Giulia Steuernagel Del Valle, Haipha Ali, John R Grigg, Ruth Hamilton, Michael B Hoffmann, Bruce Hudson, Wanda L Pfeifer, Dorothy A Thompson, Anupreet Tumber, Ajoy Vincent, Gerard C de Wit
{"title":"Detecting congenital chiasmal misrouting using multichannel VEPs: protocol for a scoping review.","authors":"Giulia Steuernagel Del Valle, Haipha Ali, John R Grigg, Ruth Hamilton, Michael B Hoffmann, Bruce Hudson, Wanda L Pfeifer, Dorothy A Thompson, Anupreet Tumber, Ajoy Vincent, Gerard C de Wit","doi":"10.1007/s10633-025-10036-z","DOIUrl":"https://doi.org/10.1007/s10633-025-10036-z","url":null,"abstract":"<p><strong>Objective: </strong>We present the protocol of a prospective scoping review which aims to understand how multi-channel visual evoked potentials (mcVEPs) are used to investigate congenital chiasmal misrouting and what outcomes are measured, interpreted and reported.</p><p><strong>Introduction: </strong>mcVEPs are used for the objective evaluation of chiasmal misrouting, which is characterized by a crossed asymmetry in the distribution of cortical responses over each hemisphere during monocular stimulation, and is often observed in patients with albinism. The application and analysis of mcVEPs varies across centers, creating a need to explore the range of practice regarding their conduct and reporting and to identify potential areas for adaptation or optimization or guidelines for specific populations.</p><p><strong>Inclusion criteria: </strong>Peer reviewed and grey literature on the use of mcVEP to detect chiasmal misrouting in humans with non-acquired pathologies will be considered. All literature providing details of mcVEP methodology for replication and specification of chiasmal misrouting will be included for review.</p><p><strong>Methods: </strong>Searches will be conducted using MEDLINE, Embase, Cochrane and Web of Science with the expertise of a librarian. The search will be conducted with no limitation on time period, but will be restricted to the Latin alphabet. Titles and abstracts will be screened by two investigators with conflicts resolved by a third investigator. Included articles will proceed with data extraction on study details including methodology, design, and outcomes. The results will be synthesized and mapped for logical understanding.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-06-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144526809","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}
Yue Liu, Hui Li, Xing Wei, Yamei Li, Yunyu Zhou, Xuan Zou, Ruifang Sui
{"title":"Seventeen-year follow-up of mitochondrial myopathy and ataxia in a Chinese family: case reports and literature review.","authors":"Yue Liu, Hui Li, Xing Wei, Yamei Li, Yunyu Zhou, Xuan Zou, Ruifang Sui","doi":"10.1007/s10633-025-10037-y","DOIUrl":"10.1007/s10633-025-10037-y","url":null,"abstract":"<p><strong>Purpose: </strong>To investigate the retinal phenotype and genetic features of a Chinese family with a diagnosis of mitochondrial myopathy and ataxia (MMYAT).</p><p><strong>Methods: </strong>We conducted a 17-year follow-up of two sisters from a Chinese family and reviewed their medical and family histories. The retinal phenotype was assessed using a multi-modal imaging technique, which includes ultra-widefield (UWF) scanning laser ophthalmoscope (SLO), UWF fundus autofluorescence (FAF), and optical coherence tomography (OCT). Whole exome sequencing (WES) was performed to detect pathogenic variants. Sanger sequencing validation and segregation analysis were further performed for the confirmation of genetic results. A literature review was conducted, analysing the data from 11 published articles encompassing 33 confirmed cases of MMYAT up to 2025.</p><p><strong>Results: </strong>Ophthalmic multimodal imaging examination revealed typical characteristics of retinal dystrophy in both patients, including binocular widespread salt-and-pepper pigmentation, macular atrophy, a mottled pattern of hypoautofluorescence, and degeneration of the ellipsoid zone. A comprehensive review of the patients' histories identified muscle weakness, ataxia, cerebellar atrophy, mild cognitive impairment, and developmental delay. Two compound heterozygous variants of the mitochondrial distribution and morphology regulator 1 (MSTO1) gene (NM_018116.3), c.971C > T (p.T324I) and c.1108G > A (p.A370P), were detected using WES. A comprehensive literature review was also conducted to gain an overview of the various symptoms associated with MMYAT.</p><p><strong>Conclusion: </strong>Our study provides a comprehensive ophthalmic characterization of MMYAT, indicating that retinal dystrophy is a key characteristic of this disease. Multimodal imaging of the retina is beneficial for diagnosing MMYAT-associated retinal dystrophy. Increased awareness and comprehensive ophthalmic examination are crucial for obtaining an early and accurate diagnosis.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144339934","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}
Stephanie Quinn, Vasileios T Papastavrou, Clare Warriner, Jill M O'Brien, Michael E Grinton, Andrew C Browning
{"title":"Early retinal electrophysiology changes in quinine overdose.","authors":"Stephanie Quinn, Vasileios T Papastavrou, Clare Warriner, Jill M O'Brien, Michael E Grinton, Andrew C Browning","doi":"10.1007/s10633-025-10034-1","DOIUrl":"https://doi.org/10.1007/s10633-025-10034-1","url":null,"abstract":"<p><strong>Purpose: </strong>To report the early and subsequent electrophysiological findings of 2 patients following quinine overdose.</p><p><strong>Methods: </strong>Serial assessments including: Medical history, visual acuity (VA), fundus autofluorescence, spectral-domain macular optical coherence tomography (OCT) and full-field electroretinogram (ffERG) were performed on 2 patients, between 2 and 47 days after quinine overdose.</p><p><strong>Results: </strong>Both patients experienced a similar clinical course. After almost total vision loss within the first 24 h, VA dramatically improved by day 3. Early OCT changes demonstrated central macula hyperautofluorescence, which coincided with a hyperreflectivity of the macular inner retina on OCT. The initial ffERG findings demonstrated changes consistent with marked inner retinal dysfunction of the cone system, affecting both the cone ON- and OFF-bipolar cell pathways. In contrast, rod bipolar cell function was unaffected in the early phase of toxicity. Between days 10 and 17, the retinal arterioles showed narrowing which coincided with attenuation of ffERG parameters of rod system inner retinal function between days 10-40.</p><p><strong>Conclusions: </strong>These cases suggest the early stages of quinine toxicity affect function of the presynaptic cone bipolar cell junction. This is then followed by retinal arteriolar attenuation and the well described electronegative scotopic ffERG.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144316111","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":"Author index for the 2025 ISCEV symposium abstracts.","authors":"","doi":"10.1007/s10633-025-10032-3","DOIUrl":"https://doi.org/10.1007/s10633-025-10032-3","url":null,"abstract":"","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144301349","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}
Shijing Wu, Ailing Sui, Qianyi Zhan, Qiuli Fu, Li Zhang
{"title":"Retinal degeneration as an initial manifestation in a patient with neuronal intranuclear inclusion disease.","authors":"Shijing Wu, Ailing Sui, Qianyi Zhan, Qiuli Fu, Li Zhang","doi":"10.1007/s10633-025-10035-0","DOIUrl":"https://doi.org/10.1007/s10633-025-10035-0","url":null,"abstract":"<p><strong>Background: </strong>Neuronal intranuclear inclusion disease (NIID) is a rare autosomal dominant, progressive neurodegenerative disorder characterized by a broad spectrum of clinical conditions, including pyramidal and extrapyramidal symptoms, cerebellar ataxia, cognitive decline and dementia, peripheral neuropathy, and autonomic dysfunction.</p><p><strong>Case report: </strong>A 56-year-old woman presented night blindness since her youth, followed by tremors. She complained diminished vision in the left eye attributed to the presence of a macular hole. Ophthalmic examination showed obvious retinal degeneration in both eyes. Genetic testing result identified a heterozygous CGG repeat expansion in NOTCH2NLC gene. Visual acuity of left eye improved after vitrectomy.</p><p><strong>Conclusions: </strong>We reported detailed clinical features and genetic analysis of a new Chinese NIID patient who ocular symptom was the initial manifestation. And this was the first report of surgical case of a macular hole in NIID patient.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144293504","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}
Herman E Talsma, Gerard C de Wit, Charlotte C Kruijt, Stefan H L Zwerver, Maria M van Genderen
{"title":"Nystagmus characteristics and their impact on pattern-reversal VEP in patients with albinism.","authors":"Herman E Talsma, Gerard C de Wit, Charlotte C Kruijt, Stefan H L Zwerver, Maria M van Genderen","doi":"10.1007/s10633-025-10026-1","DOIUrl":"https://doi.org/10.1007/s10633-025-10026-1","url":null,"abstract":"<p><strong>Purpose: </strong>To describe the association between nystagmus characteristics and pattern-reversal VEP (prVEP) P100 amplitude and peak time in patients with albinism.</p><p><strong>Methods: </strong>We analyzed the prVEP (60' and 15' checksize) and nystagmus recordings from 47 patients with albinism, 17 with a relatively good visual acuity (≤ 0.3 logMAR), and 30 with a relatively poor visual acuity (≥ 0.6 logMAR). The nystagmus waveforms were classified into two types: dominantly pendular and dominantly jerk. We correlated the nystagmus type, amplitude, frequency, and percentage of low velocity (PLOV) to P100 amplitude and peak time.</p><p><strong>Results: </strong>For most patients (87%), reproducible responses were observed for the large checks (60'). Among patients with good visual acuity, 94% had reproducible responses of which the majority (82%) of P100 amplitudes fell within the normal reference range. In contrast, although 83% of patients with poor visual acuity showed reproducible responses, only a minority (17%) of P100 amplitudes were within the normal range. The P100 amplitude to 60'check sizes was statistically correlated with PLOV (r = 0.58, p < 0.0001), nystagmus type (r = -0.55, p < 0.0001), and nystagmus amplitude (r = -0.39, p = 0.0092). Patients with relatively good visual acuity and jerk nystagmus exhibited the highest PLOV and the largest P100 amplitude (p < 0.0001). In contrast, there was no significant correlation between P100 peak time and any nystagmus parameters in patients with good or poor visual acuity.93% normal peak time. For the small checks (15'), 76% of patients with good visual acuity, still showed reproducible responses, with the majority (71%) of P100 amplitudes falling within the normal reference range. In contrast, among patients with poor visual acuity, only 3% (1 patient) showed reproducible responses, but with amplitudes below the normal range. For the patients with good visual acuity, PLOV showed a significant correlation with P100 amplitude. P100 peak time was normal for 77% (10/13) of these patients.</p><p><strong>Conclusions: </strong>For the prVEP with 60' checks, nystagmus in patients with albinism predominantly affects the P100 amplitude but not the P100 peak time. For 15' checks the amplitude is often so small that clear responses are no longer discernable, especially in patients with poor visual acuity.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144207978","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}
Jonathan A Alexis, Prathiba Ramakrishnan, Matthew K Kenworthy, Jennifer A Thompson, Enid S Chelva, Fred K Chen
{"title":"CTNNA1-associated retinal dystrophy: novel multimodal imaging and electrophysiology features.","authors":"Jonathan A Alexis, Prathiba Ramakrishnan, Matthew K Kenworthy, Jennifer A Thompson, Enid S Chelva, Fred K Chen","doi":"10.1007/s10633-025-10027-0","DOIUrl":"https://doi.org/10.1007/s10633-025-10027-0","url":null,"abstract":"<p><strong>Purpose: </strong>To describe multimodal imaging and electrophysiology features of CTNNA1-associated retinal dystrophy in a family with p.(Leu318Ser) substitution.</p><p><strong>Methods: </strong>Three family members including a 48-year-old male proband, his 52-year-old sister, and their 67-year-old mother, were evaluated with multimodal imaging and electrophysiology. The proband, referred with suspected Best's disease, underwent a retinal dystrophy panel and two affected family members were target sequenced for the familial variant.</p><p><strong>Results: </strong>The NM_001903.5:c.953T > C variant in CTNNA1 segregated with affected family members. They maintained a visual acuity of 20/25 or better throughout 2-4 years of follow-up. The proband exhibited butterfly-shaped pigment dystrophy whilst his sister had no macular lesions, and their mother had foveal pigmentary changes. All three displayed peripheral retinal reticular pigmentation with variable atrophy. Microperimetry demonstrated enlarging paracentral scotoma in the proband whilst Esterman binocular suprathreshold test showed reproducible peripheral loss in the proband's sister. Multifocal electroretinography (ERG) confirmed central macular dysfunction in the proband. In all three, full-field ERG showed mildly delayed dark-adapted (DA) 0.01 b-wave and DA3.0 a-wave, and a light-rise of < 1.7 in one or both eyes on electro-oculography (EOG).</p><p><strong>Conclusions: </strong>CTNNA1-associated retinal dystrophy due to p.(Leu318Ser) has a unique peripheral retinal phenotype despite variable macular involvement. Reduced EOG light-rise and peripheral reticular pigmentation should raise suspicion of CTNNA1 in butterfly-shaped pigment dystrophy.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144198463","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}
Nicole Manfredonia, Hannah M Follett, Phyllis Summerfelt, James A Kuchenbecker, Joseph Carroll, Ching Tzu Yu
{"title":"Evaluating the repeatability of the multifocal electroretinogram in the cone dominant 13-lined ground squirrel.","authors":"Nicole Manfredonia, Hannah M Follett, Phyllis Summerfelt, James A Kuchenbecker, Joseph Carroll, Ching Tzu Yu","doi":"10.1007/s10633-025-10010-9","DOIUrl":"10.1007/s10633-025-10010-9","url":null,"abstract":"<p><strong>Purpose: </strong>To assess the repeatability of the multifocal electroretinogram (mfERG) in the 13-lined ground squirrel (13-LGS).</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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).</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":"117-126"},"PeriodicalIF":2.6,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12216646/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143604279","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}
Henrike Marie Nowitzki, Michael B Hoffmann, Khaldoon O Al-Nosairy
{"title":"DTL versus skin electrodes in recording of multifocal pattern electroretinogram and multifocal photopic negative response.","authors":"Henrike Marie Nowitzki, Michael B Hoffmann, Khaldoon O Al-Nosairy","doi":"10.1007/s10633-025-10014-5","DOIUrl":"10.1007/s10633-025-10014-5","url":null,"abstract":"<p><strong>Objective: </strong>To compare the photopic negative response of the multifocal ERG (mfERG<sub>PhNR</sub>) and the multifocal pattern electroretinogram (mfPERG) using DTL electrode (E<sub>DTL</sub>) vs skin electrode (E<sub>SKIN</sub>) in healthy young and old adults.</p><p><strong>Methods: </strong>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<sub>DTL</sub> and E<sub>SKIN</sub>. 5-way ANOVAs were applied to investigate the following factors on mfERG<sub>PhNR</sub>: 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).</p><p><strong>Results: </strong>Amplitudes of mfERG<sub>PhNR</sub> [mfPERG]-based E<sub>SKIN</sub> recording were reduced to 32-38% [37-38%] compared to E<sub>DTL</sub>, <math><mrow><mi>p</mi> <mo><</mo> <mn>0.001</mn></mrow> </math> . This corresponded to SNR reduction to 80% [60%], <math><mrow><mi>p</mi> <mo><</mo> <mn>0.001</mn></mrow> </math> . E<sub>SKIN</sub> based responses had shorter peak times, by 0.2-0.5 ms for N1 and P1, <math><mrow><mi>p</mi> <mo><</mo> <mn>0.05</mn></mrow> </math> , [P1: 1.5 ms, <math><mrow><mi>p</mi> <mo><</mo> <mn>0.001</mn></mrow> </math> ]. Both age groups had comparable amplitudes and SNRs, but Young had shorter peak times, by 1.5-2.2 ms for N1 and P1, <math><mrow><mi>p</mi> <mo><</mo> <mn>0.05</mn></mrow> </math> [3.7-4.2 ms for N1, P1, N2, <math><mrow><mi>p</mi> <mo><</mo> <mn>0.05</mn></mrow> </math> ]. Compared to dilated recordings, undilated mfERG<sub>PhNR</sub> amplitudes were reduced to 47-87%, <math><mrow><mi>p</mi> <mo><</mo> <mn>0.01</mn></mrow> </math> , and peak times were delayed by 2.0-11.8 ms, <math><mrow><mi>p</mi> <mo><</mo> <mn>0.001</mn></mrow> </math> .</p><p><strong>Conclusions: </strong>mfPERG & mfERG<sub>PhNR</sub> traces were similar for E<sub>DTL</sub> and E<sub>SKIN</sub>. However, for skin electrodes, amplitudes and SNRs were lower and peak times shorter. E<sub>SKIN</sub> 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<sub>SKIN</sub> normative data.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":"137-153"},"PeriodicalIF":2.6,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12137451/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143968977","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":"Case of uveitis with increased electroretinographic amplitudes following Nivolumab and Ipilimumab administration for malignant melanoma.","authors":"Kumiko Kato, Ryunosuke Nagashima, Hisashi Matsubara, Yuka Yonekawa, Yuzen Kashima, Keitaro Mizumoto, Mineo Kondo","doi":"10.1007/s10633-025-10011-8","DOIUrl":"10.1007/s10633-025-10011-8","url":null,"abstract":"<p><strong>Purpose: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>ERGs may be a useful objective test for posterior inflammation induced by administration of ICIs that is not evident in OCT images.</p>","PeriodicalId":11207,"journal":{"name":"Documenta Ophthalmologica","volume":" ","pages":"183-188"},"PeriodicalIF":2.6,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143613708","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}