, Choi KY, Chan NY … +3 more , Lo VWY, Hung AH, Chan HH
Doc Ophthalmol · 2025 Aug · PMID 40121392 · Full text
PURPOSE: This study aimed to establish a reference data set of light-adapted full-field electroretinograms (ffERG) of healthy primary and secondary schoolchildren and investigate the relationship among refraction, ocular...
PURPOSE: This study aimed to establish a reference data set of light-adapted full-field electroretinograms (ffERG) of healthy primary and secondary schoolchildren and investigate the relationship among refraction, ocular biometry, and ffERG. METHODS: Healthy children aged between 6 and 17 years were recruited. Cycloplegic spherical equivalent refraction (SER), axial length (AL) and keratometry (K) measurements were performed. Standardized ffERGs, including light-adapted flash and 30-Hz flicker, were measured using a portable device with skin electrodes. The percentiles of peak time and amplitude of a- and b-waves and 30-Hz flicker of ffERG waveform were reported, and their relationships with age, SER, AL, K were investigated. RESULTS: Among the 445 participants (45.4% female), the SER (mean ± standard deviation) was - 0.72 ± 2.06 D and AL was 23.56 ± 1.15 mm. The 90% confidence interval (CI) of 5th reference limit of amplitudes of a- and b-wave were 5.0-5.8 µV and 15.0-17.6 µV, while 95th reference limit of peak times were 13.2-13.4 ms and 29.8-30.0 ms, respectively. The amplitude and peak time of the 30-Hz flicker waveform were 21.5-23.9 µV and 26.0-26.2 ms, respectively. In general, more myopic SER, and longer AL were associated with delayed and reduced ffERG waveforms. Older age and male sex were weakly correlated with ffERGs with minimal clinical significance. CONCLUSIONS: A reference data set of light-adapted ffERG in children and adolescents was established for clinical and research purposes.
Case of uveitis with increased electroretinographic amplitudes following Nivolumab and Ipilimumab administration for malignant melanoma.
Kato K, Nagashima R, Matsubara H … +4 more , Yonekawa Y, Kashima Y, Mizumoto K, Kondo M
Doc Ophthalmol · 2025 Jun · PMID 40072695 · Publisher ↗
PURPOSE: To report our flicker electroretinographic (ERG) findings in a patient who developed uveitis after treatment with immune checkpoint inhibitors (ICIs) for a metastatic malignant melanoma. METHODS: ERGs were used...
PURPOSE: To report our flicker electroretinographic (ERG) findings in a patient who developed uveitis after treatment with immune checkpoint inhibitors (ICIs) for a metastatic malignant melanoma. METHODS: ERGs were used to monitor retinal physiology in a patient with ocular complications following systemic ICI administration. Flicker ERGs were recorded using the RETeval system before and after the ICI treatments. RESULTS: A 45-year-old woman was referred to our ophthalmologic clinic for baseline evaluations prior to initiating nivolumab/ipilimumab therapy. The patient had no ocular or ERG abnormalities at the initial visit, but three weeks after starting nivolumab/ipilimumab, she developed conjunctival hyperemia and tearing. Slit-lamp examination showed anterior chamber inflammation, and the ERGs showed a 40% increase in the amplitude from the baseline. However, optical coherence tomography (OCT) did not show any abnormalities. The anterior segment inflammation and increased ERG amplitude resolved with topical betamethasone. The patient developed significant liver damage after the second administration of nivolumab/ipilimumab, and this therapy was discontinued. Two steroid pulse therapies were followed by tapered oral prednisolone. During the follow-up period, no significant abnormalities were observed in the visual acuity or OCT images, but the ERG amplitudes increased from the first to the eighth month after the liver damage was detected. Five years later, the ERGs and OCT findings were within the normal limits, but she had developed a sunset glow fundus in both eyes. CONCLUSION: ERGs may be a useful objective test for posterior inflammation induced by administration of ICIs that is not evident in OCT images.
SLC37A3-associated retinitis pigmentosa: a case report of clinical features and three-year follow up.
Kong MD, Bailey JA, Exinor A … +3 more , Piamjitchol C, Demirkol A, Tsang SH
Doc Ophthalmol · 2025 Jun · PMID 40069519 · Publisher ↗
PURPOSE: To describe a case of SLC37A3-associated retinitis pigmentosa (RP) and associated imaging and electroretinography findings. METHODS: The patient was evaluated at Columbia University Irving Medical Center using a...
PURPOSE: To describe a case of SLC37A3-associated retinitis pigmentosa (RP) and associated imaging and electroretinography findings. METHODS: The patient was evaluated at Columbia University Irving Medical Center using a comprehensive multimodal imaging protocol that included color fundus photography, fundus autofluorescence (FAF), and spectral-domain optical coherence tomography. Functional assessments were conducted using full-field electroretinography (ERG), following the ISCEV standard protocols to ensure consistent and reproducible measurements of photoreceptor activity. RESULTS: A 58-year-old male presented with night blindness beginning in early adulthood. Family history was positive for consanguinity. Dilated fundus exam revealed mild optic disc pallor, small perivenous hyperpigmentation greater nasally, and spared foveal islands in both eyes. FAF demonstrated a hyperautofluorescent ring around the macula in a bull's eye pattern and hypoautofluorescent lesions along the arcades and nasally. ERG results supported a diagnosis of rod-cone dystrophy. Initial genetic panel testing and whole exome sequencing were inconclusive, but whole genome sequencing identified homozygous mutations in the SLC37A3 gene. DISCUSSION: This report expands upon the sparse clinical data on SLC37A3-associated RP and supports the observation that a nasal predominance of retinal pigment may be a distinguishing clinical feature. Additionally, this case underscores the diagnostic value of whole-genome sequencing, especially when initial targeted genetic panels or whole-exome sequencing yield negative results. The metabolic role of SLC37A3, potentially linked to intracellular glucose transport, suggests a novel pathway in RP pathogenesis that warrants further study. Advances in gene-specific natural history characterization will be critical as gene-targeted therapies evolve, particularly for rare genetic forms of RP such as this case.
Evaluating the repeatability of the multifocal electroretinogram in the cone dominant 13-lined ground squirrel.
Manfredonia N, Follett HM, Summerfelt P … +3 more , Kuchenbecker JA, Carroll J, Yu CT
Doc Ophthalmol · 2025 Jun · PMID 40067596 · Full text
PURPOSE: To assess the repeatability of the multifocal electroretinogram (mfERG) in the 13-lined ground squirrel (13-LGS). METHODS: 13-LGS (Ictidomys tridecemlineatus, 2 M/4F, n = 12 eyes) were equally divided between 61...
PURPOSE: To assess the repeatability of the multifocal electroretinogram (mfERG) in the 13-lined ground squirrel (13-LGS). METHODS: 13-LGS (Ictidomys tridecemlineatus, 2 M/4F, n = 12 eyes) were equally divided between 61-segment or 103-segment protocols containing two consecutive mfERG scans per session, and two total sessions performed one week apart per eye. The trough-to-peak amplitudes were analyzed under three conditions: raw, normalized to the optic nerve head (ONH), and normalized to the visual streak (VS). For intrasession repeatability, the two consecutive scans within each session were analyzed. For intersession repeatability analyses, one scan was randomly chosen from each day. RESULTS: Intrasession repeatability of the mfERG trough-to-peak amplitude data averaged at 63% (Raw), 55% (ONH), and 50% (VS). There was no significant difference in repeatability between each day's intrasession repeatability values for all normalization conditions (Raw: Wilcoxon t-test, p = 0.2334; ONH: Paired t-test, p = 0.7803; VS: Wilcoxon t-test, p = 0.3804). Intersession percent repeatability of mfERG trough-to-peak amplitude data averaged at 72% (Raw), 61% (ONH), and 53% (VS). There was a statistically significant difference between the groups (Friedman test, p = 0.0038). This was evident in the Benjamini-Hochberg method of controlling the false discovery rate (FDR) where there was a significant difference comparing Raw versus VS (p = 0.0130) and ONH versus VS (p = 0.0011). There was no difference comparing Raw versus ONH (p = 0.1076). CONCLUSIONS: Overall intrasession and intersession repeatability of mfERG amplitude was relatively poor in our sample, though not markedly different than that reported in some other species and normalization methods did result in improved repeatability. As animal models are critical for vision research, these repeatability estimates will prove useful in interpreting future data collected following interventions or in longitudinal monitoring of disease models.
Unilateral best vitelliform macular dystrophy- a case series.
Ratra D, Karra A
Doc Ophthalmol · 2025 Apr · PMID 39992563 · Publisher ↗
PURPOSE: To report 2 cases with unilateral Best vitelliform macular dystrophy (VMD) and describe their multimodal investigations findings. METHODS: Two patients in their fifties who were misdiagnosed as central serous ch...
PURPOSE: To report 2 cases with unilateral Best vitelliform macular dystrophy (VMD) and describe their multimodal investigations findings. METHODS: Two patients in their fifties who were misdiagnosed as central serous chorioretinopathy were carefully evaluated using multimodal imaging and electrooculography (EOG) investigations. RESULTS: Both patients showed neurosensory elevation at the macula in one eye only leading to reduced vision. The optical coherence tomography showed subretinal hyperreflective material lining the undersurface of the elevated retina with thinning of the overlying photoreceptor layer. There was no choroidal thickening or pachy vessels. There was no leakage seen on fluorescein angiography nor any choroidal hyperpermeability on indocyanine green angiography. There was no choroidal neovascular membrane noted. The left eye was clinically unaffected for both. The EOG showed a reduced light peak to dark trough (LP:DT) ratio in both the eyes of these patients confirming the diagnosis of Best VMD. No change was seen in the eye condition over 2 years. CONCLUSIONS: Best VMD can present in unilateral fashion in rare instances. EOG can be confirmatory along with genetic testing. It can be misdiagnosed as CSCR where multimodal imaging and EOG can help differentiate it as Best VMD.
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